tacrolimus

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.

 

 

 

Two Steps Forward, One Step Back

/ Evan / 3 Comments

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

/ 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

Day +75: Finding a Natural Weight

/ Evan / 5 Comments

As most of you know who have checked in with us at one time or another, Zoe has been on steroids for most of her life at this point. She gained a great deal of weight pre-transplant due to them, and has since retained a decent amount of it. Her percentiles for her age and size put her in the ~90%, meaning she is larger than most babies her age and length.

We’ve been slowly creeping back down from that as she started to grow again and become more mobile, she seemed to stop gaining height for a couple months during the worst of chemo and steroids. Now, she really only retains her steroid cheeks, and even those are in slow decline finally.

During this process we’ve had a heck of a time getting her meds right, as we’ve moved from IV nutrition to milk and solid foods. Zoe’s weight has gone down, her Tacrolimus level (immuno-suppressant chemo) has shot up, and her Magnesium has dropped considerably. Last week and this week have been all about trying to get her eating, trying to figure out if her Tacrolimus readings are “real” or an error of the test, trying to get her Magnesium back up.

Where we are at the moment is, Zoe’s weight is around 8.2 kilos, which is pretty close to where she should be for her size, around the 50th percentile. I’ve come to feel that she’s trying to find her “natural” weight, the weight she would be were it not for all of this mess she’s had to endure. She seems to be much spunkier than she was even a week ago, and feel more comfortable moving around. She sits up easier, rolls on her side easier, and holds herself up on her belly with ease. All part of growing up, of course, but the change seems to be related to the weight. We can feel that she’s lighter. She seems to feel it too.

Zoe’s Tacrolimus level has shot up very high, and they’re inclined to believe it is accurate now — 1800, where normal is 500 for her. This can cause all manner of problems if it continues, so we’re going to suspend it for now, until we figure out the new dosage. Her Creatinine is up as a result, and because the Tacrolimus leeches Magnesium, that is very low. She’s getting supplements of Magnesium now 3 times a day, but she has gotten so proficient at spitting it out that we are struggling to get the levels up.

nom nom

We’re crossing our fingers that all of this settles out once her weight stabilizes. She’s eating pretty well, but the change in diet has definitely wreaked havoc on her med situation. If we can get it figured out without any seizures from too much Tacrolimus, or GVHD from too little, all will be well. We’re a little anxious, however, since those two situations are a possibility at this point.

On the bright side, she is eating solids quite well, her spirits are high, and her personality is really starting to shine through with little happy noises, laughter and cute little eyebrow maneuvers whenever she’s listening to us speak.

Oh, and she’s cutting 3 teeth now.

Day +50: The Med Routine

/ Evan / 4 Comments

Things are settling out here as we work out our routines. Now that we’re out of the hospital and in each other’s hair again, everything seems at ocne more hectic and more pleasant — even the unpleasantries.

Zoe has a long routine that we go through each day to maintain the meds she needs. It is very prescribed and intricate for a lay person I’d say, it’s taken us a few days to adjust to everything we need to manage.

Right now, Zoe is still on TPN or IV nutrition, which she’ll stay on until she is eating enough milk or solids to no longer need it. That may be a few weeks, though we are working on it. She is also taking a variety of medications for a variety of purposes, all of which are monitored and adjusted with daily lab (blood) draws that we draw and take in to the hospital.

We were going in every day for the first while, but we’re already on an alternating schedule due to Zoe’s pretty amazing resilience. She has not yet needed any infusions — platelets, blood, GCSF for her WBC — and that’s given the doctors confidence that she is stable enough not to need to come in each day. Her WBC count is holding out around 3.4, and they are resisting giving her any cell growth medication at all in hopes that by stressing her immune system a little, she might begin to produce them herself more quickly.

Here’s what our schedule looks like at the moment on a clinic day:

The Situation

7:30-8am: Zoe’s alarms go off for her TPN and Lipids, which run overnight for 12 hours each night. We flush her lines with Saline and lock them with Heparin, which prevents them from clotting when not in use.

8:15: Blood draws, between 1 and 3 tubes depending on which tests she needs; these have to be dropped off at the hospital within a few hours, but we try to get them in by 9am to ensure the results are back by the time we’re in clinic.

Packing up

8:30: Zoe’s Tacrolimus infusion starts, which runs over 2 hours; this is an immuno-suppressant to keep her immune system from revving up too much, she receives medications to help her fight off infections while it’s kept suppressed. She also receives her morning Hydrocortisone infusion, which is given by hand over 5minutes.

9am: Morning oral meds. Typically 3-5 meds, including Cellcept(to prevent graft rejection), Amlodipine (for blood pressure), Vfend (to prevent infection), and Raniditine (for reflux while her GI tract heals).

Have food will travel

11am: Clinic time. Visits vary widely in length, Mondays being the longest due to our seeing the primary physician that day and getting IVIG(antibodies). Other days we may be out in an hour after they’ve checked her, if everything is looking good with her blood tests and vitals.

2pm: Second Hydrocortisone infusion of the day.

5-6pm: The daily med delivery arrives, bringing everything from syringes to batteries to actual medications in fridge packs.

6pm: The evening prep starts for infusions and TPN. The IV nutrition has to be loaded into the pumps to run overnight, and injected with vitamins. The vitamins lose their potency in the light, so they are injected just prior to administration.

6:30-7pm: Evening infusions. Zoe gets a third Hydrocortisone infusion over 5minutes, Zofran(for her stomach) over 5minutes, and we load her TPN & Lipids for the night. She also starts her second dose of Tacrolimus of the day around this time.

7:30-8pm: Evening oral meds. Usually the same as morning, though a couple are only once a day.

9pm: Tacrolimus is complete, and Zoe is asleep for the night. She typically sleeps through, with perhaps one wake-up at night.

Each of the infusions requires 5ml of saline before and after, then a 3ml Heparin to lock the line. This means Zoe goes through roughly 15 saline syringes per day, not to mention the liquid from the infusions themselves and the heparin. It’s an amazing amount of fluid for her size, good thing those kidneys held up.

Zoe & Maya Playing

Stir in one 3 year old and shake vigorously, and we have a pretty interesting schedule right now. As time goes on, we’ll cut the TPN & Lipids completely, vastly simplifying things.  We’ll hope to cut out other meds, and eventually get down to only the Tacrolimus 2x a day, if everything continues to proceed as planned.