Hi all,

This is going to be a quick update.  (Ha!  I've said that before!)

We're just starting to see how things are going for Ethan's renewed enzyme replacement therapy (ERT) and kindergarten (SCHOOL).  We're working on some things, but don't have solutions in place yet.

In a sense, it's too early to offer this update.  We don't have a lot of specific information to share.  But my classes are going to start soon.  If I don't get this update posted, it'll probably be a good while before I can get to it.

Ethan has had three ERT infusions.  He's handling the enzyme reasonably well.  We've noticed that he's not sleeping as well, generally waking up in the middle of the night and wanting to climb in bed with us. Or he's up at 5a and runs out of steam (and gets cranky) in the afternoon.  I'd say that he also appears to have more energy, generally, since starting back. But it's hard to say for sure, because it's only been a couple of weeks and he's naturally a high energy little boy.   As much as I might like it if he were sleeping more (and believe me, I'd really like it if he were sleeping more), I think it's a sign that he's got more energy or that he's feeling better and feeling more willing and able to be up and play. I want to say that he's talking more. I would't say he's talking bunches more, but it just seems to me that, well--you know--he is talking more.  (That descriptive enough for you?)  

The process of infusions is going relatively well.  When we did ERT before, we were up at Oregon Health and Sciences University.  Now we're at Legacy Emanuel.  They've got a brand, spanking new children's hospital building, and Ethan is the first patient that they've given Aldurazyme to. (Though, there's actually another Hurler's kid, who is in the study, who just moved to Portland.  She's getting her weekly ERT there as well.)  So far, the staff there is very good.  They're responsive and Ethan's meds come up from the pharmacy pretty quickly, which means we don't end up sitting around for a long time and, more importantly, it means we're out of there at a predictable time.

Ethan and I leave on the bike around 7:45.  Get up to the ward about 9.  Boogie out of the ward around 3 and we roll back in, at home, a little after 4.  It's an all day thing, but it's certainly manageable.

Sleeping off the Benadryl
The biggest hiccup with ERT is that Ethan doesn't want to take his oral pre-medications.  About 45 minutes before they start the enzyme, the protocol calls for patients to receive Tylenol (as a fever reducer) and Benedryl (as an anti-histamine).  But Ethan's gotten very, very resistant to taking oral medications. This is a kid who used to take 9-12 oral medications 2-3 times each day.  And he used to do really well at it.  Now, he fights us every step of the way, and if we do succeed in getting the medicine in his mouth, he won't swallow it.  He'll hold it in his mouth  for half an hour or more. And this is children's Tylenol, which is, like, bubble gum, or grape flavored or something.   In this case, the Tylenol is no big deal.  If we skip it, that's fine.  And we can give the Benedryl through his IV.  I cringe thinking about the day, sooner or later, when he's going to have to take something really foul tasting. And he's just going to have to take it, because he'll need the medicine.

While we're on the subject of things about ERT that Ethan doesn't like... He's not wild about having his port accessed.  His IV port is under the skin on his upper right breast.  It's about the diameter of a quarter, but it's about a 1/4 to a 1/3 of an inch tall.  It's a metal ring with a disc of rubber or silicone or some such self-sealing material in the middle.  The port is part of an IV line in his chest that runs up into his jugular vein in his neck.

This picture pretty well sums up
how Ethan feels about having his port accessed.
Though he's a lot less passive
during the putting in the needle part.
When we access the port, first we numb the skin over the port.  Before we leave the house, I apply some lidocaine cream on the skin over the port and hold that in place with some Glad press-n-seal (yes, that stuff). (Oh, did I mention that Ethan doesn't like the cream or the press-n-seal.  Getting that done and his shirt back on is a lotta fun, lemme just tell you. (Picture an octopus.)) Then, when we get to the hospital, they remove the plastic wrap and the cream and insert a largish needle into his chest.  (How big is a largish needle?  I haven't measured it, but I'd say it's about the same diameter as the needle they use when drawing blood.)  Once the needle is in, it stays in and they tape it down.



Ethan's first day/first time on a school bus
School is obviously the other new thing in Ethan's life.

Unfortunately, Oregon's full-day kindergarten law doesn't go into effect until 2015.

Ethan gets on the bus at 11:30.  School runs from 12:05 - 2:45 and he's home about 3:40.  Yeah, I know, that's a lot of time on the bus. (Believe me, I know. I'm particularly aware of it because Ethan tends to fall asleep on the way home.  He sleeps so much on the bus that he doesn't want to go to sleep at bed time.)

