Orkambi and Health Update

The last time i was at transplant clinic i talked to my doctor about how well my lungs were doing without the bronchial valves i had to fix my recent lung collapse and we agreed that if i can get my lung function over 30% then i can go on a hold from the transplant list. Well my next appointment is in March but 3 days ago at my regular clinic I blew a 31%!!! (i only got above 30 for one out of 3 but it still counts!!) I am beyond excited with my progress! From breathing with one lung to possibly going on hold, from about 105lbs to 124lbs, im kicking ass left and right!!

Blowing away the docs!!! (pun intended!)

Except for the past few days....Ive officially begun taking Orkambi. The newest drug to come out for CF. While all the studies were done with people over 40% lung function, some with lower numbers have still seen results. People warned me the side effects were brutal in the first few weeks and they werent kidding. Sweats, chest tightness, shortness of breath, body aches, coughing up an amount of mucus that i didnt even know could exist in a single body, no sleep, and lower effectiveness with my anti anxiety, anti depressants, and sleep meds. Luckily i felt only half as bad as day 2 so im pretty confident i lucked out on the side effects front!!! For a shot at even healthier lungs i can handle this but i am scheduling an admission in the next week for a tune up. I havent been feeling the best and i wanna catch it while its early so it doesnt turn into anything more serious.

Feeling Orkambi at its worst!

All in all im doing really well thanks to a new diet, lots of exercise and workouts, and being on top of my meds and airway clearance every day. After the side effects wear down and im done with iv's theres no telling what my numbers will be, but im pretty confident in this new med and myself and when March rolls around ill be ready!

Post Transplant Thoughts and Ramblings.......

Ive always loved open space, feeling like the only person for miles and miles, being in the middle of nature without a single man made noise. My favorite vacations were cabins in the woods where my days were spent hiking and kayaking, sitting on a porch eating dinner watching the sun go down over mountains and tree tops. I love waking up to foggy mornings next to a lake, drinking coffee and hearing nothing but birds. Tonight the dumbest thing happened i was watching X-men Origins : Wolverine and for some reason when it showed his house in the mountains, i got this pang of sadness. My life now consists of being in one spot, near my transplant center, constantly running to appointments and hospital stays. Im connected to my phone at all times in case "the call" comes in or i have an emergency and need to call an ambulance. At times im conflicted over transplant because i feel like it could be too early, like im still healthy enough to keep these lungs for a long time, but then times, like right now, i want new ones so badly, i want that life i love so badly. I want to hike and run and feel free. My oxygen cords are like a ball and chain and i wanna break away from them but i cant. These last few years ive tried to do all the traveling i could because i knew this was coming and as much as i did i still want more!! I cant wait for the day i drive away and stay away for a significant amount of time!!! I love my family i could never leave them for too long but my number one post transplant goal is to live somewhere new, on my own, for at least a few months. I wanna explore a new place and experience new things!!! Its so weird how that one scene got to me so badly hahaha i feel so stupid but sometimes the dumbest things really make ya think!!

If you got a second chance at life, where would you go? what would you do with it? would you wait till you were cleared to go back to your job and keep your life how it is or would you go off for a while? live out a dream for a little while?? Im so excited at the prospect of a new fuller life right now! Terrified....but excited.

brb daydreaming of new lungs, and new life.

When Bouncing Back Isnt Realistic....

Well im ecstatic that my PICC was pulled just now seeing as it was driving me CRAZY!! I think i might have started to develop an allergy to the tegaderm dressing or the chloraprep (cleaner they use) because although my lines are never comfortable this one was almost unbearable, it kept me up at night and i thought i was gonna scratch my arm right off :( Soooo glad its gone, however it hit me last night as i was trying to catch my breath while walking through my apartment, that i was trying to catch my breath while walking through my apartment....

Allow me to explain- June of this year i went to the Apostle Islands thanks to my super amazing wonderful sister who surprised me with a trip she knew i wanted to do before it got too hard for me. I wanted to kayak through the sea caves on lake superior so badly and she planned a whole vacation, drove 8 hours, rented an incredible cabin with a private dock, planned hiking trails (one with a pretty waterfall you could go behind (see bottom of page for said happy times), fished with me in a half deflated raft, and paddled her superstar ass off in a kayak in a rain storm in 40 degree water so that i could do it. Words cant describe how much fun i had and how lucky i feel i saw something so beautiful and got to spend a few days just hanging out with my family and what that meant to me is priceless. I hiked up to 5 miles uphill most of the way and i ran and was able to kayak through the waves and i felt good. I felt really really good. Now its 5 months later, my lung function is a mere 28% and after 2 weeks of IV antibiotics and a 5 day hospitalization im still gasping for air while trying to sweep my living room floor. Its disappointing to say the very least. This is the first time that ive finished a course of treatment and still feel short of breath. Usually i feel unstoppable and this time i just feel the same. I knew this day would come, when i didnt come back like i used to after antibiotics, but i didnt think it would be at 23.  Looking back on how my life was not only a few years ago but just back in June is heart breaking for me. What else will i have to give up? I live for weekends out with my friends dancing and having fun but im scared those days are coming to an end soon as well...Scary stuff!