Living on the frontlines

Here Dr. Markello is showing us this freezer full of nitrogen and "fresh" HPS blood samples. Earlier I'd asked Dr. Markello how it was they could use samples that were so old. I know that you can freeze things like this, but it still amazes me. He explained that they can take a blood sample out of here, thaw it out, and it's as if it had been sitting on the lab bench an hour.

This is one of the most special refrigerators in the world. This is the fridge that houses more than 10,000 blood samples from HPS patients going back 15 years. Dr. Markello is pulling out one of the trays to show us. Each one has more than a 100 little test tubes in it. The fridge is quite deep and there are several layers of samples. Just imagine, there are samples in there from HPS'ers that might not even be with us any more, but they're still helping research. It just goes to show how valuable each person is to the effort, and what a team effort is required.

This photo gives you a little better idea of what the place actually looks like. There are several of these little "areas" all in a row. (If I'd have only know we'd be taking more pictures, I would have put something else on - groan!)

Over the years I've been asked, probably a few hundred times, if I'd ever seen Dr. Gahl's lab. The answer was no. To be honest for some unknown reason I was dying to see it, but I never had the nerve to ask. I never, ever, wanted to be a pest, a bother, or take up a lot of time. But on this most recent trip the opportunity came to make a quick visit, and Debbie and I couldn't turn it down. It was, in fact, the highlight of our trip. Grin! Personally, I think it's kind of funny that so many of us have a curiosity about this place. I can't explain it. Perhaps it just makes us feel better to visualize it and know it's really there - HA! So, I'm going to post a few pics. A big thanks to Dr. Merideth for taking us, and for taking the pictures. This first picture is of Dr. Markello, me, Debbie and Angelina, who is one of the researchers. She is also from Puerto Rico!

As the saying goes, the third time is the charm. I've started blogging twice since arriving at NIH, but to be honest, I was just too tired to finish. Last night I started getting phone calls from some of you worried that perhaps I'd had some bad news. I'm happy to report that so far, all the test results are good news! I've just had a trouble time sleeping here, and thus went three days with about an hour of sleep a night. When I'm too tired to blog, you know I'm dog tired! Okay, so, let's get to the point. How are the lungs? Yesterday I had an abbreviated wrap up session with Kevin for the cameras. Thus, I still don't have all my test results. But, I do know that the preliminary pulmonary function test report was back. I couldn't be happier. When I entered the trial my forced vital capacity (FVC) was 82 percent of predicted. It went above 90 on the four-month and the eight-month follow ups. And yesterday, it was 94 percent of predicted, well into th...

I'm here! Although the morning at home was a bit bumpy, the trip went well. I should be dead tired, but I think the overall excitement of the day has me all stirred up. I think I may have to start asking for help with the airlines, however, which has me a bit bummed. It isn't really a vision thing. It's more an issue that traveling with all this medical "crap" is getting more complicated. TSA informed me today that the rules have changed for traveling with a CPAP machine. They don't just have to take it and test it now, but you have to remove it from the bag. So, there I was in airport security trying to take off my shoes, take out my laptop, take out my camera and now take out my CPAP with all of its cords and hoses - even though there was no line it took me a half hour to get through security. I was exhausted by the end of all that. And, not being able to see well, I felt uncomfortable having my belongings, expensive belongings, strewn from here to there ...

My cab should arrive in about a half hour. I’m a nervous wreck. I don’t get nervous about traveling, but I do get nervous about whether I’ve remembered everything I’m going to need, what will happen during the trip etc. I’ve been up most of the night. I had to finish several stories for work, although I’m not sure how great they were. My publisher might read them and think I’ve gone around the bend. To say I was a bit distracted by my trip would be an understatement. I also had to get the newsletter posted to the printer’s Web site so it could be printed while I’m away. The good news is we seem to have found a good deal on printing the newsletter. But, I’m a little nervous about the newsletter its self. First off, I had to cut some stories I really wanted to run because we had to keep the page count down to 12 for budget reasons. We had so much news about science and the various research protocols and the conference that I couldn’t run as many stories about HPS’ers as I would have like...

I need a break from my feature story, so thought I’d blog briefly about my doctor’s appointment on Wednesday. It was a mixed bag, and to be honest, I’m a bit confused. So, please take all of this with a grain of salt as I have a feeling I really don’t understand this yet. Regular readers will remember that right before my last NIH trip I started breaking out in hives, and that while I was at NIH, they got pretty bad. It was only one of several weird things to happen on that trip. Kevin gave me some medication that controlled the hives well, but knocked me on my butt! And, after about a month it seemed to me that the medication wasn’t just making me tired, it seemed to be affecting my memory. I was making really stupid mistakes at work etc. So, I went to see a local allergist/immunologist at KUMed and he put me on a pill form of Albuderol, Allegra and Hydroxychloroquine. I’d been on Allergra before, and it never seemed to do much for me. But, it seems to be working well. As part of my p...

