School Aged, Cardiac and Hematologic disorders Exam: PEDS
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1. Prepubescent
- girls @ 10-12
-boys @ 12-14
reason why girls tend to outgrow boys during school age
period
2. Psychosocial de- industry vs. inferiority
velopment for
school aged children
3. Industry vs. infe- Achieve sense of accomplishment with new skills
riority
Inferiority= when the child is unable to complete the task
and "fails"
Set tasks out for them that they are able to accomplish to
build self-esteem
Ability to get along with others better
Formation of same sex friendships
Learn to appreciate different viewpoints
Self concept is influenced by teachers and peers
Positive feedback from these individuals lead to increased
self esteem
Learn to cooperate and the value of team play
Dividing the labor and working as a team= accomplishing
a goal or winning.
Interested in how things work (may start to take things
apart to see how they work)
4. Cognitive devel- -concrete operations
opment of school -Logical thought--END OF SCHOOL AGE
aged children
Don't start out in logical thought
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School Aged, Cardiac and Hematologic disorders Exam: PEDS
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Other viewpoints
Begin to understand how others feel
Helps them to begin to develop moral compass
Conservationism
Pg. 431
"Just because something changes shape (example. Tall
skinny glass, small square glass) doesn't mean the
amount of what is in it will change.
Ability to classify
May assign order
best friend, second best friend etc..
Group like items
Able to understand the golden rule: doing to others as you
would like done to you
Relational terms and concepts
Big/small, right/left
Begin to understand concept of time
Reading: most significant achievement
5. Social development for school
aged children
We still play (Social) (work of kids is play)
Peer group = security blanket
Important in gaining independence from parents
Helps them understand hierarchies and peer interactions
Bargaining, compromise, and how to argue and still be
friends
May belong to more than on peer group
If they don't fit into a group, they may be bullied
Must be successful for positive image
Begin to develop sex role and accept others point of view.
Develop same-sex friends
Like to play games with groups and rules
Like to cook
Team sports, gymnastics, ballet, swimming, skating
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School Aged, Cardiac and Hematologic disorders Exam: PEDS
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Learn how to coordinate individual goals for the goal of the
group
Still enjoy some solitary activity, need time to decompress
Seeks attention and approval, acceptance
6. Self concept of Opinions of peers and teachers
school aged chil- Can affect self-esteem
dren can be affected by
Physical deviations become sensitivities
Can leave them to be bullied, having poor self-concept/self-esteem
7. School in
Makes them more independent
school-aged chil- Teachers become a big influence
dren
Parent responsibility: teach child to be responsible, encourage them to try new things and be independent
Evaluate child's friends- are they a good influence?, meet
the friends parents
Parents need to know children's friends and their parents
in order to prevent child from dangerous situations
Support child, listen to what went wrong (ex: bullyingmonitor and support and step in when needed)
Latch key children- children whose parents who work
10-12 hours a day, left alone without adult supervision
@ high risk of any number of things happening to them
(injury, kidnapping, burns)
8. Latch key children
children whose parents who work 10-12 hours a day, left
alone without adult supervision
@ high risk of any number of things happening to them
(injury, kidnapping, burns)
9. Discipline for
Natural consequences work well (have something taken
school aged chil- away, grounded, in school detention)
dren
10. Dishonest beTry to avoid getting in trouble
havior in school Make up stories to avoid trouble: stop it immediately
aged children
Need to get everyone's story in order to get the truth
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School Aged, Cardiac and Hematologic disorders Exam: PEDS
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11. Fears of school
aged children
Main fears: Kidnapping, medical procedures, and death
12. Sleep and rest for Do not need naps anymore
school-aged chil- 5 years of age= 11 ½ hour of sleep
dren
11 years of age= 9 hours of sleep
13. Nutrition for
Well Balanced
school aged chil- Offer lots of fruits and vegetables
dren
Decreased caloric need but appetite has not increases
Body fat increases during school age to prep for increased
