Prevalence of depressive disorders and change over time in late-stage ALS.

OBJECTIVE: To determine the prevalence of depressive disorders and symptoms in patients with late-stage ALS, to identify possible risk and protective factors associated with depression, and to determine whether depression increases as death approaches. METHODS: Semistructured interviews were conducted monthly with hospice-eligible patients with ALS and caregivers until the study endpoints of death or tracheostomy. Standardized measures were administered to assess depressive disorders and symptoms, hopelessness, spiritual beliefs, attitudes toward hastened death, quality of life, and related constructs. RESULTS: Sixty-three percent of eligible patients were enrolled. Of the 80 participants, 17 were seen only once; the number of monthly assessments for the others ranged from 2 to 18. For the 53 patients who died, median interval between last assessment and death was 30 days. At study baseline, 81% had no depressive disorder, 10% had minor depression, and 9% had symptoms consistent with major depression. Diagnoses of depression were made on 16% of 369 monthly assessments. Fifty-seven percent of patients never had a depression diagnosis at any visit, and 8% were depressed at all visits. There was no trend toward increasing depression as death approached. Presumed protective factors including spiritual beliefs, spouse as care partner, financial situation, depression in caregiver, and hospice participation did not distinguish between those who were depressed and those who were not. CONCLUSIONS: Results of multiple measures of depression and distress converged to indicate that major depression in people with late-stage ALS is rare, although transient depressive symptoms may occur, and depression does not generally increase as death approaches.

Wish to die in end-stage ALS.

BACKGROUND: In retrospective studies, estimates of hastened dying among seriously ill patients range from <2% in one national survey to as much as 20% in end-stage disease cohorts. OBJECTIVE: To examine, in prospective studies, dying patients in the months before death, in order to understand the wish to die. METHODS: Patients with advanced ALS with a high likelihood of death or need for tracheostomy within 6 months were identified. Patients were assessed monthly with an extensive psychosocial interview, including a diagnostic interview for depression. Family caregivers were interviewed on the same schedule and also after patient deaths. RESULTS: Eighty patients with ALS were enrolled, 63% of eligible patients; 53 died over follow-up. Ten (18.9%) of the 53 expressed the wish to die, and 3 (5.7%) hastened dying. Patients expressing the wish to die did not differ in sociodemographic features, ALS severity, or perceived burden of family caregivers. They were more likely to meet criteria for depression, but differences were smaller when suicidality was excluded from the depression interview. Patients who expressed the wish to die reported less optimism, less comfort in religion, and greater hopelessness. Compared with patients unable to act on the wish to die, patients who hastened dying reported reduction in suffering and increased perception of control over the disease in the final weeks of life. CONCLUSION: These findings suggest caution in concluding that the desire to hasten dying in end-stage disease is simply a feature of depression.

Objective tests for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS).

OBJECTIVE: To develop objective markers for upper motor neuron (UMN) involvement in ALS, the value of single-voxel MR spectroscopy (MRS) and transcranial magnetic stimulation (TMS) was studied. METHODS: Test results of 164 ALS patients who had MRS only (n = 91), TMS only (n = 13), or both (n = 60) were analyzed; also, 11 autopsy examinations were evaluated. RESULTS: Abnormal test results consistent with UMN involvement were found in 134 patients with clinical UMN signs: 86% on MRS, 77% on TMS, and 70% on MRS and TMS together. Among 30 patients with solely LMN signs (progressive muscular atrophy), UMN results were found in 63% on MRS, 63% on TMS, and 46% on both tests together. There was a significant association of the degree of abnormal N-acetyl aspartate/creatine ratios with UMN signs (p = 0.01). The sensitivity to detect UMN involvement was 0.86 for MRS (specificity 0.37) and 0.77 for TMS (specificity 0.38). At autopsy, all 11 patients had pathologic UMN abnormalities, including 4 with normal MRS and 1 with normal TMS in life. CONCLUSIONS: MRS is highly sensitive, somewhat more than TMS, and shows good correlation with clinical UMN signs. Combining MRS and TMS results in the same patient with further refinement may help in the early diagnosis of ALS.

Disparities in perceptions of distress and burden in ALS patients and family caregivers.

