I tēnei rā ka kōrero tātou mō te mate pai rawa atu, e pā kino ana ki te tirohanga!

Kua tīmata koe, tāu tamaiti rānei ki te kite i tētahi panoni iti o tō tirohanga tika ki ngā mea, arā, he pōuri te tirohanga? Kua kōrerotia pea tēnei ingoa e tētahi tākuta kanohi ki a koe, ki tētahi rānei o tō whānau, e tētahi o ngā tākuta kanohi? He mate onge tēnei, engari he mate ira, ka pā ki ngā kanohi e rua. Nō reira, i tēnei rā, me kōrero māmā noa iho tātou mō te āhua o tēnei mate Best, me pēhea te whanaketanga, he aha ngā tohu, me te mea he rongoā kei reira.

He aha tēnei mate Pai rawa atu?

I te whakarapopototanga, he mate ira te mate a Best e pā ana ki te retina o ō kanohi. He rite ō tātou kanohi ki te kāmera. Ko te retina te paparanga kei muri o te kanohi e āwhina ana i a tātou ki te kite i ngā whakaahua ina pā te mārama ki a ia. Ko te wāhanga o te retina kei waenganui o te retina e āwhina ana i a tātou ki te kite i ngā mea tika ki mua ko te macula. Nō reira, i roto i te mate a Best, ka ngoikore haere te macula. Ka uaua pea te kite i ngā mea tika ki mua, pērā i ngā reta me ngā kanohi o te tangata, kia mārama. Engari i te nuinga o te wā , kāore te tirohanga taha, te kite rānei i ngā mea e karapoti ana i a koe, e tino pāngia ana.

Ko te ingoa o tēnei mate Best he rerekē. I ētahi wā ka karangahia e ngā tākuta ko te 'vitelliform macular dystrophy', ko te 'vitelliform dystrophy' rānei. Ko te 'Dystrophy' he kupu hauora mō te pirau, te ngoikore rānei o tētahi okana.

Tērā tētahi atu āhuatanga pēnei, he momo 'vitelliform macular dystrophy' anō hoki. Heoi, kāore e tīmata i te tamarikitanga, engari ka whanake i a koe e pakeke haere ana, i waenganui i te 40 me te 60 tau te pakeke . Ka kiia ko te 'momo tīmatanga-pakeke'.

He pēhea te noa o te mate Best?

He uaua ki te kimi tatauranga tika mō te tokomaha o ngā tāngata kua pāngia e te mate a Best. Kāore ētahi tāngata e mōhio kei a rātou tēnei mate nā te mea kāore he tohu o te mate. E ai ki tētahi rangahau , tata ki te kotahi i roto i te 16,500 tāngata, tata ki te kotahi i roto i te 21,000 rānei, ka pāngia pea e tēnei mate. E ai ki tētahi atu rangahau, tata ki te kotahi i roto i te 15,000 . E ai ki tētahi atu, tata ki te kotahi i roto i te 10,000 . Heoi, ka taea e tātou te mārama he mate tino onge tēnei.

He aha ngā wāhanga o te mate Best?

E ono ngā wāhanga matua o tēnei mate. Kia kite tātou he aha ēnei.

1. Wāhanga I - Wāhanga Previtelliform:

Koinei te tīmatanga. I te nuinga o te wā, kāore he tohu e pā mai ana i tēnei wā.Kāore anō kia tīmata te hanga o tētahi matū kōwhai i raro i tō retina. Tērā pea ka kitea tēnei i te wā e tirotirohia ana tō kanohi.

2. Wāhanga II - Wāhanga Vitelliform:

Ko te tikanga o te kupu 'vitelliform' he 'āhua hua manu'. I tēnei wā, ka tīmata te kohikohi o tētahi matū kōwhai i raro i tō retina, i te rohe o te 'macula', te pokapū o tō tirohanga. Whakaarohia he rite ki tētahi hua manu kōwhai iti. Tērā pea kei te pai tonu tō tirohanga i tēnei wā.

3. Wāhanga III - Wāhanga Pseudohypopyon:

I tēnei wā, ka huri te matū kōwhai hei wai, ka hanga he pūwero i raro i te retina. He rite tēnei ki tētahi pupuhi iti kī tonu i te wai i roto i te kanohi.

4. Wāhanga IV - Wāhanga Vitelliruptive:

Koinei te wāhi ka tīmata te pakaru me te marara o te rauemi kōwhai i kohikohia i mua. I te pakaru o tēnei rauemi, ka pakaru pea ngā pūtau o te retina. I tēnei wā, ka tīmata pea koe ki te kite i ngā huringa o tō tirohanga, pērā i te tirohanga pōuri.

