Mina míasrɔ̃ nu geɖe tso Tuberous Sclerosis ŋu. Mègavɔ̃ o, na míaƒo nu!

Ɣeaɖewoɣi le míaƒe agbe me la, míesea dɔléle siwo womate ŋu asusu o eye wowɔa nuku vie la ŋu nya, alo? Enyo, dɔléle aɖe si ame geɖe mese nu tsoe o, gake wòle vevie ŋutɔ be woanyae nye Tuberous Sclerosis Complex (TSC) , alo Tuberous Sclerosis ko. Nusi dzɔna le esia mee nye be dɔdzẽ alo dɔdzẽ siwo menye kansa o, dona le wò ŋutilã ƒe akpa vovovowo, vevietɔ le teƒewo abe ahɔhɔ̃, ŋutigbalẽ, ayiku, dzi, kple lãkusi ene. Esia nye domenyiŋusẽfianu ƒe dɔléle. Gake mègatsi dzi o, míaƒo nu tso nusianu ŋu eme nakɔ eye wòanɔ bɔbɔe.

Nuka tututue nye Tuberous Sclerosis?

Kpuie ko la, tuberous sclerosis (TSC) nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o. Ate ŋu ana dɔdzẽ siwo menye kansa o nadzɔ ɖe wò ŋutilã ƒe akpa vovovowo, abe wò ahɔhɔ̃, ŋutigbalẽ, ayiku, dzi, ŋku, kple lãkusi ene. Ele vevie be míaɖo ŋku edzi be menye kansae dɔdzẽ siawo nye o . Esia fia be womenye dɔdzẽ siwo me afɔku le siwo kaka tso ŋutilã ƒe akpa ɖeka yi bubu me o. Gake dɔdzẽ siawo ate ŋu ahe lãmesẽkuxi vovovowo vɛ, le afisi wodzɔna le kple woƒe lolome nu.

Nɔnɔme sia mekpɔa ŋusẽ ɖe ame aɖewo dzi alea gbegbe o. Woate ŋu anɔ agbe si sɔ. Gake le ame aɖewo gome la, ate ŋu asẽ wu eye ate ŋu ahe kuxi vovovowo vɛ. Dɔléle sia va doa mo ɖa vivivi. Dzesi aɖewo ate ŋu adze egɔme tso ɖevime, gake bubuwo ate ŋu axɔ ƒe geɖe hafi woadze. Eyata amesi ŋu dɔléle sia le hiã na atikewɔlawo ƒe aɖaŋuɖoɖo kple ŋkuléle ɖe eŋu le eƒe agbemeŋkekewo katã me.

Ameka ŋue nɔnɔme sia gblẽa nu le wu? Aleke gbegbe wòbɔe?

Tuberous sclerosis (TSC) nye dɔléle si nɔa ame ŋu le vidzidzi me . Wokpɔa dɔlélea le ɖevi siwo ade afã ŋu hafi woxɔ abe ɣleti 7 ene. Gake ɣeaɖewoɣi la, dzesiawo medzena gaglãa nenema gbegbe o, eye ɖewohĩ womakpɔe adze sii o ƒe geɖe. Ne wokpɔ dɔlélea le ɖevime gɔ̃ hã la, dzesi yeyewo ate ŋu adze ne wova tsi.

Nɔnɔme sia ate ŋu adze ame sia ame dzi, metsɔ le ŋutsu alo nyɔnu nyenye, ameƒomevinyenye, alo to si me wòtso me o. Ema fia be ƒuƒoƒo aɖeke koŋ meli si ate ŋu adze edzi bɔbɔe o.

Bu alesi gbegbe esia mebɔ o ŋu kpɔ. Le nyateƒe me la, tuberous sclerosis (TSC) nye dɔléle si mebɔ kura o . Ame siwo ade 50,000 koe le United States siwo ŋu dɔléle sia le. Le xexeame katã la, ame siwo ade miliɔn ɖeka. Eyata àte ŋu akpɔ alesi gbegbe nɔnɔme sia mebɔ o la le susu me.

Aleke tuberous sclerosis (TSC) kpɔa ŋusẽ ɖe ŋutilãa dzii?

