Vawiin hian Best disease, mit hmuhna tichhe thei chungchang kan sawi dawn!

Nang emaw, i fa emaw chuan thil i hmuh dan dik takah inthlak danglamna tlem te, mit hmuh lohna (blurring) i hmu tan tawh em? A nih loh leh mit doctor-in nangmah emaw, i chhungte tu emaw hnenah he hming ‘Best disease’ tih hi a hrilh tawh mai thei em? Hei hi a takah chuan thil awm lo tak a ni a, mahse mit pahnih a nghawng thei genetic condition a ni. Chuvangin vawiin hian he Best disease hi eng nge a nih, engtin nge a lo awm tih, a symptoms te, a enkawl dan tur a awm em tih te hi thu awlsam takin sawi ila.

He natna tha ber hi eng nge ni?

A awlsam zawngin sawi ila, Best natna hi genetic condition a ni a, i mit retina a nghawng a ni. Kan mit chu camera ang mai a ni. Retina hi mit hnung lama layer awm, êngin a rawn tlak chuan thlalak kan hriat theih nan min pui a ni. Retina lai takah thil kan hmuh theihna tura min puitu chu macula an ti a. Chuvangin, Best-a natnaah chuan macula chu a chak lo zauh zauh a. Hei hian hmalam thil, lehkha leh mi hmêl ang chi, chiang taka hmuh a harsat phah thei a ni. Mahse a tam zawkah chuan , peripheral vision, emaw i chhehvel thil hmuh hian a nghawng vak lo.

He Best natna hi hming dangin an ko thin. A châng chuan doctor-te pawhin `vitelliform macular dystrophy` emaw `vitelliform dystrophy` emaw an ti thei bawk. `Dystrophy` tih hi damdawi lam tawngkam a ni a, taksa peng pakhat a chhe emaw, a chak lo emaw tihna a ni.

Hetiang dinhmun dang pawh a awm a, chu chu `vitelliform macular dystrophy` chi khat a ni bawk. Mahse, naupan lai atanga intan ni lovin, i upat deuh deuh chuan a lo thang lian zawk a, a tlangpuiin kum 40 leh 60 inkar a ni a, `adult-onset type` an ti a ni.

Best natna hi engtiang chiahin nge a hluar?

Best natna vei tak tak mi engzat nge awm tih statistics dik tak hmuh a harsa hle. Mi thenkhat chuan symptom an neih loh avangin an nei tih pawh an hre lo. Zirchianna pakhat chuan mi 16,500 zînga pakhat vêl, a nih loh leh mi 21,000 zînga pakhat vêl chuan a nei thei tih a sawi. Survey dang pakhat chuan mi 15,000 zinga pakhat vel chuan . Mi dang chuan mi 10,000 zinga pakhat a ni thei tih a sawi bawk . Mahse, hei hi natna tlemte chauh a ni tih kan hrethiam thei .

Best natna hi eng stage nge ni?

He Best natna hian stage lian paruk a paltlang thei a ni. Eng nge an nih i en ang u.

1. Stage I - Previtelliform stage-a awm tur chu:

Hei hi a bul tanna tak a ni. A tam zawkah chuan tun dinhmunah hian symptoms i nei lovang.I retina hnuaiah hian thil sen (yellow substance) a la awm tan lo. Hei hi mit enfiahnaah a thleng mai thei.

2. Stage II - Vitelliform stage: 1. A rilru a hah lutuk chuan a rilru a buai em em a.

`Vitelliform` tih thumal hi ``egg ang maia awm'' tihna a ni . Egg sen te tak te ang maia lang angin ngaihtuah rawh. Hetiang dinhmunah hian i mit a la tha mai thei.

3. Stage III - Pseudohypopyon stage: 1. A rilru a hah lutuk chuan a rilru a buai em em a.

Hetiang dinhmunah hian thil sen chu tuiah a chang thei a, retina hnuaiah cyst a siam thei a ni. Hei hi mit chhunga tuiin a khat blister te tak te ang a ni.

4. Stage IV - Vitelliruptive stage: 1. A rilru a hah lutuk chuan a rilru a buai em em a.

Hei hi a hmaa thil sen (yellow material) lo awm khawm thin chu a chhe tan a, a darh tanna hmun a ni. He thil hi a chhe zel chuan retina chhunga cell te chu a chhe thei a ni. Hetih lai hian i mit hmuhna lama inthlâk danglamna, mit hmuh lohna (blurred vision) ang chi chu i hmu ṭan thei a ni.

5. Stage V - Atrophic stage: 1. A rilru a hah lutuk chuan a rilru a buai em em a.

Hetiang dinhmunah hian yellow substance chu a bo vek tawh mai. Mahse a awmna hmunah chuan hliam leh cell chhia te chu a la awm reng a ni. He chhiatna hian mit hmuhna a tichhe zual thei a ni. Zirchiangtu thenkhat chuan hei hi Best-a natna hnuhnung ber niin an ngai.

6. Stage VI - Choroidal neovascularization (CNV) atanga lo chhuak a ni.

Researcher thenkhat chuan he natna `CNV` an tih hi Best disease stage hnuhnung ber niin an ngai. Midangte chuan he natna avanga harsatna thlentu niin an ngai. Best natna vei zinga 20% velin he natna hi an nei thei a ni. `Neovascularization` tih hi thisen kalna kawng thar siam a ni. `Choroid` chu mit `retina` leh `sclera` inkara membrane a ni. Hetiang `CNV` dinhmunah hian `choroid` layer-ah hian thisen kalna kawng thar, chak lo tak tak a lo awm a. Heng thisen kalna kawng tharte hi a chak lo em em a, thisen emaw tui emaw a leak thei a ni. Chutiang a nih chuan i mit a chhe zual thei a ni.

Best natna lanchhuahna chu engte nge ni?

A tam zawkah chuan Best natna hi mit enfiah hnuah chauh i hre thei ang, a chhan chu a chang chuan a lan chhuah dan hmuh tur a awm lo. Mahse, symptoms a lo lang a nih chuan, chungte chu:

• Mit hmuhna (blurred vision): Hmalam lama thil hmuh dik tak chu a chiang lo emaw, a fiah lo emaw angin a lang thei.
• Straight vision lama harsatna: Buaina chu i hmaa thil hmuh nghal hi a ni. Mahse all-around, emaw side vision tha tak i nei mai thei.
• Thil awm dan inthlak danglam hmuh (Metamorphopsia):Ngaihtuah teh, line dinglam i en a, mahse chu chu line wavy, drawn angin a lang che a ni. Kawngkhar khar leh window frame ang chi thilte chu lemziak angin i hmu mai thei. Hei hi `metamorphopsia` an ti a.
• Mitdelna nasa tak: Mi thenkhat chuan hun kal zelah mitdelna nasa tak an nei thei.
• Mit pahnih hmuh dan inang lo: Mit pakhata mit hmuhna chu a dang aiin a tlem thei. A nih loh leh mit pahniha mit hmuhna chu a tlahniam lo thei a, mahse degree hrang hrangin a tlahniam thei bawk.

Best natna hi eng nge a chhan?

Best natna hi genetic condition avanga lo awm a ni. A awlsam zawngin sawi ila, kan nu leh pate hnen atanga kan rochun genes inthlak danglamna avanga lo awm a ni.

Best natna hi ``autosomal dominant'' natna a ni. Hei hi medical term deuh a ni a, mahse hriatthiam a awlsam hle. ``Autosomal dominant'' tih awmzia chu naupang chuan nu leh pa pahnih atanga gene danglam chu a rochun a ngai lo va, pakhat chauh a ni. Chumi awmzia chu nu leh pa tu pawhin gene danglam tak an neih chuan an fate chuan natna vei theihna chance 50% vel an nei tihna a ni. Coin flipping a, lu lak ang mai a ni.

Mak tak maiin Best-a natna thlentu gene tho hian mit natna dang rochun retinitis pigmentosa tih chu chi hrang hrang a thlen bawk. Chumi awmzia chu he gene-a danglamna hian mit natna chi hrang hrang a thlen thei tihna a ni.

Engtin nge Best natna hi hmuhchhuah a nih?

A tam zawkah chuan doctor chuan Best natna hi kum nga leh sawm inkar i nih laiin a hmuchhuak ang a, a tam berah kum 20 i tlin hunah a hmuchhuak ang.

