Ke manaʻo nei ʻoe e nalowale mālie ana kou mana i kou mau lālā? He maʻi Motor Neuron (MND) paha kēia!

Ua manaʻo paha ʻoe ua mae kou mau wāwae i kou piʻi ʻana i ke alapiʻi, ua paʻakikī kāu mau ʻōlelo i kou kamaʻilio ʻana, a i ʻole ua hāʻule koke kekahi mea mai kou lima mai? Manaʻo paha ʻoe he mea maʻamau kēia, akā inā hoʻomau kēia mau hōʻailona, ​​​​he manaʻo maikaʻi e hopohopo iki. No ka mea, he mau hōʻailona mua paha kēia o kahi maʻi i kapa ʻia ʻo Motor Neuron Disease (MND) . No laila, e kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku i kēia lā.

He aha ka maʻi neurone motor (MND)?

I ka ʻōlelo maʻalahi, ʻo ka maʻi neurone motor (MND) he hui o nā maʻi e hoʻopilikia i kā mākou ʻōnaehana nerve. Hoʻoulu ia i ka make mālie o kā mākou mau neurons motor . I kēia manawa ke noʻonoʻo nei paha ʻoe i ke ʻano o kēia mau neurons motor. ʻO kēia nā hunaola nerve e hoʻomalu i ko mākou mau ʻiʻo. He mea nui kēia mau neurons motor no kā mākou mau hana āpau, mai ka hanu ʻana, ke kamaʻilio ʻana, ke ale ʻana, a me ka hele wāwae ʻana.

E noʻonoʻo, mai ko kākou lolo (ka mea a kākou e kapa nei he mau neurons motor luna ) hiki mai nā leka i ko kākou kaula iwi kuamoʻo. Mai laila mai, hele kēlā mau leka ma o nā neurons motor lalo a hiki i nā ʻiʻo o ko kākou kino. ʻO kēia mau neurons motor luna ka mea e haʻi aku ai i nā neurons motor lalo, "ʻAe, e hoʻoneʻe i kēia ʻiʻo e like me kēia."

I kēia manawa, he aha ka mea e hana ʻia ke hiki ʻole i kēia mau aʻalolo haʻahaʻa ke hoʻouna i nā leka i nā ʻiʻo? Hoʻomaka mālie nā ʻiʻo e nāwaliwali a emi iho (muscle atrophy) . Eia kekahi, ke hiki ʻole i nā aʻalolo haʻahaʻa ke hoʻouna i nā hōʻailona i nā aʻalolo haʻahaʻa, lilo nā ʻiʻo i ʻoʻoleʻa a pane nui ( hyperreflexia ). Hoʻolilo kēia i mea paʻakikī iā mākou ke hana i nā neʻe manawaleʻa, a hana mālie loa lākou. I ka hala ʻana o ka manawa, hiki iā mākou ke nalowale i ka hiki ke hele a kāohi i nā neʻe ʻē aʻe.

ʻO ka mea nui, ʻaʻohe lāʻau lapaʻau no ka maʻi neurone motor i kēia manawa. He mau maʻi holomua kēia e ʻoi aku ka ʻino i ka hala ʻana o ka manawa. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua i ka hōʻemi ʻana i ka hopena o ke kūlana.

He aha nā ʻano MND i loaʻa?

Hoʻokaʻawale nā ​​kauka i ka MND e like me ka hopena o nā aʻalolo motika o luna, nā aʻalolo motika o lalo, a i ʻole nā ​​​​mea ʻelua. Aia kekahi mau ʻano nui o ka MND:

ʻO ka maʻi ʻaʻai Amyotrophic lateral sclerosis (ALS)

ʻO kēia ke ʻano maʻamau o ka MND. Kapa pū kekahi poʻe iā ia ʻo ka maʻi ʻo Lou Gehrig . Hoʻopilikia ka ALS i nā aʻalolo motor o luna a me lalo. Hiki i kēia ke kumu o ka nalowale koke ʻana o ka mana o nā ʻiʻo, a laila e alakaʻi ai i ka maʻi lolo . Hoʻohana pinepine nā kauka he nui i nā huaʻōlelo ALS a me ka maʻi neurone motor.

ʻO ka maʻi bulbar holomua (PBP)

ʻO kēia ka mea e hoʻouka mai nei iā kākou.ʻO nā aʻalolo hoʻokele haʻahaʻa e pili ana i ka lolo (ʻāpana bulbar). Hoʻomalu ko mākou lolo i nā ʻiʻo e pono ai no nā mea e like me ka ʻōlelo ʻana, ka nau ʻana, a me ka moni ʻana. ʻO kekahi inoa no PBP ʻo ia ka "progressive bulbar atrophy".

ʻO ka sclerosis ʻaoʻao mua (PLS)

Hoʻopilikia wale kēia i nā aʻalolo neʻe o luna. ʻO ka hapa nui, hoʻopilikia mua kēia maʻi i nā wāwae. A laila hoʻopilikia ia i nā lima, nā lima, a me ke kino. ʻO ka hope loa, hiki iā ia ke hoʻopilikia i nā ʻiʻo i hoʻohana ʻia no ka ʻōlelo ʻana, ka moni ʻana, a me ka nau ʻana i ka meaʻai.

ʻO ka atrophy ʻiʻo holomua (PMA)

Hoʻopilikia wale ia i nā aʻalolo motor haʻahaʻa. ʻOi aku ka maʻamau o ke ʻano i nā kāne, a ulu pinepine ia i ka wā ʻōpio ma mua o nā MND ʻē aʻe i hoʻomaka i ka pākeke. ʻO nā hōʻailona mua ka nāwaliwali ma nā lima, a laila laha i ke kino haʻahaʻa. Hiki iā ia ke hoʻopilikia i ke kino a me ka hanu ʻana.

ʻO ka atrophy ʻiʻo o ka iwi kuamoʻo (SMA)

He ʻano hoʻoilina kēia e hoʻopilikia ai i nā aʻalolo neʻe haʻahaʻa. Ua manaʻo ʻia hoʻi ʻo ia ke kumu nui o ka make ʻana o nā pēpē . ʻO nā loli ʻana i ka gene `SMN1` e hopena i ka nalowale ʻana o ka protein `SMN` . Hoʻopau mālie kēia i nā aʻalolo neʻe haʻahaʻa, e hoʻoulu ai i ka nawaliwali a me ka ʻiʻo, a i ka hopena, ka make.

ʻO ka maʻi o Kennedy

Ua pili ia i kahi gene ma ka chromosome X i nā kāne. Hoʻokumu ʻia ia e nā mutations i loko o ka gene receptor androgen . I ka hala ʻana o ka manawa, ulu ka nāwaliwali ma nā lima a me nā wāwae, e hoʻomaka pinepine ana ma nā wahi pelvic a i ʻole poʻohiwi. Hiki nō hoʻi ke loaʻa ka ʻeha a me ka maneʻo ma nā lima a me nā wāwae.

ʻO ka maʻi ma hope o ka polio (PPS)

Loaʻa ia i nā poʻe i ho'ōla ʻia mai ka polio. Hiki ke loaʻa a hiki i ʻehā mau makahiki ma hope o ko lākou maʻi mua. Hiki i ka Polio ke hōʻeha nui i nā aʻalolo neʻe. ʻO nā hōʻailona o ka PPS e komo pū me ka nāwaliwali o nā ʻiʻo a me nā hono, ka luhi, ka ʻeha e hoʻonui ana i ka hala ʻana o ka manawa, ka ʻūlū ʻana a me ka nawaliwali o nā ʻiʻo, a me ka hiki ʻole ke hoʻomanawanui i ke anu.

He aha nā hōʻailona o ka MND?

ʻAʻole hiki koke mai nā hōʻailona o MND, akā, lohi iki. I nā pae mua, ʻaʻole paha e ʻike maopopo ʻia.

