Nang emaw i naute emaw hian hliam tenau tak tak atang pawhin thisen chhuah harsatna i nei em? Hemophilia A chungchang hi han sawi ila

I taksaa hliam te tak te i neih lai emaw, ha i hrual lai emaw chuan thisen chhuah chu a tawp lova a chhunzawm zel em? A nih loh leh khawiah mah hip lovin i taksaah blue bruises i nei fo thin em? I taksa pum puiah hliam i hmu em, a bik takin i fate chu a rawn tlan chhuak emaw, a kal tan emaw chuan? Hetiang thil hi a thleng a nih chuan a chhan chu hemophilia an tih natna a ni thei. He hming i hriat hian hlau suh. With the right treatment , nun pangngai, famkim takin i nung thei ang. Hemi chungchang hi vawiin hian chiang tak leh awlsam takin sawi ila.

A awlsam zawngin, Hemophilia A hi eng nge ni?

A awlsam zawngin sawi ila, Hemophilia A hi kan thisen a inzawm tha lo tihna a ni. A tlangpuiin hliam kan neih hian kan thisen chu a titawp a, thisen chhuah chu a titawp thin. Hei hi kan thisena protein bikte hian a pui a ni. Hengte hi "clotting factors" kan ti a ni.

Hemophilia A vei chuan clotting factor Factor VIII a siam tling lo. Natna nasat dan hi he Factor VIII protein hi a tlahniam dan a\angin a innghat a ni. Chumi ang chuan hmun thumah then a ni a:

• Mild hemophilia: Factor VIII level hi eng emaw chen chu a awm a.
• Hemophilia natna (moderate hemophilia): Factor VIII level hi a hniam hle.
• Hemophilia na tak: Factor VIII level hi a hniam hle emaw, a awm lo emaw a ni.

A tam zawkah chuan hei hi chhungkaw hrang hranga kal thin, genetic disease a ni. Mahse, hmun thuma ṭhena hmun khat vêlah chuan mi thar chuan chhûngkaw zînga mi tumahin an vei lohvin a vei thei a ni.

Eng thilin nge hemophilia hi a thlen?

Hei hi genes hmanga kan rochun thil a ni. Ngaihtuah teh, kan taksa mizia tinreng hi genes-in a ruat vek a ni. Hmeichhia chuan X chromosome pahnih (XX) a nei a, mipa chuan X chromosome pakhat leh Y chromosome pakhat (XY) a nei thung.

Hemophilia nena inzawm gene defect hi X chromosome ah a awm a .

Chuvangin, nu chu he gene chhia hi "carrier" a nih chuan a tlangpuiin symptoms a lantir lo. A chhan chu a X chromosome hrisel dang hian factor VIII protein mamawh tak a siam avangin. Mahse a fapain he X chromosome chhia hi a rochun chuan chu fapa chuan hemophilia a vei ang. A chhan chu tlangval hian X chromosome pakhat chauh a nei a ni. Hemi avang hian hemophilia hi hmeichhia aiin mipaah ​​a tam zawk.

Hemophilia natna lan chhuah dan chu engte nge ni?

I natna lan chhuah dan chu i dinhmun a na lo emaw, a hniam emaw, a na emaw a innghat a ni.

Thluak thisen chhuak a awm tih hriattirna

Hemophilia na tak vei chuan a lu-ah vuakna tenau tak pawh a tawk a nih chuan thluaka thisen a chhuak thei a ni. Hei hi emergency pawi tak a ni. Lu hliam leh a hnuaia symptoms te hi i neih chuan doctor ko nghal la, emergency department (ETU) hnai ber pan rawh.

• Lu na nasa tak awm reng
• Luak chhuak
• Mutthilh fo emaw, hah lutuk emaw
• Chak lo thut emaw, kal harsa emaw
• Double vision hmuh theihna
• Seizures a awm thin

Engtin nge natna hi kan hriat theih ang?

