Thisen chhuah tihtawp pawh i harsat lo em ni? Hemophilia chungchang hi i zir dawn teh ang!

Hemophilia an tih natna hi i hre tawh em ? He hming i hriat hian i hlauthawng deuh mai thei. Mahse, lungngai suh, hei hi natna hmuh tur awm lo tak a nih avangin. Vawiin hian hemophilia hi eng nge a nih tih te, a lo awm dan te, a natna lan chhuah dan te, enkawlna a awm leh awm loh te kan sawi dawn a, chu zawng zawng chu i hriatthiam theih tur awlsam takin kan sawi dawn a ni.

Hemophilia hi eng nge ni?

A awlsam zawngin sawi ila, hemophilia hi thisen atanga lo piang, naupangte zingah a lo awm thin a ni. Chumi awmzia chu i thisen a clot tha lo tihna a ni. Hei hian thisen a chhuah awlsam zawk thei a, a nih loh leh awlsam zawkin a hliam mai thei bawk.

Ngaihtuah teh, kan taksaah hian kan thisen zam tura puitu protein bik a awm a. Hengte hi ‘clotting factors’ kan ti a ni. Sipai te tak te, thisen chhuah tihtawp tura thawk ang mai an ni. Hemophilia i vei hian taksain heng ‘clotting factors’ pakhat emaw a aia tam emaw a siam chhuak tlem hle. Tichuan thisen a kham tlem a, thisen a chhuak nasa hle.

Hemophilia hi chi hrang hrang a awm a. I thisenah clotting factors awm zat a zirin a na thei a, a na lo thei a, a na lo thei bawk .

Thu lawmawm tak chu hei hi enkawl dan tur a awm tih hi a ni. Doctor-te chuan clotting factors hniam tak takte chu taksaah dah leh an tum a ni. Tunah hian hemophilia hi damdawi a la awm lo. Mahse, he natna vei te hi midangte enkawl dan tur dik tak pek an nih angin an dam rei tlangpui. Scientist-te pawhin hmanraw thar, gene therapy , tihdam theih a nih leh nih loh an zirchiang mek bawk.

Pian hnuah hemophilia hi a lo awm thei em?

Ni e, a thleng thei. Mahse a tlem hle. Hei hi ‘acquired hemophilia’ an ti a. Chu chu rochun a ni lo tihna a ni. Heta thil thleng hi kan taksa ngeia defense proteins , antibodies an tih te hian a chang chuan kan thisen zam tirtu pakhat chu a rawn bei sual fo thin. Heng antibodies dik lo taka beitute hi ‘autoantibodies’ an ti a.

Hemophilia hi natna tlanglawn tak a ni em?

Ni lo, a ni lo tak zet. Hei hi natna hmuh tur awm lo tak a ni. United States-a statistics (CDC August 2022) atanga a lan dan chuan chutah chuan mi 33,000 velin hemophilia an vei a ni. Mipa a nghawng tam ber. A tlem berah chuan hmeichhiate hian thisen zam (blood clotting factor level) hniam leh thlasik laiin thisen chhuak nasa ang chi symptoms an nei thei bawk.

Hemophilia chi hrang hrangte chu engte nge ni?

Hemophilia hi chi thum a awm a, chungte chu:

• Hemophilia A: Hei hi a tam ber a ni. Thisen zam factor number 8, factor VIII tia hriat bawk a tlakchham vang a ni., taksain a neih tlem lai hian. Mi 100,000 zinga 10 velin hetiang hi an nei thei.
• Hemophilia B: Hei hi thisen zam factor 9, a nih loh leh factor IX tlakchham vang a ni. Mi 100,000 zinga 3 velin he chi hi an nei a ni.
• Hemophilia C: Factor XI deficiency an ti bawk a, hei hi a tlem hle a, mi maktaduai khat zinga pakhat velin a nghawng a ni.

Hemophilia hi natna khirh tak a ni em?

Ni e, a châng chuan hei hi thil pawi tak a ni thei. Hemophilia na tak nei te hian nunna atana hlauhawm takin thisen an chhuah thei a, chu chu ‘thisen chhuak’ kan ti a ni. Mahse a tam zawkah chuan taksa ruh emaw, ruhkawr emaw-ah thisen an chhuak thin.

