What Is Sickle Cell Anemia?
Sickle cell anemia (uh-NEE-me-uh) is a serious
disease in which the body makes sickle-shaped red blood cells.
“Sickle-shaped” means that the red blood cells are shaped like a
"C."
Normal red blood cells are disc-shaped and look like
doughnuts without holes in the center. They move easily through your blood
vessels. Red blood cells contain the protein hemoglobin (HEE-muh-glow-bin).
This iron-rich protein gives blood its red color and carries oxygen from the
lungs to the rest of the body.
Sickle cells contain abnormal hemoglobin that causes
the cells to have a sickle shape. Sickle-shaped cells don’t move easily
through your blood vessels. They’re stiff and sticky and tend to form
clumps and get stuck in the blood vessels. (Other cells also may play a role in
this clumping process.)
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
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 blood flow in a blood
vessel. (Other cells also may play a role in this clumping process.) The inset
image shows a cross-section of a sickle cell with abnormal hemoglobin.
Overview
Sickle cell anemia is one type of
anemia.
Anemia is a condition in which your blood has a lower than normal number of red
blood cells. This condition also can occur if your red blood cells don’t
have enough hemoglobin.
Red blood cells are made in the spongy marrow inside
the large bones of the body. Bone marrow is always making new red blood cells
to replace old ones. Normal red blood cells last about 120 days in the
bloodstream and then die. They carry oxygen and remove carbon dioxide (a waste
product) from your body.
In sickle cell anemia, a lower-than-normal number of
red blood cells occurs because sickle cells don’t last very long. Sickle
cells usually die after only about 10 to 20 days. The bone marrow can’t
make new red blood cells fast enough to replace the dying ones.
Sickle cell anemia is an inherited, lifelong
disease. People who have the disease 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 who
have sickle cell trait don’t have the disease, but they have one of the
genes that cause it. Like people who have sickle cell anemia, people who have
sickle cell trait can pass the gene to their children.
Outlook
Sickle cell anemia affects millions of people
worldwide. The disease has no widely available cure. However, there are
treatments for the symptoms and complications of the disease.
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 sickle cell anemia. They know its causes, how it affects the body,
and how to treat many of its complications.
Sickle cell anemia varies from person to person.
Some people who have the disease have chronic (long-term) pain or fatigue
(tiredness). However, with proper care and treatment, many people who have the
disease can have improved quality of life and reasonable health much of the
time.
Due to improved treatment and care, people who have
sickle cell anemia are now living 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
- Sickle cell disease
What Causes Sickle Cell Anemia?
Sickle cell anemia is an inherited disease. People
who have the disease inherit two copies of the sickle cell gene—one from
each parent.
The sickle cell gene causes the body to make
abnormal hemoglobin. Hemoglobin is the iron-rich protein that gives blood its
red color and carries oxygen from the lungs to the rest of the body.
In sickle cell anemia, the hemoglobin sticks
together when it delivers oxygen to the body’s tissues. These clumps of
hemoglobin are like liquid fibers. They cause the red blood cells to become
stiff and shaped like a sickle, or “C.” The sickled red blood cells
tend to stick together and get caught in the blood vessels. (Other cells also
may play a role in this process.)
Two copies of the sickle cell gene are needed for
the body to make the abnormal hemoglobin found in sickle cell anemia.
Sickle Cell Trait
If you inherit only one copy of the sickle cell gene
(from one parent), you will not have sickle cell anemia. Instead, you will have
sickle cell trait.
People who have sickle cell trait usually have no
symptoms and lead normal lives. However, they can pass the sickle cell gene to
their children.
The following image shows how two parents who have
sickle cell trait can pass the sickle cell gene to their children.
Example of an Inheritance Pattern for Sickle Cell
Trait
The image shows how sickle cell
genes are inherited. A person inherits two copies of the hemoglobin
gene—one from each parent. A normal gene will make normal hemoglobin (A).
An abnormal (sickle cell) gene will make 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. The disease occurs in
about 1 out of every 500 African American births. Sickle cell anemia also
affects Hispanic Americans. The disease occurs in 1 out of every 36,000
Hispanic American births.
About 2 million Americans have sickle cell trait.
The condition occurs in about 1 in 12 African Americans.
What Are the Signs and Symptoms of Sickle Cell
Anemia?
