Lung function in the aging Swedish cystic fibrosis population

Respir Med. 2009 Jul;103(7):1076-82. doi: 10.1016/j.rmed.2009.01.020. Epub 2009 Feb 23.


Swedish Cystic Fibrosis (CF) care follows international guidelines in general. The only difference in our CF care package since 25 years is the physiotherapy regimen. Airway clearance therapy has since the early 1980s, from the day of diagnosis, been based upon daily physical exercise in conjunction with techniques for transporting and evacuating mucus. Postural Drainage+/-percussion and vibration has not been used in any age. The aim of this study was to evaluate our CF care package. Lung function data from the start of the study and with a 3-year interval were collected in the entire Swedish CF population > or =7 years old. Data were analysed for the age groups 7-17 and > or =18 years of age. Change of lung function over the study period was calculated. The impact of chronic Pseudomonas aeruginosa (Pa) colonisation and basal FEV(1)%p was also evaluated in a linear mixed model. Data from 99% of the country-wide CF population were available at inclusion. Mean FEV(1)%p was 90+/-21 vs 73+/-26 in the different age groups and mean VC%p was 94+/-18 vs 91+/-20. Forty percent of the adult group was > or =30 years old. Overall, 41% were chronically Pa colonised. Mean annual FEV(1)%p rate of decline was 0.77 and 0.64 in the different age groups. Lung function among Swedish CF patients is good and annual rate of decline low, even in an old cohort. The large proportion of adult patients emphasises future demands on CF care.

Publication types

  • Evaluation Study

MeSH terms

  • Adolescent
  • Adult
  • Aging / physiology*
  • Child
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis / therapy
  • Drainage, Postural / methods
  • Female
  • Forced Expiratory Volume / physiology
  • Humans
  • Male
  • Middle Aged
  • Practice Guidelines as Topic / standards
  • Prognosis
  • Prospective Studies
  • Respiratory Function Tests
  • Sweden
  • Treatment Outcome
  • Young Adult