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!

My "Get Healthy" Checklist

Guest blog by Ashley Coleman

If you asked me who I am and what I am about, you probably wouldn’t ever hear me being defined by my Cystic Fibrosis. First and foremost, I am a devoted follower of Christ. My passion is to help people find truth. The truth sets us free. Where does freedom come from? Jesus Christ. I’m defined by few things outside of Him, but Ronnie has asked me to write about my Cystic Fibrosis journey, and so I will.

Stay with me as I make this long, long journey into a few paragraphs. At the end of February 2012, I noticed I was getting sick. I had just finished a long day at work and had a really lame workout because I was so fatigued. I bought some groceries because I knew I would need a few days of rest. Little did I know, I wouldn’t be able to carry those groceries in my house. I was so out of breath, I had to call my mom to come unload my groceries. This was a Sunday. She convinced me if I was still unable to make it up the stairs without getting out of breathe that we would visit our local ER (not my CF Clinic that is 90 minutes away). Needless to say, I didn’t improve and I found myself staying in the local hospital for a night without them knowing what to do with me. I was sent home with some oral antibiotics and not even given a PFT.

A few days later, I found myself walking the halls of my CF Clinic, Tulane, to my doctor’s office unable to even carry my purse. Once again, Mom to the rescue and after much convincing my doctor admitted me. I stayed 3 nights and was released just in time for my 25^th birthday. 20 pounds lighter, lung function at 57%, first PICC line, first CF hospitalization, first real CF exacerbation; I was scared.

57% was a number I had never seen. Not me, I am healthy. Not me, I don’t really have CF. It was time to wake up and get out of this cozy place of denial and take control of my health and well being. For two weeks, I did every single IV and breathing treatment and even walked some. There was no getting past this; it was now or never. Wake up and deal with my CF or just lay here and die, literally. It was life or death for me. I did improve to 78% but that wasn’t good enough for me.

I was born a fighter. Not literally, but figuratively. I was not going down like this.

As soon as I got my PICC line out, I was cleared for vigorous exercise again and could get super sweaty. I grabbed my Insanity DVDs and sometimes did two workouts a day, walked my dog, did every single treatment, and went to the gym. I packed on some weight, a little more fat than I wanted, but over the past 18 months my body composition has changed. I have packed on solid muscle and improved my lung function to 95%. I went from 86% to 95% in just three months. At 86% my doctor’s words were “don’t expect too much improvement from here” so just imagine his reaction when he saw 95%!

My biggest and only change is exercise. I workout 30-120 minutes per day, 6 days a week. Every day I go to the gym to workout a different muscle group. I would recommend beginners to start with a 3 day a week full-body routine, but I have been weight lifting for about 3 years now. I also do some sort of cardio. Lately, it’s been Insanity videos, swimming, and running. Now that it’s getting cooler out, I am going to be cycling more. I also walk my dog 1-2 miles a day and I look forward to that increasing as well. My diet consists of anti-inflammatory foods 5-7 days a week.

So what do I suggest to improve your lung function and quality of life?

•      Add things before you start taking away. Add the anti-inflammatory foods before taking your daily Doritos away. Eventually, you will begin to relate how you feel to what you are putting in your body. FOOD is FUEL. Add a little exercise at a time. You don’t want to overwhelm and burn yourself out.
•       Do what you can with where you are and what you have. What does this mean? If you can walk to the mailbox and back 5 times do it, then do it again the next day and do it 6 times. If you can run a mile, do it. If you cannot afford a gym membership, do your best to fit that in your budget. Join a class at the gym. The YMCA turns no one away for being unable to pay.
•       Don’t do this alone. Get friends involved. Ask for accountability, whether that be friends in person or friends on CF forums. Make a commitment and do it. If you can afford it, hire a personal trainer 2-4 times a week. I am beginning online training if you are interested, but I’m not here to plug myself. A financial investment is sometimes all you need for lasting motivation.
•       Mentally prepare yourself. Tell yourself every single day that you can and you are doing this.  Tell yourself what a good job you are doing and truly believe it.
•       Hebrews 12:11 says, “No discipline seems pleasant at the time, but in the end produces a harvest of righteousness”. I posted this scripture everywhere in my house.
•       Be kind to yourself. You will not change over night. You aren’t where you want to be, but you are on your way. Remind yourself every day of this. Love yourself well enough to take care of yourself.
•        Find a balance. Be diligent with your treatments, but also don’t beat yourself up if you miss one. It’s okay. Find that healthy balance.

Please, let me know what I can do to help. My goal is to see us as a CF community (and human race as a whole), take charge of our individual health – mind, body, and spirit. We can do this. You can do this! Believe me. Believe in you.

Bio: Ashley is 26 years old and lives in Mississippi. Ashley is trying to make a difference in this world, starting with her community while seeking health in all areas of life, freedom, and happiness for herself and others. She hopes to encourage and inspire transformation, not only those with CF, but every person she comes in contact with to be healthy and strong, but most importantly tap into God's perfect plan for their life and walk in God’s promises for all of us. She is a personal trainer and aspiring writer and blogger. She is passionate about life, loving people well, laughing, running, reading, and learning. Ashley believes every day is a journey, an adventure, a learning experience, and another opportunity to live a day full of love and life. Join her on this journey by visiting her blog: http://www.findingtruth1.blogspot.com/ She would also love to hear from you. Her email is ashleycoleman87@gmail.com. 

Cystic Fibrosis and Relationships: How Do We Communicate?

Guest post by Katie Broekema

Hello everyone! This is Katie, I am back again to tell you what is going on in my life right now. To see some of the lessons I have learned from cystic fibrosis and running, click here.

If you read my bio, you saw that I am currently a graduate student at Central Michigan University. But what my bio does not tell you is the path that I took to get there. I have always thought that school was something to do to get you where you want to go, but when I was a senior in college I realized I had no idea where I wanted to go. I was a computer science and communication major; do not ask me why or how, because I could not tell you. I just fell into both fields thinking I would find what I wanted to do, and then suddenly it was graduation time and I still had no idea. Sometime early in the fall semester I followed my nose to a meeting. I had no idea what was going on, all I knew was that there was Pizza Hut pizza there; I slowly learned it was a graduate communication program recruitment visit from CMU. I figured there was no harm in applying for the program; I was shocked when I was accepted with a graduate assistantship. So I went into this program with the idea that this would buy me 2 more years to discover what I wanted to do. I have just finished my first year of that program, and I now have a totally different attitude. I know what I want to do with the rest of my life! It is thus amazing feeling, like a total sense of euphoria. I actually enjoy reading thousands of article pages: I am looking forward to writing my 150 page thesis. So now you may be asking yourself, what is this magical area of interest? Well I will tell you; it is Cystic Fibrosis.

It started out so innocently; I had to write a class paper so I picked something that I knew about and that I thought would be easy, how people with Cystic Fibrosis communicate. I did this all the time in science classes and when I had to give speeches, why would it not work now? I was duped in this process, but in a good way. Once I began my research, I remembered a conversation that I had several years ago with a high school girl who had Cystic Fibrosis. She asked me how I had told my boyfriend about my Cystic Fibrosis, because she also had Cystic Fibrosis and she wanted to tell this guy but she did not know how. This sparked my interest in how to share Cystic Fibrosis-related information with a significant other, and it is a topic that has become my thesis project. Right now I am in the process of collecting background information and starting the writing process, but as soon as that is done I would like to begin collecting data. This is where all you readers come in; I really want to talk to you and hear your thoughts! I am hoping that by the end of the summer I will be able to start interviewing people. If this is something that you are at all interested in being a part of, please send me an email at Broekema.ka@gmail.com

This initial topic has grown much more than I would have ever imagined. As I was doing my research I realized there is no research connecting communication and Cystic Fibrosis or any other genetic diseases. Having a genetic disease presents a unique set of variables and situations that need to be navigated, but there is lack of research on how this all is communicated. Therefore, I am in the beginning stages of many other research ideas involving Cystic Fibrosis and communication. If you would like to be involved, or if you have another area that you think really needs attention, let me know. My goal with my research is not to do research for research-sake, but for the real application into the lives of people with Cystic Fibrosis. So letting me know what you think is incredibly important and makes what I am doing matter. If you have any ideas, please send me an email. I would love to hear your thoughts and opinions.

Thank you all so much in advance for your help, and thank you Ronnie for letting me share my thoughts in your blog. It was such a great honor to write this for RSBR and this blog is really making a difference in a lot of people’s lives.

BIO: Katie is 22 years old and has lived in Michigan her whole life. She graduated from Albion College with a double major in computer science and communication, and she is currently working on a Master’s degree from Central Michigan University in communication; with a focus on health communication. In her spare time, Katie enjoys finding new research topics, preparing to teach her COM 101 classes, helping coach the local high school cross country and track teams, running, cooking and convincing various family members that they should get her a Pomsky puppy. (Look them up, they are adorable!) She would love to hear back from people who have read her posts, her email is Broekema.ka@gmail.com

Note from Ronnie: I would just like to thank Katie for submitting, not one, but TWO great guest posts. I hope she was able to inspire some of you to get out and get active after reading what she has learned through running with Cystic Fibrosis. Also, anyone who is interested in contributing to her thesis, please email her to "get in on the action". I believe this is a very important topic that needs to be explored and I'm really looking forward to what her research brings forth.

