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

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

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.

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:

1. To compare the raw scores of the Brigance Preschool Screen II between SCD and control groups.
2. 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.
3. To compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls
4. To assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD.
5. To assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls.

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:

1. Known diagnosis associated with significant cognitive impairment (e.g. stroke, Down syndrome, mental retardation)
2. Known diagnosis sickle cell disease (HbSS, HbSC, HbSβºthalassemia, HbSβ^+thalassemia)
3. Previously tested with Brigance Preschool Screen-II