Lisa Samson-Fang

Feeding Dysfunction is Associated with Poor Growth and Health Status in Children with Cerebral Palsy

OBJECTIVE To describe parent-reported feeding dysfunction and its association with health and nutritional status in children with cerebral palsy. DESIGN Anthropometry was measured and z scores calculated. The Child Health Questionnaire was used to assess health status, and a categorical scale (none to severe) was used to classify subjects according to severity of feeding dysfunction. SUBJECTS 230 children (9.7+/-4.6 years; 59% boys) with moderate to severe cerebral palsy were recruited from 6 centers in the United States and Canada. STATISTICAL ANALYSES Descriptive statistics, the Kruskal-Wallis and Pearson chi2 tests. RESULTS Severity of feeding dysfunction was strongly associated with indicators of poor health and nutritional status. The mean weight z scores were -1.7, -2.5, -3.3, and -1.8 among children with none, mild, moderate, or severe (largely tube-fed) feeding dysfunction, respectively (P= .003). Similar results were observed for height z score (P=.008), triceps z score (P=.03), and poor Global Health score (part of the Child Health Questionnaire) (P<.001). Subjects who were tube fed were taller (P=.014) and had greater body fat stores (triceps z score, P=.001) than orally fed subjects with similar motor impairment. For subjects exclusively fed by mouth, a dose-response relationship was observed between feeding dysfunction severity and poor nutritional status. Subjects with only mild feeding dysfunction had reduced triceps z score (-0.9) compared with those with no feeding problems (-0.3). CONCLUSION For children with moderate to severe cerebral palsy, feeding dysfunction is a common problem associated with poor health and nutritional status. Even children with only mild feeding dysfunction, requiring chopped or mashed foods, may be at risk for poor nutritional status. Parental report of feeding dysfunction with a structured questionnaire may be useful in screening children for nutritional risk.

Health status of children with moderate to severe cerebral palsy

The aim of the study was to evaluate the health of children with cerebral palsy (CP) using a global assessment of quality of life, condition-specific measures, and assessments of health care use. A multicenter population-based cross-sectional survey of 235 children, aged 2 to 18 years, with moderate to severe impairment, was carried out using Gross Motor Function Classification System (GMFCS) levels III (n = 56), IV (n = 55), and V (n = 122). This study group scored significantly below the mean on the Child Health Questionnaire (CHQ) for Pain, General Health, Physical Functioning, and Impact on Parents. These children used more medications than children without CP from a national sample. Fifty-nine children used feeding tubes. Children in GMFCS level V who used a feeding tube had the lowest estimate of mental age, required the most health care resources, used the most medications, had the most respiratory problems, and had the lowest Global Health scores. Children with the most severe motor disability who have feeding tubes are an especially frail group who require numerous health-related resources and treatments. Also, there is a relationship among measures of health status such as the CHQ, functional abilities, use of resources, and mental age, but each appears to measure different aspects of health and well-being and should be used in combination to reflect childrens overall health status.

Growth and Health in Children With Moderate-to-Severe Cerebral Palsy

BACKGROUND. Children with cerebral palsy frequently grow poorly. The purpose of this study was to describe observed growth patterns and their relationship to health and social participation in a representative sample of children with moderate-severe cerebral palsy. METHODS. In a 6-site, multicentered, region-based cross-sectional study, multiple sources were used to identify children with moderate or severe cerebral palsy. There were 273 children enrolled, 58% male, 71% white, with Gross Motor Function Classification System levels III (22%), IV (25%), or V (53%). Anthropometric measures included: weight, knee height, upper arm length, midupper arm muscle area, triceps skinfold, and subscapular skinfold. Intraobserver and interobserver reliability was established. Health care use (days in bed, days in hospital, and visits to doctor or emergency department) and social participation (days missed of school or of usual activities for child and family) over the preceding 4 weeks were measured by questionnaire. Growth curves were developed and z scores calculated for each of the 6 measures. Cluster analysis methodology was then used to create 3 distinct groups of subjects based on average z scores across the 6 measures chosen to provide an overview of growth. RESULTS. Gender-specific growth curves with 10th, 25th, 50th, 75th, and 90th percentiles for each of the 6 measurements were created. Cluster analyses identified 3 clusters of subjects based on their average z scores for these measures. The subjects with the best growth had fewest days of health care use and fewest days of social participation missed, and the subjects with the worst growth had the most days of health care use and most days of participation missed. CONCLUSIONS. Growth patterns in children with cerebral palsy were associated with their overall health and social participation. The role of these cerebral palsy-specific growth curves in clinical decision-making will require further study.

