Ingaba umntwana wakho une-PKU (iPhenylketonuria)? Masithethe nge-Sapropterin powder!

Ngamanye amaxesha, abantwana bethu kunye nathi kufuneka siphile neemeko ezithile zempilo ezikhethekileyo, akunjalo? Enye yezi meko ibizwa ngokuba yiPhenylketonuria (ekwaziwa ngokuba yiPKU ngamafutshane). Elinye lamayeza anikwa le meko yiSapropterin. Ngoko ke namhlanje, makhe sifunde ngale mayeza abizwa ngokuba yiSapropterin, njengokuba uthetha nomhlobo.

Yintoni le yeza? (Yintoni iSapropterin?)

Ngamafutshane, iSapropterin liyeza elinceda ukulawula ukuqokelelwa kakhulu kwekhemikhali ebizwa ngokuba yiphenylalanine emzimbeni wabantu abanemeko ebizwa ngokuba yiPhenylketonuria, okanye iPKU. Khumbula, iziqwenga ezincinci zeproteni ekutyeni esikutyayo zibizwa ngokuba zii-amino acids? IPhenylalanine yenye yezo amino acid. Umntu onePKU akanakuyiqhekeza ngokufanelekileyo le phenylalanine, oko kukuthi, ayigaye aze ayisebenzise njengoko kufuneka. Emva koko iqokelelana egazini kwaye inokonakalisa ingqondo kunye nenkqubo yemithambo-luvo ngokuhamba kwexesha.

Eli yeza leSapropterin linceda ekuqhekezeni loo phenylalanine emzimbeni. Emva koko, ukuqokelelana kwayo egazini kuyancipha. Rhoqo, kunye neli yeza, oogqirha bacebisa ukutya okukhethekileyo kwaye ngamanye amaxesha umthambo. Kuba, kule meko, kufuneka unciphise inani lokutya okuqulethe iphenylalanine kangangoko kunokwenzeka.

Oogqirha banokukunika eli yeza nakwezinye iimeko, ngoko ke ukuba unemibuzo, kungcono ubuze ugqirha wakho okanye ingcali yezemithi .

Ngokwesiqhelo ungathenga eli yeza kwiikhemesti ezisebenzisa amagama eebhrendi iJAVYGTOR okanye iKUVAN.

Izinto ekufuneka uzixelele ugqirha wakho ngaphambi kokuba usebenzise eli yeza

Ngaphambi kokuba uqale ukusebenzisa iSapropterin, ngokuqinisekileyo kufuneka uxelele ugqirha wakho ngezi zinto. Kuba kulapho kuphela apho banokugqiba khona ukuba eli yeza lifanelekile kuwe kwaye banike malini. Ukufihla olu lwazi kuya kukubangela iingxaki kuphela.

• Ngaba unayo nayiphi na ingxaki yokutya ? Oko kukuthi, iimeko ezifana ne -anorexia (imeko yengqondo yokunciphisa umzimba ngokungatyi) kunye ne-bulimia (imeko yokutya kakhulu uze uhlanze).
• Ingaba unomkhuhlane ?
• Ingaba unayo nayiphi na intsholongwane ?
• Ngaba unesifo sezintso ?
• Isifo sesibindiikhona i?
• Ngaba unengxaki ekwenza uzive ngathi unesifo sokuxhuzula ( ukuxhuzula ) ? Okanye wakha wanayo ngaphambili?
• Ngaba wakha waba ne-allergy kwiSapropterin, elinye iyeza, ukutya, iidayi zokutya, okanye izithinteli?
• Ukuba ukhulelwe okanye uceba ukukhulelwa (oku kubaluleke kakhulu).
• Ukuba ungumama oncancisayo .

Kubaluleke kakhulu kwimpilo yakho ukuxelela ugqirha ezi zinto ngaphandle kokuzifihla, akunjalo?

Ulisebenzisa njani eli yeza?

Kulungile, ngoku makhe sibone indlela yokusebenzisa eli yeza ngqo. Eli liyeza eliselwayo. Lifanele lithathwe kunye nokutya. Kufuneka ulandele imiyalelo ekwileyibhile kagqirha wakho. Ungaze uthathe ngaphezulu okanye ngaphantsi komlinganiselo ugqirha wakho akuxelele wona.

Le iza njengomgubo. Ufanele uyisele ngokuyinyibilikisa emanzini okanye kwijusi yeapile. Buza ugqirha wakho okanye kwikhemisti ukuba akunike umlinganiselo ochanekileyo wamanzi okanye ijusi yeapile omele uwuthathe. Umgubo kufuneka unyibilike ngokukhawuleza, nokuba uphelele. Ufanele usele iyeza kwimizuzu engama-30 emva kokuba linyibilikile. Musa ukulishiya ixesha elide ngaphezu koko.

Enye into, ungadibanisa inani elincinci lale powder kwi-applesauce (njenge-applesauce) okanye i-pudding. Buza ugqirha wakho ngayo loo nto, ukuba ukhululekile na ukwenza njalo.

