He aha ka maʻi Prion? E ʻike kākou i ke ʻano maoli o ia mea!

Ua lohe paha ʻoe i kēia 'Maʻi Prion'? Malia paha he mea hou iki kēia inoa iā ʻoe. ʻO ke kumu, he hui kēia o nā maʻi i laha ʻole ma ka honua. Eia nō naʻe, he mea nui loa ka makaʻala i kēia maʻi, no ka mea, pili pololei ia i ko kākou lolo a lilo koke nā hōʻailona i mea koʻikoʻi. Hiki ke loaʻa i loko o nā kānaka a me nā holoholona.

He aha ka maʻi Prion?

I ka ʻōlelo maʻalahi, ʻo nā maʻi prion kahi hui o nā maʻi i hoʻokumu ʻia e kahi protein maʻamau i loko o ko mākou lolo e loli maʻamau i nā protein ʻino i kapa ʻia ʻo "prions." E noʻonoʻo i kahi cell maikaʻi i loko o ko mākou kino e loli koke ana i kahi cell maikaʻi ʻole a loli.

Ke hōʻiliʻili kēia mau protein prion maʻamau ʻole i loko o ka lolo, hoʻomaka lākou e hōʻino i nā hunaola lolo. I ka pololei, he maʻi neurodegenerative kēia. I ka hala ʻana o ka manawa, hiki i kēia hōʻino ke alakaʻi i ka emi mālie ʻana o ka hana o ka lolo, e alakaʻi ana i nā kūlana e like me ka dementia . ʻO ke ʻano kēia e nalowale ana kou hoʻomanaʻo, pilikia ka noʻonoʻo ʻana, a ʻaʻole hiki ke hana i kāu hana. ʻO ka mea weliweli loa e pili ana i kēia maʻi, ʻo ia ka hoʻomaka koke ʻana o kēia mau hōʻailona a holomua koke. Hoʻopilikia kēia maʻi i hoʻokahi i loko o hoʻokahi miliona mau kānaka ma ka honua holoʻokoʻa. ʻO ke ʻano kēia he mea kakaikahi loa.

ʻO ka mea pōʻino, he mau maʻi make nā maʻi Prion. ʻO ke ʻano kēia, i ka wā e ulu ai ka maʻi, he paʻakikī ke ho'ōla. Hoʻāʻo nā kauka e kāohi i nā hōʻailona a hoʻoluʻolu i ka mea maʻi. Kōkua pū lākou i ka mea maʻi e hoʻomanawanui i ka hopena o ka maʻi i ko lākou ola, ko lākou ʻohana, a me ka poʻe e mālama ana iā lākou.

Pehea e ulu ai kēia mau maʻi prion?

ʻEkolu mau ala nui e hiki ai i ke kanaka ke hoʻomohala i kahi maʻi prion.

1. Ma ka hoʻoilina ʻana i kahi hoʻololi genetic (ua kapa ʻia kēia he maʻi prion familial).

2. Ma o ka maʻi (ua kapa ʻia kēia he maʻi prion i loaʻa).

3. Eia nō naʻe, i ka hapanui o ka manawa, hiki mai kēia maʻi me ke kumu ʻole o ka genetic a i ʻole ka maʻi o waho . Ua kapa ʻia kēia e nā kānaka noiʻi a me nā kauka he 'maʻi prion sporadic'.

I kēia manawa, e nānā kākou i kēlā me kēia o kēia mau ʻano hana me nā kikoʻī hou aku.

Nā maʻi Prion Sporadic

ʻO kēia ke ʻano maʻamau o ka maʻi prion. ʻO ka mea e hana ʻia ma ʻaneʻi, me ke kumu ʻole, ua lilo koke nā protein maʻamau i loko o ka lolo i nā 'prions' maikaʻi ʻole i ʻōlelo ʻia ma luna. ʻAʻole maopopo ke kumu pololei o kēia hana.

ʻO nā maʻi nui e pili ana i kēia mahele:

• ʻO ka maʻi Creutzfeldt-Jakob (CJD) : ʻO kēia ka 85% o nā maʻi prion sporadic. ʻO ia ke ʻano maʻamau.
• Ka hiamoe make ʻole i kekahi manawaHe mea kākaʻikahi loa kēia. ʻOi aku ka kākaʻikahi ma mua o ka insomnia make hoʻoilina.
• ʻO ka prionopathy protease-sensitive variable : He maʻi prion sporadic loa kēia.

Nā Maʻi Prion ʻOhana

Hoʻokumu ʻia kēia ʻano maʻi prion e kahi mutation i loko o kahi gene i kapa ʻia ʻo `PRNP` i loko o kā mākou mau genes. Hiki ke loaʻa ka maʻi inā paha i hoʻoilina ʻia ka mutation mai ka makuahine a i ʻole ka makuakāne (ua kapa ʻia kēia ʻo `autosomal dominant inheritance`). Ua ʻike ʻia ma mua o 50 mau mutations like ʻole i loko o ka gene `PRNP`, a hiki i kēlā me kēia mutation ke hana i nā ʻano maʻi prion hoʻoilina like ʻole.

ʻO kekahi mau maʻi e pili ana i kēia mahele:

• ʻO ka maʻi Creutzfeldt-Jakob ʻohana (CJD)
• ʻO ka maʻi Gerstmann-Sträussler-Scheinker (GSS) : He mea kākaʻikahi loa kēia. Loaʻa ia i hoʻokahi a ʻumi mau kānaka i loko o 100 miliona mau kānaka ma ka honua holoʻokoʻa.
• ʻO ka insomnia ʻohana make : ʻOi aku ka laha ʻole o kēia ma mua o ka maʻi GSS. He 50 a 70 mau ʻohana wale nō ma ka honua holoʻokoʻa e lawe nei i ka hoʻololi ʻana o ka genetic e hoʻoulu ai i kēia maʻi.
• Ua hōʻike pū ʻia nā maʻi prion ʻē aʻe i hoʻokumu ʻia e nā mutations i loko o ka gene PRNP. No ka laʻana, ua loaʻa i nā lālā he 11 o kahi ʻohana Pelekane nā hōʻailona e like me ka diarrhea, sensory autonomic neuropathy, seizures, a me ka dementia. Hoʻopilikia kēia ʻano maʻi, i kapa ʻia ʻo autonomic neuropathy, i nā mea e like me kou koko koko, ka helu o ka puʻuwai, a me ka incontinence o ka ʻōpū a me ka mimi.

Ka maʻi Prion i loaʻa

ʻO kēia ke ala maʻamau ʻole e loaʻa ai nā maʻi prion. Maanei, hiki i ke kanaka ke loaʻa i ka maʻi ma ka ʻai ʻana i ka meaʻai i haumia ʻia me nā prions (no ka laʻana, ka ʻiʻo mai ka bipi wahine me ka 'maʻi bipi pupule') a i ʻole ma ka hoʻohana ʻana i nā lako lapaʻau i haumia ʻia (no ka laʻana, i ka wā o ke ʻoki ʻana).

• ʻO Kuru ka hihia mua o kēia ʻano maʻi neurodegenerative lele i ʻike ʻia a aʻo ʻia. Ua loaʻa i waena o ka poʻe Fore o Papua New Guinea. Ua manaʻoʻiʻo ʻia ua laha ʻia ma o kekahi o kā lākou mau hana kuʻuna (e like me ka ʻai ʻana i ka ʻiʻo kanaka).

ʻO ka mea pōmaikaʻi, ʻo nā ʻano hoʻomaʻemaʻe koʻikoʻi o kēia lā no nā lako lapaʻau a me ka nānā ʻana i ka palekana meaʻai ua hōʻemi nui i ka laha ʻana o nā maʻi prion ma kēia ʻano.

He aha ka maʻi prion weliweli loa?

ʻO ka ʻoiaʻiʻo, he make nā maʻi prion āpau . ʻO ia hoʻi, inā loaʻa iā ʻoe ka maʻi, e make maoli nō ʻoe. ʻO ka maʻamau, make ka mea maʻi i loko o kekahi mau mahina a ʻekolu mau makahiki ma hope o ka hoʻomaka ʻana o nā hōʻailona o ka maʻi prion.

He aha nā hōʻailona o nā maʻi prion?

Hiki ke ʻokoʻa nā hōʻailona o ka maʻi prion, ma muli o ke ʻano o ka maʻi prion a me ka ʻāpana o ka lolo i hoʻopilikia ʻia. Akā ma ke ʻano laulā, hoʻomohala ka poʻe me nā maʻi prion i nā pilikia me kā lākou ʻōnaehana nerve e lilo mālie ana i mea ʻino.

Eia kekahi o nā hōʻailona maʻamau:

• Paʻakikī ke hele wāwae, ke kūlou (`Ataxia`)
• Ka paʻakikī o ka ʻōlelo ʻana, ka slurring o nā huaʻōlelo ('Aphasia')
• Ka huikau, ka nalowale ʻana o ke kuhikuhi
• ʻAʻole hiki ke hiamoe
• Ka nalowale o ka hoʻomanaʻo, ka paʻakikī i ka noʻonoʻo a me ka hoʻoholo ʻana
• Nā neʻe lohi a i ʻole ka ʻoʻoleʻa o nā ʻiʻo ('Nā maʻi neʻe hypokinetic')
• Ka ʻūlū koke ʻana, ka haʻalulu ʻana (`Myoclonus`)
• Nā loli o ke ʻano (e like me ka huhū, ka pihoihoi)
• Nā pilikia noʻonoʻo (e like me ka hopohopo, ke kaumaha, a i kekahi manawa nā hallucinations ʻike maka)

E noʻonoʻo, hoʻomaka koke kekahi mea kokoke loa iā ʻoe e loli. Poina iā ia nā mea, ʻaʻole hiki iā ia ke kamaʻilio e like me ka wā ma mua, paʻakikī iā ia ke hele. Pehea ka ʻeha o ka ʻike ʻana i kēia mau hōʻailona?

He aha nā pilikia o nā maʻi prion?

Hiki ke hoʻopili ʻia nā pilikia o nā maʻi prion kekahi i kekahi, e hana ana i kahi kahe o nā pilikia e hiki ke loaʻa ka hopena nui ʻaʻole wale i ka mea maʻi, akā i ka ʻohana a me nā hoaaloha e mālama ana iā lākou.

Hiki i kēia mau pilikia ke kū mai i loko o kekahi mau mahina a i ʻole hoʻokahi makahiki ma hope o ka hoʻomaka ʻana o nā hōʻailona:

• ʻAʻole hiki ke hana i kāu hana ponoʻī
• Dementia ( aneane nalowale loa ka hoʻomanaʻo)
• ʻAʻole hiki ke ʻōlelo (`Mutism`)
• ʻO Coma (ʻo kēia ke kahua hope loa)

Ke loaʻa i kekahi ka maʻi prion, lilo koke lākou i mea hilinaʻi i nā poʻe ʻē aʻe. Aia ka ʻohana a me nā poʻe ʻē aʻe ma laila e mālama iā lākou a kōkua iā lākou. I kēia manawa, ʻo ka nānā ʻana i kā lākou mea aloha e nalowale ana i ko lākou hoʻomanaʻo, hiki ʻole ke ʻōlelo, a me ka loli ʻana o ko lākou ʻano he mea hoʻoluhi nui ia no nā mea mālama.

No ke aha e loaʻa ai kēia mau maʻi prion?

He mea ʻepekema iki kēia, akā e ʻōlelo maʻalahi wau. Hoʻomaka nā maʻi prion i ka wā e lilo ai kahi protein prion maʻamau i loko o ko kākou lolo (i kapa ʻia ʻo `PrPc`) i protein ʻano ʻē a pelu hewa ʻia (i kapa ʻia ʻo `PrPSc` - he prion kēlā).

Hoʻomaka kēia mau prion ʻano ʻē (`PrPSc`) e hōʻuluʻulu, a hoʻopaʻa paha i nā protein prion maʻamau (`PrPc`), e hoʻolilo iā lākou i mau prion ʻano ʻē. Ua like ia me ka hoʻolei ʻana i kahi ʻāpala maikaʻi ʻole i loko o kahi puʻu o nā ʻāpala maikaʻi, a e lilo pū ke koena i mea ʻino. I ka hala ʻana o ka manawa, hōʻino a luku paha kēia mau protein prion ʻano ʻē i nā hunaola nerve i loko o ka lolo. ʻO ia ka manawa hiki ʻole iā ʻoe ke hele, noʻonoʻo, a kamaʻilio pono paha.

Pehea e ʻike ʻia ai ka maʻi prion?

Hiki i nā kauka ke hoʻohana i nā hoʻokolohua ma lalo nei e ʻike ai i kahi maʻi prion:

• Nā hoʻāʻo koko a me ka puncture lumbar : ʻO ka poʻe me ka hoʻololi genetic e hoʻoulu ai i nā maʻi prion hoʻoilina e nānā ʻia no nā biomarkers o ka maʻi a i ʻole ka hōʻino i ko lākou koko a i ʻole ka wai cerebrospinal (ka wai e hoʻopuni ana i ka lolo a me ke kaula spinal).
• ʻO ke kiʻi MRI lolo : Hiki i kēia ke kiʻi i nā kiʻi maopopo loa o ka lolo, no laila hiki i nā kauka ke nānā i nā hōʻailona o ka maʻi prion.
• Electroencephalogram (EEG) : Ke ana kēia i ka hana uila o ka lolo.
• ʻO ka hoʻāʻo hoʻololi i hoʻoulu ʻia e ka haʻalulu manawa maoli (RT-QuIC) : Ma kēia hoʻāʻo, ʻimi nā pathologists i nā prions i loko o ke kahe o ka iwi kuamoʻo. He hoʻāʻo hou a pololei kēia.

Aia kekahi lāʻau lapaʻau no nā maʻi prion?

ʻO ka mea pōʻino, ʻaʻole i loaʻa i nā kauka kahi lāʻau lapaʻau no nā maʻi prion a i ʻole kahi lāʻau e hiki ke kāohi i ka holomua o ka maʻi. He mau maʻi hoʻoweliweli ola nā maʻi Prion . Make ka hapa nui o ka poʻe i loko o nā mahina a i ʻole nā ​​​​makahiki o ka ʻike ʻia ʻana o ka maʻi prion.

Ke kālele nei ka lāʻau lapaʻau i ka mālama palliative . ʻO ia hoʻi ke kāohi ʻana i nā hōʻailona a me ka hoʻolilo ʻana i ka mea maʻi i ʻoluʻolu a ʻeha ʻole e like me ka hiki. No ka laʻana, hiki i nā kauka ke kuhikuhi i nā lāʻau lapaʻau no nā mea e like me:

• Nā lāʻau lapaʻau antiseizure a i ʻole nā ​​​​mea hoʻomaha ʻiʻo no ka myoclonus.
• ʻO nā opioids no ka ʻeha.

He aha ke ʻano o ka wā e hiki mai ana o kekahi me ka maʻi prion?

I kēia manawa, ʻaʻohe lāʻau lapaʻau a lāʻau lapaʻau paha no nā maʻi prion. Eia nō naʻe, ke noiʻi nei nā kānaka noiʻi i kēia ma nā ʻano ʻelua, kahi e hiki ai ke alakaʻi i ka ʻike mua ʻana o ka maʻi a me nā lāʻau lapaʻau e hiki mai ana.

