Oko kufuneka ukwazi malunga neThalassemia

Ngaba wakha weva umntu kusapho lwakho okanye umntu omaziyo esithi "une-anemia"? Ngamanye amaxesha uziva ediniwe ngalo lonke ixesha, ulusu lwakhe lubonakala lumhlophe kwaye luthambile. Ngaba oku kungaba yi-anemia eqhelekileyo? Ewe, kusenokwenzeka ukuba ayiqhelekanga, isenokuba sisifo segazi esizuzwe njengelifa esibizwa ngokuba yi-thalassemia. Musa ukoyika xa usiva eli gama. Esi asisosifo esosulelayo. Masithethe ngayo yonke into ngokulula nangokucacileyo.

Yintoni kanye kanye i-thalassemia ?

Ngamafutshane, i-thalassemia sisifo segazi esisifumana kubazali bethu. Umntu onesi sifo unemveliso engaphantsi kuneqhelekileyo yeeseli ezibomvu zegazi ezisempilweni kunye neproteni ebizwa ngokuba yi-hemoglobin .

Cinga ngako oku, iiseli ezibomvu zegazi yinkonzo yokuhambisa ioksijini ethwala ioksijini emizimbeni yethu yonke. I-Hemoglobin ifana nebhokisi ekhethekileyo epakisha loo oksijini ize iyithwale. Kumntu one-thalassemia, ezi zithuthi zokuhambisa (iiseli ezibomvu zegazi) kunye neebhokisi zeoksijini (i-hemoglobin) ziyanqongophala ngobuninzi, okanye kukho isiphene esithile kuzo. Ngoko ke, ayizizo zonke iindawo zomzimba ezifumana ioksijini eyaneleyo. Sikwabiza oku ngokuba yi-anemia .

I-Thalassemia inamagama amaninzi ahlukeneyo. Usenokuba ukhe weva la magama:

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

Ikhula njani i-thalassemia? Ngubani osengozini?

I-Thalassemia ayisosifo esinokufunyanwa ngokutya, ngamanzi, okanye komnye umntu. Yinto edluliselwa ukusuka kubazali ukuya ebantwaneni ngokupheleleyo ngokusebenzisa iijini.

Kukho iinxalenye ezimbini eziphambili zokwenza iproteni ebizwa ngokuba yi-hemoglobin. Ziyi-alpha globin kunye ne-beta globin. "Iresiphi" yokwenza ezi zinto ikwiijini zethu. Ukuba kukho impazamo kwezi jini (impazamo kwiresiphi), umzimba awuyi kukwazi ukwenza i-hemoglobin enempilo njengoko kufuneka.

• Ukuba uzuze ilifa le-gene enesiphene kumzali omnye kuphela: Usenokuba ungumthwali we-gene. Oku kuthetha ukuba usenokungabi nazimpawu okanye ungabi nazimpawu zininzi, kodwa ungadlulisela i-gene enesiphene kubantwana bakho.
• Ukuba uzuze ilifa lesi sifo kubazali bobabini: Usenokuba ne-thalassemia ebuthathaka, ephakathi, okanye enzima.

Le meko ixhaphake kakhulu kubantu bamazwe aseAsia afana neSri Lanka, uMbindi Mpuma, iAfrika, kunye namazwe aseMeditera afana neGrisi neTurkey.

Ziziphi iintlobo eziphambili ze-thalassemia?

I-Thalassemia yahlulwe yaba ziintlobo ezimbini eziphambili, kuxhomekeke ekubeni yeyiphi inxalenye yefomyula ye-hemoglobin enesiphako.

Iintlobo ezincinci ze-Alpha Thalassemia

• Imeko yomthwali: Ukuba nejini enye enesiphene. Akukho zimpawu.
• I-Alpha Thalassemia Minor: Inezakhi zofuzo ezimbini ezineengxaki. Iimpawu azikho okanye azikho kakhulu.
• Isifo seHemoglobin H: Sinezakhi zofuzo ezi-3 ezineengxaki. Iimpawu eziphakathi ukuya kwezinzima.
• I-Alpha Thalassemia Major: Ukuba nazo zonke ii-gene ezine ezineempazamo. Le yimeko embi kakhulu. Usana ludla ngokufela esibelekweni okanye emva nje kokuzalwa.

Iintlobo ezincinci zeBeta Thalassemia

• I-Beta Thalassemia Minor: Ukuba ne-gene enye enesiphene. Akukho okanye iimpawu ezincinci kakhulu (imeko yomthwali).
• I-Beta Thalassemia Intermedia: Inezakhi zofuzo ezimbini ezineengxaki. Iimpawu eziphakathi.
• I-Beta Thalassemia Major / I-Cooley's Anemia: Ubukho be-genes ezimbini ezineengxaki. Le yimeko eneempawu ezinzima kakhulu.

Zithini iimpawu ze-thalassemia?

Iimpawu zixhomekeke kuhlobo lwe-thalassemia onayo kunye nobunzima bayo. Abanye abantu basenokungabi nazo iimpawu konke konke. Kwiintlobo ezinzima, iimpawu zihlala zibonakala ngaphambi kokuba umntwana abe neminyaka emi-2 ubudala.

Nazi ezinye zeempawu eziqhelekileyo:

• Ukudinwa kakhulu nokudinwa
• Ubunzima bokuphefumla , ukuphefumla kancinci
• Ukudinwa kunye nobuthathaka
• Ulusu olukhanyayo okanye olumthubi
• Umchamo omnyama
• Umnqweno wokutya
• Ukukhula okungalindelekanga kunye nokufikisa okulibazisekileyo ebantwaneni
• Ubuso obungaqhelekanga (ingakumbi ibunzi elibonakalayo kunye namathambo esihlathini)
• Isisu esiphumileyo (ngenxa yokwanda kwesibindi okanye ipleyini)
• Intloko ebuhlungu rhoqo
• Ukubuthathaka kwamathambo nokuqhekeka lula

Ufumanisa njani ukuba une-thalassemia?

Ngokwesiqhelo, ukuba awunazo iimpawu, le meko inokufumaneka ngengozi ngexesha lovavanyo lwegazi oluqhelekileyo olwenziwa ngesinye isizathu. Ukuba uneempawu, ugqirha wakho uza kukuxilonga aze akubuze ngembali yempilo yosapho lwakho.

Emva koko, kucetyiswa ukuba kwenziwe uvavanyo lwegazi olukhethekileyo, olufana nolu:

• Ubalo lweGazi olupheleleyo (CBC): Oku kulinganisa ubungakanani beeseli ezibomvu zegazi kunye ne-hemoglobin egazini. Ezi zihlala ziphantsi kwizigulane ezine-thalassemia.
• I-Hemoglobin Electrophoresis: Oku kunokumisela ukuba zeziphi iintlobo ze-hemoglobin ezisegazini lakho kunye nokuba zingakanani kuzo.
• Uvavanyo lweMfuzo: Olu vavanyo lunceda ekuchongeni uhlobo lwe-thalassemia olunayo.

Ukuxilongwa ngexesha lokukhulelwa

Ukuba wena okanye iqabane lakho nine-thalassemia carrier, ningamvavanya umntwana wenu ukuba akanaso esi sifo ngaphambi kokuba azalwe.

• Isampuli yeChorionic Villus (CVS): Isampuli encinci yeplacenta iyathathwa ize ivavanywe malunga neeveki ezili-11 zokukhulelwa.
• I-Amniocentesis: Malunga neeveki ezili-16 zokukhulelwa, kuthathwa isampulu yolwelo lwe-amniotic olujikeleze umntwana ize ivavanywe.

Ukuba le meko ifunyenwe, uza kuthunyelwa kwi-hematologist.

