He nui rawa tetahi taha o te roro o tō pēpi? Me ako tātou mō te hemimegalencephaly!

Kua āwangawanga koe mō te āhua o te māhunga o tāu tamaiti, mō te maha o ōna mate pāwera? I ētahi wā, tera pea he mate tino onge kei muri i ēnei mea. Ko te Hemimegalencephaly tētahi o aua mate. Ahakoa he ahua uaua te ingoa, me kōrero noa iho.

He aha te Hemimegalencephaly?

Ki te kī ngāwari, ko te hemimegalencephaly he nui ake tetahi taha o te roro o tāu tamaiti (te taha roro nui) i tetahi atu. Whakaarohia tō tātou roro he pukupuku nui e rua ngā haurua, he taha maui, he taha matau. I roto i tēnei āhuatanga, kotahi anake te taha ka nui ake i te mea e tika ana. I ētahi wā ka taea tēnei whakanui te whakawhāiti ki tētahi wāhanga iti o taua taha o te roro , ka taea rānei te whakanui i te taha katoa.

Ina nui haere tetahi taha o te roro i tēnei ara, ka puta pea ētahi atu huringa i roto i te roro. Hei tauira:

• He momo pūtau ngā neuron i roto i tō tātou roro. Tērā pea kāore i te tika te whakaritenga (cytoarchitecture) kāore rānei i te tika te hanganga o te paparanga o runga o te roro (cortical dysplasia).
• Te ngaro, te pakaru rānei o te corpus callosum: He rite tēnei ki tētahi piriti e hono ana i ngā taha e rua o te roro . Mēnā ka ngaro, ka pakaru rānei, ka puta pea ngā raruraru.
• Te whakanui ake i ngā kōhao kī tonu i te wai (ventricles) o te roro i te taha e pāngia ana.

Nā te mea kāore i te tino whanakehia ngā wāhanga o te roro i tēnei ara, ka maha tonu ngā wā ka puta ngā mate hopuhopu . Ka kiia hoki tēnei he mate haurangi . He maha ngā wā, he uaua ki te whakahaere i ēnei mate hopuhopu mā te whakamahi i ngā rongoā ārai-hopu noa. Ki te kore e taea te whakahaere i ngā mate hopuhopu mā te rongoā, he maha ngā tamariki me pokanga roro (pokanga haurangi).

He aha ngā momo matua o te hemimegalencephaly?

Ae, he maha ngā momo matua o tēnei mate:

• Hemimegalencephaly motuhake: Ka pāngia anake te paparanga o waho o te roro o te tamaiti (te cerebral cortex). Kāore he pānga o te kiri, o ētahi atu okana rānei.
• Hemimegalencephaly syndromic: Kāore tēnei e pā ki te roro anake, engari ki te kiri o te tamaiti me ētahi atu okana anō hoki. Heoi anōTērā pea ka puta ngā tohu kiri i muri i te whānautanga i ngā marama, i ngā tau rānei. Waihoki, tērā pea ka nui ake tetahi taha o te tinana o te pēpi i tetahi taha (hemihipertrophy).
• Hemimegalencephaly katoa: I tēnei, ka nui haere te cerebellum o te tamaiti, tae atu ki te take roro i ētahi wā. Ka taea tēnei te puta takitahi, te puta noa rānei i te mate.

He aha ngā tohu o tēnei mate?

Ko te tohu tuatahi ka kitea pea e koe ko te "ngā spasms o tō pēpi" me te " ngā haurangi o te pēpi". He maha ngā wā ka kitea ēnei he haurangi ā-whānui. Ko te tikanga ka nekeneke tetahi taha anake o te tinana o te pēpi.

Ko ētahi atu tohu ka kitea pea ko:

• Te upoko nui (Macrocephaly).
• Te āhua o te upoko kāore i te ōrite.
• Te uaua ki ngā nekehanga pēnei i te hikoi me te oma.
• Te ngoikore me te ngenge i tetahi taha o te tinana.
• Ngā whakaroa whanaketanga: Ko te tikanga tēnei ko te tomuri ki te kōrero, ki te tākaro, ki te ako hoki i te huarahi e tika ana mō tō rātou tau.
• Ngā ngoikoretanga tirohanga: Hei tauira, ka ngaro te haurua o te whānuitanga tirohanga ina titiro ki ngā kanohi e rua (hemianopia).

He aha ngā take o te hemimegalencephaly?

Ina hoki, kāore anō kia tino mōhiotia e ngā kaipūtaiao te take o tēnei mate. E whakapono ana ētahi he ira tūturu, arā, nā te panonitanga o te DNA o te tamaiti i puta ai. Engari ehara i te mea tuku iho. Arā, kei a koe te mate, ehara i te mea ka tukuna atu ki tāu tamaiti.

Ko te mea pea, i te toru o ngā wiki o te hapūtanga, ka puta he panoni matapōkere, he panoni ā-tūpono rānei, i roto i tētahi o ngā ira e nui ana mō te whanaketanga o te roro. Kia maumahara, ehara tēnei āhuatanga i te mea nāu i mahi i te wā e hapū ana. Ehara tēnei i te he nāu, ehara hoki i te he o te pāpā.

