Yu ɔ yu pikin dɔn bigin fɔ notis smɔl chenj na di we aw yu de si tin dɛn stret bifo, we yu nɔ de si fayn? Ɔ sɔntɛm ay dɔktɔ dɔn tɛl yu ɔ sɔmbɔdi na yu famili bɔt dis nem ‘Best disease’? Dis na rili wan sik we nɔ kin apin so ɔltɛm, bɔt we kin afɛkt ɔl tu di yay dɛn. So tide, lɛ wi tɔk simpul wan bɔt wetin na dis Bɛst sik, aw i kin kam, wetin na di sayn dɛm, ɛn if tritmɛnt de.
Wetin na dis Bɛst sik?
Fɔ tɔk am simpul wan, Bɛst in sik na wan sik we de apin to yu yay we de afɛkt di retina na yu yay. Wi yay tan lɛk kamɛra. di rεtina na di layεr we de na di bak pat pan di yay we de εp wi fכ no imej dεm we layt hit am. di pat pan di rεtina we de na di midul pan di rεtina we de εp wi fכ si tin dεm stret bifo dεn kכl am di makula. So, insay Best in sik, di makula kin wik smɔl smɔl. Dis kin mek i nɔ izi fɔ si tin dɛn we de bifo, lɛk lɛta ɛn pipul dɛn fes klia wan. Bɔt bɔrku tɛm , peripheral vision, ɔr si tin dɛm wae de arawnd yu, nɔr kin afɛkt bɔrku.
Dɛn kin kɔl dis Bɛst sik wit ɔda nem. Sɔmtɛm dɔktɔ dɛn kin kɔl am bak `vitelliform macular dystrophy` ɔ `vitelliform dystrophy`. `Dystrophy` na mεdikal tεm fכ di bad bad tin כ we de wik pan wan patikyula כgan.
כda kכndyushכn de lεk dis, we na wan kayn `vitelliform macular dystrophy` bak. כltu, i nכ de stat na pikin, bכt i de divεlכp as yu de ol, כltεm bitwin 40 εn 60. dεn kכl am di `adult-onset type`.
Aw kɔmɔn na Bɛst sik?
E tranga fɔ fɛn di rayt statystik bɔt ɔmɔs pipul dɛn rili gɛt Bɛst in sik. Sɔm pipul nɔr kin ivin no se dɛn gɛt am bikɔs dɛn nɔr kin gɛt sɔm kayn sik. Wan stɔdi sho se na lɛk wan pan ɛvri 16,500 pipul dɛn, ɔ lɛk wan pan ɛvri 21,000 pipul dɛn, kin gɛt dis sik. Wan ɔda stɔdi sho se na lɛk wan pan ɛvri 15,000 pipul dɛn . Wan ɔda wan se i kin bi wan pan ɛvri 10,000 pipul dɛn . Bɔt wi kin ɔndastand se dis na sik we nɔ kin bɔku .
Wetin na di stej dɛm fɔ Bɛst sik?
Dis Bɛst sik kin go tru siks men stej dɛm. Lɛ wi si wetin dɛn bi.
1. Stej I - Prɛvitɛlifɔm stej: .
Dis na di biginin. Bɔrku tɛm, yu nɔr go gɛt ɛni sayn na dis tɛm.Wan yɔlɔ tin nɔ bigin fɔ fɔm yet ɔnda yu retina. Dɛn kin no dis bay we dɛn de du di ay ɛgzam.
2. Stej II - Vitelliform stej: .
di wכd `vitelliform` min ``eg-shaped.`` na dis stej, wan yכlכ sכbstans de bigin fכ kכmכt כnda yu rεtina, na di εria fכ di ``macula``, di sεntrכm fכ yu vishכn. Tink bɔt am lɛk se i tan lɛk smɔl yɔlɔ eg. Yu vishɔn kin stil fayn na dis stej.
3. Stej III - Pseudohypopyon stej: .
na dis stej, di yכlכ sכbstans kin bi fכ fכm εn fכm wan sist כnda di rεtina. Dis tan lɛk smɔl blista we ful-ɔp wit wata insay di yay.