Nā hōʻailona mua

• Ka paʻakikī i ka piʻi ʻana i nā alapiʻi a i ʻole ka hele pinepine ʻana ma muli o ka nāwaliwali o nā wāwae a i ʻole nā ​​kuʻekuʻe wāwae.
• ʻŌlelo ʻino (dysarthria) .
• Ka paʻakikī i ka ale ʻana i ka meaʻai .
• Ka nāwaliwali o ka paʻa ʻana ma luna o kahi mea āu e paʻa nei. Eia kekahi laʻana, he paʻakikī paha ke pihi i kahi pālule, wehe i kahi ʻōmole, a i ʻole e hāʻule mau ana nā mea ma kou lima i ka papahele.
• Nā ʻūlū a me nā ʻūmiʻi o nā ʻiʻo.
• ʻO ka pohō kaumaha ma muli o ka wīwī o nā lima a me nā wāwae.
• ʻAʻole hiki ke hoʻōki i ka ʻakaʻaka a i ʻole ka uē ʻana i nā manawa kūpono ʻole (pseudobulbar affect) . Ua like kēia me ka ʻakaʻaka ke kaumaha ʻoe, a i ʻole ke kaumaha ke hauʻoli ʻoe.

Nā hōʻailona ʻē aʻe

Ma waho aʻe o kēia mau hōʻailona mua, hiki ke ʻike ʻia nā hōʻailona ʻē aʻe:

• Paʻakikī ka hanu ʻana (hanu ʻole) .
• Paʻakikī ka hanu ʻana i ka wā e moe ana ma kahi moe.
• Nā maʻi umauma pinepine.
• Nā maʻi hiamoe (hiamoe hoʻopilikia) .
• Nā pilikia o ka nānā ʻana a me ka hoʻomanaʻo .
• Ka huikau .
• ʻEha poʻo kakahiaka .
• Luhi .

He aha ke kumu o ka MND?

E like me kā mākou i kamaʻilio ai ma mua, hoʻokumu ʻia ka MND e nā pilikia me kā mākou mau neurons motor. Hoʻōki mālie kēia mau cell i ka hana pono ʻana i ka hala ʻana o ka manawa. Eia nō naʻe, ʻaʻole maopopo i nā mea noiʻi ke kumu o kēia .

He hoʻoilina ka MND

He mau maʻi hoʻoilina kekahi mau maʻi MND, ʻo ia hoʻi, ua hoʻoili ʻia lākou ma o nā lālā o ka ʻohana ma o nā genes. I kēia mau hihia, ua hoʻokumu ʻia ka maʻi e kahi loli i loko o hoʻokahi gene. Hiki ke hoʻoilina ʻia kēia mau mea ma kekahi mau ʻano nui:

• ``Autosomal dominant``: I kēia hihia, ʻoiai inā hoʻoilina ʻoe i hoʻokahi kope o ka gene i hoʻololi ʻia mai hoʻokahi wale nō o nā mākua i hoʻopilikia ʻia, aia nō ka pilikia o ka hoʻomohala ʻana i ka maʻi.
• ``Autosomal recessive``: I kēia hihia, i mea e loaʻa ai ka maʻi, pono ʻoe e loaʻa i ʻelua kope o ka gene i hoʻololi ʻia mai nā mākua ʻelua.
• 'Hoʻoilina i hoʻopili ʻia me X': Ma kēia, lawe ka makuahine i kēia gene ma kahi chromosome X a hoʻoili i ka maʻi i kāna mau keiki kāne.

Nā kumu ʻē aʻe

Hiki ke hoʻoulu ʻia nā kūlana MND ʻaʻole i hoʻoilina ʻia e nā kumu like ʻole. Hiki i kēia ke komo pū me nā virus, nā mea ʻawahia, nā genetics, a me nā kumu kaiapuni .

ʻO wai ka mea i ʻoi aku ka nui o ka pilikia o ka hoʻomohala ʻana i ka MND?

Hoʻopilikia pinepine ka MND i nā kānaka ma waena o 60 a me 70 mau makahiki. Eia nō naʻe, hiki iā ia ke hoʻopilikia i nā keiki a me nā pākeke o kēlā me kēia makahiki. Inā loaʻa i kahi hoahānau pili o ʻoe ka MND, a i ʻole kahi kūlana like i kapa ʻia ʻo frontotemporal dementia , ua piʻi aʻe kou pilikia o ka loaʻa ʻana o ka MND.

Pehea e ʻike ai nā kauka i ka MND?

