Zanah pawh hmuh harsa i nei em? I mit a chhe zauh zauh em? Retinitis Pigmentosa (RP) chungchang hi i zir dawn teh ang!

A châng chuan zanah emaw, êng tlem laiah emaw lirthei khalh harsa i ti ṭhin a ni lawm ni? A nih loh leh, i hmaa lo kal lai hmu lovin mi i tawk tawh em? Hetiang hi i tawng tawh a nih chuan a chhan chu mit natna `Retinitis Pigmentosa` (RP) an tih vang a ni thei. Lungngai suh, hei hi thil awm dan tlangpui a ni lo, mahse hriat reng a pawimawh. A chungchang hi chipchiar zawkin han sawi ila.

`Retinitis Pigmentosa` (RP) tih hi eng nge ni? Engvangin nge a pawimawh em em?

A awlsam zawngin sawi ila, `Retinitis Pigmentosa` (RP) hi mit natna chi hrang hrang, thlah khat atanga thlah danga kai theih, hming tlangpui a ni. I mit chhunga `retina` a nghawng ber.

I mit chu film camera hlui ang maiin ngaihtuah rawh. Chu camera-a thlalak chu film-a lak a ni. Chutiang chu a ni, i mit chhungah, hnung lamah, membrane nalh tak, light-sensitive tak, retina an tih chu a awm a. Kan thil hmuh atanga êng lo chhuak chu retina-ah a tlak chuan chu êng chu electrical signal-ah a chantir ta a ni. Tichuan chu signal te chu thluak lamah a kal a, ‘Aw, hei hi chu a ni, hei hi chu chu a ni’ tih kan hmu ta a ni.

Chuvangin, retina a thawk tha lo a nih chuan camera chhunga film awm chu a buai ang mai a ni. Engtiang taka i focus pawh nise, thlalak chu a lang chiang lovang. Chutiang bawkin retina natna nei i neih chuan glasses emaw contact lens emaw i vuah pawhin i mit a nghawng dawn a ni.

Retina-ah hian nerve cell chi hrang hrang, êng dawng thei tur a awm a. Chungte chu photoreceptor cell an ti a. Chung chu chi hnih a awm a, chungte chu rod leh cone te an ni. Rod hian êng hniam takah min pui a, a bik takin zan lamah kan hmu thei. Cone hian rawng leh thil chi hrang hrang chiang takin min hmu thei a. Heng cell te bakah hian retina ah hian cell chi dang retinal pigment epithelium cells tih a awm bawk. Heng cell zawng zawng hian an thawk dun a, an thawk dun a, mit hmuhna tha tak min pe a ni.

Retinitis Pigmentosa (RP) leh inherited retinal diseases (IRDs) dangte hi heng cell te hnathawh thatna tur genes inthlak danglamna avanga lo awm a ni a, chu chu genetic mutations an ti a ni. Hei hian cell te chu zawi zawiin a tichak lo va, hna a thawk tawh lo.

RP hi natna pakhat chauh a ni lo. Natna pawl a nih avangin mi tin mit hmuh dan a inang lo thei hle. Mi tam tak chuan mitdelna an nei , mi thenkhat chuan an mit an hloh vek thei bawk. Heng mit lama inthlak danglamna te hi naupan lai atanga tan a ni tlangpui. Mahse, a châng chuan heng inthlâk danglamnate hi a thleng zawi zawi a, i hre lo pawh a ni thei. Mi thenkhat tan chuan mitdelna hi a thleng rang hle. RP chi thenkhatah chuan mit hmuhna hloh hi level engemaw takah a tawp thin.

A pawimawh ber chu Retinitis Pigmentosa hian mit pahnih a nghawng tlangpui .

RP natna lan chhuah dan chu engte nge ni? Engtin nge a nghawng che?

`Retinitis Pigmentosa` symptoms hi a lang nghal lo. Zawi zawiin an lo lang thin. A lan chhuah hmasak ber thenkhat chu hetiang hi a ni:

• Zan lama mit hmuh harsatna: Zanah thil hmuh harsa i nei tih i hmu thei a, a bik takin eng a hniam lai hian. Hei hi zan lama kawngpuiah emaw, in chhunga êng êng lo tak hnuaia kal emaw chuan a harsa thei hle.
• Dim light-a hmuh harsatna: Zanah chauh ni lovin, pindan thim deuh emaw, movie theater emaw ang chi hmunah pawh thil chiang taka hmuh a harsa thei.
• Peripheral vision-a mitdel: I thil en chu i hmu thei a, mahse i chhehvela thil awmte chu i hmu thei lo mai thei, chu chu a sir atanga lirthei lo kal emaw, mi rawn hnaih emaw te hi a ni. Hei vang hian mi thenkhat chuan thil an tawk palh a ni.

Hun a kal zel a, symptom dang a lo lang thei bawk. Chung zingah chuan:

• Eng êng êng emaw, eng eng emaw hriatna: Mi ṭhenkhat chuan êng eng emaw, êngin a rawn nuai emaw an nei thei.
• Tunnel vision (Central vision chauh neih): Hei hi tube hmanga khawvel thlir ang mai a ni. Hma lam dinglam chauh i hmu thei a, i chhehvela engmah a awm lo.
• Photophobia - Eng êng takah sensitive emaw, nuam lo emaw: Mit chu a sen a, ni êng emaw, êng eng emaw kan hmuh chuan hmuh a harsa ṭhin.
• Rangka hmuh theihna hloh: Rangkachak chu a hma angin a eng lo emaw, a fiah lo emaw pawh a ni thei.
• Mit tlem lutuk neih: A nasat chuan mit hmuhna a tlahniam nasa thei hle.

Pawimawh: Heng symptoms pakhat emaw a aia tam emaw i neih chuan a rang thei ang bera mit lam doctor pan a tha ber.

Engvangin nge `Retinitis Pigmentosa` hi a lo awm? Hei hi eng nge a chhan?

`Retinitis Pigmentosa` (RP) leh `Inherited Retinal Diseases (IRDs)` dangte awm chhan ber chu kan genes-a inthlak danglamna thenkhat `(genetic mutations)` a ni Heng genes te hi kan `(retina)` chhunga cell siam chhuaktu leh hnathawk tha tak siamtu an ni. Chuvangin, heng genes-ah hian tihsual emaw, inthlak danglamna emaw eng emaw a awm chuan, chu cell-te chuan hna an thawk tha thei lo. Hun a kal zel a, chu cell-te chu zawi zawiin an thi ta a ni. Chu chu mit hmuhna a tlahniam zauh zauh lai a ni.

Rochun a nih avangin naupangte chuan heng genetic changes te hi an nu leh pate hnen atangin an rochun thei a ni. Mahse, i chhungkuaa mi pakhatin a neih vang mai maiin i nei dawn tihna erawh a ni lo. I chhungkuaa tu mahin an neih loh pawhin i tan pawh a awm thei bawk. Chuvang chuan genetic testing hi a pawimawh hle.

Khawvelah hian he dinhmun hi engtiang chiahin nge a darh?

Europe leh America rama statistics atanga a lan dan chuan mi 3,500 atanga 4,000 zinga pakhat chuan `Retinitis Pigmentosa` a vei thei a ni. Khawvel pumah hian mi maktaduai hnih velin he natna hi an vei nia chhut a ni. Sri Lanka ramah pawh hetiang natna vei an awm bawk. Hei hi kan hriat chian a pawimawh hle a ni.

Doctor-te hian engtin nge Retinitis Pigmentosa (RP) hi an hriat theih?

RP i vei nia i rinhlelh chuan i doctor chuan test hrang hrang engemaw zat a ti ang che. Mit enfiah fo a pawimawh hle.

Mit dilated leh visual field test hmanga enfiah a ni

Hetah hian mit lam doctor emaw mit lam doctor emaw chuan a hnuaia mi ang hian a ti thin.

• Bang chunga thuziak chu chhiar turin an ti che a.
• Thil pakhat chu mit pahnih hmanga en turin a hrilh che a ni.
• I mitmenga pressure awm chu teh a ni.
• Visual field test hian i peripheral vision a enfiah thin.
• I mit pupil-in êng a chhan dan en.
• Mit chu mit chhungah eye drop dahin a dilated thin. Hei hian retina chu ngun zawkin a enfiah thei a ni.
• Retina thlalak pawh i la thei bawk.

Electroretinography (ERG) hmanga enfiah a ni

Hei hi test bik a ni. I retina-in êng a chhan dan a teh a ni. I retina-a cell hrang hrang (kan sawi tawh rod leh cone) te hnathawh that dan a teh thin. I mit hmaa light hrang hrang flashing a huam a ni. Test group pakhat, ophthalmic electrophysiology tests an tih zingah a tel a ni. Heng test te hian i mit leh thluak hian i thil hmuh chu engtin nge a process tih pawh a en bawk.

Optical Coherence Tomography (OCT) hmanga enfiah a ni

Hei hi noninvasive test a ni. He OCT scan hian i retina thickness a teh thei a, a layer te hriselna a zirchiang thei bawk. I tih tur chu target pakhat i en mai a, camera bik hmangin i mit chhung lam thlalak a la thei bawk.

`Fundus Autofluorescence (FAF)` hmanga enfiah a ni

Hei hi noninvasive ``imaging test`` a ni bawk a, mit thlalak a la thin. Retina hriselna chungchangah thu tam tak a pe thei a ni. Natna hriatchhuahna atan te, enkawl nan te, enkawlna atan te hman a ni.

Heng test te bakah hian i doctor chuan genetic test leh genetic counselor te pawh a rawt thei bawk.Tlawh tur pawh i rawt thei bawk. A chhan chu eng genetic mutation hian RP a thlen tih chiang taka i hriat chuan nakin lawkah he natna hian engtin nge a awm dawn tih leh chhungkaw member dangte a nghawng dawn em tih ngaihtuahna i nei thei a ni. Enkawlna thar thenkhat dawn a pawimawh bawk a, entirnan, gene therapy emaw, a kaihhnawih clinical trial-a tel emaw pawh a pawimawh hle.

He natna `Retinitis Pigmentosa` (RP) hi eng enkawlna nge ni? Engtin nge enkawl a nih?

Dik tak chuan tun hnaiah Retinitis Pigmentosa (RP) leh IRD dangte enkawlna kawngah hmasawnna nasa tak a awm a, a bik takin gene therapy ang chi enkawlna thar tiam a nih avangin.

Tuna RP enkawl dan thenkhat chu hetiang hi a ni:

• Low vision aids leh assistive devices hman: Magnifier chi hrang hrang, special glasses, leh technological devices thlengin a awm a, chu chuan thil pakhat chu eng nge a nih emaw, tu nge a nih emaw chu i kawhhmuh hian a hriat theih a ni.
• Ni laka invenna: Sunglass vuah a pawimawh a, êng êng laka invenna tihtlem a pawimawh a, a châng chuan êng êng tak hian RP a tichhe thei a ni.
• A kaihhnawih natna dang enkawl: RP nena lo awm thei cystoid macular edema (CME) (retina lairil a tui awmkhawm) ang chi natna enkawl.
• Mitdel enkawlna: RP vei thenkhat chuan mitdelna an nei thei. Chutiang a nih chuan surgery hmanga lakchhuah theih a ni.

Gene Therapy chungchang tlem han zir ila.

US FDA chuan RP chi khat enkawl nan gene therapy product voretigene neparvovec-ryzl (Luxturna®) chu a pawm a ni. RP65 gene copy pahniha mutation nei te chuan he enkawlna hi an hlawkpui thei a ni. Mahse, he RP chi bik hi mi tlemteah chauh hmuh tur a awm.

Tunah hian RP leh IRD chi dang tan gene therapy atan clinical trial engemawzat neih mek a ni a, chuvangin nakin lawkah enkawlna tam zawk neih beisei a ni.

RP na tak nei tan chuan retinal prosthesis an tih device ngaihtuah theih a ni.

`Retinitis Pigmentosa` (RP) lo awm tur ven dan tur a awm em?

Retinitis Pigmentosa hi thlahtute atanga lo chhuak a nih fo avangin ven kim theih a ni lo.

Amaherawhchu, i mit a hrisel thei ang bera awm theih nan thil i tih theih a awm a:

• I mit enfiah turin mit lam doctor hmu fo ang che. Hei hian harsatna eng pawh i hriat hmasak theih nan a pui ang che.
• Sunglass vuah la, êng êng tak lakah i mit venghim rawh.
• Nunphung hrisel tak zawm rawh. Chaw ei tha la, him takin exercise la.

RP i neih chuan eng nge i beisei theih ang?

Retinitis Pigmentosa hi mi zawng zawng tan kawng inang leh rate inang lo takin a kal zel lo. Hei hi a chhan chu a thlentu gene hrang hrang tam tak a awm vang a ni. Gene a zirin natnain mi tin a nghawng dan a inang lo. Chuvangin genetic testing leh genetic counselling neih a pawimawh hle.

Tuna clinical trial neih mek te, support group te, leh visual aids te chu i doctor hnenah zawt rawh.

Engtikah nge Doctor ka hmuh ang?

A tlangpuiin mit doctor pan fo thin ang che. Tin, symptom thar i neih chuan, emaw, i symptoms a na zual a nih chuan doctor pan nghal rawh. Entir nan:

• I mit a chhe zual nia i hriat chuan (clearness or color vision).
• I mitah natna thar emaw, hrehawmna emaw i neih chuan.

Hriat reng tur: Retinitis Pigmentosa hi chi hrang hrang a awm a, chi zawng zawng hian mit a tichhe vek lo. A theih ang anga i mit venhimna leh hlawkna i hmuh theihna tur kawng tha ber chu mit enfiah fo a, doctor thupek zawm hi a ni.

A tawi zawngin hei hi hre reng ang che! (In lama hruai tur thuchah)

Retinitis Pigmentosa (RP) hi natna khirh tak a ni a, ngaih pawimawh a ngai hle. Amaherawhchu, i hriat chian a, a tul anga hma i lak chuan nun pangngaia nunpui turin i in tanpui thei a ni.

• Hlau suh, hriattir rawh: RP i vei tih hmuhchhuah a nih chuan a chungchang hi hriattir tha rawh. I doctor hnenah zawhna zawt rawh.
• Checkup neih fo: I mit doctor-in a rawt angin a hun takah i mit enfiah tir thin ang che.
• Genetic testing leh counseling: I dinhmun dik tak hriat chian nan hengte hi a pawimawh hle.
• Ṭanpuina dawng rawh: Hemi chungchang hi chhungte leh ṭhiante nen sawiho rawh. A tul chuan support group-ah te tel ve rawh. Nangmah chauh i ni lo.
• Enkawlna tharte fimkhur rawh: Medical science a lo thang chho zel a. I doctor hnenah enkawlna thar leh clinical trial chungchang zawt rawh.

Mit venhimna tur thil tam tak a awm a. Beiseina bansan lova thil dik tih hi a pawimawh ber a ni.

` Retinitis Pigmentosa, RP, mit natna, mitdel, zan lama mitdel, genetic natna, retina, mit hmuhna