growth for puberty
Avoid empty calories
Eat healthy snacks
Talk to parents about the current epidemic of obesity in
childhood
Healthy Snacks
2020 National Health Goals Related to School Age Children
Reduce the proportion of children who have dental caries
(in permanent or primary teeth) to no more than 49% from
a baseline of 54.4%
Increase age-appropriate vehicle restraint system use in
children from 78% to 86%
Increase the number of states that require helmet use by
bicyclists from 19 to 27 states
Increase the proportion of public and private schools
that require students to wear appropriate protective gear
when engaged in school-sponsored physical activities
from 76.8% to 84.5%
14. Dental health in Dental caries- Lax about dental habits
school age chil- Forget or get up late
dren
Tend to get more cavities
More vulnerable to get cavities
Primary teeth- 4-8 yr
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Secondary- 12-18 yr
Need to brush teeth early and get them to enjoy the feeling
of clean teeth
Regular dental evaluation
Fluoride tx/plastic sealants recommended
15. "Avulsion"
(tooth knocked out) pg. 442
If it's permanent and you want to save it:
Hold it by the crown, keep it in milk or in the child's mouth
to preserve it, head to dentist ASAP
16. Safety for school Motor vehicle
aged children:
Most common cause of injury
Still in back seat
To prevent airbag injury
Booster seat until 9 yrs, 90 lb, or 4'9"
Airbag safety
Look both ways before crossing street
Talk to parents about teaching their children to not ride in
the bed of the truck
Drowning
Swim lessons and water safety encouraged
Teach them not to dive in shallow water (if it comes up to
your shoulder--it is considered shallow water)
Child must have life jacket on at all time on boat
Burns
Try to cook without parents present which is bad
Make sure they know how to prevent burns
Teach what to do if fire
Bodily Damage
Other
Firearm safety
Lawnmower safety
Kids do not need to be outside while mowing is going on
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Bike safety
Always wear helmet
Skating boarding: helmet, elbow and knee pads
Tool safety
Need to understand how to do it, or have supervision at all
times.
Sports
Need correct gear to safely participate in those sports
ANA: children < 16 years of age should not ride ATV's
17. Signs of cardiac Murmur
dysfunction:
Tachypnea
Difficulty feeding-- 10-15 mL of formula and then they want
to sleep
Poor weight gain (FTT)
Exercise intolerance-- older child, get really tired, really
fast when exercises.
Want to know when problems present, if they take any
meds (what they were and if it helped).
Other S&S:
Frequent infection, dyspnea, SOB, cyanosis, sweating
with feeding, chronic fatigue, edema, frequent squatting
and dizziness.
18. Past medical his- During / after birth-- Mom's history too (how was their HR,
tory in children decels?, apgars, birth wt (high birth rate infants are at an
with heart disor- increased risk of cardiac disease))
ders
Frequent infections
Chromosomal abnormalities
Medication usage-- did mom take meds during pregnancy
that caused defect?
Family Hx of cardiac defects
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Maternal infection or drug ingestion during pregnancy
Frequent fetal loss can indicate cardiac disease
Mom's do not need to diet during pregnancy, can lead to
cardiac issues
Moms who have lupus or other autoimmune diseases are
more likely to have cardiac defects
Meds like phenytoin= teratogenic
Exposure to infections like rubella can cause congenital
anomalies in the heart
19. Physical assess- Measure height and weight
ment for child
with heart disor- Inspect for overall appearance
der:
Listen for heart rhythm, rate and presence of murmurs
Assess cap refill and peripheral pulses
Assess blood pressure in arm and thigh
Look at nutritional status, clubbing, cyanosis (possible
congenital heart disease), pallor (poor perfusion) , visual pulsations in the neck, tachypnea, dyspnea, tachycardia/bradycardia depending on defect, muffled heart
sounds, murmurs, additional heart sounds.
BP in a child < 1 year the arm and thigh pressure should
be the same
>1 SBP in leg is 10-40 mm Hg higher with a wider pulse
pressure which may indicate a defect.
20. Cardiac cath
goes in and visualizes defects and structures in heart
Can be done as dx or interventional
Most invasive dx procedure
Assess pedal pulses before procedure and after to make
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School Aged, Cardiac and Hematologic disorders Exam: PEDS
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sure that the child has not developed a clot
When doing this: height is important for appropriate length
of cath, weight is important for med administration, assess
for allergies.
