I’m a 35-year-old runner, gardener, yogi, teacher, wife, and mom. I love reading young adult novels, exploring farmer’s markets, adventuring with my family and experimenting with new recipes. I don’t have cystic fibrosis. In fact, for more than half my life, I didn’t even know CF existed. I had no idea the impact it would have on my life. But then again, for more than half my life, I didn’t know Chad.
Chad, my hubby, my best friend, my person. Chad is the reason I learned about CF. Chad and I met in college. He was the manager at the best college bar in the world and I happened to be working there. Chad was the guy who put flowers in the cooler at work for me, the guy who always stuck around to talk and the guy who walked me home after a late shift. Fast forward and Chad’s the guy who I fell in love with, the guy who proposed and the man I vowed to partner with for the rest of my life.
Like I mentioned, when we met in college, I didn’t know what CF was. I now understand it, as best as a person who doesn’t live it, can understand it. I have seen Chad in awesome health – able to hike in Montana and have the courage to whitewater raft.
I have also seen him in a really scary state – unable to walk across our kitchen without stopping and unsure if he could be the person he wanted to be because of anxiety and depression. Together, we’ve navigated doctor’s appointments, clinic visits, transplant discussions, and IVF. We’ve explained and tried to normalize aging with CF to friends, our parents, and most recently, to our boys.
Normalizing CF….that can be tricky. It’s hard for others to understand that our day can’t work the way most families days work. Chad needs time, both in the morning and night, for treatment. And, as most in the CF community knows, I’m not talking a few extra minutes. I’m talking 2-3 hours a day for treatment, plus time for meditation, exercise, extra rest, medication ordering, nose rinsing, nebulizer cleaning and more. CF also means that Chad sometimes can’t keep pace with others or do all the activities the rest of the group does. As the wife, I try to balance the pace of our kids or friends with Chad’s ability so that all feel like they are doing the right thing. And, more recently as Chad becomes older and his health is harder to rebound, it’s hard to normalize the depression and anxiety that such a demanding disease brings. But we do.
Somehow, we’ve made it so CF is at least for us, normal. The boys help clean nebulizer cups, plug in Chad’s vest, and race down to the basement in the morning to be with him while he does treatment. They know dad has CF, but also know that he’s ok. In their eyes, and in mine, he’s a superhero just the way he is (maybe, he’s a superhero because of who he is). They know Chad not as a CF patient but as a dad. The dad who walks them into school every morning, who reads extra books at night, the dad who loves DC sports and the dad who builds the most incredible Lego creations they can imagine.
There are moments when being the support person and normalizing CF can be challenging. When our older son asked Chad, out of the blue, why Chad has CF and he doesn’t, alerts sounded in my head. I wanted our son to know that questions are good and that it was great to learn and try to understand cystic fibrosis, as best a 6-year-old can. But I also didn’t want my answer about recessive and dominant genes to make Chad feel inferior. This time, I didn’t hit a grand slam. I used “weaker” as another word for recessive to try to make things easier for our son to understand. While supporting one, I hurt another. The balance of feelings, learning, and doing can sometimes tip. I apologized privately to Chad later, when our son could not hear. I have learned it is to be important to keep trying and admit when I fall short.
The other night, our three-year-old was talking with Chad after they had finished reading books before bed. Chad didn’t know I could hear, but our son was asking why Chad “plugs in and shakes.” He was asking why Chad does his vest treatment. Chad, without a beat, explained that he needs to shake up the mucus in his lungs so he can play better. Chad explained that he has CF, but our son doesn’t, so our son doesn’t do the vest. There were giggles next because our son said he likes that vest and likes to be with dad while he shakes.
There are times when being a CF wife and full-time working mom is really, really hard. There are days when the list feels endless and I become frustrated that Chad can’t help or join us playing because he’s doing treatment. Most days I don’t feel like that though. Most days, I am grateful that I get to partner and parent with a man who fights harder than any other person I know. He fights to get to be with me and our children. His devotion to us is shown with every treatment.
The challenges CF brings have brought patience, empathy, and compassion to our family. We purposefully and mindfully look with gratitude at the bright parts of life – riding bikes, backyard grilling, reading in bed and laughing. We can’t change that Chad has CF. We can, though, embrace each other, every day, and do our best to spread more love.
“I am grateful that I get to partner and parent with a man who fights harder than any other person I know. He fights to get to be with me and our children. His devotion to us is shown with every treatment. ” -Julie
CFers are an incredible bunch, and Eric is no exception. Having a close view of his courage, strength, and determination has changed me for the better…and the not-so-easy times we’ve encountered have helped me grow as a person. I’ve become more thankful for the many positive things in my life, more content to enjoy simple pleasures like a night at home with my favorite person in the world. I’ve evolved into a creative problem-solver, having become a master organizer of medical supplies and sneaked cream cheese into Kraft Easy Mac to add much-needed calories. I stay cool in a crisis and am always prepared (just ask the EMTs and ER staff), thanks to some epic hemorrhages, a cancer diagnosis, and other emergencies.
Mostly, I’m stronger. As a spoonie myself, I never would have believed I could make through what we dealt with this past fall. I was getting 2-3 hours of sleep a night as I held on to my full-time job and made it to the hospital 5+ days a week to talk to the doctors and attend transplant caregiver classes. On top of that, I was fundraising for transplant expenses and doing my own medical research in my “free” time, all while the love of my life, my whole world, fought for his life and we anxiously waited to hear if he would be accepted as a transplant candidate.
And most importantly, I’ve become a believer. A believer in miracles, God, hope, love, and a small, small world. My new take on belief started the day after Eric was finally listed for a lung transplant after an emergency inpatient evaluation. It followed an episode of massive hemoptysis, two hospital transfers, a BAE, many days in the ICU, and a call from an ICU doctor saying that they would be forced to intubate Eric due to the massive strain his heart was under trying to compensate for his broken lungs—the call that left me sobbing hysterically at my desk before racing to the hospital while making impossibly difficult calls to our families.
