TY - JOUR T1 - The use of home Non-Invasive ventilation in Cystic Fibrosis in a large Uk regional paediatric Service. JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.RFMVC-2020.51 VL - 6 IS - suppl 4 SP - 51 AU - Meg Macdonald AU - Kim Lund AU - Amanda Reed AU - Elinor Burrows AU - Kevin W Southern AU - Clare Halfhide Y1 - 2020/02/13 UR - http://openres.ersjournals.com/content/6/suppl_4/51.abstract N2 - Background: There are no evidence-based guidelines when to initiate Non-Invasive Ventilation (NIV) in Cystic Fibrosis (CF) in children.Aim: To review the use of NIV in all patients with Cystic Fibrosis, within a large regional paediatric service in UK.Methods: A retrospective case note review of patients within the regional CF service, >300 children (0-18 years,) who have used NIV. For each child DOB, genes, markers of lung health and nutritional status were noted. The date, presence of humidification and mode of initial ventilation was noted. Whether the child had a chest hrCT scan pre NIV setup and any post setup complications were noted. Referral for lung transplant, as well as the current clinical condition on 1/10/2019 was noted.Results: Since 2006, 14 children have used NIV. 1 child's records were excluded as archived. Of the 13 children with CF, at the time of NIV set up, all had FEV1 <40% and 1 had Non-Tuberculous Mycobacterium. 10/13 had a gastrostomy and 2 had CFRD. The median age of NIV setup, was 13.67 (IQR 11.17,15.58). 6 had CT scan pre setup, 1/6 had a pneumothorax post setup, 5 had a CT in 4 weeks after set up, 2 had no CT.  9/13 were referred for lung transplant, 3 pre NIV setup. 2/9 received a lung transplant. On 1/10/2019, 6 young people were still alive, 2 awaiting lung transplant, 1 post-transplant, 3 being considered for lung transplant.Conclusions: Within a large paediatric CF service, NIV has been used infrequently but safely. In our population NIV has been used in those with the most severe disease as a bridge to transplant or for palliation. The role of a CT scan pre-setup needs to be evaluated and the indications for initiation need to be determined.FootnotesCite this article as: ERJ Open Research 2020; 6: Suppl. 4, 51.This is an ERS Respiratory Failure and Mechanical Ventilation Conference abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -