End of life care

Unfortunately despite optimal medical care CF remains a life limiting condition. Progression of the disease to a terminal phase is usually heralded by increased frequency and severity of respiratory exacerbations, oxygen dependence, and deteriorating lung function. The variable nature of the condition makes it difficult to predict when this phase will be reached and how long it will last (Kerem, Reisman et al. 1992).

The World Health Organisation (World Health Organisation 2003) define palliative care as –

“Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual.”

It is of vital importance to acknowledge impending death and the recognition of dying as a normal process bringing life to natural end.

An essential part of quality end of life care is advanced care planning, a process of communication between patients, health care providers and families (Martin, Emanuel et al. 2000, Conway, Balfour-Lynn et al. 2014). Every patient deserves a pain free, dignified and peaceful death. Providing sensitive and sympathetic care for the dying patient is one of the most significant things that we can do for them. The situation must be realistically discussed with the patient and family with the provision of clear and honest information (Braithwaite, Philip et al. 2011). Discussions regarding end of life care may occur with a variety of different professionals over a period of time. Patients have expressed that when discussing end of life care they wanted to do so with the team member with whom they had the closest relationship, regardless of the person’s discipline (Braithwaite, Philip et al. 2011).

They must be reassured that adequate medication will be given to alleviate symptoms. The move from “cure” to “care” must be communicated to all members of the CF team so that conflicting messages are not given. A policy of balancing the risk and benefit of interventions and investigations must be adhered to at all times and the patient’s wishes must be fully respected. Changing the emphasis of care does not imply withdrawal of treatment, but allows us to focus on providing symptom control.

Integration of palliative care into CF care is essential as some patients who die from CF are on a lung transplant waiting list. These patients are extremely ill which is associated with a significant symptom burden and the risk of death, but the hope of transplant would dramatically alter the patient’s prognosis. During this time active aggressive therapy may need to be combined with palliative therapy, therefore it is important that there are no barriers between the CF and palliative care teams delivering high quality care (Bourke and Peel 2014).

The main symptoms requiring medical management in the terminal phase of the disease may include breathlessness, difficulty clearing sputum, pain and anxiety (Bourke, Doe et al. 2009). The use of monitoring equipment, which may be very intrusive, can be reviewed and following discussion with patients and carers may be withdrawn.

Oxygen humidification, nebulised bronchodilators, regular analgesia and continuous antibiotics can help relief symptoms of breathlessness. Opiates such as morphine oxycodone and diamorphine can be effective in managing symptoms of breathlessness, cough or pain. These drugs may be administrated via oral, sub-cutaneous or intra-venous routes depending on the nature and severity of the patients symptoms. Nebulised opiates such as morphine or fentanyl have been shown to be may alleviate distressing breathlessness (Cohen and Dawson 2002, Graff, Stark et al. 2004).

The use of positive pressure non-invasive ventilation (NIV) can be off benefit in some situations for patients with breathlessness (Nava et al., 2013), however the relief of breathlessness came at a cost in terms of discomfort from the mask. Prior to initiation of NIV therapy, it is important to consideration to the possibility of discontinuation / withdrawal of NIV at some point in the future (Association for Palliative Medicine of Great Britain and Ireland, 2015).

The use of positive pressure non-invasive ventilation (NIV) can be off benefit in some situations for patients with breathlessness. Nava et al demonstrated that the use of NIV for palliation of breathlessness in patients with solid tumours was more effective than oxygen alone in terms of relief of breathlessness and need for symptomatic relief with morphine (Nava et al., 2013). The relief of breathlessness came at a cost in terms of discomfort from the mask, with a discontinuation rate of NIV of 11%. The concept that NIV can improve the sensation of breathlessness, but needs to be balanced against the discomfort of the mask was also found in the 3CPO trial examining the use of NIV versus oxygen in acute pulmonary oedema (Gray et al., 2008). The use of NIV in patients with motor neurone disease is associated with a survival benefit, as well as improvements in dyspnoea (Bourke et al., 2006). Prior to initiation of therapy, it is important to consideration to the possibility of discontinuation / withdrawal of NIV at some point in the future(Association for Palliative Medicine of Great Britain and Ireland, 2015).

Short acting benzopdiazepines such as sub-lingual lorazepam or sub-cutaneous midazolam can help with symptoms of anxiety or agitation. Psychological strategies such as CBT can help with symptoms of anxiety for patients receiving end-of-life care.

Even when formal physiotherapy may not be tolerated or causes distress, it is important that treatment in the terminal stages is not withdrawn. Massage and appropriate positioning of the patient may help to alleviate symptoms of sputum retention.

Ideally terminal care should be delivered in the preferred place of care for the patient and their family, which may be hospital, hospice, or home. It is essential that the whatever option is chosen that there is effective cooperation between the CF team and palliative care team (Sands, Repetto et al. 2011). For patients wishing to receive terminal care at home involvement of GP, district nurses, CFNS, social worker and community palliative care teams is essential. For some patients the familiarity of the CF ward and staff offers continuity of both carers and environment. It is important to encourage an open environment so that the patient does not feel isolated by people trying to avoid what is happening.

Terminal care does not end when the patient dies, as psychological and spiritual support needs to continue to the bereaved families and friends. This may be provided by individual or group counselling provided by the CF Centre, the family doctor or by other external bodies (Sands, Repetto et al. 2011). Home visits by the CFNS and social worker can be continued for an indefinite time period.

In summary, the management of the terminally ill patient should emphasise “care” and a holistic team approach. As observed by Ambroise Pare, “to heal sometimes, to relieve often and to console always”.


Bourke, S. J., S. J. Doe, A. D. Gascoigne, K. Heslop, M. Fields, D. Reynolds and K. Mannix (2009). “An integrated model of provision of palliative care to patients with cystic fibrosis.” Palliat Med 23(6): 512-517.

Bourke, S. J. and E. T. Peel (2014). “Palliative care of chronic progressive lung disease.” Clin Med 14(1): 79-82.

Braithwaite, M., J. Philip, H. Tranberg, F. Finlayson, M. Gold, T. Kotsimbos and J. Wilson (2011). “End of life care in CF: patients, families and staff experiences and unmet needs.” J Cyst Fibros 10(4): 253-257.

Cohen, S. P. and T. C. Dawson (2002). “Nebulized morphine as a treatment for dyspnea in a child with cystic fibrosis.” Pediatrics 110(3): e38.

Conway, S., I. M. Balfour-Lynn, K. De Rijcke, P. Drevinek, J. Foweraker, T. Havermans, H. Heijerman, L. Lannefors, A. Lindblad, M. Macek, S. Madge, M. Moran, L. Morrison, A. Morton, J. Noordhoek, D. Sands, A. Vertommen and D. Peckham (2014). “European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre.” J Cyst Fibros 13S1: S3-S22.

Graff, G. R., J. M. Stark and R. Grueber (2004). “Nebulized fentanyl for palliation of dyspnea in a cystic fibrosis patient.” Respiration 71(6): 646-649.

Kerem, E., J. Reisman, M. Corey, G. J. Canny and H. Levison (1992). “Prediction of mortality in patients with cystic fibrosis.” N Engl J Med 326(18): 1187-1191.

Martin, D. K., L. L. Emanuel and P. A. Singer (2000). “Planning for the end of life.” Lancet 356(9242): 1672-1676.

Sands, D., T. Repetto, L. J. Dupont, A. Korzeniewska-Eksterowicz, P. Catastini and S. Madge (2011). “End of life care for patients with cystic fibrosis.” J Cyst Fibros 10 Suppl 2: S37-44.

World Health Organisation. (2003). ” WHO Definition of Palliative Care.”   Retrieved 19/05/2014, from http://www.who.int/cancer/palliative/definition/en/.

Nava, S., Ferrer, M., Esquinas, A., Scala, R., Groff, P., Cosentini, R., Guido, D., Lin, C. H., Cuomo, A. M. & Grassi, M. 2013. Palliative use of non-invasive ventilation in end-of-life patients with solid tumours: a randomised feasibility trial. Lancet Oncol, 14, 219-27.

Association for Palliative Medicine of Great Britain and Ireland 2015. Withdrawal of Assisted Ventilation at the Request of a Patient with Motor Neurone Disease – Guidance for Professionals.