"WOOP is my safe haven": A qualitative feasibility and acceptability study of the Wish Outcome Obstacle Plan (WOOP) intervention for spouses of people living with early-stage dementia.

OBJECTIVES: As symptoms emerge and worsen in people living with dementia, their spouses can benefit from behavioral interventions to support their adjustment as a care partner. The Wish Outcome Obstacle Plan (WOOP) intervention improves the well-being of spouses of people living with dementia early in the disease course, but intervention mechanisms and opportunities for improvement are unclear. The present study gave voice to spouses who participated in a trial of WOOP, describing how WOOP was incorporated into their lives and how it could be improved for future implementation. METHOD: For this qualitative study, we conducted longitudinal semi-structured interviews among 21 spouses of people living with dementia (three interviews over three months; 63 interviews total). Codebook thematic analysis was performed. RESULTS: Three meta-themes were derived: (1) assessing baseline strengths and limitations of WOOP, (2) learning from experience, and (3) fine-tuning and sustaining WOOP. Participants described how WOOP addressed their interpersonal and emotional stressors, their responses to behaviors of the person living with dementia, and their relationship quality. Considerations for future intervention delivery (e.g., solo vs. in group settings) and instructions (e.g., encouraging writing vs. thinking through the four steps of WOOP) were identified as areas of improvement. CONCLUSIONS: WOOP was described as a practical, feasible, and desirable intervention for spouses at the early stages of their partner's dementia. Participants made WOOP easier to incorporate in their everyday lives by adapting the design into a mental exercise that they used as needed. Suggestions from participants specified how to make the everyday use of WOOP more feasible, sustainable, and applicable in a variety of contexts. TRIAL REGISTRATION: ClinicalTrials.gov HIC 2000021852.

Psychological Responses to Intermittent Activities in Children With and Without Asthma.

PURPOSE: The purpose of this study was to examine the psychological responses to intermittent activities of varying intensities and types among children with and without asthma. METHODS: A total of 37 children and adolescents (51% male, aged 8-16 y, 54% nonwhite, and 54% without asthma) participated in this study. Participants completed 5 exercises in the same order: self-paced walking, resistance activities, dance video, gamified obstacle course, and step test. In-task mood was assessed using the Feeling Scale, in-task perceived exertion was assessed via the ratings of perceived exertion scale, and postactivity enjoyment was assessed using the Physical Activity Enjoyment Scale. RESULTS: There was a significant main effect of exercise type on mood (P < .001), ratings of perceived exertion (P < .001), and enjoyment (P < .002). There was not a significant main effect of asthma status on mood, ratings of perceived exertion, or enjoyment (Ps > .05). Children with asthma reported significantly lower in-task mood during the step exercise (P < .037) and reported significantly lower postactivity enjoyment after the walk and obstacle course exercises (Ps < .03). CONCLUSIONS: Regardless of differences by asthma status for in-task mood during the obstacle course and for postactivity enjoyment during the walk and step exercises, both children with and without asthma reported high in-task mood and postactivity enjoyment during all 5 exercises.

A Rare BSEP Mutation Associated with a Mild Form of Progressive Familial Intrahepatic Cholestasis Type 2.

Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation. Here, we describe two siblings with an extremely mild form of PFIC2 inherited from heterozygous parents. The elder sibling had acute liver failure at the age of six months and both siblings had pruritus, cholestasis, coagulopathy and fat-soluble-vitamin deficiencies in infancy but have been asymptomatic past infancy. Genetic testing of the siblings revealed that each were compound heterozygotes for two missense mutations of the ABCB11 gene: p.C68Y and p.R832H. Medical treatment typical for PFIC2 has not been necessary for either patient. This is the first report of these variants following a mild course in two affected patients.

Beneficial Effect of Societal Factors on APOE-ε2 and ε4 Carriers' Brain Health: A Systematic Review.

