Sickle Cell Disease
Conditions
Keywords
Neuro cognitive deficits;, Brigance pre-school screen;
Brief summary
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
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.
Interventions
OTHERBrigance 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.
Sponsors
St. Jude Children's Research Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
3 Years to 4 Years
Healthy volunteers
Yes
Inclusion criteria
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
Design outcomes
Primary
| Measure | 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
| Measure | 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 |
Countries
United States
Outcome results
None listed