Myasthenia gravis

ʻO Myasthenia gravis kahi maʻi neuromuscular. Hoʻopili nā maʻi Neuromuscular i nā mākala a me nā aʻalolo e kāohi iā lākou.

Manaʻo ʻia ʻo Myasthenia gravis kahi ʻano o ka maʻi autoimmune. Hana ʻia kahi maʻi autoimmune ke hōʻewa hewa ka ʻōnaehana immune i nā mea olakino olakino. ʻO nā Antibodies nā protein i hana ʻia e ka ʻōnaehana pale o ke kino ke ʻike ia nā mea hōʻeha. Hiki ke hana ʻia nā Antibodies ke kuhi hewa ka ʻōnaehana pale i ke kino olakino i mea waiwai, e like me ka hihia o ka myasthenia gravis. I ka poʻe me ka myasthenia gravis, hana ke kino i nā mea ʻino e ālai i nā hunaola mai ka loaʻa ʻana o nā leka (neurotransmitters) mai nā pūnana nerve.

I kekahi mau hihia, pili ka myasthenia gravis i nā tumors o ka thymus (kahi lālā o ka ʻōnaehana pale).

Hiki i ka Myasthenia gravis ke hoʻopili i ka poʻe i kēlā me kēia makahiki. ʻO ka mea maʻamau i nā wahine ʻōpio a me nā kāne ʻelemakule.

Hoʻokumu ʻo Myasthenia gravis i ka nāwaliwali o nā mākala manawaleʻa. ʻO kēia nā mākala hiki iā ʻoe ke kaohi. ʻAʻole hoʻopili pinepine ʻia nā mākala autonomic o ka puʻuwai a me ka digestive. E nāwaliwali ka nāwaliwali o nā mākala myasthenia gravis me ka hana a hoʻomaikaʻi me ka hoʻomaha.

Hiki i kēia nāwaliwali ke alakaʻi i nā ʻōuli like ʻole, e like me:

• ʻO ka paʻakikī hanu no ka nāwaliwali o nā mākala o ka umauma
• ʻO ke kani ʻana a i ʻole ka ale ʻana i ka paʻakikī, ke kumu o ka gagging pinepine ʻana, a me ka haʻalulu ʻana
• Piʻi i ka piʻi ʻana i nā alapiʻi, hāpai hāpai ʻana i nā mea, a i ʻole ke piʻi aʻe nei mai kahi noho
• Ke kamaʻilio paʻakikī ʻana
• Ke poʻo poʻo a me nā lihilihi
• ʻO ka maʻi lōlō a i ʻole nāwaliwali o nā mākala maka
• Ka luhi
• Hoarseness a hoʻololi paha i ka leo
• Hōʻike pālua
• Ka paʻakikī e mālama mau ana i nā maka

E hana ka mea mālama ola i kahi hoʻokolohua kino. Hoʻopili kēia i kahi ʻōnaehana ʻōnaehana kikoʻī (neurological). Hōʻike paha kēia:

• ʻO ka nāwaliwali o nā mākala, me nā mākala maka e hoʻopili pinepine ʻia
• Nā reflexes maʻamau a me ka manaʻo (sensation)

ʻO nā hoʻāʻo e hana ʻia ana paha:

• ʻO nā kinetona receptor Acetylcholine e pili ana i kēia maʻi
• ʻO CT a MRI paha e nānā i ka umauma e nānā i kahi tumo
• Nerve conduction haʻawina e hoʻāʻo i ka wikiwiki o nā neʻe uila e neʻe ma waena o kahi aʻalolo
• Electromyography (EMG) e hoʻāʻo i ke olakino o nā mākala a me nā ʻalolo e kaohi i nā mākala
• Nā hoʻāʻo ʻana o ka hana pulmonary e ana i ka hanu ʻana a me ka maikaʻi o ka hana ʻana o nā māmā
• Hōʻike ʻo Edrophonium e ʻike inā hoʻohuli kēia lāʻau i nā ʻōuli no kahi manawa pōkole

ʻAʻohe lāʻau i ʻike ʻia no ka myasthenia gravis. Hiki i ka lāʻau lapaʻau ke ʻae iā ʻoe e loaʻa nā wā me ka ʻole o nā ʻōuli (remission).

Hiki i nā loli nohona ke kōkua pinepine iā ʻoe e hoʻomau i kāu hana i kēlā me kēia lā. E ʻōlelo paha ka mea aʻe:

• Hoʻomaha i ka lā holoʻokoʻa
• Ke hoʻohana nei i ka pale maka inā pilikia ka ʻike lua
• Hōʻalo i ke koʻikoʻi a me ka wela, i hiki ke hōʻino i nā ʻōuli

ʻO nā lāʻau i kauoha ʻia e hoʻopili ʻia:

• ʻO Neostigmine a i ʻole pyridostigmine e hoʻomaikaʻi i ke kamaʻilio ma waena o nā aʻa a me nā mākala
• ʻO Prednisone a me nā lāʻau ʻē aʻe (e like me azathioprine, cyclosporine, a i ʻole mycophenolate mofetil) e kāohi i ka pane ʻana o ka ʻōnaehana pale inā loaʻa nā hōʻailona koʻikoʻi a ʻaʻole i holo pono nā lāʻau ʻē aʻe.

