Management of Gastrointestinal Symptoms in Hypermobile Ehlers-Danlos Syndrome

Initial Diagnostic Approach

The American Gastroenterological Association recommends screening for hypothyroidism, celiac disease, and diabetes in patients with hypermobile Ehlers-Danlos Syndrome (hEDS) 3, 4
Assess postural vital signs to evaluate for Postural Orthostatic Tachycardia Syndrome (POTS) (heart rate increase ≥30 bpm within 10 minutes of standing) 2, 5
Consider earlier gastric emptying testing (4-hour scintigraphy) given coexisting autonomic dysfunction risk in hEDS predisposes to gastroparesis 1, 2

Implement small, frequent meals to reduce postprandial symptoms, as recommended by the American College of Gastroenterology 5
Trial a gastroparesis diet (low fat, low fiber, smaller portions) for patients with gastroparesis-like symptoms 4
The American College of Gastroenterology recommends considering supervised low FODMAP diet as second-line dietary therapy if initial measures fail 6, 5
Ensure dietary interventions include nutritional counseling to prevent restrictive eating patterns, as advised by the Clinical Gastroenterology and Hepatology society 2, 4

Start tricyclic antidepressant (amitriptyline 10 mg at bedtime) as gut-brain neuromodulator, titrating slowly to 30-50 mg for abdominal pain, as recommended by the Gut journal 6, 4
The Clinical Gastroenterology and Hepatology society advises avoiding opioids for chronic abdominal pain as they worsen GI dysmotility 1, 2, 4, 5

Increase fluid intake to 2-3 liters daily and salt intake to 10-12 grams daily, as recommended by the Praxis Medical Insights society 5
Recommend compression garments for lower body to reduce venous pooling, as advised by the Praxis Medical Insights society 5

Never prescribe NSAIDs as they worsen GI symptoms in hEDS, as warned by the Praxis Medical Insights society 4
Do not use insoluble fiber (wheat bran) as it exacerbates bloating; use soluble fiber (ispaghula) starting at 3-4 g/day if needed, as recommended by the Gut journal 6
Avoid opioid analgesics which worsen gastroparesis and constipation, as advised by the Clinical Gastroenterology and Hepatology society 1, 2, 4, 5