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Palliative care for patients with amyotrophic lateral sclerosis

Palliative Care for Patients
With Amyotrophic Lateral Sclerosis
“Prepare for the Worst and Hope for the Best”
Amyotrophic lateral sclerosis (ALS) is a devastating ter-
minal neurodegenerative disease with a highly predict-
able clinical course such that palliative care should be-
gin at or soon after diagnosis. The outcome is certain in

Dr SP is a 56-year-old neurologist specializing in the care ofpatients with amyotrophic lateral sclerosis (ALS). His past medi- most cases. The only medication approved for treat-
cal and family history are unremarkable. He is married and ment in the United States, riluzole, extends life by about
has 2 adult children. In July 2003, he noticed a right foot drop 2 months. Virtually all skeletal muscles eventually are af-
and experienced mild difficulty walking. In September, he de- fected. Multiple problems require a multidisciplinary ap-
veloped some urinary and fecal urgency and weakness in his proach including aggressive symptomatic management,
other foot. Extensive laboratory investigations were unre- rehabilitation to maintain motor function, nutritional and
markable except for a disk herniation at the thoracic level 6 respiratory support, augmentative communication de-
to 7 with indentation of the cord. The patient underwent adecompression of his thoracic spine in September 2003 and vices, and psychological support for both patients and
subsequently had 2 additional surgeries for persistent herni- families because family members so often play a central
ated disk at the same thoracic level. In mid-May 2004, his right role in management and care. Social, bioethical, and fi-
arm became weak. An electromyographic study showed de- nancial issues as well as advance directives should be ad-
nervation in the right leg muscles and chronic reinnervation dressed long before enteral feeding or assistive ventila-
changes in multiple myotomes in the right upper and lower tory support might be considered. Goals of care should
extremity. Neurological examination showed muscle atro- be assessed on an ongoing basis. Presenting the un-
phy in both legs and increased muscle tone in all extremities usual case of a patient with ALS who is also a prominent
with generalized pathological hyperreflexia. He walked withspastic gait. Sensory examination was completely normal. His neurologist specializing in ALS, we enumerate issues in
forced vital capacity was 4.97 L or 109% predicted with a maxi- management and palliative care applicable to ALS but also
mum inspiratory pressure (MIP) of 60 mm H to other fatal, progressive neurologic diseases such as
was given a diagnosis of ALS and self-referred to an ALS mul- Huntington’s chorea and late-stage Parkinson disease.
tidisciplinary clinic at a university medical center for further He was treated with riluzole, celecoxib (then in use in a Author Affiliations: Eleanor and Lou Gehrig ALS/MDA Center, College of Physi-
clinical trial), and several medications for urinary urgency cians and Surgeons, Columbia University, Neurological Institute (Dr Mitsumoto) and spasticity. Extensive recommendations were made by and Clinical Psychology in Psychiatry, Department of Psychiatry, College of Phy-sicians and Surgeons, Columbia University and New York State Psychiatric Insti- the ALS treatment team to maintain his function at home and at work, including proactive suggestions for respira- Corresponding Author: Judith G. Rabkin, PhD, MPH, Department of Psychiatry,
tory, nutritional, and communication issues.
College of Physicians and Surgeons, Columbia University and Research Scientist,
New York State Psychiatric Institute, 1051 Riverside Dr, New York, NY 10032 (jgr1
Perspectives on Care at the Close of Life is produced and edited at the University
See also Patient Page.
of California, San Francisco, by Stephen J. McPhee, MD, Michael W. Rabow, MD,and Steven Z. Pantilat, MD; Amy J. Markowitz, JD, is managing editor.
CME available online at
Perspectives on Care at the Close of Life Section Editor: Margaret A. Winker, MD,
Deputy Editor, JAMA.
2007 American Medical Association. All rights reserved.
(Reprinted) JAMA, July 11, 2007—Vol 298, No. 2 207
By July 2004, his speech became difficult. His course was tient and family. Invasive technologies may prolong life for complicated by deep venous thrombosis, and he started tak- patients at the very end stages of ALS if the patient so chooses, ing oral warfarin. By January 2006, he could no longer talk; although most do not. Research participation can provide he began to use an alphabet board with a laser pointer at- patients a sense of hope that they can make a vital contri- tached to his eyeglasses to communicate. He used glycopyr- bution to understanding the etiology and treatment of ALS.
rolate to help dry saliva but developed constipation. He lives Presenting the unusual case of an ALS patient who is also at home with his family and hired help in the home. Night- a prominent neurologist specializing in ALS, we enumer- time oxygen desaturation prompted initiation of a noninva- ate issues in the management and palliative care applicable sive ventilator. Additional medications included omepra- to ALS but also to other fatal, progressive neurologic dis- zole, lactulose, loratadine, bupropion, and budesonide nasal eases such as Huntington’s chorea, progressive supra- spray. He also took 1200 mg of Coenzyme Q10 four times a nuclear palsy, and late-stage Parkinson disease.
