Yu de gɛt inflamɛns insay yu bɔdi? Lɛ wi lan bɔt dis nyu sik we dɛn kɔl VEXAS Syndrome.

Sɔntɛnde yu kin fil lɛk se sɔntin we strenj de kɔmɔt insay yu bɔdi, lɛk inflamɛns? Sɔntɛnde yu kin gɛt fiva, yu jɔyn pen, yu gɛt prɔblɛm wit yu skin, ɛn ɔda tin dɛn? Pan ɔl we i kin tan lɛk se dɛn tin ya nɔ gɛt natin fɔ du wit dɛnsɛf, sɔntɛm di tin we kin mek ɔl dɛn tin ya kin apin na di sem sik we nɔ kin apin so ɔltɛm. Wan pan dɛn kayn tin ya na VEXAS Syndrome . Lɛ wi tɔk smɔl bɔt dis tide, bikɔs i rili impɔtant fɔ no bɔt dis.

Wetin na VEXAS Sindrom? Fɔ tɔk am simpul wan...

Fɔ tɔk am simpul wan, VEXAS Syndrome na wan sik we nɔ kin apin so ɔltɛm we di ɔtoimyun kin gɛt . Naw yu kin de wɔnda wetin na dis ɔtoimyun kɔndishɔn. Imajin, wi bɔdi gɛt wan imyun sistɛm. I tan lɛk ami we de protɛkt wi kɔntri. Di wok we dis sistɛm de du na fɔ protɛkt wi bay we i de fɛt di jem ɛn sik dɛn we de kɔmɔt na do. Bɔt sɔntɛnde, dis sem tin we de protɛkt wi, we na di imyun sistɛm, kin mistek bigin fɔ atak di wɛlbɔdi sɛl ɛn tisu dɛn na wi yon bɔdi . I tan lɛk se wi nɔ ebul fɔ no udat na wi yon ɛn udat na di ɛnimi. Na dat wi kin kɔl ɔtoimyun kɔndishɔn.

Na dat kin apin to pɔrsin wae gɛt VEXAS Syndrome. Di imyun sistɛm kin atak difrɛn pat dɛn na di bɔdi, ɛn dis kin mek di bɔdi inflamɛns ɛn swel . I tan lɛk se smɔl faya de apin ɔltɛm insay di bɔdi.

VEXAS Syndrome kin afɛkt dɛn pat ya na di bɔdi:

• To yu blɔd
• To di bon mɛrɔ
• To di blɔd vesel dɛn
• Fɔ yu skin
• Cartilage (especially na di yes ɛn nos) .
• To di jɔyn dɛn
• To di lɔng dɛn
• Fɔ di yay dɛn
• Di nem dɛn fɔ di tɛstikul dɛn na man dɛn

Imajin aw dis kin kam wit bɔku prɔblɛm we bɔku bɔku ples dɛn kin afɛkt wan tɛm. Di tin we kin mek pɔsin gɛt dis sik na we pɔsin kin chenj in jɛnɛtiks . Fɔ tɔk di kɔrɛkt tin, wan chenj na wan jin we dɛn kɔl UBA1 jin . dis UBA1 jin de prodyuz di E1 ubiquitin-aktivεt εnzym, כ E1 εnzym fכ sכt. di wok we dis εnzym de du na fכ klin di west prodak dεm lεk di protin dεm we nכ nid we de kכmכt insay wi sεl dεm εn εp fכ mek di sεl dεm we dεn dכn pwεl. I tan lɛk se sɔmbɔdi de pul di dɔti na wi os. bכt we yu gεt VEXAS Syndrome, di E1 εnzym we di UBA1 jin de prodyuz nכ de wok fayn. Wetin kin apin da tɛm de? Trash kin gɛda insay di sɛl dɛn.

De tin wae impɔtant pas ɔl na dat, if dɛn nɔr trit dis sik fayn, sɔmtɛm i kin mek pɔrsin in layf de pan denja.So, i impɔtant fɔ go to dɔktɔ if yu gɛt sɔm sayn dɛn. Dɔktɔ dɛn go gi yu di tritmɛnt we yu nid fɔ kɔntrol di sik dɛn we yu gɛt.

Wetin di nem VEXAS min?

