So much has happened , but the best part is that so much more is about to go on. On Monday, Rafi is going to go to camp for the first time. She will be with 15 other children who are all just a little older than her. She is so excited. The school she will be attending in the fall puts on this camp every summer so we are very excited that Rafi will get to be in the same environment, with the same people, when school starts. Even though she is 3 and a half years old, this is the first time she has really been with a good number of children on a daily basis. Then in August, after this camp is over, Rafi will go to something called Scribble Press for a week. They read stories, by the same author every day and then draw pictures based on the story. At the end of the week, every child gets a book of all their drawings for the week. She really is going to have a great summer, and that makes Jackie and I very happy.

Last I wrote we had just gotten back from Cincinnati. It took awhile before she could fully swallow, but she eventually did. Thankfully, we haven’t had any swallowing issues after. In April, Rafi had some blood work done and we found out she was still a little anemic so she got some red blood cells because her hemoglobin was at 6. She also needed another infusion of IVIG. IVIG is basically an off the shelf immune system. When your numbers a little low, it gives you a boost that lasts about 3 weeks.

Since April, Rafi has not had to take any of those very powerful antibiotics. That means she has the ability to fight the on again off again infections that before April required a 14 day course of those very expensive antibiotics. Get this, she hasn’t had a fever since the first week of April. So, we know that she has a fully functioning immune system now. In fact, she just had three childhood vaccinations while we were in Minneapolis.

Ah yes, we were back in Minneapolis last week for Rafi’s one year checkup. And as it turns out, Rafi loves to fly. She loves take off because the plane travels so fast and she gets pushed back into her seat. People actually commented to us how well behaved she was on the plane. We were really proud parents. Anyway, she had blood tests, the skin fragility test (aka blister test), skin biopsies and photography session, all while under anesthesia. So far the big news is how the blister test came out. In case you don’t remember, they try to form a blister by using low power suction. A person who is not affected by EB would take a little over an hour to form a blister. It took me a little over 45 minutes and Jackie about 40 minutes. Before transplant a blister formed on Rafi in about 3 and a half minutes. The next time it took 12 and half minutes and last week it took more than 20 minutes. So by that test, we are definitely headed in the right direction.

With all that good news, it is still important to understand that she still suffers from EB. We still wrap her in bandages, she still gets blisters and open wounds. Sometimes she blisters a lot, and sometimes not. Sometimes her skin is just so fragile it’s like tissue paper. Other times, it looks better. She has developed more corneal abrasions than ever before. In fact, they seem to happen once a month now and she won’t open her eyes for 4 days. It is so sad because she will say to us that she really wants to see. So we are now going to try and get her to use drops and eye gel at night to keep them less dry and thus hopefully have her suffer less eye issues.

People tell us that her skin looks a lot healthier than before so we have to rely on their opinions. There have been times when both Jackie and I have said we don’t think the transplant has worked. There have been times when we think we put Rafi through this incredibly difficult journey for nothing. Sometimes it is hard to see the positive effect the procedure has had on her. Then out of nowhere she will go through a healing phase. Like now. There are parts of her body that just look great. Her stomach has not had a problem in a long time and it used to be a bad area. Most parts of her legs look great as do most parts of her arms. Her forehead is always problematic but it seems to be moving in the right direction. People in Minneapolis said how much better it looked and I don’t think it was them saying it to just be nice. Funny enough, before we left Minneapolis in December we took Rafi to a mall where there is a carousel. She loves carousels and we promised to take her back there before we left this time. On Thursday morning, we went there and the same woman was operating the ride and she remembered Rafi.

While in Minneapolis we got to see the new hospital. We went up to the transplant floor and some of our old nurses gave us the tour of the new rooms. It could very well be the nicest hospital I have ever seen. Others will blog about it in more detail, but trust me when I say that it is truly amazing. If someone who reads this knows Dr. Wes Miller, please tell him that Rafi’s new name for him is Wild Wild Wes, he is no longer potato.

Rafi also got to see her former nurses when we were out there. In fact, Stephanie, aka hot dog, took time off from work so she could spend time with Rafi. We saw Lindsay, aka stinker, and Karen, aka egg roll, as well. Rafi is a little like former president Bush, she has a nickname for everyone, i am pizza and Jackie is coffee. Anyway, it was nice to see everyone. We were not able to visit with the people in our old building, Heritage Landing, unfortunately. We really wanted to because they were so good to us, but time got away from us and it was time to leave. We will see you guys next time I promise.

It’s almost been a year since we first went into the hospital to begin this journey. Now we are back home in New York City, in a new apartment, and still trying to stay one step ahead of EB, not often successfully either. We have been so incredibly busy since we left Minnesota that we are lucky if we get to sit down as a family for a dinner. It’s been a long two and a half months since I last wrote. I do apologize for not updating, so many have written emails asking if Rafi is doing alright. Is she doing alright? The answer is complicated.

I think I will work backwards on this one this time. We just got back yesterday from Cincinnati where Rafi had an esophageal dilatation. It was the first time we met with Dr. Richard Azizkahn and he is just wonderful. He exudes confidence in an unassuming way. Anyway, Rafi needed to have her third dilatation in a year. Turns out her esophagus was about 1.5 millimeters wide. The average size of an esophagus at her age is 14 millimeters, so it was quite a stricture. We drove the 650 miles on Tuesday, got caught in the huge storm and had to pull off the road about 70 miles from Cincinnati. It was one of the first times I was ever really afraid of driving. Rafi had the procedure on Thursday and we drove back to NY on Friday. About 24 hours in a car for an hour and a half in the operating room. As of today, Rafi is still not swallowing. Because steroids, an anti-inflammatory, are also an immunosuppressant, Rafi can’t really have them. So instead of the normal 5 doses given to those who get this done, Rafi only got one dose. Everyone is hopeful that Rafi will start swallowing in the next day or two.

Otherwise, the past two months have been about low grade fevers, being on and off some strong antibiotics, which reek havoc on her skin, and always being on the verge of going to the emergency room. She has had a lot of open wounds which get infected quickly. I think we could bath this kid in just bleach or vinegar alone and it wouldn’t help. So now on top of the staph, Rafi is also growing out klebsiella and pseudomonas. These are the first fungal issues she’s had in a long time, probably 18 months.

Although in the past week and a half, Rafi’s wounds have been healing up nicely. It’s been six months since the rituximab and they say that is what it takes to recover from it. Hopefully, that’s true and we can get back to where we where before the Epstein Barr Virus and the 4 infusions of Rituximab. We are hopeful that Rafi will now be on the upswing and will show all the positive effects of the transplant that we were seeing 8 months ago. The past month has been hard because she hasn’t been able to swallow, and therefore she can’t talk very well. This is a kid who loves to talk. So in the brief moments when she can, she is all smiles and animated. Before all this happened, she was clearly not feeling well as her behavior was different. She was difficult and moody, and this is beyond the normal 3 year old fun.

To top all of this off, I have gone back to work full time and this means I am out of the house most of the time. Unfortunately, that leaves the burden of taking care of Rafi square on Jackie’s shoulders. I feel terrible about it, but I guess there isn’t really much else I can do. I have been working from home since Rafi was born but I had to go back to an office. Jackie is strong and I have become rather useless in helping with the bandages.

I know that many of you know that I have become the new Executive Director of the Debra organization. I hope I can due some good for those that suffer from EB. There is a lot to do, so if I don’t post as often as I should, I apologize.

Wow it has been a long time since I wrote anything. I keep telling myself I have to be better about this. I am sorry but it has been busy around here. Since I last wrote anything, we’ve sold our New York apartment, rented a new one, sold our car, and leased a new one for the drive home. Yes, we are headed home to New York City in two weeks time. It will be nice to be back in New York amongst friends and family and away from this cold, cold weather. There are certainly things that we will miss about Minneapolis, but we won’t miss a high of 9 degrees in the first week of December.

Rafi has gone through a tough stretch but we are hopefully beyond that and into a healing mode. Of course, the very powerful antibiotics she is now on will certainly help. She continues to be her own worst enemy by literally scratching herself until she bleeds. Her skin is still very fragile in areas and of course she knows those areas well. With new open wounds come infections and with a still forming immune system (minus the B cells thanks to rituximab) she has a tough time fighting off the bacteria. We always know what is coming down the pike when she wakes up sweating. It is always a portent for higher fevers during the day. With that in mind, we were very proactive in getting enough medical justification together for the $1,000 antibiotic, Zyvox. Insurance just loves cutting a check for that one, but they always do, albeit kicking and screaming. I think we’ve beaten them into submission, because we didn’t even get a request for justification this time. I love that we’ve gotten the insurance company into a state of learned helplessness.

So, Rafi’s temperature curve was slowly on the rise until one day she spiked a 101.6 fever. Thankfully Tylenol took care of that and the next day she had a 100.4 while on Tylenol. That’s the day we started the antibiotic and she hasn’t had a fever since. Her self inflicted wounds are starting to look better, except those on her feet. The poor little one just has fragile feet. We hope over time enough collagen VII will build up down there.

Yesterday we had our final appointment in Minneapolis with Dr. Tolar. He considers Rafi’s transplant a success as we have evidence of collagen VII where there once was none and evidence of anchoring fibrils where there once was none. Rafi’s internal organs are all functioning very well, and she is actually growing for the first time in over a year. We have a lot to be thankful for, mostly for having such a strong kid. Rafi will certainly end up being one of the success stories from this trial. It’s amazing to me the events that have happened since Rafi was born with regards to this transplant. I remember reading about the first transplant here when Rafi was still in the NICU in New York. I remember promising Jackie that I would get us here for this procedure. I remember writing a long paper to the insurance company justifying the need for them to pay for this experimental treatment. I remember getting the call from our transplant coordinator telling us that the transplant was approved and having tears roll down my face. I remember the days of chemotherapy, of the terrible effects of those drugs, of the healing, of the wonderful doctors and nurses. I remember having the fellow, now an attending, tell us that in 3 minutes time, Rafi would be intubated to save her life from pneumonia. I remember the look on the faces of all the doctors and nurses who saw the image of her lungs and you could tell they didn’t think she was going to make it. I also remember the look on all their faces when she recovered so quickly. I’ll never forget the fear and the anguish this procedure brought on my family. But in the end, I’ll never forget looking at the picture Dr. Jakub Tolar sent me that showed a bright red line at the dermal epidermal juncture, representing collagen VII, and him writing to me, I think you should have a scotch. And now, the story continues……

I will try to post more often but the next few weeks are going to be very busy. Maybe the next post will be from our new home in New York City. Same old town, new apartment. A new start for the little one.

Just a quick update to give some good news. It’s nice to be able to write and not have to report any kind of bad news at all. In thinking about going home and having Rafi get involved in some age appropriate play time, Jackie wanted to speak with Dr. Tolar about how careful we had to be in exposing Rafi to other children who may be in day care/preschool. After all, Rafi doesn’t have a fully functioning immune system, and one that is B cell comprised on top of that. Add to that fact that flu season is upon us. Needless to say I was a tad petrified thinking about the number of germs that Rafi could potentially be exposed to.

I am a little more insistent upon keeping Rafi in a little bubble for a period of time until her immune system is mostly back. Jackie rightly wants her to have time with kids her own age. If you think about it, with a few exceptions, Rafi has not played with kids her own age in close to a year and a half. When she is at the hospital for physical therapy or some kind of therapy, Rafi will stare at the children and be infatuated with them. She needs to be around kids, and Jackie is more insistent on that fact than I am. For good reason too, I am sure Rafi is behind other kids her age when it comes to interaction skills and such. She’ll catch up, she is certainly smart enough to learn quickly how to interact with other kids, but imagine your kid not playing with other kids for close to a year and a half. It certainly makes me sad but I know it is for the best.

Anyway, yesterday, Jackie and I spoke with Dr. Tolar about this and we got the thumbs up for intelligent child interaction. What is intelligent child interaction you ask? Well, we still need to monitor, to a degree, if whoever she is going to play with has been exposed to sickness. Dr. Tolar told us we should trust the parents of her friends to be vigilant. There is a leap of faith for you for sure. Thankfully, Jackie has surrounded herself with some great mothers who will always keep Rafi’s best interests at heart.

Now for the really great news. Rafi doesn’t have to wear her blue mask as much anymore. She can go outside without the mask. I can only imagine how nice that feel of fresh air on her face is going to feel. The smell of the outdoors will hopefully make her smile. She can go into stores, at off times, without her mask. I smile just thinking about it. Rafi has surpassed another milestone and Jackie and I couldn’t be happier.

There has been a lot going on here in Minneapolis with little Rafi. The little one has needed IV medications, she’s had some low grade fevers, and one high one. We’ve had a trip to the ER and a few sprints to the BMT clinic. The trip to the ER was completely and utterly my fault and that botch led to a little surgery, but I’ll get there in good time.

Since we got out of the hospital last month from a bout of sepsis brought on by staph infections in her lines, Rafi has been up and down. She still has no B cells so anything can cause a fever and another trip into the hospital. For two weeks after our last hospital visit, Rafi was on a strong IV antibiotic that we had to give her every 6 hours. About a week after we finished that round of drugs, Rafi started to sweat at night and her daily temperatures started to increase. She also started throwing up again. It was only a matter of time before we ended up back in the hospital or here at home on IV meds. So, after a temperature spike of 102.9, and two days of IV vanco, the incident occurred.

First some other information for you EB families that still follow this at all. For two years, Jackie and I have been mixing bleach with soap and water for Rafi’s daily bath. We were never really able to control the staph infections, only keep them at bay. One day in clinic with Dr. Tolar one of the two visiting physicians from Toronto said we should try pool salt in our bath water to better control the infections. In doing some research about pool salt, I came upon some information that should have been given to us years ago. It’s a simple chemistry lesson, and one that every pool owner knows. In order for bleach to be effective you need to monitor the PH level of the water. If the water is too alkaline, chlorine loses its effectiveness. By adding soap to the water we are changing the PH levels. So, we stopped adding soap to the water, and guess what happened, we got the infections under control. Why couldn’t a dermatologist tell us this? Why couldn’t anyone tell us this? Some times this is so frustrating.

Excuse the digression. Last Wednesday, at the end of our bath and bandage change, I really screwed up. I had just cleaned her central line site, and put on the appropriate dressings and then it happened. In trying to trim one of the bandages, I cut of one of the lumens of the central line. For those that don’t know what a central line is, it’s a plastic tube that is inserted into the chest that travels into the heart. On the outside of the body, the tube splits into two lines or lumens (at least in Rafi’s case it’s called a double lumen hickman). Anyway, I cut one of the lines completely. Within seconds, both Jackie and I are running in separate directions grabbing the special clamps in case one of the lines is ruptured. It’s important to clamp off the line, because now you have a direct line to the heart that is open. Blood would start pouring out of that line shortly. We had to get to the ER quickly to get the line removed. It was 5:30pm, and we thought we would need emergency surgery on an EB kid. Not a great thought to say the least.

I am on the phone with the BMT fellow, commanding her to get in touch with a specific surgeon and one of two anesthesiologists, as well as Dr. Tolar. We get to the ER, and as it turns out, the surgeon we wanted is on call. He tells us that we can create a temporary fix to the line, he’ll make sure all EB friendly people are in the OR, and we’ll get it done in 24 to 48 hours. This was the surgeon who put in the line and Rafi’s Gtube back in January. He did both procedures and there was no collateral skin damage. None, on an EB kid. A severely affected one as well. I was amazed. This guy is truly a great surgeon so both Jackie and trust him implicitly.

The question was whether to replace the central line or give Rafi a PICC line in her arm. She still gets some blood tests and it’s likely she will need some medications in the near future. I mean she just had a high fever and was on IV meds and she has no B cells. In discussing this ad nauseum with Dr. Tolar, we decided that we would just pull the line and if she needed a PICC line later, we would throw it in. We weren’t really using the line anymore anyway. Rafi’s been off TPN for two weeks and everyone thought that the line was the reason for the fevers anyway. So, on Friday morning, we once again brought Rafi to the hospital for yet another procedure, albeit one we planned on soon enough. It went great, the procedure was completed quickly and once again, there was no skin damage whatsoever. Her line is gone. It’s yet another huge milestone that Rafi has achieved.

Since the surgery, Rafi hasn’t had a fever and she’s stopped sweating at night. Her skin is healing up nicely after a good two months of incredibly fragile skin. I think we have once again turned a corner and I hope we have some straight road ahead of us. I am really tired of the twists and turns.

In dealing with all of this, we decided to stay in Minneapolis for another month. Maybe we’ll leave in the middle of December, maybe we’ll leave toward the end of the month. All I know is that I don’t have to clean those damn lines again.

We have been out of the hospital for a little bit now. We got the blood infection under control with a lot of IV antibiotics. Actually, we just finished the course of medicine a day ago so it is nice to not have to hook her up every 6 hours. On top of all that, Rafi was having these episodes where she couldn’t swallow anything, including her own saliva. Everyday, we would have these episodes that could last 5 or 6 hours at a time, where Rafi would just drool. This went on for a full week. At some points, the mucus got so thick, well I don’t need to go into too much detail about that. We didn’t know if the cause of this was a stricture in her esophagus, blistering, fungal or viral. In order to try and rule things out, we scheduled an esophagram. This is where Rafi swallows some contrast and they xray as she swallows to see if the cause of this is a stricture in her esophagus. We did this yesterday and as it turns out, the past stricture seems to have gotten better and her throat has opened up a bit. This was a little surprising to us, but we’ll take it. AS it so happens, these episodes stopped three days ago, so we will take that too.

If you remember, Rafi had to have some infusions of Rituxan to get the Epstein Barr virus under control so it didn’t turn into non-hogkins lymphoma. Rituxan destroys the B cells, which are part of the immune system responsible for killing off infections. Her last infusion was August 10, and she still has no B cells and we are seeing a major difference in areas of her skin. I postulate that these B cells play an important role in skin fragility, because there are areas of her skin that are more fragile than pre-tansplant. Conversely, there are some areas of her body that look like completely normal skin. So, in order to try and help her along, we are going to have and infusion IVIG on Monday. An IVIG infusion consists of the donated B cells from 1000 people. It is very similar to getting a blood product, like red cells or platelets. The hope is that these B cells can help Rafi fight off any infections for a period of time, until her new, thanks to the parents of a little german girl who donated their umbilical cord blood, B cells can come back. In general, after Rituxan, it takes about 6 months for those ever important B cells to populate the body.

It is important to say with all this in mind, that all of Rafi’s cells that she produces are of donor origin. Meaning the transplant has worked for her. The chance of her losing the transplant at this juncture is very low. At the last skin biopsies, the skin chimerism showed 20% donor cells in the skin. This is still great news. We know that after a little Rafi will be back to where she was a few months after transplant. The Rituxan set us back 6 months, but we always knew for every 2 steps forward there could be 3 or 4 steps backwards.

For those that don’t know, you don’t always take steps forward when going through a stem cell transplant. Unfortunately, on October 11, Anabella Ringgold, an incredibly cute one year old, lost her battle with EB from complications stemming from the transplant. She was patient number 11 in the trial, and the third casualty. We have become very friendly with her family as they have gone through the procedure, and we feel just horrible for them. Tim, Bella’s dad, continues to find the silver lining in everything, including this tragedy. We wish them the utmost luck and courage in dealing with their loss and in trying to get back to life without their darling daughter.

This past weekend, we had some friends in from New York City with their little girl, Lillie Ann. Lillie and Rafi were in the same playgroup back home and we have become very close to her parents, Julia and Ben, over time. They drove out from New York, 1200 miles one way, to spend some time here. It was great to see them and for Rafi to play with Lillie for a few days. When they had to jump in their car for the long journey home, Lillie and Rafi broke down in tears and had temper tantrums because they didn’t want to be separated. I can honestly say that it was incredibly nice to witness a temper tantrum. This will be the only time you ever hear me say that. Now next weekend, Jackie’s father and sister will be here and I know that will make Rafi very happy.

Ok, I really have to catch up on some work now. I will absolutely post more frequently, I am sorry that my laziness has made so many worry so much. Furthermore, I will post some pictures of Rafi and her buddy, Lille Ann.

Here we are once again in the hospital. 222 days post transplant and we are in the room adjacent to our original room. Rafi has been running some very high fevers the past few days. Last night was 104.2, the night before, 104.8, ugh. We will be here in the hospital for at least 2 more nights, perhaps more. The nice thing is that We have found the source of the fevers for a change. It seems every couple of weeks Rafi spikes a fever but instead of constantly being admitted to the hospital, we’ve been able to give either IV or oral antibiotics at home that tend to do the trick. Not this time. On Monday Rafi spiked a 102.6 fever and after speaking with the clinic, we decided it best to do her bath and bandage change, and then go into the ER to get some blood cultures taken. So we did, and in the ER, after they flushed her lines, Rafi got a 104.8 fever. After some celebrex and tylenol, her fever went down so we were sent home. The next morning however she spiked another fever and it was high so into the hospital we went.

Everyone figured that there was an infection of some sort in her central line. When you flush it with saline and then spike a high fever that is a sign that all is not right. Sure enough, the cultures came back positive as a staph infection so here we are for a few days. Now we are on several IV antibiotics and for 12 hours a day, Rafi has ethanol in her central line to try and kill off the staph infection. Let’s keep our fingers crossed. The other option is to send Rafi into the OR to have the line removed and have a new one placed. We really don’t want to do that, so we are hoping the combination of ethanol, vanco, and ceftaz does the trick.

On Tuesday, when Rafi was speaking with her aunt, Emily, Emily asked if she had spent the night in the hospital. Rafi responded, “No, I was in the eerra.” Too funny.

Prior to all this happening, one of our nurses, Lyndsay, who Rafi calls, Stinker, arranged for a friend of hers to bring over their two little girls for a play date. Jackie really wants to integrate Rafi back into playing with children her own age because one of the largest pitfalls of this past year has been Rafi’s inability to have time with kids her own age. Rafi has always been a little shy and we were hoping that she might just really like playing with kids her own age now that she has come out of her shell to a large degree. On the day of the play date the two very cute little sisters came in and just hit it off with Rafi. It was very exciting for us to see Rafi actually interacting with kids her own age. We hope to be able to have some more time with that family. On another note, in a couple of weeks, we have some friends coming into town with their daughter, Lilllie Ann, who is Rafi’s age. Lillie Ann was part of the play group Rafi was involved with back at home. It will be great to see our friends and to watch Lillie Ann and Rafi create a mess out of our apartment.

I can’t believe how much time has passed since I last wrote. I am not even sure where I left off. My mistake and I will not let it happen again. A lot of people have been asking me to write something but it’s been a little hectic around here lately. Right now, we are at a constant battle against skin infections which can lead to fevers, which leads to time in a hospital. Needless to say we’ve been back on 5D and then home again. We’ve been forced to run to clinic to get blood cultures and get some IV antibiotics. So it’s been stressful to say the least. The Rituxan that Rafi received which so far has been quite effective in killing off the epstein barr virus, also wiped out her ability to kill off any kind of infection. Rituxan destroys the B cells which are part of the immune system which is responsible for fighting any type of infection. Rafi is a walking staph infection so we are constantly playing catch up against them. So, Rafi has had several courses of some pretty strong antibiotics lately. We believe the Rituxan, the antibiotics and her body trying to beat these infections has taken a toll on her skin. Her skin seems to be more fragile now than it has been in months. The same old places are getting affected and it sometimes feels like we are right back where we started. There are times when it is quite frustrating and upsetting. But then, we look at some other parts of her body and they look amazing. For instance, from her ankles to the top of her thighs look unbelievable. They really look like a normal child’s legs. A couple of other areas keep us thinking positively. But in thinking about our future plans, we’ve decided to extend our stay in Minneapolis an extra month to give her body more time to develop those really important B cells. The hospital here is so great in so many ways that it just makes sense for us to stay if there is a possibility we are going to continue to be a frequent flyer into the hospital.

So to add another complication into the mixture, bath and bandage changes have been incredibly difficult lately. Rafi is anticipating pain more now and she flies off the handle at the mere thought of changing a bandage. We’ve tried the pharmaceutical route and decided that we didn’t want to have a child on Valium constantly so we are trying a ton of different behavioral tricks. The past couple of bandage changes have been good so we hope our luck is changing. The nice thing is that most of her wounds are healing up nicely right now, so they probably don’t hurt as much as before. But in some of the past bandage changes this past month, we’ve had to apply pressure to keep her still at times. I mean we’ve pinned her down to stop her from squirming around. One such episode of hers caused a tremendous amount of damage to her left hip and buttocks. Of course these are areas that are very difficult to bandage. Anyway, before transplant, the amount of pressure I have used to keep her still would have been enough to cause some pretty horrific wounds on her. Turns out, nothing has happened so once again here is something to think positively about.

Rafi has become iphone obsessed as well. It is our fault but she really knows how to operate the device. It is really funny to see. Also her vocabulary has made a huge jump and she is conversing very well in full sentences. Of course, she has also embraced with all possible enthusiasm, the idea that she is almost three. Temper tantrums, time outs, explaining good from bad behavior is a normal daily activity. Oh what fun a three old with EB really is.

There is more to say but unfortunately our dinner is at the door and I have to go now. I promise to write more tomorrow. There is more to say.

I wish I could say that 6 months just flew by. It hasn’t. The road has been long, sometimes frustrating, sometimes nerve racking, yet we are fortunate. Rafi is taking forward strides. She is a wonder to resiliency. She makes Brett Favre look like a wimp. People have asked, knowing what you do now, would you put Rafi through this procedure? The answer is an unequivocal yes.

Yesterday was our 6 month biopsies, skin fragility test and photo session. Dr. Kooy, who was Rafi’s physician while in the intensive care unit, was the man in charge of keeping her sedated through the procedure. He thought she looked great since he last saw her. he was taken with the skin quality and coloration. Dr. Tolar was equally impressed with her progress. Overall, Rafi’s skin looks great, even though she is going through a period right now where her skin is very fragile. She has more small wounds on her neck, face, thighs and upper arms than she has in months. We all think that the 4 weeks of rituxan has made her skin fragile. The wounds do heal much more quickly than pre-transplant, the is if she doesn’t continually re-injure them. You can ask Rafi, “what happens when you scratch?”, and she will answer “I hurt myself”. Intellectually, she knows to tap instead of scratch and we witness her tapping her arms while she is asleep. So she has clearly internalized that lesson. But tapping doesn’t always work and she ends up scratching until she rips her skin in certain areas. It is a vicious cycle. Regardless, her skin generally looks great. Areas that were problematic, are not anymore. There was a time when her calves would just blister and her skin would tear from us holding them as we cleaned and bandaged her feet. No longer. There are times when she will kick like a bucking bronco and I am white knuckling her calves and still no injury occurs. She can rub her eyes without tearing her eye lids. She tripped and did a face plant the other day. Before this procedure, that would have been enough to cause rapid blister formation on her cheek which would lead to a nasty wound that would take a month to heal. She didn’t even get a mark.

In regards to the skin fragility test, this is where Dr. Tolar forms three small blisters with a suction cup of sorts. In January, it took four and a half minutes for the first blister to form on Rafi. Yesterday, it took twelve and a half minutes. It took me about forty minutes to form a blister when I did it. So, as I said, we have forward progress.

Now for the other patient. Jackie spent a few days in the hospital and then had IV drugs for a few more days while at home. She is doing much better now and is almost completely healed. It was certainly nice to have both of my ladies home at one time. While Jackie was in the hospital, Emily, Jackie’s sister and Rafi’s favorite person in the world, flew out here on a moments notice to help. We certainly would have had a much more difficult time had Emily not been here and Rafi had a blast for a week.

We are now thinking about our trip home to New York. As it stands right now, we are planning on moving back east at the end of October. It will be great to get home to see family and friends again. There is so much to do before then and I am not looking forward to packing everything back up again but so be it.

Rafi has undergone three out of four rituxan infusions. Her epstein barr viral load had gotten as high as 80,000 before the infusions. That is a tremendously high number and really signaled that if we didn’t give her the rituxan there would have been a very good chance that she would have developed lymphoma. Fortunately, her viral load is now undetectable. We have had three test results confirming that level. So for now, there is one more thing we can cross off the list to worry about.

Not so fast about the don’t worry aspect of life. Once again we have a family member as an inpatient. Jackie was admitted this afternoon to the hospital. We went to the ER this morning after Jackie had been suffering for days with an infection in her arm. We thought, and hoped, that it would resolve itself. This morning she woke up in more pain and we felt it best to get it checked out. That led to IV drugs, a couple procedures and a ticket for a night in the hospital. Hopefully, we can get her home tomorrow.

People often ask how is Rafi’s skin and do we see a difference at all. There are areas that used to always have a wound of some sort on it. Her right cheek, forehead and eyelids were problem areas before the transplant. Now they look great. She rubs her eyes and doesn’t hurt herself. Her calves used to always have worn away skin from holding them during bath and bandage changes. They don’t anymore. In fact, we are thinking of not even bandaging them anymore. We don’t bandage her wrists anymore and nothing has happened there. She is her own worst enemy though. She is constantly causing problems on her upper arms and by her stomach. Her neck is also prone to wounds. Her feet get pretty beat up as well. The worst part is, her wounds now get infected very quickly. We are in a constant battle with MRSA. Bleach baths, silver gel, and polymem are constantly employed in the fight. We hope that with time and more collagen VII production that the number of wounds decrease.

Our biggest concern right now are her feet. EB is winning the battle with her feet. It seems like they are turning inward more and I fear serious contractures are not far off in the future. I hope we can avoid the use of a wheelchair.