Nu vevitɔ si tuberous sclerosis (TSC) gblẽnae nye be lãmenugbagbevi siwo le ŋutilãa me tsina ɖekae zua ƒu suesuesuewo (tumors). Esia dzɔna zi geɖe le ahɔhɔ̃a me . Tsɔ kpe ɖe eŋu la, àte ŋu akpɔ tɔtrɔ le wò ŋutigbalẽ hã ŋu, vevietɔ ne ètsi ŋutɔ. Le nyateƒe me la, zi geɖe la, ŋutigbalẽ ƒe tɔtrɔ siawoe nyea dzesi gbãtɔ si fia be dɔlélea le ame aɖe ŋu.

Menye ema ɖeɖeko o, ke tuberous sclerosis (TSC) hã ate ŋu agblẽ nu le wò dzi kple ayiku ŋu. Ebɔ be ƒunukpeƒetetedɔ ƒomevi vovovowo dzɔna le ŋutinu siawo me. Mebɔ be ƒunukpeƒewo nadzɔ le teƒe bubuwo o.

Nukawoe nye dzesiawo? Aleke woɖea wo ɖokui fianae?

Tuberous sclerosis (TSC) ƒe dzesiwo ate ŋu ato vovo ŋutɔ le ŋutinu alo ŋutilã ƒe akpa si ŋu wògblẽ nu le nu. Woate ŋu ama dzesiawo ɖe hatsotso vevi etɔ̃ me:

• Dzesi siwo do ƒome kple ahɔhɔ̃
• Dzesi siwo do ƒome kple ŋutigbalẽ
• Tɔtrɔ bubu siwo va le ŋutilãa me le ememe

Azɔ mina míalé ŋku ɖe esiawo dometɔ ɖesiaɖe ŋu ɖe vovo.

Dzesi siwo do ƒome kple ahɔhɔ̃

Dzesi siwo do ƒome kple ahɔhɔ̃a dzɔna ne dɔdzẽ alo cortical tubers (hamartomas) va le ahɔhɔ̃a me le tuberous sclerosis (TSC) ta. Togbɔ be menye kansae dɔdzẽ siawo nye o hã la, woate ŋu agblẽ nu le ahɔhɔ̃a ŋu eye wòado kplamatse ahɔhɔ̃a ƒe dɔwɔwɔ. Kpɔɖeŋu aɖewoe nye:

• Subependymal giant cell astrocytoma (SEGA) : Esiawo nye dɔdzẽ siwo dzɔna ɖe ahɔhɔ̃a ƒe gowo dzi, vevietɔ le do sue siwo me tsi yɔ fũu (ventricles) siwo le ahɔhɔ̃a me la ƒe gowo dzi. Esiawo ate ŋu axe mɔ na ahɔhɔ̃mekawo ƒe sisi ɣeaɖewoɣi, si ana tsi naƒo ƒu ɖe ahɔhɔ̃a me kple eƒe golɔgoewo. Woyɔa nɔnɔme sia be tsi ƒe tagbɔdɔ .
• Cortical tubers (hamartomas) : Esiawo nye lãmenugbagbevi siwo menye kansa o ƒe ƒuƒoƒo. Lãmenugbagbevi siawo ƒe hatsotsowo dzɔna le ahɔhɔ̃a me, si te ɖe afisi wòle be woanɔ ŋu, gake le mɔ si me tɔtɔ le vie nu. Esia le alea elabena lãmenugbagbevi geɖe va dzɔ eye teƒe mesɔ gbɔ na wo be woatsi o.

Zi geɖe la, tuberous sclerosis (TSC) ate ŋu ahe nɔnɔme bubuwo vɛ, eye dzesi siwo do ƒome kplii hã le wo ŋu:

• Alɔ̃madɔmadɔ : Esia nye nusi bɔ ŋutɔ.
• Tsitsi ƒe megbetsitsi kple susu ƒe dɔmawɔmawɔ nyuie : Le kpɔɖeŋu me, ɖevi ate ŋu ahe ɖe megbe le nuƒoƒo, azɔlizɔzɔ, kple bubuawo me.Nusɔsrɔ̃ ƒe kuxiwo hã ate ŋu adzɔ.
• Nɔnɔme siwo do ƒome kple ahɔhɔ̃ ƒe tsitsi: Nɔnɔmewo abe autism spectrum disorder alo attention-deficit/hyperactivity disorder (ADHD) ene .

Togbɔ be alɔ̃madɔmadɔ kple tagbɔdɔléle bɔ ɖe tuberous sclerosis (TSC) me hã la, menye amesiame si ŋu dɔlélea le ye axɔe o. Ate ŋu adzɔ hã be ame ɖeɖeko naxɔ alɔ̃dɔdɔ tagbɔdɔ manɔmee.

