Final Exam Review Pt. 2
CHILD WITH SPECIAL NEEDS
9 questions
Black = Lecture Notes
Gray = Textbook Notes
1
Red = Fall 2021 Test Questions
Orange = Spring 2021 Test Questions
Blue = Spring 2021 Quiz Questions
Purple = Final Spring 2021 Test Questions
Green = Medications/Tx. to Know
 Family Centered Care
o Family functioning, communication, therapeutic relationships, role of culture, shared
decision making
o Family is the child’s primary source of strength and support
o Hospitalization alters the parental role; nurses should be role models and mentors for
engaging parents in children’s hospital routines and daily care that benefits the
Test Q: What
child
trend of care
allows for a child
 Trends in Care:
to develop social
o Normalization: establishing a normal pattern of living
skills, sense of
 Family life, delegation, routines, participation, age-appropriate activities
self, etc.
Test Q: A child has
o Home Care: normalize, lessen family disruption, maximize G&D
 Mainstreaming
established a
 promotes self-enabled routine when appropriate
normal pattern of
living with
o Mainstreaming: process of integration of the child into the classroom or daycare with
routines and agepeers
appropriate
Quiz Q: how would
 Develops sense of self, understand and respect themselves and peers,
activities?
mainstreaming
gain acceptance, socialization skills
 Normalization
benefit a child with
o Early Intervention: PT, speech therapy, OT, IFSP
Down’s Syndrome?
Test Q: Parents with a
o Managed Care: reduce barriers, quality, training of
 increased
recently diagnosed
openness
families and communities
special needs child are
 Assisting the Family with Feelings:
asking question if the
o Shock & Denial
child will ever get better.
What stage are they in?
o Adjustment
 Shock & Denial
o Reintegration & Acknowledgment
o Support System
 Cognitive Impairment = Any Type of Intellectual Disability
o Consists of 3 Components:
 IQ
 Adaptive Behavior – ability to plan, reason, solve problems, think abstractly,
and be able to meet demands for culturally appropriate demands of life
Test Q: Which
 < 18 years old at diagnosis
cognitive impairment
has genetic ties?
 Ex. Down’s Syndrome, Fragile X Syndrome
Matching.
o Etiology = social, familial, environmental, organic, and unknown causes
 Down’s Syndrome,
 Chromosomal disorders, prenatal toxin exposure, cerebral palsy, microcephaly,
Fragile X
infantile spasms
o Primary Prevention:
 Avoid Prenatal Rubella
 Smoking or Alcohol Use
Infection
 Reduce Head Injuries
 Genetic Counseling
 Future Gene Therapy
 Folic Acid
(PKU)
o Secondary Prevention:
 Early identification to avoid damages (hypothyroidism, PKU, galactosemia)
o Tertiary Prevention:
 Treatment to minimize long-term consequences
Final Exam Review Pt. 2
o


Test Q: Which of the
following are
commonly associated
with down’s
syndrome?
 Otitis Media and
Septal Defects
2
Development:
 Delayed Verbal Skills
 Socialization
 Promote Independence
 Sexuality
 Discipline
 Play & Exercise
o Care of Child with CI:
 Detailed history with special focus on self-care abilities
 Questions about abilities, approach positively
 Assess developmental age
 Communication with child appropriate to cognitive level
 Address potential loneliness
Developmental Disability = Any significant lag or delay in physical, cognitive, behavioral,
emotional or social development
 Language & cognitive skill delays, fine & gross motor skills delay
o Developmental Screening = Early Identification
Down’s Syndrome (Trisomy 21)
o Most Common Chromosome Abnormality AND Genetic Cause of CI
o Risk Factors: (exact cause unknown)
 maternal age > 35 years old (80%)
 paternal age > 55 years old (< 5%)
 genetic predisposition (97%)
o Manifestations:
 Separated Sagittal
 Excess Skin in Neck
Sutures
Folds
 Palpebral Fissures (slant
 Hyperflexible
eyes)
 Hypotonia/Muscle
 Transverse Palmar
Weakness
Crease
 Hearing and Visual
 Small Nose/Flat Bridge,
Losses
Small Ears
 Hypotonicity
 Short Rib Cage, Large
Protruding Tongue
 Wide Variations of IQ: mild  moderate
 Initial development normal; strength in sociability
 Social development may be 2-3 years ahead of mental age
 Hypersensitive to Touch & Sound
o Congenital Anomalies:
 Heart Defects – Septal Defects
 Congenital Heart Disease – AV CANAL!
 Hirschsprung
 Tracheoesophageal Fistula
 Otitis Media
 Altered Immune Function
Test Q: Most common
 Musculoskeletal – Atlantoaxial Instability
comorbidity associated
 Sexual Development/Growth
with down’s syndrome
o Comorbidities = CONGENNITAL HEART DISEASE
is:
o Treatment & Prognosis:
 congenital heart
disease
 Atlantoaxial Instability: urgent if s/s of spinal compression
Final Exam Review Pt. 2
Test Q: Which tests
would be accurate in
prenatal diagnosis of
down’s syndrome?
 CVS,
Amniocentesis, AlphaFetoprotein

Test Q: Why is Fragile
X most common in
males?
 X-Linked Recessive,
males have
nonfunctioning X
3
 Avoid activities stressful to head & neck
 May require surgical intervention
 Life Expectancy: majority live to be 60+
 Remains lower than general population
 Help Family Prevent Physical Problems – positioning, feeding, attachment, URIs,
ear infections, etc.
o Prenatal Diagnosis + Genetic Counseling:
 Offer to pregnant women of advanced maternal age or with family history
 Chromosomal Villus Sampling
 Amniocentesis
 Alpha-Fetoprotein
o Early Intervention:
 Address developmental delays and help child achieve full potential
Test Q: What
should the nurse
 Therapy, exercises, activities
promote when
 Begin ASAP
educating
o Education:
parents of a child
 Teach patients & caregiver importance of independence; ways to
with down’s
syndrome?
improve child’s independence
 ways to
 Promote Optimal Development
improve child’s
 Guidance for establishing acceptable social behavior, personal
independence
feelings of self-worth, esteem, and security
Fragile X Syndrome
o Most Common Inherited CI; 2nd Most Common Genetic Cause (after down’s)
o Males = nonfunctioning X; Females = one functioning X, one nonfunctioning X
o Etiology – X-linked Recessive; DNA Testing
o
o
Classic Physical Appearance:
 Large Head
Circumference, Long
Face & Ears, Palpebral
Fissures, Strabismus
 Palate (high-arched)




Macroorchidism
(enlarged gonads)
Hyperflexible Finger
Joints
Palmar Crease
Flat Feet
Classic Behavioral Features:
 Mild  Severe CI
 normal IQ with learning difficulties
 Delated Speech & Language
 Hyperactivity, Aggression
 Autistic-Like Behaviors
 Hearing Impairment
o Slight  Profound
 Slight  Moderately Severe: residual hearing sufficient to process linguistic
information; generally, with hearing aid
 slight (16-25 dbs.), mild to moderate (26-55 dbs.), moderate to severe
(56-70 dbs.)
Final Exam Review Pt. 2
Quiz Q: Why is a
child’s risk of hearing
loss higher if they
were in the NICU for
long periods of time?
 continuous
humming noises from
equipment

4
Severe  Profound: precludes processing of linguistic information with or
without hearing aid
 severe (71-90 dbs.), profound (>91 dbs.)
o Etiology: family history, anatomic malformation, low birth weight, ototoxic drugs
(gentamycin), chronic ear infections (OM), perinatal asphyxia or infection, prenatal
substance abuse, cerebral palsy
 Sensorineural Hearing Loss in NICU babies with continuous equipment humming
noises
o Pathology:
 Conductive Hearing Loss: middle ear [OM, foreign bodies, medications,
cerumen impaction]
 Sensorineural Hearing Loss: auditory nerve or inner ear
 Mixed Conductive-Sensorineural Loss: may follow recurrent OM with
complications
 Central Auditory Interception: organic or functional
o Therapeutic Interventions:
 Conductive Defects
 Medication/Antibiotics
Test Q: What hearing
defects are hearing aids
 Hearing Aids
not beneficial for
 Sensorineural
treatment?
 Hearing Aids NOT Beneficial
 Sensorineural
 Cochlear Implant
o Assessment:
 Lack of Startle Reflex
 Absence of Babbling by 7 Months
Final Q: What is
 General Indifference to Sounds
indicative of hearing
 ID before 3 months; no later than 6 months
loss in infants?
 Language & Educational Development, Hearing/Speech Development
 lack of startle reflex
o Lip reading, sign language, picture books
 Parental Concerns: refer for hearing evaluation
o LISTEN TO PARENTAL CONCERNS
o Prevention:
 Treatment & Management of OM Reoccurrence
 Prenatal Preventive Measures
 Avoid Exposure to Noise Pollution
 Visual Impairment
o Partially Sighted: 20/70 – 20/200
o Legal Blindness: 20/200 or less
o Etiology – perinatal or postnatal infections (gonorrhea, chlamydia, rubella, syphilis),
retinopathy of prematurity, perinatal/postnatal trauma, other disorders and visual
problems, unknown causes
o Visual Impairment:
 Myopia: nearsighted
 Amospmetropia
 Hyperopia: farsighted
 Amblyopia: lazy eye
 Strabismus: misaligned
 Cataracts
 Astigmatism: uneven
 Glaucoma
curvatures in vision
Final Exam Review Pt. 2
o
5
Assessment & Promotion:
 Infants – expect binocularity by age 6 months (depth perception)
 Response to light, pupillary response, visual evoked response, ability to
follow a target
 Childhood – Visual Acuity Testing
 Headaches, rubbing eyes, vertigo, poor school performance, tilting head
to one side, closing one eye to see
 Promoting Optimal Development – play and socialization, independence,
education, braille, audiobooks, safe environment, reassurance, orient child to
surroundings, consistency of team members
 Plan of Care:
 Safe environment, orient to surroundings, encourage independence,
consistency of team members
 Management:
 Prenatal Care/Prevention of Prematurity
 Rubella Immunizations  FOR ALL CHILDREN!!
 Periodic Screening
Final Q: What is the
 Safety Counseling
historic cause of hearing
 Education on Glasses/Contacts – involve optometry PRN
and visual impairments
 Refer to Support Groups
in children?
 Rubella
 Historic Cause of Hearing and Visual Impairments in Children = Rubella!!
 Communication Impairment (emotional & social deprivation)
o Inability to receive, process, transmit, or represent symbol systems
o Delayed Language & Speech – most common developmental delay
 Speech problems are more prevalent than language disorders
 Both types decline as children grow older
o M-CHAT – modified checklist for autism in toddlers
o Nursing Care:
 Primary Intervention = Prevention
 Understanding normal speech & language development
 Assessment, Referral, Education
 Autistic Spectrum Disorders (ASD)
o Complex brain dysfunction accompanied by broad range and severity of intellectual and
behavioral deficits. Reassure parents
 NOT RELATED TO MMR VACCINE – diagnoses at same time as vaccine
 Encompasses: Autistic Disorder, Asperger Syndrome, and Pervasive
Test Q: What is the
Developmental Disorder
best plan of care for a
o Etiology:
child with Autism?
 Unknown, Genetic Basis
 highly structured
 Appears 18-36 months of age; 4x more common in males
routines and intensive
behavior modification
o Nursing Considerations:
programs
 No cure; most promising results in highly structured routines and
intensive behavior modification programs, importance of family counseling
o Nursing Care:
 Primary Intervention = Prevention
 Understanding normal speech & language development
 Assessment, Referral, Education
Final Exam Review Pt. 2
6
 Premature and “At-Risk” Infants of CI
o Classifications:
 Birth Weight
 LBW = < 2500g
Test Q: What is the best
indicator of high-risk
 VLBW = < 1500g
infants for CIs?
 ELBW = < 1000g
 Gestational Age
 Gestational Age (best indicator)
 Pathophysiologic Problems
 Congenital Anomalies
o Nursing Care:
 Developmental Support – assessments, NICU
 Controlled Environment – auditory/noise control, cluster care
 Special Handling – kangaroo care, gentle touch, facilitative tucking
 Assessments – APGAR, GA, Skin Assessments, Vital Signs, Temperature
 Maintain thermoneutrality (overhead warmer PRN)
 Fluid & Electrolyte Balance
 IV Fluids
 Nutritional Needs
o Gavage  Nipple  Breastfeeding
o Tolerance, Success, Resistance to Feeding
 Medication Administration
 Potential for drug toxicity and adverse drug reactions
 Parent-Infant Relationships
 Initial Shock and Denial
 Adjustments to Environment
 Parental Support – family support systems, parent care skills
o Apnea of Prematurity
 Decreased Age = Increased Incidence
 Origins: Central, Obstructive, Mixed
 Monitoring: at home or in the hospital
 Treatment:
 Stimulate, Resuscitate, Document
 Pharmacologic Interventions:
o Caffeine (most widely used)
o Theophylline or Aminophylline (CNS Stimulants)
 monitor drug toxicity vs. therapeutic levels
o Respiratory Distress Syndrome (RDS)
 Pathophysiology: lack of surfactant in the lungs contributes to alveolar collapse
 affected lungs become stiffer and require more pressure to achieve
expansion, increased transudate of fluid is produced and further impairs
gas exchange
 Clinical Manifestations/Diagnostics:
 Retractions – Substernal, Subcostal, Intercostal
 Nasal Flaring
 Grunting – with stethoscope or naked ear
 Therapeutic Management:
 Ventilatory Support, Conventional Ventilation
Final Exam Review Pt. 2
o
o
o
o
o
7
Bronchopulmonary Dysplasia (BPD) & Chronic Lung Disease (CLD)
 O2 Requirements or Mechanical Ventilation >36 weeks of age
 May be marker of long-term pulmonary and neurologic outcome
 15-40% of preterm infants – inversely rated to BW and GA
Meconium Aspiration Syndrome
 Fetal Stress = Asphyxia
 Primary Asphyxia: gasping aspirations
o In utero = meconium aspiration
Infections (Neonatal & Maternal)
 Neonatal Infections –
 Neonatal Sepsis
o Early Onset (3 days post-birth): acquired during perinatal period
o Late Onset (1-3 weeks post): Nosocomial or Health-Care
Associated Infection (HAI)
o Diagnostics:
 Cultures of Blood, Urine, CSF
 CBC, HgB, Hct
o Therapeutic Management:
 Antibiotic Therapy (2, 10, 14 days)
 Oxygen, Fluids
 PRBC Transfusion
 Maternal Infections –
 During early gestation can result in fetal loss or malformations because
of the ability of the fetus to handle infectious organisms is limited and
the fetal immunologic system in unable to prevent the dissemination of
infectious organisms to various tissues
 Mother is tested for TORCHES Complex:
o Toxoplasmosis, Other (Hep. B, Parvo, HIV, Varicella, Measles,
Mumps), Rubella, Cytomegalovirus, Herpes Simplex, Syphilis
Retinopathy of Prematurity
 A disease of preterm infants that affects the blood vessels of developing retina
 Severe Cases Results In: Retinal Detachment, Severe Visual Impairment,
and Blindness
 Risk Factors: BW, GA, Hypoxemia/hypercarbia, Excessive O2
Neurological Problems:
 Hypoxic-Ischemic Brain Injury
 Most common cause of neurological impairment, results from asphyxia
before, during, or after delivery
o Ischemia and Hypoxia – simultaneously, or not
 Intra-Ventricular Hemorrhage (IVH)
 Most often in infants less than 32 weeks of gestation
 May Result in CNS/Motor Problems  Cerebral Palsy (CP), or Seizures
 Causes:
o Birth Trauma
o Hypoxic Insult
o Increased ICP or Intrathoracic Pressure
o Hypertension
Final Exam Review Pt. 2
8


