Ungave usati wambonzwa nezvechirwere cheFabry. Chirwere chinowanzoitika, sezvo chiri chirwere chisingawanzoitiki chemajini. Asi kana iwe kana mumwe munhu mumhuri yako akaonekwa kuti anacho, zvinonzwisisika kunzwa wakaremerwa. Asi yeuka, kunyange pasina mushonga wechirwere ichi, kune mishonga yakawanda yakanaka iripo nhasi inogona kukubatsira kurarama hupenyu hwakajairika sezvinobvira. Ngatitaurei nezvemishonga iyi nhasi.
Nzira huru dzekurapa: Kurapa neEnzyme
Zvichitaurwa zviri nyore, chirwere cheFabry chinokonzerwa nekushaikwa kana kusashanda zvakanaka kweenzyme inokosha mumuviri wedu. Saka chinangwa chikuru chekurapa ndechekugadzirisa dambudziko iri. Pane nzira mbiri huru dzekuita izvi.
1. Kurapa Kutsiva MaEnzyme (ERT)
Izvi zvinosanganisira kutsiva enzyme inonzi `alpha-galactosidase A (alpha-Gal A)` iyo isipo mumuviri wako. Zvakafanana nekuwedzera mafuta mumotokari kana mafuta ari mashoma. Kurapa uku kunoitwa kuburikidza netsinga (infusion ye `IV`). Kazhinji kunoitika mavhiki maviri ega ega . Mushonga mukuru uri kubvumidzwa parizvino i`agalsidase beta (Fabrazyme)`.
Kunyanya kana murume akaonekwa aine chirwere ichi, chiremba anogona kukurudzira kuti atange kurapwa uku nekukasika, kunyangwe pasina zviratidzo. Chikonzero cheizvi ndechekuti kunyangwe usinganzwe kusagadzikana, nhengo dzemukati dzakadai seitsvo nemoyo dzinogona kunge dzatotanga kukuvara. Saka kutanga kurapwa nekukurumidza kunogona kudzivirira kukuvara kwenhengo idzodzo .
Vakadzi vanowanzo batwa nechirwere cheFabry zvishoma. Saka chiremba wako anogona kumirira kusvika watanga kuratidza zviratidzo kana kusvika bvunzo dzaratidza kuti nhengo dzako dzakuvara. Vamwe vanhu vanogona kunzwa dzihwa nefivha pavanenge vachitora mushonga uyu. Asi usazvidya moyo, chiremba wako achakupa mushonga wekudzivirira chirwere ichi kuti ubatsire kudzivirira izvi.
2. Mishonga yemuromo
Uyu mushonga mutsva. Piritsi iri rinonzi `Migalastat (Galafold).` Rinoshanda nekubatsira enzyme yemuviri wako `alpha-Gal A`, iyo isiri kushanda zvakanaka, kuti ishande zviri nani. Zviri nyore kunwa piritsi pane kuribaya mutsinga.
Asi hazvishandi kune wese munhu. Mushonga uyu unoshanda muvanhu vasingasviki hafu vane chirwere cheFabry. Kushanda kwawo kana kusashanda zvinoenderana nekuchinja kwakati (magene mutations) mumajini ako. Saka usati watanga kurapwa uku, chiremba wako achaita bvunzo yemajini.
Nzira yekudzora zviratidzo?
Chirwere cheFabry chinogona kukonzera matambudziko akasiyana-siyana. Chinonyanya kuitika kuzvimba kwemakumbo, chirwere chemwoyo, uye chirwere cheitsvo. Saka pamusoro pekurapa nemaenzayimu, chiremba wako angangokupa mimwe mishonga yekubatsira kudzora zviratidzo izvi.
| Chiratidzo/Dambudziko | Kurapa kunowanzo piwa |
|---|---|
| Kuzvimba kwakanyanya uye kurwadziwa mumaoko nemakumbo | Mishonga yakaita secarbamazepine, gabapentin, kana phenytoin, iyo inowanzopihwa kune vanhu vane pfari, inopihwa kudzora marwadzo. |
| Kukuvara kweitsvo uye BP yakakwira | Mishonga yeBP yakaita seACE inhibitors inonyorerwa. Iyi inobatsira kudzivirira itsvo uku ichidzora BP. |
| Kusarongeka kwekurova kwemoyo | Mimwe mishonga yemwoyo ingangodiwa kuti mwoyo urambe uchishanda zvakanaka. |
| Matambudziko ekugaya chikafu (kurutsa, manyoka, kurwadziwa mudumbu) | Chiremba achanyora mishonga yakakodzera zvichienderana nezviratidzo izvi. |
| Matambudziko eganda (kuonekwa kwetsinga diki dzeropa) | Chiremba weganda anogona kubvisa izvi nekurapwa kuri nyore. |
| Matambudziko ekunzwa (kurira munzeve, dzungu, kurasikirwa nekunzwa) | Matambudziko aya anogona kudzivirirwa nekushandisa zvinhu zvinobatsira kunzwa. |
Miedzo inoongorora mamiriro echirwere
Nekuti chirwere cheFabry chinokanganisa muviri wako wese, zviratidzo zvinogona kuipa sezvaunokura. Zvakakosha kugara uchiziva nezveutano hwako. Izvi zvinosanganisira kuongororwa nachiremba nguva dzose .
- Kuongororwa kweropa: Izvi zvinobatsira kuona huwandu hwemasero matsvuku nemachena eropa, mazinga eelectrolyte, uye mashandiro ari kuita itsvo nechiropa.
- Kuongororwa kweweti: Tarisa kana paine mapuroteni kana ropa muweti. Ichi chinogona kuva chiratidzo chekutanga chechirwere cheitsvo.
- ECG (Electrocardiogram): Izvi zvinonyora zviratidzo zvemagetsi zvemoyo uye zvinoongorora kurova kwemoyo kusina kujairika.
- Echocardiogram: Mufananidzo wemoyo unotorwa uchishandisa mafungu eruzha. Izvi zvinogona kushandiswa kutarisa matambudziko ari mumavharuvhu nemakamuri emoyo.
- Kuongororwa kwekunzwa: Ipfungwa yakanaka kuti bvunzo iyi iitwe kanenge kamwe chete pagore.
- Kufungidzira uropi: Kuongororwa uku, kunoitwa makore maviri ega ega, kunotsvaga shanduko mutsinga dzeropa muuropi. Izvi zvinogona kubatsira kuona zviratidzo zvekutanga zvestroke.
Chikwata chekurapa chinokubatsira
Mumamiriro ezvinhu akadai, chiremba wemhuri yako haasi iye ega achakurapa. Chikwata chenyanzvi dzakasiyana-siyana chichakubatsira. Zvichienderana nezviratidzo zvako, ungangoda kuona nyanzvi idzi kanenge kamwe chete pagore.
- Chiremba wepfungwa: Kune matambudziko ane chekuita neuropi nehurongwa hwetsinga.
- Chiremba wemwoyo: Kune matambudziko ane chekuita nemoyo.
- Chiremba wepfungwa: Kune matambudziko ane chekuita neitsvo.
- Chiremba wemaziso: Kune matambudziko ane chekuita nemaziso.
Mharidzo Yekuenda Nayo Kumba
- Kunyange hazvo chirwere cheFabry chisingarapike zvachose, chinogona kurapwa zvinobudirira nekurapwa kuri kuitwa pari zvino uye vanhu vanogona kurarama hupenyu hwakajairika.
- Kune nzira mbiri huru dzekurapa: kurapa ne intravenous enzyme therapy (ERT) uye mapiritsi emuromo (Migalastat). Chiremba wako ndiye achasarudza kuti ndeipi yakakunakira.
- Kudzora zviratidzo zvakaita sekurwadziwa, chirwere chemwoyo, uye chirwere cheitsvo kwakakosha zvikuru pakuvandudza hupenyu.
- Zvakakosha kuongororwa nachiremba nenguva uye kugara uchisangana nechikwata chevanachiremba vane hunyanzvi.
- Taura pachena uye nekuvimbika nachiremba wako nezvechirwere chako, nzira dzekurapa nadzo, uye chero zvinokunetsa. Usatya, vanogara vakagadzirira kukubatsira.
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