Kungenzeka ukuthi awukaze uzwe ngesifo i-Fabry Disease ngaphambili. Yisimo esivame kakhulu, njengoba kuyisimo esingavamile sofuzo. Kodwa uma wena noma othile emndenini wakho etholakale enaso, kuyaqondakala ukuzizwa ukhungathekile. Kodwa khumbula, nakuba kungekho ukwelashwa kwalesi sifo, kunezindlela eziningi zokwelapha ezinhle ezitholakalayo namuhla ezingakusiza uphile impilo evamile ngangokunokwenzeka. Ake sixoxe ngalezi zindlela zokwelapha namuhla.
Izindlela zokwelapha eziyinhloko: Ukwelashwa ngama-enzyme
Kalula nje, isifo se-Fabry sibangelwa ukuntuleka noma ukungasebenzi kahle kwe-enzyme ebalulekile emzimbeni wethu. Ngakho-ke umgomo oyinhloko wokwelashwa ukuxazulula le nkinga. Kunezindlela ezimbili eziyinhloko zokwenza lokhu.
1. Ukwelashwa Kokufaka Ama-Enzyme (ERT)
Lokhu kuhilela ukufaka esikhundleni se-enzyme ethi `alpha-galactosidase A (alpha-Gal A)` entulekayo emzimbeni wakho. Kufana nokufaka uwoyela emotweni uma uwoyela uphansi. Lokhu kwelashwa kunikezwa ngemithambo yegazi (ukufakwa kwe-`IV`). Ngokuvamile kwenzeka njalo emavikini amabili . Umuthi oyinhloko ovunyelwe njengamanje ngale njongo yi-`agalsidase beta (Fabrazyme)`.
Ikakhulukazi uma indoda itholakale inalesi sifo, udokotela angase ancome ukuqala lokhu kwelashwa ngokushesha, noma ngabe azikho izimpawu. Isizathu salokhu ukuthi noma ungazizwa ungakhululekile, izitho zangaphakathi njengezinso nenhliziyo kungenzeka seziqalile ukulimala. Ngakho ukuqala ukwelashwa kusenesikhathi kungavimbela ukulimala kwalezo zitho .
Abesifazane bavame ukungathinteki kakhulu yisifo sikaFabry. Ngakho udokotela wakho angalinda uze uqale ukukhombisa izimpawu noma kuze kube yilapho ukuhlolwa kubonisa ukuthi izitho zakho zonakalisiwe. Abanye abantu bangase babe nokugodola nomkhuhlane ngenkathi bethatha lo muthi. Kodwa ungakhathazeki, udokotela wakho uzokunikeza i-antihistamine ukusiza ukuvimbela lokhu.
2. Imithi Yomlomo
Lokhu kuyindlela entsha yokwelapha. Leli philisi libizwa ngokuthi i-`Migalastat (Galafold).` Lisebenza ngokusiza i-enzyme yomzimba wakho ethi `alpha-Gal A`, engasebenzi kahle, ukuthi isebenze kangcono. Kulula kakhulu ukuphuza iphilisi kunokulifaka emthanjeni.
Kodwa akusebenzi kuwo wonke umuntu. Lo muthi usebenza kubantu abangaphansi kwengxenye abanesifo sikaFabry. Ukuthi uyasebenza noma cha kuncike ezinguqukweni ezithile (ukuguqulwa kwezakhi zofuzo) ezizakhi zofuzo zakho. Ngakho-ke ngaphambi kokuqala lokhu kwelashwa, udokotela wakho uzokwenza ukuhlolwa kwezakhi zofuzo.
Ungazilawula kanjani izimpawu?
Isifo sikaFabry singadala izinkinga ezahlukahlukene. Ezivame kakhulu ukuvuvukala kwamalungu omzimba, isifo senhliziyo, kanye nesifo sezinso. Ngakho-ke ngaphezu kokwelashwa ngama-enzyme, udokotela wakho cishe uzokunikeza eminye imithi ukusiza ukulawula lezi zimpawu.