Dzesi siwo do ƒome kple ŋutigbalẽ

Zi geɖe la, ŋutigbalẽ ƒe dzesiwoe nyea tuberous sclerosis (TSC) ƒe dzesi gbãtɔ . Eŋutinunyalawo gblɔ be amesiwo ŋu dɔlélea le ƒe 90% toa nusiwo gbɔna dometɔ ɖeka alo esi wu nenema me:

• Ash leaf spots : Esiawo nye melanin le ŋutigbalẽa me.Teƒe siwo amadede mesɔ gbɔ ɖo o (amadede si naa ŋutigbalẽ ƒe amadede nɔa eŋu). Teƒe siawo le abe aŋgba ɣiwo ene. Ɣeaɖewoɣi la, esesẽna be woakpɔ wo le amesiwo ƒe ŋutigbalẽ me kɔ la ŋu. Teƒe siawo klẽna le keklẽ tɔxɛ aɖe si woyɔna be ultraviolet (UV) te .
• Confetti marks : Esiawo le abe flitete ene. Gake togbɔ be ɖaƒoƒo siwo wozãna edziedzi la doa viviti wu ŋutigbalẽ si ƒo xlãe hã la, confetti-dzesi siawo ƒe amadede le keklẽm wu ŋutigbalẽ si ƒo xlãe. Esiawo ate ŋu adzɔ le woƒe ɖevime hã.
• Mo ƒe fibromas : Esiawo nye tsitsi siwo menye kansa o siwo dzɔna ɖe mo ƒe ŋutigbalẽ dzi. Ne wololo, alo ne wo dometɔ geɖe te ɖe wo nɔewo ŋu la, woate ŋu adze abe nu gã siwo woyɔna be "plaques" ene. Woate ŋu ado viviti wu ŋutigbalẽ si ƒo xlã wo.
• Asibidɛ kple afɔbidɛ ƒe ɖa : Esiawo sɔ kple mo ƒe lãkusi, gake woƒoa xlã asibidɛwo kple afɔbidɛwo ƒe asibidɛwo alo wo te. Zi geɖe la, wodzena le sɔhɛmenɔɣi eye woate ŋu atsi le woƒe agbemeŋkekewo katã me.
• Shagreen patches : Esiawo nye teƒe siwo ka siwo le ŋutigbalẽa ƒe akpa si le gota wu (epidermis) te. Zi geɖe la, wodzɔna le akɔta ƒe akpa si le anyime. Woɖi amititsetse alo weintsetse ƒe ŋutigbalẽ.

Ðo ŋku edzi be teƒe alo ʋuʋudedi siawo kpɔkpɔ le wò ŋutigbalẽ ŋu ko mefia kokoko be tuberous sclerosis le ŋuwò o. Ele vevie be ɖɔkta nado wò kpɔ.

Tɔtrɔ bubu siwo va le ŋutilãa me le ememe

Tuberous sclerosis (TSC) ƒe dɔdzẽ alo ƒunukpeƒetetedɔ ate ŋu adzɔ le ŋutilã ƒe akpa bubu geɖe. Tɔtrɔ bubuwo hã ate ŋu ava. Wo dometɔ aɖewoe nye:

• Nu ƒe tɔtrɔ : Fibromas (tumors) dona le nu me, eye aɖuawo ƒe dowo dona le aɖu ƒe ʋeʋẽ ƒe bu ta. Wokpɔa tɔtrɔ siawo le aɖuɖɔtoe me le amesiame kloe si ŋu TSC le gome. Aɖuawo ate ŋu ave eye ʋu nado le wo me le ƒunukpeƒewo ta. Dodo si le enamel la me hã ate ŋu ana dowo nado.
• Ayiku me ʋuʋudedi : Ne esiawo va le ayiku me la, woate ŋu ado kplamatse ayiku ƒe dɔwɔwɔ. Dzesiawo ate ŋu anye vevesese le akɔta alo axadzi, ʋu le aɖuɖɔ me, kple ayiku me kpewo. Ɣeaɖewoɣi la, ayiku ƒe dɔmawɔmawɔ nyuie kple ayiku me dɔdzẽ ƒomevi aɖe si woyɔna be ayiku me lãmenugbagbeviwo ƒe dɔdzẽ hã ate ŋu adzɔ.
• Tumors of the retina or optic nerves : Dɔdzẽ siawo ate ŋu ahe ŋku ƒe kuxiwo vɛ, gake esia mebɔ o.
• Dzitodzito me dɔdzẽ (dzitodzito me dɔdzẽ) : Esiawo nu sẽna wu le vidzĩwo me. Afɔkua dzi ɖena kpɔtɔna ne ame tsi. Dɔdzẽ gãwo ate ŋu axe mɔ na ʋu ƒe sisi to dzia me, gake esia hã mebɔ o.
• Aklã me ʋuʋudedi : Ne esiawo va nu sẽ la, woate ŋu ana gbɔgbɔ nasesẽ.

