predictors and course of elective long‐term mechanical ventilation: a prospective study of als...

ORIGINAL ARTICLE

Predictors and course of elective long-term mechanical ventilation: Aprospective study of ALS patients

JUDITH G. RABKIN1, STEVEN M. ALBERT2,3, TOBY TIDER2, MAURA L. DEL BENE4,

ITA O’SULLIVAN5, LEWIS P. ROWLAND3 & HIROSHI MITSUMOTO3

1Department of Psychiatry, 2Gertrude H. Sergievsky Center, Department of Neurology, Columbia University Medical Center,3Eleanor and Lou Gehrig MDA/ALS Research Center, Department of Neurology, Neurological Institute, Columbia

University Medical Center, 4Department of Neurology, New York University School of Medicine, and 5School of Nursing,

Columbia University Medical Center, New York

AbstractWe sought to characterize ALS patients who opt for tracheostomy and long-term mechanical ventilation (LTMV) andcompare them with respect to medical, psychiatric, and psychosocial measures to patients who declined tracheostomy anddied. We studied 72 ALS patients who were identified as hospice-eligible. They were assessed monthly until the endpoint ofdeath or tracheostomy. LTMV patients continued to be followed for up to 55 months. The spouse or other caregiver wassimilarly interviewed and followed. Medical and psychiatric evaluations were conducted, in addition to self-reporteddepressive symptoms, future orientation, attitudes about hastened death, religious beliefs, and quality of life. Globalcognitive capacity was assessed by caregivers. Fourteen patients chose LTMV; 58 died without LTMV. At study entry,those who later chose LTMV were younger, more had young children, had more education, and higher household incomeson average. Although their physical conditions were similar, they reported higher levels of optimism including belief inimminent cure, and more positive appraisals of their ability to function in daily life, their physical health and overall lifesatisfaction. At study entry, none who later chose LTMV were clinically depressed, compared to 26% of those who laterrefused LTMV, and their mean scores on the Beck Depression Inventory were in the ‘not depressed’ range while the meanfor patients who later died was in the ‘probable depression’ range. Fourteen percent of patients who later chose LTMV werereported by caregivers to have had at least mild cognitive problems, compared to 49% of those who later died. After anaverage of 33 months on LTMV, only about half retained high levels of optimism and enjoyment of daily life, independentof residence (home vs. facility). Two patients expressed interest in hastening death but none had asked to terminateventilation despite disease progression. However, half identified future circumstances that would render life intolerable. Atlast contact with caregivers, only one LTMV patient was reported to have major cognitive impairment. While reportingsubstantial emotional burden after LTMV, most but not all spousal caregivers continued to express satisfaction with care-giving. Our findings suggest that the choice of LTMV was not about desperation (although it may involve unrealisticexpectations of cure by some), ignorance, or inability to make wishes clear during a chaotic dying period. Rather, LTMVchoice was consistent with a sustained sense that life was worth living in any way possible, at least for some time and withincertain boundaries. ALS clinicians will need to recognize this motivation and provide appropriate clinical education to bothpatient and family.

Key words: ALS, LTMV (long-term mechanical ventilation), quality of life, depression, cognitive capacity

Introduction

When end stage ALS is reached and respiratory

failure is imminent, patients ultimately must choose

between long-term mechanical ventilation (LTMV)

or death (1). While tracheostomy is most often

considered by patients with bulbar onset who retain

adequate limb strength (2), most patients refuse this

option, accept comfort care, and die.

The prevalence of LTMV varies substantially both

within and between countries, influenced by treating

physician attitudes, insurance coverage (national or

private), and cultural standards (whether the physi-

cian, the patient or the family makes the decision)

(3). In a cross-national ALS study, the rate of

LTMV was 3% in a German sample of 121 and zero

in an English sample of 50 at a time when national

health insurance did not cover home ventilation for

ALS patients (4). In contrast, the Japanese rate is

nearly 45% (5). Japan is unusual in that the

physician may be the sole decision maker regarding

life support (3), even though discontinuation of

Correspondence: J. Rabkin, New York State Psychiatric Institute, Unit 51, 1051 Riverside Drive, New York, NY 10032, USA. Fax: 212 543 5326. E-mail:

[email protected]

Amyotrophic Lateral Sclerosis. 2006; 7: 86–95

ISSN 1748-2968 print/ISSN 1471-180X online # 2006 Taylor & Francis

DOI: 10.1080/14660820500515021

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ventilation is illegal. In addition, nearly all expenses

are covered by the government, which probably

contributes to this extremely high rate (5).

A study conducted in the early 1990s in the

United States found that the percentage of ALS

patients on LTMV at different treatment centers in

the same state varied from 1.4% to 14% and was

related to physicians’ personal attitudes toward its

use (6). While there are few data, it has been

estimated that in the United States, between 5% and

8% of ALS patients are placed on LTMV, not all of

them voluntarily (7). In fact, Moss et al. (8)

speculate that only a minority of those receiving

LTMV makes this choice in advance, and in their

study of 50 patients on LTMV, fewer than half had

done so. Perhaps the most representative estimate of

voluntary tracheostomies comes from the North

American Patient CARE Database, which now

includes 5500 patients at all stages of illness, of

whom about 35% were alive. About 3% had opted

for tracheostomy, a rate unchanged since the

database commenced in 1996 (9).