4. Stej IV - Vitelliruptiv stej: .
Na dis say di yɔlɔ tin we bin de gɛda kin bigin fɔ brok ɛn skata. As dis tin de brok dכn, di sεl dεm na di rεtina kin pwεl. Dis tɛm ya, yu kin bigin fɔ notis chenj dɛn na yu yay, lɛk we yu nɔ de si fayn.
5. Stej V - Atrofik stej: .
na dis stej, di yכlכ tin kin kכlכsכl kכlכsכl. Bɔt skata ɛn sɛl dɛn we dɔn pwɛl stil de usay i bin de. Dis damej kin mek pɔsin nɔ ebul fɔ si fayn mɔ ɛn mɔ. Sɔm pipul dɛm wae de stɔdi bɔt dis kin tink se dis na di las stej fɔ Best in sik.
6. Stej VI - Kכroydal niכvaskulεrayzeshn (CNV): .
Sɔm risach pipul dɛn kin tek dis kɔndishɔn we dɛn kɔl `CNV` as di las stej fɔ Bɛst sik. Ɔda pipul dɛn tink se na kɔmplikeshɔn fɔ di sik. Na lɛk 20% pan di pipul dɛm wae gɛt Bɛst sik kin gɛt dis sik. `Neovascularization` na di fכmeshכn fכ nyu bכdi vεsul dεm. `Kכroyd` na di mεmbran bitwin di `rεtina` εn di `sklera` na di yay. insay dis `CNV` kכndishכn, nyu, wik bכdi vεsul dεm de fכm insay di `choroid` layεr. Dɛn nyu blɔd vesel dɛn ya wik so dat dɛn kin lik blɔd ɔ wata. If dis apin, yu vishɔn kin wɔs.
Wetin na de sayn dɛm wae de sho se yu gɛt Bɛst sik?
Bɔrku tɛm, yu go ɔlrɛdi no bɔt Best in sik afta yu dɔn du yu yay ɛgzam, bikɔs sɔmtɛm nɔr kin gɛt ɛni sayn wae yu kin si. Bɔt if di sayn dɛn we de sho se yu gɛt dis sik, dɛn kin gɛt:
- Yu nɔ de si fayn: Tin dɛn we yu de si stret bifo yu kin tan lɛk se dɛn nɔ klia ɔ dɛn nɔ kin si klia wan.
- Prɔblɛm fɔ si tin stret: Di prɔblɛm na fɔ si tin dɛn dairekt bifo yu. Bɔt yu kin gɛt gud ɔl-arawnd, ɔ sayd vishɔn.
- We yu si tin dɛn we de chenj dɛn shep (Metamorphopsia):Imajin, yu de luk wan stret layn, bɔt i tan lɛk layn we de wev ɛn we dɛn drɔ. Yu kin si tin dɛn lɛk domɔt knob ɛn winda frem as dɛn drɔ. Dɛn kɔl dis `mɛtamɔrfɔpsia`.
- Bɔrku say wae pɔrsin nɔr de si fayn: Sɔm pipul kin gɛt bɔrku say wae dɛn nɔr de si fayn fayn wan as tɛm de go.
- Difrɛns pan di we aw di tu yay dɛn de si: Di vishɔn na wan yay kin smɔl pas di ɔda wan. Ɔ di vishɔn na di tu yay nɔ kin dכn ikwal, bכt i kin dכn to difrεn digri dεm.
Wetin na de kɔz fɔ Bɛst sik?
Bɛst in sik na bikɔs ɔf wan sik we de na di bɔdi. Fɔ tɔk am simpul wan, na bikɔs di jin dɛn we wi kin gɛt frɔm wi mama ɛn papa kin chenj.
