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Young adults with intellectual/developmental disabilities (YAIDD) are a vulnerable population during HCT due to their complex care coordination and adaptive needs, yet factors associated with transition preparedness are not well defined. We aimed to determine factors associated with health care transition (HCT) preparation satisfaction for YAIDD establishing care with an adult medical home.408 YAIDD or their families completed the HCT Feedback Survey 2.0 upon establishing adult care. Logistic regression models were used to determine associations between a composite of six HCT Feedback Survey questions that most correlated with the 2019 National Survey of Children's Health transition questions.YADD who had HCT preparation visits with a designated HCT clinic were 9 times more likely to have met all six composite HCT criteria after controlling for the number of technologies required and race/ethnicity (adj OR 9.04, 95% CI: 4.35, 18.76) compared to those referred from the community. Compared to patients who were referred from the community, the odds of feeling very prepared versus somewhat or not prepared were 3.7 times higher (adj OR 3.73, 95% CI: 1.90, 7.32) among patients referred from a designated HCT program.YAIDD who participated in a structured HCT program prior to transfer to adult care experienced higher transition preparation satisfaction.A structured HCT clinic model to prepare adolescents with DD for transition to adult care may improve HCT preparation satisfaction for this population.

Individuals with Spina Bifida (SB) have unique lifelong medical and social needs. Thus, when considering how to promote health and offer preventive care, providers must adapt general healthcare screening and counseling recommendations to their patients’ physical and cognitive impairments along with discerning how to monitor secondary or chronic conditions common to the population. This article provides an update on the health promotion and preventive health care guidelines developed as part of the Spina Bifida Association’s fourth edition of the Guidelines for the Care of People with Spina Bifida. The guidelines highlight accommodations needed to promote general preventive health, common secondary/chronic conditions such as obesity, metabolic syndrome, hypertension, musculoskeletal pain, and considerations for preventing acute care utilization for the SB population throughout the lifespan. Further research is needed to understand the effectiveness of preventive care interventions in promoting positive health outcomes and mitigating potentially preventable acute care utilization.

Globally, the prevalence of myelomeningocele, the most common subtype of spina bifida, is 0.2 to 6.5 per 1,000 live births. In the U.S., adults account for67% of the overall population with spina bifida.With an estimated prevalence of up to 50%, scoliosis is one of the most common and severe orthopaedic conditions in patients with myelomeningocele.The variable effects that scoliosis can have on an individual, the comorbidities associated with progressive scoliosis, and the risks associated with spine surgery call for a strong partnership and care coordination between medical and surgical teams to deliver a patient-centered approach.A coordinated, structured, planned, and incremental team approach can help individuals achieve the overall goals of functionality and independence, as well as successful transition to adulthood.Teams should consider a patient's social determinants of health (e.g., poverty or language barriers) and the effect of scoliosis on quality of life before proceeding with spinal deformity correction.

The COVID-19 pandemic has posed distinctive challenges to adolescents and young adults living with spina bifida, especially those from ethic minority populations. With this public health challenge in mind, developing a customized electronic health record to leverage registry data to promote and quantify COVID-19 vaccination uptake among this population is feasible. We provide a brief description of our activities in customizing an electronic health record to track vaccination uptake among adolescents and young adults with spina bifida (AYASB); and the lessons learned, in hopeful support of those scaling-up vaccination delivery across the globe for AYASB as they transition to adult-centered care. Thus, as providers think globally and act locally, COVID-19 immunization efforts can be implemented while providing culturally appropriate transition policies and services for individuals with neurodevelopmental disabilities.

PURPOSE: To determine characteristics associated with self-management independence and quality of life (QOL) among adolescents and young adults with spina bifida (AYASB) engaged in a spina bifida (SB) health care transition clinic. METHODS: During SB transition visits, Eighty-eight AYASB ages 14-20 completed the Adolescent/Young Adult Self-management and Independence Scale II Self-Report/SB (AMIS II-SR/SB), scores ranging from 1–7 with 7 indicating full independence in activities, and the QUAlity of Life Assessment in Spina bifida for Teens (QUALAS-T), which has two subscales, family/independence (QFI) and bowel/bladder (QBB), with scores ranging from 0-100 with 100 indicating maximal QOL score. Demographic and clinical variables were collected from the electronic medical record. RESULTS: The baseline AMIS II-SR/SB score was 3.3 (SD 1.0). Baseline scores for QUALAS-T QFI and QBB subscales were 73.8 (SD 19.9) and 63.8 (SD 25.8). Older age was associated with a higher baseline AMIS II-SR/SB score (p = 0.017). Over time, AMIS II-SR/SB total significantly improved (p

This clinical report updates a 2006 report from the American Academy of Pediatrics titled “Sexuality of Children and Adolescents With Developmental Disabilities.” The development of a healthy sexuality best occurs through appropriate education, absence of coercion and violence, and developmental acquisition of skills to navigate feelings, desires, relationships, and social pressures. Pediatric health care providers are important resources for anticipatory guidance and education for all children and youth as they understand their changing bodies, feelings, and behaviors. Yet, youth with disabilities and their families report inadequate education and guidance from pediatricians regarding sexual health development. In the decade since the original clinical report was published, there have been many advancements in the understanding and care of children and youth with disabilities, in part because of an increased prevalence and breadth of autism spectrum disorder as well as an increased longevity of individuals with medically complex and severely disabling conditions. During this same time frame, sexual education in US public schools has diminished, and there is emerging evidence that the attitudes and beliefs of all youth (with and without disability) about sex and sexuality are being formed through media rather than formal education or parent and/or health care provider sources. This report aims to provide the pediatric health care provider with resources and tools for clinical practice to address the sexual development of children and youth with disabilities. The report emphasizes strategies to promote competence in achieving a healthy sexuality regardless of physical, cognitive, or socioemotional limitations.

