Contents
Pulmonary arterial hypertension (PAH) is still a little known disease. It appears out of nowhere and mainly affects young people. The most powerful drugs improve the condition of patients and increase the chance of a longer life, but their use can be burdensome. There are also newer orally administered drugs on the market.
Lack of breath, shortness of breath, weariness, tiredness. These are usually the first symptoms of pulmonary arterial hypertension. Most people associate these ailments with an imperfect lifestyle. He most often attributes them to lack of sleep, fatigue, overwork and ignores them. After all, with age, each of us has worse physical capacity, so getting to the second floor becomes more and more problematic. Especially when we drive a car everywhere or eat too much or too much fat.
– Patients do not suspect any serious health problems. They go on a diet or sign up for classes at a fitness club. Only when they faint during exercise or are close to fainting, do they go to the doctor – says Prof. Marcin Kurzyna from the Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology, CMKP at the European Health Center in Otwock.
It is not known how long it takes before the sick person feels bad enough that he begins to seek help from a doctor. The symptoms of PAH are atypical and the disease progresses slowly. – PAH is not a heart attack or pulmonary embolism, the symptoms of which are clear and easy to diagnose – says Prof. Kurzyna.
Primary care physicians have trouble diagnosing PAH because it is a rare disease. It is estimated that in Poland 120 people are diagnosed with the disease each year. In total, about 1,1 thousand people suffer from it. people. – A general practitioner may never meet a patient with PAH – adds prof. Kurzyna.
Diagnosis of pulmonary arterial hypertension
The symptoms of PAH are unusual. They may indicate various types of respiratory or cardiovascular diseases. Usually, from the first symptoms of the disease to the first contact of the patient with a doctor, it takes about 12 months, and from 2 to 4 years until the beginning of appropriate treatment. – However, there are patients who are diagnosed with the disease after a month, and others who find out that they have PAH only after seeking help for several years. Interestingly, it has nothing to do with the place of residence – says prof. Kurzyna.
The diagnosis of PAH is made by excluding other, much more common diseases. GPs most often suspect asthma in patients first, but when spirometry does not indicate it and anti-asthma medications do not work, it is a sign that the causes of the ailments need to be looked for further. The key test is the heart echo, which shows that something is wrong in the lungs. However, it does not tell us why there were problems with blood flow in the lungs. Therefore, their cause does not have to be PAH, but may be cigarette smoking, pulmonary embolism, and emphysema. There are no clear diagnostic tools for PAH, such as microscopic examination of a tumor specimen in neoplastic diseases. Therefore, the diagnosis of PAH should be performed in centers with extensive experience in this field – believes Prof. Kurzyna.
It is slightly easier to diagnose PAH in young people, because most cardiovascular and respiratory diseases appear only in the elderly. But recently PAH is also found in people over 50-60 years of age. – This may be the result of better diagnostics and greater awareness of the disease – believes Prof. Kurzyna.
The staging of PAH is assessed using the four-level NYHA classification:
- Class I – the disease does not limit physical activity; the patient does not feel fatigue, shortness of breath, chest pain or pre-syncope
- Class II – the patient does not feel any discomfort while resting, but begins to feel worse during normal physical activity – he is tired, has shortness of breath, chest pains or presyncope
- Class III – the patient does not feel any discomfort while resting, but must limit physical activity. Even with little exertion, he feels tired, suffers from shortness of breath, chest pains, or pre-syncope
- Class IV – the patient is not able to perform any physical exertion, even while resting, he may have symptoms of right ventricular failure, shortness of breath and feel tired.
The most often referred to specialists are patients with the so-called NYHA Grade III.
Treatment of pulmonary arterial hypertension
PAH can happen to anyone, often at the best time in their life, no matter what lifestyle they lead. Part of the disease is genetic, but scientists do not know what activates the genes.
However, it is known how to treat this disease. In Poland, the reimbursement of drugs for patients with PAH was extended last year and now doctors have at their disposal drugs from three therapeutic groups. However, some limitations were introduced in the therapeutic program. – Three-drug therapy is available to patients who are NYHA class IV. In patients with NYHA class II and III, we must start with monotherapy and only when it does not work, we can add another drug or two. However, it should be emphasized that we can change the therapy after a month of observation of the patient. Therefore, a large part of decisions regarding treatment is the responsibility of doctors – says Prof. Kurzyna
However, the use of reimbursed drugs is not comfortable for patients – drugs are administered parenterally: by subcutaneous or intravenous infusions, or by inhalation. Punctures are sometimes painful, vascular ports (i.e. tubes placed in a blood vessel) put in place for infusion can lead to infection, and inhalations irritate the respiratory tract, resulting in a troublesome cough. Patients prefer oral medications, the use of which is much more convenient and does not cause the same problems as parenteral medications. Such a drug – called the active substance selexipag – has been approved in the European Union. In Poland, it is waiting for a refund
Drugs give great improvement in most patients. (There are, however, a group of patients for whom drugs do not work. For them, the only solution is lung transplantation.) Changes in pulmonary arterioles stop or even regress, although very rarely to their pre-disease state. – Drugs give the heart time to adapt to new conditions, to rest from the increasing burden. They made it possible to change the face of the disease from a fatal one, with a sentence postponed for two and a half years, to a chronic disease, which we are better able to control – says prof. Kurzyna.
Improvement is visible from week to week. Patients are feeling better, their heart health improves, and that also means they will live longer. The earlier the disease is diagnosed and treatment is started, the easier it is to slow it down, stop it, or even reverse it. Medicines allow patients to return to a normal life, for example to work, which is reflected in the economic and psychosocial situation.
