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Ask the IU Health Expert: Living fully with sickle cell disease

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Sickle cell disease impacts hundreds of thousands of Americans and, for most people living with the disease, requires complex and lifelong treatment. Riley Hospital for Children at IU Health has a team of experts dedicated to treatment of the disease in children. Below are answers to questions about the disease and how families can help children with sickle cell disease live life to its fullest.


What is sickle

cell disease?

Sickle cell disease is an inherited lifelong blood disorder that causes the body to make abnormal hemoglobin, which is an important component of our red blood cells. This causes the red blood cells to become rigid and take on a curved sickle shape. These sickled cells get caught in the blood vessels throughout the body and can cause damage. The clogging of the blood vessels is the basis for many of the complications seen in sickle cell disease, including severe pain, stroke and organ damage.


How do I know

if I have it?

Each year, 300,000 infants are born with sickle cell disease around the world. Here in the United States, we estimate 100,000 people are living with sickle cell disease. If you were born in the state of Indiana after the 1990s, you were tested for sickle cell disease when you were born. If you have the trait or disease, your parents would have been notified. Individuals who are carriers of the mutation only, called sickle cell trait, do not have the complications that can be seen with patients who have sickle cell disease and therefore do not need regular care or treatment by a hematologist. If you were born prior to this time, you might not have been tested. If you are unsure of your sickle cell status, I would encourage you to talk with your primary care physician to obtain testing.


What are the symptoms and when do they first develop?

The symptoms of sickle cell disease can vary based on the type of sickle cell disease a patient has and how anemic (low hemoglobin) they are. In general, serious infections are the top concern for infants, which is why we test for sickle cell disease at birth and start penicillin in the first few weeks of life. We also worry about the higher risk for stroke that children with sickle cell disease may have and therefore begin screening at 2 years of age. Other common symptoms as children get older are episodes of pain and acute chest syndrome, which is a complication that can include fever, cough and shortness of breath and may require a blood transfusion as treatment.


What therapies are available for sickle

cell disease?

Many of the therapies we have for sickle cell disease are supportive. However, medications such as penicillin and hydroxyurea have been instrumental in improving the care we provide for patients with sickle cell disease. Blood transfusions can manage some of the complications we see in sickle cell disease but need to be closely monitored, as too much iron can be absorbed. Stem cell transplant, the only proven cure we currently have for sickle cell disease, is an option for some patients but not all, as it requires a matched donor to provide the cells that are given to the patient. This requires an informed discussion with your hematologist and a stem cell team, such as the Stem Cell Program at Riley Hospital for Children. Further research needs to be done to increase available therapy options we have for patients with sickle cell disease, and Riley Hospital for Children’s Cancer and Blood Therapies Division is actively working to improve the care that is provided.


How can we support our children or friends who have sickle cell disease?

Children with sickle cell disease quite often live long, full lives. The use of therapies and regular doctor visits are important in managing the disease, but families can make a big impact by building healthy habits that become part of their child’s daily life. I always encourage a healthy diet, plenty of exercise, adequate sleep and good hydration. Sickle cell disease is a life-long disease that requires diligent treatment, but families and doctors can work together to help children feel their best.


What if I want to learn more and get involved?

As a nation, we have recognized the need to improve the care we provide to patients with sickle cell disease. The Comprehensive Pediatric Sickle Cell Program supports the efforts of the American Society of Hematology in “moving the needle” on sickle cell disease — calling to action researchers, physicians, patients, policymakers and others to spread the word on improving the state of sickle cell disease. Additionally, we are very fortunate in Indiana to have several community organizations focused on sickle cell disease. In Indianapolis, the Martin Center Sickle Cell Initiative has been a champion for patients and families with sickle cell disease and trait. We urge everyone to get involved and to become an advocate for patients with sickle cell disease and trait. If you want to learn more, you can contact the Comprehensive Pediatric Sickle Cell Program at Riley Hospital for Children by email or phone: SickleCell@iuhealth.org or (317) 948-1234. 


Dr. Seethal Jacob specializes in pediatric hematology and oncology with IU Health. 

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