Nancy Lyon

Interrater agreement and stability of the functional independence measure for children (weefim™): Use in children with developmental disabilities

OBJECTIVE Examination of the interrater agreement and stability of ratings obtained using the Functional Independence Measure for Children (WeeFIM) in a sample of children with developmental disabilities. DESIGN A relational design was used in which two sets of WeeFIM scores were collected under four conditions: same rater-short interval; same rater-long interval; different rater-short interval; and different rater-long interval. SETTING WeeFIM scores were collected in outpatient developmental rehabilitation centers, school programs, and the childrens homes. PARTICIPANTS Data were collected for 205 children ranging in age from 11 to 87 months. All children had a medical diagnosis of disability and were receiving habilitative-educational intervention or follow-along services including neurodevelopmental surveillance. INSTRUMENT The WeeFIM instrument examines basic daily living and functional skills in children from birth to 7 years of age. The WeeFIM is modeled after the Functional Independence Measure (FIM) for adults and includes 18 items in the following subscales: self-care, sphincter control, transfers, locomotion, communication, and social cognition. RESULTS Kappa values for items ranged from .44 to .82. Intraclass correlation coefficients (ICC) for the six subscales ranged from .73 to .98. Total WeeFIM ICC values were greater than .95 for all analyses. CONCLUSIONS The WeeFIM ratings for the 205 children with developmental disabilities participating in this investigation were consistent across raters and time.

Neurodevelopmental outcome of infants with hypoplastic left heart syndrome

The neurodevelopmental outcome of hypoplastic left heart syndrome in infants remains unclear. All 11 survivors of staged surgical repair of hypoplastic left heart syndrome received standardized neurodevelopmental assessments at one regional childrens hospital. Seven children (64%) had major developmental disabilities. Quality-of-life outcomes must be considered when management options for children with hypoplastic left heart syndrome are evaluated.

Measuring developmental and functional status in children with disabilities

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.

Functional Assessment and care of Children with Neurodevelopmental Disabilities

OBJECTIVES To determine the relationship between pediatric assessment scores and ratings by parents and teachers regarding the amount of assistance required to complete basic activities of daily living; and to examine the relationship among scores for three commonly used pediatric assessments. DESIGN Prospective correlational study. 205 children with developmental disabilities. The children ranged in age from 11 to 87 mo and included 72 females and 133 males of diverse socioeconomic and ethnic backgrounds. The children were evaluated by using the Battelle Developmental Inventory Screening Test, Vineland Adaptive Behavior Scales, Functional Independence Measure for Children (WeeFIM instrument), and the Amount of Assistance Questionnaire. RESULTS The test-retest reliability coefficients for items on the Amount of Assistance Questionnaire were found to range from 0.82 to 0.97. Correlations among subscale scores and amount of assistance ratings were highest for the WeeFIM instrument and Battelle Developmental Inventory Screening Test. The highest correlation was between WeeFIM total rating and total amount of assistance rating (r = 0.91). CONCLUSION Total WeeFIM instrument ratings and severity of disability were the best predictors of amount of assistance ratings provided by parents and teachers.

Induction Chemotherapy and Conformal Radiation Therapy for Very Young Children With Nonmetastatic Medulloblastoma: Children's Oncology Group Study P9934

PURPOSE P9934 was a prospective trial of systemic chemotherapy, second surgery, and conformal radiation therapy (CRT) limited to the posterior fossa and primary site for children between 8 months and 3 years old with nonmetastatic medulloblastoma. The study was open from June 2000 until June 2006. PATIENTS AND METHODS After initial surgery, children received four cycles of induction chemotherapy, followed by age- and response-adjusted CRT to the posterior fossa (18 or 23.4 Gy) and tumor bed (cumulative 50.4 or 54 Gy) and maintenance chemotherapy. Neurodevelopmental outcomes were evaluated and event-free survival (EFS) results were directly compared with a previous study of multiagent chemotherapy without irradiation (Pediatric Oncology Group [POG] trial 9233). RESULTS Seventy-four patients met eligibility requirements. The 4-year EFS and overall survival probabilities were 50% ± 6% and 69% ± 5.5%, respectively, which compared favorably to the results from POG 9233. Analysis showed that the desmoplastic/nodular subtype was a favorable factor in predicting survival. Our 4-year EFS rate was 58% ± 8% for patients with desmoplasia. Whereas seven of 10 patients who had disease progression before CRT had primary-site failure, 15 of 19 patients who progressed after CRT had distant-site failure. Neurodevelopmental assessments did not show a decline in cognitive or motor function after protocol-directed chemotherapy and CRT. CONCLUSION The addition of CRT to postoperative chemotherapy in young children with nonmetastatic medulloblastoma increased event-free survival compared with the use of postoperative chemotherapy alone. Future studies will use histopathologic typing (desmoplastic/nodular versus nondesmoplastic/nodular) to stratify patients for therapy by risk of relapse.

