The ALS Patient Care Database (ALS C.A.R.E) (1996-2005) was the first large-scale effort to track the disease course and outcomes of patients with amyotrophic lateral sclerosis. The project enrolled over 6000 patients from more than 300 clinical sites in the USA. Long-term follow-up was conducted through clinical assessment, patient self-reported questionnaires, and caregiver assessment. The ALS C.A.R.E. provided researchers and clinicians with the opportunity to gain insights into factors that may influence disease progression and to develop management plans that optimized patient function and quality of life. The database was endorsed by the World Federation of Neurology. This collection showcases publications about the project and project research.


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Recently Published

  • The Amyotrophic Lateral Sclerosis Patient Care Database

    Miller, Robert G.; Champion, Stacey M.; Forshew, Dallas A.; Anderson, Frederick A. Jr. (2009-01-01)
  • Factors correlated with NPPV use in ALS

    Jackson, Carlayne E.; Lovitt, Steven; Gowda, Neelam; Anderson, Frederick A. Jr.; Miller, Robert G. (2006-06-07)
    In spite of emerging evidence of therapeutic benefit from non-invasive positive pressure ventilation (NPPV), only a minority of ALS patients use this therapy. We examined factors which correlate with use of NPPV in ALS patients. Data were analyzed from the ALS CARE Database on the use of NPPV in patients with FVC less than 50% of predicted and probable or definite ALS based on modified El Escorial criteria. Of the 403 eligible patients, 146 (36%) used NPPV. NPPV compliance was strongly correlated with symptoms of dyspnea and orthopnea as well as with the use of other therapies including PEG tubes, augmentative speech devices, and riluzole. Male gender and household income >$80,000 were also associated with higher NPPV use. There was no correlation between age, race, type of insurance, forced vital capacity, duration of symptoms, ALSFRS-R, caregiver burden or quality of life with the use of NPPV. These data suggest that the factors which are most closely associated with NPPV utilization are symptomatic orthopnea and dyspnea. The findings may be useful in designing prospective studies to examine the factors which might explain the underutilization of NPPV and the optimal use of this treatment.
  • Changes in the management of ALS since the publication of the AAN ALS practice parameter 1999

    Bradley, W. G.; Anderson, Frederick A. Jr.; Gowda, Neelam; Miller, Robert G. (2004-12-01)
    OBJECTIVE: To determine if the publication in 1999 of the AAN Practice Parameter on ALS was associated with an improvement in the standard of management of the disease. METHODS: Data on 646 patients enrolled in the ALS CARE database and on 465 patients who died in the period May 2001 to November 2002 were compared with similar data obtained from the database from 1996 to May 1999. RESULTS: The specialty ALS clinics were the most important source of information about ALS. The internet was a source for 39%. The treatment of sialorrhea, pseudobulbar emotional lability, and failure of swallowing and breathing had all improved significantly in the period after the publication of the Practice Parameter. However many patients still did not receive a gastrostomy tube or non-invasive positive pressure ventilation when indicated by the Practice Parameter, mainly because of lack of patient compliance. Cost was the main reason why 41% of patients did not receive riluzole, though they spent a third of the cost of this medication on alternative medicines. CONCLUSIONS: The publication of the AAN Practice Parameter was associated with improvement in the standard of care. Most cases in the database come from specialized ALS centers, and further information on the community care of ALS patients is needed.
  • Use of noninvasive ventilation in patients with amyotrophic lateral sclerosis

    Lechtzin, Noah; Wiener, Charles M.; Clawson, Lora; Davidson, Matthew C.; Anderson, Frederick A. Jr.; Gowda, Neelam; Diette, Gregory B. (2004-03-19)
    INTRODUCTION: Noninvasive positive pressure ventilation (NIPPV) is associated with improved survival in amyotrophic lateral sclerosis (ALS) and has been widely recommended. The extent of NIPPV use in ALS patients and the factors associated with its use have not been studied. METHODS: A cross-sectional study using the ALS Patient Care Database. Analyses were performed to assess the association of patient and care characteristics with use of ventilatory support. RESULTS: 1458 patients were studied. 15.6% used NIPPV and 2.1% used invasive mechanical ventilation. Patients who used NIPPV were significantly more likely to be male and have higher income than those who did not. They were also more likely to have a gastrostomy tube, lower vital capacity, more severe disease, bulbar involvement and poorer general health status as measured by the SF-12 and Sickness Impact Profile. Multivariate analysis revealed that lower FVC, higher income and use of gastrostomy tube were independently associated with use of NIPPV. CONCLUSIONS: NIPPV is used more than seven times as frequently as invasive ventilation in ALS patients. Patients who use NIPPV have more severe disease than those who do not use any respiratory intervention. Patients with lower income are less likely to use NIPPV, which raises concerns about disparities in the care of patients with ALS.
  • Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction

    Mitsumoto, H.; Davidson, Matthew C.; Moore, D.; Gad, N.; Brandis, M.; Ringel, S.; Rosenfeld, J.; Shefner, J. M.; Strong, M. J.; Sufit, R.; et al. (2003-09-18)
    OBJECTIVE: To compare characteristics of ALS patients with and without percutaneous endoscopic gastrostomy (PEG). METHODS: Using the ALS Patient Care Database, data from patients with and without PEG with ALS Functional Rating Scale-bulbar subscale (ALSFRSb) scores < or = 5 were analyzed; follow-up data were also collected. RESULTS: PEG use was markedly increased with declining ALSFRSb scores. Demographics did not differ, but ALSFRS composite scores and bulbar and arm subscale scores were lower (P<0.0001). PEG patients used significantly more assistive devices, multidisciplinary care, home care nurses and aides, had more frequent physician and emergency department visits and hospital admissions (P<0.0001), and had lower health status based on the mini-SIP scale (P=0.0047). PEG use varied greatly between ALS centers. In the follow-up study, positive impact of PEG was noted in 79 % of PEG patients but in only 37.5% of patients who received PEG later, based on a small number of patients. PEG use showed no survival benefit. CONCLUSION: Patients did not receive PEG until bulbar function was severely reduced and overall ALS had markedly progressed. PEG may have been performed too late to demonstrate survival benefits. Aggressive proactive nutritional management appears essential in patients with ALS. To determine whether PEG provides benefits, it must be performed at earlier stages of the disease and prospectively studied.
  • The ALS Patient Care Database: insights into end-of-life care in ALS

    Mandler, R. N.; Anderson, Frederick A. Jr.; Miller, Robert G.; Clawson, Lora; Cudkowicz, M.; Del Bene, M. (2001-12-18)
    OBJECTIVE: To study clinical practices and patient outcomes near the end of life in amyotrophic lateral sclerosis (ALS). BACKGROUND: Patients, families, and healthcare providers face several dilemmas in selecting and delivering care near the end of life in ALS. Published data on clinical practices and their benefits during end-of-life care for ALS patients consist of anecdotal reports based on small case series or individual case reports. METHODS: Data were obtained from 1014 American and Canadian patients with ALS who died while participating in a large observational registry (the ALS Patient Care Database) during the past four years. Following death, a caregiver or family member provided data for each patient using a standard questionnaire. Data were principally generated through American and Canadian ALS multidisciplinary centers of excellence. RESULTS: Most patients died peacefully (90.7%) and 62.4% died in a hospice-supported environment. Advance directives were in place for 88.9% of patients and were followed in 96.8%. Among the 67 patients who exhibited distress in the dying process, symptoms included breathing difficulties (82.1%), fear/anxiety (55.2%), pain (23.9%), insomnia (14.9%), and choking (14.93%). Oxygen was given to 52.6% of patients, and pain medications were given to 74%. CONCLUSION: These data suggest that palliative care at the end of life was relatively well managed for most patients with ALS who participated in this study; nevertheless, several opportunities for improvement were identified.
  • Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology

    Bradley, W. G.; Anderson, Frederick A. Jr.; Bromberg, M.; Gutmann, L.; Harati, Y.; Ross, M.; Miller, Robert G. (2001-08-15)
    BACKGROUND: The American Academy of Neurology (AAN) ALS Practice Parameter was published in April 1999. The ALS CARE Database has been collecting data on the management of patients with ALS in North America since 1996. OBJECTIVE: To compare the management of patients with ALS in North America as recorded in the ALS CARE Database with the recommendations of the AAN ALS Practice Parameter. METHODS: Data were analyzed from 2018 patients at enrollment and from 373 of these patients who died between enrollment and May 1999. RESULTS: Eighty-two percent of the enrolled patients reported that they had been given enough information about ALS. Only 54% of patients with drooling were receiving medication for this problem. Only 41% of those who reported being depressed most of the time were receiving antidepressant medications. Only 28% of those with dyspnea and only 9.2% of those with a forced vital capacity <40% predicted were receiving noninvasive positive pressure ventilator support. Only 30% of those with moderate to severe dysphagia had a gastrostomy tube. Half of the patients who died did so at home, but only 47% of them received residential hospice services. Although 89% of patients who died were recorded as having done so peacefully, 17% were reported to have had breathing difficulties (i.e., respiratory distress), 8% anxiety, 3.3% pain, and 2.5% choking. Advance directives were in place for 90% of the patients who died, and in 97% of cases these directives were followed. CONCLUSIONS: These findings indicate that in the 3-year period prior to the publication of the AAN Practice Parameter, many but not all patients received the care that is recommended in that parameter; there were deficiencies, particularly in the key areas of gastrostomy and noninvasive positive pressure ventilation.
  • Assessing health status quality of life in ALS: comparison of the SIP/ALS-19 with the ALS Functional Rating Scale and the Short Form-12 Health Survey. ALS C.A.R.E. Study Group. Clinical Assessement, Research, and Education