Best in sik na ``autosomal dominant'' kכndishכn. Dis na smɔl mɛdikal wɔd, bɔt i izi fɔ ɔndastand. ``Autosomal dominant'' min se pikin nכ nid fכ inhεrit di כltεr jin frכm di tu mama εn papa, jכs wan. Dat min se if ɛni wan pan di mama ɛn papa gɛt di jin we dɛn dɔn chenj, dɛn pikin dɛn gɛt lɛk 50% chans fɔ gɛt di sik. I tan lɛk se yu de flip kɔyn ɛn gɛt ed.
I sɔprayz fɔ no se di sem jin we de mek pɔsin gɛt Bɛst in sik kin mek bak sɔm kayn ɔda sik we pɔsin kin gɛt frɔm in yay we dɛn kɔl retinitis pigmentosa. Dis min se di chenj we dis jin de chenj kin mek yu gɛt difrɛn kayn sik dɛn na di yay.
Aw dɛn kin no bɔt Bɛst sik?
Bɔrku tɛm, dɔktɔ go no se yu gɛt Bɛst in sik we yu ol bitwin fayv ɛn tɛn ia, ɔr pasmak we yu ol 20 ia.
We yu go to dɔktɔ we de kia fɔ yu yay fɔ yu ay prɔblɛm, dɛn go aks yu fɔs bɔt yu mɛdikal istri, aks yu if ɛnibɔdi na yu famili gɛt ay prɔblɛm, dɔn dɛn go du yu yay ɛgzam. Apat frɔm dat, dɛn kin du sɔm spɛshal tɛst dɛn bak. Fɔ ɛgzampul:
- Fluorescein angiography: Dis na imej tɛst. Dɛn kin put spɛshal day insay wan vein na yu an ɛn tek pikchɔ dɛn bɔt di blɔd vesel dɛn we de insay yu yay. Dis kin ɛp fɔ si if ɛni lik ɔ abnɔmal tin de na di blɔd vesel dɛn.
- Optical coherence tomography (OCT): Dis na noninvasive imaging test bak. i de yuz layt bim fכ tek kכros-sekshכnal imej dεm fכ di bak pat pan yu yay, spεshal wan di rεtina εn di makula. I tan lɛk se yu de kɔt kek ɛn luk insay. i kin no tin dεm lεk di tik we di layεr dεm na di rεtina tik, εni swel, εn fכ wata we de bכku.
- Kɔlɔ fundus foto: Dis kin tek klia pikchɔ dɛn fɔ yu retina, di blɔd vesel dɛn we gɛt fɔ du wit am, ɛn di optik nɛv ed. Dis kin ɛp yu fɔ si if ɛnitin de we tan lɛk yɔlɔ eg.
- Ophthalmic electrophysiology: Dis na rili sɔm tɛst dɛn we dɛn kin du. Dɛn tɛst ya de mɛzhɔ di kɔrɛnt ilɛktrik aktiviti we kin apin we wi yay de ansa to layt. dis kin mek yu no aw di sεl dεm na di rεtina de wok fayn fayn wan.
- Jɛnɛtik tɛst: Dɛn tɛst ya kin kɔnfɔm wit shɔ if ɛni chenj de na yu jin dɛm we gɛt fɔ du wit Bɛst sik.
Sɔntɛnde, di dɔktɔ kin want fɔ tɔk to ɔda pipul dɛn na yu famili ɔ ivin chɛk dɛn yay, bikɔs dis na sik we pɔsin kin gɛt frɔm in mama ɛn papa.
Aw dɛn kin trit Bɛst sik?
Te tide, no mɛrɛsin nɔ de fɔ di Bɛst in sik. Na dat na di fɔs tin we wi nid fɔ ɔndastand.
Bεst mεnεj de sik bay wae yu de simptom dεm εn di stej wae de sik de. We yu dɔn no se yu gɛt di sik, i rili impɔtant fɔ mek yu de chɛk yu yay ɔltɛm. Na da tɛm de nɔmɔ yu go ebul fɔ no kwik kwik wan ɛni chenj na yu yay ɛn du di tin dɛn we yu nid.
