Richard D. Stevenson

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.

Promotion of Physical Fitness and Prevention of Secondary Conditions for Children With Cerebral Palsy: Section on Pediatrics Research Summit Proceedings

Inadequate physical fitness is a major problem affecting the function and health of children with cerebral palsy (CP). Lack of optimal physical activity may contribute to the development of secondary conditions associated with CP such as chronic pain, fatigue, and osteoporosis. The purpose of this article is to highlight the content and recommendations of a Pediatrics Research Summit developed to foster collaborative research in this area. Two components of physical fitness—muscle strength and cardiorespiratory fitness—were emphasized. Although there is evidence to support the use of physical fitness interventions, there are many gaps in our current knowledge. Additional research of higher quality and rigor is needed in order to make definitive recommendations regarding the mode, intensity, frequency, and duration of exercise. Outcome measurements have focused on the body functions and structures level of the International Classification of Functioning, Disability and Health (ICF), and much less is known about effects at the activities and participation levels. Additionally, the influence of nutritional and growth factors on physical fitness has not been studied in this population, in which poor growth and skeletal fragility have been identified as serious health issues. Current intervention protocols and outcome measurements were critically evaluated, and recommendations were made for future research.

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.

The Development of Normal Feeding and Swallowing

The development of feeding skills is an extremely complex process influenced by multiple anatomic, neurophysiologic, environmental, social, and cultural factors. Most children negotiate the necessary developmental sequence without significant difficulties. An understanding of the development of normal feeding abilities aids the pediatrician in monitoring this remarkable process in his or her normal patients. This understanding also helps equip the pediatrician who is challenged by a child with complex feeding problems. The following statements summarize the major elements of feeding development. 1. Structural integrity is essential to the development of normal feeding and swallowing skills. Infant anatomy differs from adult anatomy. Anatomic changes associated with growth affect feeding function. 2. Normal infant feeding is reflexive, under brainstem control, and does not require suprabulbar input. As feeding development progresses, basic brainstem-mediated responses come under voluntary control through the process of encephalization. 3. The mature swallow consists of a voluntary oral-preparatory phase, a voluntary oral phase, and involuntary pharyngeal and esophageal phases. The infant swallow does not have a voluntary oral-preparatory and oral phase but is otherwise similar. 4. The neurophysiologic control of feeding and swallowing is complex and involves sensory afferent nerve fibers, motor efferent fibers, paired brainstem swallowing centers, and suprabulbar neural input. Close integration of sensory and motor functions is essential to the development of normal feeding skills. 5. Feeding development, although dependent on structural integrity and neurologic maturation, is a learned progression of behaviors. This learning is heavily influenced by oral sensation, fine and gross motor development, and experiential opportunities. 6. The basic physiologic complexity of feeding is compounded by individual temperament, interpersonal relationships, environmental influences, and culture. 7. The main goal of feeding is the acquisition of sufficient nutrients for optimal growth and development. Malnutrition may result directly from feeding problems and may also help perpetuate them. 8. Protection of the airway during swallow is a reflexive, multileveled function consisting of the apposition of the epiglottis and aryepiglottic folds and the adduction of both false and true vocal folds.

Psychometric properties of the quality of life questionnaire for children with CP

This paper describes the development and psychometric properties of a condition‐specific quality of life instrument for children with cerebral palsy (CP QOL‐Child). A sample of 205 primary caregivers of children with CP aged 4 to 12 years (mean 8y 5mo) and 53 children aged 9 to 12 years completed the CP QOL‐Child. The children (112 males, 93 females) were sampled across Gross Motor Function Classification System (GMFCS) levels (Level I=18%, II=28%, III=14%, IV=11%, V=27%). Primary caregivers also completed other measures of child health (Child Health Questionnaire; CHQ), QOL (KIDSCREEN), and functioning (GMFCS). Internal consistency ranged from 0.74 to 0.92 for primary caregivers and from 0.80 to 0.90 for child self‐report. For primary caregivers, 2‐week test‐retest reliability ranged from 0.76 to 0.89. The validity of the CP QOL is supported by the pattern of correlations between CP QOL‐Child scales with the CHQ, KIDSCREEN, and GMFCS. Preliminary statistics suggest that the child self‐report questionnaire has acceptable psychometric properties. The questionnaire can be freely accessed at http://www.deakin.edu.ac/hmnbs/chase/cerebralpalsy/cp_qol_home.php

CLINICAL CORRELATES OF LINEAR GROVWH IN CHILDREN WITH CEREBRAL PALSY

The purpose of this cross‐sectional study was to determine correlates of linear growth in children with cerebral palsy (CP). 171 children with CP were measured and their charts reviewed, z scores were calculated for weight (Wz) and height (Hz). Hz correlated positively with Wz and head circumference, and negatively with age, the presence of spastic quadriplegia, non‐ambulation and seizures. The correlation between Hz and age was stronger when non‐ambulatory children were analysed separately. Multiple linear regression resulted in only Wz and age contributing significantly to the variance in stature as measured by Hz. These results provide preliminary evidence that nutritional status is a major correlate of growth in CP. The finding that linear growth worsens with age independent of nutrition suggests that other factors also influence growth in CP.

Growth and Nutrition Disorders in Children with Cerebral Palsy.

Growth and nutrition disorders are common secondary health conditions in children with cerebral palsy (CP). Poor growth and malnutrition in CP merit study because of their impact on health, including psychological and physiological function, healthcare utilization, societal participation, motor function, and survival. Understanding the etiology of poor growth has led to a variety of interventions to improve growth. One of the major causes of poor growth, malnutrition, is the best-studied contributor to poor growth; scientific evidence regarding malnutrition has contributed to improvements in clinical management and, in turn, survival over the last 20 years. Increased recognition and understanding of neurological, endocrinological, and environmental factors have begun to shape care for children with CP, as well. The investigation of these factors relies on advances made in the assessment methods available to address the challenges inherent in measuring growth in children with CP. Descriptive growth charts and norms of body composition provide information that may help clinicians to interpret growth and intervene to improve growth and nutrition in children with CP. Linking growth to measures of health will be necessary to develop growth standards for children with CP in order to optimize health and well-being.

