Glycogen Storage Disease – StarchWars

Sanctuary in Florida!

Posted on 03. Jun, 2007 by BMW in GSD Resources, Glycogen Storage Disease, research

Though the trip to Florida is challenging with four children under the age of 6, we come home recharged and more prepared to continue the non-stop battle of Glycogen Storage Disease. As my wife has mentioned in the previous post, we have learned and gained experience that helps us travel the country with our little ones while keeping them safe and us sane. What I want to write about though is the experience at the hospital and what we were able to come back with.

First of all the hospital stay was great. It’s a night and day experience staying at Shands Hospital in the CRC (Clinical Research Center) and any other hospital we’ve stayed in for GSD care. Instead of being on pins and needles the entire time wondering if the current nurse understands what is going on we felt that we could relax a little and not have to feel like we needed to continuously watch the nursing staff to remind them how critical a schedule they were on. There was no having to wait while everyone scrambles (or in some cases doesn’t scramble) to find some formula or cornstarch (normally we would bring our own from home) and try to track someone down to take sugar levels that are 15 minutes past due. It was actually somewhat of a relieving feeling being at Shands and our entire family was much less stressed. In fact, I was even able to enjoy playing Foosball and Video games with the kids in the play area.

The best part of course of the hospital experience was the amount of time that we were able to converse with Dr. Weinstein. He was there often to talk with us and to spend time with our children. We were constantly soaking up more information on Glycogen Storage Disease and it was great to have answers to our questions. He addressed every concerned that we had and presented great insight, and modifications to the kids changing metabolic needs. He even smoothed things over with the school which was a tremendous blessing (I believe that my wife will be writing more on the school).

We were ecstatic to learn how well our children have been doing. All of the children’s test results came back as a normal child’s would. Their livers are looking great and our oldest two have recovered tremendously over this last year. Our first having had the most damage to overcome because of the length of time that it took to diagnose him (He was 3 and a half) did not have the incredible growth that our second but had recovered from weakened bones, an extremely inflamed liver, elevated cholesterol and triglycerides and chronic fatigue. He also made an incredible recovery in mental awareness and though we feel that permanent brain damage may be there he is working hard and making good progress in that area as well.

Our second also recovered from the same things that his older brother did, but not having the severity of damage he was able to also grow at an incredible rate this past year, and even though he is one and a half years younger then his brother, they are often mistaken as twins. This is exciting for us, since with the treatment that we had between diagnosis and finding Dr. Weinstein he had plateaued in growth.

The third in line just made it through the first year and a half of Glycogen Storage Disease survival and we were finally able to teach him the ways of the starch. Since cornstarch can’t be used in the first 9 – 18 months (of course he was closer to 18), that stage is particularly difficult to keep the kid stable. So it’s a huge relief to add one more kid to the 24 hour starch regimen.

Of course the youngest is still awaiting his turn to join his brothers in the starch clan, but he is doing well and we just need to keep him that way for another six months to a year. Hopefully he’ll cooperate and it will be closer to six months.

Since we were looking at puppy options for our GSD dog project, we were able to take the kids to see Little Red and the other GSD dogs. The kids loved it and though we may not adopt one of the non-carrying pups from that litter the kids were more excited about our pursuit of bringing a dog into the home.

Unfortunately our plane and hospital schedule did not afford for us to take the kids anywhere fun, but hopefully we can slot that in next year. For now… we need to tough it out another year and hope for the best.

May the starch be with you.