Umzimba wethu, ukuba sithetha ngokuchanekileyo, ngumatshini ontsonkothileyo nomangalisayo. Ukuze yonke into ekulo matshini isebenze ngokufanelekileyo, uhlobo oluthile lweprotheyini olubizwa ngokuba yi-'enzymes' lubalulekile. Khawuthelekelele, ngamanye amaxesha ngenxa yezizathu zemfuza, i-enzyme ethile isenokungaveliswa emzimbeni womntu okanye ingaveliswa ngomlinganiselo omncinci. Isifo sikaPompe sisifo esingaqhelekanga kodwa esinzima esenzeka ngale ndlela. Namhlanje siza kuthetha ngonyango olukhethekileyo olunikwayo ukulawula esi sifo, oko kukuthi, isitofu esibizwa ngokuba yi-Alglucosidase alfa.
Yintoni i-Alglucosidase alfa?
Ngamafutshane, i-Alglucosidase alfa yinaliti yangaphandle ye-enzyme engasebenzi kakuhle emzimbeni womntu onesifo sePompe. Oku kufana nokuzisa umntu wangaphandle ukuba enze umsebenzi othile xa umsebenzi engekho. Le ndlela yonyango ibizwa ngokuba yi -Enzyme Replacement Therapy (ERT) kwisayensi yezonyango.
Eyona nto ibalulekileyo ekufuneka uyikhumbule apha kukuba olu asilonyango lwesi sifo. Nangona kunjalo, olu nyango lunokulawula iimpawu, lunciphise ubuthathaka bemisipha, kwaye luphucule kakhulu umgangatho wobomi bomguli. Eli yeza linokufumaneka kwimarike phantsi kwamagama eempawu ezifana ne-`Lumizyme` okanye i-`Myozyme`.
Izinto ekufuneka uzixelele ugqirha wakho ngaphambi kokuba usebenzise eli yeza
Ngaphambi kokuba uqalise naluphi na unyango oluthile, kubaluleke kakhulu ukwazisa ugqirha wakho ngembali yakho epheleleyo yempilo. Okukhona ugqirha wakho egqibelele ngempilo yakho, kokukhona unyango luya kukhuseleka kwaye luphumelele ngakumbi. Ngoko ke, ukuba kukho naziphi na kwezi zilandelayo ezisebenza kuwe, qiniseka ukuba uyazikhankanya.
- Isifo sentliziyo: Ukuba unesifo sentliziyo okanye iingxaki ezinxulumene nentliziyo.
- Uxinzelelo lokuphefumla: Ukuba unengxaki yemiphunga okanye nakuphi na ukuphefumla okunzima.
- Ii-Allergy: Ukuba kukho nantoni na onayo malunga ne-allergy, ngokuqinisekileyo kufuneka usixelele. Ingakumbi:
- Ukuba ukhe waba ne-allergy kule mithi okanye nakweyiphi na izithako zayo, njenge-mannitol.
- Ukuba une-allergy kwi-hamster okanye iiproteni zeempuku (oku kubalulekile kuba ezi zisetyenziswa ekwenziweni kwamanye amayeza).
- Ukuba une-allergy kuyo nayiphi na enye imithi, ukutya, izithinteli okanye iidayi.
- Ukukhulelwa: Ukuba ukhulelwe ngoku, okanye uzama ukukhulelwa kungekudala.
- Ukuba ungumama oncancisayo: Ukuba ungumama oncancisayo umntwana.
Lunikwa njani olu nyango?
Eli yeza alilophilisi lokuliginya, kwaye alilo nenaliti enokujovwa ekhaya.
I-Alglucosidase alfa yi-intravenous infusion (IVI). Ngamafutshane, inikwa ngendlela efanayo nesisombululo se-saline.
Olu nyango lunikwa kuphela esibhedlele okanye kwikliniki ekhethekileyo, phantsi kweliso elibukhali loogqirha kunye nabasebenzi bonyango . Baza kuhlala bejonga izinga lentliziyo yakho, uxinzelelo lwegazi, kunye nokuphefumla kwakho ngexesha lonyango.
Ingaba ninika abantwana abancinci oku?
Ewe, olu nyango lunokunikwa nokuba lunikwe iintsana ezineminyaka eyi-1 ubudala. Nangona kunjalo, ngugqirha wabantwana kuphela onokwenza isigqibo. Kukho amanyathelo akhethekileyo okhuseleko ekufuneka alandelwe xa kunikwa umntwana olu nyango.
Yintoni omawuyenze ukuba ugqithisile?
Oku kunikwa esibhedlele, ngoko ke amathuba okuba oku kwenzeke mancinci kakhulu. Nangona kunjalo, ukuba ucinga ukuba usele amayeza amaninzi kakhulu, yazisa abasebenzi bezonyango ngoko nangoko. Kwimeko engxamisekileyo, ungatsalela umnxeba iZiko loLwazi lweeTyhefu leSizwe kwiSibhedlele sikaZwelonke saseColombo . Okanye kufuneka uye kwiYunithi yoNyango oluNgxamisekileyo (ETU) ekufutshane.
Kuthekani ukuba ndiphoswa lunyango?
Kubaluleke kakhulu ukuya kwintlanganiso yakho ecwangcisiweyo. Ukuba ngesizathu esithile awukwazi ukuya, musa ukuyiphosa, kodwa fowunela ugqirha wakho okanye isibhedlele ngoko nangoko ukuze ulungiselele omnye umhla nexesha.
Zithini iziphumo ebezingalindelekanga?
Njengakweminye imithi, olu nyango lunokuba nemiphumo emibi. Nangona uninzi lwale miphumo luncinci, kukho izinto ekufuneka sizilumkele ngakumbi. Masijonge oku kwitheyibhile ukuze kube lula ukukuqonda.
| Uhlobo lwesiphumo esibi | Iimpawu |
|---|---|
| Iziphumo ebezingalindelekanga ezinzulu ekufuneka zixelwe kugqirha ngoko nangoko |
|
| Iziphumo ebezingalindelekanga ezingaqhelekanga, kodwa kufuneka zixelwe kugqirha ukuba ziyaqhubeka |
Ngaba kukho naziphi na iingxaki ngamanye amayeza?
Eli yeza ngokubanzi alinazo iindlela ezininzi zokunxibelelana namanye amayeza. Nangona kunjalo, kubalulekile ukuxelela ugqirha wakho malunga nayo yonke imithi oyithathayo (kuquka iivithamini kunye neemveliso zezityalo), kunye nokuba uyatshaya okanye uyasela utywala.
Umyalezo Wokuya Ekhaya
- I-Alglucosidase alfa lunyango lwesifo sikaPompe oluthatha indawo ye-enzyme engekhoyo emzimbeni, kodwa ayilonyango lwesifo.
- Olu nyango lunikwa esibhedlele kuphela, ngomthambo (ukufakwa) phantsi kweliso likagqirha.
- Ngaphambi kokuba uqale unyango, xelela ugqirha wakho imbali yakho epheleleyo yempilo, ingakumbi nayiphi na i-allergies.
- Funa uncedo lwezonyango ngokukhawuleza ukuba ufumana iimpawu ezinjengobunzima bokuphefumla, iintlungu zesifuba, okanye iimpembelelo eziqatha ze-allergy.
- Ungaze uphoswe ngumhla ocwangcisiweyo wonyango. Ukuba kukho naluphi na uvakalelo olungathandekiyo, yazisa ugqirha kwangaphambili.
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