Living on the frontlines

Regular readers will have noticed – I haven’t exactly been blogging a lot lately. I’m sure you won’t be surprised when I say a lot has been going on here. I had such a perfect report from my trip to NIH, and yet every since I’ve been back it seems like it’s been one thing after another. Dizzy spells, bleeding sores around my stoma etc. We are so short staffed at work and each problem is more time away from the job, as well as from all the things that are important to me. My apartment is filthy. I finally made it to the grocery store and got some healthy food, but I’ve been living on vending machines and takeout for weeks now. Obviously, that can’t be doing good things for my health, but I’ve either been sick, or in such a frantic state trying to catch up that two hours to walk to the grocery, shop, and walk home have seemed like a luxury I couldn’t afford. Work is stressful anyway, but when you feel like you’re down in a pit, and every time you try to climb out the earth just gives way

This week is a big week in HPSland. This coming weekend is the second annual HPS Family Conference in Puerto Rico. I can’t tell you how much I wish I was there. Just as they did last year, HPS Network Puerto Rico has done an awesome job generating media interest and putting the conference together. It’s also an exciting conference because nearly all of our doctors from NIH will be there. That is a real testament to their commitment to us – I know it wasn’t easy to make that possible. A very special thanks to Dr. Gahl, Dr. Markello, Dr. Guchicco, Dr. Merideth and Dr. Yao for being willing to invest so much of their personal time on this project. Kevin, our nurse practitioner, and Jose, the scheduler a.k.a translator, a.k.a problem solver (think Radar and Clinger from M.A.S.H.) have been in Puerto Rico since this past weekend. They’ve been traveling the island with Hilda, a nurse who has worked with the HPS patients at NIH for years. She retired last year and now works with us as a volun

The past two weeks haven’t exactly been the best weeks. While I’ve had some health problems, thankfully I’m not really “super sick.” Instead, I seem to be annoyingly sick. Maybe sick isn’t even the right word. As I had mentioned before, week before last I was at work and suddenly had these dizzy spells and felt as though I were going to faint. We guessed that maybe it was the antibiotics I was taking for a staph infection. Since I’d already been on them six days, we decided to quit them and see if the dizzy spells went away. They did, sort of. I slept huge chunks of last weekend. I have a tendency to sleep when I’m sick, or about to get sick. But, given my stress levels lately, I chalked up the 12-hour naps to pure exhaustion. And maybe that’s right. Maybe I’m just worn out. Monday went fairly well at work, but about a half hour before I was supposed to leave to go home I felt “the itch.” It was a “code brown” – an ostomy leak in progress. Since it was so close to the end of the day, I

Don’t worry, I’m not lost. I am, however, having some health issues, life balance issues, major stress issues etc. So, that’s why you haven’t seen much blogging lately. Today I had to come home early from work, once again, thanks to a “code brown.” We’ll blog about that later. But, while I was laying on my bed, upset and in a bit of pain, I was listening to NPR and heard the most delightful segment on Talk of the Nation . It helped me calm down. They were talking about lost and found items . They interviewed the guy in charge of lost and found at Grand Central Station about all the wacky things people have lost, and the extent to which they’ve gone to find the owners. Another guy was interviewed who has started a magazine called Found that looks like a blast to read. I’m going to have to check it out further. Essentially, his magazine is full of stories about found notes, lost items etc. They were very interesting - some sad and some funny. If anyone’s interested in little entertainin

I was a bit sorry I had to mark such a huge event, the opening of the GI protocol for Hermansky-Pudlak Syndrome at the NIH, with such a quick post the other day. So, here are some more thoughts on this subject. First, if you’re interested in more information, you really need to call Donna at the HPS Network. If you call or e-mail me, I’m just going to tell you to contact her. I wasn’t at the meeting she had with Dr. Yao about the specifics of the protocol, so what I know about it is general information only. The other quick little disclaimer I want to throw out here is that these are just my thoughts as a totally non-medical person. So, if anyone out there wants to correct me or take exception with my thoughts on this subject, please have at it. The opening of the GI protocol is indeed a big event for us. Huge! Exciting! A major step forward! I’m thrilled. A few of you wrote to ask me if this was indeed the first HPS GI protocol at NIH, remembering a call for volunteers that went out

