I-CF yemfuza: Luchaphazela njani unyango lwakho

Umxholo

• Zibangelwa njani utshintsho lwemfuza?
• Zeziphi iintlobo zotshintsho ezinokubangela iCF?
• Ngaba utshintsho kwimfuza luchaphazela njani ukhetho lonyango?
• Ndiyazi njani ukuba unyango lulungile kumntwana wam?
• Ukuthatha

Ukuba umntwana wakho une-cystic fibrosis (CF), ke iigeni zabo zidlala indima kwimeko yabo. Imfuza ethile ebangela ukuba i-CF yayo ichaphazele iintlobo zamayeza ezinokubasebenzela. Kungenxa yoko le nto kubaluleke kakhulu ukuqonda indima edlalwa kwi-CF xa usenza izigqibo malunga nokhathalelo lwempilo lomntwana wakho.

Zibangelwa njani utshintsho lwemfuza?

I-CF ibangelwa lutshintsho kumgaqo wokuziphatha we-cystic fibrosis transmembrane (ICFTRUhlobo). Olu hlobo lunoxanduva lokuvelisa iiproteni zeCFTR. Xa ezi proteni zisebenza ngokufanelekileyo, zinceda ukulawula ukuhamba kwamanzi kunye netyuwa zingena ziphuma kwiiseli.

NgokweCystic Fibrosis Foundation (CFF), izazinzulu zichonge ngaphezulu kwe-1,700 yeentlobo ezahlukeneyo zotshintsho kuhlobo olunokubangela iCF. Ukuphuhlisa i-CF, umntwana wakho kufuneka azuze njengelifa iikopi ezimbini eziguqulweyo ze ICFTR Uhlobo-olunye kumzali ngamnye ophilayo.

Kuxhomekeka kuhlobo oluthile lokutshintsha kwemfuza analo umntwana wakho, abanako ukuvelisa iiproteni zeCFTR. Ngamanye amaxesha, banokuvelisa iiproteni ze-CFTR ezingasebenzi kakuhle. Oku kusilela kubangela ukuba i-mucus yakheke emiphungeni yabo kwaye ibabeka emngciphekweni weengxaki.

Zeziphi iintlobo zotshintsho ezinokubangela iCF?

Izazinzulu ziye zaphuhlisa iindlela ezahlukeneyo zokuhlela utshintsho kwi ICFTR Uhlobo. Okwangoku bahlela ICFTR Ukutshintsha kwemfuza kumaqela amahlanu, ngokusekwe kwiingxaki ezinokubangela:

• Iklasi 1: Uguquko kwimveliso yeprotein
• Iklasi 2: ukuguqulwa kweeproteni
• Iklasi 3: ukuguqula utshintsho
• Iklasi 4: Utshintsho olwenziwe
• Iklasi yesi-5: Utshintsho lweprotein olungonelanga

Iindidi ezithile zotshintsho kwimfuzo anazo umntwana wakho zinokuba nefuthe kwiimpawu abazikhulisayo. Inokuchaphazela ukhetho lwabo lonyango.

Ngaba utshintsho kwimfuza luchaphazela njani ukhetho lonyango?

Kwiminyaka yakutshanje, abaphandi baqalise ukuthelekisa iintlobo ezahlukeneyo zamayeza kwiindidi ezahlukeneyo zotshintsho kwi ICFTR Uhlobo. Le nkqubo yaziwa ngokuba yi-theratyping. Inokunceda ugqirha womntwana wakho ukuba abone ukuba loluphi uhlobo lonyango olulungele bona.

Kuxhomekeke kubudala bomntwana wakho kunye nemfuza, ugqirha wabo unokumisela imodyuli yeCFTR. Olu didi lwamayeza lunokusetyenziselwa ukunyanga abanye abantu abane-CF. Iindidi ezithile zeemodyuli zeCFTR zisebenza kuphela kubantu abaneentlobo ezithile ze ICFTR Ukutshintsha kwemfuza.

Ukuza kuthi ga ngoku, iU.S.

• ivacaftor (iKalydeco)
• I-lumacaftor / ivacaftor (iOrkambi)
• i-tezacaftor / ivacaftor (iSymdeko)

Malunga nabantu abangama-60 ekhulwini abaneCF banokuzuza kwelinye lala machiza, inikela ingxelo yeCFF. Kwixesha elizayo, izazinzulu zinethemba lokuvelisa ezinye iindlela zonyango zeCFTR ezinokunceda abantu abaninzi.

Ndiyazi njani ukuba unyango lulungile kumntwana wam?

Ukufunda ukuba umntwana wakho angazuza na kwimodyuli yeCFTR okanye olunye unyango, thetha nogqirha wakhe. Ngamanye amaxesha, ugqirha wabo unokuyalela iimvavanyo ukuba afunde ngakumbi ngemeko yomntwana wakho kunye nendlela abanokuthi basabele ngayo kunyango.

Ukuba iimodyuli zeCFTR azilungelanga umntwana wakho, ezinye iindlela zonyango ziyafumaneka. Umzekelo, ugqirha wabo unokumisela:

• amafinyila
• bronchodilators
• iyeza lokubulala iintsholongwane
• ii-enzymes zokwetyisa

Ukongeza ekumiseleni amayeza, iqela lezempilo lomntwana wakho lingakufundisa indlela yokwenza ubuchule bokususa umoya (i-ACTs) ukukhupha kunye nokukhupha i-mucus kwimiphunga yomntwana wakho.

Ukuthatha

Iindidi ezininzi ezahlukeneyo zotshintsho kwimfuza zinokubangela iCF. Iindidi ezithile zotshintsho kwimfuzo anazo umntwana wakho zinokuchaphazela iimpawu kunye nesicwangciso sonyango. Ukuze ufunde okungakumbi ngeendlela zonyango zomntwana wakho, thetha nogqirha wakhe. Kwiimeko ezininzi, ugqirha wabo uya kuncoma ukuvavanywa kofuzo.