What Is Sickle Cell Anemia?
Sickle cell anemia is a serious condition in which
the red blood cells can become sickle-shaped (that is, shaped like a
“C”).
Normal red blood cells are smooth and round like a
doughnut without a hole. They move easily through blood vessels to carry oxygen
to all parts of the body. Sickle-shaped cells don’t move easily through
blood. They’re stiff and sticky and tend to form clumps and get stuck in
blood vessels.
The clumps of sickle cells block blood flow in the
blood vessels that lead to the limbs and organs. Blocked blood vessels can
cause pain, serious infections, and organ damage.
Normal and Sickled Red Blood Cells
in Blood Vessels
Figure A shows normal
red blood cells flowing freely in a blood vessel. The inset image shows a
cross-section of a normal red blood cell with normal hemoglobin. Figure B shows
abnormal, sickled red blood cells clumping and blocking the blood flow in a
blood vessel. The inset image shows a cross-section of a sickled red blood cell
with abnormal strands of hemoglobin.
Sickle cell anemia is an inherited, lifelong
condition. People who have sickle cell anemia are born with it. They inherit
two copies of the sickle cell gene, one from each parent. People who inherit a
sickle cell gene from one parent and a normal gene from the other parent have a
condition called sickle cell trait.
Sickle cell trait is different from sickle cell
anemia. People with sickle cell trait don’t have the condition, but they
have one of the genes that cause the condition. Like people with sickle cell
anemia, people with sickle cell trait can pass the gene on when they have
children. To learn more about sickle cell trait, see the section on
causes of sickle cell anemia.
Anemia
Anemia (uh-NEE-me-uh) is a condition in which a person’s blood has a
lower than normal number of red blood cells, or the red blood cells don’t
have enough hemoglobin (HEE-muh-glow-bin). Hemoglobin is an iron-rich protein
that gives blood its red color and carries oxygen from the lungs to the rest of
the body.
Red blood cells are made in the spongy marrow inside
the large bones of the body. Bone marrow constantly makes new red blood cells
to replace old ones. Normal red blood cells last about 120 days in the
bloodstream and then die. Their main role is to carry oxygen, but they also
remove carbon dioxide (a waste product) from cells and carry it to the lungs to
be exhaled.
In sickle cell anemia, a lower-than-normal number
of red blood cells occurs because sickle cells don’t last very long.
Sickle cells die faster than normal red blood cells, usually after only about
10 to 20 days. The bone marrow can’t make new red blood cells fast enough
to replace the dying ones. The result is anemia.
Outlook
Sickle cell anemia affects millions of people
worldwide. There are excellent treatments for the symptoms and complications of
the condition, but in most cases there’s no cure. (Some researchers
believe that bone marrow transplants may offer a cure in a small number of
cases.)
Over the past 30 years, doctors have learned a
great deal about the condition. They know what causes it, how it affects the
body, and how to treat many of the complications. Today, with good health care,
many people with the condition live close to normal lives and are in fairly
good health much of the time. These people can live into their forties or
fifties, or longer.
Other Names for Sickle Cell Anemia
- Hemoglobin SS disease
- Hemoglobin S disease
- HbS disease
- Sickle cell disorders
- Sickling disorder due to hemoglobin S
- SCD
What Causes Sickle Cell Anemia?
Sickle cell anemia is an inherited condition.
People with sickle cell anemia inherit two copies of the sickle cell gene, one
from each parent.
The sickle cell gene makes abnormal hemoglobin.
Hemoglobin is the protein inside red blood cells that carries oxygen to all
parts of the body and gives blood its red color.
In sickle cell anemia, the abnormal hemoglobin
sticks together when it gives up its oxygen to the tissues. These clumps are
like liquid crystals that cause red blood cells to become stiff and shaped like
a sickle, or “C.” It takes two copies of the sickle cell gene for the
body to make the abnormal hemoglobin found in sickle cell anemia.
Sickle Cell Trait
People who inherit only one copy of the sickle cell
gene (from one parent) will not have sickle cell anemia. They will have sickle
cell trait.
People who have sickle cell trait generally have no
symptoms and lead normal lives. Like people with sickle cell anemia, however,
they can pass the sickle cell gene on to their children. The following image
shows how two parents with sickle cell trait pass along the sickle cell gene.
