The Nurses

Role

The nurses role in a

patient with Cystic
Fibrosis is significant.
Patients with CF will
have other care
providers seeing them,
for example respiratory
therapy. Its important
that the nurse take into
consideration the
instructions that the
other provider gives the
patient to be sure the
patient understands and
complies with them. The
patient should always
be first priority so when
a patient doesnt
understand the nurse
can explain or advocate
and reach out for
further assistance. Its
especially important
that the nurse is
knowledgeable of the
disease. Respiratory

Lets test your knowledge!

1. Which of the following clinical manifestations are related to hypoxia?
a. Hemoptysis
b. Cough
c. Finger Clubbing
d. Chest Pain
2. What kind of disorder is Cystic Fibrosis?
a. Autosomal Recessive
b. Autosomal Dominant
c. Sex-linked Disorder
d. Mitochondrial Disorder
3. A child with Cystic Fibrosis would present with what clinical manifestations? (Select all that apply)
a. Jaundice
b. Rectal Prolapse
c. Weight Gain
d. Inadequate Salt Depletion
e. Sweat Abnormalities
Answers: 1. C 2. A 3. A, B, E

Nursing Diagnosis for a patient with Cystic Fibrosis:

1. Risk for ineffective airway clearance related to increased production of thick mucus. Client will demonstrate
effective coughing and clear breath sounds.
2. Risk for infection related to thick tenacious mucus and harboring of bacterial organisms. Client will state
symptoms of infection and demonstrate appropriate hygienic measures such as hand washing and oral care.
3. Risk for impaired gas exchange related to ventilation perfusion mismatch. Client will maintain clear lung fields
and verbalize understanding of oxygen supplementation and other therapeutic interventions.

Cystic Fibrosis
A quick guide to what Cystic Fibrosis is, what signs and symptoms to look for, and what the nurses
role is for a patient with Cystic Fibrosis.

What
is Cystic
by Lindsay Johnson
Fibrosis?
Cystic Fibrosis (CF) is an
autosomal recessive disorder,
meaning its inherited from ones
parents. Both parents must
possess at least one copy of the
gene in order for it to pass to
the offspring, however, because
they carry the gene does not
mean that the gene will present
in the patient. This disorder
affects the electrolytes and
water transport of the cells in
the respiratory, digestive, and
reproductive tracts. The
mutation causes an inability of
epithelial cells to conduct
chloride and therefore transport
water across the mucosal
surfaces. This leads to thick
secretions and potentially
obstructions. CF is strongly
associated with mucus plugging,
as well as inflammation and
infection of the lungs leading to
respiratory failure. Mucus
plugging is due to increased
amounts of mucus, dehydration
of the airway with thickened
mucus, and the impaired
function of the cilia because the
mucus adheres to the epithelium
where the cilia are present.

What are the clinical manifestations?

Clinical manifestations of CF vary based on progression of the disease and age of the patient. Overall, one can expect
thick secretions leading to a chronic cough accompanied by purulent sputum or mucoid. Because of the thick secretions
on the lungs a patient will want to expel them; forceful coughing can lead to vomiting and erosion of lung tissue near
bronchial arteries leads to hemoptysis. Patients of all ages can also present with tachypnea, wheezing/crackles due to
the fluid on the lungs, dyspnea on exertion, chest pain, and respiratory distress with chest retractions, cyanosis, and
barrel chest related to the use of using accessory muscles to breathe, recurrent sinusitis, and development of nasal
polyps. Occasionally, a patient may develop finger clubbing due to hypoxia. Newborns are at risk for a meconium ileus
with delayed or no passage of meconium stool. Infants and Children demonstrate increased frequency of large,
greasy, malodorous stools. Accompanied by recurrent abdominal pain, distention, and lack of weight gain. Jaundice,
gastrointestinal bleeding, and rectal prolapse can occur. They may also show sweat abnormalities and excessive salt
depletion. Kissing a child with CF can lead to a salty taste because of this excessive salt depletion. Males are generally
sterile because they have no vas deferens. Male children have undescended testicles. Females have delayed secondary
sexual development, however fertility is not always compromised and only somewhat decreased.

How to diagnose CF?

Diagnosis is generally based on a thorough patient history and physical exam looking for the clinical manifestations
mentioned previously. Sweat tests can be completed to confirm diagnosis. Genetic testing can detect mutations of the CF
gene. Additional testing like a chest radiography can show hyperinflation and peribronchial thickening.

How to treat CF?

When

treating CF patients the goal is to maximize ventilation, diffusion, and nutrition. This is done by:
Clearing airways of mucus
Avoiding and controlling respiratory infections
Reducing inflammation of airways
Optimal nutrition intake
Managing disease complications

Who is at risk?
Like mentioned earlier, cystic fibrosis affects those with parents who are both carriers. Meaning both of them carry at
least one of gene with the CF mutation. Median life expectancy for CF patients is 30 years, with males living exceptionally
longer. Patients are starting to live into their 40s and 50s now.