Identification of malnutrition in children with cerebral palsy: poor performance of weight-for-height centiles.

This study aims to evaluate the use of the United States National Center for Health Statistics (NCHS) weight‐for‐height centiles (WHC) in screening children with cerebral palsy (CP) for depleted body fat and to identify an alternate screening method. Growth data from 276 children aged from 3 to 12 years with CP were analyzed retrospectively. Height or a proxy for height, mid‐upper arm circumference, weight, and skinfold thicknesses were recorded. Mid‐upper arm fat area was calculated for each participant. The sensitivities and specificities of WHC and a number of alternative anthropometric screening methods for identifying participants with severely depleted fat stores were determined. WHC <10th centile failed to identify 45% of children with severely depleted fat stores. Triceps skinfold thickness <10th centile identified 96% of malnourished children. WHC standards lack adequate sensitivity for identification of severely depleted fat stores in children with CP. Use of triceps skinfold thickness, using cut‐off value of <10th centile for age and sex, is recommended to screen for suboptimal fat stores in children with CP.

Effects of bisphosphonates in children with osteogenesis imperfecta: an AACPDM systematic review

This systematic review of the effects of bisphosphonate treatment in children with osteogenesis imperfecta was conducted using the American Academy for Cerebral Palsy and Developmental Medicine methodology for developing systematic reviews of treatment interventions (Revision 1.1) 2004. Despite a large body of published literature, there have been only eight studies with a sufficiently high level of internal validity to be truly informative. These studies confirm improvement in bone density. Many, but not all studies, demonstrate reduction in fracture rate and enhanced growth. There has been extremely limited evaluation of broader treatment impacts such as deformity, need for orthopedic surgery, pain, functioning, or quality of life. Short‐term side effects were minimal. Which medication and dosing regimen is optimal and how long patients should be treated are unclear. This body of evidence would be strengthened by a larger controlled trial, because many studies lacked adequate power to evaluate stated outcomes. These studies do not address the impacts of bisphosphonates in children with milder forms of osteogenesis imperfecta and severe forms that are not due to mutations in the type I pro‐collagen gene (e.g. types VII and VIII). Additional research is needed into treatment of infants. More studies evaluating medication choices, optimal dosing, duration of treatment, post‐treatment impacts, and long‐term side effects are necessary.

Effects of gastrostomy feeding in children with cerebral palsy: an AACPDM evidence report.

The objective of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) evidence reports is to provide the biomedical research and clinical practice communities with the current state of evidence about various interventions for the management of developmental disabilities. The AACPDM evidence reports aggregate all that has been published about outcomes of an intervention for a medical condition, gauge the credibility (i.e. strength of the internal validity) of that evidence, and identify gaps in our scientific knowledge. Methodology for developing and procedures for approving these systematic reviews can be found on the internet at www.aacpdm.org in the AACPDM Database of Evidence Reports, which contains initial and updated reports.

Linear growth velocity in children with cerebral palsy.