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.

Do you cough in public?

I was so excited to answer this question as I know plenty of other cysters and fibros struggle with this issue - coughing in public. I hope this answer gives you the confidence to cough like there is no tomorrow...even if you makes others uncomfortable.

I have a question for the CF "expert"  

So i've got that LOVELY CF cough goin' on right now. Luckily right now it's mostly dry so I don't have too much mucus coming up. But you know the drill, a coughing spell every few minutes or so. How should i handle going to dance? Should I skip out until my cough hopefully calms down a little bit? Because I don't want CF to hold me back any, but i also know the realistics of it. I mean I wouldn't want to dance with someone who kept coughing, even if I was told it wasn't contagious. Because a few of the classes we rotate with all the partners which means gettin' all up and close and personal with everybody multiple times through. I can exercise here at the house for the time being, but i also love dance and miss the people (i've been gone doing a CF study for the past 2 weeks so I haven't been able to see them or go dancing. Sadness!!) . What's the smart thing to do? I want to do what i love, but I also want to be smart and considerate of others.

Alright, first things first, I'm humbled you'd even care what I think about this. Second, I'm no expert, and I actually recommend doing the exact opposite of anything I suggests to be on the safe side :) Third, I'd love to share my thoughts with you!

Let's establish this - coughing is a good thing. Yeah it's annoying (both to us and others), but it's playing a vital role in keeping our lungs clear. I myself am a cougher whether I'm at 30% lung function or 100% lung function. In fact, I give much credit to my cough for keeping my lungs relatively clear my entire life. Sometimes I bring up junk and sometimes I don't. In fact, I've coughed about 10 times since writing this response and have only brought up the goobery goo on two of those coughs.

Coughing can indicate that you are experiencing an exacerbation or outside sickness, but it can also be an indication of asthma, allergies or nothing.

So now that you know coughing is completely okay (at least in my eyes), what do you do about it in public?

I think you know my answer - I cough, and I don't care where I am. I'm at the gym 6 days a week. 3 of the days I do partner work in a fitness class and three of those days I'm in a small room with others doing a core class. Needless to say, I'm coughing near, on and around all of them.

Apparently I don't have a lot of pictures of me coughing.
This was the only one I could find off-hand and it's very old!

If I end up with a new partner, and I've already started coughing before the workout even began, I usually go with one of three phrases that all start with "Don't worry, I'm not going to get you sick....

a) I have Cystic Fibrosis and part of it is that I cough a lot, especially during workouts.

b) I have asthma and my lungs are extra tight today.

c) I'm just a cougher.

One of those three answers always seems to put my partner at ease and we can move on with the class.

When it comes to coughing in public, frankly, I'm not very considerate - especially if I'm doing something beneficial for my health. The truth of the matter is that clearing out my lungs and doing something that in the long run is beneficial to me and my family is WAY more important than the thoughts, stares and feelings of others.

It reminds me of a blog I wrote a while back about being selfish. There are times in this CF life that you HAVE TO be selfish. The funny thing is that often, by being selfish, we are doing one of the most selfless things that we could ever do. The blog is here if you're interested: http://runsickboyrun.blogspot.com/2012/07/are-you-selfish-i-hope-so.html

So what do I think the smart thing to do is? Dance and cough your little booty off!!

Sometimes life calls for us being comfortable with making others uncomfortable.

Hope this helps :)

The Start of My Kalydeco Journey

Guest post by Angie 

On January 31, 2012 the CF community received amazing news.  The FDA approved Kalydeco, the first drug to treat the root cause of CF.  However, Kalydeco is only effective for individuals that have the G551D mutation.  I, like many other DDF508’s, celebrated the wonderful news but was still holding out for the drug that would help the mutations that I had.  That’s right… I said had. 

About a month ago I took my 2 year old son to see a pediatric pulmonologist, because just like his Daddy, he has asthma.  The doctor and I discussed the fact that I had CF and my son’s newborn screen did not come back with any genetic markers for the disease.  He thought this was very strange knowing that my son would automatically be a carrier since I have the disease.  The doctor decided to add a CF mutation panel to the labs he was already ordering for my son. 

Fast forward to one week later and I received an email notification that my son’s test results were available.  I logged on to his EHR (gotta love technology) and saw the CF mutation panel.  I clicked on the result fully expecting to see DF508 since I had been genotyped as a DDF508.  Instead, I got the shock of my life when his test showed G551D.  What?!?!  The only logical conclusion I could come to was that I must have a G551D mutation.  But, that was impossible, right?  I had a cheek swab in the late nineties during an initiative sponsored by the CFF to get everyone’s genotype into the CF portal.  It was after 8pm on a Thursday night, so I couldn’t call any of our physicians.  So, where did I turn?  The CysticLife community.  I posted a rambling question asking if there could be any other logical explanation.  Of course I received lots of wonderful feedback from the CL community.  Everyone drew the same conclusion that I had.

