The future is mine!

There has been an amazing development this month for the Cystic Fibrosis community. The FDA has approved a medication called Orkambi that will treat the underlying cause of Cystic Fibrosis in CFers with the double delta gene type (my gene type). This medication will act as a pause button of sorts helping to stop the progression of CF from getting worse!
I really can't express what a big deal this is for the community and for myself personally. In the past I'd never really considered planning my life more then 1 year our at a time, and with this new drug I could have a whole future out there!
I don't think people on average realize what a privilege it is to have a future. I've spent so much time from my life preparing for it to end and now I feel like this weight is being lifted and I can focus on a tomorrow that is less foggy and unsure. There is no day guaranteed for any person, but when you live with this cloud over you that any wrong move could put your tomorrow in jeopardy you really start to notice more when the sun comes out.

I was admitted into the hospital around the middle of May for the third time this year, the doctors were really concerned that my health had deteriorated so much in the 5 month span that 2015 had started they were looking into lung transplant evaluations and giving me around 2 years left to live if things continued progressing as they were. The hard part is hearing that and knowing that you are doing everything you can already to get healthy. I was doing my best to improve my health and the outcome was just the opposite, it was getting worse.  Finding the motivation to keep moving forward and to keep doing your best isn't easy and there are days where you refuse to get out of bed because it just feels useless but the important thing is pushing past all those negative thoughts and just doing it all anyway.
I worked really hard through the month of June and for the first time in two years I saw a positive increase on my health! My lung function improved 6% to 36%. My numbers have been that high since November of 2013! The number sounds so small, but it feels so great! I was really nervous when I went in to do the breathing test, but so excited when the technician told me the numbers that I insisted on doing more to aim for a high percentage and in the extra 3 tests I did I improved my percentage by 3!  That tiny bit of optimism I had made a huge difference in my tests so I can only imagine what the optimism I have for live can do with my time.

I was also incredibly received to a point of tears that I was showing signs of improvement in another area, my weight. I have been struggling with my weight since December after having influenza. I dropped over 40 pounds in 3 months which is incredible considering i was hardly at the proper weight to begin with. Talking about my struggle with weight gain is difficult because so many people ignore the fact that for me it is a challenge. I have a very strict diet that is difficult to complete, even though it may seem like the dream diet to some people it isn't as easy as it looks. Anyhow, I managed to gain 8 lbs in the few weeks I was out of the hospital finally pulling me back over the 100b mark. I would have been grateful for 5 lbs but a whopping 8! Again it sounds like such a small number, but so much work and effort went into the little 8 that is feels like a monumental victory!

I don't have another appointment until October and I am really scared that the start of Autumn and winter will throw all the progress I've made away. I plan on working hard and improving all of my numbers even more! I hope to get my lung function back into the 40% range and my weight at least over 120 lbs by my October appointment. I also plan on continuing or restarting my training for a half marathon! I completed my first half marathon November of 2014 and failed at the one I had registered for this past May but I am confident with these improvements I'll be able to finish another one in no time!

I am so excited for the future! After all we have to keep moving forward!

I promise...

On my last hospital admission a few weeks ago the floor doctor told me that I needed to quit making plans of traveling because my health wasn’t stable enough for it. I was told that all plans needed to be put on hold until I had a lung transplant and I was healthier. For a reasonably healthy person this is probably reasonable advice, but for someone in my position it’s detrimental. The reality of having a chronic progressive disease like Cystic Fibrosis is that I have no guarantee of being healthier in the future; in fact I am guaranteed just the opposite.  

I thought long and hard while I was in the hospital about how my life would be if I canceled all my travel plans, and quit my job and stayed home full time. There are a lot of things to consider when you are in this position because decisions like this don’t affect just you, but also everyone around you. I lay in bed and I seriously considered what the doctor had said, but the more I thought about it the more depressed I became. What is life without the things you enjoy? If I could live a hundred more years by doing nothing was it really worth it? I couldn’t justify giving up my life, especially for a healthier future that may never happen. I called and spoke to my primary CF physician about it and he told me he wouldn’t tell me what to do but he highly advised against my travel plans. He also took the opportunity to remind me that my health is a full time job by itself.

I go in on the 30^th for my first clinical checkup in months, I’ve been admitted more times this year than I have actually been in the clinic. I am hopeful that my lung function and weight has increased, but if neither of those things have happened I may have to face the consequences of continuing on with a lung transplant evaluation. I’ve decided that if I have come to a point where my years are numbered that I will not spend it biding my time and waiting, I am going to make the commitment to live my life in the loudest most joyous ways that I can. I refuse to sacrifice happiness for time. This blog will be my journal through what could be the rest of my life.

Eventually we all have to handle the idea of our own mortality, this is the promise I am making to myself.

I promise that today I will live a life I have never lived before, I will go on an adventure and explore places and ideas I have never before. This time I have will be used to make memories for a time when memories will be all that is left of me.