TRAINING and EDUCATION
Patient Education
THE PEDIATRIC PATIENT LIVING
WITH SICKLE CELL DISEASE
Sickle cell disease (SCD) is the most common genetic disorder in the United States, with
about 100,000 American who have the disease and another 2 million who carry the trait.
As people live with SCD for years, management of this disease and healthy living are
important components of care, particularly for the pediatric population. Read below for more
information about the diagnosis, treatment, and prognosis of SCD in pediatric patients.
This “Patient Education” tear sheet was produced in collaboration with the Children’s
Sickle Cell Foundation, Inc. (cscfkids.org).
What Is Sickle Cell Disease?
How Is Sickle Cell Disease Diagnosed?
SCD is a group of inherited red blood cell disorders.
Normal red blood cells are soft, round, and able to move
through small blood vessels (veins and arteries) easily in
the body to deliver oxygen. A person with SCD has red
blood cells that, under certain conditions, become hard,
sticky, and pointed. When they go through the blood
tube, they clog the flow and break apart. This can cause
pain, organ damage, and a low blood count. SCD occurs
when a person inherits two abnormal hemoglobin genes
from his or her parents. A person is born with abnormal
“sickle hemoglobin,” and it is present for life.
Babies of all races should be screened for sickle cell hemoglobin at birth. A simple blood test, called the hemoglobin electrophoresis test, can be conducted to identify
if a child is a carrier of the sickle cell trait or has the
disease. Every state and the District of Columbia perform
the sickle cell test at birth to help get babies born with
SCD into care as soon as possible.
Other types of traits that may be identified on the test
results include:
What Is Sickle Cell Trait?
Abnormal hemoglobin is inherited from parents who
may be carriers of the sickle cell trait (SCT) or who have
SCD. If an individual only inherits one sickle gene, it is
SCT, while those who inherit two sickle cell genes have
SCD. SCT is not a mild form of SCD. People with SCT
usually do not have any of the symptoms of SCD and live
a normal life.
• hemoglobin S trait
• hemoglobin C trait
• hemoglobin E trait
• hemoglobin Barts, which indicates an alpha
thalassemia trait
• beta thalassemia trait
What Are the Complications Associated
With Sickle Cell Disease?
Complications from the sickled cells blocking blood flow
and breaking apart include:
• pain
• stroke
• increased risk of infection
• leg ulcers
• bone damage
• yellow eyes or jaundice
• early gallstones
• lung blockage
• kidney damage and loss of body water in urine
Cut out and give to a patient
• eye damage
• low red blood cell counts (anemia)
• delayed growth
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