Masifunde ngeyeza iMiglustat. Eli lipilisi elisetyenziselwa ukunyanga isifo iGaucher!

Usenokuba awuzange uve kangako ngeli yeza libizwa ngokuba yiMiglustat. Isizathu soku kukuba liyeza elikhethekileyo elinikwa imeko yezonyango engaqhelekanga. Ngoko ke namhlanje siza kuthetha ngeli yeza libizwa ngokuba yiMiglustat, kunye nesifo elisinyangayo elibizwa ngokuba yiGaucher. Ukuba wena okanye umntu omaziyo unikwe eli yeza, olu lwazi luya kuba lubaluleke kakhulu kuwe.

Luhlobo luni lonyango iMiglustat? Isebenza ntoni?

Ngamafutshane, iMiglustat liyeza elinceda ukugcina amanqanaba aqhelekileyo e-enzyme enceda ekuqhekezeni uhlobo lwamafutha emzimbeni wethu. Lisetyenziswa ngokukodwa ukunyanga isifo se-Type 1 Gaucher .

Ngoku usenokuba uzibuza ukuba yintoni isifo seGaucher. Masiqonde oko kancinci. Isifo seGaucher sisifo semfuza. Okwenzekayo koku kukuba umsebenzi we-glucocerebrosidase, i-enzyme efunekayo ukuqhekeza ngokufanelekileyo iintlobo ezithile zamafutha emzimbeni wethu (ingakumbi i-glucocerebroside), uyancipha okanye awukho kwaphela. Emva koko ezi ntlobo zamafutha zigcinwa kwiindawo ezahlukeneyo zomzimba, umzekelo, kwi-spleen, esibindini, nakwi-bone mongo. Oku kunokubangela iingxaki ezahlukeneyo zempilo. Ngoko ke, into eyenziwa liyeza iMiglustat kukunciphisa ukwakheka kwamafutha agqithisileyo emzimbeni.

Eli yeza lidla ngokuthengiswa phantsi kwegama lebrendi elithi Zavesca . Kodwa khumbula, ngamanye amaxesha oogqirha banokulisebenzisa eli yeza nakwezinye iimeko. Ngoko ke ukuba unemibuzo, kungcono ubuze ugqirha wakho okanye ingcali yezemithi.

Zeziphi izinto ekufuneka ndizixelele ugqirha wam ngaphambi kokuba ndisebenzise eli yeza?

Ngaphambi kokuba uqale ukusebenzisa iMiglustat, kukho izinto ezimbalwa ekufuneka uzixelele ugqirha wakho. Ukungazikhankanyi ezi zinto kunokubangela iingxaki ezingeyomfuneko. Ngoko ke thetha nogqirha wakho ngezi zinto.

• Ukuba uneengxaki zemithambo-luvo , oko kuthetha ukutsha, ukuba ndindisholo, ukurhawuzelelwa kwamalungu omzimba.
• Ukuba unesifo sezintso .
• Ukuba une -allergy kwiMiglustat, naliphi na elinye iyeza, ukutya, iidayi zokutya, okanye izithinteli, sixelele, nokuba yinto encinci.
• Ukuba ukhulelwe okanye uzama ukukhulelwa, oku kubaluleke kakhulu.
• Ukuba ungumama oncancisayo.

Xa ugqirha esazi ngezi nyaniso, unokugqiba ukuba eli yeza likulungele okanye alikulungele na, kwaye asebenzise njani ngononophelo.

Ndingayisebenzisa njani kanye kanye le yeza?

Kubaluleke kakhulu ukwazi kakuhle indlela yokusebenzisa eli yeza. Landela imiyalelo kagqirha kunye nelebheli kagqirha enikiweyo yikhemesti.

• Eli yeza lifanele lithathwe ngomlomo neglasi yamanzi.
• Sela amayeza akho ngamaxesha athile , njengoko ugqirha wakho ekuyalele. Umzekelo, ukuba athathwa kathathu ngemini, thatha kanye ngaloo maxesha.
• Ungaze uthathe amayeza angaphezu kwalawo ugqirha wakho akuxelele wona.
• Kwakhona, ungayeki ukuthatha amayeza ngequbuliso ngaphandle kwengcebiso kagqirha wakho. Ukwenza njalo kunokuyenza mandundu imeko yakho.

