Sickle Cell Anemia

Sickle cell anemia is a severe hemolytic anemia resulting from the

inheritance of the sickle hemoglobin gene (HbS), which cause a
defective hemoglobin molecule.

Risk of Inheritance
 African-Americans mostly
 Aboriginal tribes of India

Clinical Manifestations
Symptoms and complications result from chronic hemologysis or
thrombosis.

 Anemia, with hemoglobin value in the 7 to 10 g/dL range.

 Jaundice is characteristic, usually obvious in the sclera.
 Bone marrow expands in childhood, sometimes causing
enlargement of bone of the face and skull.
 Tachycardia, cardiac murmurs and often cardiomegaly are
associated with chronic anemia.
 Dsyrhytmias and heart failure may occur in adults.
 There is severe pain in various parts of the body. All tissues and
organ are vulnerable and susceptible to hypoxic damage or true
ischemic necrosis at any time.
 Sickle cell crisis: painful crisis, aplastic crisis, or sequestration
crisis.

1
  Acute chest syndrome: rapidly falling hemoglobin level,
tachycardia, fever and bilateral infiltrates seen on chest
radiographs.

Diagnostic Evaluation
 Hemoglobin and hematocrit levels, blood smear (usually normal)
 Diagnosis is confirmed with hemoglobin electrophoresis
 Isoelectric focusing, high-performance liquid chromatography
techniques
 Sickling occurs whether patient has sickle trait or sickle cell
anemia; only electrophoresis shows a distinction

Medical Management
 Many antisickling drugs are undergoing trials; currently
hydroxyurea has been shown to be effective (increases fetal
hemoglobin production).
 Bone marrow transplantation is a potential cure but is limited
because of lack of donors or organ damage.
 Chronic transfusions with red blood cells are done for exacerbation
of anemia, for prevention of complications, or to improve response
to infection.
 Pulmonary function is monitored, pulmonary hypertension is
treated early, if found.
 Infections and acute chest syndrome, which predispose to crises,
are treated promptly.
 Incentive spirometry is performed to prevent pulmonary
complications; Bronchoscopy is done to identify source of
pulmonary disease.
2
  Fluid restriction (not addressive hydration) may be beneficial;
steroids may be useful.
 Folic acid therapy is administered daily for increased marrow
requirement.
 Supportive care involves pain management, oral or intravenous
hydration, supplemental oxygen, physical and occupational
therapy, physiotherapy, cognitive and behavioral intervention, and
support groups.

Assessment
 Question patients in crisis about factors that could have
precipitated the crisis and measures used to prevent crises.
 Assess all body system, with particular emphasis on pain, swelling,
and fever.
 Elicit symptoms of cerebral hypoxia by careful neurologic
examination.
 Carefully assess respiratory system including breath sound and
oxygen saturation levels.
 Assess for signs of cardiac failure by radiography.
 Assess for signs of dehydration and history of fluid intake.
 Assess current and past medical management particularly chronic
transfusion therapy, hydroxyurea use and prior treatment for
infection.
 Assess for the presence of any infectious process.
 Monitor hemoglobin and hematocrit, and reticulocytes count and
compare with baseline levels.

Major Nursing Diagnoses

3
  Pain related to tissue hypoxia due to agglutination of sickled cell
within blood vessels
 Risk for infection
 Powerlessness related to illness-induced helplessness
 Knowledge deficit regarding prevention of crisis

Collaborative problems/Potential Complications

 Hypoxia, ischemia, infection, and poor wound healing leading to
skin breakdown and ulcers
 Dehydration
 Cerebrovascular accident (stroke)
 Anemia
 Heart failure, pulmonary hypertension, and acute chest syndrome
 Impotence, priapism
 Renal dysfunction
 Substance abuse related to chronic pain
 Poor compliance

Nursing interventions
MANAGING PAIN

 Use the patient’s subjective description of pain and pain rating on a

pain scale to guide the use of analgesics.
 Support and elevate any joint that is acutely swollen until the
swelling diminishes.

4
  Teach the patient relaxation technique, breathing exercises, and
distraction to help the pain.
 When the acute painful episode has diminished, implement
aggressive measures to preserve function (physical therapy,
whirlpool baths, and transcutaneous nerve stimulation).
PREVENTING AND MANAGING INFECTION

 Monitor the patient for signs and symptoms of infection.

 Initiate prescribed antibiotics promptly.
 Assess the patient for signs of dehydration.
 Teach the patient to take prescribed oral antibiotics at home, if
indicated, emphasizing the need to complete of antibiotic therapy;
assist the patient to identify a feasible administration schedule.
PROMOTING COPING SKILL

 Enhance pain management to promote a therapeutic relationship

based on mutual trust.
 Focus on the patient’s strengths rather than deficits to enhance
effective coping skills.
 Provide opportunities for the patient to make decisions about daily
care to increase feelings of control.
MINIMIZING KNOWLEDGE DEFICIT

 Teach the patient with sickled cell anemia the situation that can
precipitate a sickled cell crisis and step they can take to prevent or
diminished such crises (keep warm, maintain adequate hydration,
avoid stressful situation).

PROMOTING SELF CARE

5
LEG ULCER
PRIAPISM LEADING TO IMPOTENCE
CHRONIC PAIN AND SUBSTANCE ABUSE