ʻO Insomnia Familial Famalial

Anter

• He aha nā hōʻailona?
• He aha ke kumu?
• Pehea ia ʻike?
• Pehea e mālama ʻia ai?
• Noho me FFI

He aha ka hiamoe ʻohana make?

ʻO ka hiamoe ʻohana ʻeha loa (FFI) kahi maʻi hiamoe pinepine ʻole e holo i loko o nā ʻohana. Hoʻopili ia i ka thalamus. Mālama kēia hanana lolo i nā mea nui he nui, e like me ka manaʻo a me ka hiamoe ʻOiai ka hiamoe nui o ka hiamoe, hiki i ka FFI ke kumu i kahi ʻano o nā hōʻailona ʻē aʻe, e like me nā pilikia ʻōlelo a me ka dementia.

Aia kekahi ʻano ʻē aʻe i kapa ʻia ʻo insomnia make ʻole sporadic. Eia nō naʻe, he 24 wale nō mau hihia i hoʻopaʻa ʻia e pili ana i ka makahiki 2016. ʻIke liʻiliʻi ka poʻe noiʻi e pili ana i ka insomnia fatal sporadic, koe wale nō me he mea lā ʻaʻole ia he genetic.

Loaʻa iā FFI kona inoa ma kahi ʻāpana mai ka mea pinepine i ka make i loko o hoʻokahi makahiki o ʻelua mau ʻōuli e hoʻomaka ana. Eia nō naʻe, hiki ke hoʻololi i kēia kaʻina manawa mai kēlā me kēia kanaka.

ʻO kahi ʻohana o nā kūlana i ʻike ʻia he prion maʻi. ʻO kēia mau ʻano laha ʻole ke kumu o ka nalo ʻana o nā hunaola nerve i ka lolo. ʻO nā maʻi prion ʻē aʻe e pili ana i ka maʻi kuru a me Creutzfeldt-Jakob. Aia wale nō ma kahi o 300 nā hihia o nā maʻi prion i hōʻike ʻia i kēlā me kēia makahiki i United States, e like me ka Johns Hopkins Medicine. Manaʻo ʻia ʻo FFI kekahi o nā maʻi prion rarest.

He aha nā hōʻailona?

Kūleʻa nā ʻōuli o FFI mai kēlā me kēia kanaka. Hōʻike lākou ma waena o nā makahiki o 32 a me 62. Eia nō naʻe, hiki i lākou ke hoʻomaka i ka wā ʻōpio a makua paha.

ʻO nā hōʻailona kūpono o ka wā mua FFI me:

• pilikia e hiamoe ana
• pilikia e hiamoe ana
• ʻūlū mākala a me nā spasms
• ʻoʻoleʻa o ka mākala
• neʻe a keʻehi ʻana ke hiamoe
• nalo ʻai ʻole
• hōʻemi wikiwiki holomua

ʻO nā ʻōuli o ka FFI ʻoi aku ka nui i kēia:

• hiki ʻole ke hiamoe
• deteriorating ʻike a me ka noʻonoʻo hana
• nalowale o ka hoʻohuihui ʻana, a i ʻole ataxia
• hoʻonui i ke kahe o ke koko a me ka helu o ka puʻuwai
• sweating nui loa
• pilikia i ka ʻōlelo ʻana a me ka ale ʻana paha
• lilo kaumaha maopopo ʻole
• fiva

He aha ke kumu?

Hoʻokumu ʻia ʻo FFI e ka hoʻololi o ka gen PRNP. ʻO kēia hoʻololi ke kumu o ka hoʻouka ʻana i ka thalamus, ka mea e kaohi i kāu pōʻai hiamoe a ʻae i nā ʻāpana like ʻole o kou lolo e kamaʻilio me kekahi.

Ua manaʻo ʻia he maʻi neurodegenerative holomua. ʻO kēia ke kumu o ka lilo ʻana o kāu thalamus i mau neʻena nerve. ʻO kēia nalo o nā pūnaewele ke alakaʻi i ka laulā o nā hōʻailona ʻo FFI.

