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!