Patient Stories

I was diagnosed with a congenital heart defect at birth. My pediatric cardiologist, Dr. Andrew Warren, had told my family that my heart condition was rare and therefore, is classified as a large ventricular septal defect and a double inlet ventricle. Basically, I was born without the septum between my ventricles – a VERY large hole, that was not able to be repaired. I had my first heart surgery at 6 months old. I had five heart surgeries by the time I was 3-years-old (1999), including one open heart surgery, which was the Fontan procedure. The doctors told my parents that this procedure could save my life, but that I might have trouble in school and not be able to do physical activities, similar to most CHD children. As well, I was on a gastronomy feeding tube, from around 6-months-old until I was 4-years-old. This was because the occupation of eating made me lose weight as it was too much work for me and my heart. I needed to be tube fed in order to get the nutrients my body required. However, as a toddler I was very active. My parents say I was constantly dancing to Barney and anytime music was played. As soon as my feeding tube was removed, they signed me up for dance class at a local dance studio. My cardiology team was hesitant, but my parents did not want to hold me back and knew the signs when it might be too much for me and to stop. Furthermore, I joined school that year as well; however, much smaller than everyone else in my class.

From there, I continued to take baby aspirin every day and visits to my cardiologist went from every 6 months to yearly. As a child, I also participated in gymnastics, figure skating, soccer, and softball. However, at the age of 10 I became a competitive dancer. I still had symptoms but knew my limits, knew when I needed a break and knew what to do to get myself back on the dance floor. With dance, I was able to travel all over the world. I competed and trained in Toronto, Montreal, Boston, Florida, and Paris, France. I won many top awards; and on top of this, I was above average in academics at school, taking advanced level classes. In 2014, I started a Neuroscience degree at Dalhousie University, while I continued to dance competitively on the university’s dance team and teach dance at the studio where I grew up. In 2018, I graduated from Dalhousie on the Dean’s list and started a Masters degree in Occupational Therapy at Queen’s University in Kingston, Ontario.

With all of this, I say that my Fontan procedure has been a huge success. It saved my life, and so much more. Limited research had my cardiology team hesitant to engage in the vigorous physical activity of competitive dance and take on a full-time class-load at school. However, my parents wanted to see how much I could do, knowing what to do if it was all too much. I continue to have symptoms to this day, with some days better than others. However, I am able to better manage myself and live my life the way I want. I decided to become an Occupational Therapist due to my lived experience. Many children born with congenital conditions that will impact their function and quality of life become hesitant to participate and engage in occupations and activities that other children do. I thank my parents every day for allowing me to participate in many activities, ensuring that I was occupationally balanced – which I now know as vital for an any individual’s health and wellbeing. With my experience, I want to encourage parents, families, children and adult clients to find ways to engage in occupations an activities that are meaningful to them, in any way they can! Furthermore, I believe all children with a congenital heart defect or other congenital condition, should have an Occupational Therapist on their team. An Occupational Therapist understand their limits and restrictions but also knows the value and evidence of being engaged in occupations that are meaningful and fulfilling; and great at being creative in how they engage with their limitations.

Although the procedure was fairly new in 1999 with limited evidence of long-term effects, I am living proof that it was very successful. The team had warned my parents of the life I may have and that a heart transplant may be required when I was 16-years-old. The procedure did not completely fix my heart defect as I still have an arrhythmia and fenestration (leakage) where the intraventricular valve was closed off. However, I sometimes forget that I have heart condition, because of how successful the Fontan procedure was, and how it allowed my heart to function in a way that supports my active lifestyle!

Hello,

My name is Matthew.

I am 35 years old. I live in Hamilton, Ont and I was born with CHD. I was born with the famous Tricuspid Atresia. But if we all want to go into detail. Tricuspid Atresia with transposition of the great arteries.

My mother has told me that when I was born, I had all the looks of a healthy baby. 3 days after being born my parents took me home and everything seemed fine. It was when I was five weeks old that my mother noticed that I was crying more and was giving her a hard time while she tried to feed me. My parents took me to the fanmily doctor and after a very quick visit he immediately ordered my parents to take me to the emergency unit at the Toronto’s Hospital for Sick Children. This would be the day when my parents would find out that I was born with a congenital heart defect. After hours of testing a few cardiologists informed my parents just how serious my defect was. They were told the name of my disease, how I was not born with a normal, healthy heart. They were told about my future. They were told how my disease would affect the other organs in my body. Also learning that I would also need to have a surgery named The Fontan surgery within the next few year. My parents were even more devastated when they were told  that I would need to have my first surgery that night so I can live those few years before the Fontan surgery. When they were told that I had 50/50 chance at surviving my first surgery they were terrified but quickly signed the approval papers so that I would be in surgery that same night. My first surgery was a banding of the pulmonary artery. The first surgery was a success but of course, I would not be able to go home for a few more weeks. After fourteen days my parents were told that I needed my second surgery because the band around the pulmonary artery was too tight and needed to be loosened. From there I stayed at the Sick Childrens Hospital for seven more months. 

