Wetin na Pɔfyuria? Nɔ fred, lɛ wi tɔk bɔt am simpul wan.

Yu dɔn ɛva gɛt bɔn ɔ blista na yu skin we yu nɔ go ebul fɔ bia we yu de go na di san? Ɔ yu dɔn ɛva gɛt bad bad bɛlɛ pen wit nɔys ɛn vɔmit fɔ no rizin? Dɛn sayn ya difrɛn frɔm di sik dɛn we pipul dɛn kin gɛt . Sɔntɛm biɛn dis na wan sik we nɔ kin apin so ɔltɛm we bɔku pipul dɛn nɔ ivin yɛri bɔt. Dat na wan pan dɛn sik dɛn de we dɛn kɔl Pɔfyuria . Nɔ fred we yu yɛri dis nem. Tide, wi go tɔk bɔt dis rili simpul wan ɛn di we we yu go ɔndastand.

Fɔ tɔk am simpul wan, wetin na Pɔfyuria?

Fɔ ɔndastand dis, lɛ wi tink fɔs bɔt wan smɔl faktri na wi bɔdi. wan rili imכtant protin de we de gi wi rεd bכdi sεl dεm dεn rεd kכla εn de kכri כksijεn frכm di lכng to di rεst כf di bכdi. Dɛn kɔl am ‘heme’. Dis hεm de kכmכt na wi bכdi insay wan sεs stεp, lεk wan asembli layn. Jɔs lɛk aw dɛn kin gɛda motoka. pan εvri stεp, spεshal protin dεm we dεn k כl εnzym de wok.

Naw imajin, wetin go apin if wan step na dis prodakshɔn chen stɔp, ɔ if wan wokman (ɛnzym) nɔ wok fayn? Di nɛks step nɔ go ebul fɔ mek, ɛn di raw matirial dɛn kin gɛda, nɔto so? semweso, we difεkt apin na wan stεp we dεn de prodyuz hεm, di kεmikכl dεm we dεn prodyuz na di stεp we bin de bifo, we dεn kכl ‘pכfyurin' εn ‘pכfyrin prεkursכr', de bigin fכ kכlכm na di bכdi. Na di kεmεkal dεm we de kכmכt na di bכdi de mek di sayn dεm fכ pכfyuria. Dɛn tin ya kin pwɛl di nervɔs sistɛm ɔ di skin.

Wetin na di men kayn pɔrfyuria?

Dɛn kin sheb pɔrfyria to tu men kategori, bay aw di sayn dɛm de sho. We yu ɔndastand dis klasification, i go ɛp yu fɔ ɔndastand di sik fayn fayn wan.

sεvεra כda sכbtayp dεm de כnda dεn tu men tכp dεm ya. fכ egzampl, di tכp we dεn kכl `Acute Intermittent Porphyria (AIP)`, we de fכl כnda akyu pכfyuria, εn di tכp we dεn kכl `Porphyria Cutanea Tarda (PCT)`, we de fכl כnda di kכtan pכfyuria, nכ kin kכmכn.

Wetin na de trig wae kin mek yu gɛt sɔm kayn sik?

Pan ɔl we pɔfyuria na tin we pɔsin kin gɛt, nɔto ɔltɛm di sayn dɛn kin apin. Sɔm tin dɛn de wae kin mek yu gɛt sɔm kayn sik, ɔr "atak". Dɛn kɔl dɛn tin ya ‘trig’. Fɔ avɔyd dɛn tin ya na di bɛst we fɔ mɛn di sik.

• Sɔm mɛrɛsin dɛn: Sɔm mɛrɛsin dɛn we de mek pɔsin fil pen , pils fɔ kɔntrol pikin, anestetik, ɛn sɔm mɛrɛsin dɛn we de mek pɔsin gɛt sik kin mek dis apin. If yu gɛt pɔfyuria, yu fɔ rili tɔk to yu dɔktɔ bifo yu tek ɛni mɛrɛsin.
• Rɔm ɛn smok : Fɔ drink pasmak ɛn fɔ smok, mɔ di sem tɛm, na di men tin dɛn we kin mek pɔsin gɛt dis sik.
• Di it ɛn fast: Tin dɛn lɛk it we nɔ gɛt bɔku kab , strikt it we yu de it fɔ lɛf fɔ it bɔku bɔku it, ɛn we yu de fast kin mek yu gɛt di sik.
• Stret na yu maynd ɛn bɔdi: Tin dɛn lɛk fɔ wok pasmak, strɛs na yu maynd, ɛn ɔpreshɔn kin afɛkt am.
• Infεkshכn: Di sayn dεm kin apin bikɔs ɔf di strεs we ɛni ɔda sik ɔr infεkshɔn kin put pan di bɔdi.
• San layt: San layt na di men tin we kin mek pipul dɛn gɛt Cutaneous Porphyria.

Ustɛm yu fɔ go to dɔktɔ? Aw dɛn kin du di diagnosis?

Bikɔs dis sik nɔ kin bɔku ɛn di sayn dɛm kin tan lɛk ɔda kɔmɔn sik dɛm, sɔmtɛm i kin tek sɔm tɛm fɔ no if i gɛt am. I impɔtant fɔ go to dɔktɔ if yu gɛt ɛni wan pan dɛn sik ya, mɔ if dɛn kin apin ɔltɛm.

Na akyu pɔfyuria

If yu gɛt siriɔs pen na yu bɛlɛ wit sɔm sayn dɛm lɛk fɔ nɔs, vɔmit, kɔnstipɛshɔn, at rit we de go ɔp, ay blɔd prɛshɔn, mɔsul wik, kɔnfyushɔn, ɔ siz, i rili impɔtant fɔ go na ɔspitul wantɛm wantɛm, mɔ na Imejɛnsi Tritmɛnt Yunit (ETU), bikɔs dis sik kin mek yu layf de pan denja sɔntɛnde.

Insay Kutan Pɔfyuria

If yu skin gɛt blista, sos, inflamɛns, rɛd, ɔ skrach izi wan afta yu dɔn de na di san, go to dɔktɔ we de mɛn yu skin.

Di we aw dɛn kin no di sik:

1. Mɛdikal ɛgzamin: Fɔs, di dɔktɔ go chɛk yu, ɛn tek tɛm aks yu bɔt yu sik ɛn famili mɛdikal istri.

2. Labɔrɔtɔri tɛst: If dɛn tink se pɔfyuria de, dɛn go tɛst yu urine, blɔd, ɛn sɔntɛm yu stɔl sɛmpul fɔ di lɛvɛl dɛn we pɔfyurin de. If i siriɔs, di urine kin tɔn dak rɛd ɔ pepul kɔlɔ.

3. Jεnεtik Tεst: If dεn kכnfכm di sik bay labכtכri tεst, dεn kin du jεnεtik tεst fכ no di spεsifi k jεnεtik mכtεshכn we mek i kam.

Wetin na di tritmɛnt dɛn we yu kin gɛt?

Di tritmɛnt fɔ pɔfyuria kin difrɛn difrɛn wan bay di kayn sik ɛn aw i siriɔs. Pan ɔl we no mɛrɛsin nɔ de, dɛn kin kɔntrol di sayn dɛm ɛn liv nɔmal layf.

Tritmɛnt fɔ Akyu Pɔfyuria

We di sayn dɛn kin rili bad (akyu atak), dɛn kin nid fɔ go na ɔspitul ɛn tritmɛnt.

• Gi salin (IV fluid): Balans di bɔdi in sɔl ɛn wata.
• di hεm Injεkshכn: kכntrol di hεm dεfisεns na di bכdi εn ridyus di כmכnt כf di pכfyurin we de kכmכt.
• Gi glukכs: If yu gi shuga lεk dεkstroz insay yu bכdi כ bay mכt i de gi signal fכ di liva fכ ridyus di pכfyurin prodakshכn.
• Ɔda tritmɛnt dɛn: Dɛn kin tek mɛrɛsin fɔ mek pɔsin fil pen bad bad wan, ɛn mɛrɛsin fɔ mek pɔsin nɔ fil fayn ɛn fɔ vɔmit.
• Nyu drɔgs lɛk Givosiran (Givlaari): Fɔ pipul dɛm wae gɛt dis sik ɔltɛm, dɛn kin gi dis mɛrɛsin ɛvri mɔnt fɔ ridyus dɛn frɛkuɛns ɛn fɔ mek dɛn nɔr bɔku.

Tritmɛnt fɔ Cutaneous Porphyria

• Fɔ protɛkt yusɛf frɔm di san:Dis na di tin we impɔtant pas ɔl. Fɔ wɛr lɔng sliv, hat, ɛn fɔ put sanskrin wit ay levul protɛkshɔn, lɛk SPF 50+, impɔtant.
• Phlebotomy: Dis na fɔ pul smɔl blɔd (lɛk wan yunit) na di bɔdi ɛvri sɔm wik. Dis kin ɛp fɔ ridyus di ɛkstra ayɔn na di bɔdi ɛn i kin ɛp fɔ pul di pɔfyurin dɛn we dɔn gɛda na di liva.
• Mεdikeshכn dεm: If yu tek lכw dכz dεm lεk klorokwin כ haydroksiklorokin i kin mek dεn pul di pכfyurin dεm na di liva kwik kwik wan.