Dɛn ne Prion Yare? Momma yɛnhwehwɛ nea ɛyɛ pɛpɛɛpɛ!

So woate ‘Prion Yare’ yi ho asɛm pɛn? Ebia saa din yi yɛ foforo kakra ma wo. Nea enti a ɛte saa ne sɛ eyi yɛ nyarewa kuw bi a wɔn ho yɛ na koraa wɔ wiase. Nanso, ɛho hia paa sɛ yɛma yɛn ani da hɔ wɔ saa yare yi ho, efisɛ ɛka yɛn amemene no tẽẽ na yare no ho sɛnkyerɛnne no mu yɛ den ntɛm ara. Ebetumi aba nnipa ne mmoa nso mu.

Dɛn ne Prion Yare?

Sɛ yɛbɛka no tiawa a, prion nyarewa yɛ nyarewa kuw bi a protein a ɛteɛ a ɛwɔ yɛn amemene mu a ɛsakra wɔ ɔkwan a ɛnteɛ so ma ɛbɛyɛ protein a epira a wɔfrɛ no ``prions.'' Fa no sɛ nkwammoaa pa bi a ɛwɔ yɛn nipadua mu a ɛdan nkwammoaa bɔne a ɛsakra mpofirim.

Sɛ prion protein ahorow a ɛnteɛ yi boaboa ano wɔ amemene no mu a, efi ase sɛe amemene mu nkwammoaa. Sɛ yɛbɛka no pɛpɛɛpɛ a, eyi yɛ yare a ɛma ntini mu yare . Bere kɔ so no, saa ɔsɛe yi betumi ama amemene no dwumadi so atew nkakrankakra, na ɛde tebea horow te sɛ adwenemhaw aba . Eyi kyerɛ sɛ wokae ade, ɛyɛ den ma wo sɛ wubesusuw nneɛma ho, na wuntumi nyɛ w’adwuma. Ade a ɛyɛ hu sen biara wɔ yare yi ho ne sɛ saa sɛnkyerɛnne ahorow yi fi ase mpofirim na ɛkɔ so ntɛmntɛm. Saa yare yi ka nnipa bɛyɛ ɔpepem biako biara mu biako wɔ wiase nyinaa. Eyi kyerɛ sɛ ɛntaa nsi koraa.

Prion nyarewa yɛ nyarewa a ɛyɛ awerɛhow a ekum nnipa . Eyi kyerɛ sɛ, sɛ yare no sɔre pɛ a, ɛyɛ den sɛ wɔbɛsa. Nnuruyɛfo bɔ mmɔden sɛ wobedi sɛnkyerɛnne ahorow no so na wɔama ɔyarefo no ho atɔ no sɛnea wobetumi. Wɔboa ɔyarefo no nso ma ogyina nkɛntɛnso a yare no nya wɔ n’asetra, n’abusua, ne wɔn a wɔhwɛ wɔn so no ano.

Ɔkwan bɛn so na prion nyarewa yi sɔre?

Akwan titiriw abiɛsa na obi betumi afa so anya prion yare.

1. Ɛdenam awosu mu nsakrae a wonya fi awo mu (wɔfrɛ eyi abusua prion yare) so.

2. Ɛnam ɔyare mmoawa so (wɔfrɛ eyi prion yare a wonya).

3. Nanso, mpɛn pii no, yare yi ba a enni awosu mu ade biara anaasɛ abɔnten so ɔyare mmoawa biara . Nhwehwɛmufo ne nnuruyɛfo frɛ eyi sɛ ‘prion yare a ɛba bere ne bere mu’.

Afei ma yɛnhwɛ akwan yi mu biara mu nkɔ akyiri kakra.

Prion Nyarewa a Ɛba Bere ne Bere Mu

Eyi ne prion yare a abu so sen biara. Nea ɛkɔ so wɔ ha ne sɛ, esiane ntease biara a ɛda adi nti, protein ahorow a ɛfata a ɛwɔ amemene no mu no dan ‘prion’ bɔne a yɛadi kan aka ho asɛm no mpofirim. Wɔda so ara nnim nea enti a eyi ba ankasa.