Inheritance Pattern for Sickle Cell Trait
The illustration shows how sickle
cell genes are inherited. A person inherits two copies of the hemoglobin
gene—one from each parent. A normal gene will produce normal hemoglobin
(A). An abnormal (sickle cell) gene will produce abnormal hemoglobin (S). When
each parent has a normal gene and an abnormal gene, each child has: a 25
percent chance of inheriting two normal genes; a 50 percent chance of
inheriting one normal gene and one abnormal gene; and a 25 percent chance of
inheriting two abnormal genes.
Who Is At Risk for Sickle Cell Anemia?
Sickle cell anemia affects millions of people
worldwide. It’s most common in people whose families come from Africa,
South or Central America (especially Panama), Caribbean islands, Mediterranean
countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
In the United States, sickle cell anemia affects
about 70,000 people. It mainly affects African Americans, with the condition
occurring in about 1 in every 500 African American births. Hispanic Americans
also are affected; the condition occurs in 1 out of every 1,000 to 1,400
Hispanic American births.
About 2 million Americans have sickle cell trait.
About 1 in 12 African Americans has sickle cell trait.
What Are the Signs and Symptoms of Sickle Cell
Anemia?
The signs and symptoms of sickle cell anemia are
different in each person. Some people have mild symptoms. Others have very
severe symptoms and are often hospitalized for treatment. Although sickle cell
anemia is present at birth, many infants don’t show any signs until after
4 months of age.
The most common signs and symptoms are linked to
anemia and pain. Other signs and symptoms are linked to some
of the complications of the condition.
Anemia
The general signs and symptoms of anemia are
fatigue (tiredness), pale skin and nail beds, jaundice (yellowing of the skin
and eyes), and shortness of breath.
Pain (Sickle Cell Crisis)
Sudden episodes of pain throughout the body are a
common symptom of sickle cell anemia and are often referred to as “sickle
cell crises.”
A sickle cell crisis occurs when the red blood
cells sickle (become “C” shaped) and stick together in clumps. The
clumps block the flow of blood through the small blood vessels (capillaries) in
the limbs and organs.
Sickle crises can cause acute or chronic pain.
Acute pain is the most common type. This is sudden pain that can range from
mild to very severe. The pain usually lasts from hours to a few days. Chronic
pain usually lasts for weeks to months. Chronic pain can be hard to bear and
mentally draining. This pain may severely limit daily activities.
Almost all people with sickle cell anemia have
painful crises at some point in their lives. Some have a crisis less than once
a year. Others may have 15 or more crises in a year.
Many factors can contribute to a sickle cell
crisis. Often, more than one factor is involved and the exact cause can’t
be identified. Factors that occur in your body and aren’t under your
control can cause a sickle cell crisis, such as an infection.
Factors that you can control also can affect
whether you have a sickle cell crisis. For example, dehydration (when your body
doesn’t have enough fluid) can increase your chances of having a sickle
cell crisis. Drinking plenty of fluids so your body is hydrated can often help
decrease the chance of a crisis.
The most common sites affected by sickle cell
crises are the bones, lungs, abdomen, and joints. The blocked blood flow can
cause pain and organ damage.
Complications of Sickle Cell Anemia
Complications of sickle cell anemia come from the
effects of sickle cell crises on different parts of the body.
Hand-Foot Syndrome
When sickle cells block the small blood vessels in
the hands or feet, pain and swelling along with fever can occur. One or both
hands and/or feet may be affected at the same time. Pain may be felt in the
many bones of the hands and feet. Swelling usually occurs on the back of the
hands and feet and moves into the fingers and toes. This may be the first sign
of sickle cell anemia in infants.
Splenic (Sequestration) Crisis
The spleen is an organ in the abdomen that filters
out abnormal red blood cells and helps fight infection. Sometimes, the spleen
traps many cells that should be in the bloodstream and it grows large. This
causes anemia. Blood transfusions may be needed until the body can make more
cells and recover. If the spleen becomes too clogged with sickle cells, it
can’t work normally. It begins to shrink and stop working.
Infections
Both children and adults with sickle cell anemia
have a hard time fighting infections. Sickle cell anemia can damage the spleen.
Infants and young children with a damaged spleen
are more likely to get infections that can kill them within hours or days.
Pneumonia is the most common cause of death in young children who have sickle
cell anemia. Meningitis, influenza, and hepatitis are other infections that are
common in people with sickle cell anemia.
