CF (cystic fibrosis) is a genetic disease that primarily affects the lungs and gastrointestinal tract as well as the liver and reproductive system. If two carriers of the gene have a child then the child has a 25% (1 in 4) chance of being born with cystic fibrosis.
I have been asked many times about my illness, but when asked on the spot I can never really say enough about it to sum it up in detail. Even the people I hold closest and dearest to me don’t know the seriousness of its impact on me, and they’ll never know but I hope this helps the person reading to at least understand a little bit about it, and be less uncomfortable around the topic of chronic illness. If you know someone with cystic fibrosis maybe it will help you to have an idea of what they are going through, the fears they are facing that you know nothing about and the reasons they cancel plans last minute. If you are feeling low, maybe my story will help you to see that there is something to be happy and thankful for, even in the worst situations.
Hell is a scary place to be on your own, feeling lifeless, in a constant fight with your body. This is the norm for someone with CF. Every day is a struggle for us just to breathe. Imagine how it might feel if you had to bring a spit cup to bed with you at night and you couldn’t sleep with less than 5 pillows because if you did you’d probably drown in your own mucus. Now imagine how terrifying it would be to not know if you’ll survive long enough through the night to be woken by the light of the next day, to hear the sound of your lungs crackle like a bowl of Rice Krispies and to spew dangerous amounts of blood from your mouth and shrug it off like it’s no big deal. Exercise is not to lose weight but rather to maintain your lungs at the condition they’re in and hopefully even improve them, 2 to 3 hours of your day is spent voluntarily coughing up mucus so that you can breathe with a little more ease and you’re so exhausted afterwards that you collapse on the couch and spend another two hours sleeping when you’re done, so you forget to take the nebs you’re supposed to take and now your day is half over and you’re back to square one, coughing your lungs up again, and someone makes you laugh but you try your best to restrain it because you’re afraid they might end up on the floor. Imagine if you couldn’t tie your shoe laces without the aid of an O2 tank hanging by your side and knowing that little tank is your only real dependable in this world.
All you can eat without taking a fist of tablets is a fruit salad and a bag of your favourite Haribo cola bottles except you can’t have those either because the complexity of this disease means that you became diabetic when you were 15 due to your pancreas’ lack of function. So many weeks, sometimes months at a time are spent in hospital that it becomes a second home to you and you become so institutionalized that going home takes time to adjust to. When you eat and forget your enzymes, you suffer every consequence for ever having sinned in your life X10. You forcibly say goodbye to your friends without ever getting a chance to say the word. Imagine doing all you can and never seeing any result for the hard work you put in, being told to eat as much you possibly can of whatever unhealthy, fatty foods you like and never gaining a pound. That part sounds great, but it’s not. Most of the time you don’t have the appetite to enjoy it and when someone is constantly nagging at you to eat, it does nothing but put you off your food so it becomes a chore. You didn’t eat your house and home out of it today so you have to be fed through a tube tonight, and every other night except the one you insist on having to yourself this weekend so you can feel normal.
I was a healthy child but as I grew up I started getting sicker and having frequent hospital admissions. My teen years were a struggle and in a way I feel they were robbed from me. I’m not complaining about these things, after all this is the only life I have ever known and I happy that I am still well enough to be here typing this. In fact, it’s amazing. The reality is that cystic fibrosis is a degenerative disease that worsens with time and age. Life expectancy in Ireland for someone with cf is hard to predict, but people are now living well into their 30s and beyond. In the North it is it well into the 40s where facilities are better, and in Canada it is now 51. Facilities in hospitals are hugely important to prevent cross infection and improve survival rates. But enough about statistics. I am not a statistic and there’s no point sailing through life thinking of the ifs, buts and maybes so I guess all we can do is take all that with a pinch of salt and get on with it. But nevertheless, the numbers are still there and all that it means is that there is an extreme shortage of organ donors in Ireland and people are dying because of it.
I have heard all the insensitive comments: “Give up the fags!” , “Ah it could be worse, you could have cancer” (which I did, thank you!), “Oh.. doesn’t that mean you’ll die soon?” they used to upset me but now it’s water off a duck’s back. And yeah, of course sometimes they still get to me if I’m already having a rough day but then I realize in many of these cases, I’m mistaking people’s lack of knowledge for ignorance. They don’t know me or anything about me. How could they possibly know that the reason I sound like an 80 year old smoker isn’t because I smoke?
Sure I have lost many friends, more than I can count on on both hands.. some were a surprise and happened unexpectedly, others knew their end was coming and accepted it with grace. I’ll never forget my friend Keely. She was the healthiest example of a girl with CF I ever knew. We wrote back and forth to each other on Facebook as we could never meet face to face. She was younger than me and asked my advice from time to time. she attended a different hospital to me but I remember her telling me that she couldn’t breathe when she stood up and her doctor didn’t believe her. If I’m honest, she was young and I thought she may be slightly over exaggerated too. Her O2 was in the normal range, and she was hardly ever in hospital. To look at her you’d say she was the picture of health. About 2 months later Keely was transferred to St. Vincent’s where I attended. She had one to one nursing and was on 24 hour oxygen. Immediately after assessment she was placed on the list for double lung transplant, but it didn’t come on time and she died. Keely is just one of many friends I have lost to CF. It never gets any easier and I have just heard news today that another young person has lost their battle to this cruel disease.
