What is the management approach for Ehler's Danlos Syndrome (EDS)?

From the Guidelines

The management of Ehlers-Danlos Syndrome (EDS) requires a multidisciplinary approach focused on symptom management, injury prevention, and improving quality of life, as there is no cure for this genetic connective tissue disorder. Treatment typically includes physical therapy with gentle strengthening exercises to stabilize joints and improve muscle tone without overextending hypermobile joints. Pain management is essential and may involve both non-pharmacological approaches (heat/cold therapy, TENS units) and medications such as acetaminophen, NSAIDs, and occasionally low-dose opioids for severe pain 1.

Key Management Strategies

• Joint protection strategies are crucial, including avoiding hyperextension, using braces or splints when needed, and modifying activities to prevent dislocations.
• Cardiovascular monitoring is important, particularly in vascular EDS, with regular echocardiograms and blood pressure management, as well as consideration of medical therapy such as celiprolol, a beta blocker with vasodilatory properties, although its use is based on limited data 1.
• Gastrointestinal symptoms may require dietary modifications, proton pump inhibitors for reflux, or medications for gastroparesis or irritable bowel symptoms.
• Psychological support through counseling or cognitive behavioral therapy helps patients cope with chronic pain and limitations.
• Patients should avoid high-impact activities, heavy lifting, and extreme joint movements that could cause injury.

Special Considerations

• For women with vascular Ehlers-Danlos syndrome considering pregnancy, shared decision-making is essential 1, given the increased risk of vascular events and wound complications during labor and delivery.
• Regular follow-up with specialists including geneticists, rheumatologists, cardiologists, and physical therapists ensures comprehensive care tailored to each patient's specific EDS subtype and symptom profile.
• Surgical repair in vascular Ehlers-Danlos syndrome carries an increased risk due to vascular fragility and associated bleeding complications, and the decision to intervene should involve a Multidisciplinary Aortic Team and shared decision-making 1.

From the Research

Management Approach for Ehler's Danlos Syndrome (EDS)

The management approach for Ehler's Danlos Syndrome (EDS) involves a multidisciplinary treatment plan, as there is no single cure for the condition 2. The goal of treatment is to manage symptoms, prevent complications, and improve quality of life.

Treatment Modalities

Some of the treatment modalities used to manage EDS include:

• Physical therapy: to improve joint stability, strength, and flexibility 3, 4, 5, 6
• Occupational therapy: to improve daily functioning and adapt to limitations 3, 4
• Pain management: using a variety of techniques, including medication, alternative therapies, and lifestyle modifications 3
• Surgical interventions: to repair or stabilize joints, or to address other complications 3, 2
• Cognitive-behavioral therapy: to address psychological aspects of the condition, such as anxiety and depression 4

Multidisciplinary Care

Multidisciplinary care is essential for managing EDS, as it involves a team of healthcare professionals working together to address the various aspects of the condition 4, 5, 2. This team may include:

• Physiatrists
• Physical therapists
• Occupational therapists
• Pain management specialists
• Psychologists
• Surgeons

Lifestyle Modifications

Lifestyle modifications can also play an important role in managing EDS, such as:

• Avoiding heavy lifting or bending
• Using assistive devices to reduce strain on joints
• Engaging in regular exercise, such as yoga or swimming, to improve flexibility and strength
• Getting enough rest and managing stress
• Eating a healthy, balanced diet to support overall health and well-being 3, 4, 5, 6