Young Adult with End-Stage Cystic Fibrosis: A Missed Opportunity for Implementing Timely Palliative Care

Written by Sigrid Ladores, Erin Currie, Laura Debiasi, Jessica L. Corcoran, & Marie Bakitas

doi: https://doi.org/10.13178/jnparr.2019.0901.0904

Abstract

Background: Cystic fibrosis is a chronic, progressive, life-limiting genetic disease that affects approximately 70,000 individuals worldwide. While advances in clinical care have improved overall patient outcomes, a cure is still not available. For those with advanced disease, palliative care may offer strategies to improve quality of life. Clinical goals and clinical problem(s): This case report describes a young adult female, KS (pseudonym), living with severe lung disease secondary to cystic fibrosis.

Assessment: KS was not eligible for lung transplantation due to various psychosocial, physical, and mental factors. Despite being offered palliative care on several occasions, she declined.

Implementation: Palliative care is part of the interdisciplinary approach to caring for patients with an incurable illness, and especially for KS who will likely never receive a lung transplant. It is clear that KS did not understand the purpose and benefits of palliative care.

Evaluation and Discussion: This is an unfortunate example of a missed opportunity for palliative care involvement before a medical crisis arises. Implications for improving education of patients, families and providers, and changing clinical practice to integrate specialized palliative care services early before a medical crisis arises are presented.

Keywords: cystic fibrosis, palliative care, transplantation