Thisen chhuah tawh lohna tur cut te tak te pawh i nei lo em ni? Hemophilia chungchang hi han sawi ila!

Hliam te tak te i neih emaw, khawi emaw laiah i vuak chuan rei vak lo hnuah thisen chhuah chu a reh ta a ni lawm ni? Chu chu kan taksaa thisen zam (natural process) vang a ni. Mahse han ngaihtuah teh, mi thenkhat chuan hetiang hian awlsam taka thisen chhuah hi an bansan lo. Hliam te tak te nei pawhin an thisen a chhuak rei hle. Taksa chu a sen a, a hliam bawk. Hetiang dinhmun thlentu hi natna hrik (hemophilia) an tih a ni thei. Hemi chungchang hi mi tam takin hre chiang lo mah se, hei hi kan hriat tur pawimawh tak a ni. Hemi chungchang hi awlsam takin, i hriatthiam theih dan tur angin sawi ila.

Hemophilia hi eng nge ni tak tak?

A awlsam zawngin sawi ila, hemophilia hi kan thisen a inzawm tha lo tihna a ni. Kan thisenah hian protein bik a awm a, chu chuan thisen a chhuah tan tirh atangin a titawp thei a ni. 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 factor pakhat emaw a aia tam emaw hi a siam chhuak tawk lo. Chumi awmzia chu cut tlemte pawh hian hun rei tak thisen a chhuah thei tihna a ni. A chang chuan taksa chhungah thisen a chhuak thei a, a bik takin ruh leh ruhkawr ah thisen a chhuak thei bawk. Hei hian nunna a tichhe thei a ni.

Doctor-te chuan he natna nasat dan hi chi thum-ah an ṭhen a, thisenah clotting factors awm zat a zirin.

• Mild hemophilia: Thisen chhuah hi hliam lian tak emaw, operation emaw hnuah a awm tlangpui.
• Moderate hemophilia: Hliam tenau te pawh hian thisen chhuah tam lutuk a thlen thei. Taksa pawh a rawn sen mai thei.
• Hemophilia na tak: A chhan hriat loh pawhin chhungril leh pawnah thisen a chhuak thei.

Hei hi tihdamna famkim awm lo mah se, enkawlna thar awm tawhte nen chuan thisen chhuahna control theih a ni a, a tam zawk chu nun pangngaia hman theih a ni.

Hemophilia chi hrang hrang lian ber ber a awm em?

Ni e, he natna hi chi thum a awm a. Taksa chhunga clotting factor tlakchham chi hrang hrang atanga siam an ni. Hei hi chiang taka hrethiam tur chuan a hnuaia table hi en la.

He natna lan chhuah dan engte nge ni?

Hemophilia chhinchhiahna ber chu thil danglam emaw, thisen chhuah tam lutuk leh hliam na lutuk emaw a ni. Heng symptoms nasat dan hi hemophilia na lo, na lo, na tak i neih leh neih lohah a innghat a ni.

Thisen chhuah tam lutuk

Ngaihtuah teh, chhan awm lovin hnar thisen a chhuak nghal a. A nih loh leh i kut zungtang tlemte emaw, operation hnuah emaw pawh nise, thisen chhuahna chu rei tak a reh lo. Nausen te tak te hian he natna hi an neih chuan an hmuiah pawh infiamna an dah a, thisen an chhuak thei bawk.

Bruising a awm

Kan vun hi a hnuaia thisen a luang chuan a sen a ni. Hemophilia vei te hi thil tenau takin a rawn nuai pawhin awlsam takin an blue thei a, an hliam thei bawk. Naute te tak te hian khawi emaw laiah an lu a rawn deng a nih chuan "goose eggs" an tih chu lump lian tak tak an nei thei a ni.

Joint Pain leh Swelling a awm

Hei hi hemophilia natna khirh ber a ni. Taksa chhungah thisen a chhuak thei a, a bik takin ruhkawr, khup, kekawrte, leh ke ruhteah te a chhuak thei a ni. Chumi hnuah chuan ruhte chu a hring a, a na a, khawih pawhin a lum bawk. Nausen naupangte chunga hetiang thil a thlen hian an tap chhunzawm zel thei a, khup an duh lo thei a, an kal duh lo thei bawk.

Eng thilin nge hemophilia hi a thlen?

Hemophilia hi genetic disease a ni fo thin a, thlah khat atanga thlah dangah a kai darh thin.Chu chu kan nu emaw, kan pa emaw atanga kan rochun genes inthlak danglam vang a ni tihna a ni. Hei hi tlem tal i hrethiam zawk ang u.

Kan taksa hi genes hmangin clotting factors siam turin hriattir a ni. Heng genes-a inthlak danglamna emaw, chhiatna emaw a awm chuan chu thupekte chuan hna a thawk tha lo. Chumi rah chhuah chu taksain a mamawh zat clotting factors a siam chhuak lo a ni.

He genetic defect hi X chromosome-ah a awm a ni.

• Hmeichhe naupang pakhat hian X chromosome pahnih (XX) a nei a, pakhat chu a nu atanga lak niin pakhat chu a pa atanga lak a ni.
• Mipa naupang pakhat hian X chromosome pakhat leh Y chromosome pakhat (XY) a nei a. X chu nu atanga lo chhuak a ni a, Y chu pa atanga lo chhuak a ni.

