September is National Sickle Cell Month in the U.S.
Sickle cell disease (SCD) is one of the most common inherited blood conditions. 90,000-100,000 people in the U.S., mainly Blacks or African Americans, have SCD. An additional 2 million people carry the sickle cell gene that allows them to potentially pass the disease on to their children.
Among those with SCD, their red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle-shaped cells die early, which causes a constant shortage of red blood cells. When a person doesn’t have enough red blood cells, the have a condition called anemia. Also, when these mis-shaped blood cells travel through small blood vessels, they can get stuck and clog the blood flow. When this happens, it results in severe pain and can lead to organ damage and serious infections.
The good news is that people with SCD can live productive lives and enjoy most of the activities that people without SCD do. There are some things that people with SCD can do to stay as healthy as possible:
- Get regular checkups with a primary care doctor to help prevent serious problems.
- Prevent infections. Wash hands. Get vaccinated. Take penicillin.
- Learn healthy habits. Drink 8 to 10 glasses of water every day and eat healthy food. Try not to get too hot, too cold, or too tired.
- Get support. Find a patient group or other community organization that can provide information, assistance, and support.
THE NEED TO SCREEN FOR SICKLE CELL
- Sickle Cell disease is an inherited, complex disorder with multisystem manifestations that require specialized comprehensive care to achieve optimal outcomes. Newborn screening is a special set of tests that help identify babies who are at risk for certain conditions. A newborn baby may look healthy, but can have a serious condition that cannot be seen.
- Prior to universal newborn screening for sickle cell disease(SCD), many infants were not identified until they experienced clinical problems during infancy or early childhood, including painful swelling of the hands and feet, pneumococcal sepsis or meningitis, severe anemia and acute splenic sequestration, acute chest syndrome, pallor, jaundice or splenomegaly. Identification of infants with SCD through neonatal screening has decreased disease related mortality and morbidity and also provides an opportunity to educate parents, providers, and other caregivers about the child’s disorder before symptoms develop.
How to be screened?
- Infants – Before every baby in Indiana goes home from the nursery, he or she has a small amount of blood taken from his or her heel. This is called a heel stick. The blood from the heel stick is sent to the Indiana Newborn Screening Laboratory and is used to test for a specific group of conditions including sickle cell and other hemoglobinopathies. All abnormal hemoglobin results are forwarded to the state follow-up program, the pediatrician and a DNA reference laboratory for confirmatory testing.
- Children and Adults – Testing for sickle cell disease and trait can be done at your family doctor. A hemoglobin electrophoresis is used to identify a person’s hemoglobin type.
Newborn screening in Indiana – The Sickle-SAFE Program
Newborn screening in Indiana for hemoglobin disease started in 1985. Follow-up was completed by community services agencies. Since 2009, all infants identified by the newborn screening laboratory are referred to the Sickle-SAFE Program located at the Indiana Hemophilia & Thrombosis Center. This program ensures timely confirmatory testing, provides disease specific education to parents/guardians, primary healthcare providers, and caregivers, ensures that all patients with a sickling disorder are started on prophylactic antibiotics by two months of age and provides on-going follow-up through the first year of life to ensure all infants have access to care consistent with National Heart, Lung, Blood Institute (NHLBI) recommendations.
In recognition of National Sickle Cell Disease Know Your Sickle Status – GET TESTED!