Ngaba unengxaki ngemithambo yakho yegazi? Masithethe nge-GPA (i-Granulomatosis ene-Polyangiitis)!

Ngaba ngamanye amaxesha uziva ngathi umzimba wakho unzima kakhulu, unentlungu kwiindawo ezithile ngaphandle kwesizathu, okanye uhlala uziva ngathi unomkhuhlane, kodwa awufani nomkhuhlane oqhelekileyo? Ngamanye amaxesha, emva kwezi zinto, kunokubakho isifo esingava nto ingako ngaso, kodwa kubalulekile ukwazi. Leyo yenye yezo meko zibizwa ngokuba yi-GPA.

Yintoni iGPA (iGranulomatosis enePolyangiitis)? Masiyiqonde ngokulula!

Kulungile, ngoku masibone ukuba yintoni le GPA (iGranulomatosis enePolyangiitis). Ungakhathazeki, ndiza kuyichaza ngokulula. Yayibizwa ngokuba yi-`(Wegener's Granulomatosis)`. Kodwa igama elithi `(iGranulomatosis enePolyangiitis)` elisetyenziswayo ngoku lithetha lukhulu ngesi sifo.

Ngamafutshane, i-GPA kukudumba okanye ukudumba kwemithambo yegazi yakho. Oogqirha bayibiza le nto ngokuthi "i-vasculitis." Cinga ngale ndlela: imithambo yegazi kuyo yonke imizimba yethu ifana nemibhobho yamanzi. Xa kukho into engahambi kakuhle kule mibhobho, ukuhamba kwegazi kuyaphazamiseka, akunjalo? Yiloo nto eyenzekayo apha.

Ngoku kukho ezinye izinto ezimbini ezikhethekileyo ngale GPA:

• I-Polyangiitis: Oku kuthetha ukuba ukudumba kwenzeka kwimithambo yegazi eyahlukeneyo emzimbeni wakho, ingakumbi emincinci . Akupheleli nje kwindawo enye.
• I-Granulomatosis: Oku kunokuvakala ngathi ligama elintsonkothileyo, kodwa kuthetha nje ukuba amaqhuqhuva amancinci, anamaqhuqhuva, avuthayo abizwa ngokuba yi-granulomas ayenzeka kwimithambo yegazi yakho nakwamanye amalungu omzimba. La maqhuqhuva anokonakalisa imithambo yegazi kunye namalungu omzimba wakho.

Ngoko ke, kukho iintlobo ezininzi zale meko "ye-vasculitis", kodwa uhlobo olubizwa ngokuba yi-GPA luchaphazela kakhulu imithambo yegazi emincinci emzimbeni wethu. Ngenxa yokuba le mithambo yegazi emincinci ijikeleze umzimba wonke, i-GPA inokubangela iingxaki kwiindawo ezininzi zomzimba, ingakumbi amalungu anemithambo yegazi emininzi emincinci. Rhoqo, ichaphazela inkqubo yakho yokuphefumla (inkqubo yokuphefumla) kunye nezintso.

Oku kuvutha kwemithambo yegazi kunokubangela ukuba idumbe, iqhume, kwaye ide yophe. Kwakhona, imithambo yegazi inokuncitshiswa ngenxa yamanxeba. Kwenzeka ntoni ke ngoko? Izicubu zethu azifumani ioksijini kunye nezondlo ezizidingayo. Ngamanye amaxesha, i-GPA inokubangela imeko ebizwa ngokuba yi-"pulmonary-renal syndrome", apho igazi liphuma kwizintso nasemiphungeni.

Ziziphi iimpawu ezibonakala xa i-GPA ikhula?

Ngoku mhlawumbi ucinga, 'Kulungile, ukuba esi sisifo esifana nesi, singasibona njani? Zithini iimpawu?' Makhe sithethe ngaloo nto.

Njengoko bekutshiwo ngaphambili, i-GPA idla ngokuchaphazela inkqubo yokuphefumla kunye nezintso. Nangona kunjalo, inokuchaphazela nezinye izitho ezininzi kunye nezicubu, kunye nempilo-ntle yakho ngokubanzi. Oku kungenxa yokuba ijolise kwimithambo yegazi emincinci emzimbeni wonke.

