States of Profound Paralysis with Intact Cognition
Challenging ethical issues arise in neurological disorders that produce chronic profound paralysis of voluntary muscles while preserving consciousness and cognition. Disorders that produce this tragic state include the locked-in syndrome, the late stage of amyotrophic lateral sclerosis (ALS), the final stage of Duchenne muscular dystrophy and other progressive neuromuscular diseases, and high-cervical spinal cord transection with ventilator dependency. Medical decision making in these patients is complicated because they cannot move or physically manipulate their environments and have marked communication impairment despite their generally preserved cognitive functioning.
These patients retain the capacity to make health-care decisions because the illnesses producing paralysis usually spare centers controlling consciousness and cognition. Physicians thus have a duty to obtain valid consent for or refusal of life-saving treatment (LST) from the patient, not from a surrogate decision maker. Physicians responding to refusals of LST by profoundly paralyzed patients have a heightened duty to assure the validity of their consent or refusal of treatment by optimizing patients’ physical, psychological, communicative, and rehabilitative condition. Physicians responding to treatment decisions of paralyzed patients should: (1) educate patients fully about their disease course, treatment, and rehabilitation; (2) optimize patients’ communication abilities to permit them to retain the highest degree of control; (3) fully rehabilitate them using modern therapies of neuro-rehabilitation; (4) adequately diagnose and treat concomitant depression; and (5) provide loyal, attentive medical care assuring availability, continuity, emotional support, and encouragement that the physician will support the patient’s decision. This chapter focuses on the tragic plight of these patients, the duties of physicians to assist them in making health-care decisions, the compassionate balance of physicians’ paternalistic encouragement for them to live against their faithful willingness to stop LST once the patient has refused it validly, and the responsibility of physicians to provide for their rehabilitative, palliative, and terminal care.
PARALYZING NEUROLOGICAL DISORDERS THAT SPARE COGNITION
The paradigmatic chronic neurological conditions causing profound paralysis but sparing cognition that I consider in this chapter are ALS, locked-in syndrome, Duchenne muscular dystrophy, and high-cervical spinal cord transaction with ventilator dependency. My analysis applies to other conditions producing the same combination of neurological signs.
Amyotrophic Lateral Sclerosis
ALS is an acquired, progressive, irreversible, paralyzing, incurable, and fatal neuromuscular disease in which affected patients retain relatively normal cognition throughout the illness. Although recent reports stress the presence of cognitive dysfunction as the illness progresses,1
cognitive impairment usually is not severe enough to interfere with the ability of the ALS patient to provide valid consent to or refusal of treatment. Affected patients develop the subacute onset of progressive, asymmetric muscle weakness leading to diffuse paralysis, muscle atrophy, and fasciculations, with anarthria, aphonia, dysphagia, and progressive respiratory insufficiency. Patients with ALS usually die within several years of disease onset from inexorable respiratory failure, if their ventilation is not supported mechanically. With tracheostomy, gastrostomy, and respiratory support, including tracheostomy positive-pressure ventilation (TPPV), more prolonged survivals are possible.2 Specific treatments to slow disease progression have only a limited effect.
cognitive impairment usually is not severe enough to interfere with the ability of the ALS patient to provide valid consent to or refusal of treatment. Affected patients develop the subacute onset of progressive, asymmetric muscle weakness leading to diffuse paralysis, muscle atrophy, and fasciculations, with anarthria, aphonia, dysphagia, and progressive respiratory insufficiency. Patients with ALS usually die within several years of disease onset from inexorable respiratory failure, if their ventilation is not supported mechanically. With tracheostomy, gastrostomy, and respiratory support, including tracheostomy positive-pressure ventilation (TPPV), more prolonged survivals are possible.2 Specific treatments to slow disease progression have only a limited effect.
