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When Beata read on the Internet that she would live with her illness for no more than two years, she decided to quit her studies to enjoy her life.
Before the correct diagnosis was made, she was an anemic for six months, according to doctors, and an asthmatic for the next four months. Ultimately, it turned out that he did not suffer from any of these diseases. Her condition turned out to be much more dangerous.
Incorrect diagnosis
– When I was 22, I started to get tired quickly – says Beata from Toruń. – Upon entering the third floor, I was panting like a panting dog. Running up to the bus almost ended in fainting – he recalls. Her family doctor ordered her to perform a blood count. It turned out that the hemoglobin was below normal. The diagnosis was: heavy menstrual periods causing anemia. She started taking iron. The hemoglobin went up, but the symptoms did not go away. Desperate, she went to another doctor – an internist. This time she was suspected of having asthma. The new drugs did not improve well-being either. Every physical exertion meant breathlessness. The internist suggested a psychiatric consultation. She did not make it on time, because running up the university corridor to the classroom ended in losing consciousness. When she woke up, the ambulance service was taking her to the hospital. There, her heart echoed. It turned out that he most likely suffered from pulmonary arterial hypertension.
Symptoms of pulmonary arterial hypertension
Pulmonary arterial hypertension is a disease in which the blood pressure in the pulmonary artery rises significantly above normal levels. The cause is the overgrowth of small pulmonary arterioles. Blood cannot flow freely through the pulmonary vessels, which increases the pressure. In addition, the blood cannot take up as much oxygen in the lungs as the whole body needs. And yet it is from there that oxygen is distributed to all organs. Therefore, the first and main symptom is shortness of breath, a feeling of lack of air, shortness of breath especially during exercise. The heart starts to work faster to pump more and more blood to the organs demanding oxygen. As a consequence, its failure occurs. Other symptoms that develop with the development of the disease are fatigue and general exhaustion, dizziness, fainting, tightness or pain in the chest, especially with exertion, swelling of the ankles or legs – swelling, and a rapid or irregular pulse.
A rare disease
After returning from the hospital, she began to search the internet. She read that the survival time in this disease is 2 years. She couldn’t believe it. It turned out that the disease is very rare. In Poland, about 300-400 people suffer from it. – Why me? The question kept coming back. – I did not smoke, I ate healthy, I even went to volleyball in high school. What did I deserve – Beata wondered. She also read that the disease can be hereditary. However, no one in her family suffered from this disease. Completely broken, she went with her mother for a consultation to the Department of Internal Diseases of the Chest, Institute of Tuberculosis and Lung Diseases in Warsaw.
Incidence of pulmonary arterial hypertension
Pulmonary arterial hypertension can develop in newborns, children, and adults of both genders. Most often, however, it occurs between the ages of 30 and 50. In younger age groups, women are sick more often than men. In many cases, it is not known what triggers the pulmonary artery lesions. In a small proportion of patients, family history and the presence of mutations in certain genes indicate the hereditary nature of the disease. It should be emphasized, however, that neither the presence of a genetic mutation nor the emergence of risk factors mean that the disease will develop. In contrast, only about 20% of people with the abnormal gene will develop it. Systemic tissue diseases, HIV infection and portal hypertension are also among the risk factors for the development of the disease.
Modern drugs for pulmonary hypertension
In Warsaw, the diagnosis was confirmed. Beata asked the doctor who looked after her if she would suffer during the two years she had left. Dr hab. Marcin Kurzyna, MD, a cardiologist, listened to her life plans with surprise. “I will quit my studies because if I’m going to die, it doesn’t make sense,” she said. – Why should you die? – He asked. “Because you live with this disease for no more than two years,” she replied. “This is historical data from before the modern drug age,” replied the doctor. He explained to her that for two years in Poland, patients in Poland have been able to receive free medications that allow them to live with pulmonary hypertension as with a chronic disease. Although pulmonary arterial hypertension remains an incurable disease, new drugs inhibit the development of this disease. They reduce mortality by more than 40% within 3-4 months of their use. In addition, treatment improves physical performance. If drug therapy is unsuccessful, in some cases it may be necessary to undergo a single or both lung transplant, and sometimes a combined heart and lung transplant.
Diagnosis of pulmonary arterial hypertension
According to Dr. Kurzyna, late diagnosis is a huge problem. Meanwhile, treatment of pulmonary arterial hypertension should be started as soon as possible. However, it is very difficult due to the nature of the symptoms that can also accompany many other diseases, and also appear only in the advanced form of the disease. For this reason, it can take months or even years to get a proper diagnosis. “The disease is so rare that an average physician has no chance of encountering it,” says Dr. Kurzyna. That is why many doctors do not even suspect it. It is good if the patient goes to a cardiologist or a specialist in lung diseases. The echo test of the heart allows for a preliminary diagnosis, which is confirmed by other specialist tests.
Health restrictions
Beata started taking medications. She felt better. She continues her studies. – A person diagnosed with pulmonary hypertension should not give up on an active lifestyle, as long as this activity does not cause pain or other disturbing symptoms – says Dr. Kurzyna. There are some limitations – weight control, a diet to limit salt and fluid intake, no smoking or drinking alcohol. It is also necessary to prevent pregnancy as it is life-threatening. – It may end with the death of the woman and the death of the fetus – warns Dr. Kurzyna. He talks about his patient who developed pulmonary arterial hypertension during pregnancy. Her father died suddenly at the age of forty. When the older brother was diagnosed with pulmonary arterial hypertension, doctors concluded that the disease could have killed his father. Because the brother started to heal in a very advanced state of disease, he died. – My patient has been thoroughly examined. She seemed to be healthy. Willing to fulfill her wish for motherhood, she became pregnant. Unfortunately, pregnancy accelerated the development of a previously latent disease. She died shortly after giving birth. The child was saved. So her mother lost three of her closest relatives as a result of this disease. He now lives with his granddaughter who – hopefully – will never develop pulmonary arterial hypertension – says Dr. Kurzyna.
Text: Halina Pilonis