Yonke into ekufuneka uyazi malunga nePulmonary Arterial Hypertension

Umxholo

• Iimpawu ze-pulmonary arterial hypertension
• Oonobangela boxinzelelo lwegazi kwimiphunga
• Ukuchongwa koxinzelelo lwegazi kwimiphunga
• Unyango lwe-pulmonary arterial hypertension
• Amayeza
• Ugqirha
• Utshintsho kwindlela yokuphila
• Ukulindela ubomi kunye ne-pulmonary arterial hypertension
• Amanqanaba oxinzelelo lwegazi kwimiphunga
• Ezinye iintlobo zoxinzelelo lwegazi kwimiphunga
• Ukuxela kwangaphambili koxinzelelo lwegazi kwimiphunga
• Uxinzelelo lwegazi lwe-pulmonary kwiintsana ezisandul 'ukuzalwa
• Izikhokelo ze-pulmonary arterial hypertension
• Q:
• A:

Yintoni uxinzelelo lwegazi olusisiseko?

Uxinzelelo lwegazi lwe-pulmonary arterial (PAH), eyayisaziwa njenge-pulmonary hypertension, luhlobo olunqabileyo loxinzelelo lwegazi. Ichaphazela imithambo yakho yemiphunga kunye nee-capillaries. Le mijelo yegazi ithwala igazi lisuka kwigumbi lasekunene lentliziyo yakho (i-ventricle yasekunene) iye kwimiphunga yakho.

Njengoko uxinzelelo kwimithambo yakho yemiphunga kunye nemithambo yegazi encinci isakha, intliziyo yakho kufuneka isebenze nzima ukupompa igazi kwimiphunga yakho. Ixesha elingaphezulu, oku kuyenza buthathaka imisipha yentliziyo yakho. Ekugqibeleni, inokukhokelela kukusilela kwentliziyo kunye nokufa.

Akukho lunyango lwe-PAH, kodwa iindlela zokhetho ziyafumaneka. Ukuba unayo i-PAH, unyango lunokunceda ekunciphiseni iimpawu zakho, ukunciphisa amathuba akho okuba neengxaki, kunye nokwandisa ubomi bakho.

Iimpawu ze-pulmonary arterial hypertension

Kumanqanaba okuqala e-PAH, usenokungabinazo iimpawu ezibonakalayo. Njengoko imeko iya isiba mandundu, iimpawu ziya kubonakala ngakumbi. Iimpawu eziqhelekileyo zibandakanya:

• ubunzima bokuphefumla
• ukudinwa
• isiyezi
• isiqaqa
• uxinzelelo lwesifuba
• iintlungu zesifuba
• ukubetha ngokukhawuleza
• ukubetha kwentliziyo
• Imibala eluhlaza okwesibhakabhaka kwimilebe yakho okanye ulusu
• ukudumba kwamaqatha okanye imilenze
• ukudumba ngamanzi ngaphakathi kwisisu sakho, ngakumbi kumanqanaba okugqibela emeko

Unokukufumanisa kunzima ukuphefumla ngexesha lokuzilolonga okanye ezinye iintlobo zomsebenzi womzimba. Ekugqibeleni, ukuphefumla kunokuba nzima ngexesha lokuphumla, nako. Fumana ukuba ungazibona njani iimpawu zePAH.

Oonobangela boxinzelelo lwegazi kwimiphunga

I-PAH ikhula xa imithambo yemiphunga kunye nemithanjana yegazi ethwala igazi lisuka entliziyweni yakho lisiya kwimiphunga yakho lincitshisiwe okanye latshatyalaliswa. Oku kucingelwa ukuba kubangelwa ziimeko ezahlukeneyo ezinxulumene noko, kodwa esona sizathu sokuba kutheni i-PAH isenzeka asaziwa.

