Glycogen Storage Diseases
PHS Home Care for Patients With Glycogen Storage Diseases
Pediatric home care is what we do. For more than two decades, PHS has provided compassionate, comprehensive, specialized, high quality in-home care to pediatric patients, no matter what their medical challenges.
A multidisciplinary PHS team collaborates to do everything possible to help our patients with glycogen storage disease live life to the fullest – at home.
- If intravenous hydration, nutrition or medication is ordered for home, PHS pharmacists, infusion nurses, and dietitians support the patient’s needs.
- PHS provides 24/7 on-call assistance to families receiving our service.
- PHS home care nurses provide scheduled visits or round-the-clock care.
- If respiratory support is needed, PHS respiratory therapists educate and support children, families and caregivers on home monitoring equipment, oxygen therapy and ventilator therapy.
- The PHS medical director attends team care rounds and presents medical updates and new treatment modalities in caring for patients with glycogen storage disease.
- A clinical social worker provides support in locating additional information, programs, services and community resources specific to the patient’s diagnosis.
- PHS provides medical equipment and supplies needed by a patient with glycogen storage disease.
Meet PHS Patient Leo
About Glycogen Storage Diseases
Glycogen storage disease is an inherited disorder caused by the buildup of a complex sugar, called glycogen, in the liver. When a person has GSD, the liver cannot control the use of glycogen and glucose. Certain enzymes are missing that control the change of sugar into its storage form, or release glucose from glycogen. Many sugars (including glucose) are found in foods and are used by the body as a source of energy. After a meal, blood glucose levels rise. The body stores extra glucose that is not needed right away as glycogen in the liver and muscles. Later, as the blood glucose levels in the body begin to drop, the body uses this stored energy.
These sugars, stored in the form of glycogen, need to be processed by enzymes in the body before they can carry out their functions. If the enzymes needed to process them are missing, the glycogen, or one of its related starches, can build up in the liver, causing problems.
There are at least 10 different types of GSDs, with some more understood than others. The types are put into groups based on the enzyme that is missing. The most common forms of GSD are types I,II, III and IV. About one in 20,000 people can have a type of GSD. The types of GSD include:
- Type O – Lewis’ disease – Liver
- Type Ia & b – von Gierke’s disease – Liver, kidneys, intestines; Ib – also impacts blood cells
- Type II – Pompe’s disease – Muscles, heart, liver, nervous system, blood vessels
- Type III – Forbes-Cori disease – Liver, heart, skeletal muscles, blood cells
- Type IV – Andersen’s disease – Liver, brain, heart, muscles, skin, nervous system
- Type V – McArdle’s disease – Skeletal muscles
- Type VI – Hers’ disease – Liver, blood cells
- Type VII – Tarui’s disease – Skeletal muscles, blood cells
- Type IX – Liver, muscle, rarely heart
- Type XI – Fanconi-Bickel syndrome – Liver, kidneys, intestines
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