1 Prepping for Transplant

A Hard Shift to Make

I grew up knowing that I had cystic fibrosis (cf). Having a chronic illness is hard to hide from children when they have to take pills before every meal and do aerosol masks. Besides the clinic appointments every three months, daily aerosol masks and chest percussions, and being required to eat chips every night for the extra calories, I had a fairly normal childhood.

As a December baby, I was the smallest in my class at school; New Brunswick, where we lived at the time, went by the calendar year for classroom placements. My parents debated keeping me in kindergarten another year to give me a chance to catch up, but by that time, I had a group of close friends with whom I wanted to stay for grade one. Having cf didn’t affect my friendships, and although everyone knew I had it, the only difference about my school experience compared to theirs was that I had to go to the office every recess for a nutritional drink called Ensure for extra calories. Dad always did presentations for the school at the start of the year to explain cf and fundraise through the Zellers cf Moonwalk (now called the Walk to Make Cystic Fibrosis History). My friends understood why I had to do aerosol masks during sleepovers and why I took so many pills with each meal. We didn’t talk about it, and it wasn’t a big factor in my life growing up.

Living with cf was part of our lives—my older sister, Amy, has it too—but it was not something that defined us. Our parents never let us use having cf as an excuse to get out of anything, and I think having each other reinforced the idea that we were not special because we had a disability. I use the word disability in relation to cf and while other people may not like the word for themselves, I feel it is accurate for me. While having cf may not have been causing me disablement when I was younger, it still limited my life in some ways. Although I did my best not to be defined by having cf, it would be naïve for me to say that it did not affect my life in any way.

I was hospitalized quite often as a toddler but Amy and I were both relatively healthy as children. We were only hospitalized once in our teens for two weeks for what we called a “tune-up.” It was a relatively easy hospitalization as well. We went to the iwk clinic, thinking only I would be hospitalized, but it turned out Amy was faring much worse than I was. My health was improved but they stuck me in the hospital anyway, “since Amy was there.” I guess the doctors figured it was all the same to my parents if they had one or two children in the hospital, not that I remain bitter to this day about Amy causing me to be hospitalized. It was actually fine: we spent two weeks getting iv antibiotics, playing Yahtzee in the hospital’s family room, and annoying the physiotherapist because we wouldn’t stay in our room so we often missed our physiotherapy time. Those things, and a cute volunteer named Josh who gave us a Calvin and Hobbes book when we left, are about all I remember from those two weeks.

two young girls playing in a toy car buggy and pretending to fill it with gasoline. — Allison (left) and her sister, Amy, at the iwk Health Centre play-park in 1991 during a hospitalization. Allison was admitted frequently as a young child due to her failure to gain weight. (Donna Watson)

As I’ve grown older, I’ve realized what a huge blessing it has been to have a sister with the same disease. I know that’s weird to say. I should want her to be healthy. And it’s not like I want her to have a fatal disease. It’s just that it has been helpful to have someone to talk to who’s had the same experiences as me. I could complain to her when I had stomach cramps and she wouldn’t panic and tell me to go to the hospital; instead, she would make comments like, “That sucks,” or, “Maybe you forgot to take your digestive enzymes.” I think that we pushed each other, even if we didn’t realize it at the time. I mean, if she could hike for an hour, so could I. There was no reason for me not to do anything, because there she was with the same disability as me, so who was I to complain. Of course there were times when it would have been better to stop and let our bodies rest, so in that regard we occasionally pushed ourselves too far—more so as we got older and our lungs started to decline.

It’s hard to know where the line is. Having cf means that you spend many days simply pushing yourself to get out of bed in the morning. It can be a struggle to do “simple” tasks like showering or cooking supper. And then when you want to go out with friends or go for a bike ride, it can be even more exhausting. Once I reached my twenties, if I had stopped doing activities because I felt tired or short of breath, I would have rarely left the house. And rarely leaving the house starts the downward spiral of feeling worse, which leads to never leaving the house, which leads to feeling worse...and so on. It’s a terrible cycle.

