Ðe wòdze abe ɖe viwòa ƒe lãmekawo le gbɔdzɔgbɔdzɔm enea? Mina míaƒo nu tso (Becker Muscular Dystrophy) sia ŋu!

Ède dzesii kpɔ be azɔlizɔzɔ, duƒuƒu, alo atrakpui lialia sesẽna na ɖevi alo sɔhɛ aɖewo vie wu ame bubuwoa? Alo ɖe nèse le ɖokuiwò me kpɔ be woƒe lãmekawo le gbɔdzɔgbɔdzɔm vivivia? Ðewohĩ nusitae nye nɔnɔme si ŋu míele nu ƒom tsoe egbea, si woyɔna be `(Becker Muscular Dystrophy)`. Mègatsi dzi o, mina míaɖe nusianu me le nya bɔbɔewo me.

Nukae nye `(Becker Lãmetsiŋusẽ ƒe Dɔléle)`? Ne míagblɔe kpuie ŋutɔ la...

`(Becker Muscular Dystrophy)`, si woyɔna hã be `(BMD)` kpuie la, nye domenyiŋusẽfianu ƒe nɔnɔme si mebɔ o. Nusi dzɔnae nye be lãmeka siwo le ŋutilãa me la gbɔdzɔna vivivi eye woƒe dɔwɔwɔ dzi ɖena kpɔtɔna. Ne míagblɔe tututu la, domenyiŋusẽfianu ƒe nɔnɔme aɖee nye esia. Ŋutsuviwo kple ŋutsuwo koŋue nɔnɔme sia gblẽa nu le. Nusitae nye `(X-linked inheritance)`, si fia be wonyie tso vidadaa gbɔ (ne enye amesi tsɔe) yi ŋutsuvi gbɔ.

Zi geɖe la, lãmeka ƒe gbɔdzɔgbɔdzɔ sia dzea egɔme tso wò afɔwo kple akɔta, eye le ɣeyiɣi aɖe megbe la, ate ŋu akaka ɖe wò abɔta, si fia wò tame.

Le lãmekawo ƒe dɔmawɔmawɔ nyuie ƒomevi siwo wonya fifia dome la, BMD ate ŋu anye ƒomevi etɔ̃lia si bɔ wu le ame tsitsiwo dome, le myotonic dystrophy kple facioscapulohumeral dystrophy megbe.

Vovototo kae le `(Becker Muscular Dystrophy)` kple `(Duchenne Muscular Dystrophy)` dome?

Ðewohĩ èse nɔnɔme aɖe si woyɔna be `(Duchenne Muscular Dystrophy)` alo `(DMD)` ŋu nya kpɔ. `(BMD)` kple `(DMD)` siaa tso domenyiŋusẽfianu ɖeka ƒe tɔtrɔ gbɔ, si nye domenyiŋusẽfianu si ŋlɔa protein si woyɔna be `(dystrophin).` Protein sia si woyɔna be `(dystrophin)` le vevie ŋutɔ na míaƒe lãmekawo ƒe lãmesẽ.

Gake vovototoae nye esi:

• Dystrophin protein aɖeke kloe mele amesi ŋu DMD le ƒe lãmekawo me o.
• Dystrophin aɖe le amesi ŋu BMD le ƒe lãmekawo me, gake mesɔ gbɔ o.

Eyata nɔnɔme `(BMD)` mesẽ vie wu `(DMD)`, eye dzesiawo dzena eye woyia ŋgɔ blewu wu `(DMD)`. Gake dzesiawo sɔ le akpa gãtɔ me le wo ame evea siaa gome.

Amekae dɔléle sia (Becker Muscular Dystrophy) gblẽa nu le wu?

Abe alesi míegblɔe va yi ene la, ŋutsuwo koŋue gblẽa nu le BMD ŋu. Gake nyɔnu siwo tsɔa BMD (i.e., amesiwo tsɔ domenyiŋusẽfianu si hea dɔlélea vɛ gake womeɖea dzesi aɖeke fiana o) ate ŋu adze dzesiwo gɔme ɣeaɖewoɣi. Ke hã, zi geɖe la, womesẽna nenema gbegbe o, eye womesẽna kura o.

