Hemophilia B hi i hria em? I tichhia ang u!

Mi thenkhat chu hliam tenau tak atang pawhin thisen an chhuak chhunzawm zel tih i hre ngai em? A nih loh leh chhan awm lovin i taksa peng thenkhat chu a sen a, thisen chhuak a awm tih i hre tawh em? Heng thilte chhan pakhat chu vawiin a kan sawi tur dinhmun Hemophilia B. Lungngai suh, he thu hi i hriatthiam theih tur awlsam takin kan sawi dawn a ni.

Hemophilia B hi eng nge ni?

A awlsam zawngin sawi ila, hemophilia B hi thisen chhuahna natna a ni a, rochun a ni. Kan thisen zam tura puitu gene pakhata mutation a awm chuan a thleng thin. Hemophilia B vei te hian thisen zam tura puitu protein pawimawh tak factor IX (factor 9, F9, FIX tia hriat bawk) an nei tlem hle. Enkawl loh chuan he natna hi nunna atana hlauhawm tak a ni thei. Thu lawmawm tak chu doctor ten factor 9 thlakna hmangin an enkawl mek a ni. Enkawlna thar, gene therapy ang chi te pawh zirchian mek a ni.

He dinhmun hian engtin nge i taksa a nghawng?

Hemophilia chi dang ang bawkin, hemophilia B vei te hian hliam an neih emaw, an zai emaw, ha lakchhuah emaw an nih chuan an thisen a chhuak tam zawk a, a tlangpui aia rei an chhuak thin. He natna hian mipa a nghawng tam ber a, mahse hmeichhiaah pawh a nghawng thei bawk.

Doctor-te chuan hemophilia hi a na lo, a na lo, a na lo hle niin an sawi . Hemophilia B na tak nei te chu an ruh chhungah thisen a chhuak thei a ni. Hei hi a hrehawm hle a, hun kal zelah arthritis ang chi dinhmun a thlen thei bawk. Mi thenkhat chuan ruh chhia te chu paih chhuakin thlak leh turin operation an mamawh a ni. An thluaka thisen a chhuak thei bawk a, chu chuan nunna a tichhe thei a ni.

Hemophilia B hi engtiang chiahin nge a hluar?

United States-a statistics atanga a lan dan chuan mipa 100,000 zinga 4 velin hemophilia B. Hmeichhia pawhin an vei a, mahse engzat chiah nge tih erawh sawi a harsa hle. A chhan chu heavy period ang chi symptoms nei mahse mi tam tak chuan hemophilia nen a inzawm lo niin an ngai.

Hemophilia A leh B hi eng nge an danglamna i hria em?

Hemophilia A leh B te hi thisen chhuahna lama inherited disorder an ni vek a. A lan chhuah dan chu inang tlang hle mah se, a thlentu genetic mutations erawh a inang lo. Zirna hrang hrangah chuan hemophilia B natna lan chhuah dan hi hemophilia A natna aiin a na lo deuh thei tih hmuhchhuah a ni a, hei hian hemophilia B hi natna khirh tak a ni bawk tihna a ni a, mahse a vei te hian thisen chhuah tam lutuk chungchangah harsatna an nei tlem zawk thei a ni. A danglamna dang thenkhat chu hetiang hi a ni:

• Zirchianna atanga a lan dan chuan hemophilia B vei te hian ruh chhungah thisen a chhuak tlem zawk (hemathroses) a, ruh a tichhia tlem zawk bawk.
• Hemophilia B vei zingah hian mahni insiamthatna (spontaneous bleeding) hi a tlem hle.
• A chang chuan hemophilia enkawl lai hian taksain antibody a siam chhuak a, chu chuan enkawlna a tibuai a ni. Mahse, hemophilia B vei te hian heng harsatna te hi an nei tlem zawk thin.

Engvangin nge hei hi a hmain ‘Krismas natna’ tia koh a nih?

