NIH needs HPS’ers to share “liquid gold”

People with HPS types 1 and 4, over the age of 18, are needed to participate in a research study at the National Institutes of Health investigating the lung disease of Hermansky-Pudlak Syndrome. Volunteers will be asked to undergo a lung lavage to collect cells from the lung.

A lung lavage involves inserting a tube with a tiny camera on the end into the upper lung, and then squirting saline fluid into the lungs to cause the volunteer to cough. The coughing causes the lungs to sluff off cells which are then suctioned up as fluid. (The HPS community has nick named the fluid “liquid gold” because of its value to research on the pulmonary fibrosis of HPS).

Patients are technically awake for the procedure, but are given drugs so that they have no memory or awareness of the procedure.

Some medical conditions may prohibit one from volunteering.

Those who have already undergone lung lavages can re-volunteer every six months.

If you would like us to add your name to a list of possible volunteers, please e-mail Heather Kirkwood at: hkdawn@yahoo.com.

Comments

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...

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...

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.

Living on the frontlines

Search This Blog