Xa ufumana inxeba elincinci, ukopha kuyayeka emva kwexesha elithile, akunjalo? Oku kungenxa yendlela emangalisayo emizimbeni yethu. Kodwa kwabanye abantu, ukopha akukuthinteli lula oku. Nokuba inxeba elincinci linokubangela ukuba ukopha kuqhubeke. Namhlanje, siza kuthetha ngemeko ebizwa ngokuba yi-hemophilia, abantu abaninzi abaye beva ngayo kodwa abayazi ngokwenene. Ngaphambi kokuba soyike ngayo, masiqonde ukuba yintoni kanye kanye.
Uyazi njani ukuba une-hemophilia?
Ngamafutshane, naphi na apho kukho ukopha emzimbeni wethu, into efana 'neplagi' iyakheka ukuze kumiswe ukopha. Oku sikubiza ngokuba yi-fibrin clot. Ukuze kwenziwe eli hlwili, iiproteni ezininzi ezikhethekileyo egazini lethu kufuneka zisebenzisane. Ezi zizinto esizibiza ngokuba "zizinto ezivala igazi."
I-Hemophilia yimeko apho enye yezi zinto zokuqunjelwa kwegazi, ngakumbi i-factor VIII okanye i-factor IX, ingabikho okanye inciphe kwasekuzalweni. Emva koko, i-blood clot ayikheki kakuhle, kwaye ukopha kuyaqhubeka ngaphandle kokuyeka.
Ukuba ugqirha wakho ukrokrela ukuba unale meko, uza kwenza uvavanyo oluninzi egazini lakho.
- Inyathelo lokuqala: Kuthathwa isampulu yegazi lakho kwaye ilebhu ivavanya ukuba kuthatha ixesha elingakanani ukuba lijiye.
- Inyathelo lesi-2: Ukuba ezo mvavanyo zibonisa naziphi na izinto ezingaqhelekanga, kuya kwenziwa uvavanyo oluthe ngqo ukuze kubonwe ukuba ingakanani i-factor VIII kunye ne-factor IX egazini lakho.
Olu vavanyo lunokunceda ugqirha wakho ukuba afumanise ukuba loluphi uhlobo lwe-hemophilia onayo (i-Hemophilia A okanye i-B), kunye nobunzima bayo.
Ziziphi iindlela zonyango lwe-hemophilia?
Unyango lwe-hemophilia luyahluka ngokuxhomekeke kuhlobo kunye nobukhali bemeko onayo. Umzekelo, ukuba une -hemophilia encinci , unokufuna unyango kuphela emva kokwenzakala okanye ngaphambi kotyando.
Nangona kunjalo, ukuba une -hemophilia enzima , apho uphuma igazi rhoqo, kuya kufuneka uqhubeke nokuthatha unyango rhoqo ukuthintela ukopha kunye nokuthintela amalungu akho ukuba angonakali kwaye abe buthathaka.
Kukho iindlela ezininzi eziphambili zonyango:
- Ukutshintsha i-depleted clotting factor (Unyango lokubuyisela)
- Amanye amayeza athile
- Unyango lweziphumo ebezingalindelekanga ezifana nokopha emalungwini
Ukubonelela nge-clotting factor engafanelekanga
Olu lolona nyango luphambili. Oku kuquka ukunika umzimba wakho idosi ye-clotting factor engekhoyo ngemithambo yegazi (ngaphakathi okanye nge-IV).
- I-Factor VIII kubantu abane -hemophilia ANika.
- I-Factor IX inikwa abantu abane -hemophilia B.
Ezi zinto zenziwa ngeendlela ezimbini. Enye ivela kwi-plasma yegazi enikelweyo . Enye zizinto ze-DNA ezenziweyo okanye eziphinda-phindweyo ezenziwe kwilebhu . Iintlobo ezintsha zezinto ezikhoyo namhlanje zenzelwe ukuhlala ixesha elide emzimbeni. Ke ngoko, akufuneki zifakwe rhoqo njengoko bezisenza ngaphambili.
Amanye amayeza athile
Ukongeza kunyango lwezinto ezibangela ukugabha kwegazi, kukho nezinye iindlela zonyango zanamhlanje ezinokunceda ekulawuleni ukopha kwegazi. Ugqirha wakho uza kukuchazela ezi zinto.
