PULMONARY

FIBROSIS


OBJECTIVES
Understand the basic pathophysiology of pulmonary
fibrosis
Identify presenting symptoms of the disease
Know what diagnostic tests are performed to confirm
the diagnosis
Discuss some of the treatment options
PATHOPHYSIOLOG
Y
Excess scar tissue build
up in the lungs
Decreases the ability of
the lungs to expand
Causes less oxygen to
be exchanged in the
lungs
Leads to hypoventilation
and hypercapnia

(Huether, 2012)
VIDEO
http://www.youtube.com/watch?v=Gh2Ovh0uAss

PRESENTING SYMPTOMS
Dyspnea on exertion
Diffuse inspiratory crackles
Cough
Hypoxemia
At rest and worsens with activity
Clubbing
Chronic hypoxemia as the disease progresses






(King, 2011)
PREVELANCE
Usually seen in middle-aged to elderly adults
Range 55-75 years old
2/3 of people diagnosed are over 60 (Rafii, 2013)
13-20/100,000
Men are diagnosed more often than women



(King, 2011)


DIAGNOSITC TESTS
CT
PFT
CXR
Lung biopsy



(King, 2011)

RISK FACTORS
Smoking
Exposure to metal and wood dust
Taking amiodarone
Genetic link
Only in 0.5-3.5% of cases
Hx of pulmonary diseases
Acute respiratory distress syndrome, TB, sarcoidosis,
rheumatoid arthritis


(King, 2011)

TREATMENT
No current cure
Oxygen
Corticosteroids
Cytotoxic drugs
Clinical trials in progress
Lung transplant
Only treatment that prolongs life in patients with pulmonary
fibrosis
Most die waiting for transplant
Should be referred at diagnosis for best chances of receiving
lung transplant

(King, 2011)

SURVIVAL RATE
Average survival rate is around 3-5 years after
diagnosis
Prognosis is worse with
Hx of smoking
Age >70
Low BMI
Pulmonary hypertension



(King, 2011)

PATIENT SCENARIO- HPI
The patient is a 76 year-old white male who came into
the ER complaining of shortness of breath during
minimal activity and a persistent cough. Noticed
increasing difficulty breathing for past 3 months.
Relaxing with no activity decreases symptoms. On
assessment auscultated diffuse crackles in the lungs
LABS/TESTS
Patient had CT scan and lung biopsy
Both indicated pulmonary fibrosis
Doctor ordered PFT to determine current respiratory
function and to track the progress of the disease in the
future
NURSING DIAGNOSES
Impaired gas exchange r/t excess scar tissue in the
lungs secondary to pulmonary fibrosis aeb shortness of
breath with exertion
Activity intolerance r/t imbalance between oxygen
supply/demand aeb patient inability to walk short
distances
Powerlessness r/t effects of illness and oncoming death
aeb no motivation in activities or events
NCLEX QUESTION #1
Which of the following diagnostic
tests is required to make the final
diagnosis?
A. PFT
B. CXR
C. ABG
D. CT
NCLEX QUESTION #2
Which of the following is not a
presenting symptom of pulmonary
fibrosis?
A. dyspnea on exertion
B. chest pain
C. cough
D. diffuse crackles
REFERENCES
Ackley, B. J., Ladwig, G. B. (2011). Nursing diagnosis handbook. St.
Louis, MO. Mosby, Inc.
Huether, S. E., & McCance, K. L. (2012).Understanding
Pathophysiology (5th ed.). St. Louis, MO: Mosby. ISBN: 978-
0-323-07891-7
King, T. E., Pardo, A., & Selman, M. (2011). Idiopathic pulmonary
fibrosis. The Lancet, 378(9087). Retrieved from http://
www.sciencedirect.com.ezproxy.lib.usf.edu/
science/article/pii/S0140673611600524
Rafii, R., Juarez, M. M., Albertson, T. E., & Chan, A. L. (2013). A
review of current and novel therapies for idiopathic pulmonary
fibrosis. Journal of Thoracic Disease, 5. Retrieved from http://
www.ncbi.nlm.nih.gov/pmc/articles/PMC3548009/