Table 4 Patient- and caregiver-reported impact of epidermolysis bullosa on activities of daily living
Does epidermolysis bullosa… | Patient-reported | Caregiver-reported | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|
Overalla N = 63 | Simplex n = 21 | Junctional n = 8 | Dominant dystrophic n = 14 | Recessive dystrophic n = 19 | Overalla N = 93 | Simplex n = 34 | Junctional n = 7 | Dominant dystrophic n = 17 | Recessive dystrophic n = 34 | |
Affect ability to move around home? n (%) | ||||||||||
Severely | 5 (7.9) | 0 | 1 (12.5) | 2 (14.3) | 2 (10.5) | 6 (6.5) | 0 | 1 (14.3) | 0 | 5 (14.7) |
A lot | 12 (19.0) | 3 (14.3) | 2 (25.0) | 4 (28.6) | 3 (15.8) | 16 (17.2) | 6 (17.6) | 3 (42.9) | 4 (23.5) | 3 (8.8) |
A Little | 32 (50.8) | 11 (52.4) | 4 (50.0) | 6 (42.9) | 10 (52.6) | 52 (55.9) | 21 (61.8) | 1 (14.3) | 8 (47.1) | 21 (61.8) |
Not at all | 14 (22.2) | 7 (33.3) | 1 (12.5) | 2 (14.3) | 4 (21.1) | 19 (20.4) | 7 (20.6) | 2 (28.6) | 5 (29.4) | 5 (14.7) |
Affect ability to bathe or shower? n (%) | ||||||||||
Yes, needs help every time | 4 (6.3) | 0 | 0 | 0 | 4 (21.1) | 40 (43.0) | 9 (26.5) | 4 (57.1) | 5 (29.4) | 22 (64.7) |
Yes, needs help most of the time | 4 (6.3) | 0 | 1 (12.5) | 2 (14.3) | 1 (5.3) | 5 (5.4) | 1 (2.9) | 0 | 2 (11.8) | 2 (5.9) |
Yes, sometimes needs help | 17 (27.0) | 1 (4.8) | 2 (25.0) | 6 (42.9) | 7 (36.8) | 18 (19.4) | 6 (17.6) | 1 (14.3) | 4 (23.5) | 6 (17.6) |
No, no impact | 36 (57.1) | 19 (90.5) | 5 (62.5) | 6 (42.9) | 6 (31.6) | 25 (26.9) | 16 (47.1) | 2 (28.6) | 5 (29.4) | 2 (5.9) |
N/A | 2 (3.2) | 1 (4.8) | 0 | 0 | 1 (5.3) | 5 (5.4) | 2 (5.9) | 0 | 1 (5.9) | 2 (5.9) |
Affect ability to write? n (%) | ||||||||||
Cannot write due to EB | 2 (3.2) | 0 | 0 | 0 | 2 (10.5) | 2 (2.2) | 0 | 0 | 1 (5.9) | 1 (2.9) |
Easier to type | 15 (23.8) | 4 (19.0) | 1 (12.5) | 5 (35.7) | 4 (21.1) | 21 (22.6) | 8 (23.5) | 2 (28.6) | 2 (11.8) | 8 (23.5) |
Difficult to grip pen | 14 (22.2) | 4 (19.0) | 6 (75.0) | 2 (14.3) | 2 (10.5) | 19 (20.4) | 5 (14.7) | 0 | 3 (17.6) | 11 (32.4) |
Does not interfere | 32 (50.8) | 13 (61.9) | 1 (12.5) | 7 (50.0) | 11 (57.9) | 27 (29.0) | 10 (29.4) | 4 (57.1) | 5 (29.4) | 8 (23.5) |
N/A | 0 | 0 | 0 | 0 | 0 | 24 (25.8) | 11 (32.4) | 1 (14.3) | 6 (35.3) | 6 (17.6) |
Affect ability to eat? n (%) | ||||||||||
Always rely on tube | 2 (3.2) | 0 | 0 | 0 | 2 (10.5) | 13 (14.0) | 1 (2.9) | 2 (28.6) | 0 | 10 (29.4) |
