Lɛ wi lan bɔt Leber Hereditary Optic Neuropathy (LHON) insay simpul wɔd dɛn!

Yu tink se yu nɔ de si bɛtɛ smɔl smɔl ɛn ɔltin bigin fɔ blɔk? Sɔntɛm i bigin na wan yay, ɛn afta sɔm mɔnt, di ɔda yay sɛf bi dis kɔndishɔn? Yu go mɔs de wɔri bad bad wan bɔt dɛn tin ya. Tide wi go tɔk bɔt wan sik we nɔ kin bɔku we kin mek dɛn gɛt dɛn sik ya, bɔt dɛn kɔl am Leber Hereditary Optic Neuropathy, ɔ ``(LHON)``.

Wetin na Leber hereditary optik nyuropathy (LHON)?

Fɔ tɔk am simpul wan, Leber hereditary optic neuropathy, ɔ LHON fɔ shɔt, na wan jɛnɛtik kɔndishɔn we de pas frɔm jɛnɛreshɔn to jɛnɛreshɔn. Di men tin we kin apin na we yu nɔ de si fayn igen. We bɔku pipul dɛn kin gɛt am, dɛn kin bigin fɔ si fayn ɛn smɔl smɔl i kin wɔs fɔ lɛk siks mɔnt so. Sɔntɛnde, i kin bigin na wan yay ɛn afta dat i kin afɛkt di ɔda yay sɔm mɔnt afta dat. Bɔku tɛm, i kin bigin we i smɔl ɔ we i yɔŋ. I sɔri fɔ no se bɔku pipul dɛn we gɛt LHON kin blaynd bay lɔ. Dis min se dɛn nɔ de si bɛtɛ te di lɔ se dɛn de si dɛn as blaynd pipul dɛn.

Dɛn kin kɔl am bak di sik we dɛn kɔl Leber. Dat na bikɔs di dɔktɔ we bin stɔdi am fɔs na Dɔktɔ Tiodɔ Liba. Di wɔd "hereditary" min se na sɔntin we yu gɛt . "Optic neuropathy" min se na sik we de afekt yu optik nerve . Yu optik nɛv tan lɛk di kebul we de kɔmɔt na kamɛra to yu bren. di tin dεm we yu de si wit yu yay, dat na di signal dεm we yu de si, na dis optik nεv de kכri to yu bren. Na de di bren de "si." So, if dis optik nerve dεm dεm dεm, dat na wan pan di men we dεm we yu kin lכs yu vishכn.

Difrɛn kayn LHON dɛn de?

Yes, if LHON de men wan afekt yu optik nerves, we min se na di onli simptom we yu de si yu, dεn kכl am di standad LHON kכndishכn. Dis na de kɔndishɔn wae bɔrku pipul dɛm wae gɛt Leber sik kin gɛt.

Bɔt, na smɔl tɛm nɔmɔ , sɔm pipul dɛn kin gɛt ɔda sayn dɛn wit LHON. Dɛn tin ya kin afɛkt ɔda pat dɛn na dɛn nervɔs sistɛm, ɛn sɔm tɛm dɛn kin ivin afɛkt dɛn at. Dɔktɔ dɛn kin kɔl dis sik "Leber plus." Dis kin kɔmplikt smɔl, bikɔs i kin mek ɔda wɛlbɔdi prɔblɛm, nɔto jɔs prɔblɛm wit yu yay.

Aw kɔmɔn tin na LHON?

Dɛn nɔ no ustɛm LHON kɔmɔn. Bɔt dɛn dɔn tɔk se pan ɛvri 25,000 to 50,000 pipul dɛn, wan pan dɛn kin gɛt dis sik. I notis bak se 80% to 90% pan di pipul dɛm wae gɛt LHON na man dɛm .

Wetin na di sayn dɛm fɔ LHON (Leber’s disease)?

`(LHON)` de mek di vishכn lכs de apin we nכ de pen εn sכmtεm (subakutely)., we min se i kin apin smɔl smɔl fɔ sɔm mɔnt. Di fɔs chenj dɛn we yu go notis na yu sɛntral vishɔn . Sɛntral vishɔn na wetin yu de si we yu luk stret bifo - di vishɔn we yu de yuz fɔ drayv, rid buk, ɛn no fes. Yu sɛntral vishɔn kin bigin fɔ luk blɔk, ɔ yu kin gɛt blak spat na di midul pan yu fil fɔ si, lɛk blaynd spat. Dis kin bigin na wan yay ɛn afta dat i kin spre to di ɔda yay.

