College of Nursing

Sickle Cell Anemia

(Hematology)




Submitted by:
BSN 4-1 Group 3
Masenas, Ayr Hershel D.
Millamena, Cirmarie Hope B.
Pastores, Flora Angeli D.
Puedan, Jenivic
Torevillas, Jeth Viel




September 25, 2014
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opportunities in the art, sciences and
technology through quality instruction
and responsive research and
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historic Cavite recognized for
excellence in the development
of globally competitive and
morally upright individuals.

Republic of the Philippines
CAVITE STATE UNIVERSITY
DON SEVERINO DE LAS
ALAS CAMPUS
Indang, Cavite

Sickle Anemia
Sickle cell anemia (uh-NEE-me-uh) is the most common form of sickle cell disease (SCD). SCD
is a serious disorder in which the body makes sickle-shaped red blood cells. Sickle-shaped
means that the red blood cells are shaped like a crescent.
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 an iron-rich protein called
hemoglobin (HEE-muh-glow-bin). This protein carries oxygen from the lungs to the rest of the
body.
Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle
hemoglobin causes the cells to develop a sickle, or crescent, shape.
Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs
and organs. Blocked blood flow can cause pain and organ damage. It can also raise the risk for
infection.










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 blocking blood flow in a blood vessel. The
inset image shows a cross-section of a sickle
cell with abnormal (sickle) hemoglobin forming
abnormal strands.

Sickle cell anemia is an inherited form of anemia that occurs when the sickle cell gene is
inherited from both parents. This results in an abnormality of the oxygen-carrying molecule
(hemoglobin) in red blood cells (RBCs) that causes the normally round red blood cells to become
deformed into a "sickle" shape. This sickle shape causes early destruction of the red blood cells
(hemolysis), resulting in chronic anemia. It also causes the red blood cells to become trapped in
small blood vessels, which blocks the circulation and causes severe pain and injury to many
organs by interfering with their blood supply. Called sickle cell crisis, this problem occurs when
the patient with sickle cell anemia experiences cellular oxygen deprivation. This might occur
from overexertion, infection, high altitude, or cold.

The areas most often affected by the blocking action of sickle cells:
Lungs
Liver
Bones
Muscle
Brain
Spleen
Penis
Eyes
Kidneys

Red blood cells are made in the spongy marrow inside the larger bones of the body. Bone
marrow is always making new red blood cells to replace old ones. Normal red blood cells live
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, the abnormal 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.
A genetic problem causes sickle cell anemia. People with the disease are born with two sickle
cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait.
About 1 in 12 African Americans has sickle cell trait.
A child who inherits 1 normal gene from a parent and 1 mutated gene from the other is a
"carrier." A person who is a carrier wont have sickle cell disease, but may still pass the mutated
gene on to his or her children.
Pathophysiology:


















Diagnosis:
History: Individuals with sickle cell anemia manifest the features of chronic anemia, including
pale skin and mucous membranes, fatigue, and decreased exercise capacity. They may also have
a yellowing of their eyes and skin (jaundice), frequent infections, and poor wound healing. In
addition, there are usually periodic episodes of severe pain in the joints, muscles, chest,
or abdomen (sickle cell crisis). Episodes of constant erection of the penis (priapism) may occur
in two-thirds of men with the condition, and symptoms of stroke are not uncommon
Physical exam: Physical findings may include a pale complexion (pallor), yellowing of the skin
(jaundice), fever, elevated respiratory and heart rates, bleeding (hemorrhages) in the retina, and
leg ulcers. The spleen is initially enlarged and then becomes abnormally small after repeated
infarction (auto infarction). A young child may appear small for his or her age, while an older
child may experience delayed growth and puberty. The adult is usually thin with long
extremities, narrow shoulders and hips, a curved spine, and a barrel chest. Individuals with
splenic sequestration may have low blood pressure secondary to shock. They may also exhibit
signs of congestive heart failure and neurologic deficits.
Tests: A complete blood count (CBC) shows a marked anemia with low numbers of red blood
cells, together with elevated numbers of white blood cells and platelets. A sickle prep test can
demonstrate the sickle shape of the red blood cells. A specific blood test (hemoglobin
electrophoresis) demonstrates the abnormal hemoglobin itself, called hemoglobin-S. If a stroke is
suspected, a CT scan of the head is indicated for evaluation. A spinal tap is performed to
evaluate for suspected meningitis. Peripheral smear can help in the visualization of blood cells.
Arterial blood gas (ABG), electrocardiogram (ECG), chest x-ray (CXR), bone x-ray, bone scan,
urinalysis, liver function tests (LFTs) and abdominal ultrasound may also be indicated.
Blood Test
A blood test can check for hemoglobin S the defective form of hemoglobin that underlies
sickle cell anemia. A simple blood test, done at any time during a person's lifespan, can detect
whether he or she has sickle hemoglobin. However, early diagnosis is very important.
In the United States, all States mandate testing for sickle cell anemia as part of their newborn
screening programs. The test uses blood from the same blood samples used for other routine
newborn screening tests. The test can show whether a newborn infant has sickle hemoglobin.
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 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 few months of life.
Doctors also can diagnose sickle cell disease 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.)
Testing before birth can be done as early as 10 weeks into the pregnancy. This testing looks for
the sickle hemoglobin gene, rather than the abnormal hemoglobin that the gene makes.
Complications:
Individuals with sickle cell anemia are at risk for damage to major organs because of poor
circulation and sickle cell crises. Common complications include gallstones; damage to the
spleen (autosplenectomy), with resultant susceptibility to serious infections; damage to the lungs
(acute chest syndrome), bones (avascular necrosis), kidneys (papillary necrosis), and heart;
strokes; and blindness over time (a result of impaired circulation to the retina and retinal
bleeding). All these complications may occur or become worse during pregnancy.
Approximately one-quarter of individuals with the condition have neurological complications
such as stroke and transient ischemic attack (TIA).


Treatment:
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, organ damage, and strokes; and control complications (if they occur). Researchers are
investigating new treatments such as blood and marrow stem cell transplants, gene therapy, and
new medicines.
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 a bone marrow transplant.
The management focuses on prevention and treatment of sickle crises and complications.
Individuals are advised to avoid high altitudes and dehydration, treat infections promptly, and
receive preventive vaccinations against pneumococcal pneumonia, Haemophilus influenzae type
B, and meningococcus.
For most individuals, the only daily treatment is folic acid supplements, but oral hydroxyurea is
sometimes used regularly to reduce the frequency of crises. Treatment of acute crises often
requires hospitalization and consists of rest, pain medications given by injection or through a
vein (intravenous),intravenous fluids, oxygen, blood transfusions, and individualized treatments
for any infections or complications.
Bone marrow transplants are currently the only potential cure for sickle cell anemia. In this
treatment the patient's bone marrow (which makes the sickled red blood cells) is replaced with
bone marrow from another individual without sickle cell disease. However, it is difficult to find
the right bone marrow donor, and the drugs needed to make the transplant possible are highly
toxic.

