Koj puas tau pom tias koj cov zis tsaus me ntsis, tej zaum txawm tias muaj ntshav, thaum sawv ntxov? Koj puas muaj teeb meem nrog kev xav tias nkees txhua lub sijhawm, xav tias dawb? Cov no yuav yog cov tsos mob ntawm tus kab mob ntshav tsawg tsawg uas ntau tus neeg tsis tau hnov txog hu ua PNH, lossis Paroxysmal Nocturnal Hemoglobinuria. Tsis txhob ntshai thaum koj hnov lub npe no. Cia peb tham txog txhua yam yooj yim thiab meej.
PNH yog dab tsi tiag?
Hais yooj yim xwb, PNH yog thaum peb lub cev tiv thaiv kab mob ua yuam kev tawm tsam thiab rhuav tshem peb cov qe ntshav liab uas noj qab nyob zoo. Xav txog seb qhov xwm txheej no zoo li ib pab tub rog uas yuav tsum tiv thaiv ib lub teb chaws, tawm tsam cov neeg ntawm lub teb chaws ntawd.
Qhov no yog vim muaj kev hloov pauv caj ces hauv qee cov hlwb hauv peb cov pob txha. Yog li ntawd, cov hlwb ntshav liab uas tsim los ntawm cov pob txha ntawd yuav qaug zog me ntsis. Cov hlwb tsis muaj zog no yooj yim pom thiab rhuav tshem los ntawm peb lub cev tiv thaiv kab mob.
- Kev puas tsuaj ntawm cov qe ntshav liab: Hauv kev kho mob, qhov kev tawg ntawm cov qe ntshav liab no hu ua hemolysis .
- Xim zis hloov pauv: Hemoglobin yog ib qho protein uas pom muaj nyob rau hauv cov qe ntshav liab uas nqa oxygen. Thaum cov qe ntshav tawg, cov hemoglobin no raug tso tawm mus rau hauv cov ntshav, lim los ntawm lub raum, thiab tso tawm hauv cov zis. Qhov no hu ua hemoglobinuria . Qhov no yog vim li cas cov zis zoo li tsaus, xim av, lossis xim ntshav. Qhov no feem ntau pom tseeb tshaj plaws hauv cov zis thaum sawv ntxov.
Tiam sis nco ntsoov tias cov tsos mob ntawm PNH tsis zoo ib yam rau txhua tus. Ib txhia neeg yuav tsis pom muaj kev hloov pauv ntawm cov xim zis li.
Lwm yam teeb meem loj ntawm tus kab mob no yog kev pheej hmoo ntawm cov ntshav txhaws . Qee cov ntshav txhaws no tuaj yeem ua rau tuag taus. Tsis tas li ntawd, tus kab mob no tuaj yeem cuam tshuam tsis yog cov qe ntshav liab xwb, tab sis kuj tseem cuam tshuam cov qe ntshav dawb uas tawm tsam kev kis kab mob thiab cov ntshav platelets uas tiv thaiv kev los ntshav.
Cov kev kho mob rau tus kab mob PNH yog dab tsi?
Kev kho mob rau PNH nyob ntawm koj cov tsos mob thiab qhov hnyav ntawm koj tus mob. Koj tus kws kho mob yuav tham nrog koj seb kev kho mob twg zoo tshaj plaws rau koj. Lub rooj hauv qab no teev qee qhov kev kho mob tseem ceeb uas muaj tam sim no.
| Txoj kev kho mob | Hais yooj yim xwb, ua li cas lawm? |
|---|---|
| Kev kho mob Androgen | Cov no yog ib hom tshuaj hormones txiv neej. Lawv ua rau cov pob txha pob txha ua haujlwm thiab ua rau cov qe ntshav liab ntau ntxiv. Lawv pab txo cov ntshav tsis txaus. |
| Cov tshuaj tiv thaiv kev coagulant | Lawv tiv thaiv cov ntshav txhaws kom tsis txhob lo rau hauv cov hlab ntsha. Cov no muaj nyob rau hauv daim ntawv txhaj tshuaj lossis tshuaj ntsiav. |
| Kev hloov ntshav | Thaum cov qe ntshav liab lossis cov platelet qis heev, qhov tsis txaus no yuav raug them rov qab los ntawm kev hloov ntshav sab nraud. |
| Kev hloov pob txha | Nov yog tib txoj kev los kho PNH kom zoo . Tab sis nws muaj kev pheej hmoo heev. Yog li ntawd, nws tsuas yog siv rau cov mob hnyav tshaj plaws xwb. |
| Cov Tshuaj Tiv Thaiv Ntxiv | Lawv tsom mus rau qhov ntawm lub cev tiv thaiv kab mob uas rhuav tshem cov qe ntshav liab thiab nres qhov txheej txheem ntawd. Soliris thiab Ultomiris yog cov tshuaj no. |
| Cov yam tseem ceeb ntawm kev loj hlob | Nws pab kom muaj cov qe ntshav liab lossis dawb ntau ntxiv los ntawm kev txhawb nqa cov pob txha. |
| Lwm cov tshuaj | Tus kws kho mob kuj tseem sau ntawv kom noj cov tshuaj folic acid, cov tshuaj ntxiv hlau, cov tshuaj steroids, thiab cov tshuaj tiv thaiv kab mob raws li qhov xav tau. |
Yog li ntawd, Empaveli no yog dab tsi?
Empaveli yog ib qho tshuaj tshiab thiab muaj txiaj ntsig zoo rau kev kho mob PNH.
Xav txog peb lub cev tiv thaiv kab mob zoo li ib txoj saw hlau uas muaj cov protein sib txuas ua ke. Ib feem ntawm txoj saw hlau no hu ua 'Complement system'. Hauv PNH, lub cev no ua haujlwm ntau dhau thiab rhuav tshem cov qe ntshav liab.
Cov tshuaj qub dua (piv txwv li eculizumab) tsom mus rau qhov chaw hu ua C5 hauv cov saw no. Lawv tsuas yog tiv thaiv kev puas tsuaj sab hauv cov hlab ntsha (intravascular hemolysis).
Tiamsis cov tshuaj tshiab no, hu ua Empaveli, tsom mus rau qhov chaw ua ntej, hu ua C3. Qhov zoo ntawm qhov no yog tias nws tswj kev puas tsuaj hauv cov hlab ntsha, nrog rau kev puas tsuaj hauv cov kabmob xws li lub siab thiab lub zais zis (extraped hemolysis).
Yog li ntawd, Empaveli tej zaum yuav muaj txiaj ntsig zoo rau cov neeg mob uas tseem muaj ntshav tsis txaus thiab cov neeg uas tsis tau zoo tag nrho los ntawm cov tshuaj yav dhau los.
Yuav siv Empaveli li cas?
Empaveli yog ib hom tshuaj uas koj siv tau hauv tsev.
- Txoj Kev: Qhov no yog kev txhaj tshuaj subcutaneous .
- Zaus: Feem ntau muab ob zaug hauv ib lub lim tiam .
- Kev Noj Tshuaj: Koj tus kws kho mob yuav hloov koj cov tshuaj los ntawm kev kuaj xyuas qib ntawm cov enzyme hu ua 'Lactate Dehydrogenase (LDH)' hauv koj cov ntshav. Cov qib LDH ntsuas seb cov qe ntshav liab raug rhuav tshem ntau npaum li cas.
- Yuav siv li cas: Muaj ob txoj hauv kev siv qhov no. Ib qho yog siv lub twj me me uas xa cov tshuaj mus rau hauv lub cev li 30-60 feeb. Qhov thib ob yog lub tshuab txhaj tshuaj ib zaug xwb uas siv rau ntawm lub plab.
Tsis txhob txhawj, koj tus kws kho mob lossis tus kws tu mob uas tau kawm tiav yuav qhia koj siv qhov no ua ntej.
Tej yam yuav tsum paub txog qhov ua tau zoo thiab kev nyab xeeb ntawm cov tshuaj no
Qhov kev vam meej ntawm Empaveli tau lees paub hauv ob txoj kev tshawb fawb loj hauv kev kho mob, PEGASUS thiab PRINCE.
