Ua ʻike anei ʻoe i ka MSA (Multiple System Atrophy), kahi maʻi e hoʻopilikia ana i kekahi mau ʻōnaehana kino?

E noʻonoʻo, ua paʻakikī koke ʻoe i ka hele ʻana, nalowale kou kaulike, a manaʻo i ka poniuniu a hāʻule i kou kū ʻana mai kahi noho. He mea maʻamau ka makaʻu nui i ka wā e hoʻomaka koke ai kēia mau mea. ʻOiai ʻaʻole mākou e nānā nui i kēia mau mea i kekahi manawa, hiki iā lākou ke lilo i mau hōʻailona mua o kekahi mau maʻi laha ʻole. I kēia lā e kamaʻilio mākou e pili ana i kekahi maʻi neurological laha ʻole, akā koʻikoʻi loa. ʻO ia ka Multiple System Atrophy , kahi maʻi a mākou e kapa nei he mau kauka a me nā maʻi (MSA) no ka pōkole. ʻOiai he kumuhana paʻakikī iki kēia, e kamaʻilio maʻalahi mākou e pili ana iā ia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

I ka ʻōlelo maʻalahi, he aha kēia MSA?

He maʻi neurological laha ʻole ka MSA kahi e nāwaliwali mālie ai a make ai nā ʻāpana o ko kākou lolo. I ka hala ʻana o ka manawa, nalowale mālie nā hiki a me nā hana o ke kino i kāohi ʻia e kēia mau ʻāpana i hōʻino ʻia o ka lolo. ʻO ka ʻoiaʻiʻo, he kūlana kaumaha loa kēia, no ka mea, ʻo ka hopena o kēia maʻi he make.

I ka wā ma mua, ua kapa nā kauka i kēia hōʻiliʻili o nā hōʻailona ma ʻekolu mau inoa. ʻO lākou kēia:

• ka maʻi Shy-Drager
• ʻO ka atrophy olivopontocerebellar sporadic
• ʻO ka degeneration striatonigral

Akā ma hope mai, ua ʻike nā kānaka noiʻi he nui nā hiʻohiʻona like o kēia mau maʻi ʻekolu. No laila, ua hoʻohui lākou iā lākou a pau a kapa iā lākou ʻo "Multiple System Atrophy" (MSA). ʻO ke ʻano o "Multiple System" ʻo ia hoʻi "he mau ʻōnaehana." ʻO ia hoʻi, hoʻopilikia kēia maʻi i kekahi mau ʻōnaehana i loko o ko kākou kino. Aia nā hōʻailona i nā ʻāpana o ka lolo i hōʻino ʻia. ʻO ia ke kumu e ʻike ai kēlā me kēia kanaka i kahi hui like ʻole o nā hōʻailona.

Ma hope mai, ua hoʻokaʻawale hou ʻia ka maʻi, ma muli o nā hōʻailona i hōʻike ʻia. No laila, aia ʻelua ʻano o MSA.

ʻO wai ka mea i pilikia nui i ka hoʻomohala ʻana i ka MSA?

Hoʻopilikia pinepine ka MSA i nā pākeke, ʻoi aku hoʻi ma hope o 30 mau makahiki. ʻIke nui ʻia nā hōʻailona ma waena o 50 a me 59 mau makahiki. Hiki i kēia maʻi ke hoʻopilikia i kekahi, me ka nānā ʻole i ke kāne.

He maʻi laha ʻole kēia. Wahi a nā helu helu, aia wale nō 0.6 a 0.7 mau hihia hou no 100,000 poʻe i kēlā me kēia makahiki. ʻO ke ʻano kēia, ʻaʻole kēia he maʻi maʻamau i loko o ko kākou kaiāulu.

Pehea ka hopena o ka maʻi MSA i ko mākou kino?

Hoʻopilikia ka MSA i nā ʻāpana like ʻole o ka lolo e make. Pili nā hōʻailona i nā ʻāpana o ka lolo i hōʻino ʻia. ʻO nā ʻāpana nui o ka lolo i hoʻopilikia ʻia:

• ʻO Basal Ganglia: Aia kēia mau mea ma waenakonu o ka lolo. Ua like lākou me kahi kikowaena e hoʻopili ai i nā ʻāpana like ʻole o ka lolo kekahi i kekahi. Ma o kēia ʻāpana e hui pū ai nā ʻāpana like ʻole o ka lolo a hana pū.
• ʻO ka lolo: ʻO kēia kahi e kāohi ʻia ai nā kaʻina hana aunoa i loko o ko kākou kino e pono ai no ke ola. Eia kekahi laʻana, nā mea e like me ka hanu ʻana, ka helu o ka puʻuwai, a me ke koko. Hana koke ʻia kēia mau mea me ka ʻole o ko kākou noʻonoʻo ʻana iā lākou, ʻeā? ʻO kēia ke kikowaena kaohi.
• ʻO ka Cerebellum: Aia kēia ma ke kua o ke poʻo, kokoke i ke kumu. ʻO kēia ka ʻāpana nui o ko kākou lolo e hoʻonohonoho i nā neʻe ʻana o ko kākou kino a mālama i ko kākou kaulike. Eia kekahi, ua ʻike ʻia i kēia manawa e ka noiʻi ua pili pū kēia ʻāpana i ko kākou mau manaʻo a me ka hoʻoholo ʻana.

