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Sickle Cell Pain
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What
is sickle cell disease?
Sickle cell disease is the name given to a group of illnesses
that are a result of red blood cells that become misshapen.
While there is no cure for sickle cell disease, there are
precautions for preventing painful sickle cell crises and
treatments to provide relief during pain crises. The most
common type of sickle cell disease is sickle cell anemia.
In 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.
Sickle cell crisis
Not 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.
People who get sickle cell crises find that there are several
factors that may cause a crisis to start. These include:
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Having
an infection, such as a cold or the flu |
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Cold
weather |
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Being
overly tired or fatigued |
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Exercising
too hard |
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Being
dehydrated (not drinking enough water and other fluids) |
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Not
having enough oxygen in the blood |
Sickle
cell crises often become so painful that people have to go
to the emergency room to get treated immediately with pain
medications.
Who gets
sickle cell disease?
In 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.
Sickle 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:
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Family
members may have two genes for healthy hemoglobin. These
family members will have no sign of sickle cell disease. |
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Family
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. |
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Family
members may have two genes for sickle hemoglobin. These
family members will have sickle cell disease. |
People
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
the disease.
What are the symptoms of sickle cell disease?
Children 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.
The 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.
Other than pain, there are additional health problems that
those with sickle cell disease may experience.
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People
with sickle cell disease are more likely to get infections.
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Sickle
cell disease can cause anemia (or a low blood count),
which can make people feel tired. |
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Organs
(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. |
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People
with sickle cell disease may suffer from "acute chest
syndrome", which is difficulty breathing and severe
chest pain. |
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People
with sickle cell disease may have problems with their
eyes as a result of blockages in small blood vessels. |
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Skin
ulcers, or sores on the skin that do not heal well may
develop, especially on the lower legs. |
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People
with sickle cell may become jaundiced, meaning that the
whites of the eyes or the skin turn a yellowish color. |
Family
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.
Treatment
for sickle cell disease
Unfortunately, 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.
Some 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.
General medical
treatments
Doctors 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 well.
Some patients may be prescribed hydroxyurea, a medication
that might reduce the chance that the red blood cells will
change into the sickle shape.
Also, doctors recommend that patients with sickle cell take
extra folic acid, a vitamin.
Folic acid is reduced in sickle cell patients due to the nature
of this disease. The increased requirement for folic acid
is common to all patients who have this acquired hematologic
disorder. The usual requirement is 1 mg per day orally for
the rest of the patient’s life. Folic acid prevents severe
anemia in sickle cell disease.
Treatment
for sickle cell pain
The 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. 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.
Still, 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.
Other medications that may be prescribed by a doctor for relief from sickle cell pain are:
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Hydroxyurea (e.g., Hydrea and Droxia) |
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Anti-inflammatory medications, such as ibuprofen, aspirin, or Vioxx |
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Steroids, such as prednisone |
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Tricyclic antidepressants, such as amitriptyline |
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Anticonvulsants, such as Gabapentin |
Non drug treatments
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.
Also, 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.
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Transcutaneous
electrical nerve stimulation (TENS) – a low intensity
electrical current can sometimes be effective for pain
relief. |
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Physical
therapy – gentle exercises and heat and cold treatments
can help with sickle cell disease pain. |
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Relaxation
techniques – learning how to relax can help patients
cope better with the illness and pain. |
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Massage
– especially at the first signs of a crisis, massage
can decrease muscle stiffness and potentially reduce pain. |
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Acupuncture
and acupressure – can be useful in helping to relieve
stress and manage pain. |
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Distraction
– engaging activities (such as hobbies, video games
and movies) that change the patient's focus can help relieve
stress and pain. |
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Psychotherapy
– speaking with a mental health professional about
the stress and frustration of sickle cell disease and/or
learning cognitive/behavioral techniques for coping with
the disease can be beneficial. |
How
you can obtain treatment for pain
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Find
medical practitioners who understand sickle-cell-related
pain. |
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Ensure
regular visits to sickle cell pain specialists. |
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Ask
questions of doctors and discuss your concerns. |
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Take
pain medication as prescribed. |
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Massage
sore or painful areas if the physician approves. |
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Encourage
the use of relaxation techniques to relieve stress and
decrease pain. Here is more
information on relaxation techniques. |
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Find
ways to avoid and manage stress in the household. |
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Engage
in activities you enjoy. |
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Watch
for signs of pain or other discomfort. |
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Rate
and record pain in a daily pain journal. |
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Contact
your medical practitioner if pain is not relieved by medication. |
Sickle Cell Links
Organizations
American
Sickle Cell Anemia Association (ASCAA)
10300 Carnegie Avenue
Cleveland Clinic / East Office Building (EEb18)
Cleveland, OH 44106
(216) 229-8600
Provides a nationwide directory of support groups that includes
groups for both patients and parents. Other services performed
by ASCAA include testing, education, counseling, support services,
newborn testing, lead screening, genetic counseling, and an
online bulletin board where you can discuss issues related
to sickle cell with peers.
National
Institutes of Health National Heart, Lung, and Blood Institute
2 Rockledge Centre
6701 Rockledge Drive
MSC 7950, Room 100420
Bethesda, MD 20892-7950
(301) 435-0055
This is the primary National Institutes of Health organization
for research on sickle cell anemia.
Sickle Cell
Advocates for Research and Empowerment, Inc. (S.C.A.R.E.)
P.O. Box 630127
Bronx, NY 10463
(718) 884-9670
S.C.A.R.E. is an advocacy group working to empower sickle
cell patients and their families.
Sickle
Cell Disease Association of America
4221 Wilshire Boulevard
Los Angeles, CA 90010
(800) 421-8453
This website provides answers to frequently asked questions
about sickle cell disease and offers an online chat room where
patients and caregivers can communicate with peers.
Websites
MEDLINEplus
Health Information
MEDLINE is a service of the National Library of Medicine that
provides excellent links to websites for a variety of topics
related to sickle cell disease.
Sickle
Cell Information Center
Georgia Comprehensive Sickle Cell Center at Grady Health System
This is one of the most comprehensive sickle cell sites and
has information provided and reviewed by an advisory board
composed of physicians and other health care professionals.
The types of information provided include medical, newborn
screening, research, and educational materials for health
care professionals.
Nemours
Foundation
This site, which is geared toward the parents of sickle cell
patients, provides medical information on the disease.
Español/Spanish Information
Anemia
Falciforme (March of Dimes Birth Defects Foundation)
Datos
Sobre la Anemia Falciforme (Facts About Sickle Cell Anemia)
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