My parents were finally allowed to take him home in June of 1985. The pulmonary banding surgery works and I had healthy life for the next few years. Learning how to walking, how to speak, how to slowly eat on my own and yes, learning how to use the bathroom on my own. As we all know, times flies and those three and a half years flew by. Finally, when I turned four years old my cardiologist informed my parents that it was time for me to have the Fontan surgery. The surgery they learned about only a few years earlier. This surgery would also be done at Toronto Hospital for Sick Children.

 In 1988 I had Fontan surgery. Once again in detail the surgery was a Right Atrial – Pulmonary Artery Fontan with Damus-Kaye-Stansel Anastomosis. I spent the next three weeks in the hospital recovering until my parents were allowed to take me home once again. That same year my mother gave birth to my first brother. His name is Eric and he was born on September 22^nd 1988. I lived a normal life normal, well, as normal of a life that a TA patient can live. I went to school, had lots of friends but when it came to gym classes I was limited. The teachers always kept a close eye on me and I was too young to understand why. There was a positive to all that non sense in grade school. 

Twice a year I would have clinic at Toronto’s hospital for sick children. I would have clinic or Mondays or Fridays so of course as a child, I looked at this as a three day weekend. Those clinic days always included a stress test on the stationary bike, an echocardiogram, an ECG, some blood and a visit with my cardiologist at the end of the day. Some visits I would have to take home a holter monitor. For those of you born in the 1980’s or older you must remember those huge cassette tape hotler monitors. Those were so much fun! Of course, as the years went on the hotler monitor every year changed as they became smaller.

 In the summer time I would always go and play street hockey or soccer with my friends. With no teachers around and my parents at home. I would really take advantage of that moment. Up until the age of eighteen I would never really ask too many questions about my disease. My mother would try to explain to me sometimes when I would come home after playing soccer with my friends but I would never really listen to her. I was a kid turning into a teenager. Going to high school making more, turning sixteen and learning how to drive and of course turning 18, graduating and going to my high school prom. 

I have never tried smoking and never have taken any drugs. I did have the common sense to know that I did have a heart issue. Not being able to join the weight lifting gym class and those scars down the center of my chest that reminded me every morning that I wasn’t as healthy as my friends. But just dealing with clinic appointments at that time I thought to myself that I was fine! I still had a long life to live. That all changed when I was transferred from the Sick Childrens Hospital to the Toronto General Hospital. 

That is when doctors became more detailed with me. I would ask questions and was always surprised with the answers I really had no idea the seriousness of my illness. My cardiologist at Toronto General Hospital made sure that I understood everything from that moment on. Here is a moment where I really started to pay attention.

 I was twenty-one years old and I had just been hired as an apprentice mechanic for transit buses. Two months later I went for my now, yearly, clinic appointment and told my cardiologist about my future career. He got onto his computer and typed out a letter and handed to me when it was printed. The letter stated to my employer that due to my health I will not be to continue with my apprenticeship. As upset as I was, I fully understood why. Working as a mechanic on a city bus is VERY different. The main reason was the weight. Taking off the wheel on a car and taking off the wheel of a city bus is about a 50lbs difference. I was limited to 15lbs. Which, I did not know at the time. I went to work the next day and handed the letter to my shop manager. Too my surprise he offered me a job in the parts department. There wasn’t anything in the parts department that was over 15lbs. When new tires for the buses came into the shop one of the mechanics would take care of it and put the tires away for me. I was very happy and continued to work in the parts department for a few years. Until I decided to get my commercial BZ license and become a transit operator.

Everything was perfect. I had a career, working for the government, owned a car for the winter and owned another car for the Spring, Summer and Fall. I was also starting to save up for a house. I had done this all on my own and I was only 25 years old. But, in 2011, my life would change once again. I had just turned twenty-six a few months earlier but on February 4^th I woke up very confused and in extreme pain with what I thought was a migraine. I was taken to Toronto General and after a CT scan I was told that I had a brain abscess and that I needed emergency surgery. 

