Okudingeka ukwazi nge-Thalassemia

Wake wezwa othile emndenini wakho noma umuntu omaziyo ethi "unesifo sokuntuleka kwegazi"? Ngezinye izikhathi bazizwa bekhathele ngaso sonke isikhathi, isikhumba sabo sibukeka simhlophe futhi sithambile. Ingabe lokhu kungaba yisifo sokuntuleka kwegazi esivamile? Yebo, kungase kungabi yinto evamile, kungaba yisifo segazi esizuzwe njengefa esibizwa ngokuthi i-thalassemia. Ungesabi uma uzwa leli gama. Lesi akusona isifo esithelelanayo. Ake sixoxe ngakho konke kalula nangokucacile.

Iyini ngempela i-thalassemia ?

Kalula nje, i-thalassemia isifo segazi esisithola kubazali bethu. Umuntu onalesi simo ukhiqiza amangqamuzana abomvu egazi anempilo angaphansi kokuvamile kanye neprotheni ebizwa ngokuthi i-hemoglobin .

Cabanga ngakho, amangqamuzana abomvu egazi ayinkonzo yokulethwa ethwala umoya-mpilo emizimbeni yethu yonke. I-Hemoglobin ifana nebhokisi elikhethekile elipakisha lowo moya-mpilo bese liwuthwala. Kumuntu one-thalassemia, lezi zithuthi zokulethwa (amangqamuzana abomvu egazi) namabhokisi omoya-mpilo (i-hemoglobin) ziyantula ubuningi, noma kukhona iphutha elithile kuwo. Ngakho-ke, akuzona zonke izingxenye zomzimba ezithola umoya-mpilo owanele. Siphinde sikubize lokhu ngokuthi i-anemia .

I-Thalassemia inamagama amaningi ahlukene. Kungenzeka ukuthi uke wezwa la magama:

• I-Alpha thalassemia
• I-Beta thalassemia
• I-anemia kaCooley
• I-Mediterranean anemia

I-thalassemia ikhula kanjani? Ubani osengozini?

I-Thalassemia akuyona isifo esingasithola ngokudla, ngamanzi, noma komunye umuntu. Kuyinto edluliselwa kusuka kubazali kuya ezinganeni ngokuphelele ngokusebenzisa izakhi zofuzo.

Kunezingxenye ezimbili eziyinhloko zokwenza iphrotheni ebizwa ngokuthi i-hemoglobin. Ziyi-alpha globin kanye ne-beta globin. "Iresiphi" yokwenza lezi zinto isezakhini zethu zofuzo. Uma kukhona iphutha kulezi zakhi zofuzo (iphutha kuresiphi), umzimba ngeke ukwazi ukwenza i-hemoglobin enempilo njengoba kudingeka.

• Uma uzuze ifa lofuzo olunephutha kumzali oyedwa kuphela: Ungase ube ngumthwali walo. Lokhu kusho ukuthi ungase ungabi nazimpawu noma ube nezimpawu ezimbalwa kakhulu, kodwa ungadlulisela ifa lofuzo olunephutha ezinganeni zakho.
• Uma uzuza izakhi zofuzo ezinephutha kubazali bobabili: Ungase ube ne-thalassemia emnene, ephakathi nendawo, noma enzima.

Lesi simo sivame ukubonakala kubantu emazweni ase-Asia njengeSri Lanka, eMpumalanga Ephakathi, e-Afrika, kanye namazwe aseMedithera njengeGrisi neTurkey.

Yiziphi izinhlobo eziyinhloko ze-thalassemia?

I-Thalassemia ihlukaniswe ngezinhlobo ezimbili eziyinhloko, kuye ngokuthi iyiphi ingxenye yefomula ye-hemoglobin enephutha.

Izinhlobo Ezincane ze-Alpha Thalassemia

• Isimo somthwali: Ukuba ne-gene eyodwa enephutha. Azikho izimpawu.
• I-Alpha Thalassemia Minor: Inezakhi zofuzo ezimbili ezinephutha. Izimpawu azikho noma zincane kakhulu.
• Isifo se-Hemoglobin H: Sinezakhi zofuzo ezintathu ezinephutha. Izimpawu eziphakathi kuya kwezinzima.
• I-Alpha Thalassemia Major: Ukuba nazo zonke izakhi zofuzo ezine ezinephutha. Lesi yisimo esibi kakhulu. Ingane ivame ukufela esibelethweni noma ngokushesha ngemva kokuzalwa.

Izinhlobo Ezincane ze-Beta Thalassemia

• I-Beta Thalassemia Minor: Ukuba ne-gene eyodwa enephutha. Azikho noma izimpawu ezincane kakhulu (isimo sokuthwala).
• I-Beta Thalassemia Intermedia: Inezakhi zofuzo ezimbili ezinephutha. Izimpawu ezimaphakathi.
• I-Beta Thalassemia Major / I-Cooley's Anemia: Ukuba khona kwezakhi zofuzo ezimbili ezinephutha. Lesi yisimo esinezimpawu ezinzima kakhulu.

Ziyini izimpawu ze-thalassemia?

Izimpawu zincike ohlotsheni lwe-thalassemia onayo kanye nobunzima bayo. Abanye abantu bangase bangabi nazo nhlobo izimpawu. Ezinhlotsheni ezinzima, izimpawu zivame ukuvela ngaphambi kokuba ingane ibe neminyaka emi-2 ubudala.

Lezi ezinye zezimpawu ezivamile:

• Ukukhathala okukhulu nokukhathala
• Ubunzima bokuphefumula , ukukhwehlela
• Isiyezi nobuthakathaka
• Isikhumba esikhanyayo noma esiphuzi
• Umchamo omnyama
• Isifiso sokudla
• Ukukhula okubambezelekile kanye nokuthomba okulibazisekile ezinganeni
• Isimo sobuso esingajwayelekile (ikakhulukazi ibunzi namathambo ezihlathi avelele)
• Isisu esiphumile (ngenxa yokukhula kwesibindi noma udende)
• Ubuhlungu bekhanda obuvamile
• Ubuthakathaka bamathambo nokuphuka kalula

Uthola kanjani ukuthi une-thalassemia?

Ngokuvamile, uma ungenazo izimpawu, lesi simo singatholakala ngengozi ngesikhathi sokuhlolwa kwegazi okuvamile okwenziwe ngesinye isizathu. Uma unezimpawu, udokotela wakho uzokuxilonga futhi akubuze ngomlando wezokwelapha womndeni wakho.

Ngemva kwalokho, kunconywa ukwenza ukuhlolwa kwegazi okukhethekile, okufana nalokhu:

• Ukubalwa Kwegazi Okuphelele (i-CBC): Lokhu kulinganisa inani lamaseli abomvu egazi kanye ne-hemoglobin egazini. Lokhu kuvame ukuba kuphansi ezigulini ezine-thalassemia.
• I-Hemoglobin Electrophoresis: Lokhu kunganquma ukuthi yiziphi izinhlobo ze-hemoglobin ezisegazini lakho nokuthi zingakanani zazo.
• Ukuhlolwa Kwezakhi Zofuzo: Lokhu kuhlolwa kusiza ukuhlonza ukuthi yiluphi uhlobo lwe-thalassemia onalo.

Ukuxilongwa ngesikhathi sokukhulelwa

Uma wena noma umlingani wakho niyithwali ye-thalassemia, ningayihlola ingane yakho ukuthi ayinaso yini lesi sifo ngaphambi kokuba ingane izalwe.

• Ukusampula Kwe-Chorionic Villus (CVS): Isampula encane ye-placenta iyathathwa futhi ihlolwe cishe emavikini ayi-11 okukhulelwa.
• I-Amniocentesis: Cishe emavikini angu-16 okukhulelwa, kuthathwa isampula yoketshezi lwe-amniotic oluzungeze umntwana bese luhlolwa.

