Fast Confession - Double lung transplantee Nikola Borešová: My body did not accept the first pair of lungs, I waited a year and a half
Nikola Borešová is only 26 years old. However, few people in their fifties can imagine what she has already experienced. This beautiful young girl suffers from an incurable, so-called invisible disease, cystic fibrosis. Patients with this disease usually don't live past the age of 30. Their lungs fail. But Nikola doesn't let anything like that get to her. When things got rough, she was given new lungs. And when her body decided not to accept them, she went for it again. In an interview with LP-Life.com, she talked about what a person with unclear breathing experiences, and what she can and cannot plan for the future.
Many people can't even imagine what it means to have cystic fibrosis. You've had it since birth?
It's congenital. Everyone’s lungs produce mucus, but patients with this disease can't cough the mucus out, it settles in our lungs. Thus, the lung capacity decreases, until finally a lung transplant is needed.
But it's not the same for all fibrosis patients.
On the one hand, it is the same for everyone because cystic fibrosis always affects the lungs. The conclusion is always that transplantation is the only option left to give us the ability to live a normal life.
If you've had it since childhood, you must have had a lot of limitations compared to your healthy peers. What were some of those?
I had to practice increased hygiene, increased sanitary measures. Take, for example, going to the swimming pool with this disease. There are some restrictions from the get-go, since there are bacteria in the pools. Yet, for us, it's more crucial. A normal person wouldn't even know it, but for us, it could settle on our lungs and cause inflammation and things like that. Another thing tied to cystic fibrosis is poor digestion, we don't digest food well enough. We have to take pills to keep what we eat in our bodies.
Did you have to inhale?
We have to train our lungs from birth, so we have to exercise, inhale different preparations to get the mucus out, so that it doesn't settle on the lungs, because then the inflammation starts. It could even be a more serious infection, pneumonia, that sort of thing. We have to exercise since birth, train our lungs to expand, because our lungs slowly weaken over time and then they don't expand as much as they should, which results in reducing their capacity.
Do you feel that when you breathe? When we're talking to each other right now, do you feel it, can you breathe easily?
Now that the transplant's done, yes. But with cystic fibrosis, it depends on the situation. For me, it really kicked in during high school, I started having lower lung capacity, shortness of breath, that sort of thing. I was exempted from gym class, sprints didn't do me any good, my lungs couldn't handle it.
At what age did you start to notice that you were sick?
Not until high school, really. Fortunately, I had a normal childhood, I went to school normally, I had friends, we had fun. It didn't affect me, I just coughed more. We had to tell people about that, so they didn't think I was sick and think I was going to get them infected. I was coughing more, I had to take antibiotics more often and I stayed home from school longer sometimes. I had a higher non-attendance rate than anyone else at school.
Did your parents ever tell you how worried they were when they found out they had a sick baby?
When they found out, they didn't really know what cystic fibrosis was, what it entailed. It wasn't a thing before 1990, I was born in 1995. It had already been around for five years, but it was still something new. Now I'm 26 years old and I still find myself learning new things about the disease. But of course, they were scared at the beginning, before they started giving me the right medication and got it under control. I was in the hospital for six months, and of course, that's very hard on any parents. I had all sorts of drips, tubes in me. It was very emotionally taxing for my parents.
From what I've read, cystic fibrosis has a terrible prognosis. They say the patients die before they're 30.
I was told the average life expectancy is 25 years. I was 14 when I read that. But it depends on the individual patient. It's just an average. It varied based on whether the person has other diseases, a lot of people have diabetes on top of that, which I luckily avoided, also high or low blood pressure, kidney or liver disease. All of these can come together, and then it depends on how they manage it.
When were you first told you had to have a lung transplant?
That was when I was in high school. I was approaching my 18th birthday when they told me I should consider it.
Did they conclude that from your lung x-ray or something?
It was a continuous process, nothing sudden, it was getting worse gradually. The lung capacity went lower and lower. When the capacity drops below 30 percent, you should start to address it and see how fast the progression is. If the capacity is dropping rapidly or slowly. There are different tests for that, and to get them all done, you could spend six months just making the rounds to make it on the transplant list.
At 30 percent capacity, I don't think you can walk up a flight of stairs.
You still can. You do run out of breath, but it depends on the individual. It's all in the head. If you believe you can't walk up the stairs, you can't. Before my first transplant, I had a lung capacity of, if I remember it correctly, something like 18 percent when they included me in the list.
How long did you wait for your first set of lungs?
Those first ones I got quite abruptly. I got listed sometime in the summer. I was feeling good, I was thinking that I probably shouldn't even be listed yet, that there were people worse off than me, that since I could still walk the stairs, even though with some supplemental oxygen perhaps, I'm still not in that rough of a shape. But then one morning I woke up with my breathing way worsened, I started turning blue. I had a pneumothorax of the lung, that's a ruptured lung. That was kind of walking the line. Because with me having cystic fibrosis, the paramedics didn't know if they could do that classic procedure where they puncture the chest and suck out the air that escaped from the lung to inflate it. They didn't know what it would do to me, so they left it to the Motol hospital. When they did that in Motol, my other lung burst as well. So I didn't have any at that point. They gave me ten days while they'll be searching for a donor. Luckily, I think one was found the very next day.
So they gave you ten days, and if you didn't get a new set of lungs in those ten days, you would have died?
Yeah, since the chances to make it are too slim at that point.
Didn't they reinflate your lungs with a drain?
No, they didn't. Given the decrepit state the lungs were in, at 18% capacity they had no chance of getting them to work anymore.
Were you conscious then?
No, I was asleep.
How did your parents react?
It must have been crazy for them.
What if the lungs were never found? You would have died!
It's better now. There's a lady who was put on an ECMO as well, holding the record at 130 days. I guess it could be done these days, but it was different then, and I was in a worse shape on top of that. It all boiled down to me having a slim chance to make it. Fortunately, the lungs were found.
Do you know whose they were?
I know they came from a man, but everything about that is classified.
When you woke up with new lungs, how was your breathing?
I honestly didn't believe the doctor that I'd really had a transplant. When I collapsed, they just told me they'd put me to sleep so I could rest. Then when I woke up, I thought they had admitted me to the hospital, I got better and it was only the second day after the collapse.
How many days did you spend in artificial sleep?
About four. But I didn't believe the doctor that I had a transplant at first. When you wake up, you're still a little groggy, you've got various tubes all around you, you're on painkillers, not really aware of anything.
How do they operate on the lungs, does it go through the shoulder blade?
Around the breasts, under the breast, where the underwire sits.
So you have two scars under your breasts?
Yeah.
So you say couldn't believe it, but how was your breathing?
When I was still on the painkillers, I could feel pressure in my chest. I was like, wow, that's perhaps even worse than before the transplant. I was scared of what was happening. You think you're going to feel relief after the transplant, but then they found out I had fluid in there and it was pressing on my chest. That was the reason why I didn't feel any improvement, on the contrary, something else entirely. But then, as the swelling went down gradually, it got much, much better. It took time, though.
What does one do after a transplant?
As you lie down, the muscles atrophy, especially in the legs. They try to get you back on your feet as soon as possible, because if you lay down for too long, you could get pneumonia. The longer you lie down, the worse it gets for you. A physical therapist was coming to us, she got us exercising lying down, then sitting up, over time we started to walk the halls.
You're speaking in the plural. Were there more of you?
There was a rotation of staff, usually some other transplantee arrived as well, as little as two days apart. We'd see each other in the hallway, then someone else would arrive. We communicate through social media as well. I feel like we're speaking in the plural form every time we talk.
There aren't many of you with cystic fibrosis, though.
There are fewer of us than, say, people with pulmonary hypertension. There’s probably more of them.
Nevertheless, you needed a second pair of lungs. Why was that?
Unfortunately, the first set didn't take, I had a rejection. It's a reaction where the body knows there's a foreign object or organ in the body and tries to expel it. They give us immunosuppressants, medication to suppress the immunity so the body doesn't fight it so much. I had a stronger immunity at the time despite the pills, and my body didn't want to accept the new lungs.
What's going on at that point?
You can't see it on the outside, but you start to get short of breath, it's harder to breathe when you walk up a flight of stairs or when you just walk normally. You already know something is wrong, especially when you were breathing well, and suddenly there's a hint of it going back to the way it used to be. I don't know how others experience it, I felt pressure under my neck, like something was always placed on my chest.
How long after the transplant did this happen?
It happened after six months. I had my first rejection two days after the transplant, but they thought it was a coincidence. But my body kept fighting it. I've had problems with it from the beginning.
So the whole round of testing, listing and waiting for lungs started again?
No, it held for four years. We tried to keep the lungs at all costs, not knowing how the other set would turn out. Second transplants don’t happen much. I only have one kidney as well.
How did that happen to you?
I was a year old then. I have coagulation problems, I had an embolism, but luckily the blood vessel got only blocked in my kidney.
Is that related to cystic fibrosis?
No, it's not.
So your immunity is weaker than other people's now.
Yeah, it is.
So you say you spent four years fighting to keep your lungs.
The doctor's been trying to come up with all kinds of tests. I've been even going on dialysis to keep the lungs working as long as possible.
Did you do anything other than stay in the hospital for those four years?
Yes, I went to work.
What kind of work do you do?
I work in a clothing store now, before that I worked in a betting agency. It didn’t hinder me from working.
Are you on a disability pension?
I am, I have the third degree. We can't be under any strain, in a dusty environment, ideally have minimal people around, especially at this time when it's risky. For us, even the regular flu is dangerous.
So when did you get your second pair of lungs?
A year ago, in August 2020.
Do you know whose they are?
I don't. They're something else entirely this time around, though. It's functioning at 70%, which is 100% from my point of view. But it depends on height, weight, gender. Some transplantees have over 100%, depending on what lungs they get.
How are you faring in the COVID era, with your transplanted lungs? Have you been going out at all?
I tried to stay home or with family, didn't go out to restaurants, limited contact with friends. We didn't know what to expect, the doctors didn't know what it could do to us, even the standard flu is a risk for us. It also depends on the patient's condition. If they contract something, for example the COVID, and they have another disease, even just a bladder infection, it means they may end in worse condition than if I contracted it now, as I am free of infection. It depends on each patient's situation.
I'm assuming you haven't had the COVID.
No, I didn't.
When the vaccine came, was it a lifesaver for you?
I was happy I can do at least something rather than doing nothing. I don’t think this will take me down, given all the other chemicals I already have in me. If me getting vaccinated would help the situation, why not go for it? But then again, for those who are healthy and don't want to get vaccinated, I don't blame them. It's their life and their choice. Everyone thinks - it can't happen to me, and even if it did, I will have a fine course. But for me, I'd rather prevent it than end up in the ICU and hope for the best.
You know all too well what it's like to be on a lung ventilator.
Exactly. Especially when I was waiting for my second transplant. I ended up on a ventilator before they even put me on the list. It's a really bad feeling when you know you're dependent on a ventilator and you're not on the transplant list yet. I was at 15% at the time, so the prospects were poor. Even just the emotional strain of not being on the list was enough. You’re waiting for the transplant while being in a critical condition, plus you have to go for tests and the doctors are also taking into consideration whether you can make it through the operation at all.
How long did you wait for the second set of lungs?
A year and a half.
How long were you on a ventilator?
Less than a month, luckily. Fortunately, my health has settled down a bit, and I've gotten stable enough that they were able to get me listed. It was pure chance. There's a lot of people waiting for lungs, and there are no guarantees they're gonna get them.
How are you doing now? What do you have to do, what do you have to watch out for? Do you do some physical therapy or inhalations?
I have an inhaler at home, I inhale. I also have to exercise at home, including my chest, so that my chest muscles don't get cramped or stiff. That's important too, when the muscles contract a little, the function of the airways is reduced as well. I have to keep fit, even though I'm no athlete, I have to keep in shape.
What helps best, running, swimming?
Yes, exactly.
You still go to work. Are you able to focus on your personal life or love life with all that going on?
Sure. I've been engaged for a year, we're planning a wedding for next year. There's a lot of things getting arranged.
Can you get pregnant?
We can, but there hasn’t been any woman yet to become pregnant after a transplant here. She'd have to cut back on the immunosuppressant pills, so the doctors are worried the lungs will stop working then. They don't have the experience to say - go for it.
Are you gonna do it?
I'm on the fence. I'm still young, but this is the second time I've been given the chance to have new lungs. It's still 50/50 inside. If we're gonna do it, we'd better do it under medical supervision.
What's your message to healthy people in particular?
My message would be that they should find a priority to live for. Find something and pursue it. Have a goal. Everybody has to have something to live for.
