Fibrosis File

Merry Christmas! Thanks to input from people dealing with the disease, the Food and Drug Administration (FDA) has approved two new drugs, Nintedanib (tradename Ofev® from Boehringer Ingelheim-BI) and Pirfenidone (tradename Esbriet® from InterMune), to help Idiopathic Pulmonary Fibrosis (IPF). It is the first time the FDA has approved any treatment for IPF.

image080The pharmaceutical companies have been doing clinical trials in IPF for about 15 years. Most of these trials have been neg- ative and no potential medication has ever been approved by the FDA. Earlier this year, successful studies on the two drugs were published in the New England Journal of Medicine which helped convince the FDA to approve these medications. The drugs hope to lower the rate of lung function decline, as well as decrease acute exacerba- tions (flare-ups).

The drugs are used separately. It is un- known how the two drugs might interact with one another. Both have possible GI side effects as nausea and vomiting. Studies combining the drugs are sure to be in the future as a possible treatment for those with IPF.

We don’t know whether one is better than the other. The recommended dose for Pirfenidone is three capsules three times a day, whereas Nintedanib, it is one capsule twice a day. Almost two thirds of peo ple who took Nintedanib had diarrhea. That might sway the physician to go with Pirfenidone instead of Nintedanib.

You probably won’t notice a big differ- ence in your condition when you start these drugs. What they have been proven to do is to reduce the rate of deterioration in your lung which is difficult to predict and differs in each person.

Hopefully one day we can look back and see people survived longer and performed better after taking these medicines.

This is a long overdue good start in treat- ment options for IPF!

Unfortunately, the medication is very expensive – without insurance it would cost $92,000 per year! We have heard of people getting Pirfenidone (Esbriet) for $25/month after approval. If you and your doctor have decided this is the drug that is right for you, you may be eligible for the Esbriet Start Now program to get your first 45 days of therapy at no cost. Visit careconnect/patients on the Internet or

call 1-844-372-7438 for more information. Our Canadian friends have been paying approximately $150 for a month’s supply of 90 pills. Esbriet has been available in Canada since 2012.

Boehringer Ingelheim-BI has a support program called OpenDoor that you can find out more about financial help for Ofev at 1-866-673-6366 or patient-support.php

The Pulmonary Fibrosis Foundation has a Patients Support Community where mem- bers have been discussing gifts for the person who has IPF. You might want to leave this list someplace where the right people can see it!

  • I would relish “homemade” coupons to run an errand, pack and take a package to the post office or even help decorate or put away decorations, yard clean- up or gardening. Maybe even doing laundry, changing sheets on the bed, or putting away clean clothes. I find the gift of someone’s time is invaluable and precious.
  • A gift card to the grocery or pharmacy would be most For my friends with pets, I buy three or six months worth of their pet’s routine meds (like Trifexis), or put a credit on their account with their vet.
  • Most people with PF agree that we feel colder than most. Soft, warm socks, lounge pants or a cuddly warm throw is a good non-food Gloves or mittens that can convert to fingerless would be nice. A selection of teas and hot choco- late packets are handy and useful.
  • Something homemade is always good.
  • Gift cards to restaurants, especially restaurants that deliver.
  • The best gift given to me in this past year has been my my iPad.
  • A prepaid gift of housecleaning. My helper uses only vinegar to clean as most household chemicals really bother me.
  • I watch a lot of movies, documentaries and read iBooks. You can’t go wrong with prepaid gift cards for iTunes.
  • image081-2I would adore it if someone sent a mas- sage therapist to my I don’t go out for massages because I’m so exhausted getting there and getting back that it spoils the whole experience.