Monday, August 24, 2015

Hospital Time!

I had a sick visit clinic appointment today.  Recently I have been more short of breath and have been having some lung pain.  And since I am supposed to be starting the new drug Orkambi soon, I wanted to be sure I was in tip top shape.  The first few weeks on it people can be more short of breath and tight and since that is normal for me, I don't want to make it any worse.

So Wednesday I will be going inpatient at MGH for the first time, for two weeks.  Then when I get out I can start Orkambi and see if it works on me!

Also, I did a 24 hour urine collection last month to see how my kidneys are functioning.  Well the place we used screwed everything up so I need to do it again.  Clinic tried so hard to understand the results with no luck.  And for some reason the place put down I only had 500 mLs of urine which is nothing when I know I had at least 2100 mLs since I looked before I dropped it off.  So I get to do that again tomorrow so I can bring it with me when I am admitted and then MGH can handle it.  He also mentioned that I might need to see a renal doctor after we get the results.  But we are going to give Tobra another try which I like because my lungs always respond very well to it.

Saturday, August 15, 2015

Guest Blog on the CFF

Guest blog post up on the Cystic Fibrosis Foundation's new blog!!!

My Decision to Stop Working

I will be doing another one in a few weeks about Orkambi.

Check it out, I was super excited to do it :)

Monday, August 3, 2015

Game Changer

I have discussed this before, but now that reality is happening.  Orkambi has been approved and is available to those with DDF508 mutations.  ME.

Vertex' drug has been approved and my life could change.  Dramatically or barely noticeably.

So as I sit here thinking about what could happen I find myself floating in a sea of endless emotions.  I am excited I may be able to go back to work.  Excited I may be able to earn money again, and not just collect SSDI.  I may get the chance to grow old with my husband.  To see my niece and nephews graduate and get married and have babies of their own.  To watch my own step kids do these things.  I may get to experience all the things that old people experience.  The good and the bad.

So why am I feeling this barrage of emotions?  And why are there some not so happy ones mixed in there?

Because I have lived almost 35 years with this disease.  I have come to know my body, my progression, my health.  I know when I am a little out of sorts or a lot.  I know what to expect of it, most of the time.  I am pretty vanilla.

I have also lived my whole life expecting to die at any point.  That my life has a early expiration date that is gaining momentum as I age.  I have put aside thoughts of ever having to bury family members and never having to bury a husband.  Never seeing anyone get old and never having to worry about it for myself.

But this medication could change all that.  I don't know what to expect.  I do know to expect the unexpected.  I know I have to think about retirement, real retirement.  I have to think about old people diseases and aging.  I have to think about watching my parents age.  All these scary things that most people don't even consider because they are a part of a long life.  But I never thought about a long life.  Hell I thought I would be dead by 26 but here I am almost 35 and alive and kicking.

So what do I do now?  The answer is complicated.  I know I have to sit back and relax and see if I even get any help from Orkambi when I can get it.  And then I can start to think about the rest.  About growing old....with my husband.

Maybe I will be able to sit on the porch on rocking chairs and watch our grand-kids play in the yard...maybe, just maybe....