6 Month Studies

/ Evan / 10 Comments

Zoe had her 6 months post-transplant studies recently, I wanted to offer some of those numbers for families who are looking for another marker in where HLH kids might be after that amount of time. Obviously every child is different, and each has their own set of struggles that might be different from the next, but I believe that more publicly available information is helpful. I’ll go over some numbers, and then offer a reference point for them in parentheses so that you can see where she falls.

  • Ferritin: 95 (7-91 normal) – This is the marker that helped diagnose Zoe’s HLH originally, it was up around 5000 at that time; it has since returned to normal
  • Cortisol: .5 (5-25) – Her Cortisol level is low, and we’ve consistently had a hard time raising it; she receives Hydrocortisone multiple times a day, but were she to get sick in any way she would need “stress” doses to help her fight
  • Vitamin D: 15 (30-100) – Her Vitamin D level is also low, perhaps in part due to it being winter and in part due to her limited outside time, in addition to consequences of her treatment; she receives Poli-vi-sol vitamin supplements each day, but she will be starting a further supplement next week
  • FK-506: 4.4 (5-20) – Tacrolimus is a little low, we’ve been working to get it up without raising it too quickly
  • Magnesium: 1.8 (1.9-2.6) – On target, a little low perhaps; if you are a regular reader you may recall that Zoe takes Magnesium supplements due to consequences of her treatment and being consistently low until recently; restoring these levels naturally takes a great deal of time without an oral supplement
  • Sodium: 137 (134-146) – Good
  • Hemoglobin: 11.8 (10-13.5) – Good
  • Platelets: 327 (150-400) – Good
  • Bilirubin: .5 (.4-1.5) – Good; Zoe never had any danger with liver function that we know of, we are lucky; some transplant families have to watch this number closely for awhile
  • Creatinine: .2 (.1-.6) – Good; her kidney function appears to have returned fully after a scare pre-transplant
  • WBC: 8.1 (3.8-14) – Excellent! One of the items we all watch most closely post-transplant; Zoe has not dropped below 6.0 for awhile, we’re very excited about her stability here
  • Engraftment Analysis: >98%, no pre-transplant recipient cells detected; This means that, based on the test used, Zoe’s marrow is still greater than 98% donor, as it was at her first test post-transplant; We’re right where we want to be on this test
  • General Mental Status: “Alert, Smiling, Age appropriate in speech and responses”

Beyond that, developmentally, Zoe is on the cusp of walking. She’s been crawling for a few weeks, started cruising after a week or two of crawling, and is now trying to stand freely. She is babbling quite a bit, knows how to say Mama and Dada and Aya (Maya), as well as a few other “words”. As best we can tell, she is doing great. Her first birthday is on Monday, January 10th.

We’ve had quite a few bumps post-transplant, some of which have come and gone fast enough that I didn’t document them here, but nothing life-threatening or indicative of a return of HLH.

Our task now is to keep on keeping on: keep ourselves and our 3 year old illness free by avoiding iffy situations during cold/flu season, and thereby keep Zoe healthy; get her Cortisol and Vitamin D levels up, and maintain her other meds. In another 6 months we hope to be done with most of her current medications, though it is possible she may continue to need supplements. Go Zoe!

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.

Thankful

/ Evan / 2 Comments

Thanksgiving came and went in a flash this year. It seems like there is so much going on here, but each time someone asks I’m at a loss to describe where we are.

Michelle and the girls are back in the house now. Major renovations to make the house Zoe-ready have completed, and so they were finally able to return full time to our home. We’ve been living elsewhere out of suitcases in apartments or relatives’ homes for most of the year, and the State of the Family reflects that. Our clothes are spread far and wide, our linens and half the components of our house in storage or bags from the renovations, and our routines are in shambles.

We’re doing our best to relearn what it is to have all of us under one roof. Zoe was born and entered the hospital shortly thereafter as a 2 month old, and we have had little opportunity to settle our daily routines with her in a more “normal” state — a state that doesn’t include hospital stays, hours of meds, daily clinic visits and/or physical therapy, finally.

Despite the chaos, we are thankful. Thankful above all that Zoe appears to be making her way clear of this horrible disease, and that we’re well on our way through the long span of time where she has to be kept away from so much of life. Thankful that she is young enough not to remember any of this when we finally exit the tunnel. Thankful that our family is still intact, given how hard this year has been. Thankful for our relatives and friends for supporting us.

Zoe is doing very well right now. Her White Blood Cell count is remaining stable around 4.5 without any medications to boost it, and her other indicators are great. Her height and weight have finally returned to something more reasonable, she’s sitting right around 40th percentile for both now. Her height continues to climb in the percentiles over time, we believe she lost some growth time during her treatments and she’s beginning to catch up now. In time perhaps she will be able to make up for the months she has lost. Her weight was really high, then low after she came off her IV nutrition, and now she is on solids and it seems to have stabilized.

In terms of development, Zoe is moving along pretty well. She is not yet walking or crawling, but she is doing some scooching and little fish flop-crawls where she gets up on all fours, flops forward and then repeats to reach something. We’re excited that we may see some actual crawling in the coming weeks. It’s taken a long time to build up her core strength after so much time on her back and unable to develop her abdomen.

She is also getting increasingly verbal with her babbling. Michelle is convinced that her first word will be Mama from the sounds she’s making, but I like to tell her I think it’s going to be Grizzly, our dog. She’s doesn’t find that very amusing. 🙂

We haven’t had any more big scares lately, though we do see some rashes now and then that cause no end of stress until they go away. Right now she has a little bit of rash around her nose and under her eyes, but we believe (as do her docs) that it’s new foods and allergens being rubbed there by little paws. We try to keep on top of keeping her hands clean, but with babies it’s hard. She will rub food in her eye in a flash. Hopefully this will clear up soon so that we can stop jumping at shadows.

As Michelle and I begin to look forward, we’ve begun to start new creative projects. Michelle has begun keeping a journal online at a site called ClunkyShoe (it’s a little joke we have), where she is writing about her experiences and thoughts on family and life, with less emphasis on disease.

I have been putting occasional updates on my old blog at evanz.org, however I suspect some of my time may end up going to another site called HLHForum.com. I’ll be writing more about it in the near future, but in short I’m trying to provide a place for parents, family and folks interested in supporting patients with similar disorders to meet, discuss information, and hopefully support one another. I don’t want it to be about any one child, hence a separate site from this one. Please feel free to take a look if you are interested, I’ll be trying to update it more when I can find time.

I’ll continue to update this site whenever there is new news about Zoe, hopefully every few weeks or on special occasions.

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.