Friday, July 22, 2011

T+99: Home

Final clinic visit. From left: Dana (CNA in the Clinic), Kim and
Teresa (Nurse Coordinators), Sarah, Todd, Ethan, Dr. Troy Lund;
front: Ann, Child Life Services 
After six and a half months in Minnesota, we're finally home!  In fact, we've all been home for a few days. Sarah arrived in Minneapolis last Tuesday, the day before Ethan's exit conference. We had the exit conference with on Wednesday. Sarah and Ethan flew out Thursday morning. And finished the last bit of cleaning and packing before following with the packed minivan.

I'm sorry for not posting earlier, but when we're home we've got all the house stuff and dog walking in addition to the kid stuff keeping us busy, so it's often harder to find time to write.  In fact, I'm not sure how long this post will be.  Ethan's eyes have already peeked open, so I expect him to wander out shortly and announce the official start to the day.

We're all very, very happy to be home. Ethan has explored the house rediscovering and reclaiming old toys like long lost friends. He's really, really happy to have larger range to roam without having to put on his isolation mask just to go down to the kitchen or playroom.

Caleb, in a tree at cousin Brian's farm. 
While Ethan and I have been away, Caleb had a fantastic time at his "wilderness" day camp.  Once or twice a day now, he'll point out and name a type of flower or tree or plant that he spies.  (He can name more plants than I can, not that that is particularly difficult.)  The camp increased his confidence and his comfort level outside and just generally exposed him to a bunch of new things.  Which, I think, is what summer camp is supposed to do.  Caleb has also started putting things together cognitively and verbally. It's a pleasure to see and hear that his wheels are really turning!

At the moment, we're trying to settle into some type or routine.  It's a process of discovery and evolution.  Ethan and I still seem to be on Minnesota time, waking up at 5:00 or 5:30.  Sarah's been getting up early, too.  So, we're having long, relaxing mornings in our own space.  It all feels rather decadent.

Medically, Ethan is still doing very well, though they drew blood for a new set of chimerisms before we left Minnesota and we still haven't seen those numbers. His chimerisms show his engraftment level and having one marker in the 90s (good) and one marker in the teens (bad) will be a continual source of worry for us even though the doctors say that they won't worry too much unless that 90s marker drops below 50.

Ethan in all his steroid glory last December.
I've also been watching Ethan's weight pretty closely.  About a month ago, Ethan dropped a full kilo (2.2 lbs) in one week.  At 14.6 kgs, he looks a lot different than he did in December when he was a 17 kg pudgy, steroid plumped, little elf. Ethan saw his primary care physician yesterday and she confirmed what Sarah had suspected, that his weight/height are actually pretty much in balance now, so this isn't a bad weight for him to be.  His weight's been stable for the last month, so I suppose I shouldn't worry so much about it.  But I feel like a mother from the old country, "What?  Look at you!  You're all skin and bones!  Come on in and have some fried chicken, and here, have some more potatoes, and some chocolate milk and rhubarb pie.  And when you're done with that, there's a lovely girl down the street I want you to meet.  Oh!  You two would make a lovely couple."

In insurance news, we've been receiving the summary of charges that they've paid for Ethan's care in Minnesota. The current total for this transplant is $855.566.81, added to the cost of his first transplant and Ethan is certainly our million dollar boy. The cost to Sarah and I for the transplant is much, much less. Our total out of pocket will certainly be something less than $3,000 including the travel and lodging costs.  Once they approved our request for a referral to Minnesota, our insurance company, Kaiser Permanente, really took care of us. The benefits were excellent.

Caleb in his cape after the day's "magic camp."  Picture
taken at an outdoor concert featuring Grandpa
Bob's community symphony.
Unfortunately, since I quit my job, we need to change insurance companies.  We've been extending our Kaiser coverage under COBRA, but now that we're back, it no longer makes sense to do that and we're moving over to Sarah's Blue Cross/Blue Shield program.   It's a decent plan, but the bone marrow transplant benefit is capped at $250K.  So you can see why we didn't move over there sooner.

