Created at:1/13/2025
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I-Antihemophilic factor iyimithi esindisa impilo eshintsha iphrotheni yokujiya engatholakali kubantu abane-hemophilia A. Cabanga ngayo njengokunika igazi lakho isithako esibalulekile esidingayo ukuze lenze amahlule futhi lime ukopha kahle.
Le mithi iqukethe i-Factor VIII, iphrotheni esiza igazi lakho ukuba lijiye ngokujwayelekile. Uma une-hemophilia A, umzimba wakho awukhiqizi le phrotheni eyanele noma ukhiqiza inguqulo engasebenzi kahle. Ukujova kwe-antihemophilic factor kungena ukuvala leli gebe, ukusiza ekuvikeleni izigameko zokopha eziyingozi.
I-Antihemophilic factor iyindlela egxilile ye-Factor VIII, iphrotheni yokujiya kwegazi abantu abane-hemophilia A abangenayo. Inikezwa nge-IV ukuze ibuyisele ngokushesha ikhono legazi lakho lokujiya.
Le mithi iza ngezinhlobo ezimbili eziyinhloko: ezitholakala nge-plasma (ezitholakala egazini lomuntu elinikelwe) kanye ne-recombinant (eyenziwe e-lab usebenzisa ubunjiniyela bezakhi zofuzo). Zombili izinhlobo zisebenza ngendlela efanayo emzimbeni wakho. Udokotela wakho uzokhetha inketho engcono kakhulu ngokusekelwe ezidingweni zakho ezithile kanye nomlando wezokwelapha.
Imithi icutshungulwa ngokucophelela futhi ihlolwe ukuze kuqinisekiswe ukuthi iphephile futhi iyasebenza. Izindlela zokukhiqiza zesimanje zenze le mikhiqizo yaphepha kakhulu kunaleyo eyayikhona emashumini eminyaka edlule, ngokuhlola okukhulu izifo ezithathelwanayo.
I-Antihemophilic factor iphatha futhi ivimbele ukopha kubantu abane-hemophilia A, isimo sezakhi zofuzo lapho igazi lingajiyi kahle. Kuyisistimu yokusekela yomzimba wakho lapho ukujiya kwemvelo kwehluleka.
Odokotela banquma le mithi ngezimo eziningana ezibalulekile. Ungase uyidinge ukuze umise izigameko zokopha ezisebenzayo, kungakhathaliseki ukuthi zivela ekulimaleni, emsebenzini wokuhlinzwa, noma ukopha okuzenzakalelayo emalungeni nasemisipheni. Iphinde isetshenziswe ngokuvimbela ngaphambi kokuhlinzwa okuhleliwe noma izinqubo zamazinyo ukunciphisa ingozi yokopha.
Abanye abantu abanegazi eliningi elibukhali bathatha imithamo ejwayelekile ukuvimbela ukuphuma kwegazi ngokuphelele. Le ndlela, ebizwa ngokuthi ukwelashwa ngokuqapha, ingasiza ukuvikela amalunga akho nemisipha ekulimaleni okungadalwa ukuphuma kwegazi okuphindaphindiwe ngokuhamba kwesikhathi.
I-Antihemophilic factor isebenza ngokufaka esikhundleni se-Factor VIII protein engekho egazini lakho okwesikhashana. Uma ifakwe, iqala ngokushesha ukusiza igazi lakho ukuthi lenze amahlule ngokujwayelekile.
Uma ulimala, umzimba wakho uqala inqubo eyinkimbinkimbi ebizwa ngokuthi i-clotting cascade. I-Factor VIII idlala indima ebalulekile kule nqubo, isiza ukuguqula amanye amaprotheni e-clotting abe amafomu awo asebenzayo. Ngaphandle kwe-Factor VIII eyanele, le cascade ibambeka, futhi ukuphuma kwegazi kuqhubeka isikhathi eside kunokuba kufanele.
