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Chad’s Perspective: 

Starting this blog post was a hard thing for me as putting this down on paper is not easy for me. When Dan asked if I would be willing to share something about CF to help promote Cystic Fibrosis awareness month I was all in but actually writing it was a whole different story. Where do I start? What do I want to share? Should I talk about the gift of CF, the challenges of CF, what life is like with CF, life with kids and CF? All questions that kept swirling in my mind so I thought I would just start and let it lead where it may.

I was diagnosed at the age of three and at that time my parents were told that I would most likely not live long enough to see my 12th birthday. Well 32 (almost 33 years) have passed since then and we are still here kicking at 35 as a college graduate, working full time, married to the most incredible woman I know and a father to two of the kindest and funniest little humans (I may be slightly biased but they are the best!).

From hospital stays, to missing school, to surgeries to today’s battles with depression and anxiety, the journey has not been without its struggles and its challenges. But with the struggles comes a true appreciation for the little moments and the little victories that happen every day is what amazes me. Growing up, I played sports constantly, never really thought about the future as honestly, I didn’t know what it was going to be. I didn’t think about the consequences. I mean, I managed a bar in a college town when people could still smoke indoors.  But, if I had not done that I would not have met Julie. Yes, did that have a negative impact on my health, in the long run, sure it did, but what would my life be now?  Most likely without Julie and my kiddos, the support system that focuses my fight and encourages me to keep fighting for every breath.

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Yes, it is a fight.  There are days when it hurts to walk up a flight of steps or you have to stop when walking from your car to the parking lot. There are moments when you are by yourself with your kids and you have a panic attack scraping ice off your car.  The panic is real because it is hard to breathe and so you have to ask them to get out of the car and come in the house so daddy can calm himself down.  And it’s hard to explain it to a 6 and a 3-year-old in a way that makes dad “normal” and keeps them feeling safe. The fight and the struggle are constant.  

It is not easy to remember which pills you take this month and which ones you take next month. It’s not easy to remember to order the meds far enough in advance that they can be processed and shipped so you don’t miss a day. It is not easy to say no to friends, your wife or your kids because you have to do treatments or you are just in so much pain that you need to sit or you simply don’t have the energy.

It is a struggle to come to grips as you watch your lung function fall for six months with no answer as to why or no sign of it stopping. It is a struggle to come to grips with the fact that a life-saving transplant could be the only thing that saves you and allows you to continue living a life that you had cherished for so long. It is painful to watch loved ones struggle to understand the what we are going through and to face the wrath of our anger or the sadness of depression of it when it all becomes too much to handle and our hard candy shell comes cracking apart.

But for all the struggles, the pain, and the constant fight to take a simple breath there is a hope, a love of life and immense strength that comes with CF. There is an ability to find the smallest of good and joy in a situation that to others would be so dark. There is an appreciation for that simple breath, the ability to let air fill the lungs that are so often taken for granted. It’s crazy to think that breathing can be taken for granted but stop and ask yourself when was the last time you really noticed your belly and chest rising as your lungs filled with air and stopped to wonder how incredible the act of breathing really is.

Having CF (and with the help of meditation) has allowed me to stop and take stock in the fact that I woke up and filled my lungs full with air (full being the relative term), to love the moments when the windows are down and the sunroof is open and the music’s too loud and I look crazy signing at the top of my lungs (usually to Mumford and Sons, Widespread Panic, maybe some Michael Jackson or anything else with a beat and deep bass). To cherish the moments in the early morning hours when I hear the stampede of little feet running down the stairs and across the living room through the kitchen and down the next set of stairs to the basement to say “good morning” and hang with dad (who is doing treatments) before breakfast and school.

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Some would say that “they have CF but CF does not have them,” I would say that yes I have CF and CF does have me, it helps defines who I am, it decides how I live my life and the decisions I make each and every day. But that is a good thing.  It has not stopped me from doing the things I have wanted to do or love the people that I love. I believe that in all of us with CF it creates a drive to outlast this disease as best we can and embrace the moments that make up each breath!

As I have sufficiently rambled my way through this (and I thank you for making it this far), I write all this on the page not looking for sympathy or empathy but to spread awareness into the struggles of cystic fibrosis (and all those it touches).  I hope I was able to highlight the incredible resilience, compassion, and strength of the CF Community. I will leave you a simple question, “What will you do today to make someone else smile?”

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“But for all the struggles, the pain, and the constant fight to take a simple breath there is a hope, a love of life and immense strength that comes with CF. There is an ability to find the smallest of good and joy in a situation that to others would be so dark.”  – Chad

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 Eric’s Perspective:

Most chronic, invisible diseases are like an iceberg. Even once you think you’ve got a complete picture of the shape of the monster, you haven’t seen the largest, scariest part. CF isn’t something that just flares up sometimes and lets you live your life the rest of the time; it’s also not something that can be solved by living a healthy lifestyle. Some CFers may prolong the inevitable through exercise, organic veggies, and meditation—but regardless, the disease will have its day. There’s no beating CF, no remission, no prevention, no cure. It seeps into every second of every day of your life and dictates every decision you make, from work to relationships and everything in between.

