Living on the frontlines

I finally heard from the University of Kansas Medical Center pulmonary lab, and I’m having my pulmonary function tests on July 7th. Pulmonary function tests, as medical tests go, aren’t bad. All you have to do is breathe into a big tube. The hardest part of the test is getting your lips to fit around the mouth piece and keeping the nose clip on. But, these are the tests that cause me the most anxiety. They are the biggest predictor of how my lungs are doing, and how quickly they may be going downhill. This time I’m not sure if it’s possible to get really good news. While my FVC was still in the normal range a year ago (very good news), several of the secondary scores weren’t so hot. These are early predictors of pulmonary fibrosis. The rate of the development of fibrosis varies greatly in Hermansky-Pudlak Syndrome. Some people seem to have a very rapid development, while for others the process is very slow and happens over many years. You never know. I’m very much on the edge for being

Yesterday I returned to my office to find more than 200 e-mails (those were the lagit ones) in my work e-mail. They were mostly press releases and it took me until noon to skim them, print out the good ones, and file them in the appropriate places or pass them on to our intern. I came across one that had me working very hard to contain my giggles, or I’d have to explain what was so funny to the rest of the office. The press release was for a new start-up company that is marketing toilet paper sponsorships to the trade show industry. Essentially company x at xyz show pays so much money for the right to print and display their logo and booth number on all the toilet paper being used in the convention center. I laughed out loud. It’s an idea I’ve seen done before. But, my mind being so obsessed with Hermansky-Pudlak Syndrome outreach, the first thing that came to my mind was the annual Digestive Week show for doctors that specialize in digestive issues. We’ve wanted to attend this show fo

This is a photo of the Royal Sonesta Hotel on Bourbon Street. I can't take credit for this one however. It came from the New Orleans Metropolitan Convention and Visitors Bureau. The airport shuttle deposited me at the Royal Sonesta Hotel on Bourbon Street in the heart of the French Quarter. I have no idea what the rack rate for the hotel is, but I can say if you ever go to New Orleans and want to see the Quarter, this hotel’s location is perfect. The lobby is beautiful with white marble, quasi Victorian decor and the sort of large chandeliers that could only exist in a hotel lobby. There’s a lovely courtyard with a fountain at the center of the hotel. My room was, well, a hotel room, but it had a plasma TV and a set of French doors that opened onto a balcony overlooking the courtyard. Considering all the drunken chaos right outside the hotel, inside things were amazingly quiet. The hotel sales staff had left me a bottle of champagne and two glasses. I wonder who they think was joi

As the plane descended through the clouds over New Orleans, just as the sun was setting, it seemed as though most of the passengers were craning their necks for a glimpse of the city – a bird’s eye perspective on the last 10 months. I was just as bad as the rest holding my monocular to the window trying to see the streets below as the altitude lessened. I couldn’t see much. There were pockets that were bright and lit up by street lights – a baseball diamond with those high-powered florescent outdoor lights – but then again there were streets dotted by the occasional streetlight, but not much else. I wondered if these were devastated areas full of unoccupied houses, or if I simply couldn’t see well enough to see the lights on in smaller buildings. When I stepped onto the jet way from the plane that unmistakable New Orleans air hit me – thick and heavy the way humidity is in the South, as if the air is wrapping you up in a stifling hot blanket. The airport terminal looked like a ghost to

I’m back! And, I’ve got tons to share about my trip. But, I’m exhausted so I probably won’t do it tonight. I’ll probably also have to blog about it in pieces. For now, all I can say is that considering all that New Orleans has been through in the last 10 months, the city is amazingly resilient. I didn’t get a chance to venture beyond the convention center and the French Quarter because I was so busy covering the events of the show. I know from the attendees I spoke with that rented cars to go look beyond downtown that vast parts of the city are still in ruin. But, the parts I saw can best be summed up by this picture. The city is still there. It’s still beautiful and vibrant and unique – and although it was in good enough shape to host the 17,000 tourists that came to town for the American Library Association’s show, it still isn’t back to normal. As you can see from this picture, the iron work, the French influence and the Cajun spirit is still there, but like the city, there’s a bit

