I zun rawng a danglam tawh em? I rilru a hah em? He thisen lama harsatna awm lo (PNH) chungchang hi i zir chiang ang u .

Kan thisen kal tlanga thisen luang chhuak chu nunna pe thei lui ang mai a ni lawm ni? He thisen chhunga thisen sen (red blood cells) te hian kan taksa chhunga cell tin mamawh oxygen an phur a. Chuvângin, heng thisen sen pawimawh tak takte hi a chhe nghâl a, a chhan hriat loha tihchhiat a nih chuan eng nge thleng ang tih han ngaihtuah teh? A nihna takah chuan a hlauhawm deuh. Vawiin hian thisen natna hmuh tur awm lo tak, mahse pawimawh tak, kan zavaia kan hriat chian a ngai kan sawi dawn a ni. Hei hi Paroxysmal Nocturnal Hemoglobinuria, a tawi zawngin PNH an ti a.

A awlsam zawngin PNH chu eng nge ni?

PNH hi thisen natna hmuh tur awm lo tak a ni. Hetiang a nih chuan , kan thisena thisen sen (red blood cells) te hi chhan awm lovin a chhe vek a ni. A dik tak chuan kan taksa hriselna (immune system) hian heng thisen sen (red blood cells) te hi a tichhe vek a ni. Hei hi a chhan chu he natna vei te thisen sen (red blood cells) hian anmahni vengtu protein chi khat bik an tlachham vang a ni. Chuvangin kan taksa immune system hian ‘kan cells’ angin a hre thei lo. Hetianga thisen sen (red blood cells) te hi rang taka an tihchhiat chuan kan ruhro (bone marrow) hian thisen sen thar (red blood cells) thar a siam thei lo a, chu chu a chakna ang chiah hian a siam thei lo. Tichuan taksaa thisen awm chu a tlahniam ta a ni. Hetiang dinhmun hi anemia kan ti a ni . Tin, he natna hlauhawm dang chu thisen kalna kawng chhunga thisen zam awm theihna a tipung a ni. PNH natna hian mi zawng zawng a nghawng dan inang vek lo. Mi thenkhat chuan symptoms na lo tak tak an nei thin. Nun pangngaiin an khawsa thei a ni. Mahse mi thenkhat tan chuan natna hi a na deuh deuh thei a ni. Damdawi leh thisen pek chhunzawm zel a ngai mai thei.

Eng thilin nge PNH natna hi a thlen?

Hetah hian a pawimawh ber chu PNH hi pianpui natna a ni lo tih hi a ni. Chumi awmzia chu nu atanga fa hnenah a kal lo tihna a ni. Tin, natna hrik kai theih a ni lo bawk. A nihna takah chuan natna hmuh tur awm lo tak a ni. America ang ramah hian kum tin cancer vei thar maktaduai 1.8 vel hmuhchhuah a nih hian PNH vei 400 atanga 500 vel chauh hmuhchhuah an ni tih han ngaihtuah teh. He natna hi kan ruhro (bone marrow) a stem cells-a gene pakhat `PIGA` an tih (mutation) avanga lo awm a ni. A awlsam zawngin sawi ila, thisen sen siamna factory, ruhro (bone marrow)-in ‘thupek’ a dawnah hian tihsual tlem a awm a. Hetiang tihsual avang hian thisen sen (red blood cells) lo awm mekte hian chu venhimna protein coating chu an hmu lo.

Nang pawhin heng symptoms te hi i nei ve em?

Thisen sen (red blood cells) a chhiat chuan an chhunga hemoglobin an tih red pigment chu thisenah a chhuak ta a ni. Heng bawlhhlawh te hi zun chhunga kal (kidney) kaltlangin taksa atanga lakchhuah a ni. Chuvangin, zun rawng chu a tlangpui aia sen, sen, a nih loh leh a dum zawkin a lang thei . Mahse, PNH vei zawng zawng hian zun rawng hrang hrang an nei vek lo. Damlo zinga a chanve chauhvin he symptom hi an nei thin. Tin, a bik takin zan lama an zun chhuah emaw, zing an thawh hmasak ber emaw-ah chuan a thim hle bawk. He symptom ber bakah hian symptom dang engemaw zat a awm thei bawk. Eng nge an nih i en ang u.

He natna hi tuin nge vei tam ber?

Doctor-te sawi dan chuan PNH vei theihna hi mi thenkhat tan chuan a sang deuh hlek a ni.

• Ruhro chak lo nei tan : Eng emaw chhan vanga ruhro hnathawh a chak lo a nih chuan.
• Aplastic Anemia an tih natna nei tan : Hei hi ruhro (bone marrow) tichhe thei natna a ni bawk. He natna vei te hi a hnuah PNH vei an hlauhawm hle.
• Asian hnam chhuak mite tan: He natna hi hnam dang aiin Asia mite zingah a tam zawk.

PNH hi kum 30 hnuai lam puitlingah hmuhchhuah a ni tlangpui a, mahse naupang zawkah pawh a awm thei a, naupang leh nausen te pawh a awm thei bawk.

Engtin nge natna hi kan hriat theih ang?

PNH hi natna hmuh tur awm lo tak a nih avangin leh a natna lan chhuah dan pawh natna dang nen a inang em em a, a chang chuan natna hriatchhuah nan hun rei tak a la ngai thei a ni. A bik takin mi zun rawng danglam lo tan chuan doctor-in hei hi a rinhlelh loh chuan diagnosis a tikhawtlai thei a ni. Natna hriat chian nan i doctor chuan i natna lan chhuah dan leh i damdawi lam chanchin a zawt hmasa ang che. Tichuan, thisen test chi hrang hrang a order ang. Chung zinga PNH finfiah nan test tha ber leh dik ber chu thisen test bik `Flow Cytometry` tih hi a ni. He test hian i thisena chu thisen venna protein chu red blood cell engzat nge a tlakchham tih chiang takin a teh thei a ni.

PNH enkawl dan tur chu engte nge ni?

Vanneihthlak takin tunah hian PNH enkawlna atana enkawlna tha tak tak engemaw zat a awm tawh a ni. I doctor chuan i dinhmun a zirchiang ang a, i tana enkawlna tha ber tur chu a ruat ang.

• Immune system suppressors: Damdawi eculizumab (Soliris) leh ravulizumab ( Ultomiris ) te hian kan immune system hian thisen sen a tihchhiat loh nan hna a thawk a. Chungte chuan thisen zam theihna a ti tlem bawk.
• Steroids: Hengte hi a chang chuan thisen sen (red blood cells) tihchhiatna control nan an hmang thin.
• Thisen tithianghlimtu: 1.1.Hengte hi thisen zam awm theihna tihziaawmna tura pek a ni.
• Thisen pek dan: Taksa thisen a tlem lutuk chuan pawn lam atanga thisen pek a ni a, chu chuan thisen sen (red blood cells) a tipung a ni.

Tin, taksa chhungril lama thisen sen (red blood cells) tam zawk siam chhuahna tura puitu enkawlna a awm bawk.

• Vitamin te hi folic acid ang chi te an ni
• Mihring thanlenna factor
• Androgen, mipa hormone a ni

Bone marrow transplant hian PNH hi a tidam vek thei a ni. Mahse, surgery risky leh complex tak a nih avangin doctor-te chuan enkawlna dang zawng zawng a hlawhchham chuan chauh an rawt a ni. PNH Sinhala, Paroxysmal nocturnal hemoglobinuria, thisen natna, red blood cell chhe, zun dum, ruhro natna, thisen tlakchhamna