Ungayelapha kanjani i-phenylketonuria nokuthi ungakugwema kanjani ubunzima

-Delile

• 1. Ukwelashwa ngokudla okunempilo
• Ulunikeza kanjani ubisi lwebele ngokuphepha
• 2. Ukusetshenziswa kwezithako zokudla okunomsoco
• Izinkinga ezingenzeka ze-phenylketonuria
• Ungakugwema kanjani

Ukunakekelwa nokwelashwa kwe-phenylketonuria enganeni kufanele kuqondiswe ngudokotela wezingane, kepha ukunakekelwa okuyinhloko ukugwema ukudla okunothe nge-phenylalanine, okuyikudla ikakhulu okunothile ngamaprotheni, njengenyama, inhlanzi, ubisi, ushizi namaqanda. Ngakho-ke, abazali bezingane ezine-phenylketonuria kufanele banake ukudla kwengane yabo, ekhaya nasesikoleni.

Ngaphezu kwalokho, ukuncelisa ibele okukhethekile kufanele futhi kuqondiswe kahle ngudokotela wezingane, ngoba ubisi lwebele luqukethe i-phenylalanine, yize incane kakhulu kunaleyo etholakala kumafomula amaningi ekhemisi. Okufanelekile, inani le-phenylalanine yengane efinyelela ezinyangeni eziyisithupha ubudala kufanele ligcinwe phakathi kuka-20 kuye ku-70 mg we-phenylalanine nge-kg yesisindo somzimba ngamunye.

Kubalulekile ukuthi ukwelashwa kwe-phenylketonuria kulandelwe ngokuya ngemikhombandlela kadokotela wezingane kanye nochwepheshe wezokudla ukuvimbela izinkinga ezivela, ezihlobene kakhulu nokuthuthuka kwesistimu yezinzwa.

1. Ukwelashwa ngokudla okunempilo

Ukwelashwa ngokudla okunempilo kuyindlela eyinhloko yokugwema izinkinga zesifo, ngoba kungukudla lapho kungenzeka khona ukulawula amazinga e-phenylalanine egazini, ngaleyo ndlela kugwenywe izinkinga zesifo. Kubalulekile ukuthi indlela yokudla iqondiswe ngusondli ngokwemiphumela yokuhlolwa kwengane okumele yenziwe njalo ukuhlola amazinga e-phenylalanine egazini.

I-Phenylalanine itholakala ekudleni okuningana, kokubili isilwane nemifino. Ngakho-ke, ukulawula lesi sifo nokugwema izinkinga kubalulekile ukugwema okunye ukudla, okufana nalokhu:

• Ukudla kwezilwane: inyama, ubisi nenyama imikhiqizo, amaqanda, inhlanzi, ukudla kwasolwandle, nemikhiqizo yenyama efana nesoseji, isoseji, ubhekeni, ham.
• Ukudla komsuka wesitshalo: ukolweni, isoya nokunye okuvela kukho, amantongomane, ubhontshisi, uphizi, udali, amantongomane, amakinati, ama-walnuts, ama-alimondi, ama-hazelnut, ama-pistachio, amantongomane ephayini;
• Ama-sweeteners ane-aspartame;
• Imikhiqizo equkethe ukudla okungavunyelwe njengesithakonjengamakhekhe, amakhukhi, u-ayisikhilimu nesinkwa.

Izithelo nemifino kungadliwa yi-phenylketonurics, kanye noshukela namafutha. Kungenzeka futhi ukuthi uthole emakethe imikhiqizo eminingana ekhethekile eyenzelwe lezi zilaleli, njengelayisi, i-pasta kanye nama-crackers, futhi kunezindlela zokupheka eziningi ezingasetshenziswa ukukhiqiza ukudla okuphansi kwe-phenylalanine.

Bheka uhlu lokudla okunothe ku-phenylalanine.