Locked-In Syndrome
The locked-in syndrome is an acquired state of acute de-efferentation, classically produced by a large infarction or hemorrhage in the pontine tegmentum and base, producing quadriplegia, pseudobulbar palsy, and paralysis of horizontal eye movements. Once the acute encephalopathy resolves, locked-in patients usually remain awake and alert, breathe spontaneously, and have normal cognition, to the extent it can be tested accurately. Inexperi-enced examiners may incorrectly diagnose them as comatose because of their profound paralysis, pinpoint pupils, and seeming unresponsiveness. They can be taught to communicate with voluntary vertical eye movements and eyelid movements, which typically are the only volitional movements they retain. Most affected patients do not survive longer than a few months, but a small group of otherwise healthy young patients who have suffered basilar artery occlusion can survive for years in a chronic locked-in syndrome.3 Some chronic locked-in patients have been taught to communicate in Morse code with vertical eye movements or through computerized systems triggered by eye movements, enabling them to communicate complex thoughts successfully.4 Two young patients in a locked-in syndrome from pontine infarction caused by basilar artery occlusion have “dictated” books poignantly describing their plight and their sources of joy and suffering, with the assistance of colleagues who carefully recorded and translated their eye movements into words.5 A narrative of another young locked-in patient, emphasizing the patient’s suffering, his difficulties in communication, and the ethical issues in his treatment was featured in the BMJ recently.6 A similar state of locked-in syndrome can result from the final stage of progressive neuromuscular diseases including ALS.7
Duchenne Muscular Dystrophy
The muscular dystrophies are a heterogeneous and incurable group of genetic disorders of muscle metabolism in which inexorably progressive muscle weakness develops over many years. Duchenne muscular dystrophy (DMD), affecting 1 in 3,500 boys, is an X-linked recessively transmitted disease that begins in early childhood with weakness of the pelvic and shoulder girdle musculature and progresses to generalized paralysis. Because of progressive weakness, DMD patients usually become confined to a wheelchair by ages 8 to 12 years. At this point they begin to develop progressive paralysis of respiratory muscles that, without ventilatory support, leads to their death, usually between ages 20 and 25 years. Cardiac failure accounts for 10-20% of deaths.8 Although glucocorticoid therapy may reduce the rate of progression of muscle weakness, DMD patients treated with glucocorticoids survive no longer than those untreated.9
Spinal Cord Trauma
Severe trauma to the spinal cord above the fourth cervical segment produces spastic quadriplegia, sensory loss below the level of the lesion, and respiratory paralysis because of damage to the phrenic nerves or nuclei. Not only do such patients lack all motor and sensory function below the neck, they require tracheostomy, endotracheal intubation, and tracheal positive-pressure ventilation (TPPV) for the remainder of their lives. Complete spinal cord transection produces permanent and profound motor, sensory, respiratory, and autonomic deficits. Neurological function from incomplete transactions, spinal cord
compressions, and other injuries may spontaneously improve to some extent for up to a year or so and then stabilize.10
compressions, and other injuries may spontaneously improve to some extent for up to a year or so and then stabilize.10
TELLING PATIENTS THE TRUTH ABOUT DIAGNOSIS AND PROGNOSIS
Once the diagnosis of ALS has been made confidently or once the prognosis for recovery is determined to be poor in the patient with locked-in syndrome or spinal cord transection, physicians are faced with breaking the bad news to patients and families. Most physicians understandably experience psychological discomfort in discussing the diagnosis and the prognosis of hopeless neurological disorders. Most clinicians ultimately choose to state the painful truth, although doing so is difficult.11
In the past, some physicians believed that patients with incurable disorders should not be told their diagnosis and prognosis. Instead, these physicians preferred to employ vague euphemisms. In the case of ALS, for example, they may have told their patients that they suffered from a “viral nerve disorder.” In an attempt to justify their purposeful deception, they resorted to one or more of the following lines of rationalization: (1) no good is produced by telling the patient the exact diagnosis, because the disease in untreatable; (2) harm is produced in confirming the diagnosis because it removes all the patient’s hope for recovery, improvement, or continued life; (3) the patient does not truly wish to know the real diagnosis and would be happier if the stated diagnosis remained a vague euphemism; (4) because there is no way to prove that the clinical diagnosis is correct, it is immoral to make the patient suffer given the physician’s uncertainty; and (5) the patient probably would not understand the physician’s explanation of the diagnosis and prognosis.12
A utilitarian ethical analysis balances the harms and benefits of deceiving patients to test if the harms are justified by the benefits. When patients are deceived about their diagnosis and prognosis, the harms of this deception are numerous and nearly always greatly outweigh the benefits. For example, the fact that ALS may be incurable does not mean it is untreatable. Riluzole may provide a modest prolongation of life.13 Symptomatic treatments are available for the common complications of ALS, including respiratory failure, dysphagia, and spasticity.14 The patient can maintain hope by participating as a subject in clinical trials of pharmaceutical agents being tested for their efficacy against ALS. Most significantly, knowing the diagnosis and prognosis permits the patient to make important decisions about marriage, career, financial matters, genetic testing, and end-of-life issues in a timely manner and to make an informed decision whether to consent to TPPV or gastrostomy.
