Hematology and Immunity of Children Flashcards
reduced number of red blood cells
decreased availability of hemoglobin
reduced volume of packed red blood cells
anemia
how is anemia caused
blood loss or destruction of RBCs
inadequate suply or loss of iron
iron deficiency anemia
who are at higher risk for iron deficiency anemia
infancy to 36mos
adolescence
what are general signs of iron deficiency anemia
irritability anorexia lethargy tachycardia pallor fatigue
in iron deficiency anemia why would an infant be over weight?
called “milk babies” because they are getting excessive milk intake and not enough iron is in the milk
what 3 interventions are used for iron deficiency
diet
iron supplements
blood transfusion is severe
when giving iron supplementation what should be given with it
vitamin c because it helps absorption
when giving iron what should you NOT give with it
vit d (milk) it decreases absorption
how should oral iron supplements be given
at side of mouth towards back of teeth
and brush teeth after administration!! iron causes tarry green stools too
boomerang looking RBCs blocking blood flow
sickle cell anemia
what are the 4 triggers to vaso-occlusion in sickle cells anemia
hypoxemia
dehydration
change in body temp
infection
can sickled RBCs reverse itself over and over
yes! until the point in time it stays sickeled
in sickle cell anemia why do we not see any symptoms until middle to late infancy
because the fetal hemoglobin cells do not sickle and are with them until 6mos of age
what are the general signs of sickle cell anemia
smaller in height/weight than peers
delay in sexual maturation
chronic anemia
how is sickle cell tested after birth
finger or heel stick that gets results in 6 min
sickledex
what are the 4 “crises” of sickle cell anemia (we will only be tested over the first one)
vaso-occlusive
sequestration
aplastic
hyperhemolytic
this crisis of sickle cell anemia is known as the “pain event or episode”
vaso-occlusive crisis
in the vaso-occlusive sickle crisis very painful and symptoms of pain are a result of
the obstruction at THAT SITE (lasting 4-6 days)
what are sickle cell crisis management
rest hydrate analgesics lyte replacements blood replacement (temporary) antiobiotics
what medication is used for sickle cell crisis
hydroxyurea (increases fetal Hgb)
and short term oxygen therapy (* remember it does NOT reverse sickling but will help them breath and relax) *prolonged oxygen use can depress been marrow
why do vaso-occlusive crisis pts not become addicted to meds
because their crisis only lasts about 4-6 days
*since we are not worried about them becoming addicted, their pain meds should be admin on a schedule!
what is important teaching of sickle cell pts
seek early tx for fever 101.3 or greater prophylactic med (penicillin and immunizations) adequate hydration avoid COLD compresses avoid low O2 environments avoid contact sports rest
body makes less HGB than normal because RBCs are damaged, no beta protein, so the RBC is destroyed prematurely
B thalassemia aka
Cooley’s anemia aka
mediterranean anemia
B thalassemia kids rely on frequent ______ ______ and because of this and hemolyzed RBC we get _____ overload. ____ ______ tries to make more RBCs and there is a build up in the _____ ______ making the _____ bigger
blood transfusions; iron; bone marrow; bone marrow; bones
what does a child with B thalassemia look like
large head, enlarged maxilla, protrusion of the lip and upper teeth, shorter in stature
skin takes on bronze appearance (excess iron)
excess iron storage in tissues of the body
hemosiderosis
excess iron storage in tissues that results in cellular death
hemochromotosis
what is the goal for B thalassemia pt
maintain adequate HGB levels by transfusion program
*lifelong and are usually every 3-5wks
in B thalassemia pt what is used to remove iron from the body
chelating agents (deferoxamine)
what teaching is done for deferoxamine (removes iron from body in B thalassemia pt)
admin at night while sleeping for 8-10 hrs
can be IV or Subq for 5-7 days
admin with vit C
avoid iron wish foods
turns urine reddish
*deferasirox is the same thing but is PO and used for children older than 2
if a child with B thalassemia has a splenectomy what is important to teach the family/pt
