Wise Choices for You & Your Family
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WHAT IS:
GLYCOGEN STORAGE
DISORDER (GSD) Some types of GSD cannot be treated and some very severe
cases may lead to liver, heart and respiratory failure. However,
when a child has a form of treatable GSD, the treatment plan
may help reverse the symptoms of GSD and allow him to live
a normal life.
For patients that are diagnosed with a type of GSD that affects
the liver, the main goal of the treatment plan is to keep the
blood sugar at a normal level. Infants under the age of two
are sometimes fed glucose through a feeding tube to keep
the blood sugar stable. Patients over the age of two are fed
small portions of carbohydrates during the day to keep the
blood sugar at a normal level. Some patients with a type of
the disease referred to as Type IV experience liver failure and
benefit from a liver transplant.
Patients diagnosed with the types of GSD that affects the
muscles are put on a treatment plan designed to help avoid
the muscle cramps and fatigue brought on by exercise. This
is done with a combination of a carefully planned diet and a
carefully regulated exercise plan.
Glucose is a simple sugar that is the main energy source
for our body and we get it from many of the foods we eat.
Normally, our bodies use proteins called enzymes to turn
glucose to glycogen. The glycogen is then stored in the
liver and muscles for the body to use as energy later. When
the energy is needed later by the body, other enzymes will
convert the glycogen back into glucose. Glycogen Storage
Disease (GSD) is a group of inherited metabolic disorders
caused by a defect in the genes that govern the production
of these enzymes. The result is an abnormal build-up of
glycogen in the liver and muscles.
GSD is usually diagnosed in childhood. The symptoms of
GSD will vary between different types of GSD but most
types have these symptoms in common: enlarged liver,
slow growth, muscle cramps & low blood sugar.
HOO’s Healthy
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