Yu tink se ɛnibɔdi na yu famili, mɔ pikin, gɛt wan sik we dɛn bɔn wit ɔmon? Yu kin no bɔt prɔblɛm dɛn lɛk we uman dɛn nɔ kin gro fayn, we uman kin bɔn kwik kwik wan, ɛn we uman dɛn nɔ kin gɛt mɔnt. wan jεnεtik kכndyushכn we nכ kin kכz dεn tin ya dεn kכl am ‘Congenital Adrenal Hyperplasia’. Wi go kol am CAH fo shot. Tide, wi go tɔk smɔl bɔt dis ɛn wan nyu tritmɛnt we dɔn kam fɔ am.
Fɔ tɔk am simpul wan, wetin na CAH?
CAH na wan jenɛtik kɔndishɔn we de mek di adrenal gland dɛn, we na tu smɔl gland dɛn we de ɔp di kidni dɛn, nɔ de mek kɔtisol, we na wan ɔmon we impɔtant fɔ wi layf.
Tink bɔt wi bɔdi lɛk faktri. we wan pan di prכdak dεm (kכtisol) na dis fכktri rכn lכw, di kכntrol sεnta na di bren (pituitary gland) de vεst εn i de sεnd signal (ACTH) to di adrenal gland dεm fכ wok tranga wan. biכs fכ dis pasmak stimulashכn, pan כl we di kכtisol prodakshכn nכ de inkrεs, insted i de stat fכ prodyuz mכr man כmon dεm we dεn kכ l ‘androgens’ .
Dis ɔmon we nɔ balans kin mek bɔku prɔblɛm dɛn:
- uman pikin dεm: We dεn bכn dεm, di εksternal jεnitalias kin tan lεk di wan dεm fכ man pikin.
- Fɔ ɔl tu di pati dɛm: prɔblɛm lɛk we pɔsin nɔ de gro fayn, we i de bɔn kwik kwik wan, ɛn we i nɔ ebul fɔ bɔn pikin kin apin leta na layf.
Apat frɔm dat, sɔm pipul dɛn we gɛt CAH kin dɔn ridyus bak fɔ mek ɔda impɔtant ɔmon we dɛn kɔl aldosterone. If dis ɔmon nɔ de, CAH kin rili bad, we kin mek pɔsin shɔk, kɔma, ɔ ivin day.
70 ia ol tritmɛnt ɛn nyu op
Fɔ lɔng tɛm, di wangren tritmɛnt fɔ CAH na fɔ riples di ɔmon kɔtisol we nɔ bɔku na di bɔdi wit bɔku stɛroyd , lɛk haydrokɔtizɔn. Bɔt if yu tek dɛn bɔku bɔku stɛroyd ya fɔ lɔng tɛm, dat kin mek yu gɛt bɔku bad bad tin dɛn. Di wan dɛn we kin bɔku pan dɛn na we pikin dɛn kin gɛt bɔku bɔku bɔdi, dɛn kin gɛt ay blɔd prɛshɔn, dɛn bon dɛn kin wik, ɛn dɛn nɔ kin gro fayn.
Bɔt naw gud nyus de! Afta 70 ia, di FDA dɔn gri fɔ di fɔs ɛn di wan ol mɛrɛsin we dɛn mek spɛshal fɔ CAH, we na crinecerfont (brand nem: Crenessity).
Aw dis nyu drɔg (Crinecerfont) de wok?
Dis na di tin we impɔtant pas ɔl. Dis nyu mɛrɛsin nɔ tan lɛk di ol tritmɛnt, i de wok difrɛn we.
‘Crinesurfont’ nɔ de ad ɔmon frɔm ɔdasay. Bifo dat, dis drɔg de go dairekt to di kɔntrol sɛnta na di bren (di pituitary gland) ɛn i de blok di rilis fɔ di signal we dɛn nɔ want (ACTH) we de mek di adrenal gland dɛn wok.
Fɔ tɔk am simpul wan, dis tan lɛk fɔ ɔf di ‘switch’ fɔ wan sistɛm we nɔ nid. dis de kכntro di bכdi in prodyushכn fכ ‘androgen’ כmon dεm we nכ nid.
di big advantej fכ dis na dat biכs dεn de kכntro di androgen lεvεl, di doz fכ stεroyd dεm we dεn nid fכ mεnten di כmon bεlε kin rεdכks bכku bכku wan . Dis min se di sayd ɛfɛkt dɛm we stɛroyd kin kam wit kin ridyus bak bad bad wan.
| Di kwaliti we pɔsin gɛt | Ol tritmεnt (high stεroyd dכz) . | Nyu tritmɛnt (Crinecerfont + lɔw stɛroyd dos) . |
|---|---|---|
| Di we aw pɔsin de wok | fכ gi di rεdכks kכtisol כmon na do. | fכ blכk di signal dεm we dεn nכ want (ACTH) frכm di bren εn fכ kכntrכl di androjen prodakshכn. |
| Di men tin we dɛn want fɔ du | Dɛn nid fɔ tek ay dos fɔ kɔntrol di ɔmon lɛvɛl. | Lכs di androgen lεvεl, ridyus di doz כf stεroyd dεm we dεn nid. |
| Di sayd ɛfɛkt dɛn we kin apin | Prɔblɛm dɛn we kin de fɔ lɔng tɛm lɛk we yu gɛt bɔku bɔku bɔdi, we yu nɔ kin gro fayn, ɛn we yu bon dɛn kin wik. | Potεnshal fכ ridyus stεroyd-rεlatεd sayd ifekt dεm. Dɛn dɔn ripɔt smɔl smɔl sayd ɛfɛkt dɛm (ed pen, taya). |
Udat dis mɛrɛsin fit fɔ?
