Supporting Sickle Cell Sufferers

The simplest way to help babies born with sickle cell disease is to donate blood. Here's why.

Over the past few months our hearts have gone out to victims of hurricanes and earthquakes, violence and other events that have resulted in harm to families. Our humanity prompts us to offer help, whether it be financially or by providing items in need, such as water, clothes and food. As a community, we combine our efforts to help in any way that we can. But sometimes we want to give a more personal gift. We also want that gift to help someone in our community. A blood transfusion is that perfect gift.

Blood transfusions are given for a variety of reasons. While we often think about them in the setting of trauma or surgical procedures, blood transfusions are also important for the treatment of blood disorders, including sickle cell disease.

Sickle cell disease is an inherited disorder of the blood that affects the hemoglobin protein and causes red blood cells to have an abnormal shape. Sickle cell disease is actually one of the most common genetic disorders and most frequently affects individuals who have ancestors from Sub-Saharan Africa, the Mediterranean basin, the Middle East and India. There are about 600 children in our area affected by the disease; we see just over 200 kids at St. Jude. Children with sickle cell disease may begin to experience complications related to their disease as young as five to six months of age and these complications continue throughout their lives.

Typical blood cells are round and smooth, but the blood cells of patients with sickle cell disease develop into abnormal sharp, “sickled” or C-shape cells. The shape of red blood cells is crucial to their ability to easily flow through the small vessels in the body and provide adequate oxygen supply to these areas. Sickled red blood cells are unable to flow smoothly through blood vessels, which can cause devastating symptoms for these patients. One of the most common problems is intense pain that occurs when the sickled red blood cells get stuck or clogged in an area. Additionally, the sickled red blood cells have a shorter life span than healthy cells, leading to a red blood cell shortage, or anemia, in most patients with sickle cell disease.

Currently there are very limited treatment options for patients with sickle cell disease. The St. Jude Affiliate Clinic at Novant Health was proud to be part of the L-glutamine (Endari) trial, which led to FDA approval of this treatment in July 2017. Hydroxyurea is another medication that is FDA approved and considered to be a standard of care in sickle cell disease. The only potentially curative treatments are bone marrow transplant and emerging gene therapy treatments.

Red blood cell transfusions are a mainstay of therapy and can be life-saving for patients with sickle cell disease. Transfusions dilute out the sickled red blood cells, giving the patient healthier cells. Transfusions can significantly reduce the severity of the child’s disease, decreasing their risk for stroke, acute chest syndrome, pain and other complications. We rely on the community to donate blood and help ensure that we can continue to administer this life-saving treatment to our patients.

Community involvement is critical because some patients have unique proteins on their red cells that are only seen in donors from their own ethnic group. There are cases when we are unable to obtain red blood cell units due to shortages or rare blood types and, at times, we even have to make national or international requests to obtain units.

The process of donating blood is fairly simple and can be done through a variety of sites. For a donor, it involves little more than registering, filling out a health questionnaire, giving a blood sample, having an IV put in, and waiting less than an hour to donate one unit of blood. However, for the recipients of these units, a unit of blood can make a life-saving difference.

Through blood donation, our community can improve the health and quality of life of children with sickle cell disease, as well as many others. Until a cure is found, our sickle cell community needs you! For more information on how to donate blood, visit The Red Cross or Community Blood Center of the Carolinas.

New to the Charlotte area, Dr. Jenny McDaniel recently joined the St. Jude Affiliate at Novant Health Hemby Children’s Hospital. She enjoys helping patients with all kinds of blood disorders.

Dr. Paulette Bryant has been practicing hematology in Charlotte since 2005. She loves helping pediatric patients at the St. Jude Affiliate at Novant Health Hemby Children’s Hospital experiencing hematology or oncology disorders.

For more information on keeping your child safe and well, visit Pediatric Surgical Associates at pedsurgical.com.