I headed to the hospital that morning feeling relieved that Eric had finally been placed on the transplant waiting list. The relief didn’t last long: the hemoptysis, which had been held at bay for over a week, returned and grew more severe throughout the day. Not long after I arrived, Eric got a call from an out-of-state number on his cell phone, which he ignored. A few seconds later, I got a call from a number I didn’t recognize. It was a transplant coordinator, calling to inform us that a pair of donor lungs had been located and were a match for Eric, he was at the top of the list. We were in complete disbelief. Getting “THE CALL” less than 24 hours after joining the wait list? Certain this would turn out to be a “dry run”, we kept the news to ourselves and tried to enjoy the day together, keeping the news to ourselves but growing more and more worried as the hemoptysis got worse and the doctors told us nothing more could be done to stop the bleeding. I helped Eric wash his hair and we talked about the transplant education classes he’d missed as an inpatient.
As the day progressed, we continued to wait for updates on the status, expecting each call to end in disappointment. Eric, exhausted from coughing, fighting for oxygen, and the flurry of surgical preparations, thankfully fell asleep. That left me alone in the dark hospital room, worrying about the rising level of blood in his culture cup and awaiting more news from the transplant coordinator. We finally got the call around midnight—the lungs were a go! Less than 36 hours after being listed, Eric was in surgery, and not a moment too soon. When I spoke with his surgeon the next day (after 12 hours in surgery), he told me that Eric’s lungs were in horrible shape, with a number of blood vessels ready to burst.
Add to that all of the amazing things that I witnessed as Eric recovered post-transplant—breathing room air and walking as soon as he was weaned off sedation and walking nearly a mile just a couple of days after surgery. Then, a few weeks later, we were contacted by the donor’s family following a news story…and now the donor’s mother calls Eric her “bonus son.”
More and more, I think CFers get a few more miracles than the average person; maybe it’s so they can share their unique stories, inspire the people who know and love them and leave their incredible marks on the world.
“More and more, I think CFers get a few more miracles than the average person; maybe it’s so they can share their unique stories, inspire the people who know and love them and leave their incredible marks on the world.” – Marissa
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.
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.
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?”
“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
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.
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.
“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
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.
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 lives. 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.
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.
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.
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”.
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.
“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.
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.
We may not look sick, but turn our bodies inside out and they would tell different stories.
INHALE, EXHALE, now again INHALE, EXHALE, 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 breath, constant 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!
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.
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.
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.
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 depression, anxiety, scene of loneliness, stress, pain, bad 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.
Have you ever wanted to be a Hero, what is a Hero these days anyways?
I am sure if you ask around you’ll get some interesting answers. As the world has evolved the definition of a hero has changed. The dictionary defines a hero as “a person who is admired for great or brave acts or fine qualities”. Examples of heroes range from mythological figures, such as Gilgamesh, Achilles, and Iphigenia, to historical figures, such as Joan of Arc, modern heroes like Florence Nightingale, Gloria Steinem, Martin Luther King Jr., Mahatma Gandhi to fantasy fictional heroes including Superman and Batman.
To a transplantee, a Hero is their organ donor. I know in my eyes this is true, two separate individuals saved my life allowing me to continue my place on earth with borrowed time. I admire them as does my family and friends for the brave and unselfish gift they have given me. My organ donors who had no clue who I was, gave me life! Wouldn’t you be proud to know when your life has ended you’ve given the chance for life to someone to begin theirs? Passing the light of life on!
I raise the question as April is National Organ and Tissue Donation Awareness Month and it also coincides with spring. To me, spring means life, new growth, and new beginnings. Speaking from a transplantees point of view, that’s exactly what my transplant has given me. A LIFE! A life that has allowed me to grow, experience new adventures and beginnings that I never even thought would have been possible.
You used to be able to register as an organ donor on your driver’s license however this is no longer the case. The best way to check if you’re a donor is to look on the back of your new Ontario health card (if you still have your “red and white” health card maybe it’s time to update just for this reason), on the right side in small print, it will read “donor” or “donneur” in French. If it doesn’t say that, you’re not registered.
” Yes I am an organ donor, A guy I went to high school with got a liver transplant and it changed his life 100% and as I saw a Facebook post of his which explained why it’s so important”.
You may ask yourself why Ontario switched from using a driver’s license to our health card. Not everyone has a driver’s license, whereas a health card is more practical. Paper donor cards are no longer valid you now need to register with a provincial agency. You should also tell your family and friends of your wishes. By telling your friends, you are allowing them to support your family when the time comes and the support is most important. A lot of organ donors are lost due to lack of communication about your wishes.
My personal experience with organ donation makes relating a lot easier for me. After undergoing two double lung transplants, Organ Awareness is an incremental part of our family. This very amazing selfless gift has changed our family dynamics. Moments that never would have been possible are now memories.
It’s allowed me see my older brother become a father to two beautiful girls. My middle brother and I have become closer, making him stronger. We share our thoughts and know that we always have one another to talk and listen to. My twin sister has overcome her own challenges and is now a teacher. Most important our twin connection is very much alive.
My mom and dad have been able to see me surpass my life expectancy with Cystic Fibrosis. They have been able to see their youngest son live his dreams, become stronger but most important it has allowed them to see me transform from a boy into a man. Something that is very special for a parent. This is especially important knowing that when I was born the life expectancy for CF was 16 years old. It has given me the chance of making many special and important connections with so many people thus enriching our lives.
“An organ donor? Of course, I am! Our family has experienced and had been inspired from transplantation. We were ALL given extra time on both occasions. Without living such experiences, I might not have been able to see the beauty, love, life, and happiness that one act creates. I would be happy to do as such for another family”.
By registering your consent your donation decision is recorded and is made available to the right people at the right time, and is shared with your loved ones. When you register your consent to donate organs and tissue, the information is recorded by the Ministry of Health and Long-Term Care. When needed, The Trillium Gift of Life Network will request confirmation of your consent to donate organs and tissue through the ministry. It is the Trillium Gift of Life Network’s practice to reaffirm consent to donate with the family. In almost all cases, families honor and respect their loved one’s donation decision if they have evidence that it’s what they wanted. So, it’s important to register your consent to donate so that your family can be advised of your decision.
Throughout my life, I have been able to see both sides of the organ donation journey from the giving to receiving. To personally know what it’s like to be on the other side of the fence is just as remarkable. Any loss of life is a dark moment, but to know you have helped bring light back into another person/family’s life, can be a gift itself. It can help bring light back into the very dark grieving moment you’re in.
