I want to be a bookseller

Last weekend P and I decided to take a drive.  It was New Year’s weekend and with three days off, he was starting to get bored and I was restless having everyone at home with me.  There were a few books I wanted to pick up so we decided to take a trip to our favorite used book store up in the Ipswich area.  We meandered up and when we pulled into the parking lot we saw the building was EMPTY!  The whole thing!  No used book store and no antiques shop below.  Just a sign saying they would be back after lunch…..no forwarding address, no we moved sign.  NOTHING.  I was (still am!) devastated!!!  We loved that place.  Not to mention finding a used book store that isn’t part of the Salvation Army or in Barnes and Noble is near impossible. 

As we drove away I had the very strong desire to open my own book store.  A quaint little shop here in our town where we would sell some new, old and maybe even rare books.  We could have a small little coffee station in the store and a lounge area to read.  Free wifi of course and I could bake some bread and make some jams to sell.  During the slow hours I could relax and crochet and read.  I wouldn’t have to have the store open 10am to 9pm like retailers; a reduced hour schedule like noon to 7pm would be perfect.  Maybe only a few days a week too.  I go have book club meetings and maybe even work with the library on things. 

I thought of it all.  I got totally wrapped up in it, even telling my mom she could move up here and work it with me. 

But it isn’t possible.  At least not right now. 

But WHY?

Am I just scared and using my health as an excuse?  Or would it really be a bad idea?  I love to read.  I would love to have my own shop.  I have the perfect location in mind.  I could find funding I am sure (grants for women owners seem to be a good place to start). 

But could I do it?  And should I do?  And will I do it?

Maybe…someday….maybe…never…

FDA Advisory Panel Recommends Approving Vertex Drug for DDF508!!!

HERE

What does this mean for me?  This means that if on July 5th the FDA approves this new drug I could be turned around.  My life could go back to normal.  I could get a job, buy a nice house, stop doing so many treatments, grow old with my husband.

CRAZY!!!

Happy Mother's Day!!!

Today's post is dedicated to the mother's of "sick" kids.  This does not mean Cystic Fibrosis only.  I mean any mother who is raising a child with any type of "issue" whether it is physical, mental or emotional.

Parenting is tough.  From my few years as a step mother I have learned how difficult it can be, and I didn't even start off with a baby!  I was lucky enough to take on an 8 year old (though the older they are the more issues they come with too since you can't "mold" to your liking lol) and skip the diapers, potty training and terrible twos.  There is a lot of give (you) and take (them) and not much reward.  It truly is a thankless job.  And now that we have a teenager in the house, it is even harder.  You can see the young child in them struggling to override those awful hormones, but hormones win 90% of the time!  At the end of the day, it is still a great job.

For those mothers out there who deal with all of these things and then they raise a child with an illness, well you are saints.  So here is a little thank you from me to you (not all encompassing since I know I will miss a few!).

Thank you for:

taking us to all those doctor appointments
not letting us dwell on our illness
letting us live our lives
not keeping us bubbled up
feeding, clothing, bathing etc us when we were sick
telling us it would be ok
visiting us in the hospital
holding our hands when we got bad news (and good news!)
raising awareness and money for us
fighting for us in schools for what we needed to succeed
watching us grow into the people we are today because of you
helping us figure ourselves out
not letting us give up
being the best support system we could ask for
hugs
kisses
love
kindness
the kick in the ass we needed when we rebelled against the illness
knowing the illness didn't define us and letting us figure out what did
watching as our dreams came true
watching as our dreams crashed down (and holding our hands)
lifting us up
bringing us back to reality
nagging us to do our meds
nagging us to eat
nagging us to play outside
nagging us to be responsible
and well, just EVERYTHING


Please post any thank yous in the comments.

And thank you MOMS!!! (and single dads who have to do both!!!)

Making Great Strides in CF

Holy Shit!  I can't believe how long it has been since I last blogged.  Life has been super busy for me.

On April 8th I was fortunate to take part in a guidelines meeting at the CFF in Bethesda.  I felt truly honored to be a part of something so important.  Plus I got to see an old nurse from 1990-1996 and see the CF director of my old clinic in Philly.  To be involved was very gratifying.  I try to do my part, I raise awareness, I make fun crochet goodies for CF fundraisers, I do Great Strides and I blog (well maybe not so much anymore lol).  But this, this was different.  This is something that will affect all of us CF adults in the future.  And it was amazing.  So much so I filled out an application to be a part of the patient advisory council!!!