Dis sik go wɔs insay di nɛks sɔm mɔnt. Yu kin bigin fɔ lɔs yu kɔlɔ vishɔn bak - we min se yu nɔ go si sɔm kɔlɔ ɔ yu nɔ go ebul fɔ difrɛns. di vishכn we de lכs kin stεbyul insay siks mכnt to wan ia afta di fכs simptom dεm bigin. Bay dis tɛm, bɔrku pipul dɛn kin si kin bi 20/200 ɔr wɔs, we na di lɛvɛl we dɛn kin blaynd bay lɔ. Yu kin stil gɛt sɔm layt fɔ no, bɔt yu go gɛt fɔ lan fɔ liv wit lɔw vishɔn. Imajin, i tan lɛk se di wɔl de go blɔk wantɛm wantɛm.

Wetin na di simptom dɛm fɔ "Leber's plus"?

As wi bin dɔn tɔk, pipul dɛm wae gɛt "Leber's plus" kin gɛt difrɛn ɔda sayn dɛm apat frɔm wae dɛn nɔr kin si fayn fayn wan. Sɔm pan dɛn tin ya na:

• Muvmɛnt dizayd , fɔ ɛgzampul trem.
• Bεlεns εn kכdכnayshכn prכblεm dεm (ataxia) . Lɛk fɔ stɛp we yu de waka, ɔ nɔ ebul fɔ ol tin fayn.
• Prɔblɛm fɔ kɔndɔkt di at , lɛk di at bit we nɔ de bit ɔltɛm (ari ritmia).
• Simptom dɛm fɔ multiple sclerosis (MS) , lɛk wae yu mɔsul dɛn wik ɛn yu taya pasmak.

Wetin na di tin dɛm wae kin mek pɔrsin gɛt Leber hereditary optic neuropathy?

Leber in hereditary optic neuropathy na wan sik we de na di maytochondrial sik . na jεnεtik sik we yu kin gεt tru di maytochכndria dεm na yu sεl dεm. Naw yu kin de wɔnda wetin na mitochondria. Fɔ tɔk am simpul wan, di maytochondria dɛn tan lɛk smɔl smɔl pawa jenɛratɔ dɛn we de insay yu sɛl dɛn. Dɛn kin tek ɔksijɛn ɛn tin dɛn we de mek yu bɔdi fayn ɛn mek di ɛnaji we yu sɛl dɛn nid fɔ wok. I tan lɛk jenɛra we de gi pawa to wi os.

so, insay maytochondrial sik dεm, dis prכsεs we de prodyuz enεji de disrupt. Dɔn di sɛl dɛn nɔ kin gɛt di ɛnaji we dɛn nid. Bikɔs ɔf dis, sɔm sɛl dɛn kin stɔp fɔ wok fayn, ɔ ivin day.

we yu maytochכndria nכ de wok fayn, di pat dεm na yu bכdi we de dip bכku pan di maytochכndria enεji na dεn fכs wan dεm fכ fil di ifekt dεm. Di men pat pan dɛn tin ya na di pat dɛn na yu yay, mɔ yu optik nɛv.. if yu gεt inof difεktiv maytochכndria, dεn pat dεm ya nכ de gεt di enεji we dεn nid fכ wok fayn fayn wan. Dis na wetin kin apin pan di sik we dɛn kɔl Leber. di rizulεt na dat yu optik nεv de dכn sכmtεm (`deteriorate`) . Dis na aw yu de lɔs yu yay wit `(LHON)`.

Aw dɛn kin gɛt LHON frɔm dɛn prɔpati?

LHON de kכz bay wan jin mכtεshכn na di DNA na yu maytochכndria. spεshal wan, mכtεshכn dεm na di jin dεm MT-ND1, MT-ND4, MT-ND4L, כ MT-ND6 de mek LHON.

Impɔtant tin na dat, yu kin gɛt ɔl yu maytochondria frɔm yu mama, nɔto frɔm yu papa. So, na mama dεm nכmכ kin pas dis mכtεshכn to dεn pikin dεm. Ivin if di papa gɛt di sik, i nɔ go pas dis muteshɔn to in pikin dɛn.

כltu, nכto כlman we de kכri dis jin mכtεshכn go divεlכp simptom dεm fכ `(LHON)`. So, sɔm pipul dɛn nɔ kin ivin no se dɛn gɛt dis jin we de chenj. Dis na tin we kɔmplikt smɔl, nɔto so? dis min se ivin if di mama gεt dis jin mכtεshכn, di pikin kin gεt di sik כ nכ kin gεt di sik.

