We start week 4 of the miglustat treatment today. The dosage he actually takes is small for now, as his body gets accustomed to the medicine. We had a rough start to begin with, however.
Miglustat requires a bit of a restrictive diet, especially one for a 2-year-old who can be a picky eater when he wants, is used to getting a Carnation Instant Breakfast (TM), all the mashed potatoes and mac & cheese he can eat, and occasional “cookie night with Daddy”. He now has to be carb-restricted and lactose-free. So right off the bat, he had some problems.
The lack of sugary goodness make him incredibly lethargic for almost the entire first week. We also had an issue that because he was so tired, he didn’t feel like eating, which caused a problem in administering the Ibuprofen he now takes three times a day, always with food. The only good part was that he slept in, which helps Daddy a lot.
By Thursday night, however, he and I had a great dinner, he ate pretty well (steak), but as usually he was ready to head to bed early. We closed the curtains in his room, and were preparing to get his PJ’s when he started crying for a moment, and then (hope you’re not eating right now) he spewed slightly used steak and other assorted former yummies all over the floor. He then cried for a bit, and plopped his head on my shoulder, spent. I made sure he was okay, got him in his PJ’s, and cleaned up as best I could while he crashed on my couchbed.
The next morning, I knew his tummy might be still a little upset, so I added the miglustat to some water, which he lazily drank over the course of an hour or so. We watched some TV and rested, but as he seemed to get more tired, he got cranky, began to drag his head along, then threw up all his water and medicine. This came with some dry-heaves and was just a visibly terrible experience for him. Finally, the following (Saturday) morning, he threw up his sausage and eggs breakfast, before promptly collapsing in sleep.
Something obviously had to give.
We reduced his dosage by half, as we wanted to try to get him some medicine, and see how that went. OH BOY….
We’ve had zero emesis since then, and the child now eats like a MAN. Sometimes four meals in a day, or double helping at breakfast. Multiple eggs, sausage patties, burgers (2 at a time!!), steaks, blueberries, strawberries, asparagus, celery, now even some shrimp! And I found some lactose-free low-sugar ice cream bars he can have after dinner. The boy throws down on some food, is unrelenting, and has a fit when he’s hungry. I love it — except for the fact now that our lazy mornings have turned into him kicking me until I’m up and awake and making breakfast NOW DAD NOW I’M HUNGRY PLEASE FILL MY TINY BOTTOMLESS BELLY OR I WILL WAKE THE NEIGHBORS!!! It’s like I’m living with the dog from the Beggin’ Strips commercial.
With the blessing of Dr. Utz, we continued with the half dosage for 2 weeks (ending yesterday). So today, we went half-dose in the morning, and half-dose with dinner. All seems to be well so far (toes crossed — can’t cross fingers while I’m typing).
Other notes: I’m not sure if it’s from the behind-kicking he took that first week or what, but his ataxia (trouble walking) seems to be getting worse. I’d like not to think that it’s degeneration, and that he’ll bounce right back soon. We are looking at getting him a new walker, one that has straps and support built in so he doesn’t fall. On a slightly more heartbreaking note, he’s losing strength in his eyes, at least in his ability to keep them straight, so much of the time he has a slightly crossed eye. When he looks at you, it’s both heartbreaking and adorable. Part “dawwwwwww”, part ” 😦 “. His vision seems to be fine, as he can still spot a paci from 20ft. away, and still happily watches Thomas The Train while I’m in the shower.
On a more positive note, we raised nearly $800 from the restaurants who donated to our cause on Sept. 10th, so thank you to the owners and managers who helped out, as well as you kind and hungry readers. We are planning another round on his birthday, March 18th, and all who had participated in this go-round have pledged to do so again, but we are looking to add quite a few more. We also moved the gamer day to the day before, March 17th (anyone know any leprechaun video games?), and we also have a couple other ideas planned for that weekend.
Also, he has a new bed, happily donated to him by his older cousin Nate. So no more aerobed, he now sleeps in his own almost-big-boy toddler bed, at least for the half a night that he sleeps in his own room before he wakes up needing some Daddy snuggle time.