Living on the frontlines

This weekend hasn't been the best one ever in the health department. This back pain is really something else. Kevin sent me home with a whole host of new scripts, among them Oxycodone for the back pain. The pain management is a bit of a challenge because I can't take anti-inflammatories because they cause bleeding, and I can't take stuff with morphine because I'm allergic. The Oxycodone seems to be working very well for the back pain (which they now think is related to the bowel flare up and not the kidneys.) It doesn't knock it totally out, but it does soften it to a annoying, but bearable, dull ache as opposed to a sharp, shoot-me-now-I-can't-move pain. But, I think the drug is giving me constipation. I need to ask if this is possible. It definately irritates my tummy, but suddenly in the last two days I've only emptied my pouch three times and I've got pretty bad cramps in my belly. Usually if I'm not having the runs I empty it four or five times

It's been a very long week here at NIH, but we should wrap up tomorrow and to be honest, I couldn't be happier about my test results. So, first the news we all wait to hear about every four months. My lung function numbers were essentially all the same as last time. That means the nice little bump my FVC had in December is holding steady - and we all know it isn't thanks to my brilliant work out schedule these past few months. Every time I come they ask me if I think I'm on the Pirfenidone. This is the way I look at it. I'm in the trial. If I'm getting it great. If I'm not then I'm just helping do the work to eventually get to the goal. I could make myself crazy trying to figure out if I'm on the real stuff or not. In the first few months, I did think about it a lot. But now, I've sort of settled down on that front. I can't do anything about it, so I intend to assume I'm on the drug and move on. The odds are in my favor, and if I'm n

Today was a long day at NIH - a.k.a Club Med. My hives made their appearance last night, all night. I was miserable. Poor Karen had to put up with me scratching and moaning all night. I don't think either one of us got any sleep. I got them to give me some Benadryl (they took all my drugs on the way in the door.) It wasn't enough. I had them call poor Kevin at 10:30 pm to put in orders for more drugs. I felt so bad potentially getting him out of bed, but I knew it was getting worse and if we didn't call then, they'd be waking him up in the middle of the night. It didn't help much. I itched, and itched and tried not to complain because i didn't want them to wake Kevin unless I was just dying. When Kevin arrived this morning I was in quite a state. He's ordered a whole battery of tests and now we're trying allergy meds. He also ordered X-rays of all my sore joints and lots and lots of blood work. I hate to cut this short, but I've got to go......stay t

I'm going to keep this short because we're tired, but Karen and I have arrived at NIH. I feel so honored. When Karen got here they'd given her a private room, and she complained and said she wanted to room with me. Isn't that sweet? Grin! As before, you'll have to put up with my horrid typing and spelling as I have to type directly into the blog here, and frankly, the screen is hard to read. I had a hive-free day, but right now I'm covered once again. Groan!

Usually when I’m getting ready to go to NIH I get really nervous the few days before. I go through this whole mental routine trying to prepare for any bad news I might get. This time, however, thanks to benadryl and allergies, it’s midnight before I leave and I’m not even finished doing laundry! (Thank God I’ve got an afternoon flight!) I haven’t had much time to stew about the trip or what might happen. Frankly, I’ve been a walking zombie because of the allergies. Who needs anti-anxiety medication? Just take a benadryl! HA! This is probably a blessing. I am actually anxious about the trip. For a change, I’m not terribly worried about my pulmonary function tests. I think I’m doing okay there. I’m more worried about whether they’ll think my digestive issues are acting up (although if they are it’s still mild) and I’m a bit worried about what they might find in the extra labs they’re planning to run. Because of the joint pain etc. they’re testing me for several auto immune issues. Today

This has not been a good week for blogging, but I thought I’d post and let you know I am alive. Grin! I’m just dopy. Thursday morning about 2:00 am I woke up itching intensely from head to toe. I turned on the light and my body was covered in hives. They were big red splotches with large white bumps in the middle of them. I took one of the little pink benadryl pills and went back to bed. After a half hour I was still itching and trying not to scratch. I got up and took another pill and that seemed to do the trick. By morning the hives were gone, but I was in druggy land. Functioning at work was horrible. Finally the drug started to wear off after lunch, but the itching returned (although the hives weren’t as big). The same thing happened on Friday and today. I seem to be able to keep them at bay with benadryl, but the benadryl knocks me on my butt, to be quite frank. So, that’s why I haven’t been blogging. It’s been a chore just to stay awake!