Please don’t be alarmed if you don’t see a few posts for a few days. I’m going to try to post over the weekend, but not sure if I’ll have access or time. I’m going to cover the first major, citywide convention and trade show to be held in New Orleans since Katrina. It’s an exciting opportunity. It’s not very often, unfortunately, that I get to cover stories in person. I’m a bit nervous though because I feel like I’ve got a lot of loose ends. Usually when you go to a show, you register weeks in advance and get a nice neat schedule of where you’re supposed to be and when. For this event, however, everything came together to allow me to go at the last minute. I don’t have a schedule and I’ve got a very short time to interview a lot of people. As great as I’d been feeling lately, these past two days haven’t been good ones. I’ve been sick to my stomach at night, the joint pain is worse, and while my tummy feels better during the day I do feel super tired all of a sudden. All I really want t

Meet Kevin. Kevin takes more ribbing and abuse from we HPS'ers and he does it with complete tolerance and grace. We LOVE to tease Kevin, partly because he can be so serious sometimes, and partly because we get such a kick out of "the look." You can see "the look" in these photos. You can sort of picture the photo caption being, "OMG, I can't believe I"m dealing with these crazy albino people!" HA! Kevin would never in a million years actually say that! The first picture is from Sheena's recent visit. As I said, Kevin can be very serious (as he should be) and apparently he was telling her some things, and she whipped out her camera and got him! The second photo has a bit o f a story behind it. A few years ago at the HPS conference Kevin was hanging out in the hotel bar with a bunch of us. Kevin's willingness to share a beer with the HPS'ers and come and hang out in the bar goes miles towards people trusting him. I'm sure no one

Today I got a call from Kevin O’Brien, our nurse practitioner at NIH that looks out for all the patients with Hermansky-Pudlak Syndrome. He was calling to find out how I’d been and talk about when to bring me back to the NIH next. Kevin had heard I’d been coughing a lot from other HPS patients. I told him that back in April. Of course, now that he’s asking about it, I’m not coughing as much. “How do you feel? How’s your breathing?” Kevin asked. Seems like a reasonable question, but I couldn’t help but laugh. “Kevin,” I said, “You know every time I come back to the NIH I’m convinced I’m having more trouble breathing, and every time there’s been little if any change of my FVC. You’re asking me?” Of course I’m worried. Every time I have a hacking fit, and then can’t catch my breath afterwards for sometimes 10 minutes, I have visions of these little macrophages in my lungs, all fired up and foamy. Every time I get a cold I wonder what’s brewing in there. “I’m a hypochondriac about my lungs

I’ve been looking for some answers about the Google AdSense program, and I can’t seem to find them, so I’d love to exchange information with any other bloggers that have enrolled in the Google AdSense program. I’ve read through all their info, and I can’t figure out exactly how bloggers get compensated for the ads placed on their blogs? Granted, I’m not exactly that tech savvy, but I do work in publishing so I know something about ads. I’m hoping maybe I just didn’t see the answer in there some how. From what I read it seems that you’re paid on some kind of formula that combines click-throughs and impressions. But, what’s the formula? I’m not exactly running a mega blog here, and I don’t want to clutter it up with ads for just pennies. What is the cost per 1000 impressions? What counts as an impression? Do you have to get click throughs on any ads? I just think it’s strange that they don’t offer up this magical formula so you could look at your site stats. and make some kind of educate

It’s been a little while since I’ve written about my current personal health situation. Perhaps that’s because when you’re feeling pretty good – it’s an out-of-sight, out-of-mind matter. My cough, while still not gone, is much improved. My breathing is also doing pretty well. I’m barely using my emergency inhaler. And, perhaps best of all, my joint pain is much improved. It’s still there sometimes, but it’s much less frequent and much less severe. I can go for a walk, or to the gym or whatever, and I’m not miserable. My fatigue has also been better these past few weeks. I still don’t think I’m as peppy as a lot of other people my age, but I’m not coming home every night and crashing for several hours. Between feeling better, and having an intern that’s taking some of the pressure off my day, I’ve actually been catching up on my personal life. You have no idea how many things go untaken care of when you’re working 70 plus hours a week and feeling sick all the time. It’s been months sinc

