Ithemba elitsha kwabo baneHemophilia A: Funda ngeAltuviiio!

Ngaba wena okanye umntu ohlala nawe uneHemophilia A? Ukuba kunjalo, mhlawumbi uyazi kakuhle ubunzima bokuyeka ukopha nokuba kukwinxeba elincinci, kunye nesidingo sokujova rhoqo. Oku ngokwenene ngumceli mngeni otshintsha ubomi. Kodwa ngoku kukho iyeza elitsha elinokusetyenziswa ukunyanga le meko, kwaye lingasetyenziswa kube kanye ngeveki. Namhlanje, sithetha ngelo yeza elibizwa ngokuba yiAltuviiio.

Yintoni iHemophilia A? Inganceda njani i-Altuviiio?

Ngamafutshane, i-hemophilia A yimeko yemfuza edluliselwa kumzali ukuya emntwaneni. Umntu onesi sifo unezinga eliphantsi kakhulu le-Factor VIII, iproteni ebalulekileyo enceda ukujiya kwegazi.

Cinga ngegazi lethu njengodaka oludibanisa udonga. Xa kuvela inxeba, olu daka kufuneka ludibane ukuze luvalwe. I-Factor VIII ifana nesamente edibanisa olu daka. Ngenxa yokuba umntu one-hemophilia A akanayo le 'samente' (i-Factor VIII), kuthatha ixesha elide ukuyeka ukopha enxebeni.

Le meko inokuba buthathaka, ibe phakathi, okanye ibe nzima, kuxhomekeke kwinqanaba le-factor VIII. Ngenxa yokuba esi sifo sizuzwe njengelifa (X-linked), sixhaphake kakhulu kumadoda, kwaye amatyala anzima kakhulu ayabonakala kuwo. Abafazi nabo banokuchaphazeleka, kodwa iimpawu zihlala zincinci.

Umntu one-hemophilia enzima unokopha amalungu, izihlunu, okanye amalungu angaphakathi ngaphandle kwesizathu esicacileyo, kungekuphela nje ngenxa yokusikeka okuncinci. Oku sikubiza ngokuba "kukuphuma kwegazi okuzenzekelayo." Ukuba oku akuphathwanga kakuhle, kunokubangela umonakalo wexesha elide kwaye kube yingozi ebomini.

Unyango oluqhelekileyo kukufaka i-recombinant factor VIII protein, eyenziwa kwilebhu. Oku kusetyenziswa ngeendlela ezimbini:

1. Unyango "olufunekayo": Ukunika inaliti kuphela xa kuvela ukopha ukuze kuthintelwe ukopha.

2. Unyango lokuthintela: Ukuthintela ukopha kwizigulana ezineemeko ezinzima, inaliti eqhubekayo inikwa ngokweshedyuli emiselweyo (umzekelo, kabini okanye kathathu ngeveki).

Kulapho i-Altuviiio ingena khona. Nangona uninzi lwamayeza e-factor VIII akhoyo lufuna iidosi ezimbini okanye ezintathu ngeveki, i-Altuviiio yidosi enikwa kanye ngeveki. Oku kungenxa yokuba isakhiwo sayo esikhethekileyo seprotheyini (`Fc-VWF-XTEN`) sivumela i-factor VIII ukuba ihlale izinzile kwaye isebenza emzimbeni ixesha elide.

Ulisebenzisa njani eli yeza?

I-Altuviiio liyeza elenziwe ngolwelo elifakwa emthanjeni. Oko kukuthi, i-IV (efakwa ngemithambo yegazi). Eli lidla ngokunikezelwa yingcali yezempilo esibhedlele okanye eklinikhi.

Ibhokisi yamayeza inebhotile yepowder, isirinji yolwelo, kunye nezinye izixhobo ezifunekayo. Okokuqala, kufuneka udibanise umgubo kunye nolwelo ukuze uphinde udibanise iyeza. Ukuze wenze oku, kufuneka usebenzise iadaptha ekhethekileyo ukufaka ulwelo oluvela kwisirinji kancinci kwibhotile. Emva koko, kufuneka ujikelezise ibhotile kancinci kancinci ukuze unyibilikise umgubo ngokupheleleyo. Okulandelayo, kufuneka utsale ulwelo ubuyele kwisirinji.

