Yini i-Antihemophilic Factor VIII kanye ne-von Willebrand Factor (Indlela yokungenisa ngemithambo)? Izimpawu, Izimbangela, & Nokwelashwa Kwasekhaya

I-Antihemophilic Factor VIII kanye ne-von Willebrand Factor iyimithi esindisa impilo enikezwa ngemithambo ukuze isize igazi lakho ligqame kahle. Lokhu kwelashwa kuthatha indawo yamaprotheni okugqama angashodi noma anezinkinga kubantu abanezifo ezithile zokuphuma igazi, okusiza ukuvimbela izigameko zokuphuma igazi eziyingozi.

Umzimba wakho ngokujwayelekile wenza lawa maprotheni ukuze amise ukuphuma kwegazi uma ulimala. Uma lawa maprotheni engasebenzi kahle noma eshoda, ngisho nokusikwa okuncane kungaholela ezinkingeni ezingathi sína zokuphuma igazi ezingeke zime zodwa.

Yini i-Antihemophilic Factor VIII kanye ne-von Willebrand Factor?

Le mithi iqukethe amaprotheni amabili abalulekile okugqama kwegazi asebenza ndawonye njengeqembu. I-Factor VIII isebenza njengeprotheni elisizayo elisheshisa inqubo yokugqama, kuyilapho i-von Willebrand Factor isiza ama-platelet egazi lakho ukuthi anamathele ndawonye ukuze akhe amahlule.

Le mithi yenziwa nge-plasma yegazi yomuntu enikelwe ngayo ebilokhu icutshungulwa ngokucophelela futhi ihlanzwa. Inikezwa ngqo emgudwini wakho wegazi ngemithambo, ngokuvamile esibhedlela noma emtholampilo lapho ochwepheshe bezokwelapha bengakuqapha eduze.

Lokhu kwelashwa kuklanyelwe ngokuqondile abantu abangawenzi ngokwanele lawa ma-factor okugqama ngokwemvelo. Ithatha isikhashana indawo yalokho okushodayo emzimbeni wakho, okusiza ukubuyisela ukusebenza okuvamile kokugqama kwegazi.

Kuzizwa kanjani ukuthola lokhu kwelashwa?

Abantu abaningi bazizwa ukufakwa kwemithambo njengokucindezela okuncane, okufana nokuthatha igazi ukuze kwenziwe umsebenzi welabhu. Uma imithi isiqala ukugeleza, ngokuvamile ngeke uzwe lutho olungajwayelekile emgudwini wakho wegazi.

Ngesikhathi sokufakwa, ungase uhlangabezane nemiphumela emincane engemihle. Lokhu kungabandakanya ikhanda elincane, ukuzwa ufudumele noma ukhanya, noma ukucanuzela kwenhliziyo. Le mizwa ivame ukudlula ngokushesha futhi akufanele ikhathaze.

Abanye abantu babika ukuzizwa benamandla noma bekhululekile ngemva kokwelashwa, ikakhulukazi uma bebhekene nezinkinga zokuqhubeka zokuphuma igazi. Ithimba lakho lezokwelapha lizokubhekisisa ngesikhathi nangemva kokufakwa komuthi ukuze liqinisekise ukuthi uyaphendula kahle.

Yini ebangela isidingo salokhu kwelashwa?

Isidingo salo muthi sivela ezifweni zokuphuma igazi ezigadla ngofuzo ezithinta indlela igazi lakho eligqama ngayo. Lezi zimo zihamba emindenini futhi zikhona kusukela ekuzalweni, nakuba izimpawu zingase zingaveli kuze kube kamuva empilweni.

