The Cystic Fibrosis Symptom Progression Survey (CF-SPS) in Arabic: A tool for monitoring patients’ symptoms

Norrish, Catherine; Norrish, Mark; Fass, U.; Al-Salmani, Majid; Lingam, Ganji Shiva; Clark, Fiona; Hebal, Kallesh

doi:10.5001/omj.2015.04

Cited by 3 publications

(6 citation statements)

References 43 publications

(44 reference statements)

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“…Limitations of existing PROMs may include the length of commonly used CF-specific PROMs, which could reduce patient compliance and increase data entry burden. Newly developed CF-specific PROMs identified in this study were substantially shorter, 33 61 79 demonstrating that researchers require less burdensome CF-specific PROMs. Another limitation may be inadequacy of paediatric measures as currently, no validated PROMs exists to measure data in 0–6 year olds.…”

Section: Discussionmentioning

confidence: 83%

“… 59 Several CF-specific PROMs were developed or initially validated during the last decade. These included the CF Respiratory Symptom Diary (CFRSD), 26 CF Symptom Progression Survey, 33 CF Symptom Diary 60 and the Respiratory Symptoms in CF. 61 …”

Section: Resultsmentioning

confidence: 99%

“…Two studies assessing patient views towards PROMs found that parent caregivers were satisfied with the questionnaires. 33 34 Salek et al 3 observed that 76% of patients with CF in their study would be willing to complete the CFQoL at every clinic visit. Overall, as most studies did not report the patient burden of PROMs to their patient populations, this review has found limited information on acceptability of PROMs for patients.…”

Section: Acceptabilitymentioning

confidence: 95%

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A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

2020

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BackgroundTo determine patient-reported outcome measures (PROMs) which may be suitable for incorporation into the Australian Cystic Fibrosis Data Registry (ACFDR) by identifying PROMs administered in adult and paediatric cystic fibrosis (CF) populations in the last decade.MethodsWe searched MEDLINE, EMBASE, Scopus, CINAHL, PsycINFO and Cochrane Library databases for studies published between January 2009 and February 2019 describing the use of PROMs to measure health-related quality of life (HRQoL) in adult and paediatric patients with CF. Validation studies, observational studies and qualitative studies were included. The search was conducted on 13 February 2019. The COnsensus-based Standards for the selection of health Measurement INstruments Risk of Bias Checklist was used to assess the methodological quality of included studies.ResultsTwenty-seven different PROMs were identified. The most commonly used PROMs were designed specifically for CF. Equal numbers of studies were conducted on adult (32%, n=31), paediatric (35%, n=34) and both (27%, n=26) populations. No PROMs were used within a clinical registry setting previously. The two most widely used PROMs, the Cystic Fibrosis Questionnaire—Revised (CFQ-R) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL), demonstrated good psychometric properties and acceptability in English-speaking populations.DiscussionWe found that although PROMs are widely used in CF, there is a lack of reporting on the efficacy of methods and timepoints of administration. We identified the CFQ-R and CFQoL as the most suitable for incorporation in the ACFDR as they captured significant effects of CF on HRQoL and were reliable and valid in CF populations. These PROMs will be used in a further qualitative study assessing patients’ with CF and clinicians’ perspectives toward the acceptability and feasibility of incorporating a PROM in the ACFDR.PROSPERO registration numberCRD42019126931.

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Section: Discussionmentioning

confidence: 83%

Section: Resultsmentioning

confidence: 99%

Section: Acceptabilitymentioning

confidence: 95%

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A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

2020

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“…Cystic fibrosis (CF) is a chronic, debilitating and life‐limiting autosomal recessive genetic disorder that occurs primarily in the Caucasian population or populations of European descent 1 . Affecting over 100,000 individuals worldwide, with the highest prevalence in Ireland, Australia, and North America, respectively, it is considered the most common life‐threatening genetic condition globally 2,3 . CF is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes for chloride channels located on the apical membrane of epithelial cells 4 …”

Section: Introductionmentioning

confidence: 99%

Critical disease burdens of Australian adults with cystic fibrosis: Results from an online survey

