GSD Ia is characterized by hypoglycemia (low blood sugar). Failure of glucose metabolism causes glycogen accumulation leading to hypoglycemia, lactic acidosis causing low blood pH, coma, and death. Affected puppies are weak and they rarely survive more than a few weeks.

Management of affected puppies consists of a high-starch diet fed frequently throughout the day to control hypoglycemia with only small amounts of protein and fat in the diet to provide required nutrients. Experimental gene therapy has successfully treated this disorder in dogs.

There are many responsibilities to consider when breeding dogs. Regardless of test results it is important that your dog is in good general health and that you are in a position to care for the puppies if new responsible owners are not found. For first time or novice breeders, advice can be found at most kennel club websites.

This disease is autosomal recessive meaning that two copies of the mutation are needed for disease signs to occur. A carrier dog with one copy of the GSD mutation can be safely bred with a clear dog with no copies of the GSD mutation. About half of the puppies will have one copy (carriers) and half will have no copies of the GSD mutation. Puppies in a litter which is expected to contain carriers should be tested prior to breeding. Carrier to carrier matings are not advised as the resulting litter may contain affected puppies. Please note: It is possible that disease signs similar to the ones caused by the GSD mutation could develop due to a different genetic or clinical cause.