I kēia lā e kamaʻilio mākou e pili ana i ka maʻi maikaʻi loa, ka mea e hoʻopilikia ai i ka ʻike maka!

Ua hoʻomaka anei ʻoe a i ʻole kāu keiki e ʻike i kahi loli iki i ke ʻano o kou ʻike pololei ʻana i nā mea i mua, he pohihihi o ka ʻike? A i ʻole ua haʻi aku kekahi kauka maka iā ʻoe a i ʻole kekahi o kou ʻohana e pili ana i kēia inoa ʻo 'Best disease'? He ʻano laha ʻole kēia, akā he ʻano genetic hiki ke hoʻopilikia i nā maka ʻelua. No laila i kēia lā, e kamaʻilio kākou ma nā ʻōlelo maʻalahi e pili ana i ke ʻano o kēia maʻi Best, pehea e ulu ai, he aha nā hōʻailona, ​​a inā he lāʻau lapaʻau.

He aha kēia maʻi Maikaʻi Loa?

I ka ʻōlelo maʻalahi, ʻo ka maʻi ʻo Best kahi maʻi genetic e hoʻopilikia ana i ka retina o kou mau maka. Ua like ko mākou mau maka me ke kāmela. ʻO ka retina ka papa ma ke kua o ka maka e kōkua iā mākou e ʻike i nā kiʻi ke pā mai ka mālamalama. ʻO ka ʻāpana o ka retina ma waenakonu o ka retina e kōkua iā mākou e ʻike pololei i nā mea i mua ua kapa ʻia ʻo ka macula. No laila, i ka maʻi ʻo Best, nāwaliwali mālie ka macula. Hiki i kēia ke paʻakikī ke ʻike pololei i nā mea i mua, e like me nā leka a me nā helehelena o ka poʻe. Akā i ka hapa nui o ka manawa , ʻaʻole i hoʻopilikia nui ʻia ka ʻike peripheral, a i ʻole ka ʻike ʻana i nā mea a puni ʻoe.

Ua kapa ʻia kēia maʻi ʻoi loa e nā inoa ʻē aʻe. I kekahi manawa hiki i nā kauka ke kapa iā ia he 'vitelliform macular dystrophy' a i ʻole 'vitelliform dystrophy'. ʻO 'Dystrophy' kahi huaʻōlelo lapaʻau no ka hōʻino ʻana a i ʻole ka nāwaliwali o kekahi ʻano.

Aia kekahi maʻi ʻē aʻe e like me kēia, ʻo ia hoʻi kekahi ʻano o ka 'vitelliform macular dystrophy'. Eia naʻe, ʻaʻole ia e hoʻomaka i ka wā kamaliʻi, akā e ulu aʻe i kou wā ʻelemakule, ma waena o 40 a me 60 mau makahiki. Ua kapa ʻia ʻo ia ʻo ke ʻano 'adult-onset type'.

Pehea ka maʻamau o ka maʻi Best?

He mea paʻakikī ke loaʻa nā helu kikoʻī e pili ana i ka nui o ka poʻe i loaʻa i ka maʻi ʻo Best. ʻAʻole ʻike kekahi poʻe ua loaʻa iā lākou ia mea no ka mea ʻaʻohe o lākou mau hōʻailona. Wahi a kekahi noiʻi ma kahi o hoʻokahi i loko o 16,500 poʻe, a i ʻole ma kahi o hoʻokahi i loko o 21,000, paha i loaʻa iā ia. Wahi a kekahi noiʻi ʻē aʻe ma kahi o hoʻokahi i loko o 15,000 . Wahi a kekahi, he hoʻokahi paha i loko o 10,000 . Eia naʻe, hiki iā mākou ke hoʻomaopopo he maʻi laha ʻole kēia.

He aha nā pae o ka maʻi Best?

Hiki i kēia maʻi maikaʻi loa ke hele ma o ʻeono mau pae nui. E nānā kākou i ke ʻano o lākou.

1. Kahua I - Kahua Previtelliform:

ʻO kēia ka hoʻomaka loa. ʻO ka hapa nui o ka manawa, ʻaʻole ʻoe e loaʻa i kekahi mau hōʻailona i kēia manawa.ʻAʻole i hoʻomaka kahi mea melemele e hana ma lalo o kou retina. Hiki ke ʻike ʻia kēia ma ka hanana i ka wā o ka hoʻokolokolo maka.

