Created at:1/13/2025
I-Maralixibat iyimithi ekhethekile esiza ukwelapha ukulunywa okukhulu okubangelwa izimo ezithile zesibindi ezinganeni. Isebenza ngokuvimba izithuthi ezithile emathunjini akho eziphinda zimunce ama-bile acids, okuyizinto ezingakwazi ukwakheka futhi zibangele izimpawu ezingakhululeki lapho isibindi sakho singasebenzi kahle.
Le mithi imelela ukuqonda okukhulu emindenini ebhekene nezifo zesibindi ezingavamile. Nakuba ingelaphi, i-maralixibat inganikeza ukukhululeka okukhulu ekulunyeni okukhulu okuvame ukwenza impilo yansuku zonke ibe yinselele ezinganeni ezinalezi zimo.
I-Maralixibat iyimithi yomlomo engaphansi kwezigaba zemithi ebizwa ngokuthi i-ileal bile acid transporter (IBAT) inhibitors. Cabanga ngayo njenge-blocker ekhethekile evimbela amathumbu akho ukuthi angaphindi amunce ama-bile acids, okuvumela ukuthi izinto eziningi ziphume emzimbeni wakho ngokwemvelo.
Imithi iza njengesixazululo somlomo esenzelwe izingane ngqo. Yenzelwe ukuthi ithathwe ngomlomo, ngokuvamile ixutshwe nokudla noma iziphuzo ukuze kube lula ezigulini ezincane ukuthi ziyithathe njalo.
Lo muthi musha emakethe, njengoba uthuthukiswe ngqo ezigulini ze-pediatric ezinezimo zesibindi ezingavamile. Ayisetshenziswa kakhulu ngoba iqondise izimo ezithile zezokwelapha ezithinta inani elincane lezingane.
I-Maralixibat isetshenziswa ikakhulukazi ukwelapha i-cholestatic pruritus ezinganeni ezinohlobo lwe-Alagille syndrome. I-Cholestatic pruritus yigama lezokwelapha lokulunywa okukhulu, okuqhubekayo okwenzeka lapho ama-bile acids akheka emzimbeni wakho ngenxa yezinkinga zesibindi.
I-Alagille syndrome yisimo esingavamile se-genetic esithinta isibindi, inhliziyo, nezinye izitho. Izingane ezinalesi simo zivame ukuzwa ukulunywa okukhulu okungaphazamisa ukulala, isikole, nemisebenzi yansuku zonke. Ukulunywa kungaba kubi kangangokuthi kuthinta ikhwalithi yempilo yazo kakhulu.
Umuthi kungenzeka futhi ucabangwe kwezinye izifo zesibindi se-cholestatic ezinganeni, kodwa lokhu kusetshenziswa akuvamile. Udokotela wakho uzohlola ngokucophelela ukuthi i-maralixibat ilungele yini isimo nezimpawu zengane yakho.
I-Maralixibat isebenza ngokuvimba iphrotheni ethile emathunjini akho ebizwa ngokuthi i-ileal bile acid transporter (IBAT). Le phrotheni ngokuvamile isiza umzimba wakho ukuba uphinde umunce ama-bile acids emathunjini akho abuyele emsingeni wegazi lakho.
Lapho leli thutha livinjelwe, ama-bile acids amaningi adlula emathunjini akho futhi aphume emzimbeni wakho ngamathumbu. Lokhu kusiza ukunciphisa inani lama-bile acids azungeza emsingeni wegazi lakho, okunganciphisa umuzwa wokulunywa.
Umuthi ubhekwa njengomuthi oqinile ngokulinganisela ngenjongo yawo ethile. Akuyona imithi ejwayelekile kodwa kunalokho ukwelashwa okuqondiwe okubhekana nenkinga ethile kakhulu endleleni umzimba wakho oqhubula ngayo ama-bile acids.
I-Maralixibat kufanele ithathwe ngendlela enqunywe ngudokotela wakho, ngokuvamile kanye ngosuku ekuseni. Umuthi uza njengesixazululo somlomo esingahlanganiswa nokudla noma iziphuzo ukuze senze kube mnandi ezinganeni.
Unganika lo muthi ngaphandle noma ngaphandle kokudla, kodwa ukuwuthatha nokudla kungasiza ukunciphisa ukuphazamiseka kwesisu. Imindeni eminingi ikuthola kuwusizo ukuhlanganisa isixazululo nenani elincane le-applesauce, iyogathi, noma ijusi elithandwa yingane yabo.
Kubalulekile ukusebenzisa idivayisi yokulinganisa eza nomuthi ukuze kuqinisekiswe ukulinganisa okunembile. Ungasebenzisi izipuni zasendlini, njengoba zingahluka ngosayizi futhi zingahlinzeki ngomthamo ofanele.
Zama ukunika umuthi ngesikhathi esifanayo nsuku zonke ukuze usize ukusungula umkhuba. Lokhu kuvumelana kusiza ukugcina amazinga aqhubekayo omuthi ohlelweni lwengane yakho.
Ubude bokwelashwa nge-maralixibat buyahluka ngokuya ngesimo sengane yakho nokuphendula emuthini. Ezinganeni ezinomdlavuza we-Alagille, lokhu ngokuvamile kuyindlela yokwelapha yesikhathi eside engase iqhubeke iminyaka.
Udokotela wakho uzohlola njalo inqubekela phambili yengane yakho futhi alungise uhlelo lokwelapha njengoba kudingeka. Ezinye izingane zingase zibone ngcono ekulunyeni emasontweni ambalwa, kanti ezinye zingase zithathe isikhathi eside ukuzwa izinzuzo.
Isinqumo sokuqhubeka noma ukuyeka ukwelashwa kufanele senziwe ngaso sonke isikhathi ngokubonisana nomhlinzeki wakho wezempilo. Bazocabangela izinto ezinjengokuthuthuka kwezimpawu, imiphumela emibi, kanye nesimo sempilo yengane yakho.
Njengayo yonke imithi, i-maralixibat ingabangela imiphumela emibi, nakuba kungewona wonke umuntu oyitholayo. Ukuqonda okukulindele kungakusiza ukuthi uzizwe ulungiseleleke kakhulu futhi wazi ukuthi ungaxhumana nini nomhlinzeki wakho wezempilo.
Imiphumela emibi evame kakhulu ihlobene nezinguquko zesistimu yokugaya ukudla, okwenza umqondo ngendlela umuthi osebenza ngayo emathunjini akho.
Imiphumela emibi evamile ongase uyibone ihlanganisa:
Le miphumela emibi yokugaya ngokuvamile iyathuthuka njengoba umzimba wengane yakho uzivumela emuthini. Kodwa-ke, ukuqunjelwa okuqhubekayo kungase kube ngokuqondene futhi kufanele kuxoxwe nodokotela wakho.
Imiphumela emibi engavamile kodwa ebucayi edinga ukunakekelwa kwezempilo ngokushesha ihlanganisa:
Uma uqaphela noma yiziphi zalezi zinkinga ezingathi sína, xhumana nodokotela wakho ngokushesha. Bangasiza ekutholeni ukuthi ngabe izimpawu zihlobene yini nomuthi futhi balungise ukwelashwa uma kudingeka.
I-Maralixibat ayifanele wonke umuntu, futhi zikhona izimo ezithile lapho lo muthi kufanele ugwenywe noma usetshenziswe ngokuqapha okwengeziwe. Udokotela wakho uzobukeza ngokucophelela umlando wezokwelapha wengane yakho ngaphambi kokuyinika lo muthi.
Izingane okungafanele zithathe i-maralixibat zihlanganisa lezo ezinazo:
Ukuqapha okukhethekile kuyadingeka ezinganeni ezinemibandela ethile engase ikhulise ingozi yezinkinga ezivela emiphumeleni yomuthi ohlelweni lokugaya ukudla.
Udokotela wakho uzobheka neminye imithi ingane yakho eyithathayo, njengoba eminye imithi ingasebenzisana ne-maralixibat. Nqamula uhlu oluphelele lwazo zonke izidakamizwa, izithasiselo, namavithamini ingane yakho ewathathayo.
I-Maralixibat itholakala ngaphansi kwegama lomkhiqizo elithi Livmarli e-United States. Leli yilo kuphela igama lomkhiqizo elitholakala njengamanje ngalo muthi.
I-Livmarli yakhelwe ngqo njengesixazululo somlomo seziguli ze-pediatric. Azikho izinhlobo ezivamile ze-maralixibat ezitholakalayo okwamanje, njengoba kungumuthi omusha.
Umkhiqizo womuthi onegama lomkhiqizo uza nemiyalelo ethile kanye nezinsiza zokulinganisa eziklanyelwe ukulinganisa okunembile ezinganeni. Njalo sebenzisa imikhiqizo eza nemiyalelo kadokotela wakho ukuqinisekisa ukulinganisa okufanele.
Ezinye izindlela zokwelapha i-cholestatic pruritus ezinganeni zilinganiselwe, yingakho i-maralixibat imelela intuthuko enkulu kangaka. Kodwa-ke, zikhona ezinye izindlela udokotela wakho angazicabangela.
Ukwelashwa kwendabuko kokulunywa esifweni sesibindi kufaka phakathi:
Lezi ezinye izindlela zisebenza ngezindlela ezihlukene futhi zingasetshenziswa zodwa noma ngokuhlanganyela ne-maralixibat. Udokotela wakho uzoqoka indlela engcono kakhulu yokwelapha ngokusekelwe esimweni kanye nezimpawu zengane yakho.
Izindlela ezingezona imithi ezifana nokunakekela kahle isikhumba, ukusebenzisa insipho ethambile, nokugcina indawo ipholile futhi inomswakama nazo zingasiza ekuphatheni izimpawu zokulunywa.
