To port or not to port?

That was really never a question for me.

I've been getting PICC lines for the last 20 years or so and with nearly (over?) 50 under my belt, the answer to getting a port or not was an easy one - If it ain't broke, don't fix it. Well, that was the easy answer.

Recently, I've had issues with my PICC lines that started making me open to the idea of actually entertaining a port. It started with PICC line placements taking longer and longer because of so much scar tissue and switching to the other arm when the first one was "shut off". Then there was the time (last year on Thanksgiving) that the PICC line got stuck in my arm the day of discharge and the worry that came along with thinking it may have snapped and was now making it's may through my body. The straw that broke the camel's back, and my tag line, was this last hospital stay in April when they had to leave the line short and weren't able to do any blood draws from it. That became particularly sucky when I had to spend two weeks in the ICU and 35 days total in the Hole with multiple stabs and blood draws.

In short, it's broken.

After I was released, the decision was made - next time I get a tune-up, I'm going with a port.

Well, that time is now.

A port was placed last week and the procedure was actually faster and less painful than getting a PICC placed. There was (and still is) a recovery period with some pain, but it was the same, albeit a longer period with a port, with the PICC.

I was always asked why I didn't get a port (see my answer above), but I thought it would be fair to all of you guys to really think about the answer and try to articulate it in a blog. Here is my attempt.

1. I never liked the idea of leaving the hospital with a medical object in my body. In fact, I'm still not wild about that idea, but it is what it is. What I loved about the PICC line was the fact that when I went home after a hospital stay, I felt like I left the hospital at the hospital. Let me remind you (maybe contrary to what you might think), I don't think about my cystic fibrosis that often. I'm too busy with life. I'm too busy thinking about others' cystic fibrosis. Frankly, I don't see a reason to think about my disease. Doesn't move the ball forward. By leaving with a port in my chest, I'm afraid that I may think about CF more than I want to when I look into the mirror...although I guess you could
argue that the 6 inch scar on my belly would do that.

2. A port always seemed to be the "next (unwanted) step" in the CF life. If you haven't noticed, I like to be different. I don't find my identity in having a disease and therefore try to avoid doing "disease things". I don't know how to put that more eloquently. If I was supposed to live a certain way, feel a certain way or die a certain way because of cystic fibrosis, I would fight like hell not to. I've always been good at compartimentalizing my CF and separating myself from all of the sickness and dying around me, while still being fully immersed in the community. Getting a port just felt like I was taking a step towards being more disease and less Ronnie.

3. I've always been worried about any limitations, real or perceived, with a port. The first two objections I had can be easily overcome as they are mostly mental. I've always prided myself on my mental approach to (CF) life (thanks Mom!!) and there is no doubt that I'll be able to look past (or justify) the medical object in my body and the next step in the CF life, but it's hard to look past limitations that could be physical ones. I've heard the stories. Friends of mine who had to stop certain workouts because of the port. A gentleman in the community who had to change careers due to a port. I don't like being limited. Part of my comeback story has been the ability to push myself in the gym and live life like a nut. If I feel at all inhibited in doing so, I'm afraid of the mental impact caused by the physical limitation. With that said, we've all seen some amazing athletes with CF and a port.

So there you have it. I tried to be as honest and thoughtful with this post in identifying the road mental block to getting a port. Now that the port is in my chest, there is no turning back. I'm all in. I will own this silly little button in my chest and use it as a reminder to push myself even harder to avoid taking the "next step" in this CF life.

Plus, my wife thinks it's sexy. And that my friends, doesn't suck :)

Hemoptysis in the Hospital

This has proven to be a different kind of hospital stay for me so far. Many of my stays are exactly the same in terms of how I feel coming in and how I feel going out. I have the usual chest tightness, increased cough, increased sputum, decreased energy, and the list goes on. Generally, I come into the hospital not feeling great in the first few days always have me feeling even worse due to the poison antibiotics running through my veins. After the first few days I start to incrementally improve over the course of the next two weeks usually to be discharged feeling like a million bucks.

This stay started out like so many before it but a recent turn of events have made it unlike any other.

On Wednesday night I was falling in and out of sleep watching Netflix. I woke up around 12:45am or so and decided to put the head of my bed down to be a little more comfortable. And then it happened, a feeling that many of my fellow cysters and fibros the world over recognize, the rattling of the chest and the gurgling in the throat. I knew immediately what is happening as it is a sensation I have felt many times before.

Only a representation of what I remember it looking like :)

I got out of the bed and walked into the bathroom and spit out a mouthful of bright, red, fresh blood directly into the toilet. I proceeded to cough up a generous amount of blood into the toilet before turning to the sink as the blood splashing upon the toilet water and dripping onto the floor was not painting a pretty picture. At this point there was blood on the toilet seat, on the floor around the toilet, in the toilet and on the walls near the toilet. As I was transitioning from the toilet to the sink I let out a cough that I couldn't quite hold in and blood spattered on the faucets and wall behind the sink. I leaned over the sink turned the water on and tried to clean as I coughed. I was doing my best to not get the blood everywhere, as evidenced by my hands which were now covered, but my attempts failed as the bathroom looked like a murder scene and I was the prime suspect.

In between one of my coughing fits I managed to press the call light to try to get some assistance. At this point I was feeling a bit lightheaded and worried that I wouldn't be able to stand much longer. I can't imagine what my nurse thought as she walked into my room well after midnight not finding me in my bed but instead covered in blood in my bathroom. If it's any indication her first words were, "Oh no!!"

Within minutes there was a rapid response team in my room. I stayed in the bathroom until the blood ceased and when I walked out and saw 8 people standing in my room or immediately outside of it and I said, “It's not that big of a deal guys!". I was sat down in the chair and the questions ensued.

Yes, this is happened before.
No. There hasn't been this much blood since 2009.
Yes, I feel lightheaded.
No, I don't feel dizzy.

Since the active bleeding stopped and my vitals did not look alarmingly bad, they just decided to order an x-ray and different blood panels.

On Thursday morning I felt like I had coughed up a lot of blood the night before.  I had body and joint aches and my lungs were experiencing a bit of pain.  I also sat up for the rest of the night as to try and avoid another bleed.  The rest of the morning was normal with doctors coming in and out of my room and breakfast nourishing my body. Then around 1:30pm or so, it happened again.

This time I grabbed a nearby bucket that they had given me the night before as I new they would want to get a measurement. The rapid response team was again called and as a result of my prior history and the fact that I had coughed up an additional 100 mL on top of the estimated 150 to 200 mL the night before, I was sent to the ICU for observation. They wanted to be sure I was in a place that I could get immediate attention if a massive hemoptysis visited me again.

After consultation with the pulmonary team and interventional radiology it was decided that a bronchial artery embolization (BAE) was needed. I've had two of these procedures in the past, once done to both lungs and once done to only one side (forget which one). I was admitted to the ICU on Thursday afternoon and wheeled down to have the procedure on Friday at 5 PM.

Not my lung. My bleed was from my left BA and they used foam and beads.

The procedure took about two hours to complete and according to the surgeon's notes and also the fact that I have not coughed up any more blood, it seems to be a success! I can will bleed again in the future and just pray that it will be at least another 6 years before it is an episode that needs to be addressed.

I was moved back to my regular floor late Saturday night and I'm very grateful to the ICU nurses who took care of me, the interventional radiologists who did my procedure, all those involved on the rapid response teams, my nurses and techs on 3NE, my pulmonary and CF teams, and all my family and friends.

Side Note: I have to brag a little bit about my family support. Mandi was at our home in Chandler when the second episode of hemoptysis occurred.  In fact, she happened to FaceTime me as I was holding the bucket of blood. The concern on her face was of course expected and immediate. I told her not to worry and that I would call her with an update. Before I had the chance to call, she let me know that the girls and her were on their way up to meet my in-laws at a halfway point between our houses. Eric and Nancy didn't think twice about taking the girls for an unknown period of time even though they had just picked up dear friends from the airport who were in town for a visit. I am so very grateful. I'd be remiss if I didn't mention that my wife ended up driving back to Phoenix the next day to get the girls and bring them back down the very same day. They arrived shortly after my procedure and were with me while recovering (Friday night) and the entire weekend. When my wife wasn't by my side, my mom was. And finally, I received many texts and prayers of support from my family and friends.

And finally, honor and praise to my Lord and Savior Jesus Christ who guided the hands of the IR team, protected my wife on the roads and sustained my health and spirit throughout the entire process. God is always good.

"I Lived" Because of Christine

I was very impressed (that would be an understatement) when I saw the new One Republic music video "I Lived" for the first time. It may be the best awareness video that has ever been put out in the cystic fibrosis community. It is rare to find an awareness piece that does such a great job in striking the perfect balance between education and inspiration. 

"I Lived" by One Republic

The music video below features real-life CFer Bryan Warnecke talking about what CF is, how it's treated, what his expectations are, his fears, his blessings and everything in between. What I took note of however, which may ultimately be most important for CF patients and their families, may not be what others noticed. Before I get into that, please watch the video below:  

Be Who You Want To See

So what struck me most about that video? His parents. I wish I could give them a hug and a high-five and say "job well done". They put their son in the best position to live out the title of the song. That video was much more about life than it was about CF. And when our parents allow us to live, in spite of CF, we can often look back and credit their approach for our successes as CFers.

Did you see how they raised Bryan? And in return, Bryan's attitude toward a life with CF?

"It's just one of those things that really makes you appreciate life. Makes you appreciate where you are as a person." - BW

That would not be his attitude if he was raised to fear a life with CF. If he was made to think that life gave him the short-end of the stick.

I do not know them personally, but I guarantee they modeled that mentality through action and words.

Judging by the clips, the last thing on their mind was that CF was a life-altering or life-shortening disease. Judging by those clips, they parented their son Bryan, and not the disease cystic fibrosis.

A Life In Spite of CF

So what did living life in spite of CF look like to them?

• Falling into a pit of foam, in a public play place (eeeeeeek!!!) at 1:17
• Riding a bike and falling over without a parents fear that their son is somehow "more fragile" at 1:47
• Riding in a tractor down a dusty road (oh no!!!!!!) at 1:56
• Water skiing in a lake (please make it stop!!!!) at 2:07 and 2:11 (with mom cheering in the background)
• Tumbling over in an electric ATV then being "put back on the saddle" and encouraged to try again at 2:19
• Boogie boarding in the ocean at 2:26
• Jumping head first into a pool, maybe public (ahhhhhh!!!), at 2:35
• Snow skiing at a presumably high altitude at 2:58
• Back to the dusty tractor ride at 3:00
• Playing hockey, which I assume means he's in a public locker room with his team at some point (I can't take it anymore!!!), at 3:24 

A Life Ruled by CF

Now, I didn't randomly pick those scenes out of thin air. I picked them because they are all things my mom would have prevented me from doing 34 years ago. When I was diagnosed with CF, my mom forgot that she had a son and instead only remembered that I had a disease.

Does that make her a bad mother? Absolutely not! She did the only thing she instinctually knew how to do as a mom - protect me. Her protection came in the form of shielding me from the outside world in fear that I would become "more sick".

If she would have continued parenting me like that, there is no doubt that I'd be dead.

My Life Changed

By the grace of God, at the age of 5 or so, my very imperfect mom with the very imperfect kid made the most perfect decision, to let me live. In fact, it was this quote by my doctor that started it all...

"If you continue doing what you are doing, not only will Ronnie be somewhat physically disabled his entire life, but he'll become mentally disabled as well...and that's far worse"

That's when my life changed.

She no longer parented the disease, she just started to parent Ronnie, her rambunctious son.

Mind you, this was still tough sledding for my mom. Not allowing the disease to make every decision for her didn't feel natural. There were many times where her heart and her head didn't agree. She had to practice parenting me apart from the disease. It was a decision that she chose to make every single day. That doesn't mean it was easy.

The fear was still there. The agony still hovered over her at times. The doubt crept into her mind from time to time. You know what though? She never let me see that. She would cry herself to sleep almost every night, something I didn't know until about 6 years ago. I never saw that.

After she popped the bubble she formed around me, the only mom I remember was the mom that told me to live, and live well. The mom who sacrificed her afternoons and weekends by having me in sports year round. The mom who made my health our families number one priority, while still be a fantastic mother to my two younger brothers. The mom who would come home physically and mentally exhausted from work, yet made sure to hand pound my chest, back and sides for 45 minutes like she had done before leaving for work in the morning. The mom who took a job that she was overqualified for, and not all that interested in, because it had the best medical insurance in the state. The mom who made me believe that if I put my health first, I was capable of anything. The mom who talked about when I would go to college/get married/make her a grandma, and never "if". The mom who made cystic fibrosis feel like such a small part of our lives, even though most of her energy went to managing it. The mom who raised a boy who was proud of having CF, because she made it clear that she was proud of me - the boy with brown hair, buck teeth, a loud voice, fast feet, quick wit, and oh yeah, cystic fibrosis.

I'm asked all of the time, "where does your attitude come from?". That's easy. My mom.

My Mom, The Perfect Mirror

My mom made me into the man I am today by using CF, and it's inevitable challenges, as another tool in her tool belt. She had me convinced that CF helped mold me into the boy that was the "apple of her eye". She always talked about what a blessing CF was to our family. She was quick to point out the opportunities that came along because of cystic fibrosis. She often reminded me of all of the great people I met and friends I had that would have never been a part of my life without CF.

I loved my life with CF, because my mom loved our life with CF.

Frankly, if she would have shown me that she hated CF, I would have thought that she hated me, too. If she would of shouted "CF sucks", I would have heard "our life sucks".

I never once got that feeling from my mom. I haven't heard her speak a bad word about CF to this day.

She perfectly modeled everything she wanted to see in her son and for that I am forever grateful. Like Bryan's parents, she made sure that a life with CF was a life full of living.

The only reason I'm able to write this blog 34 years after I was diagnosed with cystic fibrosis - with her granddaughter tucked safely in bed and my wife nestled up on the couch -  is because I had a mom who believed, from the bottom of her heart, that I would.

"I Lived" because of Christine. I love you mom.

Things That Make My Lungs Feel Worse: Strong Scents

Last week, I posted about things that make my lungs feel awesome. Actually, it started out as a blog of me talking about various things, but I ended up writing about exercise for so long that I just made it a blog focusing on one thing: Exercise. If you missed that blog, you can check it out here (you should check it out just for the memes I included). In the months to follow, I plan on writing blogs about other things that make for #awesomelungs, and if you'd like to be involved in that, please let me know!

Anyway, on to the topic at hand. Now, I've already pointed out the number one thing that I believe make my lungs feel awesome, so what about the number one thing that make them feel worse, or not so awesome? The most obvious answer is a lack of exercise or treatments, but I wanted to get a little more micro-focused (is that even a word??) with this blog and talk about one thing that others may not think about that could make their lungs, or the lungs of their child, feel worse.

That my friend, is a strong scent of pretty much any kind. (Maybe I should rephrase, a strong chemical scent of any kind, roast beef is okay) Now, notice I threw the word strong in there. This doesn't necessarily mean that just a little whiff of something fragrant in the air is okay for my lungs, but I can tell you unequivocally that the stronger the scent, the worse my lungs feel. What do I mean by worse? Usually tight. There are some scents, or scent dispersers, namely burning incense, that make my lungs feel like a deflated balloon that cannot take in air.

I do realize that the smoke burning from the incense stick could, and most likely is, contributing to the tight lungs, but I assure you that whatever is being burned into the air to create the smell is not helping.

Same goes for strong wall plug-ins, perfume, cologne and please listen if you fall into this category cigarette smoke on clothing.

**Steps on soap box**

I know, I get it, you smoke outside only. You have been addicted to nicotine since you were 14 and even though you now have a child with a terminal disease that primarily impacts the lungs, you still continue to smoke.

I won't address the fact that IMO it is an entirely selfish decision to continue to smoke when having a child, period. I won't even talk about how disrespectful it is to your child or the community they are a part of. Lastly, you're an adult, so you know the negative way that cigarettes impact your health, so no need to go there.

What I am going to address is this: If there is smoke anywhere present - car, house, clothing, mouth, skin, air, patio, etc - it can, and most likely is, harmful to your child. Please stop. I know it's hard, but so is watching you smoke. Your child may not feel disrespected now, but I promise, they will. They may not think your smoking is impacting their health negatively, but I promise, it is. Just stop. Please.

**Okay, give me a second to step off of the soap box...Alright, back on solid ground.**

Back to the rest of the scents and scent related crimes. Here is another one, if you are a respiratory therapist, please don't come into my room smelling like a bottle of perfume or cologne. I have to sit there and breathe it in for an hour. It doesn't feel nice. I'm glad you smell pretty, but your here to help make my lungs clear, not smell nice for the new doctor who just started working your floor.

I know, candles make you feel relaxed, but do you need to burn 41 of them around the house? I have a pretty strong candle detector and my lungs start tightening up around candle number five.

I like chlorine in my pool water, but pool water mixed in with my chlorine? Tights lungs here I come!

Okay, I can see this blog starting to become a diatribe of every scent I've ever encountered and what I think about it so I will stop. I'll just leave it at this, if you have to question whether or not the scent is too strong, then the scent is probably too strong. If you don't need it, don't use it.

And lastly, please stop smoking.

If you want to get involved in the conversation, leave a comment here about what make your lungs feel worse or tweet me @RunSickboyRun! I'd love to link to any blog you write about what can make your lungs feel worse, either now or over the years. Send me a link to your blog so I can share it!!

Can One Email Save Two Lives??

It has come to my attention that two girls in the cystic fibrosis community are in desperate need of our help. In this case, “help" can come in the form of an email from YOU and others in the CF community.

Here's what I know:
A couple of years ago Arkansas State Medicaid denied coverage of Kalydeco for four girls in need of the medication. Through the hard work and dedication of Beth Sufian (lawyer with CF herself) and her law partner, two of the girls were able to receive Kalydeco recently. Unfortunately, that still leaves two of the girls in Arkansas who are in desperate need of the medication. Their health is in decline, and time is limited. Action needs to be taken now!

The doctors of both girls have petitioned Vertex pharmaceuticals for a compassionate use of Kalydeco. The response to these pleas has been disappointing. Vertex’s response to the pleas was that they would meet with Arkansas State Medicaid. A meeting is good, but the medication would be better. A meeting will not stop the rapid decline in the health of these two girls. 

Vertex has the ability, under the law, to provide the girls with Kalydeco as early as tomorrow! The FDA allows for on-label compassionate use of any approved drug of which both of these girls qualify.

One can only imagine what these girls and their families must be thinking. The same family who participated in fundraising efforts that provided the money to accelerate the research for this drug, is now being denied access to it. Does that sound at all fair to you?

What YOU can do:
You can copy and paste the following text into the body of an email and to send it Charles Johnson, VP of Global Medical Affairs at Vertex. His email address is charles_johnson@vrtx.com. Let him know that this is unacceptable. Feel free to use any or all of the email provided or write your own. Please copy ronniesharpe@cox.net and info@cff.org to any email sent. 

***

Mr. Johnson - 

It saddens me to hear that Vertex is refusing to provide Kalydeco to the two girls in Arkansas; despite having the ability to provide it under compassionate use. As a community we have worked very faithfully for many years to raise funds for this very drug; for you. I understand you can’t help everyone who is denied coverage, but it would be nice to see you try. The CF community stands up and helps one another. We work tirelessly to raise these funds with the expectation they will be used to improve the health and lives of those living with cystic fibrosis. My hope is that you will serve this community like we serve each other. Thank you in advance for doing what is always right, not what is always profitable.

Sincerely, 

Your Name

***

Are you tapped into social media? You can also help on Facebook and Twitter!

