One More Week

One more week to go on Ceftaz...yaaaaaay *sarcasm* I can deaccess on Monday.   I'll be down there anyway for a derm appointment so I am going to go in after to do PFTs.

My numbers are up some 1.05L, up from .85L.  33, almost 34%.  Still not up to my usual 1.11-1.13L but its an improvement.  I don't expect much more since its only Ceftaz I'm on and 2 orals.  Which really sucks because next infection will drop me low again.

She wants me to get a hearing test next time I come in since I haven't ever had one.  Glad.  I don't think my hearing is horrible but I know certain pitches I can't hear.  Whether its age or TOBRA related who knows.

That's all for now.

Home from the Hospital

I am home from the hospital.  I actually got out on Monday but I have been so busy finishing up my paper, that I turned in Wednesday, and appointments, that I haven't been able to update.

I was admitted last Wednesday night and for some reason on Thursday afternoon I spiked a fever.  102.3 at its highest.  No flu, no blood infection.  Nothing out of the ordinary except that fever.  Tylenol brought it down and by Friday night I was back to normal.

The doctors started me on Q36 for the Tobra this time since we always end up at that point anyway.  Seemed to work well.  Then I came home.  I had 3 doses total when the nurse drew my labs Wednesday morning and I was told yesterday to stop taking it.  Kidneys are not happy.  WTF!?  So now I am on oral Bactrim, oral Cipro and IV Ceftaz, aka. cat piss.  Hoping my FEV1 came up a bit so that I can stop the IV Ceftaz before Christmas.  I have clinic on Tuesday.  My body never responds to orals.  This is just wonderful.

Yesterday, Thursday, I had my follow up appointment to my colonoscopy.  It was a little disconcerting.  My colon polyp was adenoma as usual.  That wasn't concerning.  But, like I mentioned before they were finally able to biopsy that pesky cyst on my pancreas.  Turns out it isn't CF related at all.  Its a precancerous cyst related to my Lynch Syndrome.  Like the adenoma in my colon, if left untreated it has the potential to turn to a cancerous tumor.  "Biopsy of your pancreatic cyst revealed benign cells and CEA level 825 with amylase less than 3.  This may be consistent with a mucincous type of precancerous pancreatic cancer."  Fabulous.  We didn't' discuss removing the cyst.  Honestly I was a bit in shock that it WAS something that I forgot to ask about surgery.  She wants to monitor it every 6 months with MRI/MRCP's again, and if it grows, I can talk to a pancreatic surgeon then.  I am going to email her about just having it removed.  I don't want that shit growing.  On the bright side, I don't need mammograms just yet...

That is all for me.  School is over and I am doing lots of crocheting and sleeping.  I will post an update next week after my clinic appointment.

It's That Time Again

Yup it is time for IVs!!!  This way I will feel fabulous (or as fabulous as someone with my lung function can lol) for Christmas and visiting my family.

I blew some really shitty numbers today.  FEV1 of 28%, .85L ha!  Last time, Sept 17, I blew 1.11L, 36%!!!  Holy drop batman!!!  I haven't seen numbers like that since I did the Vertex study in Jan 2011.  I was glad to see them so low because then she wouldn't suggest Cipro and Prednisone first.  She did get an Xray done to make sure I didn't have a collapsed lung or anything like that.  And I don't think I do or I would have heard back by now. 

BCH and BWH made some changes and all CF patients up to age 35 have to be admitted to BCH unless they are listed for transplant or already transplanted.  Luckily for me, I am technically listed for transplant even though I am inactive on the list.  Seems weird to say that.  But that means I can stay at BWH and not get used to another new hospital.  Yayyyyyyy!!!  

So it looks like tomorrow night I will be admitted and then I can be home on Monday, just in time to finish up my grad class.  Lots of editing of my paper and crocheting will get done!  Plus I starting getting a new magazine so I have 2 of them to bring with me.  I won't be bored that is for sure!

Time again for IVs I think

I had to bump my clinic appointment up to this Tuesday from December 17th.  Its only a 2 week bump but with Thanksgiving there was no way I could get in any earlier.

Anyway, my lungs have been really horrible lately.  I am super SOB, super tight, and in a good amount of pain 24/7.  Since my doctor took me off of maintenance Motrin a few months back I deal with minor pain constantly.  But nothing I can't handle.  However, the last few weeks I have been popping it like candy again.  I try really hard to avoid taking it but the pain gets so bad and I get so uncomfortable I have to take it.

I am also back up to 4 treatments a day and sometimes 5.  I barely make it 4 hours before I am sucking back the meds.  Sometimes, particularly at night, I don't even wait that long.  I just can't breathe at all.  I could barely make it up the flight of stairs, going super slow, at the library today and that was only 1.5 hours after my morning treatment, when I am supposed to be in my "best shape."

And the junkiness.  Can't forget the increase in the wonderful mucus.  Not much, but for me it is.

I really hope she just goes straight to IVs and does not want to try Cipro and prednisone first.  I go to PA right after Christmas and I don't want to feel like shit when I am there.  I always do and now I have a chance to be in better shape BEFORE I go down.  I don't know how it will all work anyway since we are no longer inpatient at Brigham and Women's but instead admitted to Children's.  This should be fun... I'm also worried she will want to start IVs but only at home and not admit me since we are in Flu season.  I like starting my course inpatient.  Its nice to have those few days to relax and not do household work, or cook.

The good news is if I do go inpatient I can finish up my final paper and some crochet projects that I have.  My last day of class is December 11th and then I am off for almost 6 whole weeks.  Being inpatient will give me some time to finishing editing my 38 page paper (ha its supposed to be 20ish opps).  Seriously I am really looking forward to a few days to rest.  I don't do that at home, that's for sure.

My appointment is on Tuesday and if I am lucky I will go in on Wednesday or Thursday.  This way too, if I have to do 3 weeks I will be done by Christmas, just ha!

Nothing out of the ordinary

I realize its been almost 2 months and I have no excuse, only an apology.  Just really busy.  I guess that is a good thing right?  Lots of things coming up this month though.  I have an ENT appointment on the 15th and my annual colonoscopy/endoscopy on the 22nd.  I will keep you all posted on the results of those.

Other than that...just a bit more junky than normal.  Coughing a good amount and actually bringing junk up.  I don't see my doc until December 17th so crossing my fingers I last that long.  I have that sample of the TOBI podhaler sitting in my drawer but I am not really excited to try it.  We shall see...

Fabulouso

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

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

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

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

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

I could not have been happier with today.

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.

Nothing Here

I realized today its been over a month since I last blogged.  Sorry.  Not much to report which is a good thing I guess!

I have a clinic appointment in a few weeks so after that I can update.

Classes start back on September 4th and I will be in the last stretch of my Master's.

Other than that...nadda

Hope all is well.

They Say the Numbers Don't Mean Anything

Well Tuesday I was de-accessed at clinic.  So glad.  I was totally over IVs.  Unfortunately instead of my lungs improving with another week they went down.

I was a little annoyed at first though.  When doing them, the RT had the screen faced away from me so I couldn't really see the results.  I usually see them.  No biggie.  She was a new one for me.  So after the first blow she says "oh good you went up from last time."  So I am thinking sweet maybe I hit 40%!!!  Second one she says "even better!"  So I am pumped to get the print out.

I don't know where she was looking.  My guess is FVC/FEV1 instead of FEV1.  That is the only one where I am slightly better than last week.  My FEV1 was 1.03L at best.  Remember last week I was 1.10L.  And before the hospital I was 1.13L (.95L got me admitted).  WTF?  I was not going with another week of IVs though.  I feel fine.  I wouldn't have guessed my numbers were down.

I also lost 3lbs from the week before.  I was shocked.  My home scale stayed the same.  To be honest I am not worried.  I don't eat as much during the summer and with the exercise, I am bound to shed a few pounds.  If I lose too much more than I will start to worry.  And once fall hits again I will pack the pounds on again I am sure.

She also gave me a one week trial of the TOBI Podhaler.  I am supposed to wait 2 weeks then give it a go.  I am nervous though considering the reactions I get from any inhaled antibiotics.  I don't want to be spazzy for a week.  Plus I am back to feeling great at the gym and I don't want to ruin that!  So I don't know if I will try it or not...

I also found out I am culturing Steno Malt again.  Bleh.  So I started a 2 week course of Bactrim last week.  Could be why my numbers are still down, but I was culturing it when I was in patient too.  So it doesn't explain why they went down from last week, only that they are down overall...maybe.

Feeling Better...Almost Normal

Well let's see what has been going on the past two weeks?  I was admitted on the 28th, Friday.  Originally I was supposed to go in Thursday, then it was pushed to Monday then it was Friday.  Luckily they did Friday because there was no way I would have been out on time for the 4th festivities had I gone in on Monday, the 1st.  My TOBRA levels were high after just two doses so they stopped it for one day and restarted me at a lower dose and every 36 hours.  I am doing that schedule now at home too.  I am also on Zoysn every 8 hours.  My normal IVs schedule.

I had clinic follow up today.  My numbers are back up to 1.10L, where May and June I was 1.13L and 1.11L respectively.  So I am pretty much back to base line.  We are doing one more week though to see if I can get anymore from these old blowers.

I also got a new toy!!!  The NP gave me a spacer and an Rx for a duoneb inhaler.  So now when I am out and need a treatment I don't have to fret, or breathe like shit.  I can keep it in my purse and use it when I need it in place of a nebulized treatment.  This will be great for amusement parks and the beach!!!

As far as feeling better, I am getting there.  My O2 is almost normal with exercise, but my tolerance is way down since its been almost 8 weeks since I had to slow my pace.  So I need to build that back up some.  I am coughing up a storm, all dry inflammed coughs.  No fun.  I miss my mucusy cough from when I was a kid...

Till next week.....

Hospital Time!!!

I was SO glad to see my numbers were horrible today!!!  It confirmed what I KNEW and also made it so I didn't have to beg and plead for a round of IVs!

Last time I was 1.11L 38% this time my highest was .95L 33%.  But my other 2 were .90L and .89L so that .95L was way up there!!!  Since I have started using the correct predicted values on myself, I am really at 30% with my highest and 28% with the lowest.  (The NHANES is the one used by most CF centers and I believe is the accepted one for the CFF).  Anyhow, we are looking at Thursday afternoon/evening to go in...hopefully.  The coordinator was out today so she will have to set it all up tomorrow.  If I can't go in Thursday then it is Monday and that I will not be happy with.  Thursday next week is the 4th of July and I don't want to miss the fireworks and parade!

The Fevers That Just Won't Quit

Wow 9 days?!?!  Sorry folks.  Been hectic around here.

My lungs have not been cooperating with me at all.  Nothing ER visit worthy but definitely looking forward to Tuesdays clinic visit.  If she doesn't think I need to be admitted I will have a HISSY FIT and a half.

My O2 with exercise is absolute shit.  Normally I walk at 3.5 with bursts at 3.8.  Right now I am GASPING at 2.7.  And my O2 is hovering at 90%.  Heart rate has been as high as 171.  Wednesday I started with fevers.  Tonight again, its 101.  Hasn't been that high since the flu of April.  Hoping I am pushing myself too hard and my body is just tired and fighting something.  If I hit 102, no worries I will haul my butt to the ER I promise.  I finished my prednisone and Cipro on Tuesday.  No difference, and really, I think I am slightly worse.  Fevers say that at least.

Thursday I still did my personal training session, but we modified it a lot.  Some days I do walking lunges and other exercises that require me to walk while holding weights.  On a good day they make me SOB, so I requested we cut all walking exercises out.  I tried one squatting exercise and made it through one set before I said no more on that one.  I couldn't do it.  She even commented that I was breathing MUCH heavier than normal.  And I only completed about 2/3 of what I normally do.  Still not too bad all things considering though.  Of course, I watch other people with their trainers and I see the amount of things they do and it just exhausts me ha!  They complete at least 1.5 if not 2 times the amount of exercises that I do.  Granted I am functioning at less than 40% lung function so I need to pat myself on my back for that.  And I am seeing results physically so that is excellent.

Thursday I also had my follow up with the GI docs.  My MRI looked good, nothing concerning and no need to see the Pancreas surgeon yet.  I am to schedule my colonoscopy, endoscopy, and endoscopic ultrasound for mid-November.  Then schedule a follow up with her in December to go over the results.  Once we get those tests again, we will have come full circle in a year and we can go from there.  If everything looks good then we can just monitor the pesky cysts.

Anyway, I wanted to give a small update.  I will be sure to post after Tuesday's appointment.

Amazing Difference in 3 Months from Exercise!!!

This is the story of a Cyster's desire to improve her body and her health.  This is a story with a happy plot line.  This story is not over yet.

Three months ago I joined a new gym.  I had been at my old one for a while but it was boring, I didn't like the atmosphere and I felt like a puny girl.  Then the end of February I found a flyer in the mail-room of our condo.  It was for a women's only gym down the street.  Closer than the other gym!!!  I decided to check it out.  It was a bit more pricey but it had a good vibe and they offered classes for teens which was perfect for my step-daughter.  I was hooked!  I signed up that day and have not looked back since.

With the gym membership (and an annual fee) you can get a fitness consult to see where you are body wise. It was $90 and you get 4 appointments.  I couldn't say no.  March 13, 2013 I had my first consult.  I was nervous.  I felt fat and gross and so out of shape.  I say I FELT that way because while I was out of shape, I was far from fat.  

Here are some stats from that consult:

Weight: 126.2 lbs

BMI: 22.4

Body Fat: 30.8%

Fat Mass: 38.9 lbs

Cardio Fitness: 28.9 (needs improvement - NI)

Grip Strength: 51.6 (Fair)

Push ups (on your knees): 7 (NI)

Sit and Reach: 13.2 (NI)

Plank: 11 (Good)

Vertical Jump: 7.7 (NI)

Fast forward to today.  It has been 3 months, and only 6 personal training sessions.  I exercise 5-6 days a week.  I do cardio for 4 days, strength training one day a week and then I also do Tone It Up.  I am eating better, I am working out, and I am seeing results (and I realize I sound like a dam infomercial!).  

Here are today's stats:

Weight: 121.2 lbs

BMI: 21.5

Body Fat: 28.4% (28% is normal)

Fat Mass: 34.4 lbs

Cardio Fitness: 37.8 (Very Good)

Grip Strength: 52.9 (Fair)

Push ups (on your knees): 25 (Very Good)

Sit and Reach: 22.86 (NI)

Plank: 11 (Good)

Vertical Jump: 12.7 (NI)

Now for the kicker:

I lost 14.25 inches from my body!!!

3/4" from my neck

1.5" from my shoulders

1.5" from my chest

2.5" from my waist

2.5" from my hips

2.0" from each of my thighs

I lost 4.5 lbs of body fat and decreased my Body Fat% by 2.4%.  

My biggest surprise is not the 14" that I lost, but the improvement in my cardio functions!  Granted, today I could not finish the treadmill test (they increase the incline and I insisted that once I hit 90% O2 we stop since my lungs are not fully cooperating), whereas last time I forced myself to finish no matter what.  But my recovery time for my HR was much better this time around.  

I knew I lost weight, I knew I lost inches.  But I had no idea the results were this fabulous.  My trainer asked if I wanted to be on the testimonial board and I said yes!!!  After 6 sessions to see such an improvement, it makes me so happy.  I realize now that the hard work IS paying off.  I may not get back to work, but I know I will be healthier, and if I keep this up, when I get a transplant, hopefully my recovery and chances of survival will increase with the added health on the rest of my body.  

I love this!!!

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...

Drama Queeeeeeen!!!

I feel like a dram queen every night.  I know, me?!?!

The Prednisone has been great giving me energy and keeping the zzz's away, but it hasn't been great opening up my tight lungs.

Last night I started having a small, tolerable, panic attack because I was about to do my FIFTH breathing treatment for the day, only 2 hours after the last one.  I NEVER do that!!!  But I was about to go to bed and  my lungs were so sore and hurt so much, and were so tight, I wanted to try and open them.  Didn't work.  As I climbed into bed next to P, complaining about the pain and the uncomfortableness, I felt like some drama queen looking for attention...

We got home from our cabining weekend away around noon yesterday.  I immediately went into Prednisone induced overdrive cleaning and putting things away.  The laundry room shelves got re-arranged.  The TV stand in the bedroom got cleaned and sorted.  The floors were vacuumed.  The fridge was pulled out and I scrubbed behind it as well as the whole outside of it.  5 loads of laundry were done (4 sorted and put away).  And we took Major to the park to play for a bit.  All of that - besides the laundry - were done by 6pm.

I know I overdid it.  But we relaxed on Saturday and my lungs were having a hissy fit then too.  I am going to TRY to take it easy today, exercise, crochet, read for classes, and see if my lungs don't want to jump out of my body by 10pm again.

I also wish CF doctors could feel this pain and understand that yes Motrin on a daily basis in the dose I was taking is not fabulous for my kidneys/liver whatever, but fuck man, MY LUNGS HURT.

I was able to get an appointment for June 25th to follow up with the regiment I am on.  She said 3 weeks when I left but the scheduling was all screwy so I said I would call end of this week to schedule after July 1.  Decided to make it exactly 3 weeks (which ironicly the appointment I made is the same one I cancelled to go in last week to see her), so that if this does not help, I can get in to the hospital and start IVs before my 2nd summer class starts July 9.

I Wanna Bulk UP!

No not really!!!  But let's hope my plan of action doesn't make me.

Clinic was a bit disappointing.  As I posted last time I feel like ass, like complete and total shit.  So what happened at clinic?

Nothing...ok stuff happened but I am eh about it all.

FEV1 the same.  I went from 1.13L to 1.11L, 39%-38%.  Nothing worrisome there.  But no one seems to care that when I have an appointment at 9:30am my numbers are going to be waaaay better than when it is at 11am (this was a moved appointment so I had to make it that early if I wanted to go before June 26th).  2 hours post-treatment is great for me in the mornings.  4 hours post-treatment not so much.  Get me in the afternoon and that is my PERFECT time because that is pretty much how I feel from about noon till I go to bed around mid-night.  9am is NOT my normal lung feeling, nor capacity.  But that doesn't matter in the medical world apparently...Anyway.

When I explained my symptoms I felt like my doc was thinking I was lying.  Like I was looking for IVs for fun.  Yeah fun.  Let me tell you how much fun diarrhea, nausea and pure exhaustion are.  But they are worth it when you feel great after.

So instead what did I get?

An x-ray to make sure nothing was wrong in there.  Only some extra smudge on the lower lobes.
2 weeks of 750mgs of Cipro twice a day.
1 week of 20mgs prednisone twice a day
1 week of 20mgs prednisone once a day
30 days of 30mgs of Prevacid twice a day (been having a lot of extra heartburn lately)

Hoping the prednisone opens me up.  If it doesn't I am going to be so mad.  I can't fit a hospitalization in until beginning of September without missing classes and  I don't want to do that.

Oh well.  Camping this weekend and maybe the steroids will make it easier for me to bike ride, go on a scavenger hunt, and swim....

Vorrei fare un viaggio a Italia

Just a quick update on me since I don't have any more CF related questions to answer.  May is about over as well and I hope we were able to spread awareness to the world.

My energy level and health have been going south for the past couple of weeks.  Well really since the flu.  But the past week or so its been very noticeable.  I am up to 4 treatments a day (vs 3 normally) and I would do 5 if there was enough time in the day.  Today at the gym I had to lower my walking speed because my O2 did not want to go above 90%.  Craptastic.  I have clinic next Wednesday so it will probably be IV time.  

I started classes again last week.  Loving it so far.  Its on the Renaissance!!!  Takes me back to my trip to Florence and Rome and really makes me want to go back!  Someday...It is also making me want to learn Italian.  Again, someday...

In sad news, a friend of mine passed away yesterday from CF complications.  She had been in the hospital for weeks and I was following the updates though I was never fully sure of what was going on.  But I saw the news this morning that she is gone and breathing easy now.  Sad....Please say prayers, send good vibes etc to her family, her husband, her daughter that they may find peace at this time.

CF Awareness Post 3 - Differences in Treatments

For this one I asked a few of my non-American CF friends to help me.  Since the question deals with treatments in other countries, I could not answer.  Thank you to Julie , Alice  and Tracy for helping!!!
A little background on my helpers.

Julie is from Canada.  She still has her original lungs.  
Alice is 5+ years post transplant living in South Africa.  
Tracy is 5+ years post transplant and living in Australia.

Question: Hey, Amy! I have a CF question... how does CF treatment vary between countries? Or, maybe another perspective would be, how does America differ from other countries in its CF treatment? I know each individual case is unique, but it seems like if I've read a blog based in Canada vs the UK vs America that each demonstrate a different approach to their treatments. Some examples might be: when a person is listed for a transplant, or how aggressively something is treated, or what conclusions are drawn from the same data… is the nation’s healthcare the #1 factor? Is it more dependent on the medical team you’re with? Or is there a general impression that depending on where you live, you can expect a certain response? I’ve read a few blogs where the individual may have gone on ECMO, for example, and it seemed like that was only an option for a few days, while others may remain on ECMO for many weeks and that is okay… though I know there’s no one-size-fits-all approach. So this rambling post is to ask how much does WHERE you are have to do with HOW you’re treated?

Answer:

Tracy: Australia does not have the vest.  

Alice: CF treatment in SA differs quite a bit depending on weather you live in a big city or not and if you've got health insurance or not. Where I grew up (smaller city) treatment depended on what I wanted, if I felt I needed IV's they gave me IV's. If I said I wanted to try oral antibiotics first then they let me. Here in Johannesburg, I live now post transplant, they treat the CF patients WAY more aggressively. Not sure about the ECMO, but I doubt they'll transplant someone on ECMO (but that might also be because they do only about 5-7 lung transplants a year so the odds of someone surviving long enough on ECMO is slim).  

Julie:  Canada has universal healthcare and I have private through my disability plan. CF patients in Ontario have a provincial drug plan that covers almost everything, and what it doesn't cover like Pulmozyme (waiting for the patent to run out on it) we have section 8's that cover the cost. I don't pay anything for any of my drugs, so everything, including Boost, insulin, enzymes everything is covered.

CF exacerbations are treated depending on how I feel.  If I want to try orals we do and if I want to skip than we skip and do IVs, or if I feel I can get over it on my own we do nothing. It depends on how much of a drop I get in FEV1 if I'm just down around 35% from 39% we'll try orals, if I'm down anything below 35% we usually do IVs. And those if we can help it are usually done outpatient because they like to keep us out the best they can. We have isolation rooms but we all know there are so many bugs that can be picked up in the hospital. So as long as we're not terribly sick we can opt for outpatient PICC line and home care. But I have a friend who refuses PICC lines, so she has to go in patient for the full course of IVs.  I find each CF person is treated on a person to person basis, some people are treated more aggressively while others may not be, but they still get good healthcare. If that makes sense. I mean if you want to be treated aggressively they will, if you don't then they trust you know your body. 

