GSD

   GSD...what is it? Glycogen Storage Disease is a rare birth defect in which your liver is missing a gene. This disease is near and dear to my heart, my grandson Zachary was born with this missing gene. 1 in 100,000 children worldwide (1 in 20,000 in Mexico) will be born with this debilitating disease. It is caused by both parents of an individual with an auto-somal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition until your child shows signs of "not growing", usually by the 5-6 month check-up. What occurs is the liver stores any sugar that the body does not use which in turn will enlarge the liver and cause problems to other organs.
   The "medicine" for this disease to keep the child alive is.... are you ready... "cornstarch"! It is mixed with water & ingested or given though a G-tube every 2-3 hours. The reason cornstarch is so effective is that it is a very thick liquid  (typically used as a gravy thickener) and it takes 2-3 hours for the body to digest it (it breaks down at the same rate as the body uses it). This dose repeated every 2 to 3 hours, 24/7, 365 in order to keep their glucose level stable, This life-saving natural starch is the lifeline for a GSD patient.
   There is hope on the horizon! This disease has been cured in Maltese dogs! (GSD type Ia occurs naturally in this breed). Without treatment, all of the dogs die within hours of birth. Even with medical therapy, no dog with the disease had survived for more than 4 weeks when this work started and now they are all alive and doing well.
   We are very excited to be starting human trials soon to cure this dreadful disease.
Sadly, this rare disease has no Government funding to find a cure because the numbers are so low. We, the families & friends create fund-raising events to bring awareness to GSD.

Thank you from all GSD people looking forward to a more normal life!
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