Terminal care and cystic fibrosis

Terminal care

Christine Etherington.Jan, 2001. Terminal care [online]. Seacroft and St James's University Hospitals, Leeds, UK. Available from http://www.cysticfibrosismedicine.com

It is a sad but true fact that even with optimal medical care cystic fibrosis remains a life limiting condition. The terminal phase is usually heralded by increased frequency and severity of respiratory exacerbations, oxygen dependence and declining 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. It is of vital importance to acknowledge impending death. 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 can be done 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 with all members of the CF caring team so that conflicting messages are not given to the patient. At all times 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 important 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 ventilation. This only prolongs the process of dying with no reasonable hope of survival.

The most distressing symptoms are breathlessness, cough and sputum retention. Humidification, nebulised bronchodilators and antibiotics can be of help. If these measures fail to provide symptom control, opiates (e.g. diamorphine) are the treatment of choice. Nebulised morphine can help to alleviate the feeling of breathlessness. In the severely breathless or distressed patient diamorphine is given as a continuous infusion, either subcutaneously or intravenously. This allows for optimum symptom control and can allow for increased dosage at any time. Opiates are always given in association with anti-emetics to prevent associated nausea. The palliative care team are often involved to ensure the best combination of drugs is given for each specific patient. 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.

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. The wishes of the patient often defines the location of care. Strenuous efforts must be made to provide full support for the dying patient at home. This involves the GP, district nurses, CF liaison nurses 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. All monitoring equipment needs to be reviewed, the use of which can often be highly intrusive. In summary, terminal care involves emphasis on care and a team approach, as observed by Ambroise Pare "to heal sometimes, to relieve often and to console always".