Ngaba ugqirha wakho wakubhalela iipilisi zeSapropterin nawe? Makhe sithethe ngale nto ngokweenkcukacha!

Molo! Namhlanje siza kuthetha ngeyeza elingaqhelekanga, kodwa kwabanye abantu libaluleke kakhulu ebomini babo. YiSapropterin leyo. Ukuba wena, umntu omaziyo, okanye umntwana wakho unesifo esingaqhelekanga esibizwa ngokuba yi-'Phenylketonuria', ekwaziwa ngokuba yi-'(PKU)' ngamafutshane, mhlawumbi ukhe weva ngeli yeza. Ngoko ke, makhe sibone ukuba yintoni kanye kanye eli yeza, indlela elinceda ngayo umzimba wethu, kunye nezinto ekufuneka sizilumkele ngakumbi xa silisebenzisa.

Okokuqala, masibone ukuba yintoni iPhenylketonuria (PKU)?

Ngamafutshane, i-'Phenylketonuria' yimeko yemfuza efunyanwa njengelifa. Umntu onesi sifo akanakuyicoca kakuhle aze ayicubungule i-amino acid ebizwa ngokuba yi-'Phenylalanine'. Usenokuba uzibuza ukuba yintoni i-'Phenylalanine'? Ifumaneka ekutyeni okuninzi okuneproteni esikutyayo, umzekelo, ubisi, inyama, intlanzi, amaqanda, kunye namandongomane.

Ngoko ke, kumntu one-'PKU', le 'phenylalanine' (iPhenylalanine) iqala ukuqokelelana emzimbeni. Kufana nokuqokelelwa kwenkunkuma encinci. Oku kuqokelelana akulunganga, ingakumbi kwingqondo. Ukuba oku akulawulwa ebuntwaneni, uphuhliso lwengqondo lunokuphazamiseka, kwaye iimeko ezifana neengxaki zophuhliso lwengqondo, utshintsho lokuziphatha, kunye nesifo sokuwa zinokwenzeka. Yiyo loo nto abantu abane-'PKU' kufuneka balandele ukutya okukhethekileyo okuncinci kwi-'phenylalanine' (iPhenylalanine).

Ngoko ke iSapropterin inceda njani kule nto?

Kulapho ke iyeza elithi `Sapropterin` (Sapropterin)` lingena khona. Alilonyango lwe-`PKU`, kodwa linceda ekunciphiseni izinga le-`Phenylalanine` (Phenylalanine) emzimbeni wezigulane ezithile ze-`PKU` (hayi zonke). Oko kukuthi, eli yeza linceda umzimba ukuba uqhekeze i-`Phenylalanine` (Phenylalanine)` (Phenylalanine) ngcono kancinci. Kodwa kufuneka lisetyenziswe kunye nokutya okukhethekileyo. Ugqirha uya kugqiba ukuba eli yeza lifanelekile kuwe okanye kumntwana wakho, kwaye lingakanani na elifanele linikelwe.

Eli yeza linokuba phantsi kwamagama eempawu ezifana ne-``JAVYGTOR`` okanye ``KUVAN``.

Zeziphi izinto ekufuneka ndizixelele ugqirha ngaphambi kokuba ndiqalise eli yeza?

Ukuba wena okanye umntu osebenzisa eli yeza unenye yezi meko zilandelayo, ngokuqinisekileyo kufuneka uxelele ugqirha wakho ngaphambi kokuba uqale ukusebenzisa eli yeza. Kungcono ungazifihli ezi zinto, kuba zinokuchaphazela impilo yakho.

• Ngaba unayo nayiphi na ingxaki yokutya engaqhelekanga? Umzekelo, i-anorexia okanye i-bulimia.
• Ngaba unomkhuhlane okanye uhlobo oluthile losulelo?
• Ngaba unesifo sezintso?
• Ngaba unesifo sesibindi?
• Ngaba wakha wahlaselwa kukuxhuzula, okufana nokuxhuzula?
• Ngaba une-aleji kwiSapropterin, amanye amayeza, ukutya, iidayi, okanye izithinteli?
• Ukhulelwe? Okanye unethemba lokukhulelwa?
• Ingaba ungumama oncancisayo?

Kubaluleke kakhulu ukuxoxa ngokukhululekileyo nogqirha ngako konke oku.

Kulungile, ndingayisebenzisa njani le yeza?

Oku kukwayinto ekufuneka uyazi kakuhle. Kufuneka ulandele ngqo oko kuthethwa ngugqirha.

