Ingaba umbono wakho awuboni kakuhle ebusuku? Awukwazi ukubona macala omabini? Masifunde ngeRetinitis Pigmentosa (RP)!

Ngaba wena okanye umntwana wakho nivakalelwa kukuba umbono wenu awubonakali kakuhle ebusuku? Ngaba ngamanye amaxesha nidibana nezinto ezingaphakathi endlwini yenu xa ukukhanya kuphantsi ngokuhlwa? Okanye nibona iimoto ezivela kumacala omabini zisiza kuni ngequbuliso ngelixa nihamba endleleni? Usenokuba anizange nizinike ngqalelo kangako ezi zinto. Kodwa ezi zisenokuba ziimpawu ekufuneka nizixhalabise ngazo. Namhlanje siza kuthetha ngesifo samehlo esibangela ezo mpawu, kodwa abantu abaninzi kwilizwe lethu abangazi kakuhle. YiRetinitis Pigmentosa, okanye (RP) ngamafutshane.

Ngamafutshane, yintoni iRetinitis Pigmentosa (RP)?

I-Retinitis Pigmentosa (RP) ayisosifo siqhelekileyo. Ligama eliqhelekileyo leqela lezifo zamehlo ezizuzwe njengelifa, oko kukuthi, ezizuzwe njengelifa ngokwemfuza. Kule meko, eyona ndawo ibuthathaka yeliso lakho, ngasemva, ibizwa ngokuba yi-retina.

Khawuthelekelele ukuba iliso lakho lifana nekhamera elungileyo. Ifilimu eqengqelekayo yale khamera ibizwa ngokuba yi-`(Retina)`. Xa ukukhanya okuvela ngaphandle kungena elisweni kuze kubethe le `(Retina)`, oko kukuthi, ifilimu, kudala imiqondiso yombane kwaye iye engqondweni. Ingqondo itolika ezo miqondiso njengomfanekiso kwaye isinika imvakalelo 'yokubona'. Ngoku khawuthelekelele, nokuba ikhamera yakho intle kangakanani na, ukuba ifilimu ekuyo yonakele, ngaba ifoto oyithathayo iya kuphuma icacile? Ayizukuphuma, akunjalo? Yiloo nto eyenzekayo kwi-RP. Iiseli ezikwi-`(Retina)` kancinci kancinci ziyabuthathaka kwaye zingasebenzi. Emva koko umbono wethu uyanyamalala kancinci kancinci.

Le yimeko esizalwa sinayo. Kodwa iimpawu zihlala ziqala ukubonakala xa umntwana efikisa okanye xa efikisa. Okokuqala, umbono wasebusuku uqala ukuncipha. Emva koko, umbono ongaphandle uyanyamalala kancinci kancinci. Ngokuhamba kwexesha, lo mbono uba mxinwa ngakumbi, kwaye xa umntu eneminyaka engama-40 ubudala, abantu abaninzi bancipha kakhulu ekuboneni. Abanye basenokude babe ziimfama ngokusemthethweni. Kodwa utshintsho lunzima kangangokuba kuthatha ixesha elithile ukuba abanye abantu baqonde ukuba esi sisifo.

Lithetha ukuthini eli gama?

Igama elithi "Retinitis Pigmentosa" liyinkohliso kancinci. Kwizonyango, ukuba igama liphela ngo-"-itis", lithetha 'ukudumba' okanye 'ukudumba'. Umzekelo, njenge `(Appendicitis)`. Kodwa i-RP ayikokudumba kwe `(Retina)`. Okwenzekayo apha kukuba iiseli ze `(Retina)` ziyathamba kancinci kwaye ziphele. Ke ngoko, igama elifanelekileyo loku ngu `(Retinal Dystrophy).`

Ngoko ke lithetha ukuthini igama elithi "Pigmentosa"? Linxulumene nale meko. Xa iiseli ze-photoreceptor kwi-retina yethu zisifa, zikhupha umbala. Lo mbala ubekwa kwi-retina. Xa ugqirha ehlola iliso, unokubona la mabala embala agciniweyo. Yiyo loo nto igama elithi "Pigmentosa" longezwa kweli gama.

