No News is Good News

IMG_0272No news is good news, it’s trite but true for us. I have not written here recently because in the past, I’ve tried to time updates to medical news. Thankfully we haven’t had any developments to share.

For those who have followed our story, Zoe is doing wonderfully. She’s begun her 2nd year of preschool now, has made many friends, been able to participate in play dates, beach trips (in full sun!), and everything else any family might do. She is strong, bright, curious and feisty, and has no limitations that we have discovered that stem from her transplant or the steroids and other medications required to survive HLH.

With regard to HLH Journal, I have not yet decided how to handle the future of this site. I would like to provide more opportunities for those who find their way here to connect with and support one another directly, something I am investigating. I would like to provide more links to information and support resources, something I need to spend time researching so that I can be more comfortable with what I share.

At the same time, I don’t want Zoe to feel forced to contend with pictures or stories of events that she barely recalls as she grows older. My current plan is to leave the information and pictures here intact indefinitely, but to gradually curtail the new pictures I share so that there is at least some public distinction between Zoe’s experience as a very young child, and who she is as a young woman.

IMG_0486HLH Journal continues to rank very highly for certain web searches for a number of reasons, but due in large part to the scarcity of information about the disease when I began writing. As such I will try to guide the site more to a general perspective as we draw away from a life of daily contention with HLH.

I continue to receive notes and comments from people all over the world who have discovered this site and and are beginning their own difficult journey. To those who write and comment, thank you.

I know how terrifying and confusing it can be, and I wish I were able to offer more to you than what I’ve shared here and some kind words or advice. Please know that our heart goes out to you as you take your journey, but that it is not a hopeless one. HLH can be beaten.


Another Year

It seems another year has gone by, this our first in a long time that hasn’t seemed completely overwhelming. The first half of the year saw us dealing with a few remaining scares, some testing, and continued stress, but as summer came and passed, the load began to lighten. Now we are looking up and realizing that there is a road ahead that may not be lined with hospital visits, and we are grateful.

Zoe’s health is, to put it simply, wonderful. We feel extraordinarily lucky to be where we are. So many families continue fight to get their children and loved one through their illnesses, or mourn their losses. We are acutely aware of how fortunate we are to still have Zoe with us, much less for her to be as healthy as she is.

If you recall the last medication that Zoe was taking earlier in the year was her Hydrocortisone supplement, which we have now successfully cut. There was some question of whether she would always need to take a supplement, as some patients’ bodies never return to making it in sufficient quantity, but we have. Her minimum level needed to be over 5.0, and it’s now holding at 8.0 naturally.

She has continued to grow, though it is still too hazy to tell whether or not her pace is on track to return to ‘normal’ or if she will endure some loss of height. She is currently slightly smaller than most children her age, but she is still so young, just about to turn 3, that she has plenty of time for some growth spurts to occur. A minor concern, in the scheme of things.

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Zoe’s energy level is astonishing, she is even more active than her older sister was at this age, and that’s saying something. She also seems to have a resilience that I don’t see very often in kids her age. She shrugs off knocks and tumbles and pains of all kinds, rarely crying about them. I like to attribute it to the amount of difficulty and pain she has had to endure having raised her threshold for pain in general.

There are days when she bursts into a torrent of words or a song with a big smile, twirling around or bouncing up and down, and I just tear up spontaneously. It’s hard to know how much these things affect you in the midst of them, but as time passes we are able to see.

Every parent must have their moments of awe. Mine are always tempered with an awareness of what might have been, and that emotion strikes deeply.

From here we move ahead. Zoe is turning 3 soon, with a full two years of that life consumed by the process of curing her disease, but with so much life ahead of her now. She has been in school now for a year, and has been enjoying it so much that she will move to full 5-day weeks of school this spring.

Tempus Fugit

Summer is here, and it’s hard to believe so much time has passed since our last update. Sorry! As time passes it becomes harder and harder to revisit the memories of our experiences and write about them here. In time I’m sure that will get easier.

First, a brief note about pictures on the site: in order to make available many of the pictures lost when the site was hacked earlier this year and had to be recreated, I have added an image gallery. There should a menu at the top with many of the photos that were originally available. This is meant for families who might be interested in what to expect as treatment progresses, good and bad.

As for us, well we’ve had some bumpy weeks and some fantastic weeks, but we are still moving forward with our two happy-go-lucky girls. Zoe is growing, happy, and looks great. Her >98% graft remains intact and we have no reason to believe that will ever change at this point.

We have had some ongoing issues with her skin, many of which have been documented here, but until recently we have had little understanding of the whys of what’s been going on. We’ve thought that it could be HLH rashes, sun sensitivity, MRSA and allergies all at one time or another.

A few weeks back we had another rather nasty virus go through the house starting with Michelle and Maya, then Zoe. When Zoe got sick she was fine at first, but this particular virus carried a skin component (some itchy red spots) that were benign in most of us, but set off a severe skin reaction for Zoe.

We spent 4 days in the hospital when the breakout happened, it looked very similar to Chicken Pox and because Zoe has not been vaccinated against everything yet (she has been unable to), we had to be safe. After a matter of hours she was covered nearly head to toe in red bumps, and the hospital did a full work up on her to figure out what was going on. In the end, what came back to us was a virus (the bumps and fevers), and a type of Eczema or dry skin.

At this point Zoe is fully recovered, but we have moved past thinking that this is all sun sensitivity, and are looking at treating her for Eczema and a few allergies only. Things could certainly be worse, and as usual every little scare sends us back to dark days, but we have been assured that there is no reason to fear anything major at this point, we simply need to take extra care of her skin.

As far as treatment goes, the last remaining medication Zoe is on at this point is Hydrocortisone. We’re working on weaning that even now, but everything else has been discontinued. We appear to be at the end of the ordeal by and large, and now we need to keep her healthy and happy and deal with the long term effects, if there are any, as they appear.


A Scare-free Halloween?

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

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.