Tuesday, September 17, 2013


Today's clinic went a little better than I had thought it would.  They just updated their PFT cart so now there isn't a flexible tube, but instead a rigid stand.  They also changed the predicted values to the standard.  So before I was switching to my calculations, for this blog, I now don't have to do that.  My "new" predicted is 3.06L which is only slightly below what I was using at 3.10L.  The difference is really insignificant (1/2%).   I was expecting to see my % go down because of the new predicted values....

So now on the results of said clinic.  My FEV1 went up slightly instead!  I was at 1.03L in July, exactly 2 months ago, and this time I was 1.11L.  My % stayed the same at 36%, but now it is a true 36%.  I shall take that 3% increase (my calculations were 33% for 1.03L last time - are you as confused as me?!?!).  However, I am still lower than I was right before I was admitted back in June when I was at 1.13L.  But that is a small difference.

My weight was down 5 lbs, but my appetite has been sluggish and with the exercise it is to be expected.  She was not worried about it because I feel FABULOUS.

Yes FABULOUS.  I feel great.  There were no issues to report.  No extra coughing.  No fevers.  No chest pain (besides my regular which hasn't been as bad as I had expected given my no Motrin usage now).  Nothing, nadda, zilch, zero.  I told her my O2 has been higher with rest - usually I am around 93-94% on room air at rest, sometimes 95% if I sit long enough - but lately it has been as high as 97-98%!  And my exercise tolerance has improved dramatically too.  I have started doing rolling hills on the treadmill and strength training twice a week.  Once with the trainer and once on my own.  I don't push myself as hard as she does, but its easier to lift heavier than you can with a spotter.

I was told to come back in three months (something I haven't done since moving up here) and call if there are any issues in the mean time.

I could not have been happier with today.

Sunday, September 15, 2013


When you have CF you spend your life waiting for the ball to drop on your health.  You live life as best as you can: you go to college, you get a job in your field, you stop when you need to and in between you fit in hospital stays, IVs and therapies daily.

But what happens when you hit your 30s, you can't work because your health is too precarious, and you have been evaluated for a double lung transplant, and then you learn that you MIGHT get a new drug that could push that expiration date to over 60?!

It sounds so exciting and amazing and just plan AWESOME.

But it also sounds scary and emotional and weird.


Well that's because you spent your whole life up until this point thinking that you would never ever see 40.  That making it to almost 33 is a miracle in itself.  And that the thought of living to 60+ is just impossible.  I know to someone who hasn't had to deal with this it probably sounds ridiculous.  But let me explain.

I don't remember when I became fully aware of CF and its consequences.  I always knew my life was going to be shorter but I don't think I ever truly THOUGHT about it.  Not until 15 or so years ago anyway.  Then I began to think about my life expectancy.  I went to college right after high school and I got a job in my field right after college in the fall of 2001.  I worked in my field for 8 years, almost to the month (November 2001 to December 2009).  I participated in the 401k offered by all my employers but I don't have one anymore.  I took the penalties when I moved up to Boston and took that money to survive on until I was approved for SSDI. When we would have meetings in work with the 401k company I would tell them I was not going to live to retirement age so what were my options?  Just get it anyway.

I have never thought about burying my parents or living without them.  I have always assumed I would be buried first.  I have never thought about old age and spending 30 years with my husband.  I always assumed I would be dead by 40.  I never expected to see my niece and nephew graduate high school, college, or get married.  I haven't worried much about things because I was expecting to be dead by 40.

Now I am faced with the possibility of ALL of those things.  I might have to bury my parents.  I might spend the next 30 years with Peter.  I might see my niece and nephew grow up and get married.  I might make it to retirement age and I might not be prepared.

When you expect to die early you live your life completely different than someone who expects to retire some day. And not just in "living" life, but in preparing for old age.  Why prepare if there is no need for it?  I wanted to keep my 401k in a 401k when I "retired" in 2009 so that my family wouldn't have to be burdened with my funeral costs.  I was told by them not to worry about it, to take the money to survive on then.  You think differently.

How does one cope with this new prospect of life?  Especially for someone who likes to be prepared, how do you cope with the possibility of NOT being prepared to retire?  Kalydeco, when it comes out for DDF508 mutations, will change my life forever.  I will live to retirement age.  I will grow old with Peter.  I will be able to go back to work.  But its going to be like starting out at 35.  Those 14 years where I was working, and then not working, will be like they didn't exist.  I will have to start from scratch.  And let me tell you how scary that thought is.

I don't want anyone reading this to think I am being ungrateful.  Believe me, I am thoroughly ecstatic that I will get to do all that I wanted to do again without needing new lungs.  I won't have to worry about the threat of cancer being exacerbated tenfold because of immuno-suppressant medications. I might get the chance to be as close to normal as possible.  And that is amazing and exciting to me.

But I still have to deal with things I never thought I would have to.  I guess it makes me human and normal to now think about things that my husband and brother and friends think about.  To worry about retiring and having money to do it.  To worry if I will spend the rest of my life working somewhere I hate to get a good retirement, or if I will do something I love for 30 years.  The possibilities are endless, and though they are scary and exciting, they are inevitable.

I hear there are survivors groups for people with CF living over the age of 40.  Maybe we need more of these, for those who will be changing their life's outlook completely in just a few short years...or less...

Tuesday, September 10, 2013



It's a greedy little bastard that keeps us from doing things we KNOW are right.


It's one of my many faults.


It's something I WILL overcome.

Why can we not admit when we do something that is wrong, and we are called out on it, and we know that we need to change our ways?  How hard is it to modify your attitude slightly so you can do this tiny little thing that you know needs to be done to make someone happy?

Apparently it is very hard ha!  But I WILL change it.  I am working on it.  I am trying my hardest to not let my pride get the better of me.  I have to do this, not just for me, but for those around me.