Kodi mukudziwa za Beta Thalassemia? Tiyeni tikambirane mwachidule!

Kodi mudamvapo za matenda otchedwa 'thalassemia'? Mwina wina m'banja mwanu kapena mnzanu ali ndi vutoli. Uwu ndi matenda okhudzana ndi magazi athu. Lero tikambirana za mtundu umodzi waukulu wa thalassemia , Beta Thalassemia . Ngakhale dzinali lingamveke lovuta pang'ono, tikambirana za matendawa mosavuta, m'njira yomwe mungamvetse.

Kodi Beta Thalassemia ndi chiyani?

Mwachidule, beta thalassemia ndi matenda a magazi omwe thupi lathu silingathe kupanga hemoglobin moyenera. Tsopano mwina mukufunsa kuti, "Hemoglobin ndi chiyani?" Hemoglobin ndi puloteni yomwe imapezeka m'maselo ofiira a magazi omwe ali ndi chitsulo chambiri. Ndipotu, ndiye chinthu chachikulu chomwe chimapezeka m'maselo ofiira a magazi.

Taganizirani izi motere: hemoglobin ili ngati galimoto yomwe imathandiza maselo athu ofiira kunyamula mpweya. Maselo ofiira awa amanyamula mpweya kupita ku maselo ena ndi minofu m'thupi lonse. Chifukwa chake, maselo athu amagwiritsa ntchito mpweya uwu kupanga mphamvu.

Beta thalassemia ndi imodzi mwa mitundu iwiri ikuluikulu ya thalassemia. Mu izi, pali kusintha kwina (kusintha kwa majini), ndiko kuti, cholakwika chaching'ono, m'majini omwe ali mu puloteni yathu ya hemoglobin. Chifukwa cha cholakwika cha majini ichi, thupi lathu silingathe kupanga bwino puloteni ya beta-globin mu hemoglobin.

Kodi beta thalassemia imakhudza bwanji thupi langa?

Pamene kupanga kwa beta-globin m'thupi lathu kutsika, maselo ofiira a m'magazi athu amawonongeka. Kenako maselo ofiira a m'magazi owonongekawa amachotsedwa mwachangu m'magazi. Tsopano tangoganizani, chimachitika ndi chiyani ngati maselo ofiira atsopano sangathe kupangidwa kuti alowe m'malo mwa omwe atayika? Pamenepo ndi pamene kuchepa kwa magazi , kapena kusowa kwa magazi, kumachitika.

Zizindikiro za kuchepa kwa magazi m'thupi zimachitika tikakhala kuti tilibe maselo ofiira okwanira onyamula mpweya kupita ku minofu ya thupi lathu. Kenako minofuyo silandira mpweya wokwanira. Zizindikiro za kuchepa kwa magazi zomwe zimabwera ndi beta thalassemia zimatha kukhala zochepa mpaka zazikulu , kutengera momwe maselo ofiira m'magazi anu alili ochepa.

Ndani ali pachiwopsezo chachikulu chotenga beta thalassemia?

Beta thalassemia ndi matenda a majini omwe amabadwa nawo . Izi zikutanthauza kuti ana amalandira chilema cha majini chokhudzana ndi matendawa kuchokera kwa makolo awo. Anthu ambiri omwe ali ndi beta thalassemia amakhala m'maiko monga Africa, Mediterranean, Middle East, India ndi Southeast Asia. Komabe, chifukwa cha kusamuka kwa anthu padziko lonse lapansi, odwala beta thalassemia tsopano akunenedwanso ku Northern Europe ndi North America. Matendawa akupezekanso m'dziko lathu, Sri Lanka.

Kodi beta thalassemia ndi yofala bwanji?

Kodi mukudziwa kuti thalassemia ndiye chifukwa chachikulu cha kuchepa kwa magazi m'thupi? Odwala atsopano ambirimbiri a beta thalassemia amapezeka chaka chilichonse. Komabe, nkhani yabwino ndi yakuti chifukwa cha kusintha kwa njira zodzitetezera monga kuyezetsa magazi kuti adziwe anthu omwe ali ndi vuto la majini a thalassemia, chiwerengero cha milandu chachepa pang'ono .

