Several months ago I wrote about a tennis partner who was slowly dying of idiopathic pulmonary fibrosis (IPF). We both knew there was no cure for this disease. Nor could I foresee any help for many years. I was wrong on that point, but I was right about my message of hope.
I always tell seriously ill patients the story of the man sentenced to death by the king. Because the king was a lover of horses, the man received a reprieve by promising he'd teach the king's horse to fly within one year. His friends laughed at him, but he explained, "Within a year the king may die, I may die or the horse may die. Besides, within a year the horse may learn to fly." The last thing to lose is hope.
Patients with IPF face a grim future. The average length of life from the time of diagnosis of IPF is usually two to five years. Fortunately, fate is occasionally kind, as some patients have lived as long as 20 years.
In some cases, the course of the disease remains fairly stable. This was the case of my tennis friend. But then it became apparent that the disease was beginning to progress. During tennis games he developed a dry, hacking cough, chest discomfort, but even more ominous, progressive shortness of breath along with weakness and fatigue.
IPF is associated with extensive, progressive scarring of lung tissue. The alveoli (air sacs) in lungs stiffen and become thick, decreasing the amount of oxygen in the bloodstream. In effect, IPF is a slow strangulation of the body's vital oxygen supply.
During final days, any physical activity becomes an extreme effort. As my friend remarked, "It requires hours to do anything. It's like being trapped underwater and trying to surface for air." It's easy to understand how doctors treating these cases feel frustrated, as there's little to offer in treatment.
Sir William Osler, one of this country's greatest physicians, felt this way when a colleague asked how he treated another difficult problem, emphysema. Osler replied, "I send these patients to Egypt." His startled colleague asked, "Is it for the climate?"
"No," Osler replied, "it's the farthest place I can think of to send them." It was his way of saying he had no treatment to offer them.
I had no idea when I wrote about IPF several months ago that in this short interval I'd have good news for some IPF patients. A new medication called Esbriet (pirfenidone) is now available in Germany, France, Sweden, Austria, Denmark, Norway, Iceland, 27 countries in the European Union and has just been approved by Health Canada.
Dr. Charles Chan, professor of medicine and a respirologist at the University of Toronto, once shared Osler's frustration, but now he's says, "After a decade of failed clinical trials of multiple innovative drugs for IPF, Esbriet is an exciting one for IPF patients. Finally we have a treatment option with proven benefit for patients with this challenging disease."
Esbriet is designed to fight a two-pronged attack on the underlying problems of idiopathic pulmonary fibrosis, namely the inflammation and tough scarring that eventually choke and kill lung tissue.
Esbriet accomplishes this task by decreasing the formation of cells called fibroblasts that are responsible for triggering scarring and fibrosis of air sacs in the lungs. In addition, Esbriet has an anti-inflammatory effect which helps to slow down the progression of the disease and thereby preserves exercise tolerance.
The usual dose is one oral tablet three times a day. Fortunately, Esbriet is well tolerated by most patients. A few complain of nausea, fatigue, diarrhea and heartburn.
Even if Esbriet had been available several months ago, it would not have helped my friend, as his IPF was too advanced at that time, but Esbriet can help decrease the progression of those who suffer from the mild to moderate form of IPF. This is a huge step in the right direction to fight this challenging disease.
Osler would be pleased to know Egypt need no longer be an option.
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