Malunga neepesenti ezili-15 ukuya kwezingama-20 zamatyala, i-PAH izuzwa njengelifa, ngokwe-National Organisation for Rare Disorders (NORD). Oku kubandakanya ukuguquka kwemfuza okunokwenzeka kwi IBMPR2 zofuzo okanye ezinye izakhi zofuzo. Utshintsho emva koko lunokudluliselwa kwiintsapho, luvumela umntu onolu tshintsho ukuba abe nakho ukuphucula kamva i-PAH.

Ezinye iimeko ezinokubakho ezinokunxulunyaniswa nokuphuhlisa i-PAH kubandakanya:

• isifo sesibindi esinganyangekiyo
• isifo sentliziyo esibelekwe
• ukuphazamiseka kwezicubu ezithile
• usulelo oluthile, njengosulelo lwe-HIV okanye i-schistosomiasis
• iityhefu ezithile okanye iziyobisi, kubandakanya iziyobisi ezithile zokuzonwabisa (i-methamphetamines) okanye okwangoku ngaphandle kwentengiso

Ngamanye amaxesha, i-PAH ikhula ngaphandle kwesizathu esaziwayo. Oku kwaziwa njenge-idiopathic PAH. Fumanisa ukuba i-idiopathic PAH ifunyaniswa njani kwaye inyangwa njani.

Ukuchongwa koxinzelelo lwegazi kwimiphunga

Ukuba ugqirha wakho ukrokrela ukuba unayo i-PAH, baya kuthi ba-odole uvavanyo olunye okanye nangaphezulu ukuvavanya imithambo yakho yentliziyo kunye nentliziyo.

Uvavanyo lokufumanisa i-PAH lunokubandakanya:

• i-electrocardiogram ukukhangela iimpawu zoxinzelelo okanye izingqisho ezingaqhelekanga entliziyweni yakho
• i-echocardiogram ukuvavanya ubume kunye nokusebenza kwentliziyo yakho kunye nokulinganisa uxinzelelo lwe-pulmonary artery
• X-ray yesifuba ukuze ufunde ukuba imithambo yakho yemiphunga okanye igumbi lasekunene lentliziyo yakho landisiwe
• Ukuskena i-CT okanye ukuskena i-MRI ukukhangela amahlwili egazi, ukunciphisa, okanye ukonakala kwimithambo yakho yemiphunga
• catheterization yentliziyo elungileyo ukulinganisa uxinzelelo lwegazi kwimithambo yakho yemiphunga kunye ne-ventricle elungileyo yentliziyo yakho
• Uvavanyo lomsebenzi wepulmonary ukuvavanya amandla kunye nokuhamba komoya kungena nangaphandle kwemiphunga yakho
• Uvavanyo lwegazi ukukhangela izinto ezinxulumene ne-PAH okanye ezinye iimeko zempilo

Ugqirha wakho unokusebenzisa olu vavanyo ukujonga iimpawu ze-PAH, kunye nezinye izinto ezinokubangela iimpawu zakho. Baza kuzama ukulawula ezinye izizathu ezinokubakho ngaphambi kokufumanisa i-PAH. Fumana ulwazi oluthe kratya malunga nale nkqubo.

Unyango lwe-pulmonary arterial hypertension

Okwangoku, akukho nonyango eyaziwayo ye-PAH, kodwa unyango lunokunciphisa iimpawu, ukunciphisa umngcipheko weengxaki, kunye nokwandisa ubomi.

Amayeza

Ukukunceda ukulawula imeko yakho, ugqirha unokumisela iyeza elinye okanye nangaphezulu:

• Unyango lweprostacyclin ukuze unciphise imithambo yakho yegazi
• Isinyibilikisi se-guanylate esinyibilikayo sokunyusa imithambo yakho yegazi
• i-endothelin receptor antagonists ukuvimba umsebenzi we-endothelin, into enokubangela ukunciphisa imithambo yegazi
• ii-anticoagulants zokuthintela ukwenziwa kwamahlwili egazi

Ukuba i-PAH inxulumene nenye imeko yezempilo kwimeko yakho, ugqirha wakho unokumisela amanye amayeza ukunceda ukunyanga loo meko. Banokuhlengahlengisa nawaphi na amayeza owathathayo ngoku. Fumana ngakumbi ngamachiza anokuyalelwa ngugqirha wakho.

Ugqirha

Kuxhomekeka ekubeni imeko yakho imbi kangakanani, ugqirha wakho unokucebisa unyango. I-septostomy ye-Atrial ingenziwa ukunciphisa uxinzelelo kwicala lasekunene lentliziyo yakho, kwaye ukufakelwa kwemiphunga okanye intliziyo kunye nemiphunga kunokuthatha indawo yomzimba owonakeleyo.

Kwi-septostomy ye-atrial, ugqirha wakho uya kukhokela i-catheter ngenye yeemvini zakho eziphambili kwigumbi eliphezulu lasekunene lentliziyo yakho. Kwigumbi eliphezulu le-septum (umcu wethishu phakathi kwamacala asekhohlo nasekhohlo entliziyweni), ohamba ukusuka ekunene uye ekamelweni eliphezulu lasekhohlo, baya kwenza ukuvulwa. Emva koko, baya kufaka ibhaluni encinci kwincam yecatheter ukuze banciphise ukuvulwa kwaye babangele ukuba igazi likwazi ukuhamba phakathi kwamagumbi aphezulu entliziyo yakho, ukunciphisa uxinzelelo kwicala lasekunene lentliziyo yakho.

Ukuba unetyala elibi le-PAH elihambelana nesifo esibi semiphunga, ukufakelwa kwemiphunga kunokucetyiswa. Kule nkqubo, ugqirha wakho uyakususa enye okanye zombini imiphunga yakho kwaye ayitshintshe ngemiphunga evela kumnikeli welungu.

Ukuba unesifo sentliziyo esomeleleyo okanye ukusilela kwentliziyo, ugqirha wakho unokucebisa ukufakelwa kwentliziyo ukongeza ukufakelwa kwemiphunga.

Utshintsho kwindlela yokuphila

Ukutshintsha indlela yokuphila ukuze uhlengahlengise indlela otya ngayo, ukuzilolonga rhoqo, okanye eminye imikhwa yemihla ngemihla kunokunceda ukunciphisa umngcipheko weengxaki ze-PAH. Oku kubandakanya:

• ukutya ukutya okusempilweni
• ukuzilolonga rhoqo
• ukunciphisa umzimba okanye ukugcina ubunzima obunempilo
• ukuyeka ukutshaya

Ukulandela isicwangciso sonyango esinconyelwe ngugqirha kunokunceda ukunciphisa iimpawu zakho, ukunciphisa umngcipheko weengxaki, kunye nokwandisa ubomi bakho. Funda ngakumbi ngeendlela zokhetho ze-PAH.

Ukulindela ubomi kunye ne-pulmonary arterial hypertension

I-PAH yimeko eqhubekayo, oko kuthetha ukuba iya isiba mandundu ngokuhamba kwexesha. Abanye abantu banokubona iimpawu ziba mandundu ngokukhawuleza kunabanye.

Uphononongo luka-2015 olupapashwe kuvavanyo lweminyaka emihlanu yokuphila kwabantu abanamanqanaba ahlukeneyo e-PAH kwaye bafumanisa ukuba njengoko imeko iqhubeka, izinga lokusinda kweminyaka emihlanu liyancipha.

Nantsi iminyaka emihlanu yokuphila kwabaphandi abafunyenweyo kwinqanaba ngalinye.

• Iklasi 1: 72 ukuya kwiipesenti ezingama-88
• Iklasi 2: 72 ukuya kwiipesenti ezingama-76
• Iklasi 3: 57 ukuya kwi-60 yepesenti
• Iklasi 4: 27 ukuya kwiipesenti ezingama-44

Ngelixa lungekho unyango, ukuhambela phambili kunyango kuye kwanceda ukuphucula imeko yabantu abane-PAH. Funda kabanzi malunga namazinga okusinda kubantu abane-PAH.

