What is the recommended management approach for a patient with hypermobile Ehlers-Danlos syndrome (hEDS) and bulimia?

Management of Hypermobile Ehlers-Danlos Syndrome (hEDS) and Bulimia

Patients with hEDS who develop bulimia require a specialized multidisciplinary approach that addresses both conditions simultaneously, as they share complex pathophysiological connections related to gastrointestinal dysfunction, disordered eating patterns, and psychological factors.

Understanding the Connection Between hEDS and Bulimia

The relationship between hEDS and bulimia is multifaceted:

1. Gastrointestinal dysfunction:

   • Patients with hEDS commonly experience gastrointestinal symptoms including visceral hypersensitivity, abdominal pain (affecting 69% vs 27% in non-hEDS individuals), postprandial fullness, constipation (73% vs 16%), and diarrhea (47% vs 9%) 1.
   • These symptoms can trigger or exacerbate disordered eating behaviors as patients attempt to manage digestive discomfort.

2. Avoidant/restrictive eating patterns:

   • Up to 62.1% of hEDS patients alter their diet due to symptoms 2.
   • Fear of eating and low interest in food (consistent with Avoidant Restrictive Food Intake Disorder) are strongly associated with dietary alterations in hEDS 2.

3. Psychological factors:

   • Anxiety disorders are common in hEDS and can contribute to both conditions.
   • Chronic pain and poor quality of life in hEDS may increase vulnerability to eating disorders.

Assessment Approach

Medical Assessment

• Evaluate for gastrointestinal symptoms typical in hEDS (reflux, dyspepsia, constipation, diarrhea)
• Screen for comorbid conditions common in hEDS:
  • Postural Orthostatic Tachycardia Syndrome (POTS)
  • Mast cell activation disorder
  • Autonomic dysfunction

Nutritional Assessment

• Assess nutritional status and patterns of restriction
• Screen for specific ARFID patterns using validated tools
• Evaluate for malnutrition which can further impair gut function 3

Psychological Assessment

• Screen for anxiety, depression, and somatization
• Assess for specific eating disorder behaviors using tools like DEPS-R or mSCOFF 3

Treatment Algorithm

Phase 1: Stabilization

1. Medical stabilization:

   • Address acute medical concerns related to bulimia (electrolyte abnormalities, dehydration)
   • Avoid rapid changes in glycemic control which can lead to treatment-induced complications 3

2. Nutritional intervention:

   • Establish regular eating patterns using a structured meal plan
   • Focus on symptom management rather than strict dietary rules
   • Consider enteral nutrition support if oral intake is severely compromised 3

Phase 2: Integrated Treatment

1. Gastrointestinal symptom management:

   • For inflammatory symptoms: Consider fecal calprotectin testing; if >150 mg/g, consider endoscopic assessment 4
   • For functional symptoms: Focus on managing visceral hypersensitivity which often mimics dysmotility 3

2. Psychological treatment:

   • Pharmacotherapy: Fluoxetine 60mg/day has established efficacy for bulimia nervosa and can maintain response for up to 52 weeks 5
   • Cognitive behavioral therapy adapted for both conditions
   • Motivational interviewing techniques to improve treatment adherence 3

3. Physical therapy:

   • Low-resistance exercise to improve joint stability by increasing muscle tone 4
   • Avoid high-impact activities that may exacerbate joint symptoms

Phase 3: Long-term Management

1. Regular monitoring:

   • Monthly outpatient appointments with dietitian trained in both eating disorders and hEDS 3
   • Regular assessment of nutritional status and eating behaviors
   • Monitoring of gastrointestinal symptoms

2. Nutritional approach:

   • Gradually transition from structured meal plans to more flexible eating
   • Address specific food fears related to gastrointestinal symptoms
   • Carefully consider the role of carbohydrate counting and food weighing, which may be triggering 3

Multidisciplinary Team Requirements

The optimal management requires coordination between:

• Gastroenterologist with knowledge of hEDS
• Dietitian with expertise in both eating disorders and hEDS
• Mental health professional (psychiatrist/psychologist)
• Physical therapist
• Pain specialist
• Geneticist (for family counseling)

Special Considerations

• Weight management: Weight gain associated with improved symptom control should be discussed proactively
• Medication effects: Monitor for side effects of medications that may impact gastrointestinal symptoms
• Realistic goals: Set small, achievable goals rather than aiming for perfect symptom control or eating behaviors
• Quality of life focus: Prioritize interventions that improve overall functioning and quality of life

Pitfalls to Avoid

• Treating the conditions in isolation rather than with an integrated approach
• Focusing solely on weight restoration without addressing underlying gastrointestinal symptoms
• Overlooking the psychological impact of chronic physical symptoms
• Implementing rapid dietary changes that may worsen gastrointestinal symptoms
• Failing to provide adequate psychological support alongside nutritional interventions

By addressing both conditions simultaneously with a coordinated multidisciplinary approach, outcomes for patients with hEDS and bulimia can be significantly improved.