My life has been based on numbers, predictions, percentages, baselines, decisions made by a team of doctors. I am not saying that I have not been a part of any of these decisions, in fact, I am the final call on all the end decisions made. It’s my story, right? Whether I chose to let this story end or add a new chapter into my life is all up to me.
I would say over the last 6-7 months I have been trying to change myself for the better, to believe the unknown chapters of my life will continue. Guess you could say I have been trying to rebuild myself for the better, make myself stronger! What’s the saying “Better, Faster, Stronger” right? Sometimes it’s not always faster, but you get the point. We all have flaws that we aren’t proud of in our lives. Some help us stay motivated when we fall off track and others lead us in directions that may not give us a chance to recover from. But in the end, it’s all a life lesson and as long as we gain from it, we can recover for the better, but you have to choose to want to be better.
Growing up with Cystic Fibrosis (CF) I never let people really know about my disease. I didn’t want to be judged or looked at me differently. After all, I tried my hardest to blend in or act as normal as any another kid. I might have had the odd coughing fit but besides my short stature and my cough, I acted as normal as I could. I was feistier than most kids my age but that came with the territory. Dealing with the many different daily obstacles that living with CF, presented my character grew. Hell, I even fooled myself at times that I was as normal as my friends until it came time to take my meds or do any of daily therapies.
I was diagnosed with CF at birth. At that time CF wasn’t well known. To this day when I meet someone I don’t announce that I have CF right away or that I have had two double lung transplants. “I never wanted CF to control my life, I wanted to have control over my CF life, or at least have as little footprint in my life as possible”. I don’t want people to judge me or treat me differently or base their first impression on or what I have gone through. There is a good saying I like to believe in “I am not what you think I am. You are what you think I am”.
March 22nd will be my 15th year lung anniversary (March 21st will be 7 years from my second lung transplant) what are the odds of my anniversary’s being a day apart? I have experienced an additional 15 amazing years of my life all because two heroes saved me. I owe everything to my two organ donors as well as their families. Their unselfish act has given me the ability to continue my story here on earth.
We’re all here for a reason whether it’s to recreate, help build this world for the better, save a life or even provide guidance for others, we are born with a destiny to be fulfilled. Some may not even know they have achieved the task and continue with their life, some may not even reach the goal life has set for them. But one thing we all can do is become an organ donor. You never know who you’re going to save it could even be your own family or friend.
Fifteen years ago I made a decision, not only would it change my life but also the lives around me that followed my life’s journey. I would like to think of myself as an individual who leaves a self-impression on others and to let them see another side to life. We take life too seriously most of the time and need to let loose more often than we allow.
I hope to leave to all those that have touched my life an important message. There will always be time to be serious. Life can pull us in many directions causing stress and anxiety. Balance is critical, make sure to make time to have fun, to try new things. Life is meant to be experienced for the moment. Don’t live a little, live a lot! For this reason, I tend to say “yes” to most things that I should sometimes say “no” to. We never know what opportunities lay ahead. New stories are contrived by taking chances; new road opens up new opportunities.
Being faced with many negative predictions, I guess I have grown to like a good challenge in my life.
Growing up with CF I have been faced with numbers all of my life. These numbers include everything from statistics on mean average age, life expediency, odds, predictions, values, goals, medications, the list goes on.
Having CF has definitely challenged my life, I candy coat nothing. The dark and scary times this disease has brought rough sailing waters my way are many. I try to avoid sharing these episodes with people. Seeing smiles on their faces makes me happy. I prefer this to the sullen concerned look of my family and friends when they find out I have another storm to face.
Living with CF has definitely meant that I have had to navigate through turmoil. The side effects from the medication I have been on the majority of my life have been responsible for much of it. CF harbors fungal bacteria that can change a successful lung transplant into sinking ship. I personally have had to face two double Lung transplants, Lymphoma, MRSA (Methicillin-Resistant Staphylococcus Aureus), Squamouse Cell Carcinoma, Diabetes (Cystic Fibrosis Related Diabetes), 15 nasal polyps’ removals, Pleurisy, Bowel Obstructions, Rejection, Aspergillus, Pseudomonas, Scopulariopsis Brevicaulis, Osteoporosis. The countless small procedures include a G-tube, Ports, Pic lines, Chest Tubes. I am also written up in the medical textbooks as having the highest Blood Sugar levels the team has ever seen come in through Emergency. I am on deck for a third transplant. These challenges have molded me into who I am today. They build my morals; strengthen my psyche, expanding my character in this story called “Life”. It’s taught me that you can’t stop the waves but you can learn how to surf.
Strangely one of the many challenges CF has given me over my life is a problem most people would love; weight gain. For those that know me, I am a bottomless garbage can. People either find it disgusting how much food I can eat or wish they could eat as much as I do and not gain an ounce. In reality gaining weight has been impossible for me for the last few years. If I want to keep the options of a 3rd transplant open it is crucial that I maintain a healthy weight.
Having CFRD (Cystic Fibrosis Related Diabetes) doesn’t help the weight problem. CFRD is manageable however it needs to be followed closely. I am constantly calculating, adding and subtracting in my head how many carbohydrates and sugars I am about to consume. This is how I determine the amount of insulin I need. I have found that a 15 to 1 scale works well for me. That’s for every 15 grams of carbohydrates/sugars I’ll take 1 unit of insulin. Between counting carbohydrates, sugars, and calories to maintain weight my days are continually filled with math.
Once a year I travel to St. Michael’s hospital to the CF clinic for my annual assessment. St Michael’s Hospital is home to the largest adult CF program in North America. In addition to providing multidisciplinary care to 400 adults with CF, they are an active site for clinical research and training.
Cystic fibrosis is caused by a genetic mutation that breaks an ion channel in tissue lining cells. The channel is necessary to properly make sweat, mucus, and digestive juices. Results of the mutation include thick mucus in the respiratory tract that can create a haven for deadly pathogens, as well as gastrointestinal issues and pancreas damage. The type of mutation, type of pathogens that settle in the lungs, pancreatic status, quality of care, and diet can all affect health outcomes.
The majority of the tests performed in my CF clinic are the same as the transplant clinic at TGH such as PFT’s (pulmonary function test) and X-rays. Blood work is similar. Some of the different tests would be a Vitamin K level. They also follow my blood sugars more closely and have more experience with CFRD.
Vitamin K is a fat-soluble vitamin and its absorption from the gut is dependent on bile salt and pancreatic lipase secretion stimulated by dietary fat. People with CF are at risk of developing vitamin K deficiency due to fat malabsorption as a consequence of pancreatic insufficiency and bile salt deficiency. Other risk factors include CF-related liver disease, frequent antibiotic therapy, inadequate dietary intake and short gut syndrome resulting from bowel resection work. A sputum sample is usually required as patients with CF will harbor bad fungus in their lungs or sinuses as well.
I also see a dietitian as eating well and maintaining good nutritional status is very important in the treatment of CF. In Canada, the Cystic Fibrosis Evidence has shown that improving and maintaining good nourishment in CF results in better functioning lungs as well as overall outcome and well-being. This has benefited Canadian CF patients from the US with an estimated increase of 10-year survival bringing the current average age to 50.9 now. This is a huge achievement as the life expectancy when I was born was 15 years old. The aim of nutritional management of CF is to support normal growth and development throughout life.
People with CF have increased nutrition and energy requirements. Often in CF normal digestion of food is affected. This can be due to a reduction in the number of digestive enzymes released by the pancreas. These enzymes are vital for the digestion of normal food. If these enzymes are not present food cannot be broken down and absorbed by the body, therefore maintaining your weight and nutritional wellbeing could be a struggle. This condition is often referred to as ‘pancreatic insufficiency’.
