Ua nalowale koke anei kou lohe a ua manaʻo wale ʻoe ua poniuniu? Ke lohe wale nei ʻoe i kahi walaʻau i loko o kou mau pepeiao? ʻOiai ke manaʻo nei ʻoe he mau mea maʻamau kēia, i kekahi manawa ma hope o kēia mau mea aia paha kahi maʻi i lohe ʻole ʻia e mākou, akā pono mākou e makaʻala. I kēia lā ke kamaʻilio nei mākou e pili ana i ia ʻano maʻi. ʻO kēia maʻi ʻo Neurofibromatosis Type 2, a i ʻole NF2 no ka pōkole.
I ka ʻōlelo maʻalahi, he aha ka Neurofibromatosis Type 2 (NF2)?
He maʻi hoʻoilina ka NF2 e hoʻopilikia ana i kā mākou ʻōnaehana nerve. Hoʻokumu ʻia ia e kahi loli i loko o kahi gene e kāohi ana i ka ulu ʻana o nā cell i loko o ko mākou kino. ʻO kēia loli hoʻoilina ke kumu o ke kino e hana i nā cell he nui loa. Hiki i kēia mau cell keu ke hōʻiliʻili a hana i nā tumors.
Akā, mai hopohopo, ʻo ka hapa nui o kēia mau puʻupuʻu he maikaʻi ʻole , ʻo ia hoʻi, ʻaʻole lākou he maʻi kanesa. Hoʻokumu nui ʻia kēia mau puʻupuʻu i loko o:
- ʻO nā aʻalolo peripheral nā aʻalolo e hoʻolōʻihi i nā ʻāpana o ko mākou kino, e like me nā lālā.
- ʻO ka membrane ma waena o ko kākou iwi poʻo a me ka lolo (meninges).
- Iwi kuamoʻo.
- ʻO nā aʻalolo e hoʻopili ai i ka lolo a me ka pepeiao o loko, nā mea e kōkua ai i ka lohe a me ke kaulike o ke kino.
ʻO NF2 kahi maʻi e pili ana i kahi hui o nā maʻi i kapa ʻia ʻo Neurofibromatosis. Hoʻopilikia nui kēia hui o nā maʻi i ko mākou ʻili a me ka ʻōnaehana nerve.
Pehea ka maʻamau o kēia kūlana NF2 i loko o ke kaiāulu?
He ʻano maʻi laha ʻole kēia. Wahi a nā helu helu, hoʻopilikia ka NF2 i hoʻokahi i loko o 33,000 mau pēpē i hānau ʻia ma ka honua holoʻokoʻa. Ma kahi o 3% o nā maʻi āpau i ʻike ʻia me ka neurofibromatosis he mau maʻi NF2.
He aha nā hōʻailona o NF2?
Hiki ke ʻokoʻa nā hōʻailona o NF2 mai kēlā kanaka a kēia kanaka. Aia lākou i kahi o nā puʻupuʻu i loko o kou kino a me ko lākou nui. ʻIke mua ka hapa nui o ka poʻe i nā hōʻailona i hoʻokumu ʻia e nā puʻupuʻu i loko o nā aʻalolo e hoʻopili ana i ka pepeiao i ka lolo.
E nānā kākou i kēia mau hōʻailona e hoʻomaopopo pono ai iā lākou:
| Māhele ʻano | Nā hōʻailona i ʻike pinepine ʻia |
|---|---|
| Nā hōʻailona mua e pili ana i ke aʻalolo auditory | |
| Nā pilikia kaulike | Pōʻino koke |
| Nā pilikia lohe | Ka nalowale mālie o ka lohe |
| Ke kani nei i nā pepeiao | Ke lohe nei i kahi leo kani i loko o nā pepeiao (Tinnitus) |
| Nā hōʻailona neurological ʻē aʻe | |
| Nā hiʻohiʻona ʻē aʻe |
|
Hoʻomaka pinepine nā hōʻailona e ʻike ʻia ma waena o ka wā kamaliʻi a me 30 mau makahiki. Akā hiki i ke ʻano ke hoʻopilikia i kekahi i kēlā me kēia makahiki. I nā pākeke, ʻo nā aʻalolo e hoʻopili ana i ka pepeiao i ka lolo ka mea mua e hoʻopilikia ʻia. ʻO ia ke kumu e ʻike mua ʻia ai nā pilikia lohe. Eia naʻe, i nā keiki, ulu pinepine nā puʻupuʻu i loko o ka lolo a i ʻole ke kaula kuamoʻo. Inā ʻike ʻia nā hōʻailona i ka wā kamaliʻi a i ʻole ka wā ʻōpio, hiki ke hōʻike ʻia ua ʻoi aku ka koʻikoʻi o ka maʻi.
