Thalassemia chungchanga hriat tur thilte: A awlsam zawngin sawi ila

Chau leh chau takin i inhria fo em? I vun chu a hring deuh mai thei em? Hengte hi thil random mai a ni lo va, he thisen tlakchhamna, chu chu thisen tlakchhamna hi hei vang pawh a ni thei. Vawiin hian ‘Thalsemia’, thisen nena inzawm natna, pianpui natna, kan rama mi tam tak zingah a hluar hle tih kan sawi dawn a ni. He hming i hriat hian hlau suh. Hemi chungchang hi awlsam takin i hrethiam vek ang u.

Thalassemia hi eng nge ni tak tak?

A awlsam zawngin, thalassemia hi pian tirh atanga rochun thisen natna a ni . Natna hrik kai theih a ni lo. Hetiang dinhmun hian kan taksain hemoglobin hrisel tak a siam chhuah theih loh phah a ni.

Tunah chuan hemoglobin hi eng nge ni tih i ngaihtuah mai thei. Hemoglobin hi kan thisen sen chhunga protein bik awm a ni. ‘Delivery service’ angin hna a thawk a, chu chuan kan taksa pum puiah oxygen a keng tel a ni. He package hemoglobin an tih hi taksa chhunga cell tinah thisen sen (red blood cells) an tih lirthei hmangin a thleng thin.

Thalassemia vei chuan an taksaah hemoglobin hrisel siamchhuahna a tlahniam a. Chuvangin taksaa thisen zam (red blood cells) hrisel tak tak awm zat pawh a tlahniam bawk. Hetiang dinhmun hi thisen sen tlahniam hi anemia , a nih loh leh ‘thisen tlakchhamna’ kan tih hi a ni. Taksa cell ten an mamawh oxygen an dawn loh chuan symptom hrang hrang a lo lang tan thin.

Chu chu hetiang thil hi i hre mai thei tihna a ni:

• Chauhna leh hahna nasa tak awm reng.
• Thâwk harsa.
• Khua a vawt tih hriatna.
• Lung a na hle tih a hriat.
• Vun dum (pale skin) a ni.

Eng thilin nge thalassemia hi a thlen? Tute nge risk sang zawk?

Thalassemia hi kan genes a chhiat vang a ni. Heng genes te hi ‘program code’ angin ngaihtuah la, chutah chuan kan taksa chhunga thil awm zawng zawng hi engtin nge a insiam tur tih thupek a awm a. Heng gene atanga thupek angin hemoglobin protein hi siam a ni. Heng thupekteah hian thil dik lo a awm a nih chuan, a then a awm loh chuan taksain hemoglobin hrisel a siam thei lo. Heng gene chhia te hi kan nu leh pate hnen atangin kan rochun thin. Chuvang chuan thlahtu natna (hereditary disease) an ti a ni.

Hemoglobin molecule pakhat hi protein chain pali atanga siam a ni a, chungte chu alpha globin chain pahnih leh beta globin chain pahnih a ni.

• Alpha globin chain siam nan hian gene pali (nu leh pa pakhat atanga pahnih) a ngai a ni.
• Beta globin chain siam nan hian gene pahnih (nu leh pa pakhat atanga pakhat) a ngai a ni.

Heng alpha emaw beta chain siamtu gene-ah hian chhiatna a awm chuan thalassemia a awm thin. Natna nasat dan hi genes chhia zat atanga teh a ni.

Thalassemia chi hrang hrangte chu engte nge ni?

Thalassemia hi natna nasat dan a zirin main level engemaw zatah then theih a ni. Chu chu, asymptomatic carrier state (trait) atanga minor, intermediate, leh major thlengin a awm thei tihna a ni. A na ber, Thalassemia Major hi enkawl chhunzawm zel a ngai tlangpui.

Thalassemia hi chi hnih ah then a ni a, chu chu genetic defect hi alpha chain emaw beta chain emaw ah a awm leh awm loh a zirin a ni.

1. Alpha Thalassemia natna a ni

2. Beta Thalassemia natna a ni

Heng chi hnih hi a hnuaia table atang hian chipchiar zawkin i hrethiam ang u.

Thalassemia natna lan chhuahna awm thei te chu engte nge ni?

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

Symptoms nei lo case te

Alpha gene defect pakhat emaw, beta thalassemia minor ang chi natna na lo tak emaw i neih chuan symptoms i nei lo mai thei . A nih loh leh, hahna tlemte ang chi thil i nei thei bawk.

Symptoms na lo leh na lo tak a awm

A nasat zawk, beta thalassemia intermedia ang chi te bakah hian thisen tlakchhamna natna langsar lo tak tak bakah hian a hnuaia mite hi a thleng thei a ni:

• Naupangte thanna zawi zawi.
• Puberty hun sawn hlat.
• Ruh dik lo (e.g. ruh natna).
• Spleen lian (hei hi natna hrik dotu taksa peng a ni).

Symptom na tak tak a awm

Thil na tak, alpha gene pathum chhiatna (Hemoglobin H natna) emaw beta thalassemia major (Cooley’s anemia) ang chiah hian naupang kum 2 a tlin hmain symptoms a lang chhuak thin. A chunga kan sawi takte bakah hian a hnuaia mite hi hmuh tur a awm thei bawk.

• Chaw ei chakna hloh.
• Vun dum emaw, sen emaw (jaundice ang chi).
• Zun dum, tea rawng a ni.
• Hmai ruh a lo thang dan pangngai lo.

He natna hi engtin nge dik taka kan hriat theih ang?

Thalassemia natna (moderate and severe thalassemia) hi naupan lai atanga hmuhchhuah a ni fo thin. Hei hi a chhan chu naupang dam chhung kum hnih hmasa berah symptoms a lo lang tan vang a ni. I doctor chuan i natna hriat theih nan thisen test hrang hrang a ti thei ang.

• Complete Blood Count (CBC): Hei hian thisen sen zat, a lian leh te, leh hemoglobin level te a teh a ni. Thalassemia vei te hian thisen sen hrisel tak tak an nei tlem zawk a, an lian lo zawk pawh a ni thei.
• Iron level testing: He test hi iron tlakchhamna leh thalassemia thliar hrang nan a pawimawh hle.
• Hemoglobin Electrophoresis: He test hi beta thalassemia hriat theihna atana hman bik a ni.
• Genetic Testing: Heng test te hi alpha thalassemia finfiah nan leh natna kengtute hriatchhuah nan hman a ni.

Thalassemia enkawl dan tur chu engte nge ni?

Enkawl dan tur chu a natna nasat danah a innghat a ni. Mild case-ah chuan enkawl a ngai lo fo. Amaherawhchu, natna khirh tak tak tan chuan enkawlna chi hrang hrang a awm a.

• Thisen pek: A awlsam zawngin, ‘thisen pek’ . Mi hrisel thisen chu vein hmangin damlo hnenah pek a ni a, chu chuan thisen sen hrisel leh hemoglobin level a siam that leh thin. Beta thalassemia major vei te hian thisen pek fo a ngai a, a tlangpuiin kar 2-4 danah thisen pek an ngai thin.
• Iron Chelation Therapy: Thisen pek fo hian taksaa iron level chu a tul lovah a tisang thei a ni. Hei hi ‘iron overload’ an ti a. He iron tam lutuk hian thinlung leh thin ang chi taksa peng hrang hrang a tichhe thei a ni. Chuvangin he iron tam lutuk hi taksa atanga paih chhuaktu damdawi te hi ‘iron chelation’ an ti a. Hengte hi pill angin ei theih a ni.
• Folic Acid Supplements: Folic acid hi taksain thisen cell hrisel a siam theih nan pek a ni.
• Bone Marrow Transplant: Hei hi thalassemia tidam kim thei damdawi awmchhun a ni . Mahse, surgery risky tak leh complex tak a ni. Hei hian damlo nena inmil tak, a tlangpuiin unaupa, donor hrisel tak a mamawh a ni.

Harsatna awm thei te, enkawl dan te, leh dam chhung zawng te zir rawh.

Enkawl that loh chuan harsatna lian ber chu thinlung, thin leh hormone siamtu glands te tihchhiat a ni a, a bik takin iron overload vang a ni. Chuvangin, a ruat anginIron chelation hmanga enkawl hi a pawimawh hle.

Dam rei zawngah chuan thalassemia minor i neih chuan dam rei zawng pangngai tak i beisei thei a ni. Natna hniam tak emaw, na tak emaw i nei a nih pawhin, enkawlna (thisen pek leh iron lakchhuahna) i zawm dik chuan dam rei leh hrisel tak i neih theihna chance tha tak i nei a ni Thinlung natna hi thihna thlentu ber a ni. Chuvangin skip lovin iron removal treatment tih hi a pawimawh hle.

He natna hi ven theih a ni em? Tin, enkawl chhunzawm zel a ngai bawk

Thalassemia hi genetic disease a nih avangin kan veng thei lo. Mahse, genetic testing hian nangmah emaw i kawppui emaw hi thalassemia gene kengtu in nih leh nih loh hriat theih nan a pui thei che a ni. Fa neih i tum a nih chuan he thu hi hriat a pawimawh hle. Thurawn dang dil duh chuan genetic counselor emaw i doctor emaw pan rawh.

Thalassemia i vei chuan thisen test (CBC) neih fo a ngai ang a, iron level enfiah a ngai bawk ang. I doctor chuan kum khatah vawi khat tal i thinlung leh thin hnathawh test tir turin a rawt thei bawk.