In addition to PFTs, nocturnal pulse oximetry or polysomnography are useful tests for evaluating the possibility of nocturnal hypopnea.3,52 Even when tests fail to show a specific threshold of weakness, NIPPV is recommended for the treatment of these respiratory symptoms. In the absence of these symptoms, when respiratory surveillance demonstrates a decline in FVC of less than 50%, PaCO2 of greater than 45 mm Hg, or nocturnal desaturations of less than 88% sustained over 5 minutes, NIPPV should be started (Table 2).57
Table 2 — Managing respiratory weakness
|As respiratory capacity decreases:|
|Indications for NIPPV|
|Management of persistent symptoms after NIPPV|
NIPPV is most commonly delivered through an oral or nasal mask with bi-level positive pressure. This can be used nocturnally, as needed during the day, or nearly continuously. Some patients with ALS—particularly those with advanced bulbar weakness—cannot tolerate masks58; however, recent studies have shown that tolerance of NIPPV predicts improved survival—to a median 10 to 12 months.59,60 NIPPV also improves QOL after 1 month of use, which is maintained for more than 1 year.61,62
Other methods of augmenting respiratory function include chest percussion, manual cough assist, and a mechanical insufflator/exsufflator (MIE). The MIE works with a mouthpiece that provides abrupt decrements in expiratory pressure, mimicking a cough. This technique may be useful in patients—including those who cannot voluntarily cough—to generate clinically effective cough flow rates,63 improving secretion clearance. High-frequency chest wall oscillation, which uses a vest with inflated sections that pulsate at frequencies of 5 to 20 Hz, may slow the decline of FVC.64-66
When other options for noninvasive pulmonary assistance fail, the last remaining treatment for patients in impending respiratory failure is LTMV through tracheotomy. Patients seldom choose this option; however, those who do tend to be younger and to have young children, increased income, increased education, and increased optimism.35
If a patient chooses LTMV, it should be undertaken when pulmonary symptoms are declining with upcoming respiratory failure. Waiting for actual respiratory failure adds unnecessary morbidity and risk of complication to the transition. Nonetheless, many patients do not decide to undertake LTMV until a respiratory emergency occurs.52
Patients can sometimes live nearly 3 years after starting LTMV, although many experience severe progressive systemic weakness mimicking a locked-in state. Despite this, the large majority of patients were glad that they chose this care option, and it has been noted that depression is remarkably absent in this group.35
LTMV requires 24-hour supervision and care, which can be provided at a skilled nursing facility or at home. Patients with LTMV who live at home require at least 2 full-time caregivers so that one may be with the patient at all times to address any problems that develop with the ventilator and other technical needs.
ALS has heightened public awareness and increased public policy debates about end-of-life issues more than most other terminal illnesses. Physician-assisted suicide is practiced in ALS more than in other terminal illnesses in Oregon, where the procedure is legal,67 and in a 60 Minutes broadcast on November 24, 1998, millions of persons observed Jack Kevorkian, MD, euthanize a patient with ALS. In the Netherlands, 35% of patients with ALS plan to die by euthanasia and another 4% by physician-assisted suicide.68
Many physicians are uncertain about their legal and ethical duties regarding end-of-life issues.69 An important component of ALS care is discussion of end-of-life issues such as code status and advance directives about respiratory and feeding decisions. Because patients are often reluctant to consider these issues, it is important that the physician or nurse caring for the patient proactively address them. It is also important for physicians to discuss their ethical and legal obligations to the patient in regard to end-of-life care.
A living will, durable power of attorney, and other documents are needed to dictate patients' wishes about care to be received or withheld.70,71 Because advance directives do not ensure appropriate care, it is important that the patient discuss the directives with the physician and family.13,14,72,73 The article "Empowering the individual with ALS at the end-of-life: disease-specific advance care planning" by Benditt and colleagues,70 which appeared in the December 2001 issue of Muscle & Nerve, includes an ALS-specific directive that patients may find useful.
Home care is often inadequate and results in a large burden on caregivers.74 In contrast, hospice services often allow patients to die in their own homes with less suffering.75,76 Hospice care has been shown to be helpful in reducing symptoms of pain, sialorrhea, and dyspnea.77 Its use should be discussed early with patients.78,79
Significant symptoms in terminal phases of ALS include choking, breathing difficulties, pain, and fear.76,80 Pain in particular—which despite popular misconceptions is common and can be severe at any stage of ALS—is most often described in the last month of life, with prevalence ranging from 34% to 88%.81-83 Pain can be managed with narcotic and nonnarcotic analgesics. Stretching and range of motion exercises are also helpful both in prevention and treatment of pain.3
Notably, most caregivers report that patients with ALS die with dignity and in a peaceful manner.
Although curative treatments are not yet an option in the care of patients with ALS, management of symptoms and complications improves QOL. A multidisciplinary approach involving respiratory, physical, and occupational therapists; social workers; and counselors is helpful in the care of these patients. A proactive approach to discussing and planning for future disease progression is helpful in avoiding unnecessary complications, maximizing QOL, and providing patients with a sense of control.
The authors wish to thank Peter Donofrio, MD, director of the Vanderbilt ALS Clinic and MDA Clinic and division chief of Neuromuscular Disorders at Vanderbilt University in Nashville, Tennessee, for his helpful editorial comments on the manuscript.
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CHRISTOPHER LEE, MD, is a resident and AMANDA C. PELTIER, MD, MS, is assistant professor of neurology, Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee.