Uma uthola ukusikeka okuncane, ukuphuma kwegazi kuyaphela ngemva kwesikhashana, akunjalo? Lokhu kungenxa yendlela emangalisayo emizimbeni yethu. Kodwa kwabanye abantu, ukuphuma kwegazi akukuvimbeli lokhu kalula. Ngisho nokusikeka okuncane kungabangela ukuba ukuphuma kwegazi kuqhubeke. Namuhla, sizokhuluma ngesimo esibizwa ngokuthi i-hemophilia, abantu abaningi abake bezwa ngaso kodwa abasazi ngempela. Ngaphambi kokuba sesabe ngakho, ake siqonde ukuthi siyini ngempela.
Wazi kanjani ukuthi une-hemophilia?
Kalula nje, noma kuphi lapho kukhona ukopha emzimbeni wethu, kwakheka into efana 'ne-plug' ukuze kumiswe ukopha. Lokhu sikubiza ngokuthi i-fibrin clot. Ukuze kwakheke leli hlule, amaprotheni amaningi akhethekile egazini lethu kudingeka asebenze ndawonye. Lokhu yilokho esikubiza ngokuthi "izici zokugoba."
I-Hemophilia yisimo lapho esinye salezi zici zokujiya kwegazi, ikakhulukazi i-factor VIII noma i-factor IX, singabi khona noma sinciphe kusukela ekuzalweni. Ngemuva kwalokho, i-blood clot ayakheki kahle, bese ukuphuma kwegazi kuqhubeka ngaphandle kokuyeka.
Uma udokotela wakho esola ukuthi unalesi simo, uzokwenza izivivinyo eziningana egazini lakho.
- Isinyathelo sokuqala: Kuthathwa isampula yegazi lakho bese kuhlolwa ilebhu ukuthi kuthatha isikhathi esingakanani ukuthi lijiye.
- Isinyathelo 2: Uma lezo zivivinyo zibonisa noma yikuphi ukukhubazeka, kuzokwenziwa izivivinyo eziqondile ukuze kubonakale ukuthi ingakanani i-factor VIII kanye ne-factor IX egazini lakho.
Lezi zivivinyo zingasiza udokotela wakho ukuthi anqume ukuthi hlobo luni lwe-hemophilia onayo (i-Hemophilia A noma i-B), kanye nobunzima bayo.
Yiziphi izindlela zokwelapha i-hemophilia?
Ukwelashwa kwe-hemophilia kuyahlukahluka kuye ngohlobo kanye nobukhali besimo onaso. Isibonelo, uma une -hemophilia encane , ungadinga ukwelashwa kuphela ngemva kokulimala noma ngaphambi kokuhlinzwa.
Kodwa-ke, uma une -hemophilia enzima , lapho uphuma khona igazi njalo, kuzodingeka uqhubeke nokwelashwa njalo ukuze uvimbele ukuphuma kwegazi futhi uvimbele amalunga akho ukuba angalimali futhi abe buthaka.
Kunezindlela eziningana zokwelapha eziyinhloko:
- Ukufaka esikhundleni se-depleted clotting factor (Ukwelashwa Kokufaka Esikhundleni)
- Eminye imithi ethile
- Ukwelashwa kwemiphumela emibi njengokuphuma kwegazi emalungeni
Ukunikeza isici sokugoba esingenaso
Lokhu ukwelashwa okuyinhloko. Lokhu kuhilela ukunikeza umzimba wakho umthamo we-clotting factor entulekayo ngemithambo yegazi (ngokusebenzisa i-intravenous noma nge-IV).
- I-Factor VIII kubantu abane- hemophilia ANikeza.
- I-Factor IX inikezwa abantu abane- hemophilia B.
Lezi zinto zenziwa ngezindlela ezimbili. Enye ivela ku-plasma yegazi elinikelwe . Enye iyizinto ze-DNA zokwenziwa noma ezihlanganisiwe ezenziwe elabhorethri . Izinhlobo ezintsha zezinto ezitholakalayo namuhla zenzelwe ukuhlala isikhathi eside emzimbeni. Ngakho-ke, akudingeki zijovwe kaningi njengoba zazinjalo ngaphambili.
Eminye imithi ethile
Ngaphezu kokwelashwa ngezinto ezibangela ukuqhekeka kwegazi, kuneminye imithi yesimanje engasiza ekulawuleni ukopha kwegazi. Udokotela wakho uzokuchazela yona.
