Yonke into ekufuneka uyazi ngeThalassemia

Umxholo

• Iimpawu zethalassemia
• Oonobangela bethalasia
• Iindidi ezahlukeneyo zethalassemia
• Uxilongo thalassemia
• Izinketho zonyango lwe-thalassemia
• Thalassemia beta
• I-Thalassemia enkulu
• Imithombo yeendaba yeThalassemia
• I-alpha yeThalassemia
• IHemoglobin H
• IHydrops fetalis
• I-Thalassemia kunye ne-anemia
• Thalassemia kunye nemfuza
• Thalassemia encinci
• Thalassemia ebantwaneni
• Ukutya kwe-thalassemia
• Ukuxela kwangaphambili
• Izinto ozilindile emhlabane
• I-thalassemia ichaphazela njani ukukhulelwa?
• Imbonakalo

Yintoni ithalassemia?

I-Thalassemia sisifo segazi esizuzwe njengelifa apho umzimba wenza uhlobo olungaqhelekanga lwehemoglobin. IHemoglobin yimolekyuli yeprotini ekwiiseli ezibomvu zegazi ezithwala ioksijini.

Ukuphazamiseka kubangela ukutshatyalaliswa okugqithisileyo kweeseli ezibomvu zegazi, ezikhokelela kwi-anemia. I-anemia yimeko apho umzimba wakho ungenayo ngokwaneleyo iiseli ezibomvu zegazi eziqhelekileyo.

I-Thalassemia izuzwe njengelifa, oko kuthetha ukuba ubuncinci omnye wabazali bakho kufuneka abe ngumntu ophethe esi sifo. Kubangelwa kukutshintsha kwemfuzo okanye ukucinywa kwamaqhekeza athile abalulekileyo emfuza.

I-Thalassemia encinci yindlela ephantsi kakhulu yesifo. Zimbini iindlela eziphambili zethalassemia ezinzulu ngakumbi. Kwi-alpha thalassemia, ubuncinci enye yealpha globin yemfuza inokutshintsha okanye ukungaqheleki. Kwi-beta thalassemia, ii-beta globin genes ziyachaphazeleka.

Ngalunye lwezi ntlobo ze-thalassemia zinee-subtypes ezahlukeneyo. Eyona fomu unayo iya kubuchaphazela ubukhali beempawu zakho kunye nembonakalo yakho.

Iimpawu zethalassemia

Iimpawu zeThalassemia ziyahluka. Ezinye zezona ziqhelekileyo zibandakanya:

• ukukhubazeka kwamathambo, ngakumbi ebusweni
• umchamo omnyama
• ukukhula okucothayo kunye nophuhliso
• ukudinwa kakhulu kunye nokudinwa
• isikhumba esimthubi okanye esimthubi

Asinguye wonke umntu oneempawu ezibonakalayo ze-thalassemia. Iimpawu zokuphazamiseka zihlala zibonisa kamva ebuntwaneni okanye ebusheni.

Oonobangela bethalasia

I-Thalassemia yenzeka xa kukho ukungaqheleki okanye ukuguquka kwenye yohlobo oluchaphazelekayo kwimveliso yehemoglobin. Ilifa eli lokungaqheleki kubazali bakho.

Ukuba kuphela ngomnye wabazali bakho ophethe i-thalassemia, unokuhlakulela uhlobo lwesifo esaziwa ngokuba yi-thalassemia encinci. Ukuba oku kuyenzeka, ngekhe ubenazo iimpawu, kodwa uya kuba ngumntu ophetheyo. Abanye abantu abane-thalassemia encinci bahlakulela iimpawu ezincinci.

Ukuba bobabini abazali bakho bangabathwali be-thalassemia, unethuba elikhulu lokuzuza njengelona hlobo lubi sesi sifo.

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Iindidi ezahlukeneyo zethalassemia

Zintathu iintlobo eziphambili ze-thalassemia (kunye nee-subtypes ezine):

• beta thalassemia, ebandakanya ii-subtypes ezinkulu kunye ne-intermedia
• i-alpha thalassemia, ebandakanya ii-subtypes hemoglobin H kunye ne-hydrops fetalis
• thalassemia encinci

Zonke ezi ntlobo kunye nee-subtypes ziyahluka ngokweempawu kunye nobukhali. Ukuqala kunokuhluka kancinci.

Uxilongo thalassemia

Ukuba ugqirha wakho uzama ukuxilonga i-thalassemia, baya kuthatha isampuli yegazi. Baya kuthumela le sampuli kwilebhu ukuvavanywa i-anemia kunye ne-hemoglobin engaqhelekanga. Ingcali yeelebhu iya kujonga igazi phantsi kwemicroscope ukubona ukuba iiseli ezibomvu zegazi zimile ngendlela engaqhelekanga.