OBJECTIVES: To examine agreement between end-stage ALS patients and their family caregivers on indicators of physical and psychological status at the end of life. METHODS: Patient-caregiver pairs completed monthly interviews in patient homes. Patients were asked to rate their current pain, energy, suffering, depression, control over ALS, optimism, interest in hastened death, weariness from ALS, will to live, and how burdened they thought caregivers were on Visual Analogue Scales. Caregivers completed identical ratings of patients as well as a measure of their own burden. Both independently completed the ALS Functional Rating Scale-Rev. (ALSFRS-R), a measure of patient disability and physical function. RESULTS: A total of 69 patient-caregiver pairs participated. For measures of physical function, kappa ranged from 0.49 to 0.83, indicating moderate to excellent agreement. Patient and caregiver composite ALSFRS-R scores were highly correlated (r = 0.92, p < 0.001). Agreement between patients and caregivers was high for ratings of patient pain, control over ALS, optimism, and will to live, and this level of agreement remained high over multiple assessments. In pairwise analyses, caregivers rated patients as having less energy, greater suffering, and greater weariness than patients indicated for themselves, whereas patients rated caregivers as more burdened than caregivers reported for themselves. CONCLUSIONS: Caregivers can accurately report information about a patient's physical function at the end of life. However, patients and caregivers each overestimated the psychosocial impact of the disease on the other.

Impact of spirituality and religiousness on outcomes in patients with ALS.

The Project of Death in America Study at Columbia Presbyterian Medical Center enrolled 121 patients with ALS from 1996 through 1997, 46 of whom participated in a study assessing the effects of religiousness and spirituality (attachment to life, mental health, support group, health care proxy, and attitudes toward death) on outcomes (technology and death). Spirituality or religion influenced use of percutaneous endoscopic gastrostomy, noninvasive assisted ventilation, tracheotomy, and attitudes toward the dying process.

Prospective study of palliative care in ALS: choice, timing, outcomes.

To understand palliative care choices among people with ALS, it is important to follow a group of recently diagnosed patients and record when patients reach well-defined palliative care milestones. We began following such a cohort prospectively in 1996, when 121 ALS patients were enrolled from a tertiary care clinic. These patients are assessed every 4 months to determine their experience with ameliorative and palliative care. Domains include adjuvant therapies (e.g. speech therapy), adaptive aids (e.g. wheelchair use, augmentative communication), home health care, PEG placement, pulmonary support, health care directives, psychosocial care (e.g. participation in support group, pastoral counseling), and hospitalization. The median follow-up time for the cohort, to date, was 12 months. In the group that entered the cohort within 1 year of their diagnosis (n=93), 53.8% have died, 22.6% have had PEG, 19.4% have used Bi-PaP, and 4.3% have had a tracheostomy. Many patients did not take advantage of palliative care options before death; for example, 36.6% used hospice, 48% had signed a power of attorney form, and 18% had 'do not resuscitate' orders in their medical charts. Examining time to such endpoints captures important features of patient and family experience with the disease.

A prospective study of preferences and actual treatment choices in ALS.

OBJECTIVE: To determine whether ALS patients' preferences for ameliorative or life-extending technologies elicited early in the disease were related to later treatment choices. METHODS: In this prospective cohort study, 121 patients were seen at a tertiary ALS care center and followed for a median of 12 months. At baseline, patient preferences for use of tracheostomy and percutaneous endoscopic gastrostomy (PEG) placement were elicited. All patients received the same educational information before being interviewed about treatment preferences. Patients were then followed to determine if patients who viewed the interventions favorably at baseline were significantly more likely to use the interventions over follow-up. RESULTS: Six to twelve percent of patients were certain they wanted tracheostomy and 28.2% wanted PEG. Preferences were related to later treatment choices: 20% of patients who found tracheostomy acceptable had one in the follow-up period, compared with 3.4% of those not in favor (p < 0.001). For PEG, similar findings were obtained: 48.5% who initially found it acceptable had PEG, versus 8.1% of those not in favor of this treatment (p < 0.001). Patients who found the interventions acceptable were more likely to be recently diagnosed, expressed greater attachment to life, and showed greater declines in pulmonary function over follow-up. CONCLUSIONS: Patients with ALS were able to express their preferences for life-extending or ameliorative technologies and made choices consistent with these preferences. However, patient preferences may change over time, and clinical education efforts are required throughout the course of disease.