NURS 421
Nursing Grand
Rounds
Cystic Fibrosis in
Pediatrics
Annette Fernandez
Client History & Assessment
 15mo
newly diagnosed with cystic fibrosis
 Diagnosed
admission
with CF 2 mos before current
Current admission chief complaint:
cough & congestion / LLL
pneumonia
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Relationship of dx to CF
CF – thick mucus
Increased Na and Cl secretion – Cl sweat test
Increased secretion increases mucus viscosity
Chronic presence of thick mucus increases
risk for infection
CF predisposes patients to frequent lung
infections
Client History & Physical
Assessment
 Diminished
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lung sounds – clear after CPT
CPT done manually with Albuterol Nebulizer
Patient was going to be fitted for vest
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 5th percentile growth chart weight
 25th percentile growth chart height
 Great
grandmother briefly discussed
illness in her family and closeness among
family members – shared values/beliefs in
this family - culture
CPT – Research Article
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“Comparison of Settings Used for High – Frequency
Chest Wall Compression in Cystic Fibrosis”
Research take-aways:
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Possible advantage to using higher-pressure/variablefrequency settings for high frequency chest wall
compression devices in cystic fibrosis patients
Unbiased comparison
Further investigation is needed to assess long-term
outcomes – respiratory exacerbations, PFT, quality of life
Kempainen, R., Milla, C., Dunitz, J., Savik, K., Hazelwood, A.,
Williams, C., & ... Billings, J. (2010). Comparison of settings used for
high-frequency chest-wall compression in cystic
fibrosis. Respiratory Care, 55(6), 695-701.
Expected Developmental
Stage
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Erikson: Autonomy vs. Shame and Doubt
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After infants gain trust, they begin to realize that
their behavior is their own
Dependency creates a sense of doubt in their
own abilities
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Self feeding, making the individual choice to
turn away from medications, playing with
stethoscope
Piaget: Sensorimotor stage
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Progress from reflex activity, through simple
repetitive behaviors, to imitative behaviors
Playing with the stethoscope – learning
purposeful movement
 “imitating” older sister
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Identification of Nursing
Problems/Plan of Care
 Nursing
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problems:
Risk for impaired gas exchange
Altered nutrition: less than body
requirements
Risk for infection
Risk for activity intolerance
Interrupted family process
Actual/Potential Problems
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Risk for impaired gas exchange (potential)
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O2 sats were above 96% when spot checked on
RA – gas exchange was not an actual problem
Altered nutrition – less than body requirements
(actual)
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Weight in 5th percentile – low end of normal
limits (5-95%)
Diet high-fat, high-calorie, no enzyme
No significant weight changes
Actual/Potential problems
con’t
 Risk
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Admitted to hospital with infection –
pneumonia
increased thick secretions due to CF
 Risk
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for infection (actual)
for activity intolerance (potential)
Walked to play room with great
grandmother
Appeared to have no issues moving
around, unlabored breathing
Actual/Potential problems
con’t
 Interrupted
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family process
New diagnosis
Family handled it very well – constant
visitors
Family fed, played, observed treatment
Great grandmother discussed illness in the
family
Care given to patient
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Traditional/Collaborative:
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High-fat, high calorie diet
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Duo cal packet with soy milk (water/juice
restriction)
Sputum culture
Spot check pulse ox
PICC line
CPT
Medication
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Albuterol, Pulmozyme, Antibiotics
Care given to patient
 Alternative:
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Access to play room
“play” with stethoscope
“play” interaction with patient to build a
sense of familiarity and trust
Teaching/Discharge
 When
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to call physician/911
Fever, itchy, achey, sleeps less, urinates less,
dry mouth, cracked lips
Immediately seek care if:
 Coughing
blood, pain in abd that does not
go away, trouble breathing, blue nails/lips
 Use
of nebulizers at home/when to
 Chest compression vest
Teaching/Discharge
 Antibiotic
use – to full term even if
“appearing better”
 Diet – high calorie/high protein
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Add creams, butter, cheese, peanut butter,
mayonnaise, powdered milk, high calorie
drinks like carnation, pediasure, etc.,
vitamins supplementation, salt
Questions??