Yini i-Antihemophilic Factor (Recombinant, Glycopegylated-exei)? Ukusetshenziswa, Imiphumela, & Umhlahlandlela Wokwelapha

I-Antihemophilic factor (recombinant, glycopegylated-exei) iyinguqulo eyenziwe e-lab yento yokuhlanganisa igazi i-VIII esiza igazi lakho ukuthi lenze amahlule ngendlela efanele. Lo muthi wenzelwe ngqo abantu abane-hemophilia A, isimo lapho umzimba wakho ungenzi ngendlela eyanele yaleli phrotheni ebalulekile yokuhlanganisa igazi. Ingxenye ethi "glycopegylated" isho ukuthi ishintshiwe ukuze ihlale isikhathi eside emzimbeni wakho, ngakho udinga imijovo embalwa.

Yini i-Antihemophilic Factor (Recombinant, Glycopegylated-exei)?

Lo muthi uyisithuthukisi esenziwe ngamanye sezinto ezi-VIII, iphrotheni igazi lakho eliyidingayo ukuze lihlanganise ngendlela ejwayelekile. Uma uthola ukusikeka noma ukulimala, into e-VIII isiza ukuqala inqubo yokuhlanganisa igazi eyeka ukopha. Abantu abane-hemophilia A abenzi into e-VIII eyanele noma benza inguqulo engasebenzi kahle.

Inguqulo ye-recombinant yenziwa e-laboratory kusetshenziswa ubuchwepheshe obuthuthukisiwe be-biotechnology, hhayi emikhiqizweni yegazi lomuntu. Lokhu kuyenza iphephe ekuthelelaneni okutholakala egazini. Ukuguqulwa kwe-glycopegylated kungeza ama-molecule akhethekile asiza umuthi ukuthi uhlale usebenza egazini lakho isikhathi eside kunezinto ezijwayelekile ze-VIII.

Kuzwakala kanjani ukwelashwa ngalo muthi?

Uzothola lo muthi ngomjovo we-IV, ngokuvamile emthanjeni engalweni yakho. Umjovo ngokwawo uzizwa ufana nokuthola igazi noma ukuthola omunye umuthi we-IV. Abantu abaningi bachaza ukuthi njengokuchofoza okusheshayo okulandelwa umuzwa opholile njengoba umuthi ungena egazini lakho.

Ngemuva komjovo, ungase uzwe ukukhululeka wazi ukuthi ikhono lakho lokuhlanganisa igazi libuyiselwe. Abanye abantu bahlangabezana nemiphumela emincane efana nekhanda noma isiyezi, kodwa abaningi abazizwa lutho oluhlukile nhlobo. Umuthi usebenza buthule ohlelweni lwakho ukuvimbela noma ukwelapha izigameko zokopha.

Yini ebangela isidingo salo muthi?

I-Hemophilia A iyisizathu esiyinhloko sokuthi uzodinga lo muthi. Lesi simo sezakhi zofuzo senzeka lapho umzimba wakho unezinkinga ngofuzo olutshela amaseli akho ukuthi enze kanjani i-factor VIII. Ngaphandle kwe-factor VIII eyanele esebenzayo, igazi lakho alikwazi ukujiya kahle, okuholela ekuphumeni kwegazi isikhathi eside.

Izici eziningana zingadala isidingo salokhu kwelashwa okuthile:

• I-hemophilia A eyazuza ngofuzo kubazali bakho
• Izigameko zokuphuma kwegazi ezinzima ezidinga ukwelashwa ngokushesha
• Ukuhlinzwa okuhleliwe noma izinqubo zamazinyo
• Ukulimala okubangela ukuphuma kwegazi ngaphakathi noma ngaphandle
• Ukuphuma kwegazi emalungeni okungalimaza i-cartilage namathambo

Ngezinye izikhathi, abantu bathuthukisa i-hemophilia A kamuva empilweni ngenxa yezimo ze-autoimmune lapho umzimba wabo uhlasela i-factor VIII yawo. Lolu hlobo olutholiwe ludinga ukwelashwa kokufaka esikhundleni se-factor.