Acute Chest Syndrome
Acute chest syndrome is a life-threatening
condition linked to sickle cell anemia. It's similar to pneumonia and is caused
by an infection or by sickle cells trapped in the lungs. People with this
condition usually have chest pain, fever, and an abnormal chest x ray. Over
time, lung damage may lead to
pulmonary arterial hypertension (see below).
Delayed Growth and Puberty in Children
Children with sickle cell anemia often grow more
slowly and reach puberty later than other children. A shortage of red blood
cells (anemia) causes the slow growth rate. Adults with sickle cell anemia
often are slender or small in size.
Stroke
Sickle-shaped red blood cells may stick to the
walls of the tiny blood vessels in the brain. This can cause a stroke. This
type of stroke occurs mainly in children. The stroke can cause learning
disabilities or more severe problems.
Eye Problems
The retina, a thin layer of tissue at the back of
the eye, takes the images you see and sends them to your brain. When the retina
doesn’t get enough blood, it can weaken. A weak retina can cause serious
problems, including blindness.
Priapism
Males with sickle cell anemia may have painful and
unwanted erections called priapism (PRI-a-pizm). This happens because the
sickle cells stop blood flow out of an erect penis. Over time, priapism can
damage the penis and lead to impotence.
Gallstones
When red blood cells die, they release their
hemoglobin, which the body breaks down into a compound called bilirubin. When
there is too much bilirubin in the body, stones can form in the gallbladder.
Gallstones can cause steady pain that lasts for 30
minutes or more in the upper right side of the belly, under the right shoulder,
or between the shoulder blades. The pain may happen after eating fatty meals.
People with gallstones may have nausea, vomiting, fever, sweating, chills,
clay-colored stool, or jaundice (yellowish color of the skin or whites of the
eyes).
Ulcers on the Legs
Sickle cell ulcers (sores) usually begin as small,
raised, crusted sores on the lower third of the leg. Leg sores occur more often
in males than in females and usually appear between the ages of 10 and 50. The
cause of leg ulcers is not clear. The number of ulcers can vary from one to
many. Some heal rapidly, but others persist for years or come back after
healing.
Pulmonary Arterial Hypertension (High Blood
Pressure)
Damage to the small blood vessels in the lungs
makes it hard for the heart to pump blood through the lungs. This causes blood
pressure in the lungs to increase. This condition is called pulmonary arterial
hypertension. Excessive shortness of breath is an important symptom linked to
this problem.
Multiple Organ Failure
Multiple organ failure is rare, but serious. It
happens when a person has a sickle cell crisis that causes two out of three
major organs (lungs, liver, or kidney) to fail. Symptoms linked to this
complication are a fever and changes in mental status such as sudden tiredness
and loss of interest in your surroundings.
How Is Sickle Cell Anemia Diagnosed?
Early diagnosis of sickle cell anemia is very
important so that children who have the condition can get proper treatment.
In the United States, 44 States, the District of
Columbia, Puerto Rico, and the U.S. Virgin Islands now test all newborns for
sickle cell anemia. In the other six States, you can request a sickle cell
test.
The test uses blood from the same blood samples
used for other routine newborn screening tests. It can show whether the newborn
infant has sickle cell anemia or sickle cell trait. If the first test shows
some sickle hemoglobin, a second blood test is done to confirm the diagnosis.
The second blood test looks at how hemoglobin moves
in an electric field, a process called electrophoresis (ee-LEK-tro-for-EE-sis).
Sickle hemoglobin moves differently than normal hemoglobin. Electrophoresis is
usually used to diagnose older children and adults.
It’s also possible to identify sickle cell
anemia before birth. This is done using a sample of amniotic fluid or tissue
taken from the placenta. (Amniotic fluid is the fluid in the sac surrounding a
growing embryo. The placenta is the organ that attaches the umbilical cord to
the mother’s womb.) This test can be done as early as the first few months
of pregnancy. It identifies the sickle gene, rather than the hemoglobin it
makes.
How Is Sickle Cell Anemia Treated?
Effective treatments are available to help relieve
the symptoms and complications of sickle cell anemia, but in most cases
there’s no cure. Some researchers believe that bone marrow transplants may
offer a cure in a small number of cases. Researchers are looking for new
treatments for sickle cell anemia, including gene therapy and safer and more
effective bone marrow transplants.
