Iyini i-Polycythemia Vera? Izimpawu, Izimbangela, Nokwelashwa

I-Polycythemia vera yisifo esingavamile segazi lapho ubukhali bomthambo wakho benza amaseli abomvu egazi amaningi kakhulu. Lokhu kwenzeka ngenxa yokuguquka kwezakhiwo ze-genetic okutshela umzimba wakho ukuthi uqhubeke ukukhiqiza amaseli abomvu egazi ngisho noma awadingi. Cabanga ngakho njengomshini ongabukwazi ukucima umugqa wakho wokukhiqiza, udala imikhiqizo eminingi kunokudingekayo.

Nakuba lesi simo sizwakala sikhathaza, abantu abaningi baphila impilo egcwele, esebenzayo ngokuphathwa okuqondile. Ikhono ukukuqonda ukuthi kwenzekani emzimbeni wakho nokusebenzisana eduze neqembu lakho lokuphatha impilo ukugcina konke kuqondile.

Iyini i-polycythemia vera?

I-Polycythemia vera uhlobo lomdlavuza wegazi oluthiwa yi-myeloproliferative neoplasm. Ubukhali bomthambo wakho, olufana nomshini wegazi womzimba wakho, luqala ukukhiqiza amaseli abomvu egazi amaningi kakhulu ngenxa yokuguquka kwe-genetic mutation. Le mutation isebenza njengokusuka okwephukile okuhlala kunamathele esimweni 'esivuliwe'.

Amaseli abomvu egazi engeziwe enza igazi lakho libe nzima kunokujwayelekile, kufana nendlela uju olugeleza kancane ngayo kunamanzi. Le nhlanzi ingenza kube nzima ukuba igazi ligeleze ngokukhululekile ngemithambo yegazi yakho. Abantu abaningi abanesimo esinjalo banamanani aphezulu amaseli amhlophe egazi nama-platelets.

Lokhu akuyona into oyenzile okungalungile noma obungayivimba. Ukuhluka kwe-genetic kwenzeka ngokungahleliwe ezimweni eziningi, futhi akuyona into oyithola kubazali bakho noma uyidlulisela kubantwana bakho.

Ziyini izimpawu ze-polycythemia vera?

Abantu abaningi abane-polycythemia vera ababonisi izimpawu ekuqaleni, yingakho lesi simo sivame ukutholakala ngesikhathi sokuhlolwa kwegazi okujwayelekile. Uma izimpawu zivele, ngokuvamile zihambisana nokuthola amaseli abomvu egazi amaningi kakhulu negazi elikhulu.

Nazi izimpawu ezivame kakhulu ongazihlangabezana nazo:

• Ukuzizwa ukhathele noma ubuthakathaka, ngisho noma uthole ukulala okwanele
• Izinhlungu zikhanda ezibukeka zivele ngaphandle kokulindelekile
• Ukudideka noma ukuzizwa ukhanya ekhanda
• Ukukhahlela kwesikhumba, ikakhulukazi ngemuva kokugeza ngamanzi afudumele
• Ukuzizwa ugcwele noma ukungakhululeki esiswini sakho esiphezulu sokhohlo
• Ukuphefumula kancane ngesikhathi semisebenzi evamile
• Izinkinga zobuso noma ukubona amabala
• Ubomvu ebusweni, ezandleni, noma ezinyaweni

Abanye abantu nabo bahlangabezana nezimpawu ezingavamile kodwa eziphawulekayo. Lezi zingafaka ukopha okungavamile noma ukweluka, imizwa yokushisa ezandleni nasezinyaweni, noma ukopha okukhulu ebusuku. Ungase futhi uqaphele ukuthi izikhwama ezincane zopha isikhathi eside kunokujwayelekile noma ukuthi uqhakaza kalula kunangaphambili.

Ukukhahlela kufanele kunakekelwe ngokukhethekile ngoba kuqondile kulesi simo. Ngokuvamile kuzwakala njengezinzipho ezincane kuyo yonke isikhumba sakho futhi kuya kubi ngamanzi afudumele. Lokhu kwenzeka ngoba amaseli egazi engeziwe akhipha amakhemikhali adukisa isikhumba sakho.

Yini ebangela i-polycythemia vera?

