Tuesday, November 5, 2013

8 Weeks tomorrow!

I'm going in for my 8 week followup appointment tomorrow. I'm guessing that my PFT's are in the low 60's% range, which feel like what my new baseline is(up from low 50's% pre-study drug). 2 weeks ago I did 1 week of oral Cipro, it feels like it did the trick and now feels like my PFT's went back up and my energy level has aswel.

This was the first October in I cant remember how long that I haven't had to get hospitalized or get IV's. It has been a nice change. With the weather cooling down and people starting up there fire places I have had a bit more chest tightness and wheezing. As much as the study drugs have changed how my lungs/sinuses are working I still have to do all of my nebs (7%HTS, Tobi) and exercise as much as possible to keep my lungs clear and working as good as possible. The drugs gave me a big bump in my PFT's, but I still cough(it is more productive, and easier to get stuff out) still do all of my nebs, still have to exercise and rest. I still have CF, but feel like I have a few extra years and will be able to do a bit more with them. Time to go to work.

Tuesday, October 15, 2013

Went to clinic

I awoke on Monday feeling full of goo, so I did my nebs, took the dog for a good walk, went on a hour bike ride, did more nebs at home and was still full of crap. Time to go to clinic! My PFT's were down again 57%.  Still up from my old baseline, but the weekend of extra RT's, rest, and tacos didn't help me any. Time to start some Cipro! It it weird saying to my doc that I'm sick but my PFT's are up from my old baseline. I think my new/being on the Vertex drugs baseline is in the low 60% range. I'm still very happy with where my lungs are at, I just see where they can be......

October has always been a hard month for my lungs. Can this be the first of no IV's? I can do a week of oral Cipro standing on my head. That is easy. I just need to make sure I'm doing everything I can to stay off the IV's. I don't want to spend any time locked up, or deal with any of the other shit that goes with it, not sleeping next to my wife, loosing some lbs, eating bad hospital food, not riding my bike or climbing, and missing yoga in the park! Onward and upward!

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October, ick

Wendsday was my 4 week evaluation. I'm guessing my pfts were down around 60% (they can't tell you at the visit). I knew they would be from not doing any RT treatments last weekend due to the bleed Friday night. Thursday and Friday this week I felt like I was fighting a cold. I've doubled up on my RT's and been eating raw garlic and am feeling a bit better. I still have more goo than 2 weeks ago but I'm working on getting it out.

Saturday, October 5, 2013

Friday night

Uhh, I was feeling kind of "off " for the last 1/2 of the day. Tired, had a bad sinus headache, and was ready for bed at 7 PM. Yes I'm old, and happy about it. Robyn and I ended up getting into bed at 9 and  had watching some Star Trek Deep Space Nine. After laying down for a few minuts I could tell something was going on in my lungs. I coughed, not hard but could tell I was bleeding. Robyn and I got up and I started bringing up more. I would cough and get some blood out and with in 20 seconds I would have to bring up more. We were getting ready to go to the ER, but then it stopped. Start to finish it lasted less than 10 minuts, and maybe 3/4 of a cup of blood. Not that much, my last big bleed was 2-3 cups and it also stopped as quickly. So no trip to the ER! Just some shaken nerves and not a great night of sleep.

I went to yoga in the park with some friends this morning. It felt good and helped me relax. I spent the rest of the day taking it easy. No RT's today, I felt like I need to let my lungs rest a bit. Tomorrow back on the bike, nebs, I might some enchiladas for dinner.

I don't think the bleed was from the from the drugs. It has been very windy the past 2 days and the air has been full of dust. I know I'm a bit of a bleeder and most of the time I can trace it to something in the air, but it still shakes me up a little.

Wednesday, October 2, 2013

21 days

Quick disclaimer, this is just a personal journal of what I have been experancing on the 24 week VX 809/ VX770 study for CFers that have the double delta F 508 gene type. Please just remember that we all respond differently to treatments......

My lungs haven't felt this good in 10 years. I've now been in the vertex drug trail for 3 weeks. My PFT's have gone from 53% to 64%. I'm a lucky man to be getting the goods, let's hope that I don't start growing hair on my palms.

It all started about 8 weeks ago. It was my 31st birthday and I was just finishing 2 weeks of IVs, my PFT's were stuck at 52% and my weight was down to 145lbs. It was time to get off the IVs and see if I could gain back some lung capacity and put on lbs. Within 2 weeks I had gotten my weight up to 150, and my lungs were feeling somewhat better. Around that same time I went in for my first screening visit for the Vertex combo drug trial. It was very uneventful, PFT's, EEG, blood work, and a exam. Next was a eye exam where everything was also good. 

3 weeks ago
I arrive at the hospital at 9 A.M. on a Wendsday to get started. More PFT's, EEG, blood work and another exam. All checked out and I was given 3 weeks of the study drug or placebo. The rest of the day was spend killing time between getting EEG's and visiting the nurses on 4 East (my home away from home). At the end of the first day it had been a 8 hour day at the hospital.

The next 2 days I started bringing up more goo. I was thinking that I was getting sick or getting the drugs or my lungs were being affected by some environmental change. I had prepared myself for getting the placebo so I wouldn't be crushed if I didn't see any changes. I knew that after the 6 month study that I would be able to get into a 2 year continuation study where I would be getting the goods if I wasn't already.

But I kept seeing changes, more and more goo coming out of my lungs. Less hard coughing, stuff just started coming up easier. By Saturday night I was 60% sure that I was getting the goods, and by the end of Sunday I was sure of it.

2 Weeks ago
It was starting to get serious. I could take bigger deeper breathes, extra sinus stuff was coming out of my head, and my GI system was working much better. Soft easy poops. But I started feeling guilty. Why was I getting these magic pills when I have lost a few friends over the past 2 years to this shitty disease. But I got over it, and started thinking that I was doing my part to get the drugs approved for everybody.

By the middle of the week I started feeling very tired like I was getting a cold. I was resting lots, eating lots, trying to get some exercise every day but still was feeling kind of crummy. I didn't have a temperature, just felt worn out. The next Monday I went to clinic and found that my weight had gone up to 156, and that my PFT's were 64%! My doc (non study doc) was very excited for me, I hadn't had PFT's that high is almost 10 years. But I told him that I was still feeling kind of worn out like I was fighting something. We did some blood work and I found out the my white blood cell count was the highest it has ever been. But over the next few days I started feeling better.

I went to my 15 day study follow up and asked my Doc what he thought of my white blood cell count being up. His theory was that getting all of the surface goo out had uncovered lots of very old mucus that had been bacteria heavy and I was just having a reaction to that stuff. It seemed to work time line wise, and could not think of any better ideas on why I had been feeling not so hot.

The visit was the same PFT's(they cant tell you what your numbers are), EEG's, blood work and lots of  waiting around. It ended up being a 6 or so hour day, and I got my next bunch of drugs.

I have now been on the drugs for the past 3 weeks. Things have settled down, I feel like I'm at my new normal. PFT's feel like they are in the low to mid 60's, I feel like I'm moving a lot more air in and out of my lungs, my mucus is still greenish but down in the amount I'm brining up, I'm guessing 1/3 of what it was. I feel very lucky and a little guilty for getting these drugs. It's just kind of weird. It seems to be the closest thing to a "cure" I have ever heard about. I still have a bit of scaring to my lungs, years of mucus that has built up in the bottom of my lungs that I'm still working on getting out. I think all of the big changes are over with and now have to work on my normal routine of taking care of myself. 7% HTS, Tobi 1x a day, EXERCISE, lots of good high quality home cooked food, and all of my regular meds. Time to go eat more and go to the gym.