Hei hi thawnthu ngaihnawm tak a ni. Kum 100 chuang zet chu doctor-te chuan hemophilia chi khat chauh a awm niin an ngai a. Mahse kum 1952 khan zirchiangtu pakhat chuan hemophilia vei mi pasarih (pakhat hming chu ‘Christmas’) thisen plasma hmangin hemophilia vei dangte a enkawl a. Mak tak maiin enkawlna chuan hna a thawk ta! Chutih lai tak chuan zirchiangtute chuan Mr. Christmas hian hemophilia chi danglam tak a nei tih an hre ta a, chu chu gene mutation danglam tak avanga lo awm a ni tihna a ni. Chuvangin he hemophilia chi hnihna hi ‘Christmas factor’ emaw ‘Christmas disease’ emaw an ti ta a ni. A hnuah hemophilia B an ti leh ta a ni.

Eng thilin nge hemophilia B hi a thlen?

Sawi tawh ang khan hemophilia B hi genetic bleeding disorder a ni. Hemophilia B i neih chuan factor 9 awm zat tichhe thei gene danglam tak i rochun tihna a ni a, a tlangpuiin F9 an tih gene a awm a, chu chuan factor 9 siam dan a hrilh che a, he F9 gene hi a inthlak danglam a, chu chuan factor 9 level a tihhniam a, a nih loh leh factor 9 a ti bo vek a, gene danglam tak a siam a, a tam zawkah chuan mipa naupangte hian hemophilia B hi an rochun a nih chuan nute hi natna kengtu an ni.

Hetiang hian a thleng thin:

• Kan nu hnen atangin X chromosome pahnih, kan pa hnen atangin X chromosome pakhat leh Y chromosome pakhat kan hmu vek a.
• Hmeichhiain a X chromosome pakhatah F9 gene pangngai lo a neih chuan hemophilia B carrier a ni ang a, mahse symptoms a lantir lo ang. Hei hi a X chromosome dang ah F9 gene pangngai a neih vang a ni.
• He hmeichhia hian a fapa hnenah F9 gene pangngai lo nei X chromosome chu a pe thei a ni. Fapate hian X chromosome pakhat chauh an neih avangin hemophilia B an nei ang.

A châng chuan, nu aṭanga fapaah hian spontaneous mutation hi rochun a ni lo.Hemophilia B hi involution an tih process vang pawh a ni thei bawk. Hei hi egg leh sperm inzawmkhawma embryo siam a, chu chu a inthen darh a, cell thar a siam tan chuan a thleng thin. Heng cell tharte hian cell hmasa lama awm genes copy an keng tel a ni. Chuvângin, a châng chuan heng gene-te hi copy a nih hian tihsual, a nih loh leh mutations a thleng thei a ni. F9 gene copy a nih laia tihsual a awm chuan i fa hian hemophilia B a nei thei a, a nih loh leh hemophilia B kengtu a ni thei bawk.(Historians leh medical researcher-te chuan England lalnu Victoria-i chungah hetiang hi a thleng niin an ngai. A chhungte zingah tumah hemophilia nei lo mah se, hemophilia a vei tak zet a ni. He natna hi a fate hnenah a kai a ni. Chu naupangte chu Spain leh Russia lal chhungkua-a innei chuan an fate pawhin he natna hi an vei ve bawk. A hnuah hemophilia B a ni tih hmuhchhuah a ni leh ta a ni.)

Hemophilia B natna lan chhuah dan chu engte nge ni?

Hemophilia B symptoms hi mild, moderate, leh severe anga classified a nih thu kan sawi tawh a. Doctor-te chuan hengte hi thisena factor 9 level a zirin an thliar hrang thin.

