Direct care staff and parents'/legal guardians' perspectives on end-of-life care in a long-term care facility for medically fragile and intellectually disabled pediatric and young adult residents.

OBJECTIVE: Children and young adults with severe disabilities and their families are faced with enormous challenges throughout the lifespan, including admitting the child to a long-term care facility (LTCF) and making end-of-life (EOL) care decisions. While children are residents of these specialized LTCF, the majority of their daily care, even up until death, is provided by nursing aides or habilitation aides (HAs) with limited training and educational backgrounds compared with other licensed healthcare providers. The purpose of this study was to determine the impact of a resident's EOL experience on the primary HAs and parents/guardians. METHOD: Thirty-five resident deaths occurred at Hattie Larlham Center for Children with Disabilities (HLCCD) between January 1, 2006 and February 28, 2009. The HAs and parents/legal guardians were identified for each death and invited to complete three surveys per resident (FAMCARE, Impact of Events Scale (IES)-revised, and Perspective on End-of-Life Care) to assess their experience. There were 112 surveys mailed to 62 HAs and 47 surveys mailed to 47 parents. RESULTS: Forty-two surveys were returned from 18/62 HAs (response rate 29%) and 11/47 parents/legal guardians completed the surveys (response rate 23%). The FAMCARE survey found that parents were more satisfied with the EOL care than were the HAs. The IES-revised found no difference in traumatic responses from either group. Comments from the Perspective on End-of-Life Care survey were analyzed qualitatively for common themes including pain control, respect, decision making, environmental needs, resources, and support. SIGNIFICANCE OF RESULTS: Because of a low response rate, it was difficult to draw significant conclusions; however, several interesting trends were noted regarding the number of deaths HAs experienced, satisfaction with care, and distress. The special needs of this population and their caregivers can provide crucial insights into interventions (e.g. chaplaincy support, debriefings, anticipatory counseling, environmental changes) that might be of benefit for any caregiver or parent of a child with a long-term, chronic condition, particularly involving developmental disability.

Anthropometric measures: poor predictors of body fat in children with moderate to severe cerebral palsy.

AIM: This purpose of our study was to assess and compare anthropometric measures of adiposity and direct measurement of percentage body fat by dual emission X-ray absorptiometry (DXA) in children with cerebral palsy (CP). We also compared our results in children with CP with results from a national sample of typically developing children from the National Health and Nutrition Examination Survey. METHOD: Anthropometry and DXA were obtained from 58 participants with CP (25 females, 33 males; Gross Motor Function Classification System levels III-V; mean age 13 y 1 mo [SD 3 y], range 8-18 y). Height was estimated from knee height, which was measured with knee height calipers; weight was measured on a sitting scale. The relation between percentage body fat measured by DXA and z-scores of each of the anthropometric measures (body mass index, mid-upper arm circumference, triceps skinfold, and mid-upper arm fat area) was assessed by linear models. Agreement analysis was performed to assess the ability of each anthropometric measure to predict percentage body fat by DXA. RESULTS: None of the anthropometric measures were adequately associated with percentage body fat by DXA. All anthropometric methods tended to underestimate percentage body fat in children with CP. INTERPRETATION: Single anthropometric measures do not perform well in predicting percentage body fat in children with or without CP. Further work is needed to develop clinically useful and simple assessments that will predict percentage body fat and to determine the relation between percentage body fat and health to guide clinical practice.

Assessment and correction of skinfold thickness equations in estimating body fat in children with cerebral palsy.

AIM: To assess the accuracy of skinfold equations in estimating percentage body fat in children with cerebral palsy (CP), compared with assessment of body fat from dual energy X-ray absorptiometry (DXA). METHOD: Data were collected from 71 participants (30 females, 41 males) with CP (Gross Motor Function Classification System [GMFCS] levels I-V) between the ages of 8 and 18 years. Estimated percentage body fat was computed using established (Slaughter) equations based on the triceps and subscapular skinfolds. A linear model was fitted to assess the use of a simple correction to these equations for children with CP. RESULTS: Slaughter's equations consistently underestimated percentage body fat (mean difference compared with DXA percentage body fat -9.6/100 [SD 6.2]; 95% confidence interval [CI] -11.0 to -8.1). New equations were developed in which a correction factor was added to the existing equations based on sex, race, GMFCS level, size, and pubertal status. These corrected equations for children with CP agree better with DXA (mean difference 0.2/100 [SD=4.8]; 95% CI -1.0 to 1.3) than existing equations. INTERPRETATION: A simple correction factor to commonly used equations substantially improves the ability to estimate percentage body fat from two skinfold measures in children with CP.

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.

Urolithiasis with topiramate in nonambulatory children and young adults.

Urolithiasis occurs infrequently in the pediatric population, where metabolic factors play a primary role in the pathogenesis of stone formation. Topiramate, an antiepileptic drug, is associated with a kidney stone in 1.5% of patients in published clinical trials. However, this risk may be much higher in certain populations with multiple preexisting risk factors. We performed a retrospective review of all nonambulatory and neurologically impaired individuals in a long-term care facility. Three groups were involved: those with no exposure to antiepileptic drugs, those on antiepileptic drugs other than topiramate, and those who had been treated with topiramate. Thirteen of 24 (54%) individuals on topiramate monotherapy or polytherapy developed clinical evidence of urolithiasis after a mean duration of 36.4 months. Our results suggest that nonambulatory and neurologically impaired individuals in a long-term care facility appear to be at higher risk of developing kidney stones with topiramate than previously reported.

Growth and nutritional status in residential center versus home-living children and adolescents with quadriplegic cerebral palsy.

OBJECTIVE: To describe growth and nutrition in nonambulatory youth (<19 years of age) with cerebral palsy (CP) living in residential centers compared with similar youth living at home. STUDY DESIGN: A multicenter, cross-sectional, single observational assessment of 75 subjects living in a residential care facility compared with 205 subjects living at home. Primary outcome measures included anthropometric measures of height, weight, triceps, and subscapular skinfolds, and mid-upper-arm muscle area. Z scores were calculated from reference values for healthy children. Age, use of a feeding tube, and Gross Motor Functional Classification System (GMFCS) level were included as important confounders. RESULTS: Use of a feeding tube was associated with higher skinfold Z scores, and a significantly higher percentage of the residential subjects had a feeding tube. Height, weight, and arm-muscle area Z scores all diverged (negatively) from reference values with age, and the residential subjects were on average older than the home-living subjects. After controlling for age, GMFCS level and use of a feeding tube, residential living was associated with significantly greater weight, height, skinfold thicknesses, and mid-arm muscle area Z scores. CONCLUSION: Poor growth and nutrition in children with CP is a prevalent, important, and complex problem. Although factors intrinsic to the condition of CP likely play a significant role, it is also clear that environmental factors, including the living situation of the child, can have an impact.