Ulunikeza kanjani ubisi lwebele ngokuphepha

Yize izincomo ukukhipha ubisi lwebele ekudleni kwengane, kusetshenziswa ubisi lwekhemisi kuphela ngaphandle kwe-phenylalanine, kusenokwenzeka ukuncelisa ingane i-phenylketonuric, kepha kulokhu kuyadingeka:

• Yenza ukuhlolwa kwegazi enganeni njalo ngesonto ukuze uhlole amazinga e-phenylalanine egazini;
• Bala inani lobisi lwebele oluzonikezwa ingane, ngokuya ngamanani we-phenylalanine egazini lengane nangokuya ngokuqondiswa udokotela wezingane;
• Bala inani lobisi lwekhemisi ngaphandle kwe-phenylalanine, ukuqedela ukondla kwengane;
• Ngompompi, susa inani elifanele lobisi lwebele umama angalunika ingane;
• Sebenzisa inqubo yebhodlela noma yokuxhuma kabusha ukondla ingane.

Kubalulekile ukukhipha i-amino acid phenylalanine ekudleni, ukuze ingane ingabi nezinkinga ekukhuleni ngokomzimba nangokwengqondo, njengokuncipha kwengqondo. Bona ukuthi ukudla kufanele kube njani ku-phenylketonuria.

2. Ukusetshenziswa kwezithako zokudla okunomsoco

Njengoba ukudla komuntu one-phenylketonuria kuvinjelwe kakhulu, kungenzeka ukuthi akanalo inani lamavithamini namaminerali adingekayo ekusebenzeni kahle komzimba nasekuthuthukiseni okulungile kwengane. Ngakho-ke, isazi sokudla okunempilo singancoma ukusetshenziswa kwama-supplements kanye namafomula wokudla okunempilo ukuze kuqinisekiswe ukukhula okufanele kwengane nokukhuthaza impilo yayo.

Isengezo esizosetshenziswa sikhonjiswa ngongoti bokudla ngokweminyaka, isisindo somuntu kanye namandla wokugaya enganeni, futhi kufanele inakekelwe impilo yonke.

Izinkinga ezingenzeka ze-phenylketonuria

Izinkinga ze-phenylketonuria zivela lapho ukuxilongwa kungenziwanga kusenesikhathi noma lapho ukwelashwa kungalandelwa ngokwemikhombandlela yodokotela wezingane, ngokuqongelelwa kwe-phenylalanine egazini, engafinyelela ezindaweni ezithile zobuchopho futhi iholele ekwakhiweni kwezinguquko ezihlala njalo, ezinje njengo:

• Ukubambezeleka ekuthuthukisweni kwengqondo;
• Ukukhula kwengqondo okuncane;
• I-Microcephaly;
• Ukungasebenzi kahle;
• Izinkinga zokuziphatha;
• Ukwehla kwe-IQ;
• Ukuntuleka okukhulu kwengqondo;
• Ukudlikizela;
• Ukuthuthumela.

Ngokuhamba kwesikhathi, uma ingane ingelashwa ngendlela efanele, kungaba nobunzima ekuhlaleni nasekuhambeni, ekuphazamisekeni kokuziphatha nasekukhulumeni okubambezelekayo kanye nokukhula kwengqondo, ngaphezu kokucindezeleka, isithuthwane kanye ne-ataxia, okuwukulahlekelwa amandla okulawula.

Ungakugwema kanjani

Ukugwema izinkinga, kubalulekile ukuthi ukuxilongwa kwalesi sifo kwenziwe ezinsukwini zokuqala ngemuva kokuzalwa kwengane ngokuhlolwa kwesithende. Uma umphumela uba muhle, kubalulekile ukuthi ukwelashwa kwenziwe ngokuyalelwa ngudokotela wezingane.

Ngaphezu kwalokho, kulezi zimo kubalulekile ukuthi kwenziwe ukuhlolwa okujwayelekile ukubheka impilo ejwayelekile yengane futhi, ngaleyo ndlela, kukhombise ushintsho ekudleni nakwizithasiselo zokudla.

Izivivinyo zokulandelela zivame ukwenziwa masonto onke kuze kube yilapho ingane iba nonyaka owodwa ubudala. Izingane eziphakathi kweminyaka emibili nengu-6 ubudala ziyasiphinda isivivinyo njalo ezinsukwini eziyi-15 futhi, kusukela eminyakeni engu-7 ubudala, ukuhlolwa kwenziwa kanye ngenyanga.