I’ve always known that having cf meant having a shortened life expectancy. But knowing the facts while healthy is different from having a doctor suddenly tell you that your options are to die in one to three years or take a chance with a lung transplant. Some people with cf have traumatic stories about the first time they realized they were born with a life-shortening illness. I don’t have that story. I feel like I always knew, though I must have learned it at some point, either at the cf clinic or from Amy. According to our parents, Amy found out when our cousin told her, “You’ll never graduate from high school because you’re going to die early.” However, Amy doesn’t remember that happening, so clearly she wasn’t traumatized either.

I realize it can be scary for children to learn they won’t live as long as their friends. But while we were growing up, that was not how we thought about it. Our parents were more focused on what we would do with the time we had. There was no point in thinking about a shortened lifespan when we could focus on the present. Why spend the time you have worrying about a future that may never come? I think it also helped that I was fairly healthy through high school, so I didn’t feel as though I was being “left behind.” I filled out university applications and dreamed of a career along with the rest of my friends. The only real difference was that I laughed when anyone mentioned pensions or retirement planning. Because I had spent so little time in the hospital, dying and being acutely sick were abstract concepts.

The first time I found it emotionally difficult to have a shortened life expectancy was on my twentieth birthday. It hit me then that I had reached the halfway mark of my predicted lifespan. It wasn’t the best time to be sad and pouty because my family was spending a week of the school Christmas break in the Bahamas. We were vacationing at the home of a missionary couple who were spending the week back in the United States; we couldn’t pass up the opportunity of having a place to stay in the Caribbean. Although we almost didn’t get into the country because Mom and Dad weren’t prepared for customs questions such as, “Who are you staying with?” and “What is the house address?” Their responses of, “With friends we’ve never met,” and, “The address is written on a piece of paper in the car they left in the parking lot for us, and the keys are in the dash,” were not the answers border security wanted to hear.

Somehow, they did let us into the country and we enjoyed our first-ever Christmas on the beach. It was a great vacation, and we were thrilled to have our first Christmas away from home. Amy, who was working as a nurse in neonatal intensive care at the time, couldn’t come with us because nurses have the worst work schedules; she called us, envious, while we were enjoying Christmas Day and she was getting pooped on by babies. Despite the pleasant Christmas, however, on my twentieth birthday a few days later, I was feeling gloomy because I had possibly reached “my halfway point.”

I knew that there was no guarantee I would live until I was forty or that I would die the day I turned forty, but on that birthday I mourned everything that I would miss. It is hard to describe what I was feeling at that moment. It was essentially that I was too old to have accomplished so little, and that I was not going to have enough time to fit in everything I wanted from life in the mere twenty years I had left. In retrospect, I realize how ridiculous I was behaving, but I also think it was important for me to process these thoughts at the time. I think realizing that I wouldn’t be able to save adventures and trips for after I retired meant that I was more adventurous in my twenties than I might otherwise have been.

Just as I grew up knowing that I had a fatal disease, I also knew that one day I might need a lung transplant. In the ’90s, a lung transplant was still considered a new medical procedure: the first one done on a person with cf was in 1988. It was a medical breakthrough for the cf community that was starting to be discussed as a possibility as an end-of-life treatment. Nonetheless, growing up, the idea was so abstract that it held no real meaning or fear. My family would often talk about it as something that both Amy and I would probably one day need. We would joke casually about “when I need new lungs,” or, “I hope this isn’t the infection that requires me to need new lungs.”

Even with my awareness that I might one day require and be eligible for a transplant, it didn’t make it any easier to accept when the day arrived. When the doctor first mentioned starting the workup process for a lung transplant, I burst into tears and refused to talk about it. I was in denial about how sick I was and didn’t want to accept that I had reached the point of needing a transplant. I had had a string of infections over a few years—several that required hospitalization with strong iv antibiotics—so I knew my lungs were not doing well, but I could not believe I was at the point of needing new ones. They only do that to people who have a life expectancy of less than three years. I couldn’t be at that point. Could I?