Naw dɛn dɔn gri fɔ ‘Crinesurfont’ fɔ CAH pasɛnt dɛn we dɔn pas 4 ia . I nɔto fɔ tek ples fɔ stɛroyd, bɔt na tin we dɛn ad to stɛroyd.Wan ad-ɔn tritmɛnt.
- Fɔ pikin dɛn: Bikɔs pikin dɛn stil de gro, if dɛn tek bɔku stɛroyd, dat kin rili afɛkt dɛn fɔ gro. So, dis mɛrɛsin, we kin ridyus di doz fɔ stɛroyd, kin rili yusful fɔ dɛn. Dis kin impɔtant mɔ we pɔsin de yɔŋ, we i nɔ kin izi fɔ kɔntrol di ɔmon dɛn.
- Fɔ big pipul dɛm: Dis tritmɛnt kin gi rilif bak to big pipul dɛm wae de sɔfa wit sayd ɛfɛkt dɛm wae dɛn tek stɛroyd fɔ lɔng tɛm, ɔr wae nɔr kin izi fɔ mek dɛn kɔntinyu fɔ gɛt stebul ɔmon lɛvɛl.
Lan bɔt di sayd ɛfɛkt ɛn sef
Insay klinik trial, na sɔm smɔl smɔl, tɛmporari sayd ɛfɛkt dɛn nɔmɔ dɛn dɔn ripɔt wit dis drɔg.
- Fɔ big pipul: taya, ed pen, diziz, jɔyn pen.
- Fɔ pikin dɛn: ed we de at, bɛlɛ we de at, taya, nos we de stɔp.
Impɔtant tin na dat, bɔku pan dɛn sik ya nɔ bin de apin so ɔltɛm, ɛn di nɔmba fɔ di wan dɛn we bin gɛt fɔ stɔp fɔ tek di mɛrɛsin bin rili smɔl.
Speshal Wonin: Yu fɔ no bɔt Adrenal Kraysis!
We yu de tek dis mɛrɛsin, i rili impɔtant fɔ no bɔt wan siriɔs tin we dɛn kɔl ‘adrenal crisis’. Fɔ tɔk am simpul wan, dis kɔndishɔn kin apin if di bɔdi nɔ gɛt di ‘kɔtisol’ we i nid insay di tɛm we i strɛs bad bad wan (e.g., siriɔs infɛkshɔn, ɔpreshɔn, aksidɛnt).
So, tɔk to yu dɔktɔ ɛn mek shɔ se yu no ustɛm yu fɔ tek ɛkstra stɛroyd doz we yu gɛt imejensi ("stress dose") . Dɔn bak, no bɔt di sayn dɛm fɔ ‘adrenal kraysis’ (e.g., fɔ vɔmit pasmak, dayarɛa, diziz, nɔr de no natin) ɛn mɛmba fɔ go na ɔspitul ɔ imejensi rum (ETU) wantɛm wantɛm if dis kayn tin apin.
Aw a go no if dis mɛrɛsin rayt fɔ mi/mi pikin?
Dis disizhɔn fɔ bi we yu tɔk to yu dɔktɔ, mɔ wit di dɔktɔ we de mɛn di sik dɛn we de mek yu bɔdi . Yu dɔktɔ go tink bɔt bɔku tin dɛn lɛk aw yu blɔd tɛst, aw yu de gro, yu wet, yu blɔd prɛshɔn, ɛn di ɔmon we yu gɛt naw, fɔ no if dis mɛrɛsin fayn fɔ yu.
Dis nɔto mɛrɛsin fɔ CAH, bɔt big op de se i go ɛp fɔ mek di sikman dɛn gɛt bɛtɛ layf, fɔ ridyus di sayd ɛfɛkt dɛn we stɛroyd kin gɛt, ɛn mek i izi fɔ yuz di ɔmon.
Mɛsej we dɛn kin kɛr go na os
- CAH min sewan jεnεtik kכndishכn we di adrenal gland dεm de prodyuz sכm kכtisol εn mכr andrכjen כmon dεm.
- Crinecerfont na nyu tritmɛnt we dɛn dɔn mek spɛshal fɔ CAH, we na di fɔs wan fɔ dis kayn we insay 70 ia.
- Dis nɔto mɛrɛsin we de tek ples fɔ stɛroyd. Na mɛrɛsin we dɛn kin gi apat frɔm stɛroyd ɛn i kin ɛp fɔ ridyus di doz fɔ stɛroyd we dɛn nid.
- Di men bɛnifit na di ebul fɔ ridyus di lɔng tɛm sayd ɛfɛkt dɛm we stɛroyd kin gɛt.
- Mek shɔ se yu aks yu dɔktɔ bɔt di imejensi kɔndishɔn we dɛn kɔl 'adrenal crisis' ɛn di "stress dose" we yu fɔ tek da tɛm de.
- I impɔtant fɔ tɔk to dɔktɔ we spɛshal pan ɔmon sik dɛn fɔ no if dis tritmɛnt fayn fɔ yu ɔ yu pikin.
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