I am entirely too knowledgeable about my poo. When I saw the doctor in February and started to complain about GI issues, she explained to me that having an ostomy, my poo wouldn’t be like “normal poo.” I was nice – but in my head I thought, “no kidding – I’ve had an ostomy for 14 years. I’m well aware of what my poo is normally like.” Monday was a pretty good day. No joint pain, not even a little. In the evening I went to an awesome folk music concert (I’ll blog about that later). On the way my friends and I stopped at a new Hawaiian restaurant in the neighborhood. The food was great! The company was refreshing. It was really nice to get out for a change. We made our way to the concert a tad late and took some seats in the back. The band started, and I felt it. My pouch was filling up as if someone had turned on a faucet. It pretty much behaved its self the rest of the evening and, after one quick escape to the restroom, we enjoyed the evening. I got home and went to bed. Within an ho

The fervor over the soon-to-be-filmed movie “Albino Farm” continues to grow. I noticed today that the guestbook on the Web site had been shut down. I thought maybe it was just me, but several others have reported the same problem. That’s okay. The point was to be noticed. Someone must have noticed. In the past few days I’ve been able to take a deep breath about the movie. I’m still pissed as hell, to put it very bluntly, spitting mad and not about to let the issue drop – but once you’ve lit the fire you’ve got to give it just a little while to burn. Given all the press generated about the Da Vinci Code last year, I find it hard to imagine the project’s principles were completely unaware that what they were doing might be offensive – but I guess it is possible. I know the leadership of NOAH is now aware of the issue and so before posting some more links and ways that we can express our displeasure – I think we should give it a few days. Let’s see if NOAH has any luck opening a dialog wi

This is the windmill that is in the center of the town of Osterholz. I know it very well. I spent many hours drawing it in high school art class. Grin! Home is a funny concept. If you’ve always lived in the same place, you know where you call home. For me, having moved every three years of my childhood, home isn’t just where I live today – it’s the places I’ve lived with the happiest of memories. I look back on the places I’ve lived and I feel “homesick” – even though if I returned to them today no one would know me and everything would have changed. I’m not sure I’d want to go back to some of these places, even if I could. They are so wonderful in my memory, I’d be afraid I might spoil them. One of those places for me was Osterholz-Sharmbeck, a town outside of Bremen in northern Germany. Osterholz-Sharmbeck, as I remember it, sits on the edge of the Teufelsmoor (Devil’s Moor). I lived in a village outside of Osterholz from 1987 to 1991. When I look back at that period of my life, I

I am not “evil.” I am not a “freak.” I am not your literary device. I need to vent. Please indulge me. I am not possessed by the devil. I don’t have magical powers and I’ve never killed anyone. I don’t dwell in caves (although my eyes are sensitive to light.) Lastly, I can’t conduct electricity. I am a person with albinism (HPS type 1 to be specific). Instead, I go to work every day. I pay taxes and vote and participate in my community. I spend many, many hours doing volunteer work. I hate housework and love to paint, mostly watercolors and acrylic. Just like everyone else, I go out with friends, go grocery shopping and although I never actually buy lottery tickets, I still dream about what I’d do with a million dollars. In short – my life is ordinary. But these myths about albinism do affect me – even if they do sound outrageous and bizarre to the sensible person. Just a few weeks ago a couple of teenagers going down Metcalf yelled “Ghost Freak” out their car window at me. Last year s

