Ðe viwòa vuvu zi ɖekaa? Mina míaƒo nu tso Domenyinyi ƒe Angioedema (HAE) ŋu!

Ède dzesii kpɔ be viwò sue la vuvu zi ɖeka le woƒe ŋutilã ƒe akpa vovovowo, ɣeaɖewoɣi woƒe mo, asiwo, kple afɔwo? Alo ɖe viwòa le gbɔgbɔtsixe ƒe dzesiwo ɖem fia tsɔ kpe ɖe dɔgbo ƒe veve ŋua? Ðewohĩ nɔnɔme aɖe si mebɔ kura o gake wòle vevie ŋutɔ la le megbe na dzesi siawo. Egbea míele nu ƒom tso nɔnɔme mawo dometɔ ɖeka ŋu, si nye `Hereditary Angioedema (HAE). Mègatsi dzi o, míaƒo nu tso esia ŋu le mɔ bɔbɔe aɖe si gɔme nàte ŋu ase nu.

Nukae nye `Domenyiŋusẽ ƒe Ʋukawo ƒe Ʋuʋudedi (HAE)` sia?

Kpuie ko la, `Hereditary Angioedema` (si woyɔna hã be `HAE` kpuie) nye nɔnɔme si mebɔ kura o, si tso domenyiŋusẽfianu (si fia be woate ŋu atso dzidzimewo me). Enaa viwòa ƒe ŋutilã ƒe akpa vovovowo vuvu. Vuvu sia ate ŋu adzɔ le teƒe siwo woate ŋu akpɔ le gota, abe abɔwo, afɔwo, kple mo ene, alo ɣeaɖewoɣi le teƒe siwo míate ŋu akpɔ o, abe ɖevia ƒe yaƒoƒomɔ̃wo (gbɔgbɔ) alo nuɖuɖumeŋusẽ (dɔgbowo) ƒe lãkusiwo ene .

Fifia ɖewohĩ ànɔ nusitae vuvu sia dzɔna ŋu bum. Ʋuʋudedi sia dzɔna elabena ʋuka suesuesue siwo le ɖevia ƒe ŋutilã me, siwo woyɔna be ``Capillaries,'' la dona le wo me eye woƒoa ƒu ɖe lãkusi siwo ƒo xlãe me. Ne tsi sia ƒo ƒu la, ate ŋu axe mɔ na ʋu alo ʋukawo ƒe sisi to wo me. Esesẽ be woagblɔ ɣeyiɣi si me vuvu sia kple eƒe dzesi siwo do ƒome kplii (si woyɔna hã be ``HAE ƒe amedzidzedze'') adzɔ. Azɔ hã, ɣeaɖewoɣi la, esia ate ŋu atɔ ne avu sue aɖe, gake ɣeaɖewoɣi la, ate ŋu asẽ ale gbegbe be wòate ŋu ade ame ƒe agbe afɔku me.

Le atikewɔwɔ me la, nya "angio" (`Angio-`) fia ʋukawo. "Edema` (`-edema`) fia vuvu si tso tsi ƒe ƒoƒo ɖe lãmekawo me gbɔ. `Angioedema` ƒomevi geɖewo li, wo dometɔ ɖesiaɖe ƒe nusiwo gbɔ wòtsona to vovo. `HAE` ye nye esi mebɔ o wu le esiawo dome. Woyɔa esia be `congenital`, si fia be wodzia ame kple nɔnɔmea."

Ne wokpɔ HAE le viwòa ŋu la, àte ŋu aka ɖe nusi nàkpɔ mɔ na ŋu kura o. Gake esia nyanya ate ŋu akpe ɖe ŋuwò nàna wò susu naɖe dzi ɖi: Wole numekuku wɔm tso atike yeye siwo woazã na ƒe ɖesiaɖe me tɔwo siwo ŋu HAE le, siwo dome ɖeviwo hã le, ŋu. Wò ɖɔkta ate ŋu akpe ɖe ŋuwò nàse dɔdamɔnu siwo sɔ na viwòa kple nusiwo nàkpɔ mɔ na le etsɔme gɔme.

Ðe `HAE` ƒomevi aɖewo lia?

Ẽ, togbɔ be `HAE` nye `angioedema` ƒomevi aɖe hã la, ɖɔktawo ma `HAE` ɖe ƒomevi bubu geɖewo me:

• Ƒomevi I HAE: Esiae nye ƒomevi si bɔ wu . HAE dɔnɔwo katã ƒe 85% lɔƒoe ƒomevi sia le wo ŋu. Esia tsoa ɖevia ƒe ŋutilã ƒe protein tɔxɛ aɖe si woyɔna be C1-inhibitor (si wogayɔna be C1-INH) si mesɔ gbɔ o gbɔ. Ne C1-INH protein sia mesɔ gbɔ o la, dɔdzẽ kple vuvu ate ŋu adzɔ le ŋutilãa me. Wogayɔa esia be C1-INH ƒe anyimanɔmanɔ.
• HAE Ƒomevi II: Esiae nye ƒomevi evelia si bɔ wu. Le go sia me la, ɖevia ƒe ŋutilã wɔa C1-INH protein, gake mewɔa dɔ nyuie o.
• HAE si me C1-INH si sɔ le: Esiae nye ƒomevi si mebɔ boo o . Ðevia ƒe C1-INH protein ƒe agbɔsɔsɔ kple eƒe dɔwɔwɔ sɔ, gake nu bubuwo nana wòvuvuna.

Ne wotsɔ ƒomevi eve gbãtɔwo (HAE Ƒomevi I kple Ƒomevi II) ƒo ƒui la, edzea ame abe 1 le 50,000 ɖesiaɖe me dzi . Ema fia be le dukɔ abe United States ene me la, ame siwo ade 6,000 ye xɔa HAE sia ƒomevi. Numekulawo menya amesiwo tututu HAE le siwo ƒe C1-INH ƒe agbɔsɔsɔme sɔ o, gake wogblɔ be ƒã ŋutɔ.

Nukae nye HAE ƒe dzesiwo?

HAE ƒe dzesiwo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ. Dzesi siwo bɔ dometɔ aɖewoe nye:

• Vuvu siwo dzena le ɖevia ƒe ŋutilã ƒe akpa vovovowo, le kpɔɖeŋu me , abɔwo, afɔwo, ŋkume, nuyiwo, kple vidzinuwo .
• Dzesi siwo fia be dɔdzẽ le ɖevia ƒe nuɖuɖumeŋusẽ me (Gastrointestinal (GI) tract). Esia ate ŋu anye ʋuʋudedi, ʋuʋudedi, dɔgbo ƒe ʋuʋu, kple aɖuɖɔtoe .
• Dzesi siwo ɖevia ƒe nu, ŋɔti, kple yaƒoƒomɔ̃wo ƒe vuvu hena vɛ . Esiawo ate ŋu anye nu mimi sesẽ, aɖe si vuvu, gbeɖiɖi si le ʋuʋum ne èle gbɔgbɔm, kple gbe ƒe tɔtrɔ .

Vevietɔ wu: Ne gbɔgbɔ alo nu mimi le sesẽm na viwòa la, ƒo ka na xɔ si te ɖe ŋuwò si kpɔa nudzɔdzɔ kpatawo gbɔ alo kplɔ viwòa yi kɔdzi enumake. Yaƒoƒomɔ̃wo ƒe vuvu nye HAE ƒe kuxi sesẽ aɖe ŋutɔ, si ate ŋu awu ame. Ate ŋu awu ame ne womewɔ atike nɛ kaba o.

HAE mehea aɖuɖɔtoe vɛ o. Esiae nye vovototo vevitɔ si le HAE kple ʋuka me ʋu ƒe sisi ƒomevi bubuwo abe ʋuka me ʋu ƒe sisi kpata ene dome. Gake ame aɖewo siwo ŋu HAE le ate ŋu axɔ ʋuʋudedi dzĩ si meʋãna o hafi vuvua nadzɔ, si ate ŋu anye amedzidzedze si gbɔna ƒe dzesi.

Zi geɖe la, `HAE ƒe amedzidzedze` xɔa abe ŋkeke etɔ̃ va ɖo atɔ̃ ene . `Amedzidzedze` siawo vaa zi ɖeka eye wodzona, eyata esesẽna be woagblɔ ɣeyiɣi si tututu woadzɔ kple alesi woadzɔe.

Ɣekaɣie HAE ƒe dzesiwo dzea egɔme?

Zi geɖe la , HAE ƒe dzesiwo dzea egɔme tso ɖevime alo ƒewuiwo me . Woate ŋu asẽ ɖe edzi le sɔhɛmenɔɣi. Ðevi aɖewo ate ŋu adze dzesiwo kpɔkpɔ gɔme le woƒe ƒe 2 xɔxɔ me ke . Ame siwo ŋu HAE ƒomevi I alo II le la dometɔ siwo ade 50% dzea dzesiwo gɔme ne woxɔ ƒe 10. Le sɔhɛmenɔɣi megbe la, amedzidzedze siawo ate ŋu adze egɔme edziedzi eye woasẽ ɖe edzi. Amesiame kloe si ŋu HAE ƒomevi I alo II le la ƒe dzesiwo dzena le wo ŋu ne woxɔ ƒe 20.

Ame siwo ŋu HAE le siwo ƒe C1-INH ƒe agbɔsɔsɔme sɔ la te ŋu dzea egɔme emegbe vie – zi geɖe la, le woƒe ƒewuiwo ƒe nuwuwu alo woƒe tsitsi ƒe gɔmedzedze.

Nukae nye nusiwo hea HAE ƒe amedzidzedze vɛ?

Me me kɔ ɣesiaɣi be nuka tututue nye ``trigger`` si hea HAE ƒe amedzidzedze vɛ o. Ke hã, ``nu siwo ʋãa ame`` siwo wokpɔ la dometɔ aɖewoe nye:

• Nuveviwɔame le ŋutilã me alo afɔku sue aɖe: Tsɔe be viwòa dze anyi esime wònɔ fefem gbeɖeka.
• Aɖutikewɔwɔ: Nuwo abe aɖu ɖeɖe alo aɖu dede eme ene.
• Amekoko: Amekoko , eɖanye sue alo gã o.
• Nuteɖeamedzi alo seselelãme ƒe nyaƒoɖeamenu: Suku dodokpɔ alo dzre sue aɖe gɔ̃ hã kple xɔ̃wò ate ŋu akpɔ ŋusẽ ɖe edzi.
• Dɔlékuiwo ƒe dɔlékuiwo: Nuwo abe dzamezã kple dzamezã ene.
• Kamedefefe aɖewo: Le kpɔɖeŋu me, nuwo abe agbalẽŋɔŋlɔ atraɖii, ƒoƒo kple akplɔ, alo tomekuku kple aŋe ene.

Ðewohĩ màse nusi ʋãa viwòa wòdzea edzi gɔme enumake o. Gake ate ŋu aɖe vi ŋutɔ be nàŋlɔ ɣeyiɣi si me amedzidzedzea dzɔ kple nusi ɖevia nɔ wɔwɔm ɣemaɣi (le kpɔɖeŋu me, ne ɖevia dze dɔ alo nu te ɖe edzi) .

Nuka tututue gbɔ HAE tsona?

HAE ƒomevi I kple II siaa tso domenyiŋusẽfianu si woyɔna be SERPING1 ƒe tɔtrɔ (alo tɔtrɔ) gbɔ. Domenyiŋusẽfianu sia gblɔa ale si viwòa ƒe ŋutilã awɔ protein si woyɔna be C1-INH. Ne HAE ƒomevi I le viwò ŋu la, domenyiŋusẽfianu ƒe tɔtrɔ sia fia be woƒe ŋutilã mate ŋu awɔ C1-INH si sɔ o. Ne HAE ƒomevi II le viwò ŋu la, woƒe ŋutilã ate ŋu awɔ C1-INH, gake domenyiŋusẽfianu ƒe tɔtrɔ fia be protein la mewɔa dɔ nyuie o.

Numekulawo gale nusiwo gbɔ HAE tsona me kum kple C1-INH ƒe agbɔsɔsɔ si sɔ, gake wonya be domenyiŋusẽfianu siwo gbɔna ƒe tɔtrɔ ate ŋu gbɔe wòtso:

• `F12`
• `ANGPT1`
• `PLG`
• `KNG1`

Le go aɖewo me la, HAE ate ŋu anɔ anyi domenyiŋusẽfianu ƒe tɔtrɔ aɖeke si wokpɔ dze sii manɔmee. Ðɔktawo yɔa esia be "HAE-si womenya o."

Domenyiŋusẽfianu ƒe tɔtrɔ siwo hea HAE vɛ nana protein aɖewo siwo le ɖevia ƒe ŋutilã me mewɔa dɔ nyuie o. Protein siawo kpena ɖe tsiwo ŋu wotoa ɖevia ƒe ʋuka suewo (capillaries) me nyuie. Eyata ne protein siawo mewɔ dɔ nyuie o la, tsiwo dona eye woƒoa ƒu ɖe lãkusi siwo ƒo xlã ɖevia ƒe ʋukawo me. Emae nye nusi hea HAE ƒe dzesiwo vɛ.

Ðe HAE tso ƒomea mea?

Ẽ, wonyia HAE ƒe dome le autosomal dominant ƒe ɖoɖo nu. Kpuie ko la, domenyiŋusẽfianu ɖeka si mesɔ o koe wòhiã hafi wòate ŋu ahe dɔlélea vɛ. Ðevi ate ŋu anyi domenyiŋusẽfianu sia si mesɔ o ƒe dome tso wo dada alo wo fofo gbɔ.

Ame geɖe siwo ŋu HAE le la ƒe ƒome ŋutinya me dɔlélea le. Ke hã, ɣeaɖewoɣi la, ame ate ŋu axɔ domenyiŋusẽfianu ƒe tɔtrɔ le vome (si nye "de novo" alo "yeye" ƒe tɔtrɔ) ƒome ŋutinya aɖeke manɔmee.

Aleke ɖɔktawo wɔna kpɔa esia?

Ne viwòa ɖe HAE ƒe dzesiwo fia la, ɖɔkta awɔ nu siwo gbɔna:

• Ewɔa ŋutilãmedɔdada .
• Woabia nya wò tso viwòa ƒe dzesiwo kple eƒe atikewɔwɔ ŋutinya ŋu.
• Wowɔa ʋudodokpɔwo tsɔ léa ŋku ɖe ɖevia ƒe `C1-INH` ƒe agbɔsɔsɔ kple eƒe dɔwɔwɔ ŋu .
• Le go aɖewo me la, wowɔa domenyiŋusẽfianu dodokpɔ be woakpɔe ɖa be domenyiŋusẽfianu ƒe tɔtrɔ aɖewo li siwo hea HAE vɛ hã.

Nukawoe nye atike siwo wozãna na HAE?

Atike ƒomevi vevi eve li na amesiwo ŋu HAE le:

• Atike siwo wobia tso ame si: Esiawo nye atike siwo wonana ne HAE dze ame dzi ko. Le kpɔɖeŋu me, atikewo abe Berinert® alo Kalbitor® ene. Atike siawo nono kaba ne dzesiawo dze egɔme vɔ megbe ate ŋu aɖe amedzidzedzea ƒe sesẽ dzi akpɔtɔ eye wòaxe mɔ ɖe kuxi siwo ate ŋu ade ame ƒe agbe afɔku me nu.
• Atike siwo woatsɔ axe mɔ ɖe dɔlélea nu: Esiawo nye atike siwo ɖea afɔku si le amedzidzedze me dzi kpɔtɔna. Kpɔɖeŋu aɖewoe nye Cinryze®, Haegarda®, alo Takhzyro®. Ðɔkta ate ŋu aŋlɔ atike siawo na wò be nàzã le nusi wonya be eʋãa ame (abe mɔzɔzɔ yi aɖudɔdala gbɔ ene), alo be nàzãe ɣeyiɣi didi, le viwòa ƒe nuhiahiãwo nu.

