July 30, Day +161

July 30th, 2010

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.

July 20, Day +151

July 20th, 2010

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.

July 16, Day +147

July 15th, 2010

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.

Day +131, June 30

June 30th, 2010

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.

Day +124, June 23

June 23rd, 2010

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.

Day +123, June 22

June 22nd, 2010

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……………

Day +120, June 19

June 19th, 2010

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……

Day +119, June 18

June 18th, 2010

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…………

Day +118, June 17

June 17th, 2010

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.

Day +115, Monday June 14

June 14th, 2010

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