Management of Ehlers-Danlos Syndrome with Postural Orthostatic Tachycardia Syndrome
POTS Management
- The American College of Cardiology recommends increasing fluid and salt intake to expand blood volume and improve orthostatic tolerance in patients with POTS 1
- The use of compression garments, particularly lower body, can reduce venous pooling and improve orthostatic tolerance in patients with POTS 1
- Blood volume expanders such as fludrocortisone can enhance sodium retention and increase blood volume in patients with POTS 1
- Heart rate control with low-dose propranolol (beta-blocker) can reduce tachycardia in patients with POTS 1
- Vasoconstrictors such as midodrine can be used in patients with significant orthostatic hypotension whose lives are considerably impaired despite standard clinical care 1, 5
Gastrointestinal Symptom Management
- The American Gastroenterological Association recommends the use of antiemetics such as ondansetron and prokinetics such as metoclopramide for nausea and vomiting in patients with EDS and POTS 1, 6
- Acid suppressants such as proton pump inhibitors and antispasmodics such as hyoscyamine can be used to manage abdominal pain in patients with EDS and POTS 6
- The use of osmotic laxatives such as PEG 3350 and stimulant laxatives such as bisacodyl can help manage constipation in patients with EDS and POTS 1, 6
- Antidiarrheals such as loperamide and bile acid sequestrants such as cholestyramine can be used to manage diarrhea in patients with EDS and POTS 1, 6
Mast Cell Activation Syndrome (MCAS) Management
- The use of H1 receptor antagonists such as cetirizine and H2 receptor antagonists such as famotidine can help manage dermatologic and abdominal symptoms in patients with MCAS 4
- Mast cell stabilizers such as cromolyn sodium can be used in refractory cases of MCAS 4
- Avoiding known triggers such as certain foods, alcohol, and strong smells can help manage MCAS symptoms 1, 4
Dietary Interventions
- The use of small, frequent meals can help reduce postprandial symptoms in patients with EDS and POTS 1
- Specialized diets such as a low FODMAP diet and a low-histamine diet can be beneficial in managing GI symptoms in patients with EDS and POTS 1, 4
Diagnostic Considerations
- Testing for POTS in patients with EDS who report orthostatic intolerance is recommended, with a heart rate increase ≥30 bpm within 10 minutes of standing 2, 3
- Gastric emptying studies can be considered in patients with upper GI symptoms and POTS 5
- Evaluating for functional defecation disorders with anorectal manometry or defecography can be beneficial in patients with lower GI symptoms 5
Common Pitfalls and Caveats
- Overuse of opioids for pain management can worsen GI symptoms in patients with EDS and POTS 1, 6
- Excessive dietary restrictions without proper nutritional guidance can be harmful in patients with EDS and POTS 1
- Failure to recognize the multisystemic nature of these conditions can lead to inadequate management 1, 6