I have referred to this site a number of times and it is usually successful in answering most questions you have thought of (and never thought of!): Cystic-L

It is actually a free e-mail service/support group for patients, families, friends and health-support workers of PWCF (people with Cystic Fibrosis) that started up in 1994. I used to subscribe to it, but then my inbox got increasingly crowded, so I opted to visit the 'handbook' section of the site instead. I recently found a new appreciation for this website when I clicked on the PFTs Explained For You! link. Amazing how after 22 years, I can still be confused by all the numbers on this test. The Handbook is a little outdated in some sections, but still very useful.

What is a PFT Test?
Here is a great video that shows what it is: PFT Test

The only difference with this one is that I am usually sitting down and my face turns a nice shade of lobster red. I also often see floaters/stars by the time I am done blowing, then I spend a lot of time talking to the respirologist so I can avoid doing it again. By the way, this shows one attempt--depending on how you fair, you will usually do multiple attempts until you have done the absolute best you can do (the average is 3-4 attempts).

I will try to explain more in-depth what a PFT test is later this week, but here is the gist of it:

• A PFT test measures the volume of air in your lungs and how much air you can blow out in one second (there are also many, many other things it tests **See Handbook above:" PFTS Explained for You!"** )
• This is important because it compares the functioning of your lungs to other people your age and size
• It helps determine what state your lungs are in, e.g. if you are stable, sick, need to be referred for a lung-transplant, etc.

My favourite part about going back to school is stocking up on necessary (and unnecessary) supplies. What can I say? White out, pens, file folders, notepads, sticky notes and binders all lead to my uncontrollable excitement. Maybe this is why I always end up with office jobs—the post-its get me every time.

So today I went to Dollarama with the intention of purchasing a few birthday cards and—as is usually the case with Dollarama—ended up walking out with much more than I had planned, including some lovely additions to my school supply collection. I only purchased a few file folders because I don’t really need much this year, but I realized that my list of back to school supplies has extended to include some rather unusual items.

Additional back to school necessities for Sept. 2009:

One of the most important weapons for avoiding colds, the flu and nasty bacteria—either of which could easily be a one-way ticket to months of IVs, lengthy hospital stays or transplant status. I wash my hands like crazy, but because I don’t travel with a kitchen sink and soap (my purse is not big enough) antibacterial is the only way. I am glad to see more places are setting up purell pump stations and more people are actually using it. It makes my job—of staying healthy as long as possible—a lot easier.

• Clorox Wipes

For the times people don’t wash their hands properly (we’ve all seen them in the bathrooms and been grossed out—you know who you are!!) and the times people sneeze and cough all over shared computers, equipment, etc. Clorox wipes are essential for any computer lab, which I consider a boardroom for bacteria plotting to take over the world.

• Cold FX

When Clorox and antibacterial fails you, there is Cold FX, which is plan C. Apparently the extra strength works wonders when you have the first signs of a cold or flu (e.g. sore throat). I think this September I will take the milder everyday version for preventative reasons, my faith has yet to be restored in my immune system.

• Vitamin C

Yet another immune system booster that is no big deal to add to my pill regime. I’m not big on drinking OJ unless I have a ridiculous sugar crash (I’m not diabetic, but my body likes to surprise me with sugar lows for the heck of it) sooo I prefer the pill form.

• Flu Shot and H1N1 Shot

I did not get my flu shot last year because I kept getting sick… so I've got to get on that this year. Also, because of the Mex—er, swine pigs (sorry, I’m a terrible person) I must weasel my way into getting the H1N1 shot. I was told that people with respiratory problems are always high priority anyway, so I will most likely be getting it (and it might be two shots). The media is already hyping up the back to school pandemic, so I’m not feeling so hot about adding that concern to my agenda. I really don’t think H1N1 will fit into my schedule this semester—sorry swine flu!

Fun fact, if someone is “high-risk” for flu complications in your household/someone you are close with, you should also get your flu shot because you can be a “carrier” (have no symptoms, but pass it on). That is probably a housewarming gift you should skip.

Other possible additions to my back to school shopping list: oil of oregano, tea tree oil and other immune-system boosting valuables.

Let’s start this Wednesday night off with the “c” word: compliance. It’s the word you hate to hear and it means you have to do what you are told when are told, no matter how much you bitch and moan about it.