Zi geɖe la, dzesiawo dzea egɔme tso ƒe 5 va ɖo ƒe 15. Gake ame aɖewo ate ŋu akpɔ dzesiawo emegbe.

Aleke gbegbee `(Becker Muscular Dystrophy)` bɔe?

Le nyateƒe me la, BMD nye dɔléle si mebɔ o.Nɔnɔmea dzea vidzĩ 3 va ɖo 6 dzi le vidzĩ 100,000 ɖesiaɖe si wodzi me. Eye abe alesi míegblɔe ene la, ŋutsuviwoe wògblẽa nu le wu.

Nukae nye `(Becker Muscular Dystrophy)` ƒe dzesiwo?

Zi geɖe la, BMD ƒe dzesiwo dzea egɔme tso ƒe 5 va ɖo ƒe 15 dome, gake ate ŋu ava emegbe. Nusi dzɔnae nye be lãmekawo ƒe gbɔdzɔgbɔdzɔ dzina ɖe edzi vivivi le ɣeyiɣi aɖe megbe. Eyata, dzesi siwo bɔ wue nye:

• Atrakpuiwo lialia sesẽ.
• Azɔlizɔzɔ sesẽ, kple sesẽ si le dzidzim ɖe edzi le ɣeyiɣi aɖe megbe.
• Kamedede ƒe ŋutete dzi ɖe kpɔtɔ (ɖeɖiteameŋu ƒe seselelãme ne wodze agbagba vie gɔ̃ hã).
• Lãmetsiwo ƒe vevesese kple/alo lãmekawo ƒe ʋuʋu (abe lãmekawo ƒe ʋuʋu ene).
• Amewo ƒe anyidzedze enuenu.
• Afɔbidɛ ƒe zɔzɔ.
• Ðeɖiteameŋu ƒe seselelãme ɣesiaɣi (Ðeɖiteameŋu).

Bu eŋu kpɔ, ne viwòa mele du ƒum hele fefem abe tsã ene o, eye wògblɔ be "Dada, ɖeɖi te ŋunye" le azɔlizɔzɔ vie megbe gɔ̃ hã, alo ne ɖeɖi tea eŋu kabakaba wu ɖevi bubuwo ne ele fefem le suku la, anyo be nàtsi dzi ɖe eŋu vie.

Tsɔ kpe ɖe esia ŋu la, BMD ate ŋu ahe dzesi bubuwo vɛ:

• Dzitodzito me dɔléle : Esia nye nusi ŋu wòle be míaɖɔ ŋu ɖo.
• Yagbɔgbɔ sesẽ.
• Vovototo aɖewo le nusɔsrɔ̃ me (abe ɣeyiɣi didi hafi woase nu aɖewo gɔme wu bubuwo ene).
• Ŋutilã ƒe dadasɔ kple nuwɔwɔ aduadu ƒe bu.

Dzitodzito me dɔléle alo lãmekawo ƒe gbɔdzɔgbɔdzɔ si nu mesẽ ŋutɔ koe ate ŋu anɔ nyɔnu siwo tsɔa BMD. Wobu akɔnta be amesiwo tsɔa dɔlékuiawo dometɔ siwo ade 22% axɔ dzesiwo, gake esia toa vovo ŋutɔ tso ame ɖeka gbɔ yi bubu gbɔ.

Nukae hea `(Becker Lãmetsiŋusẽ ƒe Dɔléle)` vɛ?

BMD nye domenyiŋusẽfianu ƒe nɔnɔme si ƒe dome wonyi. Domenyiŋusẽfianu si ŋlɔa protein si woyɔna be dystrophin ƒe tɔtrɔ gbɔe wòtsona. Dystrophin le vevie ŋutɔ be lãmeka siwo le míaƒe ŋutilã me nasẽ eye woali ke.

Eyata ne tɔtrɔ va le `(dystrophin)` domenyiŋusẽfianu sia me la, womewɔa `(dystrophin)` protein la o, alo agbɔsɔsɔ si wowɔna dzi ɖena kpɔtɔna ŋutɔ. Esia wɔe be le ɣeyiɣi aɖe megbe la, lãmekawo gbɔdzɔna eye wodzea gbegblẽ gɔme.