Nyarewa atitiriw a ɛka saa kuw yi ho ne:

• Creutzfeldt-Jakob yareɛ (CJD) : Eyi na ɛma prion nyarewa a ɛba bere ne bere mu no 85%. Ɛyɛ nea wɔtaa de di dwuma sen biara.
• Dae a wontumi nna a edi awu a ɛba bere ne bere mu: Eyi ho yɛ na yiye. Ɛho yɛ na mpo sen awosu mu nna a wontumi nna a edi awu.
• Variable protease-sensitive prionopathy : Eyi nso yɛ prion yare a ɛntaa nsi koraa a ɛba bere ne bere mu.

Abusua mu Prion Nyarewa

Saa prion yare yi fi nkwaadɔm mu nsakrae a ɛba awosu mu abɔde bi a wɔfrɛ no `PRNP` mu wɔ yɛn awosu mu nkwaadɔm mu. Yare no betumi aba sɛ ebia nkwaadɔm mu nsakrae no fi ɛna anaa agya no hɔ (wɔfrɛ eyi `autosomal dominant inheritance`). Wɔahu nkwaadɔm mu nsakrae ahorow bɛboro 50 wɔ `PRNP` awosu mu abɔde mu, na nkwaadɔm mu nsakrae biara betumi de awosu mu prion yare ahorow aba.

Nyarewa bi a ɛka saa kuw yi ho:

• Abusua mu Creutzfeldt-Jakob yare (CJD) .
• Gerstmann-Sträussler-Scheinker (GSS) yare : Eyi ho yɛ na yiye. Ɛba nnipa biako kosi du wɔ nnipa ɔpepem 100 mu wɔ wiase nyinaa.
• Abusua mu nna a wontumi nna a edi awu : Eyi ho yɛ na mpo sen GSS yare no. Mmusua 50 kosi 70 pɛ na wokura awosu mu nsakrae a ɛde yare yi ba no.
• Wɔabɔ prion nyarewa afoforo a ɛnam nkwaadɔm mu nsakrae a ɛba PRNP awosu mu abɔde mu so nso ho amanneɛ. Sɛ nhwɛso no, Britania abusua bi mufo 11 nyaa yare no ho sɛnkyerɛnne te sɛ akisikuru, nkate mu ntini mu yare, akisikuru, ne adwenemhaw. Saa tebea yi a wɔfrɛ no autonomic neuropathy no ka nneɛma te sɛ wo mogya mmoroso, wo koma bɔ, ne wo dwensɔtwaa ne wo nsu a wuntumi nhyɛ mu.

Prion Yare a Wonya

Eyi ne ɔkwan a ɛnyɛ nea wɔtaa fa so nya prion nyarewa. Ɛha yi, obi betumi anya yare no denam aduan a prions ayɛ mu fĩ a obedi so (sɛ nhwɛso no, nam a efi nantwi a ɔwɔ ‘nantwi a wabɔ dam yare’) anaasɛ ɔde aduruyɛ nnwinnade a efĩ wom a ɔde bedi dwuma (sɛ nhwɛso no, bere a wɔreyɛ oprehyɛn no).

• Kuru ne obi a odi kan a ɔwɔ saa ntini mu yare a ɛde ɔyare mmoawa ba yi a wɔahu na wɔayɛ ho nhwehwɛmu. Wohuu no wɔ Forefo a wɔwɔ Papua New Guinea no mu. Wogye di sɛ wɔnam wɔn amanne ahorow bi (te sɛ nnipa nam a wodi) so trɛwee.

Nea eye ne sɛ, nnɛyi akwan a emu yɛ den a wɔfa so siesie nnuruyɛ mfiri ne sɛnea wɔhwɛ aduan a ahobammɔ wom so no ama prion nyarewa a ɛtrɛw wɔ saa kwan yi so no so atew kɛse.

