He aha te Amyotrophic Lateral Sclerosis (ALS)? Ngā Tohutohu, Ngā Take, me te Maimoatanga

Ko te amyotrophic lateral sclerosis, e kiia ana ko te ALS, he mate neurology e haere tonu ana, e pā ana ki ngā pūtau io e whakahaere ana i te nekehanga o ngā uaua e whakahaerehia ana e te hinengaro. Ka pakaru haere ngā pūtau motor neuron, ā, ka mutu te mahi tika. Ka pā te ngoikore o ngā uaua, ka tīmata pea i tētahi wāhi, ka horapa haere ki tō tinana i te wā.

He aha te ALS?

E pēnei ana te ALS ki ngā pūtau motor neuron i roto i tō roro me tō tuaiwi. Ko ēnei pūtau io motuhake he karere, e tuku ana i ngā tohu mai i tō roro hei whakahaere i ngā uaua e taea te nekehanga, pēnei i ngā ringa, ngā waewae, me te mata.

Ka ngoikore ngā pūtau motor neuron, ka pakaru te hononga i waenganui i tō roro me ngā uaua. Ka ngaro haere te kaha o ngā uaua ki te whiwhi i ngā tohu tika, ka pā te ngoikore, te wiri, me te ngaro rawa atu o te mahi i ngā wāhi e pā ana.

Ko te kupu ‘amyotrophic’ e kī ana ‘kāhore he kai mō ngā uaua’, ā, ko te ‘lateral sclerosis’ e kī ana ki te pakari o te kiko io i ngā wāhi motuhake o te tuaiwi. Ahakoa tōna ingoa uaua, ko te tikanga o te ALS ko te ngaro o te kaha o tō tinana ki te whakahaere i te nekehanga o ngā uaua i te wā.

He aha ngā tohu o te ALS?

Ka tīmata haere ngā tohu o te ALS, ā, ka rerekē pea i te tangata ki te tangata. Ka tīmata pea ngā tohu tuatahi i tētahi wāhi motuhake i mua i te horapa ki ētahi atu wāhi o tō tinana.

Ko ngā tohu tuatahi tino noa:

• Te ngoikore o ngā uaua i ngā ringa, ngā takakau, ngā waewae rānei e āwhina ana i ngā mahi o ia rā
• Te wiri, te koikoi rānei o ngā uaua, ina koa i ngā ringa, ngā pakihiwi, te arero rānei
• Te uaua ki ngā pukenga motuka pai, pēnei i te pihi i ngā kakahu, te tuhituhi rānei
• Te tū, te hinga rānei i te nuinga atu i te noa
• Te kōrero pororaru, ngā huringa rānei i tō reo
• Te uaua ki te horomi, te ngau kai rānei

Ka haere tonu te mate, ka kite pea koe i te ngoikore o ngā uaua e pā ana ki tō kaha ki te haere, te whakamahi i ō ringa, te kōrero mārama rānei. Ka kino haere ngā tohu i ngā marama ki ngā tau, ahakoa ka rerekē te tere i waenganui i ngā tangata.

He mea nui kia mōhio kei te pā te ALS ki tō whakaaro, ki tō mahara, ki ō āhua rānei. E mau tonu ana tō hinengaro ahakoa ka puta ngā tohu tinana, he mea whakamarie, he wero hoki tēnei mō te tini o te iwi.

He aha ngā momo ALS?

E rua ngā momo matua o te ALS e whakaatuhia ana i runga i te whāinga me te take. Ka āwhina tēnei ki te whakamārama i tō āhuatanga motuhake.

Ko te ALS sporadic te nuinga o ngā keehi, 90-95%. Ka puta tēnei momo kahore he take whānau mārama, he take ira rānei. Ko te nuinga o te hunga kua tohua he ALS sporadic, ā, kei te mahi tonu ngā kairangahau ki te kimi i ngā take.

Ko te ALS whānau e pā ana ki te 5-10% o te hunga e mate ana, ā, ka haere i roto i ngā whānau. Ko te take o tēnei momo ko ngā rerekētanga ira e tukuna ana mai i ngā mātua ki ngā tamariki. Mēnā he ALS whānau tōu, ka kitea pea he tauira o te mate i roto i ngā mema whānau maha i ngā whakatupuranga.

I roto i ēnei momo matua, ka whakaatu ngā tākuta i te ALS i runga i te wāhi i puta ai ngā tohu tuatahi. Ka tīmata te ALS waewae ki te ngoikore o ngā ringa, ngā waewae rānei, ā, ka tīmata te ALS bulbar ki ngā raruraru kōrero, te horomi rānei.

He aha ngā take o te ALS?

Kāhore e tino mōhiotia te take o te ALS, he mea whakatoi pea tēnei ina rapu koe i ngā whakautu. E whakapono ana ngā kairangahau he whakakotahitanga uaua o ngā take ira, taiao, me te noho.

Mō te ALS whānau, ko ngā rerekētanga ira te take matua. Ko ngā ira tino noa ko te C9orf72, SOD1, TARDBP, me te FUS. Ka pā ēnei rerekētanga ira ki te mahi o ngā pūtau motor neuron, ka mate haere.

I te ALS sporadic, kāhore e tino mōhiotia ngā take. Kei te rangahau ngā kairangahau i ētahi take pea:

• Ngā poizon, ngā matū rānei o te taiao e pā ana ki ngā pūtau motor neuron
• Ngā mate huaketo e pā ana ki te whakautu mate koretake
• Ngā raruraru ki te tukatuka pūmua i roto i ngā pūtau io
• Te ahotea oxidative e pā ana ki ngā wāhanga pūtau
• Te mumura i roto i te pūnaha io

Ko ētahi keehi onge pea e hono ana ki ngā take morearea, pēnei i te mahi hoia, te mahi tinana kaha, te pā ki ētahi matū rānei. Heoi, ko te nuinga o te hunga e pā ana ki ēnei mea kāhore e pā ki te ALS, e whakaatu ana he maha ngā take e tika ana kia puta ai te mate.

Nōhea te haere ki te tākuta mō ngā tohu ALS?

Me toro atu koe ki tētahi kaiwhakarato hauora mēnā ka kite koe i te ngoikore o ngā uaua, te wiri, ngā huringa kōrero, te horomi rānei e roa atu i ngā wiki. He mea nui te aromatawai wawe, he maha hoki ngā mate e rite ana ki ngā tohu ALS, ā, ka āwhina te tohu wawe ki te whakarite kia whiwhi koe i te tiaki tika.

Rapua te awhina hauora mēnā ka uaua ki ngā mahi o ia rā, pēnei i te tuhituhi, te haere, te kōrero mārama rānei. Ahakoa he maha ngā take o ēnei tohu, ka āwhina te aromatawai kia kitea he aha te mea kei te puta, kia whakakorea ngā mate atu e taea te whakaora.

Kaua e tatari mēnā kei te uaua ki te horomi, te manawa rānei, i te mea pea ka hiahia ēnei tohu ki te tiaki tere. Ka taea e tō tākuta te aromatawai mēnā ka hiahia ēnei huringa ki te whakauru tere, ki te whakahaere rānei mā te tiaki arā.

He aha ngā take morearea mō te ALS?

He maha ngā take ka piki ake ai tō tūponotanga ki te whiwhi ALS, ahakoa te mea kei a koe ngā take morearea, kāhore e tikanga ka whiwhi koe i te mate. Ka āwhina te mōhiotanga ki ēnei take ki te whakarite i tō āhuatanga.

Ko te tau te take morearea tino nui, ko te nuinga o te iwi e whiwhi ana i te ALS i waenganui i ngā tau 40 me te 70. Ko te tīmatanga nui kei te 55 tau te pakeke, ahakoa ka pā pea te mate ki ngā tāne taitamariki, ki ngā kaumātua rānei.

He wāhi te ira tangata, ko ngā tāne te tino tūponotanga ki te whiwhi ALS i mua i te 65 tau. I muri i te 70 tau, ka iti haere tēnei rerekētanga, ka rite te tūponotanga i waenganui i ngā tāne me ngā wāhine.

