Treatment compliance in children and adults with cystic fibrosis

J Cyst Fibros. 2008 Sep;7(5):359-67. doi: 10.1016/j.jcf.2008.01.003. Epub 2008 Mar 4.


Objectives: To determine treatment compliance and how compliance was perceived by patients, parents and by a multidisciplinary team in Cystic Fibrosis (CF) patients. Also to analyse the relative importance given to each of the prescribed treatments, reasons for non-adherence and to investigate possible predictors of therapeutic compliance.

Patients and methods: 34 CF patients (21 females), aged between 1.6 and 40.6 years, attending an outpatient CF clinic.

Design: cross-sectional. A self-administered questionnaire was given to all patients whilst attending a programmed visit which was used to determine compliance to different treatments (physiotherapy, nutritional supplements, respiratory and digestive medications). Patients were subjectively classified as compliant or non-compliant by medical staff involved in their care.

Results: Treatment compliance was greater for digestive (88.2%) and respiratory medication (61.8%), compared to physiotherapy (41.2%) or nutritional supplements (59%). CF patients considered digestive medication indispensable (94.1%), compared to nutritional supplements (44.1%). Whilst 26.4% of CF patients considered that respiratory medications or nutritional supplements influenced little or nothing in their quality of life. Comparing age groups younger patients were found to be more compliant (10.4 vs. 20.5 years p=0.008) and had less severe disease (Shwachman score 83.2 vs. 73.9 p=0.048).

Conclusions: CF patients had greater treatment adherence when prescribed digestive and respiratory medications, compared to physiotherapy or nutritional supplements. Therapeutic adherence was found to worsen with age and disease severity, however improved with treatments which were perceived by patients as more important or had a greater influence in their quality of life.

MeSH terms

  • Adolescent
  • Adult
  • Chi-Square Distribution
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Cystic Fibrosis / therapy*
  • Female
  • Humans
  • Infant
  • Male
  • Patient Compliance*
  • Statistics, Nonparametric
  • Surveys and Questionnaires