Idiopathic Pulmonary Fibrosis

Question: Describe about idiopathic pulmonary fibrosis. Answer: Idiopathic pulmonary fibrosis is a respiratory disease where the development of scar tissue, or fibrosis, occurs in the pulmonary muscles, making them thicker and restricting the flow of oxygen into the bloodstream. Such blockage in pulmonary circulation leads to cerebral hypoxemia, along with hypoxia in the other body tissues, ultimately causing respiratory failure (Sarkavas et al., 2013). Emphysema implies accumulation of air in the alveolar sacs leading to their disruption and is one of the key symptoms of COPD (Chronic obstructive pulmonary disorder). Symptoms and triggers of CPFE have been found to be quite similar to that of COPD (smoking, viral infection, genetic defects), and pulmonary rehabilitation measures have been found to be quite useful in case of COPD patients (Das et al., 2013). However, such interventions havent shown any considerable success in the case of CPFE patients (Tomioka et al., 2016). Therefore, this study aimed to investigate the benefits of aerobic retraining or respiratory exercising CPFE patients can derive, as an alternative to pulmonary rehabilitation. In this study, the subject is a 65-year old Caucasian man, suffering from CPFE, who has been an intensive smoker for almost four decades. He was reported to have developed several co-morbidities along with CPFE, including hypertension, type II diabetes and depression along with a long history of chronic bronchitis. A thoracic HRCT scan revealed intralobular thickening of the alveolar septum and interstitium, along with the loss of respiratory bronchioles indicating a combined syndrome of lower-lobe fibrosis with upper-lobe emphysema (Sarkavas et al., 2013). As an allied therapy of CPFE along with pulmonary rehabilitation, aerobic retraining program was carried out for four consecutive weeks, five days a week and two sessions per day, where each session lasted for up to 30 minutes. The retraining program comprised of an aerobic exercise session, which included exercising on a treadmill, followed by a breathing session, comprising of diaphragmatic breathing and respiratory muscle training. These sessions were being monitored continuously by measuring the heartbeat, dyspnoea level, oxygen saturation, respiratory rate and blood pressure of the patient (Jankowich Rounds, 2012). Elevated partial pressure of oxygen and reduced partial pressure of carbon-dioxide after exercising allowed for the reduction in the concentration of the long term oxygen therapy administered to the patient (2.5L/min was reduced to 1.5 L/min). This seems to have stemmed from the improved health of the patients respiratory muscles, which also augmented his exercising capacity. Also, systolic pressure in the pulmonary artery was shown to have improved, along with decreased dyspnoea, decreased post-exercise VAS and better performance (133% improvement) in the 6-minute walk test. However, no improvement in lung function was observed. In conclusion, it can be stated that this study has sufficiently demonstrated the beneficial effects of physical exercise on the psychological well-being and the quality of life of CPFE patients, while the role of pulmonary rehabilitation has not been discussed. This study conclusively established the short term benefits of aerobic training in CPFE patients, while long-term benefits need to be investigated through further research along with developing pulmonary rehabilitation programs specifically suited to the needs of CPFE patients. Development of additional exercise and treatment methodologies, together with aerobic training, is likely to be much more effective in giving new hope for the life of people with CPFE. References: Das, J., Murphy, D., Keane, M. P., Donnelly, S. C., Dodd, J. D., Butler, M. W., Mitchell, P. (2013). Idiopathic Pulmonary Fibrosis With Emphysema Has A More Severe Response To Smoke Than Other Forms Of Usual Interstitial Pneumonia With Emphysema.Am J Respir Crit Care Med,187, A3732. Jankowich, M. D., Rounds, S. I. (2012). Combined pulmonary fibrosis and emphysema syndrome: a review.CHEST Journal,141(1), 222-231. Sarkavas, J. A., Karkhanis, V. S., Joshi, J. M. (2013). Pulmonary Fibrosis and Emphysema.Indian J Chest Dis Allied Sci,55, 97-99. Tomioka, H., Mamesaya, N., Yamashita, S., Kida, Y., Kaneko, M., Sakai, H. (2016). Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease.BMJ open respiratory research,3(1), e000099.