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End of Life Care for Patients with Cystic Fibrosis

End of life planning can be difficult for the one dying and for their loved ones. However, by taking the steps to plan for what one wants, the person who is dying gets to shape their ending. Few in the cystic fibrosis (CF) world faced end-of-life issues more eloquently than CF activist and patient Claire Wineland. During a TEDx talk in which she discussed facing her own death, she said:

You can suffer and be okay. You can suffer and still make something. The quality of your life isn’t determined by whether you’re healthy or sick or rich or poor, not at all. It’s determined by what you make out of your experience as a human being.

How end of life planning and care is different with CF

Usually, end of life planning includes deciding where the person wants to spend their final days (for example, at home versus in the hospital), which treatments they want to receive, and what type of palliative care and/or hospice care they want to receive. Palliative care provides relief from the symptoms of illness and can be a part of care at any stage of the disease. Hospice care includes palliative care and focuses on the quality of life at the end of life.1,2

However, end-stage cystic fibrosis is more unpredictable than other terminal illnesses, such as cancer. With cancer, active treatment stops because treatments have no benefit to their health or quality of life. Palliative care and symptom management become the focus for cancer patients. With CF, palliative care providers work alongside the regular treatment team, playing an active role in caring for people with CF, sometimes for years. With end-stage CF, active treatment continues in conjunction with palliative care because:

  • People with CF who are very sick sometimes recover
  • Active treatment helps ease difficult end-stage symptoms
  • Active treatment is necessary if the person is still a candidate for lung transplant3

By making these decisions ahead of time, the person who is dying can provide helpful information for their families and friends, as well as ensure their individual values are reflected in these decisions.1,3

Components of end of life planning

End of life planning encompasses more than just medical care. In addition to making decisions about health care, end of life planning can include legal and financial decision-making, organ donation, funeral and memorial planning, and determining what legacy someone wants to leave for others, or how they want to be remembered.

What are advance directives?

Advance directives are written instructions on the type of care that is wanted if someone is critically ill or dying. When someone is too ill or hurt to express their wishes, an advance directive provides a legal document that specifies the person’s wishes. Also called a living will, it often includes instructions on the use of machines that may prolong life (such as dialysis), the use of resuscitation if breathing or heartbeat stops, tube feeding, and/or organ or tissue donation.

Another document that can be helpful is a durable power of attorney for health care, which chooses a person to be a health care proxy, another person to make decisions if the patient cannot.1,2

Questions to consider for end of life planning

Conversations about death and dying may be difficult but understanding the patient’s wishes and priorities allow the patient to have control over choices made at the end of their life. Some questions that can help shape a discussion include:

  • What is your understanding of the situation and its potential outcomes?
  • What are your biggest fears and concerns? What are your hopes?
  • What goals are most important to you?
  • What trade-offs are you willing to make, and what ones are you not?
  • What is the course of action that best serves this understanding?4

Priorities at end of life

For those who are sick and nearing the end of life, the priorities go beyond just being safe and living longer. The chance to shape one’s story is essential to sustaining meaning in life.

  • To share memories
  • To pass on wisdom and keepsakes
  • To settle relationships
  • To establish their legacies
  • To make peace religiously or spiritually
  • To ensure that those they leave behind will be okay4
Written by: Jessica Johns Pool | Last reviewed: September 2019
  1. Medline Plus. National Institutes of Health. End of Life Issues. Available at: https://medlineplus.gov/endoflifeissues.html. Accessed 6/13/2019.
  2. National Institute on Aging. Providing Care and Comfort at the End of Life. Available at: https://www.nia.nih.gov/health/publication/end-life-helping-comfort-and-care/introduction. Accessed 6/13/2019.
  3. Cystic Fibrosis Trust. Planning for end of life. Available at: https://www.cysticfibrosis.org.uk/~/media/documents/life-with-cf/information-and-support/planning-for-end-of-life.ashx?la=en. Accessed 6/13/2019.
  4. Gawande, Atul. Being Mortal: Medicine and What Matters in the End. New York: Metropolitan Books, Henry Holt and Company, 2014.