Usenokuba awukaze uve ngesifo sikaFabry ngaphambili. Sisifo esixhaphakileyo, kuba sisifo esingaqhelekanga esibangelwa yimfuza. Kodwa ukuba wena okanye umntu osapho lwakho ufunyenwe enaso, kuyaqondakala ukuba uzive uxinezelekile. Kodwa khumbula, nangona kungekho nyango lwesi sifo, kukho unyango oluninzi oluhle olufumanekayo namhlanje olunokukunceda uphile ubomi obuqhelekileyo kangangoko kunokwenzeka. Masithethe ngezi ndlela zonyango namhlanje.
Iindlela eziphambili zonyango: Unyango lwe-Enzyme
Ngamafutshane, isifo sikaFabry sibangelwa kukunqongophala okanye ukungasebenzi kakuhle kwe-enzyme ebalulekileyo emzimbeni wethu. Ngoko ke injongo ephambili yonyango kukusombulula le ngxaki. Zimbini iindlela eziphambili zokwenza oku.
1. Unyango Lokutshintshwa Kwe-Enzyme (ERT)
Oku kuquka ukutshintsha i-enzyme ethi `alpha-galactosidase A (alpha-Gal A)` engabikho emzimbeni wakho. Kufana nokongeza ioyile emotweni xa ioyile incinci. Olu nyango lunikwa ngemithambo yegazi (i-`IV` infusion). Ihlala isenziwa rhoqo emva kweeveki ezimbini . Iyeza eliphambili elivunyiweyo ngoku yi-`agalsidase beta (Fabrazyme)`.
Ingakumbi ukuba indoda ifunyenwe inale sifo, ugqirha unokucebisa ukuba iqale olu nyango ngoko nangoko, nokuba akukho zimpawu. Isizathu soku kukuba nokuba awuziva ungonwabanga, amalungu angaphakathi anjengezintso nentliziyo asenokuba sele eqalile ukonakala. Ngoko ke ukuqala unyango kwangethuba kunokuthintela umonakalo kuloo malungu .
Abafazi badla ngokungachaphazeleki kakhulu sisifo sikaFabry. Ngoko ke ugqirha wakho angalinda ude uqale ukubonisa iimpawu okanye ude uvavanyo lubonise ukuba amalungu akho omzimba onakele. Abanye abantu banokuba nokugodola kunye nomkhuhlane ngelixa bethatha olu nyango. Kodwa ungakhathazeki, ugqirha wakho uza kukunika i-antihistamine ukunceda ukuthintela oku.
2. Amayeza Omlomo
Olu lunyango olutsha. Le pilisi ibizwa ngokuba yi-`Migalastat (Galafold).` Isebenza ngokunceda i-enzyme yomzimba wakho ethi `alpha-Gal A`, engasebenzi kakuhle, ukuba isebenze ngcono. Kulula kakhulu ukusela ipilisi kunokuyifaka emthanjeni.
Kodwa ayisebenzi kuwo wonke umntu. Eli yeza lisebenza kubantu abangaphantsi kwesiqingatha abanesifo sikaFabry. Nokuba liyasebenza okanye alisebenzi kuxhomekeke kutshintsho oluthile (utshintsho lwezakhi zofuzo) kwiizakhi zofuzo zakho. Ngoko ke ngaphambi kokuba uqale olu nyango, ugqirha wakho uza kwenza uvavanyo lwezakhi zofuzo.
Ungazilawula njani iimpawu?
Isifo sikaFabry sinokubangela iingxaki ezahlukeneyo. Ezona zixhaphakileyo kukudumba kwamalungu omzimba, isifo sentliziyo, kunye nesifo sezintso. Ngoko ke ukongeza kunyango lwee-enzyme, ugqirha wakho usenokukunika amanye amayeza okunceda ukulawula ezi mpawu.