Zithini iimpawu ze-RP?

Njengoko besitshilo ngaphambili, ezi mpawu zibonakala kancinci kakhulu. Zinokukhula kancinci kancinci ngokuhamba kweminyaka. Ngoko ke kunokuba nzima ukuzibona kwasekuqaleni.

Makhe sibone ukuba zeziphi ezi mpawu. Ukuze siqonde kakuhle, masizahlule zibe ngamacandelo amabini.

Kutheni le meko isenzeka?

Isizathu esiphambili soku kukutshintsha kwezakhi zofuzo . Ngamafutshane, izakhi zofuzo zethu ziqulathe imiyalelo epheleleyo yendlela imizimba yethu eyenziwe ngayo nendlela esebenza ngayo. Oku kufana neplani esetyenziselwa ukwakha indlu. Ukuba kukho utshintsho okanye isiphene kule plani, oko kukuthi, kwiizakhi zofuzo, oko kukuthi, izinto ezenziwe ngayo, oko kukuthi, amalungu omzimba wethu, zisenokungakhuli okanye zingasebenzi kakuhle.

Izazinzulu zifumanise phantse iinguqu ezili-100 ezinjalo zemfuza ezinokubangela i-RP. Ungayifumana njengelifa le siphene semfuza kumama okanye kutata wakho. Okanye, utshintsho olutsha lwemfuza lunokwenzeka emzimbeni wakho ngengozi, ngaphandle kokuba nabani na kusapho lwakho abe nalo.

Ngenxa yokuba kukho iintlobo ezininzi zezakhi zofuzo, i-gene nganye yonakalisa i-retina ngokwahlukileyo. Yiyo loo nto i-RP yahluka ukusuka kumntu ukuya komnye. Abanye abantu balahlekelwa kukubona ngokukhawuleza. Abanye balahlekelwa kukubona kancinci kancinci. Ngamanye amaxesha i-RP inokuhamba nezinye iimpawu ezininzi kwaye inokuvela njengenye imeko, njenge-Usher syndrome.

Kwenzeka ntoni ngokwenene ngaphakathi kweliso?

Masingene nzulu kancinci sibone ukuba kwenzeka ntoni ngaphakathi kweliso. Kwi-"Retina" ebesithethe ngayo ngaphambili, kukho uhlobo oluthile lweseli olubona ukukhanya. Sibiza ezi `'Photoreceptors''. Ezi ziiseli ezithatha ukukhanya okungena elisweni, ziguqule zibe yisignali yombane, zize zithumele engqondweni.

Kukho iintlobo ezimbini eziphambili ze-photoreceptors:

• Iinduku: Ezi zisinika umbono ebumnyameni, oko kukuthi, ekukhanyeni okuphantsi. Azikwazi ukwahlula imibala, zisinika umbono omnyama nomhlophe kuphela. Kukho izigidi zezi `(Iinduku)` kwi `(iRetina)` yethu, ingakumbi kumacala omabini e `(iRetina)`.
• Iikhowuni: Ezi zizinto ezisinceda sibone imibala kunye neenkcukacha ezintle ngokucacileyo ekukhanyeni okuhle, oko kukuthi, emini.

Kwi-RP , iintonga zihlala zonakala kuqala . Yiyo loo nto iimpawu zokuqala zibonakala zinciphile ebusuku kwaye zilahlekelwa yimbono engaphandle. Ekuhambeni kwexesha, ii-cones nazo ziqala ukonakala. Kulapho ke izinto ezifana nokubona imibala nokusebenza kufutshane zichaphazeleka khona.

Ugqirha uxilonga njani esi sifo?