contact health professional of all fevers of 101 or greater * risk for sepsis in a child with asplenia
what are the main differences in sickle cell anemia and B thalassemia
sickle cell anemia the RBCs sickle
and are characterized by crisis
B thalassemia anemia the RBCs are fragile, chronic hypoxia, and iron overload due to blood transfusion
what children are at higher risk for lead poisoning
less than 6y/o poverty living in older rent prop urban setting children with anemia
what are general signs of anemia
pallor
tachycardia
fatigue
weakness
how is lead poisoning assessed
universal screening at 1 and 2 years of age
target screening
why is lead poisoning so bad
crosses the BBB and causes severe neurological effects
*serum lead tests should be taken and if suspected then X-rays can be taken
if a child has <10mcg of lead in their blood what should be done
reassess/rescreen in 3 months environmental assessment (ask)
if a child has 10-44mcg of lead in their blood what should be done
reassess/rescreen <3months environmental investigation (go look)
if a child has >45mcg of lead in their blood what should be done
chelation therapy (removes lead) consider hospitalization
what should nurse teach when it comes to prevention of lead poisoning
HH
damp mop floors and window seals
frequent washing of pacifiers and toys
why is chelating therapy given more than one dose and for a period of time
because lead moves between blood, soft tissues, organs, bones, and teeth. make sure it gets it all
during chelation therapy in lead poisoning how should you protect kidneys
adequate hydration
what is important to know about lead poisoning tx succimer
oral for 19 days and it comes in capsule or sprinkle
what is important to know about lead poisoning tx calcium disodium edetate
IM or IV but its very painful IM so you should apply EMLA an hour before injection
prolonged bleeding anywhere from the body
hemophilia
what is the hallmark sign of hemophilia
hemarthrosis= bleeding into a joint capsule, “target joints”
what are symptoms of hemarthrosis (in hemophilia)
swelling
redness
pain
loss of movement
what is important for you to NOT do when a child has a swollen area at a joint (due to hemophilia)
NEVER aspirate it will just cause more bleeding
how can we prevent bleeding (hemophilia)
exercise and PT (no contact activity tho)
water pick for teeth
if IM needed hold pressure for 15 min
venapuncture instead of heel/finger sticks
what does a child usually say they feel if they are experiencing hemophilia in certain are
feels pressure and “bubbling”
for bleeding (hemophilia) what is the first thing done
factor replacement but while we wait RICE should be done rest ice compress elevate
what is important to know about admin of factor replacement
admin 2-3x per week as prophylactic but DO NOT have it premixed because it has a short lifespan
in a hemophilia pt what med is contraindicated
aspirin because it will cause them to bleed more *acetaminophen can be used
excessive destruction of platelets and usually follows a viral illness
Immune thrombocytopenia
what are symptoms of immune thrombocytopenia
easy bruising
petechiae
bleeding from MMM
prolonged bleeding from abrasions
when should you restrict activities in pt who have IT
when their platelets are <50,000
what meds are given for IT pt
steroids
immune globulin
anti D antibody
*goal of all 3 is to prolong survival rate of the existing platelets
why is a splenectomy put off until about 5 years of age in a pt who has IT
to decrease chance of infection
decreases and eventually eliminates a persons immune system
HIV
for children under 13 the transmission of HIV is ______ spread
vertical (late in pregnancy
L and D
breastfeeding)
why are infants born to HIV mothers test positive for HIV
due to antibodies from the mom (maternal antibodies persist up to 18 months)
what are CM of HIV in children
lymphadenopathy hepatosplenomegaly oral candidiasis chronic reoccurent diarrhea FTT developmental delay parotitis
How will HIV be managed with meds
ART and treating opportunistic infections (due to low immune system) *tx will be LIFELONG
in pt with HIV what type of vaccines will they be able to receive