Aleke Becker Muscular Dystrophy nyi domee? Esia nye nusi gɔme wòle be nàse vie!

`(BMD)` nye domenyinu le mɔ si woyɔna be `(X-linked recessive inheritance)` nu. Azɔ mina míase nya sia gɔme bɔbɔe.

• X-kadodo fia be domenyiŋusẽfianu si gbɔ BMD tsona la le X domenyiŋusẽfianu dzi. Abe alesi nènyae ene la, gbɔdɔdɔ ƒe domenyiŋusẽfianu eve le mía si, X kple Y.
• Recessive fia be hafi dɔléle sia nate ŋu adzɔ la, ele be domenyiŋusẽfianu si sɔ ƒe kɔpi eveawo siaa (domenyiŋusẽfianu ɖesiaɖe kloe ƒe kɔpi eve le mía si) nanye dɔlékui ƒe tɔtrɔ alo tɔtrɔ si hea dɔlélea vɛ.

Gake nu vevitɔ kekeakee nye esi:

• X domenyiŋusẽfianu ɖeka le ŋutsuwo (XY) si. Eyata, ne gbɔdzɔgbɔdzɔ aɖe le domenyiŋusẽfianu si sɔ me le X domenyiŋusẽfianu ɖeka ma dzi la, esɔ gbɔ be wòahe `(BMD)` vɛ.
• X domenyiŋusẽfianu eve (XX) le nyɔnuwo si . Eyata hafi dɔléle si do ƒome kple X nadzɔ la, zi geɖe la, ele be domenyiŋusẽfianu ƒe kɔpi eveawo siaa nagblẽ. Gake woyɔa nyɔnu siwo ƒe domenyiŋusẽfianu si gblẽ le X domenyiŋusẽfianu ɖeka ko dzi be "ametsɔlawo." Zi geɖe la, dɔlékui siawo meɖea dzesi aɖeke fiana o. Gake ƒã ŋutɔ la, dzesi siwo mesẽ o alo esiwo mesɔ gbɔ o ate ŋu ado mo ɖa.

Azɔ kpɔ alesi esia yia edzi le dzidzimewo me ɖa:

• Le vidada si nye amesi tsɔa nu (si ƒe domenyiŋusẽfianu gblẽ le X domenyiŋusẽfianu ɖeka dzi) gome la :
• Ne wodzi viŋutsuvi la, mɔnukpɔkpɔ 50% li be viŋutsua naxɔ nɔnɔme si nye `(BMD)`.
• Ne vinyɔnuvi le asiwò la, mɔnukpɔkpɔ 50% li be wòanye amesi tsɔe.
• Le vifofo si ŋu (BMD) dɔlélea le gome la :
• Mate ŋu atsɔ dɔléle sia ana viaŋutsuwo o (elabena vifofoa tsɔa Y domenyiŋusẽfianu la naa viaŋutsua).
• Gake vianyɔnuawo katã anye amesiwo tsɔa wo (elabena vifofoa naa X domenyiŋusẽfianu si gblẽ la vinyɔnua).

Èse egɔmea? Esia adze nusi sesẽ vie, gake ne míagblɔe kpuie ko la, ŋutsuvi ate ŋu axɔ esia tso dadaa gbɔ wu, ne dadaa nye amesi tsɔe.

Aleke wokpɔa `(Becker Muscular Dystrophy)` dzea sii?

Ne wosusu be BMD le mia alo viwòa ŋu la, anɔ eme be wò ɖɔkta awɔ ŋutilãmedɔ, ahɔhɔ̃mekawo dodokpɔ, kple lãmekawo dodokpɔ. Woabia nya wò tso wò dzesiwo kple wò atikewɔwɔ ŋutinya hã ŋu, eye nenye be dɔléle sia tɔgbe nɔ wò ƒomea me tɔ aɖe hã ŋu.

