Kana chiremba wako akakuudza iwe kana mudiwa wako kuti vane chirwere cheGaucher, unogona kunzwa kutya uye kutya. Izvozvo zvakajairika. Asi chinhu chakakosha ndechekuti kune mishonga yakawanda yakanaka iripo nhasi inogona kukubatsira kurarama hupenyu hwakanaka nechirwere ichi. Kunyangwe pasina mushonga wechirwere ichi, mishonga iripo inogona kukubatsira kudzivirira kukuvara kwenhengo dzako uye kugadzirisa zviratidzo zvako kuti ugone kurarama hupenyu hune hutano. Saka usazvidya mwoyo, ngatitaure nezvazvo zvakadzama.
Mamwe marapirwo akakodzera vanhu vakuru chete. Mamwe marapirwo akagadzirirwa mamwe marudzi echirwere cheGaucher. Zvakare, kana iwe kana mwana wako muine zviratidzo zvisina kusimba, mungasada kurapwa zvachose. Kana zvakadaro, mungada kuona kana zviratidzo zvenyu zviri nani uye motanga kurapwa chete kana zvichidikanwa. Izvi zvese zvinosarudzwa nachiremba wenyu.
Nzira huru yekurapa: Enzyme Replacement Therapy (ERT)
Uku ndiko kurapwa kunonyanya kushandiswa uye kunoshanda. Vakuru nevana vane chirwere cheGaucher cherudzi rwekutanga nerwechitatu vanogona kurapwa uku.
Zvichitaurwa zviri nyore, chirwere cheGaucher chirwere chinodzikira simba remuviri rekugadzira enzyme chaiyo. Nekuda kweizvozvo, mamwe mafuta mumuviri haagone kupatsanurwa zvakanaka uye anochengetwa munzvimbo dzakadai sechiropa, spleen, uye bone marrow. Kurapa Kutsiva Enzyme (ERT) kunosanganisira kupa muviri enzyme isipo kunze, sejekiseni.
Kurapa uku kwakafanana nekutsiva girobhu remwenje rakatyoka mumba medu neritsva. Kunobatsira muviri kuti ushande zvakanaka zvakare nekupa izvo zviri kushaikwa kunze.
Ndezvipi zvakanakira kurapwa kweERT?
- Kuvandudza chiropa netsandanyama zvakazvimba: Nhengo idzi, dzakakura nekuda kwechirwere ichi, dzinoitwa diki zvakare uye mashandiro adzo anovandudzwa.
- Kuderedza Anemia: Izvi zvinobatsira kudzora anemia, iyo inokonzera kuneta uye kushaya simba muvanhu vazhinji.
- Kusimbisa mapfupa: Kunoderedza marwadzo emapfupa, kunoasimbisa, uye kunoadzivirira kuti asatyoke nyore nyore.
Zvisinei, zvakakosha kuyeuka kuti ERT haishande zvakanyanya mukutonga zviratidzo zveuropi kana zvetsinga (zvakadai sepfari) zvinoitika muchirwere cherudzi rwechitatu cheGaucher. Izvi zvinodaro nekuti mushonga uyu une simba shoma rekupfuura kubva muropa redu uchienda muuropi (chinodzivirira ropa neuropi).
Kune mhando nhatu huru dzemishonga yeERT dziri kushandiswa pari zvino:
- Imiglucerase (Imiglucerase - Cerezyme)
- Taliglucerase alfa (Taliglucerase alfa - Elelyso)
- Velaglucerase alfa (VPRIV)
Mushonga uyu unopihwa semushonga wesaline (mushonga wemutsinga), kuburikidza nechubhu diki uchipinda mutsinga. Kazhinji unofanira kuenda kuchipatara kana kiriniki mavhiki maviri ega ega kuti uwane kurapwa uku mukati memaawa 1-2.
Mishonga yemuromo
Pamusoro pekurapa kweERT, kunewo mapiritsi emuromo anogona kutorwa. Aya akanyorwa zvakananga kuvanhu vakuru vane chirwere cheType 1 Gaucher. Mapiritsi aya anoshanda nekuderedza kuunganidzwa kwemafuta mumuviri atakataura nezvawo kare.
| Zita remushonga uyu | Tsananguro uye mashandisirwo |
|---|---|
| Eliglustat (Eliglustat - Cerdelga) | Uyu mushonga mutsva. Unofanira kutorwa kaviri pazuva. Zvisinei, kune vamwe vanhu vanobvisa mushonga uyu nekukurumidza mumiviri yavo (zvinogadzirisa muviri nekukurumidza), ungasashanda zvakanyanya. |
| Miglustat (Miglustat - Zavesca) | Izvi zvinopihwa kuvanhu vakuru vane chirwere cheGaucher cherudzi rwekutanga chisina kunyanya kuipa asi vasina kupindura kurapwa kweERT. Kapusule imwe chete inofanira kutorwa katatu pazuva. |
Mimwe mishonga inobatsira
Kune mamwe marapirwo akawanda aripo ekudzora matambudziko akasiyana-siyana echirwere cheGaucher.
