Pum natna nasa tak leh chhan awm lova hriatna hloh... He natna hmuh tur awm lo tak hi i zir dawn teh ang? (Hepatic Porphyria natna hlauhawm tak) .

Chhan hriat lohvin, tuar theih loh khawpin pum na i nei nghal mai ang tih han ngaihtuah teh. Chumi rual chuan i ke ruhte chu a hring vek a, i muhil dawn ang maiin i inhria a, i rilru a buai bawk. Doctor engemawzat kan hmuh hnu leh test tam tak kan tih hnu pawhin a natna chhan dik tak hi hriat theih a ni lo. Nangmah emaw, i hriat emaw hian hetiang thil hi i tawng tawh em? A chhan chu kan rama mi tam takin an hriat ngai loh genetic condition hmuh tur awm lo tak a ni mai thei. Vawiin hian chutiang dinhmun pakhat, Acute Hepatic Porphyria , emaw AHP chungchang kan sawi dawn a ni.

A awlsam zawngin Acute Hepatic Porphyria (AHP) hi eng nge ni?

AHP hi genetic disorder awm lo tak a ni a, kan nervous system leh a chang chuan kan vun a tichhe bawk. A tlem em avangin mi 100,000 zinga 5 velin a nghawng a ni. Symptoms rang tak, na tak, hrehawm tak tak a thlen thei. A châng chuan, hengte hi nunna atana hlauhawm tak a ni thei bawk.

Hei hi hrethiam tur chuan kan taksa chhunga thisen awm chungchang tlem han sawi ila. Kan thisen sen ah hian protein pakhat `` Hemoglobin '' tih a awm a. A hna ber chu kan thawk chhuah huna lung chhunga oxygen lo lut chu la chhuakin taksa peng leh tissue dang zawng zawngah a thlen vek a ni. Oxygen kengtu ‘delivery service’ ang mai a ni.

He protein siamna atana pawimawh component dang pakhat ``Hemoglobin'' tih a awm a, chu chu ``Heme'' kan ti a ni.

AHP natna vei taksaa thil thleng chu he ``Heme'' an tih component siam chhuahna kawngah hian engtik lai pawha mamawh enzyme pakhat tlahniam emaw, hloh emaw hi a ni. Dish siam lai ang mai a ni a, thil pakhat a bo chuan dish chu a siam tha thei lo.

He enzyme tlakchhamna hi kan liver ah a thleng ber a ni . Liver hi `(Hepatic)` kan ti bawk. Chuvangin he natna hi `Acute Hepatic Porphyria` tiin an vuah a ni.

Tunah chuan `(Heme)` kan siam theih loh hunah eng nge thleng? A atana hmanraw raw materials, `(Porphobilinogen – PBG)` leh `(Aminolevulinic acid – ALA)` te chu liver-ah a pung khawm tan a. Heng toxins te hi thisenah an pungkhawm a, taksa pum puiah an fang kual a, a bik takin kan nervous system a tichhe nasa hle. Chu nerve chhiatna avang chuan natna nasa tak , rilru hahna , leh rilru hahna ang chi symptoms a lo chhuak thin.

AHP natna chi hrang hrangte chu engte nge ni?

AHP hi chi li a awm a. Type tin hi ``(Heme)`` siamna kawngah enzyme hrang hrang tlakchham vang a ni. Mahse pali hian liver leh nervous system a nghawng ber a ni.

Porphyria natna hrik (AIP) .

Hei hi AHP vei 80% , a tam zawk tihna chi a ni. Hetah hian enzyme `(Hydroxymethylbilane synthase – HMBS)` tlakchhamna a awm a. A chhan chu `(HMBS)` gene inthlak danglam vang a ni. Damdawi lamah chuan hei hi `(mutation)` gene kan ti a. Hei hi a chang chuan nu leh pa atanga fate rochun theih a ni. A nih loh leh a chang chuan chhungkaw history nei lo mi pakhatah random takin a thleng thei bawk.

AHP symptoms hi engtin nge a awm?

AHP vei zawng zawng hian symptom inang an nei vek lo. Mi thenkhat chuan an dam chhungin symptom engmah an nei lo mai thei. Mahse mi thenkhat chuan attack na tak tak an nei fo thei. Heng symptoms te hi hriat chian a pawimawh hle.

Natna tizualtu trigger te chu engte nge ni?

AHP vei chuan nun pangngaiin a nung thei a ni. Mahse, thil engemaw chuan natna hi a tichhuak thei a ni. Hengte hi fimkhur a pawimawh hle.

• Damdawi thenkhat: A bik takin damdawi thenkhat antibiotics `(Barbiturates)` leh `(Sulfa)` awmna te. AHP i neih chuan damdawi eng pawh i ei hmain doctor nen inbiakna neih hmasak a pawimawh.
• Zu in: Zu in hi he natna tizualtu ber a ni.
• Strict dieting or fasting: Calorie leh carbohydrates (starch) tlem zawk ei hian natna a ti na zual thei.
• Hormone inthlak danglamna: Hormone level inthlak danglamna, a bik takin hmeichhiaah, an thlasik hun nena inzawm.
• Stress leh Infection: Taksa chunga stress chi hrang hrang, khawsik emaw, khawsik emaw ang chi natna hrik kai theih pawhin natna a tizual thei a ni.

Engtikah nge doctor i hmuh ang?

Heng symptoms te hi i neih chuan a pangngai anga hnawhchhuah loh tur a ni. Damdawi lam thurawn lak a pawimawh hle a, a bik takin a hnuaia mi ang hian:

• Sawifiah loh, pum na nasa tak lo awm leh thin i neih chuan.
• Neurological symptoms, rilru hahna leh chak lohna te chu a ruala a awm chuan.
• I chhungkuaa tu emaw hian natna `(Porphyria)` a nei tih i hriat chuan.
• I zun chu dark red/brown color a lo ni tih i hriat chuan.

Natna nasa tak, tuar theih loh, thawk harsa, rilru buai emaw, taksa peng hloh emaw i neih chuan damdawi in Emergency Treatment Unit (ETU)-ah kal nghal rawh.

AHP enkawlna tur a awm em?

Hei hi genetic disease a nih avangin damdawi a la awm lo. Mahse, natna zual zel tur control leh venna tur damdawi tha tak tak a awm a.

A nasat chuan damdawi inah dah a ngai a, enkawl a ngai bawk.

• A bulpui ber chu natna lo chhuahna chhan (trigger) hriatchhuah a, tihtawp a ni.
• Natna nasa tak control nan painkiller chak tak tak pek thin a ni.
• Nausea leh luak chhuak control nan damdawi pek thin a ni.
• Taksa glucose (sugar), chu chu vein kaltlangin saline nena glucose pek a nih chuan liver-a toxins siamchhuahna a tlahniam thei a ni.
• Treatment bik angin , `(Heme)` injection te hi `(Hemin infusions)` tih a ni. Hei hi pawn lam atanga `(Heme)` pek a, "`(Heme)` i nei a, siam belh a ngai tawh lo," tiin liver-ah signal thawn a, toxin siam chhuah tihtawp a ni.
• Hei bakah hian tunah chuan natna zual fo thinte tana natna lo awm leh tur venna atana genetic technology hmanga siam chhuah damdawi tha tak tak a awm tawh bawk.

I enkawltu doctor chuan i tana enkawlna tha ber a ruat ang.

AHP, Acute Hepatic Porphyria, Porphyria, Pum na, Thluak lam natna, Genetic natna, Heme, Hemoglobin, Porphyrin, Natna hmuh tur awm lo