Le dodokpɔ siawo me la, ɖɔktaa ate ŋu akpɔ nusiwo le abe:

• Lãmetsi siwo le afɔwo kple akɔtawo me la vuvu.
• Togbɔ be lãmeka siwo le akɔta ƒe akpaa ate ŋu adze gã ne wokpɔ wo zi gbãtɔ hã (woyɔa esia be ``pseudohypertrophy'' ), le nyateƒe me la, wogbɔdzɔna. Ðeko wòle abe ɖe wofu tso ememe yi gota ene.
• Akɔta ƒe ƒoƒo (scoliosis) kple akɔta ƒe nɔnɔme madeamedzi aɖewo.
• Le kpɔɖeŋu me, lãmekawo ƒe dɔmawɔmawɔ nyuie, lãmekawo, lãkusiwo, kple ŋutigbalẽ ƒe tete tegbee le afɔkpodzi kple afɔwo me (wo ƒe tete) .

Dodokpɔ kawoe wozãna tsɔ dea dzesi `(Becker Muscular Dystrophy)`?

Ne wò ɖɔkta susui be BMD le ŋuwò alo viwòa la, ate ŋu aɖo aɖaŋu na wò be nàwɔ dodokpɔ siwo gbɔna:

• Creatine kinase ʋudodokpɔ: Ne lãmekawo gblẽ la, woɖea enzyme aɖe si woyɔna be creatine kinase ɖe ʋua me. Ame si ŋu BMD le ate ŋu akpɔ creatine kinase sia ƒe agbɔsɔsɔ si sɔ gbɔ zi gbɔ zi atɔ̃ alo esi wu nenema.
• Domenyiŋusẽfianu ƒe ʋudodokpɔ: Domenyiŋusẽ ƒe dodokpɔ sia si léa ŋku ɖe Dystrophin domenyiŋusẽfianu ƒe tɔtrɔ aɖe ŋu la ate ŋu anya BMD godoo.

Ne woɖo kpe edzi be BMD le ŋuwò alo viwòa ŋu la, wò ɖɔkta ate ŋu aɖo aɖaŋu hã be woawɔ Electrocardiogram (EKG) alo Echocardiogram atsɔ akpɔe ɖa be dzitodzito me lãmekawo ƒe kuxi siwo ate ŋu atso BMD gbɔ hã.

Aleke wodaa Becker Muscular Dystrophy ƒe dɔléleae?

Nublanuitɔe la, atike aɖeke meli na BMD fifia o. Eyata atikewɔwɔ ƒe taɖodzinu vevitɔe nye be woaɖu dzesiawo dzi eye woalé agbenɔnɔ ƒe nyonyome nyuitɔ kekeake me ɖe asi.

Atike vevi eve li na BMD:

1. Corticosteroids: Le kpɔɖeŋu me, atikewo abe prednisolone ene. Esiawo kpena ɖe ame ŋu be lãkusi ƒe dɔwɔwɔ nyona ɖe edzi, woɖea scoliosis ƒe tsitsi dzi kpɔtɔna, dzitodzito me dɔléle ƒe dzɔdzɔ blewu, eye wòdidia ame ƒe agbenɔƒe ɖe edzi.

2. Gbugbɔgaɖɔɖo: Esiawo kpena ɖe dɔnɔa ŋu wòléa eƒe dɔwɔwɔ ƒe ŋutete me ɖe asi ɣeyiɣi didi wu eye wònana eƒe agbenɔnɔ nyona ɖe edzi.

• Lãmesẽfefewɔwɔ kpena ɖe ame ŋu wòdoa ŋusẽ lãmekawo.
• Nuƒoƒo ƒe atikewɔwɔ, dɔdamɔnu, kple modzakaɖeɖe ate ŋu akpe ɖe gbesiagbedɔwo ŋu.

Tsɔ kpe ɖe esia ŋu la, atikewɔmɔnu bubuwo li siwo ate ŋu akpe ɖe ame ŋu le BMD me:

• Ʋuʋu ƒe kpekpeɖeŋunuwo na nuwo abe azɔlizɔzɔ ene - e.g. atikplɔwo, bunɔkekewo, akɔtaɖonuwo.
• Atike siwo wozãna na `(Dzitodzito me dɔléle)` - e.g. `(ACE mɔxenuwo)` kple `(beta-mɔxenuwo)` .
• Amekoko be woakpe ɖe ame ŋu le scoliosis kple contractures me.
• Ne gbɔgbɔ sesẽ va nu sẽ (yagbɔgbɔ ƒe dɔmawɔmawɔ nyuie) , ate ŋu ahiã be woaɖe yagbɔgbɔ (amekoko si wowɔna tsɔ ʋua yagbɔgbɔ) kple gbɔgbɔ si wowɔ kple asi.

Nya nyuiae nye be wole atike yeye geɖe siwo woatsɔ ada `(BMD)` la dom kpɔ fifia eye woate ŋu ado ŋugbe le etsɔme. (Gbegɔmeɖeɖe ƒe vodada le dzɔtsoƒe, ele be wòanye Sinhalagbe: Nya nyuiae nye be atike yeye geɖe siwo woatsɔ ada `(BMD)` la le dodokpɔ wɔm le dɔdaƒe fifia. Woate ŋu anye ŋugbedodo le etsɔme.)

Dzɔgbenyuietɔe la, wole atike yeye geɖe siwo ate ŋu ada BMD la dom kpɔ fifia, eye míate ŋu akpɔ mɔ be nu nyuiwo ado tso wo me le etsɔme.

Ðe woate ŋu axe mɔ ɖe Becker Muscular Dystrophy nua?

Esi wònye be BMD nye domenyiŋusẽfianu ƒe dɔléle ta la, naneke meli míate ŋu awɔ atsɔ axe mɔ ɖe enu o.

Gake ne BMD le ŋuwò, alo ne ètsi dzi be ɖewohĩ BMD alo domenyiŋusẽfianu bubu aɖe le ye ŋu la, ƒo nu tso eŋu na wò ɖɔkta hafi nàdzi vi.Enyo ŋutɔ be nàdi domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo.

Nukae nye Becker Muscular Dystrophy ƒe ŋgɔdonya?

Ame si ŋu BMD le ƒe mɔkpɔkpɔ ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Esia nye nuwɔametɔnyenye ƒe ŋgɔyiyi vivivi. Gake nuwɔametɔnyenyea ƒe sesẽme to vovo. Ame aɖewo ate ŋu ahiã bunɔkeke, evɔ ame bubuwo ya ahiã be woazã azɔlizɔzɔ ƒe kpekpeɖeŋunuwo (atikplɔ, atizɔtiwo) ko.

Ke hã, ne dzidɔ alo gbɔgbɔtsixe tsia ame aɖe si ŋu ``(BMD)`` le la, woate ŋu aɖe woƒe agbenɔƒe dzi akpɔtɔ.

Kuxi siwo ate ŋu ado mo ɖa le BMD tae nye:

• Dzi ƒe kuxiwo, vevietɔ `(Cardiomyopathy)`.
• Gbɔgbɔtsixe si gbɔgbɔtsixe ƒe lãmekawo ƒe gbɔdzɔgbɔdzɔ hena vɛ.
• Aklãdɔ alo gbɔgbɔtsixe ƒe dɔléle bubuwo.
• Le ɣeyiɣi aɖe megbe la, nuwɔametɔnyenyea dzina ɖe edzi eye amea megate ŋu wɔa eƒe dɔ le eɖokui si o.
• Ƒu ƒe gbagbã.

Nukae nye ame si ŋu `(Becker Muscular Dystrophy)` le ƒe agbenɔƒe?

Zi geɖe la, ame si ŋu ``BMD'' le ƒe agbenɔƒe nɔa kpuie vie. Eyae nye be, anɔ ƒe 40 va ɖo ƒe 50 dome. ``Dilated cardiomyopathy'' (nɔnɔme si me dzi ƒe lãmeka gbɔdzɔna eye wòlolona) ye nye nusi koŋ wua ame.

Aleke mawɔ akpɔ ame aɖe si ŋu `(BMD)` le dzi? Alo aleke malé be na ɖokuinye?

Ne BMD le ŋuwò la, ele vevie be nàxɔ atike nyuiwo atsɔ axe mɔ ɖe BMD ƒe kuxiwo abe dzidɔ kple gbɔgbɔmekuxiwo ene nu alo ada wo. Ate ŋu aɖe vi hã be nàge ɖe kpekpeɖeŋunaha aɖe si me nàte ŋu agblɔ wò nuteƒekpɔkpɔwo le eye nàdo go ame bubu siwo se nu gɔme na wò.

