Ngaba nawe unengxaki yokubona ebusuku? Ngaba amehlo akho aya ewohloka kancinci kancinci? Masifunde nge-'Retinitis Pigmentosa' (RP)!

Ngamanye amaxesha uba nobunzima bokuqhuba ebusuku okanye kwiimeko zokukhanya okuphantsi, akunjalo? Okanye, ngaba wakha wangqubana nomntu ngaphandle kokumbona esiza kuwe? Ukuba ukhe wahlangabezana noku, unobangela usenokuba yimeko yamehlo ebizwa ngokuba yi-'Retinitis Pigmentosa' (RP). Ungakhathazeki, le ayisiyongxaki iqhelekileyo, kodwa kubalulekile ukuba uyiqaphele. Makhe sithethe ngayo ngokweenkcukacha.

Yintoni i-'Retinitis Pigmentosa' (RP)? Kutheni ibaluleke kangaka?

Ngamafutshane, igama elithi 'Retinitis Pigmentosa' (RP) ligama eliqhelekileyo leqela lezifo zamehlo ezinokudluliselwa kwizizukulwana ngezizukulwana. Lichaphazela kakhulu i-'retina' engaphakathi kweliso lakho.

Cinga ngeliso lakho njengekhamera yefilimu yakudala. Ifoto kwezo khamera ithathwe kwifilimu. Yiyo loo nto, ngaphakathi kweliso lakho, ngasemva, kukho inwebu ethambileyo neqondayo ekukhanyeni ebizwa ngokuba yi-retina. Xa ukukhanya okuvela kwizinto esizibonayo kuwela kwi-retina, kuguqula oko kukhanya kube ziimpawu zombane. Emva koko ezo zimpawu ziya engqondweni size sibone, 'Owu, oku kukuthi, oku kukuthi.'

Ngoko ke, ukuba i-retina ayisebenzi kakuhle, kufana nokuba ifilimu ekwikhamera yonakele. Nokuba ugxile kangakanani na, umfanekiso awuyi kuphuma ucacile. Ngokufanayo, ukuba une-retina enesifo, nokuba unxiba iiglasi okanye iilensi zoqhagamshelwano, kuya kuyichaphazela indlela obona ngayo.

I-retina ineentlobo ezikhethekileyo zeeseli zemithambo-luvo ezisabela ekukhanyeni. Ezi zibizwa ngokuba ziiseli ze-photoreceptor. Zimbini iintlobo zezi: ii-rods kunye nee-cones. Ii-rods zisinceda sibone ekukhanyeni okuphantsi, ingakumbi ebusuku. Ii-cones zisinceda sibone imibala kunye neenkcukacha ezincinci ngokucacileyo. Kunye nezi seli, kukho olunye uhlobo lweseli kwi-retina olubizwa ngokuba ziiseli ze-retinal pigment epithelium. Zonke ezi seli zisebenza kunye kwaye kunye ukusinika umbono olungileyo.

I-Retinitis Pigmentosa (RP) kunye nezinye izifo ze-retina ezizuzwe njengelifa (ii-IRD) zibangelwa lutshintsho kwiijini ezenza ezi seli zisebenze ngokufanelekileyo, ezibizwa ngokuba zii-genetic mutations . Oku kubangela ukuba iiseli zibe buthathaka kancinci kancinci kwaye ziyeke ukusebenza.

I-RP ayisosifo sinye. Ngenxa yokuba liqela lezifo, ukubona kunokwahluka kumntu nomntu. Abantu abaninzi baqala ukubona kancinci , kwaye abanye abantu banokulahlekelwa kukubona kwabo ngokupheleleyo. Olu tshintsho lokubona ludla ngokuqala ebuntwaneni. Kodwa ngamanye amaxesha olu tshintsho lwenzeka kancinci kangangokuba usenokungaluboni. Kwabanye abantu, ukulahleka kokubona kwenzeka ngokukhawuleza. Kwezinye iintlobo ze-RP, ukulahleka kokubona kuyeka kwinqanaba elithile.

Eyona nto ibalulekileyo kukuba i-Retinitis Pigmentosa idla ngokuchaphazela amehlo omabini .

Zithini iimpawu ze-RP? Ikuchaphazela njani?