After all the enthusiasm about the Puerto Rican Day Parade, alas the HPS crowd was absent. One of the challenges of working with an all-volunteer group full of people with major medical problems is that sometimes people get sick. We had a contingent of three working on the parade, and the week of the event one was in the hospital, one was in the hospital with her daughter and the third wasn’t hospitalized, but was instead at home worshipping the porcelain God, if you know what I mean. Just an update for those that were wondering how the parade went.

At the end of every stay at the National Institutes of Health (NIH), the doctors do a “wrap up” session where they go over all your test results, explain what they mean, answer any questions, and give you copies to take home to your own doctor. After a week’s stay the moment of truth arrived – the wrap up session. I was intent to make the most of this session. The way I saw it, I’d just donated a week of my time to the research, submitted to every test without complaint, and so I’d earned the right to spend some time with Dr. Gahl and pick his brain about everything I’d read over the summer. (Not he doesn’t gladly take the time to answer questions.) I’d written out three pages of questions. This was my big chance to talk to the world’s foremost expert – and I wasn’t going to blow it. I remember the floor was quite full of patients that week, and so Dr. Gahl, his fellow, a small army of students and I walked up and down the hall trying to find an empty room to talk in private – or at le

Congrads Holly! This is a photo of the Tillman family. If you're a regular reader, you've read about Karen Tillman, a very good HPS friend. Well, here's her daughter, just recently graduated from high school, and her husband who is the nicest guy you could ever want to meet. He's stood by Karen through all her medical issues and takes wonderful care of her. They're just the neatest family you could ever want to meet. And, if you want to want to read Karen's blog, check it out at myspace.com-karenshpskorner.com.

After a very long summer I traveled to the National Institutes of Health (NIH) in Bethesda, Md. to undergo the most extensive physical exam you can ever imagine. As I left, I believed I had Hermansky-Pudlak Syndrome (HPS), but was unsure what else I might learn from the trip. The idea of spending a week all alone in the hospital waiting to discover my fate was, well, scary and surreal at the same time. After nearly two months at Wesley Medical Center in Wichita in 1993, (what I referred to as staying at “the Wesley”) I had made a point of having as little to do with hospitals as possible. I’m a medical chicken at heart, scared to death of needles or pretty much anything with the potential to hurt, so the idea that I was volunteering for a week of scrutiny signified how concerned I really was about my prognosis. I needed to know where I stood. I remember thinking on the plane on the way to Washington that what I learned during the week would likely change the rest of my life – and it di

There are certain times of year that I associate with certain HPS-related events in my life, and this is one of them. It was four years ago about this time when I figured out what having Hermansky-Pudlak Syndrome could mean long term. Sometimes I tell my diagnosis story in an abbreviated way because it’s too complicated for most five-minute presentations – but I consider my HPS diagnosis date to be the first week in August, 2002. The truth is I was first told I had Hermansky-Pudlak Syndrome when I had my colon removed in February, 1993. It was hardly much of a diagnosis. I’d call it a lucky, yet uninformed, guess. My blood work before the surgery showed clotting times twice what they should be. Before undergoing such a major surgery, my mother insisted that a hematologist be brought in and this anomaly explained. So, a hematologist was summoned, scratched his head, and disappeared into the hospital’s medical library for a day. (This was before the Internet made looking everything up so

Speaking of the mystery HPS'ers, meet my friend Sheena who was so kind as to share a few of her pictures from her recent trip to the National Institutes of Health (NIH). Sheena has albinism, colitis and bleeding issues, but does not have HPS. There's something going on with her platelets, but we don't know what yet. This is Sheena waiting for her first NIH appointment. The second photo is Sheena with my brother Ryan, who also has HPS. Ryan and his friend Julie are the unofficial NIH welcoming party a nd tour guides since they live in the area.