To think your heart may have saved a father with congestive heart failure allowing him a chance to see his daughter get married, enjoying their special dance before she begins a new chapter of her life. Or the freedom you could give a young boy that needs a new kidney, dramatically transforming his life from being a prisoner to his dialysis machine to living a normal life. You would allow him to grow up as a regular boy no longer missing moments with his friends and family because of his illness.
Imagine someone who is blind, suddenly being able to see for the very first time of their life. Visually explosive their first sunrise will be forever engrained in memory. Not only do they feel the soft gradual warmth of heat dancing across their face, but now engage in the full experience of a sunrise. As the darkness surrenders to light transforming shadows into actual objects. Glowing amber hues of gold and orange playfully mix into brilliant pinks and purples slowly folding away the blues in the sky, as if the sky is a sea of cotton candy. As the sun begins to rise kissing the horizon slowly away till they meet again for next day. The fresh smell of wet dew as it slowly evaporates into the air. They are in awe of the gift that has allowed them to see the world, to experience colours and light. No longer living their life as if their eyes are closed but now opened. Finally, they can cross off watching a sunrise from their bucket list.
“Yes I am a donor, I would be honored if I could die in a way that allows others to live, to have more time. I feel that it is owed for a variety of positive and wonderful reasons”.
With the increase of organ awareness and education more of these stories can be written and shared. By having more people become organ donors the gifts of life increases. You may now understand why we as transplant recipients call our donors Hero’s. They not only save our lives but grant us new chances that may have never been possible.
Today, in Ontario, there are over 1,528, people waiting for a lifesaving organ transplant (As of December 31, 2016). This is their only treatment option, every 3 days someone will die because they did not get their lifesaving transplant in time. I myself was almost part of that statistics of being one of the people that didn’t get to see the next day of their life.
The year 2002, I was in end stage Cystic Fibrosis at the age of 16 years young, having signed a DNR (Do Not Resuscitate) as I was told: “Living on a Life support isn’t living at all”. As much as I didn’t want to face the facts that my life was hanging on a thread it was right in front of me. My lungs could no longer keep up, they were failing from the inside out. I would cough and vomit blood daily from my lungs. I was drowning from my own body fluids. As hard as my body was fighting it was rapidly becoming a downhill battle! As I clung to life, I slept – my energy totally depleted. I barely had the strength to put on a smile when visitors came to see me. My only hope of survival was a lung transplant. I needed a hero to step forward into my life.
Let’s talk about some facts here in Canada regarding organ and tissue awareness, 31% of Ontarians are registered donors. That’s 3.7 million out of an eligible 12.1 million. 90% majority of Canadians support organ and tissue donation but less than 20% have made plans to donate.
Where is the missing connection, is it education on the awareness of organ donation or is it the confusion of becoming a donor? I recently asked my family and friends and the circle around them a few questions.
The questions were:
Are you an organ donor?
If yes why so, more importantly, if no why not?
Are you in a room with other people? Can you ask if they are an organ-and-tissue-donor?
And lastly, do you feel there is an easier way to have people register?
My inner circle all answered “Yes” they are registered organ or tissue donors. As the question was spread out through the middle circumference of the circle there were more mixed answers. Some answered with, ” I am not sure how do I find out?“ or “No, I’ve never thought about it“.
It was clear to me that my personal experience influenced the decision of my inner circle.
Throughout the post, I’ve shared with you some of the people’s thoughts on becoming organ donors!
” Dam straight I am an organ donor! I’d rather know that other people live because of these useless pieces of flesh. I remember once hearing someone talk about how precious our bodies are, but we are like cars. You taught me that. If I can fix another person by something I don’t need, super!”
Out of the individuals, I had asked organ donation affected them in different ways.
It never ceases to amaze me that my personal journey has encouraged others to become an organ donor. It’s a network of strangers trying to help a community of people they do not know and will probably never meet, so others can live a better life.
Fun Fact, did you know ONE donor can save up to 8 lives through organ donation and enhance the lives of up to 75 more through the gift of tissue! One more fact, AGE alone does not disqualify someone from becoming a donor. The oldest organ donor was over 90 and the oldest tissue donor was over 100. There’s always potential to be a donor; it shouldn’t stop you from registering.
The next item on my list was is there an easier way to have people register as an organ-and-tissue-donor?
The two main answers were it should be mandatory, or at least have an opt-out a system where everyone starts as an organ-and-tissue-donor and if they chose not to be they may opt out of doing so. The other suggested method is education. There are a lot of people that do not know how the program works, not due to ignorance just due to confusion and miscommunication. Quite a few people also stated that if the people, who do not want to be part of the program, should not be given the chance of receiving an organ if ever the need arose. I didn’t want to go into details regarding that topic at this time as I feel it would pull away from the main point here. Many religions support organ and tissue donation or respect an individual’s choice.
“My mom convinced me to become an organ donor, actually I didn’t notice it was an option when I was getting my health card, my mom pointed out the section on the form and I checked it. She said they are no use to me when I die. Also, my cousin had died young and from his death, 4 people got to live”.
There are many countries that have implemented the opt-out system already including Argentina, Australia, Chile, Colombia, Europe, and Singapore. The numbers of transplant patients waiting to receive a transplant are low. If I lived in Australia or any of these countries that have the opt-out system, the time spent on the waiting list could have been a lot shorter. It might have changed the future for many people that didn’t make it to the point of receiving their phone call that an organ was available.
Another interesting method being used for blood donation is in Sweden. When a donor’s blood has been used to save someone’s life, the donor will receive a text message telling them their blood has made the difference in saving a life! I am sure that’s a rewarding feeling knowing that you have been incremental is saving someone – a selfless heroic act.
“Yes, because I think it’s the most incredible final gift that I can give, the chance of providing a new beginning to another human being”.
I think Sweden’s approach with their blood bank is a great idea. It allows the donor the chance to feel that their donation has made an impact on someone’s life. It’s a great way of saying thank you! I have received blood transfusions many times throughout my life. I wouldn’t be here if there weren’t blood donors, they are just as important as organ donors. So remember there are other ways you can part take with donations. Consider donating blood when and if possible.
Now for a little education, this year in Ontario there has been 96 donors that have been able to donate their organs or tissue. Out of the 96 donors, 61 have been deceased, 35 are living donors. An extra 14 deceased donors from outside Ontario are not included in that statistics. Donor– definition: At least one organ has been retrieved and transplanted. To date, 13,300 Ontarians have received a lifesaving organ transplant since 2003.
