Glycogen storage disease ixa


What should I do if my child has glycogen storage disease IXa?

Children with glycogen storage disease IXa (GSD IXa) should have their livers checked by a liver specialist, also known as a hepatologist. An endocrinologist that specializes in glycogen storage disorders may also be involved and assist in providing guidance with nutritional support to prevent hypogylcemia (low sugar levels). A geneticist may also serve as a resource to assist in the medical management of a child with GSD IXa and serve as a liason between the other specialists involved in your child's medical care.

Some doctors recommend children with GSD IXa should also have a cardiac (heart) evaluation. A dietitian may also be a helpful resource to assist in establishing a feeding schedule for your child to prevent prolonged fasting that can lead to hypoglycemia.

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What is the treatment for glycogen storage disease IXa?

What is the treatment for glycogen storage disease IXa?

People with glycogen storage disease type IXa (GSD IXa) have trouble retrieving stored glycogen from the liver for energy in between meals. Instead of providing energy, the glycogen can build up in the liver and muscles, causing low blood sugar, seizures, lethargy and difficulty breathing if left untreated. The treatment for GSD IXa is to make sure your child eats frequently to keep their blood sugar levels up. A nutritionist or dietitian can work with you to develop a healthy diet and feeding schedule for your child. Your medical team may also recommend your child eat a mixture of cornstarch and water to make sure they have enough energy in their body throughout the night. Symptoms of GSD IXa usually improve with age as a child grows and is able to sustain longer periods of fasting. Fasting can also occur in the setting of illness or during periods of physiological stress like a surgery. It is important to be in contact with your child's doctors when your child is sick or requires a medical or surgical procedure in which fasting is recommended. IV nutrition or a sugar solution may be provided during these periods of time to prevent life-threatening hypoglycemic episodes from occurring.

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