Cut te tak te atang hian thisen i chhuak nasa em? Hemophilia chungchang hi han sawi ila

Nangmah emaw, i chhungte zinga mi emaw, hliam tenau i neih hian thunun theih lohvin thisen chhuak ang maiin i inhriat ngai em? A nih loh leh hliam tlemte i neih hnuah pawh i taksaah blue spot lian tak tak i nei em? Heng thilte hi thil pangngai angin hnawl suh. A chhan chu, hei hi hemophilia tih hming pu dinhmun chhinchhiahna a ni thei a ni. Ngaihtuah buai suh, vawiinah hian thumal awlsam takin engkim kan sawi dawn a ni.

A awlsam zawngin, hemophilia hi eng nge ni?

Hemophilia hi genetic condition a ni a , kan thisen zam dan a nghawng a ni . A tlangpuiin kan hliam hian rei vak lo hnuah thisen chhuah hi a reh thin. Hei hi kan thisena protein bik ( clotting factors an tih) te hian an thawk dun a, thisen zam (blood clot) an siam vang a ni. Mahse, hemophilia vei te hian thisen zam tura puitu heng protein zinga pakhat hi an tlachham a ni. Hei vang hian kan hliam hian thisen chhuah hi a reh awlsam lo.

Hemophilia hi chi hnih a awm a.

Tin, hemophilia C an tih chi hi a tlem hle a, Factor XI tlakchham vang a ni.

Hemophilia natna lan chhuah dan chu engte nge ni?

A natna hi a nasat dan azirin symptoms a inang lo. A châng chuan chhan hriat lohvin thisen a chhuak thei bawk. Heng chhinchhiahnate hi awlsam takin hriat theih a ni a, a bik takin naupang tê têteah chuan .

Naupang tê leh naute zînga natna lan chhuah dan: 1.1.

• Pian tirh laia lu chhunga thisen chhuak: Nausen thenkhat chuan an pian tirh lamah lu chhungah thisen a chhuak thei.
• Kal tan tirh a ruh a hring: I fate ruh, khup leh kekawrte chu a kal tan emaw, a kekawr emaw a tan veleh a hring a, a sen vek a nih chuan i ngaihtuah tur a ni.
• Hriselna te tak te atang pawhin blue spot lian tak tak: Bump te tak te pawh hian taksaah hian spot lian tak tak, dum dum a rawn lang thei a.
• Hnar thisen chhuak leh ha zung thisen chhuak: Ha chhuah lai emaw, ha sil lai emawa hmui atanga thisen chhuak fo.
• Zun emaw ek emaw-ah thisen a awm .

Hemophilia na lo tak nei chuan puitling a nih thlengin a lan chhuahna a nei lo thei. He natna hi a chang chuan ha lak chhuah ang chi thil tenau tak takah thisen chhuah tam lutuk a awm chuan hmuhchhuah a ni.

Engtin nge he natna hi a lo awm?

Hei hi a buaithlak deuhin a lang thei a, mahse hriatthiam a awlsam hle. Hemophilia hi genetic natna a ni a . Hei hian nu emaw pa emaw atanga lo chhuak genes chhiatna avanga lo awm a ni tihna a ni.

• Nu atanga fapa thlengin: Hemophilia thlentu gene chhia chu X chromosome-ah a awm a. Naupang mipa (fapa) chuan a nu hnen atangin X chromosome a dawng a, a pa hnen atangin Y chromosome a dawng bawk. Chuvangin nu chu natna kengtu a nih chuan, chu chu a X chromosome pakhatah he chhiatna hi a nei tihna a nih chuan, a fapain a hnen atangin X chromosome chhia chu a dawng theihna chance 50% a awm a ni. Chutiang a nih chuan fapa chuan hemophilia a nei ang.
• Hmeichhia (fanu) te chu carrier an lo ni ta: Naupang hmeichhia chuan X chromosome pahnih (pakhat chu a nu hnen atangin, pakhat chu a pa hnen atangin) a dawng a. A nu hnen atangin X chromosome chhia chu dawng mahse, a pa hnen atanga X chromosome hrisel tak a hmuh avangin natna hi a nei lo tlangpui. Mahse, natna phurtu an lo ni ta a ni . Chumi awmzia chu an fate hnenah natna an kai thei tihna a ni.
• Pain hemophilia a neih chuan: Hemophilia vei pa chuan a fapate hnenah natna hi a kai ngai lovang a chhan chu fapate hian an pa hnen atangin Y chromosome chauh an rochun thin a ni. Mahse, a fanute zawng zawng hian a hnen atangin X chromosome chhia chu an rochun vek avangin chu fanu zawng zawng chu natna kengtu an ni vek dawn a ni.

