Management Plan for Hypermobile Ehlers-Danlos Syndrome (hEDS)
A multidisciplinary team approach focusing on symptom management, physical therapy, and prevention of complications is the cornerstone of treatment for patients with Hypermobile Ehlers-Danlos Syndrome, as there is currently no curative therapy available. 1
Diagnosis and Assessment
Diagnosis requires comprehensive clinical evaluation using the 2017 diagnostic criteria:
Genetic testing is critical to exclude other EDS subtypes and related conditions, as 26.4% of patients initially diagnosed with hEDS may have alternative genetic diagnoses requiring different management approaches 2
Physical Therapy and Rehabilitation
Physical therapy is the primary intervention for hEDS management, focusing on:
Avoid aggressive stretching and joint mobilization techniques that may exacerbate hypermobility 4
Focus on functional training that mimics daily activities to improve quality of life 5
Pain Management
First-line approach:
- Non-opioid analgesics (acetaminophen, NSAIDs) for acute pain
- Heat/cold therapy for symptomatic relief
- Bracing or taping for unstable joints
Second-line approach:
- Low-dose neuromodulators (e.g., mirtazapine) may help with pain and improve food tolerance in patients with gastrointestinal symptoms 6
- Avoid opioids due to risk of dependence and potential to worsen gastrointestinal symptoms
Cardiovascular Management
Baseline echocardiogram to evaluate for aortic root dilatation (occurs in 25-33% of hEDS patients) 1
Regular cardiovascular follow-up for patients with identified aortic anomalies 1
Consider beta blockers for patients with vascular manifestations, though evidence is stronger for vascular EDS than hEDS 1
Gastrointestinal Management
Screen for and manage comorbid functional gastrointestinal disorders
Optimize oral nutrition and avoid unnecessary parenteral nutrition
Parenteral nutrition should be avoided in hEDS patients except in cases of severe, progressive, life-threatening malnutrition 6
Psychological Support
Screen for anxiety disorders, which are common comorbidities
Cognitive behavioral therapy to address pain catastrophizing and fear-avoidance behaviors
Education about the chronic nature of the condition and self-management strategies
Prevention of Complications
Patient education on joint protection strategies and avoiding activities that increase risk of dislocation
Careful surgical planning when procedures are necessary, with attention to tissue fragility
Avoid activities with high risk of trauma or joint injury
Special Considerations
Pregnancy planning should include consultation with specialists familiar with hEDS
Occupational therapy assessment for workplace/home modifications to reduce joint stress
Regular reassessment of symptoms and treatment efficacy
Cautions and Pitfalls
Avoid aggressive physical therapy approaches that may cause iatrogenic injuries 4
Be aware that patients with hEDS may have higher rates of central line infections if parenteral nutrition is used 6
Do not dismiss extra-articular manifestations (autonomic dysfunction, fatigue, etc.) as unrelated to hEDS
Recognize that current evidence for rehabilitation interventions in hEDS is limited, with few high-quality studies available 5
The management of hEDS requires ongoing coordination between specialists, with the primary care physician often serving as the central coordinator of care 7. Regular monitoring and adjustment of the treatment plan based on symptom progression and response to interventions is essential for optimizing quality of life and reducing morbidity in these patients.