After two weeks, Ethan's had good days and bad days at school. Or, I should say, he's had good days and bad days going to school.   He often doesn't want to get on the school bus or he doesn't want to get off the school bus and go to class. But once we get over that significant hurdle, he loves being at school with the other kids.

Last Monday, I got a call from his teacher about 15 minutes after the bus arrived.  Ethan had gotten off of the bus, but refused to go into the school.  The school asked me to come by and help.  It's a 20 minute drive to his school, and on the way I stopped and picked him up some McDonald's french fries.  When I got to the school at 12:45, Ethan was standing outside, in the bus lane, with three staff members standing around him to prevent him from walking into the road.   He'd been standing out there for about 40 minutes and was in full melt-down at that point, crying and throwing his backpack at the teachers to try to get them to move out of the way.

If he'd have been that fussy for me, I would have carried him into the classroom and held him until he calmed down and realized that the other kids were playing and having fun without him.

As it was, the teachers aren't allowed to work with him physically. Really, the only thing they could do was try to talk to him and convince him to calm down and go to school.  Sorry, once he gets beyond a certain point, there's no way you're going to be able to talk down a stubborn kid at his level of development once he reaches full tantrum.  Hell, even I brought french fries to break the cycle with something positive and give him some time to settle.

The teachers went inside and Ethan and I sat on the curb, next to the bus lane and calmed down.  After about 15 minutes, Ethan was ready to go inside.  We found his classmates in the gym and Ethan ran right over to them and started playing.

The following day, Ethan was upset getting on the bus and I figured we were in for a repeat performance.  This time, I drove over to the school and met the bus there.  I hung out in the car until I saw that Ethan didn't come off the bus with the other kids. At that point I walked over and intervened, picked him up and carried him into class.  I sat with him through circle time and saw him get settled into the second activity, then snuck out.

That evening, his teacher and I talked by phone.  When I asked her, she said, yes, she would have preferred to have more time before I intervened.  And she's right, I should have given her more time to let her try different things.  But I also knew, there was very little chance he was going to settle down and cooperate.

Since then, we've started sending Ethan to school with one of his toys.  He loves showing his toys to the other kids and it helps give him something positive to focus on. That works some of the times, but it's not a panacea.

Are you seeing a common thread?  I think I've talked before about the fact that Ethan's become a stubborn kid, prone to tantrums, and throwing and scratching. I don't want to give the impression that he's always a terror.  These types of major tantrums may only happen once or twice a week. When he's in a good mood, Ethan's a great kid.  He's happy, quick to laugh, wants to cooperate and to please.  But we deal with less drastic lack of cooperation multiple times a day, whether it's getting ready to go somewhere, or changing a diaper or brushing teeth.

He's a kid, all kids are like that.  I get that.

But MPS sucks, and there are lots of things that Ethan's going to have to endure no one would like, whether that's taking some Tylenol or going to school or getting his port accessed, or even just brushing his teeth.

And MPS sucks because it has affected his hearing and his brain, which has delayed his ability to communicate effectively, making reasoning with him about the same as reasoning with a 2-3 year old.  Except Ethan is 5 and about as big and strong as most 5 year olds.

Too often, Sarah and I feel like we don't have any tools to work with him positively and effectively.  It becomes a vicious, negative cycle.  He won't do something that we need to do, changing a diaper, or brushing  teeth, or getting his port accessed, so we have to hold him down to get it done.  So he hates it more and fights more next time.  It's a fight that Sarah, Ethan, and I are all losing.

We're losing the fight, and we're his parents.  We can hold him, not harshly, but physically hold him, to help him settle or to accomplish whatever it is that needs doing.  His teachers, his nurses, they can't do that.  If talking him down doesn't work, they can sit there with him, but they can't get him into the classroom or get him to take his medicine.

This has been an issue for a while.  In fact, we talked with his pediatrician about it in the spring, and it had been an ongoing issue even then. At that point, we'd agreed to wait until we did his annual neuropsych exam.  That got pushed back and moved to Minnesota to coincide with the ERT study.  So his behavior has just stewed and simmered.

We finally got the results of Ethan's neuropsych testing and it's about as expected, so we pushed again for some sort of referral for help with his behavior and we got the name of a pediatric behaviorist.  This is a pediatrician who has special training in medically related behavior issues.  We feel fortunate to have found someone who did some work at NIH focusing on metabolic diseases like Hurler.   We're scheduled to meet him three times between mid-October and early-November, so hopefully soon we'll have some new tools to put in our bag o' tricks.

