Ðe viwòa ƒe mo biã ɣesiaɣi eye ɖeɖi tea eŋua? Ðe wòate ŋu anye Thalassemia?

Ðe wòdze abe ɖeɖi tea viwò suea ŋu ɣesiaɣi enea? Ðe meƒua du nɔa fefem abe ɖevi bubuwo ene oa? Ðe wòtsia dzi vevie be yeaɖu nua? Ðe wòse le eɖokui me kpɔ be yeƒe mo biãa? Enye nusi sɔ be vidada alo vifofo ɖesiaɖe navɔ̃ ŋutɔ ne ekpɔ nu siawo tɔgbe. Togbɔ be zi geɖe la, nɔnɔme siwo sɔ gbɔe dzesi siawo ate ŋu atso hã la, ɣeaɖewoɣi la, atikewɔwɔ me nɔnɔme aɖe ate ŋu anɔ megbe na esia, abe Thalassemia ene. Eyata egbea, mina míaƒo nu tso nusi Thalassemia nye, alesi wògblẽa nu le míaƒe ŋutilã ŋui, kple atike siwo wozãna nɛ ŋu.

Nuka tututue nye Thalassemia?

Kpuie ko la, thalassemia nye domenyiŋusẽfianu ƒe dɔléle si gblẽa nu le míaƒe ʋu ŋu . Menye ame si xɔa ame me o. Ema fia be mekaka tso ame ɖeka me yi bubu me abe dzamezã ene o. Enye nusi ƒe dome míenyi tso mía dzilawo gbɔ to míaƒe domenyiŋusẽfianu dzi.

Be míase nya sia gɔme nyuie vie la, mina míaƒo nu tso ʋumenugbagbevi dzĩ siwo le míaƒe ʋu me ŋu vie gbã. Bu ʋumenugbagbevi dzĩ siawo be wonye ʋumenugbagbeviɣidɔ si tsɔa ɔksidzin yia míaƒe ŋutilã katã me. Woyɔa ʋu si wotsɔna ɖoa amewo gbɔ ƒe dɔwɔƒe sia, si nye tasiaɖam si tsɔa ɔksidzin, be hemoglobin . Hemoglobin nye protein tɔxɛ aɖe si le ʋumenugbagbevi dzĩwo me. Esiae tsɔa ɔksidzin tso míaƒe lãkusiwo me eye wòmaae ɖe lãmenugbagbevi ɖesiaɖe si le ŋutilãa me la me.

Ame si ŋu thalassemia le mate ŋu awɔ protein sia si woyɔna be hemoglobin wòade edeƒe o. Abe alesi ko ne wole ʋu wɔm ene la, ne ʋua ƒe akpa aɖewo mesɔ gbɔ o alo ne wozã eƒe akpa siwo bɔbɔ ɖe anyi wu la, ʋua mawɔ dɔ nyuie o. Esia wɔnɛ be ʋumenugbagbevi dzĩ siwo le lãmesẽ me ƒe xexlẽme dzi ɖena kpɔtɔna le ŋutilãa me. Míeyɔa nɔnɔme sia si me ʋumenugbagbevi dzĩwo mesɔ gbɔ ɖo o be ʋusɔgbɔdɔ .

Eyata ne lãmenugbagbevi siwo le ŋutilãa me mekpɔ oxygen agbɔsɔsɔme si sɔ o la, womate ŋu awɔ ŋusẽ si hiã o. Esia tae thalassemia dɔnɔwo kpɔa dzesi vovovowo ɖo.

Amekawoe le afɔku me wu be woaxɔ dɔléle sia?

Wosusu be domenyiŋusẽfianu ƒe tɔtrɔ siwo hea esia vɛ la dzɔ le amegbetɔwo me gbã abe ametakpɔnu aɖe tso asrãdɔ me ene. Eyata amesiwo ƒe domenyiŋusẽfianu do ƒome kple nuto siwo me asrã bɔ ɖo, abe Afrika, Europa ƒe dzigbe, kple dukɔ siwo le Ɣetoɖoƒe, Anyiehe, kple Ɣedzeƒe Asia ene la le afɔku gã aɖe me be woaxɔ thalassemia. Esi wònye be Sri Lanka hã nye Anyiehe Asia dukɔ ta la, thalassemia tsɔlawo kple dɔnɔwo le míaƒe dukɔmeviwo dome.

