I cut te tak te pawh hian thisen a chhuah a titawp lo em ni? Hemophilia chungchang hi kan sawi dawn em ni?

I naupan lai khan, i tlu a, i khup emaw, i elbow emaw a hliam a, thisen chhuahna chu rei vak lo hnuah a tawp mai thin tih i hre reng em? Chu chu thil mak a ni lo maw? Thisen kan chhuah hian kan taksa hian a titawp thei tur system a nei a, chu chu thisen zam tirtu system a ni. Mahse mi thenkhat an awm a, he thisen zawrh dan hian an taksaah hna a thawk tha lo. Chu chu hemophilia tih hming pu dinhmun kan sawi lai a ni. Hei hi a pawi deuh thei a, mahse enkawl dan dik a nih chuan enkawl that theih tur condition a ni.

Hemophilia hi eng nge ni tak tak?

A awlsam zawngin, hemophilia hi kan thisen a inzawm tha lo tihna a ni a, chu chu kan thisen chhuah hian awlsam taka a tawp lo tihna a ni . It is mainly a genetic disease , chu chu gene kaltlangin thlah khat atanga thlah dangah a kai darh thei tihna a ni.

I kut ah hian hliam te tak te i nei ang tih han ngaihtuah teh. A tlangpuiin kan thisena protein ṭhenkhat, clotting factors , an tihte chuan hliam chu an khar a, thisen chhuah chu an titawp a ni. Wall chhunga khur patching ang mai a ni.

Mahse hemophilia vei tan chuan heng clotting factors zinga pakhat hi a tam tawk lo a, a nih loh leh chu factor chuan hna a thawk tha lo. Chu chu buaina chu a ni. Hei vang hian hliam tenau tak atang pawhin rei tak thisen an chhuak thei a, a nih loh leh awlsam takin an hliam thei bawk.

Thisen a clot dan mai mai

Thisen kan chhuah tan hian a tihtawp dan tur kawng engemaw zat a awm.

1. Thil thleng hmasa ber chu thisen kalna kawng a inkhai (constrict) thin. Tichuan thisen chhuak zat chu a tlahniam deuh a.

2. Tichuan, kan thisena cell te te, platelet an tih te chu hliamna hmunah an lo kal a, an inzawm khawm a, dam te tak te chi khat an siam ta a ni.

3. A dawtah chuan a hmaa ka sawi tawh clotting factors te chu activate an ni a, pakhat hnu pakhatin hna an thawk a, fibrin tih hming pu mesh chak tak an siam a, platelet barrier chu a tichak zual a, thisen chhuah chu a titawp vek a ni.

Hemophilia-a thil thleng chu he step pathumnaah hian clotting factor pakhat a awm lo a, chuvang chuan clot chak tak chu a lo awm lo.

Hemophilia chi hrang hrang a awm em?

Ni e, hemophilia hi chi hnih a awm a, chu chu eng clotting factor nge tlakchham tih a zirin a ni.

Hemophilia natna hi a ni

Hei hi hemophilia chi khat a ni ber. Clotting factor VIII a neih tlem vang emaw, a hnathawh that loh vang emaw a ni.

Hemophilia natna hi B

Hetiang chi hi Krismas Natna an ti bawk . Thisen zamna factor 9-na (Factor IX) tlakchham vang a ni.A tawk lo tih chu a pawi lo, a thawk tha lo a nih pawhin a pawi lo.

A chi hnih symptoms hi a inang hle. Amaherawhchu heng chi hnih hi enkawl dawnin dik taka hriat chian a pawimawh hle. A chhan chu factor tlahniam tawh angin enkawlna pek tur a ni.

He dinhmun pawizia hi

Hemophilia nasat dan hi mi hrang hrangah a inang lo thei a, chu chu taksaa deficient clotting factor awm zat a zirin a ni.

