Tranexamic Acid PAI-1 Deficiency Pfizer HALF LIFE:2-11 hours Dosing Calculation Topic Dose (oral) I.V. 10 mg/kg Q 6-8 hours Oral 25 mg/kg/dose (max 1.5 g/dose) Notes Do not use in the presence of hematuria or thrombosis. Generally not recommended for children <12 yrs. IHI does use in younger children based upon weight; discuss with treating […]
Tranexamic Acid PAI-1 Deficiency HALF LIFE: 2-11 hours Dosing Calculation Topic Dose (oral) Adult 1300 mg Q8 hours x 5 days Pediatric >12 yrs 15-20 mg/kg Q8 hours x 5 days Notes Do not use in the presence of hematuria or thrombosis. Generally not recommended for children <12 yrs. IHI does use in younger children […]
Aminocaproic Acid PAI-1 Deficiency HALF LIFE: 1-2 hours Various Manufacturers Dosing Calculation *Use adult dosing for children > 30 kg. Topic Dose (oral) Pediatric < 30 kg 100 mg/kg PO Q 6 hours Adult* > 30 kg 3 grams (6 tabs) PO QID while awake Topic Acute Bleeding Dose (I.V.) Loading 4-5 g during 1st […]
FXIII A Subunit Deficiency Only Novo Nordisk Standard t1/2HALF LIFE: 7.1 days Recombinant 3rd Gen Dosing Calculation 35 IU/kg once monthly Trough goal for FXIII Activity: ≥ 10% Notes Treat FXIII Deficiency patients with prophylaxis Rare No dose adjustment for pediatric patients Transfer Device Storage Refrigerate at 2 – 8°C (36– 46°F). Vial sizes: 2500 […]
FXIII A or B Subunit Deficiency CSL Behring Standard t1/2HALF LIFE: 6.6 ± 2.29 days Plasma-derived Dosing Calculation 40 IU/kg; Adjust dose ±5 IU per kg to maintain FXIII activity trough goal %Prophylaxis dosing: Q 28 days Peri-operative dose adjustment:8-21 days since last dose: consider additional dose based on trough level Over 21 days […]
FX Deficiency Bio Products Laboratory Ltd Standard t1/2HALF LIFE: 30.3 hours Plasma-derived Dosing Calculation # Units = Weight (kg) X % correction X VD (0.5) Dosing goals: Bleeding episode: 25% correction Pre-operative: 70-90% correction Post-operative: 50% correction Notes Suggested prophylaxis: 25 IU/kg once weekly. Discuss with treating physician. Transfer Device Storage Store in refrigerator or at room temperature 2 –30°C. […]
FVII Deficiency Novo Nordisk Standard t1/2 HALF LIFE: 4-6 hours Recombinant 2nd Gen Dosing Calculation 15-30 mcg/kg every 4-6 hours until hemostasis achieved Monitoring: PT, FVII Coagulant Activity Notes Final solution contains 1 mg/mL of rFVIIa. Transfer Device Storage Store between 2 – 25°C(36 – 77°F). Available in vials and prefilled syringes: 1, 2, 5, 8 […]
Protein C Deficiency Baxalta Standard t1/2HALF LIFE: 7.8-9.9 hours Plasma-derived Dosing Calculation *After resolution of acute bleeding episode Topic Dosing in IU/kg Initial Dose Subsequent Doses Maintenance Dose* Acute Episode/ Short-term Prophylaxis 100- 120 60-80 Q 6 hrs 40-60 Q 6-12 hrs Long-term Prophylaxis n/a n/a 45-60 Q 12 Rare hrs Notes Monitoring Goals: […]
Congenital Plasminogen Deficiency Type 1 (hypoplasminogenemia) Kedrion/Liminal Standard t1/2HALF LIFE:Adult: 32.4-38.5 hoursPediatric: 36.3-40.3 hours Plasminogen, human tvmh Dosing Calculation 6.6 mg/kg administered every 2-4 days Notes FDA 6/2021 Transfer Device Storage Store at 20-25°C (68-77°F) 50 mL vial: 68.8 mg of plasminogen +12.5 mL sterile water for injection Administration: Intravenous through syringe disc […]
Afibrinogenemia/Hypofibrinogenemia Octapharma Standard t1/2HALF LIFE (hours):Age >16 yrs: 82.3 ± 20Age <16 yrs: 69.9 ± 8.5 Plasma-derived Dosing Calculation # mg = [ Target level mg/dL – Measured level mg/dL / 1.8] X weight (kg)When Fibrinogen level unknown: 70 mg/kg Topic Goal Fibrinogen Minor Bleeding 100 mg/dL Major Bleeding 150 mg/dL Notes Target fibrinogen level of 100 mg/dL should […]
