My New Treatment: O to the 2

I recently started a new treatment that was probably long overdue and needed for quite some time. At the request of our brand new adult CF doctor, I did a 6 minute walk test before I was discharged from my last hospital stay (May). She wanted to rule out anything funky as to why I wasn't recovering quite as quickly as I did before, and the 6 minute walk test was one of the tests she ordered to get more information on my health status. It was a good thing she did.

Leading into the test, I informed them that I would really push it because that's exactly what I do in the gym. I didn't just want to casually walk to see how my body would respond. I wanted to really put my body under some stress to see how it would respond. I walked pretty much as fast as my short little legs would take me. In 6 minutes, I walked .4169 miles, but by oxygen saturation went from a baseline of 93% (which was actually a little lower than normal) to 82% about 3 minutes into the walk. I required 4 liters of oxygen to keep my o2 saturation above the "acceptable" number of 88%. In short, I failed the test.

I was far from disappointed. In 2009, I did an overnight sleep study that showed my oxygen saturation dipping to the lower 80's when I slept. With that info, I was prescribed an oxygen concentrator to use at night with 2 liters of oxygen flowing into me as I slept. 2009 was probably the first year in over a decade that I didn't wake up with a headache and actually felt rested in the morning. Why did I put up with sluggish mornings and headaches for a decade? Only thing I can think of is ignorance. At the time, I don't know that I even recognized oxygen as a treatment option while I slept. Starting oxygen overnight was an absolute life changer for me.

So, with that in mind, I had high hopes for oxygen supplementation during my workouts. I certainly was looking forward to (hoping for?) getting an extra boost during my workouts and not experiencing fatigue so quickly.

After setting up everything with home health, I received 6 mini oxygen tanks and a shoulder bag to carry the bottle around. I am supposed to use 2 to 4 liters depending on the vigorousness of the exercise. Based on my needs and consumption, I am allowed up to 22 bottles a month. (So far, that's been plenty). The first change I made was exchanging the shoulder bag for a backpack. Walking around the gym with a shoulder bag was pretty inconvenient and the backpack has proven to be a valuable upgrade.

So, have I noticed any positive effects from the oxygen supplementation at the gym? Honestly, nothing stands out, but it is noticeable. I'd say the most obvious impact is recovery time. I am certainly able to "catch my breath" faster and resume my work out. There may be a bit more muscle endurance (especially for big muscle groups like legs), but I'm not totally convinced of that. Even with the minimal noticeable effects, it's still be a great decision to start the oxygen supplementation at the gym. We of course all know about the stress that the body, especially the heart, goes through when it's deprived of oxygen, so there's no doubt that is something that I'd like to avoid.

There are potential negatives for some of you out there if you're deciding whether oxygen supplementation during exercise is right for you.

You have to keep up on your oxygen orders. This is just one more phone call to make and schedule to coordinate among the many we are already doing.

It's another potential insurance battle. I ended up having to do another 6 minute walk test that was documented in a different way (failed that one also) and my doctor had to write a special letter justifying it's need and make multiple phone calls before it would be paid for.

One more thing in your already full arms. I'm quite a sight walking into the gym. I usually have one (or two) kids in my arms, my water bottle, phone, headphones around my neck, and now oxygen backpack on. I completely realize that this is a first world problem ;)

You'll get strange looks. I'm convinced that most people at my gym believe that I'm doing some sort of altitude training. For those at the gym that have the courage to actually ask what's going on however, it's a great opportunity to introduce the world of CF to them.

Conclusion: None of the potential negatives above outweigh the potential positives for me when it comes to oxygen supplementation while exercising. I believe it's been a good decision for me and one I believe that all who see a drop in their oxygen saturation while exercising should at least consider.

**As a bonus, I don't get strange looks anymore when I cough. I mean, the guy on oxygen has to cough right? As I said above, I get strange looks, but they have more to do with a dude under 70 wearing oxygen. Double bonus - I don't think people look at me as the SARS guy or Captain Contagious like they use to when I cough my head off...although in a weird way, I kind of enjoyed that ;)

Power Inverter for "Powerless" Treatments

This past 4th of July weekend the family and I took off up north to escape the 100+ degree heat here in beautiful Phoenix. We camped about 10 miles north of Strawberry, AZ at 7800 feet complete with tents, shelter for eating, propane, camp fires (kind of), s'mores, no bathrooms and no electricity. No electricity generally makes it tough to live the CF life as power is generally required for most of our treatment regiment. I use a inCourage Vest system and a Mobilare 50psi compressor for all 3 or 4 of my treatment sets, so for me, electricity is a must. I haven't been camping for years and years without a cabin full of 120v outlets, so I've never needed to come up with a solution for electricity before. That is, until we decided to "rough it" this past weekend.

Here was my solution:

Pictured above is the Wagan 1500W Power Inverter. I purchased it from Fry's Electronics and if you're interested in ordering one for yourself, you can see more info by clicking here. 

