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

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) http://www.youtube.com/watch?v=Gh2Ovh0uAss

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 13-20/100,000 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) - CT or lung biopsy are needed for diagnosis

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 d

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/