I-Polycythemia vera ibangelwa yi-genetic mutation ebizwa nge-JAK2 V617F cishe ku-95% yezimo. Le mutation iyenzeka kumaseli obukhali bomthambo wakho futhi ibabuza ukwenza amaseli abomvu egazi njalo, ngisho noma umzimba wakho unamaseli anele. Kufana nokuthi une-thermostat ebanjelwe futhi ayiphenduli kubushushu bangempela.

Le ntshintsho ye-genetic ayisiyona into ozalwa nayo ezimweni eziningi. Kunalokho, ithuthukiswa ngesikhathi esithile ekuphileni kwakho, ngokuvamile eminyakeni engama-50 noma engama-60. Ososayensi abayiqondi ngokugcwele ukuthi kungani le mutation iyenzeka, kodwa kubonakala sengathi iyenzeka ngokungahleliwe.

I-5% esele yabantu abane-polycythemia vera inezinguquko ezahlukene, njengokuguquka kwe-CALR noma ama-MPL genes. Lezi nazo zithinta indlela ubukhali bomthambo wakho okhiqiza amaseli egazi ngayo, kodwa azivamile kakhulu.

Kubalulekile ukwazi ukuthi lesi simo asiwumkhuhlane, futhi awenzanga lutho ukubangela. Izici zemvelo, izinqumo zokuphila, noma ukucindezeleka azikhuthazi i-polycythemia vera. I-mutation nje iyenzeka njengomunye wemisebenzi yaseli engahleliwe engase iyenzeke njengoba sihlupheka.

Nini okufanele ubona udokotela nge-polycythemia vera?

Kufanele uxhumane nodokotela wakho uma uhlangabezana nezimpawu eziqhubekayo ezingenazo izincazelo ezicacile. Lokhu kubaluleke kakhulu uma unawo ama-symptom amaningi ndawonye, njengokukhathala okuqhubekayo okuhlangene nezinhlungu zikhanda nokukhahlela kwesikhumba.

Gcina amehlo akho ezimpawu eziphazamisa impilo yakho yansuku zonke noma ebonakala ziba zimbi ngokuhamba kwesikhathi. Uma uzizwa ukhathele kakhulu amasonto amaningi, unamazinyo zikhanda avamile, noma uqaphela ukuthi isikhumba sakho sikhahlela kakhulu ngemuva kokugeza ngamanzi afudumele, lezi zidinga ingxoxo nomhlinzeki wakho wezempilo.

Funani ukunakekelwa kwezokwelapha ngokushesha uma uhlangabezana nobuhlungu esifubeni, ukuphefumula okunzima kakhulu, izinhlungu zikhanda ezinzima ngokuzumayo, noma izimpawu ze-blood clot njengobhlungu oluzumayo olwenze olukhulu. Lezi zingase zibonise izinkinga ezinkulu ezidinga ukwelashwa okusheshayo.

Ungalindi uma unawo ukuguquka kobuso, ukudideka, noma izimpawu zesikhathi sokuqhaqhazela njengobunqunu obuzumayo ohlangothini olulodwa lomzimba wakho. Nakuba lezi zingavamile, igazi elikhulu ku-polycythemia vera ngezinye izikhathi lingabangela izinkinga zokujikeleza ezidinga ukunakekelwa okusheshayo.

Ziyini izici ezingozi ze-polycythemia vera?

Ukuqonda ukuthi ubani onamathuba amaningi okukhula kwe-polycythemia vera kungasiza ekubekweni isimo sakho. Noma kunjalo, khumbula ukuthi ukuba nezici ezingozi akusho ukuthi uzothola ngokuqinisekile lesi simo, futhi ukungabi nazo akusho ukuthi awusoze uthola.

Izici ezingozi eziyinhloko zifaka:

• Iminyaka engaphezu kwama-60, nakuba ingase ibonakale kubantu abadala abancane
• Ukuba owesilisa, njengoba amadoda enamathuba amaningi okuba nayo
• Ukuba nobuzwe lwesiJuda, ikakhulukazi isizwe sesiJuda sase-Ashkenazi
• Ukuvezwa ngaphambili emazingeni aphezulu kokuqhuma

Iminyaka yiyona nto ebaluleke kakhulu engcupheni, ngabantu abaningi abaxilongwe phakathi kweminyaka engama-50 kuya kwengu-75. Lesi simo singavamile kakhulu kubantu abangaphansi kweminyaka engama-40, nakuba ngezinye izikhathi singase sibe khona kubantu abadala abancane futhi ngisho, ngokungavamile, kubantwana.