Facebook:

Vertex’s 2014 second quarter financial results stated Vertex had $1.22 Billion dollars in cash.  The only drug Vertex sells is #Kalydeco. Yet they refuse to provide the drug to two girls in Arkansas who have been denied access through Medicaid, even though they can, according to law. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

Two girls in Arkansas whose health is declining are being denied coverage by Medicaid for #Kalydeco, a drug which could help them drastically. Legally, #Vertex can provide compassionate use of #Kalydeco while their legal case continues. Yet they are doing nothing to help. Help these girls. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

It’s important for a company to do the right thing. #Vertex is not in the case of two girls denied coverage for #Kalydeco by Medicaid. Show your support for fellow Cysters and their families. http://ow.ly/A5NCi

#Fighttheinjustice #supplydontdeny

Doing what’s right is always more important than doing what’s profitable. http://ow.ly/A5NCi #Fighttheinjustice #supplydontdeny

Twitter:

@vertexpharma Doing what’s right is always more important than doing what’s profitable. http://ow.ly/A5NCi #Fighttheinjustice #supplydontdeny #Kalydeco

@vertexpharma How can a company with $1.22 billion dollars in cash not provide the compassionate use of #Kalydeco? #cysticfibrosis http://ow.ly/A5NCi

@vertexpharma The Arkansas cysters need #Kalydeco now, not a meeting! http://ow.ly/A5NCi #supplydontdeny #cysticfibrosis

I can't thank you enough for doing this and taking time out of your busy day to help these girls.

You can read more about the girl's case here: http://online.wsj.com/articles/costly-drug-vertex-is-denied-and-medicaid-patients-sue-1405564205

Things That Make My Lungs Feel Awesome: Exercise

PSA: Video games will not make your lungs feel awesome.

As an "old man" with CF, I've figured out over the years what things I can do to make my lungs feel awesome and what things I can do, or not do, to make them feel eh, not so much. I realize that, as with everything with CF, some of these things will work for others while some may not. Here's what I do know, nothing changes unless something changes. So, if you see something in the coming weeks that you may want to try, give it a shot. A real shot. A real honest shot. I used to try and take shortcuts with my lungs when I was younger and it took me many years, and lots of pain, to realize that there are no shortcuts when it comes to health. Being at our best health-wise comes down to two things - good decisions and hard work.

So with that, here is probably the #1 thing that I have found make my lungs feel awesome...

Exercise.

You probably saw me going there. We've all heard it and we probably hear it quite a bit. But what exercise exactly? Well, the short answer is to do any exercise that you'll commit to and do consistently. For me, that has been High Intensity Interval Training (HIIT). According to the ever so wise Wikipedia, HIIT is "a form of cardiovascular exercise. Usual HIIT sessions may vary from 4–30 minutes. These short, intense workouts provide improved athletic capacity and condition, improved glucose metabolism, and improved fat burning." Did you catch that? Improved athletic capacity (ie better shape, ie in general, better lungs) and improved glucose metabolism. For those of us in the CF community, that's a win-win. Now, some of you read "improved fat burning" and thought, "My doc says I cannot lose any weight!!" and therefore dismissed HIIT as an option for you. Can HIIT promote weight loss? Yup. Does it have to? Nope. If putting on weight is a struggle for you, you can safely do HIIT but you must increase your calories. You must eat before your workout, to give your body the energy to carry you through the workout (and not burn your fat in the process) and you must eat after your workout (to aid in the recovery process and not allow your body to use its fat stores). It absolutely can be done. And, if you want an even better shot at maintaining, or even gaining weight after including some HIIT into your routine, may I suggest that you add a weight lifting routine to your schedule as well.  Again, you must eat right to take full advantage of your hard work, but it can be done! 

And back to the lungs, HIIT (or other cardio exercise) is like doing an albutertol treatment at the gym. I was actually in a study that showed this was the case. Exercise opened up the airways, and kept them open for an extended period of time. Plus, doesn't it just come back to the old tried and true phrase "if you don't use it, you lose it"? If you don't use your lung (function), you'll lose your lung function. I like to think of my lungs as a muscle I'm working out just as I would workout my chest, biceps or legs. Instead of lifting weights to work out my lungs, I'm taking deep breaths. I'm coughing. I'm pulling for air (in a good way of course!).

The hardest thing about exercise is getting starting. Most people I talk to (CF or not) don't exercise for two main reasons: time and knowledge.

This just made me laugh.

I don't think I've ever met a person who thinks they have a lot of time to spare. It's in our human nature to always feel busy...even if we're not. The bottom line is we all make time for things that are important to us. I have not been writing in this blog consistently for much of 2014. Reason? I have felt like I've had no time to devote to writing. I've felt pulled in a million different directions and didn't think I had any time to spend on RSBR. The truth, I have been busy, but I've been devoting my time to things that I either felt were more important, have the potential to have a greater impact on the CF community or things that I enjoy more. So I've had plenty of time, it's just been focused on other things. (Note: One of those is exercise which I devote about 2 hours a day to). To make time for exercise, it must be important to you. You need to find some kind of external or internal motivation for doing it and doing it consistently. If I gave you 1 million dollars to exercise daily, would you do it? Of course! You'd make the time no matter what! Unfortunately, I can't pay you to exercise (I wish I could), but I would argue that better health is worth a lot more than 1 million dollars (to me anyhow!!).

Ask Unknown Cystic if he thinks it was worth making the sacrifice to exercise every day. He works A LOT and didn't think he'd have anytime to fit exercise into his life. What did he do? He got creative! I believe they call that "if there's a will there's a way". He decided that if he was going to be working, then he'd be walking too. Check out the results he's had after walking on a treadmill during his working hours for the past year: One Year of Using a Treadmill Desk. The proof is in the pudding.

I understand that many of us can't afford a treadmill in our home, but don't let that be your reason for dismissing his story. The point isn't that we all must walk while we're working, the point is that we all must just do something. Commit. To. ANYTHING. Active.

Forgive me if I'm making it sound too easy, but in reality, it can be. It's just a matter of making it a priority and doing it. Don't have 1 hour a day, how about 10 minutes? Is 10 minutes an optimal amount of time for exercise? Nope. Is it more optimal than 0 minutes? Absolutely!

How can you get started? I'm glad you asked. Pick a time today that you're going to do something active and commit to doing it. Reading this really late at night? Pick a time tomorrow. How much time? At least 10 minutes and no more than 45 minutes. What's active? Anything that makes it harder for you to breathe as a result of exerting effort. That could be from sitting up and down on your couch multiple times to running 5 miles. Whatever it is, if you did nothing so far today that could be considered active, you'll already be two-steps of today, tomorrow (or later today!). 

This is all about improving yourself. Not being better than anyone else, just a better version of yourself. You can do it, just take the first step...of many, many steps.

Need help? Just ask. You can leave a comment here or find me on CysticLife.

If you want to get involved in the conversation, use #awesomelungs on Twitter and tweet @ me yo!! I'd love to link to any blog you write about what you do to make your lungs feel awesome. Send me a link to your blog so I can share it!!

I won't guarantee that it will be fun, but it will be worth it!!

Enlightening Conversation About Vertex Results

I thought it would be good to share a conversation I had today with a momma in the community that wasn't exactly pleased with my thoughts/comments/commentary on the Vertex announcement yesterday. Many of you may feel the same way...

"I am upset at the comments you made yesterday and the one today."

What specifically upset you? (see paragraph towards the end of this post for her response to this question)

"I am surprised that you would post things that could upset parents and other CF'ERS that have new hope. I understand that you have strong feelings and emotions about this combo and they are rightfully yours to have."

Right. And I'll always be honest, even if that means a few feathers are ruffled or I have some push back. And if posting something accurate and truthful on FB takes away the hope of some, I am sorry. With that said, I also included parts in my statement that would support the hope felt like "There are also people who blew the doors off of old PFT results!!!!!" or "I'm not saying that this is not good news." or "And I've always been consistent in saying that we do not need to see positive numbers for it to be a success. Remaining steady would be a BIG WIN for the CF community."

"However, the post yesterday was used by others to continue to be hateful to many in the community."

I cannot control what others do, and I will never censor myself based upon the possibility of others using my words out of context. That would not be authentic and I certainly won't put on a false facade - with what I say or what I don't.

"It may not be what you intended but it came across as you being mad that you were not able to get them at a time when you were younger."

Honestly, I have no clue how that came across to you in what I said. Do you think I would volunteer 1000's of hours a year in the CF community, if I were mad or bitter in any way at the opportunities afforded to the younger generation? Does that sound like a guy who thinks "life isn't fair"? I thought that was pretty clear when I said, "My biggest goal, and frankly why I got so involved with the CF community, is to make the life experience of the next generation of CFers, and the generation after that, better than my own. I don't want to see the CF patients of the 90's, 00's and 10's experience the same struggles as I did, a patient of the 80's."

"I have always respected you and seen you as such a godly man. (I even spoke of how you were such to my mother yesterday when everyone else was posting hateful comments)."

I'm certainly happy to hear that you think (or thought?) of me in that way. I do my best to show God's love through my approach and interaction with others, and unfortunately, I'm pretty sure I fail everyday. I can assure you if I served a God that wasn't gracious, I'd be screwed.

"To see your post after saying these things was disappointing. I hope that you see that your posts can be hurtful and truly upsetting to some."

Again, I am sorry if anyone was hurt by my commentary yesterday. This was in direct response to seeing statements like "I'll finally be able to breathe again" or "This may be my last hospital stay ever!!" or "We'll basically be cured." or "I can't wait to take those pills and for this to all be over!". We still have a long way to go. As it stands right now, the combo drugs will not support the statements above for the majority of people with CF. Less exacerbations? Probably. Less frequent hospital stays? Probably. Maintain lung function for longer? Probably. Cure? No. No hospital stays ever again? Probably not. No more treatments? Probably not.

"You have been given a wonderful platform to be such a strong witness for the Lord. I am always so thankful for your standing up for what is right and true."

I will continue to do so.

"Please know that this is not intended to be mean, just expressing how this has made one Christian woman feel."

Not a problem. I didn't think it was mean at all. It's hard to articulate feelings through an online medium such as FB. Expressing oneself, and having different feelings towards the same object/result/person is what personal expression is all about.

"I want this combo to give you decades more to work for the Lord and be with your family as much as I want it for my little girl."

Amen. I want the combo to work for your little girl with every fiber of my being. I would gladly give up any potential decades I have left to see that happen.

"On a side note, the lawsuit against Vertex being brought forward by the state of MA makes me question the validity considering their health care system. Just a thought."

The lawsuit is about people purchasing the stock based on "grossly overstated" results by said company and then losing 100's of thousands of dollars when that information was redacted...all the while executives of said company potentially made 100's of thousands of dollars by selling a portion of their shares before the results were corrected.

"I think the most upsetting part was saying I hate to be that guy type of comment. It kind of starts you off with a negative attitude. I did not see the comments from people that you mentioned. I do not think this is the end of treatments or anything for my daughter. But I do think it could keep her where she is until something better comes along which is more than I've had before. I know that most parents feel hope but know this is not a cure. For those of us who have a realistic view of the combo, the post was like someone coming into the first glimmer of hope we've had and screaming "just kidding!". Because we look up to you so much....I don't think you are bitter and that's why I even said something because it seemed out of character. I know you can't control what others do; however, if you had read what some were saying you might have held off on.that comment for a few days. It's ugly out there...and it just gets worse. It's hard for a parent of a child with CF that deals so much to see the negativity we've seen from this announcement. There are so many that have been so hateful about this not helping them that they don't want anyone else to have hope. It seems like such a miserable life for them and it's hard to understand. Your posts may not have upset me on another day but after dealing with all the negativity it was hard to see yours. I do not look at you differently now. I just wanted to let you know how it is from a mommy's view right now. There was such excitement for Kalydeco and we can't have the same joy that's all we want. I know you want that too. Thanks for responding! And I want address the other comments on the lost because I don't feel it's okay to stir the pot. But feel free to share this without my name." 

Totally understand where you are coming from [withheld]. I can see why this may have felt like I was piling on. It was never intended to be that way. I was trying to provide some history and context to a community that has felt let down many times in the past based on announcements like this only to discover it's not what they thought it would be. I'm sure I could have worded it better or, as you suggested, wait for the flames to die down a bit before throwing some gasoline on them.

On a side note: Did you know that I am currently on the drug and I think it helps? Maybe that would have been beneficial for me to say?

"I had no idea. I am thankful you are and are doing well on it! I hope it keeps you healthy and at home with your family more so than not! It would probably put out the fire if you said that ;)"

Haha, maybe I'll mention it then. I didn't see PFT improvement, but I think it gave me the ability to fight off infection quicker and I have remained stable for longer periods of time.

Proceed with Caution - Vertex's Big News

For those of you who haven't heard, Vertex announced the results of it's big Phase III combo drug that may cover roughly 50% of the community. You can read the press release here.

I'm so pumped to see the joy and hope exhibited by so many in the CF community today. I hate to "be that guy", but I did want to throw out a bit of context and history to be sure that we are all managing our expectations:

1. The Phase 3 study showed an average increase in lung function of 3% Context: Cayston showed a 2% increase in it's Phase 3 head-to-head study against TIS.

2. The results have not been completely vetted by those outside of Vertex. Context: This is the same company that is now being sued for "grossly overstated" results in the Phase 2 trial of the combo drugs - http://ow.ly/yp7L2

3. About 46 people dropped out of the Phase 3 study because of complications. Context: Extrapolate this to the potential users of this combo, and you'd have around 1400 people with DDF508 who still wouldn't benefit from this drug. You'd also have to include those who saw no benefit but elected to stay in the trial. (NOTE: There are also people who blew the doors off of old PFT results!!!!!)

I could go on, but I'm feeling like Captain Buzzkill. I just see a lot of celebrating without facts (or misinformation) and want peeps out there to understand that we have a long way to go for the next generation of CFers. With that said, if this drug has the potential to in essence halt the decline in lung function at an early age, I'd cut off my left arm if that meant that everyone had access to it.

#captainbuzzkill

Added after reading feedback: Yes, celebrate!! Any move towards a better life experience while managing cystic fibrosis is a win. I'm not saying that this is not good news. Selfishly, anything that could possibly extend my life even a minute with my wife and daughter, is something worth being excited about. My biggest goal, and frankly why I got so involved with the CF community, is to make the life experience of the next generation of CFers, and the generation after that, better than my own. I don't want to see the CF patients of the 90's, 00's and 10's experience the same struggles as I did, a patient of the 80's. Progress doesn't only come in the form of a pill, but I'll sure as heck take one if/when it does!

And I've always been consistent in saying that we do not need to see positive numbers for it to be a success. Remaining steady would be a BIG WIN for the CF community. Shoot, not having to work so hard to maintain what I currently have is something I'd pay good money for :)

(I took this from my FB page as I know many of you are not friends of mine on there yet you read this blog. I'd love to hear your thoughts)

Limited Exercise Research

I thought this was a very interesting article highlighting the limited research on exercise. I've highlighted the biggest take-aways if you don't have the time to read the whole thing.

“A lot of physiologists come into the discipline because they fundamentally like exercise,” Martin Gibala, an exercise physiologist at McMaster University in Ontario, told me. “But you learn very quickly that there’s not a lot of research money out there to fund applied studies.” On matters as simple as how many sets and reps best promote muscle growth, Mr. Gibala explained, “We can’t nail down the answer.”
Even if the funding were there, Mr. Gibala says, “That’s not state-of-the-art research that you’re going to publish in the best journals and advance your career.” Instead, he says, physiologists study questions of basic science, “like the molecular signaling proteins that regulate skeletal muscle adaptation.”

I also thought of the CF community and what we're trying to do in promoting patient-centered or patient-driven research when I read this...

The human body is an adaptation machine. If you force it to do something a little harder than it has had to do recently, it will respond — afterward, while you rest — by changing enough to be able to do that new hard task more comfortably next time. This is known as the progressive overload principle. All athletic training involves manipulating that principle through small, steady increases in weight, speed, distance or whatever.
So if your own exercise routine hasn’t brought the changes you’d like, and if you share my vulnerability to anything that sounds like science, remember: If you pay too much attention to stories about exercise research, you’ll stay bewildered; but if you trust the practical knowledge of established athletic cultures, and keep your eye on the progressive overload principle, you will reach a state of clarity.

I think we all see people in the CF community that are doing the things and making the choices that work for them. In some cases, there is a large cohort of the CF community that has bought into a certain lifestyle or treatment or "culture" that seems to be having positive effects. I look forward to identifying those a doing what we can to promote research around those areas.

If you have any comments, I'd love to hear them.

http://www.nytimes.com/2014/05/25/opinion/sunday/fitness-crazed.html?_r=1

The Choices We Make

When I made the decision to come to the hospital, it was not an easy one. The way I was feeling, my energy level, my increased cough, my increased mucus production, my lung pain and my lung function test results all pointed to an easy decision, but it was certainly not so.

Mandi and I had been planning on a trip to South Carolina with my father-in-law and mother-in-law and family friends for months. The tickets had been purchased the schedules rearranged and we were ready to go. That "we" is now just Mandi and Mckenna. They left on a plane for South Carolina this morning.

It's funny as it seems every time there is a decision whether or not to come into the hospital, there is always a balancing act. I have to decide how to weigh the perceived sacrifices with the hopeful
outcomes. And really, the hopeful outcome is the only reason I come into the hospital. If I didn't have that hope that I could and would get better there be no reason for me to be in here. Thankfully I still have that hope, and have never lost it. When I come in for a tune-up, I do get better. I do become the best version of myself. It make take 2 weeks, 3 weeks or more, but it does happen.

So what led to this decision? The easy answer is that my lungs felt beat up. I was unable to exercise like I'm used to. I found myself getting winded after a flight of stairs. It was hard to take a deep breath because of the pain that I experienced each time. With me, it becomes a vicious cycle. Less than effective workouts contribute to more mucus buildup which contributes to more inflammation which contributes to a harder time breathing. It's up to me to break that cycle. Sometimes I can do it through increased treatments or increased exercise, but other times it takes the decision to come in for a tuneup.

When I went to clinic and talked it over with the team they thought it was a pretty good idea that I come in. They knew about my looming trip to South Carolina and told me that I would have their full support if I decided to try to hang on and make the trip. We all knew that the decision to come into the hospital would mean that I was choosing not to go to South Carolina but to go into the Hole. It was not an easy decision, but there is no doubt that there was a right one. I had to put my health first.

I don't have many regrets in life, but as it relates to CF, I have one big one. There was a time in my life when health certainly did not come first. My social life came first. My work came first. My girlfriend was my priority. My friends won out over my treatments. Delaying hospital stays to go on vacation was the norm.

And after years and years of making those decisions and putting everything else in front of my health, I am now paying for it.

I have to work so hard now to stay healthy. I have to put in so much time each day to keep my energy at a level that my wife my daughter deserve. So much time is spent doing my treatments or at the gym that I wish it could be spent at the park with my daughter. I now have to miss family vacations to come into the hospital.

If I would've kept my health first, as the first priority in my life, would I have to make the sacrifices now? Of course I do not know the answer for sure, but I'd be willing to bet that I wouldn't have to work so hard now. I wouldn't have to take so much time away from my family when life is really important. I probably wouldn't be missing this trip to South Carolina.

That's the bed I made and now I lay down in it.

So what's the point? If you're reading this and you're struggling with putting your health first, I beg of you, don't make the same mistakes that I did. There will come a time in your life that you will wish you didn't have to work so hard because of choices in the past. When we were younger many of us thought that we were just “living life”. Let me tell you as a man with a wife and a daughter - This is life. I'm now in a life that matters. I have two beautiful girls who count on me to be at my best. That doesn't mean that if you don't have a family in the future you won't be living “the life”. But I promise you that there is something down the road worth sacrificing for. The truth of the matter is you're going to have to work hard now or work hard later, but hard work and sacrifice will happen.

I encourage you to make that sacrifice now, and to work hard now, at the chance that you won't have to work so hard later.

My Torn Pleural Adhesion

Mandi mentioned some of the things that may have contributed to my current hospital stay on a blog a few blogs ago, so I figured I would talk about one of them.

It was the night of April 3rd and somewhere around 3 AM I felt a slight tug and some pain in my left lung. Now, I have had similar feelings before so I wasn't too worried and I took some deep breaths thinking it would pass. The pain did not pass but it didn't really get worse so I figured I would just sleep it off and address it in the morning. About 3 hours later the pain had intensified to a pain I had not felt up until that point in my life (in my lungs).

