From the Guidelines
Ehlers-Danlos Syndrome Type 3, also known as Hypermobility EDS (hEDS), should be managed with a multidisciplinary approach focusing on symptom control, including physical therapy, pain management, and monitoring for complications, as there is no cure. This approach is crucial as hEDS is associated with a range of gut disorders, including acid reflux, abdominal pain, and constipation, as well as autonomic dysregulation, particularly postural orthostatic tachycardia syndrome (PoTS) 1.
Key Management Strategies
- Physical therapy to strengthen muscles around unstable joints
- Pain management with NSAIDs like ibuprofen (400-800mg every 6-8 hours) or acetaminophen (500-1000mg every 4-6 hours)
- Low-dose tricyclic antidepressants such as amitriptyline (10-25mg at bedtime) for chronic pain
- Avoiding activities that cause joint subluxation or dislocation
- Using braces or splints for unstable joints and practicing good posture
- Regular cardiovascular exercise within tolerance limits, with swimming and cycling being gentler options
Considerations for Gastrointestinal Issues
- Patients with hEDS often experience gastrointestinal symptoms that may mimic dysmotility, but true dysmotility is less common 1
- Opioid use can exacerbate gastrointestinal dysmotility and should be avoided when possible, with a focus on controlled opioid withdrawal and alternative pain management strategies 1
- Parenteral nutrition (PN) should be avoided in patients with hEDS unless there is severe, progressive, life-threatening malnutrition, as these patients are at higher risk for PN-related complications 1
Multidisciplinary Approach
- Involving rheumatologists, physical therapists, and pain specialists in the management of hEDS
- Monitoring for complications like autonomic dysfunction, gastrointestinal issues, and psychological impacts
- Early diagnosis and a holistic approach to management can provide the best outcomes for patients with hEDS 1
From the Research
Ehlers-Danlos Syndrome Type 3
Ehlers-Danlos Syndrome (EDS) is a multifaceted disease that can present with various types of pain, including musculoskeletal pain syndromes 2. The hypermobile type of EDS (hEDS) is one of the 13 subtypes recognized, characterized by hyperextensible skin, hypermobile joints, and tissue fragility 3.
Symptoms and Diagnosis
Hypermobile EDS is diagnosed by applying the 2017 diagnostic criteria, which includes joint hypermobility, skin findings, and joint pains or recurrent dislocations 4. The condition is inherited in an autosomal dominant pattern but does not have a known genetic mutation to help with diagnosis. Patients with hypermobile EDS may also experience extra-articular symptoms such as anxiety disorders, chronic pain, fatigue, orthostatic intolerance, functional gastrointestinal disorders, and pelvic and bladder dysfunction 4.
Treatment Options
The treatment of EDS is complex and requires a comprehensive approach. Some of the treatment options include:
- Physical therapy: Occupational therapy and bracing were found to be effective in improving symptoms in patients with EDS, with 70% of patients reporting improvement 2.
- Complementary therapies: Complementary therapies such as traditional Chinese therapies, herbal medications, and marijuana are widely used by patients with EDS, with some reporting significant pain relief 5.
- Non-opioid medications: Non-opioid medications, such as non-steroidal anti-inflammatory drugs and acetaminophen, are commonly used to manage pain in patients with EDS 5.
- Surgical management: Surgical management may be necessary for patients with shoulder and knee instability, but it is essential to consider the patient's connective tissue vulnerabilities and potential side effects 3.
- Opioid management: Opioids should be used cautiously in patients with EDS, and personalized plans for opioid tapering may be necessary for those on long-term opioid therapy 6.
Management and Support
The central goals of therapy for patients with hypermobile EDS are managing symptoms, preventing joint injury, and educating patients about their condition 4. Primary care physicians play a key role in initial recognition, diagnosis, and patient education, as well as overseeing and coordinating the multidisciplinary team required for these patients. Patients with hypermobile EDS may benefit from physical and occupational therapy, psychological support, and self-management strategies 4.