Causes of death among United States decedents with ALS: An eye toward delaying mortality.

OBJECTIVE: To report multiple cause of death (MCOD) occurrence among patients in the United States with amyotrophic lateral sclerosis (ALS). METHODS: Using death certificate data for all ALS deaths from 50 U.S. states and the District of Columbia, 2011-2014, we tabulated MCOD, used association rules mining (ARM) to determine if MCOD occurred together, and calculated standardized mortality odds ratios (SMOR) for select causes, comparing ALS with other U.S. decedents. RESULTS: Among 24,328 death certificates, there were 25,704 MCOD, excluding ALS. ALS was listed as the sole cause of death in n = 11,263 (46%). The most frequent causes of death co-occurring with ALS were respiratory failure (n = 6503; 25.3%), cardiovascular disease (n = 6077; 12.6%), pneumonia (n = 1345; 5.2%), and pneumonitis (n = 856; 3.3%). The SMORs among ALS decedents compared with non-ALS decedents for falls and accidents were 3.4 (95% CI 2.6, 4.3) and 3.0 (95% CI 2.2, 4.2), respectively. From ARM analysis, falls and accidents were both associated with injuries. The most common causes identified were weakly to very strongly associated with being an ALS decedent compared with other U.S. deaths, with SMOR point estimates ranging from 1.3 to 51.1. INTERPRETATION: This study provides information about the natural history of ALS. With knowledge that some causes of death may be preventable, healthcare providers may be able to optimize patient care and possibly postpone mortality and reduce morbidity. Moreover, this study located gaps in data; medical certifiers completing death certificates for ALS decedents should ensure all MCOD data are recorded.

Survival and epidemiology of amyotrophic lateral sclerosis (ALS) cases in the Chicago and Detroit metropolitan cohort: incident cases 2009-2011 and survival through 2018.

Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disorder. The National ALS Registry launched surveillance projects to understand the distribution of ALS in targeted geographic cohorts. OBJECTIVE: To describe the demographics, incidence, and survival of persons with ALS (PALS) identified in the Chicago and Detroit area population-based cohort. METHODS: Neurologists in the catchment area provided case reports for eligible ALS cases diagnosed and/or cared for from 1 January 2009 through 31 December 2011. Crude incidence rates were calculated for 2009-2011 and stratified by race and ethnicity. Using data from the National Death Index through 2018, we modeled the effect of patient covariates on mortality using the Cox proportional hazard regression. RESULTS: Of the 574 cases, 372 (64.8%) were diagnosed from 2009 to 2011. The combined crude incidence rates for 2009, 2010, and 2011 were 1.44, 1.53, and 1.73 cases per 100,000 person-years, respectively. Of the 486 subjects with complete survival data, 81% were deceased at the end of follow-up. Median survival time was 2.2 years, with 30% and 9% of subjects surviving past 5 and 10 years after diagnosis, respectively. Additionally, female PALS and PALS with longer time between symptom onset and diagnosis experienced longer survival. Nonwhites also experienced longer survival than Whites, except for those cases diagnosed in the younger age categories. CONCLUSION: Understanding the survival of ALS patients can aid in understanding variable prognostic factors, which can potentially extend survival and improve disease management.

Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016.

Objective: To estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2016 using data from the National ALS Registry (Registry). Established in 2009, the Registry collects data on ALS patients in the U.S. to better describe the epidemiology of ALS, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with the disease. Methods: To identify adult prevalent cases of ALS, the Registry compiles data from three national administrative databases (maintained by the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration). To ascertain cases not necessarily included in these databases and to better understand risk-factors associated with ALS and disease progression, the Registry also includes data collected from patients who voluntarily enroll via a web portal to complete online surveys. Results: In 2016, the Registry conservatively identified 16,424 adult persons who met the Registry definition of ALS for an age-adjusted prevalence rate of 5.2 per 100,000 U.S. population. The pattern of patient characteristics (e.g., age, sex, and race/ethnicity) has not changed from previous Registry reports. Overall, ALS was more common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of ALS cases were persons aged 18-39 years. Males had a higher prevalence rate of ALS than females overall and across all data sources. Conclusions: Data collected by the National ALS Registry are being used to better describe the epidemiology and demographics of ALS in the U.S.

History of vigorous leisure-time physical activity and early onset amyotrophic lateral sclerosis (ALS), data from the national ALS registry: 2010-2018.