A patient who does not receive a diagnosis from a physician after a reasonable assessment period will visit other physicians, often undergoing repetitive, expensive, wasteful, painful, and potentially dangerous tests until finally he is given a diagnosis. Because of the initial deception, he may distrust physicians and be suspicious about future medical care and honest communication. The patient also will have lost valuable time by undergoing unnecessary repetitive tests, which will unjustifiably delay his opportunity to make adequate plans for the future.
A deontological ethical analysis asks if physicians who advocate deceiving patients about their diagnosis would be willing to state such a policy publicly, have everyone know that they would practice deception in analogous cases, and advocate that all other physicians practice in a similar deceptive pattern. As discussed in chapter 2, neither a utilitarian nor a deontological analysis can justify the paternalism of diagnostic deception in such cases.
Surveys of patients with ALS, multiple sclerosis, and other incurable, chronic neurological disorders reveal that nearly all patients desire to learn their diagnoses and prognoses.15 Physicians should tell patients the truth about their diagnosis and prognosis, but should tell them in a way that maximizes empathy, hope, and reassurance. Patients should be given the opportunity and encouraged to seek second opinions. As clinicians explain how common complications can be treated, they should also tell their patients to think seriously about what kinds of treatment
they would wish to receive in the progressive phases of their illness. The ability of patients to understand and retain control of future treatment decisions is therapeutically beneficial. As Christine Cassel phrased it, “autonomy is therapy” for the ALS patient.16
they would wish to receive in the progressive phases of their illness. The ability of patients to understand and retain control of future treatment decisions is therapeutically beneficial. As Christine Cassel phrased it, “autonomy is therapy” for the ALS patient.16
Patients may respond to bad clinical news, such as the diagnosis of ALS, by adopting a sequence of psychological coping strategies that encompass denial, blame, intellectualization, disbelief, and acceptance. They may exhibit one of several affective responses to the news including anger, fear, anxiety, helplessness, hopelessness, shame, relief, or guilt. Timothy Quill and Penny Townsend pointed out that physicians can help a patient adjust to bad clinical news in several ways. They should assure continuity of follow-up care, be available for continuous psychological support, provide necessary information, minimize the patient’s isolation, and respond to the patient’s physical and emotional discomfort.17
The American Academy of Neurology evidence-based practice parameter on the care of ALS patients states that physicians should offer the diagnosis of ALS to the patient who wants to know, or to a family member if the patient prefers.18 The diagnosis should be communicated in person, not by telephone, in a calm, quiet, unhurried manner and in a supportive environment. The physician should assess the patient’s knowledge of the disease and warn the patient that bad news is forthcoming. The physician should acknowledge and explore the patient’s emotional reaction to the news. The physician can provide printed material about the disease and instructions on how to contact support and advocacy organizations. In the conversation, the physician should reassure the patient that he or she will continue to provide ongoing medical care, be available to answer questions, provide advice, and treat complications as they occur, will not abandon the patient, and will offer opportunities if available to participate in clinical research. The report of the Robert Wood Johnson Foundation Workgroup on Promoting Excellence in End-of-Life Care in ALS made similar recommendations.19
Leo McCluskey and colleagues surveyed ALS patients and their caregivers to assess how well physicians broke the news of the diagnosis. Patients reported that 25% of physicians had below average or poor communication skills. Caregivers were more critical; they rated over 50% of the physicians as having poor or below average communication skills. The authors concluded that there is room for improvement in physicians’ communication skills in breaking bad news, and that they need to learn and practice established communication techniques that have been recommended by the American Academy of Neurology and other groups.20
EDUCATING PATIENTS ABOUT VENTILATORS AND OTHER FORMS OF LIFE SUPPORT
Neurologists caring for paralyzed, cognitively intact patients have the important duty to educate them about the technological treatment options available to them at different stages of their illness. This information should be presented in an objective, unbiased, and clear manner so patients can understand that each treatment decision ultimately is theirs to make. It is also reassuring for them to know that their neurologist will support them in each decision. For example, many ALS patients who consent to other supportive treatments choose to draw the line at TPPV. They are willing to receive other forms of treatment, but when their respiratory function worsens, they indicate that they would rather die than receive TPPV. Similarly, some ALS patients refuse gastrostomy tube placement, preferring to die at the time they no longer can swallow. The principal technologies about which patients should be instructed are ventilatory options, feeding options, and cardiopulmonary resuscitation.
