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Published on: February 14, 2022
Lung transplantation and end of life issues in cystic fibrosis
1Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London, UK. paulaurora@yahoo.co.uk
Insights
Lung transplantation for cystic fibrosis lung disease has poor outcomes and limited organ supply. Some children choose medical management and terminal care over this option.
Area of Science:
- Pulmonology
- Transplant Surgery
- Pediatric Care
Background:
- Lung transplantation is a treatment option for end-stage cystic fibrosis lung disease.
- Outcomes remain suboptimal, and donor organ availability is restricted.
- This influences treatment decisions for pediatric patients.
Purpose of the Study:
- To review the current landscape of lung transplantation for cystic fibrosis.
- To outline current referral guidelines and contraindications for lung transplantation.
- To discuss contemporary practices in terminal care for these patients.
Main Methods:
- Literature review of lung transplantation in cystic fibrosis.
- Analysis of current referral criteria and contraindications.
- Examination of terminal care protocols.
Main Results:
- Lung transplantation for cystic fibrosis has historically yielded poor results.
- Limited organ availability is a significant barrier to transplantation.
- Alternative management, including terminal care, is a consideration for some patients.
Conclusions:
- Lung transplantation for cystic fibrosis remains challenging due to outcomes and organ scarcity.
- Careful consideration of referral guidelines and contraindications is essential.
- Terminal care represents a valid alternative pathway for select pediatric patients.
Abstract:
Lung transplantation has been available as a therapeutic option for patients with end-stage cystic fibrosis lung disease for over 15 years, but the outcome following transplantation remains poor, and the supply of organs limited. For this reason some children opt to continue with medical treatment followed by terminal care rather than undergo transplantation. This article summarizes the current status of lung transplantation, including current referral guidelines and contraindications and also addresses current practice in terminal care.
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