He lay under a respirator for six months, waiting for his death. It happened otherwise. Today, she helps others suffering from a rare disease whose characteristic symptom is bruising. – To draw attention to our problem, on the occasion of Rare Diseases Day in the streets of Polish cities, we will be handing out blue lip-shaped lollipops to passers-by – says Piotr Manikowski, President of the Polish Association of People with Pulmonary Hypertension and Their Friends.
How long did it take doctors to diagnose your condition?
– That was 10 years ago. I was 28 years old and I couldn’t climb the stairs to the first floor. Even getting dressed or washing made a huge effort for me. I was choking, I was short of breath, I felt a prickling in my chest. Doctors suspected anemia, asthma, pulmonary embolism and neurosis. I even took tranquillizers. Of course, it didn’t help, because the diagnosis was wrong. When after 6 months I came to Warsaw to see prof. Adam Torbicki with suspected pulmonary embolism, he finally diagnosed idiopathic pulmonary hypertension.
Did you know what this diagnosis means?
– Not initially. I thought – I’ll take the high blood pressure pills and I’ll recover. It was only on the Internet that I read that it is a rare disease, affecting only 400 people in Poland, and that without treatment, half of them die within two years of diagnosis. I worked as an IT specialist. The diagnosis was related to the transition to a disability pension. My wife was then three months pregnant. I knew my condition was a burden for her. Unfortunately, I felt worse and it turned out that the only salvation for me was a lung transplant. The Ministry of Health financed this operation for me in Vienna.
How has it changed your life?
– I felt like a dog on a leash. I could do everything that was impossible before the transplant, because the effort was no longer difficult for me. Unfortunately, after three years, the malaise returned. The transplant was rejected.
Have you lost hope?
– Fully. I was in the hospital on a ventilator for six months and waited for my death. I was unconscious most of the time, though I had flashes of awareness. I remember morning washing, meals and medications – such everyday mechanical activities.
Why did you lose faith that the disease could be overcome?
– Before the transplant, I was told that this is the last resort and that if I fail, there is no plan “B”. So when physiotherapists came and tried to move my body, because I had been lying there for several months, it seemed so pointless to me, because I didn’t wait for anything anymore. Besides, the feeling of breathlessness was as severe as if you had to put a plastic bag over your head and tighten it around your neck. I just wanted it to be over.
And then there was a new chance for treatment …
– I was qualified for the second transplant, which also took place in Vienna. After a month, I returned to Poland full of strength.
How has this changed you?
– It’s been four years since the transplant. But you never know what will happen anyway. That’s why I live short term. I do not make distant plans, I do not chase money, but I enjoy life, every moment. My family, wife and sons are a great joy for me. I became involved in the activities of the Polish Association of People with Pulmonary Hypertension and Their Friends, of which I am the president.
Patients with pulmonary hypertension need support – what?
– Knowledge about this disease in society, as in the case of other rare diseases, is nonexistent. A healthy person cannot even imagine that a young person who cannot catch his breath often stops and pretends to write a text message so as not to arouse a sensation. Those sick who use wheelchairs when they get up to get into a car or a room also arouse a sensation, because it turns out that they are not paralyzed. That is why the Association promotes information about this disease everywhere.
This knowledge is also needed by doctors …
– Yes, because the disease is diagnosed too late. And because the drugs available today inhibit the progression of the disease, it’s important to start treatment before high blood pressure wreaks havoc on the body.
What problems is the association struggling with?
– In Poland, patients have access to drugs for pulmonary hypertension under therapeutic programs, but they are eligible for them only when the disease reaches a significant stage of advancement. Doctors believe that treatment should be started as soon as possible, because the inhibition of disease development will start at an earlier stage. So we are trying to convince the minister of health to change the eligibility criteria for the program. The reception of drugs is also a problem. Previously, the hospital could send her by courier. Today, patients have to do it in person. It is a terrible journey for those in serious condition. I know a sick woman who travels from the Tri-City to Otwock.
We would also like there to be several centers specialized in pulmonary hypertension in Poland, where doctors, having contact with many patients, could conduct research and improve the methods of therapy. As the disease is rare, it is difficult for a physician to gain clinical experience when caring for only one or a few patients.
What have you prepared for Rare Disease Day?
-28 February this year in Warsaw and on February 29 this year. In Krakow, Bydgoszcz and the Tri-City, a special “blue brigade” of our Association will appear on the streets and in selected shopping centers, which under the slogan “When you are out of breath …” will conduct an educational campaign on pulmonary hypertension. The action will be inaugurated with a performance in Warsaw – on February 28 this year. 12-00 in front of Metro Centrum. Anyone interested will be able to see the artistic vision of the fight against the disease performed by actors from the Puszka Theater. During the campaign, educational leaflets and blue lip-shaped lollipops will be distributed in all of the above-mentioned cities – the symbol of the campaign, because patients’ mouths turn purple.
Pulmonary hypertension is a progressive, fatal disease that affects the lungs and heart. It is characterized by high blood pressure in the pulmonary arteries. The mortality rate in pulmonary arterial hypertension is sometimes higher than in some cancers, including breast cancer and colorectal cancer. People suffering from this serious disease need support as it affects many aspects of everyday life, such as walking up stairs, walking and getting dressed. Common symptoms of the disease are shortness of breath, blue lips and tiredness. Symptoms are non-specific, so pulmonary hypertension is often confused with asthma or other diseases, and it takes months to years to make an accurate diagnosis. In Poland, 400 people are treated for this disease.
Interviewer: Halina Pilonis