Igazi elihamba ngemithambo yethu lifana nomlambo onika ubomi, akunjalo? Iiseli ezibomvu zegazi kweli gazi zithwala ioksijini efunekayo kwiseli nganye emzimbeni wethu. Khawucinge nje ukuba kuya kwenzeka ntoni ukuba ezi seli zibomvu zegazi zibaluleke kakhulu ziqhekeka ngequbuliso zize zitshatyalaliswe ngaphandle kwesizathu esicacileyo? Ngokwenene kuyoyikisa kancinci. Namhlanje siza kuthetha ngesifo segazi esingaqhelekanga, kodwa esibaluleke kakhulu, ekufuneka sonke sisiqaphele. Oku kubizwa ngokuba yiParoxysmal Nocturnal Hemoglobinuria, okanye
i-PNH ngamafutshane.
Ngamafutshane, yintoni i-PNH?
I-PNH sisifo segazi esingaqhelekanga. Kwimeko enjalo
, iiseli ezibomvu zegazi egazini lethu ziyatshatyalaliswa ngaphandle kwesizathu. Ukuchaneka,
inkqubo yethu yokuzikhusela komzimba iyatshabalalisa ezi seli zibomvu zegazi. Oku kungenxa yokuba iiseli ezibomvu zegazi zabantu abanesi sifo azinalo uhlobo oluthile lweproteni ezibakhuselayo. Ke ngoko, inkqubo yethu yokuzikhusela yomzimba ayinakuzibona 'njengeeseli zethu'. Xa iiseli ezibomvu zegazi zitshatyalaliswa ngokukhawuleza ngale ndlela, umongo wethu wethambo awunakwenza iiseli ezibomvu zegazi ezintsha ngesantya esifanayo. Emva koko igazi emzimbeni liyancipha. Le meko siyibiza ngokuba
yi-anemia . Kwakhona, enye into eyingozi yesi sifo
kukuba yandisa umngcipheko wokuqhekeka kwegazi ngaphakathi kwemithambo yegazi. Isifo se-PNH asichaphazeli wonke umntu ngendlela efanayo. Abanye abantu baneempawu ezincinci kakhulu. Banokuphila ubomi obuqhelekileyo. Kodwa kwabanye, esi sifo sinokuba nzima ngakumbi. Banokufuna ukuqhubeka bethatha amayeza kunye notofelo-gazi.
Yintoni ebangela isifo se-PNH?
Eyona nto ibalulekileyo apha kukuba i-PNH
ayisosifo sokuzalwa. Oko kuthetha ukuba ayidluliselwa kumama ukuya kumntwana. Kwaye ayisosifo esosulelayo. Enyanisweni sisifo esingaqhelekanga. Khawuthelekelele, kwilizwe elifana neMelika, xa kuchongwa malunga ne-1.8 yezigidi zezigulana ezintsha zomhlaza minyaka le, zifunyanwa kuphela zizigulana ze-PNH ezingama-400 ukuya kuma-500. Esi sifo sibangelwa kukuguquka kwejini ebizwa ngokuba yi-'PIGA' kwiiseli ze-stem kwi-bone marrow yethu. Ngamafutshane, kukho impazamo encinci 'kwimiyalelo' efunyanwa yi-bone marrow, eyifektri eyenza iiseli ezibomvu zegazi. Ngenxa yale mpazamo, iiseli ezibomvu zegazi ezenziwayo azifumani loo mpahla yeproteni ekhuselayo.
Oogqirha abakakwazi ukuqonda ukuba kutheni le jini ye-'PIGA' itshintsha ngequbuliso, okanye kutheni abanye abantu kuphela bevelisa ezi seli zibomvu zegazi ezingaqhelekanga.
Ngaba nawe unazo ezi mpawu?
Xa iiseli ezibomvu zegazi ziqhekeka, umbala obomvu obizwa ngokuba yi-hemoglobin ngaphakathi kuzo ukhutshwa egazini. Ezi zinto zingcolileyo zisuswa emzimbeni ngezintso ezikwimchamo. Ngenxa yoko, umbala womchamo unokubonakala
ubomvu, umdaka, okanye umnyama kunesiqhelo . Nangona kunjalo, ayingabo bonke abantu abane-PNH abanombala owahlukileyo womchamo. Isiqingatha kuphela sezigulane esifumana olu phawu. Kwaye lumnyama ngakumbi kumchamo odluliselwa ebusuku okanye xa bevuka kusasa. Ukongeza kolu phawu luphambili, kunokubakho ezinye iimpawu ezininzi. Masibone ukuba zeziphi.
| Uphawu | Ingcaciso elula |
|---|
| Ukudinwa rhoqo | Ngenxa yokuba zimbalwa iiseli ezibomvu zegazi ezithwala ioksijini emzimbeni, uziva udiniwe kakhulu nangona sele wenze umsebenzi omncinci. Umzimba wakho uziva ungenabomi. |
| Ukuqhawukelwa ngumphefumlo | Uziva unesizungu xa uhamba kancinci okanye unyuka izinyuko. Isizathu soku kukuba umzimba wakho awufumani ioksijini oyidingayo. |
| Ukudumba okulula nokopha igazi | Kuvela amabala aluhlaza okwesibhakabhaka emzimbeni. Kwanenxeba elincinci lithatha ixesha ukuyeka ukopha. |
| Intloko ebuhlungu rhoqo | Intloko ebuhlungu inokubangelwa, ingakumbi ngenxa yotshintsho egazini. |
| Ihlwili legazi (iThrombosis) | Le yeyona ngxaki iyingozi kakhulu. Amahlwili egazi anokubakho kwimithambo yemilenze, isisu, okanye ingqondo. |
| Amachaphaza abomvu phantsi kolusu | Amachaphaza amancinci abomvu avela kumphezulu wesikhumba anokubangelwa kukuphuma kwegazi okuncinci phantsi kwesikhumba. |
| Ukungasebenzi kakuhle ngokwesondo kumadoda | Kwiimeko ezinzima zesifo, amadoda anokuba nobunzima bokukhupha isidoda ngenxa yemiphumo ebangelwa kukujikeleza kwegazi. |
Ngubani onokuthi afumane esi sifo?
Ngokutsho koogqirha, umngcipheko wokuba ne-PNH uphezulu kancinci kwabanye abantu.
- Kwabo banomongo obuthathaka : Ukuba ngesizathu esithile umsebenzi womongo ubuthathaka.
- Kwabo banesifo esibizwa ngokuba yi -Aplastic Anemia : Esi sisifo esichaphazela umongo wethambo. Abantu abanesifo basengozini yokuhlaselwa yi-PNH kamva.
- Kubantu abazalwa eAsia: Esi sifo sixhaphake kakhulu kubantu baseAsia kunakwamanye amaqela eentlanga.
I-PNH idla ngokufunyaniswa
kubantu abadala abakwiminyaka yabo yama-30 , kodwa inokubakho nakubantu abancinci, kuquka abantwana abancinci kunye neentsana.
Indlela yokuxilonga esi sifo?
Ngenxa yokuba i-PNH sisifo esingaqhelekanga kwaye iimpawu
zayo zifana nezezinye izifo, ngamanye amaxesha kungathatha ixesha ukufumanisa esi sifo. Ingakumbi kumntu onombala womchamo ongatshintshiyo, ukuba ugqirha akakukrokreli oku, ukuxilongwa kunokulibaziseka. Ukuze afumanise esi sifo, ugqirha wakho uza kuqala akubuze ngeempawu zakho kunye nembali yakho yezonyango. Emva koko, uza kuyalela uvavanyo lwegazi olwahlukeneyo. Kwezi,
uvavanyo oluchanekileyo noluchanekileyo lokuqinisekisa i-PNH luvavanyo lwegazi olukhethekileyo olubizwa ngokuba yi-'Flow Cytometry'. Olu vavanyo lunokulinganisa ngqo ukuba zingaphi iiseli ezibomvu zegazi ezingenayo loo protein ikhuselayo egazini lakho.
Ziziphi iindlela zonyango ze-PNH?
Ngethamsanqa, ngoku kukho iindlela ezahlukeneyo zonyango ezisebenzayo zokulawula i-PNH. Ugqirha wakho uza kuvavanya imeko yakho aze amisele unyango olulungele wena.
- Izinto ezithintela inkqubo yomzimba yokuzikhusela: Amayeza afana ne-eculizumab (Soliris) kunye ne-ravulizumab ( Ultomiris ) asebenza ngokuthintela inkqubo yethu yomzimba yokuzikhusela ekutshabalaliseni iiseli ezibomvu zegazi. Ezi zikwanciphisa umngcipheko wokuqhekeka kwegazi.
- IiSteroids: Ezi ngamanye amaxesha zisetyenziselwa ukulawula ukutshatyalaliswa kweeseli ezibomvu zegazi.
- Izinto zokunciphisa igazi:Ezi zinikwa ukunciphisa umngcipheko wokuqhekeka kwegazi.
- Utofelo-gazi: Xa inani legazi lomzimba liphantsi kakhulu, igazi linikwa ngaphandle ukuze kwandiswe inani leeseli ezibomvu zegazi.
Ukongeza, kukho unyango olunceda umzimba ukuba uvelise iiseli ezibomvu zegazi ezingaphezulu ngaphakathi.
- Iivithamini ezifana ne-folic acid
- Into yokukhula komntu
- I-Androgen, ihomoni yamadoda
Ukufakelwa umhlaza wethambo kunokunyanga ngokupheleleyo i-PNH. Nangona kunjalo, kuba lutyando oluyingozi kakhulu nolunzima, oogqirha bacebisa ukuba lusetyenziswe kuphela xa zonke ezinye iindlela zonyango zingaphumelelanga.
Umyalezo Wokuya Ekhaya
- I-PNH ayisosifo sokuzalwa okanye esosulelayo. Sisifo esingaqhelekanga segazi esibangelwa kukutshintsha kwejini kumongo wethambo.
- Iimpawu eziphambili ngumchamo omnyama ngenxa yokuqhekeka kweeseli ezibomvu zegazi, ukudinwa kakhulu, ubunzima bokuphefumla , kunye nomngcipheko wokuqhekeka kwegazi.
- Ukuba uqhubeka unale mpawu, ungoyiki kwaye ubone ugqirha ofanelekileyo ngokukhawuleza. Ingakumbi xelela ugqirha wakho ngale nto.
- Esi sifo sinokuchongwa ngokuchanekileyo ngovavanyo lwegazi olukhethekileyo olubizwa ngokuba
Flow Cytometry . - Ngoku kukho unyango olusebenzayo kakhulu lokulawula esi sifo nokuthintela iingxaki, ngoko ke kubaluleke kakhulu ukulandela iingcebiso zonyango ezifanelekileyo.
I-PNH Sinhala, i-Paroxysmal nocturnal hemoglobinuria, izifo zegazi, ukuqhekeka kweeseli ezibomvu zegazi, umchamo omnyama, izifo zomongo wethambo, i-anemia
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