Yiziphi izimo lo muthi osetshenziselwa ukuzelapha?

Lo muthi ngokuyinhloko welapha i-hemophilia A, kodwa odokotela bawusebenzisa ezimweni eziningana ezithile. Umgomo oyinhloko uhlala ukubuyisela ikhono legazi lakho lokujiya ngokujwayelekile nokuvimbela ukuphuma kwegazi okuyingozi.

Nazi izimo eziyinhloko nezimo lapho lo muthi usiza khona:

• I-hemophilia A enzima (ngaphansi kuka-1% womsebenzi ojwayelekile we-factor VIII)
• I-hemophilia A emaphakathi (1-5% yomsebenzi ojwayelekile we-factor VIII)
• I-hemophilia A elula ngesikhathi sezigameko zokuphuma kwegazi ezinkulu
• I-hemophilia A etholwe ezimweni ze-autoimmune
• Ukwelashwa kwe-prophylactic ukuvimbela izigameko zokuphuma kwegazi
• Ukulungiselela ngaphambi kokuhlinzwa kwabantu abane-hemophilia A

Udokotela wakho uzoqoka umthamo ofanele neshejuli ngokusekelwe emazingeni akho athile e-factor VIII nomlando wokuphuma kwegazi. Abanye abantu badinga imithamo yokuvimbela ejwayelekile, kanti abanye badinga kuphela ukwelashwa ngesikhathi sezigameko zokuphuma kwegazi.

Ingabe izigameko zokuphuma kwegazi zingaxazululeka ngaphandle kwalo muthi?

Kubantu abane-hemophilia A, izigameko zokuphuma kwegazi azivamile ukuzixazulula ngokwazo. Umzimba wakho awunawo umthamo owanele we-factor VIII ukwakha amahlule afanele, ngakho ukuphuma kwegazi kuvame ukuqhubeka isikhathi eside kakhulu kunokujwayelekile. Ngaphandle kokwelashwa, ngisho nokulimala okuncane kungaba kubi.

Ukusikeka okuncane nokuklwebheka kungase ekugcineni kuyeke ukuphuma kwegazi ngokucindezela nesikhathi, kodwa ukuphuma kwegazi kwangaphakathi noma ukuphuma kwegazi emalungeni cishe ngaso sonke isikhathi kudinga ukufakwa kwe-factor. Ukulibazisa ukwelashwa kungaholela ekulimaleni kwamalunga unomphela, ukuphuma kwegazi kwemisipha, noma ukuphuma kwegazi kwangaphakathi okusongela impilo.

Yingakho abantu abaningi abane-hemophilia A besebenzisa ukwelashwa kwe-prophylactic, bethola imithamo ejwayelekile ukuvimbela izigameko zokuphuma kwegazi ngaphambi kokuthi ziqale. Le ndlela ithuthukise kakhulu izinga lempilo futhi yanciphisa izinkinga zesikhathi eside.

Ingabe le mithi inikezwa kanjani?

Le mithi inikezwa kuphela ngomjovo we-intravenous, okusho ngqo emsingeni wakho nge-vein. Awukwazi ukuyithatha ngomlomo ngoba uhlelo lwakho lokugaya ukudla luzohlukanisa amaprotheni ngaphambi kokuthi asebenze. Indlela ye-IV iqinisekisa ukuthi i-factor VIII ifika ngqo lapho idinga ukuya khona.

Abantu abaningi bafunda ukuzijova ekhaya ngemva kokuqeqeshwa okufanele eqenjini labo labanakekeli bezempilo. Le nkululeko ivumela ukwelashwa ngokushesha lapho kwenzeka izigameko zokuphuma kwegazi. Inqubo yokujova ngokuvamile ithatha imizuzu embalwa nje uma usukhululekile ngobuchwepheshe.

Udokotela wakho uzobala umthamo wakho ngokusekelwe esisindweni sakho, ubukhulu bokuphuma kwegazi, kanye namazinga akho e-factor VIII. Abanye abantu badinga imijovo njalo ezinsukwini ezimbalwa zokuvimbela, kanti abanye bayayidinga kuphela ngesikhathi sezigameko zokuphuma kwegazi.

Yini inqubo yokwelashwa yezokwelapha yale mithi?

Indlela yakho yokwelashwa incike ekutheni usebenzisa yini lo muthi ukuvimbela noma ukwelapha ukopha okusebenzayo. Ukuvimbela, ngokuvamile uzothola imijovo kabili kuya kathathu ngesonto. Ezindaweni zokopha, ungase udinge imithamo evame kakhulu kuze kube yilapho ukopha kuyeka.

Nansi indlela yokwelashwa kwezokwelapha evame ukusebenza ngayo:

• Umthamo wokuqala obalwa ngokuya ngesisindo sakho kanye nezinga le-factor VIII eliqondiwe
• Ukuqapha amazinga akho e-factor VIII ngokusebenzisa ukuhlolwa kwegazi
• Ukulungiswa komthamo ngokuya ngempendulo yakho kanye namaphethini okopha
• Ukuqokwa okulandelayo njalo ukuze kuhlolwe ukusebenza kahle
• Izindlela eziphuthumayo zezigameko zokopha okukhulu

Ithimba lakho lezempilo lizokufundisa ukuthi ungazibona kanjani izinhlobo ezahlukene zokopha nokuthi nini okufanele ufune ukunakekelwa kwezempilo ngokushesha. Bazokusiza futhi ukuthi uthuthukise uhlelo lokwelashwa olwenzelwe wena oluhambisana nendlela yakho yokuphila kanye namaphethini okopha.

Kufanele ngifune nini ukunakekelwa kwezempilo ngenkathi ngisebenzisa lo muthi?

Kufanele uthinte udokotela wakho ngokushesha uma uhlangabezana nokopha okukhulu okungaphenduli emthozini wakho ovamile wokwelashwa. Lokhu kungase kubonise ukuthi umzimba wakho uqala ukuthuthukisa ama-antibody ngokumelene ne-factor VIII, okudinga izindlela zokwelashwa ezihlukile.

Funa ukunakekelwa kwezempilo okuphuthumayo uma uhlangabezana:

• Ukulimala ekhanda noma ukopha kwangaphakathi okusolwayo
• Ukuphuma kwegazi okukhulu emalungeni ngobuhlungu obukhulu nokuvuvukala
• Ukuphuma kwegazi okuqhubekayo naphezu kokwelashwa okufanele kwe-factor VIII
• Izimpawu zokungezwani komzimba nezinto ezithile njengobunzima bokuphefumula noma ukuvuvukala ebusweni
• Amaphethini okopha angavamile noma izinhlobo ezintsha zokopha

Kufanele futhi uthinte umhlinzeki wakho wezempilo uma uqaphela noma yiziphi izinguquko endleleni umthamo wakho ovamile olawula ngayo ukopha. Ngezinye izikhathi izidingo zomzimba wakho ziyashintsha ngokuhamba kwesikhathi, futhi uhlelo lwakho lokwelashwa lungase ludinge ukulungiswa.

Ziyini izici eziyingozi zokudinga lo muthi?

Into enkulu yengozi ukuba ne-hemophilia A, evame ukuzuzwa kubazali bakho. I-Hemophilia A ithinta ikakhulukazi amadoda ngoba imfuzo ye-factor VIII itholakala ku-X chromosome. Amadoda anayo i-X chromosome eyodwa kuphela, ngakho-ke uma ethola imfuzo enezinkinga, athuthukisa i-hemophilia.

