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Sickle cell disease

About sickle cell disease in children

Sickle cell disease is an inherited disorder of the red blood cells. For a child to have sickle cell disease both parents must carry the sickle cell trait. (or another variant hemoglobin (like C trait, thalassemia trait, etc)

It is the most common inherited blood disorder in the United States and most commonly impacts individuals of African and Mediterranean descent.

In the United States, sickle cell disease affects:

  • 1 in approximately 360 African-American newborns
  • 1 in approximately 16,300 Hispanic-American newborns

What are the types of sickle cell?

There are multiple types of sickle cell disease; they differ in severity and complications. The four most common are:

  • Homozygous sickle cell (Hemoglobin SS disease)
  • Hemoglobin SC disease
  • Sickle beta thalassemia zero
  • Sickle beta thalassemia plus

What are the symptoms of sickle cell?

Sickle cell is a disorder of red blood cells. The red blood cells’ main job is to carry oxygen to all  parts of the human body. If you have sickle cell, your red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle.”

The sickle cells die early, which causes a shortage of red blood cells. Since every bit of us needs oxygen to function well, sickle cell can impact any part of the body.

Common symptoms include:

  • Pain, which is the hallmark of the disease
  • Anemia (Which can cause fatigue, paleness, yellowing of the skin and eyes (jaundice), or shortness of breath.)
  • Infections
  • Lung complications such as asthma
  • Stroke
  • Splenic sequestration (Sickled red blood cells can become trapped in the spleen. The spleen becomes larger and is frequently painful.)
  • Swelling of hands and feet

Our sickle cell program partners with specialists across CHoR to make sure our patients have comprehensive care to treat the disease and all of its impacts on the body.   

Causes of sickle cell

Sickle cell is a genetic condition that is inherited when a child receives two sickle cell genes.

People who make hemoglobin S in their red blood cells have inherited the sickle cell gene from one or both parents. Children who inherit the sickle cell gene from both parents will have sickle cell disease (SS).

The sickle cell gene tells the body to make abnormal hemoglobin.

  • A patient who has Homozygous sickle cell (Hemoglobin SS disease) has inherited a two sickle cell gene (S), one from each parent.
  • People with any other type of sickle cell disease have inherited the S gene from one parent and another abnormal hemoglobin gene from the other parent.

Children who inherit the sickle cell (S) gene from only one parent and the normal genes for hemoglobin A from the other parent, will have the sickle cell trait (AS) but not the disease. They generally have no symptoms of the disease and lead normal lives but can pass the gene on to their children.

When two people with the sickle cell trait have a baby, they can have a child with normal hemoglobins (AA), sickle cell trait (AS) or the SS type of sickle cell disease.

How is sickle cell anemia diagnosed?

Virginia’s standard newborn screening tests all newborns for sickle cell disease.  

Virginia has a network of comprehensive sickle cell centers, with a goal to have every newborn who is diagnosed establish care within the network by 2 months of age.

There are several tests that help determine what type of hemoglobin is made by a person's red blood cells. These include:

  • Hemoglobin electrophoresis
  • Isoelectric focusing
  • Chromatography

How do our expert hematologists treat sickle cell?

Our sickle cell program follows patients from birth until high school graduation. We work closely with patients and families to ensure a smooth transition to VCU Health’s adult sickle cell program, the only comprehensive program of its kind in Virginia. 

Learn more about our sickle cell care