The signs and symptoms of sickle cell anemia vary.
Some people have mild symptoms. Others have very severe symptoms and often are
hospitalized for treatment.
Sickle cell anemia is present at birth, but 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 the disease’s
complications.
Signs and Symptoms Related to Anemia
The most common symptom of anemia is fatigue
(feeling tired or weak). Other signs and symptoms of anemia include:
- Shortness of breath
- Dizziness
- Headache
- Coldness in the hands and feet
- Pale skin
- Chest pain
Signs and Symptoms Related to Pain
Sudden pain throughout the body is a common symptom
of sickle cell anemia. This pain is called a "sickle cell crisis." Sickle cell
crises often affect the bones, lungs, abdomen, and joints.
A sickle cell crisis occurs when sickled red blood
cells form clumps in the bloodstream. (Other cells also may play a role in this
clumping process.) These clumps of cells block blood flow through the small
blood vessels in the limbs and organs. This can cause pain and organ damage.
The pain from sickle cell crises can be acute or
chronic, but acute pain is more common. Acute pain is sudden and can range from
mild to very severe. The pain usually lasts from hours to a few days. Chronic
pain often lasts for weeks to months. Chronic pain can be hard to bear and
mentally draining. This pain may severely limit your daily activities.
Almost all people who have sickle cell anemia have
painful crises at some point in their lives. Some have these crises less than
once a year. Others may have 15 or more crises in a year.
Many factors can play a role in a sickle cell
crisis. Often, more than one factor is involved and the exact cause isn’t
known. You can control some factors. For example, your risk for a sickle cell
crisis increases if you’re dehydrated (your body doesn’t have enough
fluid). Drinking plenty of fluids can lower your risk for a painful crisis.
Other factors, such as an infection, you can’t control.
Painful crises are the leading cause of emergency
room visits and hospitalizations of people who have sickle cell anemia.
Complications of Sickle Cell Anemia
The effects of sickle cell crises on different parts
of the body can cause a number of complications.
Hand-Foot Syndrome
Sickle cells can block the small blood vessels in
the hands or feet. This condition is called hand-foot syndrome. It can lead to
pain, swelling, and fever. One or both hands and/or feet may be affected at the
same time.
You may feel the pain in the many bones of the hands
and feet. Swelling often occurs on the back of the hands and feet and moves
into the fingers and toes. Hand-foot syndrome may be the first sign of sickle
cell anemia in infants.
Splenic Crisis
The spleen is an organ in the abdomen. Normally, it
filters out abnormal red blood cells and helps fight infection. In some cases,
the spleen may trap cells that should be in the bloodstream. This causes the
spleen to grow large and leads to anemia.
If the spleen gets too clogged with sickle cells, it
won’t work right. This can cause the spleen to shrink. If this happens,
you may need
blood
transfusions until your body can make more cells and recover.
Infections
Both children and adults who have sickle cell anemia
have a hard time fighting infections. This is because sickle cell anemia can
damage the spleen, an organ that helps fight infections.
Infants and young children who have damaged spleens
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 who have sickle cell anemia.
Acute Chest Syndrome
Acute chest syndrome is a life-threatening condition
linked to sickle cell anemia. It’s similar to pneumonia. The condition is
caused by an infection or sickle cells trapped in the lungs.
People who have this condition usually have chest
pain and fever. They also often have abnormal
chest
x ray results. Over time, lung damage from acute chest syndrome may lead to
pulmonary
arterial hypertension (PAH).
Pulmonary Arterial Hypertension
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 rise.
Increased blood pressure in the lungs is called
pulmonary arterial hypertension, or PAH. Shortness of breath and problems with
breathing are the main symptoms of PAH.
Delayed Growth and Puberty in Children
Children who have sickle cell anemia often grow more
slowly than other children. They also reach puberty later. A shortage of red
blood cells causes the slow growth rate. Adults who have sickle cell anemia
often are slender or smaller in size than other adults.
Stroke
Two forms of
stroke can occur in people who have sickle cell anemia. One
form occurs when a blood vessel in the brain is blocked. The other form occurs
when a blood vessel in the brain bursts.
A stroke can cause learning disabilities and/or
lasting brain damage, long-term disability, paralysis (an inability to move),
or death.
