How to manage gut motility issues in patients with Ehlers-Danlos syndrome (EDS)?

Management of Gut Motility Issues in Ehlers-Danlos Syndrome

Treatment for gut motility issues in Ehlers-Danlos syndrome (EDS) should be directed at the main symptoms, using as few drugs as possible, avoiding high doses of opioids and anticholinergics, and avoiding unnecessary surgery. 1

Understanding EDS and Gut Motility

Hypermobile Ehlers-Danlos syndrome (hEDS) with gastrointestinal manifestations is complex, as most patients with EDS don't have true dysmotility of the small bowel, but rather visceral hypersensitivity. Symptoms may mimic dysmotility (neuropathy), and EDS can contribute to dysmotility, particularly when postural orthostatic tachycardia syndrome (POTS) or other factors like opiate use are present 1.

Gastrointestinal symptoms are remarkably common in EDS patients:

• 62.3% of hEDS patients have at least one GI symptom at diagnosis 2
• Among patients tested for dysmotility, 76.2% have some form of dysmotility 2
• POTS is an independent predictive factor for GI dysmotility in these patients 2

Diagnostic Approach

1. Exclude mechanical obstruction - CT abdomen with oral contrast; if radiology is inconclusive, trial a low fiber (residue) diet or liquid diet 1

2. Evaluate contributing factors:

   • Medication effects (especially opioids and anticholinergics)
   • Psychosocial factors
   • Nutritional status (weight loss can affect gut function)
   • Presence of POTS (significantly increases risk of dysmotility) 2

3. Targeted investigations (when not on drugs affecting motility):

   • Screen for hypothyroidism, celiac disease, and diabetes
   • Consider manometry, radioisotope transit studies
   • Autoimmune screens if indicated 1

Management Algorithm

1. Symptom-Based Treatment

For abdominal pain:

• Avoid opioids which worsen dysmotility
• If patient has taken long-term opioids, consider gradual supervised withdrawal with pain specialist involvement 1
• Address visceral hypersensitivity which is common in EDS

For nausea/vomiting:

• Consider a venting gastrostomy for persistent vomiting 1
• Avoid cyclizine which can worsen dysmotility 1

For constipation/diarrhea:

• Initiate bowel regimens to avoid and treat constipation
• Consider prokinetic GI medications 1

2. Nutritional Management

Step 1: Try oral supplements/dietary adjustments if malnourished or at risk 1

Step 2: If oral feeding unsuccessful and patient not vomiting, try gastric feeding 1

Step 3: If gastric feeding unsuccessful, try jejunal feeding via nasojejunal tube initially, then consider more permanent options if successful 1

Step 4: If jejunal feeding fails (often due to abdominal distension or pain during infusion) and patient is malnourished, consider parenteral support 1

3. Address Comorbidities

For POTS (present in many EDS patients with dysmotility):

• Management of orthostatic symptoms may improve GI function
• Consider autonomic testing 1, 2

For mast cell activation disorder (common in EDS):

• Evaluate and treat if present, as it can exacerbate GI symptoms 1

Special Considerations and Pitfalls

1. Avoid unnecessary surgery - Bariatric or other GI surgeries in EDS patients can lead to complications including diverticula formation, adhesions, and worsening dysmotility 3

2. Beware of superior mesenteric artery syndrome - This is reported in patients who have suddenly lost weight but remains contentious; risks of surgery should be carefully considered 1

3. Medication cautions:

   • Opioids worsen dysmotility and can lead to narcotic bowel syndrome 1
   • Use supplemental oxygen therapy cautiously and monitor SpO2 continuously 1
   • Polypharmacy is a significant concern in EDS patients 4

4. Multidisciplinary approach - These patients need coordinated care including gastroenterology, pain management, rheumatology, nutrition, and psychology 1

Treatment Goals

The aims of treatment for EDS patients with gut dysmotility should be to:

• Reduce symptoms (pain, vomiting, distension, constipation/diarrhea)
• Reduce morbidity and mortality
• Achieve a normal BMI
• Improve quality of life 1

By following this structured approach and being aware of the unique challenges in EDS patients, clinicians can better manage gut motility issues and improve outcomes in this complex patient population.