Isapropterin

Umxholo

• Ngaphambi kokuba uthathe i-sapropterin,
• I-Sapropterin inokubangela iziphumo ebezingalindelekanga. Tshela ugqirha wakho ukuba ngaba ezinye zeempawu zinzima okanye azihambi:
• Ezinye iziphumo ebezingalindelekanga zinokuba nzulu. Ukuba ufumana naziphi na kwezi mpawu biza ugqirha ngokukhawuleza:
• Iimpawu libanda kungaquka oku kulandelayo:

I-Sapropterin isetyenziswa kunye nokutya okuthintelweyo ukulawula amanqanaba egazi e-phenylalanine kubantu abadala nakubantwana kwinyanga eyi-1 ubudala nangaphezulu abane-phenylketonuria (PKU; imeko yokuzalwa apho i-phenylalanine inokwakha egazini kwaye ibangele ukwehla kobukrelekrele kunye nokwehla kwamandla gxila, khumbula, kwaye uququzelele ulwazi). I-Sapropterin iyakusebenza kuphela kubantu abathile abane-PKU, kwaye ekuphela kwendlela yokuxelela ukuba i-sapropterin iyakunceda isigulana esithile kukunika iyeza ixesha elithile kwaye libone ukuba inqanaba le-phenylalanine liyancipha. I-Sapropterin ikudidi lwamayeza abizwa ngokuba zii-cofactors. Isebenza ngokunceda umzimba ukuba wophule i-phenylalanine ukuze ingazokwakha egazini.

I-Sapropterin iza njengecwecwe kunye nanjengomgubo oxutywe nolwelo okanye ukutya okuthambileyo kwaye uthathe ngomlomo. Ihlala ithathwa kanye ngemini ngokutya. Thatha i-sapropterin malunga nexesha elifanayo yonke imihla. Landela izikhokelo kwileyibhile yakho yonyango ngononophelo, kwaye ubuze ugqirha wakho okanye usokhemesti ukuba achaze nayiphi na indawo ongayiqondiyo. Thatha i-sapropterin ngokuchanekileyo njengoko kuyalelwe. Musa ukuthatha ngaphezulu okanye ngaphantsi kwayo okanye uthathe rhoqo kunokuba umiselwe ngugqirha wakho.

Ukuba awukwazi ukuginya amacwecwe, beka inani lamacwecwe e-sapropterin oxelelwe ukuba uthathe ikomityi equlathe ii-ounces ezi-4 ukuya kwezi-8 (1/2 ukuya kwi-1 indebe okanye i-120 ukuya kwi-240 yeemililitha) zamanzi okanye ijusi ye-apile. Gquba umxube okanye utyumle amacwecwe ngecephe ukunyibilikisa iipilisi. Amacwecwe anokunganyibiliki ngokupheleleyo; kusenokubakho iziqwengana ezincinane zeethebhulethi ezidadayo ngaphezulu kolwelo. Xa iipilisi zichithwa ikakhulu, sela wonke umxube. Ukuba iziqwenga zamacwecwe zihlala kule ndebe emva kokuba usele lo mxube, galela amanzi okanye i-apile engaphezulu kule ndebe kwaye uyisele ukuze uqiniseke ukuba uyawaginya onke amayeza. Qiniseka ukuba usele wonke umxube kwisithuba semizuzu eli-15 emva kokuba uyilungisile. Iipilisi zeSapropterin zinokutyunyuzwa kwaye zixutywe nokutya okuthambileyo okufana neapilesauce kunye nepudding.

Ukulungiselela i-sapropterin powder, yongeza iziqulatho zepakethi (okanye) iipowder ezine-4 ukuya kwezi-8 ii-ounces (1/2 ukuya kwi-1 indebe okanye i-120 ukuya kwi-240 yeemililitha) zamanzi okanye ijusi yeapile, okanye ukutya okuncinci okuthambileyo okufana neapilesauce okanye ipudding. Xuba umgubo kulwelo okanye ukutya okuthambileyo kakuhle kakhulu de umgubo unyibilike ngokupheleleyo. Qiniseka ukuba usele okanye utye wonke umxube ukuze ufumane idosi epheleleyo. Yitya okanye usele lo mxube kwisithuba semizuzu engama-30 ulungiselela.

Ukuba ungumzali okanye umnakekeli unika umgubo kumntwana onobunzima obungama-22 eekhilogram (10 kg) okanye ngaphantsi, kuya kufuneka ufumane imiyalelo ethile kugqirha malunga nokuba mangaphi amanzi okanye incindi yeapile oza kuyisebenzisa, kwaye malini umxube oza kuwunika umntwana wakho. Linganisa isixa samanzi okanye isiselo seapile osisebenzisayo ngekomityi yeyeza kwaye usebenzise isirinji yomlomo yokulinganisa ukuze unike idosi umntwana. Yilahle nayiphi na ingxubevange eseleyo emva kokuba kunikwe idosi.

