Living on the frontlines

Sea lions by Fisherman's Warf.

San Francisco was a bit chilly. One evening we were walking around and Ashley and Karen were freezing. We walked by a t-shirt vendor with really cheap sweat shirts, so to stay warm, they each bought one.

You'll have to forgive me for not blogging tonight. If I get a moment, I'll try to throw up some more pictures. This evening I'm working on a little snag that has cropped up in regard to the drug trial. It seems United Airlines and the Transportation Security Administration representatives at Dulles Airport are requiring our patients coming and going from Puerto Rico (in other words most of them) to have a U.S. passport. According to my sources at the State Department and the Department of Homeland Security, they shouldn't need to have a passport. After all, Puerto Rico is a U.S. territory and Puerto Ricans are American citizens. But, it seems the counter staff and the guys standing at security missed that part in American history class. So, I'm in the process of writing to both of these entities (after many phone calls today trying to figure out how to file a complaint). I'm sure we'll get it taken care of. But, the problem is occupying my time right now. P

Pictured here is Judy Simpson, President of the Pulmonary Hypertension Association and Past President of PAR. She served as MC for this event. Our first meeting at the American Thoracic Society (after we’d spent the morning eating a very fattening breakfast that certainly was not Weight Watcher’s friendly, but ohhh sooo good) was a first-time event for the society. PAR – the Public Advisory Roundtable – has decided it wants to reach out to the general public during ATS. I wasn’t in on the planning of this event, so I’m not 100 percent certain of all the details, but essentially it was open to lung patients of all stripes to get together and network. About 60 patients from the greater San Francisco area turned out to hear a mix of patient and doctor speakers. Afterwards there was a fofo food naushing and networking session. There was one doctor who gave an especially phenomenal talk. I’d love to give him credit, but I can’t even begin to spell his name correctly and I can’t find it writ

It seems I have a new disorder to add to my collection. Before I went to ATS, regular readers may remember, my thyroid numbers were off at the NIH, and that my local doc. had run some follow up tests here at home. I called today to check on the results and it seems the local doc. concurs with Kevin. They believe I have something called Hashimoto’s Syndrome. It’s actually not that rare, does run in my family and is very easily treated. I’ll start taking a thyroid hormone at a low dose at first to see if things improve. I was elated at the news, to be honest. Kevin at NIH (and pretty much everyone else) has been on my case about losing weight for some time, but especially since I entered the drug trial. And I have tried. Of all the diets I’ve tried, I’ve found Weight Watchers to be the easiest to comply with. I like the points system. But, even when I’m on my best behavior, carefully logging everything, exercising etc I never lose anything. In fact, I gain. With all of my other health is

Of course we couldn't go to San Francisco without riding a street car! We rode the street car to Lombard Street so that we could walk down the street instead of up! Grin! But, it seemed as though we had to wait forever!

San Francisco is known for its hills. Here's a look at just one such street, a steep hill, from a distance.

I don't know if everyone will be able to see this in the photo, but we found this interesting plant when we were wandering around San Francisco. The flowers looked like feathers. I couldn't resist and had to touch them - they were sort of sticky feeling.

For the sake of maintaining my symptom log, I’ve got to interrupt all of this great ATS news for a little whining. Grin. I think my body loves San Francisco. The weather was very cool, making it pleasant to wear long sleeves to protect from the sun if necessary. I’ve been told it’s like that all the time, although it’s hard to imagine. No wonder it’s so expensive to live there – it’s heaven! On the way to ATS I had a layover in Denver. I walked out of the jetway and almost instantly started sneezing, wheezing and breaking out in hives. (And I was already allergy medicated.) I had to dig out the abuderol. My asthma, however, isn’t like the asthma of most people I know. They have an “attack” of sorts. For me, I just start coughing a lot and feeling easily winded. I was completely out of breath walking from one gate to another – a trip that ordinarily wouldn’t phase me. But, once I arrived in San Francisco there wasn’t even a hint of allergy hell. At home, I never forget to take my allerg

Another thing we did on our free time Saturday morning was to go walking on Fisherman's Warf. This is just a photo from the side of the pier.

