End of Life in Cystic Fibrosis
Cystic fibrosis (CF) is a rare genetic disease that is seldom mentioned in the context of palliative care, yet it profoundly affects the quality of life of patients and families. Currently, 30,000 people in the United States and 70,000 worldwide are living with CF.[1]
CF was once considered a fatal disease with a very short life expectancy, but advances in medical care have extended life for many patients diagnosed with CF such that it has become a chronic condition with a median survival of 40 years. There is still no cure for CF, however, so patients must be supported through advanced care planning and end-of-life decisions.
In a recent article, Price and Knotts[2] present a case study and discussion of three key components of nursing care for patients with CF at the end of life. The case patient is a 29-year-old, married woman with two young children. This tragic story is nevertheless very informative, and captures not only the medical and treatment advances in CF but also the role of the nurse in providing optimum care. It emphasizes many factors that are the foundation of palliative care: excellent symptom management, time for all family members to say goodbye, care for children, spiritual support, and inclusion of staff members who have had a long relationship with the patient and family.[3,4,5]
Communication, Comfort, and Closure
The case discussion addresses the three key components of care at end of life: communication, comfort, and closure. In this patient with terminal CF, these components addressed the following:
Communication: facilitating interdisciplinary communication and collaboration between nursing staff and other healthcare providers (primary care, palliative care, social workers, respiratory therapists, physical therapists, chaplains, and child-life services), and consistency of bedside nurse caregivers to facilitate clear communication between all staff
Comfort: pain and anxiety management, relief of dyspnea, airway clearance, ventilator management
Closure: patient's legacy, memories, saying goodbye to family members and others
Together, these three components serve as a useful framework for clinicians to apply to other patients and to other serious or life-threatening illnesses.
Of special note in this article is that the authors end the paper with a thoughtful discussion of the barriers encountered in this case and opportunities to improve care. These included a heavy workload for the nurse assigned to care for the patient on the day that withdrawal from ventilator support was planned, the family's request to "turn off the computer" at the bedside, and the emotional toll that the episode took on the staff. This case highlights the need for support of the bedside nurse caring for the patient and family.
Medscape Nurses © 2017 WebMD, LLC
Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.
Cite this: The Bedside Nurse's Role at End of Life - Medscape - Oct 20, 2017.
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