I’ve never really had an issue with compliance, we’ve been pretty steady from the get-go and we are hardly ever separated. Sure, there are minor blips here and there, my leash gets a little loose and like an excited dog in the park I try to break free and chase the squirrels, mistaking a loose leash for a thing called freedom—but really all I end up doing is strangling myself and nursing the after effects of poor judgment. This is all figuratively speaking of course; I chase cute irresistible bunnies, not squirrels. Let’s be reasonable.

I am a little bit of a doctor’s pet in that I very, very rarely skip mask or physio treatments (I might miss part of one during the odd special occasion) and this is quite an accomplishment considering I have done three treatments daily for as long as I can remember (four treatments daily when I am sick, but that gets a little much). In fact, my friends are pretty surprised to learn all the shit I do when I stay over for a weekend. I would guesstimate that treatments take up 5 hours of my day, sometimes more, depending on the type of day it is. For instance: the “PEP” mask (which is a mask you breath into that creates positive pressure thus making you cough up the junk trapped in your lungs) supposedly takes 20 minutes. I say supposedly because it very rarely takes 20 minutes because of all the coughing it causes—40 minutes is more like it. Speaking of PEP I am always told I have “perfect” technique too… wow I’m such a brownnoser...

Anyway enough of my bragging and back to the issue of compliance, it is probably THE most important aspect of keeping healthy (or stable, which is what I consider “healthy”) with CF. So that is why I don’t miss treatments (not to say I am a complete angel). I suppose it is because I’ve been in the hospital easily over 10 times (I have lost count, so I just say 10+) and would do anything to stay the heck out of there. But mostly it is because my lungs are greedy little devils that require constant attention—missing one treatment=no sleep and coughing fits for moi.

But there are only so many hours in the day…

It is amazing where you can find the time if you really look. I am a bit of a scheduler, which is helpful, although I don’t follow times to a tee I have a general idea of when I do things. For instance, when I have a full time job (which I do in the summer) I wake up early for my mask + pep, do another one right when I get home (or after a snack) and another one around 11 at night. Strangely enough it is when I am on these tight schedules that I strive, because I am aware my time is so compressed and I am less likely to waste it. And yes, I am even able to fit in a workout (though I was much better with that last summer opposed to this summer…) and there is still time for downtime as well. It IS possible, just a little bit exhausting at times and you have to make a conscious decision about whether or not it is worth it for you.

Here’s my biggest reason for being compliant:

The day when I do go on the active transplant list I don’t want any regrets. No “What if I had taken better care of myself? What if I hadn’t skipped those treatments?” Regret is a horrible feeling and not one that I wish to experience when it comes to my health. Once the damage has been done there is no turning back. I want to know that when my time with these lungs has come to an end that I have made every last-ditch effort to keep these babies going—I do have a Scottish background after all and we like to make things last as long as possible.

Tonight I decided to sway from my regular holiday routine of eating, shopping and otherwise acting like a poster child for half of the seven deadly sins, choosing instead to hop on the treadmill and sweat like a pig.

It is important for everyone to exercise, but when you have infection-hungry lungs it is even more important. You would think that a transplant assessment hanging over my head would be motivation enough to workout daily, but as of late 1-2 times a week seems to be my max. If the amount of calories I consumed reflected in my BMI I could qualify for Biggest Loser and get my ass kicked by angry/hungry trainers—but sadly this is not the case and I will have to kick my own butt into shape. I’m not nearly as mean.

In order to do this I will have to actually set aside time to workout, which sounds simple enough but there are always better things to do like: sleep, shop, watch True Blood, search for future jobs, read texts from last night—you know the drill. I kept saying it would be easier to exercise more when I was done work; well, I’ve been done for two weeks now… We all know the “I have no time because of work” line is BS anyway. Who was I kidding?

Introducing my fitness goals for this month:

• Workout 3 times a week (30 mins cardio plus weight baring)
• 15 minutes at 4 mph on the treadmill (I currently do 10 minutes)

Let’s see what kind of trainer I can be.

I come across quite a few interesting websites and blogs regarding CF from time to time, so I will start sharing them here.

