Sapropterin

Zamkati

• Musanatenge sapropterin,
• Sapropterin ikhoza kuyambitsa zovuta. Uzani dokotala wanu ngati zina mwazizindikirozi ndizolimba kapena sizichoka:
• Zotsatira zina zoyipa zingakhale zazikulu. Ngati mukumane ndi izi, itanani dokotala wanu nthawi yomweyo:
• Zizindikiro za bongo zingaphatikizepo izi:

Sapropterin imagwiritsidwa ntchito limodzi ndi zakudya zoletsedwa kuti muchepetse magazi a phenylalanine mwa akulu ndi ana a 1 wazaka zakubadwa kapena kupitilira omwe ali ndi phenylketonuria (PKU; chibadwidwe chomwe phenylalanine imatha kukhala m'magazi ndipo imayambitsa kuchepa kwa luntha komanso kuchepa yang'anani, kumbukirani, ndikukonzekera zambiri). Sapropterin amangogwira ntchito kwa anthu ena omwe ali ndi PKU, ndipo njira yokhayo yodziwira ngati sapropterin ingathandize wodwala wina ndi kupereka mankhwalawa kwakanthawi ndikuwona ngati mulingo wake wa phenylalanine ukutsika. Sapropterin ali mgulu la mankhwala otchedwa cofactors. Zimagwira ntchito pothandiza thupi kuwononga phenylalanine kotero kuti sizingakule m'magazi.

Sapropterin amabwera ngati piritsi komanso ngati ufa wosakanizidwa ndi zakudya zamadzi kapena zofewa ndikumwa. Nthawi zambiri amatengedwa kamodzi patsiku ndi chakudya. Tengani sapropterin mozungulira nthawi yomweyo tsiku lililonse. Tsatirani malangizo omwe mwalandira mosamala, ndikufunsani dokotala kapena wamankhwala kuti afotokoze gawo lililonse lomwe simukumvetsa. Tengani sapropterin monga momwe adauzira. Musamamwe pang'ono kapena kumamwa pafupipafupi kuposa momwe adanenera dokotala.

Ngati simungathe kumeza mapiritsi, ikani chiwerengero cha mapiritsi a sapropterin omwe munauzidwa kuti mutenge chikho chomwe chili ndi ma ouniki 4 mpaka 8 (1/2 mpaka 1 chikho kapena 120 mpaka 240 milliliters) amadzi kapena madzi apulo. Muziganiza osakaniza kapena kuphwanya mapiritsi ndi supuni kupasuka mapiritsi. Mapiritsiwo sangasungunuke kwathunthu; Pakhoza kukhalabe ndi tinthu tating'onoting'ono tomwe timayandama pamwamba pamadzi. Mapiritsiwa akatha kusungunuka, imwani chisakanizo chonse. Ngati zidutswa za mapiritsi zitsalabe mu chikho mutamwa chisakanizocho, tsanulirani madzi ambiri kapena msuzi wa apulo mu chikhocho ndikumwa kuti mutsimikizire kuti mumeza mankhwala onse. Onetsetsani kuti mumamwa chisakanizo chonse pasanathe mphindi 15 mutakonzekera. Mapiritsi a Sapropterin amathanso kuphwanyidwa ndikusakanizidwa ndi zakudya zofewa monga maapulosi ndi pudding.

Kuti mukonze ufa wa sapropterin, onjezerani zomwe zili mu paketi (s) ndi ma ouniki 4 mpaka 8 (1/2 mpaka 1 chikho kapena 120 mpaka 240 milliliters) amadzi kapena madzi apulo, kapena chakudya chochepa chofewa monga maapulosi kapena pudding. Sakanizani ufa mumadzi amadzimadzi kapena ofewa bwino mpaka ufa utasungunuka. Onetsetsani kuti mumamwa kapena kudya chisakanizo chonse kuti mupeze mlingo wathunthu. Idyani kapena imwani chisakanizocho pasanathe mphindi 30 mutakonzekera.

Ngati ndinu kholo kapena wowasamalira akupereka ufa kwa mwana yemwe amalemera makilogalamu 22 kapena kuchepera, muyenera kupeza malangizo achindunji kuchokera kwa adotolo za kuchuluka kwa madzi kapena madzi apulo oti mugwiritse ntchito, komanso kuchuluka kwa zomwe zakonzedwa chisakanizo choti mupatse mwana wanu. Pezani kuchuluka kwa madzi kapena msuzi wa apulo womwe mukugwiritsa ntchito ndi chikho cha mankhwala ndikugwiritsa ntchito sirinji ya pakamwa kuti muyese ndikupatsa mwanayo mlingo. Kutaya chisakanizo chilichonse chomwe chatsala pambuyo poti mlingo waperekedwa.

Dokotala wanu akuyambitsani pa adose ya sapropterin ndipo amayang'ana magazi anu phenylalanine mulingo pafupipafupi. Ngati mulingo wanu wa phenylalanine sukuchepera, dokotala akhoza kukulitsa mlingo wa sapropterin. Ngati mulingo wanu wa phenylalanine sukuchepa pakatha mwezi umodzi wothandizidwa ndi sapropterin, inu ndi dokotala mudzadziwa kuti vuto lanu silikugwirizana ndi sapropterin. Dokotala wanu angakuuzeni kuti musiye kumwa mankhwalawo.

Sapropterin itha kuthandizira kuwongolera magazi a phenylalanine, koma sangachiritse PKU. Pitirizani kumwa sapropterin ngakhale mukumva bwino. Osasiya kumwa sapropterin osalankhula ndi dokotala.

Funsani wamankhwala kapena dokotala wanu kuti mumupatseko zidziwitso za wopanga kwa wodwalayo.

Mankhwalawa amatha kupatsidwa ntchito zina; funsani dokotala wanu kapena wamankhwala kuti mudziwe zambiri.

Musanatenge sapropterin,

• Uzani dokotala ndi wazamankhwala ngati simukugwirizana ndi sapropterin kapena mankhwala aliwonse.
• Uzani dokotala wanu komanso wamankhwala mankhwala ndi mankhwala osapatsirana, mavitamini, zowonjezera zakudya, ndi mankhwala azitsamba omwe mukumwa kapena omwe mukufuna kumwa. Onetsetsani kuti mwatchulapo izi: levodopa (ku Sinemet, ku Stalevo); methotrexate (Otrexup, Rasuvo, Trexall, ena); PDE5 inhibitors monga sildenafil (Revatio, Viagra), tadalafil (Cialis), ndi vardenafil (Levitra); proguanil (ku Malarone), pyrimethamine (Daraprim), ndi trimethoprim (Primsol, ku Bactrim, Septra). Dokotala wanu angafunike kusintha kuchuluka kwa mankhwala anu kapena kukuyang'anirani mosamala zotsatira zake.
• auzeni adotolo ngati mudakhalapo ndi vuto la anorexia (vuto la kudya lomwe munthu amadya pang'ono kapena / kapena amachita masewera olimbitsa thupi kwambiri kuti akhalebe ndi thupi lochepera lomwe limawoneka ngati labwinobwino msinkhu wake ndi kutalika kwake) kapena vuto lina lililonse zimakupangitsani kukhala osadya mokwanira, kapena chiwindi kapena matenda a impso.
• uzani dokotala wanu ngati muli ndi malungo kapena ngati mukudwala nthawi iliyonse mukamalandira chithandizo. Malungo ndi matenda zingakhudze kuchuluka kwanu kwa phenylalanine, chifukwa chake dokotala angafunikire kusintha mlingo wanu wa sapropterin.
• Uzani dokotala wanu ngati muli ndi pakati, konzekerani kutenga pakati, kapena mukuyamwitsa. Mukakhala ndi pakati mukatenga sapropterin, itanani dokotala wanu.

Muyenera kupitiliza kutsatira zakudya zochepa za phenylalanine mukamamwa sapropterin. Tsatirani malangizo a dokotala ndi wazakudya mosamala. Osasintha zakudya zanu mwanjira iliyonse osalankhula ndi dokotala kapena wazakudya.

Ngati mukukumbukira zomwe mwaphonya tsiku lomwelo tsiku lomwelo, tengani mlingo womwe mwaphonya mukangokumbukira. Komabe, ngati simukumbukira mpaka tsiku lotsatira, dumpha mlingo womwe umasowa ndikupitiliza dongosolo lanu lokhazikika. Musatenge mlingo woposa umodzi tsiku limodzi kapena kumwa kawiri kuti mupange omwe mwaphonya.

