World Sickle Cell Day

June 17, 2016

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2K14-0202-PVK_3793 Jamal Whyte - for Sickle Cell brochure

Sunday, June 19 is World Sickle Cell Day.  World Sickle Cell Day was presented as a resolution by the United Nations in 2008. The goal of this day is to recognize sickle cell anemia is a public health problem and promote education about this genetic condition.

Sickle cell disease is one of the most common inherited disease worldwide with more than 400,000 babies affected each year. In the United States alone, approximately 12,000 babies are born every year with sickle cell disease. Sickle cell disease can occur in any race or ethnic group, but is most common in people of African or Hispanic descent. In the U.S., one in every 500 black-Americans and one in every 3,600 Hispanic Americans have sickle cell disease.

Red blood cells should be shaped like a disc that looks like a doughnut with the hole filled. This shape allows red blood cells to flow freely in the blood vessels. Sickle cell disease is an inherited blood disorder of hemoglobin, a protein in the red blood cell. Sickle hemoglobin causes the red blood cells to change into a crescent (like a moon) or sickle shape. Sickle shaped red blood cells tend to clump together and block blood flow in small blood vessels. Additionally, they do not survive as long as a red blood cell without sickle hemoglobin. A combination of a low blood count (anemia) and blood vessel blockage is responsible for the complications of sickle cell disease.

In the U.S., sickle cell disease is diagnosed through the newborn screening process. In the state of Texas, this testing is performed twice: once shortly after birth and then again at 1 week of age. The results of these tests go directly to the physician identified by the child’s parents, with the recommendation the child be referred to a specialized center, like Texas Children’s Sickle Cell Center. This screening allows us to diagnose and identify children with sickle cell disease before complications occur.

Having a child diagnosed with sickle cell disease can be scary for parents but treatment of sickle cell disease and prevention of complications allow most patients to live into their 40s to 50s. This begins with the initiation of penicillin shortly after birth to prevent life threatening infections. It is important for these children to be seen in a clinic that specializes in sickle cell disease where parents can receive education on the disease and complications, screening for those complications and disease modifying treatments like hydroxyurea or chronic transfusions.

Texas Children’s Sickle Cell Center is the largest center of its kind in Texas, and one of the largest in the U.S. Our multidisciplinary team is made up of board-certified pediatric hematologists, hematology-trained nurse practitioners and physician assistants, research staff, nurse coordinators and social workers. Texas Children’s Sickle Cell Center offers ambulatory services, including outpatient blood transfusion, a sickle cell pulmonary clinic and genetic counseling, all in one location. Children with sickle cell disease can also be seen in our clinic at Texas Children’s Hospital West Campus.

Our program conducts state-of-the-art clinical and laboratory research aimed at gaining a better understanding of the disease, preventing its complications and ultimately finding a cure. The center’s research funding is provided by government grants and the philanthropic support of a variety of community-based organizations. This partnership allows us to offer even more novel therapies to the thousands of children we treat.

Together with education and awareness we can remove the stigma associated with this condition. We can be the voice of the thousands of people who are diagnosed with this condition each year. So this World Sickle Cell Day let us celebrate the millions of people living with sickle cell disease and focus our attention on finding more treatments and inevitably a cure for this condition.

Post by:

Amber M. Yates, MD

Dr. Amber Yates is Assistant Director of the Clinical Division where she oversees outpatient care. She is also Co-Director of the Sickle Cell Program and a member of the Hematology Center.

Her primary clinical interest is in the care of children with sickle cell disease and other...

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