Concordance between site of onset and limb dominance in amyotrophic lateral sclerosis.

BACKGROUND: Focality of onset of amyotrophic lateral sclerosis (ALS) is not understood. Attempts to implicate physical exercise in the aetiology of ALS have provided inconsistent results. If physical use of a limb were important in defining the site of onset, then handedness might be expected to influence the side of upper limb-onset disease and footedness likewise in lower limb-onset ALS. METHODS: ALS patients registered with an internet-based support site were invited to complete an online questionnaire concerning site of onset of symptoms and their dominant hand and foot. A binomial test of proportions was used to investigate the null hypothesis that handedness and footedness do not influence side of onset in upper and lower limb-onset ALS, respectively. RESULTS: 343 ALS patients with limb-onset disease were studied. For upper limb-onset patients, there was concordance for side of onset and handedness (64%; p<0.0006). For lower limb-onset patients, concordance for side of onset and footedness was absent. The frequency of left handedness was commensurate with that found in the general population. INTERPRETATION: These results are potentially consistent with the hypothesis that exercise influences pathogenesis in ALS since routine physical demands on the upper limb are heavily influenced by limb dominance, whereas in the lower limbs the commonest function is standing or locomotion, which uses both legs equally. However, there may also be an inherent cortical vulnerability underlying upper limb-onset laterality, possibly influenced by changes in neuronal connectivity and cortical excitability in relation to handedness and reflected by the "split hand" phenomenon consistently observed in ALS.

Measuring function in advanced ALS: validation of ALSFRS-EX extension items.

BACKGROUND: With the aid of assistive technology, some patients with amyotrophic lateral sclerosis (ALS) are able to live for several years past the lowest measurable level of function on the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS-R), a widely used end-point in ALS assessment. There is a research need to monitor patient function at the end of life, particularly in the face of severe impairment or 'locked in syndrome'. METHODS: We used an online community for people with ALS (PALS) (PatientsLikeMe) to construct and pilot a number of new items to add to the ALSFRS-R scale to improve its sensitivity at lower levels of physical function in patients with advanced ALS. RESULTS: Ten new scale items were generated by a survey of PALS with advanced disease. These were added to the existing ALSFRS-R and data were received from 326 PALS at baseline with 169 PALS (52%) completing a 1-week test-retest and 218 PALS (67%) completing a 3-month retest [corrected]. CONCLUSIONS: Three new items were selected which conformed to the existing factor structure of the ALFRS-R. These relate to the ability to use fingers to manipulate devices, ability to show emotional expression in the face, and ability to get around inside the home. Real-world validation is the next step to assess the utility of the ALFRS-EX.

The forgotten component of the quality triad: can we still learn something from "structure"?

BACKGROUND: Quality assessment was founded on structural measures, such as accreditation status of facilities, credentialing of providers, and type of provider. Recent efforts in measures development have focused on processes and outcomes because research has suggested that structural measures are not strong markers of the quality of care at the health plan or provider levels. Nevertheless, the literature on the quality of health care contains a number of examples illustrating the potential application of structural measures to the assessment of quality. The continued development of measures of structure-which would at least measure aspects of the physical environment, working conditions, organizational culture, and provider satisfaction--may be helpful because generalizing from studies of process and outcome requires specification of the conditions under which these linkages are found. A ROAD MAP FOR MEASURES DEVELOPMENT: The Leapfrog Group of large purchasers has promoted the application of three patient safety "leaps" that are, in essence, structural measures: the use of computerized physician order entry, the selective referral of patients to high-volume providers for certain procedures, and the availability of board-certified critical care specialists in intensive care units. Structural measures, like process and outcomes measures, face the same challenges of standardization, reliability, validity, and portability. Field testing of potential measures will be required to examine the feasibility and added value of these measures in real-world settings. CONCLUSION: Research to date suggests that a new cadre of structural measures of health care quality, which have largely been overlooked in the recent measures development boom, have the potential to fill in important gaps in our ability to assess quality.

Longitudinal study of psychiatric symptoms, disability, mortality, and emigration among Bosnian refugees.