Ready for another Fun Fact? You are five times more likely to need an organ transplant during your lifetime than to have the opportunity to donate one. On average, only three percent of hospital deaths occur in circumstances that may lead to organ donation. This is not the case for tissue donation, which can take place in most cases when someone has died, as long as the tissue is suitable for transplantation! If you were wondering organ and tissue donation does not impact funeral plans. An open casket funeral is possible if that’s the fear of some individuals.
By registering, you are essentially making a decision to help save lives after death through organ and tissue donation. By making this decision, you give hope to patients like me for a life-saving or life-enhancing transplant and their families.
Even though you have registered as a donor the final decision is up to your family so make sure you have discussed your wishes and how important it is to you of being an organ-and-tissue-donor.
“I am an organ donor firstly because of your influence on my life but secondly I would be regardless because I think I won’t need them. I don’t agree with waste and I think everything should be used to its fullest potential. Maybe that’s weird, but why just throw me away?”
Once a family has consented to donation, testing is done to confirm the medical suitability of the organs and tissue, and to determine which patients waiting for organ transplantation are the best match for the donated organs. This includes a medical and social history of the donor, similar to the questionnaire completed when donating blood. Trillium Gift of Life Network works closely with a team of healthcare professionals to support grieving families through this process, and to ensure that as many people as possible benefit from the generous gift of organs and/or tissue.
In desperate times when an organ is critically needed, the transplant team can manipulate the size of the organ. Patients needing a liver or lung can have partial portions of the organ transplanted. In dealing with lungs the team can cut the lungs down and transplant just the lobes. This was the case for my first transplant, due to the fact of my small stature finding lungs small enough for me was challenging. The team and I could no longer waste any more time, they knew I couldn’t fight much longer. It’s quite amazing that this is possible.
My first donor was able to save four lives, their heart, kidney, liver and lungs were all used to bring new hope into other lives. As well many victims with extensive wounding or trauma, people suffering from necrotizing fasciitis or purpura fulminans, burn victims, even skin cancer victims received skin grafts enhancing their lives. It goes to show the team will use everything possible as long as the organs and skin are healthy, clear from any infection or disease.
What are the most common organ transplants performed?
Lung transplant surgery can replace one or both diseased lungs. Lungs can come from either a deceased or living donor. A transplant may be recommended if you have a chronic obstructive pulmonary disease, cystic fibrosis, idiopathic pulmonary disease.
Heart transplant replaces a damaged/diseased heart with a healthy one from a donor who has died. This is the final treatment option for people with heart failure where all other options have failed. Heart failure may occur due to congenital heart disease, coronary heart disease, damaged heart valves, heart muscles, or viral infections. As with other transplants, you must take medication to prevent your body from rejecting the new heart.
Liver Transplant Your liver helps fight infection and cleans your blood. It helps digest food and stores energy for when you need it. Transplanted livers can come from a deceased or living donor.
Small bowel transplant replaces a diseased or shortened small bowel with a healthy bowel from a donor. A small bowel transplant is considered when complications develop from total parenteral nutrition (TPN) or when a person is unable to tolerate this form of feeding. TPN nutrition is where liquid nutrition is given through a drip.
Islet cell transplantation replaces only the part of the pancreas that makes insulin. It is a treatment for people with type 1 diabetes who have trouble controlling their blood sugar level. Islet transplants are done alone or together with a kidney transplant. Transplanted islet cells come from deceased donors.
Kidney transplant operation places a healthy kidney into your body. This kidney takes over the work of the two kidneys that failed, so you no longer need dialysis. Transplanted kidneys come from a deceased or living donor.
Cornea transplant also called keratoplasty, is a surgical procedure to replace part of your cornea with corneal tissue from a donor. Your cornea is the transparent, dome-shaped surface of your eye that accounts for a large part of your eye’s focusing power.
Bone transplant is a surgical procedure used to fix problems with bones or joints. Bone grafting, or transplanting of bone tissue, is beneficial in fixing bones that are damaged from trauma, or problem joints. It’s also useful for growing bone around an implanted device, such as a total knee replacement.
Skin transplant is a type of graft surgery involving the transplantation of skin. The transplanted tissue is called a skin graft. Skin grafting is often used to treat: Extensive wounding or trauma. Burns.
A transplant is a type of surgery where an organ or tissue is removed from one person (the donor) and given to another (the recipient) to replace the recipient’s damaged organ or tissue.
An organ is a part of the body made up of various types of tissues that perform a particular function. A tissue is a collection of similar cells that perform a special function. There are hundreds of diseases that affect organs and tissues to the point that a transplant is required, but not everybody that suffers from disease requires one.
“Yes, I am on Organ donor. I made the decision to become one after my cousin’s life was saved not once but twice, thanks to organ donation. I would like to give a chance for someone else’s life to be saved like my cousin”
Just like in cartoons, myths and legends the Hero comes in all different shapes and sizes. This is true in reality as well!
Eight years after my first lung transplant I required another transplant as the lungs I had received were being rejected from my body. I joined the transplant list in August 2009, I was sick, on oxygen in need of a transplant; however, I wasn’t critical at this point.
My second lung transplant, the donor’s lungs didn’t require reshaping or cutting. This could have meant two things, one my lungs came from a small adult or two a child. This brings me to a post I added in April 2013 called Ryan’s Story of Hope. The story is from a donor’s family that I know, their son Ryan was only 10 years old when he passed. Ryan died in 1997 from a cerebral brain hemorrhage. At the age of ten Ryan already knew how important organ donation was. In actual fact, he would go around and ask people if they had signed their donor cards, and tell them the importance of becoming a donor. Ryan was able to give life back. He donated his corneas, lungs, liver, kidneys, pancreas and heart valves With Ryan’s gift he was able to change the status of these recipients’ lives from existing to living. Ryan’s gift enabled a university student the ability to see. A woman no longer needed insulin and dialysis; she was able to work again, returning back to a normal life.
The next recipient of Ryan’s gift hits close to home for me as we share a lot of similarities. A boy of 16, with Cystic Fibrosis, had received his lungs. Without the double lung transplant, he would have died. Not only was he able to return to school, he could do things that previously were impossible. Running was no longer just a dream. He could roller blade with his friends, what once were dreams for this boy became reality.