Dɛn ne prion nyarewa a ɛyɛ hu sen biara?

In fact, all prion diseases are fatal . Ɛne sɛ, sɛ wunya yare no a, akyinnye biara nni ho sɛ wubewu. Mpɛn pii no, ɔyarefo no wu wɔ asram kakraa bi kosi mfe abiɛsa akyi bere a prion yare ho sɛnkyerɛnne afi ase no.

Dɛn ne prion nyarewa ho sɛnkyerɛnne?

Prion yare no ho sɛnkyerɛnne betumi ayɛ soronko, a egyina prion yare no ko ne amemene no fã a ɛka no so. Nanso mpɛn pii no, nnipa a wɔwɔ prion nyarewa no nya ɔhaw ahorow wɔ wɔn ntini mu a ɛyɛ den nkakrankakra.

Eyinom ne sɛnkyerɛnne ahorow a ɛtaa ba no bi:

• Ɛyɛ den sɛ wobɛnantew, ɛyɛ nwonwa (`Ataxia`) .
• Ɔkasa a ɛyɛ den, nsɛmfua a wɔde di dwuma ɔkwammɔne so (`Aphasia`) .
• Adwene a ɛyɛ basaa, adwene a ɛyɛ basaa
• Asoroben
• Nkae a ɛyera, ɛyɛ den sɛ obesusuw nneɛma ho na asi gyinae
• Nneɛma a ɛkɔ brɛoo anaasɛ ntini a ɛyɛ den (`Hypokinetic movement disorders`) .
• Mpofirim ara a ɛwosow, ɛwosow (`Myoclonus`) .
• Nipasu mu nsakrae (sɛ nhwɛso no, obi bo fuw, ne ho yɛ basaa) .
• Adwene mu haw ahorow (e.g. dadwen, adwenemhaw, ne ɛtɔ mmere bi mpo a aniwa so nsusuwii hunu) .

Fa no sɛ, obi a ɔbɛn wo paa fi ase sakra mpofirim. Ne werɛ fi nneɛma, ontumi nkasa sɛnea na ɔyɛ kan no, ɛyɛ den ma no sɛ ɔbɛnantew. Sɛ wuhu saa sɛnkyerɛnne ahorow yi a, ɛhaw wo dɛn?

Dɛn ne nsɛnnennen a efi prion nyarewa mu ba?

Wobetumi de prion nyarewa mu nsɛnnennen no abata wɔn ho wɔn ho ho, na ɛde nsɛnnennen a ɛtoatoa so aba a ɛnyɛ ɔyarefo no nko na ebetumi anya nkɛntɛnso kɛse, na mmom abusua ne nnamfo a wɔhwɛ wɔn nso so.

Saa nsɛnnennen yi betumi aba wɔ asram kakraa bi anaa afe biako mu bere a yare no ho sɛnkyerɛnne afi ase no:

• Obi a ontumi nyɛ n’ankasa adwuma
• Dementia ( ɛkame ayɛ sɛ obi ntumi nkae ade koraa) .
• Kasa a wontumi nkasa (`Mutism`) .
• Coma (eyi ne ade a etwa to) .

Sɛ obi nya prion yare a, ɔde ne ho to afoforo so ntɛm ara. Abusua ne afoforo wɔ hɔ sɛ wɔbɛhwɛ wɔn na wɔaboa wɔn. Saa bere yi nyinaa, sɛ wɔbɛhwɛ sɛnea wɔn dɔfo no nkae ade, ontumi nkasa, na wɔn nipasu sakra a, ɛyɛ adwennwen kɛse ma wɔn a wɔhwɛ wɔn no.

Dɛn nti na prion nyarewa yi ba?

Eyi yɛ nyansahu mu asɛm kakra, nanso mɛka no tiawa. Prion nyarewa fi ase bere a prion protein a ɛfata a ɛwɔ yɛn amemene mu (a wɔfrɛ no `PrPc`) dan protein a ɛnyɛ ne kwan so, a wɔabobɔw no yiye (a wɔfrɛ no `PrPSc` - ɛno ne prion).

