Ungayinyanga njani i-phenylketonuria kunye nendlela yokuphepha iingxaki

Umxholo

• 1. Unyango olusempilweni
• Ungalunika njani ubisi lwebele ngokukhuselekileyo
• 2. Ukusetyenziswa kwezongezelelo zesondlo
• Iingxaki kunokwenzeka phenylketonuria
• Ungakuphepha njani

Unonophelo kunye nonyango lwe-phenylketonuria emntwaneni kufuneka ikhokelwe ngugqirha wabantwana, kodwa olona nyango luphambili kukuphepha ukutya okutyebileyo kwi-phenylalanine, ikakhulu ukutya okunotye kakhulu kwiiproteni, njengenyama, intlanzi, ubisi, itshizi kunye namaqanda. Ke, abazali beentsana ezine-phenylketonuria kufuneka babeke esweni ukutya komntwana wabo, ekhaya nase sikolweni.

Ukongeza, ukuncancisa kuphela kufuneka kugxile kugqirha wabantwana, kuba ubisi lwebele luqukethe i-phenylalanine, nangona incinci kakhulu kunaleyo ifumaneka kwiifomula ezininzi zekhemesti. Ngokufanelekileyo, inani le-phenylalanine lomntwana ukuya kwiinyanga ezi-6 ubudala kufuneka ligcinwe phakathi kwe-20 ukuya kwi-70 mg ye-phenylalanine nge-kg yobunzima bomzimba.

Kubalulekile ukuba unyango lwe-phenylketonuria lulandelwe ngokwemiqathango yezikhokelo zonyango kunye nesondlo sokuthintela iingxaki ezivela, ezihambelana ikakhulu nokuphuhliswa kwenkqubo yemithambo-luvo.

1. Unyango olusempilweni

Unyango lwezempilo yeyona ndlela iphambili yokuphepha ukubakho kwesi sifo, kuba kukutya okunokwenzeka ukulawula amanqanaba e-phenylalanine egazini, ukuthintela iingxaki zesi sifo. Kubalulekile ukuba ukutya kukhokelwe ngumondli ngokweziphumo zovavanyo lomntwana ekufuneka lwenziwe rhoqo ukuvavanya amanqanaba e-phenylalanine egazini.

I-Phenylalanine inokufumaneka kukutya okuninzi, kokubini izilwanyana kunye nemifuno. Ke, ukulawula isifo kunye nokuphepha iingxaki kubalulekile ukuthintela ukutya, okunje:

• Ukutya kwezilwanyana: inyama, ubisi kunye nemveliso yenyama, amaqanda, intlanzi, ukutya kwaselwandle, kunye neemveliso zenyama ezinje ngesausage, isoseji, ibhekoni, ham.
• Ukutya kwemvelaphi yezityalo: ingqolowa, isoya kunye nezinye izinto, ii-chickpeas, iimbotyi, ii-ertyisi, iilentile, amandongomane, amandongomane, ii-walnuts, iiamangile, iihazelnuts, iipistachios, iipine nuts;
• Iiswiti kunye ne-aspartame;
• Iimveliso eziqulathe ukutya okungavumelekanga njengesithakoezifana namaqebengwana, iikeyiki, iayisi khrim nesonka.

Iziqhamo kunye nemifuno zingatyiwa yi-phenylketonurics, kunye neeswekile kunye namafutha. Kuyenzeka ukuba ufumane kwimarike iimveliso ezithile ezizodwa ezenzelwe abaphulaphuli, ezinje ngerayisi, ipasta kunye nabaqhekezi, kwaye kukho iindlela zokupheka ezininzi ezinokusetyenziselwa ukuvelisa ukutya okusezantsi kwi-phenylalanine.

Jonga uluhlu lokutya okutyebileyo kwi-phenylalanine.

