Exercise performance and quality of life in children with cystic fibrosis and mildly impaired lung function: relation with antibiotic treatments and hospitalization

Vandekerckhove, Kristof; Keyzer, M.; Cornette, J.; Coomans, Ilse; Pyl, F.; Baets, Frans De; Schelstraete, Petra; Haerynck, Filomeen; Wolf, Daniël De; Daele, S. Van; Boone, Jan

doi:10.1007/s00431-017-3024-7

Cited by 15 publications

(16 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The value of 6MWT in CF though is mixed. In patients with mild to moderate airflow obstruction, the 6MWT distance was found not to be different from that of aged matched healthy controls and tests of maximal exercise capacity are recommended to detect the presence of early disease [11, 12]. However, in more severe CF disease, such as our population, the 6MWT has been used as a prognostic indicator for transplantation.…”

Section: Discussionmentioning

confidence: 95%

Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

et al. 2019

View full text Add to dashboard Cite

Background Treatment of patients with Cystic Fibrosis homozygous for the Phe508del gene, with Lumacaftor / Ivacaftor (LUM/IVA) improves outcomes in patients with FEV1 > 40% predicted. We set out to observe the most sensitive clinical measure that would change with treatment in terms of exercise capacity or lung function in adults with severe lung disease as defined by an FEV1 < 40% predicted when clinically stable. Methods 10 adults homozygous for the Phe508del received LUM/IVA. We assessed; six minute walk test (6MWT), spirometry, gas transfer (DLCO), plethysmography, and nitrogen multiple breath washout (MBW) at baseline, 4, 12, 24 and 52 weeks. Comparison was made with 10 matched historical controls that had been observed over 12 months. Results There was a significant improvement in 6MWT by 4 weeks of treatment; with a mean increase of 78 m (SD 62.3) and this increased to 118.1 m (SD 80.9) (ANOVA p = 0.006) by 52 weeks. Significant improvements were also seen in the resting heart rate and the oxygen saturation (SaO2) after 6 min walking. A significant improvement was not seen in FEV1 though until 24 weeks, though this was maintained at 52 weeks (ANOVA, p = 0.0004). There were no significant differences seen in the MBW or DLCO. After 12 months treatment with LUM/IVA, in comparison to historical controls; the 6MWT increased by 118 m (SD 80.9), but fell in the controls − 61.3 m (SD 31.1). FEV1; LUM/IVA led to an increase of 0.398 L/min, compared to a fall in the controls − 0.18 (SD 0.2). Conclusion In adults homozygous for Phe508del with severe disease, treatment with LUM/IVA results in a clinically significant improvement in 6MWT that was evident at 4 weeks and maintained at 52 weeks. Improvement in exercise tolerance is an important outcome to consider in those with more severe airways disease. Trial registration This was an observational trial conducted on individuals who became eligible to receive LUM/IVA. All investigations were carried out as part of routine clinical care. The trial was registered in retrospect on the 13/5/2019 on the Australian New Zealand Clinical Trials registry; ACTRN12619000708156 .

show abstract

Section: Discussionmentioning

confidence: 95%

Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

et al. 2019

View full text Add to dashboard Cite

show abstract

“…concluded that physical fitness in children is associated with a lower risk of hospitalization [30]. Vandekerckhove et al [5] correlated last year hospitalization of children with CF with physical health, school functioning and total HRQoL score. Hospitalization rates in this population reflect pulmonary exacerbations, morbidity, mortality and are associated with worse disease management [31,32].…”

Section: Discussionmentioning

confidence: 99%

“…Clinical Score. limiting activities of daily living, decreasing healthrelated quality of life (HRQoL), and life expectancy [5].…”