Here's a little better picture of Lombard Street (thanks to Wikipedia). While you can see the overall street better, it's a Web shot so the resolution isn't as good as the pictures I took - thus why I didn't upload it larger.

Here we are walking down Lombard Street.

Usually when Donna and I go to ATS, it's strictly a working trip. We're so seldom in the same location to work together that we work the show by day, and work on grants and other such HPS projects by night. This year, however, with Ashley and Karen along, Donna decided we needed to live a little. So, we did some limited sightseeing when time would allow. One evening we caught a cable car to the top of Lombard Street, a famous street in San Francisco know for being "the most crooked street in America." The street has been featured in a number of movies, video games and TV shows. Among them: The Love Bug (1969), and the video game Grand Theft Auto (not that I've ever played that game.) Apparently there's a yearly big wheel race down the hill. But on this day I think Karen, Ashley and I were the main attraction. The street has stairs for walkers, however the stairs are very uneven and Ashley and Karen are relatively new cane users. Not to mention none of us, es

Another pic of the 2007 ATS crew! As you look at us, can you imagine that at least twice someone commented to Donna about her three daughters? Poor Donna! Ahh, the power of suggestion I guess.

The booth at work! I must confess, we already knew the three docs in the booth in this picture. But, I wanted to snap a picture when I wouldn't drive anyone off with the camera - grin! But, there were several times when we had several doctors at the booth!

This is the picture of a big achievement for the Hermansky-Pudlak Syndrome Network. There are so many people who should be posing in this picture with us. We’re standing around a little sign that the American Thoracic Society gave us to put by our booth. It’s “thanking” us for participating in the ATS Partners in Research program. I put that in quotes because we are the ones who are thankful for the opportunity. It seems almost funny that they’d thank us. Essentially we had to commit to giving ATS $25,000 a year for two years. The money would be matched dollar for dollar by ATS and awarded as a grant to a promising researcher (this time it was Dr. Lisa Young.) That meant that not only did we have to get on the ATS radar as a worthy cause, but we had to come up with this money – a tall order for our shoe string budget. Getting the attention of ATS was a careful process. Four years ago Donna and Marie found out that ATS accepted scientific posters from patient organizations and that the

We're pointing to Dr. Lisa Young's photo on the pillar. We're so proud of her!

As I flew home from the American Thoracic Society, the entire plane could have been supported by the air I was floating on – squeeze me, squish me, make me sit in the middle seat – I didn’t care. I was euphoric. Coming home from Hermansky-Pudlak Syndrome events can sometimes be a downer. Suddenly you’re back in the real world where no one seems to understand the alternate universe that inhabits my brain – HPSland. I wanted to shout to the entire plane full of families, doctors, business people and senior citizens, jump up and down, and tell them what an event had just taken place. I’ll no doubt eagerly share some of the photos from the ATS show with friends and family, and they’ll politely look at them and probably never appreciate what they’re seeing. A while back one of the leaders in the ATS shared with Donna, our president, that early in his career he’d tried to publish a paper about HPS, but the disease was so rare, so obscure at that time, that the publication couldn’t find anyon

My HPS friend Karen T recently had a great post on her blog about “The spoon theory.” (Check it out at: http://www.myspace.com/karenshpskorner ). Essentially, the spoon theory is a tool for explaining what it’s like to live with a chronic illness. For people with chronic health problems, even those of us with relatively mild health issues at present, life isn’t the limitless set of choices most people wake up with every morning. Instead, it’s a process of budgeting your time and energy to live the best life possible. The spoon theory makes this point by giving a healthy person an allowance of spoons for the day. As the daily events unravel, the sick person explains what must be budgeted for, and takes away a spoon for each task. Donna last week had a similar educational tool. A friend of hers had made the point to her boss by putting a stickie note on her clothes every time something happened in her day that related to her disease. For example: Got up late because I couldn’t sleep. My

I have so much to tell about our American Thoracic Society trip, but frankly, I've run out of steam for the evening. So, just a picture tonight. This is Ashley Appell and Karen Tillman, our favorite "booth babes." Ashley is wearing some magnets that light up in her ears that we got from the National Heart Lung and Blood Institute booth. They lit up and blinked and were a great attention getter - they made people stop just long enough to start a conversation about HPS. Karen and Ashley did a great job manning the booth!