Hemophilia natna lan chhuah dan chu engte nge ni?

A lan chhuahna pawimawh ber chu thil danglam emaw, thisen chhuah tam lutuk emaw, hliam na lutuk emaw a ni.

A lan chhuah dan tlangpui

Hemophilia vei te hian accident tenau tak tak atang pawhin hliam lian tak tak an nei thei a ni. Chumi awmzia chu vun hnuaiah thisen a leak tihna a ni.

• Operation hnuah te, ha lakchhuah hnuah te, a nih loh leh kut zungtang hliam mai mai emaw pawh ni se , thisen chhuahna chu a tlangpuiin hun rei tak a awm thei a ni.
• Hnar thisen hi chhan awm lovin a lo awm nghal thei a ni.

Bruising/bleeding i neih zat chu hemophilia na tak, a hniam lam, a na lo tak i neih leh neih lohah a innghat a ni.

• Hemophilia na tak nei te hian chhan hriat lohvin thisen an chhuak fo thei.
• Hemophilia natna (moderate hemophilia) vei te hian accident na tak an tawk a nih chuan hun danglam takin thisen an chhuak thei a ni.
• Hemophilia na lo tak nei te hian operation lian tak emaw, accident lian tak emaw an neih hnuah chauh thisen chhuah dan pangngai lo an nei thin.

Symptom dang pawh a awm thei bawk:

• Taksa chhunga thisen inzawmkhawm avanga ruh natna . Kawngpui, khup, hip leh kekawrte ang chi hmunte chu a na thei a, a hring thei a, khawih pawhin a lum thei bawk.
• Thluak ah thisen a chhuak . Hei hi hemophilia na tak nei tan chuan a thleng tlem hle. Thluak-a thisen chhuak i neih chuan lu na reng reng, mit hmuh lohna, emaw, mutthilh lutuk emaw i nei thei a ni . Hemophilia i vei chuan heng symptoms te hi i neih chuan doctor i pan nghal tur a ni.

Nausen leh naupang tê têa natna lan chhuah dan

A chang chuan nausen mipa serh tan a, a tlangpui aia thisen a chhuah chuan hemophilia hi hmuhchhuah a ni. A dangah chuan naupangte hian an pian hnu thla engemaw zat hnuah symptoms an nei thin. A lan chhuah dan tlangpui chu:

• Thisen chhuak: 1.1.Naute hian infiamna te tak te an seh hian an kâ aṭangin thisen a chhuak thei a ni.
• Lu chunga hnah hring (swollen lumps): Nausen leh naupang tê tête chuan an lu an vuak hian lump lian tak tak, zungbun (goose eggs) an nei thei.
• Tap fo te, rilru hahdam lohna te, leh kekawrte emaw, kea kal emaw duh lohna: Hengte hi taksa ruh emaw, ruhkawr emaw-ah thisen a lut a nih chuan a thleng thei a ni. Chu hmun chu a hliam, a hring, khawih pawhin a lum a, naute chu khawih tlem pawhin a na thei bawk.
• Hematomas: ‘Hematoma’ tih hi nausen leh naupang tête vun hnuaia thisen zam khawlkhawm a ni. Heng hematomas chi hrang hrangte hi injection hnuah pawh a awm thei bawk.

Hemophilia hi engte nge ni?

Heng ‘clotting factors’ te hi kan taksa chhunga gene thenkhat atanga siam a ni. Hereditary hemophilia-ah hian heng ‘clotting factors’ siam chhuahna tur kaihruaitu genes te hi an inthlak danglam a, chu chu an inthlak tihna a ni. Heng mutated genes te hian ‘clotting factors’ dik lo an siam thei a, ‘clotting factors’ tling lo an siam thei bawk. Mahse, hemophilia vei zinga 20% vel chu mahni insiamthatna (spontaneous) an ni a, chu chu chhungkaw zingah tumahin an la vei lo a nih pawhin an vei thei tihna a ni.

Engtin nge hemophilia hi rochun a nih?

