Annette Majnemer

A New Look at Outcomes of Infants With Congenital Heart Disease

This article provides an overview of a longitudinal study on a cohort of 131 newborns and young infants with congenital heart defects who required open heart surgery. The rationale for the study design is provided as well as a summary of the procedures used to evaluate these children prior to surgery, at discharge after surgery, 12-18 months later, and at 5 years of age. Results demonstrate that a substantial proportion of these infants had neurologic and developmental abnormalities prior to surgical repair. Developmental delays were common in children with both cyanotic and acyanotic heart defects, and these deficits persisted to school entry. A number of medical, surgical, demographic and environmental factors were significantly associated with developmental outcomes suggesting a multifactorial etiology to brain injury. Limited educational and rehabilitation resources for this cohort at early school age suggests that service needs may need to be more carefully planned for this high-risk population. Lessons learnt from this prospective study are highlighted as well as future directions for research and clinical practice.

Level of motivation in mastering challenging tasks in children with cerebral palsy

Aimu2002 The aim of this study was to describe and identify factors associated with motivation in children with cerebral palsy (CP).

Behavioural problems in school age children with cerebral palsy

BACKGROUNDnAlthough behavioural problems are frequent in children with Cerebral Palsy (CP), the exact nature of these difficulties and their relationship with intrinsic or extrinsic factors are just beginning to be explored.nnnAIMnTo describe and characterize behavioural problems in children with CP and to determine the nature of any relationships with child and family characteristics.nnnMETHODSnIn this cross-sectional study, children with CP between 6 and 12 years of age were recruited. Children were assessed using the Leiter Intelligence Test, the Gross Motor Function Measure, the Strengths and Difficulties Questionnaire (SDQ), the Vineland Adaptive Behavior Scales and questionnaires on demographic factors. Parents level of stress was measured with the Parenting Stress Index.nnnRESULTSnSeventy-six parents completed the SDQ. Using the Total Difficulties Scores, 39.4% of the sample scored in the borderline to clinically abnormal range. Peer problems were the most common (55.3%). High parental stress was consistently associated with behavioural difficulties across all domains of the SDQ. Not surprisingly, better socialization skills and a lower parental stress were correlated with more positive behaviours.nnnCONCLUSIONnBehavioural difficulties are common in children with CP and appear not to be associated with socio-demographic variables and physical and cognitive characteristics. These difficulties are an important correlate of parental distress. This study emphasizes the need to recognize and address behavioural difficulties that may arise so as to optimize the health and well-being of children with CP and their families.

Developmental and functional outcomes in children with global developmental delay or developmental language impairment

Preschool children diagnosed with either global developmental delay (GDD) or developmental language impairment (DLI) were reassessed during their early school years with standardized developmental (Battelle Developmental Inventory [BDI]) and functional (Vineland Adaptive Behavior Scale [VABS]) outcome measures. Of an original cohort of 99 children with GDD and 70 children with DLI assessed and diagnosed at a mean age of 3 years 5 months (SD 1.1) and 3 years 7 months (SD 0.7) respectively, 48 children (34 [71%] males) with GDD and 43 children (36 [84%] males) with DLI were reassessed at a mean age of 7 years 4 months (SD 0.9) and 7 years 5 months (SD 0.7) respectively. The overall total mean BDI score for children with GDD was 66.4 (SD 4.3) versus 71.9 (SD 8.2) for children with DLI (p=0.002). On each subdomain of the BDI, except communication, mean scores for the GDD group were significantly lower than for the DLI group (p<0.05). Similarly, the VABS total score for the GDD group was significantly lower than for the DLI group (p<0.001). For each subdomain of the VABS, the GDD group scored significantly lower than the DLI group (p<0.001). The proportion of children falling below meaningful cut‐offs on the outcome measures selected was significantly higher for those initially diagnosed with GDD. Preschool diagnosis of either GDD or DLI has later prognostic validity with regard to persisting developmental and functional deficits.