Ugqirha wakho uza kukuqalisa kwi-adose ye-sapropterin kwaye uya kujonga inqanaba lakho le-phenylalanine yegazi rhoqo. Ukuba inqanaba lakho le-phenylalanine alinciphi, ugqirha wakho unokunyusa idosi yakho ye-sapropterin. Ukuba inqanaba lakho le-phenylalanine alinciphi emva kwenyanga e-1 yonyango ngethamo eliphezulu le-sapropterin, wena nogqirha wakho niyakwazi ukuba imeko yenu ayiphenduli kwi-sapropterin. Ugqirha wakho uyakuxelela ukuba uyeke ukuthatha amayeza.

I-Sapropterin inokunceda ukulawula amanqanaba egazi le-phenylalanine, kodwa ayizukuyinyanga i-PKU. Qhubeka nokuthatha i-sapropterin nokuba uziva uphilile. Sukuyeka ukuthatha i-sapropterin ngaphandle kokuthetha nogqirha wakho.

Buza usokhemesti okanye ugqirha wakho ngekopi yolwazi lomenzi wesigulana.

Eli yeza linokumiselwa ezinye izinto; buza ugqirha wakho okanye usokhemesti ngolwazi oluthe kratya.

Ngaphambi kokuba uthathe i-sapropterin,

• xelela ugqirha wakho kunye nosokhemesti ukuba ngaba uxabisa i-sapropterin okanye amanye amayeza.
• xelela ugqirha wakho kunye nosokhemesti ukuba yeyiphi na amayeza kagqirha kunye nalawo angabhaliswanga, iivithamini, izongezo zesondlo, kunye nemveliso yamayeza owathathayo okanye oceba ukuyithatha. Qiniseka ukuba ukhankanya nayiphi na kwezi zinto zilandelayo: levodopa (eSinemet, eStalevo); methotrexate (Otrexup, Rasuvo, Trexall, nabanye); PDE5 inhibitors ezinje nge-sildenafil (Revatio, Viagra), tadalafil (Cialis), kunye nevardenafil (Levitra); Iproguanil (eMalarone), i-pyrimethamine (iDaraprim), kunye ne-trimethoprim (Primsol, eBactrim, Septra). Ugqirha wakho unokufuna ukutshintsha amayeza akho okanye akubeke esweni ngononophelo kwiziphumo ebezingalindelekanga.
• xelela ugqirha wakho ukuba ukhe wanayo ianorexia (ingxaki yokutya apho umntu atya kancinci kwaye / okanye asebenzise kakhulu ukugcina ubunzima bomzimba buthathwa njengesiqhelo kubudala bakhe kunye nokuphakama) okanye nayiphi na imeko kubangela ukuba ungondli kakuhle, okanye isibindi okanye isifo sezintso.
• xelela ugqirha wakho ukuba unefiva okanye ukuba uyagula nanini na ngexesha lonyango lwakho. Umkhuhlane kunye nokugula kunokuchaphazela inqanaba lakho le-phenylalanine, ke ugqirha wakho unokufuna ukulungelelanisa idosi yakho ye-sapropterin.
• xelela ugqirha wakho ukuba ukhulelwe, cwangcisa ukukhulelwa, okanye uncancisa. Ukuba ukhulelwa ngelixa uthatha i-sapropterin, tsalela ugqirha wakho.

Kuya kufuneka uqhubeke nokulandela ukutya okuphantsi kwe-phenylalanine ngelixa uthatha i-sapropterin. Landela ugqirha wakho kunye nezikhokelo zesondlo ngononophelo. Sukutshintsha indlela otya ngayo nangayiphi na indlela ungakhange uthethe nogqirha wakho okanye isondlo.

Ukuba ukhumbula idosi ephosiweyo kamva ngalo mini, thatha idosi ephosiweyo kwakamsinya nje ukuba uyikhumbule. Nangona kunjalo, ukuba awukhumbuli kude kube lusuku olulandelayo, tsiba idosi ephosiweyo kwaye uqhubeke neshedyuli yakho yesiqhelo yedosi. Musa ukuthatha ngaphezulu kwedosi enye ngosuku olunye okanye uthathe idosi ephindwe kabini ukwenza enye ephosiweyo.

