An article published this week in the new edition of Annals of the American Thoracic Society, provides an essential overview of health indicators that are important to patients diagnosed with cystic fibrosis (CF). Study investigators from University of British Columbia, School of Population and Public Health, BC, Canada, conducted a thoroughly researched literature review with the aim of identifying socio-demographic and clinical factors associated with health-related quality of life (HRQoL) among adolescents and adults with CF.
CF is a chronically progressive life-threatening disease, caused by a genetic mutation that affects the normal functioning of the lungs and digestive system. In the US, approximately 1,000 new cases are diagnosed with more than 75% of those patients under the age of two. An estimated 30,000 children and adults in the U.S., and 70,000 worldwide, have the disease. With advancements in treatment modalities, the clinical picture has changed dramatically from the 1950s when few children lived to attend elementary school, to today, where individuals with CF have a lifespan of approximately 30 years, with many patients living into their forties.
With an increase in the number of individuals diagnosed with CF reaching adulthood, many researchers are investigating the psycho-social factors that are of particular importance to ensuring that the increased quality of life is good from a health standpoint. This is usually done by assessing HRQoL, a broad, multidimensional concept that usually includes self-reported measures of physical and mental health. Oftentimes, in CF studies The Cystic Fibrosis Questionnaire-Revised (CFQ-R 14+) is the most widely accepted method to quantify HRQoL. It is this type of research that the study authors reviewed to identify patterns of socio-demographic and clinical factors associated with CF.
The study investigators analyzed data from 23 articles published from 1989 to 2014 with information on patients 14 and older. The relationships between socio-demographic (age, gender, body-mass index (BMI), socioeconomic status and employment) and clinical (percent of forced expiratory volume (FEV1%) predicted, pulmonary exacerbation, co-morbidities) factors with the CFQ-R 14+ was assessed.
The major study finding was that FEV1% predicted and pulmonary exacerbations have the broadest impact on HRQoL for patients with CF. Other findings of interest included:
- Males reported higher Physical Functioning and lower Body Image scores than females
- BMI was positively correlated with Body Image and Weight
- Age was negatively correlated with Treatment Burden
This study adds to the body of knowledge assessing the psychosocial and clinical health indicators that are important to the CF patient population.
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