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A special kind of bravery

This past Friday the Food and Drug Administration (FDA) hosted a public meeting to discuss the needs of patients with Idiopathic Pulmonary Fibrosis. I have a lot to say about the meeting, but I want to begin by saying that it was one of the toughest medical meetings I’ve ever been a part of. My hat is off to all of those with pulmonary fibrosis, their families and the advocates, who took part on behalf of the thousands more who could not. It was a meeting full of tears and sorrow and frustration. I am told that even one of the doctors from the FDA got teary. While I don’t want to make people cry, hearing that really moved me. It makes you feel like you were heard.

Now, onto the meeting.

The Hermansky-Pudlak Syndrome community has a long way to go in our work with the FDA. The past 22 years (long before I was ever involved) has been a journey of learning new things – medicine, politics, non-profit law etc. Until now, we haven’t had much to do with the FDA because our research hadn’t progressed to the point of having a possible treatment. The NIH has handled the issues involved with the FDA and setting up our drug trials. Now, we are entering a new age, and it is time to tackle understanding the ins and outs of the FDA world.

You’ll notice that the FDA chose to have a public meeting about Idiopathic Pulmonary Fibrosis and not pulmonary fibrosis. I noted that they also had a public meeting about bleeding disorders, and not Hemophilia or Von Wilibrands. Why then is it necessary to only include those with IPF in the pulmonary fibrosis public meeting? By its very name, it excludes people like us. It is the use of an old and increasingly outdated paradigm. As treatments become available there will be some that work better for some patients, and others that work better for other patients.

Take the world of cancer treatments, for example. Years and years ago there were treatments for cancer. Now, they can choose a drug they believe will best work for an individual given the specific type of cancer they have, and even their genetic profile. Someday it could be the same for pulmonary fibrosis. By defining the problem by using the word idiopathic as if the unknown was a disease and not simply a lack of knowledge, the FDA is risking impeding access to life saving treatments to people like us – and someday others. After all, the Sept. 11th responders that have developed pulmonary fibrosis, or the families that know PF runs in their family tree – are they really idiopathic? (Even though most have the IPF diagnosis.)

I think it is a matter of education. The pulmonary fibrosis community in general is only barely understanding and embracing this idea. The FDA, which is a recent player to our world, is really only going off what they’ve been told from the pulmonary fibrosis world. It is a matter of some controversy and debate and will likely require a great deal of advocacy work on our part. That’s okay. Now is the time to make that happen, and this public meeting was the first baby step in what will be an ongoing process for us.

I was so moved by the representatives that spoke on behalf of pulmonary fibrosis patients. I know Donna was very upset that I was listening to this testimony. She was, and rightly so, worried about how upsetting it would be for me given the decline in my health. Family of those who have lost loved ones to pulmonary fibrosis offered extremely graphic descriptions of their relatives’ last days. Others talked about how much pulmonary fibrosis has changed their lives and what a devastating effect it has had, not only on them, but on their entire families. Of course it wasn’t easy to hear. On the other hand, in some ways, it was vindication for some of the things I am starting to notice about my own health.

As I start to notice things that are harder to do now than they used to be, I find I often have a tremendous amount of guilt. I ask myself, is it really my health, or is it in my head? Is there a reason, or am I just feeling lazy? It was affirming to hear others in pretty much the same state of lung disease talk about the same issues and problems.

I want to blog about some of my own answers to the FDA’s questions, but that will have to wait for another time.

The one thought I couldn’t shake through the trauma of the day is this – Imagine having all of these same problems (coupled with legal blindness) and then, when after years and years there is finally a treatment, being left behind?


For now, I just want to thank the FDA for having the public meeting and listening to patients. I want to urge the FDA to make sure that patients with HPS are not left behind suffering and dying when there is a treatment that could help. Lastly, I want to thank Donna and all the other brave advocates who stood up, shared their very souls, and gave us all a voice!

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