Imajin, fɔ no rizin, wantɛm wantɛm yu gɛt bɛlɛ we yu nɔ go ebul fɔ bia. Along wit dat, yu limbs go numb, yu fil laik yu go faint, en yu fil nauseous. Ivin afta dɛn dɔn go to bɔku dɔktɔ dɛn ɛn du bɔku tɛst dɛn, dɛn nɔ kin ebul fɔ no di rayt tin we mek di sik kam. Yu ɔ pɔsin we yu sabi dɔn gɛt dis ɛkspiriɛns? Sɔntɛm di tin we kin mek i apin na wan sik we nɔ kin apin we bɔku pipul dɛn na wi kɔntri nɔ ivin yɛri bɔt. Tide wi go tɔk bɔt wan pan dɛn kayn sik ya, Acute Hepatic Porphyria , ɔ AHP.
Fɔ tɔk am simpul wan, wetin na Akyu Ɛpatik Pɔfyuria (AHP)?
AHP na wan sik wae nɔr kin bɔrku pan jɛnɛtiks wae kin afɛkt wi nervɔs sistɛm ɛn sɔmtɛm wi skin. I nɔ kin bɔku so dat i kin ambɔg lɛk 5 pan ɛvri 100,000 pipul dɛn. I kin mek yu gɛt sɔm kayn sik dɛn we kin mek yu fil bad wantɛm wantɛm, we kin mek yu fil pen. Sɔntɛnde, dɛn tin ya kin mek pɔsin in layf de pan denja.
Fɔ ɔndastand dis, lɛ wi tɔk smɔl bɔt di blɔd we de na wi bɔdi. wi rεd bכdi sεl dεm gεt wan protin we dεn kכl `` Hεmoglobin ''. di men wok we i de du na fכ pik di כksijεn we de kam insay di lכng dεm we wi de blo εn gi am to εvri כda כgan εn tisu dεm na di bכdi. I tan lɛk ‘delivery service’ we de kɛr ɔksijɛn.
כda kכmכpכnt de we implεnt fכ mek dis protin we dεn kכl ``Hεmoglobin'', εn wi kכl am ``Hεm'' .
wetin kin apin na di bכdi fכ pכsin we gεt AHP sik na we wan εnzym we nid fכ dכn כ lכs na sכm stej insay di prכsεs fכ prodyuz dis kכmכpכnt we dεn kכl ``Heme''. I tan lɛk we dɛn de kuk di it, if wan tin nɔ de, dɛn nɔ go ebul fɔ pripia di it fayn.
di dεfisit fכ dis εnzym de apin mεntal wan na wi liva . Wi kin kɔl di liva bak `(Hepatic)`. Na dat mek dɛn dɔn gi dis sik di nem `Acute Hepatic Porphyria` .
Naw, wetin kin apin we wi nɔ ebul fɔ mek `(Heme)`? di raw mεtirial dεm we dεn bin yuz fכ am, we na `(Porphobilinogen – PBG)` εn `(Aminolevulinic acid – ALA)`, de bigin fכ kכmכt na di liva. Dɛn pɔyzin ya kin gɛda na di blɔd ɛn travul ɔlsay na di bɔdi, mɔ we kin pwɛl wi nervɔs sistɛm. Na bikɔs ɔf da nerve damej de mek sayn dɛn lɛk bad bad pen , swɛt , ɛn nɔys kin kam.
Wetin na di men kayn AHP sik?
Fo men kayn AHP de. εvri tכp de kכz bay we difrεn εnzym dεn nכ de insay di prכsεs fכ mek ``(Heme)``. Bɔt ɔl dɛn 4 ya kin afɛkt di liva ɛn di nervɔs sistɛm mɔ.
Akyu intamitεnt Pכfyuria (AIP) .
Dis na di kayn we de afɛkt 80% pan di wan dɛn we gɛt AHP, dat na, di mɔtalman. insay dis, di εnzym `(Hydroxymethylbilane synthase – HMBS)` de dכn. di rizin fכ dis na di chenj na di `(HMBS)` jin. Insay mɛrɛsin, wi kin kɔl dis `(mutation)` jin . Sɔntɛnde, dis kin kɔmɔt frɔm mama ɛn papa to pikin dɛn. Ɔ sɔmtɛm i kin apin randomly pan pɔrsin wae nɔr gɛt ɛni famili histri.
Aw di AHP simptom dɛm kin fil lɛk?
Nɔto ɔlman wae gɛt AHP gɛt di sem sayn dɛm. Sɔm pipul dɛn nɔ kin gɛt ɛni sayn atɔl ɔl dɛn layf. Bɔt sɔm pipul dɛn kin gɛt bad bad atak ɔltɛm. I impɔtant fɔ no dɛn sayn ya.
| Di bɔdi sistɛm we dɛn afɛkt | Di sayn dɛm we dɛn kin si bɔku tɛm |
|---|---|
| Abdominal (we gɛt fɔ du wit di bɛlɛ) . | Bɛlɛ pen we yu nɔ ebul fɔ ɛksplen (dis na di men sayn ), nɔs, vɔmit, kɔnstipɛshɔn. |
| Nɛvɔ sistɛm | Kכndishכn dεm lεk fכ sכmtεm sכmtεm, wik, mכsul dεm de pen εn wik, εn rεli paralayz. |
| Di we aw pɔsin de tink | Nɔr de rɛst, wɔri, kɔnfyushɔn, hallucinations. |
| At ɛn blɔd prɛshɔn | Hat de bit kwik kwik wan, blɔd prɛshɔn de go ɔp. |
| Ɔda tin dɛn we de apin | Yurin we de tɔn dak rɛd ɔ brawn (espɛshali we di sik bad bad wan). |
Bikɔs dis sik nɔ kin bɔku, bɔku tɛm dɛn kin mistek kɔl dɛn sayn ya fɔ ɔda kɔmɔn sik dɛn, we kin mek dɛn nɔr kin te fɔ no bɔt dis sik.