I contacted my clinic the next day and they ordered a CF mutation panel for me.  I had my blood drawn and the waiting began.  I was still trying not to get my hopes up, but I think it was too late.  I received the phone call on a Thursday morning.  I had just sat down at my desk at work and my cell rang.  I recognized the number of my CF clinic.  I answered and the nurse said “I have some wonderful news.”  That was all I needed to hear.  I started blubbering.  I have the G551D mutation!!! I could not believe it.  I cannot even describe what it felt like to hear that news.  I can tell you it ranked right up there with the first moment I looked into the eyes of both my babies.  This news actually allowed me to picture holding my grandbabies.  I started the ball rolling that day to have Kalydeco delivered to my door and begin my new lease on life! 

Thank you for taking the time to read my story.  I can hardly believe it when I read it myself, but I am living it every day and it is wonderful!  I will be posting weekly updates about my progress with Kalydeco.  Please feel free to ask any questions or leave comments through this blog.  I look forward to sharing my journey with you!  

Angie's BIO:
I am a 36 year old married, working mother of two beautiful children. I was diagnosed with CF when I was 7 months old. When I was 6 years old my mom decided to become a respiratory therapist. I grew up with my own private RT!!  I was first hospitalized at age 8 and so began the annual hospitalizations. In my twenties I sometimes averaged two hospitalizations a year, but its pretty much just once a year now. I went through the usual rebellion from my late teens to early twenties. Luckily, I have been able to maintain stable lung functions for over 10 years. My FVC averages around 80-90% pred, and my FEV1 is usually around 63% pred.  

Note from Ronnie: I am so thrilled that Angie has agreed to give weekly updates of her progress on Kalydeco!! Her story is amazing, and I know I for one, am really looking forward to hearing about where this story leads. I can only hope that it's not only to improved lung function, but LIFE function as well!!

True CF Awareness

I was sitting down to write my own post, and just before I clicked on the little pencil to write something, another post caught my eye in my blog roll. It happens to be one of my favorite Life Blogs written by Lauren, a kick butt cyster who I have never heard offer up an excuse. If some of you mommas out there are looking for a college-aged girl to motivate your daughter, this would be the girl to do it. She's funny, smart, athletic and just an all-around great gal! She has a "take the bull by the horns" attitude that I love, and I find myself agreeing with many of her thoughts. What's nice, is she puts her thoughts out there much more eloquently than I and I'm sure offends much less people than I do :)

Please take the time to read this post, you'll be glad you did. When you're done, visit Lauren and read all of her other great stuff at I Have CF...So What?!?!

This image has been traveling around Facebook today and it sort of took me back a little bit. This blog is not meant to offend anyone, it is just my thoughts. Though I love the CF Foundation, and though I understand that it's trying to tug at people's heartstrings in order to get them to donate... I couldn't help but think that for me, and for many CFers I know... this isn't the truth. Sure, there are many many CFers who feel like they are breathing through a straw every day, who are on oxygen, and who are awaiting a lung transplant. However, there are also many CFers who are breathing easy with CF and who haven't reached a point where it takes their breath. It is unfair to portray all people with CF as breathing through a straw, because the fact is, it is possible to breathe with CF. Sure, some days I've experienced what it feels like to not be able to breathe, to cough until I'm red in the face, etc. However, if the public thinks that this is what CF is like every day, they will be strongly mistaken. There can be healthy days. No wonder why so many parents are so confused and afraid when their child is diagnosed, because sadly, there are ads like these that make it seem that this is the reality of all CFers at all ages and at all stages of their lives. 

Every time I tell someone (who has some idea about what the illness is) that I have CF I get a familiar response. I can see the look on their face, trying to figure out how I, someone who looks relatively healthy except for the cough, has this horrible disease, like maybe I'm lying. I sometimes feel like I should to show them my PICC line scars, show them the insides of my infected lungs, take them to the doctor with me to prove its true, because most people don't understand the complexity of CF. I've had people tell me that they had friends who have died from the disease, friends who are always in the hospital, and that they thought that it was an early killer. I'm not saying that any of these instances are false or rare. CF can kill you, it can make you extremely sick.... but you can also live with CF. Because of the way CF is portrayed in order to get people to donate, some people are lead in the wrong direction. We don't see the healthy people in this ad. Why doesn't it say... "Because of all of the donations that lead to the research and new drugs the CFF has helped to bring to fruition, some people with CF DON'T breathe through a straw. Thank you." 

