Ua hopohopo paha ʻoe e pili ana i ke ʻano o ke poʻo o kāu keiki liʻiliʻi, a i ʻole e pili ana i nā ʻōʻō pinepine i loaʻa iā ia? I kekahi manawa, aia paha kahi maʻi lapaʻau kakaʻikahi loa ma hope o kēia mau mea. ʻO ka Hemimegalencephaly kekahi o ia ʻano maʻi. ʻOiai paha he paʻakikī iki ka inoa, e kamaʻilio maʻalahi kākou e pili ana.
He aha ka Hemimegalencephaly?
I ka ʻōlelo maʻalahi, ʻo ka hemimegalencephaly ka wā e ʻoi aku ka nui o kekahi ʻaoʻao o ka lolo o kāu keiki (cerebral hemisphere) ma mua o kekahi. E noʻonoʻo i ko kākou lolo e like me kahi puʻupuʻu nui me ʻelua hapalua, kahi ʻaoʻao hema a me kahi ʻaoʻao ʻākau. Ma kēia kūlana, hoʻokahi wale nō ʻaoʻao e ulu nui aʻe ma mua o ka mea e pono ai. I kekahi manawa hiki ke kaupalena ʻia kēia hoʻonui ʻana i kahi ʻāpana liʻiliʻi o kēlā ʻaoʻao o ka lolo , a i ʻole hiki ke hoʻonui ʻia ka ʻaoʻao holoʻokoʻa.
Ke nui aʻe kekahi ʻaoʻao o ka lolo ma kēia ʻano, hiki ke hana ʻia nā loli ʻē aʻe i loko o ka lolo. Eia kekahi laʻana:
- ʻO nā neurons kekahi ʻano o nā cell i loko o ko kākou lolo. ʻAʻole paha lākou i hoʻonohonoho pono ʻia (cytoarchitecture) a i ʻole ʻaʻole paha i hana pono ʻia ka papa luna o ka lolo (cortical dysplasia).
- ʻO ke kino callosum i nalowale a i ʻole i hōʻino ʻia: Ua like kēia me ke alahaka e hoʻopili ana i nā ʻaoʻao ʻelua o ka lolo . Inā nalowale a hōʻino ʻia paha, hiki ke loaʻa nā pilikia.
- Ka hoʻonui ʻia ʻana o nā lua piha i ka wai (ventricles) o ka lolo ma ka ʻaoʻao i hoʻopilikia ʻia.
No ka mea, ʻaʻole i hoʻomohala pono ʻia nā ʻāpana o ka lolo ma kēia ʻano, hiki ke loaʻa pinepine nā hopu ʻana . Kāhea pū mākou i kēia he epilepsy . I ka manawa pinepine, he paʻakikī ke kāohi i kēia mau hopu ʻana me nā lāʻau antiseizure maʻamau. Inā ʻaʻole hiki ke kāohi ʻia nā hopu ʻana me ka lāʻau lapaʻau, pono nā keiki he nui e hana i ke ʻoki lolo (ʻoki epilepsy).
Aia kekahi mau ʻano nui o ka hemimegalencephaly?
ʻAe, aia kekahi mau ʻano nui o kēia ʻano:
- ʻO ka hemimegalencephaly i hoʻokaʻawale ʻia: Hoʻopilikia wale kēia i ka papa waho loa o ka lolo o ke keiki (cerebral cortex). ʻAʻohe pili o ka ʻili a i ʻole nā kikowaena ʻē aʻe.
- ʻO ka hemimegalencephaly syndromic: Hoʻopilikia kēia ʻaʻole wale i ka lolo, akā i ka ʻili o ke keiki a me kekahi mau ʻāpana ʻē aʻe. Eia naʻeHiki ke ʻike ʻia nā hōʻailona ʻili i nā mahina a i ʻole nā makahiki ma hope o ka hānau ʻana. Eia kekahi, ʻoi aku ka nui o kekahi ʻaoʻao o ke kino o ke pēpē ma mua o kekahi (hemihipertrophy).
- ʻO ka hemimegalencephaly holoʻokoʻa: Ma kēia, e hoʻonui ʻia ka cerebellum o ke keiki a i kekahi manawa ʻo ka lolo. Hiki ke hana ʻia kēia ma ke ʻano kaʻawale a i ʻole ka maʻi ʻeha.
He aha nā hōʻailona o kēia maʻi?
ʻO ka hōʻailona mua āu e ʻike ai, ʻo ia ka loaʻa ʻana o kāu pēpē i nā "spasms pēpē a i ʻole nā seizures ". ʻO kēia mau mea maʻamau he mau seizures focal. ʻO ke ʻano kēia, hoʻokahi wale nō ʻaoʻao o ke kino o ka pēpē e ʻoni paha.
ʻO nā hōʻailona ʻē aʻe e ʻike ʻia paha:
- Poʻo i hoʻonui ʻia (Macrocephaly).
- ʻAno like ʻole o ke poʻo.
- Ka paʻakikī me nā neʻe ʻana e like me ka hele wāwae a me ka holo ʻana.
- Ka mae a me ka nāwaliwali ma kekahi ʻaoʻao o ke kino.
- Nā lohi o ka hoʻomohala ʻana: ʻO ia hoʻi ka lohi i ke kamaʻilio ʻana, ka pāʻani ʻana, a me ke aʻo ʻana ma ke ʻano kūpono i ko lākou mau makahiki.
- Nā hemahema o ka ʻike: No ka laʻana, nalowale ka hapalua o ka laulā i ʻike ʻia ke nānā aku me nā maka ʻelua (hemianopia).
He aha ke kumu o ka hemimegalencephaly?
ʻO ka ʻoiaʻiʻo, ʻaʻole maopopo loa i nā kānaka ʻepekema ke kumu o kēia maʻi. Manaʻo kekahi he genetic ia, ʻo ia hoʻi, ua hoʻokumu ʻia e kahi loli i ka DNA o ke keiki. Akā, ʻaʻole ia he hoʻoilina. ʻO ia hoʻi, loaʻa iā ʻoe ke ʻano a ʻaʻole ia he mea āu e hoʻoili ai i kāu keiki.
ʻO ka mea paha, ma kahi o ke kolu o ka pule o ka hāpai ʻana, e hana ʻia kahi loli kūhelu, a i ʻole ka loli koke, i kekahi o nā genes koʻikoʻi no ka ulu ʻana o ka lolo. E hoʻomanaʻo, ʻaʻole i hana ʻia kēia kūlana e kekahi mea āu i hana ai i ka wā hāpai. ʻAʻole kēia kou hewa a i ʻole ka hewa o ka makuakāne.
He aha nā kumu pilikia e hoʻopilikia ai i kēia kūlana?
Hiki ke loaʻa ka hemimegalencephaly me nā kūlana genetic ʻē aʻe. ʻO ke ʻano kēia, ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o ka hemimegalencephaly i nā keiki me kēia mau kūlana:
- ʻO ka maʻi nevus Epidermal
- ʻO ka maʻi ʻo Klippel-Trenaunay
- ʻO ka maʻi ʻo McCune-Albright
- ʻO ke ʻano Neurofibromatosis ʻAno 1 (NF1)
- ʻO ka maʻi CLOVES
- ʻO ka maʻi Proteus
- ʻO ka Hypomelanosis o Ito
Pehea e ʻike ʻia ai ka hemimegalencephaly?
I mea e ʻike pono ai inā ʻo kēia ke ʻano, e nānā mua ke kauka o kāu keiki i kāu keiki a nīnau iā ʻoe e pili ana i ka mōʻaukala olakino o kāu keiki. A laila, hiki iā lākou ke hana i kēia mau hoʻokolohua:
- ʻO kahi scan MRI lolo.
- Hoʻāʻo EEG (Electroencephalogram). Ke ana kēia i ka hana uila o ka lolo.
Me ka ʻenehana holomua o kēia au, hiki ke ʻike ʻia kēia maʻi i kekahi manawa ʻoiai ke pēpē i loko o ka ʻōpū "i ka wā hāpai." Hana ʻia kēia ma ka hana ʻana i kahi scan MRI kūikawā o ka lolo o ke pēpē "fetal MRI".
Pehea e mālama ʻia ai?
ʻO ka pahuhopu nui o ka mālama ʻana i ka hemimegalencephaly ka kaohi ʻana i nā hopu ʻana. Hiki i kēia mau hopu ke hoʻomaka i kekahi manawa i ka wā ʻōpiopio loa a he paʻakikī pinepine ke kaohi me ka lāʻau lapaʻau (kūʻē i ka lāʻau).
Hiki i kēia kūlana ke hoʻoulu i nā ʻano ʻano kūpilikiʻi like ʻole. He mea nui e hoʻomaopopo he hiki i kahi kūpilikiʻi e kū mai ana i ka wā kamaliʻi, ʻoiai inā hoʻomaka ia i ka wā ʻōpiopio, ke loaʻa ka hopena koʻikoʻi i ka ulu ʻana o ke keiki. Hiki i nā kūpilikiʻi i kāohi ʻole ʻia ke hōʻino nui i ka ulu ʻana a me ka ulu ʻana o ke keiki, a me ka hōʻino ʻana i ka lolo e ulu ana. No laila, he mea nui e hoʻōki i ka kūpilikiʻi i ka hikiwawe loa.
Hoʻomaka ka mālama ʻana no nā hopu ʻana me ka lāʻau anti-seizure. Hiki i kekahi mau keiki ke kāohi i ko lākou mau hopu ʻana no ke koena o ko lākou ola me kēia mau lāʻau. Akā nui ka poʻe i loaʻa i ka maʻi epilepsy kūʻē i ka lāʻau. A laila hana ʻia kahi ʻoki kino i kapa ʻia he hemispherectomy. Pili kēia i ka wehe ʻana i ka ʻāpana i hoʻopilikia ʻia o ka lolo o ke keiki (cerebral cortex) a i ʻole ka hoʻokaʻawale ʻana iā ia mai kēlā ʻaoʻao o ka lolo.
Ke hana ʻia kēia ʻoki kino, i kekahi manawa ua kapa ʻia ʻo ia he "functional hemispherectomy." Ma kēia ʻoki kino, ʻoki a hoʻokaʻawale ke kauka ʻoki kino i nā ʻiʻo a me nā aʻalolo e hoʻopili ana i kekahi ʻaoʻao o ka lolo o ke keiki i kekahi, akā hiki ke waiho ʻia ka ʻaoʻao i hoʻonui ʻia o ka lolo i loko o ke poʻo. Ma kahi "anatomical a complete hemispherectomy," hoʻokaʻawale ʻia kekahi ʻaoʻao o ka lolo mai kekahi ʻaoʻao, e wehe loa ana i ka ʻaoʻao ʻē. Pono e hana ʻia kēia ʻoki kino e kahi neurosurgeon i loaʻa ka hoʻomaʻamaʻa kūikawā i ke ʻoki kino epilepsy.
He paʻakikī anei ke ʻoki ʻana e wehe i ka hapalua o ka lolo (Hemispherectomy)?
ʻO ka ʻoiaʻiʻo, he ʻoki lolo paʻakikī loa ka hemispherectomy. Aia kekahi mau kumu e ʻoi aku ai ka paʻakikī o ka hana ʻana i kēia ʻoki ma muli o ke ʻano hemimegalencephaly.
ʻO ka mea mua, ʻo ia ka loaʻa pinepine ʻana o nā kīʻaha koko ma ke ʻano ʻē. No laila, he paʻakikī ke loaʻa a ʻokiʻoki iā lākou i ka wā o ke ʻoki ʻana. I kekahi manawa hiki i kēia ke hoʻokahe nui i ke koko.
Eia kekahi, no ka mea, ʻano ʻē ke ʻano o ka lolo holoʻokoʻa o ke keiki, ʻaʻole ʻike pinepine ʻia nā hōʻailona a ke kauka lapaʻau e hoʻohana ai e ʻike i nā ʻāpana pākahi o ka lolo. Eia kekahi, hiki i nā pēpē a me nā keiki liʻiliʻi ke nalowale i ka nui o ke koko i ka wā o kēia ʻoki kino. Pono e nānā pono ʻia kēia a pono e hoʻololi i ke koko inā pono.
No laila, he mea nui loa ia he hui akamai a ʻike ka hui lapaʻau e mālama nei i kāu keiki i kēia ʻoki kino. ʻOi aku ka palekana no kāu keiki ke hana ʻia kēia ʻoki kino ma kahi haukapila e hana mau ana i ka hemispherectomy no nā pēpē me ka hemimegalencephaly a he nui ka ʻike ma kēia kaʻina hana.
He aha ka wānana no ke keiki me kēia maʻi?
ʻO ke ʻano o ka ulu ʻana o ke kūlana o kāu keiki e hilinaʻi nui ʻia i ka maikaʻi o ke kāohi ʻana i ke kūpono a inā paha aia ka ulu ʻana o ka lolo ma kekahi ʻaoʻao a i ʻole nā ʻaoʻao ʻelua.
He ʻano maʻi like ʻole ka Hemimegalencephaly e hoʻopilikia ai i kēlā me kēia keiki. ʻO ke ʻano kēia, hiki ke ʻokoʻa loa nā hopena mai kekahi keiki a i kekahi.
- Loaʻa paha i nā keiki he nui kekahi pae o ke kīnā noʻonoʻo .
- ʻO kekahi mau keiki i loaʻa i ka ʻiʻo i kaohi maikaʻi ʻia, kokoke paha i ka maʻamau ko lākou akamai.
- Akā no kekahi mau keiki ʻē aʻe, hiki ke hoʻopilikia nui ʻia nā mea e like me ka hele wāwae, ke kamaʻilio ʻana, a me ka ulu ʻana o ka noʻonoʻo.
ʻO ka hapa nui o nā keiki me ka hemimegalencephaly e loaʻa ka nāwaliwali ma kekahi ʻaoʻao o ke kino (hemiparesis) . He ʻano lolo lolo kēia. Loaʻa paha i kekahi mau keiki ka nāwaliwali iki, aʻo kekahi poʻe he nāwaliwali koʻikoʻi. Loaʻa paha i kekahi mau keiki kahi maʻi i kapa ʻia ʻo homonymous hemianopsia, kahi i nalowale ai ka hapalua o ko lākou ʻike maka. Hiki i nā keiki he nui ke hele a kamaʻilio, akā ʻaʻole hiki i nā keiki āpau. Pono kekahi mau keiki i nā mea kōkua hānai kūikawā, aʻo kekahi poʻe hiki ke ʻai maʻamau.
Ua ʻike ʻia kahi noiʻi hou ma hope o ke ʻoki ʻana:
- Ua oki loa ke ʻano hoʻomāinoino ma 68% o nā keiki.
- He 43% o nā keiki he naʻauao awelika a hemahema iki paha.
- Ua hiki i ka 26% o nā keiki ke ʻōlelo ma ke ʻano kūpono i ko lākou mau makahiki.
- Ua hiki i ka 21% o nā keiki ke heluhelu ma kahi pae kūpono.
Hiki ke pale ʻia ka hemimegalencephaly?
ʻOiai ʻaʻole maopopo i nā kānaka ʻepekema ke kumu o kēia maʻi, ʻaʻohe ala e pale aku ai.
Pehea ʻoe e mālama ai i kahi keiki me kēia maʻi?
Inā loaʻa i kāu keiki ke ʻano Hemimegalencephaly, ʻo iaPono kahi hui lapaʻau nui. Me ka hui lapaʻau o kāu keiki, hiki iā ʻoe ke hoʻomohala i kahi hoʻolālā e kūpono i nā pono o kāu keiki. Hiki i ka hui lapaʻau o kāu keiki ke komo pū me:
- Nā Kauka Lapaʻau Keiki
- Nā kauka lolo
- Nā kauka ʻokiʻoki lolo
- Nā loea hoʻomohala
- Nā mea hoʻōla ʻōlelo, nā mea hoʻōla hana, a me nā mea hoʻōla kino
- Nā mea lawelawe mālama ola ʻē aʻe.
He aha nā nīnau āu e nīnau ai i ke kauka o kāu keiki?
Inā ua ʻike ʻia kāu keiki me ka hemimegalencephaly, hiki iā ʻoe ke nīnau i ke kauka i kēia mau nīnau:
- He aha ke ʻano o ka hemimegalencephaly i loaʻa i kaʻu keiki?
- Aia kekahi mau maʻi ʻē aʻe i loaʻa i kaʻu keiki?
- He aha ka lāʻau lapaʻau maikaʻi loa?
- He aha ka manaʻo o kaʻu keiki?
- He aha kā mākou e hana ai e hōʻemi i kāna mau hōʻailona?
E Māmā a me Pāpā, e loaʻa iki ke koa! (Leka Lawe-Home)
Ua ʻike mākou he mea hoʻohoka nui a he mea hoʻopūʻiwa ka ʻike ʻana i kāu keiki e loaʻa ana i ka maʻi ʻōʻili, a i ʻole ke aʻo ʻana ua loaʻa iā lākou kahi maʻi e like me Hemimegalencephaly. Akā e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia pū ka hui lapaʻau e mālama ana i kāu keiki me ʻoe i kēlā me kēia ʻanuʻu o ke ala.
Inā ʻoe e hakakā nei e hoʻoponopono i kēia kūlana, e nīnau i kāu kauka e pili ana i nā hui kākoʻo e kōkua ana i nā ʻohana o nā keiki me ka hemimegalencephaly. ʻO ke kamaʻilio ʻana me nā mākua o nā keiki me kēia maʻi e hiki ke kōkua iā ʻoe e hōʻemi i kou mau makaʻu a hoʻomaopopo i ke kūlana o kāu keiki. Hiki iā lākou ke lilo i kumu ikaika nui nou i ka wā e aʻo ai kāu keiki e noho me ke kūlana.
ʻO ka Hemimegalencephaly, ka ulu ʻana o ka lolo ma ke ʻano ʻē, ka maʻi epilepsy pēpē, ke ʻoki ʻana o ka lolo, nā lohi o ka ulu ʻana, nā kūlana genetic, nā maʻi neurological
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