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The purpose of this study was to identify factors associated with health-related quality of life (HRQOL) in individuals with myelomeningocele. Eighteen female and 16 male patients with myelomeningocele (mean age 13y, SD 6y; range 4 to 27y) were prospectively enrolled. Twenty-four of the patients had lesions at or above the L4 level, and 11 were wheelchair dependent. Twenty-five patients had shunted hydrocephalus; mean IQ of the cohort on testing was 85 (SD 18; range 36 to 111). Functional independence was measured in three areas (self-care, mobility, and social cognition) using the Functional Independence Measure for Children (WeeFIM). The Support Function Scale (social supports available to the family), the Amount of Assistance Questionnaire (assistance required for activities of daily living), and the Health Utilities Index II (HUI-II; perceived health status measure) were completed by participants or their parents. Each patient's HRQOL was quantified using a 'feeling thermometer', which is a plastic 'thermometer' scaled from 0 (least desirable health state) to 100 (perfect health). Results of multiple regression analysis using HRQOL as the dependent variable revealed that the Amount of Assistance Questionnaire, WeeFIM self-care, and HUI-II were significantly correlated with quality of life ratings (p < 0.025). Maximizing functional independence should be a priority in improving HRQOL in individuals with myelomeningocele.

This study compared performance on the Functional Independence Measure for Children (WeeFIM), the Battelle Developmental Inventory Screening Test (BDIST), and the Vineland Adaptive Behavior Scales (VABS) in children with developmental disabilities. The three instruments were administered to 205 children with identified disabilities. All 205 children were tested using the WeeFIM instrument. The BDIST was administered to 101 children and the VABS to the remaining 104 children. Administration was counterbalanced and randomized across all three instruments. A proportional sampling plan was used to select the 205 children, who ranged in age from 11 to 87 months. A variety of medical diagnoses and levels of severity of motor, cognitive, and communication impairments were systematically included in the sample. Correlations (r) among subscales for all three instruments ranged from 0.42 to 0.92. Correlations for total scores ranged from 0.72 to 0.94. Analyses of potential moderator variables found no significant relation between age and severity of disability (r=0.05) or between socioeconomic status (SES) and severity of disability (r=0.21). Correlations with age were strongest for those subscale scores involving gross and fine motor skills. Correlations with SES and subscale scores ranged from 0.03 to 0.18. The three instruments provide important information regarding childhood performance in motor, self-care, communicative, cognitive, and social skills. The WeeFIM instrument requires less administration time and provides information directly relevant to evaluating functional outcomes for children with disabilities and their families.

Etiology of the high rates of growth failure in children with cerebral palsy (CP) remains unclear. The purpose of this study was to evaluate the relation between growth failure in preterm infants with cystic periventricular leukomalacia (CPVL) and neonatal health complications. The population consisted of all preterm infants (51) with a gestational age of <33 weeks who were admitted to the Children's Hospital of Buffalo from 1988 to 1993 and who had CPVL. Out of the 41 survivors with CPVL who were followed, 39 developed CP and 18 developed growth failure during infancy. At the time of greatest growth failure, the majority (72%) of infants had signs of undernutrition as defined by the Waterlow (1972) classification. Oral feeding impairment was the sole risk factor for the occurrence of growth failure. Undernutrition appears to be important in the occurrence of growth failure in preterm infants with CPVL and CP.

The reliability of the Functional Independence Measure for Children (WeeFIM) was examined in 37 non-disabled children and 30 children with disabilities, from 12 to 76 months of age. The WeeFIM is derived from the Functional Independence Measure (FIM) and includes 18 items involving six functional subscales. Stability was assessed by administering the WeeFIM instrument to each child's caregiver on two occasions separated by 7 to 14 days. Intraclass correlation co-efficients (ICCs) for individual items ranged from 0.90 to 0.99. The ICC for the six WeeFIM subscales ranged from 0.94 for social cognition to 0.99 for transfers and locomotion. The ICC value for total WeeFIM test-retest reliability was 0.98 for children with disabilities and 0.99 for children without disabilities. Equivalence reliability was examined by comparing ratings obtained when using personal assessment with ratings collected during a telephone interview. No statistically significant differences were found for individual items, subscale scores or total WeeFIM values.

Functional status was formally assessed in 149 of 153 surviving members of an extremely preterm (< or = 28 weeks) birth cohort born at one tertiary center between 1983 and 1986. The children were observed in the completion of motor, speech and self-care tasks, and administered either the Vineland Daily Living Skills Scale (VDLS) or the Functional Independence Measure for children (WeeFIM). 31 children had major neurodevelopmental impairment. Only 5 per cent were considered to have severe functional limitation. The prevalence of functional limitation varied by definition: 11 children were limited using the WeeFIM instrument and 35 using the VDLS instrument. These findings suggest that the majority of extremely preterm children are functional at kindergarten entry, but will require continuous monitoring of academic skills.

Oral feeding of children with severe dysphagia and multiple disabilities may result in hypoxemia. Pulse oximetry was used to monitor hemoglobin saturation (SpO2) during oral feeding of five children with multiple disabilities who were referred because of food refusal or coughing and fatigue during feeding. Modified barium videofluoroscopic swallow studies demonstrated deglutition abnormalities. SpO2 values were within the normal range at rest, but routine, upright oral feeding resulted in significant degrees of hypoxemia. The pharyngeal stage of deglutition was abnormal in all five children. In three, the periods of hypoxemia were dependent on food texture. Awareness of meal-time hypoxemia contributed to the decision to use gastrostomy-tube feedings for the other two children. Pulse oximetry during oral feeding should be considered for all children with severe dysphagia and multiple disabilities.

A consecutive series of 137 patients with cerebral palsy living in institutions and aged between 18 and 30 years was examined for minor malformations, using a modified Weighted Anomaly Score. After examination, the patients were divided into two groups, based on whether their cerebral palsy was prenatal or postnatal in onset. A further subset of prenatal-onset patients with unidentified etiology was also analysed. Both the prenatal group with known etiology and the subgroup with unknown etiology had significantly more minor malformations than the postnatal group. These results suggest that multiple minor malformations may indicate prenatal etiology of cerebral palsy.

Three patients with spastic cerebral palsy and no associated movement disorder--each of whom presented with loss of functional skills and delay in the definitive diagnosis of cervical myelopathy--are reported, in order to increase awareness of the possibility of cervical spine pathology in these adults. The possibility of myelopathy should be investigated when considering the etiology of functional deterioration. A functional neurological examination for all multiply disabled individuals is proposed as a reference for future comparison.