BACKGROUND: Apolipoprotein-E (APOE) ε4 and ε2 are the most prevalent risk-increasing and risk-reducing genetic predictors of Alzheimer's disease, respectively. However, the extent to which societal factors can reduce the harmful impact of APOE-ε4 and enhance the beneficial impact of APOE-ε2 on brain health has not yet been examined systematically. METHODS: To fill this gap, we conducted a systematic review searching for studies in MEDLINE, Embase, PsycINFO, and Scopus until June 2023, that included: (a) 1 of 5 social determinants of health (SDH) identified by Healthy People 2030, (b) APOE-ε2 or APOE-ε4 allele carriers, (c) cognitive or brain-biomarker outcomes, and (d) studies with an analysis of how APOE-ε2 and/ or APOE-ε4 carriers differ on outcomes when exposed to SDH. RESULTS: From 14 076 articles retrieved, 124 met the inclusion criteria. In most of the studies, exposure to favorable SDH reduced APOE-ε4's detrimental effect and enhanced APOE-ε2's beneficial effect on cognitive and brain-biomarker outcomes (cognition: 70.5%, n: 74/105; brain-biomarkers: 71.4%, n: 20/28). A similar pattern of results emerged in each of the 5 Healthy People 2030 SDH categories, where finishing high school, having resources to satisfy basic needs, less air pollution, less negative external stimuli that can generate stress (eg, negative age stereotypes), and exposure to multiple favorable SDH were associated with better cognitive and brain health among APOE-ε4 and APOE-ε2 carriers. CONCLUSIONS: Societal factors can reduce the harmful impact of APOE-ε4 and enhance the beneficial impact of APOE-ε2 on cognitive outcomes. This suggests that plans to reduce dementia should include community-level policies promoting favorable SDH.

Therapeutic Use of Music, Dance, and Rhythmic Auditory Cueing for Patients with Huntington's Disease: A Systematic Review.

BACKGROUND: Studies have assessed the therapeutic effect of music, dance, and rhythmic auditory cueing for patients with Huntington's disease (HD). However, the synthesis of evidence in support of their positive impact on symptoms is lacking. OBJECTIVE: We conducted a systematic literature review to evaluate the potential benefits of music, dance, and rhythm on the cognitive, psychiatric and motor function in patients with HD. METHODS: Two- and three-keyword searches and a manual search identified medical literature published from 1999 through 2019. We considered literature that assessed outcomes of art-based rehabilitation programs or individual modalities for persons with early, middle, or advanced HD. Structured analysis was conducted using data entry tables with categories for patient health status, art methods, and outcomes. RESULTS: Seven articles and six abstracts met eligibility criteria, of which nine evaluated art-based rehabilitation programs. Studies mainly assessed cognitive, psychiatric, and motor functions through music, dance, or rhythm modalities. Although results were conflicting, in summary improvements to motor function were dependent on disease severity and more responsive to art therapy programs than rhythm-motor synchronization. Benefits to global cognition that resulted from rhythmic training correlated with microstructural changes. Qualitative data verified a positive impact on language production, chorea, behavior, and quality of life. CONCLUSIONS: Our review has shown a potential benefit of music, dance, and rhythm for patients with HD, which is particularly important for a disease that has no cure. Art forms seemed to affect cognitive, psychiatric, motor, psychosocial, and neuroanatomical domains. However, evidence is preliminary, warranting further investigation to establish the foundation for this field.

A Rare BSEP Mutation Associated with a Mild Form of Progressive Familial Intrahepatic Cholestasis Type 2

ABSTRACT Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation. Here, we describe two siblings with an extremely mild form of PFIC2 inherited from heterozygous parents. The elder sibling had acute liver failure at the age of six months and both siblings had pruritus, cholestasis, coagulopathy and fat-soluble-vitamin deficiencies in infancy but have been asymptomatic past infancy. Genetic testing of the siblings revealed that each were compound heterozygotes for two missense mutations of the ABCB11 gene: p.C68Y and p.R832H. Medical treatment typical for PFIC2 has not been necessary for either patient. This is the first report of these variants following a mild course in two affected patients.(AU)