Iimpawu ze-'Retinitis Pigmentosa' azibonakali ngequbuliso. Zibonakala kancinci kancinci. Nazi ezinye zeempawu zokuqala:

• Iingxaki Zokubona Ebusuku: Usenokufumanisa ukuba unengxaki yokubona izinto ebusuku, ingakumbi xa ukukhanya kuncinci. Oku kunokuba nzima xa uhamba esitalatweni ebusuku okanye ngaphakathi endlwini ekukhanyeni okuncinci.
• Iingxaki zokubona ekukhanyeni okufipheleyo: Kunokuba nzima ukubona izinto ngokucacileyo, kungekuphela nje ebusuku, kodwa nakwiindawo ezifana negumbi elimnyama kancinci okanye iholo yemiboniso bhanyabhanya.
• Iindawo ezingaboniyo embonweni ongaphandle: Nangona unokubona into oyijongileyo, usenokungakwazi ukubona okukungqongileyo, njengesithuthi esivela ecaleni okanye umntu osondelayo. Yiyo loo nto abanye abantu bengqubana nezinto ngengozi.

Ekuhambeni kwexesha, ezinye iimpawu zinokuvela. Ezi ziquka:

• Imvakalelo yokukhanya okukhazimlayo okanye okubengezela: Abanye abantu banokufumana ukukhanya okukhazimlayo okanye imvakalelo yokubethwa ngumbane.
• Umbono weTunnel (Ukuba nombono ophakathi kuphela): Oku kufana nokujonga ihlabathi ngetyhubhu. Ungabona kuphela okuthe ngqo phambili, kwaye akukho nto ikujikelezileyo.
• I-Photophobia - Ukuba novelwano okanye ukungakhululeki ekukhanyeni okuqaqambileyo: Amehlo aba luhlaza okwesibhakabhaka kwaye kuba nzima ukuwabona xa evezwa lilanga okanye izibane ezikhanyayo.
• Ukulahlekelwa yikhono lokubona imibala: Imibala isenokungabonakali ikhanya okanye icacile njengoko yayinjalo ngaphambili.
• Ukuba nombono ophantsi kakhulu: Kwiimeko ezinzima kakhulu, umbono unokuncipha kakhulu.

Kubalulekile: Ukuba unenye okanye ngaphezulu kwezi mpawu, kungcono ukubona ugqirha wamehlo ngokukhawuleza.

Kutheni i-'Retinitis Pigmentosa' ikhula? Yintoni unobangela woku?

Eyona nto ibangela i-`Retinitis Pigmentosa` (RP) kunye nezinye i-`Inherited Retinal Diseases (IRDs)` kukutshintsha okuthile `(i-genetic mutations)` kwiijini zethu . Ezi jini zezona zivelisa iiseli kwi-`(retina)` yethu kwaye zenze zisebenze ngokufanelekileyo. Ngoko ke, xa kukho impazamo okanye utshintsho kwezi jini, ezo seli azikwazi ukusebenza ngokufanelekileyo. Ekuhambeni kwexesha, ezo seli ziyafa kancinci kancinci. Kulapho ukubona kuncipha kancinci kancinci.

Ngenxa yokuba lufuzo, abantwana banokulufumana olu tshintsho lwemfuza kubazali babo. Kodwa akuthethi ukuba uza kuba nale meko kuba nje kukho umntu kusapho lwakho onalo. Kunokwenzeka ukuba nawe ube nale meko ngaphandle kokuba nabani na kusapho lwakho abe nayo. Yiyo loo nto uvavanyo lwemfuza lubalulekile.

Ixhaphake kangakanani le meko ehlabathini?

Ngokwezibalo zaseYurophu naseMelika, umntu omnye kubantu abayi-3,500 ukuya kwi-4,000 unokuba ne-'Retinitis Pigmentosa'. Kwihlabathi liphela, kuqikelelwa ukuba malunga nezigidi ezibini zabantu abanesifo esinjalo. Kukho abantu abanaso eSri Lanka. Ngoko ke, kubalulekile ukukwazi oku.

Oogqirha bayixilonga njani iRetinitis Pigmentosa (RP)?

Ukuba ukrokrela ukuba une-RP, ugqirha wakho uza kwenza iimvavanyo ezahlukeneyo ukuze akuhlole. Kubaluleke kakhulu ukuba uhlolwe amehlo rhoqo.

Uvavanyo lwamehlo oluvundlileyo ngovavanyo lwentsimi ebonakalayo

Kule meko, ugqirha wamehlo okanye ugqirha wamehlo wenza oku kulandelayo:

• Bakuxelela ukuba ufunde umbhalo oseludongeni.
• Ikuxelela ukuba ujonge into ngamehlo omabini.
• Uxinzelelo olusemehlweni akho luyalinganiswa.
• Uvavanyo lwentsimi ebonakalayo lujonga umbono wakho ongaphandle.
• Ukubukela indlela iliso lakho elisabela ngayo ekukhanyeni.
• Iliso liyavuleka ngokufaka amaconsi eliso esweni. Oku kuvumela i-retina ukuba ihlolwe ngokusondeleyo.
• Ungathatha nemifanekiso ye-retina.

