"Kui ma poleks teda õigel ajal kohanud, ma poleks
praegu elus."
AOP Health on seadnud endale ülesande aidata niinimetatud harvikhaigustega inimesi. Patsientide isiklik lood aitavad meil mõista nende erivajadusi, et uurida uusi ravivõimalusi, neid välja töötada ja turule tuua.
Harvikhaigusega patsiendi jaoks tähendab see haigus pidevat vastasseisu selliste teemadega nagu nende seisundi aktsepteerimine, pädev arstiabi ja sageli ka sotsiaalkindlustusprobleemid. Raskused hõlmavad peamiselt juurdepääsu spetsialiseerunud ravikeskustele, diagnostilisi uuringuid ja patsiendi jaoks asjakohase teabe kättesaadavust - harvikhaiguste valdkonnas on need tegurid aja, sisu ja kulude osas paras väljakutse.
Minu lugu
Polina Strelnikoval on pulmonaalne arteriaalne hüpertensioon ja tal oli väga õnnelik, et sai väga varakult pöörduda spetsialisti, prof. Irene Langi poole.
Millised olid teie haiguse esimesed sümptomid või nähud?
Minu meeleolu halvenes kuu aja jooksul väga tõsiselt. Tundsin, et pole võimeline spordiga tegelema. Esialgu võtsin kaalus palju juurde, mille tõttu proovisin jälle sporti teha, kuid ebaõnnestunult. Tundsin end halvemini kui varem.
Siis sain aru, et minuga on midagi valesti.
How does patient arrive at the diagnosis?
"The common symptoms could be associated with the common diseases of the heart or of the lungs. The key assessment to detect PAH is to make echocardiography which is the ultra sound of the heart and then it is necessary to exclude or to confirm lung diseases."
Prof Pavel Jansa
Kuidas tundsite end algperioodil, pärast ravi alustamist?
"Eriti ravi alguses olin täielikus meeleheites. See oli minu jaoks väga keeruline aeg. Isegi veel täna on mul halbu päevi, kui ma kahtlen ravi tõhususes. Kuid head päevad on ülekaalus, kuna ravi aitab mind ja see on kõige olulisem."
Minu arstid olid väga toetavad, eriti ravi alustamisel. Usaldan oma raviarste täielikult, sest ainult nemad saavad selgitada, mida ma läbi elan ja mis mu organismis toimub.
Teisest küljest on minu kaastöötajatel ja perel väga raske minu harvikhaigusega toime tulla. Nad kõik arvavad, et see on lihtsalt südamehaigus või kopsuhaigus, mida nagu tavaliselt saab hõlpsalt ravida. Kuid see pole lihtsalt südamehaigus ega kopsuhaigus!
Millist nõu annaksite endale täna?
Kui ma saaksin ajas tagasi minna, oleksin agressiivse ravimeetodi kasutusele võtnud palju varem. Vajasin paari nädalat, et selle üle järele mõelda, enne kui langetasin otsuse ravi kasuks.
Kuidas läheb teie igapäevaelus - kuidas te kirjeldaksite haigusega elamist?
Ma pean oma elu suuresti normaalseks. Käin tööl ja saan ka reisida.
Teatud ülesannete jaoks vajan lihtsalt palju rohkem aega. Minu hommikuste toimetuste aeg on pikenenud tunnist pooleteise tunnini.
Duši all käimine on tavaliselt katastroof, sest ma pean pumba eemaldama ja see on üsna vaevarikas protseduur. Aga ma saan hakkama.
"Muidugi ei saa ma sporti teha, mis on mulle isiklikult väga raske. Algselt oli see masendav, kuid nüüd on kõik korras. Tegelikult ei tunne ma end teisiti ega eriti haigena."
Raviarst
Professor Lang räägib meile PAH-ist:
"Kroonilise trombemboolse kopsuhüpertensiooni õige diagnoosi kindlaksmääramiseks kulub keskmiselt ligikaudu 14 kuud. Haigusprotsess algab aga palju varem - ajal, mil kellelgi pole kopsu hüpertensioonist veel aimugi. Ma tean ühte juhtumit, mil haiguse avaldumiseks kulus 30 aastat."