Moving out of Kaiser is kind of a melancholy thing.  Once Kaiser approves something, the benefits are very good, and we've loved the speed and record sharing of their referral system.  When we've gotten a referral, typically we've had an appointment set up within 24 hours and get into see the doctor within the next week, unlike our prior experience with independent doctors.  It's also been nice that each of the doctors has had full access to Ethan's records.

Caleb and Grandpa Bob at the Oregon City Farmer's Market
But....  As we move away from transplant care and start to focus more on assessing and repairing the damage that Hurler's has done, we'll need a multi-disciplinary team of doctors who have seen Hurler's before.  There's just no way we're going to find those folks within the HMO system.  We need to be able to see an endocrinologist in Seattle or a neurologist in California or an orthoHMO's.  In exchange though, we get choice.  Which is what we need at this point.

The big downside of having to move out of our HMO is that we loose access to Ethan's primary care physician.  We've been working with her since before Ethan's first transplant.  She's very good at looking at Ethan as a whole kid and balancing and prioritizing what's going on with him.  And she's been a great big help in other areas and very easy to work with and we will be sorry to loose her.   Which means we've got to find a new primary care doc for Ethan.

We've talked with Ethan's transplant doc and with Ethan's current PCP about what we should look for in a new pediatrician for Ethan.  Clearly, first and foremost, it needs to be someone who enjoys working with complex cases.  In other words, they've got to want to have him in their practice. They have to be willing to accept the fact that Ethan will see a lot of specialists, so they may not get to work with him a lot, but that he will generate an exceptional amount of paperwork coming across their desks, reports form the specialists, lab results, x-rays, CTs, MRIs.  A good PCP will take the time tow go through all of that enough to keep a good command of his care picture and needs and a really good doc will read it closely enough to find where the specialists have made mistakes or are overlooking something.  For us, a new PCP needs to be accessible and willing to communicate with us directly and not shift us off to their nurse or medical assistant.  There are just too many people involved in Ethan's care for us to loose things in translation. And finally, our PCP needs to be honest with us.  Because if you get right down to it, the specialists will always have another thing that they can try.  It will be a good PCP who looks at the whole picture and says, you know what, we may not be able to push this any further.

It's hard to believe that I've only been home 7 days.  It feels like a lot longer, even though I still can't remember where things are in the kitchen.  Even though our days are calm, it feels like we're running, or at least at a brisk jog.   That seems par for the course around here.

Thanks for checking in on us.  We'll try to write more soon.

Friday, July 8, 2011

2 hours, 2 mg of Melatonin & 5 ml of Benadryl

2 hours, 2 mg of melatonin and 5 ml of benadryl.  That's what it took to get Ethan to sleep tonight. 

For Ethan and I, the past three weeks have been all about building routines.  We are making progress, but tonight just goes to show that progress ebbs and flows.

I've talked about Ethan's sleep issues here before. He can take an hour or so to go down for a nap, then will routinely take an hour, if not two, to go to sleep at night.  Then only to wake up in the middle of the night for at least a couple of hours. I can not even begin to count the number of hours that Sarah and I have spent holding him rocking him, or laying quietly next to him to try to get him to go to sleep. And I don't want to try to add up the hours that we've spent with him when he's woken up in the middle of the night.

Taken one night at a time, it doesn't seem like so big of an issue.  But over the course of almost three years now, it's been the cause of unneeded frustration, stress and exhaustion for all of us.  

The past few weeks have been nice because I've been able to focus exclusively on Ethan.  When it comes to bedtime and sleep routines, I've been able to eliminate almost all of the distractions.  The Ronald McDonald House has fantastic storm shutters that will make a room almost completely pitch black, so there's very little light shining on things that might distract him.  The family dogs aren't here and aren't barking at someone walking down the street just as he starts to nod off. And if he wakes up in the middle of the night, I'm able to keep him in bed and work with him and try to coax him back to sleep.  I don't have to take him into the living room so we don't wake up Caleb, as has been the case over the last few months. 