Hemophilia A leh B chi hnih hi mipat hmeichhiatna nena inzawm natna an ni. X-linked recessive angin an inherit a ni. Chu chu engtin nge a lo awm tih i lo en ang u:

• Kan nu hnen atangin chromosome set khat kan dawng vek a, kan pa hnen atangin chromosome set khat kan dawng bawk. I nu hnen atanga X chromosome i hmuh a, i pa hnen atanga X chromosome i hmuh chuan hmeichhia i ni tihna a ni. I nu hnen atanga X chromosome i hmuh a, i pa hnen atanga Y chromosome i hmuh chuan mipa naupang i ni tihna a ni. A awlsam zawngin sawi ila, nu hian a fate chu X chromosome a pe fo thin. I sex chu pain X emaw Y chromosome emaw a pek che a\angin a innghat a ni.
• Hmeichhiain a X chromosome pahnih zinga pakhat chauha he ‘clotting factor’ siamtu gene-ah chhiatna a neih chuan hemophilia kengtu a ni thei a, mahse a X chromosome dangah gene hrisel tak a neih avangin symptoms a lantir lo mai thei.
• Hetiang X chromosome chhia nei carrier nu hian fa mipa a neih chuan naupang chuan X chromosome chhia chu a rochun theihna chance 50% a awm a ni. Chutiang a nih chuan naupang mipa chuan hemophilia a nei ang.
• Chu nu chuan fanu a neih chuan naupang pawhin X chromosome chhia chu a rochun theihna chance 50% a awm. Mahse, a pa hnen atanga X chromosome hrisel tak a rochun avangin symptoms a lantir lo mai thei. Tichuan chu fanu chu phurtu a ni bawk ang.

Chu chu hmeichhiain X chromosome chhia a rochun chuan hemophilia kengtu a ni tihna a ni. Symptoms a nei lo mai thei, mahse a fate hnenah natna hi a kai thei a ni. Naupang a neih apiangin hemophilia rochun theihna chance 50% an nei vek a ni.Naupang mipa hian hemophilia an rochun chuan symptoms an lantir tam zawk a, a chhan chu an pa hnen atanga X chromosome hrisel tak an dawn loh vang a ni.

Hmeichhe naupangte pawhin hemophilia symptoms an nei em?

Ni e, a thleng thei. Mahse a symptoms hi a tlangpuiin a na lo hle. Entirnan, hmeichhia hemophilia gene kengtu hian normal clotting factors a nei tlem thei. Chutiang a nih chuan a period laiin thisen a chhuak nasa lutuk a, a hliam awlsam hle a, nau a pai hnuah thisen a chhuak nasa lutuk thei a, a ruh chhungah thisen a chhuak thei a, ruh natna a thlen thei bawk.

Doctor-te hian engtin nge hemophilia hi an hriat theih?

Doctor chuan medical history kimchang a zawt hmasa ang che a, physical exam a ti ang. Hemophilia symptoms i neih chuan i chhungkaw history pawh an zawt ang. Test an ti thei a, chungte chu:

• Complete Blood Count (CBC): Hei hi thisen chhunga cell chi hrang hrang en nana tih a ni.
• Prothrombin Time test (PT): Hei hian i thisen a zang chak zia a enfiah thei a ni.
• Partial Thromboplastin Time test (PTT): Hei hi test dang a ni a, thisen a tlak hun chhung tehna a ni.
• Specific clotting factor test(s): He thisen test hian clotting factor bik, factor VIII leh factor IX te level a teh thin.

Heng clotting factor level te hi engte nge ni?

Clotting factors hi thisen chhuahna control thei thil a ni. Doctor-te chuan hemophilia hi i thisenah heng clotting factors awm zat a zirin a na lo, a na lo, a na lo hle niin an sawi a:

• Clotting factors normal level 5% leh 30% inkar nei te hian mild hemophilia an nei thin.
• Clotting factors normal level 1% leh 5% inkar nei te hian hemophilia moderate an nei thin.
• Clotting factor pangngai 1% aia tlem nei te hian hemophilia na tak an nei thin.

Hemophilia hi eng enkawlna nge ni?

Doctor-te chuan hemophilia hi clotting factors hniam tak tak level tihpun emaw, clotting factors awm lote thlak emaw hmangin an enkawl thin. Hei hi replacement therapy an ti a .