Wetin na de trig wae de mek de sik wɔs?
Pɔsin wae gɛt AHP kin liv nɔrmal layf. Bɔt sɔm tin dɛn kin mek pɔsin gɛt di sik. I rili impɔtant fɔ no bɔt dɛn tin ya.
- Sɔm mɛrɛsin dɛn: Ɛspɛshali sɔm mɛrɛsin dɛn lɛk antibayɔtik we gɛt `(Barbiturates)` ɛn `(Sulfa)`. If yu gɛt AHP, i impɔtant fɔ tɔk to yu dɔktɔ bifo yu tek ɛni mɛrɛsin.
- Fɔ drink rɔm: Na rɔm na di men tin we kin mek dis sik wɔs.
- Strikt it ɔ fast: If yu it it we nɔ gɛt bɔku kalori ɛn kabɔhaydrɛt (stach) i kin mek di sik wɔs.
- di chenj dεm na di כmon dεm: di chenj dεm na di כmon lεvεl dεm, spεshal wan pan uman dεm, we kכnεkt wit dεn mεnstral saykl.
- Strɛs ɛn Infɛkshɔn: Ɛni kayn strɛs na di bɔdi, ivin wan sik lɛk fiva ɔ kol, kin mek di sik wɔs.
Ustɛm yu fɔ go to dɔktɔ?
If yu gɛt dɛn sik ya, duya nɔ dismis dɛn as nɔmal. I rili impɔtant fɔ go to dɔktɔ, mɔ pan dɛn kayn tin ya:
- If yu gɛt siriɔs bɛlɛ pen we yu nɔ ɛksplen, we de kam bak.
- If nyurolɔjik simptom dɛm lɛk fɔ swɛt ɛn wik kin apin di sem tɛm.
- If yu no se sɔmbɔdi na yu famili gɛt di sik `(Porphyria)`.
- If yu notis se yu urine de tɔn dak rɛd/brawn kɔlɔ.
If yu gɛt siriɔs sayn dɛm, lɛk pen we yu nɔ go ebul fɔ bia, i nɔ izi fɔ blo, yu maynd kɔnfyus, ɔ yu bɔdi nɔ de wok fayn, go na ɔspitul in Imejɛnsi Tritmɛnt Yunit (ETU) wantɛm wantɛm.
Tritmɛnt de fɔ AHP?
Bikɔs dis na sik we pɔsin kin gɛt frɔm in bɔdi, dɛn nɔ gɛt ɛni mɛrɛsin yet. Bɔt, tritmɛnt dɛn de we rili fayn fɔ kɔntrol ɛn mek di sik nɔ wɔs.
If i siriɔs, dɛn nid fɔ go na ɔspitul ɛn tritmɛnt.
- Di men tin na fɔ no di tin we mek di sik bigin fɔ bɔn ɛn stɔp am.
- Dɛn kin gi strɔng pen kil fɔ kɔntrol di bad bad pen.
- Dɛn kin gi mɛrɛsin fɔ kɔntrol di nɔys ɛn vɔmit.
- bay we yu gi di bכdi glukכs (shuga), dat na glukכs wit salin tru wan vein, di tכxin dεm we de prodyuz na di liva kin rεdכks.
- as spεsifi k tritmεnt , dεn de du `(Heme)` injεkshכn `(Hemin infushכn)`. dis kin bi bay we dεn gi `(Heme)` na do, sεnd signal to di liva se, "Yu gεt `(Heme)`, yu nכ nid fכ mek nכ mכr," εn stכp di prodyushכn fכ tכxin dεm.
- Apat frɔm dis, naw dɛn gɛt mɔdan mɛrɛsin dɛn we dɛn de mek tru jenɛtik tɛknɔlɔji fɔ mek di sik nɔ kam bak fɔ di wan dɛn we gɛt di sik we dɛn kin gɛt ɔltɛm.
Di dɔktɔ we de chɛk yu go no di tritmɛnt we go fayn fɔ yu.
Mɛsej we dɛn kin kɛr go na os
- Acute Hepatic Porphyria (AHP) na wan sik we nɔ kin apin so ɔltɛm we kin mek pɔsin gɛt siriɔs sik. Bɔt, bɔrku pipul dɛm wae gɛt dis sik kin liv nɔrmal layf.
- di men simptom na di apinεns fכ nyurolכjik simptom dεm (numbness, wikεd) wit siriכs bεlε pen we dεn nכ εksplen.
- I rili impɔtant fɔ avɔyd tin dɛn we kin mek di sik wɔs, lɛk sɔm mɛrɛsin, rɔm, ɛn fast.
- If yu gɛt sɔm kayn sayn dɛn we yu go sɔprayz, mɔ if pɔsin na yu famili gɛt dis sik, mek shɔ se yu go to dɔktɔ.
- Naw, tritmɛnt dɛn de we rili fayn fɔ kɔntrol dis sik ɛn fɔ manej di flare-up. So nid nɔ de fɔ fred.
AHP, Akyu Ɛpatik Pɔfyuria, Pɔfyuria, Bɛlɛ we de at, Nyurolɔjik sik dɛn, Jɛnɛtik sik dɛn, Ɛm, Ɛmoglobin, Pɔfyrin, Sik dɛn we nɔ kin bɔku
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