Namhlanje siza kuthetha ngesifo esingcono kakhulu, esichaphazela ukubona!

Ngaba wena okanye umntwana wakho niqalile ukubona utshintsho oluncinci kwindlela enibona ngayo izinto ngqo phambili, ukubona okufipheleyo? Okanye mhlawumbi ugqirha wamehlo ukuxelele okanye umntu othile kusapho lwakho ngeli gama elithi 'Isifo esiBalaseleyo'? Esi sisifo esingaqhelekanga, kodwa esifuzo esinokuchaphazela amehlo omabini. Ke namhlanje, masithethe ngokulula malunga nokuba siyintoni esi sifo siBalaseleyo, indlela esikhula ngayo, zithini iimpawu, kwaye ingaba kukho unyango.

Sisiphi esi sifo sibalaseleyo?

Ngamafutshane, isifo sikaBest sisifo semfuza esichaphazela i-retina yamehlo akho. Amehlo ethu afana nekhamera. I-retina lulwandla olungasemva kweliso olusinceda sibone imifanekiso xa ukukhanya kuyibetha. Inxalenye ye-retina ephakathi kwe-retina esinceda sibone izinto ngqo phambili ibizwa ngokuba yi-macula. Ngoko ke, kwisifo sikaBest, i-macula iba buthathaka kancinci kancinci. Oku kunokwenza kube nzima ukubona izinto ngqo phambili, njengoonobumba nobuso babantu, ngokucacileyo. Kodwa uninzi lwexesha , umbono ongaphandle, okanye ukubona izinto ezikungqongileyo, akuchaphazeleki kangako.

Esi sifo siBalaseleyo sibizwa ngamanye amagama. Ngamanye amaxesha oogqirha banokuthiwa yi-'vitelliform macular dystrophy' okanye 'vitelliform dystrophy'. I-'Dystrophy' ligama lezonyango elichaza ukuwohloka okanye ukuba buthathaka kwesitho esithile.

Kukho enye imeko efana nale, ekwaluhlobo lwe-'vitelliform macular dystrophy'. Nangona kunjalo, ayiqali ebuntwaneni, kodwa ikhula njengoko ukhula, ngesiqhelo phakathi kweminyaka engama-40 nama-60. Ibizwa ngokuba yi-'adult-onset type'.

Ixhaphake kangakanani isifo seBest?

Kunzima ukufumana izibalo ezichanekileyo zokuba bangaphi abantu abanesifo sikaBest. Abanye abantu abazi nokuba banaso kuba abanazo iimpawu. Olunye uphando luthi malunga nomntu omnye kwabayi-16,500, okanye omnye kwabayi-21,000, usenokuba naso. Olunye uphando luthi malunga nomntu omnye kwabayi-15,000 . Olunye luthi mhlawumbi ngumntu omnye kwabayi-10,000 . Nangona kunjalo, sinokuqonda ukuba esi sisifo esingaqhelekanga .

Zithini izigaba zesifo esiBalaseleyo?

Esi sifo siBalaseleyo sinokudlula kwizigaba ezintandathu eziphambili. Masibone ukuba zeziphi.

1. Inqanaba loku-1 - Inqanaba le-Previtelliform:

Esi sisiqalo nje. Ixesha elininzi, awuyi kuba nazimpawu okwangoku.Into etyheli ayikaqali ukwakheka phantsi kwe-retina yakho. Oku kunokufunyaniswa ngengozi ngexesha lovavanyo lwamehlo.

2. Inqanaba lesiBini - Inqanaba leVitelliform:

Igama elithi `vitelliform` lithetha ``okwimo yeqanda``. Kweli nqanaba, into etyheli iqala ukuqokelelana phantsi kwe-retina yakho, kwindawo ye``macula``, embindini wombono wakho. Cinga ngayo njengeqanda elincinci elityheli. Umbono wakho usenokuba usemhle kweli nqanaba.

3. Inqanaba lesi-3 - Inqanaba le-pseudohypopyon:

Kule nqanaba, into etyheli inokuba lulwelo ize yenze i-cyst phantsi kwe-retina. Oku kufana ne-blister encinci egcwele ulwelo ngaphakathi kweliso.

4. Inqanaba lesi-4 - Inqanaba le-Vitelliruptive:

Kulapho izinto ezimthubi ezazihlala ziqokelelana ziqala khona ukuqhekeka nokusasazeka. Njengoko ezi zinto ziqhekeka, iiseli ezikwi-retina zinokonakala. Ngeli xesha, unokuqala ukubona utshintsho embonweni wakho, njengokubona okufipheleyo.

5. Inqanaba V - Inqanaba lokungapheleli:

Kule nqanaba, into etyheli iphantse yaphela. Kodwa amanxeba kunye neeseli ezonakeleyo zihlala apho zazikhona. Lo monakalo unokuphazamisa umbono ngakumbi. Abanye abaphandi bacinga ukuba eli linqanaba lokugqibela lesifo sikaBest.

