If yu ɔ pɔsin we yu sabi gɛt Fabry sik , yu go dɔn yɛri bɔt wan mɛrɛsin we dɛn kɔl Galafold. Na di fɔs ɛn di wan ol mɛrɛsin we dɛn kin tek fɔ it fɔ Fabry sik. Ɔda tritmɛnt fɔ Fabry sik, lɛk ɛnzaym riplesmɛnt tɛrapi, na injɛkshɔn. Galafold na wan kayn "chaperone" tεrapi wae de woke na komplit difrεnt we. I nɔ de wok fɔ ɔl kayn Fabry sik. Bɔt i kin rili ɛp pipul dɛn we gɛt sɔm chenj dɛn na di jin we de mek pɔsin gɛt di sik.
Wetin na Fabry sik? Aw Galafold de wok?
Fɔ tɔk am simpul wan, Fabry sik na wan sik we nɔ kin apin so ɔltɛm we pɔsin kin gɛt frɔm mama ɔ papa to pikin. Pipul wae gɛt Fabry sik gɛt wan ɛnzaym wae dɛn kɔl alpha-galactosidase A (alpha-gal) na dɛn bɔdi. Bɔt dis ɛnzaym nɔ de wok fayn. dis na biכs wan mכtεshכn de na di jin we de tεl am fכ mek dis εnzym.
Alfa-gal na wan rili imכtant εnzym we de insay bכku sεl dεm na wi bכdi. di men wok we i de du na fכ brok wan fεt tin we dεn kכl GL-3 . so, we dis εnzym nכ de wok fayn, di tin we dεn kכl GL-3 de bigin fכ kכlכm insay di bכdi in sεl dεm. As tɛm de go, dis tin we kin gɛda kin mek impɔtant pat dɛn na di bɔdi pwɛl bad bad wan, lɛk di kidni dɛn , di at , di skin, di bren, ɛn di nervɔs sistɛm .
Tu men kayn Fabry sik de.
| Di kayn sik (Type) . | Diskripshɔn & Simptom dɛn |
|---|---|
| Klasik tayp | Dis na de kayn sik wae de pasmak. Bɔrku tɛm, di sayn dɛm kin sho wae dɛn smɔl. Yu kin gɛt pen, lɛk we yu an ɛn fut kin bɔn ɛn nɔ gɛt bɛtɛ trɛnk, yu bɛlɛ ɛn yu kin gɛt prɔblɛm wit yu bɔdi, ɛn yu kin gɛt rɛd-pɔpul pat pan di skin bitwin yu nɛv ɛn yu ni. Sɔm pipul dɛn kin gɛt bak se dɛn nɔ kin swet, dɛn skin kin dray, ɛn dɛn kin fil bad bad wan. |
| Tayp we kin bigin let | Dis na di kayn Fabry sik we kin bɔku pas ɔl. I milda pas di klas we dɛn kin yuz fɔ mek am. Pan ɔl we di sik kin de frɔm we dɛn bɔn am, di sayn dɛn nɔ kin apin we i smɔl. Bifo dat, di sayn dɛm kin bigin fɔ sho na di 30 ia ɔr leta. |
as pipul dεm we gεt Fabry sik de ol, GL-3 de kכntinyu fכ kכmכt na dεn sεl dεm. Dis kin mek yu gɛt siriɔs wɛlbɔdi prɔblɛm as tɛm de go. Bɔku pipul dɛn kin gɛt prɔblɛm wit dɛn kidni, ɛn dis kin mek dɛn kidni nɔ wok fayn . Dis sik kin pwɛl di at ɛn di blɔd vesel dɛm bak , ɛn dis kin mek yu gɛt at atak ɔr strok.
di men gol fכ trit Fabry sik na fכ gi di bכdi wan `alpha-gal` εnzym we de wok. Pan ɔl we dis nɔ de rivɛns di damej we dɔn ɔlrɛdi apin, i de ɛp fɔ mek dɛn nɔ gɛt damej tumara bambay.
Dɛn kin gi bɔku pipul dɛn Enzyme Replacement Therapy (ERT) fɔ dis. dis involv fכ injεkt wan εnzym we de wok fayn fayn wan we dεn prodyuz na do insay di bכdi. afta dat di bכdi kin ebul fכ yuz da εnzym de fכ brok dכn GL-3.
Bɔt Galafold de wok difrɛn we. di aktv ingridiεnt na Galafold na wan dכg we dεn kכl `migalastat`. wetin i de du na, i de atak di `alpha-gal` enzym we de insay di sikman in yon bכdi, bכt i nכ stebul. imajin se di `alpha-gal` εnzym na yu bכdi de 'swכl' sכmtεm, 'unstεbul'. So, i nɔ go ebul fɔ wok fayn. wetin di dכg we dεn kכl Galafold de du na, lεk in padi, i de atak dis ‘swεl’ εnzym εn ‘stεbiliz’ am. Dɔn da ɛnzaym de kin ebul fɔ du in wok fayn fayn wan. Dis na wetin wi kin kɔl "Chaperone Therapy" .
