Patients with progressive lung scarring hold onto hope

A man sentenced to death by the king was granted a reprieve of one year. He discovered the king was a lover of horses and promised within a year he would teach the king's horse to fly. His friends laughed at him. But the man explained, "Within a year the king may die, or the horse may die, or I may die. Besides, who knows? The king's horse may learn to fly." I recently told this story of hope to a friend who was becoming increasingly incapacitated by idiopathic pulmonary fibrosis, a disease with no known cure.

IPF is the most common interstitial lung disease (ILD), a group of diseases affecting the tissue and space around the air sacs in the lungs. These diseases are more rare than asthma, chronic obstructive lung disease or even lung cancer, but they are severe and very life-limiting. Yet most people and some doctors have never heard of this problem.

I never suspected my tennis partner had IPF. But in recent years it became apparent something was wrong. He developed a dry hacking cough, chest discomfort, progressive shortness of breath, fatigue and weakness.

This happens because IPF causes extensive, progressive scarring of lung tissue. The alveoli (air sacs) in the lungs stiffen and become thick, decreasing the amount of oxygen in the bloodstream. In effect, IPF is a slow strangulation of the body's oxygen supply, essential for life. My friend remarked, "It takes hours to do anything. It's like being trapped under water and trying to surface for air."

As the disease progresses and the amount of functioning lung tissue diminishes, other problems make life increasingly difficult. Pressure may build up in pulmonary arteries putting strain on the heart, which then begins to fail.

In many cases doctors cannot pinpoint a cause for ILD. But in others the cause is obvious. When miners inhale coal dust or asbestos, for instance. Others develop scarring of the lung by working long hours sandblasting or bird farming. There is also a condition called hypersensitivity pneumonitis often caused by inhaling dust contaminated with bacterial, fungal or animal products.

Smoking makes the illness worse. In addition, some connective tissue diseases such as rheumatoid arthritis, scleroderma, sarcoidosis and radiation therapy to the chest cause lung scarring. As readers know, I have repeatedly warned about the dangers of excessive and often needless radiation therapy.

The diagnosis of pulmonary fibrosis is made by lung doctors using lung function tests, high-resolution CAT scans of the chest and/or a lung biopsy. A pathologist can confirm the presence of scarring.

Unfortunately there are no approved treatments for IPF except for double lung transplant. In the past, doctors used a combination of prednisone and azathioprine with an antioxidant to treat IPF. Recently, however this approach was found to be much more harmful than placebo and is no longer recommended. That's why it's so important for patients to take charge of their own lives. This means trying to avoid infection and making sure they are vaccinated against pneumonia.

Knowing you have an incurable disease with just a few years to live is never easy to accept. Patients desperately hope the diagnosis is wrong and often frantically search the Internet looking for a cure. And the more they delve into IPF the more confused they become, particularly when no apparent cause for the scarring can be found.

The average length of life from the time of IPF diagnosis is two to five years. But the course of the disease varies from patient to patient. For some its course is rapid and for others its a slow gradual one. Fortunately, fate can be kind as some patients have lived as long as 20 years.

What about the future? Recently the Canadian Pulmonary Fibrosis Foundation, along with a team of researchers at the University of Toronto, began exploring the use of new anti-scarring therapies in fighting this disease. There is always hope. Those inflicted with IPF may live to see the king's horse has learned to fly.

Republished from the Winnipeg Free Press print edition June 22, 2012 A21