Ehlers-Danlos Syndrome (EDS) (also known as "Cutis hyperelastica") is a group of inherited connective tissue disorders, caused by a defect in the synthesis of collagen (a protein in connective tissue). Connective tissue helps support the skin, muscles, ligaments and organs of the body. Depending on the individual mutation, the severity of the syndrome can vary from mild to life-threatening. There is no known cure. Treatment is supportive.
Most common is Hypermobility type:
Diagnosis is made primarily on clinical observations. The major signs and symptoms include:
Loose, unstable joints that are prone to: sprains, dislocations, subluxations (partial dislocations), hyperextension (double jointedness)
High and narrow palate, usually resulting in dental crowding
Muscle weakness, often made worse by cold weather
Early onset of osteoarthritis (usually develops in mid-30s)
Cardiac effects: some degree of Dysautonomia or Valvular heart disease
There is no known cure for Ehlers Danlos Syndrome. The treatment is supportive. Physical therapy, occupational therapy, and orthopedic instruments (e.g., wheelchairs, bracing) may be helpful. One should avoid activities that cause the joint to lock or overextend.
As i already told you there is no specific therapy.
Surgical repair and tightening of joint ligaments require careful evaluation of individual patients, as the ligaments generally do not hold sutures.
If you have bruises you should be evaluated for other bleeding disorders.
You should go for 6 mothly ultrasound to detect aneurysms/heart problems in an early stage.
There is a risk of uterine rupture, bleeding and other complications of pregnancy.
So you have to go for cardiac evaluation every six months, and followed by gynaecological precautions and then joints to be evaluated.
This is purely symptomatic treatment and evaluation every 3-6 months.
Some individuals have negligible symptoms while others are severely restricted in their daily life. Extreme joint instability, pain, and spinal deformities may limit a person's mobility. Most individuals will have a normal lifespan. However, those with blood vessel involvement have an increased risk of fatal complications.
Ehler Danlos Syndrome is a lifelong condition. Affected individuals may face social obstacles related to their disease on a daily basis.
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