Di nem VEXAS na wan kɔmbaynshɔn fɔ di fɔs lɛta dɛm fɔ sɔm wɔd dɛm wae de ɛp fɔ no dis sik. Lɛ wi si wetin dɛn bi:

• V - vakuol dεm: dis na rawnd, εmpti spεs dεm we de fכm insay abnכmal sεl dεm. yu kin si dεn vakuol dεm ya na di bon mכro sεl dεm fכ pipul dεm we gεt VEXAS Syndrome.
• E - E1 εnzym: lεk aw wi bin se bifo, dis na di E1 εnzym we nכ de wok fayn biכs fכ wan mכtεshכn na di UBA1 jin.
• X - X-linked: Yu no se na tu kromozom dεm de sho wi sεks. uman dεm gεt XX kromozom dεm εn man dεm gεt XY kromozom dεm. di mutated UBA1 jin we de mek VEXAS Syndrome de na di X kromozom.
• A - Autoinflammation: Dis na di mεdikal nem fכ inflameshn we de apin we di imyun sistεm atak in כwn bכdi.
• S - Somatic: di jεnεtik mכtεshכn we de mek VEXAS Syndrome na wan kayn mכtεshכn we dεn kכl "somatic." Dis min se i kin apin randomly ɛn nɔto frɔm mama ɛn papa dɛn kin gɛt am . Dis min se yu nɔ fɔ wɔri se yu pikin dɛn go gɛt am jɔs bikɔs yu bin gɛt am.

Yu ɔndastand aw di nem VEXAS kam? Ɛni wan pan dɛn lɛta ya de tɛl wan impɔtant tin bɔt dis sik.

Aw kɔmɔn tin na VEXAS Syndrome?

Dis na rili wan sik we nɔ kin apin so ɔltɛm . Masta sabi bukman dɛn se ivin na kɔntri lɛk Amɛrika, dis sik kin ambɔg lɛk wan pan ɛvri 13,000 pipul dɛn. Pan ɔl we di statystik dɛn na Sri Lanka nɔ kɔrɛkt, i klia se dis na tin we nɔ kin apin so ɔltɛm.

Wetin na di sayn dɛm wae de sho se yu gɛt VEXAS Syndrome?

Di men sayn fɔ dis sik na inflamɛns . So, di ɔda sayn dɛm de dipen pan usay na di bɔdi di inflamɛns de apin. Si if yu gɛt ɛni wan pan dɛn sayn ya:

• Bɔku tɛm, a kin gɛt fiva.
• Di ɔksijɛn we de na di blɔd we nɔ bɔku (haypoxemia) .
• Difrɛn difrɛn skin rash ɛn ɛkzema.
• Di bɔdi we de swel.
• Pen na di jɔyn dɛn.
• Kɔf.
• I nɔ izi fɔ blo (dyspnea).
• Di yay dɛn we de rɛd.
• Ed de at.
• di tεstikul dεm we de swεla na man dεm (orchitis).

If wan ɔ mɔ pan dɛn sayn ya nɔ de igen, i go fayn fɔ mek yu go to dɔktɔ pas fɔ tek am se na kɔmɔn sik.

Wetin mek VEXAS Syndrome kin apin? Wetin na di kɔz?

as wi bin dכn tכk bכt, di mεn kכz fכ dis na di jεnεtik mכtεshכn na di UBA1 jin . jεnεtik mכtεshכn na chenj dεm na wi DNA. DNA sikyud. we di sεl dεm divayd, dat na we di sεl dεm mek kכpi fכ dεn sεf, sכmtεm pat pan dis DNA sikεns kin de na di rכng ples, i nכ kכmplit, כ i kin dεm. Na da tɛm de di sayn dɛm fɔ di jenɛtik kɔndishɔn kin apia.

wetin kin apin to pכsin we gεt VEXAS Syndrome na dat di UBA1 jin nכ de wok fayn, εn di E1 εnzym nכ de prodyuz lεk aw i fכ prodyuz. nכmal wan, di E1 εnzym lεk "klin" insay wi sεl dεm. Di wok we i de du na fɔ klin dɔti, lɛk ol ɛn pwɛl protin dɛn we de insay di sɛl dɛn. Bɔt we yu gɛt VEXAS Sindrom, dis "klin" tim nɔ de wok fayn. Wetin kin apin da tɛm de? di protin dεm we dεn dכn pwεl εn di dɔti dεm kin gɛda insay di sεl dεm. We wi imyun sistɛm si dis dɔti we dɔn gɛda, i kin tink se infɛkshɔn ɔ trɛt de de. Bɔt bikɔs rili no infɛkshɔn nɔ de de, di imyun sistɛm kin atak di tisu we gɛt wɛlbɔdi. Na dat kin mek pɔsin gɛt inflamɛns. Tink bɔt am lɛk di dɔti trak we nɔ de klin di os ɛn di dɔti we de gɛda.

