Skip to main content

TGIF!

Thank God It’s Friday! (Well, actually it’s Saturday, but I fell asleep before I got this posted.) I don’t think I’ve been so happy to see a Friday in a long time – and I look forward to Fridays all week long.

This was just a rough week. All week I never did hear from my doctor about the pulmonary function test. I have no idea if anyone has sent the final report to Kevin, or what Dr. Kennedy even thinks of the results. When I called for the third time, I was told that it takes ten days to process test results – and not to worry, if it was an emergency the test results are reviewed daily and someone would have called. The pulmonary lab told me my doctor would have the report by late Monday. That was five days ago.

The only way a PFT would be an emergency would be if I were upstairs in the hospital on a vent or something. It isn’t that kind of test. But, I would have thought that if someone had noticed my FVC had fallen from 94 to 69, they might have wanted to have a conversation with me about it. I said on the messages that I left that I was trying to work this out with NIH etc. At the very least I’d like to know if anyone faxed the report to Kevin.

I love my doctor, but the support staff sometimes drives me nuts. You go to all this effort to educate your doctor about your rare disease, and then some gatekeeper, who doesn’t get the whole HPS or NIH thing, is probably sitting on this report somewhere. This drives me nuts. It’s like when you’ve got a cold, and you try to get a same-day appointment, or at least the following day – but my doctor is never available. So, I see someone else and it’s like starting all over again to try to get them to understand why you’re there for a cold, and not just pat you on the head and send you home to drink orange juice.

This has also been a rough week because I’ve been in ostomy hell. Usually if I’m having a lot of leaking, I can tell why. There’s some skin ulcer, or stoma bleeding problem or something. But this time everything looked great – yet I had to go flying out of work twice this week. By the time I’d had it leak five or six times, I was having skin problems. So, finally I had to, yep you guessed it, miss another day of work to go to see the ostomy nurse – who pretty much didn’t have anything to tell me. I didn’t sleep well for several nights because either I was leaking, or I was afraid I’d leak. That doesn’t exactly help one’s mood, or one’s writer’s block. Can we say WHHHIIIIIINNNNNEEEEE!

Yes, I was happy to see this week committed to the history books.

Comments

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

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.

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