Le atikeŋutinunyalawo ƒe nya nu la, atike siwo wobia tso ame si la le vevie eye woɖea ame ƒe agbe. Ne viwòa le mɔxexeɖedɔléle nu ƒe atikewo zãm gɔ̃ hã la, ele be atike siawo siwo wobia tso esi la nanɔ anyi ɣesiaɣi, le afisiafi (le aƒeme kple suku).

Ðɔktaa agblɔ atike siwo tututu viwòa hiã, ale si wòana wo, kple ɣeyiɣi si wòana wo. Aɖe atike siwo woate ŋu ana viwòa hã me le ƒe si wòxɔ nu. Kpɔ egbɔ be yewɔ ɖe mɔfiameawo dzi pɛpɛpɛ, eye nàkpɔ egbɔ be yebia nyawo ne nane li si ŋu mèka ɖe edzi o.

Aleke gbegbee nye lãme ahaya kaba le atikewɔwɔ vɔ megbe?

Ne viwòa zã atike siwo wobia tso esi la, eƒe lãme adze egɔme kaba vie le HAE ƒe amedzidzedze me. Ele be eƒe dzesiwo nanyo ɖe edzi le miniti 30 va ɖo gaƒoƒo eve me tso esime wòdze atikewɔwɔ gɔme. Wò ɖɔkta ana nyatakaka bubuwo wò tso alesi nàwɔ akpɔ atikewɔwɔa gbɔ le aƒeme kple nusi nàkpɔ mɔ na ŋu.

Aleke woawɔ akpɔ ɖevi si ŋu HAE le dzi?

Àte ŋu ase le ɖokuiwò me be yemate ŋu awɔ naneke o ne èkpɔ viwòa wòle fu kpem le HAE ƒe amedzidzedze me. Ke hã, nu geɖe li nàte ŋu awɔ na viwòa do ŋgɔ na amedzidzedze, le edzeɣi, kple emegbe:

• Na atike siwo nèdi la nanɔ asiwò ɣesiaɣi: Esiawo ate ŋu axɔ ame ɖe agbe. Ele be nàte ŋu ade dzesi ɣeyiɣi si me viwòa hiã atike siawo. Ele be nànya ale si nàna wo hã. Wò ɖɔkta agblɔ nya geɖe tso esia ŋu na wò.
• Mègabu HAE ƒe amedzidzedze abe nulɔdɔ ene o: Atikewo abe antihistamines kple epinephrine, siwo wonana zi geɖe na nulɔdɔwo, makpe ɖe HAE ƒe amedzidzedze ŋu o. Eyata mègana atike siawo viwòa o.
• Nya nu tso nusiwo ate ŋu ahe amedzidzedze vɛ ŋu: Nusiwo aʋã ame to vovo tso ame ɖeka gbɔ yi bubu gbɔ. Ŋlɔ nusiwo ate ŋu ahe viwòa ƒe amedzidzedze vɛ eye nàgblɔe na wò ɖɔkta. Dze agbagba nàna viwòa nate ɖa tso nu mawo siwo ʋãa ame la gbɔ alesi nàte ŋui. Ne màte ŋu aƒo asa na wo o la, bia wò ɖɔkta tso atike siwo woatsɔ axe mɔ ɖe enu ŋu.

Ɣekaɣie wòhiã be nàyi ɖɔkta gbɔ?

Kpɔ ɖɔkta le nɔnɔme siawo me:

• Ne èsusu be `Hereditary Angioedema` ƒe dzesiwo le viwò ŋu.
• Ne dzesi yeye siwo do ƒome kple HAE ƒe amedzidzedze do, alo ne dzesi siwo li fifia trɔ.
• Ne edze abe viwò ƒe atike mele dɔ wɔm abe alesi wokpɔ mɔe ene o.

Ɣekaɣie wòhiã be nàyi xɔ si me wowɔa dɔ kpata le?

Ne viwòa to nusiwo gbɔna dometɔ aɖe me , yi xɔ si me wokpɔa dɔnɔwo gbɔ kpata le enumake:

• Ne esesẽ be woami.
• Ne gbɔgbɔ sesẽ na wò.
• Ne èkpɔ dzesi aɖewo be aɖe alo ŋɔti vuvu (ne wona atike wò le amedzidzedze ƒe dzesi gbãtɔwo me gɔ̃ hã).

