Sickle Cell Disease

Question 1

Sickle Cell Disease: Pathogenesis

Answer 1

Hemoglonipathy

Mutation chromosome 11 substitution V for G in beta hemoglobin chain (HbS)

Decreased O2 tension - sickle shape - hemolysis, microvascular occlusion, ischemia, hemolytic crisis

Question 2

Sickle Cell Disease: Comorbidities

Answer 2

Cardiac- cardiomegaly, CHF,

Resp- pulmonary HTN, acute chest syndrome

Neuro- deficits, painful crises

Renal- CKD/CRF

Other- retinopathy, aseptic necrosis of femoral head, asplenia (encapsulated organism infxns)

Question 3

Sickle Cell Disease: Exchange Transfusion

Answer 3

Reduces fraction Hgb S to <40%
Not advised, multiple transfusions = risk transfusion-related complications
However, important ensure adequate hemoglobin
Therefore, require preop Hct 30%

Question 4

Sickle Cell Disease: Sickle Prevention

Answer 4

Avoid hypoxemia, hypotension, hypothermia, acidosis, hypovolemia
Provide supplemental O2, IVF for hydration, maintain Hct 30-40%, fluid warmers/forced air warming units, provide adequate postop pain control, avoid hypoventilation

Question 5

Sickle Cell Crisis: Treatment

Answer 5

Pain control, oxygen, hydration, treat infxn, adequate Hct/exchange transfusion