Assess for diaper rash if they have one=NO HEART CATH
Pre op:
Record height and weight
Allergies
Assessment
Preparation - developmentally appropriate
NPO 6-8 hours
Post op:
Continuous monitoring: cardiac apnea monitor/pulse ox
Bed rest with extremities straight and pressure: 4-6 hr for
venous cath; 6-8 hr for arterial cath
Vital Signs
Always assess dressing for bleeding
If bleeding=apply pressure
Assess site and extremity
Advance feedings as tolerated
Discharge Preparation
Family-Centered Care Box pg. 741
21. Most common
ventral septal defect
congenital heart
disease
22. Things to be concerned about regarding congenital heart disease:
Respiratory infection
Polycythemia
Vomiting, diarrhea
Fever
23. Atrial septal defect
If the oval foramen (b/w R and L atrium) does not close
after birth we have congenital defect
24. Circulatory
Heart lies more horizontally
changes at birth Apex is higher in the chest
Ventricle walls similar in thickness
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Shift from R sided dominance at birth to L sided dominance
Higher heart rate in infancy
Lower blood pressure in infancy
Infants have a high resting HR and are therefore unable to
increase the HR to increase their Cardiac Output
25. Defects with increased pulmonary blood
flow
ASD, VSD, PDA
26. Ventricular sep- Most common type of congenital cardiac disorder
tal defect:
Abnormal opening between the wall of right and left ventricles
Allow oxygenated blood to flow back to right ventricle
Increases pulm blood flow and increases pressure in pulmonary artery causing hypertrophy of right ventricle
Occurs in 4 to 5 out of 1,000 live births
Opening in the septum between the two ventricles
27. Assessment of S&S of HF- At about 4-8 weeks of age shunting begins
ventricular septal Infant may exhibit easy fatigue
defect
Loud harsh murmur heard at left sternal border at third or
fourth interspace
A thrill may be present-- feel vibration on chest when the
child breaths
Tire easy on exertion and with eating-- may have FTT
May have frequent respiratory infections, SOB and edema
28. Dx of VSD:
see RV hypertrophy and may see pulm artery dilation
Chest X-ray
ECG
Echocardiography
MRI
29. Management of
VSD:
1/2 of VSDs will close on their own by age 2 yrs
Diuretic and digoxin to help prevent fluid accumulating in
the lungs
Closure can be accomplished by septal occluder that can
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be placed in heart catheterization
Monitor postoperatively for arrhythmia
30. Atrial septal defect:
Abnormal communication between the two atria
Abnormal opening in the wall b/w right and left atria where
foramen ovale was in fetal circulation
Allows oxygenated blood to flow from left atrium back into
right atrium which increases blood to lungs
Will have harsh systolic murmur at 2-3rd intercostal space
Causing an increased blood flow to right side of the heart
Right ventricular hypertrophy
Increased pulmonary blood flow
More common in girls than boys
80% close on their own
If not closed by 1-3, put in septal occluder or patch to close
the hole
Need to monitor for arrhythmia after the procedure
31. patent ductus ar- Fetal structure that connects the pulmonary artery to the
teriosus
aorta
Increase work of L side of heart and causes pulmonary
congestion or increased blood flow back to lungs
Closure is complete by 7-14 days of life; full closure may
not occur until 3 months of life
More common in girls than boys
May note continuous machine like murmur
In preterm population incidence may be 20-60%
Management:
If closure does not occur infant is at risk for CHF
IV indomethacin or ibuprofen to close
Ductal ligation in infants
At 6 months to 1 year of age can have interventional
cardiac catheterization to place coil
32. Pulmonary
stenosis
Narrowing of the pulmonary valve or pulmonary artery
-leads to RV hypertrophy
-MCC: valve leaflets have thickened and don't open properly
33.
