I fa hian heng chhinchhiahna mak tak takte hi a nei em? Long-chain fatty acid oxidation disorder (LC-FAODs) chungchang hi i zir dawn a ni.

I fa te chu rei vak lo a khelh hnuah a chau em? A nih loh leh, a damlo, ei duh lo, emaw, mutthilh ang maiin a lang reng em? A châng chuan hengte hi thil pangngaiah kan ngai a, mahse hei hi a hnuaiah hian genetic condition hmuh tur awm lo tak, mahse na tak tak a awm thei a ni. Vawiin hian chutiang dinhmun pakhat, Long-Chain Fatty Acid Oxidation Disorders, emaw LC-FAODs emaw chungchang kan sawi dawn a ni.

A awlsam zawngin, heng LC-FAOD te hi engte nge ni?

Hei hi hming hi a buaithlak deuh a, mahse a awlsam zawngin i sawi ang u. Kan taksa hi lirthei ang mai a ni. Hnathawh theih nân chakna, a nih loh leh fuel a mamawh a ni. Kan chaw ei hi kan taksa tana fuel a ni. He chaw hi chakna ah a chantir dan hi metabolism an ti a .

Kan ei tur, olive oil , sangha , avocado, leh sa te hian ‘fatty acids’ an tih thil a pai a. Hengte hi taksa tan chakna tha tak a ni. Heng fatty acids te hi chi hrang hrang a awm a. Chung zingah chuan ‘long-chain fatty acids’ an tih chi hian chakna lama chantir turin enzyme bik a mamawh a ni.

Naupang LC-FAODs tih hming pu genetic condition neia piangte hian an taksaah chu enzyme bik chu an tlachham a ni. Chuvangin an taksa hian heng long-chain fatty acids te hi energy ah a chantir thei lo.

Chuti a nih chuan eng nge thleng?

1. Thil pawimawh tak tak, thinlung , thluak, thin , leh taksa peng hrang hrangte hian chakna mamawh an dawng lo.

2. Fatty acids chakna atana chantir theih loh chu chu taksa peng hrang hrangah chuan a pung khawm a, a tichhe tan thin.

Hei hian harsatna lian tak tak a thlen thei a , chu chu heart disease , liver failure , thisen sugar level tlahniam nasa tak a ni . Chuvangin, he natna hi hriat hmasak a pawimawh hle a, ei leh in control tha tak neih a pawimawh hle .

He natna lan chhuah dan engte nge ni?

Mi tin hian natna lan chhuah dan a inang lo thei. Enzyme bo chi leh natna nasat danah a innghat a ni. Mi thenkhat chuan tleirawl emaw, puitling emaw thlengin symptom an nei lo mai thei. Mahse a nasat chuan a natna lan chhuah dan chu a dam chhung thla engemaw zat chhungin a lang tan thin.

Nausen piang tharte thinlung ruh natna lan chhuah hmasak ber chu ``cardiomyopathy`` a ni. Hei hian thawk harsa, chaw pek harsa, leh mut tam lutuk te a huam a ni. Nausen leh naupang tê tête hian thin lama harsatna an neih hmasak ber a ni thei a, chu chu mit leh vun a sen (jaundice), leh thisen sugar level hniam (hypoglycemia) te hi a ni.

Hemi chungchanga thil awm thei lian ber berte leh a kaihhnawih symptoms te hi han thlir ila.

Eng thilin nge he natna hi a thlen?

Hei hi genetic disease a ni vek a . Chu chu nu leh pa atanga fate rochun a ni tihna a ni. A awlsam zawngin, a chhan chu kan sawi tawh ang khan fatty acids tichhe thei enzyme siam chhuah dan tur hrilhtu gene-a mutation vang a ni.

Naupangin natna a vei theih nân, nu leh pa pahnih aṭangin gene ṭha lo copy chu an rochun a ngai a ni . Nu leh pa pakhat chauh atanga an rochun chuan naupang chu ``carrier'' a ni. Symptoms an nei lo ang a, mahse an fate hnenah gene chu an pe chhuak thei a ni.

Hei hi natna hrik kai theih a ni lo. Mi pakhat atanga mi dang hnenah engti kawng mahin a kai thei lo.

Engtin nge natna hi kan hriat theih ang?

