Ehlers-Danlos Syndrome (EDS)

OVERVIEW FROM THE MAYO CLINIC

 MAYO CLINIC LINK FOR FULLER INFORMATION

DEFINITION

Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provide strength and elasticity to the underlying structures in your body.

INHERITED

Different types of Ehlers-Danlos syndrome are associated with a variety of genetic causes, some of which are inherited and passed on from parent to child. If you have the most common form, hypermobile Ehlers-Danlos syndrome, there's a 50% chance that you'll pass on the gene to each of your children.

TREATMENT

There is no cure for Ehlers-Danlos syndrome, but treatment can help you manage your symptoms and prevent further complications.

Pain Management. Over-the-counter pain relievers — such as acetaminophen (Tylenol, others) ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve) — are the mainstay of treatment. Stronger medications are only prescribed for acute injuries.

Physical therapy Joints with weak connective tissue are more likely to dislocate. Exercises to strengthen the muscles and stabilize joints are the primary treatment for Ehlers-Danlos syndrome

LIFESTYLE AND HOME REMEDIES

If you have Ehlers-Danlos syndrome, it's important to prevent injuries. Here are a few things you can do to safeguard yourself.

Choose sports wisely. Walking, swimming, tai chi, recreational biking, or using an elliptical machine or a stationary bike are all good choices. Avoid contact sports, weightlifting and other activities that increase your risk of injury. Minimize stress on your hips, knees and ankles.

Rest your jaw. To protect your jaw joint, avoid chewing gum, hard rolls and ice. Take breaks during dental work to close your mouth.

Wear supportive shoes. To help prevent ankle sprains, wear laced boots with good arch support.

Improve sleep. Body pillows and super-dense foam mattresses can provide support and cushioning for painful joints. Sleeping on your side may also help.

Coping and support

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. 

Increase your knowledge. Knowing more about Ehlers-Danlos syndrome can help you take control of your condition. Find a doctor who's experienced in the management of this disorder.

Tell others. Explain your condition to family members, friends and your employer. 

Build a support system. Cultivate relationships with family and friends who are positive and caring. It also may help to talk to a counselor or clergy member. Support groups, either online or in person, help people share common experiences and potential solutions to problems.


LINK TO THE CLEVELAND CLINIC EDS INFORMATION 

SOME HELPFUL ADDITION INFORMATION FROM CLEVELAND CLINIC

DEFINTION

Connective tissues support your organs and hold parts of your body in place. They’re made of two proteins: collagen and elastin. EDS affects your body’s ability to produce collagen the way it should. People with EDS have weaker collagen. This means their connective tissue isn’t as strong or supportive as it should be.

Healthcare providers classify Ehlers-Danlos syndrome into 13 types based on where it affects you and the symptoms it causes. Experts estimate that 1 in 5,000 people has Ehlers-Danlos syndrome.

INHERITANCE

Most people with Ehlers-Danlos syndrome don’t have a known genetic mutation, so providers usually diagnose it based on your symptoms and medical history.

Most types of Ehlers-Danlos syndrome don’t affect or lower your life expectancy. 
If you have EDS that affects your blood vessels (vascular Ehlers-Danlos syndrome), you might have an increased risk of experiencing a stroke or other fatal vascular issues.



Hypermobile EDS
People with hEDS may have:

joint hypermobility
loose, unstable joints that dislocate easily
joint pain and clicking joints
extreme tiredness (fatigue)
skin that bruises easily
digestive problems, such as heartburn and constipation
dizziness and an increased heart rate after standing up
problems with internal organs, such as mitral valve problems or organ prolapse
problems with bladder control (urinary incontinence)
Currently, there are no tests to confirm whether someone has hEDS.

Classical EDS (cEDS) is less common than hypermobile EDS and tends to affect the skin more.

People with cEDS may have:

joint hypermobility
loose, unstable joints that dislocate easily
stretchy skin
fragile skin that can split easily, especially over the forehead, knees, shins and elbows
smooth, velvety skin that bruises easily
wounds that are slow to heal and leave wide scars
hernias and organ prolapse
EDS can be inherited, but it happen by chance in someone without a family history of the condition.

The 2 main ways EDS is inherited are:

autosomal dominant inheritance (hypermobile, classical and vascular EDS) – the faulty gene that causes EDS is passed on by 1 parent and there's a 1 in 2 chance of each of their children developing the condition
autosomal recessive inheritance (kyphoscoliotic EDS) – the faulty gene is inherited from both parents and there's a 1 in 4 chance of each of their children developing the condition
A person with EDS can only pass on the same type of EDS to their children.

For example, the children of someone with hypermobile EDS cannot inherit vascular EDS.

The severity of the condition can vary within the same family.