Kukho izifo ezixhaphakileyo phakathi kwethu, kunye nezifo ezingaqhelekanga, oko kukuthi, ezingaqhelekanga. Mhlawumbi awukaze uve ngesifo esinjalo. Namhlanje siza kuthetha ngesifo esingaqhelekanga, kodwa esibalulekileyo esinxulumene negazi esibalulekileyo ukusilawula ngokufanelekileyo. Igama laso yiParoxysmal Nocturnal Hemoglobinuria, esiyibiza ngokuba yiPNH ngamafutshane.
Ngamafutshane, yintoni i-PNH?
I-PNH ayisosifo semfuza, kodwa sisifo esidluliselwa kubazali okanye esidluliselwa ebantwaneni. Ingakhula nangaliphi na ixesha. Ngamafutshane, umntu onesi sifo unesifo apho
inkqubo yomzimba yokuzikhusela , inkqubo esikhusela kwizifo, ihlasela ngempazamo kwaye itshabalalise iiseli zayo ezibomvu zegazi. Cinga ngayo njengekhaka elenziwe ngeeproteni ezikhethekileyo ezisikhusela kwizifo. Ezinye iiseli ezibomvu zegazi emzimbeni wezigulane ze-PNH azinalo ikhaka elikhuselayo, oko kukuthi, ezo proteni zikhethekileyo. Ngoko ke inkqubo yomzimba yokuzikhusela iyaziqonda ezi seli ngaphandle kwekhaka, icinga ukuba zingumhlaseli oyingozi emzimbeni kwaye izitshabalalise. Nangona le meko inokuba yingozi ebomini,
ngonyango olufanelekileyo, unokulawula iimpawu, unciphise iingxaki kwaye uphile ubomi obuhle. Esi sifo asichaphazeli wonke umntu ngendlela efanayo. Abanye abantu banokungonwabi okuncinci. Abanye banokuchaphazeleka kakhulu yiyo.
Umngcipheko ophambili kukuqhekeka kwegazi. Malunga nama-30% ezigulane ze-PNH zisengozini yokuphuhlisa ukuqhekeka kwegazi ubuncinane kanye ebomini babo.
Yintoni isizathu soku?
Isizathu sesi sifo kukuguquka kwejini. Kodwa oku
akuyonto izuzwe njengelifa. Oko kuthetha ukuba asiyonto uyifumana kubazali bakho, kwaye asiyonto oyidlulisela kubantwana bakho. Olu lutshintsho olungaqhelekanga lwejini olwenzeka ebomini. Ngenxa yolu tshintsho lwejini, umongo wakho uqala ukuvelisa iiseli ezibomvu zegazi ezingaqhelekanga ezingenazo ezo proteni zokukhusela. Ngoko ke ekubeni ezi seli zingakhuselekanga yinkqubo yomzimba yokuzikhusela, ziyaqhekeka lula. Kwizonyango, sibiza le nkqubo yokuqhekeka kweeseli ezibomvu zegazi
njenge-hemolysis . Abanye oogqirha bakholelwa ukuba i-PNH inxulumene nobuthathaka kwimongo wethambo. Ngokukodwa, abantu abanemeko ebizwa ngokuba yi-aplastic anemia banamathuba amaninzi okufumana i-PNH. Kwakhona, abantu abane-PNH banokufumana i-aplastic anemia kamva. Kule meko ebizwa ngokuba yi-aplastic anemia, umongo wethambo uyeka ukuvelisa iiseli ezintsha zegazi.
Zithini iimpawu ze-PNH?
Esi sifo sibizwa ngegama lenye yeempawu zaso eziphambili. I-Paroxysmal ithetha "ukwenzeka ngequbuliso", i-Nocturnal ithetha "ebusuku", kwaye i-Hemoglobinuria ithetha "ukukhutshwa kwe-hemoglobin kumchamo". I-Hemoglobin yiproteni enika igazi umbala walo obomvu. Ngoko ke, xa iiseli ezibomvu zegazi ziqhekeka, le hemoglobin ikhutshwa emchameni,
kwaye umchamo ophuma ebusuku okanye kusasa unokuba mnyama (njenge-cola) okanye ubomvu . Malunga nama-50% abantu abane-PNH banamava olu phawu. Iimpawu zibangelwa ikakhulu zizizathu ezintathu:
Okukhona unezisele zegazi ezingasebenzi kakuhle emzimbeni wakho, kokukhona unokufumana iimpawu ezingakumbi.