| Izimpawu/Inkinga | Ukwelashwa okuvame ukunikezwa |
|---|---|
| Ukuvuvukala okukhulu kanye nobuhlungu ezandleni nasezinyaweni | Imithi efana ne -carbamazepine, i-gabapentin, noma i-phenytoin, evame ukunikezwa isifo sokuwa, inikezwa ukulawula ubuhlungu. |
| Ukulimala kwezinso kanye nomfutho wegazi ophezulu | Kunikezwa imithi yokucindezela igazi efana ne -ACE inhibitors . Le mithi isiza ukuvikela izinso ngenkathi ilawula ukucindezeleka kwegazi. |
| Ukuphazamiseka kwenhliziyo | Eminye imithi yenhliziyo ingadingeka ukuze kugcinwe ukusebenza kahle kwenhliziyo. |
| Izinkinga zesistimu yokugaya ukudla (ukuhlanza, isifo sohudo, ubuhlungu besisu) | Udokotela uzokunikeza imithi efanele ngokusekelwe kulezi zimpawu. |
| Izinkinga zesikhumba (kuvela imithambo yegazi emincane) | Udokotela wesikhumba angazisusa lezi zifo ngokwelashwa okulula. |
| Izinkinga zokuzwa (ukukhala ezindlebeni, isiyezi, ukulahlekelwa ukuzwa) | Lezi zinkinga zingagwenywa ngokusebenzisa izinsiza zokuzwa. |
Ukuhlolwa okuqapha isimo sesifo
Ngenxa yokuthi isifo sikaFabry sithinta umzimba wakho wonke, izimpawu zingaba zimbi njengoba ukhula. Kubalulekile ukuhlala uqaphile impilo yakho. Lokhu kuhlanganisa ukuhlolwa njalo kwezokwelapha .
- Ukuhlolwa kwegazi: Lokhu kusiza ekuhloleni inani lamaseli akho abomvu namhlophe egazi, amazinga e-electrolyte, kanye nendlela izinso zakho nesibindi ezisebenza ngayo.
- Ukuhlolwa komchamo: Hlola iphrotheni noma igazi emchameni. Lokhu kungaba uphawu lokuqala lwesifo sezinso.
- I-ECG (i-Electrocardiogram): Lokhu kurekhoda izimpawu zikagesi zenhliziyo futhi kuhlole amaphethini okushaya kwenhliziyo angajwayelekile.
- I-Echocardiogram: Isithombe senhliziyo sithathwa kusetshenziswa amaza omsindo. Lokhu kungasetshenziswa ukuhlola izinkinga ngama-valve namakamelo enhliziyo.
- Ukuhlolwa kokuzwa: Kungumqondo omuhle ukuthi lokhu kuhlolwa kwenziwe okungenani kanye ngonyaka.
- Ukuthwebula izithombe zobuchopho: Lokhu kuhlolwa, okwenziwa cishe njalo eminyakeni emibili, kubheka izinguquko emithanjeni yegazi ebuchosheni. Lokhu kungasiza ekuboneni izimpawu zokuqala zesifo sohlangothi.
Ithimba lezokwelapha elikusizayo
Kulesi simo, udokotela womndeni wakho akuyena yedwa ozokwelapha. Ithimba lochwepheshe emikhakheni eyahlukene lizokusiza. Kuye ngezimpawu zakho, kungadingeka ukuthi ubonane nalaba ochwepheshe okungenani kanye ngonyaka.
- Udokotela Wezinzwa: Ngezinkinga ezihlobene nobuchopho kanye nesimiso sezinzwa.
- Udokotela Wenhliziyo: Ngezinkinga ezihlobene nenhliziyo.
- Udokotela Wezinso: Ngezinkinga ezihlobene nezinso.
- Udokotela wamehlo: Ngezinkinga ezihlobene namehlo.
Umlayezo Wokuya Nawe Ekhaya
- Nakuba isifo sikaFabry singelapheki ngokuphelele, singaphathwa ngempumelelo enkulu ngemithi yamanje futhi abantu bangaphila impilo evamile.
- Kunezindlela ezimbili zokwelapha eziyinhloko: ukwelashwa nge-enzyme ngemithambo yegazi (ERT) kanye namaphilisi omlomo (Migalastat). Udokotela wakho uzonquma ukuthi yikuphi okungcono kakhulu kuwe.
- Ukulawula izimpawu ezifana nobuhlungu, isifo senhliziyo, nesifo sezinso kubaluleke kakhulu ukuthuthukisa ikhwalithi yokuphila.
- Kubalulekile ukuhlolwa kwezokwelapha ngesikhathi futhi uhlale uxhumana nethimba lezokwelapha elingochwepheshe.
- Khuluma ngokukhululekile nangokwethembeka nodokotela wakho ngesimo sakho, izinketho zokwelapha, kanye nanoma yiziphi izinkinga ongase ube nazo. Ungesabi, bahlala bekulungele ukukusiza.
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