Ingabe nawe unenkinga yokubona ebusuku? Ingabe ukubona kwakho kuya ngokuya kubohloka? Ake sifunde nge-'Retinitis Pigmentosa' (RP)!

Ngezinye izikhathi uba nobunzima bokushayela ebusuku noma ezimweni zokukhanya okuphansi, akunjalo? Noma, wake washayisa umuntu ngaphandle kokumbona eza ngakuye? Uma uke wabhekana nalokhu, imbangela kungaba yisimo samehlo esibizwa ngokuthi i-'Retinitis Pigmentosa' (RP). Ungakhathazeki, lesi akusona isimo esivamile, kodwa kubalulekile ukuqaphela. Ake sixoxe ngakho ngokuningiliziwe.

Kuyini i-'Retinitis Pigmentosa' (RP)? Kungani ibaluleke kangaka?

Kalula nje, igama elithi 'Retinitis Pigmentosa' (RP) liyigama elijwayelekile leqembu lezifo zamehlo ezingadluliselwa ezizukulwaneni ngezizukulwane. Lithinta kakhulu i-'retina' engaphakathi kweso lakho.

Cabanga ngeso lakho njengekhamera yefilimu yakudala. Isithombe esikulawo makhamera sithathwe efilimini. Kunjalo-ke, ngaphakathi kweso lakho, ngemuva, kukhona ulwelwesi oluthambile kakhulu noluzwela ukukhanya olubizwa ngokuthi i-retina. Lapho ukukhanya okuvela ezintweni esizibonayo kuwela e-retina, kuguqula lokho kukhanya kube izimpawu zikagesi. Bese lezo zimpawu ziya ebuchosheni bese sibona, 'O, lokhu yilokho, lokhu yilokho.'

Ngakho-ke, uma i-retina ingasebenzi kahle, kufana nokuthi ifilimu ekhamera yonakele. Kungakhathaliseki ukuthi ugxila kangakanani, isithombe ngeke siphume sicacile. Ngokufanayo, uma une-retina egulayo, noma ngabe ugqoka izibuko noma ama-contact lens, kuzothinta umbono wakho.

I-retina iqukethe izinhlobo ezikhethekile zamaseli ezinzwa asabela ekukhanyeni. Lawa abizwa ngokuthi amaseli e-photoreceptor. Kunezinhlobo ezimbili zalawa: izinduku nama-cone. Izinduku zisisiza sibone ekukhanyeni okuphansi, ikakhulukazi ebusuku. Ama-cone asisiza sibone imibala nemininingwane emincane ngokucacile. Kanye nalawa maseli, kukhona olunye uhlobo lweseli ku-retina olubizwa ngokuthi amaseli e-retinal pigment epithelium. Wonke lawa maseli asebenza ndawonye futhi ndawonye ukuze asinike umbono omuhle.

I-Retinitis Pigmentosa (RP) kanye nezinye izifo ze-retina ezizuzwe njengefa (ama-IRD) zibangelwa izinguquko ezakhiweni zofuzo ezenza la maseli asebenze kahle, okubizwa ngokuthi ukuguqulwa kwezakhi zofuzo . Lokhu kubangela ukuthi amaseli abe buthaka kancane kancane futhi ayeke ukusebenza.

I-RP akuyona isifo esisodwa. Ngenxa yokuthi iyiqembu lezifo, ukubona kungahluka kumuntu nomuntu. Abantu abaningi baqala ukubona okuphansi , kanti abanye abantu bangase balahlekelwe umbono wabo ngokuphelele. Lezi zinguquko zokubona zivame ukuqala ebuntwaneni. Kodwa ngezinye izikhathi lezi zinguquko zenzeka kancane kangangokuthi ungase ungaziboni. Kwabanye abantu, ukulahlekelwa umbono kwenzeka ngokushesha. Kwezinye izinhlobo ze-RP, ukulahlekelwa umbono kuyama ezingeni elithile.

Okubaluleke kakhulu, i-Retinitis Pigmentosa ivame ukuthinta amehlo womabili .

Ziyini izimpawu ze-RP? Kukuthinta kanjani?

