Unoziva nezveBeta Thalassemia here? Ngatitaurei nezvazvo zviri nyore!

Wakambonzwa nezvechirwere chinonzi 'thalassemia' here? Pamwe mumwe munhu mumhuri mako kana shamwari yako ane chirwere ichi. Ichi chirwere chine chekuita neropa redu. Nhasi tichataura nezveimwe yemhando huru dzethalassemia , Beta Thalassemia . Kunyangwe zita iri ringave rakaoma zvishoma, tichataura nezvaro zviri nyore, nenzira yaunogona kunzwisisa.

Chii chinonzi Beta Thalassemia?

Zvichitaurwa zviri nyore, beta thalassemia chirwere cheropa umo miviri yedu isingakwanise kugadzira hemoglobin nemazvo. Zvino ungave uchibvunza kuti, "Chii chinonzi hemoglobin?" Hemoglobin ipuroteni inowanikwa mumasero edu matsvuku eropa ine iron yakawanda. Kutaura zvazviri, ndiyo inonyanya kukosha mumasero matsvuku eropa.

Funga nezvazvo nenzira iyi: hemoglobin yakafanana nemota inobatsira masero edu matsvuku eropa kutakura okisijeni. Masero matsvuku aya anotakura okisijeni kune mamwe masero nematishu mumuviri wese. Saka, masero edu anoshandisa okisijeni iyi kugadzira simba.

Beta thalassemia ndeimwe yemhando mbiri huru dzethalassemia. Mune izvi, pane shanduko (kuchinja kwemajini), kureva, chikanganiso chidiki, mumajini ari muprotein yedu yehemoglobin. Nekuda kwekanganiso iyi yemajini, muviri wedu haugone kugadzira protein ye beta-globin muhemoglobin.

Beta thalassemia inokanganisa sei muviri wangu?

Kana kugadzirwa kwe beta-globin mumuviri wedu kwaderera, masero edu matsvuku eropa anokuvara. Zvadaro masero matsvuku aya anokuvara anobviswa nekukurumidza muropa. Zvino fungidzira, chii chinoitika kana masero matsvuku matsva eropa asingagone kugadzirwa kuti atsive akarasika? Ndipo panomuka anemia , kana kushomeka kweropa.

Zviratidzo zveanemia zvinoitika kana tisina masero matsvuku eropa akakwana ekutakura okisijeni kuenda kunhengo dzemuviri wedu. Zvadaro nhengo idzodzo hadziwani okisijeni yakakwana. Zviratidzo zveanemia zvinouya ne beta thalassemia zvinogona kubva pazvishoma kusvika pazvikuru , zvichienderana nekuti nhamba yemasero matsvuku eropa rako yakaderera sei.

Ndiani ari panjodzi huru yekuva ne beta thalassemia?

Beta thalassemia chirwere chemajini chinogarwa nhaka . Izvi zvinoreva kuti vana vanogara nhaka yehurema hwemajini hunokonzerwa nechirwere ichi kubva kuvabereki vavo. Vanhu vazhinji vane beta thalassemia vanogara munyika dzakadai seAfrica, Mediterranean region, Middle East, India neSoutheast Asia. Zvisinei, nekuda kwekutama kwevanhu vachienda kune dzimwe nyika, varwere vane beta thalassemia vari kutaurwawo kuNorthern Europe neNorth America. Chirwere ichi chiri kuonekwawo munyika yedu, Sri Lanka.

Beta thalassemia yakajairika zvakadii?

Waizviziva here kuti thalassemia ndiyo inonyanya kukonzera anemia inogarwa nhaka? Zviuru zvevarwere vatsva vane beta thalassemia vanoonekwa gore rega rega. Zvisinei, mashoko akanaka ndeekuti nekuvandudzika kwematanho ekudzivirira akadai sekuongororwa kwevanhu vane thalassemia gene defect, huwandu hwevanhu vane chirwere ichi hwaderera kusvika pamwero wakati .

Chii chinokonzera beta thalassemia?

Chikonzero chikuru che beta thalassemia idambudziko remajini (mutation) rinotadzisa kugadzirwa kwe beta-globin mumuviri wedu. Sezvatatotaura, hemoglobin inoumbwa nemapuroteni mana: macheni maviri e alpha-globin uye macheni maviri e beta-globin . Zvikanganiso mu alpha-globin chain zvinokonzera alpha thalassemia, uye zvikanganiso mu beta-globin chain zvinokonzera beta thalassemia. Kana chimwe chezvikanganiso izvi zve globin chisina kukwana, masero matsvuku eropa anokuvadzwa uye anoparadzwa.

Unogara nhaka yegene defect ye beta thalassemia nenzira ye autosomal recessive pattern . Izvi zvinoitika kana vabereki vese vemuviri vaine kopi imwe yegene rakakanganisika uye kopi imwe yegene rakajairika. Muchimiro chakanyanya kuipa che beta thalassemia, unogara nhaka kopi yegene rakakanganisika kubva kuvabereki vese.

Zvisinei, kashoma, beta thalassemia inogona kukonzerwa nekugara nhaka gene rimwe chete re beta-globin rakakanganisika. Izvi zvinonzi autosomal dominant pattern .

Ndeapi marudzi e beta thalassemia?

Kuoma kwechirwere chako kunoenderana nehuwandu hwemajini asina kunaka aunotora nhaka uye nzvimbo yechirwere chacho. Mamwe masero asina kunaka emajini haagone kugadzirwa zvachose (anonzi beta-zero thalassemia ). Mamwe masero asina kunaka anokonzera kugadzirwa kwe beta-globin shoma (anonzi beta-plus thalassemia ).

Kune mhando shoma huru dze beta thalassemia:

• Beta thalassemia major (Cooley's anemia): Iyi ndiyo mhando yakanyanya kuipa ye beta thalassemia. Muizvi, majini ese ari maviri e beta-globin haasipo kana kuti haana kunaka. Beta thalassemia major yava kunzi "transfusion-dependent thalassemia" nekuti vanhu vane chirwere ichi vanoda kuwedzerwa ropa kwehupenyu hwavo hwese.
• Beta thalassemia intermedia: Izvi zvinogona kukonzera anemia iri pakati nepakati. Mumamiriro ezvinhu aya, majini ese ari maviri e beta-globin haasipo kana kuti haana kunaka. Zvisinei, vanhu vane beta thalassemia intermedia havawanzodi kupa ropa kwehupenyu hwavo hwese.
• Beta thalassemia minor (inozivikanwawo se beta thalassemia trait): Ichi chirwere chinowanzo kukonzera anemia iri nyore. Gene rimwe chete re beta-globin harisipo kana kuti harina kunaka. Vamwe vanhu vane beta thalassemia minor vanogona kunge vasina zviratidzo.

Ndezvipi zviratidzo zve beta thalassemia?

Zviratidzo zvako zvinoenderana nekuti beta thalassemia yako yakanyanya sei. Semuenzaniso, munhu ane beta thalassemia minor anogona kunge asina zviratidzo kana kuti ane anemia iri nyore. Vanhu vane beta thalassemia intermedia uye kunyanya beta thalassemia major vanogona kunge vaine zviratidzo zviri pakati nepakati kana zvakanyanya.

Zviratidzo zvishoma

Beta thalassemia minor (beta thalassemia trait) inowanzobatanidzwa nezviratidzo zve anemia zvishoma zvakaita se:

• Kuneta nguva dzose.
• Dzungu kana kushaya simba .
• Kurwadziwa nemusoro nguva nenguva.
• Ganda rakachenuruka .

Zviratidzo zviri pakati nepakati uye zvakakomba

Zviratidzo zvakanyanya zvinosanganiswa ne beta thalassemia major. Zvimwe zvezviratidzo izvi, zvichienderana nekuoma kwechirwere chako, zvinogonawo kuwanikwa kune vanhu vane beta thalassemia intermedia. Kuwedzera kune zviratidzo zvishoma, unogona kusangana ne:

• Kutadza kufema zvakanaka panguva yekurovedza muviri.
• Kuwedzera kwekurova kwemoyo ( kurova kwemoyo ).
• Kutsvuka kweganda kana kuchena kwemaziso ( jaundice ).
• Weti yakasviba kana kuti yakaita setii.
• Kukura zvishoma nezvishoma kana kunonoka kukura.
• Kuzvimba kwedumbu .
• Kushaya simba kana kuremara kwemapfupa emaoko, makumbo, uye kumeso.

Vacheche vadiki vane beta thalassemia iri pakati nepakati kana yakanyanya vanogona kusazorora zvakanyanya , uye vanogonawo kubatwa nezvirwere kakawanda.

Beta thalassemia inoonekwa sei?

Beta thalassemia inowanzoonekwa muhudiki . Chimiro chakanyanya kuipa, beta thalassemia major, chinoonekwa muhudiki husati hwasvika makore maviri, zvichireva kuti muhudiki. Chiremba wako achaongorora beta thalassemia zvichienderana nezviratidzo zvako uye zvabuda muropa rako.

Ndezvipi zviedzo zvekuongorora chirwere?

