Validation of the fine motor subtest of the Bayley-III with children with sickle cell disease using Rasch analysis.

BACKGROUND: Children with sickle cell disease (SCD) are at risk for fine motor (FM) delays; however, screening for FM impairments is not common among young children with SCD. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) is the most commonly used performance-based developmental assessment. We aim to determine if the FM subtest of the Bayley-III is structured hierarchically in accordance with development and comprehensively evaluates FM development in children with SCD. METHODS: Bayley-III assessments were completed between October 2009 and December 2013. The Bayley-III FM screening test, a shorter and more rapid method of assessing for FM impairments, was not directly administered to participants. Screening test scores were calculated from full Bayley-III scores. RESULTS: Rasch analysis was performed using WINSTEPS. Sixty children with SCD were included in the final Rasch model. The Rasch-generated Wright map, which jointly positions items and persons on the same latent trait, illustrated that the FM items were slightly skewed towards more challenging items, indicating more difficult items may be overrepresented. High item separation values were reported (17.4), and item outfit statistics were less than 1.7. More than one third of items demonstrated overfit, indicating possible item redundancy. The FM subtest and the screening test, a shorter and faster method of assessing skills, were highly correlated (r = 0.993, p < 0.001). CONCLUSION: The Bayley-III FM subtest is structured hierarchically, aligning with motor development, and comprehensively evaluates FM development in children with SCD. The test could be improved by reordering items, removing overfitting items and modifying screening test items to capture all ranges of development. The screening test is comprehensive and has high potential clinical utility among children with SCD.

Development and feasibility of a home-based education model for families of children with sickle cell disease.

BACKGROUND: Children with sickle cell disease (SCD) commonly have cognitive deficits, even among toddlers. Much medical literature emphasizes disease-based factors to account for these deficits. However, the social environment plays a large role in child development. To address the specific needs of early childhood, a monthly hospital-based education program was initiated to educate parents about child development. Education sessions were poorly attended (20-25%) and deemed unsuccessful. This study describes the development and implementation of a home-based education service to teach parents about SCD, developmental milestones and positive parenting techniques. METHODS: This was a prospective, single-arm intervention to study the feasibility of a home-based caregiver education program for families with infants and toddlers with SCD. Parents of children aged 0-3 years with SCD from one Midwestern hospital were approached to participate in a home-based program. The program followed the Born to Learn™ curriculum provided through the Parents as Teachers™ National Center. Reminder calls or texts were provided the day before each visit. Results of the first twenty-six months of the program are presented. RESULTS: A total of 62% (56 of 91) of families approached agreed to participate; all were African American. The majority of caregivers were single mothers with a high school education or less and whose children had Medicaid for health coverage. The phenotypes of SCD represented in this sample were similar to those in the general SCD population. Over 26 months, 39 families received at least one home visit. Parents of infants (younger than 8 months) were more likely to participate in the home-based education program than parents of older children, (Fisher's exact test, p < .001). CONCLUSIONS: For participating families, home-based visits were a feasible method for reinforcing clinic education. About 43% of eligible families participated in the education, a two-fold increase in the poor attendance (20%) for a previous hospital-based program. A home visitation program for parents of infants with SCD could offer an effective approach to helping these children overcome adverse environmental conditions that are compounded by the complexities of a chronic health condition.

Prevalence of daily medication adherence among children with sickle cell disease: a 1-year retrospective cohort analysis.

The objective of this study is to determine the prevalence of adherence to daily medications among children with sickle cell disease (SCD). Prescription records for 12 months were obtained from participants who had insurance in a Medicaid-based single health maintenance organization. Adherence was measured as a ratio between the number of expected days and the observed days between two refill periods for daily medications. A total of 93 children were studied. The average refill prescription rate was 58.4%. More formal strategies are required to identify barriers to prescription refills among children with SCD.

Feasibility of a Community-Based Sickle Cell Trait Testing and Counseling Program.

BACKGROUND: Sickle cell trait (SCT) screening is required at birth in the United States; however, adults rarely know their SCT status prior to having children. PURPOSE: Assess feasibility of a community-based SCT education and testing intervention. METHODS: Participants were recruited from eight community sites to complete an educational program and offered a hemoglobin analysis. A genetic counselor met individually with participants to discuss lab results. RESULTS: Between July 14, 2010 and May 31, 2012, 637 participants completed the educational program. Five hundred seventy (89.5%) provided a blood sample, and 61 (10.9%) had SCT or other hemoglobinopathies. The genetic counselor met with 321 (56.3%) participants. CONCLUSIONS: Community-based SCT testing shows initial feasibility and may increase the number of individuals who know their trait status.

Chronic blood transfusion therapy practices to treat strokes in children with sickle cell disease.

PURPOSE: To identify variations in practices used by nurses for pediatric patients with sickle cell disease (SCD) receiving chronic blood transfusion therapy for strokes. DATA SOURCES: Descriptive study of a convenience sample of 11 nurses who care for children with SCD from nine institutions completed a closed-ended questionnaire consisting of 37 items. Responses reflected practice experience with a total of 189 transfused patients with SCD. CONCLUSIONS: A wide range of nursing practices exists for blood transfusion therapy for children with SCD and strokes. Manual partial exchange transfusion (66%) was the most commonly used method for blood transfusion in children with strokes reported among the nurses surveyed. Simple transfusions and erythrocytapheresis account for 21% and 13% of the practices reported. Opportunities exist to establish evidence-based nursing care guidelines to improve the care of children with strokes receiving blood transfusion therapy. IMPLICATIONS FOR PRACTICE: A wide range of local standard care guidelines for blood transfusion therapy exists. The results of this survey indicate that partial manual exchange transfusion is the most commonly used method of chronic blood transfusion therapy in children with SCD and stroke despite the fact that the magnitude of benefit in comparison with simple transfusion has not been established. Factors such as peripheral venous access, compliance with current chelation regimen, and the presence of antibodies are important considerations in the choice of method.