When I rolled out of bed I had to walk with a hunch as it was the kind of pain that almost brought me to my knees. I made it to our couch in the living room and I just sat there rubbing the area trying to take deep breaths thinking again that hopefully it was something I could breathe through. The pain only got worse. I was unable to take very deep breaths and when I did the air seemed to get forced out of my lungs. I was unable to speak properly and I would make more jumbled moans than actual words. It was the first time this kind of pain with these kind of symptoms had ever occurred.

Mandi took one look at me sitting on the couch writhing in pain and said "we're going to the ER”. Usually I would fight her on something like this, thinking that I could fight it on my own or that it would pass shortly–I put up no such fight. She grabbed me some clothes got Mckenna together and out the door we went.

We made our way to the biggest hospital within striking distance and Mandi dropped me off in front of the ER entrance. There was no receptionist present as I clung on to a sign which was assisting me in keeping me up right. A security guard noticed this and rushed to get me a wheelchair to sit in. He then went back into a different room to grab some assistance. Just then Mandi and Mckenna came through the doors, which was good timing, because I was having trouble having enough air to answer the nurses questions (I was later told they figured I had a massive kidney stone). I of course told them about cystic fibrosis and that I've had similar pain before but not to this level. I shared with them that if I had to guess what a pneumothorax or a popped lung felt like, this would be it.

They brought me back to a bed and immediately administered an albuterol treatment and oxygen. They then took me back to x-ray and I also had a CAT scan performed. This was my first time at this hospital so they had no record of me or any x-ray images or CAT scan results from past. They reached out to one of my pulmonologists in Tucson to compare notes and results of the different tests. Fortunately, as time started to pass the pain started to subside.

They concluded that I must of torn a pleural adhesion. Which in layman's terms, which of course I asked for, in my case means that they felt I ripped some scar tissue in my lung. I had never even thought of this being a possibility. I mean it intuitively makes sense, and I understand that you can of course rip scar tissue, I just never thought of it actually happening. I know that I have scar tissue all over both lungs and I'm surprised I guess that it took this long to happen. Being as active as I am and using as my lungs as much as I do I feel blessed that I've avoided it up until this point. I'm also very thankful that it did last, that is the pain, “only” 5 hours or so. I mean I had some residual pain for the days that followed but nowhere near the initial pain that I felt.

It was suggested that I should do some exercises that would stretch the cavity wall and that I didn't do anything too aggressive for at least a week or so. They also suggested that I get these “lung stretches” into my daily or weekly routine to try and avoid another torn pleural adhesion in the future. I did have a cold the week before this happened and am wondering if that contributed to this event as well.

I also think this contributed to my downward trend over the course of the following month. A downward trend which ultimately led to me being where I am today, the Hole. It wasn't the whole story however--there were other factors that led to me being in here which I will get into in later blogs.

Has anybody else out there ever been told they tore a pleural adhesion? I'd like to hear your story!

Questions from a Reader: No Silly Questions!

A couple questions sent my way that may help others out there...

Good night, Ronnie, 
I'm really very impressed by your great work about CF community. It's amazing and very inspiring. I wrote that I'm from Russia and now I live in Portugal. Also I've been in German clinic with my child and read a lot on CF forums in England. I spoke with many doctors and physiotherapists everywhere. But I have to say that your community, your actions, your positivity are great and unique.
I'm a little bit shy to ask some questions on the board as the answers seem to be very easy for most of the community here. But still I have some. And I decided to ask you. I'm sorry.
What is PFT? The level of oxigen in the lungs? That the doctors usually measure with a special clip on the finger?What is FEV?When do your doctors prescribe IV? I've read tons of information about that. But still i'm puzzled as all the cases are very individual and it seems that doctors have different point of view. Is there some "rule" or everything depends on the patient/symptoms?Do the doctors prescribe IV as prophylaxis without bacterias and symptoms?Do your doctors prescribe ANY antibiotics as prophylaxis without bacterias or/and symptoms? 

I understand that the questions are too silly and you might not have had any time to answer. I'm sorry.

First, thank you for your kind words. I'm honored to do anything I'm able to do for the community.

I'll answer your questions to the best of my ability...

PFT explained: http://ihavecfsowhat.blogspot.com/2012/03/awesome-pfts.html

The special clip on the finger measures oxygen saturation explained here: http://www.health.harvard.edu/diagnostic-tests/oxygen-saturation-test.htm

FEV1 is covered in the blog I provided above.

There is no rule when prescribing IVs or oral antibiotics. As you get into adulthood, much of it is a team decision. For me, I request IVs when I can no longer sustain my exercise at level consistent with me being healthy. When I was younger, we would base much of it on how I felt and how my PFTs were.

Yes, there are some doctors who are more proactive and will give antibiotics at the first sign of infections. Some will also prescribe with no sign of infection but other symptoms.

I'm on Azithromycin all of the time, it is an oral antibiotic. I also inhale either Cayston or Gentamicin each month and both are antibiotics.

Hope this helps! Let me know if you have any other questions. 

Running Rehab: An Unscientific Experiment

Guest post by Kay

After being diagnosed with CF at age 21...I had spent the better part of my life telling myself that I was an exception to the rule, that my case of CF was mild and falsely thought there were probably no others with the disease that were doing so well. Sure, I had my moments during exacerbations when I worried and wondered if I’d get to see my daughter grow up...but for the most part I did as I pleased and was hospitalized on average once a year. I’d been a runner since h.s. track and when my daughter was young I had a sign taped to my alarm clock that read “RUN OR DIE” so I’d get up and run before her Dad went to work. But over the years my FEV1 had inched down slowly and I’d left the world of the moderately affected and dipped under the 50% FEV1 level and into the world of the severely affected. Life got harder. It became difficult to jog. I’d get so winded after just a quarter mile jog that I took to doing a jog/ walk type of workout and even that was so hard I began to skip more and more days. My daughter had asked me to run a 5k at a 1/2 marathon she and her dad were doing and when I told her I couldn’t even run a mile she gave me a bit of a hard time saying “anyone can run a mile...you’re just using CF as an excuse”. Ugg. So a week later when my nurse coordinator forwarded a Jerry Cahill video where he was running with oxygen...I was all over it!

I contacted Jerry through Facebook and he told me about using a wrist oximeter and putting the “tank” into a camelback backpack for jogging. I asked my Doctor who always had a witty sense of humor if I used oxygen for exercise if I would become oxygen dependent...his reply “we are all oxygen dependent” haha. But “no, it won’t mean you’ll need to supplement O2 for everyday things”. It took a few weeks and a little experimenting, first with liquid oxygen (not good it can’t take the jarring of the jogging and all the O2 would expel after just a short distance). I exchanged 3 back packs until I got one that had more padding and was comfortable to carry the M6 tank. And then I got in a routine...quickly running 3 and 4 miles at a time. I decided to train for the first annual “Run to Breathe” that Jerry was organizing for BEF in Central Park and invited my daughter to join me. Jerry provided encouragement to the jogging posts I would make on Facebook and we joked about being the last ones on the 10k course. Raceday came and the adrenaline and crowds had me jogging at a pace much faster than home. Central Park is relatively flat in comparison to the Fox River Valley where I trained. I was pleased with the time and my daughter and I had a fabulous 4 day trip to NYC.