For today, I found this site called Club CF. Sounds oh-so-exclusive, but there is a section for caregivers too. I liked this site because it had a section for all ages (20+, 30+, 40+, etc.) and it had some inspirational stories. It seems a little bare right now, but I think it will catch on.

I may not be a marathon runner, but I imagine the most satisfying part of running a marathon would be triumphantly crossing the finish line to the cheers of slightly less-athletic folk—not all that exercise crap in between. After a marathon week (which flew by much faster than I anticipated—I guess I made good time?) I have finally crossed the finish line. I grabbed a Tim Horton’s right after.

Today was very brief and almost relaxing. My parental units and I had a visit with the social worker that lasted just over an hour. The basis of the meeting focused on drug coverage and financial security before and after tx. OHIP is not a guardian angel that has your back at anytime—as most of you have probably discovered—and thankfully I am still under my parents’ private insurance, effectively avoiding the pricey CF drugs that can cost hundreds of dollars each month. Post-tx some of the medication price tags get even bigger, one in particular costs upwards of 1000 dollars a month. Guess I’ll have to cut back on shoes and all things pretty. On the plus side, this is the perfect excuse to build up my emergency supply of pumps and boots now for future use.

Financially, post-tx planning is a lot of work. I am required to apply for the Trillium Drug Program, even though it will not really help me out too much because my parents are multi-millionaires according to the Canadian government (HA HA HA). Unfortunately I am a victim of the middle-class squeeze and not too much can be done about it. I also asked about ODSP (collecting disability) but I have to either be in school just part-time (which I am doing this year anyway) and I cannot work at all. That doesn’t really fly with me because I like to keep busy and not feel terribly useless, so I will work until I absolutely cannot anymore.

Looks like I have a lot of financial planning to do for the future. I can remain under my parents’ insurance as long as I am a student or until I turn 25, whichever comes first. I’ll have my diploma in journalism and BAA by next year…Masters, anyone?

It is important to have all this stuff set up now because once you go on the list there is no telling when you will get “the call.” The shortest wait was apparently six hours, the longest wait was three years. She said not to be surprised if you have to wait two years.

Before we tied up the meeting we got to the more personal questions that generally make me feel useless and juvenile like: do you do the cooking and housekeeping? Do you do the laundry? Grocery shopping? (Okay, I do the last one sometimes) and my answer to all of the above was “No, my mom does… that too, that too…” Publicly I am ashamed, but privately I am okay with NOT doing these things until I move out, officially grow up and settle into my lucrative career as a professional journalist… at age 30.

I really liked this social worker, she shared my sense of humor and was not too touchy-feely, which is a quality I despise in a social worker (e.g. "How do you feel? Are you sad? Are you mad? Do you hate your life?" etc.) That mushy junk turns my stomach.

Nice way to end the week!

Shout out to my family for coming along this week and a special thank-you to my Cystic Sista, H, who has been very supportive the past few months and gave me a very special friendship necklace to wear during my assessment week--I wore it everyday! (I will try to post a pic later... for now you should check out all her nice stuff at http://www.gothattitude.com)

**FYI for those of you who were trying to make comments on my posts and it wasn't working I THINK I fixed it now...**

After an amazing sleep-in and huge breakfast, we headed over to TGH for our appointment with the anesthesiologist, Dr. Karen McRae at noon. Let me just say, fascinating appointment. She described absolutely everything that will happen from how they retrieve donor organs to how they do the operation and threw in tidbits of interesting information. For instance, did you know that hearing is the most difficult sense to block once you are put to sleep? This is why you will hear rare accounts of people “remembering” their surgery—it is because they overheard something during their surgery. In order to avoid this type of memory, tx patients are given “amnesia” drugs after—erasing your memory, though it would have been cooler if they did it Men In Black flashy pen styles.

After this I prepared mentally for the dreaded MUGA scan, which my Cystic Sista, HArmstrong (I’ll just refer to her as H), had already warned me about. The MUGA scan determines how well your heart can handle stress, which is obviously very important for such a huge operation. H was not kidding, the test was hard! The first part was deceivingly easy, all I had to do was lie there while they injected radioactive tracer into an IV in my arm. They took pictures with a giant camera for about 30 minutes--the technician made a corny joke about them treating me like a pawn when I am a Bishop. Then came the second part of the test, which was designed by the devil. While lying down (and with the giant camera on top of me) I had to pedal (up to 40 on a gage… she originally said 50, but I couldn’t make it there) for as long as I could. Well, ladies and gents, pathetically enough I only made it for about one minute. I wasn’t pouring with sweat or anything, but I was soooo out of breath and I felt my heart thumping in my ears (also they told me to stay as still as possible, which meant I had to grip the side of the table because my body weight alone is not enough to keep the pedals from pushing me up…).

So I think the exercise portion of the MUGA succeeded at making me feel like a failure, as exercise tests usually do. Maybe I would have done better if I had my oxygen (for some reason they couldn’t supply it for me there…) or maybe not. In any case I am glad that is over and done with! I had a massive throbbing headache right after the test and for the remainder of the afternoon.

Before I left TGH I did one last blood test as a token of my appreciation (only three vials this time) to check PRA levels in my blood. This just checks for your immunity to other people (and I think other things too, but I can’t remember at this point.)

Back home tonight and I have Thursday off! All the ‘hard’ tests are officially over.

I woke up a little groggy today, only got about 5 hours of broken sleep. I tried to be as quiet as possible as I did my mask at 6:30 (my mom, dad and sister were still sleeping in our hotel room). We headed over to TGH at 7:30 for an echocardiogram (echo), which is basically just an ultrasound of your heart. This will show how well my heart pumps blood and provides a closer look at how the valves function. It is a really easy test; all I do is lie there while the technician places a wand on my chest. He explained everything I was seeing and hearing on the monitor, so I was getting my education… even if I just pretended to understand some things…

Since I was in and out pretty fast, we went back to the hotel room to meet my sister and go out for breakfast. Our next appointment was with the Transplant Coordinator nurse, Michelle. She told us a lot of stuff that we had already researched and knew, but thankfully we had because there was A LOT of information. She gave me a huge blue book that tells you exactly what to expect pre-tx, during tx and post-tx. It is even broken down into an hour-by-hour basis. To date, I have only read about 5 pages—I need digestible chunks of info and some of it is hard to chew.

A Glimpse Into the Future

Michelle told us that 90% of tx patients experience at least one of two complications: rejection or infection. So you have to expect that you WILL get rejection and infection. Rejection, because you are introducing something foreign to your body. For instance, when you get a splinter your body wants to attack and destroy and get it out of you. So, in order to avoid this type of assault on your brand new sparkly lungs, tx patients are put on immunosuppressants (medications that lower the immune system and ‘trick’ your body into accepting your new tenants) for the rest of their lives. Of course, without a strong immune system you are open to the next complication: infection.

Anyway, those are two complications that are to be expected. Along with a host of other complications that are tag-a-longs. In response to these complications there is a long list of combative medications, which can also lead to more complications! Mind you, looking at the list of medications, I have already been on some of them including the most evil of them—prednisone, which I have been on for most of my life already. So who knows?

The message of the meeting was basically this: Transplant is a treatment NOT a cure. This is what makes it difficult for a lot of people deciding whether or not to go on the list. Essentially you are trading one set of familiar problems for another set of unfamiliar problems.

The Verdict on Living Donors

We also asked about the possibility of living donors. It has not been done at TGH yet and it seems to be discouraged—rightfully so, because it is a huge decision with questionable ethics. With a living donor donation a lobe is taken from two separate donors, which are then placed into the recipient. Those two lobes become the recipients two lungs. The donors have to be a certain height taller than the recipient, which makes me an ideal candidate because I am tiny (it is usually done on people who are smaller or children). However, the donors will lose a percentage of their lung function permanently and are at risk for complications like infection. While I would feel just fine with 2 lobes (normal lungs have 5 lobes) because my body is adjusted to crappy lung function, the donors would feel pretty horrible because they are accustomed to full lung function. Also, unlike the liver, the lungs do not regenerate and grow back.

Donors would also have to undergo more rigorous testing than me and I would have to have five potential candidates PLUS caregivers/support people for the donors (they would only pick two donors of the five) and me. SO doesn’t look like living donation will be an option for me.

Other things I've learned…

People with Cystic Fibrosis are not allowed to hang out after tx, even if both of them have had a tx.

