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Chest Syndrome

What is Chest Syndrome?

Chest syndrome is a common cause of hospitalization in children with sickle cell anemia. Chest syndrome is a term to describe the chest pain, fever, and "pneumonia-like" cough in a person with sickle cell disease. Many times, it is mistaken for pneumonia. This problem can be fatal in the child with sickle cell disease.


What Causes Chest Syndrome?

Although the reasons are not clearly understood, it is believed that "sickled cells" clump together in the small blood vessels either in the lungs or moves there from somewhere else in the body. Sometimes this is triggered by a lung infection like pneumonia. Chest syndrome can also develop right before, during, or after an episode of pain in the abdomen or bones. There are no methods available to separate pneumonia from chest syndrome caused by blocked vessels. That is why your child may be treated like they have both.


What are the Symptoms of Chest Syndrome?

  • Chest pain that may be severe
  • Fever of 101F or 38.5C or higher
  • Very congested cough
  • Trouble breathing
  • Fast breathing
  • You may see your child's ribs "suck in" when they breathe in

If you see any of these symptoms in your child, visit your doctor or clinic immediately!


Treatment

Your child will need to have blood drawn to check the complete blood count (CBC) and any infection in the blood. X-rays of the lungs will also be taken. 

Most children with chest syndrome are admitted to the hospital so that they can be watched closely. Pain medicine for the chest pain, oxygen, and IV fluids may be given to your child. Sometimes a blood transfusion is necessary. They will also take a strong antibiotic to fight any infection. 


Outcome

With proper therapy, children with chest syndrome usually do very well. However, some children have repeated episodes. The long-term effects of chest syndrome on lung function during adulthood is unknown. More research is needed.