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

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

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

Contemplation

For the past few weeks, I’ve been looking for some guidance some extra light to inspire me, due to the fact I no longer know what I want or know where I want to be. I know there are still things in life I want and see myself accomplishing, I still set goals. It’s not that I have given up hope. However, I do know there are some places I don’t want to be. Basically due to the reason my 15^th lung anniversary and lung assessment was coming up I’ve been a little stressed. In a way, I’ve been looking for more reinsurance in my life and purpose.

I’ve been wanting to write an article that’s different to my usual blog about my yearly update regarding my lungs. I’ve been having mixed feelings regarding a 3^rd transplant, based more on whether it is worth it. I haven’t shared this with anyone other than my middle brother Matt and I think he’s found it difficult as he looks up to me a lot.

Just last week, I told Matt how I felt regarding a 3^rd transplant and what came out of my mouth didn’t really sound like me. Those who know me would say it wasn’t me at all. It was March 16^th the day after my lung assessment, Matt came to attend the appointment on the 15^th he came to listen in on what my doctors had to say as well to be there for support. The day after he texted me asking how I was feeling post assessment, and I gave him an honest answer.

I told him, “Meh, something probably to talk about more than in a text. In the long run of it, I need to get my life in order as to what I am going to do with my life. Otherwise, what’s the point to go through another transplant and continue this boredom.  Sounds selfish I know but when there’s someone else out there that could use lungs, at this point right now I would choose that someone else receives them”.

His response “That sounds honest. Not selfish”.

I responded with “I know that. But others would have a different opinion how I see it”.

His response was “How do you think they may see it?”

And I told him straight up “Like I am giving up!”

I don’t want it to seem like I am giving up, I’ve never been someone that has given up on anything. Partially because I have a team that works behind me very hard and have never given up on me yet. I also have an amazing support group that stands behind me 110%.

He asked me a good question today that has changed my entire view on things lately or at least reminded me how I got here or what got me here. At the time I was writing a new article for my blog, his comment totally through me off course, having me change what I am going to post first.

He sent me a text asking “Can I ask you two kind of hard questions?”

I responded with “What’s that”?

His question was “First. Why did you agree to your first transplant, and second, why did you agree to the second one?”

I first answered him with “Would you like to know what has changed from then until now? Due to the comment, I gave you last week? Is that what you’re trying to figure out?”

He told me. Nope that he totally gets how I feel. Again he reminded me that he didn’t think it’s selfish at all. And stated, “Anyone who sees it as selfish is being selfish themselves.”

So I gave him his answer and I said “I have your answer, I am just trying to write something at this moment. But in a short answer, it would be DRIVE and HOPE that got me through both transplants. At both times I felt there was a future ahead of me. I am not saying there isn’t one now. However being younger there had been more room for error I guess you could say. I had something to look forward to is basically what I am saying. Traveling, meeting new people, going to school, the potential of living on my own. There are others as well but these are just some of them. Let’s just say it this way. At the current state, I am at right now, it’s not that I hate my life, it’s more the chapter I am in is very dry.

I am lost; the author responsible for this novel has currently lost interest in the leading character. There’s too much silence in my life at the moment and I hate silence. We all have a story to tell, I am just worried that I’ve told my story and there’s nothing left to tell. I am in the land of limbo. Figuratively speaking and obviously, if I am not happy with my story I need to change it. Change my story, change my life. I know that I need help but at the same place where do, I start. It’s difficult as I don’t know what kind of story I want to start, therefore how do I change my life?”

I brought up the hate for silence, it’s not that I really hate silence more to do with I fear it. It’s a linking connection of over thinking. It starts back to being in the hospital when I was younger at Sick Kids while being admitted. In the middle of the night after my family would go home. I would wake up alone to the sound of silence of white noise. I know supportively I had people that cared for me, however waking up in the night with no one there hearing the white noise of electricity running through the wall giving power to the medical equipment around me would always start my mind over thinking, what would lay ahead of me, not stopping until I would exhaust myself of overwhelment and finally passing out. 