I also don't like that this ad tries to bring pity to the CF population. It's black background and choppy lines connote darkness. "You can stop when you've had enough. But people with cystic fibrosis can't. It's how they live every day..." What do you think when you read that line? "Oh my goooooodnesss, their lives are so horrible and difficult." And yeah... sometimes it is. But do we really need people to feel bad for us because of it? If there is anything I hate it is pity. Don't see me as someone suffering, see me as strong for getting through it with a smile on my face. Yes, I live with CF every day, and yes I am frustrated that I can't make it go away.... but don't cry for me, because I'm not crying for me. I'm doing what I can to survive, and I'm learning so much in the mean time from my hardships. 

It is important to recognize the complexity of CF.  That while there are people who are struggling to survive, who's lives are taken by CF... there are also those people who live with the illness, who's days are numbered but who have both sick days AND healthy days. The CF Foundation has created so much opportunity for people with CF, and they continue to do so. However don't be fooled by certain marketing techniques. This ad is good in that it tries to get people to understand what its like to live with CF at its worst stages... showing that there needs to be medication out there to prevent CFers from getting to that point. But at the same time, it is time that awareness comes to the forefront. Not just awareness about the bad parts of CF, but awareness of the great developments have been made, the healthy lives that have been created, and the true nature of the complexity of this chronic illness. So that new parents are no longer afraid that there child will be breathing through a straw their whole lives, so that someone that is new to CF might not see me as lying on my death bed, so that we can take even larger steps toward a cure without having to evoke pity in our donators.

Thank you so much for that post Lauren. I know that myself, and many other in the CF community, feel the exact same way.

The PFT Game

Lauren over at I Have CF...So What?! posted a great blog today about PFTs - what they are and how she did. We often hear that we can't recover lung function once it's gone. Oh yeah? Don't tell that to Lauren.

Here's an excerpt from her blog and I would highly encourage you to head on over there to read the rest...

It's been a while since my last blog, mostly due to the fact that I saved all of my homework over spring break for AFTER spring break, leaving me a nice week of relaxation, followed by a week of hectic homework and studying. Now that I have some free time I can finally write my blog about how incredible my PFT's were 2 weeks ago. 

First of all I'm gonna take a second to explain exactly what PFTs are because lots of my friends and family are familiar with them, but I've never really explained them in depth. The machine (which is now portable and is just hooked up to a laptop), involves a tube, a mouthpiece, and a device to measure the volume inhaled and exhaled in the lungs. I am given a pair of nose-clips to ensure I don't breathe out of my nose at all. Usually the PFT tech will ask me if I prefer being coached to which I always reply YES. For some reason, I like to have a cheerleader when doing my test. The testing starts with "easy breathing" for which little blips show up on the computer screen. I am then enthusiastically told to take a "BIG BREATH IN" and quickly blow as forcefully and quickly as I can. And then the PFT tech usually says something like "push push push push all the way out come onnnn, litttle mooooore" until I'm red in the face and I watch a little dial on the screen go all the way around like a speedometer. I then take a minute to catch my breath for another round. During the next round, the computer measures how I'm doing in comparison to the last one, so a little ticker will increase up to 100 and then turn green, meaning I beat my last result. This is very exciting to see and makes doing PFTs feel like a game, as I get more and more pleased with my results.

Click here to read what exactly PFTs measure and how Lauren "blew the box off" of her latest test!


Great job cyster!!!

Question from Reader: Family Support

What did your family or friends do to help you through your most difficult times with CF?

I think the most important thing my family and friends did to help me through my most difficult times of CF was to not only show me empathy (not sympathy), but more important than that, was to hold me accountable. We all know that we can do everything right and still become sick from CF. We also know that there are some of us who don't do everything right and become sick from CF. There were times in my life that I was that guy. What I loved about my friends and family is they would always remind me that I was not taking enough responsibility for my own health. They would always encourage me to do my treatments and to exercise so I could be the best possible version of myself.

Did your family ever stop you from participating in anything because of your CF? In what ways did they push you to do things to help you?

After that frank talk with my doctor when I was about 5 years old my family never stopped me from participating in anything. In fact, they did just the opposite. I played sports pretty much year-round from the age of about 6 years old and on. My mom and stepdad supported this by taking me to each and every practice and encouraging me to be my best. They were also very good at reminding me in a loving way that the only way to be the best possible version of myself on the playing field was to be the best possible version of myself in regards to my CF life.

