TABLE 3

Lower respiratory symptoms of Swiss children and adults with primary ciliary dyskinesia (n=74)

TotalChildren <18 yearsAdults ≥18 years
Number of participants74 (100)24 (100)50 (100)
Cough
 Daily/often53 (72)16 (67)37 (74)
 Sometimes/rarely20 (27)7 (29)13 (26)
Never1 (1)1 (4)0 (0)
Daily cough, long severe attacks8 (11)3 (13)5 (10)
Daily cough, short and mild46 (62)13 (54)33 (62)
Daily cough only on exercise/drug inhalation9 (12)2 (8)7 (14)
Cough worst during the day54 (73)14 (58)40 (80)
Problems due to cough
 Gastro-oesophageal reflux11 (15)4 (17)7 (14)
 Vomiting8 (11)4 (17)4 (8)
 Hernia3 (4)0 (0)3 (6)
 Urinary incontinence6 (8)1 (4)5 (10)
Sputum production
 Daily/often53 (72)16 (67)37 (74)
 Sometimes/rarely/only during physiotherapy20 (27)8 (33)12 (24)
 Never1 (1)0 (0)1 (2)
Sputum quantity expectorated per day
 1 teaspoon12 (16)4 (17)8 (16)
 1 tablespoon19 (26)5 (21)14 (28)
 50–60 mL16 (22)4 (17)12 (24)
 100–120 mL7 (9)0 (0)7 (14)
 Unknown (usually swallowed)/no sputum20 (27)11 (46)9 (18)
Wheezing
 Daily/often9 (12)2 (8)7 (14)
 Sometimes/rarely37 (50)10 (42)27 (54)
 Never/not reported28 (38)12 (50)16 (32)
Triggers for wheeze
 Colds/infections23 (31)5 (21)18 (36)
 Exercise/sports23 (31)7 (29)16 (32)
 House dust7 (9)2 (8)5 (10)
 Pollen2 (3)0 (0)2 (3)
 Pets2 (3)1 (4)1 (2)
 Cold air9 (12)1 (4)8 (16)
 Changes in weather9 (12)1 (4)8 (16)
 Intense laughter or crying7 (9)2 (8)5 (10)
Wheeze improves with inhaled medication22 (30)6 (25)16 (32)
Shortness of breath
 Daily/often15 (20)2 (8)13 (26)
 Sometimes/rarely34 (46)7 (29)27 (54)
 Never/not reported25 (34)15 (63)10 (20)
Triggers for shortness of breath
 Colds/infections18 (24)6 (25)12 (24)
 Exercise/sports38 (51)7 (29)31 (62)
 Walking up stairs30 (41)4 (17)26 (52)
 Daily activities/rest7 (9)0 (0)7 (14)
Dyspnoea mMRC score#
 024 (48)
 115 (30)
 22 (4)
 32 (4)
 Not reported7 (14)
Chest pain
 Daily/often3 (4)1 (4)2 (4)
 Sometimes/rarely37 (50)5 (21)32 (64)
 Never/not reported34 (46)18 (75)16 (32)
Triggers for chest pain
 Colds/infections17 (23)2 (8)15 (30)
 Exercise/sports18 (24)4 (17)14 (28)
 Walking upstairs10 (14)2 (8)8 (16)
 Daily activities/rest15 (20)2 (8)13 (26)

Data are presented as n (%). mMRC: modified Medical Research Council dyspnoea scale. #: only asked in adults.