Looking back, I don’t know why I got a shock because that is the reality of this illness. It shows no mercy to anyone hit by it and when it strikes you, it hits hard and you never see it coming. When it takes you, it doesn’t just take you, it takes your family, your friends, the nurses who looked after you, the catering staff that fed you, the man who gave you his heart and the acquaintances you made that you never thought were important. It’s prisoners normally young and zestful. I am not afraid of death. I have already stared it in the face and won, but that was not fate. That was luck, and many of friends were not so lucky. Tomorrow is not promised to anyone. Just because today is good does not mean that tomorrow will follow suit.
I never worried about what the future held for me til I was facing the fact that I might not live to see it. In December of 2011 I was put on the list for a liver transplant and remained no.1 on the list for 8 months in both Ireland and England. I was never a religious person but found myself praying more than the current pope at the time and making promises to God that I knew were unrealistic and I would never be able to keep. Naturally, I became depressed and started thinking of ways to end my life. I felt so sick I didn’t think I could endure any more pain, physically or mentally, and had some other personal stuff going on at the time. The only thing that stopped me from overdosing was a conversation I had with my sister two weeks before, and the fact that there had been two suicides in my town that week already. My family had been through enough and I didn’t want to be looking down on them drying their tears. But desperation changes a person, it makes you do and say things you wouldn’t normally think of. I decided I had to take control and change my attitude if I was going to stand a chance at survival.
During the wait I had two major scares that set me back and made me inactive on the list. After that, I fought hard to stay well enough to have the operation but after the second scare, I honestly thought it was game over for me and had accepted it. Two weeks later I was brought into theater and my life changed from then on. It’s amazing what the brain has the capability to overcome. But that’s life, whether you are living with a chronic illness or battling depression, maybe you have had issues with family, alcohol, drugs, whatever it may be. We all have something to be afraid of and if we didn’t know suffering, we’d never know joy because the thing about pain is it demands to be felt.
I thought that these things only happened to other people and never me. I knew the likelihood of needing a transplant some day, I just didn’t ever want to believe it. And why did I get mine and Keely didn’t get hers? It’s not fair, but that’s life and life is not fair. Someday I will probably need a lung transplant (taking into note that I say probably because a part of me is in denial) but that’s a bridge I’ll cross when I come to it. Even now, I think I’m the only person in Ireland not freaking about Ebola… because somehow my mind still refuses to believe that anything like that could happen to me. The real fear is not the illness itself, the real fear is the fears that circle around it and cause you to question your ability to cope with the physical and emotional every day struggles that it entails. The thoughts that you may never have kids, if you do you won’t live to see them grow up, you will never watch them get married and you will never tell stories to your grandchildren. These are the fears I and every other person with CF have, but I am working on them because I don’t want them to halt my happiness or derail me from my dreams.
I will admit, with an illness like this it is hard to allow new people to get to know you. It is difficult to let them in because that means letting down your guard of protection and opening up. I’ve hardened up so much that I’ve destroyed relationships or not allow them to develop because of my biggest fear: getting close to people. I’m afraid not only for me, but for them. Afraid that they will get to know me, care for me, maybe even love me and want to be there for me and want to be by my side when I’m sick. I don’t fear that they won’t be able to handle it because I know that if they love me enough, they will. They won’t mind me bringing a spit cup to bed with me, they’ll understand that I can’t go out Saturday night because I didn’t take enough enzymes and now I’m crippled over in cramps, they’ll make time to see me when I’m in hospital whether I like it or not and support me through it because they know that I’m scared. I don’t have the heart to put somebody through that, however then there’s the other side of that fear. I’m afraid that they won’t want to be with someone who who’s ‘sick’, they wont want to deal with the emotional burden that’s carried with it, and I can’t say I’d blame them which is why I do what I do nine times out of ten. I withdraw myself before they have a chance to tear down my walls. Sometimes it’s the right decision, but mostly it’s not. I have driven so many people away because of that fear, I just have to learn to get past it and find a way to live without it controlling who walks in and out of my life.
There are so many reasons to be negative when you are sick, but there are just as many positives if not more. You see the little things for what they are (the bigger things) and learn to appreciate the beauty in a moment. Part of me would swap it in the morning for a stress free life but the other part of me would fight tooth and nail for it because my life has taught me so many things. And it’s true, I should be more considerate. I should be less selfish and more thoughtful. I should do more for charity, I should try to make amends with the people I’ve fallen out with, I should say thank you more, apologize when I’m wrong, stand up for myself and try to be a better person every day, but I’m doing the best I can and I’m doing okay at it. I’m focused, I’m driven and I’m determined to make my life work out. I don’t care if I’m rich or poor as long as long as I have my health and good friends and family around me, I’m happy.
“I will breathe. I will think of solutions. I will not let my worry control me. I will not let my stress break me. I. will. simply. breathe. And it will be okay… Because I don’t quit.” [Shayne McClendon]