Glycogen Storage Disease – StarchWars

Shaken, not stirred.

Posted on 29. Mar, 2007 by Jillian in GSD Resources, Glycogen Storage Disease, Glycogen Storage Disease Type 1A, corn starch

I often have people ask “Cornstarch? Really? Why? How?”. So, I thought it might be nice to write a post in response.

Glycogen Storage Disease type 1a kids like mine cannot regulate their own blood sugar. So we give them cornstarch every 4 hours, 24 hours a day. Even in the middle of the night. My third child (15 months) just barely started tolerating cornstarch, but is on an every three hours schedule, so yes, the time and amount vary with each child and the amount even varies with what time of day it is.

Since our kids have gastrostomy tubes (g-tubes) “starching” them is pretty easy. They have no choice. Some people prefer to drink the cornstarch, but it must be raw (uncooked) and it is a considerable amount- several tablespoons at least. That is a battle we have chosen not to fight-yet.

Every week, using a gram scale, we spend about an hour measuring cornstarch into small snack sized Ziploc bags http://www.ziploc.com. We then write the time that that dose must be given and the initial of the recipient since the dosages are different with all of our kids. Then we store all of the day time bags downstairs in a small drawer in the kitchen, and all of the night-time bags upstairs in the bathroom next to the boys’ bedroom. We have found that Rubbermaid makes a drawer that fits Ziploc bags perfectly.

Every night at 10:00pm starch, we lay out the 2:00am and 6:00am starch bags so we can tell at a glance if we’ve really woken up and starched them or if it was only a dream. Sleep depravation does crazy things to your memory.

To administer the starch all we do is pour a 3oz jar of ProSobee (soy) infant formula (http://store.enfamil.com/nursette_3_fl_oz.html ) into the Ziploc bag, and shake. It’s easy to feel if there are any clumps of cornstarch when you use a bag as opposed to a container. Then we suck up the mixture with two 60cc syringes, insert the tube into the kid’s g-button, slowly push in the cornstarch mixture, and rinse with about 15ccs of water. At night the boys don’t even wake up. It’s also important to shake the cornstarch instead of stirring because cornstarch will stick to the spoon. Pre-mixing the solution will also make the cornstarch less effective.

One last tip. Argo and Kingsford brand cornstarch are the purest, last the longest, and apparently taste the best (http://cornstarch.com/index.htm) . We buy our Argo cornstarch in 25lb bulk packages at Winco Foods for only $20. For now that lasts about three weeks, not a bad price to pay for life-saving “medicine”.