I-Sapropterin

-Delile

• Ngaphambi kokuthatha i-sapropterin,
• I-Sapropterin ingadala imiphumela emibi. Tshela udokotela wakho uma kukhona kulezi zimpawu ezinzima noma ezingapheli:
• Eminye imiphumela emibi ingaba mibi. Uma uhlangabezana nenye yalezi zimpawu shayela udokotela wakho ngokushesha:
• Izimpawu zokudlula ngokweqile zingafaka okulandelayo:

I-Sapropterin isetshenziswa kanye nokudla okunomkhawulo ukulawula amazinga e-phenylalanine egazi kubantu abadala nasezinyangeni ezineminyaka engu-1 nangaphezulu ezine-phenylketonuria (PKU; isimo esizalwa naso lapho i-phenylalanine ingakha egazini futhi ibangele ukwehla kobuhlakani kanye nokwehla kwekhono gxila, khumbula, futhi uhlele imininingwane). I-Sapropterin izosebenza kuphela kubantu abathile abane-PKU, futhi ukuphela kwendlela yokutshela ukuthi ngabe i-sapropterin izosiza yini isiguli esithile ukunikeza umuthi isikhashana bese ibona ukuthi izinga layo le-phenylalanine liyancipha yini. ISapropterin isekilasini lemithi ebizwa ngama-cofactors. Isebenza ngokusiza umzimba ukuthi wephule i-phenylalanine ngakho-ke ngeke yakhe egazini.

I-Sapropterin iza njengethebhulethi nanjengempuphu ezohlanganiswa nokudla okumanzi noma okuthambile bese ikuthatha ngomlomo. Imvamisa kuthathwa kanye ngosuku ngokudla. Thatha i-sapropterin ngasikhathi sinye nsuku zonke. Landela izinkomba ezikulebuli kadokotela wakho ngokucophelela, bese ubuza udokotela noma usokhemisi wakho ukuthi akuchaze nganoma iyiphi ingxenye ongayiqondi. Thatha i-sapropterin njengoba iqondisiwe. Ungayithathi kancane noma uyithathe kaningi kunokuyalelwa ngudokotela wakho.

Uma ungeke ukwazi ukuwagwinya amaphilisi, beka inani lamathebulethi e-sapropterin otshelwe ukuthi uwathathe enkomishini equkethe ama-ounces angu-4 kuya kwayi-8 (1/2 kuya ku-1 inkomishi noma amamililitha ayi-120 kuya ku-240) wamanzi noma ijusi le-apula. Hlanganisa ingxube noma uqede amaphilisi ngesipuni ukuqeda amaphilisi. Amaphilisi kungenzeka angancibiliki ngokuphelele; kusengaba khona izingcezu ezincane zethebulethi ezintantayo phezulu kuketshezi. Lapho amaphilisi ehlakazwa kakhulu, phuza yonke ingxube. Uma izingcezu zamaphilisi zihlala endebeni ngemuva kokuphuza ingxube, uthele amanzi amaningi noma ijusi le-apula enkomishini bese uyiphuza ukuze uqiniseke ukuthi uyayigwinya yonke imithi. Qiniseka ukuthi uphuza yonke ingxube phakathi kwemizuzu engu-15 ngemuva kokuyilungisa. Amaphilisi eSapropterin nawo angachotshozwa ahlanganiswe nokudla okuthambile njenge-appleauce kanye nepudding.

Ukulungisa i-sapropterin powder, engeza okuqukethwe iphakethe (lama) lempuphu ngama-ounces ayi-4 kuya kwayi-8 (1/2 kuya ku-1 inkomishi noma ama-milliliters ayi-120 kuya ku-240) wamanzi noma ijusi le-apula, noma inani elincane lokudla okuthambile njenge-appleauce noma uphudingi. Hlanganisa impuphu ekudleni okungamanzi noma okuthambile kahle kakhulu kuze kube yilapho impuphu iqothuka ngokuphelele. Qiniseka ukuthi uphuza noma udla yonke ingxube ukuze uthole umthamo ophelele. Yidla noma uphuze le ngxube kungakapheli imizuzu engama-30 ulungiselele.

Uma ungumzali noma umnakekeli unikeza impuphu enganeni enesisindo esingamakhilogremu ayi-10 noma ngaphansi, uzodinga ukuthola imiyalo ethile kudokotela mayelana nokuthi ungakanani amanzi noma ijusi le-apula ongalisebenzisa, nokuthi malini okulungiselelwe ingxube ongayinikeza ingane yakho. Linganisa inani lamanzi noma lejusi le-apula olisebenzisayo ngenkomishi yomuthi bese usebenzisa isipontsho somlomo sokulinganisa ukuze unikeze ingane umthamo. Phonsa noma iyiphi ingxube esele ngemuva kokunikezwa umthamo.

