Sickle Cell Anemia

Question 1

What type of inheritance is sickle cell anemia?
what is sickle cell anemia?

Answer 1

Autosomal recessive
C11 mutated CAG to CTG on 6th codon vs malaria

Question 2

Sx of sickle cell anemia?
what happens in sickle cell crisis?

Answer 2

Manifests when low HbF so around 6 months
General anemia Sx
Crisis (triggers = cold, infection, dehydration)

sickle chest
vaso occlusive
splenic sequestration

Question 3

Acute crisis for sickle cell Tx?

Answer 3

Morphine
BB
O2
Abx prophylaxis

Question 4

is a Px with osteomyelitis has sickle cell anemia, what will be the cause?

Answer 4

Salmonella

Question 5

Dx of sickle cell anemia?

Answer 5

Normocytic normochronic anemia
high reticuloblasts
GS = Hb electrophoresis (HbS)

Screening = Day 5-9 Guthrie heel prick

Question 6

Tx of sickle cell anemia?

Answer 6

Transfusion
Splenectomy
Folate sumps
Fe chelation
BM Transplant

Question 7

what does sickle cell increase risk of?

Answer 7

stroke and neonatal jaundice