Back home from the trip and I wasn’t feeling particularly well. When I went to the clinic I had the worst PFT results of my 49 year old life with Cystic Fibrosis. FEV1 was 36%. It scared me. Badly. I had been getting to know other CFers through social media and a few were on the transplant list. One was at 30% FEV1 so a personal score of 36% was alarming. I did not understand it. I’d been running about 20 miles a week and instead of getting better I was getting worse. I’d lost a lot of weight which had never been a problem as I am pancreas sufficient. But with all that running I could not eat enough. I got a tune up and recovered to 42% where I stayed for a couple years.

Chicago winters can be rough and long. This one has been the worst in my memory with temps dipping down to -18 degrees F at times. I was sick in October, choosing to do a 21 day tune up, and then mid December a bug that was going around at work settled in which took me six weeks to get over with Cipro and Prednesone. January rolled around and I still wasn’t myself and I looked at the option of IVs again and turned around and literally ran in the other direction. I decided to hire a running coach and see if having someone with expertise in building a program could help motivate me. I’d been reading the journey about those pre and post transplant go through with a mandatory “rehab” period both before and after. So Coach GP and I embarked upon a Running Rehab program for these old CF lungs of mine. He said “I’m going to be in your pocket”. By that he meant he would hold me accountable for the workouts and text me and ask me what I’d done each day. Coach is in the NYC area and travels a lot, so I’ve never met him...but working virtually worked very well. We spent a couple weeks seeing what I could do and just getting used to the idea. It was all on the treadmill as cold air is not my friend. My knees hurt, I was tired...but determined. Then he gave me regimented workouts that included interval training and progressive runs. I chose to just “do as I was told”. It made things simpler to just be assigned a workout and do it and not have to make a daily decision on what to do and how far to go. Wimping out was not an option. I let him be the boss of my schedule. The schedule was tough as I work two jobs but I fit it in most days, sometimes getting to the gym at 8pm, sometimes awakening at 5am to fit it in. A month went by and my knees no longer hurt. One day I started running without hooking up to the O2 tank (just forgetfulness) and thought, wow, my O2 sat seems ok...lets see how far I can go without it. I made it a full mile before the O2 sats dropped below 90%. Psyched!!! I hooked up for the rest of the workout and considered that bit of un-supplemented running true progress! Days that followed when I tried it again did not fair so well only making it to 1/4 mile before I needed O2. Every day is different, I learned. Storm and weather shifts affected my lungs, stress at work affected my lungs, but I started to see that pushing myself to run on the worst of those days ALWAYS had me feeling better after the run.

Then, the day came around I’d been training for. The clinic appointment. The day before I’d come home from work to do treatments and had a terrible coughing fit that lasted 30 minutes. It wore me out and my lungs reacted by tightening up. I thought ugg, I’m going to do terrible on my PFTs tomorrow. The next day arrived and I jumped a train and headed down to the city for my appointment. My lungs felt really tight and my expectation was that I may need prednisone to open them up again and if my FEV1 was low...I might even have to do IVs. Ugg. But, my first blow into the tube....showed 52%. Wow. Even though I wasn’t feeling particularly well, my airways were far more functional than before the running rehab. I’m optimistic my score would have been higher if not for those storms rolling in and putting me in a funk.

So, “what have you learned...from the past month Miss K?”, Coach asked. “I have good days and bad days” I said. He said “you respond well to multi tier training on many levels but it has to be very carefully measured between both not overdoing it and undergoing it”. It had become apparent that just running long, slow runs and adding more and more miles as I had done previously was not the right approach. My current program has me running a mix of intervals and progressive runs Tu, Th, Sat.  On MWF & Su I am doing weights and some cross training. I plan on working out 7 days a week, knowing I may miss one as I listen to my body and may need to take a day of rest when muscles and resolve are fatigued. Running more and running longer didn’t seem to pay off the way varied running has and please note: it’s also way less boring :-)

I’d recommend if you’re having trouble kick starting yourself to find a coach to work with. Athlete training programs used for healthy folks can also do great things for those of us with CF. A coach that can understand that there are some limitations but also not be afraid to push may be just the thing to get you started in the right direction. Exercise IS medicine!

Does CF Consume Your Life?

Do you feel like CF consumes your life?

I get asked quite a bit if it feels like CF consumes my life since I do so many treatments and make sure that I exercise daily. I of course don't feel like my life is all about CF, but I thought it would be handy to put it into numbers to see if I could make it more clear. So, for this little exercise, I'll take 2012 vs. 2008. I'm taking these two years because they seem to be the average year before and after I got serious, or better put, "re-serious" about my health.

2008 in hours
Treatments: 547
Exercise: 182
Hospital Stays: 1680
Total hours devoted to cystic fibrosis: 2409 or 28% of all available hours

2012 in hours

Treatments: 1095

Exercise: 547

Hospital Stays: 720

Total hours devoted to cystic fibrosis: 2362 or 26% of all available hours

So when you look at those numbers, it looks like a wash right? I'm still devoting basically the same number of hours per year towards "taking care of business" when it comes to CF. However, looking deep into the numbers you'll see that in 2012 I am spent double the amount of time doing treatments, triple the amount of time exercising but less than half of the time in the hospital when compared to 2008. I don't know about you, but I would MUCH rather be doing treatments and exercising vs. being in the Hole. 

There are a couple more important numbers however that need to be thrown into this equation:

2008 lung function

Highest high: 79% FVC 68% FEV1

Lowest low: 64% FVC 54% FEV1

2012 lung function

Highest high: 87% FVC 74% FEV1

Lowest low: 78% FVC 63% FEV1

**It should also be noted that

a) I started a new predicted model in 2009 that would have actually had my 2012 numbers even higher compared to my 2008 numbers and

b) Since "on average" "they" say that "lung functions declines by 2% per year", my net gain is actually even higher!

Now, CF is definitely more about lung function and those numbers don't always correlate with how I feel. I can tell you this without a doubt though, if I had to quantify how I felt in 2012 vs. how I felt in 2008, it wouldn't even be close.

In 2012 I could...

breathe better.

laugh longer.

run further.

sleep sounder.

expect a brighter future.

So if 2012 was an average year of awesomeness, 2008 wouldn't have even of registered on the same scale.

We all know that cystic fibrosis is about more than just numbers. But sometimes, numbers can help make things more clear. By looking at the numbers above, it's clear that the more I did for my health, the more my health did for me. Sure, a quarter of one's life devoted to anything seems like a lot, but I can tell you this, I've enjoyed the other 75% of my life a whole lot more in 2012 than I did back in 2008.