Nawa amakhondishini amakhulu adinga lokhu kwelashwa:

• I-Hemophilia A - uma umzimba wakho ungenzi amaprotheni e-Factor VIII ngokwanele
• Isifo se-von Willebrand - uma i-von Willebrand Factor yakho ingasebenzi kahle noma ingekho
• Ukuntula kwe-Factor VIII kanye ne-von Willebrand Factor - isimo esingavamile esithinta womabili amaprotheni
• Izifo zokuphuma igazi ezitholakele - ngezinye izikhathi zivela kamuva empilweni ngenxa yezinye izimo zezokwelapha

Lezi akuzona izimo ongazithola komunye umuntu noma ozithuthukisa ngezindlela zokuphila. Zenzeka ngenxa yezinguquko ezingeni lakho lezakhi zofuzo ezithinta indlela umzimba wakho owenza ngayo amaprotheni okugqama.

Loku kwelashwa liyisibonakaliso sani noma uphawu luni?

Ukudinga lokhu kwelashwa kubonisa ukuthi unesifo sokuphuma igazi esithinta ikhono legazi lakho lokugqama ngokujwayelekile. Lokhu akuyona uphawu ngokwalo, kodwa kunalokho ukwelashwa kwezokwelapha kwezimo ezingaphansi.

Izimo ezingaphansi lokhu kwelashwa okubhekisela kuzo zihlanganisa izinhlobo eziningana zezifo zokuphuma igazi:

• I-Hemophilia A - isifo esivame kakhulu sokopha esiqatha, esithinta kakhulu amadoda
• Uhlobo 2N lwesifo se-von Willebrand - lapho i-von Willebrand Factor ingakwazi ukuthwala kahle i-Factor VIII
• Uhlobo 3 lwesifo se-von Willebrand - uhlobo oluqatha kakhulu, lapho womabili amaprotheni ephansi kakhulu
• I-hemophilia etholakele - isimo esingavamile lapho amasosha akho omzimba ehlasela izinto zakho zokujiya igazi
• Izimo zokushoda ezihlanganisiwe - izimo ezingavamile kakhulu ezithinta amaprotheni amaningi okujiya igazi

Udokotela wakho uzobe enze izivivinyo ezithile zegazi ukuze athole ngqo ukuthi yisiphi isimo onaso. Lokhu kubasiza ukuthi bakhethe ukwelashwa okulungile kanye nomthamo wesimo sakho esithile.

Ingabe izifo zokopha zingathuthuka zodwa?

Ngeshwa, izifo zokopha ezizuzwe ngomlando azinyamalali zodwa ngoba zibangelwa izinguquko zofuzo. Umzimba wakho uzoqhubeka nokuba nenkinga yokwenza noma ukusebenzisa lawa maprotheni okujiya igazi ngaphandle kokwelashwa kwezokwelapha.

Kodwa-ke, izindaba ezinhle ukuthi ngokwelashwa okufanele, abantu abaningi abanezifo zokopha bangaphila impilo ejwayelekile, esebenzayo. Ukwelashwa njalo kusiza ekuvimbeleni izigameko zokopha eziqatha futhi kuvikela amalunga akho nezitho emonakalweni.

Abanye abantu abanezinhlobo ezithambile zalezi zimo kungenzeka abadingi ukwelashwa ngaso sonke isikhathi. Udokotela wakho uzosebenza nawe ukudala uhlelo lokwelashwa oluhambisana nezidingo zakho ezithile nendlela yakho yokuphila.

Izifo zokopha zingalawulwa kanjani ekhaya?

Ngenkathi ungeke ukwazi ukwelapha isimo esiyisisekelo ekhaya, kunezindlela ezibalulekile zokuzivikela nokuphatha impilo yakho phakathi kokwelashwa kwezokwelapha.

