World Sickle Cell Awareness Day was officially designated June 19, and the international awareness day is observed annually with the goal to increase public knowledge and an understanding of sickle cell disease, and the challenges experienced by patients and their families and caregivers.
This year marks the 10 anniversary of World Sickle Cell Day, and SiNERGe, in collaboration with Sickle Cell Disease Association of America, Inc. (SCDAA), is launching a 24-hour awareness campaign this year, called “Shine the Light on Sickle Cell.” This national awareness effort encourages community-based organizations, individuals, and anyone concerned about sickle cell disease.
Friends, family, neighbors and others in your community, as people around the nation and across the globe, host and hold local gatherings to shine the light on sickle cell disease.
A leading voice in Sickel cell disease awareness is Dr. M. Laurence Noisette. She was born and raised in Port-au-Prince, Haiti and completed a training in pediatrics after the devastating 2010 earthquake in a resource-limited environment.
Dr. Noisette is a hemotology/oncology specialist who amplifies in her teaching that Sickle cell disease by definition is a group of inherited red blood cell disorders. People who have sickle cell disease have an abnormal protein in their red blood cells.
In the United States, most people who have sickle cell disease are of African ancestry, but the condition is also common in people with a Hispanic background. Because the disease runs in families, couples planning to have children can have genetic testing.
Early signs and symptoms of sickle cell disease include swelling of the hands and feet; symptoms of anemia, including fatigue, or extreme tiredness; and jaundice. Over time, sickle cell disease can lead to complications such as infections, delayed growth, and episodes of pain, called pain crises. Most children who have sickle cell disease are pain-free between crises, but adolescents and adults may also suffer with chronic, ongoing pain. Over a lifetime, sickle cell disease can harm a patient’s spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.
A blood and bone marrow transplant is currently the only cure for sickle cell disease, and only a small number of people who have sickle disease are able to have the transplant. There are effective treatments that can reduce symptoms and prolong life. Early diagnosis and regular medical care to prevent complications also contribute to improved well-being. Sickle cell disease is a lifelong illness. The severity of the disease varies widely from person to person.
One rarely ventured subject regarding the disease is cross-racial inheritance.
Specifically, can bi-racial children
inherit Sickle cell disease?
Yes, they can. Sickle cell disease can affect people of ANY race or ethnicity.
Sickle cell disease, an inherited disorder of the red blood cells, is more common in African Americans in the U.S. compared to other ethnicities—occurring in approximately 1 in 365 African Americans. However, there are other groups of people who also may inherit sickle cell disease.
It is common in Africa, in Mediterranean countries (such as Greece, Turkey, and Italy), the Arabian Peninsula, India, Spanish-speaking regions in South and Central America, and parts of the Caribbean. In all those regions, both dark and light skin people can carry copies of the sickle cell genes.
What's the difference between
sickle cell disease and sickle cell trait?
Just like the color of their skin and eyes, children with sickle cell disease are born with it. Sickle cell disease is an autosomal recessive genetic disease, meaning that children inherit the disease from both parents. Without getting too technical, a child must have two copies of the sickle cell gene—one from each parent—to have sickle cell disease.
If a person only has one copy of the sickle cell gene and no other hemoglobin variant, then he or she does not have sickle cell disease. These people have sickle cell trait, and usually do not have any health-related problems because of the gene. They can still pass on this one copy of the sickle cell gene to their children, however.
How do I know if my child
has sickle cell disease?
Every state in the U.S. provides a newborn screening test for sickle cell trait and sickle cell disease. Each year, the screening tests identify about 2,000 babies born with the disease. It's a good idea to ask your child's doctor for the results of this test and discuss them together. If newborn screening results are not available and your child's doctor is concerned that your child might have sickle cell disease, he or she can also order a different blood test.
M. Laurence Noisette, MD, FAAP