What are the treatment options for a hypermobile Ehlers-Danlos syndrome (hEDS) flare?

Treatment of Hypermobile Ehlers-Danlos Syndrome (hEDS) Flares

Physical therapy with low-resistance exercise combined with myofascial release techniques is the cornerstone of acute flare management, supplemented by targeted symptom-specific interventions based on the predominant manifestation (musculoskeletal pain, gastrointestinal symptoms, or autonomic dysfunction). 1

Immediate Flare Management

Musculoskeletal Pain (Most Common Flare Type)

• Avoid opioids entirely for pain management, especially when gastrointestinal manifestations are present, as they worsen outcomes and increase complications 1
• Implement physical therapy focused on low-resistance exercises to increase muscle tone and improve joint stability during the acute phase 1
• Add myofascial release techniques to address soft tissue overuse injury and reduce pain 1
• Consider trigger point injections for localized musculoskeletal pain as an interventional option 2
• Peripheral nerve blocks may be used for specific joint pain (shoulder, knee) when conservative measures fail 2

Gastrointestinal Flares

• Treat based on the specific GI symptom pattern rather than using a one-size-fits-all approach 3, 1
• For nausea and early satiety suggesting gastroparesis: start metoclopramide 5-10mg three times daily before meals 4
• Implement a gastroparesis diet with small, frequent meals (5-6 per day), low fat (<40g/day), low fiber, prioritizing liquid calories 4
• For bloating and alternating bowel symptoms: trial a low-FODMAP diet as second-line intervention 4
• All dietary interventions must include nutritional counseling to prevent restrictive eating patterns and avoidant/restrictive food intake disorder (ARFID) 4

Autonomic Dysfunction (POTS) Flares

• Increase fluid intake to 2-3 liters daily and salt intake to 10-12 grams daily for immediate volume expansion 4
• Apply lower body compression garments (30-40 mmHg) during upright activities to reduce venous pooling 4
• If conservative measures fail within days, consider pharmacological treatments for volume expansion, heart rate control, and vasoconstriction with cardiology or neurology consultation 3

Mast Cell Activation Syndrome (MCAS) Flares

• Start H1 receptor antagonist (cetirizine 10mg daily) combined with H2 receptor antagonist (famotidine 20mg twice daily) when MCAS is confirmed 4
• Identify and avoid specific triggers including certain foods, alcohol, strong smells, temperature changes, mechanical friction, emotional distress, pollen, mold, opioids, NSAIDs, and iodinated contrast 3
• Consider mast cell stabilizers as adjunctive therapy 3
• Implement a low-histamine diet only if MCAS is confirmed through appropriate testing, not for isolated GI symptoms 4

Psychological and Behavioral Support

• Implement brain-gut behavioral therapies due to increased rates of anxiety and psychological distress in patients with hypermobility 1
• Provide psychological support as part of the multidisciplinary approach, as anxiety disorders and chronic pain are common extra-articular manifestations 5

Multidisciplinary Coordination During Flares

• Coordinate care among gastroenterology, cardiology or neurology, nutrition, pain management, and physical medicine specialists 4
• Regular follow-up with the multidisciplinary team is required for all hEDS patients, not just during flares 1

Critical Pitfalls to Avoid

• Never prescribe opioids for chronic pain management in hEDS, particularly with GI manifestations 1
• Avoid parenteral nutrition except in life-threatening malnutrition as a temporary bridge, due to increased catheter-related bloodstream infection risk 4
• Do not perform MCAS testing for isolated GI symptoms without evidence of multisystem involvement (flushing, urticaria, wheezing, anaphylaxis-like episodes) 4
• Ensure nutritional counseling accompanies all dietary interventions to prevent restrictive eating patterns 3, 4