Bɔku tɛm yu pikin kin gɛt fiva fɔ natin? Lɛ wi lan bɔt dɛn SAID ya (Systemic Autoinflammatory Diseases)!

Sɔntɛnde yu smɔl pikin kin jɔs gɛt fiva we i nɔ gɛt kol ɔ ɛni ɔda sik? Yu tink se di fiva kin kam, i kin stɔp insay sɔm dez, dɔn i kin kam bak afta sɔm dez? Sɔm mama ɛn papa dɛn kin gɛt dɛn kayn ɛkspiriɛns dɛn de. So tide wi go tɔk bɔt dis kayn tin we kin mek pɔsin gɛt fiva ɔltɛm, we nɔ kin izi fɔ fɛn. Dɛn kɔl dis SAID (Systemic Autoinflammatory Diseases) . Trade, dɛn bin de kɔl dis `(Periodic Fever Syndromes)` ɔ `(Rɛkɔrɛnt Fiva Sindrom)`.

Wetin na dɛn SAID ya? Lɛ wi ɔndastand am simpul wan!

Fɔ tɔk am simpul wan, SAID na wan grup fɔ sik dɛn we kin kam bikɔs ɔf wan prɔblɛm we de mek wi bɔdi nɔ de wok fayn ɔ we de mek wi ebul fɔ kɔntrol di tin dɛn we wi bɔdi bɔn wit . If na so i bi, if wi nɔ gɛt ɛni infekshɔn na do (lɛk vayrɔs ɔ baktri), wi bɔdi kin jɔs mek inflamɛns. I tan lɛk se wi bɔdi in difens sistɛm de go insay ɔvadrayv.

Tink bɔt am, wi gɛt wan grup fɔ sojaman dɛn insay wi bɔdi (dat na di imyun sistɛm). Dɛn wok na fɔ fɛt di jem dɛm we de mek sik we dɛn kam insay.Bɔt na pɔsin we gɛt SAID in bɔdi, sɔmtɛm dɛn sojaman ya kin jɔs de agyu ɛn mek prɔblɛm insay di bɔdi.

Naw yu kin de wɔnda if dis na sɔntin lɛk `(autoimmune diseases)` (dat na, autoimmune diseases). fכ egzampl, `(rheumatoid arthritis)` כ `(lupus)` na di sem tin. Nɔ, dɛn tu tin ya difrɛn smɔl. Insay ɔtoimyun sik dɛm, wetin kin apin na dat di imyun sistɛm we wi bɔdi gɛt ɔ we de adap kin mistek atak in yon wɛlbɔdi sɛl dɛm. Bɔt insay SAID, di prɔblɛm de wit di innate immune system.

SAID dεm n כ kin bכku pas כtoimyun sik dεm. Bɔku pan dɛn na hereditary , we min se na wan jenɛtik mutation ɔ variant de mek dɛn.

SAID kin bigin we dɛn smɔl ɔ we dɛn smɔl . Bɔt nɔto so i kin bi ɔltɛm. Yu pikin kin gɛt episod ɔ atak fɔ fiva ɛn ɔda sayn dɛm. Yu pikin kin nɔ gɛt ɛni sik atɔl bitwin di atak dɛn. No mɛrɛsin nɔ de fɔ SAID. Bɔt sɔm tritmɛnt dɛn de we kin ɛp fɔ kɔntrol di sik dɛn bɔku tɛm.

Wetin na di men kayn SAID dɛm?

Naw, di wan dɛn we de stɔdi bɔt dis dɔn no lɛk 60 kayn SAID dɛn, ɛn dɛn de fɛn ɔda wan dɛn. Lɛ wi tek wan luk pan sɔm pan di kayn dɛn we pipul dɛn kin gɛt.

• Famili Mɛditarenian Fiva (FMF): Dis na di mɔs kɔmɔn pan di jɛnɛtik inhɛrit, fiva sindrom we kin kam bak. I kin mek di pikin in bɛlɛ, in chɛst, ɛn in jɔyn dɛn wam wit pen .
• Fiva we kin kam wan wan tɛm, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Dis kin bigin pan yɔŋ pikin dɛn bifo dɛn ol 4. Dis sik kin stɔp fɔ insɛf afta dɛn dɔn ol 10 ia.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Dis kin apin we dɛn smɔl, we dɛn de yɔŋ, ɛn sɔm tɛm dɛn kin ivin big.
• Mevalonate kinase deficiency (MKD): Fכs dεn bin de kכl dis haypa-IgD sεndrכm, dis kin bigin pan pikin dεm we nכ rich 1 ia yet.
• NLRP3-associated autoinflammatory diseases: Dɛn bin de kɔl dis bifo tɛm krayopyrin-associated periodic syndromes (CAPS). Tri ɔda sɔbtayp dɛn de insay dis.
• Still’s disease (AOSD) we kin bigin fɔ big pipul dɛn (AOSD): Dis na wan we we kin bigin fɔ big pipul dɛn we gɛt sistɛm juvenile idiopathic arthritis (JIA).
• NOD-2-associated granulomatous disease (Blau syndrome): Dis kin bigin bifo i ol 4. I kin mɔs afɛkt di pikin in skin, in yay, ɛn in jɔyn dɛn .

