Fort Worth, Texas,
16
June
2020
|
11:22 PM
America/Chicago

Let's Learn About ... Sickle Cell Disease

Sickle Cell Disease (SCD) causes an abnormal shape of the red blood cells. The red blood cells are shaped like crescents instead of circles. They don’t last long and they can get stuck in the blood vessels, which blocks the blood flow, often resulting in pain and organ damage.

SCD is a group of inherited red blood disorders affecting an important protein in the red blood cell. This protein is called hemoglobin. Hemoglobin helps the red blood cell to carry oxygen to all the different parts of the body. In sickle cell disease, the body produces one or more abnormal types of hemoglobin. This means that the ability of the red blood cell to carry oxygen to all parts of the body is changed. Sickle Cell Disease is a lifelong illness that can result in serious health problems.

There are different forms of Sickle cell disease which are named depending on whether a person inherits a sickle cell trait from each parent or if the person inherits sickle cell trait from one parent and a different abnormal hemoglobin trait from the other parent. Some common types of sickle cell disease include SS disease also known as Sickle Cell Anemia, Sickle Hemoglobin C disease, and Sickle Beta Thalassemia.

Sickle cell disease affects about 100,000 people nationwide. Among the many misconceptions of SCD, African Americans are not the only race affected by the disease. While the African American race makes up a reported 60 to 80 percent of Americans diagnosed, people of East Indian, Middle Eastern, Hispanic and Mediterranean descent may also be affected.

The cure for SCD lies in bone marrow transplants, and while siblings typically are the best matches for this disease, anyone willing to donate can be tested.

Donating bone marrow requires less than 5% of bone marrow cells and is typically taken from the hip of the donor or through a non-surgical procedure in which it is collected through an IV.

While Stem cell transplant can provide a cure for Sickle Cell Disease, it is not an easy cure. There can be many complications. Some patients may not engraft, meaning the donor cells may not grow, and the patient’s own cell grow back instead or a second transplant is needed. There can also be challenges with finding a donor. So unfortunately we cannot consider stem cell transplant to be a universal cure that is available to all with the disease. More research for a universal cure is needed. There are small trials with gene therapy being done currently which I hope will prove successful to offer the chance of cure to even more people with Sickle Cell Disease.

The Sickle Cell Program at Cook Children's Sickle Cell Center serves more than 400 children annually across northern Texas and adjoining states. The program offers testing, diagnosis and treatment for the many types of sickle cell disease. Research is also an important part of the program as the team seeks new ways to treat and prevent the many complications that can occur as a result of this disease. Click here to learn more.

Cook Children's Hematology and Oncology Center is actively involved in sickle cell disease research, including stem cell transplantation. The recognized team of researchers work with leading organizations across the U.S. and around the globe to find solutions to treating, preventing and curing the disease. As a research facility, Cook Children's also has a number of clinical trials available and we are able to match our patients to trials at other facilities. 

The Sickle Cell Program at Cook Children's Sickle Cell Center serves more than 400 children annually across northern Texas and adjoining states.

Our program offers testing, diagnosis and treatment for the many types of sickle cell disease. Research is also an important part of our program as we seek new ways to treat and prevent the many complications that can occur as a result of this disease.

If your child has been diagnosed, you probably have lots of questions. We can help. If you would like to speak to one of our staff, please call our offices at 682-885-4007.

Other resources on this topic:

Get to know Clarissa Johnson, M.D.

Dr. Johnson enjoyed reading as a child, every summer participating in the summer book club at the library which gave out coupons to McDonalds for filling up the form with all the books that were read. Some of the books she read included fictional novels about children with illness such as leukemia and Lupus. This sparked an interest in being a "research scientist" which eventually evolved into an interest in being a doctor. Given her love of working with children, she decided to become a pediatrician. Due to a general interest in math and science, she completed her undergraduate degree in chemical engineering at Tuskegee University. She then attended Case Western Reserve University for medical School. Pediatrics residency training was completed at Cincinnati Children's Hospital and pediatric hematology fellowship at Duke University Medical Center.

She encountered many patients with various blood disorders including sickle cell disease during training. This experience focused her attention on Hematology and specifically sickle cell disease. Her research during fellowship training investigated the effect of certain therapies on the red blood cells of people with sickle cell disease.

Dr. Johnson has been working at Cook Children's medical center since 2009 with a focus in treating blood disorders. She continues to have a special interest in sickle disease care. She served on the Texas Sickle Cell Advisory committee to provide recommendations to the state legislature regarding ways to raise awareness about the disease across the state. She serves as medical staff for Camp Jubilee, the annual summer camp for area children with sickle cell disease.

When away from the medical center, she enjoys attending dance performances and Broadway shows as well as traveling and reading.

To make an appointment with Dr. Johnson, click here or call 682-885-4007.

 

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