That’s it for now. It’s only a matter of time before Rafi starts the nightly ritual of waking up often. I am alone tonight and it could be a long one.

Wow, ten days went by pretty quickly. I keep telling myself that I really need to write an update and then it’s the next morning. I have certainly gotten enough comments/complaints about my current inability to post something in a timely fashion. Before I start, here is a little treat:

Rafi and her magazines

This photo makes me laugh every time I look at it. Let me answer one quick question before I get deluged with it, no, she is not on her potty.

Last I wrote was to say that the inflamed lymph nodes were due to the epstein barr virus. Since then, her viral load increased significantly and then dropped to undetectable levels. I think I may have to buy some stock in the company that makes Rituxan. She’s had two out of her four doses so far and it seems to be quite effective. It looks like Rafi has stared down one more complication and overcome with flying colors. She is impressive.

Unfortunately, Rafi has embraced the terrible twos at this point. A little late perhaps, but definitely with the intensity of a college freshman at their first fraternity party. Overall, she is a very well behaved, polite child, who is really coming out of her shell, and then the beast hits. To top that off, she wants an iPhone. “Where is my iPhone?” I tell her that she has a couple of phones including a blackberry, but that is not good enough. Ugh, it starts early.

I have to get ready for Rafi’s bath and bandage change now, so today is a very short posting. She’s doing great.

So much has happened in the past four days that it might just be impossible to jot it all down. We’ve been to the ER, been in the OR, and were readmitted to our old hood, unit 5D. The hardest thing to figure out right now is where to start. I guess it’s best to talk about the relationship between the epstein barr virus, ptld and lymphoma. I made reference recently to a serious complication that can arise from the epstein barr virus. After all the research I did about transplant in general, ptld, or post transplant lymphoproliferative disease, really took me by surprise. The epstein barr virus is really just a type of cell within your immune system that proliferates through your body and causes infectious mono. In a person with a normal immune system, the symptoms can be a pain, they may take a while to get over, but eventually your T cells (suppressor and killer cells) get control of your B cells and you overcome the virus.

Unfortunately, those, like Rafi, who have just had a transplant, take an immunosuppressant to keep those T cells in check. T cells are really responsible for transplant rejection so you don’t want those T cells to be to numerous at first. Problem is those T cells are needed to keep those B cells in line. If the B cells are allowed to proliferate, they can mutate into a rather virulent form of cancer or lymphoma. In the case of allogeneic stem cell transplant recipients, the type Rafi had, the outcomes once you get to non-hodgkins lymphoma are not good at all. So last week, we learned that Rafi’s epstein barr virus had come back with a vengeance. She had a viral load of 2400, then 17000 then 33600. This called for a full body CT Scan to see if she had any enlarged lymph nodes. Sure enough, she had some pretty large nodes in her neck and in her armpits. This was enough to send our blood pressure through the roof. Due to the CT scan findings, we had to schedule a lymph node biopsy. Rafi would have to go into the operating room, go under anesthesia and have some cells taken from one or two lymph nodes to see if the inflamed lymph nodes were caused by the epstein barr virus or if it had morphed into ptld or lymphoma.

One of the only things that kept me sane, I can’t speak for Jackie on this one, is I knew Rafi had a good T cell profile at 100 days post transplant. Plus, the first line of defense against the virus, ptld and lymphoma for that matter, is to get off the immunosuppressant so that the T cells can come into the picture. It’s takes a while to get off the drug, Rafi’s taper down schedule was almost 3 months. She was in the middle of getting off the cyclosporine when the virus hit plus we then accelerated her taper to best allow her T cells to come in. Still none of this is a guarantee of anything.

On Sunday, Rafi spiked a fever. Normally that earns you at least a two day ticket into the hospital. The attending physician was quite logical and figured it was due to the virus, all of our numbers looked good, so he said we should just go to the ER, get some blood taken so they could run some tests just to make sure there was no bacterial infection, take a broad spectrum antibiotic and then go home. The next morning we were due into the hospital at 5:30am for surgery so it wouldn’t be a big deal. Jump to 6:30am on Monday, we are in preop, still waiting on anesthesia to come to the room to tell us who would be intubating Rafi. Jackie and I told surgery that we would only work with one of two anesthesiologists or we would cancel the surgery at the last minute. There was no way we were going to witness another traumatic intubation that would lead to enough inflammation to block her airway again. It just wasn’t going to happen. We made this perfectly clear to anyone who would listen, and to one or two others who didn’t want to hear it.

At close to 7am, after us pushing the nursing staff, in comes a representative who tells us that neither of our choice doctors were available to work with Rafi and someone else was scheduled. Jackie and I both looked at this woman and said, OK we are leaving right now. We made this perfectly clear and the surgery is off. Five minutes later, one of our choice anesthesiologists, walks in to our little preop room and tells us he would be our physician for the procedure. Jackie and I both let out a sigh of relief. Rafi had to have this procedure, and we just think this man is great. His name is Dr. Sarpal and he is the nicest guy who generally cares about his patients and does his best to make you, the parent, feel comfortable with what he is going to do and how he is going to do it. Knowing that I was going to be in the OR with him, he tells Jackie and I that he is not going to intubate Rafi, and instead use propofol to keep her sedated. He knew we didn’t want to have her intubated, he knew her history, had already intubated her twice, and felt that she could tolerate the procedure without the breathing tube. We really love this guy.

A few hours and two lymph nodes biopsied later, we were in postop and Rafi spikes a fever. We were due to be discharged in 40 minutes, when I got on the phone with the attending physician at the blood and marrow transplant clinic. She knows Rafi, Jackie and I well, and knows we would fight her on getting admitted. Dr. Margi MacMillan made some good points, so logic and safety won the day. We would be heading back to unit 5D just to be sure the fevers were not caused by anything else except the epstein barr virus.

There was one advantageous aspect to being the hospital today. Rafi was due to get her first dose of Rituximab today and there can be some pretty serious infusion reactions while you are getting the drug. It takes about 8 hours for the drug to be administered as well. Rituximab is a drug that targets B cells. While not technically chemotherapy it generally does the same thing without the toxicity, but infusion reactions can be fatal. Sure enough, Rafi had a reaction. She doesn’t complain about anything. I saw this kid go through hell with a smile on her face. Today she looks at me and says, “Daddy pick me up!” But not in a good way. As sh eis in my arms, I feel the trembling become more severe. I can feel her heart racing and she is complaining. So we slowed down the administration of the rituximab and gave her some demerol. That and some good time in daddy’s arms as she looked at mommy calmed her down. She stopped complaining and actually took a very short map. She will need three more doses of the rituximab, but we are told the first dose is the worst.

We’ve been on edge since Monday morning waiting for the biopsy results. We are admitted to the hospital, planning on leaving tomorrow, and in walks the attending who we have never met. The biopsy results are in, they came back faster than we anticipated and I literally stop breathing. Then I hear those words:

“There is no evidence of lymphoma”

I swear a tear just dropped off my face. The inflamed lymph nodes were caused by the virus and nothing else.

There really is nothing more to say.

I am sorry it has been such a long time since I last updated. The past week Jackie and I have been a little obsessed with our new iPhone 4s. They are fun toys. One of these days the novelty will wear off.

So, once again a lot has happened since I last updated. To recap, Rafi tested positive twice for the epstein barr virus (ebv) and then tested negative twice. We thought Rafi had beaten this latest set back. Turns out we were a little hasty with that thought. She has tested positive again the past two times. Unfortunately, her viral load has gotten very high. The EB virus can lead to some potentially serious complications post transplant. It is important to remember though that correlation does not equal causation. A high percentage of those suffering these severe complications have been EBV positive. Having the virus does not mean she will develop these complications though. However, Rafi’s viral load has raised suspicions. This gets us very nervous.

Today Rafi had a full body CT scan to see if she had any inflamed lymph nodes. Turns out, she does. Not exactly the news we were looking to hear. Again, this in itself only means she has a viral infection. We are trying to determine if we need to biopsy either of the enlarged lymph nodes, or if we just move ahead with a drug called Rituximab. More about this in a later post.

On a brighter note, we took Rafi to her first movie this past weekend. There is small theater just across the river that never gets crowded. So we took Rafi there to see Toy Story 3. Goodbye King Fu Panda, Shrek, Cars, Nemo, hello Woodie and Buzz. We take her into the theater not knowing how long she would last in a dark theater, with a large screen and a loud sound system. I didn’t expect to last the entire movie. it took a little over an hour before Rafi said, “I want to go home”. She got a little scared at one scene and that was that. Personally, I was impressed at how long she made it. She didn’t even talk very much during the film and when she did we would say to her “Use your inside voice” and she did.

Other than that, Rafi is now actually peddling her tricycle. She is running around the house and having a ball. She loves her bubbles and her nurses that come to the house daily. You would never know she has an active viral infection. Other than the inflamed lymph nodes, she is completely asymptomatic.

We are now off the immunosuppressant and the blood pressure medicine. Believe it or not, she now takes less medicines on a daily basis than she did before the transplant. So that’s exciting.

The cost of the transplant is now coming into focus. I have entered into excel all of the explanation of benefits from blue cross blue shield. As of right now, the hospital has charged a little over $542,000. I still don’t have many charges yet. As a matter of fact, I don’t have a single charge from March and have not received many from February either for that matter. Those two months will be expensive ones as well. The insurance company has paid a total of $292,000, or about 54% of the total bill. I tell you I am baffled at the system. I know how to play it, but it just makes no sense to me. The hospital will charge, $100 for something and the insurance company will discount it $42.03. HUH? Whatever.

Is there anyone reading this from the Washington, DC area? If so, a friend of mine, who is the father of an EB child, is testifying in front of a Senate committee about the need for more funding for research for orphan diseases and EB in particular. If you live in or will be in the DC area on July 21, he sure could use some support. If we could get some EBers there that would be great. If you are able to attend please email me and I will give you further details. He has worked exceptionally hard in making this happen, so some support is deserved.

OK I am sorry it has been so long. It’s been a bit of a stressful week and we are generally positive that we have turned the corner. A lot has happened since my last post so let me get everyone up to date. We are now home and have been since this past weekend. Rafi went from being a high risk for intubation to doing pretty well. She surprised a lot of people. I wish I could say that was the last time I would write that statement.

The day we left the hospital we were informed that Rafi tested positive for the Epstein Barr virus (EBV). About 90 – 95% of us have this virus lying dormant in our system. It’s really just mono. So for the normal person, suffering from an outbreak of this virus is trying but in the end no huge deal. In the immunocompromised, particularly those who just had a transplant, the virus can prove quite dangerous if left unchecked. Unfortunately, there really isn’t much to do about it. The first line of defense is to quickly reduce the amount of immunosuppressants so that the body’s T cells can proliferate and hopefully overtake the rapidly producing B cells. The risk of taking this course of action is rejection of the transplant. Luckily, Rafi was already being weaned, aka tapered, off the cyclosporine.

So, Rafi tested positive with a viral load of 4000 and a retest showed the viral load had grown to 9400. Boy did we get worried. With her viral load increasing and considering what her presenting symptoms from the week earlier had been, add to that an inflamed lymph node, and we were quite on edge. We tested her blood for a third time to see if EBV load had increased. I really don’t want to go into detail about what could have happened. Right now, it just isn’t worth it. Needless to say, and thankfully, we found out this afternoon that Rafi tested negative for EBV.

Once again Rafi has surprised people. I am not sure anyone expected this. Maybe a smaller number, but not a total abatement. She did have a good T cell profile at her 100 day milestone and we were already tapering her off the CSA. My first question was, can there be a false negative in this case. I was told the test is usually very accurate, and that confirmed my research. So, to be sure, we are going to retest her blood again for EBV on Friday. Let’s keep our fingers crossed.

Rafi is still aspirating thin liquids, as our barium swallow test demonstrated. However, when the liquid is thickened to a nectar or a honey consistency there is no aspiration and this shows us that the dilatation was successful. WE can only assume at this point that the traumatic intubation from the dilatation, caused enough inflammation to interfere with her ability to swallow. Of course, the virus may have also played a role in this. This issue should resolve itself in about a month.

Tomorrow we are going back to the hospital to have an ultrasound. We still have to keep an eye on her pericardial effusion. Other than that, Rafi is doing quite well. She has really bounced back and is once again laughing and romping around the apartment. Toilet training is going quite well to top it all off. In fact, Rafi will yell at Jackie, “I am NOT DONE” when Jackie tries to get her off her toilet. It’s very hard not to laugh, but of course, yelling at mommy is bad behavior. Rafi also hasn’t vomited in days. We are also not giving her the tube feeds at night and instead are venting her gtube. We will have to reverse that soon, but for now I think giving her esophagus a break from vomiting is a good thing.

Now that I am breathing a little easier, I hope to be able to sleep well tonight. Both Jackie and I are exhausted and need some sleep. Speaking of that, and I’ll leave you with this, Rafi is no longer taking ambien at night and she is sleeping better than ever.

Rafi has not been intubated up to now. After some time, we were able to reduce the amount of oxygen she was getting down to about 35%, or 6 liters. On that amount of oxygen, Rafi has been able to keep her saturation rate up at 100%. She is slowly getting better but we are still not out of the woods. Without supportive oxygen, Rafi’s oxygen saturation rate in her blood will go down to low levels. Right now she is wearing a mask as she sleeps. It is difficult to keep that mask on as she she moves quite a lot as she sleeps. That means, neither Jackie nor I get much sleep, or any at all. We tried to hook up a system where humidified oxygen gets blown at her from a couple of different directions. As it turns out, that is just not good enough to keep her levels up. Hopefully, in the next day or two Rafi will better be able to tolerate room air.

We keep adding specialists to our list of doctors. The latest is a cardiologist. So far, nobody is too worried about the fluid around her heart. It is not impairing her heart function, but we need to keep an eye on it. We are also looking into the idea that the dilation did nothing. When Rafi takes a drink, she coughs. We hope that the general inflammation is causing the issue. We’ll find out in the coming days. We really can’t let her drink and this is proving to be difficult. She loves her water and ice and all she does is ask to drink. Unfortunately, we can’t let her drink right now. I don’t know how to keep telling a two and a half year old that she can’t drink any water.

As I type there is a portable x-ray machine being rolled into the room. Needless to say, it’s been a fun few days. Change that, a fun few hours. We are in the woods, pretty deep right now. Since Friday, Rafi’s fevers have been getting higher, reaching a peak of 104.7. The doctors here are testing for everything under the sun to see what is going on. We have taken enough blood from her for cultures tp see if anything bacterial is going on, a nasal swab for viruses, a ct scan of her lungs and sinuses for signs of infection. Needless to say, once again, Rafi has been through the ringer.

It gets worse. This morning, Rafi’s breathing became very labored. She was working very hard for breath and her oxygen saturation rates started falling. The gave her a few nebulizer treatments (like using afrin) and a dose of steroids to reduce inflammation. On top of all this, we learned that Rafi has a pericardial effusion, or fluid around the heart. Rafi was within minutes of being intubated once again and transferred to the pediatric intensive care unit. Everything is about trends. Was she holding a oxygen saturation rate of 95% on 100% oxygen for a long time or was she struggling to stay that high? She was trending down. Her heart rate was through the roof, and her breathing very labored and rapid.

The BMT doctors believed that it was probably time for intubation. Pulmonologists were called, anesthesiologists were brought into the loop and the PICU was consulted. We were travelling down that road pretty quickly. Apparently, Jackie and I made a strong enough case to give the steroids a fighting chance at working before we just intubated. To my untrained eye, it seemed that Rafi started to trend upward. We have her on an oxygen mask and she is now receiving 60% oxygen and her oxygen saturation levels are in the upper 90s. A couple of hours ago, she was on 100% oxygen and struggling to stay at an oxygen saturation rate of 95%. I know it sounds encouraging and one might think that we are headed out of the woods. She is still getting a lot of support however. Room air is about 22% oxygen. Her heart rate is still very fast and she is still breathing hard and fighting for air at times. We are nowhere near out of trouble yet. At any time, Rafi may need to be intubated for an unknown period of time.

More later……………

Another day with more fevers. Tuesday is now the earliest we can be discharged because of the severity of Rafi’s fevers. She has spiked some pretty high fevers, but tylenol is keeping them at bay. Just as we think we are over the fevers, another one, slightly higher than the last, keep popping up. She has an obvious infection around her gtube site and another one that just sprung up just above her left hip. She has a few others as well, on the back of her neck, on her left hand, right foot and on an ear. On top of that, Rafi has been getting more blisters recently and we hope that trend will stop soon. I told Rafi before I left the hospital this evening, that her father’s day present to me was going to be a day free from fevers. Hopefully, she listens.

It is easy to get a little scared about the prospect of bouncing in and out of the hospital. There are those that get readmitted at day +165, +295, +81, etc. Our hope is that the antibiotics kick in, do their job and we can go home soon, maybe Tuesday.

We are pretty comfortable in saying that the dilatation has helped out. Rafi is not gagging as much and not vomiting as much as the last week. So we got that going for us. Hopefully, as the irritation subsides in her throat, Rafi will start eating again and the vomiting will cease.

I am too tired to write anymore this evening. I haven’t slept in what feels like weeks and that’s probably not far from the truth. Jackie has not slept much either so think some positive thoughts for the little one. We need Rafi to make some serious health strides here.

Until tomorrow……

Today was a long day that ended with Rafi as an inpatient back on the BMT floor, 5D. The plan is to be back home on Sunday, but the best laid plans……

Jackie and I woke up at four this morning (it’s now 10:30pm) and woke Rafi up at 4:45am so that we could get to the hospital by 5:30 for the esophageal dilatation at 7:30. For once, we were actually on time. We even had coffees in our hands. Rafi got her Versed and we headed into the OR. I’ll save you from all the gory details, but the intubation did not go as smoothly as the other three she has undergone, while the dilatation went very well. Her stricture did in fact narrow the opening of the esophagus once again, so our timing was perfect. This time, the interventional radiologist was able to dilate the opening to eight millimeters, versus six the first time. Rafi’s tongue took a bit of a beating during the intubation so there was some blood and when Rafi finally came out of the anesthesia she was not a happy camper. It took Rafi a long time to completely get out of the haze of anesthesia this go around. Before the procedure, Rafi had a low grade fever, that abated before the surgery. After however, she was “ping ponging” between the fever that would make us an inpatient here, 100.5, and a more normal temperature. Just as we were about to be discharged, Rafi spiked a 101.1 fever that escalated to 102.2. Three hours later, she was down to 98.7. Everyone involved felt it was diligent to admit Rafi and give her a course of IV antibiotics just in case. So, here we are again, in a different room, but back on 5D.

It’s frustrating to be back on 5D. We had made so many strides forward and now here we are again. I know it is not uncommon for this to happen, and this is certainly not our fault, but it really stinks nonetheless. As I sit here, listening to her breathing and occasionally coughing slightly (which provokes a cry of pain), I have a flood of memories coming back. I see, as clear as day, the wounds on her face and body she suffered after the chemotherapy. I see the expression on her face demonstrating how awful she felt for awhile. I remember sitting by her side, trying to keep her oxygen saturation rate up futily as we headed for eight days of intubation. So to be sure, I just did a measurement of her oxygen saturation rate because it sounded like getting in oxygen was tough. She was in the mid nineties on room air, which is just fine. At home, Jackie and I were worried about her saturation rate when she slept. Now I know that when she sounds like that, she is still doing alright. So I feel a little better. But I am tired and a little afraid to go to sleep. I have a tendency to sleep pretty hard at times and I am worried that I won’t hear her at an important time. Mind you I am sleeping a whopping five feet away from her. I am guessing that I am not going to get much sleep tonight anyway.

She did eat a popsicle this evening though. She took huge bites, for her, without gagging, so that’s great. Twenty four hours ago, she would have vomited after the first bite, so we are getting somewhere. She refused water and medicine though, but we I can live with that for the moment.

Until tomorrow…………

Lots of good news and a trying situation all at the same time. Rafi is now producing collagen VII and there is evidence of anchoring fibrils. Both of which are things that she did not have before the transplant. The anchoring fibrils are the latches that allow the dermis to adhere to the epidermis, and collagen VII is the primary protein responsible for their development. The hallmark of recessive dystrophic epidermolysis bullosa is the lack of collagen VII and the absence of anchoring fibrils. So now we have two pieces of scientific evidence that this procedure is working. That is very exciting. Two pieces of evidence that might one day suggest that she no longer has this horrible genetic disorder. That is very exciting.

The past 118 days have been trying at best and stressful most of the time. The thing about transplant is that the risks don’t end for a long time. I can now look at Rafi and seriously question if some of the things she is experiencing is due to chronic graft versus host disease. This is a potential complication that can manifest itself at any time and the severity runs the gamut of mild to fatal. It can be treated with steroids, which have many side effects and unintended consequences, and it is best to catch it early. Steroids are immunosuppressants, so taking them make the person taking them much more vulnerable to infections. She is not suffering from chronic graft versus host disease presently, but we have to be vigilant about checking for it so that we could start treating it in it’s early stages and thus limit the negative consequences.

Unfortunately, Rafi continues to vomit regularly and has recently stopped eating altogether. She is constantly telling us she is hungry but when the food arrives, no dice. She has had one esophageal dilatation, and tomorrow she is having her second one. This came about rather quickly. We really didn’t have time to celebrate the incredible news. When she drinks water, she coughs. She can no longer tolerate her own secretions. She coughs throughout the night and will vomit with little to no warning. This is scary because of the aspiration risk and the chance that this will send us right back into the pediatric intensive care unit where Rafi was intubated for eight days. We originally had the dilatation scheduled for the 29th of June, but considering her symptoms of a stricture seemed to escalate significantly during the past couple of days, we felt it imperative to push the envelope and get Rafi into the operating room as quickly as possible. So tomorrow at 5:30am, we will arrive at the hospital for a 7:30am procedure. Rafi will be intubated and the interventional radiologist will thread a guide wire through Rafi’s gtube, up through the stomach into her esophagus and then inflate a balloon to widen her esophagus. We knew that she would have to have this done more than once, but having her intubated again is just downright scary. However, not sleeping for fear of not getting to her in time when she is about to vomit is wearing thin.

That’s it for now. I have to be up at 3:30am to get ready to go the hospital. I will write another update tomorrow after the procedure.

It seems as if Rafi has grown up so much in a week. Jackie started toilet training Rafi and she is really getting it. I’ll save you the gory details. Suffice it to say that Rafi is taking to her potty like a fish in water. After two and a half long years, we are finally able to have Rafi go to sleep on her own. We no longer have to pat her back so she can fall asleep. All it took was a bribe. We told her that when she was a big girl and went to sleep on her own, we would get her a tricycle. Well, it took about 2 days for Rafi to fall asleep and earn that tricycle. See below for her first tricycle ride along the Mississippi river.

There is really big news that I am hesitant to discuss but it is impossible not to mention. Dr. Tolar, after looking at Rafi’s electron microscopy of her 100 day biopsy, emailed us with some pretty exciting news. He thinks he sees evidence of an anchoring fibril. If confirmed, it would be the first scientific evidence of this procedure working on Rafi. Like all recessive dystrophic epidermolysis bullosa kids, Rafi lacks the anchoring fibrils that holds the dermis to the epidermis. Well, hopefully, this is the first evidence of her developing some. That would mean that she is producing colllagen VII. Words can’t express how exciting this is for us. Anchoring fibril growth at day 100?

Can you just see the smile?

Into a Bigger World

Taking a break from the tricycle and walking down the Mississippi

I didn’t realize it had been so long since my last post. So here is all the news.

As of yesterday, we only have to go to clinic once a week for blood tests and we see doctors every other week. We can stop one of the antibiotics she is on. Plus, we got the go ahead to wean Rafi off her immunosuppressant, aka gengraf. It takes 8 weeks to stop taking this drug. Once we are off this drug, we should be able to get her off her last blood pressure medicine. In 5 days, we finish the course of one other antibiotic as well. Slowly but surely, we are getting rid of the drugs.

After 100 days, Rafi is still 100% engrafted. That means the cells floating around in her blood are of donor origin. This is great news, as the odds of her rejecting the transplant are now pretty low. Hence the reason we can wean off the gengraf. Furthermore, the biopsy results showed that 30% of the cells in the skin they took are of donor origin. That is also great news. What i can say is that area of her body used to be a trouble area. Now the skin looks great in that area. Her feet and calves were a source of many wounds and blisters before the transplant. Her calves have been amazing for awhile now, free of wounds and blisters for some time. Her right foot has had a blister, that we lanced, and two days later the foot looked absolutely normal, as if there was no blister. This never happened before transplant. Another example is on May 15th, Rafi had a pretty terrible wound right at the crux of her foot and calf. On May 17th, it was significantly better. By the 23rd, it was healed. Before the transplant, this wound would have taken a month to heal. Her hands also look great. She’s getting better, slowly but surely.

We are still working on the eating, and on increasing the amount of formula she gets on a daily basis. So far, we’ve been giving her feeds overnight, and we are increasing the volume methodically. We do this to make sure her stomach can handle to volume and the sugar. So far so good. We have a way to go but I hope to be able to increase our volume adjustments significantly within the next week.

Rafi’s spirits have never been better. She is talking up a storm, loves to paint, play zingo (bailey I think I hate you for that one) and not listen. She’s turning out to be a very smart little girl, so I am quite interested to see her in twenty years. I better start saving for college, she’s going to an Ivy league.

Triple digits baby. Day 100 is a great milestone to hit. We are on the upswing as well, which is nice. Her infections seem to be going away thankfully. She is on enough antibiotics so one would have to assume this would be the case. We are getting more formula into her on a daily basis but we have a way to go. I think we are going to have to rely on TPN, aka IV nutrition, for a long time. In order to be free of the TPN we need to get her 10x the nutrition we are getting into her now. It’s a daunting task but we have time on our side. We are just getting Rafi’s GI tract working now, and while we are taking slow steps to increase her intake, I have a feeling that all of a sudden we will be able to double or triple intake in a short period of time. The real big step will be getting her to eat solid, or at least soft, foods. She just doesn’t want to eat many things, and the old standbys just don’t exist anymore. We’ll figure out something soon enough.

We tried not giving Rafi Ambien last night and that backfired. We even tried hydroxyzine but that did absolutely nothing. This kid is never going to sleep. It’s funny because she will not sleep well at night and then, out of nowhere, sleep until 9am. I’d give anything for her to sleep through the night and wake up at six in the morning.

During the day, Rafi is having a good time. That is when she is not being a typical 2 and a half year old. Fact is she is pretty amazing. I know I say that a lot but it is true. She’s smart, funny and loving, and she gets more so every day. She can even spell her name now, count to ten in spanish and hold conversations. She is opinionated, and that’s not really a surprise as she is Jackie and my child.

We are seeing changes in her skin. She is still blistering, but it seems to be less. She can still tear her skin, and she does. But her new skin is softer and the coloration is normal. The milia on her face is clearing up and her hair is growing in nicely. She does get some rashes on her body, but we think that is a combination of all the drugs she is on and a little heat rash. Then it clears up.

We are going to try and figure out some way to celebrate with Rafi tomorrow, that one more milestone has been hit. That we are one day closer to having a healthy child. That she is a great kid who is incredibly tough and one hell of a fighter.

Today was a difficult day. We learned that patient #9, McKenzie, is not making new healthy cells. She has had two separate infusions of cord blood, from two different donors, and neither one has allowed her to create new healthy cells that are free of EB. In short, the transplants didn’t work. Our hearts go out to Missy and Greg, and of course to Kenzi. Both Jackie and I wish that there was something we could do, something that we could say that would ease the pain and sadness they are experiencing right now.

Yesterday, Rafi went back into the hospital to have her 100 day biopsies. We are hopeful that the five pieces of her skin they took from her will show increased levels of collagen VII production. After she was put under we took off all her bandages, the biopsies were collected, photographs were taken, and we wrapped her back up. We were home by the early afternoon. They also did the blister test, but we will repeat that because after 60 minutes, and no blister formation, we turned off the machine. It took me 47 minutes to form a blister, and it took Jackie 36 minutes, so we have reason to doubt that the reading on Rafi was accurate.

Rafi continues to have skin infections, but her wounds heal very quickly. There are some that are proving more difficult and her white blood count is still very high, so in order to be safe, we started a course of linezolid. It’s a very strong antibiotic that should help with her MRSA infections. Of course, insurance still has yet to approve the use of the drug, so if they deny it, and we lose the appeals, it will cost us $1,000. We didn’t want to wait for the insurance company to come to a decision and risk the MRSA becoming potentially very dangerous for Rafi. There are worse ways to spend a thousand dollars.

Tonight, we started feeding Rafi as she sleeps. This is the first time we have done this in over three months. We need to get more calories into her and while we wait for insurance to decide on approving feeding therapy, not to mention physical and occupational therapies as well, we moved forward with the formula feeds at night. These decisions seem like no brainers to me, but I am not an insurance company that is beholden to it’s shareholders. Rafi already gets IV feeds over night, but that only supplies approximately 63% of her daily caloric needs. We didn’t want to start this until we had her vomiting under control to a degree. We’ve been getting about two ounces of formula in a day in addition to some smoothie and puffed corn. So we are headed in the right direction, and this seems to have helped her vomiting some. So we hope by adding more, this will not only increase her caloric intake, increase her wound healing and growing, it will also decrease the vomiting. Only time will tell.

With all that being said, Rafi is in very good spirits. She is cruising around, talking up a storm and telling us that she’ll “be right back”. By the way, her new favorite story is Snow White, so all I can say to that is the more things change the more they stay the same.

