TABLE 5.
Dose of clotting factors received to manage haemostasis in WGH
| FVIII deficiency | FIX deficiency | |||
|---|---|---|---|---|
| EHL a | SHL b | EHL a | SHL c | |
| Most recent delivery | ||||
| In preparation for/during | ||||
| n | 0 | 3 | 0 | 1 |
| Median (range) loading dose, IU/kg | 50.0 (50.0–50.0) | 40.0 (NA) | ||
| n | 0 | 3 | 0 | 0 |
| Median (range) total dose, IU | 3000.0 (2900.0–4000.0) | |||
| Within 6 weeks postpartum | ||||
| n | 0 | 2 | 1 | 0 |
| Median (range) dose, IU/kg | 37.5 (25.0–50.0) | 50.0 (NA) | ||
| Most recent surgery/major dental procedure | ||||
| In preparation for/during | ||||
| n | 2 | 9 | 0 | 2 |
| Median (range) loading dose, IU/kg | 50.0 (50.0–50.0) | 47.8 (20.0–51.4) | 72.0 (50.0–94.0) | |
| n | 1 | 9 | 0 | 2 |
| Median (range) total dose, IU | 830.0 (NA) | 2500.0 (1500.0–4000.0) | 4200.0 (1000.0–7400.0) | |
| Within 2 weeks after | ||||
| n | 1 | 2 | 0 | 0 |
| Median (range) dose, IU/kg/wk | 50.0 (NA) | 41.0 (32.0–50.0) | ||
| Spontaneous, traumatic or joint bleeds, and/or bleeds from minor dental procedure | ||||
| For first bleed | ||||
| n | 9 | 3 | ||
| Median (range) loading dose, IU/kg | 26.0 (11.0–50.0) | 50.0 (40.0–60.0) | ||
| n | 7 | 3 | ||
| Median (range) total dose, IU | 2000.0 (1750.0–14,400.0) | 6000.0 (60.0–6000.0) | ||
| For most recent bleed | ||||
| N | 6 | 1 | ||
| Median (range) loading dose, IU/kg | 25.4 (13.0–75.0) | 50.0 (NA) | ||
| N | 4 | 1 | ||
| Median (range) total dose, IU | 3700.0 (2000.0–5898.0) | 3270.0 (NA) | ||
Abbreviations: EHL, extended half‐life; FIX, factor IX; FVIII, factor VIII; NA, not applicable; rFIX, recombinant factor IX; rFIXFc, recombinant factor IX Fc fusion protein; rFVIII, recombinant factor VIII; rFVIIIFc, recombinant factor VIII Fc fusion protein; SHL, standard half‐life; WGH, women and girls with haemophilia.
EHLs included either rFVIIIFc (ELOCTATE®, Sanofi Genzyme) or rFIXFc (Alprolix®, Sanofi Genzyme) for those with FVIII or FIX deficiency, respectively.
SHLs included rFVIII (Kogenate®, Bayer HealthCare LLC; Advate®, Baxalta US Inc.; Helixate® FS, Bayer HealthCare LLC; Nuwiq®, Octapharma AB; Xyntha®, Wyeth Pharmaceuticals LLC, a subsidiary of Pfizer Inc) or antihemophilic factor/von Willebrand factor complex (Humate‐P®, CSL Behring GmbH).
SHL was rFIX (BeneFIX®, Wyeth Pharmaceuticals LLC, a subsidiary of Pfizer Inc).