Living on the frontlines

I can’t believe that in just a few days I’ll be back at NIH for my 16-month follow-up visit in the clinical trial for pirfenidone to treat the pulmonary fibrosis of HPS. I’m happy to report I think this will be a very boring trip. When it comes to HPS and pulmonary fibrosis, I’m quite happy to be a very boring patient. Usually I start to get somewhat anxious a few weeks before time to go. The closer the trip gets, the more fixated I become on it. I start analyzing my breathing and probably become a huge hypochondriac. It’s a coping mechanism to steel myself against any bad news. This time, however, I’m more nervous that I might be jinxing the process. I feel great. My breathing has been terrific. Even my digestive issues have been much improved since about mid October. I’m really showing up with a few minor complaints – the skin infection that won’t go away, the sore by my stoma that hasn’t completely healed in a year, and the need to have a brief chat with Dr. Merideth about our futur

After such a long time with barely any postings, I hate to post a grumpy post, but I’m feeling very, very grumpy right now thanks to Time Warner Cable. I am not a happy customer. As many of you know, I’ve been absent from the blogosphere because my “big” computer pretty much croaked. It’s been dying a slow death for about a year now, and although I’m typing on it currently, I know I still must buy a new computer soon. I plan to make the big purchase by Dec. 31 for tax reasons. In an effort to set something up at least temporarily, I tried to connect Ryan’s old laptop to the high speed modem. I installed the drivers etc and nothing worked. I called the cable company’s customer service line, and for days now have been told I could expect to wait more than an hour for a customer service representative. Well, I’ve had things to do and couldn’t wait more than hour. But, last night was a slow night. I was just watching TV, so I called and sat on hold while listening to the TV and working on

The HPS Network has received our first donation from Google donate on the Web site!!!! Actually, the donation was made in early December and for some reason there was a delay in Google reporting it to us – something we’re working on to improve. But, I’m so excited! Just a reminder, donations to the HPS Network are tax deductible as we are a 501c3 organization.

I’m in blogging withdraw! My computer that has been dying a slow and painful death for some time has finally breathed its last. I’ve been computer shopping, which for me is like trying to buy a car. In the meantime, the wireless connection I can occasionally get at home has been very weak. I no sooner get online and it dies out. Thus, I haven’t been blogging. So, a very merry yet late Christmas to everyone! I went to the Christmas Eve candle light service at my church and it was so beautiful. They turn off the lights at the end and just have the spotlight on the alter, the soloist and the Christmas tree. I wish they had left it that way throughout the service because it was so beautiful, even before the candles were lit. I sat behind a family that seemed to have a number of young cousins visiting and they were all having a ball playing the pews, crawling under the pews, peeking over the pew at me. And I was horrible because I only encouraged them, they were so cute. I looked at their d

This is from the Kaiser Family Foundation. They have a Health Policy Report that comes out pretty regularly. Some of the numbers about what actually got approved were in here. If I weren't on deadline, I'd pull out just the stuff we're interested in - but I just can't do it right now. Sorry folks! But, for those who can't wait! Grin - Kaiser Daily Health Policy Report Kaiser Health Disparities Report: A Weekly Look At Race, Ethnicity And Health --> Capitol Hill Watch Congress Approves Fiscal Year 2008 Omnibus Budget Package; Bush Expected To Sign [Dec 20, 2007] The House on Wednesday voted 272-142 to approve a $555 billion omnibus budget package that includes the fiscal year 2008 Labor- HHS -Education appropriations bill and the 10 other unapproved FY 2008 appropriations bills, as well as $11.2 billion in emergency funds and $70 billion in additional funds for the wars in Iraq and Afghanistan, CQ Today reports (Clarke/Higa, CQ Today, 12/19). The Labor-HHS-Educati

