Pulmonary function, symptoms, and quality of life in cystic fibrosis: a cross-sectional analysis of the InSPIRe:CF trial Public
Smirnova, Natalia (Spring 2024)
Abstract
Background: People living with cystic fibrosis (CF) experience a high symptom burden. Due to the changing landscape of CF in the era of modulator therapy, we sought to examine the epidemiology of symptoms and the association between pulmonary function, symptoms, and quality of life.
Methods: Using baseline data from a trial of specialist palliative care in adults with CF, we conducted a cross-sectional study on symptom prevalence and distress. We then examined association between pulmonary function and quality of life (measured with the Functional Assessment of Chronic Illness Therapy Total Score), as well as between individual symptoms and quality of life.
Results: Among 262 participants, median age was 33, and 78% were on modulator therapy. The most common symptoms were lack of energy (n = 194, 74%) and cough (190, 73%), whereas the most distressing were difficulty sleeping (range 0-4, mean 2.19, SD 1.15) and pain (mean 2.04, SD 1.1). In an unadjusted model, we observed a non-significant trend toward lower quality of life with worse pulmonary obstruction—compared to participants with mild obstruction, those with moderate obstruction had quality of life score 7.46 points lower (95% CI -15.03 to 0.10) and those with severe obstruction had a score 9.98 points lower (95% CI -21.76 to 1.80). However, this association was no longer statistically significant in the adjusted model, which may reflect confounding due to sex, age, BMI, and modulator therapy. The symptoms that impaired quality of life the most were extrapulmonary: lack of energy (average quality of life score -29.8, 95% CI -36.8 to -22.8), feeling sad (-29.8, 95% CI -35.6 to -23.9) and worrying (-28.7, 95% CI -34.9 to -22.5).
Conclusions: There was no statistically significant association between pulmonary function and quality of life. The symptoms that were associated with the lowest quality of life were extrapulmonary. CF clinicians may consider screening for common symptoms that affect quality of life the most (lack of energy, worrying, difficulty sleeping, feeling irritable, pain, and shortness of breath). These symptoms may identify people living with CF who are most at risk for a decreased quality of life and may benefit from additional support.
Table of Contents
Table of Contents
Introduction
Methods
Results
Discussion
Figure 1. CONSORT diagram.
Table 1. Baseline characteristics of participants
Figure 2. Symptom prevalence and distress among adults with CF.
Table 2. Baseline characteristics of participants by degree of obstruction
Table 3. Differences in Functional Assessment of Chronic Illness Therapy (FACIT-Pal) quality of life score by patient characteristics among adults with cystic fibrosis
Table 4. Multivariate linear regression model of forced expiratory volume in one second (FEV1) and quality of life among adults with cystic fibrosis
Table 5. Multivariate linear regression model of individual symptoms and quality of life among adults with cystic fibrosis
Table 6. Association between the 14 most prevalent symptoms on the Memorial Symptoms Assessment Scale–Cystic Fibrosis scale and quality of life, stratified by modulator therapy use.
Table 7. Association between the 14 most prevalent symptoms on the Memorial Symptoms Assessment Scale–Cystic Fibrosis scale and quality of life, stratified by depression comorbidity.
Table 8. Association between the 14 most prevalent symptoms on the Memorial Symptoms Assessment Scale–Cystic Fibrosis scale and quality of life, stratified by anxiety comorbidity.
Table 9. Symptom prevalence and association with quality of life
References
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