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. 

When The CFF Gets It Wrong

by Julie Desch and found on her blog "Sick and Happy".

Six months ago, the National Cystic Fibrosis Foundation announced an update of their infection prevention and control policy.  As stated on their website:

• Indoor events: At any Foundation-sponsored indoor event or meeting, including gatherings such as chapter committee meetings, only one person with CF may be present and he or she will be designated in close consultation with event chairs and key event volunteers.
• Outdoor events: At Foundation-sponsored outdoor events or gatherings, people with CF need to maintain a distance of at least 6 feet from each other.

As stated by the CEO of the CFF, Robert Beale, the intent of the change of policy was “… we want to be sure that we are doing all we can to reduce the risk of cross-infection among people with the disease. The health and well-being of people with CF is our top concern — it is at the heart of all we do.”

At the risk of incurring the wrath of what most in the CF world regard as a beloved organization run by those with only the best intentions for all of us with CF, I would like to respectfully disagree with Dr. Beale, and the Infection Prevention and Control Committee charged by the CFF to come up with an updated and revised version of the 2003 Infection Control Recommendations for Patients with Cystic Fibrosis.

Don’t get me wrong.  The CFF does an amazing job raising money and awareness in the fight to cure CF.  The millions of dollars raised by over 75 chapters and branch offices of the CFF provide the ongoing gravitational force that has led to a tidal wave of new discoveries and lately, some promising new treatments for a disease that we all love to hate.  There is no doubt that the fight to cure this ghastly disease would be nowhere near the finish line, as it appears to be rapidly approaching now, if it were not for the Cystic Fibrosis Foundation.

Indeed, the Foundation is lauded for birthing the concept of “Venture Philanthropy,” in which non-profit fundraising organizations provide funding for small pharmaceutical companies to develop new drugs for orphan diseases…companies that would otherwise be unable to do the necessary research.  The idea is brilliant, and the proof of concept is none other than Vertex Pharmaceutical, maker of Kalydeco, AKA Blue Lightening, or as I like to call it, The Little Blue Pill That Was The Beginning To The End Of CF As We Know It (I need to work on an acronym).  Bob Beale and his team deserve abundant credit for this.

But, you and I are a big part of the fund raising process.  We fund raise.  We educate others.  We tell our stories.  When we were younger and cuter, they were our pictures on the fund raising literature.  After all, this is about us, right?  Yet here is where the CFF has completely left us out of the equation.  We are SO far out of the equation, in fact, that the concept that we might actually be able to decide for ourselves if we want to risk attending a CFF event has not even been considered–at least not that I can discern.

Instead, the CFF will be introducing at their next annual conference, a 92 item document, listing all of the ways adults and children with CF should be protected from cross infection.  Note that the passive nature “be protected” as opposed to “protect themselves.”  I mean what I say and I say what I mean.  This document covers everything from telling us to avoid construction sites to containing our secretions.  They include the directive to avoid all social contact with each other, to avoid car rides with each other, to not share utensils, to stand no closer than six feet from each other if we are outside, and to please not exercise together (they didn’t say please). The Foundation has no hesitancy telling us what to do.

Generally, I can take this all with a grain of salt and know that they are in fact, simply doing their job, and attempting to educate us about the risks that we face when we come in contact with another person with CF whose lung microbiome is different from ours.  NEWS BREAK: Bugs might mix and match, and we could end up on the wrong side of that equation.  I am well aware of that, and I don’t intend to hug you the next time I see you (I am assuming that you, dear reader, have CFTR “issues” as I do).  But, perhaps there are a few of us out there who didn’t see Grey’s Anatomy, and don’t yet know that we are all co-pariahs and need to avoid each other like the plague.  If so, I’m sure the new edition of the best-selling Infection Control Recommendations for Patients with Cystic Fibrosis will not be for naught.

But when they specifically mandate my behavior, I get pissed.

To read this rest of this blog, please click here. You'll want to read the rest of this!!!

