Umzimba wethu, uma sikhuluma iqiniso, ungumshini oyinkimbinkimbi kakhulu futhi omangalisayo. Ukuze konke okukulo mshini kusebenze kahle, uhlobo olukhethekile lweprotheyini olubizwa ngokuthi 'ama-enzyme' lubalulekile. Cabanga, ngezinye izikhathi ngenxa yezizathu zofuzo, i-enzyme ethile ingase ingakhiqizwa emzimbeni womuntu noma ingakhiqizwa ngenani elincane. Isifo i-Pompe yisifo esingavamile kodwa esibi esenzeka ngale ndlela. Namuhla sizokhuluma ngokwelashwa okukhethekile okunikezwa ukulawula lesi sifo, okungukuthi, umuthi wokugomela obizwa ngokuthi i-Alglucosidase alfa.
Kuyini i-Alglucosidase alfa?
Kalula nje, i-Alglucosidase alfa iwumjovo wangaphandle we-enzyme enganele emzimbeni womuntu onesifo se-Pompe. Lokhu kufana nokuletha umuntu wangaphandle ukuze enze umsebenzi othile lapho isisebenzi singekho. Le ndlela yokwelapha ibizwa ngokuthi i-Enzyme Replacement Therapy (ERT) kwisayensi yezokwelapha.
Into ebaluleke kakhulu okufanele uyikhumbule lapha ukuthi lokhu akulona ikhambi lalesi sifo. Kodwa-ke, lokhu kwelashwa kungalawula izimpawu, kunciphise ubuthakathaka bemisipha, futhi kuthuthukise kakhulu ikhwalithi yokuphila kwesiguli. Lo muthi ungatholakala emakethe ngaphansi kwamagama emikhiqizo efana ne-`Lumizyme` noma i-`Myozyme`.
Izinto okudingeka uzitshele udokotela wakho ngaphambi kokuthatha lo muthi
Ngaphambi kokuqala noma yikuphi ukwelashwa okuthile, kubaluleke kakhulu ukwazisa udokotela wakho ngomlando wakho wezempilo ophelele. Uma udokotela wakho enempilo ephelele, ukwelashwa kuzoba okuphephile futhi okuphumelelayo. Ngakho-ke, uma kukhona okulandelayo okusebenzayo kuwe, qiniseka ukuthi ukusho.
- Isifo senhliziyo: Uma unesifo senhliziyo noma izinkinga ezihlobene nenhliziyo.
- Ukucindezeleka kokuphefumula: Uma unesifo samaphaphu noma ubunzima bokuphefumula.
- Ukungezwani komzimba: Uma une-allergies kunoma yini, kufanele usitshele nakanjani. Ikakhulukazi:
- Uma uke waba nokusabela kokungezwani komzimba kulo muthi noma kunoma yiziphi izithako zawo, njenge-mannitol.
- Uma une-allergy kuma-hamster noma amaprotheni egundane (lokhu kubalulekile ngoba asetshenziswa ekukhiqizweni kwemithi ethile).
- Uma une-allergy kunoma yimuphi omunye umuthi, ukudla, izithasiselo noma udayi.
- Ukukhulelwa: Uma ukhulelwe njengamanje, noma uzama ukukhulelwa maduze.
- Uma ungumama oncelisayo: Uma ungumama oncelisayo ingane.
Lokhu kwelashwa kunikezwa kanjani?
Lo muthi awuyona iphilisi lokugwinya, futhi awuyona umjovo ongawunikezwa ekhaya.
I-Alglucosidase alfa iyi-intravenous infusion (IVI). Kalula nje, inikezwa ngendlela efanayo nesisombululo sikasawoti.
Lokhu kwelashwa kunikezwa kuphela esibhedlela noma emtholampilo okhethekile, ngaphansi kokuqashwa okuseduze kodokotela nabasebenzi abahlengikazi . Bazohlala beqapha izinga lokushaya kwenhliziyo yakho, umfutho wegazi, kanye nokuphefumula ngesikhathi sokwelashwa.
Ingabe lokhu nikunikeza izingane ezincane?
Yebo, lokhu kwelashwa kunganqunywa ngisho nasezinsaneni ezincane ezinenyanga eyodwa ubudala. Kodwa-ke, udokotela wezingane kuphela ongenza isinqumo. Kunezinyathelo zokuphepha ezikhethekile okumele zilandelwe lapho kunikezwa lokhu kwelashwa enganeni.
Yini okufanele uyenze uma uphuza ngokweqile?
Lokhu kunikezwa esibhedlela, ngakho-ke amathuba okuba lokhu kwenzeke mancane kakhulu. Kodwa-ke, uma ucabanga ukuthi usuthathe lo muthi omningi kakhulu, yazisa abasebenzi bezokwelapha ngokushesha. Esimweni esiphuthumayo, ungashayela iNational Poison Information Centre eColombo National Hospital . Noma kufanele uye e -Emergency Treatment Unit (ETU) eseduze.
Kuthiwani uma ngiphuthelwa ukwelashwa?
Kubaluleke kakhulu ukuya esimisweni sakho esihleliwe. Uma ngesizathu esithile ungakwazi ukuya, ungasidluli, kodwa shayela udokotela wakho noma isibhedlela ngokushesha ukuze uhlele olunye usuku nesikhathi.
Yimiphi imiphumela emibi elindelekile?
Njenganoma yimuphi umuthi, lokhu kwelashwa kungaba nemiphumela emibi. Nakuba iningi lalokhu lincane, kunezinto ezithile okudingeka siziqaphele kakhulu. Ake sibheke lokhu etafuleni ukuze kube lula ukukuqonda.
| Uhlobo lomphumela ongemuhle | Izici |
|---|---|
| Imiphumela emibi kakhulu okufanele ibikwe kudokotela ngokushesha |
|
| Imiphumela emibi evame ukungabi mibi kakhulu, kodwa okufanele ibikwe kudokotela uma iqhubeka |
Ingabe zikhona izinkinga ngeminye imithi?
Lo muthi ngokuvamile awunazo izindlela eziningi zokusebenzisana neminye imithi. Kodwa-ke, kubalulekile ukutshela udokotela wakho ngayo yonke imithi oyithathayo (kufaka phakathi amavithamini nemikhiqizo yamakhambi), nokuthi uyabhema noma uyaphuza utshwala.
Umlayezo Wokuya Nawe Ekhaya
- I-Alglucosidase alfa iyindlela yokwelapha isifo sikaPompe esithatha indawo ye-enzyme engekho emzimbeni, kodwa akuyona ikhambi lalesi sifo.
- Lokhu kwelashwa kunikezwa esibhedlela kuphela, ngomthambo (ukufakwa) ngaphansi kokuqashwa ngudokotela.
- Ngaphambi kokuqala ukwelashwa, tshela udokotela wakho umlando wakho wezempilo ophelele, ikakhulukazi noma yikuphi ukungezwani komzimba.
- Funa usizo lwezokwelapha ngokushesha uma uhlangabezana nezimpawu ezinjengobunzima bokuphefumula, ubuhlungu besifuba, noma ukusabela okukhulu kokungezwani komzimba.
- Ungalokothi uphuthelwe usuku lwakho lokwelashwa oluhleliwe. Uma unenkinga, yazisa udokotela kusenesikhathi.
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