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Assessment and
managment of
pulmonary
stenosis
Assessment
Infants may exhibit signs of mild right-sided CHF- include
enlarged liver and spleen and occasional edema
If narrowing is severe cyanosis may be present
Systolic ejection murmur
Second heart sound is usually widely spit
Echocardiography and ECG show right ventricle hypertrophy
In kids, one-side HF quickly progresses to complete HF
Management
Depends on severity of stenosis
Balloon through cardiac cath or replace valve (prosthetic
valve- heparin)
34. Aortic stenosis
Stenosis or stricture of the aortic valve
MC problem with valve itself
Normally has 3 flaps- needed for proper blood flow
Increased pressure and hypertrophy occur in the left ventricle
Pulmonary edema may occur
Assessment:
Can be asymptomatic
ECHO or ECG show left ventricle is hypertrophied or
thickened
Systolic murmur
Decreased cardiac output; weakened pulses, hypotension, tachycardia, and inability to suck for long periods of
time
Management:
Stabilization with beta-blocker or calcium channel blocker
to reduce ventricular hypertrophy
Surgery- use balloon to open, shave muscle, or replace
valve depending on cause
If insert prosthetic valve, have to be on heparin for rest of
life to prevent clots
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35. Coarctation of
the aorta
Narrowing of the lumen of the aorta due to a constricting
band (just passed where oxygenated blood travels to upper body)-- near ductus arteriosus
High pressure before narrowing lower pressure after narrowing
If pressure gets to high child is at risk for aneurysm, aortic
rupture of CVA
36. Assessment and
management of
coarctation of the
aorta
Assessment:
Absence of femoral pulses
Collateral circulation may develop and enlarged arteries
may be seen on the ribs as obvious nodules
BP in arms will be at least 20 mmHg higher than in the legs
Bounding upper body pulses
Management:
Intervention angiography using a balloon catheter (done
1st)
Surgery removes the narrowed portion of the aorta
In order for this to work the child needs to be at the majority
of their ht or have achieve they greatest part of their ht
If child is still growing, it can cause repair to reopen which
can be deadly
Can be maintained prior to surgery with digoxin and diuretics
37. Tetralogy of fall- This disorder causes a decreased pulmonary blood flow,
out
increase ventricular pressure (R to L shunting and poorly
oxygenated blood going into systemic circulation causing
cyanosis)
Four anomalies:
Pulmonary stenosis-- causes a decreased amount of
blood to be able to increase pulmonary circulation
VSD (ventricular septal defect)-- allow blood flow from R
to L ventricle
Dextroposition of aorta (enlarged aorta)-- seems that the
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aorta is coming out the right and the left ventricles and this
can cause problems with blood flow through the heart
Hypertrophy of the right ventricle-- decrease effectiveness
of right ventricular contraction
38. Assessment of May not have cyanosis after birth
tetralogy of fallot As they are active they show signs of cyanosis
Perform ECHO to see blood flow through heart- can see
pulm stenosis and RV hypertrophy
Cardiac cath to look at all the different defects and see if
all 4 are present
Loud harsh murmur or soft scratchy localized murmur
Polycythemia will develop- increased RBCs
Causes viscous blood
Severe dyspnea
Growth restriction
Clubbing of fingers
Smaller for age or FTT due to poor oxygenation
Hypercyanotic or "Tet" Spells
Results in cyanosis around mouth, fingers, toes and eyes
Pt at risk for emboli, seizures, loss of consciousness and
death
39. Therapeutic
Surgery- to repair each defect
management for Decrease hypercyanotic episodes- knee to chest position
tetralogy of fallot (improves pulmonary blood flow)
Older kids and squat to have same effect
Propranolol (Inderal)
Oxygen-- don't over oxygenate
Normal O2-- in 70s to mids 80s
If in the 90s getting too much O2
Morphine sulfate to increase pulmonary artery dilation
Full surgical repair can be done and without complications
can have a normal life
40. Transposition of The aorta arises from the right ventricle instead of the
the great arteries left, and the pulmonary artery arises from the left ventricle
instead of the right
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Creates 2 closed circuits of circulation and is incompatible
with life. Must have VSD or ASD present to allow oxygenated blood into systemic circulation
MC in large for gestational age infants
Assessment
Cyanotic from birth- born blue
Murmurs may be present- if have a ASD or VSD
Echocardiography shows enlarged heart
Management
Cyanosis is from lack of oxygenated blood reaching the
body. If ASD or VSD is not present the ductus arteriosus
must be kept open with prostaglandin PGE
4-7 days: perform arterial switch to correct circulation
41. Truncus arterio- One large vessel arises from left and right ventricle-- allow
sus
mixing of oxygenated and deoxygenated blood
One vessel coming from BOTH of the ventricles.
Increase blood to pulmonary circulation and decreases
blood flow to systemic circulation
As these children reach school age they will have to have
graft repeated because they have outgrown it.