Izinto eziningana zikhuphula amathuba okudinga le mithi:

• Umlando womndeni we-hemophilia A
• Ubulili besilisa (yize abesifazane bengaba ngabathwali futhi ngezinye izikhathi bathinteke)
• Izigameko zangaphambilini zokuphuma kwegazi ezidinga ukufakwa kwe-factor
• Amazinga aphansi e-factor VIII
• Indlela yokuphila esebenzayo ekhuphula ingozi yokulimala
• Izimo ezithile ze-autoimmune ezingabangela i-hemophilia etholakele

Ubudala bungadlala indima, njengoba izingane ezincane nabantu abadala abasebenzayo bavame ukuba nezigameko eziningi zokuphuma kwegazi ngenxa yokuwa, ukulimala kwezemidlalo, noma imisebenzi ejwayelekile yobuntwana. Ithimba lakho lezempilo lingakusiza ukuthi uqonde izici zakho eziyingozi.

Ziyini izinkinga ezingaba khona zale mithi?

Abantu abaningi bayayibekezelela kahle le mithi, kodwa njengayo yonke imithi, ingabangela imiphumela emibi. Imiphumela emibi evame kakhulu imnene futhi yesikhashana, njengekhanda elibuhlungu, isiyezi, noma isicanucanu esincane. Lokhu kuvame ukuthuthuka njengoba umzimba wakho ujwayela ukwelashwa.

Nawa ama-complications angaba khona okufanele uwaqaphele:

• Ukuphendula komzimba okusukela ekuqhumeni kwesikhumba okuncane kuya ku-anaphylaxis enkulu
• Ukuthuthukiswa kwama-inhibitors (ama-antibodies avimbela i-factor VIII)
• Ukuphendula kwendawo yokujova njengobuhlungu, ukuvuvukala, noma ukulimala
• Amahlule egazi uma i-factor VIII eningi yakheka ohlelweni lwakho
• Umkhuhlane noma izimpawu ezinjengomkhuhlane ngemuva kokujova

Ukuthuthukiswa kwama-inhibitors yilona complication elibi kakhulu lesikhathi eside, elenzeka cishe kuma-20-30% abantu abane-hemophilia A eqinile. Udokotela wakho uzoqapha lokhu ngokuhlolwa kwegazi okuvamile futhi alungise ukwelashwa kwakho uma kudingeka.

Ingabe lo muthi uphephe ngabantu abanezimo ezinye?

Lo muthi ngokuvamile uphephe kubantu abaningi abane-hemophilia A, kodwa izimo ezithile zidinga ukuqapha okwengeziwe. Udokotela wakho uzobukeza ngokucophelela umlando wakho wezokwelapha ngaphambi kokuqala ukwelashwa ukuze aqinisekise ukuthi kuphephile esimweni sakho esithile.

Abantu abanezimo ezilandelayo badinga ukuqashwa okukhethekile:

• Isifo senhliziyo noma ingozi yamaqhubu egazi
• Isifo sesibindi esithinta izici zokujiya kwegazi
• Isifo sezinso esithinta ukususwa kwemithi
• Izifo ze-Autoimmune
• Ukuphendula komzimba okwedlule emikhiqizweni ye-factor VIII

Ukukhulelwa nokuncelisa ngokuvamile kuthathwa njengokuphephile ngalo muthi, njengoba amaprotheni engaweli ngaphesheya kwe-placenta noma angene obisini lwebele ngamanani amakhulu. Kodwa-ke, udokotela wakho uzokuqapha eduze ngalezi zikhathi.

Yini lo muthi ongase udidaniswe nayo?

Lo muthi ungase udidaniswe neminye imikhiqizo yegazi noma ama-concentrate e-clotting factor. Igama elide kanye nemigomo yezobuchwepheshe kungakwenza kube lula ukuxuba nemithi ezwakala ngendlela efanayo. Njalo qinisekisa ukuthi uthola umuthi ofanele ngokuqinisekisa igama lomkhiqizo futhi ubuze umhlinzeki wakho wezempilo.