Eye Problems
Sickle cells also can clog the small blood vessels
that deliver oxygen-rich blood to your eyes. This can damage the
retinas—thin layers of tissue at the back of your eyes. The retinas take
the images you see and send them to your brain.
Without enough blood, the retinas will weaken. This
can cause serious problems, including blindness.
Priapism
Males who have sickle cell anemia may have painful
and unwanted erections. This condition is called priapism (PRI-a-pizm). It
happens because the sickle cells block 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. The body breaks down this protein into a compound called bilirubin.
Too much bilirubin in the body can cause stones to form in the gallbladder.
Gallstones may 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 who have gallstones may have nausea (feeling
sick to the stomach), vomiting, fever, sweating, chills, clay-colored stools,
or jaundice (a 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. These sores usually appear between the ages of 10 and
50.
The cause of sickle cell ulcers isn’t clear.
The number of ulcers can vary from one to many. Some heal quickly, but others
persist for years or come back after healing.
Multiple Organ Failure
Multiple organ failure is rare, but serious. It
happens if you have a sickle cell crisis that causes two out of three major
organs (lungs, liver, or kidney) to fail.
Symptoms of 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. Children who have the disease need prompt and proper treatment.
In the United States, all States mandate testing for
sickle cell anemia as part of their newborn screening program.
The test uses blood from the same blood samples used
for other routine newborn screening tests. It can show whether a newborn infant
has sickle cell anemia or sickle cell trait.
Test results are sent to your baby’s primary
care doctor. This is the doctor who you name on the papers you fill out at the
hospital before giving birth. It’s important to provide correct contact
information to the hospital. This allows your baby’s doctor to get the
test results as quickly as possible.
If the test shows some sickle hemoglobin, a second
blood test is done to confirm the diagnosis. The second test should be done as
soon as possible and within the first month of life. If your baby needs a
second test, the primary care doctor may send you to a hematologist. This is a
doctor who specializes in blood diseases and disorders.
It’s also possible for doctors to diagnose
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 looks for the sickle cell gene, rather than the
hemoglobin that the gene makes.
How Is Sickle Cell Anemia Treated?
Sickle cell anemia has no widely available cure.
However, treatments can help relieve symptoms and treat complications. The
goals of treating sickle cell anemia are to relieve pain; prevent infections,
eye damage, and
strokes; and control complications (if they occur).
Bone
marrow transplants may offer a cure in a small number of sickle cell anemia
cases. Researchers continue to look for new treatments for the disease. These
include gene therapy and improved bone marrow transplants.
Specialists Involved
People who have sickle cell anemia need regular
medical care. Some doctors and clinics specialize in treating people who have
the disease. Hematologists and pediatric hematologists specialize in treating
adults and children who have blood diseases and disorders.
Treating Pain
Medicines and Fluids
Mild pain often is treated with over-the-counter
medicine and heating pads. Severe pain may need to be treated in a
hospital.
The usual treatments for acute (short-term) pain
crises are fluids and pain-killing medicines. Fluids help prevent dehydration,
a condition in which your body doesn’t have enough fluids. Fluids are
given either by mouth or through a vein.
Common medicines used to treat pain crises include
acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics
such as meperidine, morphine, oxycodone, and others. (Narcotic abuse and
addiction are issues that must be considered in any pain control plan.)
Treatment for 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 with NSAIDs or acetaminophen.
Hydroxyurea
If you have severe sickle cell anemia, you may need
a medicine called hydroxyurea. This medicine helps reduce the number of painful
crises you have. Hydroxyurea is used to prevent painful crises, not to treat
them when they occur.
Given daily, this medicine reduces how often painful
crises and acute chest syndrome occur. People taking the medicine also need
fewer blood transfusions and have fewer hospital visits.
Early studies of hydroxyurea in children show that
they have improved growth. These studies also suggest that this medicine may
help preserve organ function.
Hydroxyurea can cause serious side effects,
including an increased risk for dangerous infections. People who take
hydroxyurea must be carefully watched. Your doctor may need to adjust the dose
of this medicine to reduce the risk for side effects.
Doctors are studying the long-term effects of
hydroxyurea on people who have sickle cell anemia. Studies are under way to
find out whether hydroxurea prevents organ damage or other sickle cell
complications.
Talk to your doctor about the risks and benefits of
taking hydroxyurea.