People who have sickle cell anemia need regular
medical care. Some doctors and clinics specialize in treating people with the
condition. Doctors specializing in sickle cell anemia are often hematologists
(doctors who treat people with blood disorders)—or pediatric hematologists
(if they also treat children).
Goals of Treatment
The goals of treating sickle cell anemia are to
relieve pain; prevent infections, eye damage, and strokes; and control
complications if they occur. The treatments include medicine, blood
transfusions, and specific treatment for complications.
Treating Pain
Mild painful crises can be managed with treatments
such as over-the-counter medicine and heating pads. However, severe pain may
need to be treated in a hospital. Painful crises are the leading cause of
emergency room visits and hospitalizations of people with sickle cell anemia.
The usual treatments for acute (short-term) pain
crises are pain-killing medicines and fluids, given either by mouth or through
a vein, to prevent dehydration (a condition in which your body doesn’t
have enough fluids). The pain-killing medicines most often used are
acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics
such as meperidine, morphine, oxycodone, and others.
The treatment of patients who have mild-to-moderate
pain usually begins with NSAIDs or acetaminophen. If pain continues, a narcotic
may be added. Moderate-to-severe pain is often treated with narcotics. The
narcotic may be used alone or together with NSAIDs or acetaminophen. Narcotic
abuse and addiction are pain management issues that must be considered in any
pain control plan.
A medicine called hydroxyurea may be given to
adults and older adolescents with severe sickle cell anemia to reduce their
number of painful crises. This medicine is used only to prevent these crises,
not to treat them when they occur. Given daily, hydroxyurea can reduce the
frequency of painful crises and of acute chest syndrome. People taking the
medicine also may need fewer blood transfusions.
People taking hydroxyurea must be watched carefully
because the medicine can cause serious side effects, including an increased
risk of dangerous infections. Some evidence suggests that long-term use of
hydroxyurea can cause tumors or leukemia. Because of these risks, the medicine
is usually only used in adults and older teenagers with severe sickle cell
anemia. Although hydroxyurea is being tested in infants and children at this
time, it won’t be approved for use in children until its long-term effects
can be more closely studied.
Preventing Infections
Infection is a major complication of sickle cell
anemia. In fact, pneumonia is the leading cause of death in children with the
condition. Other infections common in people with sickle cell anemia include
meningitis, influenza, and hepatitis. If a child with sickle cell anemia shows
early signs of an infection, such as fever, seek treatment right away.
To prevent infections in babies and young children,
treatments include:
- Daily doses of penicillin. Treatment may begin as
early as 2 months of age and continue until the child is at least 5 years old.
- Vaccinations for pneumonia, meningitis,
influenza, and hepatitis.
- A yearly flu shot.
Adults who have sickle cell anemia also should have
flu shots every year and be vaccinated for pneumonia.
Preventing Eye Damage
Sickle cell anemia can damage the blood vessels in
the eyes. Parents should ask their child’s doctor about regular checkups
with an eye doctor who specializes in diseases of the retina. The retina is a
thin layer of tissue inside the back of the eye. Adults with sickle cell anemia
also should have regular checkups with an eye doctor.
Preventing Strokes
Stroke prevention and treatment is now possible for
children and adults who have sickle cell anemia. Starting at age 2, children
with sickle cell anemia often receive regular ultrasound scans of the head
(this is called transcranial Doppler ultrasound). These scans are used to
monitor blood flow in the brain.
The scans allow doctors to find out which children
are at high risk for a stroke and treat them with regular blood transfusions.
Routine blood transfusions have been found to greatly reduce the number of
strokes in children.
Blood Transfusions
Blood transfusions are used to treat worsening
anemia and sickle cell complications. A sudden worsening of
anemia due to an infection or enlargement of the spleen is a common reason for
a blood transfusion. Some, but not all, patients need transfusions to prevent
life-threatening events such as stroke or pneumonia.
Regular blood transfusions do have side effects,
and patients must be carefully watched. Side effects can include a dangerous
buildup of iron in the blood (which must be treated) as well as an increased
risk of infection from the transfused blood.
Treating Other Complications
Acute chest syndrome is a severe and
life-threatening complication in children and adults who have sickle cell
anemia. Treatment usually requires hospitalization and may include oxygen,
blood transfusions, antibiotics, pain medicine, and monitoring the body’s
fluids.
