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Kuyini umfutho wegazi ophakeme we-arterial?

I-pulmonary arterial hypertension (PAH), eyayaziwa ngokuthi i-primary pulmonary hypertension, uhlobo olungajwayelekile lomfutho wegazi ophezulu. Ithinta imithambo yakho ye-pulmonary kanye nama-capillaries. Le mithambo yegazi ithwala igazi lisuka egumbini elingezansi kwesokudla lenhliziyo yakho (i-ventricle yangakwesokudla) liye emaphashini akho.

Njengoba ingcindezi esemithanjeni yakho yamaphaphu nemithambo yegazi emincane yakhula, inhliziyo yakho kufanele isebenze kanzima ukupompa igazi emaphashini akho. Ngokuhamba kwesikhathi, lokhu kunciphisa imisipha yenhliziyo yakho. Ekugcineni, kungaholela ekuhlulekeni kwenhliziyo nasekufeni.

Alikho ikhambi elaziwayo le-PAH, kepha izindlela zokwelashwa ziyatholakala. Uma une-PAH, ukwelashwa kungasiza ekunciphiseni izimpawu zakho, kwehlise amathuba akho ezinkinga, futhi kwandise impilo yakho.

Izimpawu ze-pulmonary arterial hypertension

Ezigabeni zokuqala ze-PAH, ungahle ungabi nazimpawu ezibonakalayo. Njengoba isimo siba sibi kakhulu, izimpawu zizobonakala kakhulu. Izimpawu ezijwayelekile zifaka:

  • ukuphefumula kanzima
  • ukukhathala
  • isiyezi
  • ukuquleka
  • ingcindezi yesifuba
  • ubuhlungu besifuba
  • ishayela ngokushesha
  • ukushaya kwenhliziyo
  • umbala obala okwesibhakabhaka ezindebeni zakho noma esikhunjeni
  • ukuvuvukala kwamaqakala noma imilenze yakho
  • ukuvuvukala noketshezi ngaphakathi kwesisu sakho, ikakhulukazi ezigabeni zakamuva zesimo

Ungakuthola kunzima ukuphefumula ngesikhathi sokuzivocavoca noma ezinye izinhlobo zomsebenzi womzimba. Ekugcineni, ukuphefumula kungaba nzima ngezikhathi zokuphumula, futhi. Thola ukuthi ungazibona kanjani izimpawu ze-PAH.


Izimbangela ze-pulmonary arterial hypertension

I-PAH iba lapho imithambo yegazi yamaphaphu kanye nama-capillaries athwala igazi lisuka enhliziyweni yakho liye emaphashini akho kucinana noma kubhujiswa. Lokhu kucatshangwa ukuthi kudalwa yizimo ezahlukahlukene ezihlobene, kepha imbangela ngqo yokuthi kungani i-PAH yenzeka ayaziwa.

Cishe kumaphesenti angama-15 kuya kwangama-20 amacala, i-PAH izuzwa njengefa, ngokusho kweNational Organisation for Rare Disorders (NORD). Lokhu kufaka ukuguquka kwezakhi zofuzo okungenzeka kufayela le- BMPR2 zofuzo noma ezinye izakhi zofuzo. Izinguquko zingadluliselwa emindenini, zivumele umuntu onolunye lwalezi zinguquko abe namandla okuthuthuka kamuva kwe-PAH.

Ezinye izimo ezingaba khona ezingahlotshaniswa nokwakha i-PAH zifaka:

  • isifo esingalapheki sesibindi
  • isifo senhliziyo esizalwa naso
  • ukuphazamiseka kwezicubu ezithile
  • izifo ezithile, njengokutheleleka nge-HIV noma i-schistosomiasis
  • ubuthi obuthile noma izidakamizwa, kufaka phakathi izidakamizwa ezithile zokuzithokozisa (i-methamphetamines) noma njengamanje imakethe yokunciphisa ukudla

Kwezinye izimo, i-PAH iqala ngaphandle kwesizathu esaziwayo esihlobene. Lokhu kwaziwa njenge-idiopathic PAH. Thola ukuthi i-idiopathic PAH itholakala kanjani futhi yelashwa kanjani.


Ukuxilongwa komfutho wegazi ophezulu wegazi

Uma udokotela wakho esola ukuthi ungaba ne-PAH, kungenzeka ukuthi ba-oda ukuhlolwa okulodwa noma ngaphezulu ukuze kuhlolwe imithambo yenhliziyo yakho yenhliziyo.