Nansi isu elibalulekile lokuphatha ekhaya elingasiza ukukugcina uphephile:

• Gwema imisebenzi enobungozi obukhulu bokulimala, njengemidlalo yokuxhumana noma ukusebenzisa amathuluzi abukhali ngokunganaki
• Gcina ikhithi yosizo lokuqala enezinto eziningi futhi wazi ukuthi ungasebenzisa kanjani ingcindezi efanele ezinxebeni ezopha
• Gqoka ubucwebe obuxwayisa ngezokwelapha obukhombisa isifo sakho sokopha
• Thatha imithi ebekiwe ngqo njengoba uqondisiwe, okuhlanganisa nanoma yikuphi ukwelashwa kokuvimbela
• Hlala uxhumana njalo nethimba lakho lezempilo futhi ubike noma yikuphi ukuphuma kwegazi okungavamile
• Gcina inhlanzeko enhle yamazinyo ukuze uvimbele ukuphuma kwegazi emagunjini kanye nezinqubo zamazinyo

Kubalulekile futhi ukutshela bonke abahlinzeki bakho bezempilo ngokuphathelene nesifo sakho sokopha ngaphambi kwanoma yiziphi izinqubo. Lokhu kuhlanganisa odokotela bamazinyo, odokotela abahlinzayo, ngisho nodokotela wakho wokunakekelwa okuyisisekelo ukuze uthole ukunakekelwa okuvamile.

Yini ukwelashwa kwezokwelapha kwezifo zokopha?

Ukwelashwa kwezokwelapha kugxila ekufakeni esikhundleni sezinto zokuhlanganisa ezingekho nokuvimbela izigameko zokopha. Uhlelo lwakho lokwelashwa luzolungiswa ngokuqondile esimweni sakho nokuthi sibi kangakanani.

Izinketho zokwelashwa eziyinhloko zihlanganisa:

1. Ukwelashwa kokufaka esikhundleni se-Factor - ukufakwa njalo kwamaprotheni okuhlanganisa angashodi
2. Ukwelashwa kwe-Prophylactic - ukufakwa kokuvimbela ukuze kugcinwe amazinga afanele e-factor yokuhlanganisa
3. Ukwelashwa okudingekayo - ukufakwa okunikezwa lapho ukuphuma kwegazi kwenzeka noma ngaphambi kwezinqubo
4. I-Desmopressin (DDAVP) - umuthi ongakhulisa ukukhiqizwa komzimba wakho we-factor kwezinye izimo
5. Imithi ye-Antifibrinolytic - izidakamizwa ezisiza ukuzinzisa amahlule egazi uma esekhiwe

Udokotela wakho uzoqapha impendulo yakho ekwelashweni ngezivivinyo zegazi ezivamile. Bazolungisa uhlelo lwakho lokwelashwa njengoba kudingeka ukuze baqinisekise ukuthi uthola ukuvikelwa okungcono kakhulu ekuphumeni kwegazi.

Kufanele ngibone nini udokotela ngezinkinga zokopha?

Kufanele uthinte ithimba lakho lezempilo ngokushesha uma uhlangabezana nokuphuma kwegazi okukhulu okungami ngezinyathelo zosizo lokuqala olujwayelekile. Lokhu kubaluleke kakhulu uma wazi ukuthi unesifo sokuphuma kwegazi.

Funani ukunakekelwa kwezimo eziphuthumayo zezokwelapha ngalezi zimo:

• Ukulimala ekhanda noma izinhlungu zekhanda ezinzima ezingase zikhombe ukuphuma kwegazi kwangaphakathi
• Ukuphuma kwegazi okukhulu okugcwalisa amabhandeshi noma okungami ngemuva kwemizuzu engu-15 yokucindezela okuqondile
• Izinhlungu emalungeni nokuvuvukala okungase kukhombe ukuphuma kwegazi kwangaphakathi
• Ukuqina okungavamile okuvela ngaphandle kokulimala noma okukhula ngokushesha
• Igazi emchameni, endleni yokuzithiba, noma ematheni
• Izinhlungu ezinkulu zesisu noma zomhlane ezingase zikhombe ukuphuma kwegazi kwangaphakathi

Ungalindi ukubona ukuthi ukuphuma kwegazi kuyazima yini ngokwako. Ngezifo zokuphuma kwegazi, ngaso sonke isikhathi kungcono ukufuna usizo ngokushesha kunokuthi kamuva, njengoba ukwelashwa kwasekuqaleni kuvimbela izinkinga.

