Haemophilia chu eng nge ni? A chanchin awlsam takin i zir ang u.

Hliam te tak te i neih chuan rei vak lo hnuah thisen chhuah chu a reh a ni lawm ni? Chu chu kan taksaa defense mechanism mak tak avang a ni. Mahse mi thenkhat tan chuan he thisen zam (blood clotting process) hi a thleng tha tawk lo. Hliam te tak te pawhin thisen chhuak chhunzawm zel a thlen thei. Vawiin hian hemophilia kan sawi dawn a, hei hi thisen zam tha lo tak, thlahtute atanga lo chhuak a ni.

Engtin nge hemophilia ka vei tih ka hriat theih ang?

A awlsam zawngin kan taksain thisen a chhuah tan chuan chemical reaction chain-in a titawp tan thin. Chumi atanga a tawpah chuan thisen zam (Fibrin clot) a lo awm ta a ni. Kan thisena protein bik engemaw zat hian he thisen zam hi siam turin a pui thin. Hengte hi "clotting factors" emaw Clotting Factors emaw kan ti thin.

Hemophilia hi thisen zam tirtu zinga pakhat, a bik takin Factor VIII emaw Factor IX emaw a bo emaw, a tamna a tlahniam emaw a ni. Chumi awmzia chu thisen chu a pangngaiin a clot lo tihna a ni.

Doctor-in hemophilia i vei nia a rinhlelh chuan thisen test vawi tam tak an pe ang che a, i thisen a zam ṭhat dan an enfiah ang. Heng test hrang hrangte hi:

• I thisen sample lak a ni a, lab-ah chemical bik nen chawhpawlh a, chutah chuan thisen zam a lo awm em tih enfiah a ni a, a lo awm a nih chuan engtiang chiahin nge a lo awm rang tih enfiah a ni.
• Heng test te hi a dik lo a nih chuan i thisena Factor VIII leh Factor IX level tehna tur specialized blood test tam zawk i nei ang.

Hemophilia hi eng enkawlna nge ni?

Hemophilia hi enkawlna hrang hrang a awm a. I mamawh enkawlna chi chu hemophilia i neih chi leh a nasat danah a innghat ang.

Entirnan, hemophilia na lo tak i neih chuan hliam i neih emaw, operation atana inbuatsaih emaw chauhvin enkawl i mamawh mai thei. Mahse, hemophilia na tak i vei a, thisen i chhuah fo chuan thisen chhuah loh nan leh i ruhte chu pianphung leh chak lohna laka humhim turin enkawl mumal i mamawh mai thei.

Enkawl dan tlangpui chu hetiang hi a ni.

Damdawi hman bikte hi ngun zawkin han thlir ila.

Hemophilia A vei te chu factor VIII pek an ni a, hemophilia B vei te chu factor IX pek an ni bawk. Heng thilte hi kawng lian pahnih hmanga siam a ni:

1. Thisen plasma pek chhuah

2. Synthetic/Recombinant DNA atanga lak chhuah

Tunah chuan factor chi thar zawk chu taksaah rei zawk awm thei tura duan a ni tawh a, chu chu a hma aiin injection tlem zawk a ngai tihna a ni.

Thil dang leh enkawl dan tur

Hemophilia nena lo awm harsatna dang engemaw zat pawh kan ngaihven a ngai bawk.

Thisen chhuak ruh

Hei hi hemophilia natna khirh ber a ni. Khup, khup, ke ruh ang chi ruh (joint)-a thisen chhuah a nih chuan factor therapy lak nghal a pawimawh hle. Chutianga tih a nih loh chuan ruh chu a chhe vek thei a, hun kal zelah a na a, a hring a, a che harsa thei bawk. Thisen a chhuah chuan doctor-in a tih angin chawlh la, ruh chu ice la. Natna leh hliam a reh hnuah chuan ruh chhunga che leh chakna neih leh theih nan physiotherapy hi a pawimawh hle.

Taksa tihchakna

Hemophilia neih hian in lama awm reng a ngai tihna a ni lo. Mahse, hliam tuar thei contact sports atanga inthiarfihlim hi a finthlak hle. Entirnan, infiamna rugby leh boxing te hi a inhmeh lo hle. Amaherawhchu, tui chawi, kea kal, leh cycle khalh (a him taka) ang chi exercise te hi taksa tan a tha hle. I doctor nen eng exercise nge i tana tha tur leh i tih loh tur tih sawipui a tha ber.

Enkawlna hun chhunga harsatna awm thei te chu engte nge ni?

Tuna enkawlna hman mekte hi a him hle nachungin, harsatna pahnih hriat tur a awm.

1. Thisen atanga kai natna: Tun hma kum 1970 leh 1980 chho vel khan thisen hmanga factor therapy hmanga HIV leh Hepatitis ang chi virus kai theihna a sang hle. Mahse tunah chuan chutiang lam ngaihtuah a ngai tawh lo.Tunlai hian thisen petute hi uluk takin screening an nei thin. Thisen zawng zawng hi virus awm leh awm loh enfiah vek a ni a, chumi hnuah damdawi atana hman hmain inactivate turin special process hmanga enkawl a ni. Chuvangin tunah chuan risk a tlem hle. Mahse, hemophiliac kan nih angin hepatitis A leh B laka vaccine lak a pawimawh hle.

2. Immune system inthlak (Inhibitors): A chang chuan, kan taksa immune system hian pawn lam atanga factor protein kan pek chu ‘hmêlma’ tiin a tisual a, a bei tan thin. Hei hi inhibitor siamna kan ti a . Tichuan factor therapy kan pek hian hna a thawk tha lo. Hei hi a buaithlak deuh a ni. Chuvangin i doctor chuan i (or i fa) thisen chu hetiang ang hi a awm em tih enfiah fo ang che.