Wetin na di sayn dɛm fɔ SAID? Aw wi kin no dɛn?

Di sayn dɛm fɔ SAID kin bigin we dɛn smɔl. Di sayn we kin apin mɔ ɛn we kin sho klia wan na fiva we kin apin ɔltɛm ɔ we kin apin ɔltɛm . Pipul dɛn kin stil nɔr gɛt ɛni sayn bitwin di atak dɛm. Bɔt ɔda sayn dɛn kin de we spɛshal fɔ ɛni kayn SAID.

Lɛ wi luk di patikyula sayn dɛm fɔ sɔm kayn SAID wae wi dɔn tɔk bɔt ɔp:

• FMF: Dis kin mek di pikin in bɛlɛ, in chɛst, ɛn in jɔyn dɛn fil bad bad wan, wit inflamɛns . Sɔntɛnde, rash kin kam na di ɔnda leg ɔ ankles. Imajin, wan pikin gɛt bɛlɛ at ɛn fiva wantɛm wantɛm. We di mama ɛn papa de wɔri ɛn kɛr dɛn go na ɔspitul, dɛn kin tink se na apɛndiks. Bɔt afta dat, i kin stɔp ɛn kam bak afta sɔm dez.
• PFAPA: Dis kin mek yu gɛt sɔm sayn dɛn lɛk we yu trot de at, yu mɔt de at, ɛn di limf no dɛn we de na yu nɛk we swel .
• TRAPS: Dis kin mek yu kol, di mכsul dεm de pen na di chεst εn an , εn wan rεd rεshכn we de mek yu fil pen we de bigin pan di an εn leg dεm εn spre כlsay na di bכdi.
• MKD: Na dis wan tuYu kin fil bad wit kol, ed pen, bɛlɛ at, yu nɔr kin want fɔ it, ɛn yu kin gɛt sɔm kayn sik lɛk flu.
• NLRP3 sik dɛm: Dɛn kin mek yu gɛt sɔm sayn dɛm lɛk we yu gɛt skin rɔsh, ed we de at, we yu nɔ de fil fayn, yu jɔyn pen, ɛn yu yay we de rɛd (kɔnjɔktiviti) .
• AOSD: Dis na wan sik wae kin mek yu skin gɛt rash, jɔyn pen, ɛn mɔsul pen . Sɔm pipul dɛn kin gɛt bak dɛn trot we de at, dɛn bɛlɛ kin at, dɛn kin taya pasmak, ɛn dɛn bɔdi kin at.
• Blau syndrome: Dis kin mek yu pikin in an, in leg, ɔ in bɔdi gɛt lɛsin na in skin . I kin mek yu jɔyn dɛn pen ɛn yu yay pen bak .

Wetin mek dɛn SAID ya kin apin? Wetin na di tin dɛn we kin mek i apin?

Bɔku pan di SAID dɛn na sik dɛn we pɔsin kin gɛt we i kam pan jɛnɛtiks . dat min se, εvri sεndrכm de kכz bay wan vεryushכn (vεryכnt) insay wan spεsifi k jin.

Lɛ wi luk di jin dɛm we de inflɔws sɔm men kayn SAID dɛm:

• FMF: Dis de kכz fכ wan jin we dεn kכl `(MEFV jin)`. dis jin de instrכkt aw fכ mek wan protin we dεn kכl `(pyrin)`.
• PFAPA: Dɛn nɔ dɔn fɛn di rayt rizin fɔ dis yet .
• TRAPS: Dis de kכz fכ wan jin we dεn kכl `(TNFRSF1A jin)`. dis na wetin de gi instrכkshכn fכ mek wan protin we dεn kכl `(tכmכr nεkrכsis fכktכ rεsεptכr - TNFR)`.
• MKD: di rizin fכ dis na wan jin we dεn kכl `(MVK jin)`. dis de instrכkt di prodyushכn fכ wan protin we dεn kכl `(mevalonic kinase)`.
• NLRP3 sik dεm: Dis kin kכz fכ wan jin we dεn kכl `(NLRP3 jin)`. dis de gi instrכkshכn fכ mek wan protin we dεn kכl `(cryopyrin)`.
• AOSD: Dɛn nɔ dɔn fɛn di rayt rizin fɔ dis yet .
• Blau sεndrכm: Dis kin kכz fכ wan jin we dεn kכl `(NOD2 jin)`. dis de instrכkt di prodyushכn fכ wan protin we dεn kכl `(NOD2)`.