He aha ngā āhuatanga mōrearea e pā ana ki tēnei āhuatanga?

Ka pā te hemimegalencephaly me ētahi atu mate ira. Ko te tikanga o tēnei, ka nui ake te mōrearea o ngā tamariki e whai ana i ēnei mate ki te pāngia e te hemimegalencephaly:

• Te mate pukupuku kiriuhi
• Te mate Klippel-Trenaunay
• Te mate McCune-Albright
• ``Neurofibromatosis Momo 1 (NF1)''
• 'Te mate CLOVES'
• 'Te mate Proteus'
• Te Hypomelanosis o Ito

Me pēhea te tautuhi i te hemimegalencephaly?

Hei whakatau mēnā koinei te āhua, ka tirohia tuatahitia e te tākuta o tāu tamaiti tāu tamaiti, ā, ka pātai atu ki a koe mō te hītori hauora o tāu tamaiti. Kātahi ka mahia pea e rātou ēnei whakamātautau e whai ake nei:

• He matawai MRI roro.
• Whakamātautau EEG (Electroencephalogram). Ka ine tēnei i te ngohe hiko o te roro.

Mā te hangarau matatau o ēnei rā, ka taea te tautuhi i tēnei mate i ētahi wā i roto tonu i te kōpū o te pēpi "i te wā e hapu ana." Ka mahia tēnei mā te whakahaere i tētahi matawai MRI motuhake o te roro o te pēpi "MRI o te pēpi".

Me pēhea te rongoā?

Ko te whāinga matua o te rongoā i te hemimegalencephaly ko te whakahaere i ngā mate hopuhopu. Ka tīmata pea ēnei mate hopuhopu i ētahi wā i te tamarikitanga, ā, he uaua ki te whakahaere mā te rongoā (ātete ki te rongoā).

Ka taea e tēnei āhuatanga te whakaputa i ngā momo mate pāwera. He mea nui kia mōhio ko te mate pāwera e puta ana i te tamarikitanga, inā koa ka tīmata i te pēpi, ka pā nui ki te whanaketanga o te tamaiti. Ka taea e ngā mate pāwera kore e whakahaeretia te kino kino ki te tipu me te whanaketanga o te tamaiti, me te kino hoki ki te roro e whanake ana. Nō reira, he mea nui kia mutu te mate pāwera i te wā e taea ai.

Ka tīmata te rongoā mō ngā mate hopuhopu mā te rongoā ārai-hopu. Ka taea e ētahi tamariki te whakahaere i ō rātou mate hopuhopu mō te toenga o ō rātou oranga mā ēnei rongoā. Engari he tokomaha ka pāngia e te mate haurangi ātete-rongoa. Kātahi ka mahia he pokanga e kiia nei ko te hemispherectomy. Ko te tikanga o tēnei ko te tango i te wāhanga e pāngia ana o te roro o te tamaiti (cerebral cortex) me te wehe rānei i tērā taha o te roro.

Ina mahia tēnei pokanga, ka kiia i ētahi wā ko te "hemispherectomy mahi." I roto i tēnei pokanga, ka tapahia, ka wehea hoki e te taote pokanga te kiko me ngā io e hono ana i tētahi taha o te roro o te tamaiti ki tētahi taha, engari ka taea te waiho i te taha nui rawa o te roro ki roto i te angaanga. I roto i te "hemispherectomy tinana, katoa rānei," ka wehea tētahi taha o te roro i tētahi taha, ka tangohia katoatia te taha rerekē. Me mahi tēnei pokanga e te taote pokanga roro he tohunga motuhake ki te pokanga mate haurangi.

He uaua te pokanga hei tango i te haurua o te roro (hemispherectomy)?

Ina hoki, he tino uaua te pokanga roro i te hemispherectomy. He maha ngā take he uaua ake ai te mahi i tēnei pokanga nā te mate hemimegalencephaly.

Ko te mea tuatahi, he rerekē te āhua o ngā oko toto. Nō reira, he uaua ki te kimi me te tapahi i te wā e pokanga ana. I ētahi wā ka nui rawa te toto ka puta.

Hei tāpiritanga, nā te mea he rerekē te āhua o te roro katoa o te tamaiti, he maha ngā wā kāore e kitea ngā tohu e whakamahia ana e te taote pokanga hei tautuhi i ngā wāhanga takitahi o te roro. Waihoki, ka ngaro pea te toto nui i ngā pēpi me ngā tamariki nohinohi i te wā o tēnei pokanga. Me tino aroturukitia tēnei, ā, me whakakapi te toto ina hiahiatia.

Nō reira, he mea tino nui kia whai pūkenga, kia mātau hoki te tīma hauora e rongoā ana i tāu tamaiti ki tēnei mahi pokanga. He haumaru ake mā tāu tamaiti te mahi i tēnei pokanga i roto i tētahi hōhipera e mahi tonu ana i te hemispherectomy mō ngā pēpi he hemimegalencephaly, ā, he nui hoki ō rātou wheako ki tēnei tikanga.