Background: Previous research has suggested that vigorous physical activity (VPA) during adolescence and early adulthood is associated with ALS. The National ALS Registry (Registry) collects physical activity data from persons with ALS. Objective: To examine the association between vigorous VPA and early onset ALS, defined as a diagnosis before age 60, among patients enrolled in the Registry. VPA was defined as engaging in dynamic exercise for at least 10 minutes in a session that caused heavy sweating or large increases in breathing or heart rate. Methods: A cross-sectional study was conducted of 5463 ALS patients with VPA history and 956 ALS patients who never engaged in VPA. Patient characteristics were collected via online surveys in the following areas: demographic, lifetime VPA history, and initial onset of symptoms. General linear modeling was used to estimate mean age of diagnosis and to compute 95% confidence intervals. Results: Patients who reported engaging in VPA at least moderately (three times a week) during early adulthood were more likely to have an ALS diagnosis earlier compared to patients who did not (p < 0.0001). After controlling for year of birth, statistically significant associations between those reporting VPA at age 15-24 and 25-34 and diagnosis of ALS earlier (p = 0.0009, p = 0.0144 respectively). Conclusion: Patients with ALS who had a history of VPA before age 35, were significantly more likely to be diagnosed with ALS before age 60 compared to patients with ALS who never engaged vigorously. More research is needed in the relationship between VPA and early onset ALS.

High incidence of brain and other nervous system cancer identified in two mining counties, 2001-2015.

Two counties in Montana, Deer Lodge and Silver Bow (DL/SB), have two Superfund sites, as well as an active copper and molybdenum mine in SB. The population living in proximity to these sites are exposed to additional metals and some have been shown to be neurotoxic, especially for children; thus, this study focused on the incidence of brain and other nervous system cancers. The Montana Central Tumor Registry data was used to identify the cases in DL/SB and the remaining 54 counties of Montana (comparison group). After controlling for sex, cancer stage, and year of diagnosis, we found an incidence rate ratio for DL/SB versus comparison group of 6.28 (95% CI: 2.32-17.02) for children ages birth to 4 years, and 3.95 (95% CI: 1.66-9.38) for adults age 30-34 years. The high incidence rate of the brain cancer in the two age groups requires public health action.

Fecundity and Infertility Among Women with Disabilities in the United States.

Background: Knowledge of fecundity and infertility in women with a disability (WWD) is limited. This study aims to compare the fecundity and infertility experiences of women with a self-identified disability, in domains of sensory (hearing and vision loss), cognitive (difficulty concentrating, remembering, and making decisions), and physical (difficulty walking or climbing stairs) disabilities, with those without a self-identified disability. Materials and Methods: Cross-sectional survey data from the National Survey of Family Growth (NSFG), 2011-2015, were analyzed. A final analytic sample of 383 women from the NSFG, aged 18-44 years, was included to study the fecundity rates of WWD and women without a disability, using the current duration approach. Results: Fecundity hazard ratios (FHRs) and the proportion of women experiencing infertility after 12 months of actively attempting pregnancy were reported for the comparison group and by each disability type. Women with a self-identified cognitive disability experienced significant decreases in fecundity (FHR = 0.56; 95% confidence interval [CI]: 0.30-0.88) when adjusting for age, education level, parity, living in a metropolitan area, and infertility services. Furthermore, the estimated proportion of infertile women without a disability was 0.38 (95% CI: 0.23-0.62) versus 0.51 (95% CI: 0.34-0.72) for women with a self-identified cognitive disability. Conclusions: Women with a self-identified cognitive disability experienced significant decreases in fecundity.

Parents' stories of sensitive and insensitive care by paediatricians in the time leading up to and including diagnostic disclosure of a life-limiting condition in their child.

UNLABELLED: This study explored parents' experiences of care by paediatricians in the time leading up to and including diagnostic disclosure of a life-limiting condition in their child. RESEARCH METHODS: Qualitative exploration using in depth interviews with a purposive sample of 30 families whose child was diagnosed with a life-limiting condition. RESULTS: Parents' stories centred upon their need to have their concerns and needs as parents validated by paediatricians. Demonstrable qualitative differences were shown to exist between the sensitive and insensitive clinical practices of paediatricians. Parents' stories identify the need for clinical practice to be based upon a humanistic approach to care. These also identify how sensitive paediatricians achieved this ideal through meeting the 'double obligation' of combining technical skills with an understanding of their needs as parents.