Ungave wakaona kuti mwana wako mudiki anoita seaneta zvishoma kupfuura vamwe vana, ane dambudziko rekubata mutsipa wake wakatwasuka, kana kuti ari kuwedzera uremu zvishoma nezvishoma. Kana kuti, semunhu mukuru, unonzwa kuneta zvisina kujairika uye dzungu here paunokwira masitepisi kana kufamba daro diki? Chikonzero cheizvi chingave chirwere chisingawanzoitiki chausati wambonzwa nezvacho. Ndizvo chaizvo zvatiri kutaura nezvazvo nhasi.
Zvichitaurwa zviri nyore, chii chinonzi Pompe Disease?
Funga nezvemuviri wedu sefekitori hombe. Fekitori iyi inoda vashandi vakasiyana (mapuroteni/ ma enzyme ) kuti ishande. Chirwere chePompe chinoitika kana chimwe chevashandi (puroteni chaiyo) inopwanya glycogen , rudzi rweshuga mumuviri wedu, uye ichiburitsa simba chikashaikwa kana kuti chisiri kushanda nemazvo.
Zvino sezvo mushandi uyu aenda, pachinzvimbo chekushandurwa kuita simba, rudzi irworwo rweshuga inonzi glycogen inotanga kuungana mukati memasero emuviri wedu, kunyanya munzvimbo dzakadai semhasuru, moyo , nechiropa . Zvakafanana nezvinhu zvakaunganidzwa mufekitori. Kuunganidzwa uku kweglycogen kunokuvadza nhengo idzodzo uye kunoshayisa simba mashandiro adzo.
Chirwere ichi chinozivikanwawo se `GAA deficiency` kana `type II glycogen storage disease ( GSD )`.
Chii chinokonzera chirwere ichi?
Chirwere chePompe chirwere chinokonzerwa nemajini . Izvi zvinoreva kuti tinochigara nhaka kubva kuvabereki vedu. Zvisinei, kuti uve nechirwere ichi, unofanira kugamuchira majini asina kunaka echirwere ichi kubva kuna amai vako nababa vako .
Kana munhu akagara nhaka jini rimwe chete risina kunaka, haazoratidzi zviratidzo zvechirwere. Asi ndiye anotakura chirwere ichi. Izvi zvinoreva kuti anogona kupa jini risina kunaka kuvana vake.
Ndezvipi zviratidzo zvechirwere ichi?
Zviratidzo zvechirwere ichi, zera rekutanga kwacho, uye kuoma kwechirwere zvinogona kusiyana zvikuru kubva pamunhu kusvika pamunhu. Chinogona kukamurwa muzvikamu zviviri zvikuru.
| Nguva yekutanga kwechirwere | Zviratidzo zvinowanzoonekwa |
|---|---|
| Rudzi rwekutanga kwevacheche (Kubva pamwedzi mishoma kusvika pagore) |
|
| Rudzi rwekutanga kunonoka (Pazera ripi zvaro, kubva paudiki kusvika pakukwegura) |
|
Nzira yekuziva chirwere nemazvo sei?
Nekuti zviratidzo izvi zvinowanzofanana nezvezvimwe zvirwere, kuongororwa kwechirwere kunogona kuoma zvishoma. Chiremba wako anogona kubvunza mibvunzo yakaita seiyi kuti akubatsire kunzwisisa mamiriro acho ezvinhu:
- Unonzwa usina simba, unowa kakawanda, kana kuti unonetseka nekufamba, kumhanya, kana kukwira masitepisi here?
- Une dambudziko rekufema here, kunyanya husiku kana kuti paunenge wakarara pasi?
- Une musoro here paunomuka mangwanani?
- Unonzwa kuneta zvakanyanya here mukati mezuva rese?
- Pane mumwe munhu mumhuri yako ane zviratidzo izvi here?