Leg ulcers can be painful, and patients may be
given strong pain medicines. Ulcers can be treated with cleansing solutions and
medicated creams or ointments. Skin grafts may be needed if the condition
continues. Bed rest and keeping the legs raised to reduce swelling are helpful,
although not always possible.
Gallbladder surgery may be needed if the presence
of gallstones leads to gallbladder disease.
Priapism can be treated with fluids or surgery.
Regular Health Care for Children
Children with sickle cell anemia should get regular
health care, just like children without the condition. They need to have their
growth checked and to get the usual shots that all children receive.
It’s recommended that before age 2, children
with sickle cell anemia see the doctor often (sometimes every 2 or 3 months).
After age 2, children may need to see the doctor less often, but usually at
least every 6 months. These visits are a time for parents to talk with their
child’s doctor and ask questions about the child’s care. Parents
should ask about eye checkups and the need for an ultrasound scan of the brain.
Until age 5, daily penicillin is given to most
children who have sickle cell anemia. Many patients are prescribed a vitamin
called folic acid (folate) to help prevent some of the complications of sickle
cell anemia.
New Treatments
Today, research on sickle cell anemia is looking at
bone marrow transplants, gene therapy, and new medicines. The hope is that
these studies will provide better treatments for sickle cell anemia.
Researchers also are looking for a way to predict the severity of the
condition.
Bone Marrow Transplant
Bone marrow transplant can be a very effective
treatment for sickle cell anemia, but because of its risks, only some patients
can or should have this procedure.
The bone marrow transplant procedure is risky and
could result in serious side effects and even death. It’s usually used
only for younger patients with severe sickle cell anemia, but the decision is
made on a case-by-case basis.
Bone marrow used for a transplant must come from a
closely matched donor, usually a close family member, who doesn’t have
sickle cell anemia.
Gene Therapy
Gene therapy is being studied as a possible
treatment for sickle cell anemia. Researchers are looking to see whether a
normal gene can be planted in the bone marrow of a person with sickle cell
anemia, and thus cause the body to produce normal red blood cells. Researchers
also are studying the possibility of treatment to “turn off” the
sickle cell gene or “turn on” a gene that makes red blood cells
behave normally.
New Medicines
New medicines being studied are:
- Butyric acid. This is a food additive that may
increase normal hemoglobin in the blood.
- Clotrimazole. This is used now to treat fungus
infections. This medicine helps prevent the loss of water from a red blood cell
and can keep the cell from turning into a sickle cell.
- Nitric oxide. This may make sickle cells less
sticky and keep blood vessels open. People with sickle cell anemia have low
levels of nitric acid in their blood.
How Can Sickle Cell Anemia Be Prevented?
Sickle cell anemia is an inherited condition.
Children who inherit the genes for sickle cell anemia can’t prevent
developing the condition, although they can take
steps to reduce complications.
People who are in groups that are at high risk for
sickle cell anemia and who are planning to have children may want to consider
genetic counseling beforehand. A counselor can help you understand your risk of
having a child with the condition and help explain the choices that are open to
you. You can get information about genetic counseling from health departments,
neighborhood health centers, medical centers, and clinics that care for people
with sickle cell anemia.
A procedure called pre-implantation genetic
diagnosis can improve the chance that two people with sickle cell trait will
have a child with normal hemoglobin. Eggs from the mother and sperm from the
father are mixed together in a laboratory dish. The eggs that become fertilized
are checked for sickle cell anemia. Fertilized eggs that don’t have sickle
cell genes are then implanted in the mother’s womb. This is a complex
procedure done only in a few places, and it doesn’t always work.
Living With Sickle Cell Anemia
With good health care, many people with sickle cell
anemia can live productive lives, have reasonably good health much of the time,
and live longer today than in the past. Many people who have sickle cell anemia
now live into their forties or fifties, or longer.
If you have sickle cell anemia, it’s important
to take good care of yourself, do what you can to prevent sickle cell crises,
and get regular medical care. Find out all you can about your condition and
learn what signs to watch out for.
To take care of your health, you should maintain
healthy lifestyle habits. These include:
- Eating healthy. Your doctor also may recommend a
vitamin (folic acid) to take every day to help your body make new red blood
cells.
- Drinking at least 8 glasses of water every day,
especially in warm weather.
