So woate "Porphyria" ho asɛm pɛn? Momma yɛnka yare a wɔntaa nhu yi ho asɛm!

Adeyɛ a ɛyɛ den na ɛyɛ nwonwa yiye a ɛkɔ so wɔ wo nipadua mu ne biribi a wɔfrɛ no "Heme" a wɔyɛ. Saa Heme yi ho hia ma Hemoglobin a ɛma yɛn mogya kɔla kɔkɔɔ na ɛde oxygen kɔ nipadua no nyinaa mu no yɛ. Ɛnyɛ ɛno nko, na mmom saa Heme yi boa wɔ dwumadi afoforo pii a ɛho hia mu wɔ yɛn nkwammoaa mu. Nanso, ɛtɔ mmere bi a, ɔkwan a wɔfa so yɛ Heme yi betumi asɛe. Ɛno ne bere a tebea horow a ɛka nyarewa kuw bi a wɔntaa nhu a wɔfrɛ no "Porphyria" ho ba. Mma ɛnhaw wo, momma yɛnka eyi ho asɛm tiawa.

Dɛn ankasa ne Porphyria?

Sɛ yɛbɛka no tiawa a, porphyria yɛ din a wɔtaa de frɛ nyarewa kuw bi a wɔntaa nhu a ɛka ɔkwan a wɔfa so yɛ heme wɔ yɛn nipadua mu no. Susuw ho saa kwan yi so, heme a wɔyɛ no te sɛ adeyɛ a ɛyɛ den a ɛwɔ anammɔn awotwe. Saa anammɔn yi mu biara hwehwɛ sɛ enzyme pɔtee awotwe na ama ayɛ adwuma yiye. Enzyme yɛ protein ahorow a ɛma nnuru yɛ adwuma ntɛmntɛm wɔ yɛn nipadua mu. Nnuru a wɔfrɛ no "porphyrins" ne "porphyrin precursors" nso ka saa adeyɛ yi ho.

Afei susuw ho hwɛ, sɛ enzyme bi a ɛwɔ anammɔn awotwe yi mu biako mu anyɛ adwuma yiye a, dɛn na ɛba? Ɛhɔ na ɔhaw no fi ase. Te sɛ nkɔnsɔnkɔnsɔn mu adeyɛ no, sɛ saa enzyme no ntumi nyɛ adwuma a, porphyrin ne porphyrin a edi kan a ɛbae ansa na ɛreba no fi ase boaboa ano wɔ nipadua no nkwammoaa mu. Ɛyɛ nnuru a ɛboaboa ano yi na ɛde porphyria ho sɛnkyerɛnne ahorow ba.

Ebia woresusuw ho sɛ, "Dɛn nti na saa enzyme yi ntumi nyɛ adwuma yiye?" Mpɛn pii no, nea ɛde ba ne awosu mu nsakrae. Ɛne sɛ, nsakrae bi ba awosu mu abɔde bi a ɛkyerɛw enzyme bi a ɛka ho wɔ heme a wɔyɛ mu. Saa yare yi a wɔfrɛ no porphyria no taa fi awosu mu. Ɛne sɛ, wobetumi de saa awosu mu nsakrae yi akɔ abusua no mufo afoforo nkyɛn. Sɛ ɛba saa a, ebia wɔwɔ porphyria nso anaasɛ wɔyɛ wɔn a wɔde yare no kɔ.

Nanso mma ɛnhaw wo. Sɛ wuhu sɛ wowɔ saa yare yi a, nnuruyɛfo betumi aboa wo ma woadi wo sɛnkyerɛnne ahorow no ho dwuma, na ebia wɔasiw ano mpo. Eyi betumi aboa ma nkɛntɛnso a tebea horow yi nya wɔ w’asetra so no so atew.

So porphyria ahorow bi wɔ hɔ?

Yiw, porphyria ahorow bɛyɛ awotwe na ɛwɔ hɔ. Nnuruyɛfo frɛ wɔn nyinaa bom sɛ "porphyrias." Nnuruyɛfo begyina wo sɛnkyerɛnne ahorow so akyerɛ porphyria ko a wowɔ ne baabi a saa nnuru foforo a madi kan aka ho asɛm no boaboa ano wɔ wo nipadua mu.