Saa prions a ɛnyɛ ne kwan so (`PrPSc`) yi fi ase boaboa ano, anaasɛ ɛkyekyere, prion protein a ɛteɛ (`PrPc`), na ɛdan prions a ɛnyɛ ne kwan so. Ɛte sɛ nea wobɛtow apɔw-mu-teɛteɛ bɔne agu apɔw-mu-teɛteɛ pa a wɔaboaboa ano mu, na nea aka no nso bɛkɔ bɔne. Bere kɔ so no, prion protein ahorow a ɛnteɛ yi sɛe ntini mu nkwammoaa a ɛwɔ amemene no mu no anaasɛ ɛsɛe no. Ɛno ne bere a wuntumi nnantew, nsusuw nneɛma ho, anaasɛ wuntumi nkasa yiye.

Ɔkwan bɛn so na wohu prion yare?

Nnuruyɛfo betumi de nhwehwɛmu a edidi so yi adi dwuma de ahu prion yare bi:

• Mogya mu nhwehwɛmu ne akyi berɛmo a wɔtow : Wɔhwehwɛ nnipa a wɔwɔ awosu mu nsakrae a ɛde prion nyarewa a wonya fi awo mu ba no mu hwɛ sɛ ebia biomarkers a ɛkyerɛ yare anaasɛ ɔsɛe wɔ wɔn mogya anaa amemene mu nsu (nsu a atwa amemene ne akyi berɛmo ho ahyia) mu anaa.
• Amemene mu MRI mfonini : Eyi tumi twa amemene no ho mfonini a emu da hɔ yiye, enti nnuruyɛfo betumi ahwehwɛ prion yare ho sɛnkyerɛnne.
• Electroencephalogram (EEG) : Eyi na ɛkyerɛ sɛnea amemene no mu anyinam ahoɔden yɛ adwuma.
• Real-time quaking-induced conversion assay (RT-QuIC) : Wɔ sɔhwɛ yi mu no, ɔyare ho animdefo hwehwɛ prions wɔ akyi berɛmo mu nsu mu. Eyi yɛ sɔhwɛ foforo koraa a ɛyɛ pɛpɛɛpɛ.

So aduru bi wɔ hɔ a wɔde sa prion nyarewa?

Nea ɛyɛ awerɛhow no, nnuruyɛfo nnya nhuu prion nyarewa ano aduru anaa aduru bi a ebetumi asiw yare no nkɔso ano. Prion nyarewa yɛ nyarewa a ɛde nkwa to asiane mu . Nnipa dodow no ara wuwu wɔ asram kosi mfe pii mu bere a wɔahu sɛ wɔanya prion yare no.

Ayaresa no twe adwene si ɔhwɛ a ɛma obi ho dwo so . Eyi kyerɛ sɛ wobedi yare no ho sɛnkyerɛnne ahorow so na wɔama ɔyarefo no ho atɔ no na onni ɛyaw biara sɛnea wobetumi. Sɛ nhwɛso no, nnuruyɛfo betumi akyerɛw nnuru ama nneɛma te sɛ:

• Nnuru a wɔde ko tia akisikuru anaa nnuru a ɛma ntini mu dwo ma myoclonus.
• Opioids a wɔde sa ɛyaw.

Daakye bɛn na obi a ɔwɔ prion yare no wɔ?

Saa bere yi de, wonni prion nyarewa ano aduru anaa ayaresa biara. Nanso, nhwehwɛmufo reyɛ eyi mu nhwehwɛmu wɔ akwan abien so, na ebetumi ama wɔahu yare no ntɛm na wɔasa wɔn yare daakye.

`RT-QuIC` sɔhwɛ a yɛadi kan aka ho asɛm no aboa ma wɔahu prion yare ansa na ɔsɛe a wontumi nsakra no aba amemene no so. Nhwehwɛmufo nso resua prions ho ade de ahwehwɛ akwan a wɔbɛfa so asiw protein ahorow a ɛnteɛ yi a ɛbɛhyehyɛ no ano na wɔasiw protein a ɛteɛ no ano na anyɛ nea ɛnteɛ.