We yu bigin, yu nɔ go nid ɛni tritmɛnt. Bɔt if yu gɛt ɔda mistek dɛn we de mek yu nɔ ebul fɔ si tin, lɛk fɔ si fa, yu go nid fɔ yuz glas. Bɔrku tɛm, pipul dɛm wae gɛt Best’s disease kin gɛt prɔblɛm wit distans vishɔn. Dɔn bak, if yu gɛt katarakt, yu go nid fɔ du ɔpreshɔn pan yu katarakt.
Bɔt if yu gɛt di sik we wi bin dɔn tɔk bɔt trade we dɛn kɔl choroidal neovascularization (CNV), we na fɔ mek nyu wik blɔd vesel dɛn, yu dɔktɔ kin tɛl yu fɔ du tritmɛnt dɛn lɛk dis fɔ stɔp dɛn nyu blɔd vesel dɛn de fɔ gro:
- di anti-vaskulεr εndoteyl growth fכktכ (Anti-VEGF) ejen dεm: Dis na rili mεdikeshכn dεm. Dɛn kin injɛkt dɛn drɔgs ya na di vitreous cavity insay yu yay. Di gol na fɔ stɔp dɛn nyu blɔd vesel dɛn we de lik ɛn mek dɛn smɔl.
- Laser therapy: Dis tritmɛnt de yuz laser bim fɔ sial ɔ pwɛl di abnɔmal blɔd vesel dɛm.
- Fɔtodaynamik tɛrapi (PDT): Dis na tritmɛnt we kɔmpleks smɔl. Na ya, dɛn kin injɛkt wan spɛshal mɛrɛsin na yu bɔdi, dɔn dɛn kin mek di mɛrɛsin mek layt sɛnsitiv, ɛn dɛn kin yuz lɛsa bim pan di blɔd vesel dɛn we di sik afɛkt, ɛn i kin pwɛl dɛn ɛn mek dɛn nɔ lik blɔd.
Apat frɔm dat, yu dɔktɔ kin tɛl yu fɔ yuz tin dɛn we de ɛp yu we yu nɔ de si bɛtɛ, lɛk tin dɛn we de mek yu si di tin dɛn we yu de si, spɛshal layt, ɛn sɔftwia we izi fɔ rid.
Risach pipul dɛn dɔn ɔlrɛdi de chɛk if dɛn kin yuz jɛnɛtik matirial fɔ trit ɔ fɔ mek i nɔ gɛt dis sik. Di jin tɛrapi stil de na di risach stej, bɔt dɛn op se i go gi fayn fayn rizɔlt tumara bambay.
Yu tink se dɛn kin ridyus di risk fɔ gɛt Bɛst sik?
Fɔ tɔk tru, nɔrtin nɔr de wae yu kin du fɔ mek yu nɔr gɛt Best in sik. Na wan jenɛtik kɔndishɔn. Bɔt if pɔsin na yu famili gɛt Bɛst sik, ɔ if yu kam fɔ no se yu gɛt am, i go fayn fɔ mek yu gɛt kɔyl fɔ yu jɛnɛtiks bifo yu bɔn pikin. Wan pɔsin we de gi advays bɔt yu jɛnɛtiks kin luk yu famili histri ɛn ɛksplen aw yu pikin dɛn kin gɛt di sik.
Wetin kin apin if a gɛt Bɛst sik?
Best in sik nɔto sik we de kil pɔsin. Dat min se i nɔ de mek pɔsin in layf de pan denja. Bɔt, lɛk aw wi bin dɔn tɔk, i nɔ kin mɛn. Yu nɔ go blaynd ɔltogɛda frɔm Bɛst in sik. Bɔt sɔntɛm yu nɔ kin si bɛtɛ. Dat min se i kin tranga smɔl fɔ du ɛvride wok, bɔt yu nɔ go lɔs yu yay kpatakpata.
Aw a kin kia fɔ misɛf?
Sɔm it dɛn de we fayn fɔ mek yu yay gɛt wɛlbɔdi.
"Gud it rili impɔtant fɔ di yay."
