Izinto ekufuneka uzazi ngeThalassemia: Masithethe ngayo ngokulula

Ngaba uhlala uziva udiniwe kwaye udiniwe? Mhlawumbi ulusu lwakho lumhlophe kancinci? Ezi asizozinto nje ezingaqhelekanga, mhlawumbi le anemia, oko kukuthi, ukungabikho kwegazi, inokuba ibangelwa yile nto. Namhlanje sithetha nge-'Thalassemia', isifo sokuzalwa esinxulumene negazi, esixhaphakileyo kubantu abaninzi kwilizwe lethu. Musa ukoyika xa usiva eli gama. Masiqonde yonke into ngale nto ngokulula.

Yintoni kanye kanye iThalassemia?

Ngamafutshane, i-thalassemia sisifo segazi esidluliselwa kwilifa esifunyanwa kwasekuzalweni . Asisisifo esosulelayo. Esi sifo sithintela imizimba yethu ekuveliseni i-hemoglobin eyaneleyo nesempilweni.

Ngoku usenokuba uyazibuza ukuba yintoni i-hemoglobin. I-hemoglobin yiproteni ekhethekileyo efumaneka ngaphakathi kwiiseli zethu ezibomvu zegazi. Isebenza 'njengenkonzo yokuhambisa' ethwala ioksijini kuyo yonke imizimba yethu. Le phakheji ebizwa ngokuba yi-hemoglobin ihanjiswa kuyo yonke iseli emzimbeni zizithuthi ezibizwa ngokuba ziiseli ezibomvu zegazi.

Umntu one-thalassemia unemveliso ephantsi ye-hemoglobin esempilweni emzimbeni wakhe. Ke ngoko, inani leeseli ezibomvu zegazi ezisempilweni emzimbeni nalo liyancipha. Le meko yeeseli ezibomvu zegazi ezinciphileyo yile nto siyibiza ngokuba yi-anemia , okanye 'ukungabikho kwegazi'. Xa iiseli zomzimba zingafumani ioksijini eziyidingayo, iimpawu ezahlukeneyo ziqala ukubonakala.

Oko kuthetha ukuba ungaziva izinto ezinje:

• Ukuhlala uziva udiniwe kakhulu kwaye udinwe kakhulu.
• Ubunzima bokuphefumla.
• Ndiziva ndibanda.
• Ndiziva ndinesizungu.
• Ulusu oluphaphathekileyo.

Yintoni ebangela i-thalassemia? Ngubani osengozini enkulu?

I-Thalassemia ibangelwa sisiphene kwiijini zethu. Cinga ngezi jini njenge 'khowudi yenkqubo' equlethe imiyalelo yokuba yonke into emzimbeni wethu kufuneka yenziwe njani. Iproteni ye-hemoglobin yenziwa ngokwemiyalelo evela kwezi jini. Ukuba kukho into engalunganga ngale miyalelo, okanye ukuba inxalenye yazo ayikho, umzimba awukwazi ukwenza i-hemoglobin enempilo. Sizuza ezi jini zinesiphene kubazali bethu. Yiyo loo nto ibizwa ngokuba sisifo esizuzwe njengelifa.

Imolekyuli ye-hemoglobin yenziwe ngeetsheyini ezine zeproteni: iitsheyini ezimbini ze-alpha globin kunye neetsheyini ezimbini ze-beta globin.

• Kufuneka ii-genes ezine ukuze kwenziwe ii-alpha globin chains (ezimbini ezivela kumzali ngamnye).
• Kufuneka ii-genes ezimbini ukuze kwenziwe ii-beta globin chains (enye ivela kumzali ngamnye).

Ukuba kukho isiphene kwiijini ezenza ezi ntambo ze-alpha okanye ze-beta, i-thalassemia iyenzeka. Ubunzima besifo bumiselwa linani leejini ezinesiphene.

Ziziphi iintlobo eziphambili ze-thalassemia?

I-Thalassemia inokwahlulwa ibe ngamanqanaba aliqela aphambili ngokusekwe kubukhali besifo. Oko kukuthi, inokwahluka ukusuka kwimeko yokuthwala iimpawu (uphawu), ukuya kwimeko encinci, ephakathi, kunye nenkulu. Uhlobo olunzima kakhulu, i-Thalassemia Major, ludla ngokufuna unyango oluqhubekayo.

I-Thalassemia yahlulwe yaba ziintlobo ezimbini eziphambili, kuxhomekeke ekubeni isiphene semfuza sikwimixokelelwane ye-alpha okanye imixokelelwane ye-beta.

1. I-Alpha Thalassemia

2. I-Beta Thalassemia

Masiqonde ezi ntlobo zimbini ngokweenkcukacha kwitheyibhile engezantsi.

Ziziphi iimpawu ezinokubakho ze-thalassemia?

Iimpawu ozifumanayo zixhomekeke kuhlobo lwe-thalassemia onayo kunye nobunzima bayo.

Amatyala angenazo iimpawu

Ukuba unesiphako esinye se-alpha gene okanye imeko encinci efana ne-beta thalassemia minor, usenokungabi nazimpawu konke konke . Okanye ungaziva ngathi udiniwe kancinci.

Iimpawu ezibuthathaka neziphakathi

Kwiimeko ezingembi kangako, ezifana ne-beta thalassemia intermedia, ukongeza kwiimpawu ze-anemia ezingembi kangako, oku kulandelayo kunokwenzeka:

• Ukukhula kancinci kwabantwana.
• Ukufikisa okulibazisekileyo.
• Ukungasebenzi kakuhle kwamathambo (umz. i-osteoporosis).
• Ukwandiswa kwespleen (eli lilungu elilwa nosulelo).