During my last visit in December 2016, I had asked the Dietitian to help me with my weight gain. After looking at all my recent tests, height, current weight, blood sugars and daily activity I was given a goal of 3500-4000 calories a day. If I get an infection or become ill I would need to increase this goal to at least 4500 calories due to my body burning more energy.
Normal calorie intake for most people is anywhere from 2000-2500 a day, therefore I was quite surprised with my goal due to my size. For those who don’t know me, I am not very big. Soaking wet I would weigh 71 pounds and if I was stretched out about 140 cm.
I took this goal into consideration and started to do some research on high fat/calorie foods that were healthy as well as meal planning. I wanted to go into more detail hoping to discover any opportunity that could add extra weight. In addition, I wanted to improve my exercise program so I could strengthen not only my lungs but my entire body.
My plan was to double my meals every day as well try and eat snacks in between. Unfortunately, this first plan failed for two reasons. You see I was out most of my days so trying to eat high fat/calorie healthy foods was hard to find, not to mention the amount of money I was spending eating out. Secondly, by mid afternoon I was too full and felt so uncomfortable even breathing was more laboring at times. It almost felt like I committed my day to just eating, it became a full-time job.
I took a step back and considered what needed to be fixed. Before long I came up with a few ideas. Number one was I couldn’t start right away with a goal of 4000 calories. I needed to stretch out my stomach and get comfortable with consuming all of this food. So I aimed for a daily goal of 2800-3000 calorie intake and then increased by 500 calories every two weeks.
I also figured that starting my day with a large breakfast followed by a smoothie gave me 1/3rd of my calories. That way if I got caught up in my day, consuming the rest of my calorie goal wasn’t as hard. I also learned to snack more often throughout the day eating more mixed peanuts and cheeses and adding in the extra Ensure Plus. The challenge was to do all this while maintaining my blood sugar. After all, if I didn’t maintain my blood sugar there was no point in trying to put on any weight. So not only was I counting calories I was following my carbohydrates and sugars closely to stick with my sliding scale I had talked about earlier.
For those that are looking for a good tool for tracking your calories as well a fitness guide, download the app “My Fitness Pal” it’s supported by Under Armour. It’s a great app that will help you reach your goals as well give you fitness advice! It also will take your workout into consideration, as well how many steps you put into your day and readjust your calorie goal for the end of the day; a perfect way to stay on top of your goal.
The sole purpose of my weight gain and blood sugar management was due to my Transplant team. This was one of their recommendations last year that in order to be a candidate for a 3rd transplant I would need to gain weight and muscle and have a better control over my blood sugars. I’ll admit in the past I didn’t always pay full attention to my blood sugars.
I have started to see some small improvements over the past four months with my weight gain. I can thank some good friends and one of my brothers for encouraging me to stay on course. Included are some interesting late night meal outings, a few visits to Mc Donald’s by maxing out their custom burger menu by adding more than six paddies and all the extra toppings. Some local diners have even created some new milkshakes specially designed to help me get those last few hundred calories in. Personally, I would skip the deep fried mars bar, bacon, banana and peanut butter combo. Thanks to my team of supporters I am heading in the right direction, now the most important part is maintaining and finding a balance weight to stay at once I get there.
I am mostly followed by my Transplant team at Toronto General Hospital (TGH). TGH is home to one of the largest lung transplant programs in the world. It’s dedicated to helping patients through every stage of a successful transplant, offering specialized services to patients with even the most complex conditions. TGH has been the world’s first on many levels, including single and double lung transplantation for the long term. In 2016 the team at TGH preformed 145 lung transplants that’s a 13% increase from 2015. This year the team at TGH will be celebrating their 34th year of successful lung transplants in Toronto! This is a great success for the hospital and team of the program. The fact that they have been able to extend terminally ill patient’s lives for the last 34 years is beyond amazing and makes me proud to say that I am part of this program.
