Sickle Cell Disease

Question 1

what is sickle call disease?

Answer 1

disease results from a mutation in the Beta-globin gene

Question 2

what are the 5 characteristics of all sickle cell based diseases?

Answer 2

• vaso-occlusive events
• hemolytic anemia
• vasculopathy (blood vessel disease)
• widespread organ damage
• early mortality

Question 3

Hb-S form of hemoglobin belongs to what disease?

Answer 3

sickle cell disease

Question 4

is a result of glutamic acid replaced by valine on chromosome 11

Answer 4

sickle cell hemoglobin (HbS)

Question 5

what causes the painful episodes, acute chest syndrome, and osteonecrosis associated with sickle cell?

Answer 5

increased viscosity

Question 6

how is sickle cell anemia diagnosed?

Answer 6

genetic testing to determine which chromosomes are changed

Question 7

what can mask the effects of HbS?

Answer 7

increased concentrations of HbF

Question 8

osteonecrosis due to sickle cell anemia is usually at which 2 joints?

Answer 8

hip and shoulder

Question 9

what might you see on a blood smear of a patient with suspected sickle cell anemia?

Answer 9

sickled cells
target cells
nucleated RBCs
Howell-Jolly bodies

Question 10

why are vaccinations important in people who have diagnosed sickle cell disease?

Answer 10

they are more susceptible to infections and illnesses

Question 11

what is the most common treatment of sickle cell disease?

Answer 11

hydroxyurea to increase HbF