Iimpawu eziqhelekileyo ezibangelwa kukwaphuka kweeseli ezibomvu zegazi kunye ne-anemia
| Ndiziva ndidiniwe kakhulu kwaye ndibuthathaka | Ukudinwa nokuba sele ndenze umsebenzi omncinci. |
| Intloko ebuhlungu | Intloko ebuhlungu rhoqo. |
| Ubunzima bokuphefumla | Ukuphefumla kancinci nokuba uhamba umgama omfutshane. |
| Ukubetha kwentliziyo | Ukuziva ngathi intliziyo yakho ibetha ngokukhawuleza. |
| Isisu esibuhlungu | Intlungu yesisu ngaphandle kwesizathu. |
| Ubunzima bokuginya | Ubunzima bokuginya ukutya okanye izinto ezilulwelo. |
| Ulusu olukhanyayo okanye olumthubi | Ukutyheli kwesikhumba kunye namehlo amhlophe. |
| Ukugruzuka lula | Nokuba ukwenzakala okuncinci kunokubangela imivumbo emikhulu. |
| Ukungasebenzi kakuhle ngokwesondo kumadoda | Ubunzima bokufumana okanye ukugcina ukuvuseleleka ngexesha lokwabelana ngesondo. |
Kubaluleke kakhulu: Ihlwili legazi yimeko eyingozi efuna unyango olungxamisekileyo. Ziqaphele ezi mpawu zilandelayo.
| Indawo apho igazi liqhekeke khona | Iimpawu ekufuneka uzijonge |
|---|
| Ulusu | Indawo ebomvu, ebuhlungu, okanye evuvukileyo eluswini. |
| Ingalo okanye Umlenze | Intlungu engalweni/emilenzeni, ukuziva ushushu, kunye nokudumba. |
| Isisu | Intlungu ebuhlungu esiswini, izilonda, kunye nokopha. |
| Ingqondo | Intloko ebuhlungu kakhulu enokuhlanza okanye ingenawo, ukuxhuzula, ubunzima bokushukuma, ukuthetha, okanye ukubona. |
| Imiphunga | Ubunzima bokuphefumla, iintlungu ezibukhali esifubeni njengemela, ukukhohlela igazi, ukubila. |
Ukuba unesikrokro sokuba unehlwili legazi elinje,
Yazisa ugqirha wakho ngoko nangoko. Yiya kwiSebe lezeMpilo eziNgxamisekileyo (ETU) elikufutshane nawe ngaphandle kokulibazisa, ingakumbi ukuba ufumana ubunzima bokuphefumla, ubunzima bokuthetha/ukubona, intloko ebuhlungu ngequbuliso, iintlungu zesifuba, okanye ukuxhuzula. Indlela yokuxilonga ngokuchanekileyo isifo?
Xa usiya kugqirha wakho, uza kukubuza ngeempawu zakho kunye nembali yakho yezonyango. Emva koko uza kukuxilonga aze akuyalele uvavanyo lwegazi. Uvavanyo oluphambili olwenziwayo koku luvavanyo lwegazi olukhethekileyo olubizwa ngokuba yi-flow cytometry . Oku kukuqinisekisa ukuba iiseli zakho ezibomvu zegazi zinezi proteins zikhuselayo. Ukongeza, inani leeseli zakho zegazi (inani elipheleleyo legazi) kunye namanqanaba esinyithi nawo ayajongwa. Ngamanye amaxesha isampuli yomongo wethambo nayo inokufuneka. Ziziphi iindlela zonyango ze-PNH?
Kukho iinjongo ezimbini eziphambili zokunyanga i-PNH. Enye kukunciphisa iimpawu, enye kukuthintela iingxaki eziyingozi (ingakumbi amahlwili egazi). Unyango olufumanayo luya kuxhomekeka kubukhali bemeko yakho.- Izondlo ezongezelelweyo: I-Folic acid kunye nezongezo zesinyithi zinikwa ukulawula i-anemia.
