I fa chu a sen a, a chau reng em? Thalassemia a ni thei ang em?

I naupang tê chu a chau reng niin a lang em? Naupang dang angin a tlan kual a, a inkhelh thin lo em ni? Chaw ei duh lohna nasa tak a nei em? A rilru a hahdam tawh ngai em? Nu leh pa tu pawhin hetiang thil an hmuh hian hlauhna nasa tak an neih hi thil pangngai a ni. Heng symptoms te hi dinhmun pangngai avanga lo awm thei a nih fo laiin, a chang chuan hei hi a hnung lamah hian damdawi lam thil a awm thei a, chu chu Thalassemia te hi a ni. Chuvangin vawiinah hian Thalassemia hi eng nge a nih, kan taksa a nghawng dan te, a enkawlna tur engte nge ni tih te han sawi ila.

Thalassemia hi eng nge ni tak tak?

A awlsam zawngin, thalassemia hi kan thisen tichhe thei natna pianphung a ni . Natna hrik kai theih a ni lo. Chu chu khawsik angin mi pakhat atanga mi dangah a darh lo tihna a ni. Kan genes hmanga kan nu leh pate hnen atanga kan rochun thil a ni.

Hei hi tlem tal hrethiam zawk tur chuan kan thisena thisen sen (red blood cells) chungchang tlem han sawi hmasa ila. Heng thisen sen (red blood cells) te hi kan taksa pum puia oxygen phurtu delivery service angin han ngaihtuah teh. He delivery service-a lirthei, chu chu oxygen phur cart chu hemoglobin . Hemoglobin hi thisen sen chhunga protein bik a ni. Hei hi kan lung atanga oxygen phur chhuaktu a ni a, taksa cell tinah a sem darh thin.

Thalassemia vei chuan he protein hemoglobin an tih hi a siam tling thei lo. Car siam lai ang bawkin, a tul thenkhat a bo emaw, a hnuai lam hman a nih chuan car chu a thawk tha lo ang. Hei hian taksaa thisen zam (red blood cell) hrisel tak takte a tihtlem phah a ni. Hetiang dinhmun hi thisen sen hniam (low red blood cells anemia) kan ti a .

Chuvangin taksaa cell-te hian oxygen an hmuh tur dik tak an hmuh loh chuan an mamawh chakna an siam chhuak thei lo. Chuvang chuan thalassemia vei te hian symptom hrang hrang an nei thin.

He natna vei theihna hlauhawm ber chu tute nge ni?

Hei hi a thlentu genetic mutations hi mihringah malaria laka invenna atan a lo piang hmasa ber niin an ngai. Chuvangin, malaria vei tamna hmun, Africa, Europe khawthlang lam, leh Asia chhim, chhim leh chhim lam ramte nena genetic ties nei te chu thalassemia vei an hlauhawm zawk a ni. Sri Lanka pawh hi South Asia ram a nih avangin kan mipui zingah thalassemia carrier leh damlo an awm a.

Eng thilin nge thalassemia hi a thlen? Ngun zawkin han thlir ila.

Chu "oxygen cart" hemoglobin an tih ka sawi tawh kha protein chain pali atanga siam a ni. Chung zinga pahnih chu alpha globin chain an ti a, a dang pahnih chu beta globin chain an ti bawk.

Heng chain siamna tur "instructions" emaw "code" emaw hi kan nu leh pa hnen atanga kan rochun genes atang hian kan hmu thin. Heng genes te hi dish siamna recipe angin ngaihtuah rawh. Recipe-ah hian tlin lohna emaw, tihsual emaw a awm chuan dish chu a siam tha thei lo. Chutiang bawkin heng gene-ah hian tlin lohna emaw, tlakchhamna emaw a awm chuan alpha emaw beta globin chain emaw a lo piang tha lo ang. Chu chu thalassemia an tih natna a lo awm hun a ni.

• Alpha globin chains: Hengte hi siam tur chuan gene 4 a ngai a. Nu hnen atangin pahnih leh pa hnen atangin pahnih.
• Beta globin chain: Hengte hi siam tur chuan gene pahnih a ngai a. Nu hnen atangin pakhat leh pa hnen atangin pakhat.