Ukuhlolwa kokuthola i-PAH kungafaka:

  • i-electrocardiogram ukuhlola izimpawu zohlobo noma isigqi esingajwayelekile enhliziyweni yakho
  • i-echocardiogram ukuhlola ukwakheka nokusebenza kwenhliziyo yakho nokukala ukucindezela komthambo wamaphaphu
  • i-X-ray yesifuba ukuze ufunde ukuthi imithambo yakho yamaphaphu noma ikamelo elingezansi kwesokudla lenhliziyo yakho likhulisiwe
  • Ukuskena kwe-CT noma ukuskena kwe-MRI ukubheka amahlule egazi, ukuncipha, noma ukulimala emithanjeni yakho yamaphaphu
  • i-catheterization yenhliziyo engakwesokudla ukukala umfutho wegazi emithanjeni yakho yamaphaphu kanye ne-ventricle efanele yenhliziyo yakho
  • ukuhlolwa komsebenzi wamaphaphu ukuhlola amandla nokuhamba komoya ungene noma uphume emaphashini akho
  • ukuhlolwa kwegazi ukuhlola izinto ezihlobene ne-PAH noma ezinye izimo zezempilo

Udokotela wakho angasebenzisa lezi zivivinyo ukuhlola izimpawu ze-PAH, kanye nezinye izimbangela zezimpawu zakho. Bazozama ukukhipha ezinye izimbangela ezingaba khona ngaphambi kokuthola i-PAH. Thola eminye imininingwane ngale nqubo.


Ukwelashwa komfutho wegazi ophezulu wegazi

Njengamanje, alikho ikhambi elaziwayo le-PAH, kepha ukwelashwa kunganciphisa izimpawu, kunciphise ubungozi bezinkinga, futhi kwandise impilo.

Imithi

Ukusiza ukuphatha isimo sakho, udokotela wakho angakunikeza umuthi owodwa noma eminingi elandelayo:

  • ukwelashwa kwe-prostacyclin ukukhulisa imithambo yakho yegazi
  • izinto ezincibilikisayo ze-guanylate cyclase zokuvuselela imithambo yegazi yakho
  • abaphikisi be-endothelin receptor ukuvimba umsebenzi we-endothelin, into engadala ukunciphisa imithambo yegazi yakho
  • ama-anticoagulants ukuvimbela ukwakheka kwamahlule egazi

Uma i-PAH ihlobene nesinye isimo sempilo kuwe, udokotela wakho angakunikeza eminye imithi yokusiza ukwelapha leso simo. Bangase futhi balungise noma yimiphi imithi oyiphuzayo njengamanje. Thola okuningi ngemithi udokotela wakho angakunikeza yona.

Ukuhlinzwa

Ngokuya ngesimo sakho ukuthi sinzima kangakanani, udokotela wakho angancoma ukwelashwa ngokuhlinzwa. I-septostomy ye-Atrial ingenziwa ukunciphisa ingcindezi ohlangothini lwesokunene senhliziyo yakho, futhi ukufakelwa kwamaphaphu noma kwenhliziyo namaphaphu kungathatha indawo yezitho noma izitho ezilimele.

Ku-septostomy ye-atrial, udokotela wakho kungenzeka aqondise i-catheter ngomunye wemithambo yakho emaphakathi egumbini elingaphezulu lesokudla senhliziyo yakho. Ekamelweni eliphezulu le-septum (isiqeshana sezicubu eziphakathi kwezinhlangothi ezingakwesokudla nangakwesobunxele senhliziyo), sidlula ukusuka kwesokudla siye kwesokunxele, bazokwakha imbobo. Okulandelayo, bazofaka ibhaluni elincane ekugcineni kwepayipi ukuze kunwebe imbobo futhi kubangele negazi likwazi ukugeleza phakathi kwamagumbi aphezulu enhliziyo yakho, likhulule ingcindezi ohlangothini lwesokunene senhliziyo yakho.

Uma unesimo esibi se-PAH esihlobene nesifo samaphaphu esibi, ukufakelwa kwamaphaphu kunganconywa. Kule nqubo, udokotela wakho uzosusa elilodwa noma womabili amaphaphu akho bese ewabeka ngamaphaphu avela kumnikeli wesitho.

Uma futhi unesifo esibi senhliziyo noma ukwehluleka kwenhliziyo, udokotela wakho angancoma ukufakelwa kwenhliziyo ngaphezu kokufakelwa kwamaphaphu.

Izinguquko zendlela yokuphila

Izinguquko zokuphila ukuze ulungise indlela odla ngayo, ukuzivocavoca umzimba, noma eminye imikhuba yansuku zonke kungasiza ekunciphiseni ubungozi bezinkinga ze-PAH. Lokhu kufaka phakathi:

  • ukudla ukudla okunempilo
  • ukuzivocavoca umzimba njalo
  • ukuncipha noma ukugcina isisindo esinempilo
  • ukuyeka ukubhema ugwayi

Ukulandela uhlelo lokwelashwa olunconyiwe lukadokotela wakho kungasiza ekunciphiseni izimpawu zakho, kunciphise ubungozi bezinkinga, futhi kwandise impilo yakho. Funda kabanzi mayelana nezinketho zokwelashwa ze-PAH.

