Ingabe umchamo wakho ushintshile umbala? Ingabe uzizwa ukhathele? Ake sifunde ngalesi sifo segazi esingavamile (i-PNH)

Igazi eligeleza emithanjeni yethu lifana nomfula onikeza ukuphila, akunjalo? Amaseli abomvu egazi kuleli gazi athwala umoya-mpilo odingwa yiwo wonke amaseli emzimbeni wethu. Ngakho cabanga ukuthi bekuzokwenzekani uma la maseli abomvu abaluleke kakhulu ephuka ngokuzumayo futhi ebhujiswa ngaphandle kwesizathu esicacile? Empeleni kuyethusa kancane. Namuhla sizokhuluma ngesifo segazi esingavamile kakhulu, kodwa esibaluleke kakhulu, okudingeka sonke sisiqaphele. Lokhu kubizwa ngokuthi i-Paroxysmal Nocturnal Hemoglobinuria, noma i-PNH ngamafuphi.

Kalula nje, iyini i-PNH?

I-PNH isifo segazi esingavamile kakhulu. Kulesi simo , amangqamuzana abomvu egazini lethu ayabhujiswa ngaphandle kwesizathu. Uma sikhuluma iqiniso, amasosha omzimba ethu ayawabhubhisa lawa maseli abomvu egazi. Lokhu kungenxa yokuthi amangqamuzana abomvu egazi abantu abanalesi sifo awanalo uhlobo olukhethekile lweprotheni ewavikelayo. Ngakho-ke, amasosha omzimba wethu awakwazi ukuwabona 'njengamaseli ethu'. Lapho amangqamuzana abomvu ebhujiswa ngokushesha ngale ndlela, umnkantsha wethu awukwazi ukwenza amangqamuzana amasha abomvu egazi ngesivinini esifanayo. Khona-ke igazi emzimbeni liyancipha. Lesi simo sibiza ngokuthi i-anemia . Futhi, esinye isici esiyingozi salesi sifo ukuthi sandisa ingozi yokuqhekeka kwegazi ngaphakathi kwemithambo yegazi. Isifo se-PNH asithinti wonke umuntu ngendlela efanayo. Abanye abantu banezimpawu ezincane kakhulu. Bangaphila impilo evamile. Kodwa kwabanye, lesi sifo singaba sibi kakhulu. Kungadingeka baqhubeke nokuphuza imithi kanye nokumpontshelwa igazi.

Yini ebangela isifo se-PNH?

Into ebaluleke kakhulu lapha ukuthi i-PNH ayisona isifo esizalwa naso. Lokho kusho ukuthi ayidluliselwa kusuka kumama iye enganeni. Futhi akusona isifo esithelelanayo. Empeleni yisifo esingavamile kakhulu. Cabanga, ezweni elifana neMelika, lapho kutholakala khona iziguli ezintsha zomdlavuza ezingaba yizigidi ezingu-1.8 minyaka yonke, kutholakala iziguli ze-PNH ezingu-400 kuya ku-500 kuphela. Lesi sifo sibangelwa ukuguquka kwezakhi zofuzo okuthiwa i-'PIGA' kumaseli okuqala emnkantsheni wethu wamathambo. Kalula nje, kunephutha elincane 'emiyalweni' etholwa umnkantshe, okuyifektri eyenza amaseli egazi abomvu. Ngenxa yaleli phutha, amaseli egazi abomvu akhiwayo awatholi leso sivikelo samaprotheni.

Ingabe nawe unalezi zimpawu?

Uma amangqamuzana abomvu egazi ephuka, umbala obomvu obizwa ngokuthi i-hemoglobin engaphakathi kwawo ukhishwa ungene egazini. Le mikhiqizo engcolile isuswa emzimbeni ngezinso ezisemchameni. Ngakho-ke, umbala womchamo ungase ubonakale ubomvu, unsundu, noma umnyama kunokuvamile . Kodwa-ke, akuwona wonke umuntu one-PNH onombala ohlukile womchamo. Ingxenye yeziguli kuphela ehlangabezana nalesi sibonakaliso. Futhi kumnyama kakhulu emchameni odluliselwa ebusuku noma lapho bevuka ekuseni. Ngaphezu kwalesi sibonakaliso esiyinhloko, kungase kube nezinye izimpawu eziningana. Ake sibone ukuthi ziyini.