• Ezi pilisi mazithathwe ngomlomo xa kutyiwa .
• Sela amayeza owanikwe ngugqirha wakho, kanye njengoko etshilo kwileyibhile kagqirha, ngomlinganiselo omiselweyo, ngexesha elimiselweyo.
• Iipilisi zingaginywa ziphelele ngamanzi, okanye zinganyibilikiswa kwii-ounces ezi-4 ukuya kwezi-8 (malunga ne-120 ukuya kwi-240 milliliters) zamanzi okanye ijusi yeapile.
• Okona kubaluleke kakhulu: Ufanele uyisele kwimizuzu eli-15 emva kokuba inyibilikile. Musa ukuyishiya ixesha elide ngaphezu koko.
• Ukuba ufuna iipilisi zinyibilike ngokukhawuleza, ungazityumza okanye uzixube kakuhle ngecephe ngelixa zinyibilika.
• Ngamanye amaxesha, ungabona iziqwenga ezincinci zidada emanzini ngaphandle kokuba iipilisi zinyibilike ngokupheleleyo. Oku kuqhelekile. Ukuba kukho iziqwenga ezincinci ezisele kwiglasi emva kokusela iyeza, yongeza amanzi amancinci okanye ijusi yeapile, uyixube, uze uyisele nayo. Ngaleyo ndlela, uya kufumana idosi epheleleyo yeyeza.
• Enye indlela kukutyumza iipilisi uze uzixube nokutya okuncinci, okufana ne-applesauce okanye i-pudding.
• Kubaluleke kakhulu ukusela amayeza akho ngexesha elifanayo yonke imihla, ngexesha elifanayo yonke imihla. Musa ukuthatha ngaphezu kokuba ugqirha wakho ekuxelele, okanye uyeke ukuthatha amayeza akho ngaphandle kokumcela.

Ikhethekile ukuba inikwe abantwana abancinci:

Ukuba unika umntwana wakho eli yeza, ngokuqinisekileyo kuya kufuneka uthethe nogqirha wakho wezilwanyana ngalo. Nangona eli yeza linokunikwa iintsana ezineminyaka eyi-1 ubudala kwiimeko ezithile ezikhethekileyo, kukho izinto ekufuneka uzilumkele. Ngoko ke, landela kanye oko kuthethwa ngugqirha.

Ungenza ntoni ukuba uthatha amayeza amaninzi kakhulu?

Ukuba oko kuyenzeka, oko kukuthi, ukuba ucinga ukuba uthathe amayeza amaninzi kunokuba ubucinga, fowunela iziko lolawulo lwetyhefu elikufutshane okanye igumbi likaxakeka esibhedlele ngoko nangoko. Musa ukuchitha ixesha.

Inqaku elibalulekileyo: Eli yeza lelakho kuphela. Musa ukunika omnye umntu iyeza lakho, nokuba uneempawu ezifanayo nezakho.

Kuthekani ukuba ndiphoswa lidosi leyeza?

Ukuba oku kuyenzeka, thatha idosi oyiphosileyo ngokukhawuleza xa ukhumbula. Nangona kunjalo, ukuba sele kuza kufuneka uthathe idosi elandelayo, yitsibe idosi oyiphosileyo uze uthathe idosi elandelayo ngexesha elicwangcisiweyo. Ungaze uthathe iidosi ezimbini ngaxeshanye.

Ngaba kukho amanye amayeza anokusebenza nala mayeza?

Ewe, kusenokwenzeka ukuba zikhona. Kukho amayeza aliqela anokusebenzisana neSapropterin. Umzekelo:

• I-Avanafil `(avanafil)`
• I-Levodopa `(levodopa)`
• I-Methotrexate
• I-Pemetrexed
• I-Pyrimethamine
• I-Sildenafil `(sildenafil)`
• I-Tadalafil `(tadalafil)`
• I-Vardenafil `(vardenafil)`

Olu luhlu aluphelelanga. Ngoko ke, xelela ugqirha wakho ngawo onke amayeza owasebenzisayo (amiselwe ngugqirha okanye athengiswa ngaphandle kwemvume kagqirha), amayeza emifuno, iivithamini, kunye nezongezo zokutya ozisebenzisayo. Kwakhona, xelela ugqirha wakho ukuba uyatshaya, usela utywala, okanye usebenzisa iziyobisi ezingekho mthethweni. Ezinye izinto zinokuchaphazela eli yeza.

Yintoni endifanele ndiyilumkele ngakumbi xa ndisebenzisa eli yeza?

Le yinxalenye ebaluleke kakhulu.

