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Peds Exam 3A > Hematologic Disorders > Flashcards

Hematologic Disorders Flashcards

1
Q

what is anemia

A

decrease in RBC’s or hemoglobin concentration below normal

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2
Q

what causes anemia

A

hemorrhage
hemolysis
bone marrow suppression
absence of iron, B complex vitamins, erythropoietin

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3
Q

signs and symptoms of anemia

A 
anorexia
pallor
skin breakdown
jaundice
tachycardia and tachypnea
altered neurologic status/behavior changes
weakness/ low activity tolerance
gum hypertrophy 
smooth tongue
blood in urine or stool
infections
cold intolerance
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4
Q

how does anemia effect your circulatory system

A 
hemodilution
decreased peripheral resistance
increased cardiac circulation and turbulence (may have murmur, to lead to cardiac failure)
cyanosis
slowed growth
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5
Q

how do you treat anemia?

A

treat underlying cause
transfusion after hemorrhage if needed
nutritional intervention for deficiency anemias

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6
Q

supportive care of anemia

A

IV fluids to replace intravascular volume
Oxygen
Bed rest

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7
Q

nursing considerations for anemia

A 
prepare child/family for lab test
decrease o2 demands
safety
good hand washing and mouth care
maintain normal body temp
prevent complications
support family
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8
Q

clinical manifestations of iron deficiency anemia

A 
irritability, anorexia
pallor of skin and mucous membranes 
mild growth retardation
exercise intolerance
frequent infections
cognitive delays and behavioral changes
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9
Q

causes of iron deficiency anemia

A

inadequate iron stores at birth
deficient dietary intake r/t rapid growth rates during infancy, toddler, and adolescence, excessive milk intake, poor general eating habits, exclusive breath feeding after 6 months
impaired iron absorption

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10
Q

how do you prevent iron deficiency anemia?

A

breast milk or commercial infant formula for the first 12 months of life
limit formula to 1 liter a day (32 oz)
limit milk to less than 24 oz/day

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11
Q

management of iron deficiency anemia

A

nutritional supplements; by age 6 months
(iron fortified formula, iron fortified cereal)
iron supplements ( ferrous sulfate)
blood transfusions in serious cases

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12
Q

nursing implications for iron deficiency anemia

A 
pay attention to milk and iron intake
determine and eliminate the cause
provide food rich in iron ( green leafy veg, beans, beef, lentils)
teach parents to administer supplements
administer iron safely
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13
Q

how to administer oral iron

A

best absorbed in an acidic environment (give with apple or orange juice)
give with straw or back of mouth past teeth
rinse mouth and brush teeth after administration

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14
Q

what to teach parents about administering oral iron

A

measure accurately, increase fluids and fiber

iron can be deadly, lock up meds, keep out of reach

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15
Q

what foods/drinks do you avoid with oral iron?

A 
antacids
coffee
tea
dairy products
eggs
whole grains
avoid all of these 1 hour before or two hours after administration
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16
Q

adverse effects of iron administration

A 
nausea
gastric irritation
constipation
diarrhea
anorexia
staining of teeth
tarry stools 
overdose is LEATHAL
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17
Q

what is sickle cell anemia

A

autosomal recessive hemolytic anemia
mist common in African Americans, Mediterranean, middle eastern, and Indian descents
usually asymptomatic until 6m

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18
Q

pathophysiology of SCA

A

HgB S has altered amino acid structure making it less soluble than HgB A
RBC’s become rigid, fragile, and rapidly destroyed. cells loose ability to flow easily through tiny capillaries, and obstruct blood flow
microscopic obstructions lead to engorgement and tissue ischemia
RBC’s with HbG S live less than 20 days

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19
Q

complications of SCA

A 
acute painful episodes ( sickle cell crisis)
stroke
sepsis
acute chest syndrome
reduced visual acuity
chronic leg ulcers
delayed growth and development
delayed puberty
priapism
enuresis (bed wetting)
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20
Q

what causes sickle cell crisis

A

anything that increases the bodies need for oxygen, or alters the need for oxygen
trauma
infection, fever
physical and emotional stress
increased blood viscosity due to dehydration
hypoxia ( r/t altitude, no pressurized plane cabins, hypoventilation, vasoconstriction due to hypothermia)

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21
Q

vaso-occlusive (VOC) thrombotic event (SCA)

A

painful event/ episode
most common type of episode- PAINFUL
stasis of blood with clumping of cells in microcirculation causing ischemia/infarction
s/s- fever, pain, tissue engorgement

22
Q

Splenic sequestration (SCA)

A

life threatening- death can occur within hours
blood pools in spleen
s/s- profound anemia, hypovolemia, shock

23
Q

Aplastic Crisis (SCA)

A

diminished production and increased destruction of RBC’s
triggered by viral infection or depletion of folic acid
s/s- profound anemia, pallor

24
Q

acute chest syndrome ( SCA)

A

similar to pneumonia
VOC or infection results in sickling in lungs
chest pain, fever, cough, tachypnea, wheezing, and hypoxia
repeated episodes may lead to pulmonary HTN

