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Ehlers-Danlos Syndrome...less medical jargon in a 'Quick Glance' format!
Ehlers-Danlos syndrome is divided into six major groups, based on specific diagnostic criteria: . . . Hypermobility type This is the most common form. It may affect as many as one in 10,000 to 15,000 people. Signs and symptoms include: . . . Classical type This type probably affects about one in 20,000 to 40,000 people. Signs and symptoms include: . . . Vascular type This is one of the most serious forms of Ehlers-Danlos Syndrome. It affects an estimated one in 250,000 people. Signs and symptoms include: . . . Kyphoscoliosis type This uncommon form has few cases reported worldwide. Signs and symptoms include: . . . Arthrochalasis type Arthrochalasis is rare. Only about 30 cases have been reported worldwide. Signs and symptoms include: . . . Dermatosparaxis type This form is also rare, with only about a dozen cases reported worldwide. Signs and symptoms include: Other types There are other rare types of Ehlers-Danlos Syndrome. Some of these are so rare that they've only been described in a few families. In addition, the relationship of some of these types to the syndrome as a whole isn't well-defined. Causes: The subtypes of Ehlers-Danlos syndrome are caused by a variety of genetic alterations that disrupt the normal production of collagen. Collagen is a fibrous protein that gives strength and elasticity to connective tissues — skin, tendons, ligaments, cartilage, and organ and blood vessel walls. These genetic mutations, which are passed on from parent to child, alter normal enzyme activity, leaving connective tissues weak and unstable. . . . Variety of inheritance patterns Most EDS types are passed along in an inheritance pattern called autosomal dominant. This means you only need one copy of the disease-causing mutation, inherited from either parent, to develop signs and symptoms of the disease. If you inherit the mutation, there's a 50 percent chance that you'll pass the gene on to each of your children. Two EDS types — kyphoscoliosis and dermatosparaxis — are passed along in an inheritance patter called autosomal recessive. This means you need two copies of the mutated gene to develop the disease. If you inherit only one copy, you're considered a "carrier" of the disorder, but you likely won't develop signs or symptoms. Treatment: There's no way to reverse the genetic alterations that cause EDS. Treatment focuses primarily on managing individual signs and symptoms and preventing further complications. Much of this consists of self-care strategies to protect your joints and prevent excessive bruising and injuries. Your health care team may include specialists from a variety of different fields, including medical genetics, surgery, pediatrics, orthopedics, cardiology and ophthalmology, to name a few. . . . Surgical considerations If you're to undergo surgery, make sure your surgeon knows you have EDS. Your surgeon may use adhesive tape or medical glue rather than stitches to close up incisions, because stitches may tear out of fragile skin. Surgery is sometimes required to repair joints damaged by repeated dislocations. Elective surgery isn't recommended. . . . Managing pain If you have chronic joint pain, talk to your doctor about ways to help manage the pain. This might include use of pain relievers or physical therapy or both. A physical therapist can help you develop an exercise program to strengthen your muscles, which in turn will help stabilize your joints. In general, people with EDS need to avoid activities that put pressure on locked joints, such as weightlifting. Your therapist or doctor can prescribe appropriate exercises for you. Although a few reports indicate that vitamin C may help reduce the severity of kyphoscoliosis symptoms, this hasn't been established. Talk to your doctor if you're thinking about taking a vitamin C supplement.
Ehlers-Danlos Syndrome 
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