CONTEXT: Evidence is emerging that psychiatric disorders are common in populations affected by mass violence. Previously, we found associations among depression, posttraumatic stress disorder (PTSD), and disability in a Bosnian refugee cohort. OBJECTIVE: To investigate whether previously observed associations continue over time and are associated with mortality emigration to another region. DESIGN, SETTING, AND PARTICIPANTS: Three-year follow-up study conducted in 1999 among 534 adult Bosnian refugees originally living in a refugee camp in Croatia. At follow-up, 376 (70.4%) remained living in the region, 39 (7.3%) were deceased, 114 (21.3%) had emigrated, and 5 (1%) were lost to follow-up. Those still living in the region and the families of the deceased were reinterviewed (77.7% of the original participants). MAIN OUTCOME MEASURES: Depression and PTSD diagnoses, based on Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) criteria and measured by the Hopkins Symptom Checklist-25 and the Harvard Trauma Questionnaire, respectively; disability, measured by the Medical Outcomes Study Short-Form 20; and cause of death, determined by family interviews with review of death certificates, if available. RESULTS: In 1999, 45% of the original respondents who met the DSM-IV criteria for depression, PTSD, or both continued to have these disorders and 16% of respondents who were asymptomatic in 1996 developed 1 or both disorders. Forty-six percent of those who initially met disability criteria remained disabled. Log-linear analysis revealed that disability and psychiatric disorder were related at both times. Male sex, isolation from family, and older age were associated with increased mortality after adjusting for demographic characteristics, trauma history, and health status (for male sex, adjusted odds ratio [OR], 2.63; 95% confidence interval [CI], 1.17-5.92; living alone, OR, 2.40; 95% CI, 1.07-5.38; and each 10-year increase in age, OR, 1.91; 95% CI, 1.34-2.71). Depression was associated with higher mortality in unadjusted analysis but was not after statistical adjustment (unadjusted OR, 3.12; 95% CI, 1.55-6.26; adjusted OR, 1.85; 95% CI, 0.82-4.16). Posttraumatic stress disorder was not associated with mortality or emigration. Spending less than 12 months in the refugee camp (OR, 11.30; 95% CI, 6.55-19.50), experiencing 6 or more trauma events (OR, 3.34; 95% CI, 1.89-5.91), having higher education (OR, 1.90; 95% CI, 1.10-3.29), and not having an observed handicap (OR, 0.11; 95% CI, 0.02-0.52) were associated with higher likelihood of emigration. Depression was not associated with emigration status. CONCLUSIONS: Former Bosnian refugees who remained living in the region continued to exhibit psychiatric disorder and disability 3 years after initial assessment. Social isolation, male sex, and older age were associated with mortality. Healthier, better educated refugees were more likely to emigrate. Further research is necessary to understand the associations among depression, emigration status, and mortality over time.

Patient-centered quality measures for Asian Americans: research in progress.

We aim to develop and validate a questionnaire that examines quality of care from the patient's perspective for limited-English-proficient Asian Americans (AA) of Chinese and Vietnamese descent. We will conduct focus groups of patients to identify issues important to them, with an emphasis on communication and access to care. We will then draft a questionnaire and test its validity using standard survey research methods and direct observation of patient-provider encounters. Subsequent field testing will involve face-to-face patient interviews 1 month after an outpatient visit. We will evaluate alternate modes of administration to test feasibility and to maximize response. The result of our study will be a validated, culturally sensitive, patient-centered instrument that measures health care quality for limited-English-proficient AA patients. Our research will provide a template for developing future quality measures for other vulnerable populations.

Work transitions and health in later life.

