Living on the frontlines

For any of the HPS’ers that haven’t been keeping up with the HPS adults Yahoo! group, and who know Sheena from conference – Sheena is having bowel surgery tomorrow. Please keep her in your prayers. This sort of thing is never fun. I know Sheena will be happy to just have it over with.

I came across this video clip on YouTube. Ben Morrison is a comedian that has Crohn's. The GI issues of Hermansky-Pudlak Syndrome have been on my mind a lot lately. We've had a bit of a GI epidemic lately. Currently we've got Sheena about to have surgery, Karen about to have a scope, Matt recovering and hopefully returning to his internship, and Katy's ongoing problems - just to name a few! Trust me - there are more out there. HPS does involve a Crohn's-like element for about 20 percent of us. Keeping your sense of humor is incredibly important to being able to live with a chronic illness. I have to admit, sometimes I'm not feeling very ha, ha - but stuff like this helps. I know there are some of you who would choose a few different words here and there - but I liked this!

I'll post in a second to explain what this is all about.

Since May when I added several new medications, my energy levels have been much better – but this week I’ve felt a bit tired and I don’t really know why. This morning I slept until noon for a grand total of 14 hours of sleep. That seems a bit much. My joints, while not horrible, had also improved and now are acting up a bit. And the sores around my stoma, while slightly improved, are still sore and bleeding. That’s a pain because it makes me nervous every time I leave the house that I’m going to have a problem. I had some blood work done on Friday. So, maybe that will tell us something.

Often there are things going on in HPSland that I just can’t blog about in detail in order to respect privacy. But this last week a few things happened that I want to mention in general terms. Two HPS’ers agreed to give an incredible gift to HPS research, and to all of us, by making a gift of their tissue to the NIH. In both cases they were having surgery and contacted the Network to ask if the bits being removed could be of value to research. Donna was in Washington this week on Capitol Hill and at a meeting of the Genetic Alliance and at NIH with Ashley. When I called to tell her of the offers, and ask what needed to be done, she got almost teary on the phone. She said that for years she’s had to ask families to donate tissue during the worst of times. She was so humbled that people are now coming forward to make such an offer. Sometimes NIH accepts the offer, and sometimes they don’t. It depends on the organ involved, the nature of the reason it’s being removed and the overall histo...

Later this year the HPS Network will launch our first capital campaign. Many not-for-profits have annual capital campaigns to help raise money for operating costs. You’ve more than likely received letters in the mail with a particular charity’s story and an appeal for donations. To have an effective campaign, we are currently developing a fundraising database – a collection of names and addresses of people who might be interested in making a donation, however modest, to the HPS Network. And, we’re appealing to you for help with this process. The people most likely to be interested in helping our efforts to find treatments, and one day the cure, for HPS are those closest to us – our grandparents, uncles, aunts, cousins, friends and even our doctors. We’re asking our HPS community to send in the names and addresses of their contacts that they think would be good to add to our database. Then, most likely around Thanksgiving, those contacts will receive an appeal letter from the HPS Networ...

This event actually happened in June, but I just got some pics for the newsletter and I thought I'd share one here. Some of our families in New York City, led by Liz Diaz, marched in the Puerto Rican Day Parade as a way to do outreach. HPS is very rare, but it does occur in 1 out of every 1,800 births in Puerto Rico. As a result, there are many people with HPS in the Puerto Rican community - and many don't know they have it. We try to do outreach to help families get an accurate diagnosis and be able to do everything they can to stay as healthy as possible. If anyone lives in the New York area and wants to march in the parade next year, let the Network know. Liz did tell the board that it's not a great event if you're not feeling well. They had to stand on the hot street for most of the morning and into the heat of the afternoon before the parade actually started. It was very tiring. If you plan to go, bring your sun screen!