ʻO Amyotrophic lateral sclerosis (ALS)

ʻO Amyotrophic lateral sclerosis, a i ʻole ALS, he maʻi ia o nā pūnana nerve i ka lolo, ka lolo a me ka iwi kuamoʻo e kāohi ana i ka neʻe ʻana o ka mākala manawaleʻa.

ʻIke ʻia ʻo ALS ʻo Lou Gehrig maʻi.

Hoʻokahi ma 10 mau hihia o ALS ma muli o kahi kīnā kīnā. ʻAʻole ʻike ʻia ke kumu i nā hihia ʻē aʻe.

I ka ALS, neoneo a make paha nā pūnana nerve mīkini (neurons) a ʻaʻole hiki ke hoʻouna hou i nā leka i nā mākala. ʻO kēia ka hopena e alakaʻi i ka nāwaliwali o nā mākala, twitching, a me ka hiki ʻole ke hoʻoneʻe i nā lima, nā wāwae, a me ke kino. Hele a mānoanoa ke kūlana. Ke pau nā hana o nāʻiʻo i ka umauma, lilo i paʻakikī a hiki ʻole i ka hanu.

Hoʻopili ka ALS ma kahi o 5 o kēlā me kēia 100,000 poʻe ma ka honua holoʻokoʻa.

Loaʻa i kahi lālā o ka ʻohana kahi hoʻoilina hoʻoilina o ka maʻi he mea weliweli ia no ALS. ʻO nā pilikia ʻē aʻe e pili pū me ka lawelawe pūʻali koa.

ʻAʻole hoʻomohala nā ʻōuli a hiki i ka hala ʻana o 50 mau makahiki, akā hiki iā lākou ke hoʻomaka i ka poʻe ʻōpio. Loaʻa i ka poʻe me ALS ka nalo o ka ikaika a me ka hoʻohui ʻana o ka muscle a laila lilo i mea hiki ʻole ʻole iā lākou ke hana i nā hana maʻamau e like me ke piʻi ʻana i luna, e puka ana i waho o kahi noho, a me ka ale ʻana paha.

Hiki i ka nāwaliwali ke hoʻololi i nā lima a me nā wāwae, a i ʻole ka hiki ke hanu a ale paha. Ke piʻi a maikaʻi ʻole ka maʻi, ʻoi aku ka nui o nā pūʻulu puʻupuʻu e ulu i nā pilikia.

ʻAʻole pili ka ALS i nā naʻau (ʻike, ʻala, ʻono, lohe, hoʻopā). Hiki i ka hapa nui o ka poʻe ke noʻonoʻo maʻamau, ʻoiai kahi helu liʻiliʻi e ulu i ka dementia, e hana ana i nā pilikia me ka hoʻomanaʻo.

Hoʻomaka ka nāwaliwali o ka mākala i hoʻokahi ʻāpana o ke kino, e like me ka lima a lima paha, a hele a ʻino a hele a hiki i ka mea penei:

• ʻO ka hāpai hāpai paʻakikī, piʻi piʻi ʻana i nā ʻanuʻu, a me ka hele wāwae
• Hanu paʻakikī
• Pilikia ʻana i ka paʻakikī - ʻūlū maʻalahi, paheʻe, a i ʻole gagging
• Kulu ke poʻo ma muli o ka nāwaliwali o nā ʻāʻī o ka ʻāʻī
• Nā pilikia kamaʻilio, e like me ke ʻano lohi a haʻi ʻole paha o ka ʻōlelo (slurring of words)
• Hoʻololi leo, hoarseness

ʻO nā hopena ʻē aʻe e pili pū ana:

• Kaumaha
• Pākuʻi kaʻiʻo
• ʻOʻoleʻa puʻupuʻu, i kapa ʻia spasticity
• ʻO nā kuʻina Muscle, i kapa ʻia nā fasc fascations
• Kaumaha emi

E nānā ka mea mālama ola iā ʻoe a nīnau e pili ana i kāu mōʻaukala a me nā ʻōuli.

Hōʻike paha ka hoʻokolohua kino:

• Ka nāwaliwali, e hoʻomaka pinepine nei i hoʻokahi wahi
• Haʻalulu ka mākala, spasms, twitching, a i ʻole ka nalo ʻana o nāʻiʻo nāʻiʻo
• Twitching o ke alelo (maʻamau)
• Nā reflexes maʻamau
• Hele wāwae ʻoʻoleʻa a paʻakikī paha
• Hoʻoemi a hoʻonui paha i nā reflexes ma nā hono
• Ka paʻakikī ke kaohi ʻana i ka uē a me ka ʻakaʻaka ʻana (i kekahi manawa i kapa ʻia he incontinence ʻuhane)
• Loaʻa o reflex gag

ʻO nā hoʻāʻo e hana ʻia ana paha:

• Nā hoʻokolohua koko e kāpae i nā kūlana ʻē aʻe
• Hōʻike hanu e ʻike inā hoʻopili ʻia nā mākala māmā
• ʻO Cervical spine CT a i ʻole MRI e ʻike pono ʻaʻohe maʻi a ʻeha paha i ka ʻāʻī, hiki ke hoʻohālikelike iā ALS
• ʻIke uila e ʻike ai i nā aʻa a i ʻole nā ​​mākala e holo pono ʻole
• ʻO ka hoʻāʻo genetic, inā aia kahi moʻolelo ʻohana o ALS
• Head CT a i ʻole MRI e kāpae i nā kūlana ʻē aʻe
• ʻAlllelo noiʻi e ale ana
• Spinal tap (lumbar puncture)

ʻAʻohe lāʻau i ʻike ʻia no ALS. ʻO kahi lāʻau i kapa ʻia riluzole e kōkua i ka hoʻolohi i nā hōʻailona a kōkua i ka poʻe e ola iki.