Many patients and some neurologists incorrectly believe that mechanical ventilation is an “all or none” phenomenon for ALS, muscular dystrophy, and other progressive neuromuscular diseases that cause respiratory failure. They wrongly conceptualize their choice as to accept TPPV or have no ventilatory support at all. In fact, several intermediate ventilatory options are available that usually compensate
successfully for lesser states of neuromuscular respiratory failure. ALS patients frequently hypoventilate during sleep (“sleep-disordered breathing”) in the early stage of the illness, producing nocturnal awakening, headache, anorexia, lethargy, and daytime drowsiness. Sleep-disordered breathing may be treated successfully by noninvasive nocturnal nasal ventilation using continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) by oronasal mask.21 These devices can be put on and taken off easily and do not require tracheostomy or endotracheal intubation. The noninvasive positive-pressure devices, generally referred to a noninvasive positive-pressure ventilation (NPPV), however, are not capable of compensating for the more severe degrees of ventilatory failure that inevitably occur later in the course of ALS and DMD.22
successfully for lesser states of neuromuscular respiratory failure. ALS patients frequently hypoventilate during sleep (“sleep-disordered breathing”) in the early stage of the illness, producing nocturnal awakening, headache, anorexia, lethargy, and daytime drowsiness. Sleep-disordered breathing may be treated successfully by noninvasive nocturnal nasal ventilation using continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) by oronasal mask.21 These devices can be put on and taken off easily and do not require tracheostomy or endotracheal intubation. The noninvasive positive-pressure devices, generally referred to a noninvasive positive-pressure ventilation (NPPV), however, are not capable of compensating for the more severe degrees of ventilatory failure that inevitably occur later in the course of ALS and DMD.22
Studies of patients with ALS and DMD show high success rates of using NPPV. Several studies of NPPV in ALS disclosed that its successful use increased patients’ survival.23 The principal problems making patients intolerant of NPPV were bulbar symptoms, especially the sensation of choking from the inability to clear secretions. Patients who are intolerant of NPPV can be offered TPPV.24 Several studies have shown improved quality of life for ALS patients receiving NPPV.25 A study of the effect of NPPV on ALS patients and their caregivers found that respiratory muscle weakness had a greater effect than overall ALS severity on patients’ rating of their quality of life, that NPPV improved patients’ quality of life despite worsening paralysis from ALS, and that NPPV did not significantly increase caregiver burden or stress.26 However, the relatively low percentage of eligible ALS patients referred for NPPV suggests that many physicians are unaware of its benefits.27
Children with early respiratory failure from DMD or spinal muscular atrophy also do well with NPPV. A recent study of children with mild forms of respiratory failure resulting from DMD or spinal muscular atrophy showed that NPPV reduced symptoms of headache and sleepiness, reduced hospitalizations and health-care costs, and had no adverse impact on their quality of life.28 Nevertheless, in a British survey of physicians’ attitudes and practices, 13% of physicians claimed that NPPV resulted in a poor quality of life for DMD patients.29
Patients should be told that all mechanical breathing assistive devices including NPPV and TPPV are adaptable for home use, assuming that financial, nursing, and other logistical arrangements are satisfactory.30 Positive-pressure ventilators that are small, quiet, and easily portable have been developed for home and wheelchair use. Knowing that such technology exists can influence patients in deciding whether they want to receive chronic TPPV treatment.