2. Kahua II - Kahua Vitelliform:

ʻO ke ʻano o ka huaʻōlelo `vitelliform` he ``ʻano hua manu.`` I kēia pae, hoʻomaka kahi mea melemele e hōʻiliʻili ma lalo o kou retina, ma kahi o ka ``macula``, ke kikowaena o kou ʻike. E noʻonoʻo iā ia me he hua manu melemele liʻiliʻi lā. Maikaʻi nō paha kou ʻike i kēia pae.

3. Pae III - Pae Pseudohypopyon:

I kēia pae, hiki i ka mea melemele ke lilo i wai a hana i cyst ma lalo o ka retina. Ua like kēia me kahi pehu liʻiliʻi i piha i ka wai i loko o ka maka.

4. Kahua IV - Kahua Vitelliruptive:

ʻO kēia kahi e hoʻomaka ai ka mea melemele i hōʻiliʻili ʻia e wāwahi a hoʻopuehu. I ka wā e haki ai kēia mea, hiki ke hōʻino ʻia nā cell i loko o ka retina. I kēia manawa, e hoʻomaka paha ʻoe e ʻike i nā loli i kou ʻike, e like me ka ʻike pōuliuli.

5. Kahua V - Kahua Atrophic:

I kēia pae, ua aneane nalowale loa ka mea melemele. Akā, aia nā ʻeha a me nā hunaola i hōʻino ʻia ma kona wahi i noho ai. Hiki i kēia ʻino ke hoʻopilikia hou aku i ka ʻike. Manaʻo kekahi poʻe noiʻi ʻo ia ke pae hope loa o ka maʻi ʻo Best.

6. Pae VI - Choroidal neovascularization (CNV):

Manaʻo kekahi poʻe noiʻi ʻo kēia kūlana i kapa ʻia ʻo `CNV` ʻo ia ke kahua hope loa o ka maʻi Best. Manaʻo kekahi he pilikia ia o ka maʻi. Ma kahi o 20% o ka poʻe me ka maʻi Best e loaʻa paha kēia kūlana. ʻO `Neovascularization` ka hoʻokumu ʻia ʻana o nā kīʻaha koko hou. ʻO `Choroid` ka membrane ma waena o ka `retina` a me ka `sclera` o ka maka. Ma kēia kūlana `CNV`, hoʻokumu ʻia nā kīʻaha koko hou a nāwaliwali i loko o ka papa `choroid`. He nāwaliwali loa kēia mau kīʻaha koko hou a hiki iā lākou ke hoʻokahe i ke koko a i ʻole ka wai. Inā hana ʻia kēia, hiki i kou ʻike ke ʻino aʻe.

He aha nā hōʻailona o ka maʻi Best?

ʻO ka hapa nui o ka manawa, e ʻike wale ʻoe e pili ana i ka maʻi ʻo Best ma hope o ka hoʻokolokolo maka, no ka mea, i kekahi manawa ʻaʻohe hōʻailona ʻike ʻia. Akā inā ʻike ʻia nā hōʻailona, ​​​​hiki iā lākou ke komo pū me:

• ʻIke maka pōwehiwehi: ʻO nā mea i ʻike pololei ʻia i mua e ʻike ʻia he maopopo ʻole a palalulu paha.
• Nā pilikia me ka ʻike pololei: ʻO ka pilikia ka ʻike ʻana i nā mea i mua ou. Akā, loaʻa paha iā ʻoe ka ʻike maikaʻi a puni, a i ʻole ka ʻike ʻaoʻao.
• ʻIke i nā mea e loli ana ke ʻano (Metamorphopsia):E noʻonoʻo, ke nānā nei ʻoe i kahi laina pololei, akā ʻike ʻia ia iā ʻoe he laina i kaha ʻia a piʻi. E ʻike paha ʻoe i nā mea e like me nā ʻūpā puka a me nā mōlina puka makani i kaha ʻia. Ua kapa ʻia kēia he 'metamorphopsia'.
• Poho nui o ka ʻike: Hiki i kekahi poʻe ke loaʻa ka pohō nui o ka ʻike i ka hala ʻana o ka manawa.
• ʻOkoʻa ka ʻike ma waena o nā maka ʻelua: ʻOi aku ka liʻiliʻi o ka ʻike ma kekahi maka ma mua o kekahi. A i ʻole, ʻaʻole paha e emi like ka ʻike ma nā maka ʻelua, akā, e emi paha i nā kekelē like ʻole.

He aha ke kumu o ka maʻi Best?

Hoʻokumu ʻia ka maʻi ʻo Best e kahi maʻi hoʻoilina. I ka ʻōlelo maʻalahi, hoʻokumu ʻia ia e kahi loli i nā genes a mākou e hoʻoilina ai mai ko mākou mau mākua.

