Australian Cystic Fibrosis Data Registry Annual Report 2015

Governance & Management • The Australian Cystic Fibrosis Data Registry (ACFDR) commenced data collection in 1998 under management of its funder, Cystic Fibrosis Australia and with the support of an Advisory Committee of senior cystic fibrosis physicians until mid–2016. From September 2016, operational management of the Registry was transferred to the Department of Epidemiology and Preventive Medicine, Monash University. • In accordance with the Australian Commission on Safety and Quality in Healthcare’s Operating Principles and Technical Standards for Clinical Quality Registries (2008) and Framework for Clinical Quality Registries (2014), the ACFDR has transitioned to a centralised ethics and governance process. The ACFDR received ethics approval for this from the Alfred Hospital Human Research Ethics Committee (HREC) in February 2017. • In December 2016 the previous Advisory Committee was transitioned to a multidisciplinary Steering Committee comprising cystic fibrosis physicians, an allied health representative, a consumer representative, a health service representative, the CEO of Cystic Fibrosis Australia, and Monash University academics. A revised ACFDR Protocol and new Data Access Policy was endorsed by the Steering Committee at its first meeting. • The new Protocol reflects extended aims for the Registry including to support ongoing review and development of the ACFDR’s dataset and clinical indicators; to review and monitor trends in patient outcomes as measured by key indicators over time; to facilitate clinical trial data collection; and to undertake data linkage studies with other significant datasets, registries and biobanks. • The ACFDR has enhanced its communication with stakeholders through initiation of a quarterly newsletter. Registry Demographics & Diagnosis • At the end of 2015, the ACFDR held records of 3,379 Australians with cystic fibrosis (CF), believed to capture over 90% of the eligible population of patients with CF. This includes 98 new diagnoses since 2014. • The median age of the Registry population was 18.8 years at 31 December 2015, an increase from 18.4 years reported in 2014. Similarly, the proportion of the Registry population that is adult (18 years and over) increased to 52.0 percent in 2015, from 51.1 percent in 2014. • CF diagnosis was generally reported (73%) within the first 3 months of age, and was determined following neonatal screening in approximately 50% of reported cases. • The proportion of patients who were pancreatic insufficient was 82.2%, and the proportion with sweat chloride levels of ≥ 60 mmol/L was 71%. • The most common genetic mutation remains F508del which is present in 92.1% of participants. Clinical Measures • Clinical information collected by the ACFDR included respiratory infections, medical complications, lung function and nutritional measures. • Summary of lung function findings: – Approximately 5% of 6–11 yr age group have FEV1 values < 70% predicted – Approximately 13% of 12–17 yr age group have FEV1 values < 70% predicted – 16% of adult (18+ yr) males and 12% of adult females have FEV1 values < 40% predicted – Only 16.2% of adults have FEV1 values ≥ 90% predicted (normal). • Summary of nutritional findings: – Median height percentile for 2–5 yr children is above 60%, but falls below 50% for 6–11 yr and below 45% for 12–17 yr age groups; – 45.4 percent of males and 46.7 percent of female children and adolescents were below the 50th percentile for BMI; – Approximately 56–7% of adults had an average BMI score in the 20 to 25 range; – 25% of adult females and 15% of males had an average BMI score < 20. • The proportion of CF patients with gastro–oesophageal reflux, insulin–dependent diabetes or osteoporosis is 33.4%, 20.5% and 5.9% respectively. Patient Management • Antibiotic therapy use summary – 93.6% of CF patients were prescribed continuous or PRN antibiotic therapy in 2015, and more than 88% of patients in each age group; – Half of antibiotic users used inhaled antibiotics, with this proportion generally greater in older age groups. • Lung transplantation data – 44 patients were accepted for lung transplants, and 30 patients actually received a lung transplant. • Mortality – 17 patients were reported to have died in 2015. Registry Quality Assurance • Similar to international Registry comparisons, completeness of ACFDR data varies depending on the data item, but also varies by hospital. Interpretation of data analysis should therefore be undertaken with this in mind. • ACFDR data completeness ranged from a high of 95–100% for Demographic, Clinical Measurement, and Genotype data; but was much lower for Complications and Social data, at < 50%. Future Developments • The ACFDR under guidance of its Steering Committee, is evolving its analysis of Registry data, with an aim to including greater time–series and sub–cohort analysis, and defining a suite of risk adjusted benchmarked clinical indicators that measure best practice care. • The ACFDR will undergo continued refinement of its dataset including proposed changes to diagnostic criteria. • The development of training modules to support site analysis of their own data. • The further enhancement of the ACFDR information on the Registry’s website.