Uma udokotela wakho ekutshela noma othandekayo wakho ukuthi unesifo iGaucher, ungase uzizwe ushaqekile futhi wesaba. Lokho kuyinto evamile. Kodwa into ebalulekile ukuthi kunezindlela eziningi zokwelapha ezinhle ezitholakalayo namuhla ezingakusiza uphile impilo ekhululekile ngalesi simo. Nakuba kungekho ikhambi lalesi sifo, izindlela zokwelapha zamanje zingakusiza ukuvimbela ukulimala kwezitho zakho nokuphatha izimpawu zakho ukuze uphile impilo enempilo. Ngakho ungakhathazeki, ake sixoxe ngakho ngokuningiliziwe.
Ezinye izindlela zokwelapha zilungele abantu abadala kuphela. Ezinye izindlela zokwelapha zenzelwe ngqo izinhlobo ezithile zesifo seGaucher. Futhi, uma wena noma ingane yakho ninezimpawu ezincane kakhulu, kungenzeka ningadingi ukwelashwa nhlobo. Kuleso simo, kungadingeka ukuthi nibone ukuthi izimpawu zenu ziyathuthuka yini bese niqala ukwelashwa kuphela uma kudingeka. Konke lokhu kunqunywa udokotela wakho.
Indlela eyinhloko yokwelapha: Ukwelashwa Kokufaka I-Enzyme (ERT)
Lolu uhlobo lokwelapha oluvame kakhulu nolusebenza kahle kakhulu. Abantu abadala nezingane ezinesifo se-Gaucher sohlobo 1 nohlobo 3 bangathola lolu hlobo lokwelashwa.
Kalula nje, isifo se-Gaucher yisimo lapho ikhono lomzimba lokukhiqiza i-enzyme ethile lincipha khona. Ngenxa yalokho, amanye amafutha emzimbeni awakwazi ukuhlukaniswa kahle futhi afakwa ezindaweni ezifana nesibindi, udevu, kanye nomnkantsha wamathambo. Ukwelashwa Kokufaka I-Enzyme Replacement Therapy (ERT) kuhilela ukunikeza umzimba i-enzyme engekho ngaphandle, njengomjovo.
Lokhu kwelashwa kufana nokufaka isibani esisha endlini yethu esikhundleni sesibani esiphukile. Kusiza umzimba ukuthi usebenze kahle futhi ngokuhlinzeka ngalokho okuntulekayo ngaphandle.
Yiziphi izinzuzo zokwelashwa kwe-ERT?
- Ukuvuselela isibindi esivuvukele kanye nobende: Lezi zitho, eziye zanda ngenxa yalesi sifo, zenziwa zibe zincane futhi umsebenzi wazo uyathuthuka.
- Ukunciphisa i-Anemia: Lokhu kusiza ukulawula i-anemia, okubangela ukukhathala nobuthakathaka kubantu abaningi.
- Ukuqinisa amathambo: Kunciphisa ubuhlungu bamathambo, kuwaqinise, futhi kuwavimbele ukuthi aphuke kalula.
Kodwa-ke, kubalulekile ukukhumbula ukuthi i-ERT ayisebenzi kahle ekulawuleni izimpawu zobuchopho noma zesimiso sezinzwa (njengokuxhuzula) ezenzeka kwisifo se-Gaucher sohlobo lwesithathu. Lokhu kungenxa yokuthi umuthi unamandla alinganiselwe okudlula usuka egazini lethu uye ebuchosheni (isithiyo segazi nobuchopho).
Kunezinhlobo ezintathu eziyinhloko zemithi ye-ERT esetshenziswayo njengamanje:
- I-Imiglucerase (I-Imiglucerase - i-Cerezyme)
- I-Taliglucerase alfa (i-Taliglucerase alfa - i-Elyso)
- I-Velaglucerase alfa (VPRIV)
Lo muthi unikezwa njengesixazululo sikasawoti (umuthi we-IV), ngepayipi elincane ungene emthanjeni. Ngokuvamile kufanele uye esibhedlela noma emtholampilo njalo emavikini amabili ukuze uthole lokhu kwelashwa zingakapheli amahora angu-1-2.
Imithi Yomlomo
Ngaphezu kokwelashwa kwe-ERT, kukhona namaphilisi omlomo angathathwa. Lawa anqunyelwe ngqo abantu abadala abanesifo se-Type 1 Gaucher. Lawa maphilisi asebenza ngokunciphisa ukuqongelela kwalawo mafutha emzimbeni esikhulume ngawo ngaphambili.
| Igama lomuthi | Incazelo kanye nokusetshenziswa |
|---|---|
| I-Eliglustat (Eliglustat - Cerdelga) | Lona umuthi omusha kakhulu. Udinga ukuthathwa kabili ngosuku. Kodwa-ke, kwabanye abantu abaphula lo muthi ngokushesha emizimbeni yabo (ama-metabolizer asheshayo), ungase ungasebenzi kahle. |
| I-Miglustat (Miglustat - Zavesca) | Lokhu kunikezwa abantu abadala abanesifo se-Gaucher sohlobo 1 esingatheni kuya kwesiphakathi abangakaphenduli ekwelashweni kwe-ERT. I-capsule eyodwa kufanele ithathwe kathathu ngosuku. |
Ezinye izindlela zokwelapha ezisizayo
Kunezinye izindlela zokwelapha eziningana ezitholakalayo zokulawula izinkinga ezahlukahlukene zesifo seGaucher.
