Day +100

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 +13

We had a visit from Dr. S yesterday afternoon, and it was very positive overall. Zoe was feeling good and smiling, and we were able to discuss her status.

Her WBC was 0.1 yesterday, but on Mondays they do a manual differential, which means that they actually look under a microscope and count the cells. The daily cbc is done by machine. So they actually saw white cells under a microscope, no possibility of machine error there. Dr. S felt that we are exactly on track for the start of cell growth, maybe a few days early — day 14 he said was typical, we were day 12.

We talked about the Reduced Intensity Conditioning study that Zoe is on, which Dr. S heads, and so far they have used this protocol with 7 other patients. Only one of them had any notable GVH, most of them had very very mild if any at all. Zoe has had a very mild bit of GVH on her cheek (the redness seen in recent photos), but he felt it was at best a 1 on the scale, so not a concern.

All 7 of the kids they have used her protocol on have had successful grafts. Very very good news. They feel that Zoe is shaping up exactly as expected, which in medicine I gather is a great thing. Wonderful news.

Zoe’s mild tissue breakdown in her mouth from mucositis is healing, as is her bum. We haven’t needed the “open air” strategy or any special medicines for it for a couple days now, just her cream.

The WBC count today was again 0.1, which means we seem to be holding steady at the moment with some cells. It will likely go up and down, what we’re hoping for initially is 0.3 or so for a few days, that will indicate some stable progress with cell growth.

Assuming things continue as expected from here, we’re looking at a nice long stretch of waiting while Zoe’s cells grow. We may see some increased redness from GVH if the cells rush in too quickly, but it’s not anticipated. Zoe may have a higher chance of that because she is the youngest on the study, and she got the most concentrated cell dose, which is a good thing in most respects. It does mean her cells may grows fast enough to generate a reaction, however. We shall see.

Zoe has been feeling well enough to start playing with the bottle again. She’s been unwilling to take it for more than a moment, we assume due mostly to mouth pain, but it’s a good sign that she’s willing to take a little now as she’s healing.

Day +11: Happy Fourth!

Zoe is doing well today, much improved from yesterday I would say. She is sleeping the day away, and that’s probably for the best. Whenever she is awake she has a hard time getting comfortable, and she has a hard time being held for very long, so it’s not easy to console her.

No uptick in her WBC counts today, sad to say. Everyone here is confident she’s begun engraftment, however. She’s still flushing regularly, skin very warm to the touch at times, and her temperature is staying around 99.5.

We did up her pain medication late last night after having a hard time getting her settled. She is set up on a PCA machine to automatically dose the medication, with “bumps” of it for rough patches if needed via a button. Zoe doesn’t react well to the button, however, she gets very itchy at the sudden higher dosage, then it fades and she is in pain again, so we tend to increase the drip instead. It’s a smaller amount than the button, but it keeps her more at ease when it’s consistent.

Still Cheeky

Zoe’s creatinine came back 0.1, which is suspiciously low given yesterday’s 0.6. Dr. S felt it unlikely that’s accurate, but it does seem likely that it has gone down not up, so we’re ok there. She continues to respond really well to the Lasix, so her weight is stable.

Which is all a long way of saying we’re holding steady. All is well, she’s doing what she’s supposed to — growing a new immune system.

Have a great holiday!

Day +10: The Worst of It (We Hope)

Things are a little tense here at the moment, we’re trying to get through what we hope is the worst of the entire process. We’ve been told for awhile now that around Day +10 we’d see the worst of the mucositis, the worst of the post-chemo struggle, the worst of the fluid retention. That seems to be where we are now.

Zoe's Lounger

Doctors are still feeling very positive about Zoe’s progress. She’s handled everything really well, and her mucositis has not been as bad as it could be by any stretch. She doesn’t have any sores in her mouth right now, just redness and irritation.

She is definitely uncomfortable though. Her pain medication (Fentanyl) has been upped a little more to help her get through this phase, and that has helped with both her blood pressure and her comfort.

The primary concern at this point is the fluid retention. Zoe is noticeably puffier, and we’re trying to let her spend as much time as we can in an upright position to make sure fluid drains well and she can breath without supplemental oxygen. Her oxygen saturation levels are still good, so we are keeping fingers crossed she’s not going to need a tube.

Zoe’s Creatinine, which is a marker for kidney function, has gone up to 0.6, which is significantly up from the 0.2 or 0.3 where it’s been stable these last weeks. We are assuming that that is because of the Lasix she is getting for fluid retention, but we haven’t heard confirmation of that just yet. There are other options for fluid retention that are less hard on the kidney, so we may see a switch to one of those today. We’ll know more later on, we tend to see the docs around 4-5pm most days.

If this is the worst of it, and it is the worst so far at least, then we will be relieved. If on the other hand things continue to get worse as they have these last few days, then we’ll be more nervous. I know from reading the stories of other families that getting through this period is really hard but that it does end, so we’ll just try and keep our eyes on that end of the tunnel.

Almost there.

Update: Feeling a little calmer after a visit from Dr. S. He was excited when he saw her, where I have been very nervous. Turns out he feels that her current condition is the beginning of engraftment — fluid retention, pink-ish flush on the skin. This is a very good thing if he’s correct, and I trust he is. He was not concerned with anything, he felt Zoe doing well since she does not need oxygen at the moment and is flushing out the fluids well so far.

Flushed & Feeling Yucky

Fever rash engraftment, fever rash engraftment — say it as fast as you can, try to keep that in mind”, he said.

We’re likely to see fevers, which can be quite severe, and skin rashes which usually start on the hands if she has them. The rashes are signs of GVH, or graft vs host, and are expected to some extent. There are multiple variations of GVH, most of them mild.

So. We’re upping her Lasix to 3x a day to keep ahead of the fluid build up, we don’t want to “fall behind” on that I’m told. We have canceled her antibiotic, however, since her line cultures are all negative and that medication is contributing to her higher creatinine.

The nurse coordinator who brought us in and took us step by step through the first stages of this stopped by yesterday, it was good to see her. She’s been following Zoe’s numbers each morning, and she was very encouraging.

As she started to leave after we had spoken for awhile, she turned and, smiling, said, “Don’t hold me to it, but I think you might get a little July 4th present. I think we might see a tick up in Zoe’s WBC count.”

Let’s hope she’s right!