Sickle Cell Disease

Sickle cell disease can cause problems all over the body. Because most patients with sickle cell disease have anemia, they may have decreased energy. They can also develop jaundice and/or gallstones from the broken red blood cells.

Because sickle-shaped cells reduce the flow of blood through a person's blood vessels, patients may experience:

• Episodes of pain and/or infection
• Damage to organs (lungs, kidneys, liver, spleen)
• Damage to joints (usually hips and knees)
• Priapism
• Stroke, in more severe cases

It's important for your child to have regular appointments with his/her hematologist to monitor blood count and any current symptoms. If your child has an acute pain crisis, fever, or other concerning symptom, seek medical care immediately.

Around 100,000 people have sickle cell disease in the United States.

While a child of any race or nationality can be born with sickle cell, it occurs most often among African-Americans.

Sickle cell disease occurs in:

• Around 1 out of every 365 African-American births
• Around 1 out of every 16,300 Hispanic-American births

Testing for sickle cell disease is part of mandatory newborn screening in Illinois and across the United States.

Physicians use a blood test to confirm or rule out the presence of sickle cell disease or the sickle trait. Early diagnosis is key to the success of ongoing management of sickle cell.

Until recently, stem cell transplantation was the only cure for sickle cell disease.

However, new gene therapy treatments have been approved by the FDA and represent a major step forward for sickle cell treatment.

Treatment for sickle cell disease varies greatly and depends on the severity of symptoms. Ongoing treatment plans often involve:

• Antibiotics to prevent and/or treat infections
• Blood transfusions
• Pain relievers
• Practicing healthy habits like drinking lots of water, washing hands properly, and avoiding things that trigger sickling like smoking and exposure to cold temperatures

Depending on the severity of your symptoms, your doctor may recommend use of some of the following medicines:

• Hydroxyurea, which stimulates production of fetal hemoglobin and has been shown to decrease painful episodes.
• Crizanlizumab (Adakveo), which can help reduce pain crises.
• L-glutamine oral powder (Endari), which helps lessen pain and fatigue.
• Voxelotor (Oxbryta), which helps prevent red blood cells from sickling and return to a normal shape.