6. Inqanaba lesi-6 - Ukwanda kwemithambo yegazi kwi-Choroidal (CNV):

Abanye abaphandi bacinga ukuba le meko ibizwa ngokuba yi-'CNV' linqanaba lokugqibela lesifo iBest. Abanye bacinga ukuba yingxaki yesi sifo. Malunga nama-20% abantu abane-'Best disease' banokuba nale meko. 'I-'Neovascularization' kukwakheka kwemithambo yegazi emitsha. 'I-'Choroid' yi-membrane ephakathi kwe-'retina' kunye ne-'sclera' yeliso. Kule meko ye-'CNV', imithambo yegazi emitsha nebuthathaka yakha kumaleko we-'choroid'. Le mithambo yegazi emitsha ibuthathaka kangangokuba inokuvuza igazi okanye ulwelo. Ukuba oku kuyenzeka, umbono wakho unokuba mandundu.

Zithini iimpawu zesifo sikaBest?

Ixesha elininzi, uza kufumanisa ngesifo sikaBest kuphela emva kokuhlolwa kwamehlo, kuba ngamanye amaxesha akukho zimpawu zibonakalayo. Kodwa ukuba iimpawu ziyavela, zingabandakanya:

• Umbono ofipheleyo: Izinto ezibonakala zingaphambili zisenokubonakala zingacacanga okanye zingacacanga.
• Iingxaki zokubona ngqo: Ingxaki kukubona izinto ngqo phambi kwakho. Kodwa usenokuba nombono olungileyo kuyo yonke indawo, okanye umbono osecaleni.
• Ukubona izinto zitshintsha imilo (iMetamorphopsia):Khawuthelekelele, ujonge umgca othe tye, kodwa ubonakala kuwe njengomgca ogobileyo notsaliweyo. Ungazibona izinto ezifana nezikhonkwane zomnyango kunye neefreyimu zeefestile njengoko zitsaliwe. Oku kubizwa ngokuba yi-'metamorphopsia'.
• Ukulahlekelwa kakhulu kukubona: Abanye abantu banokulahlekelwa kakhulu kukubona ngokuhamba kwexesha.
• Umahluko ekuboneni phakathi kwamehlo amabini: Umbono kwelinye iliso unokuba mncinci kunelinye. Okanye umbono kuwo omabini amehlo usenokunganciphi ngokulinganayo, kodwa usenokuncipha ukuya kumanqanaba ahlukeneyo.

Yintoni imbangela yesifo sikaBest?

Isifo sikaBest sibangelwa yimeko yemfuza. Ngamafutshane, sibangelwa lutshintsho kwiimfuza esizizuza kubazali bethu.

Isifo sikaBest sisifo esithi "autosomal dominant". Eli ligama lezonyango, kodwa kulula ukuliqonda. "Autosomal dominant" lithetha ukuba umntwana akufuneki afumane i-gene eguquliweyo kubazali bobabini, kodwa kufuneka abe mnye kuphela. Oko kuthetha ukuba ukuba omnye wabazali une-gene eguquliweyo, abantwana babo banethuba elingama-50% lokufumana esi sifo. Kufana nokuphosa ingqekembe uze uqhawuke iintloko.

Okumangalisayo kukuba, ijini efanayo ebangela isifo sikaBest ikwabangela ezinye iintlobo zesifo samehlo esizuzwe njengelifa esibizwa ngokuba yi-retinitis pigmentosa. Oku kuthetha ukuba utshintsho olukule jini lunokubangela iintlobo ngeentlobo zezifo zamehlo.

Isifo sikaBest sifunyanwa njani?

Ixesha elininzi, ugqirha uya kuxilonga isifo sikaBest xa uphakathi kweminyaka emihlanu nelishumi ubudala, okanye uninzi xa uneminyaka engama-20 ubudala.

Xa undwendwela ingcali yokhathalelo lwamehlo ngenxa yengxaki yamehlo, baya kuqala bakubuze ngembali yakho yezonyango, babuze ukuba kukho umntu kusapho lwakho onengxaki yamehlo, baze emva koko benze uvavanyo lwamehlo. Ukongeza, banokwenza novavanyo oluthile olukhethekileyo. Umzekelo:

• I-Fluorescein angiography: Olu luvavanyo lomfanekiso. Idayi ekhethekileyo ifakwa emthanjeni osengalweni yakho kwaye kuthathwe imifanekiso yemithambo yegazi engaphakathi kweliso lakho. Oku kunokunceda ukubona ukuba kukho ukuvuza okanye ukungaqheleki kwimithambo yegazi.
• I-Optical coherence tomography (OCT): Olu luvavanyo lokujonga olungenalo uhlaselo. Lusebenzisa imisebe yokukhanya ukuthatha imifanekiso enqamlezileyo yomva weliso lakho, ngakumbi i-retina kunye ne-macula. Kufana nokusika ikhekhe uze ujonge ngaphakathi. Lunokubona izinto ezinje ngobukhulu beengqimba ze-retina, nakuphi na ukudumba, kunye nokwakheka kolwelo.
• Ifoto ye-fundus enemibala: Oku kuthatha imifanekiso ecacileyo ye-retina yakho, imithambo yegazi ehambelana nayo, kunye nentloko ye-optic nerve. Oku kunokukunceda ubone ukuba kukho na iidiphozithi ezifana neqanda elityheli.
• I-electrophysiology yamehlo: Olu luhlu lweemvavanyo. Ezi mvavanyo zilinganisa umsebenzi wombane ongabonakaliyo owenzeka xa amehlo ethu esabela ekukhanyeni. Oku kunokunika umbono wokuba iiseli ezikwi-retina zisebenza kakuhle kangakanani.
• Uvavanyo lwemfuza: Olu vavanyo lunokuqinisekisa ngokuqinisekileyo ukuba kukho naluphi na utshintsho kwiimfuza zakho olunxulumene nesifo seBest.

Ngamanye amaxesha ugqirha usenokufuna ukuthetha namanye amalungu osapho lwakho okanye ade ahlole amehlo abo, kuba esi sisifo esidluliselwa kwilifa.

Iphathwa njani isifo sikaBest?

Nanamhlanje, akukho nyango lwesifo sikaBest. Yiloo nto yokuqala ekufuneka siyiqonde.

Eyona ndlela ingcono yokulawula esi sifo ngokusekelwe kwiimpawu zakho kunye nenqanaba lesi sifo. Nje ukuba uxilongwe, kubalulekile ukuba uhlolwe amehlo rhoqo. Kulapho ke kuphela apho unokukhawuleza ubone naluphi na utshintsho emehlweni akho kwaye uthathe amanyathelo afunekayo.

Kwinqanaba lokuqala, usenokungadingi naluphi na unyango. Nangona kunjalo, ukuba unezinye iimpazamo zokuhombisa ubuso, ezifana nokubona kude, unokufuna ukusebenzisa iiglasi. Abantu abanesifo sikaBest badla ngokuba neengxaki zokubona kude. Kwakhona, ukuba unesifo se-cataract, unokufuna utyando lwe-cataract.

Kodwa ukuba unemeko ebesithethe ngayo ngaphambili ebizwa ngokuba yi-choroidal neovascularization (CNV), eyile kwakheka kwemithambo yegazi emitsha ebuthathaka, ugqirha wakho unokucebisa unyango olufana nolu ukuze kuthintelwe ukukhula kwemithambo yegazi emitsha:

• Izinto ezilwa nokukhula kwemithambo yegazi (i-Anti-VEGF): Ezi ziimithi. Ezi ziyobisi zifakwa kwi-vitreous cavity engaphakathi kweliso lakho. Injongo kukuyeka ukukhula kwemithambo yegazi emitsha, evuzayo nokuyinciphisa.
• Unyango lweLaser: Olu nyango lusebenzisa imisebe yelaser ukuvala okanye ukutshabalalisa imithambo yegazi engaqhelekanga.
• Unyango lwe-Photodynamic (PDT): Olu lonyango oluntsonkothileyo kancinci. Apha, kufakwa iyeza elikhethekileyo emzimbeni wakho, emva koko iyeza lenziwa libe novakalelo ekukhanyeni, kwaye umqadi welaser ujoliswe kwimithambo yegazi echaphazelekayo, uyonakalisa kwaye uyithintele ekuphumeni kwegazi.

Ukongeza, ugqirha wakho unokucebisa ukuba usebenzise izixhobo zokunceda ukubona kancinci, ezifana nezixhobo zokukhulisa, ukukhanya okukhethekileyo, kunye nesoftware efundeka lula.

Abaphandi sele bephanda ukuba ingaba izinto zemfuza zingasetyenziswa na ukunyanga okanye ukuthintela esi sifo. Unyango lwemfuza lusesekwinqanaba lophando, kodwa kunethemba lokuba luya kuvelisa iziphumo eziphumelelayo kwixesha elizayo.

Ngaba umngcipheko wokufumana isifo esingcono ungancitshiswa?

Inene, akukho nto unokuyenza ukuthintela isifo sikaBest. Sisifo semfuza. Kodwa ukuba kukho umntu kusapho lwakho onesifo sikaBest, okanye ukuba ufumanisa ukuba unaso, kunokuba kubalulekile ukufumana ingcebiso yemfuza ngaphambi kokuba ube nabantwana. Umcebisi wemfuza unokujonga imbali yosapho lwakho aze achaze umngcipheko wokuba abantwana bakho bafumane esi sifo njengelifa.