He aha nā ʻano puʻupuʻu e ulu ana ma NF2?
Nui nā ʻano puʻupuʻu like ʻole e hiki ke ulu i loko o NF2. E nānā koke kākou iā lākou.
| ʻAno Puʻupuʻu | I ka ʻōlelo maʻalahi... |
|---|---|
| ʻO Schwannomas | He mau puʻupuʻu kēia o ka ʻōnaehana nerve e ulu ana mai nā pūnaewele i kapa ʻia ʻo Schwann. ʻO ka hapa nui o lākou e ulu ana i loko o nā aʻalolo e hoʻopili ana i ka lolo i ka pepeiao o loko (vestibular schwannomas). Hiki iā lākou ke ulu i loko o nā aʻalolo e kaohi ana i ka neʻe ʻana o ka maka, ka neʻe ʻana o ke alelo, a me ke ale ʻana. |
| Nā Meningioma | He ʻano puʻupuʻu e hana ʻia ana i loko o ka membrane pale (meninges) ma waena o ka lolo a me ke poʻo. |
| Nā Glioma | He ʻano puʻupuʻu lolo kēia e ulu ana mai nā pūnaewele i kapa ʻia he mau pūnaewele glial. Hoʻokumu pinepine lākou a puni nā aʻalolo optic. |
| ʻO nā Ependymomas | He ʻano glioma nō hoʻi kēia. Hoʻomohala ia i loko o ka lolo a me ke kaula spinal. Kū mai ia mai nā pūnaewele e hana ana i ka wai cerebrospinal (CSF) i loko o ka lolo a me ke kaula spinal. |
No ke aha e ulu ai ʻo NF2? He aha ke kumu?
ʻO ke kumu nui o kēia, he loli ia o ka gene i kapa ʻia ʻo Neurofibromin 2 (NF2) i loko o ko kākou kino. Kōkua kēia gene i ka hana ʻana i kahi protein i kapa ʻia ʻo 'merlin'. ʻO ka hana nui o kēia protein, ʻo ia ke kāohi i ka ulu ʻana o nā puʻupuʻu. ʻO ia hoʻi, he mea hoʻopau puʻupuʻu ia.
No laila, i ka wā e loaʻa ai kahi kīnā i loko o kēia gene NF2, ʻaʻole i hana pono ʻia ka protein 'Merlin'. A laila māhelehele kekahi mau pūnaewele i loko o ko mākou kino me ka ʻole o ke kāohi ʻia, hoʻonui, a hui pū e hana i nā puʻupuʻu.
Hiki ke hoʻoilina ʻia kēia ʻano hoʻoilina mai nā mākua a i nā keiki. Ua kapa ʻia kēia he ʻano autosomal dominant. Akā no nā kānaka he nui, ʻaʻole ia he hoʻoilina. ʻO ke ʻano kēia, hiki ke loaʻa kēia ʻano hoʻoilina i ka wā hāpai, me ka ʻole o ka mōʻaukala ʻohana.
He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?
Inā ʻaʻole i kāohi ʻia ka NF2, hiki ke loaʻa kekahi mau pilikia. ʻO kēia mau mea:
- Ka nalowale piha ʻana o ka lohe
- Ka nalowale o ka ʻike
- Ka pōʻino mau loa o ke aʻalolo
- Ka hōʻiliʻili ʻana o ka wai ma ka lolo (hydrocephalus)
ʻOiai ʻaʻole kēia mau puʻupuʻu he maʻi kanesa, aia kahi manawa liʻiliʻi e lilo ai kekahi mau puʻupuʻu NF2 i maʻi kanesa, he mea kākaʻikahi loa. No laila, he mea nui loa nā nānā lapaʻau maʻamau.
Pehea e ʻike ʻia ai ka NF2?
Ke hele ʻoe i ke kauka, e nānā pono mua ʻo ia iā ʻoe, e nīnau e pili ana i kāu mau hōʻailona a inā paha ua loaʻa i kekahi o kou ʻohana kēia maʻi. A laila, hiki iā ia ke hana i kekahi mau hoʻokolohua e hōʻoia i ka hōʻailona:
- MRI (Magnetic Resonance Imaging) scan: Hiki i kēia ke ʻike maopopo i nā ʻōpū i loko o ka ʻōnaehana nerve a me ka ʻili.
- Hoʻāʻo lohe: E nānā i kou pae lohe.
- Hoʻokolokolo maka: E nānā no ka cataracts a i ʻole nā pilikia maka ʻē aʻe.
- Ka hoʻāʻo ʻana i ka hoʻoilina: No ka hōʻoia ʻana inā he kīnā i loko o ka gene NF2.
