Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis

Pattie, Phillip; Ranganathan, Sarath; Harrison, Joanne; Vidmar, Suzanna; Hall, Graham; Foong, Rachel E.; Harper, Alana; Ramsey, Kathryn; Wurzel, Danielle; Cf, Arest

doi:10.1016/j.jcf.2021.11.005

Cited by 4 publications

(5 citation statements)

References 35 publications

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“…There were almost no differences between age groups and the assessment of their social functioning (61.90 vs. 61.91 vs. 63.89), which shows that all CF patients suffer in that part of their life regardless of their age. Social functioning was the lowest-scored domain in the study by Pattie et al [ 33 ] conducted among children aged 6 to 10 years old. Vitality and body image were scored the lowest by the 14 years and older patients.…”

Section: Discussionmentioning

confidence: 99%

“…Although spirometry is not the ideal tool and it does not give information about inflammation or pulmonary remodelling, and, according to some findings, in young children is not strongly correlated with their quality of life, it is still the standard examination performed on patients with CF in the facility where our study was conducted [ 11 , 33 , 41 ]. The 2-year study by Aquino et al showed that routine spirometry significantly improved lung function and was associated with better identification of pulmonary exacerbations [ 42 ].…”

Section: Discussionmentioning

confidence: 99%

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Polish Cystic Fibrosis Patients’ Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study

Humaj-Grysztar,

Rachel,

Bonior

2024

Healthcare

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Cystic fibrosis (CF) is a disease characterized by long-term and troublesome symptoms that affect the patient’s life. This study aimed to assess and compare the health-related quality of life (HRQoL) of Polish CF patients and identify factors influencing it. The study group consisted of 79 patients (6 to 42 years old), who filled in an age-appropriate Cystic Fibrosis Questionnaire-Revised. Medical data were collected from each patient’s medical records. The domains with the highest HRQoL median were eating problems (88.89), digestive symptoms (77.78) and physical functioning (75.00). The lowest-rated domain was social functioning (61.90). Age negatively correlated with eight domains, and most strongly with treatment burden (rho = −0.474). Physical functioning positively correlated with all spirometry parameters, and most strongly with FEV1% (rho = 0.588). Treatment burden, body image and respiratory symptoms were positively correlated with all spirometry parameters except PEF%. Present exacerbations reduced scores in almost all domains, and in the MANCOVA model they were a significant factor differentiating patients’ HRQoL. The univariate analysis of MANCOVA showed the significant effects of both health condition (F = 8.32, p = 0.005) and the COVID-19 pandemic (F = 5.89, p = 0.018) on social functioning domain, and of the place of residence on body image (F = 5.60, p = 0.21). A decreasing HRQoL with increasing age and during exacerbations indicates that it is important to focus on these aspects of patients’ lives and ensure they received the necessary support from their healthcare providers.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Polish Cystic Fibrosis Patients’ Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study

Humaj-Grysztar,

Rachel,

Bonior

2024

Healthcare

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“…Still, longitudinal trials have shown inconsistent results [50,51]. Studies comparing LCI and CFQ-R scores in children with CF have also been contradictory [52][53][54][55].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis

Walicka‐Serzysko¹,

Postek²,

Borawska-Kowalczyk³

et al. 2023

JCM

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Background: Properly evaluating respiratory system dysfunction is essential in children with cystic fibrosis (CF). This prospective study aimed to assess the course of early lung disease based on multiple breath nitrogen washout (MBNW), impulse oscillometry (IOS), and conventional techniques, such as spirometry and body plethysmography. Methods: Over a 2 year recruitment period, subjects with CF aged 7–18 performed pulmonary function tests (PFTs). Moreover, the nutritional and microbiological status, frequency of pulmonary exacerbations (PExs), and patients’ health-related quality of life (HRQoL) were assessed. Results: The mean age of the children (n = 69) was 14.09 ± 3.26 years; F/M 37/32. Spirometry-based diagnoses of normal lung function (forced expiratory volume in 1 s, FEV1 ≥ 90%pred), mild (FEV1 70–89%pred) and moderate (FEV1 40–69%pred) lung diseases were established in 34 (49.3%), 25 (36.2%), and 10 (14.5%) patients, respectively. An elevated lung clearance index (LCI > 6.98) was observed in 85% of the subjects with normal FEV1. The presence of Pseudomonas aeruginosa infection (n = 16) and the number of PExs treated with IV antibiotics were associated with significantly worse PFT results. Conclusions: MBNW and IOS are more helpful tools than conventional techniques in assessing early lung disease in CF. LCI is a more useful parameter for detecting functional abnormalities than FEV1 in school-age children.

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“…Better lung function in people with CF is strongly associated with improved quality of life and survival. The most often used pulmonary function measurement parameter is forced expiratory volume at 1 second percent predicted (FEV1pp) and this is also one of the indicators of CF care quality 4,5 …”

Section: Introductionmentioning

confidence: 99%

“…The most often used pulmonary function measurement parameter is forced expiratory volume at 1 second percent predicted (FEV1pp) and this is also one of the indicators of CF care quality. 4,5 Oral health status (OHS) is affected by CF. However, there is a lack of evidence for the relation between CF and dental caries.…”

Section: Introductionmentioning

confidence: 99%

Parent's report on oral health‐related quality of life of children with cystic fibrosis

Sisman

Peker

Gökdemir

et al. 2022

Pediatric Pulmonology

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Background: Health-related quality of life (HRQoL) scales are now widely used in children with cystic fibrosis (cwCF) which reflects the course of the disease. In this cross-sectional study, our primary aim was to compare the Pediatric Oral Health-Related Quality of Life (POQL) and Oral Health Score (OHS) between cwCF and healthy group. Our secondary aim was to evaluate the association between Pseudomonas aeruginosa (PA) colonization, pulmonary function test, OHS and POQL in cwCF. Methods:The study population (age ranging 6-14) included 55 cwCF followed at the Marmara University Division of Pediatric Pulmonology compared with 50 healthy peers. A survey consisted of general questions (age, sex, etc.) and the POQL instrument were filled by parents. The decayed, missing, and filled teeth for both primary (dft) and permanent dentition (DMFT) was detected according to WHO criteria. Data like current body mass index (BMI z score), colonization status with PA, predicted value for forced expiratory volume in 1 second (FEV1pp), and any hospitalizations during the previous year were obtained from their medical.Differences between the groups were evaluated using Chi-square and Mann-Whitney U test with a significance level set at 0.05.Results: There was no significant difference between PA-colonized cwCF and healthy controls in DMFT (p = 0.916). For all domains of POQL (emotional function, social function, role function), scores of cwCF were significantly better than healthy controls (p < 0.05). There were no statistically significant differences between all domains of POQL scores in PA colonized and non-PA colonized cwCF' POQL scores (p > 0.05). Conclusion:Although POQL scores of cwCF were encouraging, dental caries prevention and regular follow-ups should be taken into consideration.

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Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis

Cited by 4 publications

References 35 publications

Polish Cystic Fibrosis Patients’ Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study

Polish Cystic Fibrosis Patients’ Health-Related Quality of Life and Its Influencing Factors: A Cross-Sectional, Single-Centre Study

Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis

Parent's report on oral health‐related quality of life of children with cystic fibrosis

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