Management of Gastrointestinal and Hematological Symptoms in hEDS/POTS

Diagnostic Priorities

• Gastric emptying scintigraphy is the priority test for patients with early satiety, nausea, and abdominal pain, as abnormal gastric emptying is more common in hEDS/POTS than the general population 1
• Celiac disease serological testing should be performed earlier than usual in hEDS patients with any GI symptoms, as celiac disease risk is elevated in this population 2

POTS Management

• The American College of Cardiology recommends increasing fluid intake to 2-3 liters daily and salt intake to 10-12 grams daily for volume expansion in patients with POTS 4
• Lower body compression garments (30-40 mmHg) should be worn during upright activities to reduce venous pooling in patients with POTS 4

Symptom-Directed GI Management

• Metoclopramide 5-10mg three times daily before meals is recommended as first-line prokinetic for gastroparesis in patients with hEDS/POTS 4
• A gastroparesis diet consisting of small, frequent meals (5-6 per day), low fat (<40g/day), low fiber, with liquid calories prioritized is recommended for patients with gastroparesis 1, 4

MCAS Evaluation and Treatment

• MCAS should be suspected if a patient has multisystem symptoms including flushing, urticaria, wheezing, or anaphylaxis-like episodes, and MCAS testing should not be performed for isolated GI symptoms alone without evidence of generalized mast cell disorder 1, 2
• H1 receptor antagonist, such as cetirizine 10mg daily, and H2 receptor antagonist, such as famotidine 20mg twice daily, are recommended for MCAS treatment 4

Dietary Interventions

• A structured dietary approach starting with a gastroparesis diet is recommended, with consideration of a low-FODMAP diet as second-line for bloating and alternating bowel symptoms, and a low-histamine diet only if MCAS is confirmed 1, 4
• All dietary interventions must include nutritional counseling to avoid restrictive eating patterns and development of avoidant/restrictive food intake disorder (ARFID) 1, 5

Multidisciplinary Coordination

• Hematology, gastroenterology, cardiology or neurology, nutrition, and pain management specialists should be involved in the care of patients with hEDS/POTS to ensure comprehensive management of symptoms 1, 3, 5
• Parenteral nutrition should be avoided in hEDS/POTS except in life-threatening malnutrition as a temporary bridge to rehabilitative therapies, due to the increased risk of catheter-related bloodstream infections in this population 5