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Two Steps Forward, One Step Back

/ Evan / 3 Comments

It seems like the nature of treatment that we are never able to quite get clear of things before we have a setback. We progress, things improve, and then something happens and we are back where we were a few months ago in terms of medications and precautions. It IS improvement in a big picture sense, but it’s bittersweet and stressful day to day.

Zoe is back on track now, and our worst fears have been put to rest. She does not have a recurrence of HLH, her graft came back at >98% again, the same as before and a clear sign that her transplant has taken and HLH is gone (forever we hope). The fear of a return of HLH was due to a rash that appeared very similar to an HLH rash, but the chimerism result reassures us that is not the explanation.

GvHD Rash before treatment

She does however have GvHD, which has required her to restart Tacrolimus and go on a short term run of Prednisolone, the old nasty steroid, again. Why she has GvHD was a big point of confusion to us given her chimerism results, so I will do my best to explain briefly.

Zoe has a new immune system, and that immune system is completely donor material. This is the result we want, since her old immune system was dysfunctional and susceptible to HLH. Her new immune system however has not yet come to terms with it’s new home, meaning her old body is still considered a threat to it to some degree. When left unsupressed, her new immune system attempts to fight her old body, causing the rashes and other GvHD symptoms.

We had gotten her off of almost all of her meds slowly over months of weaning, when the rashes started to appear. It seems that she is not quite ready to be off of them, so we have restarted the routine and we’ll see where she is in another 3 months or so.

There is a question of whether or not she will be ready to enter pre-school this fall, but I’ve been told by reliable sources — Zoe’s two teacher-grandparents — that she is too young to need to be in school anyway. Yes it was what we had planned and yes it would be helpful, particularly socially, but it’s not going to hold her back to wait another 6 months or year to be safe.

In the meantime, she is back to looking good and feeling good most days, which is a relief. We are going to finally be able to remove her port despite the complications of these past weeks, so that too is progress. At this point I’m wishing that we had started putting little patches on Zoe’s well-worn diaper bag for each medical scare, not unlike notches in a gunslinger’s holster or emblems on a jet fighter. She’d have them racked up by now.

 

 

The Spring of our Discontent

/ Evan / 4 Comments

Where to begin? So much has happened in the past couple of months since my last update. First, an apology for a lack of news for those still following Zoe’s progress. My father became ill in April, and over the course of 2 months was in and out of the hospital. He lost his fight on May 26th, his health was just too poor and immune system too weak to fight off infections. A bitter irony, to be sure.

Michelle and I have been juggling the girls, work, and illness of one kind or another now for so long, it has become our norm. We’re still hoping for that break in the clouds though, when Zoe’s health is clear and the rest of the family remains in good health as well. Oh for the days of just work and family.

Zoe has generally done very well in recent months, with one unsettling exception. She has had rashes that have come and gone regularly, but seemed to get worse as we tapered her tacrolimus, which is the immuno-suppressent medication that she was expected to be on for approximately a year post-transplant. We’ve passed that point and so continued with her taper, but when we actually stopped it, the rashes seemed to get worse to the point where we felt we had to restart.

Michelle and the girls went to Duke on Monday, and their level of concern was high over the rashes. So high, there was even talk of a return of HLH, though we have to believe that’s not possible. The thinking until now was that the rashing was the result of a drug interaction (possibly sun sensitivity, a side-effect of tacrolimus) or something else, perhaps hay fever or an allergy. There is also a risk it could be GvHD.

Zoe’s last chimerism test to determine how much of her immune system is donor and how much was hers was very positive, >98% donor. As I understand the science, this should mean little or no GvHD, which is why everyone has continued to feel the rash was something else. It has proven persistent enough and has increased not decreased, so we have to find the cause.

We’ve ordered a new chimerism and we’re very anxious and scared about the results. Hopefully the result is the same and there is something else going on with her skin. If her graft has begun to dip, things will get much harder for Zoe again. She was due to have her port out in a week — that’s not looking like it will happen yet.

Zoe’s mood is good, she’s had a great few months learning to play with her sister and having new experiences since the end of flu season. She’s been able to play with new kids, visit lots of places she didn’t get to go this past year, and even spend a week at the beach (inside during the day). She looks good, not withstanding the rash, and when she’s not itchy she feels good.

