Uma une-hemophilia, cishe uyazi ukuthi kungakhungathekisa futhi kwesabise kangakanani ukopha njalo, ngezinye izikhathi ngaphandle kwesizathu, futhi ube nenkinga yokuvimba
ukopha ngisho noma kuncane kakhulu. Kungenzeka ukuthi usuvele uthatha imithi yokwelapha ukopha kwakho, noma ungase uthatha imithi yokuvimbela. Kodwa-ke, ngokuthuthuka kwesayensi yezokwelapha, manje kunezindlela eziningi zokwelapha ezintsha nezisebenzayo zalesi simo. Ngakho-ke namuhla, ake sixoxe ngomuthi omusha, ohlukile. Ubizwa ngokuthi i-Qfitlia (fitusiran).
Okokuqala, ake sibone, kungani ukopha kungayeki kubantu abane-hemophilia?
Kalula nje, kunezinhlobo ezikhethekile zamaprotheni ezisiza ekuqhekekeni kwegazi. Lokhu sikubiza ngokuthi
" izici zokujiya ." Njengoba nje kudingeka udaka ukuze kubanjwe izitini lapho kwakhiwe udonga, lezi zici zokujiya kwegazi zibalulekile ukuze kumiswe ukopha. Umuntu one-hemophilia akakwazi ukugaya igazi kahle.
- Abantu abane -hemophilia A abanayo into ebizwa ngokuthi i-Factor VIII.
- Abantu abane -hemophilia B abanayo into ebizwa ngokuthi i-Factor IX.
Uma lezi zici zincipha, ukukhiqizwa kwesinye isinyathelo esibalulekile enkambisweni yokujiya kwegazi, okubizwa ngokuthi
i-thrombin , kuyancipha. I-Thrombin iyisici esiyinhloko esenza ukuthi igazi lijiye futhi liyeke ukuphuma kwegazi. Ngakho-ke lapho i-thrombin incipha, kuba nzima ukuyeka ukuphuma kwegazi uma kwenzeka. Uyaqonda? Ngokuvamile, lokho ebesikwenza njengokwelashwa isikhathi eside kangaka ukwenza ngokwenziwa i-clotting factor (isici 8 noma 9) entulekayo emzimbeni wakho bese uyinika
njengomjovo emthanjeni. Kodwa umuthi we-Qfitlia esikhuluma ngawo namuhla usebenza ngendlela ehluke ngokuphelele.
Ngakho-ke uyini lo muthi omusha obizwa ngokuthi i-Qfitlia (fitusiran)?
Lona umuthi osebenza ngendlela exakile kakhulu. Sikhulume ngaphambilini ngeqiniso lokuthi igazi lidinga into ebizwa ngokuthi i-thrombin ukuze lijiye. Emizimbeni yethu, kukhona enye iphrotheni elawula ukukhiqizwa kwale thrombin, okungukuthi, iyamisa ukukhiqizwa kwe-thrombin eyengeziwe. Ibizwa ngokuthi
i-antithrombin .Cabanga ngale ndlela. Une-blood clotting factor (thrombin) emzimbeni wakho esiza i-blood clotting yakho. Kodwa kukhona esinye isici (i-antithrombin) esilawula futhi siyivimbele ekusebenzeni. Ku-hemophilia, ukukhiqizwa kwe-factor esiza (i-thrombin) kuqala kuphansi. Ngakho-ke lapho i-factor evimbela (i-antithrombin) isebenza ngokujwayelekile, i-blood clotting iyancipha kakhulu. Lokho okwenziwa yi-Qfitlia
ukunciphisa inani le-antithrombin eliyivimbelayo. Ngemuva kwalokho, inani le-thrombin elikhiqizwa ngumzimba wakho ngokwemvelo livunyelwe ukusebenza kahle ngaphandle kokuvinjelwa. Lokhu kusho ukuthi isebenzisa uhlelo lomzimba ukukhuthaza i-blood clotting, kunokukunikeza i-clotting factor oyilahlile. Lona umuthi wokuqala wokunciphisa i-antithrombin osetshenziswa ekwelapheni i-hemophilia.
Ake sichaze kabanzi umehluko phakathi kwalezi zindlela ezimbili zokwelapha.
Ungawuqonda kalula lo mehluko kusukela kuthebula elingezansi.
| Isici | Ukwelashwa Kwendabuko (Ukufakwa Kwezinto Ezithile) | Ukwelashwa kwe-Qfitlia (fitusiran) |
|---|
| Ukusebenza kahle | Isici sokujiya kwegazi esingekho emzimbeni sinikezwa ngaphandle. | Inciphisa i-antithrombin, evimbela ukujiya kwegazi, futhi ivumela umzimba ukuthi usebenzise i-thrombin yawo kakhulu. |
| Indlela yokunikeza | Ngokuvamile ngemithambo yegazi. | Ngaphansi kwesikhumba (ngaphansi kwesikhumba). |
| Imvamisa | Kungadingeka izikhathi eziningana ngesonto. | Kanye ngenyanga noma njalo ezinyangeni ezimbili (izikhathi ezingu-6-12 ngonyaka). |
| Kulabo abanezithibi | Ukwelashwa okuvamile kwezinto akusebenzi, futhi kudingeka ezinye izindlela zokwelapha ezikhethekile (ama-ejenti okudlula) | Lokhu kwelashwa kungasetshenziswa ngempumelelo nakubantu abanezithibi (ama-antibodies alwa nezici). |
Ingabe lo muthi ungifanele futhi ngiwusebenzisa kanjani?