An important question most may still ask and to clear the confusion in the air I have posted links of where you can check as well register as a donor.
How can you tell if you’re a registered organ donor?
Check online whether it be your computer or smartphone click from your province it will take two minutes to find out and if you’re not you’re at the right site to become one.
One stop shop link for all provinces is http://www.cantransplant.ca/home/organ-and-tissue-donation-consent/ select what province you’re from and the site will direct you to the correct place to register as an organ donor. I have also listed the links for each province.
YT, NT, and NU – http://www.hss.gov.yk.ca/organdonation.php
If you live in the USA try this site https://www.dmv.org/organ-donor.php
Each province, state, and country are different but you can easily type into Google where you live and how to find out. When you register your consent for organ and tissue donation, you are letting those waiting know that you would help them if you could.
You don’t just have to be the hero of your own story, become someone else’s hero. Ryan Doige wanted to be a fireman when he grew up, a hero, his selfless gift of giving back made him a hero – he saved many lives.
During the month of April when spending time with friends and family bring up the topic of organ donation. Discuss the idea of becoming an organ donor. See if you can help start a gift of life for someone that you may not even know. We are all Hero’s in training.
I leave you with these questions:
Are you an organ donor?
If you answered “YES” what made you decide to become one, is the reason of you becoming an organ donor strong enough for you to encourage others to do so?
If you answered “NO” is there a reason that is holding you back?
There is no right or wrong answer here; we all have our own opinion and rights.
If you feel comfortable please share what helped you make your decision in the comment section.
My life has been based on numbers, predictions, percentages, baselines, decisions made by a team of doctors. I am not saying that I have not been a part of any of these decisions, in fact, I am the final call on all the end decisions made. It’s my story, right? Whether I chose to let this story end or add a new chapter into my life is all up to me.
I would say over the last 6-7 months I have been trying to change myself for the better, to believe the unknown chapters of my life will continue. Guess you could say I have been trying to rebuild myself for the better, make myself stronger! What’s the saying “Better, Faster, Stronger” right? Sometimes it’s not always faster, but you get the point. We all have flaws that we aren’t proud of in our lives. Some help us stay motivated when we fall off track and others lead us in directions that may not give us a chance to recover from. But in the end, it’s all a life lesson and as long as we gain from it, we can recover for the better, but you have to choose to want to be better.
Growing up with Cystic Fibrosis (CF) I never let people really know about my disease. I didn’t want to be judged or looked at me differently. After all, I tried my hardest to blend in or act as normal as any another kid. I might have had the odd coughing fit but besides my short stature and my cough, I acted as normal as I could. I was feistier than most kids my age but that came with the territory. Dealing with the many different daily obstacles that living with CF, presented my character grew. Hell, I even fooled myself at times that I was as normal as my friends until it came time to take my meds or do any of daily therapies.
I was diagnosed with CF at birth. At that time CF wasn’t well known. To this day when I meet someone I don’t announce that I have CF right away or that I have had two double lung transplants. “I never wanted CF to control my life, I wanted to have control over my CF life, or at least have as little footprint in my life as possible”. I don’t want people to judge me or treat me differently or base their first impression on or what I have gone through. There is a good saying I like to believe in “I am not what you think I am. You are what you think I am”.
March 22nd will be my 15th year lung anniversary (March 21st will be 7 years from my second lung transplant) what are the odds of my anniversary’s being a day apart? I have experienced an additional 15 amazing years of my life all because two heroes saved me. I owe everything to my two organ donors as well as their families. Their unselfish act has given me the ability to continue my story here on earth.
We’re all here for a reason whether it’s to recreate, help build this world for the better, save a life or even provide guidance for others, we are born with a destiny to be fulfilled. Some may not even know they have achieved the task and continue with their life, some may not even reach the goal life has set for them. But one thing we all can do is become an organ donor. You never know who you’re going to save it could even be your own family or friend.
Fifteen years ago I made a decision, not only would it change my life but also the lives around me that followed my life’s journey. I would like to think of myself as an individual who leaves a self-impression on others and to let them see another side to life. We take life too seriously most of the time and need to let loose more often than we allow.
I hope to leave to all those that have touched my life an important message. There will always be time to be serious. Life can pull us in many directions causing stress and anxiety. Balance is critical, make sure to make time to have fun, to try new things. Life is meant to be experienced for the moment. Don’t live a little, live a lot! For this reason, I tend to say “yes” to most things that I should sometimes say “no” to. We never know what opportunities lay ahead. New stories are contrived by taking chances; new road opens up new opportunities.
Being faced with many negative predictions, I guess I have grown to like a good challenge in my life.
Growing up with CF I have been faced with numbers all of my life. These numbers include everything from statistics on mean average age, life expediency, odds, predictions, values, goals, medications, the list goes on.
Having CF has definitely challenged my life, I candy coat nothing. The dark and scary times this disease has brought rough sailing waters my way are many. I try to avoid sharing these episodes with people. Seeing smiles on their faces makes me happy. I prefer this to the sullen concerned look of my family and friends when they find out I have another storm to face.
Living with CF has definitely meant that I have had to navigate through turmoil. The side effects from the medication I have been on the majority of my life have been responsible for much of it. CF harbors fungal bacteria that can change a successful lung transplant into sinking ship. I personally have had to face two double Lung transplants, Lymphoma, MRSA (Methicillin-Resistant Staphylococcus Aureus), Squamouse Cell Carcinoma, Diabetes (Cystic Fibrosis Related Diabetes), 15 nasal polyps’ removals, Pleurisy, Bowel Obstructions, Rejection, Aspergillus, Pseudomonas, Scopulariopsis Brevicaulis, Osteoporosis. The countless small procedures include a G-tube, Ports, Pic lines, Chest Tubes. I am also written up in the medical textbooks as having the highest Blood Sugar levels the team has ever seen come in through Emergency. I am on deck for a third transplant. These challenges have molded me into who I am today. They build my morals; strengthen my psyche, expanding my character in this story called “Life”. It’s taught me that you can’t stop the waves but you can learn how to surf.
Strangely one of the many challenges CF has given me over my life is a problem most people would love; weight gain. For those that know me, I am a bottomless garbage can. People either find it disgusting how much food I can eat or wish they could eat as much as I do and not gain an ounce. In reality gaining weight has been impossible for me for the last few years. If I want to keep the options of a 3rd transplant open it is crucial that I maintain a healthy weight.
Having CFRD (Cystic Fibrosis Related Diabetes) doesn’t help the weight problem. CFRD is manageable however it needs to be followed closely. I am constantly calculating, adding and subtracting in my head how many carbohydrates and sugars I am about to consume. This is how I determine the amount of insulin I need. I have found that a 15 to 1 scale works well for me. That’s for every 15 grams of carbohydrates/sugars I’ll take 1 unit of insulin. Between counting carbohydrates, sugars, and calories to maintain weight my days are continually filled with math.
Once a year I travel to St. Michael’s hospital to the CF clinic for my annual assessment. St Michael’s Hospital is home to the largest adult CF program in North America. In addition to providing multidisciplinary care to 400 adults with CF, they are an active site for clinical research and training.
Cystic fibrosis is caused by a genetic mutation that breaks an ion channel in tissue lining cells. The channel is necessary to properly make sweat, mucus, and digestive juices. Results of the mutation include thick mucus in the respiratory tract that can create a haven for deadly pathogens, as well as gastrointestinal issues and pancreas damage. The type of mutation, type of pathogens that settle in the lungs, pancreatic status, quality of care, and diet can all affect health outcomes.
The majority of the tests performed in my CF clinic are the same as the transplant clinic at TGH such as PFT’s (pulmonary function test) and X-rays. Blood work is similar. Some of the different tests would be a Vitamin K level. They also follow my blood sugars more closely and have more experience with CFRD.
Vitamin K is a fat-soluble vitamin and its absorption from the gut is dependent on bile salt and pancreatic lipase secretion stimulated by dietary fat. People with CF are at risk of developing vitamin K deficiency due to fat malabsorption as a consequence of pancreatic insufficiency and bile salt deficiency. Other risk factors include CF-related liver disease, frequent antibiotic therapy, inadequate dietary intake and short gut syndrome resulting from bowel resection work. A sputum sample is usually required as patients with CF will harbor bad fungus in their lungs or sinuses as well.
I also see a dietitian as eating well and maintaining good nutritional status is very important in the treatment of CF. In Canada, the Cystic Fibrosis Evidence has shown that improving and maintaining good nourishment in CF results in better functioning lungs as well as overall outcome and well-being. This has benefited Canadian CF patients from the US with an estimated increase of 10-year survival bringing the current average age to 50.9 now. This is a huge achievement as the life expectancy when I was born was 15 years old. The aim of nutritional management of CF is to support normal growth and development throughout life.
People with CF have increased nutrition and energy requirements. Often in CF normal digestion of food is affected. This can be due to a reduction in the number of digestive enzymes released by the pancreas. These enzymes are vital for the digestion of normal food. If these enzymes are not present food cannot be broken down and absorbed by the body, therefore maintaining your weight and nutritional wellbeing could be a struggle. This condition is often referred to as ‘pancreatic insufficiency’.
During my last visit in December 2016, I had asked the Dietitian to help me with my weight gain. After looking at all my recent tests, height, current weight, blood sugars and daily activity I was given a goal of 3500-4000 calories a day. If I get an infection or become ill I would need to increase this goal to at least 4500 calories due to my body burning more energy.
Normal calorie intake for most people is anywhere from 2000-2500 a day, therefore I was quite surprised with my goal due to my size. For those who don’t know me, I am not very big. Soaking wet I would weigh 71 pounds and if I was stretched out about 140 cm.
I took this goal into consideration and started to do some research on high fat/calorie foods that were healthy as well as meal planning. I wanted to go into more detail hoping to discover any opportunity that could add extra weight. In addition, I wanted to improve my exercise program so I could strengthen not only my lungs but my entire body.
My plan was to double my meals every day as well try and eat snacks in between. Unfortunately, this first plan failed for two reasons. You see I was out most of my days so trying to eat high fat/calorie healthy foods was hard to find, not to mention the amount of money I was spending eating out. Secondly, by mid afternoon I was too full and felt so uncomfortable even breathing was more laboring at times. It almost felt like I committed my day to just eating, it became a full-time job.
I took a step back and considered what needed to be fixed. Before long I came up with a few ideas. Number one was I couldn’t start right away with a goal of 4000 calories. I needed to stretch out my stomach and get comfortable with consuming all of this food. So I aimed for a daily goal of 2800-3000 calorie intake and then increased by 500 calories every two weeks.
I also figured that starting my day with a large breakfast followed by a smoothie gave me 1/3rd of my calories. That way if I got caught up in my day, consuming the rest of my calorie goal wasn’t as hard. I also learned to snack more often throughout the day eating more mixed peanuts and cheeses and adding in the extra Ensure Plus. The challenge was to do all this while maintaining my blood sugar. After all, if I didn’t maintain my blood sugar there was no point in trying to put on any weight. So not only was I counting calories I was following my carbohydrates and sugars closely to stick with my sliding scale I had talked about earlier.
For those that are looking for a good tool for tracking your calories as well a fitness guide, download the app “My Fitness Pal” it’s supported by Under Armour. It’s a great app that will help you reach your goals as well give you fitness advice! It also will take your workout into consideration, as well how many steps you put into your day and readjust your calorie goal for the end of the day; a perfect way to stay on top of your goal.
The sole purpose of my weight gain and blood sugar management was due to my Transplant team. This was one of their recommendations last year that in order to be a candidate for a 3rd transplant I would need to gain weight and muscle and have a better control over my blood sugars. I’ll admit in the past I didn’t always pay full attention to my blood sugars.
I have started to see some small improvements over the past four months with my weight gain. I can thank some good friends and one of my brothers for encouraging me to stay on course. Included are some interesting late night meal outings, a few visits to Mc Donald’s by maxing out their custom burger menu by adding more than six paddies and all the extra toppings. Some local diners have even created some new milkshakes specially designed to help me get those last few hundred calories in. Personally, I would skip the deep fried mars bar, bacon, banana and peanut butter combo. Thanks to my team of supporters I am heading in the right direction, now the most important part is maintaining and finding a balance weight to stay at once I get there.
