From the Guidelines
Treatment for Ehlers-Danlos Syndrome (EDS) should focus on a multidisciplinary approach to manage symptoms, prevent complications, and improve quality of life, as there is no cure for this genetic connective tissue disorder. The primary approach includes physical therapy to strengthen muscles around hypermobile joints, which helps stabilize them and reduce dislocations 1. Pain management is essential and may involve over-the-counter medications like acetaminophen or NSAIDs for mild pain, while more severe pain might require prescription medications such as tramadol or low-dose naltrexone.
Key Considerations
- For skin fragility, patients should avoid trauma, use mild soaps, moisturize regularly, and consider vitamin C supplements (500-1000mg daily) to support collagen formation.
- Cardiovascular monitoring is crucial for certain EDS types, particularly vascular EDS, requiring regular echocardiograms and blood pressure management 1.
- Gastrointestinal symptoms may be addressed with dietary modifications, adequate hydration, and medications for specific issues like acid reflux or constipation 1.
- Patients should wear medical alert bracelets, avoid high-impact activities, and work with a multidisciplinary healthcare team including geneticists, rheumatologists, cardiologists, and physical therapists.
Specific Recommendations
- Patients with hypermobile EDS (hEDS) and Hypermobile Spectrum Disorders (HSD) should be managed with a focus on gastrointestinal and autonomic symptoms, as these conditions are often associated with a range of gut disorders and autonomic dysregulation 1.
- Women with EDS, particularly those with vascular EDS, should be closely monitored during pregnancy and delivery due to the increased risk of rupture of the gravid uterus and vessel rupture 1.
- Noninvasive vascular imaging is preferred in patients with vascular EDS to avoid fatal complications associated with invasive imaging 1.
From the Research
Treatment Options for Ehlers-Danlos Syndrome (EDS)
The treatment options for EDS can be categorized into several types, including:
- Complimentary/alternative treatments, such as massage therapies and heat therapy 2, 3, 4
- Pharmacological treatments, including opioids, nonsteroidal anti-inflammatory drugs, and neuropathic modulators 2, 3, 4
- Physical and occupational therapy, which can help improve joint stability and reduce pain 2, 3, 5, 6
- Surgical interventions, which may be necessary to repair joint dislocations or other complications 3, 5
- Bracing and splints, which can help stabilize joints and reduce pain 2, 3
Efficacy of Treatment Options
The efficacy of these treatment options can vary depending on the individual patient and the specific type of EDS. Some studies have reported that:
- Occupational therapy and bracing can be effective in improving symptoms and reducing pain 2
- Opioids and surgical interventions can be effective in managing acute and chronic pain 3
- Complimentary therapies, such as marijuana and traditional Chinese therapies, may also be effective in alleviating pain and improving quality of life 4
- Physical therapy can be effective in improving joint stability and reducing pain, but may also carry a risk of iatrogenic injuries 6
Challenges in Treatment
Despite the availability of these treatment options, there are several challenges in managing EDS, including:
- Limited understanding of the mechanisms of pain and other symptoms in EDS 2
- Difficulty in accessing appropriate treatment and diagnosis 3, 5
- High risk of iatrogenic injuries, particularly with physical therapy 6
- Need for a comprehensive and multidisciplinary approach to treatment, incorporating multiple therapies and interventions 2, 3, 4, 5, 6