Life Goes On

February 2nd, 2012 § Comments Off § permalink

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.

 

 

 

Change of Address

November 3rd, 2011 § Comments Off § permalink

I’ve recently moved this site from its original home where it was part of my personal blog, to its own address at HLHJournal.com. Nothing should have changed substantially, all of the content found there is now in the new location, all links should be intact. I have also updated the look of the site somewhat to give it a cleaner presentation, and to put more of the posts and pictures on the front page.

In addition to these changes, I have added a community forum. This feature allows visitors a place leave a message, information, or a personal story or anecdote. Simply register at the right of the page with an email address and name (pseudonyms are fine), and leave a message.

I have received numerous contacts in these past 20 months from parents and relatives of children with HLH. Without exception I appreciate these contacts and welcome the opportunity to answer questions and try to help in some small way parents who are trying to work through what Michelle and I have gone through already. We know how hard it is, and we appreciate those who were there to answer questions for us.

My hope is that visitors to the site will continue to feel free to contact me directly via email at hlhjournal@gmail.com, however anyone who wishes to leave a public message for others should do so as well.

If there is new research, new procedures, new data of any sort that might be useful, this is one place to post that information where it is likely to be seen by newly diagnosed families searching for answers. If anyone is aware of programs which offer financial assistance or other types of support, that sort of information is also very useful, as going through treatment often requires one or both parents to cut back on or stop working for a time.

There is also a place for families to post their stories where others may see them, shouldthey wish. Currently many of these stories are scattered about the web or hidden behind walls on sites like CarePages, which is fine, but my feeling is that there is also a need for newly diagnosed families to be able to read about others’ experiences.

As always please contact me at hlhjournal@gmail.com with any questions or suggestions.

A Scare-free Halloween?

October 20th, 2011 § Comments Off § permalink

Fall is here, and everything around the house with two young girls is about pumpkins, cupcakes and costumes. The skeleton on the door has a bow in (her?) hair, and plans for turning the little path in our yard into a haunted stroll are well under way. Things are more calm than they have in a long, long time. I believe I had forgotten what calm was like.

Zoe has continued to grow up: she runs, she jumps, she howls, she colors, she tries to do everything her sister does, and she never stops moving until she collapses (or we do). And, as she has grown we’ve continued to make steady progress with her health.

In the past two months we’ve discontinued her Prednisolone and her Septra, two of her worst remaining meds for very different reasons. The Septra because it makes her highly sun-sensitive and rashes continue to be a battle for us, and Prednisolone because it affects her natural growth rate, moods and weight. It was a tremendous relief to cut first one, then with no adverse effects becoming obvious, the other.

There have been struggles, nevertheless, to remind us that we have to stay vigilant with her. She has had a couple of outbreaks of MRSA, something that seems to come up now and then, and she has had rashes. First it was the sun sensitivity rashes, then sniffly nose dripping on the face rashes, then something-in-the-fall-air rashes, with a smattering of not-sure-why rashes. So far though, nothing approaching our big scare over the summer that forced her back on to the steroid, and nothing we have been unable to treat with her bevy of creams and ointments.

Zoe has begun school, and that has been a big adventure for her. We’ve kept her out several times when other kids have serious symptoms or when she is not completely healthy, but she’s gotten to attend enough to fall in love with it. She sees Maya at the door getting ready in the morning and she runs off, grabs her ladybug backpack, and charges out the door to wait by the car to be loaded in, howling and giggling all the way. This routine took a matter of days to perfect, and we’re thrilled that she has taken to it. We had some concerns, given her closeness with her Mom this last year, that she might have separation anxiety. Not this one. In the classroom door and ‘bye bye Mommy’ she goes.

The other milestone we’ve seen of late is Zoe’s first vaccination. Due to her suppressed immune system, she’s been unable to receive the typical doses children her age receive, and instead has been protected by her medications. She got her flu shot a couple of weeks ago though, and so far so good.

I have been told by several people in several ways, “these kids are amazing”, over the past 1.5 years, referring to the transplant kids, and it couldn’t be more true. Zoe sat and watched the nurse give her the shot, and barely budged — no crying, no resisting –just curiosity. As I watched her I had a little action movie script writing itself in my head:

“What’s that thing, some sort of needle? pfft,” Zoe says, pretty eyes laughing at the thought, “I’ve seen worse lady.  Bring it on.”

 

 

Surviving Summer

August 22nd, 2011 § Comments Off § permalink

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.

Two Steps Forward, One Step Back

June 15th, 2011 § 3 comments § permalink

It seems like the nature of treatment that we are never able to quite get clear of things before we have a setback. We progress, things improve, and then something happens and we are back where we were a few months ago in terms of medications and precautions. It IS improvement in a big picture sense, but it’s bittersweet and stressful day to day.

Zoe is back on track now, and our worst fears have been put to rest. She does not have a recurrence of HLH, her graft came back at >98% again, the same as before and a clear sign that her transplant has taken and HLH is gone (forever we hope). The fear of a return of HLH was due to a rash that appeared very similar to an HLH rash, but the chimerism result reassures us that is not the explanation.

GvHD Rash before treatment

She does however have GvHD, which has required her to restart Tacrolimus and go on a short term run of Prednisolone, the old nasty steroid, again. Why she has GvHD was a big point of confusion to us given her chimerism results, so I will do my best to explain briefly.

Zoe has a new immune system, and that immune system is completely donor material. This is the result we want, since her old immune system was dysfunctional and susceptible to HLH. Her new immune system however has not yet come to terms with it’s new home, meaning her old body is still considered a threat to it to some degree. When left unsupressed, her new immune system attempts to fight her old body, causing the rashes and other GvHD symptoms.

We had gotten her off of almost all of her meds slowly over months of weaning, when the rashes started to appear. It seems that she is not quite ready to be off of them, so we have restarted the routine and we’ll see where she is in another 3 months or so.

There is a question of whether or not she will be ready to enter pre-school this fall, but I’ve been told by reliable sources — Zoe’s two teacher-grandparents — that she is too young to need to be in school anyway. Yes it was what we had planned and yes it would be helpful, particularly socially, but it’s not going to hold her back to wait another 6 months or year to be safe.

In the meantime, she is back to looking good and feeling good most days, which is a relief. We are going to finally be able to remove her port despite the complications of these past weeks, so that too is progress. At this point I’m wishing that we had started putting little patches on Zoe’s well-worn diaper bag for each medical scare, not unlike notches in a gunslinger’s holster or emblems on a jet fighter. She’d have them racked up by now.

 

 

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