Ko ētahi atu take morearea pea:

• Te hītori whānau o te ALS, ngā mate neurology e hono ana rānei
• Te mahi hoia, ina koa i ngā wāhi i tonoa atu ai
• Te mahi tinana kaha, te hākinakina rānei i ngā taumata ngaio
• Te pā ki ētahi matū, konganuku, rānei
• Te whakakī, ka piki ake pea te tūponotanga
• Te whara o te matenga, ngā whara o te roro rānei

Ko te nuinga o te hunga e pā ana ki ēnei take morearea kāhore e pā ki te ALS, ā, he tokomaha ngā tāngata e pā ana ki te ALS kāhore he take morearea e kitea ana. Ko te wāhi o ngā take morearea e kī ana pea he tūponotanga teitei, ehara i te tino.

He aha ngā raruraru pea o te ALS?

Ka puta haere ngā raruraru ALS i te wā e haere tonu ana te ngoikore o ngā uaua, e pā ana ki ngā pūnaha tinana rerekē i te wā. Ka āwhina te mōhiotanga ki ngā raruraru pea ki a koe, ki tō rōpū hauora rānei ki te whakarite i te tiaki whānui.

Ko ngā raruraru manawa tētahi o ngā raruraru tino nui. Ka ngoikore ngā uaua manawa, ka pā pea te poto o te manawa, ina koa i te takoto, ngā mate manawa maha, te ngenge rānei i te kaha atu e hiahiatia ana ki te manawa.

Ka pā ngā raruraru horomi, e kiia ana ko te dysphagia, ki ētahi raruraru:

• Ngā wā e ngākau kino ana i te wā e kai ana, e inu ana rānei
• Te mate huango aspiration mai i te kai, te wai rānei e tomo ana ki ngā pūkahukahu
• Te kore kai, te ngaro taimaha rānei i te uaua ki te kai
• Te maroke i te iti o te inu wai

Ka puta pea ngā raruraru kōrero i te wā e ngoikore ana ngā uaua e whakahaere ana i te kōrero. Ka pā pea tēnei ki te motu hapori me te riri, ahakoa ka āwhina ngā hangarau tautoko ki te pupuri i tō kaha ki te kōrero pai.

Ko ngā raruraru nekehanga ko te piki o te tūponotanga hinga, ngā pupuhi pehanga mai i te nekehanga iti, te pakari rānei o ngā hononga. I tua atu, ka pā pea ki ētahi te huringa o ngā kare-a-roto, te pouri rānei, he whakautu tika tēnei ki ngā wero o te noho tahi me te ALS.

Ahakoa ka rongo pea ēnei raruraru he nui rawa, ka taea te whakahaere pai i te nuinga mā te tiaki hauora tika, ngā taputapu tautoko, me te tautoko mai i ngā tohunga hauora e mōhio ana ki te tiaki ALS.

Me pēhea te tohu ALS?

Ko te tohu ALS e whakawhirinaki ana ki te tirohanga hauora me te whakakore i ētahi atu mate, i te mea kāhore he whakamātautau tino. Ka roa pea te tukanga i ngā marama, ā, ka whakauruhia ngā momo aromatawai.

Ka tīmata tō tākuta ki te hītori hauora taipitopito me te whakamātautau tinana, e titiro ana ki ngā tauira o te ngoikore o ngā uaua me ētahi atu tohu neurology. Ka whakamātautau rātou i ō whakautu, te kaha o ngā uaua, te whakahaere rānei hei kimi i ngā wāhi morearea.

He maha ngā whakamātautau tohu e āwhina ana ki te tautoko i te tohu:

• Ko te Electromyography (EMG) e ine ana i te mahi hiko i roto i ngā uaua, ka taea te kite i te kino o ngā io
• Ngā rangahau ara ia e aromatawai ana i te pai o te haere o ngā tohu hiko i roto i ngā io
• Ngā whakaahua MRI o tō roro me tō tuaiwi e whakakore ana i ētahi atu mate neurology
• Ngā whakamātautau toto e tirotiro ana i ngā mate huaketo, ngā mate autoimmune, ngā raruraru metabolic rānei
• Te whakamātautau ira mēnā he hītori whānau o te ALS

Ka hiahia pea te tohu ki te wā me te manawanui, i te mea me tirotiro marie ngā kairangahau i te whāinga o ngā tohu, me te whakakore i ngā mate pēnei i te multiple sclerosis, ngā mate pūngāwerewere o te tuaiwi, te neuropathy taha rānei e pā ana ki ngā tohu rite.