A brief rundown of the nitty gritty...
Price: 179.99
Continuous Use: 1500W
Peak Surge Power: 3600W
AC Voltage Output: 110V
Optimum Efficiency: 90.00%
No Load Current Draw: less than 0.95A
Input Voltage Range: 10V to 15V
Low Battery Alarm: DC 10.5 +/- 0.5V
Auto Low Battery Shutdown: DC 9.5 +/- 0.5V
Cooling Fan: Yes
AC Receptacles: Triple

Now, I bought the 1500W which was way more wattage than required to run my Vest and neb compressor. My Vest requires 500W and I'm assuming that my compressor requires much less than that. A step down to the 1000W inverter only provides two AC receptacles and I wanted the third in case I had to charge my phone or make a cup of coffee while I did my treatments :) Plus, having 1500W gave me the option to use the power inverter for a variety of other things...

Options are good

I had the inverter installed at a local shop called Direct Audio and the job was done in less than 3 hours and cost me 85 bucks out the door. They installed it under the driver's side seat with the AC outlets facing the backseat. I usually had my Vest and compressor plugged in and sitting on the ground near me while I sat in a chair. When it was raining, I simply sat in the front seat and left the equipment in the backseat. (I've also already done a full treatment set on the way to the airport while I was driving. Talk about a time saver!!)

Surrounded by pines!

Grounded.

Nice view during treatment time

When I did my treatments I always had the car running as to not run the chance of draining my battery (you also must turn the power to the inverter off when not in use). That was recommended to me by the guy who installed the inverter and it certainly made sense to me. So, as long as I had fuel in the tank, I was in the clear to do my treatments whenever I felt like it. Having the freedom to go camping and not have the worry about keeping my health as the number one priority was definitely worth the price tag of 264.99+tax.

Mckenna loves treatment time with Daddy

A couple other answers to questions I have been asked:

I didn't worry about sterilizing my neb cups in between treatment sets. I figured it would do no more harm than the 10+ years I washed them in the dishwasher about once a month. If I was worried about it however, I could have boiled water or plugged in my baby bottle sterilizer.

You can keep your Pulmozyme cold in the same place you keep your food cold. Ours was in a cooler full of ice.

I wasn't worried about disturbing other campers. We were very secluded. Even so, it wouldn't have bothered me and would have given me the opportunity for a little CF awareness ;)

I went to the bathroom in a hole that I dug by a tree. It was glorious.

I didn't feel a major difference in the power supplied to my Vest or compressor. I could possibly be convinced that my Vest was a bit weaker, but it wasn't super apparent. 

Final Thoughts:

The inverter did it's job and I would give it two big thumbs up. It will certainly provide more freedom and options down the road and I am very pleased with my decision to pull the trigger and pay the money. I would do it again in a heartbeat.

Mckenna even got in on the picture taking action!

Please leave me any questions you may have about the power inverter and I will do my best to answer them.

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.

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.

Can You Really Gain Back Lung Function?

Question from reader

Question: 

So I know I've read things in your blog and have heard stories of CFers who had lung functions in their 30s and somehow managed to get it all the way back up to the 50s, 60s or even 70s. I'm pretty sure I even saw that you yourself were down in the 30s at one point. Do you have any idea how people (or yourself) have gained so much lung function back? How many breathing treatments a day does it take? How many hours of exercise? How much of a miracle?

Last year I was training for a marathon with my lung function in the high 60s, doing great. In July, I ended up being rushed into emergency surgery for a bowel obstruction and twisted intestine and a month afterward my lung function plummeted to the 20s and 30s. A year later I still can't recover. My lung function is now 27% and I'm going next month to meet a transplant team. It's super disappointing to have your feet knocked out from under you like that and even more disappointing when I've quadrupled my breathing treatments, tried exercising my butt off, and prayed every night for a miracle to no avail. I know there's a point when you're lungs can't repair themselves, but I also just can't believe that could happen in ONE month after surgery. I'm just wondering if there is something else I could be doing that others have done.

Answer: 

Thank you for taking the time to send me an email. Hopefully I can help you in some way :)

Generally, the people I see bounce back from lower lung function are from 3 camps - 

1) Never took care of themselves, almost died and got scared into changing

2) Took okay care of themselves, got in a routine that didn't change much, slower decline and then finally realized that if they continued doing what they were doing they were going to die 

3) Took great care of themselves, hit a rough patch or a big event, sharp decline and then bounced back after changing a few things up.

There is of course other situations out there, but these are the three main ones that I see. There are also some who experience one or more of these situation during the course of their lifetime (If your curious, I was mainly #2). 

To answer your direct questions, it's generally increased breathing treatments and a regular, intense and consistent exercise regiment that gives people the best chance to bounce back. For me, it took 18 months of running 6 days a week and doing 4 treatment sets a day (4 to 6 hours devoted to CF care) for me to go from a 50% to a 75% (I went into the hospital in the 20's and after a week in ICU I blew a 31%). I've been able to maintain my 75% by doing 3 to 4 treatment sets a day and exercising 6 days a week for the last 3 years. I devote 1 to 2 hours a day to some type of exercise and/or physical activity.

It's funny that you mention the bowel obstruction as the catalyst for you to see a sharp decrease in lung function. One of my largest declines in lung function also came after a bowel obstruction and removal of part of my small intestine. I've been able to get most of that lung function back, but it's been over a decade (obviously a lot happened in the last ten years, and much of it was me not taking great care of myself, or as some people call it, college.)

With all of that said, here's my advice: Put yourself in the best position to succeed in and every day. Treat each day as a new day to be your absolute best and better than the person you were the day before. Don't get discouraged by the numbers. It's much more important how you feel. If you don't think you can push anymore, push harder. A lot of gain can be made during the time of resistance. Sleep is just as important as activity. This doesn't mean that you sleep the day away, but it does mean that you may not be able to have any late nights for a while. I always found that my best mucus clearing workouts came first thing in the morning as the sun was rising! If your aren't seeing the results you want, either by the way you feel or by the numbers, change up your routine (medications, exercises, treatments, breathing techniques, etc). And probably most importantly, never accept a place you don't want to be and never stop pushing until you hit your ceiling. How do you know you have hit your ceiling? You never will, so that means you're in this for the long haul. The only way I'll know that I maxed out is after I'm dead; know what I'm saying?

If you do what I outlined above, and you still don't see the results you'd like, that's okay! You'll still have your head held high because you know you gave it your best shot. Remember, failure only occurs when you don't try. If you try, no matter the result, you're a winner.   

Please let me know if you have anymore questions!!

.............

If you have any questions that you'd like to send my way, please email me at ronnie@cysticlife.org!

It's Good, but It's Not THAT Good

I've heard a lot of different reactions to my blog a few weeks back about the clinical trial I'm currently on which will remain nameless. One particular reaction or thought that I wanted to address goes some like this,

"Oh, that's awesome!! I can't wait until it's available because I hate CF and I hate treatments."

I guess I'll just be the one to put it out there - You can take any med currently being studied in the pipeline, and if approved, it will still not take CF away. Now this can mean different things to different people. Obviously, there will be people who get on the right "miracle" drug and it totally flips their life upside down and they start living as though CF didn't exist. I have a feeling that will be rare.

I think it's safe to say that most of us will still feel some if not all the effects of CF daily, but hopefully, less often and to a lesser degree. Many of us will still have to do treatments if we want to be the best version of ourselves.

Case in point: Today's workout was extremely hard and I was dragging booty the entire class. It was hard to catch my breath. I felt low on energy. I was doing more resting than usual and I was light-headed most of the class.

I thought for a second, "Where are you now _______ (name of study drug)?" You see, just because I've had fantastic results from the drug, or the placebo, doesn't mean that CF, or in most cases, just regular life and human reactions, go bye-bye.

I got to bed later than normal yesterday. I didn't sleep as well. I ran/walked 4 miles yesterday and I'm pretty sure my legs remembered. I ate like a 20 year-old college student this past weekend. I'm sure I could have given better effort during my treatments while in San Diego. I was out of my regular routine for 3 days. I think it just all added up.

Here's my point, no matter what drug comes out next, it won't change the fact that we're going to have to work hard. We're still going to have to make good decisions and treat our bodies with respect. The hope of course is that when we do screw up (as I am king at this) the penalty to pay maybe won't be so steep. Maybe, and Lord willing, the next generation will have no clue what "our CF" feels like. All of this of course is only speculation.

The last thing we can afford to do is wait on a med that may or may not be a "game changer". We all need to keep our nose to the grindstone and kick some CF booty each and everyday. This looks like doing our treatments, living an active lifestyle and putting our health first. If we can do that, there is no doubt that we will be the best version of ourself.

I'll leave you with this quote from the great Larry Bird which I think nicely ties up this blog with a nice ribbon. He said, "I find that the harder I work, the luckier I get."

Couldn't have said it better myself.

I wish I wasn't FORCED to do treatments.

I remember the good old days as if they were yesterday. Those were the days I was able to do treatments on my own schedule, whenever I wanted, and work cystic fibrosis around my life. Those were the days when my doctors recommended 2 treatment sets a day and 3 if I wasn't feeling quite up to snuff. Those were the days I was running around like crazy and it felt like I barely had enough time to get in even the 2 recommended treatment sets a day.

In those days my treatment sets consisted of albuterol, Pulmozyme and the Vest. I only did TOBI when I was in the hospital, or when my docs convinced me that I needed a month or 2 at home. If I could go back to the good old days, I would. (If only to correct the mistakes I made concerning my health)

If I took better care of myself when I was younger and actually had less responsibility and no one truly dependent on me, there is no doubt that I'd be a better version of myself today. I firmly believe that I'd be a better husband, father and friend.

It's funny, because now I have real responsibility in my life and I do truly have less time for treatments and exercise.  Back in the day I was filling up my time doing many things that didn't have any real impact on the world nor did I have a family that I was required to put first. I often think back on the “good old days” and wonder what in the heck was I doing?

[SIDE NOTE: They wanted me to do at most 90 minutes of treatments and 30 minutes of exercise a day back in the "good old days". Today, I often do 90 minutes of treatments in one sitting. It's not uncommon for me to put in 240 to 300 minutes of treatments and exercise each day.]

Back in the "good old days" doing my treatments was a choice, a choice that I wish I would have made more often. I would have been much better served to have treated my body well when I was healthier than to take my health for granted, as I did, and end up at a place that I wish I never experienced.

For those that are new to this blog I'll give a very brief summary of what really changed my CF journey:

When I was younger I was very active and I never missed any treatments. I was very active and I didn't miss any treatments because those were the rules that I had to abide by to live with my parents. When I moved out I started making my own choices with regards to treatments and exercise. I didn't always make the best choices. I saw my lung function steadily decline from 97% in 2000 (the year I moved out of my parent's house), to a baseline of 70% in 2007 (I was in the hospital for 30 days every 3 months),  to an all-time low of somewhere in the 20's in 2009 (I was too sick to blow in the ICU and blew a 31%, 10 days into a 52 day stay). I decided during that hospital stay that I could no longer exercise only “when I had the time” and that 2 treatment sets a day (which I was actually doing consistently at this point) just simply wasn't going to cut it anymore. I wanted to live.  

After leaving the hospital in March of 2009 I made a dramatic change. I started exercising and/or working out every single day. I started doing 4 treatment sets a day, no matter what. I added any additional medication that I could take. I added an inhaled antibiotic every month. I decided that if I was going to take care of myself, the way that I should, I was going to go all in. If I wanted the results I was aiming for (an FEV1 of 75% after they told me to be happy if I got it up to 55%), I knew that my health was no longer a choice, but a "have-to". 

Through hard work, dedication, and the grace of God, I was able to regain and surpass all of the lung function that I had lost in the previous 10 years. 

I sit here today writing this blog feeling better than I have in a long, long time. The cool thing is that I'm able to say on most days that I feel better than the day before. I recently had an FVC of 92% and that's something I haven't done since 2001. My FEV1 baseline is 75% now, and I haven't seen that baseline since 2003. My life is fuller and more enjoyable than at any time in those years I was making my own decisions concerning my health care.

It's not all good news though - I am FORCED to do treatments. I am forced to exercise. I am forced to put my health first.

If I don't put my health first, even for a day, I feel it. I can no longer miss a treatment here and workout there and not feel a negative impact from that decision. If I want to be at my best, I have to do all my treatments; I have to get to the gym; I have to put my health first.

Back in the “good old days” I could miss a few treatments without consequence. Back in the “good old days” I could sit on the couch for days on end and still have the lung function of most of my peers. Back in the “good old days”, health could be more than a few notches down on my priority totem pole and I'd still be able to live a full and active life.

Not today.

If I miss treatments today, my lungs feel tight, my breathing is suppressed, and my lungs are junky. If I don't move around for an extended period of time, I feel incredibly lethargic, I get very achy, and taking a full breath is nearly impossible. If I don't put my health first and at the top of my totem pole, I wouldn't be able to take Mckenna to the park every morning; I wouldn't be able to tackle life with Mandi; I wouldn't  be writing in this here blog.

When I look back on the “good old days” I realize that though they were good, they weren't great. In all honesty, they were pretty pointless.

The love I had for life back then pales in comparison to the love I have for my wife today. The fulfillment I got from the "good old days" isn't even in the same universe as the fulfillment I get from being a daddy.  I'm able to write about the "good old days" with a new purpose, a purpose that wouldn't have been discovered if I continued to live in the "good old days".

Things would be a lot different today if I would have taken better care of myself in the "good old days". If I would have put my health first when I had the choice, maybe I wouldn't be forced to put it first today. Choices in life are a great luxury to have. When I had that luxury, I made really poor choices. Today, with that luxury gone, my only choice is to live a life full of treatments and exercise if I want to be the best version of myself each and every day.

I don't have many regrets in this life, but I do wish that I would have listened when they tried to tell me that the "good old days" pale in comparison to what God had in store for me.

I can promise you this - I would have made better choices.

Cystic Fibrosis ALWAYS Gets Worse

Cystic Fibrosis always gets worse. It can't get better once you reach ____% lung function. Once you start going into the hospital multiple times a year, you'll probably go multiple times a year for the rest of your (short) life. When you lose lung function, you can't get it back.

Any of that sound familiar? I really hope it doesn't, but I have a feeling that some of you have heard all of those things a time or two. I know I did growing up, and the sad part is, I actually let myself believe some of it. I fell for the part of CF getting worse year after year hook, line and sinker. I was reserved to the fact that I would lose a little bit (or a lot bit) of lung function each year and there wasn't much I could do about it.

I mean, that's what my friends with CF said. That's what I read on the Internet. That's what I heard some CF docs say. It must be true then right?

Wrong. It's wrong for so many different reasons that I don't have the time to list them in this blog, but I'll tell you the biggest reason that it's wrong...

I got caught up in thinking about all of the things that CF could do and lost focus on what I was doing.

That becomes the problem. When we are so concerned with what could happen with our health that we let it distract us from actually doing something about our health now, we have an issue. I totally get it as much of society is stricken with the "it-must-be-somebody-else's-fault" bug.

If we don't feel well, it has to be because of CF. If we're having a bad day, it must be because of CF. If we're mean to our friends, it must be because of CF. If we flunk a test, it must be because of CF. If our lung function declines, it must be because of CF. And on and on and on we go, blaming anyone and in this case, anything else, before we're willing to look in the mirror and realize that we're the problem.

It took me 8 years of declining lung function and 52 days in the hospital to realize that. It took me not being selfish for the first time in my life and actually putting a special girl's needs in front of my own by actually taking care of myself the way that I should. It got cemented when I brought another special girl into this world and made a promise to her that I would always work my booty off.

What's it going to take for you to change?

And I know, I know, there are people out there who will say, "I never miss treatments and I always exercise, but my lung function continues to get worse." Yes, that can be the case (I used to exercise and do my treatments too while seeing my lung function decline - I just wasn't doing as much as I obviously needed to). It however is the case that you're lung function will decline if you don't do your treatments and you don't exercise. . It is also that case that some people wait for far too long before they finally decide to "never miss treatments and always exercise". To be honest, I was almost to that point 4 years ago, but by the grace of God was able to fight back.

So what brought this blog on? I had my 1 month post-hospital clinic visit yesterday and for the first time in 12 years, I had a FVC of 92%. My FEV1 was 75% which is still up from my "baseline" of 50% in 2009 that they said I'd probably stay at. They were wrong.

If you'd like to get healthy, do what you do in spite of CF. No matter how CF decides to act tomorrow, you just make sure to out work it. If your disease is being a little more aggressive, than you must be that much more aggressive. If things are getting better and CF is slowing down a bit, celebrate by kicking it's booty even harder.

I know you can do it, now you just have to believe that you can and will do it!!

Turning Back the Clock

Great question from a reader:

I have a few questions for you, I remember reading on your blog about how you raised your PFTs. I was recently told I can't raise mine and nothing motivates me more than someone saying I can't. I obviously went through the stage every CF patients goes through and stops or halfway does their medicine and treatments and stops working out. So my question is what all did you do to raise yours? What kind of exercise? And did you eat differently?

I was told in 2009 that I should get comfortable with blowing a 50%. As with you, that motivated the heck out of me. It took two years of crazy hard work and dedication, but I was able to blow a 73%. Since then, I've remained steady and haven't had too many drastic dips. I've even been able to inch them up a little more.

For two years, this was my schedule -

Albuterol/Atrovent 4 times daily
Pulmozyme 2 times daily
7% 2 times daily
Inhaled antibiotic 2-3 times daily
Vest 4 times daily for AT LEAST 30 minutes at a time
Ran for 20 to 45 minutes 5 days a week
Took daily walks and bike rides
Lifted weights 3 to 4 times a week

I didn't really eat all that differently.

As you see though, it was a big commitment and obviously one that has paid off for me. When I blew that 73% in 2011, it was my highest number since 2003. So you can't tell me that we can't "turn back the clock"!!

Anyway, hope this helps and please let me know if you have any other questions 

A Common Frustration

I wanted to post this email and my response as I'm sure plenty of you out there can relate.

I read your blog every time you post and I just love all you do for the CF community. I am not sure what exactly my point is in the email except that I guess I am a bit discouraged. I read your blog and see stories like today with your guest blogger and I am so happy for her but discouraged at the same time. I guess I don't understand how _____ can be so compliant, do all his meds, exercise, treatments, and yet we are still sitting at high 50's/low 60's on pfts with IV's and hospital now almost every 2-3 months! It just doesn't make sense to me. 

We are doing everything they say and pfts just go down. I know that colonized pseudo, apergillus, and yeast are all to blame in his lungs along with the worse sinus disease our doc has ever seen in a kid all that sinus disease is with many surgeries and he does daily nose sprays, ointments, and sinus rinses. But I see others say when their pfts go down its because of lack of compliance and exercise and then when they do those things they see improvement. But he is compliant!  

I guess I am just frustrated that we are literally doing EVERYTHING we can including now adding a nose nebulizer to dispense antibiotics straight up his nose and I am still watching my kids pfts go down. :( Again, not sure why I emailed except I am a bummed mom that just doesn't get CF ya know? Makes no sense to me. 

Thanks for your blog and all you do.

Your frustration and feeling of being discouraged is totally understandable. I'm sorry to hear that ____ is struggling so much at this time. Not cool for a kid his age to be held back!!

My response to you would be nothing earth shattering, but maybe something I say will help.

 - First, even though it feels like this has been going on forever, it could also just be a season in his life. We never know how long the season will last, but we do know that we have to keep our head up so we can recognize the next season when it comes.

 - When I was his age, I also had PFT numbers that dipped into the 50's. 

 - My best guess, which I'm sure is the best guess of the docs, is that's _____ sinuses are the big issue. Sounds like they are the driving force behind his uphill battle with his lungs. I'm happy to hear you added a sinus neb.

 - If what you're doing now isn't working, do something else. There is something out there that will help. At least, that's always my attitude. Could be a med. Could be a treatment. Could be exercise. Could be more hours. Could be prayer. Could be a combination of a bunch of things. Continue to tweak until you find the right combo. There will be road blocks and speed bumps along the way, but you have to keep driving.

 - All we are called to do is the possible. If we take care of the possible each and every day, there is no doubt that God will take care of the impossible.

 - Continue to be the strong mom that you are. CF is unpredictable. For _____, you must be that rock that is always predictable. He's always watching and takes his cues from you.

Again, nothing I said there is something you haven't heard before. Unfortunately, there isn't a magic pill. All we have is what we do each and every day that will determine our outcome. We can do everything right and still be disappointed in the outcome, but we must remember that our expectations aren't always in line with the Will of the Father. We all have our ceiling when it comes to our health, our only job is to make sure we strive to do everything to find out where that ceiling is.

I'll continue to pray for ____ and your family.

My Journey to Compliance

Guest blog by Aubrey Bean

I want to start by first thanking Ronnie for this amazing and inspiring blog, and also for the opportunity to share my story.

My name is Aubrey, and I am a 24 year old from a little town in NH. I was diagnosed with Cystic Fibrosis when I was six days old, after I had surgery for meconium ileus. I was a healthy and (very) lucky kid, always having high PFTs and never having to "work" for them. I started enzymes when I was born, but didn't start any nebs or the vest until middle school. I had IVs when I was 8, 16, and 19, and those PICCs weren't because I was sick, but because my numbers happened to slip slightly below my 100% baseline. I spent my first 20 or so years of life being blissfully unaware of the possible repercussions of this disease. However, throughout my 4 years away at college, when treatments happened when it was convenient, and exercise happened never, I slowly saw my lung function decline to 85%. Every appointment I repeatedly promised myself that I would get it back up “eventually.”

Finally “eventually” had to happen now, when in my last semester of college the noncompliance and hatred for any kind of exercise finally caught up to me. In May 2011 when I was a college senior, 2 weeks before graduation, I saw numbers that stunned me, and even caused me to shed a few tears. I blew a 61%. I was sent into the hospital shortly after I graduated college, and was even more stunned by the fact that after 4 weeks of IV antibiotics my numbers improved only slightly. To make a long and pretty complicated story short, I spent the next 7 months in and out of the hospital, including close to a week in the ICU with pneumonia, unable to get out of bed. I spent those months battling a few different medication allergies, new bacteria, and even a fungal infection in my lungs. Finally in January, after my third case of pneumonia in less than 6 months, at 34% lung function, I made the difficult but necessary decision to quit my job, one I had been offered in October. I hated that I had to quit my job. I hated the fact that I lost 50% of my lung function in just one year. But more than that, I was scared that it would never come up again.

  

During my hospital stay in January 2012, I constantly heard the voices of multiple doctors telling me that pneumonia causes permanent damage and they weren’t sure how much lung function I would be able to recover. I even had one of them tell me I should shoot for 50% lung function, but not much higher. I left the hospital hearing those words over and over in my head. Their voices became an echo, at first scaring me, but suddenly I turned it around, and those words became my motivation. I realized that all I could do was try. I thought even if these doctors are right, I at least want to make a strong effort to prove to them and most importantly to myself, that no matter how much CF knocked me down in the past year, I was going to stand up and try my best to come back. So that’s what I did. I started to exercise (with the help of RSBR and other awesome CF blogs!) and made a vow to never skip a treatment, unless it was absolutely necessary.

I started slowly, walking on the treadmill until my lungs started to ache. My walks started getting longer, and feeling a little better. After a few weeks of just walking, I started to run, (slowly) and soon I was running very small lengths at a time. It was hard and extremely challenging. A couple times I almost made myself sick I coughed so hard, and other times I spent my ride home from the gym crying, wondering why I couldn't do more than walk. I was frustrated more than satisfied, and I was in pain more than comfortable, but I wasn't giving up! I continued to run more, and exercising became a lot easier and more pleasant within a few months. I kept at it about 5 times per week, and it was all worth it when in August of 2012 my hard work paid off and I ran a mile for the first time. Ever. Today, it is still worth it.

Sitting here writing this today, I'm reliving my 2011-2012 journeys and I realize just how different my life is, and how different my lungs are! I got another job in November, and have not been sick once. Also, last week I went to clinic, and I blew FVC 80 FEV1 65 (And I’m shooting for higher next time!), over 30% higher than last February. My PFTs increased by that thirty percent because of hard work, patience, a lot of faith, and a lot of treatment compliance. In the last year, I have missed one treatment (for my sister's wedding!!) and last week I ran 2.5 miles on the treadmill.

My last hospitalization was January 2012. Coincidence? I don’t think so!

In the last few years, CF has taught me so much about myself and life in general. I've learned life lessons I never would have learned otherwise. I have also learned that as much as I am being pulled in so many directions every day, I know that treatment compliance and exercise are the most important aspects of my life to never let go of, and I am very grateful for that lesson. I've also learned that although CF is tough, and may knock me down sometimes, I have proved to myself that I am tougher and I will always try my hardest to fight back.

Bio: My name is Aubrey and I am 24 years old. I live in southern New Hampshire, and have been seen by doctors in Boston, MA my whole life. I graduated from Merrimack College in North Andover, MA in 2011, with a degree in Spanish. I am currently employed at a bank in NH and I really like it! In my free time I like hanging out with friends, family, and my dog, singing, dancing, shopping, and of course working out and trying to stay as healthy as possible!!

Note from Ronnie: I can't thank Aubrey enough for sharing her story with us. We're seeing this type of story happen more and more, so I hope that it's a huge encouragement for the community. Maybe it can also serve as a wake-up call to those in college who have put their health on the back burner. People ask me all the time where they can find motivation - well, here it is!! The proof is in the pudding so they say. Thanks again cyster!!

Interested in contributing a guest blog about you and the fact that you kick some CF booty?!? Email me your blog idea at ronnie@cysticlife.org.

We Need More Options!!

Near the end of January, I was invited to Washington DC to speak about the need in the CF community for more treatment options, especially, treatment options that take less time. Here's what I said:

Good afternoon ladies and gentlemen. I want to first and foremost thank you for allowing me to be a part of this important process.