Iiseli ezibomvu ezime ngendlela engaqhelekanga luphawu lwe-thalassemia. Ingcali yeelebhu inokwenza uvavanyo olubizwa ngokuba yihemoglobin electrophoresis. Olu vavanyo lwahlulahlula iimolekyuli ezahlukeneyo kwiiseli ezibomvu zegazi, ezivumela ukuba zichonge uhlobo olungaqhelekanga.

Ngokuxhomekeke kuhlobo kunye nobunzima be-thalassemia, uvavanyo lomzimba lunokunceda ugqirha wakho ukuba enze isifo. Umzekelo, udakada owandisiweyo kakhulu unokucebisa ugqirha wakho ukuba unesifo sehemoglobin H.

Izinketho zonyango lwe-thalassemia

Unyango lwe-thalassemia luxhomekeke kuhlobo kunye nobukhali besifo esichaphazelekayo. Ugqirha wakho uya kukunika ikhondo lonyango eliza kusebenza kakuhle kwimeko yakho.

Olunye unyango lubandakanya:

• utofelo-gazi
• ukufakelwa umongo wethambo
• amayeza kunye nezongezelelo
• utyando olunokwenzeka ukususa udakada okanye inyongo

Ugqirha wakho unokukuyalela ukuba ungathathi iivithamini okanye izongezo ezineentsimbi. Oku kuyinyani ngakumbi ukuba ufuna utofelo-gazi kuba abantu abafumanayo baqokelela i-iron eyongezelelweyo umzimba ongenakuyikhupha ngokulula. Intsimbi inokwakha izicwili ezinokubulala.

Ukuba ufumana utofelo-gazi, unokufuna unyango lwe-chelation. Oku ngokubanzi kubandakanya ukufumana inaliti yemichiza ebopha ngentsimbi kunye nezinye izinyithi ezinzima. Oku kunceda ukususa i-iron eyongezelelweyo emzimbeni wakho.

Thalassemia beta

I-beta thalassemia yenzeka xa umzimba wakho ungenako ukuvelisa i-beta globin. Imfuza emibini, enye evela kumzali ngamnye, ifunyenwe njengelifa ukwenza i-beta globin. Olu hlobo lwe-thalassemia luza kwiindawo ezimbini ezinzulu: i-thalassemia enkulu (i-Cooley's anemia) kunye ne-thalassemia intermedia.

I-Thalassemia enkulu

I-Thalassemia enkulu yindlela ebaluleke kakhulu ye-beta thalassemia. Ikhula xa i-beta globin genes ilahlekile.

Iimpawu ze-thalassemia ezinkulu ngokubanzi zibonakala ngaphambi kokuzalwa kwesibini komntwana. I-anemia enzulu enxulumene nale meko inokusongela ubomi. Ezinye iimpawu kunye neempawu zibandakanya:

• ukuxhamla
• ubumhlophe
• usulelo rhoqo
• ukungathandi ukutya
• ukusilela ukuchuma
• i-jaundice, etyheli kolusu okanye abamhlophe bamehlo
• amalungu andisiweyo

Olu hlobo lwe-thalassemia luhlala luluqilima kangangokuba lufuna ukutofelwa igazi rhoqo.

Imithombo yeendaba yeThalassemia

I-Thalassemia intermedia yifom engaphantsi kakhulu. Ikhula ngenxa yotshintsho kuzo zombini ijini beta globin. Abantu abane-thalassemia intermedia abadingi gazi.

I-alpha yeThalassemia

I-alpha thalassemia yenzeka xa umzimba ungakwazi ukwenza i-alpha globin. Ukwenza i-alpha globin, kufuneka ubeneejini ezine, ezimbini kumzali ngamnye.

Olu hlobo lwe-thalassemia luneentlobo ezimbini ezinzulu: isifo se-hemoglobin H kunye ne-hydrops fetalis.

IHemoglobin H

IHemoglobin H ikhula njengaxa umntu elahlekileyo ezintathu zealpha globin zofuzo okanye amava atshintsha kwezi zemfuza. Esi sifo singakhokelela kwimicimbi yethambo. Izidlele, ibunzi, kunye nomhlathi zisenokugqitha. Ukongeza, isifo sehemoglobin H sinokubangela:

• inyongo
• udakada owandiswe ngokugqithisileyo
• ukungondleki

IHydrops fetalis

I-Hydrops fetalis yindlela ebaluleke kakhulu ye-thalassemia eyenzeka ngaphambi kokuzalwa. Uninzi lweentsana ezinale meko zizelwe zingafanga okanye zife kungekudala emva kokuzalwa. Le meko ikhula xa zonke iialpha globin zofuzo zitshintshiwe okanye zilahlekile.