Determinants of developmental outcomes in a very preterm Canadian cohort

Objectives Identify determinants of neurodevelopmental outcome in preterm children. Methods Prospective national cohort study of children born between 2009 and 2011 at <29u2005weeks gestational age, admitted to one of 28 Canadian neonatal intensive care units and assessed at a Canadian Neonatal Follow-up Network site at 21u2005months corrected age for cerebral palsy (CP), visual, hearing and developmental status using the Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III). Stepwise regression analyses evaluated the effect of (1) prenatal and neonatal characteristics, (2) admission severity of illness, (3) major neonatal morbidities, (4) neonatal neuroimaging abnormalities, and (5) site on neurodevelopmental impairment (NDI) (Bayley-III score < 85, any CP, visual or hearing impairment), significant neurodevelopmental impairment (sNDI) (Bayley-III < 70, severe CP, blind or hearing aided and sNDI or death. Results Of the 3700 admissions without severe congenital anomalies, 84% survived to discharge and of the 2340 admissions, 46% (IQR site variation 38%–51%) had a NDI, 17% (11%–23%) had a sNDI, 6.4% (3.1%–8.6%) had CP, 2.6% (2.5%–13.3%) had hearing aids or cochlear implants and 1.6% (0%–3.1%) had a bilateral visual impairment. Bayley-III composite scores of <70 for cognitive, language and motor domains were 3.3%, 10.9% and 6.7%, respectively. Gestational age, sex, outborn, illness severity, bronchopulmonary dysplasia, necrotising enterocolitis, late-onset sepsis, retinopathy of prematurity, abnormal neuroimaging and site were significantly associated with NDI or sNDI. Site variation ORs for NDI, sNDI and sNDI/death ranged from 0.3–4.3, 0.04–3.5 and 0.12–1.96, respectively. Conclusion Most preterm survivors are free of sNDI. The risk factors, including site, associated with neurodevelopmental status suggest opportunities for improving outcomes.

Pediatric Cerebral Palsy in Africa: Where Are We?

Cerebral palsy is the most common cause of physical disability in children worldwide. However, little is reported on this condition in the African context. Doctors from 22 countries in Africa, and representatives from a further 5 countries outside Africa, met to discuss the challenges in the evaluation and management of children with cerebral palsy in Africa and to propose service needs and further research. Basic care is limited by the poor availability of diagnostic facilities or medical personnel with experience and expertise in managing cerebral palsy, exacerbated by lack of available interventions such as medications, surgical procedures, or even regular therapy input. Relevant guidelines are lacking. In order to guide services for children with existing disabilities, to effectively target the main etiologies and to develop preventive strategies for the continent, research priorities must include multicenter collaborative studies looking at the prevalence, risk factors, and treatment of cerebral palsy.

Developmental and Functional Abilities in Children With Cerebral Palsy as Related to Pattern and Level of Motor Function

Abilities among school-aged children with cerebral palsy with different patterns and levels of motor function were evaluated. Children within spasticity patterns (33 with quadriplegia, 25 with hemiplegia, 19 with diplegia) and Gross Motor Function Classification System levels were compared (level I, walking = 47%; level II-III, restricted ambulation = 18%; level IV-V, wheelchair needs = 34%,). Outcomes included measures of intelligence, behavior, motor, and functional limitations (communication, daily living, socialization). Motor performance and prosocial behaviors were lower for children with quadriplegia (F = 16.13, 12.71; P < .0001), with no differences for behavioral difficulties between spasticity groups. Prosocial behaviors were different between level IV-V and other groups (F = 16.25, P < .0001). Functional limitations were more likely for children with quadriplegia (P < .0001), but not diplegia or hemiplegia, and for children in level IV-V, but similar for level I and level II-III. Children with quadriplegia, or level IV-V, are more likely to exhibit limitations, whereas children with better motor function (I-III), hemiplegia, or diplegia, exhibit diverse capabilities. A holistic assessment approach is essential to ensure that limitations are addressed comprehensively.

