Ngaba wakha waqaphela ukuba umchamo wakho umnyama kancinci, mhlawumbi ude ube negazi, kusasa? Ngaba unengxaki yokuziva udiniwe ngalo lonke ixesha, uzive uphaphathekile? Ezi zinokuba ziimpawu zesifo segazi esingaqhelekanga abantu abaninzi abangazange beve ngaso esibizwa ngokuba yi-PNH, okanye i-Paroxysmal Nocturnal Hemoglobinuria. Musa ukoyika xa usiva eli gama. Masithethe ngayo yonke into ngokulula nangokucacileyo.
Yintoni kanye kanye i-PNH?
Ngamafutshane, i-PNH ithetha ukuba amajoni ethu omzimba ahlasela ngempazamo aze atshabalalise iiseli zethu ezibomvu zegazi ezisempilweni. Khawucinge nje, okwenzekayo apha kufana nomkhosi omele ukhusele ilizwe, uhlasela abantu belo lizwe.
Oku kungenxa yokuba kukho utshintsho lwemfuza kwezinye zeeseli ezikumongo wethu wamathambo. Ngenxa yoko, iiseli ezibomvu zegazi eziveliswa yilo mongo zibuthathaka kancinci. Ezi seli zibuthathaka zinokubonwa kwaye zitshatyalaliswe lula yinkqubo yethu yokuzikhusela komzimba.
- Ukutshatyalaliswa kweeseli ezibomvu zegazi: Ngokwezonyango, oku kuqhekeka kweeseli ezibomvu zegazi kubizwa ngokuba yi-hemolysis .
- Umbala womchamo uyatshintsha: I-Hemoglobin yiproteni efumaneka kwiiseli ezibomvu zegazi ezithwala ioksijini. Xa iiseli ziqhekeka, le hemoglobin ikhutshwa egazini, ihluzwe zizintso, ize ikhutshwe kumchamo. Oku kubizwa ngokuba yi-hemoglobinuria . Yiyo loo nto umchamo ubonakala umnyama, umdaka, okanye unombala wegazi. Oku kudla ngokubonakala kakhulu kumchamo wasekuseni.
Kodwa khumbula ukuba iimpawu ze-PNH azifani kuye wonke umntu. Abanye abantu basenokungaluboni utshintsho kumbala womchamo kwaphela.
Enye ingxaki enkulu yesi sifo kukwanda komngcipheko wokuqhekeka kwegazi . Amanye ala mahlwili anokuba yingozi ebomini. Kwakhona, esi sifo sinokuchaphazela kungekuphela nje iiseli ezibomvu zegazi, kodwa neeseli ezimhlophe zegazi ezilwa nosulelo kunye neeplatelets eziyeka ukopha.
Ziziphi iindlela zonyango zesifo se-PNH?
Unyango lwe-PNH luxhomekeke kwiimpawu zakho kunye nobunzima bemeko yakho. Ugqirha wakho uza kuxoxa nawe ukuba loluphi unyango olukulungeleyo. Itheyibhile engezantsi idwelisa ezinye zeendlela eziphambili zonyango ezikhoyo ngoku.
| Indlela yonyango | Kalula nje, kwenzeka ntoni? |
|---|---|
| Unyango lwe-androgen | Ezi luhlobo lwehomoni yamadoda. Zivuselela umongo wamathambo kwaye zonyusa imveliso yeeseli ezibomvu zegazi. Zinceda ekunciphiseni i-anemia. |
| Izinto ezilwa nokugabha kwegazi | Zithintela ukwakheka kwamahlwili egazi kwimithambo yegazi. Ezi zifumaneka njengeenaliti okanye iipilisi. |
| Utofelo-gazi | Xa amanqanaba eeseli ezibomvu zegazi okanye iiplatelet ehla kakhulu, ukusilela kuyahlawulelwa ngokutofelwa igazi ngaphandle. |
| Ukufakelwa komongo wethambo | Le yindlela yodwa yokunyanga ngokupheleleyo i-PNH. Kodwa iyingozi kakhulu. Ngenxa yoko, isetyenziswa kuphela kwiimeko ezinzima kakhulu. |
| Ii-Inhibitors ezincedisayo | Zijolise kwinxalenye yenkqubo yomzimba yokuzikhusela etshabalalisa iiseli ezibomvu zegazi kwaye ziyeke loo nkqubo. I-Soliris kunye ne-Ultomiris zezi ntlobo zamayeza. |
| Izinto ezibangela ukukhula | Inceda ukwandisa imveliso yeeseli zegazi ezibomvu okanye ezimhlophe ngokuvuselela umongo wethambo. |
| Amanye amayeza | Ugqirha ukwanika i-folic acid, izongezo zesinyithi, ii-steroids, kunye ne-immunosuppressants xa kufuneka. |
Ngoko ke yintoni le Empaveli?
I-Empaveli liyeza elitsha nelisebenza kakuhle kakhulu lokuthintela isifo elisetyenziselwa ukunyanga i-PNH.
Cinga ngenkqubo yethu yomzimba yokuzikhusela njengoludwe lweeproteni ezidibeneyo. Inxalenye enye yale nkqubo ibizwa ngokuba yi-'Complement system'. Kwi-PNH, le nkqubo iyasebenza kakhulu ize itshabalalise iiseli ezibomvu zegazi.
Amayeza amadala (umz. i-eculizumab) ayejolise kwindawo ekule chain ebizwa ngokuba yi-C5. Ayemisa kuphela ukutshatyalaliswa ngaphakathi kwemithambo yegazi (i-intravascular hemolysis).