Yu tink se ɔlman kin yuz Galafold?
nכ, dεn kin כnli yuz galafold pan pipul dεm we gεt spεsifi k tכp amenable mכtεshכ n na di jin we de mek di `alpha-gal` εnzym. di `alpha-gal` εnzym we dis mכtεshכn de mek stil ebul fכ wok, bכt i tu nכ stebul fכ wok fayn fayn wan. Galafold de atak da ɛnzaym de, i de mek i stebul, ɛn mek i wok.
sכm Fabry pasεnshכn dεm nכ de prodyuz di `alpha-gal` εnzym atכl, כ di εnzym we dεn prodyuz nכ de wok atכl. Galafold nɔ go gɛt ɛni yus to dɛn kayn pipul dɛn de. Bikɔs no ɛnzaym nɔ de na di bɔdi fɔ mek i stebul. Enzyme replacement therapy (ERT) na in fayn fɔ dɛn.
So, bifo yu dɔktɔ gi yu Galafold, i go du spɛshal tɛst fɔ no us kayn jenɛtik mutation yu gɛt. Dis go sho if dis mɛrɛsin go bɛnifit yu ɔ nɔ go bɛnifit yu.
Aw yu kin yuz dis mɛrɛsin?
Dis pat rili impɔtant bikɔs fɔ gɛt di ful bɛnifit fɔ di mɛrɛsin, yu nid fɔ yuz am kɔrɛkt wan.
- Aw fɔ tek: Galafold na kapsul. Dɛn kin tek am ɛvri ɔda de , dat na, ɛvri ɔda de.
- Taym fɔ di de: I rili impɔtant fɔ tek am di sem tɛm ɛvride. Fɔ ɛgzampul, if yu tek am 8 oklɔk mɔnin di fɔs de, yu fɔ tek am 8 oklɔk mɔnin di nɛks de bak.
- Di tin we impɔtant pas ɔl: Dɛn fɔ tek dis mɛrɛsin we yu ɛmti bɛlɛ . Dat min se yu nɔ fɔ it ɛnitin fɔ 2 awa bifo ɔ 2 awa afta yu tek di mɛrɛsin.
- Wan ɔda impɔtant tin: Dɔn bak, nɔ drink ɛnitin we gɛt kafin (lɛk ti, kɔfi, sɔm sɔft drink) fɔ 2 awa bifo yu tek di pil ɛn 2 awa afta yu tek di pil. Insay dis tɛm, yu kin drink wata, frut drink dɛn we nɔ gɛt fayv, ɔ drink dɛn we nɔ gɛt kafin.
Fɔ ɛp yu fɔ mek yu nɔ miks yu de, dis mɛrɛsin de insay wan spɛshal paket. Dɛn kɔl am `blista kad.' I gɛt we fɔ mak di de dɛn. Yu pul di blista kad di de we yu tek di mɛrɛsin, ɛn di de we yu nɔ tek di mɛrɛsin, yu pul di ɛmti sɛklɔ na in ples. Dis we ya, yu kin mak di kad ɛvride ɛn nɔ mis di de we yu nid fɔ tek di mɛrɛsin.
Sɔm sayd ɛfɛkt ɛn prɔblɛm dɛn de wit ɔda mɛrɛsin dɛn?
Sɔm pipul dɛm wae de tek Galafold kin gɛt smɔl risk fɔ gɛt infekshɔn na dɛn respiratory infection (kold, trot we de at) ɛn urinary tract infection. Di bɛst we fɔ mek yu nɔ gɛt di sik na fɔ was yu an ɔltɛm, nɔ de nia pipul dɛn we sik, ɛn drink bɔku wata.
Di tin we impɔtant pas ɔl na dat, if yu gɛt ɛni prɔblɛm we de mɔna yu, we nɔ kɔmɔn we yu tink se na bikɔs ɔf dis mɛrɛsin, nɔ ɛva stɔp fɔ tek am fɔ yusɛf . Mek shɔ se yu tɔk to yu dɔktɔ.
Apat frɔm dat, as wi bin dɔn tɔk bɔt, na kafinSo, i kin bi se Galafold nɔ kin wok fayn. So tek tɛm wit tin dɛn we gɛt kafinɛ. I rili impɔtant bak fɔ tɛl yu dɔktɔ bɔt ɛni ɔda mɛrɛsin, vaytamɛn, ɔ tradishɔnal mɛrɛsin we yu de tek.
Mɛsej we dɛn kin kɛr go na os
- Galafold na spɛshal ɔral mɛrɛsin fɔ sɔm kayn Fabry Disease.
- dis de wok bay we i de stεbyul wan εnzym we dεn כlrεdi na yu bכdi, bכt i nכ stebul, εn mek i wok. Yu nid fɔ du wan jenɛtik tɛst fɔ no if dis fayn fɔ yu.
- Dɛn fɔ tek di mɛrɛsin ɛvri ɔda de, pan ɛmti bɛlɛ. Nɔ it ɛn drink tin dɛn we gɛt kafinɛ 2 awa bifo yu tek di mɛrɛsin ɛn 2 awa afta yu tek di mɛrɛsin.
- Dis mɛrɛsin nɔr go ɛp yu fɔ gɛt di sik wae yu gɛt naw, bɔt i go ɛp fɔ mek yu nɔr gɛt pwɛl hat tumara bambay frɔm de sik.
- If yu gɛt ɛni sayd ɛfɛkt ɔ prɔblɛm, tɔk to yu dɔktɔ wantɛm wantɛm.
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