Chiremba wako achaongorora chirwere che beta thalassemia nekutora ropa riri nyore uye kuriongorora. Kuongororwa kunogona kusanganisira:

• Kuverengwa Kweropa Kwakakwana (CBC): Kuongororwa kweCBC kunopa ruzivo nezvemasero ako eropa, kusanganisira masero ako matsvuku eropa. Kunogona kuratidza kana uine nhamba shoma yemasero matsvuku eropa, kana ari madiki pane akajairwa, ane chimiro chisina kujairika, kana kuti akachenuruka (akatsvuka zvishoma). Zviratidzo izvi zvinogona kuva zviratidzo zve thalassemia.
• Kuwanda kwemasero matsvuku eropa: Masero matsvuku eropa asati akura anonzi mareticulocytes. Kuwanda kwemasero matsvuku eropa kushoma kunoreva kuti muviri wako hausi kugadzira masero matsvuku eropa akakwana. Zvisinei, mu thalassemia major, kuwanda kwemasero matsvuku eropa kunowanzo kukwira. Izvi zvinodaro nekuti muviri wako uri kuedza kugadzira masero matsvuku eropa akawanda kuti utsive kuparadzwa kwemasero matsvuku eropa ane hemoglobin isina kujairika.
• Kuongororwa kwemajini emasero: Kuongororwa uku kunobvumira chiremba wako kunyatsoongorora hemoglobin yako uye kuona chikanganiso chemajini chine chekuita ne beta thalassemia.
• Hemoglobin electrophoresis: Kuongororwa uku kunoyera mhando dzakasiyana dzemapuroteni ehemoglobin muropa rako. Mu beta thalassemia, mamwe marudzi emapuroteni ehemoglobin anowedzera, nepo mamwe achidzikira.

Kana paine fungidziro yekuti mwana wako asati aberekwa angave akagara nhaka yejini rakakanganisika, chiremba wako anogona kuita bvunzo inonzi Chorionic Villus Sampling (CVS) kana Amniocentesis panguva yekuzvitakura. CVS inoongorora chikamu cheplacenta kuti ione zviratidzo zvechikanganiso chemajini. Placenta ndiyo nhengo inobatsira iwe nemwana wako kuchinjana zvinovaka muviri panguva yekuzvitakura. Amniocentesis inoongorora mvura yakakomberedza mwana wako kuti ione zviratidzo zve beta thalassemia.

Beta thalassemia inorapwa sei?

Kazhinji uchafanira kushanda nechikwata chevanochengeta varwere chinosanganisira nyanzvi dzakasiyana-siyana. Zvakakosha kushanda nenyanzvi inorapa zvirwere zvine chekuita neropa, nyanzvi yeropa .

Sarudzo dzekurapa dzingasanganisira:

• Kuwedzerwa ropa: Munhu ane beta thalassemia major angangoda kupa ropa kakawanda (zvichida mavhiki maviri ega ega). Munguva iyi, unowana ropa kubva kune anopa ropa. Masero matsvuku eropa anobva mukupa ropa uku anobatsira kutakura okisijeni kuenda kumatishu mumuviri wese.
• Kurapa nesimbi yakacheneswa: Iron chikamu chakakosha cheprotein yehemoglobin inobatsira kutakura okisijeni. Zvisinei, iron yakawandisa inogona kukuvadza. Kurapa nesimbi yakacheneswa kunogona kudzivirira simbi yakawandisa.
• Zvinowedzerwa zveFolic acid: Folic acid inogona kubatsira muviri wako kugadzira masero matsvuku eropa. Kana uine beta thalassemia minor, chiremba wako anogona kukurudzira izvi. Kana chirwere chako chakanyanya kuipa, ungangoda kunwa folic acid pamusoro pekupa ropa nguva dzose.
• Luspatercept: Kana uine thalassemia major, unogona kupihwa jekiseni rinonzi luspatercept mavhiki matatu ega ega kuti muviri wako ugadzire mamwe masero matsvuku eropa. Luspatercept inobatsira kuderedza anemia muvarwere vane beta thalassemia vanopa ropa.
• Kuisirwa masero emapfupa nemasero emuviri:Unogona kugamuchira masero emapfupa emugoho kubva kune anopa. Mamasero aya ndiwo anozova masero matsvuku eropa. Beta thalassemia inogona kurapwa nekutsiva masero emapfupa emugoho nemasero emapfupa anobva kune anopa ane hutano. Zvisinei, kuwana anopa anoenderana idambudziko. Zvakare, rudzi urwu rwekuisirwa ropa runoonekwa senzira ine njodzi huru.

Ndezvipi zvinetso kana migumisiro yekurapwa?