Uma une-hemophilia A noma B futhi uneminyaka engaphezu kwengu-12 ubudala,
udokotela wakho angase acabangele ukuthi lo muthi ukulungele yini. Into ebaluleke kakhulu ukuthi ungasetshenziswa nakubantu asebethole izindlela zokuvimbela (ukumelana) nokwelashwa kwangaphambilini kwezinto. Kodwa-ke, ngaphambi kokuqala lokhu kwelashwa, udokotela wakho uzokwenza
ukuhlolwa kwegazi okuningana. 1.
Izinga le-Antithrombin: Lokhu kulinganisa ukuthi ingakanani i-antithrombin egazini lakho. Umthamo we-Qfitlia unqunywa ngokusekelwe kuleli nani. 2.
Ukusebenza kwesibindi: Isibindi sakho siqashwe ngaphambi nangesikhathi sokwelashwa. Lo muthi unikezwa
njengomjovo ngaphansi kwesikhumba (ngaphansi kwesikhumba). Lokhu kusho ukuthi awunikezwa emthanjeni. Wena noma umnakekeli ungakunika wona ekhaya ngemva kokuqeqeshwa. Unikezwa esikhumbeni sesisu, ethangeni, noma ngemuva kwengalo engenhla. Kufanele ugweme izindawo ezinemivimbo noma ukusikeka. Uma usuvele uthatha ezinye izindlela zokwelapha ze-hemophilia, kungadingeka uyeke noma ushintshe lezo zindlela zokwelapha ngemva kokuqala i-Qfitlia.
Udokotela wakho uzokuchazela lokhu ngokucacile.
Ungalokothi uyeke ukuthatha imithi yakho yamanje noma uyeke ukuthatha i-Qfitlia ngaphandle kweseluleko sikadokotela wakho. Ukwenza kanjalo kungandisa ingozi yakho yokopha.
Ingabe kufanele futhi siqaphele imiphumela emibi? Singayivimbela kanjani?
Njenganoma yimuphi umuthi, i-Qfitlia ingaba nemiphumela emibi. Kubalulekile ukuzazi futhi uthathe izinyathelo zokuzivimbela.
- Amahlule Egazi : Ngenxa yokuthi lo muthi ukhuthaza ukujiya kwegazi, akuvamile kakhulu ukuthi amahlule egazi angafuneki akheke ngaphakathi kwemithambo yegazi. Udokotela wakho uzonquma umthamo ofanele wezinga lakho le-antithrombin ukuze anciphise le ngozi.
Kubaluleke kakhulu ukuhlolwa kwegazi njalo njengoba udokotela wakho encoma. Amazinga e-antithrombin avame ukuhlolwa ngenyanga yokuqala, yesithathu, yesihlanu, neyesithupha yokwelashwa. Lena yindlela umthamo ofanele wemithi onganqunywa ngayo kanye nendlela yokunciphisa le ngozi.
- Ama-enzyme esibindi aphezulu: Abanye abantu bangase babe nama-enzyme esibindi aphezulu. Yingakho odokotela behlola njalo isimo sesibindi ngokuhlolwa kwegazi .
- Amatshe enyongo kanye nokuvuvukala kwenyongo: Uma uhlangabezana nezimpawu ezinjengobuhlungu obukhulu besisu, isicanucanu, nokuhlanza , yazisa udokotela wakho ngokushesha.
- Ukutheleleka: Kukhona ingozi encane yokutheleleka endaweni yokujova. Ngakho-ke, kubalulekile ukujova njalo ngendlela ehlanzekile futhi njengoba kuqondiswe udokotela wakho. Imikhuba emihle yokuhlanzeka, njengokugeza izandla njalo, nayo ibalulekile.
Ucwaningo lubonise ukuthi abantu abane-hemophilia (kokubili labo abane-inhibitors nabangenayo) abathathe i-Qfitlia babeneziqephu ezimbalwa kakhulu zokopha kunalabo abangazange bathathe noma yiluphi uhlobo lokwelashwa lokuvimbela. Futhi, abantu abane-inhibitors abashintshele kwezinye izindlela zokwelapha baya e-Qfitlia babeneziqephu ezimbalwa zokopha. Kodwa khumbula ukuthi le miphumela ingahluka kuye ngomuntu nomuntu.
Umlayezo Wokuya Nawe Ekhaya
- I-Qfitlia (fitusiran) iyindlela entsha yokwelapha i-hemophilia A ne-B esebenza ngendlela ehlukile.
- Lokhu kusebenza ngokunciphisa inani le-antithrombin, elivimbela ukujiya kwegazi, esikhundleni sokunikeza umzimba amandla okujiya aphansi ngaphandle.
- Lo muthi uwumjovo ngaphansi kwesikhumba, hhayi emthanjeni, futhi uvame ukunikezwa kaningi, njengakanye ngenyanga noma njalo ezinyangeni ezimbili.
- Udokotela wakho uzonquma ukuthi lokhu kwelashwa kulungile yini kuwe ngemva kokuhlolwa kwegazi okuningana.
- Ukuze ugweme imiphumela emibi, kubalulekile ukuhlolwa kwegazi (ikakhulukazi i-antithrombin kanye nokusebenza kwesibindi) ngezikhathi ezithile njengoba kunqunywe udokotela wakho.
- Gwema ukuyeka ukwelashwa kwe-Qfitlia ngaphandle kweseluleko sezokwelapha nganoma yisiphi isizathu.
I-Hemophilia, i-Hemophilia, i-Qfitlia, i-fitusiran, ukujiya kwegazi, ukopha, isici sokujiya kwegazi, i-antithrombin, ukwelashwa kwe-hemophilia, umuthi omusha, imiphumela emibi
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