Humanities
Skip to main content Skip to main navigation menu Skip to site footer
  • Register
  • Login
  • Menu
  • Home
  • Current
  • Archives
  • Announcements
  • About
    • About the Journal
    • Submissions
    • Editorial Team
    • Privacy Statement
    • Contact
  • Register
  • Login

Journal of Education, Health and Sport

Cystic fibrosis - Ways to improve the quality of life of patients
  • Home
  • /
  • Cystic fibrosis - Ways to improve the quality of life of patients
  1. Home /
  2. Archives /
  3. Vol. 20 No. 1 (2023) /
  4. Short Articles

Cystic fibrosis - Ways to improve the quality of life of patients

Authors

  • Kamila Świercz Wojewódzki Szpital im. Św. Ojca Pio w Przemyślu https://orcid.org/0000-0003-0783-270X
  • Magdalena Majcher 1 Wojskowy Szpital Kliniczny w Lublinie https://orcid.org/0000-0003-3998-090X
  • Dominika Górska 1 Wojskowy Szpital Kliniczny z Polikliniką w Lublinie https://orcid.org/0000-0002-2444-4891
  • Monika Majcher Wojewódzki Szpital Specjalistyczny im. Stefana Kardynała Wyszyńskiego w Lublinie https://orcid.org/0000-0002-4924-9048
  • Aleksandra Karwańska 5 Wojskowy Szpital Kliniczny z Polikliniką w Krakowie https://orcid.org/0000-0001-6917-6985
  • Agata Pikulicka SZPITAL SOLEC w Warszawie https://orcid.org/0000-0003-1693-8127
  • Małgorzata Sierpień 1 Wojskowy Szpital Kliniczny z Polikliniką SPZOZ w Lublinie https://orcid.org/0000-0002-0119-2775
  • Piotr Brzychczy Szpital im J.Dietla w Krakowie https://orcid.org/0000-0002-9607-9942
  • Aleksandra Kulbat V Wojskowy Szpital Kliniczny z Polikliniką w Krakowie https://orcid.org/0000-0002-3981-4242
  • Mateusz Kulbat V Wojskowy Szpital Kliniczny z Polikliniką w Krakowie https://orcid.org/0000-0003-3421-5512

DOI:

https://doi.org/10.12775/JEHS.2023.20.01.001

Keywords

cystis fibrosis, treatment, health education, life comfort, quality of life

Abstract

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.

Downloads

  • PDF

Published

2023-04-11

How to Cite

1.
ŚWIERCZ, Kamila, MAJCHER, Magdalena, GÓRSKA, Dominika, MAJCHER, Monika, KARWAŃSKA, Aleksandra, PIKULICKA, Agata, SIERPIEŃ, Małgorzata, BRZYCHCZY, Piotr, KULBAT, Aleksandra and KULBAT, Mateusz. Cystic fibrosis - Ways to improve the quality of life of patients. Journal of Education, Health and Sport. Online. 11 April 2023. Vol. 20, no. 1, pp. 11-16. [Accessed 28 June 2025]. DOI 10.12775/JEHS.2023.20.01.001.
  • ISO 690
  • ACM
  • ACS
  • APA
  • ABNT
  • Chicago
  • Harvard
  • IEEE
  • MLA
  • Turabian
  • Vancouver
Download Citation
  • Endnote/Zotero/Mendeley (RIS)
  • BibTeX

Issue

Vol. 20 No. 1 (2023)

Section

Short Articles

License

Copyright (c) 2023 Kamila Świercz, Magdalena Majcher, Dominika Górska, Monika Majcher, Aleksandra Karwańska, Agata Pikulicka, Małgorzata Sierpień, Piotr Brzychczy, Aleksandra Kulbat, Mateusz Kulbat

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0

Stats

Number of views and downloads: 476
Number of citations: 0

Search

Search

Browse

  • Browse Author Index
  • Issue archive

User

User

Current Issue

  • Atom logo
  • RSS2 logo
  • RSS1 logo

Information

  • For Readers
  • For Authors
  • For Librarians

Newsletter

Subscribe Unsubscribe

Tags

Search using one of provided tags:

cystis fibrosis, treatment, health education, life comfort, quality of life
Up

Akademicka Platforma Czasopism

Najlepsze czasopisma naukowe i akademickie w jednym miejscu

apcz.umk.pl

Partners

  • Akademia Ignatianum w Krakowie
  • Akademickie Towarzystwo Andragogiczne
  • Fundacja Copernicus na rzecz Rozwoju Badań Naukowych
  • Instytut Historii im. Tadeusza Manteuffla Polskiej Akademii Nauk
  • Instytut Kultur Śródziemnomorskich i Orientalnych PAN
  • Instytut Tomistyczny
  • Karmelitański Instytut Duchowości w Krakowie
  • Ministerstwo Kultury i Dziedzictwa Narodowego
  • Państwowa Akademia Nauk Stosowanych w Krośnie
  • Państwowa Akademia Nauk Stosowanych we Włocławku
  • Państwowa Wyższa Szkoła Zawodowa im. Stanisława Pigonia w Krośnie
  • Polska Fundacja Przemysłu Kosmicznego
  • Polskie Towarzystwo Ekonomiczne
  • Polskie Towarzystwo Ludoznawcze
  • Towarzystwo Miłośników Torunia
  • Towarzystwo Naukowe w Toruniu
  • Uniwersytet im. Adama Mickiewicza w Poznaniu
  • Uniwersytet Komisji Edukacji Narodowej w Krakowie
  • Uniwersytet Mikołaja Kopernika
  • Uniwersytet w Białymstoku
  • Uniwersytet Warszawski
  • Wojewódzka Biblioteka Publiczna - Książnica Kopernikańska
  • Wyższe Seminarium Duchowne w Pelplinie / Wydawnictwo Diecezjalne „Bernardinum" w Pelplinie

© 2021- Nicolaus Copernicus University Accessibility statement Shop