I’ve got a week to go before my eight month check up as part of the Pirfenidone trial to treat the pulmonary fibrosis of HPS. It was supposed to be a relatively quick visit. I need to get some blood work, pulmonary function tests, six-minute walk test, provide a stool sample do and a 48-hour urine collection. No big deal. I’m going with my friend Karen, who is having some other procedures done, so I planned a pretty flexible trip so I could be with her. As it turns out, that was a blessing because I may need to be there an extra day or two as well. I spoke with Kevin about this joint pain issue, the fatigue etc. He’s planning some extra blood work for me to test for any auto-immune disorders such as arthritis and lupus. He also wants me to do a GI consult as some of this could be related to a GI flare up. Better safe than sorry. Hopefully the problem is easily treated and isn’t anything too serious that could interfere with my ability to participate in the research. While these problem

Just a quick note to say I'm okay. I've been really bad about returning e-mail this week, and blogging. My joints are acting up and my fingers hurt, which makes typing ohhh so fun! So, I've been limiting my typing to things I need to do for work. Actually, this evening my joint pain is gone! But, I've had a lot of work things to catch up on. So, stay tuned. I had a long talk with Kevin yesterday about the upcoming NIH visit. I'll blog about that soon! In the meantime, please forgive me for not keeping up! Grin!

Today was not one of my better days. Get out that cheese for the whine! I hate complaining because I know it could be worse. This morning I woke up about 5:00 am because my joints were hurting. My ankles, wrists and fingers ached, but especially my ankles. The worst thing about waking up at 5:00 am as opposed to say, 2:00 am, is that it’s close enough to the actual time you’re supposed to get up that you can’t really get back to sleep. So, I got up, ran a very, very hot bath, and soaked my joints. It did help. I went back to bed, turning on the radio to listen to NPR. I knew I couldn’t go back to sleep, but I wasn’t ready to wake up either. All day today my joints ached. Thank God my fingers don’t hurt as bad as my ankles, but still, I was having some trouble typing. I’m also still feeling easily tired. I seem to start out okay in the morning, but somewhere around mid afternoon it feels like I’ve been drugged and it’s all I can do to make it to the magical hour when I can leave. By the

I am, bar none, the world’s worst dieter – yet I’m constantly trying, falling off the wagon, and trying again. I know everyone has lots of excuses, but it isn’t easy (a subject for another blog entry.) For the past few weeks I’ve been on the “kick.” I know Kevin is going to give me hell when I’m at NIH, and I’m just working and praying for even the slightest success to keep him, and the nutritionist, off my back. Even just a few measly pounds would be nice – anything to prove that I’m actually working on this problem. One area I was particularly struggling with was beverages. I like fufu coffee – the stuff that doesn’t actually taste like coffee because it’s so loaded down with sugar and cream. Knowing that such drinks can really get you, I’d been avoiding them – that is until my most recent battle with fatigue. I’m so tired before the work day even starts that I’ve naughtily been stopping at the deli in the basement of my office building for a little Starbucks treat. I need the caffei

Ingredients 1 to ¼ pound chicken breasts cut into pieces 1 to 2 tablespoons vegetable oil 2 cans (14 ounce) of chicken broth 1 package (16 ounces) frozen corn thawed 1 can Rotel 1 medium green pepper chopped into small pieces 1 medium sweet red pepper chopped into small pieces 2 tomatoes chopped into pieces 1 teaspoon ground cumin ½ teaspoon garlic powder (Note: I go heavy on the garlic and cumin – it’s the cumin that gives it that zesty southwest taste.) In a large skillet, sauté the chicken until lightly browned. Transfer to a 5-quart slow cooker. Stir in the remaining ingredients. Cover and cook on low for seven to eight hours. Stir before serving. (Note: if you add some water to extend the chicken broth you can add more veggies like onions and mushrooms. Also, when you serve it up, try garnishing with fresh cilantro cut up very small.)

