Living on the frontlines

This year our HPS team at the American Thoracic Society attended an awesome patient program about the common issues faced by all patients with lung disease, no matter what it is. I loved the approach and found several of the presentations really great. It also gave us some ideas that we hope to bring to the HPS Conference session line up, provided we can find a speaker to deal with them. Rather than explain the sessions, I"ll just send you to the story I wrote for the HPS Network website: - https://www.hpsnetwork.org/en/news/2014-06-25/hpsers-attend-living-your-best-life-with-lung-disease-at-the-american-thoracic

I can’t tell you how thrilled I was a few weeks ago when one of my favorite NPR programs, Marketplace, featured Hermansky-Pudlak Syndrome. I’m catching up on the blogging, so am just now able to comment on it. I’ve actually been messaging another one of my favorite NPR programs, Science Friday, about HPS and the special issues of rare diseases for years. I still think some of our issues that are in common with all rare diseases would make a great Science Friday program. I was very pleased with the Marketplace story. In their typical way, they took a very complex and potentially dry subject and made it accessible to the general public and interesting. If you would like to hear the story, go to: http://www.marketplace.org/topics/health-care/sufferers-rare-diseases-options-are-rare-too

Today we added two new HPSers to the international HPS patient registry. This puts us at 94 to go on this year's Hundred People Search. Keep up the great outreach! 

I am usually a pretty even keel sort of person. I don’t have a lot of rapid mood swings or have extremes. Since learning I need a lung transplant and have to leave my community here, however, I’ve discovered this battle is as much emotional as it is physical. Sometimes lately it’s been an hour by hour fight. Sometimes I feel very confident that everything is going to be okay. This process will require not just one miracle – getting new lungs – but lots of little miracles along the way. So far, it is as if all those little miracles are just falling into place one after the other. It feels like no matter how long the odds, somehow everything will work itself out and be okay. Other times I feel very overwhelmed, sad and panicked. I have a lot of fears about what’s ahead, and at times, they seem to take over. There have been a few days where I literally felt sick to my stomach and had headaches that I think could very likely be attributed to anxiety. It is possible that a two-hour MRI is i...

A week ago today my neighborhood haunt, Homers, organized a benefit concert to help with the unexpected expenses surrounding trying to get a lung transplant. Words will never be able to express how grateful I am to them for taking on this project! They did an awesome job! A number of different musicians played and some of the baristas that are artists donated art for a silent auction. They raised more than $1,800 to help with expenses. The generosity was overwhelming. I am so blessed. I am blesses to have so many great friends and people willing to pull out all the stops to help me. The event was almost like a going away party. I saw friends from the neighborhood, friends from work and friends from Camp Fire where I volunteered years ago. It was such a great experience to see friends from so many different parts of my life all together. The music and artwork were awesome. Honestly, I felt like crying much of the evening but I managed not to do it. I was afraid that if I did, I’d lose i...

The HPS Network is a sponsor of the Lung Epithelium in Health and Disease Federation of American Societies for Experimental Biology (FASEB) meeting. Several HPS researchers will be presenting at the meeting including Dr. Lisa Young, Dr. Susan Guttentag and Dr. Michael Beers. Read more at: https://www.hpsnetwork.org/en/news/2014-06-17/hps-network-co-sponsors-faseb-meeting

If you live in or near the Delaware area, join Amber Klein and her family and friends as they raise money for the HPS Network. Help them work towards funding the cure! Way to go Amber, Amber's dad, and their whole gang of supporters!!!! Learn more here: https://www.hpsnetwork.org/en/events/2014-06-28/jakes-wayback-burger-hps-benefit

Today we added two new HPSers to the international patient registry of those with Hermansky-Pudlak Syndrome. Keep up the great outreach efforts! 

This is just a quick update since I wasn’t able to post anything last week. I have so much to blog about as usual. On the medical front no news so far. Things are still up in the air. It’s stressful for me and my family. But, there isn’t much I can do about it right now. I’m also planning a return trip to NIH, but don’t have dates. Homers hosted a benefit concert for me on Friday night. It was AWESOME!!! I want to blog about that all on its own though. So, the update is things are still in limbo and it is making everyone crazy. Grin!

There is a LOT to do to prepare to move to Virginia. Thankfully, I’ve had a lot of help getting information, apartment hunting etc. from Ryan (my brother) and Sara (my sister-in-law). For the next two weeks, the move planning is on hold. I’m still working on issues that have to be resolved for the move, but I put the apartment on the back burner for now. I can’t get into specifics, but the medical strategy might be changing a bit and it might be October until I can move. It might not, and I can move sooner. The thing is I won’t know the answer to some of these medical issues up in the air for at least two weeks. The emotional part of me is a little grateful for the minor reprieve. I’m trying very hard to not get too emotional about things. I try very hard to just focus on what has to be done because it just has to be done. Still, these past few weeks I’ve been so tense and under so much emotional stress. Every little thing I do to get to Virginia seems hard because it’s something I’m d...

My recent trip to the American Thoracic Society (ATS) was the first time I’d ever traveled with oxygen. This oxygen world is a whole new wilderness for me. I sometimes wonder if my frustrations and complaints sound silly to those who have been at this longer and have already figured it all out. I didn’t know I’d need oxygen soon enough for my local provider to get me a portable oxygen concentrator for the trip. I’m not really sure why this is. You’d think they’d keep a few around as it doesn’t seem like that much of an unusual request. But, rules are rules. So, I ended up having to rent a portable oxygen concentrator for the trip. The problem is I need continuous oxygen at night. I “bleed” the oxygen into my CPAP machine at night. Turns out this makes the whole process more complicated. Instead of the nice, light, looks-like-a-purse concentrator, I got the very heavy (uses the word portable loosely) concentrator. It came on a “cart” like a luggage cart people used to use before everyon...

The HPS Network needs to locate HPS patients that participated in the phase III clinical trial of Pirfenidone at the National Institutes of Health. The drug Pirfenidone showed encouraging results in a recently reported Phase III study for those with idiopathic pulmonary fibrosis. Now, the drug company, Intermune, is re-applying for approval of Pirfenidone by the Food and Drug Administration (FDA). The HPS Network is advocating that the FDA also allow the drug to be used to treat the pulmonary fibrosis of Hermansky-Pudlak Syndrome (HPS). To help make the case, the NIH must document how those who were in the drug trial are doing now. This will require a return trip to the NIH. If you were in the Phase III study of Pirfenidone at the NIH, please contact the HPS Network at: 1 (800) 789-9HPS.