Section: Introductionmentioning

confidence: 99%

Physical activity and quality of life of children and adolescents with cystic fibrosis: a cross-sectional study

et al. 2020

View full text Add to dashboard Cite

Introduction: Regular physical activity (PA) is part of outpatient care offered to most people with cystic fibrosis (CF). Objective: To determine the frequency of the self-reported PA and, secondarily evaluate associations between PA with clinical variables and health-related quality of life (HRQoL) in pediatric and adolescent CF subjects. Method: In this cross-sectional study, subjects between 6 and 17 years of age with a confirmed diagnosis of CF were recruited in the outpatient clinic. The subjects answered questions about the practices of PA, HRQoL questionnaire and clinical variables were collected. Subjects were classified into two groups according to weekly PA practice: PA performed ≥ 3 times/week and performed PA ≤ 2 times/week. Results: 66 subjects completed the study, 72.7% (n=48) had PA ≥ 3 times/week and 27.2% (n=18) had PA ≤ 2 times/week. Only twelve children (18,2%) reached the recommendations of the World Health Organization (WHO) to practice moderate to vigorous physical activities daily. The mean age of the subjects evaluated was 12.3 ± 3.2 years, forced expiratory volume in one second 90 ± 24.1% and forced vital capacity 95 ± 20.4%. The group that performed PA ≥ 3 times/week had a better clinical score (p=0.033), a lower number of hospitalizations in the year (p=0.002), a lower number of days hospitalized in the last year (p=0.020) and better score for the physical (p=0.003) and emotion (p=0.048) domains in HRQoL questionnaire. Conclusion: This study concluded that most subjects did not reach the WHO recommendations for the practice of PA. However, individuals who practice PA at least 3 times/week have better HRQoL, clinical score and fewer hospitalizations.

show abstract

“…Segundo os pais/cuidadores, a maioria das crianças e adolescentes sentem cansaço na prática dos esportes em algum momento e, também foi percebido que a dispneia está presente. Vários estudos têm demonstrado que a tolerância ao exercício de crianças/adolescentes está reduzida quando comparadas com crianças sem a doença e que declina com o aumento da idade (STEVENS;OADES;WILLIAMS, 2015;VANDEKERCKHOVE et al, 2017;WEIR et al, 2017).…”

Section: Discussionunclassified

Qualidade de vida de crianças/adolescentes com fibrose cística segundo a percepção de seus pais/cuidadores

Silva

Fernandes

Venâncio

et al. 2019

R. bras. Qual. Vida

View full text Add to dashboard Cite

OBJETIVO: Avaliar a qualidade de vida (QV) de crianças/adolescentes com fibrose cística (FC), segundo a percepção dos pais/cuidadores.MÉTODOS: Estudo do tipo observacional transversal de caráter quantitativo, realizado na Associação de Pais e Amigos dos Excepcionais (APAE) com pais/cuidadores de crianças/adolescentes com FC. Participaram 29 pais/cuidadores, com idade entre 21-59 anos, de ambos os sexos. Foi respondido a versão Proxy do questionário DISABKIDS – Módulo Fibrose cística para pais/cuidadores, que avalia a QV das crianças segundo a percepção deles. Para verificar a correlação entre AS variáveis sociodemográficas e a QV foi utilizado a coeficiente de correlação de Spearman.RESULTADOS: No domínio impacto, 17 (58,5%) pais/cuidadores percebem que suas crianças, em algum momento, sentiram-se cansadas com a prática de esportes e 13 (44,8%) sentem falta de ar. No domínio tratamento da doença, 13 (44,8%) pais/cuidadores relataram que os filhos se sentem incomodados com o tempo gasto no tratamento; o mesmo número de pais/cuidadores relataram em algum momento percebem seus filhos incomodados em relação ao tratamento fisioterapêutico. Houve correlação negativa e significativa do impacto da doença com a idade das crianças/adolescentes (r=-0,52, p=0,004) e com o número de filhos (r=-0,55, p=0,002).CONCLUSÕES: Os pais/cuidadores perceberam que os aspectos físicos relacionados ao esporte e à dispneia são os fatores do impacto da doença que mais prejudicam seus filhos. Além disso, os maiores incômodos, segundo os pais/cuidadores, estão no tempo gasto com o tratamento clínico e com a fisioterapia. Foi mostrado que quanto mais nova a criança menor o impacto na QV e que quanto maior o número de filhos pior é a QV no que diz respeito ao impacto da doença.

show abstract

Exercise performance and quality of life in children with cystic fibrosis and mildly impaired lung function: relation with antibiotic treatments and hospitalization

Cited by 15 publications

References 31 publications

Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

Physical activity and quality of life of children and adolescents with cystic fibrosis: a cross-sectional study

Qualidade de vida de crianças/adolescentes com fibrose cística segundo a percepção de seus pais/cuidadores

Contact Info

Product

Resources

About