Assessment
Infant is cyanotic from birth-- blue at birth
There is a VSD present with a murmur
Identification is made through echocardiography
Have to have graft placed to replace missing vessels
Need second surgery at school age to replace original
graft since it does not grow with them
42. Hypoplastic Left Accounts for 1.4-3.8%
Heart Syndrome Left ventricle is nonfunctional (it is underdeveloped)
Can be misdiagnosed in utero as a 2 chambered heart
Mitral and aortic valve atresia
Assessment
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Infant may not show cyanosis until hours after birth
Once ductus arteriosus closes
X-ray may show right side hypertrophy
They don't eat well, tire easy and have tachypnea, tachycardia and hypothermia
Management
To keep the blood mixing prostaglandin is given to keep
PDA open
Nitrogen mixed with oxygen-- decrease PO2 and increase
pulmonary resistant
Allow R side of heart to shift more blood to left side and
aorta
Only true fix or cure is a heart transplant
Requires a three stage surgery-- patches if cannot get
transplant
Norwood done within first week
Glenn done 3-8 months later
Fontan done 18 mth-3yrs of life
43. Congestive heart CM:
failure
Tachycardia is usually seen first
Tachypnea
Hepatomegaly (right sided)
Irritable and restless-- from abdominal pain cause by liver
distention
Lower extremity edema occurs later in children than adults
Orthopnea (left sided)-- blood accumulates on pulm system
Rales-- d/t pulmonary congestion
Bloody sputum-- d/t broken capillaries in the lungs under
increase pulmonary pressure
Cyanosis-- from pulm edema
Apical heartbeat is displaced
Will do an echo to determine the cause of the HF
Can determine ventricular hypertrophy with an ECP
Enlarged heart
Galloping heart rhythm
Accentuated third heart sound
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CM of infants:
Tachypnea and tachy at rest
Dyspnea
Retractions
activity intolerance/feeding intolerance
More subtle
May become breathless
Tires easily
Diaphoretic
Difficulty sucking
Abrupt weight gain-- fluid retention
Enlarged liver-- hepatomegaly
Periorbital edema
44. Therapeutic
management of
CHF:
Reduce the workload of the heart
Evacuate accumulated fluid with diuresis
Slowing heart rate and strengthening cardiac function with
inotropic drugs
Reducing afterload with vasodilator
REST: needed
Need a neutral thermal environment-- don't let them get
cold/shivering
May have fluid/sodium restriction
May need K+ supplement if taking furosemide
Tx any existing infection
Semi-folwers positions to reduce respiratory effort
May have to sedate irritable child
Decrease environmental stimuli to keep them calm.
45. Medications for
CHF:
Diuretics: furosemide (Lasix)
Increase contractility: digoxin
Decrease afterload: proranolol, or captopril
46. Oxygen and CHF Provide oxygen if child has dyspnea, hypoxemia, or
cyanosis
Monitor pulse oximetry
Monitor nares if using prongs
Humidified oxygen more comfortable
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47. Digoxin adminis- Family teaching
tration
Apical pulse
Family needs to know how to do it
Take stethoscope home with them
Narrow margin of safety
0.8-2 =therapeutic range
Administer same time
Use clearly marked syringe
Do not change amount
If a dose is omitted call health care provider
Give 1 hour before or 2 hours after a feeding (to avoid
being lost with spit up)
If a dose is vomited do not repeat
Keep follow up appointments
48. Digoxin toxicity
Harder to monitor in infants
Monitor apical heart rate
Observe for anorexia, nausea, vomiting, dizziness, diarrhea, headache, arrhythmia, and bradycardia
Monitor serum levels
49. Cardiac surgery Baseline vitals
pre-op
Initial BP, then have child lay down and rest for about 15
mins to use for the baseline BP after surgery
Record height and weight
For drug calculations and to check blood loss and edema
after surgery
Digoxin usually held 24 hours prior
Can cause arrhythmias while they are in surgery
Enema prep
Helps reduce strain on the heart post operatively.
