Uthalessemia yini? Izimpawu, Izimbangela, Nokwelashwa

Uthalessemia yisifo segazi elivela ekuzuzeni, elithinta indlela umzimba wakho okwenza ngayo i-hemoglobin, iprotheni eziceleni ezipula umoya ezihambisa i-oksijini kulo lonke umzimba wakho. Uma une-thalassemia, umzimba wakho ukhulula i-hemoglobin enempilo nezinye izicelo ezipula umoya kunokujwayelekile, okungabangela ukungabikho kwamagazi nokukhathala.

Lesi simo esidluliselwa ezizukulwaneni sidluliselwa kubazali kubantwana nge-jini. Nakuba kungase kubonakale kukhulu ekuqaleni, abantu abaningi abane-thalassemia baphila impilo egcwele, esebenzayo ngokunakekelwa okufanele kwezokwelapha nokwesekwa. Ukuqonda isimo sakho yisinyathelo sokuqala sokululama.

Uthalessemia yini?

Uthalessemia uqala lapho umzimba wakho une-jini ephosakele elawula ukukhiqizwa kwe-hemoglobin. Cabanga nge-hemoglobin njengezimoto ezincane zokuthumela egazini lakho ezihambisa i-oksijini emaphaphwini kuwo wonke umzimba wakho. Uma lezi zimoto zilimele noma zingekho, izitho zakho azitholi i-oksijini eyanele ukusebenza kahle.

Isimo sivela ngezindlela ezahlukene, kusukela kokulula kakhulu kuya kokubi kakhulu. Abanye abantu bane-thalassemia elula kangangokuba abazi lutho ngayo, kanti abanye badinga ukwelashwa njalo kwezokwelapha. Ubunzima buxhomeke ekutheni yiziphi i-jini ezihlaselekile nokuthi zingaki ezithwala i-thalassemia.

Umkimbi wakho uzama ukubuyisela ukungabikho kwezicelo ezipula umoya ezempilo ngokusebenza nzima. Lo msebenzi owengeziwe ungathinta i-spleen, isibindi, nenhliziyo ngokuhamba kwesikhathi, kungakho ukunakekelwa okufanele kwezokwelapha kubalulekile kangaka.

Ziyini izinhlobo zeThalassemia?

Kunezinhlobo ezimbili eziyinhloko ze-thalassemia, eziqanjwe ngegama lengxenye ye-hemoglobin ethintekile. I-alpha thalassemia kwenzeka lapho i-jini ezenza ama-alpha globin chains elahlekile noma ashintshiwe. I-beta thalassemia kwenzeka lapho i-jini ezenza ama-beta globin chains ingasebenzi kahle.

I-alpha thalassemia inezinhlobo ezine kuye ngokuthi zingaki i-jini ezithintekile. Uma i-jini eyodwa ilahlekile, ungase ungabi nazimpawu nhlobo. Uma i-jini ezimbili zithintekile, ungase ube nokungabikho kwamagazi okulula. I-jini ezintathu eziphulukile zibangela ukungabikho kwamagazi okukhulu, kanti i-jini ezine eziphulukile yiyona ndlela ebuhlungu kakhulu.

I-beta thalassemia nayo ivela ngezindlela ezahlukene. I-beta thalassemia minor kusho ukuthi uthwala i-jini eyodwa ephosakele futhi ngokuvamile unezimpawu ezilula noma azinayo. I-beta thalassemia major, ebizwa nangokuthi i-Cooley's anemia, yiyona ndlela ebuhlungu kakhulu evame ukudinga ukuthuthukiswa kwegazi njalo.

Ziyini izimpawu zeThalassemia?

Izimpawu ze-thalassemia zingase zihluke kakhulu kuye ngohlobo olukhona nokuthi lubi kangakanani. Abantu abaningi abanezimpawu ezilula banesimpawu ezimbalwa noma abanaso, kanti abanye bangase babhekane nezibonakaliso ezinzima kakhulu ezithinta impilo yabo yansuku zonke.