Uvavanyo lwe-Electroretinography (ERG)

Olu luvavanyo olukhethekileyo. Lulinganisa indlela i-retina yakho esabela ngayo ekukhanyeni. Lulinganisa indlela ezisebenza ngayo iiseli ezahlukeneyo kwi-retina yakho (ii-rods kunye nee-cones esithethe ngazo). Lubandakanya ukukhanya kwezibane ezahlukeneyo phambi kwamehlo akho. Luyinxalenye yeqela lovavanyo olubizwa ngokuba yi-ophthalmic electrophysiology tests. Olu vavanyo lukwajonga indlela amehlo akho kunye nengqondo yakho ezisebenza ngayo oko ukubonayo.

Iskeni se-Optical Coherence Tomography (OCT)

Olu luvavanyo olungangenisi gazi. Olu vavanyo lwe-OCT scan lunokulinganisa ubukhulu be-retina yakho kwaye luhlalutye impilo yeeleya zayo. Okufuneka ukwenze kukujonga ithagethi, kwaye ikhamera ekhethekileyo ithatha imifanekiso yangaphakathi kweliso lakho.

Uvavanyo lwe-`Fundus Autofluorescence (FAF)`

Olu vavanyo "lokufota" alungenisi ntsholongwane oluthatha imifanekiso yeliso. Lunokubonelela ngolwazi oluninzi malunga nempilo ye-retina. Lusetyenziselwa ukuchonga, ukunyanga, kunye nokubeka esweni isifo.

Kunye nezi mvavanyo, ugqirha wakho unokucebisa uvavanyo lwemfuza kunye nomcebisi wemfuza.Ungacebisa nokutyelela. Kuba ukuba ufumanisa ukuba yintoni kanye kanye ebangela utshintsho lwemfuza i-RP, ungafumana umbono wendlela esi sifo esiya kuziphatha ngayo kwixesha elizayo kunye nokuba siyawachaphazela na amanye amalungu osapho. Kubalulekile ukufumana unyango olutsha, umzekelo, unyango lwemfuza, okanye ukuthatha inxaxheba kwizilingo zeklinikhi ezinxulumene noko.

Ziziphi iindlela zonyango zesi sifo esithi `Retinitis Pigmentosa` (RP)? Iphathwa njani?

Enyanisweni, kubekho inkqubela phambili enkulu kunyango lwe-Retinitis Pigmentosa (RP) kunye nezinye izifo ze-IRD kwiminyaka yakutshanje, ingakumbi ngesithembiso sonyango olutsha olufana nonyango lwezakhi zofuzo.

Nazi ezinye zeendlela zokulawula i-RP okwangoku:

• Ukusebenzisa izixhobo zokuncedisa ukubona okuphantsi kunye nezixhobo zokuncedisa: Kukho iintlobo ngeentlobo zeemagnifiers, iiglasi ezikhethekileyo, kwanezixhobo zobuchwepheshe ezinokuchonga into okanye ukuba ngubani into xa uyikhomba.
• Ukuzikhusela elangeni: Kubalulekile ukunxiba iiglasi zelanga nokunciphisa ukuvezwa kukukhanya okuqaqambileyo, kuba ngamanye amaxesha ukukhanya okuqaqambileyo kunokwenza i-RP ibe mandundu.
• Ukunyanga ezinye iimeko ezinxulumene noko: Ukunyanga iimeko ezifana ne-cystoid macular edema (CME) (ukuqokelelwa kolwelo embindini we-retina) olunokwenzeka nge-RP.
• Unyango lwe-cataracts: Abanye abantu abane-RP banokuba ne-cataracts. Ukuba oku kuyenzeka, banokususwa ngotyando.

Masifunde kancinci ngeGene Therapy.

I-FDA yase-US ivumile imveliso yonyango lwezakhi zofuzo ebizwa ngokuba yi-voretigene neparvovec-ryzl (Luxturna®) ukunyanga uhlobo oluthile lwe-RP. Abantu abaneenguqu kuzo zombini iikopi ze-RP65 gene banokungenelwa kolu nyango. Nangona kunjalo, olu hlobo oluthile lwe-RP lufumaneka kuphela kubantu abambalwa.

Kukho uvavanyo oluninzi lwezonyango oluqhubekayo okwangoku malunga nonyango lwezakhi zofuzo kwezinye iintlobo ze-RP kunye ne-IRD, ngoko ke kulindeleke unyango olungakumbi kwixesha elizayo.

Kubantu abane-RP enzima kakhulu, kukho iimeko apho isixhobo esibizwa ngokuba yi-retinal prosthesis sinokuqwalaselwa.

Ngaba ikho indlela yokuthintela uphuhliso lwe-'Retinitis Pigmentosa' (RP)?

Ngenxa yokuba i-Retinitis Pigmentosa idla ngokuba yimfuza, ayinakuthintelwa ngokupheleleyo.

Nangona kunjalo, kukho izinto onokuzenza ukuze ugcine amehlo akho esempilweni kangangoko:

• Bona ugqirha wamehlo rhoqo ukuze uhlolwe amehlo akho. Oku kuya kukunceda ubone naziphi na iingxaki kwangethuba.
• Nxiba iiglasi zelanga kwaye ukhusele amehlo akho ekukhanyeni okuqaqambileyo.
• Landela indlela yokuphila enempilo. Yitya kakuhle kwaye uzilolonge ngokukhuselekileyo.

Yintoni onokuyilindela ukuba une-RP?

I-Retinitis Pigmentosa ayiqhubeki ngendlela efanayo okanye ngesantya esifanayo kumntu wonke. Oku kungenxa yokuba kukho ii-genes ezininzi ezahlukeneyo ezibangela oku. Ngokuxhomekeke kwi-gene, indlela esi sifo esichaphazela ngayo umntu ngamnye yahlukile. Yiyo loo nto kubalulekile ukufumana uvavanyo lwe-genetic kunye nengcebiso ye-genetic.

Buza ugqirha wakho malunga novavanyo lweklinikhi lwangoku, amaqela enkxaso, kunye nezixhobo ezibonakalayo.

Ndifanele ndimbone nini ugqirha?

Njengomgaqo jikelele, bona ugqirha wakho wamehlo rhoqo ngexesha eliqhelekileyo. Kwakhona, ukuba ubonakalisa iimpawu ezintsha, okanye ukuba iimpawu zakho ziya zisiba mandundu, bona ugqirha wakho ngoko nangoko. Umzekelo:

• Ukuba uvakalelwa kukuba umbono wakho uya usiba mbi (ukucaca okanye ukubona imibala).
• Ukuba ufumana intlungu entsha okanye ukungakhululeki emehlweni akho.

Khumbula: Kukho iintlobo ezininzi ze-Retinitis Pigmentosa, kwaye ayizizo zonke iintlobo ezibangela ukulahleka kokubona ngokupheleleyo. Eyona ndlela ilungileyo yokukhusela nokuxhamla kumbono wakho kangangoko kunokwenzeka kukwenza uvavanyo lwamehlo rhoqo kwaye ulandele imiyalelo kagqirha wakho.

Ngamafutshane, khumbula oku! (Umyalezo Wokubuyela Ekhaya)

I-Retinitis Pigmentosa (RP) yimeko enzima ekufuneka ithathwe nzulu. Nangona kunjalo, ukuba uyazi kwaye uthatha amanyathelo afunekayo, ungazinceda uphile ubomi obuqhelekileyo.

• Musa ukoyika, fumana ulwazi: Ukuba ufunyenwe une-RP, yazi kakuhle ngayo. Buza ugqirha wakho imibuzo.
• Ukuhlolwa rhoqo: Hlola amehlo akho rhoqo njengoko kucetyisiwe ngugqirha wakho wamehlo.
• Uvavanyo lwemfuza kunye neengcebiso: Oku kubaluleke kakhulu ukuqonda imeko yakho ngqo.
• Fumana inkxaso: Thetha nosapho kunye nabahlobo ngale nto. Joyina amaqela enkxaso ukuba kuyimfuneko. Awuwedwa.
• Lumka ngonyango olutsha: Isayensi yezonyango ihambela phambili. Buza ugqirha wakho malunga nonyango olutsha kunye novavanyo lweklinikhi.

Zininzi izinto onokuzenza ukuze ukhusele amehlo akho. Eyona nto ibalulekileyo kukwenza into elungileyo ngaphandle kokulahla ithemba.

I- Retinitis Pigmentosa, RP, izifo zamehlo, ukulahleka kokubona, ubumfama basebusuku, izifo zemfuza, i-retina, ukubona