I will get to uploading new pictures and videos, I promise.

100 day biopsies coming up next week. 100 days is a big milestone to hit. If we had decided to not live here for a year, we would be planning our trip home now. It’s hard to imagine that after everything Rafi has been through that we would have the option of going back to NY. I am glad that we planned on staying here for the full year for many reasons. There are still several things that we need to work on with Rafi. She is still not eating solid food, although we are starting to get some formula into her. Slowly but surely, we will increase the amount of formula she ingests until we can stop relying on the TPN. It will take some time but we’ll get there.

We just cut her one of her blood pressure medications in half, so that’s nice. It’s our hope that in the next few weeks we will be able to discontinue the cyclosporine as well. That will lead to us cutting out two other blood pressure medications. We can’t wait. The less medication that goes into her, the better her stomach will become. However, speaking of drugs, we switched from prilosec to protonix and we think this has really helped her reflux. She has been vomiting less and we can only attribute that to the change in the drug. Of course, to this point, insurance has denied the protonix even though we have documented evidence that when she uses another drug the PH level in her stomach changes. The funnies t part about it, is that the protonix is actually cheaper on a monthly basis than the prevacid they used to support.

Today we had another esophagram to see if the dilatation was successful. We saw that there is an area of her esophagus that is more narrow than the rest and it is pretty high up but a small stricture. The nice thing is that it hasn’t changed since after the dilatation. So while it could be wider, we are going to wait on getting another dilatation until a later date.

Rafi is just getting over several MRSA skin infections now. They drove her white blood count up to 24, which is pretty high. The nice thing is that she is healing very quickly from her wounds. She is probably healing faster than I do at this point. We have taken pictures of her right foot at each bath and bandage change and the healing progression has been quite impressive. When this particular wound appeared on the 15th of this month, it was a pretty nasty wound. Six days later, I would say it is close to 80% healed. Prior to the transplant, it would take almost a month to get to this point.

Her hair is growing back. I can’t wait to see her with hair again. It looks like it is coming in black as night. It will suit her face nicely. Other than that, Rafi has been in very good spirits. She is enjoying her time with her new nurses. She actually cried the other day when one of her nurses left for the day. I just wish we could get her around some other kids. It would be very nice for her to have some contact with kids her own age. It’s been a long time since she has been around other kids and it is going to be awhile before she has any real friends again. I hope it doesn’t affect her development in a negative way. We do have to get her strength up though, because if we were to send her to preschool now, she would get trampled over. These are not my words. Her physical therapist said it. So at least we will have some time to get her close to age appropriate before we send her into the jungle.

Time to sleep and get some rest.

We’ve certainly had some close calls the past couple of days. I was certain that we were going to be back on the halls on 5D yesterday. Rafi’s fever was climbing up a little, although we couldn’t trust the thermometers we had. Yesterday, we had two thermometers, one you can put on the temple and we had a cheap under the arm thermometer. The temperatures were all over the place. With the temple one, it one place it would rad 98.3 and in another place it would read 101.6. We knew she didn’t have that kind of fever so we pulled out the axillary thermometer. That gave us different readings within a 15 minute time period. WE had a clinic appointment anyway so we figured we would just get a reading then. Turns out she had a low grade fever that kept declining as the appointment went on. Phew.

We are dealing with some skin infections at this point and we think this has resulted in her elevated white counts. Yesterday, her WBC was 24. That’s pretty high and is a sign of infection. We are goign to get that checked again this morning at clinic. The wounds are getting better and we hope this leads to a lower WBC. I have a theory about all of this. Rafi’s cyclosporine (CSA) level was over 500 the other day, and the goal is to be between 200 – 400. CSA is an immunosuppressant so I wouldn’t be surprised if the increased level was partly responsible for Rafi’s inability to clear the skin infections and thus the low grade fever. I am sure there are people who will argue both sides of this theory, but now that the CSA level is back down in the mid 200s she seems to be fighting off the infections. I am not one to believe too much in coincidences.

In the next couple of days Rafi starts her feeding, occupational and physical therapies. We are looking forward to the feeding therapy the most. We were going to start feeding therapy last year in NY but it, of course, got denied by insurance, and instead of appeal the decision we figured we would wait until after the transplant. Three days after the insurance company denied the feeding therapy, they approved the transplant.

Rafi’s language development continues to amaze us. She is a very funny and loving little girl. If Jackie or I stub a toe or make a noise, Rafi comes over and says “Mommy (or Daddy as the case may be), you ok?” She is laughing more, speaking in complete sentences, ignoring us and just causing trouble the way a two and a half year old should.

Life has been a little busy in the Kopelan house this past week. We haven’t had a nurse for the past couple of days and Rafi has become a little more demanding of our time. This is a good thing of course. She constantly wants “to play” and run around. This would be all the more great if this all lead to Rafi sleeping well at night. She doesn’t. We have been having some vomiting issues before bed and during the night. Rafi is a prime example of Pavlov’s theories. Due to the vomiting, Rafi has figured out that she doesn’t want to go to sleep. I hear, “Daddy, I’m done sleeping” a little often for my taste. Anyone have something better than Ambien? Yes, we’ve tried melatonin, with no success.

Our big hope is when Rafi starts eating more that the vomiting will stop. She is constantly saying she is hungry but then won’t eat when food is in front of her. We’ve been trying to get some formula into her, through the gtube, and I think we are going to need to be a little more aggressive in that venture. She starts feeding therapy next week so we are hopeful that will help.

As to blistering and skin tears, Rafi is healing more quickly than before the transplant. I would say she is getting less blisters on a daily basis as well. She is, however, still getting blisters, and her wounds still get infected, as we are battling some small infections now.

Her hair on her head is starting to grow back in. The cyclosporine has really kicked that endeavor into high gear. She has hair everywhere, on her shoulders, on her back, and on her tushy. The most pronounced areas are her eyebrows and her eyelashes. Before this hair growth is over, I will get my wish to have played with a chimpanzee for a period of time.

I will post more frequently going forward.

It was quite nice to spend Mother’s Day home as a family. Yes, we are still home and Rafi continues to do very well. I apologize for not updating sooner, but as it turns out, I am getting older by the second and need more sleep than I ever have before. Getting older sure does stink. Anyway, over the weekend we were given a two day pass away from clinic. It was almost like old times when we didn’t have any doctor appointments. We have an appointment today to check her cyclosporine levels and general blood work. Today we also started up our daily nursing so Rafi will have to get used to having someone else in the house with whom to play. So far, by the sound of it, Rafi does not appreciate it. We always said she needs to have her coffee in the morning before she becomes a nice child.

We no longer vent her gtube all day and all night. This is a welcomed change. Her vomiting episodes have decreased, now just two times a day, going to sleep and waking up. I want to get her off the antiemetic, zofran, because I don’t think it does any good at all. We give it at eight o’clock and she throws up at nine. I also think it is good to just get her off as many medications as possible. Speaking of that, we no longer use hydroxyzine or neurontin, and we very rarely use any pain medication, including tylenol. Although we still have supplies of all of them in case.

Now we are trying to get her to eat some food. This has been difficult at best. She needs to start augmenting the TPN with her own caloric intake. We are starting to put some formula in her stomach every couple of hours and attempting to get her to eat regular food as well. She will say “I’m hungy” and then not want to eat what we put in front of her. Right now the only thing she will eat is the smoothie I make for her. I blend up strawberries, blueberries, bananas and orange juice and add in some baby oatmeal for some additional calories. She doesn’t eat much but it is a start. She did eat a quarter of a popsicle yesterday though, so some headway has been made.

I know everyone wants to know if she is blistering less or is seem to be getting better in any real way. Jackie and I just commented yesterday how we seem to have to lance fewer blisters. so that’s something. She does still tear herself up a little, so I can’t say her skin is any more durable than before, but she seems to heal more quickly. This is not scientific in the slightest, but this is what it seems to be now.

I promise to post a video or two tonight or tomorrow. I really have to catch up on some work and some bills today. This will be a week long process if that is any indication at all. Between clinic visits and bath and bandage changes, I am going to be a busy boy. but I will post one or two.

Until tomorrow…………

After 85 days in the hospital we left and came home. When we got into the apartment, Rafi became a completely different child. In the hospital, she wouldn’t walk without holding a hand. This afternoon everything was “by myself”. She walked all over the place, threw balls around and dared us to get the ball before she could. She was happy to be home. She even started to eat some food, something she would not do in the hospital. But the greatest thing was when we walked into the door, put Rafi down on the ground and she uttered one of those lines only a two and a half year old would say. Upon entering the apartment, Rafi said, “Mommy, where’s my broom? You need to clean”.

She has a point. Our place is quite messy to say the least. Our dining room table is a repository for mail, papers from the hospital, baseball hats, batteries, etc. The kitchen island is littered with syringes and medicine bottles, directions and cards from people who sent us food and gifts. But it could be worse, we could still be in the hospital.

I have a couple of very funny videos I will post tomorrow or over the weekend. One of them is of Rafi strumming her new Dora guitar that my parents sent to her. She is literally strumming this toy guitar and bopping her head to and fro. It’s great.

We also got some test results back today from her sixty day biopsies. Rafi is fully engrafted. In other words, 100% of the cells floating around her body are from the donor. This is the same result as we got from the day thirty biopsies but it’s nice to know the number has not decreased. Also, from the small area of her left thigh where they biopsied, 11% of the cells in the skin are donor cells. We are still waiting to see if she is producing more collagen 7 but we should know that in the next week. We are told not to expect any increase in collagen 7 production until day 100 but we are hopeful.

Now the key is to stay out of the hospital. Keep your fingers crossed, we like it here.

Until tomorrow……

As you may know, we try to keep Rafi abreast of all her treatment options as we go through this journey. So as we get ready to leave the hospital, we have had discussions with Rafi about it. After several heated conversations, Rafi has decided that she wants to stay in the hospital for a little longer. We know this because she decided to spike a fever today of 101.1. That earns us a two day ticket on 5D. Clearly she is adamant about staying put where there is room service and people come to see her all the time and she is the center of attention. Tomorrow or Tuesday we are going to have another esophagram where Rafi will drink some more barium and have a lot of x-rays to see if the dilatation was successful or not. She has been throwing up a lot of mucus and coughing a little when she drinks so everyone feels this is the right thing to do.

Rafi has become very attached to the idea of a nurse. Yesterday when we were going to wash her legs and put on new bandages, Rafi said:

Rafi: “OOO, bath and bandage change, who is going to help us?”
Jackie: ” No one pookie, daddy and I are going to do it.”
Rafi: ” No, call the nurse”.
Brett: “It’s alright Raf we can do it, it’s easy today”.
Rafi: “No, call the nurse, we need help”.

Due to the fact the our nurse yesterday was new to us and was unfamiliar with Rafi’s case we decided to move ahead without help and we washed and changed her bandages. Rafi’s big thing now is to ask us to call the nurse all the time. After she throws up, she asks us to call the nurse so they can see the product of her work. She just cracks me up.

Anyway, Rafi is now producing more mucus and throwing up a little more again. Plus, she spiked the fever. She had an xray today to look at her lungs and from an untrained eye, mine, her lungs don’t look any worse than before and in some ways they look better. We hope this is just a little bump in the road on the way to her full recovery from the pneumonia. So, really what’s a few more days in 5D?

Until tomorrow…………

Not really much to say today. We are planning on being discharged on Monday or Tuesday latest. We really can’t wait to be at home.

I posted a new video of Rafi 66 days after transplant. Just go to the Pictures/Video Page and you can’t miss it.

Until tomorrow……….

Rafi had her esophageal dilatation yesterday and it went very well. I was in the operating room the entire time with her and while we may have to have it done again in a couple of weeks you really couldn’t ask for a better outcome. She even had some Jamba Juice this afternoon and that made her happy. For the past week, Rafi has been asking for water and ice and she keeps saying “I’m hungy” (that’s the way she pronounces it), so we can’t wait to feed her starting tomorrow. We keep telling her that the hospital is out of popsicles but they will go shopping soon, and that the kitchen is closed. Finally, we explained to her what was happening and even though she is only two and a half, it seemed like she understood. Jackie and I told her that we were going to have a procedure done that would stop her from throwing up and we would be able to let her eat when it was over. She accepted it because she is always telling us, “I am scared of throwing up”.

The doctors were worried that the stricture was atypical because of how long it was. However, the interventional radiologist felt it was smaller than others thought and the doctors in Cincinnati agreed that she should undergo the procedure. As it turned out, the stricture wasn’t long and in one area her esophagus was narrowed down to 2 millimeters and they dilated it to 7 millimeters. The goal now is to get her to eat some soft foods starting tomorrow because that might help further dilate it or at least keep it from closing up again. She didn’t have any retching last night and, to me, that is an encouraging sign. I am much less worried that she will aspirate again and end up sending us back to the ICU.

It’s hard to believe that we have been in the hospital for 77 out of 80 days. That is a long time and we are very much looking forward to coming back to our Minneapolis apartment. Now I am pushing the doctors to discharge us from the hospital as fast as possible. Hopefully, we will be back in our apartment, as a family, by Wednesday the latest. Jackie’s father and sister are coming into town so it would be nice for everyone to be out our place instead of in the hospital on top of each other.

Rafi has started to blister again in some areas. We knew that this was going to happen and that they healing process is a long one, so we are discouraged at all. The fact is she still has very fragile skin. The latest bskin engraftment rates show an 11% chimerism. In other words, the results from area in which they biopsied showed that 11% of the cells in the skin were donor cells. Thirty days prior, and in a different biopsy site, there was a 14% chimerism. So we can say this is working, but it is going to take awhile adn we expected that finding. However, the new skin she is getting is softer and more “normal” looking so that keeps us going.

Her attitude is amazing and she doesn’t cease to amaze us. She now understands that we are from New York City but we are currently living in Minneapolis. It is so funny to hear her say Minneapolis. Not quite as funny as when she says oxycodone but it makes us laugh nonetheless. One of the big issues when we get home is we are going to have to wean her off her television habit. I swear it could be worse than weaning her off some of the drugs she’s gotten. When she is uncomfortable now, she tells us to “push the button and call the nurse”. At bath and bandage change time, she asks Jackie and I, “who is going to help us?” It’s really funny. Anyway, Madagascar 2 is her new favorite movie and at least we can all laugh together at Moto Moto. She still quotes Kung Fu Panda and gets very excited when we put the movie on. There are some nurses who come into the room to see her do this and they laugh. Rafi anticipates the lines and says them a good 30 seconds before they are spoken. She is definitely the person you hate in a movie theater.

Anyway, another day and another $15 to $20,000 to the insurance company. We really can’t wait to go home.

Until tomorrow……

Tomorrow at 11:30, or so it is scheduled, Rafi will go into the operating room, yet again, to undergo an esophageal dilatation. An interventional radiologist will thread a guide wire through Rafi’s G-tube, through her stomach and into her esophagus and then inflate a balloon at the narrowing passage to stretch that part of the upper GI tract. The hope is that this procedure will widen the the esophagus enough to allow Rafi to handle her own secretions, aka swallow her spit and mucus, and eat food and drink liquids. Rafi has been retching a lot lately and the fear is that she will, once again, aspirate some of this and develop another pneumonia. This procedure, if successful, will hopefully stop the retching as well. It was a bit of a fight to get us to this procedure as there are risks involved. However, from a risk/reward basis goes, it really makes sense to have the procedure. The two barium swallow tests showed a a narrowing of the esophagus so this is necessary for Rafi to undergo, whether now or later.

Other than that, Rafi is doing incredibly well. There is a lot to be excited about and we understood and expected there to be complications or hurdles in this journey. She’s now lost the rest of her hair, so she no longer looks like a little Jack Nicholson. She looks very cute bald. I’ve taken more videos of her, and her bald head, and will post some new ones in the coming days. It is really about time I did that. I will update more thoroughly tomorrow after the procedure, being that it is now midnight and we are sure to have a long day tomorrow.

Until tomorrow……….

Today has been a long day. First thing this morning Rafi had a CT scan to see how her lungs are progressing. Then we learned that Rafi has an issue in her esophagus but because they only did a modified barium swallow test, we would need to get a full one to accurately diagnose the issue. So this afternoon, Rafi traveled back down to the pediatric imaging department to have more x-rays done as she drinks some white chalky liquid flavored to taste something like apple. Without fear and without complaint Rafi sat still for the CT scan and she drank the pepto like substance in different positions as they blasted her with x-rays.

The results of all these tests seem to indicate that the pneumonia was caused by her aspirating something. Being that we weren’t feeding her anything, it had to be her saliva and mucus. So now, our doctors are in conversation with the GI doctors about the risks of undergoing an esophageal dilatation at her early stage of recovery from transplantation. I see it a bit differently right now. If we agree that it was likely an aspiration that sent her to the ICU, then we are still at risk of her aspirating again. That is the known. The rest is unknown. The decision should have been made already to move ahead. Every hour the doctors discuss strategies or risk versus reward contingencies, Rafi is at risk of aspiration and potentially being intubated again. In fact, our fellow postulates that Rafi is actually mildly, yet consistently, aspirating. I will not wait around another half a day as doctors debate about hypotheticals. In the words of a Ghostbuster, “let’s show this bitch how we do things downtown.” I am a New Yorker, which means I believe that time is of the essence, and this needs to be done sooner than later. They will have to see things my way, because I know they are not going to like me mad.

I would write more but I am just exhausted. Until tomorrow……..

Another day, another procedure on the horizon. I am starting to feel like Al Pacino in the Godfather saga. Just as we seem to be moving forward towards leaving the hospital something else pops up. After the modified barium swallow test, results seem to indicate that Rafi has some esophageal strictures. This furthers the diagnostic notion of the pneumonia being caused by aspiration. Therefore, Rafi will be required to go back into the operating room and have an esophageal dilatation. While this isn’t the worst thing in the world, and many, if not most, EB kids will eventually have one done, and complications are rare, we feel terrible for our little girl. She has been through so much the past two and a half months that we just want the procedures to be over. We want her to be able to run around and enjoy her time as a child.

Other than that, Rafi is doing very well. She has not required an oxygen mask since yesterday morning. She’s been happy, smiling and laughing more than ever. She has even been interacting with some of the nurses she has gotten to know and feel comfortable around. Overall, there is a lot to be happy about.

As we know more I’ll report it.

Until tomorrow….

Looks like somewhere along the line I added a day in the count from transplant. Oh well. It’s day +65 on April 25, 2010.

We’ve had a couple of really great days. Rafi continues to improve pretty dramatically. Today she did not wear an oxygen mask all day. Right now as she sleeps, she is not wearing one either. This is quite an accomplishment at her stage of recovery. Another huge accomplishment is that Rafi has been out of bed and walking around. Now understand when I say she was walking around, she walked for about 60 feet. However, keep this in perspective, considering she was medically paralyzed for 8 days and kept unconscious for 2 more, that’s a pretty far stroll. She also stood for about 15 minutes with one of her physical therapists. The therapist was ecstatic.

Yesterday, the ICU attending came into our 5D room for a social visit. She was so happy to see Rafi sitting up and talking profusely. She said it “warmed her heart to see this”. She couldn’t believe how well Rafi was sitting up and talking. The doctor didn’t even put on a stethoscope. The fact that Dr. Parvin came for a social call is one of the things that sets this hospital apart from others. We really appreciated it that she came for a visit to see Rafi. Rafi, of course, didn’t care in the slightest.

Tomorrow Rafi will have a Barium Swallow test. The doctors here think it is still a possibility that the pneumonia was caused by an aspiration. Plus, after intubation for more than 48 hours, the body may develop a problem with swallowing that may lead to a tendency to aspirate. So, tomorrow we will get to see if Rafi has any issues with her esophagus or her throat. I have discussed the test with Rafi so she knows what to expect. She tells me she is scared, but after I explain it’s just drinking a good tasting drink and having some pictures taken of her throat, she seems to not mind going through it. There is a caveat however, I have to hold her hand.

Rafi is still having some withdrawal symptoms but they are lessening in severity and in frequency. Going to sleep still requires both dilaudid and versed, but it could be a lot worse. Before she went to sleep tonight, we called Jackie at home and while on the phone Rafi asked her to remember to bring her pink crocs back. Right before her second drug infusion, Rafi looked at me and said, “Mommy loves me”.

I do want to mention how good her skin looks. There is definitely a difference in the tone and quality of her skin. In some places it is quite dramatic. I can’t comment on the idea if she blisters more or not because her activity level is no where near where it was or where it will be, so to comment would be fruitless. What I can say is that her skin is soft and the coloration is completely normal. It’s truly amazing. She has completely healed from all the wounds she suffered from the chemotherapy and early transplant days. Looking back on those pictures now and comparing them to the current day is quite inspiring.

Until tomorrow…….

Yesterday we were able to bring Rafi back to her old room on 5D. The room is easily four times larger than our ICU room, so it’s nice to have the breathing space (pun intended). It’s nice to be back. The people here on 5D were very worried about Rafi and are happy to have her back. Of course, Rafi is back to her normal self, meaning she ignores everyone unless Jackie and I leave the room. When we leave though, she is a chatty little girl. Clearly I am going to have large telephone bills when she is a teenager.

Rafi is still wearing her oxygen mask to help keep her oxygenation up. Today, we started taking it off for periods of time to see how she does on just room air. Generally she is able to keep her saturation rate to the low to mid 90s, percent wise. hen after a little time has passed, she’ll drop down to the mid 80s, percent wise and we put the mask back on. Jackie and I are both a little competitive so we are really looking for her to be 98% or above before we feel totally comfortable. As Jackie likes to say, she excels at everything and she did get a 9.9 on the APGAR so we’ve decided that Rafi is a high 90s kind of girl. I think by the end of the day tomorrow, she should be able to make it most of the day or night without the mask.

Withdrawal is an ugly experience. She doesn’t suffer it too badly but when she does we are liberal and free with the drugs. However, we use them judiciously. To give you an idea she is allowed to get both versed and dilaudid on an hourly basis. We’ll go for five hours without giving her anything. Other than the occasional shakes, she suffers from the withdrawal when she is tired. Tonight when I asked her nurse to give her the dilaudid and the versed, Rafi said, “yeah, that will make me feel better.” Pretty soon, I can hear Rafi saying to the nurse “If you don’t give it to me, I’ll get another nurse who will.”

It looks like we are going to be here in the hospital for another week or two. They want to be absolutely sure she is completely healthy before she leaves this floor again. They want Rafi to not require the oxygen mask for a few days and to be free of the antibiotics before a discharge is possible. Jackie and I have no problem with the plan and are in no hurry to push the envelope. In a couple of more days, we are going to have a follow up CT scan to see what her lungs look like at that point. She’s been afebrile now for about three days and her white blood count has decreased. All good signs.

In short, Rafi has progressed very nicely. In actuality, she has gotten better far faster than anyone expected. As we now know, people here were incredibly worried about Rafi, and they are amazed by how fast she has recovered. Earlier tonight, I let Rafi fall asleep on me as we sat in the lazy boy chair. After about two hours and our night nurse, Jackie, poking around, I felt it was time to put Rafi into her bed. Nurse Jackie asked me if I didn’t mind holding her for such long periods given what we just went through. Truth is, I don’t mind at all. In fact, I like it a lot. It’s really good to have my little girl back. For awhile there, I wasn’t sure that was going to happen. But once again, she beats expectations. What an amazing little girl.

Please keep McKenzie Gray in your thoughts and send some positive vibes her way. Kenzi is patient #9 here, and she is going to need another cord blood transplant in a couple of days. As of yet, she hasn’t started creating any white blood cells. We are hopeful that this is her complication on her journey. She is a beautiful nine year old girl who was adopted by her mother and father, Missy and Greg.

Until tomorrow………………

A day without intubation is a good day. Of course, Rafi had to wear an oxygen mask because she still requires some assistance getting enough oxygen flowing through her system. While she doesn’t love the mask, she knows it is much better than having a tube down her throat. Because she still needs a little help, we are still in the ICU, but we should be able to go back to our old room on 5D tomorrow.

Rafi has had a couple of rough days, so I would like to do something for her when she goes back to 5D. I haven’t figured out just what yet, but I’ll get there. She’s had tubes stuck down her nose to suction out her lungs and she just hates that with a passion; at times she’s had trouble breathing to the point where she has worn herself out trying to get enough air into her lungs; she’s tired herself out trying to sit up and hold her head up by herself;and, at other times, she just has not been able to get comfortable. As each hour goes by, she gets a little stronger, and a little more able. Right now, as I sit by her bedside and type this, she is not wheezing, or auto-peeping (trying to expand her lungs a little more by forcing pressure down there) and she is breathing more easily, so that is great. She also has been able to cough up her secretions so that cancels out the need for suctioning. Therefore, things are definitely looking up for the little one.

We hope now that her elevated white blood count will continue to drop, since it’s been very high. We also hope that she continues to be afebrile (without fever). These trends indicate that the infection is clearing up and should lead to a happier little Rafi in the days to come. The real big issue we are having now is withdrawal. After 8 days of intubation and a lot of sedatives and pain killers, we have to slowly scale back her dosages. We may have been a little aggressive on this earlier and she was exhibiting some signs of withdrawal. Not anymore as we got Versed back on board. The goal is to use it sparingly, as we have, but there is no reason she should have to experience a difficult transition.

This past week and a half has been an emotional roller coaster filled with lack of sleep and doubt. It’s nice to see Rafi bouncing back so well. We are thankful for the care she has gotten here and for all the support we have been given from everyone that reads this site or the updates on Facebook. Hopefully, tomorrow I will pen the update from our old room on 5D. Then I hope we will be able to go back to our apartment as a family once again in about five days.

Until tomorrow………..

On the eighth day in the PICU, Rafi was extubated. She is now breathing on her own and nothing could make us happier. It’s been a very difficult week, but Rafi continually amazes us with her strength and desire to become healthy. Words can’t express how hard it is to see your child lying there with a tube in her mouth, unable to move, having a machine breathe for her. It’s just awful. Believe it or not, there were times when it was more difficult as we got closer to the time when the tube was going to come out. She became more lucid and was scared and upset at having a tube in her mouth. There were times Rafi looked at Jackie or me, and started to cry because she was scared. Watching tears come out of her eyes, her mouth open with no sound emanating was heartbreaking.

While that was incredibly difficult, seeing her without the tube in her mouth breathing on her own was amazing. It took awhile for her to be able to speak but when she did she told Jackie, “I love you mommy”. The point is, after her septic episode, after the pneumonia, Rafi took a huge step forward. Hopefully, she’ll be able to leave the ICU tomorrow and we’ll go back to our old room on 5D and eventually get to go home.

When I left the hospital this evening, Jackie was sitting in the foldout chair with Rafi in her lap. It was great to see. Rafi was resting on her and Jackie was very happy. Like Rafi, I can breathe a little easier this evening. Tomorrow Rafi will breathe a little easier, talk a little more, and gain a little more strength.

Until tomorrow……

We were supposed to have Rafi extubated yesterday. Yet, today she was scheduled to have her 60 day biopsies. After much discussion with the doctors and amongst ourselves, we all decided that it was best to leave Rafi intubated until today. She was going to have to be sedated for the biopsies so we all decided it was in Rafi’s best interest to keep her intubated through the biopsies. With that in mind, we were planning on extubating this afternoon. That didn’t happen. The new PICU attending felt it best to give Rafi one more night on the ventilator to try and even out some of her blood gases. In the scheme of things what’s one more night, right?

Well, it is turning out to be a nightmare. Poor Rafi wants that tube out of her mouth so badly. The doctors have cut back her sedatives and Rafi is asleep for twenty minutes at a time before she is up for twenty minutes. When awake, she is visibly upset at having ot be in bed with the tube. I’ve watched her cry and hold up her arms for me to pick her up. I really with I could pick her up and take away some of the fear. Even though she is on some pretty potent sedatives, she is very lucid. She answers yes and no questions appropriately and the PICU attending says she “isn’t a cheap date”. Only one more night. It could be worse.

What I can tell you at this point, is that the quality of Rafi’s skin has improved. Some of that is due to inactivity for sure, but we can see a distinct change in her skin. It’s very exciting. I haven’t wanted to report things that I hoped to see. I wanted to report things that I know.

Until tomorrow……..

This kid is just amazing. Earlier today she was switched over from the oscillator to the ventilator and she has done incredibly well. So well in fact, that the plan is to extubate her tomorrow. That is really amazing considering how ugly her lungs looked five days ago. There really is nothing else to say but she keeps amazing those who are in charge of her care.

Rafi was being given a drug that literally paralyzed her while she was intubated. We took her off that drug this afternoon and gave her some propofol to keep her nice and sedated. We really don’t want Rafi moving around much for fear of her hurting herself while she has the tube in her. Mainly the tube could hurt the inside of her mouth and her throat pretty badly. In switching her over from the paralytic, Rafi became mildly lucid as she moved around. It was great to see. She could answer yes and no questions.

Later on, as we were repeatedly increasing the dosage of the propofol to higher and higher levels to keep her sedated, Rafi was very clearly and appropriately responding to yes and no questions. This surprised the fellow and the attending, being that her dosage was so high. To give you an idea, the fellow was telling us how 150 should be plenty on the dosage because most children did well on that. We laughed at him. At 200, she was answering yes and no questions. We asked Rafi is she wanted Tylenol, she shakes her head yes. Do you want to watch The Incredibles, she shakes her head no. No one ever listens when we tell them she will need larger amounts of sedation.

If all goes well tomorrow, we could be back in our room on 5D by Monday evening. That is incredible considering they told us the average stay on 5C was two to three weeks. Tomorrow will be Rafi’s sixth day.