No laila, ke hōʻino ʻia kēia mau ʻāpana o ka lolo, hoʻomaka nā kaʻina hana āpau i kāohi ʻia e kēlā mau ʻāpana e hele hewa. No ka laʻana, ke hōʻino ʻia ka `(brainstem)`, aia nā pilikia koʻikoʻi me nā kaʻina hana aunoa e like me ke koko.

He aha nā hōʻailona nui o MSA?

I loko o ka MSA, he mea maʻamau kekahi mau hōʻailona i nā ʻano ʻelua. Eia kekahi, aia nā hiʻohiʻona e pili ana i kēlā me kēia ʻano. ʻO ka hiʻohiʻona nui i maʻamau i nā ʻano ʻelua, ʻo ia ka hana pono ʻole o ka autonomic . I ka ʻōlelo maʻalahi, ʻaʻole hana pono nā kaʻina hana e hana koke ʻia i loko o ko mākou kino.

Nā Hōʻailona o ka Dysfunction Autonomic

• ʻO ka Orthostatic Hypotension: ʻO kēia ka hōʻailona mua a ka poʻe he nui e ʻike ai. Ke kū koke ʻoe mai kahi noho a moe paha , hāʻule koke kou koko . Hiki i kēia ke hana i ka poniuniu, ka ʻike maka pohihihi, a me ka hāʻule ʻana.
• ʻAʻole hiki ke kaohi i ka mimi a me ka ʻōpala: Hiki ke loaʻa ka incontinence urinary a me ka incontinence fecal incontinence ke hiki mai ka wā āu e manaʻo ʻole ai.
• Ka hana pono ʻole o ka moekolohe: ʻO nā pilikia e like me ka hana pono ʻole o ka erectile, ʻoi aku hoʻi i nā kāne, e kū mai ana i ka wā o ka moekolohe.
• Nā pilikia hiamoe: ʻOi aku ka ʻike ʻia o kahi kūlana i kapa ʻia ʻo "(REM) sleep behavior disorder". Ma kēia, ke moeʻuhane ʻoe, hoʻoneʻe ʻoe i kou kino, uē, a hoʻoluliluli i kou mau lālā i ka pane ʻana i kāu mau moeʻuhane.
• Ka emi ʻana o ka hou ʻana (Anhidrosis): Ua emi nui ka hiki o ke kino ke hou.
• ʻO nā hōʻailona e like me nā pilikia ʻike, ka waha maloʻo, ka apnea hiamoe, a me ka paʻa ʻana he mea maʻamau nō hoʻi.

Nā ʻano noʻonoʻo a me nā naʻau

Ma kahi o ka hapakolu o nā maʻi MSA e ʻike i nā pilikia me ko lākou hiki ke noʻonoʻo a me ka noʻonoʻo. Loaʻa iā lākou ka paʻakikī ke kāohi i ko lākou mau manaʻo, hiki ke alakaʻi i nā ʻano pilikia olakino noʻonoʻo like ʻole.

• Hopohopo
• Kaumaha
• Ke uē ʻana a ʻakaʻaka paha me ke kūpono ʻole
• Nā hoʻouka kaua panic
• Nā manaʻo e hōʻeha a pepehi paha iā ʻoe iho

Nā Hōʻailona e pili ana i ka Neʻe ʻana

ʻOkoʻa kēia mau ʻano ma muli o nā ʻano ʻelua o MSA a mākou i kūkākūkā ai ma mua.

He aha ke kumu o ka MSA?

ʻAʻole ʻike ʻia ke kumu kikoʻī o MSA . Eia nō naʻe, ke manaʻo nei nā kānaka ʻepekema ua hoʻokumu ʻia e kahi protein i kapa ʻia ʻo alpha-synuclein . Ua manaʻo ʻia e hōʻiliʻili kēia protein i nā ʻano like ʻole o ka lolo, e hōʻino ana i nā hunaola lolo. ʻO ka mea kupanaha, ua manaʻo ʻia hoʻi kēia protein like ke kumu o ka maʻi Parkinson.

