Brian T. Rogers

The Functional Independence Measure for Children (WeeFIM) Conceptual Basis and Pilot Use in Children With Developmental Disabilities

Few tools are available to pediatricians for tracking and monitoring disability status in children. We describe the conceptual basis and pilot use of the Functional Independence Measure for Children (WeeFIM) . Our pilot use of this instrument in children with limb deficiency, Downs syndrome, spina bifida, cerebral palsy, and extreme prematurity demonstrates that the WeeFIM is a valid measure for tracking disability in preschool age and middle childhood. The WeeFIM measures the impact of developmental strengths and difficulties on independence at home, in school, and in the community. This allows the pediatrician to prioritize interventions for enhancing comprehensive functional outcomes and supporting families.

Silent aspiration prominent in children with dysphagia.

Children with neurologically-based dysphagia are at high risk for silent aspiration. Aspiration can lead to complications such as acute pneumonia and chronic lung disease. Thorough evaluation of the oral, pharyngeal, and esophageal phases of swallowing is crucial for patients with dysphagia. The videofluoroscopic modified barium swallow study (MBS) is the procedure of choice for children to delineate the pharyngeal and upper esophageal phases of the swallow that can only be inferred by bedside clinical assessment. This study describes attributes of aspiration and pharyngeal motility in a large sample of infants and children assessed with MBS. Aspiration was observed in 48 (26%) of 186 children, primarily on liquid before or during swallows. Aspiration was trace (less than 10% of a bolus) and silent in 94%. Relationships to clinical history and implications for management are discussed. Given the lack of objective clinical information to identify children at risk for aspiration, MBS should be considered in all children with severe dysphagia to rule out or confirm aspiration.

Horseback riding in children with cerebral palsy: effect on gross motor function

The effects of recreational horseback riding therapy (HBRT) on gross motor function in children with cerebral palsy (CP: spastic diplegia, spastic quadriplegia, and spastic hemiplegia) were determined in a blinded study using the Gross Motor Function Measure (GMFM). Seventeen participants (nine females, eight males; mean age 9 years 10 months, SE 10 months) served as their own control. Their mean Gross Motor Function Classification System score was 2.7 (SD 0.4; range 1 to 5). HBRT was 1 hour per week for three riding sessions of 6 weeks per session (18 weeks). GMFM was determined every 6 weeks: pre-riding control period, onset of HBRT, every 6 weeks during HBRT for 18 weeks, and 6 weeks following HBRT. GMFM did not change during pre-riding control period. GMFM Total Score (Dimensions A-E) increased 7.6% (p<0.04) after 18 weeks, returning to control level 6 weeks following HBRT. GMFM Dimension E (Walking, Running, and Jumping) increased 8.7% after 12 weeks (p<0.02), 8.5% after 18 weeks (p<0.03), and remained elevated at 1.8% 6 weeks following HBRT (p<0.03). This suggests that HBRT may improve gross motor function in children with CP, which may reduce the degree of motor disability. Larger studies are needed to investigate this further, especially in children. with more severe disabilities. Horseback riding should be considered for sports therapy in children with CP.

Characteristics of dysphagia in children with cerebral palsy.

Videofluoroscopic modified barium swallow (VMBS) examinations may provide clinically relevant information regarding deglutition in children with cerebral palsy and dysphagia. A retrospective review of clinical evaluations and VMBS studies on 90 consecutive children with cerebral palsy and dysphagia was completed. Most children were referred because of concerns regarding airway protection during oral feedings. Most children had multiple disabilities and 93% were nonambulatory. The majority of children were totally dependent for oral feedings (80%). Oral and pharyngeal phase abnormalities were present in almost all patients. Abnormalities of deglutition were observed only while swallowing specific food textures in the majority of patients. Aspiration of specific food textures was significantly more common than aspiration of all food textures (p<0.0001). Finally, aspiration was silent in 97% of the patients. VMBS studies can provide clinicians with valuable information regarding the most appropriate food textures and rates of oral feeding for children with cerebral palsy and dysphagia.

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.

Risk factors for major neurodevelopmental impairments and need for special education resources in extremely premature infants.

The purpose of this cohort study was to determine the incidence of and risk factors for major neurodevelopmental impairments among survivors of extreme prematurity. The study cohort comprised 100 infants born between 24 and 28 weeks of gestational age at one tertiary center from 1983 to 1984. Twenty-five infants (25%) died; 75 (75%) survived until follow-up (mean, 60 months). Standardized neurodevelopmental and psychometric assessments were performed in blind fashion on 68 of the 75 surviving children (91% follow-up). Informal assessments (parent, teacher, and physician reports) were obtained instead for seven (9%) children who had relocated outside of the area. Overall, 19 children (25%) had one or more major impairments: mental retardation, 9; cerebral palsy, 4; multiple impairments, 5; and blindness, 1. Despite a high prevalence of impairments, 95% of children (n = 71) were functionally independent [corrected]. Special educational resources were definitely necessary for seven (9%) and possibly needed for 36 (48%) additional children. Univariate analyses revealed four significant risk factors for cerebral palsy: hydrocephalus (relative risk = 12.2), grades III and IV intraventricular hemorrhage (relative risk = 5.8), 5-minute Apgar score lower than 7 (relative risk = 5.7), and bronchopulmonary dysplasia (relative risk = 5.5). Hydrocephalus was the only significant risk factor observed for mental retardation (relative risk = 5.4). Risk factors predicting a need for special education resources included sepsis (relative risk = 24.9), low socioeconomic status (relative risk = 16.3), and nonwhite race (relative risk = 3.0). Thus our data suggest that biomedical factors appear to confer the greatest risk of major impairments; sociodemographic factors appear to have a significant impact on educational risk in extremely premature infants who do not die. Continued follow-up with biomedical and developmental-social interventions appears warranted to decrease the risk of educational underachievement in this population.

HYPOXEMIA DURING ORAL FEEDING OF CHILDREN WITH SEVERE CEREBRAL PALSY

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 faiigue 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.

Cystic periventricular leukomalacia and type of cerebral palsy in preterm infants

A case series design was used to identify cases of cystic periventricular leukomalacia (N = 31) identified by neurosonography at one regional tertiary intensive care nursery. Patients were preterm infants born at < or = 32 weeks of gestation who had cysts involving predominantly the middle-posterior or posterior periventricular regions. Neurodevelopmental evaluations were made for 26 (96%) of 27 survivors. All infants assessed had cerebral palsy (i.e., 54% quadriplegia, 42% diplegia, and 4% hemiplegia). Most cognitive delays and all sensory impairments occurred in children with quadriplegia. Periventricular cysts were most extensive on parasagittal, anteroposterior views. The parasagittal, anteroposterior extent of periventricular cysts was most accurate in predicting the type and severity of motor and cognitive disabilities. Quadriplegia was associated with larger and more extensive cysts.

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.

FUNCTIONAL STATUS OF EXTREMELY PRETERM INFANTS AT KINDERGARTEN ENTRY

Functional status was formally assessed in 149 of 153 surviving members of an extremely preterm (<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 neurodevelopment impairement. Only 5 per cent were considered to have severe functional limitation. The prevalence of functional limitation varied by defination: 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 functinal at kindergarten entry, but will require continuous monitoring of academic skills.