Wednesday, November 16, 2016

Orkambi & the Hospital

I haven't really felt up to blogging or doing anything since I got sick. I have been having a few breathing problems and more asthma attacks the last two weeks. I had my regular Cystic Fibrosis Clinic check up on Tuesday of last week. I knew it wouldn't be great, but I had no idea how bad it really was...

At clinic we found out my lung function dropped majorly from my "baseline" of 58% to around 38%.  My Respiratory therapist responded "Dang, that's transplant range, we gotta get those numbers up."

It's scary to think one sickness, one medication and my lung function can go from 58% to 30s. Where I struggle to breathe and could be listed for a transplant. But I have faith, hope, and trust. This is why. My doctor(s) at the CF Center are very proactive!

My doctor told me I had a couple options:
1. be admitted
2. Stop Orkambi
3. Keep doing Orkambi, but switch up some of my meds at home and call in a few days for update

Of course... I decided option 3. I do not want to give up on Orkambi, not yet and who ever wants to be admitted.

Doc K also explained that one of the reasons I felt like I was drowning or suffocating could be because the Orkambi was actually working. She said that all the mucus (gunk) over the years builds up, falls, and sits at the bottom of the lungs. Over time this extra weight in the bottom of the lungs is what slowly takes the elasticity out of the lungs, stretching them out. This is why the forced exhaled volume is the most important factor in lung function, it measures the elasticity. She said  Orkambi helps to allow more sodium chloride through the cells and move gunk along better (instead of gunk getting stuck and causing infections). Which is why Orkambi is so beneficial, it helps the gunk move properly. So less infections, time in hospital, or build up of gunk in the bottom of lungs, helping us to keep our lung elasticity.

Which is why I wasn't ready to give up. Doc explained that all the gunk over years accumulated in my lungs and now my Orkambi is moving it up. Which means it is clogging up majority of my lungs right now. So I went home with option three. after clinic A few days later I felt a lot worse. I had a minor fevers and  something like a sinus infection on top of it. I kept blowing my nose, coughing, and was sooo tired. I knew it was time for hospitalization.

So here I am in the hospital. I was admitted on Monday and I'm not sure how long I will be in. I'm still getting 101-102 fevers, coughing tons, lung function same horrible number, and get tired easily. My appetite is down and I'm constantly blowing my nose. BUT, I'm getting some more stuff out sometimes after Respiratory. Which is the key to clearing out my lungs. So we are taking it day by day. We have to make sure my lung function doesn't drop more & see how my body responds to the IVs antibiotics, 4 nebulizer treatments & CPT daily.

One small sign of hope it is starting to move stuff around in my lungs is this: For years I have cultured the same strain of pseudomonas. It is a strain resistant to all antibiotics and I have always cultured it. Well, my last culture showed a different strain they hadn't seen before. They believe it is an older strain that got covered up with the mucus over years and just sat in the bottom of my lungs. They new strain that I'm culturing is not resistant to Meropenem. So we can treat this strain with a Meropenem IV and see if I can keep bringing stuff up. I'm not found of Meropenem, last time I was on it I had diarrhea (C. diff related) for 6 months post hospital. UGH. So hopefully, I adjust quickly this time. But this new strain gives me a glimmer of hope to hang on to for now.

It is exciting to this new strains that were buried really deep are surfacing, but at the same time how long can I hold out feeling miserable and out of breath with low lung function. Especially since a few other CF friends said once they dropped from Orkambi (even after off the med) they couldn't get their lung function back up). So we have to be careful, since I'm already so low.

Right now it is day by day. I will keep everyone posted. Thanks to those who visited, sent the hospital-grams, or sent mail. I appreciate it, even while feeling miserable I am very bored. lol.


  1. Sorry to hear that you are going through this rough patch; hopefully it - (the mucus in your lungs, and you out of the hospital) clears out soon. You can beat it!

    1. Thanks! I hope so too, but at the same time, I'm willing to do some hospital time if it means gaining back my lung function! :-) and Thank you!!!

  2. Thinking about you and hoping you are improving. If I can help entertain you in any way, please let me know ��

    1. Very slowly, but I do believe I am improving. I've been taking extra real estate certifications classes online and watching Downton Abbey marathon form :-) and thanks!!!

  3. Hang in there! Orkambi can be such a rough drug to start. I was pretty miserable for about four months, but after 14 months on Orkambi I am glad I stuck it out. Of course, you know your body and your limits. Good luck!

    1. I'm sticking with it so far. I don't mind rough patch or even a long one IF my lung function comes back up! I have been reading about your experience and it has really been helping me!
      Thank you!!! Glad you are seeing the positives of it now!

  4. This means that you'll be given money to help to cover the hospital bills your medical aid might not be capable of paying and also to help with making up for any income that you're losing out on because of being in hospital.