Sickle cell anemia

Definition

Sickle cell anemia is an inherited form of anemia — a condition in which there aren't enough
healthy red blood cells to carry adequate oxygen throughout your body.

Normally, your red blood cells are flexible and round, moving easily through your blood vessels.
In sickle cell anemia, the red blood cells become rigid, sticky and are shaped like sickles or
crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can
slow or block blood flow and oxygen to parts of the body.

There's no cure for most people with sickle cell anemia. However, treatments can relieve pain
and help prevent further problems associated with sickle cell anemia.

Symptoms

Signs and symptoms of sickle cell anemia usually show up after an infant is 4 months old and
may include:

 Anemia. Sickle cells are fragile. They break apart easily and die, leaving you chronically short on
red blood cells. Red blood cells usually live for about 120 days before they die and need to be
replaced. However, sickle cells die after only 10 to 20 days. The result is a chronic shortage of
red blood cells, known as anemia. Without enough red blood cells in circulation, your body can't
get the oxygen it needs to feel energized. That's why anemia causes fatigue.
 Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell
anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood
vessels to your chest, abdomen and joints. Pain can also occur in your bones. The pain may vary
in intensity and can last for a few hours to a few weeks. Some people experience only a few
episodes of pain. Others experience a dozen or more crises a year. If a crisis is severe enough,
you may need hospitalization so that pain medication can be injected into your veins
(intravenously).
 Hand-foot syndrome. Swollen hands and feet may be the first signs of sickle cell anemia in
babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of their
hands and feet.
 Jaundice. Jaundice is a yellowing of the skin and eyes that occurs because of liver damage or
dysfunction. Occasionally, people who have sickle cell anemia have some degree of jaundice
because the liver, which filters harmful substances from the blood, is overwhelmed by the rapid
breakdown of red blood cells. In people with dark skin, jaundice is visible mostly as yellowing of
the whites of the eyes.
 Frequent infections. Sickle cells can damage your spleen, an organ that fights infection. This
may make you more vulnerable to infections. Doctors commonly give infants and children with
sickle cell anemia antibiotics to prevent potentially life-threatening infections, such as
pneumonia.
  Delayed growth. Red blood cells provide your body with the oxygen and nutrients you need for
growth. A shortage of healthy red blood cells can slow growth in infants and children and delay
puberty in teenagers.
 Vision problems. Some people with sickle cell anemia experience vision problems. Tiny blood
vessels that supply your eyes may become plugged with sickle cells. This can damage the retina
— the portion of the eye that processes visual images.

When to see a doctor

Although sickle cell anemia is usually diagnosed in infancy, if you or your child develops any of
the following problems, see your doctor right away or seek emergency medical care.

 Any signs or symptoms of stroke. If you notice any one-sided paralysis or weakness in the face,
arms or legs, confusion, trouble walking or talking, sudden vision problems or numbness or a
headache call 911 or your local emergency number right away.
 Swelling in the hands or feet.
 Abdominal swelling, especially if the area is tender to touch.
 Fever. People with sickle cell anemia have an increased risk of infection, and fever can be the
first sign of an illness.
 Pale skin or nail beds.
 Yellow tint to the skin or the whites of the eyes.

Causes

Sickle cell anemia is caused by a mutation in the gene that tells your body to make hemoglobin
— the red, iron-rich compound that gives blood its red color. Hemoglobin is a component of
every red blood cell. It allows red blood cells to carry oxygen from your lungs to all parts of your
body, and to carry carbon dioxide waste from throughout your body to your lungs so that it can
be exhaled.

Under normal circumstances, your body makes healthy hemoglobin known as hemoglobin A.
People with sickle cell anemia make hemoglobin S — the S stands for sickle.

The sickle cell gene is passed from generation to generation in a pattern of inheritance called
autosomal recessive inheritance. This means that both the mother and the father must pass on the
defective form of the gene for a child to be affected.

People with sickle cell trait have one normal hemoglobin gene and one defective form of the
gene. So their bodies make both normal hemoglobin and sickle cell hemoglobin. Their blood
may contain some sickle cells, but they generally don't experience symptoms. However, they are
carriers of the disease, which means they can pass the defective gene on to their children.