Transitioning to Middle Adulthood

Guest post by Leah Sands

Erik Erikson, social scientist, defined "middle age" as the period between ages 40-65, although the US census has often linked it to the ages of 35-50. Therefore, I feel that I am now at the point where I'm transitioning from early adulthood into middle adulthood. My thoughts on this have been hovering in my head as I realize what this means.

As a child and teenager, I remember looking at adults with gray hair as inferior to my world. They always exuded some kind of confidence as they went about their business. Most of them had horrible fashion sense in my opinion. And why should they be so happy and carefree? After all, they were "old".

After recently celebrating my entrance into my 30's, I began to contemplate a lot of realities about my life. It basically began when I injured my back a few weeks prior. This caused me some major pain issues and an inability to be very active. It made me question why I was having this type of back problem because "I'm too young" to be having these issues already.

In addition, I've even become much more aware of educational and media commentary on the changes that start to occur after you reach age 30 - as if there's some automatic switch that changes you from young and thriving to old and degenerating.

"Young and thriving" - not always the case with people with CF. I was fortunate enough to have been diagnosed when I was born due to meconium ileius; although, I still had difficulties gaining weight and maintaining it. My childhood days were filled with swingsets, coloring, forts, and sleepovers. My daily routine also included enzymes, antibiotics, vitamins, inhaled medications, and chest therapy, which was usually accompanied by exciting games of 'I Spy'.

But as I got older, I came to realize that all of this extra daily routine stuff was actually really a desperate effort to keep me alive and functioning normally. I began reading books from the library, secretly, about CF - only to come to realize my true fate. The encyclopedia told me I'd be dead by age 18. Several non-fiction books told gruesome truths of the tortures of CF. I'd spend nights in my room recording my cough so I could replay it to myself to see how bad it really sounded to others. My family would casually redirect me or suggest alternate options when discussing my future and a family of my own. All of this collectively made me understand that I would never see myself as an old person. No sense in planning things out that far. Plus, who wants to be old anyways.

But hey, I'm still alive. I'm 30 and still surviving. Not only am I surviving, but I'm thriving....And how do I know this?.....I'm now a whopping 192 pounds! Yep, thriving all right! It is true what they say - your metabolism slows as you age. Not only that, but the pregnancy weight you gain also sticks with you for life. And yes, I've even been pregnant - something I didn't think was ever possible. Now I see my two boys running around our back yard and I can only be grateful for all the wonderful things in my life. Not only have I achieved my personal and family goals, but also my educational and career goals. I finished my graduate degree last year and landed my dream job on my 30th birthday. So thriving is an understatement to say the least - I'm living a dream!

Amongst all of these achievements, that I originally thought were impossible, I have maintained my health. I feel guilty and sad when a friend with CF has a difficult time or passes away. Why isn't this me? So many people aren't able to live their dreams and accomplish their goals, so what makes me so special? Everyone deserves a chance.

So I look in the mirror and evaluate my life. I can almost look into my soul and see all of the feelings I have inside. I can look into my eyes and relive the memories that have made me who I am today. I can look into my hair and realize that I'm starting to get grays --- WHAT?!?!?!?!

I never, in a million years, would have thought I'd see this day. Should I pluck them out? Color them? This brings awareness to the fact that I'm not so young anymore and I want to look young. But the other part of me wants to embrace this change. I want to cherish the fact that I'm old enough to get them. I've made it to a point where so many others have not, a point where I hadn't planned for.

I've made it to being....old.

Leah's Bio: I am a 30-year-old cyster, married to my life partner, and we have two beautiful boys ages 4 and 2. I work as an application analyst for medical practices, have an MBA, and also hold a license as a registered nurse. I love playing organized sports, such as soccer and softball. I love spending time outside with my boys teaching them how to play sports and jumping on the trampoline. I also enjoy photography, scrapbooking, and anything crafty really. I am very fortunate that my health has remained stable over the years and my mutations are double DF508.

Note from Ronnie: I can't thank Leah enough for reaching out to me and contributing this blog. We are such a "large" small community and can really learn a lot from each other's story. It's always great to hear stories of cysters my age who are not only living with CF, but THRIVING with CF. Growing up in my generation, we were always left to wonder just what age we would make it to. Now, because of all of the wonderful advances in CF care, we are seeing the next generation carry less of that burden!

My dear friend...

I'm taking this day off of blogging to honor one of my best friends in the CF community.

We'll miss you Marisa and I promise that I will never forget the great times we had together. That first embrace with your sister must have been amazing :)

Want to live a "normal" life? Inquire within.

Guest Post by Ayn Learn

My name is Ayn Learn. I’m twenty-two years old, and was diagnosed at birth with DeltaF508.

Ronnie has asked me to share my story about my college experience with CF. So here goes...

