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While the severity of symptoms can vary widely, the most common ones are stretchy, fragile skin and extra-flexible joints.

EDS is divided into several types, based on a specific set of symptoms. The most common types are:

Classical type (formerly types I and II) EDS
This type affects about 1 in 10,000 to 20,000 people. Signs and symptoms include:

• Loose joints
• Highly elastic, velvety skin
• Fragile skin that bruises or tears easily
• Redundant skin folds, such as on the eyelids
• Slow and poor wound healing leading to wide scarring
• Noncancerous fibrous growths on pressure areas, such as elbows and knees; fatty growths on the shins and forearms
• Muscle fatigue and pain
• Heart valve problems (mitral valve prolapse and aortic root dilation)

Hypermobility type (formerly type III) EDS
This type affects approximately 1 in 10,000 to 15,000 people. Signs and symptoms include:

• Loose, unstable joints with many dislocations
• Easy bruising
• Muscle fatigue and pain
• Chronic degenerative joint disease
• Advanced premature osteoarthritis with chronic pain
• Heart valve problems (mitral valve prolapse and aortic root dilation)

Vascular type (formerly type IV) EDS
This type of EDS is rare, but it's one of the most serious. It affects an estimated 1 in 100,000 to 200,000 people. Signs and symptoms include:

• Fragile blood vessels and organs that are prone to tearing (rupture)
• Thin, translucent skin that bruises easily
• Characteristic facial appearance, including protruding eyes, thin nose and lips, sunken cheeks and small chin
• Collapsed lung (pneumothorax)
• Heart valve problems (mitral valve prolapse and others)

Other types of EDS
The other types of EDS are extremely rare. Some have 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.

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The types of Ehlers-Danlos syndrome are caused by a variety of genetic alterations (mutations), passed on from parent to child, 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 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 need only one copy of the disease-causing mutation, inherited from either parent, to develop signs and symptoms of the disease. If you inherit the mutation, each of your children will have a 50 percent chance of inheriting the mutation from you.

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Most people with Ehlers-Danlos syndrome live a relatively normal life, although there may be restrictions on physical activity. EDS doesn't affect your intelligence. Complications depend on your symptoms and type of EDS, but some common ones include:

• Prominent scarring
• Difficulty with surgical wounds — stitches may tear out, or healing may be incomplete
• Chronic joint pain
• Joint dislocation
• Early onset arthritis
• Premature aging with sun exposure

People with vascular type EDS are at risk of serious complications because of the fragility of blood vessels and organs. Serious complications of vascular type EDS include tearing (rupture) of major blood vessels, such as a ruptured or dissected artery or an aneurysm, or rupture of organs, such as the intestines or uterus. These complications can be fatal. About 1 in 4 people with vascular type EDS develop a significant health problem by age 20, and more than 80 percent develop complications by age 40. The median age of death is 48 years.

Some people with Ehlers-Danlos syndrome may develop osteoporosis. Osteoporosis is generally treated with prescription medications to increase bone density. Specific physical therapy exercises may help, too.

Pregnancy and EDS
Most women with EDS can have successful pregnancies. However, having EDS puts you at higher risk of premature delivery, postpartum hemorrhage and poor wound healing after delivery. Anesthesia during labor also can be difficult.

If you have vascular type EDS, pregnancy can be dangerous. The increase in blood volume and cardiac output puts more demand on your fragile blood vessels and organs, and there's a high risk of aortic or uterine rupture, both of which can be fatal.

If you have EDS and want to start a family, talk to your doctor first. Your doctor may suggest monitoring your pregnancy closely for any signs of complications. In some cases, your doctor may advise you against becoming pregnant because of the high risk of complications.

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What you can do
When you go in to see your doctor, take along a detailed family history, especially if family members have died of blood vessel or organ ruptures. Because EDS is an inherited condition, an accurate family history is important for diagnosis.

What to expect from your doctor
Your doctor will ask you detailed questions about your symptoms and family history. He or she will also complete a thorough physical examination. In many cases, a diagnosis of EDS can be made based only on your symptoms and family history.

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Your doctor will likely begin with a complete physical examination and a careful recording of your medical and family histories. Extremely loose joints, fragile or stretchy skin, and a family history of EDS is often enough to make a diagnosis of EDS.

