The purpose of this study was to assess the contractile and non-contractile content in thigh muscles of patients with Duchenne muscular dystrophy (DMD) and determine the relationship with functional abilities. Magnetic resonance images of the thigh were acquired in 28 boys with DMD and 10 unaffected boys. Muscle strength, timed functional tests, and the Brookes Lower Extremity scale were also assessed. Non-contractile content in the DMD group was significantly greater than in the control group for six muscles, including rectus femoris, biceps femoris-long head and adductor magnus. Non-contractile content in the total thigh musculature assessed by MRI correlated with the Brookes scale (rs=0.75) and supine-up test (rs=0.68), as well as other functional measures. An age-related specific torque increase was observed in the control group (rs=0.96), but not the DMD (rs=0.06). These findings demonstrate that MRI measures of contractile and non-contractile content can provide important information about disease progression in DMD.

BackgroundDuchenne muscular dystrophy (DMD) is characterized by muscle damage and progressive loss of muscle function in male children. DMD is one of the most devastating genetically linked neuromuscular diseases for which there is currently no cure. Most clinical studies for DMD utilize a standard protocol for measurement exploring pathophysiology, muscle strength and timed tasks. However, we propose that examining broader components of health as emphasized by the International Classification of Functioning, Disability and Health-Children and Youth Version (ICF-CY) may be of great value to children and their families, and important outcomes for future clinical trials.MethodsFifty boys with DMD and 25 unaffected age-matched boys completed two self-report measures: the Children’s Assessment of Participation and Enjoyment and the Pediatric Quality of Life InventoryTM 4.0. We investigated differences between the two groups with regard to participation in life activities and perceived quality of life (QoL). Additionally, we compared participation in activities and QoL in both cohorts of younger and older boys.ResultsParticipation in physical activities was significantly lower in boys with DMD than unaffected boys. Perceived QoL was markedly diminished in children with DMD relative to unaffected controls, except in the emotional domain. The amount of time boys engage in an activity, as well as participation in social activities, declined for our older boys with DMD but no changes were observed for our older unaffected boys. For both groups, QoL remained constant over time.ConclusionsThe ICF-CY provides a conceptual framework and specific terminology that facilitates investigation of the consequences of impairment in children and youth. Our study is one of the first to explore participation in a cohort of boys with DMD. It was not surprising that activities of choice for boys with DMD were less physical in nature than unaffected boys their age, but the consequences of less social engagement as the boys with DMD age is of great concern. Results from our study underscore the need to further evaluate activities that children elect to participate in, with special emphasis on facilitators and barriers to participation and how participation changes throughout the course of a disease.

This pilot study explored activity patterns in children with and without ASD and examined the role of sensory responsiveness in determining children’s level of competence in activity performance. Twenty-six children with high functioning ASD and twenty-six typically-developing children 6–12 years old were assessed using the Sensory Profile and the Child Behavior Checklist. Results reflect differences in the types of activities and jobs/chores engaged in by children with ASD compared to children without ASD. Significant differences were seen in overall level of competence in activities, social, and school performance. Children demonstrating more frequent Sensory Sensitivity and Sensory Avoiding had significantly lower competence scores than children with fewer behaviors in these domains, suggesting that sensory responsiveness may impact the ability to participate successfully.

Chronic illnesses account for approximately 75% of all healthcare costs in the United States today, resulting in functional limitations and loss of independence, as well as increased medical expenditures. The elderly population is at a higher risk for developing chronic conditions, increasing their risk for disabilities. Given the rapid growth of the aging population, and the chronic illnesses, disabilities, and loss of functional independence endemic to elders, novel methods of rehabilitation and care management are urgently needed. Telehealth models that combine care coordination with communications technology offer a means for managing chronic illnesses, thereby decreasing healthcare costs. We examined the effects of a Veterans Administration (VA) telerehabilitation program (Low Activities of Daily Living [ADL] Monitoring Program-LAMP) on healthcare costs. LAMP is based on a rehabilitative model of care. LAMP patients received adaptive equipment and environmental modifications, which focused on self-care and safety within the home. LAMP Care Coordinators remotely monitored their patient's vital signs and provided education and self-management strategies for decreasing the effects of chronic illnesses and functional decline. The matched comparison group (MCG) received standard VA care. Healthcare costs 12 months preenrollment and 12 months post-enrollment were examined through a difference-in-differences multivariable model. Using actual costs totaled for these analyses, no significant differences were detected in post-enrollment costs between LAMP and the MCG. For LAMP patients, the provision of adaptive equipment and environmental modifications, plus intensive in-home monitoring of patients, led to increases in clinic visits post-intervention with decreases in hospital and nursing home stays.

BackgroundMany adolescents and young adults with chronic illness or disability often fail to develop the self-management skills necessary to independently handle medical and self-management routines. In light of these needs, we are developing iMHere 2.0 (Interactive Mobile Health and Rehabilitation), a mobile health (mHealth) system to support a self-management program.ObjectiveOur objective was to gather data from persons with brain and spinal cord anomalies (BSA) and their caregivers to better understand how mHealth would be most helpful in supporting them to proactively manage daily self-care routines and to access medical care as needed. The specific purpose was not only to gather feedback and to gain increased insight into the design of the new version of iMHere, but also to gather perspectives of new groups, namely adolescents as young as 12 years and their parents and/or caregivers.MethodsOur project employed focus group sessions and surveys to collect data from participants with BSA, as well as their caregivers. A total of six focus group sessions were conducted on four separate occasions until the data gathered reached saturation. The objectives of our focus group sessions were to better understand ways to develop mHealth systems to support self-management, to promote independence, to motivate long-term system use, and to prevent medical problems that lead to hospitalizations and emergency room visits for youth and young adults with BSA.ResultsA total of 16 youth and young adults with BSA and 11 caregivers participated in the sessions. Within and among our groups, the following five overarching themes emerged from the data: (1) make it easy, (2) engage, (3) educate and prepare, (4) motivate and support, and (5) personalize. Participants shared their perspectives and detailed information about mHealth apps that would be important for independence in self-care and self-management.ConclusionsOur findings suggest that most individuals keep their mobile phones with them at all times and typically use a mobile phone for social media, music, photos, and texting. Our qualitative analysis indicates that youth and young adults with BSA, as well as their caregivers, acknowledge the importance of being actively engaged in developing and using mHealth apps that monitor and manage their health care needs. Information gleaned from these focus group sessions and surveys have provided data to refine the iMHere 2.0 mHealth prototype platform that we have developed.