Ua kōkua ka hoʻāʻo `RT-QuIC` i ʻōlelo ʻia ma luna nei i ka ʻike ʻana i ka maʻi prion ma mua o ka hiki ʻana mai o ka hōʻino hiki ʻole ke hoʻihoʻi ʻia i ka lolo. Ke aʻo nei hoʻi nā kānaka noiʻi i nā prions e ʻimi i nā ala e hoʻōki ai i ka hoʻokumu ʻia ʻana o kēia mau protein maʻamau a pale i nā protein maʻamau mai ka lilo ʻana i mea maʻamau.

He aha kaʻu e hana ai inā loaʻa iaʻu kahi maʻi prion?

Ma muli o ka holomua wikiwiki ʻana o ka maʻi prion, he mea paʻakikī paha iā ʻoe ke hana i kāu hana ponoʻī. Inā loaʻa iā ʻoe kēia maʻi, he manaʻo maikaʻi e hoʻopau i kahi "kuhikuhi mua" .

He mau palapala kānāwai nā kuhikuhi mua. Nā Laʻana:

• Nā palapala kauoha ola : Inā loaʻa iā ʻoe kahi maʻi make e like me ka maʻi prion, hiki i kēia palapala ke kuhikuhi i nā mea āu e makemake ai e hana iā ʻoe (e.g. inā makemake ʻoe i kekahi mau lāʻau lapaʻau).
• Mana paʻa o ka loio no ka mālama ola kino (DPA): Hiki i kēia palapala ke inoa i ka mea nāna e hoʻoholo i kāu mau hoʻoholo mālama ola kino inā ʻaʻole hiki iā ʻoe ke ʻōlelo nou iho.

Ma ka hoʻolālā ʻana i nā mea e like me kēia ma mua, hiki iā ʻoe ke hōʻemi i ke kaumaha e ʻike ai ʻoe a me kou ʻohana ma hope.

He aha inā loaʻa i kekahi o ka ʻohana kahi maʻi prion hoʻoilina?

I kekahi manawa, hoʻoilina nā kānaka i nā hoʻololi genetic e hoʻonui ai i ko lākou pilikia o ka loaʻa ʻana o ka maʻi prion. Hiki i nā hoʻokolohua genetic ke hoʻoholo inā loaʻa iā ʻoe kekahi a ʻoi aku paha o nā hoʻololi gene e hoʻoulu ai i ka maʻi prion. Hiki i kēia mau hoʻokolohua ke hōʻike i kāu pilikia kikoʻī.

He hoʻoholo pilikino ka loaʻa ʻana o kahi hoʻāʻo genetic no kahi maʻi make. ʻAʻole makemake kekahi poʻe e ʻike inā aia lākou i ka pilikia. Inā makemake ʻoe e loaʻa kahi hoʻāʻo genetic, e noi i ke kauka no kahi kuhikuhi. No ka laʻana, ma ʻAmelika Hui Pū ʻIa, aia nā ʻoihana e like me ka "National Prion Disease Pathology Surveillance Center" no kēia. Aia kekahi mau kauka loea ma Sri Lanka e hiki ke aʻo aku i kēia mea.

Pehea ʻoe e mālama ai i kahi lālā o ka ʻohana me ka maʻi prion?

Hiki i kahi maʻi e like me kēia ke hoʻohuli i kou ola o kēlā me kēia lā i lalo. ʻOiai ʻoe e kū nei i ka haʻalulu a me ke kaumaha o ka lohe ʻana ua loaʻa i kāu mea aloha kahi maʻi make, pono ʻoe e hoʻolālā pehea ʻoe e mālama ai i kou ʻohana. Eia kekahi mau manaʻo:

