Treatment Options for Idiopathic Pulmonary Fibrosis

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Idiopathic pulmonary fibrosis (IPF) is not curable, but it is treatable. Thankfully, new medications have been approved just since 2014 which are making a difference in symptoms, the quality of life, and progression for people living with the disease. In contrast, medications used until very recently have been deemed to cause more harm than good for some people with IPF. If you've been diagnosed with this disease, make sure you don't get discouraged by older information.

Goals of Treatment for Idiopathic Pulmonary Fibrosis

The damage that has occurred in IPF is by definition irreversible; the fibrosis (scarring) that has occurred cannot be healed. Therefore, the goals of treatment are to:

  • Minimize further damage to the lungs. Since the underlying cause of IPF is damage followed by abnormal healing, treatment is directed at these mechanisms.
  • Improve breathing difficulty.
  • Maximize activity and quality of life.

Since IPF is an uncommon disease, it is helpful if patients can seek out care at a medical center which specializes in idiopathic pulmonary fibrosis and interstitial lung disease. A specialist likely has the most recent knowledge of available treatment options and can help you pick one that's best for your individual case.

Medications for Idiopathic Pulmonary Fibrosis

Tyrosine Kinase Inhibitors

In October of 2014, two medications became the first drugs approved by the FDA specifically for the treatment of idiopathic pulmonary fibrosis.

These medications, pirfenidone and nintedanib, target enzymes called tyrosine kinase and work by reducing fibrosis (antifibrotics).

Very simplistically, tyrosine kinase enzymes activate the growth factors that cause fibrosis, therefore these medications block the enzymes and hence the growth factors that would cause further fibrosis.

These medications were found to have several benefits:

  • They reduced disease progression by half during the year patients took the drug (it is now being studied over longer periods of time).
  • They decrease the functional decline in lung function (the decrease in FVC is less) by half.
  • They result in fewer exacerbations of the disease.
  • Patients using these drugs had better health-related quality of life.

These medications are generally tolerated quite well, which is very important for a progressive disease without a cure; the most common symptom being diarrhea.

N-Acetylcysteine

In the past n-acetylcysteine was often used to treat IPF, but more recent studies have not found this to be effective. When broken down it appears that people with some gene types may improve on the medication, while those with another gene type (another allele) are actually harmed by the drug.

Proton Pump Inhibitors

Of interest is one study done using esomeprazole, a proton pump inhibitor, on lung cells in the lab and in rats. This medication, commonly used to treat gastroesophageal reflux disease, resulted in increased survival in both the lung cells and in rats. Since GERD is a common precursor to IPF, it's thought that acid from the stomach aspirated into the lungs may be part of the etiology of IPF.

 While this has yet to be tested in humans, certainly treatment of chronic GERD in individuals with IPF should be considered.

Lung Transplant Surgery

The use of a bilateral or single lung transplant as a treatment for IPF has been steadily increasing over the last 15 years and represents the largest group of people waiting for lung transplants in the United States. It carries significant risk but is the only treatment known at this time to clearly extend life expectancy.

Currently, the median survival (time after which half of people have died and half are still alive) is 4.5 years with a transplant, though it’s likely that survival has improved during that time while medicine becomes more advanced.

The survival rate is higher for bilateral transplants than a single lung transplant, but it’s thought that this has more to do with factors other than the transplant, like the characteristics of the people who had one or two transplanted lungs.

Supportive Treatment

Since IPF is a progressive disease, supportive treatment to ensure the best quality of life possible is extremely important. Some of these measures include:

  • Management of concurrent problems.
  • Treatment of symptoms.
  • Flu shot and pneumonia shot to help prevent infections.
  • Pulmonary rehabilitation.
  • Oxygen therapy - Some people are hesitant to use oxygen due to the stigma, but it can be very helpful for some people with IPF. Certainly, it makes breathing easier and allows people with the disease to have a better quality of life, but it also reduces complications related to low oxygen in the blood and reduces pulmonary hypertension (high blood pressure in the arteries that travel between the right side of the heart and the lungs).

Co-Existing Conditions and Complications

Several complications are common in people living with IPF. These include:

  • Sleep apnea
  • Gastroesophageal reflux disease (GERD)
  • Pulmonary hypertension – High blood pressure in the arteries of the lungs makes it harder to push blood through the blood vessels going through the lungs, so the right side of the heart (right and left ventricle) has to work hard.
  • Depression
  • Lung cancer – Roughly 10 percent of people with IPF develop lung cancer

Once you're diagnosed with IPF, you should discuss the possibility of these complications with your doctor and devise a plan on how you can best manage or even prevent them.

Online Support Groups and Communities

There is nothing like talking to another person who is coping with a disease like you. Yet, since IPF is uncommon, there’s likely not a support group in your community. If you are receiving treatment at a facility that specializes in IPF, there may be in-person support groups available through your medical center.

For those who do not have a support group such as this—which probably means most people with IPF—online support groups and communities are a great option. In addition, these are communities that you can seek out seven days a week, 24 hours a day when you really need to touch base with someone.

Support groups are helpful in lending emotional support for many people and are also a way to keep up on the latest findings and treatments for the disease. Examples of ones you can join include:

A Word From Verywell

The prognosis of idiopathic pulmonary fibrosis varies widely, with some people having rapidly progressive disease, and others who remain stable for many years. It’s hard to predict what the course will be with any one patient. The median survival rate was 3.3 years in 2007 versus 3.8 years in 2011. Another study found that people aged 65 and over were living longer with IPF in 2011 than they were in 2001.

Even without the newly approved medications, care appears to be improving. Don't rely on older information you find, which is likely out of date. Speak with your doctor about the options outlined here and which is best for you.

Sources:

Antoniou, K., Tomassetti, S., Tsitoura, E., and C. Vancheri. Idiopathic pulmonary fibrosis and lung cancer: a clinical and pathogenesis update. Current Opinions in Pulmonary Medicine. 2015 Sep 18. (Epub ahead of print).

de Boer, K., and J. Lee. Under-recognised comorbidities in idiopathic pulmonary fibrosis: a review. Respirology. 2015 Sep 13. (Epub ahead of print).

O’Riordan, T., Smith, V., and G. Raghu. Development of Novel Agents for Idiopathic Pulmonary Fibrosis: progress in target selection and clinical trial design. Chest. 2015 May 8. (Epub ahead of print).

Raghu, G. et al. Diagnosis of idiopathic pulmonary fibrosis with high-resolution CT in patients with little or no radiological evidence of honeycombing: secondary analysis of a randomized, controlled trial. Lancet Respiratory Medicine. 2014. 2(4):277-84.

Spagnolo, P., Maher, T., and L. Richeldi. Idiopathic pulmonary fibrosis: Recent advances on pharmacological therapy. Pharmacology & Therapeutics. 2015. 152:18-27.

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