Sɛ mewɔ prion yare a, dɛn na ɛsɛ sɛ meyɛ?

Esiane sɛ prion yare no kɔ so ntɛmntɛm nti, ebia ɛbɛyɛ den ama wo sɛ wobɛyɛ w’ankasa adwuma. Sɛ wowɔ saa yareɛ yi a, ɛyɛ adwene pa sɛ wobɛma ``advance directive'' awie.

Akwankyerɛ a wɔadi kan de ama no yɛ mmara kwan so nkrataa. Nhwɛso ahorow:

• Apɛgyadeɛ a ɛte aseɛ : Sɛ wowɔ yareɛ a enni sabea te sɛ prion yareɛ a, saa krataa yi bɛtumi akyerɛ deɛ wopɛ sɛ ɛto wo (e.g. sɛ wopɛ ayaresa bi anaasɛ wompɛ).
• Durable power of attorney for healthcare (DPA): Saa krataa yi betumi abɔ wɔn a ɔbɛyɛ w’akwahosan ho gyinaesi ahorow din sɛ wuntumi nkasa mma w’ankasa bio a.

Sɛ wudi kan yɛ nneɛma a ɛte sɛɛ ho nhyehyɛe a, wubetumi atew adwennwen a wo ne w’abusua behyia akyiri yi no so.

Na sɛ obi a ɔwɔ abusua no mu wɔ prion yare a wonya fi awo mu nso ɛ?

Ɛtɔ mmere bi a, nkurɔfo nya awosu mu nsakrae a ɛma asiane a ɛwɔ hɔ sɛ wobenya prion yare no yɛ kɛse. Awosu mu nhwehwɛmu betumi ahu sɛ wowɔ awosu mu nsakrae a ɛde prion yare ba no biako anaa nea ɛboro saa. Saa sɔhwɛ ahorow yi nso betumi akyerɛ asiane pɔtee a wowɔ.

Awosu mu nhwehwɛmu a wɔbɛyɛ de ahu yare bi a ekum nnipa no yɛ obi ankasa gyinaesi. Nnipa binom mpɛ sɛ wohu sɛ ebia wɔwɔ asiane mu anaa. Sɛ wopɛ sɛ woyɛ awosu mu nhwehwɛmu a, bisa oduruyɛfo ma ɔmma wo nkɔma wo. Sɛ nhwɛsoɔ no, wɔ Amerika no, ahyehyɛdeɛ te sɛ ``National Prion Disease Pathology Surveillance Center`` wɔ hɔ ma yei. Nnuruyɛfo atitiriw nso wɔ Sri Lanka a wobetumi de afotu ama wɔ asɛm yi ho.

Wobɛyɛ dɛn ahwɛ abusua muni bi a ɔwɔ prion yare?

Yare a ɛte sɛɛ betumi adan wo da biara da asetra nyinaa ayɛ basaa. Bere a woredi ahodwiriw ne awerɛhow a wubenya bere a wote sɛ wo dɔfo no wɔ yare a enni sabea ho dwuma no, ɛsɛ sɛ woyɛ nhyehyɛe nso sɛnea wobɛhwɛ w’abusua muni no. Nyansahyɛ ahorow bi ni:

• Yɛ ɔhwɛ nhyehyɛe : Ɛdenam wo tebea so no, ebia w’abusua muni behia ɔhwɛ wɔ baabi a wɔte, anaasɛ ebia obehia ɔhwɛ wɔ fie, a ɔde ne ho bɛto abusua, nnamfo, ne n’ankasa akwahosan ho aboafo so. Ebia wo dɔfo no behia mmoa wɔ da biara da nnwuma te sɛ aguaree a ɔbɛkɔ ne adidi mu.
• Yɛ nhyehyɛe ma wɔhwɛ wɔn wɔ ayaresabea : Prion yare yɛ yare a edi awu a ebetumi aba mpofirim na asɛe ntɛmntɛm. Sɛ wudi kan yɛ nhyehyɛe ma wɔhwɛ wɔn wɔ ayaresabea a, wo dɔfo no betumi anya bi bere a ohia no.
• Yɛ tebea a ɛhɔ yɛ komm : Nnipa a wɔwɔ prion nyarewa no taa te nka wɔ ɔkwan soronko so wɔ nsɛm a esisi mpofirim ho (sɛ nhwɛso no, sɛ wɔde wɔn nsa bɛka obi, wɔte dede kɛse, sɛ wɔbɛtra mmeae a nnipa ahyɛ hɔ ma). Ebia wɔn ho bedwiriw wɔn anaa wɔn bo afuw. Sɛ wɔhwɛ nneɛma a atwa wɔn ho ahyia so a, ebetumi aboa wɔn ma wɔn ho adwo wɔn na wɔagye wɔn ahome.

Wɔ bere a ɛte sɛɛ mu no, ɛho hia sɛ nea ɔhwɛ no no susuw wɔn ho nso. Wo nso wuhia ahomegye ne mmoa. Mmɔ mmɔden sɛ wo nkutoo wobɛsoa adesoa yi.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Mpɛn pii no, prion nyarewa ho sɛnkyerɛnne no mu yɛ den ntɛmntɛm. Sɛ wo anaa w’abusua mu obi wɔ prion yare a, ɛsɛ sɛ wo ne wo nnuruyɛfo yɛ adwuma de di yare no nkɛntɛnso ho dwuma.

Titiriw no, sɛ sɛnkyerɛnne ahorow a yɛadi kan aka ho asɛm (te sɛ nkae a ɛyɛ den, nantew a ɛyɛ den, kasa a ɛyɛ den, nipasu a ɛsakra) fi ase mpofirim na ɛyɛ kɛse ntɛmntɛm a, hwehwɛ aduruyɛfo afotu ntɛm ara.

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Sɛ wuhu sɛ wo anaa w’abusua mu obi wɔ prion yare a, ebetumi ayɛ ahodwiriw kɛse. Wo ne w’abusua wɔ nsɛnnennen pii mprempren, na pii bɛsɔre bere a yare no kɔ so no. Nsɛmmisa bi a ɛbɛboa wo ma woate nea wobɛhwɛ kwan ase ni:

• Wobɛyɛ dɛn ahu sɛ mewɔ prion yare bi?
• Prion yare bɛn na mewɔ?
• Ɛno ka me dɛn?
• Dɛn na ɛsɛ sɛ meyɛ akyi?
• Dɛn na metumi ayɛ de ahwɛ m’abusua muni?
• So ɛsɛ sɛ m’abusua no nya awosu ho afotu ne nhwehwɛmu?

Nea etwa to no, nneɛma a ɛsɛ sɛ yɛkae

Prion nyarewa yɛ nyarewa kuw bi a wɔntaa nhu, na edi awu a ɛka w’amemene. Prion ho sɛnkyerɛnne betumi aba mpofirim na asɛe ntɛmntɛm. Ebetumi ayɛ awerɛhow sɛ wubehu sɛ wo anaa obi a wodɔ no wɔ yare bi a enni ano aduru anaa aduru biara a ɛbɛtumi asiw ano.

Nanso, ayaresa ahorow bi wɔ hɔ a ɛbɛboa ma wɔadi yare no ho sɛnkyerɛnne ahorow ho dwuma . Na nhyehyɛe ne nnwuma ahorow wɔ hɔ a wɔde bɛboa wɔn a prion nyarewa aka wɔn ne wɔn a wɔhwɛ wɔn no. Sɛ wo anaa obi a wodɔ no wɔ prion yareɛ a, nsuro sɛ wobɛbisa nsɛm na woahwehwɛ mmoa. Ɛnyɛ wo nko ara.

` Prion Yare, Amemene Yare, Ntini mu Yare, Dementia, CJD, Awosu mu Yare