Sickle Cell Disease AAHN1

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University of Saint Louis Tuguegarao
College of Health and Allied Sciences
Masters of Science in Nursing







ADVANCE ADULT HEALTH NURSING I

ADVANCE PATHOPHYSIOLOGY





Conceptual Approach in Pathophysiology

Sickle Cell Disease


















Prepared by:

ARAO, Sheila A.









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Case Scenario

Case A 25-year-old African American man is admitted to your service with the diagnosis of a sickle cell
pain episode. He was admitted to the hospital six times last year with the same diagnosis, and he was
last discharged 2 months ago. Again he presented to the emergency room complaining of abdominal
and bilateral lower extremity pain, his usual sites of pain. When you examine him, you note he is febrile
to 101F, respiratory rate 25 breaths per minute, normal blood pressure, and slight tachycardia of 100
bpm. Lung examination reveals bronchial breath sounds and egophony in the right lung base. His oxygen
saturation on 2 L/min nasal cannula is 92%. Besides the usual abdominal and leg pain, he is now
complaining of chest pain, which is worse on inspiration. Although he is tender on palpation of his
extremities, the remainder of his examination is normal. His laboratory examinations reveal elevated
white blood cell and reticulocyte counts, and a hemoglobin and hematocrit that are slightly lower than
baseline. Sickle and target cells are seen on the peripheral smear

I. INTRODUCTION (Case Definition)

The first description of sickle cell disease was by a Chicago physician, James B. Herrick, who noted in
1910 that a patient of his from the West Indies had an anemia characterized by unusual red cells that
were "sickle shaped".
Sickle cell anemia 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. 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. 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 contain enough hemoglobin.
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. Sickle cell anemia is an
inherited, lifelong disease. People who have the disease are born with it. They inherit two genes for
sickle hemoglobinone from each parent. People who inherit a sickle hemoglobin gene from one
parent and a normal gene from the other parent have a condition called sickle cell trait. Sickle cell trait is
different than sickle cell anemia. People who have sickle cell trait don't have the disease. Like people
who have sickle cell anemia, people who have sickle cell trait can pass the sickle hemoglobin gene to
their children.
II. PREVALENCE AND POPULATION AT RISK

4,000 to 5,000 pregnancies are at risk for sickle cell disease each year in the U.S. 6 to 9 million infants
are born each year with sickle cell disease in Africa. Sickle cell disease occurs in 1 in 600 African-
American infants. Sickle cell anemia accounts for 60% to 70% of sickle cell disease in the U.S. Highest
prevalence among people of African, African-American, Mediterranean (Italian, Sicilian, Greek), Middle
Eastern, East Indian, Caribbean, and Central or South American descent. 8% to 10% of African-American
neonates in the U.S. are carriers of the sickle cell trait. 25% to 30% of neonates in western Africa are
carriers of the sickle cell trait.

III. RISK FACTORS: ENVIRONMENTAL, PERSONAL AND DEVELOPMENTAL FACTORS

1. Age- Affected patients characteristically are asymptomatic until approximately 4 to 6 months of age
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- Median age at death is approximately 42 years for men and 48 years for women
2. Race- Highest prevalence among people of African, African-American, Mediterranean (Italian, Sicilian,
Greek), Middle Eastern, East Indian, Caribbean, and Central or South American descent
- Occurs more commonly in people (or their descendants) from parts of the world such as Sub-
Saharan Africa, where malaria is or was common, but it also occurs in people of other ethnicities
3. Genetics- Sickle cell anemia is a genetic disorder caused by an autosomal recessive single gene defect
in the -globin chain of HbA, which produces HbS.