Ɛni risk de fɔ mek yu gɛt LHON?

Dis na kwɛstyɔn bak we rili impɔtant. Risach pipul dεm stil nכ gεt klia aidia wae mek sכm pipul dεm we gεt di jin mכtεshכn de divεlכp sכmtεm dεm fכ `(LHON)` εn כda wan dεm nכ de divεlכp.

Bɔt sɔm pruf dɛn sho se di strɛs we pɔsin kin gɛt na in bɔdi ɛn di pɔyzin we de na di say we i de kin mek di sik bigin. dis min se dεn εksternal fכs dεm ya de ad adishכnal strεs to di sistεm dεm we dεn כlrεdi כnda strεs bikoz fכ di jεnεtik mכtεshכn. As tɛm de go, de aidia na dat dɛn strɛs ya kin bɔku ɛn leta kin mek yu gɛt sɔm kayn sik.

Na sɔm tin dɛn we kin mek pɔsin gɛt prɔblɛm:

• Fɔ smok.
• Yuz rɔm.
• Fɔ gɛt pɔyzin we de na di envayrɔmɛnt (fɔ ɛgzampul, sɔm peshɛnt sayd dɛn, industrial kemikal dɛn).
• Fɔ gɛt ɔda sik dɛn we de na di sistɛm.
• Sivɛri saykilɔjik strɛs .

Aw dɛn kin no se pɔsin gɛt LHON?

Yu ay kea spɛshal pɔsin go du sɔm standad ay ɛgzam fɔ chɛk yu yay ɛn fɛn di kɔz fɔ di prɔblɛm. Dɛn nɔr kin ebul fɔ si ɛnitin wae nɔr fayn wit yu yay ɔr yu optik nerve na di fɔs stej, we kin bi insay di fɔs siks mɔnt afta di sayn dɛm bigin. Di damej kin bi mɔr klia afta di fɔs siks mɔnt afta di sayn dɛm bigin.

If yu dɔktɔ sɔspɛkt LHON, i go tɛl yu fɔ du di jenɛtik tɛst . Dis na di onli we fɔ no fɔ tru if yu gɛt wan pan di jɛnɛtik muteshon we wi bin tɔk bɔt. Dis tɛst na di wangren we fɔ kɔnfirm di diagnosis.

Tritmɛnt ɔ kɔmplit mɛrɛsin de fɔ LHON?

I sɔri fɔ no se naw, no mɛrɛsin nɔ de fɔ LHON. Di onli drɔg we di FDA dɔn gri fɔ naw na wan sintetik fɔm fɔ Coenzyme Q10 we dɛn kɔl Idebenone. Randomized controlled trials dɔn sho sɔm improvement in visual acuity in pipul dɛm wae gɛt LHON. Ɔda mɛrɛsin dɛn we tan lɛk dis de bak na di risach pat.

Risach pipul dɛn de stɔdi bak bɔt jin tɛrapi , we dɛn kin yuz as tritmɛnt fɔ LHON tumara bambay. Bɔt dɛn tin ya stil de na di risach stej. So, na dis tɛm, i nɔ kin izi fɔ op fɔ gɛt kɔmplit mɛrɛsin. Bɔt we dɛn de fɔ kɔntrol di sik dɛn ɛn kɔntrol di we aw pɔsin de si tin we go pwɛl mɔ ɛn mɔ te to sɔm mak.

Wetin na di lukin-grɔn wit LHON?

We pɔsin nɔ de si fayn bikɔs ɔf LHON, i kin apin kwik kwik wan, i kin rili bad, ɛn bɔku tɛm i kin de fɔ ɔltɛm . Bɔku pipul dɛn gɛt fɔ lan fɔ liv wit lɔw vishɔn. Lכw vishכn na mכdarεt to siriכs digri fכ si fayn, bכt i nכ min fכ blaynd kכmplit. mכdarεt lכw vishכn na vishכnal akyu tεst we de 20/70. Sivεr lכw vishכn na vishכnal akyu tεst we de 20/200 כ less. Dis na rili wan fayn fayn we aw yu kin si tin we yu kin ebul fɔ si.

