Ðewohĩ èkpɔ ɖevi aɖe si le lãmesẽ me, eƒua du henɔa fefem, vivivi la, azɔlizɔzɔ alo nuƒoƒo sesẽna nɛ, alo eƒe nuwɔna nɔa tɔtrɔm vivivi. Abe dzila ene la, ne wonɔ edzi dzem nyuie le gɔmedzedzea me, eye emegbe wose le wo ɖokui me be yewo vi ƒe ŋutetewo le gbegblẽm ɖe edzi le ɣeyiɣi aɖe megbe la, esesẽna be nàgblɔ vɔvɔ̃ kple dzimaɖitsitsi si nèsena le ɖokuiwò me la ɖe nyagbewo me. Nusi gbɔ esia tso ate ŋu anye dɔléle aɖe si mebɔ o si ŋu mía dometɔ aɖeke mese kpɔ o. Egbea míele nu ƒom tso dɔléle mawo tɔgbe ƒe ƒuƒoƒo aɖe ŋu. Emae nye ʋumenugbagbeviɣidɔ .
Kpuie ko la, nukae nye ʋumenugbagbeviɣidɔ?
Menye dɔléle ɖeka koe ʋumenugbagbeviɣidɔ nye o. Enye dɔlélewo ƒe ƒuƒoƒo aɖe si gblẽa nu le míaƒe lãmeka siwo le titina (si nye ahɔhɔ̃ kple aklã) ŋu. Ðɔktawo le nu yeyewo dim tso eŋu ɣesiaɣi. Fifia la, wode dzesi dɔléle sia ƒomevi siwo ade 52.
Leukodystrophies ƒomevi siawo dometɔ geɖe nye domenyiŋusẽfianu . Eyae nye be wonyi dome tso dzilawo gbɔ na viwo. Ɣeaɖewoɣi la, ɖevia te ŋu ɖea dzesiwo fiana le edzidzi megbe teti. Gake woate ŋu adzi ɖevi aɖewo kple domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ si do ƒome kple dɔlélea, gake le gɔmedzedzea me la, woate ŋu adze abe ɖe wole lãmesẽ me keŋkeŋ ene.
Nu vevitɔe nye be nɔnɔme siawo nɔa ŋgɔ yim, si fia be ɖevi si le lãmesẽ me le gɔmedzedzea me ate ŋu adze dzesiwo gɔme emegbe.
Nukae nye dɔléle sia ƒe dzesiwo?
Nu ɖeka aɖe le ʋumenugbagbeviɣidɔ siawo katã ŋu: wogblẽa nu le myelin ƒe akɔta si le ahɔhɔ̃a me ŋu.
Kpɔe ɖa le susu me be elektrikka aɖe le míaƒe aƒea me. Plastik-kplo aɖe le akɔbli ka si le eme la ŋu, alo? Emae na elektrikŋusẽa te ŋu yia afisi tututu wòle be wòayi, evɔ medona le eme o. Nenema ke ami ɣi aɖe si woyɔna be ‘myelin’ ƒo xlã míaƒe lãmekawo. Myelin-kplo sia ƒe dɔe nye be wòtsɔa lãmekawo ƒe gbedasiwo yia ahɔhɔ̃a me pɛpɛpɛ eye wòtsɔa wo tsoa ahɔhɔ̃a me yia ŋutilãa ƒe akpa aɖewo. Ne myelin-kplu sia gblẽ la, míaƒe lãmekawo mete ŋu wɔa dɔ nyuie o.
Leukodystrophy ƒomevi ɖesiaɖe gblẽa nu le myelin ƒe akɔta sia ŋu le mɔ vovovowo nu, eyata kuxi siwo ɖeviwo ate ŋu ado goe ate ŋu ato vovo ŋutɔ.