Ngaba ndifanele ndinike abantwana abancinci eli yeza?

Ukuba unika abantwana abancinci eli yeza, kufuneka uthethe nogqirha wabantwana ngalo uze ulandele iingcebiso zakhe. Kunokufuneka unonophelo olukhethekileyo.

Kuthekani ukuba uthatha amayeza amaninzi kakhulu? (Umthamo ogqithisileyo)

Ukuba ucinga ukuba ngempazamo usele eli yeza kakhulu, fowunela iziko lolawulo lwetyhefu elikufutshane nawe okanye igumbi likaxakeka esibhedlele ngoko nangoko. Musa ukuchitha ixesha.

Inqaku elibalulekileyo: Eli yeza limiselwe wena kuphela. Musa ukulinika omnye umntu nangasiphi na isizathu , nokuba baneempawu ezifanayo nezakho.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Ngamanye amaxesha ungaphoswa yidosi yamayeza. Ukuba oko kuyenzeka, ungakhathazeki.

• Thatha idosi oyiphosileyo ngokukhawuleza nje ukuba uyakhumbula.
• Nangona kunjalo, ukuba sele kusondele ixesha lomthamo wakho olandelayo xa ukhumbula, yeka umthamo ophosiweyo uze uthathe umthamo olandelayo ngexesha elicwangcisiweyo.
• Ungaze uthathe iidosi ezimbini okanye ezingaphezulu ukuze ubuyisele idosi ongayithathanga. Ukwenza njalo kunokubangela iziphumo ebezingalindelekanga.

Ziziphi ezinye iindlela ezinokwenzeka xa usebenzisana nale mithi?

Kukho amayeza athile anokubangela iingxaki xa esetyenziswa ngeMiglustat. Oko kukuthi, anokunciphisa ukusebenza kweMiglustat okanye anyuse iziphumo ebezingalindelekanga.

• Elinye iyeza elinokuthi lichaphazele oku liyeza elibizwa ngokuba yi-imiglucerase .

Olu luhlu aluphelelanga. Ngoko ke, xelela ugqirha wakho malunga nazo zonke ezinye iziyobisi (ezibhalwe ngugqirha kunye nezingengogqirha), imifuno, izongezo zokutya, nokuba uyatshaya, usela utywala, okanye usebenzisa iziyobisi ezingekho mthethweni. Ezinye izinto zinokuchaphazela amayeza akho.

Zeziphi izinto ekufuneka uzilumkele xa usebenzisa eli yeza?

Kukho izinto ezimbalwa ekufuneka uzilumkele xa usebenzisa iMiglustat.

• Bona ugqirha wakho ukuze akuhlole rhoqo. Oku kuya kunceda ugqirha wakho abone ukuba uqhuba njani kwaye ukuba amayeza akho asebenza kakuhle na.
• Kusenokufuneka wenze umsebenzi othile ngelabhoratri ngelixa usela eli yeza. Ugqirha wakho uza kukuxelela ngale nto.
• Abanye abantu banokuba norhudo xa besebenzisa eli yeza. Ukuba oku kuyenzeka, thetha nogqirha wakho malunga nento onokuyenza ngako. Uza kukunika iingcebiso ezifanelekileyo.

Imiyalelo ekhethekileyo yokukhulelwa kunye namadoda:

Le yinxalenye ebaluleke kakhulu, ingakumbi kubafazi namadoda acinga ngokuqala usapho.

• Abafazi: Ukuba uceba ukukhulelwa, okanye ucinga ukuba ukhulelwe, xelela ugqirha wakho ngoko nangoko. Kukho umngcipheko weziphumo ebezingalindelekanga ezibi kumntwana ongekazalwa. Ngoko cela ugqirha wakho ukuba akunike ulwazi oluthe kratya malunga noku.
• Amadoda: Musa ukucwangcisa ukuba nguyise womntwana ngelixa usela eli yeza kwaye emva kweenyanga ezi-3 emva kokuyeka ukusebenzisa eli yeza. Sebenzisa iindlela zokuthintela ukukhulelwa ezisebenzayo ngeli xesha. Kubaluleke kakhulu ukuthetha nogqirha wakho ngale nto.

