Navarre Beach, FL

It has been about 10 days since I finished IVs.  We took advantage of fall break and my husband having an extra long weekend by heading to Navarre, Florida.  One perk of being in the military is that you end up having friends all over which makes for a good reason to take vacations.  We were able to catch up with friends that we were stationed with at our last base.  They had a few back to back assignments overseas with Florida being their new assignment.  It was great getting to see them and hang out.  It was a good relaxing weekend.  The weather was cloudy, so not great beach hanging out weather.  I was a happy girl as long as I got to put my feet in the sand and water.  I would have been ok if I just got to see it.  The beach really is my favorite place and makes me a very happy person.  It is very relaxing to me.   The ocean/gulf amazes me every time I see it because it seems as though it is never ending.

My husband was able to play golf with his friend so he was a happy camper.  The golf course is his happy place.  As far as I’m concerned he can drop me off at the beach and he can go golf.  He is perfectly content with that also.  I was able to have some girl time while sitting on the beach which was nice.

It was my step-sons first time ever seeing the beach.  He said, “the beach is my new favorite place.”  We were staying with our friends so he was not able to take a friend but he didn’t miss any opportunity to mention that he really wished he had a friend there.  There are many more years of vacations/trips kid, in due time.  hahaha  Our friends have a son his age so it worked out good.

I, of course, wished we could have stayed longer but life continues thus we had to return.  I left some medicine behind in the fridge which has to stay cold so that just means I need to go back…oh darn.  It was a great get away.

Now onto health stuff:  I have felt “ok” since finishing IVs 10 days ago.

Many of you know about the newly approved drug Orkambi that is for my mutations of CF (2 copies of delta f508).  I was on the study for this drug when we first moved to TN 2 years ago.  I made it through the 6 months of the study but chose not to continue to the roll over where you remain on the study drug until it is approved.  I personally did not see improvements or major changes that warranted continuing the study drug in my opinion.  Now that it is approved my nurse asked if I wanted to give it another try now that they had to dosage figured out.  I said sure.  I got approved for it and it was delivered.  It is a very expensive drug to the tune of about $20,000 per month.  It sat on my counter for a while because I knew what the side effects were and I wasn’t ready to endure them because I was already sick.  I didn’t want to start it when my husband was out-of-town for work incase I wasn’t able to do my normal stuff.  Then I started IVs, so that was another 3 weeks.  Then we went out-of-town for 5 days.  I was really good at putting it off as you can see.  haha

I took my first dose and with in a few hours I was very short of breath and my chest was tight.  I would cough a lot trying to open my airways.  I did lots of breathing treatments hoping they would help.  I figured I would give it a go for the night dose as well.  I was up at 3:30am needing a treatment.  My lungs felt like they couldn’t expand enough to get the air in which is a scary feeling.  I decided not to continue the Orkambi.  I don’t have it in me right now to deal with the chest tightness and shortness of breath.  My asthma isn’t play nice, so to add in another component is not worth it right now.  There are many people who are seeing great results with Orkambi and others who are taking it and not seeing any significant improvement to steadiness but continue anyway.  I am one that doesn’t like to take a medicine if I don’t have to.  If I can’t see any pros to it then I don’t continue.

Orkambi works by correcting the misfolded CFTR protein, which is the root cause of the F508del mutation.  Basically it is supposed to make the CFTR fold the right way so that the CF body will process thing correctly.  I know that some will say that I didn’t give it a good enough effort or that I am missing the “cure.”  This is not a cure.  It is a huge step in the right direction.  I am the only one who has to live with my CF and endure all the issues I have to.  If it was you, you may do things different which is fine but this is my life I am living.

Until next time…