Eyona nto ibalulekileyo: Jonga ngononophelo phambi kokusebenzisa iyeza. Ulwelo olulungisiweyo kufuneka lucace, lungabi nambala okanye lube mthubi okhanyayo . Musa ukulusebenzisa ukuba lunamabala okanye amasuntswana. Iyeza elilungisiweyo kufuneka lisetyenziswe zingadlulanga iiyure ezi-3.

Ungadinga iibhotile ezingaphezulu kwesinye kwidosi yakho oyimiselweyo. Ukuba kunjalo, kuya kufuneka udibanise amayeza avela kwiibhotile ezininzi kwisirinji enkulu oza kuyinikwa ngugqirha wakho.

Nje ukuba idosi ilungile, kufakwa ityhubhu ekhethekileyo kwisirinji, eqhagamshelwe kwinaliti efakwe emthanjeni. Emva koko isirinji icinezelwa kancinci ukuze kufakwe iyeza emzimbeni. Oku kungathatha naphi na ukusuka kwimizuzu eli-1 ukuya kweli-10.

Ziziphi iingozi ezithile zale yeza?

Ngamanye amaxesha, imizimba yethu yenza into efana 'nezilwi' nxamnye nale protein yeFactor VIII elawulwa ngaphandle. Ngokwezonyango, ezi zibizwa ngokuba zi-inhibitors okanye ii-antibodies. Ukuba ezi inhibitors zenziwe, ziyayitshabalalisa iFactor VIII, nto leyo eyenza ukuba eli chiza lingasebenzi kakuhle.

Akukacaci ukuba oku kuxhaphake kangakanani kubantu abasebenzisa i-Altuviiio. Kodwa ungakhathazeki, ugqirha wakho uza kujonga igazi lakho rhoqo ukuze abone ukuba ezi zithinteli sele ziphuhlile na.

• Ukuba izithinteli ziphantsi, ugqirha unokunyusa umthamo wamayeza.
• Ukuba amanqanaba akho okuthintela aphakama kakhulu, usenokungakwazi ukusebenzisa i-Altuviiio. Ukuba oku kuyenzeka, ugqirha wakho uza kukuchazela ezinye iindlela zonyango ezenzelwe ngokukodwa abantu abane-inhibitors.

Zifumene ntoni ezi zifundo?

Izifundo ezenziweyo ukuze kubonwe ukusebenza kakuhle kwe-Altuviiio zivelise iziphumo ezilungileyo kakhulu. Masiyijonge kwitheyibhile elula.

Ezi ziphumo zibonisa ukuba i-Altuviiio liyeza elisebenzayo kakhulu ekuthinteleni ukopha kwegazi nokulawula ukopha ukuba kuyenzeka.

Ezinye izinto ezimbalwa ekufuneka uzazi

• Isebenza ngokukhawuleza kangakanani le mithi?

I-Altuviiio inyusa amanqanaba akho e-factor VIII kwangoko emva kokufakwa inaliti. Oku kunciphisa umngcipheko wokopha ngokukhawuleza. Nangona kunjalo, ungayeki ukuthatha iyeza ngaphandle kwengcebiso kagqirha wakho . Ukuze ugcine amanqanaba akho e-factor VIII ephezulu, kufuneka uwathathe njengoko kucwangcisiwe veki nganye.

• Ingaba iyasebenzisana namanye amayeza?

Ngokwazi kwethu, i-Altuviiio ayikabikho ingxelo yokuba isebenzisana namanye amayeza. Nangona kunjalo, kubalulekile ukuxelela ugqirha wakho ngayo yonke imithi oyithathayo, kuquka neevithamini.

• Ingaba eli liyeza elikhethekileyo?

Ewe. I-Altuviiio liyeza 'elikhethekileyo'. Oku kuthetha ukuba lisenokungafumaneki kwikhemesti eqhelekileyo, kwaye linokufuneka lifunyanwe kwikhemesti ekhethekileyo yesibhedlele okanye ngenkqubo ebonelelwe ngurhulumente. Ugqirha wakho uza kukhokela kule nto.