My name is Ronnie Sharpe. I’m a 32 year old CF patient. I’ve come here today to tell you about my life and what you can do to improve it. I’m a University of Arizona Alum. I’m a native of Arizona. I’m a brother. I’m a Christ follower. I’m a son. I’m a friend. I’m a sports fanatic. I’m an exercise enthusiast. I’m a business owner. I’m a cystic fibrosis patient. Most importantly, I’m a husband and a father.

I want to stress just how fortunate I feel to be able to wear all of these titles.  I have an incredible life and I am blessed to be exactly where I am today, as the future didn’t always look so bright for me when I was born with cystic fibrosis in the early 80’s.

Cystic Fibrosis, the disease, and how it affects the body, hasn’t changed over the years. It’s still the same genetic mutations, affecting the way our cells operate within our body.  What HAS changed however is the medications and the treatment options available to us over the years. I am here today, thanks to people like you helping to usher in new therapies to this community. It is these medications and medical advances that allow me to be here today, decades older than the expiration date given to my mom when I was born. It is these options that have allowed me to say “I do” to my wife and watch my daughter be born last year.

With that said however, we still don’t have enough OPTIONS, and current medications aren’t enough. We are certainly leaps and bounds ahead of where we have been, but as a community we NEED more. What works for some may not work for others, that’s why OPTIONS are so important. It’s so important that we can try a variety of medications to see what our body responds to; so we have the opportunity to put ourselves in the best position to succeed and take care of ourselves as best we can.

As the OPTIONS available to us have grown, our life expectancy has increased, our health has improved and our quality of life HAS gotten better.

And, if you ask me, quality of life is one of the biggest improvements we can ask for. Added years are important, but if you cannot live and live well than I feel there is little point in increasing life expectancy, which brings me to the something else you can bless me with today; MORE TIME.

I know many, if not all of you, cannot understand the treatment burden that CF puts on my life, but all of you can understand time, because we all value it and it’s worth just as much to you as it is to me.

  

Like all of you, I have a lot of things I want and need to do during a day. I need to succeed at my job. I need to do work around the house. I want to spend time singing Hokey Pokey to my daughter. I want to watch TV on the couch with my wife.

But there is one aspect of my days that I have to fit all of that around, that many of you will never understand: my daily care routine for my Cystic Fibrosis. My treatment routine, currently, dictates my days, my schedule, and my routine and in many ways, my life. I actually ran a stopwatch to give you an idea of what I’m talking about.

On Monday, I spent  3 hours 12 minutes and 56 seconds  doing cystic fibrosis related treatments and exercise. That is an average day for me. To give you an idea, that is over 22 hours a week,  over 96 hours a month and over 1,150 hours per year.  I spend 48 full days a year doing CF related treatments and exercise!!

Any treatment that I can take that isn’t a huge burden on my time really excites me. Any potential treatment option that I can take that can potentially “give me time back” excites me even more.

So today, I am asking you for those two things: More options and more time.  A positive recommendation for Bronchitol will do both.

Making a change isn't easy.

I had a pretty general question for ya, and i saw how much u like questions. I am 19 years old and never been really compliant with my meds. i was never able to motivate myself long enough to keep doing them. i recently admitted myself into the hospital because ive felt worse than i ever had. I was told to watch your talk about cf and bag of tricks and i found you very insirational. in fact your the only person that has really gotten through to me and i realize i have to take my meds so i dont feel this crappy but im scared i may slip back into a depressive state about it.. I wanted to know how you kept doing your meds after your major scare, because you must have created a habit of not doing your meds consistantly n thats very hard to break out of. I to remember just beingon albuterol and chest pts when i was younger. times sure have changed just wish i could do it as easily.

............

I'm happy to hear that you're open to change your habits concerning your health and I hope that you start to feel better. With that said, yes, I've been in your shoes.

The biggest thing for me, was to stop being selfish. All of the choices I was making that in turn had my health declining was all about what I wanted to do and what made me happy. Yet, everyone around me, who cared for me, was very sad about how sick I was getting. They were sad to see me become the shell of the man I once was. They didn't like to see me struggle to breathe. They hated seeing CF "hold me back".

After I had my "come to Jesus" moment I realized that it wasn't CF holding me back, but that it was me. It was a culmination of all of the bad choices that I had been making. Simply put, I wasn't putting my health first. And as you can probably guess, life isn't as awesome with bad health.

When I made a change, I decided to make a schedule and stick to it no matter what. I also committed to doing "everything right" for as long as I was doing "everything wrong" which in my case was 8 years. I see too many people that commit to doing their treatments for a month or two, don't see the change they had hoped for, and then quit. I didn't think it was realistic to erase 8 years of bad decisions in two months, or even two years; I committed for the long haul.

This commitment meant exercising regularly, increasing my daily treatments and being pro-active about hospital stays.

All I can say, is that it's worth it. I feel great. I spend less time in the hospital. I look better. I breathe better. My mind is better.

Hope that helps a little bit. I'm always here for a chat or any question that you may have.

Ronnie

Vest too big and ineffective?

Question: Yet another question. This one is about the vest. They ordered mine while I was pregnant. But now I'm not sure if the fit is right & if it's working as well because of that. It poofs up quite a bit even with the straps pulled as tight as possible. I personally don't feel as if it's doing too much good. Just wondering if it truly does make a difference. 