Ukuba unale mpawu, kuya kufuneka ubonane nogqirha wamehlo (Ophthalmologist). Uza kuhlola amehlo akho. Uvavanyo lwamehlo oluqhelekileyo lunokufumanisa iimpawu zale meko.

Kukho iimvavanyo ezininzi ezenziwa rhoqo ukuqinisekisa isifo:

• Uvavanyo lweNdawo yokubona: Oku kulinganisa umbono wakho ongaphandle, nto leyo ebonisa ukuba ungabona kangakanani emacaleni. Oku kunokunceda ekuqinisekiseni ukuba unombono we-tunnel.
• Isibane esiqhekekileyo kunye neFundoscopy: Kusetyenziswa isixhobo esikhethekileyo ukuhlola ingaphakathi lelihlo lakho (i-retina). Oku kunokujonga ukuba akukho mibala enxulumene ne-RP.
• Imifanekiso yeRetina: Imifanekiso yeretina enesisombululo esiphezulu ithathwa ukuze kufundwe utshintsho kuyo.
• Uvavanyo lwe-ERG (i-Electroretinogram): Olu luvavanyo olukhethekileyo kancinci. Okwenziwayo apha kukulinganisa imiqondiso yombane ekhutshwa yi-`(i-Retina)` yakho ukuze ubone ukuba isabela njani ekukhanyeni. Kwi-RP, le miqondiso ibuthathaka kakhulu.
• Uvavanyo lweMfuzo: Olu vavanyo lungenziwa ngokuthatha isampuli yegazi nokufumanisa ukuba yeyiphi kanye kanye ingxaki yemfuza ebangela esi sifo.

Zithini iindlela zonyango zoku?

Le yeyona nto ndingathandi ukuyiva. Ngelishwa, akukho nyango lwe-Retinitis Pigmentosa okwangoku. Kodwa, ungalilahli ithemba. Zininzi iindlela zokunceda abo baphila nesi sifo nokwenza ubomi bube lula.

Ugqirha wakho angakuthumela kwiinkonzo zokulungisa umbono. Ezi zingabandakanya:

• Izixhobo zokubona - iiglasi zokukhulisa, isoftware ekhethekileyo.
• Unyango lomsebenzi - Uqeqesho lwendlela yokwenza imisebenzi yemihla ngemihla kunye nokukhubazeka kokubona.
• Imfundo ekhethekileyo okanye iinkonzo zomsebenzi.
• Amaqela eengcebiso okanye enkxaso.

Okwangoku, izazinzulu ziyaqhubeka nokuphanda unyango lwesi sifo. Okwangoku kukho iindlela ezahlukeneyo zonyango ezivavanyiweyo ezibonise iziphumo ezithembisayo:

• Unyango lweZiza: Eli lelona themba likhulu. Okwenziwayo apha kukuzama ukulungisa i-gene enesiphene. Okwangoku, kukho unyango olunye oluvunyiweyo lohlobo olunye lwe-gene, `(RPE65).` Kodwa uphando luyaqhubeka ngokukhawuleza nakwezinye ii-gene.
• Izongezo zeVithamin A: Kwezinye iintlobo ze-RP, ukuthatha idosi efanelekileyo yeVithamin A kuye kwafunyaniswa ukuba kuyayicothisa inkqubela yesi sifo. Kodwa oku kubaluleke kakhulu: Ungaze uthathe iVithamin A ngaphandle kokubonisana nogqirha wakho. Abanye abantu banokwenzakala ngokuthatha iVithamin A eninzi. Ngoko ke esi sisigqibo esinokwenziwa ngugqirha wakho kuphela.
• I-Retinal Prosthesis: Esi sisixhobo se-elektroniki esifakwe ngaphakathi kweliso. Sinokubonelela ngokubona okuthile. Nangona kunjalo, iziphumo ziyahluka kumntu nomntu. Ungabuza ugqirha wakho ukuba olu khetho lulungile na kuwe.