He maʻi "autosomal dominant" ka maʻi ʻo Best. He ʻano lapaʻau kēia, akā maʻalahi ke hoʻomaopopo. ʻO ke ʻano o ka "autosomal dominant" ʻaʻole pono ke keiki e hoʻoilina i ka gene i hoʻololi ʻia mai nā mākua ʻelua, hoʻokahi wale nō. ʻO ia hoʻi, inā loaʻa i kekahi o nā mākua ka gene i hoʻololi ʻia, loaʻa i kā lākou mau keiki ma kahi o 50% ka manawa e loaʻa ai ka maʻi. Ua like ia me ka hoʻolei ʻana i kahi ʻāpana kālā a loaʻa nā poʻo.

ʻO ka mea kupanaha, ʻo ka gene like e hoʻoulu ai i ka maʻi ʻo Best ke kumu pū kekahi i kekahi ʻano o ka maʻi maka hoʻoilina ʻē aʻe i kapa ʻia ʻo retinitis pigmentosa. ʻO ke ʻano kēia, hiki i nā ʻokoʻa o kēia gene ke hoʻoulu i nā ʻano maʻi maka like ʻole.

Pehea e ʻike ʻia ai ka maʻi Best?

ʻO ka hapa nui o ka manawa, e ʻike ke kauka i ka maʻi ʻo Best ke ʻoe ma waena o ʻelima a me ʻumi makahiki, a i ʻole ma ka nui loa ke 20 ou makahiki.

Ke kipa aku ʻoe i kahi loea mālama maka no kahi pilikia maka, e nīnau mua lākou iā ʻoe e pili ana i kou mōʻaukala olakino, e nīnau inā he pilikia maka ko kekahi o kou ʻohana, a laila e hana i kahi hoʻokolokolo maka. Eia kekahi, hiki iā lākou ke hana i kekahi mau hoʻokolohua kūikawā. Eia kekahi laʻana:

• ʻO ka angiography Fluorescein: He hoʻāʻo kiʻi kēia. Hoʻokomo ʻia kahi kala kūikawā i loko o kahi aa koko i kou lima a lawe ʻia nā kiʻi o nā kīʻaha koko i loko o kou maka. Hiki i kēia ke kōkua i ka ʻike inā he mau leaka a i ʻole nā ​​​​​​mea ʻē i loko o nā kīʻaha koko.
• ʻO ka tomography coherence optical (OCT): He hoʻāʻo kiʻi ʻaʻole invasive kēia. Hoʻohana ia i nā kukuna kukui e lawe i nā kiʻi ʻokiʻoki o ke kua o kou maka, ʻo ia hoʻi ka retina a me ka macula. Ua like ia me ka ʻoki ʻana i kahi keke a nānā i loko. Hiki iā ia ke ʻike i nā mea e like me ka mānoanoa o nā papa o ka retina, kekahi pehu, a me ka hōʻiliʻili ʻana o ka wai.
• Kiʻi paʻi kala o ka fundus: Lawe kēia i nā kiʻi maopopo o kou retina, kona mau kīʻaha koko e pili ana, a me ke poʻo o ke aʻalolo optic. Hiki i kēia ke kōkua iā ʻoe e ʻike inā he mau waihona melemele e like me ka hua manu.
• ʻO ka electrophysiology ophthalmic: He ʻano hoʻokolohua kēia. Ana kēia mau hoʻokolohua i ka hana uila maalea e hana ʻia ke pane ko mākou mau maka i ka mālamalama. Hiki i kēia ke hāʻawi i kahi manaʻo o ka hana maikaʻi o nā cell i loko o ka retina.
• Nā hoʻokolohua hoʻoilina: Hiki i kēia mau hoʻokolohua ke hōʻoia me ka maopopo inā he mau loli i kāu mau genes e pili ana i ka maʻi Best.

I kekahi manawa makemake paha ke kauka e kamaʻilio me nā lālā ʻē aʻe o kou ʻohana a nānā paha i ko lākou mau maka, no ka mea, he maʻi hoʻoilina kēia.

Pehea e mālama ʻia ai ka maʻi Best?

A hiki i kēia lā, ʻaʻohe lāʻau lapaʻau no ka maʻi ʻo Best. ʻO ia ka mea mua e pono ai kākou e hoʻomaopopo.

ʻOi aku ka maikaʻi o ka mālama ʻana i ka maʻi ma muli o kāu mau hōʻailona a me ke kahua o ka maʻi. Ke ʻike ʻia, he mea nui e hana ʻoe i nā hoʻokolohua maka maʻamau. A laila hiki iā ʻoe ke ʻike koke i nā loli i kou mau maka a hana i nā hana e pono ai.