Ukungena kancane engcupheni kubantu besizwe sesiJuda kubonakala kuhambisana nezici ze-genetic, kodwa lesi simo singathinta abantu banoma yiluphi uhlobo lobuzwe. Ukuhlangana kokukhanya kuvela ezifundweni zabantu ababekwe ekuqhumeni kwamabhomu noma ezingozi zenuzi, kodwa ukuxilongwa okujwayelekile kwe-X-ray noma i-CT scans akukhuphuli ingozi yakho.

Okubaluleke kakhulu, iningi labantu abanezi zici ezingozi abazitholi i-polycythemia vera. Lesi simo sihlala singavamile, sithinta abantu ababili kuphela kwabayi-100,000.

Ziyini izinkinga ezingase zenzeke nge-polycythemia vera?

Nakuba i-polycythemia vera ilawuleka ngokwelashwa okuqondile, kubalulekile ukuqonda izinkinga ezingase zenzeke ukuze ukwazi ukusebenzisana nodokotela wakho ukuze uzivimbe. Igazi elikhulu namaseli egazi engeziwe ngezinye izikhathi angabangela izinkinga zokujikeleza nokugqwala kwegazi.

Izinkinga ezivame kakhulu zifaka:

• Ama-blood clots emithanjeni noma emithambekeni
• Ukuhlushwa yinhliziyo noma isikhwama
• Ubukhulu bespleen
• Izinkinga zokopha, naphezu kokuba namaseli egazi amaningi
• Uxinzelelo lwegazi oluphezulu
• Amatshe ezinso
• I-Gout evela emazingeni aphezulu e-uric acid

Ama-blood clots yiyona nto ebuhlungu kakhulu ngoba igazi elikhulu aligelezi ngokukhululekile ngemithambo yegazi yakho. La ma-clots angakha ezinyaweni zakho, emaphashini, noma kwamanye amalungu abalulekile. Yingakho udokotela wakho cishe azokuncoma ukwelashwa ukuze uthinye igazi lakho futhi unciphise inani lamaseli abomvu egazi.

Ngokuthakazelisayo, abanye abantu abane-polycythemia vera nabo bahlangabezana nezinkinga zokopha. Lokhu kubonakala kuphikisana, kodwa kwenzeka ngoba ama-platelets engeziwe awasebenzi kahle njalo. Ungase uqaphele ukuthi uqhakaza kalula noma ukuthi izikhwama ezincane zopha isikhathi eside kunokulindelekile.

Ezimweni ezingavamile, i-polycythemia vera ingathuthuka ibe yizifo zegazi ezinkulu njenge-myelofibrosis noma i-acute leukemia. Lokhu kwenzeka ku-20% yabantu futhi ngokuvamile kuthatha iminyaka eminingi. Ukulandelela okujwayelekile kusiza udokotela wakho ukuthi abone noma yikuphi ukuguquka ekuqaleni.

I-polycythemia vera ixilongwa kanjani?

Ukuxilonga i-polycythemia vera kuqala ngokuhlolwa kwegazi okubonisa ukuthi unawo amaseli abomvu egazi amaningi kakhulu. Udokotela wakho uzobheka i-hematocrit yakho (iphesenti lamaseli abomvu egazi egazini lakho) namanani e-hemoglobin. Ku-polycythemia vera, la manani aphezulu kunokujwayelekile.

Iviki yokuxilonga ebalulekile ibheka i-JAK2 mutation kumaseli akho egazi. Njengoba cishe i-95% yabantu abane-polycythemia vera banale ntshintsho ethile ye-genetic, ukuyithola kusiza ekuqinisekiseni ukuxilongwa. Uma ukuhlolwa kwe-JAK2 kungabi musa, udokotela wakho angase ahlole ezinye i-mutations njenge-CALR noma i-MPL.

