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The Pain of Sickle Cell

Wednesday, May 17th, 2006
|CWK Executive Producer





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“People with sickle cell anemia have real pain, they need to be taken seriously and they need to be treated aggressively.

– Dr. Michael Ziegler, Children’s Healthcare of Atlanta






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It’s the most commonly inherited blood disorder in the United States.


Sickle cell anemia affects about one out of every 500 African-Americans. It can also affect some people of Arab, Indian and Mediterranean ancestry.


For parents of children with the disease, one of its most heartbreaking, common symptoms is pain.


Seven-year old Marcayla was brought into the ER after falling off her bicycle – but her pain is from sickle cell.


“If you were going to give the pain a number – you know the numbers? Ten’s the worst and zero’s no pain – what number would you give this pain?” asks Dr. Michael Ziegler of Children’s Healthcare of Atlanta.


“Ten,” Marcayla cries.


The pain from sickle cell comes from a genetic trait. Instead of normal round and flexible blood cells that move easily through the body, people with the disease have crescent or sickle-shaped cells, which impair blood flow.


“Their blood doesn’t flow through the small blood vessels as well as someone with normal red blood cells does,” says Dr. Ziegler, “and this can result in very significant, severe pain for them.”


A sickle cell pain crisis, as it is called, can be triggered by a lot of factors: a lack of fluids, exposure to cold or even stress. A pain crisis can last for hours, or even days.


“People with sickle cell anemia have real pain, they need to be taken seriously and they need to be treated aggressively,” says Dr. Ziegler.


Marcayla is given morphine and anti-inflammatory medicine for her pain, as well as intravenous fluids. Her pain is so severe, however, she has to be admitted to the hospital.


“In a situation like Marcayla’s,” says Dr. Ziegler, “where her pain is persisting and requiring some fairly strong medication, we want to have her in the hospital so we can continue aggressive care to get her healthy – but also to be able to watch her real close and make sure no other significant problems develop.”


More aggressive and earlier medical screening, combined with follow up treatments, has helped to greatly improve and extend the lives of people with sickle cell anemia.


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Tips for Parents

  • The symptoms of sickle cell disease can vary from person to person. Some symptoms include painful and swollen hands and/or feet, fatigue, paleness, shortness of breath, yellowing of the skin and eyes, eye problems and delayed growth in children. (American Sickle Cell Association)

  • Treatment for children with sickle cell can include taking penicillin daily to prevent infection – and folic acid supplements, to help the body produce new red blood cells. Kids with sickle cell can play and exercise, but should avoid getting overtired, overheated or too cold. (Nemours Foundation – Center for Children’s Health Media)

  • Some signs of an impending sickle cell pain crisis are thirst, fatigue, irritation and/or the eyes turning yellow. A person having a sickle cell crisis should receive medical treatment as soon as possible. (Sickle Cell Society)

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References

  • Sickle Cell Disease Association of America
  • Centers For Disease Control and Prevention
  • U.S. National Library of Medicine/ National Institutes of Health

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