day, vitamin E and ␣-lipoic acid. His condition has contin-ued to deteriorate with more generalized muscle weakness and ALS: Diagnosis, Prognosis, Etiology
spasticity. In January 2006 his forced vital capacity was 1.88 Amyotrophic lateral sclerosis is a progressive neurodegenera- L or 42% predicted with a maximum inspiratory pressure of tive disorder of unknown cause and pathogenesis, with no 60 mm or higher. By the spring of 2007 he had become com- known cure.3 It is characterized by progressive loss of motor pletely paralyzed but has been relatively stable in the past year.
neurons; up to 80% may be lost before the first clinical symp- He can still use his eyes to communicate using an alphabet toms appear.4 Incidence is 0.2 to 2.4 cases per 100 000 popu- board and has done so recently in a public speech.
lation, with men more often affected than women and whites Dr SP was interviewed by a Perspectives editor on March more than nonwhites.5 Onset usually occurs in middle and 29, 2006, with his wife and son by his side. Using a laser late life. More than 90% of cases are sporadic while 5% to 10% pointer attached to his eyeglasses, Dr SP pointed to letters are familial (predominantly autosomal dominant).3 Among on an alphanumeric board, spelling out the words of his an- cases of familial ALS, 15% to 20% are attributable to the SOD1 swers, which were repeated aloud by his son and wife for gene mutation (although other mutations also have been rec- confirmation. After an initial few minutes of orientation, this ognized).6 While the etiology remains unknown, several plau- seemingly laborious mode of communication smoothed to sible hypotheses are currently under investigation.7-11 near conversational speed, as is apparent from the sophis- Diagnosis in early stages may be difficult and is often made tication and complexity of the responses.
9 to 11 months12 or longer13 after symptom onset. Diagnos-tic criteria (El Escorial Diagnostic Criteria-Revised, http: PERSPECTIVES
// require signs in more than 1 of 4 body DR SP (THE PATIENT): Each day focus on what you can regions, evidence of both lower and upper motor neuronal do . . . I’ve always tried to prepare for the worst and hope for degeneration, and progressive spread within or between re- the best. . . . Neurologists don’t like surprises. They like every- gions. There is no definitive diagnostic test. Median sur- thing planned out very carefully . . . vival is about 3 years and 5- and 10-year survival is 8% to TP (THE PATIENT’S WIFE): We’ve pretty much put our lives 16% in the absence of long-term mechanical ventilation on hold and SP is our primary focus. . . . We try to enjoy some- (LTMV).14 With LTMV, survival can be 15 years or longer.15 thing every day, even if it’s just a TV show or a movie, a trip to Both presenting symptoms and course vary consider- ably. Limb weakness is the initial symptom for about 60% DR A (THE TREATING NEUROLOGIST): We don’t want to do to 80% of patients,16,17 as it was for Dr SP. As noted by Row- anything to increase the quantity of someone’s life if the qual- land et al,3 painless difficulty with buttons or turning a key ity cannot also be increased . . . are ominous symptoms in midlife. Although there are no The diagnosis of ALS carries with it an inevitably fatal prog- typical stages, the weakness becomes more severe and more nosis. In the absence of curative treatments, the focus is on areas of the body are affected over time, perhaps with muscle enabling the patient to achieve maximal function and in- cramps and weight loss. The course is progressive with no dependence at each stage of illness by providing relief of the periods of remission. Stable plateaus are rare.3 Nearly all sys- multiple symptoms that develop over time.1 Patients diag- tems may become involved eventually, except for sphinc- nosed with ALS thus require palliative care, which, broadly ter control and eye movements which are usually but not defined, “seeks to prevent, relieve, reduce or soothe the symp- always spared. In the United States, most patients do not toms of disease or disorder without effecting a cure.”2 Be- choose to use LTMV and usually die of respiratory failure.
cause no single specialist can address the myriad problems As in all terminal diseases, predicting how long a patient that develop as the illness progresses, multidisciplinary teams will live is difficult, although older age at onset and bulbar are essential in providing optimal comprehensive care. Cli- presentation are negative prognostic factors.4,18 In late- nicians face a delicate balance in effectively managing the stage illness, loss of respiratory capacity is used as a crite- multiplicity of symptoms, while preserving the dignity and rion for hospice eligibility, implying a prognosis of 6 months autonomy of the patient and minimizing the fears of the pa- or less.19 Timing of discussion of advance directives and treat- 208 JAMA, July 11, 2007—Vol 298, No. 2 (Reprinted)
2007 American Medical Association. All rights reserved.
ment preferences must be tailored to the individual’s ill- management of initial symptoms and an overview of prob- ness course, readiness, and imminence of respiratory crisis.
lems to anticipate (eg, making safety changes in the house).
