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.