«I’ve been living in a pandemic for nine years. Cystic fibrosis is a terrible torment ». Marta’s story

– I don’t know what free breathing is. I breathe as if a pillow had been placed against my face and there was concrete in my lungs. You are trying your best, but it gets stiffer and stiffer. Cystic fibrosis is a terrible torment – says Marta, one of the oldest people with cystic fibrosis in Poland. Due to the disease, the woman has been living as in a pandemic for nine years. What happens to the body of a patient with cystic fibrosis, what is everyday life like in this disease, as well as the chance to come back to life that appeared – she told Medonet.

1. Cystic fibrosis is a genetic disease. It impairs the respiratory system, and the digestive tract and other organs also cause problems
2. The cause of the disorder is the thick mucus that the body produces, which clogs the lungs and pancreas and is a breeding ground for bacteria
3. Marta is 49 years old and is one of the oldest people with cystic fibrosis in Poland (the average life expectancy with this disease in our country is approx. 25 years)
4. Due to the high risk of infection, the woman has been living like in a pandemic for nine years. There is no way to go to the gallery or the cinema. Inhalations and drains take eight hours a day and you cannot give up on them, because it will only get worse
5. The hope for Martha is a drug which, as she says herself, “hits the real cause of the disease and literally turns it off”. Unfortunately, in Poland it is not reimbursed, the monthly cost of Marta’s therapy is approx. PLN 80 thousand. zloty
6. You can find more such stories on the Onet homepage.

Monika Mikołajska listened to it

It was safer not to talk about cystic fibrosis

From the moment I was born, my mother saw that there was something wrong with me. As a nurse, she knew this very well. When I was little, I went through a collapse. I just walked away for a few minutes, died – to put it bluntly. Only seven years later, after many visits to various hospitals, I was diagnosed with cystic fibrosis. It was 1980. Completely different times. However, I can say that cystic fibrosis gave me some really extreme experiences in my life.

A few years later, during a gastroscopy to which I was put down, I had a clinical death. I wasn’t completely aware of it, I thought I was fine. It was only when I regained consciousness that I was surprised that there was great excitement around, everyone was running, not letting me get out of bed. What did this have to do with cystic fibrosis? This disease makes the body very weak, and the difficulty in breathing it causes is a high risk, for example during anesthesia.

At school and in college, no one but my family and friends knew I was sick. In those days, such people were rather uphill. I experienced it when I wanted to go to an art school. Students struggling with diseases or other problems were sent to a pedagogical and psychological counseling center. There, they were to receive help and guidance on choosing a further school. In my case, it was found that only a general secondary school was involved. It was a moment of breakdown, which only confirmed my conviction that it is safer not to just talk about cystic fibrosis and not show the document from the clinic anywhere.

I took my papers for art school, I passed the exams without any problems. The stay in this school was one of the miracles that happened to me in my life. There we were treated individually, which was very important for a sensitive and sick person. Anyway, this is the most appropriate and normal approach to man. I also met the same gaze at the Academy of Fine Arts, where I later studied sculpture.

Cystic fibrosis destroys the body from the inside out

If someone has never had difficulty breathing, it is difficult for him to imagine what he is going through with cystic fibrosis. And how much fear comes when you start to choke.

1. When is breathlessness a serious illness? [WE EXPLAIN]

I don’t know what a free deep breath is. I breathe as if a pillow had been placed against my face and there was concrete in my lungs. You are trying your best to breathe, but it becomes more and more stuffy. And that’s the greatest pain imaginable. There is also fear and helplessness, because you know you have no influence on it.

The cause of all these problems is the monstrously thick mucus that the body produces, which can be compared to pudding. This mucus clogs the lungs (and pancreas), but it is also a breeding ground for dangerous bacteria. We often get severe pneumonia. Continuous inflammation promotes fibrosis, the formation of edema, or the collapse of a part of the lung (atelectasis).

Photo Private archive

Cystic fibrosis is a disease that progresses. This means that as the years go by, it becomes more and more difficult to breathe, and it becomes weaker and weaker. In addition, there are disorders in the work of the pancreas, which leads to malnutrition and cachexia. In cystic fibrosis, the liver also suffers, which is devastated by the constant use of drugs (it leads to steatosis or fibrosis). The immunity is much weaker (also due to the medications taken). Simply put – cystic fibrosis destroys the body from the inside, more and more over time, although we try to keep it in check. Despite all this suffering, it is a disease that cannot be seen from the outside. I would also like to emphasize that you cannot catch it, the reasons lie in the genetic code.

I’ve been living in a pandemic for nine years

I don’t really know the so-called healthy life, or rather, I only know them from my friends’ movies and stories. In my case, everything is subordinated to cystic fibrosis. She sets limits for me that a healthy person simply does not think about. With cystic fibrosis, you can never plan anything, you cannot predict what will be in a month or a day. Maybe my condition will suddenly worsen, maybe it will be necessary to go to the hospital. All I need to do is see someone with a cold and the risk of a serious infection is very high.

This is reminiscent of the current COVID-19 situation and the most severe regime. Only me I have been living in a pandemic since 2012 and it has become my normal everyday life. A visit to a gallery or a supermarket is not an option, as is the use of buses, trams or going to the cinema. Even inviting your friends home is not easy. My lungs catch everything like glue, and when the pathogen gets into them, it will find the perfect medium. In this situation, the risk of infection and even greater strain on the body is enormous. There may be situations where the body will no longer recover from it.

However, I try to go for walks and see my friends outdoors. I go out with a special three-kilogram device on my back to help me breathe. However, I have to be careful not to overestimate my strength and watch over time – I have as much time as the batteries work.

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Cystic fibrosis is an excellent time thief. I have to do seven inhalations and three drains every day. It’s hard work and kind of breaking yourself. It doesn’t matter if I have a headache, if I have a fever, if I have enough strength or not, it just has to be done. If you let go, it will only get worse. It takes a huge part of the day. I once dared to count it – it turned out that the so-called the ordinances take about eight hours a day. Someone told me it’s like my job. Yes, the daily struggle for the lungs to be able to breathe and function is my job.

During COVID-19, my lungs slowly started shutting down

Despite great caution, wearing masks, gloves and following other rules of the regime, problems could not be avoided. I fell ill with COVID-19 in winter and I don’t know how it happened. I went through the whole illness at home completely alone, which was extremely difficult both physically and mentally. I was not admitted to the hospital in which I was treated because of SARS-CoV-2, and it was not so bad that I had to “go under a respirator”. It was only after a month that I was admitted to the ward.

I remember the time of my illness very hard. Saturation was falling, severe nosebleeds began, which were very difficult to stop. My lungs were getting so tired it was slowly shutting down, and so was my heart. The body seems to stop defending itself. Even so, I couldn’t let go of all these inhalations and drains, although the only thing a person wants in such a state is to go to bed. Here the fight goes on all the time, there is no leniency.

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Fortunately, I was able to recover, although so far I feel the effects of COVID-19. I’m still under oxygen, my heart isn’t quite steady yet. My hair is falling out in handfuls. The muscles are very very weak, actually atrophy. More than once in my life I have risen “from the dead” and learned to walk anew, so in a sense I am more resistant to such situations.

I will never forget how, in this difficult time, when no one was allowed to visit me, a foreign woman who has a child with cystic fibrosis brought me a Santa gift at my door. It was amazing.

Pills that turn off disease. The effects are amazing

Recently, there is a light in the tunnel, and in fact, a great chance to come back to life. Until now, only the symptoms of cystic fibrosis have been treated. I was given bronchodilators and mucus-thinning medications. This, however, did not stop the changes. Now, for my mutation, there is a drug that hits the real cause of the disease and literally turns it off.

In the USA, patients have had access to it for about 1,5 years. It is also used by patients in Germany and other European countries. These people say that thanks to these inconspicuous pills, the production of monstrously thick mucus stops, the person stops choking, the pancreas starts to work normally, you gain body, your strength regains. You can start working, you can regain your independence, you can start living.

Photo Private archive

Although it is impossible to talk about a full recovery, because the changes in the lungs will not regress, the effects are remarkable. After administration of the drug, even people whose lung capacity has shrunk to 12%. and the only salvation was a transplant, they no longer need surgery. It’s all hard to believe. Similar to the fact that, for example, in Germany, patients simply have this medicine for free. In Poland, unfortunately, AOTiM (Agency for Health Technology Assessment and Tariffs, which supports the minister of health in the process of making decisions related to financing services in the health care system – editor’s note) rejected the need for reimbursement of the preparation, and its price exceeds the capabilities of an ordinary person. In my case, the cost of the therapy is around PLN 80. PLN per month, and these drugs must be taken permanently (in my situation, two preparations are necessary, both are not reimbursed for my mutation).

The worst part is that this wonder drug is almost at your fingertips, but it “almost” makes a huge difference. It is as if a thick glass pane separates me from it. In response to a reimbursement inquiry, I recently received a response from the Ministry of Health that we do have antibiotics. Meanwhile, they heal infections and chronic infections in the lungs, not the disease itself.

The end of our torment is at your fingertips

The whole situation is extremely harsh. For me, it is a fight for life, but also a race against time, because we, patients with cystic fibrosis, do not have much of it. At the beginning of the year, nine people left us, all of them young people. In Poland, the average life expectancy with cystic fibrosis is about 25 years. I am 49 years old. I am one of the oldest people with this disease in Poland. The fact that I am still here is a great miracle.

Friends have long been persuading me to organize a fundraiser. Until recently, I thought it was impossible, because the price of the drug is overwhelming and who will care about my life. After going through COVID-19, it occurred to me that there is no other salvation for me. You can help Marta in her fight to come back to life – for details, see siepomaga.pl.

In this whole therapy situation, it is not only about me, but also about other patients I know. Cystic fibrosis is a terrible ordeal, while stopping it is literally at hand – there is a cure. Many patients and families with sick children have decided to go abroad just to have access to it.

We people with cystic fibrosis just feel disconnected. Maybe the problem is that our disease is not visible and therefore not much is said about it. We also do not have the opportunity to go to the ministry and stand up for ourselves. Due to the risk of infection, we cannot meet, many of us stay in hospitals or are too weak to do so. So if a wise person does not look wholeheartedly at our situation, nothing will change. And we cannot wait. If the refund comes in a year or two, many of us just won’t live to see it.

You may be interested in:

1. The most common genetic diseases. How are they made?
2. I asked: should I be happy or cry? “Rather rejoice in this misfortune”
3. «This drug is a miracle for me. I dreamed about him all these years »

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