Optimizing Parent Training to Improve Oral Health Behavior and Outcomes in Underserved Children with Autism Spectrum Disorder.

A randomized controlled trial established initial efficacy of a novel parent training (PT) intervention for improving oral hygiene and oral health in underserved children with ASD (Fenning et al., 2022), a population at risk for unmet dental needs. The present study describes our emic approach to PT development alongside treatment outcome data examining feasibility, acceptability, and engagement. Families with Medicaid-eligible children with ASD ages 3 to 13 years (85% male, 62% with intellectual disability) were assigned to receive PT (n = 60) or a psychoeducational toolkit (n = 59). Results indicate strong retention, fidelity, and adherence, with quantitative and qualitative metrics revealing high treatment satisfaction and utilization. Discussion focuses on implications for individualizing treatment to optimize engagement of underrepresented families.

Parent Training for Dental Care in Underserved Children With Autism: A Randomized Controlled Trial.

OBJECTIVE: Children with autism spectrum disorder (ASD) have difficulty participating in dental care and experience significant unmet dental needs. We examined the efficacy of parent training (PT) for improving oral hygiene and oral health in underserved children with ASD. METHOD: Families of Medicaid-eligible children with ASD (ages 3-13 years, 85% boys, 62% with intellectual disability) reporting difficulty with dental care participated in a 6-month randomized controlled trial comparing PT (n = 60) with a psychoeducational dental toolkit (n = 59). Primary outcomes were parent-reported frequency of twice-daily toothbrushing and dentist-rated visible plaque. Secondary outcomes included parent-reported child behavior problems during home oral hygiene and dentist-rated caries. Dentists were blind to intervention assignment. Analyses were intention to treat. RESULTS: Retention was high at posttreatment (3 months, 93%) and 6-month follow-up (90%). Compared with the toolkit intervention, PT was associated with increased twice-daily toothbrushing at 3 (78% vs 55%, respectively; P < .001) and 6 (78% vs 62%; P = .002) months and a reduction in plaque at 3 months (intervention effect, -0.19; 95% confidence interval [CI], -0.36 to -0.02; P = .03) and child problem behaviors at 3 (-0.90; 95% CI, -1.52 to -0.28; P = .005) and 6 (-0.77; 95% CI, -1.39 to -0.14; P = .02) months. Comparatively fewer caries developed in children receiving the PT intervention over 3 months (ratio of rate ratios, 0.73; 95% CI, 0.54 to 0.99; P = .04). CONCLUSIONS: PT represents a promising approach for improving oral hygiene and oral health in underserved children with ASD at risk for dental problems.

Utilizing telehealth to create a clinical model of care for patients with Batten disease and other rare diseases.

