1. Event and Peri-procedural Replacement Therapy in Patients with Hemophilia

2. LIMITED DATA: OBSERVATIONAL BASED THERAPY

3. EVENT BLEEDS

4. Joint Bleeding Management Episodic Therapy – Single dose 20-25 u/kg (target 40-50%) May need higher dose 40 u/kg if delay treatment >3 hrs – RCT using Kogenate as prophylaxis vs. episodic Rx Prophylaxis 25 IU/kg every other day Episodic – 40 IU/kg at onset of bleed – 20 IU/kg at 24 and 72 hours – 20 IU/kg then encouraged every other day until pain and mobility impairment completely resolved (up to 4 weeks) – Ice – Joint aspiration? If septic joint considered

5. Epistaxis Most bleeds need local measures only If prolonged (>20 minutes) or major (gushing) – Single dose 20-25 u/kg (target 40-50%) – Amicar x 5-10 days Load with 5 grams, then 2.5 grams every 6 hours – NeoSynepherine

6. Muscle Bleeds Large muscle bleed – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 20-25 u/kg every 12 hours for total 2-4 days – Based on symptoms – Ice and limit activity Small muscle sans neurovascular compromise – Initial dose 20-25 u/kg (target 40-50%) – Maintenance doses 20-25 u/kg every 12 hours for total 1-2 days – Based on symptoms – Ice and limit activity

7. Neck Soft Tissue or Tonsillar Bleeds Large muscle bleed – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg every 12 hours for total 3-5 days after bleeding has stopped – Amicar x 5-10 days Load with 5 grams, then 2.5 grams every 6 hours – Maintain and protect airway

8. Intracranial Bleeds Intracranial bleeds – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 20-25 u/kg every 12 hours for 14 days (until resolved) – Prophylaxis therafter

9. PROCEDURAL PLANNING

10. Vaccinations Nothing needed if either: – Using 25 gauge needle – Subcutaneous injection If using larger needle used for IM injection – 20-25 u/kg x 1 dose – Ice

11. Dental Procedures Single extraction – Initial dose 25 u/kg (target 50%) – Amicar x 5 days Load with 5 grams, then 2.5 grams every 6 hours Multiple extractions – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 20-25 u/kg every 12 hours for total 3 days – Amicar x 7-10 days Load with 5 grams, then 2.5 grams every 6 hours

12. Dental Procedures Root canal – Nothing needed specifically – But, the anesthesia is nerve root block Initial dose 25 u/kg (target 50%) Amicar x 5 days – Load with 5 grams, then 2.5 grams every 6 hours

13. Port or Central Venous Access Device Placement Intravenous Device Placement – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg every 12 hours for total 3 days

14. Endoscopy/Colonoscopy No biopsy needed – Initial dose 40-50 u/kg (target 80-100%) – Amicar x 5 days Load with 5 grams, then 2.5 grams every 6 hours If biopsy performed – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg x one dose 8-12 hours after the first dose then 25 u/kg every 12 hours for 2-4 additional days (total 3-5 days of Rx) – Amicar x 5-7 days Load with 5 grams, then 2.5 grams every 6 hours Advise to clip rather than cauterize if biopsy needed during the procedure

15. General Surgery – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg x one dose 6-8 hours after the first dose then 25 u/kg every 12 hours for a total 7-10 days of Rx

16. Orthopedic — Minor Minor Orthopedic Surgery – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg x one dose 6-8 hours after the first dose then 25 u/kg every 12 hours for a total 5-7 days of Rx

17. Orthopedic – Major Major Orthopedic Surgery – Initial dose 40-50 u/kg (target 80-100%) – Maintenance doses 25 u/kg x one dose 6-8 hours after the first dose then 25 u/kg every 12 hours for a total 14 days of Rx 25 units every 24 hours prn prior to physical therapy for additional 2-4 weeks as directed by duration of PT – DVT prophylaxis?