I-Sapropterin inokubangela iziphumo ebezingalindelekanga. Tshela ugqirha wakho ukuba ngaba ezinye zeempawu zinzima okanye azihambi:

• urhudo
• isicaphucaphu
• ukugabha
• isisu esibuhlungu
• intloko ebuhlungu
• ukukhwehlela, iintlungu zomqala, okanye iimpawu ezibandayo
• ukugidigida, ukuhambahamba, okanye ukuthetha kakhulu

Ezinye iziphumo ebezingalindelekanga zinokuba nzulu. Ukuba ufumana naziphi na kwezi mpawu biza ugqirha ngokukhawuleza:

• ukuvutha, ukuphefumla kancinci, ukukhohlela, ukugungxula, isicaphucaphu, irhashalala
• iintlungu kwindawo ephezulu yesisu, isicaphucaphu, ukugabha, mnyama, ukuhlala okanye isitulo segazi, ukugabha igazi

I-Sapropterin inokubangela ezinye iziphumo ebezingalindelekanga. Biza ugqirha wakho ukuba unengxaki engaqhelekanga ngelixa uthatha eli yeza.

Ukuba uhlangabezana neziphumo ebezingalindelekanga ezimandundu, wena okanye ugqirha wakho ungathumela ingxelo kwiNkqubo yoKutya kunye nezoLawulo (i-FDA) yeMedWatch yeNkqubo yokuNgxelo ngezeMeko eziGwenxa kwi-Intanethi (http://www.fda.gov/Safety/MedWatch) okanye ngomnxeba ( 1-800-332-1088).

Gcina eli yeza kwisikhongozeli lifikile, livalekile, kwaye alifumaneki ebantwaneni. Yigcine kwindawo epholileyo, eyomileyo, kude nobushushu obuninzi kunye nokufuma (hayi kwigumbi lokuhlambela okanye emotweni). Musa ukuyisusa i-desiccant (ipakethi encinci ebandakanyiwe kunyango ukuze ifunxe ukufuma).

Amayeza angafunekiyo kufuneka achithwe ngeendlela ezizodwa zokuqinisekisa ukuba izilwanyana zasekhaya, abantwana kunye nabanye abantu abanakuzisebenzisa. Nangona kunjalo, akufuneki uligungxule eli yeza kwindlu yangasese. Endaweni yoko, eyona ndlela yokulahla amayeza akho yinkqubo yokubuyisa amayeza. Thetha kumayeza wakho okanye unxibelelane nesebe lakho lenkunkuma / lokurisayikilisha ukuze ufunde malunga neenkqubo zokubuyisa kwindawo ohlala kuyo. Jonga i-FDA's Safe Disposal of Medicines website (http://goo.gl/c4Rm4p) ngolwazi oluthe kratya ukuba awunakho ukufikelela kwinkqubo yokubuyisa.

Kubalulekile ukugcina onke amayeza ngaphandle kwamehlo kunye nokufikelela kwabantwana njengezikhongozeli ezininzi (ezinje ngeeveki zeepilisi zeveki kunye nezo zamehlo, iikhrim, iipatches, kunye ne-inhalers) azinyangeki kwaye abantwana abancinci banokuzivula ngokulula. Ukukhusela abantwana abancinci kwiityhefu, soloko ukhiya iminqwazi yokhuseleko kwaye ubeke amayeza kwindawo ekhuselekileyo-leyo iphakame kwaye iphakame kwaye ingabonakali kubo kwaye ifikelele kuyo. http://www.upandaway.org

Kwimeko yokugqithisa, fowunela umnxeba wolawulo lwetyhefu kwi-1-800-222-1222. Ulwazi lukwafumaneka kwi-Intanethi kwi-https: //www.poisonhelp.org/help. Ukuba ixhoba liye lawa, laxhuzula, linengxaki yokuphefumla, okanye alikwazi ukuvuswa, ngoko nangoko lifowunele iinkonzo zongxamiseko ngo-911.

Iimpawu libanda kungaquka oku kulandelayo:

• intloko ebuhlungu
• isiyezi

Gcina lonke ixesha lokudibana nogqirha wakho kunye nelabhoratri. Ugqirha wakho uya kuyalela iimvavanyo ezithile zebhu ukujonga impendulo yomzimba wakho kwi-sapropterin.

Musa ukuvumela nabani na ukuba athathe amayeza akho. Buza usokhemesti wakho nayiphi na imibuzo onayo malunga nokuzalisa amayeza akho.

Kubalulekile ukuba ugcine uluhlu olubhaliweyo lwawo onke amayeza owasebenzisayo amayeza owasebenzisayo, kunye nezinye iimveliso ezinjengeevithamini, iiminerali, okanye ezinye izongezo zokutya. Kuya kufuneka uze nolu luhlu qho xa undwendwela ugqirha okanye ungeniswe esibhedlele. Kukwabalulekile ulwazi ukuba uphathe nawe xa kukho imeko kaxakeka.

• Kuvan^®