Kodi n’chiyani chimayambitsa beta thalassemia?

Chifukwa chachikulu cha beta thalassemia ndi vuto la majini (mutation) lomwe limalepheretsa kupanga beta-globin m'thupi lathu. Monga tanenera kale, hemoglobin imapangidwa ndi ma protein chain anayi: ma alpha-globin chain awiri ndi ma beta-globin chain awiri. Zofooka mu alpha-globin chain zimayambitsa alpha thalassemia, ndipo zofooka mu beta-globin chain zimayambitsa beta thalassemia. Ngati globin chain iliyonse ilibe vuto, maselo ofiira amagazi amawonongeka ndikuwonongeka.

Mumalandira chilema cha jini cha beta thalassemia mu autosomal recessive pattern . Izi zimachitika pamene makolo onse awiri ali ndi kopi imodzi ya jini yolakwika ndi kopi imodzi ya jini yabwinobwino. Mu mtundu woopsa kwambiri wa beta thalassemia, mumalandira kopi ya jini yolakwika kuchokera kwa makolo onse awiri.

Komabe, nthawi zambiri, beta thalassemia imatha kuchitika chifukwa cholandira jini limodzi lokha la beta-globin lolakwika. Izi zimatchedwa autosomal dominant pattern .

Kodi mitundu ya beta thalassemia ndi iti?

Kuopsa kwa matenda anu kumadalira kuchuluka kwa majini omwe ali ndi vuto komanso komwe vutolo lachokera. Ma gene ena olakwika amachititsa kuti beta-globin isapangidwe konse (yotchedwa beta-zero thalassemia ). Ma gene ena amachititsa kuti beta-globin yochepa kwambiri ipangidwe (yotchedwa beta-plus thalassemia ).

Pali mitundu ikuluikulu ingapo ya beta thalassemia:

• Beta thalassemia major (Cooley's anemia): Iyi ndi mtundu woopsa kwambiri wa beta thalassemia. Pankhaniyi, majini onse a beta-globin akusowa kapena ali ndi vuto. Beta thalassemia major tsopano imatchedwa "thalassemia yodalira kuikidwa magazi" chifukwa anthu omwe ali ndi vutoli amafunika kuikidwa magazi moyo wawo wonse.
• Beta thalassemia intermedia: Izi zingayambitse kuchepa kwa magazi pang'ono mpaka pang'ono. Pa vutoli, majini onse a beta-globin akusowa kapena ali ndi vuto. Komabe, anthu omwe ali ndi beta thalassemia intermedia nthawi zambiri safunika kupereka magazi kwa moyo wawo wonse.
• Beta thalassemia minor (kapena beta thalassemia trait): Izi nthawi zambiri zimayambitsa zizindikiro zochepa za kuchepa kwa magazi m'thupi. Pachifukwa ichi, jini imodzi yokha ya beta-globin ikusowa kapena ili ndi vuto. Anthu ena omwe ali ndi beta thalassemia minor sangasonyeze zizindikiro zilizonse.

Kodi zizindikiro za beta thalassemia ndi ziti?

Zizindikiro zanu zimadalira momwe beta thalassemia yanu ilili yoopsa. Mwachitsanzo, munthu amene ali ndi beta thalassemia minor akhoza kukhala wopanda zizindikiro kapena kukhala ndi kuchepa kwa magazi pang'ono. Anthu omwe ali ndi beta thalassemia intermedia makamaka beta thalassemia major akhoza kukhala ndi zizindikiro zochepa kapena zoopsa kwambiri.

Zizindikiro zochepa

Beta thalassemia yaying'ono (beta thalassemia trait) nthawi zambiri imagwirizanitsidwa ndi zizindikiro zochepa za kuchepa kwa magazi m'thupi monga:

• Kutopa pafupipafupi.
• Chizungulire kapena kufooka .
• Kupweteka mutu pafupipafupi.
• Khungu lotumbululuka .