Tunah chuan hei hi thlah kal zelah engtin nge a kal zel tih hi han en teh:

• Nu pakhatin he gene chhia (‘carrier’ kan ti) nei X chromosome a keng a nih chuan X chromosome hrisel dang a neih avangin symptom a lantir lo tlangpui.
• He nu fapa hian X chromosome chhia hi a rochun chuan hemophilia a nei ang a, a chhan chu a control thei tur X chromosome hrisel dang a neih loh vang a ni.
• He nu fanu hian X chromosome chhia a rochun chuan carrier a ni bawk ang. A natna lan chhuahna na lo tak tak, thlasik laiin thisen chhuak nasa tak a lantir thei bawk.

A châng chuan, hemophilia hi nausen dam chhunga a takin gene inthlak danglam vang a ni a, rochun lohvin a lo awm thei bawk. Tin, a tlem hle bawk, ​​mi pakhat immune system-in anmahni clotting factors a beih hian a thleng thei bawk. Hei hi ‘acquired hemophilia’ kan ti a.

Engtin nge doctor hian hei hi a hriat chian?

Nangmah emaw i fa emaw hian heng symptoms hi i neih chuan i doctor chuan a enfiah hmasa ang che a, i chhungte zingah hian hetiang natna hi an nei tawh em tih a zawt ang che. Tichuan, hei hi finfiah turin thisen test thenkhat an order ang.

• Complete Blood Count (CBC): I thisena cell chi hrang hrang leh a zat tehna a ni.
• Prothrombin Time test (PT): I thisen a inzawm hun chhung a enfiah thin.
• Partial Thromboplastin Time test (PTT): Hei hi thisen zam hun chhung tehna test dang a ni.
• Specific Clotting Factor Tests: Hei hian eng clotting factor nge i tlakchham tih leh eng chen nge a hniam tih chiang takin a hril a ni.

Hei hi eng enkawlna nge ni?

I enkawlna tur chu hemophilia i neih chi leh i natna nasat danah a innghat ang. Enkawlna ber chu replacement therapy a ni.

A awlsam zawngin, hei hian i taksaah i thisena hniam tak, clotting factor shot pek a huam a ni. Heng clotting factors te hi mihring thisen plasma atanga siam emaw, laboratory-a siam emaw theih a ni. He enkawlna hian thisen chhuah a veng thei a, a lo awm a nih chuan a titawp nghal vat thei bawk.

Hei bakah hian tunah hian enkawlna thar engemaw zat a awm tawh bawk:

• Marstacimab-hncq (Hympavzi®) leh Concizumab (Alhemo®) ang damdawi te : Hengte hian thisen zam tur venna protein pakhat a block a, thisen zam tura puitu enzyme a tichak bawk.
• Fitusiran (Qfitlia®): He damdawi hian kan thin (liver) hian thisen a tikhawlo thei lo (antithrombin) a siam thei lo.
• Gene Therapy: Hei hi enkawl dan hmasawn ber a ni. Hetah hian gene chhia chu thlak turin taksaah gene hrisel tak dah a ni a, chu chuan taksain a mamawh clotting factors a siam chhuak thei a ni.

Engtikah nge damdawi lam thurawn i lak nghal ang?

I taksaah danglamna eng emaw, thisen chhuah tam emaw, hliam emaw i hmuh chuan doctor hnenah hrilh nghal rawh. Tin, a hnuaia symptom na tak tak te hi i tawn chuan damdawi in pakhat Emergency Department (ETU) ah tihkhawtlai lovin kal la.

Hemophilia natna vei huna hriat tur thil

Hemophilia hi dam chhung zawng, a nih loh leh natna rei tak awm thei a ni. Chuvangin i dam chhung zawngin damdawi lam enkawlna leh enkawl i mamawh dawn a ni. Mahse, lungngai suh. Enkawlna thar a awm chuan hemophilia vei chuan natna nei lo nun nena inhnaih tak nun a hmang thei a ni.

He natna vei lai hian i rilru hahdamna tur thil thenkhat chu hetiang hi a ni:

• I taksa rihna control: I thau chhoh zel chuan i ruh (joint)-a pressure a sang chho zel a. Ruh chhunga thisen chhuak avanga ruh chhia tawhte venhimna atan taksa rihna hrisel neih hi a pawimawh hle.
• I ha enkawl \ha rawh: Ha sil \ha a pawimawh a, i ha hriselna pawh uluk taka enkawl a pawimawh. Ha lakchhuah leh root canals ang chi procedure te hian thisen a chhuah theih avangin, chutiang chu pumpelh a tha ber.
• Rilru hriselna ngaihtuah rawh: Natna khirh tak nena nun hian lungkhamna leh lungngaihna a thlen thei a. Hemi chungchang hi i doctor nen sawiho la, a tul chuan rilru lam hriselna lama counseling la rawh.
• Hrisel takin active rawh: Hliam hlauhawmna tihziaawmna tur thil tih hi i taksa leh rilru tan a tha hle. I doctor hnenah eng exercise nge i tan a tha tih zawt rawh.

Hemophilia i neih hian midangte hriat loh hliam tenau tak pawh i tan harsatna lian tak a ni thei. Table kea khup a tlak chuan ruh pakhatah thisen a chhuak thei a ni. Hnar thisen chhuak chu thawk hah nasat hnuah pawh tihtawp theih loh a ni thei. Hetiang dinhmun nena nun hi a harsa thei hle. Mahse, nangmah chauh i ni lo tih hre reng ang che. I medical team te chu i tanpui turin an awm reng a ni.