Tej zaum koj yuav tsis tau hnov txog Kab Mob Fabry ua ntej. Nws yog ib qho mob uas tshwm sim ntau heev, vim nws yog ib qho mob caj ces tsis tshua muaj. Tab sis yog tias koj lossis ib tug neeg hauv koj tsev neeg tau kuaj pom tias muaj tus kab mob no, nws yooj yim to taub tias koj yuav xav tias ntxhov siab heev. Tab sis nco ntsoov, txawm hais tias tsis muaj kev kho rau tus kab mob no, muaj ntau yam kev kho mob zoo niaj hnub no uas tuaj yeem pab koj ua neej nyob li qub li sai tau. Cia peb tham txog cov kev kho mob no hnub no.
Cov kev kho mob tseem ceeb: Kev Kho Mob Enzyme
Hais yooj yim xwb, tus kab mob Fabry yog tshwm sim los ntawm qhov tsis txaus lossis ua haujlwm tsis zoo ntawm cov enzyme uas tseem ceeb rau peb lub cev. Yog li lub hom phiaj tseem ceeb ntawm kev kho mob yog daws qhov teeb meem no. Muaj ob txoj hauv kev tseem ceeb los ua qhov no.
1. Kev Kho Mob Hloov Enzyme (ERT)
Qhov no suav nrog kev hloov cov enzyme 'alpha-galactosidase A (alpha-Gal A)' uas koj lub cev tsis muaj. Zoo li ntxiv roj rau hauv lub tsheb thaum nws tsis muaj roj ntau. Kev kho mob no yog muab los ntawm cov leeg ntshav (kev txhaj tshuaj 'IV'). Feem ntau nws yuav siv txhua ob lub lis piam . Cov tshuaj tseem ceeb uas tam sim no tau pom zoo rau lub hom phiaj no yog 'agalsidase beta (Fabrazyme)'.
Tshwj xeeb tshaj yog tias ib tug txiv neej raug kuaj pom tias muaj tus kab mob no, tus kws kho mob yuav pom zoo kom pib kho tam sim ntawd, txawm tias tsis muaj tsos mob los xij. Qhov laj thawj rau qhov no yog tias txawm tias koj tsis hnov mob dab tsi los xij, cov kab mob sab hauv xws li lub raum thiab lub plawv tej zaum yuav twb pib puas lawm. Yog li pib kho thaum ntxov tuaj yeem tiv thaiv kev puas tsuaj rau cov kab mob ntawd .
Cov poj niam feem ntau tsis tshua raug tus kab mob Fabry. Yog li koj tus kws kho mob yuav tos kom txog thaum koj pib pom cov tsos mob lossis kom txog thaum cov kev kuaj pom tias koj cov kab mob tau raug puas tsuaj. Qee tus neeg yuav mob khaub thuas thiab kub taub hau thaum noj cov tshuaj no. Tab sis tsis txhob txhawj, koj tus kws kho mob yuav muab tshuaj antihistamine rau koj los pab tiv thaiv qhov no.
2. Tshuaj Haus
Qhov no yog ib txoj kev kho mob tshiab. Cov tshuaj no hu ua 'Migalastat (Galafold).' Nws ua haujlwm los ntawm kev pab koj lub cev cov enzyme 'alpha-Gal A', uas tsis ua haujlwm zoo, kom ua haujlwm zoo dua. Nws yooj yim dua los noj cov tshuaj dua li kom txhaj rau hauv cov leeg ntshav.
Tiamsis nws tsis ua haujlwm rau txhua tus. Cov tshuaj no ua haujlwm rau tsawg dua ib nrab ntawm cov neeg uas muaj tus kab mob Fabry. Seb nws puas ua haujlwm lossis tsis ua haujlwm nyob ntawm qee qhov kev hloov pauv (kev hloov pauv ntawm cov noob caj noob ces) hauv koj cov noob caj noob ces. Yog li ua ntej pib txoj kev kho mob no, koj tus kws kho mob yuav ua qhov kev kuaj noob caj noob ces.
Yuav tswj cov tsos mob li cas?
Kab mob Fabry tuaj yeem ua rau muaj ntau yam teeb meem. Feem ntau yog o ntawm ob txhais ceg, kab mob plawv, thiab kab mob raum. Yog li ntxiv rau kev kho mob enzyme, koj tus kws kho mob yuav sau ntawv rau lwm yam tshuaj los pab tswj cov tsos mob no.