A lot happened yesterday. As far as a day in the PICU goes, it was a good day. The doctors switched Rafi from an oscillator to a traditional ventilator. She was on an oscillator to increase her oxygenation. The way the vents work are very different and the differences would take me to long to explain here. The big difference is the oscillator keeps the lung expanded while the ventilator lets the lung expand and contract. The oscillator uses less pressure to expand the lung and is thus less traumatic on the lung. She did every well on the oscillator so now on to the ventilator.

Some of the current settings on the ventilator show that there has been improvement. For instance, her PEEP number is considerably down. A PEEP is a small blow of air that keeps the lung expanded as it is contracting. In a person who is just walking around everyday, the PEEP number is 5. When Rafi was originally on the ventilator, her PEEP setting was at 14. She required a larger blow of air to keep her lung expanded as it was contracting, this is important. The lung has to be expanded a little, even when exhaling and a PEEP allows this to happen. Right now the ventilator is set to provide a PEEP of 8. Also, about 5 days ago, in order to keep Rafi’s oxygen saturation rate above 94%, she required oxygen concentration of 100% to be blown into her. Room air has a concentration of about 21% oxygen. Currently, she is getting a 40% oxygen mixture blown into her and she has an oxygen saturation in her blood of 100%. So we have considerable improvement there as well. Her peak pressures are also much improvement. This means it takes less pressure to expand the lungs. Again, this is a very promising sign. There endeth the lesson as well as just about all the knowledge of ventilators and oscillators I’ve got.

She’s still a sick a little girl, but she’s getting better. She spiked a small fever this morning, 100.6, but Tylenol took care of it. Her white blood count is still elevated and there are some other numbers which indicate an infection (increased monocytes and increased eosinophils) so Rafi is still fighting the good fight. It is assumed she had a nice case of sepsis along with the pneumonia, even though all the blood cultures haven’t shown anything as of yet. Like I said earlier, we may never get an answer to the exact cause, because she has responded well to the antibiotics (fever is generally gone and lungs are improving) the assumption can be made that is was a bacterial infection.

Jackie and I really do appreciate all the words of encouragement and thoughts and prayers. It has made a very difficult time a little easier.

It’s now 6am on Friday morning. There really hasn’t been much to report. We are really in a wait and see mode at the moment. The doctors all think she was trending in the right direction and the plan was to possibly take her off the oscillator on Friday and put her on a traditional ventilator for a few more days. I am thinking that is probably a little aggressive in the timing, but we’ll see.

Thanks again for all the support, we appreciate it. I’ll post more later.

I want to thank everyone for all the love and support. Know that Jackie and I appreciate it and draw strength from it. Right now Rafi is fighting. Progress is measured in baby steps right now. It’s difficult to have no answers, but getting no bad news is great news. So far, we have gotten no bad news, and that is very encouraging. We have been told that things are moving in the right direction and that days from now, she may be significantly better. The waiting is difficult and it is incredibly taxing seeing Rafi lying there, motionless, with a breathing tube in her mouth. Her tiny body rhythmically wiggling to the pace of the oscillator. We are told by several doctors that things are going well. We have the utmost faith in them.

Doing a bandage change is tricky at best. It now takes five people to bath Rafi and change the bandages on her trunk, chest and back. It takes two people to lift her, one to man her breathing tube, and two others to change out sheets and remove bandages. It’s a timely and carefully orchestrated production. The key is to make sure Rafi tolerates the movement and the procedure. She was a champ today. She can’t tell us if she is uncomfortable so we have to watch some of her stats on a screen. If her heart rate or respirations increase we know she is not happy. Today that did not happen. I will never be able to comprehend how tough this little girl really is. I’ve said it before, she is the toughest person I know.

We’ve gotten the results from the endoscopy and the sigmoidoscopy and they are good. As of now, there is no GVHD. So that’s one less thing to worry about right now. There also seems to be some improvement in her lung function which is also incredibly encouraging. The thought now is that there is a bacterial infection that caused this pneumonia. Her fevers have come down, although still moderately high, so we hope this trend will continue.

Rafi did respond today to holding my hand. She squeezed my hand on several occasions and it warmed my heart. While we know she is not completely aware of her surroundings, I believe she knows when Jackie or I are holding her hand. She is on several medications to keep her comfortable and completely still. She is thankfully not lucid, but there is some awareness. Hopefully soon she will again ask us to pat her back, pick her up, read a book, or watch King Fu Panda. All indications point to the reality that this will happen, the question is just when. This is when you realize that patience is truly a virtue.

I wish I could explain the medical information here, but I am out of my comfort zone. I have a peripheral understanding of MAPS, and PEEPS and such but I can’t effectively communicate their importance right now. Maybe in the next day or two as I begin to truly gain an understanding of them and how those numbers relate to her situation, I will report it and try to explain it. For right now, she is doing as well as anyone could expect. She is getting better slowly. As they’ve told us on several occasions, days are the measuring stick. Patience, patience, patience.

Until tomorrow………

Today was a long day indeed. Rafi had scopes inserted from above, below and in the middle today. GI looked at her digestive tract and told us that her insides looked very healthy. On a positive note, and this is according to him, there wasn’t any sign of EB damage in any of her GI tract. Plus, he didn’t see any overt signs of gvhd and he would be surprised if the biopsies came back as positive for it. So we got that going for us.

The pulmonologist also told us that he was very happy with what he saw in her chest as well. Problem is he only went down a little bit with the scope. This means we didn’t see the area of disease. We’ll see what the stains show tomorrow. Part of the procedure was flushing some saline solution into he lungs and then suctioning it out. The idea is for some diseased cells to get caught in the saline and then we can look at the cells and figure out an origin. The stains tell us if it is fungal, bacterial, etc. So far, we know it is not fungal.

We also switched her from a ventilator to an oscillator. The oscillator is better for her lungs because it uses less pressure to inflate and deflate the lung. This will lead to less lung damage (if any at all) over the long haul. A ventilator uses a greater amount of pressure to expand the lung. The oscillator can keep the lung expanded for greater periods of time without subjecting them to an excessive amount of pressure at each respiration. This has also helped her to expel more carbon dioxide than before as well. Her blood gas numbers and her general saturation numbers have been better as well. So we have a win on several levels.

She has been spiking some pretty big fevers today, with a high of 105.0. It’s really terrible to see her like this all the time. The next 48 hours will tell us a lot. Then again it may not tell us anything. That’s the thing with this. We may never know what caused her fevers and her pneumonia. We have a couple of the doctors thinking it is an aspiration pneumonia. Meaning, during one of her vomiting sessions, Rafi inhaled some of the vomit and it caused a chain reaction that led to this. There has also been another off the wall idea, toxic shock. Staph releases toxins into the blood, she has a ton of staph, always has, and it may have all caught up with her. Of course, both theories could be wrong.

I am literally falling asleep at the computer so it is time for me to bid you goodnight.

Until tomorrow………….

Today was a scary day. Tomorrow will be a scary day. Rafi’s x-ray showed a subtle pneumonia. By the time we had a ct scan it was a serious pneumonia. So serious in fact, that Rafi is now intubated on the pediatric intensive care unit. As it turns out, Rafi was in respiratory distress and the doctors and nurses moved quickly to make sure we could be one step ahead of an emergent situation. Instead of waiting for the last possible second, and involved in a scenario where we had no control, it was decided to sedate Rafi and intubate her. We need to find out the cause of the pneumonia and treat it accordingly.

For the foreseeable future, a machine will breathe for Rafi. She will be drugged to the point where she is not in the moment. This poor child has been through so much. Thankfully she is strong and her general overall health will help her in her fight now. I miss her smile, her laugh and her comments, and it’s only been a few hours. I held her hand and there was no response. I want to hear her say “Daddy pick me up please” so badly it hurts. I am relying on her strength and her determination now. She is resilient and she will get better.

Tomorrow they are going to do several tests on her. She is going to have a bronchoscopy, a sigmoidoscopy and an endoscopy. The sigmoidoscopy and endoscopy are to check for acute graft versus host disease. The bronchoscopy is to try and diagnose the cause of her pneumonia. Basically, they are going to put a scope into her lungs, inject some fluid and then take it out to see if the cause of it is due to virus, bacteria or fungus. There is no guarantee that we will get an answer from this procedure. We are hopeful though.

We know these types of complications are not unusual for transplant. Rafi had just done so well to date that we were hopeful that we would be the odd case of a transplant without a complication. Oh well, it was not to be. Days are now measured in weeks but the hours crawl by. She will probably be intubated for weeks now. The damage in her lungs will take time to heal. We are hopeful.

Rafi is strong and she will get better. Until tomorrow…………

I wanted to write something witty as to why we are still in the hospital. I wanted to be able to give an idea of when we could expect to be out of here and back home. Problem is Rafi’s fevers won’t go away and they spike at the most inopportune time, like when vitals are due. As of 5:45am this morning, she has pneumonia. We had an x-ray at 2am, and the radiologist made the diagnosis a few hours later. Her oxygen saturation level has gone down significantly and we are trying to keep an oxygen blow by as close as possible. It is proving difficult. I have to go and take care of Rafi now.

Today was the fourth day out of the hospital and the first day back in it. We figured there was no way we could pass up a stay with room service. Rafi wretched at 6am and she was quite warm, so I took her temperature. It was 101.4. I figured she just threw up so maybe she is just warm from that. An hour later, we took her temperature again and it was 102.4. That earned us a minimum 48 hours back on 5D. After getting off the telephone with the fellow on call, Wes, we took showers, packed two bags and went back to the hospital. We got our old room and got the first dose of two different IV antibiotics. Wes asked us, if there is a next time, to be a little more expedient in checking back into the hospital. He told us it is very important to get that first dose of antibiotics into Rafi. Well, we know better now.

We knew that we might have to get readmitted at some point, so we are not upset or discouraged. This is just another step in the journey. By late this afternoon, Rafi no longer had a fever and was in good spirits. She told Jackie and I that she wanted to go home and that she didn’t want to stay there. However, sh seemed to understand when we explained it would only be for a couple of days.

We washed and redressed her feet yesterday, and I was interested to see how the blister on her right foot would look. As it turns out, the blister was no longer there, in fact the area looked healed. There was no trace of a blister ever existing. I was shocked. She has never healed that quickly before. There was another blister on the foot so I am excited to see if the result is the same the next time we change the bandages. Unfortunately, it looks as if Rafi has some staph growing in her wounds so we will have to really wash those affected areas tomorrow with bleach. I am guessing the infection(s) are the cause of the fever so I hope to be able to clear them quickly. We’ve cleared infections quickly before so I am not that concerned.

Please send some positive thoughts to Kenzi Gray and Sam Sheridan. Kenzi is right in the middle of the difficult time of the transplant and is fighting hard. Sam is still battling the after effects of transplant, and can use a good break.

Rest in peace Karen Fuccello. Karen was my aunt by marriage and was an incredibly nice woman who touched many. She will be sorely missed.

Until tomorrow………

Sometimes a change of scenery does a body good. In the hospital, Rafi wouldn’t walk without holding our hands. Now she is running all over the place without help. In the hospital, Rafi wouldn’t stand up on her own, she would always make us pick her up and put her down on her feet. Not anymore. Her physical therapists would be very proud of her. We’ve also had two different nurses the past two days and Rafi has really liked both of them. She will even leave Jackie and I and hang out with her new “friends”.

It’s been great to see her improve a little each day. The increased activity has taken a small toll on her skin though. She did get a blister on one of her feet, I am more than a little interested to see what it looks like now. Did it heal faster? Did it get worse? We will find out later today during bath and bandage change. Speaking of bath and bandage change, for the past two we have only given her tylenol. We think that when the strong pain medications kick in and make her feel a little loopy, she anticipates that something bad is going to happen and she acts out. So, for now, we stopped giving her the dilaudid for bath and bandage changes and she has been great.

We have to go to clinic this morning, so I am going to sign off for now.

We are home. We left the hospital today after 56 days. Rafi was so happy to see the car and take a ride. But, this didn’t compare to when we entered the house and she saw all her toys that she hasn’t played with in two months. “I get something else”, was heard for awhile.

It amazes me how much stuff we either had or accumulated in the hospital. Now we have the daunting task of unpacking everything, taking inventory of all our supplies, becoming organized and getting into a medicine dispensing routine. Regardless of everything ahead of us, it’s just good to be home.

We finally got her skin engraftment rate today. 14% of the cells in her skin are donor cells. The doctors wanted to see anything above 0%, so we are happy with the results.

I have more stuff to get from the car so I am going to sign off for the night.

In the words of Paul Simon,

“Homeward Bound, I wish I was homeward bound”

Tomorrow is the day, we don’t have to wish anymore. Unless the little one spikes another fever, we are going to go home. I don’t think I have to tell you that it will be nice. Rafi will get to run around her apartment, play with her toys, and sleep in her bed. She’ll laugh more, she’ll heal better.

There really isn’t much to say about her health at this point. Her numbers are great, and her wounds are healing. Do we see any alleviation of EB? No, she still gets blisters that we have to lance. Sure she is getting less blisters, but her activity level is less than before transplant. We will see what happens when we get home and she starts moving around. I can’t wait to see it. She seems to be healing faster, but I am wary of reporting what I want to see and not what actually happens. Basically, it is still to early to tell, but we remain very hopeful. Now we just have to get her to eat.

Hopefully tomorrow I will update from home.

I guess Rafi just loves the hospital and the people here. There is really no other explanation for the fever that popped up this morning, then immediately went away, earning us two more days here. Of course, she didn’t have a fever since that one spike. I think she is waiting for Lindsay to come back to be the one to discharge us. On top of that, our Aerobed decided that it had enough of the hospital and went into full cardiac arrest last night. So now we are sleeping on the hospital provided cot. I am medicating prior to sleep with Advil and will have three tablets on the table for first thing in the morning.

Jackie left earlier this afternoon to go home and clean all of Rafi’s toys before our eventual departure. After a long nap, Rafi and I spent a nice afternoon together outside on the swings and then back in the room watching movies. She finally fell asleep at 11:30ish, in all fairness we did nap together in the lounger earlier around 9ish watching, you guessed it, Kung Fu Panda. Hopefully she will stay asleep all night with the ambien and dilaudid on board. She has been powering through those and waking up in fits and spurts the past few nights, and then taking ridiculously long naps in the afternoon. Keep your fingers crossed.

I haven’t posted her numbers in a long time, so I might as well now:
WBC = 11.4
ANC = 8.0

Off to do some research about Eosinophils and what they mean. Dr. Troy Lund has been making fun of me and my google habit and I am trying to come up with the reason Rafi’s EOS number is high. It is nothing to be worried about in any way shape or form, but I am a curious sort so I must find an answer. He tells me to stop searching and I keep hitting him new theories. All of them wrong in his eyes. I must find the answer. Until tomorrow…….

Thanks to everyone who is trying to help with the diarrhea. We appreciate it and will try to use the ideas. Jackie has always believed that the diarrhea was caused by all the antibiotics. The doctors tell us this is a very normal post transplant situation in that her gut is taking more time to adjust and start up again. I have been away from the hospital all day shampooing the rugs and furniture in preparation for Rafi’s return home, so I don’t know if the volume of her diarrhea has diminished. It was a lot less earlier today, so we’ll see. We are just trying to avoid any fevers at this point, because that is the main reason we would have to stay in the hospital longer. So far so good, even though we had a close call earlier in the evening. Jackie told me Rafi had a 100.3 degree fever. 100.5 earns you a stay in the hospital for at least 48 hours, so we are prepared to bathe her in ice water, have her eat popsicles non-stop, or sleep on a cooling blanket.

Really not much happened today at all. We are just biding our time. We took Rafi out into the sixty five degree weather and just cruised around. Hopefully she is sleeping now peacefully so Jackie can get some rest. The next couple of days are going to be extremely busy getting the apartment ready and packing up the hospital room.

Until tomorrow…………

The sigmoidoscopy and the endoscopy were scheduled for this morning at 11:30. At 10:15 our transport came and took us down to pre-op. Rafi got her dose of versed to make the trip into the operating room and her time on the table in front of several total strangers in a brightly lit, albeit green, room fun. The propofol and ketamine IV combination was very effective in keeping her sedated and pain free. The first thing the GI doctor did was try to remove the gtube. This proved to be rather difficult as Rafi’s opening into her stomach has healed so nicely and it has actually narrowed. Problem is once we got the gtube out, the endoscope would not fit into the hole so we were unable to look inside the stomach and peer up into the esophagus. I was actually quite interested to look at her esophagus to see what damage, if any, has been caused by EB. We then continued on to the second phase of the procedure, where the GI doctor put an endoscope up Rafi’s lower GI tract to take some biopsies to check for GVHD. This was all accomplished and it was interesting to get a good look inside the large intestines, rectum and sigmoid colon. She, the GI doctor, took 5 biopsies and told me that everything looked great. There were no ulcerations, or damage of any kind, in fact everything looked quite healthy.

When that was done we attempted to put the Gtube back. Well, that was not going to work. The hole had become too small with scar tissue and it was proving near impossible to replace it. The doctor didn’t want to hurt Rafi in any way, so again we had a nice little catch 22. One of the nurses thought of trying to put an slightly larger rigid instrument into the hole to “stretch” it out temporarily. That worked well, so I attempted to convince the doctor to get a new endoscope and let’s take a peak. I was very surprised that she was hesitant to do this and I wasn’t. I figured we were there, Rafi was sedated, doing very well, the doctor was showing a tremendous amount of consideration and care in working on Rafi, so I felt we could go for it. Clearly she didn’t expect this from me and we had to wait for a new endoscope to arrive. After a couple of minutes, the doctor tried to remove the larger instrument from the hole and she got nervous that she may have caused a blister on the outside of the opening. I swear she absolutely got upset at the possibility of creating a blister. So, she didn’t want to wait for the endoscope and thought it best to just replace the Gtube with a new one. So once again, we were unable to get a peak inside Rafi’s gut. While I know she doesn’t read this blog, I would like to tell her that she did not cause a blister. I will pass that on to her through our attending here.

After the procedure we took Rafi directly back to our room here on 5D. She slept for a ridiculous amount of time. She didn’t wake until 5pm. It is my night in the hospital, so I thought I was doomed for a long night. I had the movies picked out, Cars then Kung Fu Panda, then The Incredibles followed by Shrek if necessary. Not quite half way into Kung Fu Panda, she looked at me and said:

Rafi: “Daddy, I’m tired. I lie down now. You lie down with me?”
Brett: “I can’t Rafi, your bed is too small for me and for you at the same time”
Rafi: “OK, I lie down in my bed and you lie down in your bed.”
Brett: “I’ll tell you what, you lie down, I’ll pat your back and then I’ll lie down in my bed.”
Rafi: “OK”

That conversation happened around 9:30. I still can’t believe it.

Earlier in the evening, we got word that the biopsy results came back and there is no GVHD. My shoulders got a little lighter and a big smile came across my face. No GVHD, I really like the ring of that.

As it stands right now, as long as Rafi doesn’t spike a fever, we are home bound on Monday. That will be our 55th day in the hospital. The doctors and nurses here have been great, and Jackie and I have become fond of several of them. We will miss them, but I can’t wait to be home all the time. Here’s one other thing Rafi said tonight that I will leave you with and then I’ll call it a night.

Rafi: “We not home Daddy”
Brett: “No this is not our apartment, we are home for now”
Rafi: “No, Daddy this not home. Home is Minneapolis”

Too funny.

Until tomorrow…………

Tomorrow morning, Rafi is going to have an endoscopy and some biopsies to see if graft versus host disease is the reason she is putting out so much stool. We have dreaded this procedure for so long that it’s a bit surreal that we are going to have to subject Rafi to it now. She has endured so much to this point but in then end what is just one more test. I think after it is over it will have been harder on Jackie and I than on Rafi. So far, that has rung true with every test and procedure. We’ve worried about how she would fair through some things and each and every time she has surpassed our expectations and shown that we worried about nothing. I guess that will always, and always has been, the case in being parents.

We did finally give her some food today though. Once the decision was made to undergo the endoscopy, Rafi was allowed to have her popsicle. Not that a popsicle is food, or the soft ice cream we gave her, but i think the chicken noodle soup counts. Soup is her new favorite. I never thought baked beans would play second fiddle. Then again, Shrek has now been replaced.

Other than the diarrhea issue, Rafi is doing exceptionally well. Her numbers from the blood tests are still great. She hasn’t needed a platelet or red blood cell infusion in a week or more. Her temperature has been normal for almost four days now and her blood pressure is very much under control. We spent time outside in her pink convertible again today. She just has so much fun in the car. Jackie is tiring of hearing me say. we need to get one at home. I just can’t get over how much better it is than our fancy and expensive stroller.

It is quite possible that we get to bring Rafi home with us on Monday. That will be nice.

Until tomorrow……….

Well we are still in the hospital for the next few days. Why you ask? We’ve conquered the fevers and overcome the high blood pressure. The blood engraftment studies have come back and shown some good results. However, we can’t reduce the amount of diarrhea she is having. After two days of no food or water, her stool output is the same. Conventional wisdom is pointing to graft versus host disease (GVHD). GVHD is when the new cells see the body as the problem and attack it. Manifestations run the gamut from mild to very severe. The only way to diagnose GVHD of any kind is to biopsy. While it hasn’t been officially decided yet, we are just about positive that Rafi will undergo an endoscopy. Up to this point, Jackie and I have endeavored to never have Rafi undergo an endoscopy. And there have been those that have wanted to in the past. Sticking an endoscope up her anus into the lower GI tract, can’t be good for an EB child. But it’s becoming clear that we can’t avoid it. If she does have the particular type GVHD they are considering, then this is the type to have because an eight week course of steroids should treat it. Rafi has done so well through every stage of this journey that we can only assume she will do just fine through the endoscopy.

They’ve told us that no child goes through a transplant without a complication. Up to now, we’ve had no complications. It was too good to be true and we did have to expect something. So let’s hope that if this is the complication she has to undergo, then it be a mild case that can be treated easily with steroids.

Some have asked if we can expect her EB symptoms to subside now that engraftment has happened. The truth is we don’t know. We have to remember that Rafi is patient number eight. There just isn’t enough short term let alone long term data to know anything. It is our hope that Rafi’s new cells will create more collagen VII and allow the skin to bind to itself and for the mucousal membranes to be less affected. It is our hope that this procedure we put her through will be curative. We just don’t know and as of now, there is no information available to point in either direction. We know the others are doing well and for now, we’ll take that.

Until tomorrow…..

We got some more test results back yesterday. It’s always dangerous to give a guy like me some information and a computer with Google. Her blood tests are back and she is now 100% donor cells for neutrophils and 96% for t-cells. Of course I had to do research as to what the CD3 cell engraftment meant, and that’s why I didn’t post last night. I was emailing Drs. Wagner and Tolar at 1am asking them to correct any incorrect assumptions I was making based on my research. I read journal articles stating that high levels of CD3 cells was a potential indicator of graft versus host disease. My mistake was not taking into account the difference between percentages and actual numbers. Suffice it to say, they emailed me back early in the morning and came by today to alleviate our fears.

Some more good news is that Rafi has been without fevers for the past two days and her blood pressure is now under control. It’s amazing what an adult size dose of a beta blocker for a two year old will accomplish. The only thing left now is reducing the amount of diarrhea. She is going to the bathroom a lot, and has been for a period of time. So when researching the GVHD aspects of high CD3 engraftment at such an early date on top of the excessive stool, it started to make me wonder if Rafi has some GVHD. We’ve been told that from all indications she doesn’t have GVHD but we need to see the source of the diarrhea. It could be GVHD but it is way to early to tell. Dr. Tolar told us that he highly doubts it because the color of the stool is fine (an indicator of GVHD is color of stool) and she doesn’t seem to be in any discomfort besides the diaper rash. But just to be safe, we stopped her food and liquid intake for a day to see if the volume decreases. This has not made Rafi a happy camper. She loves her water with ice and she was hungry for a good part of the day. We also had a GI consult just in case we have to take a peak inside and do a small biopsy. That will be determined in the next day or two.

We are still waiting on the skin engraftment reports. These results will tell us what percentage of cells in the skin are from the donor. Hopefully we will have those in the next day or two, which should correspond with our discharge.

Rafi is generally in great spirits lately. She is sleeping a lot better since we added the Ambien. She is also napping during the day. These are both firsts for us and may take a little getting used to. Today was a beautiful day, low to mid 70s, so we left the hospital again and played on the swings and took a short walk up the Mississippi River and watched the college kids play some soccer and football. It was nice. I am looking forward to more time outside with the little one and Jackie.

Until tomorrow with hopefully more good news……….

Rafi no longer has an infection in her blood. She had cultured out staph on her central line a few days ago. We thought that this was the source of her continued fevers. To kill the infection in her line, she had all sorts of antibiotics and they put ethanol in her lines to help kill the bug. Problem now is that she is still having a fever. She is drinking and eating well and we are going to start the gtube feeds again tomorrow. We’ve conducted some extra tests to try and find the source of the fevers. Fact is they might just be fevers because she is creating more white cells. But just in case, we cultured phlegm looking for viruses. Generally, we just hope these fevers will go away and we can go home soon.

We did take Rafi for an extended walk outside today. We wrapped her up in a sweater and a blanket, put her in her little pink car and cruised Washington, Delaware and Harvard streets. She was having fun out in the beautiful day and then decided she was hungry for baked beans and chicken soup. So back we went to our temporary home on unit 5D.

Her white blood and her absolute neutrophil counts are both very high. Truth be told, we don’t even pay too much attention to the numbers anymore. We look more at her platelet counts and her hemoglobin. Both of which are great. She hasn’t needed a platelet or a red blood cell infusion in a while so we hope this means we have engraftment.

As to her wounds, she is still getting some blisters and her skin is still quite fragile. While on her versed and dilaudid before her bandage change today she hit her face on the crib and up came a blister. We do think she heals a little faster though. Her feet look really great and they are generally one of her trouble spots. About a week ago, Rafi cultured out some MRSA and heavy staph. Now the wounds no longer look infected, the smell is gone and they look quite good. It’s amazing what some antibiotics, some bleach and some Restore silver dressings can do.

Time for me to catch up on a lot of sleep. Until tomorrow………..

I know, I know, it’s been a few days since I have posted. Sorry. I had a couple of friends, Peter and Tore, in from out of town and we have been out and about. Jackie was nice enough to stay in the hospital for the past two nights so I could spend the time with them. It was a welcomed distraction for me and as it turns out, for Rafi as well. She has decided that Peter and Tore are not Jackie’s and my friends, but rather they are her friends. Las night when the three of us left for the evening Rafi got very upset and cried. An unusual reaction from her. This morning when I told Rafi that I was going to go and pick up our friends, Tore and Peter, she said, “No, no, no they are my friends.”

Rafi is still spiking fevers but we seem to have gotten her blood pressure under control. As it turns out, the automatic blood pressure machine isn’t quite as accurate as one would hope. Plus, we doubled her dose of one of her beta blockers and the two factors did the trick. Rafi is also very close to her admission weight now, only 100 grams off, so that’s another good sign. She is drinking fluids orally and can eat a good amount of chicken noodle soup. She continues to do extremely well through this process, even though our hospital stay has become a little longer than we thought. The doctors tell us she is not only doing very well for an EB patient going through a transplant, but also any child going through a transplant.

Her spirits have been better than we imagined they could be at this point. Nurses who haven’t seen her in a week or more say she is a completely different child. There are times that she actually engages in conversation. Jackie and I have learned that Rafi does much better with people when we leave the immediate vicinity. So we are trying to cut the cord at times.

We got word that NY Medicaid is allowing us to get a full time nurse when we leave the hospital. A nurse at home will prove to be very helpful for Jackie and I. A nursing agency I contacted awhile ago, before we even came to Minnesota, was willing to file the appropriate paperwork to get enrolled in NY Medicaid. Add to that the outstanding help of Katie at Skip of NY, who shepherded the entire process, we will be able to have the help we need when we get out. It will be nice for Rafi to have another adult presence besides Jackie and I.

We have been waiting on the results of two tests to see how the transplant is working so far. We got the results of one of the tests and it wasn’t the more important of the two. The immunoflourescent mapping did not show an increase in collagen VII at the dermal epidermal junction to date. While that sounds horrible, we want a higher level of collagen VII at the junction, Dr. Tolar explained that this finding was expected and that they don’t usually see an increase in the levels of collagen VII until at least day +60. So we now eagerly await the rflp test results which will show the percentage of engraftment.

Until tomorrow…………

We’ve decided to give Ambien another chance. By that you should have surmised that it didn’t work. The little one can power through all kinds of drugs. Take for example, her blood pressure is still high, no matter how many drugs or of what concentration or dosages they are. To give you an idea, Rafi is currently on 6 blood pressure medications and her pressure is still high. It’s probable that the immunosuppressant (cyclosporine) is responsible for the hypertension. Of course, certain types anti-hypertension drugs increase the kidney’s level of sodium. This leads to hypertension. Nice little catch-22 we find ourselves in here. I am more inclined now to push the doctor to fall back on the blood pressure dugs, clear the kidneys of the sodium with diuretics and then see where we are. Because at this point, just adding drugs or increasing dosages is getting a little insane. Plus, repeating the same behavior and expecting a different result is the definition of insanity.

