Mut tlakchham avanga thihna thlentu natna hmuh tur awm lo tak? Hemi (Fatal Familial Insomnia) chungchang hi i zir chiang ang u.

Zanah pawh i mu lo em ni? Mut hnuah darkar tam tak chhung i toss leh turn thin em? Hei hi a takah chuan mi tam tak zingah chuan harsatna awm fo a ni. Mahse, hetiang mut theih lohna hi thihna pawh thlen thei, thlahtute natna hmuh tur awm lo tak takte chhinchhiahna hmasa ber a ni thei tih i ngaihtuah ngai em? Lungngai suh, he natna hi i nei tihna a ni lo. A chhan chu hei hi khawvela natna hmuh tur awm lo tak a nih vang a ni. Mahse hei hi hriat chian a pawimawh hle. Chuvangin vawiin hian he natna hlauhawm leh hmuh tur awm lo tak hi kan sawi dawn a ni.

Fatal Family Insomnia (FFI) chu eng nge ni?

A hmingah "Insomnia" tih thumal awm mahse, FFI hi a takah chuan mutna lama harsatna a ni lo. FFI hi genetic disorder hmuh tur awm lo tak a ni a, genes hmanga thlah khat atanga thlah dangah a darh zel a ni. A awlsam zawngin sawi ila, kan thluaka protein pakhatin a lo awm a, chu chu a inthlak danglam a, thluak a tichhia a ni. He natna hi a tlem em avangin mithiamte chuan khawvel pumah chhungkaw 30 velah mi 100 vel chauhvin he natna thlentu gene hi an nei tih an sawi. Chuvangin i mut theih loh chuan FFI vang a nih theihna chance chu a tlem hle a, maktaduai khatah pakhat vel a ni. Natna ang chiah hi non-genetic variant a awm bawk a, chu chu chhan awm lovin a lo awm nghal tihna a ni. Sporadic Fatal insomnia (sFI) an ti a. Mithiamte chuan engtin nge a lo awm tih chiang takin an la hre lo.

Engvangin nge he natna hi a lo awm? Eng nge a chhan?

FFI thlentu ber chu kan taksaa gene `PRPN` a mutation vang a ni. He gene hian protein pakhat `PrP` siam chhuahna a control a ni. He protein hnathawh dik tak hi hriat chhuah ni lo mah se, gene-a mutation avang hian he protein hi a pianzia dik lo takin a lo piang ta a ni. Tichuan kan taksa tan toxic a lo ni ta a ni. Hêng protein tha lo tak tak, pianzia dik lo tak takte hi kan dam chhûngin thluak hmun thalamus -ah an pung khâwm zauh zauh a ni. Thalamus hi thil pawimawh tam tak, kan mut, ei duhna, leh kan taksa lum leh vawt te thunun theitu a ni. Hun a kal zel a, heng toxic proteins te hian thalamus an tihchhiat hian a chhiatna chuan FFI symptoms a rawn lang chhuak thin. He genetic mutation hi autosomal dominant takin thlah hrang hrangah a kal zel a ni. Chumi awmzia chu natna vei tur chuan i nu emaw i pa emaw hnen atangin mutated gene copy khat i rochun a ngai tihna a ni. I nu leh pa zinga pakhatin he gene mutation hi a neih chuan nang pawhin i vei theihna chance 50% i nei a ni.

He natna lan chhuah dan engte nge ni?

FFI natna lanchhuahna hi kum 40 leh 60 inkar atanga lo lang tan a ni tlangpui a, a bul tanna a na lo hle nachungin heng chhinchhiahna te hi a na nghal vat thei a ni. Heng symptoms te hi eng nge ni tih han thlir ila.Heng symptom tam tak hi mut that lohna leh thluak chhiatna avang te pawh a ni. Lungchhiatthlak takin damlo tam zawk chu an natna lan chhuah tirh atanga thla 6 atanga thla 36 (kum 3) chhungin heart attack emaw, natna hrik kai dang emaw vangin an thi thin.

He natna hi engtin nge doctor hian a hriat theih ang?

I doctor-in FFI i neih a rinhlelh chuan test eng emaw zat an ti ang a, chu chu finfiah turin an ti ang.

• Chhungkaw damdawi lam chanchin zawhna: Hei hi thlahtute atanga lo chhuak natna a nih avangin chhungkaw zingah hian hetiang natna lan chhuah dan hi tu emaw chuan an nei tawh em tih hriat a pawimawh hle.
• Taksa enfiah : Symptoms te uluk taka enfiah thin a ni.
• Genetic testing : Thisen sample pakhat chu `PRPN` gene-a mutation awm leh awm loh enfiah a ni.
• Mut dan zirchianna: Damdawi in pakhatah hian hmanraw bik hnuaiah mut dan enfiah thin a ni.
• Spinal Fluid Testing: Spinal cord atanga cerebrospinal fluid lak chhuah enfiah .

A then phei chuan natna hmuhchhuah hmaa mi a thih chuan FFI hi autopsy neih laiin thluak enfiahin a finfiah theih a ni.

Hei hi eng enkawlna nge ni?

FFI enkawlnaah hian natna lan chhuah dan enkawl leh damlo chu a theih ang anga hahdamna pek hi a pawimawh ber a ni. Natna hmuh tur awm lo tak a nih avangin standard treatment regimen a awm lo. Health professional team, neurologist leh psychiatrist te chuan damlo enkawlna tur dik tak chu an rel dawn a ni. Enkawlnaah hian a tlangpuiin:

• Vitamin leh ei tur dang pek belh.
• Ei leh in inthlau tak pek.
• I natna lan chhuahna tizualtu damdawi dang i ei a nih chuan titawp la, thlak rawh .
• Doctor-in a tih angin mutthilhna atana pui turin sedatives emaw melatonin emaw thenkhat pek.
• Psychological relief atana counseling pek .

Nakin lawka chhungkaw member dangte hnena natna a darh loh nan doctor chuan chhungkaw member zawng zawngte chu genetic testing nei turin a fuih mai thei.

Engtikah nge Doctor ka hmuh ang?

Ka sawi nawn leh a, mut harsatna i neih chuan FFI a nih theihna chance a hniam hle, a hniam hle. Chuvangin a tul lovah ngaihtuah buai suh. Amaherawhchu, rei tak mut harsatna i neih chuan, emaw, i mut theih loh ruala i rilru buai emaw, kal harsa emaw ang chi symptom dang i neih chuan doctor pan nghal tur a ni. A chhan chu chutiang chhinchhiahnate chu FFI a ni lo mai thei a, mahse enkawl theih natna dang chhinchhiahna a ni thei bawk. Chuvangin diagnosis mumal tak neih a, a tul anga enkawlna zawn a pawimawh hle. Fatal Family Insomnia, FFI, mut theih lohna, genetic natna, prion natna, mut harsatna, thluak natna