Those of us in the Hermansky-Pudlak Syndrome community know not only what a great doctor, but also what a great person, Dr. William Gahl is - so it's no surprise to us that he was honored this year by the National Organization for Rare Disorders (NORD). Below is the announcement. I know many of you are anxious to hear how the American Thoracic Society meeting went, and I'll be posting more about that shortly. One of the things that was truly amazing about this week, however, is that the HPS Network was making those little steps towards the cure on both coasts this last week. While we were at ATS, the Network was represented by Ryan (my brother), and Kristie and her mom Jackie on the East Coast. They went to the dinner in honor of Dr. Gahl and the other award recipients. Ryan and crew got to also meet Dr. Gahl's wife and two of his four children. I didn't know this, but Dr. Gahl has a son serving in Iraq. There's another thing to add to our prayer list! Honorees Anno

I’m being haunted by lawn mowers. They’re everywhere I go as if they’re chasing me. Kansas, after all, is a prairie, even in suburbia. This morning was a lovely morning, cool yet sunny. As I stood waiting for the bus, there was a man mowing that little strip of grass between the street and the sidewalk. My eyes started to well up, my nose started to run. I didn’t dare go out for lunch and leave the nice, air-conditioned air of the office building. When I caught my bus this evening there were a bunch of men on those giant industrial mowers, the kind where you stand on the back, mowing right by the bus stop. And when I got off the bus at the dry cleaners to pick up my suits, and then walked the mile home – it seemed as though every block had at least one lawn mower going. This is crazy. I’m supposed to be dealing with “real” medical issues here. Or, I’m supposed to be living in absolute glee that I’m not dealing with “real” medical issues. How is it possible that it seems as though my wo

One thing that separates ultra-rare disorders from other health problems is that the experience of seeing a new doctor for the first time can be stressful, even scary. Yesterday I saw a new family doctor for the first time. I prepared for the event as though I were taking a final exam. When I made the appointment, I’d asked for more time than the usual 15 minute block because I knew I had a lot of ground to cover. I expected I’d have to explain Hermansky-Pudlak Syndrome, what it is, and where I am in the disease process. Then I’d have to explain the drug trial and the study drug. Then I had all the test results from NIH to go over, the new medications, the abnormal thyroid findings, and the ongoing allergies, diarrhea, hives and fatigue. And after all that, I’d need this new doctor to fill out my FMLA paperwork. It was a tall order, and after some cajoling I did get the scheduler to give me a double slot. How nice it would be to be able to walk into any doctor’s office and have them kn

Below is a press release from Intermune, the company that makes Pirfenidone. Not only is this drug being tried for idiopathic pulmonary fibrosis but also for the pulmonary fibrosis of Hermansky-Pudlak Syndrome. Just as good news from our trial is good news for the greater pulmonary fibrosis community, so is good news about their trial also good news for us. The sooner this drug is available for those that need it, the better. InterMune Completes Patient Enrollment in Phase 3 'Capacity' Program Evaluating Pirfenidone in Patients With IPF BRISBANE, Calif., May 16 /PRNewswire-FirstCall/ -- InterMune, Inc. (Nasdaq: ITMN ) today announced that patient enrollment has been completed in CAPACITY, the company's Phase 3 clinical program to evaluate pirfenidone as a treatment for patients with idiopathic pulmonary fibrosis (IPF). "We are very pleased to have completed enrollment of the CAPACITY program seven months ahead of the original schedule, and with 194 more patients tha

