Oko kufuneka ukwazi malunga neepilisi zeMigalastat (zesifo sikaFabry)

Ngaba ugqirha wakho ukunike wena okanye umntu omaziyo ukuba athathe iyeza elibizwa ngokuba yiMigalastat ? Eli asiloyeza lixhaphakileyo kwilizwe lethu. Ngoko ke usenokuba nemibuzo emininzi malunga neli yeza. Kuqhelekile ukucinga izinto ezifana nokuthi "Kuza kwenzeka ntoni ngale nto?", "Ndingayithatha njani?", "Ngaba kuya kubakho naziphi na iziphumo ebezingalindelekanga ezicaphukisayo?" Ungakhathazeki, masithethe ngayo yonke le nto ngokulula nangokucacileyo.

Kalula nje, yintoni iMigalastat?

IMigalastat liyeza elikhethekileyo elinikwa abantu abaneSifo sikaFabry. Ngoku usenokuba uzibuza, "Yintoni iSifo sikaFabry?"

Cinga ngemizimba yethu njengomzi-mveliso omkhulu. Kule mzi-mveliso, kukho abasebenzi abaninzi abancinci abanceda yonke into isebenze ngokufanelekileyo. Sibabiza ngokuba zii-enzymes . Emzimbeni womntu onesifo sikaFabry, i-enzyme ethile ayiveliswanga ngokuchanekileyo , okanye ayisebenzi ngokufanelekileyo. Umsebenzi ophambili wale enzyme kukuqhekeza uhlobo lwento enamafutha (lipids) ngaphakathi kwiiseli zethu.

Ngoku, kwenzeka ntoni xa le enzyme ingasebenzi? Ezo zinto zinamafutha ziqala ukuqokelelana kancinci kancinci kwizitho ezibalulekileyo ezifana namehlo, izintso kunye nentliziyo . Kufana nothuli olunamathelayo kumbhobho. Ekuhambeni kwexesha, oku kunokubangela iingxaki ezahlukeneyo zempilo.

Nantsi into eyenziwa ziipilisi zeMigalastat: Zinceda loo enzyme ingasebenzi kakuhle. Kufana nokunika amandla kancinci kumsebenzi odiniweyo uze umxelele uthi, "Kulungile, ngoku yenza umsebenzi wakho ngokufanelekileyo." Eli yeza libuyisela loo enzyme lize liqalise ukuqhekeza amafutha agciniweyo. Oku kubangela ukuncipha kweempawu kunye nomngcipheko ophantsi weengxaki zempilo ezinzulu.

Izinto ekufuneka uzixelele ugqirha wakho ngaphambi kokuba uqale amayeza

Ngaphambi kokuba uqale iMigalastat, kubaluleke kakhulu ukuthetha nogqirha wakho ngokukhululekileyo. Okukhona besazi ngakumbi ngempilo yakho, kokukhona benokukunyanga ngokukhuselekileyo. Qiniseka ukuba ubaxelela ngezi zinto ngokukodwa.

• Isifo sezintso : Ukuba unesifo esinxulumene nezintso, nceda usixelele ngaso.
• Ii-Allergies : Une- allergy kwiMigalastat okanye nakweyiphi na enye iyeza, ukutya, okanye idayi.) Ukuba ikho, yitsho ngokuqinisekileyo.
• Ukukhulelwa : Ukuba ukhulelwe okanye uceba ukukhulelwa, nceda usixelele ngako.
• Ukuba ungumama oncancisayo: Kwazise nogqirha ukuba ungumama oncancisayo.

Yeyiphi indlela engcono yokusebenzisa eli yeza?

Ukuze ufumane okuninzi kweli yeza, kubaluleke kakhulu ukulisebenzisa ngokuchanekileyo. Nceda ukhumbule le miyalelo ngononophelo.

• Sela ngamanzi: Ginya le pilisi yonke ngeglasi yamanzi.
• Musa ukuphula, ukutyumza, okanye ukuhlafuna: Ipilisi mayiginywe yonke. Ungaze uyiqhekeze, uyityumze, okanye uyihlafune ngaphambi kokuba uyithathe.
• Yitya ungenanto esiswini: Le yeyona nto ibalulekileyo. Ufanele uyithathe le yeza ungenanto esiswini. Oko kuthetha ukuba, kufuneka uyithathe kwiiyure ezi-2 ngaphambi okanye kwiiyure ezi-2 emva kokutya.
• Umzekelo olula: Khawucinge ukuba usela amayeza akho ngo-8 kusasa. Emva koko kufuneka utye de kube yintsimbi yesi-6 kusasa. Emva kokusela amayeza akho, ungaphinda utye emva kwentsimbi ye-10 kusasa. Oko kuthetha ukuba isisu sakho kufuneka sibe singenanto ubuncinane iiyure ezi-4 phakathi kokusela amayeza akho.
• Oko unokukusela: Kulungile ukusela amanzi (alula, anencasa, okanye anencasa), ijusi yeziqhamo engenazo iiswekile ezongeziweyo, kunye neziselo ezine-carbonated ezingenayo i-caffeine ngeli xesha.
• Musa ukuyeka de ugqirha wakho akuxelele ukuba: Qhubeka usebenzisa eli yeza de ugqirha wakho akuxelele ukuba uyeke.