Matt’s question today has me thinking, my mind has been turning. I was hoping he could answer my own question to help me write a part of my blog. It wasn’t until I asked him did I see that I finally discovered how much I have impacted his life for the better.  He pointed out I have probably helped others with my story, with my experience and presence in their lives. This gave me the reassurance to find the light I’ve been looking for. It reminded me why I have chosen many times to fight for my life. It has brought back the lost drive and hope that had vanished from my life.

I asked him “Over the past 15 years of my transplants what stands out to you? Achievements, change, the story’s that kind of thing”.

He told me it was simple, he reminded me it was what was and wasn’t there after. Movies that weren’t punctuated by coughing fits were one thing. There was something really nice about knowing you were able to breath easy. The other part was you whistling, it warmed my heart to hear you make that noise.

Then he really told me something, he said there is one other thing. I’ve struggled with my purpose in life, a lot. But watching you survive, it gave me a new purpose. EXISTENCE TO EXIST. It became the thing that fueled him while he was going through a very difficult time of his life.

And then he assured me….

That it wasn’t just him I’ve helped.

The next part that he told me has really lit the fire inside of me; it brought back the warrior that has fought so hard to be here in life. My purpose of why I continue with the journey of my battle of transplantation. I have always found pleasure in helping others and give back by showing them there’s hope throughout my story of my life and that saying goodbye shouldn’t always be an option. My true biggest fear is saying goodbye to someone, you’re practically saying game over to me. It’s usually why I’ll tell you “see you later but never goodbye”.

Matt reassured me that he was sure this story would be echoed with a lot of people, that I just haven’t helped him. He understood how I felt currently in life. And that he’s not suggesting that what he felt makes it easier for me. In fact, if like him, it would make it harder. But that’s the part of what goes on as we get older. It gets harder. And for me, he felt that isn’t fair. This is why he didn’t think I am selfish.

I’ve helped a lot of people, and have earned my freedom to do what I want and to be responsible for all my own actions. My writing can go a long way to help that, as well my art.

He said “But Dan, you’re a project on your own. The story you give us is personal and special for each and every one of us. That maybe your author might not be writing your story to you right now. But they are for us and all of the people you’ve touched.”

“Purpose is a hard thing to define, especially when you’ve set such a high bar.”

I’ve learned if you want to get rid of a fear the best way to release it is by shedding light on the topic. We need to come out from the dark. We’re taught as kids if we are afraid of the dark to keep a flashlight or night light on or near. Instead of keeping those fears underneath our beds or in the dark closest expose them. The longer we keep them hidden the longer we remain weak and powerless. In order to build strength, you must work toward what is holding you back. We lift weights against gravity to build the force of our muscles; it’s the same idea with our mind. Allow purpose, light, and reason into your life and your mind will grow brighter.

This has been probably one of the most personal things I have ever shared in my life.  I share this with you in hopes that it will help someone else. You can’t allow fear to control your life. Expose your fear and you will get the help you need.

People that inspire others have a gift; it’s not taught, it comes from knowing experience by being in situations that we wouldn’t normally find ourself’s in. I am proud that I have been able to inspire others and help them with guidance in their lives. Its part of the reason why I continue with my life, I enjoy helping people.

I want to thank my brother Matt for reminding me why I climbed out of that lion’s den and continue to fight; you have helped me relight the fire within.

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 

In For An Oil Change

Getting hooked up to the plasmapheresis machine.

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

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

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

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

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

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

There are three kinds of rejection after a transplant:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Dan receiving Plasmapheresis

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

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

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

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

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

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

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

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

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

Plasmapheresis Machine

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

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

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

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

A Campaign With A Twist

It’s an odd way of a campaign, but Scarpa makes a good point of how most of us value objects and money instead of giving a life!!
‘I buried my car, but everyone thought it was absurd when I said I was going to do that. Absurd is bury their organs, which could save many lives. Nothing is more valuable. Be a donor, tell your family.’ 

