SCD

Question 1

routine newborn screening for SCD

Answer 1

Hbg S

Question 2

if newborn screening is + a ________ test is done

Answer 2

second

Question 3

sickling is caused by a mutation in the gene that tells the body to make

Answer 3

hemoglobin

Question 4

overall goal of helping patients with SCD

Answer 4

improve tissue perfusion to organs, tissues, and nerves

Question 5

pain due to

Answer 5

occlusion

Question 6

what makes the cells have normal shape again

Answer 6

oxygenate and hydrate

Question 7

clinical manifestations do not occur in children until?

Answer 7

4-6 months

Question 8

dactylitis

Answer 8

inflammation of digits

Question 9

AVN (avascular necrosis) of the ________ is a common complication in SCD

Answer 9

femoral head

Question 10

the collapse of the femoral head occurs in 90% of the patients within

Answer 10

5 years of diagnosis of osteonecrosis

Question 11

what triggers crisis of SCD

Answer 11

stress (dehydration, infection, mental, stress)

Question 12

cardio pulmonary complications

Answer 12

acute chest syndrome (life threatening), chronic lung disease (which can lead to pulmonary HTN)

Question 13

priapism

Answer 13

painful long lasting erections as blood is trapped in the erection chambers

Question 14

ways to fix priapism

Answer 14

intra cavernous injections to narrow and decrease blood flow to the penis and aspirate blood from the penis

Question 15

bone marrow slows its production or RBCs due to infection. Decrease in RBCs causing paleness, fatigue, and rapid pulse. Can be fatal

Answer 15

aplastic crisis

Question 16

organs are deprived of blood and oxygen (cells become trapped in the organs and occlude blood flow)

Answer 16

organ damage

Question 17

complications

Answer 17

CNS (stroke), cardio pulmonary, gallstones, priapism, leg ulcers, stroke, acute chest syndrome, pulmonary hypertension, organ damage, aplastic crisis, vision problems, frequent infections

Question 18

blood pools in spleen

Answer 18

splenic sequestration crisis

Question 19

SCD symtoms

Answer 19

jaundice, fatigue, back pain, arm and leg pain, foot swelling, joint pain, hand swelling, breathlessness

Question 20

transfusion therapy used to

Answer 20

treat certain complications and prevent complications

Question 21

analgesic meds

Answer 21

demerol, morphine, nubain

Question 22

treatment of SCD

Answer 22

HYDRATION, warmth, rest, avoid crisis, pneumoccoccal/FLU vaccine, avoid sudden changes in temp

Question 23

what may you have to do with many tranfusions

Answer 23

meds to bind iron and get rid of it

Question 24

med that stimulates fetal hemoglobin.

Answer 24

hydroxyurea (Droxia, Hydrea)

Question 25

SE of hydroxyurea

Answer 25

increases risk of infection and long term use may cause leukemia

Question 26

oral med used to decrease excess iron

Answer 26

deferasirox (exjade)