Table 1.
Patient and caregiver demographics
| DM1 | MM | All | |
|---|---|---|---|
| Patients | |||
| n | 24 | 9 | 33 |
| Age, years [median (SD, range)] | 47.3 (11.51, 33–72) | 45.8 (17.44, 19–75) | 46.9 (12.94, 19–75) |
| Age of first symptom, years [median (SD, range)] | 23.7 (14.67, 0–60) | 29.6 (15.99, 5–53) | 25.2 (115.0, 0–60) |
| First symptoms age <20 years (%) | 9 (37.5) | 2 (22.2) | 11 (33.3) |
| Years since first symptom [median (SD, range)] | 23.6 (12.43, 5–46) | 16.0 (11.64, 0–35) | 21.7 (12.50, 0–46) |
| Taking medication for disease-specific issues [n (%)] | |||
| Yes, currently | 10 (41.7) | 6 (66.7) | 16 (50) |
| Yes, in the past | 2 (8.3) | 0 | 2 (6.3) |
| No, never | 12 (50.0) | 2 (22.2) | 14 (43.8) |
| No, never taken or considered at all | 8 (33.3) | 2 (22.2) | 10 (31.3) |
| No, never taken but discussed with doctor or heard about from a friend | 4 (16.6) | 0 | 4 (12.6) |
| Employment status [n (%)] | |||
| Yes, full-time | 5 (20.8) | 2 (22.2) | 7 (21.2) |
| Yes, part-time | 8 (33.3) | 0 | 8 (24.2) |
| No employment | 9 (37.5) | 4 (44.4) | 13 (39.4) |
| Student | 0 (0.0) | 1 (11.1) | 1 (3.0) |
| Retired | 2 (8.3) | 1 (11.1) | 3 (9.1) |
| Other | 0 (0.0) | 1 (11.1) | 1 (3.0) |
| DM1-Activ-C score [median (SD, range)] | 35.3 (12.80, 8–50) | 16.0 (12.49, 10–46)a | 32.2 (13.58, 8–50) |
| Caregivers | |||
| n | 14 | 5 | 19 |
| Age, years [medium (SD, range)] | 58.0 (12.76, 42–76) | 45.2 (8.96, 38–55) | 54.6 (13.00, 38–76) |
| Age of person they care for, years [median (SD, range)] | 34.9 (15.13, 4–66) | 10.7 (14.15, 2–27)a | 30.4 (17.4, 2–66) |
| Employment status [n (%)] | |||
| Yes, full-time | 6 (42.9) | 5 (100.0) | 11 (57.9) |
| Part-time | 2 (14.3) | 0 | 2 (10.5) |
| No employment | 4 (28.6) | 0 | 4 (21.1) |
| Student | 0 | 0 | 0 (0.0) |
| Retired | 1 (7.1) | 0 | 1 (5.3) |
| Other full-time caregiver | 1 (7.1) | 0 | 1 (5.3) |
| DM1-Activ-C score [medium (SD, range)]—for patients ≥ 16 years of age only | 26.2 (16.22, 0–49) | 12.8 (10.94, 2–28) | 25.8 (17.2, 0–49) |
| Caregiver Strain Index Rating | |||
| Score [medium (SD, range)] | 6.7 (3.87, 1–13) | 9.0 (1.58, 7–11) | 7.3 (3.53, 1–13) |
| Categories [n (%)] | |||
| No additional stress | 0 (0.0) | 0 (0.0) | 0 (0.0) |
| Some additional stress, possible need for intervention | 7 (53.8) | 0 (0.0) | 7 (38.9) |
| High level of stress, i.e. score ≥ 7 | 6 (46.2) | 5 (100.0) | 11 (61.1) |
SD standard deviation, DM1 myotonic dystrophy type 1, MM mitochondrial myopathy
aSignificant difference between groups p < 0.05