We are trying to remain optimistic that we’re still on course and that this is just a confusing detour. Everything has gone nearly perfectly so far, it would be heartbreaking to have to restart any portion of her treatment, and a nightmare to contemplate another transplant and the huge risks that that would entail. I’ll update again when we have news to share.

 

httpv://www.youtube.com/watch?v=_Ja5VaPXyTg

Post-transplant Work-up

/ Evan / 4 Comments

Today was Zoe’s 100 day post-transplant “work-up”, which means we shuffled through a long day at the Duke Children’s Hospital clinic subjecting Zoe to the same battery of tests that she received before her transplant. She had an echo-cardiogram, the “scream” test for pulmonary function, full blood draws and x-rays. We did not have to have another CT scan, nor did we have to do the nuclear kidney/liver function test. Zoe’s Creatinine and Bilirubin (markers of kidney and liver function) give us no reason to believe she isn’t recovering or fully recovered from her cyclosporine treatments.

The good news is that everything came back just fine so far. Zoe is a fine screamer, her pulmonary test came back better than it did pre-transplant, her xrays looked fine, and her blood counts were almost all in the normal range. The great news is that she did not need “G”, her WBC count is up to 5.6 on it’s own. She’s gone 2 weeks now without a boost, and the counts went up not down. We’re optimistic this means she’ll never need it again.

The visit felt very much like a cap to our experience at Duke. Most of the same tests, only this time with an eye toward making sure everything is fine so we can go home, rather than being fine so that she can be admitted and subjected to chemotherapy. The feeling was entirely different.

Zoe still has a bit of thrush, but given her newfound love of spitting out her medications, we decided to pursue the nuclear option to clearing it up. They gave her a purple dye-like medication that coats the inside of her mouth and throat and suppresses the thrush. The upside to this is it only takes 3 applications over 3 days, the downside is that it is really strong dye.

It’s permanent on pretty much everything except skin I’m told, so Zoe has to wear a little bib for the next few days, and everything she puts near her mouth will end up purple. As we were finishing up the application of it, the nurse lifted Zoe up to a sitting position a little too quickly and she spit up a bit. In a heroic attempt to save her clothes from the purple menace, I caught the spitup in my hand, and was rewarded with a purple hand for my troubles. The dress still didn’t make it. After that, we put on her bib and I gave up any hopes of joining the secret service.

There was a bluegrass band playing in the lobby for the duration of our visit, giving an interesting soundtrack to our Family Circle trek all over the hospital for our tests. There were definitely more smiles than usual everywhere we went, as a result. All in all, a great day.

Day +100

/ Evan / 10 Comments

It’s been an incredibly long year. We’ve largely set aside our lives as we turned our focus to seeing our newborn through a terrible, rare and unforeseeable disease and all of it’s consequences. When we began this journey, I honestly didn’t think we’d be where we are today. I didn’t think Zoe would make it through this nearly unscathed. I feared for her very life based on the statistics, feared she would develop a neurological disability, feared she would suffer from any number of other permanent side effects to her treatment.

Today, Day +100, I can say that it appears we have made it. Zoe is bright and healthy, eating on her own, off most of her medications, and officially an HLH survivor. There continue to be worries, but increasingly they stem from having an infant and all of the peculiarities that each child has, and not from having a sick infant specifically.

On Monday she is scheduled to get her central lines removed, which is another very big day. She’s had lines in her chest now since she was about 2 1/2 months old, the majority of her life. We’re hopeful that, once they are out, she’ll feel much more spry and ready to work on her crawling. As of now, she doesn’t like being on her belly for very long, the lines and clips dig into her chest a bit.

It’s a little hard to say where we go from here. Both Michelle and I are looking at life changes as a result of this, and both of us, and Maya, feel irrevocably changed by the entire experience. I wouldn’t wish it on anyone, but it has been an amazing experience in both the highs and the lows. We’ve found new ways to bond as a family even as we’ve been forced to spend too much time apart. We’ve found ways to grow as people as anyone might, faced with unexpected hardship. We’ve watched our girls grow up even as the world seems out of control around them, and come out of it, well, just fine to our amazement.