We discovered the source of her fevers today. Turns out Rafi has a little bacterial growth in her blood. They think it is a variation of staph and they should be able to clear it up pretty quickly once we get the sensitivity results. Those results will tell us what antibiotic will work on the infection. This is not uncommon at all and one of the other side effects of the cyclosporine is an increased risk of infection. Go figure. We also found out that she once again has some MRSA growth on some wounds. She’s tested positive for MRSA once before but we were able to beat it pretty easily. Hopefully, we can do it again with a lot of bleach, and some silver impregnated bandages along with the antibiotics she is taking. We were warned by Drs. Wagner and Tolar that these EB kids tend to have some issues right at the time of discharge. Well we didn’t break that mold.

Rafi’s numbers are still great as are her spirits. She even played with a veritable stranger this afternoon. There is a program here, called Care Partners, where people volunteer their time to play with the kids, give you a break, run errands for you, etc. This one woman came in and Rafi was engaging and talkative and played with her for a long time. We were surprised being that Rafi is very shy. To give you an idea, Lisa, one of our nurses who is with us a lot of the time, is finally being addressed as “she”. “What she doing?”

Rafi actually fell asleep on her own this evening. I can’t remember the last time that has happened. So we put in the Ambien about fifteen minutes ago (about a half an hour after she fell asleep) and we plan on giving her some longer acting pain medication (dilaudid) in about forty five minutes. I hope this cocktail will keep her asleep for a period of time. Conventional wisdom is telling me to sleep now stupid.

One last thing, for those that are going to follow on this journey. You are going to have to get ultrasounds at some point and if your child has open wounds or very sensitive skin, you may be worried about the direct contact of the wand to the skin. We were. As it turns out, using Vigilon or Cool Magic gel sheets work very well in protecting the skin and still letting the technician to get good pictures of the organs.

Off to bed if that’s possible, until tomorrow………………

It’s been a busy couple of days. We were originally scheduled to exit the hospital yesterday but alas, it was not to be. Rafi started having fevers again and her blood pressure is still pretty high so we are still on 5D and will be for a few more days. Today, we had another CT scan to investigate the possible source of the fevers, then she had an ultrasound to look at her kidneys to see if they were the source of the high blood pressure. All tests showed she is doing very well. So we are still treading in the land of unknown but nobody is worried. She has no signs of GVHD which is comforting. GVHD is when the new cells see the body as the problem. The manifestations run the gamut of serious to mild. So far so good, so we are still smiling.

Actually we are smiling a little more today. Rafi hasn’t slept for the past six nights. It’s been tiring at best, and frustrating continually. Our savior nurse, Lindsay, whose not been on the floor recently, came back on duty and got Rafi some Ambien. We hope this will let Rafi get a good night sleep. Which means we can hopefully get some sleep as well. We need it almost as badly as Rafi does. Jackie is in the hospital tonight so please keep your fingers crossed.

Now for the bigger news. Yesterday was Rafi’s skin biopsies and skin fragility test. The skin fragility test is one in which the doctors, using a suction device, force a blister to appear. We did this before the transplant started and it took less than four minutes for the first blister to appear, and just over four minutes for two more to appear. A person with normal collagen VII levels will take about forty five minutes to an hour for a blister to form. Yesterday, blister number one formed in just over fourteen minutes. The next two blisters took over twenty minutes to form. In and of itself, this test doesn’t really tell us anything because there could be a number of factors that make the difference. Nonetheless, I choose to believe it is the first indication of success and of wonderful things to come. We are hopeful that this is the first sign that the transplant is working. We should know more by the end of the week. We are waiting on the blood tests that show engraftment and the skin biopsies, so I’ll have more to tell later on this week.

Rafi continues to make everyone around her laugh. She doesn’t engage people directly but the things she says entertains and surprises those around her. Her favorite thing is to now quote the movie, Kung Fu Panda. At any given time of the day or night, the movie is playing. She is also quite taken with The Incredibles as well. Other than her books, her new found love is stickers. We’ve never let her have them before, but the really are a great reinforcement for her walking. There are rolls of stickers at the nurses station so we constantly try to entice her to walk the hallways back and forth to get to those rolls. Each time she gets there, she gets another sticker or two. She now has a box full of stickers with Mickey Mouse, Dora, Elmo, or whatever is available. You can hear her down the hallway saying “I see the stickers” or “look, stickers”.

It’s time to pay some bills and try to get to bed at a very early hour. Until tomorrow………..

Well it looks like we are going to spend a few extra days in the hospital. Rafaella had another fever today and she has to be without a fever for at least two days before we can leave. Oh well. Tomorrow Rafi will have her first set of skin biopsies since transplant. Neither Jackie and I can wait to see the results from this test. Rafi is completely off her pain medications although we can still get some relief for her if it is necessary. Her blood pressure is still running high so we took her off the steroid medication for nausea and we hope this will help.

I hope Rafi will sleep through the night, or at least a good portion of it, this evening. We’ve had five straight days of literally no sleep. She wants to sleep but can’t seem to stay asleep for long periods of time. We’ll see what happens this evening. I hope it isn’t too rough on Jackie. We have a long day tomorrow and we will both need our wits about us.

I will write a more thorough update tomorrow after the skin biopsies. Until tomorrow………..

Sorry for not posting last night. I was too exhausted to even write one coherent sentence. I was up the entire night on Thursday with Rafi. Will someone please teach my daughter how to sleep at night? Please? Pretty please? Of course last night, she didn’t sleep either. That’s four nights in a row that Rafi hasn’t slept. She also hasn’t had an afternoon nap on any of those days. I have no idea how this little girl is functioning. She sleeps early in the night for about 2 hours and then she is up asking for her back to be pat. We pat her back until she is asleep, make our way the 3 feet to our bed and by the time with Jackie or I lay down, Rafi is asking for her back to be pat. This goes on all night. Finally around 7:30am, Rafi falls asleep for an hour and a half. That’s it. All day. So now it is Saturday night, it’s 10pm, it’s my turn in the hospital and I just got Rafi to sleep 10 minutes ago.

We have developed a plan for tonight. We think that Rafi is waking up because she is uncomfortable. We’ve lowered her pain medications in anticipation of us getting out of here and I think that has backfired to a degree. Therefore, we are going to give her a dose of dilaudid when she is asleep and see if she stays asleep. Why dilaudid you ask? Well, fentanyl has a much shorter half life than dilaudid. In other words, dilaudid will last longer. Wish us luck.

I got a text message from Jackie this morning around 6:30am that said, “She has a fever, 101.9″. My reply was, “So much for leaving the hospital on Monday.” The threshold for a fever is 100.5. If we are home and her temperature rises to 100.5, we have to get readmitted for a couple of days. So it is looking more likely that we will leave the hospital on Tuesday or Wednesday at this point. We’ve already been here for 39 days, what’s a couple more?

Being that she is shedding the fluid weight, we stopped the diuretics. We have less than a kilo to go before she is back at her admission weight. That leaves only one other issue at this point, her blood pressure. We just can’t seem to get it under control. She is on five medications and this plan just isn’t working. I am going to remind the plethora of people who will be outside our door at 8:30ish tomorrow morning that the definition of insanity is repeating the same action and expecting a different outcome. I am sure that will go over well, but I don’t care.

Rafi is getting more chatty as the days go by. She is speaking in complete sentences now. Pretty good for a 28 month old. Not with the nurses though. She doesn’t really talk to them. I don’t know why. We were hoping our stay here would help alleviate her unbelievable shyness. Not so much. But tonight’s conversation centered around her eating. She told me she was hungry even though we just had dinner a few minutes before. The she broke out the line of the week. But first a little background. Rafi has never been a big eater. She stores food in the front of her mouth like a confused squirrel. She’ll keep it there for hours. It’s pretty impressive really. Anyway, here’s our conversation before bed:

Rafi: “Daddy I don’t know how to eat.”
Brett (trying to suppress laughter) : “That’s true Rafi but we’ll teach you and then you can eat anything you want.”
Rafi: “I have to put the food in the side of mouth with my tongue, and then chew like this (demonstrates).”
Brett (trying very hard not to laugh): “That’s right Rafi, very good, we can practice in the morning, time for sleep.”
Rafi: “Daddy, you get me a green chair so you, me and mommy can eat together.”
Brett: “Rafi, you already have a green chair at home. We can all eat together.”
Rafi: “We can eat together?”
Brett: “Yes, booba-loo”
Rafi: “OK, we eat Pizza and green waffles together. I’m tired”
Two Minute Pause
Rafi: “Daddy you don’t leave.”
Brett: “Sweat Pea, I am tired and let me show you were I sleep.” I sit her up and show her the bed 3 feet away. “You see the bed, I am tired, there is mommy and daddy’s bed, I am not going to leave.”
Rafi: “You tired too Daddy?”
Brett: “Yes Rafi. I am going to sleep very soon.”
Rafi: “You lie down with me and I pat your back?”

She just cracks me up. Total non-sequitur, I thought I would keep every empty syringe today and then list the medications she gets in her gtube. These are in no particular order and these are only the medications she gets in her gtube.

1. Ondansetron – 2.5 mls
2. Melatonin – 3 mls
3. Acyclovir – 5 mls
4. Gengraf – .4 mls
5. Gabapentin – 2.3 mls
6. Lansoprazole – 4 mls
7. Nifedipine – 1 ml
8. Metoclopramide – 1 ml
9. Midazolam – 3 mls
10. Gengraf – .4 mls
11. Ondansetron – 2.5 mls
12. Acyclovir – 5 mls
13. Atenolol – 1.5 mls
14. Acyclovir – 5 mls
15. Lansoprazole – 4 mls
16. Dexamethasone – 2.5 mls
17. Amlodipine – 2.5 mls
18. Fluconazole – .5 mls
19. Metoclopramide – 1 ml
20. Amlodipine – 2.5 mls
21. Acyclovir – 5 mls
22. Nifedipine – 1 ml
23. Gabapentin – 2.3 mls
24. Nifedipine – 1 ml
25. Acycolvir – 5 mls
26. Atenolol – 2.5 mls

That’s 66.4 mls of medicine today, or a little more than a quarter of a cup, and that doesn’t include a couple of others that are still running IV. Anyway, it’s 11:30, her blood pressure is high, this after a dose of nifedine under the tongue, and I have informed everyone that there will be no more blood pressure medicine given for awhile. Let’s see what happens. If it gets to high, we can give her whatever fast acting medication they have. I am just testing a theory at this point. Also, she is still asleep. So I got that going for me.

Here are her counts from this morning. She did not get another dose of G-CSF. On Thursday, her ANC was 1.3, on Friday it was 1.5 and you can see below for todays.

WBC – 5.4
ANC – 3.3

Until tomorrow……….

Another day another $15,000, and another drop in the WBC and the ANC. The drops in the counts are completely normal. However, in trying to ascertain when the drop will plateau, one doctor will say 3 to 5 days and another will say 5 to 7 days. It’s a good thing I already have confidence in the doctors because they might have well said, you can’t get there from here, or we’re open 24 hours, just not in a row, to answer my questions. Regardless, tomorrow, or at least at 4am, they are going to draw more blood than normal for her RFLP tests. This test will allow us to look at Rafi’s DNA and see if the transplant is working. Basically, you do the test to clarify cell origin. They can see if Rafi’s cells are from Jackie and I. If they are, the transplant is not working well, if they aren’t then it is. Please understand this is a very dumbed down explanation of the results of this test. Dr. Tolar told us this morning that it takes about a week to find out the results. It will certainly not come fast enough for me or for Jackie.

Once again Rafi did not sleep last night unless Jackie was patting her back. This means that Jackie didn’t sleep. We figured the kid would be in a terrible mood all day, sleep a lot and make me miserable tonight. Well, she didn’t sleep all day, she was in a great mood, but it did take me until almost 10:30 to put her to asleep. It’s just about midnight now and she hasn’t moved an inch. I am hoping she doesn’t move for another 7 hours.

Jackie’s father and sister arrived into town today and that in itself makes Rafi happy. Add to that fact, that we went to the swings and then walked down by the river in our extended trip from the hospital, and Rafi had another good day. Plus, and here is the exciting part, Rafi ate quite a lot for dinner tonight. She just kept on eating. That’s amazing because she was never like that before. I know, everyone is going to now say the transplant is working, here is the proof. Don’t say that. Think about how you would feel if you didn’t have a bite of food for a month and then all of a sudden you started to feel better. Chances are you would eat a lot as well. Regardless, it was great to see and fun to be a part of. First she wanted her beans, then she wanted a sandwich, then she wanted a popsicle, then she wanted another one, and finally, she wanted chicken soup. Top that all off with the movie, The Incredibles and then bed and you have a wonderful night.

This weekend is going to prove to be quite busy I am sure. If we are going to get out of here on Monday, which is still the plan, we have a lot to do. We have to scrub the apartment, shampoo the rugs and the furniture and pack this room up. We have a lot of cleaning to do, but I can assure you it will definitely be the only time in my life where I will love cleaning up.

WBC = 2.7
ANC = 1.3

I told you they dropped. Hopefully, they won’t drop much more and they will start climbing slowly. It’s my understanding that the numbers are going to go up and down for a long time going forward and we might need some more infusions of G-CSF. I am not concerned. Rafi has done amazingly well till now, so I have to assume she will continue to do amazingly well in the future.

Until tomorrow……………………

Every day we see Rafi getting a little better even when she sometimes takes a step back. I was a little premature in saying we broke the patterns, or perhaps the fake wood was responsible. She slept horribly last night, which meant I really didn’t sleep at all. We decreased her fentanyl again and that made her a little uncomfortable. At one point she was receiving 1.2 mcg/kilo/hour and now she is down to .3 mcg/kilo/hour. She was also nauseous throughout the night and she told me at around 2am, “my stomach hurts” and “I am scared”. I ask her what she is scared of and she says, “I am scared of throwing up”. There really isn’t much to say to her at that point besides, “Mommy and I understand you are scared, and it’s OK to be scared. We all throw up sometimes but it makes you feel better.” So I pat her back and try to get her to breathe slowly. I ask her to breathe “in through the nose and breathe out through your mouth slowly”. What kills me, and just makes me so darn proud, is that she listens and she does it and she eventually drifts off back to sleep. For ten minutes. Finally she asks me to “push the button for the nurse, I want medicine”. She speaks in full sentences. It cracks me up.

Problem is this went on all night last night. Thank goodness Jackie shows up early to the hospital. I slept for an hour and a half and then got another nap in a little later. This journey is tiring, even though we are only along for the ride. Jackie and I are both amazed at those that do this alone. It is impressive.

Rafi’s numbers did drop again but only a little bit. Actually they dropped less than expected. We are seeing the benefits of the white blood cells and are amazed at what those cells are capable of accomplishing. She is really looking significantly better. Plus, she started to shed to excess water weight. She was down almost half a kilo yesterday and is now less than a kilo above her admission weight. You can really see the difference in her body. The bandages fit differently, she can make a fist with her hands. Her eyes are open wide and they are bright with life. It’s great to see. We hope this continues especially since we decreased her dosage of the diuretic she is on. The process of weaning her off the IV medications and transitioning her to oral medications has started in earnest. We want to get her out of the hospital on Monday after her second set of skin biopsies. In that endeavor, we are stopping the ativan altogether, and giving her one anti-nausea and one antibiotic through the gtube now. In speaking with the doctors, we all agreed to keep her on the fentanyl a little longer as we transition her medications as those may increase her discomfort a little. We’ll see. Rafi has a way of constantly surprising us.

We again left the indoors and ventured outside to the swings late in the afternoon. Sam and Marybeth Sheridan (Sam is patient #7) joined us at the swings. It was nice to spend the time with them. Sam really is an incredible young woman. She has fought this disease for years and this transplant with strength, dignity and grace that I hope Rafi will emulate.

As we come closer to the end of this leg of the journey, I realize how relieved I am. We are far from being out of the woods, and that fact was driven home yesterday when Zach passed away. He, by the way, did not suffer from EB. He was however, significantly further along the transplant road and our other neighbor is more than 240 days past her transplant day. It makes me realize that there are still battles to be fought, sleepless nights ahead of us, potential readmissions to the hospital as well. We still have blood pressure issues and the risk of graft versus host disease and infections from bacteria and viruses to guard against. There are probably a host of things I have even thought of yet. But still, I am relieved. Rafi has fought through weeks of 104+ fevers, blood blisters that wouldn’t drain, wounds over 90% of her body and still she smiles, she laughs, she plays. She has even started to eat. She loves her popsicles, baked beans and chicken soup. She is a warrior, and that gives me some comfort.

Until tomorrow…….

Two patterns are broken, finally. Rafi had a good night sleep with Jackie in the hospital last night and today was a very fun day for the little one. No longer is a good day followed by a terrible day. I am knocking on the fake wood right now as I type this. This morning Rafi walked, and even trotted, all over the hallways on the fifth floor. Then later in the afternoon, she rode a tricycle for the first time in her life. She loved it. Every nurse on the floor was telling me that now I have to get one for her.

Today was the first day without G-CSF and her numbers really dropped. It was an expected consequence and there is nothing to worry about, but it is amazing how well that drug works. Yesterday she had a WBC of 8.6 and an ANC of 6.5. Today she had a WBC of 4.3 and an ANC of 2.4. I am sure Rafi’s numbers will be lower tomorrow as well.

Rafi did eat a lot of food today. Early this afternoon she had a little peanut butter and jelly sandwich, a little macaroni and cheese and a decent amount of chicken soup. Then this evening she got to have her favorite, baked beans. She shoved them in her mouth so fast, it was like she was afraid we were going to take them away from her at any second. We are told that it is a great sign that she is eating so we will let her eat her little heart out.

Rafi has done really well through her stem cell transplant but it’s important to remember that this procedure is very serious and very dangerous. Earlier this morning our next door neighbor passed away. We are told he left this world very peacefully and didn’t feel any pain. We didn’t know him, and we only knew his mother from passing her in the hallways. No formal introductions ever happened, but you don’t need them on this floor. I saw Jackie’s face as she was talking to his Mom and I knew. We wish there was something we could do for them. I felt their pain, but I felt lucky at the same time. It’s the first time I have felt lucky regarding Rafi’s medical condition. I just wish it wasn’t at their expense. Zach, may you rest in peace and may your parents find peace and some comfort in the near future.

Until tomorrow………………

As expected we had a very good day. Her numbers jumped again and my math skills are not advanced enough to count that high. What this means is that her last dose of G-CSF was last night. It’s very exciting. Come tomorrow we are going to start transitioning Rafi from IV medications to oral medications. It will take a few days for all this to happen but it is a very important step for us to leave the hospital. It takes a few days because she gets a lot of medications (some every 4 hours) and her stomach would not be able to handle all of them at once. Metaphorically, it’s similar to training for a marathon. You wouldn’t be able to run one the first time you try.

What made today great was we unhooked Rafi from all her pumps and “tubies”, exited the hospital, smelled the fresh air, Rafi felt the wind on her face, and she got to go on the swing. She loves to sit in a swing and go back and forth. If it wasn’t for the mask, we would have seen a smile from ear to ear. She didn’t want to get off the swing but she was cold, even though wrapped in a blanket and in a sweater, so we had to go back inside. For a short time, I felt like a regular family who took their kid into the park on a weekend. It was nice to see Jackie and Rafi so happy. It was a refreshing feeling that I look forward to having again tomorrow. Hopefully soon, we will be able to get Rafi to a carousel where she can sit on a horse, a green horse of course. I’ll post the video of her on the swing in the next day or two.

She had her physical therapy as well and she was a champ. She walked all over the hallways, very quickly at that. I would throw things down the hall and she would go and fetch it. Dr. Wagner commented that she wasn’t a dog. It didn’t faze me in the slightest, Rafi was running around working those leg muscles. It was great to see.

Today in the New York Times there was an article about EB and the treatments being developed. You can see the article here: Rays of Hope in Battling An Agonizing Disease

They refer to the work here at the University of Minnesota and Dr. Wagner is quoted. I think it is funny that they call bone marrow transplants for this disease more controversial than gene correction. But I will let you all decide how to view it.

Patient #9 starts her chemotherapy at 4am tomorrow. You can go to her caringbridge.org site and read about her progress by searching for mckenziegray. We wish Kenzi and her family all the luck in the world as they start their journey. We’ll be knocking on their door tomorrow morning.

Until tomorrow……..

A discernible pattern has emerged these last couple of weeks. Whenever Jackie stays the night in the hospital, it’s a long night. She has certainly gotten the shorter end of the stick lately. Hopefully, she is sound asleep by now. As it turns out, the night was merely a portent. We had vomit, diarrhea, a slight fever and much crankiness. She retained a good amount of fluid over night so that didn’t help at all. First thing in the morning she was literally attached to me, then she was attached to Jackie. Then she just wasn’t feeling well all day. To top it all off, she had a reaction to the red blood cell transfusion. She started to sweat and throw up. That was fun.

We had to change the bandages on her trunk and the hickman line dressing as well. We’ve been giving her versed for that but today it just didn’t work as well as other days. She was quite uncooperative. When Rafi is uncooperative, she does damage. To herself of course. By the time she calmed down and stopped being just out of control, it was bed time. Indubitably, Rafi decided this was the time she had to watch Kung Fu Panda. Thankfully she was in a good mood. She became talkative and generally hilarious. She was cracking up the nursing staff and one of the residents came in to see her because she heard Rafi was in such a good mood.

During this time and out of nowhere, Rafi tells me she wants to ride a horse, a green horse, at the carousel. It took me awhile to figure out what she was saying but I finally understood. Yesterday we watched Mary Poppins and she loves the carousel part. As this conversation progressed, we decided to go outside tomorrow and go to the swings. She got very excited. Then we had the following conversation:
Rafi: “we go home then?”
Daddy: “we are home for right now”
Rafi: “we not home, we in the hospital. Why am I in the hospital?”

I thought, oh know how am I going to answer this in under a few minutes. I just wanted her to go to sleep. Fearing a long conversation was unfolding I figured I would just cut to the chase.

Daddy: “we are here to get you all better”
Rafi: “OK, you put medicine in my tubies”

I don’t care if it is pouring rain tomorrow, this kid is going the swings. I will go and buy a huge umbrella if necessary. Some of the things coming out of her mouth tonight were downright hilarious. I honestly can’t remember any of the things she said right now. I just know she had people laughing, and made Carrie (our middle of the evening nurse) a little amazed when Rafi asked for oxycodone and tylenol, by name, because her stomach hurt.

So we are hopeful that tomorrow is a better day. If there is an actual pattern, it will be a very fun day tomorrow with her. Oh yeah I almost forgot, she had a huge jump in numbers today again.

WBC = 5.4
ANC = 3.3

It’s quarter to twelve right now, and we just did the second vitals check in 10 minutes. Her blood pressure is still high (this after three different blood pressure medications) and she is getting her platelet infusion. So there is no real point to go to bed right now as I will have to be up in 45 minutes to take more vitals. So I will be tired tomorrow. Oh well.

Until tomorrow………..

Another day and another drive through the tunnels. Being that it is Saturday, the hospital hallways were empty so we took full advantage and sped around in Rafi’s convertible. She’s gotten to driving with one hand on the wheel with the other hand all to willing to hit the horn. She is definitely a New Yorker. Today was also the first day that she didn’t have a fever. We came close on a couple of occasions but we she didn’t break the threshold.

Rafi continues to do exceptionally well through this process. Her wounds are healing up very nicely as the ever important numbers continue to rise. Today she had a WBC of 3.9 and an ANC of 2.3. Rafaella was chatty and smiling a lot today. On the down side, she is still fighting with the nausea and the occasional vomiting but she seems to be accepting of the fact that she has to throw up sometimes. When she is clearly nauseas we tell her it’s OK to throw up as Mommy and Daddy do it all the time. At one point, I was in the bathroom running water and she asked if I was vomiting. Jackie told her I was and then Rafi asked me “You OK?” She also still has some pain and she tells us when and where it hurts. She knows which pump is the one that controls the fentanyl and has even pointed to it before when she needs a bolis. Our little drug addict is too cute.

Rafi is clearly on the mend and it is great to see. She ate some vanilla and chocolate ice cream today and we will start to give her some of her favorite foods, baked beans and yogurt, starting tomorrow. She is still about a kilo and a half above her admit weight but that will resolve itself at some point. I think some of her stomach pain is from the fluid retention and we were told by our nurse that Rafi’s body will ache and hurt as she creates all these new cells. But it is important to note that the amount of pain medication Rafi gets in her IV has been cut in half and she requires significantly less bumps on a daily basis. Tomorrow we are going to start to cycle her TPN (her nutrition that she receives through her hickman line). Instead of getting the TPN on a 24 hour basis, we will knock it down to 16 hours. Eventually she will only get it when she sleeps. At the same time, we are going to start to transition her IV medications to oral medications. In order for us to leave the hospital this has to happen. We are getting closer to home.

I am not looking forward to cleaning up the hospital room and bringing everything back to the apartment. We have amassed a ton of stuff there. Plus, we have to get our apartment cleaned like it was a crime scene. We’ll have the furniture cleaned, the rugs shampooed and while we are at it, the blinds and windows as well. Of course Jackie and I will have to clean up the apartment so that we can have a firm come in to clean the apartment.

Thank you to everyone for all the emails, text messages, voice mails, offers of food delivery, you name it. All the support has been wonderful and we appreciate it so very much. Until tomorrow…………

WBC = 2.7 ANC = 1.9

Poor Jackie had a long difficult night with Rafi. The little one was uncomfortable and vomited several times throughout the night. They had to give Rafi ativan and fentanyl to calm her down. The end result was that Jackie didn’t get to sleep at all. Before even hearing the details from Jackie when I got here, I surmised it was a difficult night based on the fact that I had received a text from Jackie at 5:30am that started off with “FYI” and ended with “she’s now sleeping on my chest”.

Adorned in her pink hat, pink crocs, blue hospital outfit and mask, Rafi climbed into her pink convertible beetle and we headed into the tunnels today. We got to leave unit 5D for the first time in the mid afternoon and cruised the hospital. When we finally got back to the room, Rafi said “that was fun”. So we promised her we would do it again tomorrow. I think I am going to run home tomorrow and bring back my computer speakers and affix them to the back of the pink car, hook them up to an ipod and blast some theme music. Jackie thinks Rafi would like Bob Marley but I think playing “The Joker” from Steve Miller would be hilarious. Rafi is most definitely a midnight talker.

A lot of people have asked now that the transplant is done, is she now considered cured. I wish we could say that. Let’s assume, in a best case scenario, that her new cells are producing normal levels of collagen VII. The theory goes that new cells migrate to areas of the body that need them most. So basically the body triages itself and sends the new cells to those areas first. Being that her body is just ramping up in the development of new cells, and other areas are still getting affected, there is a long list of places the new cells have to attend to and of course the body is shorthanded for the time being. So it will take time to attend to current wounds, ones that will occur, and to produce enough cells to do their everyday work of fighting infections and general bacteria and viruses that are ever present. The body needs time to produce enough cells to tackle that long laundry list and then create enough in excess to start loading collagen VII up that will eventually lead to the formation of the anchoring fibrils that she has lacked all her life. This is going to take time to work completely. Probably two to three years if everything goes according to a best case scenario. So far it has.

By the way, it was the film Lucas that I referenced in an earlier update. Just trying to keep you all on your toes. Jaime S was the first to get it. Congratulations, I would say you’ve dated yourself, but you are too young to know the damn movie. It must be the board games.

Until tomorrow…..

We never made it into the tunnels today. For every good day sometimes there is a less good day, if that makes sense. Rafi was just plain exhausted. She slept for 3 hours on my chest. She’s done that with Jackie on other days as well. Her body is working hard to create those new cells and sometimes a day full of excitement results in a another day full of nothing. It’s OK, it’s just part of the journey.

Her numbers climbed exponentially however. Rafi now has an ANC of 1.7 and a WBC of 2.8. It is pretty incredible how fast those numbers climb. G-CSF has certainly helped and she will stop receiving her daily infusions when her ANC reaches 2.5. We can expect her ANC to drop precipitously when that happens. Then the hope is that the ANC climbs again on its own. If it drops below 1, she will again receive the drug.

Those increased numbers have certainly helped her wounds. She is healing pretty quickly. We should be back to where we were before the chemotherapy started in about a week. Now if we can just get that excess kilo off her we would be all set. She has stayed pretty steady at 1.3 kilos above her admit weight.

Our overly educated and incredibly optimistic fellow is aiming to get us out of the hospital this coming Wednesday. Being that I am a betting man, I would put money down on a week from tomorrow. I am more than happy to develop a matrix and offer boxes if people are interested. Over/under perhaps?

Geri, I was not channeling the Lost Boys. Try again. The hint is in the spelling.

We will definitely get her out and about into the tunnels tomorrow. I am sure she’ll have more energy tomorrow, so in the end she will have more fun than if we pushed her today.

Until tomorrow…….

Twas a good day today, twas a very good day indeed. To really get the day started in the right direction, she had a huge jump in her numbers. Her white blood count came in at 2.1 and her absolute neutrophil count came in at .9. Today was the first day that there was no capital “L” next to her ANC number. Ten days ago there was an “LL” next to the number. Excuse the expression here, but that’s “some good shit”.

Rafi was animated, friendly, and enthusiastic today. She played a little basketball with her physical therapist, she interacted with her dedicated Care Partner, she put on the blue mask and went for a ride and a walk in the hallways of 5D. She was just cracking everyone up today. She actually said to me this afternoon “put medicine in my tubies, daddy”. She calls her IV lines “tubies”. To be honest, I am not sure I have heard her string so many different words together before today. She has always been a child with a strong verbal ability. A month ago the speech/feeding therapist here told us she was at a 3 year old level, and if you know Rafi that is not a surprising revelation. However, I think after not talking for almost a month, she jumped two years. In deference to the late Corey Haim, she may now suffer from lucaplakia (sp on purpose). Does anyone out there know the reference?