If yu de na di mɔdaret ɔ siriɔs say na dis rɛnj , bɔku tɛm na lɔk tin. Di tritmɛnt kin mek difrɛns, bɔt di jin we yu gɛt kin mek difrɛns pas ɔl. Sɔm pipul dɛm wae gɛt LHON kin gɛt sɔm pan dɛn vishɔn bak wae dɛn nɔr bin de ɛkspɛkt afta lɛk wan ia wae dɛn nɔr kin si fayn fayn wan. di chans fכ dis kayn vishכn rεkכvεshכn de difrεn wit difrεn jin mכtεshכn dεm. Bɔt ivin if yu gɛt sɔm vishɔn bak, i go mɔs bi se yu go stil gɛt fɔ liv wit smɔl vishɔn.

I gɛt we fɔ mek dɛn nɔ gɛt LHON?

Pan ɔl we bɔku pipul dɛn we kin gɛt di jin dɛn we gɛt fɔ du wit LHON nɔ kin ɛva gɛt dis sik, naw dɛn nɔ no we fɔ mek dɛn nɔ ebul fɔ si am. If yu tek antiɔksidant ɛn avɔyd nyurotoksin lɛk rɔm ɛn tabak, dat kin mek yu nɔ gɛt bɔku prɔblɛm, bɔt dɛn nɔ dɔn pruv dis.

Mɛmba se ɔl uman dɛn we gɛt dɛn jin ya go pas dɛn to dɛn pikin dɛn. Fɔ advays yu bɔt yu jɛnɛtiks kin ɛp yu fɔ tink bɔt dis prɔblɛm. If pɔsin na yu famili gɛt dis sik, ɔ if yu kam fɔ no se yu gɛt dis jin muteshon, i rili impɔtant fɔ gɛt kɔyl fɔ yu jɛnɛtik bifo yu bigin famili.

If yu no bɔt wan famili histri bɔt LHON, ɔ na sɔntin we yu nɔ bin de ɛkspɛkt fɔ yu, fɔ lɔs yu vishɔn wantɛm wantɛm kin bi sɔntin we de mek yu fred ɛn we de mek yu at pwɛl. Yu ɛn yu dɔktɔ nɔ go ɔndastand wetin de apin fɔs. Bɔt we yu dɔn no bɔt am, yu go gɛt bɔku tin fɔ lan as yu de ajɔst to yu nyu rial layf.

Mɛmba se, nɔto yu wan de pan dis joyn - wɔl de fɔ ɛp yu. Ɔganayzeshɔn, ikwipmɛnt, ɛn mental wɛlbɔdi sɔpɔt de fɔ di wan dɛn we nɔ de si bɛtɛ.

Fɔ dɔn, tin dɛn we wi fɔ mɛmba

Okay, so a op se yu geht sohm aidia boht di `(LHON)` we wi tok boht. Dis na smɔl kɔmplikɛt tɔpik, bɔt i rili impɔtant fɔ no bɔt am.

• (LHON) na wan sik we pɔsin kin gɛt frɔm in mama ɛn papa we kin pwɛl di optik nɛv ɛn mek i nɔ de si fayn igen.
• dis kin bi f כ wan jεnεtik mכtεshכn na di maytochכndrial DNA, we dεn kin gεt frכm mama to pikin .
• Bɔrku tɛm, de sik kin kam wae pɔrsin yɔŋ, ɛn smɔl smɔl pɔrsin nɔr kin si ɛni pen.
• apat frכm di vishכn, כda nεv sεstem prכblεm kin apin na di kכndyushכn we dεn kכl "Leber plus" .
• Tin dɛm lɛk fɔ smok ɛn rɔm kin bi risk fɔ mek yu gɛt di sik.
• Naw, no patikyula mɛrɛsin nɔ de, bɔt mɛrɛsin dɛn de lɛk `Idebenone` ɛn we fɔ adap to lɔw vishɔn.
• If yu gɛt ɛni dawt bɔt dis, fɔ tru, go to dɔktɔ we de mɛn yu yay ɛn du yu jenɛtik tɛst.
• Fɔ liv wit dis sik kin tranga, bɔt nɔr de yu wan, gɛt ɛp.

A op se dis infɔmeshɔn go ɛp yu. If yu gɛt ɛni ɔda kwɛstyɔn bɔt dis, nɔ shek fɔ tɔk to dɔktɔ. Stay wit wɛlbɔdi!

` Leber hεridita optik nyuropati, LHON, vishכn lכs, optik nεv, jεnεtik mכtεshכn, maytochכndrial sik dεm, hεriditi blayndnes