Home/Hospital Services

As we work through that, we're also gearing up for some potentially difficult discussions with the school system about home/hospital instruction for Ethan.

When we were planning Ethan's resumption of ERT, we expected that Ethan would do the first three months of infusions in the hospital, then we would move to home infusion.  With home infusions, we expected that Ethan wouldn't miss much or any of his half-day kindergarten. 

It turns out, we didn't really think that through very well.  The first thing we realized is that Ethan is in afternoon kindergarten.  So clearly, we wouldn't be able to start a six hour infusion at 4:00 pm.  Heck, even if he was in a.m. kindergarten, we would't have had time to start it if we're working with a home care nurse that clocks out at 5 p.m..   Then we found out that Sarah's health insurance doesn't cover home infusion services unless the patient is home bound.  Sure, we could appeal all of that with the insurance company, but we've fought enough insurance battles and I'm sure we'll have more in the future.  When we added it all up, we decided to stay with the infusion center at the hospital. 

Well, that means that Ethan's going to be out of school one day a week for the next 2 years.   

I called the district special ed director to arrange for home or hospital instruction to cover some of the time missed.  Her response was that even missing one day a week, Ethan would still receive all of the instruction called for in his individualized education plan (IEP).   

She said that the district would arrange home or hospital instruction for a student who would be out consecutive days.  But, since Ethan was only out one day a week, it was feasible to include that material in class the next day. 

So, wait a minute, I've got a developmentally disabled child, who is going to miss 1/5 of his instructional hours to get medical treatment, and we're just okay with that?  

I understand that the ratio of classroom instruction to home or hospital tutoring is something like one hour for every full day of school missed.  So we're really only talking about 1/2 hour of tutoring a week.  But next year, if he's in a full-day first grade program, we're still just okay with him missing a day a week?

Sarah and I have started researching the law on this to see what provisions are available.  The U.S. special education law (IDEA) and Section 504 (which covers students with medical issues) both state that educational services should be available in any necessary setting (school, home, hospital, institution, etc..) but evidently, it's left to the states to define the programs that implement that requirement.  Massachusetts law, for example, states that home or hospital instruction is available to any student missing more than 2 weeks of school in an academic year, even if those days aren't consecutive.  But there's no similar statute in Oregon. 

It is not a completely bleak picture.  There is a state funded hospital school at Legacy.  I talked to the teachers there last week.  They are happy to work with Ethan on a weekly basis even without the support of Ethan's home district.  They've been fantastic.  BUT, I really want to have it spelled out that Ethan's home district has a responsibility to provide that instruction. 

At the moment, we're still researching this.  I've got some inquiries out to an Oregon MPS VI family that's done ERT for years. Hopefully, they'll be able to tell us that, yes, they received home/hospital instruction as a part of their daughter's IEP, which would indicate that there's a precedent in the state.   And I've got a call into the education person at the company that makes Ethan's enzyme. Hopefully, they'll be able to provide information about how this is handled in other states and that might lead us somewhere. 

Once we have a feel we've settled on a good argument and case, we're planning to request a meeting with Ethan's IEP team.  Hopefully, if we walk through this issue with the educators, we can incorporate the services into his IEP, then the administrators who gave us static will have to carry it out. 

Lol. So much for a quick update.  

See ya' in December.


Erica J. Thiel said…
I know your likely tired of me saying it but ask the Behavioral person what his thoughts would be on even 1-2 days a week of in-home intense therapy for Ethan, he does not have autism but has a lot of the traits (common w MPS I) and there are kids that have benefited from these programs.

Also if you where to go to home care eventually many school aged families do infusion on the wknd., was just talking to another family tonight whose daughter also has MPS I and is in Kindergarten and they infuse on Sun. You'd be surprised how flexible home care nurses are. I know of atleast 4 other families w school aged children doing the same.
Y said…
Todd, I am only repeating what Erica is saying, but the home infusion fight is something that will be worth your time with the insurance agency. We were fortunate enough to have a very good, and very flexible home care nurse that worked with us through a few schedule changes. Also, the infusion doesn't generally take 6 hours if the nurse isn't waiting for the pharmacy and is completely dedicated to Ethan's care. You can also look at having the infusion time reduced (we're down to 3 hours for Max's infusion). I think letting Ethan play at home during the infusion may help reduce his stress and make it a more positive experience for all. We used to do the infusion at the hospital and would spend at least 6 hours in a hospital room which was never fun and left both Max and I really stressed out and pissed off at the end. Finally, I believe it's cheaper for insurance companies to pay for home infusions and mail order pharmaceuticals than it is for a hospital room and everything that goes along with it.