Living on the frontlines

I feel great! I feel better than I’ve felt in months! Friday night I cranked up the stereo, broke out the cleaning supplies, and started to work. I managed to get the kitchen and living room done before the smells and dust got to me. And I sang. I sang, panting sometimes from the cleaning stuff, but I belted out the words to some of my favorite songs as if I were on Broadway. I’m sure my neighbors really enjoyed the show! But, after my neighbor’s party the other night, his big screen television with person-sized speakers (in a little apartment) and his own singing kicks – I figure paybacks are hell buddy. So, this is my confession for the week. I love to sing. Mind you I don’t really have a voice, or any musical talent to speak of – but when I’m feeling down singing almost always makes me feel better. How can you be depressed and sing at the same time? And Friday night I was so happy to have the energy to do more than be an ornament on my couch that I couldn’t help but sing. Oh how I e

6:30 a.m. I look at the clock and leap out of bed. I’d set the alarm for 6:00 a.m. because I wanted to get to work early. I’m behind at work, thanks to the bout of fatigue, and I want to get to work early to catch up. Leaping wasn’t such a good idea. As soon as my feet hit the ground I feel aching in my ankles. Sometimes I get joint pain in my ankles, knees, wrists and fingers – it’s a mystery that comes and goes. So far, no one’s been able to explain why. This morning my ankles hurt worse than usual. As I try to rush to the bathroom one of them buckles underneath me and I fall into my Gazelle (exercise machine). My leg scraps against something on the side and now there’s a scrape on the upper part of my thigh about the length of my thumb. It’s not deep, but is bleeding. I stumble to the bathroom, get the appropriate gauze to hold pressure, and then stumble back to bed and listen to NPR. It’s now 7:00 a.m. I’ve missed the early bus. I can still make the next one. 7:50 a.m. I’m missing

The following is an article that ran in today's edition of the Reidsville Review in North Carolina. It says it all. Hmmm....although copy editing isn't my strong point, perhaps I could get a job as a copy editor in Reidsville. But hey, we're thrilled to get some coverage on HPS! PRAYER, LAUGHER THE ANSWER Reidsville woman battles rare disease. By Miranda Baines Staff Writer Wednesday, March 22, 2006 Reidsville native Karen Tickle Tillman, who now makes her home in Greensboro, is beating the odds in her struggle against a rare disease, Hermansky-Pudlak Syndrome. Tillman said most people diagnosed with the disease do not survive past their late 40's or early 50's, but at 41, she is optimistic. "I've always been a fighter," asserted Tillman. What is HPS? HPS is a rare genetic disorder that was discovered by two Czechoslovakian physicians in 1959. The disease causes albinism, vision impairment and a tendency to bleed. Tillman said only 800 people worldwide

I got my test results today. Chest x-ray was clear (although I’m still wondering where the wheezing is coming from if everything is okay). Blood work couldn’t have been more perfect. In fact, it was better than usual. My thyroid, which flirts on the fine line of being problematic, was well within the normal range. Hemoglobin was 13.6 – perfect. And, I’m feeling better. Maybe it was just a virus. I’m going in for a sleep study Sunday night, however. Apparently I should be having one every couple of years anyway – news to me, but it does make sense. All and all good news, and now I’ve got to turn my attention to the huge pile of stuff I’m behind on because I couldn’t function last week.

I feel like I’m starting to emerge from the fog. I actually only yawned a few times at work today, although my concentration was still lacking. That feeling of constantly wanting to do nothing but lie down and sleep is so frustrating, and it’s been so nice to have a day free from it today. More bombshells on the work front – but I’m not going to get into that here. Yet, even with those I didn’t want to go lie down and tune out the world. Hey, this is progress! I’m still very worried about whatever the doc. thinks he heard in my lungs on Friday though. Of course, no phone call today with any test results. AAAAHHHH…..this is my big doctor gripe. I feel crappy enough to haul my rather large backside into the doctor’s office, shell out money for cab fare and take time off work – so obviously I’m quite concerned or I’d stay at home, eat chicken noodle soup and wade out the storm. But, if your test results come back and nothing much is wrong, you often never get a phone call. Maybe I’ll get

