Being Brave

brave
[brāv]

ADJECTIVE
ready to face and endure danger or pain; showing courage:
"a brave soldier" · [more]

synonyms: courageous · valiant · valorous · intrepid · heroic · [more]

That's Oxford's definition of "brave." Brave means different things to different people. To me, bravery is sort of like emotional adrenaline. We don't use it until the situation absolutely calls for it. Some think being brave means masking your fears. Others think it means managing your fears. Then there are even others that think being brave is dependent on the odds one faces. I'm not sure how I would define it.



I couldn't have said it better.

As another doctor's appointment rapidly approaches on Thursday, I have the usual fears. I always am a bit nervous but it certainly doesn't help that I'm on antibiotics for a cough I can't seem to shake while just finishing up with a sinus infection along with medication side effects (vertigo) that I've dealt with for a few months. Add to that, several people that I've known have died, each of them younger than me thanks to this monster of a disease.

I can tell depression is setting in. I find myself often times wanting to sleep or just sit outside and watch my dogs walk around. My at-home PFT tests are down. Today I found myself looking at old pictures of better moments. The last few days I find myself wishing nightfall would come sooner so I can just put my head on a pillow and pass out and not feel guilty about it like I do when I pass out during the day. Of course after a 2 month drought, mother nature picked a fine time to start raining. Depression and rain for me go together like peanut butter and jelly. "Terms of Endearment" better not be the HBO afternoon movie!



Depression is absolutely overwhelming.

There are days when I'm tired and don't want to work out especially recently. There are moments when I feel like my body is breaking down. It's days like these that I have to fight the apathy and find the determination to battle. I have to finish my workout no matter how difficult. I have to go to the gym no matter how much it pains me or no matter how many odd looks I get from coughing. I have to do all of my treatments regardless of how tired I am. I have to be, well, brave.

I'm often asked why I talk so much about cystic fibrosis. Think about this for a second. I do over two hours of treatments a day. That's more time than I spend eating, exercising or even just relaxing. I take 40 pills a day. So even when I have a break from my therapy, I need enzymes in order to eat. You still wonder why my mind constantly thinks about cystic fibrosis? It's always around me. Take my kids. I love them very much but the only way they could be conceived was through IVF which my wife and I had to do because of...you guessed it, cystic fibrosis. Traveling anywhere fun still requires me to pack up two separate bags of medication and therapy equipment. And every 3 months I have to deal with the anxiety of heading to another doctor's appointment that determines whether we keep the course or hospitalize me. No wonder there are days that I feel I'm going insane.

Cystic fibrosis is as difficult a disease as you'll find. I'm at risk for so many things besides a lung transplant. The list is plentiful: sinusitis, osteoporosis, infertility, certain forms of cancer, pancreatitis, CF-related diabetes (CFRD) and liver disease. CF is like the WEB MD cocktail. I'm overwhelmed just by writing about it. And I take more pills than people twice my age. I take so many meds that if I had to write them all down I'd probably develop carpel tunnel.

I talk so much about cystic fibrosis because if I bottled it up inside I would lose it. I know from experience. In college, I lost it. As a grown man in my thirties, I lost it. If I didn't have a psychiatrist, a psychologist, a sponsor and one of the most amazing women in the world who accepted my marriage proposal (Andrea, that's you just to be clear), I don't think I'd ever find my sanity.

Yet still, there's a part of me that won't surrender to cystic fibrosis. It's that same part that keeps me focused on fitness on the bad days and amused by a joke on the even worse ones.

I'm scared about Thursday. The crazy thing is I have no idea how it will go. There are appointments I go in feeling like a champ and leaving as if I have a week to live because the results sucked. I have appointments where I'm shocked how great everything looked because I felt like crap. That's the nature of the beast.

The good news is that I can tell that the sinus infection has gotten much better. The vertigo seems to be near the end as well. Since starting a new antibiotic, the cough has gotten better though it's still present when I run. The key for me is to continue to work out at the highest level and run like there is no tomorrow. That is not just my goal till Thursday...that is my goal forever.

Look. Not every appointment is going to be a great one. I wish it was. Some appointments test a CF patient both mentally and physically. I wish bad appointments were like the ones when I was little when my biggest concern was whether they had the cherry-flavored lollipops available at the checkout counter.

I'm going in there tomorrow knowing that I'm working my tail off and doing my treatments and taking my pills as asked. I even go above and beyond in some cases. I literally can't do much more. If my numbers are down as expected, so be it. The important thing is that I don't feel horrible. I just know that I have an infection. It's up to the doctors to give me the right meds to get better. The ball is in their court.