Currently I am looking into organizing two paint night fundraisers for my walk team.  One will be in the Philly area and the other in the Boston area.  As soon as I get dates and details nailed down I will be sure to post.

I was inpatient in both November and January/February.  The January admission was much needed.  I started pulmonary rehab and I was on 3L of O2 with exercise!!!  I am back to not needing it now that I am out thankfully.  But I was super low, the lowest I have been since I moved up here.  I didn't even get a chance to prepare for my admission.  I went to see her on Tuesday and I wanted to go visit my family for "Christmas" that weekend and I had to cancel.  I talked her into letting me wait one night so I could get my things together etc.  Yeah it was that bad.  But I started to feel better in a week or so.  Unfortunately I didn't bounce back like I thought I would.  I was down to 27% and I only bounced up to 31%.  Down from 34% in December.  I go back April 28th and we will see if I jumped anymore now that the cold weather is gone.

Now for clinic update.  I have been considering switching my CF clinic.  Currently I am seen at the Children's hospital.  Now while I love my doctor, I am not keen on the whole children's thing.  Plus there is a disconnect between the clinic and inpatient.  And there is a lot of pressure to do home cleanouts vs inpatient because of the cost of CF care inpatient to the hospital.  So I met with the NP over at MGH today and I really liked it.  I have to decide what to do soon.  Like I said before, I have clinic on the 28th and I told the NP I would make a decision after that.  My pro con list has been read and re-read hundreds of times.  It is not a choice I can make lightly.  But on the easier side, I can keep all my cancer care and transplant appointments at BWH for the foreseeable future.  I asked both about switching CF centers and that has no bearing on either thankfully.

So there you have it.  Six months of updates in a few paragraphs.  I will be sure to update after my appointment.  And on anything else that may arise in the meantime.  I would like to get back to blogging so bear with me while I get in the habit again <3

Not sure what to do with my time

I am so booooooored!!!

I have spent the past three years working my ass off day and night to write papers and read book after book for school.  So now I find myself with all this time.  Time to sit and think and contemplate and I just don't like it.  I already started a to-do list but I don't want to knock through all 28 items in just a few weeks.  And a few of them are for later in the year.  Some cost money and those I just can't do yet.

I have never been one to sit on my ass and do nothing.  That is why getting sicker worries me.  I get bored easily.  I don't need to be totally immersed in something, but I hate not having a set goal or a routine.  Before school ended I had my days planned pretty much.  There was always school work, housework and the gym.  Now its just housework and the gym.  I don't have many crochet orders (actually I have none at the moment just some random projects I am working on), but even those aren't holding my interest.

It has only been what 12 days since graduation and I am already going stir crazy.  I need to come up with a routine quickly.  It would help if the weather would turn summery!!!  This 58* and cloudy weather is not helping me at all.

I can't go back to work.  There is no more schooling for me to do.  TV is boring as all hell.  I can't find anything on Netflix.  And I think I am in a rut lol.  Maybe I need a few days to sleep and then I will feel energized to do things?

Surviving

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.

WHY???

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...

PRIDE

Pride

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

Pride

It's one of my many faults.

Pride

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.

Reflecting on the past 15 years of my life

Fifteen years ago yesterday I graduated from high school.  So much has changed in those years...

Random highlights:
January 1998 President Clinton's "I did not have sexual relations with that woman, Ms Lewinsky."
April 1998, Animal Kingdom at WDW opened for the first time
May 1998 Frank Sinatra dies
September 1998 Google is founded
November 1998 Elmo's World launches
(source secondary source)

CF Related Highlights since 1998:
1998 Specialized clinical research centers are designated as the Foundation’s Therapeutics Development Network.
2000 Foundation-supported scientists map the entire genetic structure of the most common cause of CF lung infections — the Pseudomonas aeruginosa bacterium. Researchers can identify the function of specific genes and find ways to turn off the bad ones.
2003 CFFT-supported scientists at Structural GenomiX, Inc., determine the three-dimensional structure of a portion of the CFTR protein, opening the door to more drug discovery opportunities.
2004 CFFT-supported studies in Australia and at the University of North Carolina show that hypertonic saline helps clear CF mucus. It is proven to improve lung function and reduce hospital stays, and becomes a therapeutic option.
2006 VX-770, an oral drug in development by Vertex Pharmaceuticals, Inc., with support from the Foundation, enters clinical trials. VX-770 is one of the first compounds to attack the root cause of CF, and works at the cellular level to open chloride channels that do not function correctly in people with the disease.
2007 Vertex selects a second potential drug known as VX-809 for development. Like VX-770, VX-809 addresses the root cause of CF, but it works by helping the defective CF protein move to its proper place in the cell.
2008 The Foundation and Vertex achieve a “proof of concept,” showing that it is possible to treat the root cause of CF. During Phase 2 studies of VX-770, trial participants, all of whom carry the G551D mutation of CF, show unprecedented improvements in key signs of the disease.
2010 The FDA approves a new inhaled antibiotic called Cayston® (aztreonam for inhalation solution) for the treatment of CF. Developed by Gilead Sciences, Inc., Cayston offers a much-needed antibiotic alternative for CF patients who battle recurrent lung infections and develop resistance to existing antibiotics.
2011 The Foundation announces that Phase 3 clinical trials of VX-770 showed profound results. Those receiving the drug demonstrated the highest increase on a lung function test seen in any clinical trial of a CF drug. Vertex submits a New Drug Application to the FDA for VX-770 under the trade name Kalydeco™.
2011  Results from the first part of an ongoing Phase 2 trial testing Kalydeco in combination with VX-809 show promising results in people with the most common CF mutation, Delta F508.
2012 The FDA approves Kalydeco™ for people with the G551D mutation of CF ages 6 and older. The drug is the first to address the underlying cause of CF and opens exciting new doors to research and development that may lead to a cure for all people living with the disease.
2012 Results from a Phase 2 trial of Kalydeco in combination with VX-809 show a significant improvement in lung function in people with two copies of the most common CF mutation, Delta F508.
(source)

For me:

I am not where I had expected I would be 15 years post-graduation.  I thought I would be an interior designer working at some high level company in Philadelphia, married with kids, living in a beautiful home in the suburbs.

I am not where I expected I would be, but I am where I WANT to be. My health may be shit, my ability to work may be gone for now, but I am married to the most amazing man I could ever dream of and I am the step-mother to two kids.  They may not be my own flesh and blood, but they I do everything in my power to make sure they will be contributing members of society, and good ones.

I may not have the biggest house, or hell, even live in Pennsylvania anymore, but I have a great life and I would not trade it for the world!!!

I never thought that 15 years later I would be living in Boston.  I never thought I would have married and honeymooned in WDW.  I never thought I would have my baby making parts removed to reduce the risk of cancer.  I never thought I would ever be referred to a double lung transplant clinic.  I never thought I would ever meet the man of my dreams online, THANKS to CF!  I never thought I would make so many wonderful friends because of this retched disease.  And I never thought I would ever stop working.  Then again, I did always think I would be dead at 26 or by 40...so...

CF Awareness Post 1

My wonderful CF friend Cindy asked why are people with cf so much BETTER than everyone else? I mean, they are clearly superior to the rest of the human race. What causes that?

My answer on FB was:

Well Cindy, we CFers are so rad because someone or something gave us such shitty genes they decided we need the AWESOME genes to make up for it. So we are some bad-ass, sexy, down to earth, mo-fo's who take shit from no one. We rock.

The longer version is this:

CFers are amazing and awesome because we deal with a ton of shit on a daily basis and the only way to get through it is to be awesome.  We need to have kick ass personalities to make up for the short life span and the coughing uncontrollably at the most awkward moments.  Our sense of humor needs to be such that we can laugh at ourselves over the silliest and most disgusting things.  We need to live life to the fullest in only a short time so we don't have time to waste on the stupid shit.  

Frankly, we are who we are because of CF.  And no one can take that away from us ;)  Whether you think your CFer is awesome or not, they are fighting a hard battle that only gets worse as the years go on.  Remember that the next time they are too tired to do something, or just want to hang out at home.  We are stubborn as all hell too and won't tell you when we feel like shit, unless we really feel shitty.  Sometimes saying, "I'm a little tired let's stay in," means I feel like shit and a need a rest.  

Treat your CFer with kindness and respect and love.  We all want and need it, CF or not.

Sorry for the Slacking

I just realized that its been almost 2 weeks since I posted!!!  I am slacking and I apologize for that.  Life has been hectic around here.

Last Monday, as most of you know, the Boston Marathon was bombed, killing 3 and wounding over 200 people.  Friday one suspect was killed and the other was taken into custody at a Boston hospital where he remains.  Living in the suburbs of Boston this was a scary event.  When they shut Watertown down (I have a friend that lives there) I was nervous for her and nervous that the guy got out.  Friday was filled with ups and downs as the country watched the events unfold.  We were thrilled when the guy was captured.  Now we can rest easier in this great city.

Besides the tragic events that unfolded last week, I have been swamped with school work.  I have two papers due May 2nd, one of which is written, just needs to be proofread.  The other I have started to write, but I also have another paper due this Thursday I am concentrating on.  Plus my assistanship work needs to be finished by May 1st.  I don't think I went to bed before 2am all last week.  And up again early to get going on the work.  I have not slacked on exercising which is usually the first thing I cut out.  I have forced myself to go every day no matter what.  I read on the treadmill so the readings for class at least get done.

I have also been stressing a bit too.  Next Thursday, the day both final papers are due, I am also presenting at my school's Graduate Research Day seminar.  I have to get up in front of students and teachers and read my paper from last semester, then answer questions people may have.  I am SCARED SHITLESS.  Luckily I have an Ativan left over from my MRI a few weeks ago.  I plan on taking that bad boy shortly before I get up there.  My professor said to plan for 20 minutes in front of them.  Fabulous....Family and friends are welcome so P is going to see about coming with E.  I am honored and I refuse to turn away such a great opportunity.  This will look amazing on my academic resume as well as just my work one.  My paper is on Queen Elizabeth I and her involvement with the Puritans during her reign.  Who knew that working my ass off last semester while trying to plan a wedding, get married, have an extended honeymoon, and lose 2 friends would make me write a dam good paper.  I knew it was good, I loved it when I wrote it and re-read it.  But I had no idea it was THAT good lol.  I should really take more credit for the hard work I put in to school huh?

I have transplant clinic on Thursday.  We shall see how that goes...

Death Wrapped in a Blanket Fort

The death of friends with CF open up many doors.  There are the floodgates of tears when you lose a friend.  There are the floodgates of emotions for someone who died from your disease.  But there are also the doors with a bunch of unanswered questions behind them.  And you find yourself asking these questions on a blog at 1am (then modifying them in the morning).

Did they know they were dying?
Did they know they were being taken off of life support?
When they were told they needed to be vented were they scared and worried they wouldn't come off of it?
What was the end like for them?
Did they know it was there?
Did they hear people talking, know people were near them?
Did they have any regrets?

You ask these questions because their death is your death.  You see their end as your end.  And though it may not happen for a few years, it still looms large over your head. Wanting answers to something you can never ask.  Wondering how it will be for you.  If their death really will be your death.  And always, always wondering if you lived enough, loved enough, and left enough impressions that when you do die, you won't be forgotten easily.

For me I wonder if my new life is a true representation of me.  Do the people in my life know me?  Do I know me?  Will they say "oh she was x, y and z" and be right?  Or will my "original" friends say "no she was like a, b and c" and will they be right?

Death makes you look at your life in an entirely different light.  And though I have been fortunate enough to not lose someone since January, someone out there, on my friends list, may not be there tomorrow.  And each post about sickness, or more IVs, or the need for O2 continuously, reminds me of this.

I love each and every one of my friends, but some days, that blanket fort on the bed calls my name more ferociously than the previous day and it takes all my strength to not go and hide...

Groundhog Day

I wanted to write this long post about how I am feeling lately.  But I just don't feel like it.  And that statement alone sums it all up....I am in a funk....I need to get out of it.  I am blah with a capital B L A H.

I think a lot of it has to do with the lack of female body parts, and the lack of being able to see someone about it.  My surgeon told me I have to see my PCP, but I can't get in to my PCP until after May!  If I didn't like her so much I would ditch her and find someone new....P wants to anyway.  Maybe after February I will consider it.

If you ever saw the movie Groundhog Day, that is how my life feels right now....same thing day in and day out.  I HATE IT.  I need change.  I need something different.  Not P, definitely NOT P.  I love him, he needs to stay where he is.  But the rest....I dunno.......

*sigh*

I need to have Patience and Faith...

I have some friends on Facebook that are old coworkers.  These are people I spent 40-50+ hours a week with prior to my health taking a dive and having to quit.  I enjoy reading up on their lives and seeing what they are up to now.

But at the same time I hate seeing how they are progressing in their careers and life and I am sitting here, idle, waiting for my chance to excel again.  And knowing that there is a chance that I may never work or accomplish my dreams ever again.

I miss the working life so much.  Some days I want to delete these people from my friends list so I don't have to see the advancements they are making, or the babies they are having.

But why should I do that?  And its not them, it really is ME.  I need to just get over my life and remember that each person is different and each person lives life differently.  This life is what is destined for me and no matter what, I can not change it.  I can't change the fact that I will NEVER have a baby of my own.  Or that I can't work full time right now because of my health.  I could work, I could spend every free second I have making sure my health stays stable, and I could miss out on my family.  Or I could continue on this path and have patience that one day I WILL go back to work, and that I WILL be amazing, and I WILL do all that I have always wanted to do.

I need to have patience and faith.....

A Fun Filled Weekend!

We had a great fun filled weekend.  On Saturday we did family day at Canobie Lake Park.  Its a small amusement park with water rides, regular rides and all the bad food you can eat.  We made sure to get in right when it opened so that some of the rides would have shorter lines.  Worked well.  I personally do not do rides and only went on a few (log flume, sky ride, antique cars).  Nothing of any speed or "thrill".  Fine by me I am not a rides person.

P, E, L and A went on just about everything that they could.  P is a nut and just loves rides!

This is the view from the top of the log flume before we dropped down.  Yes I screamed like a baby!

This is the view looking down from the sky ride.  I admit I was nervous on this too!

This is Un-tamed.  P, E, L and A went on this and I think they all had a blast!  L screamed  bloody murder and P looked like he was having the time of his life!

Today we went and finally picked up my new bike.  P and I got new ones last weekend but we had Toys R Us put mine together (P's was the floor model).  Honestly I would never let them do it again.  But hey, 18 speeds we never would have figured it out.  So instead we are going to have people who know what they are doing look them over and fix things.

After we got the bike, P, E and I went for a bike ride.  It was great!  I forgot how much work it is to pedal on a real bike not a stationary one in the gym.  Unfortunately I will need to wear my O2 for this as well.  After going up a rather small hill I felt my heart racing and like it was going to jump out of my throat.  Checked and it was 178!  O2 was 92% though so go me ha!  178 is way too high and I don't need to go into cardiac arrest on a bike ride.

It was a great exercise filled weekend and I am looking forward to a somewhat relaxing week with small fun things planned to do with E.

Everything Happens for a Reason?

Perhaps!

Tonight in class I found out that I was awarded a research fellowship!  It is with my current professor for the fall and spring terms of this upcoming school year.  I can't tell you how absolutely STOKED I am about this! My professor told me about it in class tonight.  I have not gotten any official correspondence yet, but the names are sent to the professor for approval so he showed me the email and it is ME!  If I had decided to apply for that job AND gotten it then I would be screwed.  So everything works out in the end.

Here is the description:

Graduate Research Assistantships are designed to link a graduate student with a faculty member on a meaningful research project.  During the assistantship period, the graduate research assistant will work directly with a faculty member on a project, which may lead to a presentation at a conference, a joint publication, or other significant professional activity.  The assistantships are awarded to students who are fully admitted into a master’s degree program.  They are intended to encourage and assist superior students in pursuing their course of graduate study; to provide students with professional development opportunities, and to provide support for faculty research, scholarship, and creative activities.  The assistantships are competitive and are based on the requirements listed below.  The assistantship includes 10 hours of work per week at $12 an hour in the fall and spring semesters when classes are in session.  Assistants receive full tuition remission for fall and spring semesters when classes are in session, except for consortia, directed studies and theses courses.  The student is responsible for all associated course fees.

Decision Made

Well as much as I was thinking that Wayne Gretsky was correct when he said "you miss 100% of the shots you never take", I had to accept the realization that I just should not apply for the job.

Believe me, I would love to.  But the truth is, I am not the type of person to just work somewhere for 3 months and then quit.  Even if I hate it I will stay much longer than needed.  If I were to get sick again and need to stop in 3 or even 6 months I would feel obligated to work twice as long and end up much sicker than I am now.

So for me, the choice to bypass this opportunity is the right one.  I can finish school without any worries.  I can concentrate on my health.  And in 18 months if they are still hiring or hiring again I can apply and see how it goes.  Even if I don't have my transplant by then.  But at least I can give it my all at that point and not have to juggle school, full time work and a family.

Its a tough choice but it had to be made.  I remember how long I struggled on deciding to stop working 30 months ago.  I struggled for over a year with the choice before finally agreeing that it was worth my while to do it.  I don't think I have that kind of leeway anymore with my health.

Oh well....life goes on eh?

Self Perception

Don't worry this isn't another weight related post.  This one is all about the inside.

With Kalydeco's wonderful results and the start of phase 3 for Vertex 809 (combo med for those with DF508)  with amazing results in phase 2, its a possibility that we could stop CF progression where it is right now in some patients and perhaps even reverse some of the damage.  This is a huge step in the right direction to make CF much more manageable.

But it brings into ones mind some thoughts that wouldn't be there otherwise.  For me these thoughts always played a part deep down in my thinking.  I have often wondered what a "cure" would mean to me.  Disclaimer Kalydeco is NOT a cure.  It restores function to a non working protein in the cell.  CF is still CF, this med just helps the underlying cause and helps to fix it.

I hover around 40-43% FEV1 when I am healthy.  My shortness of breath is daily.  My ability to function normally has decreased drastically over the past 4 years.  Yet this new med could help me gain some of that all back.  I could potentially go back to work before receiving a double lung transplant.  I could stay off that list for many many more years.

However, there is also the negative side to this thinking that we must dive into.  What if I don't last long enough for 809 to be approved and started?  I am not content where I am right now, feeling like my life is on hold until I get a transplant, whether that is 1 year or 10 years from now.  I feel like I have stopped everything and I am not truly living.  I struggle with wanting to take my meds and doing everything by the book because if I don't I would get sicker faster and then transplant could come sooner and I could start my life over.  But we all know that transplant is not a guarantee.  I might not survive the surgery.  Or I might not make it home after waking up and trying to learn to live with new lungs.

But having my original lungs offers more of a guarantee to life than transplant does.  What I need to do is change my perception so that I can become more comfortable with my life as it is now.

I need to look at what I AM doing and focus on the positives there.  I need to step back and realize that I AM living my life right now, in preparation for when I can breathe again and do everything I have always wanted to do again.

I am a stay at home wife/mother who goes to graduate school and takes care of her health on a daily basis.  I am doing what I need to do now so that in 5 years I can do what I want to do then.  My life is not on hold yet.  My life is progressing different than anticipated but it is still progressing.  And I need to focus on THAT and not the what ifs.

Kalydeco could allow me to finish my degree and get a job before I get transplanted.  Or it might do nothing and I will still need the transplant.  But I have to continue on as if I am going to live forever and one day live and breathe with shiny new healthy lungs.

Something to do with Myself

Lately I have been feeling really antsy and bored with my life.  The whole not working thing has finally caught up with me.  Not too shabby that I have been on SSDI for almost 2 years now and just starting to get bored.  Of course last year I did 9 months of work and I have been in school, so that lessened the boredom load.

However, the past few weeks I have just been on edge.  And it isn't a depression type edge where I need my meds again.  Its just a feeling of not doing anything and doing the same things over and over.

I thought not working would be absolutely FABULOUS!  And it was for a while.  I worked on my health, I crochet'd, I read a bunch of books, I went back to school, I started cooking dinner most nights, I got to sleep in every day, I could visit family in PA whenever I felt like it.  But then you watch the same shows over and over again.  You crochet enough that your hands hurt.  You read as many books as you can.  You are broke and can't take those trips to PA as often. And then you have nothing.  Believe me, there are more books for me to read and more crocheting to be done, but I need something else.  I need to be doing something that takes me out of the home.  Having more friends would really help, but I am at a disadvantage there.