I am dead tired. It’s been a long week. Yesterday I was worried that I wasn’t going to get everything finished that needed to be finished by the time I had to leave for NIH. My computer has been acting up again, and while I love my laptop, I’ve discovered it’s not ideal for marathon computer sessions (something that has me rethinking my new computer purchase strategy.) So, I stayed at work to try to finish up some things until 10:00 pm Thursday night. Today I felt like I was in the last leg of an endurance race. I’m so tired, and yet there is so much to do. I had to be sure I made some decisions about what we’re going to cover in the October issue so that our editorial assistant would have something to work on while I’m away. At the same time, since we’re down on staff, we’re trying to get some of the stories finished early to allow for extra glitches in layout and production. We’re using freelancers to help edit, and they work for other people besides just us – a fact I’m especially s...

First, let me apologise for not blogging more this week. I actually have a lot to say, but am very crunched for time. My boss had her baby on Monday, a little girl of six pounds and 18 inches long. She sounds small, but her mom is tiny. If she’s been any bigger I would have been worried! Grin! This means that at work it’s just me, our publisher, and our editorial assistant. I’m swamped. I’m going to be at NIH next week, so not only am I doing two jobs, but I’m going to be out a week. And, I ended up spending the better part of yesterday at KUMed for my immunologist appointment. I also finally got to visit Annette. So, here are just a few updates and teasers… 1. Thanks to everyone for praying for Annette. It means a lot to her. They’ve revised her diagnosis – they now believe she’s probably had leukemia for several months and that indeed there’s probably a genetic link. They believe that instead of AML that she actually has ALL, which involves the lymph nodes. They’ve told her that t...

I know that many of you must be sick of hearing about GINA – but folks, we’re almost there! The House of Representatives has passed this bill. An overwhelming majority of Senators have said they’ll support the bill. Now they need to step up and do it. Please write your Senators today and ask them to not only support GINA – the Genetic Information Non Discrimination Act – but demand that it brought to a vote! Help GINA progress through the SenateTell the Senate to take action on GINA! The Genetic Information Nondiscrimination Act, or GINA, (S.358) is on the verge of passing, after 12 long years! The House bill (H.R.493) passed 420-3 on April 25 and the Senate bill has been reported out of committee. We just need the full Senate to vote on the bill to get it to the President’s desk! Tell your senators to push for GINA to come to the floor for a vote! A list of senators and their contact information is below. Please take a few minutes to tailor the sample letter below on your letterhead a...

I’m just sitting here waiting on a phone call and thought I’d post a brief health update. I’m happy to report not much is happening on the GI front. I had a little trouble yesterday afternoon, but I sort of asked for it. I ate Mexican food for dinner. And, it was worth a little misery – yummy. In fact, now that I’m feeling better, my appetite is back. Boy is it back – can I keep the happy intestines, but send the appetite back? After a few weeks of not even being able to eat the minimum weight watchers points in a day, now I want to eat EVERYTHING! I feel hungry CONSTANTLY! This is just so unfair. If, after all that GI misery, I actually managed to lose a pound or two, at this rate I’m going to gain it back just in time for NIH. Perfect. Just perfect! Yesterday and Sunday I completely blew my allotted points – and frankly, not blowing the bank by 20 or 30 points was a major achievement of will power. I told Kevin I didn’t mind sharing everything with the film folks except one thing – w...

Today I’m feeling much better about Annette’s situation. Not that things aren’t very serious, but I’m really pleased with the care she’s getting at KUMed. I think she is as well. The doctors are telling her she has the AML type of leukemia, but that they think they’ve caught it early and they think it’s confined to the bone marrow. She’s been scanned and gone over every which way these past few days as the doctors check to make sure there isn’t any other cancer anywhere. So far every test has come back clean. They’ve also been completely checking out every organ system before they start Annette on chemotherapy. Everyone seems very upbeat about her long term prognosis, although it’s going to be a tough year ahead. The kids will be going to Nebraska to stay with their grandparents and will be enrolled in school there for now. They are telling Annette that even when she gets out of the hospital in about six weeks, she won’t be up to caring for two small children – not to mention the bugs ...

