There are no guidelines for the assessment of gas exchange or timing and mode of NIPPV initiation in patients with CF. The British Thoracic Society guideline states ‘there is insufficient evidence to recommend its routine use in patients with CF’. 5 The aim of this study was to establish current practice with regard to investigation of respiratory failure, factors leading to NIPPV initiation and extent of the use of this modality across UK and Australasian (ANZ) paediatric CF centres. A semi-structured questionnaire consisting of 21 closed and open-ended questions was sent to the lead CF consultant and CF physiotherapists of specialist paediatric CF centres in the UK (n¼27) and ANZ (n¼14). The response rate was 88% (25 UK centres, 11 ANZ centres), representing a total of 5954 children. Twenty-three children (0.39%) from 13 centres were using NIPPV (11 UK and 12 ANZ). The median (range) age of NIPPV initiation was 14 (6e17) years and the median (range) usage of NIPPV per night was 8 (3e10) h. Eleven of the 36 centres (31%) reported that they have a protocol for NIPPV initiation, but it was unclear whether this was specific for CF. The preferred mode of NIPPV was bi-level NIPPV (75%), followed by single-level preset pressure ventilation (19%), volume control single-level ventilation (3%) and continuous positive airways pres- sure (3%). Nasal masks were the most frequently used interface (47%), followed by full face masks (38%), mouthpieces (13%) and nasal pillows (2%). Less than half of the CF centres (17/36) undertook CF sleep studies. SpO 2 monitoring alone was most commonly used (51% of centres), followed by SpO 2 and trans- cutaneous carbon dioxide monitoring (22%). Full polysomography was less frequently used as a first-line investigation (16.2%). Assessment of respiratory failure differed between childhood CF centres, with different definitions for hypoxia and hypercapnia in use (table 1). The principal reasons for initiating NIPPV included in an acute exacerbation, as a bridge to transplant and as an adjunct to physio- therapy. There were 19 reported NIPPV failures in children with CF from 10 centres. Reasons for failure included claustrophobia, inability to tolerate pressure, discomfort, poor initial set-up, parent or patient anxiety and poor adherence. Four adverse advents were reported (issues with mask fitting and pressure sores, n¼2; retained secretions, n¼1; and pneumothorax, n¼1). NIPPV is rarely used in UK and ANZ paediatric CF populations, probably due to improved patient outcomes and the very low prevalence of respiratory failure in childhood CF. Bi-level NIPPV is the preferred mode of ventilation. However, there is no agreed definition of hypoxia and hypercapnia, no uniformity in assessing gas exchange and no standard protocol for the indications and institution of NIPPV in children with CF. As very few of the expected benefits of NIPPV have been proven, particularly in the paedi- atric CF population, and as there is a high frequency of NIPPV failure, the need for future research in this area is highlighted, beginning with the need for protocols to be developed and evaluated. Acknowledgements The authors acknowledge Dr Michelle Chatwin and Dr Ian Balfour-Lynn, Royal Brompton Hospital, London, UK N Collins, 1 A Gupta, 2 S Wright, 3 L Gauld, 3,4 D Urquhart, 2,4 A Bush 2 1 Department of Physiotherapy, Royal Brompton Hospital, London, UK; 2 Department of Paediatric Respiratory Medicine Royal Brompton Hospital and Imperial College, London, UK; 3 Royal Brisbane Children’s Hospital, Brisbane, Australia; 4 Mater Children’s Hospital, Brisbane, Australia Correspondence to Nicola Collins, Physiotherapy Department, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; n.collins@rbht.nhs.uk Competing interests None. Provenance and peer review Not commissioned; externally peer reviewed. Accepted 19 May 2010 Published Online First 3 September 2010 Thorax 2011;66:538e539. doi:10.1136/thx.2010.139063 REFERENCES 1. Fauroux B, Le Roux E, Ravilly S, et al. Long-term noninvasive ventilation in patients with cystic fibrosis. Respiration 2008;76:168e74. 