With each pregnancy, two people with sickle cell traits have:

 A 25 percent chance of having an unaffected child with normal hemoglobin

 A 50 percent chance of having a child who also is a carrier
  A 25 percent chance of having a child with sickle cell anemia

Risk factors

The risk of inheriting sickle cell anemia comes down to genetics. For a baby to be born with
sickle cell anemia, both parents must carry a sickle cell gene.

The gene is particularly common among people with African, Spanish, Mediterranean, Middle
Eastern and Indian ancestry. In the United States, it most commonly affects blacks and
Hispanics.

Complications

Sickle cell anemia can lead to a host of complications, including:

 Stroke. A stroke can occur if sickle cells block blood flow to an area of your brain. Stroke is one
of the most serious complications of sickle cell anemia. Signs of stroke include seizures,
weakness or numbness of your arms and legs, sudden speech difficulties, and loss of
consciousness. If your baby or child has any of these signs and symptoms, seek medical
treatment immediately. A stroke can be fatal.
 Acute chest syndrome. This life-threatening complication of sickle cell anemia causes chest
pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection or
by sickle cells blocking blood vessels in your lungs. It requires emergency medical treatment
with antibiotics, blood transfusions and drugs that open up airways in your lungs. Recurrent
attacks can damage your lungs.
 Pulmonary hypertension. About one-third of people with sickle cell anemia will eventually
develop high blood pressure in their lungs (pulmonary hypertension). Shortness of breath and
difficulty breathing are common symptoms of this condition, which can ultimately lead to heart
failure.
 Organ damage. Sickle cells can block blood flow through blood vessels, immediately depriving
an organ of blood and oxygen. In sickle cell anemia, blood is also chronically low on oxygen.
Chronic deprivation of oxygen-rich blood can damage nerves and organs in your body, including
your kidneys, liver and spleen. Organ damage can be fatal.
 Blindness. Tiny blood vessels that supply your eyes can get blocked by sickle cells. Over time,
this can damage the retina — the portion of the eye that processes visual images — and lead to
blindness.
 Skin ulcers. Sickle cell anemia can cause open sores, called ulcers, on your legs.
 Gallstones. The breakdown of red blood cells produces a substance called bilirubin. Bilirubin is
responsible for yellowing of the skin and eyes (jaundice) in people with sickle cell anemia. A high
level of bilirubin in your body can also lead to gallstones.
 Priapism. Men with sickle cell anemia may experience painful erections, a condition called
priapism. As occurs in other parts of the body, sickle cells can block the blood vessels in the
penis. This can damage the penis and eventually lead to impotence.
Preparing for your appointment

Sickle cell anemia is usually diagnosed through genetic screening done when a baby is born.
Those test results will likely be given to your family doctor or pediatrician. However, if sickle
cell anemia is diagnosed you'll probably then be referred to a doctor who specializes in blood
disorders (hematologist) or a pediatric hematologist.

Because appointments can be brief, and there's often a lot of ground to cover, it's a good idea to
be well prepared for your appointment. Here's some information to help you get ready, and what
to expect from your doctor.

What you can do

 Write down any symptoms you've noticed, including any that may seem unrelated to the
reason for which you scheduled the appointment.
 Bring a family member or friend along, if possible. Sometimes it can be difficult to remember all
the information provided during an appointment. Someone who accompanies you may
remember something that you missed or forgot.
 Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the
most of your time together. List your questions from most important to least important in case
time runs out. For sickle cell anemia, some basic questions to ask your doctor include:

 What's the most likely cause of my child's symptoms?

 Are there other possible causes?
 What kinds of tests are needed?
 What treatments are available, and which do you recommend?
 What types of side effects are common with these treatments?
 Are there any alternatives to the primary approach that you're suggesting?
 What's my child's prognosis?
 Are there any dietary or activity restrictions?
 Are there any brochures or other printed material that I can take with me? What Web sites do
you recommend?

In addition to the questions that you've prepared, don't hesitate to ask questions during your
appointment at any time that you don't understand something.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve
time to go over points you want to spend more time on. Your doctor may ask:

 When did you first notice these symptoms?

 Have they been continuous, or occasional?
 Does anything seem to improve these symptoms?
 What, if anything, appears to worsen the symptoms?
 Does anyone in your family have sickle cell anemia?
  Have you ever been told you have a trait for sickle cell anemia?