Hauv ob txoj kev tshawb fawb, cov neeg mob uas tau noj Empaveli muaj cov hemoglobin ntau dua li cov neeg uas tau noj lwm yam kev kho mob. Lawv kuj tau txais cov ntshav tsawg dua.
Cov kev mob tshwm sim thiab kev nyab xeeb
Cov kev mob tshwm sim tseem ceeb uas pom nrog Empaveli yog:
- Mob, liab, lossis o ntawm qhov chaw txhaj tshuaj: Qhov no yog ib txwm muaj. Koj tuaj yeem nrhiav kev pab los ntawm kev siv lub hnab dej khov lossis muab nws tso rau hauv dej sov.
- Kev pheej hmoo ntawm kev kis kab mob ntau ntxiv: Vim tias cov tshuaj no tswj ib feem ntawm lub cev tiv thaiv kab mob, muaj kev pheej hmoo ntawm kev kis kab mob rau qee yam kab mob (tshwj xeeb tshaj yog cov kab mob encapsulated).
Yuav kom txo qhov kev pheej hmoo no, koj tus kws kho mob yuav muab tag nrho cov tshuaj tiv thaiv kab mob uas koj xav tau ua ntej koj pib noj Empaveli. Koj yuav tsum khaws Daim Npav Kev Nyab Xeeb rau Tus Neeg Mob nrog koj thaum koj noj tshuaj thiab rau 2 lub hlis tom qab koj tsum tsis noj nws. Qhov no yuav pab cov neeg uas kho koj paub tias koj tab tom noj tshuaj no yog tias muaj xwm txheej ceev.
Qhov kev pab cuam Empaveli REMS yog dab tsi?
Lo lus no nyuaj me ntsis, tab sis hais yooj yim xwb, nws txhais tau tias yog ib qho kev tiv thaiv tshwj xeeb . Vim muaj kev pheej hmoo ntawm kev kis tus kab mob nrog cov tshuaj zoo li Empaveli, cov kev pab cuam no raug siv los ntawm cov koom haum tsoomfwv.
Nyob rau hauv qhov kev pab cuam 'REMS (Kev Ntsuam Xyuas Kev Pheej Hmoo thiab Kev Txo Qhov Kev Pheej Hmoo)' no:
1. Tus kws kho mob uas sau ntawv rau koj cov tshuaj yuav tsum tau sau npe rau hauv qhov kev pab cuam no.
2. Lub tsev muag tshuaj uas muab cov tshuaj rau koj kuj yuav tsum tau ntawv pov thawj rau qhov no.
3. Kws kho mob, koj yuav tsum paub zoo txog tej kev pheej hmoo ntawm cov tshuaj thiab cov tsos mob ntawm kev kis kab mob (kub taub hau, mob lub cev, mob taub hau hnyav).
Qhov no ua kom koj paub txog cov txiaj ntsig thiab kev pheej hmoo ntawm cov tshuaj no thiab siv nws kom muaj kev nyab xeeb.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- PNH yog ib yam kab mob uas tsis tshua muaj tab sis koj tswj tau uas koj lub cev tiv thaiv kab mob ua rau cov qe ntshav liab puas tsuaj.
- Empaveli yog ib txoj kev kho mob tshiab zoo heev rau PNH uas tiv thaiv kev puas tsuaj ntawm cov qe ntshav.
- Cov tshuaj no tuaj yeem ua rau muaj kev kis kab mob, yog li nco ntsoov mus txhaj tshuaj tiv thaiv kab mob uas koj tus kws kho mob muab rau koj. Ceev faj txog txhua yam cim qhia tias muaj kev kis kab mob.
- Empaveli muaj nyob rau hauv ib txoj kev ua haujlwm tshwj xeeb (REMS). Nco ntsoov khaws Daim Npav Kev Nyab Xeeb ntawm Tus Neeg Mob uas koj tau txais nrog koj.
- Yog koj muaj lus nug txog koj tus mob lossis kev kho mob, thov nrog koj tus kws kho mob tham yam tsis muaj kev yig.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus