Evenings have been full of a tired and fussy two year old, a six year old who wants to do whatever activity is going on in the house, and two tired parents.
Before we left Oregon, I saw the schedule for Ethan's workup, and thought that it was a ridiculous schedule for a two year old. But I figured, they work with a lot of young Hurler's kids they must have factored in his age. I was right, it was a ridiculous schedule. Each day has had 4-6 appointments in a row, each running from an hour to three hours, leaving us an hour or so for lunch, and no time at all for Ethan's nap.
Tuesday morning's schedule started with three hours of testing in neuropsychology. By the end of the first hour, it was clear that Ethan wasn't focusing. I couldn't blame him, he hadn't had a nap the day before and didn't make up for the sleep overnight. He was just tired. From that point, I started asking that they take the appointments for occupational therapy and physical therapy off of this week and move them onto next week so that Ethan would have more rest this week and we wouldn't be wasting everyone's time asking him to work through things that he was just too tired for. That also opened up some windows in the schedule for Ethan to get more naps.
Other schedule snafu's include scheduling Ethan for a head and chest C/T scan and expecting a two year old to hold his head completely still for one minute while they shove him through this big machine. Needless to say, we didn't get those shots, and we've rescheduled them with sedation next week.
Aside from the mad schedule and the resultingly mad kiddo, it's been a pretty good week for Ethan. So far he's been evaluated by cardiology, dermatology, pulmonology, endocrinology, audiology, neuropsychology, otolaryngology, ophthalmology and radiology. He's had electrocardiograms, echocardiograms, chest x-rays, hearing and vision tests. He's had a skin biopsy to determine if his ongoing rash is eczema or GVHD (turns out it's eczema, at least at this point). And, he's had his central line evaluated, and we've met with other doctors, coordinators and social workers.
Still to come, Monday 5:40 a.m. check in at the OR for a sedated CT scan, ear check (EUA), eye check (ERG), and lumbar puncture. Also next week, he's got completion of the neuropsych evaluation, occupational therapy and physical therapy appointments that were moved from this week, as well as a neurology consult.
On Thursday, I wasn't feeling well--I think something I ate was coming back to haunt me. So Sarah took over the afternoon appointments. Today, Ethan's having his enzyme replacement therapy, so it's his quietest day all week.
All of this is intended to ensure that Ethan is okay to undergo transplant, to identify any issues that may arise during transplant, and to provide baseline info for p/t and o/t therapy. There is some concern about the RSV he had in December; pulmonology wants to review the CT scan to ensure there are no lingering signs of infection and endocrinology wants to ensure that his adrenal system hasn't been impacted. But, so far his tests and evaluations look very good and nobody has raised any significant concerns.
I will say that this workup is far, far more extensive than what he received in Portland, and each of the doctors who has seen him has extensive experience evaluating Hurler's patients within their specialty, which is a nice change.
We did learn that they've changed their mind about his transplant regime. When we talked with Dr. Orchard by phone in November, he said he thought that they would decide to go with a fully ablative, full-toxicity regime, as they would with a first transplant because it had been a year since Ethan's first transplant. On Monday we learned that they've decided to go with a partially ablative or reduced toxicity protocol. (A fully ablative regime aims to clear out all of the marrow from the bone, and a partially ablative regime just seeks to clear out enough of the existing marrow for the donor cells to find space and begin to engraft.
Assuming that everything continues to go well, they anticipate admitting him and starting chemotherapy on Friday, 1/14, which will be day T-21. He'll continue chemotherapy through T-2. On T-1, he'll receive low dose total body irradiation, and his donor cells (from a donated and stored umbilical cord unit) will be administered on T+0.
If there are no significant complications, Ethan could be released from the hospital at about T+35. Or if there are complications it could be a week, a month, or many months after that. We'll be here in MN until at least T+100 as that seems to be the magic date for transplant patients to be released back to their home hospitals for follow-up care.
The above was written Friday afternoon, while Ethan was napping during his infusion. It's, now about 6:40 on Saturday morning. Ethan woke up about 2:30. Sarah worked with him until 4:30 when I woke up and relieved her. I worked with him until 5:30 before dosing him with Benadryl to help him sleep. Lemme see how much more of this I can get done before Ethan wakes up and wants to use his computer.