Tomorrow we get to meet our 4th attending. The attendings are the physicians responsible for the day to day care of Rafaella. We see them every morning during rounds and then in the room a little while later for a discussion. We started out with Dr. Jakub Tolar, then Dr. Margi MacMillan, then Dr. Paul Orchard, and tomorrow we break in Dr. Angela Smith. Jackie and I have had some experience in hospitals and in some places there is animosity between the nursing staff and the attending physicians. Not here. We’ve become friendly enough with the nurses where they would, and do, tell us their opinions on things and on people. What I can tell the families that follow us is that, without exception so far, the physicians are great and the nurses are wonderful.

We decreased Rafi’s pain medication by 20% today. While she had to get a couple bumps (one time infusions for acute discomfort) she required significantly less than previous days. She was nauseous a couple of times and she did retch once but again, today was significantly better than previous days. Clearly we are moving in the right direction. Dr. Wagner stopped by and we are all very interested to see the results of the skin biopsies (which will happen in about two weeks) and that will tell us the level of engraftment. Engraftment is when the donor stem cells start creating new blood cells that circulate through the body. With a cord blood transplant, the average time to engraftment (3 days of over .5 ANC) is 21 – 35 days. On day +19, today, Rafi had her third day of her ANC being over .5. She generally excels at things. Anyway, the results of the skin biopsies will tell us how well the engraftment process is going. Is she producing collagen VII, the one she was previously missing? Are her new cells migrating to the skin? We will find out.

Tomorrow is going to be a good day. We will hopefully be able to leave the 5th floor and explore the tunnels below the hospital with Rafi. She loves going into the tunnels. First and foremost, she loves saying the word. The floors undulate and she loves walking up and down the hallways. She has made passing people laugh before when she says “we are going down, weeeeeee”. It would make her very happy to spend some time in her little pink beetle in the tunnels. Dr. Wagner has given us the provisional thumbs up. I am looking forward to seeing the numbers, cruising the tunnels and grinning from ear to ear.

Until tomorrow…………………

After 28 days in the hospital we left the room for the first time. We got Rafi to put on the blue mask she will be required to wear for some time when out in public and we put her into a radio flyer with wheels and cruised around the hallways of 5D. Just getting her to wear the mask was difficult, but she continually surprises us. Previously, she had only held the mask up to her face for five to ten seconds at a time and never let us put it on her. So we made a deal with her. We put a hat on her and told her she could wear her sunglasses if she wore the same mask, the blue mask, that Jackie and I were wearing. After a little negotiating, and Jackie’s work on the mask, Rafi let us put it on her, and we left the room.

Today we started to wean her off some of the drugs she routinely takes. We stopped her Zofran (anti-nausea) drip and decreased her pain medication dosage. There is a lot to accomplish in what seems like an incredibly short period of time but we’ll get there in due time. Rafi will determine how fast we reach these new milestones, and I have a feeling that she will continue to surprise and amaze us.

She did have some bouts of nausea today and she was a little uncomfortable at times. At one point her temperature rose to 101, but that is well within Tylenol’s ability to handle. She did drink some water and asked for her Dum-Dums and popsicles. She has really done so well through this procedure. She is once again talking a lot, and surprising us with what comes out of her mouth. We are far from where we were before we started, and far from healed, but Rafi is getting there, little by little.

Today her white blood count was 1.3 and her absolute neutrophil count was .5. I am looking forward to seeing the numbers tomorrow. But most of all, I am looking forward to the time when Jackie and I can say to Rafi, “want to go for a car ride? Let’s go home”.

Until tomorrow………

Dr. Wagner came in to see us late in the afternoon today to tell us that he had gotten several emails from EB families commenting on how well Rafi was doing. Of course he knew all her numbers and the ins and outs of her progress to date, that was a given. He just wanted to drop in to see how things were going. I think he feels the need to show his face and reassure us (and all the other BMT families for that matter) that he is on top of the case. He doesn’t need to, there are a plethora of people watching over Rafi, but he does anyway. However, I really think he came to see us because earlier in the morning during rounds with the resident, the fellow, the nurses, the pharmacist and nutritionist, the attending, Dr. Orchard, told Jackie they thought there was a possibility Rafaella could be discharged within a week.

Within a week? Really? That would be amazing. Both Jackie and I just smiled. It would be so nice to be at our apartment here instead of separated every night. We could eat better. I can’t emphasize enough how fresh lettuce and spinach with chick peas, corn, black beans, broccoli, red peppers, celery, cucumbers, black olives, artichoke hearts and beets with some olive oil and balsamic vinegar is going to taste. I am going to have to eat that way for the next month once we are out of here. I am sure my cholesterol and blood pressure are at all time highs at this point. While the cafeteria here isn’t bad for hospital food, it is still hospital food. Plus, it is obvious that all our well intentioned friends who send us delicious cookies and brownies, really want to put us into the cardiac wing. I am a “cookieholic”, a cookie monster if you will, but after this I am not sure I am going to be able to eat another one for awhile.

I think within a week is a little over optimistic on their part, but I like the way they are thinking. Rafi has a lot to accomplish before discharge can happen. First and foremost, she has to be afebrile (without fever) for three days. Today was probably the first day where she didn’t have a fever for two hours. Then of course, she spikes a 103.3 fever. That’s 1.6 points lower than before, so at least we are heading in the right direction. Rafi needs to be weaned off the IV antibiotics and must be able to tolerate them through her gtube. She also needs to be weaned off the fentanyl. That’s plenty of milestones to hit for now.

Did I mention that her WBC is 1.0 and her ANC is .5?

Did I mention that her numerous wounds are looking better? It’s amazing what some white cells can do.

It was also a nice day because, for a good extended period of time, Rafi was a lot like her old self. She was chatty, she was smiling, and she wanted to play. The last time she spoke this much was about a month ago. Thanks to the speech and feeding therapist, her favorite thing is now Dum-Dum lollipops. So when I came back into the room this afternoon, after a trip to Walmart to get her the Dum-Dums, and after I put the bag of 300 away in the closet I hear, “Daddy, where are my Dum-Dums?” Now this is funny because she has only heard them called that once. It’s days like today when I wish I could have the video camera going all day.

Until tomorrow……

WBC = .9
ANC = .4

With the exception of the bandage change, it was a pretty good day. Her fever was down and for awhile we even had a normal reading. Granted, she was on Celebrex and Tylenol at the time, but we’ll take it. Nothing is too small to be overlooked or appreciated. She was even on her feet and walking around a little today. We did increase her pain medication dosage and that seems to have made a difference as well. I know it sounds like we increase the fentanyl frequently, and we do, but right now the best thing is to keep her as comfortable as possible. She is still battling the mucusitis and we hope the increasing white counts are going to help. We know they will but it does take some time. Just when we thought we had turned a corner, with regards to the mucusitis, it proves to still be an issue. We can see the effect it has on her mouth and we can only assume what it is like internally. We do know it is an issue because there is some blood when we vent the gtube and when she vomits. The biggest indicator is that Rafi complains about her throat and stomach at times. This does happen with all blood and marrow transplant kids, and unfortunately all you can do is wait it out. Her counts are coming in quickly and we know that will make a world of difference in the coming days.

She is starting to heal. Some of the wounds on the back of her thighs and on her face are looking better. It will take some time for them to be completely gone but just as important, the rate of new wounds seems to be decreasing. She is still very itchy but the bouts of serious pruritis seem to be less intense and of shorter duration. Without any real scientific evidence, I believe we can say that we see a small change even this early in the game. We haven’t made it back to baseline yet but we are headed in that direction. Again, nothing is to small to be overlooked or appreciated.

Thankfully, we’ve added another movie into her repertoire at this point. Shrek is still her out and out favorite, but she now allows me to watch Kung Fu Panda with her as well. Thank goodness for that, because Shrek was only funny the first 2,000 times. Every once in a while I can sneak in the Muppet Movie as well and she seems to really get a kick out of Fozzy Bear. I forgot how many cameo appearances there are in that movie, so for now it is entertaining.

That’s it for now. Until tomorrow…….

I know you want to see the WBC and ANC numbers. I understand. After they draw the blood at 4am for the CBC and differentials (for the one or two people who haven’t seen an episode of ER, Grey’s Anatomy, or St. Elsewhere for that matter – and I am pretty sure General Hospital never mentioned a real test of any kind – CBC stands for complete blood count) I am chomping at the bit waiting for those numbers to come back. I want them the second the results are posted. On the couple of occasions when we’ve gotten the CBCs but not the differentials (that’s where you see the ANC) and they needed to draw more blood and send it to the lab, we’ve climbed the walls waiting. So, I’ll tell you the numbers…………..without making you wait too much longer.

Rafaella started to develop a cough a couple of days ago. We thought itchy throat, a little mucus build up, no big deal. But the cough has gotten progressively worse, more frequent and having a “wetter” quality. The coughing usually precipitated a vomiting session. That triggered our third visit to CT for a scan of her sinuses and her chest. Being the weekend, the pediatric imaging was closed so we had to go to the adult room. In the pediatric room, they project a “Nemo like” fish tank onto the inner walls of the CT machine for the kids to look at and enjoy. They also have other toys for distraction. All of this helps tremendously. Needless to say they don’t do this in the adult room. Turns out we were worried for no reason. Even though Rafi was visibly nervous, she held still and allowed the techs to take pictures of both areas. It takes a special person to be able to disregard their fear of a situation and carry on.

The days are mixed at this point. There are highs and there are lows. She is still febrile and needs to be held and then she wants to paint. The swings are immediate, there is no transition period. The nights unfortunately are long. Jackie usually gets the worse of them, but not tonight. Every twenty minutes can’t come fast enough. I am glad that I slept earlier in the evening because it is now 3:30am and I’ve been up with her for the past 3 hours. She is very uncomfortable and has been passing stool and urine frequently. I am assuming she is cramping quite a bit based on where she tells me it hurts and how her body is convulsing. Every twenty minutes she can have another additional dose of the fentanyl. She had her last bump 25 minutes ago.

I just found out that she needs more platelets tonight. That makes three times in 24 hours. They’ve increased the threshold at which it triggers a transfusion. Basically, it means she gets more transfusions and in addition to normal vital checks, you have 3 more to do, at the beginning of the transfusion, after 10 minutes and then after an hour. We think the increased platelets has helped with those blood blisters that didn’t drain. Since we increased the platelet threshold she hasn’t had one of the non draining blisters. So we got that going for us.

The results from the CT scan all came back negative so that’s great news. I think it is enough of the American Idol waiting game for you. Her WBC was .7 and her ANC was .3.

I have to go and do some vitals now, then again in 10 minutes and again in an hour. I am going to try and get some sleep for 45 minutes or so while I can. Until next time.

White blood count = .6
Absolute Neutrophil Count = .2

Isn’t that a nice way to start off an update? The numbers are climbing and we couldn’t be happier about it. Now we just want those new cells to do their respective jobs and jump start the healing process. There is a good amount of healing to do and we can’t wait to start seeing the benefits of those climbing numbers. How nice it will be when we are able to just put her in a bathtub and let her play with some silly toys. We can’t wait to see her splash around and make a complete and utter mess. Maybe one day soon (a year or two perhaps) we will be able to see that little wish come true.

For every step forward there is going to be some backwards motion. We expect that and today was no different from our expectations. She is still running pretty high fevers and retaining a good amount of fluids. Right now Rafi is approximately two and a half pounds heavier than she was when we were admitted. You can tell how bloated she is pretty easily but the real test is how fast your arms fall asleep when you hold her for long periods of time. When those fevers spike, she wants and needs to be held. And held, and held, and held. During those times at least you can say that you have a hot compress keeping those muscles relaxed.

There are probably several reasons why she continues to have these fevers. She could have an infection somewhere and her body is responding the way it should except there are not enough white cells to kill the infection. They could be due to the amount of inflammation in her body. It’s hard work for the body to produce all these new cells and that could cause the fevers. They could also be the side effect of all the drugs she is on. Or it can be “E”, all of the above, or “F” for that matter, none of the above. Right now nobody is overly concerned about the fact Rafi has fevers, only that they tend to be very high. Tylenol with Celebrex and an icepack usually do the trick over time.

I say that there is some backwards motion because today she wasn’t very active. She just didn’t feel well and decided to play that “I’m sick card”. For good reason mind you. But she is an impressive fighter and after I left the hospital this evening, she apparently woke up in a great mood. Jackie even got her to try on the mask she will have to wear when we leave the hospital. This was a huge accomplishment. Rafi even asked to brush her teeth. That’s another huge step forward. Before the transplant, Rafi loved to brush her teeth. Actually, she loved holding the tooth brush and sucking the strawberry flavored tooth paste from the bristles and then dunking the brush into a cup of water and making a mess. She did brush a little but that was only a means to get the strawberry flavor. So tonight, she asked to brush her teeth and I can’t help but crack a smile.

There is another small issue arising now. Up to this point, she has been really great about getting her blood pressure, and her vitals in general, taken. We would ask her which arm she wanted to use for the “arm hug”. She was getting very used to it and a real champ about getting it done. However, now it hurts her arm. The amount of times she gets her blood pressure taken would be enough to make any arm kind of sore. So we started on the calf. Now that hurts. I guess we’ll figure something out. Also, even though we coat the thermometer with aquafor, it is starting to leave a mark under her arms. Eventually she’ll build up some scar tissue under there and we are concerned that the temperature readings will no longer be accurate. As it is now, each underarm will give us different temperatures. One will show a reading of 2 degrees less than the other. The small things are what get you in the end.

It’s ten o’clock now and I still have to do some work. Thank you to everyone for all the encouragement and support. It has been more helpful than I can put words to. Still, and it’s important to understand, the best way to help Rafaella and all the other people who suffer from Epidermolysis Bullosa, and those that will suffer from this horrible disorder, is to spread the word about it. Let’s increase the institutional knowledge about EB. Let’s pressure our politicians to force insurance companies to pay for the bandages that these people need. There are insurance companies that put a $1,500 yearly cap on these special bandages. We use about $5,000 a month. Spread the word. Remember, motion creates action. Action creates funding and funding creates services for those that need them and helps to create treatments and hopefully a cure. Jackie, Rafaella and I are lucky. I’ve always believed that luck is the intersection of preparation and opportunity. But, I have come to learn that there are other factors at play. If we spread the word about EB we can increase those other factors as well as the opportunities that exist. Now go to it.

Tomorrow is March 6 and it is Jackie’s and my anniversary. We’ve been married for six years now. There is no way I could have survived these past two years without her. While we don’t cherish the idea of spending the day in a hospital, we are really looking forward to a year of health and happiness.

Rafi was up and walking around today. Actually she was cruising, for almost an hour. It doesn’t sound like much, but when you’ve been running 104.9 fevers for a week straight, it’s pretty amazing. She painted with water colors, drank water and apple juice from her special straw as well. We even coaxed some laughs from her. Overall it was a good day. OK, based on the fact that her WBC was .5 and her ANC was .1, that made it a great day. I am looking forward to hearing tomorrow’s report.

But she also suffered from some fevers and she threw up twice. She has been in some pain and her pain medication, fentanyl, was increased yet again to provide more comfort. Her blood pressure has been running a little high, so they increased her amlodipine a little. Amodipine relaxes the smooth muscle in the arterial wall and thus lowers blood pressure. It takes a little time to get used to all the drugs that are coming in and out of this room. Currently, Rafi is on 17 drugs (19 if you count TPN and lipids). I can’t wait to see all the explanation of benefit statements from the insurance company undoubtedly coming my way. There are antifungals, antacid, anti-virals, blood pressure, antibiotics, immunosuppressants, growth factors, anti-itch, pain medications, anti-nausea and liver drugs. Better living through chemistry. You have to wonder, much like you need a vacation when you get back from a vacation, is she going to need drugs to get off of these drugs? For every action there is an equal and opposite…….

Until tomorrow…………

By the time I was able to write an update last night, I was just too tired to write. In general, yesterday was a good day with a few exceptions. She started to smile more and even let out a small laugh or two. I attribute that to her white blood cell count climbing to .3. This morning I realized that I need to clarify a small fact. In order for Rafi to leave the room her ANC count (absolute neutrophil count) needs to be .5, not her white blood count. The ANC is a measure of a specific type of white blood cell that is responsible for fighting infection. For all intents and purposes it is the immune system. Once her WBC reaches .5, then they start measuring the ANC.

As luck would have it, on Day +13, Rafi had a WBC of .5 and an ANC of .1.

For now there just isn’t anything else to say. I will update again this evening.

To the surprise of Dr. Orchard (but not to the MD, PhD Fellow, Heather), Rafi’s WBC was .2 today. He was betting that the number would go back down to <.1. I like conservative doctors, so I had no problem with him not wanting us to get our hopes up. But La Di Da, take a look at that, 100% growth. Come on new cells, come out come out wherever you are, you’ve got lots of work to do.

There sure is a lot of work to do. Rafi threw up on three occasions today. She hates to vomit and will fight and fight with herself not to do it. This evening, she was saying “NO!” as I was getting her into position (on her side or face down) as quickly as I could before the inevitable. Heave number one ends and Rafi says, “I’m done” followed by heave number two, “I’m done”, and finally heave number three and this time she was right. Asleep on my shoulder three minutes later. Hopefully she stays asleep for awhile, she needs the downtime now.

Even though those white cells are starting to come in (can I get a HOORAY!) she continues to struggle through this. Her fevers just won’t let up, she continues to retain fluids, her skin is more affected and today she was unconscious more than she was conscious. This is the time in transplant that the kids do sleep a lot more and I am thankful for that. We increased her dose of fentanyl because we all think she is pretty uncomfortable. Hopefully that will help in her, and my, quest for rest.

Come on white cells, you got work to do. I can’t wait to have my child back. For right now, she is a shell of her former self. I want to see her run around the apartment and laugh the way she does in the Ball Player video. I want her bloating to subside so I can take a good luck at her face and not see a child who just went 5 rounds with Ali. I miss her voice. She doesn’t speak much and when she does it’s one or two words spoken very softly. I miss her laugh. I am glad we got a video camera, cause I can watch the videos over and over again and hear her laugh whenever I want. Although I appreciate them, I want her off the pain medications. I know all of this will happen soon enough. Rafi, version 2, coming into my home in the early spring of 2010. The one million dollar girl. I know, I am being a little impatient.

Patient # 9, McKensie Grey, is now in Minneapolis and had her hickman line placed today. I believe that she is scheduled to become an inpatient on our floor on the 12th of March. She will have a site on Caring Bridge, so please feel free to search the site for her story. We could end up having three EB kids here at the same time, as I know Sam Sheridan is heading back up here next week as well. We are going to have a big EB party. Please no stickers…….

We are in double digits. Ten days after transplant and twenty days in the hospital. Rafi’s white blood count is still at .1. Not less than .1, so we are still smiling. On top of all the other issues, we have to deal with these large blood blisters that just won’t drain. It’s frustrating because we know they can’t be very comfortable for Rafi. In trying to drain these new blisters, I have punctured them repeatedly with a large needle (18 gauge) to no avail. I have used a scalpel on one to no avail. I even used surgical scissors to no avail. We even tried warm water compresses for more than half and hour after cutting the blister open, to no avail. Everyday is something new so hopefully in the next day or two they will resolve themselves. At least her white cell count isn’t <.1.

She is still running fevers but Tylenol with Celebrex seem to lower them after a time. The mucusitis in her mouth seems to have gotten a little better, but we know it is still in the rest of the GI tract. All of a sudden her voice will get hoarse and she’ll want either Jackie or I to pick her up, and she’ll say she’s scared. That’s when we know to call the nurse for a bump in her fentanyl. We were hoping that we had possibly turned a little corner today with regards to the mucusitis, considering she was talking more and being a little more animated. Plus, her mouth looked a little better today. We could actually see her teeth. Then, of course, she threw up some blood earlier this evening and we knew we were a little premature in our thinking. Tomorrow is another day and hopefully Rafi will feel a little better for longer periods of time. It’s a marathon, not a sprint and for every step forward there could be half a step back so we remain hopeful.

Bandage changes still are a real issue and we are trying new things all the time. We tried using Versed before the bandage change yesterday. She had it once before and I was ready to buy stock in the company that makes it after I saw how effective it was. It was hilarious. That was then and this is now. Rafi was in her Versed happy place until we tried to start the bandage change. We ended up being forced to give her some ativan to calm her down and when she fell asleep we were able to wash each foot and put on new bandages. We discussed her reactions to the bath and bandage changes in rounds this morning and Dr. Orchard thinks that the minute she feels the drugs taking effect she anticipates a procedure and goes off the farm. Sometimes we forget she is still two years old and can be difficult. Now we are going to try doing a bandage change over an entire day without the sedatives and see if that helps. Send us your positive vibes, we’ll need them.

The days in the hospital go by without any downtime. There is always something to do in the care for Rafi. Diaper changes alone have become difficult and require more pain medication bumps. We are constantly cutting bandages to replace the ones that get covered in urine or feces, or what passes as feces now a days. Rafi has a way of waiting until we are finished with a diaper change before springing the need to start all over again on us. I swear it feels like 5 hours a day are allocated to getting ready for a diaper change and then actually engaging in the change. The lasix certainly doesn’t help in that regard. Rafi was ready to be toilet trained before this started, so I can’t wait to get back to that point.

Part of the problem is that the back of her thighs are a total mess. Those wounds would be enough to cripple any one of us. She is significantly more affected by the EB wounds these days than ever before. We expected this and the doctors forewarned us as well. While we know they bother her even through the constant dose of fentanyl, I can’t help but admire her strength. One of the side effects of the pain medications is itchiness and it really hits her hard. We’ve taught her to tap herself instead of scratching herself but sometimes she just can’t help it. In general, she listens and suffers through the itchiness like a champ. To really understand what she goes through, the next time you get an itch, don’t scratch it, no matter what. Give it a try and see if you can do it.

People ask me if I find writing this cathartic at all. Sometimes it is but the most rewarding part is our morning ritual. Jackie and I have our coffee together and I read her what I wrote the night before and then we read the comments people leave and the emails people send. It’s nice. Knowing that Rafi’s journey touches so many people and reading what people write brings us together as a family. It’s a little oasis in the desert for a short time.

My cousin, Emilie, had her bat mitzvah this weekend and we wish we could have been there. Emilie and her sister, Charlie, were flower girls at our wedding. The bat mitzvah is a big step into adulthood and we are proud of her. Jackie and I want her to know that we really appreciate her taking the opportunity to light a candle and to say a prayer for Rafi. Congratulations and welcome to a larger world. We love you and we miss you.

Our nephew, Nathaniel, also took a step into a larger world recently. He broke his first bone. Welcome to the club. I think I was his age when I was in my first cast. I am sorry we missed your birthday, but we will see you soon. We love you and heed some advice from your uncle, milk the cast for all it is worth.

Always a little drama in our household, so why would today be any different. As I told everyone in an earlier update, we started to track WBC (white blood counts) at day +7. We weren’t expecting anything on that day and not really expecting anything for a few more days. The first real milestone is to have a reading of .5. At that level we can leave the room and wander the hallways, as long as there is no fever. On days +7 and +8 our WBC was <.1. Today there was no < sign. That’s my first milestone. It really doesn’t mean anything yet, but hell, it made me smile. I guess it could mean that the cells are starting to grow. It may not mean that, but I choose to believe they are.

Things started off great. Except for the fact that her Gtube fell out again. For the second time, it just plain fell out. Not sure why the balloon deflated less than 10 days after it was reinserted and inflated. But it did. We decided the mickey button was defective and got a new one. We had to move quickly before the hole in her stomach started to close up. We had about half and hour. About ten minutes later we had the new gtube and one of our nurses attempted to put it back in. There doesn’t seem to be much science to it. Find hole, grease up the balloon and push. Lindsay has had a lot of experience with this and she is a good nurse so we said, sure, go for it. After a couple of attempts, I told Lindsay there was no way Jackie and I wanted Rafi to go into surgery so put some elbow grease into it. We wanted to avoid surgery for the obvious reasons but also because she has mucusitis and intubating her could really cause some damage, some more inflammation and I didn’t want any bacteria to have an opportunity to get down there. In the end, Lindsay was successful.

Of course, we were going to do a bath and bandage change right at that time so we had to postpone it. We could have moved forward, but Rafi had already undergone one traumatic experience. So we waited. We gave her Versed (the great drug she got before surgery – the one with the street name “the amazin”) and once again the sedative/anti-anxiety drug did not work. It was an exercise in futility. But we really had to wash those legs and feet. So we waited until she fell asleep. Then we waited until she was very asleep. Then we procrastinated. Finally we gave one foot a try. She was asleep until the bleach/soapy water hit her foot. She just whined and was clearly uncomfortable. We got through it. Then waited after we finished the first calf and foot for her to fall back to sleep to repeat the process. We finally did it without causing damage or tears.

Rafi continues to do well overall from an internal organ/general health perspective. Her skin is really taking a beating though. She now gets pretty big blood blisters that don’t want to drain no matter how many holes I put in them with an 18 gauge needle. That is not a thin needle mind you. I had to use a scalpel on a blister on the back of her neck. We had to explain to the fellow who was trying to talk me out of using it, what we do in EB. That blister came out of nowhere and grew so quickly and if I didn’t do anything it would have taken over the entire back of her neck. I had punctured it in several places with a large needle and the damn thing would not drain. This is counterintuitive to me. With Rafi’s low platelet count, I thought that a blood blister would have to drain easily given the numerical impossibility Guess not.

Let’s hope her WBC continues to climb. Please send some really strong positive vibes/thoughts/prayers to samantha sheridan. She is EB patient #7 and she is still have a rough go of it and a pick me up is really needed there.

Those of us that deal with EB on a daily basis get accustomed to the fact that there are times when our kids are in pain. It is a terrible fact of life with EB. There is a term in psychology for it, learned helplessness. Sometimes it can be overwhelming, other times it just makes you mad. But you carry on and you take your next breath and feel your heart beating strongly against the bottom of your throat and you try your hardest to not let your kid see you in pain. It’s important to do that. To take your next breath. To look forward and fight through the learned helplessness. To instill the thought that the pain doesn’t have to own you, that you can live past it, live through it, and hopefully one day, live without it.

Today was a difficult day. Earlier in the morning, without any real warning, and while she was still asleep, Rafi threw up. Even though she was on her side we were worried that she aspirated and had some fluid in her lungs. She had some signs of respiratory distress, flared nostrils, breathing from the stomach and a slight “raspiness” in her breaths. Our nurse agreed and in came the portable x-ray machine. Everything turned out fine but there is some fluid in those lungs, but apparently nothing to be worried about. Jackie figured out that we inadvertently did not vent her gtube overnight. We had the extension wrapped in an infant diaper (to catch the excretions, but I didn’t take the cap off the extension). So what we think happened was that fluid built up and after almost 11 hours of sleep enough accumulated and made Rafi sick. On top of that, Rafi is now retaining a good amount of fluid so that didn’t help her breathing. She’s gained about a pound a half so far. It’s our hope that Rafi will respond to the additional doses of lasix and get rid of the weight.

The mucositis just keeps getting a little worse and it is clear that, at times, she is uncomfortable. We are giving her more bumps of the fentanyl to try and keep her as pain free as we can. There are times that she fights through it, raises her head with a smile or gets excited about something. She likes to look for Shrek in our DVD case and for a brief second of two, when she eventually finds it and says “ooo, Shrek, I found it” it’s like we are back home and she is her normal self.

Then the realities of the stem cell transplant take over. She is still running very high fevers, she is bloated to the point of not really looking like herself, and she is drugged enough to not being completely in the moment. I am fine with that last part though. It’s a fine line in keeping her drugged enough to not feel pain, to not compromise her respirations, and to keep her aware of the world around her. We are in the truly difficult time of transplant so for the next two weeks if she is not aware that it is Sunday or that it is sunny outside, so be it.

It’s now Sunday morning, I fell asleep typing this last night, so now I am back at the hospital just finishing a bagel and coffee with Jackie. It was a long night for the two of them. At some point in the night, they started blowing humidified air by her to hopefully clear up some of the mucus in her airway. There are also doing more cultures for influenza A and H1N1 to see if we can figure out why she is still running these consistent and high fevers.

Off to start the day, a new day, and hopefully a better one.

Apparently, Rafi has a pretty bad case of mucositis. The doctors, nurses, residents, and fellows all say so. We know it’s there, it’s obvious, but I don’t know how much worse it gets, or if it gets worse, at this point. I really have to be more diligent about putting the caphosol in her mouth. Nobody is really able to discuss severity of her case with me because it affects kids differently, with various levels of severity, depending on a million factors. They’ll look at her mouth and you can see it in their eyes and they say things like, “How’s her pain level now?” or “ooooh yeah, I see the mucositis has set in.” Here’s what I do know for sure, she is handling it very well. Yes she gets IV pain medications round the clock, but her strength and toughness is evident. Our nurse tonight was very surprised that Rafi was still putting food into her mouth, let alone dancing on the bed this evening. Rafi was so funny as some 70′s disco music was playing in the background. She was holding onto the hand rails of her crib and dancing up a storm. I am not sure i have seen her dance so well before. We can now add she has rhythm to her long list of traits. We were laughing and Rafi was just enjoying herself. It was great.

