What is sickle cell disease?

Sickle Cell Disease is the most common genetic disease in African-Americans, affecting about one in 400 newborns. It is an inherited defect in blood that causes normally round blood cells to take on a sickle shape. These sickle-shaped cells clog the bloodstream, creating obstructions that result in severe medical complications.

Living with sickle cell disease

In spite of these complications, children with sickle cell disease in St. Louis and across the country are living better lives because of advances in research performed in medical centers like St. Louis Children's Hospital and Washington University School of Medicine.

The cornerstone of improving the quality of life for children with sickle cell disease and their parents is education about their disease. It's part of the Center's job to make sure families have the knowledge they need to navigate through a myriad of complications.

Going to school with sickle cell disease -- educating the patient and the educators

An old problem that has received new attention is the poor school performance of children with chronic illness. Similar to other children with chronic illness, children with sickle cell disease are at increased risk for school absenteeism related to their illness, but unlike most other children with chronic illness, children with sickle cell disease have a high frequency of strokes. Approximately 25 of 100 children with sickle cell disease will have a stroke prior to completing high school.

The center has recently recognized the importance of children with sickle cell disease performing well in school and some of the obstacles preventing them from doing well. These obstacles may include teachers' lack of understanding about the disease in the school, increased school absenteeism rate because of illness, and the presence of silent strokes.

To maximize the educational outcomes of children with sickle cell disease, the division has developed the Stay in School Program.  In this program, the division’s School Liaison engages our patients along with their parents, teachers, and school administrators to ensure that students have appropriate educational supports.  All children with sickle cell disease should have a 504 plan to allow accommodations at school for their medical needs.  Children may qualify for an Individualized Education Plan (IEP) if they have specific learning disabilities.  The School Liaison assists families with the process of requesting and maintaining these educational supports.  Children with sickle cell disease may also be referred for detailed neurocognitive testing and psychological support to identify and address specific school concerns.

Guidance for parents and primary care physicians of sickle cell patients

The Parent Education Program has developed age appropriate guidelines (roadmaps) to assist the primary care physician in caring for children with sickle cell disease. These roadmaps are intended to help with follow-up visits to the primary care physicians and hematology offices. They include immunization schedules and guidelines for preventive healthcare measures specific for children with sickle cell disease.