Izimpawu ze-'Retinitis Pigmentosa' aziveli ngokuzumayo. Zivela kancane kancane. Nazi ezinye zezimpawu zokuqala:

• Izinkinga Zokubona Ebusuku: Ungase uthole ukuthi unenkinga yokubona izinto ebusuku, ikakhulukazi uma ukukhanya kuphansi. Lokhu kungaba nzima uma uhamba emgwaqweni ebusuku noma ngaphakathi endlini ekukhanyeni okuncane.
• Izinkinga zokubona ekukhanyeni okufiphele: Kungaba nzima ukubona izinto ngokucacile, hhayi nje ebusuku, kodwa nasezindaweni ezifana negumbi elimnyama kancane noma ibhayisikobho.
• Izindawo ezingaboni emehlweni: Ngisho noma ungabona okubukayo, ungase ungakwazi ukubona okukuzungezile, njengemoto evela eceleni noma umuntu osondelayo. Yingakho abanye abantu beshayisa izinto ngengozi.

Ngokuhamba kwesikhathi, ezinye izimpawu zingase zivele. Lezi zimpawu zifaka:

• Umuzwa wokukhanya okukhanyayo noma okukhanyayo: Abanye abantu bangase babe nokukhanya okukhanyayo noma umuzwa wokushaywa umbani.
• Umbono womhubhe (Ukuba nombono ophakathi kuphela): Lokhu kufana nokubuka umhlaba ngepayipi. Ungabona kuphela okuqondile phambili, futhi ungaboni lutho olukuzungezile.
• I-Photophobia - Ukuzwela noma ukungakhululeki ekukhanyeni okukhanyayo: Amehlo aba luhlaza okwesibhakabhaka futhi kuba nzima ukubona uma evezwa elangeni noma ekukhanyeni okukhanyayo.
• Ukulahlekelwa ikhono lokubona umbala: Imibala ingase ingabonakali ikhanya noma ingabonakali njengoba yayinjalo ngaphambili.
• Ukuba nombono ophansi kakhulu: Ezimweni ezimbi kakhulu, umbono ungancipha kakhulu.

Okubalulekile: Uma unesinye noma ngaphezulu salezi zimpawu, kungcono ukubona udokotela wamehlo ngokushesha okukhulu.

Kungani i-'Retinitis Pigmentosa' ikhula? Iyini imbangela yalokhu?

Imbangela eyinhloko ye-`Retinitis Pigmentosa` (RP) kanye nezinye 'Izifo Ze-Retinal Ezizuzwe Njengefa (IRDs)` izinguquko ezithile '(ukuguqulwa kwezakhi zofuzo)' ezizakhini zethu zofuzo . Lezi zakhi zofuzo yizo ezikhiqiza amaseli ku-`(retina)` yethu futhi ziwenze asebenze kahle. Ngakho-ke, uma kukhona iphutha noma ushintsho kulezi zakhi zofuzo, lawo maseli awakwazi ukusebenza kahle. Ngokuhamba kwesikhathi, lawo maseli ayafa kancane kancane. Yilapho ukubona kuncipha kancane kancane.

Ngenxa yokuthi kuyifa, izingane zingazuza lezi zinguquko zofuzo kubazali bazo. Kodwa akusho ngaso sonke isikhathi ukuthi uzoba nalesi simo ngoba nje othile emndenini wakho enaso. Kungenzeka futhi ukuthi ube nalesi simo ngaphandle kokuba othile emndenini wakho abe naso. Yingakho ukuhlolwa kofuzo kubalulekile.

Lesi simo sisakazeke kangakanani emhlabeni?

Ngokwezibalo eYurophu naseMelika, umuntu oyedwa kwabangu-3,500 kuya ku-4,000 angaba ne-'Retinitis Pigmentosa'. Emhlabeni wonke, kulinganiselwa ukuthi abantu ababalelwa ezigidini ezimbili bahlushwa yilesi simo. Kukhona nabantu abanalesi simo eSri Lanka. Ngakho-ke, kubalulekile ukuqaphela lokhu.

Odokotela bayixilonga kanjani i-Retinitis Pigmentosa (RP)?

Uma usola ukuthi une-RP, udokotela wakho uzokwenza izivivinyo eziningana ezahlukene ukuze akuhlole. Kubaluleke kakhulu ukuhlolwa kwamehlo njalo.

Ukuhlolwa kwamehlo okuvulekile ngokuhlolwa kwensimu ebonakalayo

Kulokhu, udokotela wamehlo noma udokotela wamehlo wenza okulandelayo:

• Bakutshela ukuthi ufunde umbhalo osodongeni.
• Ikutshela ukuthi ubheke into ngamehlo womabili.
• Ukucindezela emehlweni akho kuyalinganiswa.
• Ukuhlolwa kwensimu ebonakalayo kuhlola umbono wakho ongaphandle.
• Ukubuka indlela inhlamvu yeso lakho esabela ngayo ekukhanyeni.
• Iso liyavuleka ngokufaka amaconsi esosweni. Lokhu kuvumela i-retina ukuthi ihlolwe eduze.
• Ungathatha nezithombe ze-retina.