• Mild hemophilia: Mild hemophilia vei te hian thisen zam (blood clotting factors) pangngai aia tlem an nei tlem zawk. Hliam lian tak an neih emaw, operation an neih emaw, ha lakchhuah emaw an neih chuan rei lo te chhung chauh thisen an chhuak thei. Thil pawi tak a thlen hma chuan hemophilia an vei tih pawh an hre lo mai thei.
• Moderate hemophilia: Heng mite hian clotting factors an nei tlem zawk. Hliam tenau tak tak atang pawhin thisen an chhuak nasa lutuk thei. A châng chuan, chhan awm lovin thisen an chhuak thei (spontaneous bleeding).
• Hemophilia na tak: Heng mite hian thisen zam (blood clotting factors) an nei tlem hle. A chhan hriat lohvin ruh leh ruh (muscle)-ah thisen an chhuak thei a ni. A hliam tenau ber pawhin harsatna lian tak a thlen thei.

Engvangin nge hetiang hi a lo awm? A chhan engte nge ni?

Hemophilia hi genetic condition a ni fo thin , chu chu nu leh pa atanga fate hnenah genes hmanga kai a ni tihna a ni.

Inheritance atanga a lo chhuah dan (X-linked inheritance) .

Hemophilia gene hi X chromosome-ah a awm a. I hria em, hmeichhia hian X chromosome pahnih (XX) an nei a, mipa erawh chuan X chromosome pakhat leh Y chromosome pakhat (XY) an nei thung.

• Naupang hmeichhia: A nu hnen atangin X pakhat a hmu a, a pa hnen atangin X pakhat a hmu bawk.
• Naupang mipa: Nu hnen atangin X pakhat leh pa hnen atangin Y pakhat a hmu.

Tunah chuan hemophilia thlentu gene chhia chu X chromosome-ah a awm a nih chuan,

• Mipa naupangin chu X chromosome chhia chu a nu hnen atangin a rochun chuan hemophilia a vei ang. A chhan chu X chromosome pakhat chauh a neih vang a ni. A chungah chhiatna a awm chuan a rulhna tur X dang a awm lo.
• Hmeichhe naupangin a nu emaw a pa emaw hnen atangin X chromosome chhia a rochun a, mahse X chromosome hrisel tak a neih chuan symptoms a lantir lovang. Mahse carrier a ni dawn a ni . Chumi awmzia chu hemophilia nei lo mah se, a gene chhia chu a fate hnenah a pe chhuak thei tihna a ni. A tlem hle a, hmeichhe carrier te pawhin symptoms na lo tak tak an nei thei bawk.

Chuvangin hemophilia hi mipaah ​​a awm tam ber a ni .

Ngaihtuah teh, nu pakhat chu hemophilia kengtu a nih chuan a fa mipa piang apiangin hemophilia vei theihna chance 50% an nei vek a ni. Chutiang bawkin naupang hmeichhia zawng zawng hian carrier nih theihna chance 50% an nei vek a ni.

Chhungkaw hrang hrangah a kal em? (Spontaneous mutations a awm thin) 1.1.

Chhungkaw zinga mi tu emaw chuan hemophilia vei a ngai reng reng lo. A châng chuan 30% vêlah chuan hemophilia hi gene pakhata spontaneous mutation angin a lo awm thei a ni. Chutiang a nih chuan, chhûngkaw zînga mi dangin he natna hi an lo nei tawh lo mai thei.

Hemophilia natna lan chhuah dan chu engte nge ni? Engtin nge a hriat chhuah?

Hemophilia natna lan chhuah dan hi a natna nasat dan azirin a inang lo. A lan chhuah dan tlangpui chu:

• Hripui lian leh thuk tak awm fo: Bump te tak te pawh hian hliam lian tak a thlen thei.
• Hliam avanga thisen chhuak chhunzawm zel, ha lakchhuah emaw, operation hnua thisen chhuak chhunzawm zel.
• Chhan awm lovin hnar thisen a chhuak.
• Gums thisen chhuak.
• Ruh chhunga thisen chhuak (hemarthrosis): Hei hi a na hle. A ruh chu a hring a, a lum a, a che tha thei lo. Khup, khup, leh ke ruh te ang chi ruh (joint) te hi a natna a tam ber. Hun rei tak chhung chu hei hian ruh a tichhia thei bawk.
• Muscle chhunga thisen chhuak: Hei hian natna leh hliam a thlen bawk.
• Zun emaw, bawlhhlawh emaw hmanga thisen chhuak.
• Nausen tan: A chang chuan a chhinchhiahna hmasa ber chu serh tan hnua thisen chhuak reng a ni. A nih loh leh injection an pek hnuah a hmun chu a hring nasa hle a, thisen a chhuak thei bawk.
• Thil pawi tak, mahse hmuh tur awm lo tak chu thluaka thisen chhuak hi a ni. Hei hian symptoms, lu na na tak, luak chhuak, mutthilh, leh seizures te a thlen thei a ni. Hei hi emergency a ni.

Engtin nge doctor chuan hei hi hemophilia a ni tih a hriat? (Diagnosis) tih a ni.

Nangmah emaw, i fa emaw hian heng symptoms te hi i nei a nih chuan a tha ber chu doctor pan hi a ni. Doctor chuan a hnuaia mi ang hian diagnosis a siam tlangpui ang:

1. Chhungkaw chanchin zawh: Chhungkaw zingah hemophilia emaw thisen chhuah harsatna dang emaw nei an awm leh awm loh enfiah.

2. Taksa endikna neih a ni: A hliam, ruh hring, etc. enfiah.

3. Thisen test tih a ni: 1.1.

• Screening test: Hengte hian thisen a tlakbuak theih hun chhung a teh thin. Entirnan, PTT (Partial Thromboplastin Time) leh PT (Prothrombin Time) ang chi test te hi a ni. Hemophilia-ah hian PTT value hi a rei thei hle.
• Clotting factor assays: Hengte hi Factor VIII leh Factor IX te hi a tlakchham em tih hriat nan hman a ni a, eng chen nge a tlakchham tih hriat nan hman a ni. Hei hi hemophilia chi leh nasat dan hriat nan hman a ni.

Naupai lai pawhin chhungkaw history-ah hemophilia vei an awm a nih chuan naute hian hemophilia a vei em tih enfiah theih a ni.

Hei hi eng enkawlna nge ni? (Tihdamna)

Hemophilia hi tihdam theih loh natna ni mahse, enkawlna tha tak tak a awm a.Heng enkawlnate hian thisen chhuahna control leh venna kawngah a pui thei a, nun tha zawk i neih theih nan a pui thei bawk.

Replacement Therapy hmanga tihdam theih a ni

Hei hi a enkawlna ber a ni. Chu chu taksaah thisen zam (Factor VIII emaw Factor IX) a tlakchham chu vein (intravenous emaw IV infusion) hmanga pek a ni. Heng thilte hi mihring thisen plasma atanga siam emaw, genetic technology (recombinant products) hmanga siam a ni.

He enkawlna hi kawng hnih a tih a ni a:

1. Prophylactic therapy: Hetah hian factor hi kar khatah vawi eng emaw zat taksaah pek thin a ni a, thisen a chhuah hmain . Hei hian thisen chhuak thut a veng thei a, a bik takin ruhkawr chhungah a veng thei a ni. Hei hi hemophilia na tak nei tan tih a ni tlangpui.

2. On-demand therapy: Hemi ah hian thisen chhuah tan hnuah chauh factor hi pek thin a ni. He enkawlna hi mild hemophilia vei leh preventive therapy hmang lo tan hman a ni.