I took some video of the dancing but I am not sure I am going to post it. Rafi is pretty beat up right now. In other words, the EB has really decided to rear its ugly head and leave it’s mark on her forehead, face and neck. I knew she was beat up, aka has more of her body affected, it’s plain to see, but I really didn’t know how bad until I started watching some of the videos we have posted on the site. It’s a marked difference. Her skin really has taken a beating lately. She has always had a good amount of superficial wounds but now areas that haven’t been affected lately, are. Thank goodness she is on the pain medication.

We actually had part of the day when she didn’t have a fever. We gave her Tylenol and Celebrex and that seemed to do the trick. We also increased the amount of fentanyl she gets on an hourly basis as well as the amount we give her when she needs a short term bump. She also is getting a blood pressure medicine added to her list of dailies. Time to head off to sleep.

Today was a good day. Jackie is all better and back by Rafi’s side. I am sitting at home sipping a 21 year old single malt scotch, compliments of a buddy from New York. A dear friend from Boston sent us a great care package of cookies, hand lotion and a little pink dog for Rafi that their young son picked out. A few days ago we got a great care package from the mothers in Rafi’s play group at home. They sent pictures of all her friends and the requisite cookies. The pictures are up on the wall so Rafi can see them whenever she wants. Another dear friend sent us a collage that is also up on the wall. It’s nice to have all the pictures up because we can make the hospital room a bit more homey. But most important, Rafi is quite happy because her best friend, Emily, Jackie’s sister, is in town for the week. When Emily is around the pecking order is Emily, Jackie and then me. My parents were here over the weekend and Jackie’s dad is coming this weekend. It’s nice to have the family around.

Due to Rafi’s consistent high fevers, we had to have a CT scan this morning to see if there were any infections that the daily blood tests didn’t catch. This can be quite scary for an adult let alone a 2 year old, but Rafi handled it like a champ. She was a little scared but didn’t fight at all and stayed completely still. Jackie and I were very proud of her. The results came back negative. The cultures from the nasal swab also came back negative. So for the moment, everything is right in the world. She is retaining some fluid but the lasix is really taking care of that.

Rafi was more active today than she has been in a week. I attribute this to Emily. Rafi was talkative, playful and smiling. She even played a little golf with the physical therapist. But the thing I will remember most from today is that Rafi entered a larger world when she ate her first lollipop. Mucositis be damned, the lollipop wins.

Tomorrow we start looking at her ANC counts. ANC is the acronym for Absolute Neutrophil Count and it is a measure of the number of white blood cells that are neutrophils, which are the key components of a healthy immune system. A normal range is 1,500 – 8,000 (1.5 – 8.0). The first real milestone is to get her to a count of 500. Once we reach that number for a couple of days without a fever, we can leave the room and roam the hallway. That will be nice for Rafi. So let’s hope those neutrophils come in quickly.

I am going to sign off now and watch a little television, finish the laundry and then get some much needed sleep. Please don’t hesitate to ask us any question you want us to address.

It’s hard to imagine that we’ve been in this hospital room for 15 days already. Actually, I take that back, it’s easy to imagine, the clock on the wall can run backwards at times. Especially considering, I’ve watched Shrek at least 200 times, Bob the Builder and Pooh Bear more times than I can count, and let’s not even mention the little red fuzzy monster. Conventional wisdom states that we are now in the really difficult phase of the transplant. I am not sure what is worse, the current manifestations on my daughter, the anticipation of things yet to come or the things yet to come. What I do know is that it is exhausting and that’s how I know it’s been 15 days.

For all intents and purposes, Rafi is doing exceptionally well. She is a sick little girl, but, honestly, it could be a lot worse. Her internal organs are in great shape, her numbers are all within acceptable limits, she breathes
well, her coloring is good. She’s been running fevers above 104 daily and that takes it’s toll on her body. Her skin is taking a beating and that takes a toll on us. At this point the nature of a blister is changing so we are trying to figure out the best way to address each new one. For instance, the blisters now tend to be blood blisters that just don’t drain. I find that counter-intuitive given that her platelet count is so low. I would assume that they drain faster and more easily with smaller holes. It is what it is. The problem is that the blisters can just get that much bigger because of the thicker, heavier fluid build up. It is what it is. One of the worst things now is she is more itchy than we’ve ever experienced. That means more self inflicted damage. Dr. Wagner tells us that the itchiness is one of the first things to dissipate. That will be a wonderful milestone.

Other than that, Rafi has had a platelet infusion and is getting her second one now. She as also given more blood earlier because her hemoglobin was low. Our new nightly ritual starts at 12:30 am, when she wakes up with a very hoarse cry. I have to change her clothing and some bandages at this point, because they’ve given her lasix to make sure she doesn’t retain too much fluid, and well, diapers without the elastic borders are a tad useless. The mucositis has had time to build up so she is in a little pain and this scares her. Add to those facts that she is once again running a fever, due for Tylenol, and it all makes for a perfect storm. I feel bad for her and we try to talk it out. We try to breathe through it, deep breaths in and slow breaths out and I bounce around the room a little. So, after a little ativan and a bump in fentanyl, and about 45 minutes on my shoulder, she’s back to sleep.

Jackie came down with a touch of food poisoning so I’ve been in the hospital for the past three nights. I swear I think it is harder for her to not be in the hospital than to be here. The three nights here alone just makes me respect my predecessors all the more. How you all had the strength and fortitude to live with no sleep and deal with the stress by yourselves for such extended periods amazes me. It makes me proud of not only the EB kids, and I am very proud of them, but also of you. I know I’ve said this before but your strength, courage and determination is inspirational. I am lucky Jackie is here and that she is as strong as she is.

Here’s a small world story for you before I finally lay my head down on a pillow. My cousin, who is a junior in college, interviewed a young woman for what I think is a student leadership council position. On this woman’s application, she wrote that she had a rare disorder. My cousin, immediately knew the look of EB, and emailed her after the interviews. Jackie, my cousin, told her the story of Rafi, and asked if she also had EB. Turns out this young woman has EB, has a father that sits on the board of DEBRA with me, and was willing to talk to our family about the ways to lead a normal life with EB. Pretty amazing.

A day with no bad news is a good day for sure. The day started out pretty rough mostly because Rafi and I didn’t get much sleep and then she had a moderately violent vomiting episode. Being that we stopped her gtube feeds the night before she didn’t have anything to throw up except some mucous, so that was fun. Of course this happened as I was changing a diaper and the back of her thighs are pretty beat up. She was thrashing about as I was trying to do two things. First, I was attempting to keep her on her side or her stomach so she didn’t aspirate anything and at the same time trying not to do more damage to her lower half. Her skin is breaking down, so she has more wounds that aren’t healing which is no surprise as she has no white cells to help in that process. Thankfully, I kept her on her side and I didn’t do any further damage. I honestly have no idea how that all worked out especially considering I didn’t have a cup of coffee in me yet.

Following that fun joy ride, Rafi had a nose swab to check for a couple of viruses, like influenza. We did not like the idea of doing this to the little one. However, she’s had some pretty high fevers that are consistent and the doctors felt we needed to rule everything out. So we gave her some ativan to calm her down so she wouldn’t jerk around and potentially hurt herself. In hindsight, we probably didn’t need the drug but you can’t be too safe. I put her on my lap, and cradled her head to limit her movement. The swab itself took two seconds and she understandably got pretty upset, but Jackie was able to calm her down after ten minutes. While we are still waiting on the actual culture, which could take days, we got back results that came back negative.

Because we gave Rafi the ativan we decided to postpone the bandage change until later in the afternoon. We wanted to avoid giving her too much ativan within a short period of time and we wanted to have the Tylenol within it’s peak so we had to wait. We paid for that miscalculation as it proved to be a difficult bandage change. Based on her reaction to the soapy, saline-like water solution, we believe her skin is just more sensitive after the chemotherapy. We gave her a bump in pain medications but that really didn’t help. We think she was anticipating pain, after the first jolt, and was scared of it so she was shaking some. But we had to do it, and in the end she endured and then passed out. I am putting good money down that Jackie is going to have a long night for a couple of reasons, not the least important factor being the time and duration of the nap Rafi had after the bandage change. I think a large coffee with an extra shot of espresso might be justified for Jackie come the morning.

So far this has been a tough road but it hasn’t been impossible to navigate to this point. While that could change at any time, and it probably will, we haven’t doubted for one second our decision to move ahead. I know there are others who want to follow in our footsteps but are questioning their fortitude and the risk reward ratio. Know this, we are under the best of care here. The doctors and nurses are top notch and they stop at nothing to care for your child. They are willing, eager and able to learn about caring for an EB child and they will work with you to address your special needs. If you do decide to move ahead, as we have, come with your eyes open, be well rested, and have a support network ready to spring into action.

The hair is falling out, and it just isn’t falling out, it is getting everywhere. Before we moved to Minnesota, we had two beautiful, big, hairy, dogs and they didn’t shed as much as Rafi is right now. When I got here this morning I saw a couple of hairs on her pillow, but by nightfall, she was leaving a trail of hair everywhere she went. The hats they have here are made by volunteers but they seem to be all made of wool. To a child that already suffers from incredible itchiness, wool is like watching 7 hours of David Blaine. So tomorrow I am going to try and find a nice soft, cotton hat for her.

I would say that she had a pretty good day today. She walked all over the room for a period of time, stood up a lot, laughed a lot and got a good nap in. Her mouth didn’t really bother her so much today, but let’s attribute that to the 24 hour drip of fentanyl (her new pain medication). After the dilaudid/ativan slight overdose the other night we thought it prudent to change her medication to one that is fast acting but doesn’t stay in the body for very long. It seems to be doing the trick just fine. However, she is still running consistent and high fevers. The Tylenol is not quite bringing them down as effectively as before, but add an ice pack and in conjunction they work together well enough. She also needed a transfusion today as her platelets and hemoglobin were low.

We are constantly reminded by the doctors that the worst is yet to come. Even though we’ve prepared ourselves for this, it’s a little scary to be told that over and over again. This may be one time where the anticipation is not worse than the actual event. Thankfully, I haven’t had that hard of a time staying positive. Even though it really stinks to see Rafi feeling terrible when she spikes those high fevers, her entire face changes and her body language is like nothing I have seen before from her, she gives off a strength. She really is a tough little kid.

But it isn’t always easy. This experience is exhausting. Driving the 3 miles to the hospital this morning was a little difficult. I just felt a little sorry for myself and for Jackie. The things we’ve had to give up, the pain we see in our daughter’s eyes, the anguish in Rafi’s tone when we won’t let her scratch the never ending itch, they get to you on occasion. Then I remember how strong Rafi is, and I am competitive as hell and can’t let a two year old best me.

“All these trials, will soon be past, look for something built to last”

So after 3 days living with her news cells that will change her blood type from A positive to O positive, I have no choice but to think she will now be here for the long haul and I am positive again just looking forward to a pretty bad cup of coffee coming my way.

I just realized that in the last few updates while I have given some general information I have neglected to let you know how Rafi is faring. Overall, she is doing well and she is in very good spirits. The doctors are very happy with her progress so far. She has been running some high fevers, 104ish, but Tylenol tends to reduce the fevers to a more acceptable level. We also use ice packs and a cooling blanket when needed. When her fevers start to run high, she becomes a fifth appendage for Jackie or me. Her activity level has been much lower although we try to get her up and walking around as much as possible. She has the beginnings of mucositis and she complains about her mouth at times. However, she ate some clementines last night and that was a little surprising. You would think that acidic fruit and mouth wouldn’t really go together so well. Mostly, she just acts like she has the flu.

I’ve gotten some questions about what happens now that the stem cells are in her body. It’s important to understand that the journey is really just beginning. Her condition will get much worse before it gets better. Rafi no longer has the ability to create any new cells that would fight infection, heal wounds, or beat bacteria. The human body has an incredible amount of bacteria in it and viruses may be present that lie dormant. Now that there is no immune system, the bacteria and viruses can become active and attack the body. It takes time for the new cells to migrate to the bones and begin to create new bone marrow where healthy cells are created that will form an immune system. From the bone marrow, red (cells that carry oxygen), white (immune system cells) and platelets (cells that allow blood to clot) are created. On average, it’s takes about 23 days for the body to start to create a these new cells and about a year for there to be an effective immune system. With that in mind, Rafi is getting a lot of antibiotics and other medications to protect her, as well as immunosuppressants that will help the new cells to begin the process of creating a new immune system and cells that heal her wounds. She will get many transfusions (red cells and platelets) to help her. The key now is make sure her current wounds and the new ones that are springing up don’t get infected. That’s why mucositis is such a concern. There is a tremendous amount of bacteria in the human mouth and the inflammation and ulceration of the mucosal lining gives the bacteria a doorway into the blood stream. So now is a dangerous time. We understand that Rafi is going to have to walk through fire before she gets better. It’s starting now but the real nightmare starts to happen in a few days. Hell can last for a few days or a few weeks.

It’s been a long day for little miss Rafi and I am very much on edge. As I type, I am listening to Rafi breathe and staring at the clock at the lower right hand side of the screen. I am waiting for her breathing to return to normal and time seems to be moving very slowly. I would be very surprised if I slept at all tonight.

Rafi’s been running fevers for the past two days. Her highest one has been 103.7, but Tylenol has been able to knock them down to normal levels very quickly. The fevers are to be expected so we are not worried about them. However, the doctors are telling us that the probable reason for the fevers is mucositis. That came quick. I really didn’t expect it for another 3 or 4 days. Mucositis is the inflammation of your mucous membranes, which are tissues that line your entire gastrointestinal tract. This affects your mouth, esophagus, stomach, intestines, rectum and anus. It has been reported to feel like a bad sunburn or heartburn. It is the main cause for pain, and some serious complications, in BM transplantation. So of course, Rafi had to develop it quickly. I know it is just starting but it is already horrible. Her voice is different than it was a day ago. Her cries are weaker and raspier and her voice is hoarse. Thank goodness for IV pain medications.

The old cliche is true however, “be careful what you wish for”. She was pretty uncomfortable and agitated when we put her to bed this evening so we gave her some ativan and, in addition to her IV dilaudid (which is new as of today), we gave her an additional little bump of dilauded. Well, it turns out all of that was a little much and her breathing pattern was completely different than normal. I really can’t describe how helpless I felt as I watched her clearly uncomfortable and struggling. It’s now almost three hours after the breathing became a little labored and she is doing better. Her breathing has become more regular although she sounds very congested. Her pupils are still a little small (a classic sign of being over medicated) and probably will be for about 2 more hours. Hopefully, she will sleep for a long time.

As for yesterday, the stem cell transplantation was easy and quick. She received 3 ounces of cord blood which took about 20 minutes to go into her. Then four hours later she received her mesenchymal stem cells which took about 15 minutes to transfuse. We had heard that with cord blood, there is a distinct smell that lasts for some time, up to days in some cases. Well, it smells like creamed corn. It really smells like green giant creamed corn. It drove Jackie crazy, because she has the most sensitive nose on the planet. I happen to like corn, so I wasn’t all that phased by it. But Rafi was in a good mood after it all until her temperature rose to above 103. Thankfully, the Tylenol took care of that in under an hour.

I thought the day was going to be a little anticlimactic, like taking aspirin. I was wrong. I’ve never been very excited to take aspirin. I’ve been thankful they exist, but never counted down the minutes until I swallow them. Although to be honest, I have counted down the minutes until they worked.

I would write a more complete update but I am exhausted and need the sleep. I will write some more tomorrow.

Jackie had a long night last night. She was changing diaper after diaper and she didn’t sleep a minute. Rafi declared at a little after 1am that she was done sleeping. Then again, she told Jackie the same thing again a little after 4am. While there was diarrhea, Rafi did not vomit. This is a big victory. It is our hope that we can control the vomiting, in order to spare her esophagus, by having her on two separate anti-nausea medications and by venting her Gtube regularly. I am sure we won’t be successful all the time, but sometimes is better than never. Rafi also needed a dose of Lasix last night as she was not meeting her urine output requirements. Quite frankly I don’t really have a great understanding of the math behind that. She would generally meet her two hour marks but not her shift change marks????? It doesn’t matter, Lasix is harmless when wearing a diaper. Because she spiked a fever, Jakie had to take her to a different area of the hospital for an x-ray. Finally, Rafi needed a blood transfusion last night. She is going to get plenty of them so it wasn’t surprising or worrisome.

The night however, was far from a prelude to the day. Rafi had a great day. Which means Jackie and I had a good day as well, although I have no idea how Jackie was still standing by 3pm. While Rafi did have some fevers (high as 101.8) she was in good spirits, laughed a lot and went through a completely successful bandage change. We decided to only do half the body at a time (lower half one day, upper body the next). Today we changed the leg and feet bandages and tomorrow we’ll change the arm and chest bandages and wash her hair. We gave her Dilaudid, and a small dose of Ativan today and it worked really well. We hope this type of success will continue. The only problem is now we are doing bath and bandage changes every day. At first I didn’t like the idea because that meant giving her the Ativan and Dilaudid every day, but then I came to my senses. It’s not like we have anywhere else to go or anything else to do and she is going to eventually be on a continuous pain medication drip anyway. In the end, it’s a win win.

I took a very funny picture of Rafi today. She was wearing her original Hello Kitty sunglasses, you know the purple ones all over the pages of People and US Magazines, and I had the camera out so I asked her to give me the thumbs up. Of course she did this because she is a total and utter ham for the camera. I’ll post the picture on the site tomorrow when I am at home. It’s just one of those pictures that can really make you smile. Here’s one other thing that really made me laugh today. She told me her finger hurt, so we did our little stretching exercises, which makes her feel better, and when we were done I looked at my hand. Rafi tapped me on my forearm and says to me, “Daddy, your finger hurts? You want Tylenol?” It was a good think I didn’t have any liquid in my mouth because I would have sprayed her.

So, tomorrow is transplant day. Do I really need to say more than that? I don’t think so. So I won’t.

Jackie and I really appreciate all the good wishes, thoughts, prayers, emails, guestbook entries, text messages, phone calls and voice mails. It’s been really heartwarming and great to know so many people stand with us in this fight. I know one day Rafi is going to read over this site and she’ll smile. She’ll know how hard Jackie and I worked to get us here, she’ll see the trials and tribulations and most importantly, she’ll know how many people cared about her and she’ll feel good knowing how important she was to so many people. For that I thank all of you, you’ll have made my daughter’s day some time in the future.

The other thanks I want to express is to the families who have come before us. They did not have the benefit of being able to read daily updates as Jackie and I have. They are the real pioneers of this procedure. But what I really want to thank them for is their willingness to be supportive of us at any time of the day or night. I have now met 4 of the 6 families who have undergone this. With one other I have emailed and I look forward to meeting him and his daughter one day. Without exception, each has offered their sage advice, their experience, a shoulder and their phone numbers. I can’t express my gratitude well enough. What I can do is say, others are coming here after us, some without their spouse and with other children and I promise to try and help them as best I can.

By the way, I just want to say how proud I am of my wife. I don’t say it enough. But I will say this, there is no one else I would rather have in my foxhole. I love her a whole lot.

Not a day passes when I don’t admire my daughter more. She is a tough little fighter. She constantly surprises Jackie and I with her strength and ability to rise above her circumstances. I am proud to call her my daughter. Last night was a long night but it was just a prelude to today. At 4am, after changing her diaper for the “upteenth” time, after a dose of Lasix, and after cleaning up a mess of vomit a couple of hours before, I thought I could finally lay my head down for at least an hour and a half. Relieved is a word that comes to mind. Then I hear:

Rafi: “Daddy”
Me: “Hey Rafi”
Rafi: “Daddy”
Me: “Yes honey, do you want me to pat your back?”
Rafi: “Daddy, put cream on my arm.”

She was itchy but instead of scratching her skin off her arm, she asked me to put some Alwyn cream, which relieves the itchiness for her, on her. She’s just so funny. I couldn’t help but smile and not worry about losing more sleep. She knew that the cream would help. I should also mention that she requests Tylenol by name when she is uncomfortable and I just love the way she pronounces it.

At 6am, we vented her gtube, which means leaving the tube open so that air and undigested food can escape the stomach, thereby making her more comfortable and less likely to throw up in the morning. Thankfully, after that she slept for 2 glorious hours. When I awoke at 8am, Jackie was in the room waiting for Rafi and I to wake.

We planned on having a bath and bandage change today and that meant giving the chloral hydrate and Dilaudid cocktail another try. This time we were going to give both at the same time (an hour before the start time), with a second dose of Dilaudid about 2 hours later. We didn’t get very far because Rafi had a terrible reaction to the chloral hydrate. The other two times she received the drug she was agitated but calmed down. Not this time. At first she just looked like a drunken sailor. She was playing with Sarah, her occupational therapist and having fun, but she was clearly intoxicated. And then, she went into a rage that I have never seen before. She screamed for 2 hours straight, violently thrashed around and nothing worked to calm her down. They call this a paradoxical effect. She has the same reaction to Benadryl. After creating some new wounds and exacerbating existing ones, we gave her some Ativan to calm her down and let her go to sleep. It was difficult for everyone to see because of the sheer violence of it. She slept for about 3 hours and woke up, still a little loopy from all the drugs, but in a better mood.

After a little coaxing, Jackie got Rafi to stand up to get some exercise. The day before she was finally walking around finding the bean bag animals, Katie, her physical therapist hid throughout the room. We want her to be as active as possible at this point. It makes Rafi happy to be active even though she now needs to be coaxed into it. She is a little scared by the hospital and all the new faces but we thought we may have turned a corner after her experiences with Katie and the time she spent with Daina yesterday. Daina is her nurse in Minneapolis who helped with the bandage changes before our hospital admission. She adores Daina, who really goes above and beyond the call of duty with Rafi. After bandage changes, Daina would stay at the house and play with Rafi for long periods of time. Without fail, every time we say bath and bandage change Rafi says with a smile, “Daina’s coming.”

Anyway, Jackie got Rafi to stand up and move around a little after the failed bandage change. Two minutes later, Rafi’s Gtube fell out. The whole thing just dislodged from her stomach. Needless to say we were a little taken aback, to say the least. Thankfully, and to their credit, everyone on 5D moved quickly to get a GI specialist in the room, to get more Ativan, to help change a diaper soiled with diarrhea as well as the leg bandages which were also soiled, and to help distract and contain Rafi as the GI doctor reinserted the Gtube and inflated the balloon that keeps the Gtibe in place. Even though it was placed back in the hole through her gut, there was a slight worry that Rafi would have to undergo surgery to replace the Gtube. The 5D team got radiology to bring up a portable x-ray machine to make sure the Gtube was placed properly. I though Rafi would freak out during this whole saga. She didn’t. She was amazing. As it turns out, the Gtube was placed well, we learned how to keep the balloon inflated and Rafi was once again drugged up.

Soon after I got home, Rafi ran her first fever of 100.6. This triggers blood cultures and an x-ray. This time, Rafi would have to wear a mask to protect her against airborne germs as she was brought down to imaging. We are waiting the test results but now she is on a stronger anitbiotic for the confirmed staph infection, and an antibiotic for pseudomonas, should that be an additional infection. I can now say we are truly part of the EB transplant club. Keep your fingers crossed that the results come back in our favor.

I am happy to say that today we are done with the fludarabine. Only one more dose of the cytoxan to go. We will still have to change diapers every two hours until Thursday at around noon though. We’ve started the immunosuppressants today, Mycophenolate Mofetil (MMF) and Cyclosporine A (CSA). There are, of course, a host of potential side effects. I could list them but the side effects seem to be the same for every drug. What I particularly love is with the MMF, the side effects are diarrhea and constipation………Hmmm, don’t they cancel each other out?

Although we didn’t culture at the time, it is pretty obvious that Rafi has once again developed a staph infection on her foot. It also looks like it has developed under her arm as well. We cultured the arm this morning and are awaiting results, but we already know the answer. Nothing happens in our household without some drama. So we are treating with IV antibiotics before the staph has a chance to go systemic, aka in her blood stream, and put her at any real risk. Add that little wrinkle to the fact that the side effects from the chemotherapy are starting to be a little more pronounced and we have a real ballgame.

It’s amazing to me that we are this close to transplant. For all intents and purposes we are at day -2. It’s very exciting. I know the actual transplant is a little anticlimactic but the symbolic nature of it is pretty amazing. We could have a new chance at life. While the past two years have been difficult at best and the next two will be anything but easy, it’s 4 or 5 years from now that I am thinking about. We are not only changing Rafi’s destiny but also Jackie’s and mine. Our life together, as well as our separate lives, have really been limited and pushed to the point of breaking at times. I am not the same person anymore, and part of me never will be the same. Many of my worst traits are now more pronounced. As I know some of you understand, EB gives you nothing but agida and takes away so much. The transplant isn’t a rebirth but it is a new start.

I’ll try to work harder at not taking so many things and people for granted. We have some really great friends that have been very supportive in so many ways. Some have disappeared and others have come out of the woodwork and really stepped up to the plate. As I see it now, I haven’t always been the best friend, the best family member, the best father or the best husband. For that, and to those people, I apologize. I hope, after this is over 4 or 5 years down the line, I’ll be strong enough, that there will be enough left, to make myself a better person for those that have stuck beside me, Jackie and Rafi.

It’s time to change a diaper now. I hope Jackie is deep under the covers, tucked in tight and sleeping soundly right now. She has two years of sleep to catch up on and she deserves the rest.

We are now halfway done with the cytoxan and the fludarabine. It’s really nice to say that we only have two more days of chemotherapy. Two more days. Two more days. I really like the sound of that.

Rafaella was born on the 19th and she will get her new stem cells on the 19th. As you know, she is the eighth patient with Epidermolysis Bullosa and both Jackie and I were born in the 8th month. There is a nice symmetry to all that. Let’s go from the sublime to the ridiculous for a moment. If you subtract 8 from 19, you get the month Rafi was born. You see how I just took that a little too far. You know what, I’ll grasp at some straws to stay positive and hopeful.

In general, Rafi is doing very well. All the important numbers are still well within acceptable parameters, and her kidneys and bladder are working very well. Most importantly, she only has some bouts of vomiting and diarrhea. We know it is still really early in the process, but no bad news is great news.

The larger dose of chloral hydrate did not completely have the intended effect we hoped for. I can’t say Rafi was sedated or even calmed down as compared to other times. We are going to alter the timing of the drugs and see if that helps at all. The hospital, in their ultimate wisdom, heard our argument about allocating nursing time for the bath and bandage changes and they agreed to provide help. I love it when a plan comes together.

Off to sleep now, while I have the chance.

A few people have asked some questions, many similar ones, so here you go:

Rafi’s weight has fluctuated a little but has generally stayed around 11kg. It’s possible her weight may change more now as we increase the amount of nutrition she receives via gtube, and she retains more water. Overall though, she has not lost any weight and has actually gained a small amount. She is significantly less active now than while at home so that certainly plays a role.

As to what and how she is eating, we are definitely seeing a shift to more gtube feeds. Before this started, most of her nutritional needs were met by drinking her “ba-bas” of pediasure. She did eat some other foods like oatmeal with either a strawberry/blueberry smoothie or applesauce with brown sugar and cinnamon mixed in. She loves baked beans, yogurt, american cheese, chicken soup, really soaked honey nut cherrios, tuna fish, chicken salad, bagels and Jackie’s fresh cookies out of the oven. But recently, and as is expected, we’ve noticed she is taking in less food orally and instead relying more on gtube feeds. We’ve been started putting her medications in through her gtube as well. This says a lot because Rafi always loved her bubblegum flavored prevacid and atarax. We do order some room service in for her twice a day to try and get her to continue eating. Sometimes it works, other times not. At some point, she wont even tolerate gtube feeds and we will start giving TPN (total parenteral nutrition). TPN supplies all her daily nutritional needs through her hickman line (aka, her central venous catheter). Everyone ends up requiring TPN for a period of time and we will try and give her as much food orally and through her gtube as possible as her journey continues. If we can do that, research has shown that she will recover from the procedure quicker.

As to Rafi’s normal EB care, Jackie and I do everything. We’ve also been told not to expect any significant help during bath and bandage changes. It’s not a question of the nurses wanting to get involved, because they do. It’s more an issue of more patients to each nurse and time constraints. I am going to have a conversation with the nurse manager to lobby for help for a chunk of time and will make this fight as far up as I have to at this point. I believe it is vitally important for a nurse to be involved in the bath and bandage change. Starting now, we need to be hyper-vigilant about infections in the wounds. If there was a hint of discoloration on the bandages the wounds need to be thoroughly assessed for infection. It is imperative to catch an infection early and be aggressive in treating it. The last thing we need is for a topical infection to turn systemic when she has no immune system. I would hope that nursing care during the one time when her body and skin is exposed and able to be assessed would be part of the treatment plan for all EB patients. Plus, many complications, like GVHD, present themselves as rashes and the bath and bandage change is the best time to make a full assessment. Starting today, I will make the case for nursing coverage during bath and bandage changes. Ideally, I would like to get one or two nurses trained in EB care who would be responsible for assisting parents in the bath and bandage changes for all the future EB kids as well. The more I think about it, the more important I think it is. I just want to emphasize that the nurses want to be involved and the help we have gotten has been great.

There was also a question as to the chemotherapy drugs. Today is the second day that Rafi is getting doses of the next two chemotherapy drugs. These drugs are the reason why we have to change her diapers every two hours. We are on schedule and February 19th is transplant day.

Some people have questions about the cost of the transplant in general. There are many factors that will affect the total cost of the procedure in the end. For instance, something as simple as how many days you are in the hospital will have a significant impact. In general, if you don’t have insurance help, you’ve got to plan on this costing $1 million dollars.

