AG2- Sickle cell disease and Thalassemia Flashcards
What is Sickle cell disease (SCD)?
abnormal form of Hgb in erythrocytes causing them to sickle
What are types of SCD?
sickle cell anemia, sickle cell thalassemia, sickle cell Hgb C disease, and sickle cell trait
What are sickling episodes most commonly triggered by?
Low oxygen tension in the blood
What are findings that hemolysis of RBCs is occuring?
jaundice, elevated bilirubin levels, decreased levels of Hgb, reticulocytes, and folate.
What changes occur in the spleen with SCD?
becomes smaller and nonfunctional because of repeated scarring
What symptoms may a person experience during a sickle cell crisis?
severe pain, fever, swelling, tenderness, tachypnea, HTN, N/V
Possible complication of lack of oxygen to the kidneys?
AKI
Pulmonary complications associated with SCD?
PNA, tissue infarction, fat embolism, MI, cor pulmonale
Cardiovascular complications of SCD?
heart becomes ischemic and enlarged, leading to heart failure
What is Thalassemia?
group of diseases involving inadequate production of normal Hgb and therefore a decrease in erythrocyte production
Symptoms of Thalassemia minor?
frequently asymptomatic and mild to moderate anemia with microcytosis (small cells) and hypochromia (pale cells)
Symptoms of Thalassemia major?
delayed physical and mental development, pallor, splenomegaly, hepatomegaly, and jaundice from hemolysis of RBCs, chronic bone marrow hyperplasia causing expansion of the marrow space, thickening of the cranium and maxillary cavity.
Why are fluids and electrolytes important in the management of SCD and SCC?
to reduce blood viscosity and maintain renal function
Ways to avoid a sickle cell crisis?
avoid dehydration and hypoxia
How is thalassemia major treated?
with blood transfusion or exchange transfusions in conjunction with oral deferasirox or IV or subcut deferoxamine to reduce the iron overloading that occurs with frequent transfusion therapy.
