Sickle cell anemia

• The defect in β -chain, position 6 → glutamic acid substituted by valine

• 3 types of crisis
o hemolytic→ further anemia
o sickling → pain & vaso-occlusive
o Aplastic → may cause death.
• Ethnic background: Mediterranean and African-American.
• In infancy Hb F is protective.
• % of Hb S
o trait (mild)
o < 50% is Hb S
o sickling do not occur under normal physiological conditions
o may happen under extreme conditions e.g severe hypoxemia.
o Disease (severe)
o Hb S > 75% may up to 95%
o Could be associated with other abnormal Hb e.g Hb C
o Pt with Hb SC have a normal Hb level, but at a greater risk of sickling.
• Pre-op you need to knew what is the result of the Hb electrophoresis.
• ↑ mortality with ↑ in the number of the painful crisis in adult per-year.
• O2-Hb curve shifted to the Right, with P50 of 31 mmHg.
• The sickle cells are more fragile with shorter life span
• Pt may treated with Hydroxy-urea to ↑ Hb F level, pre-op
• ↓ peri-op risk with ↑ Hb F level
• Pre-op Mx:
o Traditional approach:
o PRBC over several days pre-op→ ↓ erythropiosis→ ↓ retics count → this will
↓ Hb S, and it may fall below 40%
o Conservative approach:
o Traetment will be given when necessary to ↑ Hb > 10
o It is effective as the traditional, and may avoid risk of transfusion.
• Anesthesia Consideration:
A →OSA→ due to tonsiller hypertrophy
B →Acute chest syndrome
 Lung infarction and fibrosis→ Cor-pulmonal
 Pneumonia
C→ MI, RV & LV dysfunction → CHF
 GI-Hepatic→ mesenteric ischemia, gall stone, Aotu-splenectomy → risk of
infection ( pneumococcal vaccine)
Renal →CRF due to multi-infarction.
Obstetrics→↑ risk of pre-term labor,↑ perinatal mortality,↑ risk of placenta
previa and abruption placenta.
CNS→stroke, seizure , and intracranial hemorrhage
MSK → ulcers and myonecrosis→ risk of ischemia with position
Metabolism→↓ cholinesterase level
• Crisis ↑ by
o Dehydration.
o Hypoxia
o Acidosis
o Hypothermia and fever.
• What to do?
o ↑ FiO2
o hydrate well
o monitor acid-base status
o maintain normothermia
• tourniquet ??
o if yes→ exsanguinate the limb well and minimize the tourniquet time.
• Anticipated problems:
o Crisis→ hemolytic, ischemic
o transfusion reaction→ hemolytic, due to multi-Tx → allu-immunization.