Yiziphi izici eziyingozi zokuthuthukisa izifo zokuphuma kwegazi?

Izifo eziningi zokuphuma kwegazi zizuzwa ngofuzo, okusho ukuthi zidluliselwa emindenini. Isici esiyinhloko esiyingozi ukuba nabazali noma izihlobo ezinezimo ezinjalo.

Izici eziyingozi ezibalulekile zihlanganisa:

• Umlando womndeni wezifo zokuphuma kwegazi - isibikezeli esiqinile
• Ukuba yindoda - nge-hemophilia A, ethwalwa ku-X chromosome
• Ukuba nabazali abathwala izakhi zofuzo zesifo sokuphuma kwegazi
• Izizinda ezithile zobuhlanga ezinamazinga aphezulu ezifo ezithile zokuphuma kwegazi
• Ubudala obuthuthukile - ngezifo zokuphuma kwegazi ezitholakele ezithuthuka kamuva empilweni
• Izimo ze-Autoimmune - ezingase zivuse izifo zokuphuma kwegazi ezitholakele

Uma unomlando womndeni wezifo zokuphuma kwegazi, ukwelulekwa ngezofuzo kungakusiza ukuthi uqonde izingozi zakho. Lokhu kubaluleke kakhulu uma uhlela ukuba nezingane.

Ziyini izinkinga ezingaba khona zezifo zokuphuma kwegazi ezingelashwanga?

Ngaphandle kokwelashwa okufanele, izifo zokuphuma igazi zingaholela ezinkingeni ezingathi sína ezithinta izinga lempilo yakho nempilo iyonke. Izindaba ezinhle ukuthi iningi lalezi zinkinga lingavinjelwa ngokunakekelwa kwezempilo okufanele.

Izinkinga ezingaba khona zihlanganisa:

• Umonakalo wamalunga ovela ekuphumeni igazi okuphindaphindiwe emalungeni, okuholela ku-arthritis nokunyakaza okulinganiselwe
• Ukuphuma igazi kwemisipha okungabangela ubuhlungu, ukuvuvukala, nomonakalo unomphela
• Ukuphuma igazi okungaphakathi okusongela impilo, ikakhulukazi ebuchosheni noma ohlelweni lokugaya ukudla
• I-anemia enkulu evela ekulahlekelweni igazi okungapheli
• Izinkinga ngesikhathi sokuhlinzwa noma izinqubo zamazinyo
• Imiphumela engokwengqondo evela ekuphileni ngokwesaba izigameko zokuphuma igazi

Ngokwelashwa kwesimanje, iningi labantu abanezifo zokuphuma igazi lingakugwema ngokuphelele lokhu. Ukunakekelwa kwezempilo okuvamile nokulandela uhlelo lwakho lokwelashwa kuyisihluthulelo sokuhlala unempilo futhi usebenza.

Ingabe ukwelashwa nge-Factor VIII kanye ne-von Willebrand Factor kulungile noma kubi ezifweni zokuphuma igazi?

Lokhu kwelashwa kulungile ngokuqinisekile kubantu abanezifo zokuphuma igazi - kuvame ukusindisa impilo futhi kungathuthukisa kakhulu izinga lempilo. Izinzuzo zidlula kakhulu izingozi kubantu abadinga lo muthi.

Ukwelashwa kunikeza izinzuzo ngokushesha ngokubuyisela ikhono legazi lakho lokujiya kahle. Lokhu kusho izigameko ezimbalwa zokuphuma igazi, ukulimala okuncane kwamalunga, kanye nekhono lokubamba iqhaza emisebenzini ejwayelekile.

Njengayo yonke imithi, kungaba khona imiphumela emibi, kodwa ukusabela okungathi sína akuvamile. Iningi labantu likubekezelela kahle ukwelashwa futhi libona ukuthuthuka okukhulu ezimpawini zabo nasempilweni yabo yonke.

Yini lokhu kwelashwa okungadidaniswa nayo?

Ngezinye izikhathi abantu badida lokhu kwelashwa okukhethekile kwe-clotting factor neminye imikhiqizo yegazi noma imithi. Kubalulekile ukuqonda ukuthi wamukelani nokuthi kungani kukhethwe ngokuqondile isimo sakho.

Lokhu kwelashwa kungadidaniswa nalokhu:

• Ukumpontshwa kwegazi - okuthatha indawo yegazi lonke kunokuba amaprotheni athile okugcwalisa igazi
• Ukumpontshwa kwe-plasma - equkethe amaprotheni amaningi ahlukene, hhayi lawo owadingayo kuphela
• Ezinye izinto ezigxilisiwe zokugcwalisa igazi - ezingaqukatha inhlanganisela ehlukene yamaprotheni
• Imithi yokugcwalisa igazi eyenziwe ngamachiza - esebenza ngendlela ehlukile kunokwelashwa ngokufaka esikhundleni
• Uketshezi olujwayelekile lwe-IV - olungenawo ama-clotting factors

Iqembu lakho lokunakekelwa kwezempilo lizoqinisekisa ukuthi uqonda kahle ukuthi yikuphi ukwelashwa okutholayo. Unganqikazi ukubuza imibuzo ngemithi yakho nokuthi kungani kuyisinqumo esingcono kakhulu sesimo sakho esithile.

Imibuzo evame ukubuzwa mayelana nokwelashwa kwe-Factor VIII kanye ne-von Willebrand Factor

Ingabe iseshini ngayinye yokwelashwa ithatha isikhathi esingakanani?

Ukumnika okuningi kuthatha phakathi kwemizuzu engama-30 kuya emahoreni ama-2, kuye ngomthamo wakho nokuthi umzimba wakho usabela kanjani. Ithimba lakho lokunakekelwa kwezempilo lizokuqapha ngalesi sikhathi ukuze liqinisekise ukuthi uzizwa ukhululekile futhi usabela kahle ekwelashweni.

Ngizodinga ukwelashwa kaningi kangakanani?

Ukuvama kokwelashwa kuyahlukahluka kakhulu kuye ngesimo sakho esithile nobukhulu. Abanye abantu badinga ukumnika izikhathi eziningana ngesonto ukuze bavimbele, kanti abanye badinga ukwelashwa kuphela lapho kuvela ukopha noma ngaphambi kwezinqubo. Udokotela wakho uzokwenza isimiso esikulungele.

Ngingahamba ngenkathi ngithola lokhu kwelashwa?

Yebo, abantu abaningi abanezifo zokopha bahamba ngempumelelo. Uzodinga ukuhlela kusengaphambili ngokuxhumana nezikhungo zokwelashwa lapho uya khona futhi mhlawumbe uthwale izinto eziphuthumayo. Ithimba lakho lokunakekelwa kwezempilo lingakusiza ukuthi ulungiselele uhambo oluvikelekile.

Ingabe zikhona imiphumela yesikhathi eside yalokhu kwelashwa?

Kubantu abaningi, imiphumela yesikhathi eside iyakhuthaza kakhulu - impilo engcono, izigameko ezimbalwa zokuphuma igazi, kanye nempilo engcono. Ngokuvamile, abanye abantu bangase bakhe amasosha omzimba enza ukwelashwa kungasebenzi kahle, kodwa odokotela bakho bayakubheka lokhu futhi bangashintsha ukwelashwa uma kudingeka.

Ingabe lokhu kwelashwa kukhokhelwa umshwalense?

Izinhlelo eziningi zomshwalense zikukhokhela lokhu kwelashwa ngoba kuthathwa njengokudingekayo ngokwezokwelapha kubantu abanezifo zokuphuma igazi. Kodwa-ke, imininingwane yokukhokhelwa iyahluka, ngakho-ke kubalulekile ukusebenza neqembu lakho lezempilo kanye nenkampani yomshwalense ukuze uqonde izinzuzo zakho ezithile kanye nanoma yiziphi izindleko ezingaba khona.