Nukae hea tuberous sclerosis (TSC) vɛ?

Tuberous sclerosis (TSC) nye domenyiŋusẽfianu ƒe dɔléle . Esia fia be tɔtrɔ (nu gbagbewo ƒe tɔtrɔ) le míaƒe DNA me gbɔe wòtsona . Lãmenugbagbevi geɖe siwo le míaƒe ŋutilã me ma eye lãmenugbagbevi yeyewo va ɖoa wo teƒe. Protein siwo tea dɔdzẽ nu lae kpɔa dɔwɔwɔ sia dzi.

Protein siawo kpɔa alesi lãmenugbagbeviwo mamãe kple lãmenugbagbeviwo ƒe lolome dzi. Bu nusi adzɔ ne protein siawo meda sɔ o ŋu kpɔ? Emegbe, lãmenugbagbevi siwo lolo akpa, siwo sɔ gbɔ akpa, dzea tsitsi gɔme. Ne afi aɖeke mele wo si woayi o la, wotsina le wo ɖokui si eye wozua dɔdzẽ. Emae nye nusi dzɔna le tuberous sclerosis me.

Ðe esia nye nusi dzɔ tso dzidzimewo mea?

Domenyiŋusẽfianu ƒe tɔtrɔ siwo hea tuberous sclerosis (TSC) vɛ ate ŋu adzɔ le mɔ eve nu:

• Ɣeaɖewoɣi : Esiawo nye vodada siwo dzɔna le vome le fufɔfɔ megbe teti (si fia be ne tsinyenye kple azi ƒo ƒu wɔ fugboe vɔ). Ele abe ŋɔŋlɔdzesiwo ƒe vodada aɖe le agbalẽ aɖe si wota me ene. Esiawo bɔ wu dɔléle siwo ƒe dome wonyi.
• Domenyinu : Tuberous sclerosis (TSC) dɔnɔ siwo ade akpa etɔ̃lia nyia dome tso dzila gbɔ.

Aleke wokpɔa yɔmekpe ƒe dɔléle (TSC)?

Eŋutinunyalawo ma dɔlélea ƒe dzesiwo kple dzesiwo ɖe hatsotso vevi eve me: nɔnɔme veviwo kple nu suesuesuewo . Be woakpɔe be tuberous sclerosis (TSC) le ŋuwò godoo la, ele be nɔnɔme vevi eve alo esi wu nenema nanɔ ŋuwò. Esi wònye be dzesiwo ate ŋu ado mo ɖa le ɣeyiɣi aɖe megbe ta la, ɖɔkta ate ŋu ade wò nɔnɔmea hatsotso me be "TSC si ate ŋu adzɔ" nenye be nɔnɔme vevi ɖeka alo nɔnɔme sue eve koe le ŋuwò.

Nu vevi siwo le eme

• Ne mede ɖeke o la, aŋɔ ƒe aŋgba ƒe teƒe etɔ̃ ya teti (ele be teƒe ɖesiaɖe nakeke milimeta 5 ya teti).
• Mo/fibroma eve ya teti alo fibroma plaque ɖeka ƒe amesinɔnɔ le mo/ta dzi.
• Asibidɛ alo afɔbidɛ ɖeka ya teti ƒe fibroma ƒe amesinɔnɔ.
• Shagreen-ti ɖeka ya teti ƒe amesinɔnɔ.
• Retina ƒe abi siwo wu ɖeka ƒe amesinɔnɔ.
• Lãmetsi aɖewo koŋ ƒe tsitsi le ahɔhɔ̃a me alo le edzi.
• Tsitsi ƒomevi tɔxɛ aɖe le dzia me.
• Dɔdzẽ ƒomevi aɖe koŋ si menye kansa o (dɔdzẽ nyui) si gblẽa nu le lãkusi, ayiku, alo ʋukawo ŋu ƒe amesinɔnɔ.