Nukae hea thalassemia vɛ? Na míalé ŋku ɖe eŋu nyuie.

"Oxygen-keke" ma si woyɔna be hemoglobin si megblɔ va yi la nye protein kɔsɔkɔsɔ ene. Woyɔa esiawo dometɔ eve be alpha globin kɔsɔkɔsɔwo, eye woyɔa eve mamlɛawo be beta globin kɔsɔkɔsɔwo.

Míexɔa "mɔfiame" alo "kɔda" siwo woatsɔ awɔ kɔsɔkɔsɔ siawo tso domenyiŋusẽfianu siwo ƒe dome míenyi tso mía dzilawo gbɔ la me. Bu domenyiŋusẽfianu siawo be wonye nuɖaɖa si wotsɔ wɔa nuɖuɖu. Ne vodada alo vodada aɖe le nuɖaɖaa ŋu la, womate ŋu aɖa nuɖuɖua nyuie o. Nenema ke ne vodada alo gbɔdzɔgbɔdzɔ aɖe le domenyiŋusẽfianu siawo ŋu la, alpha alo beta globin kɔsɔkɔsɔwo mawɔ nyuie o. Ɣemaɣie nɔnɔme aɖe si woyɔna be thalassemia dzɔna.

• Alpha globin kɔsɔkɔsɔwo: Esiawo hiã domenyiŋusẽfianu 4 hafi woate ŋu awɔ. Eve tso dadaa gbɔ eye eve tso fofoa gbɔ.
• Beta globin kɔsɔkɔsɔwo: Esiawo hiã domenyiŋusẽfianu eve hafi woate ŋu awɔ. Ðeka tso dadaa gbɔ eye ɖeka tso fofo gbɔ.

Thalassemia ƒomevi si nàxɔ nɔ te ɖe alpha alo beta kɔsɔkɔsɔ siawo dometɔ si me domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ le dzi. Domenyiŋusẽfianu siwo gblẽ ƒe xexlẽme dzie wokpɔa dɔlélea ƒe sesẽme.

Thalassemia ƒomevi vevi kawoe nye?

Woma thalassemia ɖe akpa vevi vovovowo me le alesi dɔlélea nu sẽe nu. Eyae nye be thalassemia ƒe nɔnɔme, thalassemia sue, thalassemia intermedia, kple thalassemia major . Le carrier nɔnɔme me la, ɖewohĩ dzesi aɖeke manɔ ŋuwò o. Alo ɖewohĩ ʋusɔgbɔdɔ si nu mesẽ ŋutɔ koe le ŋuwò. Thalassemia major ye nye dɔléle sesẽtɔ kekeake si hiã be woawɔ atike nɛ.

Wole ƒomevi vevi eve me, le alesi domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔa le alpha kɔsɔkɔsɔwo alo beta kɔsɔkɔsɔwo me nu.

1. Alpha Thalassemia ƒe dɔléle

Nɔnɔme sia dzɔna ne domenyiŋusẽfianu 4 siwo wɔa alpha globin kɔsɔkɔsɔwo dometɔ ɖeka alo esi wu nenema gblẽ. Dzesiawo ƒe sesẽme nɔ te ɖe domenyiŋusẽfianu siwo gblẽ ƒe xexlẽme dzi. Mina míalé ŋku ɖe esia ŋu le mɔ bɔbɔe aɖe nu be míase egɔme.

2. Beta Thalassemia ƒe dɔléle

Nɔnɔme sia dzɔna ne domenyiŋusẽfianu eve siwo wɔa beta globin kɔsɔkɔsɔwo dometɔ ɖeka alo evea siaa gblẽ (ɖeka tso vidadaa gbɔ, ɖeka tso fofoa gbɔ).

• Domenyiŋusẽfianu ɖeka si gblẽ: Woyɔa nɔnɔme sia be beta thalassemia minor alo carrier state. Dzesiawo nu mesẽna kura o.
• Domenyiŋusẽfianu eve siwo gblẽ: Dzesiawo ate ŋu atso esiwo le titina va ɖo esiwo nu sẽ.
• Woyɔa domedome nɔnɔmea be Thalassemia intermedia .
• Woyɔa dɔléle si nu sẽ wu be beta thalassemia major alo Cooley ƒe ʋusɔgbɔdɔ . Ame siawo hiã atikewɔwɔ ɣesiaɣi.

Nukawoe nye thalassemia ƒe dzesiwo?

Dzesi siwo nèkpɔna la nɔ te ɖe thalassemia ƒomevi si le ŋuwò kple eƒe sesẽ dzi bliboe.

Dzesi aɖeke mele eŋu o alo dzesi siwo nu mesẽ ŋutɔ o

Ne alfa domenyiŋusẽfianu eneawo dometɔ ɖeka koe bu na wò la, ke ɖewohĩ dzesi aɖeke madze le ŋuwò o. Ne alpha domenyiŋusẽfianu eve alo beta domenyiŋusẽfianu ɖeka gblẽ la, ɖewohĩ dzesi aɖeke madze ŋuwò kura o. Alo ɖewohĩ ʋusɔgbɔdɔ ƒe dzesi siwo nu mesẽ o, abe ɖeɖiteameŋu ƒe seselelãme ɣesiaɣi ene, koe anɔ ŋuwò.

Dzesi siwo le domedome (Thalsemia Intermedia) .

Ame siawo ate ŋu akpɔ dzesi bubuwo tsɔ kpe ɖe ʋusɔgbɔdɔ suewo ŋu.

• Nukpɔkpɔ ƒe kpododonu: Ðevi ƒe kɔkɔme kple eƒe kpekpeme medzina ɖe edzi le eƒe ƒexɔxɔ nu o.
• Ƒewuiwo ƒe heheɖemegbe.
• Ƒu ƒe dɔmawɔmawɔ nyuie:Nɔnɔmewo abe ƒumeŋuɖui ene.
• Aɖuɖɔtoe si lolo: Aɖuɖɔtoe nye ŋutinu aɖe si le míaƒe ƒodo ƒe miame si kpena ɖe mía ŋu míewɔa avu kple dɔlékuiwo.

Dzesi sesẽwo (Thalassemia Major) .

Dzesi sesẽwo dzena le nɔnɔmewo abe alpha domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ etɔ̃ (Hemoglobin H dɔléle) kple beta thalassemia major (Cooley ƒe ʋusɔgbɔdɔ). Zi geɖe la , dzesi siawo dzea egɔme dzena hafi ɖevia xɔ ƒe eve .

Tsɔ kpe ɖe dzesi siwo le etame ŋu la, àte ŋu akpɔ esiawo:

• Nuɖuɖua mevivina o.
• Ŋutilã si le ɣie alo ɣie (jaundice).
• Aɖuɖɔ viviti (si ƒe amadede le abe tii ene).
• Mo ƒe ƒuwo ƒe masɔmasɔ (le taɖu ƒe ƒuwo ƒe tsitsi fũ ta).

Aleke woawɔ anya dɔléle sia?

Zi geɖe la, wokpɔa thalassemia si sɔ gbɔ eye wòsẽna le ɖevimenɔɣi, elabena dzesiawo dzea egɔme dzena le ɖevi ƒe agbenɔƒe ƒe eve gbãtɔwo me. Wò ɖɔkta ate ŋu aɖe gbe be woado ʋu vovovowo kpɔ atsɔ aɖo kpe dɔlélea dzi.

• Ʋu Xexlẽme Blibo (CBC): Esia dzidzea hemoglobin agbɔsɔsɔme si le ʋua me, ʋumenugbagbevi dzĩwo ƒe xexlẽme, kple woƒe lolome. Ʋumenugbagbevi dzĩ siwo le lãmesẽ me mesɔ gbɔ ɖe amesiwo ŋu thalassemia le o eye hemoglobin hã mesɔ gbɔ o. Ʋumenugbagbevi dzĩawo hã ate ŋu anɔ sue wu alesi wòle.
• Reticulocyte count: Esia dzidzea ʋumenugbagbevi dzĩ siwo wowɔ yeyee ƒe xexlẽme. Esia ate ŋu ana nàkpɔe ɖa be wò ƒumeŋuɖui le ʋumenugbagbevi dzĩwo wɔm wòsɔ gbɔ hã.
• Iron ŋuti numekukuwo: Dodokpɔ sia kpena ɖe ame ŋu wòdea dzesii nyuie nenye be iron ƒe anyimanɔmanɔ alo thalassemia gbɔe wò ʋusɔgbɔdɔ tso.
• Hemoglobin electrophoresis: Esia nye dodokpɔ vevi aɖe koŋ si woatsɔ anya beta thalassemia.
• Domenyiŋusẽfianu dodokpɔ: Wozãa dodokpɔ sia tsɔ dea dzesi alpha thalassemia.