Fracture rate in children with cerebral palsy

Objectives: To determine the prevalence of previous fracture, the rate of fracture over time and associated risk factors for fracture in children with moderate or severe cerebral palsy (CP). Study design: Three hundred and sixty-four children with moderate-to-severe motor impairment (Gross Motor Function Classification System III, IV and V) enrolled in a multi-centre, region-based longitudinal study of growth, nutrition and health. Of these, 297 had baseline fracture information and 261 children had at least one follow-up assessment. Median duration of follow-up was 1.6 years, for over 600 person-years of follow-up. Results: Forty-six (15.5%) children reported 62 previous fractures at baseline assessment. Children with a history of fractures at baseline were older (mean age 11.9 vs. 8.9 years, p < 0.0001) and had greater body fat (triceps z-score −0.01 vs. −0.68, p = 0.0003) than children with no previous fracture. Twenty children (6.7%) reported 24 fractures during the follow-up period. Factors associated with risk of fracture during the follow-up period were higher body fat ( p = 0.03), gastrostomy use ( p = 0.05) and previous fracture ( p = 0.10). Based on 24 fractures in 604.5 person-years of follow-up, the rate of fracture was 4.0 per hundred children (4.0%) per year. For children with a history of fracture at baseline, the fracture rate was 7.0% per year; for children with gastrostomy, 6.8% per year; and for children with high triceps skinfold, 9.7% per year. Conclusions: Children with moderate or severe CP are at high risk for fracture. Children with greater body fat, feeding gastrostomy and prior history of fracture are at highest risk and may benefit most from intervention. Further longitudinal study and clinical trials in children with CP are needed to better understand the factors contributing to fracture risk in this population and the best methods of prevention and treatment. Objetivos: Determinar la prevalencia de fracturas previas, la tasa de fracturas con el tiempo y factores de riesgo asociados para fracturas en niños con parálisis cerebral de severa a moderada. (CP). Diseño del estudio: 364 niños con deficiencias motoras de moderada a severa (Gross Motor Function Classification System III, IV y V) fueron incluidos en un estudio multicéntrico longitudinal basado en regiones, en relación al crecimiento, nutrición y salud. De estos, 297 tenían información de referencia inicial de fracturas, y 261 niños tenían por lo menos una evaluación de seguimiento. La duración media del seguimiento fue de hasta 1.6 años, para más de 600 seguimientos de personas por años. Resultados: 46 (15.5%) niños reportaron 62 fracturas previas en la evaluación de referencia inicial. Los niños con una historia de fracturas en la referencia inicial tenían más edad (edad promedio 11.9 vs. 8.9 años, (p<0.0001) y tenían mayor grasa corporal (marcador –z del tríceps −0.01 vs. −0.68, p = 0.0003) que los niños sin fractura previa. Veinte niños (6.7%) reportaron 24 fracturas durante el período de seguimiento. Los factores asociados con el riesgo de fractura durante el período de seguimiento fueron mayor grasa corporal (p = 0.05), uso de gastrostomía (p = 0.05) y fractura previa (p = 0.10). En base a 24 fracturas en 604.5 seguimientos persona-año, la tasa de fracturas fue de 4.0 por cien niños (4.0%) por año. Para niños con historia de fractura en la referencia inicial, la tasa de fractura fue 7.0% por año; para niños con gastrostomía, 6.8% por año; y para niños con un mayor pliegue del tríceps, 9.7% por año. Conclusiones: Los niños con CP de moderada a severa están en un alto riesgo de tener fracturas. Los niños con mayor grasa corporal, con gastrostomía y con una historia previa de fractura, están en alto riesgo y pueden beneficiarse de la intervención. Se necesitan estudios longitudinales y pruebas clínicas en niños con CP a futuro para entender mejor los factores que contribuyen al riesgo de fractura en esta población y establecer los mejores métodos de prevención y tratamiento.

The relationship between fractures and DXA measures of BMD in the distal femur of children and adolescents with cerebral palsy or muscular dystrophy

Children with limited or no ability to ambulate frequently sustain fragility fractures. Joint contractures, scoliosis, hip dysplasia, and metallic implants often prevent reliable measures of bone mineral density (BMD) in the proximal femur and lumbar spine, where BMD is commonly measured. Further, the relevance of lumbar spine BMD to fracture risk in this population is questionable. In an effort to obtain bone density measures that are both technically feasible and clinically relevant, a technique was developed involving dual‐energy X‐ray absorptiometry (DXA) measures of the distal femur projected in the lateral plane. The purpose of this study is to test the hypothesis that these new measures of BMD correlate with fractures in children with limited or no ability to ambulate. The relationship between distal femur BMD Z‐scores and fracture history was assessed in a cross‐sectional study of 619 children aged 6 to 18 years with muscular dystrophy or moderate to severe cerebral palsy compiled from eight centers. There was a strong correlation between fracture history and BMD Z‐scores in the distal femur; 35% to 42% of those with BMD Z‐scores less than −5 had fractured compared with 13% to 15% of those with BMD Z‐scores greater than −1. Risk ratios were 1.06 to 1.15 (95% confidence interval 1.04–1.22), meaning a 6% to 15% increased risk of fracture with each 1.0 decrease in BMD Z‐score. In clinical practice, DXA measure of BMD in the distal femur is the technique of choice for the assessment of children with impaired mobility.