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengazo zonke iziyobisi, iMiglustat inokubangela iziphumo ebezingalindelekanga, nangona ingenguye wonke umntu ozifumanayo. Ezinye iziphumo ebezingalindelekanga ziyingozi kwaye kufuneka uxelele ugqirha wakho ngoko nangoko ukuba uyazibona. Ezinye zihlala zingekho mbi kakhulu, kodwa ukuba ziyaqhubeka, kulungile ukuxelela ugqirha wakho .

Iziphumo ebezingalindelekanga ekufuneka uzixelele ugqirha wakho ngoko nangoko:

Ukuba ufumana naziphi na kwezi mpawu, xelela ugqirha wakho okanye umboneleli wezempilo ngaphandle kokulibazisa.

• Ukutsha, ukungaziva mnandi, iimvakalelo zokurhawuzelela – ingakumbi emalungwini.
• Utshintsho kumjikelo wokuya exesheni .
• Utshintsho embonweni – umbono uba mfiliba, njengokubona kwiindawo ezimbini.
• Ukungcangcazela – Ukungcangcazela okungalawulekiyo kwamalungu omzimba.
• Ukopha okanye ukukrwela okungaqhelekanga – Ukuba unemikrwelo engaqhelekanga emzimbeni wakho, okanye ukuba ukopha akupheli nokuba kukwenzakala okuncinci.

Iziphumo ebezingalindelekanga ezingezizo ezinzulu, kodwa ekufuneka zixelwe kugqirha wakho ukuba ziyaqhubeka:

Ezi zifo zihlala ziphela emva kweentsuku ezimbalwa, kodwa ukuba ziyaqhubeka okanye ziyakukhathaza, xelela ugqirha wakho .

• Urhudo
• Isiyezi
• Intloko ebuhlungu
• Ukulahlekelwa ngumdla wokutya
• Inani eliphantsi lesidoda – Le yinto echaphazela amadoda.
• Isicaphucaphu, ukuhlanza
• Ukuphazamiseka kwesisu , igesi
• Ukuhla ukusinda

Khumbula, olu luhlu aluphelelanga. Ukuba ubona into engaqhelekanga engekho kolu luhlu, xelela ugqirha wakho ngayo. Nxibelelana nogqirha wakho ukuze ufumane iingcebiso zonyango malunga nemiphumo emibi.

Uligcina njani eli yeza?

Kubaluleke kakhulu ukugcina amayeza ngokufanelekileyo.

• Yigcine kude nabantwana abancinci.
• Gcina kubushushu begumbi, phakathi kwe-15 kunye ne-30 degrees Celsius (59 kunye ne-86 degrees Fahrenheit) . Musa ukuyibeka kwindawo eshushu kakhulu okanye ebandayo.
• Musa ukusebenzisa amayeza aphelelwe lixesha. Lahla kakuhle.

Okokugqibela, amanqaku ambalwa abalulekileyo (Umyalezo Wokuya Ekhaya)

Kulungile, ngoko ke sinethemba lokuba uyiqonda kakuhle le chiza elibizwa ngokuba yiMiglustat.

• Khumbula, eli nqaku lisisishwankathelo nje. Aligubungeli zonke iinkcukacha.
• Ukuba unemibuzo engaphezulu malunga nale yeza, nceda uzive ukhululekile ukubuza ugqirha wakho, ikhemisti, okanye omnye umboneleli wezempilo.
• Ngenxa yokuba isifo seGaucher sisifo esingaqhelekanga kwaye iMiglustat liyeza elithile, kubaluleke kakhulu ukulandela imiyalelo kagqirha wakho.
• Sela amayeza akho ngokuchanekileyo nangexesha elifanelekileyo. Qaphela iziphumo ebezingalindelekanga. Bona ugqirha wakho ngemihla ebekiweyo.

Ndikunqwenelela impilo entle!

I- Miglustat, i-Zavesca, isifo seGaucher, i-enzyme, iziphumo ebezingalindelekanga zamayeza, amayeza kagqirha, isifo seGaucher