Ziziphi iindlela zonyango lwe-thalassemia?

Unyango luxhomekeke kubukhulu bemeko. Umntu onemeko engephi efana ne-thalassemia minor usenokungadingi naluphi na unyango, kodwa iimeko ezinzima zifuna unyango lobomi bonke.

Iindlela eziphambili zonyango zezi:

1. Utofelo-gazi

Izigulane ezine-thalassemia enzima zidinga utofelo-gazi ukuze zifumane iiseli ezibomvu zegazi ezisempilweni kunye ne-hemoglobin. Ngokuqhelekileyo kufuneka zitofelwe igazi rhoqo kwiiveki ezi-2-4 . Oku kubanika amandla okuqhubeka nemisebenzi yabo yesiqhelo ngaphandle kokudinwa.

2. Unyango lweChelation

Ngenxa yokunikela ngegazi rhoqo kunye neemeko zonyango, ubungakanani besinyithi emzimbeni bunokwanda ngokungeyomfuneko. Oku sikubiza ngokuba yi-"Iron Overload." Le sinyithi eyongezelelweyo inokuqokelelana kwizitho ezibalulekileyo ezifana nentliziyo nesibindi ize yonakalise.

3. Ezinye iindlela zonyango

• Ukufakelwa kweBone Marrow okanye iStem Cell: Ukufakelwa kweBone Marrow kumntu ophilileyo ngamanye amaxesha kunokunyanga ngokupheleleyo i-thalassemia. Nangona kunjalo, oku akwenziwa rhoqo ngenxa yeengozi kunye nobunzima bokufumana umntu ofanelekileyo.
• Utyando: Abanye abaguli bane-spleen ekhulisiweyo, efuna ukususwa ngotyando (i-spleenectomy).
• Amayeza: Amayeza anjenge-`Luspatercept (Reblozyl)` kunye ne-`Hydroxyurea` asetyenziselwa ukunceda ukuvelisa iiseli ezibomvu zegazi kunye nokulawula iimpawu.
• Izongezo: Ugqirha wakho unokucebisa iivithamini ezifana ne-folic acid, enceda ekwenzeni iiseli ezibomvu zegazi. Nangona kunjalo, musa ukuthatha izongezo zesinyithi ngaphandle kwengcebiso kagqirha nangasiphi na isizathu.

Ungaphila njani usempilweni xa une-thalassemia?

Ukunyamekela ezi zinto kubaluleke kakhulu kumntu ophila ne-thalassemia ukuze aphile ubomi obusempilweni nobonwabisayo.

• Landela imiyalelo kagqirha wakho: Landela unyango lukagqirha wakho njengoko luchazwe, ngexesha, kwaye uqiniseke ukuba uyaya kwiikliniki.
• Ukutya okunempilo: Yitya ukutya okunesondlo kunye neziqhamo nemifuno emininzi. Buza ugqirha wakho ukuba ufuna ukunciphisa ukutya okune-iron eninzi (inyama, intlanzi, iimbotyi) kunye nokutya okune-iron eninzi.
• Gonyelwa: Fumana zonke izitofu ezicetyiswa ngugqirha wakho, ingakumbi ezo zifana neyeza lomkhuhlane, kuba usenokuba semngciphekweni omkhulu wokosuleleka.
• Umthambo:Yenza umthambo olula ofanele umzimba wakho. Thetha nogqirha wakho ngawo.
• Ucoceko: Hlala kude nabantu abagulayo. Hlamba izandla zakho rhoqo.
• Kuphephe ukutshaya notywala: Ezi zinto azilunganga entliziyweni nasemathanjeni akho.
• Impilo yengqondo: Ukuphila nesifo esinganyangekiyo kunokukudinisa ngokweemvakalelo. Ukuba uziva uxinezelekile okanye ulusizi, thetha nosapho, abahlobo, okanye umcebisi wezempilo yengqondo .