Ungalunika njani ubisi lwebele ngokukhuselekileyo

Nangona isincomo sikukhuphela ngaphandle ubisi lwebele kukutya kosana, usebenzisa ubisi lwekhemesti kuphela ngaphandle kwe-phenylalanine, kusenokwenzeka ukuba uncancise umntwana i-phenylketonuric, nangona kunjalo kufanelekile:

• Yenza uvavanyo lwegazi kumntwana veki nganye ukujonga amanqanaba e-phenylalanine egazini;
• Bala inani lobisi lwebele oza kulinika umntwana, ngokwexabiso le-phenylalanine egazini lomntwana nangokukhokelwa ngugqirha;
• Bala inani lobisi lwekhemesti ngaphandle kwe-phenylalanine, ukugqiba ukondla kosana;
• Ngempompo, susa ubungakanani obufanelekileyo bobisi lwebele umama angalunika umntwana;
• Sebenzisa ibhotile okanye indlela yokunxibelelana ukondla umntwana.

Kubalulekile ukukhuphela ngaphandle i-amino acid phenylalanine ekutyeni, ukuze umntwana angabinangxaki ekukhuleni ngokomzimba nangokwengqondo, njengokudodobala kwengqondo. Jonga ukuba ukutya kufanele ukuba njani kwi-phenylketonuria.

2. Ukusetyenziswa kwezongezelelo zesondlo

Njengoko ukutya komntu one-phenylketonuria kuthintelwe kakhulu, kunokwenzeka ukuba akanalo inani leevithamini kunye neeminerali eziyimfuneko ekusebenzeni kakuhle komzimba kunye nophuhliso oluchanekileyo lomntwana. Yiyo loo nto ingcali yezondlo ingacebisa ukusetyenziswa kwezixhasi kunye neefomula zesondlo ukuqinisekisa ukukhula okufanelekileyo kosana kunye nokukhuthaza impilo yalo.

Isongezelelo ekufuneka sisetyenzisiwe siboniswa yingcali yesondlo ngokobudala, ubunzima bomntu kunye namandla okucola kosana, kwaye kufuneka igcinwe kubo bonke ubomi.

Iingxaki kunokwenzeka phenylketonuria

Iingxaki ze-phenylketonuria zivela xa isifo singenziwa kwangoko okanye xa unyango lungalandelwa ngokwesikhokelo sikagqirha, ngokuqokelelwa kwe phenylalanine egazini, enokufikelela kwiindawo ezithile zobuchopho kwaye ikhokelele kuphuhliso lotshintsho olusisigxina, olunje njengo:

• Ukulibaziseka kuphuhliso lwengqondo;
• Ukukhula kwengqondo okuncinci;
• IMicrocephaly;
• Ukusebenza kakhulu;
• Iingxaki zokuziphatha;
• Ukuncipha kwe-IQ;
• Ukunqongophala kwengqondo;
• Ukuxhuzula;
• Iinyikima.

Ngokuhamba kwexesha, ukuba umntwana akaphathwa kakuhle, kunokubakho ubunzima ekuhlaleni nasekuhambeni, ekuziphatheni okungalunganga kunye nasekulibaziseni intetho nasekukhuleni kwengqondo, ukongeza kuxinzelelo, isifo sokuwa kunye ne-ataxia, kukuphulukana nolawulo lokuhamba ngokuzithandela.

Ungakuphepha njani

Ukuthintela iingxaki, kubalulekile ukuba ukuxilongwa kwesi sifo kwenziwe kwiintsuku zokuqala emva kokuzalwa komntwana kuvavanyo lwesithende. Ukuba iziphumo ziqinisekile, kubalulekile ukuba unyango lwenziwe ngokokukhokela kwabantwana.

Ukongeza, kwezi meko kubalulekile ukuba kwenziwe iimviwo rhoqo ukujonga impilo yomntwana kwaye, ngenxa yoko, ukubonisa utshintsho kwindlela yokutya nakwizongezo zokutya.

Iimviwo zokulandelela zihlala zenziwa ngeveki de umntwana abe neminyaka eli-1 ubudala. Abantwana abaphakathi kweminyaka emi-2 nama-6 ubudala baphinda iimviwo rhoqo kwiintsuku ezili-15 kwaye, ukusukela kwiminyaka esi-7 ubudala, uvavanyo lwenziwa kube kanye ngenyanga.