5. Wāhanga V - Wāhanga ngoikore:

I tēnei wā, kua tata ngaro katoa te matū kōwhai. Engari kei te noho tonu ngā koha me ngā pūtau kua pakaru ki tōna wāhi i mua. Ka taea e tēnei kino te whakararu i te tirohanga. E whakaaro ana ētahi kairangahau koinei te wāhanga whakamutunga o te mate a Best.

6. Wāhanga VI - Te whanaketanga o te toto o te korokoro (CNV):

Ki tā ētahi kairangahau, ko tēnei āhuatanga e kiia nei ko te `CNV` te wāhanga whakamutunga o te mate Best. Ki tā ētahi he raruraru o te mate. Tata ki te 20% o te hunga whai mate Best ka pāngia pea e tēnei āhuatanga. Ko te `neovascularization` te hanganga o ngā oko toto hou. Ko te `Choroid` te kiriuhi i waenganui i te `retina` me te `sclera` o te kanohi. I roto i tēnei āhuatanga `CNV`, ka puta mai ngā oko toto hou, ngoikore i roto i te paparanga `choroid`. He tino ngoikore ēnei oko toto hou, ā, ka taea e rātou te turuturu toto, te wai rānei. Ki te tupu tēnei, ka kino haere tō tirohanga.

He aha ngā tohu o te mate Best?

I te nuinga o te wā, ka kitea noa te mate a Best i muri i te tirotiro kanohi, nā te mea i ētahi wā kāore he tohu e kitea ana. Engari ki te puta mai ngā tohu, ko ēnei pea:

• Te tirohanga pōuri: Ko ngā mea e kitea tika ana, tērā pea ka āhua kore mārama, ka pōuri rānei.
• Ngā raruraru o te tirohanga tika: Ko te raruraru ko te kite i ngā mea kei mua tonu i a koe. Engari tera pea he pai tō tirohanga ā-taha, ā-ao rānei.
• Te kitenga i ngā mea e huri ana te āhua (Metamorphopsia):Whakaarohia, kei te titiro koe ki tētahi rārangi tika, engari ki a koe he rite ki tētahi rārangi piupiu, he rārangi kua tuhia. Tērā pea ka kite koe i ngā mea pēnei i ngā kakau tatau me ngā anga matapihi kua tuhia. Ka kiia tēnei ko te 'metamorphopsia'.
• Te ngaronga nui o te tirohanga: Ka pāngia pea ētahi tāngata e te ngaronga nui o te tirohanga i roto i te wā.
• Te rerekētanga o te tirohanga i waenga i ngā kanohi e rua: Tērā pea he iti iho te tirohanga o te kanohi kotahi i te kanohi o tētahi. Kāore rānei e rite te hekenga o te tirohanga o ngā kanohi e rua, engari ka rerekē te hekenga.

He aha te take o te mate Best?

Ko te mate a Best te take o tētahi mate ira. He māmā noa iho, nā te panoni o ngā ira i riro mai i ō tātou mātua.

He mate "autosomal dominant" te mate a Best. He kupu hauora tēnei, engari he māmā noa te mārama. Ko te tikanga o te "autosomal dominant" kāore te tamaiti e whiwhi i te ira kua whakarerekētia mai i ngā mātua e rua, engari kotahi anake. Ko te tikanga, mēnā kei tētahi o ngā mātua te ira kua whakarerekētia, e 50% te tūponotanga o ā rātou tamariki ki te pāngia e te mate. He rite ki te whiu moni ka whiwhi upoko.

He mea whakamiharo, ko te ira kotahi e puta ai te mate a Best ka puta ai hoki ētahi momo mate kanohi tuku iho e kiia nei ko te retinitis pigmentosa. Ko te tikanga o tēnei, ka taea e ngā rerekētanga o tēnei ira te puta ai ngā momo mate kanohi.

Me pēhea te tautuhi i te mate Best?

I te nuinga o te wā, ka tātaritatia e te tākuta te mate a Best ina kei waenganui i te rima me te tekau tau te pakeke, i te nuinga rānei ina 20 tau te pakeke.