I was kept at the hospital and in one day I had the surgery. I do not remember much because I was in so much pain that I had blacked out at one time in my hospital room in front of my cousin and my mother. I was given some medications by IV so I can sleep and not feel the pain anymore. The next morning, I woke up in the ICU confused. I was afraid I did not remember the last 24 hours. God bless my parents, there they were again, 26 years after my first surgery signing approval papers for an emergency surgery. Standing over me holding my hand telling me that I was going to be ok and not to be afraid.

 The surgeons told me that the room was ready and it was time for the surgery. I have had three surgeries before this but I do not remember anything about the pre and post surgery experience or how it feels to be put to sleep for a surgery. This was going to be the first time that I will actually remember. That scared me even more and while I was looking up at the ceiling of the surgery room and trying to calm myself down with breathing exercises the nurses were placing the electrodes on me, wrapping the blood pressure monitor on the left arm, the o2 sensor on my right finger and a mask that went over my nose and mouth. Then I realized the there was a team of nurses and surgeons. Two surgeons and eight nurses and not to mention all these machines in the surgery room. I was too nervous to even look at them and try to figure out exactly what they were. After a few minutes one of the nurses said to me that it was time to begin and that I will need to counting backward starting at ten. My eyes started to close at eight and I was completely asleep by six. This surgery took four hours and when I woke up in the ICU again. I was fine. No issues at all. As if nothing had happened in the past 48 hours.

 I stayed at the month for about a month strictly for precaution, I had a MRI once a week and had blood work done on that same day. Finally, I was allowed to go home but my cardiologist and my new neurologist told to not to return to work for six months. Nor was I allowed to drive my own vehicle for also six months. As upset at that as I was, I gave my car keys and my license to my father knowing that he would not give them back to me until the six months was up. At the six month mark I had an MRI scheduled and my neurologist called me and said it would be fine if I wanted to drive myself to the hospital. Of course, I said yes and my father gave me back my keys and license.

 A week went by until one Tuesday evening I was driving around the city when I started to feel sick and started experiencing double vision. I drove myself into a Walmart parking lot and called my father and asked if he could please pick me up, I did not feel good. My father was on his way when I started to feel even worst. I closed my eyes thinking it would help. It did not, that is when I had my first seizure. My father found me in my car and called 911.Thenext time I woke I was in the ICU of the Hamilton General Hospital. Right away the doctor told me that I had a seizure and because of the brain abscess I now had epilepsy. My license was suspended a month later and I have not driven car since 2012. Just like that, I could no longer work or drive. I had to sell one of my vehicles and everything that I had worked for the past five years was now gone. I took it very hard. A month later I was told I could work but I would have to choose a different career. Again, I took that very hard as well. 

 I thought to myself that I will stay home for the rest of the year and start job searching in 2012. In 2012, February 2012, to be exact. I developed an autoimmune disease called Warm Autoimmune Hemolytic Anemia. I had a surgery to remove my spleen in the hopes of curing the disease but that did not work. I was put on prednisone to bring up my immune system. Doctors said that there might have been a link with my heart disease but they were not certain considering the medication was now helping me recover and I was having blood work done once a week at Princess Margaret Hospital. June 2012 was also a wild month. My CBC numbers went from 153 to 88 in two weeks. I developed blood clots in my leg, lungs and one in my heart. My immune crashed, even while on prednisone, and it caused the multiple blood clots that I now had. My prednisone was increased from 15mg to 120mg in one day. I was put on IV Heparin for three weeks as the blood clots decreased in size. The fourth week of June I was shown how to use heparin needles at home. I would need to inject myself with heparin twice a day. 9am and 9pm this was also an interesting moment in my life. Going out with friends and having to inject heparin into myself in front of everyone. My friends had a good laugh at this. I was not laughing! My stomach was purple fun all the injections. I had to do this for six months until the prednisone dosage started to decrease so I can start taking warfarin.

As the years went by, I stayed home and I was on ODSP. Doctors did not want me to go to work with a compromised immune system, epilepsy and heart disease. On October 2015 I had a minor heart attack. I was aware of everything that was going on and was taken to the Hamilton General Hospital by ambulance. I was cardioverted that night and stayed at the hospital over night while the cardiologist at Hamilton General got in contact with my cardiologist in Toronto to work out a plan before I was sent home. 

The next few years I had a few angiograms and a couple ablations done at Toronto General Hospital. My clinic dates also changed from once a year to every six months. Of course, I was still not allowed to return back to work so I just tried my best to stay healthy and loose the weight that I had gained because of the prednisone. In June 2017 I reached 175lb. June was a horrible month. My father passed away June 26^th just a few weeks before his 62^nd birthday. I was very close to my father. It was very hard for me to deal with the fact that he was no longer here and I missed him dearly, and of course I still do. 

Eleven months later, May 2018, I was sitting at home when suddenly my left arm started to go numb. The first heart attack that I had in 2015 my left arm did not go numb. But I did feel very unwell. This heart attack, my left arm did start to go numb and I was feeling very sick. I knew had to get to Toronto General Hospital right away. Because I was a congenital patient, I knew only Toronto General Hospital would know how to help me. I took a baby aspirin as well as my warfarin and I called my cousin, who lived twenty minutes away. Ten minutes by highway. 

She and her friend came to pick me up and we went. I called my mother who was at work and told her to meet me at Toronto General and told her I felt very sick. I did not want to tell her that I was probably having a heart attack to worry her. When I got to Toronto General Hospital, I hurried into the emergency unit. I was checked in right away and was given an ECG. I felt ok I guess because of the aspirin and warfarin I had just taken at the same time but the ECG showed something else.

Minutes after the ECG my heart started to slow down. Nurses came in the room with a mobile echocardiogram and started checking on my heart. As this was happening my left arm was starting to go numb again as I was looking at the monitor watching my heart rate go down. The last number I remembering seeing was my heart beating at 26bpm. That was when I went into cardiac arrest.  

Cardiac arrest is an unreal event. You do not want to experience this. With no warning you can’t hear anything except your heart beat. That only last a few seconds before everything goes black. For me, I compare it to shutting off a vintage television. Where the screen would go black with a very thin white line across the screen which quickly turns into a small white dot. That is what happened to me. Everyone has different experiences but for me that is what cardiac arrest was for me. I was cardioverted twice while given CPR with chest compressions. Later on my cardiologist told me the whole event lasted a couple minutes. 

Coming out of the cardiac arrest is a totally different experience again. I woke up screaming while a room full of emergency room nurses and doctors running around trying to help me while I was trying to push everyone away. I only stopped when I saw my mother standing at the door. When I was stable the ER cardiologist and the on-call congenital cardiologist went to go and speak to my mother. They told her that I needed a pacemaker and it had to be done that night. Because of the Tricuspid Atresia and Fontan surgery my pacemaker would have to be placed in my abdomen. Once again, my mother, who was now doing this alone, signing approval papers for me to have the pacemaker put in. They pacemaker surgery went well and the pacemaker was working great. I stayed in the hospital for just over a week. This time staying in the ICU.

When I went home everything seemed fine. I was even following the heart healthy diet. Three weeks later I was right back in the hospital. I was as white as a ghost and having trouble walking. After days of testing, some test I have never had before, I was told that my thyroid had to be removed. My cardiologist, along with other doctors, told me that the steroids I was on for so many years covered up my thyroid issue. I was taken off the steroids back in November 2017. It was only a matter of time before my thyroid issue would cause the cardiac arrest. 

I stayed in the hospital from June 2018 to December 2018. I was very weak and was barely able to walk. At one time staying in my hospital bed for two weeks. It got worse and worse until I was put on a diaper! I had never felt so alone in my entire life. I could not do anything for myself and needed nurses to help me in the shower while I sat on the commode. There were days I tossed all my medication across the room. At one time I did not take my epilepsy pills for three days. With low hormones at the time and not taking my medication I experienced delirium for an entire week.

I do not remember a single thing that happened that entire week. Almost every cardiologist and neurologist in the hospital would check on me every day and adjusted medications until I finally came out of delirium. One day I met a nurse who was in training. I let him practice his first blood work on me, I didn’t mind at all. After that he sat next to me just talking and he told me that he was going to help me and I would be walking on my own in a month. I laughed at him and told him he was crazy. He would visit me every shift he had and I finally agreed to letting him help me. He gave me exercises to do every day and he got me a walker and peddler and signed them both out under my name so I can use the peddler while I sat in my chair reading a book or listening to music. Every time I wanted to use the walker I would ask a nurse to walk with me. I tried to walk three times a day. 

And! Just like he said! November 2018, exactly one month after meeting him. I was walking around the hospital on my own for a month until I was sent home. It is because of him I have been able to walk since 2018. 

Now, the year of the transplant! 2019 started off great. No more prednisone, new pacemaker, I was active and I felt great! When April came around I started to notice that my legs were becoming larger. Especially while I was sitting and my stomach kept growing to the point where at one time I looked like I was pregnant. At this time I use to drink a lot of coca cola which is something no heart patient should be drinking but I was stubborn. I would say to myself what is the big deal? I am eating healthy so why can I not drink pop? I decided not to drink pop for a week and see if there was any difference? I drank only water and sometimes a little bit of juice when taking my medications. My stomach went down a little bit but my leg were still large.

