Glycogen Storage Disease- What to Expect the First Year

Posted on 31. Jul, 2007 by Jillian in corn starch, Education, Glycogen Storage Disease, Glycogen Storage Disease Type 1A, GSD Resources, Hospitals

Babies with GSD 1a can be a bit of a challenge. The most obvious challenge is controlling blood sugar. Each bundle of joy is different, but in general they are between 9 and 18 months before they can tolerate the magical cornstarch “medicine”. So, what do you do in the meantime?

While we were in the NICU with our third baby (the first to be diagnosed in infancy), we set up a schedule that maintained his blood sugar levels. Every two hours he would be fed 1 ½ ounces of Enfamil ProSobee and I would nurse him on the odd hours. Obviously, his dosage went up as he grew, and we discontinued breast feeding after 3 weeks. With our fourth baby, we chose not to nurse, just feed 1 ½ ounces every two hours. What he didn’t eat, we tubed.

Some parents choose to continue to feed every two hours throughout the night- usually via ng or g-tube. With other GSD kids in the house, this was a bit too impractical for our family. We choose to hook him up to a feeding pump at night. The pump is scary because the tubing can become disconnected at night, and transitioning him off of a continuous feed is tricky as well. Their insulin levels are higher during a feed, so after about 20 minutes off of the feed, they plummet. Scary, and not fun to deal with. This is a photo of three of our boys on feed pumps during their bout with RotaVirus. Normally, only the baby is using a pump at night.

While we love g-tubes, it is also important to only use them as necessary. Many infants lose the concept of “I put something in my mouth, I swallow, and I feel full.” if they are fed too much through their g-tube. I am fortunate enough to have two Speech and Language Pathologist sisters whose job it is, in part, to re-teach how to eat. So far we’ve avoided many problems, but it has been a lot of work for poor babies.

We have also had to deal with developmental delay. Even with good metabolic control, our GSD babies have had a few issues- mostly due to not wanting to lay on their tummies with a g-tube. They were also somewhat late in wanting to sit up without assistance. I found the best product for that! I had been working for weeks trying to get our baby to sit by himself. We’d done the normal- sitting him on the floor with pillows around him, and the extreme- sessions of balancing him on exercise balls to improve balance. My aunt sent us an excellent new baby chair by the Prince Lionheart company called a “bebe pod”. WE LOVE IT!!!! He could finally sit up and play with his toys while watching his brothers run amuck. Within two weeks he could sit without assistance. When our fourth baby was showing the same signs, we got out the babe pod, and without the crazy therapy sessions he could sit without a problem. Thank you Aunt Debbie!

Starting solid foods is also a bit of a dilemma. I still haven’t figured that one out completely. It is very difficult to control blood sugar while introducing new foods and the child becomes more and more active. The time between introducing solid foods and becoming more active, and the time the infant tolerates cornstarch, is by far the most difficult in the first year. We’ve just begun that stage again with our fourth baby and I believe it just might drive me crazy this time- as if I weren’t crazy enough already.

Disclaimer: Every child is different. It is important to follow your specialist’s advice. Our pediatrician fought us tooth and nail about nursing him even a little bit. Breast milk contains lactose which is not good for GSD babies. We took precautions to monitor lactate levels, and it was only used as a supplement for the first few weeks of his life. We, as parents, felt very strongly that I should. We didn’t feel the same way with our next baby. I’ve learned that it’s important to trust parental instincts after getting as much medical information as possible. Perhaps this is why we like Dr. Weinstein so much. He doesn’t push us in any direction, he just gives us the information we need to make good decisions regarding their health care.