A Family Who Embraces Each Other

Julie’s Perspective:

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

Marissa’s Perspective:

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

The Gifts Of A Fight With CF

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.

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

Life’s Fragility

 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.

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

But We Are Warriors

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 Ashley’s 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.

“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

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 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.

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.

CAN YOU SEE ME NOW

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.

BREATHE

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! 

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.

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 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.

HERO-AS-SELF

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.

Just to make it clear your current or past medical history does not prevent you from registering to be a donor. Individuals with serious illnesses can, sometimes, be an organ and/or tissue donors. Each potential donor is evaluated on a case-by-case basis.

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.

Pancreas transplant the pancreas makes insulin and enzymes that help the body digest and use food. A common reason for this type of damage is diabetes.

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.

ON – https://www.beadonor.ca

BC – https://register.transplant.bc.ca/verification

AB – https://myhealth.alberta.ca/Pages/OTDRHome.aspx?selected=overview

SK – http://www.cantransplant.ca/saskatchewan-organ-donation.php

MB – https://www.signupforlife.ca/

QC – http://www.signezdon.gouv.qc.ca/index.php?accueil-sansjs

NL – http://www.easternhealth.ca/Give.aspx?d=2&id=2223&p=323

NB – http://www2.gnb.ca/content/gnb/en/departments/health/Hospital-Services/content/organ_donation.html

PE – https://www.princeedwardisland.ca/en/information/health-pei/organ-and-tissue-donation?utm_source=redirect&utm_medium=url&utm_campaign=organ-and-tissue-donation

NS – http://www.novascotia.ca/dhw/msi/docs/MSI-Organ-Tissue-Donation-Form.pdf

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.

Think green, recycle yourself!

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.

“I want to be remembered for the life I gave, as well the life I lived!”

Register Below Become an ORGAN DONOR

Life is A Card Game

Today is a special day, as well as yesterday even the day after tomorrow will be and so on for myself and others. March 21st marks 6 years since my second double lung transplant, followed by March 22nd making it 14 years from my first transplant. My family, friends and I acknowledge them as lung anniversaries just a little extra celebration that we are thankful that I am here today. Every day after are special, they are extra days, hours that I can tack onto those 14 years of being here enjoying life and making memories with all of you.

Recovering in ICU from the second double lung transplant.

I count each day as a blessing and I am so grateful for my organ donors and their family’s for giving me the gift of life not once but twice. As well the hard work from the Doctors and nurses that have helped me along this journey. Last year was just a little reminder to me for how important life really is and how lucky I am to have received these gifts.

My last blog post was written last February 2015 “In for an Oil Change” talks about how my lungs were in rejection and the treatment that the transplant team wanted me to follow. Plasmapheresis, IVIG, and IV Pulse Steroids. I always planned on giving an update on how I was doing after this treatment, I just didn’t know how and when was the right time to post it. What better time than a day like today; so special but what can I say EVERYDAY IS SPECIAL.

February 13, 2015, I was discharged from Toronto General Hospital after my Plasmapheresis treatment was completed. I had received my first few courses of my new treatment called IVIG and would require another 6 to 12 depending on my body’s tolerance. It would mean coming down to the transplant day unit at TGH each month and spending a few hours hooked up to an IV.

The IVIG was set up to treat the antibodies that were found during my last lung bronchoscopy, you might have already looked up what IVIG is, if not let me give you a little information on the treatment. 

IVIG is a blood product that is ministered through an IV. It is made up of immunoglobulins sort from IG, which is also called antibodies. These antibodies are clean antibodies that are to replace the ones that were attacking my transplanted lungs. Antibodies help protect the body from germs, such as viruses, or in my case, my body thought my transplanted lungs were viruses.

The treatment of IVIG is the IG that is prepared from the plasma collected from a large number of normal individuals, usually between 10,000-50,000, who have been carefully screened to make sure they are healthy and do not harbor certain infectious diseases. The plasma contains a broad range of specific antibodies to many different types of bacteria and viruses. Each plasma donor must be acceptable as a blood donor according to the strict rules enforced by the Canadian Association of Blood Banks. Donors are screened for travel or behavior that might increase the risk of acquiring an infectious disease. Only the IVIG is purified from the pooled plasma. To commercially prepare the IVIG for patients with primary immunodeficiency diseases, the immunoglobulin must first be purified (extracted) from the plasma. 