Many ALS patients and some DMD patients continue to lead highly satisfying lives despite being on long-term home TPPV.31 Despite their obvious disability, they may be quite satisfied with their life quality. One study showed that ALS patients on long-term TPPV were not more depressed than ALS patients not requiring ventilators.32 Another study showed that health-care personnel systematically underestimated the quality of life of paralyzed patients on long-term TPPV.33 In fact, ALS patients’ self-assessed quality of life is completely independent of their level of physical functioning.34 Studies of children receiving home TPPV for respiratory failure from DMD or spinal muscular atrophy show that the technology can be successful and unburdensome.35 Nevertheless, one survey disclosed that 25% of Canadian physicians did not discuss the option of long-term TPPV with DMD patients.36
The adequacy of health-care insurance coverage is the major factor in the patient’s decision to maintain home ventilation. Home nursing care represents the greatest component of this expense.37 Patients should be told that they can be maintained in their home for several years, given sufficient insurance coverage and family support. The renowned British theoretical physicist Stephen Hawking is an unusual example of an ALS patient who has experienced prolonged survival and continues to live successfully at home with a portable positive-pressure ventilator, computerized voice synthesizer, and daily nursing care.
Assisted feeding options include nasogastric tubes, gastrostomy tubes, and jejunostomy tubes. Nasogastric (“Dobhoff”) tubes are suitable for temporary use, but they irritate the
nasopharynx and esophagus when in place for longer than a month. The feeding gastrostomy tube inserted by percutaneous incision under endoscopic guidance (PEG tube) is an ideal long-term solution for patients who wish to maintain hydration and nutrition when they can no longer swallow. Feeding jejunostomy tubes require a more lengthy and complicated surgical procedure for insertion and more skilled nursing care for maintenance. Jejunostomy tubes avoid the risk of gastroesophageal reflux that complicates the use of some PEG tubes, but they have a smaller gauge than PEG tubes and therefore, require longer daily periods of continuous infusion with a mechanical feeding pump.38
nasopharynx and esophagus when in place for longer than a month. The feeding gastrostomy tube inserted by percutaneous incision under endoscopic guidance (PEG tube) is an ideal long-term solution for patients who wish to maintain hydration and nutrition when they can no longer swallow. Feeding jejunostomy tubes require a more lengthy and complicated surgical procedure for insertion and more skilled nursing care for maintenance. Jejunostomy tubes avoid the risk of gastroesophageal reflux that complicates the use of some PEG tubes, but they have a smaller gauge than PEG tubes and therefore, require longer daily periods of continuous infusion with a mechanical feeding pump.38
For most ALS patients, the PEG tube represents the ideal solution to the problems of maintaining nutritional intake, administering medications, and preventing aspiration. Most ALS patients are satisfied with the use of their PEG device.39 The PEG tube usually should be inserted at about the time the patient’s forced vital capacity falls to 50% of predicted value.40
Cardiopulmonary resuscitation (CPR) as a treatment option should be discussed with the patient far in advance of when it might be needed. Many ALS patients refuse to be resuscitated or intubated. They should not be intubated if they develop pulmonary failure, but instead treated with opioid drugs and other medications to reduce air hunger as discussed later in this chapter.