First, a little background. I was a sick baby. I was cut out early and kept in the hospital for five weeks because of CF complications. A meconium ileus, massive mucus and water in the lungs, and a collapsed lung. The doctor’s told my parents that it was a miracle I was alive and that I wouldn’t make it to adolescence.

When I was four, my father started me running. My father is a distance runner and naturally he passed his passion on to his kids. Every morning my dad and I would go on a run, rain or shine. I clocked in serious mileage as a child and adolescent, and by age eight I was averaging 40 -50 miles a week, which is an average of about 6 miles a day. My lungs grew strong. I had amazing lungs growing up, and because of that, I never really thought about CF. The CF doctors would tell me that I had the best lungs of a CF child they had ever seen.

I grew up thinking that my CF was only a mild case. Even though my parents told me that the running was the key to my health, because I had never experienced anything too severe, I didn’t believe them. I didn’t realize that it was running an hour to two every day that was saving my lungs, and my life.

When I was eleven CF began to fight back harder, but I kept running and was able to battle it. I did my nebs everyday and I ran every single day. Combined with yearly piccs, I was able to stay ahead of the game and CF was only an “occasional” disruption to my normal life.

I ran cross country and track and field throughout high school. I had my share of piccs and hospital stays, battling pseudomonas strains all the time, but I never really felt unhealthy. When I graduated, my lung function was in between 77- 86%.

I went off to college and joined my schools cross country team. This was a time for freedom.

I was living in a coed dorm with my best friend, away from home for the first time. I didn’t think I needed to make CF a priority. It was embarrassing, too, doing nebs in front of my roommate and our friends in the dorm. I didn’t want to be labeled as ‘the sick girl.’ So I lost interest in taking care of myself. I was really relaxed with my treatments and did them maybe every other day at best. I did not make my health a priority at all.

Halfway through my first quarter I started feeling bad, and I began to experience extreme difficulty in running. Every step I took it felt like I was drowning. Running became hard and unpleasant.

A few weeks after that, I coughed up blood for the first time. This was terrifying. I had been coughing into tissues all night in the dorm room, and when I woke up I saw that those tissues were filled with nothing but bright red blood. I rushed to bathroom and coughed up mouthfuls of blood, probably at least half a cup total. I had never been so freaked out. I made an appointment with the CF clinic and proceeded to do the stupidest thing I could have done in this situation.

I quit running.

I can honestly say that that was the biggest mistake of my life.

The Biggest. Mistake. OF MY LIFE.

The CF appointment shortly after revealed pseudomonas and a decline in lung function to about the low 60s. I did a picc line while living in the dorms and my roommate and friends watched me ‘shoot up.’ For someone who didn’t want to appear different or sick, I wasn’t too happy.

Sadly, though, this wasn’t the wake up call that I needed.

Once I finished 3 weeks of treatment, I went back to my old habits of inconsistency. I wasn’t running now, and I wasn’t doing the vest (I didn’t have one at the time, never having need for it before because of all the running). I wasn’t doing my treatments consistently. I pretty much became a couch potato, my only exercise being the walk to my classes.

I still believed that my CF was “mild” and that I wasn’t like all the others who would get sick and die if they didn’t take care of themselves. I wanted so badly just to be normal. But I wasn’t. I continued to need more picc lines and soon I couldn’t even make it three months without coughing up blood and needing another picc.

I watched my lung function decline each month. In a year and a half, I watched my lung function drop from about 70% to 47% ... Simply because I stopped running and I wasn’t consistent with my treatments.

My CF clinic stopped treating adults and sent me away to a new clinic two hours away. After this, I felt abandoned. I felt like everyone was giving up on me and now, when I would try to do my nebulizers every day, it seemed to be too late. Nothing was working and my lung function continued to decline. I was sitting in the hospital with yet another picc, when I realized that things needed to change or I was going to die. I finally heard what my parents had been yelling to me over and over, what I had been too stubborn to hear:

That it is running that keeps you healthy. That it’s doing your treatments every day that keeps you alive.

It was both a horrible and wonderful moment. In that moment I realized that all of this was my fault. Sure, having CF wasn’t my fault. But not taking care of myself, slacking off on treatments, and refusing to exercise – that was all on me. But it was also wonderful, because for so long I had felt helpless; I had felt that CF was winning and that there was nothing I could do to stop it. Suddenly, I had the upper hand in this battle, and I knew the secret to winning this CF war. Nebulizers and medicine are only as affective as what they can reach. If your lungs are plugged up with mucus, the medicine isn’t go to be as helpful. If your lungs are too plugged up with mucus, medicine will only help so much. You have to get the junk out of your lungs. Airway clearance, in my case running, is the most important weapon we have against CF.