Preventing Complications
Blood transfusions are commonly 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, people who have sickle cell
anemia need blood transfusions to prevent life-threatening problems such as
stroke or
pneumonia.
Having routine blood transfusions can cause side
effects. Patients must be carefully watched. Side effects can include a
dangerous buildup of iron in the blood (which must be treated) and an increased
risk of infection from the transfused blood.
For more information, see the Diseases and
Conditions Index
Blood
Transfusion article.
Infections
Infections are a major complication of sickle cell
anemia. In fact, pneumonia is the leading cause of death in children who have
sickle cell anemia. Other common infections linked to sickle cell anemia
include meningitis, influenza, and hepatitis.
If a child who has 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.
- All routine vaccinations (such as a yearly flu
shot) plus the meningococcal vaccine.
Adults who have sickle cell anemia also should have
flu shots every year and get vaccinated for pneumonia.
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 who have sickle cell anemia also should have
regular checkups with an eye doctor.
Strokes
Stroke prevention and treatment is now possible for
children and adults who have sickle cell anemia. Starting at age 2, children
who have sickle cell anemia often get routine ultrasound scans of their heads.
This is called transcranial Doppler ultrasound. These scans are used to check
blood flow in the brain.
The scans allow doctors to find out which children
are at high risk for a stroke. These children are then treated with routine
blood transfusions. This treatment has been found to greatly reduce the number
of strokes in children.
Treating Other Complications
Acute chest syndrome is a severe and
life-threatening complication of sickle cell anemia. Treatment usually requires
hospitalization and may include oxygen, blood transfusions, antibiotics, pain
medicine, and checking the body's fluids.
If you have leg ulcers due to sickle cell anemia,
you 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 (a painful erection in males) can be
treated with fluids or surgery.
Regular Health Care for Children
Children who have sickle cell anemia need regular
health care (just like children who don't have the disease). They need to have
their growth checked. They also need to get the routine shots that all children
get.
All children younger than 2 need to see the doctor
often. Children who have sickle cell anemia may need additional checkups. After
age 2, children who have sickle cell anemia 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. Talk with your
child's doctor about eye checkups and whether your child needs an ultrasound
scan of the brain.
Until age 5, daily penicillin is given to most
children who have sickle cell anemia. Doctors also give many children a vitamin
called folic acid (folate) to help prevent some sickle cell complications.
New Treatments
Research on bone marrow transplants, gene therapy,
and new medicines for sickle cell anemia is ongoing. 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 disease.
Bone Marrow Transplant
A bone marrow transplant can work well for treating
sickle cell anemia. This treatment may even offer a cure in a small number of
cases.
However, the procedure is risky and can lead to
serious side effects or even death. Because of this, only some people can or
should have this procedure.
Bone marrow transplants usually are used only for
young patients who have severe sickle cell anemia. However, the decision to
give this treatment is made on a case-by-case basis.
Bone marrow used for a transplant must come from a
closely matched donor. This is usually a close family member who doesn't have
sickle cell anemia.
Researchers continue to look for ways to reduce the
risks of this procedure.
Gene Therapy
Gene therapy is being studied as a possible
treatment for sickle cell anemia. Researchers want to know whether a normal
gene can be put in the bone marrow of a person who has sickle cell anemia. This
would cause the body to make normal red blood cells.
Researchers also are studying whether they can "turn
off" the sickle cell gene or "turn on" a gene that makes red blood cells behave
normally.
New Medicines
Researchers are studying several new medicines for
sickle cell anemia. These include:
- Butyric acid. This is a food additive that may
increase normal hemoglobin in the blood.
- Nitric oxide. This medicine may make sickle cells
less sticky and keep blood vessels open. People who have sickle cell anemia
have low levels of nitric oxide in their blood.
- Decitadine. This medicine increases hemoglobin F
levels (this type of hemoglobin carries more oxygen). It may be a good choice
instead of hydroxyurea.
How Can Sickle Cell Anemia Be Prevented?
You can't prevent sickle cell anemia because
it’s an inherited disease. However, you can take steps to reduce its
complications. (For more information, see "Living
With Sickle Cell Anemia.")
People who are at high risk for sickle cell anemia
and are planning to have children may want to consider genetic counseling. A
counselor can help you understand your risk of having a child who has the
disease and help explain the choices that are available to you.
