Nangmah emaw i hriat emaw Fabry natna vei i nih chuan Galafold tih damdawi i hre tawh ngei ang. Fabry natna damdawi ei hmasa ber leh awmchhun a ni. Fabry natna enkawlna dang, enzyme replacement therapy te hi injection hmanga pek a ni. Galafold hi "chaperone" therapy chi khat a ni a, a hnathawh dan chu a danglam vek a ni. Fabry natna chi hrang hrang tan a thawk lo. Mahse, natna thlentu gene-a mutations engemaw zat nei tan chuan a \angkai hle thei a ni.
Fabry natna chu eng nge ni? Galafold hian engtin nge hna a thawh?
A awlsam zawngin sawi ila, Fabry natna hi genetic condition hmuh tur awm lo tak a ni a, nu leh pa atanga fate hnena kai theih a ni. Fabry natna vei te hian an taksaah enzyme pakhat alpha-galactosidase A (alpha-gal) an nei a. Mahse he enzyme hian hna a thawk tha lo. Hei hi a chhan chu he enzyme siam tura hrilhtu gene-ah hian mutation a awm vang a ni.
Alpha-gal hi enzyme pawimawh tak a ni a, kan taksa cell tam takah a awm a. A hnathawh ber chu GL-3 tih hming pu thil thau tak tihchhiat hi a ni. Chuvangin, he enzyme hian hna a thawh that loh chuan GL-3 an tih chu taksa cell chhungah a awm khawm tan ta a ni. Hun a kal zel a, he thil khawlkhawm hian taksa peng pawimawh tak tak, kal , thinlung , vun, thluak, leh nervous system -te chu nasa takin a tichhe thei a ni.
Fabry natna hi chi hnih a awm a.
| Natna chi hrang hrang (Type) 1.1. | Thuziak & Symptoms te |
|---|---|
| Classic type a ni | Hei hi natna chi khat na ber a ni. Naupan laia symptoms hi a lang tlangpui. Natna i nei thei a, chu chu kut leh kea kang leh hliam te, pum leh chaw ei lama harsatna te, leh navel leh khup inkar vun sen hring (red-purple) te a ni thei. Mi thenkhat chuan thawmhnaw a tlahniam a, vun a vawt a, lumna laka an hriatna nasa lutuk pawh an nei thei bawk. |
| Late-onset type a ni | Hei hi Fabry natna chi khat a ni ber. Classic form aiin a milder zawk. Natna hi pian tirh atangin awm mah se, naupan lai hian a lan chhuah dan a lang lo. Chu ai chuan kum 30 emaw a hnu lamah emaw symptoms a lang tan thin. |
Fabry natna vei te hi an upat deuh deuh chuan GL-3 hi an cell-ah a pung zel a. Hei hian hun kal zelah hriselna lama harsatna lian tak a thlen thei a ni. Mi tam tak chuan kal lama harsatna an nei a, chu chuan a tâwpah chuan kal a tichhe thei a ni . He natna hian thinlung leh thisen kalna kawng a tichhe thei bawk , heart attack emaw stroke emaw a thlen theihna a tipung bawk.
Fabry natna enkawlna tum ber chu taksain `alpha-gal` enzyme hnathawk thei pek hi a ni. Hei hian chhiatna lo thleng tawh chu a tidanglam lo nain, nakin lawka chhiatna a thlen loh nan a pui a ni.
Hemi atan hian mi tam tak chu Enzyme Replacement Therapy (ERT) pek an ni. Hei hian pawn lam atanga siam enzyme hnathawk tha tak taksaah inject a huam a ni. Chumi hnuah chuan taksa chuan chu enzyme chu hmangin GL-3 chu a ti chhe thei ta a ni.
Mahse, Galafold hian hna a thawk dan chu a danglam vek a ni. Galafold-a active ingredient hi damdawi `migalastat` an tih a ni. A tih chu, damlo taksa ngeia awm `alpha-gal` enzyme-ah a inzawm a, mahse a nghet lo. I taksaa `alpha-gal` enzyme chu tlem a ‘swollen’, ‘unstable’ deuhin han ngaihtuah teh. Chuvangin a thawk tha thei lo. Galafold an tih damdawi hian a tih chu a thian ang bawkin he enzyme ‘swollen’ hi a inzawm a, a ‘stabilize’ a ni. Tichuan chu enzyme chuan a hna chu a thawk tha thei tawh ang. Hei hi "Chaperone Therapy" kan tih chu a ni.
Mi zawng zawngin Galafold hi an hmang thei ang em?
No. Galafold hi `alpha-gal` enzyme siamtu gene-a amenable mutation chi khat bik nei tan chauh hman theih a ni. He mutation atanga siam `alpha-gal` enzyme hian hna a la thawk thei tho a, mahse a nghet lo lutuk a, a thawk tha thei lo. Galafold hian chu enzyme chu a inzawm tlat a, a stabilize a, a thawk thei ta a ni.
Fabry damlo thenkhat chuan `alpha-gal` enzyme hi an siam chhuak lo tak zet a, a nih loh leh an siam chhuah enzyme hian hna a thawk lo tak zet a ni. Galafold hian chutiang mite tan chuan engmah a tangkai dawn lo. A chhan chu taksaah hian a ti nghet thei tur enzyme a awm lo. Enzyme replacement therapy (ERT) hi an tan a tha zawk.
Chuvangin, i doctor-in Galafold a pek hmain, eng chi genetic mutation nge i neih tih chiang taka hriat theih nan special genetic test a ti ang. Hei hian he damdawi hi i tan a hlawkpui dawn nge dawn lo tih a hril ang.
He damdawi hi engtin nge i hman?
He part hi a pawimawh hle a, a chhan chu damdawi hlawkna kimchang hmu tur chuan dik taka hman a ngai a ni.
- Ei dan: Galafold hi capsule a ni. Ni hnih danah lak a ni , chu chu ni hnih danah a ni.
- Nitin hun: Nitin hun khata ei a pawimawh hle. Entirnan, ni khatnaah zing dar 8-ah i ei a nih chuan a tuk zingah pawh zing dar 8-ah i ei tur a ni.
- A pawimawh ber chu: He damdawi hi chaw ei loh laiin ei tur a ni . Chumi awmzia chu damdawi ei hma darkar 2 emaw, ei hnu darkar 2 emaw chhung chu engmah ei loh tur a ni.
- Thil pawimawh dang: Tin, caffeine awmna engmah (tea, coffee, soft drink thenkhat ang chi) pill ei hma darkar 2 leh ei hnu darkar 2 chhung chu in loh tur a ni . Hemi chhung hian tui, non-fiber fruit drinks, non-caffeinated carbonated drinks te i in thei bawk.
I ni a inhmeh loh nan he damdawi hi special package-ah a awm a. `Blister card' an ti a ni. Ni chhinchhiah dan a nei a. Damdawi i ei ni khan blister card i phelh a, damdawi i ei loh ni chuan a hmuna empty circle chu i phelh bawk. Chutiang chuan nitin card chu i mark thei a, damdawi ei tur ni chu i tlai lo thei bawk.
Damdawi dang hian side effect leh harsatna a nei em?
Galafold ei thin thenkhat chuan thawkna lama natna (khawsik, rilru na) leh zun kawng natna te an nei tlem thei hle. Infection laka invenna tha ber chu kut sil fo te, damlo laka inthiarfihlim te, tui in tam te hi a ni.
A pawimawh ber chu, he damdawi avanga lo awm thei nia i ngaih symptoms buaithlak, danglam tak i neih chuan nangmah chauhin ei kha bansan ngai suh I doctor nen inbia ngei ang che.
Tin, a hmaa kan sawi tawh ang khan caffeineChuvangin Galafold hi a hlawk lo zawk mai thei. Chuvangin caffeine awmna thilah fimkhur rawh. Tin, damdawi dang, vitamin emaw, traditional medicine emaw i ei lai doctor hnenah hriattir a pawimawh hle bawk.
Take-Home Thuchah a ni
- Galafold hi Fabry Disease chi hrang hrang tan chuan ei tur bik a ni.
- Hei hian i taksaa enzyme awm tawh, mahse a nghet lo chu a stabilize a, a thawk thei a ni. Hei hi i tan a tha em tih hriat nan genetic test i tih a ngai a ni.
- Damdawi hi ni hnih danah ei tur a ni a, pum ruak a ei tur a ni. Damdawi ei hma darkar 2 leh ei hnu darkar 2-ah caffeinated in tur ei leh in loh tur.
- He damdawi hian tuna i natna lan chhuah dan hi a ti tha lo mai thei a, mahse nakin lawka natna avanga chhiatna thleng tur venna atan a pui dawn a ni.
- Side effect emaw harsatna i neih chuan doctor nen inbia nghal ang che.
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