Mit lama harsatna neia mit enkawltu specialist i pan chuan i damdawi lam chanchin an zawt hmasa ang che a, i chhungte zingah mit lama harsatna nei an awm leh awm loh an zawt ang che a, chutah chuan mit enfiahna an nei leh ang. Chu bakah, specialized test thenkhat pawh an ti thei bawk. Entir nan:

• Fluorescein angiography: Hei hi imaging test a ni. I kut zungtang pakhatah dye bik inject a, i mit chhunga thisen kalna kawngte thlalak lak a ni. Hei hian thisen kalna kawngah leak emaw, thil danglam emaw a awm leh awm loh hriat nan a pui thei a ni.
• Optical coherence tomography (OCT): Hei hi noninvasive imaging test a ni bawk. Light beam hmangin i mit hnunglam, a bik takin retina leh macula te cross-sectional image a la thin. Cake cut a, a chhung lam en ang mai a ni. Retina layer thickness, eng swelling, leh fluid buildup te ang chi thil a hmu thei.
• Color fundus photography: Hei hian i retina, a kaihhnawih thisen kalna kawng leh mit nerve lu te chiang takin a la thei a ni. Hei hian yellow egg ang chi deposit a awm leh awm loh a hriat theih nan a pui thei che a ni.
• Ophthalmic electrophysiology: Hei hi a takah chuan test hrang hrang a ni. Heng test te hian kan mit hian êng a dawn hian electrical activity fiah lo tak tak a awm tih a teh a ni. Hei hian retina chhunga cell te hnathawh that dan tur ngaihtuahna a pe thei a ni.
• Genetic tests: Heng test te hian i genes ah Best disease nena inzawm danglamna a awm leh awm loh chu chiang takin a nemnghet thei a ni.

A châng chuan doctor chuan i chhûngkaw member dangte biak emaw, an mit pawh enfiah emaw a duh thei a, hei hi thlahtute aṭanga lo chhuak a nih avângin.

Best natna hi engtin nge enkawl a nih?

Vawiin thleng hian Best-a natna hi damdawi a la awm lo. Chu chu kan hriatthiam hmasak ber tur a ni.

I symptoms leh natna stage a zirin natna hi a enkawl tha ber. Vawikhat hmuhchhuah a nih chuan mit enfiah fo a pawimawh hle. Chumi hnuah chauh chuan i mitmenga inthlak danglamna eng pawh chu rang takin i hre thei ang a, a tul angin hma i la thei ang.

A tir lamah chuan enkawlna engmah i mamawh lo mai thei. Mahse, refractive error dang, farsightedness ang chi i neih chuan glasses i hman a ngai mai thei. Best natna vei te hian distance vision lamah harsatna an nei fo thin. Tin, mitdel i neih chuan mitdel surgery i neih a ngai mai thei bawk.

Mahse , a hmaa kan sawi tawh, choroidal neovascularization (CNV) tih natna, thisen kalna kawng chak lo thar siamna i neih chuan, i doctor chuan chutiang thisen kalna kawng thar lo thang lian zel tur tihtawp nan hetiang damdawi hi a rawn rawt thei ang:

• Anti-vascular endothelial growth factor (Anti-VEGF) agents: Hengte hi a takah chuan damdawi a ni. Heng damdawi te hi i mit chhunga vitreous cavity ah inject a ni. A tum ber chu chutiang thisen kalna kawng thar, leak tak takte lo thang lian chu tihtawp a, tihtlem a ni.
• Laser therapy: He enkawlna hian laser beam hmangin thisen kalna kawng dik lo chu a seal off emaw, a tichhia emaw a ni.
• Photodynamic therapy (PDT): Hei hi a buaithlak deuh hlek a. Hetah hian damdawi bik i taksaah inject a ni a, chutah chuan damdawi chu light-sensitive-ah siam a ni a, laser beam hmangin thisen kalna kawng a nghawngte chu a tichhe a, thisen a chhuah loh nan a titawp a ni.

Chu bâkah, i doctor chuan mitdelna hmanrua, magnifying device, special lighting, leh chhiar awlsam software te hman tûrin a rawt thei bawk .

Zirchiangtute chuan genetic material hmanga natna enkawl leh venna atana hman theih a nih leh nih loh an zirchiang tawh a ni. Gene therapy hi research stage-ah a la awm a, mahse nakin lawkah result hlawhtling tak a chhuah theih beisei a ni.

Best disease vei theihna hi a tlahniam thei ang em?