My next clinic appointment was in May. On that day my cardiologist said the reason I was retaining water was because there could be a chance, I was experiencing Heart Failure. My cardiologist gave me lasix to take home and said that this should help me and that it would slow down the heart failure. I was in good shape and other then the water retention I felt fine.

After a month the lasix was not working. I started to look even bigger and that was when I called my cardiologist for an appointment asap. The day of the appointment I had blood work done and had an echocardiogram with an ECG done. I had an hour before I would see my cardiologist so I went and had lunch. I asked my mother to come with me to clinic that day, so after lunch we both went to see my cardiologist. When my cardiologist walked into the room another congenital fellow did as well. They both sat down with serious looks on their faces and I started to get worried. I asked them to just please tell what was going on.

My cardiologist went on and told me that I was in Fontan Heart Failure. My fontan circulation was now failing and a fontan revision was not an option. I was absolutely devastated! I was lost for words and I did not want to look at my mother. I knew if I did that, we would both start to cry. I tried to keep everything inside and asked about a transplant. That talk took over an hour so much information that I actually stopped paying attention. I could not get the idea of a heart transplant out of my head. My cardiologist gave me a prescription for lasix and I went home. 

I was extremely quiet for the following week as I saw my stomach get larger and I now needed two pillows to sleep. I could not lay on my back anymore with having trouble breathing. I called my cardiologist and told them about what was going on. They asked me to come into the hospital and expect to be admitted. When I arrived at the hospital my cardiologist already got a room for me on the 5^th floor. There I was told that I will stay there for a few days while a tube would be inserted into my stomach and the tube would be connected to a drain bag. In three days, I had dropped 12lbs of fluid. I thought to myself that I did not want to do this every other week. I wanted the transplant no matter how scared I was.

On September 22^nd, the same day my first brother was born, I was admitted into the hospital for testing to see if I would qualify for a heart transplant. 

This was my situation

• Failing Fontan Circulation
• Anatomical issues: Severely dilated RA, Moderate-severe MR 
• Moderate AR through native pulmonary valve ( neoAI )
• Function issues: Moderately dilated LV with mildly impaired systolic function
• Renal impairment
• Fontan associated Liver disease
• Prior right atrial thrombus with pulmonary emboli
• Warm Autoimmune Hemolytic Anemia 
• Splenectomy

With all that on my plate I pushed forward. I was either not going to qualify for a heart transplant and live life with whatever time I had left. Or I was going to qualify for a heart transplant and live a new life.

September 23^rd I was put on Milrinone and lasix IV infusion and started my fight for a heart transplant. I had a CT, Full body xray, echocardiogram, many xrays and an ECG every other morning the first few weeks. There were weeks where I did not have any test planned. There were many ups and downs and at one time I even wanted to give up and just leave the hospital. Arguments with doctors, nurses, friends and even family. I was extremely stressed out the first few weeks. I just kept telling myself that I had to change my mind set and forget about the last few weeks. I did this by spending an hour on the stationary every morning and going for walks around the hospital a few times a day. Eating horrible hospital and drinking only water, even when taking medications.

I also had a few psychology tests and had seen a therapist to make sure that I was mentally stable and understood what a post transplant life would be like the first year. I also had to read a heart transplant book and understand the surgery itself, understand the medications and what they were used for and a lot of other things that I had no idea about. It was rough reading about how compromised my immune system will be. How I would have to use sun screen and a hat every time I left the house on a sunny day because of the risk of getting sick cancer. The very risk diet I will have to follow the rest of my life and how I will not be able to leave Ontario for the first year or until I was given approval from my cardiologist. 

Finally, it came, the answer I was waiting for! On the morning of November 19^th my cardiologist walked into my room, shock my hand and gave me a hug. I was approved for the heart transplant. Every nurse, doctor, cleaning staff member and kitchen staff member that I knew came to congratulate me that day. I actually celebrated by going down to the cafeteria and buying a cheeseburger. I actually felt sick after eating it so I knew for sure I was on the right path. I had no intention, no desire about going to eat foods that would only do harm before the transplant. Every morning I still rode the bike for an hour and every day I would go for walks around the hospital. The entire time thinking about life with a new heart. 