Nu suesuesue siwo le eme

• Confetti ŋutigbalẽ ƒe abi.
• Do ene alo esi wu nenema ƒe amesinɔnɔ le aɖuawo ƒe lãkusi me.
• Kpe eve alo esi wu nenema ƒe amesinɔnɔ le nu me.
• Retina ƒe lãkusiwo.
• Kuku siwo wu ɖeka ƒe amesinɔnɔ le ayikuwo me.
• Kpe siwo menye kansa o (tsitsi nyuiwo) le ŋutinu alo teƒe bubuwo.

Dodokpɔ siwo wowɔna tsɔ dia dɔlélewoe

Esi wònye be tuberous sclerosis (TSC) ate ŋu ahe dzesi kple ŋusẽkpɔɖeamedzi vovovowo vɛ ta la, dodokpɔ geɖe li woate ŋu awɔ. Dodokpɔ siawo ato vovo le wò dzesiwo nu. Wò ɖɔkta ate ŋu aɖe dodokpɔ siwo nèhiã kple nusita wole wo wɔm la me nyuie wu. Dodokpɔ siwo bɔ wu le tuberous sclerosis (TSC) ŋu dometɔ aɖewo le ete. Domenyiŋusẽfianu dodokpɔ nye afɔɖeɖe mamlɛtɔ si woatsɔ aɖo kpe TSC ƒe dɔléle aɖe dzi eye woade dzesi tɔtrɔ tɔxɛ si le TSC domenyiŋusẽfianu me.

Dodokpɔwo hena dzesi siwo do ƒome kple ahɔhɔ̃

Ne dzesi siwo do ƒome kple ahɔhɔ̃a le ŋuwò, vevietɔ ne alɔ̃madɔmadɔ le ŋuwò (alo ne kpeɖodziwo li be ɖewohĩ alɔ̃madɔmadɔ le ŋuwò) la, anɔ eme be woawɔ nɔnɔmetata siwo wotsɔ dia dɔlélewoe kple dodokpɔ bubuwo na wò. Scan kple dodokpɔ siawo dometɔ aɖewoe nye:

• CT scan (Kɔmpiuta dzi tomography - CT scan) .
• MRI scan (Magnetic resonance imaging - MRI ƒe fotoɖeɖe) .
• EEG (Electroencephalogram - EEG) (Esia doa ahɔhɔ̃a ƒe elektrikŋusẽ ƒe dɔwɔwɔ kpɔna)

Ne ɖɔkta aɖe susui be susu ƒe dɔmawɔmawɔ nyuie le ŋuwò la, ate ŋu aɖo aɖaŋu na wò be nàwɔ susu ƒe dodokpɔ tɔxɛ aɖewo. Dodokpɔ siawo kpena ɖe ame ŋu wòdea dzesi kuxi siwo le alesi wò ahɔhɔ̃ kple tamebubu ƒe dɔwɔwɔwo wɔa dɔe ŋu. Zi geɖe la, esiawo dometɔ aɖewoe nye kuxiwo alo dɔ siwo ku ɖe nuwo abe ŋkuɖoɖonudzi, ʋɔnudɔdrɔ̃, kple nyametsotsowɔwɔ ene ŋu.

Dodokpɔwo hena dzesi siwo do ƒome kple ŋutigbalẽ

Lãmedodokpɔ le vevie ŋutɔ le tuberous sclerosis (TSC) ƒe dɔléle didi me. Woate ŋu akpɔ nɔnɔme sia ƒe dzesi kple dzesi geɖe le ɖevimenɔɣi. Dodokpɔ aɖewo li siwo ate ŋu akpe ɖe ɖɔkta ŋu wòaɖo kpe edzi be TSC le ŋuwò. Wo dometɔ siwo bɔ wue nye:

• Wood’s lamp examination : Esia nye dodokpɔ si me womedea nu ame me o si zãa ultraviolet kekeli tsɔ kpɔae ɖa be aŋgba ƒe aŋgbawo ƒe teƒeteƒewo klẽna hã.
• Skin biopsy : Woɖea ŋutigbalẽ sue aɖe hedonɛ kpɔna.

Dodokpɔwo hena tɔtrɔ ememetɔ siwo le ŋutilãa me

Domenyiŋusẽfianu dodokpɔ nye dɔwɔnu vevi aɖe le TSC didi me. Dɔnɔ siwo le 75% va ɖo 95% ƒe domenyiŋusẽfianu ƒe tɔtrɔ le TSC1 alo TSC2 domenyiŋusẽfianuwo me. Ate ŋu adzɔ be dɔlélea nadze ame dzi ne nugbagbevi ƒe tɔtrɔ si woate ŋu ade dzesii manɔmee, gake esia mebɔ o.