Udat de pan big risk fɔ gɛt VEXAS Syndrome?

Stɔdi dɔn sho se man dɛn kin gɛt dis sik. I kin pasmak bak pan pipul dɛm wae dɔn pas 50. Dis min se di chenj dɛm wae kin kam wit dɛn jɛnɛtiks wae kin kam wit di ej kin ple wan pat.

Wetin na di prɔblɛm dɛn we kin apin we pɔsin gɛt VEXAS Syndrome?

If di inflamɛns we VEXAS Syndrome kin mek kin afɛkt yu bon mɛrɔ , i kin mek yu gɛt wan sik we dɛn kɔl bon mɛrɔ we nɔ de wok fayn. Dis kin mek pɔsin in layf de pan denja.

Dipen pan usay di inflamɛns de apin, pɔrsin wae gɛt VEXAS Syndrome kin gɛt ɔda wɛl bɔdi prɔblɛm, lɛk:

• Lukimiya ( wan kayn kansa na blɔd) .
• Mayokarditis (inflameshɔn na di at mɔsul) .
• Anemia we nɔ gɛt bɛtɛ blɔd
• Dɛmatitis ( we de mek yu skin inflamɛns) .
• Chondritis (inflameshɔn na di katilej) .
• Vasculitis (we de mek di blɔd vesel dɛn wam) .
• Atraytis ( we de mek yu jɔyn dɛn wam) .
• Blɔd we de klɔt na di dip vein (Dip Vein Thrombosis - DVT) .
• Kolaytis (inflameshɔn na di big intestinal) .

Luk aw siriɔs tin kin kam wit dis. Na dat mek i impɔtant fɔ no am kwik kwik wan ɛn fɔ trit am.

Aw dɔktɔ dɛn kin no se yu gɛt VEXAS Syndrome?

Dɔktɔ go no se yu gɛt VEXAS Syndrome bay we i go chɛk yu bɔdi ɛn du tɛst fɔ yu jenɛtik . I go tek tɛm luk bak pan yu sik dɛn ɛn aks yu bɔt aw lɔng yu dɔn gɛt dɛn sik ya.

Bɔt di wangren we fɔ no fɔ tru if yu gɛt VEXAS Syndrome na bay we yu de tɛst yu jɛnɛtiks. Insay dis, yu dɔktɔ go tek yu blɔd, skin, ia, ɔ ɔda tisu ɛn sɛn am na lɛb. di tεknishכn dεm we de de go tεst yu DNA fכ si if yu gεt di mכtεshכn na di UBA1 jin we de mek VEXAS Syndrome.

Wetin na di tritmɛnt dɛm fɔ VEXAS Syndrome?

Di men tritmɛnt dɛm wae de naw fɔ dis sik na:

• Kɔtikosterɔyd dɛn kin ridyus di inflamɛns.
• Di tin dɛn we de mek yu bɔdi nɔ ebul fɔ fɛt di sik kin mek yu imyun sistɛm slo.
• If yu bon mɛrɔ de sho sayn dɛn fɔ se i nɔ wok, yu go nid fɔ transplant yu bon mɛro . we dεn transplant bon mכro kin ridyus di siriכs fכ sכm כtoimyun kכndyushכn dεm bak.

Dɛn kin sɛn yu bak to dɔktɔ we de mɛn yu bɔdi , we na dɔktɔ we spɛshal pan jɔyn ɛn ɔtoimyun sik dɛn, we go gi yu di kɔrɛkt gayd fɔ trit dɛn sik ya.

Yu tink se dɛn kin ebul fɔ avɔyd VEXAS Syndrome?

Bɔt i sɔri fɔ no se naw, no we nɔ de fɔ mek dɛn nɔ gɛt dis sik . di mכtayshכn na di UBA1 jin de apin randomly, witout eni kכz we dεn no. So wi nɔ go ebul fɔ stɔp am bifo tɛm.

