Systemic Mastocytosis (SM) hi eng nge ni? Damdawi Ayvakit chungchang i zir ang u.

I doctor chuan natna hlauhawm tak Systemic Mastocytosis tih i nei tih a hrilh che em ? Chu thu i hriat chuan hlauthawng leh lungkham deuhin i inhria mai thei. A pangngai hle mai. Mahse, lungngai suh. Tunah chuan chutiang dinhmun enkawlna tha leh tangkai tak tak a awm ta. Vawiin hian damdawi Ayvakit (avapritinib) i pek tawh chungchang kan sawi dawn a ni. He natna leh damdawi chungchang zawng zawng hi awlsam tak leh chiang takin i hrethiam vek ang u.

Systemic Mastocytosis (SM) hi eng nge ni tak tak?

A awlsam zawngin sawi ila, Systemic Mastocytosis hi kan taksaa cell chi khat ‘mast cells’ an tih chu a lo thang lian lutuk a, hei hi thil awm lo tak a ni. Heng mast cells te hi kan immune system a tel ve an ni. Kan ram defence force te ang bawkin. Ram dang thil taksaah a luh chuan heng cell te hi an thawk chhuak a, ‘histamine’ ang chi chemical an chhuah chhuak thin.

Thosiin a seh che chuan chu hmun chu a hnim a, a sen vek ang tih han ngaihtuah teh, a dik em? Chu chu he histamine avang hian a thleng thin. Mahse Systemic Mastocytosis vei tan chuan heng mast cells te hi taksaah thunun theih lohvin an pungkhawm thin. Vun, ruhro emaw, chhungril lam taksa peng hrang hrang liver leh spleen-ah te an pung khawm thin. Chumi avang chuan taksain allergic symptoms a nei thei a, chu chu a chhan hriat lohvin a awm thei.

"Systemic" tih thumal hian hmun khat chauh ni lovin taksa pum pui a nghawng thei tihna a ni. He natna hi chi hrang hrang a awm a, a na lo, slow- growing (indolent) atanga advanced, cancerous form thlengin a awm a ni.

Engtin nge Ayvakit hi ka tan a tha em tih ka hriat theih ang?

I doctor chuan Systemic Mastocytosis chi hrang hrang i neih tih chiang takin test hrang hrang a nei ang. Chu test result leh i symptoms te chuan Ayvakit hi i tan a tha em tih a hril ang. Tunah hian Ayvakit hi puitlingah Systemic Mastocytosis chi hrang hrang enkawl turin phalna pek a ni.

He Ayvakit damdawi hian engtin nge taksaah hna a thawh?

Ayvakit hi damdawi pawl pakhat, ‘kinase inhibitors’ an tih zingah a tel a ni. Kinases hi enzyme protein chi khat a ni a, kan taksa chhunga cell inkara signal thawn chhuahnaah a pui thin. Heng signal te hi cell than leh inthen darh ang chi thil atan a pawimawh hle.

Systemic Mastocytosis vei tam tak chuan mast cell a protein awm KIT an tih chu mutation an nei a . He mutation hian KIT protein chu a overactive tir a, chu chuan mast cells te chu control theih lohvin a tipung ta a ni.

Ayvakit damdawi hian chu mutated KIT protein chiah chu a target a, a hnathawh chu a titawp ta a ni.. Signal dik lo pe chhuak tower silencing ang maiin. Hei hian mast cells siam tam lutuk chu a titawp a, symptoms a ti tlem a, taksa peng hrang hrang chhiatna a control bawk.

Engtin nge Ayvakit pills hi ka hman ang?

Ayvakit hi pill a ni. A ei dawna a pawimawh ber chu pum ruak a ei tur a ni . Chumi awmzia chu, chaw ei hma darkar khat tal, emaw, chaw ei hnu darkar hnih tal ei tur a ni tihna a ni. Chutah chuan damdawi chu taksain a hip lut tha ber a ni.

Dose pek dan tlangpui chu hetiang hi a ni:

• Advanced Systemic Mastitis TOS (AdvSM) tan chuan nitin vawi khat 200mg ei thin tur a ni.
• Indolent Systemic Mastocytosis (ISM) tan chuan nitin vawi khat 25mg ei thin tur a ni.

Hei hi a tlangpuiin hun rei tak enkawlna a ni. I doctor chuan damdawi chu a atanga result tha tak i hmuh hma leh side effect na tak i neih loh hma loh chuan ei chhunzawm zel turin a hrilh ang che. Side effect i neih chuan i doctor chuan i dose tihhniam emaw, rei lo te chhung tihtawp emaw, tihtawp vek emaw a rel thei a ni.

Ayvakit hlawhtlinna chungchangah hian research-in eng nge a sawi?

Ayvakit damdawi hman tangkai dan leh a him dan zirchiang tura clinical trial pathum neih tawh chungchangah thu hriat tur a awm.

AdvSM (severe) tan chuan: Zirna pahnih, EXPLORER leh PATHFINDER-ah hian Ayvakit ei zinga 57%, a nih loh leh a chanve aia tam te chu an natna lan chhuah dan a tlahniam a, a natna taksa peng hrang hrangah hnathawh dan engemaw zat a lo harh leh bawk. Results lantir zinga a chanve chuan chu hmasawnna chu thla 38 chhung, kum 3 aia tam emaw chhung chu an vawng reng a ni.

ISM (mild type) tan: PIONEER study-ah chuan Ayvakit ei thinte hian placebo ei thinte aiin symptoms (itching, pum na, leh hahna ang chi) an nei tlem zawk tih a chiang hle. Hei hian he damdawi hian nunphung tihchangtlunna kawngah nasa takin thlamuanna a pe tihna a ni.

Heng zirchiannate hian Ayvakit hi Systemic Mastocytosis enkawlna tha tak a ni tih a tarlang. Mahse, natna a tidam kim lo tih hre reng la, a rah chhuah chu mi zawng zawng tan a inang vek dawn lo tih hi hre reng ang che.

Damdawi dangah harsatna a awm thei em? (Drug nena inzawmna) .

Ni e, Ayvakit hian damdawi thenkhat nen a inzawm thei. A bik takin kan liver-a enzyme CYP3A tichhe thei te hi. Damdawi thenkhat chuan taksaa Ayvakit level a tisang thei a, side effect a tisang thei a, damdawi thenkhat chuan Ayvakit level a tihhniam bakah a thatna a ti tlem thei bawk.

Chuvangin i damdawi ei zawng zawng (natna dang damdawi, vitamin, hnam damdawi, supplement) te chu i doctor hnenah hriattir ngei ngei tur a ni. Chumi hnuah chuan harsatna a neih leh neih loh a enfiah thei a, a tul chuan i damdawi dangte chu a thlak thei a, Ayvakit hman dan tur pawh a thlak thei bawk.

Tin, thisen tithianghlimtu, warfarin (Coumadin, Jantoven) , i ei a nih chuan Ayvakit atanga thluaka thisen chhuah theihna i nei tlem thei bawk. Chuvangin, chutiang damdawi i ei a nih chuan i doctor chuan ngun zawkin a lo en reng ang che.

Ayvakit damdawi hi engtin nge kan hmuh theih ang?

Ayvakit hi prescription-only damdawi a ni a, a tlangpuiin hematologist emaw oncologist emaw-in a pe thin.

Hei hi damdawi dawr pangngaia i hmuh theih tur damdawi a ni lo . ‘Specialty pharmacy’ ah lei theih a ni. I doctor chuan chu specialty pharmacy-ah chuan damdawi chu a forward tawh chuan, damdawi chu i in emaw, i doctor office-ah emaw an thawn thei ang.