After a couple of weeks of working with him to improve his nap and sleep issues, it occurred to me that I was taking an hour to get him down for a two hour nap and then taking two hours to get him to sleep at night.  It started to dawn on my slow-witted brain that maybe what I was really doing was just shooting myself in the foot; and that in trying to get him down for a nap, I was just making things more difficult in the evenings. 

I was complaining about this to some of the other parents on the MPS Forum, and they all just laughed at me and at the pure folly of trying to get a Hurler's kid to nap when they don't want to.  They said all of their kids had stopped napping years before and far earlier than their 'normal' peers. 

In hindsight, I think we just got conditioned to fighting Ethan to sleep all of the time.  He was so colicky from about 2 months through about 6 months old that each evening from 5p to 8p he would just wail and thrash bloody murder and we would swaddle him tightly and walk him and rock him and walk him and rock him and sit with him until he fell asleep. This kind of behavior recurred, though not to the same extent, in the spring after his first transplant when--we think--his blood enzyme level from the first transplant started to decline.

I think we got into the habit of fighting him to sleep.  We also believed that he was young enough that naps were important for his development. We did know that sleep issues are very common in MPS II and MPS III kids, but once we got through that colic phase, we never stopped to think that there was a long-term hyperactivity or sleep disorder component in Hurler's post transplant. There doesn't appear to be much in the literature, but from talking with other parents and from our own experience, I'm increasingly of the opinion that  he does suffer from hyperactivity and will continue to do so, at some (minor?) level post transplant. 

After getting laughed at by the other Hurler's parents, I stopped putting Ethan down for his mid-day nap.  That seems to be working very well--but not perfectly. Since then, without his nap, Ethan has settled at bed time fairly quickly, almost always falling asleep within 30 minutes without melatonin or benadryl.  

Without his nap, he's also slept through the night each night with only two exceptions.  One night, he had evidently woken up sometime after 3 and about 3:30, he came into the bedroom, woke me up, and told me to come into the living room to see this really cool episode of Caillou that he was watching on the iPad.   He wasn't very happy when I told him we wouldn't be doing that, turned off the show, and brought him back to bed, and it took him a couple of hours and some benadryl to settle back down. This morning I woke up at 6:30 to find that Ethan was already up and was out in the living room watching Caillou again.  (He hadn't woken me up this time, so I guess he'd learned that I don't like to watch Caillou in the middle of the night.)  I have no idea how long he'd been up.  Could've been 20 minutes, could've been hours.

Before I move on to other more succinct and possibly more interesting topics, I have to add a huge public thank you to our wonderful Care Partner volunteer, Sue. Sue got a first hand introduction to Ethan's sleep issues on Tuesday when she stayed with Ethan for six hours while I went to the UMinn Emergency Room. 

Tuesday, at dinner time, I was trying to open a can of baked beans for Ethan--which is one of the few things that he will consistently eat at the moment. I had just bought a new can opener from Target the week before, and the thing turned out to be a complete piece of junk.  It only got the can open half way before dying completely and forever. In what was clearly not my brightest moment, I figured I could pry the half of the lid up with a spoon.  Yes, I knew at the time that it wasn't a great idea.  I hoped I could get away with it.  I was wrong.

When the spoon slipped--as it was, I suppose, bound to do, the can lid opened a nice gash on my index finger. One nice thing about being us is that we have a fair  bit of medical and first aid stuff around the house.  So I wrapped some gauze around it and after soaking through six pads, I called Sue to see if she could sit with Ethan while I went to the ER to get it treated and for a tetanus shot.

Sue was in the middle of cooking dinner with her adult son, but she's such a sweetheart that she dropped that and drove a half hour over to RMH to take care of Ethan while I addressed the results of my stupidity.   It took me six hours to be seen and treated at the UMinn ER.  Meanwhile, Ethan gave Sue a merry chase about going to bed.   The little stinker had to be exhausted because that was the night the first Caillou episode and he'd been up for two hours in the middle of the night.  Even so, he would not settle for her and kept trying to wobble exhaustedly out into the living room; where, one assumes, he was hoping to find either me or Caillou.   When I checked with Sue something after 11, he still had not gone down, so I told her where the benadryl was and told her to do what needed to be done. I think he finally got to sleep at 1130 or so. 

