Troll Stroll!

So about a week ago I was over at my apartment cleaning and sorting through the few things that were still there and I was listening to the radio and making a fool of myself singing and dancing through my chores. Well the DJ came on and said that the 106th caller (the station is Kiss 106.1) would win tickets to the Troll Stroll to benefit the Cystic Fibrosis Foundation! Well I was all over that! I fumbled around trying to get my phone out of my pocket, dropped it, tried to catch it but only suceeded in hitting it half way across my apartment. I snatched it up and started dialing. Busy signal. Thank goodness for redial. I must have called 10 times and was about to give up when the DJ answered the phone! I won the tickets!! WOOHOO!!! I talked to the DJ for a couple minutes, told him how stoked I was since I have CF, and it turns out his cousin had CF. Unfortunately the DJ was going to be working so he couldn't go to the Stroll.



The Troll Stroll is a pub crawl. It takes place in the Fremont section of Seattle. This year 12 bars participated. We got a t-shirt and drink tickets for all the bars good for a 7 ounce beer. Each bar had several beers to choose from. We only made it through 6 bars before we got sick of all the screaming college kids and I started to get drunk. But we had a really great time! Each bar also had specials on food and donated part or all of the proceeds to CF.



You're probably wondering why on earth they call it the Troll Stroll. Well, under the Fremont bridge is this guy.

PTC124, my thoughts.

So here we are on the verge of a potential cure for CF. Its the thing that I've been hearing is 10 years away for my entire life. And I don't know how to feel about it. Assuming this works would I want it? Now, before half of you out there in blogger land read that and flip out let me explain.

I can't seem to decide how I feel about remaining exactly where I am for the rest of my life. If this works I'm looking at potentially 50 years of THIS. By this I mean mid-50s lung function. Sure I could regain some function back without having mucus plugging up my airways but the damage that has been done isn't going anywhere. Do I want another 50 years of struggling up a flight of stairs, panting while doing laundry, and walking a step behind everyone because I just can't keep up. I might complain about my health but I've never been angry about it because there was always the option of a transplant somewhere on my horizon. I would think "I might feel like crap now, but someday I'll have new lungs and it will be worth it." Would I like to live another 50 or so years? Sure. But do I want to live another 50 years feeling like crap? I'm not so sure that I do.

I'm doing this study for all the CF kids who might benefit from it. For the ones that haven't been born yet. This drug might not do a thing, but if it does work there are some kids somewhere down the line that will never have to ask themselves if they want to live the rest of their lives with half their lung function. They'll just live, and CF won't take away their dreams, or their lives.

PTC124 Screening.

Today I was screened for the PTC124 study. If you haven't heard about this one yet then check this out: http://www.ptcbio.com/3.1.1_genetic_disorders.asp

So this drug is pretty promising for people with nonsense mutations (which I have one, yay me!), it has the potential to turn me into a carrier rather than a CF patient. I'll write up another blog in a day or two with my personal thoughts on all this, today I want to cover what's involved in the study.

My day started with fasting (everyone's favorite right?) for my blood work. I got to the hospital around 7:40 and ran to the coffee stand for an extra hot latte so that I could get my coffee fix as soon as my blood was drawn (I live in Seattle, gotta set your priorities). I signed the consent forms and my nurse got set up to access my port for my blood draw. Luckily she was able to get it on the first shot (you may recall past troubles with that). Its so nice having a port to do blood draws from!! I think there were about 9 tubes of blood and my veins poop out after about 5 so one poke and done is GREAT! I had ordered breakfast just before my blood draw so by the time we were done I could eat (yay!).

Next came some pretty standard medical history questions, then an NPD. If you don't know what an NPD is check this out: http://www.hopkinscf.org/main/whatiscf/diag_testnasal.html
We had a really hard time with my NPD. Part of my sinus surgery in December involved turbinate reduction, meaning they just cut away some tissue to widen my nasal passages. The problem is that without that turbinate tissue there wasn't anything to hold the catheter in place to do the NPD. They couldn't get it all on the right side, the stupid thing just would NOT stay in. On the left side they couldn't get it to the last measurement without causing me serious pain. It felt like a gremlin was in my nose going nuts with a hot poker. They think I might have a polyp back there that they couldn't see. They were able to get it to stay in at the median point though and got the test. Luckily the company said they would accept it on one side or else I would have been out of the study before it even really got going! This was also with the 3rd catheter they tried! My sinuses have been burning since then. I've had NPDs before and they weren't nearly this bad, so don't let my horror story keep you from doing it.

The doctor came in for a quick exam and then I had PFTs, which we all know is a pulmonary function test. Mine were right at baseline in the mid 50s.

After lunch the nurse showed me the phone I'll be using to keep a diary of my symptoms. I have to do it every night while on the study (a year!) but it just takes a few minutes. I did a test run while I was there. It asks about my activity levels, cough, and appetite mostly. It has an alarm that goes off every day to remind me to do my diary. For every day I do my diary I get a point, my points add up and I get to redeem them for gift cards! Sweet! The phone they give me is pretty cool, too bad I don't get to keep it and they have most of the functions disabled.

Then I had a sweat chloride test. I haven't had one of these since I was a baby so I don't know what the process was back then, but these days its pretty cool. First they put a couple of electrodes on my arm that activate my sweat glands. Those stay on a for a few minutes and then they put a little collector on there. It collects my sweat, then when there is enough they draw it out and put it into a little vial to be sent off and tested.

Next was radiology! I had a chest CT and a renal ultrasound. Good news, I have lungs and kidneys! When I got back up to my room I got hooked up to a vest thing that recourds my coughing. The vest part isn't too bad, it looks like a workout shirt, but it hold all the wires that run from a collar with a microphone on it to the actual little device that does the recording. Then I have to carry that around in fanny pack. This really is kind of a pain. I have to wear this thing for 24 hours which means I get to sleep in it. They gave me a pillow with a pocket in it for when I sleep so I won't roll over onto a hard plastic lump. I can take it off to use my vest though.

You might be wondering why this study is SO intensive. I mean, why a sweat chloride test and an NPD when they already know I have CF? This is what the doctor told me. The company wants to get this drug through the FDA. This drug shows a huge amount of promise for the 900 or so people on the ENTIRE planet that have one of the nonsense mutations. They are looking for ANY positive changes in ANY of these tests to take to the FDA to push it through. If my sweat chloride number goes down it could mean the drug works (assuming I get the actual drug, not the placebo). If my CT improves the drug could be working. This company is trying hard to push a drug through that they will make very little money off of. 900 people is not a very big market after all.

Been busy!

You'll have to exuse my lack of posting lately. I've been so busy moving in with J, and this past week I haven't felt very good. Nothing too bad, just off. I had a bit of a sore throat and some post nasal drip for a few days. Thankfully that went away! Then yesterday I had a horrible stomache ache! I really think I was on the verge of a bowel obstruction. But I took a lot of Miralax and it seems to have done the trick. I feel MUCH better today.

Today was pretty nice so we took the little one to the zoo. That was fun. I LOVE the zoo. A lot of the animals were out and about.

I'm looking forward to my trip to Boston and meeting so many of my CF-world friends!