- Amayeza athile: Kukho amayeza afana ne-Eculizumab (Soliris) kunye ne-Ravulizumab (Ultomiris) athintela ukuqhekeka kweeseli ezibomvu zegazi. La mayeza anikwa ngemithambo yegazi. Aluncedo ekulawuleni i-anemia, ukunciphisa isidingo sotofelo-gazi lwangaphandle, kunye nokunciphisa umngcipheko wokuqhekeka kwegazi. I-Pegcetacoplan (Empaveli) lelinye iyeza elitsha elisetyenziselwa le njongo.
- Utofelo-gazi: Ukuba i-anemia inzima, uncedo lwexeshana lunokufunyanwa ngokunikela ngegazi kumntu ophilileyo.
- Izithibi zegazi: Izithibi zegazi zinikwa abantu abasengozini enkulu yokuqhekeka kwegazi.
- Ukufakelwa kweeseli zeBone Marrow Stem Cell : Le yindlela ekuphela kwayo yokunyanga ngokupheleleyo i-PNH. Oku kuquka ukususa umongo wethambo onesifo kunye nokufakelwa iiseli ze-stem kumntu ophilileyo (ngokufuthi umntakwabo). Nangona kunjalo, kuba le yinkqubo enobungozi kakhulu kwaye iyinkimbinkimbi, idla ngokucetyiswa kuphela kubantu abaselula abanesifo esibi kakhulu.
Ugqirha wakho uya kugqiba ukuba loluphi unyango olufanelekileyo kuwe. Kubaluleke kakhulu nokuzinyamekela.
Xa uhlala ne-PNH, kubaluleke kakhulu kunangaphambili ukuzinyamekela.- Ukutya okulungileyo: Yitya ukutya okunesondlo. Ukutya ukutya okune-iron eninzi (isipinatshi, inyama) kunye nento etyebileyo kwi -vitamin C (iiorenji, iitangerines) kunceda umzimba ukuba ufunxe i-iron ngcono.
- Ukuzilolonga: Ukudinwa kunokwenza kube nzima ukuzilolonga. Buza ugqirha wakho ukuba zeziphi izilolonga ezifanelekileyo kuwe.
- Zikhusele kwiintsholongwane : Hlamba izandla zakho rhoqo. Ziphephe iindawo ezinabantu abaninzi kunye neendawo apho kukho abantu abagulayo. Xelela ugqirha wakho ngoko nangoko ukuba unesifo somkhuhlane.
- Impilo yengqondo: Kuqhelekile ukuba uzive ulusizi, unomsindo, kwaye uxhalabile xa ufumanisa ukuba unesifo esingaqhelekanga esifana nesi. Ukuthetha ngezi mvakalelo nomntu othembekileyo okanye umcebisi kunokuba luncedo kakhulu.
- Ukukhulelwa: Ukuba ungumfazi oceba ukukhulelwa, qiniseka ukuba uqhagamshelana nogqirha wakho ngaphambi kokuba ukhulelwe. I-PNH inokuba yingozi kuwe nakumntwana wakho ngexesha lokukhulelwa.
Umyalezo Wokuya Ekhaya
- I-PNH sisifo segazi esingaqhelekanga esibangelwa kukuguquka kwezakhi zofuzo, kodwa asizuzwa njengelifa.
- Eyona ngxaki iphambili neyingozi yesi sifo kukuqhekeka kwegazi. Hlala uqaphela iimpawu zaso (intloko ebuhlungu kakhulu, iintlungu zesifuba, ubunzima bokuphefumla).
- Nangona oku kunokuba yingozi ebomini, kukho unyango olusebenzayo kakhulu olufumanekayo namhlanje lokulawula iimpawu nokuphucula umgangatho wobomi.
- Nangona ukufakelwa komongo wethambo kuphela konyango olunyanga ngokupheleleyo lwe-PNH, lunobungozi obukhulu kwaye lufanele kuphela izigulana ezikhethiweyo.
- Kubaluleke kakhulu ukuhlala unxibelelana rhoqo nogqirha wakho kwaye ulandele imiyalelo yakhe.
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