brothers

brothers

Sunday, October 30, 2011

Emerging from Transplant

Mr. Big Boy on the plane.  He did great on
on the flights!
Friday afternoon, as Ethan and I walked out of his last six month eval. appointment, I called Sarah to share my joy and relief.

27 months after diagnosis.  24 months, almost to the day, after his first transplant. We think we've finally reached a point where we can comfortably say that this transplant was a success and that Ethan will survive.

Earlier in the week, we had received really encouraging engraftment numbers.  Ethan's CD15 marker, the genetic marker that controls the production of the alpha-L-iduronidase, is 100% donor.  Ethan's CD3 marker, which suggests the likelihood that his body might start attacking or rejecting the graft, was 82%--up from 30% at the end of August.  (And the doctors say that it will likely come up some more.)

The hard truth is the transplant is a treatment for Hurler's, it's not a cure.  We don't know how long Ethan will live.  From what we've seen of older transplant kids and Ethan's individual risk factors, I'd say he's got a very good shot of making it into his twenties. There's are a few promising research projects that could help him make it into his thirties or even forties.

Between now and then, whenever "then" might be, we will try to fill each day with fun, laughter and love. Now that he's through transplant, we need to shift our focus some and work to balance his ongoing medical needs and interventions with helping improve his quality of life.

Our journey and Ethan's fight with MPS is far from over. During our time in Minnesota, we received some good news about his hips and legs, some difficult news about his neurological development, and have already scheduled his next surgery (carpal tunnel release in April).  But there will be time enough to talk about those in a future post later this week.

For now, it's enough just to breathe a sigh of relief.

Saturday, October 22, 2011

T+190: Rehab & Back to Minn

A huge thank you to everyone who donated to help fund research and support MPS kids and their families. The MPS Sponsor a Child for a Cure Run was held on October 2.  With your help, we raised over $1,500!!  It may seem a small amount compared to the cost of research and testing.  Please know that your generous donations combine with others' and it all adds up.  Thank you.

Ethan 09/11.  He got a little
sunburn on his nose and
kept picking at it.  It took
forever to heal!!
(I think that's a Nerf gun in his hands.
Watch out!!)
Ethan and I have been spending a lot of time at home.  He typically has three to four appointments a week.  Some of those are out and about and a couple of them are early intervention educators who come out to our place.  Ethan's been stable enough that we only need to check in for blood work every other week. And we've finally gotten Ethan's rehab therapy geared up and we'll be at speech and occupational therapy once a week each.  Regardless, the appointments are typically in the morning, and aside from that we've got a lot of downtime.  We're both really happy to see Caleb come home from school at 2:30 and it gets even better when Mom comes home at 5:45.

Rehab
If you'd asked me a month ago, I would have said that speech rehab was going to be the most challenging part of Ethan's care over the next year.  He is still essentially non-verbal with a functional daily vocabulary of 5-10 words and another 5-10 signs. But after watching him through a couple of terrible occupational therapy sessions, I'm starting to think that OT (rehab to improve his daily functioning)  will be just as challenging as the speech issues, if not more.


We all enjoyed a quick three day weekend
at the Oregon Coast at the end of
September.
Last month, Ethan had a horrible occupational therapy evaluation.  After the first activity, he had a complete meltdown and wailed for the rest of the hour. That session really got me thinking that we had to expand our focus and put as much time and energy on OT as we do on speech therapy.  Occupational therapy focuses on a lot of daily life skills, fine motor stuff like writing, but also daily life skills such as dressing himself and brushing his teeth, academic skills like sitting down and focusing on an assignment for a period of time.

As Ethan has gotten older and started forming more definite opinions about what he likes and wants to do, and everything else, he's had a harder time transitioning and become much less cooperative when you challenge him to do something that he doesn't want to do.  There are a ton of reasons for this: he's non-verbal so he can't fully communicate his needs; he hasn't had a lot of structure in his life because of his medical condition and hospitalizations; there are poorly understood neurochemical and neurological changes that impact behavior; and to cap it all off, he's three and three can be a pretty frustrating age to be.

Ethan's difficulties transitioning and focusing make it more difficult for him to make progress in rehab, and will slow his learning and development even further.

Coming out of that horrible OT evaluation there were some bright spots and some bigger challenges.  One of the huge positives is that the speech therapist, Britteny, that we're working on is turning out to be absolutely fantastic, in just about every way possible. It looks like she will be very effective in working with Ethan and she's taught me a lot about what to look for in a good therapist.

Caleb had fun at his school's annual
Jog-A-Thon.  He did great!!
Britteny saw Ethan come out of that OT evaluation, the week before she was scheduled to do her speech evaluation with him, so she got a sneak preview of him.  The following week when we showed up for Ethan's speech eval, he wasn't in a good mood again, and I was afraid that we were headed for another frustrating and unproductive hour of crying.  Britteny came out into the waiting room and started working with him, both distracting him and giving him some cues and structures about what we were going to do next and when.  She really walked him back from the brink and disarmed him.

Over the next hour she continued to impress me.  One of the rehab challenges for Ethan is that he's three years old and he wants to play.  Britteny did a fantastic job structuring rehab activities that were instructive, appropriately observational and fun.  Ethan's worked with two other speech therapists.  Both of them struggled with providing fun, age appropriate activities that still maximized opportunities for teaching and learning. The other thing that I really like about her approach is that she recognizes that Ethan needs to develop an understanding of schedule and an ability to focus, so she's incorporating a lot of OT strategies into her work.