Hemophilia natna nei lo B

Factor 9 level 6% leh 49% inkar nei te hian hemophilia B na lo tak an nei a, a natna lan chhuah dan hi a na lo hle. A châng chuan, mild hemophilia B vei te chu puitling anga hriat an ni a, operation atana an inbuatsaih lai te, naute an neih dawn lai te, hliam na tak an tuar hnuah te, a nih loh leh hmeichhiaah chuan thlasik thisen chhuak nasa tak avanga enkawl an nih lai te pawhin an hmuchhuak thin.

Hemophilia hniam lam B

Factor 9 level 1% leh 5% inkar nei te hian hemophilia B na lo tak an nei a, a natna lan chhuah dan pawh a na lo hle bawk. Hetiang natna vei naupangte hian thla 18 vel atanga an lan chhuah tan tlangpui.

Heng naupangte hian symptoms an nei thei a, chungte chu:

• Bruises: An bruise leh bruise awlsam hle.
• Thisen chhuah dan danglam: Surgery i neih emaw, thisen chhuak hliam emaw, ha lakchhuah emaw i neih chuan a tlangpui aia tam leh rei tak i chhuak thei.
• Mahni thisen chhuak: A tlem berah chuan chhan hriat lohvin thisen chhuah a intan thei.

Hemophilia na tak B

Factor 9 level 1% aia tlem nei te hian hemophilia B na tak an nei a, a symptoms hi a na hle. Naupang thenkhat chu an pian tirh atangin thisen an chhuak tan thei a, an pian hnu rei vak lovah emaw, serh tan hnuah emaw thisen an chhuak tan thei bawk. Naupang dangte chuan an pian hnu thla engemaw zat hnuah symptom an nei lo thei. A lan chhuah dan tlangpui chu:

• Thisen chhuah: Nausen naupang leh naupang tê tête chu an kâah thil te tak te, infiamna ang chi a luh chuan an kâ aṭangin thisen a chhuak thei a ni.
• An lu-a hnim hring (swollen lumps): Nausen te tak te hian hmun dangah an lu a rawn deng a nih chuan goose egg ang maia lumps an nei thei a ni.
• Tap fo, chawlh hahdam loh, emaw, kekawr leh kea kal duh loh:Hengte hi naute hian an taksa chhunga taksa ruh emaw, ruh emaw-ah thisen a chhuah chuan a thleng thei a ni. Hmun thenkhatah chuan a sen leh a hring ang maiin an lang thei. An khawih pawhin an lum thei a, naute pawh a na thei bawk.
• Hematomas: Hematoma chu thisen zam, vun hnuaia awm khawm a ni. Naute pawhin injection a pek hnuah hematoma a nei thei bawk.
• Thâwk lama harsatna: A châng chuan, naupang lei chu thisen chhuah vangin a hring thei a, thawkna kawng a tikhawlo thei bawk.

Doctor-te hian engtin nge hei hi an hriat?

Doctor-te chuan hemophilia B hi taksa enfiahna an neih a, ruhkawr hliam leh hliam ang chi symptoms te an zawng a, chhungkuaah hian hemophilia emaw thisen lama harsatna dang emaw nei an awm em tih an zawt a ni.

Hei bakah hian a hnuaia test te hi tih thin a ni bawk.

• Complete Blood Count (CBC): Hei hian thisen chhunga cell chi hrang hrang leh an awm zat a enfiah thin.
• Prothrombin Time (PT) test: Hei hian i thisen a zang chak zia a test thin.
• Activated Partial Thromboplastin Time test: Hei hi thisen zam a lo awm theih nan eng chen nge a rei tih tehna a ni bawk.
• Fibrinogen test: Thisen zam tura puitu fibrinogen an tih protein awm zat tehna test a ni.
• Clotting factor test: Hei hian hemophilia chi dik tak leh natna nasat dan hriat theihna a pui a ni.

Hei hi eng enkawlna nge ni?

Doctor te chuan hemophilia B hi factor replacement therapy hmangin an enkawl fo thin . Hei hian concentrated factor 9 chu vein pakhatah inject a ni. He concentrated factor 9 hian factor awm lo chu thlak turin hna a thawk a, thisen chhuah tam lutuk tur venna emaw, thisen chhuah a thlen hunah control emaw a pui thin.