Ngamanye amaxesha, iintlungu zemisipha kunye nentlungu yamalungu (i-arthritis) zinokubangelwa yi-vasculitis kwizicubu zakho ezidibeneyo.Kunye nezinto ezinje, ungaziva ungonwabanga . Kwakhona, ukudinwa rhoqo, ukungabi namdla wokutya, kunye nokwehla kobunzima ziimpawu eziqhelekileyo.

Iimpawu zenkqubo yokuphefumla

Abantu abaninzi baqala ngokurhanela ukuba bane-GPA baze bafune unyango ngenxa yeempawu zokuphefumla. Kwinqanaba lokuqala, ezi mpawu zinokufana nomkhuhlane oqhelekileyo, umkhuhlane, okanye umkhuhlane. Nangona kunjalo, zihlala ixesha elide kunesiqhelo. Jonga ukuba unayo na kwezi:

• Intloko ebuhlungu okanye uxinzelelo oluqatha kwindawo ye-sinus.
• Usulelo lweSinus luhlala lusenzeka.
• Kukho impumlo evuzayo rhoqo, kwaye kwakheka amaxolo ajikeleze iimpumlo.
• Impumlo yam ivakala ngathi ixinene.
• Ukopha rhoqo impumlo ngaphandle kwesizathu.
• Ndibuhlungu indlebe.
• Ndiva intlungu esifubeni sam.
• Ndikhohlela rhoqo.
• Ukuziva uphelelwa ngumphefumlo (njengokukhohlela).

Njengoko i-GPA iba mandundu ngakumbi, usenokuba nokuvuvukala (i-polychondritis) kwe-cartilage ezindlebeni zakho, empumlweni, nasemqaleni. Oku kunokubangela iimpawu ezinzulu ngakumbi, ezinje:

• Impumlo idumba kakhulu kangangokuba umphezulu wempumlo ungena ngaphakathi (oku kukwabizwa ngokuba yimpumlo yesali).
• Umqala, okanye inxalenye ephezulu yomphimbo womoya, uyancitshiswa (subglottic stenosis). Oku kunokubangela ilizwi elirhabaxa kunye nesandi somlozi xa uphefumla (ukubetha umoya okanye i-stridor).
• Ukudumba kwendlebe yangaphakathi kunokubangela isiyezi (vertigo) kunye nokulahlekelwa kukuva.

Kwakhona, usenokuba uphuma igazi emiphungeni yakho. Ukuba oku kuyenzeka, usenokuba nobunzima bokuphefumla okanye ukukhohlela igazi .

Iimpawu ezinxulumene nezintso

Usenokungaziva ntlungu kwizintso zakho xa une-GPA. Nangona kunjalo, unokubona utshintsho kumchamo wakho. Umzekelo, umchamo onamagwebu okanye igazi kumchamo wakho. Kwakhona, ukudumba kobuso okanye imilenze (i-edema) lolunye uphawu lwesifo sezintso.

Ukuba une-GPA, oogqirha baya kuzihlola izintso zakho rhoqo, nokuba awuzange ube nazimpawu. Oku kungenxa yokuba i-GPA isenokungazichaphazeli izintso zakho kwizigaba zokuqala, kodwa ekuhambeni kwexesha, ngokuqinisekileyo iya kuzichaphazela izintso zakho.

Iimpawu ezinxulumene namehlo

I-GPA inokuchaphazela namehlo akho. Ingabangela ukudumba kumphezulu okanye ngaphakathi kweliso. Oku kunokubangela iintlungu zamehlo, uxinzelelo, ukubomvu, kunye nokudumba . Kwiimeko ezinzima, ukulahleka kokubona kunokwenzeka.

Iimpawu zolusu

I-Vasculitis kulusu lwakho inokubangela iimpawu ezifana nezi:

• Umphezulu wolusu ujongeka njengomfanekiso oluhlaza okwesibhakabhaka-obomvu (ulusu olunamabala).
• Kuvela amabala amfusa, abomvu, okanye amdaka (i-purpura).
• Amaqhuma aqinileyo (iiqhuma) okanye amaqhuma amancinci afana namaqhuma (ii-papules) anokuvela eluswini.
• Kunokwenzeka ukuba kubekho amanxeba angapholiyo (izilonda).