- Exercising regularly, but not to the point that
you become very tired. Drink lots of fluids when you exercise. Talk with your
doctor about how much exercise is right for you.
- Getting enough sleep and rest. Tell your doctor
if you think you may have a sleep problem such as snoring or sleep apnea (a
condition in which you stop breathing for short periods during sleep).
- Limiting the amount of alcohol you drink.
- Quitting smoking.
Along with healthy lifestyle habits, there are some
things you can do to help prevent a sickle cell crisis:
- Contact your doctor if you have any signs of an
infection, such as a fever or trouble breathing. Get treated right away. You
may want to avoid decongestants, such as pseudoephedrine, that can constrict
blood vessels.
- Avoid extremes of heat and cold. Wear warm
clothes outside in cold weather and inside of air-conditioned rooms. Don’t
swim in cold water or climb at high altitudes without extra oxygen.
- Reduce the stress in your life. Talk to your
doctor if you’re depressed or having problems on the job or with your
family. Support from family and friends as well as a support group can help you
cope with daily life. If possible, don’t seek jobs that will require
strenuous physical labor, expose you to extremes of heat and cold, or involve
long work hours.
- Don’t travel in airplanes where the cabin
isn’t pressurized (that is, no extra oxygen is pumped into the cabin). If
you must travel in such an airplane, talk to your doctor about how to protect
yourself.
Regular medical checkups and treatment are
important.
- Checkups may include tests for possible kidney,
lung, and liver diseases as well as any side effects from medicines you take.
See a sickle cell anemia expert regularly.
- Learn the symptoms of a stroke and report them to
your doctor promptly. Symptoms of stroke can include a lasting headache,
weakness on one side of the body, limping, or a sudden change in speech,
vision, or hearing. A change in behavior also can be a symptom of a
stroke.
- Get a flu shot and other vaccinations to prevent
infections.
- See your dentist regularly to prevent infections
and loss of teeth.
- See an eye doctor regularly to check for damage
to your eyes.
- Get treatment and control any other medical
conditions you might have, such as diabetes.
- Talk with your doctor if you’re pregnant or
planning to become pregnant. You will need special prenatal care. Sickle cell
anemia can become more severe during pregnancy, with more painful crises. Women
with sickle cell anemia also are at an increased risk for an early birth or a
low-birth-weight baby. You can have a healthy pregnancy with early prenatal
care and frequent checkups.
Coping With Pain
Pain is different for each person. Pain that one
person can live with is too much for another person. Work with your doctor to
make a pain management plan that works well for you. It may include both
over-the-counter and prescription medicines. Talk with your doctor about how to
safely use narcotic pain medicines.
Other ways to manage pain include using a heating
pad, taking a hot bath, resting, or getting a massage. Physical therapy might
help to relieve your pain if it can help you relax and strengthen your muscles
and joints. Counseling and self-hypnosis may help. Also helpful are activities
that keep your mind off the pain, such as watching TV and talking on the phone.
Caring for a Child With Sickle Cell Anemia
If your child has sickle cell anemia, you should
learn as much about the condition as possible. This will help you recognize
early signs of problems, such as fever or chest pain, and seek early treatment.
Sickle cell centers and clinics can give you information and counseling to help
you handle the stresses of coping with this serious, chronic condition.
Frequent Doctor Visits
Your child will need to see the doctor often for
blood tests and to be checked for any possible damage to his or her lungs,
kidney, and liver. It’s recommended that before age 2, children with
sickle cell anemia see the doctor every 2 or 3 months. After age 2, children
may need to see the doctor at least every 6 months.
Talk to your child’s doctor about your
child’s treatment, how often the doctor should see the child, and the best
ways to help keep your child as healthy as possible.
Preventing Infection
Call your child’s doctor if your child has a
fever or if you notice any signs of infection, such as trouble breathing. Keep
a thermometer on hand, and know how to use it. Call a doctor if your child has
a temperature above 101 degrees Fahrenheit (38.5 degrees Celsius). To prevent
infection, most children will be given:
- Daily penicillin up to age 5.
- A flu shot every year after 6 months of age.
- A regular vaccination against pneumonia. (This
also is given to children who don’t have sickle cell anemia.) Children
with sickle cell anemia also get two doses of a stronger pneumonia vaccination
after 2 years of age. Doses of this stronger vaccination are given several
years apart.
- Vaccination against meningitis after the age of
2.