I want to update you all on what has happened with the NIH budget etc. but I'm a bit swamped right now. This article just ran in the San Jose Mercury News, however, and I thought it was excellent. I'll put the link at the end. The reality is we've got to help the general public understand why the NIH is so important. There's a place for people to leave comments at the end of the article on the newspaper's Web site. I'd urge you to click through and let at least this one news outlet know why the NIH's budget is so critical. Playing politics with medical funding By Myrl Weinberg Article Launched: 12/18/2007 01:35:38 AM PST At a time when many are questioning the federal government's expenditures, there is one agency that's completely worthy of appropriation - the National Institutes of Health. Right now, millions of Americans who suffer from various diseases are depending on President Bush and congressional lawmakers to stop their partisanship and fund

The registration forms aren’t out yet. Not all the speakers are confirmed yet. But, for those that want to get a jump start on their plans for the 2008 HPS Network Family Conference, here’s a little information to help you get started. As I’ve blogged before, the dates of the conference had to be moved this year because the hotel is undergoing renovations. The conference will be March 28-30, 2008. The hotel room block is open if you want to go ahead and reserve your room. When the conference was in February, there weren’t a lot of groups just jumping up and down to meet in the cold of New York and the hotel has traditionally been able to be very flexible with us about rooms. In March, however, the demand for rooms is a bit greater. We had to sign a hotel contract that has something called an “attrition clause.” It’s pretty common practice, but it means that if we put, for example, 100 rooms in the room block, and our members only book 70 of them, then we’ve got to pay for the other 30

Some time ago I blogged about the National Federation of the Blind's Science Youth Slam. One of my friends was one of the adults, and one of the kids I know from Camp Fire got to participate (and is in this video). I thought the video clip might be inspiring to blind/visually impaired kids that are dealing with issues in science class, or who really would like to follow a career into a scientific or mathematical field. It's the sort of thing I would have never thought about as a kid. And, you'll also notice, there are several kids participating that have albinism! Grin!

I am sorry to report that the GINA bill to prohibit genetic discrimination was not included in the latest omnibus bill and this, as far as I can tell anyway, seems to be dead for the year. This means that next year, once again, we'll have to lobby for GINA. So close, and yet so far. I am waiting on news from the Coalition for Genetic Fairness to find out what the next steps will be. I just thought you'd want to know. I can't help but feel like GINA is the butt of some political game being played - why else would senators who have time and time again backed GINA, voted for GINA, suddenly turn their backs on it? Someone, or several someones, are not being straight up about their stand. I can't help but feel disappointed.

Gee, what do you know, it really is possible to make a action/horror flick and leave people with albinism out of it. Who knew? Grin – okay, enough of the sarcasm. Bravo to Will Smith and creators of the film “I Am Legend” for choosing to leave people with albinism out of the mix and instead feature “zombies.” The last time this story was made into a movie, the zombies apparently had albinism. It’s so nice to have an example of someone in Hollywood trying to do the right thing. Check out Dr. Reece’s blog at: http://skinema.blogs.com/skinema_dermatology_in_th/2007/12/albinos-mia-in.html

You can not discriminate against me because I might develop a given health problem. You can not use my genes against me. It isn't right, but apparently there are a few senators having a tough time with the concept. Here's the latest GINA (Genetic Non-Discrimination Act) update. The House is working this weekend to get an omnibus bill together for Monday. Your calls have helped – but efforts to block it have been stepped up with another hold on the bill: Senator McConnellH.R. 493, the Genetic Information Nondiscrimination Act (GINA) Summary: The Genetic Information Nondiscrimination Act (GINA) would prohibit insurers and employers from discriminating based solely on a person’s genetic information. Title I protects an individual from insurance discrimination. Title I applies to employer-sponsored group health plans, health insurance issuers in the group and individual markets, Medigap insurance, and state and local non-federal governmental plans.Title II prohibits the use of gene

Some of you have e-mailed to ask how I make the painted eggs. So, thought I’d reply to everyone at once and just post it here. It’s pretty easy. I cheat and buy paper mache eggs at the craft store. You can get them in all sizes ranging from little ones to huge ones. They typically range in price from 30 cents to a dollar. I make up my own designs and sketch them onto the egg. Then, I paint the egg with wood sealer to make them sturdier and so the paper doesn’t absorb so much paint that the colors aren’t bright. Next I paint the eggs. When I’m finished and they’re quite dry, I paint several coats of varnish over the finished product to make it shiny and to protect it further. That’s it! Hopefully I’ll have the Mrs. Clause egg done shortly. They do take a lot of time. You can do the exact same thing with wooden eggs.