The COVID-19 pandemic transformed the delivery of healthcare across the world. Telehealth has emerged as the primary method for providing healthcare early in the pandemic. Patient and healthcare provider views of the effectiveness of telehealth services are encouraging and support the long-term use of telehealth services in clinical practice. Telehealth may provide a strategy that has far-reaching benefits for diverse patient populations, such as patients with Batten disease and other rare diseases, who face additional barriers to accessing subspecialty healthcare services. The aims of this paper, through the experience of a single Batten Disease Center of Excellence, are to (1) review the benefits and barriers involved in the delivery of telehealth services to patients with rare diseases; (2) discuss components of a model for clinical care that utilizes telehealth services for patients with Batten disease; (3) discuss limitations and future directions of using telehealth in patients with rare diseases. Healthcare systems should consider building clinical models that utilize telehealth services to provide multidisciplinary services to patients with rare diseases. There are numerous benefits in using telehealth that can enhance and expand service delivery between the patient and clinician. Telehealth services can also improve provider-to-provider communication and collaboration when providing clinical care to individuals with rare diseases. Although there are many benefits to utilizing telehealth services in provision of care to patients with rare diseases, it is important to consider factors that may limit or add additional barriers prior to implementing telehealth services. There is a need for future collaborative research to examine and compare the effectiveness and outcomes of telehealth services with standard of care services that are provided in-person. Future research should also examine how to reduce the challenges and barriers associated with the implementation of telehealth services. Plain language summary: What is telehealth? Telehealth is defined by the US Department of Health Resources and Services Administrations1 as the "use of electronic information and telecommunication technologies to support long-distance clinical healthcare, patient and professional health-related education, public health, and health administration. Technologies include video conference, the internet, store-and-forward imaging, streaming media, and terrestrial and wireless communication." What was the aim of this review? This review was conducted to guide a clinical model using telehealth services for patients with Batten disease and other rare diseases based on the experiences of a single Batten Disease Center of Excellence. Why is this important? Individuals with rare diseases may face multiple barriers to accessing clinical services. Local doctors and treatment providers, such as speech therapists, occupational therapists, physical therapists, and psychologists, may not have knowledge of rare diseases or how to manage symptoms and disease progression, or how to guide treatment services. Other barriers may also include:• Lack of local resources;• Increased caregiver stress;• Difficulty obtaining a correct diagnosis.There are numerous benefits to using telehealth services for both patients with rare diseases, such as:• Convenience;• Cost savings;• Improved access to care;• Ability to see multiple providers that can help with symptom monitoring, assessment, and treatment services. Where do we go from here? It is important to consider limitations when creating a model for clinical care for patients with rare diseases. Some limitations to think about are:• Clinician and organization familiarity with telehealth;• Reimbursement and coverage from insurance companies for telehealth;• Security and privacy of patient information;• Training of telehealth providers;• Logistical factors, including use of equipment, internet/connectivity, and technical troubleshooting.Future directions should involve collaborative research that studies the effectiveness, feasibility, and perceptions of families of rare diseases and providers that use telehealth for clinical healthcare services. Research should also further study and consider ways to improve barriers and challenges associated with implementing telehealth systems into existing healthcare systems.

Behavioral Markers of Emergent Stranger Anxiety in Infants and Toddlers with Fragile X Syndrome.

Studying anxiety in neurogenetic syndromes may inform the intersection of biological and developmental risks, facilitating effective and targeted interventions. We longitudinally examined stranger fear in infants and toddlers with fragile X syndrome (FXS; n = 46) and typical controls (n = 33), as well as associations between observed stranger fear and rating scales of anxiety, withdrawal and autism features within FXS. Results indicated atypical facial fear in FXS, although facial fear did not index anxiety, autistic symptoms or social withdrawal. Instead, lower withdrawal was associated with decreased distress vocalizations across age, and higher autistic symptoms were associated with lower intensity escape behaviors. Early stranger fear in FXS reflects both typical and atypical dimensions and may help index emergence of social anxiety in this population.

Stranger Fear and Early Risk for Social Anxiety in Preschoolers with Fragile X Syndrome Contrasted to Autism Spectrum Disorder.

This study investigated behavioral indicators of social fear in preschool boys with fragile X syndrome (FXS) with a low degree of autism spectrum disorder (ASD) symptoms (FXS-Low; n = 29), FXS with elevated ASD symptoms (FXS-High; n = 25), idiopathic ASD (iASD; n = 11), and typical development (TD; n = 36). Gaze avoidance, escape behaviors, and facial fear during a stranger approach were coded. Boys with elevated ASD symptoms displayed more avoidant gaze, looking less at the stranger and parent than those with low ASD symptoms across etiologies. The iASD group displayed more facial fear than the other groups. Results suggest etiologically distinct behavioral patterns of social fear in preschoolers with elevated ASD symptoms.

HPA axis function predicts development of working memory in boys with FXS.

The present study examines verbal working memory over time in boys with fragile X syndrome (FXS) compared to nonverbal mental-age (NVMA) matched, typically developing (TD) boys. Concomitantly, the relationship between cortisol-a physiological marker for stress-and verbal working memory performance over time is examined to understand the role of physiological mechanisms in cognitive development in FXS. Participants were assessed between one and three times over a 2-year time frame using two verbal working memory tests that differ in complexity: memory for words and auditory working memory with salivary cortisol collected at the beginning and end of each assessment. Multilevel modeling results indicate specific deficits over time on the memory for words task in boys with FXS compared to TD controls that is exacerbated by elevated baseline cortisol. Similar increasing rates of growth over time were observed for boys with FXS and TD controls on the more complex auditory working memory task, but only boys with FXS displayed an association of increased baseline cortisol and lower performance. This study highlights the benefit of investigations of how dynamic biological and cognitive factors interact and influence cognitive development over time.