o
o
Volpe Classification:
o Catastrophic Deterioration – rapid deterioration to coma or
deep stupor
 Respiratory Abnormalities, Cardiac Arrhythmias
 Fixed Pupils, Decerebrate Posturing, Generalized Tonic
Seizures, Flaccid
o Saltatory Deterioration – subtler, signs may stop altogether
then reappear
 Altered LOC, Hypotonia, Subtle Abnormal Eye Position
 Decreased spontaneous or abnormal movements and
an abnormally tight popliteal angle
o Clinically Silent Deterioration – often overlooked clinically
 Sudden, unexplained decrease in hematocrit may be the
only clinical sign of IVH
Grading and Prognosis:
o Higher Number = Poorer Outcome
 I  III
o Neurological Consequences, Unpredictability
o Focus on Prevention of IVH
Hypoglycemia
 Signs:
 Irritability, Jitteriness
 Eye Rolling, Poor Feeding
 Seizures, Hypotonia
 Cyanosis, Apnea
 Management:
 Bolus of IV Glucose – if unable to immediately feed
o If using D-15 or D-25 – must infuse via central catheter
(UAC/UVC)
Drug Exposed Infants
 S/S of Withdrawal in Neonates:
 Irritability, Tremors, Shrill Cry
 Hypertonic Muscles
 Poor feeding, Vomiting, Diarrhea
 Seizures
 Sleep Disturbances, Fever
 Diaphoresis
 Nasal Stuffiness, Sneezing
 Tachypnea, Hyperactivity
o NALOXONE = Avoid if Infant is Suspected of Drug Exposure
 May precipitate neonatal WD symptoms
 Opiate Exposure
 Cocaine Exposure: unlikely to show NAS
 Toxicology Screening
 Neonatal/Pediatric Effects – following intrauterine exposure
 Marijuana Exposure
 Methamphetamine Exposure: appear to be dose related
Final Exam Review Pt. 2
9


Test Q:
T/F – Fetal Alcohol
Syndrome is 100%
preventable.
 TRUE
o
Higher incidence in cleft lip/palate, cardiac defects, preterm delivery,
and placental abruption
 Small Head Circumferences and Low Birth Weights
 Newborn Brady/Tachycardia
 Delays in Gross and Fine Motor Coordination
 Behavioral Changes – decreased arousal, increased stress, alterations in
movement
Alcohol Exposure
 Fetal Alcohol Syndrome (FAS): leading cause of cognitive impairment
o Cognitive & Motor Delays
 Microcephaly, Poor Coordination, Hypotonia
o Hearing Disorders
o Facial Dysmorphia
 Short palpebral fissures, smooth philtrum, thin upper
lip, short upturned nose
o Irritable, Hyperactive
Tobacco Exposure
Polydrug Exposure


ADHD
 Complete/Thorough Multidisciplinary Evaluation
 Behavior Checklist/Assessment Tools – IQ, Hand-Eye Coordination, Visual &
Auditory Perception, Comprehension & Memory
 Therapeutic Management:
 Behavioral Therapy, Classroom Structures
 Family Education/Counseling, Environmental Manipulation
 Medication – not all children benefit
o Stimulants: Dexedrine, Amphetamine, Adderall, Ritalin
 Side Effects: insomnia, anorexia, hypertension
 Long-Term Use  Suppressed Growth
 Psychotherapy, Psychologic, Social Therapies
 Special Needs Children Reaction to Hospitalizations  minimize loss of control
o Infants to Preschool: separation anxiety, protest, despair, detachment/denial
Test Q: A 1-year old
o Early Childhood: separation anxiety greatest reaction of protest – goal-directed
child’s reaction to
hospitalization will
o Childhood to Adolescence: loneliness, fear, anger, sadness, stress/regression,
be what?
cooperation, sleep disturbances
 Separation
o Families:
Anxiety
 Parents – stressed, angry, guilt
 Siblings – lonely, left out, stressed
 Minimizing Loss of Control:
o Promote freedom of movement, parent/child contact
o Prevent fear of bodily injury, maintain routine & independence of child
o Patient-family-centered care nursing
 Pediatric Nursing & Consent
o Informed Consent = age of majority/competence
o Assent = process of obtaining informed consent < 18 years old
Final Exam Review Pt. 2





10
Child is informed and willing, developmentally appropriate awareness, patient
educated on expectations, understanding assessed, soliciting expression of
child’s willingness
o Emancipated Minor = legally underage, recognized to have legal capacity of adult
 Pregnancy, marriage, high school graduation, independent living, military
o Treatment NOT Requiring Consent – STIs, mental health, alcohol/drug dependencies,
pregnancy, contraceptive advice (exception – sterilization)
Temperature Regulation in Children
o Set Point, Fever, Hyperthermia
 Treatment = DANTROLENE (1st line treatment of malignant hyperthermia)
o Febrile Seizure
 Antipyretics, Anticonvulsants – little evidence; Antipyretics = increase comfort
Infection Control:
o Standard – all contact with any bodily secretion
o Airborne – mask, gown, gloves (N95/Hood)
o Droplet – mask, gown, gloves
o Contact – gown, gloves
Restraining Methods:
o Behavioral Restraints – antianxiety medications, relaxation techniques
o Positioning for Procedures = swaddle (analgesia, sedation)
o Physical Restraints – therapeutic hugging, jacket restraints,
Test Q: On what
mummy/swaddle, limb restraints
group are physical
Alternative Feeding Techniques:
restraints used on
o Gavage Feedings (NG/OG/NJ)
most?
 Gavage = gravity; bolus
o G-Tube/J-Tubes
 Pumps – continuous or intermittent feedings
o Totally Parenteral Nutrition (TPN)
 Used when GI feeding is not possible – central line vs. peripheral line; intralipid
infusion
o Use pacifier during alternative feedings – nonnutritive suckling improves digestion
Final Exam Review Pt. 2
11
CARDIOVASCULAR DYFUNCTION
9 questions
 Congenital Heart Disease (CHD) – abnormal anatomical defect that results in abnormal cardiac
function (CHF/Hypoxemia) and is present at birth
Final Q: Matching: CHD
o results in altered hemodynamics, flow, and pressure
and AHD.
 2 Categories –
 CHD: results in
 CHF: defects that result in left-to-right shunting
CHF/Hypoxemia
 AHD: seen in in normal
 Hypoxemia: defects that result in decreased pulmonary
heart or CHD
blood flow cause cyanosis
 Maternal Risk Factors for CHD –
 Rubella in Pregnancy, Fetal Drug Exposure, Increased Maternal Age,
Maternal Metabolic Disorders, Genetic Factors, Environment, etc.
 Genetic Association with CHD –
 Familial Tendency; Unknown Genetic Reason
 CHD Presentation –
 Cardiac Failure, Cyanosis, Poor Feeding
 Left-to-Right Shunt, Altered Pulmonary Blood Flow
 Acquired Heart Disease – disease process that occur after birth; seen in normal heart or CHD
o Result of: infection, autoimmune response, disease process, environmental factors,
familial tendencies
Embryology/Fetal Circulation:
o Development begins at 4 weeks and is complete by 8 weeks’ gestation (note: lungs
nonessential in utero) – heart beats on day 28
Test Q:
 Structural Defects: can be ID’d by 18-20 weeks
something about
o
Structures:
fetal structures
of DA and PO
 Foramen Ovale (opening between atria)
 Ductus Arteriosus (opening between great vessels – aorta & pulmonary aa.)
 Conduction Tissue – maintains orderly and effective pumping action (SA Node = Pacemaker)
o VSD = Damage to Conduction Tissues
 Bypass may need to be done, pacemaker for permanent damage
 ECG review:
o Evaluates Conductive Tissue & Records Electrical Activity
 P Wave – atrial depolarization n
 PR Interval – delay AV node  filling ventricles
 QRS Complex – depolarization of ventricles  main pumping action
 T Wave – ventricular repolarization
 QT Interval – time taken for ventricular depolarization n& repolarization n
 ST Segment – ventricular repolarization begins (flat)
o 3-lead: right – white, left - smoke (black) over fire (red)
o 5-lead: right – white over green, left – black over read, brown in middle
o 12 -lead: diagnostic & selectively identifies – 6 second strip to compare
 Cardiac Output = HR x SV
o Preload – volume of blood returning to the heart (filling of the valves during diastole);
circulating blood volume
 Starling’s Law = an increase in ventricular end-diastolic (caused by increased
preload) increases stroke volume
Final Exam Review Pt. 2
Test Q: Best
indicator to
measure CO of
a pt. with a low
preload?
 I&Os, Pedal
Pulses