A tlangpuiin hemophilia B na lo leh na lo tak nei te hian operation ang chi thil an tih a ngaih hunah chauh he enkawlna hi an mamawh thin. Mahse, hemophilia B na tak vei te chuan factor replacement therapy an mamawh fo thei a ni.

Enkawl lai hian harsatna a awm thei em?

He damdawi ei thinte hi harsatna thenkhat a awm thei bawk. A bulpui ber chu inhibitor leh virus infection lo awm hi a ni .

Inhibitor te pawh a awm

Inhibitor hi taksain factor replacement chu a thisen pangngaia tel anga a pawm tawh loh chuan a lo awm thin. Heng inhibitor te hian factor replacement therapy chu a thawk tha thei lo. Hei hian thisen chhuah tihtawp emaw tihtlem emaw a ti harsa hle. Thisen chhuahna lama harsatna nasa tak nei, factor therapy dose sang tak dawng mekte chuan he harsatna hi an nei tam zawk. Doctor-te chuan factor 9 bypass lovin thisen zam titawp thei thil dang hmangin an enkawl a ni.

Viral natna hrik kai te

A tlem berah chuan hepatitis C ang chi virus infection hi factor substitute hman a nih hian a awm thei a, thisen pek atanga siam a ni. Mahse, tuna test dan hman mek hmang hian he hlauhawmna hi a hniam zawk hle.

Hetiang thil a thlen theihna tur hlauhawmna hi tihziaawm theih a ni em?

Hemophilia B hi rochun natna a nih avangin i fate hnena a lo awm loh nan emaw, i fate hnena a thlen loh nan emaw i veng thei lo. A rochun dan (genetic process) chungchang tlem han zir belh ila:

• Hmeichhia i nih a, i X chromosome pakhatah F9 gene pangngai lo (carrier i ni tihna) i neih chuan i fa mipa te hian hemophilia B vei theihna chance 50% an nei a, i fa hmeichhia te pawhin carrier an nih theihna chance 50% an nei bawk.
• I fanute, he gene rochuntute hian an fapate hnenah hemophilia B hi an pe thei a ni. An fanute hi carrier an ni thei.
• Hemophilia B vei i fapate hian he chromosome hi an fanute hnenah an pe thei a ni.
• Mipa i nih a, hemophilia B i vei chuan i fapate hian he natna hi an nei lovang. Mahse i fanute zawng zawng chu F9 gene pangngai lo nei chromosome carrier an ni vek ang.

Hetiang dinhmunah hian Genetic counselling hi a pawimawh hle. Nangmah emaw, i chhungte tu emaw hian hemophilia an vei a nih chuan fa i neih hmain genetic counselor nen inhmuh hmasak phawt a tha.

Naupai ka nih chuan ka naute hian he natna hi a nei em tih hi ka hre lawk thei ang em?

Ni e, i ti thei. Doctor ten i umbilical cord atanga thisen sample an la thei a, clotting factors a awm leh awm loh an test thei bawk. Chutiang chuan nang leh anni chuan i naute i neih huna i beisei tur leh thisen chhuah lohna tura harsatna awm lo tura i tih tur te chu in hre lawk thei ang.

Hemophilia B ka vei chuan eng nge ka beisei ang?

Hemophilia B hi natna khirh tak a ni. Doctor ten an tidam kim thei lo. Mahse, ruh natna nasa tak leh damdawi lama harsatna dangte venna emaw tihziaawm thei emaw enkawlna a awm. Hemophilia B vei tam zawk chuan enkawlna hmangin hriselna tha tak an nei thei a ni.

