Magnetic resonance imaging of the anterior compartment of the lower leg is a biomarker for weakness, disability, and impaired gait in childhood Charcot-Marie-Tooth disease.

INTRODUCTION: Biomarkers of disease severity in Charcot-Marie-Tooth disease (CMT) are required to evaluate early responses to treatment. In this study we used magnetic resonance imaging (MRI) to evaluate the relationship between muscle volume and intramuscular fat accumulation with weakness, disability, and impaired gait in affected children and adolescents. METHODS: Fifty-five participants underwent MRI of the anterior compartment of the lower leg. Muscle and fat volumes were calculated. Strength was measured using hand-held dynamometry, disability using the CMT Pediatric Scale, and 3-dimensional gait analysis using an 8-camera Vicon Nexus motion capture system. RESULTS: Lower muscle volume was significantly associated with reduced dorsiflexion strength, increased disability, impaired gait profile score, and foot drop. Intramuscular fat accumulation was associated with reduced dorsiflexion strength and impaired gait profile score. DISCUSSION: The MRI protocol described was feasible, reliable, and sensitive to the magnitude of weakness, disability, and walking difficulties in children with CMT. Muscle Nerve 59:213-217, 2019.

A Case Review: Integrating Lewin's Theory with Lean's System Approach for Change

The complexity of healthcare calls for interprofessional collaboration to improve and sustain the best outcomes for safe and high quality patient care. Historically, rehabilitation nursing has been an area that relies heavily on interprofessional relationships. Professionals from various disciplines often subscribe to different change management theories for continuous quality improvement. Through a case review, authors describe how a large, Midwestern, rehabilitation hospital used the crosswalk methodology to facilitate interprofessional collaboration and develop an intervention model for implementing and sustaining bedside shift reporting. The authors provide project background and offer a brief overview of the two common frameworks used in this project, Lewin's Three-Step Model for Change and the Lean Systems Approach. The description of the bedside shift report project methods demonstrates that multiple disciplines are able to utilize a common framework for leading and sustaining change to support outcomes of high quality and safe care, and capitalize on the opportunities of multiple views and discipline-specific approaches. The conclusion discusses outcomes, future initiatives, and implications for nursing practice.

Life concerns and perceptions of care in adolescents with mental health care needs: a qualitative study in a school-based health clinic.

Health clinics within schools are designed to provide comprehensive care to the student population. Little research has been conducted to identify how effectively school-based health clinics are meeting the mental health needs of adolescents. This qualitative study explored the perceptions of mental health needs of 18 inner-city teens between 12 to 18 years of age with diagnosed behavioral or mental health issues. A single, face-to-face, semi-structured interview was used to examine students' concerns and attitudes toward their health care needs and services. Results revealed concern for their personal and family relationships, education and vocational goals, health maintenance, and financial independence. Most pressing resource needs related to their ability to receive health maintenance and coping skills and to adapt to complex life challenges they faced in their urban neighborhood. Participants remarked that if the clinic did not identify their needs for mental health care, they would most likely have not received services. When mental health care needs were identified, individual counseling was used to support dialogues for optimal mental and physical health.

Replicating and redesigning ankle-foot orthoses with 3D printing for children with Charcot-Marie-Tooth disease.

BACKGROUND: Children with the most common inherited neuropathy, Charcot-Marie-Tooth disease (CMT), are often prescribed ankle-foot orthoses (AFOs) to improve walking ability and prevent falls by reducing foot drop, postural instability, and other gait impairments. These externally worn assistive devices are traditionally custom-made using thermoplastic vacuum forming. This labour-intensive manufacturing process often results in AFOs which are cumbersome due to limited design options, and are associated with low acceptability, discomfort, and suboptimal impact on gait. The aim of this study was to determine how 3D printing can be used to replicate and redesign AFOs in children with CMT. METHODS: Traditional AFOs, 3D printed replica AFOs (same design as traditional AFOs), 3D printed redesigned AFOs and a shoes only control condition were compared in 12 children with CMT. 3D printed AFOs were manufactured using material extrusion in Nylon-12. 3D gait analysis (temporal-spatial, kinematic, kinetic), in-shoe pedobarography and self-reported satisfaction were used to compare conditions. The primary kinematic and kinetic outcome measures were maximum ankle dorsiflexion in swing and maximum ankle dorsiflexor moment in loading response, to capture foot drop and an absent of heel rocker. RESULTS: The 3D printed replica AFOs were comparable to traditional AFOs for all outcomes. The 3D printed replica AFOs improved foot position at initial contact and during loading response and significantly reduced pressure beneath the whole foot, rearfoot and forefoot compared to the shoes only. The 3D printed redesigned AFOs produced a device that was significantly lighter (mean -35.2, SD 13.3%), and normalised maximum ankle dorsiflexor moment in loading response compared to shoes only and traditional AFOs. SIGNIFICANCE: 3D printing can be used to replicate traditional handmade AFOs and to redesign AFOs to produce a lighter device with improved biomechanics by incorporating novel design features.

