Is Sickle Cell Disease?
cell disease is the name given to a group of illnesses that
are a result of red blood cells that become misshapen. The
most common type of sickle cell disease is sickle cell anemia.
most people, red blood cells are round and smooth. This round
and smooth shape allows the cells to move easily through the
blood vessels. People with sickle cell disease have abnormal
hemoglobin, which is a protein found in the red blood cells.
The sickle cell hemoglobin can cause the red blood cells to
change shapes, from the normal round shape into a crescent
(or sickle) shape. The sickle shaped red blood cells are hard
and sticky and do not pass well through blood vessels. As
a result, blood vessels can sometimes become clogged, preventing
blood from flowing through them. A clogged blood vessel is
called an "occlusion." Occlusions in blood vessels can lead
to a painful sickle cell crisis.
all people with sickle cell disease have crises. About 30%
rarely or never have the pain caused by a crisis. About 50%
have only a few crises throughout their lives. And about 20%
have frequent, severe sickle cell crises.
who get sickle cell crises find that there are several factors
that may cause a crisis to start. These include:
an infection, such as a cold or the flu
overly tired or fatigued
dehydrated (not drinking enough water and other fluids)
having enough oxygen in the blood
cell crises often become so painful that people have to go
to the emergency room to get treated immediately with pain
Gets Sickle Cell Disease?
the United States, sickle cell disease is most common among
African-Americans. One in 500 African-American children in
this country is born with sickle cell disease. About 72,000
people in the US currently have sickle cell disease, and it
affects people all over the world.
cell disease is not contagious, meaning that it cannot be
spread from person to person through the air or through contact.
Rather, sickle cell disease is inherited. This means that
the disease is passed through families, from parents to their
children. Each time a baby is born, he or she has two genes
that determine the make-up of the hemoglobin -- one that comes
from the mother and one that comes from the father. In families
with a history of sickle cell disease, there are three possibilities:
members may have two genes for healthy hemoglobin. These
family members will have no sign of sickle cell disease.
members may have one gene for healthy hemoglobin and one
gene for sickle hemoglobin. This is called having the
"sickle cell trait." People with sickle cell trait are
not sick and often do not know they have the sickle cell
gene, unless they are tested.
members may have two genes for sickle hemoglobin. These
family members will have sickle cell disease.
with any family history of sickle cell disease, or who have
the disease, may want to speak with a doctor before having
children to find out how likely a child is to be born with
Are the Symptoms of Sickle Cell Disease?
may start to have symptoms of sickle cell disease as young
as 6 months of age. Babies who are suffering from sickle cell
symptoms may be irritable or cranky and cry even when their
parents do everything they can to comfort them. Also, children
with sickle cell disease may develop more slowly and have
difficulties in school because of the pain from the disease.
most common symptom of sickle cell disease is pain. During
a painful crisis, pain may seem to come from the bones, usually
in the arms, hands, legs, feet, or back. Also, there may be
pain in the stomach or abdomen, or in the chest.
than pain, there are additional health problems that those
with sickle cell disease may experience.
with sickle cell disease are more likely to get infections.
cell disease can cause anemia (or a low blood count),
which can make people to feel tired.
(such as the liver and kidneys) can be damaged if too
many large blood vessels in the area are blocked and the
organs do not get enough oxygen.
with sickle cell disease may suffer from "acute chest
syndrome," which is difficulty breathing and severe chest
with sickle cell disease may have problems with their
eyes as a result of blockages in small blood vessels.
ulcers, or sores on the skin that do not heal well, may
develop, especially on the lower legs.
with sickle cell may become jaundiced, meaning that the
whites of the eyes or the skin turn a yellowish color.
members of people with sickle cell disease are also affected
by the illness. It may be stressful to deal with a child or
loved one who has special health needs and often requires
trips to the hospital. Parents and other family members may
feel frustrated with the health care system and the schools
for their lack of understanding about the illness.
for Sickle Cell Disease
there is no cure for sickle cell disease. The good news is
that there are treatments and precautions that can help treat
the disease, prevent painful sickle cell crises, and relieve
pain during sickle cell crises.
sickle cell crises can be prevented by avoiding the factors
that bring on the crises. This includes avoiding exposure
to cold weather, getting plenty of sleep and rest, being careful
not to exercise too hard, and drinking plenty of fluids.
may prescribe a medication to help with anemia, such as erythropoietin
(a medication given as a shot to improve the blood count).
Blood transfusions are sometimes used to improve anemia, as
patients may be prescribed hydroxyurea, a medication that
might reduce the chance that the red blood cells will change
into the sickle shape.
doctors recommend that patients with sickle cell take extra
folic acid, a vitamin.
terrible pain of a sickle cell crisis often requires a trip
to the emergency room. Some sickle cell patients have had
bad experiences in emergency rooms and hospitals when health
care professionals questioned their pain and why they wanted
pain medications. Some may even have been accused of being
addicted to the pain medications. But patients and health
care professionals should know that wanting medications to
help the severe pain of sickle cell crisis does not make a
patient a drug addict, and does not mean that they are trying
to "get high" from the medications. These bad experiences
may lead sickle cell patients to distrust health care professionals.
It is important that patients are able to find a doctor who
they trust to believe them and take good care of them.
patients and their family members may worry about addiction
to pain medications, particularly opioids (also called narcotics).
However, for many patients opioid medications can be extremely
helpful, particularly during a painful crisis. Patients who
have many crises can sometimes benefit from taking opioid
medications daily, along with additional pain medication during
crises. The daily opioids can help reduce the number of crises
and make the pain less severe.
medications that may be prescribed by a doctor for relief
from sickle cell pain are:
medications, such as ibuprofen
antidepressant medications or anticonvulsant medications
that also help relieve pain
A doctor may recommend that people with sickle cell disease
see a physical therapist. Physical therapists can use gentle
exercises and heat and cold treatments to help with sickle
cell disease pain.
it may be beneficial for people with sickle cell disease to
see a psychologist, social worker or psychiatrist to get help
coping with the illness or learn techniques that may be helpful
in controlling pain. It can also be helpful for family members
of patients with sickle cell disease to speak with a mental
health professional about the stress and frustration they
may feel. For more information on sickle cell pain and treatments, visit our Pain Medicine section.
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