Zizindikiro zocheperako komanso zoopsa

Zizindikiro zoopsa kwambiri zimagwirizanitsidwa ndi beta thalassemia major. Zina mwa zizindikirozi, kutengera kuopsa kwa matenda anu, zitha kupezekanso mwa anthu omwe ali ndi beta thalassemia intermedia. Kuwonjezera pa zizindikiro zochepa, mutha kukumana ndi izi:

• Kuvuta kupuma panthawi yochita masewera olimbitsa thupi.
• Kuwonjezeka kwa kugunda kwa mtima ( kugunda kwa mtima ).
• Kuoneka kwachikasu pakhungu kapena maso oyera ( jaundice ).
• Mkodzo wakuda kapena wamtundu wa tiyi.
• Kukula pang'onopang'ono kapena kuchedwa kukula.
• Kutupa m'mimba .
• Kufooka kapena kupunduka kwa mafupa a manja, miyendo, ndi nkhope.

Makanda aang'ono omwe ali ndi beta thalassemia yochepa kapena yoopsa amatha kukhala osakhazikika kwambiri , ndipo amathanso kutenga matenda pafupipafupi.

Kodi beta thalassemia imapezeka bwanji?

Matenda a beta thalassemia nthawi zambiri amapezeka ali ana . Mtundu woopsa kwambiri, beta thalassemia major, umapezeka asanakwanitse zaka ziwiri, kutanthauza ali mwana. Dokotala wanu adzazindikira matenda a beta thalassemia kutengera zizindikiro zanu ndi zotsatira za mayeso a magazi.

Kodi mayeso oyezetsa matenda ndi ati?

Dokotala wanu adzazindikira beta thalassemia mwa kutenga magazi osavuta ndikuwafufuza. Mayeso angaphatikizepo:

• Kuchuluka kwa Magazi Okwanira (CBC): Kuyeza kwa CBC kumapereka chidziwitso chokhudza maselo anu a m'magazi, kuphatikizapo maselo anu ofiira a m'magazi. Kungasonyeze ngati muli ndi maselo ofiira ochepa a m'magazi, ngati ndi ang'onoang'ono kuposa masiku onse, ali ndi mawonekedwe achilendo, kapena ndi otumbululuka (ofiira pang'ono). Zizindikiro izi zitha kukhala zizindikiro za thalassemia.
• Kuchuluka kwa maselo ofiira a m'magazi: Maselo ofiira osakhwima amatchedwa ma reticulocytes. Kuchuluka kwa maselo ofiira a m'magazi ochepa kumatanthauza kuti thupi lanu silipanga maselo ofiira okwanira. Komabe, mu thalassemia major, kuchuluka kwa maselo ofiira a m'magazi nthawi zambiri kumakhala kokwera. Izi zili choncho chifukwa thupi lanu likuyesera kupanga maselo ofiira ambiri kuti libwezeretse kuwonongeka kwa maselo ofiira a m'magazi omwe ali ndi hemoglobin yolakwika.
• Kuyesa majini a maselo: Kuyesa kumeneku kumathandiza dokotala wanu kufufuza bwino hemoglobin yanu ndikupeza vuto la majini lomwe limakhudzana ndi beta thalassemia.
• Hemoglobin electrophoresis: Kuyesa kumeneku kumayesa mitundu yosiyanasiyana ya mapuloteni a hemoglobin m'magazi anu. Mu beta thalassemia, mitundu ina ya mapuloteni a hemoglobin imawonjezeka, pomwe ina imachepa.

Ngati mwana wanu wosabadwayo akukayikiridwa kuti walandira jini lolakwika, dokotala wanu angachite mayeso otchedwa Chorionic Villus Sampling (CVS) kapena Amniocentesis panthawi ya mimba. CVS imayesa gawo la placenta kuti aone zizindikiro za vuto la majini. placenta ndi chiwalo chomwe chimathandiza inu ndi mwana wanu kusinthana zakudya panthawi ya mimba. Amniocentesis imayesa madzi ozungulira mwana wanu kuti aone zizindikiro za beta thalassemia.

Kodi beta thalassemia imachiritsidwa bwanji?

Nthawi zambiri muyenera kugwira ntchito ndi gulu losamalira lomwe limaphatikizapo akatswiri osiyanasiyana. Ndikofunikira kwambiri kugwira ntchito ndi katswiri yemwe amachiritsa matenda okhudzana ndi magazi, katswiri wa magazi .

Njira zochiritsira zingaphatikizepo:

• Kuika magazi: Munthu amene ali ndi beta thalassemia major angafunike kupereka magazi pafupipafupi (mwina milungu iwiri iliyonse). Pa nthawiyi, mumalandira magazi kuchokera kwa wopereka magazi. Maselo ofiira a magazi ochokera ku kupereka magazi kumeneku amathandiza kunyamula mpweya kupita ku minofu m'thupi lonse.
• Chithandizo cha iron chelation: Iron ndi gawo lofunika kwambiri la puloteni ya hemoglobin yomwe imathandiza kunyamula mpweya. Komabe, iron yambiri ingakhale yoopsa. Chithandizo cha iron chelation chingalepheretse chitsulo chochuluka.
• Zakudya zowonjezera za folic acid: Folic acid ingathandize thupi lanu kupanga maselo ofiira a magazi. Ngati muli ndi beta thalassemia minor, dokotala wanu angakulangizeni izi. Ngati vuto lanu ndi lalikulu, mungafunike kumwa folic acid kuwonjezera pa kupereka magazi nthawi zonse.
• Luspatercept: Ngati muli ndi thalassemia major, mungapatsidwe jakisoni wotchedwa luspatercept milungu itatu iliyonse kuti thupi lanu lipange maselo ofiira ambiri. Luspatercept imathandiza kuchepetsa kuchepa kwa magazi m'thupi mwa odwala omwe ali ndi beta thalassemia omwe amapereka magazi.
• Kusamutsa mafupa ndi maselo oyambira:Mukhoza kulandira maselo oyambira a m'mafupa kuchokera kwa wopereka. Maselo oyambira awa ndi omwe pambuyo pake amakhala maselo ofiira a magazi. Beta thalassemia imatha kuchiritsidwa mwa kusintha maselo oyambira a m'mafupa anu ndi maselo oyambira ochokera kwa wopereka wathanzi. Komabe, kupeza wopereka wofanana ndi wopereka ndi kovuta. Komanso, mtundu uwu wa kusamutsa umaonedwa ngati njira yowopsa kwambiri.

Kodi mavuto kapena zotsatirapo zake ndi ziti?

Vuto lofala kwambiri la kupereka magazi ndi kuchuluka kwa chitsulo . Mukapereka magazi, thupi lanu limalandira maselo ofiira a magazi ndi chitsulo chowonjezera. Mukapereka magazi mobwerezabwereza, chitsulo chochulukirapochi chingamangire ndikuwononga ziwalo zofunika monga chiwindi, mtima, ndi kapamba. Ngati mumapereka magazi nthawi zambiri, lankhulani ndi dokotala wanu za kangati komwe muyenera kumwa mankhwala a chitsulo kuti muchotse chitsulo chochuluka m'thupi lanu.

Kodi mungachepetse bwanji chiopsezo chokhala ndi beta thalassemia?

Simungachepetse chiopsezo chanu chotengera vuto la majini lomwe limakhudzana ndi beta thalassemia. Komabe, mutha kuletsa mwana wanu kuti asalandire. Ngati inu kapena mnzanu muli pachiwopsezo chokhala ndi vuto la majini ndipo mukuyembekeza kukhala ndi mwana, lankhulani ndi dokotala wanu za kuyezetsa beta thalassemia.

Kodi beta thalassemia ingachiritsidwe?