Nazi izimpawu ezivamile ongazihlangabezana nazo:

• Ukukhathala nobuthakathaka obungaphuli ngokuphumula
• Isikhumba esimhlophe, ikakhulukazi esobala ebusweni, ezindebeni, noma ezimpahleni zezinzipho
• Ukuphefumula okufushane ngesikhathi semisebenzi evamile
• Izandla nezinyawo ezibandayo
• Ukukhungatheka noma ukukhanya ekhanda
• Ubuhlungu ekhanda obuvela njalo
• Ubunzima bokuqonda noma ukugxila

Ezimweni ezimbi kakhulu, ungase uqaphele izimpawu ezengeziwe. I-spleen yakho ingase ikhule, ibangele ukugcwaliswa noma ukungakhululeki ngasezandleni zakho eziphezulu ngakwesobunxele. Abanye abantu bathuthukisa i-jaundice, okwenza ama-whites amehlo abo nesikhumba sabo sibonakale simhlophe.

Abantwana abane-thalassemia ebuhlungu bangase babe nokulibaziseka kokukhula nokuthuthukiswa. Bangase futhi bathuthukise izinkinga emathanjeni, kufaka phakathi ukuguquka kwama-bone obuso okuyenza ibuso libe nobukhulu obuhlukile. Lezi zimpawu zithuthukiswa ngoba umzimba usebenza nzima kangaka ukwenza izicelo ezipula umoya eziningi.

Yini ebangela uThalassemia?

Uthalessemia ubangelwa ukushintsha noma ukuguqulwa kwe-jini okutshela umzimba wakho ukuthi wenze kanjani i-hemoglobin. Uzuza lezi zinguquko ze-genetic kubazali bakho, okusho ukuthi isimo sihamba emindenini. Akuyona into ongayibamba kwabanye noma uyithuthukise kamuva ekuphileni kwakho ngenxa yokukhetha kwakho impilo.

Isimo sivame kakhulu kubantu abazalwane babo bevela ezingxenyeni ezithile zezwe. Lokhu kuhlanganisa i-Mediterranean, i-Afrika, i-Middle East, i-South Asia, ne-Southeast Asia. Isizathu sokuthi i-thalassemia ivame kakhulu kulezi zindawo sihambisana nokuvikelwa kwe-malaria okuhlinzekwe yi-thalassemia trait kubazali.

Uma bobabili abazali bethwala i-thalassemia genes, abantwana babo banethuba elikhulu lokuzuza lesi simo. Uma umzali oyedwa enesici, abantwana bangaba abathwali bona.

Nini okufanele ubona udokotela ngeThalassemia?

Kufanele ubona udokotela uma uhlangabezana nokukhathala okuqhubekayo okungaphuli ngokuphumula noma ukulala. Lokhu kubaluleke kakhulu uma ukukhathala kuphazamisa imisebenzi yakho yansuku zonke noma kubonakala kubi kakhulu kunokukhathala okuvamile okuvela emisebenzini emikhulu noma ekuhluphekeni.

Hlela i-aphoyintimenti uma uqaphela isikhumba esimhlophe, ikakhulukazi ebusweni, ezindebeni, noma ngaphansi kwezinzipho zakho. Ezinye izibonakaliso zokuqapha zihlanganisa ubuhlungu ekhanda obuvamile, ukukhungatheka, noma ukuphefumula okufushane ngesikhathi semisebenzi eyake ikuzwakalisa kulula.

Uma uhlela ukuba nezingane futhi wazi ukuthi i-thalassemia ihamba emndenini wakho, kuwukuhlakanipha ukukhuluma nomxhumanisi we-genetic ngaphambi kokukhulelwa. Bangakusiza ukuqonda amathuba okudlulisela lesi simo kubantwana bakho nokuxoxa ngezinketho zakho.