Dik tak chuan Best natna venna tur hian engmah tih theih a awm lo. Genetic condition a ni. Mahse i chhungkuaa mi tu emaw hian Best natna a vei a nih chuan, emaw, i vei tih i hriat chuan fa i neih hmain genetic counselling lak hmasak a pawimawh mai thei. Genetic counselor chuan i chhungkaw chanchin a en thei a, i fate’n natna an rochun theihna tur a hrilhfiah thei bawk.

Best natna ka neih chuan eng nge thleng ang?

Best's natna hi thihna thlen thei natna a ni lo. Chu chu nunna atana hlauhawm a ni lo tihna a ni. Mahse, a hmaa kan sawi tawh ang khan tihdam theih a ni lo. Best natna atang hian mitdel vek i ni lovang. Mahse, mit hmuhna a tlahniam mai thei. Chumi awmzia chu nitin hna thawh chu a harsa deuh mai thei a, mahse i mit i hloh vek dawn lo tihna a ni.

Engtin nge ka in enkawl ang?

Mit hriselna atana tha ei tur thenkhat a awm.

"Ei leh in tha hi mit tan a pawimawh hle."

Entirnan, kar khata ni engemaw zat sangha ei te, i eitur ah thlai hring, thei leh nut te telh te hi i mit tan a tha hle. Hengte hian mit tana tha tak tak omega-3 fatty acids leh vitamins te a pe a ni.

Best’s disease i neih chuan medical checkup neih fo a pawimawh a, a bik takin mit endik a pawimawh. I mit hmuhnaah emaw, i hriselna tlangpuiah emaw danglamna i hmuh chuan doctor hnenah hrilh nghal rawh. I doctor hnenah zawhna i zawt thei bawk a, chungte chu:

• " Fa neih leh neih loh tur ngaihtuah tura min pui turin genetic counselor i recommend thei ang em?"
• "He natna hi clinical trial-ah ka tel thei em?"
• " Lo vision lama specialist optometrist i recommend thei ang em?"
• " Gene therapy hi ka tan chuan enkawlna option a ni em?"

Best natna leh Stargardt natna hi eng nge an danglamna?

Stargardt disease leh Best disease te hi genetic disease an ni ve ve a, an pahnih hian mit chhunga center of vision (the macula) a nghawng a ni. Hei hian straight-ahead vision a nghawng tihna a ni.

Mahse heng natna pahnih hi gene hrang hrang inthlak danglamna avanga lo awm a ni.

Stargardt natnaah chuan i retina-ah yellow emaw white emaw flecks i hmu thei. Mahse Best disease-ah chuan macula hnuaiah hian cyst lian tak, sen, egg yolk ang maia oval ang chi i hmu thei. Chuvangin, heng natna pahnih hi heng symptoms leh genetic testing hmang hian a thliar hrang thei a ni.

Mit hmuhna tichhe thei dinhmun nena nun chu thil harsa tak a ni thei. Mahse Best natna i neih chuan thil awlsam zawka siam thei tur resources leh service tam tak a awm a ni. An chanchin hi doctor zawt rawh.

A tawp berah chuan thil hriat reng tur

Okay, chuvang chuan Best disease chungchang hi kan sawi nasa hle. I recap leh ang u:

• Best natna hi thlahtute atanga lo piang a ni a, mit chhunga mit hmuhna hmunpui (the macula) a nghawng a ni. Hei hian mitdelna a thlen thei a ni.
• Dam kim thei lo mah se, nunna atana hlauhawm a ni lo. Hmuh lohna a awm thei a, mahse mitdelna famkim a awm lo.
• He natna hi hriat hmasak a pawimawh hle a, medical test neih reng a pawimawh hle.
• `CNV` ang chi complication enkawlna tur a awm.
• Chhungkaw ruahmanna siamtute tan chuan genetic counseling hi thil pawimawh tak a ni thei.
• Mit tana \ha chaw ei leh mit \ha lo hman \hin hian nun a ti awlsam thei hle.

Nangmah chauh i ni lo tih hre reng ang che. Heng natnate hi i nunpui theitu doctor, counselor leh technique hrang hrang an awm a. A pawimawh ber chu chak taka awm a, a tul anga thupek zawm hi a ni. Zawhna emaw rinhlelhna emaw i neih chuan i doctor nen zalen takin inbia ang che.

` Natna tha ber, Natna tha ber, mit natna, mit hmuhna hmun, macula, genetic natna, mit hmuh lohna, mit enfiah