Lived experiences for individuals with cystic fibrosis who have undergone lung transplantation: a qualitative study

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Background

Cystic Fibrosis (CF) significantly affects the respiratory system, often requiring lung transplantation in advanced stages. This life-saving procedure presents substantial challenges and uncertainties. While there is existing research on support and information needs post-lung transplant from various perspectives, this study aims to specifically address the unique experiences and challenges faced by individuals with CF during both the pre-transplant and post-transplant periods.

Methods

Twenty-three lung-transplanted individuals with CF participated in this exploratory qualitative study. Data was collected through individual semi-structured interviews and analyzed using inductive content analysis.

Results

Participants faced physical and mental challenges, including fatigue, depression, and anxiety. The waiting period involved isolation, dependence on family, and guilt. Post-transplant, they dealt with relief but also severe pain and adjusted to a new identity. Participants highlighted the importance of taking immunosuppressive medications as prescribed, even though the regimen was complicated and these medications had side effects. Participants stressed the need for earlier and more open dialogue with healthcare professionals and better emotional preparation for the transplant process, including preparedness for pain and previously inadequately addressed concerns such as depression and anxiety.

Conclusions

This study underscores the significant physical and emotional challenges individuals with CF face during lung transplantation, highlighting the need for comprehensive, person-centered care. Psychological support, effective post-transplant pain management, and early palliative care may be beneficial approaches to improve the patient experience. Nurses can play a pivotal role in this process by ensuring clear communication, managing pain, educating patients on immunosuppressive regimens, and advocating for holistic care.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12912-025-02774-x.

Related collections

Most cited references 42

Record: found
Abstract: found
Article: not found

Consolidated criteria for reporting qualitative research (COREQ): a 32-item checklist for interviews and focus groups.

A Tong, P SAINSBURY, J. Craig (2007)

Qualitative research explores complex phenomena encountered by clinicians, health care providers, policy makers and consumers. Although partial checklists are available, no consolidated reporting framework exists for any type of qualitative design. To develop a checklist for explicit and comprehensive reporting of qualitative studies (in depth interviews and focus groups). We performed a comprehensive search in Cochrane and Campbell Protocols, Medline, CINAHL, systematic reviews of qualitative studies, author or reviewer guidelines of major medical journals and reference lists of relevant publications for existing checklists used to assess qualitative studies. Seventy-six items from 22 checklists were compiled into a comprehensive list. All items were grouped into three domains: (i) research team and reflexivity, (ii) study design and (iii) data analysis and reporting. Duplicate items and those that were ambiguous, too broadly defined and impractical to assess were removed. Items most frequently included in the checklists related to sampling method, setting for data collection, method of data collection, respondent validation of findings, method of recording data, description of the derivation of themes and inclusion of supporting quotations. We grouped all items into three domains: (i) research team and reflexivity, (ii) study design and (iii) data analysis and reporting. The criteria included in COREQ, a 32-item checklist, can help researchers to report important aspects of the research team, study methods, context of the study, findings, analysis and interpretations.

0 comments Cited 10543 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: found

Is Open Access

CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine

Miquéias Lopes-Pacheco (2020)

Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR mRNA and protein expression, function, stability or a combination of these. Although CF leads to multifaceted clinical manifestations, the respiratory disorder represents the major cause of morbidity and mortality of these patients. The life expectancy of CF patients has substantially lengthened due to early diagnosis and improvements in symptomatic therapeutic regimens. Quality of life remains nevertheless limited, as these individuals are subjected to considerable clinical, psychosocial and economic burdens. Since the discovery of the CFTR gene in 1989, tremendous efforts have been made to develop therapies acting more upstream on the pathogenesis cascade, thereby overcoming the underlying dysfunctions caused by CFTR mutations. In this line, the advances in cell-based high-throughput screenings have been facilitating the fast-tracking of CFTR modulators. These modulator drugs have the ability to enhance or even restore the functional expression of specific CF-causing mutations, and they have been classified into five main groups depending on their effects on CFTR mutations: potentiators, correctors, stabilizers, read-through agents, and amplifiers. To date, four CFTR modulators have reached the market, and these pharmaceutical therapies are transforming patients' lives with short- and long-term improvements in clinical outcomes. Such breakthroughs have paved the way for the development of novel CFTR modulators, which are currently under experimental and clinical investigations. Furthermore, recent insights into the CFTR structure will be useful for the rational design of next-generation modulator drugs. This review aims to provide a summary of recent developments in CFTR-directed therapeutics. Barriers and future directions are also discussed in order to optimize treatment adherence, identify feasible and sustainable solutions for equitable access to these therapies, and continue to expand the pipeline of novel modulators that may result in effective precision medicine for all individuals with CF.