| Iimpawu/Ingxaki | Unyango oluhlala lunikwa |
|---|---|
| Ukudumba okukhulu kunye nentlungu ezandleni nasezinyaweni | Amayeza anjenge -carbamazepine, i-gabapentin, okanye i-phenytoin, adla ngokunikezelwa kwisifo sokuwa, anikwa ukulawula iintlungu. |
| Ukonakala kwezintso kunye noxinzelelo lwegazi oluphezulu | Kunikwa amayeza oxinzelelo lwegazi afana ne -ACE inhibitors . La mayeza anceda ekukhuseleni izintso ngelixa elawula uxinzelelo lwegazi. |
| Ukungahambi kakuhle kwentliziyo | Amanye amayeza entliziyo anokufuneka ukuze kugcinwe ukusebenza kakuhle kwentliziyo. |
| Iingxaki zenkqubo yokugaya ukutya (ukuhlanza, urhudo, iintlungu zesisu) | Ugqirha uza kunika amayeza afanelekileyo ngokusekelwe kwezi mpawu. |
| Iingxaki zolusu (kubonakala imithambo yegazi emincinci) | Ugqirha wesikhumba angazisusa ezi ngonyango olulula. |
| Iingxaki zokuva (ukukhala ezindlebeni, isiyezi, ukulahlekelwa kukuva) | Ezi ngxaki zingaphetshwa ngokusebenzisa izixhobo zokuva. |
Uvavanyo olujonga imeko yesifo
Ngenxa yokuba isifo sikaFabry sichaphazela umzimba wakho wonke, iimpawu zinokuba mandundu njengoko ukhula. Kubalulekile ukuhlala uqaphela impilo yakho. Oku kuquka ukuvavanywa rhoqo ngugqirha .
- Uvavanyo lwegazi: Olu vavanyo lunceda ekujongeni ukubala kweeseli zakho ezibomvu nezimhlophe, amanqanaba e-electrolyte, kunye nendlela ezisebenza ngayo izintso zakho nesibindi.
- Uvavanyo lomchamo: Jonga iproteni okanye igazi kumchamo. Oku kungaba luphawu lokuqala lwesifo sezintso.
- I-ECG (i-Electrocardiogram): Le irekhoda imiqondiso yombane yentliziyo kwaye ijonga iipatheni zentliziyo ezingaqhelekanga.
- I-Echocardiogram: Umfanekiso wentliziyo uthathwa kusetyenziswa amaza esandi. Oku kungasetyenziselwa ukujonga iingxaki kwiivalvu kunye namagumbi entliziyo.
- Uvavanyo lokuva: Kungumbono olungileyo ukuba olu vavanyo lwenziwe ubuncinane kanye ngonyaka.
- Uvavanyo lwengqondo: Olu vavanyo, olwenziwa phantse rhoqo emva kweminyaka emibini, lujonga utshintsho kwimithambo yegazi engqondweni. Oku kunokunceda ekuchongeni iimpawu zokuqala zestroke.
Iqela lezonyango elikuncedayo
Kwimeko enjalo, ugqirha wakho wosapho akanguye yedwa oza kunyanga. Iqela leengcali kwiinkalo ezahlukeneyo liza kukunceda. Ngokuxhomekeke kwiimpawu zakho, unokufuna ukubona ezi ngcali ubuncinane kanye ngonyaka.
- Ingcali yezifo zengqondo: Ngeengxaki ezinxulumene nengqondo kunye nenkqubo yemithambo-luvo.
- Ingcali yentliziyo: Ngeengxaki ezinxulumene nentliziyo.
- Ingcali yezifo ze-Nephrologist: Kwiingxaki ezinxulumene nezintso.
- Ingcali yamehlo: Ngeengxaki ezinxulumene namehlo.
Umyalezo Wokuya Ekhaya
- Nangona isifo sikaFabry singenakunyangwa ngokupheleleyo, sinokulawulwa ngempumelelo kakhulu ngonyango lwangoku kwaye abantu banokuphila ubomi obuqhelekileyo.
- Kukho iindlela ezimbini eziphambili zonyango: unyango lwe-enzyme yemithambo yegazi (ERT) kunye neepilisi zomlomo (Migalastat). Ugqirha wakho uya kugqiba ukuba yeyiphi efanelekileyo kuwe.
- Ukulawula iimpawu ezifana neentlungu, isifo sentliziyo, kunye nesifo sezintso kubaluleke kakhulu ukuphucula umgangatho wobomi.
- Kubalulekile ukuba uhlolwe ngugqirha ngexesha kwaye uhlale unxibelelana rhoqo neqela lezonyango eliyingcali.
- Thetha ngokukhululekileyo nangokunyaniseka nogqirha wakho malunga nemeko yakho, iindlela zonyango, kunye nazo naziphi na iingxaki onokuba nazo. Ungoyiki, basoloko bekulungele ukukunceda.
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