I chhungkuaa mi pakhatin hemophilia a vei a nih chuan i naupai laiin i naute chu he natna hi i test tir thei ang. Mahse, heng test-te hian hlauhawmna engemaw zat a nei a, chuvangin i doctor nen in sawiho tur a ni.

Naupang tê tête zingah hemophilia natna khirh tak chu an dam kum khatnaah hmuhchhuah a ni tlangpui. I fa chu a inher tan a, a rawn tlan tan a, an taksaah hliam sang tak a hmu tan a nih chuan i doctor nen sawiho rawh.

Doctor chuan hetiang zawhna hi a zawt thei che a ni:

• Engtin nge heng hliam leh thisen chhuahna te hi a lo awm?
• Eng chen nge thisen chhuah hi a awm tawh?
• Naupangte ei tur damdawi a awm em?
• I chhungte zingah thisen zam harsatna nei an awm em?

Tichuan, thisen test eng emaw zat neih a ni ang a, a natna hmuhchhuah a nih leh nih loh finfiah a ni ang. Hematologist hnenah pawh refer i ni thei bawk.

Engtin nge enkawl a nih?

Enkawl dan chu natna nasat dan te, i kum leh i mimal mamawh danah a innghat a ni. Enkawlna tum ber chu taksaa factor VIII protein bo chu thlak a ni. Hei hi factor replacement therapy an ti a . Hei hian natna hi tidam kim lo mah se, thisen chhuahna control-na atan a pui thei a, nun pangngaia i nun theihna tur a siamsak thei bawk.

Enkawlna chi hnih a awm a, chungte chu:

• Prophylactic Therapy: Thisen chhuahna tur nghah ai chuan, thisen chhuah loh nan factor VIII hi schedule ruat sain injection neih fo thin. Hei hi hemophilia na tak nei tan chuan a pawimawh hle.
• On-demand Therapy: Thisen chhuah a awm chauhvin enkawlna dawng .

Factor VIII vaccine hi chi hnih a awm a, chungte chu:

1. Recombinant Factor VIII: Genetic engineering hmanga laboratory-a thil siam chhuah. Hei hi tunlaia hman tlanglawn ber a ni.

2. Plasma atanga chhuak Factor VIII: Mihring thisen plasma atanga factor lak chhuah.

Hemophilia A na lo leh na lo tak nei te pawhin Desmopressin (DDAVP) tih damdawi an hmang thei bawk. Hei hian factor VIII dahkhawm chu thisenah a chhuah tir a, hei hian hna a thawk a ni.

Hei bakah hian thisen zam a chhiat lohna tur Tranexamic Acid ang chi damdawi ei te pawh ha lakchhuahna ang chiah hian hman a ni bawk.

Engtin nge nitin nun i enkawl?

Hemophilia natna vei lai hian himna chungchang tlem tal i ngaihtuah a ngai a ni.

• Active takin awm rawh: Exercise hian taksa ruh a tichak a. Muscle chak tak hian ruh (joint) tan venhimna tha tak a pe a ni. Mahse, rugby leh boxing ang chi contact sports hi pumpelh rawh. Swimming, kea kal, leh cycle khalh (helmet vuah) te hi duhthlan tur tha tak a ni. I doctor nen eng thiltih nge i tana tha tur tih sawipui rawh.
• Naupang venhim: Naupang tê tê i neih chuan infiam laiin khup khuhna, elbow pad leh helmet vuah tur a ni. I in chhunga furniture hrual hrual (sharp-edged furniture) lakah inveng rawh.
• Ha hriselna: 1.1.Ha thianghlimna tha tak vawng reng rawh. Nitin ha silfai thin ang che. Hei hian ha lakchhuah leh ha enkawlna lian tham tak tak mamawhna a tihziaawm thei a ni. Ha enkawltu i pan hmain hemophilia i vei tih hrilh hmasa phawt ang che.
• Damdawi ei loh tur: Natna tidamtu aspirin leh NSAID (e.g., ibuprofen, diclofenac) te hian thisen zam a titawp zual thei a ni. Doctor rawn hmasa lovin damdawi engmah ei loh tur a ni.