So wonna anadwo nso? So wototo wo ho na wodannan wo ho nnɔnhwerew pii bere a woada akyi? Eyi yɛ ɔhaw a abu so wɔ yɛn mu pii mu ankasa. Nanso, so woasusuw pɛn sɛ nna a wontumi nna yi betumi ayɛ awosu mu yare bi a wɔntaa nhu koraa a ebetumi ama obi awu mpo ho sɛnkyerɛnne a edi kan? Mma ɛnhaw wo, menka sɛ wowɔ saa yare yi. Efisɛ eyi yɛ yare a ɛntaa nsi koraa wɔ wiase. Nanso ɛho hia paa sɛ yehu eyi. Enti ɛnnɛ yɛbɛka yareɛ a ɛyɛ hu na ɛntaa nsi koraa yi ho asɛm.
Dɛn ne Abusua mu Dae a Ekum Nnipa (FFI)?
Ɛmfa ho sɛ asɛmfua "Insomnia" wɔ ne din mu no, FFI nyɛ nna mu haw ankasa.
FFI yɛ awosu mu yare a ɛntaa nsi a ɛnam awosu mu nkwaadɔm so fi awo ntoatoaso so kɔ awo ntoatoaso so. Sɛ yɛbɛka no tiawa a, ɛyɛ tebea a protein bi a ɛwɔ yɛn amemene mu a ɛsakra na ɛsɛe amemene no de ba. Yare no ho yɛ na araa ma abenfo ka sɛ nnipa bɛyɛ 100 pɛ na wɔabɔ amanneɛ wɔ mmusua bɛyɛ 30 mu wɔ wiase nyinaa sɛ wɔwɔ awosu mu abɔde a ɛde yare yi ba. Enti sɛ wuntumi nna a, hokwan a ɛwɔ hɔ sɛ efi FFI no sua koraa, bɛyɛ ɔpepem biako biara mu biako. Afei nso, yare koro no ara a ɛnyɛ awosu mu nneɛma bi nso wɔ hɔ, a ɛkyerɛ sɛ ɛba mpofirim a ntease biara nnim. Wɔfrɛ no Sporadic Fatal nna a wontumi nna (sFI). Abenfo da so ara nnim ɔkwan pɔtee a ɛkɔ so.
Dɛn nti na saa yare yi ba? Dɛn na ɛde ba?
Nea ɛde FFI ba titiriw ne nkwaadɔm mu nsakrae a ɛba awosu mu abɔde a nkwa wom `PRPN` mu wɔ yɛn nipadua mu. Saa awosu mu abɔde yi na ɛhwɛ protein bi a wɔfrɛ no `PrP` a wɔyɛ so. Ɛwom sɛ wonhuu sɛnea protein yi yɛ adwuma pɛpɛɛpɛ de, nanso nsakrae a ɛba awosu mu nkwaadɔm no mu no ma wɔyɛ protein yi wɔ ɔkwan a ɛnteɛ so. Afei ɛbɛyɛ awuduru ma yɛn nipadua.
Susuw ho hwɛ, sɛ sɛ́ anka yɛde bɔɔl a ne nsusuwii teɛ bɛhyɛ afiri bi mu no, yɛde bɔɔl a ɛsono ne nsusuwii bɛhyɛ afiri mu a, anka afiri no bɛkyere na abubu. Eyi ne nea ɛkɔ so wɔ ha nso.
Saa protein ahorow a awuduru wom a ɛnteɛ yi boaboa ano nkakrankakra wɔ yɛn nkwa nna nyinaa mu wɔ amemene no fã bi a wɔfrɛ no
thalamus . Thalamus yɛ beae a ɛhwɛ nneɛma a ɛho hia pii so, te sɛ yɛn nna, aduan ho akɔnnɔ, ne yɛn nipadua mu hyew. Bere kɔ so no, sɛ protein ahorow a awuduru wom yi sɛe thalamus no a, ɔsɛe no ma FFI ho sɛnkyerɛnne ahorow no da adi. Wɔde saa awosu mu nsakrae yi fa awo ntoatoaso ahorow mu wɔ ɔkwan a
ɛyɛ autosomal dominant so. Eyi kyerɛ sɛ ɛsɛ sɛ wunya awosu mu abɔde a wɔayɛ no foforo no biako fi wo maame anaa wo papa hɔ na ama woanya yare no.
