Clinical review

ABC of palliative care: Non-malignant conditions

Much medical practice is still concerned with control of symptoms rather than cure, and doctors spend considerable time palliating and modifying symptoms associated with incurable disease.

The role of specialists in palliative medicine is to offer what has been learnt about palliation of malignant disease to those caring for patients with progressive, incurable, non-malignant conditions and to share and exchange best practice. Many symptoms experienced by cancer and non-cancer patients are similar: cancer patients' symptoms may be more severe, but those of non-cancer patients tend to be more prolonged.

The approach to controlling pain in progressive non-malignant conditions can be adopted from the strategy for managing cancer related pain. After an accurate diagnosis of the pain, appropriate treatment can be started: the principles of the World Health Organisation's analgesic ladder (discussed in the first article of this series) apply equally to non-cancer patients. The strength of analgesia chosen depends on the severity of the pain, and the choice of adjuvant analgesic depends on the pathogenesis of the pain.

Doctors may be concerned about giving opioids to patients with chronic non-malignant pain. This is a justifiable worry if a systematic approach to selecting patients is not adopted. In the absence of malignancy, underprescribing of opioids for pain is not uncommon. When opioids are prescribed and effective patients will, reasonably, request regular or increased doses. Pseudoaddiction, the seeking of drugs to control pain rather than to feed psychological dependence, should be identified.

	Advanced respiratory disease

Top Advanced respiratory disease Advanced cardiac disease Advanced neurological disease

The commonest chronic respiratory disorder requiring palliation is chronic obstructive pulmonary disease. Like many non-malignant conditions, however, the clinical course is not easy to predict, and a patient's life span with this condition can be decades. The illness may be characterised by frequent exacerbations followed by recovery to baseline status.

Management
If standard treatments—including oxygen, bronchodilators, antibiotics, and corticosteroids—do not adequately relieve symptoms opioids and benzodiazepines should be considered for breathlessness.

An immediate release formulation of morphine will generally ease resistant breathlessness, but use must be judged on an individual basis. Morphine should be started at a low dose, 2.5-5.0 mg every 4 hours, and titrated to an effective dose. For patients with carbon dioxide retention, careful monitoring is vital, and the frequency of dosing may have to be reduced. If an opioid is given a laxative must also be prescribed.

Often patients are anxious, and judicious use of a low dose benzodiazepine can be helpful. Care should be exercised with diazepam as, even at low doses, it will tend to accumulate because of its long half life. Lorazepam is shorter acting.

Discussion, explanations, and planning and non-drug measures are an integral part of management. Rehabilitation is desirable when possible and should be tailored to the individual patient.

	Advanced cardiac disease

Top Advanced respiratory disease Advanced cardiac disease Advanced neurological disease

The management of cardiac disease at all stages has a substantial palliative component, and, unlike management of cancer, there are few opportunities for cure. This section focuses on palliative care in cardiac failure, as this is the final common pathway in most patients with advanced cardiac disease who do not die suddenly.

Prevalence—Cardiac failure affects 1-2% of the adult population, and the prevalence rises steeply with age (to more than 10% of those aged over 70). It is a disabling and lethal condition that also has a detrimental effect on quality of life. Up to 30% of affected patients require hospitalisation in any year (120 000 admissions annually in the United Kingdom). Mortality is higher than in many forms of cancer, with a 60% annual mortality with grade 4 heart failure and an overall five year mortality of 80% in men.

Clinical aspects—There are several important similarities to and differences from cancer. The now seldom used term cardiac cachexia is as apposite a term in 1997 as it was 40 years ago. Advances in antianginal therapy and interventional techniques have reduced the importance of pain as a dominant feature of cardiac failure.

Management

General
Patients with advanced cardiac failure will be faced with frequent admissions to hospital. Since patients much prefer home management if possible, this should be recognised. Cardiac liaison nurses similar to the highly developed Macmillan system would reduce the number of admissions by early detection of worsening clinical features and by ensuring that patients' homes met all the necessary requirements.

