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.


Anonymous said…
I wonder and many of us non-transplant attenuated MPS I pts/families have talked about is sleep issues a problem in MPS I in general regardless of sleep - and un-related to apnea. First being cortisol deficient his normal circadian ryhtm is naturally going to be off - normal is high in the morning, low at night but those of us taking hydrocortisone can vouch that ortisol repalcement doesnt quite re-balance the sleep issues circadian/morning-to-night levels of cortisol as well as normal non-med cortisol replacement thus probably another reason why he is not as tired at night - I am always more wide awake at night by every measure! Second you factor joint issues/comfort regardless if there is pain or not (pain will add another whole separate element to sleep) and you add in other meds which can likely mess with the body and you add in MPS in general - its very likely this disorder does have an impact on all MPS I pts.

Glad your almost home! Sorry for sp errors - am typing in the dark as my 5 yr old nephew sleeps behind me :)
Kelly Waddell said…
Just remember, we are laughing at you in love! :P We've all been there, and done that. And even with Melatonin (and cutting out the mid-day naps they don't want), the sleep issues are not completely solved. They are just better. And better is always good.

So glad to read that you will be going home soon. I pray that the tests go well, and the trip goes smoothly!
dehydration said…
This melatonin posting, completely useful..