I hah reng, tlem i kal laiin thawk hah, zing lama zun dum dum (dark brown urine) ang chi symptoms i nei em? A châng chuan hengte hi thisen lama natna hmuh tur awm lo tak, Paroxysmal Nocturnal Hemoglobinuria, emaw, keini doctor-te’n kan tih angin PNH emaw chhinchhiahna a ni thei bawk. He hming i hriat hian hlau suh. Tlem a buaithlak viau nachungin he natna leh a enkawlna thar Fabhalta tih hi a awlsam leh chipchiar takin sawi ila.
PNH hi eng nge ni tak tak?
A awlsam zawngin sawi ila, PNH hi thisen lama harsatna awm lo tak, genetic defect avanga lo awm a ni. Kan thisen sen (RBC) te hian an chhehvel ah hian protective protein coating an nei a. Mahse, PNH-ah chuan he protective coating hi a lo awm lo.
Kan thisen sen (red blood cells) te hi ralthuam nei lo sipai angin han ngaihtuah teh. Hetiang venhimna tel lo chuan kan taksa ngei pawh hi a buai hle a, heng thisen sen hrisel tak takte hi hmelma anga ngaihin a bei a, a tichhe tan ta a ni. Hetianga thisen sen (red blood cells) a hun hmaa a chhe lutuk hi damdawi lam chuan hemolysis kan tih hi a ni . He chhiatna hi thisen kalna kawng chhungah (intravascular) leh thisen kalna kawng pawnah (extravascular) a thleng thei a ni.
Hetianga thisen zam (red blood cells) a chhiat chuan a lan chhuahna ber chu thisen tlakchhamna (anemia) leh thisen zam (blood clots) a ni thei. Heng thisen zamte hi a chang chuan nunna atana hlauhawm tak a ni thei. PNH vei chuan heng symptom tam tak hi a nei thei a, a nih loh leh tlemte chauh a nei thei bawk.
| PNH natna lan chhuah dan tlangpui | |
|---|---|
| Chauhna emaw chak lohna emaw reng reng | Thâwk harsa |
| Lu na | Lungphun mumal lo emaw, rang tak emaw |
| Pum natna | Vun awlsam taka hliam thei |
| Zun rawng dum (brown), a bik takin zanah emaw, zing lamah emaw | Vun hmun bik a sen, natna, leh vun hring (thisen chhuak chhinchhiahna a ni thei) . |
PNH enkawlnaah hian thisen pek te, thisen tlakchhamna atana folic acid emaw iron tablet emaw, anticoagulants, leh thisen sen (red blood cells) te chu immune system-in a beih loh nan a venghimtu damdawi te a ni. Enkawlna i dawn tur chu i natna lan chhuah dan leh a nasat danah a innghat ang.
Damdawi Fabhalta hian engtin nge hna a thawh?
PNH injection atana pek thin damdawi eculizumab (Soliris) emaw ravulizumab (Ultomiris) te hian kan immune system thenkhat complement system an tih, C5 an tih chu block in hna a thawk thin. Hengte hian intravascular hemolysis a tihtawp laiin, mi thenkhat chuan extravascular hemolysis vangin thisen tlakchhamna an nei chhunzawm zel thei.
Hei hi Fabhalta an tih damdawi ei hmasak ber a pawimawhna hmun a ni.
Kan immune system hi kan ram humhimtu sipai angin han ngaihtuah teh. PNH-ah chuan he sipai thenkhat hi an buai a, kan thisen sen hrisel tak takte chu an bei thin. Fabhalta hian he buaina siamtu protein factor B an tih chu a block a ni. Hei hian immune system-in thisen sen (red blood cells) a beih chu a titawp a ni. Hei hian thisen sen hrisel tak tak a tipung a, natna lan chhuah dan a ti tlem a, thisen pek ngai a ti tlem bawk.
He damdawi hi engtin nge ka hman ang?
Fabhalta hi capsule a ni a, ni khatah vawi hnih a pumin ei tur a ni a, ei tur nen emaw ei loh pawhin ei tur a ni. Capsule chu tichhia suh, tikehsawm suh, chew suh. Dose i miss a nih chuan i hriatreng veleh ei la, a bak chu a hma ang bawkin ei leh rawh. PNH damdawi dang (eculizumab emaw ravulizumab emaw ang chi) i ei tawh a nih chuan i doctor chuan Fabhalta lama inthlak dan dik a hrilhfiah ang che.
Pawimawh tak: He enkawlna i tan hma kar hnih tal i vaccine mamawh zawng zawng i pek vek a pawimawh. Hei hian pneumococcal vaccine leh meningitis vaccine te a huam a ni. I vaccine i dawng thei lo a, Fabhalta i tan thei lo a nih chuan i doctor chuan vaccine te chu a pe ang che a, chutih rual chuan antibiotics course a tan tir ang che.
I natna lan chhuah dan a lo tha zawk a nih pawhin damdawi lam thurawn tel lovin Fabhalta ei kha bansan suh . Chutianga i tih chuan i thisen sen (red blood cells) te chu a chhe chak lehzual thei a ni.
Engtin nge he damdawi hian nghawng a neih dan hi zirchian a nih?
Fabhalta hman tangkai dan leh a him leh him loh finfiah nan zirchianna lian pahnih neih a ni. An pahnih hian PNH vei leh a vanga thisen tlakchhamna nei damlote an tel a ni.
1. APPLY-PNH study: Hei hi PNH damdawi dang (eculizumab emaw ravulizumab emaw) thla 6 tal ei chung pawha thisen tlakchhamna nei te an ni. Pawl hnihah an inthen a, pawl khat chu Fabhalta pek an ni a, pawl dang chuan damdawi hlui chu an ei chhunzawm zel a.
