Sickle Cell Anemia

SICKLE CELL ANEMIA
BY AISHA TAMBWE
OBJECTIVES
At the end of the session students should be able to
Define sickle cell
Risk factors
Pathophysiology
Clinical manifestations
Investigations
Medical & nursing management
INTRODUCTION
• Hemoglobin is the main substance of the red blood cell. It helps red
blood cells carry oxygen from the air in our lungs to all parts of the
body. Normal red blood cells contain hemoglobin A. Normal red
blood cells that contain hemoglobin A are soft and round and can
squeeze through tiny blood tubes (vessels). Normally, red blood cells
live for about 120 days before new ones replace them.
RED BLOOD CELL
INTRODUCTION
• People with sickle cell conditions make a different form of
hemoglobin A called hemoglobin S (S stands for sickle).
INTRODUCTION
• Sickle cell disease is found predominantly in people of African descent
and less often in people who have descended from the
Mediterranean countries, the Middle East, or aboriginal tribes of
India.
• The HbS gene is inherited, with some people having the sickle cell
trait (a carrier, inheriting one abnormal gene) and some having sickle
cell disease (inheriting two abnormal genes).
INTRODUCTION
Examples:
• If one parent has Sickle Cell Anemia (SS disease) and the other is
Normal, all of the children will have sickle cell trait.
• If one parent has Sickle Cell Anemia (SS) and the other has Sickle Cell
Trait (AS), there is a 50% chance (or 1 out of 2) of having a baby with
either sickle cell disease or sickle cell trait with each pregnancy.
• When both parents have Sickle Cell Trait, they have a 25% chance (1
of 4) of having a baby with sickle cell disease with each pregnancy.
PATHOPHYSIOLOGY
• The defective hemoglobin molecule assumes a sickle shape when
exposed to low oxygen tension. These long, rigid RBCs become lodged
in small vessels and can obstruct blood flow to body tissue. If
ischemia or infarction results, the patient may have pain, swelling,
and fever.
PATHOPHYSIOLOGY
• The sickling process takes time; if the erythrocyte is again exposed to
adequate amounts of oxygen (eg, when it travels through the
pulmonary circulation) before the membrane becomes too rigid, it
can revert to a normal shape. For this reason, the “sickling crises” are
intermittent
CLINICAL MANIFESTATION
Symptoms of sickle cell anemia vary and are only somewhat based on
the amount of HbS. Symptoms and complications result from chronic
hemolysis or thrombosis.
• Anemia, with hemoglobin values in the range of 7 to 10 g/dL.
• Jaundice is characteristic, usually obvious in the sclera.
• Bone marrow expands in childhood, sometimes causing enlargement
of bones of the face and skull.
• Tachycardia, cardiac murmurs, and often cardiomegaly are associated
with chronic anemia.
• Dysrhythmias and heart failure may occur in adults.
• Virtually any organ may be affected by thrombosis, but the primary
sites involve those areas with slower circulation, such as the spleen,
lungs, and central nervous system.
• There is severe pain in various parts of the body. All tissues and
organs are vulnerable and susceptible to hypoxic damage or ischemic
necrosis.
• Sickle cell crisis: sickle crisis, aplastic crisis, or sequestration crisis.
• Acute chest syndrome: fever, cough, tachycardia, and new infiltrates
seen on the chest x-ray.
• Pulmonary hypertension is a common sequela of sickle cell disease,
and often the cause of death.
Assessment and Diagnostic Findings
The patient with sickle cell trait usually has a normal hemoglobin level,
a normal hematocrit, and a normal blood smear.
In contrast, the patient with sickle cell anemia has a
• Low hematocrit level .
• Sickled cells on the smear.
• The diagnosis is confirmed by hemoglobin electrophoresis
MEDICAL MANAGEMENT
• Transfusion therapy: Has been shown to be highly effective in several
situation.
• Fluid restriction may be beneficial. Corticosteroids may be useful.
• Folic acid is administered daily for increased marrow requirement.
• Supportive care involves pain management (aspirin or NSAIDs,
morphine, and patient-controlled analgesia), oral or IV hydration,
physical and occupational therapy, physiotherapy, cognitive and
behavioral intervention, and support groups.
NURSING DIAGNOSES
• Acute pain related to tissue hypoxia due to agglutination of sickled
cells within blood vessels.
• Risk for infection.
• Risk for powerlessness related to illness-induced helplessness.
• Deficient knowledge regarding prevention of crisis.
NURSING MANAGEMENT
Managing Pain
• Use patient’s subjective description of pain and pain rating on a pain
scale to guide the use of analgesic agents.
• Support and elevate any joint that is acutely swollen until swelling
diminishes.
Preventing and Managing Infection
• Monitor patient for signs and symptoms of infection.
• Initiate prescribed antibiotics promptly.
NURSING MANAGEMENT
Promoting Coping Skills
• Enhance pain management to promote a therapeutic relationship
based on mutual trust.
• Focus on patient’s strengths rather than deficits to enhance effective
coping skills.
Increasing Knowledge
• Teach patient about situations that can precipitate a sickle cell crisis
and steps to take to prevent or diminish such crises (eg, keep warm,
maintain adequate hydration, avoid stressful situations).
NURSING MANAGEMENT
Monitoring and Managing Potential Complications
• Protect the leg from trauma and contamination.
• Use aseptic technique to prevent nosocomial infections.
Potential Complications
• Hypoxia, ischemia, infection, and poor wound healing leading to skin
breakdown and ulcers.
• Dehydration .
• Cerebrovascular accident (CVA, brain attack, stroke) .
• Anemia.
• Acute and chronic renal failure.
• Heart failure
• Pulmonary hypertension..
Potential Complications
• Impotence .
• Poor compliance .
• Substance abuse related to poorly managed chronic pain .
• Acute chest syndrome .
Assignment
• Define thalassemia
• Identify different types of sickle cell.
• Describe sickle cell crisis and its management.

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