1 pkg. frozen hash browns (the kind with the onions and peppers in already) 1 16 oz container of sour cream 1 pkg. cream cheese 1/3 of a 32 oz pkg. of Velveeta 2 cups of shredded cheese (any kind you like – even mix and match – I use the pre-shredded Mexican blend from the grocery) 2 cups finely chopped celery 2 cups crushed Special K cereal Pre-heat the oven to 350 degrees. Thaw the hash browns just enough that you can break up the big frozen chunks so that they can mix with other ingredients. (They do not need to be fully thawed.) In a large bowl, mix the hash browns and sour cream. Add in the cream cheese and Velveeta by breaking them up into small cubes, and then stirring into the mixture. Add in the celery and shredded cheese. In a 13 in. x 9 in. pan, spread out the mixture evenly. Take the crushed up cereal and spread over the top. Bake at 350 degrees for an hour. This recipe is fattening, but always a hit at potluck dinners and very kid friendly.

Karen just sent this to me and I thought you all would enjoy it. I thought it was a riot! Here we are, Donna and the dancing albino elves! http://www.elfyourself.com/?id=1381004397

I posted the video clip about the Braille Graffiti project in Portland, Oregon in the midst of the ice storm. While I was able to get the clip up, I wasn’t able to explain why I posted it. To be honest, I was just curious about what all of you, especially those that are also blind/visually impaired, thought of the project. As some of you know, I’m a big fan of Current TV. Current broadcasts what they term “pods” – short, independently-produced video clips about a certain topic. They’re sort of like mini documentaries. The other night I was watching and along came this pod about the Braille Graffiti public art project. I wasn’t sure what to make of it. My first reaction, fair or not, was suspicion. I still have a lot of questions and perhaps one day Scott, the artist, will find us here and be able to answer some of them. Suspicion probably isn’t the fair response to have – but it’s been learned after watching too many people with little understanding of blindness or the blind community

An update on the Genetic non-discrimination Act... As regular readers know, the GINA bill, the Genetic Non-Discrimination Act, is a piece of legislation the Hermansky-Pudlak Syndrome Network has supported as part of a coalition formed by the Genetic Alliance. This bill has been trying to get through Congress for more than a decade. It would prohibit discrimination based on one’s genetic pre-disposition to develop a given medical problem. This year we came very, very close – but one Senator from Oklahoma gummed up the works for the entire thing and it got buried. Fast forward to the budget debate. The White House and Congress are battling over the budget. You might remember my being upset with the White House for wanting to cut the NIH budget. Well, Congress is fighting back by creating a mega bill – an omnibus bill including all sorts of things to essentially try to make it impossible to oppose passing the bill. And, unfortunately, that means the pork rush is on too. But, one positive

After all the hype, the great ice storm of 2007 seems to have pretty much overlooked southern Kansas City. Northern Kansas City was hit harder, but after all my worry, my power (thankfully!) never went out. I left work early on Monday to make a mad dash to the pharmacy, bank and grocery store. They were all packed. The grocery store was total chaos. But, I managed to get home with all my drugs, some cash and some staples before the sleet started coming down. I tend to get a bit skiddish about ice storms. In 2002 we had a horrible ice storm in Kansas City and I didn’t have power for 10 days. The house was so cold that the water in the toilet froze. I stayed in the house for a bit with candles and a loaf of bread, but eventually ended up staying with friends – a different one every night depending on who had power. It was not fun at all. I haven’t posted mostly because things are just crazy at work right now. But, I did want you all to know I’m okay – and warm! And I’ll explain this Brai

