Autosomal dominant genetic disorder leading to sickling and hemolysis
Precipitants include hypoxemia, hypovolemia, infection, other --> sickling--> veno-occlusive disease--> end organ ischemia
Risk Factors
African descent
Family hx
Sx worse if air quality poor
Symptoms
Recurrent bouts of pain during crises due to veno-occlusive disease
Variable severity based on genotype--> degree of sickling
Acute chest syndrome: Sob/CP from ischemia, infection, fat embolism, hemolysis
Acute priapism, abdominal pain (ischemia)
Chronic sx: multi-site pain, leg ulcers, sepsis from fxnl asplenia, joint pain from avascular necrosis, stroke, biliary colic or cholecystitis from bilirubin gall stones; sob from restrictive lung dz, plum htn