Patients who voluntarily choose LTMV may

continue to experience enjoyment, companionship,

and a sense that life is worth living. In a study of 18

patients on LTMV who retained some communica-

tion skills, compared to 126 ALS patients who had

not reached the point of respiratory insufficiency, the

LTMV patients were not distinguishable in terms of

psychological distress, hopelessness or perceived

stress (10). In another study of 19 ALS patients

with LTMV, 17 of the patients and 16 of the

caregivers said they would make the same decision

again (11).

Although LTMV can add months or years of life,

progressive paralysis may eventually result in a

‘locked-in’ state. In a Japanese study, 70 ALS

patients with LTMV were followed for 15 years

(12). Among those on LTMV for more than five

years, 51% had little or no way to speak or

communicate in any other way. The most common

cause of death was pneumonia, followed by cardi-

ovascular causes.

Home care of patients on LTMV is challenging.

The financial cost is great, with estimates ranging up

to $200,000/year (5) including 24-h care. If home

assistance is obtained through an agency, employ-

ment of a registered nurse is required to protect

against medical liability claims in cases of an

accident (11). The costs of home care and the

necessary equipment are seldom entirely covered by

health insurance in the United States (13). Demands

on both patient and caregivers are great; constant

vigilance is required to prevent accidents, and

quality of life may be substantially diminished for

both families and patient (5).

In the sparse literature about successful home

LTMV, factors that may be important in this choice

include medical status and communication ability,

psychiatric status and outlook, finances, and avail-

ability of supportive family or other caregivers (11).

In one report, religious beliefs influenced choice of

LTMV (14).

We found no prospective studies of ALS patients

who opted for tracheostomy voluntarily. To help fill

this gap in the literature, we conducted the current

study. We set out to determine whether ALS

patients with respiratory failure who choose tra-

cheostomy differ from those who refuse, in a

prospective study of hospice-eligible patients. A

second question in this exploratory study concerned

the effects of passage of time on the mood and

outlook of patients after tracheostomy. The domains

of interest included sociodemographic variables,

medical, cognitive and psychiatric status, future

orientation; social support, religious or spiritual

faith, and perceived caregiver burden.

Methods

Sample

Despite efforts to recruit patients from multiple sites,

95% were enrolled in the Eleanor and Lou Gehrig

MDA/ALS Center at Columbia University (New

York). The clinic coordinator identified prospective

participants whose forced vital capacity was v50%,

a value associated with a life expectancy of less than

six months (15). Eligible patients were non-

demented, spoke English, were not using mechanical

ventilation at baseline, could communicate at least

‘‘yes’’ and ‘‘no,’’ and lived within a 3-h driving

radius of the medical center. Caregivers identified by

patients were mostly spouses, parents or adult

children. A few were friends involved in the patient’s

care and management. The study was conducted

between January 2000 and June 2004. Nine LTMV

patients and their caregivers were seen again in

January 2005, with final follow-up telephone calls to

caregivers in July 2005.

Measures

Caregivers. To reduce patient burden, only the

caregivers were asked for demographics, current

use of assistive devices, and financial information.

Caregivers were also asked about the patient’s

earliest symptoms to identify bulbar or spinal

onset, and ability to perform activities of daily

living as well as the ALS Functional Rating Scale

described below. Given reports of frontotemporal

dementia in ALS patients (16), caregivers were also

asked to assess patients’ cognitive function in three

areas: overall mental status, comprehension, and

memory, on 4-point scales ranging from 05normal

to 35significant impairment (17). Emotional lability

was also rated on a 4-point scale. Word finding

problems were not assessed.

Elective LTMV: predictors and course 87

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Patients. In addition to some open ended questions,

the following standardized measures were admi-

nistered at each visit except where noted, with short

forms used when feasible. More detailed scale

descriptions are given in Rabkin et al (18).

Patient Health Questionnaire (PHQ)19. Because of

limitations in communicating, interactive queries

and follow-up probes needed for the Structured

Clinical Interview for DSM-IV (SCID), the gold

standard diagnostic evaluation in psychiatry, are

often not feasible for these patients. We therefore

used the PHQ-9; it assesses the 9 DSM-IV criteria

for major and minor depressive disorders. Even

patients who communicate only with eye movements

were able to respond.

Beck Depression Inventory (BDI-PC)20. This is a 7-

item abbreviated version of the 21-item BDI, a self-

report screen used to assess severity of depressive

symptoms. Scores below 4.0 signify absence of

depression, while scores of 4+ are consistent with

depressive disorders. The 21-item standard form

was administered to caregivers on all occasions (21).

Beck Hopelessness Scale (BHS)22. We used a 10-item

short form to assess outlook about the future, e.g. ‘‘I

look forward to the future with hope and enthu-

siasm’’ and ‘‘When I look ahead to the future, I

expect I will be happier than I am now’’. It is usually

used as a measure of psychopathology associated

with depression, but in ALS it may serve rather to

assess realism vs. denial. In our report, total scores

are doubled to approximate full scale scores for

purposes of comparison with standardized cut-offs.

Holland Systems of Beliefs Inventory23. This is a 15-

item self-report trait measure with higher scores

indicating belief in a sustaining role of religion and

spirituality.

Schedule of Attitudes toward Hastened Death

(SAHD)24. This 20-item true/false scale includes

items designed to assess the wish to live, for

example, ‘‘Because my illness cannot be cured, I

would prefer to die sooner rather than later’’. The

total score was the sum of items, and ranged from 0

to 20.

Quality of Life Enjoyment and Satisfaction

Questionnaire (QOL)25. We used the 15-item short

form that inquires about satisfaction with various

domains of daily experience. In addition to a total

score, we also examined two items separately: overall

well-being and satisfaction with life.

Manne Perceived Partner Support Scale 26. We used a

modified version of a scale described by Manne et al.

One subscale inquires about frequency of positive

behaviors (e.g. ‘‘let me know he/she would always be

around if I needed assistance’’) and one regarding

negative behaviors (‘‘seemed not to enjoy being

around me’’). Total scores were 5–20 on the positive

scale and 7–28 on the negative scale.

ALSFRS-R27. This well validated 12-item global

scale of ALS motor dysfunction is widely used in

research and clinical settings. Lower scores signify

greater disability.

Visual Analog Scales (VAS). We included from the

outset 13 items covering the major domains of

interest from the foregoing scales that could be used

throughout the study even if progressive impairment

in communication later precluded response to the

more lengthy scales. The respondent was asked to

rate severity or amount on a 10-point scale ranging

from ‘absent or not at all’ to ‘extremely’. Both

positive and negative states were queried.

Procedures

After the clinic coordinator identified potential

participants, she called to describe the study and

seek permission for the research team to make

contact. The principal investigator then called for

further explanation and obtained consent to give the

patient’s name to an interviewer. At the initial

interview, informed consent was obtained from both

patient (all of whom were able to communicate at

study entry) and caregiver. All participants were

assured that their responses were strictly confiden-

tial. Monthly interviews were scheduled at the

patient’s convenience and almost always in the

home, and continued until patients met a study

endpoint of death or tracheostomy. Once on LTMV,

interviews were conducted every three months with

patient and caregiver. Approximately six months

after the formal study ended, they were again

interviewed in person, and six months later follow-

up telephone calls with the caregiver provided the

final update on status. All LTMV patients and

caregivers agreed to participate in these final inter-

views. The research protocol was approved by the

New York State Psychiatric Institute – Department

of Psychiatry Institutional Review Board.

Statistical analyses

Categorical data were analysed using x2, and t-tests

were used for comparison of continuous variables.

Pearson correlations were used to analyse relation-

ships between measures. Logistic regression was per-

formed to assess the independent effects of variables

that showed a univariate association with the out-

come of LTMV. All tests were 2-tailed. Since our

sample was small, we looked for trends rather than

definite results, and therefore used pv.10 to report

88 J.G. Rabkin et al.

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statistical significance. Depending on patients’ fati-

gue and communication limitations, not all assess-

ments were completed at every occasion.

Results

Sample

In our original sample of 80 patients (see 18: Rabkin

et al., 2005), 72 reached a known endpoint

(tracheostomy or death) by the end of the original

study, and constitute the group in this report. Those

excluded were five dropouts and three patients who

were still alive as of July 2005. Of the 72 patients

who reached an endpoint, 14 voluntarily chose

mechanical ventilation and 58 died. Deaths included

one patient who was intubated in an emergency

room despite explicit DNR orders; she was extu-

bated at her request and died within one week.

Another patient requested intubation for the first

time as she was actively dying. One patient in the

LTMV group who had always intended to have a

tracheostomy chose to be extubated within a week

after he endured several cardiac arrests, resuscita-

tions and implantation of a pacemaker.

For the 72 patients who reached study endpoint,

mean age at enrollment was 63 years (range 31–85,

SD513), 47% were females, 87% were white, 57%

had at least some college education, and 64% had

caregivers who were spouses. Catholicism was the

most common religious affiliation (44%).

Those who eventually died were followed monthly

for a mean of 3.6 visits (range 1–18), while those

who chose LTMV were followed for an average of

6.8 visits before tracheostomy (range 1–18). Eleven

of the 14 tracheostomy patients and their caregivers

were interviewed at least once after the procedure.

Baseline characteristics

Sociodemographic status. The strongest predictors of

LTMV were patient age and having children under

21 years of age. Patients who later chose LTMV

were on average significantly younger (Table I).

Each additional year of age was associated with a

6% reduction in probability of choosing LTMV.

Among patients under age 50, 46% chose LTMV

compared to 23% of patients aged 50–69 years and

4% of those aged 70+. Half of the patients who chose

LTMV had children under 21 years of age,

compared to 10% of those who died without

LTMV. Having young children increased the

likelihood of LTMV nearly five-fold. In a logistic

regression analysis, age and having young children

were the strongest independent predictors of

outcome. In addition, more patients who chose

LTMV had annual household incomes over

$100,000. At study baseline, 40% of those who

later died and none in the LTMV group were on

home hospice.

Medical status. Mean ALSFRS scores at study

baseline were equivalent (24.0 +/– 8.0 for the

potential LTMV group vs. 24.2 +/– 7.2 for the

others) (Table II). All had a forced vital capacity

under 50%. The prevalence of severe bulbar

Table I. Baseline features, by choice to use long-term mechanical ventilation.

Baseline indicators

Patients who died without

LTMV (n558)

Patients who opted

for LTMV (n514)

Patient sociodemographics

Age, yr¡SD 65.3¡13.0 51.4¡12.3***

Gender, % male 53.4 50.0

Education, whigh school, % 51.9 79.3

Annual household income, %

v$59,000 53.8 30.8

w$100,000 11.5 38.5

Private health insurance, % 79.2 83.3

Medicaid, % 17.0 7.1

Children, under age 21 10.0 50.0**

Patient social support

Number visitors prior week (mean) 4.5 8.0+Living arrangement, %

With spouse 62.2 69.4

With other family 15.1 16.4

Nursing home 1.9 7.1

Alone, or with paid attendants 20.8 7.1

Religion, %

Catholic 46.5 35.7

Protestant 17.2 14.3

Jew 24.1 21.4

Other/None 12.2 28.6

+pv.10, **pv.01, ***pv.001.


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symptoms at baseline did not differ among patients

who later opted for LTMV and those who did

not: 21.4% vs. 21.8% lacked speech; 28% vs. 31%

had excessive salivation and 36% vs. 32% had

gastrostomy. Similarly, the prevalence of bulbar

symptoms was not significantly different in care-

giver reports of symptom onset. For example, speech

impairment was reported as the first symptom of

ALS in 17% of those who ultimately chose LTMV

and 11% in those who did not.

Despite comparable symptom severity at baseline,

patients who later opted for LTMV appraised

symptoms and their effects more favorably than

patients who refused LTMV. On VAS ratings,

patients who chose LTMV were less weary from

the disease and on the Endicott Q-LES-Q reported

significantly greater satisfaction with their ability to

function in daily life, with their physical health, with

household activities, and with overall well-being and

life satisfaction pv.05 for all; see Table II). Patients

who chose LTMV reported a greater wish to live and

less interest in hastening death.

Cognitive status. Caregivers were asked to appraise

patients’ overall mental status, memory, and

emotional lability. Although not statistically sig-

nificant, 18% (10/55) of those who later died and

none of those who later chose LTMV were reported

to have ‘‘ some diminished mental capacity’’ or

‘‘marked slowing’’ at study entry. Twenty-six

percent (14/55) of patients who later died and 7%

(1/14) who later chose LTMV were reported to have

difficulty with comprehension of TV or reading

material. Thirty-one percent (17/55) of patients who

later died and 7% (n51) who later opted for LTMV

had mild memory problems. These numbers

overlap: cumulatively, 49% (27/55) of patients who

later died were said to have at least one cognitive

problem, compared to 14% (2/14) of those who later

had tracheostomies (x255.55, 1df, p50.018).

Table II. Baseline ALS severity, mental health, and well-being, by choice to use long-term mechanical ventilation.

Baseline indicators


LTMV (n558)

Patients who opted for

LTMV (n514)

Disease Status

ALSFRS, mean¡SD 24.2¡7.2 24.0¡8.0

No useful speech, % 21.8 21.4

Marked salivation problems, % 30.9 28.4

Tube feeding, exclusive/supplementary, % 31.5 35.8

Endicott Quality of Life Indicators, mean¡SD

Ability to function in daily life 2.8¡1.2 4.0¡0.8**

Sexual drive and interest 2.7¡1.5 3.1¡1.8

Satisfaction with economic status 3.5¡1.1 3.3¡0.5

Satisfaction with living situation 4.0¡0.9 3.8¡1.0

Ability to get around 3.0¡1.3 2.5¡1.5

Satisfaction with physical health 2.6¡1.2 3.6¡0.7*

Satisfaction with mood 3.4¡1.1 4.0¡0.8

Satisfaction with household activities 2.9¡1.4 3.7¡0.5*

Satisfaction with social relationships 4.0¡0.8 4.3¡0.7

Satisfaction with family relationships 4.3¡0.8 4.3¡1.0

Satisfaction with leisure activities 3.4¡1.3 3.8¡1.3

Overall sense of well-being 3.5¡1.3 4.1¡0.4*

Overall life satisfaction 3.6¡1.1 4.0¡0.1*

Visual Analog Scales (VAS), mean¡SD

Wish to live despite ALS 7.7¡3.1 9.8¡0.6***

Weariness from ALS 6.8¡3.1 4.7¡3.2*

Optimism 5.3¡3.0 7.2¡2.4*

Energy 5.1¡2.5 6.4¡2.1+Suffering 4.1¡2.6 3.1¡3.0

Control over ALS management 3.5¡3.0 4.3¡3.3

Anger 3.2¡2.8 4.0¡3.0

Perceived burden of care on family 6.8¡2.8 7.4¡2.6

Pain 2.6¡2.2 2.1¡2.1

Depression 3.8¡2.8 2.3¡1.8*

Interest in hastening death 3.6¡3.4 1.0¡0.0***

Attitudes toward hastened death, mean¡SD 5.9¡4.6 2.2¡0.8**

Religion and spirituality, mean¡SD

Comfort in religion, VAS 6.0¡3.9 4.9¡3.8

Comfort in spirituality, VAS 6.7¡3.6 5.6¡3.9

Holland: Religious beliefs 42.6¡14.7 42.0¡17.2

+pv.10, *pv.05, **pv.01, ***pv.001.


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However, only 15% of the problems were more than

mild, all in the group who later died, so that most

were presumably not clinically significant.

Social support. Patients who later chose LTMV had

an average of eight visitors/week (including relatives

and friends), compared to 4.5 visits/week for those

who later died. In terms of patients’ appraisal of

emotional support from their caregiver on Manne’s

positive support scale, where higher scores indicate

greater support (score range is 5–20), the respective

means were 17.4 (SD52.4) for patients who chose

LTMV and 17.9 (SD52.6) for those who did

not [t5.63, df548, p5.53). Similarly, no difference

was found between patients’ ratings of ‘‘negative

support’’ (means were, respectively, 14.9 [SD55.7]

vs. 13.1 [5.1], t5.9, 49 df, p5.38).

Financial situation. Thirty-nine percent of patients

who later chose LTMV, had household annual

incomes over $100,000, compared to 12% of those

who declined tracheostomy. The proportion with

private health insurance was similar (83% and 79%),

but the type of plan and extent of coverage may have

varied. About three-quarters of each group had at

least some help from paid home attendants.

Religious beliefs. Included in the visual analog scale

ratings were two queries about religious faith:

patients were asked, ‘‘how important is religion in

your daily life?’’ and ‘‘how comforting are spiritual

beliefs?’’. On a scale from 1 (not at all) to 10

(extremely), the means for the two study groups did

not differ significantly (Table II). On the Holland

Inventory of Religious Beliefs, mean scores (42.0

and 42.6 for the potential LTMV vs. other patients)

did not differ. Religious affiliation, shown in Table I,

also failed to differentiate the groups. Overall, there

was no evidence that religious or spiritual faith

contributed to the decision about LTMV.

Psychiatric status and future orientation. The mean

score on the seven-item Beck Depression Inventory

for future LTMV patients was in the ‘not depressed’

range while the mean for patients who later died

was in the ‘probable depression’ range (means of 1.7

[SD52.0] vs. 4.4 [SD53.9] respectively, t52.1, 53

df, p5.04) (Table III). On the PHQ, none of the

future LTMV patients had a probable diagnosis of

major or minor depressive disorder, compared to

27% (n515) of those who refused LTMV. How-

ever, 29% (n54) of future LTMV patients were

given antidepressants specifically for depression,

compared to 14% (n58) of the others.

Among the 60 patients who completed the Beck

Hopelessness Scale (a measure of future orientation

and outlook), future LTMV patients had signifi-

cantly lower scores, signifying more optimism about

the future (Table III). Patients who chose LTMV

were more likely to report faith in the future and

positive expectations for times to come. Results for

the visual analog rating of optimism confirmed this

difference: future LTMV users had a mean score of

7.2 vs. 5.3 (pv.05). On the measure of attitudes

toward hastened death, the difference between

groups was substantial (means of 6.2 [4.5] vs. 2.5

[1.4], 33 df, p5.001); future LTMV patients

reported less suffering and less interest in hastened

death. Mild emotional lability was reported for 24

patients (44%) who later died, and 29% (n54) who

later opted for LTMV.

Change with time. We were able to follow 11 patients

after tracheostomy and LTMV, and obtained

Table III. Depressive symptoms, hope, and optimism by choice to use long-term mechanical ventilation.

Indicators


LTMV (n558)

Patients who opted

for LTMV (n514)

Depressive symptoms

Patient Health Questionnaire, mean¡SD 7.9¡5.3 3.0¡3.0**

Probable diagnosis, major or minor depression, % 26.8 0.0

Beck Depression Inventory (BDI), mean¡SD 12.6¡7.3 7.8¡5.0

7-item BDI 4.4¡3.9 1.7¡2.0*

Hope

Beck Hopelessness Scale, total mean¡SD 7.8¡4.8 5.0¡3.2*

Hopelessness items, %

Look forward to future 59.6 84.6+Might as well give up 21.3 0.0+Have enough to time to accomplish goals 64.4 46.2

Future seems dark 46.8 23.1

Past experiences prepared me well 80.9 69.2

See only unpleasantness ahead 34.0 0.0*

Expect to be happier in future 42.2 61.5

Things just don’t work out 53.2 38.5

Have faith in future 51.1 76.9+Look forward to more good times than bad 56.5 100.0**

+pv.10, *pv.05, **pv.01.