o
o
12
Direct Measurements: circulating blood volume using CVP
Indirect Measurements: balance of I&Os, pedal pulses (best indicator)
 Reduce Preload By: diuretics, restrict/give fluids, I&Os, daily weights
Afterload – resistance the heart (LV) has to work against for ventricular ejection
 Increased when conditions make it hard to pump blood into circulation
 Indirect Measurements: Blood Pressure (higher = increased afterload)
 Direct Measurements: central line or SWAN (direct pressure inside
heart), SVR, PVR
 Can reduce afterload with ABCs – ace inhibitors, beta blockers, CCBs
 Improve tone after afterload – DA, EPI, NE
Contractility – ability/efficiency of heart muscles to shorten and contract (Ca+)
 Assessed by: urine output & peripheral tissue perfusion
 Give: Digoxin to increase contractility
 Pulses, extremity warmth, capillary refill
 Normal Heart Pressures
o Fetal Circulation –
 Placenta supplies blood via umbilical veins 
Blood enters 1) portal and hepatic circulation of
liver or 2) directly to IVC via ductus venous 
Test Q: Which is true
right atrium  foramen ovale  left atrium 
about fetal circulation?
aorta  head & upper extremities  SVC 
 pulmonary artery is
shunted through the
right atrium  tricuspid valve  right ventricle
ductus arteriosus to the
 pulmonary artery  shunted through ductus
descending aorta
arteriosus  descending aorta  returned to
placenta from descending aorta through
umbilical arteries
o Neonate Vascular Changes –
 Before Birth: high PVR due to collapsed fetal lung = greater pressures in the
right side. Free-flowing placenta circulation & ductus arteriosus = Low SVR
 Normal Heart Pressure – left side of heart is greater than pulmonary
vascular resistance
 Birth: umbilical cord clamp  lungs expand  postnatal circulation
 Cardiac Assessment:
o Pediatric Indicators of Cardiac Dysfunction –
 Poor Feeding, FTT
 Developmental Delays – d/t activity intolerance
 Difficulty with Air Exchange & Tissue Oxygenation
 Tachypnea/Tachycardia
o History – maternal/prenatal, neonatal, birth, and family histories
o Physical Exam
 General Appearance: Skin, Respirations, Chest Deformities
 Vital Signs: Pulse & BP
 Heart Auscultation: Check for Murmurs!
 Palpate for Liver Enlargement
 Fluid Status
 Activity & Behavior
Final Exam Review Pt. 2


Test Q: Complication
of Cardiac
Catheterization?
 Aneurysms or
Bleeding
Test Q: What is a
reason to cancel a
cardiac
catheterization?
 Severe Diaper Rash
Test Q: What is the
priority for the nurse
when caring for a pt.
who is pot-op cardiac
catheterization?
 tissue perfusion;
NV check of 6 P’s
13
Testing Cardiac Function –
 Pulse Oximetry
 Chest X-Ray: certain defects seen (Tetralogy of Fallot, AV canal,
Transposition); can be diagnostic
 EKG: if strips inconsistent – order 12-lead; can detect cardiomegaly
 Echo: detects cardiac dysfunction; ultra-high-frequency waves shows
heart structure
Cardiac Catheterization – effective but invasive; high risk
o Invasive procedure where a catheter is inserted through a peripheral blood vessel
(femoral vein) into the heart. Usually combined with angiography (contrast material
injected into circulation). Must obtain allergies before performing with dye.
 Gives oxygen saturation and pressure changes of blood within the chambers
and great vessels, cardiac output or stroke volume, & anatomic abnormalities
o Purpose:
 Palliation (Balloon Atrial Septostomy), Valvular Balloon Dilation, Diagnosis, and
for Pressures & Oxygenation
o Complications:
 Aneurysms or bleeding d/t invasive procedure through great arteries and veins
o Preprocedural Care:
Test Q: What
 Assessment: ASSESS PEDAL PULSES – assess and mark locations, clearly
should the
document; Accurate height & weight
nurse be sure of
before cardiac
 Severe Diaper Rash – cancel procedure if femoral access is required
catheterization?
 Child is usually NPO before procedure
 good pedal
 Polycythemia Infants & Children: IV Fluids prevent dehydration
pulses, child is
 Neonates: Dextrose Solution up to 2 hours to prevent hypoglycemia
NPO
o Postprocedural Care:
 Vital Signs, Neurovascular Checks (6 P’s), Assess Catheterization Site
 Apical Pulse 1 FULL MINUTE; Vital Signs q 15min. x 1hr  q 30min. x 1hr
 pulse distal to site may be week for first few hours, but
Quiz Q: Important
extremity should be warm
teaching for pt. with
 Bedrest, Leg Straight for 4-8 hours
cardiac cath.
 Restrict activity 24 hours
 bedrest, leg
 I&Os, Encourage Hydration
straight for 4-8
 Occlusive Waterproof Dressing
hours postprocedure;
replace
o Discharge Teaching:
pressure
dressing
 Remove Pressure Dressing Day After  Apply Band-Aid (change QID
day after and apply
for 2 days)
band-aid (replace
 Keep Site Clean & Dry
QID for 2 days)
 avoid bathtubs (3 days) to prevent clot dislodging
 Monitor for swelling, drainage, bleeding, leg coolness
 Monitor entry site for swelling, infection, decreased perfusion
 Acetaminophen or Ibuprofen
Final Exam Review Pt. 2
14
 Congenital Heart Disease (CHD)
o Major cause of death in 1st year of life, etiology unknown combination of:
 Maternal viral infections, increased maternal age, metabolic disorders,
chromosomal abnormalities, genetic factors, environmental factors, Rubella
 Results in blood shunting – often hear a murmur
Quiz Q: What would
o Indicators: may not appear immediately
you expect to see in a
 prenatal history, family history, poor feeding, failure to thrive,
child with CHD?
developmental delays r/t activity intolerance, tachypnea
 feeding difficulties
and/or tachycardia
o USE OF PULSE OX ON UPPER & LOWER EXTREMITIES
o Therapeutic Management:
 minimize dysfunction – evaluate CO determinates
Test Q: What is used as
 improve cardiac function
the primary tx. to reduce
 remove fluid & sodium – decrease preload (diuretics)
sodium and fluid in pt.
 Spironolactone or Lasix: mainstay of therapy to eliminate excess water
with CHD?
 Lasix or Spirolactone
and sodium
 reduce afterload: ACE Inhibitors (Captopril)
 decrease cardiac demands – cluster care, treat infections, reduce effort of
breathing (semi-fowler position), decrease oxygen consumption
 improve tissue oxygenation
Test Q: Why are CHD’s
classified no longer
identified as acyanotic vs.
cyanotic?
 some that are
classified as “acyanotic”
can have cyanosis as well
Final Exam Review Pt. 2
15
 Increased Pulmonary Blood Flow (Acyanotic)
o Patent Ductus Arteriosus, Atrial Septal Defect, Ventricular Septal Defect, AV Canal
 Blood flow to pulmonary vasculature increased, allows blood from high pressure
left heart  low pressure right heart
 left to right shunt
 CHF Symptoms: pink, difficulty with feedings, tachypnea, SOB, increased
workload of heart, tachycardia, frequent respiratory infections, murmurs
 Responsive to Oxygen; has enough RBCs
 Treatments: digoxin, ace inhibitors, fluid management, diuretics, oxygen
 untreated = irreversible pulmonary hypertension
o Patent Ductus Arteriosus (PDA): failure of ductus arteriosus to close within 1st weeks of
life (48 hours); may be caused by problems with fetal circulation (placenta issues)
 Connection between the pulmonary artery and aorta that has failed to close
 Clinical Manifestations:
 FTT, respiratory infection, CHF symptoms, common in preterm infants
Final Q: What CHD is
 Characteristic murmur (past 5-8 days of birth) with widened pulse
recognized by
pressures and bounding pulses
characteristic murmur
 Causes a left to right shunt causing blood to recirculate to the lungs (pulmonary
5-8 days past birth with
widened pulse pressure
artery over circulation)
and bounding pulses
 Treatment: (depends on presentation of the patient)
 Medically – Indomethacin (Indocin), a PgE Inhibitor
 Cath Lab – percutaneously with occlusion (coils)
 Surgically – Thoracotomy (1st choice – immediate)
o If not symptomatic, percutaneously occlude with contrast dye
o Atrial Septal Defect (ASD): failure of the foramen ovale to close resulting in persistent
fetal circulation, blood shunts from left atria to right atria
 not as much force pushing blood to right side of the heart
 Some are persistent fetal circulation, some are structural defects (hole
in filling chambers)
 May spontaneously close in 4 years of age
 Symptoms: depend on the size of the defect; (+) for murmur
 Treatment:
 Cath Lab – percutaneous patch (small ASDs)
 Surgically – patch/pericardium to close; bypass machine (large ASDs)
o Ventricular Septal Defect (VSD): failure of the septum between pumping chambers to
form; hole between pumping chambers present sooner d/t shunts being more increased
 Most common of heart defects, but frequently associated with other defects
 Prognosis: depends on location, number of defects, may close on its own in 1st
year of life
 Treatment: Surgical Closure (Percutaneous Transcatheter Device) – large VSDs
 small ones are followed up with by cardiologist
 Do NOT use – occlusive d/t clotting  stroke (left side) or PE (right side)
o Atrioventricular Canal Defect (AV Canal): incomplete fusion of the endocardial cushions
 low ASD (hole between filling)
 high VSD (hole between pumping)
 one common AV valve
 presents very early
Final Exam Review Pt. 2