Esiawo ate ŋu anye abi si me afɔku le si gblẽa nu le viwòa ƒe yaƒoƒomɔ̃ ŋu ƒe dzesiwo. Mègahe atikewɔwɔ didi ɖe megbe o. Vuvu sia ate ŋu ade ame ƒe agbe afɔku me.

Nya kawoe wòle be nàbia ɖɔktaa?

Àte ŋu abia ɖɔktaa nya siawo abe esiawo ene:

• HAE ka ƒomevie le vinyea ŋu?
• Atikewɔwɔ kae nèkafu?
• Aleke mawɔ anya ne vinyea hiã atike siwo wobia tso esi?
• Ðe vinyea hiã atike siwo axe mɔ ɖe enua? Ne nenemae la, ke ɣekaɣie?
• Ðe dɔwɔna alo nɔnɔme aɖewo li siwo wòle be vinyea naƒo asa naa?
• Nya kae nàte ŋu agblɔ tso vinyea ƒe etsɔme (nukpɔsusu) ŋu?

Aleke míawɔ akpɔ mɔkpɔkpɔ na ɖevi si ŋu HAE le?

HAE nye nɔnɔme si nɔa ame ŋu le eƒe agbemeŋkekewo katã me. Gake atikewɔwɔ ate ŋu aɖe ŋusẽ si nɔnɔmea kpɔna ɖe ɖevi ƒe agbe dzi dzi akpɔtɔ. Ate ŋu aɖe dzesi siwo ate ŋu ade eƒe agbe afɔku me, abe ɖevia ƒe yaƒoƒomɔ̃wo ƒe vuvu ene, ƒe afɔkua hã dzi akpɔtɔ.

Ðe woate ŋu axe mɔ ɖe HAE nua?

Fifia la, womenya mɔ aɖeke si dzi woato axe mɔ ɖe HAE nu o. Ne HAE le wò alo wò zɔhɛ ŋu eye nèle agbagba dzem be yeadzi vi la, .Anyo be nàƒo nu kple domenyiŋusẽfianu ŋuti aɖaŋuɖola ale be nàte ŋu ase mɔnukpɔkpɔ si li be viwòa nanyi nɔnɔme sia ƒe dome la gɔme.

Gbedasi vevi aɖe na wò.

Ne èva nya be domenyiŋusẽfianu ƒe nɔnɔme aɖe abe HAE ene le viwòa ŋu la, àte ŋu ase seselelãme vovovowo: vɔvɔ̃, dzimaɖitsitsi, tɔtɔ, kple dziɖeleameƒo. Àte ŋu abu fɔ ɖokuiwò gɔ̃ hã. Gake nu vevitɔ kekeake si wòle be nànyae nye be menye wò vodadae dɔléle sia le o.

Domenyiŋusẽfianu ƒe tɔtrɔwo dzɔna ɣesiaɣi, zi geɖe la, susu aɖeke madzemadzee o. Màte ŋu aɖu domenyiŋusẽfianu siwo dome viwòa nyi la dzi o. Gake àte ŋu awɔ akpa vevi aɖe le viwòa dzikpɔkpɔ me – ɣeaɖewoɣi la, kakaɖedzi nana wo ko be "ànɔ nyuie."

Ate ŋu aɖe vi hã be nàge ɖe dɔnɔwo ƒe kpekpeɖeŋunahawo me. Nuƒoƒo na ɖevi siwo ŋu HAE le, kpakple ame tsitsi siwo ŋu dɔlélea le dzilawo ate ŋu aɖo aɖaŋu na wò eye nàɖo to. Ate ŋu aɖo ŋku edzi na wò hã be togbɔ be HAE mebɔ o hã la, menye wò ƒomea ɖeɖekoe o.

` Domenyinu Angioedema, HAE, vuvu, C1 mɔxenu, domenyiŋusẽfianu ƒe dɔlélewo, vuvu le ɖeviwo me, angioedema