| Nuto si me dzesiawo dzena le | Kuxi siwo bɔ siwo wokpɔna |
|---|---|
| Ŋutilã ƒe dadasɔ kple eƒe ʋuʋu | Azɔlizɔzɔ sesẽ, anyidzedze enuenu, ŋutetemanɔamesi be wòaɖu ŋutilã dzi. |
| Nuwɔna kple nusɔsrɔ̃ | Nusɔsrɔ̃ ƒe kuxiwo, ŋkuɖoɖonudzi ƒe gbɔdzɔgbɔdzɔ, nuwɔna ƒe tɔtrɔ. |
| Tsitsi kple ŋgɔyiyi | Do kpo tsitsi si sɔ ɖe ƒexɔxɔ nu ɖeɖefia (e.g., nuƒoƒo ƒe megbetsitsi, azɔlizɔzɔ). |
| Sidzenuwo ƒe dɔwɔwɔ | Vivivi la, nusese, nukpɔkpɔ, kple nuƒoƒo ƒe ŋutete dzi ɖena kpɔtɔna. |
| Kuxi bubu siwo do ƒome kple lãmekawo | Yagbɔgbɔ sesẽ, aɖuɖɔtoe dzi ɖuɖu ƒe kuxiwo, alɔ̃madɔmadɔ. |
Le kpɔɖeŋu me, le ƒomevi aɖe si woyɔna be Canavan disease , me la, ɖevia ƒe kɔ ƒe lãmekawo tu, gake woƒe abɔwo kple afɔwo ya nɔa sesẽm. Ate ŋu adzɔ hã be woƒe ŋkuwo magate ŋu akpɔ nu o eye alɔ̃madɔmadɔ adze wo dzi.
Ɣeaɖewoɣi la, dzesiawo dzena le vidzidzi me. Gake le ƒomevi aɖe abe Refsum Dɔléle ene me la, dzesiawo dzena le ƒe 20 lɔƒo. Ɣeaɖewoɣi la, woate ŋu adze le esime woxɔ ƒe 50. Woate ŋu akpɔ nuwo abe ŋku ƒe ŋkume ƒe gbɔdzɔgbɔdzɔ, tosese, kple ʋeʋẽ ƒe bublu le esia me.
Esi wònye be dzesiawo to vovo kura ta la, ate ŋu asesẽ vie be woakpɔ dɔléle sia.
Nukatae dɔléle sia ƒomevi dzɔna? Nuka gbɔe wòtso?
Abe alesi míedzro eme va yi ene la, dɔléle siawo dometɔ geɖe nye domenyiŋusẽfianu ƒe nɔnɔmewo. Esia fia be domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ aɖe si tso vidadaa alo fofoa gbɔ, alo wo ame evea siaa gbɔe wotso.
Gake womenyia ƒomevi aɖewo ƒe dome o. Domenyiŋusẽfianu ƒe tɔtrɔ yeye aɖe gbɔe wotsona. Le kpɔɖeŋu me, edze abe womenyi Alexander ƒe dɔlélea ƒe dome o ene. Esia fia be ne domenyiŋusẽfianu si gblẽ la mele dzila eveawo dometɔ aɖeke si o hã la, ɖevi ate ŋu axɔe kokoko.
Eyata togbɔ be ʋumenugbagbeviɣidɔ ate ŋu anɔ ɖevi ɖeka si le ƒome ɖeka me hã la, ɖevi mamlɛawo ya maxɔe o.
Ne ʋumenugbagbeviɣidɔ le viwòwo dometɔ ɖeka ŋu eye nèle mɔ kpɔm na vi bubu la, ele vevie ŋutɔ be nàdi domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo . Esia akpe ɖe ŋuwò nàse esia kple afɔku si le vi si nàdzi le etsɔme me gɔme. Ðo dze kple wò ɖɔkta tso esia ŋu.
Aleke ɖɔktawo wɔa anya dɔléle sia pɛpɛpɛ?
Dɔléle sia didi nye nusi sesẽ vie elabena dzesiawo to vovo ŋutɔ. Eyata ele be ɖɔktawo nawɔ dodokpɔ ƒomevi vovovowo.