2. Young A, Wilson J, Kotsimbos T. Randomised placebo controlled trial of noninvasive ventilation for hypercapnia in cystic fibrosis. Thorax 2008;63:72e7. 3. Fauroux B, Boule M, Lofaso F, et al. Chest physiotherapy in cystic fibrosis: improved tolerance with nasal support ventilation. Pediatrics 1999;103:E32e40. 4. Holland AE, Denehy L, Ntoumenopoulos G, et al. Noninvasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 2003;58:880e4. 5. British Thoracic Society. BTS guideline: Non- invasive ventilation in acute respiratory failure. Thorax 2002;57:192e211. Effects of omalizumab in Aspergillus-associated airway disease The clinical spectrum of Aspergillus-associ- ated airway diseases (AAAD) includes Aspergillus-induced asthma, allergic bron- chopulmonary aspergillosis (ABPA) and bronchocentric granulomatosis. Corticoste- roids are almost always used to suppress the immunological response to the fungal anti- gens. 1 Although there are no evidence-based alternative treatment options besides steroids, the well-known adverse effects of these drugs have prompted clinicians to look beyond this standard practice and several cases of ABPA patients with very positive outcomes after omalizumab therapy have been recently published. 2e6 We recruited 18 patients (13 women; mean age 49617 years) with AAAD (2 of them had been previously diagnosed with cystic fibrosis) from 11 Spanish hospitals. All of them had been treated with omali- zumab for at least 16 weeks and they were receiving inhaled corticosteroids (daily dose 13516554 mg budesonide or its equivalent) and a long-acting b2 agonist at the moment of omalizumab initiation. Seventeen patients were being treated with oral corticosteroids at a median daily dose of 16 mg prednisone or its equivalent (IQR 6e28) and 10 with itraconazole. The mean number of albuterol puffs per day was 3.5 (range 1e8). Prior to omalizumab administration, IgE levels were (median (IQR)) 698 IU/ml (478e977) and the median absolute count of eosinophils in blood was 610 mm 3 (317e1015). Sixteen of the 18 patients had CT-diagnosed bronchi- ectasis and fleeting pulmonary opacities were identified in 10 of them. All patients showed a delayed positive skin test for Aspergillus and 17 also developed an imme- diate response. Serum Aspergillus-specific IgE was found in all patients and precipitating antibodies in serum were observed in 10. Omalizumab-treated patients were followed up for a median of 36 weeks (IQR, 28e42). The mean dose of omalizumab per week was 6086108 mg. No significant adverse effects were observed. The treatment was discontinued in five patients due to a lack of response and in another patient because of a positive test for pregnancy. The clinical and functional effects of omalizumab are summarised in table 1. In this series, the largest reported to date, omalizumab has demonstrated a beneficial effect in reducing symptoms and exacerba- tions in a group of patients with AAAD. It Table 1 Variation in hypoxia and hypercapnia definition used between CF centres in the UK and Australasia (16 centres were unsure of the definitions) Hypoxia Hypercapnia Frequent desaturations <90% Petco 2 >6.7 kPa Baseline SaO 2 <93% CO 2 >6.7 kPa for >25% sleep study SpO 2 <90% for >10% sleep study Tcco 2 rise to 6.7 kPa or rise by 0.9 kPa during sleep SpO 2 <92% for >5% sleep study CO 2 >7 kPa CO 2 , carbon dioxide; PetCO 2 , end tidal carbon dioxide pressure; SaO 2 , arterial oxygen saturations; SpO 2 , non-invasive oxygen saturations; TcCO 2 , transcutaneous carbon dioxide pressure. Thorax June 2011 Vol 66 No 6 539 PostScript on March 1, 2022 by guest. Protected by copyright. http://thorax.bmj.com/ Thorax: first published as 10.1136/thx.2010.153312 on 11 March 2011. Downloaded from