Urinary tract infections (UTIs) are one of the leading health concerns and causes of hospitalization for adults with spina bifida (SB). The risk factors, evaluation, management, and prevention of UTIs in women with SB must take into consideration their unique pelvic anatomy and function as well as the desire for pregnancy or the occurrence UTI during pregnancy. This article reviews published literature regarding this topic and offers recommendations for UTI evaluation, management, and prevention in the context of the unique pelvic floor health needs of women with SB. A systematic review was conducted using the following keywords: spinal dysraphism, spina bifida, myelomeningocele, meningocele, urinary tract infections, females, and adults. Articles were included if they were in English, published during or after 2000, peer reviewed, included women with spina bifida aged 18 or greater, and included outcomes related to urinary tract infection. No articles met inclusion criteria. As no articles were found based on the initial search criteria, articles pertaining to neurogenic bladder UTI risks, evaluation, and management were discussed to develop consensus recommendations for the unique care of UTIs in women with SB.

With an estimated 85% of individuals with spina bifida (SB) surviving into adulthood, SB-specific transition to adult healthcare guidelines are warranted to address the diverse and complex medical, adaptive, and social needs particular to this condition. This commentary discusses the SB Transition Healthcare Guidelines from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida, reviews current transition care models in which such guidelines can be implemented, and explores further research topics in SB transition care.

Down syndrome disintegrative disorder (DSDD), a developmental regression in children with Down syndrome (DS), is a clinical entity that is characterized by a loss of previously acquired adaptive, cognitive, and social functioning in persons with DS usually in adolescence to early adulthood. Initially reported in 1946 as "catatonic psychosis," there has been an increasing interest among the DS community, primary care, and subspecialty providers in this clinical area over the past decade. This condition has a subacute onset and can include symptoms of mood lability, decreased participation in activities of daily living, new-onset insomnia, social withdrawal, autistic-like regression, mutism, and catatonia. The acute phase is followed by a chronic phase in which baseline functioning may not return. No strict criteria or definitive testing is currently available to diagnose DSDD, although a comprehensive psychosocial and medical evaluation is warranted for individuals presenting with such symptoms. The etiology of DSDD is unknown, but in several hypotheses for regression in this population, psychological stress, primary psychiatric disease, and autoimmunity are proposed as potential causes of DSDD. Both psychiatric therapy and immunotherapies have been described as DSDD treatments, with both revealing potential benefit in limited cohorts. In this article, we review the current data regarding clinical phenotypes, differential diagnosis, neurodiagnostic workup, and potential therapeutic options for this unique, most disturbing, and infrequently reported disorder.

PURPOSE To describe the age of independence in intermittent self-catheterization (ISC) in a diverse patient population and identify factors associated with ISC in individuals with spina bifida. METHODS Two hundred patients with myelomeningocele or lipomyelomeningocele, who were ⩾ 3 years of age and utilized catheterization for bladder management were included. Data regarding diagnosis, functional level of lesion, race, ethnicity, presence of shunt, method of catheterization, self-management skills, fine motor skills, and cognitive abilities were collected. RESULTS Fifty-five percent of individuals were able to perform ISC with a mean age of 9.45 years (SD = 2.97) and 22.7% used a surgically created channel. Higher level of lesion and female gender were associated with a lower rate of ISC. Intellectual disability was present in 15% of the individuals able to perform ISC and in 40% of those not able to perform ISC (p= 0.0005). Existent self-efficacy regarding activities of daily living (i.e. dressing, bathing, skin care) were associated with ISC (p< 0.0001). CONCLUSIONS The average age of ISC emerged as a target for culturally-appropriate educational interventions to stimulate greater early independence. Future research on factors that may foster an 'independent spirit' early in childhood leading to self-management are warranted.

Providing comprehensive transition care for adolescents and young adults with spina bifida (AYASB) requires a structured approach to addressing chronic condition management, self-management, care coordination, and health care navigation that is adaptable to the various levels of cognitive ability, physical function, and family/community environments within the population. This commentary (1) highlights AYASB transition program needs identified in the literature and within a local community, (2) analyzes advantages and limitations of published AYASB transition care models in addressing these needs, (3) demonstrates how a spina bifida (SB) transition clinic used the Chronic Care Model (CCM) to develop a comprehensive AYASB transition program, and (4) examines the potential feasibility in adapting this model to other SB clinics. A SB-specific transition clinic based on the CCM model facilitates the complex chronic care management and transition planning for AYASB. Further study is needed to evaluate health care outcomes using the CCM for SB transition.