Table 1.
Characteristics of patients with primary ciliary dyskinesia participating in open-ended interviews and cognitive testing
| Open-Ended Interviews |
Cognitive Interviews |
|||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Child |
Parent Proxy |
Adolescent |
Child |
Parent Proxy |
Adolescent |
|||||||
| UK/I (n = 4) | North America (n = 16) | UK/I (n = 6) (9 children) | North America (n = 23) (20 children) | UK/I (n = 4) | North America (n = 16) | UK/I (n = 10) | North America (n = 4) | UK/I (n = 10) | North America (n = 7) (6 children) | UK/I (n = 8) | North America (n = 8) | |
| Participant sex | ||||||||||||
| Male | 3 | 5 | 4 | 10 | 1 | 11 | 3 | 3 | 3 | 2 | 5 | 4 |
| Female | 1 | 11 | 5 | 10 | 3 | 5 | 7 | 1 | 7 | 4 | 3 | 4 |
| Participant age | ||||||||||||
| 6–12 yr | 4 | 15 | 9 | 15 | 0 | 1 | 10 | 4 | 10 | 5 | 0 | 0 |
| 13–17 yr | 0 | 1 | 0 | 5 | 4 | 15 | 0 | 0 | 0 | 1 | 8 | 8 |
| Ethnicity | ||||||||||||
| White | 4 | 14 | 8 | 16 | 4 | 14 | 8 | 3 | 8 | 4 | 8 | 5 |
| Black | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Hispanic | 0 | 0 | 0 | 1 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 1 |
| Asian | 0 | 2 | 1 | 2 | 0 | 1 | 2 | 1 | 2 | 2 | 0 | 2 |
| Other | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Missing | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Age at diagnosis | ||||||||||||
| Years (SD) | 3 (1) | 3 (2) | 4 (3) | 4 (3) | 4 (2) | 8 (4) | 3 (3) | 6 (5) | 3 (3) | 4 (4) | 5 (3) | 6 (4) |
| Range | 1–4 | 0–7 | 0–8 | 0–10 | 3–7 | 2–15 | 0–7 | 0–11 | 0–8 | 0–11 | 0–10 | 0–11 |
| Time since diagnosis | ||||||||||||
| Years (SD) | 8 (3) | 6 (3) | 4 (2) | 7 (3) | 10 (4) | 7 (4) | 6 (4) | 4 (3) | 8 (3) | 4 (2) | 9 (3) | 9 (4) |
| Range | 4–9 | 3–12 | 1–6 | 4–12 | 5–14 | 1–15 | 2–10 | 0–8 | 3–11 | 2–7 | 5–12 | 2–14 |
| FEV1% predicted | ||||||||||||
| Mean (SD) | 80 (21) | 85 (17) | 87 (9) | 90 (18) | 90 (16) | 96 (17) | 76 (16) | 91 (20) | 86 (16) | 88 (18) | 88 (13) | 91 (17) |
| Range | 59–103 | 58–109 | 75–99 | 58–109 | 68–105 | 73–118 | 49–96 | 69–116 | 67–111 | 69–116 | 73–110 | 69–124 |
| Missing | 0 | 1 | 3 | 1 | 0 | 0 | 0 | 0 | 2 | 0 | 0 | 0 |
| disease characteristics | ||||||||||||
| Chronic cough | 4 | 16 | 8 | 19 | 4 | 16 | 8 | 4 | 10 | 6 | 8 | 8 |
| Chronic nasal congestion | 4 | 16 | 7 | 20 | 4 | 16 | 8 | 4 | 10 | 6 | 7 | 8 |
| Bronchiectasis | 1 | 14 | 2 | 20 | 1 | 12 | 1 | 4 | 2 | 6 | 4 | 8 |
| Situs inversus totalis | 0 | 7 | 5 | 10 | 2 | 5 | 4 | 1 | 2 | 1 | 2 | 3 |
| Situs ambiguous | 0 | 2 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| Cardiac disease | 0 | 1 | 2 | 2 | 1 | 1 | 1 | 0 | 1 | 0 | 1 | 0 |
Definition of abbreviation: UK/I = UK and Ireland.