Note from Ronnie: Full disclosure, I love Julie Desch. 

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.

The 4 Lessons of Cystic Fibrosis

Guest Post by Katie Broekema

Hello everyone! My name is Katie Broekema, I am a 22 year-old from Michigan who has Cystic Fibrosis. I am going to share a little bit of my Cystic Fibrosis story with you over a two-part guest blog, so after this post stay tuned to see the role Cystic Fibrosis plays in my life currently.

I was diagnosed with Cystic Fibrosis as a young child, but I never really knew what that meant until I was in high school. I was very healthy as a child; I did not even have a nebulizer until I was 16. My parents have always made a special effort to let me live a normal life, including forcing me to run cross country and track for my high school. I did not realize it at the time, but this was the best thing they have ever done for me. It has shaped the rest of my life, and the teams I have been on are the largest influences in my life. It is because of these sports that I have met my best friends, and it was with their help that I am the person I am today. The life lessons and friendships I have gained through this are unparalleled. I ran cross country and track in high school and in college, and this experience has been life-changing. I have had the most amazing support team, and they helped me become the athlete that I am today, with a 5k PR of 19:47 and 10k PR of 42:36. During this time, I have learned a couple lessons that I want to share with you because of their direct application to life with Cystic Fibrosis.

The first lesson is that you need to be willing to put in the work. Just like with running, handling your Cystic Fibrosis takes a lot of time and effort. There are definitely days in college that I did not want to get up and go to 6:00 a.m. practice, but I needed to. Doing the extra work is what made me be a better runner and a real competitor. Similarly, taking that extra time to do ALL your medication and treatments is necessary in order to be at your peak of healthiness. Just like with running, when you put in the extra effort you give yourself the opportunity to succeed. When you do not put in the effort, you rob yourself of the chance to see what can happen. I am not trying to sound pious, preaching this from the point of view that I always do my medicine, because that is not true. In fact, I didn’t do my medicine all the time during my freshman year of college and I got what I deserved; a 2-week hospital stay over Christmas. I have made it my goal to never be in there again because of something that I can control. I know that I will most likely need to go into the hospital again, but I also know that it will NOT be because I was not doing what I needed to in order to stay healthy. Unfortunately there is not a magical formula you can follow to run giant PR’s or to keep yourself super healthy, but doing what you are supposed to at least gives you a fighting chance.  

Along with the lesson about work follows the idea of trusting those who are in charge of you. For running, this is the coaches. Too often I have seen athletes who think that the coach is full of crap, and that they know better so they will do their own thing or not do all of what the coach asks of them. NO! If you are in a position where you do not trust your coach and believe in the running strategies, you need to seriously consider your position on that team; it may not be the right place for you. Similarly, in the healthcare setting doctors and the medical staff are your “coaches”. If you do not agree with the treatments and medications that are being prescribed, then you need to consider finding another place to go. It is not good for anyone if you do not do your treatments, for you or your medical staff. When you say you are doing a specific treatment, but really you are not, you are hurting yourself and the Cystic Fibrosis community as a whole. Medical professionals rely on us to tell them what does and does not help. When they see no change in you and the doctors think that you are doing a treatment but really you are not, it can cause them to doubt the treatment.  That treatment can make a difference is someone else’s life, or even yours, but you were unwilling to give it a chance.