| Dodokpɔ ƒomevi | Kpuie ko la,... |
|---|---|
| Ʋu kple aɖuɖɔ dodokpɔ | De dzesi nusiwo mesɔ o le ŋutilãa ƒe atikewo ƒe dɔwɔwɔ me. |
| MRI-mɔ̃wo ƒe fotowo | Ahɔhɔ̃ kple lãmekawo ƒe nɔnɔmetata siwo me kɔ ŋutɔ, siwo me kɔ nyuie xɔxɔ be woatsɔ akpɔe ɖa be myelin-kploa gblẽ hã. |
| CT-dɔdamɔnu siwo wozãna | Nɔnɔmetatawo dodokpɔ bubu be woatsɔ ade dzesi tɔtrɔ siwo va le ahɔhɔ̃a ƒe wɔwɔme me. |
| Domenyiŋusẽfianu dodokpɔ | Domenyiŋusẽfianu ƒe gbɔdzɔgbɔdzɔ tɔxɛ si hea dɔlélea vɛ la nyanya. |
| Susu kple susu ƒe dodokpɔwo | Ðevia ƒe tamebubu, nusɔsrɔ̃, kple ŋkuɖoɖonudzi ŋutetewo dodokpɔ. |
Ðe atike aɖe li na esia?
Le nyateƒe me la, womekpɔ atike si ŋu kakaɖedzi le na ʋumenugbagbeviɣidɔ ƒomevi akpa gãtɔ haɖe o . Ke hã, esia mefia be naneke meli si woate ŋu awɔ o.
Le atikewɔwɔ me la, ɖɔktawo ƒe susu nɔa ɖevia ƒe dzesiwo dzi ɖuɖu kple eƒe agbenɔnɔ ƒe nyonyome dzi kpɔkpɔ ŋu alesi woate ŋui. Atikewɔwɔ nɔ te ɖe ʋumenugbagbeviɣidɔ si ƒomevi ɖevia le dzi.
Atikewɔwɔ siwo bɔe nye:
- Atikewo nana be woatsɔ aɖu dzesiawo dzi (e.g., be woatsɔ aɖu alɔ̃madɔmadɔ dzi).
- Atikewɔwɔ tɔxɛwo, abe kamedede, dɔdamɔnu, kple nuƒoƒo ƒe atikewɔwɔ ene , ate ŋu akpe ɖe ɖevi ƒe ʋuʋu, gbesiagbedɔwo wɔwɔ ƒe ŋutete, kple nuƒoƒo ŋu wòanyo ɖe edzi.
- Ðewohĩ ɖevi aɖewo ahiã kpekpeɖeŋu bubu le nusɔsrɔ̃ kple nunyiame ŋu .
Le go aɖewo me la, ƒumeŋuɖui dodo ate ŋu ana dɔlélea ƒe ŋgɔyiyi nagbɔdzɔ alo atɔ te. Dzɔdzɔmeŋutinunyalawo le numekuku wɔm tso atikewɔwɔ yeyewo abe domenyiŋusẽfianu ƒe dɔdamɔnu ene hã ŋu .
Le esiawo katã megbe la, nu vevitɔ kekeakee nye be nànɔ kadodo me kple wò ɖɔkta eye nàwɔ ɖe eƒe mɔfiamewo dzi.
Gbedasi si Wotsɔ Yi Aƒeme
- Leukodystrophy nye domenyiŋusẽfianu ƒe dɔléle siwo mebɔ o ƒe ƒuƒoƒo aɖe si gblẽa nu le lãmeka siwo tsyɔa ahɔhɔ̃a dzi (myelin sheath) ŋu.
- Ne ɖevi le lãmesẽ me le gɔmedzedzea me gɔ̃ hã la, dzesiawo ate ŋu adze vivivi le ɣeyiɣi aɖe megbe eye nɔnɔmea ate ŋu agblẽ ɖe edzi.
- Ne ède dzesii be viwòa ƒe azɔlizɔzɔ, nuƒoƒo, nukpɔkpɔ, alo nuwɔna ƒe gbegblẽ si womate ŋu aɖe o la, yi ɖɔkta gbɔ kaba.
- Togbɔ be womekpɔ atike aɖeke koŋ na ƒomevi akpa gãtɔ o hã la, atikewɔmɔnu aɖewo li siwo dzi woato akpɔ dzesiawo gbɔ ahana ɖevia ƒe agbenɔnɔ nanyo ɖe edzi.
- Ðo dze kple wò ɖɔkta gaglãa tso vɔvɔ̃, ɖikeke, alo nyabiase ɖesiaɖe si le mewò ŋu.
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