Preventing a Stroke
Ask your doctor whether your child needs regular
ultrasound scans of the head. Knowing the signs and symptoms of a possible
stroke is important. These include a lasting headache, weakness on one side of
the body, limping, or a sudden change in speech, vision, or hearing. It could
even include changes in behavior.
Find Out When To Call the Doctor
Ask your child’s doctor what you should report
to him or her right away. For example, you may be asked to call the doctor
right away if your child has any signs of a stroke or infection. You may be
told to call the doctor if your child has:
- Swelling of the hands, feet, or stomach
- Skin or nail beds that are suddenly pale or skin
or eyes that have a yellow color
- Sudden fatigue (tiredness) with no interest in
his or her surroundings
- Erection of the penis that won’t go
away
- Pain in the joints, stomach, chest, or
muscles
School-aged children can often—but not
always—take part in physical education or sports, but only after approval
of the child’s doctor. Ask your doctor about safe levels of exercise for
your child.
Caring for a Teen With Sickle Cell Anemia
Just as with any chronic condition, teens who have
sickle cell anemia must manage their condition while dealing with the stresses
of the teen years—peer pressure, sexuality, independence, education, and
career goals. Specific stresses faced by teens with sickle cell anemia include:
- Body-image problems caused by a delayed sexual
maturity
- Coping with pain and fear of addiction from using
narcotic pain medicines
- Living with uncertainty, because sickle cell
anemia is unpredictable and can cause pain and damage to the body at any time
Ways to support teens with sickle cell anemia
include teen support groups and family and individual counseling.
Key Points
- Sickle cell anemia is a serious condition in
which the red blood cells can become sickle-shaped (that is, shaped like a
“C”).
- Sickle-shaped cells don’t move easily
through the blood. They’re stiff and sticky and tend to form clumps and
get stuck in blood vessels. The clumps of sickle cells block blood flow in the
blood vessels, causing pain, serious infections, and organ damage.
- Sickle cell anemia is an inherited, lifelong
condition. People who have sickle cell anemia are born with it. They inherit
two copies of the sickle cell gene, one from each parent.
- People who inherit a sickle cell gene from one
parent and a normal gene from the other parent have a condition called sickle
cell trait. People with sickle cell trait don’t have the condition, but
they can pass the sickle cell gene on to their children.
- Sickle cell anemia affects millions of people
worldwide. It’s most common in people whose families come from Africa,
South or Central America (especially Panama), Caribbean islands, Mediterranean
countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
- In the United States, sickle cell anemia affects
about 70,000 people. It mainly affects African Americans.
- The signs and symptoms of sickle cell anemia are
different in each person. Some people have mild symptoms. Others have very
severe symptoms and are often hospitalized for treatment.
- The most common signs and symptoms of sickle
cell anemia are linked to
anemia and pain (also referred to as a "sickle cell
crisis").
- Infection is a major complication of sickle cell
anemia. Pneumonia, in particular, can be a life-threatening complication in
children with sickle cell anemia. Routine treatment with penicillin and
vaccinations are used to prevent infection in children.
- Strokes are a major complication for children
with sickle cell anemia. Routine screening and early treatment can prevent
strokes.
- Early diagnosis of sickle cell anemia is very
important so that children who have the condition can get proper treatment.
Screening tests for sickle cell anemia and sickle cell trait are done on
newborn infants in most States. One blood test used to diagnose sickle cell
anemia looks at how hemoglobin moves in an electric field. This is called
electrophoresis, and it’s usually used to diagnose older children and
adults.
- Effective treatments are available to help
relieve the symptoms and complications of sickle cell anemia, but in most cases
there’s no cure. (Some researchers believe that bone marrow transplants
may offer a cure in a small number of cases.)
- Sickle cell anemia is treated with medicines,
blood transfusions, and other treatments specific to certain
complications.
- Researchers are working to develop new treatments
for sickle cell anemia, including gene therapy and safer and more effective
bone marrow transplants.
- If you have sickle cell anemia, take good care of
yourself and see your doctor regularly.
- If your child has sickle cell anemia, you should
learn as much about the condition as possible. This will help you recognize
early signs of problems, such as fever or chest pain, and seek early treatment.
Links to Other Information About Sickle Cell
Anemia
NHLBI Resources
Non-NHLBI Resources
Clinical Trials
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