Home for Christmas

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.


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.


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.


Day +1: The Honeymoon

Zoe is doing well so far, much improved from early in the week. The transplant hasn’t changed much outwardly so far, we’re not seeing any changes in behavior, no adverse reactions, and as she recovers from the chemotherapy her mood has improved considerably.

We understand that typically kids have a honeymoon of sorts from the pain and discomfort of this process right around the time of transplant. They have a window where they are recovering from chemo and aren’t receiving more, but the transplanted cells have not begun to grow yet so the pain associated with that hasn’t kicked in. That’s where we are right now.

Zoe has been chipper and playful, and we continue to be amazed at how well she is getting through this so far. We’ll be waiting for word back on her cells each day, making sure she stays healthy and clear of infections, and trying to stay sane as we rotate in and out of the hospital to be sure Maya is getting time with both Michelle and I every day.

Each day we look over the comments you leave here and the emails, and it helps us feel connected during a time when we’re forcibly isolated. Thank you everyone for your support, it’s been invaluable.


Sorry about the baby talk, it’s easy to get carried away.  🙂

Day 0: Transplant Day

Last night I spent a lot of time talking to Maya about today, trying to prepare her. We knew that it would be necessary for her to wear a mask during her time here, and I wasn’t sure how that would go. So before bedtime, I took out the masks and gave her one, then put one on myself. I helped her get hers on, and she really didn’t fuss about it at all. She seemed to understand something was going on, and she was very attentive.

I explained to her that tomorrow we were going to go to visit baby Zoe for her new birthday, and she would be getting a new immune system. I told her that when people get colds or other things that make them sick, their immune system helps them get better, but that Zoe’s immune system couldn’t help her get better and so she needed a new one. Maya took all of that in very intently, and then asked if she could keep the mask to sleep with. We finished up our stories and said goodnight.

Maya during Zoe's Transplant

It always takes a little time to get her down to sleep, especially since we’re away from home. I went back in to check on her a little later, and she had all of her stuffed animals lined up on the bed, and she was making all of their boo boos better, one by one.

Earlier in the evening I had shown her how to kiss her finger, then touch the place where an animal had a boo boo, and told her it would feel better now, but I hadn’t given it much thought at the time. Coming into her room and finding her ministering to all of her toys was really touching. She had her mask on and was kissing her finger, then touching each one.

We all headed in to the hospital mid-morning, transplant was expected to be around 11:30-12. When we arrived, Zoe was all dressed up in her special dress ready for transplant day. The previous evening had gone reasonably well, she’s been improving slowly since our really bad days yesterday and Monday. A little bit of spitting up, but she was willing to nurse, and that seemed to make her feel much better.

Cord Blood Unit

Zoe’s transplant started at 12:30, and ran until around 1:15. Her procedure is called a Hematopoietic Stem Cell Transplant, or HSCT. She received a unit of stem cells obtained from donated umbilical cord blood from the bank here at Duke University Hospital. The HLA typing for her unit was a 5/6 match, 6/6 being a perfect match, 4/6 or 5/6 being typical for transplants. She also received pre-meds of Tylenol and Benadryl to help manage any fever or allergic reactions.

They say that transplants are really uneventful most of the time, and thankfully that was our experience as well. It took about 45 minutes, and we all sat around talking, taking turns holding Zoe’s hand, and playing fun songs. Zoe’s blood pressure went up as expected, but she didn’t get any fevers or allergic reactions, which can happen.

The transplant itself is more of a transfusion in terms of what people usually think of as a transplant. Most of us think of a kidney or other organ and an operating room when we think transplant, but Zoe received hers in her hospital bed where we’ve been this past week.

Zoe during transplant

Her mood was pretty good, all things considered. Not a lot of smiles, but she remained awake the whole time despite the Benadryl, and wasn’t fussy or upset. We understand from the team here that kids often have a bit of a transplant honeymoon for a few days where they feel ok, then they may have some pains or fevers, and it can go up and down a bit until she has recovered.


From here on we’ll be watching cell counts every day. We expect she will remain at “<0.1” for awhile, but we want to see white cells grow. You’ll probably hear “Grow cells grow!” a lot from us.

As you probably noticed, we’ve been counting down the days from -23 to today, Day 0. Tomorrow is Day 1, and we’ll count up from here. Our goal is to see her cells graft and grow, counts to begin going back up, and then around Day 30 we’ll do a test to see what percentage of her cells are hers and what percent are donor cells. Assuming everything is going well, we’ll be looking toward Day 100 at that point, when we can return home.

It’s a long process. First Zoe’s life had to be saved from the active HLH, which thankfully happened quickly. Now she has had her conditioning and transplant. Next, her new immune system has to grow and we have to keep her healthy. After approximately a year, one year from today, she should be healthy enough to resume a normal life. Thankfully she is young and won’t miss the malls or crowded restaurants or school that she can’t go to but would miss if she were older.

For our family, it’s another step along the way toward being whole again.