Dɛn ne Porphyria? Nsuro, momma yɛnka ho asɛm tiawa.

So woanya ɔhyew anaasɛ wo honam ani akisikuru a wuntumi nnyina ano pɛn bere a worekɔ owia mu no? Anaasɛ woanya yafunu mu yaw kɛse a ɛma wo ho yɛ wo yaw na wofe a ntease biara nnim pɛn? Saa sɛnkyerɛnne ahorow yi yɛ soronko wɔ nyarewa a ɛtaa nya ho . Ebia yare bi a ɛntaa nsi koraa a nnipa pii nte ho asɛm mpo wɔ eyi akyi. Ɛno yɛ yare a ɛte saa no biako a wɔfrɛ no Porphyria . Mma wo ho nnsuro sɛ wote saa din yi a. Ɛnnɛ, yɛbɛka eyi ho asɛm wɔ ɔkwan a ɛnyɛ den koraa so na mobɛte ase.

Sɛ yɛbɛka no tiawa a, dɛn ne Porphyria?

Sɛ yɛbɛte eyi ase a, momma yenni kan nsusuw adwumayɛbea ketewaa bi a ɛwɔ yɛn nipadua mu ho. Protein bi wɔ hɔ a ɛho hia yiye a ɛma yɛn mogya mu nkwammoaa kɔkɔɔ no kɔla kɔkɔɔ na ɛde mframa pa fi ahurututu mu kɔ nipadua no fã a aka no mu. Wɔfrɛ no ‘heme’. Wɔyɛ saa heme yi wɔ yɛn nipadua mu wɔ anammɔn ahorow a ɛtoatoa so mu, te sɛ nhama a wɔde boaboa nneɛma ano. Te sɛ nea wɔreboaboa kar ano ara pɛ. Wɔ anammɔn biara mu no, protein soronko bi a wɔfrɛ no enzymes yɛ adwuma.

Afei susuw ho hwɛ, sɛ anammɔn biako a ɛwɔ saa nneɛma a wɔyɛ yi mu no kyekyere, anaasɛ odwumayɛni biako (enzyme) anyɛ adwuma yiye a, dɛn na ɛba? Wontumi nyɛ anammɔn a edi hɔ no, na nneɛma a wɔde yɛ no boaboa ano, ɛnte saa? Saa ara nso na sɛ sintɔ bi ba wɔ anammɔn bi a wɔde yɛ heme mu a, nnuru a wɔyɛ wɔ anammɔn a atwam no mu a wɔfrɛ no ‘porphyrins’ ne ‘porphyrin precursors’ no fi ase boaboa ano wɔ nipadua no mu. Ɛyɛ nnuru yi a ɛboaboa ano wɔ nipadua no mu na ɛde porphyria ho sɛnkyerɛnne ba. Eyinom betumi asɛe ntini anaa honam ani.

Dɛn ne porphyria ahorow atitiriw?

Wobetumi akyekyɛ porphyria mu akuw atitiriw abien, a egyina sɛnea sɛnkyerɛnne ahorow no da adi so. Sɛ wote saa nkyekyɛmu yi ase a, ɛbɛboa wo ma woate yareɛ no ase yie.

Nkyekyɛm nketewa afoforo pii wɔ saa ahorow atitiriw abien yi ase. Sɛ nhwɛso no, nea wɔfrɛ no `Acute Intermittent Porphyria (AIP)`, a ɛhyɛ porphyria a emu yɛ den ase, ne nea wɔfrɛ no `Porphyria Cutanea Tarda (PCT)`, a ɛhyɛ honam ani porphyria ase no ntaa mma.

Dɛn ne nneɛma a ɛkanyan yare no ho sɛnkyerɛnne ahorow no?

Ɛwom sɛ porphyria fi awosu mu de, nanso ɛnyɛ bere nyinaa na sɛnkyerɛnne ahorow no da adi. Nneɛma bi wɔ hɔ a ebetumi akanyan sɛnkyerɛnne ahorow, anaasɛ "ntua". Wɔfrɛ eyinom sɛ ‘triggers’. Eyinom a wobɛkwati no ne ɔkwan a eye sen biara a wobɛfa so adi yare no ho dwuma.

• Nnuru bi: Nnuru bi a wɔde kum ɛyaw , nnuru a wɔde siw awo ano, nnuru a wɔde kum akisikuru, ne nnuru bi a wɔde kum akisikuru betumi de eyi aba. Sɛ wowɔ porphyria a, akyinnye biara nni ho sɛ ɛsɛ sɛ wo ne wo duruyɛfo kasa ansa na woanom aduru biara.
• Nsa ne sigaretnom : Asanom bebrebe ne sigaretnom, titiriw bere koro mu, yɛ nneɛma atitiriw a ɛkanyan no.
• Adidi ne mmuadadi: Nneɛma te sɛ aduan a carb nnim , aduan a ɛyɛ katee a ɛma obi mu duru so tew, ne mmuadadi betumi akanyan yare no ho sɛnkyerɛnne.
• Adwene ne nipadua mu nhyɛso: Nneɛma te sɛ adwuma a wɔyɛ boro so, adwene mu adwennwen, ne oprehyɛn betumi aka no.
• Nyarewa: Nsɛnkyerɛnne betumi aba esiane adwennwen a yare anaa ɔyare mmoawa foforo biara de ba nipadua no so nti.
• Owia hann: Owia hann ne ade titiriw a ɛkanyan nnipa a wɔwɔ Cutaneous Porphyria.

Bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn? Ɔkwan bɛn so na wohu yare no?

Esiane sɛ saa yare yi ho yɛ na na ne sɛnkyerɛnne te sɛ nyarewa afoforo a ɛtaa ba nti, ɛtɔ mmere bi a ebetumi agye bere kakra ansa na wɔahu. Sɛ wowɔ sɛnkyerɛnne ahorow a edidi so yi bi a, ɛho hia sɛ wokɔ oduruyɛfo nkyɛn, titiriw sɛ ɛtaa ba a.

Wɔ porphyria a emu yɛ den mu

Sɛ wo yafunu mu yaw kɛse ka sɛnkyerɛnne ahorow te sɛ akisikuru, ɔfe, akisikuru, koma a ɛbɔ kɛse, mogya mmoroso, ntini mu mmerɛwyɛ, adwene a ɛyɛ basaa, anaa akisikuru a, ɛho hia yiye sɛ wobɛkɔ ayaresabea ntɛm ara, titiriw Emergency Treatment Unit (ETU), efisɛ ɛtɔ mmere bi a tebea yi betumi de wo nkwa ato asiane mu.

Wɔ Cutaneous Porphyria mu no

Sɛ wo honam ani nya akisikuru, akuru, ɔfe, ɛyɛ kɔkɔɔ, anaasɛ ɛyɛ mmerɛw bere a woakɔ owia mu akyi a, kɔ honam ani yare ho oduruyɛfo nkyɛn.

Ɔkwan a wɔfa so hu yare no:

1. Aduruyɛ mu nhwehwɛmu: Nea edi kan no, oduruyɛfo no bɛhwɛ wo, na wabisa wo wo yare no ho sɛnkyerɛnne ne w’abusua aduruyɛ ho abakɔsɛm yiye.

2. Nhwehwɛmu a wɔyɛ wɔ aduruyɛdan mu: Sɛ wosusuw sɛ wowɔ porphyria a, wɔbɛsɔ wo nsu, wo mogya, ne ebia wo nsu mu nhwehwɛmu ahwɛ sɛ porphyrin dodow te. Wɔ tebea horow a emu yɛ den mu no, nsu no betumi adan kɔla kɔkɔɔ anaa kɔkɔɔ tuntum.

3. Awosu mu Nhwehwɛmu: Sɛ wɔde aduruyɛdan mu nhwehwɛmu si yare no so dua a, wobetumi ayɛ awosu mu nhwehwɛmu de ahu awosu mu nsakrae pɔtee a ɛde bae.

Dɛn ne ayaresa a wobetumi apaw?

Ɛsono sɛnea wɔsa porphyria a, egyina yare no ko ne sɛnea sɛnkyerɛnne ahorow no mu yɛ den so. Ɛwom sɛ aduru biara nni hɔ de, nanso wobetumi asiw yare no ho sɛnkyerɛnne ahorow so na wɔatra ase sɛnea ɛsɛ.

Ayaresa a Wɔde Sa Acute Porphyria

Sɛ yare no ho sɛnkyerɛnne no mu yɛ den (ntua a emu yɛ den) a, ɛho hia sɛ wɔde obi kɔ ayaresabea na wɔsa no yare.

• Nkyene (IV nsu) a wɔde ma: Ma nipadua no nkyene ne nsu kari pɛ.
• Heme Injection: Di heme a enni nipadua no mu no so na tew porphyrin dodow a ɛboaboa ano no so.
• Glucose a wɔde ma: Asikre te sɛ dextrose a wɔde fa ntini mu anaasɛ wɔde ano ma no kyerɛ sɛ mmerɛbo no brɛ porphyrin a wɔyɛ no ase.
• Ayaresa afoforo: Nnuru a wɔde kum ɛyaw ma ɛyaw a emu yɛ den, ne nnuru a wɔde sa akisikuru ne ɔfe.
• Nnuru foforo te sɛ Givosiran (Givlaari): Wɔ nnipa a wɔtaa nya yare no ho sɛnkyerɛnne fam no, wɔde saa aduru yi ma ɔsram biara na ama wɔatew mpɛn dodow a ɛba ne sɛnea emu yɛ den so.

Ayaresa a Wɔde Sa Cutaneous Porphyria

• Owia ho ahobammɔ:Eyi ne ade a ɛho hia sen biara. Nsa atenten a wobɛhyɛ, kyɛw, ne aduru a ɛbɔ wo ho ban a ɛbɔ owia ho ban a ɛkorɔn te sɛ SPF 50+ a wode bɛhyɛ wo ho no ho hia.
• Phlebotomy: Eyi hwehwɛ sɛ woyi mogya kakraa bi (bɛyɛ unit biako) fi nipadua no mu adapɛn kakraa biara. Eyi boa ma dade a ɛboro so a ɛwɔ nipadua no mu no so tew na ɛboa ma woyi porphyrins a aboaboa ano wɔ mmerɛbo no mu no fi hɔ.
• Nnuru: Nnuru a ɛba fam te sɛ chloroquine anaa hydroxychloroquine betumi ama wɔayi porphyrins afi mmerɛbo no mu ntɛmntɛm.