Zing lamah i zun chu a thim deuh a, thisen pawh a chhuak thei tih i hre ngai em? I chau reng reng a, i rilru a hah em em thin em? Hengte hi thisen natna hmuh tur awm lo, mi tam takin an hriat ngai loh PNH, emaw Paroxysmal Nocturnal Hemoglobinuria an tih chhinchhiahna a ni thei. He hming i hriat hian hlau suh. Engkim hi awlsam tak leh chiang takin sawi ila.
PNH hi eng nge ni tak tak?
A awlsam zawngin sawi ila, PNH hi kan taksa hriselna (immune system) ngeiin kan thisen sen hrisel tak takte a rawn beih palh a, a tihchhiat hi a ni. Ngaihtuah teh, he hmuna thil thleng hi ram pakhat humhalh tura ngaih sipai, chu ram mipuite bei ang mai a ni.
Hei hi kan ruhro chhunga cell thenkhatah hian genetic mutation a awm vang a ni. Chumi avang chuan chu ruhro atanga thisen sen siam chu tlem a chak lo deuh a ni. Heng cell chak lote hi awlsam takin kan immune system hian a hre thei a, a tichhia thei bawk.
- Thisen sen tihchhiat: Damdawi lam hawi chuan he thisen sen tihchhiatna hi hemolysis an ti a.
- Zun rawng inthlak danglam: Hemoglobin hi thisen sen (red blood cells)-a protein awm, oxygen phurtu a ni. Cell te a chhiat chuan he hemoglobin hi thisenah a chhuak a, kal (kidney)-in a filter a, zun-ah a paih chhuak thin. Hei hi hemoglobinuria an ti a . Hei vang hian zun hi a dum, a sen, a nih loh leh thisen rawng a lang thin. Hei hi zing zun-ah a langsar ber fo.
Mahse PNH symptoms hi mi zawng zawng tan a inang vek lo tih hre reng ang che. Mi thenkhat chuan zun rawng danglamna engmah an hmu lo mai thei.
He natna avanga harsatna lian tak dang chu thisen zam a awm theihna a sang zawk a ni . Heng clot thenkhat hi nunna atana hlauhawm tak a ni thei. Tin, he natna hian thisen sen (red blood cells) chauh ni lovin, thisen var, natna dotu leh thisen chhuah titawptu platelet te pawh a nghawng thei bawk.
PNH natna enkawl dan tur chu engte nge ni?
PNH enkawlna chu i natna lan chhuah dan leh i dinhmun nasat danah a innghat a ni. I doctor chuan eng enkawlna nge i tan a tha ber tih a sawipui ang che. A hnuaia table-ah hian tuna enkawlna awm mek thenkhat tarlan a ni.
| Enkawl dan tur | A awlsam zawngin, eng nge thleng? |
|---|---|
| Androgen hmanga enkawl a ni | Hengte hi mipa hormone chi khat a ni. Ruhro (bone marrow) a tichak a, thisen sen (red blood cells) siam chhuah a tipung bawk. Anemia tihziaawmna atan an pui thin. |
| Anticoagulants a awm bawk | Thisen kalna kawnga thisen zam (blood clots) a lo awm loh nan an veng a ni. Hengte hi injection emaw pill emaw angin a awm a. |
| Thisen pek a ni | Red blood cell emaw platelet level a hniam lutuk chuan a tlakchhamna chu pawn lam thisen pek hmangin a khum a ni. |
| Bone marrow thlak danglam a ni | Hei chauh hi PNH tihdam kim theihna kawng awmchhun a ni. Mahse a risky hle. Chuvangin, natna khirh ber tan chauh hman a ni. |
| Complement Inhibitors te pawh a awm | Thisen sen (red blood cells) tichhetu immune system-a part chu an target a, chu process chu an titawp ta a ni. Soliris leh Ultomiris te hi heng damdawi chi hrang hrang hi an ni. |
| Hmasawnna lam thil | Ruhro (bone marrow) tichaktu hmangin thisen sen emaw, thisen var emaw siam chhuah a tipung thei a ni. |
| Damdawi dang | Doctor chuan a tul angin folic acid, iron supplement, steroid, leh immunosuppressant te a pe bawk. |
Chuti a nih chuan he Empaveli hi eng nge ni?
Empaveli hi PNH enkawlna atana hman thin complement inhibitor damdawi thar leh tangkai tak a ni.
Kan immune system hi protein inzawmkhawm chain angin han ngaihtuah teh. He chain a\anga hmun khat chu `Complement system` an ti a. PNH-ah chuan he system hi a thawk nasa lutuk a, thisen sen (red blood cells) a tichhia a ni.
