Cystic fibrosis - Ways to improve the quality of life of patients
DOI:
https://doi.org/10.12775/JEHS.2023.20.01.001Keywords
cystis fibrosis, treatment, health education, life comfort, quality of lifeAbstract
Introduction
Cystic fibrosis (CF) is a serious, multi-system disease. In the Caucasian population CF is the most common autosomal recessive disease, which happens for 1 person in 3,500 births [1]. In the course of CF, there is a dysfunction of many organs, mainly the respiratory and digestive systems. Symptomatic treatment of cystic fibrosis is very important. The change in daily eating habits, the use of proper breathing techniques and psychotherapy also have an impact on improving the quality of life of patients.
In the past patients died at a young age. The progress in the diagnosis and treatment of cystic fibrosis has meant that nowadays more and more people are reaching adulthood. In recent years, the median survival of patients has increased to 46.2 years. [2]
Purpose
The aim of this review is to present the current state of knowledge about ways to improve the quality and length of life of patients with CF.
Methods
The literature available in the Pubmed and Google Scholarship databases was reviewed using key phrases.
Results
Additional methods of treatment, such as: learning the correct breathing technique, oxygen therapy, physical exercise or proper nutrition, significantly improve the living conditions of patients.
References
De Boeck K. Cystic fibrosis in the year 2020: A disease with a new face. Acta Paediatr. 2020 May;109(5):893-899. doi: 10.1111/apa.15155. Epub 2020 Jan 22. PMID: 31899933.
Dickinson KM, Collaco JM. Cystic Fibrosis. Pediatr Rev. 2021 Feb;42(2):55-67. doi: 10.1542/pir.2019-0212. PMID: 33526571; PMCID: PMC8972143.
Kerem BS, Zielenski J, Markiewicz D, Bozon D, Gazit E, Yahav J, Kennedy D, Riordan JR, Collins FS, Rommens JM, et al. Identification of mutations in regions corresponding to the two putative nucleotide (ATP)-binding folds of the cystic fibrosis gene. Proc Natl Acad Sci U S A. 1990 Nov;87(21):8447-51. doi: 10.1073/pnas.87.21.8447. PMID: 2236053; PMCID: PMC54973.
ECFS patient registry annual data report [Internet]. 2017.
Available: https://www.ecfs.eu/sites/default/files/general-conte
nt-image s/worki ng-group s/ecfs-patie nt-regis try/ECFSPR_Repor
t2017_v1.3.pdf
Ratkiewicz M, Pastore M, McCoy KS, Thompson R, Hayes D Jr, Sheikh SI. Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis. World J Pediatr. 2017 Apr;13(2):129-135. doi: 10.1007/s12519-017-0015-8. Epub 2017 Feb 15. PMID: 28194692.
Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med. 2003 Oct 15;168(8):918-51. doi: 10.1164/rccm.200304-505SO. PMID: 14555458.
Cochrane Database. no chest physiotherapy for cystic fibrosis. Cochrane Database Syst Rev 2013; 9:CD001401.
These are updated Cochrane reviews, providing some rationale for the use of ACTs in CF.
Pryor JA, Tannenbaum E, Scott SF, Burgess J, Cramer D, Gyi K, Hodson ME. Beyond postural drainage and percussion: Airway clearance in people with cystic fibrosis. J Cyst Fibros. 2010 May;9(3):187-92. doi: 10.1016/j.jcf.2010.01.004. Epub 2010 Feb 12. PMID: 20153269.
McIlwaine M, Wong LT, Chilvers M, Davidson GF. Long-term comparative trial of two different physiotherapy techniques; postural drainage with percussion and autogenic drainage, in the treatment of cystic fibrosis. Pediatr Pulmonol. 2010 Nov;45(11):1064-9. doi: 10.1002/ppul.21247. PMID: 20836133.
Cox NS, Alison JA, Button BM, Wilson JW, Morton JM, Holland AE. Physical activity participation by adults with cystic fibrosis: An observational study. Respirology. 2016 Apr;21(3):511-8. doi: 10.1111/resp.12719. Epub 2015 Dec 30. PMID: 26715596.
Gaskin KJ. Nutritional care in children with cystic fibrosis: are our patients becoming better? Eur J Clin Nutr. 2013 May;67(5):558-64. doi: 10.1038/ejcn.2013.20. Epub 2013 Mar 6. PMID: 23462946.
Brownell JN, Bashaw H, Stallings VA. Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 2019 Dec;40(6):775-791. doi: 10.1055/s-0039-1696726. Epub 2019 Oct 28. PMID: 31659726.
Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988;41(6):583-91. doi: 10.1016/0895-4356(88)90063-7. PMID: 3260274.
Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013 Mar;162(3):530-535.e1. doi: 10.1016/j.jpeds.2012.08.040. Epub 2012 Oct 11. PMID: 23062247.
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Copyright (c) 2023 Kamila Świercz, Magdalena Majcher, Dominika Górska, Monika Majcher, Aleksandra Karwańska, Agata Pikulicka, Małgorzata Sierpień, Piotr Brzychczy, Aleksandra Kulbat, Mateusz Kulbat
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