they are very immunocompromised so live vaccines should NOT be given
what are cardinal signs of cancer in children
unusual mass or swelling unexplained paleness and loss of energy sudden tendency to bruise persistent localized pain or limping prolonged fever or illness frequent HA with vomiting sudden eye or vision changes excessive rapid weight loss
cancer arising from the sympathetic nervous system *more common in abdomen
neuroblastoma
firm, nontender, irregularly shaped mass in the abd that CROSSES the midline
neuroblastoma
why is a neuroblastoma called the silent tumor
because the dx has been made after it has already been metastasized to other location
what is the preferred method to see neuroblastoma tumor
CT
immature WBCs crowd out the other components of blood and depress the bone marrow production of RBCs
path of leukemia
what are the symptoms of leukemia
pallor listlessness irritability fever anorexia weight loss petechiae bruising bone/joint pain
what is the definitive dx of leukemia
bone marrow aspiration *after dx a lumbar puncture is done to see if there is brain involvment
for pt with leukemia, the _______ the leukocyte count at dx the _______ the prognosis
higher; worse
cancerous tumor arising out of the retina
retinoblastoma
cat’s eye reflex is hallmark of (white pupil)
retinoblastoma
what is the tx for retinoblastoma
radiation
removal of the eye
chemotherapy
for pt with retinoblastoma what should you prepare the parents of
expect to see an empty socket after sx
fast growing, asymptomatic, firm, lobulated mass located to ONE side of abd
wilm’s tumor
why should you not palpate the abd of a child who has wilm’s tumor
can dislodge cells and spread to adjacent and distant tissue
most frequent malignant bone tumor type in children
osteosarcoma
what are the primary tumor sites of osteosarcoma
long bones, especially the legs and distal femur
what is the definitive dx tests for osteosarcoma
radiologic studies and elevated alkaline phosphatase with some bone tumors
2nd most common malignant bone tumor in children and adolescents
arises in marrow frequently in femur, tibia, ulna, humerus etc
ewing sarcoma
in ewings sarcoma prognosis is best if no _______ has occurred at the time of dx
metastasis
what lesions of ewing sarcoma have the best potential for cure
distal lesions
what is the preferred site for bone marrow aspiration
posterior superior iliac spine
what are the phases of chemo
induction therapy
central nervous system prophylaxis
intensification/consolidation therapy
maintenance therapy
what is the goal for induction therapy
remission
what is the goal for central nervous system prophylaxis
prevent leukemia cells in CSF
what is the goal for intensification/consolidation therapy
destroy any residual leukemia
what is the goal for maintenance therapy
remain in remission
at the first sign of infiltration why should you immediately stop chemotherapy infusion
because its a sclerosing agent and can literally eat through the skin
how can you prevent anaphylaxis to chemo
thorough hx of allergies
have emergency meds and mask/bag ready
recognizing hypotension, wheezing and urticaria
what are side effects of radiation therapy
anorexia nausea vomiting extreme fatigue skin reactions
changes DNA so that it cannot reproduce and is scheduled over 1-6 weeks
radiation therapy
what is important to teach about radiation
do not try to wash mark off of site
do not expose radiated area to sun or heat
no cream or lotion on site (can misdirect radiation)
what is point of nadir
bone marrow suppression at its greatest, neutrophil count at its lowest
what do neutrophils do
fight bacterial infections
an ANC below _____ warrants evaluation
1500
ANC below _____ places child at greatest risk for infection
500
what is the ANC formula
(segs+bands)xWBCx10
prophylaxis against bacteria
trimethoprim-sulfamethoxazole
prophylaxis against fungi
nystatin
prophylaxis against virus
no live immunizations
infection increases the tendency toward bleeding
thrombocytonpenia
most likely have no conception of death
infants and toddlers
death as temporary, a departure, a kind of sleep
preschoolers
death is irreversible but not necessarily inevitable and may fear mutilation
schoolage
death is inevitable, irreversible
adolescents