Udokotela wakho uzokuqala nge-adose ye-sapropterin futhi uzohlola izinga legazi lakho le-phenylalanine njalo. Uma izinga lakho le-phenylalanine lingehli, udokotela wakho angakhuphula umthamo wakho we-sapropterin. Uma izinga lakho le-phenylalanine linganciphi ngemuva kwenyanga engu-1 yokwelashwa ngomthamo omkhulu we-sapropterin, wena nodokotela wakho niyokwazi ukuthi isimo sakho asiphenduli ku-sapropterin. Udokotela wakho uzokutshela ukuthi uyeke ukuthatha imithi.

ISapropterin ingasiza ukulawula amazinga egazi e-phenylalanine, kepha ngeke ayelaphe i-PKU. Qhubeka nokuthatha i-sapropterin noma ngabe uzizwa uphilile. Ungayeki ukuthatha i-sapropterin ngaphandle kokukhuluma nodokotela wakho.

Buza usokhemisi noma udokotela wakho ikhophi lemininingwane yomkhiqizi yesiguli.

Lo muthi ungabekelwa okunye ukusetshenziswa; buza udokotela wakho noma usokhemisi ukuthola eminye imininingwane.

Ngaphambi kokuthatha i-sapropterin,

• Tshela udokotela wakho kanye nosokhemisi uma unegciwane ku-sapropterin noma eminye imithi.
• Tshela udokotela wakho kanye nosokhemisi ukuthi imiphi imishanguzo kadokotela neyokungabhalisi, amavithamini, izithako zokudla okunempilo, nemikhiqizo yamakhambi oyithathayo noma ohlela ukuyithatha. Qiniseka ukuthi usho noma ikuphi kokulandelayo: levodopa (eSinemet, eStalevo); methotrexate (Otrexup, Rasuvo, Trexall, abanye); Ama-PDE5 inhibitors afana ne-sildenafil (Revatio, Viagra), tadalafil (Cialis), ne-vardenafil (Levitra); proguanil (eMalarone), pyrimethamine (Daraprim), kanye ne-trimethoprim (Primsol, eBactrim, Septra). Udokotela wakho angadinga ukushintsha imithamo yemithi yakho noma akuqaphele ngokucophelela ngemiphumela emibi.
• Tshela udokotela wakho uma uke waba ne-anorexia (inkinga yokudla lapho umuntu edla okuncane kakhulu futhi / noma ezivocavoca kakhulu ukugcina ngisho isisindo somzimba esincane esithathwa njengesijwayelekile ngeminyaka yakhe nokuphakama) noma esinye isimo kubangela ukuthi ungondli kahle, noma isifo sesibindi noma sezinso.
• utshele udokotela wakho uma unemfiva noma uma ugula noma kunini ngesikhathi sokwelashwa kwakho. Imfiva nokugula kungathinta izinga lakho le-phenylalanine, ngakho-ke udokotela wakho angadinga ukulungisa umthamo wakho we-sapropterin.
• utshele udokotela wakho uma ukhulelwe, hlela ukukhulelwa, noma uncelisa. Uma ukhulelwa ngenkathi uthatha i-sapropterin, shayela udokotela wakho.

Kufanele uqhubeke nokulandela ukudla okuphansi kwe-phenylalanine ngenkathi uthatha i-sapropterin. Landela izinkombandlela zikadokotela wakho nezomsoco ngokucophelela. Ungakushintshi ukudla kwakho nganoma iyiphi indlela ngaphandle kokukhuluma nodokotela wakho noma isazi sokudla okunempilo.

Uma ukhumbula umthamo ongabanjwanga kamuva ngalo lolo suku, thatha umthamo owulahlekile ngokushesha uma uwukhumbula. Kodwa-ke, uma ungakhumbuli kuze kube usuku olulandelayo, weqa umthamo ophuthelwe bese uqhubeka nesimiso sakho sokulinganisa okujwayelekile. Ungathathi umthamo ongaphezu kowodwa ngosuku olulodwa noma uthathe umthamo ophindwe kabili ukwenzela owulahlekile.

