Pathophysiology - Sickle Cell Disease Flashcards
1
Q
Explain the genetic changes that result in sickle cell disease.
A
- sickle cell disease (SCD) is an autosomal recessive disease
- chromosome 11, p arm sees a change from Glutamate to Valine in Hb
- Hb changes from HbA (adult) to HbS (sickle cell)
- This causes RBCs to “sickle”
- SCs cannot deform properly through the microvasculature and produce pain/ischemia
2
Q
What is the average life span of a sickled RBC?
A. 10-20 days
B. 30-90 days
C. 90-120 days
A
A. 10-20 days
3
Q
What is the average life span of a normal RBC?
A. 10-20 days
B. 30-90 days
C. 90-120 days
A
C. 90-120 days
4
Q
What is a common description of SCD pain?
A
“intense, sharp, throbbing”
5
Q
What findings commonly occur in the physical exam of the SCD patient?
A
- Hepato/splenomegaly
- Hepatomegaly may accompany jaundice
6
Q
What is the cause of SCD pain?
A
Ischemia in the microvasculature
