What is the treatment plan for a 17-year-old female with recurring epistaxis due to Hereditary Hemorrhagic Telangiectasia (HHT)?

Treatment Protocol for HHT-Related Epistaxis in a 17-Year-Old Female

Start with nasal moisturization and topical therapies, then escalate to oral tranexamic acid (500 mg twice daily, titrating up to 1000 mg four times daily), and reserve systemic bevacizumab for refractory cases that fail conservative measures. 1

Step 1: First-Line Conservative Management

• Initiate nasal moisturization immediately through air humidification and topical application of saline solution or gels to prevent cracking and bleeding of fragile telangiectasias 1
• This approach alone is often inadequate for many HHT patients but remains the foundation of all treatment 1
• Continue these measures throughout all subsequent treatment escalations 1

Step 2: Oral Tranexamic Acid (First Systemic Therapy)

If moisturizing therapies fail to control epistaxis, add oral tranexamic acid as the next step 1

Dosing Protocol:

• Start at 500 mg twice daily 1, 2
• Gradually increase to 1000 mg four times daily or 1500 mg three times daily as needed 1
• This recommendation has high quality of evidence and strong strength of recommendation from the Second International HHT Guidelines 1

Evidence Supporting This Approach:

• Reduces epistaxis duration by 17.3% per month in controlled trials 1
• Achieves 54% reduction in composite epistaxis endpoints (duration and intensity) 1
• Reduces rebleeding risk from 69% to 49% over 10 days 3

Critical Contraindications to Screen For:

• Absolute contraindication: Recent thrombosis 1, 2
• Relative contraindications: Atrial fibrillation or known thrombophilia 1, 2
• In this 17-year-old patient, these contraindications are unlikely but must be excluded before initiating therapy

Step 3: Systemic Bevacizumab (For Refractory Cases)

Reserve systemic antiangiogenic therapy for epistaxis that fails moisturizing therapies, oral tranexamic acid, and/or local ablative procedures 1

When to Escalate:

• Patient continues to have significant epistaxis despite adequate trial of tranexamic acid 1
• Development of anemia requiring IV iron or blood transfusions 1
• Epistaxis Severity Score (ESS) remains elevated despite conservative measures 1

Bevacizumab Dosing Protocol:

• Induction: 5 mg/kg IV every 2 weeks for 4-6 doses 1
• Maintenance: 5 mg/kg IV every 1-3 months (variable dosing) 1

Expected Outcomes:

• Mean ESS improves from 6.81 to 3.44 (50% relative reduction) 1
• 85% of patients achieve epistaxis control 1
• Substantial improvements in hemoglobin levels (3-4 g/dL increase) 1
• Reduces need for iron infusions and red cell transfusions 1

Monitoring Requirements:

• Hypertension - monitor blood pressure regularly 1
• Proteinuria - check urine protein 1
• Infection risk - assess for signs of infection 1
• Delayed wound healing - caution with any surgical procedures 1
• Venous thromboembolism (VTE) - 2% rate in large studies 1

Important Caveat:

• Risk of long-term maintenance therapy remains unknown 1
• Shared decision-making with the patient and family is crucial given her young age 1

Step 4: Surgical Considerations (Use Cautiously)

Highly invasive surgical procedures should be reserved only for cases where medical therapies fail, as benefits are often temporary 4

Evidence Against Aggressive Surgery:

• Invasive procedures initially improve epistaxis frequency and intensity, but benefits diminish within 1-9 months postoperatively 4
• Hemorrhagic symptoms often recur at similar or worsened levels 4
• Procedures cause anatomical-functional changes that complicate future management 4
• Life-threatening epistaxis can occur even after Young's procedure (nasal closure), requiring reversal 5

Limited Role for Procedural Interventions:

• Local ablative therapies may be attempted before systemic bevacizumab 1
• Office-based sclerotherapy with sodium tetradecyl sulfate shows promise but requires multiple treatments 6
• Endovascular embolization reserved for life-threatening bleeding (93.5% long-term success for idiopathic epistaxis, but only 60% temporary relief in HHT) 7

Additional Management Considerations

Anemia Monitoring:

• Check hemoglobin regularly to assess severity of bleeding 1
• For a 17-year-old female, hemoglobin goals should reflect normal values for age and gender 1
• Provide oral iron supplementation if mild anemia develops 1
• Escalate to IV iron if oral iron insufficient to maintain hemoglobin goals 1

Family Screening:

• Given the autosomal dominant inheritance pattern and family history, ensure the relative with HHT receives appropriate screening and management 1
• Screen for other HHT manifestations including GI telangiectasias and arteriovenous malformations in lungs, liver, and brain 1

Common Pitfalls to Avoid

• Do not rely solely on invasive surgical procedures as first-line therapy given their temporary effectiveness and potential complications 4, 5
• Do not skip the stepwise escalation - always try moisturization and tranexamic acid before bevacizumab 1
• Do not use topical or intranasal bevacizumab - these formulations have not shown significant benefit, unlike systemic administration 1
• Do not overlook thrombotic risk factors before starting tranexamic acid in any patient 1, 2
• Do not forget shared decision-making when considering bevacizumab, especially given this patient's young age and unknown long-term risks 1