The lungs are remarkable organs made of spongy tissue that supply oxygen, the life-sustaining gas needed by the body. As the only internal organs that are exposed to the external environment, they are vulnerable to a variety of ailments. Some, like asthma, bronchitis, or even certain cancers, can be cured. However, when it comes to idiopathic pulmonary fibrosis, or IPF, eventual death is a certainty unless the lungs are replaced.
Idiopathic pulmonary fibrosis is a progressive disease isolated to the lung that causes scarring, thickening, and damage to the tissue around and between the tiny air sacs in the lungs. This scarring makes it extremely difficult for oxygen to pass through into the bloodstream and into the brain and other organs. Shortness of breath, especially during physical activity, is common with IPF, as is extreme fatigue.
The cause of this chronic lung ailment is unknown, which is why it’s called idiopathic. Some patients inherit it, while for others it may be caused by exposure to certain types of silica dust, asbestos fibers, grain dust, bird and animal droppings, or cigarette smoke. Some people develop IPF after receiving radiation therapy for lung or breast cancer, while others develop the chronic lung damage after chemotherapy treatment, from heart medications for irregular heartbeats, or after taking certain antibiotics.
The age of diagnosis for IPF is usually between 40 and 70, but about two-thirds of patients are older than 60. Currently more than 80,000 American adults have idiopathic pulmonary fibrosis, and more than 30,000 new cases are diagnosed annually.
This disabling ailment has no treatment that can remove the fibrotic scarring within the lungs, slow the disease process, or cure it. Life expectancy after diagnosis is usually three to five years, with patients progressively getting worse, most often because of respiratory failure.
There is now new hope for patients with IPF. Two new drugs have been proven in large international studies to significantly slow the progress of IPF.
The first oral drug, pirfenidone, slowed the disease progress after just 13 weeks of treatment in the one-year study compared to placebo therapy. The drug also enhanced lung function significantly, and improved the distance that a patient could walk. Overall, the drug reduced the risk of mortality by 48 percent compared to placebo.
While researchers aren’t sure how pirfenidone works against IPF, the drug appears to have anti-inflammatory properties and it also inhibits a growth factor protein in the development of fibrosis. The drug is already approved for use in Europe, Japan, and Canada, and the FDA just approved its use in the U.S.
In two Phase III studies of more than 1,000 patients, the second drug, nintedanib, reduced patients’ annual rate of lung function decline by 48 percent and 55 percent, compared to 5 percent for those taking placebo drug.
Nintedanib works by blocking the effect of tyrosine kinases, important proteins that alert the lungs to make scar tissue. Thanks to the FDA’s breakthrough therapy designation that guarantees speedy priority review, this drug was also recently approved for use in the United States.
Two new drugs in one year. People with IPF will finally be able to breathe easier.
Where Are They Now
Both medications were FDA approved in October of 2014, months ahead of schedule. Recently, the American Thoracic Society gave important recommendations for this new class of drugs for use as treatment for IPF with confidence. Until 2017, the mechanism of the two FDA-approved drugs was not entirely known. Researchers have recently identified how the drugs slow the progression of the disease, and hope to use this information to study methods to halt pulmonary fibrosis.