ʻO nā kūlana pilikia ka hōʻeha o ka nāwaliwali o nā mākau hanu. Hiki i kēia mau hoʻouka ke hana me ka ʻole o ka ʻōlelo aʻoaʻo ke lawe ʻia ka lāʻau nui a liʻiliʻi paha. ʻAʻole mau kēia mau hoʻouka ma mua o kekahi mau pule. Pono ʻoe e ʻae ʻia i ka haukapila, kahi e pono ai ke kōkua ʻana i ka hanu me kahi ea.

Hiki ke hoʻohana ʻia kahi hana i kapa ʻia plasmapheresis e kōkua ai i ka hoʻopau ʻana i ka pilikia. Hoʻopili kēia hana i ka wehe ʻana i ka ʻaoʻao maopopo o ke koko (plasma), kahi e loaʻa ai nā mea ʻino. Hoʻololi ʻia kēia me ka plasma i hāʻawi manawaleʻa ʻia me ka ʻole o nā antibodies, a i ʻole me nā wai ʻē aʻe. Hiki paha i ka Plasmapheresis ke kōkua i ka hōʻemi ʻana i nā hōʻailona no 4 a 6 mau pule a hoʻohana pinepine ʻia ma mua o ke kaha.

Hiki ke hoʻohana ʻia kahi lāʻau i kapa ʻia ʻo immunoglobulin intravenous (IVIg)

ʻO ke ʻoki ʻana e hemo ai i ka timus (thymectomy) e hopena paha i ke kala mau ʻana a i ʻole ka liʻiliʻi o ka pono no nā lāʻau, ʻoiai ke loaʻa kahi tumo.

Inā pilikia ʻoe i ka maka, hiki i kāu kauka ke ʻōlelo aku i nā prisma aniani e hoʻomaikaʻi i ka ʻike. Paipai ʻia ke ʻoki kino e mālama i nā mākala o kou maka.

Hiki i ke olakino kino ke kōkua i ka mālama ʻana i ka ikaika o kou mākala. He mea nui loa kēia no nā mākala e kākoʻo i ka hanu.

Hiki i kekahi mau lāʻau ke hoʻopōʻino i nā ʻōuli a pono e hōʻalo ʻia. Ma mua o ka lawe ʻana i ka lāʻau, nīnau i kāu kauka inā maikaʻi inā lawe ʻoe.

Hiki iā ʻoe ke hoʻomāmā i ke koʻikoʻi o ka maʻi ma ke komo ʻana i kahi hui kākoʻo myasthenia gravis. ʻO ke kaʻana ʻana me nā poʻe ʻē aʻe i loaʻa nā ʻike a me nā pilikia maʻamau e hiki ke kōkua iā ʻoe e noʻonoʻo ʻole.

ʻAʻohe mea ola, akā hiki ke kala i ka wā lōʻihi. Hiki paha iā ʻoe ke kaohi i kekahi hana i kēlā me kēia lā. ʻO ka poʻe i loaʻa nā ʻōuli o ka maka (ocular myasthenia gravis), hiki ke hoʻomohala i ka myasthenia ākea ma kahi o ka manawa.

Hiki i ka wahine me ka myasthenia gravis ke hāpai, akā he mea nui ka mālama prenatal. Nawaliwali paha ka pēpē a koi aku i nā lāʻau no kekahi mau pule ma hope o ka hānau ʻana, akā ʻaʻole e hoʻomohala ka maʻi maʻamau.

Hiki ke kumu i nā pilikia hanu hoʻoweliweli. Kapa ʻia kēia he pilikia myasthenic.

ʻOi aku ka makaʻu o ka poʻe me ka myasthenia gravis no nā maʻi autoimmune ʻē aʻe, e like me ka thyrotoxicosis, rheumatoid arthritis, a me systemic lupus erythematosus (lupus).

Kāhea i kāu mea mālama ola inā e hoʻomohala ʻoe i nā ʻōuli o ka myasthenia gravis.

E hele i ka lumi pilikia a kāhea paha i ka helu kūloko kūloko (e like me 911) inā he paʻakikī kou i ka hanu ʻana a i ʻole ka ale ʻana i nā pilikia.

Maʻi Neuromuscular - myasthenia gravis

• Nā mākala mua ma mua
• Ptosis - hāʻule ka lihilihi maka
• ʻŌnaehana ʻōnaehana waena a me ka ʻōnaehana ʻōnaehana peripheral

Chang CWJ. Myasthenia gravis a me Guillain-Barré syndrome. I: Parrillo JE, Dellinger RP, eds. Lapaʻau mālama koʻikoʻi: nā loina o ka ʻike a me ka hoʻokele i ka makua. 5th lā. Piladelapia, PA: Elsevier; 2019: chap 61.

Sanders DB, Guptill JT. Nā maʻi o ka hoʻoili neuromuscular. 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 109.

Sanders DB, Wolfe GI, Benatar M, et al. Ke alakaʻi ʻaha kūkā laulā no ka mālama ʻana i ka myasthenia gravis: hōʻuluʻulu mana. Neurology. 2016; 87 (4): 419-425. PMID: 27358333 www.ncbi.nlm.nih.gov/pubmed/27358333.