| Umuthi (igama lomkhiqizo) | Ngobani? | Amaphuzu abalulekile |
|---|---|---|
| I-Emicizumab-kxwh (HEMLIBRA) | Kubantu abane-hemophilia A | Kuyasiza ekuvimbeleni ukuphuma kwegazi. Lokhu akunikezwa ngemithambo yegazi, kodwa njengomjovo ongaphansi kwesikhumba kanye ngesonto. |
| Izidakamizwa ezifana ne-Adynovate, i-Idelvion | Kubantu abane-hemophilia A noma B | Lezi yizinto zokwenziwa ezithuthukisiwe ezinikezwa esikhundleni se-Factor VIII noma i-IX. Lezi zihlala emzimbeni isikhathi eside. |
| I-Desmopressin (i-DDAVP) | Kubantu abane-hemophilia encane A | Lo muthi ukhulisa okwesikhashana amazinga omzimba e-factor VIII. Unganikezwa ngemithambo yegazi, njengomjovo, noma njengesifutho samakhala. |
| I-Tranexamic acid, i-Aminocaproic acid | Ezimweni ezikhethekile | Lena imithi ethathwa ngendlela yamaphilisi. Isebenza ngokuvimbela ihlule legazi ukuthi lincibilike kalula. |
| I-Obizur | Kubantu abane -hemophilia A etholakale | Lesi yisimo esikhethekile. Akuyona into ozalwa nayo. Singakhula kamuva ekuphileni ngenxa yezici ezifana nokukhulelwa nomdlavuza. Siphathwa ngohlobo lwe-factor VIII olutholakala ezingulubeni. |
Qaphela ukopha emalungeni kanye nezinye izinkinga.
Uphawu oluvamile lwe-hemophilia ukuphuma kwegazi emalungeni . Lokhu kungenzeka ezindaweni ezifana namadolo, izindololwane, nasemaqakaleni. Uma lokhu kwenzeka, ilunga liyavuvukala futhi libe buhlungu.
- Ukwelashwa: Kubalulekile ukuthola ukwelashwa kwe-factor yakho ngokushesha. Ngaphandle kwalokho, kungase kube nomonakalo ongapheli esilungwini. Ngaphezu kwalokho, udokotela wakho uzokweluleka ukuthi uphumuze isilungwini bese ufaka iqhwa . Lokhu kuzonciphisa ubuhlungu nokuvuvukala.
- Ngemva: Ngemva kokudamba kobuhlungu, ukwelashwa ngokomzimba kunganconywa futhi ukusiza ukubuyisela ukunyakaza kwamalunga namandla.
Futhi, kudingeka uqaphele ngemisebenzi yomzimba oyenzayo. Akuzona zonke imidlalo ezifanele umuntu one-hemophilia. Imidlalo yokuxhumana eyingozi ingabangela ukopha kwangaphakathi. Khuluma nodokotela wakho ukuze unqume ukuthi yikuphi ukuvivinya umzimba kanye nemidlalo okufanele wena kanye nokungafaneleki.
Ingabe ukwelashwa kungabangela izinkinga?
Nakuba ukwelashwa kuphephile kakhulu namuhla, kunezinto ezimbili okufanele uzazi.
1. Izifo ezithwalwa igazi
Esikhathini esidlule, cishe ngawo-1980, kwakunengozi enkulu yokuthola amagciwane afana ne-HIV kanye ne-hepatitis ngenxa yezinto ezisekelwe egazini. Kodwa manje akudingeki ukhathazeke ngalokho. Namuhla, abanikeli begazi bahlolwa ngokucophelela, futhi lonke igazi elinikelwe lihlolwa amagciwane futhi lihlanzwe kusetshenziswa izindlela ezikhethekile. Ngakho-ke manje ingozi yokuthola isifo ngokwelashwa iphansi kakhulu. Kodwa-ke, odokotela batusa ukugonyelwa i-hepatitis A kanye ne-B.
2. Izinguquko ohlelweni lokuzivikela komzimba (Ama-Inhibitors)
Lokhu kuyinkimbinkimbi kancane, kodwa kubalulekile ukuqonda. Ngezinye izikhathi, isimiso somzimba wethu sokuzivikela, esiwuhlelo lokuzivikela lomzimba wethu, siyaqaphela lesi sici sokujiya esisinika sona 'njengesitha'. Bese, siqala ukubhubhisa leso sici. Lokhu sikubiza ngokuthi ukwakheka "kwama-inhibitors" . Uma lokhu kwenzeka, ukwelashwa okuthathayo kungase kungasebenzi kahle. Ngakho-ke, udokotela wakho uzohlola igazi lakho njalo ukuze abone ukuthi lokhu kuyenzeka yini.
Umlayezo Wokuya Nawe Ekhaya
- I-Hemophilia yisimo sokuzalwa esithinta ukujiya kwegazi.
- Ukwelashwa okuyinhloko ukunikeza i-exogenous factor VIII noma i-IX, entula umzimba.
- Udokotela wakho uzonquma ukwelashwa okungcono kakhulu kuwe, kuye ngohlobo kanye nobunzima besimo sakho.
- Ukopha emalungeni kuvamile, futhi uma kwenzeka, kubaluleke kakhulu ukufuna ukwelashwa ngokushesha.
- Izindlela zokwelapha i-hemophilia ezinikezwa namuhla ziphephile kakhulu. Ungazesabi ngokungadingekile.
- Uma wena noma ingane yakho inalesi simo, hlalani nisebenzisana eduze nodokotela wakho futhi nilandele iseluleko sakhe.
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