Living on the frontlines

I think this is the number one question I get asked these days. It makes sense now that I’m walking around with the fashion accessory of an oxygen cannula in my nose! (Really got to come up with a way to bling the cannula!) So, here’s how I’m feeling – I’m feeling great! In fact, I feel better than I’ve probably felt in the last year. It seems so ironic that my entire life is in turmoil trying to make plans to move to be near the transplant center when for once I do feel pretty great. I think it is because after two years of coughing (a pretty severe cough the last six months), I am now on round-the-clock Codeine and am NOT COUGHING!!!! They also switched my inhalers from Serevent and Q-var to the max dose of Advair. We had switched them two years ago because these two drugs were supposed to be the same as what is in the Advair diskus but they were much cheaper. Turns out they are not as interchangeable as we had thought. Since I’ve been back on the Advair the asthma-like symptoms are ...

A screening of RARE was held at the annual meeting of the Association for Clinical Research Professionals (ACRP) in April. The screening was held at a general session and was attended by more than a thousand people who work with clinical trials in various ways. After the screening Donna and Ashley Appell spoke to the audience about their experience living with HPS. They also answered a wide range of questions from the audience. Since the event the Network has received several media enquiries and generous donations. You can see Donna and Ashley answering questions from the audience at: https://www.youtube.com/watch?v=cArkSSGBVLY&feature=youtu.be On a personal note, I have to say one of the more enjoyable things I've ever done for HPS outreach was participating in the documentary, and then attending screenings as it came out.  It has always been so interesting to hear the questions people have about the film, about HPS, about clinical trials etc. after they have seen the f...

I am finally rested up from my trip and have so much to share about our experience at the American Thoracic Society. A big shout out and thank you to everyone that helped out including: Candice and Crystal Sipe, Donna and Ashley Appell, Demetria Saffore, Kristen Brantner and Carrie Frew. I’ll start with what everyone really wants to know – what happened with the drug trials being discussed for pulmonary fibrosis??? Grin! The meeting was very exciting. I went to a class on diagnosing pulmonary fibrosis and the class just sort of fell apart into one big party. One of the docs told me, “I’ve been treating patients with pulmonary fibrosis for 30 years and for the first time I will have a real treatment to offer them.” The results of the Pirfenidone trial (the second one ordered by the FDA) were encouraging. Everyone seems to think it will be approved with no trouble this time and that it will be on the market in a year. I’m hoping for sooner, but to be realistic, a year is probably ab...

I promised I’d try to update the blog more often to keep everyone in the loop as I just can’t seem to reply to everyone when I feel I need to do it! For the next week I’m taking the week off from planning my move (well, sort of). For months I’ve been planning to attend the American Thoracic Society meeting in San Diego. I’ve attended this meeting for years and know many people in the ATS, both physicians and ATS staff. Honestly, when I was told I needed oxygen, I thought about not going this year. I thought about it for about five minutes. Grin! I decided that even with all of the chaos and haste going on in my life, I HAD to be at this meeting, even more so than previous years. First, it is an important year for Hermansky-Pudlak Syndrome at ATS. The full results of the Intermune Pirfenidone trial will be announced. Other potential drugs in the pipeline will be discussed. It’s a strategically important year to lay the groundwork for the next phase of the HPS battle, not just my own. Se...

Getting put on oxygen is one of those milestones in living with HPS that tends to be a rite of passage – and not in a good way. It’s the tangible signal that this thing you’ve been dreading happening one day has arrived. It’s like using a cane for a blind person. When you’ve got tubing on your face and air squirting in your nose, you can’t deny what’s happening. The people around you suddenly get that what is happening is serious (even if you’ve been telling them this for years and living with declining lung function). It’s just so viable! A few weeks ago when my PCP wrote a script for oxygen if I went to visit my mom, I was really shaken. This time the news that I had to move to be closer to a lung transplant center was such a big blow that the oxygen just didn’t seem as big a deal. Sometimes HPSers really resist the oxygen. I understand why. Not only is it this constant reminder of what is wrong with you; it’s also a big lifestyle adjustment. I’ve started calling it “life on the ...

I’ve put off writing this blog for days. I’ve had so much to do, but that is only part of it. I’ve been on an emotional roller coaster that would be enough to turn anyone’s stomach. I just couldn’t do it. But, like so many things in my life, I need to do it, whether I want to or not. I need to do it for me because writing is therapeutic for me. I need to do it because I’ve been so blessed by the outpouring of support from friends around the world. It simply isn’t possible for me to return all of the messages, texts, phone calls etc. I want you all to know what is happening in hopes you’ll continue to offer that support and prayer, whether I can talk to you just now or not. Lastly, I need to do it because I am committed to putting a face on Hermansky-Pudlak Syndrome (HPS). I don’t know how my words on this blog might be used one day, but I hope that they are somehow. I hope people beyond the HPS community begin to understand us, our journey and our need for their help to find a cure...