Ne èle ame aɖe si ŋu BMD le dzi kpɔm la, ele vevie be nàkpɔ egbɔ be ele atike nyuitɔ kekeake, azɔlizɔzɔ ƒe kpekpeɖeŋunu siwo wòhiã, kple atikewɔmɔnu siwo kpena ɖe eŋu be wòawɔ dɔ le eɖokui si la xɔm. Wòe nye amesi wòle be nànye woƒe taʋlila.

Ɣekaɣie wòle be makpɔ ɖɔkta tso `(Becker Muscular Dystrophy)` ŋu?

Ne wokpɔ Becker Muscular Dystrophy le ŋuwò (alo viwòa) la, ele vevie ŋutɔ be nàyi wò atikewɔlawo ƒe ƒuƒoƒoa gbɔ edziedzi be woawɔ atike nɛ eye nàlé ŋku ɖe wò dzesiwo ŋu.

Míenya be dɔléle si wokpɔna abe Becker Muscular Dystrophy ene gɔmesese kple egbɔkpɔkpɔ mele bɔbɔe o. Ate ŋu agbɔ eme. Wò atikewɔlawo ƒe ƒuƒoƒoa ana nàkpɔ egbɔkpɔnu sesẽ aɖe si sɔ ɖe wò dzesiwo nu. Ele vevie be nàkpɔ egbɔ be yele kpekpeɖeŋu si nèhiã la xɔm eye nèle wò lãmesẽ dzi kpɔm.

Kpuie ko la, nusiwo dzi wòle be míaɖo ŋkui (Take-Home Message) .

Enyo, eyata nu bɔbɔe aɖewo siwo dzi wòle be woaɖo ŋkui tso `(Becker Muscular Dystrophy)` alo `(BMD)` siwo ŋu míeƒo nu tsoe ŋue nye esi:

• ``(BMD)`` nye domenyiŋusẽfianu ƒe dɔléle si wotsɔna tsoa dzidzimewo me. Le esia me la, lãmekawo gbɔdzɔna vivivi.
• Nu siaŊutsuwoe wògblẽa nu le wu.
• Nusi gbɔ wòtsoe nye domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ si na protein la nye ``dystrophin''.
• Zi geɖe la, dzesiawo dzea egɔme tso ɖevime (le ƒe 5 va ɖo ƒe 15 dome). Dzesiawo dometɔ aɖewoe nye azɔlizɔzɔ sesẽ, ɖeɖiteameŋu, kple anyidzedze enuenu.
• Dzitodzito me dɔléle (dzi ƒe lãmekawo ƒe dɔléle) kple gbɔgbɔtsixe ate ŋu anye nɔnɔme sia ƒe kuxi veviwo.
• Fifia la, womekpɔ atike aɖeke na dɔléle sia o. Ke hã, atike vovovowo li siwo woate ŋu azã atsɔ aɖu dzesiawo dzi eye woana agbenɔnɔ nanyo ɖe edzi (e.g., corticosteroids, ŋutilãmedɔdada).
• Ne dɔléle sia le ame aɖe ŋu le ƒomea me la, nunya anɔ eme be nàdi domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo hafi adzi vi.
• Ele vevie ŋutɔ hã be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo kple atikewɔwɔ edziedzi, kpakple be wò susu nasẽ ŋu.

Mègaŋlɔ be o, menye wò ɖeka koe o. Ne èle nusia me tom la, di kpekpeɖeŋu tso ɖɔktawo, ƒometɔwo, xɔlɔ̃wo, kple kpekpeɖeŋunahawo gbɔ. Anye ŋusẽtsoƒe gã aɖe na wò!

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` Becker lãmeka ƒe dɔmawɔmawɔ nyuie, BMD, lãmeka ƒe gbɔdzɔgbɔdzɔ, domenyiŋusẽfianu ƒe dɔlélewo, dystrophin, X-kadodo, domenyiŋusẽfianu ƒe tɔtrɔ, ɖeviwo ƒe lãmesẽ, dzidɔ, ŋutilãmedɔdada