Udokotela wakho uzofuna futhi ukukhipha ezinye izimbangela zamaseli abomvu egazi aphezulu. Lokhu kungafaka ukuhlolwa kokuhlola amazinga akho oksijini, ukusebenza kwezinso, nezinye izimo ezingenza umzimba wakho ukhiqize amaseli abomvu egazi engeziwe. Ngezinye izikhathi lokhu kuhilela ukuhlolwa kwegazi okwengeziwe noma izifundo zokubonisa izithombe.

I-bone marrow biopsy ingase ibe yindlela yokuncoma kwezinye izimo, ikakhulukazi uma udokotela wakho edinga ulwazi olwengeziwe mayelana nokuthi kwenzekani ebukhali bomthambo wakho. Nakuba lokhu kuzwakala kukhathaza, ngokuvamile kwenziwa njengendlela yokwelashwa kwangaphandle nge-anesthesia yendawo futhi kunikeza ulwazi oluwusizo ekucwaningeni ukwelashwa kwakho.

Ukuphathwa kwe-polycythemia vera yikuphi?

Ukuphathwa kwe-polycythemia vera kugxile ekuthuthukiseni inani lamaseli akho abomvu egazi nokuvimbela izinkinga. Izindaba ezinhle ukuthi ngokuphathwa okuqondile, abantu abaningi abanesimo esinjalo bangaphila impilo evamile, esebenzayo. Uhlelo lwakho lokwelashwa luzolungiswa ngesimo sakho, iminyaka yakho, nezici ezingozi.

Izindlela eziyinhloko zokwelashwa zifaka:

• I-Phlebotomy (ukususa igazi njalo, njengokunikela igazi)
• I-Low-dose aspirin ukuze unciphise ingozi ye-clot
• Imithi ukuze uthuthukise ukukhiqizwa kwamaseli egazi
• Ukuphathwa kwezimpawu njengokukhahlela

I-Phlebotomy ngokuvamile iyindlela yokuqala yokwelashwa udokotela wakho azokuncoma. Lokhu kuhilela ukususa cishe ipinti legazi njalo emavikini ambalwa noma izinyanga, kufana nokunikela igazi. Iyindlela elula, ephephile eyancipha ngokuqondile inani lamaseli abomvu egazi ohlelweni lwakho futhi ikusiza ukuthi uthinye igazi lakho.

I-Low-dose aspirin ivame ukubhalwa ngoba ikusiza ukuvimbela ama-blood clots ngokwenza ama-platelets akho anganamatheli. Udokotela wakho uzokunquma uma lokhu kulungele wena ngokusekelwe engcupheni yakho yokopha nezinye izimo zezempilo.

Uma i-phlebotomy yodwa inganele, udokotela wakho angase abhale imithi efana ne-hydroxyurea noma i-interferon. Le miyeke ikusiza ukuthuthukisa ukukhiqizwa kwamaseli egazi obukhali bomthambo wakho. Imithi emisha efana ne-ruxolitinib ingase ibe yindlela yokuncoma uma ezinye izindlela zokwelashwa zingasebenzi kahle noma uma unawo ama-symptom aphawulekayo.

Ungayilawula kanjani i-polycythemia vera ekhaya?

Ukuphathwa kwe-polycythemia vera ekhaya kuhilela ukwenza izinguquko zokuphila ezingakusiza ukuthi uzizwe kangcono futhi unciphise ingozi yezinkinga. Lezi zinguquko zisebenza ndawonye nokwelashwa kwakho kwezokwelapha ukukunikeza imiphumela emihle kakhulu.

Ukugcina umzimba wakho uqinisile kubalulekile ngoba kusiza ukugcina igazi lakho lingabi nzima kakhulu. Zama okungenani ama-glasi angu-8 amanzi nsuku zonke, bese uphuza okuningi uma usebenza noma usesimweni esishisayo. Gwema ukuphelelwa amanzi ngokusebenzisa utshwala noma i-caffeine kakhulu.

Ukuzivocavoca okujwayelekile, okulinganiselwe kungasiza ekuthuthukiseni ukujikeleza kwakho nempilo yakho yonke. Ukuhamba, ukubhukuda, noma i-yoga ebushelelezi kuyizinqumo ezinhle. Noma kunjalo, gwema imisebenzi enengozi enkulu yokulimaza ngoba ungase uqhakaze kalula. Hlola njalo nodokotela wakho ngaphambi kokuthi uqale uhlelo olusha lokuzivocavoca.