For those of you who don’t know, people with CF are not allowed to hang out because we grow bugs in our lungs that can be harmful to each other. We constantly go through great lengths to avoid each other, which is especially hard when we are in the hospital or at clinic at the same time. I will probably expand on this later.

All-in-all very informative meeting.

Test One, Two...

I only had two more tests after this meeting. One was a CT scan of my chest and sinuses. Really simple, you just lie there (again) and the machine talks to you in a robotic demanding voice (breath in, hold, breath). This test was soooo much easier than it was in January. In January I was panicking about holding my breath long enough (it feels like it is about 10 seconds?) but this time I had no problem.

The last test was a six-minute walk, which is exactly how it sounds. A physiotherapist will time you as you walk and then record how much ground you were able to cover in six minutes. I did this test before when I went for my consultation in May, only this time I used my oxygen (3 L on pulse. Pulse means it gives me oxygen when I breath in, rather than a constant flow) and wore running shoes instead of heels. This time I actually broke my record (which was already pretty decent) by 34 metres! I can now cover 529 metres in 6 minutes, which is amazing! Woo! (I usually will not be walking that fast though… my fast-walking/sprinter friends can dream on...)

I was really tired after the tests today… so I napped for about an hour when I got back to our hotel room. We went out for another nice dinner at the distillery district (got lost for about 30 mins first, thanks E for your help!), then went to a pub and had sangria (I have a test tomorrow that says I can’t have any caffeine for 24 hrs before, so I replaced caffeine with alcohol). Tomorrow doesn’t start until 12, so I will get my sleep-in!

P.S. Thanks to my sister for coming today even though it was her big 2-0.

First thing I noticed about TGH? The food court. I had been fasting for 12 hours, which meant I was 1-caffiene deprived 2-ravenous and 3-easily irritated. So it didn’t help that I was walking through this amazing food court with all the trimmings (did you know TGH has two Tim Horton’s and a Starbucks? Now you do.)

I had been up since 5:30 in order to get downtown by 7:30 and we had made good timing, which excited me because it meant I could break my fast a little earlier (can you tell I dread fasting?) TGH is a beautiful hospital to navigate compared to St. Mikes, I only had to put my glasses on once to read the signs.

First, I went upstairs to register with the pre-assessment office and grab my new schedule, then headed on down to the blood lab. The waiting room was packed! There were tons of people there and thanks to my eavesdropping skills I learned that most of these people were either pre or post-tx (tx = transplant).

Unfortunately, one woman was a little too open about her tx experience (deciding to speak at a volume that could wake the dead…which I know because it woke me up) and decided to blab about how everyone should be an organ donor and it should be an automatic process, blah blah BLAH. While I am obviously pro-organ donation (and bias because I need one in the future) I am not about shoving my opinion down peoples’ throats and this woman was making me regurgitate before 9 am. Anyway, a few of us laughed in the corner, then I got called for blood work—where they proceeded to help themselves to 11 vials of my blood like it was a vampire buffet. After blood work I was a happy camper because that meant I could indulge in the awesome food court and have my morning tea!

Next I went for chest x-rays, which are pretty routine. I ended up waiting there for quite a while because they accidentally forgot about me, which is not routine… BUT I met two brothers who had already had lung transplants. One was getting assessed for his second transplant, while the other said he was going to be assessed for his third transplant soon. The second brother had his second transplant in 1994—a time when second transplants were not common—and was only given a 5% chance of survival. Obviously, he has since beat those odds!

After x-rays I met with the dietician. At the risk of sounding negative I will just come out and say it: I wasn’t a fan. Not only did she not bother to weigh or measure me, but also she rounded my height to five feet (I am flattered, but let’s be realistic here, I’m barely 4’10) and guesstimated my BMI. Ummm sorry but that is not really professional when you are evaluating someone to see if they are a suitable candidate for the biggest surgery they can have! So I wasn’t a fan of her, especially after she said my BMI was 18 and ‘just good enough’, I know for a fact it is higher! And yes, this is a sensitive subject because I work hard to get my weight on. I may even lose sleep tonight.

Anyway, after that I only had one more test left called a V/Q perfusion scan—basically you have dye injected into your vein and you lie under a camera that traces the dye to see how the blood and airflow is distributed into your lungs. Sometimes one lung will have more blood flow than the other because it has been more damaged from infection. This will serve as a guide in the future during the tx to help decide which lung to remove first.