Advance directives can be useful for ALS patients, even if they retain independent decision-making capacity throughout their illness. The directives can formalize patients’ decisions about specific treatments and can appoint a legal surrogate decision maker should the patient lose decision-making capacity, even temporarily. In the ALS Patient Care Database, 70% of enrolled patients had completed advance directives.41 John Bach cautioned that written directives that include preferences for respiratory support could be harmful unless patients are fully informed of all available treatment options.42 Advance directives also have been used successfully by patients paralyzed by spinal cord injury.43
Steven Albert and colleagues studied preferences for LST in 121 ALS patients and compared the decisions they stated earlier in the illness with choices they made a year later when their illnesses had progressed and many had required the modalities in question. They found that patients could clearly express their wishes and that most wishes did not change with increasing disease severity. For example, 20% of the patients who found tracheostomy acceptable underwent the procedure compared with only 3% of those who found it unacceptable. Similarly, 48% of patients who found PEG acceptable underwent the procedure during the study period compared to only 8% of those who found it unacceptable.44 In a later study, the same investigators found that ALS patients’ attitudes toward religion and spirituality were an important factor that influenced their later choices about receiving PEG, tracheostomy, and NPPV.45
Cultural factors contribute to ALS patients’ decisions to receive TPPV and other forms of LST. In an analysis of attitudes about mechanical ventilation for ALS patients in North America, Europe, and Japan, Gian Borasio and colleagues found cultural differences. In North America, the decision most often was patient-driven and based on a concept of patient autonomy. In Japan, the decision usually was made by the physician, with some physicians ordering ventilators and others not. In Europe, an intermediate situation existed characterized by a balance between patient and physician authority. ALS patients who formerly were not given the option of mechanical ventilation now are given that option in many European countries.46 Steven Albert and colleagues recently showed that cultural differences in mental health, particularly the presence of pessimism and suffering, affect the will to live and govern the treatment choices of ALS patients at the end of life.47
PHYSICIANS’ DUTIES TO ENHANCE COMMUNICATION IN PARALYZED PATIENTS
Locked-in patients and ventilator-dependent patients with ALS or high-cervical spinal cord injury have extreme difficulty communicating.
A principal source of their suffering is their inability to make known their feelings, wishes, fears, anxiety, concerns, and decisions. A patient’s inability to communicate effectively also creates a serious problem for physicians who are trying to respect and follow the patient’s treatment decision. Physicians often cannot know confidently whether patients fully understand the implications of each treatment decision because of their communication impairment. Physicians may even be uncertain about patients’ capacity to make decisions because such an assessment is rendered difficult by the profound communication difficulty.
A principal source of their suffering is their inability to make known their feelings, wishes, fears, anxiety, concerns, and decisions. A patient’s inability to communicate effectively also creates a serious problem for physicians who are trying to respect and follow the patient’s treatment decision. Physicians often cannot know confidently whether patients fully understand the implications of each treatment decision because of their communication impairment. Physicians may even be uncertain about patients’ capacity to make decisions because such an assessment is rendered difficult by the profound communication difficulty.
Clinicians have an ethical duty to enhance these patients’ communication abilities.48 Patients can be taught simple communication systems for yes-no responses that require them to make at least rudimentary movements with whatever muscles remain under their control. Whereas, 30 years ago, locked-in patients who retained voluntary motor control only of vertical eye movements could be taught to use Morse code with their eye movements,49 today, many new high-technology computerized communication systems are available.50 Physicians should request the services of skilled occupational and speech-language therapists, with training in the use of these communication systems to maximize the patient’s ability to consent to treatment.51 Computerized letter boards, voice synthesizers, and head or eye movement-activated computerized language systems have helped paralyzed patients to overcome profound communication blocks and have permitted them to interact socially using complex language.52 One of my patients who was locked-in from advanced ALS used a sophisticated communication device activated by laser beam reflections from his cornea that allowed him to write letters and use the Internet and electronic mail.