Amanqanaba oxinzelelo lwegazi kwimiphunga

I-PAH yahlulwe yangamanqanaba amane ngokusekwe kubukhali beempawu.

Ngokweekhrayitheriya esekwe nguMbutho wezeMpilo weHlabathi (i-WHO), i-PAH ihlelwa ngokwamanqanaba amane okusebenza:

• Iklasi 1. Imeko ayikuthinteli ukusebenza komzimba. Awunazo naziphi na iimpawu ezibonakalayo ngexesha lomsebenzi oqhelekileyo womzimba okanye ukuphumla.
• Iklasi 2. Imeko inciphisa kancinci ukusebenza kwakho komzimba. Ufumana iimpawu ezibonakalayo ngexesha lomsebenzi oqhelekileyo womzimba, kodwa hayi ngamaxesha okuphumla.
• Iklasi 3. Imeko inciphisa ngokubonakalayo imisebenzi yakho yomzimba. Uba neempawu ngexesha lokuzilolonga emzimbeni kunye nokusebenza komzimba, kodwa hayi ngamaxesha okuphumla.
• Iklasi 4. Awunakho ukwenza naluphi na uhlobo lomsebenzi womzimba ngaphandle kweempawu. Uba neempawu ezibonakalayo, nkqu nangamaxesha okuphumla. Iimpawu zokusilela kwentliziyo esekunene zihlala zikhona kweli nqanaba.

Ukuba unayo i-PAH, inqanaba lemeko yakho liya kuchaphazela indlela yakho yonyango ekhuthazwayo. Fumana ulwazi oludingayo ukuze uqonde ukuba le meko iqhubeka njani.

Ezinye iintlobo zoxinzelelo lwegazi kwimiphunga

I-PAH yenye yeentlobo ezintlanu zoxinzelelo lwegazi kwimiphunga (PH). Kukwaziwa njengeQela 1 i-PAH.

Ezinye iintlobo ze-PH zibandakanya:

• Iqela lesi-2 PH, elidityaniswa neemeko ezithile ezibandakanya icala lasekhohlo lentliziyo yakho
• Iqela le-3 PH, elinxulunyaniswa neemeko ezithile zokuphefumla emiphungeni
• Iqela le-4 PH, elinokubangelwa kukugungxwa kwegazi okungapheliyo kwimithambo yemiphunga yakho
• Iqela lesi-5 le-PH, elinokubangela iimeko ezahlukeneyo zempilo

Ezinye iintlobo ze-PH ziyanyangeka kunezinye. Thatha ixesha ufunde okungakumbi malunga neentlobo ezahlukeneyo ze-PH.

Ukuxela kwangaphambili koxinzelelo lwegazi kwimiphunga

Kwiminyaka yakutshanje, ukhetho lwezonyango luphuculwe kubantu abane-PAH. Kodwa akukabikho nyango kule meko.

Ukuxilongwa kwangoko kunye nonyango kunokunceda ekunciphiseni iimpawu zakho, ukunciphisa umngcipheko weengxaki, kunye nokwandisa ubomi bakho nge-PAH. Funda ngakumbi malunga nemiphumo unyango olunokuba nayo kwimbonakalo yakho nesi sifo.

Uxinzelelo lwegazi lwe-pulmonary kwiintsana ezisandul 'ukuzalwa

Kwiimeko ezinqabileyo, i-PAH ichaphazela iintsana ezisandul 'ukuzalwa. Oku kwaziwa njengokuqhubeka koxinzelelo lwegazi kwimiphunga yosana olusandul 'ukuzalwa (PPHN). Kwenzeka xa imithambo yegazi eya kwimiphunga yomntwana ingavuleki kakuhle emva kokuzalwa.