Understanding the learning needs of the charge nurse: implications for nursing staff development.

Charge nurses are part of the nurse manager leadership team, yet they are often appointed to their roles with minimal, if any, leadership training, education, or mentoring. Literature shows that nursing leadership affects staff retention, satisfaction, and patient outcomes. In this study, charge nurses were surveyed about their learning needs, the barriers in functioning as a charge nurse, and their view of helpful resources in performing the role of a charge nurse. Findings point to the need for staff educators to facilitate charge nurse competency through both online training and mentoring. In addition, using a transformational leadership style provides staff educators with a strong framework for ensuring nurse leadership competency.

The impact of being overweight on the mobility, temporal-spatial and kinematic aspects of gait in children with cerebral palsy.

AIM: Obesity causes altered gait patterns in typically developing children, but its effect on gait in children with physical disabilities is largely unknown. This study explores associations between body mass index (BMI), functional mobility and gait in children with cerebral palsy (CP). METHOD: An observational cross-sectional study was conducted using three-dimensional gait analysis data from 197 children with CP, Gross Motor Functional Classification System (GMFCS) levels I to III. BMI values were categorised using the Centres for Disease Control and Prevention (2000) BMI percentiles, which are specific to age and gender. Regression analyses, with GMFCS level as a covariate, explored associations between BMI category and temporal-spatial, kinematic and functional mobility variables. Analyses included children categorised as healthy weight and overweight/obese only (n = 174), with underweight children excluded (n = 23). RESULTS: 131 children (mean age 10.5 years, SD 3) were categorised as healthy weight and 43 children (mean age 9.6 years, SD 2.5) as overweight or obese. BMI was not associated with most gait variables. Increased double support time, reduced hip extension and increased ankle dorsiflexion were observed in children that were overweight, but most differences were small and of uncertain clinical significance. A lower proportion of overweight children walked independently over 500 m. CONCLUSION: We found little evidence that BMI has a substantial influence on gait patterns in children with CP but some to suggest it may affect long-distance mobility. Different research strategies are required to improve understanding of relationships between adiposity, strength and function, for effective targeting of interventions to improve mobility.

Can pedobarography predict the occurrence of heel rocker in children with lower limb spasticity?

BACKGROUND: Pedobarography software calculates the centre-of-pressure trajectory in relation to the foot to quantify foot contact patterns. This study presents two new pedobarography measures using the centre-of-pressure trajectory to assess heel rocker. METHODS: To validate these pedobarography measures against 3D gait analysis, emed®-x and Vicon Nexus gait analysis data were captured from 25 children aged 8-16 years (11 male) with unilateral (n = 18) and bilateral (n = 7) cerebral palsy or acquired brain injury. 3D gait analysis identified whether heel rocker was intact (n = 22 feet) or absent (n = 28 feet) based on centre-of-pressure at initial contact and the ankle kinematic curve between 0 and 2% of the gait cycle. Pedobarography measures calculated from the initial centre-of-pressure point were the distance to the heel (point of initial contact) and to the most posterior point of the trajectory (rollback), reported as a percentage of foot length. FINDINGS: The median point of initial contact in limbs with an intact heel rocker was 9% (range 7-12%) and median rollback was 0% (range 0-0.2%), whereas the median point of initial contact in limbs with an absent heel rocker was 58% (range 8-78%) and rollback was 18% (range 0-40%). Point of initial contact is the more accurate method for predicting heel rocker, with a threshold of 14% of foot length identifying the correct heel rocker status in 94% of cases. INTERPRETATION: Point of initial contact can assess heel rocker with high accuracy. Both point of initial contact and rollback provide sensitive information on foot strike pattern, enhancing the utility of pedobarography.

Interprofessional Collaboration and Peer Mentors for Bowel Education in Spinal Cord Injury: A Case Consultation.