Damdawi dang

• Desmopressin (DDAVP): Hei hi hemophilia A natna vei tan hman theih a ni a, taksaa Factor VIII dahkhawm (stored Factor VIII) a chhuah chhuah avangin hna a thawk a ni. Hnar spray angin emaw, injection angin emaw pek theih a ni.
• Antifibrinolytic damdawi: Entirnan , tranexamic acid . Heng damdawi te hian thisen zam (blood clot) a inthiarna (dissolution) a tikhawlo a, hna an thawk a ni. Ha lakchhuah leh hnar thisen chhuahna ang chiah hian an tangkai hle.
• Painkillers: Paracetamol ang chi thil i ei thei a, chu chuan ruh chhunga thisen chhuah avanga natna lo awm thin chu a tiziaawm thei a ni. Mahse, aspirin leh NSAID (e.g., ibuprofen, diclofenac) ang chi damdawi hi hemophilia vei tan chuan a tha lo a, thisen chhuah theihna a tipung thei a ni.

Thisen chhuah lai hian eng nge i tih thin?

Thisen chhuah huna tih tur tlemte a awm. RICE method kha hre reng ang che.

• R (Rest): Hliam ruh emaw, ruh ruh emaw chu chawlh tir rawh. A che lo turin veng rawh.
• I (Ice): Ice pack chu hliam na hmunah minute 15-20 vel hnawih la, ni khatah vawi eng emaw zat hnawih thin ang che. Hei hian a hring leh natna a tiziaawm ang.
• C (Compression): Hliam na hmun chu elastic bandage hmangin khuh la, a tight deuh theih nan.
• E (Elevation): Hliam kut emaw ke emaw chu thinlung level aia sangah dah tur a ni.

Heng thil i tih lai hian i doctor nen inbia la, a tul chuan Factor treatment la rawh.

Hemophilia natna vei dan tur

Hemophilia natna vei hi thil harsa tak a ni thei a, mahse uluk taka enkawl, hriatthiamna leh puihna chuan nun pangngai, active takin i nung thei a ni.

• Specialist doctor team atanga tanpuina: Hemophilia enkawl lai hian, .Hematologist, nurse, physiotherapist, leh social worker te awmna team atanga tanpuina lak a pawimawh hle. Enkawl dan tur te, exercise lak dan tur te, him taka awm dan tur te an hrilh ang che.
• Thil him tak ti rawh: Hemophilia vei te chuan hliam tam tak thlen thei infiamna (e.g., rugby, boxing) hi an pumpelh tur a ni. Chu ai chuan tui chawi, kea kal, leh cycle khalh (helmet vuah) ang chi exercise him tak takah tel ve rawh. I tana tha tur exercise thlang turin physical therapist nen inbia ang che.
• Ha hriselna tha tak vawn reng: Ha hring leh ha natna laka inven a pawimawh hle a, ha lakchhuah ang chi tih hian thisen chhuah a hlauhawm hle. Ha enkawltu pan chhunzawm zel ang che.
• Medical alert identification: Hemophilia i vei tih ziakna bracelet emaw card emaw keng fo ang che. Emergency-ah pawh a tangkai thei hle.
• Social support: Hemophilia vei midang leh an chhungte nena inbiakna leh thil tawn sawiho hi chakna ropui tak a ni. Chutiang pawl an awm chuan zawm ve rawh.
• Nakin hun atana beiseina: Hemophilia enkawlna thar hmuhchhuah a ni reng a. Gene therapy ang chi thil chungchangah pawh zirchianna neih mek a ni . Chuvangin nakin hun atan beiseina kan nei thei a ni.

Take-Home Thuchah a ni

Hemophilia hi thisen zam (blood clotting problem) a ni a, mahse hlauh tur a ni lo va, uluk taka enkawl theih a ni .

A pawimawh ber chu natna lan chhuahna i neih chuan doctor thurawn la vat a, dik taka hriatchhuah a, enkawlna pek dan zawm hi a ni.

Nangmah emaw, i hriatte emaw hian hemophilia an vei a nih chuan, nangmah chauh i ni lo tih hre reng ang che. I mamawh hriatna, ṭanpuina, leh enkawlna i neih chuan nang pawhin nun hrisel leh thawkrim takin i nung thei a ni. Beiseina bansan suh!

` Hemophilia, thisen chhuak, thisen zam, genetic natna, Factor VIII, Factor IX