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
3 factors for hemostasis
1.constriction of the blood vessel
2.formation of platelet plug
3.activation of coagulation cascade
3 types of hemophilia
Hemophilia A (classic hemophilia)
Hemophilia B
Von Willebrand disease (vWD)
which types of hemophilia lead to deep tissue bleeds in joint/muscle
hemophilia A & B
which types of hemophilia lead to bleeds in mucus membranes & skin surfaces
Von Willebrand Disease
what is deficient in hemophilia A
Factor VIII
what is deficient in hemophilia B
Factor IX
what is deficient in Von Willebrand disease
Von willebrand factor and factor VIII deficiency
classification of severe hemophilia A
-spontaneous bleeding WITHOUT trauma
<1% factor VIII
-most common (60-70%)
classification of moderate hemophilia A
-bleeding with mild trauma
-1-5% factor VIII
-usually detected between 1-2yrs of age d/t inc # of injuries
classification of mild hemophilia A
-bleeding with severe trauma or surgery
->5-40% factor VIII
-usually detected late
what is the normal amount of factor VIII
50-150% factor
what is needed for a diagnosis of hemophilia
-hx of bleeding episodes
-fhx of hemophilia
-lab tests: blood clotting fucntion, PTT(elevated), factor VIII assays
-PT and platelets will be normal
-DNA testing to determine carrier detection
clinical manifestations of hemophilia
-prolonged bleeding anywhere from or in the body
-hemorrhage from any trauma (mild or severe)
-excessive bruising
-subcutaneous & IM bleeding(avoid IM injects)
-spontaneous hematuria
-hematomas
-hemarthrosis
-SQ bleeds on neck, mouth, thorax, spinal cord, GI, intracranial
what is hemarthorsis?
bleeding into a joint cavity
-commonly in knees, elbows, ankles
-leads to bony changes & crippling deformities
s&s of hemarthorsis
stiffness
tingling
aching
limitations in joint movement
-pain, swelling, redness, warmth, loss of movement
management of moderate to severe hemophilia
-IV replacement of missing clotting factor
-prophylaxis therapy->periodic regular replacement (3x a week d/t clottig factor is filtered out in 12-24h, given prior to surgeries/procedures)
-on demand therapy after a bleed
management of mild hemophilia
-desmopressing (DDADP); only increases factor VIII by 30%. given nasal,SQ,IV
medications given for hemophilia
corticosteroids: hematuria or acute joint tissue bleed
-tranexamic acid & aminocaproic acid:holds clot in place once formed->blocks plasmin factor
-analgesics; dec pain
can you give NSAIDs for hemophilia?
NO
it intereferes with platelet aggregation
supportive management for hemophilia
exercise & physio
-rest, Ice, compress, elevate
-reduction of bleeding risks
-medic alert ID
-family support(educate & genetic counseling)
-good dental practices(dec bleeding and # of procedures needed)
what is immune thrombocytopenic purpura (ITP)
-autoimmune disorder that destroys platelets
-acute or chronic
-common in women and kids ages 1-4
manifested by easy bruising, bleeding from mucus membranes, internal hemorrhage(hematuria, hematemesis, melena, menorrhagia)
manifestations of ITP
-platelet count less than 150 x 10^9/L
-if <10 x 10^9/L = spontaneous bleeding (life threatening)
-rash
-pooling of blood under skin surface
supportive management of ITP
-observation if no bleeding
-activity restriction if platelets <50
-no NSAIDs or aspirin
-no platelet tranfusions d/t autoimmune reaction
medication management for ITP
-corticosteroids: given 2-4w to dec immune system (stunts growth in kids)
-IV immune globulin (IVIG); makes spleen not destroy platelets->works in 24-48h w/ chronic & no active bleed
-anti-D antibody; only for RH+ pt, dec destruction of platelets in spleen
surgical procedures for ITP
splenectomy as a last resort
