8 Treatment Recommendations for People with Sickle Cell Anemia


Picking Up Medications
Picking Up Medications. Blend Images - Jose Luis Pelaez Inc/Brand X Pictures/Getty Images

Penicillin (an antibiotic) is used in children with sickle cell anemia to prevent serious bacteria infections (prophylaxis).  Learning that penicillin prophylaxis could prevent these serious life-threatening conditions, led to sickle cell anemia being added to the newborn screening program (sometimes called the PKU test).  Starting penicillin shortly after birth changed the life expectancy in sickle cell from childhood to adulthood.  Penicillin is given twice daily as soon as diagnosis is known until at least age 5.  Penicillin is continued if the spleen is removed by surgery or if the patient had a serious infection.  


Preparing Vaccine
Preparing Vaccine. Rafe Swan/Cultura/Getty Images

In addition to the immunizations all children receive, there are two additional immunizations recommended for children with sickle cell anemia.  These immunizations protect against bacteria that cause pneumonia (Pneumovax) and meningitis (infection of the lining of the brain).  Additionally, it is recommended children with sickle cell anemia be immunized against the flu as they are at increased risk of hospitalization and complications.  

Transcranial Doppler

Cerebral Arteries
Cerebral Arteries. BSIP/UIG/Getty Images

Transcranial Doppler (TCD) is an ultrasound of the blood vessels in the brain.  Studies have shown that children with increased flow in the blood vessels of the brain are at increased risk of stroke.  It is recommended that children with sickle cell anemia be screened with TCD annually from age 2 to 16.  If increased flow is noticed, monthly blood transfusions has been shown to prevent strokes.  This test is not recommended for children with milder forms of sickle cell disease like SC disease or sickle beta plus thalassemia.  


Urine Test
Urine Test. Steve Wisbauer/The Image Bank/Getty Images

It is recommended that children with sickle cell anemia be screened for kidney disease starting at age 10.  Urine is collected and screened for the presence of protein which may indicate damage to the kidneys.  If protein is detected, additional studies will performed to determine if kidney damage is present.   

Eye Exams

Close up of eye
Close up of eye. JGI/Blend Images/Getty Images

Children with sickle cell disease (all types) should have an annual eye exam starting at age 10.  This eye exam is not looking for changes in vision, but rather damage to the retina called retinopathy.  This exam requires the eyes to be dilated and should be performed by someone familiar with sickle cell retinopathy.  Detecting retinopathy early allows more treatment options.  

Evaluation for a Fever

Young man holding thermometer
Young man holding thermometer. Emma Innocenti/Stone/Getty Images

Because of the increased risk of bacterial infections, it is recommended that all people with sickle cell disease be evaluated for a fever of 101.3F or higher.  Blood counts should be assessed for worsening anemia or infections.  Additionally, blood should be sent to look for bacterial infection (blood culture). At least one dose of antibiotic is given via a vein (IV) or in the muscle.  Depending on labs and physical examination, some patients will be followed as an outpatient and some will be admitted to the hospital.   

Blood Transfusions

Donated blood
Donated blood. ERproductions Ltd/Blend Images/Getty Images

Blood transfusions are the recommended treatment for several complications of sickle cell disease.  This includes splenic sequestration (rapid enlargement of the spleen with severe anemia), acute chest syndrome (lung complication) and stroke.  Additionally, it is recommended that people who have had a stroke or are at high risk for stroke (abnormal TCD) receive blood transfusions monthly to prevent strokes.  Transfusions can also be used before surgeries to prevent complications.  


Close up of capsule
Close up of capsule. GP Kidd/Blend Images/Getty Images

Hydroxyurea is a medication used to reduce complications from sickle cell disease.  It is recommended that hydroxyurea treatment be offered to all infants with sickle cell anemia (specifically SS or sickle beta zero thalassemia) at 9 to 12 months of age before most complications occur.  In adults with sickle cell anemia, hydroxyurea has been proven to reduce the number of painful crises and acute chest syndrome episodes as well as reduce anemia.    

Continue Reading