Nukawoe nye atike siwo wozãna na thalassemia?

Dɔdamɔnu siwo wozãna ɖaa na dɔléle sesẽwo abe thalassemia major enee nye ʋudodo kple iron ɖeɖeɖa ƒe atikewɔwɔ.

• Ʋudodo: Kpuie ko la, gotagome ʋudodoe nye esia. Wotsɔa ʋu si me ʋumenugbagbevi dzĩ siwo le lãmesẽ me le la naa ŋutilãa to ʋuka aɖe dzi. Esia nana ʋumenugbagbevi dzĩwo kple hemoglobin ƒe agbɔsɔsɔ si le lãmesẽ me gaɖoa wo me. Le thalassemia ƒe nɔnɔme sesẽwo me (e.g., beta thalassemia major) la, ate ŋu ahiã be woado ʋu edziedzi, abe kwasiɖa 2-4 ɖesiaɖe ene .
• Iron Chelation ƒe Atikewɔwɔ:Ʋudodo enuenu ƒe nugbegblẽ le ame ŋu vevie dometɔ ɖekae nye iron ƒe agbɔsɔsɔ si le ŋutilãa me ƒe dzidziɖedzi madzemadzee. Míeyɔa esia be gayibɔ ƒe agba si gbɔ eme . Iron sia si sɔ gbɔ wu ate ŋu aƒo ƒu ɖe ŋutinu veviwo abe míaƒe dzi kple aklã ene me eye wòagblẽ nu le wo ŋu. Eyata wonaa atike (atikekui alo atikekui) siwo ɖea iron sia si sɔ gbɔ ɖe ŋutilã me la ame siwo xɔa ʋu enuenu.
• Folic Acid Kpekpeɖeŋunanuwo: Wonaa folic acid be wòakpe ɖe ŋutilãa ŋu wòawɔ ʋumenugbagbevi siwo le lãmesẽ me.
• Ƒumeŋuɖui Kple Lãmenugbagbeviwo Dodo Ðe Ame Ŋu: Fifia esia koe nye atike si ate ŋu ada thalassemia keŋkeŋ . Gake le esia ta la, dɔnɔa hiã na nunala si le lãmesẽ me si sɔ bliboe. Zi geɖe la, nɔviŋutsu alo nɔvinyɔnue. Esia nye atikewɔwɔ si me afɔku le ŋutɔ eye wòsesẽ ŋutɔ.
• Luspatercept: Esia nye atike si wodona kwasiɖa etɔ̃ ɖesiaɖe. Ewɔa dɔ to kpekpeɖeŋu nana ŋutilãa be wòawɔ ʋumenugbagbevi dzĩ geɖe me.

Aleke agbenɔnɔ le kple dɔléle sia? Ðe woate ŋu axe mɔ ɖe enua?

Ne thalassemia minor le ŋuwò la, àte ŋu akpɔ mɔ be yeanɔ agbe si sɔ. Eɖanye wò dɔlélea le titina alo sesẽ o, ne èwɔ ɖe atikewɔwɔ ƒe ɖoɖo si woŋlɔ ɖi (ʋudodo kple gayibɔ ɖeɖeɖa ƒe atikewɔwɔ) dzi pɛpɛpɛ la, mɔnukpɔkpɔ nyui aɖe asu asiwò be nànɔ agbe didi.

Dɔléle sia tso domenyiŋusẽfianu me, eya ta míate ŋu axe mɔ nɛ o. Gake domenyiŋusẽfianu dodokpɔ ate ŋu akpe ɖe ŋuwò nànya ne thalassemia domenyiŋusẽfianu le wò alo wò zɔhɛa ŋu. Nyatakaka siawo nyanya le vevie ŋutɔ na srɔ̃tɔ siwo le ɖoɖo wɔm be yewoadzi vi. Ƒo nu kple wò ɖɔkta hena aɖaŋuɖoɖo bubuwo tso esia ŋu.

Mlɔeba la, thalassemia nye dɔléle si dzi woate ŋu aɖu. Atikewɔwɔ si ƒomevi nèhiã kple zi nenie nàhiãe anɔ te ɖe alesi wò nɔnɔmea nu sẽe dzi. Ele vevie be nàƒo nu faa kple wò ɖɔkta tso wò nɔnɔmea kple wò beléle na wò ɣeyiɣi didi ƒe ɖoɖo ŋu.