Ingredients 1 8 ounce package of cream cheese 1 tablespoon maraschino cherry juice 1 cup whipped cream or topping 1/3 cup sugar 12 ounce can of crushed pineapple drained ¾ cup maraschino cherries quartered 1 cup miniature marshmallows 1/3 cup chopped nuts (Note: While this by no means makes this a low-calorie recipe, you can use the fat free cream cheese and the no-calorie whipped topping and it tastes just as good with a little less damage. Also, for other colitis/ostomy people that have trouble with nuts, I leave them out.) Method Cream together the cream cheese, sugar and cherry juice. Add in the rest of the ingredients and mix together. Pour into and 8 ½ by 11 inch pan and refrigerate for one to two hours. Cut and serve.

The following is a blog entry from last year. I thought I'd repost it. Blood gases and Easter If you’re a history channel junkie, like me, Holy Week is chalked full of all kinds of interesting documentaries centering on the events of this week 2,000 odd years ago. I don’t typically have much time to watch TV – and this week was no exception – but I do listen to it in the background as I do other things. One documentary (not sure if it was on the History Channel, the Discovery Channel, or the Science Channel) was a medical look at the crucifixion. This researcher had constructed a cross in his lab fully outfitted with sensors and he persuaded volunteers to endure a mock crucifixion on his cross (taking care that they weren’t actually physically injured.) Apparently, there has been some debate over the years as to just how Jesus would have been nailed to the cross in order for events to have unfolded as they did. If the nails, for example, were driven through the palms of his hands,

Today was a frustrating day. I was just about to leave for work when I felt “the itch” again. A quick dash to the bathroom confirmed that I was leaking. There was no way to fix the problem and still catch the bus. So, I fixed the problem. What else are you going to do? As I pulled the ostomy pouch off once again there was a raw spot under my stoma and it was bleeding. Not a lot, but it doesn’t take a lot to make things leak. I thought I’d fix the problem and then call a cab and go to work. Wrong. By 10:30 pm, in the span of only a couple of hours, I went through four pouches. Thank God my insurance is currently covering the cost of my ostomy supplies. For many years the insurance didn’t cover it and I had to pay for them. I suppose it’s the years of shelling out money for them myself, but the little cash register is clicking away in my head on days like this. By afternoon things had calmed down. I was able to get some work done from home – although not nearly as much as I’d hoped. I’ve

While working on the newsletter I produce for my magazine and eating a chicken salad wrap, I heard another story on NPR that perked up my ears. It’s a kind of radar I seem to be developing for stories that have anything to do with science, genetics, health or mortality. This one had them all. A man named Ronald Bailey, the science correspondent for Reason magazine, a libertarian publication, was being interviewed on the NPR program “Day to Day” about his recent article concerning whether, if it were possible, he would want to know when he was going to die. The article its self (despite being on deadline I couldn’t focus until I’d sneaked a peek at it) was about all of our advancements in the study of genetics. It’s increasingly possible for all sorts of people, not just we HPS’ers, to get that kind of glimpse into the future. We’re finding more and more genetic links to disease and thus, it’s increasingly possible to know in advance what might make us sick, and what, in the end, might

I’m getting really tired of feeling tired. Last night I slept like a rock. I woke up once with some reflux, but otherwise slept through the night barely even disturbing the covers. I woke up feeling pretty good. My morning at work was very productive and things were humming right along until about 1:30 pm. It was as though I hit some kind of brick wall. Just like that I started feeling really, really tired. I couldn’t focus, had trouble keeping my eyes focused on the same line of text and pretty much just yearned to crawl under my desk and take a nap! I came home and slept for three hours. Several HPS’ers tried to call, but I didn’t even hear the phone ring. Finally I got up and went for about a 20-minute walk. I just didn’t have the energy for more. It wasn’t that I was having any trouble breathing – I just felt floppy, like a rag doll. And those weird hot flashes where I get all flush in the face were back this afternoon. This evening my ankles and wrists hurt – not horrible, horribl