This leads me to the next lesson that I have learned, which is to always believe in yourself. This is YOU we are talking about, who knows you better than yourself? Therefore you should ALWAYS make the final decision about your medical treatment or coaching regimen. This was a running lesson that I needed to learn the hard way. I struggled during my college career running because I did not listen to my body. I spent so much time trying to keep up with the rest of my team that I was not able to compete well during the races. An injury finally forced this rest time upon me, and it paid off when I ran my best time in both the 5k and the 10k during my senior track season. With running, this was something that was my fault. I was in a situation where my coach did not know what was going on with my body. How could he? He does not have Cystic Fibrosis; all he knows is what I tell him. Now apply this to your health in the doctor’s office. The doctors do have an idea about Cystic Fibrosis in general, but they do not know what is going on with you; what would work best for your lifestyle and for you personally. They can only learn this through a discussion with you. As an example, my doctors wanted me to be on Tobi every other month while I was in college to help keep my lungs clear. That is what the medication is supposed to do, in theory it seemed like that would be a good idea but it just did not work with my schedule. I was too busy to commit to that much treatment; and making the commitment actually made me sicker rather than healthier because I needed to sacrifice sleep to do the medication. After having a conversation with my doctor, we decided together that the best plan would be for me not to do the treatment. Without them knowing my schedule and how it made me feel, my incompliance would have seemed like an act of defiance when in reality it was a recognition on my part of what I was able to do at the time.

This leads to my final lesson, find a group of people who believe in you and are willing to be your support group. For me, my running support group and my Cystic Fibrosis support group are one and the same. This is actually very emotional for me to write this now, because they are some people who have been so significant in my life that I know I will never able to thank them enough for what they do for me. I challenge you all to find a group of people like this in your life. Find those people who push you, and make you better than you are now. They are the ones who will help make you strong and able to push past the difficult parts of this disease. This group wants you to succeed, and you want to succeed for them. Because I know once you find a group of people like this, you will be able to grow to your full potential in whatever you do.

These lessons have been instrumental in my life. Although some lessons may have taken me longer than others to learn, I know I am where I am today because I learned how to put in the extra work, I began to trust in my doctors and my coaches, I believed in myself and my abilities to discern what is best for me and I had an amazing support team. I firmly believe having these four things can also help you succeed in whatever you choose to do, like it helped me with my running and taking care of my Cystic Fibrosis. These lessons have also been helping me out with my schooling, which is something I am going to talk about in my next guest blog. Are these lessons something that you feel you have a firm grip on, or do you struggle with some of them?

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

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.

Looking for some inspiration?

I've had the honor to present you guys with many guest posts over the years from various booty kickers in the CF community. If you haven't had a chance to read them, or maybe you're new here, I would highly recommend checking them out!! I've highlighted a couple below to get you started, but you can also use the "tag cloud" on the lower right side of this website and click on "guest post". After you've read a couple, come back here and tell me what you think! Any favorites?

Kicking Cystic Fibrosis!!

My "Declaration of Compliance"

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

"I can never imagine myself running!"

These are great examples of what some cysters and fibros are doing in our community to stay healthy. You'll see that they all have something in common too, but I won't spoil it by giving you the answer.

I can't thank the peeps who have contributed to this blog over the years enough. Their stories are always inspiring and it gets a little dull for all of us if we only post about me and my family. With that said, if you are interested in being featured on RSBR, please shoot me an email at ronnie@cysticlife.org. Let me know what you'd like to write about. Can't wait to hear from you!!!

Kalydeco: First Week In

Guest post by Angie

WARNING: To those of you that are not my fellow cysters and fibros or caretakers of aforementioned cysters and fibros, this post contains discussion about mucus… wait, if that would bother you why would you be reading a blog about CF? Never mind.

I took my first dose of Kalydeco on a Tuesday night.  I took it and went to bed.  It’s hard to know if Kalydeco was the cause or not, but I had a hacky cough that is usually brought on by drainage in my throat all night.  When I got up in the morning my throat felt really dry and scratchy.  The hacky cough continued throughout the day.  The next day (Thursday) during my morning treatment I noticed that the mucus I was coughing out was thinner and lighter in color.  Could this drug already be making a difference or was it just wishful thinking? In the short time I had to research the effects of Kalydeco, I don’t think I took any action to manage my expectations.  I think I was torn between expecting to see a difference right away and thinking that would not be possible it would be more of a marathon than a sprint.  After all, it’s taken 36 years for my lungs to get to their current state, so they aren’t going to improve overnight, right? 