The car was a New Bentley Flying Spur plus $400,000 it may seem like a lot to some, but to others, a life is worth more than money. Scarpa defiantly has a different kind of Campaign and something definitely to considered.The self-made businessman, who has a degree in economics and is a black belt instructor and owner of the Institute Maruyama Aikido, a Japanese martial arts academy, explains that his decision to start storing up his treasures for the life beyond came after watching a program on Ancient Egypt.

Scarpa is not new to the idea of facing death. In 2009 he was in a coma for over two months after a superbug nearly killed him following a stomach reduction operation. He ‘died’ twice during the ordeal and was given the last rites on both occasions by a priest. Count Scarpa says he was inspired to bury his most treasured possession after watching a documentary about the Egyptian Pharaohs.

With no children to carry on the family name, Scarpa says his cockatoo, is his offspring. ‘This here is my “daughter,’ he said in the interview with Caras.  ‘She is called Filomena Leopoldina Sofia Scarpa. She is six-months-old and is endangered,’ he said. The white cockatoo – which even has her own business card – is one of the most expensive birds in the world costing around £14,000.

The Pharaohs were known for burying their beloved pets with them so they could enjoy their company in the afterlife. So far Scarpa hasn’t said whether he plans to do the same with Filomena.

The hoax included a series of photos of Scarpa digging a Flying Spur-sized hole in his backyard, copping criticism from bewildered online followers who labeled him crazy and suggested the car should instead be sold and the money donated to charity.

The truth was revealed later when Scarpa appeared at a press conference for Brazil’s national week of organ donation in front of a banner reading: “It’s absurd to bury something much more valuable than a Bentley: your organs. I am an organ donor. And you?”

To further put his friends and public at ease, Scarpa later added to his Facebook page: “I have not buried my car, but everyone thought it absurd when I said I’d do it. It is absurd to bury their bodies, which can save many lives. Nothing is more valuable. Be a donor, tell your family.” 

 

Are You A Registered Donor?

 

The BIG QUESTION, are YOU A REGISTERED DONOR?

Tomorrow May, 6. 2013 I will be at the Timberlane Athletic Club (155 Vandorf Side Rd  Aurora, ON L4G 6W7) from 10:00 – 1:00. If you are in the of Aurora or Newmarket area, please feel free to come by and registered or even come and say hello!

If you registered as a donor through your drivers licence a while back, you’re no longer are an Organ Donor. The best way to check if you are a donor is to look on the back of your new Ontario health card, where 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.

A lot of people still think that Organ Donation registration is done through a driver’s licence, and it’s not. The new way of tracking and registering as a Donor is now done through your Ontario Health Card.

Organ and tissue donors are also able to register to be a donor through the website. Trillium Gift of Life recommends people check the website even if they have the proper designation on their health card. On the site, donors can confirm they’re listed and can update their information.

One organ and tissue donor can save up to eight lives and enhance as many as seventy-five more. By registering your consent to donate today, you can positively impact the lives of many Ontarians in need of organ and tissue transplants.

TODAY IS SPINNING FOR DAN!

 

Today April 27th is Spinning for Dan, a lot of you may not know what this event is but if you’re interested please read on. 

Spinning for Dan is set up to raise awareness towards CF. Now you are asking where does the spinning part come in, my friends Devin and Elise have set up an event in front of Lululemon at the Athletica Southcenter in Calgary, Alberta where Devin will be riding his stationary bike from open to close.  We are asking if you could help support Devin by placing a donation today! If you or your company can donate anything to help the cause that would be greatly appreciated. There will be a tax receipt given out for those who require one. As well, WestJet has graciously donated two round trips tickets to anywhere WestJet flies that will be raffled and drawn for at the end of the day. 

Place a donation. 

What is Cystic Fibrosis? 

 Also known as CF. It disrupts the normal function of epithelial cells — cells that make up the sweat glands in the skin and that also line passageways inside the lungs, liver, pancreas, and digestive and reproductive systems.