Current indications for hospital admission are

• Need for intravenous therapy

• Persistent paroxysmal nocturnal breathlessness and orthopnoea

• Refractory dependent oedema, despite up to 120 mg oral frusemide twice daily

• Symptomatic postural hypotension

• Fluid leakage from lower limbs

• Development of dysrhythmias.

Dietary advice is important since patients may be obese or cachectic. Frequent small meals are preferred, which should be tailored to each patient's tastes. Tumour necrosis factor and interleukins are implicated in the causation of cachexia, and fish oils may reduce their levels. Supplements of fat soluble and water soluble vitamins may also be necessary to counteract their increased urinary loss and reduced absorption. A small amount of alcohol may help as an appetite stimulant and anxiolytic.

Reducing fluid intake to 1500 ml a day and avoiding excessively salty foods (but not to the extent of making food tasteless) will help in controlling oedema. There is evidence that exercise may lessen breathlessness and improve both quality of life and psychological wellbeing. This must be tailored to patients' individual needs.

View larger version (106K): [in this window] [in a new window]   Marked muscle wasting in the arms (left) combined with oedema of the legs (right) in a patient with advanced heart failure

Drug treatment
The main emphasis is symptom relief: drugs being given to improve prognosis should be reviewed.

Opioids, combined with antiemetic drugs if necessary, are useful for control of nocturnal breathlessness.

Diuretics also have a key role—orally, intravenously, or in combination depending on the severity of fluid retention. However, awareness of the clinical and biochemical features of overdiuresis is necessary. This can lead to postural hypotension, though other causes exist, some of which are shared by patients with cancer.

Digoxin has known symptomatic benefit in advanced heart failure. The optimal dose which avoids toxicity must be found.

Angiotensin converting enzyme inhibitors are of symptomatic benefit, and the dose should be titrated to ensure maximum benefit without adverse effects. Since many patients are volume depleted and hypotensive, small supervised test doses should be given—such as 6.25 mg captopril after 12-24 hours without diuretics. In patients unable to take angiotensin converting enzyme inhibitors other vasodilators should be considered.

Sublingual glyceryl trinitrate is helpful during episodes of breathlessness.

Vaccination—Influenza and pneumococcal vaccination are worth considering despite the advanced nature of the disease.

Counselling and psychological support
The highly developed support network for cancer patients is not available to patients with end stage cardiac disease. Counselling is certainly challenging in this situation because of the high incidence of sudden death (up to 50%) and the misconception of patients, who underestimate the seriousness of the situation. Application of many of the principles of palliative care is needed to optimise this aspect of management.

	Advanced neurological disease

Top Advanced respiratory disease Advanced cardiac disease Advanced neurological disease

Specialist palliative care services have developed a particular expertise in managing patients with progressive and advanced neurological conditions, of which motor neurone disease is perhaps the best studied example. Motor neurone disease (amyotrophic lateral sclerosis) is a disabling disorder of unknown aetiology for which there is no known cure. The mean survival is typically about three to four years.

Motor neurone disease can evoke the most negative attitudes in many medical staff, which are quite often conveyed to patients and their families. However, many of its symptoms can be alleviated by strict attention to detail and applying well established principles. While motor neurone disease is comparatively rare, many of its symptoms are common to other chronic neurological conditions. Patients with motor neurone disease require input from a range of disciplines. A strong commitment to teamwork is needed, and efficient channels of communication must be established and maintained.

The prevalence of symptoms in patients with motor neurone disease is similar to that in cancer patients. In a sample of patients referred to a hospice many of the cancer patients were referred specifically for symptom control, compared with only 15% of patients with motor neurone disease, all of whom had multiple symptoms.

Management of symptoms

Pain
Pain is common and troublesome in motor neurone disease, with 65-70% of patients reporting it to be a major symptom. Pain may occur at single or multiple sites and is often described as aching, cramping, burning, and shock-like. Some of the pain is caused by the stiffness associated with prolonged immobility and will be helped by physiotherapy and passive exercises. Additional relief can be achieved with a non-steroidal anti-inflammatory drug.

