Sickle Cell Disease Flashcards
How does sickle cell disease occur?to endo
Change from glutamic acid to valine at one AA site
frequency of sickle cell disease
Hgb SS occurs in 0.15% of African american newborns
8-10% of african americans are carriers
Pathophysiology of sickle cell disease
deoxygenation of mutant Hb leads to
- K effluc
- incr cell density/dehydration
- incr polymerization
sickled cells adhere to endothelial cells –> vasoconstriction –> decreased blood flow
lifespan reduced to 10-20 days, high reticulocytes, and bone marrow factory produces 3-4x normal amounts RBCs
clinical features of sickle cell disease
higher Hgb - painful episodes, acute chest syndromes, osteonecrosis, proliferative
lower Hgb - stroke, priapism, leg ulcers
Hand foot syndrome
no symptoms in 1st 6 months of life due to circulating Hgb
- painful, symmetric swelling of the hands and feet
- ischemic necrosis of small bones
Splenic sequestion
sudden trapping of blood within spleen occurs in infants under 2 fever, pain, resp symptoms death can occur in less than 30min Treatment -IV fluids -splenectomy
Bone pain
chronic pain from bone infarction, sickle arthritis, and aseptic necrosis of the femur
treat with NSAIDs
Other problems in sickle cell disease
strokes
kidney damage
eye problems
preventative measures
no alcohol no smoking exercise regularly 8 12oz glasses of water a day reduce stress treat infections wear warm clothes
Treatment
Stroke: exchange transfusion
priapism: analgesia, hydration, partial exchange transfusion
Resp distress: abx coverage, O2, transfuse
splenic sequestration: transfuse
hydrate
analgesia: ibuprofen, APAP, ketorolac, opiates
vaccines
BMT
Endari
L glutamine powder: reduces oxidant damage to RBCs
reduces severe complications of sickle cell disease
AEs: constipation, N/V, HA, abd pain, cough, pain
hydroxyurea
reduces frequency of painful crises by stimulating production of fetal Hgb
long term use of this drug might cause problems later in life
AEs: fever, sore throat, cough, congestion, bruising or bleeding, hematemesis, melena