Uma lesi simo sitholakala, uzothunyelwa kudokotela wegazi.

Yiziphi izindlela zokwelapha i-thalassemia?

Ukwelashwa kuncike ebunzimeni besimo. Umuntu onesifo esibucayi njenge-thalassemia minor angase angadingi ukwelashwa, kodwa izimo ezinzima zidinga ukwelashwa impilo yonke.

Izindlela zokwelapha eziyinhloko yilezi:

1. Ukumpontshelwa Igazi

Iziguli ezine-thalassemia enzima zidinga ukumpontshelwa igazi ukuze zinikezwe amangqamuzana abomvu egazi anempilo kanye ne-hemoglobin. Ngokuvamile kufanele zimpontshelwe igazi njalo emavikini ama-2-4 . Lokhu kubanika amandla okuqhubeka nemisebenzi yabo evamile ngaphandle kokukhathala.

2. Ukwelashwa Nge-Chelation

Ngenxa yokunikela ngegazi njalo kanye nezimo zezokwelapha, inani lensimbi emzimbeni linganda ngokungadingekile. Lokhu sikubiza ngokuthi "i-Iron Overload." Le nsimbi eyengeziwe ingaqongelela ezithweni ezibalulekile njengenhliziyo nesibindi bese izilimaza.

3. Ezinye izindlela zokwelapha

• Ukufakelwa kwe-Bone Marrow noma i-Stem Cell: Ukufakelwa kwe -bone marrow kusuka kumuntu onempilo ngezinye izikhathi kungayelapha ngokuphelele i-thalassemia. Kodwa-ke, lokhu akwenziwa njalo ngenxa yezingozi kanye nobunzima bokuthola umuntu ofanelekelayo.
• Ukuhlinzwa: Ezinye iziguli zine-spleen ekhulisiwe, edinga ukususwa ngokuhlinzwa (i-splenectomy).
• Imithi: Imithi efana ne-`Luspatercept (Reblozyl)` kanye ne-`Hydroxyurea` isetshenziselwa ukusiza ukukhiqiza amangqamuzana abomvu egazi nokulawula izimpawu.
• Izithasiselo: Udokotela wakho angase akuncome amavithamini afana ne-folic acid, esiza ekwakheni amangqamuzana abomvu egazi. Kodwa-ke, ungathathi izithasiselo zensimbi ngaphandle kweseluleko sezokwelapha nganoma yisiphi isizathu.

Ungaphila kanjani uphilile nge-thalassemia?

Ukunakekela lezi zinto kubaluleke kakhulu kumuntu ophila ne-thalassemia ukuze aphile impilo enempilo nejabulisayo.

• Landela imiyalelo kadokotela wakho: Landela ukwelashwa kukadokotela wakho njengoba nje kuyalelwe, ngesikhathi, futhi uqiniseke ukuthi uya emitholampilo.
• Ukudla okunempilo: Yidla ukudla okulinganiselayo okunezithelo nemifino eminingi. Buza udokotela wakho uma udinga ukunciphisa ukudla okune-iron eningi (inyama, inhlanzi, ubhontshisi) kanye nokudla okune-iron eningi.
• Goma: Thola yonke imijovo enconywe udokotela wakho, ikakhulukazi leyo efana nomjovo womkhuhlane, ngoba ungase ube sengozini enkulu yokutheleleka.
• Ukuzivocavoca:Zivocavoce kancane ngendlela evumelana nomzimba wakho. Khuluma nodokotela wakho ngakho.
• Ukuhlanzeka: Hlala kude nabantu abagulayo. Geza izandla zakho njalo.
• Gwema ukubhema notshwala: Lokhu kubi enhliziyweni nasemathanjeni akho.
• Impilo yengqondo: Ukuphila nesifo esingamahlalakhona kungakucindezela ngokomzwelo. Uma uzizwa ucindezelekile noma udangele, khuluma nomndeni, abangani, noma umeluleki wezempilo yengqondo .