IV. MECHANISM

HbS arises from a mutation substituting thymine for adenine in the sixth codon of the beta-chain gene,
GAG to GTG. This causes coding of valine instead of glutamate in position 6 of the Hb beta chain. The
resulting Hb has the physical properties of forming polymers under deoxy conditions. It also exhibits
changes in solubility and molecular stability. These properties are responsible for the profound clinical
expressions of the sickling syndromes.
Under deoxy conditions, HbS undergoes marked decrease in solubility, increased viscosity, and polymer
formation at concentrations exceeding 30 g/dL. It forms a gel-like substance containing Hb crystals
called tactoids. The gel-like form of Hb is in equilibrium with its liquid-soluble form. A number of factors
influence this equilibrium, including oxygen tension, concentration of Hb S, and the presence of other
hemoglobins.
Oxygen tension is a factor in that polymer formation occurs only in the deoxy state. If oxygen is present,
the liquid state prevails. Concentration of Hb S is a factor in that gelation of HbS occurs at
concentrations greater than 20.8 g/dL (the normal cellular Hb concentration is 30 g/dL). The presence of
other hemoglobins is a factor in that normal adult hemoglobin (HbA) and fetal hemoglobin (HbF) have
an inhibitory effect on gelation.
These and other Hb interactions affect the severity of clinical syndromes. HbSS produces a more severe
disease than sickle cell HbC (HbSC), HbSD, HbSO Arab, and Hb with one normal and one sickle allele
(HbSA).
When red blood cells (RBCs) containing homozygous HbS are exposed to deoxy conditions, the sickling
process begins. A slow and gradual polymer formation ensues. Electron microscopy reveals a parallel
array of filaments. Repeated and prolonged sickling involves the membrane; the RBC assumes the
characteristic sickled shape. (See image below.)
After recurrent episodes of sickling, membrane damage occurs and the cells are no longer capable of
resuming the biconcave shape upon reoxygenation. Thus, they become irreversibly sickled cells (ISCs).
From 5-50% of RBCs permanently remain in the sickled shape.
When RBCs sickle, they gain Na+ and lose K+. Membrane permeability to Ca++increases, possibly due, in
part, to impairment in the Ca++ pump that depends on adenosine triphosphatase (ATPase). The
intracellular Ca++ concentration rises to 4 times the reference level. The membrane becomes more
rigid, possibly due to changes in cytoskeletal protein interactions; however, these changes are not found
consistently. In addition, whether calcium is responsible for membrane rigidity is not clear.
Membrane vesicle formation occurs, and the lipid bilayer is perturbed. The outer leaflet has increased
amounts of phosphatidyl ethanolamine and contains phosphatidylserine. The latter may play a role as a
contributor to thrombosis, acting as a catalyst for plasma clotting factors. Membrane rigidity can be
reversed in vitro by replacing HbS with HbA, suggesting that HbS interacts with the cell membrane.
Sickle cells express very late antigen4 (VLA-4) on the surface. VLA-4 interacts with the endothelial cell
adhesive molecule, vascular cell adhesive molecule1 (VCAM-1). VCAM-1 is upregulated by hypoxia and
inhibited by nitric oxide.
Hypoxia also decreases nitric oxide production, thereby adding to the adhesion of sickle cells to the
vascular endothelium. Nitric oxide is a vasodilator. Free Hb is an avid scavenger of nitric oxide. Because
of the continuing active hemolysis, there is free Hb in the plasma, and it scavenges nitric oxide, thus
contributing to vasoconstriction.
In addition to leukocyte recruitment, inflammatory activation of endothelium may have an
indispensable role in enhanced sickle RBCendothelium interactions. Sickle RBC adhesion in
postcapillary venules can cause increased microvascular transit times and initiate vaso-occlusion.
Several studies have shown involvement of an array of adhesion molecules expressed on sickle RBCs,
including CD36, a-4--1 integrin, intercellular cell adhesion molecule4 (ICAM-4), and basal cell adhesion
molecule (B-CAM).[6]Adhesion molecules (ie, P-selectin, VCAM-1, a-V--3 integrin) are also expressed
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on activated endothelium. Finally, plasma factors and adhesive proteins (ie, thrombospondin [TSP], von
Willebrand factor [vWf], laminin) play an important role in this interaction.
For example, the induction of VCAM-1 and P-selectin on activated endothelium is known to enhance
sickle RBC interactions. In addition, a-V--3 integrin is upregulated in activated endothelium in patients
with sickle cell disease. a-V--3 integrin binds to several adhesive proteins (TSP, vWf, red-cell ICAM-4,
and, possibly, soluble laminin) involved in sickle RBC adhesion, and antibodies to this integrin
dramatically inhibit sickle RBC adhesion.
In addition, under inflammatory conditions, increased leukocyte recruitment in combination with
adhesion of sickle RBCs may further contribute to stasis.
Sickle RBCs adhere to endothelium because of increased stickiness. The endothelium participates in this
process, as do neutrophils, which also express increased levels of adhesive molecules.
Deformable sickle cells express CD18 and adhere abnormally to endothelium up to 10 times more than
normal cells, while ISCs do not. As paradoxical as it might seem, individuals who produce large numbers
of ISCs have fewer vaso-occlusive crises than those with more deformable RBCs.
Sickle RBCs also adhere to macrophages. This property may contribute to erythrophagocytosis and the
hemolytic process.
The microvascular perfusion at the level of the pre-arterioles is influenced by RBCs containing Hb S
polymers. This occurs at arterial oxygen saturation, before any morphologic change is apparent.
Hemolysis is a constant finding in sickle cell syndromes. Approximately one third of RBCs undergo
intravascular hemolysis, possibly due to loss of membrane filaments during oxygenation and
deoxygenation. The remainder hemolyze by erythrophagocytosis by macrophages. This process can be
partially modified by Fc (crystallizable fragment) blockade, suggesting that the process can be mediated
by immune mechanisms.
Sickle RBCs have increased immunoglobulin G (IgG) on the cell surface. Vaso-occlusive crisis is often
triggered by infection. levels of fibrinogen, fibronectin, and D-dimer are elevated in these patients.
Plasma clotting factors likely participate in the microthrombi in the pre-arterioles.
Although hematologic changes indicative of SCD are evident as early as the age of 10 weeks, symptoms
usually do not develop until the age of 6-12 months because of high levels of circulating fetal
hemoglobin. After infancy, erythrocytes of patients with sickle cell anemia contain approximately 90%
hemoglobin S (HbS), 2-10% hemoglobin F (HbF), and a normal amount of minor fraction of adult
hemoglobin (HbA2). Adult hemoglobin (HbA), which usually gains prominence at the age of 3 months, is
absent.
The physiological changes in RBCs result in a disease with the following cardinal signs: (1) hemolytic
anemia; (2) painful vaso-occlusive crisis; and (3) multiple organ damage from microinfarcts, including
heart, skeleton, spleen, and central nervous system.
V. PATHOLOGICAL CONSEQUENCES

People with sickle cell disease (SCD) start to have signs of the disease during the first year of life, usually
around 5 months of age. Symptoms and complications of SCD are different for each person and can
range from mild to severe.

Hand-Foot Syndrome
Swelling in the hands and feet usually is the first symptom of SCD. This swelling, often along with a fever,
is caused by the sickle cells getting stuck in the blood vessels and blocking the flow of blood in and out of
the hands and feet.

Pain "Episode" or "Crisis"

Pain is the most common complication of SCD, and the top reason that people with SCD go to the
emergency room or hospital. When sickle cells travel through small blood vessels, they can get stuck and
clog the blood flow. This causes pain that can start suddenly, be mild to severe, and can last for any
length of time.

Anemia
Anemia is a very common complication of SCD. With SCD, the red blood cells die early. This means there
are not enough healthy red blood cells to carry oxygen throughout the body. When this happens, a
person might have: Tiredness, Irritability, Dizziness and lightheadedness, A fast heart rate, Difficulty
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breathing, Pale skin color, Jaundice (yellow color to the skin and whites of the eyes), Slow growth and
Delayed puberty

Infection
People with SCD, especially infants and children, are more at risk for infections, especially those due to
bacteria with capsules because of damage to the spleen. Pneumonia is a leading cause of death in
infants and young children with SCD.

Acute Chest Syndrome
This can be life-threatening and should be treated in a hospital. Symptoms and signs are similar to
pneumonia. Signs and symptoms include chest pain, coughing, difficulty breathing, and fever.

Splenic Sequestration
This can be life-threatening and should be treated in a hospital. It happens when a large number of
sickle cells get trapped in the spleen and cause it to suddenly get large. Symptoms include sudden
weakness, pale lips, fast breathing, extreme thirst, abdominal (belly) pain on the left side of body, and
fast heartbeat.
Parents of a child with SCD should learn how to feel and measure the size of their childs spleen and seek
help if the spleen is enlarged.

Vision Loss
Vision loss, including blindness, can occur when blood vessels in the eye become blocked with sickle
cells and the retina (the thin layer of tissue inside the back of the eye) gets damaged. Some patients
develop extra blood vessels in the eye from the lack of oxygen.

Leg Ulcers
This usually occurs on the lower part of the leg. They happen more often in males than in females and
usually appear from 10 through 50 years of age. A combination of factors cause ulcer formation,
including trauma, infection, inflammation, and interruption of the circulation in the smallest blood
vessels of the leg.

Stroke
A stroke can happen if sickle cells get stuck in a blood vessel and clog blood flow to the brain. About 10%
of children with SCD will have a symptomatic stroke. Stroke can cause learning problems and lifelong
disabilities.

Deep Vein Thrombosis (DVT) and Pulmonary Embolism (PE)
Sickling of red cells can increase blood coagulation and induce an increased risk of blood clot in a deep
vein (DVT), or in the lung (PE) if the blood clot moves from the deep veins. People with SCD have a high
chance of developing DVT or PE. DVT and PE can cause serious illness, disability and, in some cases,
death.

Other Possible Complications
Damage to body organs (like the liver, heart, or kidneys), tissues, or bones because not enough blood
is flowing to the affected area(s)." Insert as second bullet: "Malnutrition and growth retardation among
adolescents can cause a delayed onset of puberty and, in males, infertility.
Gallstones.
Painful erection of the penis, called priapism, can last less than 2 hours or more than 4 hours. If it lasts
more than 4 hours, the person should get urgent medical help. It can lead to impotence.

VI. DIFFERENTIAL DIAGNOSIS

Hemoglobin electrophoresis of cord blood can be used to screen newborns for SCD. The sickle
turbidity testa finger-stick test with a rapid resultis used to screen children ages 6 and up.
Hemoglobin electrophoresis can then confirm the diagnosis. DNA analysis provides the most accurate
diagnosis, but it's expensive and not widely available.
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Routine hematologic tests (CBC and ferritin levels) are done to evaluate anemia. The child's height
and weight are routinely measured to identify failure to thrive. Routine assessment includes a history of
pain and signs of infection and inflammation. As the child becomes older, genetic counseling will be
needed.

VII. MANIFESTATIONS AND SURVEILLANCE

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 to be hospitalized.
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.
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.