You can get information about genetic counseling
from health departments, neighborhood health centers, medical centers, and
clinics that care for people who have sickle cell anemia.
Living With Sickle Cell Anemia
With good health care, many people who have sickle
cell anemia can live productive lives. They also can 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:
- Adopt or maintain a healthy lifestyle
- Take steps to prevent and control
complications
- Learn ways to cope with pain
If you have a child or teen who has sickle cell
anemia, you can take steps to learn about the disease and help your child
manage it.
Adopt or Maintain a Healthy Lifestyle
To take care of your health, you should adopt or
maintain healthy lifestyle habits.
Follow a healthy eating plan. Your doctor may
suggest that you take folic acid (a vitamin) every day to help your body make
new red blood cells. You also should drink at least 8 glasses of water every
day, especially in warm weather. This will help prevent dehydration (a
condition in which your body doesn’t have enough fluids).
Your body needs regular physical activity to stay
healthy. However, you should avoid exercise that makes you very tired. Drink
lots of fluids when you exercise. Talk with your doctor about how much and what
kinds of physical activity are right for you.
You also should get enough sleep and rest. Tell your
doctor if you think you may have a sleep problem, such as snoring or
sleep
apnea. Sleep apnea is a common disorder in which you have one or more
pauses in breathing or shallow breaths while you sleep.
Talk to your doctor about whether you can drink
alcohol and what amount is safe for you. If you smoke, quit. Talk to your
doctor about programs and products that can help you quit smoking.
Take Steps To Prevent and Control
Complications
Along with healthy lifestyle habits, you can take
other steps to prevent and control painful sickle cell crises. A number of
factors can cause sickle cell crises. Knowing how to avoid or control these
factors can help you manage your pain.
You may want to avoid decongestants, such as
pseudoephedrine. These medicines can tighten blood vessels and further prevent
red blood cells from moving smoothly through the 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, avoid jobs that require a lot of
physical labor, expose you to extremes of heat and cold, or involve long work
hours.
Also, don’t travel in airplanes where the
cabins aren’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.
Get a flu shot and other vaccines to prevent
infections. You also should see your dentist regularly to prevent infections
and loss of teeth. Contact your doctor if you have any signs of an infection,
such as a fever or trouble breathing. Getting treatment right away is
important.
Regular medical checkups and treatment are also
important. Checkups may include tests for possible kidney, lung, and liver
diseases. See a sickle cell anemia expert regularly. Also, see an eye doctor
regularly to check for damage to your eyes.
Learn the signs and symptoms of a stroke. They
include a lasting headache, weakness on one side of the body, limping, and
sudden changes in speech, vision, or hearing. If you have any of these
symptoms, report them to your doctor promptly.
Get treatment and control any other medical
conditions you have, such as diabetes.
Talk to your doctor if you’re pregnant or
planning to become pregnant. You will need special prenatal care. Sickle cell
anemia can worsen during pregnancy.
Women who have sickle cell anemia also are at an
increased risk for an early birth or a low-birth-weight baby. However, with
early prenatal care and frequent checkups, you can have a healthy
pregnancy.
Learn Ways To Cope 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
find ways to manage your pain. You may need both over-the-counter and
prescription medicines. Your doctor may prescribe narcotic pain medicines. If
so, ask him or her how to safely use these medicines.
Other ways to manage pain include using a heating
pad, taking a hot bath, resting, or getting a massage. Physical therapy might
help ease your pain by helping you relax and strengthening your muscles and
joints.
Counseling or self-hypnosis also may help. You may
find that activities that keep your mind off the pain, such as watching TV and
talking on the phone, are helpful.
Caring for a Child Who Has Sickle Cell Anemia
If your child has sickle cell anemia, you should
learn as much about the disease 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 stress of coping with your
child’s disease.
Ongoing Care
Your child will need to see the doctor often for
blood
tests. The doctor also will check your child for any possible damage to his
or her lungs, kidneys, and liver.
Talk to the doctor about your child’s treatment
plan, how often he or she needs checkups, and the best ways to help keep your
child as healthy as possible.
Preventing Infections
To prevent infections, make sure your child gets all
of the vaccines that his or her doctor recommends. (For more information on
vaccines, see "How Is Sickle Cell Anemia
Treated?")