Watching her work with Ethan also changed my view of the prior week's OT eval. meltdown.  I really started to think that a good OT therapist would have responded to Ethan's tantrum more effectively and  should have worked with me and with Ethan to slowly engage with him and bring him back down from the brink; all the more so because developing focus and transition skills is really a part of the occupational therapy field.

Fortunately, the staff at the rehab clinic talked, and it looks like they've placed us with a different OT therapist who is a more experienced and may be able to work with Ethan more effectively.  We've only worked with this new OT, Erica, a couple of times, but she seems quite good.

So after taking long time to get the new speech and OT evals done, I think we've got good people in place and we can get to work.

How far can Ethan Go?
On the heels of the OT meltdown and as started processing that experience, I started to think more and more about the behavioral and functional challenges that face Hurler's kids post transplant. I really wanted to start to understand whether or not there were common challenges that our kids face.  I wanted to try to understand if certain limitations were a direct result of the disease.  And I wanted and want to try to understand the pieces of this that aren't simply learned behaviors or age appropriate behaviors.

Part of me says that if we understand some of the common issues then we can better structure effective therapeutic strategies.  But I also want to try to understand where we might end up banging our heads against the wall and not make much progress because the disease is simply in the way.  In those cases, perhaps medication is an appropriate response, or maybe we just can't beat it.

I came across an article from a UMinn neuropsych researcher that tracked adaptive function In Hurler's kids post transplant. The findings in that paper were pretty discouraging.  The paper tracked 41 kids and compared their communication, daily living, socialization, and motor skills development with normal growth and development.  The paper's findings were highly generalized but show that significant limitations and impairment in each of these areas continue as the children age, and that the developmental gap between them and "normal" kids tends to widen over time as normal kids develop at a faster pace.  I'm having a very hard time with that because it suggests that we'll get less of Ethan back than we hoped, and that he'll have to fight harder to learn and develop some independence as a teenager and an adult.

Where are the limits?  What will Ethan be like when he graduates high school?  Will he be able to live on his own?  What kind of work can he or will he do?  Will he be happy?  Our immediate rehab focus is on getting Ethan ready for elementary school a few years from now.  But these are the real questions, and the real fight.

Six-Month Minnesota Check-Up
On Sunday, Ethan and I are flying to Minneapolis for Ethan's six-month post transplant check-in.  The main thing that we want to do is check Ethan's engraftment and make sure the donor cells have taken hold.  The last time we checked, at the beginning of August, Ethan's CD15 marker (which makes the enzyme) was about 95% and his CD3 went up to 30%.  The theory was that the CD3 marker might take as long as a year and a half to recover with the particular transplant protocol that Ethan received. Nonetheless, after spending over a year watching his first transplant fail, we get pretty nervous about his engraftment, and are hoping for good, good numbers.

Minnesota's standard six month visit protocol seems pretty light.  But their normal six month schedule is for a younger kid and doesn't take into account the fact that Ethan is older and some of the cumulative effects of the disease may be more of a factor for him at this point.  Ethan' new pediatrician and I have been working closely together to review what evaluations Ethan's had and what he needs.  We've been back and forth with Minnesota numerous times, and the staff there has done a fantastic job getting us additional appointments and adjusting Ethan's schedule.

On Monday, Ethan's got a surgical consult in the morning at UMinn.  Then we're heading over to see Dr. Walker at Gillette Children's for a full orthopedic work-up.

Tuesday: Surgery to remove Ethan's central line, fix his umbilical hernia, do a nerve conduction test to check for carpal tunnel syndrome and a CT scan to check the granuloma.

Wednesday: Clinic day to talk with Ethan's transplant lead, Dr. Lund, and review his labs and engraftment

Thursday: Ortho appointment to review Ethan's carpal tunnel results, followed by neuropsych and neurology.

Friday: currently free, but I think we're going to end up doing an ACTH test to see if Ethan's adrenal insufficiency has resolved.

It's a pretty full schedule and I'm hopeful that each of these appointments will be productive.

For the orthopedic workup on Monday, and for the carpal tunnel test, I really hope that the doctors will be able to give us some idea if Ethan is likely to need any surgery in the next few years. Ethan's surgery schedule will likely be a major factor in determining when I might go back to work.

At the very least, the surgery Tuesday should fix his umbilical hernia, so his belly button no longer looks like an elephant's trunk.  He's had that damn thing forever.  When we were at Minnesota for transplant the surgeons kept asking if they could fix it.  Unfortunately, they would ask about ten minutes before they wheeled him into the OR, and it was too late to arrange authorization from our then HMO.  It'll be nice to finally get it done.

They may also remove his central line, but we don't know for certain yet.  The nurses here drew blood for his transplant engraftment last Monday and shipped it off to Minnesota where they'll do the test.  If his engraftment is good, then we'll pull the line.  Ethan's had a line continuously since September 2009.  This is  his second line after he pulled out his first at T+7 for transplant 1.  It will be so nice not to have to worry about the damn thing getting infected or pulled and to be able to let Ethan have a normal bath.  When this thing comes out, I will throw it in the fire and dance around it, singing.

Unfortunately, it looks like we won't be able to get into the Ronald McDonald House.  There are about 15 families on the waiting list right now.  We can't even get on the waiting list until we get to town.  And it's unlikely that they'll burn through that list in the week that we're there.  So we'll be at the Days Inn.  I expect that we'll be dropping in at RMH pretty frequently for meals and to give Ethan some playtime, and to see some good friends that are still there.

With that, I should probably wrap this up.  We've got haircuts, pumpkin carving, baths and packing to do. Time to get moving.

Thanks for checking in.

-t