Mpɛn pii no, wobetumi akyekyɛ porphyria ahorow yi mu akuw atitiriw abien a egyina wɔn sɛnkyerɛnne ahorow so:

• Porphyrias a Ɛyɛ Hu (ɛtɔ mmere bi a wɔfrɛ no "Acute Hepatic Porphyrias") .
• Porphyrias a ɛwɔ honam ani

Porphyrias a ɛyɛ hu

Saa nyarewa kuw yi betumi de yafunu mu yaw kɛse ne sɛnkyerɛnne afoforo aba. Porphyrias a emu yɛ den ahorow bi betumi ama wo honam ani ayɛ mmerɛw wɔ owia hann ho na ama wo honam ani ayɛ kurukuruwa.

Porphyrias a emu yɛ den ahorow bi ni:

• Acute Intermittent Porphyria (AIP): Eyi ne porphyria a emu yɛ den a abu so sen biara. Ebetumi ama yafunu mu ayɛ no yaw mpofirim, nanso owia hann anaa honam ani akisikuru biara nni hɔ.
• Hereditary Coproporphyria (HCP): Nea ɛka yafunu a ɛyɛ mmerɛw mpofirim ho no, wo honam ani betumi ayɛ mmerɛw wɔ owia hann ne akisikuru ho.
• Variegate Porphyria (VP): Wɔ tebea yi mu no, wubetumi anya yafunu mu akisikuru mpofirim ne/anaasɛ honam ani akisikuru. Eyinom betumi asɛe kɛse bere a wɔde wɔn ho hyɛ owia hann mu no.
• ALAD-a enni mu porphyria: ALAD gyina hɔ ma enzyme "delta-aminolevulinic acid (ALA) dehydratase". Eyi yɛ porphyria a ɛntaa nsi koraa, na ɛtaa da sɛnkyerɛnne ahorow adi wɔ mmofraase.

Porphyrias a ɛwɔ honam ani

Saa nyarewa kuw yi de honam ani sɛnkyerɛnne ahorow ba bere a wɔde wɔn ho hyɛ owia hann mu no. Ebia eyinom bi ne ɛyaw, honam ani kɔla a ɛbɛsakra, ɛho ahonhon, ne/anaasɛ akisikuru. Ɛdenam nea ɛde ba no so no, nnipa binom betumi anya honam ani akisikuru bi a wɔfrɛ no Cutaneous Porphyrias, bere a afoforo nso rennya akisikuru.

Porphyrias a Ɛwɔ Honam ani a Ɛyɛ Blistering Ahorow:

• Porphyria Cutanea Tarda (PCT): Eyi ne porphyria a abu so sen biara.
• Congenital Erythropoietic Porphyria (CEP): "Erythropoietic" kyerɛ wo nnompe mu ntini. Eyi ne beae titiriw a porphyrins a ɛboro so no boaboa ano wɔ yare yi mu. Eyi nso yɛ porphyria a ɛntaa nsi koraa.

Porphyrias a Ɛnyɛ Akisikuru Ahorow: Cutaneous Porphyrias:

• Erythropoietic Protoporphyria (EPP) ne X-linked Porphyria (XLP): Wɔsan frɛ eyinom "the protoporphyrias" efisɛ nnuru a ɛboaboa ano wɔ saa nyarewa yi mu ne "protoporphyrin." "X-linked" kyerɛ sɛ awosu mu nsakrae bi wɔ X chromosome no so.
• Hepatoerythropoietic porphyria a ɛma mogya tu:Eyi nso yɛ porphyria a ɛntaa nsi koraa.

Dɛn ne Porphyria ho sɛnkyerɛnne ahorow?

Nsɛnkyerɛnne a wubehu ne bere tenten a ɛbɛtra hɔ no begyina porphyria ko a wowɔ no so. Nsɛnkyerɛnne no betumi ayɛ nea emu nyɛ den koraa anaasɛ emu yɛ den yiye. Ebia nnipa binom a wɔwɔ porphyria no rennya yare no ho sɛnkyerɛnne biara koraa. Nanso, wɔ tebea horow bi mu no, sɛ wɔansa saa sɛnkyerɛnne ahorow yi yiye a, ebetumi de ne nkwa ato asiane mu.

