Wake waphawula ukuthi umchamo wakho umnyama kancane, mhlawumbe ngisho nogazi, ekuseni? Ingabe unenkinga yokuzizwa ukhathele ngaso sonke isikhathi, uzizwa uphaphathekile? Lokhu kungaba izimpawu zesifo segazi esingavamile abantu abaningi abangakaze bezwe ngaso esibizwa ngokuthi i-PNH, noma i-Paroxysmal Nocturnal Hemoglobinuria. Ungesabi uma uzwa leli gama. Ake sixoxe ngakho konke kalula nangokucacile.
Iyini ngempela i-PNH?
Kalula nje, i-PNH yilapho amasosha ethu omzimba ehlasela ngephutha futhi abhubhise amangqamuzana ethu egazi abomvu aphilile. Cabanga nje, okwenzekayo lapha kufana nebutho okufanele livikele izwe, lihlasele abantu balelo zwe.
Lokhu kungenxa yokuthi kukhona ukuguquka kwezakhi zofuzo kwamanye amangqamuzana asemongweni wethu wamathambo. Ngenxa yalokho, amangqamuzana abomvu egazi akhiqizwa yilowo mongo wamathambo abuthakathaka kancane. Lawa maseli abuthakathaka angabonakala kalula futhi abhujiswe amasosha ethu omzimba.
- Ukubhujiswa kwamangqamuzana abomvu egazi: Ngokwezokwelapha, lokhu kubhidlika kwamangqamuzana abomvu egazi kubizwa ngokuthi i-hemolysis .
- Umbala womchamo uyashintsha: I-Hemoglobin iyiprotheni etholakala kumaseli abomvu egazi athwala umoya-mpilo. Lapho amaseli ehlukana, le hemoglobin ikhishwa egazini, ihlungwe yizinso, bese ikhishwa emchameni. Lokhu kubizwa ngokuthi i-hemoglobinuria . Yingakho umchamo ubonakala umnyama, unsundu, noma unombala wegazi. Lokhu kuvame ukubonakala kakhulu emchameni wasekuseni.
Kodwa khumbula ukuthi izimpawu ze-PNH azifani kuwo wonke umuntu. Abanye abantu bangase bangaboni nhlobo ushintsho kumbala womchamo.
Enye inkinga enkulu yalesi sifo ingozi eyengeziwe yokuqhekeka kwegazi . Amanye ala maqhubu angasongela ukuphila. Futhi, lesi sifo asithinteli amangqamuzana abomvu egazi kuphela, kodwa futhi namaseli amhlophe egazi alwa nokutheleleka kanye nama-platelet amisa ukopha.
Yiziphi izindlela zokwelapha isifo se-PNH?
Ukwelashwa kwe-PNH kuncike ezimpawini zakho kanye nobunzima besimo sakho. Udokotela wakho uzoxoxa nawe ngokuthi yikuphi ukwelashwa okungcono kakhulu kuwe. Ithebula elingezansi libala ezinye zezindlela zokwelapha eziyinhloko ezitholakalayo njengamanje.
| Indlela yokwelapha | Kalula nje, kwenzekani? |
|---|---|
| Ukwelashwa kwe-Androgen | Lezi zinhlobo zama-hormone abesilisa. Zivuselela umongo wamathambo futhi zandise ukukhiqizwa kwamaseli abomvu egazi. Zisiza ekunciphiseni i-anemia. |
| Ama-anticoagulant | Zivimbela ukwakheka kwamahlule egazi emithanjeni yegazi. Lokhu kuyatholakala njengemijovo noma amaphilisi. |
| Ukumpontshelwa igazi | Uma amazinga amangqamuzana abomvu egazi noma ama-platelet eba phansi kakhulu, ukuntuleka kwegazi kukhokhelwa ngokumpontshelwa igazi ngaphandle. |
| Ukufakelwa umnkantsha wethambo | Lena yindlela kuphela yokwelapha i-PNH ngokuphelele . Kodwa iyingozi kakhulu. Ngakho-ke, isetshenziswa kuphela ezimweni ezimbi kakhulu. |
| Ama-Inhibitors Okugcwalisayo | Zihlasela ingxenye yesimiso somzimba sokuzivikela ebhubhisa amangqamuzana abomvu egazi futhi ziyeke leyo nqubo. I-Soliris ne-Ultomiris yizo lezi zinhlobo zemithi. |
| Izici zokukhula | Kuyasiza ukwandisa ukukhiqizwa kwamaseli egazi abomvu noma amhlophe ngokuvuselela umongo wamathambo. |
| Eminye imithi | Udokotela uphinde anikeze i-folic acid, izithasiselo zensimbi, ama-steroid, kanye nemithi yokucindezela amasosha omzimba uma kudingeka. |
Ngakho-ke iyini le Empaveli?
I-Empaveli iwumuthi omusha futhi osebenza kahle kakhulu wokuvimbela i-PNH.
Cabanga ngesimiso sethu somzimba sokuzivikela njengochungechunge lwamaprotheni axhumene. Ingxenye eyodwa yalolu chungechunge ibizwa ngokuthi 'uhlelo Lokugcwalisa'. Ku-PNH, lolu hlelo luba namandla ngokweqile futhi lubhubhise amangqamuzana abomvu egazi.
Imithi emidala (isib. i-eculizumab) yayihlose indawo ebizwa ngokuthi i-C5 kulolu chungechunge. Yayimisa kuphela ukubhujiswa ngaphakathi kwemithambo yegazi (i-intravascular hemolysis).
