Study: Pulmonary arterial hypertension causes impoverishment of patients and their families
More than half of patients with pulmonary arterial hypertension (PAH) had to give up work, completely or partially, shows the latest study among Polish patients. In most cases, this caused more than a quarter decrease in household income.
Information on this subject can be found in the report "Pulmonary arterial hypertension - various aspects of patient`s life" published in September 2018.
According to experts, early diagnosis of PAH, fast inclusion of treatment and comprehensive care can help people suffering from this dangerous disease maintain their fitness, activity and independence for as long as possible. Pulmonary arterial hypertension (PAH) is a rare disease, but it is very severe and more dangerous than many cancers. Half of the patients die within five years of diagnosis. About 1,100 people suffer from PAH in Poland. It is most often diagnosed in people between 45 and 50 years of age. Women account for two-thirds of patients.
PAH develops due to obstruction in the small arteries in the lung, and because this limits the flow of blood through the arteries, the heart must pump it into the lungs at a higher pressure. Over time, this leads to enlargement of the right ventricle and right atrium, and ultimately to heart failure and respiratory failure.
The disease manifests itself primarily with dyspnea, initially during exercise, and in the advanced stage of the disease even during rest. Other symptoms of the disease include fatigue and weakness, fainting during physical exertion, dry cough and hoarseness, as well as bluish lips and face during physical exertion.
"The condition of a patient with arterial pulmonary hypertension very much depends on the stage at which the disease it was diagnosed the treatment started" - said Dr. Magdalena Janus from the Cardiology Department of the Transfiguration Hospital in Poznań at a press meeting where the report "Pulmonary arterial hypertension - various aspects of patient`s life" was first presented. The meeting took place in late September in Warsaw.
According to Janus, there is a small group of patients with PAH, in whom the disease was diagnosed early and aggressive treatment was quickly started. These patients do not have many activity restrictions - they do sports, run, ride a bike, and swim.
"But most of our patients with pulmonary arterial hypertension have problems with activities such as walking to a bus stop, getting to work, walking from a parking space, climbing stairs. Work hours are also a problem because (...) patients with pulmonary arterial hypertension are not able to work non-stop for six hours" - explained Dr. Janus.
This means that PAH forces many people to completely or partially resign from work, or change its conditions. In the latest study, it was respectively 32%, 23% and 3% percent patients. In 71 percent patients, occupational changes caused a decrease in household income by more than a quarter, in 52% by more than half, and in 21% by more than three-quarters.
What`s more, the disease leads to many changes in the life of the caregiver of a person with PAH, who in 50 percent cases is a spouse or partner, in 19 percent cases a child, and in 15 percent cases a parent. In the study, only 4 percent carers declared that PAH had not caused any changes in their lives.
According to Alicja Morze, the president of the Polish Association of People with Pulmonary Hypertension and Their Friends, the role of the caregiver is "priceless" for patients with PAH, but it also depends on the stage of the disease.
"Pulmonary arterial hypertension, with which I have been struggling for over 20 years, has demolished my and my loved ones` lives because it is a disease that burdens the whole family. My husband supports me in my daily struggle with the disease. I would not be able to do anything without him. He washes me, hands me clothes, because I can not do it myself, I can not get to the door to open it, I can not get to the fridge in the kitchen to take food out" - she explained at the meeting.
She added that before the disease she was a very active person. She and her husband ran an antique and university bookstore. Now, as part of her work in the association, she fights for better treatment and care of patients with PAH. Alicja Morze pointed out that the only activity she can do alone is helping patients with PAH who report to the association on the phone.
"But many patients do not have such understanding and support from their partners. Sometimes the partner leaves because of the disease. That is why early diagnosis and treatment it is so important, so that we can remain independent and work as long as possible" - she noted.
Professor Marcin Kurzyna from the Department of Pulmonary Circulation, Venous Thromboembolism and Cardiology Department of the Postgraduate Medical Education Centre, European Health Centre in Otwock emphasized that care for a person suffering from PAH must be managed by a therapeutic team. "Patients with PAH suffer not only from physical illness (...), this disease also has a profound impact on the patient`s psyche, emotionality, contacts with family and friends" - he explained.
The study shows that half of the patients experienced an extremely strong shock and fear due to the diagnosis with PAH. Prof. Kurzyna believes that in addition to doctors and nurses, therapeutic teamd should also include a psychologist, a social worker, a physiotherapist and, if necessary, a priest. Such a team is interested not only in physical symptoms, but in the whole life of the patient, it heals not only the patient`s body, but also the soul, the specialist argued.
Professor Grzegorz Kopeć, chairman of the Pulmonary Circulation Section of the Polish Society of Cardiology pointed out that in order to increase the effectiveness of PAH treatment, it is necessary to diagnose it as early as possible. There are many centres in Poland that treat patients with this disease. One of the problems is that doctors can not treat PAH patients with several medications at once. These drugs are available in our country as part of a drug program. "Currently, the standard in the treatment of pulmonary arterial hypertension is polytherapy - we know that a combination of two or three drugs works better than one drug. This is the recommendation in the European guidelines. Currently, we make efforts to use triple therapy" - said Prof. Kopeć.
Experts emphasized that in many cases the causes of the development of PAH are unknown. Those that are known include: congenital heart defects, connective tissue diseases such as scleroderma or systemic lupus, cirrhosis, HIV infection, toxins such as amphetamine, certain medications, for example appetite suppressants.
PAP - Science in Poland
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