Skip to main content

Hermansky-Pudlak Syndrome, pulmonary fibrosis and Pirfenidone – 16-month follow up

I can’t believe that in just a few days I’ll be back at NIH for my 16-month follow-up visit in the clinical trial for pirfenidone to treat the pulmonary fibrosis of HPS. I’m happy to report I think this will be a very boring trip. When it comes to HPS and pulmonary fibrosis, I’m quite happy to be a very boring patient.

Usually I start to get somewhat anxious a few weeks before time to go. The closer the trip gets, the more fixated I become on it. I start analyzing my breathing and probably become a huge hypochondriac. It’s a coping mechanism to steel myself against any bad news.

This time, however, I’m more nervous that I might be jinxing the process. I feel great. My breathing has been terrific. Even my digestive issues have been much improved since about mid October. I’m really showing up with a few minor complaints – the skin infection that won’t go away, the sore by my stoma that hasn’t completely healed in a year, and the need to have a brief chat with Dr. Merideth about our future plans.

This is going to be a very quick trip – just three days.

I feel so blessed to be a part of this trial. Of course, I don’t know if I’m getting the real pills or the placebo, but I do know that my lung function has improved since being on the medication.
Labels:

Comments

Post a Comment

Popular posts from this blog

Ratner's Cheesecake

Here's another recipe from Toby! Thanks Toby......and I'll get the others posted soon! Ratner's Cheesecake and plain cookies Ratner's was a Jewish dairy restaurant in the lower East Side of Manhattan. This recipe, from my disintegrating, no longer in print Ratner's cookbook, is the closest I've ever gotten to reproducing the rich, heavy cheesecake my mother made when I was a kid. It's worth the time it takes to prepare and every last calorie. Dough Can be prepared in advance. Makes enough for two cakes. Can be frozen or used to make cookies – see recipe below. 1 cup sugar 1 teaspoon vanilla extract 1 cup shortening 1 teaspoon lemon extract 1 cup butter 2 eggs 3 cups sifted cake flour ½ teaspoon salt 2 cups all purpose flour 1 teaspoon baking powder 1. In a bowl, combine all ingredients with hands. Refrigerate 3 -4 hours, or preferably overnight. Filling (for...
1 comment
Read more

Some good news about Pirfenidone

Below is a press release from Intermune, the company that makes Pirfenidone. They have essentially reviewed the various clinical trials going on, and decided that Pirfenidone is safe and well tolerated. That would pretty much go along with what we've observed in the HPS community as well. We have a few folks that have been on the drug since the late 90s and continue to do well. Of course, as a journalist, I do have to say consider the source - but at the same time, as someone in a Pirfenidone trial, it's good to know. Results of Comprehensive Safety Analysis of pirfenidone In IPF Patients Presented At European Respiratory Meeting - Analysis shows safety and tolerability of pirfenidone across four clinical trials - VIENNA, Sept. 14 /PRNewswire-FirstCall/ -- InterMune, Inc. (Nasdaq: ITMN ) today announced that the results of a comprehensive review of safety data from four clinical studies were presented at the 2009 European Respiratory Society Annual Congress in Vienna, Austria...
Post a Comment
Read more

The next generation with Hermansky-Pudlak Syndrome

I'm so behind on posting about the trip to Puerto Rico. Since the episode of Mystery Diagnosis on Hermansky-Pudlak Syndrome ran right after we got home, it's been a little busy. These, however, are my favorite pictures from Puerto Rico. I know, not pretty senery etc - but these little guys and gals inspire me. They are the next generation of folks with HPS, and if we keep up the hard work, they will live better lives because of it. They motivate me.
Post a Comment
Read more