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What is Sickle Cell Disease?
Sickle cell disease is an inherited disorder of the red blood cells. Red blood cells carry oxygen to all parts of the body by using
a protein called hemoglobin. Normal red blood cells contain only normal hemoglobin and are shaped like doughnuts. These cells
are very flexible and move easily through small blood vessels.
But in sickle cell disease, the red blood cells contain sickle hemoglobin, which causes them to change to a curved shape (sickle
shape) after oxygen is released. Sickled cells become stuck and form plugs in small blood vessels. This blockage of blood flow
can damage the tissue. Because there are blood vessels in all parts of the body, damage can occur anywhere in the body.
The most common types of sickle cell disease are:
Sickle cell anemia
Hemoglobin SC disease
Sickle beta-thalassemia
Types of Sickle Cell Disease
There are several forms of sickle cell disease. The most common is sickle cell anemia. Your doctor or nurse will tell you what
kind of sickle cell disease your baby has. Be sure to write down the name so that you can refer to it if your baby has to go to a
new doctor or clinic.
How Are Babies Affected?
Babies with sickle cell disease may have:
Anemia (a low number of red blood cells). People with anemia may tire easily.
Aplastic crisis. Babies with sickle cell disease may stop making red blood cells for a short time. Signs include paleness,
less activity than normal, fast breathing, and fast heartbeat. A baby with these signs must be seen quickly by the doctor.
Hand-and-foot syndrome. Babies with sickle cell disease may have pain and swelling in their hands or feet.
Painful episodes (mostly in the arms, hands, legs, feet, or abdomen). This happens when sickle cells plug blood vessels
and block the flow of blood. Doctors call this a painful episode, event, or crisis.
Severe infections. The child with sickle cell anemia is at great risk for serious infections--such as sepsis (a blood stream
infection), meningitis, and pneumonia. The risk of infection is increased because the spleen does not function normally.
Splenic sequestration crisis. The spleen is the organ that filters blood. In children with sickle cell disease, the spleen
can enlarge rapidly from trapped red blood cells. This condition is called splenic sequestration crisis and can be
life-threatening.
Stroke. This happens when blood vessels in the brain are blocked by sickled red blood cells. Signs include seizure,
weakness of the arms and legs, speech problems, and loss of consciousness. A baby with any of these signs must be
seen quickly by a doctor.
Who is Affected?
In the United States, most people who have sickle cell disease are African Americans. About 1 in 375 African-American
children has sickle cell disease. Hispanic Americans from the Caribbean, Central America, and parts of South America may
also have the disease. Sickle cell disease is also found in individuals from Turkey, Greece, Italy, the Middle East, or East India.
What Causes Sickle Cell Disease?
All forms of sickle cell disease are inherited. Children inherit genes for the disease from their parents.
Genes are substances within the father's sperm and the mother's egg that determine all of the physical characteristics of a baby.
Children inherit the genes for hemoglobin from their parents. Persons who inherit both normal and sickle hemoglobin have sickle
cell trait. Sickle cell trait is not a disease and does not change to disease. The individual sperm or egg from a person with sickle
cell trait may contain either a gene for normal hemoglobin or a gene for sickle hemoglobin.
When both parents have sickle cell trait, for each pregnancy, the chances are:
1 in 4 that the baby will have only normal hemoglobin.
2 in 4 that the baby will have both normal and sickle hemoglobin (sickle cell trait).
1 in 4 that the baby will have only sickle hemoglobin (sickle cell anemia).
The inheritance of other forms of sickle cell disease can be explained by your doctor.
How Do I Know if My Baby Has Sickle Cell Disease?
All newborn babies should be tested for sickle cell disease. Many states have screening programs that test babies born in the
hospital within a few days of birth. A blood sample is taken from the baby's heel for the sickle cell test, as well as screening tests
for several other medical conditions.