• E hana i kahi hoʻolālā mālama : Ma muli o kou kūlana, pono paha i kou ʻohana ke mālama ʻia ma kahi hale noho, a i ʻole pono paha lākou i ka mālama ma ka home, e hilinaʻi ana i ka ʻohana, nā hoaaloha, a me nā mea kōkua olakino pilikino. Pono paha kāu mea aloha i ke kōkua me nā hana o kēlā me kēia lā, e like me ka hele ʻana i ka lua a me ka ʻai ʻana.
• E hoʻonohonoho no ka mālama hospice : ʻO ka maʻi Prion kahi maʻi make e hiki ke ulu koke a lilo i mea ʻino loa. Inā ʻoe e hoʻonohonoho no ka mālama hospice ma mua, hiki i kāu mea aloha ke loaʻa iā ia ke pono lākou.
• E hana i kahi ʻano mālie : ʻO ka poʻe me nā maʻi prion he mea maʻalahi pinepine lākou i nā hanana koke (e laʻa, ka hoʻopā ʻana i kekahi, ka lohe ʻana i kahi walaʻau nui, ke noho ʻana ma nā wahi piha kanaka). Hiki iā lākou ke hopohopo a huhū paha. Hiki i ka mālama ʻana i ko lākou wahi ke kōkua iā lākou e noho mālie a hoʻomaha.

I ka manawa e like me kēia, he mea nui i ka mea mālama ke noʻonoʻo iā lākou iho pū kekahi. Pono ʻoe i ka hoʻomaha a me ke kākoʻo pū kekahi. Mai ho'āʻo e hāpai wale i kēia kaumaha.

Āhea wau e ʻike ai i ke kauka?

ʻO ka maʻamau, e ʻoi aku ka wikiwiki o nā hōʻailona o nā maʻi prion. Inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kahi maʻi prion, pono ʻoe e hana pū me kāu mau kauka e hoʻokele i nā hopena o ka maʻi.

ʻOi aku hoʻi, inā hoʻomaka koke nā hōʻailona i ʻōlelo ʻia ma mua (e like me ka nalowale o ka hoʻomanaʻo, ka paʻakikī i ka hele wāwae, ka paʻakikī i ka ʻōlelo ʻana, nā loli o ke ʻano) a ʻoi aku ka ʻino, e ʻimi koke i ke kōkua lapaʻau.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

ʻO ka ʻike ʻana ua loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kahi maʻi prion hiki ke lilo i mea hoʻohoka nui. He nui nā pilikia āu a me kou ʻohana i kēia manawa, a e kū mai ana nā mea hou aʻe i ka holomua ʻana o ka maʻi. Eia kekahi mau nīnau e kōkua iā ʻoe e hoʻomaopopo i ka mea e manaʻo ai:

• Pehea ʻoe i ʻike ai he maʻi prion koʻu?
• He aha ka maʻi prion i loaʻa iaʻu?
• Pehea ia e pili ai iaʻu?
• He aha kaʻu e hana ai ma hope?
• He aha kaʻu e hana ai e mālama ai i koʻu ʻohana?
• Pono anei koʻu ʻohana e hana i ke aʻoaʻo genetic a me ka hoʻāʻo ʻana?

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻO nā maʻi Prion kahi hui o nā maʻi kakaikahi a make e hoʻopilikia i kou lolo. Hiki i nā hōʻailona Prion ke hele koke mai a ʻoi aku ka ʻino. Hiki ke kaumaha ka ʻike ʻana ua loaʻa iā ʻoe a i ʻole kekahi āu e aloha ai kahi maʻi ʻaʻohe lāʻau lapaʻau a lāʻau lapaʻau paha e kāohi ai.

Eia nō naʻe, aia nā lāʻau lapaʻau e kōkua i ka hoʻokele ʻana i nā hōʻailona . A aia nā papahana a me nā lawelawe e kōkua i ka poʻe i hoʻopilikia ʻia e nā maʻi prion a me ko lākou poʻe mālama. Inā loaʻa iā ʻoe a i ʻole kekahi āu e aloha ai kahi maʻi prion, mai makaʻu e nīnau i nā nīnau a ʻimi i ke kōkua. ʻAʻole ʻoe hoʻokahi.

ʻO ka maʻi Prion, ka maʻi lolo, ka maʻi Neurological, Dementia, CJD, ka maʻi Genetic