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Pakhatnaah chuan, engvangin nge hemophilia-ah hian thisen chhuah hi a tawp loh?
A awlsam zawngin sawi ila, kan thisen zam tura puitu protein bik a awm a. Hengte hi " clotting factors " kan ti a ni. Wall siam dawna brick inzawmkhawmna atana mortar mamawh ang bawkin, heng clotting factors te hi thisen chhuah tihtawp nan a pawimawh hle. Hemophilia vei chuan heng clotting factors zinga pakhat hi a tlachham a ni.- Hemophilia A vei te hian Factor VIII an tih hi an tlachham thin.
- Hemophilia B vei te hian Factor IX an tih hi an tlachham thin.
Chuti a nih chuan he damdawi thar Qfitlia (fitusiran) tih hi eng nge ni?
Hei hi damdawi a ni a, mak tak maiin hna a thawk a ni. Thisen a clot theih nan thrombin an tih thil a mamawh thu kan sawi tawh a. Kan taksa ngeiah hian he thrombin siamchhuahna control tu protein dang a awm a, chu chu thrombin tam zawk siamchhuahna a titawp tihna a ni. Antithrombin an ti a .Hetiang hian han ngaihtuah teh. I taksaah thisen zam (blood clotting factor) (thrombin) i nei a, chu chuan i thisen a tikhawlo thei a ni. Mahse thil dang (antithrombin) a awm a, chu chuan a control a, a thawk thei lo. Hemophilia-ah hian a tir lamah chuan a puitu (thrombin) siam chhuah a tlem hle. Chuvangin a vengtu factor (antithrombin) hian hna a thawh dan pangngaiin thisen zam chu a tlahniam lehzual thin. Qfitlia hian a tih tur chu a vengtu antithrombin a tihtlem hi a ni. Tichuan, i taksain a pianphung a siam chhuah thrombin zat chu tihbuai lohvin a thawk tha thei ta a ni. Chumi awmzia chu taksa system ) hmangin thisen zam (clotting factor) i hloh tawh chu pe lovin, thisen zam (clotting factor) a fuih zawk tihna a ni. Hei hi hemophilia enkawlna atana antithrombin tihtlem thei damdawi hman hmasak ber a ni.Heng enkawl dan pahnih inthlauhna hi han sawifiah belh leh ila.
He danglamna hi a hnuaia table atang hian awlsam takin i hrethiam thei ang.| Mihring ziarang | Hmanlai enkawlna (Factor Replacement) 1.1. | Qfitlia (fitusiran) hmanga enkawl a ni |
|---|---|---|
| Hnathawh dan tur | Taksa chhunga clotting factor tlachham chu pawn lam atanga pek a ni. | Thisen zam tur venna antithrombin a tihtlem bakah taksain a thrombin a hmang tam zawk thei bawk. |
| Engtin nge kan pek ang | Vawi tam tak chu thisen kaltlangin. | Vun hnuaiah (subcutaneous) a awm. |
| Zinzia | Kar khatah vawi eng emaw zat a ngai mai thei. | Thla khatah vawi khat emaw thla hnih danah emaw (kum khatah vawi 6-12). |
| Inhibitor nei tan chuan | Normal factor therapy hian hna a thawk lo va, enkawlna bik dang (bypassing agents) a ngai a ni. | He enkawlna hi inhibitor (antibody against the factors) nei te tan pawh hlawhtling takin hman theih a ni. |
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Hemophilia A emaw B emaw i vei a, kum 12 aia upa i nih chuan i doctor chuan he damdawi hi i tan a tha em tih a ngaihtuah thei ang. A pawimawh ber chu factor therapy hmasa lama inhibitor (resistance) nei tawhte tan pawh hman theih a ni. Mahse, he enkawlna hi i tan hmain i doctor chuan thisen test vawi engemaw zat a nei ang . 1. Antithrombin level: Hei hian i thisena antithrombin awm zat a tehna a ni. Qfitlia dose hi he value atanga teh a ni. 2. Liver function: I liver hi enkawl hma leh enkawl lai hian enfiah thin a ni. He damdawi hi vun hnuaiah (subcutaneously) injection angin pek thin a ni. Chumi awmzia chu vein-ah pek a ni lo tihna a ni. Nang emaw, enkawltu emaw chuan training zawhah in lamah i pe thei ang. Pum, kekawr vun emaw, kut chunglam hnunglam emaw vunah pek thin a ni. Hliam emaw, hliam emaw awmna hmun chu i pumpelh tur a ni. Hemophilia damdawi dang i ei tawh a nih chuan Qfitlia i tan hnuah chuan chutiang enkawlna chu i chawlhsan emaw, i thlak emaw a ngai mai thei. I doctor chuan hei hi chiang takin a hrilhfiah ang che. Tuna i damdawi ei mek hi chawlhsan ngai suh la, doctor thurawn tel lovin Qfitlia ei kha titawp ngai suh. Chutianga i tih chuan thisen chhuah theihna a tipung thei a ni.Side effects pawh kan hre tur em ni? Engtin nge kan ven theih ang?
Damdawi dang ang bawkin Qfitlia hian side effect engemaw zat a nei thei. Anmahni hriat chian a pawimawh a, an venna atana hma lak a pawimawh.- Thisen zam: He damdawi hian thisen zam a tichak avangin thisen kalna kawng chhungah duh loh zawng thisen zam a lo awm hi a tlem hle. He hlauhawmna tihziaawmna turin i doctor chuan i antithrombin level atana dose dik tak a ruat ang.
- Liver enzymes sang: Mi thenkhat chuan liver enzymes sang an nei thei . Hei vang hian doctor te chuan thisen test hmangin liver dinhmun hi an enfiah fo thin a ni .
- Gallstones leh Gallbladder Inflammation: I pum natna nasa tak, rilru hahna, leh luak chhuak ang chi symptoms i neih thut chuan doctor hnenah hriattir nghal rawh.
- Infection: Injection na hmunah hian natna kai theihna a tlem hle. Chuvang chuan engtik lai pawhin fai tak leh doctor-in a tih angin inject reng a pawimawh. Faina dan tha, kut sil fo te pawh a pawimawh hle.
Take-Home Thuchah a ni
- Qfitlia (fitusiran) hi hemophilia A leh B enkawlna thar a ni a, kawng danglam takin hna a thawk a ni.
- Hei hian taksaa thisen zam factor hniam tak chu pawn lam atanga pek ai chuan thisen zam tikhawlotu antithrombin a tihtlem avangin hna a thawk a ni.
- He damdawi hi vun hnuaia injection a ni a, vein chhungah injection a ni lo va, a tlangpuiin pek tlem a ni a, thla khatah vawi khat emaw thla hnih danah emaw pek a ni.
- I doctor chuan thisen test vawi tam tak i neih hnuah he enkawlna hi i tan a tha em tih a hre chiang ang.
- Side effects awm lo turin doctor-in a tih angin thisen test (a bik takin antithrombin leh liver function) hun bi neia neih a pawimawh hle.
- Eng thil vang pawha damdawi lam thurawn tel lova Qfitlia enkawlna tihtawp loh tur.
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