Author: Evan

No News is Good News

/ Evan / 2 Comments

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

/ Evan / 2 Comments

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.

[youtube_sc url=”http://www.youtube.com/watch?v=aD8y4Vv-skA” theme=”light”]

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

/ Evan / 2 Comments

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

/ 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.

–

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

/ Evan / Leave a comment

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.