He aha nā lāʻau lapaʻau no NF2?
ʻAʻohe lāʻau lapaʻau no kēia. Akā, mai hopohopo, aia i kēia manawa he nui nā ʻano hana holomua e ʻike ai, mālama ai, a hoʻokele i kēia mau puʻupuʻu. Na kāu kauka e koho i ka lāʻau lapaʻau kūpono loa iā ʻoe. Hiki ke ʻokoʻa nā ʻano lapaʻau mai kēlā kanaka a kēia kanaka.
Eia kekahi o nā ʻano lapaʻau nui:
- ʻOki kino: Wehe ʻoki kino i nā puʻupuʻu e hoʻoulu ai i nā hōʻailona. Hoʻohana ʻia ke ʻoki kino e wehe i nā cataracts.
- ʻO Chemotherapy a i ʻole Radiation Therapy: Hoʻohana ʻia kēia mau lāʻau lapaʻau e hōʻemi i ka nui o kekahi mau puʻupuʻu. ʻO ka radiotherapy Stereotactic kekahi o ia ʻano hana radiation therapy holomua.
- Nā mea kōkua lohe a me ka ʻike: Hiki i ka poʻe me ka nalowale o ka lohe ke hoʻohana i nā mea e like me nā 'Hearing aids', 'cochlear implants' a i ʻole 'auditory brainstem implants'. Hiki i nā mea e like me nā maka aniani ke kōkua i nā pilikia ʻike.
- Nā kōkua ʻē aʻe: Inā pilikia ʻoe me ke kaulike a me ka hele wāwae, hiki iā ʻoe ke hoʻohana i nā mea hoʻoneʻe.
- Lāʻau lapaʻau: Kuhikuhi ke kauka i ka lāʻau lapaʻau e kāohi i nā hōʻailona e like me ka ʻeha.
ʻO ka mea nui, i kekahi manawa, inā ʻaʻole nā cysts e hoʻopilikia nui iā ʻoe, e hoʻoholo paha kāu kauka ʻaʻole e mālama iā lākou a nānā mau iā lākou. No laila, he mea nui e hana i kahi hoʻokolokolo kino, MRI scan, a me nā hoʻokolokolo pepeiao a me nā maka ma ka liʻiliʻi i hoʻokahi manawa i ka makahiki.
I ka manawa hea e pono ai iaʻu ke ʻike i ke kauka?
Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o nā hōʻailona ma lalo nei, e ʻike pono i ke kauka.
- Ka loli ʻana o ka ʻike maka.
- Nā loli o ka lohe ʻana a i ʻole ke kani ʻana o nā pepeiao.
- Ka nāwaliwali o nā ʻiʻo a i ʻole ke kulou ʻana o ka maka.
- Nā puʻupuʻu ʻili hou a i ʻole nā kiko.
- ʻO ka ʻeha poʻo mau.
- ʻEha ma nā lālā me ke kumu ʻole.
- Ke loaʻa nei i kahi maʻi (nā hopu).
ʻO ka pinepine, ʻo nā loli i ka lohe a i ʻole ka ʻike ʻana nā hōʻailona mua o kēia maʻi. No laila, inā ʻike ʻoe i kekahi o kēia mau hōʻailona, e ʻimi koke i ke kōkua lapaʻau.
Leka Lawe-Home
- ʻO ka Neurofibromatosis Type 2 (NF2) kahi maʻi genetic e hoʻoulu ai i nā ʻōpū ʻaʻole cancerous e hoʻokumu i loko o ka ʻōnaehana nerve.
- ʻO ka nalowale o ka lohe, ka poniuniu, a me ke kani ʻana o nā pepeiao (tinnitus) nā hōʻailona mua maʻamau.
- ʻOiai ʻaʻohe lāʻau lapaʻau no kēia maʻi, nui nā ʻano hana holomua loa e kaohi ai i nā hōʻailona a mālama i nā ʻōpū.
- Ma hope o ka ʻike ʻia ʻana o ka maʻi, he mea nui loa ia e noho ma lalo o ka mākaʻikaʻi mau ʻana a ke kauka, me nā kiʻi MRI, nā hoʻokolokolo pepeiao a me nā maka, a me nā nānā mau ʻana ma ka liʻiliʻi hoʻokahi i ka makahiki.
- Inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kēia mau hōʻailona, e ʻike koke aku i ke kauka kūpono no ka ʻōlelo aʻo.
💬 Comments (0)
ʻAʻohe manaʻo i kau ʻia. Hoʻohui i kāu manaʻo ma ʻaneʻi no ka manawa mua.
Hoʻohui i kāu manaʻo