I’ve got a prayer request, and for a change it isn’t for an HPS’er. Annette, the mom of my Godkids, was diagnosed with Leukemia on Thursday. She’s been sent from the small hospital where she was admitted to the University of Kansas Medical Center (my hospital) and will probably be there for several weeks at least. Annette hasn’t been very well for some time. There’s a saying I’m told gets mentioned in medical school – if you hear hooves, don’t assume it’s a horse. It might be a zebra. Maybe it’s because I live in the land of the zebras, but something seems odd about this. Lord knows I know nothing about Leukemia etc. – but Annette has been collecting various diagnoses for years. She’s had Chronic Fatigue Syndrome and Fibromyalgia for years. She’s also had all sorts of digestive issues over the years. About the time I was diagnosed with HPS (the second time) she was diagnosed with Chronic Obstructive Pulmonary Disease. She’s been on oxygen on and off for the last year. Then, this year s...

I know there are several folks out there praying for me, and I must say, today I’m in a much better mood. I’m still frustrated with many things going on right now, irritated, perturbed etc. – but at least I don’t have the overwhelming desire to bite someone’s head off today. Grin! Sleep really does help. In the midst of my little crank fest during the last two days, I found an interesting article in the New York Times called Thriving After Life’s Bum Rap . It’s the sort of article that you could read in a good mood and feel all gushy about, or read it in a bad mood and want to throw things. When I first read it I was in crank fest mode, and boy, if I’d had the energy to blog, you all would have had an eye full! I ranted in my head and went from a horrible mood to a down right you-know-what mood. Today, rereading the article, my response is somewhat more tempered. The article is about cancer survivors who were able to turn their experiences of being super sick, of dealing with a life-t...

One side effect of being so tired all the time is that, well to put it nicely, I tend to get grouchy. If I weren’t trying to be family friendly, I could think of a better word for it. That would pretty much sum up me right now – irritable. I caught the early bus home from work. Why not? It wasn’t like I was getting anything done. Blogging is easy. I can blog dead tired, well, maybe not as well as I would if I were awake – but I can fake it. Writing something a bit more technical and dry, or worse trying to edit it, is something different, especially when you’re a bit bleary-eyed. The problem is compounded now as the once empty cubes surrounding me have been filled up with new employees hired to sell advertising for a new product our company is launching. Editorial and sales just don’t mix. They aren’t selling my magazine. It isn’t a conflict of interest thing. They are all perfectly nice people. In fact, the two women across the aisle from me I especially like, although I haven’t been ...

I think the lung gods have it out for me today. It was already 90 degrees and humid when I tried to catch the bus at 8:00 am. The bus was late, and while I stood and waited there were not one, not two, but three guys on lawn mowers trying their very best to mow along the street before it gets up to 104 this afternoon. (I am highly allergic to grass.) When I arrived at work there were more mowers, plus several construction dump trucks dumping dirt and moving it around. The dirt was all kicked up in the air so to get to my office building I had to walk through the dust storm. I don’t have asthma “attacks” exactly. I don’t feel like I can’t breathe. I just get tired faster when stuff like this happens. There’s no pressure feeling in my chest or anything like that. I was already exhausted, but before I even got to the stairs that go up into my building, I was taking deep breaths. Today at work I feel as though I’m in a battle to stay alert and awake. I just want a nap. Why am I so tired?? ...

Regular readers have heard me gripe about health insurance, or lack of it, on more than one occasion. While doing some research for a story at work today (on a completely unrelated topic) I stumbled upon this nifty little map, published by the Wall Street Journal , but compiled from data from the U.S. Census Bureau . If you’re curious about how many uninsured are in your state, check it out here . Personally, I think the crisis goes way beyond the uninsured – what about the under insured? I have a Hermansky-Pudlak Syndrome friend, for example, (I won’t use her name since I haven’t asked her permission) who underwent a procedure recently. The bill came last week. She owes 20 percent of the total cost, which comes to about $1,000. She’s on disability. I ask you, how is anyone who qualifies for disability supposed to pay 20 percent of a major medical bill? And since they didn’t find anything, how much do you want to bet it won’t be the last procedure? It’s a common story. Just when you fe...

Someday we should write a list of “You know you’re an HPS’er when…” If civilians could hear some of the conversations we have. I called Ryan over the weekend just to see how he’s doing. I knew his tummy had been acting up a bit – nothing serious, but for an HPS’er it’s always a cause for concern. “How are you?” I asked. “I haven’t had diarrhea for three days,” he answered. Funny how a question like “How are you?” instantly results in a pooh report. It isn’t just Ryan. I have several other HPS friends going through bowel issues right now, and almost without fail when I ask how they’re doing, the answer is some form of an analysis of their pooh. I call it “higher bowel awareness.” It isn’t as if we do this with outsiders. But when you have a chance to communicate with someone who understands, it’s hard to resist. You want to celebrate, or complian with someone who can appreciate how you feel. So, with that in mind, I’m pleased to report I’m going on day three diarrhea free. It’s been wee...