Many of these pains will require a strong opioid, and oral morphine is the drug of choice. The doses required are generally quite low, and patients may be maintained on stable doses of morphine for long periods. Although the value of opioids in motor neurone disease is generally accepted, their use is sometimes inappropriately reserved for the "terminal" phase only.

Swallowing and nutritional difficulties
Dysphagia is a common and distressing problem, and patients are often acutely embarrassed by the dribbling and coughing associated with trying to eat, while their carers are often frustrated by how long it takes to complete a meal. Salivary dribbling will usually respond to an anticholinergic agent. If drugs are unsuccessful, radiotherapy should be considered in order to dry up salivary secretions.

The swallowing reflex is often so impaired that patients cannot maintain an adequate fluid and energy intake. They will probably have experimented with a range of foods of varying consistencies, often supplemented by high energy drinks. Despite this they feel hungry and thirsty, and at least part of their continued clinical deterioration is related to their poor nutritional status.

Nasogastric tube feeding is unpleasant and cumbersome and is generally not well tolerated. Early consideration should be given to nutritional support with percutaneous endoscopic gastrostomy tube feeding. Improved nutritional status will result in a greater sense of wellbeing and should enhance patients' quality of life. Each patient will require careful explanation of the procedure and will need ongoing information and support. Complications are rare, with a mortality of 0.3-1.0% and morbidity of 3.0-5.9%.

Respiratory complications
These are a major source of morbidity and mortality in patients with motor neurone disease. Most deaths are associated with catastrophic respiratory muscle weakness. Symptoms such as cough and breathlessness are very common and may be evident at first diagnosis. Routine assessment of pulmonary function, when interpreted in conjunction with symptoms, can provide a useful indicator of disease progression and prognosis.

Management involves a multidisciplinary approach with careful assessment, explanation, and reassurance. Correct positioning enables patients to derive maximum benefit from weakened muscle groups. Oral morphine or low dose diazepam often helps to reduce the subjective sensation of breathlessness. Respiratory tract infections are sometimes difficult to diagnose clinically because of impaired inspiratory effort: symptomatic infections should be treated with appropriate antibiotics.

Ventilatory support is often controversial, yet many patients and families cope admirably with home ventilation and derive great symptomatic benefit and maintain an acceptable quality of life. Ventilatory support should be undertaken only after a comprehensive multidisciplinary assessment. Patients must be fully aware of all that is involved and be assured of adequate support.

	Notes

Tony O'Brien is consultant physician in palliative medicine at Marymount Hospice, Cork, and Cork University Hospital. John Welsh is professor in the Olav Kerr Chair of Palliative Medicine at Glasgow University. Francis G Dunn is consultant cardiologist at Stobhill Hospital, Glasgow.

The ABC of palliative care is edited by Marie Fallon, Marie Curie senior lecturer in palliative medicine, Beatson Oncology Centre, Western Infirmary, Glasgow, and Bill O'Neill, science and research adviser, British Medical Association, BMA House, London. It will be published as a book in June 1998.

Related Article

Adequate pain relief is important in non-malignant conditions

Lesley A M Northcote
BMJ 1998 317: 281. [Extract] [Full Text]

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• O'Leary, N., Tiernan, E. (2008). Survey of specialist palliative care services for noncancer patients in Ireland and perceived barriers. Palliat Med 22: 77-83 [Abstract]  
• Fitzsimons, D., Mullan, D., Wilson, J.S., Conway, B., Corcoran, B., Dempster, M., Gamble, J., Stewart, C., Rafferty, S., McMahon, M., MacMahon, J., Mulholland, P., Stockdale, P., Chew, E., Hanna, L., Brown, J., Ferguson, G., Fogarty, D. (2007). The challenge of patients' unmet palliative care needs in the final stages of chronic illness. Palliat Med 21: 313-322 [Abstract]  
• Rosenwax, L., McNamara, B, Blackmore, A., Holman, C. (2005). Estimating the size of a potential palliative care population. Palliat Med 19: 556-562 [Abstract]  
• Northcote, L. A M (1998). Adequate pain relief is important in non-malignant conditions. BMJ 317: 281-281 [Full text]