“Hi, I’m Harley! I am 25 years old and I have Cystic Fibrosis (CF).
First, the facts. What is it? CF is a genetic disease which prominently affects the lungs and the digestive system. The gene affected by CF controls the movement of salt and water in and out of cells, which in turn inhibits the body’s natural ability to clear mucus. Those who live with CF experience a build-up of thick, sticky mucus in the lungs, digestive system, and other organs. The mucus in our lungs is the perfect breeding ground for bacteria, and being unable to clear this mucus blocks our airways, which leads to chest infections causing long term damage. This damage leaves our lungs with scarring and inflammation, causing our lung function to drop.
The build-up of mucus also blocks our pancreas, meaning that the enzymes released to digest food do not occur in someone with CF. As we are unable to digest the food we eat, it is extremely hard to maintain a healthy weight. We are advised to reach an ‘ideal’ intake of 5,000 calories per day and maintain a high fat intake. Despite a high fat, high-calorie diet, this does not lead to weight gain. Our digestive systems are under a lot of strain. We have many continuous issues with this and I will not go into detail here as I am sure you can use your imagination. The lungs and digestive system are the two organs that are most affected; however, CF does also affect bone density levels, joints, the liver, fertility, sinuses, mental health, and insulin management.
How is it treated? Treatment of CF is just as complex. Everyone with CF is different, no two people are affected in exactly the same way. Treatment plans, although consisting of similar aspects, are equally unique to the person. Nearly everyone with CF will be on a form of the following treatments: nebulizers, physiotherapy, inhalers, antibiotics, Creon (this is standard because it replaces some of the enzymes to help our body digest our food), vitamins, and a whole host of tablets. There is currently no cure for CF.
How is CF diagnosed? CF babies born today will be diagnosed through their heel prick at 4 months old. However, when I was diagnosed back in 1999, it was not that simple. I was diagnosed through a sweat test. Individuals with CF sweat more than your average person and our sweat contains a lot of salt because of the fault in our gene. The sweat test involves collecting an individual’s perspiration over a period of time up to 24 hours. The amount of salt in the sweat collection is tested and recorded.
Is there a life expectancy? Yes, children born today with CF are given an average life expectancy of 47 years old. But there are new advancements in medicine from the last year, and we should see this number extend massively. Fingers crossed!
I was born in 1995. I was my mom and dad’s first child, a happy, hungry baby. At the time, being my parent’s first child, they were unsure of what was ‘normal’ when it came to babies. Now, when we talk about the first few years of my life, they describe how they now realize there were more signs.
When I was born, I was malnourished; I had a huge round belly and tiny sticky arms and legs, I was constantly hungry and attached to a bottle most of the time. When I was a toddler, I would eat the same size dinners as my parents and still be hungry, yet I was always small. My parents went back and forth to the doctors regularly with concerns about my chest or stomach. I seemed to get ill very frequently, but the doctors kept telling my parents that I would grow out of it. They would prescribe some treatment and send them on their way. It was my health visitor who really listened to my parent’s concerns and knew I had CF based on what they were saying and what she was seeing. She helped my parents’ fight to get me tested.
This led me to my diagnosis at the age of 4, which is considered late. I was also given a life expectancy. At the age of 4, my parents were told I would be lucky to make it to 31. I cannot even imagine how my parents felt hearing that news. My life had barely just begun and they were hit with the age of 31. In the natural order of things, parents go first, then children. Parents become grandparents and children become parents. All of this left my mom and dad on this day. Their life completely turned upside down. They had a 4-year-old diagnosed with a genetic condition that would cut her life short and make everything a challenge until that time came. They also had a 1-year-old. A young couple with a young family and a huge fight on their hands. A fight for life.
In between my birth and diagnosis, my parents had my sister, still the best gift they’ve ever given me to this day. I count my blessings every day that she came before my diagnosis, as following it, my parents decided they would not have any more children. My sister does not have CF and does not even carry the CF gene, which is amazing.
Following my diagnosis, I started treatment straight away with nebulizers, physio, and tablets. Getting a 4-year-old to comply with all this strange and uninviting equipment was far from fun for my parents. I was not the most cooperating child when it came to my treatments. For the next 10 plus years, they had a daily battle when it came to convincing me to get my treatments done.
