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Sickle cell disease changes normal, round red
blood cells into cells that can be shaped like crescent moons. The name "sickle
cell" comes from the crescent shape of the cells. (A sickle is a tool with
a crescent-shaped blade.)
Normal red blood
cells move easily through your blood vessels, taking oxygen to every part of
your body. But sickled cells can get stuck and block blood vessels, which stops
the oxygen from getting through. That can cause a lot of pain. It can also harm
organs, muscles, and bones.
See a picture of
sickle cells blocking a blood vessel.
Having sickle cell disease
means a lifelong battle against the health problems it can cause, such as pain,
stroke. But many people are able to have a very good
quality of life by learning to manage the disease.
disease is inherited, which means it is passed from parent to child. To get
sickle cell disease, a child has to inherit two sickle cell
genes—one from each parent.
When a child
inherits the gene from just one parent, that child has
sickle cell trait. Having this trait means that you don't have the disease but you are a
carrier and could pass the gene on to your
Painful events (sickle cell crises) are the most common symptom of sickle cell disease. They
are periods of pain that happen when sickled cells get stuck in blood vessels
and block the blood flow. These events usually cause pain in the hands, feet,
belly, back, or chest. The pain may last for hours or for days.
People with sickle cell disease often have anemia, caused by a shortage
of red blood cells. Anemia makes you feel weak and tired. People with sickle
cell anemia may look pale or washed out. Their skin and the whites of their
eyes may have a yellowish look (jaundice).
A simple blood test can show whether a
person has sickle cell disease. Most states test for sickle cell disease before
infants go home from the hospital.
Self-care and medical treatment can help you manage pain and avoid other health problems.
Early treatment includes daily
antibiotics from 2 months to 5 years of age to help
prevent infections. Routine childhood
and adult immunizations are also important.
Managing pain is often a big part of having sickle cell disease. You can
prepare for a painful event ahead of time by creating a pain management plan
with your doctor. The plan should include what you can do at home to relieve
pain for yourself or your child. The plan should also tell you when it is best
to call a doctor or go to a hospital.
Some people need regular
blood transfusions to lower the risk of stroke and
treat anemia and other problems. Some people take medicine to prevent complications. In rare cases, a stem cell transplant might be an option.
Regular checkups are an important
part of life with this disease. People with sickle cell disease need a good
working relationship with a doctor who is an expert in treating it.
Learning about sickle cell disease:
Living with sickle cell disease:
Sickle cell disease is an inherited
blood disorder, passed from parent to child. Children with sickle cell disease
have two defective
hemoglobin S genes, one from each parent. Various forms of
sickle cell disorder occur when a person inherits one
hemoglobin S gene (sickle cell gene) from one parent and one other type of defective
hemoglobin gene from the other parent.
Normally, a person inherits two genes that tell the body to
hemoglobin A. One gene comes from each parent. People who inherit one defective hemoglobin S gene and one normal
hemoglobin A gene have
sickle cell trait. These people don't have symptoms of
sickle cell disease, and their bodies don't make sickled blood cells. But they can
pass the defective hemoglobin S gene to their children.
Painful events (sickle cell crises) in the hands or
feet, belly, back, or chest are the most common symptom of
sickle cell disease. This pain may last from hours to
days. Most people with sickle cell disease also get
When a child is born with
sickle cell disease, it isn't possible to predict which symptoms will appear,
when they will start, or how bad they will be.
Most people who have sickle cell disease
have at least mild symptoms of chronic anemia, which may include:
Severe anemia may raise the chance of a person with sickle
cell disease getting high blood pressure in the lungs (pulmonary hypertension). This can be deadly.
Painful sickle cell crisis symptoms are caused by blocked blood vessels in bones, organs, and other
tissues. This can cause extreme pain for hours or days. These
painful events can occur rarely to often. Sometimes
home treatment can help the pain. And sometimes a hospital stay is
Infants and young children may have episodes of
extreme pain in the hands, the feet, or both (hand-foot syndrome).
Normal red blood cells have a 120-day
life span. But people born with
sickle cell disease have
sickle-shaped blood cells that usually live no more
than 20 days. These sickled cells can get stuck in blood vessels, blocking blood flow.
Less blood flow can damage
the body's organs, muscles, and bones, sometimes leading to life-threatening
conditions. Sickle cell disease may cause problems such as:
When a child is born with sickle cell disease, it's
impossible to predict which problems will develop, when they will start, or how
bad they will be. During the first 6 months of life, infants have a high level
fetal hemoglobin (HbF) in their blood, which protects
them from red blood cell sickling. But dangerous complications of sickle cell
disease may quickly develop between ages 6 months and 5 years, after levels of
fetal hemoglobin decrease.
Older children and adults with sickle cell disease may have few problems. Or they may have a pattern of ongoing complications that shortens their lives. The most
common and serious problems caused by sickle cell disease are anemia, pain, and
organ failure. Stroke affects around 10 out of 100 children who have sickle cell
Other complications of sickle
cell disease include:
A child's risk of getting sickle cell disorder occurs when he or she inherits one
sickle cell gene and one other type of defective
People who inherit one defective hemoglobin S gene and
one normal hemoglobin A gene have
sickle cell trait. They don't have symptoms of sickle
cell disease, and their bodies don't make sickled blood cells. But they have a 1-out-of-2 (50%)
chance of passing the defective hemoglobin S gene to each of their
People whose ancestors were from Africa, India, the Middle
East, the Mediterranean (Turkey, Italy, Greece), and some Latin American
countries are more likely to inherit the gene that can cause sickle cell
disease. In the United States, about 2,000 children are born each year with sickle cell disease.2
For more information, see a picture of the risk
of passing on an
autosomal recessive disease such as sickle cell disease.
Call 911 or other emergency services immediately if you have
sickle cell disease and one or more of the following
symptoms are present:
Call your doctor if you or your child has any of the
Make a pain management plan with your doctor that includes where and when
to get treatment in case of a sickle cell emergency.
Painful events (crises) can be
treated at home depending on how severe the pain is
and how long you've had it. For more information, see Home Treatment.
If you or your child has sickle cell disease, try to
find a doctor who has special training for this disease. Some medical centers
and hospitals specialize in sickle cell disease treatment and support. If your
local community doesn't offer this option, look for a doctor and a pain
treatment specialist who have experience in treating
sickle cell disorders. Choose a doctor you are
comfortable with and can partner with over the long term.
following types of health professionals can diagnose and help treat symptoms of
sickle cell disease. Some of these health professionals may provide specialized
treatment or counseling.
To prepare for your appointment, see the topic Making the Most of Your Appointment.
Sickle cell disease is diagnosed when initial tests show abnormal
hemoglobin, with more testing if needed. A
sickle cell test looks for
sickle cell trait and sickle cell disease.
Doctors can diagnose sickle cell
disease before a child is born (prenatally). Couples who are at risk for
passing on this disease to their children may want to talk with a
genetic counselor about prenatal testing.
Prenatal tests include:
Sickle cell disease can be
diagnosed at birth. Most states in the United States screen all newborns for
sickle cell disease along with other common disorders. You can also ask for
Soon after birth, a sample of blood is taken from the
infant's heel and sent to a lab, where it is screened for the presence of
sickle cell hemoglobin (hemoglobin S).
If one member of a couple has
sickle cell disease or sickle cell trait, the other member should be tested
before becoming pregnant. This test requires a blood sample, which is screened
for the presence of hemoglobin S, hemoglobin C, or
If one or both members
of a couple carry a hemoglobin S
gene or another abnormal hemoglobin gene, the couple
may want to meet with a genetic counselor before becoming pregnant to learn
more about their chances of having a child with sickle cell disease. Your
doctor can help you find a genetic counselor to discuss a
Pulmonary hypertension is a severe, common problem for people who have sickle cell
disease. It can be detected early with an
echocardiogram, a painless method of measuring blood
Treatment involves getting routine tests to monitor health, managing pain events (crises), and treating related health problems as they arise.
for severe cases of
sickle cell disease may include medicines. For more information, see Medications.
When parents learn that their
sickle cell disease, it's the beginning of a lifelong
education process. Knowing as much as you can about the disease can help you
control symptoms as they arise and know what to do in emergency situations.
Starting at age 2 years, your child should get screened
every now and then with a
This test measures blood flow in the arteries of the head and neck. If test
results show a high chance for
stroke, your child may get
blood transfusions to lower the risk.1
Routine tests include:
Pain is sometimes a chronic problem for people with
sickle cell disease. Your doctor or a
pain treatment specialist can help you develop pain
management skills. These skills include distraction,
guided imagery, deep breathing,
relaxation, and positive self-talk.
Painful events can happen suddenly and unpredictably and can
become life-threatening. Bouts of severe pain can last for hours to days and
are difficult to treat. They're exhausting for caregivers as well as for the
person in pain. For more information, see the topic
Severe episodes of prolonged erection
of the penis (priapism) need evaluation by your doctor. Treatment
may include fluids (hydration), pain medicines, treatment by a
urologist, and blood transfusions.
There are also things you can do at home to manage pain. To learn more, see Home Treatment.
A series of blood
transfusions is the treatment of choice to prevent strokes and treat other
aspects of sickle cell disease. Stem cell transplant is a rare treatment. For more information, see Other Treatment.