As you can tell, the video clip below won’t play. Oh well – makes me wonder why YouTube offered me the ability to post it to a blog if it wasn’t encoded. At any rate, if you’re really interested, I’d encourage you to go to YouTube , plug in the title of the clip, and watch it. There are three others from the same film up there as well. These are the only parts of the film I’ve seen, but I was interested to discover it up on YouTube because the clips come from a film called Worlds Apart created by the Stanford Center for Bioethics . This is the same organization currently making a film with the working title “Citizen Scientists” about the rise of patient advocacy groups and their impact on scientific research. These are the folks that will be joining me for part of my next NIH trip. The Hermansky-Pudlak Syndrome Network is one of the groups they are studying as part of this project. They are looking at the process we’re undergoing for the Pirfenidone trial. It’s interesting how the proj...

This is a clip I found on YouTube that I think was part of a film created by the Stanford Center for Bioethics - the folks currently working on the film "Citizen Scientist" (working title.) They are the ones that will be following me at the NIH on my upcoming trip. I found the clip very interesting because it illustrates some of the challenges we face in the HPS community. I'll blog further about that shortly.

(Warning to the guys, or to the pooh leery) I need to post another health update. I feel great tonight! I’ve been catching up on some personal business this evening because I feel so great (and because the ER rerun was just a little too intense for me – the first time was enough.) I’m really enjoying feeling great tonight because, frankly, I’ve felt rather crappy for the last few days – literally. I blogged a few days ago about the ongoing pooh issues. Over the weekend they actually got better. No Lomodil needed – everything was “poohfect.” It’s that time of the month, and I started having cramps again. The weird thing was the cramps seemed to always happen, once again, about 30 to 45 minutes after I ate. But, unlike my typical “runny” self, this time it seemed like nothing was working its way through. Back in the day my “Crohn’s” cramps often felt like really bad woman cramps – it was often hard to tell the difference. Tuesday morning I went to work and had been there only a few minut...

Do you know why they have control groups in drug trials? Because sometimes just doing something is therapy in and of its self. Sometimes patients improve just because they think they’re going to improve. It isn’t because they’re wacky. They’re just humans trying to survive. If this was something rare or unusual, control groups wouldn’t be necessary. But they are because we need to know if the patients on the drug really do better than the ones that just think they might be on the drug. The Pirfenidone trial I’m in is a double-blind study, meaning that I don’t know if I’m getting “the real stuff” nor do my doctors know. It’s all done by random numbers. No playing favorites. No emotion. Just the facts. When I was first admitted, I did really wonder if I was getting the real stuff. I tried not to, but hey, I’m human. I couldn’t help it. I remember looking at the pills one day and wondering if the term “sugar pill” was a literal term? Could there really be sugar in those capsules? Another ...

I’ve had so much to blog about lately, and not enough energy to sink my teeth into it. The press release from the Coalition for Pulmonary Fibrosis, coupled with my drug trial anniversary coming up, has had me thinking lately. Sometimes it seems surreal. Yes, I have the occasional day when I need my albuderol inhaler, or am having some other HPSish issue, but I don’t feel “sick.” Yes, my life is interrupted by health issues way more than it should be – yes I don’t always have the energy I wish I had – but I just don’t feel “sick.” I know plenty of people who are very “sick” and thus sometimes I hesitate to ever complain. I do complain here, mostly because I wouldn’t be keeping a very good record if I didn’t – but there are days I feel very guilty about it. Then I read something like the press release below. There’s nothing there I didn’t know already. It isn’t as if I somehow ever forget what I know about pulmonary fibrosis. I can rattle off the average life expectancy, the fact that th...

I’m so, so, so happy to report that according to Jeff, Sheena’s husband, she’s on the rebound. She should be headed out of ICU soon. They’ve pulled all her lines but the picc line. Now all they need is an empty bed on the regular floor. Sheena doesn’t like to be fussed over. She’ll hate it when she realizes that we were all so worried about her. Tough cookies! The HPS community is very close, and Sheena is a much-loved member of our little community. She has such a great sense of humor. She takes the world in stride, even when the world doesn’t deserve it. She’s been around the block, several times, on the medical front and is very level headed when it comes to managing her health issues. I’m sorry to say, that comes with way too much practice. Sheena’s mom and husband must be completely exhausted. I know this has been a tough time for them. When we were at our family reunion, Ryan and I kept calling and checking on Sheena. My mom doesn’t have much to do with the HPS world, but she’s a...

