perfusion (pediatrics) Flashcards by Rebecca Penney

3 main concepts associated with perfusion

-pain
-gas exchange
-patient education

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what is sickle cell anemia

-an inherited red blood cell where hemoglobin is replaced by abnormal sickle/crescent-shaped cells

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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)

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what groups are sickle cell anemia usually seen

middle-eastern, african, meditarranian, aborignal people

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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

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screening at birth for sickle cell anemia

high performance liquid chromatography (HPLC)
hemoglobin analysis (eg. hb electrophoresis)

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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

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3 types of sickle cell crisis

-sickle crisis (vaso-occlusive crisis)
-aplastic
-sequestration

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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)

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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

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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

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main organ affected in sickle cell crisis in kids

spleen
then lungs & CNS
-heart is last

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main organs affected in sickle cell crisis in adults

lungs, liver
heart is last
(usually do not have a spleen by adulthood)

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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

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diagnostic testing for sickle cell anemia

hemoglobin electrophoresis (idenitifies normal vs abnormal RBCs, can also identify carriers)
-newborn screenings

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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

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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

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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

perfusion (pediatrics) Flashcards

Jan 16th