ʻO ka mutation kuleana no FFI i hoʻoili ʻia e nā ʻohana. He 50 pakeneka ka loaʻa o ka makua me ka mutation o ka hoʻoili ʻana i kā kā lāua keiki keiki.

Pehea ia ʻike?

Inā manaʻo ʻoe he FFI paha kāu, e hoʻomaka paha kāu kauka ma ke noi ʻana iā ʻoe e mālama i nā kikoʻī kikoʻī e pili ana i kāu ʻano hiamoe no kekahi manawa. E hoʻokau paha lākou iā ʻoe e hoʻopaʻa i ka hiamoe. Pili kēia i ka hiamoe ʻana i kahi haukapila a i ʻole ke kikowaena hiamoe ʻoiai kākau kāu kauka i nā ʻikepili e pili ana i nā mea e like me ka hana o ka lolo a me ka helu o ka puʻuwai. Hiki i kēia ke kōkua i ka pale aku i nā kumu ʻē aʻe o kāu pilikia hiamoe, e like me ka sleep apnea a me narcolepsy.

A laila, pono ʻoe i kahi scan PET. E hāʻawi kēia ʻano hoʻokolohua kiʻi i kāu kauka i kahi manaʻo maikaʻi e pili ana i ka maikaʻi o ka hana o kāu thalamus.

Hiki i ka hoʻāʻo genetic ke kōkua i kāu kauka e hōʻoia i kahi hōʻailona. Eia nō naʻe, maʻAmelika Hui Pū ʻIa, pono ʻoe i kahi moʻolelo ʻohana o FFI a i ʻole hiki iā ʻoe ke hōʻike i nā hōʻike mua i hōʻike ikaika iā FFI i mea e hana ai i kēia. Inā loaʻa iā ʻoe kahi hihia paʻa o FFI i kou ʻohana, ua kūpono ʻoe no ka hoʻokolohua genen prenatal.

Pehea e mālama ʻia ai?

ʻAʻohe mea ola no FFI. Hiki i kekahi mau hōʻailona ke kōkua pono i ka mālama ʻana i nā ʻōuli. ʻO nā lāʻau hiamoe, no ka laʻana, hiki ke hāʻawi i kahi hoʻomaha manawa no kekahi poʻe, akā ʻaʻole lākou e hana i kahi wā lōʻihi.

Eia nō naʻe, ke hana ikaika nei nā mea noiʻi i nā mālama kūpono a me nā hana pale. Hōʻike ʻo A i hiki ke kōkua i ka immunotherapy, akā makemake ʻia i kahi noiʻi hou, e like me ka noiʻi kanaka. Eia kekahi ke hoʻomau nei e pili ana i ka hoʻohana ʻana o doxycycline, kahi antibiotic. Manaʻo nā noiʻi he ala maikaʻi paha ia e pale aku ai iā FFI i ka poʻe e hāpai ana i ka hoʻololi kūʻē i kumu ʻia.

He nui ka poʻe me nā maʻi laha ʻole e ʻike he mea kōkua ke hoʻohui me nā poʻe ʻē aʻe i kahi ʻano like, ma ka pūnaewele a i ʻole i kahi hui kākoʻo kūloko. ʻO ka Creutzfeldt-Jakob Disease Foundation kahi laʻana. He nonprofit ia e hāʻawi ana i nā kumuwaiwai e pili ana i nā maʻi prion.

Noho me FFI

Hiki iā ia he mau makahiki ma mua o ka hoʻomaka ʻana o nā ʻōuli o FFI. Eia nō naʻe, ke hoʻomaka lākou, lilo lākou i mea ʻoi aku ka maikaʻi ma mua o hoʻokahi makahiki a ʻelua paha. ʻOiai ke hoʻomau nei ka noiʻi e pili ana i nā hoʻōla hiki, ʻaʻohe lāʻau lapaʻau i ʻike ʻia no FFI, ʻoiai hiki i nā mea kōkua hiamoe ke hāʻawi i kahi hoʻomaha manawa.