Ukuba une-hemophilia, mhlawumbi uyazi ukuba kunokubangela ukukhathazeka nokoyikisa kangakanani ukopha rhoqo, ngamanye amaxesha ngaphandle kwesizathu, kwaye unengxaki yokuyeka
ukopha nokuba kuncinci kangakanani. Usenokuba sele usela amayeza okunyanga ukopha kwakho, okanye usenokuba usela amayeza okukuthintela. Nangona kunjalo, ngokuhambela phambili kwesayensi yezonyango, ngoku kukho unyango oluninzi nolusebenzayo nolutsha lwale meko. Ngoko ke namhlanje, masithethe ngeyeza elitsha, elahlukileyo. Libizwa ngokuba yi-Qfitlia (fitusiran).
Okokuqala, masibone, kutheni ukopha kungayeki kwi-hemophilia?
Ngamafutshane, kukho iiproteni ezikhethekileyo ezinceda ekuqhekekeni kwegazi. Ezi sizibiza ngokuba
" zizinto ezibangela ukujiya kwegazi ." Njengokuba i-mortar ifuneka ukuze kubanjwe izitena xa kusakhiwa udonga, ezi zinto zibangela ukujiya kwegazi zibalulekile ukuze kumiswe ukopha. Umntu one-hemophilia akanayo enye yezi zinto zibangela ukujiya kwegazi.
- Abantu abane -hemophilia A abanayo into ebizwa ngokuba yiFactor VIII.
- Abantu abane -hemophilia B abanayo into ebizwa ngokuba yiFactor IX.
Xa ezi zinto zincipha, ukuveliswa kwelinye inyathelo elibalulekileyo kwinkqubo yokuqunjelwa kwegazi, elibizwa ngokuba
yi-thrombin , kuyancipha. I-Thrombin yeyona nto iphambili eyenza i-blood clot ize iyeke ukopha. Ngoko ke xa i-thrombin incipha, kuba nzima ukuyeka ukopha xa isenzeka. Uyaqonda? Ngokwesiqhelo, into ebesiyenza njengonyango ixesha elide kukwenza ngokwenziwa i-clotting factor (factor 8 okanye 9) engabikho emzimbeni wakho size siyinike
njengenaliti emthanjeni. Kodwa iyeza le-Qfitlia esithetha ngalo namhlanje lisebenza ngendlela eyahlukileyo ngokupheleleyo.
Ngoko ke yintoni le yeza litsha elibizwa ngokuba yiQfitlia (fitusiran)?
Eli liyeza elisebenza ngendlela engaqhelekanga. Besithethe ngaphambili ngenyaniso yokuba igazi lifuna into ebizwa ngokuba yi-thrombin ukuze lijiye. Emizimbeni yethu, kukho enye iproteni elawula ukuveliswa kwale thrombin, oko kukuthi, iyayimisa imveliso ye-thrombin engaphezulu. Ibizwa ngokuba yi
-antithrombin .Cinga ngale ndlela. Une-blood clotting factor (thrombin) emzimbeni wakho enceda i-blood clotting yakho. Kodwa kukho enye into (i-antithrombin) eyilawulayo neyithintelayo ukuba isebenze. Kwi-hemophilia, ukuveliswa kwe-factor encedayo (thrombin) kuqala kuphantsi. Ngoko ke xa i-factor ethintelayo (i-antithrombin) isebenza ngokuqhelekileyo, i-blood clotting iyancipha nangakumbi. Oko kwenziwa yi-Qfitlia
kukunciphisa inani le-antithrombin elithintelayo. Emva koko, inani le-thrombin eliveliswa ngumzimba wakho ngokwendalo livunyelwe ukuba lisebenze ngokufanelekileyo ngaphandle kokuthintelwa. Oku kuthetha ukuba isebenzisa inkqubo yomzimba ukukhuthaza i-blood clotting, endaweni yokukunika i-clotting factor oyilahlekileyo. Eli liyeza lokuqala lokunciphisa i-antithrombin elisetyenziswa kunyango lwe-hemophilia.
Masichaze ngakumbi umahluko phakathi kwezi ndlela zimbini zonyango.
Ungawuqonda ngokulula lo mahluko kwitheyibhile engezantsi.
| Uphawu | Unyango lwendabuko (Ukutshintshwa kwezinto) | Unyango lwe-Qfitlia (fitusiran) |
|---|
| Ukusebenza kakuhle | Into yokuqunjelwa engabikho emzimbeni inikwa ngaphandle. | Iyanciphisa i-antithrombin, ethintela ukujiya kwegazi, kwaye ivumela umzimba ukuba usebenzise i-thrombin yawo ngakumbi. |
| Indlela yokunika | Idla ngokufakwa ngemithambo yegazi. | Ngaphantsi kolusu (ngaphantsi kwesikhumba). |
| Izihlandlo | Kungafuneka amaxesha amaninzi ngeveki. | Kanye ngenyanga okanye rhoqo emva kweenyanga ezimbini (izihlandlo ezi-6-12 ngonyaka). |
| Kwabo banezithinteli | Unyango oluqhelekileyo lwezinto ezibangela umhlaza alusebenzi, kwaye kufuneka ezinye iindlela zonyango ezikhethekileyo (ii-bypassing agents). | Olu nyango lungasetyenziswa ngempumelelo nakubantu abanezithinteli (ii-antibodies ezichasene nezinto ezibangela le ngxaki). |
Ingaba eli yeza lifanelekile kum kwaye ndingalisebenzisa njani?