Hemophilia B na tak vei te hian thisen chhuah harsatna enkawl nan factor replacement injection (infusion emaw injection emaw) pek fo a ngai a ni. Doctor hnenah enkawl turin an kal thei a, an chhungte emaw, enkawltu emaw injection pe tir emaw, anmahniin injection an pe thei emaw pawh a ni thei. Mahse, hemophilia B na tak vei te hian thisen chhuah tam lutuk loh nan enkawl mumal an ngai a ni.

An hriselna leh dam chhung zawng zawng tichhe thei damdawi lam thil dang pawh an nei thei bawk. Entîr nân, hemophilia B vei tam tak chuan an khup emaw, an hip emaw-ah harsatna nasa tak an nei a, an ruh chhia chu la chhuakin, ruh thar hmanga thlak tûrin operation an neih a ngai a ni. Hemophilia B i vei chuan i doctor hnenah eng nge i beisei tih zawt la. I dinhmun leh i hriselna zawng zawng a hriat avangin information tha ber pe thei tur che mi tha ber a ni.

Ka fa hian he natna hi a nei a nih chuan eng nge ka hriat tur?

I fain hemophilia B na lo emaw, na lo emaw a neih chuan, i fain operation emaw, ha lakchhuah emaw a neih chuan thisen chhuah tam lutuk an ven theih nan i doctor hnenah a natna chungchang hriattir a pawimawh. Hetah hian rawtna dang kan rawn tarlang e. I doctor hian rawtna dang a nei thei ang:

• I fate an naupan lai chuan an high chair leh car seat-ah safety belt mumal tak a awm em tih enfiah thin ang che.
• Tlem an lo puitlin a, naupang dang nena an inkhelh hunah chuan an enkawltu leh sikul zirtirtuten naupang chu a hliam palh a, thisen a chhuah tan chuan eng nge an tih tur tih an hriat a ngai a ni.
• I fa chu thil ṭhenkhat, mi dangte nêna inbiak pawhna nasa tak emaw, tlu theihna emaw ang chi infiamna a\angin a inthiarfihlim a ngai ang.

Ka fa hian hemophilia B na tak a neih chuan engtin nge ni ang?

Hemophilia B na tak nei naupangte hian an dam chhung zawng damdawi lam enkawlna an mamawh dawn a ni – thisen chhuah loh nan emaw, tihtlem nan emaw, a natna lan chhuah dan tur enkawl nan emaw. I hmachhawn theih tur harsatna ṭhenkhat, leh ṭanpui thei tûr rawtna ṭhenkhat chu hetiang hi a ni:

• Hemophilia B vei naute hian kal an zir lai hian an bump emaw, an tlu emaw chuan thisen a chhuak tan thei a ni. Tlu zawng zawng hi ven theih a ni lo va, mahse in chhunga furniture kil hrual hrual takah chuan venhimna khuhna dah a tha.
• I fa a lo puitlin a, a tlan leh a zuang tan chuan i doctor nen safety gear helmet leh knee pads te chungchang sawipui rawh. Dik tak chuan naupang zawng zawng hian bike kan khalh dawnin helmet an bun vek tur a ni. I fa chu thisen chhuak thei khawpa inhnaih loh nan venhimna dang a mamawh mai thei.
• I fa hian thisen chhuah tihtawp nan damdawi lam enkawlna mumal tak a neih a ngai dawn a ni. Inenkawl tura kal a ngaih hunah a ṭhiante nêna hun hman theih loh leh sikul hna a thawh loh avângin a thinrim a, a lungawi lo thei bawk.
• Hemophilia natna vei naupang chuan a dinhmun hi a zirtirtute leh midangte pawhin an hria tih a hria a. Zirtîrtute leh sikula mi dangte’n ṭanpui an tum a, mahse a danglam zâwnga an cheibâwl chuan a lungawi lo mai thei.
• Naupang leh ṭhalai upa zawkte chuan an rilru put hmang hmachhawn tûr leh mi dangte chhân lêt dân hmachhawn tûrin ṭanpuina an mamawh mai thei. I fa pawh chutiang dinhmunah chuan a ding a nih chuan, i doctor nen thalai te tana programme emaw, support group emaw chungchang sawipui rawh.