My first day off the picc I went for a run. It was more like an awkward shuffle and mostly walking after that, but I did it.

It’s taken me a long time to get back into running, and I do mean a long time. It’s taken me probably a full year and a half to get back into a schedule of every day running, because knowing what to do and putting that into practice are very different things. Regardless of my years of running as a kid, running was hard now. I couldn’t jog half a mile when I started back. Running too long would make me cough and throw up and it would make me so frustrated knowing that I had been a decent runner before and now I could barely run one mile. But I kept at it.

It’s taken a long time, but I now run every morning. And every morning, I do my treatments and my vest as well.

When I rejoined my cross country team last year, and was running long distances again, my lung function shot up to 67%. From high 40’s to high 60’s. The proof is in the numbers.

So college was a hard transition. Suddenly, when you get to college, you don’t have your parents there telling you to do your treatments and make you go run or do your airway clearance. You’re on your own and you want freedom and you don’t understand that by ignoring your responsibilities to CF, you’re slowly killing yourself. The way I see it now, is that I want the best possible way of life for myself. I hate feeling sick and I hate my lungs hurting all the time. Airway clearance and nebulizers and the vest, these things were all created so that we could better our way of living – so that we can live longer and fuller lives. And if we have these amazing weapons to fight CF, running included, why on earth wouldn’t we want to use them?

It’s hard to fit in that schedule with school and work, and life often gets way too busy. But I’ve realized that you can’t ignore CF. CF was there affecting my lungs every day, whether I acknowledged it or not. But by acknowledging it, you can fight it. And yes, it is embarrassing going out in public and running while you’re coughing up mucus everywhere and puking on the sidewalk in front of your neighbors. But you’re fighting a battle and you should take pride in the fact that you’re doing everything in your power to beat CF.

Currently I do not run as many miles as I did when I was on the team, and my lung function is down again to about 58%. However, I have now graduated college and have refocused my life to getting longer and longer runs in. My goal is to get up to 10 miles before this year is over, and I know I will succeed. I’m no longer afraid of quitting running, because I recognize that running is more than a health benefit, it’s a way of life. It’s something to take pride in every day. And you feel so much better after you run.

Of course there will always be ups and down, and hospital stays and picc lines and surgeries. But what I’ve realized is that you can’t let those things stop you. You have a picc? So what. You still go for a run, just like you still do your vest and do your nebs. Airway clearance should never stop because you’re sick!

So for those who are entering college, remember to make your health a priority, always. Because once you stop taking care of yourself and lose a good deal of lung function, it’s hell getting it back. It’s much easier to keep up with your treatments every day and exercise every day, instead of spending months in the hospital.

My leaving message to everyone here is to find your exercise of choice, and learn to love it. Airway clearance through exercise is the easiest, most enjoyable, and most effective way of getting mucus out of your lungs. So take your health into your own hands. Make exercise a part of your identity, and CF doesn’t stand a chance. Whether it be running, biking, swimming, hip hop dancing, whatever you want, just do it, every day, and the world is yours.

Ayn's Bio: My name is Ayn Learn. I was born on July 5^th, 1988 in Giessen, Germany. I was premature because of Cystic Fibrosis, and was diagnosed immediately because of CF complications. I moved to America when I was two and grew up in California, where I still live today. I have one older brother, Nathan, and I’m so lucky to have him as well as my parents who are so supportive. I love my family and our three kitties so much. I know I wouldn’t be the person I am today without them. I started running when I was four and ran throughout my childhood. I’ve had twelve sinus surgeries, one intestinal surgery, one deviated septum surgery, and a lot of PICCs and hospital visits, but fortunately CF hasn’t affected my education. I graduated from high school in 2006 and graduated from college this very month, March 2011 in English Education. I’m currently coaching two middle school running teams and I absolutely love it! I now plan to become a middle school teacher and will pursue my Master’s degree in the future. CF has definitely shaped my life, but it does not define me. I have many passions and I try to live my life without letting CF control what I do. I love to sing and to write songs on the guitar, I love to write and to read, I love photography and running, and I love the fact that medical advancements are getting better and better so that we may pursue and enjoy our passions in life!

Note from Ronnie: Thank you SO MUCH Ayn for taking the time to write this inspiring and powerful blog. You make it so clear just how important exercise can be when woven into our daily routine. Like you said, if our desire is to live a normal life, then we must be willing to do what it takes to take care of ourselves. Even though it wasn't always easy, you've showed just how powerful treatments and exercise can be, even if we fall off the wagon for a bit. Sometimes, sharing the bad decisions we've made can be just what others need to hear to kick it into gear! Thank you.