Even though my own lungs are no longer part of me, I still carry the CF disease. It’s found in my pancreas, sinuses and can still harbor harmful fungal bacteria in my lungs or sinuses that can affect CF patients. Aspergillus, Pseudomonas and Scopulariopsis Brevicaulis are all CF related bacteria. In my first transplanted lungs (2002) I had a lot of issues with Aspergillus causing serious lung infections and scarring leading to critical issues with my breathing. The sole reason for my second lung transplant was rejection. In fact, rejection is the main cause of death after lung transplantation. Doctors/scientist have discovered ways to slow rejection down but to this day they have not found how to stop it.
Unfortunately, survival of lung transplantation is lower than all other solid organ transplants. The five-year survival rate is only 60%, and the 10-15 year survival rate is as low as 20%. These rates are based off the first transplant and it is believed that a second transplant survival now is quite similar. However as for a 3rd lung transplant, there isn’t enough data out there to compare, it’s basically uncharted territory.
The major cause for lung rejection stems from small airways of the transplanted lung, or graft, beginning to scar. Slowly they become completely scarred and closed up. This process is called Bronchiolitis Obliterans Syndrome (BOS), the patient will begin to have shortness of breath again like they did before the transplant.
I can vividly remember both times when I started to notice the shortness of breath with my transplanted lungs. With my first transplanted lungs, I used to play paintball once a week. I remember running across the field with paintballs flying in my direction. I was trying to dodge them like the scene from Matrix. I didn’t feel as if I was as successful as Neo from the movie. Suddenly I was struggling for air and feeling so exhausted that once I got to the other side of the course I collapsed. The feeling was so odd because mentally I felt like running was possible, but in reality, my body wasn’t allowing me to. At the time I thought it could be a small infection coming, unfortunately, I was wrong it was far more serious.
After my second lung transplant, the dawning occurred while I was walking with my dog, Sheba. We were out on our usual walk which included a few small hills. I remember having to stop a few times to get my breath. It was the same feeling that I had experienced on the paintball field, thinking about it now I felt totally disabled realizing that the unavoidable was happening once again.
After each experience with shortness of breath, I was treated for low-grade acute rejection. I would be treated with high-dose steroid pulses typical prednisone as well ATG is frequently used. Rapamycin may also be introduced as a fourth agent. Other possible therapies are total Lymphoid Irradiation and Photopheresis, which are really last resorts. These drugs are very powerful on the body and mind. Your emotions are scattered all over the place. It is definitely an emotional rollercoaster ride. You experience happiness as if it’s the best day of your life, you feel as powerful as the hulk. Suddenly you transition to sadness, depressed the anxiety builds creating a hopeless sense of panic. And then you crash making you feel as if you’re in the darkest hole you’ve ever been, feeling lonely with no one to help, you wallow in total despair.
Not only is it a box of crayons of emotions, but mood swings to the point where the smallest simplest things get under your nerves; someone just looking at you can make you snap. It takes every ounce of control at this point, not to attack anyone. Mentally it’s frustrating and can make you feel even weaker. I haven’t shared these experiences with anyone. I have never wanted anyone to think I am mentally weak or weak at all. My barrier is my armor, a thick emotional shell keeping me distant and strong. I don’t want to burden others with my problems. After all, it was “me” that chose to enter these battlegrounds. Not letting anyone think I am weak has been one of my fears. Yes, I have said to live fearlessly however darkness can sneak into our life without us knowing, it creeps up until it has built a comfortable place in our imagination waiting for the right moment to attack.
Rejection is one of the scariest words you can tell someone who has received a transplant, it means with all the anti-rejection medications you’re taking your body is still identifying the transplanted organ as a foreign object. Your body is doing the totally opposite thing you want to be done, it’s building an army to attack what you have worked so hard for, bringing you back to where you started. If only you could tell your body that this new piece of equipment is keeping you alive. This is a chance for a normal life – a second kick at the can. Everything you’ve mentally and physically prepared yourself for begins to crash down.
One of the criteria for a transplant is to have a good support team of friends and family. Preparing and surviving a transplant isn’t a walk in the park, it’s not like you just get put on a list and wait for a phone call announcing the right organ has arrived. There’s a lot of pre-work as well post work to be done. A transplant is an on-going experience and being able to talk to someone or just even having someone around at times is vitally important.