Ward

Mauleon

Arellano

et al. 2023

Pediatric Pulmonology

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Background The objective of this study was to conduct a web‐based questionnaire to investigate self‐reported phenotypes and disease burdens of individuals living in Australia and diagnosed with cystic fibrosis (CF) using a case–control study design. Methods An online questionnaire was distributed to individuals with CF and healthy control subjects. Overall health rating, medications, family history, education, clinical indicators of disease, and symptoms, including their severity and frequency, were evaluated. Results There was a total of 119 respondents consisting of 59 people living with CF and 60 controls. The CF cohort had significantly lower tertiary educational levels compared to controls. The analysis specific to the CF cohort depicted a significant correlation between the frequency of hospitalizations and the level of education in the CF cohort. Of the 26 self‐reported symptoms of CF that were analyzed, 14 were significantly higher in the people living with CF. The CF cohort reporting symptoms of chronic pain (25%) described an increase in the burden of disease, depicting a 30% longer mean hospitalization, increased consumption of medications and significant relationships with four other symptoms, including muscle aches, digestive issues, pancreatic insufficiency, and abdominal swelling. Conclusions The nationwide survey identified a diverse range of clinical manifestations experienced by the Australian CF population. Chronic pain, linked to aging and the changing landscape of disease, was a significant indicator of the burden of disease. A comprehensive understanding of the phenotypic profiles and symptom variability will contribute to future research and provide insights into the impacts of disease and the burden of therapy, particularly in children, at the start of their health journey.

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“…Increased sputum production impacts on daily activities and quality of life [1][2][3][4] and is a shared feature of many respiratory diseases. Worldwide, 545 million people have chronic respiratory conditions, with those associated with chronic sputum production including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, chronic bronchitis, and cystic fibrosis.…”

Section: Introductionmentioning

confidence: 99%

Genome-wide association study of chronic sputum production implicates loci involved in mucus production and infection

Packer

Shrine

Hall

et al. 2022

Preprint

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Background Chronic sputum production impacts on quality of life and is a feature of many respiratory diseases. Identification of the genetic variants associated with chronic sputum production in a disease agnostic sample could improve understanding of its causes and identify new molecular targets for treatment. Methods We conducted a genome-wide association study (GWAS) of chronic sputum production in UK Biobank. Signals meeting genome-wide significance (P<5x10-8) were fine-mapped and putative causal genes identified by gene expression analysis. GWAS of respiratory traits were interrogated to identify whether the signals were driven by existing respiratory disease amongst the cases and variants were further investigated for wider pleiotropic effects using phenome-wide association studies (PheWAS). Findings From a GWAS of 9,714 cases and 48,471 controls, we identified six novel genome-wide significant signals for chronic sputum production including the Human Leukocyte Antigen (HLA) locus, chromosome 11 mucin locus (containing MUC2, MUC5AC and MUC5B) and the FUT2 locus. The mucin locus signal had previously been reported for association with moderate-to-severe asthma. The HLA signal was fine-mapped to an amino-acid change of threonine to arginine (frequency 36.8%) in HLA-DRB1 (HLA-DRB1*03:147). The signal near FUT2 was associated with expression of several genes including FUT2, for which the direction of effect was tissue dependent. Our PheWAS identified a wide range of associations. Interpretation Novel signals at the FUT2 and mucin loci highlight mucin flucosylation as a driver of chronic sputum production even in the absence of diagnosed respiratory disease and provide genetic support for this pathway as a target for therapeutic intervention.

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The Cystic Fibrosis Symptom Progression Survey (CF-SPS) in Arabic: A tool for monitoring patients’ symptoms

Abstract: We recommend the CF-SPSa (Arabic version) as a valid tool for the longitudinal monitoring of symptom progression in CF in Arabic speaking populations.

Cited by 3 publications

References 43 publications

A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

Critical disease burdens of Australian adults with cystic fibrosis: Results from an online survey

Genome-wide association study of chronic sputum production implicates loci involved in mucus production and infection

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