I-Sapropterin ingadala imiphumela emibi. Tshela udokotela wakho uma kukhona kulezi zimpawu ezinzima noma ezingapheli:

• isifo sohudo
• isicanucanu
• ukuhlanza
• ubuhlungu besisu
• ikhanda
• ukukhwehlela, ubuhlungu bomphimbo, noma izimpawu ezibandayo
• ukuzulazula, ukuhambahamba, noma ukukhuluma kakhulu

Eminye imiphumela emibi ingaba mibi. Uma uhlangabezana nenye yalezi zimpawu shayela udokotela wakho ngokushesha:

• ukuphefumula, ukuphefumula, ukukhwehlela, ukuqhuma, isicanucanu, ukuqubuka
• ubuhlungu endaweni engaphezulu yesisu, isicanucanu, ukuhlanza, okumnyama, okulindile noma okunegazi, ukuhlanza igazi

I-Sapropterin ingadala eminye imiphumela emibi. Shayela udokotela wakho uma unezinkinga ezingavamile ngenkathi uthatha lo muthi.

Uma uthola imiphumela emibi kakhulu, wena noma udokotela wakho ungathumela umbiko kuhlelo lwe-Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting online (http://www.fda.gov/Safety/MedWatch) noma ngocingo ( 1-800-332-1088).

Gcina le mithi esitsheni efike ngayo, ivalwe ngokuqinile, futhi lapho izingane zingafinyeleleki khona. Yigcine endaweni epholile, eyomile, kude nokushisa okweqile nomswakama (hhayi endlini yangasese noma emotweni). Ungayikhiphi i-desiccant (iphakethe elincane elifakiwe nemithi yokwamukela umswakama).

Imithi engadingeki kufanele ilahlwe ngezindlela ezikhethekile zokuqinisekisa ukuthi izilwane ezifuywayo, izingane nabanye abantu abakwazi ukuzidla. Kodwa-ke, akufanele ukhiphe le mithi ethoyilethi. Esikhundleni salokho, indlela engcono kakhulu yokulahla imithi yakho ngohlelo lokubuyisa umuthi. Khuluma nosokhemisi wakho noma uthinte umnyango wangakini wokulahla udoti / wokusebenzisa kabusha ukuze ufunde ngezinhlelo zokubuyisa emphakathini wakho. Bheka iwebhusayithi yakwa-FDA's Safe Disposal of Medicines (http://goo.gl/c4Rm4p) ukuthola eminye imininingwane uma ungakwazi ukufinyelela kuhlelo lokubuyisa.

Kubalulekile ukugcina yonke imishanguzo ingabonakali futhi ifinyeleleke ezinganeni njengoba iziqukathi eziningi (njengezingqondo zamaphilisi zamasonto onke kanye nalawo amaconsi wamehlo, okhilimu, amabala, kanye nama-inhalers) azimeleki ezinganeni futhi izingane ezincane zingazivula kalula. Ukuvikela izingane ezisencane ekufakeni ubuthi, hlala uvala amakepisi okuphepha bese ubeka umuthi endaweni ephephile - eyodwa ephezulu futhi engekho futhi engabonakali kubo futhi efinyelela kuyo. http://www.upandaway.org

Uma kwenzeka ukweqisa ngokweqile, shayela ucingo losizo lokulawula ubuthi ku-1-800-222-1222. Imininingwane iyatholakala online ku-https: //www.poisonhelp.org/help. Uma isisulu siwile, saba nokuquleka, sinenkinga yokuphefumula, noma singakwazi ukuvuswa, shayela ngokushesha abezimo eziphuthumayo ku-911.

Izimpawu zokudlula ngokweqile zingafaka okulandelayo:

• ikhanda
• isiyezi

Gcina wonke ama-aphoyintimenti nodokotela wakho kanye nelabhorethri. Udokotela wakho uzo-oda izivivinyo ezithile zelebhu ukuze ahlole impendulo yomzimba wakho ku-sapropterin.

Ungavumeli omunye umuntu ukuthi athathe imithi yakho. Buza usokhemisi wakho noma imiphi imibuzo onayo mayelana nokugcwalisa umuthi wakho.

Kubalulekile ukuthi ugcine uhlu olubhaliwe lwayo yonke imishanguzo oyisebenzisayo kadokotela kanye nokungabhaliswanga (ngaphezulu kokuthenga), kanye nanoma yimiphi imikhiqizo efana namavithamini, amaminerali, noma ezinye izithako zokudla. Kufanele uze nalolu hlu njalo uma uvakashela udokotela noma uma ungeniswa esibhedlela. Kubaluleke kakhulu futhi nemininingwane ebalulekile ukuthi uphathe uma kwenzeka kunezimo eziphuthumayo.

• UKuvan^®