Mortality Due to Cystic Fibrosis over a 36-Year Period in Spain: Time Trends and Geographic Variations

Abstract

The aim of this study is to analyze population-based mortality attributed to cystic fibrosis (CF) over 36 years in Spain. CF deaths were obtained from the National Statistics Institute, using codes 277.0 from the International Classification of Diseases (ICD) ninth revision (ICD9-CM) and E84 from the tenth revision (ICD10) to determine the underlying cause of death. We calculated age-specific and age-adjusted mortality rates, and time trends were assessed using joinpoint regression. The geographic analysis by district was performed by standardized mortality ratios (SMRs) and smoothed-SMRs. A total of 1002 deaths due to CF were identified (50.5% women). Age-adjusted mortality rates fell by −0.95% per year between 1981 and 2016. The average age of death from CF increased due to the annual fall in the mortality of under-25s (−3.77% males, −2.37% females) and an increase in over-75s (3.49%). We identified districts with higher than expected death risks in the south (Andalusia), the Mediterranean coast (Murcia, Valencia, Catalonia), the West (Extremadura), and the Canary Islands. In conclusion, in this study we monitored the population-based mortality attributed to CF over a long period and found geographic differences in the risk of dying from this disease. These findings complement the information provided in other studies and registries and will be useful for health planning.

Cystic fibrosis-related mortality trends in Brazil for the 1999-2017 period: a multiple-cause-of-death study

“…In Spain, for the 1981-2016 period, there was an overall slight decrease in the age-adjusted mortality rate. 17 In 27 countries of the European Union, a continuous downward trend in CF-related mortality rates was observed from 1996 to 2010, although there were differences by country and sex. 18 In Italy, for the 1970-2011 period, CF-related mortality rates decreased in newborns and in children, whereas they increased in adolescents and young adults until 1990, but then decreased; however, in patients older than 19 years of age, they started to increase in 1990.…”

“…Na Espanha, no período de 1981 a 2016, houve uma ligeira redução geral da taxa de mortalidade ajustada pela idade. (17) Em 27 países da União Europeia, foi observada uma tendência contínua de redução das taxas de mortalidade relacionada à FC de 1996 para 2010, embora tenha havido diferenças conforme o país e o sexo. (18) Na Itália, no período de 1970 a 2011, as taxas de mortalidade relacionada à FC diminuíram em recém-nascidos e crianças, e aumentaram em adolescentes e adultos jovens até 1990, mas depois diminuíram; entretanto, em pacientes com mais de 19 anos de idade, as taxas começaram a aumentar em 1990.…”

Managing Cystic Fibrosis in Polish Healthcare

“…In Spaniards, the average death age for the 36-year period was 27.7 years. In the first part of the study between 1981 and 1998, the average death age was 13.7 years, while in the second part (1999–2016) it was 41.1 years [ 8 ]. The European study from 27 countries of the CF population in 1994–2010 revealed that females had a slightly higher mortality rate than males, with a downward trend observed for both genders.…”

“…However, one of the key factors seems to be the quality of healthcare received by the patients; the other is access to drug programs dedicated to the disease. In many highly developed countries with publicly funded healthcare systems, CF has no longer been regarded as a paediatric disease for many years now; it is estimated that over 50% of the population of CF patients in such countries are adults, and the average age of the population has been growing steadily [ 7 , 8 , 9 , 10 , 11 , 12 ]. Along with the significant increase of the average life expectancy of CF patients, the clinical spectrum of this complex multi-systemic disease is constantly evolving [ 13 ].…”

The Fitter the Better? Cardiopulmonary Exercise Testing Can Predict Pulmonary Exacerbations in Cystic Fibrosis

“…Hence, overall survival in CF has increased dramatically since 1992 [ 8 ], and interest has shifted toward recognizing potential predictors of pulmonary exacerbations in order to prevent worsening in patients’ clinical condition [ 9 ].…”