Retrospective analysis of an anthropometric database collected prospectively in children with cerebral palsy (CP) was carried out to evaluate linear growth velocity and identify risk factors for poor linear growth. Growth velocity measures were compared with published norms for prepubertal growth velocity and z scores were calculated. Mean growth velocity z score (Gvz) was ‐0.97 ± 1.9. Boys grew more slowly than girls (mean Gvz = ‐1.5 ± 1.9 versus ‐0.3 ± 1.7 P=0.003). Gvz did not correlate with type of CP or presence/absence of microcephaly. Young age was a risk factor for poor linear growth (mean Gvz = ‐2.40 ± 2.6 for children under 2 years of age versus ‐0.76 ± 1.9 ages 2 to 6 and ‐0.77 ± 1.6 ages 6 to 10, P=0.04). Children at nutritional risk (triceps skinfold thickness ≤5%) grew poorly (mean Gvz = ‐1.46 ± 1.5 versus ‐0.30 ± 1.8, P=0.01). For children over 2 years, those with cognitive impairments grew more slowly than those with normal cognition (mean Gvz = ‐1.25 ± 1.9 versus ‐0.12 ± 1.8, P=0.02) and non‐ambulatory children grew more slowly than ambulatory children (mean Gvz = ‐1.20 ± 1.5 versus ‐0.35 ± 1.9, P=0.03). Prepubertal children with CP grow more slowly than expected compared with age‐ and sex‐based standards. Sex, age, cognitive impairment, ambulatory status, and nutritional state are factors which may contribute to slow growth. These results add to the growing data that children with CP have unique growth patterns. Further study is needed to clarify the factors which contribute to poor linear growth in this population.

Intrathecal baclofen withdrawal simulating neuroleptic malignant syndrome in a child with cerebral palsy.

Intrathecal baclofen infusion (IBI) is being used with increasing frequency in children to treat spasticity and dystonia. In this report, we summarize the clinical course of a 9–year-old boy with quadriplegic cerebral palsy with mixed tonal abnormalities (spasticity and dystonia) experiencing withdrawal from intrathecal baclofen. His clinical course is compared to that of adults experiencing withdrawal from IBI and to neuroleptic malignant syndrome. If unrecognized, this disorder may have significant potential for morbidity and mortality. Clues to diagnosis, appropriate evaluation, and potential treatments are discussed. When a child treated with IBI presents with unexplained multiorgan system dysfunction, particularly if accompanied by evidence of rhabdomyolysis, the integrity of the IBI system must be evaluated. In some cases, evaluation might necessitate surgical exploration. Caregivers most commonly seek urgent evaluation and treatment from their primary care provider when their child experiences fever or acute illness. Primary care providers of children treated with IBI should be made aware of this clinical scenario to prevent delays in diagnosis.Intrathecal baclofen infusion (IBI) is being used with increasing frequency in children to treat spasticity and dystonia. In this report, we summarize the clinical course of a 9–year‐old boy with quadriplegic cerebral palsy with mixed tonal abnormalities (spasticity and dystonia) experiencing withdrawal from intrathecal baclofen. His clinical course is compared to that of adults experiencing withdrawal from IBI and to neuroleptic malignant syndrome. If unrecognized, this disorder may have significant potential for morbidity and mortality. Clues to diagnosis, appropriate evaluation, and potential treatments are discussed. When a child treated with IBI presents with unexplained multiorgan system dysfunction, particularly if accompanied by evidence of rhabdomyolysis, the integrity of the IBI system must be evaluated. In some cases, evaluation might necessitate surgical exploration. Caregivers most commonly seek urgent evaluation and treatment from their primary care provider when their child experiences fever or acute illness. Primary care providers of children treated with IBI should be made aware of this clinical scenario to prevent delays in diagnosis.

Interrater reliability and convergent validity of the American Academy for Cerebral Palsy and Developmental Medicine methodology for conducting systematic reviews

Aim  The aim of this study was to evaluate the interrater reliability and convergent validity of the American Academy for Cerebral Palsy and Developmental Medicine’s (AACPDM) methodology for conducting systematic reviews (group design studies).

Controversies in the Field of Hearing Impairment: Early Identification, Educational Methods, and Cochlear Implants.

One per 1,000 infants has severe to profound congenital hearing loss that, without intervention, will profoundly affect development. It is hoped that neonatal hearing screening will improve outcomes for individuals who are congenitally deaf. However, early identification will not yield the anticipated outcome gains without early intervention. The community speech and language pathologist, audiologist, pediatrician, or infant educator, in consultation with the referral audiologist, is charged with counseling the family and identifying appropriate services. This article explores available intervention choices including methods of communication and cochlear implantation.