Imiba yomngcipheko kwi-PPHN ibandakanya:

• usulelo lomntwana
• uxinzelelo olukhulu ngexesha lokuhanjiswa
• iingxaki zemiphunga, ezinje ngemiphunga engaphuhliswanga okanye isifo sokuphefumla

Ukuba umntwana wakho ufunyaniswe ene-PPHN, ugqirha wabo uya kuzama ukunciphisa imithambo yegazi kwimiphunga yabo ngeoksijini eyongezelelweyo. Ugqirha kusenokufuneka asebenzise isixhobo sokuphefumla ukuze axhase ukuphefumla komntwana wakho.

Unyango olufanelekileyo nolufike ngexesha lunokunceda ukunciphisa umngcipheko womntwana wakho kulibaziseko lokukhula kunye nokukhubazeka okusebenzayo, ukunceda ukuphucula amathuba okusinda.

Izikhokelo ze-pulmonary arterial hypertension

Ngo-2014, i-American College of Chest Physicians yakhupha unyango lwe-PAH. Ukongeza kwezinye iingcebiso, ezi zikhokelo zicebisa ukuba:

• Abantu abasemngciphekweni wokuphuhliswa kwe-PAH kunye nabo baneklasi 1 ye-PAH kufuneka babekwe iliso kuphuhliso lweempawu ezinokufuna unyango.
• Xa kunokwenzeka, abantu abane-PAH kufuneka bavavanywe kwiziko lonyango elinobuchwephesha bokuchonga i-PAH, ngokufanelekileyo ngaphambi kokuqala unyango.
• Abantu abane-PAH kufuneka banyangwe naziphi na iimeko zempilo ezinokuthi zibe negalelo kwesi sifo.
• Abantu abane-PAH kufuneka bagonywe umkhuhlane kunye ne-pneumococcal pneumonia.
• Abantu abane-PAH kufuneka bakuphephe ukukhulelwa. Ukuba bayakhulelwa, kuya kufuneka bakhathalelwe liqela lezempilo elinamacandelo ngamacandelo eliquka iingcali ezinobungcali kuxinzelelo lwegazi kwimiphunga.
• Abantu abane-PAH kufuneka baluphephe utyando olungeyomfuneko. Ukuba kufuneka benziwe utyando, kuya kufuneka bakhathalelwe liqela lezempilo elinamacandelo ngamacandelo eliquka iingcali ezinobungcali kuxinzelelo lwegazi kwimiphunga.
• Abantu abane-PAH kufuneka bakuphephe ukuvezwa kukuphakama okuphezulu, kubandakanya ukuhamba komoya. Ukuba kufuneka babonakaliswe kwiindawo eziphakamileyo, kufuneka basebenzise ioksijini eyongezelelweyo njengoko kufuneka.

Ezi zikhokelo zibonelela ngesishwankathelo sendlela yokukhathalela abantu abane-PAH. Unyango lwakho lomntu ngamnye luya kuxhomekeka kwimbali yakho yonyango kunye neempawu ohlangabezana nazo.

Q:

Ngaba akhona amanyathelo umntu anokuwathatha ukukhusela ukuphuhlisa i-PAH?

Isigulana esingaziwayo

A:

Uxinzelelo lwegazi lwe-pulmonary arterial alunakuhlala luthintelwa. Nangona kunjalo, ezinye iimeko ezinokukhokelela kwi-PAH zinokuthintelwa okanye zilawulwe ukunciphisa umngcipheko wokuba ne-PAH. Ezi meko zibandakanya isifo semithambo yegazi, uxinzelelo lwegazi, isifo sesibindi esinganyangekiyo (esihlala sinxulumene nesibindi esinamafutha, utywala, kunye ne-hepatitis yentsholongwane), i-HIV kunye nesifo esinganyangekiyo semiphunga, ngakumbi esihambelana nokutshaya kunye nokuvezwa kwendalo.

UGraham Rogers, iimpendulo ze-MDA zimele izimvo zeengcali zethu zonyango. Yonke imixholo inolwazi ngokungqongqo kwaye akufuneki ukuba ithathelwe ingqalelo njengengcebiso yezonyango.