0 comments Cited 202 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Cystic fibrosis

Michal Shteinberg, Iram J. Haq, Deepika Polineni … (2021)

Cystic fibrosis is a monogenic disease considered to affect at least 100 000 people worldwide. Mutations in CFTR, the gene encoding the epithelial ion channel that normally transports chloride and bicarbonate, lead to impaired mucus hydration and clearance. Classical cystic fibrosis is thus characterised by chronic pulmonary infection and inflammation, pancreatic exocrine insufficiency, male infertility, and might include several comorbidities such as cystic fibrosis-related diabetes or cystic fibrosis liver disease. This autosomal recessive disease is diagnosed in many regions following newborn screening, whereas in other regions, diagnosis is based on a group of recognised multiorgan clinical manifestations, raised sweat chloride concentrations, or CFTR mutations. Disease that is less easily diagnosed, and in some cases affecting only one organ, can be seen in the context of gene variants leading to residual protein function. Management strategies, including augmenting mucociliary clearance and aggressively treating infections, have gradually improved life expectancy for people with cystic fibrosis. However, restoration of CFTR function via new small molecule modulator drugs is transforming the disease for many patients. Clinical trial pipelines are actively exploring many other approaches, which will be increasingly needed as survival improves and as the population of adults with cystic fibrosis increases. Here, we present the current understanding of CFTR mutations, protein function, and disease pathophysiology, consider strengths and limitations of current management strategies, and look to the future of multidisciplinary care for those with cystic fibrosis.

0 comments Cited 196 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Ulrika Skogeland: ulrika.skogeland@ki.se

Journal

Journal ID (nlm-ta): BMC Nurs

Journal ID (iso-abbrev): BMC Nurs

Title: BMC Nursing

Publisher: BioMed Central (London )

ISSN (Electronic): 1472-6955

Publication date (Electronic): 3 February 2025

Publication date PMC-release: 3 February 2025

Publication date Collection: 2025

Volume: 24

Electronic Location Identifier: 127

Affiliations

[1 ]Department of Pediatrics, Cystic Fibrosis Centre, CLINTEC, Karolinska Institutet, Karolinska University Hospital, ( https://ror.org/056d84691) Stockholm, Hälsovägen, Stockholm, 141 86 Sweden

[2 ]Faculty of Nursing and Health Sciences, Nord University, ( https://ror.org/030mwrt98) Box 1490, Bodø, 8049 Norway

[3 ]Centre for Research Ethics & Bioethics, Department of Public Health and Caring Sciences, Uppsala University, ( https://ror.org/048a87296) Box 564, Uppsala, 751 22 Sweden

Article

Publisher ID: 2774

DOI: 10.1186/s12912-025-02774-x

PMC ID: 11792671

PubMed ID: 39901222

SO-VID: e08bea51-d550-4f5d-a525-5bb42d003d14

License:

Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.

History

Date received : 11 October 2024

Date accepted : 28 January 2025

Funding

Funded by: Region Stockholm and the National Association for Cystic Fibrosis donations funded this study.

Custom metadata

ScienceOpen disciplines: Nursing

Keywords: cystic fibrosis,lung transplantation,qualitative research,individual interviews,nurses,nursing

Data availability:

ScienceOpen disciplines: Nursing

Keywords: cystic fibrosis, lung transplantation, qualitative research, individual interviews, nurses, nursing

Lived experiences for individuals with cystic fibrosis who have undergone lung transplantation: a qualitative study

Read this article at

Abstract

Background

Methods

Results

Conclusions

Supplementary Information

Related collections

Journal of Health Sciences

Most cited references 42

Consolidated criteria for reporting qualitative research (COREQ): a 32-item checklist for interviews and focus groups.

CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine

Cystic fibrosis

Author and article information

Contributors

Journal

Affiliations

Article

History

Funding

Categories

Custom metadata

Comments

Comment on this article

Similar content 147

Most referenced authors 554