IVIG replacement therapy is generally administered either intravenously (abbreviated IVIG), or subcutaneously (abbreviated SCIG). IVIG infusions are usually given every three or four weeks. SCIG infusions may be given as often as daily, weekly, or as infrequently as every three to four weeks (similar to IVIG), depending on the specific SCIG product that is being prescribed, the patient’s age and the preferences of the patient and the prescriber.

Before the treatment, I am given a dose of Benadryl in the case of any allergic reactions from the IVIG.  My vitals are followed closely as some of the side effects include a headache, nausea, low-grade fever, chills, rash, neck/back stiffness, hair loss, and fatigue. Generally, these side effects are mild and tolerable and most often, they can be alleviated by decreasing the rate of infusion. More serious side effects, such as allergic reactions, are rare but have been reported. 

Due to my small size, the treatment only takes a few hours as the dose is based on size and weight. I am also given a large pulse of prednisone to try and treat the rejection.

This becomes a monthly routine for about 3 months, at this time there wasn’t much improvement or results, in fact, I could say my PFT’s, (oxygen saturation) was getting worse. I was really tired; my weight was dropping. I didn’t have much energy throughout the day and started a bad cough, my muscles ached as did my joints to the point I could hardly move. I also started to notice some hair loss. 

My cough was so bad it got to the point where I made an appointment with my transplant team. I went down the following day to Toronto General Hospital after calling them as the team were concerned with my condition and were following me closely. I did all the regular tests (blood, X-ray, PFTs as well a sputum culture) before the team saw me. Most of my test results hadn’t come back by the time I saw the team. They put me on a high dose of oral antibiotics and told me if I felt as if my condition was getting worse to call them and come back down.

A week passes and I still did not feel well, in fact, I felt like death, my breathing worsened my body was stiff, overall things just sucked. I heard back from the hospital and they said my sputum culture came back growing Pseudomonas. 

Pseudomonas is a bacteria/fungus that can be found grown in the blood or lungs causing infections. They wanted me to start IV antibiotics. The team felt that I could just do home IV’s all I needed was to get a pic line put in. The team was going to arrange the pic line to be put the following day as well set up home care. 

A pic line is a peripherally inserted central catheter; a form of intravenous access, it extends to the superior vena cava that can be used for a prolonged periods of time. I returned the following day to get my pic line put in and my first treatment of antibiotics at the transplant day unit. This was followed by a 2-month course of home IV treatments.

 During this time I was still getting treated with my monthly IVIG.  I was into my 5th treatment and the side effects were getting worse to the point where I could barely move. Walking up stairs took so much effort as the pain would shoot throughout my body with each step I made. Little tasks such as climbing into bed would take 5-10 minutes – so much effort and pain. Trying to open the jeep doors and climb in was physically impossible; the pain that radiated through my body was unbelievable. I felt like I was being electrocuted.  There were times I wanted to move my hands or legs mentally yet physically nothing happened. I felt crippled and depressed, my life was being tested. I spent most of my days just sitting on the couch trying to sleep as I wasn’t sleeping well at night. 

During this time I remember writing in one of my journals,

“The world you’re used to is getting smaller, my limitations are becoming a sad reality. The easiest task turns into a day’s project, making me feel useless.  I watch my body detransform into a broken down operating system.  It mentally irritates my mind erasing all the goals that had been chosen for that day.  The lack of oxygen running throughout my body and the pain that has staked its claim make simples tasks so challenging.  I need to rest and prepare for the next day, another long day in hell.”

We often make tomorrow the busiest days of our lives as we feel we can just push whatever we don’t accomplish today into another tomorrow.  Not many of us live in the moment. The past is behind us, the future ahead but each moment is only lived in the now.

My motto is to live for today. I have learned to fill my days to the fullest. Go see a friend across town or go out and see a family member or better yet go travel. Take that walk or do that drive you been thinking of, just try and stay busy. Live every day as if it’s your last, the little things do count.

What would you do if I told you tomorrow would be your last day alive?

I bet most of you wouldn’t know how to respond. You may say one thing but wouldn’t follow through.

Why is that?

During my “difficult times” last year I wasn’t living much of my life. I was simply existing; I would watch the days and nights go by slowly without being able to do the things I loved, nothing brought me joy. My mom saw a big change and was worried. I was tumbling into depression and had no energy to change it. I felt useless, withering away, couldn’t do much about it, shortness of breath plagued each moment, I couldn’t get anything done.

