June 18, 2011 Back in NY

June 18th, 2011

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.

February 5, 2011

February 6th, 2011

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.

+287, December 3 – Time to Go Back to NY

December 3rd, 2010

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.

Day +259, November 5

November 5th, 2010

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.

Halloween, Day +254

October 31st, 2010

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.

+238, October 15

October 15th, 2010

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.

September 29, Day +222

September 29th, 2010

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.

September 25, Much to Report

September 19th, 2010

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.

August 25, 6 months after transplant

August 25th, 2010

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.

August 8, Day +170

August 8th, 2010

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.