Many but not all chronically locked-in patients rate their quality of life higher than able-bodied observers might expect.53 The one factor that correlates with self-reports of adequate quality of life is retaining or learning the ability to communicate.54 Novel technologies have been employed to assist communication in paralyzed patients, including EEG-based brain-computer interfaces55 and measurements of salivary pH in response to questions.56
PHYSICIANS’ DUTIES TO ENHANCE PATIENTS’ PSYCHOLOGICAL STATUS
Physicians have a duty to assess the psychological status of their patients to assure that a potentially reversible depression does not unduly influence the patient’s decision-making process. This step is necessary to show that the patient’s decision is rational; that is, to test if the patient who refuses LST has an adequate reason. It is understandable that a patient dying of ALS or who one who has been left permanently locked-in might develop reactive depression. It is the physician’s responsibility to judge the status and relevance of the patient’s depression. Depression may be so profound that the patient makes irrational decisions in refusing treatment. In this unusual circumstance, a physician may be ethically justified in overruling a patient’s refusal of treatment, at least temporarily. Neurologists should request psychiatric consultation for patients when they suspect that potentially reversible depression may be influencing their refusal of life sustaining treatment (LST).57
Physicians should attempt to enhance the psychological status of profoundly paralyzed patients by mastering the principles of care for chronic illness. They should provide continuous care to their patient, be readily available, and exhibit personal inner strength. They should assure the patient that they will do everything possible to prevent him from suffering and to carry out his treatment choices faithfully. Unity of purpose and action among physician, family, and friends creates a milieu of moral support necessary for the patient to cope psychologically with his terminal illness.
The psychological status of patients with ALS is a strong prognostic factor in determining their outcomes. In a cross-sectional, longitudinal prospective study adjusted for confounding factors, E.R. McDonald and investigators found a higher rate of death and a shorter life expectancy in ALS patients who were depressed compared with ALS patients who possessed more healthy psychological profiles at the same
stage in their illness.58 These findings underscore the duty of physicians to identify and treat depression as well as optimize the psychological condition of their chronically ill patients.
stage in their illness.58 These findings underscore the duty of physicians to identify and treat depression as well as optimize the psychological condition of their chronically ill patients.
Surprisingly, the incidence of major depression in ALS patients is small, even among those in late-stage disease. Judith Rabkin and colleagues found that the prevalence of depression in patients with late-stage ALS was 9% and did not change as death approached, although 19% of patients expressed a wish to die.59 The depressed patients expressed more pessimism and hopelessness than those who were not depressed. The strongest predictor of depression and anxiety was the degree of social support from spouses, other family members, and friends. ALS patients with poorer family and social supports report greater psychological distress than those who are supported better.60
An important factor correlating with depression is the patient’s sense of being a burden to the home caregiver. One study showed a positive correlation between the degree of ALS patients’ sense of being a burden to their caregivers and their degree of depression and poor quality of life.61 In an interview study of patient-caregiver pairs, both caregivers and patients reported that the psychosocial stress on the partner was greater than each reported independently: patients reported being a greater burden on caregivers than caregivers perceived, and caregivers reported that the patient shouldered a greater burden from the disease than the patient reported. This disparity in perception contrasted with the otherwise high degree of congruence of both parties’ independent reports assessing the patient’s level of function, pain level, optimism, and will to live.62
In a study of 100 ALS patients and their caregivers, Linda Ganzini and colleagues found that the two major causes of suffering in ALS patients were pain and the feeling of hopelessness.63 Hopelessness is a classic sign of depression. In a separate study, the same group found that ALS patients’ feelings of hopelessness correlated with more favorable attitudes toward physician-assisted suicide.64 In a study of correlates of the wish to die among end-stage ALS patients, patients who wished to die reported less optimism, less comfort from religion, and greater hopelessness. The authors cautioned that the wish to die in end-stage ALS patients was not simply a feature of depression.65
Despite the first impression of some healthy physicians, not all patients in profound states of irreversible paralysis wish to die. In a study of the quality of life experienced by chronically locked-in patients, Anderson and colleagues found that the quality-of-life scores of these chronic patients were lower than the scores of patients with cancer but higher than those of patients with terminal illnesses. Many locked-in patients said that they lived personally meaningful lives and wished to continue despite their severe disability.66 Neurologists have published detailed accounts of courageous patients who have continued to live active and fruitful lives despite utter paralysis and dependence on TPPV.67 John Bach reported a series of 89 ALS patients maintained on successful long-term TPPV for a mean of 4.4 years.68 More recently, Hideaki Hayashi and Edward Oppenheimer reported 70 ALS patients on long-term TPPV, 33 of whom had been on TPPV for more than five years, and eight of whom were totally locked-in. They pointed out that some ALS patients may have long-term survival with TPPV.69
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
