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!

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.