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Ehlers-Danlos syndrome (EDS)

Ehlers-Danlos syndrome (EDS) refers to a group of disorders that are all connected but have different symptoms.

EDS is inherited from your parents, which means you're born with the condition. EDS affects the connective tissue in your body.

Connective tissue is throughout your body – in skin, muscles, tendons and ligaments, blood vessels, organs, gums and your eyes. It helps your body be strong and flexible.

If you have EDS, your connective tissue can stretch beyond its limits and become damaged.

There are 13 types of EDS. The most common effects are

• joints that move more than normal (joint hypermobility)
• very soft, stretchy skin
• easily damaged tissue.

EDS is rare, approximately 1 in 5,000 people have it. EDS affects women more than men and is in all races across the world.

Although you're born with the disorder, your symptoms can start at any age. Often, they're first noticed in teenagers.

EDS can take years to diagnose because it's rare. Many doctors aren't very familiar with it and the symptoms can be similar to many other conditions.

People with EDS often have other disorders, such as postural orthostatic tachycardia (POTS) and mast cell activation syndrome (MCAS).

Symptoms of Ehlers-Danlos syndrome

People with EDS may all notice different symptoms. Many of the common symptoms may not seem serious on their own but if the damaged connective tissue is in important structures, such as blood vessels, it can be very serious or life-threatening.

Common symptoms include:

• joint issues such a frequent sprains
• pain
• arthritis
• easy bruising
• wounds taking time to heal and healing with abnormal scars
• tiredness
• irritable bowel syndrome (IBS)
• hernias
• heart problems.

Diagnosing Ehlers-Danlos syndrome

Diagnosing EDS can take time. It's important to talk to your general practice team and explain your concerns. Tell them about any symptoms in other members of your family and your history of past injuries or illness.

Your general practice team will look at your skin and how mobile your joints are. There are checklists they can use and a score for joint hypermobility that will help with the diagnosis.

They may suggest tests such as skin biopsies, echocardiograms and other imaging tests to look at your joints and blood vessels.

There is a genetic test that you may have to pay for.

Treating Ehlers-Danlos syndrome

Although EDS cannot be cured, treatment can prevent further injuries or complications. You're likely to get the most benefit from a multi-disciplinary approach, which means you'll need to see several different health professionals. Each will help in their own way.

• Counsellor – helps you learn to live with a long-term condition and deal with symptoms such as pain.
• Dietitian – helps support you to eat well and manage your weight or any IBS symptoms.
• General practice team – prescribes medication such as pain relief for injuries.
• Genetic counsellor – helps you understand how EDS came from your parents and how you might pass it on to your children.
• Occupational therapist – helps you manage your daily activities.
• Physiotherapist – helps you strengthen your muscles and joints. (The physiotherapist needs to know you have EDS as some exercises can cause more damage.)
• Rheumatologist (a doctor who specialises in joint and muscle problems) – may be able to help diagnose EDS and may offer some advice on treatment.

Also,

• Braces – can protect your joints.
• Surgery – may help make your joints more stable.

Self-care for Ehlers-Danlos syndrome

There are several ways to manage EDS symptoms and improve your quality of life. These include:

• doing low-impact exercise to increase joint stability and reduce pain
• avoiding activities that may cause injury or stress to joints (such as contact sports)
• keeping a healthy lifestyle and weight to reduce stress on your joints
• wearing protective clothing and sunscreen to prevent skin injury.

It may be helpful to meet other people who have EDS. Consider contacting support groups and online communities.

  HealthInfo recommends the following pages

• Ehlers-Danlos Syndromes New Zealand

  Information to improve and support people with EDS and hypermobility spectrum disorder.

• Patient.info – Ehlers-Danlos Syndrome

  Details about EDS, how it affects people and how to treat it.

• Rare Disorders NZ

  Information and a collective voice for people with rare health disorders.

Written by HealthInfo clinical advisers. Page created September 2023.

Sources

Page reference: 1150952

Review key: HIEDS-1150952