Response: Yeah, it may be too big. But, I've found that I have to mess with it quite a bit for it to feel "just right". Be sure that you're sitting straight up and that nothing is putting pressure on the vest from any angle. Many people sit back in a chair or on the couch and that lessens the effectiveness. Mess with the shoulder straps, the clip straps, everything.

Be sure to mess with the pressure as well as the frequency. It should always be going up, or down, but never stay the same. You can find some good recommendations on CysticLife.org as far as exact settings go. And finally, actively do you're vest. Don't sit there mindlessly on the computer with a neb in your mouth taking shallow breaths repeatedly at the same rate (which I can be very guilty of). Breathe in deep, exhale fast. Breathe in slow, exhale slow. Huff cough. Take time to really cough your brains out.

There is certainly nothing magical about a vest. If we treat it as a piece of clothing that happens to shake, we'll get nothing out of it. We need to treat it as a workout that requires participation.

The Best Treatment for Cystic Fibrosis

I often get asked about the best treatment I use or do when it comes to managing (or as I like to call it, "kicking the crap out of") Cystic Fibrosis. This is probably one of the easiest questions that I get asked. The best treatment for cystic fibrosis is undoubtedly, unequivocally, no doubt about it... the one that you'll actually do and do consistently.

That's the real struggle isn't it? We all know that treatments work. Anyone who says to me that they consistently do treatments and it doesn't seem to help, generally fall into one of two different camps - They've either waited too long to decide to do treatments consistently or they have a hard time telling the truth. Is there somebody out there who has tried every different treatment option available, given it all that they have and still doesn't feel any positive effects from treatments? I'm sure there is. But I'm also sure that that person isn't you and that person isn't me.

Growing up I was good at doing the limited treatment options available to me, because my mom was good at making sure I did them. It wasn't an option in my house to miss a treatment and when I did, I suffered. No, usually not health wise, but, "what-I-wanted-to-do" wise. If I decided that a treatment wasn't going to happen a particular morning or a particular night, my parents then decided what wasn't going to happen for me later that day or the next. That could mean no hanging out with friends, no football practice, or no leaving my room unless it was for school. I'll tell you what, because they were consistent and because what they said they would do, I rarely missed a treatment!

Now, did I nail the doing all my treatment thing when I moved out of my parent's house? Nope. I nailed it about half the time. As a result, I lived about half the quality of life that I would have had I taken care of myself consistently. It wasn't until people came up to say goodbye to me in my ICU room in 2009 that I finally "woke up". The last thing I want for my cysters and fibros out there is that!

So, to my friends out there with cystic fibrosis, stop thinking about it and just do the darn thing! If that darn thing isn't working for you then find another darn thing that will. It may take some effort and commitment on your part but if you're serious about either staying or getting healthy, you can make it happen.

To the parents out there, don't allow your children the option of not doing their treatments. Sure, they can pick what to do, when to do, how to do, or where to do, but they should have no control over the to do or not to do! Follow that? And another thing, start immediately! Don't wait to implement this groundbreaking strategy until they are (fill in the blank) years old. Adherence to treatments starts from the day they are born on and so does non-adherence to treatments.

What treatment option have you found works best for you "kicking the crap out of" Cystic Fibrosis?

The Ineffective Vest Treatment

inCourage System by RespirTech

If there's one thing I'm guilty of, it's not getting the most out of my Vest each and every time I strap it on.

Here's what an ineffective Vest treatment looks like:

I sit down at my computer desk and load my nebulizer cups with whatever medicine is due. After loading the cups up I place them on my desk in the order of which I take them. After my inhaled medications are all set to go, I reach over the back of my office chair and grab my Vest jacket (For those that are wondering, I happen to use the inCourage system by RespirTech). After my Vest jacket has been buckled on and the tubes coming from the machine are attached, I'm ready to rock!

The next order of business is to decide which pre-programmed set I will use. Depending on how long that particular session will be, between 30 minutes and 1 hour, I select program 1, 2, or 3. There is also a "quick start" option, but I tend not to use that very often. After selecting the program, I start shaking and then stick a neb into my mouth.

This is where the ineffective Vest treatment begins...

As soon as the Vest starts shaking and the neb starts puffing, I immediately get on to the tasks of the day. Whether it's e-mail, bills, social media, CysticLife, surfing the web, or more e-mail -  the one thing I'm usually not as engaged in is my treatment.

My breaths tend to be shallow in nature. I barely cough the entire time that I'm shaking. I never inflate my lungs fully. I allow the Vest to continue whether or not I feel that it's effective. Then, after 30 minutes or an hour, my Vest will stop shaking and I will move on to the rest of my day.

As I alluded to earlier, there are many times that I do not cough once in a Vest session. Now, the question has to be - is that the Vest's fault or my fault? If you know me at all you know how I will answer that question.

Ineffective Vest sessions are my fault. If and when I start doing engaged Vest treatments every time I put the thing on and then continue to receive an ineffective treatment, we can talk about what role the Vest can play in not making me cough.

Until then, I need to change the way I approach a Vest treatment if I would like a different outcome.

Hers's where you come in. Have any tip or tricks that you use for a more effective Vest treatment?