The battle inside my lungs: Living with cystic fibrosis and AMR
My Story: Ella Balasa
When you first meet someone like me, you may only notice a raspy voice, a thinner frame, and a person who takes a lot of pills. But then you might hear the laboured breathing, the coughing that sounds like an interminable chest cold, the green mucus that flies up our windpipe countless times a day, and the faint popping-corn sounds from deep within the chest.
These are some of the outward signs of cystic fibrosis (CF)—a disease often characterised, however, as an “invisible illness.”
I can describe these details in this way because I have been living with CF for 30 years. Since birth, I’ve gone through multiple surgeries, dozens of hospital stays, thousands of pills and hours of treatment every day.
Thanks in part to that daily routine, someone meeting me for the first time probably won’t see my condition. It’s both a curse and a blessing to be able to conceal something that affects my life so greatly.
What is cystic fibrosis (CF)?
Cystic fibrosis is one of the most common genetic diseases in the world, but it is still considered rare, as only about 70,000 people worldwide have won the genetic lottery by acquiring this recessive gene from both parents. The gene controls how much water and chloride flow in and out of cells in the lungs, nose, and stomach. The mutations that cause cystic fibrosis prevent chloride from leaving the cells, causing the organs to become dehydrated.
In the stomach, this makes it hard to digest anything, so patients need to take extra enzymes to break down food. In the sinuses, it can trigger a constant runny nose.
In the lungs, however, it is especially dangerous. Naturally occurring mucus in the lungs becomes thick and sticky without hydration, trapping particles and bacteria with each breath. Because the body can’t clear out this sticky mucus, the trapped bacteria start the cycle of infections for people with CF.
Germs that are commonly found in the environment—the simple bacteria we come into contact with as children, digging in the yard or making pretend forts from invaders—get trapped in the lungs of those of us with CF, and when they aren’t eliminated, they invade and colonise the lungs like an occupying foreign army.
The immune system is always fighting these invaders, but unfortunately, the lung microbiome provides the perfect conditions for their occupation.
Worse, the endless battle between the immune system and the invaders triggers inflammation. Over time, this cycle of infection and inflammation leads to scarring that reduces the function of the lungs, and eventually, respiratory failure.
The battle inside
Any bacteria or viruses that I contract from others through daily interactions can further burden the immune system, exacerbating my symptoms even more.
To suppress bacterial growth, I’ve had to use countless courses of antibiotic treatments throughout my life to kill some (but not all) of the bacteria—rarely can CF infections be completely eradicated.
Over time, after many rounds of antibiotics, the bacteria have evolved resistance. In recent years, drug-resistant bacteria have become one of the most widely known public health problems, but they are already a daily reality for those of us with CF.
A desperate experiment
Spending weeks in the hospital each year getting treated for these infections that have over time become more and more difficult to treat, using the same few antibiotics cycled many times – I am running out of antibiotic options. Oral and inhaled antibiotics are no longer effective in beating back the bacterial infections, and in the past few years I have been on them nearly constantly to subdue them.
In 2019, I experienced the worse infection I had ever had. And even intravenous antibiotics lost their potency. I was on intravenous antibiotics for weeks and I wasn’t having any relief of my symptoms. I was coughing non-stop and large green mucus plugs were constantly being expelled. I was doing breathing treatments every two hours or so just to quieten the wheezing and secretions in my airways as I breathed. My lung function dropped to 18% and I was put on supplementary oxygen. My feelings of fear and desperation shot through the ceiling.
A few months earlier, I had heard about an experimental treatment called “phage therapy” to combat an infection that was resistant to available antibiotics. An alternate way to fight “superbugs,” scientists have been showing renewed interest towards in recent years. Phages are viruses that feed on very specific types of bacteria and can be used to target a specific patient’s drug-resistant infection. In my haze I wondered, could this be a viable treatment for me?
Given the severity of my infection, my advanced stage of my CF, and the dearth of options for me at that point, I felt I had nothing to lose. A few weeks later, I found myself at Yale University, sitting on a gurney, inhaling phages developed just for me.
After a one-week course of treatment, I began to clear the infection. This was truly nothing short of a miracle. So many treatments had failed or delivered mediocre results. I had used the phage treatment along with a cocktail of antibiotics that I had used many times, so I knew the addition of phage therapy was the x-factor that made the difference.
There’s a good chance I wouldn’t be here today without phages, but solutions for vexing issues like antibiotic resistance are rarely silver bullets. Rather, the goal is to spur innovation and research and try multiple approaches. Given my experience and the experiences of many others with CF and other antibiotic-resistant infections that have benefited from phage therapy when all else has failed, it seems clear that phage therapy carries promise, but it is just one approach. We need to be firing on all fronts.
Future of AMR treatments
Bacteria are incredibly adaptive and resilient so developing new antibiotics under the same classes or even some new ones won’t be effective for very long. Minimising the rate of resistance would involve innovative approaches for new anti-infective products. We need to start looking at alternate ways of attacking these organisms, through harnessing the immune system, inhibiting bacterial cell interactions, and breaking down biofilms. Some of these are in preclinical development within the cystic fibrosis space but there needs to be continued investment into research and development and support for smaller bio-techs in this arena.
Concurrently, there is a strong need for the development of rapid diagnostic bacterial testing to determine the resistance profiles to allow for effective treatment sooner that minimise the rate of resistance as well as enable hospitals to confidently use these type of novel antibiotics for patients who most need them without the fear of unnecessarily accelerating the development of antibiotic resistance.
My future and mission
With antibiotic effectiveness decreasing, the exploration and implementation of new therapies for infections will be crucial. In reality, I was very sick when I underwent phage therapy, and I still am. My lung function is still dangerously low.
At the end of 2019, I started on a new CF therapeutic called a modulator which helped me significantly, but after two years the effects of this treatment have become less pronounced.
I am in a position where I must find effective antimicrobial treatments once again.
Progress in science happens in stops and starts. In my case, the serendipity of timing presented me with an option – as risky and untested as it was – where no other options existed, and for that, I’m very grateful.
Propelling forward quickly and exploring out-of-the-box options like phage therapy is the only way to develop better solutions for people like me whose lives depend on it and who do not have time to wait. This will require action on the part of many players and stakeholders working together and it begins with sharing our voices, reaching out to policymakers, investors, researchers, and the broader public.
As I said at the start, CF is an invisible disease: despite a few outward symptoms, most onlookers would never know there’s a battle raging inside us. But one thing that does distinguish us is our determination to fight this disease for as long as we can.
On behalf of many with CF, I convey a message of gratitude for all researchers who dedicate their work to improving our lives, a plea to continue finding and fine-tuning treatments, and our assured optimism for those days to come when we will no longer hear those mucus-filled, crackling breathes—but rather the rapid whoosh of air, lungs fully expanding, delivering plentiful oxygen for our bodies to thrive.
Until that comes, I will continue advocating for a future where antimicrobial resistance won’t be the greatest threat to my life and to many others.
Ella Balasa
Ella Balasa is a patient advocate, consultant, and person living with cystic fibrosis. She was diagnosed at 18 months old and has experienced countless hospitalisations since being a child. She has committed her time to empowering patients and advancing research and healthcare strategies through her connections with researchers, pharmaceutical companies, and patient organisations. She is an advocate for the development of novel therapies for the treatment of antibiotic-resistant infections and speaks publicly to raise awareness of AMR as well as about the value of the patient perspective in research.
Ella also has a passion for writing; distilling clinical information for patient communities, and sharing about the hardships yet triumph that comes with living with a chronic illness. She has been recognised as a 2022 Health Union Social Health Award winner for Healthcare Collaborator and serves on research committees with the Cystic Fibrosis Foundation and the Patient Insights Board for Medidata. Through opportunities working with healthcare organisations and sharing her health journey, she aims to affect the healthcare landscape by raising awareness of cystic fibrosis, empowering self-advocacy in other patients, and delivering valuable patient insights.
More of her work and experiences can be found at www.ellabalasa.com.