While CF does scare me, my super-competitive side sees things differently. It's that part of me that wants the odds stacked against me. It wants to be counted out by everyone. It loves the role of the underdog, a role I've had since the day I was born. It's crazy but I think that side of me has probably saved my life and certainly my sanity.

No matter how scary things get, my goal is to keep a positive attitude and kick CF's ass.

I guess that's my definition of being brave.



Tomorrow CF will stand for Courage Found.

Live your dreams and love your life.

Andy

The Many Roles of Cystic Fibrosis

Dear cystic fibrosis,

You have played many roles in my life. Each one has played a pivotal role in the person that I have become.

10. Thief – You stole my childhood away. That’s the one thing that every person holds sacred. While most kids were dreaming of what they wanted to be in life, I was worried I would never live long enough to have a life. On the bright side, I realized at an early age what was truly important.



The face of a child with no childhood

9. Captor – While most people wake up and are free to do almost anything, I have been forced into a monotonous routine for the last 43 years of taking enzymes, administering therapy and inhaling aerosols. On the positive side, you have taught me how to be regimented and that vacation days are only for corporate America.

8. Difference-Maker – You made me feel alienated. I couldn’t go certain places like summer camp or overnight trips without my parents when I was younger. When I was older, I couldn't have kids like everyone else nor could I just pack a toothbrush and clothes when I traveled and be done with it. I've been wearing nebulizer masks since I first put on a diaper. The positive spin is that you turned me into a leader. When you’re different you have two choices. You can cry about it or make the most of it. The first two decades plus of my life, I did the former. I’m proud to say I now practice the latter.



I prefer the term "unique" to the word "different."

7. Enemy – I’ve hated you from my 8th year on when I learned who you were. On the positive side, it has allowed me to put all of my anger into one thing and to use those emotions to fight harder in the gym, raise money to defeat you and to not sweat the small stuff in life except possibly the continuous demise of my sports teams.

6. Coach – You have been yelling in my ear from day one. On the bright side, I can hear you now. Without you, I would not put such an emphasis in fitness and competing in sports. I also believe your coaching has helped me to see that I love to help others who may be in the same predicament as me while fighting a terminal disease. I've also learned to transfer those skills into my own coaching which is something I really enjoy in kids’ sports especially with my own children.



Making a difference in someone else's life makes a big difference in mine.

5. Excuse/Motivator - From my mom sending notes in gym class to me not doing something because I blamed you, you have become the ultimate excuse. I have now reversed that into you becoming the ultimate motivator. Whenever people or statistics tell me I can't do something because of you, that pushes me to try even harder. I've learned to relish the role of the underdog.



January 2016



November 2016


4. Competitor – I always knew that you didn’t take a day off. Now I look at that bit of information as a positive. I will work just as hard every day. Neither of us has had a vacation day in 43 plus years.

3. Comic relief – For years, just the mention of you scared me. You were the elephant in the room. Now I literally tell jokes about you. If I can laugh at my own chronic disease, I can do anything. Laughter cures most things. I know that now thanks to you.



Laughter truly is the best medicine.

2. Killer – I know that most people grow up having no idea how they will die. I’ve known for several decades but I’m not afraid anymore. In a way, it takes the surprise out of things and I've never liked surprises. To be honest, I kind of think I may kill you first.

1. Realist – You’ve taught me that life isn’t always fair and though I didn’t like learning that lesson, it has taught me some invaluable things. Each of us is dealt different cards but it’s how we use those cards that matters. Anger and sadness are normal emotions but don’t harp on them. Take advantage of celebratory days and just accept the fact that not all days will be full of sunshine and rainbows. Just be grateful every day that you get.

For years...

I wanted to blame you, cystic fibrosis. Now, however; I want to thank you. I wouldn’t be the person I am without you and I’ve come to learn over many years that I really like that guy. By no means does this signify that I like you but what it does mean is that I am at peace with you. It also doesn’t mean that I still don’t want to kick you in the rear end every opportunity I get but I’ve accepted the fact that you’ll get a few punches in too. Essentially I may not win every battle but I do intend to win the war.

I know you don’t hear this often but “Thank you cystic fibrosis.”

Without “you,” there’d be no “me.”



I am who I am because of cystic fibrosis.

Live your dreams and love your life.

Andy

25 Common Misconceptions Regarding Cystic Fibrosis

I make a lot of speeches outside of the CF community and when I do, I ask one simple question. "What is cystic fibrosis?" The answer I generally get is "a lung disease." While that's not totally false, it's not completely true either. That's like saying that I am a human being. There is so much more to it.



So without further adieu, here are 25 common misconceptions regarding cystic fibrosis.

