Kana iwe kana mumwe munhu waunoziva ane chirwere cheFabry, pamwe wakambonzwa nezvemushonga unonzi Galafold. Ndiwo mushonga wekutanga uye wega unonwiwa wechirwere cheFabry. Mimwe mishonga yekurapa chirwere cheFabry, yakadai se enzyme replacement therapy, inopihwa nejekiseni. Galafold imhando yekurapa kwe "chaperone" inoshanda nenzira yakasiyana zvachose. Haishande kune marudzi ese echirwere cheFabry. Zvisinei, inogona kubatsira zvikuru kune vanhu vane mamwe machinjiki mumajini anokonzera chirwere ichi.
Chii chinonzi chirwere cheFabry? Galafold inoshanda sei?
Zvichitaurwa zviri nyore, chirwere cheFabry chirwere chisingawanzoitiki chemajini chinopfuudzwa kubva kumubereki kuenda kumwana. Vanhu vane chirwere cheFabry vane enzyme inonzi alpha-galactosidase A (alpha-gal) mumiviri yavo. Asi enzyme iyi haishande nemazvo. Izvi zvinokonzerwa nekuti pane shanduko mujini inoiudza kuti igadzire enzyme iyi.
Alpha-gal ienzyme inokosha zvikuru inowanikwa mumasero akawanda mumuviri wedu. Basa rayo guru nderekuputsa chinhu chine mafuta chinonzi GL-3 . Saka, kana iyi enzyme ikasashanda zvakanaka, chinhu chinonzi GL-3 chinotanga kuungana mukati memasero emuviri. Nekufamba kwenguva, kuunganidzwa uku kunogona kukonzera kukuvara kukuru kunhengo dzakakosha dzemuviri, dzakadai seitsvo , moyo , ganda, uropi, uye tsinga .
Kune mhando mbiri huru dzechirwere cheFabry.
| Rudzi rwechirwere (Rudzi) | Tsananguro & Zviratidzo |
|---|---|
| Rudzi rwekare | Iyi ndiyo mhando yakanyanya yechirwere ichi. Zviratidzo zvinowanzoonekwa muhudiki. Unogona kunzwa marwadzo, akadai sekutsva uye kusanzwa simba mumaoko netsoka, matambudziko emudumbu nekugaya chikafu, uye ganda rine mavara matsvuku-pepuru pakati peguvhu nemabvi. Vamwe vanhu vanogonawo kunzwa kudikitira kushoma, ganda rakaoma, uye kunzwa kupisa kwakanyanya. |
| Rudzi rwekutanga kunonoka | Urwu ndirwo rudzi rwechirwere cheFabry rwakajairika. Chiri nyore kupfuura rudzi rwekare. Kunyangwe chirwere ichi chiripo kubva pakuzvarwa, zviratidzo hazvionekwi pahudiki. Pane kudaro, zviratidzo zvinotanga kuonekwa mumakore ekuma30 kana kuti gare gare. |
Sezvo vanhu vane chirwere cheFabry vachikura, GL-3 inoramba ichiungana mumasero avo. Izvi zvinogona kukonzera matambudziko makuru ehutano nekufamba kwenguva. Vanhu vazhinji vanotanga kuva nematambudziko eitsvo, izvo zvinogona kupedzisira zvaita kuti itsvo dzisashande zvakanaka . Chirwere ichi chinogonawo kukuvadza moyo netsinga dzeropa , zvichiwedzera njodzi yekurwara nemoyo kana sitiroko.
Chinangwa chikuru chekurapa chirwere cheFabry ndechekupa muviri enzyme inoshanda ye'alpha-gal'. Kunyange zvazvo izvi zvisingadzore kukuvara kwatoitika, zvinobatsira kudzivirira kukuvara mune ramangwana.
Vanhu vazhinji vanopihwa Enzyme Replacement Therapy (ERT) yeizvi. Izvi zvinosanganisira kubaya jekiseni reenzyme inoshanda zvakanaka rinogadzirwa kunze mumuviri. Muviri unokwanisa kushandisa enzyme iyoyo kuputsa GL-3.
Zvisinei, Galafold inoshanda nenzira yakasiyana zvachose. Chinhu chinoshanda muGalafold mushonga unonzi `migalastat`. Chaunoshanda ndechekuti, unonamatira kune enzyme ye`alpha-gal` iri mumuviri wemurwere, asi haina kugadzikana. Fungidzira kuti enzyme ye`alpha-gal` mumuviri wako 'yakazvimba', 'isina kugadzikana'. Saka, haigone kushanda nemazvo. Zvinoitwa nemushonga unonzi Galafold, seshamwari, unonamatira kune iyi enzyme 'yakazvimba' uye 'inoisimbisa'. Zvadaro enzyme iyoyo inogona kuita basa rayo nemazvo. Izvi ndizvo zvatinodaidza kuti "Chaperone Therapy" .
Munhu wese anogona kushandisa Galafold here?
Kwete. Galafold inogona kushandiswa chete kuvanhu vane rudzi rwakasiyana rwekuchinja mujini rinogadzira enzyme inonzi `alpha-gal`. Iyo enzyme inonzi `alpha-gal` inogadzirwa nekuchinja uku ichiri kukwanisa kushanda, asi haina kugadzikana zvakanyanya kuti ishande nemazvo. Galafold inonamatira kune iyo enzyme, inoisimbisa, uye inoita kuti ishande.
Vamwe varwere veFabry havagadziri enzyme inonzi `alpha-gal` zvachose, kana kuti enzyme inogadzirwa haishande zvachose. Galafold haizobatsiri vanhu vakadaro. Nekuti hapana enzyme mumuviri inogadzirisa mamiriro acho. Enzyme replacement therapy (ERT) ndiyo inonyanya kukodzera kwavari.
Saka, chiremba wako asati akupa Galafold mvumo yekurapa, achaita bvunzo yemajini kuti aone kuti rudzii rwekuchinja kwemajini kwaunako. Izvi zvichaona kana mushonga uyu uchizokubatsira kana kuti kwete.
Mushonga uyu munoshandiswa sei?
Chikamu ichi chakakosha zvikuru nekuti kuti uwane rubatsiro rwakakwana rwemushonga, unofanirwa kuushandisa nemazvo.
- Maitiro ekutora: Galafold ikapusule. Inotorwa mazuva ese maviri , kureva kuti, mazuva ese maviri.
- Nguva yezuva: Zvakakosha zvikuru kuitora panguva imwe chete zuva nezuva. Semuenzaniso, kana ukaitora na8am pazuva rekutanga, unofanira kuitora na8am pazuva rinotevera zvakare.
- Chinonyanya kukosha: Mushonga uyu unofanira kutorwa usina chinhu mudumbu . Izvi zvinoreva kuti haufanirwe kudya chero chinhu kwemaawa maviri usati watora mushonga kana maawa maviri mushure mekutora mushonga.
- Chimwe chinhu chakakosha: Zvakare, usanwe chero chinhu chine caffeine (senge tii, kofi, zvinwiwa zvisina kunyorova) kwemaawa maviri usati wanwa piritsi uye maawa maviri wanwa piritsi. Munguva iyi, unogona kunwa mvura, zvinwiwa zvemichero zvisina fiber, kana zvinwiwa zvine caffeine.
Kuti zvikubatsire kudzivirira kusanganisa mazuva ako, mushonga uyu unouya uri mupakeji yakakosha. Unonzi 'kadhi remabhuruku.' Une nzira yekuisa mazuva pamakadhi. Unobvisa kadhi remabhuruku pazuva raunotora mushonga, uye pazuva rausingatore mushonga, unobvisa denderedzwa risina chinhu panzvimbo yaro. Nenzira iyi, unogona kuisa makadhi zuva rega rega uye usapotsa zuva raunoda kutora mushonga.
Pane migumisiro yakaipa uye matambudziko here nemimwe mishonga?
Vamwe vanhu vanotora Galafold vanogona kunge vaine njodzi yakawedzera zvishoma yezvirwere zvekufema (dzihwa, pahuro) uye zvirwere zvemunzira yeweti. Nzira yakanakisa yekudzivirira hutachiona ndeyekugeza maoko ako nguva nenguva, kugara kure nevanhu vanorwara, uye kunwa mvura yakawanda.
Chinhu chinonyanya kukosha ndechekuti, kana ukaona zviratidzo zvinonetsa uye zvisingawanzoitika zvaunofunga kuti zvinokonzerwa nemushonga uyu, usamborega kuutora wega . Iva nechokwadi chekutaura nachiremba wako.
Pamusoro pezvo, sezvatakambokurukura, caffeineSaka, Galafold inogona kunge isingashande zvakanaka. Saka chenjerera zvinhu zvine caffeine. Zvakakoshawo kuzivisa chiremba wako nezvemimwe mishonga, mavhitamini, kana mishonga yechivanhu yauri kutora.
Mharidzo Yekuenda Nayo Kumba
- Galafold mushonga wemuromo wakakosha wemhando dzakasiyana dzechirwere cheFabry.
- Izvi zvinoshanda nekusimbisa enzyme iri mumuviri wako, asi isina kugadzikana, uye zvichiita kuti ishande. Unofanira kuita bvunzo yemajini kuti uone kana izvi zvakakunakira.
- Mushonga uyu unofanira kutorwa mazuva ese maviri, usina chinhu mudumbu. Dzivisa kudya nekunwa zvinwiwa zvine caffeine maawa maviri usati watora mushonga uye maawa maviri mushure mekutora mushonga.
- Mushonga uyu ungasavandudzira zviratidzo zvako zvemazuva ano, asi uchabatsira kudzivirira kukuvara kunokonzerwa nechirwere mune ramangwana.
- Kana uine zvimwe zvinokanganisa kana matambudziko, taura nachiremba wako nekukasika.
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