16
Av Canal Defect: Most common cardiac defect in children with Down’s
Treatment: (surgical)
 Palliative – Pulmonary Artery Band: restricts pulmonary blood flow
o Buys time for child to grow – temporary repair
o too tight  cyanosis & hypoxic spells, too loose  HF
 Corrective – close hole between pumping and filling chambers creating
two new valves out of common AV valve
o Look for problems with conductive tissue from surgery or
valvular problems in patient postoperatively
 Decreased Pulmonary Blood Flow (Cyanotic)
o Tetralogy of Fallot, Tricuspid Atresia, Pulmonary Atresia
 BF to pulmonary vasculature decreased, results in cyanosis (blue spells); allows
deoxygenated blood to shunt right to left  blood into systemic circulation
without being oxygenated
Test Q: What manifestations
 NOT responsive to oxygen – not enough RBCs to lungs
are associated with
 Hypoxemia (low SaO2), Hypoxia, Cyanosis (deoxygenated Hgb)
decreased pulmonary blood
 Clinical Manifestations: O2 Sats = 50-90%, dusky, mottled/cyanotic, chronic
flow heart defects? Select all
hypoxemia and appears blue, fatigue, exertional dyspnea, failure to thrive,
 dusky, mottled, chronic
hypoxemia and appears
difficult eating and breathing at the same time, clubbing, delayed milestones,
blue, bradycardia,
poor weight gain, bradycardia, compensatory polycythemia (clotting
polycythemia
abnormalities)
 Hypercyanotic or Tet Spells
o Infants oxygen requirements exceed the blood supply – usually
Test Q: What should the
crying after feeding – can result in sudden death
nurse’s plan of care be for a
o
If crying = squat to counteract cyanosis
child experiencing a Tet
 Knee to Chest Position
Spell?
Final Q: What should the
 put child in knee-to-chest
 Palliation – Cardiac Shunts: increases blood flow to lungs
nurse monitor with a pt.
position
who is post-op BT Shunt?
 Prostaglandins & Cardiac Shunts
 preload, hydration,
o Blalock-Taussing Shunt
bleeding r/t Aspirin
 Atrial Septostomy
o Place child on prostaglandins first  hole made by cardiac
catheter between left & right atria – can diagnose at the same
time
o Post Op: check preload, hydration, bleeding r/t aspirin
o
Tetralogy
of
Fallot:
blue
babies
Final Q: Which of the

4
Characteristics:
following are seen in
Tetralogy of Fallot?
 VSD
 polycythemia, Tet
 Pulmonary Stenosis
spells, blue appearance
 Overriding Aorta
 Right Ventricular Hypertrophy
 Symptoms: Loud Murmur, Tet Spells  Sudden Death, Seizures; Clubbing, Heart
Appears “Boot-Shaped”
Test Q: Complication of
 Blue blood going to red side
Tetralogy of Fallot?

Complication:
Compensatory Polycythemia
 Compensatory
Polycythemia
 Treatment: can only correct VSD & Pulmonary Stenosis (redilate pulmonary a.)
 Complete repair during first year of life; closure of VSD and resection of
stenosis; patch placement to enlarge right ventricle outflow tract
Test Q: Which cardiac defect
is most commonly
associated with Down’s
Syndrome?
 AV Canal Defect
Final Exam Review Pt. 2
o
o
17
Tricuspid Atresia: tricuspid valve fails to develop  no communication of right atrium
and right ventricle = no blood getting to the lungs
 Ductal dependent lesion; present when fetal circulation develops
 S/S: presents when fetal circulation closes –
o ashy/dark, poor stats, tachypneic, tachycardia
 Lots of these patients do not have pulsatile flow = given Sildenafil (Viagra) to
lower pulmonary hypertension
 Immediate intervention @ birth until surgical intervention arranged 
 Balloon Septostomy
 Prostaglandins E1 – keeps ductus open until surgical intervention
 Surgical Treatment – STAGED REPAIR
 Palliative: Central Shunt
o SVC/IVC  pulmonary artery  blood returns to heart without
pulsation
 Pts. without pulsatile flow – give Sildenafil (Viagra)
 Bidirectional Glenn Shunt
o Cavo Pulmonary Anastomosis
 Modified Fontan Procedure
Pulmonary Atresia: pulmonary valves don’t exist
 Ductal Dependent; Easier to Repair  Early Symptoms
 Children with PA: make single ventricle  back to lungs without
pulsatile flow = ventricle works
 Extreme version of Pulmonary Stenosis, total fusion of commissures and no
blood flow to the lungs
 Obstructive Pulmonary Blood Flow (Acyanotic)
o Coarctation of Aorta, Aortic Stenosis, Pulmonary Stenosis
 Impedes Blood Flow Out of Ventricles
 Obstruction on Left Side = Heart Failure
 Obstruction on Right Side = Cyanosis
 Similar to Symptoms of Low CO –
o Diminished Pulses, Poor Capillary Refill & Color
o Decreased Urinary Output & Flow to GI Tract
 r/t kidney function decreased & intolerance with
feeding & absorption problems
o CHF with Pulmonary Edema
o Coarctation of the Aorta – narrowing of aorta by arch (ductus); tightness determines
severity, makes it hard for LV to pump = left-sided heart failure
 Presentation:
 Classic – younger children
o BP Discrepancies – higher upper than lower
Test Q: Disorder with
 Causes increased pressure proximal to narrowing,
increased blood
pressure in upper
decreased pressure distal to obstruction (upper vs. lower
extremities and
body pressures)
decreased BP in lower
o Pulse Discrepancies
Final Q: What heart
extremities?
 Coarctation of Aorta
defect is associated
with pulse
discrepancies?
 Coarctation of the
Aorta
Final Exam Review Pt. 2
18

o
o
Later – Older Children
o Hypertension UPPER extremities
o Dizziness, headache, fainting, epistaxis
o Decreased/Absent Pulses LOWER Extremities
o Rib Notching
 Treatment
 Cath Lab: Balloon Dilation [increase the diameter]
o Treatment of choice for OLDER infants and children
 Surgical: end-to-end anastomosis, subclavian flap, then patch graft
o Treatment of choice for infants younger than 6 months
o Post-op: check for bleeding, clotting, distal pulses, BP upper &
lower extremities
Aortic Stenosis – narrowing of aortic valve causing resistance to blood flow from the left
ventricle
 Left-Sided Heart Failure  HF
 Clinical Manifestations –
 S/S Decreased CO (faint pulses, hypotension, tachycardia, poor feeding)
 Exercise Intolerance
 Chest Pain
 Dizziness with Standing for Long Periods
 Characteristic Murmur
 At Risk For –
 Infective endocarditis, Coronary Insufficiency, Ventricular Dysfunction
 Treatment –
 Cath Lab: Balloon Dilation
o First-Line Procedure
 Aortic Valvotomy [Rare]
Pulmonary Stenosis – narrowing of the pulmonary valve  resistance
 Right-Sided Heart Failure
 Clinical Manifestations –
 May be Asymptomatic
 Cyanosis, Some Mild HF
o Newborns with severe narrowing = cyanotic
 Characteristic Murmur
 Cardiomegaly – apparent on chest radiograph
 At Risk For –
 Infective Endocarditis
 Treatment –
 Cath Lab: Balloon Angioplasty
o Need for surgery is rare d/t wide spread balloon techniques
o Associated with few complications, highly effective
o Treatment of Choice!
 Surgery = RARE
o depending on severity
o in some cases, cardiopulmonary bypass may be necessary
Final Exam Review Pt. 2
19
 Mixed Blood Flow (Cyanotic)
o Transposition of the Great Arteries/Vessels, Hypoplastic Left Heart Syndrome
 Complex cardiac anomalies that requires mixing of blood in postnatal period
 Combination Defects: more than one defect can compound problems
or cancel them out
 Desaturated blood mixed with saturated blood; no increase or decrease in BF,
defects balance them out
 Hypoxia & Heart Failure can occur together
 Pulmonary Congestion Occurs, Cardiac Output Decreases
 Clinical Manifestations –
 Variable Picture that Combines: Desaturation & Signs of CHF
o Cyanosis not always visible
 Often Requires Multiple Surgical Interventions
o Transposition of the Great Arteries/Vessels (TGA) – parallel blood flow; blood goes to
pulmonary artery/aorta then back to the heart through the same way
 Pulmonary Artery = outflow for left ventricle
 Aorta = outflow for right ventricle
 Most common defect is patent foramen ovale – life threatening at birth
Quiz Q: What does the
 Clinical Manifestations –
nurse know when caring
 cyanosis at birth that does not improve with oxygenation
for baby with TSA?
 CHF may develop at birth or a couple of days
 another heart defect
 “egg on its side” seen on CXR
must be present to
sustain life
 abnormal position of the great arteries seen on Echo
 Treatment –
 Arterial Switch – Prostaglandin E1 given after birth to maintain PDA
Test Q: Post-Op concern
until surgery performed
following Arterial Switch?
o Common Complication = arrhythmias; may need pacemaker
 EKG to check for
o Survival Without Surgery is IMPOSSIBLE!
ischemia (Coronary
Perfusion)
o Post-Op Concerns: Coronary Perfusion
 EKG for Ischemia (coronary perfusion) enzymes for
cardiac muscle death
o Hypoplastic Left Heart Syndrome (HLHS) – underdeveloped left side of heart, small left
ventricle and aorta
 Clinical Manifestations –
 cyanosis noted after birth
 single heart sound auscultated; no murmur present
 usually fatal after 1 month with no intervention
 Therapeutic Management –
 Prostaglandin E1 given to maintain PDA until surgery (allow mixing of
blood); AVOID SUPPLEMENTAL O2
 Treatment (4 options):
 Comfort & Palliative Care
 Heart Transplant
 Staged Procedure
 Hybrid Procedure
Final Exam Review Pt. 2
Test Q: What teaching
should the nurse
prepare for a pt. with
HLHS?
 Staged Norwood
Procedure
20
o
Staged Procedure:
 Stage 1 NORWOOD – left side is nonfunctional; make
one ventricle pump for the whole body, new aorta,
blood flow ONLY through shunts (newborn)
 At risk for clots d/t Aspirin
o monitor for Reye’s Syndrome
 Viagra for pulmonary vascular reduction
 Stage 1 HYBRID – do not need bypass machine; done in
Cath lab
 If candidate, put on PgE1  Cath lab  atrial
septostomy (stent in ductus arteriosus to keep
open)  brought in later for Norwood & begin
stages
 Stage 2 Bidirectional Glenn Shunt – SVC hooked to
pulmonary artery to create passive flow, not pulsatile
 Give lots of Viagra
 Stage 3 Modified Fontan – SVC & IVC both passively
flowing in the great arteries
 Post-Op Cardiac Patient:
o Monitor chest tube for bleeding and/or tamponade – record hourly drainage
o Catheter to Indicate CO; Continuous VS [heart pressures & changes]
 Intracardiac & Intraarterial Monitoring
o Intubated overnight; extubate next day
 Care After Heart Surgery:
o Monitor and Prevent Infection & Bleeding
o Respiratory Complications: pneumothorax, pleural effusion, muscle fatigue, atelectasis
Final Q: What is a
o Pain Management; Manage Fluids & Nutrition; Elevate HOB
common
complication post Feed pt. as soon as bowel sounds present
op heart surgery
o Neutral Thermal Environment
that the nurse
o Avoid Picking Up from Under the Arms
should be aware of?
o Prevent Thromboembolism – those at risk, administer aspirin, maintain hydration
 Cardiac
o Quiet Games/Activities
Tamponade
o Continuous Monitoring For: arrhythmias, cardiac tamponade, cardiac output/perfusion,
bacterial endocarditis, HF & pulmonary HTN, pharmacological support
 Pharmacological Support –
 Enhance CO:
o Furosemide (Lasix): Adverse Effects = Hypokalemia
 Spironolactone: decreases preload
 Adverse Effects = Hyperkalemia
Test Q: Select all
o Digoxin (Lanoxin) – increases contractility; decreases HR
the medications
 Apical Pulse 1 Minute  do not give if HR is low
that can be given to
 Dig Toxicity Monitoring – bradycardia, vomiting (early),
enhance CO.
nausea/anorexia, neurological symptoms, visual
disturbances
Final Q: What would
o Captopril/Enalapril – decrease afterload
the nurse expect to
 Prevent Pulmonary HTN: Sildenafil (Viagra)
give to decrease
afterload?
 Captopril
Final Exam Review Pt. 2
 Super Ventricular Tachycardia – dysrhythmia; can be lethal, unchanged high HR
o Starts with SA node; symptoms determine treatment
 Treatment (depending on condition) –
 Valsalva Maneuver: blow through straw or splash cold water on face
 Adenosine: time out [asystole], restart the heart; very rapid IV push
(short ½ life)
 Synchronize Cardio Version: low energy shock with peak of QRS
 ACQUIRED HEART DISEASES
o Bacterial Endocarditis, Infective Endocarditis, or Subacute Bacterial Endocarditis
 Infection of the inner lining of the heart (endocardium), involving the valves
 Causes –
 Streptococci, Staphylococci, Fungal Infections
 Pathophysiology –
 Organisms in bloodstream from any site of localized infection
o May occur from routine activities (brushing teeth), catheters
 Altered BF & Turbulence
 Damage to Valvular Endothelium – form vegetation on endocardium
 Lesions may infect adjacent tissues [valves and myocardium]
 May break off and embolize [spleen, kidney, CNS]
 Clinical Manifestations –
 Malaise, Low-Grade Fever
 New Murmur/Change in Previous Murmur
 S/S of HF may present
 Signs d/t Embolus Formation Elsewhere –
o Splenomegaly
o Osler Nodes [red, painful nodes on pads of fingers]
o Janeway’s Lesions [painless spots on palms & soles]
o Splinter Hemorrhages [think, black lines]
 Major Criteria –
 Blood Cultures
IE Diagnosed If:
 Echocardiographic Evidence
 2 Major Criteria

Minor
Criteria –
1 Major + 3
Minor Criteria
 Fever
 5 Minor Criteria
 Predisposing Risk Factors [central line, IV drug use]
 Vascular and Immunologic Findings
 Therapeutic Management –
 High-Dose IV Antibiotics [2-8 weeks]
o Evaluate Effectiveness with Repeat Blood Cultures
 Little to No Change = Surgical Approach [remove
vegetation, valve replacement]
 Prevention of IE –
 Prophylactic Antibiotics: ONLY for highest risk of CHD pts.
o Take before dental work, invasive respiratory treatment, or
procedures on soft tissue infections
 Meticulous Dental Hygiene [prevent infection]
21
Final Exam Review Pt. 2
o
22
Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) – acute systemic vasculitis
of unknown cause; self-limiting (6-8 weeks) – peak in toddlers, seasonal variations
 Damage to coronary arteries & heart muscle can occur – coronary dilation
Test Q: Complication
associated with
(ECTASIA), coronary artery aneurysms (ischemia, tamponade, clotting, MIs)
Kawasaki Disease?
 Acute – abrupt high fever unresponsive to antibiotics & antipyretics –
 Coronary Artery
hospitalized
Aneurysm
o Conjunctivitis without drainage, strawberry tongue, edema in
hands and feet, extreme irritability, periungual desquamation,
arthritis, non-blistering rash, bilateral joint pain, enlarged
Test Q: Pt. presents with
lymph, cervical lymphadenopathy, decreased left vent function,
strawberry tongue, high fever,
pericardial effusion, mitral regurgitation
and is inconsolable. What

Subacute
– resolution of fever until all outward clinical signs disappear
would the nurse assume?
 Kawasaki Disease
o Still irritable, peeling of skin nails/palms, temporary arthritis
 Convalescent – no manifestations except on labs
Test Q: What
o Erythrocyte & CRP Increased; back to normal 6-8 weeks
cardiac
 Cardiac Involvement of KWD –
involvement is
seen in Kawasaki
 particularly the coronaries
Disease?
 Serious Complication – POTENTIAL MI
 potential for
Final Q: What is the
 Treatment –
MI; particularly in
appropriate treatment
 IV Immune Globulin (IVIG): stop autoimmune from occurring
the coronaries
for Kawasaki Disease?

Aspirin:
prevents
clotting,
sent
home
 IV Immune Globulin
 Nursing Care –
(IVIG) & Aspirin
 Early Diagnosis & Treatment, Prevention of CV Complications
 Monitor I&Os
 Symptomatic Relief, Address Symptoms & Pt. Irritability
 Discharge Teaching: continue aspirin through course of subacute phase
 HEMATOLOGICAL DISORDERS
o Sickle Cell Anemia
 Autosomal Recessive hereditary Hemoglobinopathy
 Ethnicity/Malaria: 1 in 12 African Americans are Carriers
 Diagnosis –
 cord blood in newborns tested
 genetic testing
 sickle turbidity test for children older than 6 months
 Assessment of Hematologic Function –
 CBC (normal life = 120 days; sickle cell hemolysis = 40 days)
 History & Assess. [fatigue, SOB on exertion, joint pain, neonatal screen]
 Energy & Activity Level
 Growth Patterns
o Sickle Cell Disease
 Autosomal Recessive Disorder, Chronic Disease, primarily African American
 Causes of Crisis –
 Decreased Oxygen Tension  Hgb S to Sickle
o Hypoxia Occurs and Causes Sickling
o Infection, Fever, Trauma
o Dehydration, Acidosis
o Hypoxia
Final Exam Review Pt. 2
23
 High Temperatures: exposure to cold
 High Altitudes
Physical/Emotional Stress (puberty)
o
Sickle Cell Crisis
 Vaso-Occlusive Crisis: painful episode/pain crisis; sickled Hgb increases risk for
CVA and vital organ infarction
 Monitor for good perfusion; large tissue infarctions occur
 Increased RBC destruction > RBC production
 Sequestration Crisis: splenic sequestration – sickled cells become fragile an
easily hemolyzed in the spleen  pooling of blood & infarction of splenic
vessels
 Clinical Signs: profound anemia, hypovolemia, shock
 May require splenectomy @ early age; results in decreased immunity
 Chest Syndrome: overwhelming pneumonia r/t inability of spleen to filter
 s/s – chest pain, fever, cough, dyspnea
 Overwhelming Infarction
 Cerebrovascular Accident – transcranial doppler annually 2-16 years old
Test Q: which of the
o Nursing & Care Management = Supportive
following should
 Hydration & Electrolyte Replacement
the nurse question
 Pain Management  DO NOT GIVE DEMEROL (Morphine, Dilaudid ok)
before
administering to a
 Promote Tissue Oxygenation, Prevent Infection (PCN)
pt. with sickle cell?
 Prevent Sickling Episodes, Early ID of Crisis
 Demerol
 Careful Monitoring of SHOCK
 Education:
 splenomegaly, dehydration, no contact sports, avoid heat & low O2
o Prognosis:
 no cure (possible bone marrow transplants), frequent bacterial infections r/t
immunocompromised
 bacterial infection = leading cause of death
 DEFECTS IN HEMOSTASIS
o Hemophilia: group of hereditary bleeding disorders that result from deficiencies of
specific clotting factors; x-linked disorder
 Diagnosis –
 amniocentesis, genetic testing for carriers
 Clinical Manifestations –
 bleeding tendencies (mild  severe), hemarthrosis, ecchymosis,
internal bleeding, hematuria, epistaxis, bleeding post procedures (minor
trauma, tooth extraction, minor surgeries, hemorrhages, bleeding
compromising airway)
 Hemophilia A  most common, factor VIII (8) deficiency
 Hemophilia B  deficiency of factor IX (9)
 Mild: bleeding with severe trauma or surgery
 Moderate: bleeding with trauma
 Severe: spontaneous bleeding without trauma
 Clinical Therapy: prevent bleeding, amniocentesis diagnosis, genetic testing,
labs, history, and exam diagnosis
 Treatment: recognize & control bleeding
o
Final Exam Review Pt. 2
24


Test Q: What should
nurse implement for
hemarthrosis?
 RICE


Institute factor replacement therapy
DDAVP (IV) – Desmopressin: help production of components that help
with clotting (mild)
 Prevent crippling effects of bleeding
 RICE for Hemarthrosis
Prognosis: near normal lives; gene therapy for future
Education:
 First aid for small cuts/abrasions
o Clean Cut = apply pressure & band-aid
o Severe = give factor VIII
 Promote Independence: regular school
o avoid contact sports; may swim or golf
 Home Care: venipuncture & factor VIII admin
 If complaining of joint pain, send immediately to school nurse
Final Exam Review Pt. 2
25
GI DYSFUNCTION/TRANSFER OF NUTRIENTS
8 questions
 Review
o Nutrition –
 Healthy Newborn: 108 kcal/kg/day
 Infant Nutritional Care –
o Solid Foods  6 months; introduced 1 at a time, every 4-7 days
o Cow’s Milk  1 years old d/t deficient source of Vitamin C, iron,
or zinc
o 2% Whole Milk  NOT UNTIL 2 YEARS!
 Limiting milk sources can decrease risk of anemia
 Toddlers: 102 kcal/kg/day; Preschoolers: 90 kcal/kg/day
 Toddler & Preschool Nutritional Concerns –
o Physiologic Anorexia (12-18 months): growth slows down,
finicky eaters
 Reassure parents this is normal, give healthy snacks
child enjoys
o Non-Nutritive Function: pleasure/comfort food, social activity
 Discourage food becoming a reward
o Parental Guidance: calm eating environment, stress free & fun,
varieties of choices, cut food into small pieces
 School Age: 1200-1800 kcal/day
Test Q: When
 Family eating habits remain large influence; peer influences @ lunch
teaching a parent
 Increased independence in food choice – remind stop eating when full
about their child’s
nutrition, what
 Midafternoon snacks = common; healthy (no midmorning snack)
would the nurse
o MyPlate: shows parents a good way to form a child’s plate
suggest?