Damdawi hlui zawk (e.g. eculizumab) te chuan he chain-a C5 an tih hmun hi an target a. Thisen kalna chhunga tihchhiatna (intravascular hemolysis) chauh an titawp a ni.
Mahse he damdawi thar, Empaveli tia koh hian a hmaa hmun, C3 tih chu a target a ni. Hei hi a thatna chu thisen kalna kawng chhunga chhiatna awmte a thunun a, chubakah liver leh spleen ang chi taksa peng chhunga chhiatna (extravascular hemolysis) te pawh a control bawk.
Chuvangin, Empaveli hi damdawi ei tawh atanga dam kim lo leh thisen tlakchhamna nei chhunzawm zel tan chuan a hlawkpui hle thei a ni.
Empaveli hi engtin nge kan hman ang?
Empaveli hi in lama i hman theih tur damdawi a ni.
- Thiltih dan: Hei hi vun hnuaia injection a ni .
- Frequency: Kar khatah vawi hnih pek thin a ni .
- Dosage: I doctor chuan i thisena enzyme `Lactate Dehydrogenase (LDH)` tih level a enfiahin i dosage a siamrem thei ang. LDH level hian thisen sen engzat nge a tihchhiat tih a teh a ni.
- Hman dan: Hei hi hman dan kawng hnih a awm a. Pakhat chu pump te tak te hman a ni a, chu chuan damdawi chu minute 30-60 chhungin taksaah a thawn lut thin. A dang chu one-time injector system a ni a, chu chu pum chhungah hnawih a ni .
Lungngai suh, i doctor emaw, trained nurse emaw chuan hei hi hman dan tur a zirtir hmasa ang che.
He damdawi hman tangkai dan leh a him dan hriat tur
Empaveli hlawhtlinna hi clinical study lian pahnih PEGASUS leh PRINCE-ah te a chiang tawh a ni.
A awlsam zawngin, zirchianna pahnihah hian Empaveli ei thin damlote chu enkawlna dang ei thinte aiin hemoglobin level a sang zawk tih a chiang hle. Thisen pek pawh an tlem hle bawk.
Side effect leh himna a awm thei
Empaveli-a side effect hmuh tur lian ber berte chu:
- Injection na hmuna natna, sen emaw, vun emaw: Hei hi thil pangngai a ni. Ice pack hnawih emaw, tui lum takah i hnim emaw hmangin hahdamna i hmu thei.
- Infection hlauhawmna a sang: He damdawi hian immune system thenkhat a tihtawp avangin bacteria infection thenkhat (a bik takin encapsulated bacteria) vei theihna a awm a ni.
Hetiang hlauhawmna tihziaawm nan hian i doctor chuan Empaveli i ei tan hmain i mamawh vaccine zawng zawng a pe vek ang che. Damdawi i ei lai leh i ei tawh loh hnu thla 2 chhung chu Patient Safety Card i keng tel tur a ni. Hei hian i enkawl mekte chu emergency a nih chuan he damdawi hi i ei tih an hriat theih nan a pui ang.
Empaveli REMS program hi eng nge ni?
Hei hi thumal buaithlak deuh a ni a, mahse a awlsam zawngin, venhimna program bik tihna a ni . Empaveli ang ruihhlo kai theihna a nih avangin hetiang programme hi sorkar agency-te'n an kalpui thin a ni.
He `REMS (Risk Evaluation leh Mitigation Strategy)` programme hnuaiah hian:
1. I damdawi petu doctor chu he program-ah hian a inziak lut tur a ni.
2. Damdawi petu che pharmacy pawh hemi atan hian certified a ni tur a ni.
3. Doctor, damdawi ei hlauhawmzia leh natna hrik (khawsik, taksa na, lu na nasa tak) a lan chhuah dan hriat chian a ngai.
Hei hian he damdawi hi a thatna leh a hlauhawmzia i hre chiang hle a, him takin i hmang thei bawk.
Take-Home Thuchah a ni
- PNH hi natna hmuh tur awm lo mahse enkawl theih a ni a, nangmah ngei pawhin i immune system hian thisen sen (red blood cells) a tichhia a ni.
- Empaveli hi PNH enkawlna tha tak, thar tak a ni a, thisen cell tihchhiatna a titawp a ni.
- He damdawi hian natna a thlen thei a, chuvangin i doctor-in a pek che shot te hi la ngei ang che. Hri kai chhinchhiahna eng pawh fimkhur tur a ni.
- Empaveli hi special mechanism of action (REMS) hnuaiah pek a ni. Patient Safety Card i dawn chu i hnenah vawng reng ang che.
- I dinhmun emaw, i enkawl dan emaw chungchanga zawhna i neih chuan hreh lovin i doctor nen inbia ang che.
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