Hemophilia B ka vei a, engtin nge ka in enkawl ang?

Hemophilia B natna vei tih awmzia chu i hriselna zawng zawng humhim turin hmalakna dang lak a ni a, chutih rualin i enkawlna pawh ngaihtuah tel bawk. Rawtna thenkhat chu hetiang hi a ni:

• Infection lakah inveng rawh: I doctor hnenah eng vaccine nge i tan a tha tih zawt rawh.
• I taksa rihna hrisel tak vawng reng rawh: I chhungril thisen chhuak leh ruh natna avanga i kal kual harsat chuan i rihna control hian a pui thei a ni.
• Exercise tih danah lut rawh: Exercise i tih laiin hliam i hlau mai thei. Active laiin thisen chhuah theihna tihtlem dan tur i doctor nen sawiho rawh.
• I stress kha enkawl rawh: Hemophilia B hi dam chhung zawnga natna a ni. Chhungkaw leh hnathawhna mawhphurhnate nêna inthlau tûrin theihtâwp chhuah belh a ngai thei a ni.
• Painkiller thenkhat ei loh tur: Hemophilia B i vei chuan aspirin emaw ibuprofen emaw ei loh tur a ni. Heng painkillers te hian thisen zam a tibuai thin.

I enkawlna tur ruahman leh engtik lai pawhin i natna lan chhuah dan, thisen chhuah danglam tak emaw, ruh natna nasa tak emaw i neih chuan doctor i pan tur a ni.

Engtikah nge emergency treatment i kal a ngaih?

Lu hliam i neih chuan , damdawi lam pan nghal tur a ni. Heng chhinchhiahnate hian i thluaka thisen chhuak (intracranial hemorrhage) i nei tih a tilang thei a ni:

• Lu na.
• Chaklohna.
• Nausea leh luak chhuak.
• Numbness emaw paralysis emaw.

Hliam chi hrang hrang atanga thisen chhuak i chawlhsan thei lo a nih chuan, emaw, chhan awm lova thisen chhuak i tan chuan doctor hmu emaw, emergency room-ah emaw kal nghal rawh.

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Hemophilia B hi natna hmuh tur awm lo dang a ni. Hemophilia A ang bawkin hemophilia B hi thisen chhuahna natna rochun a ni. Zirna hrang hrangah hemophilia B vei te hian hemophilia A vei te aiin natna lanchhuahna na lo zawk an nei thei tih a tarlang a, mahse hemophilia B hi damdawi lam, nunphung leh rilru lama harsatna engemaw zat thlentu a la ni reng a ni.

Hmeichhia he natna kengtu te hian an fate’n an rochun ang tih an hlau mai thei. Hemophilia B vei naupang nu leh pate chuan an fa chu accident-a hliam tuar lo tura venhim an ngaihtuah mai thei. Nakinah chuan an fa puitling chu hemophilia B natna vei zir turin an puih a ngai mai thei a, hemophilia B na tak vei te hian thisen chhuah tam lutuk lo turin dam chhung enkawlna an mamawh mai thei.

Mahse, beiseina a awm! Zirchiangtute chuan enkawlna thar, gene replacement leh gene therapy te chu an chhui mek a ni. Hengte hian hemophilia B na tak enkawl danah danglamna nasa tak a thlen thei a ni. Nang emaw i fa emaw hian he natna hi i neih chuan i doctor hnenah i tan a tha thei tur clinical trial chungchang zawt rawh. Damdawi lam thurawn leh enkawlna dik tak i neih chuan hemophilia B natna vei hian nun hlawhtling leh hrisel takin i nung thei tih hre reng ang che.

` Hemophilia B, thisen chhuak, genetic natna, thisen zam, Factor IX, thlahtu natna, Krismas natna