The only US Food and Drug Administration–approved Specific information, including written materials, referral to treatment for ALS is riluzole, prescribed 50 mg, orally twice support groups and to Web sites is timely (See http://www daily, which extends survival by about 2 months.20 Neu- for additional resources). Preliminary discussion rologists appreciate the availability of this approved medi- of advance directives and end-of-life issues may be broached.
cation for its role in offering patients hope, making the di- In the case of Dr SP, the diagnosis was initially uncertain agnosis more tolerable. Fatigue is a common adverse effect, and a series of surgeries were undertaken. When these pro- and modestly elevated liver enzymes also may occur. Other cedures had no effect, the patient himself made the diag- medications are being tested in ongoing clinical trials.
nosis of ALS and then went to see a neurologist specializ-ing in ALS for evaluation. In fact, he said, “I warned the Arriving at the Diagnosis of ALS
neurologist that I thought I had ALS. I wanted him to be DR SP (THE PATIENT): I knew ALS was the most likely diag- sure he would be comfortable with me as a patient before I nosis by far. . . . I came to accept the diagnosis before sharing saw him.” After the initial diagnosis was confirmed Dr SP was referred to an ALS multidisciplinary center, where hereceives ongoing care.
Exploring Alternatives
In its early stages, making the diagnosis of ALS can be diffi-
Role of the Interdisciplinary Clinic and Team
cult, and false-negative diagnoses by generalists are high.21 The TP (THE PATIENT’S WIFE): When we go to the ALS Center, he neurologist to whom the patient is referred must first deter- sees the physical therapist and . . . the occupational therapist mine that all alternative and treatable diseases, such as spon- . . . the nurse and . . . the doctor. They review his medicines dylitic myelopathy, Lyme disease, and multifocal motor neu- and test his strength. They tell him what’s on the horizon for ronopathy, have been excluded.22 Ruling out alternative ALS research. A social worker will meet with him and/or me. diagnoses may entail surgery, as in the case of Dr SP, or other The speech and communication therapist will meet with costly and invasive procedures.23,24 However, with an incur- us. . . . The dietitian meets with us and they weigh him. Also able fatal disease like ALS, if alternative plausible diagnostic the respiratory therapist measures his breathing. They’re very, options exist, we believe such efforts are justified.
Disclosing the Diagnosis
Interdisciplinary ALS Clinic
As McCluskey and colleagues25 noted, “imparting the diag- Specialized ALS centers and clinics can provide compre- nosis of ALS is a formidable task.” In their mail survey of 94 hensive care by addressing diverse issues ranging from purely patient-caregiver pairs, 50 patients, and 19 caregivers, only medical concerns to psychosocial and financial issues and 44% of patients and 52% of caregivers rated the physician’s advance planning.31,32 There are 76 multidisciplinary ALS manner of breaking the news as good or excellent. Addi- clinics throughout North America certified by voluntary dis- tional problems reported by participants in the survey related ease organizations, such as the ALS Association and Mus- to failure to discuss symptom management, ALS patient assis- cular Dystrophy Association (ALS Division). A compari- tance organizations, or clinical trials. As with any serious dis- son between multidisciplinary care and standard care in the ease, when the diagnosis is delivered, a relative or friend should Netherlands found that patients receiving multidisci- be present, and the physician should determine what the patient plinary care were more likely to receive adequate aids and already knows or expects before proceeding. Sensitivity and appliances and scored significantly higher in the domains compassion as well as a positive message are essential.26-30 of social functioning and mental health.33 Among those messages are that ALS is not contagious, that A US database of ALS multidisciplinary care was used to there are interventions to manage nearly all symptoms as they compare more than 6000 patients with 111 patients who evolve, and that a great deal of educational information is avail- received care in the community and recorded their experi- able. The patient and family should be assured that much can ences via a Web-based registry.32 Those not receiving mul- be done to provide comfort and care, that decisions will be tidisciplinary care received fewer symptomatic treatments jointly made, and that the physician will always be there for (eg, therapy for drooling received by 3% vs 32% in mul- them. Patients and accompanying family members or friends tidisciplinary clinics; treatment for pseudobulbar affect re- may not remember any details presented at this time, but they ceived 5% vs 24%). Patients cared for in the community re- never forget the tone and the underlying message of hope and ported higher rates of insensitive delivery of diagnosis (46% assurance of continued care rather than despair and aban- vs 2%).32 Overall, care in multidisciplinary clinics is asso- donment.30 BOX 1 provides some suggestions to guide these
ciated with enhanced quality of life by alleviating symp- discussions, which are based on our clinical experience.
The neurologist should schedule a follow-up meeting within Members of the multidisciplinary team may include a neu- a couple of weeks. At that time, discussion should focus on rologist expert in ALS care, ALS nurse specialist, physical 2007 American Medical Association. All rights reserved.