Tests and diagnosis

A blood test can check for hemoglobin S — the defective form of hemoglobin that underlies
sickle cell anemia. In the United States, this blood test is part of routine newborn screening done
at the hospital. But older children and adults can be tested too.

In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the
blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory,
where it's screened for hemoglobin S.

If the screening test is negative, there is no sickle cell gene present. If the screening test is
positive, further tests will be done to determine whether one or two sickle cell genes are present.
People who have one gene — sickle cell trait — have a fairly small percentage of hemoglobin S.
People with two genes — sickle cell disease — have a much larger percentage of the defective
hemoglobin.

Additional tests
To confirm any diagnosis, a sample of blood is examined under a microscope to check for large
numbers of sickle cells — a marker of the disease. If you or your child has the disease, a blood
test to check for anemia — a low red blood cell count — will be done. And your doctor may
suggest additional tests to check for possible complications of the disease.

If you or your child carries the sickle cell gene, you may be referred to a genetic counselor — an
expert in genetic diseases.

Sickle cell anemia can be diagnosed in an unborn baby by sampling some of the fluid
surrounding the baby in the mother's womb (amniotic fluid) to look for the sickle cell gene.

Once diagnosed with sickle cell anemia, your child will need to see his or her doctor more often
than most children do. For children under 2, ask your child's doctor how frequently checkups
need to be scheduled. For youngsters over age 2, at least two visits a year are usually scheduled.

Treatments and drugs

Bone marrow transplant offers the only potential cure for sickle cell anemia. But, finding a donor
is difficult and the procedure has serious risks associated with it, including death.

As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving
symptoms and preventing complications. If you have sickle cell anemia, you'll need to make
regular visits to your doctor to check your red blood cell count and monitor your health.
Treatments may include medications to reduce pain and prevent complications, blood
transfusions and supplemental oxygen, as well as bone marrow transplant.
Medications
Medications used to treat sickle cell anemia include:

 Antibiotics. Children with sickle cell anemia usually begin taking the antibiotic penicillin when
they're about 2 months of age and continue taking it until they're 5 years old. Doing so helps
prevent infections, such as pneumonia, which can be life-threatening to an infant or child with
sickle cell anemia. Antibiotics may also help adults with sickle cell anemia fight certain
infections.
 Pain-relieving medications. To relieve pain during a sickle crisis, your doctor may advise over-
the-counter pain relievers and application of heat to the affected area. You may also need
stronger prescription pain medication.
 Hydroxyurea (Droxia, Hydrea). This prescription drug, normally used to treat cancer, may be
helpful for adults with severe disease. When taken daily, it reduces the frequency of painful
crises and may reduce the need for blood transfusions. It seems to work by stimulating
production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent
the formation of sickle cells. There is some concern about the possibility that long-term use of
this drug may cause tumors or leukemia in certain people. Your doctor can help you determine if
this drug may be beneficial for you.

Assessing stroke risk

Using a special ultrasound machine (transcranial), doctors can learn which children have a higher
risk of stroke. This test can be used on children as young as 2, and those who are found to have a
high risk of stroke are then treated with regular blood transfusions.

Blood transfusions
In a red blood cell transfusion, red blood cells are removed from a supply of donated blood.
These donated cells are then given intravenously to a person with sickle cell anemia.

Blood transfusions increase the number of normal red blood cells in circulation, helping to
relieve anemia. In children with sickle cell anemia at high risk of stroke, regular blood
transfusions can decrease their risk of stroke.

Blood transfusions carry some risk. Blood contains iron. Regular blood transfusions cause an
excess amount of iron to build up in your body. Because excess iron can damage your heart, liver
and other organs, people who undergo regular transfusions must often receive treatment to
reduce iron levels. Deferasirox (Exjade) is an oral medication that can reduce excess iron levels.
It can be used in people older than 2.

Supplemental oxygen
Breathing supplemental oxygen through a breathing mask adds oxygen to your blood and helps
you breathe easier. It may be helpful if you have acute chest syndrome or a sickle cell crisis.

Bone marrow transplant

This procedure replaces bone marrow affected by sickle cell anemia with healthy bone marrow
from a donor who doesn't have the disease. It can be a cure, but the procedure is risky, and it's
difficult to find suitable donors. Researchers are still studying bone marrow transplants for
people with sickle cell anemia. Currently, the procedure is recommended only for people who
have significant symptoms and problems from sickle cell anemia.

