BSN 2C – DE LOS REYES RLE PEDIA – Prof.

Rita Penaso 06/28/2021

Nursing Management of the Child with Cystic Fibrosis

Case Scenario – A 10 -month-old male infant with a neonatal history including meconium plug is admitted with respiratory difficulty, recurrent bronchitis, and poor weight gain.
The health care provider suggests a potential diagnosis of Cystic Fibrosis.

CUES NURSING OUTCOME CRITERIA INTERVENTION RATIONALE EVALUATION DISCHARGE

DIAGNOSIS PLANNING

Subjective: Ineffective airway After 30 minutes – 1 hour Independent: MET –. After 30 M – Advise the
 10 -month- clearance r/t cystic of nursing intervention: minutes – 1 hour of parents to give their
old male fibrosis as 1. Assess vital 1. To establish baseline data nursing intervention infant medicines
infant evidenced by a. Patient will be able signs such as Albuterol
 Neonatal respiratory to breathe more 2. To establish smooth and (bronchodilators), or
history difficulty, recurrent easily by nurse 2. Establish open nurse-patient a. The patient Dornase alfa
including bronchitis and poor performing Chest rapport relationship was able to (decreases mucus
meconium weight gain. physiotherapy breathe viscosity).
plug (CPT) and 3. Obtain weight 3. Monitor weight gain and more easily
Administer Oxygen growth progress/decline E - Instruct the
Objective: as Dependent: of the infant parents of the infant
 Respiratory needed/ordered After 5 weeks of to stay in a calm and
- 1. Administer 1. Maintaining adequate nursing peaceful
wheezing/c After 5 weeks of nursing Oxygen as oxygenation intervention: environment. Home
oughing, intervention: needed/ordere a. Patient was environment must
meconium a. Patient will be able d able to be clean and it
plug to improve/gain improve/gai should have
 Gastrointes weight via intake 2. Chest 2. Promoting measures to n weight adequate lighting
tinal – poor of high physiotherapy remove pulmonary and ventilation.
weight gain protein/caloric (CPT) secretions via CPT - a
diet as well as technique used to T – In treating
adequate fluid mobilize or loose Gastrointestinal
intake, fat-soluble secretions in the lungs and symptoms of Cystic
vitamins (Vitamin respiratory tract. Fibrosis, advise the
A, D, E, K) and Collaborative: parents to give the
pancreatic enzyme pancreatic enzyme
supplements. Since the health care supplements to their
provider suggests a infant within 30
potential diagnosis of minutes of eating.
cystic fibrosis, we Taking pancreatic
should perform supplements could
1. Sweat A sweat electrolyte test detects help in issues of
Electrolyte Test the amount of sodium and Cystic Fibrosis.
or Genetic chloride in your sweat. It’s also
Testing to called an iontophoretic sweat test H - Instruct the
confirm the or chloride sweat test. This test is parents to monitor
health care usually performed on children food intake and
provider’s with suspected symptoms of Cystic weight gain of their
suggestion of Fibrosis. There are no risks infant.
potential associated with this test and is also In giving pancreatic
diagnosis of not painful. Meanwhile, Genetic enzymes, educate
Cystic Fibrosis. testing looks for changes in your the parents of the
DNA that can inform your medical infant to swallow and
care. not chew or crush
them. If the infant
can’t swallow the
capsule, advise the
parents of the infant
to open it and put it
in an ACIDIC FOOD
like apple sauce. Do
not mix in an
alkaline-based food
like yogurt, pudding
or ice cream because
this will inactive the
enzyme. Administer
these enzymes
before all meals and
snacks

O - Instruct the
parents to comeback
after a week for
follow-up check-up
of their infant and to
immediately
seek/contact medical
assistance from
health care provider
if the infant is
experiencing any
health issues and for
stay up-to-date with
vaccinations of their
infant.

D – Aid parents of
infant in planning a
high protein/caloric
diet to help infant in
gaining weight.
Also advise parents
to give infant fat
soluble vitamins –
Vitamin ADEK, to
increase fat intake.

S – Advise the
parents to seek
support system by
contacting relatives
 and friends for
additional help in
taking care of their
infant.

Guide Questions:

1. What additional history questions should the nurse ask the family? Explain the rationale for each question.

 The nurse should ask the infants’ parents whether there is a family history on both sides of Cystic Fibrosis – because Cystic Fibrosis is a genetic (autosomal recessive)
disorder that causes thick, tenacious mucus to obstruct (meconium plug) a variety of organs including lungs, liver, pancreas, small intestine, and reproductive organs.

2. What are the priority interventions for this infant and family (in order of occurrence)?

 Respiratory difficulty – airway obstruction due to mucus obstruction that may hinder the infant to breathe properly, worse case may result to respiratory failure/death
 Gastrointestinal issues – poor weight gain, which may result to imbalanced nutrition and delayed growth
o Treatments for CF focus on improving breathing and digestion, preventing and treating infections, and thinning mucus. Treatments include medicines, therapy to
clear mucus out of the lungs, and in some cases, lung transplant.

References:

a. https://www.cdc.gov/genomics/disease/cystic_fibrosis.htm - Cystic Fibrosis

b. https://www.healthline.com/health/sweat-electrolytes-test - Sweat Electrolyte Test
c. https://www.cdc.gov/genomics/gtesting/genetic_testing.htm - Genetic Testing
d. https://currentnursing.com/reviews/chest_physiotherapy.html - Chest physiotherapy (CPT)
e. https://medlineplus.gov/druginfo/meds/a694002.html - Dornase alfa
f. https://medlineplus.gov/druginfo/meds/a607004.html - Albuterol
g. https://www.healthline.com/health/pancreatic-supplements - Pancreatic Enzymes Supplements