25. Cystic fibrosis and depression are mutually exclusive.

False. People with cystic fibrosis and really any sort of chronic disease are at a higher risk for depression. In fact, CF clinics are now screening patients for mental illnesses like depression and anxiety.

24. Cystic fibrosis is just a lung disease.

Somewhat false. It is a lung disease but it also affects a lot of other parts of the body including the sinuses, digestive system, male reproductive system and bone density. It increases risk for certain forms of cancer, male infertility, osteoporosis, liver disease, diabetes (discussed later)and sinusitis. It does not affect one's sense of humor though my family might argue that point.

23. Cystic fibrosis and asthma are the same thing.

False. Want to offend someone with cystic fibrosis? Tell that person that asthma and CF are the same. They're not. First off, CF is much more life-threatening and secondly as already mentioned CF affects more than just the lungs.

22. People with cystic fibrosis meet in person all the time to discuss their disease because CF is not contagious.

Somewhat false. It's recommended that patients remain at least 6 feet apart because of the danger of bacterial cross-contamination. CF patients are NOT contagious to people who DO NOT have the disease but we can pass deadly bacterial infections to each other. It's one of the saddest and most difficult things to understand about CF.

21. CF is contagious because some people get it later.

False. As mentioned earlier, CF is not contagious other than passing bacterial infections from one patient to another. CF is a genetic disease meaning that even if you were diagnosed at 45 years old, you've still had it the whole time. Sometimes people are misdiagnosed or their symptoms were not bad enough to get checked. That's why newborn screening is so important.

20. Cystic fibrosis is difficult but not deadly.

False unfortunately. More than one patient a day dies from CF and more than nine a week. CF patients are also more prone than the average person because of our "crappy" lungs to get lung infections like pneumonia or bronchitis and some patients have even died as a result of these infections. Still the life expectancy was in the teens when I was born and now hovers around 40. In other words, we are making serious progress.

19. Since the median life expectancy is 40, people with CF don't live to 41.

False. In fact, I'm 43. Median life expectancy means pretty much an average. So some patients unfortunately died as children while others have lived into their sixties and seventies. When I was growing up people weren't even living through high school and now some patients have full-time jobs, significant others and even children. As I continue to say, "It's a great time to have cystic fibrosis."

18. 98% of males with cystic fibrosis cannot have children because they are too sick.

False. It's because males with CF including myself do not have a fully formed vas deferens which is the bridge that carries mature semen to the urethra.

17. It's easy to recognize someone with cystic fibrosis.

False. Cystic fibrosis is an invisible disease because it's difficult to tell if someone has it unless you're with them when many of them do their two hour daily treatments/therapies to remove thick mucous and allow airway clearance. It is also difficult to witness them taking their 20 to 30 enzymes each day to help with digestion. Many do both. We are kind of stealth when it comes to taking our meds so if you're not paying attention, you probably won't notice. Sometimes CF can be revealed by seeing a person's IV wires exposed as they need these to receive IV antibiotics or you might see their oxygen tanks which provide assistance to breathe for those whose lung function is severely depleted. CF patients also generally have doctor's appointments every 3 months so if you're a stalker and you follow that person to the doctor at least 4 times a year, that might be an indication that they have CF. Also, if you're a stalker, please get help.

16. Most CF patients are diagnosed because of their cough.

False, CF patients can be diagnosed through blood tests or genetic tests but most commonly are diagnosed with a sweat test that determines how much salt is in their sweat. Sweat tests are generally about 98% accurate.

15. People with CF have a tendency to be licked by dogs because dogs sense that we are great animal lovers.

False. Though I know many of us do love animals like dogs, the truth is that the faulty CFTR or Cystic Fibrosis Transmembrane Reactor will not allow chloride to reenter the cells. Sodium is therefore also blocked since it travels with chloride. In people with CF, salt then travels to the skin's surface with water and is not reabsorbed therefore making our skin really salty and also meaning we can dehydrate very easily. That's why the sweat test is used to diagnose many of us. Animals specifically dogs tend to love the salty taste. If a CF patient allows, you can taste the salt too. If not, you'll have to grab a Margarita.

14. There is no one famous with CF.

False. Occasionally CF can stand for Celebrity Found. Frederic Chopin, the legendary pianist, is rumored to have CF. Other CF celebs include Alice Martineau, a famous singer from the UK, Nathan Charles, a professional rugby player from Australia and Frankie Abernathy, star of MTV's Real World. Many celebrity families have also been struck with CF including NPR's Frank Deford, pro football quarterback Boomer Esiason, pro football quarterback Joe Flacco and legendary singer Celine Dion.

