hlh

Life Goes On

/ Evan / 2 Comments

Update: We have had a minor setback on the site, there was a virus problem with the software used here, which forced us to wipe out the website and restore everything to sure that it was fixed. Some pictures and other features are currently broken but will be updated when possible. Apologies to anyone affected.

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Where have we been? Well thankfully, we’ve had little bad news to report, and for some reason remembering to report the good falls by the way at times.

Zoe has been doing great. She continues to progress toward weaning her remaining meds, which at this point are her tacrolimus (.6ml) and hydrocortisone (1.0ml) twice daily. She has very dry skin and rashes some days, other days is perfectly normal, but the worst of it for us remains the rashiness. Could be far, far worse, and we’re grateful for where we are and how far we’ve come.

Our only scare since the last update was a high fever incident, attributed to an unknown virus “going around”. Zoe had a bad night one night as kids do when they’ve caught something, but this time we were able to get a 104° temp at home. At that point we felt it was safest to head into the hospital.

Zoe spent about 24 hours in-patient to be monitored, but her fever broke fairly quickly the next day, and we were sent home. Scary, but we’re taking it as another sign that she is doing well to fend something like that off.

Beyond that, in the recent months Zoe has remained in preschool steadily two days a week, only missing occasional classes to fend off a cold. She’s fought off several mysterious cold-like events on her own. She also turned two and had a birthday with her first real party with her preschool classmates and her sister’s friends, which was a blast.

Our only other HLH related news to report is that we were contacted recently by CCH regarding the particular genetic variation that they believe was the cause of Zoe’s HLH. We are still waiting to hear more about the request, and I may have more to share later, but the gist is that they believe they’ve made progress toward being able to more confidently diagnose kids with this variation. This could help those coming along to have more surety in knowing if a transplant is needed, so we’re hopeful it represents progress in some way. More on that when we have it.

In other news, as a Christmas gift to Michelle (and the girls), I commissioned a painting by Michelle’s favorite local artist, Stephen White. It was intended to be a commemoration for Zoe (and all of us) having survived this horrible disease, and a reminder of how close it has brought us.

We continue to hear from and about other children who are struggling to survivie HLH, and our hearts go out to them. It is our hope that Zoe’s story can be one of many survivial stories going forward, and that in some small way she offers hope to those now embarking on this journey.

 

 

 

Surviving Summer

/ Evan / Leave a comment

It’s been a roller coaster summer for us, but we’re feeling pretty confident going into Fall and School season. Zoe had took a few steps back and scared us a bit after a combination of overconfidence and overexposure to sun led to the Very Rashy Beachtrip.

Still, she recovered fairly quickly from that setback, and has since resumed the drawdown of her meds as we continue to work toward weaning her from the big ones. As of now, she continues to take Tacrolimus (and use a Tacrolimus ointment for spot treatments), Prednisolone (nearly done), Hydrocortisone daily, with Septra on the weekends. At least two of the four cause sun sensitivity, which has made for an interesting balance. And, of course, just as we think we’ve got it perfected, Summer is nearly over.

School is a dilemma for us right now. We very much want to get Zoe started, and she’s on track to begin pre-school in a few weeks. We’re going to play it by ear, and thankfully our pre-school has been incredibly patient and gracious with us, saving a spot for she and Maya when needed, and allowing us to step out when necessary during this past year and a half.

Zoe was diagnosed at 2 months, and as such she’s never really had vaccinations that a typical child would have received several times by her age, a year and half. As such she’s going into school essentially relying on the other families to have vaccinated fully to avoid getting sick. It’s a scary thought, and something we’ve worried about quite a bit, but the alternative is to keep her out for another year.

Instead, what we’re going to do is get her started, and then likely keep her out during the height of flu season until she can get her vaccines. She will almost certainly get sick — Maya did when she started school, I suspect most kids do when thrown into the germ pool for the first time — but Zoe’s immune system seems to be getting sturdier. We have to hope she can weather it.

Just us puppies here

Developmentally, she’s a fireball, and we had nothing to worry about. She’s decided she wants to start potty training almost a year earlier than her sister did. I guess that’s what having a big sister will do, she has so much to watch and emulate. Her vocabulary is growing daily as it should, and she’s stringing words together a little bit now, “nigh nigh daddeh”. She is sitting still for stories more and more, and she wants to be shown how to do things, block stacking, sorting. She’s taken to picking anything she finds on the floor and running to the trash can to throw it away now that she has learned to do that. I’m just waiting for my phone to start ringing from there.

Medically, Zoe’s labs have held up so far. She remains at >98% graft, and her ferritin and WBC counts show no indication of a return of HLH. She just needs to get through the GVH rashes without a serious incident, and we may yet make it through this. We’ve gotten fairly adept now at when and how much she can be outside, so she is able to make it to the pool, on hikes, and on car trips without being overexposed if we toe the line properly. It took a long time to get here though, it seems like.

In the end, we feel really good about where Zoe is. We continue to have our scares, but once we get through them things seem relatively OK. She has survived the transplant, survived at least one cold since then, survived a semi-serious series of GVH rashes, and she is still ok. Now, for the big one: School.

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

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.