He mea nui nā protein no ka hana ʻana o ko kākou kino. Eia nō naʻe, ke hōʻuluʻulu ʻia kēia mau protein ma nā wahi kūpono ʻole, ma ke ʻano kūpono ʻole, hiki iā lākou ke hōʻino i nā cell. ʻO ia ka mea e hana ʻia ma MSA.

Ke hoʻomau nei ka noiʻi ʻana i ke kumu e hōʻiliʻili ai kēia protein alpha-synuclein i loko o ka lolo. Ua manaʻo ʻia he kumu ia o nā hoʻololi genetic. Ma ke ʻano kūikawā, aia nā hōʻike e hiki ke hoʻoili ʻia ke ʻano MSA-C mai kēlā hanauna a i kēia hanauna i kekahi ʻano. Eia nō naʻe, ʻaʻohe loulou genetic i loaʻa no ke ʻano MSA-P.

Pehea e ʻike ʻia ai ka MSA?

ʻO kēia ka ʻāpana paʻakikī loa o ka maʻi. ʻO ia wale nō ke ala e hōʻoia ai i ka MSA me ka maopopo 100%.ʻO ke kumu o kēia, ʻaʻohe ʻenehana i kēia manawa e ʻike ai inā ua waiho ʻia ka protein alpha-synuclein i loko o ka lolo o ke kanaka ola.

Eia nō naʻe, ʻoiai ke ola nei ka mea maʻi, hiki i nā kauka ke kānalua i ka maʻi. Hoʻohana lākou i nā mea e like me nā hōʻailona, ​​​​ka mōʻaukala olakino o ka mea maʻi, ka mōʻaukala ʻohana, a me ka pane ʻana i kekahi mau lāʻau lapaʻau. I ka manawa pinepine, hiki i nā kauka ke ʻike mua i ka maʻi o Parkinson. Eia nō naʻe, i ka wā e ulu ai nā hōʻailona ʻē aʻe i ka hala ʻana o ka manawa, a i ʻole ke pau ka hana ʻana o ka lāʻau lapaʻau o Parkinson, pono paha e hoʻololi ʻia ka hōʻailona i MSA.

Nā ʻokoʻa koʻikoʻi ma waena o MSA a me ka maʻi o Parkinson

Nā hoʻokolohua diagnostics

He kakaikahi loa nā hoʻokolohua e hiki ke ʻike pololei i ka MSA. ʻO ka hapa pinepine, hana ʻia kekahi mau hoʻokolohua e kāpae i nā kūlana ʻē aʻe a hōʻiliʻili i nā hōʻike e kākoʻo i ke kānalua he kumu paha ʻo MSA.

• ʻO ke kiʻi MRI (Magnetic Resonance Imaging): Hiki i kēia ke hōʻike i kekahi manawa i ka hōʻino ʻia i nā ʻāpana o ka lolo. I loko o MSA-C, ʻoi aku hoʻi, hiki ke ʻike ʻia kahi ʻano i kapa ʻia ʻo ka hōʻailona "hot cross bun" ma kahi ʻāpana o ka lolo. Ua like ia me ka hot cross bun. Eia naʻe, hiki ke ʻike ʻia kēia hōʻailona i nā maʻi ʻē aʻe, no laila ʻaʻole ia he hōʻailona paʻa o MSA.
• Ka hoʻāʻo ʻana i ka genetic: Hiki i kēia ke nānā no nā mutations genetic e pili ana i ka protein alpha-synuclein.
• ʻO ka biopsy ʻili: Hōʻike kekahi mau noiʻi hou e hiki i kēia hoʻāʻo ke kōkua i ka hoʻoholo ʻana inā aia ka alpha-synuclein i loko o ke aʻalolo o ka ʻili. Eia nō naʻe, aia nō kēia i loko o ke kahua noiʻi.

E wehewehe kāu kauka iā ʻoe i nā hoʻokolohua āna i manaʻo ai he pono, ma muli o kou kūlana, a me ka mea e manaʻo ai mai iā lākou.

Aia kekahi lāʻau lapaʻau no MSA?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no MSA i kēia manawa . No laila, ʻo ka pahuhopu nui o ka mālama ʻana, ʻo ia ke kāohi i nā hōʻailona a mālama i kahi ola maikaʻi no ka lōʻihi e hiki ai.

ʻO ka lāʻau i hāʻawi ʻia ma muli o nā hōʻailona o ka mea maʻi a me ko lākou koʻikoʻi. No ka laʻana, hāʻawi ʻia nā lāʻau koko no ka hypotension orthostatic, nā mea hoʻomaha ʻiʻo no ka ʻoʻoleʻa, a me nā lāʻau ʻē aʻe no ka kaohi ʻana i ka mimi. Eia kekahi, he mea nui loa nā mea e like me ka physiotherapy a me ka speech therapy.