Insufficient
Dairy Intake; Vitamin D Deficiency
MyPlate
 Adolescents: 1800-2200 kcal/day
 Females need 2200 kcal/day to support growth spurt; males need 25002900 kcal/day – these decrease after growth spurt
Test Q: What is
o 4 servings of dairy for growth spurt
important
Test Q: While
nutritional
 Preparing own food & eating with friends – still need adult guidance
teaching an obesity
education for
 Vitamin D deficient
prevention class,
parents of an
which is NOT correct? o Exercise –
adolescent?
 20 min of exercise
 All well visits include nutritional/exercise screen, desirable BMI 5th – 84th %
 still needs
daily is sufficient for
adult guidance
 Healthy Heart Activity Levels =
prevention
 1 hour/day moderate to vigorous activity
 Vigorous activity 3 days/week
Test Q: Eating
 PICA: eating disorder of compulsive & excessive ingestion of food & nonfood substances at
disorder that
involves
least once a month
eating
 Starvation –
nonfood
o Severe Acute Malnutrition: caused from poor feeding & sanitary conditions
substances
 3rd world countries
 PICA
o Kwashiorkor: deficient protein with adequate calories; diet of starch & potatoes
 Thin, wasted extremities with protuberant abdomen
 general edema masks atrophy
Final Exam Review Pt. 2
26
o
Test Q: Test
to diagnose
diabetes?
 A1C
Marasmus: general malnutrition of calories & protein; decreased subcutaneous tissue
 Extreme thinness
 Obesity –
Final Q: Parent are concerned
about dark lines on child’s neck.
o Hypertension: BP screening @ 3 years of age
What should nurse respond?
o Type II Diabetes: Acanthosis Nigricans (dark line indicators)
 Acanthosis Nigricans d/t
o Hyperlipidemia: increased lipid levels
increased levels of insulin which
th
th
o BMI > 85 to 95 = overweight
could possibly indicate T2DM
 above 95th = obese
 Obesity Complications –
o Type II Diabetes, Syndrome X, Fatty Liver, Pulmonary Dysfunction, Growth Acceleration
o Depression, Low Self-Esteem, Bullying
 Obesity Diagnostics/Treatment –
o Labs: lipid panel, A1C, liver enzymes, sleep studies
o PREVENTION IS KEY; behavior modifications, family interventions, surgical interventions
(18+), exercise, diet, etc.
 Must be ready to make a change
 DISORDERS OF MOTILITY
o Hirschsprung Disease (Congenital Aganglionic Megacolon): congenital abnormality from
inadequate motility causing mechanical obstruction; absence of ganglion cells; no
Test Q: Matching GI
evacuation of stool [matching test question]
Disorder. Absence of
 Symptoms –
ganglion cells and the
 Newborn: failure to pass meconium, vomits bile, refusal to eat,
failure to pass meconium in
abdominal distention
newborn.
 Hirschsprung Disease
 Infant: FTT, constipation, vomiting, diarrhea
 Child: undernourished, anemic, abdominal distention, visible peristalsis,
palpable fecal mass, constipation, foul-smelling ribbon like stool
Final Q: Absence of ganglion
 Diagnosis –
cells, failure to pass
Test Q: What would
 Rectal Biopsy: shows no ganglion cells
meconium, foul-smelling,
the nurse prepare
ribbon-like stools.
 Diet –
for a pt. pre-op
Hirschsprung
Hirschberg surgery?
 High Calorie, High Protein, Low Fiber
Barium Enema
 Treatment –
 Surgical removal of aganglionic portion, possible colostomy
Test Q: How is Hirschsprung
o Pre-Procedure: assess for adequate nutrition, bowel emptying,
Disease diagnosed?
 rectal biopsy shows no
and abdominal distention
ganglion cells
o Post Procedure: educate parents on enterocolitis,
Final Q: What should
fecal incontinence & obstruction, ostomy care
child with Hirschsprung
be prepared for?
 Complications –
possible colostomy
 Enterocolitis: inflammation of the digestive tract d/t
infection
o Antibiotics before surgery to decrease bacterial flora
Final Exam Review Pt. 2
27
o
Gastroesophageal Reflux (GER): transfer of gastric contents into esophagus; occurs
more after meals and at night
 Risk Factors –
 prematurity, BPD, neurological impairments, asthma, CF, CP, scoliosis
Test Q: What should the
 usually resolves by 1 year of age
nurse tell the mother of a
 GER becomes a disease when complications such as FTT, resp.
4-month-old with GER?
 may resolve by 1 year
problems, or dysphagia develop  GERD
of age
 Symptoms –
 Infants:
o sitting up/forceful vomiting, irritability, excessive crying, blood
in vomit, arching of back, FTT, apnea
 Children:
o heartburn, abdominal pain, difficulty swallowing, chronic cough,
noncardiac chest pain
 Diagnosis –
 24 upper pH probe study: gold standard
 upper GI endoscopy: can exclude other disorders such as Chron’s
 Care –
Final Q: Which of the following
 small, frequent meals; thicken infant formula with rice cereal
shows understanding of the
o constant NG feedings may be needed with severe reflux and FTT
teaching for a child with GER?
 until surgery can be performed
 “I will thicken my infant’s
formula with rice cereal”
 avoid caffeine, citrus, peppermint, spicy/fried foods
 assist in weight control
 elevate HOB after meals
o no prone position
o
Quiz Q: Child
with GERD is at
risk for?
Aspiration
Gastroesophageal Reflux Disease (GERD):
Test Q: What is the
 symptoms/tissue damage from GER
disease
process that
 Causes –
results from tissue
o FTT, respiratory issues, or dysphagia
damage d/t GER?
o At Risk For: Aspiration
 GERD
 Nursing Interventions –
o same as GER
o + proton pump inhibitor (omeprazole or pantoprazole)
o H2 receptor (ranitidine, cimetidine, famotidine)
 Treatment –
o Nissen Fundoplication: takes gastric fundus & wraps around
esophagus
 Post-Op: gastric decompression by an NG tube or
gastrostomy to avoid distention in immediate post-op
period
 Should not be replaced by the nurse if
Test Q: What procedure
accidentally removed because of risk of injury
would the nurse prepare
to the operative site
to educate a patient
with GERD on?
 Nissen
Fundoplication
Final Exam Review Pt. 2
28
 INFLAMMATORY CONDITIONS
o Acute Appendicitis:
 Inflammation of the vermiform appendix; most common emergency in children
 Clinical Manifestations –
 Periumbilical Pain  RLQ; Rebound Tenderness
o McBurney’s Point: most common point of tenderness
 N/V, Fever, Diarrhea or Constipation
Test Q: Child with
 Tachycardia, Lethargy, Poor Feeding
abdominal pain, fever, and
 Decreased/Absent Bowel Sounds
vomiting. What would
treatment would the nurse
 Diagnosis –
prepare for?
 CT Scan, Ultrasound
Antibiotics, IV Fluids, NG
 CBC, UA, CRP (WBC >10,000/mm3)
Suction

Management
–
Final Q: What would the
 Appendectomy (before rupture/perforation)
nurse prepare for when a
child has appendicitis and has
 Antibiotics, IV Fluids, NG  suction (ruptured)
not yet ruptured?
 Complications –
 Appendectomy
 perforation, peritonitis, abscess, phlegmon, fistula, partial bowel
obstruction
o Meckel’s Diverticulum:
 Failure of the omphalomesenteric duct to fuse during embryonic development
 when placenta replaces yolk sac  fistula, umbilical cyst, and Meckel’s
diverticulum
 Symptoms –
 painless rectal bleeding
 abdominal pain, distention, n/v
 bloody mucus stools
 causes: fistula, umbilical cysts
 Rule of 2’s
 Diagnosis –
 Radionucleotide Scan (Meckel’s scan)
 OBSTRUCTIVE DISORDERS
o Hypertrophic Pyloric Stenosis:
 Circular muscle of pylorus thickens so pyloric canal narrows; lumen becomes
Test Q: A child is irritable, hungry,
partially obstructed  worsening hypertrophy
and vomits 30 minutes after each
 Clinical Manifestations –
feeding. An olive-shaped mass is
o Vomiting occurs 30-60 min. after feeding
seen in abdomen.
o Irritable, hungry, dehydrated, decreased weight, FTT
 Hypertrophic Pyloric Stenosis
o Olive-Shaped Mass – upper abdomen
o Gastric peristalsis visible
Final Q: What can be seen
 Management –
in Hypertrophic Pyloric
o Pyloromyotomy (good prognosis)
Stenosis?
 Pre-Op: Correct Metabolic Alkalosis with IV Fluids &
 Olive-Shaped Mass
Electrolytes, NPO
 Post-Op: may need NG tube if vomiting continues; 4-6
hours start feeding, formula after 24 hours; small,
frequent feeds
Test Q: What should be pre-op
procedure for pt. with
Hypertrophic Pyloric Stenosis?
 NPO, IV Fluids, Prep for
Surgery
Final Exam Review Pt. 2
29
o
Intussusception:
 Proximal segment of bowel telescopes into distal segment, pulling mesentery;
mesentery = angled & compressed  lymphatic/venous obstruction
 Ischemia occurs  mucus pours into intestine  JELLY STOOLS
 Clinical Manifestations –
o Sudden, acute abdominal pain
 screaming, drawing knees to chest
Final Q: A child with
intermittent severe abdominal
o Comfortable between episodes
pain is screaming, drawing
o Vomiting
knees to chest; and
o Red, currant JELLY LIKE STOOLS
comfortable between episodes.
o Sausage Shaped Mass in URQ
He has red, currant, jelly-like
o Most common in ileocecal valve (3-6 months of life)
stools.
 Intussusception
 Treatment –
o Hydrostatic Enema (gas enema)
o IV Fluids, NG Decompression, Antibiotic Therapy
o Surgery if Needed (possible bowel resection)
 MALABSORPTION SYNDROMES
o Celiac Disease (Gluten-Sensitive Enteropathy):
 autoimmune disorder triggered by ingestion of glutens; damages villi in small
intestine = malabsorption
 Food Intolerances: protein present in wheat, barley, and rye
Test Q: What foods would a
o Beans, pasta, beer, etc.
pt. with celiac Disease be
 usually appears around 1-5 years of age
able to eat: waffles, bacon,
Test Q: Education for
 Clinical Manifestations –
toast, low-fat milk, bananas?
adolescent with
 bacon, low-fat milk,
celiac disease:
 intestinal symptoms, FTT, chronic diarrhea
bananas

gluten in beer
 aphthous ulcers canker sores
 abdominal distention & pain
 fatigue, muscle wasting
 untreated = lactose intolerance
o
Short-Bowel Syndrome:
 Decreased mucosal surface area d/t small intestine resection
 decreased area for absorption of fluids, electrolytes, & nutrients
o NEED FOR TPN NUTRITION! (glucose, sucrose, etc. needed)
 Initial Phase: PN as primary source of nutrition
Test Q: What nutritional
support does a patient with
 2nd Phase: introduce eternal feeing after surgery
short bowel syndrome need?
 Formulas containing glucose, sucrose, proteins,
 TPN as primary source,
and triglycerides facilitate absorption (NG/G
introduce eternal feeding
Tube)
after surgery
Test Q: What is needed
 As enteral feeds are advanced, PN solutions is
with TPN in post-op pts.
decreased in terms of calories, amount of fluid,
with SBS?
and totally hours of infusion per day.
 glucose, proteins,
 If enteral feeds are tolerated, oral feedings
sucrose, triglycerides
should be attempted to preserve oral skills.
Final Exam Review Pt. 2