Ukwelashwa kwamathambo abuthakathaka
Ubuthakathaka bamathambo nobuhlungu kuvamile esifweni iGaucher. Uhlobo lwemithi ebizwa ngokuthi ama-bisphosphonates lusetshenziswa ukusiza ngalesi simo. Lokhu kusebenza ngokuqinisa amathambo. Izibonelo zifaka phakathi i-alendronate (i-Fosamax) kanye ne-pamidronate (i-Aredia). Lokhu kufaneleka kokubili ezinganeni nakubantu abadala.
Ukwelashwa Kobuhlungu
Ungasebenzisa imithi yokubulala izinhlungu evamile ezintweni ezifana nobuhlungu bomzimba kanye nobuhlungu bamalunga.
- Ngobuhlungu obuncane: Imithi efana ne-acetaminophen (Tylenol) noma i-ibuprofen (Advil, Motrin).
- Uma ubuhlungu obukhulu: Kungase kudingeke ukuthi uphuze amaphilisi okudambisa izinhlungu anamandla eqenjini lama-opioid , njengoba kuceliwe udokotela wakho.
Ngaphezu kwalokho, ngezinye izikhathi imithi yokucindezeleka noma imithi yokuphumula imisipha ingasiza ekulawuleni ubuhlungu.
Ukuhlinzwa
Nakuba lesi sifo singalawulwa ngemithi, kwezinye izimo kungase kudingeke ukuhlinzwa.
- Ukuhlinzwa Kwesinqe: Uma isinqe esivuvukele singanciphi ngemva kokwelashwa kwe-ERT futhi siqhubeka nokubanga izinkinga, udokotela wakho angase ancome ukususwa kwesinqe ngokuhlinzwa.
- Ukuhlinzwa Kokufaka Ilungu Esihlangwini: Uma lesi sifo silimaze kakhulu amalunga (isib. ilunga lesinqe) futhi ubuhlungu bukhulu kangangokuthi ukuhamba akunakwenzeka, ukuhlinzwa kungenziwa ukuze kufakwe ilunga lokufakelwa esikhundleni salo. Lokhu kunganciphisa ubuhlungu futhi kuvumele ukuhamba okungcono futhi.
Ukufakelwa Komnkantsha Wamathambo
Lokhu kuyindlela yokwelapha eyingozi kakhulu futhi engavamile yesifo iGaucher. Kuhilela ukufaka esikhundleni amaseli egazi alimele ngamangqamuzana anempilo. Kodwa-ke, abukho ubufakazi obucacile bokuphepha nokusebenza kwayo uma kuqhathaniswa nokwelashwa okujwayelekile njenge-ERT. Ngakho-ke, kucatshangelwa kuphela ezimweni ezikhethekile kakhulu lapho kungekho okunye ukwelashwa okuye kwasebenza khona.
Ukumpontshelwa Igazi
Lokhu kungaba wusizo kubantu abane -anemia enkulu ebangelwa yisifo seGaucher. Ukunikela ngegazi kungandisa inani lamaseli abomvu egazi, kubuyisele amandla omzimba kanye namandla.
Ukubaluleka kwendlela yokuphila: ukudla nokuzivocavoca
Ngaphezu kokuthola ukwelashwa, kubaluleke kakhulu nokugcina indlela yokuphila enhle nenempilo.
- Ukudla okunomsoco: Kubalulekile ukudla ukudla okucebile nge -calcium ne-vitamin D , ikakhulukazi ukugcina amathambo eqinile. Faka izinto ezifana nobisi, iyogathi, ushizi, imifino eluhlaza, nenhlanzi encane ekudleni kwakho.
- Ukuzivocavoca: Khuluma nodokotela wakho bese uthola ukuzivocavoca okufanele wena futhi okungakucindezeli amalunga akho. Izinto ezifana nokuhamba nokubhukuda zinhle kakhulu.
- Izithasiselo: Uma udokotela wakho ecabanga ukuthi awutholi i-vitamin D noma i-calcium eyanele ekudleni kwakho, angase akuncome izithasiselo.
Okokugcina, ososayensi nodokotela bahlala becwaninga izindlela ezintsha zokwelapha isifo iGaucher. Lokhu kubizwa ngokuthi izivivinyo zemitholampilo . Lezi zivivinyo zikuvumela ukuthi uzame izindlela ezintsha zokwelapha ezingakatholakali kabanzi. Khuluma nodokotela wakho mayelana nokuthi ungahlanganyela yini kwenye yalezi zivivinyo.
Umlayezo Wokuya Nawe Ekhaya
- Isifo iGaucher akusona isifo okufanele sesatshwe futhi silahlwe. Kunezindlela zokwelapha ezisebenzayo ezitholakalayo namuhla ezingakusiza ukulawula izimpawu zakho futhi uphile impilo enhle.
- Ukwelashwa okuyinhloko ukwelashwa kokushintsha ama-enzyme (ERT). Ngaphezu kwalokho, kusetshenziswa imithi yomlomo, imithi yokuqinisa amathambo, kanye nemithi yokubulala izinhlungu.
- Kwezinye izimo, ukuhlinzwa kwenziwa kuphela uma imithi ingakwazi ukulawula lesi simo.
- Ngaphezu kokuthola ukwelashwa, kubaluleke kakhulu nokudla ukudla okuhle okucebile nge-calcium ne-vitamin D nokwenza ukuzivocavoca okukufanele.
- Into ebaluleke kakhulu ukuhlala uxhumana nodokotela wakho njalo futhi ulandele imiyalelo yakhe ngqo. Khuluma naye ngokukhululekile nganoma yimiphi imibuzo, ukwesaba, noma ukungabaza onakho.
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