**Background:** Feeding difficulties due to functional gastrointestinal symptoms are well described in patients with hypermobile-type Ehlers-Danlos Syndrome (hEDS), particularly when comorbid with dysautonomia such as postural orthostatic tachycardia syndrome (POTS). There are currently no validated guidelines for evaluation and management of GI symptoms in hEDS. Patients may rapidly regress from eating a regular diet to requiring nutritional supplements, feeding tubes, or parenteral nutrition. The authors describe a case where multidisciplinary rehabilitative interventions successfully avoided surgical feeding tube placement.
**Methods:** This is a single case report from an urban pediatric academic medical center. The patient was a 14-year-old with hEDS (per 2017 criteria), POTS, mast cell dysregulation, chronic pain syndrome, chronic headaches, and prior concussion. Initial GI workup included an upper GI series with barium pill showing mild delay in distal esophageal emptying but normal timed barium swallow, visually normal upper intestinal endoscopy with histologic evidence of chronic gastritis, and normal MRI brain and cervical spine. Esophageal manometry was normal (Chicago Classification). The patient was diagnosed with rumination and started on diaphragmatic breathing. Prior to admission, multiple medication trials included amitriptyline, meloxicam, fludrocortisone, cromolyn, ondansetron, granisetron, omeprazole, prochlorperazine, cyproheptadine, erythromycin (stopped due to hypertension and lethargy with QTc of 466), and prucalopride. The patient had a nasogastric tube and later transpyloric feeds. A gastric emptying study was aborted due to persistent vomiting. Pyloric dilation with Botox injection was performed.
The 10-day hospitalization involved: removal of nasojejunal tube on day 1; continuous IV fluids adjusted as oral intake improved; daily occupational therapy (feeding-focused), pelvic floor physical therapy with biofeedback, and general PT for POTS; one-to-one sitter for symptom documentation and coping reinforcement; psychology support for emotional awareness and coping mechanisms; and staged advancement of oral intake by volume and texture (starting with half-carton supplement, advancing to purees, then soft foods). Medications during admission included prucalopride 2 mg daily, lansoprazole 30 mg daily, cromolyn QID, fludrocortisone, meloxicam, melatonin, hydroxyzine, milk of magnesia, and vitamin supplements. Diaphragmatic breathing was performed 15 minutes before and after oral intake.
**Key Results:** At admission, the patient had lost greater than 10% of usual body weight, had inadequate nutrient intake of 25–50% of needs, and BMI deceleration from z-score –1 to –1.9. The patient was highly motivated throughout hospitalization. Discharge criteria included oral tolerance of recommended daily caloric intake and small amounts of solid foods. At 14 months post-discharge, the patient continues to take all nutrition and hydration orally without formula. BMI improved from 19 to 22 with a 19-pound weight gain since discharge. Daily medications include prucalopride, famotidine, loratadine, meloxicam, tizanidine, salt supplementation, cromolyn, and hydroxyzine.
**Clinical Implications:** The authors recommend a symptom-based approach to GI symptoms in hEDS given the absence of standardized guidelines. They emphasize that functional GI disorders are the most commonly associated cause of symptoms, and that encouraging gut motility and altering sensory processing can be effective. They specifically recommend avoiding surgical feeding tubes or central lines if possible. Key elements of success included patient motivation, multidisciplinary intervention (feeding therapy, pelvic floor PT, psychology), and medication optimization. Systemic desensitization was used by slowly reintroducing oral nutrition while using biofeedback relaxation techniques. The authors note that prucalopride (initiated 3 weeks before admission) may have optimized treatment success, citing a study showing improved gastroparesis cardinal symptom index and quality-of-life after 4 weeks of prucalopride 2 mg daily. They also note that opiates, antihistamines, anticholinergics, and antiserotonergics (ondansetron, granisetron) may interfere with prokinetic therapy and were weaned at prucalopride initiation.