Iimpawu zenkqubo yemithambo-luvo

I-GPA inokuchaphazela nenkqubo yakho yemithambo-luvo. Ngokuqhelekileyo, ichaphazela inkqubo yakho yemithambo-luvo ejikeleze umqolo wakho - imithambo-luvo ehamba ukusuka kumqolo wakho iye kwezinye iindawo zomzimba wakho.

Ukudumba kunye nokudumba kunokonakalisa umthambo omnye kuphela, okubangela iimpawu kwindawo enye (i-mononeuropathy). Nangona kunjalo, rhoqo, imithambo emininzi iyachaphazeleka (i-polyneuropathy). Iimpawu zingabandakanya:

• Iimpawu zentshukumo yomzimba: Njengobuthathaka bemisipha.
• Iimpawu zeemvakalelo: ukungaziva, ukurhawuzelelwa, okanye iintlungu zemithambo-luvo.
• Ukungasebenzi kakuhle kwenkqubo yemithambo-luvo elawula uxinzelelo lwegazi, ukusebenza kwesinyi, okanye ukubila.

Ziziphi iingxaki ezinkulu ezinokubakho ngenxa ye-GPA?

Ukuba i-GPA ayilawulwa kakuhle, ngamanye amaxesha inokuba yingozi ebomini kwaye ibangele umonakalo omkhulu kumalungu omzimba. Ngoko ke kubalulekile ukuqaphela oku. Nazi ezinye zeengxaki ezinkulu ezinokubakho:

• Ukopha kwegazi kwimiphunga: Oku kunokubangela ubunzima bokuphefumla kunye nokungaphumeleli kokuphefumla.
• I-glomerulonephritis ekhula ngokukhawuleza: Oku kunokukhokelela ekungasebenzini kwezintso.
• Ukulahlekelwa ngumbono okanye ukuva okungapheliyo.
• Ukulahlekelwa lusu okanye ukungakwazi ukulawula izihlunu (i-peripheral neuropathy).

Yintoni ebangela ukuba i-GPA iphuhliswe?

Ngoku usenokuba uzibuza, 'Kutheni olu hlobo lwesifo lusenzeka?' Enyanisweni, i-GPA, njengezinye iintlobo ze-'(vasculitis)', yi-'(autoimmune disease' ). Oku kungabonakala kunzima ukukuqonda, kodwa ndiza kukuchaza ngokulula.

Ngokwesiqhelo, inkqubo yethu yokuzikhusela komzimba ifana nomkhuseli wethu. Ilwa nezifo kunye neentsholongwane ngokubangela ukudumba. Nangona kunjalo, kwezi zifo zokuzikhusela komzimba, inkqubo yethu yokuzikhusela komzimba iqala ngempazamo ukuhlasela iiseli zethu ezisempilweni kunye nezicubu. Kwi-GPA, inkqubo yokuzikhusela komzimba ihlasela imithambo yegazi yethu.

Ziziphi izinto ezithile ezibangela i-GPA?

Abaphandi abakayazi kakuhle into ebangela izifo ezibangelwa kukuzikhusela komzimba. Nangona kunjalo, bakholelwa ukuba zininzi izinto ezibangela ezi zifo. Ngamanye amaxesha, inkqubo yethu yomzimba yokuzikhusela ifakwa kakhulu kuxinzelelo, nto leyo enokubangela ezi ngxaki. Ezinye izifo ezinzulu zebhaktheriya kunye neentsholongwaneUphando lufumanise ukuba i-GPA yenzeka emva...

I-GPA ikwiqela lezifo ezibizwa ngokuba yi-ANCA-associated vasculitis, okanye i-AAV . Ezi ziintlobo ze-vasculitis ezibandakanya i-antibody ethile ebizwa ngokuba yi-ANCA. Abaphandi bakholelwa ukuba le ANCA idlala indima ebalulekileyo ekubangeleni ukudumba kwezi zifo, kuquka ne-GPA.

Ufumanisa njani ukuba une-GPA?

Ukuba uneempawu ze-GPA, ugqirha wakho uza kuqala akubuze ngeempawu zakho aze enze uvavanyo lomzimba. Ukuba iimpawu zakho kunye nembali yakho yezonyango zibonisa ukuba usenokuba une-GPA, baza kuyalela ukuba kwenziwe uvavanyo oluthile ukuze kuphandwe ngakumbi.

