SCD Flashcards
routine newborn screening for SCD
Hbg S
if newborn screening is + a ________ test is done
second
sickling is caused by a mutation in the gene that tells the body to make
hemoglobin
overall goal of helping patients with SCD
improve tissue perfusion to organs, tissues, and nerves
pain due to
occlusion
what makes the cells have normal shape again
oxygenate and hydrate
clinical manifestations do not occur in children until?
4-6 months
dactylitis
inflammation of digits
AVN (avascular necrosis) of the ________ is a common complication in SCD
femoral head
the collapse of the femoral head occurs in 90% of the patients within
5 years of diagnosis of osteonecrosis
what triggers crisis of SCD
stress (dehydration, infection, mental, stress)
cardio pulmonary complications
acute chest syndrome (life threatening), chronic lung disease (which can lead to pulmonary HTN)
priapism
painful long lasting erections as blood is trapped in the erection chambers
ways to fix priapism
intra cavernous injections to narrow and decrease blood flow to the penis and aspirate blood from the penis
bone marrow slows its production or RBCs due to infection. Decrease in RBCs causing paleness, fatigue, and rapid pulse. Can be fatal
aplastic crisis
organs are deprived of blood and oxygen (cells become trapped in the organs and occlude blood flow)
organ damage
complications
CNS (stroke), cardio pulmonary, gallstones, priapism, leg ulcers, stroke, acute chest syndrome, pulmonary hypertension, organ damage, aplastic crisis, vision problems, frequent infections
blood pools in spleen
splenic sequestration crisis
SCD symtoms
jaundice, fatigue, back pain, arm and leg pain, foot swelling, joint pain, hand swelling, breathlessness
transfusion therapy used to
treat certain complications and prevent complications
analgesic meds
demerol, morphine, nubain
treatment of SCD
HYDRATION, warmth, rest, avoid crisis, pneumoccoccal/FLU vaccine, avoid sudden changes in temp
what may you have to do with many tranfusions
meds to bind iron and get rid of it
med that stimulates fetal hemoglobin.
hydroxyurea (Droxia, Hydrea)
SE of hydroxyurea
increases risk of infection and long term use may cause leukemia
oral med used to decrease excess iron
deferasirox (exjade)