VIII. CLINICAL MANAGEMENT

Nursing Management

1. Manage acute crises
During an acute crisis, comfort measures, use of analgesics, and complementary approaches such as
massage and distraction are key. Healthcare providers focus on hydration, prevention of infections, and
early recognition of complications. Pharmacologic treatment is started immediately to manage pain.
Some individuals need blood transfusions to manage severe anemia. Indications for blood transfusions
include splenic sequestration, acute chest syndrome, severe anemia, and stroke.
2. Teach effective pain management measures.
Pain is a recurring factor in SCD. Pharmacologic and nonpharmacologic measures to control and/or
decrease the severity of pain episodes are essential. These include physical and occupational therapy,
physiotherapy, cognitive-behavioral therapy, and support groups. If your patient is a child, her parents
need to understand effective measures and provide them to control pain. Reading and watching
TV/videos can be effective distraction techniques. For the teen exploring hobbies, choosing physical
activities such as swimming, especially in a heated pool, can improve pain management and physical
stamina while limiting stress on joints.
3. Teach effective lifestyle modifications.
It's crucial that children and adults who have SCD learn effective lifestyle modifications. Parents need to
encourage their child to engage in normal childhood activities when possible. Play is crucial, but some
activities may need to be modified. For the teen, the task is to recognize what triggers a crisis, manage
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or eliminate these factors, and compensate for less appropriate activities with alternatives that bring a
sense of accomplishment and enjoyment (see Treating triggers).
4. Instruct when to contact the healthcare provider.
Symptoms to be reported include fever; swelling of the hands or feet; swelling of the abdomen and
lower left side with tenderness; pain in the joints, chest, or muscles; pale skin or nail beds or a yellowish
skin color (jaundice); sudden fatigue with less interest in surroundings; and an erection of the penis that
won't go away.
5. Teach stress management techniques.
Stress triggers sickle cell crises and decreases coping abilities. Learning how to effectively reduce stress
through breathing, muscle relaxation, meditation, and guided imagery can assist your patient in gaining
a sense of control. Maintaining a balance of nutrition, rest, and exercise is also important. A child with
SCD may not yet be able to identify how to effectively balance rest and exercise, so parents need to
manage this. A teen with SCD should be encouraged to participate in the process. Joining a support
group for people who have SCD or chronic diseases may be helpful. The use of social networking sites
such as Facebook and Twitter may decrease isolation and stress.
6. Encourage the acknowledgment of feelings.
Many people living with SCD find it helpful to line up supportive individuals, such as parents, teachers,
and friends. Others talk with a therapist or join a support group specifically for people with their
condition. It's important for your patient to acknowledge her emotions, talk about feelings of anger and
dependence, and recognize these as part of the illness. Encourage strategies that increase a sense of
control over your patient's life to decrease feelings of powerlessness and hopelessness. Families may
become overprotective of their children, stifling their growth and ability to manage their disease.
Adolescents, especially, need to be allowed to determine treatment strategies. Teens may be concerned
with, and need to discuss, body image issues related to delayed sexual maturity.
Cognitive-behavioral therapies teach coping skills and result in less negative thinking and even less pain.
Cognitive therapy helps a person identify thoughts, beliefs, and perceptions about a situation, as well as
how these impact behaviors. For example, pain can be perceived negatively as a sign of a problem or
positively as an indication that one's body recognizes the need to do something different. The goal is to
help patients adapt and learn to manage their illness rather than allowing the disease to control them.


Medical Management

Medicines and Fluids
Mild pain often is treated at home with over-the-counter pain medicines, heating pads, rest, and plenty
of fluids. More severe pain may need to be treated in a day clinic, emergency room, or hospital.
The usual treatments for acute (rapid-onset) pain are fluids, medicines, and oxygen therapy (if the
oxygen level is low). Fluids help prevent dehydration, a condition in which your body doesn't have
enough fluids. Fluids are given either by mouth or through a vein. Your doctor may prescribe antibiotics
if you have an infection. Treatment for mild-to-moderate pain usually begins with acetaminophen
(Tylenol

) or nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen.