Growing up with CF was not easy. I was bullied for most of my school life and because of this I didn’t talk about my health; it was on a need-to-know basis. I was ashamed.
Being different, and having people point out why I was different, made me ashamed. The bullying was almost reconfirming my views on my health. I was very negative about it when I was younger and it’s taken years to move into a more positive mindset. So the bullying was like pushing and shoving, but mostly verbal. The typical kid stuff. When CF was taught at school, the sources were old and the age expectancy shared was 16, so I had comments like, ‘You’re going to die at 16, I wish you would die now, no one likes you.’ This was something that truly stuck; even to this day, I can hear the girl saying this word for word. The cyber part was a hate page created online, and participants had to have a login for it. The hate page contained LOADS of comments about me. A school friend told me about it. I never actually saw what was written, but the school saw it and got the police involved.
My mom came into the school to do assemblies on CF in an attempt to help my peers understand and lessen the bullying. I spent most of my break times and lunch periods in the reception office at my primary school, eating my snacks and lunch with two lovely ladies. It wasn’t all dull, we had fun, they taught me all kinds of things, including how to knit, which I have now lost my knack for. Unfortunately, secondary school started the way my primary school had finished. Although there came a point a couple of years in when I had a row with a ‘friend’ and she used my CF against me. That is when I put my foot down, I was no longer letting people get away with saying cruel things about my health.
Growing up, I do not remember much about my treatment or hospital stays. The things that stick with me most are the experiences leaving me with medical trauma. The burning feeling of my intravenous antibiotics (IVs) as they ran through my veins. I would squeeze my wrist so it didn’t hurt until my nurse and mom pried my fingers off. Pure agony. My dreaded physio bed, the bane of my childhood… I would kick and scream to not have to get on that thing. The first time I took a medication called Movicol, it’s a powder you mix into water… I can still taste it. When I had an IV line placed into my foot, I could not walk properly because it was so incredibly sore. My mum removed it two days later because I was in so much pain. Another time, I was pinned down by my nurses to have an IV line placed in my arm. My veins are tiny and very wiggly, professional terminology, of course, making it extremely difficult to place anything other than a cannula and on this occasion, they were trying to place a long line. I had a nurse holding my arm down, another on my legs, and another on my head, while the doctor tried to get this line in that simply would not, I cried and screamed so much. My mom asked them to stop and they did not listen. After a few minutes, my mom demanded they stop and sent them all out of the room. I curled up into a ball in her arms and sobbed. I was 8 years old. This experience still haunts me every time I have to have a line put in. I shake uncontrollably, cry, and have to mentally prepare for the procedure. My adult specialist team manages this well and I can only have one type of line placed in order for them to treat me, a PICC line.
In my 20s, my health and treatment plan changed a lot over the years. Now I have CF, chronic sinusitis, chronic fatigue, joint issues, small airway disease, colonized pseudomonas (since age 9), moderate lung scarring, and CF-related liver disease. I am now on three nebulized medications a day, doing eight rounds of my nebulizer each day. My physio is now done with a hand-held device so I am able to do this alone. I am on two different types of inhaler, a nasal spray, 2 types of antibiotic daily, liver tablets, laxatives, painkillers, and five other tablet form medications. On an average day, I spend up to four hours completing treatments and take anything between 20-40 tablets a day. This time does not include sterilizing and preparation. Over the last 5 years, my health has deteriorated much quicker than I had expected. On average, I have around seven exacerbations a year due to chest infections, and three or four of them will be treated with a 2-week course of IV antibiotics. My IV routine consists of three admissions a day and takes a grand total of 4.5 hours, again not including sterilizing and preparation, meaning my treatment routine during these periods hits over 8 hours a day.
As you get older and hit your 20s, you expect to become more independent. In some aspects of my life I have, however, in others more than ever I have become more reliant on my parents. My joints love to cause a problem, when I have a flare-up it is completely unannounced and can happen just like that. When a flare-up hits it immobilizes me for up to 4 days. I cannot even get myself out of bed. Mentally it is challenging, it is draining, and man is it tiring. Despite all this, I try and remain positive and I have achieved a lot in my lifetime that I never imagined would be possible. I have a First-Class degree in Criminology and Psychology. I also struggled with the worst year I had ever had health-wise while studying. I have had many volunteering and professional roles including volunteering for The Samaritans and working as a Restorative Justice Worker in Youth Offending. I am now 5 months away from qualifying as a Children and Families Social Worker.
For years, I hated my health and my life expectancy. CF is not an easy thing to live with by any stretch of the imagination and it is not a choice for so many. I didn’t have a choice and neither did my family. CF didn’t allow us the privilege of choice, it chose me. However, everyone who voluntarily comes into my life has that choice. Sometimes CF is too much for people to accept. Over the years, I have lost friends and partners because of my health and that just makes it so much harder to live with. I never openly discuss my health; I still struggle to verbally discuss it now. I lived my life in a rush, a rush to get things done, my life expectancy has a permanent space in the back of my mind. I was so focused on wanting to be a mom, I would put pressure on myself and my relationships to succeed because I wanted a child so badly. I wanted to be able to spend time with the child before I died. I have arrangements with my sister and my parents, arrangements for my future children, a solid plan for when I leave them behind. Imagine being 25 and already having these arrangements in place for years. My sister, 3 years younger than me, accepts her role to take on my future children when I am no longer around. These are not conversations anyone plans to have at these ages; however, they are the reality of CF. Birthdays always scare me, they are bittersweet, another year older means another year CF did not win, but it also means another year closer to my life expectancy.
My life has not followed my ridged timeline and has not gone the way I had ever planned it would; however, it has taken me in the direction I needed to go. I never saw myself with a career or a degree, and now I am working towards my Master’s degree and a career in Social Care. I have fallen in love with my life over the last few years. I have learned my boundaries, I have discovered new coping mechanisms to help me process my health, the main one being writing, telling my story. I have built a community around myself by making connections through social media with others who have CF, even though we cannot meet in person due to cross-infection. Never underestimate the power in talking to someone else in your shoes. I have best friends who I FaceTime and talk to every day from all over the world. My support systems, my mom and dad, sister, family, friends, and CF friends help me through this minefield every day.
The best part of my story happened this year, 2020. It has not been the year any of us had ever imagined, and it has been the year that has tested my mental health around my CF more than any year before. It has also been the year that saved my life. Kaftrio/Trikafta, the most advanced CF medication that has been developed to date, became available on the National Health Service the day after I turned 25. Never did I think I would see such advancements in CF medication in my lifetime. On October 24th, 2020, I started this medication. There were happy tears all around, I gained a sense of relief and my mum and dad got the answer they had been desperately waiting for my whole life. It is not a cure, and my daily treatment routine will not change; however, it promises years, fewer infections, less damage to my body, less pain. For the first time in 25 years, I am no longer scared of getting older. No longer scared of turning 31. Reluctantly, I relieved myself of my ‘CF timeline’ earlier on in the year. Kaftrio has enabled me to relax about this now.
My biggest fear is having a family and then passing before I get to watch my children grow and my 31st birthday. But my hopes for the future are to maintain my current health so I am well enough to have a family and hopefully carry my own children. Before starting Kaftrio, I was always split about transplant and how this conversation would look. Fortunately, I haven’t had to have this conversation with my specialist team yet. Now, I’m hopeful the advances in medication will mean that I will not have to address transplant.
My advice for others would be to not let this hold you back. Find your coping mechanisms and outlets. We are always taught how to be careful and avoid infection, but with treatments and life expectancies, it can become all-consuming. Find your passion and joys in life outside of your health. We are so much more than our illness. Talk or write. Communicate with your friends or family, even if it is one person you share everything with. I have found writing really helps me process my feelings about my health and where I am at.
I now have more of the gift of time. Time to have a career, time to have a family, time to enjoy the small things, make memories live a new life unlimited. At the age of 4, my parents were told I would be lucky to make it to 31. Challenge accepted!”
This story was submitted to Love What Matters by Harlie Cottle. You can follow her journey on Instagram. Submit your own story here and sure to subscribe to our free email newsletter for our best stories, and YouTube for our best videos.
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