Good hygiene also can help prevent infections. Make
sure your child washes his or her hands often. This will help lower the chances
of getting an infection.
Call the doctor right away if your child has any
signs of infection, such as fever or 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).
Preventing a Stroke
Know the signs and symptoms of a stroke so you can
take action. Signs and symptoms include a lasting headache, weakness on one
side of the body, limping, and sudden changes in speech, vision, or hearing.
Changes in behavior also may be a sign of a stroke.
Talk with the doctor about whether your child needs
regular ultrasound scans of the head. These scans can show whether your child
is at high risk for a stroke.
Calling the Doctor
Ask your child’s doctor about when you should
call him or her right away. For example, he or she may want you to call right
away if your child has any signs of a stroke or infection. You also may need to
call if your child has:
- Swelling of the hands or feet.
- Swelling of the stomach. If the spleen gets
larger than normal, you may see or feel swelling below the lower left rib. Your
child may complain that the area feels tender.
- Pale skin or nail beds or a yellowish color on
the skin or on the whites of the eyes.
- Sudden fatigue (tiredness) with no interest in
his or her surroundings.
- An erection of the penis that won’t go
away.
- Pain in the joints, stomach, chest, or
muscles.
- A fever.
School-aged children can often, but not always, take
part in physical education or sports. However, your child’s doctor should
approve any activity. Ask the doctor about safe activities for your child.
Caring for a Teen Who Has Sickle Cell Anemia
Teens who have sickle cell anemia must manage their
condition, while also dealing with the stresses of the teen years. These teens
also face some specific stresses related to sickle cell anemia, including:
- Body-image problems caused by delayed sexual
maturity.
- Coping with pain and fear of addiction from using
narcotic pain medicines.
- Living with uncertainty. (Sickle cell anemia is
unpredictable and can cause pain and damage to the body at any time.)
Teen support groups and family and individual
counseling are ways to support teens who have sickle cell anemia.
Key Points
- Sickle cell anemia is a serious disease in which
the body makes sickle-shaped red blood cells. "Sickle-shaped" means that the
red blood cells are shaped like a "C."
- Normal red blood cells are disc-shaped and move
easily through your blood vessels. Red blood cells contain the protein
hemoglobin. This iron-rich protein gives blood its red color and carries oxygen
from the lungs to the rest of the body.
- Sickle cells contain abnormal hemoglobin that
causes the cells to have a sickle shape. Sickle-shaped cells don’t move
easily through your blood vessels. They’re stiff and sticky and tend to
form clumps and get stuck in the 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.
- Sickle cell anemia is an inherited, lifelong
disease. 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 only one sickle cell gene
(from one parent) have a condition called sickle cell trait. Sickle cell trait
is different from sickle cell anemia. People who have the condition usually
have no signs or symptoms and lead normal lives. However, they can pass the
sickle cell gene to their children.
- Sickle cell anemia affects millions of people
worldwide. In the United States, the disease affects about 70,000
people—mainly African Americans.
- The most common signs and symptoms of sickle cell
anemia are linked to
anemia
and pain. Sudden pain throughout the body is a common symptom of sickle cell
anemia. This pain is called a "sickle cell crisis." Sickle cell crises often
affect the bones, lungs, abdomen, and joints.
- Early diagnosis of sickle cell anemia is very
important. Children who have the disease need prompt and proper treatment.
- Sickle cell anemia has no widely available cure.
However, there are treatments for the symptoms and complications of the
disease. Treatments include medicines, fluids, and procedures.
Bone
marrow transplants may offer a cure in a small number of cases.
- You can't prevent sickle cell anemia because
it’s an inherited disease. However, you can take steps to reduce its
complications.
- With good health care, many people who have
sickle cell anemia can live productive lives. They also can have reasonably
good health much of the time and live longer today than in the past. If you
have sickle cell anemia, it’s important to adopt or maintain a healthy
lifestyle, take steps to prevent and control complications, and learn ways to
cope with pain.
- If you have a child or teen who has sickle cell
anemia, you can take steps to learn about the disease and help your child
manage it.
- Researchers continue to look for new treatments
for sickle cell anemia. These include gene therapy and improved bone marrow
transplants.
Links to Other Information About Sickle Cell
Anemia
NHLBI Resources
Non-NHLBI Resources
Clinical Trials
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