I did not have to wait long. The evening of December 7^th a transplant surgeon walked into my room with a big smile “Matthew! We have found a heart for you “I thought the surgeon was joking I began to laugh. But I was laughing out of joy and the first thing I said to the surgeon was “Where do I sign? “The surgeon then sat next to me and explained how the donor had Hepatitis C. I was confused, I thought hepatitis affected the liver? How can a congenital patient with TA accept a heart from a donor who once had hepatitis c? 

The surgeon explained to me that Hepatitis C had a cure and all I had to do was take one extra pill. Right away I asked for the approval paper and signed. I had to wait an hour before I could be transferred to the ICU. Right away I called my mother and told her what just happened. She and my brothers were on their way to the hospital as soon as I hung up the phone. In that hour I thought about a lot of things. 

The last 35 years of my life, thought about my brothers and my parents. Then I realized that for the just time out of all my surgeries I finally signed an approval paper on my own. Then, I thought about my father. How proud he would be of me for standing up for myself, passing all the transplant test, getting approved for a transplant and now, finally getting a heart transplant. 

I now know how my parents felt all those times they signed papers before my surgeries. The fear in the what if’s, would I wake up this time? 

I knew that I will wake up with a new heart. I will deal with the up’s and downs. I knew it wasn’t going to be easy, very far from it. I was ready

Surgeons, nurses and doctors from both The Toronto Hospital for Sick Children and the Toronto General Hospital all played a part in the transplant from the beginning to the end. My surgery took 13 hours. The total ischemic time was 4 hours and 22 minutes. 

I wish I could tell you about how it felt to be wheeled down the hall to the surgery room. How it felt to see even more surgeons, more nurses and more machines then another surgery that I have had. I can’t, words cannot describe how I felt at those moments. Only thing on my wind was that in one year, on December 7^th I will be celebrating my one year post transplant anniversary. When I woke up after the surgery, I had two questions for my mother. Am I alive and where is my pacemaker? I didn’t stay awake long enough to find out the answer. Of course, I was still very stoned! 

The first month post transplant I stayed in the hospital. I had a heart biopsy every week, along with other test. And yes, the pacemaker I only used for 16 months was now gone. I couldn’t be any happier.

I am one week shy of 8 months post transplant. I have experienced a grade 2 heart rejection back in February but that cleared up with IV steroids for three days and increased Myoforic. I hve not had any rejection since. As of July 31 2020 my prednisone is 5mg, my Prograf is 3mg twice daily and my Myoforic is 720mg twice daily. These 8 months have been up and down mentally. It is hard to come to terms with that someone lost their life so that you can have a second chance at yours. But that is what the donor wanted. All their organs to be donated it anything were to ever happen to them. I received this gentleman’s heart and I am truly forever grateful.

I am no longer a sick man. I am no longer an Adult with Congenital Heart Disease. 

I am a man who has a second chance in life. To live this new life with no limits. Where I can finally join a soccer league or join a ball hockey league. To finally learn how to swim and to finally travel to a different country. To longer be seen as a sick man in other people’s eyes.

But I will never forget where my life began. I will never forget the heart aches and disappointments. I will never forget the nurses, the doctors and the other adult congenital heart disease patients I have met over the years. I am part of a family, a special family. A family full of warriors and survivors. Babies who made it to their pre-teens. Lived their teenage years. Have been living their adult years with all type of CHD. To all Tricuspid Atresia patients and all other CHD patient Young and Old. I leave you with this

May the wind always be at your back, and the sun upon your face, and may the wings of destiny carry you aloft to dance with the stars

The journey of a thousand miles begins with one step

I feel that both quotes clearly represent the life that I have lived. And will continue to live.

God Bless, Stay Positive and Never accept the word No when it comes to you and your health

Matthew

Imagine being in elementary school and not having to go outside for recess in the winter and not having to participate in gym class…pretty cool right? Well, it was for me, but a lot of baggage camp along with that.

I was born as a normal baby. Well, that is what everyone thought. I was home no longer than 7 weeks before my life was turned upside down and inside out. I wouldn’t eat much at all and was a little blue. After a visit with the doctor, I was sent straight – not even going home to pack a bag – to Sick Kids Hospital for my first open heart surgery and remained there for a couple of months. The doctors found out that I had congenital heart disease, and if that label wasn’t bad enough they added “complex” to congenital heart disease. My diagnoses included DORV, complete AV septal defect, and hypoplastic right ventricle and Glen shunt among others.

I grew up as a normal kid playing with my friends, going out and being a pain to parents. But with all that freedom came many more heart surgeries which kept me going, until I was ready and the technology was ready for me to have the Fontan Procedure. Up until my Fontan, I was only taking Digoxin for medications.