This has to be a quick post. I’m at work and I’m on deadlines today. But, for friends and family, (mom just called from Germany worried) yes, I know there’s an ice storm headed for me. I just watched the news on my lunch break and they’re forecasting 1 to 2 inches of ice. They are warning us that we’ll lose power tonight. I’m going to get the earliest bus I can home from work. I need to go to the bank and the pharmacy on the way home. I’m out of three of my meds and I want to get cash. The last time Kansas City had a huge ice storm I was out of power for 10 days, and none of the ATMs worked. Businesses were open, but were cash only. Please don’t call my cell phone. I’m trying to keep it charged and it’s having a hard time holding a charge these days. If the power goes out and things get bad, I’ll try to call Ryan and let him know how I am. Remember my home phone goes through the computer, so if the power is out, I won’t have a home phone. Carmen will take care of the HPS adults listser

This is a latch hook rug that Karen has made for a friend as a Christmas gift. It isn't destined for the HPS Craft Collection; however, she's planning to make another rug for the Network after the holidays. She just wanted to share an example of another sort of craft project that could be done. Isn't he cute?

This egg was actually intended to be an Easter ornament, but it could be a Christmas ornament too. I painted it last year and I'm sending it to the HPS Craft Collection. In Northern Germany, where I lived when I was in high school, they have a tradition of the Easter tree, as well as a Christmas tree. The Easter tree looks more like a huge branch stuck in a base - it doesn't have any leaves etc on it. Eggs are blown out (this one is actually paper mache) and painted to celebrate Easter, and then hung on the tree. It symbolizes rebirth.

Here's a suncatcher Karen made. I think she made it when we were in the hospital together at NIH a while back.

Here are some crafts that are being sent in by Karen Tillman. She's got some door hangers, and the three items in the middle are ornaments. Darling Karen! Karen does a lot of different kinds of things, so I'll post more pics as I get a chance.

Wednesday was not a good day at work. Lets just say I was having very HPS’ish woman issues. As the day went on, the problems became worse. And when those problems are worse, it seems like everything else gets “stirred up” as well. My GI issues have been much improved lately. I wish I could tell you why, or what the “magic bullet” has been, but I have no idea. After 10 months of almost constant runs, suddenly things sort of “normalized.” That is except for Wednesday. By lunch time I’d been to the bathroom nine times. My joints were aching much, much worse than usual (lately they haven’t bothered me at all hardly) and I had that lower back pain like I had at NIH in April. It’s a sharp kind of pain and no matter what you do, lay, sit or stand, you can’t get any relief. Finally, I’d had enough. I really didn’t want to go home. I’d like to make it through at least most of the rest of the year without having to leave in the middle of the day for medical stuff. Besides, if I could hold out ju

Below are a few pictures of some crafts I've been working on lately. They are destined for the HPS Network's Craft Collection. There are a lot of us out there that don't have a lot of money to offer the HPS Network, but we do have talents to offer. Many of us do crafts, or sewing or artwork that could be valuable to the Network. Many times a year different HPS families hold fundraisers. Sometimes they hold a silent auction as part of those events. Other times we need special gifts to give to important people in the fight to cure HPS. So, some of us that do crafts have decided to form the HPS Craft Collection, a sort of "craft bank" the Network can tap into as needed. I have another pic to post, but it wouldn't upload. It's of one of my Santa eggs - stay tuned. Below are some crafts I did while waiting for my computer to reboot. My very old and very sick computer crashes about every half hour and takes five to ten minutes to restart every time. I used the t

