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.

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.

 

Life Goes On

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

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?

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.

Two Steps Forward, One Step Back

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.

 

 

The Spring of our Discontent

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

Diagnosis: One Year Later

“Well, here we are. We’ve come a long way from where we were a year ago, having just learned that our child had a rare condition that might kill her. We fought, she fought, and we’ve made it this far, and things appear to be improving as each month passes.”

I began writing this post a few days ago, thinking I could get it ready and put it up on the anniversary of her diagnosis. What I wasn’t prepared for is how strongly my emotions about the day would become as it approached, making what I wrote simply insufficient.

Zoe had her first birthday January 10th, and it was a joyous occasion, but it didn’t compare to today somehow. We all go through traumas of one sort or another in our lives, no one is really exempt, it’s more a matter of degree and timing, and what these events make of our lives. This past year has come to be the most important in my life so far I now realize, but I don’t think I realized it as it happened.

When I look at Zoe these last few days, I tend to tear up — not in sadness, but in wonder. I still can’t believe some days that we’re so lucky as to still have her. My wife and I go back and forth about how her life was saved — did we have a hand in it? Did our pediatrician earn his wings that day he made us go to the hospital? Was it the emergency room doc at WFU who just happened to give his input and start the chain of events that gave us our diagnosis who saved her? Was it Duke’s amazing transplant program? Obviously it’s a little of each. What’s certain however is that there is a degree of luck involved in it all, and I’ve come to believe that our role was to improve her odds as much as we could, to load the dice, giving her a shot at life.

We put all of our energy into learning about the disease, the procedures, the drugs, did our best to have a crash course in anything that could potentially improve Zoe’s chances. It’s impossible to say how much any of it mattered I suppose, but being involved mattered a great deal to us. We look at Zoe today and we are brimming with love, hope and pride by turns. It’s an amazing feeling, but it may take the rest of our lives to figure out what happened this year.

Where are we now?

Zoe is a vibrant, smart little girl. She’s walking, using her first words, babbling, climbing ladders and couches, diving off beds and rolling around on the floor with her sister whenever she gets a chance. She seems to be progressing exactly as her sister did, which is to say, perfectly normally.

She still has a med routine, she still has clinic visits, she still has a port running directly into the veins in her chest for blood draws, and she still is quite limited in what she can do. She cannot be around large groups of people, she can’t go to malls or the circus or farms where there is exposed debris and livestock. She has to be kept in the shade most of the time, as going in direct sun sets can cause a rash very rapidly. We have to limit who can visit our home so that we can be sure we’re not introducing any colds or flu to her “safe” zone. But, each day takes us closer to the day when she doesn’t have any restrictions and we don’t have any fears about her health.

Zoe had her most recent clinic visit on Monday, and it held a pleasant surprise — we’re switching to every-other-week clinic visits. We probably could have done so before, but we’ve had a rough time stabilizing her tacrolimus, and with the little rash scare she had awhile back we just never cut back on visits. The doctors at WFU feels she is ready now though, they have no concerns and feel her numbers are good. Her WBC is at 7.9, and her Platelets are 262, both really rock solid at this point.

Her IgG count has been dropping, but remains in the normal range. This is a count that gives us an indication of her antibodies, and she periodically gets a boost to them — her last boost was at the start of the year.

We have been weaning her off of her steroid and she is now down to a tiny dose, to be off completely soon. Zoe is currently at 40th percentile for weight, and 40th for height, which are just fine — she should be able to catch up to her norm in time.

In general we are just incredibly pleased by how things are going. It can be stressful at times still, but each month that passes brings us further and further away from the really dark times, and closer to a feeling of security with her health. It’s hard to believe looking back on where we were a year ago that we’ve made it so far.

The rooftop playground at the Brenner Children’s Hospital:

httpv://www.youtube.com/watch?v=7ufWJns-p2M

Tough Times

The last couple of months have been hard in other ways. Not because we have had any major complications ourselves, but because we’ve heard mixed or bad news over the course of flu season from several other families we’ve been following, or who were being treated as we left the hospital with Zoe. Hearing about a child passing due to complications from HLH or a similar condition is just devastating, and each time it happens we have to take stock of where we are and how grateful we are. We get to share in the victories as well, but there are too many children still fighting losing battles out there.

When you are thinking about what you can do to help, there are several options. You can support organizations like Bonemarrow.org, Histio.org, and the various foundations set up by parents to raise awareness about a particular disease.

You can also try to keep in mind that policies being enacted in various states have real consequences, and that if you are in a state where procedures like transplants of the type that saved Zoe are being limited or even removed as an option for some families, you can contact your Senator, Congressperson or other representative to voice your opinion.

Throughout this process I’ve spoken to folks who live in the U.S. as well as many who don’t, and hearing as I have several times from someone who lives in a country that doesn’t have the medical resources to treat HLH properly is saddening on a very deep level. Their options are terribly limited and this disease is relentless.

We in the U.S. have the technology and resources to treat these kids, and yet in some states we’re saying to them that we won’t if a family has the wrong insurance or is on Medicaid. No one deserves to die because they can’t afford a transplant out of pocket, and I hope those of you who read this will remember children like Zoe when it comes time to decide where budgets should be cut.