Good health care for a good life?:The case of down syndrome

People with Down syndrome have complex health care needs which are not always fully met. Health care improvements are required to better meet these needs. Quality indicators are an important tool for improving health care. However, quality indicators for health care for people with Down syndrome are scarce. Existing quality indicators focus on medical (physical) needs or the clinical setting, even though it is acknowledged that quality measures should reflect the total of quality aspects relevant to the population at stake, which may encompass aspects beyond the medical domain. These aspects beyond the medical domain are the focus of the current paper, which aims to provide insight into the way people with Down syndrome live their lives, how health care may fit in, and how this may impact the development of quality indicators. The paper is based on data originating from interviews with people with Down syndrome and their parents as well as focus groups with support staff members working in assisted living facilities for people with intellectual disability. The data revealed a lot of variation in how people with Down syndrome live their lives. Nevertheless, we were able to identify 11 topics, which we grouped into three overarching themes: (1) Being different yet living a normal life; (2) Down syndrome-(un)friendly society and services; and (3) family perspective. The variation in our data stresses the importance of health care that takes a person's life into account beyond the medical domain, as exemplified by the identified topics. Our findings also show that a good life is not merely depending on good health care supported by well-defined quality indicators, but on (support in) all life domains

Decreased antibody response after severe acute respiratory syndrome coronavirus 2 vaccination in patients with Down syndrom

The risk of a severe course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults with Down syndrome is increased, resulting in an up to 10-fold increase in mortality, in particular in those >40 years of age. After primary SARS-CoV-2 vaccination, the higher risks remain. In this prospective observational cohort study, SARS-CoV-2 spike S1-specific antibody responses after routine SARS-CoV-2 vaccination (BNT162b2, messenger RNA [mRNA]-1273, or ChAdOx1) in adults with Down syndrome and healthy controls were compared. Adults with Down syndrome showed lower antibody concentrations after 2 mRNA vaccinations or after 2 ChAdOx1 vaccinations. After 2 mRNA vaccinations, lower antibody concentrations were seen with increasing age. In this prospective cohort study that included 222 adults with Down syndrome, a significantly lower antibody response was found after SARS-CoV-2 mRNA or vector vaccination compared to healthy controls. After mRNA vaccination, lower antibodies were found with increasing age

Decreased Antibody Response After Severe Acute Respiratory Syndrome Coronavirus 2 Vaccination in Patients With Down Syndrome

UNLABELLED: The risk of a severe course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults with Down syndrome is increased, resulting in an up to 10-fold increase in mortality, in particular in those >40 years of age. After primary SARS-CoV-2 vaccination, the higher risks remain. In this prospective observational cohort study, SARS-CoV-2 spike S1-specific antibody responses after routine SARS-CoV-2 vaccination (BNT162b2, messenger RNA [mRNA]-1273, or ChAdOx1) in adults with Down syndrome and healthy controls were compared. Adults with Down syndrome showed lower antibody concentrations after 2 mRNA vaccinations or after 2 ChAdOx1 vaccinations. After 2 mRNA vaccinations, lower antibody concentrations were seen with increasing age. CLINICAL TRIALS REGISTRATION: NCT05145348

The behavioral and psychological symptoms of dementia in down syndrome (BPSD-DS) scale:comprehensive assessment of psychopathology in down syndrome

Behavioral and psychological symptoms of dementia (BPSD) have not been comprehensively studied in people with Down syndrome, despite their high risk on dementia. A novel evaluation scale was developed to identify the nature, frequency and severity of behavioral changes (83 behavioral items in 12 clinically defined sections). Central aim was to identify items that change in relation to the dementia status. Structured interviews were conducted with informants of people with Down syndrome without dementia (DS, N = 149), with questionable dementia (DS + TD, N = 65) and with diagnosed dementia (DS + AD, N = 67). Group comparisons showed a pronounced increase in frequency and severity of items about anxiety, sleep disturbances, agitation & stereotypical behavior, aggression, apathy, depressive symptoms, and, eating/drinking behavior. The proportion of individuals presenting an increase was highest in the DS + AD group and lowest in the DS group. Interestingly, among DS + TD individuals, a substantial proportion already presented increased anxiety, sleep disturbances, apathy and depressive symptoms, suggesting that these changes may be early alarm signals of dementia. The scale may contribute to a better understanding of the changes, adapting daily care/support, and providing suitable therapies to people with Down syndrome. The scale needs to be optimized based on the results and experiences. The applicability, reliability and validity require further study

Early Age at Menopause is Associated with Increased risk of Dementia and Mortality in Women with Down Syndrome

In a prospective longitudinal cohort study of dementia and mortality in persons with Down syndrome aged 45 years and older, 85 postmenopausal women were followed for a mean follow-up time of 4.3 years (range 0.0 to 7.4 years). The effect of age at menopause on age at diagnosis of dementia and survival was estimated using correlation analysis and Cox Proportional Hazard Model. We found a significant correlation between age at menopause and age at diagnosis of dementia (rho = 0.52; p <0.001), and between age at menopause and age at death (rho = 0.49; p = 0.01). Early age at menopause is associated with a 1.8 fold increased risk of dementia: Hazard Ratio (HR): 1.82 (95% Confidence Interval (CI): 1.31-2.52) and with risk of death: HR: 2.05 (95% CI: 1.33-3.16). Our study suggests that age at menopause in women with Down syndrome is a determinant of age at onset of dementia and mortalit