Poho koke ka ʻike? E aʻo kākou e pili ana i kēia maʻi laha ʻole (Primary Periodic Paralysis)

E noʻonoʻo, aia ʻoe i ke olakino maikaʻi a laila mae koke kekahi o kou mau lima a i ʻole kou mau wāwae a nalowale ʻoe. A i ʻole e nāwaliwali loa kou kino holoʻokoʻa a hiki ʻole iā ʻoe ke neʻe. He mea maʻamau ka makaʻu nui i ka wā e hana ʻia ai kekahi mea e like me kēia. Manaʻo ka poʻe he nui kēia ma muli o ka luhi a i ʻole kekahi mea ʻē aʻe. Eia nō naʻe, i kekahi manawa aia paha he kumu olakino ma hope o kēia e pono e kūkākūkā hohonu ʻia. I kēia lā ke kamaʻilio nei mākou e pili ana i kahi ʻano olakino kakaʻikahi akā koʻikoʻi loa e makaʻala.

I ka ʻōlelo maʻalahi, he aha ka Primary Periodic Paralysis (PPP)?

ʻO ka Primary Periodic Paralysis, a i ʻole PPP no ka pōkole, he hui ia o nā maʻi kakaikahi e hoʻopilikia koke i ko kākou mau ʻiʻo. ʻO ka mea e hana ʻia, me ka ʻole o ka ʻōlelo aʻo , lilo koke kou mau ʻiʻo i nāwaliwali, ʻoʻoleʻa, a i ʻole ka lolo loa. Hiki i kēia maʻi ke mau no kekahi mau minuke no kekahi poʻe, a no kekahi poʻe hiki ke mau no nā lā.

ʻO ke kumu nui o kēia aia i loko o ko kākou mau ʻiʻo. E noʻonoʻo he mau kahawai liʻiliʻi ko kākou mau ʻiʻo. ʻO kēia mau kahawai kahi e komo ai a puka ai nā minelala koʻikoʻi e like me ka potassium, sodium, a me ka calcium i loko o nā ʻiʻo. I mea e hana pono ai ka ʻiʻo, pono ke kaulike kūpono o ka nui o kēia mau minelala i loko a ma waho o ke kelepona. ʻO ka mea e hana ʻia i kekahi me PPP, ua nalowale kēia kaulike ma muli o kahi kīnā i loko o kēia mau kahawai. A laila ʻaʻole hiki i nā ʻiʻo ke hana pono.

ʻO ka hapa nui o ka manawa, hoʻomaka kēia mau hōʻailona i ka wā kamaliʻi a i ʻole ka wā ʻōpio, akā ʻaʻole paha e hōʻike kekahi poʻe i kekahi mau hōʻailona a hiki i ko lākou mau makahiki 60 a 70 paha.

He aha nā ʻano nui o kēia maʻi?

Ua māhele ʻia ka PPP i kekahi mau ʻano nui. Hoʻopili nui ʻia kēia hoʻokaʻawale ʻana e ka hemahema o ke kahawai mineral i loko o kāu mau ʻiʻo.

He aha nā kumu a me nā kumu "hoʻonāukiuki" no kēia kūlana?

Hoʻokumu ʻia ka PPP e kahi kīnā genetic . I ka ʻōlelo maʻalahi, hoʻokumu ʻia ia e kahi kīnā i loko o nā genes e kāohi ana i nā kahawai i loko o nā ʻiʻo a mākou i kamaʻilio ai ma mua. ʻO ka hapa nui o ka manawa, hoʻoilina ke keiki i kēia gene hemahema mai kekahi o ko lākou mau mākua. Eia naʻe, he mea kakaikahi loa, hiki i ke keiki ke loaʻa i ka maʻi ʻoiai inā ʻaʻohe o nā mākua kēia gene.

I kēia manawa, e nānā kākou i ka mea e hiki ai ke "hoʻoulu" i kēia nāwaliwali o nā ʻiʻo. Hiki ke ʻokoʻa kēia mau mea mai kēlā kanaka a kēia kanaka.

• ʻAi: Ka ʻai nui ʻana i nā meaʻai waiwai i ka potassium (ma ka hyperKPP) a i ʻole ka potassium haʻahaʻa (ma ka hypoKPP). Eia kekahi, ka ʻai ʻana i nā meaʻai kiʻekiʻe i nā carbohydrates (starch/sugar).
• Hoʻoikaika Kino : I ka wā e hoʻomaha ai ma hope o ka hoʻoikaika kino kaumaha.
• Hoʻomaha: Noho ma ke kūlana like no ka manawa lōʻihi, i ke ala ʻana i ke kakahiaka, a ma hope paha o ka hiamoe ʻana.
• Nā kumu ʻē aʻe: Ke kaumaha, ka ʻike ʻia ʻana i ke anu nui, ka inu ʻana i ka waiʻona, a me kekahi mau lāʻau lapaʻau (e.g., nā lāʻau hoʻomaha ʻiʻo, kekahi mau lāʻau hānō, nā lāʻau hoʻomaha ʻeha).

He aha nā hōʻailona nui?

ʻO ka hōʻailona nui ka nāwaliwali koke o nā ʻiʻo a i ʻole ka maʻi lolo piha. Hiki i kēia ke hoʻopilikia i hoʻokahi lima a wāwae paha i ka manawa hoʻokahi, a i ʻole ke kino holoʻokoʻa i nā manawa ʻē aʻe.