Sometimes rely on tube | 0 | 0 | 0 | 0 | 0 | 5 (5.4) | 0 | 0 | 1 (5.9) | 4 (11.8) |
A lot | 18 (28.6) | 0 | 3 (37.5) | 3 (21.4) | 12 (63.2) | 16 (17.2) | 3 (8.8) | 1 (14.3) | 3 (17.6) | 9 (26.5) |
A little | 16 (25.4) | 5 (23.8) | 2 (25.0) | 6 (42.9) | 2 (10.5) | 23 (24.7) | 8 (23.5) | 2 (28.6) | 7 (41.2) | 6 (17.6) |
No, eat normally | 27 (42.9) | 16 (76.2) | 3 (37.5) | 5 (35.7) | 3 (15.8) | 35 (37.6) | 22 (64.7) | 2 (28.6) | 5 (29.4) | 5 (14.7) |
N/A | 0 | 0 | 0 | 0 | 0 | 1 (1.1) | 0 | 0 | 1 (5.9) | 0 |
Affect ability to sleep? n (%) | ||||||||||
Nightly | 10 (5.9) | 1 (4.8) | 2 (25.0) | 2 (14.3) | 5 (26.3) | 15 (16.1) | 2 (5.9) | 2 (28.6) | 1 (5.9) | 10 (29.4) |
Most nights | 9 (14.3) | 1 (4.8) | 0 | 3 (21.4) | 5 (26.3) | 21 (22.6) | 8 (23.5) | 2 (28.6) | 4 (23.5) | 6 (17.6) |
A little | 30 (47.6) | 11 (52.4) | 4 (50.0) | 8 (57.1) | 6 (31.6) | 30 (32.3) | 12 (35.3) | 0 | 3 (17.6) | 15 (44.1) |
No, sleep normally | 14 (22.2) | 8 (38.1) | 2 (25.0) | 1 (7.1) | 3 (15.8) | 26 (28.0) | 12 (35.3) | 3 (42.9) | 8 (47.1) | 3 (8.8) |
N/A | 0 | 0 | 0 | 0 | 0 | 1 (1.1) | 0 | 0 | 1 (5.9) | 0 |
Affect ability to shop? n (%) | ||||||||||
Yesb | 6 (9.5) | 3 (14.3) | 0 | 2 (14.3) | 1 (5.3) | 4 (4.3) | 1 (2.9) | 0 | 1 (5.9) | 2 (5.9) |
Needs help all the time | 6 (9.5) | 0 | 1 (12.5) | 1 (7.1) | 4 (21.1) | 9 (0.7) | 2 (5.9) | 0 | 0 | 7 (20.6) |
A lot | 6 (9.5) | 1 (4.8) | 1 (12.5) | 1 (7.1) | 3 (15.8) | 3 (3.2) | 2 (5.9) | 1 (14.3) | 0 | 0 |
A little | 26 (41.3) | 8 (38.1) | 4 (50.0) | 5 (35.7) | 9 (47.4) | 17 (18.3) | 3 (8.8) | 2 (28.6) | 5 (29.4) | 7 (20.6) |
No, not at all | 19 (30.2) | 9 (42.9) | 2 (25.0) | 5 (35.7) | 2 (10.5) | 20 (21.5) | 10 (29.4) | 2 (28.6) | 2 (11.8) | 5 (14.7) |
N/A | 0 | 0 | 0 | 0 | 0 | 40 (43.0) | 16 (47.1) | 2 (28.6) | 9 (52.9) | 13 (38.2) |
Affect ability to play? n (%) | ||||||||||
Always | 8 (12.7) | 0 | 1 (12.5) | 2 (14.3) | 5 (26.3) | 14 (15.1) | 2 (5.9) | 3 (42.9) | 1 (5.9) | 8 (23.5) |
A lot | 21 (33.3) | 6 (28.6) | 4 (50.0) | 5 (35.7) | 6 (31.6) | 30 (32.5) | 11 (32.4) | 1 (14.3) | 5 (29.4) | 13 (38.2) |
A little | 18 (28.6) | 6 (28.6) | 3 (37.5) | 3 (21.4) | 6 (31.6) | 34 (36.6) | 11 (32.4) | 2 (28.6) | 8 (47.1) | 12 (35.3) |
No | 16 (25.4) | 9 (42.9) | 0 | 4 (28.6) | 2 (10.5) | 15 (16.1) | 10 (29.4) | 1 (14.3) | 3 (17.6) | 1 (2.9) |