Pediatric Sickle Cell Disease: Common Questions Answered

3D illustration Clumps of sickle cell block the blood vessel

New Brunswick, N.J., September 13, 2021 – The challenges for people and families living with sickle cell disease can be overwhelming. During this National Sickle Cell Awareness Month, Elizabeth Castro, APN, MSN, CCRN, pediatric advanced practice nurse at Rutgers Cancer Institute of New Jersey shares some information about the disease.

What is sickle cell disease?

Sickle cell disease is an inherited disorder of the red blood cells. It involves a defect in the hemoglobin protein which is found inside of the red blood cells. Hemoglobin is responsible for bringing oxygen from the lungs to the rest of the body. Normal red blood cells are shaped like a doughnut, they’re round and flexible which allows them to travel freely through the blood vessels. In patients with sickle cell disease, the red blood cells are rigid and shaped like a sickle or banana which prevents free travel through the blood vessels, instead there is a tendency for the red blood cells to clump together and get stuck. This blockage prevents oxygen from getting to where it needs to go which can lead to episodes of severe pain and tissue and organ damage.

How is sickle cell disease diagnosed?

In the United States, all newborn babies undergo screening tests within the first few days of life to detect serious, life-threatening diseases. A hemoglobin electrophoresis is a newborn screening blood test that can determine if the baby is a carrier of sickle cell trait or has sickle cell disease. Sickle cell disease also might be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta.

What are the biggest challenges for sickle cell patients?

Because sickle cell disease is a chronic lifelong condition, patients and families face many physical and psychosocial challenges throughout their lives. Depending on the severity of the disease, these challenges could include frequent disruptions in their daily lives. Patients may find it difficult to maintain a full time job or to attend school on a regular basis. These patients are at a high risk for stroke and other cardiac and pulmonary problems as well as being high risk for infection. Additionally, one of the most common complication of the disease is vaso-occlusive crisis (VOC), known as sickle cell crisis, which is characterized by episodes of severe pain.

What is the treatment plan for patients with sickle cell disease?

As the state’s primary referral site for sickle cell patients, Rutgers Cancer Institute as part of the Robert Wood Johnson Medical School Regional Comprehensive Sickle Cell Center is able to treat both pediatric and adult patients using well proven therapies and cutting edge clinical trials. Currently the only cure for sickle cell disease is a bone marrow transplant.  However, there are effective therapies that can minimize symptoms and prolong life.

Click for more information on Sickle Cell Disease.


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