(Reprinted) JAMA, July 11, 2007—Vol 298, No. 2 209
Box 1. Words to Say Based on the Authors’ Clinical Experience
Presenting the Diagnosis: Breaking the News
Recommending Noninvasive Ventilation
“Considering everything, the most likely diagnosis is ALS [amyo- “Many assistive devices can greatly help your breathing, which trophic lateral sclerosis], although there are no laboratory tests left unassisted may decrease your energy levels and impede your sleep at night. One such device is a noninvasive positive- “ALS is often called Lou Gehrig’s disease in the United States, pressure ventilator. It includes an easy-to-use mask that fits on or motor neuron disease and is one of the neurodegenerative your face. It should increase energy and provide better sleep.” diseases, something like Alzheimer disease or Parkinson dis- Discussing Tracheostomy and
ease. It attacks healthy adults, causing progressive paralysis of Long-term Mechanical Ventilation
muscles of the body. Despite years of research, we still don’t “Eventually, you may need more assistance with your breathing.
know its cause or have a cure. This doesn’t mean we can’t do You could consider undergoing a tracheostomy and receiving long- anything. We can do a great deal about the disease at an ALS term mechanical ventilation (LTMV), a decision that will involve your whole family and that requires thoughtful evaluation.” “You can call me or any of my staff at any time. We will keep “With tracheostomy and LTMV breathing becomes easier, aspiration pneumonia becomes less likely, and survival is pro- Prognosis
longed, which may be important for those with specific life- “The prognosis varies from one individual to another. Roughly 50% of patients die within 3 to 4 years after the beginning of “As your disease progresses, you may reach a ‘locked in state,’ the symptoms. About 10% to 20% of patients live beyond 10 making communication impossible. Some patients who can speak years, and very few patients live beyond 20 years. A number of before the procedure are not affected, but others can’t speak after factors are associated with slow progression of the disease.” (It tracheostomy. Frequent mechanical suctioning is needed to remove is helpful to share a couple of factors associated with better prog- secretions. This essential procedure causes transient gag and dis- comfort. Very few patients have infection or bleeding. For those Discussing Percutaneous Enteral Gastrostomy
who use a high-pressure cuff on the airway, tissue damage of the Feeding (PEG)
trachea may rarely occur. The costs for home care and equipmentnot covered by insurance are high. Handling the necessary equip- “Nutritional care is so important. In fact, you may burn ment and performing procedures require a lot from caregivers. The more calories than before because you may use more equipment requires permanent placement of a tube, which is con- muscles to perform routine activities than before. You may tinuously connected to a machine, in the upper airway at your neck.
also have muscle cramps, stiffness, and twitching, all of Portable machines are available so that patients may go out.” which consume extra energy. Besides moving your body, “This is a decision that is best made long before you might your muscles store energy the body needs quickly. When suddenly need it so that your family knows your wishes. We muscles become wasted, they can’t store as much energy.
can give you a lot of information and lend you a video to give Weighing yourself weekly will help you monitor your body’s you a better idea of what the experience might be like. If you find that living with an LTMV is not acceptable, you have the “Obtaining nutrition through a feeding tube reduces eating legal right to discontinue it. You should have an advance di- time, which can take an hour or more. Some patients stop eat- rective informing us of the circumstances under which you don’t ing because they get so tired. Tube feeding provides all the nour- ishment you need and can even restore lost weight. This is im-portant because weight loss is associated with decreased Recommending Hospice Care
respiratory capacity and shorter survival.” “I wish I could use hospice service as early as possible, prefer- “PEG has its risks: infections at the site of the tube, disfig- ably as soon as the diagnosis of ALS is made, although the hos- urement of body shape, and diarrhea or constipation that pice rules do not provide for that. Hospice, which is covered may not always be transient. Tube feeding is cumbersome by Medicare, provides the best palliative care and comfort. At and with loss of hand movement requires an assistant at home services, include visits by a nurse, social worker, or chap- every meal. Deciding against tube feeding is okay. You won’t lain; some home health aide coverage; and 24-hour on-call nurs- starve without it. Your body will adjust and you won’t feel ing. We work with the hospice team because we are respon- hunger; however, many patients have said, ‘Wow, why sible not only for maintaining your function, quality of life, and dignity but also for making you comfortable.” therapist (to help manage spasticity), occupational thera- professional, gastroenterologist, interventional radiolo- pist, dietitian, speech pathologist (to explain and order com- gist, orthotist (to evaluate and select or fabricate personal- munication devices), pulmonologist or respiratory thera- ized limb braces) and prostho-odontist (to evaluate and fab- pist, social worker, patient service coordinator from voluntary ricate a palatal lift to improve nasal speech). Often, 5 or 6 disease organizations, research coordinator, mental health different specialists see the patient on the same visit. Dete- 210 JAMA, July 11, 2007—Vol 298, No. 2 (Reprinted)
2007 American Medical Association. All rights reserved.
rioration is often faster than the patient and family antici- Nutritional Care and Enteral Feeding. Good nutrition
pate, and professionals can help them to identify and ad- and hydration are essential for well-being and indeed, sur- just to these changes. Staff members can help patients to vival.51 Difficulty eating is often associated with weight loss obtain insurance coverage that is initially denied and to ne- and is a common problem for ALS patients. As swallowing gotiate the best possible care. Psychosocial and spiritual difficulty increases, each meal can take an hour or more and needs, such as anticipatory mourning, may be addressed by taking oral medication becomes problematic. In our expe- the social worker or the team member with whom the pa- rience, a PEG reduces this burden on both patient and care- tient or caregiver is particularly close. Once hospice is ini- giver, so more calories can be consumed with benefit to gen- tiated, pastoral counselors are available for home visits.