I started out writing this blog without having much idea of where we were going. It was, and is, a way to cope with what we’re experiencing. I’ve always been better at coping by writing more than by talking or exercising, drinking, what have you. When I began, I decided that the best way to handle things would be with a bit of anonymity, so that if something bad did happen, I was not writing something that could become problematic for the doctors and others working hard to help us. We have made it through the gauntlet at this point, and so I’d like to offer a few thanks. There have been so many people we have encountered who have helped even in small but important ways, and I can’t thank everyone enough. Please know that we appreciate all of you and all of your help more than we could even convey.

We’d like to thank Dr. Dave Anderson of Forsyth Pediatrics, the doctor who first saw Zoe and trusted his gut to send her to the hospital earlier than expected or “required”, and in doing may have prevented major damage from the disease. Dr. Mike Kidder, who couldn’t sleep at night while he worried about Zoe and worked on her diagnosis tirelessly. The ER doctor who happened to have seen HLH before and, we’re told, first suggested it on rounds when her diagnosis continued to be elusive.

Dr. Thomas McLean and Dr. Marcia Wofford, Zoe’s primary doctors at Wake Forest University Hospital, who managed rapid and effective treatment of her disease and her recovery, putting her in a position to go into her transplant in the best possible condition.

Dr. Paul Martin and Jayne Cash, the primary transplant doctor and nurse coordinator at Duke, both of whom suffered my nearly endless questioning, pushing and challenges as we tried to be sure we were on the right course. Their patience with us was boundless. And, as silly as it sounds, the World Wide Web for existing and allowing me to give myself a crash course on this disease, ultimately helping us make good decisions where, even a decade before, we’d have been in the dark.

The entire nursing team at the Pediatric Blood and Marrow Transplant Unit at Duke University Hospital, particularly Jess, Luke, Courtney and Tameka. The transplant doctors who we saw on rotation, Drs. Parikh, Prasad, Szabolcs, Page-Chartrand, and Driscoll.

The transplant families who found us through this website, followed our progress, cheered us and advised us both publicly and privately, particularly David, Andrea, Shana and Michelle. Your counsel and support were invaluable.

Our friends and family, you know who you are, but particularly Sandra and Larry, who have set aside their lives just as we have ours, moved into our home, put thousands of miles on their cars, and taken on care of our dog, Zoe, Maya, Michelle and I whenever we needed them. We never could have done this without you.

Day +68: Changes Afoot

/ Evan / 5 Comments

Zoe had a great day at clinic — or is having, I should say. We’re still here, getting the last of her Monday infusions.

WBC count was at 2.4, so she did get GCSF again, but as I mentioned previously it seems a matter of routine now, not concern. No one is worried that she will not eventually grow her own WBC more quickly.

The big news of the day is that we’re going to try to get off of TPN. Zoe has been eating more voraciously these past few days, particularly in the evening as the effects of the TPN wane over the course of the day.

We’ll be really pushing to get her eating as much as possible so that our trial run is successful, and she does not need to return to it (ever!).

In addition we’re moving to oral steroid (hydrocortisone), which the doctor indicated may accelerate her loss of cheeks and such a little more. We have the option of trying her off of the Zofran as well, which we’ll be taking. If she has increased spit-ups and nausea, we can always go back.

Next week if all goes well we can move her to oral Tacrolimus, which would have her completely off of IV meds, setting us up to get the lines out and a porta-cath in. That would mean real baths and even some pool action, which would be exciting indeed.

Dr. P stopped by to go over everything, and when I asked confirmed that her chimera results remain at >98%. He was very non-chalant, and indicated that he has no concerns about her graft whatsoever at this point. “It would be rare indeed” were she to have a graft problem with where she is at this point, he said. That’s a big relief, I’ve been anxious, constantly waiting for something to take a turn for the worse with that. I will (try to) stop stressing now.

All of this together means we’re one step closer to that normalcy thing I’ve been talking about. Get her to oral medications, eating on her own, lines out of her chest, and she’ll be much better off. I would say much happier, but honestly I’m not sure there is such a thing as a happier baby.

And that, friends, makes me happy too.