OBJECTIVES: The goal of the analysis was to examine the association between health status and work behavior among men aged 55-69. We specifically examined the conditions under which health is most strongly associated with labor force exit and reentry. METHODS: The association between health and labor force transitions was examined using logistic regression analyses, based on data from the 1984 and 1985 panels of the Survey of Income and Program Participation. RESULTS: We found that for men aged 55-69 in the mid-1980s, poor health was positively associated with labor force exit, and negatively associated with returns to work. Although these main effects are very strong, we found that health was particularly important among individuals for whom retirement was least attractive. Health had its most substantial association with work transitions among men with working wives, as well as among men who were younger, or who had limited nonwork financial resources. Health also had a particularly strong association with work transitions among Black men, but only with reference to reentry decisions. DISCUSSION: Our results suggest that continued work may have limited appeal for men who are prepared for retirement, even when they are in excellent health.

Disability associated with psychiatric comorbidity and health status in Bosnian refugees living in Croatia.

CONTEXT: The relationship between psychiatric symptoms and disability in refugee survivors of mass violence is not known. OBJECTIVE: To determine if risk factors, such as demographics, trauma, health status, and psychiatric illness, are associated with disability in Bosnian refugees. DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional survey conducted in 1996 of Bosnian refugee adults living in a camp established by the Croatian government near the city of Varazdin. One adult aged 18 years or older was randomly selected from each of 573 camp families; 534 (93%) agreed to participate (mean age, 50 years; 41% male). MAIN OUTCOME MEASURES: Culturally validated measures for depression and posttraumatic stress disorder (PTSD) included the Hopkins Symptom Checklist 25 and the Harvard Trauma Questionnaire, respectively. Disability measures included the Medical Outcomes Study Short-Form 20, a physical functioning scale based on World Health Organization criteria, and self-reports of socioeconomic activity, levels of physical energy, and perceived health status. RESULTS: Respondents reported a mean (SD) of 6.5 (4.7) unduplicated trauma events; 18% (n=95) had experienced 1 or more torture events. While 55.2% reported no psychiatric symptoms, 39.2% and 26.3% reported symptoms that meet DSM-IV criteria for depression and PTSD, respectively; 20.6% reported symptoms comorbid for both disorders. A total of 25.5% reported having a disability. Refugees who reported symptoms comorbid for both depression and PTSD were associated with an increased risk for disability compared with asymptomatic refugees (unadjusted odds ratio [OR], 5.02; 95% confidence interval [CI], 3.05-8.26; adjusted OR, 2.06; 95% CI, 1.10-3.86). Older age, cumulative trauma, and chronic medical illness were also associated with disability. CONCLUSIONS: In a population of Bosnian refugees who had recently fled from the war in Bosnia and Herzegovina, psychiatric comorbidity was associated with disability independent of the effects of age, trauma, and health status.

The stability of preferences for life-sustaining care among persons with AIDS in the Boston Health Study.

BACKGROUND: Clinicians recognize the importance of eliciting patient preferences for life-sustaining care, yet little is known about the stability of those preferences for patients with serious disease. OBJECTIVES: To examine the stability of preferences for life-sustaining care among persons with AIDS and to assess factors associated with changes in preferences. DESIGN: Two patient surveys and medical record reviews, administered four months apart in 1990-1991. SETTING: Three health care settings in Boston. PATIENTS: 252 of 505 eligible persons with AIDS who participated in both baseline and follow-up surveys. MAIN OUTCOME MEASURES: A single question assessing desire for cardiac resuscitation and a scale of preferences for life-extending treatment conditional on hypothetical health states. RESULTS: Approximately one-fourth of the respondents changed their minds about life-sustaining care during a four-month period. Of patients who initially desired cardiac resuscitation, 23% decided to forego it four months later, and of those who initially said they would decline care, 34% later said they would accept it. Of those who initially desired any of the life-extending treatments, 25% decided to forego them four months later, and of those who initially said they would decline life-extending care, 24% later said they would accept some treatment. Patients reporting changes in physical function, pain, or suicide ideation were more likely to modify their desires to be resuscitated (all p< or =0.05). Patients lacking an advance directive, not completing high school, or becoming more severely ill were more likely to change their preferences on the Life Extension scale (p< or =0.05). Patients who discussed their preferences with at least one physician were just as likely as others to change desires for cardiac resuscitation. Age, gender, race, emotional health, clinical severity, social support, and site of care were not significant correlates of change for either measure. CONCLUSIONS: Health care providers should periodically reassess preferences for life-sustaining care, particularly for patients with progressive disease, given the instability in patient preferences. However, predictors of instability may vary with how preferences are measured. In particular, changes in health status may be related to instability of preferences for certain types of treatments.