Nsɛnkyerɛnne a Ɛfa Acute Porphyria Ho

Porphyrias a emu yɛ den fi heme a edi kan a wɔfrɛ no aminolevulinic acid (ALA) ne porphobilinogen (PBG) a ɛkɔ soro no so. Saa nneɛma yi ka sɛnea wo ntini ahorow no yɛ adwuma. Susuw ho hwɛ, biribiara fi wo ntini a wobɛkanyan so kosi aduan a wobɛyam so no, ntini ahorow no na ɛhwɛ so. Porphyria ma ntini ahorow no ntumi nyɛ adwuma yiye, na ɛde sɛnkyerɛnne ahorow a ɛte sɛ "ntua" ba.

Porphyria a emu yɛ den betumi de sɛnkyerɛnne ahorow te sɛ:

• Yafunu mu yaw a emu yɛ den
• Akisikuru ne ɔfe
• Ayamtim
• Ɛyaw a ɛba wo koko, akyi, nsa, ne/anaasɛ nan mu
• Brɛ
• Asoroben
• Adwene mu nsakrae te sɛ adwene mu naayɛ, basabasayɛ, ne nsusuwii hunu
• Adwumaden
• Koma a ɛbɔ ntɛmntɛm (Tachycardia) .
• Mogya mmoroso
• Ayɛ a ɛyɛ mmerɛw, ɛyɛ mmerɛw (Paresthesia) .
• Ntini mu mmerɛwyɛ anaa obubuafo (eyi nso betumi aka ntini a ɛboa wo ma wohome) .
• Akisikuru a ɛma obi ho kyere no
• Nsu a ɛyɛ tuntum anaa kɔkɔɔ (porphyrin ne nnuru afoforo betumi asesa nsu kɔla) .

Sɛ wowɔ awosu mu coproporphyria ``(HCP)`` anaa variegate porphyria ``(VP)`` a, ebia wo honam ani yɛ mmerɛw wɔ owia hann ho. Sɛ obi nya owia hann a, ebetumi ama ne honam ani ayɛ kurukuruwa, ne honam ani kɔla asakra, ne/anaasɛ akuru ayɛ no.

Porphyria a emu yɛ den betumi atra hɔ bere tenten ahe?

Ntua a ɛte sɛɛ betumi adi nnansa kosi nnansa. Nanso, titiriw sɛ wɔansa a, ebetumi akyɛ kɛse. Ebetumi adi adapɛn kosi asram pii ansa na yare no ho sɛnkyerɛnne no afi hɔ koraa. Ebia wubenya ntua biako anaa kakraa bi pɛ wɔ w’asetra mu. Nanso, nnipa binom betumi anya ntua mpɛn pii wɔ afe biako mu.

Porphyria a emu yɛ den betumi de nsɛnnennen a ɛtra hɔ kyɛ te sɛ mogya mmoroso, asaabo a entumi nyɛ adwuma yiye, ne nea ɛntaa nsi, mmerɛbo mu kokoram aba.

Nsɛnkyerɛnne a Ɛkyerɛ sɛ Porphyrias a Ɛnyɛ Akisikuru (EPP/XLP) .

Porphyrias a ɛwɔ honam ani no de honam ani sɛnkyerɛnne ahorow ba bere a owia hann bɔ wo no. Nea ɛka eyi ho ne owia hann tẽẽ a ɛwɔ abɔnten, ne owia hann a ɛfa mfɛnsere mu ba mu. Hann ahorow bi a wɔde nsa ayɛ nso betumi de yare no ho sɛnkyerɛnne ahorow aba. Saa porphyria yi mma yafunu mu yaw a emu yɛ den mpofirim anaa sɛnkyerɛnne afoforo a ɛyɛ "ntua" su no mma.

Sɛ wufi ase yɛ w’ade wɔ owia hann ho a, ebia mfiase no wobɛte nka sɛ wo ho ayɛ wo hyew. Sɛ woanfi owia no mu ntɛm a, ebia wubenya nea nnuruyɛfo frɛ no "phototoxic reaction." Ebia wubenya honam ani sɛnkyerɛnne te sɛ eyinom, titiriw wɔ w’anim, wo nsa, ne wo nan so:

• Ɛyɛ akisikuru
• Nnipa a wɔyɛ mmerɛw
• Rehono
• Ɛyaw a emu yɛ den wɔ baabi a ayɛ no
• Nsonsonoe nketenkete a ɛyɛ kɔkɔɔ, kɔkɔɔ, anaa bruu wɔ honam ani (`(Petechiae)`) .