Hiki ke paʻakikī ke ʻike mua i ka MND, no ka mea, ʻaʻohe hoʻāʻo kikoʻī no ia mea. Inā nāwaliwali mālie kou mau ʻiʻo me ka ʻole o ka ʻeha a i ʻole ka nalowale o ka ʻike, hiki i kāu kauka ke kānalua i ka MND.

Nā nīnau i nīnau ʻia e ke kauka

Hiki iā ʻoe ke nīnau iā ʻoe iho i nā nīnau e like me:

• He aha nā ʻāpana o ke kino i hoʻopilikia ʻia ?
• I ka manawa hea i hoʻomaka ai nā hōʻailona?
• Nā hōʻailona muaHe aha?
• Ua loli anei nā hōʻailona i ka hala ʻana o ka manawa?

Nā hoʻāʻo i hana ʻia

Hiki ke hana ʻia kekahi mau hoʻokolohua e kōkua i ka ʻike ʻana i ka maʻi:

• Electromyography (EMG): Hoʻopaʻa kēia i ka hana uila o kou mau ʻiʻo. Hiki i kēia ke kōkua i ka hoʻoholo ʻana inā paha aia ka pilikia ma nā ʻiʻo, nā aʻalolo, a i ʻole ka neuromuscular junction.
• Nā haʻawina hoʻoili aʻalolo: Ke ana kēia i ka wikiwiki o ka hoʻouna ʻana o nā aʻalolo i nā impulses.
• ʻO ke kiʻi ʻana o ka resonance imaging (MRI): Hana ʻia kahi MRI o ka lolo, a i kekahi manawa he MRI o ke kaula spinal, e nānā i nā mea ʻē aʻe e hiki ke hoʻoulu i nā hōʻailona like.

I mea e hōʻole ai i nā kūlana olakino ʻē aʻe, hiki i ke kauka ke noi i nā hoʻokolohua hou aʻe:

• Nā hoʻāʻo koko
• Nā hoʻāʻo mimi
• ʻO ke kī ʻana o ka iwi kuamoʻo (lumbar puncture)
• Nā hoʻokolohua genetic

Me ka hala ʻana o ka manawa, e ʻike maopopo ʻia nā hōʻailona o MND a hiki i kekahi manawa ke hiki ke ʻike ʻia ka maʻi me ka ʻole o nā hoʻokolohua.

He aha nā lāʻau lapaʻau no MND?

ʻAʻohe lāʻau lapaʻau kūikawā no ka maʻi neurone motor. Eia nō naʻe, ke hoʻomau nei nā mea noiʻi i ke aʻo ʻana i nā ala palekana a maikaʻi hoʻi e mālama ai i ke ʻano.

Pono ʻoe e hana pū me kahi hui o nā kauka e kōkua iā ʻoe e hoʻokele i kāu mau hōʻailona. Hiki i nā lāʻau lapaʻau MND ke komo pū me:

• Hoʻōla kino: Kōkua kēia i ka mālama ʻana i kou ikaika o nā ʻiʻo a mālama i kou mau hono i ka palupalu.
• Inā pilikia ʻoe i ka moni ʻana , hiki ke hāʻawi ʻia iā ʻoe ka meaʻai ma o kahi ʻōmole (gastrostomy tube) i hoʻokomo ʻia ma ka paia ʻōpū i loko o ka ʻōpū.

Nā lāʻau lapaʻau

Kōkua kekahi mau lāʻau lapaʻau i nā poʻe he nui me MND e hōʻoluʻolu i kā lākou mau hōʻailona:

• Baclofen: E hōʻemi i ka ʻoʻoleʻa o nā ʻiʻo a hōʻemi i nā spasms ʻiʻo.
• Phenytoin a i ʻole quinine: E hōʻemi i nā spasms uaua.
• Glycopyrrolate: E hōʻemi i ka salivation.
• Nā lāʻau antidepressants: Kōkua i ka hoʻopau ʻana i ke kaumaha.
• Benzodiazepines: No ka ʻeha e kū mai ana i ka wā e holomua ai ka maʻi.