Ukuhlolwa kwe-Electroretinography (ERG)

Lolu vivinyo olukhethekile. Lulinganisa ukuthi i-retina yakho isabela kanjani ekukhanyeni. Lulinganisa ukuthi amangqamuzana ahlukene ku-retina yakho (izinduku nama-cone esikhulume ngawo) asebenza kahle kangakanani. Luhilela ukukhanyisa izibani ezahlukene phambi kwamehlo akho. Luyingxenye yeqembu lezivivinyo ezibizwa ngokuthi izivivinyo ze-ophthalmic electrophysiology. Lezi zivivinyo zibheka nendlela amehlo akho nobuchopho bakho okucubungula ngayo lokho okubonayo.

Ukuskena kwe-Optical Coherence Tomography (OCT)

Lokhu ukuhlolwa okungahlaseli. Lokhu kuskena kwe-OCT kungalinganisa ubukhulu be-retina yakho futhi kuhlaziye impilo yezendlalelo zayo. Okudingeka ukwenze nje ukubheka ithagethi, bese ikhamera ekhethekile ithatha izithombe zengaphakathi leso lakho.

Ukuhlolwa kwe-`Fundus Autofluorescence (FAF)`

Lokhu futhi "ukuhlolwa kwezithombe" okungahlaseli okuthatha izithombe zeso. Kunganikeza ulwazi oluningi mayelana nempilo ye-retina. Kusetshenziselwa ukuxilongwa, ukwelashwa, kanye nokuqapha lesi sifo.

Kanye nalezi zivivinyo, udokotela wakho angase futhi ancome ukuhlolwa kwezakhi zofuzo kanye nomeluleki wezakhi zofuzo.Ungaphakamisa nokuvakasha. Ngoba uma uthola ukuthi yikuphi ngempela ukuguquka kwezakhi zofuzo okubangela i-RP, ungathola umbono wokuthi lesi sifo sizoziphatha kanjani esikhathini esizayo nokuthi siyawathinta yini amanye amalungu omndeni. Kubalulekile futhi ukuthola ezinye izindlela zokwelapha ezintsha, isibonelo, ukwelashwa kwezakhi zofuzo, noma ukuhlanganyela ezivivinyweni zemitholampilo ezihlobene.

Yiziphi izindlela zokwelapha lesi simo esithi `Retinitis Pigmentosa` (RP)? Siphathwa kanjani?

Eqinisweni, kube nentuthuko enkulu ekwelapheni i-Retinitis Pigmentosa (RP) kanye nezinye izifo ze-IRD eminyakeni yamuva nje, ikakhulukazi ngesithembiso sokwelashwa okusha njengokwelashwa ngezakhi zofuzo.

Nazi ezinye zezindlela zokuphatha i-RP njengamanje:

• Ukusebenzisa izinsiza-kusiza ukubona kahle kanye namadivayisi okusiza: Kunezinhlobo ezahlukene zezikhulisi, izibuko ezikhethekile, ngisho namadivayisi obuchwepheshe angabona ukuthi yini noma ubani into ethile uma uyikhomba.
• Ukuvikelwa elangeni: Kubalulekile ukugqoka izibuko zelanga nokunciphisa ukuchayeka ekukhanyeni okukhanyayo, njengoba ngezinye izikhathi ukukhanya okukhanyayo kungenza i-RP ibe yimbi kakhulu.
• Ukwelapha ezinye izimo ezihlobene: Ukwelapha izimo ezifana ne-cystoid macular edema (CME) (ukunqwabelana koketshezi phakathi kwe-retina) okungenzeka nge-RP.
• Ukwelashwa kwe-cataracts: Abanye abantu abane-RP bangase babe ne-cataracts. Uma lokhu kwenzeka, bangasuswa ngokuhlinzwa.

Ake sifunde kancane ngokwelashwa nge-Gene.

I-US FDA ivumile umkhiqizo wokwelapha izakhi zofuzo obizwa ngokuthi i-voretigene neparvovec-ryzl (Luxturna®) ukwelapha uhlobo oluthile lwe-RP. Abantu abanezinguquko kuzo zombili izinguqulo zezakhi zofuzo ze-RP65 bangazuza kulokhu kwelashwa. Kodwa-ke, lolu hlobo oluthile lwe-RP lutholakala kuphela enanini elilinganiselwe labantu.