As June approached Tanis was definitely worried.  She decided that she would take matters into her own hands. She contacted the hospital and told them she was worried for my well being and thought maybe it was time to have another clinic appointment.  She wanted me to go on home oxygen so my body wouldn’t have to work so hard. My body at this time was using so much energy towards breathing that it was breaking down muscle and body weight to be used as energy. 

I head back down for a follow-up to the hospital this time I did all the usual tests that the team looks at, before seeing me. I had an extra test the team wanted me to do called a 6-minute walk. It’s a test to see how much oxygen I was using while exercising. This test is performed to see if you require the use of added oxygen. The way this test is performed is by two 6 minute walking tests. You are constantly monitored to a desaturation device that alerts the technicians when your oxygen levels drop below 85%. The first test you walk without the aid of oxygen and use room air. The second test is done with oxygen to see if your body benefits from the use of oxygen. You aren’t told what test you are performing as it’s all done randomly because they want it to be a clean test. If your oxygen saturation drops down to 85% during the test without oxygen you may apply for the use of home oxygen.

In my case, after about 3 minutes into the test without oxygen, my O2 sats were 85% and dropping making me eligible for oxygen. For those of you that do not know what O2 sats are it’s referring to the fraction of oxygen-saturated hemoglobin relative to total hemoglobin (unsaturated + saturated) in the blood. The human body requires and regulates a very precise and specific balance of oxygen in the blood. Normal blood oxygen levels in humans are considered 95-100 percent.

I head back upstairs with my mom and brother Matt for my transplant appointment and we meet with the team. They were glad to see that I qualified for oxygen and hoped it would help with the aid of adding weight and helping with giving me more energy.  I brought up my concerns regarding the IVIG. I felt it wasn’t benefiting me as my PFTs were decreasing and my body was stiffening and my joint pain was getting worse. In fact, at this point in time, you could start to see what looked like my joints swelling up almost double in size in my hands. The Team decided that we could stop the IVIG treatments and scheduled me to see Dr. Rubin who is a Rheumatology Dr at St Michael’s. The team was interested to see if the IVIG had increased my arthritis. 

There was one more topic the team wanted to discuss with me, quite a serious topic actually. Re-transplantation was said. Once the doctor said it out loud it was if a bomb went off, the room fell silent; heaviness fell silently into the stunned silence. I could see the concerned look on my mom and brothers faces. As if I was being taken away from them. The first thing that was said after the bomb went off, was

“You mean a third lung transplant”? 

“Yes a third double lung transplant”, the doctor answered very calmly. I didn’t react the same way as my brother and mom. I remember looking the doctor straight in the face giving her the sign that she had my full attention, at the same time my brain was in overload.

I didn’t take this as a negative sign, yes the word re-transplant was just brought up but at least it is being brought up as a chance offered or at least being considered by the team. 

With the topic being mentioned this brought up some serious questions to be asked.

Question one- do they really think I needed to be re-transplanted; their answer was it really is up to me as only I knew where my quality of life was heading.

Question two has a third lung transplant ever been done in the past. This question wasn’t answered right away, but with the blank look on the team’s faces, I took it as a “NO”. In fact, there has never been a third lung transplant performed anywhere.

With everything being said I still had a smile on my face, yes I’ve just been told in a way I am dying, but I have also been told there could be another chance at life. Why would I want to give up now? I’ve been here before it’s not the best feeling in the world but I enjoy Life and when given a chance to know there are options I’ll fight and do whatever it takes. I also know I have an amazing support team behind me to pick me up whenever I need it.

This brings me back to where I ask you if you knew today or tomorrow was your last day what would you?

One thing I can say is I am blessed with are, friends and family. My grandfather always told me when you are older and you can count on one hand the total of loyal friend you have at the time and fill a full hand then you have lived a successful life. Well, thanks to many of you I know I could use all my fingers and toes and more. Knowing this has always given me the feeling as if I have an army behind me as I am always preparing for war.

The Dr asked me how I felt regarding a third transplant. I told her it’s something, definitely something to think about and I wasn’t going to turn down as I love living and enjoy life. It was just a matter of when the right time would be. I was then told if I would like to start the process of being re-listed on to the transplant list. This involves many tests that would be reported back to the team, just because the team thinks I would benefit from a third transplant does not mean I qualify for one. A series of tests need to be performed and then evaluated by a board of doctors to see if my body is strong enough to accept another transplant.