Thalassemia i vei chi chu heng alpha emaw beta chain emaw zingah hian eng chain nge gene defect nei tih ah a innghat. Natna nasat dan hi genes chhia zat atanga teh a ni.

Thalassemia chi hrang hrangte chu engte nge ni?

Thalassemia hi natna nasat dan azirin level lian engemaw zatah then a ni. Chu chu thalassemia trait, thalassemia minor, thalassemia intermedia, leh thalassemia major te a ni. Carrier state-ah chuan symptoms i nei lo mai thei. A nih loh leh thisen tlakchhamna na lo tak tak chauh i nei thei bawk. Thalassemia major hi natna khirh ber, enkawl ngai a ni.

A chi hnih a awm a, chu chu genetic defect chu alpha chain-ah emaw beta chain-ah emaw a awm leh awm loh a zirin a ni.

1. Alpha Thalassemia natna a ni

Hetiang dinhmun hi alpha globin chain siamtu gene 4 zinga pakhat emaw a aia tam emaw a chhiatna a awm chuan a thleng thin. A natna lan chhuah dan nasat dan chu gene chhia zat a zirin a ni. Hei hi hriatthiam theih nan a awlsam zawngin han thlir ila.

2. Beta Thalassemia natna a awm

Hetiang dinhmun hi beta globin chain siamtu gene pahnih zinga pakhat emaw, pahnih emaw (nu atanga pakhat, pa atanga pakhat) a chhiatna a awm chuan a thleng thin.

• Gene chhia pakhat: He natna hi beta thalassemia minor emaw carrier state emaw an ti a. Symptoms hi a na lo hle.
• Gene chhia pahnih: A natna lan chhuah dan chu a hniam lam atanga na tak thlengin a awm thei.
• A inkar boruak chu Thalassemia intermedia an ti a .
• A na ber chu beta thalassemia major emaw Cooley’s anemia emaw an ti a. Heng mite hian damdawi in enkawl reng an mamawh a ni.

Thalassemia natna lan chhuah dan chu engte nge ni?

I natna lan chhuah dan chu thalassemia i neih chi leh a nasat danah a innghat vek a ni.

Symptoms nei lo emaw symptoms na lo tak tak emaw a awm lo

Alpha gene pali zinga pakhat chauh i hloh a nih chuan symptoms i nei lo mai thei. Alpha gene pahnih emaw beta gene pakhat emaw a chhiat chuan symptom engmah i nei lo mai thei. A nih loh leh, thisen tlakchhamna (anemia symptoms) na lo tak tak chauh i nei thei a, chu chu chau reng reng ang chi hi a ni.

A natna lan chhuah dan inkar (Thalassemia Intermedia) .

Heng mite hian thisen tlakchhamna tenau (minor anemia) bakah hian symptom dang an nei thei bawk.

• Hlawhtling lo: Naupang san zawng leh rit zawng hi an kum a zirin a pung lo.
• Puberty hun sawn hlat.
• Ruh dik lo awm te: 1.1.Osteoporosis ang chi natna te.
• Enlarged spleen: Spleen hi kan pum veilam a awm, natna hrik dona atana puitu organ a ni.

A natna lan chhuah dan na tak (Thalassemia Major) .

Symptom na tak chu alpha gene defect pathum (Hemoglobin H disease) leh beta thalassemia major (Cooley’s anemia) ang chi dinhmunah a awm thin. Heng symptoms te hi naupang kum hnih a tlin hmain a lang tan tlangpui .

A chunga kan sawi takte bakah hian a hnuaia mite hi i hmu thei ang:

• Chaw chu a thlum lo.
• Vun dum emaw, sen emaw (jaundice).
• Zun dum (tea rawng) a ni.
• Hmai ruh dik lo (luruh ruh a lo lian lutuk vang).

He natna hi engtin nge kan hriat theih ang?

