What are the Ehlers-Danlos Syndromes, what is the life expectancy of the disorder and what is joint hypermobility?

EHLERS-DANLOS Syndromes are a group of rare, inherited disorders which affect the body's connective tissue and can lead to spontaneous ruptures of major blood vessels.

Entrepreneur Trevor Jones, who previously appeared on Millionaire Matchmaker died earlier this month from the condition.

A family friend revealed that the 34-year-old had died from an 'event' linked to the syndrome on October 9.

So what are the symptoms, and does it affect life expectancy? Here's what we know…

What are the Ehlers-Danlos Syndromes?

EDS affects the body's connective tissue – which normally provides support in the skin, tendons, ligaments, blood vessels, internal organs and bones.

Some symptoms are shared between the disorders, including an increased range of joint movement (hypermobility), stretchy skin, and fragile skin that cuts and bruises easily.

For some, EDS is a mild condition – while, for others, their symptoms can be disabling. In rare severe cases, EDS can be life threatening.

Hypermobile EDS is the most common form of the disorder, while in rarer cases people have Classical or Kyphoscoliotic EDS.

Symptoms of cEDS can include hernias and organ prolapse.

While symptoms of kEDS include spine curvature, weak muscle tone from childhood and fragile eyes.

Vascular EDS is also rare, but it is the most serious form – because it affects the blood vessels and internal organs, and can cause them to split open, causing life-threatening bleeding.

Symptoms of vEDS include:

  • Skin with visible small blood vessels, especially on the upper chest and legs
  • Fragile blood vessels that can bulge or tear – resulting in serious internal bleeding
  • A risk of organ problems, such as the bowel tearing, the womb tearing in late pregnancy and/or a partial collapse of the lung
  • Hypermobile fingers and toes
  • Unusual facial features (thin nose and lips, large eyes and small earlobes)
  • Varicose veins
  • Delayed wound healing

What is joint hypermobility?

Hypermobility means that your joints are more flexible than other people.

If this causes you pain, it could be Joint Hypermobility Syndrome or Hypermobile EDS, which are very similar conditions. Symptoms include:

  • Joint hypermobility
  • Loose/unstable joints that dislocate easily
  • Joint pain/clicking joints
  • Extreme tiredness/fatigue
  • Skin that bruises easily
  • Digestive problems like heartburn and constipation
  • Dizziness or an increased heart rate after standing up
  • Problems with internal organs
  • Problems with bladder control

There is currently no test to confirm if someone has hEDS, and most patients can live a normal life.

You should see you GP if you:

  • Often get pain/stiffness in your joints or muscles
  • Keep getting sprains/strains
  • Keep dislocating your joints
  • Have poor balance/co-ordination
  • Have thin, stretchy skin
  • Have digestive problems like diarrhoea or constipation

If you suspect that you have one of the rarer forms of EDS, you should see your GP.

What's the life expectancy for Ehlers-Danlos Syndrome?

Life expectancy depends on the type of EDS.

vEDS can be a life shortening illness, because of the possible organ and vessel rupture.

The other forms don't tend to affect life expectancy, and you should be able to live a normal life.

Some people may be advised to avoid heavy lifting and contact sports, depending on the severity of their EDS, and will have to wear joint protection when exercising.

Low risk exercise like swimming and pilates is recommended.

For more info on EDS, please visit the NHS website.

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