eral health and stamina. The American Academy ofNeurology Practice Guideline,52 using evidence-based medi- Progressive Nature of ALS: Symptom Management
cine, recommends that PEG be considered for those with DR A (THE TREATING NEUROLOGIST): Everything we recom- dysphagia and weight loss, although many neurologists re- mend is aimed at trying to do both [address quality as well as main reluctant.53,54 Ways to approach discussing PEG place- quantity of life]. Because he is aware of the importance of nutri- ment are provided in Box 1. A PEG can be most safely placed tion, [Dr SP] has been meticulous about weighing himself and when respiratory capacity is still greater than 50% of pre- not losing weight. A lot of the credit goes to his family. . . . they dicted.52 Although there have been no randomized trials to spend hours every day feeding him. . . . That’s why he has agreed demonstrate that PEG prolongs survival, malnutrition is an to getting a PEG tube, because that will . . . hopefully free up pre- independent risk factor for a worse prognosis in ALS.14,55 cious time for him and his family that he won’t need to spend eating. In a study of 55 patients, low body mass index substan- From the outset, patients should be reassured that many tially increased risk of death.51 Dr SP decided to have a PEG, solutions have been devised for the multiple problems that as reported in his physician’s interview.
may evolve. Symptoms that may develop in no particular or- Dyspnea. Shortness of breath occurs when the muscles
der include limb weakness,16 cramps,4 spasticity,36 pain,37 dys- of respiration become weak. It may be the presenting symp- arthria, sialorrhea,38 fatigue and insomnia,39 depres- tom or may develop years later, first occurring on exertion sion,10,40,41 fear and anxiety, involuntary emotional expression and then when lying down. Later it may cause or exacer- disorder,39,42 constipation, aspiration,43 and laryngospasm.44 bate insomnia. When respiratory difficulty begins to inter- Cognitive Impairment. Traditionally ALS was believed to
fere with sleep or forced vital capacity declines substan- spare memory and cognition,45 but as many as half of pa- tially (usually below 50% of predicted), NIV is usually tients with ALS may have at least mild cognitive impair- recommended.52 More recently, maximum inspiratory pres- ment.46 Disturbances in frontal lobar function have been re- sure56 or nasal sniff pressure57 has been found to be more ported in 28% to 48% of patients with ALS.47 Frontotemporal reliable than measuring forced vital capacity. In our expe- dementia (FTD) has been specifically associated with ALS.48 rience, nearly all patients agree to try NIV when advised to Its diagnosis, using the Neary criteria that include early loss do so. Sometimes patients with severe bulbar dysfunction of insight, and supportive diagnostic features such as mental cannot tolerate it while others leave installed equipment un- rigidity,49 is complicated by the range of disabilities due to ALS used. Longitudinal studies show that NIV can prolong life (eg, inability to speak or write). Although untreatable, FTD approximately 12 to 18 months58 and improves its quality is important to recognize because its presence may substan- by alleviating shortness of breath, improving sleep, and in- tially interfere with the patient’s capacity to make treatment creasing mental alertness and energy to pursue social ac- tivities.59 In a randomized trial, NIV lengthened the time that Although decisions to initiate supportive interventions to quality of life scores remained above 75% of baseline by at help the patient cope with symptoms of the disease are nearly least 3 months compared with those receiving standard care.58 always straightforward, management of nutritional insuffi- In an observational study, scores on a standardized quality ciency with enteral feeding and of respiratory insufficiency with of life measure increased as much as 25% after initiation of noninvasive ventilation (NIV) or LTMV are more compli- NIV for 16 patients, despite disease progression, compared cated, more enduring, and can be more controversial. Pa- with a control group of 11 patients who declined NIV.60 Later tients may want to postpone percutaneous endoscopic gas- in the course of the disease, treatment to extend life re- trostomy (PEG) or they may refuse it outright. Although, in quires tracheostomy and LTMV, which sustains respiration our experience, most patients accept NIV, LTMV raises more indefinitely, although disease progression continues un- issues. The rapid progression of ALS can make adjusting to abated.61 LTMV is discussed in greater depth below.
new treatment decisions difficult for patients and families, butdelay in advance care planning can make some interventions Impact of ALS on Informal Caregivers, Family, and Friends
less effective or no longer possible (eg, PEG placement after TP (THE PATIENT’S WIFE): This family of ours pulled together respiratory deterioration). The challenge for the physician is and is really strutting its stuff. These children are wonderful. to help ensure that the patient receives desired treatment but We’re a very close family. SP set us up that way. . . . We’ve pretty not exert undue pressure before the patient and family are ready.
much put our lives on hold and SP is our primary focus. 2007 American Medical Association. All rights reserved.
(Reprinted) JAMA, July 11, 2007—Vol 298, No. 2 211
care get more onerous at the same time that it becomes less Box 2. Approximate Costs of Various
safe to leave them unattended.64 The household requires dras- Interventions for Patients With Amyotrophic
tic changes, and, if financially able, family members may have Lateral Sclerosisa
to stop working to provide care. Ironically, the insured familymember may be unable to stop working because family health Costs Related to Percutaneous Enteral Gastrotomy
insurance is job related. Despite their own need for support, caregivers may be unable or unwilling to attend support groups or counseling so that they may stay home with the patient.
Even with paid assistance, ALS caregivers are estimated to spend an average of 11 hours per day with the patient.65 Food (6 cans of 8-ounce Ensure Plus): $260 per month Emotional Burden. In studies involving ALS patients and
caregivers, factors independently related to caregiver’s per- ceived burden are the degree of the patient’s disability and dis- Respiratory Aids
ease duration (themselves correlated), which is not necessar- ily alleviated by participation in support groups or number of available substitute caregivers.63 In another study, a global mea- sure of caregiver distress was found to increase over time andto be exacerbated by the patient’s emotional lability, number of other dependents, and negative (critical) feedback from so- cial network members.66 Caregivers who reported greater bur- den also reported more depressive symptoms, although frank Mobility Aids
clinical depression was the exception.63,67 Impact of the Patient on the Caregiver. In contrast to pa-
tients with Alzheimer disease, whose caregivers are the most Low-tech power wheelchair: $5000 to $8000 studied, ALS patients retain the capacity to appreciate care- High-end power wheelchair: $10 000 to $40 000 giving, to express affection, and to actively participate in their lives and relationships even if mute or immobile. Thus, care- Communication Aids
giver burden can be profoundly offset when satisfaction withcaregiving is high, and this is often related to the apprecia- tion the patient provides to the caregiver.68,69 Financial Aspects and Burden of Care. Out-of-pocket
costs for families with ALS patients are great. Costs for me- Medication
chanical ventilation and 24-hour care range up to $200 000
a year (BOX 2).61,70 For those without pharmaceutical cov-
Riluzole, 50 mg taken orally twice daily: $550 per month erage, out-of-pocket costs for riluzole are about $550 per Home Assistance
month to achieve an average 2 additional months of life.
The goal of preserving the patient’s quality of life and au- Licensed practical nurse: $35 to $45 an hour tonomy at home for as long as possible requires expensive equipment and home renovation including power wheel-chairs, rotating beds, powered seat lift recliner chairs, a me- aThese costs are based on 2007 information from vendors in the chanical lift, specialized computer equipment, a vehicle New York City area. Cost varies by location, as does insurancecoverage for the listed items.
equipped with hand controls, and a van equipped with rampand lift. Although some items are covered by insurance, out-of-pocket expense can amount to thousands of dollars per year.
WP (THE PATIENT’S SON): I’m thankful my family was finan- Box 2 presents costs of equipment and services provided by cially secure enough that I could move home and not have to work vendors in the New York City area. The extent of insurance another job to help support my family. I can spend my time not coverage depends on the patient’s policy. It is crucial to have only helping him but also spend time just being with him. an experienced ALS team work with patients and their fami- Caregiving Responsibilities. Although caregivers of all se-
lies to provide advice regarding effective use of their insur- riously ill patients with medical conditions or dementia face ance coverage. The Muscular Dystrophy Association and ALS complex demands,62 perhaps no caregivers are more chal- Association can provide limited support.
lenged than those of ALS patients. With progressive disease,patients with ALS need increasing levels of assistance with Professional Staff Support
nearly all activities of daily living.63 In addition, their ability DR A: The one thing I want to emphasize is that working with to communicate diminishes and the physical demands of their these patients has always been hard . . . in this line of work where 212 JAMA, July 11, 2007—Vol 298, No. 2 (Reprinted)
2007 American Medical Association. All rights reserved.
every person with the disease will die eventually. . . . I also reallyvalue the support groups that we have with our staff. . . . I feel Box 3. Triggers for Initiating Discussion About
like I’m in here for the long haul and I want to pace myself, End-of-Life Issues in Patients With Amyotrophic
Lateral Sclerosis Including Prognosis, Advance
Dr A highlights the staff stressors and coping strategies Directives, and Hospicea
to avoid burnout. It is important to recognize one’s own vul- The patient or family asks—or opens the door—for end- nerability to burnout and to recognize signs of stress.71 Team of-life information and interventions (elicited or spontane- philosophy, team support, and collective coping strategies are significant factors in maintaining the individual staff mem- Severe psychological, social, or spiritual distress or suffering ber’s sense of competence and motivation. Those who spe- Pain requiring high dosages of analgesic medications cialize in caring for patients with ALS often know their pa-tients for longer than palliative care specialists. Thus, when a patient with ALS dies, the loss experienced by ALS pro- Dyspnea or symptoms of hypoventilation or a forced vital Loss of function in 2 body regions (regions include bulbar, Tracheostomy and LTMV
DR A: A lot of times patients don’t have to make that decision aBased on consensus among 20 ALS experts.1 [whether to prolong life by means of LTMV] . . . For most of mypatients who have had to make that decision, it has been easierbecause at that point they are so disabled by their disease thatthey’re just ready to go. It’s usually not an agonizing decision.