Tɔtrɔ geɖe siwo do ƒome kple TSC dzea egɔme ne ame le tsitsim abe fugboe ene le vidzidɔa me. Ðɔktawo ate ŋu ade dzesi tɔtrɔ siawo ɣeaɖewoɣi le beléle na wo do ŋgɔ na vidzidzi edziedzi me, vevietɔ le ultrasound dodokpɔwo wɔwɔ me, alo ne wowɔ CT alo MRI scan le vidzidzi megbe kpuie.

Nukawoe nye atike siwo wozãna na tuberous sclerosis (TSC)?

Womate ŋu ada tuberous sclerosis (TSC) keŋkeŋ o . Gake la,Woate ŋu ada dɔléle akpa gãtɔ. Zi geɖe la, atikewɔwɔ ƒe tiatia nɔ te ɖe dzesi siwo ƒomevi le ŋuwò dzi. Dɔdamɔnu siwo wozãna wue nye:

• Atikewo : Atikewo ate ŋu akpe ɖe ame ŋu le TSC ƒe dzesi geɖe me. Atike aɖewo ate ŋu aɖu ahɔhɔ̃a ƒe dzesiwo abe alɔ̃madɔmadɔ ene dzi. Atike bubuwo ate ŋu ada dzesi siwo tso dɔdzẽ kple tsitsi le ŋutilã ƒe akpa vovovowo gbɔ.
• Amekoko : Le go aɖewo me la, ehiã be woawɔ dɔ na ame be woaɖe dɔdzẽ siwo ƒo ƒu ɖe ŋutilãa me ɖa.
• Dermatology treatments : Esiawo ɖea tsitsi siwo le ŋutigbalẽa dzi alo ete la ƒe akpa siwo wokpɔna (keŋeŋ alo woƒe akpa aɖe) ɖa.

Atikewo

Le wò dzesiwo nu la, wò ɖɔkta ate ŋu aŋlɔ atike bubuwo hã na wò. Gake zi geɖe la, nusiawo nɔa te ɖe nu geɖe dzi, abe wò dzesiwo, wò lãmesẽ ŋutinya, wò ŋutɔ wò nuhiahiãwo, kple nusiwo nèlɔ̃na ene.

Atike vovovowo ate ŋu akpe ɖe ame ŋu woada TSC ƒe dzesiwo. Atike aɖewo li siwo nana dɔdzẽ ƒe tsitsi dzi ɖena kpɔtɔna, wodzudzɔa wo, alo ɖea wo dzi kpɔtɔna. Atike bubu si bɔ enye esi xea mɔ na alɔ̃madɔmadɔ. Alɔ̃madɔmadɔ nye TSC ƒe dzesi siwo bɔ wu eye afɔku le wo me la dometɔ ɖeka.

Amekoko

Tsitsi siwo dzɔna kple TSC ate ŋu ahe kuxiwo vɛ ɣeaɖewoɣi, le afisi wòdzɔna le nu. Ɣeaɖewoɣi la, ehiãna be woawɔ dɔ na ame be woaɖe tsitsi siwo le kuxiwo hem vɛ na ŋutinu alo ŋutilã ƒe dɔwɔwɔ vovovowo ɖa. Wò ɖɔkta ate ŋu agblɔ amekoko kple atikewɔmɔnu bubu siwo li na wò ŋu nya geɖe na wò.

Ŋutilãŋutinunya ƒe atikewɔwɔwo

TSC ƒe dzesi geɖe dzena. Esia ate ŋu ana nàtsi dzimaɖi, ŋu nakpe wò, eye ŋu nakpe wò. Ŋutilãŋutinunyala ate ŋu akpe ɖe ŋuwò nàɖe ŋutigbalẽ ƒe tɔtrɔ siwo dona tso TSC me dzi akpɔtɔ alo aɖe wo ɖa. Togbɔ be zi geɖe la, dɔdamɔnu siawo nyea ɣeyiɣi kpui aɖe ko hã la, woate ŋu ana tɔtrɔ gã aɖe nanɔ wò susu ƒe lãmesẽ ŋu.

Lãmesẽŋutinunya ƒe atikewɔwɔ na ŋutigbalẽ ƒe dzesi siwo do ƒome kple TSC ƒe kpɔɖeŋu aɖewoe nye:

• Cryoablation ( lãmekawo tsɔtsrɔ̃ to vuvɔ sesẽ zazã me) .
• Laser ŋutigbalẽ ƒe gbugbɔgadzɔ (laser ŋutigbalẽ ƒe gbugbɔgadzɔ) .
• Dermabrasion alo dermaplaning (ŋutilãa ƒe akpa si woɖena ɖa) .
• Excision ( amekoko sue aɖe, si sɔ kple vidzidzi ƒe dzesi aɖe ɖeɖeɖa) .
• Ŋutilã ƒe lãkusiwo

Kuxiwo/dɔdamɔnu ƒe nugbegblẽ le ame ŋu

TSC-dɔdamɔnu ƒe nugbegblẽ le ame ŋu alo kuxi siwo ate ŋu ado tso eme la to vovo ŋutɔ, vevietɔ esi wònye be dzesi vovovowo kple atikewɔmɔnu vovovowo sɔ gbɔ ŋutɔ ta. Le susu sia ta la, wò ɖɔkta ate ŋu aɖe nugbegblẽ le ame ŋu alo kuxi siwo ate ŋu ado mo ɖa me na wò nyuie wu. Ate ŋu agblɔ ɣeyiɣi didi si wòaxɔ hafi ahaya tso atikewɔwɔ me hã na wò.

Ðe woate ŋu aɖe tuberous sclerosis (TSC) ƒe afɔkua dzi akpɔtɔ alo axe mɔ ɖe enua?

Le nyateƒe me la, mɔ aɖeke meli si dzi woato axe mɔ ɖe tuberous sclerosis (TSC) nu o . Ne TSC le wò ƒomea me (dzila alo nɔviwò) ŋu la, domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo ate ŋu akpe ɖe ŋuwò nàse afɔku si le viwò ƒe domenyinyi ƒe nɔnɔmea me kple ne afɔɖeɖe aɖewo li nàte ŋu aɖe atsɔ aɖe edzi akpɔtɔ gɔme. Zi geɖe la, eŋutinunyalawo kafua aɖaŋuɖoɖo sia.

Nukae nàte ŋu akpɔ mɔ na ne tuberous sclerosis (TSC) le ŋuwò?

Ame geɖe siwo ŋu TSC le hiã na nɔnɔmetatawo dodokpɔ edziedzi, vevietɔ MRI scan, abe zi ɖeka alo zi eve ene le ƒea me. Zi geɖe la, esia dzea egɔme tso ɖevimenɔɣi eye wòyia edzi vaseɖe esime woxɔ abe ƒe 21. Ame aɖewo hiãa fotoɖeɖe edziedzi le woƒe agbemeŋkekewo katã me, be woatsɔ alé ŋku ɖe tsitsi yeyewo ŋu alo be woalé ŋku ɖe tsitsi siwo li xoxo ŋu.

TSC ƒe ŋusẽkpɔɖeamedzi ate ŋu ato vovo ŋutɔ le eƒe sesẽ nu:

• Dɔléle siwo mesẽ o : Ðewohĩ ahiã be amesiwo ŋu dɔlélea mesẽ o nano atike alo axɔ atike edziedzi, gake nɔnɔmea mekpɔa ŋusẽ ɖe woƒe agbenɔnɔ dzi boo o. Zi geɖe la, ame siawo ate ŋu akpɔ mɔ be yewoanɔ agbe didi abe amesiwo si TSC mele o ene.
• Nusiwo sɔ gbɔ : Le nɔnɔme siwo sɔ me la, dzesiawo ate ŋu ahe tɔtɔ aɖewo vɛ. Gake ne wowɔ atike na wo eye wokpɔ wo gbɔ edziedzi la, woate ŋu aɖu ŋusẽkpɔɖeamedzi siawo dzi zi geɖe. Zi geɖe la, ame siawo ate ŋu akpɔ mɔ be yewoanɔ agbe si sɔ alo anɔ kpuie vie.
• Dɔléle sesẽwo : Susu ƒe dɔmawɔmawɔ nyuie, asrã sesẽ, alo kuxi bubuwo ate ŋu anɔ ame siawo ŋu. Ðewohĩ ame aɖewo mate ŋu anɔ agbe le wo ɖokui si o, alo ate ŋu abia be woakpɔ atikewɔla bibiwo gbɔ le woƒe agbemeŋkeke akpa gãtɔ me alo le woƒe agbemeŋkekewo katã me.

Ɣeyiɣi didi kae tuberous sclerosis (TSC) nɔa anyi?

Tuberous sclerosis (TSC) nye dɔléle si nɔa anyi ɖaa, si nɔa ame ƒe agbemeŋkekewo katã me .