    Bromberg, M. B.; Anderson, Frederick A. Jr.; Davidson, Matthew C.; Miller, Robert G. (2001-03-24)
    BACKGROUND: The progressive loss of function affects the quality of life of the ALS patient. Quality of life depends on a spectrum of factors. Available instruments for assessment include questionnaires developed for generic illness-related disabilities and ALS-specific questionnaires that focus on function. METHODS: Comparisons were made between two ALS-specific questionnaires (ALS Functional Rating Scale and SIP/ALS-19) and a generic instrument (Short Form-12) given to 1,513 patients from the (North American) ALS Patient Care Database. RESULTS: The SIP/ALS-19 correlates well with the ALS Functional Rating Scale and less well with the Short Form-12. CONCLUSIONS: The SIP/ALS-19 can be used as an effective surrogate for the ALS Functional Rating Scale, with the advantage that the SIP/ALS-19 also includes questions that encompass the psychological and social domains of quality of life.
  • Risk factors in the early diagnosis of ALS: North American epidemiological studies. ALS CARE Study Group

    Brooks, B. R.; ALS CARE Study Group (2000-03-24)
    Patient-specific (endogenous) and population-specific (exogenous) risk factor analysis is identifying novel physical and chemical exposures which might be time-linked to the development of amyotrophic lateral sclerosis (ALS) and other motor neuron diseases. Electric injury in a number of case-control studies as well as prolonged exposure at work and home to agricultural chemicals in pesticides and herbicides have been identified as significant risk factors. Heavy exercise, trauma with or without bone fractures and heavy metal exposure at work have not been confirmed as risk factors. Surprisingly, occupation as a pilot or navigator has recently been identified as a potential risk factor, which will need to be confirmed. The introduction of international patient registries in North America (ALS CARE) and in Europe (ALS HPS) will facilitate future studies on the prognosis of ALS, adherence to standards of practice, quality of life and patient outcome studies. An initial survey of the ALS Patient Care Database in January 1999, when nearly 1800 patients had been entered across North America, indicated the median time from ALS onset to diagnosis is 14 months when no second opinion is requested, 12 months if the patient requests a second opinion and 10 months when the neurologist requests an additional opinion. No significant difference was found in the median time to diagnose sporadic ALS patients compared with familial ALS patients.
  • The ALS patient care database: goals, design, and early results. ALS C.A.R.E. Study Group

    Miller, Robert G.; Anderson, Frederick A. Jr.; Bradley, W. G.; Brooks, B. R.; Mitsumoto, H.; Munsat, T. L.; Ringel, S. P. (2000-01-15)
    OBJECTIVE: The ALS Patient Care Database was created to improve the quality of care for patients with ALS by 1) providing neurologists with data to evaluate and improve their practices, 2) publishing data on temporal trends in the care of patients with ALS, and 3) developing hypotheses to be tested during formal clinical trials. BACKGROUND: Substantial variations exist in managing ALS, but there has been no North American database to measure outcomes in ALS until now. METHODS: This observational database is open to all neurologists practicing in North America, who are encouraged to enroll both incident and prevalent ALS patients. Longitudinal data are collected at intervals of 3 to 6 months by using standard data collection instruments. Forms are submitted to a central data coordinating center, which mails quarterly reports to participating neurologists. RESULTS: Beginning in September 1996 through November 30, 1998, 1,857 patients were enrolled at 83 clinical sites. On enrollment, patients had a mean age of 58.6 years +/-12.9 (SD) years (range, 20.1 to 95.1 years), 92% were white, and 61% were men. The mean interval between onset of symptoms and diagnosis was 1.2+/-1.6 years (range, 0 to 31.9 years). Riluzole was the most frequently used disease-specific therapy (48%). Physical therapy was the most common nonpharmacologic intervention (45%). The primary caregiver was generally the spouse (77%). Advance directives were in place at the time of death for 70% of 213 enrolled patients who were reported to have died. CONCLUSIONS: The ALS Patient Care Database appears to provide valuable data on physician practices and patient-focused outcomes in ALS.
  • ALS care: a resource for measuring and improving ALS outcomes

    Anderson, Frederick A. Jr.; Miller, Robert G. (1996-10-01)
    The Amyotrophic Lateral Sclerosis Clinical Assessment, Research, and Education Project (ALS CARE) will conduct outcomes research and develop educational programs that benefit ALS patients and neurologists. An advisory board of neurologists, who are experts in ALS, will establish the policies governing this project and control the dissemination of aggregate data on ALS practices and outcomes. As a first step toward improving the care of ALS patients, a data coordinating center has been established in the Center for Outcomes Research at the University of Massachusetts Medical Center, which will manage a North American Database of ALS Outcomes. This voluntary database is designed to (1) guide the development of educational programs to improve the care of ALS patients and (2) provide a mechanism for neurologists to evaluate the impact of their diagnostic and therapeutic decisions in a manner that is timely, confidential, and objective.