perfusion (pediatrics) Flashcards
Jan 16th
3 main concepts associated with perfusion
-pain
-gas exchange
-patient education
what is sickle cell anemia
-an inherited red blood cell where hemoglobin is replaced by abnormal sickle/crescent-shaped cells
what can sickle cell anemia lead to
-obstruction(thrombosis forms easily)
-vascular inflammation (increases b/p & can lead to heart failure)
-increased RBC destruction (destroyed in 10-12 days, can lead to fatigue and jaundice)
what groups are sickle cell anemia usually seen
middle-eastern, african, meditarranian, aborignal people
what age group is sickle cell anemia usually undetected in
1 year of age and under d/t no s&s d/t increased amt of fetal hemoglobin which inc normal hemoglobin
screening at birth for sickle cell anemia
high performance liquid chromatography (HPLC)
hemoglobin analysis (eg. hb electrophoresis)
clincial manifestations of sickle cell anemia
-enlarged bones d/t bone marrow expanding to compensate(childhood)
-tachycardia, murmurs, enlarged heart, increased blood viscosity, heart failure, pulmonary hypertension
-hemolysis, thrombosis
-anemia, jaundice, pain
3 types of sickle cell crisis
-sickle crisis (vaso-occlusive crisis)
-aplastic
-sequestration
what happens in sickle crisis (vaso-occlusive crisis)
caused by thrombosis
-pain is major complaint
-can lead to organ damage (no perfusion)
-avascular necrosis (higher likelyhood in hips and shoulders but can happen anywhere)
what happens in aplastic sickle cell crisis
-usually secondary to parvovirus/respiratory virus
-bone marrow stops producing RBCs
-hemoglobin lvls drop rapidly which causes the aplastic crisis
-organ damage
-important to prevent infection in SCA
what happens in sequestration sickle cell crisis
-most life threatening
-sickle cells pool in 1 organ=decreases circulatory BV which lead to circulatory collapse & shock
-usually occurs in the spleen in kids, leads to decreased filtration of bacteria & creation of antibodies (leads to autosplenectomy, usually by age 10 d/y repeated sequestration)
-prone to infection
main organ affected in sickle cell crisis in kids
spleen
then lungs & CNS
-heart is last
main organs affected in sickle cell crisis in adults
lungs, liver
heart is last
(usually do not have a spleen by adulthood)
complications of sickle cell anemia
-acute chest syndrome (presents as pneumonia)
-pulmonary HTN/heart failure
-CVA
-autosplenectomy
-infection(pneumonia, osteomyelitis)
-reproductive problems(priapism, menses pain, impotence)
-renal failure
-chronic leg ulcers, dec wound healing
-blindness (d/t thrombi), retinal detachment
-avascular necrosis/bone marrow enlargement
diagnostic testing for sickle cell anemia
hemoglobin electrophoresis (idenitifies normal vs abnormal RBCs, can also identify carriers)
-newborn screenings
pharmacologic therapies for sickle cell anemia
-#1 hydroxyurea (taken for life, dec production of sickle cells, intense side effects & congenital defects)
-oral penicillin prophylaxis (2m-5y of age)
-ABX for any s&s of infection
-NSAIDS, Tylenol, aspirin, opioids(thombi pain)
-folic acid(inc RBCs)
-vaccines
-hematopoietic stem cell transplant(HSCT) not used often, needs a compatible donor
nonpharmacologic management for sickle cell anemia
-hydration(keep blood viscose, dec chances of thrombi)
-monitor for infection
-oxygen to prev/dec sickling
-exchange transfusions
-packed RBCs transfusions
-heat application(NEVER COLD)
-splenectomy