Not individually supportive | Somewhat supportive | Strongly supportive | Total (weighted) | |
Clinical features | ||||
Pancreatic insufficiency | 0 (0) | 13 (24.07) | 41 (75.93)# | 142.5 |
Infertility/CBAVD | 0 (0) | 13 (24.07) | 41 (75.93)# | 142.5 |
Bronchiectasis – diffuse | 1 (1.85) | 22 (40.74) | 31 (57.41)# | 126 |
Radiographic pancreatic fibrosis | 1 (1.85) | 33 (61.11) | 20 (37.04) | 109.5 |
Daily sputum production | 6 (11.11) | 38 (70.37) | 10 (18.52) | 87 |
Aquagenic wrinkling | 16 (29.63) | 24 (44.44) | 14 (25.93) | 78 |
Frequent need for antibiotics for chest | 10 (18.52) | 36 (66.67) | 8 (14.81) | 78 |
Vitamin A/E deficiency | 10 (18.52) | 38 (70.37) | 6 (11.11) | 75 |
Nasal polyposis | 14 (25.93) | 32 (59.26) | 8 (14.81) | 72 |
ABPA diagnosis | 13 (24.07) | 38 (70.37) | 3 (5.56) | 66 |
Bronchiectasis – asymmetrical | 19 (35.19) | 27 (50) | 8 (14.81) | 64.5 |
Radiographic rhinosinusitis | 15 (27.78) | 36 (66.67) | 3 (5.56) | 63 |
Liver disease/steatosis/cirrhosis | 29 (53.7) | 4 (7.41) | 55.5 | |
Obstructive spirometry | 20 (37.04) | 32 (59.26) | 2 (3.7) | 54 |
Osteoporosis/osteopenia | 32 (59.26) | 20 (37.04) | 2 (3.7) | 36 |
Constipation | 37 (68.52) | 14 (25.93) | 3 (5.56) | 30 |
Vitamin D deficiency | 37 (68.52) | 15 (27.78) | 2 (3.7) | 28.5 |
Airway microbiology | ||||
Burkholderia cepacia complex | 2 (3.7) | 14 (25.93) | 38 (70.37)# | 135 |
Pseudomonas aeruginosa | 23 (42.59) | 30 (55.56)# | 124.5 | |
Stenotrophomonas maltophilia | 7 (12.96) | 31 (57.41) | 16 (29.63) | 94.5 |
Mycobacterium abscessus sp. | 7 (12.96) | 32 (59.26) | 15 (27.78) | 93 |
Achromobacter species | 9 (16.67) | 30 (55.56) | 15 (27.78) | 90 |
MRSA | 12 (22.22) | 33 (61.11) | 9 (16.67) | 76.5 |
MSSA | 12 (22.22) | 36 (66.67) | 6 (11.11) | 72 |
Mycobacterium avium complex | 12 (22.22) | 37 (68.52) | 5 (9.26) | 70.5 |
Aspergillus fumigatus sp. | 22 (40.74) | 31 (57.41) | 1 (1.85) | 49.5 |
Streptococcus pneumoniae | 48 (88.89) | 6 (11.11) | 0 (0) | 9 |
Data are presented as n (%), unless otherwise stated. Total score calculated on a basis of 0, 1.5 and 3 points allocated for each count of “Not supportive”, “Somewhat supportive” and “Strongly supportive”, respectively. CF: cystic fibrosis; CBAVD: congenital bilateral absence of the vas deferens; ABPA: allergic bronchopulmonary aspergillosis; MRSA: methicillin-resistant Staphylococcus aureus; MSSA: methicillin-sensitive S. aureus. #: responses with ≥50% selection as “Strongly supportive” of need for follow-up at a specialist CF centre.