Associations between a functional independence measure (WeeFIM) and the pediatric quality of life inventory (PedsQL4.0) in young children with physical disabilities

Objectives: To determine the association between functional status, measured by the WeeFIM and health-related quality of life (HRQL), measured by the PedsQL4.0 for children with physical disabilities. To explore child, parent and service-related factors associated with each of these measures. Patients: Parents of 115 children (2–5xa0years) with physical disabilities who were referred to occupational (OT) or physical therapy (PT). Mean age of the children was 3xa0years 7xa0months (±10xa0months), 79 were boys and 67 were diagnosed with global developmental delay. Results: Children had more difficulties with self-care tasks and cognitive abilities, as compared to mobility activities on the WeeFIM. The correlation between total WeeFIM and total PedsQL4.0 was r = 0.39. WeeFIM mobility and self-care quotients were each fairly correlated with PedsQL-Physical Health Summary Score (rs = 0.29 and rs = 0.28 respectively). There was no significant association between WeeFIM cognition quotient and each of PedsQL scores (rs = 0.03–0.05). The receipt of PT services was highly associated (p<0.001) with lower scores on the PedsQL-Physical Health Summary score, PedsQL-Total score, and WeeFIM mobility quotient. Conclusion: The WeeFIM and the PedsQL4.0 appear to assess related but different constructs, supporting the need to incorporate complementary measures when measuring general health of children with disabilities.

Respect for autonomy in the healthcare context: observations from a qualitative study of young adults with cerebral palsy

BACKGROUNDnRespect for patient autonomy is a cornerstone of contemporary medical ethics and clinical practice. In its different shapes and forms (e.g. being informed, being engaged in discussions and decisions about medical care and being supported in developing healthcare preferences and choices), patient autonomy has been fostered by both paediatric and adult professional societies. The transition from paediatric to adult care creates a complex situation where autonomy for medical decisions shifts to the developing adolescent. More specific challenges to respect for autonomy may be experienced by young adults with cerebral palsy in the transition period where, for example, language and motor impairments may affect communication skills and this may be conflated with cognitive disability.nnnAIMnTo characterize perspectives towards autonomy in the healthcare context for young adults with cerebral palsy.nnnMETHODnWe carried out semi-structured interviews with 14 young adults (aged 18-25) with cerebral palsy. The audiotaped interviews were transcribed verbatim and analysed using a conventional thematic qualitative content analysis.nnnRESULTSnParticipants displayed a range of attitudes towards autonomy, suggesting that the value of autonomy is considered in light of competing values and of context. Testimonials from participants demonstrated that both contextual (e.g. ill-adapted health care, lack of specialized public transport) and relational (e.g. attitudes towards parental involvement in decision making) factors negatively or positively impact autonomy.nnnCONCLUSIONnWe observed that there were four key elements interwoven in participants characterization of autonomy: the coupling of decisional and physical autonomy, the influences of family and society on autonomy, the influence of healthcare professionals on autonomy and the need for preparation for autonomy.

International Classification of Functioning, Disability and Health (ICF) as a framework for change: revolutionizing rehabilitation.

The International Classification of Functioning, Disability and Health (ICF) utilizes domains of body functions and structures, activities and participation, as well as environmental and personal factors to fully encapsulate the concepts of health and disability. The International Classification of Functioning, Disability and Health provides a rich and holistic understanding of functioning that is particularly valuable in the setting of childhood disability and rehabilitation. With applicability that enhances a nuanced understanding of each child within their family, school, and community, the International Classification of Functioning, Disability and Health also ensures facile and meaningful communication between professionals. Use of the International Classification of Functioning, Disability and Health promotes improved treatment plans for individual children and for larger programmatic decisions. This article demonstrates how the International Classification of Functioning, Disability and Health has reinvented the language and understanding of childhood disability and rehabilitation.