o
30
Causes –
 multiple atresia, gastroschisis, necrotizing volvulus, meconium
peritonitis, chron disease, trauma
Management –
 preserve length of bowel as much as possible
 maintain optimum nutrition, growth, and development
 stimulate intestinal adaption with enteral feeding (TPN)
 minimize complications
Complications –
 most deaths r/t sepsis d/t poor care of central line
Necrotizing Enterocolitis (NEC):
 Acute inflammatory disease of the bowel where mucosal wall is damaged and
gas-forming bacteria invade; most common in preterm
 Potential Cause: decreased BF to intestines
Test Question: Matching GI
Disorders. Inflammatory disease
 Pneumatosis: air in the submucosal & subserosal surfaces of the bowel
of bowel, mucosal wall damaged,
 Clinical Manifestations –
bacteria invasion of damaged
o Distended Abdomen, Blood in Stools
areas.
o Gastric Residuals: check NG/G tube every feed (increased – red
 NEC
flag)
o Poor Feeding, Lethargy, Hypotension, Apnea, Vomiting Bile,
Test Q: Major risk factor
Decreased Urine Output, Hypothermia
for NEC?
 Diagnosis –
 Prematurity
o Sausage-Shaped Dilated Intestine, “soap suds” or bubbly
o Free air in abdomen  perforation n
o Labs: anemia, leukopenia, leukocytosis, metabolic acidosis,
Test Q: Disorder with free
electrolyte imbalance
air in the abdomen with
 Management –
“soapsuds” seen in scan.
o Hold Feeds for 24-48 hours if Asphyxiated at Birth
 NEC
o Breast Milk Preferred (enteral feeds) – IgA passive immunity
o Minimal Feeds – Trophic in VLBW (wake up gut slowly)
o Probiotics: in 1st 7 days; 14 days in preterm/VLBW infants
o Confirmed NEC:
 Discontinue all oral feeds
 IV Antibiotics
 NGT – decompression
 TPN – nutrition
o Sequelae in surviving infants include short bowel syndrome 2/2
intestinal dysfunction
o Nursing Management:
 Key Factor = Prompt Recognition
Test Q: What is associated
 Monitor VS for s/s of Bowel Perforation, Septicemia, or
with NEC?
CV Shock
 Short Bowel Syndrome
 Prevent Possible Transmissions
 Strict handwashing, isolation of confirmed cases
 Leave infant UNDIAPERED and in SUPINE or ON SIDE
Final Exam Review Pt. 2
31
 Oral feedings reinstituted ~7-10 days after treatment
Biliary Atresia:
 Inflammatory process that causes intrahepatic and extrahepatic bile duct
 fibrosis  ductal obstruction
Test Q: Matching GI Disorders.
Inflammatory process that
o occurs late in gestation, manifests a few weeks after birth
causes intrahepatic and
 Clinical Manifestations –
extrahepatic bile duct fibrosis
 Complete obliteration of extrahepatic biliary tree & ducts
leading to ductal obstruction.
 Jaundice >2 Weeks of Birth, Hepatomegaly
 Biliary Atresia
 Dark Urine
Test Q: How is Biliary
Atresia confirmed for
 Gray, Alcoholic Stools
diagnosis?
Test Q: Biliary
 Increased Serum Bilirubin
 Liver Biopsy
Atresia
 Diagnosis –
manifestations?
 Liver Biopsy = Best
Select all.
 Jaundice,
 H&P, Lab Findings
Alcoholic Stools,
o CBC, Bilirubin, Liver Function Tests
Increased Bili
o Alpha-antitrypsin, TORCH titers, other intrauterine infections
 Management of BA –
 Aggressive Nutritional Support – Enteral or TPN Feedings (Low in Na)
Test Q: What would the nurse
educate the parents on if their
 Supplement Fat Soluble Vitamins (A, D, E, K)
child has Biliary Atresia?
 Phenobarbital: stimulate bile flow
 Supplementation of Fat Ursodeoxycholic Acid (Ursodiol): decrease cholestasis, itching, jaundice
Soluble Vitamins (A, D, E, K)
 Surgery: Hepatic Portoenterostomy (KASAI Procedure)
o Prognosis: death by 3 years old if untreated; 20% survival with
KASAI Procedure; liver transplant
o
o
Esophageal Atresia & Tracheoesophageal Fistula:
 Esophagus fails to develop as continuous passage; trachea and esophagus fail to
separate (rare)
 Cardiac Anomalies Occur
 Clinical Manifestations –
o Frothy, Saliva Nose & Mouth, Drooling, Choking, Coughing
o Cyanotic, Apneic, Respiratory Distress
 Aspiration of breastmilk or saliva
o Stomach Distended by Air, Gastric Contents Regurgitated
 Broad-Spectrum Antibiotics if there is concern for
aspiration of gastric contents
Test Q: What is
o Polyhydramnios: amniotic fluid swallowed by fetus is unable to
polyhydramnios seen in?
reach GI tract to be absorbed and excreted by kidneys
 EA & TEF
 Result = Abnormal Accumulation of Amniotic Fluid, or
Polyhydramnios
 Treatment –
o Maintain Airway, Prevent Pneumonia
Test Q: What is the
o Gastric or Blind Pouch Decompression
infant with EA & TEF
o Supportive Therapy
at risk for? Select all.
 When suspected, discontinue all oral intake, initiate IV
 Tracheomalacia
 Aspiration
fluids, position to facilitate drainage r/t aspiration of
 TPN Feeding
Final Exam Review Pt. 2
32
secretions. Double lumen catheter on intermittent or
continuous low suction.
 Give Abx. if concerned for gastric content aspiration
o Surgical Repair
 Depends on early diagnosis before complications occur
and the presence of severity of associated anomalies
and illness factors, including PTB.
 Pre-Op:
 Carefully suction, position for drainage
 Blind pouch kept empty by suction
 G-Tube and Irrigations with Fluids
CONTRAINDICATED before surgery in infant
with distal TEF
 Post-Op:
 Thermoregulation, Tracheal Suctioning
 Catheter on Low-Suction or Gravity Drainage
Test Q: What nutritional
 TPN Provided
support will an infant
o Gastrostomy Tube  Gravity until
need post-op TEF
feedings are tolerated
repair?
 TPN
 Pain Management
 Observe Initial Oral Feedings
o Sterile Water  Frequent, Small
Feeding of Breastmilk/Formula
o May need to supplement by bolus or
continuous feeds
o Discharge when tolerated
 Complications –
o Tracheomalacia: weakening of tracheal wall r/t dilated proximal
pouch compressing the trachea in early fetal life
 S/S: barking cough, stridor, wheezing, recurrent
respiratory infections, cyanosis, apnea
 Nursing Care Management –
o Major Concern: Patent Airway, Prevention of Respiratory
Compromise
o OG feeding Tube
 ABDOMINAL WALL DEFECTS
o Gastroschisis:
 Bowel herniates through defect in abdominal wall on right side of umbilicus;
intestines outside of body in utero, peritoneal sac NOT present
Test Q: Match Gastroschisis and
 Complications –
Omphalocele.
o simple or complicated; bowel atresia, perforation, ischemia,
Herniation is to the right of the
necrosis
umbilicus; peritoneal sac is not
 Management –
present.
 Cover exposed bowel with bag/transparent gauze (SILO BAG): reduced
 Gastroschisis
Abdominal contents herniate
over days to weeks depending on damage
through umbilical ring, with intact

IV Fluids & IV Antibiotics
Test Q: What is the nurse’s priority
peritoneal sac.
 Double Lumen NG Tube: Decompression
when a neonate is born with
 Omphalocele
gastroschisis?
 cover exposed bowel with
transparent or moist sterile gauze
(silo bag)
Final Exam Review Pt. 2
33



Test Q: Nutritional education for
post-op gastroschisis repair?
 prolonged TPN until normal
bowel function, could be days to
weeks
o
Test Q: Matching GI
Disorders. Failure of lateral
folding of abdominal wall;
covering liver, bowel, and
intestines
Test Q: GI Matching. Hernia o
that cannot be reduced
manually, fusion of umbilical
cord incomplete.
Umbilical Hernia
o
2-3x Maintenance Fluids: Replacement
THERMOREGULATION
Simple = Surgery Immediately
o Post-Op:
 Mechanical Ventilation (abdominal pressure)
 Pain Management: Morphine, Fentanyl
 Prolonged TPN
 Days  Weeks for Normal Bowel Function
Omphalocele:
 True failure of embryonic development; failure of lateral folding of abdominal
wall @ 3rd week gestation; covered by translucent sac
 Includes bowel, maybe liver
o associated with other anomalies
Test Q: What should the
nurse prepare for with a
 Management of Omphalocele –
pt.
post-op omphalocele
 Nonadherent Dressing Over Defect
repair surgery?
 IV Fluids & Antibiotics
 TPN, Mechanical
 Double lumen NG/OG: decompression
Ventilation
 Sac Resected/Reduced
o Post-Op:
 May Need Mechanical Ventilation & TPN
 Feedings Resumed: once bowel function resumes
Umbilical Hernias:
 Common, affects premature babies and low birth weight newborns
 Rarely a constricted hernia that cannot be reduced manually; occurs when
fusion of the umbilical ring is incomplete at the point where the umbilical
vessels exit the abdominal wall
 Affects premies and LBW newborns, usually resolves by 3-5 years
 Elective Surgery if not resolved
 Nursing Management –
 Appearance may be disconcerting to parents, reassure that the defect is
usually not harmful
 Taping or strapping the abdomen to flatten the protrusion does NOT aid
in resolution and can produce skin irritation; educate parents
 Observe for Hematoma or Infection
 Child may resume normal diet and activity post-op
o Strenuous Activity Restricted 2-3 Weeks
Inguinal Hernia:
 Peritoneum precedes testicles into scrotum in boys; round ligament into labia in
girls (80% of Childhood Hernias – Boys 6:1)
 Usually asymptomatic unless the abdominal contents are forced into the patent
sac, most often associated with painless inguinal swelling
 If herniated loop becomes obstructed, symptoms may include: irritability,
tenderness, anorexia, abdominal distention, and difficulty defecating
 Emergency Surgery for Incarcerated (constricted); elective otherwise
o Post-Op: keep incision dry and clean, remove wet diapers ASAP
Final Exam Review Pt. 2
34

o
No restrictions placed on infants or toddlers, but older
children should be advised against lifting, pushing,
wrestling, bike riding, and sporting events for 2-3
weeks.
If surgery is postponed, parents should be educated on s/s of
incarcerated hernia, simple measures to reduce it (warm bath,
avoid sitting in upright position, comfort measures to reduce
crying) and when to call for assistance.
 Cleft Lip & Cleft Palate:
o Incomplete fusion of the oral cavity or palates in utero d/t genetic or environmental
factors (alcohol, drugs, smoking, anticonvulsants, steroids, retinoids)
 Folic Acid = PREVENTION
 Cleft Lip: failure of the maxillary process to fuse with nasal elevations which
occurs in the 6th week of gestation
 correction at 2-3 months old; must be free from infection
 Cleft Palate: midline defect that may vary in severity, from the soft palate to the
Test Q: Why is Cleft Palate
hard palate. Occurs from failed fusion of secondary plate, which takes place
corrected at 6-12 months of age?
between 7-12 weeks of gestation.
 early repair restricts skeletal
growth and delayed repair
 correct from 6-12 months old
results in speech disorders
o early repair restricts skeletal growth and delayed repair
increases speech disorders
 Post-Op:
Test Q: What is the main
 Monitor Integrity (Infection) – Protect Operative Site
focus of post-op care of
Test Q: What would the
o Elbow Restraints
cleft palate repair?
nurse identify as a need
 Position Upright/On Back
 Protect Operative Site
for further education
 Clean Incision: NS, water, or diluted hydrogen peroxide
following surgical repair
of CP?
 IV Fluids & Clear Liquids 24 Hours
 “my child must use a
 No Straw, Tongue Depressor, or Sippy Cups
straw to drink liquids”
Test Q: Pain management
 Observe for Airway Obstruction & Hemorrhage
for pt. in immediate post-op
o Observe Vitals & O2
following CP repair?