On Saturday I was contemplating how my first few days on Kalydeco had gone.  I realized that I was coughing a lot less than I normally do.  This was a bit concerning because my family and friends have always been able to locate me by the sound of my cough.  If my cough had decreased, how would anyone find me when we were out shopping?  This is a problem I am more than glad to have!! I also noticed I had more energy.  Don’t get me wrong, I still need my weekend naps, but I think that is just because of my love for napping.

 

It’s funny, but I noticed the most changes during my first week on Kalydeco.  I’ve continued to feel this way, most of the time.  My cough still increases when the weather changes.  Since I live in the Midwest, the weather is known to change anywhere from 20 to 30 degrees in a 24 hour period.  This has always made my chest feel tight and my head feel like it was going to explode.  Since being on Kalydeco, I have noticed my cough increases with these weather changes, but I’m not having sinus headaches.  I have also had more big, old looking globs come out of my sinuses and lungs.  I take that as a good sign that some of the old stuff is breaking up.  The true test for me will be when I have my first PFT since starting Kalydeco.  I have a feeling we will be celebrating once again when that day comes!

Next Update: This truly is a wonder drug: I’ve started running (although I use the term “running” loosely)!

Note from Ronnie: If you missed Angie's first blog about discovering that she did in fact carry the G551D mutation after many years of thinking she didn't, click here (you can also find her bio). She also gives an update about her emotions after/during receiving her first bottle of "the hope diamond" here. 

Kalydeco: The Hope Diamond??

Guest post by Angie

Who knew such fanfare could surround such a tiny bottle of pills?  I certainly didn’t, but I do now! After I received the news that I had one G551D mutation, my clinic sent my information along with a prescription into Vertex (the company that makes Kalydeco) so they could begin the process of making sure my insurance would cover the drug.  My clinic told me that it would probably take about two weeks to get Kalydeco because of the authorization process with my insurance and the pharmacy processing time.  I had already waited 36 years, what was another 2 weeks?  Also, this wonderful news just happened to hit the day before we were leaving on a week’s vacation… so let the party begin!

About a week after my clinic sent my information to Vertex (and towards the end of vacation), I received a call from Vertex letting me know my insurance would be covering Kalydeco.  They also told me that my copay would be $250 for a one month supply.  Not really a surprise as I already pay this for a couple of my other drugs.  However, the case manager also told me that with their copay assistance program my copay would only be $15 a month!!  Now, I know everyone was suffering from a little sticker shock after Kalydeco received FDA approval and we heard how much the drug was actually going to cost.  Vertex immediately stated that no one would go without the drug.  It seems they are living up to their end of the bargain.

Once my insurance had given the thumbs up, Vertex sent the script to the pharmacy I had chosen.  There are only a handful of pharmacies that can distribute Kalydeco.  I chose to receive mine from the CF Services pharmacy.  At the beginning of the next week (after arriving home from a fabulous vacation!), I received a call from CFS to verify my shipping address and telling me that I had to sign for the drug when it arrived.  I confirmed the delivery address and was told I would receive another call just to verify the address one more time.  Were they shipping me the Hope diamond?!?!  I received the second verification call and was told my prescription would be shipped that day via overnight delivery so I would have it in my hot little hands the next day!!

I am not an overly emotional person by any means.  Some might say I’m not even a mildly emotional person.  But, the weeks that led up to Kalydeco arriving at my door were very emotional.  It was one of the most wonderful feelings in the world.  The day that I started Kalydeco was also quite a celebration.  We toasted the arrival with some tasty champagne and my husband prepared a wonderful dinner.  I decided that I would take my doses at 9am and 9pm since you are supposed to take it every twelve hours.  You also have to take Kalydeco with fatty food.  That is not a problem for me!! My inaugural dose was taken with double stuff oreos and milk.

 

Coming next week: My first week on Kalydeco!!

Note from Ronnie: If you missed Angie's first blog about discovering that she did in fact carry the G551D mutation after many years of thinking she didn't, click here. The story is quite amazing!!