Do you ever wonder why you are where you are today? How did you end up in your chosen field? What attracted you to it in the first place, and would you have made a different choice under different circumstances? Recently my friend Tina was a kind of mentor/camp counselor at a special summer “camp” for blind and visually impaired kids interested in science, engineering or mathematics careers. I wish I could have been a fly on the wall during the week of exploration into all sorts of things such as astronomy, chemistry and biology. The idea of going to “science camp” would have never caught my interest back in my school days. I would have avoided it like the plague. Today I find myself wishing I had a stronger foundation in these areas – thanks in no small part to this HPS mystery brewing inside me. I guess I fit my chosen profession, journalism, in one respect – it drives me nuts to have a question, and not be able to find the answer. After returning from NIH I was chatting with my mom

First, let me apologize for the quick and dirty post. (See last entry – grin!) I really want to blog at length about this, but I’m under some big bad deadline pressure right now. It will have to wait until the weekend. But, I do want to get the word out that the GI protocol is open. This is exciting news as it’s the first treatment protocol at NIH devoted to the bowel issues of Hermansky-Pudlak Syndrome. The tough part about it, however, is that they are only accepting 10 patients and those 10 patients have to meet a pretty long list of requirements, plus not have any of the long list of exclusionary criteria. That means that not everyone I’d like to see get into this protocol is going to be able to do it. If you do have bowel issues related to HPS, and you are interested in going to NIH for treatment, please contact the HPS Network and check it out. It can’t hurt to find out if you’d meet the criteria, and even if you don’t, it’s helpful for us to know you’re out there, you’re having

I feel like one of those bobble toys where the head spins around and then pops off. My phone was ringing with Hermansky-Pudlak Syndrome calls before I even got out of the shower this morning. Frankly, that was the highlight of my day. I love working on HPS things, even on the days when the calls are tough and emotional. And today there have been some tough and emotional things going on in HPSland. I feel like it’s what I’m supposed to be doing. But that doesn’t pay my bills. Work pays the bills, and work is a big stress pit right now. I can’t get into all the reasons why, but the fact that my boss is on maternity leave and our editorial assistant, while eager and smart, is a bit wet behind the ears, isn’t helping. It really would have been ideal if we’d been able to hire him well before my boss left for her leave. He needs to be trained, and I’m doing a lousy job of training him because I can barely keep my head above water. Our publisher is running the ship while our editor-in-chief i

Since stopping the antibiotic I’ve been feeling better and haven’t felt like I was going to pass out. That was scary. I’m not real interested in doing that again. I have been very, very tired, however. Maybe fighting something off? Who knows. But then last night I was sleeping soundly, even dreaming, until I woke up about 3:30 am gasping for breath. I seem to have a talent for being able to unfasten all four clasps on my CPAP mask and toss it across the room without ever waking up. Although my current mask is the best one so far, I still have a tendency to do this. I like to sleep on my side, and I have a feeling that the mask’s seal somehow gets unsealed, and in my sleep I find the escaping air annoying. So, off the mask comes. When I woke up I was maskless, so I suspect I’d had some kind of apnea event and my body, trying to breathe, woke me up. Just a theory mind you. The weird thing was I couldn’t seem to catch my breath. I was breathing okay, yet I had this feeling like it wasn’t

Below is a press release from the Coalition for Pulmonary Fibrosis regarding an advertising campaign they are launching to try to get Congress to approve more funding for research on Idiopathic Pulmonary Fibrosis? Bravo to the Coalition! I’ll let you read the release, and then I’ll make some comments at the end. Coalition for Pulmonary Fibrosis Launches Aggressive Ad Campaign Directed at United States Congress Organization Urges Congress to Increase Federal Funding for Deadly, Incurable Disease Click Here to View our Advertisements San Jose, Calif. (Sept. 4, 2007) -The Coalition for Pulmonary Fibrosis (CPF) is launching an advertising campaign aimed at Members of Congress in support of its National IPF Awareness Week Sept. 22-29, 2007. The campaign, which includes both print and online ads, will kick off on Sept. 6 and urge Members of Congress to take action to increase research funding for idiopathic pulmonary fibrosis (IPF), and to improve education and awareness of the disease in th

The last few days had the sort of excitement I could really do without, thank you very much. I’ve missed another two days of work and given that we’re so short staffed, this is not good. Wednesday evening I ventured down the street to the drug store about a mile away. I felt so tired. The drug store has been under renovation for nearly a year. I think someone is making a career out of the project. That means that every time you go to get something, it’s been put in a different place. And this time the mission was of a female nature. I went to the pharmacy and tried to get the attention of the female pharmacist, but she was busy so the guy had to help me. He had an African sort of accent and couldn’t seem to understand what I was after. How do you explain what a maxi pad is to a guy who doesn’t seem to speak English? He took me over to the bladder control section. This was not working, and I was feeling increasingly tired and getting cranky. So, a female manager stepped in to help, but