When I got back to the apartment at 1 a.m., poor Sue looked bone weary. And the wonderful lady had another half hour to drive to get back home before she could get to sleep then wake up just a few hours later to go to work. Even then, Sue was back out two nights later to sit with Ethan for a couple of hours so that I could grab a haircut and a starbucks.


If you've made it this far...
I really should end this entry here and go get some sleep myself.  But if you've made it this far, I should tell you how Ethan's doing on more consequential issues.

Medically, Ethan's doing absolutely great. His blood counts are very good. His energy and mood are great.  And we're seeing improvement in both his speech and occupational therapy work. 

Speech therapy is very slow going with him.   Ethan understands a good deal.  You can give him multi-step directions and he will understand what you're saying and act on it. My guess is that he's working on an age appropriate or almost age appropriate level there.  But, it's very difficult to get him to say a word or make a sign on his own.  He'll usually do it when prompted, but outside of the 15 or 20 words that he's most comfortable with, he won't initiate a word or a sign.  

Even though he's almost three years old, I've started to think that we've basically lost a full year with him due to the failure of the first transplant and the additional glycosaminoglycan (gag) build up in his brain and in the fine motor tissue around his mouth and jaw. Dr, Lund says that that storage material will be cleared over the first six months post transplant as his body produces the alpha-L-iduronidase that it's been missing, and that his learning rates will start to approach that of normal kids.   

We are starting to see some progress here.  He more readily reproduces some of the signs, sounds and words that we've covered a lot.  But it's very slow going.  And while he's very expressive and able to get his broad point across, he can't yet tell us exactly what he wants.  The best example here is food.  He has started reliably making the sign for eat.  But he can't tell us what he wants to eat and it turns into a lot of pointing, "This?", "No.", "This?" etc...Though he has started going to the refrigerator and getting out whatever he wants himself.  That's been both and improvement and a help. 

He's made more progress in occupational therapy..  OT covers fine motor skills used in writing, picking things up,and manipulating objects.   A month ago, the OT therapist described his pincer skills (picking things up with your thumb and forefinger) as a 12 month level on his right hand and a 9 month level on his left hand.  Over the last couple of weeks, we've seen that improve by leaps and bounds.  It's not perfect, but it's a much more coordinated and precise effort than it was.  His ability to turn his wrists has also improved.  And today, the OT therapist also said that she thought the size of his knuckles had visibly reduced.  So perhaps some GAG buildup is being cleared there as well.

Ethan and I have also been working a lot on his eating.  His weight at admit for transplant was 15 kgs.  A month ago it was 15.4kg, then in one week it dropped to 14.4kg, a loss of a full 2.2 lbs in one week.   Since then it's stabilized at 14.7kg, which is good.  But it's tough to find things that he will eat and it's a lot of work to maintain that weight. (Ideally, I'd like to see him back north of 15kg.)  There are a lot of factors in play here.  The chemotherapy screws with your taste buds and what tastes good to you.  Some of the meds he's on cause nausea.  And yesterday, I found out that the adrenal insufficiency can cause a salt craving.

So for his appetite, I'm just trying to eliminate possible causes. In consultation with his doctors, I've upped his anti-nausea meds.  And I've upped his hydrocortisone to see if there's a salt craving we can eliminate. And I'm taking a lot more time with his morning routine, giving him a full chance to eat before I start shoving meds down his throat. I hope we'll start to see some progress on that next week, but it's too soon to tell. 

In other news, Ethan's got a final sedated CT scan on Monday to check the granuloma in his lungs.  Then Tuesday, it's speech therapy.  Sarah flies in Tuesday afternoon.  Wednesday, we've got our exit conference.  Then Sarah and Ethan fly out Thursday morning and I'll follow with the car.

With any luck we'll all be back together in Portland next Saturday!

Sorry, it's late now and I don't have the energy to put any pictures in here right now.  But you can see some recent shots here.