I felt as though I had failed myself by needing a transplant before Amy. As she was the older sister, I thought she should have to go first. I also struggled with the fact that even though she was the older sibling, she was doing much better than me health-wise. I conveniently forgot that she never seemed to get as many lung infections as me, bounced back from infections quicker, and was healthier overall. It took getting pneumonia twice in one year, and a long hospital stay, before I started to admit that I was going to need a transplant.

In fact, when I had pneumonia and was subsequently hospitalized in November 2012, that was the first time I had the physical feeling that I was dying. It made me panicked and slightly depressed. I had no energy, threw up all the time, and had a lot of back pain. It was terrifying to be that ill.

After that hospitalization, I somehow convinced the doctor to let me return to work, even though she was quite reluctant. I was not yet ready to concede that I needed to stop. It was a hard winter, and I struggled at work and at home. Working took up all of my energy, so I would spend my time off in a state of near- exhaustion, recovering. I was in a cycle that couldn’t last forever, but I wanted it to last as long as possible. I knew that as soon as I stopped, I would not be returning to work any time soon.

During that time, I was fortunate that I had access to my workplace’s mental health program. It gave employees access to counsellors either by phone, email, or in face-to-face meetings. I opted for the email route because I found I could express myself better through writing. I also found that it helped me work through my emotions when I had to think about what to write, put it down coherently, and reread the text to make sure it made sense to someone else. The counsellor was remarkably helpful as I worked through a lot of my anxieties around needing a lung transplant and dealing with death. She had me do exercises about my fears and gave me a lot of questions to ponder. When I was anxious that I was becoming depressed, she helped me realize that it was normal to feel sad and hopeless when encountering something in life of such great magnitude.

I made it through most of the winter feeling tired, coughing more than usual, being short of breath, but overall persevering. I tried to hide my exhaustion at the hospital where I worked as a recreational therapist. It became increasingly difficult as nurses would hear me coughing up phlegm in the bathroom, and patients would wonder about my shortness of breath. I tried to pass it off as a cold, but this also became harder as the months progressed. I just didn’t have the energy anymore to get through the workday cycle.

At the time, I was organizing a two-week trip to visit my brother, David, who was living in Spain. I love planning trips and travelling, so researching beaches on the Canary Islands was a great distraction. Then my cough increased to even scarier- than-normal levels, my appetite plummeted, and I started throwing up massive amounts of phlegm. My back hurt, I struggled to stand up while showering, and found it painful to breathe deeply. I had pneumonia again. It was all over.

I went to the local hospital for an x-ray. As soon as the results were available, the Halifax cf nurse called and told me to “get here now with an overnight bag.” When I arrived to see the team, the doctor immediately pulled me off work “for the foreseeable future,” and told me if I went to Spain, I would return in a body bag. She was not one to beat around the bush. Instead of going to Europe and having a fun vacation, I ended up in the hospital. Testing for a lung transplant began in full force.

There are only five transplant clinics in Canada—none in Atlantic Canada—so all patients from the Halifax clinic are assessed by the Toronto transplant team. If approved for a transplant, the patient must move to Toronto to wait, as the transplant team requires the person to be living within a two-and-a-half-hour drive of the hospital.

One of the common misconceptions about lung transplants is that every person with cf will eventually get the chance to have one. While hospitals are performing more and more lung transplants every year, it’s still not a viable option for everyone. There are many factors that make a person ineligible, and the teams are rather strict about whom they will allow. There is such a limited number of donor lungs that they need to make sure recipients have the best possible chance of succeeding with the lungs available. Also, a lung transplant is done as an end-stage procedure for people with cf, so a person has to be quite ill, but not so sick that they can’t recover from the surgery.