Sometimes, additional tests are ordered to confirm an EDS diagnosis or to determine the type of EDS you have. They include:

• Genetic tests. If your family's genetic mutation is already known, a DNA test can confirm vascular type EDS. Prenatal DNA testing and preimplantation genetic diagnosis, a method that tests embryos obtained by in vitro fertilization, may be available.
• Skin biopsy. A diagnosis of vascular type EDS can be confirmed by checking for abnormalities in the collagen produced by your skin cells. In this test, a small sample of your skin is removed and examined under a microscope.
• Echocardiogram. To check for the heart conditions that can occur with some types of EDS, your doctor may recommend an echocardiogram, which is an ultrasound of your heart. An echocardiogram provides real-time images of your heart in motion, and can help identify abnormalities in the heart muscle and valves.

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There is no cure for EDS, but treatment can help you manage your symptoms and prevent further complications.

Physical therapy
Your doctor may refer you to a physical or occupational therapist with specific exercises to strengthen your muscles without causing injury. For most people with EDS, strengthening muscles helps to stabilize joints and reduce muscle fatigue and pain. In addition, your doctor or physical therapist may recommend specific braces to help stabilize joints.

Drugs
Your doctor may prescribe medication for joint or muscle pain, including nonsteroidal anti-inflammatory drugs or topical anesthetics.

Surgical considerations
In rare cases, surgery is recommended to repair joints damaged by repeated dislocations. However, healing from any surgery is difficult when 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.

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If you have a personal or family history of Ehlers-Danlos syndrome and you're thinking about starting a family, you may benefit from talking to a genetic counselor — a health care professional trained to assess the risk of inherited disorders. Genetic counseling can help you understand the inheritance pattern of the type of EDS that affects you and the risks it poses for your children.

©1998-2012 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.
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If you have EDS, it's important to prevent injuries and protect your skin and joints. Here are a few things you can do to safeguard yourself.

• Avoid injury. Avoid contact sports, weightlifting and other activities that increase your risk of injury.
• Use protective gear. Toddlers and young children with severe EDS often dislocate joints and frequently fall down, especially when learning to walk. Consider using protective clothing, guards or padding to protect your child from tumbles and falls.
• Reduce the clutter. To prevent falls and injuries at home, keep walkways and doorways clear of clutter. Avoid loose rugs and electric cords, which can increase your risk of tripping and falling.
• Use assistive devices. Several devices are available to help decrease stress on your joints, such as jar openers, utensils with wide handles, long-handled combs and bath sponges.
• Use mild soaps and sunscreen. To protect easily damaged skin and to guard against premature aging, use mild soaps and wear sunscreen when you're outside.

©1998-2012 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.
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Coping with a lifelong illness is challenging. Depending on the severity of your symptoms, you may face challenges at home, at work and in your relationships with others.

Here are some suggestions that may help you cope with the challenges of Ehlers-Danlos syndrome:

• Increase your knowledge. Knowing more about EDS can help you take control of your condition. Find a doctor who's experienced in the management of EDS and learn as much as you can about the type of EDS you have.
• Tell others. Explain your condition to family members, friends and your employer. Ask your employer about any accommodations that you feel will make you a more productive worker. It's up to you how much information you divulge to your co-workers. You may want to prepare appropriate responses for people who ask questions.
• Build a support system. Cultivate relationships with family and friends who are positive and caring. It may also help to talk to an unrelated third party, such as a medical social worker, counselor or clergy member. Some people find help by joining a support group for people with EDS or people with chronic illnesses. The Ehlers-Danlos National Foundation's Web site has information on local and regional support groups.

Helping your child cope
If you are a parent of a child with EDS, consider these suggestions to help your child:

• Maintain normalcy. As much as possible, treat your child like normal children. Ask others — grandparents, aunts, uncles, teachers — to do the same.
• Be open. Allow your child to express his or her feelings about having EDS, even if it means being angry at times. Parents of children with EDS have sometimes encountered suspicions of child abuse because of frequent bruises and cuts. Make sure your child's teachers and other caregivers know about your child's condition. Review with them appropriate caregiving skills, particularly in the event of a fall or injury.
• Promote activity. Allow your child to participate in physical activities with appropriate boundaries. Discourage contact sports while encouraging non-weight-bearing activities, such as swimming. Your child's doctor or physical therapist may also have recommendations.
• Find the best routine. Work with your child's teachers and school administrators to make any necessary modifications in his or her schedule or responsibilities. These modifications may include giving your child extra time to move from class to class, providing him or her with an extra set of textbooks so that these books won't need to be carried home, and making arrangements for assignments to be sent home when your child misses school because of his or her condition.

©1998-2012 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.
Read this article on Mayoclinic.com.