He aha te āhua o te tamaiti e pāngia ana e tēnei mate?

Ko te whanaketanga o te mate o tāu tamaiti e whakawhirinaki ana ki te pai o te whakahaere i te taunga o te roro, me te mea kei tetahi taha, kei ngā taha e rua rānei te whanaketanga rerekē o te roro.

He mate whānui te hemimegalencephaly e pā ana ki ia tamaiti, ki ia tamaiti. Ko te tikanga he rerekē rawa ngā hua mai i tētahi tamaiti ki tētahi tamaiti.

• He maha ngā tamariki ka pāngia e te hauātanga hinengaro i tētahi taumata.
• Ko ētahi o ngā tamariki he pai te whakahaere i ō rātou haurangi, tera pea he tata noa te mātauranga o rātou.
• Engari mō ētahi atu tamariki, ka tino pāngia pea ngā mea pēnei i te hīkoi, te kōrero, me te whanaketanga hinengaro.

Tata ki te katoa o ngā tamariki e pāngia ana e te hemimegalencephaly ka pāngia e te ngoikore i te taha kotahi o te tinana (hemiparesis) . He momo pararutiki roro tēnei. Tērā pea he ngoikore ngawari tō ētahi tamariki, ko ētahi he ngoikore nui. Tērā pea he mate tō ētahi tamariki e kiia nei ko te hemianopsia homonymous, arā, ka ngaro te haurua o tō rātou tirohanga. He maha ngā tamariki ka taea te hikoi me te kōrero, engari kāore e taea e ngā tamariki katoa. Me hiahia ētahi tamariki ki ngā āwhina whāngai motuhake, ko ētahi ka taea te kai noa.

I kitea i roto i tētahi rangahau tata nei i muri i te pokanga:

• Kua mutu katoa te mate urutā mō te 68% o ngā tamariki.
• 43% o ngā tamariki he toharite, he paku ngoikore rānei te hinengaro.
• E 26% o ngā tamariki kua taea te kōrero i te reo e tika ana mō ō rātou tau.
• E 21% o ngā tamariki kua taea te pānui i te taumata e ranea ana.

Ka taea te ārai i te hemimegalencephaly?

I te mea kāore anō ngā kaipūtaiao kia tino mōhio he aha te pūtake o tēnei mate, kāore he huarahi hei ārai.

Me pēhea te tiaki i tētahi tamaiti me tēnei mate?

Mena kei tō tamaiti te mate Hemimegalencephaly,E hiahiatia ana he tīma hauora nui. Ka taea e koe te whakawhanake i tētahi mahere e tutuki ai ngā hiahia o tāu tamaiti me te tīma hauora o tāu tamaiti. Tērā pea kei roto i te tīma hauora o tāu tamaiti:

• Ngā Tākuta Tamariki
• Ngā tohunga roro
• Ngā taote pokanga roro
• Ngā tohunga whanaketanga
• Ngā kaiwhakaora kōrero, ngā kaiwhakaora mahi, me ngā kaiwhakaora tinana
• Ētahi atu kaiwhakarato hauora.

He aha ngā pātai me pātai atu koe ki te tākuta o tāu tamaiti?

Mena kua tāpaetia tō tamaiti he hemimegalencephaly, ka taea e koe te pātai atu ki te tākuta i ēnei pātai:

• He aha te momo hemimegalencephaly kei taku tamaiti?
• He mate hauora anō kei taku tamaiti?
• He aha te maimoatanga pai rawa atu?
• He aha te tirohanga a taku tamaiti?
• He aha tā tātou e taea ai te whakaiti i ōna tohumate?

Māmā rāua ko Pāpā, kia maia! (Karere hei tango ki te kāinga)

E mōhio ana mātou he tino ohorere, he tino mamae hoki te kitenga i tāu tamaiti e pāngia ana e te mate haurangi, te rongonga rānei kei a ia tētahi mate pēnei i te Hemimegalencephaly. Engari kia maumahara, ehara i te mea ko koe anake. Kei a koe te tīma hauora e rongoā ana i tāu tamaiti i ia taahiraa o te ara.

Mena kei te raru koe ki te whakatau i tēnei āhuatanga, pātai atu ki tō tākuta mō ngā rōpū tautoko e āwhina ana i ngā whānau o ngā tamariki e pāngia ana e te hemimegalencephaly. Mā te kōrero ki ngā mātua o ngā tamariki e pāngia ana e tēnei mate ka āwhina i a koe ki te whakaiti i ō mataku me te whakaae ki te mate o tāu tamaiti. He puna nui rātou hei whakapakari i a koe i a tāu tamaiti e ako ana ki te noho me te mate.

` Hemimegalencephaly, te whanaketanga roro rerekē, te mate haurangi pēpi, te pokanga roro, te whakaroa whanaketanga, ngā mate ira, ngā mate pūnaha io