Thalassemia na tak leh na tak hi naupan lai atanga hmuhchhuah a ni fo a, naupang dam chhung kum hnih chhungin a natna lanchhuahna a lo lang tan thin a ni. I doctor chuan thisen test hrang hrang a ti thei a, chu chuan a natna chu a finfiah thei a ni.

• Complete Blood Count (CBC): Hei hian thisenah hemoglobin awm zat, thisen sen (red blood cells) awm zat leh a lian leh te a teh a ni. Thalassemia vei te hian thisen sen hrisel an nei tlem zawk a, hemoglobin an nei tlem zawk bawk. Thisen sen (red blood cells) pawh a pangngai aia te zawk pawh a ni thei.
• Reticulocyte count: Hei hian thisen sen thar siam chhuah zat a teh a ni. Hei hian i ruhro hian thisen sen (red blood cells) a siam tling em tih hriat theihna a pe thei che a ni.
• Iron study: He test hian i thisen tlakchhamna hi iron tlakchham vang nge thalassemia vang tih dik taka hriat theih nan a pui a ni.
• Hemoglobin electrophoresis: Hei hi beta thalassemia hriat theihna atana test pawimawh tak a ni.
• Genetic testing: He test hi alpha thalassemia hriat theihna atan hman a ni.

Thalassemia enkawl dan tur chu engte nge ni?

Thalassemia major ang chi natna khirh tak takte enkawlna standard chu thisen pek leh iron-removal therapy te hi a ni.

• Thisen pek: A awlsam zawngin sawi ila, hei hi pawn lam thisen pek a ni. Thisenah thisen sen hrisel tak tak awm chu vein hmangin taksaah a lut a. Hei hian thisen sen hrisel leh hemoglobin level a siam tha leh thin. Thalassemia natna khirh tak (e.g., beta thalassemia major)-ah chuan thisen pek fo a ngai thei a, kar 2-4 danah thisen pek a ngai thei bawk .
• Iron Chelation hmanga enkawlna:Thisen pek fo hian a nghawng lian ber pakhat chu taksaa iron level a tul lo taka tihpun hi a ni. Hei hi iron overload kan ti a ni . He extra iron hian kan thinlung leh thin ang chi organ pawimawh tak takah a lo pungkhawm thei a, a tichhia thei bawk. Chuvangin thisen dawng fo thinte chu taksa atanga he extra iron paih chhuaktu damdawi (pill emaw injection emaw) pek an ni.
• Folic Acid Supplements: Folic acid hi taksain thisen cell hrisel a siam theih nan pek a ni.
• Bone Marrow leh Stem Cell Transplant: Tun dinhmunah chuan hei hi thalassemia tidam kim thei damdawi awmchhun a ni. Mahse, hei atan hian damlo chuan donor hrisel tak, a inmil tawk tak a mamawh a ni. A tam ber chu unaupa emaw, unaunu emaw a ni. Hei hi enkawlna risky tak leh complex tak a ni.
• Luspatercept: Hei hi kar thum danah vaccine pek thin a ni. Taksain thisen zam (red blood cells) tam zawk siam chhuahnaah a pui a, a thawk a ni.

He natna nen hian nun hi eng ang nge ni? A veng thei ang em?

Thalassemia minor i neih chuan dam rei zawng pangngai i beisei thei. I dinhmun chu a hniam emaw, a na emaw pawh nise, enkawl dan tur ruahman (thisen pek leh iron removal therapy) i zawm dik chuan dam rei theihna chance tha tak i nei a ni.

He natna hi genetic a nih avangin kan veng thei lo. Mahse, genetic testing hian nangmah emaw i kawppui emaw hian thalassemia gene hi in nei em tih hriat theih nan a pui thei che a ni. He thu hriat hi fa neih tum nupa tan chuan a pawimawh hle. Hemi chungchanga thurawn dang dawng turin i doctor nen inbia ang che.

A tawpah chuan thalassemia hi natna enkawl theih a ni. Enkawlna i mamawh chi leh i mamawh zat chu i dinhmun nasat danah a innghat ang. I dinhmun leh hun rei tak chhunga i enkawl dan tur chungchangah i doctor nen inbiakna zau tak neih a pawimawh.