Tag Archives: esbriet

29Oct/14

Mom’s Story: No More Cliff Diving, Part Four

Note: While I am posting this on Mom’s behalf, she wrote it. No one can tell her story as well as she can.

The future for me is to hopefully get on one of the only drugs that has shown to slow the progression of this disease. It has been used in other countries for several years, but is still not approved here. The FDA has said it will be, but they have said that for two years.** It’s only being used here in studies for “compassionate use”. If you go to Canada, it is several thousand dollars a month. There are other ways to get the medication that fall into sort of a gray area for the FDA, but that’s all I’m going to say about it. I’m willing to do anything I can to slow the progression of my disease.There are also some supplements many doctors and clinics suggest may help. I am using three of them which my insurance does not cover.

There is so much more I could talk about, but now I want to say something about family and friends. NO ONE gets this far or continues on without both. My daughter set up the GoFundMe site for donations. Friends and family have been so generous and I thank you all!

 

I have to thank my husband of forty years for always having my back and this long journey has been no different. He is still impatient with hospitals and sitting around as most men seem to be, but I can’t fault him for that one bit. He really is my friend, the love of my life and super dependable. Everyone who meets him loves him and he is a fantastic father. I think sometimes my family would keep him over me. Thanks honey for a wonderful life so far and I know you think the words to a Cat Stevens song was written for us, “I’m lookin’ for a hard headed woman, one who’ll make me do my best….”  Well, you know the rest.

My fantastic adult kids and their spouses, you have always made me proud and never let me down. You are kind and generous and loving and smart and resilient. The moments you hate the way the world works is almost always because an underdog is being treated so unfairly, or because stupidity has reared its ugly head again. So since song lyrics keep appearing I will just say, “You are my sunshine, my only sunshine…” keep singing you know it goes.

Mom and dad, you are the best parents on the planet. Words will never express how I feel. Mom, I am doing that thing people do where you take two fingers to my eyes the point to your eyes. I call it the “Eye Connection” and you know what I mean. We do not need words. We just get each other, even if it hurts sometimes.

Sisters, I am stuck in the middle with you… isn’t that a song too? When it counts the most we lock arms and nobody gets by us. I don’t need to say anymore because sister language is only understood by sisters and we always have something to say.

Friends and what I call my other kids, this is for you. I thank you all for so many memories and all the ones still to be. I have made it a point in my life to befriend only kind people and hope only kind people would befriend me. I like that we are different, yet the same and that what I just said you understand. We are all just a bit crazy.

Hey BFF (and you know who you are) fact of life: After Monday and Tuesday even the calendar says WTF, and we have said that a lot.

One final thought, as I carry on with an uncertain future, knowing this disease will probably be my fate since now I can no longer cliff dive. Winston Churchill once said, “I am ready to meet my maker. Whether my maker is prepared for the ordeal of meeting me is another matter.”

 

**Esbriet/Pirfenidone and another drug for the treatment of Pulmonary Fibrosis were approved by the FDA on October 15, 2014 after this was written but prior to it being published.

20Oct/14

The Cost of Dying

I had planned to publish the final installation of No More Cliff Diving (part two, part three) last week, but in light of the recent announcement of the FDA’s approval of Esbriet and Ofev I wanted to devote an entry to that. And, I needed a few days to stew contemplate.

On October 15, 2014 the FDA announced it was approving the use of Esbriet/ pirfenidone and Ofev/ nintedanib for the treatment of Idiopathic Pulmonary Fibrosis in the United States. Don’t get me wrong, I applaud the FDA for finally approving not one, but two drugs for the treatment of this devastating and terminal illness. However, I can’t help but think about all the patients for whom this news is too little too late. About all the patients that died waiting for something like pirfenidone in the U.S.

While I publicly retweeted the news from Pulmonary Fibrosis Foundation’s twitter feed, privately I scoffed at their generous use of “prompt” in relation to how long the approval took.

According to a press release on Drugs.com, Intermune first submitted a New Drug Application to the FDA on November 4, 2009. I completely understand the need for due diligence, particularly in respect to prescription drugs, but pirfenindone is not a new drug.

It has been in use since 2008 in Japan under the trade name Pirespa, and in India as Pirfenex, since 2010.  I could go on.

According to the Coalition for Pulmonary Fibrosis, approximately 40,000 people die from pulmonary fibrosis every year. So… that’s about 160,000 people dead in the four complete years since the initial drug application to the FDA. Sure, I’m biased; I’m emotionally invested. But I doubt the families of those 160,000 people (remember I’m not counting 2009 or 2014 so the estimate is on the low side)  think the FDA’s approval was all that prompt either.

An article in the NY Times published the same day as the approval stated Esbriet would cost roughly $94,000 a year.

“Roche said the wholesale price of Esbriet would be about $7,800 a month, or about $94,000 a year — two to three times what the drug sells for in Canada and Europe.”

And that’s down from one account I read (I’ll link to it, if I can find it again) that estimated the cost to be closer to $100,000. Either way the exorbitant cost will likely prevent patients, many of whom have been disabled by this disease, from receiving the drugs that can slow the progression of their disease.

The Pulmonary Fibrosis Foundation has alluded to a possible subsidy in some of their tweets, but I haven’t been able to find any concrete information on it.