| Cov tsos mob/teeb meem | Kev kho mob feem ntau yog muab |
|---|---|
| Mob o thiab mob hnyav heev rau tes thiab taw | Cov tshuaj xws li carbamazepine, gabapentin, lossis phenytoin, uas feem ntau yog muab rau kev mob qaug dab peg, yog muab los tswj qhov mob. |
| Kev puas tsuaj rau lub raum thiab ntshav siab | Cov tshuaj kho ntshav siab xws li ACE inhibitors raug sau ntawv kom noj. Cov no pab tiv thaiv lub raum thaum tswj ntshav siab. |
| Lub plawv dhia tsis xwm yeem | Tej zaum yuav xav tau lwm yam tshuaj kho mob plawv kom lub plawv ua haujlwm tau zoo. |
| Teeb meem ntawm lub cev zom zaub mov (ntuav, raws plab, mob plab) | Tus kws kho mob yuav sau ntawv kom siv cov tshuaj raws li cov tsos mob no. |
| Teeb meem ntawm daim tawv nqaij (cov hlab ntsha me me tshwm sim) | Kws kho mob tawv nqaij tuaj yeem tshem tawm cov no nrog kev kho mob yooj yim. |
| Teeb meem hnov lus (nrov nrov hauv pob ntseg, kiv taub hau, tsis hnov lus zoo) | Cov teeb meem no tuaj yeem zam tau los ntawm kev siv cov khoom pab hnov lus. |
Cov kev kuaj mob uas saib xyuas tus kab mob
Vim tias tus kab mob Fabry cuam tshuam rau koj lub cev tag nrho, cov tsos mob tuaj yeem hnyav zuj zus thaum koj laus zuj zus. Nws yog ib qho tseem ceeb kom koj saib xyuas koj txoj kev noj qab haus huv. Qhov no suav nrog kev kuaj mob tsis tu ncua .
- Kev kuaj ntshav: Cov no pab xyuas koj cov ntshav liab thiab ntshav dawb, cov electrolyte, thiab koj lub raum thiab lub siab ua haujlwm li cas.
- Kev kuaj zis: Xyuas seb puas muaj protein lossis ntshav nyob hauv cov zis. Qhov no tuaj yeem yog ib qho cim qhia ntxov ntawm kab mob raum.
- ECG (Electrocardiogram): Qhov no sau cov teeb liab hluav taws xob ntawm lub plawv thiab xyuas seb puas muaj cov qauv plawv dhia tsis zoo.
- Echocardiogram: Ib daim duab ntawm lub plawv raug thaij los ntawm kev siv cov suab nthwv dej. Qhov no siv tau los xyuas seb puas muaj teeb meem nrog cov qhov valve thiab cov chambers ntawm lub plawv.
- Kev kuaj pob ntseg: Nws yog ib lub tswv yim zoo kom ua qhov kev kuaj no tsawg kawg ib xyoos ib zaug.
- Kev thaij duab lub hlwb: Qhov kev kuaj no, ua txhua ob xyoos ib zaug, nrhiav kev hloov pauv hauv cov hlab ntsha hauv lub hlwb. Qhov no tuaj yeem pab txheeb xyuas cov cim qhia ntxov ntawm kev mob stroke.
Pawg neeg kho mob uas pab koj
Hauv hom mob no, koj tus kws kho mob tsev neeg tsis yog tib tus uas yuav kho koj. Ib pab neeg kws tshwj xeeb hauv ntau qhov chaw yuav pab koj. Nyob ntawm koj cov tsos mob, koj yuav tsum tau mus ntsib cov kws tshwj xeeb no tsawg kawg ib xyoos ib zaug.
- Kws Kho Mob Hlwb: Rau cov teeb meem ntsig txog lub hlwb thiab lub paj hlwb.
- Kws Kho Mob Plawv: Rau cov teeb meem ntsig txog lub plawv.
- Kws Kho Mob Ntsws: Rau cov teeb meem ntsig txog lub raum.
- Kws Kho Mob Qhov Muag: Rau cov teeb meem ntsig txog qhov muag.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- Txawm hais tias Kab Mob Fabry tsis tuaj yeem kho tau tag nrho los xij, nws tuaj yeem tswj tau zoo heev nrog kev kho mob tam sim no thiab tib neeg tuaj yeem ua neej nyob li qub.
- Muaj ob txoj kev kho mob tseem ceeb: kev kho mob enzyme hauv cov hlab ntsha (ERT) thiab cov tshuaj ntsiav noj (Migalastat). Koj tus kws kho mob yuav txiav txim siab seb qhov twg zoo tshaj plaws rau koj.
- Kev tswj cov tsos mob xws li mob, kab mob plawv, thiab kab mob raum yog qhov tseem ceeb heev rau kev txhim kho lub neej zoo.
- Nws yog ib qho tseem ceeb uas yuav tsum tau mus kuaj mob raws sijhawm thiab sib txuas lus tas li nrog pab pawg kws kho mob tshwj xeeb.
- Tham nrog koj tus kws kho mob txog koj tus mob, kev kho mob, thiab tej yam koj txhawj xeeb txog. Tsis txhob ntshai, lawv yeej ib txwm npaj pab koj.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus