Sapropterin (indlela yokumathisa ngomlomo)
Imikhiqizo etholakalayo
Kuvan
Mayelana naleli khambi
ISapropterin isetshenziselwa ukunciphisa amazinga e-phenylalanine egazini labantu abane-phenylketonuria (PKU). Amazinga aphezulu e-phenylalanine (i-amino acid) egazini angabangela ukonakala kokukhulu komkhondo, okuhlanganisa ukuhlupheka kwengqondo okukhulu, ukuhlanza, ukuzamazama, noma ukunciphisa ikhono lokufunda. Lolu dokotela ngokuvamile lunikezwa kanye nokuzila ukudla okukhethekile. Lolu dokotela lutholakala kuphela ngomyalelo wodokotela wakho. Lo mkhiqizo utholakala ngezindlela zokulinganisa ezilandelayo:
Ngaphambi kokusebenzisa leli khambi
Ekucatshangeni kokusebenzisa imithi, izingozi zokuthatha imithi kufanele ziqhathaniswe nezinzuzo ezizoletha. Lo ngumqulu ozothatha wena nodokotela wakho. Kule mithi, okulandelayo kufanele kucabangelwe: Tshela udokotela wakho uma uke waba nanoma yiluphi uhlobo oluqondile noma oluvamile lokuphazamiseka kule mithi noma kunoma iyiphi enye imithi. Tshela futhi ochwepheshe bakho bezempilo uma unezinye izinhlobo zokuphazamiseka, njengokudla, izibhebhe, izigcini, noma izilwane. Ngomkhiqizo ongenalo iresiphi, funda ilebula noma izithako zephakheji ngokucophelela. Izifundo ezifanele azikwenzelwanga ubuhlobo bobudala nemiphumela yesapropterin kubantwana abaneminyaka engaphansi kwenyanga. Ukuphepha nokuphumelela akukaqiniswa. Izifundo ezifanele ebuhlotsheni bobudala nemiphumela yesapropterin azikwenzelwanga kubantu abadala. Ukuphepha nokuphumelela akukaqiniswa. Ayikho izifundo ezifanele kubesifazane zokunquma ingozi yengane uma usebenzisa le mithi ngesikhathi sokuncelisa. Linganisa izinzuzo ezingaba khona nezingozi ezingaba khona ngaphambi kokuthatha le mithi ngesikhathi sokuncelisa. Nakuba ezinye izidakamizwa kufanele zingasetshenziswa ndawonye, kwezinye izimo imithi emibili ehlukene ingasetshenziswa ndawonye ngisho noma kungenzeka ukuhlangana. Kulezi zimo, udokotela wakho angafuna ukushintsha umthamo, noma ezinye izinyathelo zokuphepha zingadingeka. Uma uthatha le mithi, kubalulekile ukuthi ochwepheshe bakho bezempilo bazi uma uthatha noma iyiphi imithi ehlwini elingezansi. Ukuhlangana okulandelayo kukhethwe ngokusekelwe ekubalulekeni kwabo okungenzeka futhi akuyona yonke into. Ukusebenzisa le mithi nanoma iyiphi imithi elandelayo ngokuvamile akunconywa, kodwa kungadingeka kwezinye izimo. Uma kokubili imithi ilotshiwe ndawonye, udokotela wakho angashintsha umthamo noma ukuthi uyayisebenzisa kangakanani enye noma zombili imithi. Ezinye izidakamizwa akufanele zisetshenziswe ngesikhathi sokudla noma ngesikhathi sokudla izinhlobo ezithile zokudla ngoba kungenzeka ukuhlangana. Ukusebenzisa utshwala noma utshako nanoma iyiphi imithi kungabangela ukuhlangana. Xoxa nochwepheshe bakho bezempilo ngokusetshenziswa kwemithi yakho ngokudla, utshwala, noma utshako. Ukuba khona kwezinye izinkinga zezokwelapha kungathinta ukusetshenziswa kwale mithi. Qiniseka ukuthi utshela udokotela wakho uma unezinye izinkinga zezokwelapha, ikakhulukazi:
Indlela yokusebenzisa leli khambi
Thatha leli lemithi njengoba nje udokotela wakho ekuqondisa. Ungayithathi ngaphezulu, ungayithathi kaningi, futhi ungayithathi isikhathi eside kunalokho udokotela wakho akuqondisile. Ukwenza kanjalo kungandisa amathuba ezingozi eceleni. Le mithi kufanele ifike ne-insert yolwazi lwabaguli. Funda futhi ulandele lezi zinkomba ngokucophelela. Buza udokotela wakho uma unemibuzo. Kungcono ukuthatha le mithi ngokudla ngesikhathi esifanayo nsuku zonke. Landela imiyalo yodokotela wakho ngokucophelela mayelana nanoma iyiphi i-diet ekhethekile. Kubalulekile ukuthi ugcine i-diet enenani elinqunyiwe le-phenylalanine. Abahlengikazi abaphezulu kuka-10 kilograms abasebenzisa i-powder yokuxazulula ngomlomo: Phala okuqukethwe kwe-100 mg- noma i-500 mg-phakheji ku-4 kuya ku-8 ounces (1/2 kuya ku-1 ikomidi) yamanzi noma ijusi le-apple. Hlanganisa kahle. Phuza le nhlanganisela phakathi nemizuzu engama-30. Ungayifaka futhi enanini elincane lokudla, njenge-applesauce noma i-pudding ngaphambi kokuthatha. Abahlengikazi abaphezulu kuka-10 kilograms abasebenzisa amathebulethi: Ungagwinya amathebulethi wonke noma uwaxube ku-4 kuya ku-8 ounces (1/2 kuya ku-1 ikomidi) yamanzi noma ijusi le-apple ngaphambi kokuthatha. Kungathatha imizuzu embalwa amathebulethi ukuba ahlale. Ukuze wenze amathebulethi ahlale ngokushesha, ungawahlanganisa noma uwaphucule. Phuza le nhlanganisela phakathi nemizuzu engama-15. Ungaphuca amathebulethi futhi uhlanganise enanini elincane lokudla okulula, njenge-apple sauce noma i-pudding ngaphambi kokuthatha. Umthamo wale mithi uzobahlukile kubantu abahlukene. Landela imiyalo yodokotela wakho noma imiyalo esemthethweni. Ulwazi olulandelayo luhlanganisa kuphela imithamo yale mithi. Uma umthamo wakho uhlukile, ungawushintshi ngaphandle kokuthi udokotela wakho akuqondise ukwenza kanjalo. Inani lemithi oyithatha lixhomeke emandleni emithi. Futhi, inani lemithamo oyithatha nsuku zonke, isikhathi esivunyelwe phakathi kwemithamo, nobude besikhathi oyithatha imithi kuxhomeke enkingeni yezokwelapha oyisebenzisela imithi. Uma uphuthelwa umthamo wale mithi, uthathe ngokushesha okukhulu. Noma kunjalo, uma sekusondele isikhathi somthamo wakho olandelayo, suka umthamo ophuthelwe bese ubuyela esimisweni sakho sokuthola umthamo ojwayelekile. Ungawandisi imithamo. Gcina kude nabantwana. Ungagcini imithi eliphelile noma imithi engasadingeki. Buza ochwepheshe bakho bezempilo ukuthi kufanele ulahle kanjani noma iyiphi imithi ongayisebenzisi. Gcina imithi esitsheni esivalekile emazingeni okushisa kwegumbi, kude nokushisa, ukufuma, nokukhanya okuqondile. Gcina ungaganduki. Gcina ibhodlela livaliwe ngokuqinile. Gcina imithi ebhodleleni lokuqala owanikwe kwi-pharmacy.
Umshwana wokuzihlangula: I-August iyinkundla yolwazi lwezempilo futhi izimpendulo zayo azihlanganisi iseluleko sezokwelapha. Njalo thintana nochwepheshe bezokwelapha onelayisensi eduze nawe ngaphambi kokwenza noma yiluphi ushintsho.