We have a CF gentleman in the ICU right now waiting for his second set of lungs and he is on ECMO and has been for a long time, we use it as a bridge to transplant... where you know the situation with Rhi, at her transplant hospital being on ECMO makes you untransplantable while on it. That is more probably varying from center to center, just like some transplant hospitals want you 3 years post cancer and some want 5. 

We have all the same drugs you do in the US as far as nebs. We have access to the Vest but it isn't covered under our healthcare plan nor is it usually covered through most private insurance because it's coming from another country... from what I've heard anyways.  And there are people in Canada with the VEST, I know quite a few but they raised the money or was donated it to them. As for myself, since there's not really any proof that the VEST is that superior to manual CPT I stick with my flutter and hubby beats me when I ask him to. Other people still have percussors, mine crapped out and died years ago and I gave up on them because they're always dying.

Exercise is a big part, we see a physio therapist with every clinic visit and clinic visits around here are mandatory every 3 months for everyone no matter their lung function.  So is nutrition, we see a dietitian every 3 months as well.

My clinic has strict infection control. We have isolation rooms for in patient and if you have certain bugs you are not allowed to leave your room. At clinic they don't let us stay in the waiting room very long, we're shuttled of to a private room to try and keep us all separate. Cystic Fibrosis Canada is trying to adapt the American CF control policy and many people (mainly adults) aren't happy with it.

Hyperion - a CF Film

I was contacted by the writer to get the word out about this film he is trying to get produced.  If you can take a minute to check out the website and watch the short clip.  Its worth it.  Its less than 3 minutes long so won't  tie you up all day.

HYPERION

The writer and director was inspired by Eva Markvoort.  You remember Eva and her 65 Red Roses Documentary.

The director is also working with Emily from Rock CF and the CFF to make sure facts are correct.

Looks like a good film and I look forward to it coming out.

Everything is Vanilla Right Now

Last Tuesday I had my clinic appointment.  I also met with the respiratory therapist to go over my vest settings and airway clearance tactics.  It is a new thing they are doing to try to get people more aware of how to use their devices and improve their health.  Nothing really changed with my settings.  She mentioned I should schedule in breaks between frequency intervals so I can do some huff coughing.  Its hard when I am not all gooey to get stuff up.  I told her I do the vest not because I feel better when I do it, but because if my lungs still crap out I can say well I am doing everything.  Its the truth.  And at least I am doing it!  Faithfully!  Seriously its been like 3 months now and I have missed a few days to being sick and that is it!  Considering it had been months before this is like wicked amazing.

Clinic was clinic.  Nothing changed.  I am more run down and feeling crappy but I also had the flu and she told me to finish the 2 weeks of orals I was given and see how I feel.  I go back in June and if I am crappy still I will see about IVs.  This is the longest stint without IVs since I moved up here.  I attribute it to my exercise regime.  Whether or not that is true I don't know, but I feel pretty good considering its been almost 5 months since my last course.  My lung function was the same 38%.  Weight was down 3lbs thanks to the flu.  O2 normal, HR normal, BP normal.  Booooring lol.  Nothing at all exciting, and quite frankly I will take it.

I am a little disappointed though.  My clinic is participating in the next Vertex trial and I was asked to do it, if I wanted to.  Unfortunately you have to have an FEV1 of 40% or higher, and even with a treatment I don't hit that mark.  So I am still out.  Kind of sad.  I enjoy the studies.  Maybe more exercise will bump me up more and I can do another one....this makes two in two months I was shot down.  le sigh...

CF Awareness Post 2 - What is CF?

My second question comes from a fellow CFer as well.  Her question was meant more as a joke but I decided to take it and answer it!

What is CF?

CF means Cystic Fibrosis.  CF is much easier to say and write especially in long conversations and posts.

But what IS CF?

CF is a genetic disease that affects many aspects of the body.  It varies from person to person but it affects mainly the lungs, the pancreas, the digestive system, and the reproductive organs in both sexes.  Some patients have issues with their kidneys, liver, joints, bone density, and heart.  These are secondary issues caused by medications and low oxygen respectively.

The low down dirty reason for CF is the inability of cells to transfer sodium chloride across its membrane.  The result is an abundance of salt in the body.  We CFers tend to be a very salty bunch.  If you look at my legs on a hot summer day they are dry as a bone but covered in a white film....lick me and drink a Margarita!  

This leads to a build up of thick sticky mucus in the lungs which harbor bacteria and cause lung infections.  These lung infections, over time cause a decrease in lung function because of scarring.  Eventually the lungs don't function properly and can not get enough oxygen to the rest of the body, requiring supplemental O2 to be worn.  The decrease in lung function ultimately causes a CFer to either pass away or require a double lung transplant.  These lung infections can be treated with oral antibiotics, IV antibiotics, and inhaled antibiotics.  As well as the use of the airway clearance device the Vest, accapella, the flutter and manual chest physiotherapy (CPT).  Bronchodialators (sp?) are also used to keep the airways open so the medicines can get in there and do their work.  As the diease progresses though, orals and inhaled meds may not work.  As is the case with me.  Orals work sometimes, and inhaled antibiotics are out of the question because I have reactive airways.  Meaning when I inhale something my lungs don't like, they close up and cause severe bronchospasms.  NOT FUN.  Basically I have a massive asthma attack that only Prednisone can get rid of.

In a good portion of CFers, but not all, the pancreas does not work properly.  The pancreas produces enzymes which break down food put in the body to get nutrients.  In some CF patients this does not happen so we must take enzymes WITH EVERYTHING.  If we want the nutrition from the food we must take the pills.  There are a few different enzymes on the market and it takes time to figure out which works for you.  Me personally, I don't take pills with every meal because I have a tendency to get backed up fast.  I take them with my big meals but my 2 small meals a day I skip.  I also have to take a ducolax and Miralax every day.  If not, I risk getting a blockage from my slow moving digestive system.  We learn that the digestive system works in about 6 hours or so.  For me, and many of my CF friends, we might not "see" that meal on Monday until Wednesday.  Bloating and the CF belly are quite common with CF patients.

CFRD is also caused by a reduction in the function of the pancreas.  CF related diabetes.  Kind of like regular diabetes, but not quite.  Sugars need to be monitored, insulin may have to be taken.  I am new to the whole CFRD thing and only monitor with diet so I am not as up to speed on this aspect as I am the rest.  Many CFers don't reach CFRD until after transplant because of the use of Prednisone to reduce rejection, but many do get it prior.

The reproductive organs are also affected.  For many CF men the vas deferens is missing so sperm is not able to make it out when ejaculated.  I do have a few CF male friends who have fathered children the natural way.  But this is rare.  Most need to see a fertility specialist to have the sperm taken out and artificially inseminate the woman.  For women, getting pregnant can be easier than the man getting a woman pregnant.  Though it varies from person to person.  Some women just never get pregnant without help and others find they have "opps'" easily.  There is a extra amount of mucus lining the cervix which can block the sperm from getting through.  This is normal, to see mucus there, but because of the excess mucus in a CFer's body, there is way more here than is normal.  Once pregnant there are risks to carrying the baby and all that, but it is done, and has been done by many many CF women.  Please talk to your CF doctor about it.

The secondary issues such as the kidneys, liver and heart are caused by medications and lack of O2.  All of the medications taken on a daily basis are processed through the kidneys or liver and over time they can wear out causing the patient to need a transplant.  This may happen before the lungs give out or after.  And does not always happen.  Heart issues can also occur because of its need to make up for the lungs declining. Tachycardia is a common issue among CFers.  This is a fast heart rate, I believe above 110 BPM resting.

Check out this chart for more issues we CFers can encounter.

(source)

For more information visit the Cystic Fibrosis Foundation's website.

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.

May is CF Awareness Month!!!

How are YOU spreading awareness?

Me?  Well I have decided that each day I will take a friend / or stranger's question and post the answer as a reply on here and on Facebook.  FB will be the short version and the longer on on here.   Some days I may have 2 posts if I need to update about my health etc.  But there will be a post everyday.  That is my blogger challenge!

This is what I posted to the FB world:

Yesterday was the start of Cystic Fibrosis Awareness Month! Taking a cue (and some of her words) from Anne, I am doing this. Each day I will pick a question and write about it. I can give the short answer here and the long answer on my blog. Ask whatever you want to know about me and CF, CF, lung transplant etc. If I don't know the answer I have a stash of some dam good friends that can probably answer the question, especially if it pertains to the post-transplant aspect of it. Seriously ask anything!!! If you have a question you want to ask but not others to know about you can send me a message. Otherwise, happy thinking, and asking!!! ♥ ♥ ♥

***Just a reminder, any answers I give are from my own personal experience with CF and the information I've gained on my journey living with this disease. Please don't take any of what I say as advice or the only answer. Each CF'er has their our own story and their own routine as prescribed by their care team.***

Start thinking and posting those questions to me!!!

Bitten by the Flu Bug!!!

Yup, it got me, a little late in the season but it got me.  It started with Peter last weekend and I picked it up this weekend.  My CF doctor told me to come to the ER if my fever spiked again and since it was 103 this morning, we decided to take a trip.  Some fluids, a nose swab and a chest x-ray later and it was determined I have Flu B.

They are keeping me over night to monitor me and make sure my breathing doesn't get any worse.  Its very hard to cough since it feels like I have glass shards in my chest, but hopefully the TamiFlu and fluids will help with that.  Looking forward to being back to normal and getting my paper done this week.  I finished one yesterday and printed it out so I am going to make use of my time in here and get the other one done as well as the assistantship stuff and practice for my presentation on Thursday.  I have so much to do this week it really sucks the flu had to hit NOW!!!

I have an appointment with my CF doctor next Tuesday, the 7th, so we will see if this flu did anything to my numbers.  I was expecting to be put on IV antibiotics next week, but who knows now.  Right now I am just getting IV fluids, no antibiotics.  But that could change tomorrow depending on what my culture comes back with.

I might as well update on everything while I am here right....Thursday I started working out with my personal trainer.  LOVE IT!  I was so sore on Friday but a good sore.  I am looking forward to being in shape and toned up.  I cannot wait.  Thursday I also had transplant clinic and I got another clean bill of health and a "see you in 6 months" as I walked out the door.  Love that too!  I go back in 6 months and that will be right when I am finished with the PT so maybe my numbers will have improved some.  She was excited for me when I told her I want to look into going back to work.  She thought that was a great idea, as long as I figured all the SSDI and disability issues out.  I've got a year to do it...I had seen them right before the honeymoon from hell so I was telling her all about that and she was so shocked my lungs crapped out as much as they did on the plane.  She said they would have had to make an emergency landing had I not been wearing my O2 for sure.  She said some people just respond totally different to flying than others....yeah ya think lol.

So that is all.  Hopefully I am out of here tomorrow and back home curled up in my bed.  It took me forever to get out of the house today because I didn't want to move.  103* fevers will do that to you...

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...

Kicking Ass and Taking Names!

Yup that's right.  I am kicking some serious health ass and taking names along the way.  OK maybe not actually taking names, but I could if there were people to take names from....

Anywho...

I have been at the gym 6 days a week for the past few weeks and I am LOVING IT.  I take Pilates and Yoga on the weekends and tonight I did a Body Pumps class with barbells and weights and all that fun jazz.  I took it two weeks ago too.  There is one on Monday nights which I might start going to.  Its a dam good workout!  And considering right now I have less than one full lung working, I am kicking some SERIOUS ASS!  However, I wanted to attempt the Couch to 5k again, so I decided to try 30 seconds of jogging.  Yeah serious fail.  My lungs laughed at me, then died.  My O2 plummeted, but weirdly enough, my HR stayed about the same.  Went up a few points but I expected it to sky rocket.  So looks like I won't be running any marathons in the next few months.  But that is OK.

I have also been doing my vest every single night!  That makes almost six whole weeks of doing it, even when I went to visit family!  And today I did it TWICE!  Say whaaaaaaaaaaaaaaaat.  I know!

In other news...I dropped one of my summer classes.  So now I will be taking just the two.  I decided that three was way too many and bound to land me in the slammer.  Two I can handle.  And I can switch up my program some so I take one class this Fall and then an independent study next Spring and be done.  The IS is a portfolio class, and it entails just revising papers you want to include in your portfolio.

That is all for now.  I have a clinic appointment in May again so I will see if this exercising ass kicking is doing anything for my numbers.

Study Disqualification and More

Clinic went well today.  For the first time in a few years I am not trying an oral antibiotic on the first appointment after a round of IVs.  I feel OK, not fabulous, but not bad, and since my numbers are roughly where they were last time (post bronchodilator) I can wait and see her again in 2 months.  If I make it that long I will be ecstatic.

Since I had to meet with my professor this morning at 10 am and clinic was at 12:30, I knew I would be in need of a treatment.  My numbers reflected this.  I blew at my highest at 1.01L, 35%.  Last clinic I was 1.16L, 40%.  But I was trying to get into a Pulmozyme study so I asked if I could have an albuterol neb and try again.  We did!  I hit 1.15L, 40%....but it was not good enough.  The calculations they use for the study had me at 37.5%.  Sad :(  This is the first time I have ever been disqualified from a study based on FEV1 being too low. I am not happy about that.  What can you do though right?

Holding steady I am.  I registered for classes yesterday and my summer will be BUSY.  3 classes, one is a 12 week class, one is a six week class, overlapping the last six weeks of the first and the third class is a full week institute that will take place simultaneously with the other two classes.  So the third week of July I will have 3 classes!  My week is going to be hectic!  Monday I will be at school from 8:30-4:30, then Tuesday, Wednesday and Thursday I will be there from 8:30am-9:30pm and Friday again from 8:30-4:30.  Can we say fevers and sleep that weekend?  I don't care though.  I need to finish by December and to do so I need to take three classes this summer and two this fall.  Plus this will test me out on something I am thinking about...

Going back to work.

I have been working out at the gym five days a week doing cardio, but will be adding strength training in shortly.  Sunday's I take a Yoga class too.  My goal is to work my butt off (literally as well as figuratively), and see if I can get my heath more stable and get it up.  If I can get to 45% and stay off of IVs for 6 months, then I will seriously consider working again.  P is not excited about it, but he said he will support me....but only if it won't make me sicker faster.  I agree with him.  I don't want to go back to work and in three months have to stop again and be listed for transplant.  But I have nine months to think about it, weigh my options and decide if it could work.

Port Flushes

I finally did it!  Finally, like its been years or something HA!

I accessed and flushed my own port, all by myself!  P was there for moral and comical support.  He recorded the whole thing on my iPad in case I passed out so we could watch it later.  No worries, he would have helped me up off the floor....after he finished laughing of course.  That man loves me, and I him!

The fact that I did not pass out, did not vomit and was not squeamish at all is astounding!  I did stall for a few minutes before poking myself, but that was it.  Being able to do this on my own will save me a bunch of money and time in the long run.  Doubtful I will access myself mid-IVs but de-accessing on days the nurse is coming to change my dressing will be a necessity!

My Foreign Cities: A Memoir

Upon finishing the advanced copy, I was told by the publisher that I could write up a review and post it on my blog if I wanted to.  So here goes nothing!!!

My Foreign Cities Facebook Page

Amazon page

This is the first book based on CF that I have read.  There are a couple others out there, one of which I own, but I just have not gotten around to reading them yet.  Maybe it was the "pressure" from the publisher, or the shortness of the book that made me want to get right to reading it.  But whatever it was, I grabbed the book and didn't let go until it was finished (figuratively - it was my companion at the gym all week long).

Right from the start I was pulled in to the story.  Having CF I have a slight advantage over the average reader.  I was curious what Stephen's life was like.  Was he healthy growing up?  How did he and Liz meet? Did he get a transplant and was it successful?  Was he still alive?  All these questions were answered as I read along, getting to know Stephen and Liz and their life together.  I could commiserate with him and his hospital stays.  I could taste the meds as he inhaled them.  I could chuckle at the tackle box pill sorter.  I knew what all these things felt like, personally.  And its very reassuring to read about it in print and not just online.

The memoir is written by Liz, about her and Stephen's life as they lived with CF and the double lung transplant.  It gives a great perspective from the spouse's point of view, something that we as CFers will never understand.  After reading the memoir, I can better understand what P feels and goes through though.  Circumstances are different of course, but the fears, the hopes, the anxiety's and the sadness are all the same.    I have asked P to read it.  I know he will....in time.

NY Times states that this memoir is a modern day love story.  I agree.  The story begins with Liz and Stephen as just friends in school, then it slowly winds its way to love.  Their life is built up in front of your eyes, reeling you in and making you crave more.  I could feel the love, and the tenderness grow with each page I read.  The awkwardness of new love.  The questions of whether or not loving a CFer is a good idea.  Knowing you have no say in it - its all up to your heart.  I could feel my tummy tighten with anticipation as Stephen got the call.  I swallowed a few tears as Stephen had some setbacks.  I smiled as Liz talked about life now.

I don't want to give anything away, I want you all to read it when it hits shelves on April 8th.  You are welcome to pre-order online or with your local bookstore if you wish.  I highly recommend this book to anyone who lives with CF, knows someone who does, or who just wants to read a sweet story about two people who live as much as they can in the amount of time they have together.  

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...

