Newly FDA-Approved Drugs Give Hope to People with Pulmonary Fibrosis

Lung disease was considered untreatable until now

Pulmonary Tuberculosis

Those who suffer from idiopathic pulmonary fibrosis (IPF) may be breathing a little easier. In October 2014, the Food and Drug Administration approved two new drugs for IPF — the first ever produced that can treat this disease.

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IPF is a scarring or thickening of the lungs that has no known cause. As the scarring worsens, it becomes more and more difficult for the person with the disease to breathe. The new drugs offer hope to people who are suffering from IPF, a disease which has thus far been universally fatal.

“This is an exciting time in fibrosis treatment,” says pulmonologist Leslie Tolle, MD. “Prior to approval of these new drugs, we had no treatment options for IPF. Now we have two.”

How do you know if you have IPF?

IPF is a serious disease that usually affects middle-aged and older adults (most between the ages of 50 and 70). About 100,000 people are currently affected in the United States. Doctors diagnose an estimated 30,000 to 40,000 new cases each year.

The progression of the disease varies from person to person. In some people, it gets worse quickly. In others, it develops over a much longer period of time.

There are a lot of illnesses that can cause difficulty breathing, so shortness of breath alone may not mean you have IPF. It’s time to call your doctor if you experience shortness of breath and any of the following:

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  • Breathing that is harder, faster, or shallower (you are unable to take a deep breath)
  • Having to lean forward when sitting to breathe comfortably
  • Frequent headaches
  • Sleepiness or confusion
  • Fever
  • Dark mucus when you cough
  • Blue fingertips or skin around your fingernails

What the drugs do for IPF

The two new drugs, Ofev® and Esbriet®, are not a cure for IPF. However, they do slow down the disease’s progression.

“IPF is so difficult to treat because it’s a scarring-related condition and scarring is permanent and very difficult to reverse,” says Dr. Tolle. “Both new drugs are designed to prevent further scarring, rather than to reverse the scarring that’s already there.”

According to Dr. Tolle, anyone who has been diagnosed with IPF should be evaluated in a specialty center that is comfortable prescribing these new drugs.

“Without these medications, IPF is a death sentence,” he says. “But with them, that may no longer be the case.”

Is one drug better than the other?

At this point, doctors don’t yet know if one drug does a better job of slowing the progression of IPF.

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“The two drugs haven’t been studied head-to-head,” Dr. Tolle says. “At this point, decisions on which drug to prescribe are being based on the patient’s other medical conditions, their previous history of side effects to other medications, and the patient’s preferred dosing regimen.”

As of now, it is not known whether or not there are added benefits to taking the two drugs together.

“Both drugs are very expensive, so I doubt the insurance companies will pay for both without knowing whether or not it would be beneficial,” Dr. Tolle says. “More research still needs to be done.”

Cleveland Clinic is currently conducting a number of clinical trials to test other medications for their effectiveness in treating IPF.

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