Generally, the criteria is that a person’s forced expiratory volume in one second (fev1) has to be less than 30% of the predicted output for someone of their age and weight. Once a person’s fev1 drops below 30%, statistically, they have a life expectancy of one to three years and are now considered eligible for the end-stage procedure. Having pneumonia dropped my fev1 to a new low of 22%, and while it did jump back up to 28% after the hospitalization, the increase was mostly due to the strong antibiotics pulsing through my veins.

The other criterion to be accepted into the transplant program is that the other organs have to be functioning as well as possible. People with cf tend to have weak hearts due to the fact that our hearts struggle to make up for lung deficiencies. While the lungs attempt to get air in and out, the rest of the body struggles to keep everything running. The kidneys and liver can also be weakened for people with cf as years of medication and antibiotics tend to stress any organ trying to keep toxins out of the body. Post-transplant medications are especially hard on the kidneys and liver, so doctors need to make sure that everything is as healthy as possible before dumping even more medication in.

During my hospitalization in February 2013, I completed a lot of the pre-transplant workup. I did a six-minute walk test, an x-ray, ct scan, and massive amounts of blood work. I was discharged back home after three weeks of antibiotics and waited to hear from the overlords in Toronto as to whether or not I would qualify to move on to the next stage of testing. It was an anxious few months. It seemed as though I was testing to qualify for a game I didn’t want to play. Will I be too healthy? Will I be too sick? Will my liver be damaged from that one night I drank too much? The Halifax doctors basically told me that I could be denied for any reason: being too healthy or too sick, having something else wrong with me, not checking my blood sugar levels every day, looking at them the wrong way…well, you get the idea.

As I had been pumped full of antibiotics, my lung function jumped up by 10%. This was great for my health and showed that I had responded well to the antibiotics, but then the doctor mentioned that my lung function may have improved too much for a transplant. Nonetheless, she told me that “we should probably still go ahead with the process.” I agreed, thinking, “Of course you should go ahead with the process. Two weeks ago you were panicking that catching a cold would be the death of me and now I’m suddenly good to go?” I was baffled. In the end, I was just on a medication high; once the medication was out of my system, my lung function returned to almost where it had been before I got pneumonia.

There is an effect that Amy and I like to call the “antibiotic high,” which is the feeling we get after being hospitalized. We feel 100% better after being pumped full of antibiotics for two or three weeks, as the medication suppresses any cough, phlegm production, and shortness of breath. Our lung functions jump up for a while, and we feel ready to take on anything. However, after about a week off the medication, when the antibiotics wear off, we go back to feeling as we did before we went into the hospital—only, hopefully, a few percentage points better. Thankfully, at the time, I managed to hang on to the antibiotic high and didn’t crash back down to 22% until over a year after that hospitalization. Although I was still ill during that time, I was not declining rapidly and was perceived as stable enough to be able to wait in Toronto for lungs as well as to survive the actual operation. I was in the sweet spot of being sick but not too sick.

Waiting for the results of transplant testing is a mind game. Like I’ve said, you have to be sick enough to require the transplant but healthy enough to survive the surgery and rehabilitation. You also have to not have anything else wrong with you and be mentally prepared for the trauma that comes with the surgery and recovery. Providing you aren’t rejected, you move to Toronto to wait for the surgery and are started on an intense physiotherapy program. Sometimes, because of all the exercise during physiotherapy, people show an improvement in their lung function, causing them to become “too healthy” for a transplant. Then they have to wait until their lung function drops—but of course, it can’t drop too low because then they wouldn’t have the ability to survive the surgery. People continue to play the “transplant game,” trying to stay in the elusive zone between being too healthy and being terminally ill, all while trying to be perfect patients so one day they might win, maybe, a new set of lungs.

I understand why the majority of people listed for transplant are on anti-anxiety medication. The process causes a lot of stress. While I waited, I started having trouble sleeping—not that I had been sleeping well before the testing, due to all my coughing and needing to be propped up on three pillows to make breathing easier. Now, in addition to that, the “what if” game was keeping me up. Instead of taking sleep medication that tended to give me nightmares or left me feeling groggy in the morning, I started listening to podcasts to lull me to sleep.

One immediate outcome from all the testing I had to undergo was that my six-minute walk test showed I needed to be using oxygen while exercising. Even with all my obvious health problems, I was not mentally ready for that step. To me, requiring oxygen meant that this was the end of my life, and, less dramatically, the end of being able to do anything I enjoyed. Once I started using oxygen to help me breathe, there was no going back, and it was a visible reminder that I was dying.

During my recreation therapy internship at a nursing home for my recreation therapy degree, I had been surprised to find myself uncomfortable around the people who required oxygen. I kept trying to shake off the discomfort and found that it was the people who required oxygen and were bedridden who distressed me more than those who used oxygen but were still mobile and active. The few people who were bedridden on oxygen were usually frail, and they were a stark reminder that one day that would probably be me, only at a much younger age. I felt as though I was being shown my future, and it terrified me.

I remained in denial for a long time about requiring oxygen, even though the team told me I needed it only “upon exertion,” which is often the first step for a person in lung decline. The idea is that since the body is limited by being unable to breathe, if that person wears oxygen, the body will not struggle as much and can therefore exercise harder and longer. The more we exercise, the stronger we become and the easier it is to breathe. It does not necessarily help improve lung function, but it does help strengthen the muscles around the lungs, making the body healthier overall.

Another problem with being prescribed oxygen to wear “upon exertion” was, at that time, most activities were exhausting for me. I was self-conscious wearing it in public, so while I would strap it on at home to lift weights or stretch, I did not like using it around other people. As I had been strongly encouraged to start exercising regularly, I signed up for the local gym but was too nervous carrying in my oxygen. Consequently, I wasn’t able to do much. I would walk slowly on the treadmill, which, while better than nothing, was not as helpful as it could have been. Afterward I would use oxygen to help recover, but it was not quite the same. I became less self-conscious as time went on, and once winter was over, I put the tanks in my bike panniers while cycling or I would throw one in a backpack the few times Isaiah, my partner, and I went on short hikes. I still refused to wear oxygen in public as I felt it would be an obvious sign that I was ill, and I hated that idea.

During the months I waited to hear from the team in Toronto, I did feel okay physically; I was no longer acutely sick. The regular exercise seemed to help and my lungs were holding steady with a fev1 of 26%. However, I thought a lot about death and dying and what it would be like to die. I wasn’t as panicked as I had been back in November, but I was still scared overall. I admit that I spent a lot of time crying in the bathroom about what would happen if I didn’t get the transplant, what would happen if I did get the transplant, or what would happen if I went for the transplant and it didn’t happen. It wasn’t a continuous feeling of anxiety but rather something that would hit me from time to time in quiet moments. To work through these feelings, I would cry for a short spell and then carry on with life.

As a way to deal with my emotions, I turned to my support system and had a lot of fascinating conversations with my friends about life, death, dying, and post-death. Some people were uncomfortable talking about death with me but most seemed to know that I just needed someone to listen while I worked through my fears. I know some people feel as though it’s morbid to talk about death, but this was my coping strategy. If I was going to die, I was going to die well-informed and have the best possible death. I figured we, as a society, spend so much time planning and thinking about what happens when someone comes into the world, we should spend at least some time figuring out what to do when a person leaves. The more I read blogs about dying and what happens to an actual corpse, the more comfortable I became. It was much more cathartic for me to learn as much as I could about the situation than to deny its possibility. And I could no longer deny the fact that I was not getting better.

In April 2013 I learned that I had passed the initial round of testing and was due to be in Toronto during the second week of July for round two. At that point, I still didn’t know if I even wanted to have the transplant, but I knew that I had to at least do the testing to see if I would be accepted.