Sɛ w’awofoɔ mu baako wɔ saa gene mutation yi a, wowɔ hokwan 50% sɛ wo nso wobɛnya bi. Dɛn ne yare yi ho sɛnkyerɛnne?
Mpɛn pii no, FFI ho sɛnkyerɛnne fi ase da adi
bere a wɔadi mfe 40 kosi 60. Ɛwom sɛ efi ase yɛ mmerɛw koraa de, nanso saa sɛnkyerɛnne ahorow yi betumi ayɛ kɛse ntɛm ara. Momma yɛnhwɛ nea saa sɛnkyerɛnne ahorow yi yɛ.
Nsɛnkyerɛnne a edi kan Nsɛnkyerɛnne a ɛba bere a yare no akɔ anim akyi
| Nsɛnkyerɛnne no su | Nkyerɛmu |
|---|
| Asoroben | Nsɛnkyerɛnne titiriw ne nea edi kan koraa ne sɛ bere kɔ so no, nna a wontumi nna no mu yɛ den. |
| Adwene a ɛyɛ basaa ne ɛyɛ den sɛ wode w’adwene besi biribi so | Nkae ne nsusuwii a asɛe esiane amemene a asɛe nti. |
| Nsakrae a ɛba nipadua mu | Mogya mmoroso, koma bɔ ntɛmntɛm, mu duru a ɛso tew a wontumi nkyerɛkyerɛ mu, fifiri a ɛboro so (Hyperhidrosis) , ne nipadua mu hyew a wontumi nni so. |
| Aniwa so haw ne adaeso ahorow | Anisoadehu mmɔho abien ne adaeso a emu da hɔ yiye, a ɛyɛ nwonwa ɛmfa ho mpo sɛ ɛntaa mma sɛ wobɛda de. |
| Ataxia a ɛma obi nya ɔyare | Sɛ obi ntumi nni nipadua no kankan so, te sɛ nea ɔhinhim bere a ɔnam. |
| Adwene mu su ahorow | Nneɛma a wontumi nhu a wuhu (Hallucinations), adwene a ɛyɛ basaa kɛse (Delirium). |
| Ɛyɛ den sɛ wobɛmene ade (Dysphagia) . | Aduan anaa nsu a wontumi nmene. |
Nsɛnkyerɛnne yi mu pii fi nna a wonnya ne amemene a ɛsɛe nyinaa. Awerɛhosɛm ne sɛ, ayarefo dodow no ara wuwu wɔ komayare anaa tebea afoforo a ɛde ɔyare mmoawa ba mu
wɔ asram 6 kosi 36 (mfe 3) akyi bere a yare no ho sɛnkyerɛnne no fii ase no.
Ɔkwan bɛn so na oduruyɛfo hu yare yi?
Sɛ wo duruyɛfo susuw sɛ wowɔ FFI a, wɔbɛyɛ nhwehwɛmu pii de asi so dua.
- Abusua aduruyɛ ho abakɔsɛm a wobebisa: Esiane sɛ eyi yɛ awosu mu yare nti, ɛho hia yiye sɛ wuhu sɛ ebia abusua no mu obi anya yare no ho sɛnkyerɛnne a ɛte saa ara anaa.
- Nipadua mu nhwehwɛmu : Wɔhwehwɛ yare no ho sɛnkyerɛnne ahorow mu yiye.
- Awosu mu nhwehwɛmu : Wɔsɔ mogya bi hwɛ sɛ ebia nsakrae bi aba `PRPN` awosu mu nkwaadɔm mu anaa.
- Dae Ho Adesua: Wɔhwɛ sɛnea obi da wɔ nnwinnade titiriw bi ase wɔ ayaresabea.
- Akyi berɛmo mu Nsuo Sɔhwɛ: Amemene mu nsuo a wɔayi afiri akyi berɛmo mu nhwehwɛmu .
Wɔ tebea horow bi mu no, sɛ obi wu ansa na wɔahu yare no a, wobetumi asi FFI so dua denam amemene no mu nhwehwɛmu a wɔbɛyɛ bere a wɔreyɛ funu no so.