In my fantasy life, I secretly hope that one day some curious or enterprising young doctor or researcher develops an interest in Hermansky-Pudlak Syndrome, and then says to themselves, “Gee, I wonder what’s it’s like to live with HPS?” In this little fantasy, this doctor or researcher is enterprising enough to hop onto some blog search engine, like Technorati or Google blog search, and stumble upon either my blog, or that of one of the other HPS’ers. Far fetched, I know. Yet we in the HPS community strive to put a face to our medical maladies. So often the bench researchers doing the truly life saving and ground breaking work never get to meet someone with HPS (except for the mice.) We hope that by knowing us, they better understand just how important and valuable their work is to us. Donna Appell, President of the HPS Network, and her family even plan family vacations around which labs they’d like to visit – just so the researchers can meet Ashley, her daughter. So, it’s in that vein,

Just a quick update so no one thinks anything bad has happened to me. As wonderful as interns are, there’s still some tasks you can’t pass on because they’re just not trained yet – and the magazine is supposed to go to press on Tuesday. So, while my life since our intern is much, much better, this is just a super busy week. I was hoping to update the blog tonight – but I had a mission meeting at church and then came home to four phone calls. Now, it’s time for bed and I haven’t even started to do some of the stuff I was hoping to do tonight. Oh well. But, hey, I’m here and I’m doing fine. No worries! Well, there are some, but…..grin! Okay, I have got to go to bed.

This evening I’ve been working on designing a handbill for the HPS’ers that will be marching in the Puerto Rican Day Parade in New York City. I’m anxious to find out if this outreach effort yields any results – I can only imagine that it would. Hermansky-Pudlak Syndrome is the most common genetic disorder in Puerto Rico, affecting 1 out of every 1,800 Puerto Ricans. So, I can’t help but think that if we can gain a little exposure there, we’ll find some new people, either in New York or perhaps relatives of New Yorkers on the island. They’ve ordered T-shirts for the occasion and I think I’ve managed to get in on the act at least to get a T-shirt. They’re going to be red, and I think they’re going to say on the back “HPS Fighting for the cure with every last breath.” I’m also excited because my friends Carmen and Elsie have made some headway on outreaching to the local media in their areas. I still feel the pressure of the clock, however.

My little personal corner of hell, i.e. work, has just gotten much happier with the arrival of a summer intern. God bless interns! I can’t even begin to tell you what a difference it has made in just a week’s time. I actually worked a 40-hour week this week, occasionally made it to the gym, ate actual healthy home-cooked food and as soon as I get the bathroom clean, my house is reasonably in order. Imagine that! It’s almost a normal life! Our intern had an awesome first week. She learns fast. She needs very little supervision. She’s eager to do anything you ask. She doesn’t complain. She’s organized and efficient. I’m in heaven! She’s been able to do a lot of the things that just bog me down. For example, as much as I love daily news, it isn’t always terribly compatible with putting out a magazine. No matter what stage of production we’re in, I spend half my day running down news briefs. While our intern doesn’t know the industry, and doesn’t have the contacts to figure out what’s news

I’ve never been a big fan of mice. After a particularly traumatic experience with a Norwegian rat while living in Northern Germany, I’ve just had a kind of rodent phobia. Thus, it’s ironic that my future health is inextricably tied to colonies of little furry mice, passing their lives away in sterile little cages and tucked away in various labs across the world. While at the American Thoracic Society meeting in San Diego, the lives, and thus benefits, of these little creatures were a major topic of consideration. My very uneducated, and completely non-medical understanding of the debate is something like this. Scientists need mice to study potential therapies for pulmonary fibrosis, but as of yet, there seems to be no perfect mouse model. The thing about mice is they don’t live very long. Many genetic causes of pulmonary fibrosis develop over time, and mice that might have the genes to develop fibrosis typically die before it ever happens. Living in those nice little sterile cages, fre