The Boston AIDS Survival Score (BASS): a multidimensional AIDS severity instrument.

OBJECTIVES: This study developed a new acquired immunodeficiency syndrome (AIDS) severity system by including diagnostic, physiological, functional, and sociodemographic factors predictive of survival. METHODS: Three-hundred five persons with AIDS in Boston were interviewed; their medical records were reviewed and vital status ascertained. RESULTS: Overall median (+/- SD) survival for the cohort from the first interview until death was 560 +/- 14.4 days. The best model for predicting survival, the Boston AIDS Survival Score, included the Justice score (stage 2 relative hazard [RH] = 1.25, 95% confidence interval [CI] = 0.80, 1.96; stage 3 RH = 1.76, 95% CI = 1.15, 2.70), a newly developed opportunistic disease score (Boston Opportunistic Disease Survival Score; stage 2 RH = 1.35, 95% CI = 0.90, 2.02; stage 3 RH = 2.10, 95% CI = 1.38, 3.18), and measures of activities of daily living (any intermediate limitations, RH = 1.84, 95% CI = 1.05, 3.21; any basic limitations, RH = 2.60, 95% CI = 1.44, 4.69). This model had substantially greater predictive power (R2 = .17, C statistic = .68) than the Justice score alone (R2 = .09, C statistic = .61). CONCLUSIONS: Incorporating data on clinically important events and functional status into a physiologically based system can improve the prediction of survival with AIDS.

Pathways to labor force exit: work transitions and work instability.

The purpose of this study is to examine alternative pathways to labor force exit among older men. Based on the life course perspective, we distinguish between crisp exits from the labor force, which are characterized as being unidirectional, and blurred transition patterns, which include repeated exists, entrances, and unemployment spells. Using longitudinal data from the 1984 Survey of Income and Program Participation, we find that one-quarter of the sample of men aged 55 to 74 at first interview experienced at least one transition in labor force status over a 28-month observation period. Fewer than half of these can be characterized as crisp exists from the labor force. Our multivariate analysis suggests that blurred transition patterns are likely part of an effort to maintain economic status in later life.

The validity of self-reported health-care utilization by AIDS patients.

OBJECTIVES: To examine the validity of self-reported health-care utilization among persons with AIDS. DESIGN: A comparison of survey data with information collected from medical and financial records. METHODS: Personal interviews provided information on utilization within a 4-month period for inpatient admissions (n = 296), ambulatory visits (n = 284), and hours of homecare (n = 106). Risk group, socioeconomic characteristics, disease stage, functional status, memory, and respondent's recall ability were also measured. Reporting error was defined as the difference between self reports and medical/financial records. Variations among subgroups of patients were examined using t tests and multiple regression. To determine whether reporting errors affected analysis of utilization data, we compared coefficients from parallel utilization models using each data source to predict use/non-use and total utilization. RESULTS: Mean overall reporting errors were small and not significantly different from zero. Reporting errors were lowest for hospital admissions and highest for homecare. High utilizers underreported all types of services. The interviewer evaluation of recall was an independent and significant predictor of reporting errors for admissions and ambulatory visits. Reporting errors varied by selected subgroup characteristics, but the direction and significance of the error depended on the type of utilization measured. In the parallel utilization models, few differences appeared between models using self-reports and medical/financial records to identify correlates of use/non-use, but some differences between the models of total utilization were apparent. CONCLUSIONS: Self-reports of utilization by AIDS patients with a recall period of 4 months or less provide, on average, valid data for analytic purposes. However, caution should be applied to reports by high or low users or by respondents judged by interviewers to have major recall problems.

The role of reluctance to give up life in the measurement of the values of health states.