Dɛn ne ayaresa ahorow a wɔde sa eyi?
Ɛho hia sɛ yɛka no pefee sɛ mprempren aduru biara nni wiase a ebetumi asa yare yi koraa.
FFI ayaresa twe adwene si yare no ho sɛnkyerɛnne ahorow a wobedi so na wɔama ɔyarefo no ahotɔ pii sɛnea ɛbɛyɛ yiye biara so. Esiane sɛ ɛyɛ yare a ɛntaa nsi koraa nti, ayaresa nhyehyɛe biara nni hɔ a wɔahyɛ da ayɛ. Akwahosan ho adwumayɛfo kuw bi a ntini ho ɔyaresafo ne adwenemyare ho oduruyɛfo ka ho bɛbom ahwɛ ayaresa a ɛfata ma ɔyarefo no. Mpɛn pii no, nea ɛka ayaresa ho ne:
- Vitamin ne aduannuru foforo a wɔde bɛma.
- Aduan a ɛkari pɛ a wɔde bɛma.
- Sɛ worenom nnuru afoforo a ɛma wo yare no ho sɛnkyerɛnne no mu yɛ den a, gyae anaa sesa .
- Nnuru bi a ɛma obi dwo anaa melatonin a wɔde ma de boa ma ɔda, sɛnea oduruyɛfo kyerɛ no.
- Afotu a wɔde bɛma wɔ adwene mu ahotɔ ho .
Nea ɛbɛyɛ na yare no antrɛw ankɔ abusua no mufo afoforo so daakye no, oduruyɛfo no betumi atu abusua no mufo nyinaa fo sɛ wɔnkɔyɛ awosu mu nhwehwɛmu.
Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?
Mesan ka bio, sɛ wowɔ ɔhaw wɔ nna mu a, hokwan a ɛwɔ hɔ sɛ ɛbɛyɛ FFI no sua koraa, yiye. Enti mma ɛnhaw wo wɔ ho a ɛho nhia.
Nanso, sɛ ɛyɛ den ma wo sɛ wobɛda bere tenten, anaa sɛ wowɔ sɛnkyerɛnne afoforo te sɛ adwene a ɛyɛ basaa anaa ɛyɛ den sɛ wobɛnantew ka wo nna a wuntumi nna ho a,
kɔ wo duruyɛfo nkyɛn ntɛm ara. Efisɛ ebia saa sɛnkyerɛnne ahorow no nyɛ FFI, nanso ebetumi ayɛ tebea foforo a wobetumi asa ho sɛnkyerɛnne. Enti, ɛho hia yiye sɛ wubehu yare no yiye na wohwehwɛ ayaresa a ɛho hia.
Nkrasɛm a Wɔde Kɔ Fie
- Fatal Familial Insomnia (FFI) nyɛ nna mu ɔhaw a ɛtaa ba. Ɛyɛ awosu mu yare a ɛntaa nsi koraa a ekum nnipa a ɛsɛe amemene no.
- Sɛ mpo ɛyɛ den ma wo sɛ wobɛda a, hokwan a ɛwɔ hɔ sɛ ɛbɛyɛ FFI no sua koraa, enti mma wo ho nnyɛ wo hu a ɛho nhia.
- Saa yare yi ho sɛnkyerɛnne titiriw ne nna a wontumi nna, na bere kɔ so no, emu yɛ den. Afei nso , ntini mu sɛnkyerɛnne te sɛ kankyee mu haw ne adwene a ɛyɛ basaa nso ba.
- Sɛ obi wɔ w’abusua mu wɔ FFI na worenya nna ho haw a, hwehwɛ aduruyɛfo afotu ntɛm ara.
- Sɛ wowɔ nna ho haw biara a ɛtra hɔ kyɛ anaa sɛnkyerɛnne afoforo a ɛfa ho a, hwɛ hu sɛ wobɛkɔ wo duruyɛfo nkyɛn na wabu tebea afoforo a wobetumi asa no afi hɔ.
Fatal Family Insomnia, FFI, nna a wontumi nna, awosu mu nyarewa, prion yare, nna ho haw, amemene mu nyarewa
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