Today I finally talked with my company’s HR department about the Family Medical Leave Act (FMLA) and my various health problems. It was much harder than I ever realized it would be. I should have had this conversation some time ago, but I suppose I didn’t realize just how much I was dreading it. My medical life is an open book. It wasn’t so much that I minded sharing what’s happening to me. Perhaps it was a psychological thing more than anything else – I had to formalize how Hermansky-Pudlak Syndrome is impacting my work life. I had to “petition” for consideration related to this disorder – and I was very surprised at how that impacted me. I really didn’t expect to have any sort of a reaction to it. I’m not particularly close to anyone in HR so it was like talking to a stranger about my issues. I explained the clinical trial at NIH. I had to explain why it is I’m traveling to Bethesda every four months for this drug trial and not seeking out something closer to home. The turn the conve

Lately I’ve been feeling so much better than I did the last part of January and most of February. The weather has been beautiful here, and I’ve been walking at least 45 minutes a day and not feeling totally crappy afterwards. Sometimes my schedule won’t allow me to do 45 minutes at one go, so I break it up into two walks. Maybe that’s not the best, but I figure it’s better than nothing. I’m just so happy that walking feels good and that I don’t feel wiped out when I get home! But, lately I’m back to feeling tired again. I hate fatigue. It’s one of those “symptoms” that’s so hard to nail down. It can be caused by any myriad of things. It can be totally debilitating, and yet often nothing shows up on blood work. It isn’t “severe” since it doesn’t hurt – yet it’s hard to explain to the people around you that when you say “I’m tired” you don’t just mean you didn’t sleep well the night before or you need a nap. I mean I’M TIRED! Last Thursday evening I wasted much of the evening dosing afte

The following tidbit ran in the recent issue of Oprah's magazine. I just wanted to remind everyone to try to use Goodsearch, at least for part of your Web surfing. Our numbers have been falling on Goodsearch. But, the thing I love about this fundraiser is we're not asking anyone to spend more than 30 seconds a day and we're not asking them do something they're not already doing. Best of all, we're not even asking them for money! So, just a reminder to use Goodsearch and share it with family and friends. O-Zone: Pennies From Heaven April 2006 Imagine if every time you logged on and looked up the caloric content of the snack you just ate, you earned money for charity. With each search you launch on goodsearch.com, about 1 cent goes to the charity or school of your choice. You simply select an organization from a database of thousands or add your own, then tell all your friends, tell your friends to tell their friends and so on. If 1,000 people with the same charity se

There's still more work to be done for the Genetic Non-discrimination Act. The Congress is on their spring break, but when they get back they'll likely be taking up this bill for debate and a vote. Let's make sure our Congressional representatives come back to their offices only to find us clamouring for their support of this bill. There's a link here for the Genetic Alliance's Action Alert. As many of us have blogs, I'd also encourage you to not only write to your Congress people, but blog about how you want them to support the legislation and list them by name. I've noticed that every time I list representatives by name, I get a hit from the Congressional ISP. I think elected representatives pay more attention to the blogosphere than they used to - so give them something to find! Get your friends to repost it as well! (And to call or e-mail their reps. GINA doesn't just impact people with Hermansky-Pudlak Syndrome - it impacts everyone with genes!) So,

You all crack me up! To date the two topics I’ve received the most e-mail about were my opinions about the TV drama Gray’s Anatomy and now, Mr. Boom Boom. Oh if Mr. Boom Boom had any idea the attention he’s getting throughout the world! I’m happy to report that all was bliss in our little apartment complex on Saturday. I know Boom Boom was home because I heard his TV on (at a nice low, normal, level) Saturday evening. When I didn’t hear him stir all day I did start to worry. But, I’m guessing he had quite the hangover after his Friday night jam session. This evening Boom Boom hasn’t been home. AAAHHHH – bliss! Quiet! Yes! My earphones, however, stopped working. I panicked. It’s such a pain to return anything when you don’t drive. But, it turns out the on and off switch for the headphone set is separate from the one on the base. So, I’d turned off the base, but not the earphones. The earphones are powered by AAA batteries, so of course, after running for two days they died. I think I ne