| Iyeza (igama lophawu) | Yekabani? | Amanqaku abalulekileyo |
|---|---|---|
| I-Emicizumab-kxwh (HEMLIBRA) | Kubantu abane-hemophilia A | Inceda ekuthinteleni ukopha. Oku akunikwa ngemithambo yegazi, kodwa kunikwa njengenaliti engaphantsi kwesikhumba kanye ngeveki. |
| Iziyobisi ezifana ne-Adynovate, i-Idelvion | Kubantu abane-hemophilia A okanye B | Ezi zizinto eziphuculweyo ezenziweyo ezinikwa endaweni yeFactor VIII okanye IX. Ezi zihlala emzimbeni ixesha elide. |
| I-Desmopressin (i-DDAVP) | Kubantu abane-hemophilia encinci A | Eli yeza linyusa okwethutyana amanqanaba omzimba e-factor VIII. Linokunikwa ngemithambo yegazi, njengenaliti, okanye njenge-spray yempumlo. |
| I-Tranexamic acid, i-Aminocaproic acid | Kwiimeko ezikhethekileyo | La ngamayeza athathwa ngendlela yeepilisi. Asebenza ngokuthintela ukuqhekeka kwegazi ekunyibilikeni lula. |
| I-Obizur | Kubantu abane -hemophilia A efunyenweyo | Le yimeko ekhethekileyo. Ayisiyonto ozalwa nayo. Ingakhula kamva ebomini ngenxa yezinto ezifana nokukhulelwa kunye nomhlaza. Inyangwa ngohlobo lwe-factor VIII olufumaneka kwiihagu. |
Lumka xa kuphuma igazi emalungeni nakwezinye iingxaki.
Uphawu oluqhelekileyo lwe-hemophilia kukuphuma kwegazi emalungeni . Oku kunokwenzeka kwiindawo ezifana namadolo, iingqiniba, namaqakala. Xa oku kusenzeka, ilungu liyadumba kwaye libe buhlungu.
- Unyango: Kubalulekile ukufumana unyango lwakho lwe-factor ngoko nangoko. Kungenjalo, umonakalo osisigxina kwilungu unokubakho. Ukongeza, ugqirha wakho uya kukucebisa ukuba uphumze ilungu kwaye ufake umkhenkce . Oku kuya kunciphisa iintlungu kunye nokudumba.
- Emva koko: Emva kokuba intlungu iphelile, unyango lomzimba lunokucetyiswa ukunceda ukubuyisela amandla kunye nokuhamba kwamalungu.
Kwakhona, kufuneka ulumke ngemisebenzi yomzimba oyenzayo. Ayizizo zonke imidlalo ezifanelekileyo kumntu one-hemophilia. Imidlalo enobungozi, edibanisayo inokubangela ukopha ngaphakathi. Thetha nogqirha wakho ukuze agqibe ukuba zeziphi imithambo kunye nemidlalo efanelekileyo nengafanelekanga kuwe.
Ngaba unyango lungabangela iingxaki?
Nangona unyango lukhuselekile kakhulu namhlanje, kukho izinto ezimbini ekufuneka uzazi.
1. Izifo ezithwalwa ligazi
Ngaphambili, malunga neminyaka yoo-1980, kwakukho umngcipheko omkhulu wokufumana iintsholongwane ezifana ne-HIV kunye ne-hepatitis ngenxa yezinto ezisekelwe egazini. Kodwa ngoku akufuneki ukhathazeke ngaloo nto. Kule mihla, abanikeli begazi bahlolwa ngokusondeleyo, kwaye lonke igazi elinikelweyo livavanywa iintsholongwane kwaye licocwe kusetyenziswa iindlela ezikhethekileyo. Ngoko ke ngoku umngcipheko wokufumana isifo ngenxa yonyango uphantsi kakhulu. Nangona kunjalo, oogqirha bacebisa ukuba ugonyelwe i-hepatitis A kunye ne-B.
2. Utshintsho kwinkqubo yomzimba yokuzikhusela (ii-Inhibitors)
Oku kunzima kancinci, kodwa kubalulekile ukukuqonda. Ngamanye amaxesha, inkqubo yomzimba yethu yokuzikhusela, eyinkqubo yokuzikhusela yomzimba wethu, iyayiqonda le nto yokujiya kwegazi esiyinika yona 'njengotshaba'. Emva koko, iqala ukutshabalalisa loo nto. Oku sikubiza ngokuba kukwenziwa "kwezithinteli" . Ukuba oku kuyenzeka, unyango oluthathayo lusenokungasebenzi kakuhle. Ke ngoko, ugqirha wakho uya kuvavanya igazi lakho rhoqo ukuze abone ukuba oku kuyenzeka na.
Umyalezo Wokuya Ekhaya
- I-Hemophilia yimeko yokuzalwa echaphazela ukujiya kwegazi.
- Unyango oluphambili kukunika i-exogenous factor VIII okanye IX, enganelanga emzimbeni.
- Ugqirha wakho uza kugqiba unyango olulungele wena, kuxhomekeke kuhlobo kunye nobunzima bemeko yakho.
- Ukopha kwamalungu kuqhelekile, kwaye ukuba kuyenzeka, kubaluleke kakhulu ukufuna unyango ngokukhawuleza.
- Unyango lwe-hemophilia olunikezelwayo namhlanje lukhuselekile kakhulu. Musa ukuzoyika ngokungeyomfuneko.
- Ukuba wena okanye umntwana wakho unale meko, soloko usebenzisana nogqirha wakho kwaye ulandele icebiso lakhe.
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