Fɔ ɛgzampul, fɔ it fish fɔ sɔm dez insay di wik, ɛn fɔ ad grɛn vɛjitebul, frut, ɛn nɛt to yu it, i fayn fɔ yu yay. dis dεm de gi nyutriεnt dεm we fayn fכ di yay lεk omega-3 fεt asid εn vaytam in dεm.
If yu gɛt Bɛst in sik, i impɔtant fɔ mek yu de chɛk yu yay ɔltɛm, mɔ fɔ chɛk yu yay. If yu notis ɛni chenj na yu yay ɔ yu jenɛral wɛlbɔdi, tɛl yu dɔktɔ wantɛm wantɛm. Yu kin aks yu dɔktɔ kwɛstyɔn dɛn bak lɛk:
- " Yu kin rεkomεnd wan jεnεtik kכnsεlכ fכ εp mi fכ disayd if a fכ bכn pikin?"
- "A fit fɔ tek pat pan klinik trial fɔ dis sik?"
- " Yu kin rekomend optometrist we spɛshal pan low vision?"
- " Na jin tɛrapi na tritmɛnt opshɔn fɔ mi?"
Wetin na di difrɛns bitwin Bɛst sik ɛn Stargardt sik?
Stargardt sik ɛn Bɛst sik na ɔl tu jɛnɛtik sik dɛm, ɛn dɛn ɔl tu kin afɛkt di sɛntrɔm fɔ si na di yay (di makula). Dis min se di we aw pɔsin de si stret, i kin afɛkt am.
Bɔt dɛn tu sik ya kin kam bikɔs difrɛn jin dɛn kin chenj.
Insay Stargardt sik, yu kin si yɔlɔ ɔ wayt fleks na yu retina. bכt insay Bεst sik, yu kin si wan big, yכlכ, lεk eg-yכlk lεk oval-shεp sist כnda di makula. So, dεn kin no dεn tu sik ya bay dεn simptom dεm ya εn jεnεtik tεst.
Fɔ liv wit wan sik wae de mek pɔrsin nɔr de si fayn kin tranga. Bɔt if yu gɛt Bɛst in sik, bɔrku tin ɛn savis de wae kin ɛp fɔ mek tin izi. Aks yu dɔktɔ bɔt dɛn.
Fɔ dɔn, tin dɛn we wi fɔ mɛmba
Okay, so wi don tok plenti boht Best disease. Mek wi rikap:
- Bεst in sik na wan kכndyushכn we dεn kin gεt we de afekt di sεntrכm fכ si na di yay (di makula). Dis kin mek i nɔ ebul fɔ si fayn.
- Pan ɔl we dɛn nɔ go ebul fɔ mɛn am kpatakpata, i nɔ de put in layf pan denja. I kin apin we pɔsin nɔ de si fayn, bɔt i nɔ kin blaynd ɔl.
- I rili impɔtant fɔ no di sik kwik kwik wan ɛn du mɛrɛsin tɛst ɔltɛm.
- Tritmɛnt de fɔ kɔmplikeshɔn lɛk `CNV`.
- Jɛnɛtik advays na sɔntin we kin impɔtant fɔ di wan dɛn we de plan fɔ mek famili.
- If yu it tin dɛn we fayn fɔ di yay ɛn yuz tin dɛn we go ɛp yu fɔ si fayn, dat kin mek yu layf izi.
Mɛmba se nɔto yu wangren de. Dɔktɔ, advays, ɛn tɛknik dɛn de we go ɛp yu fɔ liv wit dɛn sik ya. Di tin we impɔtant pas ɔl na fɔ kɔntinyu fɔ strɔng ɛn fala di instrɔkshɔn dɛn we yu nid. If yu gɛt ɛni kwɛstyɔn ɔ dawt, tɔk to yu dɔktɔ opin wan.
` Bεst sik, Bεst sik, yay sik, vishכnal sεnta, makula, jεnεtik sik, vishכn lכs, ay ɛgzam











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