# Exercise as it relates to Disease/Effect of fibrosis on exercise ability and capacity
## What is Pulmonary Fibrosis

Pulmonary Fibrosis is a disease where scar tissue forms in the small air sacks (alveoli) of the lungs, causing shortness of breath and coughing, progressively getting worse until it takes the patient’s life. The most common form of pulmonary fibrosis is Idiopathic Pulmonary Fibrosis (IPF) which effects about 10 per 100,00 people in Australia each year.

## Symptoms and Diagnosis

Idiopathic Pulmonary Fibrosis has no known cause. Patients tend to be diagnosed in their early 60's. There are rare cases where it can develop sooner but this is not very common. The first symptom to develop is most commonly a dry unexplained cough that progressively worsens, this is then followed by a shortness of breath and decreased exercise ability. There are multiple ways in which IPF can be diagnosed.

## Treatment

Treatment of IPF is very limited. There is no cure and only a few medications have shown to be beneficial. Corticosteroids is one of the medications that has had success when treating IPF with 10-30% of patients reported positive effect, but results vary from patient to patient. Another medication that is said to work for some patients are immunosuppressive drugs, but again, results are varied.
There have been new studies however that has given people suffering with IPF new hope. Two new studies have been carried out in the USA and the initial results seem extremely positive. The two drugs tested in the studies were nintedanib and pirfenidone. Patients that took Nintedanib for one year showed a 50% reduction in declining lung function and those patients who took Pirfenidone for a year showed a 48% reduction in disease progression.

## Exercise Recommendations

For people suffering from Idiopathic Pulmonary Fibrosis exercising can be extremely difficult due to the lungs reduced ability to diffuse air in and out of the blood stream. As the scar tissue in the lungs increases, the amount of functional lung reduces which makes breathing very difficult.
This being said, people suffering from pulmonary fibrosis should continue to exercise. It may be difficult because the lungs are already under considerable stress and increasing the stress doesn't seem to make much sense, but inactivity will do more harm than good. By continuing to exercise, IPF patients will cause their lungs to adapt to working under more severe stresses. This is beneficial because it will help patients deal with day to day activities. Their lungs will become used to performing under the excess stresses and simple tasks will being to not cause such severe shortness of breath, therefore they will be able to perform tasks more simply.
Taking this information into account, it is suggested that IPF patients should participate in what ever level of exercise they are able to perform due to the fact that maintaining and continuing exercising will help improve their quality of life.

## Further Information

* The Lung Foundation
* Pulmonary Fibrosis Information
* The American Lung Foundation
* Healthline

## Reference List

* 1 2 3 Zisman. D, 2011, Idiopathic Pulmonary Fibrosis Pathogenesis and Theraputic Approaches, Adis Data Information, 408-412pg
* ↑ Mapel DW, Samet JM, Coultas DB., 1996, Corticosteroids and treatment of idiopathic pulmonary fibrosis: past, present, and future. Chest, 110 (4): 1058-6
* 1 2 Luckhardt, 2011, New Insights into the Pathogenesis and Treatment of Idiopathic Pulmonary Fibrosis, Adis Data Information, Volume 8, 987-992
* ↑ Iwai K, Mori T, Yamada N, et al. 1994, Idiopathic pulmonary fibrosis: epidemiologic approaches to occupational exposure. Am J 42. 670-5
* 1 2 3 Gibbons. G, 2011, What Is Idiopathic Pulmonary Fibrosis?, National Heart, Lung and Blood Institute.
* ↑ Tzanakis N, Samiou M, Lambiri I, et al. 2005, Evaluation of health- related quality-of-life and dyspnea scales in patients with idiopathic pulmonary fibrosis: correlation with pulmonary function tests. Eur J Intern Med; 16 (2): 105-1
* ↑ Flaherty KR, Martinez FJ., 2000, The role of pulmonary function lular constituents and their relationships with lung histopathol- testing in pulmonary fibrosis. Curr Opin Pulm Med; 6 (5): 404-10