Kubantwana, qapha izimpawu zokulibala kokukhula, ukutheleleka okuvamile, noma ukushintsha ekuthandeni ukudla. Abantwana abane-thalassemia bangase futhi babonakale bekhungathekile kakhulu noma babe nobunzima bokuhambisana nezingane zabo ngesikhathi semisebenzi yomzimba.

Ziyini izici ezingozi zeThalassemia?

I-risk factor yakho enkulu ye-thalassemia yimbali yomndeni wakho ne-ethnic background. Isimo siwumoya, ngakho ukuba nabazali noma izihlobo ezine-thalassemia kukhuphula kakhulu amathuba akho okuba nayo.

Nazi izici eziyinhloko ezingozi okufanele uzazi:

• Ukuba nabazali abathwala i-thalassemia genes
• Ubuzwe base-Mediterranean, i-Afrika, noma i-Middle East
• Ubuzwe base-South Asia noma i-Southeast Asia
• Umbali womndeni wokungabikho kwamagazi noma izifo zegazi
• Ukushada ngaphakathi komndeni (ukushada ngaphakathi komndeni)

Ubuzwe base-geographic udlala indima ebalulekile ngoba i-thalassemia yathuthukiswa ezindaweni lapho i-malaria yayivamile khona. Ukuphatha i-thalassemia gene empeleni kuhlinzeka ngokuvikelwa okuthile ngokumelene ne-malaria, kungakho i-trait yaba yivamile kulezi zizwe ngokuhamba kwesikhathi.

Kubalulekile ukukhumbula ukuthi ukuba sengozini enkulu akusho ukuthi une-thalassemia. Abantu abaningi abaphuma kulezi zizwe abanalo isimo, kanti abanye abantu abangenazo izici ezingozi ezicacile bangase babe ngabathwali.

Ziyini izinhlanganisela ezingaba khona zeThalassemia?

Nakuba abantu abaningi abane-thalassemia elula baphila impilo evamile, izinhlobo ezimbi kakhulu zingabangela izinhlanganisela uma zingalawulwa kahle. Ukuqonda lezi zinkinga ezingaba khona kukusiza ukusebenza neqembu lakho lokukhathalela impilo ukuvimbela noma ukunciphisa.

Izinhlanganisela ezivamile kakhulu zithinta izitho zakho ezisebenza nzima ukubuyisela ukungabikho kwezicelo ezipula umoya ezempilo:

• Ukugcwala kwe-iron okuvela ekuthuthukisweni kwegazi okuvamile
• I-spleen ekhulu engase idinge ukususwa ngokusebenza
• Izinkinga emathanjeni, kufaka phakathi i-osteoporosis nokuguquka kwama-bone
• Izinkinga zenhliziyo ezivela ekuqokeleleni kwe-iron noma ekuhluphekeni
• Ukonakala kwesibindi okuvela e-iron eyengeziwe
• Ukulibaziseka kokukhula kubantwana
• Ukulibaziseka kokuthomba kubantu abasha

Ukugcwala kwe-iron kubaluleke kakhulu ngoba umzimba wakho awunalo indlela yemvelo yokususa i-iron eyengeziwe. Ngemuva kwesikhathi, le iron ingaqala enhliziyweni yakho, esibindini, nakwezinye izitho, okungabangela ukonakala okukhulu.

Izindaba ezinhle ukuthi ukwelashwa kwamanje kungavimbela noma kulawule izinhlanganisela eziningi ngokuphumelelayo. Ukuqapha njalo nokulandela uhlelo lwakho lokwelashwa kunciphisa kakhulu ingozi yakho yokuthuthukisa izinkinga ezinkulu.

Uthalessemia utholakala kanjani?

Ukutholakala kwe-thalassemia ngokuvamile kuqala ngezivivinyo zegazi ezilinganisa izici ezahlukene zezicelo zakho ezipula umoya. Udokotela wakho uzobheka inani lakho eliphelele legazi, elibonisa inani, usayizi, nendlela yezicelo zakho ezipula umoya, kanye namanani akho e-hemoglobin.