Mankhwala okhawo omwe alipo pakali pano a beta thalassemia ndi kusamutsidwa kwa mafupa ndi maselo oyambira kuchokera kwa wopereka wofanana. Komabe, kupeza wopereka wofanana nthawi zambiri kumakhala kovuta. Ngakhale achibale sangaganizidwe kuti ndi opereka oyenera.

Ofufuza pakali pano akuphunzira njira zina zochizira matenda a beta thalassemia, monga kusintha majini omwe ali ndi vuto ndi majini athanzi ( gene therapy ). Ntchitoyi ikadali pachiyambi.

Kodi munthu amene ali ndi beta thalassemia amakhala ndi moyo wautali bwanji?

Beta thalassemia ndi matenda ochiritsika . Anthu omwe ali ndi mitundu yofatsa komanso yocheperako ya beta thalassemia, monga minor ndi intermedia, amatha kuyembekezera kukhala ndi moyo wabwinobwino ngati atsatira malangizo a dokotala wawo. Komabe, beta thalassemia major ingafupikitse moyo wawo. Chomwe chimayambitsa imfa kwambiri pa vutoli ndi mtima wosagwira ntchito chifukwa cha chitsulo chochuluka.

Lankhulani ndi dokotala wanu za matenda anu malinga ndi kuopsa kwa matenda anu.

Ndi mafunso ati omwe ndiyenera kufunsa dokotala wanga?

Funsani dokotala wanu kuti muyenera kuyezetsa magazi kangati komanso kulandira chithandizo, komanso kusintha kwa moyo wanu kuti muthane ndi vuto lanu.

Mafunso ena omwe mungafunse:

• Ndi mtundu wanji wa beta thalassemia womwe ndili nawo?
• Ndi chithandizo chamtundu wanji chomwe ndiyenera kulandira kuti ndisamalire vuto langa?
• Kodi chiopsezo cha mavuto omwe angabwere chifukwa cha chithandizo chingachepe bwanji?
• Kodi ndiyenera kuyezetsa magazi kangati kuti ndione momwe ndikumvera?
• Ndi mayeso amtundu wanji a magazi omwe ndidzalandira?
• Ndi kusintha kotani kwa moyo wanga (monga zakudya, masewera olimbitsa thupi) komwe ndiyenera kusintha kuti ndisamavutike ndi vutoli?
• Kodi ndili woyenera kuikidwa maselo oyambira?
• Kodi pali mwayi wotani woti ana anga nawonso adzadwala matenda a beta thalassemia?

Mmene mumakhalira ndi beta thalassemia zimadalira momwe matenda anu alili ovuta. Mosasamala kanthu za mtundu wa beta thalassemia, ndikofunikira kugwira ntchito ndi akatswiri odziwa bwino matenda ndi kuchiza thalassemia.

Kupeza chithandizo choyenera kungateteze kuchepa kwa magazi m'thupi ndikuchepetsa chiopsezo cha mavuto monga kuchuluka kwa chitsulo m'thupi.

Kugwira ntchito ndi gulu lachipatala lomwe limayang'anira bwino matenda anu ndikupereka dongosolo losamalira lomwe limakonzedwa bwino kungathandize kukonza thanzi lanu.

Pomaliza, zinthu zoti muzikumbukira

Matenda a Beta Thalassemia si chinthu choopa, koma ndi matenda omwe amafunika kuthandizidwa bwino . Ngati inu kapena munthu amene mumamudziwa ali ndi vutoli, ndikofunikira kwambiri kutsatira upangiri woyenera wachipatala, kupeza mayeso ndi chithandizo chofunikira . Kumbukirani, simuli nokha, ndipo pali madokotala ndi ogwira ntchito zachipatala omwe angakuthandizeni. Chifukwa chake, musaope kulankhula nawo ngati muli ndi mafunso. Tikukhulupirira kuti izi zikuthandizani kukhala ndi moyo wathanzi!

Beta thalassemia, thalassemia, kuchepa kwa magazi m'thupi, hemoglobin, kusintha kwa majini, kupereka magazi, kuchuluka kwa chitsulo m'thupi