Friday was a bad day. There are the kind of bad days where nothing at work goes right, or you break your small toe, or maybe your dog pees on the carpet and you burn dinner. Friday wasn’t that kind of a bad day. It was much worse. The trouble is it may be weeks before I can publicly explain it all on a blog. The part I can’t explain isn’t directly about Hermansky-Pudlak Syndrome, but as in so many things, HPS colors my reaction to it – and my strategy for dealing with it. Part of Friday’s bombshell (there was more than one) does have to do with HPS. Friday I went to the doctor to whine about my fatigue officially. I really like this doctor. I’m glad I held out and didn’t go to see anyone else just to get in earlier. He didn’t pat me on the head or make me feel silly. Instead he ordered what he termed “the fatigue work up.” He took blood, ordered another sleep study to see if my CPAP settings are still correct, and then he listened to my chest. As he moved the stethoscope around he kept

Wouldn’t it just figure that after a day of barely being able to function, it’s almost midnight and I feel the best I’ve felt all day – alert and not as though I’m living in the clouds. I skipped church tonight just to take a bit of the pressure off and give me a bit of time to lie around. But, we’ve got several grants for the HPS Network due, and they’re too big a funding opportunity to be caught lounging around. Donna has been stuck with most of the work, despite my many promises to help, and I feel terrible. I feel doubly terrible because I know how much she’s done for all of us when she was way too ill to be working. Today I finally broke down and made a doctor’s appointment to go in and whine about my fatigue. Not only am I not due for a visit for another month, but I dread going to the doctor with the simple complaint that “I’m tired.” I have a feeling he’ll ask me a few questions, tell me I have a virus and to quit doing so much, pat me on the head, and send me home. That’s espe

Nope – I haven’t disappeared. Nothing’s wrong. I’ve just been super tired. So stinking tired! I’m so stinking tired of being tired. But, as I haven’t posted anything here in days (as if anyone is reading this anyway) I thought I might surface long enough to announce all is okay with the world. The thought occurred to me that this might be a good idea after sharing with the very first person, a fellow HPS friend, that I was toying with this blog. “What do you think of the idea?” I asked after a lengthy explanation about my ill-defined goals, purpose and concerns that this would become a Hermansky-Pudlak Syndrome whine fest. “I think it’s a great idea!” she so nicely replied. She then told me of a Web site she’d found shortly after her diagnosis that a man with pulmonary fibrosis had created. He was trying for a lung transplant and was using the Web site to tell his story. “Only thing is,” she explained, “The posts just stopped. It was kind of depressing. He probably died.” So, lest anyo

“I’ve had this cough since before Christmas,” said the lady sitting across the table from me at church on Tuesday. I looked at the pork chop and apples on my plate. “Really?” I replied. Panic is what I thought. My mind was racing to find a tactful way to move tables when the woman sitting beside me announced that she was getting over pneumonia. Great, I thought. I’m surrounded. Our experts in Hermansky-Pudlak Syndrome strongly warn us against getting colds, and especially respiratory infections. They believe the fibrosis in our lungs may result from insults like these that happen slowly over time. Avoiding colds and coughs could, over time, ensure I’m around a bit longer. “Don’t be afraid of offending people if you need to get up and move away from someone coughing,” Kevin, our nurse practitioner told me in one of his “talks” during a recent visit to the National Institutes of Health. Easier said than done. His words rang through my mind as I nibbled on a carrot and tried to inconspicu

In a society where, whether it's conscious or subconscious, most people judge you based on the superficial trappings of life – what you look like, whether you look like them, and how big and impressive a pile of toys you’ve gathered – it’s a true blessing to find a good friend. I feel blessed to know my friend Tina. In everything Tina does she tries to do what she believes is right. While we may not share an identical set of beliefs or agree on everything, Tina has the capacity to love people for who they are and where they are – a rare combination of qualities. Tina has been an awesome support to me since being diagnosed with HPS. When I’m at the National Institutes of Health, Tina never fails to call or surprise me with flowers or a teddy bear – even though the visits have become such a routine part of my life most people, even some family, don’t take notice of them. It means a lot because the visits are never routine to me – they always have the potential for bad news – so even