5 Things I Wish You Knew

Staying alive is hard work, and my body sucks at it. There are a lot of things I wish people knew about living with Cystic Fibrosis, but they take time to explain and sometimes it’s difficult. I’ve been asked a lot of questions but many of them force me to evaluate my whole life and coming up with a quick answer is just not possible. The #1 question I get asked the most is “does it hurt when you breathe?” There is no easy answer for this and it always makes me feel put on the spot. Half the time my answer comes out as something like, "... I don’t know I haven’t really thought about it, not today I guess”. There are days when it really does hurt to breathe, but I feel like the pain I experience isn’t what people envision. Most of the time the pain comes from the muscles in my rib cage, like 9 times out of 10 that’s where my pain (personally) lies.

Anyway that’s beside the point. There are real things I wish people knew about my life with the disease, so I am going to create a hopefully ongoing segment recording them, for now I have the first 5. These will range from very broad things to very specific things, I hope you find this informative and helpful.

5 things I wish people knew about my life with Cystic Fibrosis:

1.      My body is literally starving itself. My pancreas doesn’t work properly so my body doesn’t absorb the nutrients in food when I eat. I am dependent on pills before each meal or snack to give my body the enzymes that my pancreas would produce normally, but those pills still aren’t enough. On top of the pills I require a handful of vitamins. It gets frustrating when people remind me how lucky I am to be skinny without having to “work” for it. I may not have to work out or exercise to keep my slim figure, but make no mistake I suffer for it. Every day is a fight to gain weight and stay healthy and my body fights me every step of the way.

      I am on a diet of like 6,000 calories a day, but that doesn’t mean they can be unhealthy calories. It gets even more difficult when I am having a flare up because I often have zero appetite and have to force myself to eat. This is a common problem for people with CF, many will have feeding tubes placed so they can gain the weight they need.

2.      Even the most subtle changes in the air can make it difficult to breathe. My body is able to adjust to temperature and humidity fairly well, but that adjustment takes time. I really don’t know how to explain this so an averagely healthy person could understand it, so I’m going to try and describe my experience with what I’m talking about.

     When I leave my warm and cozy house in the winter just to walk to the car that blast of cold air will take my breath away, which I think is something a lot of people experience but having CF it takes several minutes to catch my breath again. If you add in the fact that I have to keep moving to the vehicle it’s an extra strain on my lungs and heart. Now if the car is already preheated I have to go through this process all over again because that nice warm air is like a second shock to my lungs even though they probably still aren’t used to the cold. There are somedays where it will take over 15 minutes for me to calm myself down after getting in the car.
     That is a case of an extreme temperature change but I can have the exact same reaction just walking down the frozen aisle at the grocery store, or walking from room to room when the air conditioning doesn’t circulate evenly. I can feel even the smallest differences in humidity and temperature and they all force my body to adjust to them.

3.      Showing an interest in my well-being makes you the coolest person in the world. I went nearly two decades without having friends that showed any inkling of interest in my health or than wishing me better. It wasn’t until college I experienced my first set of friends who went out of their way to educate themselves on the disease, and that meant the world to me.

4.      Getting better really just means feeling less sick. Having a flare up and ending up in the hospital is what I call getting sick, so when I come home people are expecting me to be “better”, which I am normally feeling better, but people often confuse that with being healthy. I am unfortunately no healthier now than I was before my last admission, tragically the nature of Cystic Fibrosis means that I may even be in worse shape.

5.      And maybe the most important point of all, sick people are allowed to be happy. I will say this millions of times because there seems to be some kind of stigma that sick or disabled people must be miserable and unhappy when it’s just not the case. People with disabilities can smile, and laugh. The phrase “but you don’t look sick” can be so well intended but it’s a really ignorant thing to BELIEVE. People will say it in some cases trying to make someone feel better about themselves and it can come from a good hearted place but it’s really damaging at times. When you tell someone they don’t look sick you’re practically telling them they’re lying about their health condition. So just remember there is a very small correlation between my health and my happiness and if I physically represent my health I’d probably hardly look better than a corpse.

Thank you for reading through my list. These aren’t listed in any number of importance, and some of them are just the frustrations from things I experience. It’s my hope that if people can understand some of the invisible thoughtless struggles I experience day to day they will realize how important it is to make CF stand for Cure Found.

Look who's getting Orkambi!

This is it, it’s finally happening. The doctor sent my prescription off for Orkambi yesterday afternoon! I don’t think I’ve ever been so excited for a medication or so anxious. I have been e-mailing my doctor non-stop since the medication was approved by the FDA on July 3, now a few weeks later I’m waiting for it to really arrive. I still have to wait for confirmation from the pharmacy, and there is no word on whether or not insurance will cover it yet, I am really hoping they will because filling out the assistance program paperwork will just delay the meds arrival. This medication will cost a whopping $259,000 a year, but for some people with Cystic Fibrosis it could mean life or death.

I fall into a danger zone for the medication. People with PFT’s below 40 have reported good results but also some danger side effects including shortness of breath and lowered lung function. If I get lucky and have no side effects in the first two weeks hopefully I’ll be good to go!  The medication is still brand new so it requires blood level checks for liver function every three months, which is a small price to pay compared to the reward.

So what does this medication do? Well to explain that I have to explain CF a little better. Cystic Fibrosis is often called a lung disease, but really it’s a genetic condition. This medication is really neat because it actually targets the broken genes and helps to correct them and function better. Orkambi consists of two separate medications that have their own function, without getting to specific one medication helps the gene’s work better and the other helps the body receive the fixed genes better. This may not be the perfect explanation but this is how I understand it. This medication isn’t going to fix the damage that’s already been done to my body over the years, meaning the scar tissue in my lungs and my lung function itself won’t improve as a direct result from the medication BUT with my genetics doing what they’re supposed to do it will greatly slow down the progress of the disease. It’s kind of like taking the disease progression and pushing the slow motion button. That will give me the opportunity to focus on healing the damage I have now which over time will hopefully increase my lung function and also extend my life.

Back in June my doctor was extremely concerned about my condition because it was deteriorating at such a rapid rate, but now with the prospects of this new medication I may have a shot of getting better for the first time in a long time.

That being said this medication only works on certain people with CF and it does not help everyone. I am lucky that I have the gene’s that this medication is effective in. Even with this medication it’s still really important that we keep fighting for a cure, we’re so close I really believe we can do it!  

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!