Lumacaftor ne-ivacaftor (indlela yokumathisa ngomlomo)
Imikhiqizo etholakalayo
Orkambi
Mayelana naleli khambi
Ukuhlanganiswa kweLumacaftor ne-ivacaftor kusetshenziswa ukwelapha i-cystic fibrosis (CF) kubantu abaneminyaka engu-1 nangaphezulu. Kusetshenziswa kuphela kulabo abanezi-copies ezimbili ze-F508del mutation ku-CF transmembrane conductance regulator (CFTR) gene yabo. Udokotela wakho uzosebenzisa uvivinyo ukuhlola i-mutation ngaphambi kokuthi uthole le miyeke. Lona umuthi utholakala ngomyalelo wodokotela kuphela. Lo mkhiqizo utholakala ngezindlela zokulinganisa ezilandelayo:
Ngaphambi kokusebenzisa leli khambi
Ekuceleni kokusebenzisa imithi, izingozi zokuthatha imithi kufanele ziqhathaniswe nezinzuzo ezizokwenzeka. Lo ngumqululo owenzayo wena nodokotela wakho. Kulo muthi, okulandelayo kufanele kucabangelwe: Tshela udokotela wakho uma uke waba nanoma iyiphi impendulo engavamile noma ebangelwa uketshezi kulo muthi noma kunoma iyiphi eminye imithi. Tshela futhi ochwepheshe bakho bezempilo uma unezinye izinhlobo zoketshezi, njengokudla, izibhebhe, izigcini, noma izilwane. Ngomkhiqizo ongenalo iresiphi, funda ilebula noma izithako zephakheji ngokucophelela. Izifundo ezifanele azikwenzelwanga ubuhlobo bobudala nemiphumela yokuxuba kwe-lumacaftor ne-ivacaftor kubantwana abaneminyaka engaphansi konyaka. Ukuphepha nokuphumelela akukaqiniswa. Izifundo ezifanele ebuhlotsheni bobudala nemiphumela yokuxuba kwe-lumacaftor ne-ivacaftor azikwenzelwanga kubantu abadala. Ayikho izifundo ezifanele kwabesifazane zokunquma ingozi yengane uma usebenzisa lo muthi ngesikhathi sokuncelisa. Linganisa izinzuzo ezingaba khona nezingozi ezingaba khona ngaphambi kokuthatha lo muthi ngesikhathi sokuncelisa. Nakuba ezinye izimithi kufanele zingasetshenziswa ndawonye nhlobo, kwezinye izimo imithi emibili ehlukene ingasetshenziswa ndawonye ngisho noma kungenzeka ukuhlangana. Kulezi zimo, udokotela wakho angafuna ukushintsha umthamo, noma ezinye izinyathelo zokuphepha kungadingeka. Uma uthatha lo muthi, kubaluleke kakhulu ukuthi ochwepheshe bakho bezempilo bazi uma uthatha noma iyiphi imithi ehlwini elingezansi. Ukuhlangana okulandelayo kukhethwe ngesisekelo sokubaluleka kwabo okungenzeka futhi akuyona yonke into. Ukusebenzisa lo muthi nanoma iyiphi imithi elandelayo akunconywa. Udokotela wakho anganquma ukungakwelaphi ngalo muthi noma ashintshe ezinye izimithi ozithathayo. Ukusebenzisa lo muthi nanoma iyiphi imithi elandelayo ngokuvamile akunconywa, kodwa kungadingeka kwezinye izimo. Uma kokubili imithi ilotshiwe ndawonye, udokotela wakho angashintsha umthamo noma ukuthi uyisebenzisa kangakanani enye noma zombili imithi. Ukusebenzisa lo muthi nanoma iyiphi imithi elandelayo kungabangela ukwanda kengozi yezinye izingozi ezibekiwe, kodwa ukusebenzisa kokubili imithi kungaba ukwelashwa okuhle kuwe. Uma kokubili imithi ilotshiwe ndawonye, udokotela wakho angashintsha umthamo noma ukuthi uyisebenzisa kangakanani enye noma zombili imithi. Ezinye izimithi akufanele zisetshenziswe ngesikhathi noma eduze kwesikhathi sokudla ukudla noma ukudla izinhlobo ezithile zokudla njengoba kungenzeka ukuhlangana. Ukusebenzisa utshwala noma utshaba nezinye izimithi kungabangela ukuhlangana. Ukuhlangana okulandelayo kukhethwe ngesisekelo sokubaluleka kwabo