Terminal Care

The Leeds Method of Management. April, 2008. Terminal care [online]. Leeds Regional Adult and Paediatric Cystic Fibrosis Units, St James's University Hospital, Leeds, UK. Available from http://www.cysticfibrosismedicine.com


It is a sad but true fact that even with optimal medical care Cystic Fibrosis (CF) remains a life limiting condition. The terminal phase is usually heralded by increased frequency and severity of respiratory exacerbations, oxygen dependence and poor 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. The majority of deaths occur in patients on the transplant waiting list due to lack of donor organs.

The World Health Organisation (WHO) produced a definition of palliative care in 1990 which was revised in 2003 and now reads:

“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 an early identification and impeccable assessment and treatment of pain and other problems; physical, psychosocial and spiritual” (World Health Organisation, 2003).

It is of vital importance to acknowledge impending death. An important part of quality end of life care is advanced care planning, a process of communication between patients, health care providers and families (Martin et al, 2000). 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. Clear and honest information needs to be given. 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 minimal intervention and invasive investigations must be adhered to at all times and the patient’s wishes must be fully respected.

The prospect of transplantation should never influence terminal care. To offer false hope may lead to feelings of anger from the relatives and may deny the opportunity to say farewells. It is our responsibility to prepare the patient for death without, however, destroying all hope.

Changing the emphasis of care does not imply withdrawal of treatment, but allows us to focus on providing symptom control. It is completely inappropriate to maintain life by the use of artificial invasive ventilation if there is no prospect of transplantation. This only prolongs the process of dying with no reasonable hope of survival. The use of non-invasive ventilation during this time can be difficult, but when used appropriately can provide very effective relief from distressing symptoms. It is vital that its limitations are discussed with both the patient and the family, especially with regard to the timing of its withdrawal. The most distressing symptoms can be breathlessness, cough, chest pain and sputum retention. Humidification, nebulised bronchodilators, regular analgesia and continuous antibiotics can help. Nebulised morphine four hourly and/or high flow oxygen given via elephant tubing may alleviate distressing breathlessness. If these measures fail to provide symptom control, opiates (e.g. diamorphine) are the treatment of choice. There are many different ways of administering opiates. For acute symptoms requiring short term relief liquid preparations are available (Oramorph® or OxyNorm®). For longer relief of symptoms slow release tablets are given twice daily (MST Continus® or OxyContin®). For severe distress or breathlessness medication can be given as a continuous infusion (Diamorphine or Morphine Sulphate). In the severely breathless or distressed patient diamorphine is given as a continuous infusion, either subcutaneously or intravenously. This allows optimum symptom control and increased dosage can be given at any time. The choice of opiates varies according to the patient’s individual needs. The newer opiates such as OxyNorm® and OxyContin® are now prescribed first line as they are associated with a lower incidence of side effects. Opiates are always given in association with anti-emetics (cyclizine is the first choice) to prevent associated nausea and vomiting. The most effective combination therapy given via a 24 hour syringe driver is diamorphine, plus cyclizine and midazolam (an effective anxiolytic). Doses are titrated to symptom control. There is no ceiling to the doses of opiates needed. The specialist palliative care team are available to give advice on all aspects of care and the best combination of drugs for the individual patient. They can also provide advice on planning for discharge and community palliative care if required.

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. The aim of feeding is to prevent starvation and dehydration rather than to promote weight gain and the patient and family is always involved in decisions about omission or withdrawal. Oxygen therapy is continued unless specifically requested to be withdrawn by the patient or relatives.

Emotional support for the patient and their relatives is of paramount importance throughout. Empathy, and time to listen, are often more important than knowing what to say. Following death, bereavement counselling can be provided. Home visits by the Cystic Fibrosis Nurse Specialist (CFNS) and social worker can be continued for an indefinite time period.

The wishes of the patient often decide the location of terminal care. Strenuous efforts must be made to provide full support for the dying patient at home. This involves the GP, district nurses, CFNS, social worker and McMillan Nurses. The familiar 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. The use of monitoring equipment, which can be very intrusive, needs to be reviewed.

We must not forget the impact that a patient’s death has on the other patients on the ward at the time of death. We must allow these patients time to discuss their feelings and worries regarding their own mortality which is inevitable at this difficult time. It may also impact on their care with regard to future hospital admissions.

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”.



Martin DK, Emanuel LL, Singer PA. Planning for the end of life. Lancet 2000; 356: 1672-1676. [PubMed]

World Health Organisation (WHO), Geneva, Switzerland. Definition of terminal care, 2003.

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