Yafunu mu yaw a emu yɛ den ne adwene a ɛyera a ntease biara nnim... Momma yensua yare a ɛntaa nsi yi ho ade? (Acute Hepatic Porphyria) .

Fa no sɛ, esiane ntease biara nti, mpofirim ara wo yafunu mu yɛ wo yaw a wuntumi nnyina ano. Ɛno ka ho no, wo nsa ne anan no yɛ mmerɛw, wote nka sɛ wobɛtotɔ piti, na wote nka sɛ wo ho ayɛ wo yaw. Bere a wɔakɔ nnuruyɛfo pii nkyɛn na wɔayɛ nhwehwɛmu pii akyi mpo no, wontumi nhu nea ɛde yare no bae ankasa. So wo anaa obi a wunim no anya saa osuahu yi? Ebia nea ɛde ba ne awosu mu tebea bi a ɛntaa nsi koraa a nnipa pii a wɔwɔ yɛn man yi mu nte ho asɛm mpo. Ɛnnɛ yɛrebɛka tebea a ɛte saa biako ho asɛm, Acute Hepatic Porphyria , anaa AHP.

Sɛ yɛbɛka no tiawa a, dɛn ne Acute Hepatic Porphyria (AHP)?

AHP yɛ awosu mu yare a ɛntaa nsi a ɛka yɛn ntini ne ɛtɔ mmere bi a yɛn honam ani. Ɛntaa mma araa ma ɛka nnipa 100,000 biara mu bɛyɛ 5. Ebetumi de yare no ho sɛnkyerɛnne a emu yɛ den a ɛyɛ yaw aba mpofirim. Ɛtɔ mmere bi a, eyinom betumi de nkwa ato asiane mu.

Sɛ yɛbɛte eyi ase a, momma yɛnka mogya a ɛwɔ yɛn nipadua mu no ho asɛm kakra. Yɛn mogya mu nkwammoaa kɔkɔɔ no kura protein bi a wɔfrɛ no `` Hemoglobin ''. N’adwuma titiriw ne sɛ ɛbɛfa mframa pa a ɛba ahurututu mu bere a yɛrehome no na ɛde akɔ nipadua no mu akwaa ne ntini foforo biara mu. Ɛte sɛ ‘delivery service’ a ɛde oxygen kɔ.

Ade foforo bi wɔ hɔ a ɛho hia na ama wɔayɛ saa protein yi a wɔfrɛ no ``Hemoglobin'', na yɛfrɛ no ``Heme'' .

Nea ɛkɔ so wɔ obi a ɔwɔ AHP yare no nipadua mu ne enzyme bi a ɛho hia wɔ bere bi mu wɔ adeyɛ a ɛma wɔyɛ saa ade yi a wɔfrɛ no ``Heme'' no so tew anaasɛ ɛyera. Ɛte sɛ bere a wɔrenoa aduan bi no, sɛ ade biako ayera a, wontumi nsiesie aduan no yiye.

Enzyme yi a enni hɔ no ba yɛn mmerɛbo mu titiriw . Yɛsan frɛ mmerɛbo no `(Hepatic)`. Ɛno nti na wɔato saa yare yi din `Acute Hepatic Porphyria` .

Afei, dɛn na ɛba bere a yentumi nyɛ `(Heme)`? Nneɛma a wɔde yɛɛ no, a ɛne `(Porphobilinogen – PBG)` ne `(Aminolevulinic acid – ALA)`, fi ase boaboa ano wɔ mmerɛbo no mu. Saa awuduru yi boaboa ano wɔ mogya no mu na ɛnam nipadua no mu nyinaa, na ɛsɛe yɛn ntini ahorow no titiriw. Ɛyɛ saa ntini a ɛsɛe no nti na sɛnkyerɛnne te sɛ ɛyaw a emu yɛ den , akisikuru , ne akisikuru sɔre.

Dɛn ne AHP yare ahorow atitiriw?

AHP ahorow atitiriw anan na ɛwɔ hɔ. Ɔkwan biara fi enzyme soronko bi a enni hɔ wɔ adeyɛ a wɔde yɛ ``(Heme)`` mu. Nanso anan no nyinaa ka mmerɛbo ne ntini ahorow no titiriw.

Porphyria a Ɛyɛ Ntɛmntɛm (AIP) .

Eyi ne ɔkwan a ɛka AHP ayarefo 80% , kyerɛ sɛ, dodow no ara. Wɔ eyi mu no, enzyme `(Hydroxymethylbilane synthase – HMBS)` a enni hɔ. Nea enti a ɛte saa ne nsakrae a ɛba `(HMBS)` awosu mu abɔde mu. Wɔ nnuruyɛ mu no, yɛfrɛ eyi `(mutation)` gene . Ɛtɔ mmere bi a, wobetumi anya eyi afi awofo hɔ ama mmofra. Anaasɛ ɛtɔ mmere bi a ebetumi aba kwa wɔ obi a onni abusua abakɔsɛm biara mu.

