Chapter 31
Hematology
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Objectives
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Sickle Cell Disease
• Sickle cell disease
– Inherited disorder that affects red blood
cells
– Sickle cell trait
– Mainly affects African Americans and
Hispanic Americans
– Average life expectancy is about 45 years
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Normal Red Blood Cells
• Bone marrow produces red blood cells
• Normal red blood cells
– Smooth, round
– Move easily through blood vessels
– Live about 120 days
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Sickle Cell Disease
• Body produces a defective hemoglobin
molecule
• Red blood cells become stiff and shaped like
a sickle, or “C”
• Sickled red blood cells usually die after
about 10 to 40 days
• Sickle cell anemia
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Sickle Cell Crisis
• Sickled red blood cells
– Carry less hemoglobin than normal RBCs
– Do not move easily through blood vessels
– Have a tendency to stick together and
form clumps
• Sickle cell crisis
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Assessment Findings and Symptoms
• Signs and symptoms
usually do not develop
until after age 4 months
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Assessment Findings and Symptoms
• Fatigue
– Most common symptom of sickle cell
anemia
• Pain of sickle cell crisis
– Acute (sudden)
• Mild to severe
• Usually lasts hours to several days
– Chronic (long-term)
• Typically lasts for weeks to months
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Assessment Findings and Symptoms
• Hand-foot syndrome
– Results from the blockage of blood
vessels in the hands or feet by sickle cells
– Painful swelling
– Affect one or both hands and/or feet at the
same time
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Assessment Findings and Symptoms
• Ulcers (sores) develop on the lower third of
the leg in some patients with sickle cell
disease.
• The cause is unknown.
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Assessment Findings and Symptoms
• Acute chest syndrome
– Occurs because of an infection or
blockage of the blood vessels in the lungs
by blood clots or sickled red blood cells
– Signs and symptoms
•
•
•
•
Fever
Chest pain
Coughing
Difficulty breathing
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Assessment Findings and Symptoms
• Splenic crisis
– Sickled red blood cells become trapped in
the spleen
– A leading cause of death in children with
sickle cell disease
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Assessment Findings and Symptoms
• Scene size-up
• Ensure your safety
• Put on appropriate PPE
• Request advanced life support personnel
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Assessment Findings and Symptoms
• Primary survey
• Position of comfort
• Perform a physical examination
• Vital signs, medical history
• Reassess as often as indicated
• Document
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Hemophilia
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Hemophilia
• Hemophilia
– Inherited bleeding disorder
– Caused by an abnormality of a bloodclotting factor
– Can be acquired if an individual forms
antibodies to the clotting factors in his or
her bloodstream
– Usually occurs in males
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Assessment Findings and Symptoms
• Primary signs and symptoms
– Easy bruising
– Excessive bleeding
• External
• Internal
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Assessment Findings and Symptoms
• Internal bleeding
– Is not visible
– Occurs inside body tissues and cavities
– Bleeding in the brain
– Bleeding into the muscles and joints
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Emergency Care
• Scene size-up
• Ensure your safety
• Put on appropriate PPE
• Make sure suction is within arm’s reach
at all times
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Emergency Care
• Perform a physical examination
• Vital signs and medical history
• Assess carefully for signs of bruising,
obvious bleeding, and signs that suggest
internal bleeding
• Control external bleeding, if present.
• Reassess as often as indicated
• Record all patient care information on a PCR.
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Questions?
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