Download Chap 44 - TeacherWeb

Chapter 44 Hematologic Disorders
 Assessment- pg 1283 & 1290
 Review structure & function of blood
Bone marrow aspiration
 Sites-iliac crests or spines due to
larger marrow compartments in
childhood. Neonates-anterior tibia
 Position-prone
 Topical anesthetic
 Conscious sedation may be used
 Pressure to site followed by pressure
drsg
Transfusion
 Packed RBC’s-help minimize risk of
fluid overload
 Rate-10 -15 ml/kg/hr
 Signed consent
 VS every 15 min first hr, then every
30 min.
 Reactions-1287
Transplantation
 IV infusion of hematopoietic stem
cells from bone marrow obtained
from bone marrow aspiration or
peripheral or umbilical blood from a
donor
 Common procedure for aplastic
anemia, sickle-cell, thalassemia, &
leukemia
 Cytoxan IV to prevent rejection
 Fever & chills most common reaction
 Tylenol, valium, & benadryl may be
given
 Restrictions r/t immunosuppression
Splenectomy
Laparoscopic surgery
Susceptible to infections
Penicillin prophylactic for 1-2 yrs
Pneumococcal & meningococcal & HIB
vaccines as well as others
 Report any S&S of infection-cough,
fever, general malaise




Anemias
 Sickle cell & hemophilia-inherited
 Iron-deficiency-preventable
 Aplastic anemia-congenital or
acquired
Disorders of RBC’s
 Acute blood loss-control bleeding by
correcting underlying cause. Supine
position, keep warm, administer NS or RL
IV, infuse plasma or blood
 Aplastic anemia-mgt-bone marrow
transplantation
 Iron-deficiency-most common-iron fortified
formula first yr of life. Iron supplements.
Older children-other diseases & diets
 Feosol(1294)
Hemolytic Anemias
 Sickle Cell-autosomal recessive defect
among African Americans
 Begins to produce signs at about 6 mons of
age-fever, anemia, swelling of hands & feet
& protruding abdomen. Sclera becomes
yellow & susceptible to infections
 Sickle cell crisis-sudden, severe onset of
sickling usually following an URI or
dehydration
 Chart-pg 1297;
 Mgt-Pain relief, adequate hydration &
oxygenation. Tylenol, IVF’s, IV
morphine, hydroxyurea(antineoplastic
agent), monitor I&O, blood
transfusion, oxygen
 Focus on preventing recurring crisis
 Immunizations
 Penicillin as prophylaxis
 Care Plan 1297-1299
 Thalassemias-abnormalities of beta
chain of adult hemoglobin frequently
Mediterranean descent-also African &
Asian heritage
 Minor-produce both normal &
defective hemoglobin. Only sign may
be pallor, require no tx, & life
expectancy is normal
 Major (Cooley’s or Mediterranean).
 Effects-pg 1302
 Mgt-digitalis, diuretics & low sodium
diet. Transfusion of packed RBC’s
every 2-4 weeks. Splenectomy may
be necessary. Bone marrow or stem
cell transplantation can offer a cure
Disorders of Blood Coagulation
 ITP-result of a < in the # of circulating
platelets-may result from increased rate of
platelet destruction d/t antiplatelet
antibody that destroys platelets
 Assessment-miniture petechiae or lg areas
of asymmetric ecchymosis most prominent
over legs. Epistaxis or bleeding into joints
may also occur. Platelet count as low as
20,000
 Mgt-oral prednisone & IV
immunoglobin. May require platelet
transfusion.
 Usually limited to 1-3 mons. Prevent
from injury
 Henoch-Schonlein Syndrome-caused by
increased vessel permeability
 Assessment-purpural rash typically on
buttock’s, posterior thighs & extensor
surface of arms &. legs. Lesions change to
pink maculopapules, become bright red
then fade leaving brown macular spots.
Joints tender & swollen, abd pain, N&V,
bloody stools.
 Mgt-prednisone & tylenol. Course of 4-6
wks. Chronic nephritis possible
complication
Blood Coagulation Disorders
 DIC-acquired disorder of blood
clotting that results from excessive
trauma or some underlying stimulus.
An imbalance exists between clotting
activity & fibrinolysis. Increased
coagulation & a bleeding defect at the
same time
 Assessment-uncontrolled bleeding from
puncture sites from injections or IV’s.
Ecchymosis & petechiae from on the skin.
Toes & fingers appear pale, cyanotic or
mottled & feel cool.
 Mgt-Stop underlying insult causing
bleeding. IV heparin to discourage blood
coagulation, fresh frozen plasma, platelets,
& fibrinogen may be administered.
 Hemophilia A (Factor VIII
Deficiency)Male child’s factor VIII is
deficient-not absent-varying degrees
from mild-severe. Blood will
eventually coagulate. Often
recognized first after circumcision
 Assessment-lower extremities usually
become bruised (bumping into things).
Joints become swollen & painful-may result
in loss of mobility. May have severe
bleeding into GI system
 Mgt-Administration of Factor VIII.
Powdered forms can be stored at homereconstituted when needed. DDAVPstimulates the release of Factor VIII may
also be helpful. May also give Factor IX .
Prevent injury, apply ice & immobilize area
injured. Don’t give Ibuprofen.
 Von Willebrand’s Disease-affects both
sexes, Along with Factor VIII
deficiency-there is also an inability of
platelets to aggregate. Blood vessels
can’t constrict, bleeding time is
prolonged.
 Epistaxis is a major problem
 Mgt-factor VIII & DDAVP
 Christmas Disease (Hemophilia B,
Factor IX Deficiency)
 Mgt-concentrated factor IX
 Hemophilia C (Factor XI Deficiency)
 Mgt-DDAVP or transfusion of fresh
blood or plasma