Phakathi kovavanyo olusetyenziselwa ukufumanisa ukuba unayo na i-GPA kukho:

• Uvavanyo lwegazi: Oku kukwajonga ubukho bee-antibodies ze-ANCA ezikhankanyiweyo ngaphambili.
• Uvavanyo lomchamo: Ukujonga igazi kunye neproteni kumchamo wakho.
• Ii-X-ray zesifuba kunye nee-CT scans: Ezi zijonga imeko yemiphunga yakho.
• I-biopsy: Iqhekeza elincinci lesicubu lithathwa kwimiphunga, kwiisinuses (iimpumlo), okanye kwizintso lize lihlolwe phantsi kwe-microscope ukuze kubonwe ukuba kukho naziphi na iimeko ezithile zokudumba.

Ziziphi iindlela zonyango ze-GPA (Wegener's Granulomatosis)?

Kulungile, ngoku makhe sijonge indlela i-GPA enyangwa ngayo. Oogqirha banyanga i-GPA ngokusebenzisa amayeza anciphisa ukudumba kwaye acinezele inkqubo yomzimba yokuzikhusela (ii-corticosteroids kunye nezinye izinto ezithintela amasosha omzimba).

Amanye ala mayeza anamandla ngakumbi kunezinye. Ukudibanisa amayeza ugqirha wakho awacebisayo kuya kuxhomekeka kubukhali bemeko yakho kunye nomngcipheko weziphumo ebezingalindelekanga.

Unyango olusisiseko lwe-GPA esebenzayo:

• Kunikwa iidosi eziphezulu ze-corticosteroids (umz., i-prednisone). Ezi dosi zincitshiswa kancinci kancinci ngokuhamba kwexesha.
• `(Rituximab)`: Eli liyeza elitsha lebhayoloji. Eli ngoku lolona nyango luqhelekileyo olusetyenziswa kakhulu.
• I-Cyclophosphamide (kwiimeko ezinzima) okanye i-methotrexate (kwiimeko ezibuthathaka): La mayeza akwasetyenziselwa ukunyanga umhlaza. Nangona kunjalo, xa enikwa i-GPA (kwiidosi eziphantsi kakhulu), asebenza njenge-immunosuppressants.
• I-Avacopan: Eli yeza lingasetyenziswa njengonyango olongezelelweyo lokunciphisa umthamo wee-corticosteroids.

Ukuba uneengxaki ezinzima, ungadinga unyango olongezelelweyo. Umzekelo:

• I-Dialysis (ukuba izintso aziphumeleli)
• Ukufakelwa amalungu omzimba

Imeko ye-GPAXa sele kufikelelwe kwinqanaba "lokuxolelwa", oko kuthetha ukuba iimpawu azisekho kwaye isifo singasasebenzi, kunikwa amayeza angenamandla ukugcina loo meko. Oku kudla ngokuquka:

• `(Rituximab)`
• Amayeza okunciphisa amajoni omzimba angenamandla: Umzekelo, `(azathioprine)`, `(methotrexate)` okanye `(mycophenolate mofetil)`.

Kuyo yonke indlela onyangwa ngayo, uza kuqhubeka novavanyo lokujonga imeko yakho kunye nendlela osabela ngayo kunyango. Ugqirha wakho uza kulungisa ummiselo wakho kunye nomthamo ngokusekelwe kwindlela osabela ngayo.

Khumbula, onke amayeza anokuba nemiphumo emibi. Anokwenzeka nangaliphi na ixesha ngexesha lonyango. Ke ngoko, kusenokufuneka ukutshintsha amayeza ukuze kuthintelwe okanye kuncitshiswe eminye imiphumela emibi. Zonke ezi zigqibo kufuneka zenziwe ngokubonisana nogqirha wakho.

Liza kuba njani ikamva kule meko? (Imbono)

I-GPA yimeko yobomi bonke efuna unyango lobomi bonke. Ngonyango, isifo sinokuphela kwaye iimpawu zinokunyamalala. Nangona kunjalo, iimpawu zinokubuya kwakhona (ukubuyela umva).

Kusenokufuneka uthathe amayeza rhoqo ebomini bakho ukuze ulawule ukuphinda ubuye. Ngenxa yokuba la mayeza acinezela amajoni omzimba wakho, kuya kufuneka ulumke ngakumbi ukuze uzikhusele kwezinye izifo.

Ngaba kunokwenzeka ukuba uphile ubomi obuqhelekileyo ngeGPA?

Ngonyango olusebenzayo, ungaphila ubomi obuqhelekileyo nge-GPA. Njengakwezinye izifo ezingapheliyo, kunokubakho amaxesha okuphumla nokubuyela umva kwesi sifo. Ngoko ke indlela esiqhubeka ngayo esi sifo inokuba phezulu nasezantsi kancinci.

Ingakanani iminyaka yobomi bomntu one-GPA?

Ngaphandle konyango, umyinge wobomi obulindelekileyo bumalunga neenyanga ezintlanu, apho abantu abangaphantsi kwama-50% baphila ngaphezu konyaka. Nangona kunjalo, ngonyango, izifundo ezilandelayo zibonisa ukuba abantu abangaphezu kwama-80% basaphila emva kweminyaka esibhozo . Ngoko ke ungabona ukubaluleka kokufumana unyango, akunjalo?

Yintoni eyona nto ibangela ukufa kwi-GPA?

Ngaphambi konyango, ukungasebenzi kakuhle kwamalungu omzimba yeyona mngcipheko mkhulu wokufa kwi-GPA. Ngexesha lonyango, xa inkqubo yakho yomzimba yokuzikhusela ibuthathaka, usengozini enkulu yosulelo olusongela ubomi .

Ndingazinyamekela njani ngelixa ndiphila neGPA?

Xa uphila nesifo esinganyangekiyo sokuzikhusela komzimba esifana ne-GPA, kubalulekile ukuba nolwazi oluthile ngomzimba wakho. Nokuba uzive usempilweni, kufuneka uhlale ukhuselekile.

Thatha amanyathelo okuzikhusela kwizifo eziqhelekileyo ngelixa usela amayeza okunciphisa amajoni omzimba. Ukuba ubona naziphi na iimpawu ezintsha okanye ezingaqhelekanga,Yazisa ugqirha wakho ngoko nangoko.

I-GPA ingakuchaphazela ngeendlela ezininzi, ezinye zazo ezisongela ubomi. Ukuqonda ukuba kukho into engalunganga nokufumana unyango kwangoko kunokwenza umahluko omkhulu.

Abantu abaninzi banokuphila ubomi obungenazo iimpawu, ubuncinane okwethutyana, ngonyango. Kodwa iqela lakho lezonyango liza kuqhubeka nokujonga imeko yakho. Baza kuba ngamaqabane akho obomi bonke ekugcineni impilo yakho.

Izinto ezibalulekileyo ekufuneka uzikhumbule kweli nqaku (Umyalezo Wokuya Ekhaya)

Kulungile, makhe ndishwankathele ezinye zezinto ekufuneka uzikhumbule koko sithethe ngako:

• I-GPA (i-Granulomatosis ene-Polyangiitis) sisifo esichaphazela imithambo yegazi, ingakumbi imithambo yegazi emincinci. Sidla ngokuchaphazela inkqubo yokuphefumla kunye nezintso.
• Iimpawu ziyahluka. Iimpawu zingabandakanya iimpawu ezifana nomkhuhlane, ubunzima bokuphefumla, ukopha kwempumlo, amaqhakuva eluswini, iintlungu zamalungu, iingxaki zamehlo, kunye notshintsho kumchamo.
• Le inokuba yimeko enzima kakhulu, ngoko ke kubaluleke kakhulu ukufuna iingcebiso zonyango ngokukhawuleza ukuba uneempawu.
• Kukho unyango olusebenzayo olufumanekayo. Olu nyango lunokulawula isifo kwaye lusigcine siphilile.
• Le yimeko ehlala ihleli, ngoko ke kufuneka uqhubeke uphantsi kweliso likagqirha, usebenzise amayeza amiselweyo ngokuchanekileyo, kwaye unyamekele umzimba wakho.
• Ungakhathazeki! Ngolwazi kunye neengcebiso ezifanelekileyo zonyango, ungaphila ubomi obuhle nale meko. Awuwedwa, kwaye kukho oogqirha abanokukunceda.

GPA , iGranulomatosis enePolyangiitis, iWegener's Granulomatosis, iVasculitis, Ukudumba kwemithambo yegazi, Izifo ezizikhusela ngokwazo emzimbeni, Izifo zezintso, Izifo zokuphefumla