Brain complications in children with sickle cell disease

Children with sickle cell disease are at risk for brain damage because their irregularly shaped sickle cells can interrupt blood flow to the brain. Complete clogging of blood flow to the brain can lead to an “obvious” stroke. This occurs in about 10% of children with sickle cell disease before the age of 14 with the highest period of risk between 3 and 7 years. In addition, damaging strokes that do not cause obvious symptoms but can be seen on MRI images of the brain occur in at least 25% of school-age children with sickle cell disease. These so-called “silent strokes” may result in learning and/or behavior problems.

Are all learning and behavior problems due to clogging of blood flow in the brain?

No. Negative brain effects that result in school difficulty may also occur in the absence of “obvious” or “silent” strokes because of anemia and decreased oxygen to the brain.

Pain events and fatigue may result in multiple absences from school or interfere with attention. These factors also contribute to school difficulty in children with sickle cell disease.

What are the symptoms of an “obvious” stroke?

Some warning signs of a stroke are:

  • Weakness or numbness of a body part
  • Dimness of vision, loss of vision or double vision
  • Severe headache, dizziness or unsteadiness while walking
  • Seizures
  • Change in behavior or change in school performance without obvious cause
  • Excessive sleepiness

Apart from an “obvious” stroke, what other brain complications may occur?

A decrease in general brain functioning has been observed in children with sickle cell disease. This could lead to difficulty with educational skills, especially in math and reading. Other effects that have been observed include problems with attention and decision-making skills such as planning and organizing, difficulty acquiring language skills, and difficulty retrieving information from memory.

When do these brain problems start?

The age at which this damage starts varies from child to child. In general, these problems begin early in life when brain development is most critical. For example, researchers have found that on average, infants with sickle cell disease perform worse on tests of brain function compared to children without sickle cell disease.

How can I reduce the risk of brain complications?

Monitor blood flow in the brain by running ultrasound (Transcranial Doppler) and MRI tests regularly. Your doctor can tell you more about these tests in detail. Talk to your child’s school regarding brain/learning complications from sickle cell disease. Teachers are less likely to blame your child’s school difficulty on “laziness” if they are informed about your child’s health. Schedule special tests for learning and thinking (neuropsychological testing) to assess your child’s strengths and weaknesses, especially if your child has difficulties in school. Repeat testing as needed to monitor changes. You will be able to use this information to plan for specific educational needs.

Can I get help if my child is diagnosed with a learning or behavior difficulty?

Yes. Children with sickle cell disease may qualify to receive support services within the educational environment. Your child may receive help either through Section 504 of the Rehabilitation Law of 1973 or through the Individuals with Disabilities Education Act (IDEA).

How does § 504 of the Rehabilitation Law of 1973 support children with sickle cell disease who have learning problems?

This law guarantees equal access and treatment for individuals with disabilities in agencies (such as public schools) that receive federal money. Individuals with disabilities are “people who have a mental or physical disability that limits major life activities, including learning.”

How does the Individuals with Disabilities Education Act (IDEA) support children with sickle cell disease who have learning problems?

This law requires public schools to work with parents of children with disabilities to create an Individualized Education Plan (IEP) to meet the child’s needs. Some examples of services include classroom modifications, speech/language therapy,occupational therapy, physical therapy, counseling, transportation, medical assistance and other resources.

What is the process for getting help for my child under IDEA?

To get help, start by requesting a special education evaluation from your school. The school’s Admission, Review and Dismissal (ARD) team is required to complete a thorough evaluation within 60 days of a written request. If the ARD team determines that your child qualifies, it will develop an Individualized Education Plan (IEP) for your child. You, as a parent, and any other professionals you choose can be part of your child’s ARD team. Ask the social worker and learning support specialists at Texas Children’s Sickle Cell Center for advice on forming an effective ARD team and developing an IEP for your child.

How is § 504 different from IDEA?

The procedures under Section 504 are not as strict as those of IDEA. Under Section 504, the school has greater discretion in developing a 504 Plan. In addition, unlike IDEA, Section 504 does not require your consent to test and place your child. Section 504 policies vary greatly between schools.