Bone marrow transplant requires a lengthy hospital stay. After the transplant, you'll need drugs
to help prevent rejection of the donated marrow.

Treating complications
Doctors treat most complications of sickle cell anemia as they occur. Treatment may include
antibiotics, vitamins, blood transfusions, pain-relieving medicines, other medications and
possibly surgery, such as to correct vision problems or to remove a damaged spleen.

Experimental treatments
Scientists continue to gain new insights into the symptoms and causes of sickle cell anemia.
Some possible new treatments being studied include:

 Gene therapy. Because sickle cell anemia is caused by a defective gene, researchers are
exploring whether inserting a normal gene into the bone marrow of people with sickle cell
anemia will result in the production of normal hemoglobin. Scientists are also exploring the
possibility of turning off the defective gene while reactivating another gene responsible for the
production of fetal hemoglobin — a type of hemoglobin found in newborns that prevents sickle
cells from forming.
 Butyric acid. Normally used as a food additive, butyric acid may increase the amount of fetal
hemoglobin in the blood.
 Clotrimazole. This over-the-counter antifungal medication helps prevent a loss of water from
red blood cells, which may reduce the number of sickle cells that form.
 Nitric oxide. Sickle cell anemia causes low levels of nitric oxide, a gas that helps keep blood
vessels open and reduces the stickiness of red blood cells. Treatment with nitric oxide may
prevent sickle cells from clumping together.
 Nicosan. This is an herbal treatment in early trials in the U.S. Nicosan has been used to prevent
sickle crises in Nigeria.

Lifestyle and home remedies

Taking steps to stay healthy is critical for anyone with sickle cell anemia. Eating well, getting
adequate rest and protecting yourself from infections are good ways to maintain your health and
prevent crises.

Infants and children with sickle cell disease need to receive regular childhood vaccinations.
Children and adults with sickle cell anemia also should have a yearly flu shot and be immunized
against pneumonia.

If you or your child has sickle cell anemia, follow these suggestions to help stay healthy:

 Take folic acid supplements daily, and eat a balanced diet. Bone marrow needs folic acid and
other vitamins to make new red blood cells.
 Drink plenty of water. Staying hydrated helps keep your blood diluted, which reduces the
chance that sickle cells will form.
  Avoid temperature extremes. Exposure to extreme heat or cold can trigger the formation of
sickle cells.
 Reduce stress. A sickle crisis can occur as a result of stress.
 Exercise regularly, but don't overdo it. Talk with your doctor about how much exercise is right
for you.
 Use over-the-counter medications with caution. Some medications, such as the decongestant
pseudoephedrine, can constrict your blood vessels and make it harder for the sickle cells to
move through freely.
 Fly on airplanes with pressurized cabins. Unpressurized aircraft cabins may not provide enough
oxygen. Low oxygen levels can trigger a sickle crisis. Additionally, be sure to drink extra water
when traveling by air, as pressurized cabins can be dehydrating.
 Avoid high-altitude areas. Traveling to a high-altitude area may also trigger a crisis because of
lower oxygen levels.

Coping and support

If you or someone in your family has sickle cell anemia, you may want help with the stresses of
this lifelong disease. Sickle cell centers and clinics can provide information and counseling. Ask
your doctor or the staff at a sickle cell center if there are support groups for families in your area.
Talking with others who are facing the same challenges you are can be helpful.

It's especially important to find ways to control — and cope with — pain. Different techniques
work for different people, but it might be worth trying heating pads, hot baths, massages or
physical therapy. Prayer, family and friends also can be sources of support.

If you have a child with sickle cell anemia, learn as much as you can about the disease and make
sure your child gets the best health care possible. A child with sickle cell disease has special
needs and requires regular medical care. Your doctor can explain how often to bring your child
for medical care and what you can do if he or she becomes ill.

Prevention

If you carry the sickle cell trait, you may wish to see a genetic counselor before trying to
conceive a child. A genetic counselor can help you understand your risk of having a child with
sickle cell anemia. He or she can also explain possible treatments, preventive measures and
reproductive options.