Umuthi ujikeleza emgudwini wakho wegazi amahora amaningana kuya ezinsukwini, kuye ngokuthi umzimba wakho uwuchitha ngokushesha kangakanani. Ngesikhathi esinjalo, igazi lakho lingahluleka ngempumelelo enkulu, likuvikele ekuphumeni kwegazi okuyingozi.
I-Antihemophilic factor inikezwa njalo njengomjovo we-intravenous (IV), kungaba ngumhlinzeki wezempilo noma nguwe uqobo uma uqeqeshiwe. Umuthi kufanele ungene ngqo emgudwini wakho wegazi ukuze usebenze ngempumelelo.
Ngaphambi komjovo ngamunye, uzodinga ukuxuba umuthi uma uza njengempushana. Landela imiyalelo yokuxuba ngqo njengoba udokotela wakho noma usokhemisi ekukhombise. Sebenzisa kuphela amanzi akhethekile (i-diluent) eza nomuthi, ungalokothi uthole amanzi ompompi noma ezinye iziphuzo.
Umjovo kufanele unikezwe kancane kancane ngemizuzu eminingana. Ukushesha kungabangela imiphumela emibi engakhululeki njengokukhukhula noma ukushaya kwenhliziyo okusheshayo. Gcina umuthi efrijini kodwa vumela ukuthi ufike ezingeni lokushisa legumbi ngaphambi kokuxuba nokujova.
Abantu abaningi bafunda ukuzijova ekhaya, okuhlinzeka ngoshintsho oluningi kanye nokwelashwa okusheshayo ngesikhathi sokopha. Ithimba lakho lezempilo lizokufundisa izindlela ezifanele zokujova futhi likusize uzizwe uqiniseka ngale nqubo.
Ubude bokwelashwa nge-antihemophilic factor buqondene nesimo sakho somuntu ngamunye nesizathu sokuthi uyayithatha. Ezikhathini zokopha okusebenzayo, ungase udinge imithamo izinsuku ezimbalwa nje kuze kube yilapho ukopha kuyeka.
Uma uhlinzwa noma wenza inqubo yamazinyo, ukwelashwa kuvame ukuqala ngaphambi kwaleyo nqubo futhi kuqhubeke izinsuku eziningana ngemuva kwayo. Udokotela wakho uzoqapha amazinga akho e-Factor VIII futhi alungise isimiso sokwelashwa ngokusekelwe ekuqhubekeni kwakho kokuphola.
Ukwelashwa kokuvimbela, abanye abantu bathatha imithamo ejwayelekile izinyanga noma iminyaka. Le ndlela yesikhathi eside inganciphisa kakhulu izikhathi zokopha futhi ivikele amalunga akho ekulimaleni. Udokotela wakho uzobuyekeza njalo ukuthi le ndlela isafanele yini kuwe.
Ungayeki ukwelashwa ngokuzumayo ngaphandle kokukhuluma nodokotela wakho kuqala. Ukuyeka kungazelelwe kungakushiya ubhekene nokopha okukhulu, ikakhulukazi uma une-hemophilia eqinile.
Abantu abaningi bayayithanda i-antihemophilic factor kahle, kodwa njenganoma imuphi umuthi, ingabangela imiphumela emibi. Izindaba ezinhle ukuthi ukusabela okukhulu akuvamile, futhi imiphumela eminingi emibi imnene futhi yesikhashana.
Imiphumela emibi ejwayelekile ongase uyithole ihlanganisa ukusabela okuncane endaweni yokujova njengobubomvu, ukuvuvukala, noma ubumnene. Abanye abantu baphawula futhi ukuthi banomuzwa wokuba nekhanda elibuhlungu, isiyezi, noma ukunambitheka kwensimbi emlonyeni wabo ngesikhathi noma ngemuva kokujova.
Nawa imiphumela emibi ejwayelekile engenzeka:
Lezi zimo zivame ukuzixazulula ngokushesha zodwa futhi azidingi ukumisa ukwelashwa.