A châng chuan naupangin a taksaah hemophilia nei lo mah se, mahni insiam danglam (spontaneous mutation) hmangin natna hi a nei thei a ni.

Engtin nge natna hi kan hriat theih ang? (Diagnosis) tih a ni.

Hetiang dinhmun hi pian hnu lawkah hriat chhuah a ni tlangpui. Chhungkaw chanchin enin, naute thisen chhuah dan danglam tak a neih chuan hriat theih a ni. Naute pian hnua umbilical cord atanga thisen sample lak pawhin test theih a ni.

Nangmah emaw, i fa emaw hian heng symptoms te hi i neih chuan Doctor pan nghal tur a ni. Doctor chuan i chhungte zingah hetiang natna hi an nei em tih a zawt hmasa ang. Tichuan thisen test engemaw zat an ti ang.

• Thisen zam test: Hengte hian i thisen a khalh chak dan leh a khalh that dan a enfiah thin.
• Factor Assay: Harsatna a awm tih hmuhchhuah a nih chuan he test hian eng hemophilia chi nge i neih (A emaw B emaw) tih leh a nasat dan chiang takin a hriat theih a ni.

Enkawlnate chu engte nge ni?

Hlauh tur a awm lo, tunlai hian hemophilia enkawlna tha tak tak a awm. A tum ber chu taksaa thisen zam (blood clotting factor) tlakchhamna siam that leh hi a ni.

• Clotting Factor Replacement: Hei hi a enkawlna ber a ni. Factor VIII hi hemophilia A tan thisenah pek a ni a, Factor IX hi hemophilia B tan thisenah pek a ni a, hei hi damdawi inah tih theih a ni a, a nih loh leh in lama ti turin training i pe thei bawk.
• Hormone hmanga enkawlna: 1.1.Desmopressin hi mild hemophilia A enkawlna atana pek a ni a, taksain Factor VIII a chhuah tir a, a tichak a ni. Injection angin emaw, hnar spray angin emaw a awm thei.
• Anticoagulants: Heng damdawi te hian thisen zam chu a hmin rang lutuk loh nan a titawp a ni. Ha lakchhuahna ang chiah hian an hmang thin.
• Physical Therapy: Joint-a thisen chhuak fo hian an che thei lo thei a ni. Physical therapy hian chutiang ruhte hnathawh chu a siam tha leh thei a ni.

Hemophilia avanga harsatna awm thei te

Enkawlna mumal an dawn loh chuan harsatna engemaw zat a awm thei a, a bik takin hemophilia natna khirh tak takah chuan.

• Joint damage: Khup, elbow, leh ankles ang chi joint-a thisen chhuak fo hian a tichhe vek thei a, a na a, a che harsa thei bawk.
• Thluak chhunga thisen chhuak: Hei hi harsatna hlauhawm ber a ni. Thluak chhunga thisen chhuah hi lu hliam vang emaw, a nasat ber ṭhenkhatah chuan chhan hriat loh vang emaw a ni thei. Hei hian chak lohna nghet tak a thlen thei a, thihna pawh a thlen thei bawk. Lu hliam na tak i neih chuan damdawi in pakhata Emergency Department (ETU) pan nghal tur a ni.
• Thâwk harsa: Thâwk laiah thisen a chhuak a nih chuan a hring hian thawk harsatna a thlen thei a ni.

Fa neih i tum a, chhungkaw history-ah hemophilia i vei a nih chuan genetic counseling i la thei ang. Hei hian carrier i nih leh nih loh leh i fa tana i risk chu eng nge ni tih i hriat theih nan a pui thei che a ni. Tin, embryo hrisel thlangin in-vitro fertilization ang chi hmangin i nausen pum chhungah dah theih a ni bawk.