There is an in vitro fertilization procedure that improves the chances for parents who both carry
the sickle cell gene to have a child with normal hemoglobin. This procedure is known as
preimplantation genetic diagnosis. First, eggs are taken from the mother. Then, sperm is taken
from the father. In a laboratory, the eggs are fertilized with the sperm. The fertilized eggs are
then tested for the presence of the sickle cell gene. Fertilized eggs free of the sickle cell gene can
be implanted into the mother for normal development. However, this procedure is expensive and
not always successful.
What is Sickle Cell Disease?
Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle
cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of
hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have
difficulty passing through small blood vessels.

When sickle-shaped cells block small blood vessels, less blood can reach that part of the body.
Tissue that does not receive a normal blood flow eventually becomes damaged. This is what
causes the complications of sickle cell disease. There is currently no universal cure for sickle cell
disease.

Hemoglobin – is the main substance of the red blood cell. It helps red blood cells carry oxygen
from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A.
Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are
soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live
for about 120 days before new ones replace them.

People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S
(S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as
normal red blood cells (normally about 16 days). They also become stiff, distorted in shape and
have difficulty passing through the body’s small blood vessels. When sickle-shaped cells block
small blood vessels, less blood can reach that part of the body. Tissue that does not receive a
normal blood flow eventually becomes damaged. This is what causes the complications of sickle
cell disease.

Types of Sickle Cell Disease

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS),
Sickle-Hemoglobin C Disease (SC)
Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

What is Sickle Cell Trait?

Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are produced
in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease.
People with sickle cell trait are generally healthy.

Inheritance

Sickle cell conditions are inherited from parents in much the same way as blood type, hair color
and texture, eye color and other physical traits. The types of hemoglobin a person makes in the
red blood cells depend upon what hemoglobin genes the person inherits from his or her parents.
Like most genes, hemoglobin genes are inherited in two sets…one from each parent.

Examples:

If one parent has Sickle Cell Anemia and the other is Normal, all of the children will have sickle
cell trait.

If one parent has Sickle Cell Anemia and the other has Sickle Cell Trait, there is a 50% chance
(or 1 out of 2) of having a baby with either sickle cell disease or sickle cell trait with each
pregnancy.

When both parents have Sickle Cell Trait, they have a 25% chance (1 of 4) of having a baby with
sickle cell disease with each pregnancy.

How will I know if I have the Trait?

A SIMPLE PAINLESS BLOOD TEST followed by a laboratory technique called Hemoglobin

Electrophoresis will determine the type of hemoglobin you have. When you pass an electric
charge through a solution of hemoglobin, distinct hemoglobins move different distances,
depending on their composition. This technique differentiates between normal hemoglobin (A),
Sickle hemoglobin (S), and other different kinds of hemoglobin (such as C, D, E, etc.).

Medical Problems

Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice
and the formation of gallstones.

The sickle cells also block the flow of blood through vessels resulting in lung tissue damage
(acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism
(painful prolonged erection). It also causes damage to most organs including the spleen, kidneys
and liver. Damage to the spleen makes sickle cell disease patients, especially young children,
easily overwhelmed by certain bacterial infections.

Treatment

Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in
the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus
bacteria and folic acid supplementation.

Treatment of complications often includes antibiotics, pain management, intravenous fluids,

blood transfusion and surgery all backed by psychosocial support. Like all patients with chronic
disease patients are best managed in a comprehensive multi-disciplinary program of care.

Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crises, risk
of stroke and other complications. Because red blood cells contain iron, and there is no natural
way for the body to eliminate it, patients who receive repeated blood transfusions can
accumulate iron in the body until it reaches toxic levels. It is important to remove excess iron
from the body, because it can gather in the heart, liver, and other organs and may lead to organ
damage. Treatments are available to eliminate iron overload.

Promising Treatment Developments

In search for a substance that can prevent red blood cells from sickling without causing harm to
other parts of the body, Hydroxyurea was found to reduce the frequency of severe pain, acute
chest syndrome and the need for blood transfusions in adult patients with sickle cell disease.
Droxia, the prescription form of hydroxyurea, was approved by the FDA in 1998 and is now
available for adult patients with sickle cell anemia. Studies will now be conducted to determine
the proper dosage for children.

Other treatment options in clinical development include new, more convenient options than
current therapies to eliminate iron overload caused by repeated blood transfusions.

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.
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 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.
 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.