Kuza kwenzeka ntoni ukuba ndine-Best Disease?

Isifo sikaBest asisosifo sibulalayo. Oko kuthetha ukuba asisongeli ubomi. Kodwa, njengoko bekutshiwo ngaphambili, asinyangeki. Awuyi kuba yimfama ngokupheleleyo ngenxa yesifo sikaBest. Kodwa usenokuba nombono ophantsi. Oko kuthetha ukuba kunokuba nzima kancinci ukwenza imisebenzi yemihla ngemihla, kodwa awuyi kulahlekelwa kukubona ngokupheleleyo.

Ndingazinyamekela njani?

Kukho ukutya okulungele impilo yamehlo.

"Ukutya okunempilo kubaluleke kakhulu emehlweni."

Umzekelo, ukutya iintlanzi iintsuku ezimbalwa ngeveki, kunye nokongeza imifuno eluhlaza, iziqhamo, kunye namandongomane ekutyeni kwakho kulungile emehlweni akho. Ezi zinto zibonelela ngezondlo ezinobuhlobo namehlo ezifana ne-omega-3 fatty acids kunye neevithamini.

Ukuba unesifo sikaBest, kubalulekile ukuba uhlolwe rhoqo ngugqirha, ingakumbi uhlolo lwamehlo. Ukuba ubona naluphi na utshintsho ekuboneni kwakho okanye kwimpilo yakho ngokubanzi, xelela ugqirha wakho ngoko nangoko. Ungambuza ugqirha wakho imibuzo efana nale:

• " Ungandicebisa umcebisi wezemfuza ukuba andincede ndigqibe ukuba ndingaba nabantwana na?"
• "Ngaba ndifanelekile na ukuthatha inxaxheba kuvavanyo lwezonyango lwesi sifo?"
• " Ungacebisa ugqirha wamehlo ogxile kwizifo ezingaboni kakuhle?"
• " Ngaba unyango lwe-gene luyindlela yonyango kum?"

Yintoni umahluko phakathi kwesifo iBest kunye nesifo iStargardt?

Isifo seStargardt kunye neBest disease zombini zizifo zemfuza, kwaye zombini zichaphazela umbindi wombono elisweni (i-macula). Oku kuthetha ukuba umbono othe ngqo uyachaphazeleka.

Kodwa ezi zifo zimbini zibangelwa kukutshintsha kwezakhi zofuzo ezahlukeneyo.

Kwisifo iStargardt, ungabona amabala atyheli okanye amhlophe kwi-retina yakho. Kodwa kwisifo iBest, ungabona i-cyst enkulu, etyheli, efana neqanda, efana ne-oval efana ne-yolk phantsi kwe-macula. Ngoko ke, ezi zifo zimbini zinokwahlulwa ngezi mpawu kunye novavanyo lwemfuza.

Ukuphila nemeko esongela ukubona kunokuba nzima. Kodwa ukuba unesifo sikaBest, kukho izixhobo ezininzi kunye neenkonzo ezinokukunceda wenze izinto zibe lula. Buza ugqirha wakho ngazo.

Okokugqibela, izinto ekufuneka uzikhumbule

Kulungile, ngoko ke sithethe kakhulu ngesifo esiBalaseleyo. Masiphinde sichaze:

• Isifo sikaBest sisifo esidluliselwa kwilifa esichaphazela umbindi wombono elisweni (i-macula). Oku kunokukhokelela ekungabonini kakuhle.
• Nangona ingenakunyangwa ngokupheleleyo, ayisongeli ubomi. Ukubona okuphantsi kunokubakho, kodwa ukungaboni ngokupheleleyo akwenzeki.
• Kubaluleke kakhulu ukufumanisa isifo kwangethuba kwaye kwenziwe uvavanyo lwezonyango rhoqo.
• Kukho unyango lweengxaki ezifana ne-'CNV'.
• Ingcebiso ngemfuza yinto enokubaluleka kwabo baceba usapho.
• Ukutya ukutya okulungele amehlo nokusebenzisa izixhobo zokunceda amehlo angaboni kakuhle kunokwenza ubomi bube lula.

Khumbula, awuwedwa. Kukho oogqirha, abacebisi, kunye neendlela ezinokukunceda uphile nezi meko. Eyona nto ibalulekileyo kukuhlala uqinile kwaye ulandele imiyalelo efunekayo. Ukuba unemibuzo okanye amathandabuzo, thetha ngokukhululekileyo nogqirha wakho.

Isifo esingcono, Isifo esingcono, izifo zamehlo, iziko lokubona, i-macula, izifo zemfuza, ukulahleka kokubona, ukuvavanywa kwamehlo