Saa nneyɛe yi yɛ yaw kɛse na ɛtaa di nna abien kosi anum. Ɛsɛ sɛ wobɔ mmɔden sɛ wobɛtra dan mu na woadwo mmeae a ayɛ no nwini (sɛ nhwɛso no, wode mframa a ɛyɛ nwini a efi mframa a wɔde yɛ adwuma mu ba).

Nsɛnkyerɛnne a Ɛkyerɛ sɛ Cutaneous Porphyrias a Ɛyɛ Blistering

Nkwammoaa a wɔfrɛ no Porphyria Cutanea Tarda (PCT) .

Nnipa a wɔwɔ PCT betumi anya sɛnkyerɛnne te sɛ:

• Wora mu akisikuru (a ɛtaa ba nsa akyi) .
• Nkuruwankuruwa
• Honam ani kɔla a ɛsakra
• Wora a ɛyɛ den
• Wora a ɛyɛ mmerɛw a ɛyɛ mmerɛw sɛ wobɛtetew bere a wopira anaasɛ wɔhyɛ wo so ketewaa bi mpo
• Nwi a enyin dodo (mpɛn pii no wɔ anim, moma so nkyɛnkyɛn, anaa mmeae te sɛ abɔso) .

Porphyria a Wɔde Awo Nsu Mu (CEP) .

Saa porphyria a emu yɛ den yi fi ase da sɛnkyerɛnne ahorow adi wɔ awo akyi bere tiaa bi anaasɛ wɔ n’asetra mfiase pɛɛ. Nsɛnkyerɛnne a edi kan ne nsu kɔkɔɔ. Esiane sɛ akokoaa no mpaboa yɛ kɔkɔɔ nti, wɔtaa sɔ nnuruyɛfo hwɛ sɛ wobedi kan ahwɛ sɛ ebia ɔwɔ akisikuru.

CEP ho sɛnkyerɛnne ne sɛnkyerɛnne afoforo bi ne:

• Honam ani akisikuru a emu yɛ den mpo bere a wɔde owia hann kakraa bi mpo anaa hann a ɛma hann di dwuma no
• Nnompe mu akisikuru no nya ɔyare mmoawa, na ɛma nnompe mu nyarewa ne nnompe sɛe
• Anim no afã horow bi a ɛyera (aso ne hwene mu ntini) .
• Nse a ɛdannan fitaa-biribiri
• Anemia - Wei kyerε sε mogya mu nkwammoaa kɔkɔɔ nni mogya no mu. Ɛtɔ mmere bi a, ɛho hia sɛ wɔde mogya ma.
• Akisikuru a ɛyɛ kɛse
• Nkwammoaa mu nkwammoaa dodow a ɛba fam (`(Nsu mu nkwammoaa dodow a ɛba fam)`) .

Dɛn na ɛde porphyria ba?

Nsakrae a ɛba awosu mu nkwaadɔm bi mu na ɛde porphyria a emu yɛ den ba. Nanso, esiane sɛ wunya awosu mu nsakrae bi fi awo mu nti, ɛnkyerɛ sɛ wubenya porphyria ho sɛnkyerɛnne.Nnipa pii a wɔwɔ awosu mu nsakrae a ɛbata porphyria ho no nni sɛnkyerɛnne biara. Eyi kyerɛ sɛ awosu mu nhwehwɛmu nkutoo ntumi nsi so dua sɛ wɔahu yare no. Ɔkwan biako pɛ a wobɛfa so ahu no yiye sɛ ebia porphyria a emu yɛ den na ɛde wo sɛnkyerɛnne ahorow no ba ne sɛ wobɛsɔ wo nsu mu ahwɛ sɛ ebia ALA ne PBG dodow wɔ mu anaa.

Abenfo gye di sɛ nneɛma afoforo te sɛ hormone, nnuru bi, ne nnuan a wudi betumi adi dwuma wɔ ntua a emu yɛ den a efi ase no mu. Sɛ wɔwoo wo a wowɔ awosu mu nsakrae a ɛne porphyria a emu yɛ den wɔ abusuabɔ a, nneɛma afoforo yi betumi ama ntua ayɛ mmerɛw.

Nneɛma a ebetumi akanyan ntua ahorow yi ne:

• Mmea nna mu ahoɔdennuru dodow a ɛkɔ soro (sɛ nhwɛso no, wɔ luteal fã a ɛwɔ wo ɔsram biara brayɛ mu) .
• Nnuru bi (sɛ nhwɛso no, nnuru a wɔde da, nnuru a wɔde siw awo ano) .
• Nsã a wɔnom boro so
• Sigaretnom
• Carbohydrate a ɛba fam a wudi (sɛ nhwɛso no, bere a woredi mmuada anaasɛ wudi aduan titiriw bi akyi) .

Nneɛma a Ɛde Porphyria Cutanea Tarda (PCT) Ba .

PCT yɛ porphyria bi a ɛtaa ba mpanyimfo mu, na ɛyɛ soronko kakra wɔ porphyria ahorow afoforo ho. Nnuruyɛfo frɛ no "nneɛma a wɔanya de ba." Eyi kyerɛ sɛ wubetumi anya tebea no sɛ wunni awosu mu nsakrae mpo a.

Mpɛn pii no, asiane abien anaa nea ɛboro saa bom sɛe heme a wɔyɛ no sɛnea ɛsɛ. Nneɛma a ɛtete saa no bi ne:

• Estrogen a wɔde di dwuma (sɛ nhwɛso no, nnuru a wɔde siw awo ano anaa aduru a wɔde si hormone ananmu) .
• Nsã a wɔnom boro so
• Iron dodow a ɛkɔ soro wɔ nipadua no mu (`(Iron overload / hemochromatosis)`) .
• Hepatitis C yare mmoawa
• HIV a ɛma obi nya ɔyare mmoawa
• Sigaretnom

Ɔkwan bɛn so na nnuruyɛfo hu yare yi?

Nnuruyɛfo hu porphyria denam wo nipadua mu nhwehwɛmu ne nhwehwɛmu a wɔyɛ wɔ lab so. Ebia wobɛyɛ sɔhwɛ ahorow yi mu biako anaa nea ɛboro saa:

• Mogya mu nhwehwɛmu
• Nsu mu nhwehwɛmu
• Nsu mu nhwehwɛmu

Wo duruyɛfo bɛkyerɛkyerɛ nhwehwɛmu a ɛsɛ sɛ woyɛ ne nea nhwehwɛmu biara betumi ahu mu. Ebia wo duruyɛfo nso bɛkamfo akyerɛ sɛ wɔnyɛ awosu mu nhwehwɛmu mma wo ne afoforo a wɔwɔ w’abusua mu. Eyi betumi ahu nsakrae pɔtee bi a ɛba awosu mu nkwaadɔm mu a ebetumi ama obi anya porphyria.

Ɔkwan bɛn so na wɔsa porphyria?

Ayaresa a wubetumi apaw no gyina porphyria ko a wowɔ ne sɛnea ɛka wo so. Wo duruyɛfo bɛkyerɛkyerɛ nea eye ma wo tebea pɔtee no mu. Nneɛma a ɛfa nneɛma nyinaa ho a wubetumi ahwɛ kwan ni.

Porphyrias a Ɛyɛ Hu a Wɔsa

Sɛ wowɔ porphyria a emu yɛ den a, ebia ɛho behia sɛ wɔde wo kɔ ayaresabea kɔhwɛ wo bere a woanya "ntua" no. Wɔ ayaresabea no, nnuruyɛfo bɛyɛ nea edidi so yi mu biako anaa nea ɛboro saa:

• Nnuru a wɔde ma ma ALA ne PBG dodow so tew (hemin infusion) .
• Nnuru a wɔde ma de di ɛyaw, akisikuru, ne/anaasɛ akisikuru tebea ho dwuma
• Nsu a wɔde IV (a wɔde fa ntini mu) a wɔde ma
• Wo electrolyte dodow a wobɛhwɛ so na sɛ ɛho hia a, wobɛsan ahyɛ mu ma
• Nsakrae a ɛba w’adwene mu a wobɛhwɛ so yiye

Nea ɛbɛboa ma woasiw ntua ano daakye no, ebia wo duruyɛfo bɛkyerɛw aduru bi a wɔfrɛ no givosiran ama wo. Eyi yɛ aduru a wɔde twitwiw nipadua no ɔsram biara a ɛma ALA ne PBG a ɛba boro so no gyae.

Ɛho behia nso sɛ wokwati nneɛma a ɛkanyan wo te sɛ:

• Nnuru bi
• Mmuadadi ne akwan bi a wɔfa so siw calorie ano hye
• Nsã a wɔnom
• Tawa a wɔde di dwuma
• Owia a wobɛda (sɛ wowɔ VP anaa HCP a) .

Cutaneous Porphyrias a Wɔsa

Ɛmfa ho Cutaneous Porphyria ko a wowɔ no, ɛho hia sɛ wobɔ wo honam ani ho ban na amma woasiw sɛnkyerɛnne ahorow ano. Mpɛn pii no, aduru a wɔde siw owia ano nkutoo nnɔɔso. Ade a eye sen biara a ɛsɛ sɛ woyɛ ne sɛ wobɛkwati sɛ wobɛtra owia mu sɛnea wubetumi biara. Sɛ ɛsɛ sɛ wokɔ owia mu koraa a, hyɛ ntade a ɛbɔ wo ho ban fi owia ho. Wo duruyɛfo betu wo fo wɔ nea eye ma wo ho. Ebia ɛho behia nso sɛ wokwati hann ahorow bi a wɔde nsa ayɛ.

Ayaresa a wɔde sa EPP/XLP

Ebia wo duruyɛfo bɛkyerɛw aduru bi a wɔfrɛ no afamelanotide ama wo. Eyi yɛ ade ketewaa bi a wɔde hyɛ nipadua mu. Wo duruyɛfo de bɛto wo yafunu mu were no ase. Aduru a wɔde fi nipadua no mu no bɛboa ma ɛyaw a owia a wɔde hyɛ nipadua mu de ba no so atew. Wo duruyɛfo nso bɛhwɛ wo mmerɛbo dwumadi so daa na wahwɛ ahu sɛ vitamin D dodow a ɛwɔ wo mu no so ntew.

Nnipa a wɔwɔ EPP/XLP no taa nya akisikuru. EPP/XLP ayarefo binom nso betumi anya mmerɛbo mu yare. Wɔ tebea horow a emu yɛ den mu no, mmerɛbo no betumi ayɛ adwuma yiye. Ebia eyi bɛhwehwɛ sɛ wɔde mmerɛbo foforo ma, na ɛtɔ mmere bi a wɔde nnompe mu ntini foforo ma akyi.

Ayaresa a wɔde sa PCT

Ebia oduruyɛfo no bɛkyerɛw nneɛma te sɛ:

• Phlebotomy : Sɛ woyi mogya kakraa bi fi nipadua no mu daa a, eyi dade a ɛboro so fi nipadua no mu. Eyi betumi aboa sɛ dade a ɛboro so no resiw heme a wɔyɛ no kwan a.
• Hydroxychloroquine a wɔmfa nni dwuma pii: Saa aduru yi boa ma woyi porphyrin a ɛboro so fi wo nipadua mu. Wɔtow gu wo nsu mu.

CEP a wɔde sa yare

Ɛho hia yiye sɛ wobɛbɔ wo ho ban afi owia hann ne fluorescent akanea ho na amma akisikuru ne nsɛnnennen biara amma. Sɛ akisikuru ba a, ebia ɛho behia sɛ wɔde nnuru a ekum ɔyare mmoawa di dwuma. Sɛ ɔyare no mu yɛ den a, ebia ɛho behia sɛ wɔnom nnuru a wɔde kum ɔyare mmoawa a wɔnom anaa wɔde fa IV mu.