I wrote a post quite a while ago about fear and how you can’t let it control your life, fear can damage the mind once it takes its grip. Fear is not a stranger we all have shared it at some time in our lives. There maybe a few individuals that say they’re fearless however have they really tasted fear? Fear is silent it will sneak into our minds making us question ourselves. A little fear helps to keep us on track. Contemplating fear keep you focused when making those important decisions in our lives.
March, 15th 2017, I had my 15th lung assessment. I have talked about what happens on this day previously however for those that aren’t familiar with my past, or transplantation I’ll go briefly into a typical lung assessment appointment. The purpose of my lung assessment is to follow up with progress (or decline) of my transplant. Unfortunately, I say decline at this time because the possible reality of being re-listed for a 3rd transplant means that a decline has occurred with my health. This is due to the rejection. The small airways that I previously talked about have been scarred causing them to close up making less room for oxygen to be made and more difficulty breathing.
Before I see the doctors I have various tests done. They include blood samples to measure drug levels, organ function levels such as liver and kidney as the drugs I take to suppress my immunes can be very hard on these organs.
I then go for an X-ray, the x-ray is to show if there is any possible fluid or infection growing in my lungs. X-rays will also indicate any visible cause for decreased lung function such as a collapsed lung even rejection. The team also likes to compare if there has been any change from the year before.
My chest X-Rays from my 15th-year lung assessment
My PFT’s are used more at this point to monitor the rejection damage in my lungs. In November 2016 my lung volume was quite high, since then I experienced a few incidents of Pseudomonas. These occurrences have caused more damage to my lungs making my PFTs fall more than 10%. In fact, they are at the lowest they’ve been in the longest time .62. This makes getting around a little more difficult than normal as well I get tired quickly.
PFT Results from the last 6 months
I’ve been trying not to let this slow me down or interfere with my days. For the past few weeks, I have been put on a heavy course of antibiotics to treat the Pseudomonas flair up. I can only hope with exercise and recovery that I can bring this number back up. Some days are better than others, which simply mean there are days that take a little more effort than others to get things done. However, I still haven’t let the Pseudomonas stop me. I can feel the shortness of breath creeping in while I am working out or walking. In the past I would try to ignore this occurrence; I’ve decided just to take things a little slower.
I even called my transplant coordinator before they contacted me letting her know how I was feeling. Typically I would wait until things got worse initiating a visit to emergency or an unscheduled clinic appointment. This time they reassured me through a sputum sample that my shortness of breath was caused by Pseudonymous. If the flare-up continues much longer I will probably be admitted to the hospital for an intravenous course of antibiotics as the team do not want to take any risks with my lungs.
With my second transplanted lungs, this time around I have exchanged the issues that I had with Aspergillus in my first transplanted lung with Pseudomonas in my second transplant causing a more difficulty and obstacle to jump through.
After my PFT’s I had my 6-minute walk. It is a test where you walk for 6 minutes and the values of your sats, pulse rate, blood pressure, and shortness of breath as well tiredness of legs and distance are all measured and calculated. Due to the decline in my PFTs the team wanted to see if I would benefit from the use of oxygen during this test. With 4 LPM of oxygen administered my lungs handled the test very well. The results indicated the active Pseudomonas has taken a toll on my lung function. I was still able to keep par with the predicted values of someone my stature. By stature I mean, size, gender, weight, age, they need to do this as if they compared my results to someone that is much taller than me or weighing more it’s obviously going to be different results. I walked a distance of just over 511 m in 6 minutes working out to being 96% of the predicted limit. At rest, my oxygen rate was 98% a pulse rate of 95 bpm during the test my oxygen rate dropped as low as 91% and my pulse rate increased to a 138 bpm. After my test during the recovery portion, I recovered very quickly almost to the point of where I started at rest. At recovery, my oxygen rate was 97% and a heart rate of 103 bpm.
