Table 3. Clinical characteristics of survey respondents.
| Adults ≥ 18 y N = 106 |
Children/Teenagers 10–17 y N = 21 |
Children (proxy version) | ||
|---|---|---|---|---|
| 6–9 y N = 11 |
1–5 y N = 8 |
|||
| Type of hemophilia | ||||
| Hemophilia A | 89 (84.0%) | 19 (90.5%) | 10 (90.9%) | 8 (100%) |
| Hemophilia B | 17 (16.0%) | 2 (9.5%) | 1 (9.1%) | 0 |
| Hemophilia severity | ||||
| Mild | 13 (12.3%) | 4 (19.0%) | 0 | 1 (12.5%) |
| Moderate | 33 (31.1%) | 2 (9.5%) | 3 (27.3%) | 2 (25.0%) |
| Severe | 60 (56.6%) | 15 (71.4%) | 8 (72.7%) | 5 (62.5%) |
| Inhibitors | ||||
| Yes | 14 (13.2%) | 1 (4.8%) | 3 (27.3%) | 2 (25.0%) |
| Does not know | 27 (25.5%) | 1 (4.8%) | 1 (9.1%) | 2 (25.0%) |
| Age at the time of diagnosis | ||||
| During pregnancy/at birth | 12 (11.3%) | 3 (14.3%) | 4 (36.4%) | 5 (62.5%) |
| First year of life | 40 (37.7%) | 11 (52.4%) | 5 (45.5%) | 3 (37.5%) |
| 1–5 y | 21 (19.8%) | 7 (33.3%) | 2 (18.2%) | 0 |
| 6–10 y | 10 (9.4%) | 0 | 0 | – |
| ≥ 11 y | 13 (12.3%) | 0 | 0 | – |
| Does not know | 13 (12.3%) | 0 | 0 | 0 |
| Prophylaxis treatment | 35 (33.0%) | 16 (76.2%) | 9 (81.8%) | 4 (50.0%) |
| Type of factor concentrate | ||||
| Plasma-derived | 42 (39.6%) | 5 (23.8%) | 0 | 0 |
| Recombinant | 33 (31.1%) | 11 (52.4%) | 10 (90.9%) | 6 (75.0%) |
| Bypassing agent | 6 (5.7%) | 1 (4.8%) | 1 (9.1%) | 1 (12.5%) |
| Does not know | 14 (13.2%) | 2 (9.5%) | 0 | 0 |
| Urgent hospital visits due to hemophilia a : yes | 51 (48.1%) | 13 (61.9%) | 9 (81.8%) | 4 (50.0%) |
| Number of visits | 4.73 (4.44) Md = 3 range: 1–16 |
3.31 (2.14) Md = 2 range: 1–7 |
3.89 (3.26) Md = 2 range: 1–10 |
5.25 (3.76) Md = 5 range: 1–10 |
| Hospitalization due to hemophilia a : yes | 12 (11.3%) | 4 (19.0%) | 2 (18.2%) | 2 (25.0%) |
| Number of days | 12.58 (11.73) Md = 7.5 range: 4–39 |
5.67 (3.22) Md = 7 range: 2–8 |
1.5 (0.71) Md = 1.5 range: 1–2 | 5.0 (2.83) Md = 5 range: 3–7 |
| Bleeding episodes a : yes | 71 (67.0%) | 15 (71.4%) | 6 (54.5%) | 4 (50.0%) |
| Number of bleeding episodes | 14.94 (16.90) Md = 9 range: 1–84 |
4.53 (3.36) Md = 3 range: 1–12 |
5.0 (2.53) Md = 4.5 range: 2–8 | 6.0 (4.69) Md = 6.5 range: 1–10 |
| Joint deterioration: yes | 90 (84.9%) | 12 (57.1%) | 5 (45.5%) | 2 (25.0%) |
| Number of affected joints | 4.16 (2.55) Md = 4 range: 1–10 |
1.67 (1.16) Md = 1 range: 1–4 |
1.00 (1.00) Md = 1 range: 1–1 |
1.50 (0.71) Md = 1.5 range: 1–2 |
| Most affected joint b : yes | 72 (67.9%) | 8 (38.1%) | 3 (27.3%) | 0 |
| Knee | 37 (51.4%) | 1 (4.8%) | 0 | 0 |
| Ankle | 29 (40.3%) | 3 (14.3%) | 2 (18.2%) | 0 |
| Elbow | 14 (19.4%) | 4 (19.0%) | 1 (9.1) | 0 |
| Hip | 6 (8.3%) | 0 | 0 | 0 |
| Shoulder | 3 (4.17%) | 0 | 0 | 0 |
| Comorbidities | ||||
| HIV | 14 (13.5%) | – | – | – |
| Hepatitis C | 25 (24.0%) | – | – | – |
| Pain due to hemophilia | ||||
| In the previous year | 82 (77.4%) | 16 (76.2%) | 9 (81.8%) | 4 (50.0%) |
| Lasting over 3 months | 65 (61.3%) | 13 (61.9%) | 6 (54.5%) | 2 (25.0%) |
| More than once a week | 43 (40.6%) | 2 (9.5%) | 1 (9.1%) | 0 |
| Pain with more impact | N = 82 | N = 16 | N = 9 | N = 4 |
| Ankle | 31 (37.8%) | 7 (43.9%) | 5 (55.5%) | 1 (25.0%) |
| Knee | 30 (36.30%) | 2 (12.5%) | 3 (33.3%) | 0 |
| Elbow | 8 (97%) | 3 (18.8%) | 1 (11.1%) | 1 (25.0%) |
| Shoulder | 5 (6.1%) | 0 | 0 | 0 |
| Hip | 5 (6.1%) | 0 | 0 | 0 |
| Others | 3 (3.6%) | 4 (25.1%) | 0 | 2 (50.0%) |
| Pain duration c (months) | 137.70 (136.46) Md = 96 range: 1–612 |
28.67 (38.03) Md = 6 range: 1–108 |
18.00 (22.03) Md = 8.5 range: 1–60 |
4.50 (0.71) Md = 4.50 range: 4–5 |
| Pain frequency c | ||||
| Always present (a) | 12 (14.6%) | 0 | 0 | 0 |
| Daily (b) | 15 (18.3%) | 0 | 0 | 0 |
| Weekly (c) | 12 (14.6%) | 1 (6.3%) | 0 | 0 |
| Only during movement (d) | 21 (25.6%) | 4 (25.0%) | 4 (44.4%) | 0 |
| Bleeding episodes only (e) | 9 (11.0%) | 6 (37.5%) | 4 (44.4%) | 4 (100%) |
| (c) + (d) | 2 (2.4%) | 0 | 0 | 0 |
| (c) + (d) + (e) | 3 (3.7%) | 0 | 0 | 0 |
| (d) + (e) | 8 (9.8%) | 5 (31.3%) | 1 (11.1%) | 0 |
Notes: Continuous variables are presented as mean (SD), median (Md), and range. Categorical variables are presented as n (%).
a
Data reporting to the previous year.
b
More than one response option is possible.
c
Concerning the pain with more impact.