Sapropterin ikhoza kuyambitsa zovuta. Uzani dokotala wanu ngati zina mwazizindikirozi ndizolimba kapena sizichoka:

• kutsegula m'mimba
• nseru
• kusanza
• kupweteka m'mimba
• mutu
• chifuwa, kupweteka pakhosi, kapena kuzizira
• kunyinyirika, kuyendayenda, kapena kulankhula kwambiri

Zotsatira zina zoyipa zingakhale zazikulu. Ngati mukumane ndi izi, itanani dokotala wanu nthawi yomweyo:

• kupuma, kupuma movutikira, kutsokomola, kuthamanga, kunyansidwa, zidzolo
• kupweteka kumtunda kwakumimba, nseru, kusanza, wakuda, kudikira kapena chopondapo chamagazi, kusanza magazi

Sapropterin ikhoza kuyambitsa zovuta zina. Itanani dokotala wanu ngati muli ndi zovuta zina mukamamwa mankhwalawa.

Mukakumana ndi zovuta zina, inu kapena adokotala mutha kutumiza lipoti ku pulogalamu ya Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting pa intaneti (http://www.fda.gov/Safety/MedWatch) kapena pafoni ( 1-800-332-1088).

Sungani mankhwala awa mu chidebe chomwe chidabwera, chatsekedwa mwamphamvu, komanso chosafikira ana. Sungani pamalo ozizira, owuma, kutali ndi kutentha ndi kutentha kwambiri (osati kubafa kapena galimoto). Musachotse desiccant (paketi yaying'ono yophatikizidwa ndi mankhwala oti mutenge chinyezi).

Mankhwala osafunikira ayenera kutayidwa munjira zapadera zowonetsetsa kuti ziweto, ana, ndi anthu ena sangamwe. Komabe, simuyenera kuthira mankhwalawa mchimbudzi. M'malo mwake, njira yabwino yoperekera mankhwala anu ndikadongosolo lobwezera mankhwala. Lankhulani ndi wamankhwala wanu kapena lemberani dipatimenti yakunyumba / yobwezeretsanso kwanuko kuti muphunzire zamapulogalamu obwezeretsanso mdera lanu. Onani tsamba la FDA's Disposal of Medicines webusayiti (http://goo.gl/c4Rm4p) kuti mumve zambiri ngati mulibe pulogalamu yobwezera.

Ndikofunika kuti mankhwala onse asamawonekere komanso kuti ana asamafikire ngati muli zidebe zambiri (monga mapiritsi a mlungu ndi mlungu ndi omwe amatsikira m'maso, mafuta, zigamba, ndi opumira) sizolimbana ndi ana ndipo ana aang'ono amatha kuzitsegula mosavuta. Pofuna kuteteza ana ang'ono kuti asatenge poyizoni, nthawi zonse tsekani zipewa zachitetezo ndikuyika mankhwalawo pamalo otetezeka - omwe ali pamwamba ndi kutali komanso osawonekera ndi kufikira. http://www.upandaway.org

Mukadwala mopitirira muyeso, itanani foni yothandizira poyizoni pa 1-800-222-1222. Zambiri zimapezekanso pa intaneti pa https://www.poisonhelp.org/help. Ngati wozunzidwayo wagwa, watenga khunyu, akuvutika kupuma, kapena sangathe kudzutsidwa, nthawi yomweyo itanani anthu azadzidzidzi ku 911.

Zizindikiro za bongo zingaphatikizepo izi:

• mutu
• chizungulire

Sungani maimidwe onse ndi dokotala wanu ndi labotale. Dokotala wanu amalamula mayeso ena a labu kuti muwone kuyankha kwa thupi lanu ku sapropterin.

Musalole kuti aliyense azimwa mankhwala anu. Funsani wamankhwala wanu mafunso aliwonse omwe muli nawo okhudzana ndi kudzaza mankhwala anu.

Ndikofunika kuti musunge mndandanda wamankhwala onse omwe mumalandira komanso osalembetsa (owerengera) omwe mukumwa, komanso zinthu zilizonse monga mavitamini, michere, kapena zakudya zina. Muyenera kubwera nawo pamndandandawu nthawi iliyonse mukapita kukaonana ndi dokotala kapena ngati mwalandiridwa kuchipatala. Ndikofunikanso kuthana nanu pakagwa mwadzidzidzi.

• Kuvan^®