Luhi anei kāu keiki liʻiliʻi ma hope o ka pāʻani ʻana no kekahi manawa? A i ʻole ke ʻano maʻi mau nei ʻo ia, ʻaʻole makemake e ʻai, a hiamoe paha? I kekahi manawa manaʻo mākou he mau mea maʻamau kēia, akā ma hope o kēia he maʻi genetic kakaikahi loa, akā koʻikoʻi loa. I kēia lā ke kamaʻilio nei mākou e pili ana i kekahi o ia ʻano maʻi, ʻo Long-Chain Fatty Acid Oxidation Disorders, a i ʻole LC-FAODs .
I ka ʻōlelo maʻalahi, he aha kēia mau LC-FAOD?
He inoa paʻakikī iki kēia, akā e mālama kākou i ka maʻalahi. Ua like ko kākou kino me ke kaʻa. Pono ia i ka ikehu, a i ʻole ka wahie, e hana. ʻO ka meaʻai a kākou e ʻai ai, ʻo ia ka wahie no ko kākou kino. ʻO ke kaʻina hana e hoʻololi ai i kēia meaʻai i ikehu ua kapa ʻia ʻo ka metabolism .
ʻO nā meaʻai a mākou e ʻai ai, e like me ka ʻaila ʻoliva , ka iʻa , nā ʻavokado, a me ka ʻiʻo, aia kahi mea i kapa ʻia ʻo 'nā waikawa momona'. He kumu ikehu maikaʻi kēia no ke kino. Nui nā ʻano o kēia mau waikawa momona. I waena o lākou, ʻo ke ʻano i kapa ʻia ʻo 'nā waikawa momona kaulahao lōʻihi' e pono ai kahi enzyme kūikawā e hoʻololi iā lākou i ikehu.
ʻAʻohe o nā keiki i hānau ʻia me kahi maʻi genetic i kapa ʻia ʻo LC-FAOD i kēlā enzyme kūikawā i loko o ko lākou kino. No laila, ʻaʻole hiki i ko lākou kino ke hoʻololi i kēia mau waikawa momona kaulahao lōʻihi i ikehu.
He aha ka mea e hana ʻia i kēlā manawa?
1. ʻAʻole i loaʻa i nā ʻōkana koʻikoʻi e like me ka puʻuwai , ka lolo, ke akepaʻa , a me nā ʻiʻo ka ikehu e pono ai.
2. Hōʻiliʻili nā waikawa momona i hiki ʻole ke hoʻololi ʻia i ikehu i loko o kēlā mau ʻōkana a hoʻomaka e hōʻino iā lākou.
Hiki i kēia ke alakaʻi i nā pilikia koʻikoʻi e like me ka maʻi puʻuwai , ka hāʻule ʻana o ke akepaʻa , a me nā pae kō koko haʻahaʻa loa. No laila, he mea nui loa ka ʻike mua ʻana i kēia maʻi a mālama i ka kaohi ʻana i ka ʻai kūpono .
He aha nā hōʻailona o kēia maʻi?
Hiki ke ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka. Aia ia i ke ʻano o ka enzyme i nele a me ke koʻikoʻi o ka maʻi. ʻAʻole paha e hōʻike kekahi poʻe i kekahi mau hōʻailona a hiki i ka wā ʻōpio a makua paha. Akā i nā hihia koʻikoʻi, hoʻomaka nā hōʻailona e ʻike ʻia i loko o nā mahina mua o ke ola.
ʻO nā hōʻailona mua o ka maʻi ʻiʻo puʻuwai i nā pēpē hānau hou he "cardiomyopathy". Hoʻokomo pū kēia me ka paʻakikī o ka hanu ʻana, ka paʻakikī o ka hānai ʻana, a me ka hiamoe nui. Hiki i nā pēpē a me nā keiki liʻiliʻi ke hōʻike i nā hōʻailona mua o nā pilikia o ke akepaʻa, e like me ka melemele o nā maka a me ka ʻili (jaundice), a me nā pae kō koko haʻahaʻa (hypoglycemia).