Ina toro atu koe ki tētahi tohunga tiaki kanohi mō tētahi raruraru kanohi, ka pātai tuatahi rātou ki a koe mō tō hītori hauora, ka pātai mēnā he raruraru kanohi tō tētahi o tō whānau, kātahi ka whakahaere i tētahi whakamātautau kanohi. Hei tāpiri, ka whakahaerehia pea e rātou ētahi whakamātautau motuhake. Hei tauira:

• Angiography fluorescein: He whakamātautau whakaahua tēnei. Ka werohia he tae motuhake ki roto i te uaua i roto i tō ringa, ā, ka tangohia he pikitia o ngā oko toto i roto i tō kanohi. Ka āwhina tēnei ki te kite mēnā he turuturu, he hapa rānei kei roto i ngā oko toto.
• Tomography arotau whatu (OCT): He whakamātautau whakaahua kore-whakaeke tēnei. Ka whakamahia ngā hihi mārama hei tango whakaahua whakawhiti-wāhanga o muri o tō kanohi, inā koa te retina me te macula. He rite ki te tapahi keke me te titiro ki roto. Ka taea e ia te kite i ngā mea pēnei i te matotoru o ngā paparanga o te retina, te pupuhi, me te putunga wai.
• Whakaahua tae o te fundus: Mā tēnei ka tangohia he whakaahua mārama o tō retina, ōna oko toto e pā ana, me te pane o te io whatu. Ka āwhina tēnei i a koe ki te kite mēnā he putunga kōwhai rite ki te hua manu.
• Te hiko o te kanohi: He raupapa whakamātautau tēnei. Ka inehia e ēnei whakamātautau te ngohe hiko ngawari e puta ana ina aro atu ō tātou kanohi ki te māramatanga. Ka taea e tēnei te whakaatu i te pai o te mahi a ngā pūtau o te retina.
• Ngā whakamātautau ira: Ka taea e ēnei whakamātautau te whakaū me te tino mohio mēnā he huringa kei roto i ō ira e pā ana ki te mate Best.

I ētahi wā , ka hiahia pea te tākuta ki te kōrero ki ētahi atu mema o tō whānau, ki te tirotiro rānei i ō rātou kanohi, he mate tuku iho tēnei.

Me pēhea te rongoā i te mate Best?

Tae noa mai ki tēnei rā, kāore anō kia kitea he rongoā mō te mate a Best. Koinei te mea tuatahi me mārama tātou.

Ko te huarahi pai ki te whakahaere i te mate ko te whakahaere i runga i ō tohu me te wāhanga o te mate. Kia kitea rā anō te mate, he mea nui kia tirotirohia ō kanohi i ia wā. Ko reira anake ka taea ai e koe te kite wawe i ngā huringa o ō kanohi, me te mahi i ngā mahi e tika ana.

I ngā wāhanga tōmua, kāore pea koe e hiahia ki tētahi maimoatanga. Heoi, ki te mea he hapa tirohanga anō ōu, pērā i te tirohanga tawhiti, me whakamahi pea koe i ngā mōhiti. He maha ngā wā ka raru te hunga whai mate Best ki te tirohanga tawhiti. Waihoki, ki te pāngia koe e te katarata, me mahi pea he pokanga katarata.

Engari, ki te mea kei a koe te āhua i kōrerotia i mua ake nei, arā, ko te choroidal neovascularization (CNV), arā, ko te hanganga o ngā oko toto ngoikore hou, ka tūtohu pea tō tākuta i ēnei maimoatanga hei aukati i te tipu o aua oko toto hou:

• Ngā rongoā ārai-pūnaha endothelial growth factor (Anti-VEGF): He rongoā ēnei. Ka werohia ēnei rongoā ki roto i te kōhao vitreous i roto i tō kanohi. Ko te whāinga he aukati i te tipu o aua uaua toto hou, turuturu, me te whakaiti i a rātou.
• Whakaora taiaho: Ka whakamahia e tēnei maimoatanga ngā hihi taiaho hei hiri, hei whakangaro rānei i ngā oko toto rerekē.
• Te rongoā whakaahua-whakaora (PDT): He maimoatanga uaua ake tēnei. I konei, ka werohia he rongoā motuhake ki roto i tō tinana, kātahi ka hangaia te rongoā kia aro ki te māramatanga, ā, ka tukuna he hihi taiaho ki ngā oko toto e pāngia ana, ka pakaru ai, ka aukati hoki i te turuturu o te toto.

Hei tāpiritanga, ka tūtohu pea tō tākuta kia whakamahia e koe ngā taputapu tirohanga iti, pērā i ngā taputapu whakanui, ngā rama motuhake, me ngā pūmanawa ngāwari ki te pānui.

Kei te rangahau kē ngā kairangahau mēnā ka taea te whakamahi i ngā rauemi ira hei rongoā, hei ārai rānei i te mate. Kei te wāhanga rangahau tonu te rongoā ira, engari e tūmanakohia ana ka puta he hua angitu ā muri ake nei.

Ka taea te whakaiti i te mōrearea o te whakawhanake i te mate Best?

He pono, kāore he mea e taea e koe te mahi hei ārai i te mate a Best. He mate ira tēnei. Engari, ki te pāngia tētahi o tō whānau e te mate a Best, ki te kitea rānei e koe kei a koe, he mea nui pea kia whiwhi koe i te tohutohu ira i mua i te whānau tamariki. Ka taea e te kaitohutohu ira te tirotiro i tō hītori whānau me te whakamārama i te mōrearea o te tukunga iho o te mate ki āu tamariki.

