In 2019, the Texas Legislature designated September as Sickle Cell Disease (SCD) Awareness Month to shine a light on this hereditary blood disorder. September is also National Sickle Cell Awareness Month.
As awareness grows, so does the capacity to help Texans living with the disorder.
According to the Centers for Disease Control and Prevention, SCD causes the body to produce crescent or sickle-shaped red blood cells. These cells have; difficulty passing through small blood vessels and block normal red blood cells from entering tissue. Parts of the body that don’t receive normal blood flow eventually become damaged. Complications can include anemia, vision loss, chronic pain, deep vein thrombosis, infection, pulmonary embolism and stroke.
Approximately 180–200 babies are diagnosed with SCD every year, according to the Texas Newborn Screening program. Approximately 6,000 babies are born with sickle cell trait (SCT) each year. This means they inherited one sickle cell gene and one normal gene. These babies do not have SCD and generally lead normal lives but can pass the sickle cell gene to their future children.
If both parents have SCT (each has one sickle cell gene), with each pregnancy the odds of having a child with SCD is 25%, SCT is 50%, and no gene variant is 25%.
Newborn Screening for SCD
In 1983, the Texas Newborn Screening Program began screening newborns for SCD. Blood samples are collected from a heel stick done 24 to 48 hours after birth and again at 1 to 2 weeks of age. The samples are sent to the DSHS Laboratory for screening. Symptoms of SCD usually appear when an infant is around 5 months old. Early diagnosis helps parents access information and specialty health care for their child.
In Texas, approximately one out of every 2,000 newborns has SCD. SCD predominantly affects Black families, who make up an average of 85% of cases in Texas over the last few years. Hispanics account for about 5%, and other ethnicities combined make up the other 10%.
SCD worsens over time. Evolving treatments can decrease complications and improve a person’s life. These treatments include stem cell transplantation, gene therapy and new drugs approved by the Food and Drug Administration.
“We now have three new medications, in addition to hydroxyurea, and our understanding of potentially curative therapies, such as bone marrow transplant, and gene therapy is rapidly growing,” said Dr. Melissa Frei-Jones, vice chair of the DSHS Newborn Screening Advisory Committee and member of the Sickle Cell Task Force. “It is important to not lose sight of the significance that less than five years ago, this disease, which affects more than 100,000 Americans, only had one approved medication. We must continue to raise awareness of sickle cell advocating for increased research funding, access to comprehensive medical care and improved quality of life.”
To learn more about SCD, watch this video featuring Dr. Titilope Fasipe and visit the DSHS Sickle Cell Disease and Centers for Disease Control and Prevention Sickle Cell Disease webpages.