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If you’re lucky enough to find yourself in a supportive relationship as a CF patient, you’ll see that your disease drives most of the major decisions you make as a couple. You and your partner will make decisions about jobs, money, home, children, pets, vacation, and just about everything else by accounting for CF. 

Love is a precious and unexpected gift in the midst of the CF trials. Knowing you aren’t alone makes all the difference in the world. And when the right person comes along, they not only give you a reason to fight all the harder but help you with all of the many everyday tasks required to stay healthy and breathing. I was blessed to find Marissa, an unbelievable loving, caring, prepared woman who carries my heart, gives me hope, and takes care of me when I need the extra help.

Though it may seem impossible, CF has a positive influence. You learn to ignore the unimportant stuff and the judgment of “successful” couples and let go of visions of a “perfect” family. All of the decisions that other couples make easily become major turning points for a couple affected by CF, so we put a lot of thought, planning, communication, and sharing into our choices. Honesty is a given because a lot of simple things can be life or death.

You spend a lot more time together than many young couples, because of a) you’re stuck in one place doing treatments/at the hospital/tethered to your oxygen, and b) you’ve learned to truly appreciate the time you have together.

Every day is a reminder of life’s fragility, between the infections, hemoptysis, and gasping for air during vicious coughing spells. Having to consider the implications of an incurable fatal disease also forces you to recognize and be grateful for the time you have together.

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“Love is a precious and unexpected gift in the midst of the CF trials. Knowing you aren’t alone makes all the difference in the world.”Eric

Ashley’s Perspective:

Living with a chronic illness is never easy, especially Cystic Fibrosis.  I was born in Denver, Colorado and diagnosed with Cystic Fibrosis at birth.  Colorado was the first state in the nation to include Cystic Fibrosis in newborn screening.  From a young age, Cystic Fibrosis affected my digestive system and my lungs, however as I became older, CF began to affect me in other areas.

In 2011, I was diagnosed with Cystic Fibrosis Related Diabetes.  This was a shock for me at first because I never thought I would have to experience taking insulin.  Over time, I began to get used to my new routine with taking insulin.

In 2015, I was diagnosed with Cystic Fibrosis Liver Disease.  I never saw this coming.  At this time of the year, I was the healthiest I had ever been and before I knew it I was being rushed to the hospital.  They discovered I had CF Liver Disease because I was bleeding internally in my esophagus.  The blood could not flow through my liver and backed up to my esophagus.  They had to perform a TIPS Procedure, seal up the bleeding vessels, and put me on the liver transplant list. 

I was in the hospital for a month and a long and slow recovery.  I have learned from CF that things can be thrown your way and that sometimes there is no way to be prepared.  Being only 19, I have experienced more than I thought I would experience.  Then again, it is different for every individual with CF.  I decided that because of what I have gone through, that I want to be able to share my story, advice, and show that it is possible to live with a genetic illness and still have a positive perspective.

With this in mind, I created a YouTube channel called Ashleys Roses as a place for others to learn about to CF, feel that they are not alone, receive advice, and laugh a little.  One thing I emphasize all of the time is, yes, Cystic Fibrosis is hard!  Sometimes we think there will never be a cure or sometimes we feel like everything is always being tossed at you, but we are warriors, we are Cystic Fibrosis Warriors.  It may feel like we are always in war, but we never go down without a fight.  Sometimes it is hard to see it in the moment, but there is always a positive, you just have to look for it.  Remember, CF does not define who you are.  You are a strong and amazing individual.

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“It may feel like we are always in war, but we never go down without a fight.  Sometimes it is hard to see it in the moment, but there is always a positive, you just have to look for it.”  – Ashley’s Wilson

A Point of View

May 22, 2017 — Leave a comment

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Perspective is the way we see things when we look at facts from a certain distance, it allows us to appreciate their true values.  These values can lead us to different illusions, giving us the ability to remove the blindfold that can overcome our lives at times.

The subtitle of my blog is “A Perspective of Another Life”, the main reason for starting my blog was to help educate those that didn’t know about Cystic Fibrosis and Transplantation. I wanted to show people that a “normal world” to some could be seen in a totally different perspective by another. It moves you to ponder,” what is normal”? What is normal for the spider is chaos for the fly.

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Since my 15-year lung transplant post, I’ve been in touch with a lot of CF’res and Transplanties sharing stories as well answering a lot of questions for those that are just entering the world of Cystic Fibrosis or Transplantation.  This has been a learning curve enlightening me that different kinds of CF mutations create different kinds of medical conditions and results. This knowledge has opened my eyes to just what Cystic Fibrosis is and how it can affect your life. It has shown me the importance of communicating and educating people, even family and friends who don’t always understand the consequences of what living with CF is like.

This gave me the idea to ask a few Cfer’s and CF Caregivers if they would be willing to write something they felt important or could share a little part of their story and show how Cystic Fibrosis has affected their life.  This research has allowed me to gain a new perspective for my readers, giving them the ability to see how important it can be to spread the awareness of Cystic Fibrosis. Let the reader enter the world of those affected by Cystic Fibrosis, what better time than during May, Cystic Fibrosis awareness month. It is my goal to end the month’s awareness off with readers not only learning new facts, but also gaining insight by sharing personal experience stories.

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The stories are heartfelt some deal directly with CF while others cross paths into transplantation, the importance of a good support system including friends, family and significant others in our lives.  The stories touch on depression and anxiety and the daily challenges that living with Cystic Fibrosis present. 

I hope these different perspectives help you as the reader realize that we do not see things as they are; we see things as we are. By entering our world for a moment, stepping into our shoes you might understand the challenges that we face everyday living with an incurable disease.

Learning is about seeing things from a different perspective. I like to think my role in life is to help people improve their vision by removing their rose-colored glasses it is then that reality becomes an education.

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CAN YOU SEE ME NOW

May 16, 2017 — 1 Comment

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You didn’t cause it. You can’t cure it. You can’t avoid it. You can’t ignore it. You can’t control it. You try to manage it while concealing it from the “normal world”.

Cystic Fibrosis (CF) is often called the invisible disease. Invisible illnesses are conditions patients have that are not obvious when looking at them. People with invisible illnesses face a lot of prejudices. Accused of faking, lying or exaggerating they are often misunderstood.

“Just because you cannot see a person’s illness doesn’t mean they don’t have one. Someone may look “OK” when they really are not. Invisible illnesses often have no cure with patients having to take medication for the rest of their lives to help control and manage their symptoms.”

Take a look at me for instance, coming from an outsider’s point of view. If you saw me walking down the street:

He was walking with a group of friends when I spied him. I noticed him immediately because of his short stature. At first, I thought what is this young boy doing hanging out with these older kids. The closer he came I realized that he wasn’t as young as I thought, in fact, he was mature and probably the same age as his friends.

They seemed very comfortable with one another laughing and joking as they sauntered along. He had a presence and seemed to have no problems keeping his stride even though his legs were much shorter than his friends. Little did I know at the time that he was a warrior; born with cystic fibrosis he challenged life on many levels.

Other than his size I had no idea that he was struggling just to breathe. Living with an invisible disease you quickly learn how to compensate. No one wants their disease to own them. As I watched them disappear down the road an old lyric came to mind.

You can’t judge one by looking at the other.

You can’t judge a book by looking at the cover.

Oh can’t you see, oh well you misjudged me.

I look like a boy, but I have C.F. undercover.

You can’t judge a book by looking at the cover.

There’s a story behind every person, a reason why they are the way they are. Think about that before you judge someone. There have been times that people have doubted me when I was not well. I’ve been told, “you look alright to me, what’s wrong with you? What’s your problem?” What they don’t understand is that they’ve got it all backward; I am not well, rather I am faking being well.

Before my lung transplant, I frequently had chronic chest pain caused by excessive coughing. Coughing was my body’s natural mechanism to try and rid my clogged lungs and airways of thick sticky mucus. Coughing helped to relieve the tightness living inside my chest. At times it felt like as if a slippery serpent was wrapping its scaly body around my lungs, strangulating and constricting my breaths. The wheezing that would escape from my lungs would be a hiss of a rattle.

The cough woke a fiery dragon, burning the lining of my lungs. If that wasn’t enough to suffer through, my stomach muscles would feel like a punching bag. The sheer pressure of coughing weighted my shoulders down and caused a deep stabbing ache between my shoulder blades. This is how I would start my day, in constant pain. I hid in the pain, showing no outward signs of the torture I was battling. The physical pain was hardwired into my system; I simply faked it pretending to be well all the time.

I appear as I want you to see me. I paint on my ‘I’m OK’ face every morning before my day and walk around with a smile, but the truth is that I am in chronic pain, always struggling, but you will never see that because I do not want to appear in that light. You may believe I am faking being sick, but I am actually faking being well, and people need to not be as quick to judge.

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The only person who could detect I was getting an infection was my Mom. She could see through my smoke screen. She by-passed the “I am fine mom, don’t worry” or “No, we don’t need to waste our time going into clinic the team isn’t going to find anything.” Her radar was incredibly accurate; before I knew it I would be in the car heading for the hospital to be admitted for a two-week course of heavy duty intravenous antibiotics.

With breathing restrictions, physical activities weren’t always easy for me.

There have been times when I needed oxygen 24/7. The sheer act of walking was a major task. I used my disability parking permit when I was out. Several times I returned to find handwritten notes tucked into my windshield stating that I abused the system. Nasty little sentences slamming me for misusing a disabled parking space. Why would someone as young and healthy as me require a disabled parking pass? In fact, I remember once coming back to my car only to find a note which read: “Someone in their prime age, has no use to abuse a benefit like that. You should be ashamed of yourself”.

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Many of those living with an invisible disease’s can relate with this experience;  even though you know you’re sick, you know as soon as you step out that people will see your lack of a wheelchair or crutches and do a double take of the handicapped spot you’ve parked in.

Yet the truth is that about 96% of disabled people have what are called an invisible illness. Because they’re invisible, people assume we’re faking all the time. The scariest thing is that many painful chronic illnesses go without acknowledgment or recognition from society, even disability circles. It can even get to the point where the illness controls that person’s life. When it’s such a major thing to that person, it’s not helpful when people dismiss it as a fabrication.

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“I didn’t realize disability had an age limit… someone should really tell my body that.”

Thinking on this topic, yet another painful memory floats up inside my head. I was learning how to drive. It was a hot humid summer night, my older brother Chris had taken me out for a lesson. We had stopped at a McDonald’s for ice-cream. Returning to my car I saw a woman make a gesture to her younger daughter regarding my size and the fact that I was driving. She openly pointed to me, motioning her hands to emphasize my small stature. While she was mocking me behind the wheel she was laughing. Not only did this make me feel disrespected it also made me mad. I secretly wanted to go over to the woman and tell her how insensitive she was. I wanted to educate her about CF and tell her how fortunate her daughter was to be healthy. But instead I got into my car and drove away. This memory sits with me. The worst thing you can do to a person with an invisible illness is, have them feel like they need to prove how sick they are. 

Depending on the mutation of the gene, Cystic Fibrosis can play a large part in children’s growth. Ciliated epithelial cells in the person that have a mutated protein lead to abnormally viscous mucus production. The poor growth in children typically presents as an inability to gain weight or height at the same rate as their peers and is occasionally not diagnosed until investigation is initiated for poor growth. The causes of growth failure are multifactorial and include lung infection, poor absorption of nutrients through the gastrointestinal tract, and increased metabolic demand due to chronic illness.

Nutritional failure in cystic fibrosis is multifactorial. Malabsorption of fat, protein, and fat-soluble vitamins is a result of insufficient production of pancreatic enzymes, which can be exacerbated by bile salt abnormalities in the presence of concurrent liver disease. Progressive pulmonary infection can lead to increased work of breathing, reduced appetite, and increased caloric needs from inflammatory catabolism. Other factors that affect nutrition include cystic fibrosis–related diabetes mellitus, altered motility of the gastrointestinal tract, and small bowel bacterial overgrowth.

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Pancreatic insufficiency results in malabsorption and maldigestion of nutrients and fat-soluble vitamins. In fact, cystic fibrosis derives its name from the cysts and fibrosis noted in the pancreas of patients with the disease. Pancreatic enzyme replacement therapy (PERT) and optimization of nutritional deficiencies can prevent growth failure and improve other outcomes in patients with Cystic Fibrosis, including quality of life, resistance to infection, and chronic lung disease, which can lead to longer life expectancy. The type of the genetic mutation causing cystic fibrosis determines whether a patient is pancreatic-sufficient or pancreatic-insufficient, although approximately 85% of patients are pancreatic-insufficient by the age of 1 to 2 years. Pancreatic sufficiency in the setting of Cystic Fibrosis is a risk factor for recurrent pancreatitis, and recurrent pancreatitis can often be a presentation for the diagnosis of cystic fibrosis

CF has me forever ensnared in a childlike body. Short stature, thin, I will be the eternal kid due to the mutation that prevented my growth. My body wasn’t getting enough nutrients at the time I hit my growth spurts and puberty. Being in my body isn’t always easy. Even though I am a grown man I struggle to live life as “normal” as possible. It’s made daily tasks a challenge as well I feel like I’ve missed out on parts of life. As I journey throughout life I’ve learned to adapt. Blend in and even though what seems like a simple task I have taken on the challenge to overcome it.

Living with an invisible illness, it’s difficult and often pointless to try to explain to someone who has no idea of the daily struggles you face. Feeling the pain, feeling unwell, feeling awful on the inside whilst looking perfectly fine on the outside is a hard act to keep up. Putting on a brave face for everyone wears you down. There are many forms of invisible illness other than CF. The invisible sufferers include Anxiety, Alzheimer’s, Chron’s, Depression, Diabetes, Epilepsy, Fibromyalgia, PTSD and many different kinds of Cancers.