Loaʻa nā lāʻau lapaʻau i kōkua i ka lohi i ka holomua o nā ʻōuli a kōkua paha i ka poʻe e ola iki.

• Riluzole (Rilutek)
• Edaravone (Radicava)

Nā hana e kāohi ai i nā ʻōuli ʻē aʻe:

• Baclofen a i ʻole diazepam no ka spasticity e hoʻopilikia i nā hana o kēlā me kēia lā
• Trihexyphenidyl a i ʻole amitriptyline no ka poʻe me nā pilikia e ale ana i ko lākou lawai ponoʻī

ʻO ka hoʻōla kino, ka hoʻoponopono hou ʻana, ka hoʻohana ʻana i nā paʻa lima a i ʻole kahi noho huila, a i ʻole nā ​​hana ʻē aʻe e pono ai e kōkua me ka hana muscle a me ke olakino laulā.

ʻO ka poʻe me ALS ka mea e lilo i ka kaupaona. Hoʻonui ka maʻi ponoʻī i ka pono no ka meaʻai a me nā calorie. I ka manawa like, paʻakikī nā ʻai me ka ʻāʻā ʻana a me ka ale ʻana i ka ʻai. I mea e kōkua ai i ka hānai ʻana, hoʻokomo ʻia i kahi ʻōpala i loko o ka ʻōpū. Hiki i kahi dietitian nāna i loea i ALS ke hāʻawi i ka ʻōlelo aʻo e pili ana i ka ʻai olakino.

ʻO nā hāmeʻa hanu pū me nā mīkini e hoʻohana wale ʻia i ka pō, a me ka ea ea mau.

Pono paha ka lāʻau no ke kaumaha inā he kaumaha ke kanaka me ALS. Pono lākou e kūkākūkā i kā lākou makemake e pili ana i ka ea hana hoʻohanohano me ko lākou ʻohana a me nā mea hāʻawi.

He mea nui ke kākoʻo Emotional i ke kāohi ʻana i ka maʻi, no ka mea ʻaʻole pili ka hana noʻonoʻo. Loaʻa paha nā pūʻulu e like me ka ʻAhahui ALS e kōkua i ka poʻe e kūleʻa nei i kēia maʻi.

Loaʻa ke kākoʻo no ka poʻe e mālama nei i kekahi me ALS, a kōkua nui paha.

I ka hala ʻana o ka manawa, lilo ka poʻe me ALS i ka hiki ke hana a mālama iā lākou iho. Kū pinepine ka make ma waena o 3 a 5 mau makahiki o ka hōʻailona. Ma kahi o 1 i ka 4 mau kānaka e ola ana ma mua o 5 mau makahiki ma hope o ka ʻike ʻana. Noho ʻoi aku ka lōʻihi o kekahi poʻe, akā pono lākou i ke kōkua i ka hanu ʻana mai kahi ea a i ʻole kahi hāmeʻa ʻē aʻe.

Nā pilikia o ALS me:

• Ka hanuʻana i ka meaʻai a wai paha (aspiration)
• Loaʻa ka hiki ke mālama iā ʻoe iho
• Hoka maikaʻi ʻole
• Pulumonia
• ʻEha puʻe
• Kaumaha emi

Kāhea i kāu mea hāʻawi inā:

• Loaʻa iā ʻoe nā ʻōuli o ALS, keu hoʻi inā he ʻohana kāu o ka maʻi
• ʻO ʻoe a i ʻole kekahi mea ʻē aʻe i ʻike ʻia me ka ALS a ʻoi aku ka maikaʻi o nā ʻōuli a i ʻole e ulu ana nā ʻōuli hou

ʻO ka hoʻonui ʻana i ka paʻakikī i ka ale ʻana, paʻakikī i ka hanu ʻana, a me nā ʻāpana o ka apnea he mau ʻōuli e pono ai ka nānā koke.

Makemake paha ʻoe e ʻike i kahi kākāʻōlelo genetic inā he moʻolelo ʻohana kāu na ALS.

Maʻi Lou Gehrig; ALS; ʻO ka maʻi neuron luna a lalo hoʻi; ʻO ka maʻi neuron kaʻa

• ʻŌnaehana ʻōnaehana waena a me ka ʻōnaehana ʻōnaehana peripheral

ʻO Fearon C, Murray B, Mitsumoto H. Nā maʻi o nā neurons kaʻa kiʻekiʻe a me lalo. I: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. ʻO Bradley's Neurology i ka hana lapaʻau. ʻEpekema 7 Piladelapia, PA: Elsevier; 2016: chap 98.

ʻO Shaw PJ, Cudkowicz ME. ʻO ka amyotrophic lateral sclerosis a me nā maʻi neuron kaʻa ʻē aʻe. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 391.

van Es MA, Hardiman O, Chio A, et al. ʻO scotrosis lateral ʻo Amyotrophic. Lancet. 2017; 390 (10107): 2084-2098. PMID: 28552366 pubmed.ncbi.nlm.nih.gov/28552366/.