BACKGROUND: Evidence shows that having a bowel management program prevents rehospitalization postacute rehabilitation discharge for persons with a spinal cord injury (SCI). Peer mentors have been used to assist patients in managing their care. PURPOSE: The aim of the study was to illustrate how interprofessional collaboration led to utilizing resources of the inpatient rehabilitation facility's peer mentor program and incorporating peer mentors into bowel education for persons with SCI. METHODS: A descriptive case review of a continuous quality improvement (CQI) project, using an eight-item Likert scale evaluation survey with three open-ended questions to assess the utility of incorporating peer mentors into bowel education. FINDINGS AND CONCLUSIONS: Twenty-seven of the 28 participants responded to the survey. Results showed that the education program was useful, should be continued, and include the peer mentors. Responses from open-ended questions included themes related to knowledge, adherence, and taking charge of one's own care. Interprofessional collaboration and involvement of peer mentors as copresenters in SCI bowel education were feasible.

Feasibility of designing, manufacturing and delivering 3D printed ankle-foot orthoses: a systematic review.

BACKGROUND: Ankle-foot orthoses (AFO) are prescribed to manage difficulty walking due to foot drop, bony foot deformities and poor balance. Traditional AFOs are handmade using thermoplastic vacuum forming which provides limited design options, is labour-intensive and associated with long wait times. 3D printing has the potential to transform AFO production and health service delivery. The aim of this systematic review was to determine the feasibility of designing, manufacturing and delivering customised 3D printed AFOs by evaluating the biomechanical outcomes, mechanical properties and fit of 3D printed compared to traditionally manufactured AFOs. METHOD: Electronic databases were searched from January 1985 to June 2018 according to terms related to 3D printing and AFOs. Studies of any design from healthy or pathological populations of any age were eligible for inclusion. Studies must have investigated the effect of customised 3D printed AFOs using any 3D printing technique on outcomes related to walking ability, biomechanical function, mechanical properties, patient comfort, pain and disability. Any other orthotic type or AFOs without a 3D printed calf and foot section were excluded. The quality of evidence was assessed using the GRADE process. RESULTS: Eleven studies met the eligibility criteria evaluating 3D printed AFOs in healthy adults, and adults and children with unilateral foot drop from a variety of conditions. 3D printing was used to replicate traditional AFOs and develop novel designs to optimise the stiffness properties or reduce the weight and improve the ease of use of the AFO. 3D printed custom AFOs were found to be comparable to traditional custom AFOs and prefabricated AFOs in terms of temporal-spatial parameters. The mechanical stiffness and energy dissipation of 3D printed AFOs were found to be similar to prefabricated carbon-fibre AFOs. However, the sample sizes were small (n = 1 to 8) and study quality was generally low. CONCLUSION: The biomechanical effects and mechanical properties of 3D printed AFOs were comparable to traditionally manufactured AFOs. Developing novel AFO designs using 3D printing has many potential benefits including stiffness and weight optimisation to improve biomechanical function and comfort.

Impact of multilevel joint contractures of the hips, knees and ankles on the Gait Profile score in children with cerebral palsy.

BACKGROUND: Children with cerebral palsy are at risk of developing muscle contractures, often contributing to pain, structural deformities and mobility limitations. With the increasing use of gait indices to summarise the findings of three dimensional gait analysis (3DGA), the purpose of this study is to determine whether there is a relationship between multilevel joint contractures and the Gait Profile Score in children with cerebral palsy. METHODS: The Gait Profile Score, calculated from 3D gait analysis, and passive range of motion, strength and spasticity of the hips, knees and ankles in the sagittal plane were measured in 145 children with cerebral palsy (mean age:11 years,4 months; SD:2 years,10 months) (83 males) enrolled in the NSW Paediatric Gait Analysis Service Research Registry from 2011 to 2016. The relationships between these physical measures and the Gait Profile Score were explored using bivariate and multivariate correlations. FINDINGS: Reduced hip extension, knee extension and ankle dorsiflexion (knee extended) range of motion were correlated with a higher (worse) Gait Profile Score (r = -0.348 to -0.466, p < .001). Children with all joints contracted had a significantly higher Gait Profile Score (mean 17.5°, SD 6.2°) than those with no contractures (mean 11.0°, SD 2.3°) or ankle contractures only (mean 12.8°, SD 5.1°) (p < .05). Knee flexion weakness, reduced hip extension and ankle dorsiflexion (knee extended) range of motion predicted 47% of the Gait Profile Score. INTERPRETATION: The Gait Profile Score is a sensitive measure for demonstrating the relationship between multilevel sagittal plane joint contractures and kinematic gait. Clinically, this supports the use of the Gait Profile Score as a simplified measure to understand the contribution of contractures to functional gait limitations. Monitoring knee flexion strength, and hip extension and ankle dorsiflexion (knee extended) range of motion may assist clinicians in prioritising interventions to improve gait in this population.

