Longitudinal assessment of health‐related quality of life in an observational cohort of patients with cystic fibrosis

Abstract

Health status and HRQOL changes were small over a 1-year period in this CF population. However, changes in respiratory symptoms and weight were associated with significant changes on relevant CFQ-R scores, indicating that this PRO is sensitive to changes in health status over time.

Adaptação cultural e propriedades psicométricas iniciais do instrumento DISABKIDS ® – Cystic Fibrosis Module – versão brasileira

“…Note that the fourth item of both versions, can cause the presented effect, since studies indicate that participants with some problem or limitation of health can present a sense of negation (22) and on the other hand parents of children and adolescents with some limitation tend to underestimate their health or even weaken it (23)(24) . Yet Abbott et al (5) indicate that CF patients tend to have a ceiling effect and caution should be taken so that it does not limit the responsiveness of the instrument, since it changes over time cannot be assigned to the interventions but the presence of this effect. * Items in the proxy version are parallel to self-version being phrased so as to allow parents or caregivers can respond the items thinking about him/her child or adolescent.…”

“…The use of instruments for measuring quality of life (QOL) and health-related quality of life (HRQOL) have been identified as an important tool to evaluate patients with CF in a progressively way and contribute to clinical protocols and therapeutic guidelines, considering emotional and social aspects, beyond the expectations of the patient in relation to their health (3)(4)(5) .…”

Prevalence of depression and anxiety in patients with cystic fibrosis and parent caregivers: results of The International Depression Epidemiological Study across nine countries

“…[1][2][3] Cystic fibrosis (CF) is the most common genetic, life-limiting chronic disease among Caucasian populations and requires a treatment regimen that takes between 2 and 4 h per day. 4 Patients have genetic mutations that affect the CF transmembrane conductance regulator protein, leading to thick mucus secretions, repeated infections and progressive failure of most organ systems (e.g., lungs, pancreas, digestive, reproductive). 5 Although recent advances in diagnosis and treatment have led to increases in lifespan, CF continues to be one of the most difficult chronic conditions to manage.…”

Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis

“…The CFQ-R physical functioning scale, CFQ-R vitality scale, CFQ-R health perceptions and CFQ-R respiratory symptoms scale (CFQ-R RSS) have been shown to correlate with FEV1 [14]. The CFQ-R RSS has been utilised successfully both in controlled trials and in longitudinal studies [15][16][17][18][19][20]. The CFQ-R RSS was used as an outcome measure in several clinical trials showing responsivity to inhaled tobramycin [18], dornase alfa [19], hypertonic saline [20] and ivacaftor [16].…”