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Univ. Prof. Irene Lang, MD
Kuidas te ravite oma patsiente?
Püüame südame paremat poolt päästa vasodilatatsiooni (veresoonte laiendamise) abil. Kui ravi õnnestub, on see väga tõhus ja leevendab südame paremale poolele avaldatavat survet.
Püüan patsiente veenda agressiivset meetodit kasutama, sest meil on mõned patsiendid, kes on tänapäeva mõistes ilmselt "normaalsed".
Professor Irene Lang on Viini Meditsiiniülikooli kliiniline kardioloog ja vaskulaarbioloogia professor. Ta juhib kopsu-veresoonkonna haiguste ambulatoorset kliinikut ja töötab interventsionaalse kardioloogia sektoris.
Spetsialist juhib ka alusuuringute laboratooriumit, kus uuritakse veresoonte obstruktsioonide bioloogiat. Põhirõhk on kroonilisel trombemboolsel pulmonaalsel hüpertensioonil.
Finding out that one has a serious, rare, chronic disease can turn the world upside down.
Coping with this situation requires not only early diagnosis, medication and therapy. A compelx approach helps to cope with this challenge - this might involve physical exersicing, psychological hygiene, support of friends and family, participation in the activities of patient organsiations etc.
"I think this is a complex disease and therefore it requires complex approach. First of all it is important to have an early diagnosis and correct medication. Then it is also important to have regular adequate physical activity. Another important aspect is to take care of mental health since PAH is a chronic disease. Having work, connection to friends and family, following hobbies - this all is supportive in coping with this disease."
Prof Pavel Jansa
European Reference Networks are virtual networks involving healthcare providers across Europe. They aim to tackle complex or rare diseases and conditions that require highly specialised treatment and a concentration of knowledge and resources.
There are 24 ERNS involving 25 European countries included Norway, over 300 hospitals with over 900 healthcare units and covering all major disease groups!
"Creating database on international level is a positive trend. For example European Reference Network collects the data and helps to put it together from 17 PAH expert centers across Europe. It is essential approache for successful treatment and research."
Prof Pavel Jansa
PAH and Pregnancy
Despite advanced therapies, pregnancy is not recommended in patients with PAH diagnosis since this is a very big stress for the cardio-vascular system.
At the same time it is not a dogma and patients can be stratified in a low and high risks groups.
Video Interview Prof Jansa
Referring to the International PAH Awareness Day on May 5, 2020 we had a chance to talk with Prof Jansa, MD, PhD, Chief of the Centre for Pulmonary Hypertension at the Department of Cardiology and Angiology, General University Hospital in Prague about PAH, its diagnosis, everyday life and current COVID-19 situation.
There is a video embedded here from the social network Youtube/Vimeo. This content is currently blocked. There is no data exchange between your browser and the social network.
You have the option of permanently activating the blocked content by clicking on the "Allow external content" button below. The content will then be displayed and will continue to be displayed in the future. By doing so, you consent to the processing of your personal data, in particular this includes consenting to the transfer of your data to the USA (Article 49(1)(a) DSGVO). If you would like to know more about the processing of your data in this particular case and the risks associated with the transfer of your data to the USA, please read our Privacy Policy. You may also revoke your consent there at any time.
Prof Pavel Jansa, MD, PhD is Chief of the Centre for Pulmonary Hypertension at the Department of Cardiology and Angiology, General University Hospital in Prague, and Associate Professor at the First Faculty of Medicine, Charles University, Prague. He is vice-chairman of the Working Group on Pulmonary Circulation of the Czech Society of Cardiology. His research interests include epidemiology of pulmonary arterial hypertension, coagulation disorders in pulmonary hypertension, and small-vessel disease in chronic thromboembolic pulmonary hypertension.