“Be an organ donor. The life you save could be that of someone you love…” This was the message that used to play on my friend V’s answering machine. He had a keen interest in organ donation. He was trying to be listed for a lung transplant, but didn’t make it. Currently I have two friends playing the transplant game – one anxiously waiting for her beeper to go off, and another trying to get on the list. Both are in need of a new pair of lungs. Someday it very likely could be me. The trouble is there aren’t enough lungs to go around. Or hearts, or livers or intestines or any other sort of organ you might be in need of. The sad truth is waiting for an organ transplant (in cases where live donors can’t be used) requires waiting around for a tragedy to befall another family. You have to wait for someone else to die to give you the gift of life. It’s the sort of tragedy they won’t see coming. They won’t have weeks and months to come to terms with a horrible situation the way those of us wit

Hack, hack, (clearing of throat) hack….sneeze. This is the way my office has sounded the last few days. I bet no one else has even noticed much, but when I hear someone a few cubes over start to cough, I immediately take note and steer clear. Maybe it’s just the dry heater air. Maybe it’s a touch of asthma on the part of a co-worker. Or maybe it’s a germ on the prowl for me. I can’t afford any respiratory infections. I can’t afford them because they’re bad for my lungs, and I can’t afford them because, frankly, I just can’t afford to be sick anymore – literally. No more unpaid days off work. No more doctor visits and definitely no more prescriptions to fill. I’ve got more than my fair share thank you very much. I noticed the cough is spreading down the cube farm. I notice other co-workers complaining of feeling run down and tired. I’ve been feeling tired too. Last night I had intentions of working on housework and an HPS project. I did neither. I laid on my couch with the fuzzy blanket

During the Thanksgiving holidays Ryan and I rented the DVD Sicko, Michael Moore’s documentary about the state of the American health care system. I’d been wanting to see it for some time, if for no other reason than the movie made such a splash and got people talking about our health care woes. You may remember that Michael Moore got in a bit of trouble for taking some of the patients he was profiling to Cuba for care. Americans aren’t supposed to travel to Cuba as part of the embargo – unless you’re a journalist. And that’s where things got sticky for Michael. He was supposed to check with the State Department first, which always struck me as a bit of a freedom of the press killer. All that aside, my feelings about the film, just like my feelings about American health care, are mixed. You can love Michael Moore. You can hate him. The guy did make a point that needed to be made. For millions of us, having the misfortune to get sick has ruined our finances and stolen our dreams. There r

Cream Cheese Enchiladas Ingredients 1 package cream cheese 1 10 oz can of condensed cream of chicken soup 1 10 oz can of green enchilada sauce 1 20 oz can of rotel drained 2 cups shredded cheese any kind you prefer (I buy the pre-shredded Mexican blend) 4 cups cooked and shredded chicken 12 flour tortillas Preheat oven to 350 degrees. Combine the cream cheese, green enchilada sauce and cream of chicken soup in a large microwavable bowl. Put it in the microwave to melt the cheese and stir until completely mixed and saucy. Add the drained rotel. Have your chicken already cooked and cut up or shredded into little pieces. (It usually takes six to seven chicken tenderloin pieces.) Get two 9 x 13 pans ready. Take a few spoonfuls of the sauce and spread it out in the bottom of the pan. It doesn’t have to be thick….just spread it around a bit to keep the enchiladas from sticking. Take a tortilla and in the middle put a moderate handful of chicken. Spoon enough sauce to cover the chicken well,

One thing I’ve had a hard time with all week was energy. Sometimes I just get to feeling so tired. I’ll even put my head down on my desk at work and fall asleep, which isn’t a good thing because I snore! So, the thing I was looking forward to most this weekend was sleeping in late until my body decided to wake up. That didn’t happen until 2:30 pm! Sometimes when I sleep these incredible amounts of time I really do think something must be wrong with me, but I’ve had my thyroid tested and I’ve had blood work, and everything is normal. So, I don’t know why I am so tired. Perhaps it was the gloomy day. Today the clouds were so dark that it felt as though the sun never came out. Usually when the clouds are dark and heavy, they either let out a torrent of rain, or blow away. They don’t normally hang there all day with such a darkness that you have to have the lights on in the house. But that was the kind of day it was here today. I normally love these kinds of days. I love to hear the wind i