It was at this point when things changed big time for me. I received the news about having my Fontan at my last cardiologist appointment at Sick Kids Hospital. So was I not only having the Fontan but I was moving to a new hospital across the street at Toronto General Hospital (“TGH”). I didn’t know anyone across the street since they didn’t offer transition nights for patients like they do now. The only good thing was that my surgeon Dr. Bill Williams was crossing over with me to do the surgery. Going into the Fontan I knew my chances were 50/50 but decided what the hell let’s go for it since it was really my only option if I wanted to have a life. The process was very hard on my family and my boyfriend (now husband) but they all supported me all the way. My Fontan was done on December 5, 1997. I remember getting wheeled into the operating room and being moved to the operating table which felt half my size and getting laughing gas which meant lights out for me. I was put on the heart and lung machine during the 12 hour surgery. The Fontan went very well and the doctors replaced my mitral valve with a mechanical one at the same time.

Recovery was where all the hard work took place. I couldn’t move an inch of my body with being in pain…coughing and sneezing was hell. It didn’t matter which side of my body I laid on I could never get comfortable. When you have open heart surgery the nurses start to get you breathing deeply so your lungs expand and don’t collapse. There is this little piece of breathing equipment to have you to blow/suck on. It looks so easy at first but truly it’s not. The goal was to blow or suck on this tube and get the ball to the top. It wasn’t something I was interested in so my dad made it fun and profitable for me. He told me that every time I got it to the top I would get quarter…so I kept at it and was at about $20 when he dad said ok that’s enough now only get a dime and then that was the end of that story. The doctors kept me in ICU almost my entire stay which was great since I got one on one attention. I was desperately trying to get home before Christmas so I did anything they told me to and I was out on December 23 just in time for turkey. At this point I was on blood thinners “Warfrin”…yes rat poison! When you get a mechanical valve you have to take a blood thinner to avoid any clots building around the valve. I had a choice between a tissue valve which I was told would last 5-10 years max and the mechanical valve 10-15 years so I went with the mechanical. To this day I still have the same valve… 22 years and still like new.

One of the major side effects to the Fontan is “arrhythmia”, which I was never told about but found out the hard way. The following May of ’98, I became really sick nothing like I had ever had before. We all thought it was the flu, I felt weak and tired, which was defiantly not normal for me. After a phone call with my cardiologist he told me to go straight to Toronto General Hospital (“TGH”) and directly to the Peter Munk Cardiac Centre skipping emergency. I was brought in by wheel chair and went into cardiac arrest (flat lined) right in the middle of the clinic due to my heart rate of 280 bpm. I stayed in TGH for a few days so the doctors could figure out what to do with me and my arrhythmia. After being there a couple of days, one night I was laying in the bed and at about 2 am I felt my heart slow down drastically and I pushed the nurses call button before I flat lined again. I remember the feeling as if it happened yesterday; the feeling was so strange; it just felt like I blacked out for a while. When I woke up, I had doctors all around me with the paddles to my chest. Unfortunately, the nurse beside me wore my breakfast, lunch and dinner from the day before. The doctor called my parents in the middle of the night and told them to get to the hospital ASAP, but wouldn’t tell them what happened. I’m not a parent so I can’t even imagine how they felt and what was going through their minds at that time. This hospital stay brought on a whole new way of life for me I didn’t want or expect. I was put on Ammiodorne for the arrhythmia, Ramapril for heart function, Lasix for water retention, and continued with the Warfrin. Also during my stay the doctors decided I needed a pacemaker for my slow heart rate which was 30-35 in resting position. I was and still am 100% dependent on my pacemaker. It continuously beats my heart at 80 bpm regardless of what I am doing.

Fast forward a year later and my pacemaker failed. I was standing in the house and felt my heart rate go from normal to super slow and could barely stand. I was rushed to TGH where I got another new pacemaker. This was a pattern for me for the next 3 pacemakers. Years later I am on my 8th pacemaker which gets replaced about every 5-6 years.

Today, my quality of life is great for someone with my complications, with no restrictions other than doing things within my own limits. I currently work full-time as a senior corporate/commercial law clerk and love it. My moto…live your life to the fullest because life is too short and you only get one chance so try anything and everything without regrets. I tell this story not to scare anyone preparing to have a Fontan but to give you strength and to assure you it’s all worth it in the end. Without my Fontan I don’t know where I would be or if I would even be here at all.