The inherited CF gene directs the body’s epithelial cells to produce a defective form of a protein called CFTR (or cystic fibrosis transmembrane conductance regulator) found in cells that line the lungs, digestive tract, sweat glands, and genitourinary system. When the CFTR protein is defective, epithelial cells can’t regulate the way chloride (part of the salt called sodium chloride) passes across cell membranes. This disrupts the essential balance of salt and water needed to maintain a normal thin coating of fluid and mucus inside the lungs, pancreas, and passageways in other organs. The mucus becomes thick, sticky, and hard to move.

Normally, mucus in the lungs traps germs, which are then cleared out of the lungs. But in CF, the thick, sticky mucus and the germs it has trapped remain in the lungs, which become infected.

In the pancreas, thick mucus blocks the channels that would normally carry important enzymes to the intestines to digest foods. When this happens, the body can’t process or absorb nutrients properly, especially fats. Kids with CF have problems gaining weight, even with a normal diet and a good appetite.

Humans have 23 pairs of chromosomes made of the inherited genetic chemical deoxyribonucleic acid (DNA). The CF gene is found on chromosome number 7. It takes two copies of a CF gene — one inherited from each parent — for a child to show symptoms of CF. People born with only one CF gene (inherited from only one parent) and one normal gene are CF carriers. CF carriers do not show CF symptoms themselves but can pass the CF gene to their children. Scientists estimate that about 12 million Americans are currently CF carriers. If two CF carriers have a child, there is a 1 in 4 chance that the child will have CF.

Almost 1,400 different mutations of the CF gene can lead to cystic fibrosis (some mutations cause milder symptoms than others). About 70% of people with CF have the disease because they inherited the mutant gene Delta F508 from both of their parents. This can be detected by genetic testing, which can be done in kids both before and after birth and in adults thinking about starting or enlarging their families.

Of all ethnic groups, Caucasians have the highest inherited risk for CF, and Asian Americans have the lowest.  1 of every 3,600 Caucasian children is born with CF. This compares with 1 of every 17,000 African Americans and only 1 of every 90,000 Asian Americans. Although the chances of inherited risk may vary, CF has been described in every geographic area of the world among every ethnic population.

Scientists don’t know exactly why the CF gene evolved in humans, but they have some evidence to show that it helped to protect earlier generations from the bacteria that cause cholera, a severe intestinal infection.

The diagnosis of CF is being made earlier and earlier, usually in infancy. However, about 15% of those with CF are diagnosed later in life (even adulthood). Symptoms involve the lungs and digestive organs and vary in severity.

A few kids with CF begin having symptoms at birth. Some are born with a condition called meconium ileus. Although all newborns have meconium — the thick, dark, putty-like substance that usually passes from the rectum in the first few days of life — in CF, the meconium can be too thick and sticky to pass and can completely block the intestines.

More commonly, though, babies born with CF don’t gain weight as expected. They fail to thrive in spite of a normal diet and a good appetite. Mucus blocks the passageways of the pancreas and prevents pancreatic digestive juices from entering the intestines. Without these digestive juices, the intestines can’t absorb fats and proteins completely, so nutrients pass out of the body unused rather than helping the body grow. Poor fat absorption makes the stools appear oily and bulky and increases the child’s risk for deficiencies of the fat-soluble vitamins (vitamins A, D, E, and K). Unabsorbed fats may also cause excessive intestinal gas, an abnormally swollen belly, and abdominal pain or discomfort.

Because CF also affects epithelial cells in the skin’s sweat glands, people with CF may have a salty “frosting” on their skin or taste “salty”. They also may lose abnormally large amounts of body salt when they sweat on hot days.

Cystic fibrosis is the most common cause of pancreatic insufficiency, but a condition called Shwachman-Diamond Syndrome (SDS) is the second most common cause. SDS is a genetic condition that causes a reduced ability to digest food because digestive enzymes don’t work properly. Some of the symptoms of SDS are similar to those of CF so it may be confused with cystic fibrosis. However, in kids with SDS, the sweat test is normal.

