Parent Educational Program for Children With Sickle Cell Disease

SCD is an inherited blood disorder that is caused by an abnormal type of hemoglobin—the
protein in red blood cells that carries oxygen to tissues. Symptoms include anemia,
infections, organ damage, and intense episodes of pain, which are called "sickle cell
crises." Children with SCD are also at risk for a variety of CNS complications, including
various types of stroke and increased blood flow to the brain. These conditions affect
neuropsychological performance and academic achievement. In children with SCD, there may also
be a relationship between CNS complications, behavioral problems, family environment
stressors, and pain symptoms. For example, children with SCD-related CNS complications have a
higher risk of developing behavioral problems than children without SCD. In turn, behavioral
problems are associated with high levels of family conflict and can be a significant stressor
for families coping with SCD. This kind of stress can then lead to increased difficulty in
dealing with SCD symptoms, particularly pain, for the child. This study will evaluate an
educational support program for parents of children with SCD, conducted either once a year or
four times a year, in terms of the program's effect on children's academic achievement. In
addition, study researchers will also evaluate the program's effect on behavioral
difficulties, pain frequency, and the family environment.

This study will enroll children with HbSS (sickle cell anemia) or HbSb-thal (hemoglobin S
beta thalassemia) SCD. Parents or caregivers of participants will be randomly assigned to
attend the educational support program meetings either once a year or four times a year for 2
years. The educational meetings with the parent/caregiver will be 45 minutes long and will
cover the following four main areas:

1. Provide education regarding the learning issues often seen with children with SCD

2. Provide information regarding special education services in the school system and how
the parent can obtain academic support for his/her child

3. Provide information on how to assist the child to better manage homework

4. Evaluate the child's current level of pain and how pain may affect school attendance

There will be three evaluation timepoints. At baseline and Year 3, children will undergo a
neurodevelopmental evaluation, including academic achievement tests. The parent and child
will complete questionnaires on emotional and behavioral functioning, stress levels, and
family functioning. At Year 2, the children will complete academic achievement tests, and the
questionnaires for the parent and child will be repeated.

Inclusion Criteria:

- Children with either HbSS (sickle cell anemia) or HbSβ-thal (hemoglobin S beta
thalassemia) who are between 6 and 12 years of age

- Child's parent or primary caregiver agrees to participate in the study

Exclusion Criteria:

- Child is monolingual in a language other than English and cannot complete standardized
testing in English

- Parent or caregiver of the child is not fluent in English or Spanish

- Child has some other developmental disability not related to SCD. This would include
Down's syndrome, autism, pervasive developmental disability, cerebral palsy, seizure
disorder, consequences of severe prematurity, or a documented closed head injury that
resulted in loss of consciousness.

- Child has been diagnosed with a significant mental health disorder that is not
responsive to behavioral or medical management. This includes severe depression,
schizophrenia, or bipolar disorder. Children whose mental health problem is
effectively treated are eligible for participation.