What is Sickle Cell Anemia?

According to the U.S. Department of Education and IDEA 2004, this is what they have to say on Sickle Cell Disease:

Regulations: Part 300 / A / 300.8 / c / 8
(8) Orthopedic impairment means a severe orthopedic impairment that adversely affects a child's educational performance. The term includes impairments caused by a congenital anomaly, impairments caused by disease (e.g., poliomyelitis, bone tuberculosis), and impairments from other causes (e.g., cerebral palsy, amputations, and fractures or burns that cause contractures).

Regulations: Part 300 / A / 300.8 / c / 9 / i
(i) Is due to chronic or acute health problems such as asthma, attention deficit disorder or attention deficit hyperactivity disorder, diabetes, epilepsy, a heart condition, hemophilia, lead poisoning, leukemia, nephritis, rheumatic fever, sickle cell anemia, and Tourette syndrome...

Sickle cell disease (SCD) refers to a group of genetic red blood cell disorders. Individuals with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. In other words, in SCD, red blood cells become hard and sticky and have a crescent-like "C" shape to them.

Hemoglobin is the protein in red blood cells that carries oxygen through out the entire body. In order for cells in tissues to receive a steady supply of oxygen, the protein in the hemoglobin takes up oxygen in the lungs and carries it to these tissues in the rest of the body.

Red blood cells with normal hemoglobin look like a disc; however, a red blood cell that has a sickle hemoglobin are more crescent shaped.

The following image shows where normal red blood cells flow freely through a blood vessel, while sickle cells will stick together and cause blockage:

According to the National Heart, Lung, and Blood Institute:

"People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genese causes a person's body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD."

There is no widely available cure for sickle cell disease; however, there are many emerging new treatments that are currently being evaluated. Younger patients with severe SCD can undergo a stem cell transplant involving the transplant of bone marrow of a healthy donor to the SCD patient. Although this can cure a small number of patients, it can have life-threatening side-effects in other patients. Other treatments include gene therapy, and new medications to stimulate production of fetal hemoglobin in order to prevent the red blood cells from sickling.

Sources:

• IDEA 2004
• National Heart, Lung, and Blood Institute
• Health Care Corner
• Centers for Disease Control and Prevention