I nā pae mua, ʻaʻole paha ʻoe e pono i kekahi lāʻau lapaʻau. Eia nō naʻe, inā he mau hewa refractive ʻē aʻe kāu, e like me ka ʻike maka mamao, pono paha ʻoe e hoʻohana i nā aniani. Loaʻa pinepine i ka poʻe me ka maʻi ʻo Best nā pilikia me ka ʻike mamao. Eia kekahi, inā loaʻa iā ʻoe nā cataracts, pono paha ʻoe e hana i ke ʻoki cataract.

Akā inā loaʻa iā ʻoe ke kūlana a mākou i kamaʻilio ai ma mua i kapa ʻia ʻo choroidal neovascularization (CNV), ʻo ia hoʻi ke kūkulu ʻia ʻana o nā kīʻaha koko nāwaliwali hou, hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me kēia e hoʻōki i ka ulu ʻana o kēlā mau kīʻaha koko hou:

• Nā mea hoʻohuihui ulu endothelial anti-vascular (Anti-VEGF): He mau lāʻau lapaʻau kēia. Hoʻokomo ʻia kēia mau lāʻau lapaʻau i loko o ka lua vitreous i loko o kou maka. ʻO ka pahuhopu ka hoʻōki ʻana i ka ulu ʻana o kēlā mau kīʻaha koko hou a leaka a hoʻemi iā lākou.
• Lapaʻau Laser: Hoʻohana kēia lāʻau i nā kukuna laser e sila ai a luku paha i nā kīʻaha koko maikaʻi ʻole.
• Lapaʻau Photodynamic (PDT): He lāʻau paʻakikī iki kēia. Maanei, hoʻokomo ʻia kahi lāʻau kūikawā i loko o kou kino, a laila hoʻolilo ʻia ka lāʻau i mea ʻike i ka mālamalama, a kuhikuhi ʻia kahi kukuna laser i nā kīʻaha koko i hoʻopilikia ʻia, e hōʻino ana iā lākou a pale iā lākou mai ka hoʻokahe ʻana o ke koko.

Eia kekahi, hiki i kāu kauka ke kuhikuhi iā ʻoe e hoʻohana i nā mea kōkua ʻike haʻahaʻa, e like me nā mea hoʻonui, nā kukui kūikawā, a me nā polokalamu maʻalahi e heluhelu.

Ke noiʻi nei nā kānaka noiʻi inā hiki ke hoʻohana ʻia nā mea hoʻoilina e mālama ai a pale aku paha i ka maʻi. Aia nō ka hoʻōla ʻana o ka gene i ke kahua noiʻi, akā ke manaʻolana ʻia nei e loaʻa nā hopena holomua i ka wā e hiki mai ana.

Hiki ke hoʻemi ʻia ka pilikia o ka loaʻa ʻana o ka maʻi Best?

ʻOiaʻiʻo, ʻaʻohe mea hiki iā ʻoe ke hana e pale aku i ka maʻi ʻo Best. He maʻi hoʻoilina ia. Akā inā loaʻa i kekahi o kou ʻohana ka maʻi ʻo Best, a inā ʻike ʻoe ua loaʻa iā ʻoe, he mea nui paha ke loaʻa ka ʻōlelo aʻoaʻo hoʻoilina ma mua o kou loaʻa ʻana o nā keiki. Hiki i ke kākāʻōlelo hoʻoilina ke nānā i kou mōʻaukala ʻohana a wehewehe i ka pilikia o ka hoʻoilina ʻana o kāu mau keiki i ka maʻi.

He aha ka mea e hana inā loaʻa iaʻu ka maʻi Best?

ʻAʻole he maʻi make ka maʻi o Best. ʻO ia hoʻi, ʻaʻole ia he mea hoʻoweliweli i ke ola. Akā, e like me ka mea i ʻōlelo ʻia ma mua, ʻaʻole hiki ke ho'ōla ʻia. ʻAʻole ʻoe e makapō loa mai ka maʻi o Best. Akā, he ʻike haʻahaʻa paha kou. ʻO ia hoʻi, he mea paʻakikī iki paha ke hana i nā hana o kēlā me kēia lā, akā, ʻaʻole ʻoe e nalowale loa kou ʻike.

Pehea wau e mālama ai iaʻu iho?

Aia kekahi mau meaʻai maikaʻi no ke olakino o nā maka.

"He mea nui loa ka ʻai maikaʻi no nā maka."

