This report presents the data collected in 2016. It is our hope that the analysis of the registry data will provide readers with information on various aspects of CF and continue to provide an important tool for monitoring the patient’s quality of care and trends. In this edition, a brief analysis of anthropometry, spirometry, common complications and therapy in transplant patients has been added.
Since its establishment in 1998, the Belgium CF Registry (BCFR) has grown steadily and had 1275 patients registered in 2016. This number excludes five patients whose diagnosis for CF was revoked and fifteen without a confirmed diagnosis. There were 23 newly diagnosed patients in 2016, among them three adults, with a median age at diagnosis of 3.8 months with a range from birth to 52.3 years. All the newly diagnosed patients were genotyped; while 22 had sweat chloride values > 60 mmol/L.
Among the patients in follow-up in 2016, 52.0% were male and 61.2% adults with a median age of 22.5 years. This can be compared to the start of the registry 17 years ago when 39.0% were adults with a median age of 14.9 years. 46.7% of the patients are homozygous for the F508del mutation while 37.0% are F508del heterozygous. The main reasons for diagnosis of CF are acute or recurrent respiratory problems (42.1%) and failure to thrive (24.4%). About 18.0% were diagnosed via neonatal screening even though Belgium has no national neonatal screening program so far. Within the year, eight deaths were reported (four of them in transplanted patients) with age at death ranging from 20.5 to 44.8 years while 17 patients benefitted from a lung transplant. About 14.0% of the patients in the registry are living with a transplant.
Among the adults, the proportion of patients with BMI < 18.0 kg/m² continues to decline from about 36.3% in 1998 to 17.4% in 2010 and 11.7% in 2016; this decline was noted also amongst the F508del homozygous patients. Amongst the patients up to 20 years, the proportion with BMI below the tenth percentile has also been declining over the years. The above suggests better nutritional management in the patients. The patient population continues to record an improvement in lung function expressed as the mean percentage of predicted FEV1. Among the F508del homozygous patients, 38.0% of the children and 5.1% of the adults had FEV1 ≥ 90.0% of predicted in 1998 compared to 52.9% and 7.0% in 2010 and 53.7% and 13.6% respectively among the children and adults in 2016.
The overall annual prevalence of Pseudomonas aeruginosa reported in 2016 was 37.5% and has been declining compared to a prevalence of 42.4% in 2012. This prevalence has been below 40.0% since 2015. The prevalence of the Burkholderia cepacia complex on the other hand had remained stable over the years since 2014 at about 3.5% There has also been a steady 14 Summ ar y increase in the prevalence of Achromobacter xylosoxidans from 5.9% in 2009 stabilizing at prevalence levels above 10.0% since 2012.
Thanks to improved disease management practises and novel treatments, the life expectancy and the quality of life of patients with CF has improved significantly when compared to CF cohorts a decade or two ago. The proportion of adult CF patients aged 18 years and above increases each year. But this progress is also accompanied by different challenges, expectations and disease related complications. In 2016, CF related diabetes had a prevalence of 24.5% and 53.1% in non- transplanted and transplanted adults respectively. Other complications reported include early osteoporosis and CF related arthritis/arthropathy. These require specialized care for the adult CF patient.