Pain
Management: Opiates (1st 24 hours)
 Opiates 1st 24 hours
o Acetaminophen
 Antibiotic Ointments: 3 Days on Suture Line
 Management –
 additional surgeries, ortho, speech therapy, audiology, social work, etc.
o Encourage Breastfeeding
 Position Upright, Burp Frequently, Special Bottle (oneway valve, special nipple), Syringe Feed if Necessary
o Weekly Weight Checks
o Require Cheek Support
 Squeeze checks together
o NOISY FEEDERS; Assure parents this is normal
 Indication the infant needs to stop feeding for a brief
moment: facial signal, elevated eyebrow, wrinkled
forehead, watery eyes
Final Exam Review Pt. 2
 Feedings –
o NG/OG Tubes: through nose or mouth, left in place or removed after feed
 Gavage, Continuous, or Medication Administration
 Check Placement  X-Ray then pH Test (<5.5)
o NGT Tubes: placed through nose or mouth to the jejunum/duodenum
 High Risk Aspiration or Regurgitation
 Must X-Ray Before Use
 ONLY Continuous Feeds
o Gastrostomy Tubes: if tube unable to pass mouth, pharynx, esophagus, or cardiac
sphincter; long term tube feeding through abdominal wall to stomach
 General/Local Anesthesia
 Clean with Soap & Water Daily
 No Check for Placement
o Parenteral Nutrition (TPN): provides total nutritional needs; filtered to remove
particulate matter
 IV Infusion of Highly Concentrated Protein, Glucose, Nutrients
 Central Line if Dextrose >13%
Test Q: Considerations with
 Peripheral Line if Dextrose <13%
TPN?
 Give Dextrose >13% in
 Management –
Central Line, and Dextrose
 New Bag/Tubing q24 hours
<13% in Peripheral Line
 2 Nurses Sign Off
 High Risk for IV Infection
35
Test Q: What
should be assessed
in a pt. who is
receiving TPN?
 IV Site and
Glucose
Final Exam Review Pt. 2


Final
Exam
Question





36
ACES & MALTREATMENT
9 questions with Social & Culture
ACEs: Adverse Childhood Experiences caused by toxic stress (constant production of cortisol) and
has long term health effects into adulthood
o Persons who have experienced ACES had 4 to 12-fold increased health risks for
alcoholism, drug abuse, depression, and suicided. Increased risks for smoking, poor selfrelated health, increased sexual partners, STIs, physical inactivity, severe obesity.
o Women and Minorities = Highest Risk for ACEs
Categories –
o Abuse: emotional, physical, sexual
o Neglect: emotional, physical
o Household Challenges: mother treated violently, substance abuse, mental illness, etc.
o Other: peer victimization, dating violence, loss of parent, community violence, trauma,
war, poverty
Risk Factors –
o Family/Individual:
 low income, low education, high parenting/economic stress, inconsistence
discipline, negative communication styles, acceptance of violence
o Community:
 High rates of violence, high unemployment, high poverty, low community
involvement, food insecurity, unstable housing
Protective Factors –
o Family/Individual:
 families with safe/nurturing relationships, good performance in school, positive
peer relationships, positive adult role models outside of the family, basic needs
met, engaging in fun family activities,
o Community:
 safe/stable housing, good daycares & preschools, violence not tolerated,
community involvement
Preventing ACEs –
Test Q: True or False –
o Strengthen Economic Support to Families
promoting social norms to
o Promote Social Norms to Protect Against Violence/Adversity
protect against
o Ensure Strong Start for Children
violence/adversity is a factor of
o Teach Skills
preventing ACEs.
 True
o Connect Youth to Caring Adults/Activities
o Intervene to Lessen Immediate & Long-Term Harm
Raising Awareness of ACEs –
Final Q: What is
considered
o CHANGE How People Think About Causes and Preventions
maltreatment?
o SHIFT Focuses from Individual Responsibility to Community Solutions
 emotional,
o REDUCE Stigma Around Seeking Help
physical, and
o PROMOTE Safe and Nurturing Relationship & Environments
sexual abused
 emotional,
Maltreatment of Infants & Children –
physical
neglect
o Maltreatment = physical, sexual, emotional, physical neglect, emotional neglect
o Risk Factors –
 Caregiver: young/uneducated, unrelated partner of child, low income, social
isolation, low self-esteem, lack of parenting knowledge, history of abuse
Final Exam Review Pt. 2


37
Child: less than 1 years old, unwanted, hyperactive, disabilities, premature
Environment: chronic stress, substance abuse, divorce, unemployment,
substitute caregivers
o
Warning
Signs –
Test Q: Which
scenario would make
 physical evidence
Test Q: Which are indications
the nurse suspect
 vague/changing history
of parental anger?
maltreatment?
 other injuries

parents who are
 vague/changing
 delay in seeking care, inappropriate responses
unreactive to child’s injuries
story of injury
o Findings of Maltreatment –
 Physical Neglect: FTT, lack of hygiene, frequent injuries, delay in seeking
healthcare, dull affect, school absences, self-stimulating activities

Emotional Neglect + Abuse: FTT, eating disorder, enuresis, sleep disturbances,
Test Q: A child has come in
self-stimulating behaviors, withdrawal, developmental delay, suicide
for CHD. Which of the
Test Q: Which of the
attempts
following would indicate
following would be
child abuse?
 Physical Abuse: bruises in various stages of healing or nonmobile
most indicative of
 unexplained burns to the
child abuse?
child, multiple fractures in different stages, burns, lacerations, fear of
neck and back

Bruises
in various
parents, lack of emotional response
stages of healing
 Sexual Abuse: bruises, lacerations, bleeding of genitalia, anus, or
mouth; STDs, UTIs, difficulty walking/standing, regressive behavior, withdrawal,
sexually explicit language inappropriate for age or development, unusual body
odor, bloody/torn underwear
 Caregiver Behaviors with Emotional Neglect/Abuse –
o Rejection, Terrorizing Child
o Ignoring Child, Verbally Assaulting
o Pressuring Excessively
 Shaken Baby Syndrome – Infant Abuse
o Forcibly shaken from excessive crying; blow to head, fist or object
 May not be seen until severe
o Findings –
 Early Normal Exam
 Vomiting, Poor Feeing, Respiratory Distress, Retinal Hemorrhages, Seizures,
Posturing, Alterations in LOC, Apnea/Bradycardia, Blindness, Unresponsiveness
o Nursing Care –
 Full Assessment for Other Injuries, NOTIFY AUTHORITIES
 Remove Child from Care of Abuser
 Make Clear/Objective Notes About Physical Findings
 Honest, Direct, and Professional
 Provide Support for Child & Parent
 Post-Traumatic Stress Disorder (PTSD): children with ACE’s & maltreatment are high risk;
caused by traumatic incident, repeated trauma, disorder, natural disaster, sexual abuse, etc.
o Initial Response: few min. to 2 hrs; increase in stress hormones (fight/flight), psychosis
o Second Phase: lasts about 2 weeks, period of calm (numbness, denial), defense
mechanisms decrease
o Third Phase: extended about 2-3 months, get worse; depression, phobias, anxiety,
conversation reactions, repetitive movements, flashbacks/obsessions
o Nursing Care –
 Referral to appropriate services, monitor behavioral changes, assist family with
coping, allow expression of feelings, prevent long term effects
Final Exam Review Pt. 2
38
SOCIAL, CULTURAL, RELIGIOUS, & FAMILY INFLUENCES
9 questions with ACEs
 Family System Theory
o Family System: a change in one, affects entire family dynamic – can initiate and react to
change; too much or too little change can cause dysfunction
o Rapid Periods of Growth/Changes  Periods of Stability
Test Q: Define
 Strengths: works for normal family, family with dysfunction or pathology,
Family System
families of varying structure or stages of lifestyle
 Limitations: more difficult to determine cause & effect
 Applications: mate selection, courtship, family communication, boundary
maintenance, power & control
o Family Stress: family cope with and responds to stressors with wide range of responses
and effectiveness
 Strengths: predict family behaviors and develop interventions, focuses on
positive contributions, used by many disciplines
 Limitations: not certain if combo of resources and coping are applicable for all
 Applications: transitions in family
 Developmental Theory –
o Stage 1: marriage & independent home; couple identity, realign relationships with
extended family, parenthood decisions
o Stage 2: families with infants; integrate into family, new parenting/grandparent roles,
maintain martial bond
o Stage 3: families with preschoolers; socialize children, adjust to separation
o Stage 4: families with school children; develops peer relationships, parents adjust to
peer and school influences on child
o Stage 5: families with teenagers; autonomy, midlife marital/career issues, parents shift
concern to older generation
o Stage 6: families as launching centers; independent identities, renegotiate marriage
o Stage 7: middle aged families; reinvest in couple identity, disabilities/death of
grandparents
o Stage 8: aging families; from work role to leisure and retirement, maintain couple and
individual functioning and adapt to aging process
 Family Structure –
o Traditional: married couple with ONLY biological children
Test Q: Child lives with
o Nuclear: 2 parents [don’t have to be married] & their children
her mother, 2 biological
o Blended: at least one step parent, step sibling, or half-sibling
siblings, and step-father.
o Extended: at least one parent, one or more children, or one or more members; other
What family structure is
than a parent or sibling
this?
Final Q: Child lives with her
 Blended
o Binuclear: parents continue role while ending spouse role
grandmother, biological
o Polygamous: multiple wives
brother, and her mother.
What is the family structure?
o Communal: cult
 Extended
o LBGTQ: same sex parents
 Parenting Styles –
o Authoritarian: try to control child with unquestioned mandates
o Permissive: exerts little or no control over child, rarely punishes
o Democratic/Authoritative: combo of authoritarian & permissive. Discuss reasons for
rules and negatively reinforce deviations. Rely on guilt & shame.
o Passive: parent uninvolved with discipline at all; child in control
Final Exam Review Pt. 2
39
 Parenting & Divorce
o Process:
 Acute: decision to separate; several months to more than a year; family stress
and chaotic atmosphere
 Transitional: family assumes unfamiliar riles with new family structure; larger
share of economic burden and altered parent-child relationships.
Test Q: What age child
 Stabilizing: post-divorce family reestablishes a stable, functioning family
would most likely blame
o Impact:
themselves for their
 Infancy (0-2): eating, sleeping, elimination disturbances & parental attachment
parent’s divorce?
 Early PreK (2-3): blames self, fear of abandonment, regressive behaviors
 3-year-old
 Later PreK (3-5): fear of abandonment, responsible for divorce and interpret as
punishment, increased aggression, fantasy
Test Q: Children may feel they
 Early School Age (5-6): depression, immature behavior, sleep disturbances, loss
are responsible for their parents’
of appetite, anxiety, may be able to verbalize feelings
divorce and interpret the

Middle School Age (6-8): panic reactions, deprivation, sadness, depression, fear,
separation as punishment. At
what age is this most likely to
insecurity, fear of abandonment, desire for reconciliation, impaired capacity to
occur?
enjoy playing, altered peer relationships, decline in school
 4 years
 Later School (9-12): more realistic, intense anger, decline in school, revenge,
loneliness, rejection and abandonment, loyalties, aberrant activities
 Adolescence (12-18): able to disengage from conflict, profound sense of loss of
family/childhood, anxiety, withdrawal
 Types of Discipline to Minimize Misbehaviors –
o Reasoning: Older Children
o Scolding: Shame & Criticism
o Behavior Modification: Reward Good Behavior, Ignoring Negative Behavior
o Time Out
Test Q: Which of the following
o Corporal: Physical Punishment
(after family) is most likely to have
 not effective long term
the greatest influence on providing
continuity between generations?
 Forces to Use When Implementing Discipline –
 Schools
o consistency, timing, commitment, unity, flexibility
o planning, behavior orientation, privacy, termination
 Sociocultural Influence of Families –
Final Q: Which
surrounding
o Surrounding Environment
environment is an
 School Connectedness: important site of health promotion
important site for
 Peer Culture
health promotion in
 Social Roles Influenced by Community
children?
Test Q: Parents of
 School
o Local Community Influences
dying child are anxious
o Social Determinants
Test Q: Most violence,
& upset because they
substance abuse,

Race,
Ethnicity
can’t find a priest for
unrealistic body images
o Social Class
last rights. What is the
come from:
o Religious & Traditional Influences
nursing diagnosis?
 Mass Media
o Mass Media Influences
 Cultural Traditions for Health –
o Physical Aspects of Caring for Body
 special clothes, food, medicine
o Feelings, Attitudes, Rituals, Actions r/t Health
o Spiritual Aspects of Health
 identity, customs, prayers, healing
Final Exam Review Pt. 2
 Health Beliefs & Practices –
o Natural
o Supernatural
o Imbalance of Forces
o Health Protection
 folk healers’ practices & remedies
 faith healing
 religious rituals
40
Test Q: Pt. thinks God will heal their
child with no other medical help.
Nurse on shift makes a derogatory
comment. What should you include
in your next statement?
 something to help the patient
cope, promote comfort, respect
cultural beliefs