If pain continues or becomes severe, stronger medicines called opioids might be needed. Talk with your
doctor about the possible benefits and risks of taking strong pain medicine, especially if the medicine
will be used for a long period.
Hydroxyurea
Severe sickle cell anemia can be treated with a medicine called hydroxyurea (hi-DROK-se-yu-RE-ah). This
medicine prompts your body to make fetal hemoglobin. Fetal hemoglobin, or hemoglobin F, is the type
of hemoglobin that newborns have. In people who have sickle cell anemia, fetal hemoglobin helps
prevent red blood cells from sickling and improves anemia. Taken daily by mouth, hydroxyurea reduces
how often painful sickle cell crises and acute chest syndrome occur. Many people taking hydroxyurea
also need fewer blood transfusions and have fewer hospital visits.
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Hydroxyurea can reduce the number of white blood cells in your blood, which can raise your risk for
infections. People who take hydroxyurea must have careful medical followup, including blood tests. The
dose of this medicine might need to be adjusted to reduce the risk of side effects. A doctor who has
knowledge about hydroxyurea can tell you about the risks and benefits of taking this medicine.
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.
Infections
Infections can be a major complication of sickle cell anemia throughout life, but especially during
childhood. Often, infections can be prevented or treated.
To prevent infections in babies and young children, treatments include:
Daily doses of antibiotics. Treatment may begin as early as 2 months of age and continue until
the child is at least 5 years old.
All routine vaccinations (including a yearly flu shot), plus the pneumococcal vaccine.
If your child has sickle cell anemia and shows early signs of an infection, such as a fever, you should seek
treatment right away. Adults who have sickle cell anemia also should have flu shots every year and get
vaccinated against pneumonia.
Eye Damage
Sickle cell anemia can damage the blood vessels in the eyes and the retinas. The retinas are the thin
layers of tissue at the back of the eyes. Regular checkups with an eye doctor who specializes in diseases
of the retina can help detect eye damage.
Strokes
Stroke prevention and treatment are now possible for children who have sickle cell anemia. Starting at
age 2, children who have sickle cell anemia should have routine ultrasound scans of the head. This is
called transcranial Doppler (TCD) ultrasound. These scans are used to check the speed of blood flow to
the brain. TCD scans allow doctors to find out which children are at high risk of stroke. Doctors can treat
these children with routine blood transfusions to reduce the risk of stroke. A doctor who has knowledge
about blood transfusions and sickle cell disease can tell you about the benefits and risks of this
treatment.
Treating Other Complications
Acute chest syndrome is a severe and life-threatening complication of sickle cell anemia. If acute
(sudden) failure of the liver and kidneys also occurs, it's called acute multiple organ failure.
Treatment for these complications usually occurs in a hospital and may include oxygen therapy, blood
transfusions, antibiotics, pain medicine, and balancing body fluids.
Leg ulcers (sores) due to sickle cell anemia can be very painful. Ulcers can be treated with cleansing
solutions and medicated creams or ointments.
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Skin grafts might be needed if the leg ulcers are ongoing. Bed rest and keeping the legs raised to reduce
swelling are helpful. If you have a lot of pain from leg ulcers, your doctor may recommend a strong pain
medicine.
Your doctor might recommend gallbladder surgery if the presence of gallstones leads to gallbladder
disease.
Priapism (a painful erection in males) can be treated with fluids, medicines, or surgery.
Regular Health Care for Children
Children who have sickle cell anemia need routine health care (just like children who don't have the
disease). They need to have their growth checked regularly. They also need to get the routine shots that
all children get.
All children younger than 2 years old should see their doctors often. Children who have sickle cell
anemia may need even more checkups. After age 2, children who have sickle cell anemia may not need
to see their doctors as often, but they usually still need checkups at least every 6 months.
These visits are a time for parents to talk with their child's doctor and ask questions about the child's
care. Talk with your child's doctor about eye checkups and whether your child needs an ultrasound scan
of the brain.
Until age 5, daily penicillin is given to most children who have sickle cell anemia. Doctors also give many
children a vitamin called folic acid (folate) to help boost red blood cell production.
Young children who have sickle cell anemia should have regular checkups with a hematologist (a blood
specialist).
New Treatments
Research on blood and marrow stem cell transplants, gene therapy, and new medicines for sickle cell
anemia is ongoing. The hope is that these studies will provide better treatments for the disease.
Researchers also are looking for a way to predict the severity of the disease.
Blood and Marrow Stem Cell Transplant
A blood and marrow stem cell transplant can work well for treating sickle cell anemia. This treatment
may even offer a cure for a small number of people.
The stem cells used for a transplant must come from a closely matched donor. The donor usually is a
close family member who doesn't have sickle cell anemia. This limits the number of people who may
have a donor.
The transplant process is risky and can lead to serious side effects or even death. However, new
transplant approaches may improve treatment for people who have sickle cell anemia and involve less
risk.
Blood and marrow stem cell transplants usually are used for young patients who have severe sickle cell
anemia. However, the decision to give this treatment is made on a case-by-case basis.
Researchers continue to look for sources of bone marrow stem cellsfor example, blood from babies'
umbilical cords. They also continue to look for ways to reduce the risks of this procedure.
Gene Therapy
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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.
IX. CONCEPTUAL MODELS/ FRAMEWORK

