This report presents the data collected in 2017. 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.
Since its establishment in 1998, the Belgian CF Registry (BCFR) has grown steadily and had 1319 patients registered in 2017. This number excludes four patients whose diagnosis for CF was revoked and fourteen without a confirmed diagnosis. There were 38 newly diagnosed patients in 2017, among them two adults, with a median age at diagnosis of 5.4 months with a range from birth to 60.4 years. All the newly diagnosed patients were genotyped; 34 had sweat chloride values > 60 mmol/L.
Among the patients followed-up in 2017, 51.8% were male and 62.2% adults, and the median age was 22.8 years. Interestingly, at the start of the registry, 18 years ago, 39.0% were adults, and the median age was 14.9 years. 46.4% of the patients are homozygous for the F508del mutation and 39.1% are F508del heterozygous. The main reasons for diagnosis of CF are acute or recurrent respiratory problems (42.2%) and failure to thrive (24.4%). About 18.7% were diagnosed via neonatal screening even though Belgium had no national neonatal screening program at the time. Within the year, six deaths were reported (two of them in transplanted patients) with age at death ranging from 11.8 to 70.4 years. Ten patients benefitted from a lung transplant, and 13.9% of the patients in the registry are living with a transplant.
Among the adults, the proportion of underweight patients continues to decline from about 31.2% in 1998 to 16.7% in 2010 and 10.8% in 2017 (BMI < 18.5 kg/m²); this decline was noted also amongst the F508del homozygous patients; 32.8% in 2000, 21.9% in 2010, and 15.8% in 2017. 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 54.8% and 11.3% respectively among the children and adults in 2017.
The overall annual prevalence of Pseudomonas aeruginosa reported in 2017 was 39.1%. This is the first time the prevalence has increased since the decline observed from 42.4% in 2012 to 37.5% in 2016. There has also been a steady increase in the prevalence of Achromobacter xylosoxidans from 5.9% in 2009, stabilizing at prevalence levels above 10.0% since 2012, but reaching 11.4% in 2017. The prevalence of the Burkholderia cepacia complex on the other hand has decreased to 2.9%, after a stable period at 3.5% over the years since 2014.
Thanks to improved disease management 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 various challenges, expectations and disease-related complications. In 2017, CF-related diabetes had a prevalence of 18.7% and 66.1% in non-transplanted and in transplanted adults respectively. Other complications reported include early osteoporosis and CF related arthritis/arthropathy. These require specialized care for the adult CF patients.