Beta thalassaemia waa dhiig-bax aan caadi ahayn oo jidhku aanu awoodin inuu soo saaro hemoglobin caadi ahaan. Haemoglobin A (haemoglobin ee waaweyn ee dadka waawayn) waxay ku jiraan laba silsiladood oo alfabe ah iyo silsilado beta globin ah. Beta thalassaemia, dhuuxa lafku ma awoodo in uu soo saaro qadar caadi ah oo ah beta globin taas oo keeneysa in yar oo aan lahayn haemoglobin A. Sidoo kale, waxaa jira xad-dhaaf alfa globin ah oo aan ku xidhnayn beta globin kaas oo keena burburka unugyada ee loo yaqaan 'hemolysis'.
Yaa Khatar Gelinaya?
Beta thalassaemia waa xaalad dhaxal ah. Waxay u baahan tahay labada waalid inay noqdaan kuwo sidayaal ah oo loo yaqaan 'beta thalassemia trait' ama yaryar. Marka labada waalid ay leeyihiin beta thalassaemia, waxay leeyihiin 1-kiiba 4 fursadood oo ah in cunug qaba cudurka beta thalassaemia. Nasiib daro, sababta oo ah tayada beta thalassaemia wax calaamad ah ma lahan, waalidku ma dareemi karaan khatartan ka hor intaanay uur lahayn.
Waa Maxay Noocyada Beta Thalassaemia?
Beta thalassaemia waxaa loo kala saari karaa labo siyaabood: isbeddelada hidde-wanaaga lagu dhaxlo ama loo baahdo.
Isbeddellada hiddaha
Beta thalassaemia eber : Ekaanshaha isbeddelka beta eber ayaa muujinaya in aan lahayn gleterin beta ah oo soo saari kara koromosoomkaas.
Beta iyo dhiig-yaraanta thalassaemia : Globin-beta waxaa loo soo saaraa qadar yar. Xaddiga qiyaasta beta globin soo saaray ayaa si aad ah u kala duwan isbadelka khaaska ah. Isku-dhafidyada qaarkood waxay soo saaraan ku dhawaad bilaa beta globin taas oo ku sameysa iyaga oo klinik ahaan la mid ah beta eber.
Baahida is-bedelka
Beta thalassaemia major: Beta thalassaemia major waxaa lagu qeexay baahida loo qabo in mudada dheer ee noolaanshaha noolasha, badanaa bishiiba (oo loo yaqaan 'hypertransfusion therapy').
Beta thalassaemia intermedia : Caadiyan caadiyan dhexdhexaad ah dhiig-yare oo ubaahan in la isugudbiyo mararka qaarkood (inta lagu jiro cudurrada, baaluqa, iwm) balse aan si joogto ah u joogin.
Beta thalassemia trait / yaryar : Tani waxay keentaa in dhiig-yaraanta asmptmooniya sida caadiga ah loo qaato tirada dhiigga ee dhameystiran.
Sidee Ayuu Beta Thalassaemia Isaga Bixiyaa?
Guud ahaan, beta thalassaemia major ayaa lagu ogaadaa shaashada cusub ee ilmaha . Dhallaankaas, imtixaanku wuxuu kaliya aqoonsan doonaa hemoglobin F (ama uurjiif), mana haoglobin A. Ilmahaas waxaa loo gudbiyaa dhakhtar ku takhasusay si loo ilaaliyo si dhow. Qaar ka mid ah bukaannada beta thalassaemia intermedia oo si xun u saameysey ayaa sidoo kale lagu ogaanayaa habkan.
Qaar ka mid ah bukaanada yar-yar ee ay saameeyeen ayaa lagu ogaan doonaa tirinta dhiigga ee buuxa (CBC). CBC-du waxay muujinaysaa dhiig-yaraanta dhiig-yar ee dhiig-yaraanta dhiigga. Tani waxay ku wareersan tahay dhiig-yaraanta birta. Cilad-sheegid waxaa lagu caddeeyey astaamo hemoglobin (oo loo yaqaanno electrophoresis). Betis thalassaemia intermedia iyo tayada tijaabadani waxay muujineysaa kor u kicinta xinjogobin A2 (qaabka 2aad ee hemoglobin) iyo mararka qaarkood F (fetal).
Waa Maxay Daweynaha Beta Thalassaemia?
Beta thalassaemia dabciga uma baahna daaweyn. Dadka qaba beta thalassaemia dabiiciga ah waxay yeelan doonaan dhiig yari muddo dheer.
Is-beddelidda: Bukaanka qaba beta thalassaemia waxay u baahan yihiin in la sii daayo. Daaweynta is-beddelka waxaa loo isticmaalaa in lagu xakameeyo wax-soo-saarka unugyada dhiigga ee lafaha lafaha.
Beta thalassaemia intermedia ayaa laga yaabaa in ay u baahdaan daaweyn isdabajis ah inta lagu jiro jirooyinka iyo qaangaarka (inta lagu jiro koritaanka).
Daaweynta Chelation Iron: Bukaanjiifka leh beta thalassaemia major wuxuu ka helaa birta xad-dhaafka ah iyada oo loo marayo gudbinta unugyada dhiigga cas. Tani macdanta xad-dhaafka ahi waxay dhaawici kartaa xubnaha, gaar ahaan wadnaha. Chelation Iron wuxuu ka caawiyaa jidhka inuu ka saaro birta. Kuwaas waxaa lagu siin karaa dawo afka ah ama faleebo hoostiisa hoostiisa. Bukaanka qaba beta thalassaemia intermedia ayaa laga yaabaa in ay kor u kiciso birta xitaa haddii maqnaanshaha dhiigga lagu shubo si kor loogu qaado nuugista birta ee xiidmaha yar.
Splenectomy : Cudurka wuxuu noqon karaa mid si weyn u weynaaday sababtoo ah burburka unugyada casriga (hemolysis) iyo sidoo kale wax soo saarka unugyada dhiigga cas ee beertiisa. Haddii baahida dhiig-shubka sare u kacdo ama unugyada kale ee unugyada dhiiggu hoos u dhacaan sababtoo ah xayawaanka cagaarshow, cudurka ayaa u baahan in laga saaro qalitaanka.
Hydroxyurea: Hydroxyurea waxaa loo isticmaalay in lagu kordhiyo wax soo saarka uurjiifka. Guul waxaa uu ahaa isbeddel.