NCLEX QUESTIONS
REFERENCES
1. The most common cause of death in
individuals with Cystic Fibrosis is:
CYSTIC FIBROSIS. (2013, JULY 13). IN MAYO
CLINIC. RETRIEVED FROM
HTTP://WWW.MAYOCLINIC.COM/HEALTH/CYSTICFIBROSIS/DS00287
a. Respiratory failure
b. Infertility
c. Rectal prolapse
d. Muskulosketel complications
2. A mother tells the nurse that her
child makes "snoring" sounds when
breathing. The nurse concludes that many
children with cystic fibrosis also have:
a. Plugged noses
b. Enlarged adenoids
c. Nasal polyps
d. Septal deviations
3. Who is most likely to be recently
diagnosed with Cystic Fibrosis?
a. An elderly African American woman
d. A middle aged man at an annual
check-up
Abigail Fish
Nurs 300
CYSTIC FIBROSIS. (2013). IN NURSING
CENTRAL. RETRIEVED FROM
HTTP://WWW.UNBOUNDMEDICINE.COM/NURSING
CENTRAL/UB/VIEW/DISEASES-ANDDISORDERS/73732/4/TUBERCULOSIS
LADWIG, G. B., & ACKLEY, B. J. (2011). GUIDE TO
NURSING DIAGNOSIS (3RD ED.). MARYLAND
HEIGHTS, MISSOURI: MOSBY/ELSEVIER.
O’SULLIVAN, B.P., & SASSETTI, C.M., (2013).
INFECTION CONTROL IN CYSTIC FIBROSIS:
SHARE AND SHARE ALIKE. THE LANCET,
381(9877), 1517.
DOI:HTTP://DX.DOI.ORG/10.1016/SO1406736(13)60740. RETRIEVED FROM CINAHL.
SCOTT, A. (2013). CYSTIC FIBROSIS.
RADIOLOGIC TECHNOLOGY, 84(5), 493-518.
RETRIEVED FROM CINAHL.
IMAGES FROM IMAGES.GOOGLE.COM
b. A newborn Caucasian male
c. A newborn African American female
with HIV
Cystic
Fibrosis
Abigail Fish
Cystic Fibrosis
Ferris State University
Date of publication
ETIOLOGY: Cystic Fibrosis is an autosomal
Complications: Due to the sticky mucous created by
recessive disease caused by defects in the CFTR
gene. CFTR codes for a protein that functions as a
chloride channel, as well as regulates the flow of
other ions across epithelial cells.
Cystic Fibrosis, there are many complications throughout
the body. Respiratory failure is the most common cause of
death. The digestive tract and the reproductive system are
also affected. Other complications include:
Incidence: CF affects nearly 30,000 children and
adults in the United States and 70,000 worldwide.
About 1,000 new cases of CF are diagnosed each
year.
Risk Factors: Family history is a major risk factor
since it is a defective gene that can be inherited
and runs in the family. Race is also an indicator.
Even though it can occur in all races, it is most
common in white people from Northern European
descent.
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Chronic Infections
Pneumothorax
Respiratory failure
Nutritional Deficiency
Rectal Prolapse
Intussusceptions
Nasal Polyps
Blocked Bile duct
Infertility (Mayo Clinic, 2013)
Pathophysiology: Cystic Fibrosis is caused by the
defects in the cystic fibrosis gene, located on
chromosome 7. This gene codes for a protein
transmembrane conductance regulator (CFTR).
Deficiencies in this gene (CFTR) cause decreased
secretion of chloride along with increased
reabsorption of sodium and water across the
epithelial cells. This results in a decreased
hydration of mucous which will cause the mucous
to become stickier than bacteria. In turn, these
combining factors promote infection and
inflammation. Secretions in the respiratory tract,
pancreas, GI tract, sweat glands, and other
exocrine tissues have increased viscosity, which
makes them difficult to clear.
LIFESPAN AND
CULTURAL
CONSIDERATIONS:
CLINICAL MANIFESTATIONS: Patients with CF
SIGNIFICANCE TO NURSING:
can experience many different symptoms with this
disease since it effects the secretions of almost
every system. Symptoms include:
The disease occurs mostly in whites whose ancestors
came from northern Europe. CF is typically diagnosed
between 6-8 months.
Cystic Fibrosis is less common in African Americans,
Native Americans, and Asian Americans.
Approximately 1 in every 20 Americans is an unaffected
carrier of an abnormal CF gene (Scott, 2013).
Because CF is a genetically inherited disease, nurses do
not need to worry about contracting the disease. It is more
important that the individual with the disease is not
exposed to infection, or in too close of proximity as
someone else with CF. The nurse must:
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Salty tasting skin
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Persistent cough, sometimes with phlegm
production
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Administer all drugs prescribed accurately and
thoroughly.

Frequent lung infections
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Wheezing or shortness of breath
Wash hands to prevent spread of infections to
patient
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Poor growth/weight gain, despite a healthy
appetite.
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Record and report all relevant information in
charting
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Greasy, bulky stools or difficultly with
bowel movements
Other providers that may be involved in CF treatment
might be: Respiratory therapist or Pulmonologist.
NURSING DIAGNOSIS
1. Ineffective airway clearance r/t
excess tenacious mucous.
2. Impaired gas exchange r/t
ventilation perfusion imbalance.
3. Imbalanced nutrition: less than
body requirements r/t
decreased absorption of fats
and nutrients.
OUTCOME CRITERIA
1. Demonstrate effective coughing
and clear breath sounds as well as
explain the significance of
changes in sputum color,
character, amount, and odor.
2. Demonstrate improved ventilation
and adequate oxygenation as
evidenced by blood gases with a
normal range for the patient
3. Progressively gain weight toward a
desired, healthy goal weight that is
within a normal range for
individual’s height and weight