duke

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

Home for Christmas

/ Evan / 6 Comments

What a year! Our life has been completely turned upside down by this disease this year, and I’m not sure we’ll even understand how much things have changed until we can look back on it.

We began the year with the birth of our second daughter, Zoe Elise, and very quickly it took a turn for the worse. When Zoe was nearly 2 months old, our toddler brought home a nasty virus from pre-school, and it made it’s way through our family until finally, after holding out for a week, Zoe started congestion and a mild fever.

Within a few days the fever began to be noticeably persistent and other warning signs began to appear, and through a back and forth with our Pediatrician, who insisted, we took her to the hospital. What began was our nearly year long ordeal with HLH. It took several weeks to bring the disease under control, but thankfully she was able to be diagnosed incredibly early, which we now know probably saved her life. The longer HLH goes undiagnosed, even a matter of weeks, can result in damage to the body that is hard to recover from.

Zoe had her early treatment at Wake Forest University Hospital and then was released for a brief window as she was prepared for her lifesaving Stem Cell transplant to replace her malfunctioning immune system with that of another child via umbilical cord blood stem cells.

Over the summer she endured a preconditioning regimen of chemotherapy to destroy her existing immune system, received her transplant, and spent the waning days of summer in a hospital bed being fed through a tube on a pain management system.

By the fall she was released, doing well so far, and we began our stay in Durham where we were required to stay close to Duke University Hospital for her constant clinical visits. After a time there, just as Winter was ready to begin, we were released to go home.

Everything about this process has gone as well as it could for us I believe, given the possibilities. We are lucky. Extraordinarily lucky. There are families who I follow regularly now who continue to endure aspects of this experience, with HLH and similar disorders, as their children literally fight for life. My heart goes out to them every time I check in. I’ve teared up many nights as I read an update online, posted at 1 or 2am, knowing what it’s like to be sleepless and helpless in that hospital room with a child struggling nearby and machines beeping all night, like the tap tap tapping of a raven. It is one of the many, many, “secret” diseases that families endure when struck by them, but which the greater public is largely unaware of.

By now, we are beginning to see what life might be like next year, a year that we have high hopes for. We are looking forward to our older daughter, who has grown up so very much this year, returning to school, which she can’t yet because of the risk of disease. Just this morning over breakfast she said “we’re all back together again Daddy”, which she says often now that we are. She had her own hardships this year, we know. Hopefully they’ll give her character, and she is certainly deeply attached to her sister already.

Zoe for her part won’t remember much of this, but she will have lingering effects, a sort of PTSD. She is already very nervous when men she doesn’t know (in particular) lean in toward her, she panics and start crying in a clear response to the doctors who she must have learned to fear. People in white coats tend to set her off when they enter a room. It’s heartbreaking. But, she is alive. She is getting healthy. She is growing. We are grateful.

This Christmas is special for all sorts of reasons, but it will be memorable for us as a turning point in our lives as a family. Among our gifts this year was a special one I wanted to share.

Maya’s Great Uncle Charles took particular interest in how she was doing this year, he seemed to see that she might be having a hard year being taken out of school, transported all around and kept from a normal childhood routine as we worked through Zoe’s disease.

He commissioned a wonderful doll house for her(and for Zoe when she is older) from a local crafts person, which we received on Tuesday just before Christmas. Maya was ecstatic, as were we — it is really an amazing gift and will certainly be an heirloom. I wanted to post a little video we shot of it and the person who made it explaining it to her by way of thanks. We, and (more importantly) she, will treasure it.

httpv://www.youtube.com/watch?v=5vcyVWiHrjg

With regard to Zoe’s medical particulars, let me offer a quick update on that front. Her WBC counts have climbed a little more, she is now holding at 8.1. Her last was closer to 6, and she seems to be holding in the low normal range now. Her Creatinine, Bilirubin, Sodium, RBC, Platelets — all are normal.

The GVHD rash she had recently has passed, and though she immediately plumped her cheeks back up in response to the steroids she had to restart, we are relieved that it went away without major consequence. We’re weaning her off of the steroid again even now. Her Tacrolimus level had been low recently, perhaps in response to her metabolic changes while growing and getting more physical, and we suspect that is a contributor to the rash.