Ukuba une-hemophilia A okanye B kwaye uneminyaka engaphezu kwe-12 ubudala,
ugqirha wakho unokuqwalasela ukuba eli yeza likulungele na. Eyona nto ibalulekileyo kukuba lingasetyenziswa nakubantu abaye bavelisa izithinteli (ukuxhathisa) unyango lwangaphambili. Nangona kunjalo, ngaphambi kokuba uqale olu nyango, ugqirha wakho uya kwenza
uvavanyo lwegazi oluninzi. 1.
Inqanaba le-Antithrombin: Eli lilinganisa ukuba ingakanani i-antithrombin egazini lakho. Umthamo we-Qfitlia umiselwa ngokusekwe kweli xabiso. 2.
Ukusebenza kwesibindi: Isibindi sakho sijongwa ngaphambi nangexesha lonyango. Eli yeza linikwa
njengenaliti phantsi kolusu (ngaphantsi kolusu). Oku kuthetha ukuba alinikwa emthanjeni. Wena okanye umntu onyamekela umntu unokukunika lona ekhaya emva koqeqesho. Linikwa kulusu lwesisu, ithanga, okanye ngasemva kwengalo engasentla. Kufuneka uziphephe iindawo ezinemivumbo okanye amanxeba. Ukuba sele uthatha ezinye iindlela zonyango ze-hemophilia, unokufuna ukuyeka okanye ukutshintsha ezo ndlela zonyango emva kokuqala i-Qfitlia.
Ugqirha wakho uya kukuchazela ngokucacileyo oku.
Ungaze uyeke ukusebenzisa amayeza akho angoku okanye uyeke ukusebenzisa i-Qfitlia ngaphandle kwengcebiso kagqirha wakho. Ukwenza njalo kunokunyusa umngcipheko wakho wokopha.
Ngaba kufuneka siqaphele nemiphumo emibi? Singazithintela njani?
Njengayo nayiphi na imithi, iQfitlia inokuba nemiphumo emibi. Kubalulekile ukuba uzazi kwaye uthathe amanyathelo okuzikhusela.
- Amahlwili Egazi : Ngenxa yokuba eli yeza likhuthaza ukujiya kwegazi, kunqabile kakhulu ukuba amahlwili egazi angafunekiyo avele ngaphakathi emithanjeni. Ugqirha wakho uya kumisela idosi efanelekileyo yenqanaba le-antithrombin yakho ukunciphisa lo mngcipheko.
Kubaluleke kakhulu ukuba uhlolwe igazi rhoqo njengoko ugqirha wakho ecebisa. Amanqanaba e-antithrombin adla ngokuhlolwa kwinyanga yokuqala, yesithathu, yesihlanu, neyesithandathu yonyango. Le yindlela enokufunyanwa ngayo idosi efanelekileyo yamayeza kunye nendlela yokunciphisa lo mngcipheko.
- Iienzymes zesibindi eziphakamileyo: Abanye abantu basenokuba neeenzymes zesibindi eziphezulu. Yiyo loo nto oogqirha behlala bejonga imeko yesibindi ngovavanyo lwegazi .
- Amatye enyongo kunye nokudumba kwenyongo: Ukuba ngequbuliso ufumana iimpawu ezifana nentlungu eqatha yesisu, isicaphucaphu, kunye nokuhlanza , yazisa ugqirha wakho ngoko nangoko.
- Izifo: Kukho umngcipheko omncinci wokosuleleka kwindawo efakwe kuyo inaliti. Ngoko ke, kubalulekile ukuhlala ufaka inaliti ngendlela ecocekileyo nanjengoko kuyalelwe ngugqirha wakho. Iindlela ezilungileyo zococeko, ezifana nokuhlamba izandla rhoqo, nazo zibalulekile.
Izifundo zibonise ukuba abantu abane-hemophilia (abo bane-inhibitors nabangenazo) abathathe i-Qfitlia babene-episodes ezimbalwa kakhulu zokopha kunabo bangazange bathathe naluphi na unyango lokuthintela. Kwakhona, abantu abane-inhibitors abatshintshele kwezinye iindlela zonyango ukuya kwi-Qfitlia babene-episodes ezimbalwa zokopha. Kodwa khumbula ukuba ezi ziphumo zinokuhluka kumntu nomntu.
Umyalezo Wokuya Ekhaya
- I-Qfitlia (fitusiran) lunyango olutsha lwe-hemophilia A kunye ne-B olusebenza ngendlela eyahlukileyo.
- Oku kusebenza ngokunciphisa ubungakanani be-antithrombin, ethintela ukujiya kwegazi, endaweni yokunika umzimba amandla okujiya aphantsi ngaphandle.
- Eli yeza liyinaliti phantsi kolusu, hayi emthanjeni, kwaye lidla ngokunikezelwa rhoqo, njengokuba kube kanye ngenyanga okanye rhoqo kwiinyanga ezimbini.
- Ugqirha wakho uya kugqiba ukuba olu nyango lukulungele na emva kovavanyo lwegazi oluninzi.
- Ukuze uphephe iziphumo ebezingalindelekanga, kubalulekile ukuba kwenziwe uvavanyo lwegazi (ingakumbi i-antithrombin kunye nokusebenza kwesibindi) rhoqo njengoko ugqirha wakho ekumisele.
- Kuphephe ukuyeka unyango lweQfitlia ngaphandle kwengcebiso kagqirha nangasiphi na isizathu.
I-Hemophilia, i-Hemophilia, i-Qfitlia, i-fitusiran, ukujiya kwegazi, ukopha, into ejiya igazi, i-antithrombin, unyango lwe-hemophilia, amayeza amatsha, iziphumo ebezingalindelekanga
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