wfu

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.

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 +82: Talking About Release

/ Evan / 4 Comments

Today’s clinic visit brought interesting news, there was talk for the first time about a release date. Release at this point means being allowed to return home and switching our long term care to Wake Forest University Hospital (Brenner). They suggested we could be released as early as two weeks from now, but I don’t think that it’s likely to happen that soon, mostly for our own reasons.

Michelle and I want to be sure, absolutely sure, that before we leave Duke’s care and the care of the doctors who now know Zoe best, that she has no dangers remaining, beyond the normal risk of infection. We want her graft to be firmly in place (it is), her counts to be at a stable level (not quite yet), and more than anything, her lines to come out.

Right now we’re having a very messy time with her feeding and medication. Zoe came off of the IV nutrition very recently, but we’re still struggling to get her intake up to where she is supposed to be. She has lost a little weight — which is probably a good thing — but we don’t want her to lose any more.

Generally at this age kids are supposed to be taking in around 30oz per day, however Zoe is closer to 15-20 most days, not including her meds. Her meds add another few oz, but the concern remains that she hasn’t found her groove yet with feeding.

In addition, her meds are a mess. We have a terrible time getting her to keep the oral meds down, but the only way to get her lines out is to go on all oral meds. A conundrum. We’ve asked to have her go back on Zofran for spit-ups in order to try and keep more of her fluids and oral meds down.

In general, her age is such that she has suddenly found her voice (fussing more than she used to), her strength (she yanks at everything, swats away syringes, etc), and her will to drool. Suddenly nothing can be easy, as if it ever was.

If I stand back, I begin to realize how far we’ve come. We’re talking about going home at some point in the next month or two, and that wouldn’t happen if she were in serious danger. Zoe’s growing up really fast now, someone opened the gates while we weren’t looking and she is off and running.

These are great things, but somehow, no matter how many steps forward we take, there still seems to be something to fret about.

I suppose at least the things we’re fretting about now are much more reasonable than life or death. I have a feeling that, once these few weeks of rough travel pass and Zoe has learned to eat her solids and her med situation is stable again, the rest will sort itself out.

It’s very hard to shift out of emergency gear though.