Measurements of functional outcomes in children with cerebral palsy

This review describes functional measures applicable to children, adolescents, and young adults with cerebral palsy. The World Health Organization (WHO) and National Center for Medical Rehabilitation Research (NCMRR) Models of Impairment, Functional Limitations, Disability, Social Limitations, and Handicap as applied to persons with cerebral palsy are discussed. Motor measurements of impairment include developmental postural control, gross motor function and motor performance rates, and various classifications of cerebral palsy severity. Measures of functional skills in daily living include the Pediatric Evaluation of Disability Inventory (PEDI), Pediatric Functional Independence Measure (WeeFIM), Vineland Adaptive Behavior Scales (VABS), Scales of Independent Behavior (SIB), and Battelle Developmental Inventory. Measures of handicap include family stressors, extended activities of daily living, and health-related quality-of-life. If the promise of legislative policies and family-centered support programs are to be realized, research is required to evaluate the functional effects of developmental therapies, assistive technologies, neuropharmacology, surgical interventions, and educational curricula. MRDD Research Reviews 3:194-203, 1997.

Developmental and functional outcomes in children with a positive newborn screen for Krabbe disease: A pilot study of a phone-based interview surveillance technique

OBJECTIVE To assess the utility of a telephone-based interview system in providing ongoing monitoring of the developmental and functional status of children with both positive newborn screens for Krabbe disease and low galactocerebrosidase activity on confirmatory testing, and to determine whether this approach provides improved compliance with follow-up compared with formal neuropsychological testing. STUDY DESIGN Infants with low galactocerebrosidase activity (as detected by the New York State newborn screening program) were eligible for this longitudinal prospective cohort study. Consenting families were interviewed by telephone at infant ages of 4, 8, 12, 18, and 24 months. Designated instruments were the Ages and Stages Questionnaires, the Clinical Linguistic and Auditory Milestone Scale, the Gross Motor Quotient, the Warner Initial Developmental Evaluation of Adaptive and Functional Skills 50, and the WeeFIM II 0-3 instrument. Assessments with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley III) were scheduled at age 12 and 24 months. RESULTS Seventeen patients were enrolled; 16 were assessed at age 12 and 18 months, and 15 were assessed at age 24 months. Scores were within the normal range on all tests of developmental and functional status, with the exception of expressive language. Only 7 patients completed the Bayley Scales of Infant and Toddler Development, Third Edition assessments; all their scores were in the normal range. CONCLUSION This telephone-based technique allows close monitoring of the developmental and functional status of children with a positive newborn screen for this neurometabolic disease, with special attention to detecting plateauing or regression of developmental milestones. Compliance is improved compared with formal neuropsychological testing.

KINDERGARTEN READINESS AND SPECIAL EDUCATIONAL RESOURCES AT AGES EIGHT TO TEN YEARS IN A COHORT OF EXTREMELY PRETERM INFANTS. † 1624

KINDERGARTEN READINESS AND SPECIAL EDUCATIONAL RESOURCES AT AGES EIGHT TO TEN YEARS IN A COHORT OF EXTREMELY PRETERM INFANTS. † 1624

8 YEAR NEURODEVELOPMENTAL AND EDUCATIONAL OUTCOME OF EXTREMELY LOW BIRTH WEIGHT (ELBW) CHILDREN IN PROPHYLACTIC SURFACTANT OR MATERNAL BETAMETHASONE CLINICAL TRIALS. † 1623

8 YEAR NEURODEVELOPMENTAL AND EDUCATIONAL OUTCOME OF EXTREMELY LOW BIRTH WEIGHT (ELBW) CHILDREN IN PROPHYLACTIC SURFACTANT OR MATERNAL BETAMETHASONE CLINICAL TRIALS. † 1623

Measuring developmental and functional status in children with disabilities.

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.