E nānā kākou i nā kūlana nui e hiki ke hana ma kēia ʻano a he aha nā hōʻailona e pili ana me lākou.
| Kūlana | Nā hōʻailona maʻamau |
|---|---|
| Nā ʻano laulā |
|
| Ke kō koko haʻahaʻa (Hypoglycemia) | |
| Hoʻonui ʻia ka nui o ka ammonia i loko o ke koko (Hyperammonemia) | |
| ʻO ke cardiomyopathy |
ʻO ka mea nui, no kekahi mau keiki, ʻike wale ʻia kēia mau hōʻailona ke maʻi lākou, pōloli, a kaumaha paha.
He aha ke kumu o kēia maʻi?
He maʻi hoʻoilina piha kēia. ʻO ia hoʻi, ua hoʻoilina ʻia mai nā mākua a i nā keiki. I ka ʻōlelo maʻalahi, ʻo ke kumu he mutation i loko o ka gene e kuhikuhi ana i ka hana ʻana o ka enzyme e wāwahi i nā waikawa momona a mākou i kamaʻilio ai.
I mea e loaʻa ai i ke keiki ka maʻi, pono lākou e hoʻoilina i kahi kope o ka gene kīnā mai nā mākua ʻelua . Inā hoʻoilina lākou mai hoʻokahi makua wale nō, he "mea lawe" ke keiki. ʻAʻole lākou e loaʻa nā hōʻailona, akā hiki iā lākou ke hoʻoili i ka gene i kā lākou mau keiki.
ʻAʻole kēia he maʻi lele. ʻAʻole hiki ke laha mai kekahi kanaka a i kekahi kanaka ma kekahi ʻano.
Pehea e ʻike ai i ka maʻi?
Ma nā ʻāina he nui, ua nānā ʻia kēlā me kēia pēpē hānau hou no kēia mau maʻi hoʻoilina. Hana ʻia kēia me kahi hāpana koko liʻiliʻi i lawe ʻia mai ke kuʻekuʻe wāwae i loko o 1-2 mau lā o ka hānau ʻana. Inā hāʻawi kēia hoʻāʻo i kahi hōʻailona o ka maʻi, hana ʻia nā hoʻāʻo hou aʻe e hōʻoia i ka maʻi.
ʻO nā hoʻokolohua nui i hana ʻia no kēia:
- Nā hoʻāʻo koko a me ka mimi: Nānā kēia i nā pae kiʻekiʻe o nā waikawa momona a me nā mea ʻē aʻe i loko o ke koko a me ka mimi.
- Ka hoʻāʻo ʻana i ka hoʻoilina: ʻO kēia wale nō ke ala e hoʻoholo pono ai inā loaʻa iā ʻoe ka LC-FAODS a, inā pēlā, ʻo ia ke ʻano.
Inā loaʻa i kāu keiki nā hōʻailona a mākou i kūkākūkā ai ma mua, he mea nui loa e ʻike koke i kahi kauka pediatric e kūkākūkā i kēia.
Pehea e mālama ʻia ai?
ʻOiai ʻaʻole hiki ke ho'ōla piha ʻia kēia maʻi, me ka mālama pono ʻana , hiki ke pale ʻia nā pilikia koʻikoʻi a hiki ke alakaʻi ʻia kahi ola maʻamau. ʻO nā lāʻau lapaʻau nui e pili ana i nā hoʻololi ʻana i ka meaʻai, nā mea hoʻohui meaʻai kūikawā, a me nā hoʻololi ʻana i ke ʻano o ka nohona.
1. ʻAi
ʻO kēia ka mea nui loa. Pono e hoʻomākaukau ʻia kēia ʻai ma lalo o ke aʻo a ke kauka meaʻai.
- E kaupalena i nā meaʻai kiʻekiʻe i nā waikawa momona kaulahao lōʻihi: Pono e kaupalena ʻia nā meaʻai e like me ka ʻiʻo, kekahi mau aila mea kanu, nā nati, a me ka iʻa.
- E ʻai i nā meaʻai waiwai i ka carbohydrate: No ka loaʻa ʻana o ka ikehu, pono ʻoe e hoʻokomo i nā meaʻai waiwai i nā carbohydrate e like me ka laiki, ka ʻuala, a me ka ʻuala i kāu papaʻai.
- E ʻai i nā meaʻai liʻiliʻi a pinepine: He mea nui e ʻai i nā manawa maʻamau i ka lā e mālama i ka pae kō koko i paʻa.
- E pale i ka hoʻokē ʻai: ʻAʻole maikaʻi ka pōloli no nā hola he 8 a ʻoi.