Gcina amehlo akho esikhuneni sakho, ikakhulukazi ukukhahlela okuvamile kulesi simo. Ukugeza ngamanzi afudumele kunokushisa kungasiza ekuthuthukiseni ukukhahlela. Abanye abantu bathola ukuthi ukufaka i-moisturizer ngesikhathi isikhumba sabo sisalimanzi kusiza, futhi ukugwema amasoseji amakhulu kungenza umehluko.

Gcina ulandelela izimpawu zakho nanoma yikuphi ukuguquka oqaphela. Lolu lwazi lusiza iqembu lakho lokuphatha impilo ukulungisa ukwelashwa kwakho njengoba kudingeka. Unganqikazi ukuxhumana nodokotela wakho uma uqaphela izimpawu ezintsha noma uma ezikhona ziya kubi.

Kufanele ulungiselele kanjani i-appointment yakho yodokotela?

Ukulungiselela i-appointment yakho kusiza ekuqinisekiseni ukuthi uthola okuningi ngesikhathi sakho nomhlinzeki wakho wezempilo. Ukufika uhleli ngemibuzo nolwazi kwenza ukuvakasha kube nomphumela futhi kusiza udokotela wakho ukuba anikeze ukunakekelwa okuhle.

Ngaphambi kwe-appointment yakho, bhala zonke izimpawu zakho, ngisho noma zibonakala zingahlobene. Faka lapho zaqala khona, zingaki zivele, nokuthi yini ezenza zibe ngcono noma zimbi. Lokhu kunikeza udokotela wakho isithombe esiphelele sokuthi uzizwa kanjani.

Letha uhlu oluphelele lwazo zonke imithi oyithathayo, kufaka phakathi imithi yokuthengwa ngaphandle komthetho, amavithamini, nama-supplements. Eminye imithi ingathinta inani lamaseli akho egazi noma ihlangane nokwelashwa kwe-polycythemia vera, ngakho-ke lolu lwazi lubalulekile.

Lungisa imibuzo mayelana nesimo sakho nezindlela zokwelashwa. Ungase ufune ukubuza ngalokho okufanele ukulindele, indlela ukwelashwa okuzoyithinta ngayo impilo yakho yansuku zonke, noma yiziphi izimpawu ezidinga ukunakekelwa okusheshayo. Ukubhala lokhu phansi kuqinisekisa ukuthi awulibali imibuzo ebalulekile ngesikhathi se-appointment.

Cabanga ngokuletha ilungu lomndeni noma umngani e-appointment yakho. Bangakusiza ukuba ukhumbule ulwazi oluxoxiwe futhi banikeze ukwesekwa. Ukuba nomuntu omunye lapho kungaba usizo kakhulu lapho ufunda ngokuxilongwa okusha noma uhlelo lokwelashwa.

Iyini into ebaluleke kakhulu mayelana ne-polycythemia vera?

I-Polycythemia vera yisimo esilawuleka esiningi labantu abaphila naso ngempumelelo amasonto amaningi. Nakuba idinga ukunakekelwa kwezokwelapha okuqhubekayo nokulandelela, abantu abaningi abanesimo esinjalo bangqhubeka nokusebenza, uhambo, futhi bajabulele imisebenzi yabo evamile ngokwelashwa okuqondile.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi ukutholakala ekuqaleni nokuphathwa okuqhubekayo kwenza umehluko omkhulu empilweni yakho yesikhathi eside. Ukusebenzisana eduze neqembu lakho lokuphatha impilo nokulandela uhlelo lwakho lokwelashwa kusiza ekuvinjeni izinkinga futhi kukugcina uzizwa kahle.

Ungavumeli lokhu kuxilongwa kukubeke noma kukunciphisa. Ngokusetshenziswa kwemithi namuhla nezindlela zokulandelela, abantu abane-polycythemia vera ngokuvamile banempilo enhle. Hlala unolwazi, landela uhlelo lwakho lokwelashwa, futhi ugcine ukuxhumana okuvulekile nabaphakeli bakho bezempilo.