Prevacid Duh

All week I have had horrible heartburn.  I have been eating Tums like they are candy.  At first I attributed it to my diet that I started Monday.  At the GI on Thursday I didn't mention it because I figured I would take 2 Prevacid in the am and see how I was by the evening.  I completely forgot about it.  Then yesterday it dawned on me....I ran to my pill container and what did I see??  NO PREVACID!  I had forgotten to put them in on Sunday with the week's pills!  Well duh!  So starting tonight my Prevacid will me back in my system!!!

(my anal OCD self did not allow me to take some yesterday.  My bottles hold one week's worth and I can't stand it when they don't line up lol.  So yes I suffered one more day...)

MRCP Results

Today was my follow up appointment for the MRCP I had back in January.  Good news is the cyst either shrunk or was miscalculated from the endoscopic ultrasound.  It is only about 1.5 cm up from 1.2 cm the time before (the endo ultrasound showed it at 2.2 cm).  Unfortunately, I need to have another MRCP early April so we can be sure.  And then in the same month, I need to meet with a pancreatic surgeon to go over my options - if I need surgery to remove it, if we can just follow it, or if we can virtually ignore it.  Hopefully we can just monitor it for now.

Bad bad CF patient!

I am a very bad CF patient.

Why?

Well...........I haven't done my vest since I got out of the hospital, almost 2 months ago!

AHHHHHHHHHHH

I know I know....the first few weeks I was sore from the port placement.  Then it turned in to me being scared that the vest would hurt the port anyway.  Then it just got out of hand.

BUT I am writing this post as I sit hooked up to the vest finally!  It is not a pleasant feeling, and I don't know if round one after not doing it for 2 months will last the whole 20 minutes.  I hear in time it will get less awkward and less irritating so that means I need to keep at it!

My port is still awkward for me.  I am extremely aware of its presence at all times of the day.  So the vest was just scary!  I can't even wear a bra because it annoys the site.  Which, thankfully, I was "blessed" with very tiny boobs and I don't need to wear one!

Lately the old lungs have been very sore and not happy so I decided I needed to suck it up and attempt the vest again.  It can't hurt....well....you know what I mean!!!

New Blog Title

For those of you who may have noticed, I changed the name of my blog.  "My journey with Cystic Fibrosis" was too vanilla for me.

From now on I will be the blogger on "Mastering the Art of Breathing, One Stitch at a Time."

I am happy with this new title!  And a huge thanks to all my wonderful friends on facebook who helped me pick a winner!!!

I will be posting a picture for behind the title once I have one I like.  There are ideas floating around my head that I need to work on.  Stay tuned!

Early Copy of a CF Memoir

I was asked to read a new book that is coming out on the market in April.  Its written by a woman whose husband has Cystic Fibrosis.

I am honored and thrilled to get a chance to read it before it hit shelves!  But my own full review will not be posted online here until it does.  Super exciting!!!

Charity's TEDx Video

Piper over at A Matter of Life and Breath had a guest blogger today.  Her message was simple.  Be an organ donor.

Check out Piper's blog for her guest blogger and this video of Charity:

Discourses from the Undead: Charity Tillemann-Dick at TEDxMidAtlantic

Our Wedding

I got so wrapped up in life after getting married I never posted any pictures!!!  How horrible of me :(

So here ya go!  A few pictures of the wedding and a few from the honeymoon...

 

"I do"

 Love the veil....and the groom!

Dress and flowers, and the hankie from my Christening

I love my dress so here is another one!

Kisses for him

Magic Kingdom

 Portable O2 made it into a few pictures!

 Micky <3

Best shot!!!  The whole wedding!!!

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?

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*

Breathe Decal

The other day I received my "Breathe" decal from Jamiebug!  I LOVE IT!  It looks fabulous on my laptop, and now when I am in the hospital and am using it, people will see it!

I also ordered a "Donate Life" decal for my car but I have not gotten that one on it yet.  Once I do I will take a picture and post it I promise.

Hop on over to her page and get some for yourself!  They help pay for her BugHugs which help transplanted patients while in the hospital.  Wonderful project by a wonderful Cyster!

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!!!

My Friends

Lately I can not stop thinking of my friends.  Shawn, Kelly and Rhi who passed away within a few short months of each other.  It makes me so incredibly sad.  These three were more than "just" CF friends.  They were close to me, special.  Shawn was even family.

Each three were at various stages of CF progression.  Shawn was almost 4 years post transplant, diagnosed with cancer (cancer did not get him - jury is still out on what really did).  Rhiannon was "just sick" in for a routine exacerbation when she declined fast and was immediately too sick to be activated on the transplant list.  Kelly had just received her wonderful new lungs, but within days, they became sick and would not work as they were supposed to.

Each of these people held a special place in my heart.

Kelly - sweet sweet Kelly.  She was a wonderful photographer.  We joked that she could fly out to photograph my wedding.  But instead maybe an anniversary down the line when she had gotten her second chance.  I got to meet her in Boston a few years ago for Paul's memorial.  She was so beautiful and sweet and artistic.

Rhiannon - Oh Rhi.  She was my first CF friend.  When I first joined the online CF world a few years ago (2008ish), she sent me a private message after a post I had made.  We were both going through similar issues with our boyfriends.  Neither wanted to deal with CF but neither was leaving either.  Well mine was halfway through the door.  We chatted often and came to realize that she was "the slightly older, slightly sicker version of me."  It was a joke we kept all this time.  We maintained that friendship and I was so excited when she married Kev.  I tried to make it to the wedding, but it wasn't possible.  I wish I had been able to meet her in person.

Shawn - Shawn is the reason I am married.  Thanks to him and his wife, P's sister, I never would have met P.  I never would have met my soul-mate.  Shawn was full of life and fun and he always knew someone to help you out.  He was a man of all trades.  He was my brother in law.  He was P's best friend.  He was our best man.

These three fabulous people were dear to me and now they are gone.  And some days, as I am driving, I think of them and I can't help but let the tears flow...