AHP ho sɛnkyerɛnne ahorow no te dɛn?

Ɛnyɛ obiara a ɔwɔ AHP na ɔwɔ yare no ho sɛnkyerɛnne koro. Ebia nnipa binom rennya yare no ho sɛnkyerɛnne biara koraa wɔ wɔn nkwa nna nyinaa mu. Nanso ebia nnipa binom taa nya ntua a emu yɛ den. Ɛho hia sɛ wuhu saa sɛnkyerɛnne ahorow yi.

Dɛn ne nneɛma a ɛkanyan yare no ma emu yɛ den?

Obi a ɔwɔ AHP betumi atra ase sɛnea ɛsɛ. Nanso, nneɛma bi betumi akanyan yare no. Ɛho hia paa sɛ yehu eyinom.

• Nnuru bi: Titiriw nnuru bi te sɛ nnuru a ekum ɔyare mmoawa a `(Barbiturates)` ne `(Sulfa)` wom. Sɛ wowɔ AHP a, ɛho hia sɛ wo ne wo duruyɛfo kasa ansa na woanom aduru biara.
• Nsa a wɔnom: Nsã ne ade titiriw a ɛma yare yi mu yɛ den.
• Adidi a ɛyɛ katee anaa mmuadadi: Sɛ wudi aduan a ahoɔdennuru ne carbohydrates (starch) nnim a, ebetumi ama yare no asɛe kɛse.
• Nsakrae a ɛba nkwaadɔm mu: Nsakrae a ɛba wɔ hormone dodow mu, titiriw wɔ mmea mu, a ɛbata wɔn brayɛ ho.
• Adwennwen ne Nyarewa: Adwennwen biara a ɛba nipadua no so, tebea a ɛde ɔyare mmoawa ba te sɛ atiridiinini anaa atiridiinini mpo betumi ama yare no ayɛ kɛse.

Bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn?

Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, yɛsrɛ sɛ mmu sɛ ɛyɛ nea ɛfata. Ɛho hia yiye sɛ wohwehwɛ aduruyɛ mu afotu, titiriw wɔ nsɛm a edidi so yi mu:

• Sɛ wowɔ yafunu mu yaw a emu yɛ den a wontumi nkyerɛkyerɛ mu a ɛsan ba bio a.
• Sɛ ntini mu sɛnkyerɛnne te sɛ akisikuru ne mmerɛwyɛ ba bere koro mu a.
• Sɛ wunim sɛ obi wɔ w'abusua mu wɔ yareɛ `(Porphyria)`.
• Sɛ wuhu sɛ wo nsu no redan kɔla kɔkɔɔ/biribiri tuntum a.

Sɛ wuhu sɛnkyerɛnne ahorow a emu yɛ den, te sɛ ɛyaw a wuntumi nnyina ano, ɛyɛ den sɛ wobɛhome, adwene mu basaayɛ, anaasɛ wo nsa ne anan a ɛyera a, kɔ ayaresabea Ayaresa a Ɛho Hia Ntɛmntɛm (ETU) ntɛm ara.

So aduru bi wɔ hɔ a wɔde sa AHP?

Esiane sɛ eyi yɛ awosu mu yare nti, wonnyaa ayaresa biara. Nanso, ayaresa ahorow a etu mpɔn yiye wɔ hɔ a wɔde siw yare no ano na wɔasiw ano.

Wɔ tebea horow a emu yɛ den mu no, ɛho hia sɛ wɔde obi kɔ ayaresabea na wɔsa yare.

• Ade titiriw ne sɛ wubehu nea ɛkanyan yare no na ama agyae.
• Wɔma ɛyaw ano aduru a ano yɛ den de siw ɛyaw a emu yɛ den ano.
• Wɔde nnuru ma de siw akisikuru ne ɔfe ano.
• Ɛdenam glucose (asikre), kyerɛ sɛ, glucose a nkyene wom a wɔde fa ntini bi mu ma nipadua no so no, awuduru a ɛba mmerɛbo no mu no betumi atew.
• Sɛ́ ayaresa pɔtee no , wɔyɛ `(Heme)` injections `(Hemin infusions)`. Wɔyɛ eyi denam `(Heme)` a wɔde ma wɔ abɔnten, de sɛnkyerɛnne bi kɔ mmerɛbo no so sɛ, "Wowɔ `(Heme)`, enhia sɛ woyɛ bio," na wogyae awuduru a wɔyɛ no so.
• Sɛ eyi da nkyɛn a, mprempren nnɛyi nnuru bi wɔ hɔ a wɔnam awosu mu mfiridwuma so yɛ de siw yare no ano bio mma wɔn a wɔtaa nya yare no.

Oduruyɛfo a ɔhwɛ wo mu no na ɔbɛkyerɛ ayaresa a ɛfata wo sen biara.

AHP, Acute Hepatic Porphyria, Porphyria, Yafunu mu yaw, Ntini mu nyarewa, Awosu mu nyarewa, Heme, Hemoglobin, Porphyrin, Nyarewa a ɛntaa nsi