Eleven years ago I started, along with a few very special and helpful volunteers, the first congenital cardiac camp for adults. “The Beat Retreat” is an amazing experience for congenital patients who never had the chance to go to camp as a kid. We have a wide variety of activities like rock wall climbing, canoeing, low ropes, archery, games and campfires. The best part of the camp is sitting around the camp fire and sharing stories. We have created such a strong bond because we just “know how it is” and can relate to each other. Even though we all have different issues we all feel alike. The camp is a 4-day long weekend every September. For more information please visit us on facebook or at www.thebeatretreatcamp.ca.

In the end, the people I truly feel for are my husband, parents and my sister. They have gone through so much and have stuck by my side through it all…thing most people could never imagine.

I was born in 1985 with a complex congenital heart disease with univentricular physiology. At birth, because I was turning blue due to a lack of oxygen,  I was taken away from my mother and placed in the ICU. I eventually made it home approximately two weeks after birth. 

At three months old, I had a Right classic shunt and by the age of three, I was undergoing a massive surgery, known as the Atriopulmonary Fontan procedure. It was a successful procedure and I was released from the hospital about three weeks after the surgery. I grew up with a relatively normal childhood, with various procedures scattered throughout, such as at age seven, when I had Subaortic reception, VSD enlargement, and litigation of BTS, and at age 11, when I had Atrial flutter requiring cardioversion. I was then placed on Warfarin for approximately four years.

By the time I was 17, as most of my friends were thinking about dating, college and their future, I was thinking about what life would be like to live with a pacemaker because I was told I would eventually need one, and now was the most opportune time to do the surgery. A revision of my Fontan surgery was done and an epicardial permanent pacemaker was implanted by the amazing Dr. Tchervenkov. I will be forever grateful for the excellent care I received from all the medical professionals at the Montreal Children’s Hospital, including the nurses in the ICU which once secretly allowed me to have popcorn and Gingerale if I promised to take my medication that day. As a side note, I have always been adverse to taking any kind of medication and much prefer to use food and a healthy lifestyle as medicine. I truly believe in the “power of mind over body”, because after I recovered from the surgery, which took most of the summer, I was off all medication and only taking 325mg then 81mg aspirin for the following 20 years.

When all the scarring was healed and I was in a better headspace, I packed my bags and moved abroad. For the next seven years, I had the most wonderful life experience anyone could ask for, living in Europe, Australia and Morocco. During that time, I returned to Canada on a regular basis to make sure all was in order. On one of my visits, at the age of 23, I underwent an interval pacemaker generator change. A few years after I moved back to Canada, when I was 28, I had a pacemaker generator change and new atrial and ventricular epicardial leads. Fast forward 8 years and time for another battery replacement or pulse generator change in 2021.

My heart defect and related health problems have not prevented me from obtaining higher education, including two Masters in law and a juris doctorate. To this day, I have had many high level professional experiences in the field of law, such as working for the United Nations, working in the fast paced corporate environment and for government entities.

 At the age of 37, I developed what is called ascites. This is essentially fluid retention and is a sign of Fontan Circulation Failure, which can happen in Fontan patients. As a result I ended up being hospitalized for a week to get rid of the fluid build up and I now take lasix and an anticoagulant. Once again, I am in the excellent care of a team of professionals, this time at the Toronto General Hospital, given that I moved to Toronto in 2019. The news of Fontan Circulation Failure initially came as a shock to me, as I had been feeling fine and skiing in California the year before and was planning yet another ski trip for that spring. It also came with a whole lifestyle adjustment, such as a low sodium diet and fluid restriction but I am learning to process it all and adjust, one day at a time.

 I am fortunate to have the love and support of friends and family, but above all I am immensely grateful for my mother who has been there for me every step of the way and has been my rock no matter how difficult things seemed at times and made sure I was able to have a childhood and great life experiences, despite my heart defect.

 Since I was born, I have been stubborn and determined to live and do everything in my power to live a healthy and long life. I have been told that I am persistent and do not give up easily. More recently though, I learned the importance of practicing self care and accepting that it is ok to not be ok some days, both mentally and physically. I am also learning how to be more patient with myself, with others and with the process. 

 I share my story for this community of strong minded individuals and for those that feel anxious or scared, in the hopes of inspiring some and reassuring others. It is not easy everyday to live the way we do and while we may look healthy to the outside world, we know deep down that we are going through a lot. There is tremendous support if we know how to find it and I encourage each and everyone of you to seek out that support, as it will be beneficial.

 So remember to appreciate the little moments in life, because after all, these are the most important moments.

 Claire

“Health is the overall basis of happiness” – Kayla Barnes