1. PULMONARY FIBROSIS

2. 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

3. PATHOPHYSIOLOGY 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)

4. VIDEO

5. 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)

6. PREVELANCE Usually seen in middle-aged to elderly adults 13-20/100,000
Range 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)

7. DIAGNOSITC TESTS CT PFT CXR Lung biopsy (King, 2011)
- CT or lung biopsy are needed for diagnosis

8. RISK FACTORS Smoking Exposure to metal and wood dust Taking amiodarone
Genetic link
Only in % of cases
Hx of pulmonary diseases
Acute respiratory distress syndrome, TB, sarcoidosis, rheumatoid arthritis
(King, 2011)

9. 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)

10. 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)

11. 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

12. 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

13. 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

14. NCLEX QUESTION #1
Which of the following diagnostic tests is required to make the final diagnosis?
A. PFT
B. CXR
C. ABG
D. CT d

15. 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

16. 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:  
King, T. E., Pardo, A., & Selman, M. (2011). Idiopathic pulmonary fibrosis. The Lancet, 378(9087). Retrieved from science/article/pii/S
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