Ngokuvamile, abanye abantu bathuthukisa imiphumela emibi ekhathazayo edinga ukunakekelwa kwezokwelapha. Ukusabela komzimba kungase kwenzeke, nakuba kungavamile ngamalungiselelo anamuhla. Izimpawu zihlanganisa ubunzima bokuphefumula, ukuvuvukala ebusweni bakho noma emqaleni, noma ukuqubuka okubanzi.
Nansi imiphumela emibi engavamile kodwa ebucayi okufanele iqashelwe:
Xhumana nodokotela wakho ngokushesha uma uhlangabezana nanoma yiziphi zalezi zimpawu ezibucayi.
Inkinga engavamile kodwa ebalulekile ukuthuthukiswa kwama-inhibitors - ama-antibody aqeda i-Factor VIII. Lokhu kwenzeka cishe kuma-15-30% abantu abane-hemophilia A enzima, ngokuvamile phakathi kwezinsuku zokuqala ezingu-75 zokuchayeka ekwelashweni.
Bambalwa kakhulu abantu abane-hemophilia A abangakwazi ukuthatha i-antihemophilic factor, kodwa izimo ezithile zidinga ukuqapha okwengeziwe noma ukwelashwa okuhlukile. Udokotela wakho uzohlola ngokucophelela umlando wakho wezokwelapha ngaphambi kokunquma lo muthi.
Abantu abanokungezwani komzimba okukhulu okwaziwayo emikhiqizweni ye-Factor VIII noma yiziphi izithako kufanele bagweme lo muthi. Uma uke waba nokusabela okukhulu komzimba emikhiqizweni yegazi esikhathini esidlule, udokotela wakho uzodinga ukucabangela ezinye izindlela zokwelapha.
Labo abanezixuku zegazi ezisebenzayo noma umlando wokuphazamiseka kokujiya kwegazi badinga ukuqashwa ngokucophelela. Nakuba kungavamile, i-Factor VIII ngezinye izikhathi ingaholela ekwakhekeni kwezixuku, ikakhulukazi kubantu asebenama-risk factor okujiya kwegazi.
Abantu abanezifo ezithile zesimiso somzimba sokuzivikela noma labo abathatha imithi ecindezela amasosha omzimba bangase babe nengozi eyengeziwe yokuthuthukisa ama-inhibitors. Udokotela wakho uzohlola izinzuzo nezingozi ngokucophelela kulezi zimo.
I-Antihemophilic factor iyatholakala ngaphansi kwamagama amaningi e-brand, ngalinye linezici ezehlukile. Udokotela wakho uzokhetha inketho engcono kakhulu ngokusekelwe ezidingweni zakho ezithile nomlando wezokwelapha.
Amabhrendi ajwayelekile atholakala nge-plasma afaka i-Humate-P, Koate-HP, kanye ne-Monoclate-P. Lezi zenziwa ngegazi lomuntu elinikelwe futhi zithatha izinqubo eziningi zokuhlanzwa nokungasebenzi kwamagciwane.
Amabhrendi e-Recombinant afaka i-Advate, Helixate FS, Kogenate FS, Novoeight, kanye ne-Nuwiq. Lezi zenziwa kusetshenziswa izindlela zobunjiniyela bezakhi zofuzo futhi aziqukethe imikhiqizo yegazi yomuntu, okuyinto abanye abantu abayithandayo.
Imikhiqizo enempilo ende njenge-Adynovate, Eloctate, kanye ne-Jivi ihlala isikhathi eside emzimbeni wakho, okungenzeka ukuthi inciphise imvamisa yokujova. Udokotela wakho uzokusiza ukuthi uqonde umehluko futhi ukhethe okusebenzela kangcono indlela yakho yokuphila.
Ezinye izindlela ziyatholakala kulabo abangakwazi ukusebenzisa i-antihemophilic factor ejwayelekile noma labo abathuthukisa ama-inhibitors. Lezi zinketho zandise amathuba okwelashwa kakhulu eminyakeni yamuva.