Just because you can’t see it, doesn’t mean it doesn’t exist.

We learn not to talk about it! We’ve learned not to talk about our illnesses. Out fear out of disbelief, patronizing phrases or yet another unhelpful answer, and because we’re sick of being made to feel over-dramatic as if it’s all in our head.

We need someone who’s willing to listen. When I say listen, I mean really listen. Not give us answers to make us feel better at the time or daft answers that, while I’m sure are said to be helpful, only make the situation worse. We just want a friend who’s going to say ‘I’m here for you’ when we need them most

“Ask questions before you judge me. I’m happy to share my story and want to educate others. My challenges/pain is real so if you don’t understand, get to know me.”

The best thing you could say to someone with an Invisible illness is to tell them “you believe in them”. Believing is acknowledging you understand what they are going through. Today we have no reason to judge anyone as we don’t know what their story in life is. Judging a person does not define who they are. It defines who you are. So remember to keep an open mind.

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We may not look sick, but turn our bodies inside out and they would tell different stories.

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BREATHE

May 8, 2017 — Leave a comment

IMG_8923INHALEEXHALE, now again INHALEEXHALE, and REPEAT, it’s easier said than done for some. Breathing is an involuntary act which most people do not think about and take for granted. To someone with Cystic Fibrosis that breath you just took would be a gift!

Many people have asked me what’s it like living with Cystic Fibrosis, does it “Hurt”? I’ve never given a clear answer as there isn’t one. I can describe what Cystic Fibrosis is to me, or how it feels like to me. But you should know, there isn’t just one kind of CF gene there are over 1,900 different types of mutations of the gene. Some are common and others are rare and found only in a few people. Certain types of are associated with different symptoms of the disease. For example, some mutations are more likely to affect the gastrointestinal tract than the lungs. However, knowing an individual’s CF mutation cannot tell you how severe that person’s CF symptoms will be.

If you’re new to the scene of Cystic Fibrosis let me enlighten you:

Cystic fibrosis is a serious genetic condition that causes severe damage to the respiratory and digestive systems. This damage often results from a buildup of thick, sticky mucus in the organs. The most commonly affected organs include the lungs, pancreas, liver, and intestines.

Cystic fibrosis affects the cells that produce sweat, mucus, and digestive enzymes. Normally, these secreted fluids are thin and smooth like olive oil. They lubricate various organs and tissues, preventing them from getting too dry or infected. In people with cystic fibrosis, however, a faulty gene causes the fluids to become thick and sticky. Instead of acting as a lubricant, the fluids clog the ducts, tubes, and passageways in the body. This can lead to life-threatening problems, including infections, respiratory failure, and malnutrition.

In my case, the mutation I have has affected my respiratory system, nose, hands/feet, brain, reproductive system, bones, pancreas, kidneys, gastrointestinal tract, and exocrine glands. So to make it clear CF doesn’t just affect your lungs, it can have an effect on the entire body. In other words, Cystic Fibrosis is a drawing of the straw kind of life.

May is Cystic Fibrosis Awareness Month!                                              

Throughout the next few posts, we will journey into the cause and effects CF can have on the body and personal life. Be sure to check back in this month for more interesting posting regarding Cystic Fibrosis.

But first, let’s try an experiment a challenge if you want to call it that. This simple exercise will give you a feeling of what it’s like to breathe like me or someone with CF.

The Challenge: Obviously it is very difficult to experience the full range of problems encountered by people living with CF, like chronic shortness of breathconstant coughing, and debilitating fatigue. It is not actually straightforward for most people with CF as the build-up of mucus in the lungs means that less oxygen is available, which causes problems with breathing normally. However, by breathing through a narrow straw, you can get some sense of what it feels like with reduced lung function. This is amplified during intense moments when breathing is a struggle such as a heavy coughing fit. There is no way for anyone to know what it truly feels like to be another person, this is one of the closer experiments to experience what it’s like to breathe as someone with CF. It’s very simple to do and there are only a few steps.

WARNING: At any time you feel light headed or extreme discomfort, STOP! 

  1. Find a medium sized drinking straw, something you would find at most restaurants/bars the longer the better, not a straw that has a larger diameter. The effect will not be as intense.

  2. Stick the straw between your lips (make sure there is a good seal around the straw and your lips), plug your nose and breathe through the straw for 60 seconds.

Several seconds in you might not think it’s so bad, but then around the 25-30 second mark, you begin to consciously think about the lack of air pouring into your lungs. Around the 35-second mark, you wonder if it’s almost over. By 60 seconds your lungs are beginning to panic a bit and your brain is screaming that you should release your lips from the straw. People with CF don’t get to stop; it’s why each breath seems so important/valuable. There can be days it feels like it takes everything just to breathe but instead of panicking we have to move forward and remind ourselves to breathe.