People with sickle cell
disease should avoid contact with anyone suspected of having
fifth disease, which is caused by parvovirus.
Parvovirus can cause the body to temporarily stop making blood cells, a severe
life-threatening problem in someone who has sickle cell disease.
Aplastic anemia can occur as a result of a shortage
of red blood cells. It can come on suddenly and is life-threatening if not
People with sickle cell disease and their
families face ongoing
stress. For help coping, see Home Treatment.
Sickle cell disease is an inherited blood disorder that is not preventable.
Home treatment for
sickle cell disease includes steps to control pain and
prevent complications of the disease. If you don't already have a home
treatment plan, ask your doctor to help you make one. Use this plan whenever
symptoms are present. Your plan may include tips for:
You can help your child cope with special needs in school by:
Children with sickle cell disease can usually exercise
and play normally if they:
Folic acid supplements are often a necessary part of
the diet for people with sickle cell disease, particularly if you aren't eating
enough folate-rich leafy vegetables (such as spinach).
Medicines that treat
sickle cell disease include hydroxyurea and various pain medicines. Some of these medicines require a prescription. Others are
over the counter. Pain medicine may work best when
combined with pain
management skills, such as distraction; guided imagery; deep
breathing; relaxation; and positive, encouraging self-talk.
Pain treatment for sickle cell disease pain varies depending on
how bad the pain is and how long the pain lasts. Medicines that treat the pain include over-the-counter pain relievers (such as ibuprofen) and
opioids (such as codeine). Opiate pain medicines are used under careful medical supervision.
Some sickle cell complications are treated with surgery. Surgery options include:
Blood transfusions may be used for sickle cell disease. They are the treatment of choice to prevent strokes and treat other aspects of this disease. They can reduce the risk of some complications and improve symptoms of severe anemia.
Stem cell transplants can cure sickle cell disease. But they are not common.
The American Pain Society is a group of scientists,
clinicians, and others. This group seeks to advance pain-related research,
educate people about pain, and change public policy and clinical practice to
reduce pain-related suffering.
NCBDDD aims to find the cause of and prevent birth
defects and developmental disabilities. This agency works to help people of all
ages with disabilities live to the fullest. The website has information on
many topics, including genetics, autism, ADHD, fetal alcohol spectrum
disorders, diabetes and pregnancy, blood disorders, and hearing loss.
The March of Dimes tries to improve the health of babies
by preventing birth defects, premature birth, and early death. March of Dimes
supports research, community services, education, and advocacy to save babies'
lives. The organization's website has information on premature birth, birth
defects, birth defects testing, pregnancy, and prenatal care.
The U.S. National Heart, Lung, and Blood Institute
(NHLBI) information center offers information and publications about preventing
The Sickle Cell Disease Association of America is a national
membership organization that provides education, research updates, and support
around sickle cell disease problems. Its Web site has the Sickle Cell Disease
Forum for people of all ages to discuss concerns about sickle cell
Goldstein LB, et al. (2010). Guidelines for the primary prevention of stroke: A guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. Published online December 2, 2010 (doi: 10.1161/STR.0b013e3181fcb238). Also available online: http://stroke.ahajournals.org/content/42/2/517.full.
Wang WC (2009). Sickle cell anemia and other sickling syndromes. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 12th ed., pp. 1038–1082. Philadelphia: Lippincott Williams and Wilkins.
Other Works Consulted
American Academy of Pediatrics, et al. (2003, reaffirmed 2007). Policy statement: Eye examination in infants, children, and young adults by pediatricians. Pediatrics, 111(4): 902–907.
Brawley OW, et al. (2008). National Institutes of Health consensus development conference statement: Hydroxyurea treatment for sickle cell disease. Annals of Internal Medicine, 148(12): 932–938.
Committee on Genetics, American Academy of Pediatrics (2002, reaffirmed 2006). Health supervision for children with sickle cell disease. Pediatrics, 109(3): 526–535.
Natarajan K, et al. (2010). Disorders of hemoglobin structure: Sickle cell anemia and related abnormalities. In K Kaushansky et al., eds., Williams Hematology, 8th ed., pp. 709–741. New York: McGraw-Hill.
National Heart, Lung, and Blood Institute, National Institutes of Health (2002). The Management of Sickle Cell Disease (NIH Publication No. 02-2117). Available online: http://www.nhlbi.nih.gov/health/prof/blood/sickle/.
U.S. Preventive Services Task Force (2007). Screening for Sickle Cell Disease in Newborns. Available online: http://www.ahrq.gov/clinic/uspstf/uspshemo.htm.
October 1, 2012
E. Gregory Thompson, MD - Internal Medicine & Martin Steinberg, MD - Hematology
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