Now, this wasn’t something I didn’t want to announce to the world yet. One I wasn’t sure if I would be put on the list as my case would have to be brought to the transplant team and from there they would decide whether or not I can be a candidate as well if they want to see what my test results looked like. A transplant is very hard on your body a third transplant is unimaginable. 

Yes, I require new lungs to live but if my heart, kidneys, liver and other organs and body aren’t strong enough to survive the battle why give me new lungs when someone else would benefit from them more.

I plan to talk about this in another blog soon as I feel there has been a lot brought up already, I promise. 

Two weeks pass by and I am on 24/7 use of oxygen. I had to go see Dr. Rubin at St Michaels hospital regarding my case with the pain throughout my bones and to see if he could answer why my joints were swollen. Once Dr. Rubin comes into the room and sees and feels my hands he instantly wanted to send me in for a few X-rays that day as well a few bone scans however the bone scans could not be performed that day and needed to be booked for the following weeks. 

After I had a few X-rays on my hands, hips, feet and spine I return to the room to see what Dr. Rubin had discovered. He was at a loss for words and couldn’t say much other than there wasn’t any extra arthritic activity going on in my body and that the X-rays showed that it wasn’t in my joints at all.  The x-rays showed swelling just before and around the joints as a blurred picture on my bones. He couldn’t give me any results yet and would like to see what the bone scans showed as well send me for a few blood tests. Some of the tests included vitamin levels. He was interested to see mainly if my vitamin A level was increased as it is a cause for increased calcium build up.

A few weeks pass by and I get a call to come in for my bone scans as well the same day a message from Dr. Rubin that my vitamin A levels were quite high. This is odd as with having Cystic Fibrosis my body doesn’t absorb vitamins well at all and I am required to take a few supplements to help with this. With this being one of the findings he asks me to stop daily vitamins as well my vitamin A and have my blood work retested the next time I am down at TGH.

I have my bone scans done completely a full body CT scan, as well my blood work drawn the same week. A bone scan is a nuclear medicine imaging test that uses bone-seeking radioactive materials or tracers (radiopharmaceuticals) and a computer to create an image of the skeleton (bones). A bone scan looks at the bones to see if there are any abnormalities, such as a fracture, tumor or infection.

You may be asking what the difference between a bone scan and X-ray is 

 A bone scan is used to show bone density and is used to show more detail it’s a highly sensitive test it can also be used to determine if a compression fracture of the vertebral body is old or new, as an old fracture will not light up and a new one will. It can locate damage caused to bones by infection, breaks or traumatic injury, and is helpful in finding bone cancer too. Bone scans are sometimes performed to rule out an inflammatory process like a small tumor or infection or an occult fracture which is small fracture not seen on an x-ray. 

Bone scans, however, cannot distinguish what a lesion represents, and therefore cannot differentiate between a tumor, an infection or a fracture. Therefore, this type of imaging study usually needs to be followed by a CT scan and/or MRI scan to better characterize the lesion. 

The results of the test reveal ‘hot’ and ‘cold’ spots. Hot spots appear darker on the image and denote an area of high tracer uptake, possibly indicating an abnormality (e.g. infection). Cold spots appear light and indicate the bone absorbed less of the tracing element. 

During the time I was waiting to hear back from Dr. Rubin, I got a call from my transplant coordinator telling me that my kidney and liver enzymes were very high and they were worried and wanted me to stop taking my drug called Voriconazole. I am treated for Voriconazole for the Aspergillus that has been growing on and off in my lungs and sinuses.

Aspergillus fumigatus is a ubiquitous fungus found worldwide in soil, decaying vegetation, foods, dust, and water. Normal hosts quickly clear a fumigatus upon exposure, whereas it often persists in the respiratory tracts of patients with CF. Reported prevalence rates for a fumigatus based on sputum cultures range from 10% to 57% in patients. Prevalence of infection may depend on clinic geography as well as individual clinics’ surveillance methods for endobronchial fungal infections. Culture positivity for A fumigatus has been associated with increasing age, antibiotic or steroid use, indwelling catheters, and Stenotrophomonas maltophilia colonization. 

The regular treatment for Voriconazole is 3 months on 3 months off however whenever I would come off the Voriconazole after my second transplant the Aspergillus would flair up making me sick and usually needed to go in for a hospital admission for IV antibiotics for several weeks. So the team came up with the idea of a new treatment of staying on Voriconazole continuously.

