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

• Compare the raw scores of the Brigance Preschool Screen II between SCD and control groups. [ Time Frame: 4 years ] [ Designated as safety issue: No ]
• 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. [ Time Frame: 4 years ] [ Designated as safety issue: No ]
• Compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls [ Time Frame: 4 years ] [ Designated as safety issue: No ]
• Assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD. [ Time Frame: 4 years ] [ Designated as safety issue: No ]
• Assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls. [ Time Frame: 4 years ] [ Designated as safety issue: No ]

• Compare the raw scores of the Brigance Preschool Screen II between SCD and control groups. [ Time Frame: 3 years ] [ Designated as safety issue: No ]
• 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. [ Time Frame: 3 years ] [ Designated as safety issue: No ]
• Compare the pass/fail rate and the raw scores between SCD patients on hydroxyurea treatment and the healthy controls [ Time Frame: 3 years ] [ Designated as safety issue: No ]
• Assess the influence of medical factors on the Brigance Preschool Screen II performance in children with SCD. [ Time Frame: 3 years ] [ Designated as safety issue: No ]
• Assess the influence of socioeconomic factors on the Brigance Preschool II results in children with SCD and controls. [ Time Frame: 3 years ] [ Designated as safety issue: No ]

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.(1)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.

The patient group will consist of 3 year-old-children with SCD who are followed at the St. Jude Children's Research Hospital Sickle Cell Center. The control group will consist of 3-year-old children attending day care in Memphis.

• Other: Brigance Preschool Screen -II
  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.
• Other: Brigance Preschool Screen -II

  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.

• Patient Group

  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

  Intervention: Other: Brigance Preschool Screen -II
• control group

  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

  Intervention: Other: Brigance Preschool Screen -II

PATIENT GROUP

Inclusion criteria:

• 3.0-<4.0 years of age
• African-American
• Diagnosis of sickle cell disease (HbSS, HbSC, HbSBetathal, HbSbeta+thal)
• 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, HbBetathal, HbSbeta+thal)
3. Previously tested with Brigance Preschool Screen-II