Inā hāʻule koke ke kō koko o kāu keiki i lalo loa, pono paha lākou e hele i ka haukapila a hāʻawi ʻia i kahi wai kō (IV) i loko o kahi wai saline i hāʻawi ʻia ma ke ʻano intravenous ma ka Emergency Treatment Unit (ETU).
2. Nā mea hoʻohui meaʻai
No ka mea, ʻaʻole hiki i kēia mau maʻi ke hoʻohana i nā waikawa momona kaulahao lōʻihi, hāʻawi ʻia lākou i kahi ʻano momona ʻē aʻe e hiki ai i ke kino ke hoʻololi maʻalahi i ikehu. Ua kapa ʻia kēia mau mea he medium-chain triglycerides (MCTs) . Loaʻa kēia mau mea ma ke ʻano he ʻaila MCT. Hoʻohui ʻia kēia mau MCT i kekahi mau waiū pēpē. E hāʻawi kāu kauka iā ʻoe i ka ʻike hou aku e pili ana i kēia.
3. Lāʻau lapaʻau
Ua ʻāpono ʻia kahi lāʻau i kapa ʻia ʻo Triheptanoin (Dojolvi) no LC-FAODs. Loaʻa wale ia me ke kauoha a kāu kauka .
4. Nā loli o ke ʻano o ka nohona
He mea nui e pale i nā mea hoʻoulu e hoʻonui ai i nā hōʻailona.
- E hōʻalo i ka hoʻoikaika kino nui: E hōʻemi i nā hana e luhi ai ke kino.
- E hoʻokele i ke kaumaha: Hiki i nā hana e like me ka yoga a me ka noʻonoʻo ʻana ke kōkua.
- E pale iā ʻoe iho mai ka maʻi: E kiʻi i nā lāʻau pale āpau a kāu kauka e ʻōlelo ai i ka manawa kūpono. E ʻimi koke i ka lāʻau lapaʻau no ke anu a i ʻole ke kuni liʻiliʻi.
He paʻakikī anei ke ola ʻana me kēia maʻi?
ʻOiaʻiʻo, ʻae. He kūlana kēia no ke ola holoʻokoʻa e pono ai ke mālama ʻia. Pono ʻoe e makaʻala i kāu meaʻai. Pono ʻoe e ʻike i ka mea e hana ai i kahi pilikia. Hiki i kēia ke hoʻokaumaha noʻonoʻo no ke keiki a me nā mākua.
No laila,
- E aʻo pono e pili ana i ka maʻi: ʻo ka ʻike ka mana nui loa.
- E ʻimi i ke kākoʻo noʻonoʻo: Mai kānalua e ʻimi i ke kōkua mai ka ʻohana, nā hoaaloha, a i ʻole ke kākāʻōlelo olakino noʻonoʻo.
- E hoʻomau i ka launa pū me kāu kauka: E nīnau iā ia inā he mau nīnau a hopohopo paha kāu.
ʻOiai he mea kakaikahi kēia maʻi, me ka mālama pono ʻana a me ka mālama ʻana, hiki i ke keiki ke loaʻa kahi ola maikaʻi. ʻO ka mea nui loa, ʻo ia ka ʻike mua ʻana i ka maʻi a hahai i nā ʻōlelo a ke kauka.
Leka Lawe-Home
- He maʻi koʻikoʻi ka LC-FAOD i hoʻoilina ʻia e pale ai i ke kino mai ka hoʻololi ʻana i kekahi ʻano momona i ikehu.
- Hiki ke ʻike ʻia nā hōʻailona e like me ka luhi nui, ka nāwaliwali o nā ʻiʻo, nā pilikia o ka puʻuwai a me ke akepaʻa, a me ke kō koko haʻahaʻa.
- ʻAʻole kēia he maʻi lele. He mea ia i hoʻoilina ʻia mai nā mākua a i nā keiki.
- ʻO nā mea nui o ka mālama ʻana he papaʻai kūikawā, nā mea hoʻopihapiha meaʻai e like me ka ʻaila MCT, a me ka pale ʻana i nā mea e hoʻonui ai i nā hōʻailona.
- Inā loaʻa i kāu keiki kekahi o kēia mau hōʻailona, e ʻike koke i kāu kauka no ka ʻōlelo aʻo. He mea nui loa ka ʻike mua ʻana.
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