Questions that involve willingness to risk or give up life often are used to measure the values of health states. In the Boston Health Study, interviews with 291 patients who had AIDS included questions about health status and current desire for resuscitation, and a series of hypothetical questions about desire for life-extending efforts if the patients found themselves in undesirable states, such as being chronically nauseous or blind. An index, "reluctance to give up life" was made from five such questions. The desire for resuscitation was related to current health status, but the general reluctance to give up life was not. Desire to be resuscitated was significantly related to current health status only when "reluctance to give up life" was "low." For people reluctant to say they will give up any life at all, questions that involve risking or trading life seem likely to be poor measures of the values of health states.

Costs of medical care and out-of-pocket expenditures for persons with AIDS in the Boston Health Study.

We examined the cost of health care and out-of-pocket medical expenditures for 189 persons with AIDS at three sites in Massachusetts: an HMO; a public hospital immunodeficiency clinic; and a group practice at a major teaching hospital. The mean cost of care during a four-month reference period was $9,093 +/- $12,683 (mean +/- standard deviation) per patient. Thirty-six percent of the cost was due to hospital care and 40% was due to home health care. Mean out-of-pocket expenditures were $429 +/- $991 (mean +/- standard deviation). Studies that focus on the cost of hospital care may seriously underestimate the medical costs of AIDS.

Correlates of employment after AIDS diagnosis in the Boston Health Study.

OBJECTIVES: The purpose of this study was to determine the impact of personal and job characteristics on the time to employment loss after diagnosis of the acquired immunodeficiency syndrome (AIDS) and to examine how job loss affects patients' income. METHODS: Data were collected from 305 patients with AIDS at three sites in Boston, Mass, between February 1990 and July 1991. Life-table methods were used to estimate the number of months employed after diagnosis. A Cox proportional hazards model was used to estimate the effect of risk factors on the probability of ceasing employment in a month. RESULTS: Seventy-six percent of respondents were working at the time of diagnosis; 53% still had a job at the time of the baseline interview, which averaged 16 months later, but about one in three was on sick or disability leave. Mental and physical demands of jobs significantly influenced the likelihood of employment loss. The loss of earnings reduced monthly income by 75%. CONCLUSIONS: Job characteristics affect the likelihood of employment loss, which in turn has a deleterious effect on income. Programs supporting persons with AIDS during the transition out of work or enabling them to modify their job demands may also reduce these problems.

Changes in insurance status and access to care for persons with AIDS in the Boston Health Study.

The purpose of this study was to measure unmet needs and changes in insurance status for persons with acquired immunodeficiency syndrome (AIDS). Thirty-six percent of the study's Boston-area respondents (n = 305) had a change in insurance coverage between AIDS diagnosis and interview. Medicaid coverage increased from 14% to 41%. Pneumocystis carinii pneumonia prophylaxis was nearly universal. Only 5% did not receive zidovudine, and intravenous drug users were at higher risk. Approximately 14% to 15% of patients reported problems in obtaining medical and dental services; Blacks, homeless persons, and those who were not high school graduates were at higher risk. Use of selected treatments for which there were clear clinical guidelines was adequate, yet disadvantaged groups were more likely than other persons with AIDS to face obstacles to other services.

Measuring public priorities for insurable health care.

The goal of this research was to develop and evaluate a way to measure the value people place on various medical services in their decisions about what health insurance should cover. A vignette approach to measuring consumer values was developed. People were asked to assign priority and desire to have insurance cover 64 different services. A national probability sample of 206 adults was interviewed by telephone. Their ratings were compared with those of a sample of 47 corporate benefits officers of Fortune 500 companies. Priorities were not significantly associated with respondent characteristics. They were positively correlated with independent assessments of the seriousness of the patient's condition and the likely efficacy of the services. Priorities and desire to cover were virtually the same when respondents were asked about insurance for a low-income population as for a general population. Two-thirds of the ratings of the public were the same as those of benefit officers. The public gave higher ratings than benefits officers to long-term care and services to relieve worries, and the public gave lower ratings to the value of treatment of substance abuse and services when the patient could be viewed as at fault. This pilot test indicates this is an efficient, feasible, useful strategy for measuring the extent to which people value various medical services that could contribute to the process of making decisions about health insurance coverage.