So after my Prince Edward Island vacation, Amy convinced me—it did not take much—to take a road trip to Newfoundland in late June 2013 as an escape from my thoughts and from my apartment. It was a much needed vacation. We drove all over Newfoundland, seeing whales and puffins, chasing icebergs, and camping in the rain. We drove to St. John’s, ducked down to visit St. Pierre and Miquelon, went back across the island, up to St. Anthony, and eventually back down to Gros Morne National Park. The struggles of travelling with a disability were much more evident during our trip to Newfoundland than they had been during any previous vacations.

As cf is a hidden disease, other people often have a hard time recognizing our level of illness. To them, Amy and I look perfectly healthy. The downside to having a hidden disability is that people make assumptions about your abilities. Normally, that would be something I embrace, as people don’t automatically assume limitations before getting to know me. However, it worked against Amy and me when we got to the island of St. Pierre. When we arrived from the ferry, we immediately went to the tourist information centre to get a map along with some information on the island. We had planned on taking a taxi to our b&b because it was outside the main touristy area, and atop a giant hill. Since the ferry was “pedestrian only,” we had assumed there would be cabs waiting when we got off to take people to their hotels. We should have known better than to assume anything while travelling to a new place, but we had not yet learned our lesson.

We asked the worker at the information centre where we could get a cab. He proceeded to tell us that “Young healthy girls like you can walk there, no problem!” He outlined on the map the route to take, ignoring our insistence that we would indeed like to take a cab. Our French was not strong enough to argue with the young Frenchman, so, annoyed, we went to the bank to get some euros and returned to the tourist centre, hoping he would be gone. Thankfully, he was. The friendly woman working at the desk called us a cab, only after asking several times if we were sure we didn’t want to walk.

Amy wanted to tell them that I was actually carrying a tank of oxygen in my backpack, so, Yes, we’re sure we don’t want to walk. However, arguing in a second language is challenging, so we just kept insisting that we would like a cab. Eventually one arrived. During the first minute of the ride, I wondered if maybe we could have walked after all. The second minute, I was happy we were in a car as the hill suddenly became crazy steep. It was a short distance but not worth struggling up. Later, we walked down the hill to tour the town and had no trouble getting a cab after our supper of crepes by the wharf. Apparently, when it’s dark and raining, people are not as insistent that you walk.

For our last few days in Newfoundland, we camped in Gros Morne National Park, did the fjord tour, drove around looking for caribou, and tried to do a little hike. That hike turned out to be a horrible decision. It was humid that day, and I was labouring to breathe. The hike was flat along the bottom of a ridge, so I didn’t think it would be difficult, but I struggled the entire time. It was one of the first times in my life where I wasn’t able to push through an inability to breathe and keep going.

Of course, I hadn’t been able to do certain activities in the past. But mostly I would avoid ventures that I knew I wouldn’t be able to do, and for the ones that ended up being borderline, I would take breaks and keep on going. For example, when we were on vacation in Greece, on one of the islands we hiked to see “Zeus’s cave.” I struggled up the hill to that site—I needed constant breaks, threw up a bit, and took many puffers—but I was able to make it to the cave. It was one of the borderline activities that I thought I could do, probably shouldn’t have, but kept going anyway.

In Newfoundland that day with my sister, I just couldn’t do it. I couldn’t walk anymore in the heat and humidity. We had to turn back, and even then, I had to stop a lot and stick my feet in the streams to cool off. That aborted hike marked a low point in my life. I had to make a significant mental adjustment, finally realizing that what I thought I could do and what I could actually do were two very different things. I was a long time admitting to myself that I was seriously ill. Even though I had always had a chronic disease, it was a hard shift to make.

Copyright

Dedication

Disclaimer

Preface

Part I Pre-Transplant

My Life until October 2013

1 Prepping for Transplant

A Hard Shift to Make