Aleke malé be na ɖokuinye?

Ne wò ɖɔkta ŋlɔ atike na wò la, ele be nànoe abe alesi wogblɔe ene pɛpɛpɛ. Mègadzudzɔ atikeawo nono ne mèƒo nu kple wò ɖɔkta o. Ne èdzudzɔe zi ɖeka la, ate ŋu ana wò dzesiwo nagatrɔ alo agado gã ɖe edzi ƒe afɔkua nadzi ɖe edzi.

Mɔ bubu siwo dzi wòle be nàto akpɔ ɖokuiwò dzi ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Nu geɖe kpɔa ŋusẽ ɖe esia dzi. Wò ɖɔktae nye ame nyuitɔ kekeake si ana mɔfiame wò le nusi wòle be nàwɔ kple nusi nàte ŋu awɔ be nàkpɔ ɖokuiwò dzi ahakpɔ wò nɔnɔmea gbɔ ŋu.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne tuberous sclerosis (TSC) le ŋuwò la, anɔ eme be ahiã be nàyi ɖɔkta gbɔ edziedzi le wò agbemeŋkekewo katã me. Esia wɔnɛ be wò ɖɔkta te ŋu léa ŋku ɖe wò nɔnɔmea ŋu eye wòléa ŋku ɖe tɔtrɔ ɖesiaɖe ŋu. Esiawo evea siaa le vevie ŋutɔ be woaxe mɔ ɖe kuxi sesẽ siwo ate ŋu ado mo ɖa le TSC ŋu nu.

Ɣekaɣie wòle be mayi Dɔwɔƒe si Kpɔa Nudzɔdzɔ Kpatawo Gbɔ (ETU) ?

Le wò dzesiwo kple afisi wò alɔ̃madɔmadɔ le nu la, dzesi aɖewo li siwo fia be ehiã be woakpɔ egbɔ enumake. Esiawo dometɔ ɖeka si bɔ wue nye Status Epilepticus (SE) .

SE nye ne èxɔ alɔ̃madɔmadɔ si didi wu miniti atɔ̃, alo alɔ̃dɔdɔ zi eve alo esi wu nenema ɖe wo nɔewo yome eye ɣeyiɣi aɖeke meli be nàhaya o. SE nye atikewɔwɔ kpata si ate ŋu ade ame ƒe agbe afɔku me . Ne èle ame aɖe si le alɔ̃madɔmadɔ lém gbɔ eye dzesi siawo dometɔ aɖe le eŋu la, ele be nàƒo ka na 911 (alo wò nutoa me xexlẽdzesi kpata) enumake.

Dzesi bubu siwo hiã be woakpɔ egbɔ ate ŋu ato vovo. Wò ɖɔkta ate ŋu aɖe dzesi siwo ŋu wòle be nàlé ŋku ɖo kple nusi nàte ŋu awɔ ne èkpɔ wo alo kpɔ wo la me na wò nyuie wu.

Mlɔeba la, nu siwo dzi wòle be woaɖo ŋkui

Tuberous sclerosis complex (TSC), alo tuberous sclerosis, nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o si nana be tsitsi siwo menye kansa o la dona le ŋutilã ƒe akpa vovovowo, vevietɔ ahɔhɔ̃ alo ŋutigbalẽ.

Mega tsi dzi o. Esi wowɔ ŋgɔyiyi le egbegbe atikewɔwɔ kple nɔnɔmetatawo ɖeɖe ƒe mɔ̃ɖaŋununya me ta la, zi geɖe la, woate ŋu alé ŋku ɖe nɔnɔme sia ŋu ahadae le alesi wòhiãe nu le agbemeŋkekewo katã me. Ame geɖe siwo ŋu TSC le ate ŋu anɔ agbe didi le lãmesẽ me.

Ne nyabiase alo dzimaɖitsitsi bubu aɖewo gale asiwò ku ɖe esia ŋu la, kpɔ egbɔ be yeƒo nu kple wò ɖɔkta. Woawoe nye amesiwo ate ŋu aɖo aɖaŋu si de pɛpɛpɛ wu eye wòanyo wu na wò.

` Tuberous sclerosis, domenyiŋusẽfianu ƒe dɔlélewo, ahɔhɔ̃ me dɔdzẽ, ŋutigbalẽ ƒe ʋuʋudedi, alɔ̃madɔmadɔ, tsitsi ƒe megbetsitsi, atikewɔwɔ