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…

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 :)

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....

10 Weeks Post Whipple

It's about time that I update this thing.  I have been really busy and that is great!

Things are pretty much back to normal here.  There is still some pain in my lower ab area but only when I cough.  I think my muscles are recovering and the trauma of coughing every few minutes is not helping.  It will go away in time I am sure.  The skin around my incision is now peeling.  I started using Mederma on it to make it less red and that's when I started peeling.  Related?  Maybe.  Not too worried though.

As far as what I was expecting post things are different.  I do not need insulin thankfully and I do still need enzymes of course.  My body does not seem to have changed much missing a piece of my pancreas.  One thing that changed is my need for Miralax daily.  I no longer need it every day.  I am still working on when to take it though and it could be a few months till I am back to knowing and on a schedule.

The results of the surgery showed that the cyst was entirely related to CF.  There were no signs of cancer at all.  Great news!!!  It does mean this surgery was technically not needed but I am glad to have had it done.  Now any other cysts that grow large like this one and read the way this one did we can chalk up to CF and not Lynch.  Transplant was pleased with the results of course and are happy to have me as a candidate again.

Here is a picture of my torso.  Its a little outdated now but nothing has changed on the recent picture.  Except maybe a few more pounds added to me ;)

Overall I feel pretty good.  My lung function was down a good amount at my clinic visit 2 weeks ago so we started oral cipro to see if it gives me a boost.  I didn't feel as low as I was but it makes sense I am down.  For the first few weeks after surgery I could not cough like I usually do.  So things have been sitting and brewing in there.  The past 7 or so weeks I have been able to cough better and to get stuff up.  My FEV1 was at .89L (29%) down from 1.15L (38%) in July before surgery.  So yeah a significant drop.

My dilemma is this though.  Why is my FEV1 so low, yet I feel like I am functioning at a much higher one?  I know I know, everyone is different, it just seems so strange to me to have an FEV1 of 29% and yet still be able to carry boxes up and down steps (slowly and immediately after my nebulized treatments and only a couple with breaks and coughing fits - but I do it!).  Why is it that I can jog on the treadmill (even if only for less than 60 seconds at a time) if my numbers are such shit?  It is this aspect of CF I just never understand.  I see 29% and I think that I should be home bound and on O2, yet I am not.  I use it to sleep and I need it if I plan on taking my running any farther.  Quality of life ey? LOL

So that is it for me for now.  I am sure that the Cipro will do nothing for me and I will end up in patient the beginning of November.  But at least I will be feeling great for the upcoming holidays!!!

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...

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...

New Name

I am debating giving my blog a new name.  In case you hadn't noticed, I revamped it some.  But now the name seems so generic.

Should I rename it?

Should I leave it alone?

Should I go to bed instead of thinking of silly things at 1am?

Vanilla CF

What is Vanilla Cystic Fibrosis?

My old CF doctor used to tell me I had Vanilla CF....meaning I was very low key, by the book, nothing out of the ordinary.  As I have gotten older I have gotten worse, but there have been no sudden drops which I was unable to gain back.  I haven't really scared anyone (besides that PICC issue a few years ago!) with a horrible case of anything.  As it stands now, Cancer is pretty much more worrisome to me than CF is.  Probably unjustly so, but that is how I see it.

Sounds pretty decent right?

So why do I feel so undeserving of it?

What do I mean?

Why do I feel like it isn't fair that I seem so "healthy" and I have friends out there struggling every day?

Or why do I feel like it isn't fair that I seem so "healthy" when I have friends who are dead?

Every day on Facebook there is another friend who is asking for prayers because their health is wacky.  Or another who is waiting to hear if their issue is their body rejecting their new lungs.  Or yet another who is on the mend from months of being in a shitty state.

While I thank God that I am healthy, I also wonder, why me?  Survivor's guilt perhaps?

Just some late night thoughts.....

And to lighten it up (HA)....I chopped my hair off today!  I am donating it to Locks of Love!!!  I know I know, Pantene doesn't charge for their wigs, but I color my hair and Pantene won't take permanent dyes, while LoL will.  12" chopped off!  I feel so naked without my hair!!!

Sleeping

A friend of mine posted about sleep patterns with advancing stages of CF and the amount of sleep needed.  It got me thinking.  Am I sleeping as much as I NEED or as much as I WANT?  And that sleep I am getting, is it detrimental to my health instead of helping?

