Wakambonzwa nezveGaucher Disease here? Pamwe kwete. Nekuti chirwere chisingawanzoitiki, hachisi chirwere chinowanzoitika munyika medu. Asi sezvo chiri chirwere chemajini chinopfuudzwa kubva kuchizvarwa kuenda kuchizvarwa, zvakakosha zvikuru kuziva nezvacho. Chirwere ichi chinogona kukonzera zviratidzo zvakasiyana-siyana, kubva pamapfupa asina simba kusvika pakuva neruvara rudiki kunyangwe paine kukuvara kudiki.
Zvichitaurwa zviri nyore, chii chinonzi chirwere cheGaucher?
Zvakanaka, ngatizvitaure seizvi. Kune enzyme yakakosha mumuviri wedu inobatsira kuputsa nekubvisa mamwe marudzi emafuta. Mumuviri wemunhu ane chirwere cheGaucher, iyi enzyme haishande nemazvo. Zvino zvinoitika ndezvekuti marudzi iwayo emafuta anochengetwa munzvimbo dzakasiyana dzemuviri, kunyanya muchiropa, muspleen, nemumafupa . Kana mafuta akaungana nenzira iyi, matambudziko akasiyana-siyana ehutano anotanga kumuka.
Parizvino hapana mushonga wechirwere ichi, asi zvichienderana nerudzi rwechirwere cheGaucher chaunacho, kune mishonga yakanaka kwazvo yekudzora zviratidzo.
Kune marudzi matatu makuru echirwere cheGaucher:
1. Rudzi rwekutanga: Rudzi urwu ndirwo rwakajairika. Harukanganisi tsinga dzemuviri. Zviratidzo zvinogona kunge zviri zvishoma kune vamwe vanhu, asi zvinogona kunge zvakanyanya kune vamwe. Dzimwe nguva panogona kunge pasina zviratidzo zvachose. Kune mishonga yakanaka yerudzi urwu.
2. Rudzi rwechipiri nerudzi rwechitatu: Mhando dzese idzi dzakakomba kupfuura rudzi rwekutanga nekuti dzinokanganisa sisitimu yetsinga, iyo iri uropi nemusana. Vacheche vane rudzi rwechipiri havawanzo rarama makore anopfuura maviri. Rudzi rwechitatu runokuvadzawo uropi, asi zviratidzo zvinoonekwa gare gare muhudiki.
Chii chinokonzera chirwere cheGaucher?
Ichi hachisi chirwere chinogona kutapuriranwa kubva kumunhu kuenda kumunhu sedzihwa. Ichi chirwere chemajini chinopfuudzwa kubva kuchizvarwa kuenda kune chimwe. Chinokonzerwa nekukanganisika kuri mujini rinonzi GBA.
Fungidzira, mwana anowana chirwere ichi chete kana akagara nhaka jini reGBA risina kunaka kubva kuna amai nababa vake. Kunyangwe kana mumwe munhu asina chirwere ichi, anogona kuramba aine jini iri risina kunaka mumuviri wake (kuva mutakuri) uye oripfuudza kuvana vake.
Chirwere ichi chinonyanya kuwanikwa pakati pevanhu vane dzinza rechiJudha (Ashkenazi Jews) kuEastern neCentral Europe.
Ndezvipi zviratidzo zvechirwere ichi?
Zviratidzo zvinogona kusiyana zvikuru kubva pamunhu kusvika pamunhu. Zvinosiyanawo zvichienderana nerudzi rwechirwere. Ngatitarisei zviratidzo zvine chekuita nerudzi rumwe nerumwe.
| Rudzi rweChirwere | Zviratidzo Zvakajairika |
|---|---|
| Rudzi 1 |
|
| Rudzi rwechipiri (Kune vacheche vari pakati pemwedzi 3-6) | |
| Rudzi rwechitatu (Zvinotanga achiri mudiki) | |
| Mwoyo weGaucher yeMitsipa yeMwoyo (Type 3c) (Rudzi rusingawanzoonekwi) |
Nzira yekuziva sei chirwere ichi?
Paunoenda kunoona chiremba ane zviratidzo izvi, anogona kukubvunza mibvunzo yakaita seiyi:
- Wakatanga rini kuona zviratidzo?
- Mhuri yako inobva kurudzi rupi?
- Pane mumwe munhu muzvizvarwa zvekare zvemhuri akambova nematambudziko ehutano akadai here?
- Pane hama kana mhuri yako yakambofa nevana vari pasi pemakore maviri here?
Kana chiremba wako achifungidzira kuti une chirwere cheGaucher, anogona kuchisimbisa nekuongororwa ropa kana mate . Vanofanirawo kuongororwa nguva nenguva kuti vaone kufambira mberi kwechirwere.
