What is EDS?

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What is EDS?
EDS stands for Ehlers-Danlos syndromes which is a group of disorders involving the connective tissues in the body. These disorders typically have similar features that include hypermobility in joints, increased skin laxity or hyperelasticity, tissue weakening, and bruising. EDS is said to be hereditary and the genes involved in these syndromes are passed on to children from their parents.

There are various types of EDS with some types merged into one to have a simpler classification. One type of EDS is called “Classical type”. This type involves “classic” features of EDS like hypermobility in joints and skin laxity. The skin appears very fragile and easily gets bruised even with minimal trauma. Because of hypermobility in joints, dislocations and scoliosis are common complications of this type of EDS. This type is also directly transferred from a parent with EDS to his/her child. Another type of EDS is called the “Hypermobility” type and from the name itself, this type involves joint hypermobility as the main feature. “Vascular” is another type of EDS and this involves the easy rupture of blood vessels and bowel which may lead to sudden death. Other types are “Kyphoscoliosis” that involves spinal deformity, “Arthrochalsia” which makes affected persons very short in terms of height, “Dermatosparaxis” which is a rare type of EDS and presents with very fragile skin, and the “Tenascin-X deficient” type which also involves hypermobility in joints, skin fragility but without the skin atrophy scars often present in “Classical” types.

There is actually no known cure for EDS. Treatment and management is actually “supportive”. In most cases, physical therapy and occupational therapy is prescribed to help patients perform day-to-day activities. Some patients also require orthopedic instruments to correct deformity and/or to aid normal joint or bodily functions. In terms of medications, these are only based on what symptoms are present.

 

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