I-Thalassemia kunye ne-anemia

I-Thalassemia inokukhokelela ngokukhawuleza kwi-anemia. Le meko iphawulwa kukungabikho kweoksijini ethunyelwa kwizicwili nakwizitho zomzimba. Kuba iiseli ezibomvu zegazi linoxanduva lokuhambisa ioksijini, inani elincinci leeseli lithetha ukuba awunayo ioksijini eyaneleyo emzimbeni nokuba.

I-anemia yakho inokuba mbi kakhulu. Iimpawu ze-anemia zibandakanya:

• isiyezi
• ukudinwa
• ukucaphuka
• ukuqhawukelwa ngumphefumlo
• ubuthathaka

I-anemia inokubangela ukuba uphume. Iimeko ezinzima zinokukhokelela kumonakalo kumzimba wonke, onokubulala.

Thalassemia kunye nemfuza

I-Thalassemia yimvelo ngokwemvelo. Ukuphuhlisa i-thalassemia epheleleyo, Zombini yabazali bakho kufuneka babe ngabathwali besi sifo. Ngenxa yoko, uya kuba neentsholongwane ezimbini eziguqulweyo.

Kwakhona kunokwenzeka ukuba ube ngumthwali we-thalassemia, apho unemfuza enye kuphela kwaye ingabi babini kubazali bobabini. Nokuba omnye okanye bobabini abazali bakho kufuneka abe unale meko okanye abe ophetheyo ngayo. Oku kuthetha ukuba uzuza ilifa kwisini esinye esiguqukileyo komnye wabazali bakho.

Kubalulekile ukuba uvavanywe ukuba omnye wabazali bakho okanye isizalwane sakho sinaso esi sifo.

Thalassemia encinci

Kwiimeko ezincinci ze-alpha, iijethi ezimbini zilahlekile. Kwi-beta encinci, uhlobo olunye alukho. Abantu abane-thalassemia encinci bahlala bengenazo iimpawu.Ukuba bayakwenza oko, kunokwenzeka ukuba ibe yi-anemia encinci. Imeko yahlulwa njenge-alpha okanye i-beta thalassemia encinci.

Nokuba i-thalassemia encinci ayibangeli zimpawu zibonakalayo, usengasiphatha isifo. Oku kuthetha ukuba, ukuba unabantwana, banokuphuhlisa uhlobo oluthile lokutshintsha kwemfuza.

Thalassemia ebantwaneni

Kuzo zonke iintsana ezizalwa zine-thalassemia minyaka le, kuqikelelwa ukuba i-100,000 izalwa ineefom ezinzima emhlabeni jikelele.

Abantwana bangaqala ukubonisa iimpawu ze-thalassemia kwiminyaka yabo emibini yokuqala yobomi. Ezinye zeempawu ezibonakalayo zibandakanya:

• ukudinwa
• inyongo
• ulusu oluthuthu
• ukungathandi ukutya
• ukukhula kancinci

Kubalulekile ukufumanisa i-thalassemia ngokukhawuleza ebantwaneni. Ukuba wena okanye omnye umzali womntwana wakho uphethe izinto, kuya kufuneka wenze uvavanyo kwangoko.

Xa ushiywe unganyangeki, le meko inokukhokelela kwiingxaki kwisibindi, entliziyweni, kunye nepeni. Usulelo kunye nokusilela kwentliziyo zezona zinto zixhaphakileyo ezisongela ubomi be-thalassemia ebantwaneni.

Njengabantu abadala, abantwana abane-thalassemia enzima bafuna ukutofelwa igazi rhoqo ukuze balahle i-iron engaphezulu emzimbeni.

Ukutya kwe-thalassemia

Ukutya okunamafutha asezantsi, ukutya okusekwe kwizityalo lolona khetho lufanelekileyo kubantu abaninzi, kubandakanya ne-thalassemia. Nangona kunjalo, kusenokufuneka unciphise ukutya okunotyebileyo ngentsimbi ukuba sele unawo amanqanaba aphezulu e-iron egazini lakho. Iintlanzi kunye nenyama zityebile ngentsimbi, ke kuya kufuneka ukhawuleze oku kwindlela otya ngayo.

Unokucinga ngokuthintela ukutya okuziinkozo okuqinileyo, isonka kunye nejusi. Ziqulethe amanqanaba aphezulu e-iron, nawo.

I-Thalassemia inokubangela ukusilela kwe-folic acid (folate). Ngokwendalo kufunyanwa kukutya okunjengamagqabi amnyama anamagqabi kunye nemidumba, le vithamini B ibalulekile ekukhuseleni iziphumo zamanqanaba aphezulu e-iron kunye nokukhusela iiseli ezibomvu zegazi. Ukuba awufumani ngokwaneleyo i-folic acid ekutyeni kwakho, ugqirha wakho unokucebisa ngesongezelelo se-1 mg esithathwa yonke imihla.