Pamusoro pemibvunzo iyi, mimwe miedzo inogona kuitwawo kuti pasave nedzimwe zvirwere. Kana paine kufungidzirwa kuti chirwere chePompe chine chirwere, miedzo iyi inoitirwa kusimbisa chirwere ichi:
- Kuongororwa kweropa: Izvi zvinotarisa kuti puroteni isina kukwana (enzyme) iri kushanda zvakanaka sei.
- Kuongororwa kwetsandanyama: Kachikamu kadiki ketsandanyama kanotorwa koongororwa nemaikorosikopu kuti vaone kuti glycogen yakawanda sei irimo.
- Kuedzwa kwemajini:Vari kutsvaga zvakananga chikanganiso chemajini chinokonzera chirwere ichi.
Chinhu chinonyanya kukosha ndechekuti zvinotora nguva kuti chirwere ichi chionekwe. Zvinogona kutora mwedzi mitatu chete kumwana, uye makore manomwe kusvika mapfumbamwe kumunhu mukuru. Saka zvakakosha kuva nemwoyo murefu.
Ndezvipi zviri kurapwa?
Kunyangwe pari zvino pasina mushonga wechirwere ichi, kune mishonga inoshanda zvikuru inogona kudzora zviratidzo uye kuwedzera hupenyu hwemurwere. Zvakakosha zvikuru kutanga kurapwa nekukurumidza sezvinobvira , kunyanya kuvacheche.
Kurapa kukuru iEnzyme Replacement Therapy (ERT) . Zvichitaurwa zviri nyore, izvi zvinosanganisira kupa muviri enzyme (protein) inoshaikwa kana kuti isina kukwana mumuviri kuburikidza nejekiseni. Kana jekiseni iri ragamuchirwa, muviri unokwanisa kupwanya shuga yeglycogen. Mimwe yemishonga inoshandiswa pakuita izvi ndeiyi:
- `Myozyme` (kazhinji kacho yevana vacheche)
- `Lumizyme`
- `Nexviazyme` (yerudzi rwekutanga kunonoka)
Pamusoro pezvo, kune mushonga mutsva unotenderwa kuvanhu vakuru vasingapindure zvakanaka pakurapwa kweERT. Unoshandisa musanganiswa wemishonga miviri inotorwa sejekiseni (`Pombiliti`) uye makapisiri (`Opfolda`).
Zvinhu zvekufunga nezvazvo kana uchirarama nechirwere ichi
Kurarama nechirwere chePompe kunogona kuoma, saka zvakakosha kuti uwane rutsigiro kwauri nemhuri yako. Unogonawo kubatana nemapoka ekutsigirana uko vamwe vanhu vane chirwere ichi vanogona kugovana ruzivo rwavo uye kuwana mazano anoshanda.
Semuenzaniso, kana chikafu chakaoma kumedza, zvinogocha zvinogona kuwedzerwa muchikafu. Vamwe vanhu vangangoda kupihwa chikafu kuburikidza nechubhu yekudyisa kuti vawane chikafu chavanoda.
Nekuti chirwere ichi chinokanganisa nhengo dzakawanda dzemuviri, unoda rutsigiro rwechikwata chevanachiremba vane hunyanzvi.
- Chiremba wemwoyo
- Chiremba wetsinga
- Chiremba weKufema
- Nyanzvi yezvekudya
Nerutsigiro rwemunhu wese tinogona kudzora zviratidzo uye kuramba takagwinya.
Mharidzo Yekuenda Nayo Kumba
- Chirwere chePompe chirwere chinoberekwa kubva kuvabereki.
- Zviratidzo zvikuru kushaya simba kwetsandanyama uye kutadza kufema zvakanaka.
- Chirwere ichi chinogona kuzviratidza mumhando mbiri: muhucheche uye mukukura kwevana.
- Nekuongorora chirwere ichi nekukurumidza uye kutanga enzyme replacement therapy (ERT), kukuvara kunokonzerwa nechirwere ichi kunogona kuderedzwa.
- Kana iwe kana mwana wako musina chokwadi nezvezviratidzo izvi, kurumidzai kuonana nachiremba uye tsvagai mazano.
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