• Ugqirha wakho uza kuhlala ejonga imeko yakho. Ngoko ke, ungaphoswa kukuya kwiikliniki ngemihla emiselweyo kunye nokuhlolwa kwegazi lakho . Ingakumbi, kufuneka ujonge rhoqo amanqanaba akho e-phenylalanine.
• Kungathatha malunga neeveki ezi-4 ukuba eli yeza lisebenze emzimbeni. Ngoko ke ungakhathazeki.
• Ukuba iimpawu zakho aziphucuki, okanye ukuba zibonakala zisiba mandundu, xelela ugqirha wakho.
• Kubaluleke kakhulu: Kuya kufuneka ulandele ukutya okukhethekileyo ngelixa usela eli yeza. Oku kuthetha ukutya ukutya okuncinci kwi-phenylalanine. Ugqirha wakho okanye ingcali yesondlo uza kukuchazela oku ngokweenkcukacha. Ungaze utshintshe ukutya kwakho ngaphandle kwengcebiso kagqirha wakho.

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengazo zonke amayeza, le inokuba nemiphumo emibi emincinci. Eminye iyingozi kakhulu, ngelixa eminye ingabi yingozi kangako.

Ukuba ezi zinto ziyenzeka, kufuneka uxelele ugqirha ngoko nangoko!

• Ukuba yi-allergy reaction: Ithetha ukurhawuzelela kwesikhumba, ukurhawuzelela, amaqhuqhuva, ukudumba kobuso, imilebe, nolwimi.
• Ukuba indle yakho imnyama okanye inegazi, okanye ukuba uhlanza igazi.
• Ubunzima bokuphefumla.
• Ukuba uziva unesiyezi, unentloko ebuhlungu, okanye ngathi uza kuwa.
• Iimpawu ezinje ngokushukuma, ukungazinzi, ukuthetha kakhulu, nokubaleka (ukusebenza ngokugqithisileyo). (Oku kuyinyani ngakumbi kubantwana abancinci)
• Ukuba uneentlungu zesisu eziqatha.

Ezi azinabungozi, kodwa ukuba ziyaqhubeka, xelela ugqirha wakho :

• Ukukhwehlela
• Indle ekhululekileyo (urhudo)
• Intloko ebuhlungu
• Isicaphucaphu, ukuhlanza
• Umqala obuhkungu
• Impumlo egobhozayo, impumlo exineneyo

Olu luhlu alubandakanyi zonke iziphumo ebezingalindelekanga. Ukuba ubona nantoni na engaqhelekanga, buza ugqirha wakho. Ungaxela iziphumo ebezingalindelekanga kwi-FDA (Food and Drug Administration) ku-1-800-FDA-1088.

Ufanele uwagcine njani amayeza akho?

• Yigcine kude nabantwana . Oku kubaluleke kakhulu.
• Gcina kubushushu begumbi (phakathi kwama-20 nama-25 degrees Celsius / ama-68 nama-77 degrees Fahrenheit).
• Gcina iyeza kwisikhongozeli sokuqala elifike naso. Vala ibhotile kakuhle.
• Musa ukusebenzisa amayeza aphelelwe lixesha. Walahle.

Okokugqibela, isikhumbuzo esincinci

Esi sisishwankathelo nje sento esiyithethileyo malunga neyeza elithi `Sapropterin`. Ukuba unemibuzo engaphezulu malunga neli yeza, nceda ubuze ugqirha wakho, ikhemisti, okanye omnye umboneleli wezempilo. Musa ukoyika nantoni na okanye uyigcine kuwe.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ngoko ke, singakukhumbuza kwakhona ngamanqaku ekufuneka uwakhumbule kwinto esithethe ngayo?

• I-Sapropterin liyeza elinceda ukulawula amanqanaba e-phenylalanine kwabanye abantu abane-phenylketonuria (PKU).
• Oku akuyondlela yonyango , kwaye kufanele kusetyenziswe kuphela kunye nokutya okukhethekileyo .
• Ngaphambi kokuba usebenzise amayeza, xelela ugqirha wakho ngazo zonke iimeko zakho zempilo kunye nawo onke amayeza owasebenzisayo.
• Ukuba uxuba iyeza, lisele kungekapheli imizuzu eli-15.
• Kubalulekile ukwenza uvavanyo olufanelekileyo ngexesha elifanelekileyo.
• Ukuba ufumana naziphi na iziphumo ebezingalindelekanga ezingaqhelekanga, xelela ugqirha wakho ngoko nangoko.
• Musa ukutshintsha indlela otya ngayo ngaphandle kwengcebiso kagqirha wakho.

Ukuba wena okanye umntwana wakho ninikwe eli yeza, landela imiyalelo kagqirha kanye njengoko enixelela. Kulapho ke kuphela apho niza kukwazi ukufumana okuninzi kweli yeza, nilawule imeko yenu ye-'PKU' kakuhle, kwaye niphile ubomi obusempilweni. Ndikunqwenelela okuhle kodwa!

I- Sapropterin, i-Phenylketonuria, i-Phenylketonuria, i-PKU, i-Phenylalanine, i-Phenylalanine, amayeza, amayeza, iziphumo ebezingalindelekanga