Akukho kutya kunokunyanga i-thalassemia, kodwa uqiniseke ukuba utya ukutya okufanelekileyo kunokukunceda. Qiniseka ukuba uxoxa ngalo naluphi na utshintsho kwindlela otya ngayo nogqirha wakho ngaphambi kwexesha.

Ukuxela kwangaphambili

Kuba i-thalassemia sisifo semfuza, akukho ndlela yokuyithintela. Nangona kunjalo, kukho iindlela onokuthi ulawule esi sifo ukunceda ukuthintela iingxaki.

Ukongeza kunyango oluqhubekayo, ukuba bonke abantu abanesifo bazikhusele kusulelo ngokugcina ezi zitofu zilandelayo:

• uhlobo haemophilus influenza b
• hepatitis
• imenococcal
• pneumococcal

Ukongeza kwindlela yokutya esempilweni, ukuzilolonga rhoqo kunokunceda ukulawula iimpawu zakho kwaye kukhokelele kwisifo sokuqaqamba komzimba. Ukusebenza okumodareyitha kuhlala kunconyelwa, kuba ukuzilolonga kakhulu kunokwenza iimpawu zakho zibe mandundu.

Ukuhamba ngeenyawo kunye nokuhamba ngebhayisikile yimizekelo yokusebenza ngamandla. Ukuqubha kunye neyoga zezinye iindlela, kwaye zilungile kumalungu akho. Isitshixo kukufumana into oyithandayo kwaye uqhubeke nokuhamba.

Izinto ozilindile emhlabane

I-Thalassemia sisifo esibi esinokubangela iingxaki ezisongela ubomi xa sishiywa singanyangwa okanye singanyangwa. Ngelixa kunzima ukukhomba ixesha lokuphila ngqo, umthetho jikelele kukuba imeko iba mandundu, i-thalassemia ekhawulezayo inokubulala.

Ngokwezinye iingqikelelo, abantu abane-beta thalassemia - eyona ndlela iqatha kakhulu- basweleka beneminyaka engama-30. Ubomi obufutshane bunento yokwenza nentsimbi ethe chatha, enokuchaphazela amalungu akho.

Abaphandi bayaqhubeka nokuphonononga ukuvavanywa kwemfuza kunye nonyango olunokwenzeka. I-thalassemia yangaphambili ifunyenwe, ngokukhawuleza unokufumana unyango. Kwixesha elizayo, unyango lwenzala lunokuphinda luvuselele i-hemoglobin kwaye lusebenzise utshintsho olungaqhelekanga lomzimba emzimbeni.

I-thalassemia ichaphazela njani ukukhulelwa?

I-Thalassemia izisa neenkxalabo ezahlukeneyo ezinxulumene nokukhulelwa. Esi sifo sichaphazela ukukhula kwamalungu okuzala. Ngenxa yoku, abasetyhini abane-thalassemia banokuhlangabezana nobunzima bokuzala.

Ukuqinisekisa impilo yakho nobabini nosana lwakho, kubalulekile ukucwangcisa kwangaphambi kwexesha kangangoko. Ukuba ufuna ukuba nomntwana, xoxa oku nogqirha wakho ukuze uqiniseke ukuba usempilweni engcono.

Amanqanaba akho e-iron ayakufuna ukubekwa esweni ngononophelo. Imicimbi esele ikhona kunye namalungu aphambili ayathathelwa ingqalelo.

Ukuvavanywa ngaphambi kokubeleka kwe-thalassemia kunokwenziwa kwiiveki ezili-11 ukuya kwezi-16. Oku kwenziwa ngokuthatha iisampulu ezinamanzi kwi-placenta okanye kwimveku, ngokulandelelana.

Ukukhulelwa kuthwala ezi zinto zilandelayo kumngcipheko kubasetyhini abane-thalassemia:

• umngcipheko ophezulu wosulelo
• isifo seswekile
• iingxaki zentliziyo
• i-hypothyroidism, okanye i-thyroid ephantsi
• ukwanda kwenani lotofelo-gazi
• Ubuninzi bamathambo

Imbonakalo

Ukuba une thalassemia, imbonakalo yakho ixhomekeke kuhlobo lwesifo. Abantu abaneendlela ezincinci okanye ezincinci ze-thalassemia banokuhlala ubomi obuqhelekileyo.

Kwiimeko ezinzima, ukusilela kwentliziyo kunokwenzeka. Ezinye iingxaki zibandakanya isifo sesibindi, ukukhula okungaqhelekanga kwamathambo, kunye nemicimbi ye-endocrine.

Ugqirha wakho angakunika ulwazi oluthe kratya malunga nembono yakho. Baza kuchaza nendlela unyango lwakho olunokukunceda ngayo ukuphucula umgangatho wobomi bakho okanye wandise ixesha lokuphila.