Orem's Self-Care Deficit Theory of NursingAccording to Polit & Henderson, each individual has the ability
to perform self-care, and are responsible for their health and the health of their dependents. Self-care is
"the practice of activities that individuals initiate and perform on their own behalf in maintaining life,
health, and well-being" (as cited in Cardinal Stritch University Library, 2011).

Orem's initial definition of nursing's concern included "man's need for self-care action and the provision
and management of it on a continuous basis in order to sustain life and health, recover from disease or
injury, and cope with their effects" (Orem, 1959, p. 3).

More simply stated, her definition of nursing's goal was "overcoming human limitations" (Orem, 1959,
p. 4) Orem's general theory of self-care deficit nursing states that a self-care demand exists when the
patient of family members are not able to provide care for him/herself and those self-care demands can
be met by the nurse (Masters, 2011). According to Sitzman & Eichelberger (2011), "Orem's Self-Care
Model describes a structure wherein the nurse assists the client, where needed, to maintain an
adequate level of self-care. The degree of nursing care and intervention depends on the degree to which
the client is able (or unable) to meet self-care needs (p. 96)." Orem's theory is made up of 3 related
theories: 1. Theory of Self-Care 2. Theory of Self-Care Deficit 3. Theory of Nursing Systems

According to Orem, "the three-part theory focuses not on individuals, but on persons in relations. Each
of the three theories has as its focus a specific dimension of the person: the theory of self-care focuses
on the self, the I; the theory of self-care deficit focuses on you and me; and the theory of nursing system
focuses on we, persons in community" (1990, p. 49). The theory of self care delineates the demands of
self-care requisites that an individual needs to meet in order to stay healthy or get better from illness.
When a person cannot meet these self-care demands, or when self-care requisites exceeds self-care
capabilites, nursing is needed. Nurses can help patients meet their self-care needs by utilizing the 5
methods in the Theory of Self Care Deficit. However, the nurse must properly assess the level of self-
care a patient requires so that the nurse can utilize the nursing system (as stated in the Theory of
Nursing System) that best fits the patient's ability to perform self-care. In the end, the nurse assists the
patient in meeting his/her self-care needs so that he/she is healthy and free of illness.

X. QUESTIONS FOR FUTURE STUDIES

XI. REFERENCE

Centers for Disease Control and Prevention from
http://www.cdc.gov/ncbddd/sicklecell/treatments.html
Mayo clinic from
Sickle Cell Disease AAHN1



11
http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/complications/con-
20019348
Medscape from
http://emedicine.medscape.com/article/205926-overview#aw2aab6b2b7aa
National Institutes of Health from
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/names.html
NHS choices from
http://www.nhs.uk/Conditions/Sickle-cell-anaemia/Pages/Introduction.aspx
nurseslabs from
http://nurseslabs.com/sickle-cell-anemia-nursing-management/