Ram tam takah chuan nausen piang thar apiang chu heng genetic diseases te hi screened vek an ni. Hei hi nau piang atanga ni 1-2 chhunga kephah atanga thisen sample te tak te lak chhuah hmanga tih thin a ni. He test hian natna a hriat chian chuan test dang neih belh lehin natna a awm tih finfiah a ni.

Chumi atana test tih ber berte chu:

• Thisen leh zun test: Hengte hian thisen leh zun chhunga fatty acid leh thil dang tam tak awm leh awm loh a enfiah thin.
• Genetic testing: Hei hi LC-FAODS i neih leh neih loh leh, a nih chuan eng chi nge i neih tih chiang taka hriat theihna kawng awmchhun a ni.

I fa hian a hmaa kan sawi tawh ang khan symptoms a neih chuan naupang doctor pan nghal a , hei hi sawipui a pawimawh hle.

Engtin nge enkawl a nih?

He natna hi tihdam kim theih ni lo mah se, enkawl dan dik a nih chuan , harsatna lian tham tak takte chu pumpelh theih a ni a, nun pangngai tak a awm thei bawk. Enkawlna lian ber berte chu ei leh in thlak danglam te, ei leh in lama tihdanglamna bik te, nunphung tihdanglam te a ni.

1. Ei leh in tur

Hei hi a pawimawh ber a ni. He diet hi dietitian thurawn anga siam tur a ni.

• Long-chain fatty acids tamna ei tur tihtlem: Sa, thlai hriak thenkhat, nut, leh sangha te hi tihtlem tur a ni.
• Carbohydrate tamna ei tur ei tam rawh: Thawhrimna i neih theih nan carbohydrate tamna ei tur buhfai, alu, leh sweet potato te i eitur ah i telh tur a ni.
• Chaw te te, ei fo thin: Thisen sugar level a nghet theih nan nitin hun pangngaia ei a pawimawh.
• Chaw nghei pumpelh: Darkar 8 aia rei riltam taka awm hi a tha lo.

2. Nutritional supplement ei tur a ni

Heng damlote hian long-chain fatty acids an hman theih loh avangin taksain awlsam taka chakna a chantir theih tur thau chi dang pek an ni. Chungte chu medium-chain triglycerides (MCTs) an ti a. Hengte hi MCT oil angin a awm a. Heng MCT te hi nausen chaw ei tur thenkhatah pawh an dah tel bawk. I doctor chuan hemi chungchang hi a hrilh belh ang che.

3. Damdawi lam

LC-FAODs atan hian damdawi pakhat Triheptanoin (Dojolvi) tih chu pawm a ni. I doctor prescription chauhvin a awm thei .

4. Nunphung tihdanglam

Symptoms tizual thei triggers te hi pumpelh a pawimawh.

• Exercise tam lutuk pumpelh: Taksa tibuaitu thil tih tlem lutuk.
• Stress enkawl: Yoga leh meditation ang chi thilte hian a ṭangkai thei hle.
• Natna laka inveng rawh: I doctor-in a rawt vaccine zawng zawng chu a hun takah la vek rawh. Khawsik emaw, khawsik emaw tenau emaw pawh ni se, enkawl vat rawh.

He natna nena nun hi harsatna a ni em?

Dik tak chuan, a ni. Hei hi dam chhung zawnga dinhmun, enkawl ngai a ni. I ei leh in chungchangah i fimkhur a ngai a ni. Emergency-a i tih tur i hriat a ngai a ni. Hei hian naupang leh nu leh pa tan pawh rilru lama harsatna a thlen thei a ni.

Chuvangin,

• Natna chungchang hi uluk takin zir rawh: hriatna hi thiltihtheihna ropui ber a ni.
• Psychological support zawng: Chhungte, ṭhiante, emaw rilru lam hriselna lama counselor emaw hnen aṭangin ṭanpuina zawng hreh suh.
• I doctor nen inzawmna nei reng rawh: Zawhna emaw, ngaihtuahna emaw i neih chuan zawt rawh.

He natna hi a tlem hle nachungin, uluk taka enkawl leh enkawl a nih chuan naupang chuan nun tha tak a nei thei a ni. A pawimawh ber chu natna hriat hmasak a, doctor thupek zawm hi a ni.