Because CF produces thick mucus within the respiratory tract, kids with CF may suffer from nasal congestion, sinus problems, wheezing, and asthma-like symptoms. As CF symptoms progress, they can develop a chronic cough that produces globs of thick, heavy, discolored mucus. They also may suffer from repeated lung infections.

As chronic infections reduce lung function, the ability to breathe often decreases. A person with CF may eventually begin to feel short of breath, even when resting. Despite aggressive medical therapy, lung disease develops in nearly all patients with CF and is a common cause of disability and shortened life span.

By performing genetic tests during pregnancy, parents can now learn whether their unborn children may have CF. But even when genetic tests confirm CF, there’s still no way to predict beforehand whether a specific child’s CF symptoms will be severe or mild. Genetic testing also can be done on a child after birth and can be performed on parents, siblings, and other relatives who are considering having a family.

After birth, the standard diagnostic test for CF is called the sweat test — an accurate, safe, and painless way to diagnose CF. In the sweat test, a small electric current is used to carry the chemical pilocarpine into the skin of the forearm. This stimulates sweat glands in the area to produce sweat. Over a period of 30 to 60 minutes, sweat is collected on filter paper or gauze and tested for chloride.

To diagnose CF, two sweat tests are generally performed in a lab accredited by the Cystic Fibrosis Foundation. A child must have a sweat chloride result of greater than 60 on two separate sweat tests to make the diagnosis of CF. Sweat test normal values for infants are lower.

Several other tests are standard parts of the routine care used to monitor a child’s CF:

Chest X-rays

Blood tests to evaluate nutritional status

Bacterial studies that confirm the growth of Pseudomonas aeruginosa, Staphylococcus aureus, or Haemophilous influenza bacteria in the lungs (these bacteria are common in CF but may not affect healthy people exposed to CF)

Pulmonary function tests (PFTs) to measure the effects of CF on breathing (PFTs are done as soon as the child is old enough to be able to cooperate in the testing procedure; infant PFTs are currently being studied)

When kids are first diagnosed with CF, they may or may not have to spend some time in the hospital, depending on their condition. If they do, they’ll have diagnostic tests, especially baseline measurements of their breathing (lung function) and a nutritional assessment.

Before they leave, their doctors will make sure that their lungs are clear and that they’ve started a diet with digestive enzymes and vitamins that will help them to gain weight normally. Afterward, they’ll probably see their doctor for follow-up visits at least once every 1 to 3 months.

The basic daily care program varies from person to person, but usually includes pulmonary therapy (treatments to maintain lung function) and nutritional therapy (a high-calorie, high-fat diet with vitamin supplements). People with CF can also take oral doses of pancreatic enzymes to help them digest food better. They may occasionally need oral or inhaled antibiotics to treat lung infections and mucolytic medication (a mucus-thinning drug) to keep mucus fluid and flowing.

A new treatment for CF, which is still being researched, is an inhaled spray containing normal copies of the CF gene. These normal genes deliver the correct copy of the CF gene into the lungs of CF patients. Since 1993, more than 100 CF patients have been treated with CF gene therapy, and test trials are underway in at least nine different U.S. medical centers and other centers around the world. Another new therapy, called protein repair therapy, aims at repairing the defective CFTR protein. Numerous medications, including a spice called curcumin, are also being tested.

Caring for a someone with CF can be tough at times, but parents need not feel alone. Doctors can usually refer them to a local support group linked to the Cystic Fibrosis Foundation.

CF definitely affects the lives of people who live with the disease but it also affects others around them such as family. I was born as a twin, Amy wasn’t born with CF. My family was the typical 1 in 4 for a child to have CF. Both my parents carried the CF gene leading to myself having CF. As I said Amy is my twin but wasn’t born with CF, however, she still had to live growing up watching her twin brother live through this disease.

Here is a little view of my sister Amy and I as we were younger.

Elise Miles, Devin Featherston

Elise Miles, Dan Pfaff, Amy Pfaff

Sixty-five roses.

