Assessing the impact of grief on quality of life, work productivity, and health outcomes for parents bereaved from SMA: A study protocol.

BACKGROUND: U.S. cost-effectiveness recommendations suggest that analyses should include all costs and effects relevant to the decision problem [1]. However, in many diseases, including spinal muscular atrophy (SMA), few studies have evaluated bereaved family outcomes after a child has died, neglecting potential impacts on their health-related quality of life (HRQoL), work productivity, and mental health. Additionally, grief-related outcomes are rarely included in economic evaluations. This manuscript outlines the protocol of a study that will estimate the HRQoL, work functioning, and mental health of bereaved parents of children with SMA type 1 to determine how outcomes vary based on parent's sex and the time since a child's death. METHODS: This study will involve two phases. In Phase 1, we will conduct a literature review to identify prior research that has measured how parental grief impacts HRQoL, work productivity, and mental health. We will also interview four bereaved parents of children with SMA type 1, stratified by parent sex and time since their child's death, and analyze findings using a thematic analysis. In Phase 2, we will develop a survey draft based on Phase 1 findings. Parents bereaved from SMA type 1 will review our survey draft and we will revise the survey based on their feedback. We will send a cross-sectional survey to approximately 880 parents bereaved from SMA type 1. We will analyze findings from the survey to investigate whether the severity of grief symptoms is correlated with HRQoL, productivity, depression and anxiety symptom severity. We will also evaluate whether the mean scores of grief and each of the outcomes vary significantly when stratified by parent sex and the time since the child's death. DISCUSSION: Our results will provide preliminary information on how parental grief can impact HRQoL, productivity, and mental health outcomes over time. Increasing the availability of family outcomes data will potentially assist organizations performing health economic evaluations, such as the Institute of Clinical and Economic Review (ICER) in the U.S. This research will also help to inform the development of future economic guidelines on this topic.

Using occupations to improve quality of life, health and wellness, and client and caregiver satisfaction for people with Alzheimer's disease and related dementias.

An evidence-based review was undertaken to answer the question, "What is the evidence for the effect of interventions designed to establish, modify, and maintain activities of daily living (ADLs), instrumental activities of daily living (IADLs), leisure, and social participation on quality of life (QOL), health and wellness, and client and caregiver satisfaction for people with Alzheimer's disease and related dementias?" A systematic search of electronic databases and application of inclusion and exclusion criteria guided the selection of 26 articles. Limited high-level evidence on ADL interventions was identified. IADL interventions for people living in the community showed promise. Tailored and activity-based leisure interventions were common and seemed to have positive impacts on caregiver satisfaction, and some interventions had positive results for client well-being and QOL. Social participation interventions focused on people with dementia still able to engage in verbal social interactions; these interventions had at least short-term positive effects.

Effectiveness of interventions designed to modify and maintain perceptual abilities in people with Alzheimer's disease and related dementias.

An evidence-based review was undertaken to answer the question, "What is the evidence for the effect of interventions designed to modify and maintain perceptual abilities on the occupational performance of people with Alzheimer's disease and related dementias?" A systematic search of electronic databases and application of inclusion and exclusion criteria guided the selection of 31 articles. Each article was critically appraised, and the evidence was synthesized. Some interventions use remaining perceptual abilities to enable people to find their way in a facility and decrease attempts at exiting. Preliminary evidence has supported use of visual stimulation and barriers. We found some evidence for the use of auditory stimuli and group therapy that aim to change perceptual abilities. Research with high-level evidence is required to validate these findings. Evidence on the benefits of Snoezelen is not conclusive for occupational performance outcomes; further research to justify its use as an occupational therapy intervention may be warranted.

The Cure SMA Membership Surveys: Highlights of Key Demographic and Clinical Characteristics of Individuals with Spinal Muscular Atrophy.

BACKGROUND: Cure SMA maintains the largest patient-reported database for people affected with spinal muscular atrophy (SMA). In 2017, Cure SMA initiated annual surveys with their membership to collect demographic and disease characteristics, healthcare, and burden of disease information from patients and caregivers. OBJECTIVE: To summarize results from two large-scale Cure SMA surveys in 2017 and 2018. METHODS: Cure SMA database members were invited to complete surveys; these were completed by caregivers for living or deceased individuals with SMA and/or affected adults. RESULTS: In 2017, 726 surveys were completed for 695 individuals with SMA; in 2018, 796 surveys were completed for 760 individuals with SMA. Data from both survey years are available for 313 affected individuals. Age at symptom onset, distribution of SMN2 gene copy number, and representation of each SMA type in the surveys were consistent with that expected in the SMA population. In the 2018 survey, the average age at diagnosis was 5.2 months for SMA type I and the reported mean age at death for this subgroup was 27.8 months. Between survey years, there was consistency in responses for factors that should not change within individuals over time (e.g., reported age at diagnosis). CONCLUSIONS: Results from the Cure SMA surveys advance the understanding of SMA and facilitate advocacy efforts and healthcare services planning. Longitudinal surveys are important for evaluating the impact of effective treatments on changing phenotypes, and burden of disease and care in individuals with SMA.

Gambling task performance in traumatic brain injury: relationships to injury severity, atrophy, lesion location, and cognitive and psychosocial outcome.

OBJECTIVE: To determine the sensitivity of the Gambling Test (GT) to the neurocognitive effects of traumatic brain injury (TBI) and to examine the cognitive, neural, and psychosocial correlates of impaired GT performance in patients with TBI. BACKGROUND: The GT is sensitive to behavioral deficits in patients with prefrontal brain damage, especially in ventral regions. Patients with TBI and behavioral deficits often have focal ventral prefrontal damage as well as diffuse damage. Analysis of the correlates of the GT in this population has implications for interpretation of the GT in other groups. METHOD: Seventy-one TBI patients were administered the GT, neuropsychological tests, and psychosocial outcome questionnaires. Patients also had high-resolution structural magnetic resonance imaging analyzed for both lesion location and tissue compartment volumes. RESULTS: The GT was sensitive to TBI in general, but not to TBI severity or quantified chronic phase atrophy. Marked impairment was observed in (but not limited to) patients with large frontal lesions. There were modest correlations between the GT and tests of working memory and executive functioning as well as between self- and other-rated real-life memory, executive, and emotional problems. CONCLUSIONS: The GT can be a useful adjunct to assessment of patients with TBI. Interpretation of GT performance in patients with complex neuropsychological deficits such as TBI should consider the influence of domain-general resources in addition to specific ventral prefrontal function.