Management of Hypermobile Ehlers-Danlos Syndrome Flares

Immediate Flare Management

The American College of Gastroenterology recommends treating gastrointestinal symptoms based on the specific GI symptom pattern rather than using a one-size-fits-all approach, with consideration of gastroparesis diet and low-FODMAP diet as needed 2, 1
For patients with musculoskeletal pain, physical therapy with low-resistance exercise combined with myofascial release techniques is recommended to increase muscle tone and improve joint stability during the acute phase 1
Avoid opioids entirely for pain management, especially when gastrointestinal manifestations are present, as they worsen outcomes and increase complications 1

For nausea and early satiety suggesting gastroparesis, start metoclopramide 5-10mg three times daily before meals, and implement a gastroparesis diet with small, frequent meals and low fat and fiber 3
All dietary interventions must include nutritional counseling to prevent restrictive eating patterns and avoidant/restrictive food intake disorder (ARFID) 3

Increase fluid intake to 2-3 liters daily and salt intake to 10-12 grams daily for immediate volume expansion, and apply lower body compression garments during upright activities to reduce venous pooling 3

Start H1 receptor antagonist (cetirizine 10mg daily) combined with H2 receptor antagonist (famotidine 20mg twice daily) when MCAS is confirmed, and identify and avoid specific triggers including certain foods and environmental factors 3, 2

Implement brain-gut behavioral therapies due to increased rates of anxiety and psychological distress in patients with hypermobility, with consideration of psychological support as part of the multidisciplinary approach 1

Coordinate care among gastroenterology, cardiology or neurology, nutrition, pain management, and physical medicine specialists, with regular follow-up with the multidisciplinary team required for all hEDS patients 3, 1

Never prescribe opioids for chronic pain management in hEDS, particularly with GI manifestations, and avoid parenteral nutrition except in life-threatening malnutrition as a temporary bridge 1, 3
Ensure nutritional counseling accompanies all dietary interventions to prevent restrictive eating patterns, and do not perform MCAS testing for isolated GI symptoms without evidence of multisystem involvement 2, 3