That’s how you help train a small child into maneuvering their tongue and lips to form the difficult sound of Cystic Fibrosis. You know that I know that, so why do doctors pick such difficult names for diseases that affect children who can’t even say spaghetti or napkin correctly? 

Dan, it’s taken me a long time to decide what story I wanted to share with you. How do you summarize a life shared?  Really, my life would not be the same without you. You have given me the confidence to wear a large range of hats. We started our lives together in the safety of amniotic fluid. Quickly I evolved into personal translator when even Mom didn’t realize that opicl was Popsicle. As we grew older I changed my hats to meet our needs from homework provider to your very own donkey.  I am sure the list could go on but that is not the point of this story, nah this story is about you and what hats you have worn for me.

On April 2^nd, we both came screaming into this world, you came first and I followed shortly.  The minutes in between really do not matter. What is important is that you came first, and thus empowering you with bragging rights for the rest of our lives. The significance of these few minutes apart officially established you as my older bro. This also decided you would have to accept the responsibility of protecting me. In one moment, in one doctor’s act, you became responsible for me by the universal act of big brotherhood.

 Now, every little sister knows big brother is code for personal tormentor. It means you have someone to punch you, to make you cry and place blame on. It also means you have someone constantly watching over you, protecting you when you don’t even know it. Mom never had to worry about chastity belts or bullies in high school… we had you.

I remember one moment during high school that made me realize how far your ever protecting shadow went. We were sitting in the front row of our grade 9 science class. I was clearly paying attention to Dr. Churkas when you nudged my arm. Slightly annoyed, I turned and noticed you were pointing to a section of our science textbook that read CYSTIC FIBROSIS. I’m not sure if you found it by accident or you went looking for it but there in our crisp new textbook was information on Cystic Fibrosis. It was the first time I had ever read about CF.  Wikipedia was floating around in someone’s mind waiting to come to fruition and the thought of finding a book about CF had never occurred to me. Seeing the text on paper made CF real. At the end of the short summary, a single sentence stated, the average lifespan for children with CF was 16. You and I were just 14.

I often think of this moment. 

It’s the day that I started to really understand. It was also the day that I realized how much you deal with. That somewhere under your big brother armor you carried the weight and not just on your shoulders but your heart too. You never complained. You never said why me. You never showed fear. No, instead, you made the choice to always be my big brother first. To protect me from all the scary things that life had to throw at us and you shouldered it, including your own reality. You could have played the pity card to get the front seat of the car, but instead, you always played the big brother.

There is one other story that I felt the need to share. This one takes place about three years ago after your 2^nd transplant. I had come to visit you in the ICU and was reading you some of the messages from the “DAN IS MY HERO” Facebook page. While reading, I could see your chest moving up and down, hear the rhythm of your new lungs working so hard to take in air. Slowly you would drift off to sleep, your breathing slowing, slowing, and slowing until… it stopped. An alarm would sound; I remember waiting, waiting to see your chest rise, for the moment your brain said breathe again but nothing happened. I guess any other person would have grabbed the doctor and freaked out but I’m your little sister and like a well-trained sister I simply just yelled at you.  “ DAN BREATHE!!”  It was like my words were an electrical shock because you sat up in bed, blue eyes wide open, and your lungs back into action and a hand gesture that stated ‘keep reading, I’m fine”.  This process went on for a large portion of the day. You would drift off and I would remind you to breathe. The funny things were, even though you were having a hard time, you still would have me read on. Faced with a huge problem or difficult situation you always seem to remain calm and level headed. Yeah, I may have had to remind you to just breathe but that is a day in the ordinary life of us.

I would not be the person I am today without you. You helped mood the caring person I am by shouldering a truth that I was just not ready for yet. You made me realize that I am worth something by fighting off boys who just were not good enough. But most of all you taught me how to be brave, stay focused and keep calm when the going gets tough. 

Thanks, big brother,  without you, I would not be complete.

Hugs

Your other half.

Those of you who can’t make it to the event today or live outside of Calgary, you can place a Donation Here!

Let’s make CF mean Cure Found!