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Sickle Cell Anemia

Sickle Cell Anemia

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Published by hollyu

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Published by: hollyu on Nov 19, 2009
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05/18/2012

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 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

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