Uma izivivinyo zokuqala zikhomba i-thalassemia, udokotela wakho uzoyala izivivinyo ezithile. I-hemoglobin electrophoresis yivivinyo elikhethekile eliqopha izinhlobo ezahlukene ze-hemoglobin egazini lakho. Lo mvivinyo ungakwazi ukuqopha uhlobo lwe-thalassemia olukhona nokuthi lubi kangakanani.

Ukuhlolwa kwe-genetic kungase futhi kunconywe, ikakhulukazi uma uhlela ukuba nezingane. Lokhu kuhlolwa kungakwazi ukuqopha ukuguqulwa kwe-jini ethile nokukusiza ukuqopha uma ungumthwali. Umbali womndeni ne-ethnic background zinikeza izinkomba ezengeziwe ezisiza udokotela wakho ekuchazeni imiphumela yokuhlolwa.

Ngezinye izikhathi i-thalassemia itholakala ngesikhathi somsebenzi wegazi ovamile noma lapho uphenya izimpawu ezifana nokukhathala noma ukungabikho kwamagazi. Ukuhlolwa ngaphambi kokukhulelwa kukhona emindenini engozini enkulu, kuvumela abazali ukwazi ukuthi ingane yabo engakazalwa izokuthinteka yini.

Yini ukwelashwa kweThalassemia?

Ukwelashwa kwe-thalassemia kuya ngohlobo olukhona nokuthi izimpawu zakho zimbi kangakanani. Abantu abanezimpawu ezilula bangase bangadingi ukwelashwa nhlobo, kanti labo abane-thalassemia ebuhlungu badinga ukunakekelwa okuphelele kwezokwelapha kulo lonke impilo yabo.

Nge-thalassemia ebuhlungu, ukuthuthukiswa kwegazi okuvamile kuvame ukuba ukwelashwa okuyinhloko. Lokhu kuthuthukiswa kwegazi kubuyisela izicelo zakho ezipula umoya ezilimele ngezempilo, kusiza umzimba wakho ukuthola i-oksijini eyiyidingayo. Abantu abaningi badinga ukuthuthukiswa kwegazi njalo emasontweni ambalwa ukuze bagcine amandla abo futhi bavimbele izinhlanganisela.

I-iron chelation therapy isusa i-iron eyengeziwe emzimbeni wakho, okubalulekile uma uthola ukuthuthukiswa kwegazi okuvamile. Lokhu ukwelashwa kusebenzisa imithi ehlanganisa i-iron futhi isize umzimba wakho ukuyisusa ngomchamo noma izisu. Ngaphandle kwalokhu ukwelashwa, i-iron ingaqala ibe namanani ayingozi ezithweni zakho.

Ukuhlinzwa kwe-bone marrow, okuthiwa futhi i-stem cell transplant, kungase kuphile i-thalassemia. Lokhu ukwelashwa kubuyisela i-bone marrow yakho nge-marrow enempilo evela kumnikelo ofanelekayo. Noma kunjalo, kunezingozi ezinkulu futhi ngokuvamile kugcinelwa izimo ezimbi kakhulu lapho kunomnikelo ofanelekayo.

I-gene therapy iyindlela yokwelashwa entsha ebonisa ithemba lokuphilisa i-thalassemia. Le ndlela ihilela ukuguqula i-jini yakho ukuze usize umzimba wakho ukukhiqiza i-hemoglobin enempilo. Nakuba isacwaningwa, imiphumela yokuqala ikhuthaza abantu abanezimpawu ezimbi zesimo.

Ungazinakekela kanjani ekhaya ngeThalassemia?

Ukunakekela wena ekhaya kuhilela ukwenza izinqumo zokuphila ezisekela impilo yakho yonke namandla akho. Ukudla ukudla okulinganiselayo okunothile kusiza umzimba wakho ukusebenza kahle ngangokunokwenzeka, ngisho noma unezicelo ezipula umoya ezimbalwa ezempilo.