He replacement therapy-ah hian mihring plasma (mihring plasma concentrate) atanga siam clotting factors emaw recombinant ‘clotting factors’ emaw pek i ni. A tlangpuiin hemophilia na tak nei te chauh hian he replacement therapy hi an mamawh fo thin. Mild to moderate hemophilia vei te chu operation an neih dawn chuan he therapy hi pek thin an ni. Antifibrinolytics pek an ni.Thisen zam a hmin lohna tur damdawi pawh pek theih a ni.

Heng blood factor concentrate te hi mihring thisen pek atanga siam a ni. Natna hrik kai theih hepatitis leh HIV kai theihna tur tihtlem nan an thianghlim a , test an ni . Heng replacement factors te hi intravenous infusion (IV) angin pek thin a ni.

Hemophilia na tak i vei a, thisen i chhuah fo chuan i doctor chuan thisen chhuah loh nan ‘prophylactic factor infusions’ tih damdawi a pe thei ang.

Enkawlnaah hian harsatna a awm em?

Mi thenkhat chuan he replacement therapy dawngtu hian antibodies , inhibitors an ti a, chu chuan clotting factors an pekte chu a bei thin. Hemi enkawlna atan hian Doctor-te chuan Immune Tolerance Induction (ITI) an tih hmangin an enkawl a ni. ITI hian nitin clotting factors injection pek a ni a, chu chuan inhibitor level tihhniam a tum a ni. Hei hi hun rei tak chhung enkawlna a ni thei a, mi thenkhat chuan thla tam tak emaw, kum tam tak emaw chhung pawh he enkawlna hi an mamawh thei bawk.

Hemophilia hi ven theih a ni em?

Ni lo, tih theih a ni lo. Hemophilia i vei a, fa i neih chuan i doctor chuan genetic test neih a rawt thei ang . Chutiang chuan nang leh i fate chuan hemophilia hi an thlah kal zelte hnena an kai theih leh theih loh i hre thei ang.

Hemophilia vei hian eng ang beiseina nge a neih ang?

Hemophilia i vei chuan i dam chhung zawng damdawi lam enkawlna i mamawh mai thei. Enkawlna i mamawh zat chu hemophilia i neih chi hrang hrang, a nasat dan leh ‘inhibitors’ i neih leh neih lohah a innghat a ni.

Hemophilia vei dam rei zawng chu engzat nge ni ang?

World Federation of Hemophilia chhinchhiah dan chuan hemophilia vei mipa dam rei zawng hi hemophilia nei lo mipa dam rei zawng aiin kum 10 velin a tawi zawk tih World Federation of Hemophilia 2012 data atanga a lan dan chuan hemophilia vei mipa dam rei zawng hi kum 10 velin a tawi zawk a ni. Mahse, hemophilia vei naupang, an naupan lai aṭanga enkawl leh enkawl an nih chuan an dam rei zawng chu a pangngaiin an awm tih an sawi .

Mahse mi zawng zawng hi kan inang vek lo. Mi pakhat tana dik chu mi dang tan chuan a dik lo thei. Nang emaw i fa emaw hian hemophilia i vei a nih chuan i doctor hnenah eng nge i beisei tih zawt la. I fa dinhmun leh hriselna tlangpui a hre ber a ni.

Engtin nge ka in enkawl ang?

Hemophilia vei te hian thisen chhuah loh nan damdawi lam enkawlna kalpui zel an mamawh mai thei. Thil tih leh damdawi thenkhat an bansan a ngai mai thei bawk. Mahse, hemophilia hian i nunah nghawng a neih dan tur enkawl nan thil tam tak i tih theih a ni.

Thil tih loh tur

Mi dangte tâna thil awlsam, tlu, a nih loh leh tlu awlsam tak takte chuan hemophilia vei tân harsatna lian tak a thlen thei a ni. Tlu emaw, na taka vuak emaw hlauhawmna sang tak siamtu thiltih an pumpelh tur a ni. Entirnan:

• Football, hockey, leh rugby ang chi infiamna khelh.
• Boxing leh wrestling ang chi thila tel.
• Motor khalh lai.
• Skateboarding hmanga khelh a ni.