The National Organization for Albinism and Hypopigmentation (NOAH) has picked up this blog in the blogs section of their Web site. I was very pleased and honored that they asked. I must, however, warn any new readers that, while I’m thrilled that anyone would want to read this blog, my intended audience is the HPS community. HPS is a type of albinism, and thus many of the issues I encounter in life are the same as anyone else with albinism. Because HPS involves other health issues, however, sometimes this blog gets pretty frank about topics that not everyone would talk about in polite conversation. Non- HPS ’ ers might be a bit rattled by some entries. And sometimes entries have nothing to do with albinism, HPS or health. Grin! This is, after all, my personal blog. I also want to point out that not everyone with HPS has the same types of health problems that I do. There are eight known types of HPS (and probably other types not yet discovered). I have HPS type 1. You can read

I must confess, I know nothing about hockey. Back in my much, much, younger days I was a figure skater. Yeah, I know, hard to imagine, but it’s true. To me, hockey players were those obnoxious boys that raced around the rink and messed up the ice. They’d skate around you as close, and as fast, as they could. Not being able to judge distance very well, it used to scare the begeebies out of me. But, I think I’m about to become a hockey fan. Regular Hermansky-Pudlak Syndrome conference attendees might remember Barry from Canada. He wasn’t at conference last year, but has been several times. He’s from Nova Scotia, Canada. Barry is a huge hockey fan, and growing up where he did, happens to know some professional hockey players. He reached out to Joey McDonald and asked for his help to promote awareness and do some fundraising for HPS. Joey graciously agreed. Joey now plays for the Long Island Islanders, the team that plays in the arena right across the street from the Marriott where the con

For those of you that wonder why I have a google ad on my blog, I assure you, I’m not trying to make a few extra bucks off of HPS. For several years my friend Tina and I organized a trivia tournament here in Kansas City in partnership with the Alpha Lions Club. Half of the revenues went to the HPS Network, and the other half went to fund blindness-related causes the Alpha Lions Club supported (mainly the Adventure Fitness Camp Fire program for blind and visually impaired kids.) Last year we decided not to do the tournament. Tina was stretched too thin in her life, and I certainly was in mine. But, I didn’t want to lose the revenue the fundraiser created for the Network. I wanted to do my little part, and frankly, I’m not flush enough to write big checks. So, I decided to put advertising on my blog. I blog every day anyway, so it required no extra work on my part. The ad raises a pretty steady average of about $120 every four months. Not mega money, but in a year it’s about what we rais

There’s one picture from our little trip to the lab that I’m not going to post. Dr. Markello introduced us to a researcher named Heidi who he referred to as one of the silent heroes of HPS research. Heidi looks at our cells in a special microscope that shoots a laser through them so they light up brilliantly. The microscope was housed in a little room in the lab, and it was hard for all of us to squeeze in there to see it – let alone for Dr. Meredith to have enough space to back up and take a good shot. Heidi fired up the microscope and showed us both a “regular” cell and an HPS cell. The dense bodies showed up as this neon orange, and the other parts of the cell were glowing neon green to yellow. You could really see the difference in the two examples. It was actually pretty easy to see! And I really, really wanted to see it. My nose was right up on the screen, so all poor Dr. Meredith could get a shot of was my rather large backside. I was so absorbed in the moment that I forgot she

Last night I slept. I finally slept well (and without the CPAP - shame on me.) Poor Ryan was treated to a night of what he's dubbed, " Snorty the dinosaur." But, I felt great today! Ryan and I met a friend of mine from college, Leanne and her husband to be, Brian. We had a wonderful day (pictures to come later.) We walked around Tidal Basin ( sp ?), through the Roosevelt Memorial and back. Then we got on the Metro and went to a great restaurant, Ebbitt (one of the oldest in the area and I'm not sure if I'm spelling that right either) right around the corner from the White House. So, although I've done it several times before, I had to go get my picture taken again in front of the White House. We were celebrating my great test results. I'm definitely in the mood to celebrate. Just like when I entered the trial, it seems like bands should be playing and confetti should be falling from the skies. Ordinary people can't possibly appreciate how great I fe