So instead, a fellow Cyster hooked me up at a local farm that does lessons for the disabled, on horseback.  How awesome is that?!?! I went today to shadow her so I could get an idea of what goes on.  Especially because the website mentions jogging with the horses for short stints.  I was a bit nervous about that, but after being there today I am confident I can handle it.  It felt great being around the horses again and to be doing something productive!

I have to send my application in and then I can start.  Just once a week I think for now as I don't want to overdo it.  I was there almost 2 hours and that was plenty for me.  Not that I wanted to leave per say, but I could feel myself getting tired.  There are some hills to walk up, nothing steep or long, but they do take their toll on you.  Especially since I am sore from all the exercise I have been doing.  I am really looking forward to starting and can't wait to help make a difference!

I Miss Working

Yep I said it.   I miss the dressing up, doing my hair, social interactions of a job.  A steady 9-5 everyday job.

Yesterday I had my first OGTT (Oral Glucose Tolerance Test) - for another blog another day I promise - and on my way I drove past a small corporate park right on Rte 1.  Its down the street from my house, maybe 10 minutes to get to it depending on traffic of course.  But I found myself wondering what places of business were in there and if they were hiring for secretary - oh sorry administrative assistant - positions and how much they paid.  I found myself day dreaming about getting up in the morning and showering, getting dressed and heading to work.  Doing the same old boring paperwork day after day but thankful that I had a job and was getting out of the house.  Enjoying the paycheck that I was bringing home weekly and relishing that I was again contributing to society.

Then reality hit.  I was exhausted from being up at 7am so I could be in Boston by 10am.  I was SOB walking into the CT clinic to have the test done.  I went home and slept for 2.5 hours because I was so tired, which beat out exercising.

If I went back to work I would have to go to bed at like 9pm, or earlier, in order to get enough sleep to possibly stay awake for the full day.  I wouldn't be able to exercise because I would be so tired all the time.  I wouldn't be able to do any type of housework or cook because I would be exhausted all the time.  I would be a miserable bitch because I would be exhausted all the time.  I wouldn't want to do anything on the weekends except stay in bed because I would be exhausted all the time.

Working again, 40+ hours a week on a set schedule is not doable for me.  Its my reality.  I need to sleep when I can.  I need to be able to rest a whole day if needed - and not just on the weekend.  I need to have the flexibility to be hospitalized when needed and not worry about my job.  It sucks and I want to work again.  I truly do.  I miss all that comes with working, yes even the days where I was bored out of my skull with nothing to do.  Because at least I was out of the house, making a living and not depending on SSDI to pay all my bills for me.

I guess it is something I can look forward to post transplant...

Ringing in the New Year!

2012 is sure to be filled with a ton of excitement. P and I get married in October, then go on our honeymoon to Hawaii.  My brother turns 30 around Thanksgiving so we are going to celebrate it with my family in PA. I am continuing my Master's classes (thanks to the disability department at school for getting my financial aid sorted out). Many new babies and weddings this year for friends and family.  Plus any exciting things that may happen along the way.  I am looking forward to a new year filled with exciting times and new adventures.

I have set some resolutions for myself for this upcoming year.  With no pressure though. These are things that I need to work on either way so I am adding them to my resolution list.  

1. Use my Wii fit to exercise and get my exercise age down from 44 to something closer to 31. I want to make sure I am not on O2 24/7 when I walk down the aisle to marry the love of my life.

2. Eat healthier foods, and cook more so the whole family eats better.  We all need to eat better and stop with the processed junk foods.

3. Pay more attention to world happenings. I am ignorant to what is going on in the world. The news always depresses me but then I don't know anything of importance.

As I ring in the new year tonight with my soon to be hubby I reflect back on the 2011 year.  So much happened! P proposed to me (by far the most exciting!). I started grad school.  We had our first family vacation together. We got a dog. I started the transplant evaluation process. My family got to visit me and see my new home.  P bought a new car. I got my hysterectomy.  My afghan won 1st prize at the local fair.  And that is all I can remember right now.  There were many little moments through out but these are the most memorable.  Unfortunately not everything was happy. We lost many CF friends.  The greatest lost for me was Bree.  I still miss her terribly and hope she is enjoying her time with those she loved and lost before her.  

I hope everyone has a happy and healthy new year and takes whatever life hands them in stride.  There will be good times and bad times but through it all we will press on.  May you all be safe this evening!!!