Ngaba ugqirha wakho ukuxelele okanye umntu kusapho lwakho ukuba unesifo esingaqhelekanga esibizwa ngokuba sisifo sePompe? Ukuba kunjalo, siza kuthetha ngamayeza aso namhlanje. Igama lale mayeza yiAvalglucosidase Alfa. Usenokuba uyazi ngegama elithi Nexviazyme. Eli gama liyinkimbinkimbi kancinci, ngoko usenokuba nemibuzo emininzi ngalo. Ungakhathazeki, siza kuthetha ngayo yonke le nto ngendlela elula kakhulu onokuyiqonda.
Kalula nje, loluphi uhlobo lonyango olu?
Khawuthelekelele ukuba imizimba yethu ifana nomzi-mveliso omkhulu. Ukuze yonke into isebenze kakuhle kule mzi-mveliso, kufuneka kubekho abasebenzi abenza imisebenzi eyahlukeneyo. Aba basebenzi sibabiza ngokuba zii-enzymes zomzimba wethu.
Abantu abanesifo sikaPompe bazalwa bengenalo i-enzyme ethile. Umsebenzi wale enzyme kukudiliza nokulawula iswekile ebizwa ngokuba yi-glycogen kwimisipha nakwiiseli zethu. Xa le enzyme ingekho, iswekile ebizwa ngokuba yi-glycogen iqala ukuqokelelana kwimisipha yethu. Kufana nenkunkuma efektri. Xa oku kuqokelelana, izihlunu ziba buthathaka, ziba nobunzima bokuphefumla, kwaye intliziyo iyachaphazeleka.
Ngoko ke, into eyenziwa leli yeza elibizwa ngokuba yi-Avalglucosidase Alfa kukutshintsha umsebenzi waloo enzyme ilahlekileyo nenciphileyo. Oko kukuthi, kufana nokuzisa umntu ovela ngaphandle ukuze enze umsebenzi ofanayo endaweni yomsebenzi onciphileyo. Xa eli yeza lingena emzimbeni ngomthambo, liqala ukuqhekeza loo glycogen iqokelelweyo lize liyisuse. Emva koko iimpawu ziyancipha kancinci kancinci, kwaye uqala ukuziva ukhululekile.
Izinto ekufuneka uzixelele ugqirha wakho ngaphambi kokuba usebenzise eli yeza
Ngaphambi kokuba uqalise eli yeza, kubaluleke kakhulu ukuba ugqirha wakho azi imeko yakho yonke yempilo. Oku kwenzelwa ukuba unyangwe ngokukhuselekileyo. Qiniseka ukuba ubaxelela ngezi zinto ngokukodwa.
- Ukuba unesifo sentliziyo .
- Ukuba unesifo semiphunga .
- Ukuba ukhe waba ne-allergy ngaphambili kule Avalglucosidase Alfa yeza, naluphi na olunye unyango, ukutya, idayi, okanye isithinteli.
- Ukuba ukhulelwe okanye uzama ukukhulelwa.
- Ukuba ungumama oncancisayo.
Kubaluleke kakhulu kukhuseleko lwakho ukuxelela ugqirha wakho olu lwazi ngaphandle kokulufihla.
Indlela yokusebenzisa eli yeza?
Eli asiloyeza onokulisebenzisa ekhaya. Linikwa phantsi kweliso likagqirha esibhedlele okanye eklinikhi.
- Iyeza elinikwa ngomthambo:Eli yeza lifakwa emthanjeni, njengetyuwa. Le nkqubo siyibiza ngokuba yi -infusion .
- Ilawulwa liqela lezonyango: Eli yeza linikwa ngugqirha oqeqeshiweyo okanye abasebenzi bomongikazi, ngoko ke akukho nto inokubangela ixhala.
Ngaba eli yeza linokunikwa abantwana abancinci?
Ewe, kwezinye iimeko, eli yeza linikwa abantwana abaneminyaka eli-1 ubudala. Kodwa lenziwa phantsi kweengcebiso zonyango ezikhethekileyo kunye namanyathelo okhuseleko. Ukuba unika umntwana wakho eli yeza, thetha neqela lakho lezonyango ngononophelo kwaye umchazele yonke into.
Wenza ntoni ukuba idosi iyanda?
Ekubeni eli liyeza elinikwa esibhedlele, amathuba okuba lisetyenziswe ngokugqithisileyo aphantsi kakhulu. Kodwa ukuba ucinga ukuba usenokuba ulisebenzise ngokugqithisileyo ngandlela ithile, ungoyiki. Kukho izinto ezimbini ekufuneka uzenze ngoko nangoko.
1. Yiya kwiYunithi yoNyango oluNgxamisekileyo (ETU) yesibhedlele ngoko nangoko.
2. Okanye, fowunela iZiko loLwazi ngeTyhefu leSizwe kwiSibhedlele sikaZwelonke saseColombo .
Le yimeko engxamisekileyo kakhulu, ngoko ke kubalulekile ukuba kuthathwe amanyathelo ngokukhawuleza.
Kuthekani ukuba ndiphoswa lidosi leyeza?
Ukusela eli yeza ngexesha nangomhla omiselweyo kubalulekile ukuze unyango luphumelele. Ukuba awukwazi ukuya esibhedlele ngomhla omiselweyo, ungaphoswa. Fowunela iqela lakho lezonyango ngoko nangoko uze ubazise. Baza kukuxelela into omawuyenze ngokulandelayo.
Amanqaku akhethekileyo ekufuneka uwaqaphele
- Ukuhlolwa kwezonyango: Ungaphoswa kukuhlolwa rhoqo kwiikliniki. Thetha nogqirha wakho malunga nokuba iimpawu zakho ziyaphucuka na, ziyanda, okanye ukuba uneengxaki ezintsha.
- Iimpendulo ze-Allergic: Abanye abantu banokuba ne-allergic reaction enzima kweli yeza okanye emva nje kokuba benikwe lona. Ukunciphisa lo mngcipheko, iqela lakho lezonyango linokukunika amanye amayeza (afana ne-piritan) ngaphambi kokuba uqale ukuwasebenzisa. Ngoko ke landela imiyalelo yabo ngokuchanekileyo.
Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?
Njengayo nayiphi na iyeza, oku kunokubangela iziphumo ebezingalindelekanga kwabanye abantu. Kodwa ayinguye wonke umntu ozifumanayo. Into ebalulekileyo kukwazi ukuba zeziphi ezo ziphumo ebezingalindelekanga kunye nento omawuyenze ukuba ziyavela.
| Uhlobo lwesiphumo esibi | Iimpawu |
|---|---|
| 🚨 Iziphumo ebezingalindelekanga ekufuneka zixelwe kwiqela lakho lezonyango ngoko nangoko | |
| Iimpendulo ze-allergy | Ukurhawuzelela kwesikhumba, ukurhawuzelela, ukuba bomvu, ukudumba kobuso, imilebe, ulwimi okanye umqala. |
| Iimpendulo zokufakwa | Intlungu esifubeni, ubunzima bokuphefumla, ukuquleka, ukuziva unesizungu. |
| 🙂 Iziphumo ebezingalindelekanga ekufuneka zixelwe kugqirha ukuba ziyaqhubeka okanye ziyakhathaza | |
| Iziphumo ebezingalindelekanga eziqhelekileyo |
|
Eyona nto ibalulekileyo kukuba, ukuba uziva into engaqhelekanga, nokuba yinto encinci, ungoyiki ukuxelela iqela lezonyango.
Ngaba kukho iingxaki ngamanye amayeza?
Ngokweenkcukacha zangoku, eli yeza alikabikwa ukuba libangela iingxaki ezinkulu kwamanye amayeza. Nangona kunjalo, nika ugqirha wakho uluhlu olupheleleyo lwamayeza owasebenzisayo (kuquka iivithamini, amayeza ezityalo, kunye nezongezo). Kwakhona, xelela ugqirha wakho ukuba utshaya okanye usela utywala. Oku kunokunceda ukuthintela iingxaki ezingeyomfuneko.
Umyalezo Wokuya Ekhaya
- I-Avalglucosidase Alfa lunyango oluthatha indawo ye-enzyme engonelanga emzimbeni wabantu abanesifo sePompe.
- Eli yeza linikwa esibhedlele, ngomthambo (i-infusion). Ngoko ke awuyi kunikwa amayeza oza kuwathatha uye nawo ekhaya.
- Ngaphambi kokuba uqale unyango, xelela ugqirha wakho ngokupheleleyo ngazo zonke iimeko zakho zempilo kunye nee-aleji.
- Ukuba ufumana naziphi na iimpawu ezinzima ezifana nobunzima bokuphefumla, iintlungu zesifuba, okanye ukurhawuzelela kolusu ngexesha okanye emva konyango, yazisa iqela lezonyango ngoko nangoko.
- Qiniseka ukuba uthatha amayeza akho kwaye uye kwiikliniki ngemihla emiselweyo. Ungaphoswa ziintlanganiso.
- Ukuba uneminye imibuzo malunga nale mithi, zive ukhululekile ukuyixoxa nogqirha wakho.
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