Gxila ekudleni okunothile i-folate, njenge-leafy greens, amabhinca, nama-cereals aqinisiwe. Umkimbi wakho udinga i-folate ukwenza izicelo ezipula umoya. Noma kunjalo, gwema izithasiselo ze-iron ngaphandle uma udokotela wakho ekuncoma ngokuqondile, njengoba i-iron eningi ingaba yingozi uma une-thalassemia.

Ukuzivocavoca okuthambile okuvamile kungasiza ekuthuthukiseni amandla akho nempilo yakho yonke. Qala kancane ngemisebenzi efana nokuhamba noma ukubhukuda, futhi ulalele umzimba wakho. Phumula uma uzizwa ukhathele, futhi ungazicindelisi kakhulu ezinsukwini lapho amandla akho ephansi.

Vimbela ukutheleleka ngokuhlanza izandla zakho njalo, uhlale unolwazi ngezithombe, futhi ugweme izixuku ngesikhathi sesizini ye-flu. Abantu abane-thalassemia bangase babe sengozini enkulu yokutheleleka okuthile, ikakhulukazi uma i-spleen yabo ikhulu noma isusiwe.

Qapha izimpawu zakho futhi ugcine idayari yokuthi uzizwa kanjani nsuku zonke. Lolu lwazi lusiza iqembu lakho lokukhathalela impilo ukulungisa uhlelo lwakho lokwelashwa nokubamba noma yikuphi ukushintsha ekuqaleni. Ungangabazi ukuxhumana nodokotela wakho uma uqaphela izimpawu ezintsha noma uzizwa kubi kunokujwayelekile.

Kufanele uzilungiselele kanjani i-aphoyintimenti yakho yokuya kudokotela?

Ngaphambi kwe-aphoyintimenti yakho, bhala zonke izimpawu zakho, kufaka phakathi ukuthi zaqala nini nokuthi yini ezenza zibe ngcono noma zimbi. Yiba ocacile ngamanani akho okukhathala, noma yiluphi ubuhlungu obuhlangabezana nalo, nokuthi lezi zimpawu zithinta kanjani imisebenzi yakho yansuku zonke.

Letha uhlu oluphelele lwazo zonke imithi oyithathayo, kufaka phakathi amavithamini nezithasiselo. Buye uqoqe ulwazi ngombhalo womndeni wakho wezokwelapha, ikakhulukazi noma yiziphi izihlobo ezine-anemia, i-thalassemia, noma ezinye izifo zegazi.

Lungiselela imibuzo yokubuza udokotela wakho ngesimo sakho, izinketho zokwelashwa, nokuthi yini okulindelekile. Eminye imibuzo ewusizo ingase ihlanganise ukubuza ngokunciphisa umsebenzi, nini okufanele ufuna ukunakekelwa okuphaphandabayo, noma ukuthi ungazilawula kanjani imiphumela ebiyelayo yokwelashwa.

Uma uke waba nezivivinyo zegazi zangaphambili noma amarekhodi ezokwelapha evela kwabanye odokotela, letha amakhophi nawe. Lolu lwazi lusiza iqembu lakho lokukhathalela impilo lamanje ukuqonda umlando wakho wezokwelapha nokulandelela ukushintsha esimweni sakho ngokuhamba kwesikhathi.

Yini eyinhloko ngeThalassemia?

Uthalessemia yisimo se-genetic esilawuleka esithinta indlela umzimba wakho okwenza ngayo izicelo ezipula umoya. Nakuba kudinga ukunakekelwa okuqhubekayo kwezokwelapha, abantu abaningi abane-thalassemia baphila impilo egcwele, esebenzayo ngokwelashwa okufanele nokwesekwa.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi ukutholakala ekuqaleni nokunakekelwa okuqhubekayo kwezokwelapha kwenza umehluko omkhulu ezingeni lakho lokuphila. Ukusebenza eduze neqembu lakho lokukhathalela impilo, ukulandela uhlelo lwakho lokwelashwa, nokwenza izinqumo zokuphila ezempilo kungakusiza ukuphatha izimpawu ngokuphumelelayo.

