What is Sickle Cell Disease?

Published 11:26 am Tuesday, June 14, 2022

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What do Larenz Tate, Tiki Barber, Tionne “T-Boz” Watkins, Albert “Prodigy” Johnson, Georgeanna Tillman Gordon, and Jourdan Dunn have in common? They all have sickle cell disease.


Dr. Joe Stephenson, hematologist/oncologist and Director of St. Luke’s Cancer and Infusion Center says that “sickle cell disease is an inherited blood disease. The disease causes anemia, periods of pain, higher rates of infection, joint complications, and other disorders.” He also said that “SCD is caused by an HBB gene mutation, affecting the shape of hemoglobin in the red blood cells which transport oxygen to all parts of the body.”

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Worldwide, about 300,000 babies are born each year with SCD. And this number is expected to grow to 400,000 a year by 2050. It’s most common in people from sub-Saharan Africa, the middle east, and India. In the United States, an estimated 100,000 people are living with SCD. Furthermore, one in thirteen blacks have the sickle cell trait, and one in every sixteen thousand three hundred Hispanics have SCD.


The life expectancy of SCD patients is directly connected to economics. In India, for example, twenty percent of children with SCD will die by two. In fact, ninety percent of SCD patients in poor countries will not survive to adulthood. In the United States, however, most SCD patients live beyond fifty years of age. But here’s the double-edged sword; people with the sickle cell trait are ninety percent less likely to be affected by severe malaria.


SCD is usually diagnosed through a blood test. If the test results are positive, your provider will likely refer you to a hematologist. For babies, every state in the United States, the District of Columbia, and the U.S. territories require that all newborns be tested for sickle cell disease. For newborn screening, blood is usually drawn from a heel prick.


SCD patients are likely to experience:


  • fatigue
  • rapid heart rate
  • episodes of pain
  • swelling of the hands and feet
  • frequent infections
  • yellow skin and whites of the eyes
  • delayed growth or puberty
  • vision problems


Because blood isn’t moving through the body as it should, patients with SCD are likely to have other health issues. Complications from SCD include stroke, lung infections, pulmonary hypertension, organ damage, splenic sequestration, blindness, leg ulcers, gallstones, priapism, deep vein thrombosis, and complications with pregnancy.


“Current management of sickle cell anemia is usually directed at minimizing pain, relieving symptoms, and preventing further complications. Treatments include medications, blood transfusions, and stem cell transplants that may even cure the disease in young patients,” says Dr. Stephenson.


Treatment options include:


  • Drug therapy with meds like hydroxyurea, L-glutamine, and voxelotor improves SCD pain episodes.
  • Penicillin in children and some adults helps to minimize life-threatening infections.
  • Childhood vaccinations are even more critical for children and adults with SCD.
  • SCD patients need to be extra vigilant in taking precautions to avoid exposure to COVID.
  • Blood transfusions can prevent stroke and minimize other complications.
  • Stem cell transplant, because of the complex risks, is reserved for those with the most severe complications. However, it is the only known cure for SCD.


In addition to strictly following doctor’s orders, the following steps may help you avoid complications of SCD:


  • A daily supplement of folic acid and a healthy diet of colorful fruits, vegetables and whole grains help produce new red blood cells. 


  • Drink plenty of water to avoid a sickle cell crisis. Try to drink eight glasses of water a day.


  • Avoid extreme heat or cold temperatures, as they can increase your risk of a sickle cell crisis.


  • Abstain from over-the-counter medications. Ibuprofen or naproxen sodium can adversely affect your kidneys. 


  • Don’t smoke.


Suppose you or your spouse has the sickle cell trait. In that case, you are encouraged to meet with a genetic counselor before trying to conceive a child. The counselor can help you understand the risks of having a child with SCD. Moreover, you will learn about possible treatments, prevention, and reproductive options.


If you have a healthcare topic of interest or a question, send me a note at Michelle.Fortune@slhnc.org.