Autosomal recessive. Abnormal HbS causes sickled RBCs, leading to vaso-occlusion and hemolysis.

Hypoxia, dehydration, infection, acidosis, extreme temps, stress, anesthesia.

Vaso-occlusive Crisis

Severe pain due to sickled cells blocking blood flow. Hallmark of sickle cell disease.

Lung occlusion by sickled cells. Sx: chest pain, fever, cough, hypoxia, infiltrates on CXR.

Sickled cells trapped in spleen/liver. Causes rapid organ enlargement, pain, ↓Hb.

Hb electrophoresis shows HbS. ↓Hb/Hct, ↑reticulocytes, ↑WBC/plts, signs of hemolysis.

Sickle Cell Management

Hydration (3-4L), O2, pain control, folic acid, hydroxyurea, antibiotics, vaccines.

Increases fetal hemoglobin (HbF), reduces sickling. Teratogenic. Monitor CBC for leukopenia.

Avoid triggers, encourage fluids, no caffeine, extend extremities during pain, no Trendelenburg.

Prolonged, painful erection due to sickling in penile vessels. Requires emergent urologic intervention.

Due to vascular occlusion. Requires regular screening (transcranial Doppler) in children.

Chronic Complications

Skin ulcers, avascular necrosis, retinopathy, renal disease, cardiomegaly, gallstones.

Spleen Function in SCD

Autoinfarction leads to functional asplenia by adulthood, ↑risk for infection (encapsulated bacteria).

Due to chronic hemolysis of fragile sickle cells, leading to elevated unconjugated bilirubin.

Heart enlarges due to chronic anemia and increased workload to maintain oxygenation.

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Sickle Cell Anemia

Question	Answer
Sickle Cell Patho	Autosomal recessive. Abnormal HbS causes sickled RBCs, leading to vaso-occlusion and hemolysis.
Sickle Cell Triggers	Hypoxia, dehydration, infection, acidosis, extreme temps, stress, anesthesia.
Vaso-occlusive Crisis	Severe pain due to sickled cells blocking blood flow. Hallmark of sickle cell disease.
Acute Chest Syndrome	Lung occlusion by sickled cells. Sx: chest pain, fever, cough, hypoxia, infiltrates on CXR.
Sequestration Crisis	Sickled cells trapped in spleen/liver. Causes rapid organ enlargement, pain, ↓Hb.
Sickle Cell Labs	Hb electrophoresis shows HbS. ↓Hb/Hct, ↑reticulocytes, ↑WBC/plts, signs of hemolysis.
Sickle Cell Management	Hydration (3-4L), O2, pain control, folic acid, hydroxyurea, antibiotics, vaccines.
Hydroxyurea	Increases fetal hemoglobin (HbF), reduces sickling. Teratogenic. Monitor CBC for leukopenia.
Sickle Cell Nursing	Avoid triggers, encourage fluids, no caffeine, extend extremities during pain, no Trendelenburg.
Priapism	Prolonged, painful erection due to sickling in penile vessels. Requires emergent urologic intervention.
Stroke Risk in SCD	Due to vascular occlusion. Requires regular screening (transcranial Doppler) in children.
Chronic Complications	Skin ulcers, avascular necrosis, retinopathy, renal disease, cardiomegaly, gallstones.
Spleen Function in SCD	Autoinfarction leads to functional asplenia by adulthood, ↑risk for infection (encapsulated bacteria).
Jaundice in SCD	Due to chronic hemolysis of fragile sickle cells, leading to elevated unconjugated bilirubin.
Cardiomegaly in SCD	Heart enlarges due to chronic anemia and increased workload to maintain oxygenation.

Created by: Wasurenboh

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