No ka poʻe me ALS, aia ʻelua mau lāʻau lapaʻau e hiki ke kōkua i ka hoʻolōʻihi ʻana i ke ola a kaohi i ka emi ʻana o ka hana: riluzole lāua ʻo edaravone .

Nui ka poʻe e komo pū ana i nā hoʻokolohua lapaʻau .

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe kekahi mea e like me ka nāwaliwali o nā ʻiʻo, kahi hōʻailona mua paha o MND, pono ʻoe e ʻike i ke kauka. ʻAʻole paha ʻoe he MND, akā ʻo ka loaʻa ʻana o kahi hōʻailona pololei i ka wā hikiwawe loa he mea nui loa ia e loaʻa ai ka mālama a me ke kākoʻo āu e pono ai.

Eia kekahi, inā he hoahānau pili kou me MND a i ʻole frontotemporal dementia a ke hopohopo nei ʻoe e loaʻa paha iā ʻoe ka maʻi, he manaʻo maikaʻi e ʻike i ke kauka. Hiki i kāu kauka ke kuhikuhi iā ʻoe i kahi kākāʻōlelo genetic e kūkākūkā i kāu pilikia.

He aha kāu e manaʻo ai i ka wā e noho ai me kēia maʻi?

He maʻi holomua ka maʻi neurone motor, no laila he mea nui e ʻimi i ke kauka e hoʻomaopopo i kou kūlana a hiki ke kōkua iā ʻoe e ʻike i ka lāʻau kūpono e mālama ai i kou kūlana.

He mea nui hoʻi ka loaʻa ʻana o kahi ʻōnaehana kākoʻo . Ke holomua nei kou kūlana, pono paha ʻoe i ke kōkua hou aku mai nā poʻe ʻē aʻe e hana i nā mea paʻakikī iā ʻoe. E kamaʻilio me nā hoaaloha a me ka ʻohana, a i ʻole e nīnau i kāu kauka e pili ana i nā ala e loaʻa ai ke kākoʻo mai kou kaiāulu. E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi.

He aha ke kūlana ola o kekahi kanaka me MND?

ʻO ka mea pōʻino, hiki i ka maʻi neurone motor ke hoʻopōkole nui i kou ola . ʻO kēia nā maʻi e holomua i ka hala ʻana o ka manawa a alakaʻi i ka make. Eia nō naʻe, ʻokoʻa ka lōʻihi o ka hiki ʻana i kēlā kiko mai kēlā kanaka a i kēia kanaka. Ola kekahi poʻe no nā makahiki he nui, i kekahi manawa he mau makahiki he ʻumi paha, ma mua o ka make ʻana mai nā hopena o kēia mau maʻi. Hiki i kāu kauka ke hāʻawi iā ʻoe i kahi ʻike maikaʻi aʻe i ka prognosis/view o kou kūlana.

ʻO ka mea nui loa - ka mea āu e ʻōlelo ai

ʻO ka ʻike ʻana ua loaʻa iā ʻoe ka maʻi neurone motor (MND) hiki ke lilo i mea weliweli a hoʻoluhi nui . E hoʻomanaʻo, ʻaʻole ia kou hewa . ʻAʻole ʻoe i hana hewa i kekahi mea. ʻAʻole ia e hoʻololi i kou ʻano - akā hiki iā ia ke hoʻololi iki i ke ola. ʻOiai paha e kū ana ʻoe i nā pilikia i ka holomua ʻana o ka maʻi, aia nā lāʻau lapaʻau e hiki ke kōkua iā ʻoe e hoʻokele i nā hopena. E hilinaʻi i kāu mau hoaaloha a me kou ʻohana no ke kākoʻo. Inā ʻoe e hakakā nei e hoʻomanawanui a makemake paha i ke kākoʻo hou aku, e kamaʻilio me kāu kauka. Hiki iā lākou ke kōkua iā ʻoe e ʻike i nā kumuwaiwai āu e pono ai, a e hōʻoia e loaʻa iā ʻoe ke kōkua āu e pono ai i ka hala ʻana o ka manawa.

ʻO ka maʻi neurone motor, MND, maʻi neurological, nāwaliwali o nā ʻiʻo, ALS, nā hunaola nerve, hoʻopau ʻana o nā ʻiʻo