By: Teddi Softley, PhD, Senior Psychologist and Paula Groncy, M.D. Medical Director, Pediatric Sickle Cell Center, Jonathan Jaques Children’s Cancer Center, Miller Children’s & Women’s Hospital Long Beach
Sickle Cell Disease (SCD) is one of the most common genetic disorders, affecting more than 100,000 Americans. In the United States the disease primarily affects African Americans. About one of every 500 African-American babies in the U.S. has SCD.
What Is Sickle Cell Disease?
SCD is an inherited blood disorder that affects red blood cells. Normal red blood cells are round, soft and flexible and can move through blood vessels easily. In SCD, the red blood cells become sickle shaped, or crescent shaped, making it difficult for the cells to pass through blood vessels. Sickle-shaped red blood cells break apart easily only living 10-20 days, whereas a normal blood cell lives for 120 days. The sickle cells also can clump together and stick to the walls of blood vessels, blocking blood flow.
What Causes Sickle Cell Disease?
SCD is inherited in the same way that people get the color of their eyes, skin and hair. In order for SCD to occur, a sickle cell gene must be inherited from both the mother and the father. Having just one sickle cell gene is called sickle cell trait (SCT) and does not cause SCD. If both parents carry SCT, then their baby has a one in four chance of inheriting SCD. Approximately 1 in 12 African-American babies are born with SCT.
Diagnosing Sickle Cell Disease
The National Institutes of Health (NIH) recommends that all newborns be screened for SCD. Testing at birth is required in every state to ensure that infants affected by SCD receive care immediately.
SCD is diagnosed by a blood test which measures and identifies the different types of hemoglobin, a protein responsible for transporting oxygen throughout the body. The amount of the abnormal sickle hemoglobin determines whether the person is a carrier of a hemoglobin trait or has a specific type if SCD.
Treatment
Currently, the only available cure for SCD is bone marrow transplant, however there are many treatment options available for SCD that are used to improve survival rates and quality of life. Blood transfusions are a common treatment for children with SCD. Children affected by SCD often require regular transfusions through their lifetime.
The Sickle Cell Program at Jonathan Jaques Children’s Cancer Center at Miller Children’s & Women’s Hospital Long Beach offers multi-disciplinary care to children affected by SCD starting in infancy and through the transition to an adult program. Patients receive regular follow-up, screening and tests and emergency care to ensure that their physical, emotional and cognitive needs are addressed in order to maximize their quality of life.
Chronic, but Manageable
Although SCD is chronic, with the help of doctors, friends and family, children with SCD can manage the condition and live their lives to the fullest.
During Sickle Cell Awareness Month and beyond, JJCCC encourages everyone to raise awareness of SCD. To help increase the availability of blood for patients, please visit MillerChildrens.org/BloodDonorCenter.
For more information about SCD, please visit MillerChildrens.org/SickleCell.