13. CFRD (Cystic Fibrosis Related Diabetes) is the same thing as Type 1 Diabetes.

False. Patients with CFRD typically experience lung function decline, weight loss and an increased rate of death. CFRD does involve severe insulin deficiency but it's not as complete as Type 1 diabetes. CFRD affects 20% of adolescents and 40 to 50% of adults with CF which are much higher percentages than Type 1 diabetes found in people without CF.

12. Everyone's CF symptoms are the same.

False. CF patients are almost always different. Some are pancreatic sufficient and don't need enzymes and some are not which many times is revealed with CFRD mentioned earlier. Some don't experience as many lung issues while others do. With lung issues comes airway clearance like the vest, postural drainage or the acapella. Not only does each of us have different symptoms but we use different meds too. There are different brands of treatments and medications. Very rarely do you find two people with the same routine.

11. Other than airway clearance and taking enzymes, there is no way to improve your health with CF.

False. Studies have shown that better nutrition and more exercise can lead to improvement but obviously airway clearance and/or enzymes should always be the priority.

10. Caucasians are the only people with cystic fibrosis.

False. One in 31 Americans are symptomless carriers of CF. Caucasians are the most common carriers with a 1 in 29 rate. The other carriers include 1 in 46 Hispanic Americans, 1 in 65 African Americans and 1 in 90 Asian-Americans. The number of people who have CF is 1 in 2,500 - 3,500 Caucasian Americans, 1 in 4,000 - 10,000 Hispanic Americans, 1 in 15,000 - 20,000 African-Americans and 1 in 100,000 Asian Americans. 1 in 25 to 27 Ashkenazi (European descent) Jews are also carriers of CF. So while not widely discussed, CF is pretty diverse.

9. The only way to have cystic fibrosis is if at least one of the parents has CF.

False. For a child to have CF, both parents must be CF carriers. Neither has to actually have cystic fibrosis. If both have the gene, there is a one in four chance that the child will have CF, a 2 and 4 chance that the child will be a carrier but not have CF and a 1 in 4 chance that the child will neither be a carrier nor have CF.

8. The U.S. has the highest CF patients per capita.

False. The "winner" is Ireland. While there are about 30,000 people in the US with CF, there are another 40,000 to 50,000 people around the world who fight this disease.

7. Insurance covers all drugs and treatments for cystic fibrosis.

False. Many people wish this was true but because of the cost, things like the vest and breakthrough drugs like Orkambi and Kalydeco are not approved by every insurance company especially in places outside of the United States. Several countries still not approve several drugs/therapy devices because of the cost verses the amount of perceived improvement these methods have on a CF patient's health. We continue to fight though.

6. The term 65 Roses came from the fact that the oldest CF patient was a 65 year old woman named Rose.

False. The term actually came from a kid who was trying to say "cystic fibrosis" to his mom but mispronounced it "sixty-five roses." The name has stuck. Just glad he didn't say 60 live roaches.

5. People with cystic fibrosis experience clubbing which means they get the urge to go out a lot.

False. Not that type of clubbing. Due to lack of oxygen, we experience something called clubbing of the fingers and toes which means the ends get wider and the nails tend to curve downward.

4. Cystic fibrosis is always capitalized.

False. This one is for the grammar police. When writing about cystic fibrosis, make sure not to capitalize it unless you're talking about something like the Cystic Fibrosis Foundation. Diseases like cystic fibrosis, cancer and diabetes are not capitalized. The only diseases that are capitalized are named after people like Lou Gehrig's disease, Lyme Disease or Alzheimer's disease.

3. Being that CF affects only 30,000 people in the US, there are not any foundations around.

False. The Cystic Fibrosis Foundation has approximately 70 chapters around the country. CF is denoted by the purple ribbon and even has its own awareness month. That would be May. One in 31 Americans are symptomless carriers or 10 million people so awareness is definitely needed. In fact, approximately 1,000 new cases of cystic fibrosis are diagnosed annually. Outside the US, there are other big CF organizations including the CF Trust in Europe and Cystic Fibrosis Australia in, well I think you can figure that out.

2. Having a lung transplant cures CF.

False. Sadly there is no cure and since a CF patient's cells still have the disease, having a transplant doesn't remove it. It just alleviates many of the lung problems that a person is having.

1. Approximately 100 genetic mutations of cystic fibrosis are tested when a person is being screened so that's all the mutations there are.

False. There are more than 1,700 cystic fibrosis genetic mutations but the top 100 are the most common and therefore are the ones that are usually screened.

In closing...

So hopefully you know a lot more about cystic fibrosis than you did before you read this post. Cystic fibrosis is quite the puzzle but it's slowly being solved by medical science. Here's to hoping that someday soon the statement "There is no cure for cystic fibrosis" is another misconception about CF.

Live your dreams & love your life.

Andy