Management of GI Symptoms in hEDS with POTS-like Features

Diagnostic Approach

The American Gastroenterological Association recommends prioritizing gastric motor function testing in patients with hEDS and POTS-like symptoms, given the high prevalence of abnormal gastric emptying in this population 1, 2
Gastric emptying scintigraphy should be obtained after excluding anatomical/structural disease, and celiac disease should be tested for earlier in the diagnostic evaluation 1, 2, 3
Anorectal manometry, balloon expulsion test, or defecography should be performed to evaluate constipation symptoms, given the high prevalence of pelvic floor dysfunction in hEDS 1, 2

The American College of Gastroenterology recommends metoclopramide 5-10mg three times daily before meals as first-line prokinetic for nausea and early satiety, with ondansetron 4-8mg as needed for breakthrough nausea 3, 5
Proton pump inhibitors, such as omeprazole 20-40mg daily, should be initiated for acid suppression in patients with heartburn 3, 5
Osmotic laxatives, such as polyethylene glycol 3350 17g daily, should be trialed as first-line treatment for constipation, with stimulant laxatives or prescription agents added if necessary 3, 5

The American Autonomic Society recommends increasing fluid intake to 2-3 liters daily and salt intake to 10-12 grams daily for volume expansion, as well as using lower body compression garments to reduce venous pooling 2, 5
Supervised exercise training programs, starting with recumbent exercise, should be implemented to improve orthostatic tolerance 2

The American Gastroenterological Association recommends implementing a gastroparesis diet, with small, frequent meals and low fat and fiber intake, to manage GI symptoms 1, 6, 5
A low FODMAP diet may be considered as second-line therapy for bloating and alternating bowel symptoms, and a low-histamine diet may be trialed if MCAS is confirmed 6, 5, 1, 2

The American Academy of Allergy, Asthma, and Immunology recommends starting H1 receptor antagonists, such as cetirizine 10mg daily, and adding H2 receptor antagonists, such as famotidine 20mg twice daily, for GI symptoms if MCAS is confirmed 5
Mast cell stabilizers, such as cromolyn sodium 200mg four times daily, may be considered for refractory cases 2, 5

The American Gastroenterological Association recommends avoiding NSAIDs, as they worsen GI symptoms in hEDS and are MCAS triggers, and never using opioids for chronic abdominal pain, as they cause narcotic bowel syndrome and worsen gastroparesis and constipation 6, 5, 3
Restrictive diets should not be implemented without nutritional guidance, as this population is vulnerable to disordered eating patterns 1, 2, 6