Pea ka whakauruhia ngā neurologist e mōhio ana ki te ALS ki tō rōpū hauora, i te mea ka āwhina tō rātou mātauranga ki te whakarite i te tohu tika me te whakarite i te mahere tiaki mai i ngā tīmatanga.

He aha te maimoatanga mō te ALS?

Ahakoa kāhore he rongoā mō te ALS i tēnei wā, he maha ngā maimoatanga ka āwhina ki te whakaiti i te whāinga o te mate, te whakahaere i ngā tohu, me te pupuri i tō kounga ora. Ko te whakaaro ki te tiaki whānui e pā ana ki ngā āhuatanga maha o te mate.

E rua ngā rongoā kua whakaaetia e te FDA e āwhina ana ki te whakaiti i te whāinga o te ALS. Ka roa pea te ora o te Riluzole i ngā marama mā te whakaiti i ngā taumata glutamate i roto i te roro. Ka whakaiti pea te Edaravone, e hoatu ana mā te whakauru IV, i te hekenga o te mahi o ia rā mō ētahi tāngata e pā ana ki te ALS.

Ko te whakahaere tohu e whakauru ana i tētahi whakaaro maha-tohunga:

• Te rongoā tinana hei pupuri i te kaha me te ngawari
• Te rongoā mahi hei āwhina i ngā mahi o ia rā me ngā taputapu whakarite
• Te rongoā kōrero mō ngā raruraru kōrero me te horomi
• Te rongoā manawa hei tautoko i te mahi manawa
• Te tohutohu kai hei pupuri i te kai tika

Ka haere tonu te mate, ka nui haere te hiranga o ngā taputapu tautoko. Pea ka whakauruhia ngā taputapu nekehanga pēnei i ngā kaikōrero, ngā waka wira rānei, ngā taputapu kōrero mō ngā raruraru kōrero, me ngā taputapu tautoko manawa.

He tino motuhake te whakaaro maimoatanga, ka whakaritea e tō rōpū hauora ngā tūtohu i runga i ō tohu motuhake, te tere o te whāinga, me ō hiahia whaiaro mō te tiaki.

Me pēhea te whakahaere i te ALS i te kāinga?

Ko te whakahaere kāinga o te ALS e aro ana ki te pupuri i te whakamarie, te ora, me te motuhake kia roa rawa. Ka taea e ngā whakarerekētanga me ngā rautaki ngāwari te whakahaere i te ora o ia rā.

He mea nui te hanga i tētahi taiao kāinga ora i te wā e huri haere ana te nekehanga. Tangohia ngā mea e tū ai, tāruatia ngā pou e pupuri ana i ngā ruma kaukau, whakaritea te mārama pai i tō kāinga, ā, whakaarohia ngā rāmapa mēnā ka uaua ngā kaupae.

Ka nui haere te tautoko kai i te wā e puta ana ngā raruraru horomi:

• Kōwhiria ngā kai ngawari e ngawari ana te ngau me te horomi
• Kai iti, maha atu ngā kai hei whakaiti i te ngenge
• Noho tika i te wā e kai ana, 30 meneti i muri mai rānei
• Mahi tahi me tētahi kaiārahi kai hei whakarite i te kai tika
• Whakaarohia ngā taapiri kai mēnā ka tūtohuhia

Ka āwhina te tiaki kaha ki te pupuri i ngā taumata mahi mō te wā roa. Whakaritea ō mahi tino nui mō ngā wā e kaha ana koe, tangohia ngā okioki, ā, kaua e mangere ki te tono awhina mō ngā mahi tinana.

He mea nui te whakarite kōrero i te wā e puta ana ngā huringa kōrero. Akohia ngā taputapu kōrero tautoko i te wā wawe, whakamahia i te wā e mārama ana tō kōrero, ā, whakaritea ngā hiahia kōrero ki ngā mema whānau.

Me pēhea te whakarite mō tō hui tākuta?

Ko te whakarite pai mō ngā hui hauora e pā ana ki te ALS ka āwhina ki te whakarite kia whiwhi koe i te painga nui mai i tō wā tahi me ngā kaiwhakarato hauora. Ka āwhina te whakarite pai ki ngā kōrero hua me te pai o te whakarite tiaki.

