EDS stands for Ehler's Danlos Syndrome.
What is EDS?
EDS is a group of inherited disorders marked by extremely loose joints, hyperelastic skin that bruises easily, and easily damaged blood vessels. Individuals with EDS have a defect in their connective tissue, the tissue that provides support to many body parts such as the skin, muscles, and ligaments. The fragile skin and unstable joints found in EDS are the result of faulty collagen. Collagen is a protein, which acts as a 'glue' in the body, adding strength and elasticity to the connective tissue.
It says that EDS is a 'group of inherited disorders.' So that means there's more than one type?
Yes, in fact, there are a large amount of types of the disorder.
- CLASSICAL TYPE ~ Type I and II; it affects approximately 1 in 20,000 to 50,000 people. Type I typically presents with severe skin involvement, and type III presents with mild to moderate skin involvement.
- HYPERMOBILITY TYPE ~ Type III; it affects 1 in 10,000 to 15,000. Mutations in either of two separate genes cause this disease, not a parent passing it down. Extreme hypermobility is the hallmark of this type. (I have this type)
- VASCULAR TYPE ~ Type IV; it affects approximately 1 in 100,000 to 250,000. The vascular type is considered one of the more serious forms of EDS because blood vessels and organs are more prone to tearing. Roughly, 1/4 of people with vascular type express a characteristic facial appearance (large eyes, small chin, thin nose and lips, lobeless ears), have a small stature with a slim build, and typically have thin, pale, translucent skin (veins can usually be seen on the chest and abdomen). About one in four people with the vascular type develop a significant health problem by age 20 and more than 80% develop life threatening complications by age 40.
- TYPES 6, 7A, 7B, & 7C ~ Kyphoscoliosis, Arthochalasis, & Dermatosparaxis; the rarest types.
There's no cure?
Nope, no cure for EDS. But different studies have shown that high doses of Vitamin C (2 g) help to preserve the joints a little longer. Vitamin C is one of the building blocks of collagen.
So how is EDS managed?
The treatment is supportive. Close monitoring of the cardiovascular system, physical therapy, occupational therapy, and orthopedic instruments (i.e. wheelchairs, bracing) may be helpful. The patient should avoid activities that cause the joint to lock or overextend. A physician may prescribe bracing to stabilize joints. Surgical repair of joints may be necessary at some time. Physicians may also consult a physical and/or occupational therapist to help strengthen muscles and to teach people how to properly use and preserve their joints. To decrease bruising and improve wound healing, some patients have responded to ascorbic acid (vitamin C). In general, medical intervention is limited to symptomatic therapy. Prior to pregnancy, patients with EDS should have genetic counseling. Children with EDS should be provided with information about the disorder, so they can understand why contact sports and certain other physically stressful activities should be avoided. Children should be taught early on that demonstrating the unusual positions they can maintain due to loose joints should not be done as this may cause early degeneration of the joints. Family members, teachers and friends should be provided with information about EDS so they can accept and assist the child as necessary.