Infiamna dang, soccer, basketball, leh baseball te pawh hian hliam tuar theihna sang tak a keng tel bawk. Heng infiamnate hi khelh i duh chuan i doctor nen invenna hmanrua hman ang chi thilte sawipui rawh.

Damdawi ei tur tha lo tak tak te

Natna tidamtu aspirin, ibuprofen, leh naproxen te hian thisen zam a veng thei a ni. Tin, heparin emaw warfarin emaw ang chi anticoagulant ei hi a tha lo.

Nun tha zawk nei tura i tih theih thilte

Hemophilia hian i nunah nghawng a neih dan tihtlem nan thil tam tak i tih theih a ni:

• Exercise tih danah lut rawh: Exercise i tih laiin hliam i tuar dawn tih i ngaihtuah mai thei. Mahse, thisen chhuah theihna tur tihtlem rualin active taka awm theih dan tur i doctor nen sawiho rawh.
• Stress enkawl : Hemophilia hi dam chhung zawnga natna a ni a, chhungkaw leh hnathawhna mawhphurhna nena inthlau turin theihtawp chhuah belh a ngai mai thei.
• Ha hriselna tha tak vawng reng rawh: Brush, flossing, leh ha enkawltu hmuh fo hian thisen chhuah theihna tur ha enkawlna mamawhna a ti tlem thei a ni. I dinhmun hi doctor hnenah hrilh theihnghilh suh.
• 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 ang.
• I chhehvela mite hriattir rawh: Hemophilia na tak i neih chuan damdawi i ei pawhin thisen chhuak nghal, thunun theih loh i nei thei a ni. Hetiang thil hi i chunga a lo thlen chuan i chhungte hriattir rawh. I fain hemophilia a vei a nih chuan i fa thisen a chhuah chuan eng nge tih tur tih chu an enkawltu leh sikul thawktute hrilh rawh.

Engtikah nge Doctor ka hmuh ang?

I taksaa inthlak danglamna, thisen chhuah tam lutuk emaw, hliam emaw i hmuh chuan doctor pan rawh.

Emergency Room-a kal hun tur

Heng dinhmunte hi emergency room-ah i kal nghal tur a ni:

• Lu na nasa tak i neih emaw, luak chhuak i neih emaw chuan heng symptoms te hian i thluak chhungah thisen a chhuak tih a tilang thei a ni.
• I ruh a hring leh/ emaw a na a nih chuan, leh factor replacement damdawi i neih loh chuan.

Doctor hnenah eng zawhna nge ka zawh ang?

Nangmah emaw i fa emaw chu hemophilia vei nia hriat i nih chuan i doctor hnenah hetiang zawhna hi i zawt mai thei:

• Kei/ka fa hian eng ang hemophilia nge kan neih?
• Eng enkawlna nge i rawt?
• Enkawlna hian eng side effect nge a neih?
• Kei/ka fa hian nitin enkawl a ngai dawn em ni?
• Eng thiltih nge kei/ka fa tan a tha lo?
• Eng damdawi nge ka/ka fa ei tur tha lo?
• He dinhmun hian ka/ka fa nunah nghawng a neih dan tur enkawl turin eng nge ka tih theih ang?

Take-Home Thuchah a ni

Hemophilia hi thisen lama natna awm lo, rochun atanga lo chhuak a ni. I nunah nghawng lian tak a nei thei a ni. Hemophilia vei te hian dam chhung zawng damdawi lam enkawlna an mamawh mai thei. Hemophilia vei naupang nu leh pate chuan an fate chu harsatna laka humhim an ngaihtuah mai thei a, chubakah hemophilia nena nun dan zirtir an ngaihtuah thei bawk.

Tunah hian doctor-te chuan hemophilia hi an tidam kim thei lo. Mahse, hemophilia natna lan chhuah lohna tur emaw, tihziaawmna tur emaw enkawlna an pe thei a ni. Hemophilia hian nitin nunah nghawng a neih tihziaawmna tura hmalakna tur i rawt thei bawk. A pawimawh ber chu thlamuang taka awm a, doctor thupek uluk taka zawm hi a ni.