Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis

2020 ◽  
Author(s):  
Emily DiMango ◽  
Daniel B. Spielman ◽  
Jonathan Overdevest ◽  
Claire Keating ◽  
Sarah Fracasso Francis ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Highly Effective

scholarly journals 94: Assessing symptom burden and quality of life in patients with cystic fibrosis in the era of highly effective modulator therapy

2021 ◽  
Vol 20 ◽  
pp. S47-S48
Author(s):  
A. Perez ◽  
K. Bischoff ◽  
D. Zhang ◽  
D. Dawson ◽  
M. Israni ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Symptom Burden ◽  
Highly Effective

scholarly journals Management of Individual Patient Expectations When Starting with Highly Effective CFTR Modulators

2021 ◽  
Vol 11 (8) ◽  
pp. 811
Author(s):  
Bente L. Aalbers ◽  
Inez Bronsveld ◽  
Regina W. Hofland ◽  
Harry G. M. Heijerman
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Lung Damage ◽  
Cell Models ◽  
Highly Effective ◽  
Near Future ◽  
Cftr Modulators ◽  
Sinonasal Symptoms ◽  
Severe Lung

Highly effective CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ELE/TEZ/IVA will become available for an increasing number of people with cystic fibrosis (pwCF) in the near future. Before the start of this therapy, many questions may arise concerning the expected effects. We assembled the currently available data from the literature about ELE/TEZ/IVA that focused on commonly asked questions from patients. Overall, the literature so far presents a very hopeful prospect of effects, not only on lung function, but also on nutritional status, sinonasal symptoms and quality of life. The effects in patients with pwCF with severe lung damage are also favorable. Treatment is generally well tolerated. In some cases, patient-derived cell models can help in predicting the effects for individual patients.

Psychosocial well-being and quality of life in siblings of children with congenital heart disease: A systematic review

2021 ◽  
pp. 136749352110129
Author(s):  
Alice S Schamong ◽  
Hannah Liebermann-Jordanidis ◽  
Konrad Brockmeier ◽  
Elisabeth Sticker ◽  
Elke Kalbe
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Associated Factors ◽  
Congenital Heart ◽  
Negative Impact ◽  
Well Being

Congenital heart disease (CHD) is a major global health problem. Until recently, the siblings of this group did not receive much attention. This review, conducted from November 2019 to October 2020, aims to summarize knowledge about psychosocial well-being and quality of life (QoL), associated factors, and interventions for siblings of children with CHD. Systematic searches were conducted in PubMed, PsycINFO, PsycARTICLES, Web of Science via EBSCOhost, and CENTRAL. Twelve articles were included. Results showed that psychosocial well-being was impaired in 14% to 40% of siblings. Negative impact of illness was highest for CHD siblings compared to siblings of children with cancer, cystic fibrosis, or diabetes. QoL was impaired in up to one-third. Siblings of children with CHD and cancer rated their QoL lower than those of siblings of children with cystic fibrosis or type-1 diabetes. Associated factors were sibling age, gender, socioeconomic status, miscarriage, previous sibling death, visibility of illness, and severity of condition. Only one of two interventions focused on siblings of CHD children. Although data are scarce and inhomogeneous, it indicates that siblings of CHD children suffer from lower psychosocial well-being and QoL than siblings of children with other chronic conditions. Interventions to improve their situation should be developed.

scholarly journals Quality of life in cystic fibrosis

Thorax ◽  
1998 ◽  
Vol 53 (9) ◽  
pp. 721-722
Author(s):  
D J FRENCH
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis

scholarly journals Quality of life in Hungarian patients with cystic fibrosis

2013 ◽  
Vol 154 (20) ◽  
pp. 784-791 ◽  
Author(s):  
Réka Bodnár ◽  
Klára Holics ◽  
Rita Ujhelyi ◽  
László Kádár ◽  
Lajos Kovács ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Self Report ◽  
Clinical Severity ◽  
Proxy Report ◽  
Parent Proxy Report ◽  
Multisystemic Disease ◽  
Cystic Fibrosis Questionnaire ◽  
Parent Proxy

Introduction: Cystic fibrosis is a progressive multisystemic disease which affects the quality of life of patients. Aim: The aim of the study was to evaluate quality of life in Hungarian patients with cystic fibrosis. Methods: Validated Hungarian translation of The Cystic Fibrosis Questionnaire – Revised was used to measure quality of life. Clinical severity was determined on the basis of Shwachman–Kulczycki score. Lung function was measured using spirometry. Results: 59 patients were included from five centres in Hungary. The relationships between 8–13 year-old children self-report and parent proxy report was 0.77 (p<0.001) in physical functioning, 0.07 (p<0.001) in emotional functioning, 0.51 (p<0.001) in eating, 0.21 (p<0.001) in treatment burden, 0.54 (p<0.001) in body image, 0.49 (p<0.001) in respiratory symptoms and 0.40 (p<0.001) in digestive symptoms domains. Conclusions: In contrast to physical domains weak correlations were observed between answers obtained from children and their parents in psychosocial domains. The perception of both patients and their parents should be assessed when measuring quality of life in paediatric patients with cystic fibrosis. Orv. Hetil., 2013, 154, 784–791.

scholarly journals 357 Factors associated with changes in health-related quality of life in children with cystic fibrosis during one-year follow-up

2017 ◽  
Vol 16 ◽  
pp. S154
Author(s):  
M. Van Horck ◽  
B. Winkens ◽  
G. Wesseling ◽  
K. de Winter-de Groot ◽  
I. De Vreede ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Health Related Quality ◽  
Factors Associated ◽  
Related Quality ◽  
Health Related ◽  
One Year
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