Ngezinye izikhathi ididaniswa nalokhu:

• Eminye imikhiqizo ye-factor VIII enezindlela ezihlukene
• I-Factor IX concentrates esetshenziselwa i-hemophilia B
• I-plasma entsha eqandisiwe noma i-cryoprecipitate
• Ezinye izici zokujiya kwegazi ezihlanganisiwe
• I-factor VIII ejwayelekile ngaphandle kokuguqulwa kwe-glycopegylated

Umehluko omkhulu ukuthi lo muthi othile uhlala isikhathi eside emzimbeni wakho, ngakho-ke udinga imijovo embalwa uma uqhathaniswa nemikhiqizo ejwayelekile ye-factor VIII. Usokhemisi wakho nethimba lezempilo bangakusiza ukuthi uqonde kahle ukuthi yini oyitholayo.

Imibuzo evame ukubuzwa mayelana ne-Antihemophilic Factor (Recombinant, Glycopegylated-exei)

Ingabe umthamo ngamunye uhlala isikhathi esingakanani emzimbeni wami?

Lo muthi uvame ukuhlala isikhathi eside kunezinto ezijwayelekile ze-factor VIII, ngokuvamile unikeza ukuvikelwa izinsuku ezi-3-4 esikhundleni sezinsuku ezi-1-2. Ukuguqulwa kwe-glycopegylated kuyawusiza ukuthi uhlale usebenza egazini lakho isikhathi eside, okusho ukuthi ungasusa imijovo yakho. Udokotela wakho uzonquma isikhathi esiqondile ngokusekelwe ekuphenduleni kwakho komuntu ngamunye kanye namazinga e-factor VIII.

Ngingahamba yini nalo muthi?

Yebo, ungahamba nalo muthi, kodwa uzodinga imibhalo efanele evela kudokotela wakho. Umuthi udinga ukuqandiswa nokusingathwa ngokucophelela, ngakho-ke uzodinga i-cooler yokuhamba namaphakethe eqhwa. Izindiza eziningi zivumela izinto zokwelapha njengezinto eziphethwe, kodwa hlola nenkampani yakho yezindiza ngaphambi kwesikhathi futhi uphethe incwadi kadokotela wakho kanye nencwadi kadokotela.

Ngizodinga yini lo muthi impilo yami yonke?

Uma une-hemophilia A, cishe uzodinga olunye uhlobo lokushintshwa kwe-factor VIII empilweni yakho yonke. Kodwa-ke, imithi emisha iyathuthukiswa engashintsha lokhu esikhathini esizayo. Abanye abantu abane-hemophilia A etholakele bangase bayeke ukudinga ukwelashwa uma isimo sabo esingaphansi sithuthuka, kodwa lokhu kuncike esimweni sakho esithile.

Ngingakwazi yini ukuzivocavoca nokudlala imidlalo ngenkathi ngisebenzisa lo muthi?

Yebo, abantu abaningi abane-hemophilia A abasebenzisa ukushintshwa kwe-factor VIII bangahlanganyela emidlalweni nasekuvivinyeni umzimba. Ukwelashwa kwe-prophylactic kuvame ukuvumela indlela yokuphila esebenzayo. Kodwa-ke, kufanele ugweme imidlalo yokuxhumana enomthelela omkhulu ethwala ingozi enkulu yokulimala. Sebenza nethimba lakho lezempilo ukuze uthuthukise uhlelo lomsebenzi oliphephile esimweni sakho esithile.

Ngingenzani uma ngiphuthelwa umthamo ohleliwe?

Uma uphuthelwa umthamo wokuvimbela, wuthatha ngokushesha lapho ukhumbula, ngaphandle uma sekuseduze isikhathi somthamo wakho olandelayo ohleliwe. Ungaphindi imithamo. Xhumana nomhlinzeki wakho wezempilo ukuze uthole isiqondiso sokuthi ungawulungisa kanjani uhlelo lwakho. Ukuphuthelwa imithamo kungakhuphula ingozi yakho yezigameko zokopha, ngakho zama ukugcina uhlelo lwakho olujwayelekile ngangokunokwenzeka.