Kurapa mapfupa asina simba
Kushaya simba kwemapfupa nekurwadziwa ndizvo zvinowanzoonekwa muchirwere cheGaucher. Rudzi rwemishonga inonzi bisphosphonates inoshandiswa kubatsira nechirwere ichi. Iyi inoshanda nekusimbisa mapfupa. Mienzaniso inosanganisira alendronate (Fosamax) uye pamidronate (Aredia). Iyi yakakodzera vana nevakuru.
Kurapa Marwadzo
Unogona kushandisa mishonga inorwadza marwadzo inowanzo shandiswa pazvinhu zvakaita sekurwadziwa kwemuviri nekurwadziwa kwemajoini.
- Kumarwadzo madiki: Mishonga yakaita seacetaminophen (Tylenol) kana ibuprofen (Advil, Motrin).
- Kana uchirwadziwa zvakanyanya: Ungangoda kutora mishonga inonyaradza marwadzo yakasimba kubva kuboka reopioid , sezvakarairwa nachiremba wako.
Uyezve, dzimwe nguva mishonga inorwisa kuora mwoyo kana kuti inozorodza tsandanyama inogona kubatsira kudzora marwadzo.
Kuvhiya
Kunyange zvazvo chirwere ichi chichigona kudzorwa nemishonga, mune dzimwe nguva kuvhiyiwa kungave kwakakosha.
- Kuvhiyiwa kwetsvina: Kana tsandanyama yakazvimba ikasaderera mushure mekurapwa kweERT uye ikaramba ichikonzera matambudziko, chiremba wako anogona kukurudzira kubviswa kwetsvina.
- Kuvhiyiwa Kwemajoini: Kana chirwere chacho chakakuvadza majoini zvakanyanya (semuenzaniso joini yehudyu) uye kurwadziwa kwakanyanya zvekuti kufamba hakugoneki, kuvhiyiwa kunogona kuitwa kuti joini iyoyo itsiviwe nejoini rekugadzira. Izvi zvinogona kuderedza marwadzo uye kuita kuti kufamba kuve nyore zvakare.
Kudyara Bone Morrow
Iyi inzira ine njodzi uye isingawanzoitiki yekurapa chirwere cheGaucher. Inosanganisira kutsiva masero eropa akakuvara neane hutano. Zvisinei, hapana humbowo hwakajeka hwekuchengetedzwa kwayo uye kushanda kwayo zvichienzaniswa nekurapa kwakajairika kwakadai seERT. Saka, inongotariswa chete muzviitiko zvakakosha apo pasina mimwe mishonga yakashanda.
Kuisirwa Ropa
Izvi zvinogona kubatsira vanhu vane chirwere chekushaya ropa chakanyanya kunokonzerwa nechirwere cheGaucher. Kupa ropa kunogona kuwedzera huwandu hwemasero matsvuku eropa, zvichidzoreredza simba remuviri.
Kukosha kwemararamiro: kudya uye kurovedza muviri
Pamusoro pekurapwa, zvakakoshawo kuchengetedza mararamiro akanaka uye ane hutano.
- Chikafu chinovaka muviri: Zvakakosha kudya chikafu chine calcium yakawanda nevhitamini D , kunyanya kuti mapfupa arambe akasimba. Isa zvinhu zvakaita semukaka, yogati, chizi, miriwo mitema, nehove diki mukudya kwako.
- Kurovedza muviri: Taura nachiremba wako uye ita maekisesaizi akakodzera iwe uye asinganyanyi kuoma pamapfundo ako. Zvinhu zvakaita sekufamba nekushambira zvakanaka.
- Mishonga yekuwedzera: Kana chiremba wako achifunga kuti hauwani vhitamini D kana calcium yakakwana kubva mukudya kwako, anogona kukukurudzira mishonga yekuwedzera.
Chekupedzisira, masayendisiti nevanachiremba vari kugara vachitsvaga nzira itsva dzekurapa chirwere cheGaucher. Idzi dzinonzi nzira dzekiriniki . Idzi nzira dzekuedza dzinokutendera kuti uedze nzira itsva dzekurapa dzisati dzawanikwa zvakanyanya. Taura nachiremba wako kana uchikwanisa kutora chikamu mune imwe yenzira idzi.
Mharidzo Yekuenda Nayo Kumba
- Chirwere cheGaucher hachisi chirwere chekutyiwa uye chekurega. Kune mishonga inoshanda iripo nhasi inogona kukubatsira kudzora zviratidzo zvako uye kurarama hupenyu hwakanaka.
- Kurapa kukuru kunoshandiswawo mukurapa kwekutsiva ma enzyme (ERT). Pamusoro pezvo, mishonga inonwiwa, mishonga inosimbisa mapfupa, uye mishonga inoderedza marwadzo.
- Mune zvimwe zviitiko, kuvhiyiwa kunongoitwa chete kana mishonga isingakwanise kudzora chirwere chacho.
- Pamusoro pekurapwa, zvakakoshawo kudya chikafu chakanaka chine calcium yakawanda nevhitamini D uye kuita maekisesaizi anokunakira.
- Chinhu chinonyanya kukosha ndechekugara uchitaura nachiremba wako uye kutevedzera mirairo yake nemazvo. Taura naye pachena nezvemibvunzo, kutya, kana kusava nechokwadi kwaunako.
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