Economics and pulmonary arterial hypertension
PAH is an expensive disease. 30-40 percent patients are forced to resign from work. This significantly lowers their standard of living. As much as 90 percent of patients declare a decrease in income after hearing the diagnosis. And as PAH is still a little-known disease, even among physicians, two-thirds of patients admitted in the report that they had difficulty applying for social benefits.
PAH costs also relatives of the sick. Over 30 percent caregivers admitted that they had to change jobs or work shorter hours, e.g. three-quarters or half-time, in order to be able to care for patients.
Modern treatment allows both the sick and their loved ones to work. This will allow for savings for the state budget. In 2017, 107 pension certificates and 16 entitlements to rehabilitation benefits were issued. In 2017, the costs related to the services provided amounted to PLN 5,6 million. The largest share in this amount were pensions due to incapacity for work, the amount of which amounted to approximately PLN 4,2 million. The costs of sickness absenteeism amounted to PLN 813 thousand. zloty.
Patients with pulmonary arterial hypertension
The disease takes the breath of patients with PAH. It was getting harder and harder for them to breathe. Until a few years ago, Michał played football and volleyball. “Now I can forget about it. I also had to give up the bike, because even the small climbs were very difficult to climb. An electric scooter is a nice alternative, it works well in the city and during family trips. My wife goes with her daughter, and I follow him on a scooter, ”he confessed in the forum of PAH patients.
Over time, even the smallest activities of everyday life are quite a feat for patients with PAH. “Even going to the store, trying on a few things or washing your head can be tiring. Sometimes I don’t have the strength to apply the balm all over my body, ”writes Hanna. But the real challenge is cleaning. Or leaving the house in windy weather. “I have a bad breath then. I walked a short distance – from car to apartment – 15 steps and I have no strength. Even walking at a slow pace, I get tired anyway, ”writes Gosia.
Doctors advise against pregnancy in patients with PAH. “The doctor said it was a miracle that I gave birth” – confessed Kasia, who was diagnosed only after the baby was born. Ela was diagnosed with the disease before her pregnancy, but the woman really wanted to have a baby. “It was really hard, but I survived. Doctors said there were times when they didn’t give me a chance anymore. ” – she confessed. Agnieszka did not decide to have a child. “I won’t be able to do it, I’m afraid that the baby will be left alone, because I … It is known that the desire to be a mother is huge, but the risk of complications is, unfortunately, greater,” she wrote.
Illness not only takes the breath away. “Chest pains are normal with our disease. That’s what my doctor told me. The heart hurts because it is inefficient… like a muscle. You must make friends with this pain … ”wrote Paweł.
“When I was diagnosed with pulmonary arterial hypertension, I felt terrible for 2 months. After a 2 km walk, I was falling. After my first bike ride, I got all swollen. My legs swell up a lot, sometimes my face too, ”wrote Darek. He has recently started taking medications and is getting better results. “I even got the go-ahead for running. Two light workouts are behind me. It’s a wonderful feeling! ” – he confessed.
Adam Concoction, the leader of the band Tears, even sitting at the table was tired, the trip was to go upstairs. He couldn’t eat. He was out of breath, even when he was not doing anything. Several times he was closer to the other world than this. The next day he felt better, but he was overcome by a state of utter discouragement to life. – I stopped having the joy of life. If euthanasia on demand was possible, I would give in to it – says Adam Konkol, leader of the band Łzy. He felt so bad twice. “Not being able to breathe is the worst thing a human can experience.” Even with my wife and child, I felt I had no strength to live any longer. I did not have the strength to move, I struggled to take a few steps, I was propping myself up against the walls, he says.
Adam is a brave person, but when famous cardiologists told him that there was nothing they could do, that it would not be better, but worse, he lost his will to live. And he wanted to live because he likes life. Doctors mentally prepared him for a simultaneous lung and heart transplant. It was supposed to be his only chance. – But life after the transplant does not have to be better. The transplant patients I spoke to complained about not feeling well, says Adam.
He did not think that medications could be the salvation. – Five years ago, someone called me and said that there was a drug that would restore my will to live, that I would be able to walk effortlessly and even ride a bicycle. It was a time when I struggled upstairs. I thought that someone was screwing me on – says Adam. After he started taking the drug, he felt better after three days. – The change was colossal. As if I had died and was born again. And when one day I went upstairs in a restaurant and didn’t even notice it, I realized that the drug was working. Then I cried. I started to function normally from the state of vegetation – admits Adam.
Thanks to therapy, he returned to the stage. He started touring with concerts, although not as intensely as before. He saves himself, but trips are not a problem for him. He hasn’t come back to playing the drums, but he plays guitar and composes. He is also not tired of a long walk, a short run or climbing stairs to the second floor. He is tired of bending down and tying his shoes while standing up.
– I have always lived like a healthy teenager, I have not succumbed to disease. I was not locked at home. Maybe this is one of the reasons why I’m still alive – says Adam, who will turn 44 in December and has had TNP since birth. She is one of the longest living people with this disease.
Doctors advised against starting a family for Adam. They said outright that he could orphan children. Before the wedding, the priest asked his future wife if she really knew what he was doing. But it was the family – his wife and children, 5- and 9-year-old today, that gave him strength. And they changed him. – I lived like a playboy, I spent my money without thinking about the future – he admits.
Today, he listens to the body and tries to save it. Thanks to the fact that the medicine that saved him was found, he no longer thinks about how to use the last moments of his life, but looks to the future with optimism. He would like to live to be 106 years old. “If my well-being worsens, I hope they’ll come up with a drug again.” And today I feel happy and fulfilled – admits Adam Konkol.