Pamusoro pezvo, MRI scan inogona kuitwa kuti ione kana chiropa kana chirwatata chakazvimba, uye bvunzo yekuwanda kwemapfupa inogona kuitwa kuti ione kusimba kwemapfupa.
Ndeipi mishonga yekurapa chirwere cheGaucher?
Nzira dzekurapa dzinoenderana nerudzi rwechirwere uye kuoma kwezviratidzo. Kana zviratidzo zviri zvishoma, hapana mushonga ungadikanwa.
Nzira huru dzekurapa
- Kurapa Kutsiva Maenzyme (ERT): Uku ndiko kurapa kukuru kune vamwe vanhu vane chirwere cheshuga cherudzi rwekutanga nerwechitatu. Izvi zvinosanganisira kupa muviri enzyme isina kukwana. Mushonga uyu unopihwa netsinga (IV) kanenge kamwe chete pamavhiki maviri ega ega. Unobatsira kuderedza anemia, kusimbisa mapfupa, uye kuporesa spleen nechiropa chakakura. Zvisinei, kurapa uku hakushande zvakanyanya pamatambudziko ane tsinga dzinokanganisa uropi. `Imiglucerase (Cerezyme)` uye `Velaglucerase alfa (VPRIV)` mishonga yerudzi urwu.
- Substrate Reduction Therapy (SRT): Aya mapiritsi anodzora kugadzirwa kwemafuta anoungana mumuviri wechirwere cheGaucher. Eliglustat (Cerdelga) neMiglustat (Zavesca) mishonga yakadaro. Inopihwa kuvarwere vakuru vane chirwere cherudzi rwekutanga vasingakodzeri kurapwa kweERT.
Chinonyanya kukosha ndechekuti, hapana mushonga unokwanisa kumisa kukuvara kweuropi kunokonzerwa nechirwere cheshuga cherudzi rwechitatu, asi vaongorori vari kuramba vachishanda pachiri.
Mamwe marapirwo anotsigira
- Kuwedzerwa ropa kuti uwane chirwere chekushaya ropa
- Mishonga yekusimbisa mapfupa nekuderedza marwadzo
- Kuvhiyiwa kwekutsiva majoini kuti zvivandudze kufamba kwemuviri
- Kuvhiyiwa kwekubvisa chiropa chakakura
- Mishonga yekudzora marwadzo
- Kutora zvimwe zvinovaka muviri zvakaita sevhitamini D necalcium kana ukakurudzirwa nachiremba wako
Kurarama nechirwere ichi uye zvaunotarisira
Nekuti chirwere ichi chakasiyana nemunhu wega wega, unofanirwa kushanda pamwe chete nachiremba wako kuti uwane nzira yekurapa yakakunakira. Kurapwa kunogona kukubatsira kurarama zviri nani uye kuwedzera hupenyu hwako.
Mwana ane chirwere cheGaucher anogona kukura zvishoma nezvishoma kupfuura vamwe vana. Vanogonawo kunge vakanonoka kuyaruka. Zvichienderana nezviratidzo, ungangoda kudzivisa mitambo yakaita sekutamba nevanhu. Vamwe vanhu vangatofanira kushanda nesimba kuti varambe vachiita basa ravo nekuda kwekurwadziwa kwakanyanya uye kuneta.
Kurarama nechirwere chakadai idambudziko, saka zvakakosha kutsvaga rubatsiro kubva kumhuri neshamwari, pamwe nekupa mazano epfungwa kana zvichidikanwa.
Mharidzo Yekuenda Nayo Kumba
- Chirwere cheGaucher chirwere chemajini chinopfuudzwa kubva kumunhu mumwe kuenda kune mumwe, kwete chirwere chinopfuudzwa kubva kumunhu mumwe kuenda kune mumwe.
- Zviratidzo zvinogona kusiyana zvikuru zvichienderana nerudzi rwechirwere uye munhu wacho. Kune vamwe vanhu, zviratidzo zvacho hazvina kunyanya kuoma.
- Zvakakosha zvikuru kuongorora chirwere ichi uye kutanga kurapwa nekukurumidza.
- Kunyangwe paine mishonga inoshanda zvikuru (ERT neSRT) yerudzi rwekutanga nezvimwe zvinhu zverudzi rwechitatu, chirwere ichi hachigone kurapwa zvachose.
- Kana iwe kana mwana wako muine chirwere ichi, zvakakosha kutevedzera hurongwa hwakakodzera hwekurapa nekushanda pamwe chete nachiremba wenyu.
- Kuwana rutsigiro kubva kumhuri nemapoka ekutsigirana kunowedzera simba repfungwa.
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