Later on that day I met up with two doctors from the lung team to discuss my results and what kind of action plan they would like to implement. The team is concerned with the dramatic dip in my PFT’s. I don’t have much room for loss in this department. The last 6 months my PFT results didn’t increase but at least remained consistent and stable. They are okay with my results as long as they don’t drop lower.
A 10% drop in your PFT’s tends to set off alarms and gets the team worried. The team is only hoping that this drop is due to the Pseudomonas that has flared up and that I can recover any lost ground after the antibiotics kick in. I too can only hope for this, whenever I get results like this I feel defeated and useless. Remember how I told you never do I want CF to control my life, I would rather control my CF. When I get results like this I feel as if CF is controlling my life, yes I’ve said I no longer have CF in my lungs but if it wasn’t for CF I would not be in this boat nor would I be affected by Pseudomonas.
Next topic was my blood results, most of my results were at normal limits only two were slightly off. One of my anti-rejection medications Prograf was low sitting at a level of 6 units (the team would like my Prograf level to be sitting around 11-15 units). It’s been a constant adjustment with my Prograf level, either being too low or too high. The team increased my morning dosage to 1.5 mg and kept my night dose to 1 mg. My magnesium level was low this would probably be due to the increase of exercise as well my body not absorbing my supplements. I need to be taking them at a different time of the day away from my other medication.
The good news – the team was happy with my weight gain, as well with my blood sugars meaning my hard work of stuffing my face, counting carbohydrates, calories and sugars are starting to pay off. I’ve increased my weight by just over 6 pounds in four months. To most people that might not seem like a lot but for someone with CF that’s a huge gain literally. I still have a good amount of ground to go but I’ve started to head in the right direction. With more weight I’ll be able to make my body stronger and healthier, setting myself up for a positive outcome in the unknown chapters I am about to face.
The last thing on the board of discussions is the topic of a 3rd transplant. At this current time, the team feels that while I am not ready for a 3rd transplant I am heading in that direction. I live in an altered dimension, I am too sick to work, but I am too healthy for a transplant. I am not complaining what so ever, it’s just a long waiting game. If anything it gives the team and science more time to come up with better ways to work with organ transplantation and rejection. The team does feel since it’s been over two years now since I was assessed for a 3rd transplant when it comes to that time they would like me to repeat some tests. They need to make sure my body is strong enough to recover from a third double lung transplant. They have to be realistic; if my body isn’t strong enough to survive the transplant the lungs need to be given to someone who is. I totally understand where they’re coming from, as I would rather see the same outcome. The positive thing is the team is very familiar with my case and what’s going on. I am well known to the team and they follow me very closely. The only thing I can do at this point is continuing building myself stronger and updating the team with any changes.
End of the day, I’ve taken all the bad and weighed it with the good and with the good you have to take the bad. Yin and Yang, find a fine balance between both and you will find the course you’re set for. Signing up for a transplant in 2002 I was told you can exchange one set of problems for another, that a transplant is no cure for you. However we are allowing you to have borrowed time, it’s been up to me what I do with this borrowed time. I can only hope that I continue to put this borrowed time to full use. It’s like taking a vacation in a timeshare without banking my time. Fifteen years ago I was knocking on death’s door, I was at end stage CF there was no hope for me other than a Lung transplant. I was given the odds and potential possibility’s of what could happen however there is no crystal ball out there determining what course I am sailing on.
However, you know what?
“I’ve come as far away from that Lion’s den that I can. As rough as these waters might get with storm clouds full of lightning bringing crackles of thunder through the air, I am not jumping ship. I prefer not to return to that den for awhile, I’ll do whatever I need to do to stay out of that cold, damp, dark, wet den. As rough as the waters may get and as dark as the sky may become, there is the sun on the other side of those clouds that will bring me warmth and calm waters where I can dream”.
Don’t look back unless you can smile, don’t look ahead if you can’t dream.
In my dreams, I am smiling.
DangerDan 
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