Yesterday evening, we got a call from the endocrinologist. Ethan's been diagnosed with adrenal insufficiency, which is likely a reaction to the steroids he was on for his anemia. He's got a new med prescription for a type of cortisone. It sounds like they can manage it with medication, and it will prove to be a temporary condition.
On Wednesday, Ethan had his central line checked out to see if the November '09 line was still in good shape for this transplant. The nurse who looked at his line also took ownership of the dressing issue and has been helping us work through different causes and options there. We think that Ethan might have developed a sensitivity to the adhesive in the Tegaderm dressing. Evidently, sensitivity or a developed allergy to these dressings is not uncommon. Torie, one of the other Hurler's kids in the house has developed a strong allergic reaction.
As a first step, to get ahead of this, we changed the kind of dressing. So instead of the biopatch, tegaderm combination like this, we've covered the insertion site with a small primapore dressing and then covered the site and his tubes with a catheter wrap.
We also came to think that the scratching had become a behavioral issue as well. Ethan's been doing most of the scratching at night. Even though Sarah and I have been taking turns sleeping with him to keep him from scratching at it, it hasn't been enough. As Sarah and I were looking for answers and options on this, we called my mother and asked her if she would make a sleeveless vest that would be thick enough to dull the scratching but soft enough that it wouldn't irritate his skin. She hopped right on that and put something together on Thursday for us. Surprisingly, Sharon, the nurse we've been working with on this, likes to sew and also whipped something up. She took a 3T t-shirt, added some quilting underneath the front of the shirt and added a velcro closure on the back so we could make it more snug.
Fortunately, the new dressing seems to be working better, so far. He's not picking at it nearly as much. The downside is that the primapore needs to be changed every other day, whereas the biopatch/tegaderm combination only needs to be changed weekly. Eventually, we'll need to go back to a dressing that we can leave in place for a full week. There are some hypoallergenic options out there that we'll explore over the next couple of weeks.
Caleb and Sarah have had a busy week as well.
Caleb started kindergarten at the Ronald McDonald House charter school on Tuesday. Unfortunately, the only other kindergarten student left just before Christmas break, so there's no one Caleb's exact age in the school. I worry about Caleb not having any peers around and how in his natural emulation of the older kids in school he may grow up more quickly than he should or is ready for. Caleb is a good kid, and I think he'll do okay. But it's just another way that his childhood isn't normal, and it tugs at me. Regardless of all of that, Caleb seems to be settling in well. His teachers say that he seems comfortable in school and he is making new friends. His teachers have also been wonderful to let him bring things to share with the class each day (train posters, science kit, and, of course, his toy hovercraft---they ran it in the gymnasium during recess.)
|Dodgeball in the bouncy house.|
Sarah's had a busy and tough week too. She's been fighting a cold since last weekend. Having a cold around here isn't a good thing, since a lot of the kids in the house are weak or immune compromised, and you don't want to get them or their parents sick. So, Sarah's been wearing surgical masks all week, even when she's asleep. She takes it off when she's alone during the day, telecommuting from our room. At dinner times, she's been getting some food and going over into the corner where she can remove her mask and eat without breathing on anyone. Fortunately, she's finally feeling better and we were all happy to see her face again yesterday.
Since it looks like Ethan will be admitted on Friday, this will be our last normal weekend for quite some time. Later this afternoon, after Ethan's mid-day nap, I think that we're going to go to the Mall of America. (Just shoot me now.) The rides and attractions for kids should be fun. But I'm really hoping we don't actually try to browse and shop. (Argh.) I'm not sure what we're going to do tomorrow. But I expect that we'll try to get out and do something fun.
A huge thank you to everyone who has sent posters, cards, toys for the kids, and care packages. I'm sorry that we haven't had time to thank everyone directly. We really appreciate it, and so do the kids.
For those of you who were wondering about RMH, I've posted some pictures on FB.
Thanks for checking in. I'll try to post a brief update Monday or Tuesday after Ethan's sedation and checks