Is Ehlers-Danlos life-threatening?

Description. Ehlers-Danlos syndrome is a group of disorders that affect connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of these conditions, which range from mildly loose joints to life-threatening complications.

Is Ehler Danlos fatal?

People who have vascular Ehlers-Danlos syndrome are at risk of often fatal ruptures of major blood vessels. Some organs, such as the uterus and intestines, also may rupture.

Can you live a full life with EDS?

EDS cannot be 'cured' but many people learn over time how to control it and live full and active lives. The day-to-day management of most types of EDS is based around the right kind of exercise, physiotherapy and pacing. In addition you should seek referrals for any associated conditions you may have.

What is the life expectancy of a person with EDS?

The median life expectancy for individuals with vascular EDS is around 48 years. Patients with kyphoscoliotic EDS — whose hallmark is a sideways curvature of the spine in combination with a hunched back — also may have a reduced life expectancy.

Is hypermobile EDS life threatening?

Patients with the classical and hypermobility forms of Ehlers-Danlos syndrome have a normal life expectancy.

EHLERS-DANLOS SYNDROME: SUPER FLEXIBLE, OR LIFE-THREATENING?

Does Ehlers-Danlos shorten life expectancy?

The long-term outlook (prognosis) for people with vascular Ehlers-Danlos syndrome is generally poor. It is often associated with a shortened lifespan. Among affected people diagnosed as the result of a complication, 25% have experienced a significant medical complication by age 20 and more than 80% by age 40.

Does EDS get worse as you age?

Many of the problems associated with EDS are progressive, meaning that they get worse over time. Kyphoscoliosis EDS (kyphosis and scoliosis) is a form of Ehlers-Danlos Syndrome that is noted for severe, progressive curvature of the spine. It worsens over time and may affect breathing by restricting lung expansion.

Does EDS qualify for disability?

If you have EDS and are unable to work because of severe symptoms from it, you may be eligible for disability benefits, including Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI).

What is the most common cause of death among individuals with vascular Ehlers Danlos?

The major cause of death is arterial dissection or rupture with organ failure.

Does EDS affect the heart?

Cardiac-valvular EDS (cvEDS) affects the connective tissue that forms the heart valves. These valves control how the blood flows out of the heart with every heartbeat. Patients with cvEDS have progressive weakening of the heart valves and may have problems such as high blood pressure.

What age does EDS usually start?

It is usually diagnosed before the age of 2 years. Fragility, bruising and sagging of the skin are severe but, surprisingly, the skin heals well. Like the other rare types, in order to inherit it you need to inherit one faulty gene from EACH of your parents.

How fast does EDS progress?

My geneticist told me that it often takes an average of 10 to 20 years to receive a diagnosis of EDS, with many people not receiving an accurate diagnosis until well into their 40s. As with many medical conditions, EDS disproportionately impacts women.

At what age is EDS diagnosed?

The age at first diagnosis peaked in the age group 5–9 years for men and 15–19 years for women (see figure 2). There was a significant difference of 8.5 years in the mean age of diagnosis between men and women (95% CI: 7.70 to 9.22): 9.6 years in EDS (95% CI: 6.85 to 12.31) and 8.3 years in JHS (95% CI: 7.58 to 9.11).

Does EDS make you look younger?

Due to the collagen in the skin being extra stretchy, many EDS patients experience a lack of wrinkles as they age. This collagen may help EDS patients look younger and can also cause skin to feel extremely soft. Doctor's even describe the skin as “velvet-soft”.

How painful is Ehlers-Danlos syndrome?

Chronic pain in the Ehlers–Danlos syndromes, especially in hypermobile EDS, is very common and may be severe. It may be widespread or it may be limited to one area of the body such as a limb. Headaches and gastrointestinal discomfort can occur as well as joint, muscle and nerve pain.

Is vascular EDS a death sentence?

I know that you're really passionate about bringing awareness to the fact that vascular EDS - it's not a death sentence, and so I appreciate you talking about that.

What mimics Ehlers-Danlos syndrome?

Tnxb-/- mice showed progressive skin hyperextensibility, similar to individuals with Ehlers-Danlos syndrome. Biomechanical testing confirmed increased deformability and reduced tensile strength of their skin.

Is Ehlers-Danlos type 3 rare?

Hypermobile Ehlers-Danlos syndrome (hEDS), which used to be known as the hypermobility type or type 3, is thought to be the most common genetic connective tissue disorder. There is no up-to-date research to tell us exactly how frequently it occurs.

Are you born with vascular EDS?

Vascular EDS is a genetic condition. It can start for the first time in someone, or be inherited from a parent. Once someone is diagnosed with vascular EDS we know there is a 50% (1 in 2) chance for any children to have inherited the condition.

What famous people have Ehlers Danlos?

2019 brought much hope to the long-silent-suffering Ehlers Danlos Community when Lena Dunham, Jameela Jamil, and SIA revealed their EDS diagnoses.

What serious health problems can come with EDS?

There are many neurological and spinal problems which have been observed to be more common in EDS, examples include migraine, early disc degeneration, Chiari 1 malformation, craniocervical instability, motor delay, and curvature of the spine. Musculoskeletal pain can start early and be chronic.

Is EDS an autoimmune?

In the United States, two million people live with rheumatoid arthritis, and another 1.5 million live with lupus. A much rarer group of connective tissue disorders is called Ehlers-Danlos syndrome. Unlike the diseases noted above, Ehlers-Danlos syndrome is not an autoimmune condition, it's an inherited disorder.

Does EDS affect the brain?

Because EDS is a connective-tissue disorder, it is not commonly associated with the brain or the nervous system. However, there is evidence that some types of EDS can affect the brain. Studies have suggested that patients with EDS might be susceptible to damage to brain cells after even a mild traumatic head injury.

What is the best treatment for Ehlers-Danlos syndrome?

Physical therapy

Exercises to strengthen the muscles and stabilize joints are the primary treatment for Ehlers-Danlos syndrome. Your physical therapist might also recommend specific braces to help prevent joint dislocations.

Can EDS cause hair loss?

Lena Dunham has opened up about suffering from hair loss, three months after revealing she has Ehler-Danlos syndrome (EDS). Dunham said her hair loss had been caused by an autoimmune disease but did not disclose the name of the condition.