Sickle Cell Comprehensive Program

Sickle cell disease (SCD), a group of inherited red blood cell disorders, creates problems with hemoglobin, a protein in red blood cells that carries oxygen throughout the body. Normal disc-shaped red blood cells flex easily to flow through even the smallest blood vessels. But sickle cell disease stiffens hemoglobin inside the cells and changes their shape into a crescent or sickle, named for a crescent-shaped hand tool used to cut grain and grass.

Nationally Recognized Care for Blood Disorders

When your child or family faces health concerns related to sickle cell disease, look no further than the Jimmy Everest Center (JEC) and the Bleeding & Clotting Disorders program at Oklahoma Children’s Hospital OU Health in Oklahoma City. At Jimmy Everest Center, you’ll find Oklahoma’s only comprehensive center for bleeding and clotting disorders in children and adults. You’ll work with a multidisciplinary team of healthcare specialists, including hematologists with extensive training in blood disorders such as sickle cell disease.

Regional Connections for Comprehensive Sickle Cell Care

At Oklahoma Children’s Hospital Jimmy Everest Center, you can take advantage of connections to the Heartland | Southwest Sickle Cell Disease Network, a regional network of the Sickle Cell Disease Treatment Demonstration Program from the U.S. Health Resources and Services Administration (HRSA).

Signs & Symptoms of Sickle Cell Disease

Because sickle-shaped cells can stick to blood vessel walls instead of flowing smoothly, they can create blockages that stop oxygen from reaching tissues, which causes sudden, severe pain and potential long-term damage. Sickle-shaped cells also burst easily, so your body typically can’t produce new red blood cells fast enough for good health, which may create a condition called anemia.

In the first year of life – often around five months of age, children who inherit the two defective genes for sickle cell disease (one from each parent) begin to show early symptoms such as:

• Painful swelling in hands and feet

• Fussiness or tiredness from anemia

• Jaundice – Yellowish skin color or in whites of the eyes

Each person experiences the effects of SCD differently and, with this lifelong illness, those effects change over time. Most changes relate to complications from the disease, including severe pain, anemia, stroke, eye problems, organ damage and infections.

Diagnosing Sickle Cell Disease

A blood test can determine if your child has sickle cell disease or sickle cell trait, a condition of inheriting only one sickle cell gene. Typically, that means your child stays healthy, but the defective gene can pass on to future generations.

Across the nation, all states now test newborns for SCD to help start treatment early. In Oklahoma, the Department of Human Services designated OU Health to lead the sickle cell newborn screening program and the adolescent/young adult transition program to ensure the best possible outcomes for Oklahoma’s future.

As you consider having children, talk with your OU Health specialists about having the test to help determine the possibility of passing on the SCD gene.

Doctors at OU Health also can diagnose SCD before birth by using a sample of amniotic fluid or placenta tissue for the test.

Treatments for Sickle Cell Disease

When you partner with Jimmy Everest Center and and the Bleeding & Clotting Disorders program at Oklahoma Children’s Hospital to treat your child’s sickle cell disease, you work with a multidisciplinary team of specialists who focus on developing the best health outcomes for your child’s specific situation.

Treatments that can help relieve symptoms, lessen complications and extend life include:

• Antibiotics such as penicillin – Help prevent infections

• Pain relievers – Help address acute and chronic pain

• Hydroxyurea – Medicine that can reduce or prevent many SCD complications by increasing the amount of fetal hemoglobin in the blood; not for everyone; not safe to take during pregnancy

• Childhood immunizations – Help prevent infections

• Blood transfusions – Help address severe anemia; may help prevent more serious complications after a stroke

Your OU Health care team may recommend other treatments, depending on specific complications.

Transplant & Cellular Therapy for Sickle Cell Disease

Bone marrow (stem cell) transplant, currently the only cure for SCD, carries substantial risk and may have serious side effects. Usually used only in children with severe SCD, bone marrow/stem cell transplantation requires a close donor match, often from a healthy brother or sister, for the best outcome.

Learn more about Transplant and Cellular Therapy at OU Health, including Oklahoma’s only pediatric stem cell/bone marrow transplant program accredited with the Foundation for the Accreditation of Cellular Therapy (FACT).

Your Expert Sickle Cell Program Care Team

When you visit Jimmy Everest Center and the Bleeding & Clotting Disorders program at Oklahoma Children’s Hospital, you and your family benefit from extensive conversations with your physicians and nurses to review every aspect of life affected by the challenge of sickle cell disease.

Your highly trained and extensively experienced multidisciplinary team members may include board-certified hematologists, advanced practice providers with expertise in blood disorders, pediatric nurse practitioners (PNP), nurse coordinators, and PhD and Master’s level clinical psychologists and social workers, as well as physical therapists, clinical research staff and a variety of expert OU Health healthcare professionals from among our 450 medical specialties available to consult on your child’s care.