Medications:
Medications used to treat sickle cell anemia include:
Antibiotics. Children with sickle cell anemia may begin taking the antibiotic penicillin
when they're about 2 months of age and continue taking it until they're at least 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). When taken daily, hydroxyurea reduces the frequency of
painful crises and may reduce the need for blood transfusions. Hydroxyurea seems to work
by stimulating production of fetal hemoglobin a type of hemoglobin found in newborns
that helps prevent the formation of sickle cells. Hydroxyurea increases your risk of
infections, and there is some concern that long-term use of this drug may cause tumors or
leukemia in certain people. However, this hasn't yet been seen in studies of the drug.
Hydroxyurea was initially used just for adults with severe sickle cell anemia. Studies on
children have shown that the drug may prevent some of the serious complications associated
with sickle cell anemia. But the long-term effects of the drug on children are still unknown.
Your doctor can help you determine if this drug may be beneficial for you or your child.
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 years, and those who are found to
have a high risk of stroke are then treated with regular blood transfusions.
Vaccinations to prevent infections
Childhood vaccinations are important for preventing disease in all children. But, these
vaccinations are even more important for children with sickle cell anemia, because infections can
be severe in children with sickle cell anemia. Your doctor will make sure your child receives all
of the recommended childhood vaccinations. Vaccinations, such as the pneumococcal vaccine
and the annual flu shot, are also important for adults with sickle cell anemia.
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.
Stem cell transplant
A stem cell transplant, also called a bone marrow transplant, involves replacing bone marrow
affected by sickle cell anemia with healthy bone marrow from a donor. Because of the risks
associated with a stem cell transplant, the procedure is recommended only for people who have
significant symptoms and problems from sickle cell anemia.
If a donor is found, the diseased bone marrow in the person with sickle cell anemia is first
depleted with radiation or chemotherapy. Healthy stem cells from the donor are filtered from the
blood. The healthy stem cells are injected intravenously into the bloodstream of the person with
sickle cell anemia, where they migrate to the bone marrow cavities and begin generating new
blood cells. The procedure requires a lengthy hospital stay. After the transplant, you'll receive
drugs to help prevent rejection of the donated stem cells.
A stem cell transplant carries risks. There's a chance that your body may reject the transplant,
leading to life-threatening complications. In addition, not everyone is a candidate for
transplantation or can find a suitable donor.
Experimental treatments:
Scientists are studying new treatments for sickle cell anemia, including:
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. Potential treatments using gene therapy are still a long way off,
however. No human trials using genes specifically for sickle cell have yet been done.
Nitric oxide. People with sickle cell anemia have low levels of nitric oxide in their blood.
Nitric oxide is 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.
Studies on nitric oxide have had mixed results so far.
Drugs to boost fetal hemoglobin production. Researchers are studying various drugs to
devise a way to boost the production of fetal hemoglobin. This is a type of hemoglobin that
stops sickle cells from forming.
Statins. These medications, which are normally used to lower cholesterol, may also help
reduce inflammation. In sickle cell anemia, statins may help blood flow better through blood
vessels.
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.
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 enlarged spleen is a common reason for a
blood transfusion.
Some, but not all, people who have sickle cell anemia need regular blood transfusions to prevent
life-threatening problems, such as stroke, spleen problems, or acute chest syndrome.
Having routine blood transfusions can cause side effects. Examples include allergic reactions and
a dangerous buildup of iron in the body (which must be treated). In general, the blood supply is
fairly safe from infections such as hepatitis and HIV.
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 may need treatment to reduce iron
levels. Deferasirox (Exjade) is an oral medication that can reduce excess iron levels.
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 into the bone marrow stem cells 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 hemoglobin gene or "turn
on" a gene that makes red blood cells behave normally.
New Medicines
Researchers are studying several medicines for sickle cell anemia. They include:
Decitabine. Like hydroxyurea, this medicine prompts the body to make fetal hemoglobin.
Fetal hemoglobin helps prevent red blood cells from sickling and improves anemia.
Decitabine might be used instead of hydroxyurea or added to hydroxyurea.
Adenosine A2a receptor agonists. These medicines may reduce pain-related
complications in people who have sickle cell anemia.
5-HMF. This natural compound binds to red blood cells and increases their oxygen. This
helps prevent the red blood cells from sickling.
Reference:

http://www.mdguidelines.com/sickle-cell-anemia

http://www.nhlbi.nih.gov/health/health-topics/topics/sca/names.html

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/complications/con-
20019348

http://www.nlm.nih.gov/medlineplus/sicklecellanemia.html

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/treatment/con-
20019348

http://www.nhlbi.nih.gov/health/health-topics/topics/sca/treatment.html

http://doctorsgates.blogspot.com/2011/01/diagram-for-effects-of-sickle-cell.html