Wetin na di layf we pɔsin we gɛt VEXAS Syndrome kin liv?

Dis na tin we rili sɛnsitiv. Di tru tin na dat, ɔlman difrɛn, ɛn di we aw VEXAS Syndrome kin afɛkt yu bɔdi kin difrɛn frɔm wan pɔsin to ɔda pɔsin. Bɔt, lɛk aw wi bin dɔn tɔk bifo tɛm, dis sik kin mek pɔsin in layf de pan denja if dɛn nɔ trit am. So tɔk opin wan wit yu dɔktɔ bɔt wetin yu go ebul fɔ ɛkspɛkt ɛn us tritmɛnt we go fayn fɔ yu. I kin ɛp yu fɔ kɔntrol yu sik dɛn ɛn rifer yu to mɛntɛl hεlth pɔrsin ɛn ɔda sɔpɔt savis dɛm if nid de.

Ustɛm a fɔ go to dɔktɔ?

If yu gɛt ɛni sayn fɔ VEXAS Syndrome, lɛk fiva, skin rash, ɔr i nɔ izi fɔ blo, mek shɔ se yu go to dɔktɔ. Bikɔs VEXAS Syndrome kin mek yu gɛt difrɛn kayn sayn dɛm wae kin tan lɛk se nɔr gɛt ɛnitin fɔ du wit am, sɔmtɛm i kin at fɔ no fɔs. Bɔt, lisin to yu bɔdi, ɛn if yu gɛt sɔm sayn dɛm wae yu nɔr ɔndastand ɔr wae nɔr gɛt ɛnitin fɔ du wit yu ɔda wɛl bɔdi, tɔk to dɔktɔ bɔt dɛm.

If dɛn dɔn ɔlrɛdi no se yu gɛt VEXAS Syndrome, ɛn yu fil lɛk se yu de gɛt nyu sik ɔr de sik wae yu dɔn gɛt de wɔs, go to dɔktɔ kwik kwik wan.

Sɔntin yu nɔ plan! If yu gɛt fiva we pas 103°F (39.5°C) fɔ pas tu awa pan ɔl we yu de trit yu na os, go na imejensi rum. If yu gɛt prɔblɛm fɔ blo, go na ɔspitul wantɛm wantɛm ɔ kɔl 911 (ɔ yu lokal imejensi nɔmba).

Us kwɛstyɔn dɛn a fɔ aks mi dɔktɔ?

We yu go si dɔktɔ, rɛdi fɔ aks kwɛstyɔn dɛn lɛk dɛn wan ya:

• A gɛt VEXAS Syndrome, ɔ na ɔda sik?
• A go nid fɔ du tɛst fɔ no if a gɛt jɛnɛtiks?
• Us kayn tritmɛnt a nid?
• Us sayn ɔ chenj dɛn a fɔ luk fɔ?
• VEXAS Syndrome na trɛt fɔ mi layf?

Dɛn kwɛstyɔn ya go bi fayn tin fɔ mek yu bigin fɔ tɔk to yu dɔktɔ.

Fɔ dɔn, fɔ dɔn, mɛmba dis (Take-Home Message) .

VEXAS Syndrome na wan sik we nɔ kin apin ɔltɛm we di ɔtoimyun sik kin kam bikɔs ɔf wan patikyula jin muteshon. I kin mek yu bɔdi inflamɛns ɛn i kin mek yu layf de pan denja if yu nɔ trit yu. Bɔt nɔ giv ɔp fɔ op . Yu dɔktɔ kin ɛp yu fɔ fɛn di rayt tritmɛnt fɔ kɔntrol yu sik dɛn.

If yu gɛt ɛni sayn fɔ VEXAS Syndrome, go to dɔktɔ. Trust yusɛf, lisin to yu bɔdi. Nɔr ignore de symptom dɛm lɛk fiva, rash, ɛn tranga fɔ blo. If yu no di sik kwik kwik wan ɛn gɛt di rayt tritmɛnt, yu go ebul fɔ liv fayn layf.

` VEXAS Sindrom, Ɔtoimyun Sik, Inflameshɔn, Jɛnɛtik Myuteshɔn, UBA1 Jin, Bɔn Mɛro