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information from a caregiver for two others. One had

dropped out of the study and then died before the

final follow-up, and one requested removal of his

ventilator after two heart attacks within two weeks of

the tracheostomy, while he was still in the hospital;

there was no opportunity to interview him, but his

wife provided information about his last days.

Last study assessments before reaching endpoint

(tracheostomy or death). Patients who planned on

LTMV continued to show fewer depressive

symptoms on the BDI short form than those who

died without LTMV (means of 2.1 [2.2] vs. 4.6

[4.2], 63 df, p5.05). Among LTMV patients, one

had a severe bipolar disorder that predated ALS

symptom onset; the psychiatric disorder was a major

problem throughout the period of observation. Nine

LTMV patients never had a diagnosis of depression

although some were assessed as many as 18 times.

Over all pre-LTMV visits, excluding the patient

with bipolar disorder, 7% of study assessments were

characterized by symptoms consistent with minor or

major depression. In contrast, patients who refused

LTMV reported symptoms consistent with major or

minor depression in 19% of their assessments.

Visual analog scale ratings also remained consis-

tent with baseline differences. At the assessment

prior to LTMV or death, the LTMV group

continued to report greater optimism (6.3 vs. 4.8,

pv.05) and wish to live (9.4 vs. 7.3, pv.01), less

desire to hasten dying (1.4 vs. 3.6, pv.001) and less

weariness (5.5 vs. 7.4, pv.05).

Patients who later chose LTMV were more likely

to use non-invasive ventilation continuously (24 h/

day) than patients who declined (4/14 or 29% vs. 3/

57 or 5%, Fisher exact test5.022). The proportion

in each group who ever used non-invasive ventilation

was not, however, statistically significant (12 of 14 or

86% vs. 37/57 or 65%, Fisher exact test5.089).

On cognitive ratings, no patients who chose

LTMV were reported to have clinically significant

impairment. In contrast, among those who later

died, nine patients did: four were said to experience

marked slowing of mentation, seven had reduced

comprehension, and two had pronounced memory

problems, according to caregiver ratings (some had

more than one problem).

Outlook before and after tracheostomy. We also

examined patient reports at the visits just before

and just after tracheostomy for the LTMV group.

After tracheostomy, no patients retained speech.

Assessments took place a median of 71 days before

initiating LTMV and 63 days after. Ten patients

were available for these analyses. Patient reports pre-

and post-LTMV were for the most part stable. The

wish to live remained high before (9.1) and after

the procedure (9.4) on the 10-point VAS scale.

Optimism declined non-significantly from 6.3 to 5.9

and weariness increased non-significantly (5.6 to

6.1), but the picture overall was of stability across

the transition to LTMV.

Longer term follow-up. The final assessments in July

2005 for the 13 patients who were seen at least

once after tracheostomy or whose caregivers gave

information in a telephone interview took place, on

average, 25 months after tracheostomy (range53–

55). At final visit or follow-up, two lived in a nursing

home, three lived alone (with 24-h nurses), and

seven lived with spouse or parent. The thirteenth

patient never left institutional care after his

tracheostomy, heart attacks, and extubation. Out-

of-pocket costs ranged from none (Medicaid

coverage for nursing home patient) to $4,000/week

(for a patient supported through a network of

friends). Several patients had time-limited insur-

ance with no sources for subsequent funding; others

worried about depleting family savings. All VAS

queries showed a modest trend increase in distress

(pain, anger, depression) and trend to diminution of

positive states (e.g. optimism, will to live) compared

to appraisals immediately after tracheostomy (data

not shown), although only two patients expressed

interest in hastening death. Most continued to derive

at least some satisfaction (mean of 5.2, median and

mode of 5 on the 10-point VAS scale) and pleasure

(mean of 5.5, median and mode of 6 on the 10-point

VAS scale) from their daily lives.

We asked the interviewer, who saw patients at

their final visit and made the follow-up calls to

caregivers, for her own subjective appraisal of their

overall life satisfaction and quality, based on all

available information. She rated five as predomi-

nantly pessimistic and unhappy, five as having some

sources of satisfaction but also distress or weariness,

and the remaining three as still satisfied with life and

satisfied with their circumstances. In all 13 subjects,

the disease had progressed to severe disability. At

this time, three communicated by computer, seven

had minimal communication capacity (e.g. could

move eyes or toes to signify letters on an alphabet

board, or to signal yes or no), and two were

locked-in and subsequently died, according to final

caregiver reports (accident with breathing tube;

unspecified medical complications). One patient

could not communicate meaningfully because of

presumed cognitive impairment even though he

retained lateral eye movements that seemed to be

random. He was the only patient whose caregiver

reported a condition consistent with dementia. For

the other patients, no caregivers reported clinically

significant impairment of any kind.

After tracheostomy, patients were asked, ‘‘Are

there circumstances that would make life intolerable,

that would make you think it would be better to die

rather than continue living?’’ At their final assess-

ment, 50% said ‘‘no’’, and the other half gave


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specific answers, such as: ‘‘going to a nursing

home’’, ‘‘worse than current situation’’, ‘‘locked in,

dementia’’, ‘‘death of my wife [caregiver]’’.