Describe in one word how it felt to breathe through the straw, now challenge your friends.

With that one word, I welcome you to post your response of the straw experiment please take photos of you and your friends during this moment and post on your social media account allowing others to be educated of your experience. Use the hashtags #strawchallenge #cysticfibrosisawareness #fight2breathe #Cysticfibrosis  #CF

Now imagine having to cope with that feeling when you are climbing the stairs, running for a bus or in the case of a child, just trying to keep up with your friends while you play.

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If you didn’t find it challenging enough, try climbing a flight of stairs, walk around the room for a while, include some kind of everyday activity, jogging in place for a minute. All while breathing through the straw with your nose pinched.

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Meet Devin Featherstone, Devin is a good friend that has been doing his part with Cystic Fibrosis Awareness. He accepted the straw challenge while hiking up in the mountains. Take his word for it”This Sucks”!

The straw challenge probably is the closest activity to experience what it’s like to breathe and live ith CF. Breathing isn’t the only challenge we CFer’s have to face and live with. There’s depressionanxietyscene of lonelinessstress,  painbad cough,  infections,  medical treatments, and nutrition just to mention a few.

I’ve experienced my challenges with all of the above symptoms. I have faced some really difficult times living with CF. Even though they may seem like dark times at the moment, I try and look at them from a positive point of view and transform them into lessons. Some may leave scars; scars are but evidence of life, scars are not signs of weakness, they are signs of survival and endurance that we have fought for something we wanted in life; LIFE IT”S SELF. I’ve started to learn to use my scars to educate others that there are ways to heal, to live, bring awareness.

Through the completion of each survival, I gain a lesson that I hope to educate others with.

I’ve walked in the darkness so that others may see the light.

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In For An Oil Change

February 5, 2015 — 11 Comments
Getting hooked up to the plasmapheresis machine.

Getting hooked up to the plasmapheresis machine.

 It has been almost 5 years since my second double lung transplant (March 2010) and 13 years since my first (March 2002). It is truly amazing how fast the time has flown by. I’m here because someone gave me the gift of life. Not a day passes that I do not stop to reflect how grateful I am. Their gift, the most unselfish gift ever, has given me the opportunity to live; to breath, to laugh and create memories.

 In the past 13 years, I have done and seen more than most people will in a lifetime. I have watched my family grow in size, enjoyed adventures all over the world, made new friends and even got my own dog (the best dog in the world – Sheba). The time has allowed me to blend into society – to feel somewhat normal and not have to worry about any of my old arduous C.F. treatments.

 Not a day goes by without my sincere gratitude and appreciation to the families that have given me this gift. That’s truly what it is, a gift of generous portions that have allowed me to share time with my family, friends, and others, whose connections I have made through social media.

 Only about 55 percent of patients survive five years after transplantation. Those rates are a little better at networking hospitals, where about two-thirds of patients can expect to survive that long. Nationwide, only a third of patients live 10 years. It is unclear what, exactly, goes wrong after the first year.

 Most patients die of what is known as chronic rejection, which causes the airways of the lung to deteriorate slowly. Doctors do not yet know how to prevent or stop this process. This is the main reason why some people are re-evaluated for a second lung transplant. The procedure is the same as the first one but with more variables. The patient still has to go through a series of tests to see if they are physically and mentally able to handle a large operation as well the medication given after transplant can lead to high depression!

 Over the last few months, my PFT’s have been slowly falling, Pulmonary function tests are a group of tests that measure how well the lungs take in and release air and how well they move gasses such as oxygen from the atmosphere into the body’s circulation. This is a common test used for anyone with lung issue. After a lung transplant, this test is done monthly to make sure there are not any complications such as infection or rejection.

There are three kinds of rejection after a transplant:

Hyperacute rejection happens within minutes after a transplant has been done. This is due to antibodies in the organ recipient’s bloodstream that reacts with the new organ and results in organ failure. During this rejection antibody molecules suddenly bind with multiple target cells leading to death.

Acute rejection this is the most common type of rejection for transplant patients, it develops generally within the first month after surgery but it may appear at any time, even years after. Lymphocytes from the thymus (t-cells) are blamed for causing acute rejection. For most organs, the only way to show unequivocally that rejection is occurring is by biopsy of that organ.

Chronic rejection is also so known as either hyperacute or acute rejection. It is caused by multiple factors: antibodies as well as lymphocytes. Transplanted lungs with chronic rejection are said to have “bronchiolitis obilterans“, a scarring problem in the substance of the lung.

 Due to the consistent drop in my PFT’s the transplant team decided to have me in for a bronchoscopy. Bronchoscopy is a test to view the airways and diagnose lung disease. It may also be used during the treatment of some lung conditions.