Sela amayeza akho ngexesha, ngexesha elifanayo yonke imihla ukuba kunokwenzeka. Musa ukuthatha ngaphezu kokuba ugqirha wakho ekuxelele. Kwakhona, ungayeki ukuthatha amayeza akho ngequbuliso ngaphandle kwengcebiso kagqirha wakho. Ukwenza njalo kunokuyenza imeko yakho ibe mandundu.

Indlela yokunika abantwana abancinci eli yeza

Kwezinye iimeko ezikhethekileyo, eli yeza linokunikwa iintsana ezineminyaka eyi-1 ubudala. Nangona kunjalo, kukho amanyathelo akhethekileyo okhuseleko. Oko kukuthi, kukho izinto ekufuneka uzilumkele kakhulu. Ngoko ke, thetha nogqirha wakho ngale nto kwaye ulandele imiyalelo yakhe ngokuchanekileyo. Ubungakanani beyeza bumiselwa ngokobunzima bomntwana.

Okubaluleke kakhulu: Eli yeza lelakho wedwa. Musa ukulinika omnye umntu, nokuba uneempawu ezifanayo nezakho. Imeko yomntu wonke yahlukile, ngoko ke akulunganga ukunika nabani na eli yeza ngaphandle kokubonisana nogqirha kuqala.

Wenza ntoni ukuba uphoswe yintlanganiso yamayeza?

Nceda, ungoyiki ngamaxesha anjalo. Ukuba uphoswe yidosi, yithathe ngokukhawuleza xa ukhumbula. Nangona kunjalo, ukuba sele kusondele ixesha lomthamo wakho olandelayo (oko kukuthi, kwiiyure ezimbalwa), yeka umthamo ophoswe ngawo uze uthathe umthamo olandelayo ngexesha elicwangcisiweyo. Ungaze uthathe umthamo ophindwe kabini.Ufanele uthathe kuphela umlinganiselo omiselweyo ngosuku. Ukuba unamathandabuzo ngale nto, buza ugqirha wakho okanye ikhemisti.

Ufanele wenze ntoni ukuba uthatha kakhulu? (Ukusebenzisa i-overdose)

Ukuba ucinga ukuba usele amayeza amaninzi kakhulu, okanye ukuba umntwana wakho usele amayeza amaninzi ngengozi, yiya kwigumbi likaxakeka lesibhedlele esikufutshane ngoko nangoko. Okanye utsalele umnxeba kwiziko lokulawula ityhefu. Oku kufanele kuthathwe njengemeko kaxakeka , ungalibazisi.

Amanye amayeza anokusebenzisana nalo mayeza (Intsebenziswano)

Kukho amayeza athile asenokungasebenzi kakuhle okanye abangele ezinye iingxaki xa esetyenziswa ngeSapropterin. Oku sikubiza ngokuba kukusebenzisana. Ngoko ke, xelela ugqirha wakho ngawo nawaphi na amayeza owasebenzisayo.

• I-Avanafil `( Avanafil )`
• I-Levodopa (iyeza lesifo sikaParkinson)
• I-Methotrexate (esetyenziselwa izifo ezifana nomhlaza kunye nesifo samathambo)
• I-Pemetrexed
• I-Pyrimethamine (iyeza elisetyenziselwa ukunyanga i-malaria)
• I-Sildenafil `(Sildenafil)`
• I-Tadalafil `(Tadalafil)`
• I-Vardenafil

Olu luhlu lusenokungabandakanyi onke amayeza. Ngoko ke, xelela ugqirha wakho ngawo onke amayeza owasebenzisayo (kuquka iivithamini, amayeza emifuno, kunye nezongezo zokutya). Kwakhona xelela ugqirha wakho ukuba uyatshaya, usela utywala, okanye usebenzisa iziyobisi ezingekho mthethweni. Ezinye izinto zinokuchaphazela amayeza akho.

Izinto ekufuneka uzilumkele xa usebenzisa amayeza

Ngelixa usela eli yeza, ugqirha wakho uza kujonga imeko yakho ngokusondeleyo. Bona ugqirha wakho rhoqo kwaye wenze uvavanyo lwegazi rhoqo. Olu vavanyo lwegazi lujonga amanqanaba akho e-phenylalanine kwaye lubone ukuba iyeza lisebenza kakuhle na. Khumbula, kungathatha ukuya kuthi ga kwiiveki ezi-4 ukuba umzimba wakho uphendule kweli yeza. Ngoko ke ungoyiki, yiba nomonde nje.

Ukuba iimpawu zakho aziphucuki, okanye ukuba ziya zisiba mandundu, xelela ugqirha wakho ngoko nangoko.

Eyona nto ibalulekileyo kukuba kufuneka ulandele ukutya okukhethekileyo ngelixa usela eli yeza. Kufuneka utye ukutya okune-phenylalanine encinci. Oku kubalulekile kwi-PKU. Ngaphambi kokuba wenze naluphi na utshintsho kwindlela otya ngayo, qiniseka ukuba uthetha nogqirha wakho okanye ingcali yokutya/ingcali yokutya. Banokukunika uluhlu lokutya okulungileyo kuwe kunye neengcebiso malunga nendlela yokulungiselela ukutya kwakho.