ferent treatment centers ranged from 1.4% to 14% and was In ALS, respiratory capacity eventually fails, even with related to physicians’ own attitudes toward its use.76 How- NIV. Patients and families are faced with deciding about ever, not all tracheostomies are planned or voluntary. In fact, LTMV intervention, and advance planning is essential to pre- Moss et al77 speculate that only a minority of those receiv- clude unwanted emergency tracheostomy in a crisis. With ing LTMV makes this choice in advance, and in their study LTMV, patients eventually may reach a “locked in” state in of 50 patients assisted by LTMV, fewer than half had done which they cannot communicate at all.15 This may be a cir- so. In a German survey, 66% of patients receiving LTMV cumstance in which patients would desire discontinuation had emergency intubation, and 81% did not give informed of ventilation but unless clearly specified in advance, deci- consent for the procedure.78 In a longitudinal study of 80 sions about how to proceed become highly problematic.
patients with ALS whose expected survival was less than 6 Prevalence of LTMV is influenced by treating physician months and who were patients in our own clinic, 14 of 80 attitudes, insurance coverage (national or private), and cul- (18%) electively received LTMV, an unusually high rate that tural standards (eg, whether the physician, the patient, or may be partly due to their late stage of illness at study en- the family makes the decision) and so varies substantially try.70 After tracheostomy, 50% identified circumstances in both between and within countries.73 In North America, re- which they would wish to discontinue treatment, such as fusing life-sustaining measures (like LTMV in the context having to go to a nursing home, being locked in, or becom- of respiratory failure) is both legal and considered respect- ing demented. In a prospective study, Albert et al79 found ful of the patient’s right to choose according to the ethical that patients with ALS had clear preferences for or against principle of autonomy, as is the right to discontinue such tracheostomy and LTMV well in advance of the point of cri- sis and that these preferences were related to actual outcomes.
A US study (n=1458) found a rate of LTMV use of 2%,34 Although no research has explored the tradeoffs of choos- while in an English sample (n=50) the rate of LTMV was 0 at ing or refusing LTMV by ALS patients, preferences regard- a time when national health insurance did not cover home ven- ing acceptance or rejection of treatment by seriously ill older tilation,74 and 3% in a German study of 121 patients.74 In con- patients have been studied in hypothetical scenarios.80 Al- trast, in a 2004 study in Japan, the rate of LTMV was 27%.31 though 99% would choose a low-burden treatment with re- The rate in Japan appears to be increasing, perhaps related to turn to current health status, most preferred death over sur- financial considerations (the Japanese government covers costs vival when their condition after treatment would entail severe of LTMV), physician support for LTMV, social pressure from functional or cognitive impairment, even when the treat- the Japanese ALS Association, and differences in cultural atti- ment was not high burden. In another study,81 community tudes about “truth-telling” and death. Historically, Japanese residents who ranged from well to terminally ill were given patients and families expected the doctor to determine treat- 5 hypothetical health states and asked about life- ment strategies and decisions, at least until quite recently.31,75 sustaining preferences. Across groups, treatment was re- Rates of LTMV also vary considerably within country. A fused when the hypothetical health outcome was regarded study conducted in the early 1990s in the United States found as worse than death. In both studies, defining states worse that the percentage of ALS patients receiving LTMV at dif- than death was a subjective judgment determined by per- 2007 American Medical Association. All rights reserved.
(Reprinted) JAMA, July 11, 2007—Vol 298, No. 2 213
One of our patients continued to go to the opera in her power Box 4. Medicare Hospice Entry Criteria for
wheelchair and portable machine, along with her 24-hour Patients With Amyotrophic Lateral Sclerosis
registered nurse, although she was totally paralyzed and mute.
Communication remains feasible for varying amounts of time, Patients Must Meet at Least 1 of the Following Criteria
using laser-assisted pointers with computers, alphabet boards, 1. Critically impaired breathing capacity as demonstrated and other assistive devices. In late stages, “yes” and “no” can be signaled with eye gaze or blinks.36 Forced vital capacity less than 30% of normal Disadvantages concern both the patient and family care- givers. Although the patient is alive, the disease progresses re- lentlessly. With LTMV, frequent (in some cases hourly) me-chanical suctioning to remove secretions is essential, and it 2A. Patient should demonstrate both rapid progression ofamyotrophic lateral sclerosis and critical nutritional im- can cause gagging and distress. The costs of home care are im- mense, and family responsibilities and burdens are great. Care-givers of patients dependent on invasive ventilator assistance Rapid progression: independent ambulation to wheel-chair or bed-bound status report substantial levels of distress, and, in the few studies ofthis population, caregivers were more likely than patients to Progression from normal to barely intelligible speech express regret about having chosen LTMV.70,84 Ideally, the patient, family, and physician have dis- Needing major assistance by caretaker in all activities of cussed end-of-life issues and preferences, and the patient’s wishes are known well in advance of a crisis.81 SP provides 2B. Critical nutritional impairment as demonstrated by all a best-case scenario. He has formulated a plan and has told his family and his physician how he wants his death to oc- Oral intake of nutrients and fluids insufficient to main- cur. He has chosen not to pursue tracheostomy and LTMV.
The frequency with which withdrawal of LTMV occurs is unknown. Some patients may ultimately require palliative se- dation.85 For patients who withdraw LMTV or choose not tohave it, palliation becomes the sole focus of interventions, in- Absence of artificial feeding methods sufficient to sus-tain life but not for relieving hunger cluding treating pain and managing other symptoms, such asdyspnea, fear, anxiety, sialorrhea, and constipation.
3. Patient should demonstrate both rapid progression ofamyotrophic lateral sclerosis and at least 1 life-threatening End-of-Life Care and Referral to Hospice
DR SP (THE PATIENT): When I become dependent on BiPAP (non- invasive ventilation or NIV) most of the day and night, one night I will go to bed without the BiPAP on and have family around me. TP (THE PATIENT’S WIFE): It sounds easy, but I don’t know Inability to maintain sufficient fluid and caloric intake how easy that’s going to be.
with 10% weight loss during past 6 months or serum Discussing the fatal nature of ALS and end-of-life deci- sion making with patients and families is not easy. Al- Specific information may be found at though the general issues are best raised soon after diagno- /cgi-bin/bookmgr/bookmgr.exe/BOOKS/M984/?FRONT sis, the actual decision regarding hospice should be faced
before respiratory capacity declines substantially. BOX 3 sum-
marizes triggers that can lead to discussion of these issues.
ceived values and beliefs. Meier and Morrison82 noted edi- BOX 4 presents the Medicare criteria for hospice referral.
torially, “When desperately ill patients and their families opt Home hospice is particularly helpful for ALS patients in for desperate treatments, they should also be asked under late-stage illness because most are essentially housebound what circumstances death would be preferable to life with due to loss of mobility, and the multiple services hospice severe impairment and whether treatments should be dis- provides at home would not otherwise be available.86 How- continued if those circumstances occur.” ever, home hospice is not generally an option for patients In brief, the overriding advantages are that LTMV secures who have decided to seek tracheostomy or LTMV or who the airway, prevents or reduces aspiration pneumonia, and by have no informal caregiver living with them.
providing automated mechanical breathing, prolongs life(Box 1).15 Some patients continue to feel that life has pur- Wish to Die and Hastened Death
pose and meaning, and they can engage in social, religious, Patients with ALS may be more likely to consider a has- recreational, intellectual, and other activities even when they tened death than other patients,87 although the large ma- lose all mobility and can move nothing except their eyes.70,83 jority neither request nor act on such a consideration. Has- 214 JAMA, July 11, 2007—Vol 298, No. 2 (Reprinted)
2007 American Medical Association. All rights reserved.
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Financial Disclosures: Dr Mitsumoto reported receiving an honorarium from Avanir
sulting from misdiagnosis of early amyotrophic lateral sclerosis. Muscle Nerve. 2006; Pharmaceuticals for a scientific advisory meeting in 2006. He reported being a con- sultant for Eisai Pharmaceutical Co from 2005 to 2007. He reported receiving a 24. Yoshor D, Klugh A, Appel SH, Haverkamp LJ. Incidence and characteristics of
research grant for a clinical trial at Columbia University from Aeolus. He also re- spinal decompression surgery after the onset of symptoms of amyotrophic lateral ported receiving education grants to Columbia University from Sanofi-Aventis, Ethena sclerosis. Neurosurgery. 2005;57(5):984-989.
Diagnostic and Bioscrips. Dr Rabkin reported no financial disclosures.
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Funding/Support: This project was partially supported by Muscular Dystrophy As-
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2007 American Medical Association. All rights reserved.
Web Resources for Amyotrophic
Lateral Sclerosis

Patient Guides
Family Caregiver Alliance
Mitsumoto H, Munsat TL, eds. Amyo- trophic Lateral Sclerosis. Patient and Family Guide to Management and Care.
ElderCare Online
abling illnesses. It also provides infor- Amyotrophic Lateral Sclerosis. New prove quality of life for the caregiver as National Alliance for Caregiving
Everyday Life With ALS: A Practical Today’s Caregiver
Guide. Tucson, AZ: Muscular Dystro- ily caregiving. It offers support to fam- WEB SITES FOR PATIENTS
Social Security Disability Planner
Muscular Dystrophy Association National Family Caregivers
qualify for benefits, outlines the appli- Medicare Rights Center
ALS Association
health care information and assistance for lic policy related to ALS, includes infor- ties receive good, affordable health care; patients, families, caregivers, and health cerns of family caregivers; and offers in- care professionals, and discusses current dividuals the opportunity to join a care- research and donation opportunities.
tact. The organization’s goal is to assist 2007 American Medical Association. All rights reserved.
(Reprinted) JAMA, July 11, 2007—Vol 298, No. 2 E1




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