Lung Exacerbations in Young CF Patients Linked to Poorer Quality of Life

Pulmonary exacerbations have a negative impact on the general health-related quality of life in people with cystic fibrosis (CF), a study reported, stressing the importance of preventing such flares in this group.

Assessing general, as opposed to disease-specific, health-related quality of life measures are needed when comparing different disease conditions and determining the usefulness of new treatments.

Researchers at Pharmerit International and Vertex Pharmaceuticals analyzed data from a clinical trial with ivacaftor (Kalydeco). The study, called STRIVE, included 161 CF patients age 12 or older (mean age, 25.5) with the G551D-CFTR mutation. It lasted for 48 weeks.

They collected data on 1,214 observations of pulmonary exacerbations, hospitalizations, lung function measured by predicted forced expiratory volume in one second (ppFEV1), and quality of life measured by the EQ-5D tool — an assessment based both on a descriptive profile and a visual analogue scale (VAS). The tool analyzes mobility, self-care, usual activities, pain or discomfort, and anxiety or depression.

Previous studies have explored how lung exacerbations and lung function using FEV measures impact quality of life, with varying results. Up to now, however, there have been no attempts to study both lung function and the presence of an exacerbation simultaneously.

The study, “Impact of pulmonary exacerbations and lung function on generic health-related quality of life in patients with cystic fibrosis,“ reported that all but 12 lung function and quality of life measurements were performed on the same day. As described in the journal Health and Quality of Life Outcomes, patients most frequently reported pain or discomfort (20.2 percent). Anxiety or depression was also common, present in 16.4 percent, and another 14.1 percent reported experiencing problems with their usual activities.

Among the 161 patients, 72 experienced a total of 146 lung exacerbations, of which 35.6 percent required hospitalization. As expected, quality of life measurements were lowest during a period of one week before or one week after the exacerbation in hospitalized patients. Ratings assessed by VAS scores in the analysis gave results similar to descriptive scales.

The analysis showed that pulmonary exacerbation, primarily when requiring hospitalization, and measures of lung function independently predict quality of life outcomes in young people. The researchers suggested that a reduction in pulmonary exacerbations would likely improve the health-related quality of life among CF patients.