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.
