Comparison of perioperative practices between CwH with and without inhibitors
| CWH WITHOUT INHIBITORS | CWH WITH INHIBITORS | P-VALUE | |
|---|---|---|---|
| Number of CwH | 14 | 7 | — |
| Preoperative investigations performed | |||
| • Full blood count (%) | 7 (50%) | 7 (100%) | 0.047 |
| • APTT (%) | 5 (36%) | 2 (29%) | 1.000 |
| • Factor assay (%) | 9 (64%) | 1 (14%) | 0.063 |
| • Recovery study (%) | 0 (0%) | 2 (29%) | 0.100 |
| • Inhibitor screen (%) | 9 (64%) | 7 (100%) | 0.123 |
| Antifibrinolytic use (%) | 6 (43%) | 4 (57%) | 0.659 |
| Mean length of stay in days (range) | 4.9 (3–7) | 8.6 (3–25) | 0.096 |
| Postoperative complications (%) | 0 (0%) | 4 (57%) | 0.006 |
| Need for CVAD revision (%) | 0 (0%) | 1 (14%) | 0.333 |
Characteristics and operative details of CwH included in the study, from all centres
| Number of CwH | 21 |
| Median age at insertion, months (range)* | 20.5 (12–110) |
| Haemophilia A (%) | 20 (95%) |
| Haemophilia B (%) | 1 (5%) |
| Deficient factor <1% (%) | 21 (100%) |
| Number of patients with inhibitors (%) | 7 (33%) |
| Median preoperative inhibitor titres (range) | 5 BU (1.5–210 BU) |
| Preoperative treatment** | |
| • Prophylaxis (%) | 9 (64%) |
| • On-demand (%) | 5 (36%) |
| Indication for CVAD insertion | |
| • Prophylaxis (%) | 13 (62%) |
| • ITI (%) | 4 (19%) |
| • Revision (%) | 2 (9.5%) |
| • Difficult venous access (on-demand treatment) (%) | 2 (9.5%) |
| Type of CVAD | |
| • Portacath (%) | 21 (100%) |
| Vessel used for CVAD | |
| • Internal jugular vein (%) | 9 (43%) |
| • Subclavian vein (%) | 9 (43%) |
| • External jugular vein (%) | 3 (14%) |
| Mean number of CVAD insertion attempts, including successful attempt (range) | 1.4 (1–4) |
Preoperative investigations performed prior to CVAD surgery and details of perioperative factor VIII cover for CVAD surgery amongst CwH without inhibitors
| SINGAPORE (% PERFORMED) | HAMILTON (% PERFORMED) | P-VALUE | |
|---|---|---|---|
| Investigations | |||
| Full blood count | 100% | 0% | 0.000 |
| APTT | 83% | 0% | 0.001 |
| Factor assay | 25% | 100% | 0.003 |
| Inhibitor screen | 25% | 100% | 0.003 |
| Factor VIII cover | |||
| Mean preoperative factor VIII dose (±SD) | 50.0 IU/kg (±7.6) | 72.4 IU/kg (±12.5) | 0.002 |
| Mean total dose used (±SD) | 8642.9 IU (±6011.9) | 8924.1 IU (±2488.6) | 0.911 |
| Mean duration of factor VIII cover (±SD) | 5.3 days (±0.9) | 6.9 days (±0.7) | 0.004 |
Details of perioperative rFVIIa cover for CVAD surgery in CwH with inhibitors
| RFVIIA COVER | SINGAPORE | HAMILTON | P-VALUE |
|---|---|---|---|
| Mean preoperative rFVIIa dose (±SD) | 160.5 mcg/kg (±99.9) | 88.2 mcg/kg (±3.8) | 0.244 |
| Mean total dose used from preoperative dose to discharge (±SD) | 3008.0 mcg/kg (±2305.9) | 2640.2 mcg/kg (±134.1) | 0.842 |
| Mean duration of rFVIIa cover (±SD) | 5.3 days (±1.7) | 9.5 days (±2.1) | 0.054 |