Dambudziko rinowanzoitika kana munhu achipa ropa nderekuti ane iron yakawanda . Paunopa ropa, muviri wako unowana masero matsvuku eropa ne iron yakawanda. Paunopa ropa kakawanda, iyi iron yakawanda inogona kuungana nekukuvadza nhengo dzinokosha dzakadai sechiropa, moyo, nepancreas. Kana uchipa ropa nguva dzose, taura nachiremba wako nezvekuti unofanira kutora iron chelation therapy kangani kuti ubvise iron yakawanda mumuviri wako.

Ungaderedza sei njodzi yekuve ne beta thalassemia?

Haugone kuderedza njodzi yako yekuwana chirwere che "gene defect" chinokonzerwa ne beta thalassemia. Zvisinei, unogona kudzivirira mwana wako kuti asachiwana. Kana iwe kana mumwe wako muri panjodzi yekuve mutakuri wechirwere ichi uye muchitarisira kuva nemwana, taura nachiremba wako nezvekuongororwa kwe beta thalassemia.

Ko beta thalassemia inogona kurapwa here?

Mushonga wega we beta thalassemia uripo parizvino kuisirwa masero emapfupa nemasero emuviri kubva kune mumwe munhu akaenzana naye. Zvisinei, kuwana munhu akaenzana naye kunowanzova kwakaoma. Kunyangwe nhengo dzemhuri dzingasaonekwa sedzisingakodzeri kupa.

Vaongorori vari kudzidza dzimwe nzira dzekurapa beta thalassemia, dzakadai sekutsiva majini asina kunaka neane hutano ( gene therapy ). Basa iri richiri muzvikamu zvaro zvekutanga.

Munhu ane beta thalassemia anotarisirwa kurarama kwenguva yakareba sei?

Beta thalassemia chirwere chinorapika . Vanhu vane mhando dze beta thalassemia dzisina kunyanya kuomarara, dzakadai sedzidiki uye dzinoshanda pakati nepakati, vanogona kutarisira kurarama hupenyu hwakajairika kana vakatevera mirairo yekurapwa nachiremba wavo. Zvisinei, beta thalassemia major inogona kupfupisa hupenyu hwavo. Chikonzero chinowanzo kukonzera rufu muchirwere ichi kushaya simba kwemoyo kunokonzerwa nekuwanda kwesimbi.

Taura nachiremba wako nezvechirwere chako zvichienderana nekuoma kwechirwere chako.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Bvunza chiremba wako kuti unofanira kuongororwa ropa kangani uye kurapwa, uye kuti ndeapi mararamiro aunofanira kuchinja kuti urarame nedambudziko rako.

Mimwe mibvunzo yaunogona kubvunza:

• Nderudzii rwe beta thalassemia rwandinarwo?
• Ndinofanira kurapwa rudzii kuti ndirarame dambudziko rangu?
• Njodzi yematambudziko anobva mukurapwa ingaderedzwa sei?
• Ndinofanira kuongororwa ropa kangani kuti ndione mamiriro angu?
• Ndichaongororwa ropa rerudzii?
• Ndezvipi zvinhu zvandinofanira kuchinja muhupenyu hwangu (semuenzaniso, kudya, kurovedza muviri) kuti ndirarame dambudziko rangu?
• Ndiri kuda kuisirwa stem cell here?
• Mikana yekuti vana vangu vachawanawo beta thalassemia ndeipi?

Kubatwa kwako ne beta thalassemia kunoenderana nekuti chirwere chako chakanyanya sei. Pasinei nerudzi rwe beta thalassemia, zvakakosha kushanda nenyanzvi dzine ruzivo mukuongorora nekurapa thalassemia.

Kuwana kurapwa kwakakodzera kunogona kudzivirira anemia uye kuderedza njodzi yezvinetso zvakaita sekuwanda kwesimbi mumuviri.

Kushanda nechikwata chehutano chinonyatsoongorora mamiriro ako ezvinhu uye chinopa hurongwa hwekutarisira hwakagadzirirwa iwe kunogona kubatsira kuvandudza fungidziro yako yechirwere.

Pakupedzisira, zvinhu zvekurangarira

Beta Thalassemia haisi chinhu chekutya, asi chirwere chinofanira kutarisirwa nemazvo . Kana iwe kana mumwe munhu waunoziva aine chirwere ichi, zvakakosha kutevedzera mazano ekurapa akakodzera, kuwana bvunzo dzinodiwa uye kurapwa . Yeuka, hausi wega, uye kune vanachiremba nevashandi vehutano vanogona kukubatsira. Saka, usatya kutaura navo kana uine mibvunzo. Tinovimba ruzivo urwu ruchakubatsira kurarama hupenyu hwakanaka!

Beta thalassemia, thalassemia, anemia, hemoglobin, majini akachinja, kupa ropa, iron yakawandisa