I usually go to bed around midnight and wake up around 6:30 when P gets up.  They leave around 7:15 and I go back to sleep.  Sometimes right away and sometimes it takes over an hour.  Lately its been the latter.  I have been having some sleeping issues and I don't like it.  Starting with waking up a few times a night.  This is not normal for me at all.  Then not being able to get back to sleep after they leave in the morning.  Its brutal!  I even upped my Melatonin intake to try to combat it, but to no avail.

Why do I sleep so much?  Do I need to get the 10 hours I aim for?  Or do I just like sleeping that much?  And if it is the latter, am I harming my body by sleeping that much more?

I have to wonder if I sleep because I am bored.  Let's think about this.  What else am I going to do during the day?  Watch TV?  Shop? Surf the net?  Its not like I have a slew of friends I can hang out with, or family I can visit.  Crafts take money, which I am lacking on.  School work only takes up so much time.  So maybe I sleep so I don't have to face the boredom of not working.  Its very possible.  But sleeping that much when I don't need to can't be all that great for my health.  Sure rest is rejuvenating, but too much is harmful....right?

Maybe I need to just get up when I wake up and then the restlessness will go away.  If I get bored then I can figure something out.

I need to win the lottery!

Medications...Today vs 3 Years Ago

First order of business is check out this post by fellow bloggy Cystic Gal.  She talks about our mutual influence in a semi-recent online study of influential CF bloggers :)  Yay to us!

This study, conducted by the makers of TOBI, is pretty nifty.  The data was taken in 2009 when I was still a fairly new blogger.  My stats have changed since then.  And the fact that I now have Twitter too.

But check it out yourself:
2011 CLIO Awards

This award/research thingy got my mind thinking last night.  Coupled with my lack of health insurance (see previous post about that one), I had much to ponder as I tried to fall asleep last night.

What did I ponder about?  Medications.  Or in my case lack of medications.  In reality I don't take that many CF medications.  Most of the daily pills in my sorter are vitamins and supplements.  For example....I take Women's One A Day multi vitamin, Calcium, Vitamin C, Vitamin D, Vitamin E, Motrin, stool softener, and melatonin.  The medications I take are Singulair, Prevacid and Azithromycin in pill form at least.  Once I stopped the anti-depressant and the hormones I have nothing!  There is Creon 24 with meals, DuoNeb 2-3 times a day, Pulmozyme once a day, 3% 1-2 times a day and Symbicort.

Gone are the days where I was taking medication after medication.  No longer do I take oral antibiotics (2-3 at a time) or inhaled antibiotics (off month I added Cipro oral).  My routine seems so slim compared to so many others.  I can do my morning and night treatments in an hour each because I don't have to use all that extra stuff.  Sounds great huh?  But the reasoning sucks.  My lung bugs don't respond to the orals like they used to, and my lungs themselves don't like anything being inhaled into them besides the ones I do already (the exception might be Symbicort.  I have noticed some tightness when I do it vs when I don't).

Its so weird to me, to read about 3 hour treatment schedules for other CFers when I barely do that in a day.  But I have been there. I have done the 2 hour treatment, the 1 hour rushed treatment and the finish in the car treatment.  I feel like the backwards patient.  The one whose regimen gets easier as she gets sicker.

So. Freaking. Weird.

A Beautiful Fall Day for Pondering

Today I sat out back watching my dog sniffing around and digging in the dirt.  I was on the last step of the deck stairs, comfortably dressed in sweats and socks.  The cool breeze felt wonderful on my face.  I sat there admiring the leaves changing, thinking how pretty they looked against a steel gray sky.  This is the reason I chose October for our wedding.

Then my mind drifted to how I felt physically.  I took a somewhat deep breath (for me anyway) and let it out slowly.  I could fill myself up, apparently halfway, according to the PFT machine.  Inside I could feel the rumble of some mucus.  Totally different than only a few days ago when I was crystal clear of goo.  My exhale had a hint of crackles in it.  I lasted a whole 4 days off of IVs before the mucus came back.  But I don't mind.  You see, I got over a week of clear, unobstructed airway inhaling.  I didn't hear crackles, I didn't feel crackles, and my doctor said I sounded amazing.  That was the best part.  I sounded amazing!  Me, going through a transplant evaluation sounding amazing!  50% of my airways were working.  How awesome is that, and considering I don't think I have ever been above 80% it is REALLY amazing.

However, this 50% and the 50% I was at 3 years ago feel entirely different.  How so I bet you are wondering? Well its not just a number.  Yes, the number itself is important for many reasons, but it really doesn't paint a great picture of how you feel.  The only thing that is similar is my coughing.  This type of cough I have had forever.  Sometimes it is mucus filled and other times it is entirely inflammation.  But either way the cough is there.  The differences are a much longer list....

Energy level - now I NEED 10 hours of sleep to function and not be miserable.  Then I was sleeping 5 maybe 6 hours a night and functioning like a champ.

Lung pain - it was almost non-existent 3 years ago.  Today I am always in some sort of lung pain.  It varies from just barely there to OMG where is the Motrin.

Mucus - I used to rarely cough stuff up.  It was there sometimes but it was very difficult to get it up.  Now (notwithstanding this resent clean-out) I can cough up goo almost daily.  Before green scared me.  Now it is a color I am used to seeing.

Exercise - though I couldn't run a mile, I could dance all night long, with a few breaks for coughing fits and some rest.  Now even the thought of dancing all night makes me want to sleep for a week!  Also the need for O2 with exercise is new from this last 50%.  Yes I did monitor my O2 and though I don't dip far I still go into the upper 80s, sometimes lower (like class on Tuesday when I walked in my O2 was 84% lol).

Travel - Loved it and it didn't bother me at all.  I could travel all the time and I didn't need to rest up like I do now.  It takes me a few days to recover from traveling now.  Whether its plane, train or car I am exhausted after visiting family and friends.  Mainly because of lack of sleep which is in point number 1.

So you see, I might be at 50% lung function right now, but I feel more like my baseline of 40%.  I guess on October 26th we will see what I am at by then, as that is my follow up transplant appointment.

Something Taboo - Death

I wanted to write about something that most people seem to hate to talk about - Death. So many people are afraid to die, and based on their religious or spiritual beliefs, there are many reasons for this. So based on my beliefs I want to talk about it.

I must first warn against any religious conversations starting. Remember this is MY opinion and my beliefs. So please keep that in mind when reading through this blog post. And also, I am not anywhere near death it is just something I have been thinking about lately.

I am not scared at all to die. Rather, I am scared for those left behind.

When I pass away all my dreams, goals and desires will end. I will have no regrets as I won't know anything after the time my body takes its last breath. However, those left behind will feel the sorrow, the pain, the shattered hopes. They will know I didn't do what I wanted or that I never got to see things I wanted to. They will live with the regrets, if they do, not me.

My niece and nephew will remember me and the times we shared together, but will it be enough? Will they regret that I didn't live closer and spent more time with them? Will they be mad I passed away.

I like to think each and everyone of us goes to Heaven and can look down on those we left behind, but I don't think it is an actuality. I know that sounds crazy. Believing they are watching over relieves some of the pain. Believing I will see my grandparents, Maggie and friends after I pass is something that makes me smile. But I don't think that happens. If it did, then wouldn't I have regrets? Wouldn't I be sad watching over my niece and nephew, not being a part of their lives anymore? So while I like to think it does happen, I also don't want to think it does. Because then, my regrets can continue, my sadness over not being with my family long enough will be stronger, and I may continue that depression from earth into heaven.

So instead I think that when we die, we die. Maybe our souls float away and go somewhere that the rest of the souls are. But maybe we don't have any idea what or who we were. Maybe we are just a bunch of old souls hanging around, waiting to be placed in a new body. I have no idea. And now my post has gone totally off track lol.

So to end this little off course ramble...To me death is an end a total end for the deceased, while the survivors must carry on, remembering the good and bad of the deceased. Death is "easy" for the one who passes.....

Blogger Challenge!!

A great bloggy friend posted a challenge for us fighting chronic illnesses. So here is my challenge response:

I think that our generation - the 20+ year olds - with CF have it much different than the "newbies" being born today. Our parents weren't aware of as much, there wasn't as much known, the medications were different, the treatments were different, things were just plain different. However, we are still here, living, getting older, having babies and transplants. So somewhere along the lines our care transitioned over to the "new" care, but did it happen in time?

When I was younger I was beat on by my mom. I know I was supposed to do Albuterol with .09% saline as my nebulized treatment. I ran around like any normal kid did, only I coughed and hacked the whole time. And I ate like a pig. But my lung function was never high. Looking over my chart I have never been above 80% and really I don't even know if I made it that high. As far back as I can remember, being in the upper 50s was my thing. Maybe I would have been higher if I had been more compliant as a teen. But as my friend mentioned in her post, when you don't notice a difference its hard to continue on.

My medications didn't make me feel better. If anything they made me feel worse. I got shaky and coughed - which at the time I despised! I wanted that "normal" teenage life. I got better as I got older, especially after my first hospitalization at age 18. But I didn't truly become compliant until my mid-twenties. Then I discovered how hard life was without my medications. Compliance wasn't so much about helping my lungs live longer, it became helping me to breathe and live my life.

I sometimes wonder if I had taken all my medications the way I was supposed to from diagnosis on, would I be facing a transplant evaluation? Or would I still be working full time and pushing myself just as hard? I will never know, but I do know I don't regret one thing I did, or did not do. I lived those years of my life as I wanted. I complied with myself. If that sends the doctors to say no you can't be transplanted then I will deal with that. But I doubt it would. I am compliant now. I do my nebs, I take my pills, I do my vest and I exercise when I have the energy. I cough up the goo when I need to. I go on IVs when I need them. I listen and I am proactive. I have changed. People can change.

Diseases are very unpredictable. They can change at the drop of a hat. They make their own rules and they don't listen when they are screamed it. If someone does what they are supposed to do 24/7 they are still going to face the "true" disease. Maybe it will be pushed back a few years or maybe it won't. But no one knows that at the start. No one knows what their life is going to be - terminal illness or not. Should you be compliant? YES. But you need to figure out what compliance means to YOU. Do you allow yourself to miss a treatment here and there because of life? That's fine. Do you skip treatments daily and probably only do a few during the week? That's not fine - that's not compliance. Compliance is doing the best you can.

Now I pass the challenge torch on to my bloggy friends. Here are the "rules":

1. Write a blog explaining your personal thoughts and experiences in dealing with CF control and progression. This could include your views on whether CF is in fact a "controllable" disease, your personal definition of compliance, your thoughts on whether (or how) someone with CF should be judged in terms of "good enough" self-care (what makes you feel judged? do you think those fears are justified? is judgment ever useful in this context?), your own struggles with control vs. unpredictability, and how you keep motivated in the face of so many questions. Or, you know, whatever you want to write about really. It's your blog.

2. Comment below with a link to your blog so that all of us can read your response. YOU DO NOT NEED TO LINK TO MY BLOG IN YOUR ANSWER. If you'd like to do so, please feel free, but this is about starting a discussion, not publicity.

3. Encourage your own readers to get in on the conversation by posting the same instructions on your blog. Remember, the more responses, the better the conversation. Let's see if we can get this one going as much as with past challenges.

4. If you don't have a personal blog (or just don't feel like going through steps 1-3), feel free to still make yourself heard by simply leaving a comment with your thoughts below.

5. Non-CFers are 100% welcome to participate, either by pulling from their own experiences or simply by offering their perspective as people, friends, and loved ones.

Pondering the fatal infection

Do you ever have a hard time sleeping or falling asleep?  Is it because your mind won't shut off and let you just relax?  Do you think of stupid shit that you would much rather not think about, but if you had to, to at least do it during daylight hours?

Yeah that was me last night and this morning.

The passing of a friend on Friday has made a few of us remember that we need to get our advanced directives in order.  Most of us have thought about it and started the process but never got around to finishing it.  Another poster on CF2chat.com let us read some of what she had written down and encouraged us to "steal" what we wanted to for ours.  So Saturday night I spent 2 hours writing mine out.  Details like me not wanting to be kept on life support if I have no chance of recovery.  My desire to be buried with Maggie when I am cremated.  Things like that.  It felt good to get it all written down and printed out.  Now I just need to get it signed.

I guess last night my mind finally took those actions to heart.

I got scared lying in bed.

What if that nasty infection that can kill me, kills me soon?

See told you it was something stupid.  OK maybe not entirely stupid since it really could happen, but why think about it at 3am?

What if all the IVs I do and all the breathing treatments and all the therapy does not stop it?  What if I wake up one morning to horrible pain and find that my one lung has stopped working entirely?  What if I get so sick I can not be placed on the transplant list?

We think we will be strong and ready when our time comes, but will we?  I think back to my PICC fiasco (I know it is NOTHING like dying so please do not think I am making light of those who have passed) and how scared I was and all I kept thinking that this was not the way I wanted to die.  I had no idea what was going on.  All I knew was I couldn't breathe and there were doctors EVERYWHERE in my room.  I didn't want that to be my time and I am so thankful that it wasn't.

But how will I be if the infection ravages my body and I am laying there, in my bed, doped up on meds to help me relax and trying to breathe?  Will I have the strength to fight back?  Will I lay there and see what God has in store for me?  Will I just give up and let myself pass?

Those are the questions that kept me up partially last night, scared and afraid, but not willing to let myself speak it, only think it.

What is a cure?

I apparently opened a can of worms this morning on Twitter when I said a cure was a laughable matter.  In my opinion I do not think a cure for anything, including CF, will ever be found.  I have two reasons for thinking this.  One being financial gain and the other being what a true "cure" really is.

****** Before I go on, please remember.  THIS IS MY BLOG therefore MY OPINIONS.  No one is right, no one is wrong.  We all have different views on everything.  Please respect this.  Any negative name calling comments to me, or to anyone who responds will be deleted. If you want to be rude, at least show your face and don't post as anon, or if you do, leave your name.  *******

Let's start with the latter - what a true "cure" really is.  This will vary for everyone.  Some people feel that just being able to treat the disease effectively with meds, and allowing the person to live to the normal lifespan is a cure. I don't see it that way.  To ME a cure is eradicating the disease.  Wiping it out so it no longer exists.  This is not possible for many many diseases, maybe even all of them.  CF for example can never be wiped out unless every single person who procreates is tested and allows their unborn fetus to be scientifically "created" so no CF genes are carried on.  The money required for this is outstanding.  Not every single CF carrier or CF patient can afford this.  Once that person is born with CF, they are going to have CF in every single gene of their body until death.  CF is genetic, it is written into our DNA.  Unless we can totally change every single gene in the body and "fix" that one malfunction, we can't sure it.  And even if that gene is fixed after the child is born, there is some damage done.  That damage starts from the beginning...maybe that child would have been heavier at birth.  Maybe that child had MI when it was born which lead to the diagnosis, and now the damage from that is there.  Even if they were to be "cured" of the gene, the damage is done; yes insignificantly perhaps, but it is still done.

We can carry this through to other diseases.  Let's use Cancer as our next example.  In a way yes it is partially cured as is.  We can remove the breasts of a woman with breast cancer and she is essentially cured as long as it hadn't spread to any other organs.  We can remove the tumor filled colon and that person no longer has colon cancer.  But they can still get cancer, in other organs.  Cancer has not been cured, that person has just been ridden of the disease for now.  It may come back and kill them, or something else might, no one knows. But as long as cancer exists, it is not cured.

My other line of reasoning for why disease will never be cured, is that there is too much money to be gained from the meds used to treat diseases.  CF alone has a HUGE financial gain factor.  A months supply of Cayston is $5000.00.  Where that money goes I have no idea, but I highly doubt the owner of the drug company drives a used Saturn and lives in a two thousand square foot home.  Why would he/she want to give up their lifestyle when a cure is found and those meds that support that lifestyle are no longer needed.  Yes the medical field, research etc is supposed to be about helping others, but if you think that no one is in it for the money you are naive.  This country was founded by the rich for the rich (whole other topic) and anyone who can take advantage of the opportunities that the country offers.  Supplying medicines and technology for those who medically need it is just another business, like cars or clothing.  Only thing different is lives are at stake.

I am well aware I may lose some friends for posting this.  Some of my followers may decide I am a ruthless, cold hearted bitch.  That's fine.  Like I said in the beginning, this is my opinion.  Maybe I would feel different if it was my child with CF and not me.  I don't know.  But this is how I feel as of right now, just me and my unscientific opinion.  A cure may be found, and I hope I am proved wrong.  Believe me, its not that I don't want them to find a cure, its just that I don't think they will.

What would you do....

for a Klondike bar....lol

No, that's not really where I was going with that but it popped in my head when I wrote it so I had to go there.

What I was really going for was,what would you do with new lungs?  What prompted this was a post by fellow blogging Cyster Piper.  It got my wheels a turning and I just had to go with it.  She talks about her amazing life with new lungs.  I will quote one piece of it since it sounded like me in a few years when I am transplanted and breathing again like I should be.  "My friends are sick of me already because I guess they didn't realize that new lungs mean bigger lung capacity (i.e., the ability to talk for hours) and more energy to run around like a madwoman. My dog pretty much refuses to walk with me anymore since I rarely have the patience to stop as often as he would like. Everyone complains that I'm too fast for them to keep up with on the street -- and I revel in every second of it."

Me...I don't even know where to start.

I know I would go dancing.  I would ride a horse.  I would spend a day at the park running around.  I would chase after puppies.  I would laugh until I was blue in the face...and not from lack of O2 like now.  I would climb to the top of Bunker Hill.  I would do a walking tour of all my favorite cities.  I would play volleyball.  I would laugh all day long.  I would sing, even though it would be out of key.  I would go camping in the woods with NO electricity.  I would laugh for hours.  I would run.  I would travel.  I would laugh.

Notice a theme?  Laughing!  Yes that's right.  I can't wait to laugh and laugh and laugh and not turn blue and red from coughing afterwards.

What would YOU do with new lungs?

Letter from a Doctor

A friend of mine posted this on CF2chat a few days ago.  It is a great letter and really lets you see into the doctor's perspective of "dealing" with us chronically ill patients.

A Letter to Patients With Chronic Disease

Dear Patients: You have it very hard, much harder than most people understand.  Having sat for 16 years listening to the stories, seeing the tiredness in your eyes, hearing you try to describe the indescribable, I have come to understand that I too can’t understand what your lives are like.  How do you answer the question, “how do you feel?” when you’ve forgotten what “normal” feels like?  How do you deal with all of the people who think you are exaggerating your pain, your emotions, your fatigue?  How do you decide when to believe them or when to trust your own body?  How do you cope with living a life that won’t let you forget about your frailty, your limits, your mortality?


I can’t imagine.


But I do bring something to the table that you may not know.  I do have information that you can’t really understand because of your unique perspective, your battered world.  There is something that you need to understand that, while it won’t undo your pain, make your fatigue go away, or lift your emotions, it will help you.  It’s information without which you bring yourself more pain than you need suffer; it’s a truth that is a key to getting the help you need much easier than you have in the past.  It may not seem important, but trust me, it is.


You scare doctors.


No, I am not talking about the fear of disease, pain, or death.  I am not talking about doctors being afraid of the limits of their knowledge.  I am talking about your understanding of a fact that everyone else seems to miss, a fact that many doctors hide from: we are normal, fallible people who happen to doctor for a job.  We are not special.  In fact, many of us are very insecure, wanting to feel the affirmation of people who get better, hearing the praise of those we help.  We want to cure disease, to save lives, to be the helping hand, the right person in the right place at the right time.


But chronic unsolvable disease stands square in our way.  You don’t get better, and it makes many of us frustrated, and it makes some of us mad at you.  We don’t want to face things we can’t fix because it shows our limits.  We want the miraculous, and you deny us that chance.


And since this is the perspective you have when you see doctors, your view of them is quite different.  You see us getting frustrated.  You see us when we feel like giving up.  When we take care of you, we have to leave behind the illusion of control, of power over disease.  We get angry, feel insecure, and want to move on to a patient who we can fix, save, or impress.  You are the rock that proves how easily the ship can be sunk.  So your view of doctors is quite different.


Then there is the fact that you also possess something that is usually our domain: knowledge.  You know more about your disease than many of us do – most of us do.  Your MS, rheumatoid arthritis, end-stage kidney disease, Cushing’s disease, bipolar disorder, chronic pain disorder, brittle diabetes, or disabling psychiatric disorder – your defining pain -  is something most of us don’t regularly encounter.  It’s something most of us try to avoid.  So you possess deep understanding of something that many doctors don’t possess.  Even doctors who specialize in your disorder don’t share the kind of knowledge you can only get through living with a disease.  It’s like a parent’s knowledge of their child versus that of a pediatrician.  They may have breadth of knowledge, but you have depth of knowledge that no doctor can possess.


So when you approach a doctor – especially one you’ve never met before – you come with a knowledge of your disease that they don’t have, and a knowledge of the doctor’s limitations that few other patients have.  You see why you scare doctors?  It’s not your fault that you do, but ignoring this fact will limit the help you can only get from them.  I know this because, just like you know your disease better than any doctor, I know what being a doctor feels like more than any patient could ever understand.  You encounter doctors intermittently (more than you wish, perhaps); I live as a doctor continuously.


So let me be so bold as to give you advice on dealing with doctors.  There are some things you can do to make things easier, and others that can sabotage any hope of a good relationship:

1. Don’t come on too strong – yes, you have to advocate for yourself, but remember that doctors are used to being in control.  All of the other patients come into the room with immediate respect, but your understanding has torn down the doctor-god illusion.  That’s a good thing in the long-run, but few doctors want to be greeted with that reality from the start.  Your goal with any doctor is to build a partnership of trust that goes both ways, and coming on too strong at the start can hurt your chances of ever having that.
2. Show respect – I say this one carefully, because there are certainly some doctors who don’t treat patients with respect – especially ones like you with chronic disease.  These doctors should be avoided.  But most of us are not like that; we really want to help people and try to treat them well.  But we have worked very hard to earn our position; it was not bestowed by fiat or family tree.  Just as you want to be listened to, so do we.
3. Keep your eggs in only a few baskets – find a good primary care doctor and a couple of specialists you trust.  Don’t expect a new doctor to figure things out quickly.  It takes me years of repeated visits to really understand many of my chronic disease patients.  The best care happens when a doctor understands the patient and the patient understands the doctor.  This can only happen over time.  Heck, I struggle even seeing the chronically sick patients for other doctors in my practice.  There is something very powerful in having understanding built over time.
4. Use the ER only when absolutely needed – Emergency room physicians will always struggle with you.  Just expect that.  Their job is to decide if you need to be hospitalized, if you need emergency treatment, or if you can go home.  They might not fix your pain, and certainly won’t try to fully understand you.  That’s not their job.  They went into their specialty to fix problems quickly and move on, not manage chronic disease.  The same goes for any doctor you see for a short time: they will try to get done with you as quickly as possible.
5. Don’t avoid doctors – one of the most frustrating things for me is when a complicated patient comes in after a long absence with a huge list of problems they want me to address.  I can’t work that way, and I don’t think many doctors can.  Each visit should address only a few problems at a time, otherwise things get confused and more mistakes are made.  It’s OK to keep a list of your own problems so things don’t get left out – I actually like getting those lists, as long as people don’t expect me to handle all of the problems.  It helps me to prioritize with them.
6. Don’t put up with the jerks – unless you have no choice (in the ER, for example), you should keep looking until you find the right doctor(s) for you.  Some docs are not cut out for chronic disease, while some of us like the long-term relationship.  Don’t feel you have to put up with docs who don’t listen or minimize your problems.  At the minimum, you should be able to find a doctor who doesn’t totally suck.
7. Forgive us – Sometimes I forget about important things in my patients’ lives.  Sometimes I don’t know you’ve had surgery or that your sister comes to see me as well.  Sometimes I avoid people because I don’t want to admit my limitations.  Be patient with me – I usually know when I’ve messed up, and if you know me well I don’t mind being reminded.  Well, maybe I mind it a little.

You know better than anyone that we docs are just people – with all the stupidity, inconsistency, and fallibility that goes with that – who happen to doctor for a living.  I hope this helps, and I really hope you get the help you need.  It does suck that you have your problem; I just hope this perhaps decreases that suckishness a little bit. Sincerely,
Dr. Rob 
Post Script: This post has generated a huge amount of conversation and interest (as witnessed by the large number of comments!).  I very much appreciate the dialogue it has spawned both here and across the web.  I’ve subsequently written follow-up posts explaining my thoughts in more detail – largely in response to the comments here.  One of them discusses in more detail my own experiences as a doctor and the second talks of the importance of  knowing and being known.  Reading these will give you a better picture of my thought process and perspective on this.
Dr. Rob

Drug Addict?

Here is something for you to ponder....Are we Cystics drug addicts?

Think about it.  

We can not function without our drugs.  We scrounge around the house for some extra cash so we can get our "fix".  We go through "withdraw" if we skip doses.  Without our meds, we could, and most likely will, die.  We have no choice and must take the drugs.

A drug addict needs their drug of choice to function.  They will do whatever they can do get money for the next fix.  They go through withdraw if they stop taking their drug of choice.  Withdraw can be so severe it leads to death.  They have become victims to the high and addiction.  

The only differences really between us and them is that we have no choice in the matter and can not stop without severe consequences, while they took the first hit and were hooked, and can, with help, beat the addiction and get clean.  

Something to think about huh?

***disclaimer***

written as fun and was not meant to portray actual drug addictions as inconsequential.

Am I only Dreaming.....

No this isn't a Bangles song. Its just me blogging about CF. Which, in a way, could be considered an eternal flame. However, that's not the point of this blog today.

Today I'm talking about the feeling of being in a dream at times. When you are having an "out of body experience" and feel like its not you with the CF. Confused yet? Stayed tuned, it only gets better, or worse, or more confusing depending on...well...me.

As I was being wheeled to the xray lab yesterday, a familiar thought passed through my mind. Is this really me? Am I really the one in a wheelchair, blanket over my lap, PICC in one arm, IV in the other and a mask over my face? Or am I imagining it all? Is this all just a dream, or a chapter in a book? Does my imagination really work THAT well?

Of course we all know the answers to those questions. YES this is me. And while I might have a great imagination, I don't think it is THAT great.

How do I come to these strange feelings you may ask? Well I am a huge reader. I always have a book I am reading and sometimes even two if I am slowly making my way through one I'm unsure of. Getting lost in the chapters, pages and words is one of my favorite past times. I have fallen in love with characters (no really, I fell in love with a man in a book I read once and even cried when the book was over because I didn't want to leave him - consider me crazy yet?), hated characters to the core, and felt pity on others. To me, reading is a way to get away from reality, and it works. So its safe to say we can see why I feel like I am in a book, reading about someone else's life, not my own.

Later on, as I lie there on my hospital bed, being beaten up by the respitory therapist du jour, the feelings come back. Its all so familiar, yet so distant. I know I've been there before, yet I don't know why. Unsure of how I got there, I succumb to the poundings and let the feelings of dreaming take over me.

Perhaps one day I'll escape dream land and find myself in reality, where I belong, and without CF. Or I might just stay in this land where my friends reside and I can think of escaping to "reality".

Stubborn to a "T"

We as CFers tend to be a stubborn and independent bunch.  I am no exception.

This week has been a lot of fun for me!  I have been decorating my boyfriend's daughter's room for her for her 8th birthday (which is Friday) while she is on vacation with her Nana in Florida.  She adores pink and princesses so I of course did just that.  Her walls are pink and one is chocolate brown.  Then her white bunk beds and new white bookcases look fabulous on the brown wall.  I got new bedding, new curtains and re-arranged the whole room.  Now it looks like a little princess' room.  I can't wait until Tuesday when she gets home so she can see it!

Of course with all of this work comes th CF side of life.  My chest hurts, my back throbs and I haven't been able to catch my breath since Monday.  OK that last one is a bit of an exaggeration but you know what I mean.  I huff and wheeze and struggle all day to breathe, yet I refuse to stop.  It's hard not being able to move your own things around so a part of me insisted that I do this all on my own, no matter the cost.  And I felt like I needed to make up for my inability to move my belongings.

This past weekend I moved the rest of my stuff up from my mom's house in PA.  I was lucky enough to have my friend Tina come down with me to help.  It was nice having someone else in the truck for the ride up this time.  Usually it is just me and it does get boring.  And I would have been really bored since the cig lighters didn't work so I couldn't use my iPod.  Anyway, packing the truck just was not in the stars for me.  I was so SOB after moving a few books, light ones too, that I had to stop and watch.  It SUCKS.  Being on the sidelines for things like that really get to me.  I am supposed to be able to do it all on my own and when I can't I feel incompetent.  Each move it gets worse too.  Last time it was only the book boxes and really heavy stuff I had to avoid  This time it was pretty much everything.  I did unload some of the boxes into the house on my own and then Peter helped me take the rest to the basement.  I carried them to the top of the steps and he took them down.  But even the 20 feet from the truck to the stairs wore me out super fast.  I was leaning on the door in no time.  It depresses me.

I know this isn't the first time I have written about this and I know it won't be the last either.  I just wish it would all go away so I could do the heavy manual lifting and moving I am used to doing.  Depending on someone else to do it is not my cup of tea.  But I insist on pushing myself until I absolutely can not breathe or until I am forced, by the people helping, to stop and relax.

Stubborn?  You know it...and I will be till the day I die...in like 30 years :)