A Spoonie Full of Sugar | In Depth

Hello!

If you're reading this, there is a good chance you've come from one of my social media pages and probably already know a little or maybe even a lot about me. For anyone else let me introduce myself.
I'm Marissa! I'm currently 27 years old and a few years ago (September 2nd, 2016) I received a double lung transplant. I'll make a whole write up explaining my transplant process and how things happened and be back with a link for it shortly. It was a crazy ride!
Now though I'm back in the midst of lung failure and working for a second transplant. I'm really hoping to have it done at the University of Michigan, the same hospital that did my first but I really won't know until we get deeper into the evaluation process. Speaking of the transplant evaluation, they're started next week! Thursday August 1st to be exact! It wasn't originally scheduled until mid September but they switched it up on me.
And that is part of the reason I wanted to re-start this blog. I was going to make a new one from scratch, with a new name all geared towards transplant #2 but I decided against that.
As you can see this blog page hasn't been used since before I received lungs, but I think what I wrote here is still important. Hopefully we'll be able to see my writing progress and get better as we go along too (fingers crossed on that).
I want this to be about more than just my second transplant process. My first transplant wasn't very typical so a lot of the things that happened have caused some serious mental health changes in my life and I really want to talk about how I react to them even now 2.5 years later.
There are also a lot of sensitive subjects I want to discuss about the process. My illness progressed so fast that one week I had plans with friends but I had to let them know that doctors didn't expect me to live that long. These are going to be sensitive subjects that you never really know how you'll handle until you're in that situation, but I can at least guide you through my thought process and how I handled everything.
I also plan on discussing the negatives that have come from my transplant. It's something I don't see discussed enough and  I think a lot of that stems from people not wanting to seem ungrateful. Openly and honestly discussing the hard and difficult parts of the transplant process pre and post does not make you ungrateful. Sure, we wouldn't be here without our donors, but we're also humans who still have good and bad days.
If you're out there and you ever need someone to talk to, don't be lonely! I'm here for you!

Well wish me luck!

Marissa Parks

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.

I Miss missing You

Do you ever listen to a song that makes you hold your breath? Something about the melody or the lyrics ring so clearly into your soul that you feel it physically ringing throughout your entire body. The song might remind you of someone you hadn't thought about in a long time and you can feel this dull pain bringing forward an empty place in your heart you'd long forgotten about. Maybe it was an old relative or a friend or lover that slipped your mind after all this time and suddenly a picture of them placed itself right at the front of your memory commanding your attention.

I've encountered this pain recently, but in a weird nostalgic way. Instead of remembering some old lost friend I was reminded of myself, this person that I used to dream of becoming that somehow through the years fell through the cracks of my mind. The dark days of my life let my mind become clouded by someone I never thought I would become. When we're children we dream of what we'll become but now that i've started reaching that age I've realized that is the entirely wrong question. In all of my dreaming of what i would become somehow i'd forgotten to dream about who I would become.
I was listening to a Fall Out Boy song earlier today and I came across their song Miss Missing You. Now I'm a nostalgic type, I tend to fall in love with anything that can bring back feelings or images of the past I guess you could call it a weakness of mine. It doesn't happen often but I came across some lyrics that left me catching my breath.

"Sometimes before it gets better the darkness gets bigger,
the person that you'd take a bullet for is behind the trigger" Fall Out Boy

Sometimes it only takes a few short words bring your whole life into perspective. I heard the words and somehow my mind went to myself as a child. I had all of these dreams and ambitions and there was never a single thing that would keep me from making those dreams come true. Now that i've aged and learned about how life "really" works so many of those dreams I had have vanished. I've found myself more disappointed lately in how my life went and at times I try to find someone or something to blame. I usually end up blaming my health, after all it's the most realistic and obvious choice. But listening to that song somehow made me realize the person behind the trigger was me.

I can do more for myself than give excuses for broken dreams. The responsibility for the collapse of the worlds I once created is solely mine and the blame can't be shared. Those broken dreams don't make me a failure though, it's the mindset that it wasn't my own fault that does.
I am long on my way to what I want to be when I grow up, but it's never to late to start dreaming of who I want to be.
I still have so much potential in me, there is a whole solar system of ideas burning inside of me and it's time they got the chance to shine.

Orkambi Side effects 1 month Update

Okay I fully intend to write more often and I have a few things started, but for now I have an Orkambi update. I was inspired to make a video update on my Orkambi side effects after I seen several CF you tubers documenting their Orkambi story daily.
So if you're interested in checking it out, here it is!

Orkambi update - How I'm handling the side effects

I began taking Orkambi a week ago today. I made the decision to take the medication knowing that there was a chance that I could experience severe side effects that could leave my health in a worse condition. Throughout the past year my health has dropped severely and if it continues to deteriorate I won't have long left to live.

Now I read all the paper work on the medication that I could get my hands on, but seeing as it is a new drug the information is very limited. When I began taking Orkambi I really didn't know what to expect, and boy has it been a long week!
Let me premise this by saying before I started the meds I had been feeling a bit congested, and I was dealing with some sinus problems spurred on by the air conditioning.
The first day I took the medication I was so wound up and anxious about looking for the side effects and making sure I was breathing right that I actually ended up having an all day panic/anxiety attack. It wasn't very fun and I exhausted myself. I have been looking forward to this med since they announced it's pre-approval, I was terrified that the side effects would take it away from me. That being said my exhausting day of anxiety probably made me sick.
The second day of the medication I had a lot of shortness of breath and coughing. Now I don't know how much this was spurred by the medication, or if I was experiencing a normal exacerbation. I had the normal symptoms of a regular CF exacerbation so that I how I treated it. I waited another day and I e-mailed my doctor and he sent me some oral antibiotics. The first time I've been on Oral antibiotics this year, the doctor previously would just directly admit me.
It's been a few days and with the antibiotics and I have already noticed a lot of improves, though I am not back to my baseline yet I hope to be soon!
Some other side effects I've noticed are that I sweat a lot. I am usually an oddly cold person, I wear sweaters all year and keep a blanket with me because I am always cold. Since i've started this medication all I do is sweat! All day and all night I feel like I am dripping sweat and it's so gross and I don't really know how to handle it because I've just never been a sweater. It's made sleeping almost impossible because I sweat all night, which leads me to my next side effect.
I am tired all the time! I've had almost non-stop fatigue since i've started the drug. I am not sure how much of this is a direct side effect of the med or if it's a side effect of the other side effects. I haven't been sleeping well since I've had an increase cough, over heating and the sweating. I don't want to put the fatigue all on the med, but I do think it is contributing to my muscle exhaustion. I haven't been quite up to do the running I was before even with the exacerbation, but this is getting better everyday!

Most of the side effects I've noticed are slowly improving and getting more manageable (or i'm just learning how to handle them). I am hoping that it continues to improve and that I can stay out of the hospital for another month at least! Optimistically I would make it to my next scheduled appointment of October 1st! I'll update my journey with Orkambi as it progresses, but for now I am going to go back to updating about my regular life again!
I really hope this medication makes a long term impact on my health so I can make real plans for the future without having to worry about being around to see them through. I am still focusing on my short term dream of designing some cute Disney themed clothes! It's not as easy as I hoped but it's going to be worth it. I hope people will love my designs so I can see them worn across the Disney parks while I'm on my trips!

Wish me luck on my journey!

What am I doing with my life?

2015 has been a really harsh year for me. I've have been grateful to accomplish so much while dealing with being so sick, but for the first time this year I my health has stopped me from accomplishing one of my dreams. But with every bit of bad news comes something good! I have finally received and been taking the new medication Orkambi! I explain with a bit more in this video:

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!  

musings before bed

There are a lot of times that I find myself questioning my decisions in life. I often wonder how the decisions i've made have effected people, but I don't mean the people around me. I try to consider the people who I had the chance to meet but never did, how many people I will still meet down the line anyway. Then I can't help but wonder how many people I run into that i've left an impression on that never say a word, have I ever?
Sometimes I spend to much time wondering who I would be if I didn't have Cystic Fibrosis. I only assume these kinds of thoughts are normal to have occasionally. Part of me is so grateful to CF, I see the world in such a beautiful way and I know it's only s a result of my CF. I just can't help but wonder what it's like to be healthy, to be kind of normal?  I've never really been a person to hold pent-up aggression about being sick or having this problem, I see my questions as being curious more than anger induced. I've always had way to strong of an imagination and far to often I let my dreams loose and they run wild crashing into reality.
Sometimes I think I dream to big, to big for anyone person. My dreams stem from this distorted idea that anything is possible if you work hard enough, and it's become apparent that some dreams aren't part of my lifes' bigger picture. Some dreams are meant to fill just a small time in our lives, and the pain that comes from their fulfillment can be just as bad as losing them all together.

A selfish wish that I could hold onto every dream forever, hold onto all those people I could meet forever. I just want to keep the dreams in my line of site ad never even blink because they could disappear. How is it possible that the correct decisions cause so much pain and anxiety? Nothing worth it is ever easy right? It wouldn't be right if I didn't consider every little variable.

And to all those people i'll never meet: I hope you have the best time.

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. 

A spoonful of sugar

People always say do things while you can because life is short, it's a statement i've heard many times and one I believe in. I've always faced life with the best reality I can, but also the most optimism, part of me never truly believed that a day would come where I would have to stop doing things because my life was coming to an end.
This past year my Cystic Fibrosis has been progressing at an alarming rate. I am teetering back and forth between the point of being evaluated for a lung transplant or being to healthy. I am reaching a point in my life where the goals I set for my future seem more like distant dreams that need to be put on hold in the hopes of my health improving.
Even though I was born with Cystic Fibrosis and have dealt with it every day of my life it is still difficult to understand and comprehend that there are things that I cannot do, especially now that i'm getting to a point where the list seems to be growing faster and faster.

I am creating this blog to help share my story, and to help inspire myself to keep finding new ways to keep the things I love most in my life even though I may not be able to to do them or be near them like I once was.

My health may be starting to fail, but I am still living I just have to start finding another way to do it.