If the test shows your baby might have sickle cell disease, the doctor will do the test again to make sure. The doctor may ask
one or both parents for blood samples to test. If your baby has sickle cell disease, the doctor will tell you as soon as possible.
What if My Baby Has Sickle Cell Disease?
If your baby has sickle cell disease, the doctor will help you find the best medical care for your child. This care could be
provided by your family doctor, a pediatrician (children's doctor), or a pediatric hematologist (children's blood specialist), or a
special sickle cell clinic. You also may want to see a counselor who can talk with you about your chances of having another
baby with sickle cell disease.
Sickle cell disease is not just a medical problem. You may have many concerns about your baby and your family--for example,
how to cope with your feelings and how to pay the medical bills. Your doctor or nurse can talk with you about your concerns.
They also can help you find a local social service agency to assist you. In many areas there are sickle cell support groups, as
well as community organizations that offer testing, education, and support to families affected by sickle cell disease.
How Can I Help My Baby?
The best way to help your baby is to learn as much as you can about the disease and to make sure your baby gets the best
health care possible. The child with sickle cell disease has special needs and must have regular medical care to stay as healthy as
possible. The doctor or nurse will explain how often to bring your baby for medical care and what you can do if your baby
becomes ill.
By 2 months of age, your baby should start taking penicillin by mouth twice each day. It is very important to give the
medicine exactly as the doctor tells you. This will help prevent life-threatening infections. Penicillin should be continued until
at least 5 years of age.
Also by 2 months of age, your baby will get a shot to protect against H. influenzae, a type of bacteria that causes an infection
which can be dangerous to people with sickle cell disease. The baby also will need a shot to protect against hepatitis B, a liver
disease. At age 2, your child should receive pneumococcal vaccine. Your child should have all the other shots that children
normally receive.
Here are some of the most important things you need to know about caring for a baby with sickle cell disease:
If your baby has a fever (over 101 degrees), you must get medical help right away. A fever in a child with sickle
cell disease can be a sign of serious medical problems. Always take your baby's temperature when your baby appears
sick. Your doctor will tell you what to do if your baby has a fever.
If any new doctor or health care provider sees your baby for any reason, explain that your baby has sickle cell disease.
A good diet is very important for all babies. Ask your doctor or other health care provider about the right foods and
liquids for your baby. Make sure your baby drinks plenty of liquids. Find out if your baby also should have vitamins or
iron.
Make sure your baby does not become overheated or chilly. Keep your baby warm. Cold baths or cold air can slow the
baby's blood flow and cause problems.
If your baby is sick, you must get medical help right away.
Any sign of illness in a child with sickle cell disease can be serious. Your baby needs to see the doctor quickly if the baby:
Is breathing fast or having a problem with breathing
Coughs frequently
Is cranky and cries more than usually
Screams when touched
Is very tired or has little energy
Is very weak
Vomits
Does not want to eat
Has diarrhea
Has fewer wet diapers
Has pain or swelling in the abdomen
Has swollen hands or feet
Has pale blue or grey lips or skin
Questions to Ask :
You should always feel free to ask any questions about sickle cell disease and how it affects you and your family. Here are
some questions you may want to ask the doctor, nurse, counselor, or social worker.
What does my baby have? How did he or she get it?
What do I have? How did I get it? How will it affect me and my family?
How often does my baby need to see you?
What medicine does my baby need? What do I need to know about giving it?
What should my baby eat and drink?
Is there anything my baby should not do?
How can I tell if my baby gets sick?
What should I do, and who should I call, if my baby gets sick?
What other help is available to my family?
Your doctor, nurse, or other caregiver may use these terms in talking with you about sickle cell disease and your child.
Acute chest syndrome.
A serious condition caused by infection or trapped red blood cells in the lungs. Fast or difficult breathing, chest pain, and
coughing are signs of acute chest syndrome in the child with sickle cell disease. A child with acute chest syndrome usually
will have to go to the hospital for treatment.
Anemia.
