Evaluating the Acceptability and Social Validity of a Caregiver-Led Technology-Based Menstrual Hygiene Management Intervention for Youth on the Autism Spectrum.

Objectives: Menstrual hygiene management (MHM) is a critical area of daily living skills for neurodiverse menstruators in which few evidence-based interventions exist. This study evaluated the acceptability and social validity of a caregiver-mediated intervention for youth on the autism spectrum utilizing behavior skills training, animated video modeling, task analysis, and telehealth services to improve changing a menstrual pad. Methods: The consultant recruited three youth and caregiver dyads to test the intervention and collect acceptability and social validity assessments. Materials varied based on the phase of the study; however, a typical trial required data collection sheets, youth's underwear, a menstrual pad, and, during intervention, the video model and hardware technology. A multiple baseline design across participants and reversal design were used to demonstrate experimental effects. Due to the sensitive nature of the study, independence and accuracy data were collected by caregivers and analyzed later by the consultant through visual analysis. Results: Overall, youth participants improved their percentage of independent and accurate steps of changing a menstrual pad. Furthermore, caregivers reported moderate to high levels of acceptability of the telehealth service delivery and animated video modeling, as well as the overall MHM training program measured by the Technology Acceptability Model-Fast Form (FF-TAM) and the Behavior Intervention Rating Scale (BIRS), respectively. Youth reported high levels of acceptability of the MHM training program as measured by the Child Intervention Rating Profile (CIRP). Conclusions: Results suggest that a technology-based, caregiver-mediated MHM intervention for neurodiverse youth would be considered acceptable and socially valid by caregiver and youth participants. Supplementary Information: The online version contains supplementary material available at 10.1007/s41252-022-00261-x.

Assessment of ataxia phenotype in a new mouse model of galactose-1 phosphate uridylyltransferase (GALT) deficiency.

Despite adequate dietary management, patients with classic galactosemia continue to have increased risks of cognitive deficits, speech dyspraxia, primary ovarian insufficiency, and abnormal motor development. A recent evaluation of a new galactose-1 phosphate uridylyltransferase (GALT)-deficient mouse model revealed reduced fertility and growth restriction. These phenotypes resemble those seen in human patients. In this study, we further assess the fidelity of this new mouse model by examining the animals for the manifestation of a common neurological sequela in human patients: cerebellar ataxia. The balance, grip strength, and motor coordination of GALT-deficient and wild-type mice were tested using a modified rotarod. The results were compared to composite phenotype scoring tests, typically used to evaluate neurological and motor impairment. The data demonstrated abnormalities with varying severity in the GALT-deficient mice. Mice of different ages were used to reveal the progressive nature of motor impairment. The varying severity and age-dependent impairments seen in the animal model agree with reports on human patients. Finally, measurements of the cerebellar granular and molecular layers suggested that mutant mice experience cerebellar hypoplasia, which could have resulted from the down-regulation of the PI3K/Akt signaling pathway.