Uma une-thalassemia noma uthwala isici, ukwelulekwa kwe-genetic kungakunikeza ulwazi oluyigugu ngokuhlela umndeni. Ukuqonda isimo sakho kukwenza ukwazi ukwenza izinqumo ezilwemi ngempilo yakho nekusasa lomndeni wakho.

Imibuzo Evamile Ngokuthinta iThalassemia

Ingaphiliswa yini i-thalassemia?

Okwamanje, ukuhlinzwa kwe-bone marrow kuphela ukwelashwa okuqinisekisiwe kwe-thalassemia ebuhlungu, kodwa kunezingozi ezinkulu futhi kudinga umnikelo ofanelekayo. I-gene therapy ibonisa ithemba njengokwelashwa okungenzeka futhi icwaningwa ezivivinyweni zeklinikhi. Abantu abaningi abane-thalassemia baphatha isimo sabo ngokuphumelelayo ngokwelashwa okuqhubekayo kunokulandela ukwelashwa.

Ingabe i-thalassemia ifana nesifo se-sickle cell?

Cha, i-thalassemia nesifo se-sickle cell ziyizifo ezahlukene zegazi ezivela ekuzuzeni, nakuba zombili zithinta i-hemoglobin. I-thalassemia ihilela ukunciphisa ukukhiqizwa kwe-hemoglobin evamile, kanti isifo se-sickle cell sikhiqiza i-hemoglobin engavamile ebangela izicelo ezipula umoya ukuba zibe yi-crescent-shaped. Noma kunjalo, zombili izimo zingabangela ukungabikho kwamagazi futhi zidinga izindlela ezifanayo zokuphatha.

Ngingaba nezingane yini uma ngine-thalassemia?

Yebo, abantu abaningi abane-thalassemia bangaba nezingane, kodwa ukwelulekwa kwe-genetic kunconywa kakhulu ngaphambi kokukhulelwa. Uma bobabili abazali bethwala i-thalassemia genes, kunengozi yokudlulisela izinhlobo ezimbi kubantwana babo. Ukuhlolwa ngaphambi kokukhulelwa kungathola i-thalassemia ezinganeni ezingakazalwa, kuvumela imindeni ukwenza izinqumo ezilwemi ngokukhulelwa kwabo.

Ingabe i-thalassemia yami izoba mbi ngokuhamba kwesikhathi?

Uthalessemia ngokwayo ngokuvamile awubi kakhulu ngokuhamba kwesikhathi njengoba kuyisimo se-genetic ozalwa naso. Noma kunjalo, izinhlanganisela ezivela esimweni noma ekwelashweni lwaso zingathuthuka uma zingalawulwa kahle. Ukunakekelwa okuvamile kwezokwelapha, ukulandela izinhlelo zokwelashwa, nokuqapha izinhlanganisela kusiza ukuvimbela isimo ekubeni lithinte impilo yakho kakhulu njengoba ukhula.

Ingabe ngidinga ukugwema ukudla okuthile uma ngine-thalassemia?

Kufanele ngokuvamile ugweme izithasiselo ze-iron nokudla okuqinisiwe kwe-iron ngaphandle uma udokotela wakho ekuncoma ngokuqondile, njengoba i-iron eyengeziwe ingaba yingozi. Gxila ekudleni okulinganiselayo okunothile i-folate nezinye izondlo ezisekela impilo yonke. Iqembu lakho lokukhathalela impilo lingakunikeza isiqondiso esithile sokudla ngokuya ngezidingo zakho zomuntu siqu nehlelo lokwelashwa.