Perceived caregiver burden. At study baseline, and

again at the last assessment before the study

endpoint of tracheostomy or death, those who

chose LTMV and those who died without LTMV

did not differ in their estimates of caregiver burden

on the VAS (see Tables II and IV). At the final

assessment in longer term follow-up, LTMV

patients reported a modestly lower estimate than

on either prior occasion (baseline: 7.4; pre-

tracheostomy56.2; final assessment56.1 on the

10-point VAS scale). The mean score for patients

who lived with a caregiver was 7.2, unchanged from

study entry.

Caregiver experience. At study entry, a trend

suggested that caregivers of future LTMV patients

were somewhat more distressed and burdened by

caregiving compared to those whose patients died

without LTMV although satisfaction with care-

giving was similar (Table V). At last assessment

before tracheostomy or death, caregivers of the

tracheostomy group reported both more caregiver

satisfaction and greater burden (these measures are

uncorrelated). At the final assessment, up to 55

months after baseline, only six of the 13 caregivers

still available for interview lived with the patient;

three patients were in nursing homes, three lived

alone with 24-h nurses, and one died in hospital

six weeks after intubation. All but one of the

‘residential’ caregiver spouses reported substantial

distress, despite having full-time aides. Their con-

cerns ranged from regret at loss of companionship

to ‘burnout’ reported by one spouse who was

considering taking a trip to Antarctica. Neverthe-

less, while reporting serious burden, most continued

to report satisfaction with the care-giving role.

Discussion

This study was unusual because it was prospective

and also because interviewers and patients continued

to participate despite massive communication chal-

lenges. However, the sample was small and findings

Table IV. Mood and mental health: last visit before tracheostomy or death.

Last assessment before event


LTMV (n558)

Patients who opted for

LTMV (n514)

Time from last interview to event (median days) 32 70.5

Depressive symptoms

Patient Health Questionnaire, mean¡SD 6.5¡5.1 2.0¡1.2+Clinical Impression, major-minor depression, % 24.5 16.6

Beck Depression Inventory-7 item, mean¡SD 4.6¡4.2 2.1¡2.2*

Visual Analog Scales, mean¡SD

Energy 4.4¡2.6 5.7¡1.8

Suffering 5.0¡2.8 4.8¡2.5

Control over ALS management 3.1¡2.9 3.7¡2.7

Anger 3.4¡2.8 3.7¡1.9

Perceived burden of care 6.9¡3.0 6.2¡3.0

Optimism 4.8¡3.1 6.3¡2.2*

Interest in hastening death 3.6¡3.3 1.4¡1.3***

Weariness from ALS 7.4¡2.8 5.5¡2.6*

Wish to live 7.3¡3.4 9.4¡1.6**

Comfort in religion 5.9¡4.0 5.5¡3.6

Comfort in spirituality 6.3¡4.0 5.7¡3.7

+pv.10, *pv.05, **pv.01, ***pv.001.

Table V. Caregiver experience.

Non-LTMV LTMV

Baseline, n566–69

Zarit caregiver satisfaction 14.0¡4.2 14.4¡5.1

Zarit caregiver burden 8.5¡4.4 11.4¡6.3

Anger, VAS 4.2¡3.0 4.2¡3.8

Burden of care, VAS 4.9¡3.0 5.7¡3.5

Beck Depression Inventory 9.6¡6.4 14.3¡13.3+

Last assessment prior to event, n545–59

Zarit caregiver satisfaction 13.4¡4.5 17.5¡4.1*

Zarit caregiver burden 9.2¡4.6 11.1¡7.3

Anger, VAS 3.5¡2.6 4.5¡3.4

Burden of care, VAS 4.3¡3.0 7.2¡3.1*

Beck Depression Inventory 8.4¡6.4 10.7¡5.2

First post-LTMV assessment (n58–11)

Zarit caregiver satisfaction 14.3¡6.1

Zarit caregiver burden 7.9¡3.5

Anger, VAS 4.2¡2.9

Burden of care, VAS 5.5¡3.3

Beck Depression Inventory 9.9¡6.1

Final post-LTMV Assessment (n57–11)

Zarit caregiver satisfaction 14.0¡4.8

Zarit caregiver burden 7.6¡4.3

Anger, VAS 2.7¡2.4

Burden of care, VAS 4.8¡3.2

Beck Depression Inventory 7.8¡10.2

+pv.10, *pv.05.


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must therefore be regarded as tentative rather than

established. Nevertheless, we documented differ-

ences between patients who eventually had tracheos-

tomies and those who refused, as well as patterns of

response over time for the LTMV patients.

Baseline opinions about LTMV persisted over

follow-up, with no change in attitudes about extend-

ing survival. This contrasts with Chochinov’s (28)

finding that the will to live was highly unstable

among 168 terminally ill cancer patients in palliative

care.

At baseline and throughout, eventual LTMV

patients differed substantially from those who

declined LTMV in appraising their current health

status, optimism level, and future expectations.

Many LTMV patients believed in a foreseeable cure

for ALS and hence the value of living with

ventilatory support to allow them to benefit from

future treatment. One patient went to Lourdes in a

wheelchair seeking a miracle cure. All patients had

been informed about the disease and its inevitable

progression, but those who eventually chose LTMV

continued to ‘‘look forward to the future with hope

and enthusiasm’’.

We were unable to evaluate patients using stan-

dard neuropsychological tests because communica-

tion was so severely restricted. However, ratings of

global cognitive status, comprehension and memory

by caregivers at study entry reflected at least mild

cognitive impairment in nearly half the patients who

declined tracheostomy, including 15% (n58) with

moderate to severe problems. Among future LTMV

patients, only one (14%) was reported to have a

cognitive problem and it was mild. Equivalent

ratings were made at the visit before death or

tracheostomy, when 51% of patients who died were

reported to have some cognitive problems, including

16% that were described as moderate or severe.

At final follow-up after tracheostomy, a mean of

33 months later, only approximate ratings of

cognitive status were feasible given absent speech

and mostly absent hand movement. Only one

patient was described as severely impaired.

LTMV patients also differed from patients who

later died in terms of depression. At study entry,

none had a diagnosis of minor or major depressive

disorder compared to 27% of those who died

without LTMV. However, more future LTMV

patients were currently being treated with antide-

pressants for depression, suggesting that effective

treatment for depression may lead some patients to

consider LTMV.

The overlap between cognitive impairment and

depression, both of which include problems with

concentration and mental acuity, may complicate

the interpretation of causality of cognitive pro-

blems. When we examined the overlap in our data,

we found that 27% of those with any cognitive pro-

blems had a current diagnosis of major or minor

depression, but among those with moderate or

severe cognitive problems, only one patient was

currently depressed. It thus seems that some mild

cognitive problems may be secondary to depression

or antidepressant medication, but more severe

problems that are likely to be clinically important

cannot be attributed to depression.

Factors that might be expected to influence

choice, such as religious affiliation, support derived

from spiritual or religious beliefs, living situations

(home vs. institution), or degree of disability, did not

differentiate those who later chose LTMV from

those who refused. However, financial status did

differ. Although we did not systematically document

all expenses, for patients living at home after

tracheostomy some caregivers reported total costs

exceeding $200,000. For example, one patient

who lived alone had two registered nurses 24 h/day

who were each paid $250/day, not covered by

insurance.

Those caregivers who live with the LTMV patient

bear tremendous responsibility not only for the

patient’s well-being but their survival, in terms of

managing the tracheostomy, suctioning the airway or

supervising aides who do so, assuring adequacy of

equipment and back-up in case of power failure, and

working out the financial arrangements to cover the

often great unreimbursed costs of LTMV care (29).

Caregivers largely or completely relinquish any but

essential outside activities and personal lives. They

report considerable distress. Most patients are aware

of this burden, which ameliorates some, but not all,

caregiver burden.

Life satisfaction and contentment, among those

who did choose LTMV and were assessed on sub-

sequent occasions, were not related to communication

level or living situation. Of the four patients rated by

the interviewer as most satisfied with their current

lives, two could communicate ‘‘yes’’ or ‘‘no’’ only by

moving their eyes or a single toe. One lived alone after

being widowed following tracheostomy, one lived with

parents, and two lived in nursing homes.

In this study, the rate of LTMV was 19%,

substantially more than other American reports

and quite possibly an artifact of our late-stage

sample; all subjects had a presumed life expectancy

of less than six months at baseline. Other reports of

tracheostomy rates include patients at all stages of

illness. Alternatively, our clinic may have unusually

high rates, or perhaps all rates in the United States

have risen in the past few years. In future studies,

prospective assessment of physician attitudes and

communications would be useful to correlate with

eventual patient choices.

Several limitations warrant consideration. Our

sample was almost entirely drawn from a single

large urban ALS center with generous staffing and

extensive services. Our sample was largely white

(ALS rates for African Americans are consistently


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lower) (30) and more affluent than the general

public. However, it is the difference between LTMV

and non-LTMV patients that is important. We were

not able to devise a method for assessing the

neurocognitive status of LTMV patients who were

living beyond their expected life span; only major

impairment could be detected, as it was for one

patient. Lack of statistical power may have pre-

cluded identification of other significant predictors

of choice of LTMV. Although we assured both

patients and caregivers of confidentiality, and asked

neutrally phrased questions, we cannot rule out

a positive reporting bias due to social desirability

or cognitive dissonance on the part of caregivers

(e.g. conflicting emotions regarding caregiving).

Nevertheless, despite these limitations, we could

identify predictors of LTMV choice. In terms of

course over nearly three years after LTMV, we

observed a modest decline in overall life satisfaction

together with a continued desire to live for these

severely limited patients whose communication

capacity was generally minimal, and whose illness

continued to progress.

Acknowledgement

This study was supported in part by the National

Institute of Mental Health R 01 MH62200 (SM

Albert). We wish to thank study participants for

sharing their beliefs and insights.

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predictors and course of elective long‐term mechanical ventilation: a prospective study of als...

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