Ubani onamathuba amaningi okuthola lesi sifo?

Ngokusho kodokotela, ingozi yokuthola i-PNH iphakeme kancane kwabanye abantu.

• Kulabo abanomongo obuthakathaka: Uma ngesizathu esithile umsebenzi womongo ubuthakathaka.
• Kulabo abanesifo esibizwa ngokuthi i-Aplastic Anemia : Lesi yisifo esithinta umnkantsha wamathambo. Abantu abanalesi sifo basengozini yokuthola i-PNH kamuva.
• Kubantu bozalo lwase-Asia: Lesi sifo sivame kakhulu kubantu base-Asia kunakwamanye amaqembu ezinhlanga.

I-PNH ivame ukutholakala kubantu abadala abaseminyakeni yabo-30 , kodwa ingenzeka nakubantu abasebasha, okuhlanganisa nezingane ezincane nezinsana.

Ungasithola kanjani lesi sifo?

Ngenxa yokuthi i-PNH iyisifo esingavamile futhi izimpawu zayo zifana nezezinye izifo, ngezinye izikhathi kungathatha isikhathi ukuthola lesi sifo. Ikakhulukazi kumuntu umbala womchamo wakhe ongashintshi, uma udokotela engasoli lokhu, ukuxilongwa kungabambezeleka. Ukuze athole lesi sifo, udokotela wakho uzoqala ngokukubuza ngezimpawu zakho kanye nomlando wakho wezokwelapha. Ngemuva kwalokho, uzo-oda ukuhlolwa kwegazi okuhlukahlukene. Kulokhu, ukuhlolwa okungcono kakhulu nokunembile kakhulu ukuqinisekisa i-PNH ukuhlolwa kwegazi okukhethekile okubizwa ngokuthi i-'Flow Cytometry'. Lokhu kuhlolwa kungalinganisa ukuthi mangaki amangqamuzana abomvu egazi aswele leyo phrotheni evikelayo egazini lakho.

Yiziphi izindlela zokwelapha i-PNH?

Ngenhlanhla, manje kunezindlela eziningana zokwelapha ezisebenzayo zokuphatha i-PNH. Udokotela wakho uzohlola isimo sakho futhi anqume ukwelashwa okukulungele.

• Izithiyo zokucindezela amasosha omzimba: Imithi efana ne-eculizumab (Soliris) kanye ne-ravulizumab ( Ultomiris ) isebenza ngokuvimbela amasosha omzimba ethu ekubhubhiseni amangqamuzana abomvu egazi. Lokhu kunciphisa nengozi yokuqhekeka kwegazi.
• Ama-Steroids: Lawa ngezinye izikhathi asetshenziselwa ukulawula ukubhujiswa kwamangqamuzana abomvu egazi.
• Izinto zokunciphisa igazi:Lokhu kunikezwa ukunciphisa ingozi yokuqhekeka kwegazi.
• Ukumpontshelwa igazi: Uma inani legazi lomzimba liphansi kakhulu, igazi linikezwa ngaphandle ukuze kwandiswe inani lamaseli abomvu egazi.

Ngaphezu kwalokho, kunezindlela zokwelapha ezisiza umzimba ukuthi ukhiqize amangqamuzana egazi abomvu engeziwe ngaphakathi.

• Amavithamini afana ne-folic acid
• Isici sokukhula komuntu
• I-Androgen, i-hormone yesilisa

Ukufakelwa umnkantsha kungayelapha ngokuphelele i-PNH. Kodwa-ke, ngenxa yokuthi kuwukuhlinzwa okuyingozi kakhulu futhi okuyinkimbinkimbi, odokotela batusa lokhu kuphela uma zonke ezinye izindlela zokwelapha zehlulekile. I-PNH Sinhala, i-Paroxysmal nocturnal hemoglobinuria, izifo zegazi, ukuwohloka kwamangqamuzana egazi abomvu, umchamo omnyama, izifo zomnkantsha wamathambo, i-anemia