Zvakajairika kunzwa kutya uye kushamisika kana chiremba akakuudza kuti mumwe munhu mumhuri yako, kunyanya mwana mudiki, ane chirwere chePompe. Chirwere chisingawanzoitiki, chinokonzerwa nemajini, saka zvakajairika kunetseka. Asi regai ndikuudzei, musazvidya mwoyo. Kunyange hazvo pasina mushonga wechirwere ichi parizvino, kune nzira dzakawanda dzekurapa dzinoshanda dzinogona kubatsira varwere kurarama kwenguva yakareba uye nematambudziko mashoma.
Mushonga mukuru wechirwere chePompe iERT (Enzyme Replacement Therapy).
Zvichitaurwa zviri nyore, chirwere chePompe chinokonzerwa nekushaikwa kweenzyme inokosha kumasero emhasuru mumiviri yedu. Kana enzyme iyi isipo, rudzi rweshuga runonzi glycogen runoungana mukati memasero, zvichikuvadza tsandanyama. Funga nezvazvo sekunge kune vashandi vashoma vanoshanda mumiviri yedu, uye tinovadaidza kuti maenzyme. Muchirwere chePompe, mushandi uyu haashande nemazvo.
Saka, Enzyme Replacement Therapy (ERT) ndipo patinopa enzyme isipo, kana kuti mushandi, kubva kunze kwemuviri. Pakuita izvi, mushonga une enzyme yakagadzirwa inonzi `alglucosidase alfa` inopihwa mumuviri kuburikidza netsinga (IV infusion). Kurapa uku ndiko chete kurapwa kwakatenderwa pari zvino kwechirwere chePompe.
Kurapwa uku kweERT kwakawedzera zvakanyanya hupenyu hwevarwere vePompe, kunyanya vacheche. Kare, zvaive zvisingawanzoitiki kuti mwana ane chirwere ichi ararame kupfuura gore.
Pasina kurapwa kweERT, vacheche vane chirwere chePompe vanokora tsandanyama dzemwoyo zvishoma nezvishoma, voita kuti dzimwe tsandanyama dzisashande zvakanaka mumuviri wavo, uye vofa mukati megore rekutanga rehupenyu hwavo. Vanorasikirwawo netsandanyama dzinodiwa kuti vafeme. Mushure mekunge kurapwa kweERT kwatanga, mamiriro ezvinhu aya akachinja zvachose. Vacheche vakatanga kurapwa kweERT vava mumakore avo ekuma20.
Sei zvichikosha kutanga kurapwa nekukurumidza?
Ichi ndicho chinhu chakakosha. Kurapwa neERT kunogona kumisa chirwere ichi kuti chisanyanya kuipa, asi hakugone kudzoreredza kukuvara kwatoitwa kumhasuru. Izvi zvinoreva kuti kana kurapwa kukanonoka, unogona kurasikirwa nekugona kufema kana kufamba.
Vanachiremba vanoedza kutanga kurapwa kweERT mukati memazuva mashoma ekutanga ehupenyu hwavo , kunyanya kuvana vacheche, mushure mekuongororwa kwechirwere ichi. Kunyangwe kunonoka kudiki kunogona kudzivirira mwana kufamba zvachose.
Kune vanhu vane chirwere chePompe chinotanga kunonoka, chiremba acharamba achiongorora uye achitarisa zviratidzo kuti aone nguva yakanakisa yekutanga ERT.
Rwendo urwu harusi rwemunhu oga - rutsigiro kubva kuchikwata chenyanzvi
Kunyange hazvo kurapwa kweERT kuchigona kuponesa hupenyu, hazvina kukwana. Kuti munhu ane chirwere chePompe ararame hupenyu hwakanaka, panodiwa timu yenyanzvi dzakasiyana-siyana. Nyanzvi yezvemajini inowanzova munhu mukuru anotarisira kurapwa.
Ona tafura iri pazasi yemhando dzenyanzvi dzingangodiwa pakuita izvi uye rudzi rwerutsigiro rwavachapa.
| Nyanzvi yekurapa/chiremba | Rubatsiro rwei rwavanowana? |
|---|---|
| Chiremba wemwoyo | Mhedzisiro pamwoyo inoongororwa uye kurapwa kwakakodzera kunopihwa. |
| Chiremba wemapapu | Vachaongorora matambudziko ekufema uye vachapa mafemota kana zvichidikanwa. |
| Chiremba wezvematumbu | Inorapa matambudziko ekumedza uye ekugaya chikafu. |
| Nyanzvi yezvekudya | Ipai chikafu chinodiwa kuti mukure zvakanaka, uye kana zvichidikanwa, kudya kwakakosha kana kupa chikafu kuburikidza nechubhu yekudyisa zvinokurudzirwa. |
| Chiremba weMiviri | Kurovedza muviri nekurapwa zvinoitwa kuti zvisimbise tsandanyama uye kuderedza kushaya simba. |
| Nyanzvi yekurapa pabasa | Kuvabatsira kuita mabasa ezuva nezuva (akadai sekupfeka, kudya, nezvimwewo) vari vega. Kana zvichidikanwa, vanodzidziswa kushandisa zvishandiso zvekubatsira zvakaita setsvimbo. |
| Nyanzvi yeKutaura | Inobatsira kukunda matambudziko ekutaura anokonzerwa nekushaikwa kwetsandanyama dzechiso nedzerurimi. |
Mimwe mishonga kunze kweERT
Mishonga inokanganisa masoja emuviri
Mimwe miviri yevana inoziva kuti kurapa uku kwe enzyme kunopihwa kunze (ERT) se 'kwekunze' uye inotanga kugadzira masoja ekudzivirira chirwere ichi. Izvi zvinonzi `CRIM-negative` condition. Kana izvi zvikaitika, kurapwa kweERT hakuzoshande nemazvo.
Kuti vadzivise izvi, vanachiremba vanotanga kurapwa ne immunotolerance induction (ITI) . Mukurapa uku, pamwe chete neERT, mishonga yakati wandei inopiwa inodzora masoja emuviri.
- Rituximab
- Methotrexate
- Immunoglobulin inopinzwa mutsinga (IVIG)
Zvinoitwa nemishonga iyi "kunyengedza" masoja emuviri kuti afunge kuti ERT chinhu chemuviri. Ipapo muviri hauzorwi neERT.
Kurapa muviri uye kurapwa kwebasa
Nekuti kushaya simba kwetsandanyama ndicho chinhu chikuru chinokonzerwa nechirwere chePompe, kurapwa kwemuviri kwakakosha. Kunogona kubatsira kusimbisa tsandanyama uye kuchengetedza mashandiro emuviri. Kurapa kwemabasa kunogonawo kubatsira kukudziridza hunyanzvi hunodiwa kuti uite mabasa ezuva nezuva uri wega.
Kurapa kwekutaura uye kwekudya
Nekuti tsandanyama dzinodiwa pakutsenga nekumedza chikafu dzinogona kunge dzisina simba, nyanzvi yekutaura inogona kubatsira pakurovedza muviri pakumedza. Uyewo, dzimwe nguva pombi yekudyisa inogona kudiwa kuti ipe mwana chikafu chinodiwa. Nyanzvi yezvekudya inogona kupa gwara rinodiwa pakuita izvi.
Basa rako rakakoshawo zvikuru.
Kana uchirarama nechirwere chePompe, mupiro wako semurwere kana mubereki unokosha zvikuru.
"Chinhu chakakosha chandakaita kwaive kudzidza nezvechirwere chePompe. Chakandipa chivimbo chikuru uye nyaradzo mukurarama nacho zuva nezuva," anodaro Ryan Colburn, ane chirwere ichi.
Saka, unofanira kushanda nachiremba wako kuti unzwisise zvaunoda uye kuti utungamirire mukuwana kurapwa kwakanakisisa. Usambofa wakatya kubvunza chiremba wako mibvunzo yaunayo nezvechirwere ichi kana kurapwa kwacho.
Mharidzo Yekuenda Nayo Kumba
- Kunyange hazvo chirwere chePompe chisingarapike zvachose, marapirwo anhasi anogona kubatsira varwere kurarama kwenguva yakareba uye hupenyu huri nani.
- Kurapa kukuru i enzyme replacement therapy (ERT). Uku kurapa kunoponesa hupenyu.
- Zvakakosha kutanga kurapwa nekukurumidza sezvinobvira, nekuti kukuvara kwetsandanyama hakugone kudzoserwa shure.
- Pamusoro peERT, rutsigiro kubva kunyanzvi dzakasiyana-siyana dzakadai sekurapa muviri, kudya kunovaka muviri, uye kutaura kwakakosha.
- Semurwere kana mubereki, unogona kuwana mhedzisiro yakanaka nekuva neruzivo rwakakwana nezvechirwere ichi uye kushanda pamwe chete nachiremba wako.
💬 Comments (0)
Hapana zvakataurwa parizvino. Isa mhinduro yako pano kekutanga.
Wedzera zvaunofunga