Wo duruyɛfo bɛhwɛ wo haemoglobin dodow so daa ahwɛ sɛ ɛho hia sɛ wɔma wo mogya anaa. Wɔ wɔn a yare no aka wɔn kɛse no fam no, ebia ɛho behia sɛ wɔde nnompe mu ntini foforo ma wɔn. Eyi nkutoo ne ɔkwan a wɔfa so sa yare no.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ wowɔ porphyria a, wo duruyɛfoɔ a wobɛkɔ akɔhwɛ wo daa (``follow-ups'') bɛyɛ w'abrabɔ fã. Wo duruyɛfo bɛkyerɛ wo bere a ɛsɛ sɛ woba wo bere a wɔahyɛ sɛ wobɛkɔ no. Saa nhyehyɛe ahorow yi ho hia na ama woatumi ahwɛ sɛnea yare no reka wo nipadua no na woahu nsɛnnennen ho sɛnkyerɛnne biara. Ɛho behia sɛ wɔyɛ mogya ne nsu mu nhwehwɛmu daa, ne nhwehwɛmu a wɔbɛyɛ de ahwɛ sɛnea akwaa afoforo yɛ adwuma.

Daakye bɛn na obi a ɔwɔ porphyria betumi ahwɛ kwan?

W’adwene gyina nneɛma yi so:

• Porphyria ko a wowɔ
• Hwɛ sɛnea aka wo kɛse
• Nsɛnnennen biara a ɛbɛsɔre

Wo duruyɛfo betumi ama woanya nea wobɛhwɛ kwan wɔ w’anim no ho adwene a edi mu sen biara.

Porphyria betumi anya wo da biara da asetra so nkɛntɛnso kɛse. Nsɛnkyerɛnne a emu yɛ den betumi asiw wo dwumadi ahorow a wotaa yɛ no kwan, na ama ayɛ den sɛ wobɛyɛ adwuma, ahwɛ w’abusua, na w’ani agye nneɛma a wode gye w’ani ho. Ebia wobɛte nka sɛ woabrɛ paa, wɔ nipadua ne adwene nyinaa mu.

Ka sɛnea wote nka kyerɛ wo duruyɛfo. Obetumi aka nneɛma ne mmoa akuw a wobetumi aboa wo ho asɛm akyerɛ wo. Sɛ wo ne porphyria mpɔtam hɔfo di nkitaho a, ebetumi aboa wo ma woasua wo tebea no ho ade pii na woanya akwan a wobɛfa so adi ho dwuma.

Nyarewa a ɛntaa nsi te sɛ porphyria a wobɛtra ase no betumi ayɛ ankonam, abasamtu, na ɛyɛ hu koraa. Ebia wobɛpɛ sɛ w’abusuafo ne wo nnamfo te sɛnea wote nka no ase. Ebia na ɛsɛ sɛ wokyerɛkyerɛ wo porphyria no mu kyerɛ afoforo na wubisa wɔn nea enti a wuntumi nyɛ nneɛma bi.

Baabiara a wowɔ no, hu sɛ ɛnyɛ wo nko ara.Ɛwom sɛ porphyria ho yɛ na sɛ wɔde toto nyarewa afoforo pii ho de, nanso nnipa pii wɔ hɔ a wɔretwɛn sɛ wɔbɛboa wo. Kɔka akuw a wɔboa porphyria wɔ intanɛt so ho, anaa ka kyerɛ wo duruyɛfo sɛ ɔmfa wo nkɔ bi. Sɛ wunya nnipa a wɔte sɛnea wote nka no ase a, ebetumi ama ayɛ mmerɛw kɛse sɛ wubedi asetra mu nkɔanim ne ɔhaw ahorow ho dwuma.

Ade a ɛho hia sen biara a ɛsɛ sɛ yesua fi eyi mu (Take-Home Message) .

Porphyria yɛ yare a emu yɛ den na ɛntaa nsi. Nanso, sɛ wɔasua wo ho ade, di wo duruyɛfo afotu akyi, na wokwati nneɛma a ɛma wo sɛnkyerɛnne no mu yɛ den a, wubetumi ne tebea no atra ase yiye. Ɛho hia sɛ wohwɛ wo nipadua, wo sɛnkyerɛnne ahorow, na wokɔ wo duruyɛfo nkyɛn bere ano. Kae sɛ ɛnyɛ wo nko ara, na ntwentwɛn wo nan ase da sɛ wobɛsrɛ mmoa.

` Porphyria, heme, porphyrin, enzymes, awosu mu nyarewa, honam ani nyarewa, yafunu mu yaw