I am mostly followed by my Transplant team at Toronto General Hospital (TGH). TGH is home to one of the largest lung transplant programs in the world. It’s dedicated to helping patients through every stage of a successful transplant, offering specialized services to patients with even the most complex conditions. TGH has been the world’s first on many levels, including single and double lung transplantation for the long term. In 2016 the team at TGH preformed 145 lung transplants that’s a 13% increase from 2015. This year the team at TGH will be celebrating their 34th year of successful lung transplants in Toronto! This is a great success for the hospital and team of the program. The fact that they have been able to extend terminally ill patient’s lives for the last 34 years is beyond amazing and makes me proud to say that I am part of this program.
Even though my own lungs are no longer part of me, I still carry the CF disease. It’s found in my pancreas, sinuses and can still harbor harmful fungal bacteria in my lungs or sinuses that can affect CF patients. Aspergillus, Pseudomonas and Scopulariopsis Brevicaulis are all CF related bacteria. In my first transplanted lungs (2002) I had a lot of issues with Aspergillus causing serious lung infections and scarring leading to critical issues with my breathing. The sole reason for my second lung transplant was rejection. In fact, rejection is the main cause of death after lung transplantation. Doctors/scientist have discovered ways to slow rejection down but to this day they have not found how to stop it.
Unfortunately, survival of lung transplantation is lower than all other solid organ transplants. The five-year survival rate is only 60%, and the 10-15 year survival rate is as low as 20%. These rates are based off the first transplant and it is believed that a second transplant survival now is quite similar. However as for a 3rd lung transplant, there isn’t enough data out there to compare, it’s basically uncharted territory.
The major cause for lung rejection stems from small airways of the transplanted lung, or graft, beginning to scar. Slowly they become completely scarred and closed up. This process is called Bronchiolitis Obliterans Syndrome (BOS), the patient will begin to have shortness of breath again like they did before the transplant.
I can vividly remember both times when I started to notice the shortness of breath with my transplanted lungs. With my first transplanted lungs, I used to play paintball once a week. I remember running across the field with paintballs flying in my direction. I was trying to dodge them like the scene from Matrix. I didn’t feel as if I was as successful as Neo from the movie. Suddenly I was struggling for air and feeling so exhausted that once I got to the other side of the course I collapsed. The feeling was so odd because mentally I felt like running was possible, but in reality, my body wasn’t allowing me to. At the time I thought it could be a small infection coming, unfortunately, I was wrong it was far more serious.
After my second lung transplant, the dawning occurred while I was walking with my dog, Sheba. We were out on our usual walk which included a few small hills. I remember having to stop a few times to get my breath. It was the same feeling that I had experienced on the paintball field, thinking about it now I felt totally disabled realizing that the unavoidable was happening once again.
After each experience with shortness of breath, I was treated for low-grade acute rejection. I would be treated with high-dose steroid pulses typical prednisone as well ATG is frequently used. Rapamycin may also be introduced as a fourth agent. Other possible therapies are total Lymphoid Irradiation and Photopheresis, which are really last resorts. These drugs are very powerful on the body and mind. Your emotions are scattered all over the place. It is definitely an emotional rollercoaster ride. You experience happiness as if it’s the best day of your life, you feel as powerful as the hulk. Suddenly you transition to sadness, depressed the anxiety builds creating a hopeless sense of panic. And then you crash making you feel as if you’re in the darkest hole you’ve ever been, feeling lonely with no one to help, you wallow in total despair.
Not only is it a box of crayons of emotions, but mood swings to the point where the smallest simplest things get under your nerves; someone just looking at you can make you snap. It takes every ounce of control at this point, not to attack anyone. Mentally it’s frustrating and can make you feel even weaker. I haven’t shared these experiences with anyone. I have never wanted anyone to think I am mentally weak or weak at all. My barrier is my armor, a thick emotional shell keeping me distant and strong. I don’t want to burden others with my problems. After all, it was “me” that chose to enter these battlegrounds. Not letting anyone think I am weak has been one of my fears. Yes, I have said to live fearlessly however darkness can sneak into our life without us knowing, it creeps up until it has built a comfortable place in our imagination waiting for the right moment to attack.
Rejection is one of the scariest words you can tell someone who has received a transplant, it means with all the anti-rejection medications you’re taking your body is still identifying the transplanted organ as a foreign object. Your body is doing the totally opposite thing you want to be done, it’s building an army to attack what you have worked so hard for, bringing you back to where you started. If only you could tell your body that this new piece of equipment is keeping you alive. This is a chance for a normal life – a second kick at the can. Everything you’ve mentally and physically prepared yourself for begins to crash down.
One of the criteria for a transplant is to have a good support team of friends and family. Preparing and surviving a transplant isn’t a walk in the park, it’s not like you just get put on a list and wait for a phone call announcing the right organ has arrived. There’s a lot of pre-work as well post work to be done. A transplant is an on-going experience and being able to talk to someone or just even having someone around at times is vitally important.
I wrote a post quite a while ago about fear and how you can’t let it control your life, fear can damage the mind once it takes its grip. Fear is not a stranger we all have shared it at some time in our lives. There maybe a few individuals that say they’re fearless however have they really tasted fear? Fear is silent it will sneak into our minds making us question ourselves. A little fear helps to keep us on track. Contemplating fear keep you focused when making those important decisions in our lives.
March, 15th 2017, I had my 15th lung assessment. I have talked about what happens on this day previously however for those that aren’t familiar with my past, or transplantation I’ll go briefly into a typical lung assessment appointment. The purpose of my lung assessment is to follow up with progress (or decline) of my transplant. Unfortunately, I say decline at this time because the possible reality of being re-listed for a 3rd transplant means that a decline has occurred with my health. This is due to the rejection. The small airways that I previously talked about have been scarred causing them to close up making less room for oxygen to be made and more difficulty breathing.
Before I see the doctors I have various tests done. They include blood samples to measure drug levels, organ function levels such as liver and kidney as the drugs I take to suppress my immunes can be very hard on these organs.
I then go for an X-ray, the x-ray is to show if there is any possible fluid or infection growing in my lungs. X-rays will also indicate any visible cause for decreased lung function such as a collapsed lung even rejection. The team also likes to compare if there has been any change from the year before.