Eia kekahi laʻana, ʻo ka ʻai ʻana i ka iʻa i kekahi mau lā o ka pule, a me ka hoʻohui ʻana i nā mea kanu ʻōmaʻomaʻo, nā huaʻai, a me nā nati i kāu papaʻai he mea maikaʻi ia no kou mau maka. Hāʻawi kēia i nā meaʻai e pono ai ka maka e like me nā omega-3 fatty acids a me nā wikamina.

Inā loaʻa iā ʻoe ka maʻi ʻo Best, he mea nui e hana i nā nānā lapaʻau maʻamau, ʻoiai nā hoʻokolohua maka. Inā ʻike ʻoe i kekahi mau loli i kou ʻike a i ʻole ke olakino laulā, e haʻi koke i kāu kauka. Hiki iā ʻoe ke nīnau i kāu kauka e like me:

• " Hiki iā ʻoe ke paipai i kahi kākā'ōlelo genetic e kōkua iaʻu e hoʻoholo inā he mau keiki kā mākou?"
• "Ua kūpono anei au e komo i kahi hoʻokolohua lapaʻau no kēia maʻi?"
• " Hiki iā ʻoe ke kuhikuhi mai i kahi kauka maka i loea i ka ʻike haʻahaʻa?"
• " He koho lapaʻau anei ka gene therapy noʻu?"

He aha ka ʻokoʻa ma waena o ka maʻi Best a me ka maʻi Stargardt?

ʻO ka maʻi Stargardt a me ka maʻi Best he mau maʻi hoʻoilina, a hoʻopilikia lāua ʻelua i ke kikowaena o ka ʻike ma ka maka (ka macula). ʻO ke ʻano kēia, ua hoʻopilikia ʻia ka ʻike pololei.

Akā, ua hoʻokumu ʻia kēia mau maʻi ʻelua e nā loli i nā ʻano genes like ʻole.

I ka maʻi Stargardt, hiki iā ʻoe ke ʻike i nā kiko melemele a keʻokeʻo paha ma kou retina. Akā i ka maʻi Best, hiki iā ʻoe ke ʻike i kahi cyst nui, melemele, e like me ka hua manu, e like me ka yolk, ma lalo o ka macula. No laila, hiki ke hoʻokaʻawale ʻia kēia mau maʻi ʻelua e kēia mau hōʻailona a me ka hoʻāʻo ʻana i ka genetic.

Hiki ke paʻakikī ke noho me kahi maʻi hoʻoweliweli i ka ʻike. Akā inā loaʻa iā ʻoe ka maʻi ʻo Best, nui nā kumuwaiwai a me nā lawelawe e hiki ke kōkua i ka maʻalahi o nā mea. E nīnau i kāu kauka e pili ana iā lākou.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻAe, ua kamaʻilio nui mākou e pili ana i ka maʻi maikaʻi loa. E hōʻuluʻulu kākou:

• He maʻi hoʻoilina ka maʻi ʻo Best e hoʻopilikia ana i ke kikowaena o ka ʻike ma ka maka (ka macula). Hiki i kēia ke alakaʻi i ka ʻike ʻino.
• ʻOiai ʻaʻole hiki ke ho'ōla piha ʻia, ʻaʻole ia he mea e hoʻoweliweli ai i ke ola. Hiki ke ʻike haʻahaʻa ʻia ka ʻike maka, akā, ʻaʻole hiki ke makapō loa.
• He mea nui loa ka ʻike mua ʻana i ka maʻi a hana i nā hoʻokolohua lapaʻau maʻamau.
• Aia nā lāʻau lapaʻau no nā pilikia e like me ka 'CNV'.
• He mea nui ka ʻōlelo aʻoaʻo genetic no ka poʻe e hoʻolālā ana i kahi ʻohana.
• ʻO ka ʻai ʻana i nā meaʻai maikaʻi no nā maka a me ka hoʻohana ʻana i nā mea kōkua ʻike haʻahaʻa hiki ke maʻalahi i ke ola.

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia nā kauka, nā kākāʻōlelo, a me nā ʻano hana e hiki ke kōkua iā ʻoe e ola me kēia mau kūlana. ʻO ka mea nui loa, ʻo ia ke noho ikaika a hahai i nā ʻōlelo kuhikuhi e pono ai. Inā he mau nīnau a kānalua paha kāu, e kamaʻilio ākea me kāu kauka.

ʻO ka maʻi maikaʻi loa, ʻO ka maʻi maikaʻi loa, nā maʻi maka, ke kikowaena ʻike maka, macula, nā maʻi genetic, nalowale o ka ʻike maka, nā hoʻokolohua maka