Elsevier

Neurologic Clinics

Volume 19, Issue 4, 1 November 2001, Pages 829-847
Neurologic Clinics

Palliative Care in Amyotrophic Lateral Sclerosis
Palliative care in amyotrophic lateral sclerosis

https://doi.org/10.1016/S0733-8619(05)70049-9Get rights and content

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Who is Involved in Palliative Care?

Palliative care in patients with ALS involves not only physicians but also a large number of different professionals (Table 2) and family members, for whom caring for the patient often becomes a full-time job. The physician's role is to coordinate the efforts and discuss the appropriate time for each intervention with the patient and family. In the United Kingdom, at least 75% of the inpatient palliative care/hospice units are involved in the care of patients with ALS.60 This figure is lower

Breaking the News

Palliative care begins by how the diagnosis is communicated to the patient.21 If the communication between physician and patient is inadequate, the patient will be left uncertain about the diagnosis, with a vague sense that he/she has some serious and probably incurable disease without any reasonable hope for the future.57 Often, such a situation leads to a pilgrimage from one doctor to another and from one hospital to another, until a physician establishes a good therapeutic relationship with

Weakness

Progressive weakness is the major symptom of ALS. Initially, patients find their stamina undergoes greater than usual day-to-day fluctuations, and they need to be reassured that this is a normal phenomenon and does not herald an increase in pace of disease progression. Active and passive physiotherapy can help in the prevention of muscle contractures and joint stiffness. The maximum exercise load may vary greatly from one day to another; as a rule, patients should never exercise to the point of

Psychologic Problems

Most, if not all, patients with ALS undergo a phase of reactive depression after diagnosis. Counseling is of paramount importance at this stage. The reported prevalence of depression in ALS varies depending on the assessment method. Although full-fledged major depression, according to Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV) criteria is infrequent (around 10%), self-reported depressive symptoms have been described in 44% to 75% of patients.24, 81 Clinically

Quality of Life in ALS

The dilemma of quality of life (QOL) in patients with ALS begins with its definition. The definition followed here is that of Professor Ciaran O'Boyle, of Dublin, who said “Quality of life is whatever the patient says it is.”58 Professor O'Boyle developed a QOL measure based on patient-generated cues, the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQOL-DW). In a randomized study,53 this scale was judged by patients with ALS as being more valid (i.e.,

Psychosocial Care

In palliative care, as defined by the WHO, “the control of psychological, social, and spiritual problems is paramount.”80 Psychosocial care is often the most important cornerstone of palliative care in ALS and cannot be administered by physicians alone. A multi-disciplinary team approach is necessary, as has been exemplified by several excellent models of care around the world.34 Palliative care in patients with ALS involves a number of different professions (see Table 2). Close collaboration

Spiritual Care and Bereavement

As with any terminal illness, spiritual care is an important but often overlooked part of palliative care. The word spiritual has several implications and is difficult to define. Sykes76 defines it as “the need to find within present existence a sense of meaning,” which may or may not involve a religious framework. A recent study indicates that spirituality or religiousness may affect the use of PEG and NIV in ALS and may be a source of comfort to the patients.51 Cases of patients whose

Terminal Phase

A retrospective survey of 171 patients with ALS showed that around 90% of the patients died peacefully, mostly in their sleep, and none of the patients choked to death.52 If patients with ALS are not artificially ventilated, the death process usually begins with the patients slipping from sleep into coma because of increasing hypercapnia. In this phase of the disease, efforts are directed solely at maintaining patient comfort.77 If restlessness or signs of dyspnea develop, morphine should be

Summary

Patients with ALS witness their progressing debilitation with a clear mind. This situation is regarded as a nightmare by most neurologists; however, the intact mentation offers patients with ALS the possibility to develop coping mechanisms that can lead to a surprisingly serene acceptance of the disease. Indeed, most professionals dealing with patients with ALS and their families would agree that they are often exceptionally pleasant and warm people. This observation, although unexplained, is

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    Address reprint requests to, Gian Domenico Borasio, MD, Department of Neurology and, Interdisciplinary Palliative Care Unit, Munich University Hospital-Grosshadern, D-81366 Munich, Germany

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    Copyright © 2001 W. B. Saunders Company. Published by Elsevier Inc. All rights reserved.