Isikhathi sokuphila esine-pulmonary arterial hypertension

I-PAH yisimo esiqhubekayo, okusho ukuthi sibi kakhulu ngokuhamba kwesikhathi. Abanye abantu bangabona izimpawu ziba zimbi kakhulu kunabanye.

Ucwaningo lwango-2015 olushicilelwe luhlolisise amazinga okusinda eminyaka emihlanu kubantu abanezigaba ezehlukene ze-PAH futhi bathola ukuthi njengoba isimo siqhubeka, izinga lokusinda kweminyaka emihlanu liyancipha.

Naba abacwaningi bezinga lokusinda leminyaka emihlanu abathola isigaba ngasinye.

  • Isigaba 1: amaphesenti angama-72 kuya kwangama-88
  • Isigaba 2: amaphesenti angama-72 kuye kwangama-76
  • Isigaba 3: 57 kuya kuma-60 amaphesenti
  • Isigaba 4: amaphesenti angama-27 kuye kwangama-44

Yize lungekho ikhambi, intuthuko yakamuva ekwelashweni isize ukuthuthukisa ukubukeka kwabantu abane-PAH. Funda kabanzi ngamazinga okusinda abantu abane-PAH.

Izigaba ze-pulmonary arterial hypertension

I-PAH ihlukaniswe izigaba ezine ngokuya ngobukhulu bezimpawu.

Ngokwenqubo esungulwe yiWorld Health Organisation (WHO), i-PAH ihlukaniswa izigaba ezine ezisebenzayo:

  • Isigaba 1. Isimo asikhawuleli umsebenzi wakho womzimba.Awunazo izimpawu ezibonakalayo phakathi nezikhathi zomsebenzi ojwayelekile womzimba noma ukuphumula.
  • Isigaba 2. Isimo sikhawulela kancane ukusebenza komzimba wakho. Uhlangabezana nezimpawu ezibonakalayo ngesikhathi somsebenzi ojwayelekile womzimba, kepha hhayi ngezikhathi zokuphumula.
  • Isigaba 3. Isimo sikhawulela kakhulu ukusebenza kwakho ngokomzimba. Uhlangabezana nezimpawu ngesikhathi sokuzikhandla okuncane komzimba kanye nokusebenza okujwayelekile komzimba, kepha hhayi ngezikhathi zokuphumula.
  • Isigaba 4. Awukwazi ukwenza noma yiluphi uhlobo lomsebenzi womzimba ngaphandle kwezimpawu. Uhlangabezana nezimpawu ezibonakalayo, noma ngezikhathi zokuphumula. Izimpawu zokwehluleka kwenhliziyo ezisemaceleni zivame ukwenzeka kulesi sigaba.

Uma une-PAH, isigaba sesimo sakho sizothinta indlela yokwelashwa yakho enconyiwe. Thola imininingwane oyidingayo ukuze uqonde ukuthi lesi simo siqhubeka kanjani.

Ezinye izinhlobo zomfutho wegazi ophezulu wamaphaphu

I-PAH ingenye yezinhlobo ezinhlanu ze-pulmonary hypertension (PH). Kuyaziwa nangokuthi yi-Group 1 PAH.

Ezinye izinhlobo ze-PH zifaka:

  • Iqembu 2 PH, elixhunyaniswe nezimo ezithile ezibandakanya uhlangothi lwangakwesokunxele senhliziyo yakho
  • Iqembu 3 PH, elihlotshaniswa nezimo ezithile zokuphefumula emaphashini
  • Iqembu 4 PH, elingabangelwa amahlule egazi angapheli ezitsheni eziya emaphashini akho
  • Iqembu 5 PH, elingavela kwezinye izimo zezempilo ezahlukahlukene

Ezinye izinhlobo ze-PH ziyelapheka kunezinye. Thatha isikhashana ukuze ufunde kabanzi mayelana nezinhlobo ezahlukahlukene ze-PH.

Isibikezelo somfutho wegazi ophezulu we-pulmonary

Eminyakeni yamuva, izinketho zokwelashwa ziye zathuthuka kubantu abane-PAH. Kepha alikho ikhambi lalesi simo.

Ukuxilongwa nokwelashwa kusenesikhathi kungasiza ukukhulula kangcono izimpawu zakho, kunciphise ubungozi bezinkinga, futhi wandise impilo yakho nge-PAH. Funda kabanzi ngemiphumela ukwelashwa okungaba nayo kumbono wakho ngalesi sifo.

