Participation and quality of life in children with Duchenne muscular dystrophy using the International Classification of Functioning, Disability, and Health

<p>Abstract</p> <p>Background</p> <p>Duchenne 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.</p> <p>Methods</p> <p>Fifty 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 Inventory^TM 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.</p> <p>Results</p> <p>Participation 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.</p> <p>Conclusions</p> <p>The 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.</p

Parental perception of facilitators and barriers to health among young children with down syndrome: a qualitative study

BackgroundDespite high rates of obesity and weight-related conditions observed in children with Down syndrome, little is known about how to prevent these conditions.PurposeThe purpose of this study was to identify parent-perceived facilitators and barriers to health for toddlers (12–36 months old) with Down syndrome.Materials and methodsWe conducted in-depth, semi-structured interviews with the mothers of 25 toddlers with Down syndrome. All interviews were conducted using Zoom Video Technology, audio recorded and transcribed before being coded in NVivo software using a structured protocol. Thematic analysis was used to identify themes in perceived facilitators and barriers to health at the level of the child, family, and community. Data were triangulated using reflective journaling, video review of child meals, and member-checking techniques.ResultsWe identified unique themes for facilitators (on the move and sound sleep) and barriers (co-occurring conditions and eating behaviors) at the level of the child. At the level of the family and community, overarching themes that were viewed as either a facilitator or barrier, depending on the context, were identified (role models matter, time is critical, the importance of place, and social support).ConclusionThese themes can help clinicians and researchers tailor their health promotion interventions to meet the unique needs of children with Down syndrome by using strength-based approaches and providing families with the tools to overcome barriers

In-Home Training for Fathers of Children with Autism: A Follow up Study and Evaluation of Four Individual Training Components

Literature regarding fathers of children with autism remains sparse, and because mothers are the more common intervening parent, few training methods have focused on fathers. Thus, we sought to evaluate effects of in-home training directed at fathers and their ability to train mothers in the same manner in which they were trained. Fathers were taught four skills commonly associated with in-home training interventions for parents of children with autism: following the child’s lead, imitation with animation, commenting on the child, and expectant waiting. Father skills were evaluated twice a week for 12 weeks during videotaped in-home father–child play sessions. Analyses included visual inspection of graphed data and statistical analyses of father skill acquisition, mother skill acquisition, and child behaviors with both parents. A multivariate repeated measures analysis of 18 dyads revealed significant increases in frequencies of fathers’ imitation with animation, expectant waiting, and commenting on the child. Child initiating rates increased significantly as did frequencies of child non-speech vocalizations. Analysis of mothers revealed significant increases in frequencies of imitation with animation, expectant waiting, and following the child’s lead. Child behaviors had similar results for father and mother sessions. Findings are consistent with those from our first study indicating that fathers can effectively implement skills that promote father–child social interactions and that children respond positively to this approach

A user-centered approach: Understanding client and caregiver needs and preferences in the development of mhealth apps for self-management

Background: Many 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. Objective: Our 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. Methods: Our 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. Results: A 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. Conclusions: Our 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

El Pueblo : diario de la República: Año V Número 1036 - 1936 marzo 27

Pediatric occupational therapy practitioners frequently provide interventions for children with differences in sensory processing and integration. Confusion exists regarding how best to intervene with these children and about how to describe and document methods. Some practitioners hold the misconception that Ayres Sensory Integration intervention is the only approach that can and should be used with this population. The issue is that occupational therapy practitioners must treat the whole client in varied environments; to do so effectively, multiple approaches to intervention often are required. This article presents a framework for conceptualizing interventions for children with differences in sensory processing and integration that incorporates multiple evidence-based approaches. To best meet the needs of the children and families seeking occupational therapy services, interventions must be focused on participation and should be multifaceted

Patient-report and caregiver-report measures of rehabilitation service use following acquired brain injury: a systematic review

Objective To review patient-report/caregiver-report measures of rehabilitation service use following acquired brain injury (ABI).Data sources Medline, APA PsycINFO, Embase and CINAHL were searched on November 2021 and November 2022. Authors were contacted if measures were not included in manuscripts/appendices.Study selection Included articles were empirical research or a research protocol, available in English and described measures of patient report/caregiver report of rehabilitation service use post-ABI via quantitative or qualitative methods. Two reviewers independently screened 5290 records using DistillerSR. Discrepancies were resolved by team adjudication.Data extraction Data extraction was piloted with high levels of agreement (k=.94). Data were extracted by a single member with team meetings to seek guidance as needed. Data included administration characteristics (reporter, mode of administration, recall period), psychometric evidence and dimensions assessed (types of services, setting, frequency, duration, intensity, qualitative aspects).Data synthesis One hundred and fifty-two measures were identified from 85 quantitative, 56 qualitative and 3 psychometric studies. Psychometric properties were reported for four measures, all of which focused on satisfaction. Most measures inquired about the type of rehabilitation services used, with more than half assessing functional (eg, physical therapy) and behavioural health rehabilitation services, but fewer than half assessing community and academic reintegration (eg, special education, vocational rehabilitation) or cognitive (eg, neuropsychology) services. Fewer than half assessed qualitative aspects (eg, satisfaction). Recall periods ranged from 1 month to ‘since the ABI event’ or focused on current use. Of measures that could be accessed (n=71), many included a limited checklist of types of services used. Very few measures assessed setting, frequency, intensity or duration.Conclusions Despite widespread interest, the vast majority of measures have not been validated and are limited in scope. Use of gold-standard psychometric methods to develop and validate a comprehensive patient-report/caregiver-report measure of rehabilitation service use would have wide-ranging implications for improving rehabilitation research in ABI

Magnetic Resonance Assessment of Hypertrophic and Pseudo-Hypertrophic Changes in Lower Leg Muscles of Boys with Duchenne Muscular Dystrophy and Their Relationship to Functional Measurements.

The primary objectives of this study were to evaluate contractile and non-contractile content of lower leg muscles of boys with Duchenne muscular dystrophy (DMD) and determine the relationships between non-contractile content and functional abilities.Lower leg muscles of thirty-two boys with DMD and sixteen age matched unaffected controls were imaged. Non-contractile content, contractile cross sectional area and non-contractile cross sectional area of lower leg muscles (tibialis anterior, extensor digitorum longus, peroneal, medial gastrocnemius and soleus) were assessed by magnetic resonance imaging (MRI). Muscle strength, timed functional tests and the Brooke lower extremity score were also assessed.Non-contractile content of lower leg muscles (peroneal, medial gastrocnemius, and soleus) was significantly greater than control group (p<0.05). Non-contractile content of lower leg muscles correlated with Brooke score (rs = 0.64-0.84) and 30 feet walk (rs = 0.66-0.80). Dorsiflexor (DF) and plantarflexor (PF) specific torque was significantly different between the groups.Overall, non-contractile content of the lower leg muscles was greater in DMD than controls. Furthermore, there was an age dependent increase in contractile content in the medial gastrocnemius of boys with DMD. The findings of this study suggest that T1 weighted MR images can be used to monitor disease progression and provide a quantitative estimate of contractile and non-contractile content of tissue in children with DMD