Living on the frontlines

This update is long overdue. With all of my medical stuff, plus all that is going on with the HPS Network right now, I've missed a few e-mails. I found one that Donna had sent me that I hadn't counted. So, we are now at 72 to go on this year's Hundred People Search. 

The Brantner family is another family that frequently has fundraisers. Words are never enough to thank them. I really wish I could have been that this one! You know wine (in moderation) is high in anti oxidants. They tell us these are good for our lungs. For years a number of HPSers have been ready and willing to volunteer for that clinical trial! Grin!!!!  Check out the cool wine bottle labels designed by Kylee, one of our younger HPSers. Her mom is the one that organized this great event. Read more about it here: https://www.hpsnetwork.org/en/news/2015-11-20/wine-lovers-sip-for-the-cure

I'so behind on so much!!! But, here's a quick blog shout out to the Klein family - Jeff and Amber. They are fundraising warriors! They have a fundraiser, it seems, like every month. They have some incredibly loyal friends that help. Thank you so much for all of your hard work!  One of the biggest obstacles the Network faces right now is funding. We are growing and our opportunities are growing, but our funds have to keep pace. Right now, even though the total funds raised for the last year is up, the budget demands to maintain our current programs has gone up more.  Thanks to Jeff and Amber, and to all of our fundraisers. You all are awesome! 

As many of you know, I just returned from the National Institutes of Health. During my visit (with my good friend Jessica) we were able to have a long talk with Kevin O’Brien, the nurse practitioner that takes care of patients with Hermansky-Pudlak Syndrome or HPS. He asked us to convey a message back to the HPS community. We are, of course, happy to help. In the past HPS patients have grown accustomed to being inpatient at the NIH (especially the HPS adults). There were reasons why this was done in the past. To be honest, I used to like being inpatient. It gave me one place to put my things. There was no schlepping things back and forth. It was easier to get some work done at NIH when you have a base of operations, sort of speak, and then came and went to tests as needed. For most of us, however, there really isn’t a medical reason for being inpatient. We know much more about HPS and thus we don’t generally have as many tests or need as much observation. There are practical reasons fo...

My friend Jessica and I in the car on the way to the National Institutes of Health If I weren’t so tired, I’d be doing cartwheels. That is saying something since, as a kid, I never managed to be able to do a cartwheel. My NIH visit couldn’t have gone any better unless they announced a way to reverse fibrosis (which isn’t here yet). My CT scan showed no changes since my last visit to NIH in May, 2014. That was when there were a lot of changes and I was told I needed to move and get ready for a lung transplant. My pulmonary function tests were all within the margin of error, except the total lung capacity score. It hadn’t changed much on other recent pulmonary function tests, so perhaps it was a blip. We have to wait to see if there is a trend. All of my blood work was perfect. My echo showed no signs of pulmonary hypertension, so if they find something on the heart catheterization we are planning soon, then it will be very mild and early stages – the best time to treat it. I don’t thi...

We've added another HPSer to the patient registry. This puts us at 73 to go on this year's Hundred People Search (HPS).