Kodwa eli yeza litsha, elibizwa ngokuba yi-Empaveli, lijolise kwindawo yangaphambili, ebizwa ngokuba yi-C3. Inzuzo yoku kukuba lilawula umonakalo ngaphakathi kwemithambo yegazi, kunye nomonakalo ngaphakathi kwamalungu anjengesibindi kunye ne-spleen (i-extravascular hemolysis).
Ngoko ke, i-Empaveli inokuba luncedo kakhulu kwizigulana ezingakaphili ngokupheleleyo kwiindlela zonyango zangaphambili kwaye eziqhubeka zine-anemia.
Ungayisebenzisa njani i-Empaveli?
I-Empaveli liyeza onokulisebenzisa ekhaya.
- Indlela: Le yinaliti efakwa ngaphantsi kwesikhumba .
- Izihlandlo: Idla ngokunikezelwa kabini ngeveki .
- Umlinganiselo: Ugqirha wakho angawulungisa umlinganiselo wakho ngokujonga inqanaba le-enzyme ebizwa ngokuba yi-`Lactate Dehydrogenase (LDH)` egazini lakho. Amanqanaba e-LDH alinganisa ukuba zingakanani iiseli ezibomvu zegazi eziqhekekayo.
- Indlela yokusebenzisa: Zimbini iindlela zokusebenzisa oku. Enye kukusebenzisa ipompo encinci ehambisa iyeza emzimbeni imizuzu engama-30-60. Enye yinkqubo yokujova kanye esetyenziswa esiswini.
Ungakhathazeki, ugqirha wakho okanye umongikazi oqeqeshiweyo uza kuqala akufundise indlela yokusebenzisa le nto.
Izinto ekufuneka uzazi malunga nokusebenza kakuhle kunye nokhuseleko lwale chiza
Impumelelo ye-Empaveli iqinisekiswe kwizifundo ezimbini eziphambili zeklinikhi, i-PEGASUS kunye ne-PRINCE.
Ngamafutshane, kuzo zombini ezi zifundo, izigulana ezithathe i-Empaveli zazinamanqanaba aphezulu kakhulu e-hemoglobin kunalezo ezithathe ezinye iindlela zonyango. Kwakhona zazinotofelo-gazi oluncinci kakhulu.
Iziphumo ebezingalindelekanga kunye nokhuseleko
Iziphumo ebezingalindelekanga ezibonwa nge-Empaveli zezi:
- Intlungu, ukubomvu, okanye ukudumba kwindawo efakwe kuyo inaliti: Oku kuqhelekile. Unokufumana isiqabu ngokufaka ipakethe yomkhenkce okanye ukuyifaka emanzini afudumeleyo.
- Umngcipheko okhulayo wosulelo: Ngenxa yokuba eli yeza lithintela inxalenye yenkqubo yomzimba yokuzikhusela, kukho umngcipheko wokuba semngciphekweni wosulelo oluthile lweentsholongwane (ingakumbi iintsholongwane ezifakwe ngaphakathi).
Ukuze kuncitshiswe lo mngcipheko, ugqirha wakho uza kukunika zonke izitofu ozidingayo ngaphambi kokuba uqale ukuthatha i-Empaveli. Kufuneka ugcine neKhadi loKhuseleko lweSigulana nawe ngelixa usela amayeza nakwiinyanga ezimbini emva kokuba uyekile ukuwasela. Oku kuya kunceda abantu abakunyangayo bazi ukuba usela la mayeza xa kukho imeko engxamisekileyo.
Yintoni inkqubo ye-Empaveli REMS?
Eli ligama elinzima kancinci, kodwa lithetha inkqubo yokukhusela ekhethekileyo . Ngenxa yomngcipheko wokosulelwa ngamayeza afana ne-Empaveli, iinkqubo ezinjalo ziqaliswa ziiarhente zikarhulumente.
Phantsi kwale nkqubo ye-`REMS (Risk Evaluation and Mitigation Strategy)`:
1. Ugqirha okunika amayeza akho kufuneka abhaliswe kule nkqubo.
2. Ikhemesti ekunika amayeza nayo kufuneka iqinisekiswe ukuba iyasebenza.
3. Gqirha, kufuneka ube nolwazi olupheleleyo malunga neengozi zamayeza kunye neempawu zosulelo (umkhuhlane, iintlungu zomzimba, intloko ebuhlungu kakhulu).
Oku kuqinisekisa ukuba uyazi kakuhle iingenelo kunye neengozi zale mithi kwaye uyisebenzisa ngokukhuselekileyo.
Umyalezo Wokuya Ekhaya
- I-PNH sisifo esingaqhelekanga kodwa esilawulekayo apho inkqubo yakho yomzimba itshabalalisa iiseli ezibomvu zegazi.
- I-Empaveli lunyango olutsha nolusebenzayo kakhulu lwe-PNH oluthintela ukutshatyalaliswa kweeseli zegazi.
- Eli yeza linokubangela usulelo, ngoko ke qiniseka ukuba ufumana imijovo enikwa ngugqirha wakho. Qaphela naziphi na iimpawu zosulelo.
- I-Empaveli inikezelwa phantsi kwendlela ekhethekileyo yokusebenza (REMS). Soloko ugcina ikhadi lokhuseleko lwesigulane olifumanayo kuwe.
- Ukuba unemibuzo malunga nemeko okanye unyango lwakho, nceda uthethe nogqirha wakho ngaphandle kokuthandabuza.
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