At the end of the week I’m leaving to go to the American Thoracic Society to do some advocacy work for Hermansky-Pudlak Syndrome. I’m going with Donna, our President and founder, and Karen, a very good HPS friend. Yesterday I talked to Donna about last-minute preparations for the trip. “Are you ready?” she asked. “Yep, I’ve got my doctor’s appointment on Wednesday so hopefully I can get the drug situation sorted out,” I answered. “Ashley’s got her appointment on Thursday,” Donna responded. “Karen’s already been to have her pyoderma checked out, just in case she needed a shot before traveling,” I answered. We then both started laughing. Most people think about packing clothes, shampoo etc. Here we are carefully planning our medical strategy for being so far away from home. This isn’t normally an issue for me – but I’d like to avoid a hive and wheezy episode. We’ve got enough excitement planned, thank you very much! These days when I travel, even for just a few days, I feel like I’ve got

Kevin called today to touch base about my digestive issues, joint pain, back pain and of course, the hives. I gave him the low down, including telling him about my little medication strike. Of course, he told me to cut it out. Get back on the meds. You’re going to have an asthma attack. You’re going to start having bronchial spasms. Stop it. I was ahead of him. I’m back in dopyville. Kevin had the results of some of the tests NIH did that had to be sent to outside labs. The great news is what ever is wrong, it’s nothing too serious because all the big, bad auto-immune stuff all came back normal. Yeah! My thyroid, however, is up to no good. I’ve got to get some more tests done at home. Kevin’s got a theory about the problem, but before blogging about it, I want to call some of my family members and ask some questions. It would be another genetic problem. Guess I was swimming in the wrong end of the gene pool up in heaven. Grin – just kidding. Actually, I’m hoping Kevin’s thoughts are co

Sometimes I wish I was a better person. But, I’m not. I am who I am and where I am, whether I like it or not. Today, I must confess, I skipped church not because I didn’t feel well (as has been the case a lot this year) but because I just wasn’t in the mood for Mother’s Day. I don’t have children, and all my “mother-type” relationships these days are a bit strained at best. I wish I could have focused on the times when that wasn’t the case. I wish I could have been more grateful for the past, for all the things my mother and my grandmother have done for me (it’s quite a list) and for those times when we were close. I wish I could have looked around the sanctuary at all the moms and been happy for them. But, some days I’m just not in the mood to fake it. Bah-humbug on motherhood (no – I don’t mean that – it’s just an emotion.) Whether we’re in good health, or going through a tough patch, Hermansky-Pudlak Syndrome often takes a toll on our lives in ways that might not always be seen by t

I am not my mother’s daughter in many ways, and one of them is that unlike my mom, I HATE the mall. I’m not a big shopper. When my mom comes home from Germany, she typically wants to spend days on end at the malls. I try to tell her that they’re all the same – they’ve got the exact same stores with the exact same merchandise no matter where you go – but she’s convinced that if you visit enough of them, eventually you’ll find something new. Because the exchange rate isn’t to the dollar’s advantage, she’s stocking up for two years. But that’s just her excuse. She truly loves the hunt. I, on the other hand, dread it all year. Today I made my annual trip to the mall. The list of things I needed was getting scary (expensive) and long. I needed new tops for work, new pants, new dress shoes, new exercise shoes, new night gowns, and new undergarments. Somehow I guess I thought that if I put off going long enough I could get all of these items in one trip. Wrong! I also thought that on a Sunday

This week, about Thursday, I started doing something very naughty, something I constantly tell everyone else not to do – I stopped taking my medications (except for the study drug). Even though my bowel has been better, and the hives and the joint pains – I’ve been functioning like a zombie. I try to read a paragraph to edit something, and I can’t remember at the end of the paragraph what was at the beginning. My boss is being very patient and understanding, but this isn’t working and eventually something has to give. I NEED to get some work done so I can go to American Thoracic Society at the end of next week relatively guilt free. I looked up all my medications, and what do you know but six of the 11 meds I take cause drowsiness. HELLO! So, for two days I quit taking them just to get a little clarity in my head and a bit of alertness for a change. It helped, although more so on Friday than on Thursday. This morning, however, my medication strike caught up with me. At about 4:00 am I

Below is an article that appeared in the local Springfield paper about The Albino Farm movie. After reading it, I was left shaking my head. As a person with albinism, there are a lot of things I wish had been included, made a bit clearer etc. but as a reporter I know how incredibly hard it is to delve into a subject you likely know little if anything about and churn out something useful in typically just a few hours. And hey, articles that appear in print have space limitations. I’m just happy the issue got covered at all. I was shaking my head, however, at the response of the movie’s producers. They don’t get it. Those that know me best know that I’m not exactly a stickler for political correctness. I’m more concerned about what people mean by the words they choose rather than the words themselves. On the “albino” issue, I won’t slap someone down for calling me “an albino” unless in context they mean it as an insult. Then, it’s “on” if you know what I mean. I prefer “person with albin

The following article appeared in the Springfield News Leader. Thanks to the paper for covering the story. It seems the movie's producers just don't get it. I'll comment on this article more when I'm at home. Just sneaking it up while on my lunch break because I know many of you were wanting an update. Movie on albinism has town buzzing Plans for a new movie entitled "The Albino Farm" prompts mother to protest, but a local student is looking forward to the show. Post Comment Springfield native Sean McEwen is gearing up for an end-of-May start date for filming his new low-budget horror movie, "Albino Farm," which takes its title from the Springfield urban legend. The legend takes its name from the supposed sightings or incidents involving people with albinism — those who inherited a condition giving them little or no pigment in their eyes, skin or hair. The folk tale has survived in several versions, but all revolve around a large farm north of Sprin

There are so many things to blog about, and so little time. Tonight I am completely exhausted. The good news is I feel a lot better today. Yes, I've still got joint pain and some back pain, but it's nothing. I actually felt clear in the head today and was able to get a lot done for work - in fact I worked an eight hour day and then came home and put in another four hours. That's why I'm tired and not blogging about all the things I want to right now. I also ate like a normal person today (actually too much probably) and had no tummy cramps or strange poo. Everything was wonderfully normal. But, that's the way bowel stuff with HPS can be, and why I worry about catching it early. It's often subtle and fleeting, like trying to shoot at a fast moving target. But, I did want to leave the url for Leroy's cancer blog, firstly because I'll probably be talking about it some over the next few weeks, and secondly becuase it's just really good. When I started th

This past February, when the conference was over and most people had left for home, I sat in the hotel bar with Donna, her family and friends that had helped out with the conference – and with the producer of the documentary film crew Nicole. I sat next to Nicole and we chatted about various project we’d worked on. I was telling her about covering the first major trade show to return to New Orleans after Katrina. I joked that Anderson Cooper was in town to speak at the show, and he was staying in my hotel. If I hadn’t been so busy covering my own story, I probably would have been trying to “run into” Cooper. I told her that secretly I returned to the hotel every night and prayed Cooper would be in the elevator when I went up to my room – just so I could have a few moments where he couldn’t escape to try to get him to do a piece on HPS. “What would you have said to him?” she asked. A reasonable question, but I didn’t have a reasonable, well thought out, articulate answer. I couldn’t con

Blogging is the absolute last thing I should be doing right now. I’ll pay for it this week. But, my joint pain is almost non-existent tonight. I’ve eaten a real meal and while it still might cause the runs, at least it isn’t painfully moving through my system. And, I’m feeling like ranting a bit. I’ve been trying to work on my stories for work, but until I do some ranting, I’m sure I won’t be able to concentrate. I blog about almost anything related to Hermansky-Pudlak Syndrome on this blog – right down to the color of my poo sometimes. I want to create an honest picture of HPS, and honestly many of us with HPS spend a lot of time on such issues that seem just too personal or irrelevant to an outsider. But there’s a part of the picture missing. There’s parts that I hesitate to blog about. I don’t mind sharing everything, but those closest to me, and other HPSers, don’t always feel the same way. I often leave out parts of the story trying to protect people, even on days when I don’t thi