Surgical prep of chest
Not over left side of the chest, through sternal bone or back
Preoperative teaching
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Make sure that the parents are there and they are oriented
to the unit
Talk to them about equipment (O2, monitors, chest tubes)
If child is old enough, explain to them what is going to
happen and what to expect when they wake up
50. Cardiac surgery Monitor fluid volume-- look for fluid volume overload
post op
Monitor UOP via foley cath should be 1mg/kg/hr
Central venous pressure monitoring
Prevent pooling of secretions
Pulmonary artery pressure
Monitor for infection
b/c in the first 24-72 hours the incision remains open to
allow swelling to decrease before they close
Monitor for hypothermia
Cooled during surgery, will have hyperthermia and warm
heat to warm them back up after surgery
Two drain tubes
Encourage child to cough and deep breath or use incentive spirometry
51. Complications
from cardiac
surgery
Hemorrhage: heparin is used during cardiopulmonary bypass
Antidote: protamine sulfate
Shock
Look for hypotension, decrease UOP, acidosis and
cyanosis
Need plasma volume expanders
Ventilation and possibly back to the OR if excessive bleeding
Congestive heart failure
Tx same pre and post op
Decrease workload of heart
Need furosemide, dig, and possibly captopril
Post cardiac surgery syndrome: after first postoperative
week
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Inflammation of the pericardium and fluid collecting in the
pleural cavity
Tx with anti-inflammatory therapy and bed rest
Postperfusion syndrome: 3-12 weeks after surgery
Occur in response to use of cardiac bypass
Fever, enlarged sleep, maculopapular rash and general
malaise
Usually short lived
52. Persistent pulmonary hypertension
When the high pulmonary vascular resistance present at
birth fails to resolve
Present because you have a higher pressure on the right
side of the heart
Vasoconstriction of the pulmonary artery because of increased pulmonary blood flow, obstruction, disease
Assessment:
Hypoxia
Poor perfusion
Dyspnea
Tachypnea
Pulse oximetry levels are low despite use of oxygen
Chest pain
Prolonged cap refill
Shock
Dizziness
ECHO will show right to left shunt across the PDA
Management:
Supportive therapy to promote vasodilation
May receive coumadin to prevent emboli
Inhaled nitric oxide
ECMO (extracorporeal membrane oxygenation)-heart/lung bypass
53. Rheumatic fever Autoimmune disorder that reacts to group A beta-hemolytic streptococcal infection
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Some people are genetically susceptible for developing
rheumatic fever after strep infection
Once you have it once, you are at risk for having it repeatedly
Occurs most often in children 6-15 years of age with peak
being 8 years
Seen in poor crowded urban areas
IF PROPER TX IS NOT RECEIVED for strep:
Child can develop rheumatic fever
54. Assessment for
rheumatic fever
nursing care guidelines (need 2 major and 1 minor or 1
major and 2 minor and ASO titer positive for strep)
Major:
Carditis
Erythema marginatum
Subcutaneous nodules
Polyarthritis
Chorea-- shakiness in extremities
Minor:
Fever
History of previous rheumatic fever; prolonged P-R interval
Arthralgia
Elevated sedimentation rate; C-reactive protein; leukocytosis
55. Therapeutic
management &
nursing care for
rheumatic fever
PREVENTION #1 (of strep infections)
Course is 6-8 weeks
Bed rest in acute phase
Antibiotic
PCN is DOC to tx strep infections and rheumatic fever
Salicylates
Monitor cardiac system
Monitor for chorea
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Nursing care:
Compliance with Drug Regimen
Low dose aspirin therapy
Tx prophylactically before invasive procedure w/ anbx
Ensure when child is dx with strep, educate parent to
complete FULL course of antibiotics
Facilitate Recovery
Rest
Good nutritional
Will need lifelong follow ups to monitor for complications
caused by rheumatic fever
Provide Emotional Support
56. Kawasaki syndrome
Acute systemic vasculitis
Infectious trigger
Peak incidence at toddler age
Self-limiting
Illness lasts 6-8 weeks
Assessment:
3 phases:
Acute phase: high fever unresponsive to meds
Trunk rashes
Conjunctivitis
Reddened and swollen hands and feet
Strawberry tongue; red cracked lips
Lethargic
Extreme irritability-- continues throughout entire course of
the illness
Subacute: fever will resolve, hands/feet will start to peel.
Greatest risk for cardiac abnormalities
Convalescence : all symptoms disappear, phase is completely over when labs have returned to normal.
Dx:
Based on S/S
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Fever more than 5 days - unresponsive to antibiotics
Diagnostic criteria - often not all are met
57. kawasaki syndrome
58. Therapeutic
management of
kawasaki
IV immune globulin (IVIG)-- 1x in first 10 days
Reduced fever and cardiac complications
Treated as blood product, will be in the hospital when they
receive this
Can take 2-8 hours depending on how fast it is going to
red
Acetylsalicylic acid (aspirin)
To control fever and inflammation
May be on it the entire 6-8 weeks
Monitor for signs of cardiac involvement
Look for murmur
Need rest
Calm, quiet, dark room.