Kuthekani ukuba uyinika abantwana?

Eli yeza lingadinga ukhathalelo olukhethekileyo xa linikwa abantwana. Ngoko ke, thetha nogqirha wakho ngalo mba kwaye ulandele imiyalelo yakhe ngokuchanekileyo.

Kuthekani ukuba ndithatha amayeza amaninzi kakhulu?

Ukuba ucinga ukuba uthathe amayeza amaninzi kunokuba ucinga, yiya kwiSebe lezeMpilo eziNgxamisekileyo (ETU) lesibhedlele esikufutshane ngoko nangoko. Okanye utsalele umnxeba iZiko loLwazi lweeTyhefu leSizwe kwiSibhedlele sikaZwelonke saseColombo ukuze ufumane ingcebiso.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Ngamanye amaxesha ungalibala ukusela amayeza akho. Ukuba oko kuyenzeka, ungakhathazeki.

• Lihlawule ixesha oliphosileyo ngokukhawuleza xa ukhumbula.
• Nangona kunjalo, ukuba sele kudlule ixesha elide ukuthatha idosi ephosiweyo kwaye kusele iiyure ezingaphantsi kwe-12 ngaphambi kwedosi elandelayo, yiyeke idosi ephosiweyo.
• Thatha idosi yakho elandelayo ngexesha eliqhelekileyo. Ungaze uthathe iidosi ezimbini ngaxeshanye ukuze ubuyisele idosi oyiphosileyo.

Ngaba kukho izinto ezingahambelaniyo nale yeza?

Zimbalwa izinto ezinokusebenzisana neMigalastat, kodwa kukho enye ephambili.

• I-Caffeine: I-Caffeine, efumaneka kwikofu, iti, nakwezinye iziselo ezibandayo, inokuphazamisa ukusebenza kakuhle kwale mayeza. Kungcono ukuphepha nantoni na equlethe i-caffeine kangangeeyure ezi-4 ozidingayo ukuze ungabi nanto esiswini.

Olu luhlu lusenokungaphelelanga. Ngoko ke, xelela ugqirha wakho ngazo zonke ezinye iziyobisi, iziqholo, iivithamini, kunye nezongezo ozithathayo. Kwakhona xelela ugqirha wakho ukuba uyatshaya okanye usela utywala.

Iziphumo ebezingalindelekanga ezinokubakho ngenxa yamayeza

Njengalo naliphi na iyeza, iMigalastat inokubangela iziphumo ebezingalindelekanga kwabanye abantu. Nangona kunjalo, ayinguye wonke umntu ozifumanayo. Makhe sijonge ukuba zeziphi ezi ziphumo ebezingalindelekanga kunye nento ekufuneka yenziwe ukuba ziyenzeka.

Olu luhlu alubandakanyi zonke iziphumo ebezingalindelekanga. Ukongeza, ukuba ufumana into engaqhelekanga, thetha nogqirha wakho ngayo.

Ugcina njani amayeza akho ekhuselekile?

Ukuze kugcinwe umgangatho weyeza, kufuneka ligcinwe ngokuchanekileyo.

• Gcina kude nabantwana kunye nezilwanyana zasekhaya.
• Gcina kubushushu begumbi (phakathi kwama-20 nama-25 degrees Celsius). Khusela kubushushu obugqithisileyo okanye kubanda kakhulu.
• Gcina iyeza kwiphakheji yalo de kufike ixesha lokulisebenzisa. Likhusele ekufumeni.
• Musa ukusebenzisa amayeza aphelelwe lixesha.

Ndingawalahla njani amayeza angasetyenziswanga okanye aphelelwe lixesha?

Akuyongcinga ilungileyo ukulahla amayeza aphelelwe lixesha enkunkuma okanye ukuwalahla endlwini yangasese. Angonakalisa okusingqongileyo. Buza ugqirha wakho okanye isibhedlele ngale nto. Ngamanye amaxesha izibhedlele zinokuba neenkqubo zokubuyisela la mayeza.

Ukuba akukho ndlela injalo aze ugqirha athi kukhuselekile ukuyilahla enkunkuma, nantsi into omawuyenze:

1. Khupha iyeza kwiphakheji.

2. Zixube kakuhle nento efana nekofu esetyenzisiweyo, umhlaba, okanye inkunkuma yeekati.

3. Beka lo mxube kwingxowa okanye kwisikhongozeli esivalekayo, uze uwulahle emgqomeni wenkunkuma.