End-of-life care in adults with cystic fibrosis
Jennifer A M Philip1, Michelle Gold, Sharon Sutherland
1Palliative Care Services, Alfred Hospital, Prahran, Victoria, Australia. jennifer.philip@alfred.org.au
Insights
Palliative care for cystic fibrosis (CF) patients faces challenges, as life-prolonging treatments continue until death. Effective end-of-life care models are needed that support CF patients awaiting lung transplantation.
Area of Science:
- Medical Research
- Palliative Care
- Pulmonology
Background:
- Cystic fibrosis (CF) is a life-limiting genetic disease causing progressive lung damage.
- Lung transplantation is a potential treatment but not always feasible.
- Palliative care for young CF patients awaiting transplantation requires specific guidance.
Purpose of the Study:
- To inform the development of an appropriate palliative care model for CF patients.
- To analyze care aspects for CF patients who died without lung transplantation.
Main Methods:
- Retrospective medical record review of CF patients who died within a 5-year period without lung transplantation.
- Quantification of preventative, therapeutic, and palliative care aspects.
Main Results:
- All 20 studied patients died in hospital.
- Do-not-resuscitate orders increased from 15% over a week before death to 90% at death (median 2 days prior).
- Opioid use increased from 5% at one week prior to death to 85% in the last 24 hours (median 36 hours prior); 85% received IV antibiotics and 90% received assisted ventilation in the last 24 hours.
Conclusions:
- Effective palliative care for CF patients presents challenges due to continued life-prolonging treatments.
- There is an urgent need for palliative care approaches that can coexist with potential lung transplantation options.
- Integrating palliative care with end-of-life management for CF patients requires further examination.
Background:
Cystic fibrosis (CF) is a life-limiting congenital disease, with most patients dying at a young age of progressive lung disease. Lung transplantation offers hope for many but may not occur. There is little to guide the provision of palliative care to this young population who maintain hope in the possibility of lung transplantation.
Methods:
To inform the development of an appropriate model of palliative care, a medical record review of CF patients dying within a 5-year period without lung transplantation was undertaken. The aspects of care that were preventative, therapeutic, or palliative were quantified.
Results:
Of the 20 records studied, all died in hospital. Only 15% of patients had a do-not-resuscitate order agreed to more than 1 week prior to death, increasing to 90% at the time of death (median of 2 days before death). Opioids were prescribed for 1 patient (5%) at 1 week prior to death, increasing to 85% of patients in the last 24 hours of life (median of 36 hours before death). During the last 24 hours of life, intravenous antibiotics continued in 85%, and assisted ventilation in 90% of subjects.
Conclusion:
We conclude that the circumstances surrounding the death of patients with CF holds challenges for their effective palliative care. CF patients continue life prolonging and preventative treatments until the last hours of life. There is an urgent need to examine palliative care approaches that may usefully coexist with maintaining transplantation options in the end-of-life care of this population.
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