Dɛn SAID ya kin mek ɔda prɔblɛm dɛn?

Yɛs, dis kin mek prɔblɛm if dɛn nɔ trit am fayn . if inflameshn kכntinyu na di bכdi, i kin mek wan kכndyushכn we dεn kכl ``amyloidosis.`` dat min se wan kayn protin de dכn na wi kidni dεm. Dis kin mek di kidni dɛn pwɛl fɔ ɔltɛm . Na dat mek i impɔtant fɔ no di sik kwik kwik wan ɛn gɛt tritmɛnt.

Aw dɔktɔ kin no dis SAIDs kɔndishɔn?

Infakt, fɔ no dɛn ɔtoinflammatory sik ya kin tranga smɔl , bikɔs dɛn sik kin falamakata ɔda siriɔs tin dɛn lɛk lupus ɔ kansa lɛk limfoma. So, i impɔtant fɔ go to dɔktɔ we gɛt spɛshal trenin fɔ inflammatory diseases, we na rεumatɔlɔjis, fɔ no ɛn manej yu pikin in kɔndishɔn fayn fayn wan.

Yu pikin in rεumatɔlɔjis go yuz sɔm tin dεm fɔ no if i gɛt SAID. I go aks yu bɔt yu pikin in sik ɛn if ɛnibɔdi na yu famili gɛt dɛn sik ya (bayolojikal famili histri). Di dɔktɔ kin tink se yu pikin kin gɛt fiva sik we kin apin ɔltɛm if:

• If di pikin gɛt fiva ɔltɛm .
• If ɛnibɔdi na di famili gɛt histri fɔ gɛt fiva bɔku tɛm lɛk dis .
• If di pikin na wan grup we gɛt fiva lɛk dis wan wan tɛm .

Us tɛst dɛn de du fɔ dis?

Fɔ mek yu no se yu gɛt di sik, di dɔktɔ we de mɛn yu pikin kin tɛl yu fɔ du bɔku tɛst dɛn. Dɛn tin ya kin bi:

• Lab tεst: dεn kin yuz tεst lεk ``C-reactive protein (CRP)`` εn ``complete blood count (CBC)`` fכ chεk fכ inflameshn na di bכdi. Dɛn tɛst ya kin bi pɔsitiv we fiva "atak" ɛn dɛn kin kam bak to nɔmal we di "atak" dɔn.
• Yurin tεst: dεn kin du urine protin tεst fכ chεk fכ εksyכs protin na di urine. insay MKD, wan urine tεst we de luk pan כrganik asid dεm (asid dεm we gεt wan kabכn atכm) kin sho εlevεt lεvεl dεm fכ mεvalכnik asid.
• Jεnεtik tεst: Dis kin chεk fכ jεnεtik vεryכnt dεm. Bɔt sɔm pikin dɛn we gɛt SAID nɔ kin gɛt di jɛnɛtik we dɛn dɔn fɛn, we kin mek dɛn nɔ gɛt di tɛst rizɔlt.

Wetin na di tritmɛnt fɔ dɛn SAID ya?

Di tritmɛnt fɔ SAID kin difrɛn difrɛn wan bay di kayn ɛn aw di sik siriɔs . Nɔr mɛrɛsin nɔr de fɔ dɛn kayn sik ya. Bɔt bɔku tɛm yu kin kɔntrol yu pikin in sik wit mɛrɛsin . If yu pikin kin gɛt sɔm atak dɛn nɔmɔ insay wan ia, yu kin ebul fɔ gi am mɛrɛsin fɔ mɛn pen lɛk nonsteroidal anti-inflammatory drugs (NSAID) fɔ ɛp fɔ ridyus dɛn sik dɛn. Bɔt if di sik rili bad, yu dɔktɔ kin tɛl yu ɔda tritmɛnt dɛn.

Na sɔm pan dɛn:

• FMF: Dɛn kin yuz wan mɛrɛsin we dɛn kɔl `(colchicine)` fɔ kɔntrol dis. Dis kin mek di inflamɛns nɔ bɔku. If di pikin nɔ ebul fɔ tek kolchisin, dɛn kin tray wan `(biologic)` kayn mɛrɛsin we dɛn kɔl `(canakinumab)`.
• PFAPA: Dɛn kin gi stɛroyd (usually prednisone) fɔ shɔt di tɛm we PFAPA "atak." Insay sɔm pikin dɛn, cimetidine, we na wan mɛrɛsin we dɛn kin yuz fɔ mɛn ɔlsa na dɛn bɛlɛ, kin ɛp bak fɔ kɔntrol di sik dɛn we dɛn kin gɛt.
• TRAPS: Wan drɔg we dɛn kɔl `(canakinumab)` kin wok bɔku tɛm fɔ dis. כlso, dεn kin rεliv di simptom dεm wit anti-inflammatory mεdikeshכn dεm we dכkta gi, lεk `(glucocorticoids)`.
• MKD: .Canakinumab na fayn tritmɛnt bak fɔ MKD. Fɔ gi NSAID ɔ stɛroyd we yu de "atak" kin ɛp bak.
• NLRP3 sik dɛm: Dɛn kin yuz imyunomodulatɔ dɛm lɛk kanakinumab, rilonasɛpt, ɛn anakinra fayn fayn wan fɔ dis.
• AOSD: Dɛn kin yuz difrɛn kayn anti-inflammatory mɛrɛsin fɔ AOSD. Sɔm ɛgzampul dɛn na stɛroyd, di sik-modifying anti-rheumatic drugs (DMARD), ɛn bayolojikal.
• Blau sindrom: Dipen pan yu pikin in simptom dɛm, dɛn kin tray fɔ yuz imyunosuppressant, tumor necrosis factor (TNF) inhibitors, ɛn/ɔ topical eye medications.

Dɛn SAID kɔndishɔn ya na pɔrmanent? Ɔ dɛn go go fa?

Sɔm ɔtoinflammatory kɔndishɔn dɛn na layflɔng kɔndishɔn . Ɔda wan dɛn kin las fɔ sɔm ia ɛn dɛn kin go we dɛn de ol. Sɔm tin kin de fɔ ɔl yu layf, bɔt as tɛm de go, di frɛkuɛns ɛn di kayn atak kin go dɔŋ. Yu pikin in dɔktɔ kin gi yu patikyula infɔmeshɔn bɔt di sik we yu pikin gɛt.

Fɔ mɛn pikin we gɛt ɔtoinflammatory disease kin tranga. If yu gɛt SAID, yu kin yuz yu pasɔnal ɛkspiriɛns fɔ bɛtɛ sɔpɔt yu pikin in kia. Yu kin yuz di tin dɛn we yu dɔn gɛt bak fɔ ɛp yu pikin fɔ ajɔst to dis sik we i go gɛt fɔ ɔl in layf.

Di tin we impɔtant pas ɔl na fɔ fɛn tritmɛnt frɔm spɛshal pipul dɛn we gɛt ɛkspiriɛns ɛn we sabi bɔt SAID. Dɛn kayn dɔktɔ ya kin ɛp fɔ kɔntrol yu pikin in sik ɛn ɛp am fɔ gɛt di bɛst pikin tɛm.

Faynal Mɛsej fɔ Tek-Hom

So, a op se yu get som aidia abaut di SAID dem we wi tok abaut tide. Mɛmba se if yu pikin kin gɛt fiva bɔku tɛm we yu nɔ kin ɛksplen, yu fɔ tɔk to dɔktɔ bɔt am . Nɔ jɔs ignore am, tink se na nɔmal tin.

• SAID na wan grup fɔ sik dɛm wae kin mek pɔrsin gɛt fiva ɛn na prɔblɛm wit di imyun sistɛm kin kam.
• Bɔku tɛm, dɛn tin ya kin kɔmɔt frɔm dɛn jɛnɛtiks ɛn dɛn kin bigin we dɛn yɔŋ.
• Pan ɔl we no mɛrɛsin nɔ de fɔ dis fɔ ɔltɛm, tritmɛnt dɛn de we go ebul fɔ kɔntrol di sik dɛn.
• I impɔtant fɔ mek yu go fɛn di rayt diagnosis ɛn tritmɛnt frɔm spɛshal dɔktɔ (Rheumatologist).
• If dɛn bigin fɔ trit am kwik, dɛn kin mek dɛn nɔ gɛt prɔblɛm dɛn lɛk amyloidosis ɛn di pikin kin liv nɔmal layf.

If yu gɛt ɛni ɔda kwɛstyɔn bɔt dis, nɔ shek fɔ aks yu famili dɔktɔ ɔ dɔktɔ we de mɛn pikin dɛn.

` SAID dεm, Sεstεmik כtoinflammatory Disizin dεm, Frεkuεnt Fiva, Jεnεtik Disiz, Fiva na Pikin dεm, Imyun Sistem, Riumatכlכjist