Managing HHT and IBS: A Dual-Diagnosis Approach
Manage IBS and HHT as separate conditions with careful attention to avoiding therapies that could worsen bleeding risk, prioritizing IBS treatments that do not interfere with HHT bleeding management, and coordinating care through a multidisciplinary team. 1
Critical Safety Considerations
Avoid NSAIDs and aspirin for IBS-related pain, as these will exacerbate HHT bleeding complications and worsen anemia. 1, 2
Exercise caution with low FODMAP diet implementation in HHT patients who already have nutritional deficiencies from chronic blood loss, as overly restrictive diets may compound iron and nutrient deficiencies. 1
Monitor for drug interactions between IBS neuromodulators and any systemic therapies used for HHT bleeding (tranexamic acid, bevacizumab). 1
IBS Management in HHT Patients
First-Line IBS Therapy
Begin with patient-centered dietary counseling that accounts for the patient's iron deficiency anemia and nutritional status from HHT-related bleeding. 1
Consider a Mediterranean diet as first-line dietary intervention rather than low FODMAP, as it provides better nutritional support for patients with chronic blood loss and anemia. 1
Implement brain-gut behavioral therapies (cognitive behavioral therapy, gut-directed hypnotherapy, mindfulness-based stress reduction) as these do not interfere with HHT management and address the psychosocial burden of both conditions. 1
Pharmacologic Management for IBS
Use low-dose tricyclic antidepressants (TCAs) for IBS pain if gastrointestinal symptoms predominate, starting at low doses (10-25 mg at bedtime). 1
Switch to selective serotonin reuptake inhibitors (SSRIs) if concurrent mood disorder is present, as low-dose TCAs will not adequately address psychological symptoms. 1
Avoid tranexamic acid for IBS symptoms, as this medication is reserved specifically for HHT bleeding control and should not be used for IBS-related complaints. 1, 3
Dietary Modifications
If low FODMAP diet is considered, ensure it is delivered by a specialist dietitian who understands both conditions and can monitor for nutritional deficiencies. 1
Use a "gentle FODMAP" approach or standard diet if the patient has moderate-to-severe anxiety or depression related to their dual diagnosis. 1
Avoid restrictive eating patterns that could worsen iron deficiency or lead to unintentional weight loss (≥5% in 6 months), which would trigger referral to specialized dietitian. 1
HHT Management Priorities
Bleeding Control Hierarchy
Follow the established HHT treatment algorithm regardless of IBS status:
Nasal moisturization with saline and humidification for epistaxis. 1, 3
Oral tranexamic acid (500 mg twice daily, titrating to 1000 mg four times daily) if moisturization fails, with contraindications including recent thrombosis, atrial fibrillation, or known thrombophilia. 1, 3
Systemic bevacizumab (5 mg/kg IV every 2 weeks for 4-6 doses, then maintenance every 1-3 months) for refractory epistaxis or moderate-to-severe GI bleeding requiring IV iron or transfusions. 1, 3
Anemia Management
Maintain hemoglobin goals appropriate for age and gender through oral iron supplementation for mild anemia, escalating to IV iron for moderate anemia. 1, 3
Monitor hemoglobin regularly as IBS symptoms may mask worsening GI bleeding from HHT telangiectasias. 1, 2
Screen for GI telangiectasias with upper endoscopy if anemia is disproportionate to epistaxis severity, as GI bleeding develops in approximately 30% of HHT patients. 1, 4
Multidisciplinary Coordination
Establish care coordination between gastroenterology, hematology, and mental health specialists, as both conditions benefit from integrated management. 1
Refer to HHT specialist center for complex cases, particularly if systemic bevacizumab is being considered or if patient has SMAD4 mutation (juvenile polyposis-HHT overlap syndrome). 1, 4
Ensure gastropsychologist involvement if patient shows moderate-to-severe anxiety or depression, impaired quality of life, or avoidance behaviors related to either condition. 1
Common Pitfalls to Avoid
Do not attribute all GI symptoms to IBS without excluding worsening HHT-related GI bleeding, especially if new anemia develops or hemoglobin drops. 1, 2
Do not perform liver biopsy in any patient with proven or suspected HHT due to catastrophic hemorrhage risk, even if investigating other conditions. 1, 4
Do not use repetitive endoscopic ablation (Argon plasma coagulation) as primary therapy for HHT GI bleeding, as this approach is outdated and systemic therapies are now preferred. 1
Do not delay HHT screening for pulmonary, cerebral, and hepatic arteriovenous malformations, as these can cause life-threatening complications independent of bleeding symptoms. 1, 4
Quality of Life Prioritization
Treatment decisions should prioritize quality of life over laboratory values alone, as both HHT epistaxis and IBS cause significant psychosocial morbidity, social isolation, and employment difficulties. 1, 2
Validate that both gastrointestinal and bleeding symptoms are real and taken seriously, providing empathy and education about gut-brain axis dysregulation in IBS and vascular pathology in HHT. 1
Adjust visit frequency to accommodate the complexity of managing both conditions and provide ongoing monitoring of bleeding, anemia, and IBS symptom control. 1