Pulmonary Fibrosis
Patient Presentation:
R.S., a 65 year old male presented to an outside hospital with increased fatigue, weakness, loss of appetite with a 12lb weight loss, dry hackling cough, chest discomfort, and SOB with exertion. The patient had been in and out of the hospital multiple times in the past 3 years with similar symptoms. R.S. had an occupational history of working in coal mines for 30 plus years. During this admission his symptoms were progressively worsened with an increase in dyspnea with activities of daily living. Patient complained he was unable to function and do normal everyday tasks anymore because he would get so short of breath. He needed his wife much more for support with dressing and walking. Patient had prescribed home O2 and was obligated to increase his oxygen consumption, as well as, use of his BiPAP®. Patient was sent to a larger hospital after not improving for two weeks for a pulmonary specialist consult.
Differential List:
pneumonia, chronic bronchitis, COPD, farmer’s lung, coal worker's pneumoconiosis, interstitial lung disease/pulmonary fibrosis, sarcoidosis, pulmonary histiocytosis X, connective tissue disease, or neuromuscular disease. All of these are restrictive lung diseases.
Labs:
CBC with differential, CMP, blood and sputum cultures, ABG, coags, antinuclear antibodies, rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, ACE testing, chest X-ray, ECHO, and high resolution chest CT scan, bronchoscopy, and most importantly pulmonary function testing.
Diagnosis:
ABG: showed respiratory acidosis. Chest x-ray and high resolution CT scan revealed bilateral reticular and nodular interstitial infiltrates and areas of “honeycombing”. Pulmonary function testing: FEV1 and FVC were significantly reduced, both around 2.5L (approximately 50% of normal, as determined by sex and age of similar population), while the FEV1/FVC ratio was WNL. A lung biopsy five years ago, revealed the presence and pattern of pulmonary fibrosis. Pulmonary fibrosis, a form of interstitial lung disease, is thought to begin with acute injury to the pulmonary epithelium, leading to chronic interstitial inflammation, with fibroblast activation and proliferation, and finally progressing to pulmonary fibrosis and tissue destruction. There are multiple causative factors leading to the disease.
Treatment:
The treatment for pulmonary fibrosis includes: combination therapy of corticosteroids, azathioprine, N-acetylcysteine, supplemental oxygen, pulmonary rehabilitation with a pulmonary specialist, and finally lung transplantation.
Outcome:
The prognosis for R.S. was poor. The fibrosis worsened and significantly decreased R.S.’s lung compliance leading to difficult breathing and poor blood oxygenation. MICU MD’s and the attending pulmonologist stated that R.S. would probably not leave the hospital setting and that his status was terminal
R.S. knew that his current condition was not good. He had fought with the disease for 5 years and it got progressively worse. He was very restless and knew the disease was going to be fatal. R.S. asked his nurse if the pulmonologists at the bigger facility could do anything more for him. He discussed with the nurse he was tired of the BiPAP® and of being in bed for two weeks struggling to breathe. The nurse contacted the MICU MD to further discuss with the patient his diagnosis and prognosis in detail. The patient understood his medical condition and after talking with the MICU/MD team and pulmonologist, decided to move to comfort care at that time. R.S. died early the next morning after withdrawal of medical treatment and supplemental oxygen.
Case created by Randi Shupp, 2012.