Kodwa lo muthi omusha, obizwa ngokuthi i-Empaveli, uhlose indawo yokuqala, ebizwa ngokuthi i-C3. Inzuzo yalokhu ukuthi ulawula umonakalo ngaphakathi kwemithambo yegazi, kanye nomonakalo ngaphakathi kwezitho ezifana nesibindi kanye ne-spleen (i-extravascular hemolysis).
Ngakho-ke, i-Empaveli ingaba usizo kakhulu ezigulini ezingakalulami ngokugcwele emithini yangaphambilini futhi eziqhubeka nokuba ne-anemia.
Ungayisebenzisa kanjani i-Empaveli?
I-Empaveli umuthi ongawusebenzisa ekhaya.
- Indlela: Lona umjovo we-subcutaneous .
- Imvamisa: Ngokuvamile inikezwa kabili ngesonto .
- Isilinganiso: Udokotela wakho angase alungise isilinganiso sakho ngokuhlola izinga le-enzyme ebizwa ngokuthi i-`Lactate Dehydrogenase (LDH)` egazini lakho. Amazinga e-LDH alinganisa ukuthi angakanani amaseli abomvu egazi ahlukaniswayo.
- Indlela yokusebenzisa: Kunezindlela ezimbili zokusebenzisa lokhu. Enye ukusebenzisa iphampu encane ehambisa umuthi emzimbeni imizuzu engama-30-60. Enye uhlelo lokujova kanye olusetshenziswa esiswini.
Ungakhathazeki, udokotela wakho noma umhlengikazi oqeqeshiwe uzoqala akufundise ukuthi ungayisebenzisa kanjani le ndlela.
Izinto okufanele uzazi ngokusebenza kahle nangokuphepha kwalo muthi
Impumelelo ye-Empaveli iqinisekiswe ezifundweni ezimbili ezinkulu zezokwelapha, i-PEGASUS kanye ne-PRINCE.
Kalula nje, kuzo zombili izifundo, iziguli ezathatha i-Empaveli zazinamazinga aphezulu kakhulu e-hemoglobin kunalezo ezathatha ezinye izindlela zokwelapha. Futhi zazinokufakwa kwegazi okumbalwa kakhulu.
Imiphumela emibi kanye nokuphepha
Imiphumela emibi eyinhloko ebonwa nge-Empaveli yile:
- Ubuhlungu, ukubomvu, noma ukuvuvukala endaweni yokujova: Lokhu kuvamile. Ungathola impumuzo ngokusebenzisa iphakethe leqhwa noma ukulifaka emanzini afudumele.
- Ingozi eyengeziwe yokutheleleka: Ngenxa yokuthi lo muthi ucindezela ingxenye yesimiso somzimba sokuzivikela, kunengozi yokuba sengozini yokutheleleka ngamagciwane athile (ikakhulukazi amagciwane ahlanganisiwe).
Ukuze unciphise le ngozi, udokotela wakho uzokunikeza yonke imijovo oyidingayo ngaphambi kokuba uqale ukuthatha i-Empaveli. Kufanele futhi ugcine ikhadi lokuphepha kwesiguli nawe ngenkathi uthatha umuthi kanye nezinyanga ezimbili ngemuva kokuyeka ukuwuthatha. Lokhu kuzosiza abantu abakwelaphayo ukuthi bazi ukuthi uthatha lo muthi uma kwenzeka isimo esiphuthumayo.
Luyini uhlelo lwe-Empaveli REMS?
Leli igama eliyinkimbinkimbi kancane, kodwa kalula nje, lisho uhlelo olukhethekile lokuvikela . Ngenxa yengozi yokutheleleka ngemithi efana ne-Empaveli, izinhlelo ezinjalo ziqaliswa yizikhungo zikahulumeni.
Ngaphansi kwalolu hlelo lwe-'REMS (Risk Evaluation and Mitigation Strategy)':
1. Udokotela okunikeza imithi yakho kumele abhaliswe kulolu hlelo.
2. Ikhemisi elikunikeza umuthi kumele liqinisekiswe ngalokhu.
3. Dokotela, udinga ukwazi kahle ngezingozi zomuthi kanye nezimpawu zokutheleleka (umkhuhlane, ubuhlungu bomzimba, ikhanda elibuhlungu kakhulu).
Lokhu kuqinisekisa ukuthi uyazi kahle izinzuzo nezingozi zalo muthi futhi uwusebenzisa ngokuphephile.
Umlayezo Wokuya Nawe Ekhaya
- I-PNH yisifo esingavamile kodwa esilawulekayo lapho amasosha omzimba wakho ebhubhisa khona amangqamuzana abomvu egazi.
- I-Empaveli iyindlela yokwelapha entsha nephumelelayo kakhulu ye-PNH evimba ukubhujiswa kwamangqamuzana egazi.
- Lo muthi ungabangela ukutheleleka, ngakho qiniseka ukuthi uthola imijovo udokotela wakho akunikeza yona. Qaphela noma yiziphi izimpawu zokutheleleka.
- I-Empaveli inikezwa ngaphansi kwendlela ekhethekile yokusebenza (REMS). Hlala ugcina ikhadi lokuphepha lesiguli olitholayo.
- Uma unemibuzo mayelana nesimo sakho noma ukwelashwa kwakho, sicela ukhulume nodokotela wakho ngaphandle kokungabaza.
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