A model to calculate the progression of the centre of pressure under the foot during gait analysis.

Pedobarography and the centre of pressure (COP) progression is useful to understand foot function. Pedobarography is often unavailable in gait laboratories or completed asynchronously to kinematic and kinetic data collection. This paper presents a model that allows calculation of COP progression synchronously using force plate data. The model is an adjunct to Plug-In-Gait and was applied to 49 typically developing children to create reference COP data. COP progressions were noted to spend 8% of stance behind the ankle joint centre, traverse lateral of the longitudinal axis of the foot through the midfoot for 76% of stance and finishing past the second metatarsal head on the medial side for 16% of stance. It is hoped the model will bridge the information gap for gait laboratories lacking pedobarography during foot assessments and will open up the possibility of retrospective research into COP progression based indices on kinematic data.

Gait patterns of children and adolescents with Charcot-Marie-Tooth disease.

Gait abnormalities reported in childhood Charcot-Marie-Tooth disease (CMT) include foot-drop, reduced ankle power at push-off and increased knee and hip flexion for swing clearance ('steppage-gait'). The purpose of this study was to describe the gait patterns of 60 children aged 6-17 years with CMT (CMTall) and distinguish differences based on functional weakness using the CMT Pediatric Scale (CMTPedS). Data were captured using Vicon Nexus system and compared to 50 healthy norms. Data were subdivided into three groups denoting increasing severity of dorsiflexion and plantarflexion weakness from the CMTPedS: no difficulty heel or toe walking (CMTND), difficulty heel walking (CMTDH), difficulty toe and heel walking (CMTDTH). Compared to healthy norms, CMTall demonstrated significantly worse gait profile score, reduced ankle dorsiflexion during swing (foot-drop), reduced ankle dorsiflexor moment in loading response and reduced external thigh-foot angle. Contrary to previous studies there were no signs of reduced ankle power or compensation through 'steppage gait' in this mild-moderately affected population. Instead, CMTall demonstrated reduced internal hip rotation and reduced hip abductor moment. When data were sub-grouped and compared to healthy norms, three different gait patterns at the ankle emerged: CMTND had a near-normal gait pattern, CMTDH presented with foot-drop, and CMTDTH had increased peak dorsiflexion and reduced ankle power generation. Several distinct and abnormal gait patterns were identified in children with CMT, with increasing gait abnormalities in more functionally severe cases. Classifying gait patterns based on disease severity might be a valuable tool in clinical decision making, assessing disease progression and phenotype-genotype correlation studies.

Spatiotemporal and plantar pressure patterns of 1000 healthy individuals aged 3-101 years.

OBJECTIVE: The purpose of this study was to establish normative reference values for spatiotemporal and plantar pressure parameters, and to investigate the influence of demographic, anthropometric and physical characteristics. METHODS: In 1000 healthy males and females aged 3-101 years, spatiotemporal and plantar pressure data were collected barefoot with the Zeno™ walkway and Emed® platform. Correlograms were developed to visualise the relationships between widely reported spatiotemporal and pressure variables with demographic (age, gender), anthropometric (height, mass, waist circumference) and physical characteristics (ankle strength, ankle range of motion, vibration perception) in children aged 3-9 years, adolescents aged 10-19 years, adults aged 20-59 years and older adults aged over 60 years. RESULTS: A comprehensive catalogue of 31 spatiotemporal and pressure variables were generated from 1000 healthy individuals. The key findings were that gait velocity was stable during adolescence and adulthood, while children and older adults walked at a comparable slower speed. Peak pressures increased during childhood to older adulthood. Children demonstrated highest peak pressures beneath the rearfoot whilst adolescents, adults and older adults demonstrated highest pressures at the forefoot. Main factors influencing spatiotemporal and pressure parameters were: increased age, height, body mass and waist circumference, as well as ankle dorsiflexion and plantarflexion strength. CONCLUSION: This study has established whole of life normative reference values of widely used spatiotemporal and plantar pressure parameters, and revealed changes to be expected across the lifespan.

Safety and efficacy of progressive resistance exercise for Charcot-Marie-Tooth disease in children: a randomised, double-blind, sham-controlled trial.

BACKGROUND: Exercise is potentially therapeutic for neuromuscular disorders, but a risk of harm exists due to overwork weakness. We aimed to assess the safety and efficacy of progressive resistance exercise for foot dorsiflexion weakness in children with Charcot-Marie-Tooth disease. METHODS: We did this randomised, double-blind, sham-controlled trial across the Sydney Children's Hospitals Network (NSW, Australia). Children aged 6-17 years with Charcot-Marie-Tooth disease were eligible if they had foot dorsiflexion weakness (negative Z score based on age-matched and sex-matched normative reference values). We randomly allocated (1:1) children, with random block sizes of 4, 6, and 8 and stratification by age, to receive 6 months (three times per week on non-consecutive days; 72 sessions in total) of progressive resistance training (from 50% to 70% of the most recent one repetition maximum) or sham training (negligible non-progressed intensity), using an adjustable exercise cuff to exercise the dorsiflexors of each foot. The primary efficacy outcome was the between-group difference in dorsiflexion strength assessed by hand-held dynamometry (expressed as a Z score) from baseline to months 6, 12, and 24. The primary safety outcome was the between-group difference in muscle and intramuscular fat volume of the anterior compartment of the lower leg assessed by MRI (expressed as a scaled volume) from baseline to 6 months and 24 months. Participants, parents, outcome evaluators, and investigators other than the treatment team were masked to treatment assignment. Analysis was by intention to treat. The trial is registered with the Australian New Zealand Clinical Trials Registry, number ACTRN12613000552785. FINDINGS: From Sept 2, 2013, to Dec 11, 2014, we randomly assigned 60 children to receive progressive resistance exercise (n=30) or sham training (n=30), and 55 (92%) children completed the trial. ANCOVA-adjusted Z score differences in dorsiflexion strength between groups were 0 (95% CI -0·37 to 0·42; p=0·91) at 6 months, 0·3 (-0·23 to 0·81; p=0·27) at 12 months, and 0·6 (95% CI 0·03 to 1·12; p=0·041) at 24 months. Scaled muscle and fat volume was comparable between groups at 6 months (ANCOVA-adjusted muscle volume difference 0, 95% CI -0·03 to 0·10, p=0·24; and fat volume difference 0, 95% CI -0·01 to 0·05, p=0·25) and 24 months (0, -0·08 to 0·12, p=0·67; and 0, -0·05 to 0·03, p=0·58). No serious adverse events were reported. INTERPRETATION: 6 months of targeted progressive resistance exercise attenuated long-term progression of dorsiflexion weakness without detrimental effect on muscle morphology or other signs of overwork weakness in paediatric patients with Charcot-Marie-Tooth disease. FUNDING: Muscular Dystrophy Association and Australian National Health and Medical Research Council.

How does rectus femoris fibrosis affect gait?

We report on a female patient with stiff-knee gait resulting from rectus femoris fibrosis, following multiple injections into the quadriceps musculature. Treatment planning and outcome were aided by instrumented three-dimensional gait analysis. One year after surgery, improvements were found in walking speed, step length, anterior pelvic tilt, knee flexion in swing, and ankle dorsiflexion in swing. Instrumented three-dimensional gait analysis was a useful tool for characterizing gait impairments and detecting changes after surgical intervention. Intramuscular injections into the rectus femoris muscle should be avoided to prevent stiff-knee gait and associated disability.

A natural history study of adolescents and young adults with sickle cell disease as they transfer to adult care: a need for case management services.

Life expectancy for adolescents with SCD now extends well into adulthood. As a result, adolescents transfer to adult care. Little empirical evidence exists to show how transfer occurs and how well the current practices now work. The aim of this study was to obtain a database on the experience of adolescents/young adults with SCD that transfer to adult care. We assessed their treatment compliance, independence, and whether they receive uninterrupted care. Data were obtained through patient and provider interviews and patient record reviews. Results indicate patients leave pediatric care without adequate transfer preparation and readiness to transfer is not the major consideration in the decision to transfer, follow-up often ceases once the patients leave pediatric care, and patients who maintain follow-up appointments are more efficient in managing their illness (self-efficacy). The model for a structured transitioning process is provided with recommendations for nurse case managers to maintain follow-up.