Ma waho aʻe o kēia hōʻailona nui, hiki i kekahi poʻe ke ʻike i nā hōʻailona ʻē aʻe ke ʻike lākou i kēia nāwaliwali:

• Ka nāwaliwali o nā ʻiʻo maka
• ʻEha ʻiʻo a me ka ʻoʻoleʻa
• Puʻuwai puʻuwai kūpono ʻole
• Paʻakikī i ka hanu ʻana a i ʻole ka moni ʻana

HypoKPP (ʻano potassium haʻahaʻa)

Hiki ke ʻike pinepine ʻia nā hōʻailona i hoʻokahi manawa i ka lā, a i ʻole pinepine ʻole i hoʻokahi manawa i ka makahiki. Hiki iā lākou ke mau no hoʻokahi a ʻelua paha hola, a i ʻole hiki iā lākou ke mau no ʻelua a ʻekolu paha lā. I ka hala ʻana o ka manawa, hiki i nā ʻiʻo ke lilo i nāwaliwali mau loa.

HyperKPP (ʻano o ka potassium i hoʻonui ʻia)

He pōkole paha ka nāwaliwali, akā hiki ke loaʻa pinepine. Hiki ke hiki koke mai nā hōʻailona, ​​a i kekahi manawa e mau ana no kekahi mau minuke. ʻOiai inā ʻaʻohe nāwaliwali, hiki iā ʻoe ke manaʻo i ka ʻūlū ʻana o nā ʻiʻo a i ʻole ka ʻoʻoleʻa.

ʻO ka maʻi Andersen-Tawil (ATS)

Ma waho aʻe o ka nāwaliwali o nā ʻiʻo, ʻike ʻia nā hōʻailona kino ʻē aʻe.

• Puʻuwai wikiwiki a ʻano ʻole paha
• Scoliosis
• Nā manamana lima me ka ʻupena
• ʻOi aku ka liʻiliʻi o nā lima a me nā wāwae ma mua o ka maʻamau
• ʻO kekahi mau loli o ka helehelena (e.g., lae ākea, pepeiao haʻahaʻa)

Pehea e ʻike pono ai i kēia maʻi?

No ka mea, he maʻi laha ʻole ka PPP a ua like kona mau hōʻailona me nā maʻi maʻamau ʻē aʻe, hiki ke lōʻihi ka manawa e ʻike pololei ai i ka maʻi. No laila, inā loaʻa iā ʻoe kēia mau hōʻailona, ​​ʻoi aku ka maikaʻi o ke ʻike ʻana i ke kauka loea i nā maʻi e pili ana i ka ʻōnaehana nerve (Neurologist) .

Hiki i kāu kauka ke nīnau i nā nīnau e like me kēia e ʻike ai i ke kūlana:

• I ka manawa hea i hoʻomaka ai kēia mau hōʻailona?
• Aia kekahi poʻe ʻē aʻe o ka ʻohana i loaʻa i ke kūlana like?
• He aha ka mea e hana ʻia ke hiki mai ka nāwaliwali?
• Ua manaʻo paha ʻoe ua hoʻoulu ʻia kēia e kahi meaʻai a hana paha?

Eia kekahi, hiki ke hana ʻia kekahi mau hoʻokolohua e hōʻoia i ka maʻi:

• Nā hoʻāʻo koko: E nānā i nā pae o ka potassium, nā hormones thyroid, a me nā mea ʻē aʻe.
• Electromyography (EMG): No ka hoʻāʻo ʻana i ka hana o nā ʻiʻo a me nā aʻalolo.
• Electrocardiogram (EKG): No ka nānā ʻana i ke kū pololei o ka puʻuwai.
• Biopsy ʻiʻo: Ke lawe ʻana i kahi ʻāpana liʻiliʻi o ke ʻiʻo a nānā iā ia.

Pehea e mālama ai a hoʻokele?

ʻO ke ʻano nui o ka lāʻau lapaʻau no kēia, ʻo ia ka ʻike a me ka pale ʻana i nā mea hoʻonāukiuki . ʻO ke ʻano kēia o ka hana ʻana i kekahi mau hoʻololi i kāu papaʻai a me ka hana hoʻoikaika kino.

Eia kekahi, aia nā lāʻau lapaʻau e kāohi i nā pae potassium i loko o ke kino.

• Ua ʻae ʻia ka lāʻau lapaʻau ʻo Dichlorphenamide (Keveyis) e mālama i ka hypoKPP a me ka hyperKPP.
• I kekahi manawa, ʻōlelo pū nā kauka i kahi lāʻau i kapa ʻia ʻo Acetazolamide (Diamox) .
• Hāʻawi ʻia nā mea hoʻohui potassium i ka poʻe me HypoKPP.
• Hiki i ke kanaka me HyperKPP ke kāohi i ka nāwaliwali i kekahi manawa ma ka inu ʻana i kahi mea inu kō (e.g., soda) i ka wā e hoʻomaka ai.

Hiki iā ʻoe ke kaohi i kēia maʻi i kahi nui ma ka hana ʻana i nā hoʻololi liʻiliʻi i kou nohona.

• ʻAi: Inā loaʻa iā ʻoe ka hyperKPP (potassium kiʻekiʻe), pono ʻoe e kaupalena i nā meaʻai waiwai i ka potassium e like me ka maiʻa, nā lā, ka broccoli, a me nā pīnati. Inā loaʻa iā ʻoe ka hypoKPP (potassium haʻahaʻa), pono ʻoe e kaupalena i nā meaʻai waiwai i ka carbohydrate e like me ka berena, pasta, laiki, a me nā mea ʻono. E kiʻi i ka ʻōlelo aʻo kūpono mai kāu kauka e pili ana i kēia.
• Hoʻoikaika Kino: E like me ka hoʻoikaika kino nui, ʻaʻohe hoʻoikaika kino he mea maikaʻi ʻole. E kamaʻilio me kāu kauka e pili ana i ka pae o ka hoʻoikaika kino kūpono iā ʻoe.