The Brigance Assessment Of Individual Neurodevelopment In Young Children With Sickle Cell Disease- 2

Study Overview
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Brief Summary
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A preliminary study was conducted involving 88 three-year-old children with sickle cell disease (SCD) who were followed at the St. Jude Children's Research Hospital Sickle Cell Center. They were offered developmental screening with the Brigance Preschool Screen-II test during their regular clinic visits from January 2006 to August 2008. Data from this work showed that 50% of 3 year old children with SCD had low developmental screening scores. In addition, the low scores were found to be associated with less parental education and with speech deficits. However they were not associated with sickle cell genotype and hemoglobin level. The primary goal of this study is to prospectively administer Brigance Preschool Screen -II to 3 year old children with SCD and 3 year old children without SCD who come from similar socioeconomic backgrounds and compare the results between the two groups.

Detailed Description
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The primary objective of this study is to compare development in 3 year old children with SCD who are not on any treatment to age matched healthy controls using pass/fail rate for the Brigance Preschool Screen II. Secondary objectives: To compare the raw scores of the Brigance Preschool Screen II between SCD and control groups. To compare the pass/fail rate and the raw scores between SCD patients who are not on any treatment and those who are being treated with hydroxyurea. To compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls To assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD. To assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls.

Official Title
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The Brigance Assessment Of Individual Neurodevelopment In Young Children With Sickle Cell Disease- 2

Conditions
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Sickle Cell Disease

Intervention / Treatment
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* Other: Brigance Preschool Screen -II
* Other: Brigance Preschool Screen -II


Participation Criteria
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Eligibility Criteria
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PATIENT GROUP Inclusion criteria: 3.0-<4.0 years of age African-American Diagnosis of sickle cell disease (HbSS, HbSC, HbSβºthalassemia, HbSβ^+thalassemia) Followed at St. Jude Children's Research Hospital Sickle Cell Center Exclusion criteria: Previous stroke Patients who are currently on a chronic transfusion program Known diagnosis associated with significant cognitive impairment (e.g. Down syndrome, mental retardation) Previously tested with Brigance Preschool Screen-II CONTROL GROUP Inclusion criteria: 3.0-<4.0 years of age African-American Attendee of day-care in Memphis area Exclusion criteria: Known diagnosis associated with significant cognitive impairment (e.g. stroke, Down syndrome, mental retardation) Known diagnosis sickle cell disease (HbSS, HbSC, HbSβºthalassemia, HbSβ^+thalassemia) Previously tested with Brigance Preschool Screen-II

Ages Eligible for Study
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Minimum Age: 3 Years
Maximum Age: 4 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patient Group<br>This study will administer Brigance Preschool Screen -II to 3 year old children with SCD followed at St. Jude Children's Research Hospital Intervention: Brigance Preschool Screen -II | Other: Brigance Preschool Screen -II<br>* Three-year-old children with SCD who are followed at the St. Jude Children's Research Hospital Sickle Cell Center will be offered developmental screening with the Brigance Preschool Screen II during their regular clinic visits. Along with the screening test, socioeconomic data will be collected using a short questionnaire. Families will be asked to participate in this study by allowing us to collect their test results, medical information and socioeconomic data to compare with data collected from the control group. Parents who decline participation in this study will still be offered the Brigance Preschool Screen II as part of their child's clinical care.<br>|
| control group<br>The control group will consist of 3-year-old children attending day care in the Memphis area and serve as a population that come from a similar socioeconomic background as the SCD patient population. Intervention: Brigance Preschool Screen -II | Other: Brigance Preschool Screen -II<br>* Children in the Control Group will be given the Brigance preschool Screen-II Screening at the daycare they attend. The STARR coordinators have selected four daycare centers that have agreed to serve as control group sites. The family will be asked not to respond or aid the child during the assessment. One parent/guardian will fill out the Socioeconomic Data Collection Form while the examiner works with the child. The screening requires the child to give both verbal and non-verbal responses. The examiner records responses on the Three-Year-Old Child Data Sheet for the Brigance Preschool Screen-II. After the screening is complete the examiner collects the Socioeconomic Data Collection Form from the parent and leaves the room to score the assessment and write recommendations. The examiner then returns to the clinic room to discuss the results with the parent/guardian.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Compare development in 3 year old children with SCD who are not on treatment to age matched controls using pass/fail rate for the Brigance Preschool Screen II. |  | 4 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Compare the raw scores of the Brigance Preschool Screen II between SCD and control groups. |  | 4 years | 
| Compare the pass/fail rate and the raw scores between SCD patients who are not on any treatment and those who are being treated with hydroxyurea. |  | 4 years | 
| Compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls |  | 4 years | 
| Assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD. |  | 4 years | 
| Assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls. |  | 4 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Neuro cognitive deficits;, Brigance pre-school screen;