A reduced number of red blood cells. Anemia occurs in persons with sickle cell disease because sickled red blood cells
do not live as long as normal red blood cells. A child with sickle cell disease cannot make red blood cells fast enough to
keep up with the rapid breakdown, so the person with sickle cell disease has fewer red blood cells than normal and is
anemic
.
Aplastic crisis.
Occurs when a child's bone marrow temporarily stops producing red blood cells. A child with aplastic crisis may appear
pale and be tired and less active than usual.
Capillaries.
Tiny blood vessels where sickle-shaped blood cells may get trapped and cause problems.
Gene.
The biological units that are passed from both parents to a child. Genes determine all of the child's characteristics--for
example, hair, eye, and skin color, foot size, height--and whether the child will have sickle cell disease or another
inherited disease.
Haemophilus influenzae.
A type of bacteria that causes infection and can lead to serious problems in the child with sickle cell disease. Babies must
receive a special vaccine beginning at 2 months of age to protect them from this condition.
Hand-and-foot syndrome
Pain and swelling of the hands and feet caused by sickle-shaped red blood cells that plug blood vessels in the hands and
feet. Often this will be the baby's first problem caused by sickle cell disease
.
Hemoglobin.
A molecule found in red blood cells that carries oxygen from the lungs to other parts of the body.
Pain event or painful episode.
Pain caused by plugging of blood vessels by sickled blood cells. Pain is most often felt in the arms, legs, back, and
abdomen. The pain may last only a few hours or as long as a week or two. The pain may be mild or so severe that pain
medicine is needed. The number of pain events a person has may vary greatly.
Sepsis.
The presence of infection in the blood stream.
Sickle cell anemia.
The most common form of sickle cell disease. Other types of sickle cell disease include hemoglobin SC disease and
sickle beta-thalassemia; there are also other, less common types of sickle cell disease.
Sickle cell disease.
A group of inherited disorders in which anemia is present and sickle hemoglobin is produced.
Sickle cell trait.
The condition in which a person has both normal and sickle hemoglobin in the red cells as a result of inheriting a normal
hemoglobin gene and a gene for sickle hemoglobin. Sickle cell trait is not a disease and does not change to sickle cell
disease. Persons with sickle cell trait may pass the sickle gene to their children.
Sickled cells.
In children with sickle cell disease, hemoglobin molecules in red blood cells stick to one another and cause the red cells
to become crescent or sickle shaped. Sickled cells cannot pass easily through tiny blood vessels.
Splenic sequestration crisis.
Occurs when a large portion of the child's blood becomes trapped in the spleen. Early signs include paleness, an enlarged
spleen, and pain in the abdomen.
Streptococcus pneumoniae.
A bacteria that causes a very serious type of pneumonia in children with sickle cell disease. Twice daily doses of penicillin
by mouth, starting at about 2 months of age, can help to prevent this life-threatening infection in children with sickle cell
anemia and sickle beta-thalassemia.
For More Information
The information in this section was taken from the Clinical Practice Guideline on Sickle Cell Disease: Screening,
Diagnosis, Management, and Counseling in Newborns and Infants. The guideline was written by a panel of experts
sponsored by the Agency for Health Care Policy and Research. Other guidelines on common health problems also are being
developed.
For more information about guidelines, or to order extra copies of this booklet, contact:
Agency for Health Care Policy and Research
Publications Clearinghouse
PO Box 8547
Silver Spring, MD 20907
Or call 1-800-358-9295 (for callers outside the US, only: 301-495-3453) weekdays, 9 am to 5 pm, Eastern time.
Document Source:
Facts About Sickle Cell Anemia: An INH Publication Online
U.S. Department of Health and Human Services, Public Health Service
Agency for Health Care Policy and Research
Executive Office Center, Suite 501, 2101 East Jefferson Street, Rockville, MD 20852
AHCPR Publication No. AHCPR 93-0564
April 1993
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