Kusenezivivinyo eziningana zemitholampilo okwamanje eziqhubekayo zokwelapha izakhi zofuzo kwezinye izinhlobo ze-RP kanye ne-IRD, ngakho-ke kulindeleke ukwelashwa okwengeziwe esikhathini esizayo.

Kubantu abane-RP enzima kakhulu, kunezimo lapho kungacatshangwa khona idivayisi ebizwa ngokuthi i-retinal prosthesis .

Ingabe ikhona indlela yokuvimbela ukuthuthukiswa kwe-'Retinitis Pigmentosa' (RP)?

Ngenxa yokuthi i-Retinitis Pigmentosa ivame ufuzo, ayinakuvinjelwa ngokuphelele.

Nokho, kunezinto ongazenza ukuze ugcine amehlo akho ephilile ngangokunokwenzeka:

• Bonana nodokotela wamehlo njalo ukuze uhlolwe amehlo akho. Lokhu kuzokusiza ukuthi ubone noma yiziphi izinkinga kusenesikhathi.
• Gqoka izibuko zelanga futhi uvikele amehlo akho ekukhanyeni okukhulu.
• Landela indlela yokuphila enempilo. Yidla kahle futhi uvivinye umzimba ngokuphephile.

Yini ongayilindela uma une-RP?

I-Retinitis Pigmentosa ayithuthuki ngendlela efanayo noma ngesivinini esifanayo kuwo wonke umuntu. Lokhu kungenxa yokuthi kunezakhi zofuzo eziningi ezahlukene ezibangela lokhu. Kuye ngezakhi zofuzo, indlela lesi sifo esithinta ngayo umuntu ngamunye ihlukile. Yingakho kubalulekile ukuthola ukuhlolwa kwezakhi zofuzo kanye nokwelulekwa kwezakhi zofuzo.

Buza udokotela wakho mayelana nezivivinyo zezokwelapha zamanje, amaqembu okusekelana, kanye nezinsiza ezibonakalayo.

Kufanele ngimbone nini udokotela?

Njengomthetho ojwayelekile, bona udokotela wakho wamehlo njalo ngesimiso esijwayelekile. Futhi, uma uba nezimpawu ezintsha, noma uma izimpawu zakho ziba zimbi kakhulu, bona udokotela wakho ngokushesha. Isibonelo:

• Uma unomuzwa wokuthi umbono wakho uya uba mubi (ukucaca noma ukubona imibala).
• Uma uzwa ubuhlungu obusha noma ukungakhululeki emehlweni akho.

Khumbula: Kunezinhlobo eziningi ze-Retinitis Pigmentosa, futhi akuzona zonke izinhlobo ezibangela ukulahlekelwa umbono ngokuphelele. Indlela engcono kakhulu yokuvikela nokuzuza ekuboneni kwakho ngangokunokwenzeka ukuba nokuhlolwa kwamehlo njalo nokulandela imiyalelo kadokotela wakho.

Ngamafuphi, khumbula lokhu! (Umyalezo Wokuya Ekhaya)

I-Retinitis Pigmentosa (RP) yisimo esibucayi esidinga ukuthathwa ngokungathi sína. Kodwa-ke, uma uqaphela futhi uthatha izinyathelo ezidingekayo, ungazisiza ukuthi uphile impilo evamile.

• Ungesabi, thola ulwazi: Uma kutholakale ukuthi une-RP, yazi kahle ngayo. Buza udokotela wakho imibuzo.
• Ukuhlolwa njalo: Hlola amehlo akho ngezikhathi ezithile njengoba kunconywe udokotela wakho wamehlo.
• Ukuhlolwa kwezakhi zofuzo kanye nokwelulekwa: Lokhu kubaluleke kakhulu ukuqonda isimo sakho esiqondile.
• Thola ukwesekwa: Khuluma nomndeni nabangane ngalokhu. Joyina amaqembu okusekelana uma kudingeka. Awuwedwa.
• Qaphela izindlela zokwelapha ezintsha: Isayensi yezokwelapha iyaqhubeka. Buza udokotela wakho mayelana nezindlela zokwelapha ezintsha kanye nezivivinyo zezokwelapha.

Kunezinto eziningi ongazenza ukuze uvikele amehlo akho. Into ebaluleke kakhulu ukwenza into efanele ngaphandle kokulahla ithemba.

I- Retinitis Pigmentosa, RP, izifo zamehlo, ukulahlekelwa umbono, ubumpumputhe basebusuku, izifo zofuzo, i-retina, ukubona