I-Maralixibat ne-cholestyramine zisebenza ngendlela ehlukile ukubhekana nezinkinga ezihlobene ne-bile acid, futhi ngamunye unokuhle kwakhe kanye nemikhawulo yakhe. Ukukhetha phakathi kwabo kuncike esimweni esithile sengane yakho kanye nokuphendula ekwelashweni.
I-Maralixibat inikeza ezinye izinzuzo ngaphezu kwe-cholestyramine. Kulula ukuyithatha ngoba iza njengesixazululo esiwuketshezi esingaxutshwa nokudla, kanti i-cholestyramine iyimpushana okungase kube nzima ukuyixuba futhi inokunambitheka okungathandeki.
I-Cholestyramine isebenza ngokubopha ama-bile acids emathunjini, kuyilapho i-maralixibat ivimba ukumuncwa kwawo kabusha. Lo mahluko endleleni yokusebenza usho ukuthi bangase basebenze kangcono ezinganeni ezihlukene noma bangase basetshenziswe ndawonye kwezinye izimo.
Udktela wakho uzocabangela izinto ezifana neminyaka yengane yakho, ikhono lokuthatha imithi, ubukhulu bezimpawu, kanye nezimpendulo zokwelashwa zangaphambilini lapho ekhetha phakathi kwalezi zinketho. Ezinye izingane zingazuza ngokuzama zombili izindlela ukuze zibone ukuthi iyiphi esebenza kangcono kuzo.
Izingane eziningi ezinokugula kwe-Alagille nazo zinezinkinga zenhliziyo, ngakho-ke lokhu kubalulekile ukukucabangela. I-Maralixibat ngokuvamile ingasetshenziswa ngokuphepha ezinganeni ezinamaphutha enhliziyo, kodwa udktela wakho uzodinga ukuqapha ingane yakho ngokucophelela.
Umuthi awuthinti ngqo ukusebenza kwenhliziyo, kodwa imiphumela emibi yokugaya ukudla efana nohudo ingaholela ekuphelelwe amanzi emzimbeni, okungase kubeke ingcindezi eyengeziwe enhliziyweni. Ithimba lakho lezempilo lizosebenzisana ukuqinisekisa ukuthi zonke izici zempilo yengane yakho ziyacatshangelwa.
Uma ngengozi unika ingane yakho i-maralixibat eningi kakhulu, xhumana nodktela wakho noma isikhungo sokulawula ubuthi ngokushesha. Ngenkathi imininingwane yokweqisa ilinganiselwe ngenxa yokusha komuthi, amanani amaningi angase abangele izimpawu ezinzima zokugaya ukudla.
Qaphela izimpawu zohudo olunzima, ukuhlanza, noma ukuphelelwa amanzi emzimbeni, futhi funa ukwelashwa uma lokhu kuvela. Gcina ibhodlela lomuthi unawe lapho ufuna usizo ukuze abahlinzeki bezempilo bakwazi ukubona kahle ukuthi yini nokuthi kungakanani okunikwe.
Uma uphuthelwa umthamo we-maralixibat, mnike ngokushesha lapho ukhumbula, ngaphandle kokuthi sekuyisikhathi esiseduze somthamo olandelayo. Kulokho, weqa umthamo ophuthelwe bese uqhubeka neshejuli ejwayelekile.
Unganiki imithamo emibili ngesikhathi esisodwa ukuze ulungise umthamo ophuthelwe, ngoba lokhu kungandisa ingozi yemiphumela emibi. Uma uvame ukukhohlwa imithamo, cabanga ukusetha izikhumbuzi zocingo noma usebenzise umhleli wemithi ukusiza ukugcina ukuqina.
Ungayeki ukunika i-maralixibat ngaphandle kokuxoxa nodokotela wakho kuqala. Isinqumo sokuyeka ukwelashwa kufanele sisekelwe ekuphenduleni kwengane yakho emuthini, imiphumela emibi, kanye nesimo sempilo esiphelele.
Udokotela wakho angase ancome ukuyeka uma ingane yakho ihlangabezana nemiphumela emibi ebalulekile edlula izinzuzo, noma uma isimo sayo sithuthuka size sibe seqophelweni lapho umuthi ungasadingeki. Bazokuqondisa kunoma yikuphi ukuncishiswa komthamo okudingekayo noma ukuqapha ngesikhathi senqubo yokuyeka.
I-Maralixibat ingasebenzisana neminye imithi, ikakhulukazi lezo ezithathwa emathunjini. Njalo yazisa udokotela wakho ngayo yonke imithi, izithasiselo, namavithamini ingane yakho ewathathayo ngaphambi kokuqala i-maralixibat.
Eminye imithi ingase idinge ukuthathwa ngezikhathi ezihlukene zosuku ukuze kugwenywe ukusebenzisana, kanti eminye ingase idinge ukulungiswa komthamo. Umhlinzeki wakho wezempilo uzokwenza isimiso semithi esiphelele esandisa izinzuzo ngenkathi sinciphisa izingozi.