Kubantu abanama-inhibitors, ama-bypassing agents afana ne-Factor VIIa (NovoSeven) noma i-activated prothrombin complex concentrate (FEIBA) angasiza igazi ukuthi lijiye ngaphandle kokudinga i-Factor VIII. Lezi zisebenza ngokwenza ezinye izingxenye ze-clotting cascade.
I-Emicizumab (Hemlibra) iyimithi entsha elingisa umsebenzi we-Factor VIII kodwa empeleni ayiyona i-Factor VIII. Inikezwa njengesijovo esingaphansi kwesikhumba esikhundleni se-IV, okutholakala ukuthi kulula kubantu abaningi.
Nge-hemophilia A emnene, i-desmopressin (DDAVP) ngezinye izikhathi ingashukumisa umzimba wakho ukuthi ukhulule i-Factor VIII egciniwe. Lokhu kusebenza kuphela kubantu abanokukhiqizwa okuthile kwe-Factor VIII okusebenzayo.
I-Antihemophilic factor isahlala iyindlela yokwelapha engcono kakhulu ye-hemophilia A, kodwa "ngcono" kuncike esimweni sakho ngasinye. Kubantu abaningi abane-hemophilia A, ukufaka i-Factor VIII kuyindlela yokwelapha esebenza kahle kakhulu.
Uma iqhathaniswa nezindlela zokwelapha ezindala, imikhiqizo yesimanje ye-Factor VIII iphephe kakhulu futhi isebenza kahle kakhulu. Zithatha izivivinyo eziqinile kanye nezinqubo zokuhlanzwa eziqeda ngokoqobo ingozi yokudluliswa kwamagciwane okwakuyinto ekhathazayo emashumini eminyaka edlule.
Ezinye izindlela ezintsha ezifana ne-emicizumab zinikeza ezinye izinzuzo, ikakhulukazi ukulula kwezifutho ezingaphansi kwesikhumba kanye nezikhathi ezide zokulinganisa. Kodwa-ke, ukwelashwa ngokufaka i-Factor VIII kuneminyaka eminingi yobumbano obuqinisekisiwe nokusebenza kahle.
Ukwelashwa okungcono kakhulu kuwe kuncike ezintweni ezinjengobunzima be-hemophilia yakho, indlela yokuphila, izintandokazi zokujova, nokuthi ngabe unamathuluzi okuthuthukile. Ithimba lakho lezempilo lizokusiza ukuthi ulinganise lezi zinto ukuze wenze ukukhetha okungcono kakhulu.
Yebo, i-antihemophilic factor iphephile futhi isetshenziswa kakhulu ezinganeni ezinomdlavuza we-hemophilia A. Eqinisweni, ukwelashwa kwasekuqaleni kubalulekile ekuvikeleni ukulimala kwamalunga nezinye izinkinga ezingenzeka ngokuphindaphinda izigameko zokuphuma kwegazi.
Izingane zingase zidinge imithamo ehlukile kunezabantu abadala ngokusekelwe esisindweni sazo kanye namazinga e-Factor VIII. Udokotela wengane yakho uzochaza umthamo ofanele futhi angawulungisa njengoba ingane yakho ikhula. Izingane eziningi nemindeni yazo zifunda ukuzenzela imijovo ekhaya, okuhlinzeka ngoshintsho lwezindlela zokuphila ezisebenzayo.
Ukuqapha njalo kubalulekile ezinganeni, njengoba zithambekele ekuthuthukiseni izithiyo kunezabantu abadala. Ithimba lezempilo lengane yakho lizobheka izimpawu zokuthuthukiswa kwezithiyo futhi lilungise ukwelashwa njengoba kudingeka.
Uma ngengozi uzinika i-antihemophilic factor eningi kakhulu, ungangeni ekuqeqesheni. Ukweqisa imithamo akuvamile ukuba kuyingozi, kodwa kufanele uxhumane nodokotela wakho noma isikhungo sokwelashwa ngokushesha ukuze uthole isiqondiso.
Okukhathaza kakhulu nge-Factor VIII eningi kakhulu yingozi eyengeziwe yamaqhubu egazi, nakuba lokhu kungavamile. Qaphela izimpawu ezinjengobuhlungu emlenzeni noma ukuvuvukala, ubuhlungu besifuba, noma ukuphefumula kanzima, futhi funa ukwelashwa ngokushesha uma lokhu kwenzeka.
Gcina irekhodi lalokho okwenzekile, okuhlanganisa ukuthi ungakanani umuthi owuthathile nokuthi nini. Lolu lwazi luzosiza ithimba lakho lezempilo ukuthi lihlole isimo futhi linikeze isiqondiso esifanele. Bangase bafune ukukuqapha eduze noma balungise umthamo wakho olandelayo.
Uma uphuthelwa umthamo ohleliwe we-antihemophilic factor, wuthatha ngokushesha lapho ukhumbula, ngaphandle uma sekuseduze isikhathi somthamo wakho olandelayo. Ungaphindi imithamo ukuze ulungise okungaphuthelwanga.
Kubantu abathola ukwelashwa kwe-prophylactic, ukuphuthelwa umthamo ngezikhathi ezithile ngeke kubangele izinkinga ngokushesha, kodwa zama ukubuyela esimisweni ngokushesha ngangokunokwenzeka. Amazinga akho e-Factor VIII azokwehla kancane kancane, okungenzeka ukuthi akushiye ubuthakathaka ekopheni.
Uma welapha isigameko sokopha esisebenzayo futhi uphuthelwa umthamo, xhumana nodokotela wakho ngokushesha. Ukopha okusebenzayo kungase kudingeke ukuthi kunikezwe imithamo kaningi, futhi ukubambezeleka kungaholela ezinkingeni ezingathi sína.
Akufanele uyeke ukuthatha i-antihemophilic factor ngaphandle kokubonisana nodokotela wakho kuqala. Kubantu abane-hemophilia A, lo muthi ngokuvamile ungukwelashwa kwempilo yonke okubalulekile ukuvimbela ukopha okuyingozi.
Uma uwuthatha ngenxa yesigameko esithile sokopha noma ukuhlinzwa, udokotela wakho uzokutshela ukuthi kuphephile nini ukuyeka ngokusekelwe emazingeni akho e-Factor VIII kanye nentuthuko yokuphola. Lesi sinqumo kufanele senziwe ngaso sonke isikhathi ngaphansi kokuqondiswa kwezokwelapha.
Kulabo abathatha ukwelashwa kwe-prophylactic, ukuyeka kungakhuphula kakhulu ingozi yakho yezigameko zokopha kanye nomonakalo wamajoyinti. Uma ucabangela izinguquko ohlelweni lwakho lokwelashwa, xoxa ngobungozi nezinzuzo ngokucophelela nethimba lakho lezempilo.
Yebo, ungahamba ne-antihemophilic factor, kodwa kudinga ukuhlela okuthile. Umuthi udinga ukuhlala efrijini, ngakho uzodinga i-cooler enama-ice pack okuthutha.
Letha incwadi evela kudokotela wakho echaza isimo sakho sezokwelapha nokuthi kungani udinga ukuthwala lo muthi. Ezokuphepha esikhumulweni sezindiza kufanele zikuvumele ukuthi uwudlulise, kodwa ukuba nemibhalo kusiza ukugwema ukubambezeleka noma izinkinga.
Pakisha imithi eyengeziwe uma kwenzeka ukubambezeleka kohambo, futhi ucwaninge izikhungo zokwelapha i-hemophilia lapho uya khona. Abantu abaningi bakuthola kuwusizo ukuxhumana ne-World Federation of Hemophilia ukuze bathole ulwazi mayelana nezikhungo zokwelapha kwamanye amazwe.