Iimpawu ezinzulu

Kwiimeko ezinzima, ezifana nesiphene kwiijini ezintathu ze-alpha (isifo se-Hemoglobin H) okanye i-beta thalassemia major (i-Cooley's anemia), iimpawu zivela ngaphambi kokuba umntwana abe neminyaka emi-2 ubudala. Ukongeza kwiimpawu ezingasentla, oku kulandelayo kunokubonwa:

• Ukulahlekelwa yintliziyo.
• Ulusu oluphaphathekileyo okanye olumthubi (njenge-jaundice).
• Umchamo omnyama, onombala weti .
• Ukukhula okungaqhelekanga kwamathambo obuso.

Indlela yokubona ngokuchanekileyo esi sifo?

I-thalassemia ephakathi neqatha idla ngokufunyaniswa kwasebuntwaneni. Oku kungenxa yokuba iimpawu ziqala ukubonakala kwiminyaka emibini yokuqala yobomi bomntwana. Ugqirha wakho unokuyalela iimvavanyo ezahlukeneyo zegazi ukuze kufunyanwe imeko.

• Ubalo lwegazi olupheleleyo (CBC): Oku kulinganisa inani leeseli ezibomvu zegazi, ubungakanani bazo, kunye namanqanaba e-hemoglobin. Abantu abane-thalassemia baneeseli ezibomvu zegazi ezimbalwa ezisempilweni, kwaye banokuba ncinci ngobukhulu.
• Uvavanyo lwenqanaba lesinyithi: Olu vavanyo lubalulekile ukwahlula phakathi kokungabikho kwesinyithi kunye ne-thalassemia.
• I-Hemoglobin Electrophoresis: Olu vavanyo lusetyenziselwa ngokukodwa ukufumanisa i-beta thalassemia.
• Uvavanyo lweMfuzo: Olu vavanyo lusetyenziselwa ukuqinisekisa i-alpha thalassemia kunye nokuchonga abathwali besifo.

Ziziphi iindlela zonyango lwe-thalassemia?

Iindlela zonyango zixhomekeke kubunzulu bemeko. Iimeko ezincinci azidingi nyango. Nangona kunjalo, kukho iindlela ezahlukeneyo zonyango kwiimeko ezinzima.

• Utofelo-gazi: Ngamafutshane, 'ukunikela ngegazi' . Igazi elivela kumntu ophilileyo linikwa isigulana ngomthambo ukuze kubuyiselwe iiseli ezibomvu zegazi ezisempilweni kunye namanqanaba e-hemoglobin. Izigulana ezine-beta thalassemia major zifuna utofelo-gazi rhoqo, ngesiqhelo rhoqo kwiiveki ezi-2-4.
• Unyango lwe-Iron Chelation: Utofelo-gazi oluqhelekileyo lunokubangela ukunyuka okungeyomfuneko kwamanqanaba e-iron emzimbeni. Oku kubizwa ngokuba yi-'iron overload'. Le iron egqithisileyo inokonakalisa amalungu omzimba afana nentliziyo nesibindi. Ke ngoko, amayeza asusa le iron egqithisileyo emzimbeni abizwa ngokuba yi-'iron chelation'. La mayeza anokuthathwa ngendlela yepilisi.
• Izongezo zeFolic Acid: I-Folic acid ibonelelwa ukunceda umzimba wenze iiseli zegazi ezisempilweni.
• Ukufakelwa kweBone Marrow: Olu lolona nyango lunokunyanga ngokupheleleyo i-thalassemia . Nangona kunjalo, lutyando oluyingozi kakhulu nolunzima. Oku kufuna umntu onikelayo osempilweni ohambelana ngokupheleleyo nesigulana, ngesiqhelo umntakwabo.

Funda ngeengxaki, unyango, kunye nobomi.

Ukuba ayinyangwanga kakuhle, ingxaki ephambili kukonakala kwentliziyo, isibindi, kunye namadlala avelisa iihomoni, ingakumbi ngenxa yobuninzi besinyithi. Ke ngoko, njengoko kuchaziwe.Unyango lwe-iron chelation lubaluleke kakhulu.

Ngokuphathelele ixesha lokuphila, ukuba une-thalassemia minor, ungalindela ixesha lokuphila eliqhelekileyo. Nokuba unesifo esiphakathi okanye esinzima, ukuba ulandela unyango olumiselweyo (utofelo-gazi kunye nokususwa kwesinyithi) ngokuchanekileyo, unethuba elihle lokuphila ubomi obude nobunempilo . Isifo sentliziyo sesona sizathu siphambili sokufa. Ke ngoko, kubalulekile ukwenza unyango lokususa isinyithi ngaphandle kokutsiba.

Ngaba esi sifo singathintelwa? Kwaye kufuneka unyango oluqhubekayo

I-Thalassemia sisifo semfuza, ngoko ke asinakuyithintela ukuba ingenzeki. Nangona kunjalo, uvavanyo lwemfuza lunokukunceda ufumanise ukuba wena okanye iqabane lakho ningabathwali be-thalassemia gene. Ukuba uceba ukuba nomntwana, kubalulekile ukwazi olu lwazi. Ukuze ufumane ingcebiso engaphezulu, bonana nomcebisi wemfuza okanye ugqirha wakho.

Ukuba une-thalassemia, kuya kufuneka uhlolwe igazi rhoqo (CBC) kwaye ujonge amanqanaba e-iron. Ugqirha wakho unokucebisa ukuba uhlolwe ukusebenza kwentliziyo yakho kunye nesibindi ubuncinane kanye ngonyaka.