Voriconazole is used to treat serious fungal infections such as invasive Aspergillosis (a fungal infection that begins in the lungs and spreads through the bloodstream to other organs). It is also used to treat esophageal candidiasis (a yeast [a type of fungus] infection that may cause white patching in the mouth and throat) and other yeast infections of the skin, stomach, kidney, bladder, and wounds. Voriconazole is in a class of antifungal medications called triazoles. It works by slowing the growth of the fungi that cause infection.

Some of the side effects are sudden behavior changes, problems with thinking or speech; upper stomach pain, itching, loss of appetite, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes); urinating less than usual or not at all; bone pain, swelling; uneven heart rate, chest pain, general ill feeling; or severe skin reaction; fever, sore throat, swelling in your face or tongue, burning in your eyes, skin pain, followed by a red or purple skin rash that spreads (especially in the face or upper body) and causes blistering and peeling.

A few days after my bone scans were complete I received a voice message from Dr. Rubin himself, and he sounded quite excited to talk about the results he found. And wanted me to come into his clinic the following day.

Once again I head down to St Michaels hospital to see him at his clinic. He saw that I was in the waiting room and called me right into an exam room so we could talk. On the screen of the computer was my bone scan, as I looked at it I could see a skeleton.  There were dark spots all over the areas where I was feeling pain or noticed large swollen areas on my body.

He instantly said,” bone growth”. I said “pardon”.  “It’s not arthritis that you are experiencing its bone growth. Throughout my body, I was experiencing new bones growing on old bones. He said No wonder you have been in severe pain.”

“So am I like wolverine”, I said in a nervous voice. He instantly replies with no I sent your case out all over the health network to see if anyone has seen or heard anything regarding this issue before. It’s very rare however the cause is an increased vitamin A level making your body increase calcium levels that are trying to repair any bone injury or even the slightest hit to a body part causing bruising. My body would send out a chemical to try and repair the injury and with this was building the bone to protect my body from future injuries.

This was all due to the Voriconazole increasing my Vitamin A levels. Also a result of my hair loss I was experiencing. The levels of Voriconazole were so high my body was starting to have a form of toxic shock. So, in the end, the IVIG was not causing the negative side effects to my body.

A month passes of being off of the Voriconazole and I start to notice a big improvement throughout my body. The pain that I was having was slowly melting away as well the swelling in my hands and feet. Dr. Rubin told me I will be left with the extra bone deposits throughout my body.  The growth of the bone will stop but he would like to follow my case as it is very unusual.

With the pain gone my body started to get back to a normal state.  It allowed me to fall back into my routine of being able to work out.  I slowly increased the intensity of these workouts, becoming more mobile and returning somewhat back to my “normal” life. I slowly started to need less oxygen to the point that now I only use it when I am working out.

I noticed a big difference in my life; I was able to see some light at the end of the tunnel. I felt almost whole again, I still knew I have a large journey to face. 

A recent photo of Sheba and I out on the trail

Last week was my 6th-year lung assessment (March, 16. 2016), it’s where I go down to Toronto General Hospital to perform a bunch of tests that the doctors can review and compare how well my lungs are performing. The team seemed to be happy with what has gone on over the past year. There are some issues that I still need to work on such as weight gain and continuing to keep my blood sugar levels low. They were also concerned about my immune suppressed drug levels being too high. My PFTs levels have stopped dropping, in fact, they went up .5%.  It’s not much as they can fluctuate up and down a little but it’s heading in the right direction. I do not have much room for any more decreases in my PFTs. 

After all the results and test were reviewed with me my team asked how I was feeling towards being put on the transplant list.  I answered that when my quality of life is no longer worth living I will consider it. 

6+8=14. Fourteen years I have been able to add to my life solely because two families knew becoming organ donors was the right thing to do. This has enabled me to “Live my life as much as possible”.  I can be that happy person who can go out and be the life of the party.

Playing cards with Tanis in ICU

The other day my Mom posted, “Imagine you are in a card game. The stakes are incredibly high. If you play the wrong hand you’ll die. If it so happens that the cards fall in your favor – you get to live – there are no guarantees for how long or how well”.

Life is a card game not everyone is dealt the best hand to play. The outcome of you winning or folding is how you play the game. Some of us have learned the art of bluffing, having fun and making it a social event. Taking a bad hand and winning despite the odds, you never know what the person you’re playing with or against may have in their hand. My Mom says I’m a card shark – I think I am.

Become an organ donor