This is one of the latest press releases from the Coalition for Pulmonary Fibrosis. Just another reason why we need more research done on pulmonary fibrosis. Pulmonary Fibrosis Mortality Rates Continue to Increase, Especially in Women Demand for PF Research Funding Greater Than Ever SAN JOSE, Calif., Aug. 1 /PRNewswire-USNewswire/ -- News out today in a major medical journal predicts that mortality rates in pulmonary fibrosis (PF) will continue to climb. The finding underscores the urgent need for increased research funding in a deadly, untreatable lung disease, says the Coalition for Pulmonary Fibrosis (CPF). Study Findings According to the study appearing in today's issue of the American Journal of Respiratory and Critical Care Medicine, published by the American Thoracic Society (ATS), mortality rates from PF have increased significantly in recent years and have risen nearly 28.4 percent in men and at a higher rate in women, with a 41.3 percent increase. The study appears in the...

I need to post an update, again mostly for my own record keeping. It has not been a great week on the health front. I’ve had a lot of diarrhea. It does improve with the Lomodil, but I’m wondering if Lomodil is really meant to be a long-term solution for this problem. I’ve also had some mild cramping about 30 minutes after I eat most of the week. I’m also back to having joint pain which is worse in the morning or if I sit still too long. And I’m back to being TIRED! That’s so frustrating for me. It really impacts my concentration and makes it hard for me to get my work done. Today I’ve slept a good part of the day. During most of the week I only slept a few hours a night because I had to keep getting up to go to the bathroom in the middle of the night. When I’m having diarrhea at night it’s very hard for me to sleep. I’m afraid I’ll fall into a deep sleep and not realize my bag is filling up – and boom – there will be a most horrible explosion! Yuk! Again, none of this is severe, but it...

This is one of the video clips I took with my camera at Grandma's 90th birthday party. If I'd have known the stories would be so good, or if I'd have thought of it ahead of time, I would have invested in more memory cards for the camera. The video isn't very good. In this clip my mom, a preacher's kid, tells about trying to get to Colorado for a family vacation on a Sunday.

For a long time I’ve wanted to find a way to share some of my favorite music here. Music is a great coping technique for me. I’m quite the shower and living room (when no one is there) singer. It’s hard to be depressed when you sing. The lyrics of my favorite music have comforted me when I’m down, made me laugh, helped me get off the couch when I didn’t really feel up to it etc. But, I don’t want to “give away” someone else’s music. I just want to give a little publicity to some of the musicians I really like and share what I’m listening to these days. Then it hit me. I could set my camera next to the speaker on my computer and make a video clip (with no video) of a few songs. The sound quality isn’t great – but hey, if you think you like it you can go out and buy the CD and have much better sound quality. Grin! So, the song I picked for this experiment is by a Christian artist named Jennifer Knapp. She’s sort of a folk/rock artist. Her music varies from pretty “rock’in” to more of a f...

I still can't concentrate and am feeling really blue today. So, I hopped up on YouTube, my new favorite site, and started looking for something funny to cheer me up. I found this video. It's a comedy routine about having an ostomy. However, in between the jokes, it shows the comedian changing his ostomy bag. I'm just warning you! Some people might not really want to see this. To me, it's nothing. I do this about twice a week unless I'm leaking. Then I do it more than that.

Beware this video is graphic! Mom, you probably won't like it.

Today was not one of my better days. By 1:30 pm I’d been to the bathroom with the runs nine times since arriving at work. When I have days like this the health issues are bad enough, but the stress worrying about what will happen next is almost as bad. Most of the time my GI days are not this bad. But the past few days haven’t been great. My joints have been acting up. I didn’t take the Lomodil this morning because last night I was kind of stopped up – and what happens – but I spend my day in the bathroom. I felt tired. My concentration was horrible. My nystagmus gets worse when I’m really tired. I haven’t slept well in several nights. I could feel my ostomy itching, which is usually the sure sign of a pending leak, so then I was feeling anxious about having this diarrhea running all over the place at work. Finally my boss sent me an e-mail wanting to know what was up because I seemed really off my game. No kidding! I felt horrible. I get so worried about my job because of the implicat...

Sheena updateJust an update to let you all know that Sheena came through her surgery well. They didn’t have to do an ostomy so I know she’ll be happy about that. Go Sheena!