All in all it was a pretty good day, everything ran extremely smoothly and on-time. The hospital environment was pretty relaxed and although I am exhausted from all the waiting around, I was more than okay because my parents booked the Delta Chelsea for two nights so I would have close sleeping quarters to the hospital. Tonight we went out for my sister’s 20th birthday dinner, ate good food, drank good wine and made the best of our hospiday (I tried to figure out a clever way to put holiday and hospital together...)

One day down, three to go.

So now that I have spent a good hour on blog aesthetics, I should probably actually BLOG. Admittedly, I have had a blog before, but not with purpose and it was usually reserved for emo-tastic, teenaged rants. Now, I proudly present to you my blog with a purpose, a rather big one at that: to document my journey with Cystic Fibrosis.

For those of you who are new to my world …

Scientifically speaking, Cystic Fibrosis (CF) is a genetic disease (you can’t catch it, no matter how badly you want to!) that affects the lungs and digestive system. A thick build-up of mucus in the lungs causes chronic infections and inflammation, which in turn causes severe breathing problems. In the digestive system, the pancreas is unable to release enzymes to the intestines to digest food because of the thick mucus blocking its way. This makes it difficult to both digest food and absorb nutrients.

Personally speaking, CF is a job that requires hard work, dedication and few benefits. On average, I commit 6 hrs a day to treatments. CF is also a character builder, a binding element for friends and family and so much more.

What does this translate to?

Well, in order to prevent these lovely infections and keep my lungs as clear as possible I must do physiotherapy three times a day (20-40 mins each) and inhaled antibiotics twice a day. I also take pills (artificial enzymes) to help me digest my food and lots of other pills to control things like GERD, inflammation and infection. If you shook me, I would probably rattle—not that I am suggesting this!

So that’s my real deal…

And for those of you that have been following me closely this past year, you know that it has been quite an interesting year health-wise for me. I spent 10 weeks on IV antibiotics and endured countless antibiotic changes due to allergic reactions and certain combos simply not working. It was a year of not-so-brag-worthy firsts including: my first time spending six weeks in the hospital (usually it is one week, two weeks max), first time spending 10 weeks on IVs, first time spending my birthday in the hospital and first time needing to use oxygen.

It was around the time of my 6-week stint at St. Mikes Hospital in Toronto (which I lovingly refer to as a 2 star hotel) that the decision was made to begin the assessment process at Toronto General Hospital (TGH) for a double lung transplant. To be clear, this does not mean I will be going onto the 'active' list and actually physically preparing for a transplant. Rather, it means that I will be going through all the tests to see if either:

1. It is too early for me to go on the transplant list and they will follow me closely in the future
2. I should go on the list now (unlikely) OR
3. I am not a suitable candidate for transplant (I am told this is very rare for people with Cystic Fibrosis because we are so young)

**FYI I have my fingers crossed for option 1**

Why am I doing this now?

Well for starters, the assessment process is a very long process (one week) that involves long days, lots of tests and meetings and lots of traveling. All of this requires energy that is simply not available when you are sick and all you desire to do it watch trashy television or What Not to Wear and lie in bed.

Secondly, CF is unpredictable and I am unpredictable. I have not had a ‘steady decline’ rather, I was once on IVs yearly (or more) when I was a patient at Sick Kids and I had significantly low lung function (so low that they suggested I be placed on the transplant list). However, my health improved and I have been able to go longer without IV treatments—for reasons unknown. My standing record is 3 years, but the time I am spending on IVs is becoming longer and infections are becoming more aggressive.

Thirdly, I am playing the number game. When CFers talk about their lung health they tend to use a lot of numbers: PFT (Pulmonary Function Tests—which is a machine you blow into that calculates how much air you can blow out) numbers, such as FEV1 are important indicators of lung health. FEV1 is important because it shows how much air you can blow out of your lungs in a second—this is an indicator of how much obstruction you have. For instance, someone my age and height should have an FEV1 of 2.25 L and my FEV1 is 0.87 L, so I essentially have a little more than half a working lung. That sounds much more dramatic than it is though...

So there is your CF 101 for now. Staying tuned for Day 1 of my transplant assessment (also referred to as tx)!