Health-related quality of life in persons with acquired immune deficiency syndrome.

Assessing health-related quality of life in persons infected with human immunodeficiency virus (HIV) is extremely important, but most available scales are too long, contain items that are not relevant for such persons, or do not assess important signs and symptoms of HIV infection. This study presents a new set of scales for assessing the symptoms and functioning of persons infected with HIV and reports data on their reliability and validity collected in face-to-face interviews with 189 patients receiving primary care. This study also assesses the associations among systems, functional impairment, and global health assessments. The scales are easy to administer, are reliable, and serve as valid measures of quality of life. Fatigue, functional status, and average severity of all symptoms were the best predictors of overall perceived health status. Psychological well-being and perceived health status were the best predictors of overall perceived health status. Psychological well-being and perceived health status were the strongest correlates of life satisfaction. When assessing the health-related quality of life of persons infected with HIV, this study recommends utilizing a comprehensive set of measures that allows one to examine both discrete symptoms and the more diffuse impact of illness on functioning, mental health, and quality of life.

Discussion of preferences for life-sustaining care by persons with AIDS. Predictors of failure in patient-physician communication.

OBJECTIVES: To assess the determinants of communication about resuscitation between persons with acquired immunodeficiency syndrome (AIDS) and their physician. DESIGN AND SETTING: Structured patient interview at a staff-model health maintenance organization (HMO), an internal medicine group practice at a private teaching hospital, and an AIDS clinic at a public hospital. PATIENTS: 289 persons with AIDS. MAIN RESULTS: Only 38% of patients had discussed their preferences for resuscitation with their physician. Using logistic regression, we found that patients were less likely to have discussed resuscitation with their physician if they were nonwhite (odds ratio [OR], 0.49; 95% confidence interval [CI], 0.24 to 0.99), had never been hospitalized (OR, 0.52; 95% CI, 0.27 to 0.99), or were cared for in the HMO (OR, 0.44 relative to the private teaching hospital; 95% CI, 0.23 to 0.82). Patients were more likely to have discussed their preferences if they were not currently taking zidovudine (OR, 1.76; 95% CI, 1.02 to 3.03) and if they had decided to defer life-sustaining therapy (OR, 2.30; 95% CI, 1.35 to 3.91). Among nonwhites, those with a nonwhite physician were more likely to have discussed resuscitation (OR, 4.38; 95% CI, 1.13 to 16.93). Of patients who had not discussed their preferences for life-sustaining care, 72% wanted to do so. Patient desire for discussion of this issue did not vary by race, severity of illness, hospitalization status, use of zidovudine, or site of care. CONCLUSIONS: A majority of persons with AIDS in this study had not discussed their preferences for life-sustaining care with their physician, despite the desire to do so. Interventions to improve patient-physician communication about resuscitation for nonwhites and other groups at risk of inadequate discussion might lead to clinical decisions that are more consistent with patient preferences.

The use of female spouse proxies in common symptom reporting.

To determine the quality of proxy health reports by telephone the 1984 Wisconsin Health Status Survey employed a repeated-measure design in an interview covering 22 recently occurring health and psychologic complaints. Comparisons on individuals in households containing two or more adults revealed a relatively weak correspondence between the respondent and proxy reports. While a previous analysis of these data found that certain characteristics thought to underlie reporting differences are not useful in explaining proxy underreporting, the current analysis focuses on spousal pairs and achieves greater success by applying somewhat more sophisticated methods. The authors examined the nature, persistence, and number of health complaints as factors in reporting bias. They find evidence that female proxies vary by symptom in their ability to report common complaints and also observed that proxy underreporting diminishes somewhat as the persistence of symptoms increases. Using multiplicative models, the authors show that the gross misclassification of complaints is concentrated in the respondent-proxy pairs with the shortest exposure to the symptom. Finally, an examination of the disagreements on all 22 health complaints simultaneously revealed that neither mutual misallocation by respondents and proxies nor a diminished health status of the individual reported on are important influences on reporting behavior.