Ka aha mēnā kei a au te mate Best?

Ehara te mate a Best i te mate whakamate. Arā, ehara i te mea mōrearea ki te ora. Engari, e ai ki te kōrero i mua ake nei, kāore e taea te rongoā. E kore koe e matapo rawa i te mate a Best. Engari, tera pea he iti tō tirohanga. Arā, he uaua pea ki te mahi i ngā mahi o ia rā, engari e kore e ngaro rawa tō tirohanga.

Me pēhea taku tiaki i ahau anō?

Tērā ētahi kai hei oranga mō ngā kanohi.

"He mea tino nui te kai pai mō ngā kanohi."

Hei tauira, he pai mō ngā kanohi te kai ika i ētahi rā o te wiki, me te tāpiri i ngā huawhenua matomato, ngā hua rākau, me ngā nati ki tāu kai . Ka whakaratohia e ēnei he matūkai e pai ana mō te kanohi pērā i ngā waikawa ngako omega-3 me ngā huaora.

Mena kei a koe te mate a Best, he mea nui kia tirotirohia tō hauora i ia wā, inā koa ngā whakamātautau kanohi. Mena ka kite koe i ētahi huringa o tō tirohanga, o tō hauora whānui rānei, me kōrero tonu atu ki tō tākuta. Ka taea hoki e koe te pātai atu ki tō tākuta pēnei i:

• " Ka taea e koe te tūtohu i tētahi kaitohutohu ira hei āwhina i ahau ki te whakatau mēnā ka whānau tamariki ahau?"
• "E āhei ana ahau ki te whai wāhi atu ki tētahi whakamātautau haumanu mō tēnei mate?"
• " Ka taea e koe te tūtohu i tētahi tākuta kanohi e tohunga ana ki te tirohanga pōuri?"
• " He kōwhiringa maimoatanga te rongoā ira māku?"

He aha te rerekētanga i waenga i te mate Best me te mate Stargardt?

He mate ira te mate Stargardt me te mate Best, ā, e rua e pāngia ana te pokapū tirohanga o te kanohi (te macula). Ko te tikanga ka pāngia te tirohanga tika.

Engari ko ēnei mate e rua ka puta mai i ngā huringa o ngā ira rerekē.

I roto i te mate Stargardt, ka kitea e koe he kōwhai, he mā rānei ngā ira i runga i tō retina. Engari i roto i te mate Best, ka kitea e koe he whēwhe nui, kōwhai, he rite ki te toene hua manu, he āhua porowhita i raro i te macula. Nō reira, ka taea te wehewehe i ēnei mate e rua mā roto i ēnei tohu me te whakamātautau ira.

He uaua te noho me te mate e mōrearea ana ki te tirohanga. Engari ki te mea kei a koe te mate a Best, he maha ngā rauemi me ngā ratonga hei āwhina i a koe ki te whakahaere i ngā mea. Pātai atu ki tō tākuta mō ēnei.

Hei whakamutunga, ngā mea hei maumahara

Āe, kua maha ā tātou kōrero mō te mate Best. Me whakarāpopoto tātou:

• He mate tuku iho te mate a Best e pā ana ki te pokapū tirohanga o te kanohi (te macula). Ka taea e tēnei te arahi ki te tirohanga ngoikore.
• Ahakoa kāore e taea te whakaora katoa, ehara i te mea mōrearea ki te ora. Tērā pea ka pōuri te tirohanga, engari kāore e puta te matapo katoa.
• He mea tino nui te tautuhi i te mate i te wā wawe, me te whai i ngā whakamātautau hauora i ia wā.
• He maimoatanga kei reira mō ngā raruraru pēnei i te `CNV`.
• He mea nui te tohutohu ira mō te hunga e whakamahere ana i tētahi whānau.
• Mā te kai i ngā kai pai mō ngā kanohi, me te whakamahi i ngā taputapu āwhina mō te tirohanga pōuri, ka māmā ake te oranga.

Kia maumahara, kāore koe i te mokemoke. He tākuta, he kaiwhakatakoto whakaaro, me ngā tikanga hei āwhina i a koe ki te noho me ēnei āhuatanga. Ko te mea nui ko te noho kaha me te whai i ngā tohutohu e tika ana. Mena he pātai, he ruarua rānei āu, kōrero noa ki tō tākuta.

` Te mate pai rawa atu, Te mate pai rawa atu, ngā mate kanohi, te pokapū tirohanga, te macula, ngā mate ira, te ngaronga o te tirohanga, ngā whakamātautau kanohi