Umfutho wegazi ophezulu we-pulmonary ezinganeni ezisanda kuzalwa

Ezimweni ezingavamile, i-PAH ithinta izinsana ezisanda kuzalwa. Lokhu kwaziwa njengokuphikelela komfutho wegazi ophezulu wamaphaphu wosana olusanda kuzalwa (i-PPHN). Kwenzeka lapho imithambo yegazi eya emaphashini engane ingavuleki kahle ngemuva kokuzalwa.

Izici zobungozi be-PPHN zifaka:

  • ukutheleleka kwengane
  • ukucindezeleka okukhulu ngesikhathi sokubeletha
  • izinkinga zamaphaphu, njengamaphaphu angathuthuki kahle noma isifo sokuphefumula

Uma ingane yakho kutholakala ukuthi ine-PPHN, udokotela wayo uzozama ukukhulisa imithambo yegazi emaphashini abo nge-oxygen eyengeziwe. Udokotela futhi angadinga ukusebenzisa umshini wokuphefumula womshini ukusekela ukuphefumula kwengane yakho.

Ukwelashwa okufanele nangesikhathi esifanele kungasiza ekwehliseni ingozi yengane yakho yokubambezeleka kokukhula nokukhubazeka okusebenzayo, okusiza ekuthuthukiseni amathuba okusinda.

Imihlahlandlela ye-pulmonary arterial hypertension

Ngo-2014, i-American College of Chest Physicians yakhipha ukwelashwa kwe-PAH. Ngaphezu kwezinye izincomo, le mihlahlandlela yeluleka ukuthi:

  • Abantu abasengozini yokuthola i-PAH nalabo abane-Class 1 PAH kufanele babhekwe ukuze kuthuthukiswe izimpawu ezingadinga ukwelashwa.
  • Uma kunokwenzeka, abantu abane-PAH kufanele bahlolwe esikhungweni sezokwelapha esinolwazi lokuthola i-PAH, ngaphambi kokuthola ukwelashwa.
  • Abantu abane-PAH kufanele belashwe nganoma yiziphi izimo zezempilo ezingaba nomthelela kulesi sifo.
  • Abantu abane-PAH kufanele bagonyelwe umkhuhlane kanye ne-pneumococcal pneumonia.
  • Abantu abane-PAH kufanele bagweme ukukhulelwa. Uma kwenzeka bekhulelwa, kufanele bathole ukunakekelwa eqenjini lezempilo elenza imikhakha ehlukahlukene elibandakanya ongoti abanolwazi lomfutho wegazi ophezulu wamaphaphu.
  • Abantu abane-PAH kufanele bagweme ukuhlinzwa okungadingekile. Uma kufanele bahlinzwe, kufanele bathole ukunakekelwa yithimba lezempilo elenza imikhakha ehlukahlukene elibandakanya ongoti abanolwazi lomfutho wegazi ophezulu wamaphaphu.
  • Abantu abane-PAH kufanele bagweme ukuchayeka ezindaweni eziphakeme kakhulu, kufaka phakathi ukuhamba emoyeni. Uma kufanele badalulwe ezindaweni eziphakeme kakhulu, kufanele basebenzise i-oxygen eyengeziwe lapho kudingeka.

Le mihlahlandlela inikeza uhlaka olujwayelekile lokuthi ungabanakekela kanjani abantu abane-PAH. Ukwelashwa kwakho ngakunye kuzoya ngomlando wakho wezokwelapha kanye nezimpawu ohlangabezana nazo.

Q:

Ngabe kukhona izinyathelo umuntu angazithatha ukuvimbela ukuthuthukisa i-PAH?

Isiguli esingaziwa

A:

Umfutho wegazi ophakeme wamapulmonary awukwazi ukuvinjelwa njalo. Kodwa-ke, ezinye izimo ezingaholela ku-PAH zingavinjelwa noma zilawulwe ukunciphisa ubungozi bokuba ne-PAH. Lezi zimo zibandakanya isifo semithambo yegazi, umfutho wegazi ophakeme, isifo esingalapheki sesibindi (esivame ukuhlobene nesibindi esinamafutha, utshwala, nesifo sokusha kwesibindi), i-HIV nesifo samaphaphu esingapheli, ikakhulukazi esihlobene nokubhema nokuchayeka kwemvelo.

UGraham Rogers, izimpendulo ze-MDA zimele imibono yochwepheshe bethu bezokwelapha. Konke okuqukethwe kunolwazi oluqinile futhi akufanele kuthathwe njengezeluleko zezokwelapha.

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