Khawucinge nje, uNalini Akka, ngelixa enika intombi yakhe ene-PKU eli yeza, ulawula ukutya kwayo njengoko ugqirha emxelele. Ngaphambi kokuba alungise ukutya, uhlala ekhangela ukutya okune-phenylalanine encinci. Xa esiya evenkileni, ufunda iilebheli ezikwiipakethi zokutya. Olu hlobo lokhathalelo luyimfuneko kule meko.

Zithini iziphumo ebezingalindelekanga?

Njengalo naliphi na iyeza, iSapropterin inokubangela iziphumo ebezingalindelekanga kwabanye abantu. Nangona kunjalo, ayinguye wonke umntu ozifumanayo. Makhe sijonge ukuba ziyintoni na.

Iziphumo ebezingalindelekanga ekufuneka uzixelele ugqirha wakho ngoko nangoko:

Ukuba ufumana naziphi na kwezi mpawu, kufuneka uxelele ugqirha wakho ngokukhawuleza. Ezi zinokuba yingozi.

• Iimpendulo ze-allergy: Ukurhawuzelela kwesikhumba, ukurhawuzelela, ukuba bomvu kwesikhumba, ukudumba kobuso, imilebe, ulwimi okanye umqala (oku kunokwenza kube nzima ukuphefumla).
• Ukungaphumli kakuhle: Ukungaphumli, ukungqunga, ukuthetha kakhulu, kunye nobunzima bokunikel’ ingqalelo.
• Ukudumba komphimbo okanye isisu: Ukulahlekelwa ngumdla wokutya, umphimbo obuhlungu, iintlungu okanye ubunzima bokuginya, ukutsha esifubeni, isicaphucaphu, ukuhlanza, ukukhwehlela okomileyo, indle enegazi okanye engapheliyo.

Iziphumo ebezingalindelekanga ezihlala zingadingi ngcebiso kagqirha, kodwa kufuneka zixelwe kugqirha ukuba ziyaqhubeka:

Ezi zifo zihlala ziphela emva kweentsuku ezimbalwa. Kodwa ukuba ziyaqhubeka, okanye ukuba ziyakukhathaza, xelela ugqirha wakho.

• Ukukhwehlela
• Urhudo
• Intloko ebuhlungu
• Impumlo egobhozayo okanye impumlo exineneyo
• Umqala obuhkungu
• Ukuhlanza

Olu luhlu lusenokungabandakanyi zonke iziphumo ebezingalindelekanga. Ukuba ubona enye into engaqhelekanga, buza ugqirha wakho ngayo.

Uligcina njani iyeza?

Kubaluleke kakhulu ukugcina eli yeza ngokufanelekileyo.

• Yigcine kude nabantwana nezilwanyana zasekhaya.
• Gcina kubushushu begumbi (phakathi kwama-20 nama-25 degrees Celsius, phakathi kwama-68 nama-77 degrees Fahrenheit). Musa ukuyibeka kwindawo eshushu kakhulu okanye ebandayo.
• Gcina eli yeza kwisikhongozeli sokuqala elalifike naso.
• Gcina ibhotile ivaliwe kakuhle ukuze ukuthintela ukufuma ukuba kungaphumi.
• Musa ukusebenzisa amayeza aphelelwe lixesha kwaye uwalahle ngononophelo. Ungacela ikhemesti yakho ukuba ikunike imiyalelo yokuba ungayilahla njani ngokufanelekileyo.

Okokugqibela, izinto ekufuneka uzikhumbule

Kaloku, sithethe kakhulu ngeSapropterin, akunjalo? Ukuphila nesifo esibizwa ngokuba yi-PKU kunokuba ngumceli mngeni, kodwa ngamayeza anje kunye neengcebiso zonyango ezifanelekileyo, wena nomntwana wakho ninokoyisa loo mceli mngeni.

"Khumbula, kubaluleke kakhulu ukusela eli yeza kanye njengoko ugqirha emyalele, ngobungakanani obumiselweyo, nangexesha elimiselweyo. Kwakhona, kubalulekile ukulandela ukutya okukhethekileyo.""Ukuba uzenza zombini ezi zinto ngokufanelekileyo, uya kukwazi ukulawula imeko yakho ye-PKU kakuhle kwaye uphile ubomi obusempilweni."

Ukuba unemibuzo, iinkxalabo, okanye amathandabuzo, musa ukuwagcina kuwe, kodwa zive ukhululekile ukubuza ugqirha wakho. Bakho ukukunceda. Awuwedwa kolu hambo, sonke sikunye nawe!

Ndikunqwenelela impilo entle wena nomntwana wakho!

I- Sapropterin, i-Sapropterin, i-PKU, i-Phenylketonuria, i-Phenylketonuria, i-Phenylalanine, i-phenylalanine, amayeza, impilo yabantwana, izifo zemfuza