okungenzeka futhi akuyona yonke into. Ukusebenzisa lo muthi nanoma yini elandelayo ngokuvamile akunconywa, kodwa kungagwenyeki kwezinye izimo. Uma kusetshenziswa ndawonye, udokotela wakho angashintsha umthamo noma ukuthi uyisebenzisa kangakanani lo muthi, noma akunikeze imiyalo ekhethekile mayelana nokusetshenziswa kokudla, utshwala, noma utshaba. Ukuba khona kwezinye izinkinga zezokwelapha kungathinta ukusetshenziswa kalo muthi. Qiniseka ukuthi utshela udokotela wakho uma unezinye izinkinga zezokwelapha, ikakhulukazi:
Indlela yokusebenzisa leli khambi
Thatha leli lemi ngokucacile njengoba kunqunyiwe udokotela wakho. Ungathathi okuningi kwalokhu, ungalithathi kaningi, futhi ungalithathi isikhathi eside kunalokho udokotela wakho akuqondile. Ukwenza kanjalo kungandisa amathuba ezingozi ezihambisana naleli lemi. Leli lemi liza ne-insert yolwazi lwabaguli. Funda bese ulandela imiyalo ngokucophelela. Buza udokotela wakho uma unemibuzo. Thatha leli lemi ngokudla okuqukethe amafutha okuhlanganisa ama-avokhado, ibhotela, i-pizza yachizi, amaqanda, amantongomane, ibhotela le-peanut, ubisi lwabesifazane abakhulelwe, ifomula yengane, noma imikhiqizo yobisi oluphelele (isib. ubisi oluphelele, i-chizi, i-yogathi). Uma usebenzisa ama-granules, hlanganisa konke okuqukethwe kwephakheji ngayinye ngethisipuni eli-1 (amamilliliters angu-5 [mL]) yokudla okuthambile okubandayo kuya ekushiseni kwekamelo (isib. izithelo ezihlutshiwe, i-yogathi enencasa, i-pudding, i-applesauce) noma uketshezi (isib. amanzi, ubisi, ubisi lwabesifazane abakhulelwe, ifomula yengane, noma ijusi). Thatha le nhlanganisela ngaphakathi kwehora eli-1 ngemuva kokuxuba. Tshela udokotela wakho uma uyeka ukuthatha leli lemi ngaphezu kweviki eli-1. Udokotela wakho angase adinge ukushintsha umthamo wakho we-Orkambi® noma eminye imithi oyithathayo. Umthamo waleli lemi uzobahlukile kubantu abahlukene. Landela imiyalo yodokotela wakho noma imiyalo esemthethweni. Ulwazi olulandelayo luhlanganisa kuphela imithamo yaleli lemi. Uma umthamo wakho uhlukile, ungawushintshi ngaphandle kokuthi udokotela wakho akuqondile ukwenza kanjalo. Inani lelimi olithathayo lixhomeke emandleni leli lemi. Futhi, inani lemithamo oyithathayo nsuku zonke, isikhathi esivunyelwe phakathi kwemithamo, nobude besikhathi othatha leli lemi kuya ngenkinga yezokwelapha oyisebenzisela leli lemi. Uma uphuthelwa umthamo waleli lemi, uthathe ngokushesha okukhulu. Noma kunjalo, uma sekusondele isikhathi somthamo wakho olandelayo, skip umthamo ophuthelwe bese ubuyela esikhathini sakho sokudla okuvamile. Ungawandisi imithamo. Uma uphuthelwa umthamo futhi kungamahora angu-6 noma ngaphansi kwesikhathi othatha khona imithi yakho ngokuvamile, thatha umthamo ngokushesha okukhulu. Uma kungaphezu kwamahora angu-6, linda kuze kube yilapho usebenzisa imithi bese uskipa umthamo ophuthelwe. Ungathathi imithi eyengeziwe ukuze uthole umthamo ophuthelwe. Gcina imithi kwisitsha esivaliwe ekushiseni kwekamelo, kude nokushisa, ukufuma, nokukhanya okuqondile. Gcina ungayigqabi. Gcina ingafinyeleleki ezinganeni. Ungagcini imithi esele iphelile noma imithi engasadingeki. Buza ochwepheshe bakho bezempilo ukuthi kufanele uyilahla kanjani noma iyiphi imithi ongayisebenzisi.
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