Four nights and three days more of changing diapers every two hours. That’s a lot of diapers and very little sleep. If you think about it, you have to change the diaper and get her back to sleep (could take 20 minutes at times) and then get myself, or Jackie herself, back to sleep. I might be better off not trying to sleep at all. I have enough work to catch up on and the hospital provides a PS2 in the room so Madden awaits.

Anyway, while Rafi is on the cytoxin and the fludarabine they pump her full of liquids to flush her system. The idea is to have the drugs exit the body as fast as they enter. Of course this means she will require some help, usually more often than not, in fully flushing the system. Therefore, she will get lasix often, a loop diuretic that prevents your body from absorbing too much salt and is wonderful in making you urinate (Bournoulli would be ecstatic). To give you an idea of how well lasix works, she will urinate 5 to 7 ounces every two hours while they are pushing fluids. Twenty minutes after a lasix dose (the resident cut the dosage in half to try it out thankfully) Rafi’s diaper held 12 ounces.

However, her Potassium level has dropped so add that to her list of liquids going through one of her five IV pumps. Moreover, she has several new additions to the drug lineup as well. She has one drug to mitigate a particularly harmful potential side effect of the cytoxin and a new antibiotic, flagyl. This drug is to fight an infection that was probably caused by her taking the Bactrim that was prescribed to fight the staph infections that initially delayed our admission. Better living through chemistry at it’s finest.

Up to now she hasn’t been hampered to much by chemotherapy side effects. They are clearly there and there are times that she doesn’t feel well. It is very easy to tell when this happens. Mostly, her eyes change shape and she holds her head in a different manner. It’s hard knowing that this is only the beginning of her feeling badly. We know it’s only for a short time that she will feel terrible but the anticipation makes it worse. She’s been putting her hands in her mouth lately as well so I can only assume that is starting to hurt a little at times.

She does have the ability to crack us up though. Apparently, Rafi loves pizza. This in itself is not very funny, except that she has NEVER had pizza. In the same vein, she also loves coffee. Unfortunately, we can no longer share food with her, so fortunately for her, she got her own piece of plain pizza. She kept asking if I was done, hoping I would say no, so that she could stare at her piece and take more bites. If she only understood she could chew and swallow the food in her mouth and have more, she would surely be a candidate for daily insulin injections.

Right now Rafi is getting her last dose of busulfan. It’s nice to be done with the first drug. Unfortunately, she is starting to feel the effects. She has some stomach cramping, some diarrhea and she’s thrown up a couple of times. With that said, the big issue up till now and a pretty large one going forward is the bath and bandage change. The nurses on the floor have been great in making the time to help us as best they can. It’s always been a three person job and now we are trying to figure out how to make it a two person job without risking injury to Rafi.

We tried some new drugs today for the bath, chloral hydrate and dilaudid. Chloral hydrate is a sedative and a hypnotic. While it worked a little bit, I wouldn’t say it was a huge success. She was actually a litlte more agitated at times. She didn’t get a large dose and we are going to try a larger one at the next change to hopefully make her calm enough to last through the entire process. She’s been a little crazy by the time we get to her calves and feet (about an hour and a half into the whole thing). It’s difficult at best when she has no bandages on and she is flailing around. It’s stressful and we are constantly afraid of creating new wounds. I’ve taken skin off her calf before when trying to control her movement and now would be a terrible time for that to happen. Keep your fingers crossed that the increased dose of chloral hydrate and the strong pain killer, dilaudid, will help keep her calm.

In writing these updates, I try to think about what I would want to know if I was thinking about going through this process with my child. Jackie and I want to provide not only anecdotal information about Rafi, but also information that is helpful for the EB families about to go through this or those thinking about it. The information I would want is not always what others want or need. Please don’t hesitate to ask specific questions.

Today, Rafi was in much better spirits and was much more animated than in the past couple of days. I am guessing that it was probably due to the fact that we didn’t do a bath and bandage change. For now we are going to avoid the ativan and oxycodone combination and try a different cocktail. We think the ativan just doesn’t work very well and it makes her a little sick to her stomach. We’ve given it to her on 4 occasions and she’s vomited twice. I think it is safe to assume it doesn’t agree with her. In addition to that, after two years of oxycodone it’s possible that she’s built up a resistance to it. We hope that the new mixture (which I will go into more detail tomorrow) will make her more tired and might let her actually sleep during the whole thing. I am hopeful this works but not unrealistic.

Rafi has decided to call her tubes a leash at this point. I find it hilarious. It’s my fault she is saying it, because I jokingly referred to all her tubes as one. Once, I said it once in passing. “Daddy what you doing with my leash?” It cracks me up.

On the topic of side effects from the chemotherapy drug, she is having some cramping. She complained this morning that her stomach hurt, then it would go away. Ten minutes later she would complain again. During rounds Dr. Tolar told Jackie that it was the busulfan. So while Jackie and I felt bad for her, we knew this was something she could handle easily. She’s so damn tough.

We have a pretty big room as far as hospital rooms go. For those that live in NYC, it’s about the size of a small studio apartment. We have a queen sized aerobed in here (which we deflate during the day) and there is room for a small round table, the hospital crib, our football goalpost (where all Rafis’ pumps are screwed onto) and of course our shelving full of bandages. They really stocked us up on all the supplies we could want, or need or ask for, for that matter. It’s great on that front. I was expecting a shortage of bandages and adhesive remover spray, etc. Nope. We have a cornucopia of products.

Not much more to report this evening. Our nurse for the evening just walked in to hook Rafi up to yet another dose of busulfan. With that, I bid you goodnight.

In about a half an hour or so, Rafi is going to get her 8th infusion of busulfan. 2 days down and 2 more to go on this drug. Halfway done with one. It’s nice to achieve little milestones. Now if we could just figure out the best way to do the bath and bandage changes in the hospital that would be great, because it’s a huge hurdle to overcome. Rafi is such a strong willed little girl that all I can think is that it is a blessing and a curse. Much more of a blessing, but there are downsides. One of the big jokes about her when we were back in NY with our occupational and physical therapists was that Rafi was just incredibly self directed. She will do what she wants when she wants to do it. Well, in that vein, hell hath no fury like a 2 year old who wants to be done with a bath and bandage change. At least I can report that she has an incredibly healthy set of lungs.

I think she is showing some signs of the effectiveness of the chemotherapy. She’s just not herself and you can see it in her face that she isn’t feeling 100%. She vomited for the first time today but that could be from the ativan we gave her earlier in the day, or it could be from the cumulative effects of all the drugs. Who knows. What I do know is that she is starting to warm up to some of the people in the hospital. The past couple of days have been a whirlwind of new faces for her. Thankfully, she had a very fun time this morning with her feeding therapist and her occupational therapist. Jackie and I were saying how nice it was to see her laugh and enjoy herself. We’ve only just started this process but we know how important it is to see the little things. It was only about an hour and a half of the day but it is the time I am going to remember most.

While she hasn’t had many more wounds or blisters, we can see that her skin quality is trending downwards. It’s impossible to know why. I have given up trying to figure out cycles and rationalize reasons why she gets worse then better. For every step forward there are sometimes two steps back. Sometimes we get two steps forward with no steps backward. There is no rhyme or reason. It’s just EB. And in the end, that’s why we are here. Even if there are two steps backward at this point we know we’ve taken four steps forward.

I am in our apartment tonight and Jackie is in the hospital room with Rafi. Hopefully they are both sleeping comfortably. We are on the road to recovery. I know it to be true. Another thing I know is that it is hard to verbalize what goes through my head as I read all the comments and emails we get from you all. It’s nice to know that there are so many people pulling for us, pulling for Rafi. I’ve been surprised by those that don’t know us, and surprised by those that do. I often think of my high school friend, Mike Weiss, who was diagnosed with MS a few years ago. His strength, courage and determination in getting out in front of his situation really inspired me and gave me a strength to deal with this that I honestly didn’t think I had. This is a quotation from his site www.mymsteam.com

“When I was first diagnosed with Multiple Sclerosis on April 26th, 2004, I experienced an overwhelming feeling that I had just lost control over my future. Instead of giving in to that feeling and letting my MS control me, I decided to take a proactive approach and started to build a support team to manage my MS……..My goal is to help others realize that they must own their MS, be proactive, and continue to do something every single day to put themselves in a position to be successful.”

His words are not only applicable to MS. They are applicable to EB as well. Jackie, like Mike, did everything she could to give Rafi the most normal life possible. She was proactive. She organized a play group, she took Rafi to music classes, she worked hard in finding the right school for Rafi to go to and got her accepted into it (not an easy task in New York City). She didn’t let EB stand in the way of Rafi living and enjoying life. Jackie, like Mike, not only speaks those words, she lives them everyday.

Just like my little Rafi and Jackie, Mike is inspirational in fighting another disease that as of yet, has no cure. He was always a strong guy, but now he is a giant who I am honored to know. He has a short, 1 minute video on his site that I urge you to go and view. If my mother was still alive today she would be incredibly proud of him. She always loved Mike and I know she would love Jackie.

This has been a long day, and yet nothing has happened. It was around 1am last night when I set my blackberry alarm to wake me at 3am. Jackie stayed with Rafi in the hospital the first night but I really wanted to be at her side when they started that first infusion of busulfan at 4am. I am not sure why I wanted to be there. It’s about as climactic as taking some aspirin. But I felt it was important to be there. I felt like it was my fatherly duty.

Yeah well I didn’t make it. My head popped up and it was 4:45am. Boy was I mad at myself. I sent Jackie a text message inquiring about the situation and, of course, there was nothing to report. 45 minutes later I was in the hospital room lying down on our not quite inflated aerobed with Jackie. And then, every couple of minutes, one of the pumps in Rafi’s room makes the same noise as the door opening. It’s really uncanny. So I am expecting another wonderful nights sleep this evening.

It’s just before 10pm now and as I am typing that damn pump just made me pick my head up to see who was coming into the room. In a few minutes two nurses will walk in and set up the fourth infusion of chemotherapy. So every time that pump goes off, I am expecting busulfan. It just happened again. Wow I could use a scotch. The first infusion was at 4am, then 10am, then 4pm and finally at 10pm. Each IV infusion lasts 2 hours and there are 16 in total. One of the things i didn’t expect was that Rafi was going to be hooked up all the time. I guess i thought she would get the busulfan and then be off her leash for awhile. Oh well. She’ll get used to it, the same way she has dealt with everything else we’ve thrown at her.

With every blood test result we get and every time I look at her I know that the timing of us being here is perfect. If we waited longer I fear that EB would have taken too much from her. Her zinc levels are low, her red blood count and hemoglobin levels are low (and both were dropping even before the chemo), she has iron deficiency anemia, and her skin is breaking down more often than before. I don’t think she would have had a long time on this earth. So I am glad we are here. As I sit here and type I can vividly remember when kashual (our transplant coordinator) called and said that blue cross was approving the transplant. I was truly overwhelmed. We had just sent in the paperwork a couple of days before. In the defense of my stoic manhood, I hadn’t had a sip of my morning coffee when she called.

I also think the busulfan hit her early. I could be wrong, but when you look at your kid’s eyes you can just see that something isn’t right. Of course, she says, “Daddy I’m fine” when I ask her how she is doing. It’s been one day and here I am looking for any sign of side effects. Although I can say that I am not worried about a self fulfilling prophecy in this case. We know it’s coming, so here we are waiting, waiting and just waiting some more.

By the way, if you want me to report on anything I haven’t touched on as of yet please don’t hesitate to email me.

I should really do some work, but I am so tired right now I cna barely keep my eyes open and Marybeth keep telling Jackie and I to sleep. So I am going to take her advice. Let’s keep Sam in our thoughts and prayers as well. She is a tough one.

Brett

It’s a little hard to grasp that we are finally here and moving forward. Strangely enough I am not nervous. I can’t say that I am excited but it’s nice to be at the point where we are changing her destiny, for better or worse. Hopefully we can eradicate her Epidermolysis Bullosa. At the very least, I hope we can let her have a less severe form of the disease. Most importantly, at 4am on Wednesday, 5 hours from now, there is no turning back. Rafi will get her first dose of busulfan in the early morning hours of day -9. Busulfan is a chemotherapy drug that kills a cell’s ability to divide. There are a host of potential side effects (vomiting, hair loss, mouth sores, etc) and we hope that Rafi will whether the four days of the drug well. It is our understanding that the side effects take a few days to manifest themselves, but we will see.

Rafi is having her first blood transfusion as I type. Her hemoglobin dropped to 7.7, and they give a blood transfusion when you dip below 8. This is the first of many transfusions in the coming months. We are not sure why she has such a low count but this was going to happen anyway from the conditioning regimen (chemotherapy and immunosuppressants). She’s also receiving some other drugs at this point. In preparation for the busulfan, they gave Rafi Keppra, an anticonvulsant. She also got her first dose of Caspofungin, an antifungal drug. From what I understand there are relatively few side effects from this drug, so that’s good, and a welcome change.

Thank you to everyone who wrote us emails, text messages and left comments on this site. Jackie and I really appreciate the support, the kind words, the thoughts and the prayers. We know this is going to be a long and possibly painful journey, for us and particularly for Rafi, and we really feel lucky to have so many people on our side.

One of the things I want to happen during this journey is to educate people about this disease and how it really hurts everyone involved. It’s my hope that each of you will pass this site along to your friends and family, and ask them to do the same, so that we can raise some awareness about this horrible disease.

Denial, anger, bargaining, depression, acceptance. How many times do I have to go through those 5 stages? Some days I might not even think of them. Then there is today. I just hate this disease. I have accepted the fact of it, of what it is doing to my child, to my wife and to me. I have accepted the fact that we need to put our daughter through a horrible procedure to hopefully allow her to get better. But sometimes the disease wins a battle in the war. It takes little pieces of me daily. I try to be hopeful and optimistic and I think some of the time I am. Other times not. I am not second guessing our decision to go through with the stem cell transplant, just venting. I am hoping this will be cathartic.

Today Jackie and I were changing her diaper and I was reading her the “I Love You” book. It’s been one of her favorites every since her former nurse, Lissette, got it for her. Anyway, each page starts with “I love you when…” whether that be when you are scared, when you are stinky, when you are silly, when you hide my keys, etc. One of the pages says “I love you when you make new friends.” Rafi’s response was “I have no friends.” My heart hit the floor. My stomach got queasy, my throat tightened up and I could feel the tears starting to well up. Another little piece ripped out and stomped upon by the heavy foot of EB. When we moved here from New York City we thought we were going to start in December, then in January, now in February. We felt it important to keep her away from other kids during cold and flu season for a couple of weeks before the transplant. Now it’s three months later. In the three months we’ve been here, Rafi has had two interactions with other children. That’s a long time to be without child interactions for a 2 year old. So before her daily dose of Sesame Street, we played some home videos of her and her friends from home. I hope that helped. I don’t ever want to hear her say that again.

Another thing I never want to hear again is “Daddy you hurt me.” She’s told me that twice recently. Once during a bath and bandage change and today. I caused a blister on the palm of her hand. I watched as the blister filled with liquid and as it grew on her hand. I watched as her body writhed in pain when it first happened. Her cries penetrating my body like a fog horn going off two feet away. Jackie and I have told every nurse whose been involved with her care that you aren’t part of the family until you’ve left a mark on her. Until you have hurt her once. I’ve seen a couple of them cry. I accept the fact that I am going to hurt her on occasion. It just makes the other stages more prevalent when Rafi verbalizes it when I do. I hate this disease.

I am beyond the denial and was never one for the bargaining. But the anger, and depression still raise their ugly heads. Especially on days like today.

I am hopeful that this transplant will lessen her pain, her itchiness, and her general discomfort. I am hopeful that this transplant will allow her to eat those green cookies that she always asks for and wants constantly. I am hopeful. I am hopeful. I am hopeful. It seems like a nice mantra and with that, I bid you goodnight.

It’s nice when all you have to report is good news. Today went exceptionally well. There was the outside chance that the hickman line wouldn’t withstand the pressure of the apheresis machine. No problem there. The collection took a little longer than originally planned because there was a low concentration of cd34 cells in her blood. So, they increased the time the apheresis machine ran and it was the right decision. Plus, we found out that the staph infections were not MRSA. That makes life a little easier. So, Rafi is now taking Bactrim for the next 7 days, with diluted bleach baths and bactroban cream on the open wounds. The plan is now to be admitted next Tuesday, February 9 and have the first dose of busulfan on February 10. That would make February 19th transplant day.

I took some video of the apheresis process and will upload it in the next day or two. You’ll get to see how great Rafaella was during the process. She continues to amaze us at every turn. The machine is loud, there are people constantly in the room and there are a lot of tubes hanging all over the place. Later in the day Rafi told Jackie and I that she had fun. Who could blame her she got to watch Cars, Shrek and Tiger and Poo.

At the end of last week, we felt it prudent to culture some of the open wounds that Rafi has to see if there were any active infections. Sure enough, the wounds cultured positive for staph. Not a real surprise and it’s something we fight against every day. We are still waiting for the sensitivity reports to see the best way to fight the infections. The sensitivity reports tell us what antibiotics will be ineffective against the infections. We are hoping that the staph is not MRSA. We won’t be surprised if some of the wounds are infected with MRSA as Rafi has already battled that little bug. We are using a diluted bleach and soap bath with plenty of bactroban (an antibiotic topical cream) on the wounds for now. So far, the wounds seem to be getting better and seem to be less infected. In speaking with Drs. Wagner and Tolar today, and after meeting with an infectious disease doctor, we decided to delay the beginning of the chemotherapy preparative regimen until she is free of infections. It is the safest and most prudent thing to do at this point. She must be free of infection before we give her that first dose of busulfan.

We are still going into the hospital tomorrow to harvest her stem cells. We go in at 7:30 am, and she will get her fifth dose of the GCSF and then get hooked up to the apheresis machine. We think it is going to take about 6 hours. It is our hope that the pressure that the machine will exert on her hickman line will not collapse it. At this point, we have some confidence that by slowing the flow of the machine down, and thereby increasing the time she is hooked up to it, that it will allow the hickman line to hold up. The pediatric surgeon who placed the hickman line believes it will work as do our other doctors. We are banking on the strength of our sheer will to make it so.

The GCSF infusions were easy on her and easy in general. We push in 1.5 – 2 mls every couple of minutes until the syringe was empty. The whole process takes 15 to 20 minutes. Rafi had absolutely no negative side effects from the GCSF. We kept asking if she was feeling alright and her answer was always “I’m fine”. Those words were always followed by “I want to go to the treasure chest.” At the BMT clinic, there are a host of donated toys in a chest for the kids to take. She’s taken a little pink pony with long dreads, an elephant notebook to draw in and, this made daddy very happy, a small nerf football. To complete the let’s make daddy happy thought, Rafi now wants to learn how to play golf. This is all because of a “life’s been good” t-shirt and a hat I have that have golfers on them. I am looking forward to playing golf with her one day.

Rafi has now amassed a small war chest of toys and “beads of courage” as she constantly receives these things from the wonderful staff at the hospital. The people there are really amazing. They have made the hospital a fun place for her and not something to be feared. She is becoming less nervous around people she has never met and I really attribute that to the way everyone treats her there. They are proactive and they are kind and they are always professional. We couldn’t ask for a better group to take care of our child.

Thank you to everyone who has emailed through the site, emailed us on our personal accounts, left comments on the site, and just sent good wishes to Jackie and I. The outpouring of support is amazing and we very much appreciate it.

Today we got our second infusion of GCSF (granulocyte colony-stimluating factor). The purpose of the GCSF is to stimulate the bone marrow to make more neutrophils and other types of white blood cells. The idea is to have Rafi create more stem cells so that we can harvest them and store them as a backup if her body rejects her new stem cells. Because the GCSF will allow her to create more while blood cells, we hope this will help her heal some nagging wounds. Unfortunately, we think she has developed a psuedomonas infection and we hope the infusions will help with that as well. The timing of this infection is just perfect of course. We are now scheduled to be admitted to the hospital on Tuesday (no longer Monday – the apheresis department was a little busy on Monday so we delayed one day) to harvest the stem cells and then receive the first chemotherapy does on Wednesday morning. Needless to say, this would set the treatment back a little. About 15 minutes ago she was running a slight fever (100.3) and we will check it again in a few minutes. She has been running around a lot and we think her body temperature is just a result of that.

We have two friends staying with us for the weekend and Rafi has been happier than I have seen her in awhile. She is constantly referring to Raniya and Julie as “her friends” as she runs around the house. It’s really nice to see her so animated. It was really funny to hear her say this morning as we were getting the GCSF infusion, “what are my friends doing?”. She really is a funny kid.

We had a rough bandage change yesterday. It took longer than normal because we had to photograph all her wounds and do as many swabs as we can. Of course the little one was as defiant as ever and was screaming at the top of her lungs and thrashing about. It could very well have been one of the hardest bandage changes we have done. I just hope I didn’t do more damage to her, but I wouldn’t be surprised if I did. We’ll certainly see tomorrow afternoon.

Let’s hope Rafi doesn’t have a fever this evening, that she doesn’t have any infections and that tomorrow’s bath and bandage change goes smoothly. Think some positive thoughts for Samantha Sheridan as she is still having a rough time at the moment. She is a great young woman who could use a run of good luck.

Thank you to everyone for all your emails, comments, thoughts and prayers. Jackie and I are both grateful and thankful for all the support. We are a little overwhelmed by the response we’ve gotten so far. Quite frankly it’s a little unbelievable.

A lot has happened since I last wrote anything, but the real news is that Rafi just went through a long week of tests, tests and more tests. We’ve spent a lot of time at the hospital this past week. Between our stints at the hospital and our normal bath and bandage changes, there hasn’t been time to do anything, let alone update. The purpose of all the tests is to make sure that she is healthy enough to undergo the chemotherapy regimen and the transplant in general. High dose chemotherapy can be really rough on some of the internal organs so they need to make sure those parts of the body have the ability to withstand the stress.

Jackie and I were worried about how she would handle some of those tests. They can be scary for an adult, let alone a 2 year old. Rafi can be a little anxious at times and we thought a CT scan and an echo would send her blood pressure through the roof and generally just scare her to the point of tears. We wanted to sedate her to make the tests as easy as possible. We did that before her surgery and the drug they gave her, Versed, was the best thing in the world. She went from being really scared in pre-op to being animated and actually talking to strangers. Without the drug, wheeling her into the OR and putting the anesthesia mask on her would have freaked her out. With the drug, she was enjoying the ride on the crib gurney and just waving at me as we walked through the hallways. Operating rooms can be scary places, with the bright lights, strangers and machines. She didn’t notice. The Versed was great. So, we wanted to ease through this week by relying on sedation. Turns out, we underestimated our little one. She was amazing. She constantly surprises us.

We found out today she is healthy. Everything checked out. Her kidneys function very well, and they didn’t find anything they didn’t expect to see. So that means we can move ahead with her first dose of GCSF tomorrow. They give her this for 5 days to stimulate stem cell growth. On Monday, we go into the hospital and harvest some stem cells. She’ll get hooked up to a machine for about 5 hours as they filter her blood and collect her stem cells to store in case she rejects her graft, aka her new stem cells from the umbilical cord blood.

However, for those that know us, nothing happens in our house without a little drama. We found out that her Hickman line may not be sturdy enough to handle the pressure the apheresis machine will exert on it. When you filter the blood, by drawing it out of the body, running it through a machine, and then replacing it back into the body, the flow of the blood creates a suction pressure on the tubes. There is a worry that the line may collapse under the pressure. If that happens, Rafi will need to go back into the operating room, have her Hickman line replaced and have an additional, albeit, temporary, wider and more rigid catheter inserted for the stem cell collection. In deciding whether to just have a temporary one inserted to avoid the uncertainty, we went to the apheresis clinic today to test her current lines and everyone thinks that they may be able to withstand the pressure. Only time will tell. We hope it will. We don’t want her to have to go back under anesthesia and into an operating room, thereby increasing a risk of infection, if it’s not necessary. Our fingers are crossed.

So the big day comes near. We go into the hospital on Monday, February 1, for the long haul. Monday will be considered day -10. On Tuesday, at 4am, she will get her first dose of busulfan. It’s a rather nasty chemotherapy drug. I guess all chemotherapy drugs are nasty though. From there the drugs start to pile on. I’ll list them in more detail in later posts. If all goes according to plan, Rafi will get her new stem cells on February 11, day 0. I say if everything goes according to plan because we still need to check if she has any active infections. On Friday, during our normal bath and bandage change, I am going to swab every open wound she has and hope she doesn’t have anything growing. I am worried that she might have some Pseudomonas on the top of her right foot and possibly on the front of her neck. If she does have an infection, that will set the schedule back a few days. It’s our hope that the GCSF and extended vinegar and bleach soaks will kill anything that is growing, if something is in fact growing at all. We are also seeing Dr. Acton tomorrow, he is the pediatric surgeon who put in the Hickman and Gtube, so he can take a look at this pimple like growth that popped up right by the Gtube. So we may have a couple of hurdles to overcome before we can start.

Guestbook
We would love for you to leave a comment for Rafi. We may not be able to get back to you as fast as we like, but we are sure you understand. Thanks……..

Jackie and I are so touched that so many of you have reached out to us.  We know it’s going to be a difficult road but all your support is invaluable.  Thank you all.  I promise to get back to everyone who has written, it just might take a few days. 

Rafaella has recovered remarkably well from the surgery and the biopsies yesterday.  She was up and running around with no pain medication today.  I have to say it is definitely due to how well the surgeon did his work.  I was expecting a decent amount of residual skin breakdown from the surgery and there has been none.  The anesthesiolgist was also amazing.  Not a mark on her face or in her mouth from the intubation or the gas mask.  I am so impressed with the quality of the work they did.  As an added bonus, she seems to not mind the double lumen hickman line that hangs down to her knees, or the new funny looking button above her belly button.  The only negative thing that came out today was that she is once again anemic from an iron deficiency.  It’s a very common form of anemia where there is a decrease in the number of red blood cells.  Without the iron, the red blood cells can’t carry oxygen efficiently.  Basically, because of this, she can have a sore tongue (which she complains of), some fatigue, some increased itchiness, brittle nails, decreased appetite, does this sound familar to you EB families?

So now we go home and allow her to heal.  I am waiting to hear when we will go in to harvest a batch of her stem cells.  In a nutshell, she will be given some medications to stimulate the bone marrow to produce more stem cells.  Rafi will get hooked up to a machine which will filter her blood and collect some cells.  The reason to do this is pretty simple.  If Rafi rejects the donor stem cells, we can infuse her cells back into her and be theoretically no worse off than we are now.  Except for the fact, of course, that she went through a conditioning regimen of chemotherapy and immunosuppressants. 

Anyway, both Jackie and I are nervous and excited that we are getting so close to this transplant that we have worked so hard to get to for almost two years.  And yet, I have such mixed emotions about this.  As a father, my job is to protect her and it’s everyone’s hope that this treatment may do that.  Yet, at the same time I am really putting her in harms way.  In the end, and this is how I sleep at night, I know no matter what happens, Jackie and I made the most informed, rational and emotional choice possible.  Leaving our home, moving here and enrolling her in this trial was absolutely the right choice.  We are giving her a chance to live a more normal life.  Hopefully, she will be able to grow into adulthood and gain her independence.  I want her to go to college, to live away from home, to have boyfriends, well maybe not have boyfriends.  But, I do want her to be able to sneak out of town for a weekend with her friends without having to schedule nursing visits and drag an extra suitcase of bandages.  Maybe all that might not happen, but right now, this treatment is the only option to potentially give her those possibilities. 

I know the road ahead is not paved in gold.  I know we will walk through fire and question our decision at several forks in the road.  I know my anxiety level will be through the roof.  I know have some control issues, or rather the inability to scede control, and this is going to test my limits for sure.  I know it will be worth it.  I think I may need valium, because I doubt I’ll have time to have my single malt scotch.

Very soon you’ll be able to read about Rafi’s journey through her stem cell transplant.  It’s our hope that the transplant will result in a cure for her epidermolysis bullosa. Two years ago, she was diagnosed with Recessive Dystrophic Epidermolysis Bullosa, Hallopeau-Siemens.  It’s been a long two years.

Rafi will be the 8th child to undergo the stem cell tranplant at the University of Minnesota under the care of Dr. John Wagner and Dr. Jakub Tolar and their team.  Rafaella, I believe, is the second child to have an unrelated donor and the second to use umbilical cord blood as a source for the stem cells.  We found a great match (5/6) and there is a high cell count in the cord blood.  Both factors are good news. 

Rafaella is the 1st child, however, to undergo the new protocol which, if all goes as planned, should allow her to heal faster, tolerate the chemotherapy regimen better and hopefully rebound quicker.  We are hopeful.  This is now the only FDA approved trial for Epidermolysis Bullosa.

Once the transplant starts, potentially at the end of January 2010, we will write everyday about what is going on, how she is doing and provide as much medical information as possible.  We had a million questions about this treatment and we hope to be able to give you the information you want.  We feel that it is our responsibility to do this and we will report the good and the bad.

This Tuesday, January 5, Rafi will have her Hickman line and gtube surgically inserted, as well as have her first set of skin biopsies taken.  We should start the transplant about 3 weeks after. 

We want to thank the previous families who have been so willing to share their experiences as they went through this journey.  Their willingness to share everything has been inspirational to us. 

In the meantime, you can see what Rafaella is like now by going to YouTube.com and searching for UWSGURU.  Three recent videos of Rafaella will be listed.  She is a wonderful child, and we hope you will enjoy the videos.

If you want to receive an email when the site goes live, email me at brettkope@gmail.com

Brett

father to Rafaella, 2 year old RDEB-HS