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

He maʻi holomua ka MSA. Ma kahi o ka hapalua o ka poʻe i hoʻopilikia ʻia e pono ai ke kōkua hele wāwae (e like me ke koʻokoʻo a i ʻole ka mea hele wāwae) i loko o 5 mau makahiki o ka hoʻomaka ʻana o nā hōʻailona. Ma kahi o 60% e pono ai kahi noho huila i loko o 5 mau makahiki. I ka 6 a 8 mau makahiki, ua moe ka hapa nui o nā maʻi ma kahi moe.

I ka holomua ʻana o ka maʻi, pono nā ʻano hana lapaʻau like ʻole e mālama i nā hana o ke kino.

• ʻO Tracheostomy kahi hana e hana ʻia ai kahi puka liʻiliʻi ma ka ʻāʻī a hoʻokomo ʻia kahi paipu e kōkua ai i nā pilikia hanu.
• ʻO ka hānai ʻana i ka meaʻai ma o kahi paipu ka hāʻawi ʻana i ka meaʻai ma o kahi paipu ke hiki ʻole i ka mea maʻi ke ale.
• ʻO ka paipu a i ʻole nā ​​​​kaʻina hana ʻoki ʻē aʻe e kaohi ai i ka mimi a me ka ʻōpala.

ʻO ka maʻamau, ʻo ka awelika o ke ola no ka mea maʻi me MSA mai ka manawa o ka ʻike ʻia ʻana ma waena o 6 a me 10 mau makahiki. I kekahi mau hihia ʻoi aku ka koʻikoʻi, hiki ke ola a hiki i 15 mau makahiki. Eia nō naʻe, inā koʻikoʻi loa ka maʻi, hiki ke pōkole loa ke ola.

ʻO ka make ke kumu nui o nā pilikia o ka maʻi. Eia kekahi laʻana:

• Pneumonia.
• Sepsis (ka lāʻau make koko) ma muli o ka maʻi ʻōpū mimi.
• ʻO ka make koke i hoʻokumu ʻia e nā pilikia me ka kaohi ʻana o ka lolo i ka hanu ʻana i ka wā hiamoe.

Pehea lā kākou e mālama ai i ko kākou poʻe aloha?

I ka ʻoi aku o ka ʻino o nā hōʻailona o ke kanaka me MSA i ka hala ʻana o ka manawa, hiki iā lākou ke lilo i mea hiki ʻole ke noho kūʻokoʻa. Hiki iā lākou ke nalowale i ka hiki ke noʻonoʻo, ʻōlelo, a hoʻoholo i nā hoʻoholo no lākou iho.

No laila, he mea nui loa ke kamaʻilio e pili ana i ka wā e hiki mai ana me kāu poʻe aloha i ka wā mua o ka maʻi, ʻoiai ke hoʻomaopopo nei ka mea maʻi. Inā ʻoe i loko o kahi kūlana kahi hiki ʻole iā ʻoe ke hoʻoholo, he mea maʻalahi loa ia no nā mea āpau inā hiki iā ʻoe ke kūkākūkā mua i nā hoʻoholo e pono e hana ʻia e pili ana i kāu mālama lapaʻau a hoʻomākaukau i nā palapala kānāwai.

Āhea ʻoe e ʻike ai i ke kauka?

ʻO ka nui o nā hōʻailona mua o MSA nā mea āu e kūkākūkā ai me ke kauka.

• Moekolohe.
• ʻO ka poniuniu mau a me ka nalowale o ka ʻike i ke kū ʻana.
• Nā pilikia hiamoe, ʻoiai hoʻi ka apnea hiamoe.

Inā ua haʻi aku kāu kauka iā ʻoe he maʻi neʻe kou e like me ka maʻi Parkinson, he mea nui e kamaʻilio e pili ana i nā loli o kāu mau hōʻailona. ʻOiai inā ʻaʻole holo pono ka lāʻau lapaʻau (levodopa) no ka maʻi Parkinson, he hōʻailona nui kēlā e loaʻa paha iā ʻoe ka MSA.

He maʻi koʻikoʻi a make ka MSA. He ʻoiaʻiʻo ʻaʻohe lāʻau lapaʻau no ia mea. Eia naʻe, hiki ke mālama ʻia nā hōʻailona he nui. Ma o kēlā lāʻau lapaʻau, hiki ke mālama ʻia ke ʻano o ke ola o ka mea maʻi ma kahi pae maikaʻi no nā makahiki he nui. He manawa kūpono kēlā manawa e hoʻolilo hauʻoli ai me kā lākou poʻe aloha a hauʻoli i ke ola piha.