With regard to her physical development, she is moving right along. She started crawling within a week of my last post, and she’s already beginning to cruise a bit (moving along things while standing). The night before she started crawling in full, when we could see she was close, Michelle, Maya and I all started doing laps around the den on our hands and knees in a big circle around Zoe. We like to think we inspired her 🙂

Her strength has really improved and she’s into absolutely everything she can find, as she should be. Her sister follows her around the house excitedly and loves to have her nearby when playing. Zoe is in the 50th percentile range for both weight and height.

httpv://www.youtube.com/watch?v=Y2m1Ow2y8uM

I’d like to wish all of you a Merry Christmas or Happy Holiday. We have really loved getting to speak to so many new people and meet wonderful families this year, despite all of the hardships. Your support in comments and advice both privately and publicly has been a boon to us, and we thank you.

httpv://www.youtube.com/watch?v=eFsyj9SfWAc

Reality Check

/ Evan / 1 Comment

These past few months we’ve found that it’s really too easy to lose sight of the reality of this disease when things are going well. I suspect it’s due in part to how hard things have been when Zoe had an active disease and in the 2-3 months post-transplant when she was in pain or discomfort often. Once those times pass, all we want to do is forget the bad.

We had a bit of a wake up call these past two weeks. Zoe developed a bit of a rash around her mouth and nose, and at first we were convinced it was a slight food allergy. Even the doctors felt it was nothing to be concerned with (yet). Another week went by, and instead of disappearing, the rash spread slightly and began to make us very nervous. We took Zoe in to her home hospital, Wake Forest, and after some consultation decided to take her straight back to Duke to decide what to do.

At Duke we determined that Zoe does have some slight GVHD, which is very distressing since she hasn’t had any worth noting this entire time. We’ve begun treating it with Prednisone, which is one of the steroids we had hoped never to see again. While on the one hand the steroids saved her life, on the other, all of the worst side-effects Zoe has had to endure are the result of the steroids. Our goal is to nip this and get her back off of it as soon as possible. Zoe is days from crawling we believe, she is crawling a couple steps here and there already, a little more each day, but a return to steroids could change things.

Turn your head for a moment and anything can happen with these two

Zoe is also getting a little Nystantin cream for a couple of spots on her skin, and some Nystantin liquid for her thrush, which is still hanging on and never seems to go completely away.

Zoe’s spirits are very high despite these issues, and she is growing well — she’s up to 67th percentile for height now — so it’s possible the GVHD will only be a minor speed bump in her progress. Having her do so well for so long then have the rashes appear is certainly disconcerting though. Hopefully it’s not a sign of anything major.

We have another milestone visit to Duke coming up, we’ll see how things look then.

Update:

After a few days on the steroids, Zoe’s rash has cleared up nicely. She’s in good spirits and we’re back on track we hope, we’ll know more at our next milestone clinic visit.

30 Days of Light

/ Evan / 3 Comments

We’ve now gone 30 days since Day +100, and things continue to slowly improve. Our last few visits have seen Zoe’s WBC counts between 5.5 and 6.0, holding fairly steady. That puts her in the low normal range, and she hasn’t had any growth medication since that last time.

The biggest improvement overall has come in the form of a medication, one that we wished we had started months ago, called Reglan. It’s a bowel treatment medication that suppresses spit-ups and speeds along digestion. Zoe has had the worst time transitioning to solid foods while still healing from her chemotherapy, and as a result we’ve really struggled with her ability to keep food down.

Since the introduction of Reglan, however, she has had almost no major incidents and has gained some weight and is growing consistently. She began this medication after we pushed for a solution to her bowel issues, which is why I mention it. I’m not sure it’s standard treatment in these cases, but the results we’ve seen have been fantastic. I know I sound like a commercial, but this was a really big leap forward for us and I want to mention it in case others have this issue.

We have finally left Durham, and the girls and Michelle are currently staying in Asheville with family while some work is done on our house. I’m back home for the moment trying to make sure things move along with the renovations — we had to have some things done to our older home before Zoe could safely return.

It’s been a huge relief to leave Durham, we had really begun to feel we’d forgotten what it was like to be anywhere else. We are grateful for the “clean” apartments set up by the Evanosky Foundation for the use of transplant families, but as nice as they were, they weren’t home. Another couple weeks and we hope to all be in one place again.

Zoe is progressing well developmentally. She’s not crawling yet, and we’re beginning to think she may just skip it. My mother tells me I did at her age, so who knows. Zoe is able to stand when held or against something supporting her for decent amounts of time now, but she is also rocking on her hips a lot and scootching around. We’ll see soon what path she decides to take.

All in all, things are progressing well. With the continuing construction on our house and the lateness of the year though, it’s quickly becoming clear that by the time we’re all back together and healthy, this entire year can be written off to getting Zoe through this. It’s better than the alternative of course, but it’s still hard to believe the year is almost over and we’re just now on our way home. If it weren’t for writing it all down, I’m not sure I could tell you exactly what all happened, it’s been such a blur.

PS – Please excuse the horror movie reference in the title, but it is Halloween, after all.