 The following day after my bronchoscopy I received a call from my transplant coordinator letting me know that they had found the Influenza virus in one of the washing from the bronchoscopy. Influenza is a real bad flu for most people but for people with chronic illnesses/transplanties such as myself, can cause more issues as well damage my lungs. I was put on a 5-day treatment of Tamiflu; in addition, I took some time off work just to so I could recover a little faster. I returned to work at the end of the week feeling much better.

 A few days after my return to work, I received a call from my transplant coordinator telling me that they had the rest of my test results back from my bronchoscopy. I was kind of worried, as I always believed that no new is good news. The test results came back showing I had developed antibodies and some rejection. As I listened I could feel my heart racing, I felt light headed and a knot of dread formed in my stomach. I was told to come in the following week to have my blood and PFT’s done.

 Antibodies and rejection are two things someone after a transplant do not want! Antibodies are large Y-shaped proteins in the plasma of the blood cell. They are recruited by the immune system to identify and neutralize foreign objects like bacteria and viruses. Antibodies can also develop through exposure to foreign tissue, through pregnancy, blood transfusion or a previous transplant. The main concern of finding antibodies or proteins in the blood are that they will identify and attack foreign tissue, such as transplanted donor organs.

 It should be clear why rejection is not good for any transplant recipient. Almost every person who has had a transplant encounters acute rejection. When a person receives an organ from someone else during transplant surgery, that person’s immune system may recognize that an organ is a foreign object. This is because the person’s immune system detects that the antigens on the cells of the organ are different or not “matched.” Mismatched organs, or organs that are not matched closely enough, can trigger a blood transfusion reaction or transplant rejection.

 This is why the transplant teams react quickly when both of these issues come up in a patient’s results. When I head down to Toronto General Hospital to do my monthly blood and PFT’s, there are few things the team would look for in the blood during these tests.

WBC tells us if your white blood cells have increased (usually a sign of infection) or decreased (indicating a lower defense against infection).

HCT measures your hematocrit, the percentage of red blood cells present in your blood. Red blood cells carry oxygen to all parts of the body. When your HCT is low, you may feel tired or have little energy.

PLTS measures the level of platelets in your blood. Platelet cells form blood clots when your body is injured. Low platelet levels may cause you to bruise easily and to bleed for a longer time period.

 There are also a couple of drug levels to be drawn as some of the medication taken can be too high causing toxic levels in the patient. Drug toxicity is also a major concern as the body has basically been poisoned leading to severe central nervous system (CNS) toxicity, manifested by confusion, cortical blindness, quadriplegia, seizures, and coma.

 Blood work is also a monthly requirement after transplant as it helps to monitor all drug levels. It also helps to monitor kidney and liver levels as the medication taken after a transplant is very hard to these two organs.

Heading for my PFT’s, I still felt a little short of breath. I had been experiencing shortness since my run in with the Influenza virus. Once my PFT’s were completed I was asked if I was feeling short of breath as I had dropped another 30% in my lung function.

 Thirty percent is a large amount to lose in just over a month. These results set off alarms for the doctors to step in and figure out what is going on and what is the best form of treatment.

 The next day I received a call from my transplant coordinator telling me I needed to come into the clinic on Monday. There was no need to redo my PFT’s but the team wanted me to repeat my blood work as well have an antibodies count drawn and a Xray to see if there was any infection in my lungs.

 My mom (Tanis) decided to tag along to my clinic appointment as she had some questions of for her own and wanted to see what the doctors had to say. We met with the doctor and she told us that she was worried about the antibodies they found. The transplant team speculated that I had developed some kind of virus in November or just before as that is when I started to show signs of my PFT’s dropping. This would also explain why I could have developed these antibodies all of a sudden.

 Now you probably have come to the conclusion that antibodies are good, for most people as it helps them fight off virus and illness. But for someone who has had a transplant or needs to be imunnesupressed they are not. Imunnesupressed involves an act that reduces the activation or efficiency of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reaction to the treatment of other conditions.

 The formation of these antibodies and the protein they make are not a good thing for me. My immune system goes into attack mode making proteins to fight off what they think is an alien organism which in this case is my lungs.

 What are the treatments to remove this protein and antibodies; this was the first question that was asked. Plasmapheresis is the treatment that they use to help stop rejection. In addition to Plasmapheresis, they also recommend a large pulse therapy of the drug prednisone which is a treatment used in chronic rejection. Pulse therapy is a discontinuous I.V. infusion of a high dose of prednisone (more than 250 mg) given for one or more days.

 Plasmapheresis is a little more complicated, a few more steps are involved in this procedure. Simplified I think of a large trash can or an oil change for the body – the blood being the oil.

Dan receiving Plasmapheresis

Dan receiving Plasmapheresis

 It is a procedure similar to dialysis where my blood is spun in a centrifuge, filtered through a machine that separates it into plasma and blood cells. The plasma is removed and replaced with another solution, such as saline solution, albumin, anticoagulant and the reconstituted solution is then returned back to my body. The anticoagulant is added so the chances of blood clots are less likely to happen, as a blood clot could cause a stroke.

 Removing the plasma also removes the antibodies that have been sent out to attack my lungs. Plasmapheresis is used as a treatment for many conditions mostly including autoimmune disorders. While there are risks, the procedure is pretty safe.

 The reason why the albumin is added back to the blood is because it makes up 60% of the total protein in our blood and plays many roles. It keeps fluid from leaking out of blood vessels, nourishes tissues, and transports hormones, vitaminsdrugs and ions like calcium throughout the body.

 For the Plasmapheresis treatment to go smoothly I needed a central line put in. My veins have been well used in the past with all of my hospital admissions. This procedure is usually done in a room like an OR Room as there is some special equipment required (CT scan and an Ultrasound Machine). I did not get my central line input in till a few days after my admission, as there had been a long waiting list. This will push back my hospital stay a little longer than planned.

 My central line was inserted on the right side of my neck, not a pleasant location, however, the job was done. I could officially start making maple syrup, I mean to start my Plasmapheresis treatment. It felt as if I was tapped like a maple tree, to collect sap. Unbeknownst to me, I developed “taco neck” this would be my own medical term. Let me explain what “taco neck” is; it when you tilt your head left or right on a 20-25 degree angle as if you were eating tacos so all that delicious filling slides right into your mouth. However I was not eating tacos, it was just that my neck was stiff and sore. It may be convenient while eating a taco, however, having your neck in that position for two day’s will cause some discomfort not to mention look a little funny. This un-medical term “taco neck” was a result of the catheter being too tight resulting in my neck muscles tensing up. This only lasted for two day’s so I was quite relieved once it went away, allowing myself to sleep a little better at night.

 A central venous catheter, also called a central line, is a long, thin, flexible tube used to give medicines, fluids, nutrients, or blood products over a long period of time, usually several weeks or more. A catheter is often inserted in the arm or chest through the skin into a large vein. The catheter is threaded through this vein until it reaches a large vein near the heart. A catheter may be inserted into the neck if it will be used only during a hospital stay.

 I had a reaction to the Plasmapheresis during my first treatment. My blood pressure dropped considerably causing dizziness, light-headedness, some loss of concentration, a little blurred vision in my right eye, nausea, my feet/hands and head felt cold clammy and my skin was a little pale. My breathing was shallow; I was really tired as well I developed a migraine which lasted over 8 hours. They attributed this to the fact that I had been given my blood pressure medication before the procedure. In addition, I reacted to the blood thinners that were given during the Plasmapheresis.

 My second treatment went a lot better, I was not given my blood pressure medication before going down and the blood thinner was decreased. My blood pressure did drop however not as low and I did not have any of the other symptoms.

 The Plasmapheresis treatment is a minimum 5-day course; I asked the nurse what the cost of this procedure would be if I did not live in Ontario. She asked me to guess, I came up with $1,000 each session. She told me I was very close and was impressed, then informed me that each session can cost up to $1,300-$2,000 per procedures. Thus a five-session course of Plasmapheresis can cost anywhere up to $5,000-$10,000, (not including hospital stay) now that is one expensive oil change! Another reason to be thankful for the health care provided in our country.

Plasmapheresis Machine

Plasmapheresis Machine

 Once I have finished the five-day treatment of the plasmapheresis the transplant team will decide if they want to go ahead with the pulse treatment of prednisone. They would like to wait until I am finished the plasmapheresis as they want to deal with each issue at a time or wait till I have had my 3rd treatment of plasmapheresis. The reason for this is so the pulse drug given through the IV will not be cleaned out of my blood during the next plasmapheresis.

 Once again my support team (friends, family, individuals I have never met over social media and my transplant team) have been by my side the entire time. Words can not describe how much this means to me. It helps to build my spirits when they are low. It helps to give me back my sanity when I am on the verge of losing it after endless hours of sitting around my hospital prison.

 Your continued support has helped me to be the fighter. Your encouragement lifts my spirits. Once again I find myself standing in that dark, chilly, damp, lonely lion’s den. As I stand I feel the cold loose sand in-between my toes, my feet lightly sink with each step. However, I will not fall. Your support allows me to stare deep into the powerful green-yellow glow of the lion’s eyes. I will not let fear overcome me. Your energy fills me with the strength and power necessary to put the lions to sleep, allowing me the chance to climb out of the lion’s pit once again, scarred but even stronger!

My greatest thank you is given from myself and my family!

National Organ and Tissue Donation Awareness Week