2. APPOINT-PNH study: Hei hi damlo, a hmaa PNH atana complementary medicine dang la lote an ni. Fabhalta pek vek an ni.
Heng zirchianna result, kar 24 hnuah pawh hian a tichak hle.
| Benefit dawn a ni | Nghawngchhuah |
|---|---|
| Hemoglobin level a sang chho zel | Fabhalta ei zinga 82% aia tam chuan hemoglobin chu 2 g/dL-in a pung a, damdawi dang ei zinga pakhat mah an nei lo. |
| Thisen pek loh tur | Fabhalta ei zinga 95% chuan zirchianna hun chhungin pawn lam thisen an mamawh lo a, damdawi dang ei zinga 50% chauh an ni. |
Heng zirchiannate result hi ni mah se, i result chu mi hrang hrangah a inang lo thei tih hre reng ang che. Thutlukna siam hmain doctor nen damdawi hman thatna leh hlauhawmna te sawiho hmasak a tha ber.
Engtin nge damdawi hian hna a thawk em tih i hriat theih ang?
Fabhalta enkawlna a hlawhtlin chuan i thisen sen leh hemoglobin level a sang tan ang. Hetiang hi i tawng thei a ni:
- Energy a tipung a, hahna a tlahniam bawk
- Zun rawng dum a tlahniam
- Ei duhna a tipung a, pum na a ti tlem bawk
- Pawn lam thisen pek ngai tihziaawmna
- Nitin hna awlsam taka thawk thei
Enkawlna result hi mi tin a inang lo a nih avangin i natna lan chhuah dan record la, i doctor i hmuh apiangin i doctor nen sawiho rawh.
Side effects leh engtin nge kan enkawl ang?
Damdawi dang ang bawkin Fabhalta hian side effect a nei thei a ni. A tam ber chu lu na, luak chhuak, luak chhuak, pum na, vun natna te hi a ni. Khawsik natna lanchhuahna leh bacteria emaw virus emaw infection dang pawh a awm thei bawk.
Infection na tak tak ngaihven ngai!
He damdawi hian immune system thenkhat a tihbuai avangin bacteria chi hrang hrang atanga natna khirh tak tak kai theihna a awm a ni. A bik takin *Streptococcus pneumoniae*, *Neisseria meningitidis*, leh *Haemophilus influenzae type B* te hi a ni. Heng hri kai te hi hriatchhuah leh enkawl vat loh chuan nunna atana hlauhawm tak a ni thei.
Infection laka invenna tha ber chu vaccination recommended zawng zawng lak vek a ni , i kut silfai tha la, damlote laka inthiarfihlim hi a ni. A hnuaia symptoms te hi i neih chuan doctor hnenah hrilh nghal la, a nih loh leh hospital emergency room (ETU) hnai ber pan rawh.
| Infection na tak a awm tih hriattirna | |
|---|---|
| Phlegm nei emaw nei lo emaw khawsik, rilru na leh khuh, thinlung na rang | Taksa na leh khawsik ang chi symptoms te |
| Vun hring (clammy skin) a ni | Lu na tak tak, khawsik, luak chhuak, leh luak chhuak, emaw, kawng/ hnungzang na tak tak |
| Rilru tibuai | Vision a danglam thin |
Side effect eng emaw i ngaihtuah chuan doctor nen inbia nghal rawh. Damdawi atanga poison i rinhlelh chuan Colombo National Hospital-a National Poisons Information Center ah i call thei ang .
Enkawl lai hian eng thisen test nge ngai?
I doctor chuan i hriselna leh damdawi i dawn dan enfiah nan thisen test eng emaw zat a ti ang.
- Thisen sen (RBC) leh hemoglobin level: 1.1.Hengte hi PNH-ah enfiah fo thin a ni. Chu chuan enkawlna i chhân lêt dân hriat theih nân leh thisen pêk i mamawh leh mamawh loh hriat nân a ṭanpui a ni.
- Thisen thau level: Fabhalta hian i cholesterol leh triglyceride level a tisang thei a ni. Chuvangin, i doctor chuan enkawl hma leh enkawl lai hian fasting lipid profile test neih a rawt ang.
Damdawi dangah harsatna a awm thei em?
Ni e, damdawi thenkhat chu Fabhalta nen an inzawm thei. Damdawi thenkhat, Rifampin ang chi te hian Fabhalta thatna a ti tlem thei a ni. Fabhalta hi damdawi thenkhat, Gemfibrozil ang chi te nen ei hi a tha lo.
Chuvangin, i damdawi ei zawng zawng – prescription damdawi, over-the-counter damdawi, vitamin, traditional medicine, leh supplement thlengin i doctor hriattir a pawimawh hle .
Take-Home Thuchah a ni
- PNH hi thil awm lo tak a ni a, taksain a taksain a venghimtu protein layer a tlakchham avangin thisen zam (Red blood cells) a tichhe vek a ni.
- Fabhalta hi PNH tan damdawi thar ei tur a ni a, immune system-a malfunction tihtawp a, thisen sen (red blood cells) venhimna hmangin hna a thawk a ni.
- He damdawi hi ei tan hma hian meningitis leh pneumonia ang chi natna laka vaccine lak tawh a ngai a ni.
- Inenkawl laiin natna khirh tak tak chhinchhiahna i neih chuan khawsik, lu na nasa tak, emaw, buaina emaw i neih chuan doctor thurawn zawng nghal rawh.
- I natna lan chhuah dan a tha zawk a nih pawhin doctor thurawn tel lo chuan damdawi ei kha bansan ngai suh.
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