My PFT’s are used more at this point to monitor the rejection damage in my lungs. In November 2016 my lung volume was quite high, since then I experienced a few incidents of Pseudomonas. These occurrences have caused more damage to my lungs making my PFTs fall more than 10%. In fact, they are at the lowest they’ve been in the longest time .62. This makes getting around a little more difficult than normal as well I get tired quickly.
I’ve been trying not to let this slow me down or interfere with my days. For the past few weeks, I have been put on a heavy course of antibiotics to treat the Pseudomonas flair up. I can only hope with exercise and recovery that I can bring this number back up. Some days are better than others, which simply mean there are days that take a little more effort than others to get things done. However, I still haven’t let the Pseudomonas stop me. I can feel the shortness of breath creeping in while I am working out or walking. In the past I would try to ignore this occurrence; I’ve decided just to take things a little slower.
I even called my transplant coordinator before they contacted me letting her know how I was feeling. Typically I would wait until things got worse initiating a visit to emergency or an unscheduled clinic appointment. This time they reassured me through a sputum sample that my shortness of breath was caused by Pseudonymous. If the flare-up continues much longer I will probably be admitted to the hospital for an intravenous course of antibiotics as the team do not want to take any risks with my lungs.
With my second transplanted lungs, this time around I have exchanged the issues that I had with Aspergillus in my first transplanted lung with Pseudomonas in my second transplant causing a more difficulty and obstacle to jump through.
After my PFT’s I had my 6-minute walk. It is a test where you walk for 6 minutes and the values of your sats, pulse rate, blood pressure, and shortness of breath as well tiredness of legs and distance are all measured and calculated. Due to the decline in my PFTs the team wanted to see if I would benefit from the use of oxygen during this test. With 4 LPM of oxygen administered my lungs handled the test very well. The results indicated the active Pseudomonas has taken a toll on my lung function. I was still able to keep par with the predicted values of someone my stature. By stature I mean, size, gender, weight, age, they need to do this as if they compared my results to someone that is much taller than me or weighing more it’s obviously going to be different results. I walked a distance of just over 511 m in 6 minutes working out to being 96% of the predicted limit. At rest, my oxygen rate was 98% a pulse rate of 95 bpm during the test my oxygen rate dropped as low as 91% and my pulse rate increased to a 138 bpm. After my test during the recovery portion, I recovered very quickly almost to the point of where I started at rest. At recovery, my oxygen rate was 97% and a heart rate of 103 bpm.
Later on that day I met up with two doctors from the lung team to discuss my results and what kind of action plan they would like to implement. The team is concerned with the dramatic dip in my PFT’s. I don’t have much room for loss in this department. The last 6 months my PFT results didn’t increase but at least remained consistent and stable. They are okay with my results as long as they don’t drop lower.
A 10% drop in your PFT’s tends to set off alarms and gets the team worried. The team is only hoping that this drop is due to the Pseudomonas that has flared up and that I can recover any lost ground after the antibiotics kick in. I too can only hope for this, whenever I get results like this I feel defeated and useless. Remember how I told you never do I want CF to control my life, I would rather control my CF. When I get results like this I feel as if CF is controlling my life, yes I’ve said I no longer have CF in my lungs but if it wasn’t for CF I would not be in this boat nor would I be affected by Pseudomonas.
Next topic was my blood results, most of my results were at normal limits only two were slightly off. One of my anti-rejection medications Prograf was low sitting at a level of 6 units (the team would like my Prograf level to be sitting around 11-15 units). It’s been a constant adjustment with my Prograf level, either being too low or too high. The team increased my morning dosage to 1.5 mg and kept my night dose to 1 mg. My magnesium level was low this would probably be due to the increase of exercise as well my body not absorbing my supplements. I need to be taking them at a different time of the day away from my other medication.
The good news – the team was happy with my weight gain, as well with my blood sugars meaning my hard work of stuffing my face, counting carbohydrates, calories and sugars are starting to pay off. I’ve increased my weight by just over 6 pounds in four months. To most people that might not seem like a lot but for someone with CF that’s a huge gain literally. I still have a good amount of ground to go but I’ve started to head in the right direction. With more weight I’ll be able to make my body stronger and healthier, setting myself up for a positive outcome in the unknown chapters I am about to face.
The last thing on the board of discussions is the topic of a 3rd transplant. At this current time, the team feels that while I am not ready for a 3rd transplant I am heading in that direction. I live in an altered dimension, I am too sick to work, but I am too healthy for a transplant. I am not complaining what so ever, it’s just a long waiting game. If anything it gives the team and science more time to come up with better ways to work with organ transplantation and rejection. The team does feel since it’s been over two years now since I was assessed for a 3rd transplant when it comes to that time they would like me to repeat some tests. They need to make sure my body is strong enough to recover from a third double lung transplant. They have to be realistic; if my body isn’t strong enough to survive the transplant the lungs need to be given to someone who is. I totally understand where they’re coming from, as I would rather see the same outcome. The positive thing is the team is very familiar with my case and what’s going on. I am well known to the team and they follow me very closely. The only thing I can do at this point is continuing building myself stronger and updating the team with any changes.
End of the day, I’ve taken all the bad and weighed it with the good and with the good you have to take the bad. Yin and Yang, find a fine balance between both and you will find the course you’re set for. Signing up for a transplant in 2002 I was told you can exchange one set of problems for another, that a transplant is no cure for you. However we are allowing you to have borrowed time, it’s been up to me what I do with this borrowed time. I can only hope that I continue to put this borrowed time to full use. It’s like taking a vacation in a timeshare without banking my time. Fifteen years ago I was knocking on death’s door, I was at end stage CF there was no hope for me other than a Lung transplant. I was given the odds and potential possibility’s of what could happen however there is no crystal ball out there determining what course I am sailing on.
However, you know what?
“I’ve come as far away from that Lion’s den that I can. As rough as these waters might get with storm clouds full of lightning bringing crackles of thunder through the air, I am not jumping ship. I prefer not to return to that den for awhile, I’ll do whatever I need to do to stay out of that cold, damp, dark, wet den. As rough as the waters may get and as dark as the sky may become, there is the sun on the other side of those clouds that will bring me warmth and calm waters where I can dream”.
Don’t look back unless you can smile, don’t look ahead if you can’t dream.
In my dreams, I am smiling.