Clear soft diet
At risk for dehydration
Monitor I&O
Provide mouth care & skin care
Provide family support
Cautious reassurance regarding dx
Offer them respite care
Caution parents children should not receive routine immunizations while taking IVIG
Coronary artery bypass may be necessary in future
59. Patient/family
teaching r/t
Kawasaki
Meds:
Low dose ASA
Compliance:
Daily temp; daily meds
Regular follow-up
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Pediatric cardiologist may have to have ECHO to determine if cardiac involvement is present
How to monitor cardiac ischemia
CPR teaching
Discuss potential of infarction
60. Sickle cell anemia:
-An autosomal recessive disorder, causing sickle hemoglobin
-Sickle hemoglobin caused RBC's to become sickle
shaped when stressed
Low oxygen tension
Low blood pH
Increased blood viscosity
Can be brought on by dehydration or hypoxemia
No S/S for SCA till after 4-6 mo. Due to presence of fetal
hemoglobin
61. S&S of SCA:
Obstruction caused by sickling of cells
Can lead to crisis
Vaso-occlusion: extremely painful
leading to ischemia and crisis,
resulting in tissue and organ injury
Profound anemia- aplastic crisis
Hyperhemolytic crisis
Destruction of sickled cells
acute S&S:
pain in joints, bones and abdominal (can be mild/severe,
short/long lasting, triggered by: stress, cold, heat, dehydration)
62. SCA symptoms
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63. Types of crisis r/t Spleen Sequestration crisis
sickle cell
Large amount of pooling in blood in spleen/liver, can cause
shock and splenomegaly
Aplastic crisis
Severe, transient decrease in RBC production, usually
from a virus
Causes severe anemia
Acute chest syndrome-- will present like PNA
Occurs in adolescents, an area in lungs where the sickle
cells had adheres caused decreased oxygenation
Symptoms: fever, tachypnea, wheezing, cough and PNA.
64. Chronic S&S of
SCA:
Anemia: destruction of the cells results in this
Cardiomegaly
Cholelithiasis
Delayed growth/development
May be smaller than normal
Delayed puberty or absence of puberty
65. Dx of SCA
Prenatal possible
Via amniocentesis or CVS.
Neonatal screenings
Mandatory blood clot screen when born
Early Diagnosis
ESSENTIAL
Sickle turbidity test (Sickledex)
Electrophoresis: take a sample of blood, separate the
protein/hbg with high voltage to look for specific proteins
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that make the cell "sickle" shape.
CBC
elevated reticulocyte count
Differential shows sickled cells
66. Management of
SCA
Home Care
Prophylactic antibx- infections can bring on a crisis.
Do not participate in sports or long distance running
Lack of oxygen can lead to crisis
Do not fly in under pressurized aircraft
If they have a viral infection:
Keep patient hydrated, dehydration can lead to a crisis.
seek medical intervention with fever--ASAP
prevention & recognition
Hospitalization
Pain management
8-10 pain level: opioid
Tx pain as it presents
treat underlying conditions
Infection, dehydration
IVFs
O2
antibx
67. Hemophilia:
Bleeding disorders
Caused by deficiency of clotting factors: VIII, IX, XI
Most common: Hemophilia A (VIII)
Passed on through males with X linked recessive inheritance
Passed from mother to male offspring
68. S&S of hemophil- Prolonged bleeding
ia
Hemorrhage into joints and muscles
Can cause joint deformities
Repeated bleeds into joints can cause deformities and
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severe pain
Hematomas / bruising
No contact sports
Good options would be long distance running or anything
that they do not have a risk to cause bleeding
Internal bleeding most dangerous
In GI tract, peritoneal cavity or CNS
69. Tx of hemophilia IV clotting factor ASAP after injury or bleeding
DDAVP- synthetic form of vasopressin
Kids 8 and over can give it to themselves (depending on
maturity)
Expensive tx
Prophylactic infusions of clotting factors
Prevention of bleeding
Toddlers: no sharp corners, wear helmet to protect head
from falls, carpeting instead of hardwood floors
Avoid contact sports
Safe environment for toddlers
Avoid contact sports
Physical therapy to strengthen muscles / joints
Can decrease the # on spontaneous episode
MEDIC alert bracelet
Cautiously use NSAIDS--bleeding risk, inhibits platelet
function
Avoid IM injection, finger stings, and ASPIRIN
NO CURE--- do not grow out of it
Potential for gene therapy in the future, not approved yet
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Family support/home care teaching
Keep child safe/prevent bleeding episode
Med admin @ home
Weekly infusion, every other week, or monthly infusion
(Depends)
May need genetic counseling
You can ID carriers of hemophilia through DNA testing
Want the kid to have responsibility in relation to disease
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