Khumbula ukuthi ukuba ne-polycythemia vera akusho ukuthi ubuthakathaka noma ukuthi udinga ukuphila ngokwesaba. Kusho nje ukuthi unesimo esidinga ukuphathwa, njengokusabela noma uxinzelelo lwegazi oluphezulu. Ngenhlanhla efanele, ungaqhubeka uphela impilo egcwele nesebenzayo.

Imibuzo evame ukubuzwa mayelana ne-polycythemia vera

Ingabe i-polycythemia vera uhlobo lomdlavuza?

Yebo, i-polycythemia vera ngokusemthethweni ihlukaniswe njengohlobo lomdlavuza wegazi, kodwa yahlukile kakhulu kwalokho abantu abaningi abacabanga ngakho njengomdlavuza. Yisimo esikhula kancane esingavamile ukusabalala kwamanye amalungu omzimba wakho. Ngokwelashwa okuqondile, abantu abaningi abane-polycythemia vera baphila isikhathi eside sokuphila futhi bagcina impilo enhle. Igama elithi "umdlavuza" lingesaba, kodwa lesi simo silula kakhulu kunomdlavuza onamandla.

Ingabe i-polycythemia vera ingaphiliswa?

Okwamanje, ayikho impilo ye-polycythemia vera, kodwa ilapheka kakhulu futhi ilawuleka. Abantu abaningi baphila nalesi simo iminyaka eminingi ngaphandle kokuthi kuchaphazele impilo yabo yansuku zonke. Ukwelashwa kugxile ekulawuleni izimpawu, ukuvimbela izinkinga, nokugcina impilo yakho. Ucwaningo luyaqhubeka ngokwelashwa okusha, futhi abantu abaningi abane-polycythemia vera baphila isikhathi eside sokuphila ngokunakekelwa kwezokwelapha okuqondile.

Ingabe ngizodinga ukwelashwa kokususa igazi phakade?

Abantu abaningi abane-polycythemia vera badinga uhlobo oluthile lokwelashwa okuqhubekayo, kodwa ubukhulu nohlobo kungase kuhluke ngokuhamba kwesikhathi. Abanye abantu badinga i-phlebotomy njalo ekuphileni kwabo, ngenkathi abanye bengase bakwazi ukuphathwa ngemithi kuphela. Uhlelo lwakho lokwelashwa luzolungiswa ngokusekelwe ekulawuleni kahle kwamaseli akho egazi nokuthi uzizwa kanjani. Abantu abaningi bathola ukuthi ukwelashwa kuya kuncipha uma isimo sabo silawulwa kahle.

Ingabe ngingaba nabantwana uma ngine-polycythemia vera?

Abantu abaningi abane-polycythemia vera bangaba nabantwana, kodwa ukukhulelwa kudinga ukulandelela okukhethekile nokunakekelwa. Lesi simo singandisa ingozi yama-blood clots nezinye izinkinga ngesikhathi sokukhulelwa, ngakho-ke uzodinga ukusebenzisana eduze phakathi kodokotela wakho we-hematologist nodokotela wakho we-obstetrician. Eminye imithi esetshenziswa ekwelapheni i-polycythemia vera kufanele iyeke ngesikhathi sokukhulelwa, kodwa kukhona ukwelashwa okunye okutholakalayo. Ukuhlela kusengaphambili nokusebenzisana nabaphakeli bezempilo abanolwazi kubalulekile.

Ngamakhandlela mangaki ngizodinga ukuhlolwa kwegazi nokubukwa?

Ekuqaleni, cishe uzodinga ukuhlolwa kwegazi njalo emavikini ambalwa ukuze ulandelele ukuthi ukwelashwa kwakho kusebenza kahle kangakanani. Uma amaseli akho egazi eqondile, ukuhlolwa kungase kwenzeke njalo ezinyangeni ezimbalwa. Abantu abaningi babona i-hematologist yabo njalo ezinyangeni ezingu-3-6 zokuhlola okujwayelekile, nakuba lokhu kungase kuhluke ngokusekelwe esimweni sakho. Ukulandelela okujwayelekile kubalulekile ekutholeni noma yikuphi ukuguquka ekuqaleni nokulungisa ukwelashwa njengoba kudingeka. Ngezinye izikhathi, abantu abaningi bathola ukuthi ama-appointment abo aba yinto evamile futhi awavame kakhulu.