Discontinuing Continuos Feeds

Posted on 29. Aug, 2007 by Jillian in corn starch, Glycogen Storage Disease, Glycogen Storage Disease Type 1A, hypoglycemia

A necessary evil until the kids tolerate cornstarch, continuous feeds are a blessing and a curse.

When our older boys were first diagnosed with GSD our former GSD specialist put them on cornstarch every four hours during the day, and then on a continuous feed during the night so that we could “sleep through the night” (any GSD caregiver will now, undoubtly, be laughing hysterically at the thought). The dream of sleeping through the night turned into a nightmare. The boys would toss and turn so much during the night that the tube on the feeding bag that connected to the g-tube in their stomach would invariably come apart during the night. This is bad for two reasons.
#1. It means the boys aren’t getting enough calories to keep their blood glucose levels up, and
#2. With the tube unkinked, it means that any formula in their stomach could now leak out through the same tube.

I believe I can speak for all GSD 1a parents out there when I say “AAAAHHHHH!!!!!”

To help prevent the tubes from disconnecting, we used every type of tape we had, including, but not limited to, masking, medical, electrical and even duct tape. It helped, but we were still up at least once a night to reconnect the tubing and change bed sheets where the formula had leaked. We also bought a bed wetting alarm pad which was the best thing we could have done. If the tubes came apart, they would leak on the pad which would then send an ear-piercing alarm to wake up the parents while the boys slept peacefully through it. It definately isn’t fool-proof, but it does help considerably.

To make things worse our former specialist did not know, and therefore did not tell us, that when a person with Glycogen Storage Disease type 1a is put on a feeding pump their insulin levels raise to balance their blood sugar. When you discontinue a continuous feed, their insulin levels are still set at high, and their blood sugar drops even faster than it normally would without food.

We were told by our former “specialist” that the best way to transition them off of a continuous feed was to simply stop the feed and give them a dose of cornstarch in a couple of hours. There was also no need to test their blood sugar since they were just getting plenty of calories while on the feeding pump. It should come as no surprise then that while the boys were significantly more active and alert after being put on cornstarch, within a month or so their progress had again plateaued.

After a year of incomplete GSD treatment we were finally able to go to Florida and see Dr. Weinstein. What a wonderful experience. The boys gained more from his treatment than I ever thought possible. They still continue to improve. They both had huge growth spurts, and their minds have been much more alert. My oldest son had 3 therapists at the time that all independently mentioned that he had improved more in the first month right after Dr. Weinstein, than he had in the entire 9 months they had been seeing him previously. What more of a testimony to do 24 hour cornstarch do you need?