25
Q

prognosis of SCA

A

no cure (except possibly bone marrow transplant)
frequent bacterial infections
bacterial infection can lead to death in young children
stokes are at an increased risk

26
Q

palliative tx of SCA

A

erythropoietin or hydroxyuria

PCN prophylaxis

27
Q

nursing considerations for crisis management of SCA

A 
maintain hydration
maintain oxygenation (avoid altitudes, hot weather, manage stress)
pain management ( kids require more pain meds than normal child)
28
Q

Cooley’s anemia ( Beta Thalassemia)

A 
inherited blood disorder
both parents must carry disorder
HgB synthesis reduced or entirely absent
chronic hypoxia
detected in infancy or toddlerhood (pallor, FTT, severe anemia, hepatosplenomegaly)
29
Q

manifestations of Cooley’s anemia ( beta thalassemia)

A

severe anemia
bronzed skin
bone pain, skeletal deformities, fractures
iron overload r/t transfusions, treat with chelating drugs

30
Q

medical tx of cooley’s anemia (beta thalassemia)

A

diagnosed with Hgb electrophoresis

blood transfusion to maintain normal levels (side effect hemosiderosis)

31
Q

how do you treat hemosiderosis

A

iron-chelating drugs- binds to excess iron for excretion by kidneys
IV or subq over 8-10 hours multiple times/week
may be given at home with IV pump per parents
can be given orally
also give oral vitamin c to facilitate iron binding

32
Q

nursing care for cooley’s anemia ( beta thalassemia)

A

administer RBC
monitor chelation therapy (n/v/d, decreased appetite, rash, increased liver enzymes, neutropenia, adequate hydration is VITAL

33
Q

what is SCID

A

absence of both humeral and cell mediated immunity

unknown cause

34
Q

clinical manifestations of SCID

A 
early, frequent infections
FTT
chronic diarrhea
persistent thrush
adventitious lung sound r/t pneumonia
low levels of immunoglobulins
stem cell replacements
BUBBLE KIDS- live in a sterile environment
35
Q

medical/nursing management of SCID

A 
IV immunoglobulin
sterile environment
bone marrow transplant 
prevent exposure to infections
support and educate family
36
Q

HIV in kids

A

transmission from + mom prenatal, perinatal, breast milk
blood transfusions
unprotected sex

37
Q

why are adolescents most at risk for HIV

A

drugs and unprotected sex

38
Q

how do you treat newborns born to a positive HIV mom

A

antiviral therapy for first month of life

39
Q

medical management of kids with HIV

A

antivirals therapy for infants born to positive moms for first month of life
aggressive antibiotic therapy for infections
modified immunization schedule
prophylaxis against opportunistic infections

40
Q

what is ITP

A

acquired hemolytic disorder
destruction of platelets in the spleen
Bleed very easily

41
Q

treatment of ITP

A 
supportive
anti-D antibody- plasma derived immunoglobulin that causes transient anemia by clearing the antibody coated RBC
IVIG
steroids for 1-2 weeks
splenectomy and IVIG if chronic
42
Q

nursing considerations for ITP

A

educate parents
assess for bleeding
prevent trauma
avoid unnecessary procedures, needle sticks, suppositories, rectal temps
apply pressure for 5 minutes after venipuncture
follow up care in home or clinic

43
Q

what is hemophilia

A

deficiency of specific clotting factors

hereditary

44
Q

hemophilia A

A

classic hemophillia
deficient of factor VIII
males most affected
degree of bleeding depends on amount of clotting factor and severity of a given injury

45
Q

manifestations of hemophilia

A 
bleeding tendency 
symptoms may not occur until 6 months
hemarthrosis- bleeding into the joint space (knee, ankle, elbow, leading to impaired mobility)
ecchymosis (bruising)
epistaxis 
bleeding after procedures
46
Q

early signs of hemarthrosis

A

stiffness, tingling, or ache followed by decreased mobility, warmth, redness, welling and severe pain

47
Q

medical management of hemophilia

A 
prevent bleeding ( no contact sports, riding scooters, PAD child during activities)
replacement of deficient factor ( VIII or DDAVP)
regular exercise and PT
48
Q

nursing interventions for hemophilia

A

prevent bleeding
safe environment, use of protective equipment
avoid ASA
sub q instead of IM
venipuncture vs heel stick, no suppositories
electric razors
avoid contact sports
soft toothbrush
recognize and control bleeding (pressure x15min, RICE)
be suspicious of headache, slurred speech, altered LOC, black tarry stools

49
Q

HS purpura

A

unknown etiology
occurs 6m - 16yrs
generalized vasculitis of dermal capillaries
extravasation of RBC’s, produces petechial skin lesions
inflammation and hemorrhage in GI tract
may lead to ongoing nephrotic syndrome

50
Q

therapeutic management/nursing of HS purpura

A 
most cases resolve without treatment
corticosteroids and anticoags from severe or persistent cases
symptomatic treatment
maintain hydration
monitor renal function

Peds Exam 3A (5 decks)

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