Pupurihia tētahi tāpuhi tohu taipitopito i waenganui i ngā hui, e tuhi ana i ngā huringa kaha, ngā tohu hou, ngā raruraru rānei ki ngā mahi o ia rā. Whakauruhia ngā tauira motuhake o ngā mahi kua uaua ake, me te wā i kite tuatahi ai koe i ēnei huringa.

Whakaritea ō pātai i mua, ā, whakaritea ngā mea tino nui:

• He aha ngā huringa e tumanakohia ana e au?
• He maimoatanga hou, he whakamātautau rongoā rānei e tika ana kia whakaarohia e au?
• He aha ngā taputapu tautoko ka āwhina mai ki ahau inaianei?
• Me pēhea te pupuri i toku motuhake mō te wā roa?
• He aha ngā rauemi kei te wātea mō toku whānau?

Mauria tētahi rārangi katoa o ngā rongoā, ngā taapiri, me ngā taputapu tautoko kei te whakamahi koe. Whakauruhia ngā mōhiohio mō te mahi pai, me te kore e homai te painga e tumanakohia ana.

Whakaarohia te kawe mai i tētahi mema whānau, hoa rānei ki ngā hui. Ka taea e rātou te awhina ki te mahara ki ngā mōhiohio nui, te ui i ngā pātai pea ka wareware koe, te tuku i ngā tirohanga atu mō ngā huringa kua kitea e rātou.

He aha te mea nui mō te ALS?

He mate neurology nui, e haere tonu ana te ALS, engari ko te mōhiotanga ki a ia ka whakamana koe ki te mahi tahi me tō rōpū hauora me te pupuri i te kounga ora. Ahakoa ka rongo pea te tohu he nui rawa, he tokomaha ngā tāngata e pā ana ki te ALS e kite tonu ana i te tikanga, te harikoa, te hononga hoki i roto i ō rātou oranga.

Ko te matua o te noho pai me te ALS ko te tiaki whānui, te whakarite wawe, me ngā pūnaha tautoko kaha. Ka taea e ngā hangarau tautoko hou, ngā rautaki whakahaere tohu, me ngā rōpū tiaki maha-tohunga te whakautu i ngā wero e puta mai ana.

Kia mahara ka rerekē te pā o te ALS ki te tangata ki te tangata, me ngā tere rerekē o te whāinga me ngā tauira tohu. Ka motuhake tō wheako, me whakarite ngā maimoatanga ki ō hiahia me ō hiahia motuhake.

Kei te haere tonu te rangahau ki te ALS, me ngā maimoatanga me ngā whakaaro hou e rangahautia ana. Ko te hono atu ki tō rōpū hauora me ngā whakahaere ALS ka āwhina ki te ako i ngā kōwhiringa me ngā rauemi hou.

Ngā pātai e ui pinepinetia ana mō te ALS

He mate mate tonu te ALS?

I tēnei wā, e kiia ana he mate mate te ALS, engari ka rerekē te ora i waenganui i ngā tangata. He tokomaha ngā tāngata e ora ana mō ngā tau maha i muri i te tohu, ā, he tokomaha ngā tāngata e tere ana te whāinga. Ko te 20% o te hunga e pā ana ki te ALS e ora ana i te rima tau neke atu i muri i te tohu, ā, he iti te pēkenga e ora ana mō te wā roa atu. Kei te haere tonu ngā maimoatanga hou me te tiaki tautoko ki te whakapai ake i ngā hua me te kounga ora.

Ka taea te aukati i te ALS?

I tēnei wā, kāhore he huarahi e mohiotia ana ki te aukati i te ALS, ina koa te momo sporadic e pā ana ki te nuinga o te iwi. Mō te ALS whānau, ka āwhina te tohutohu ira ki ngā whānau ki te mōhio ki ō rātou tūponotanga, engari kāhore e taea te aukati. Heoi, ko te pupuri i te hauora pai mā te whakakori tinana, te kore whakakī, te whakaiti rānei i te pā ki ngā poizon taiao pea ka tautoko i te hauora neurology whānui.

Kei te pā te ALS ki te whakaaro me te mahara?

Ko te nuinga o te hunga e pā ana ki te ALS e pupuri ana i te whakaaro me te mahara noa i te wā o to rātou mate. Heoi, ko te 15% o te hunga e pā ana ki te ALS ka whanake hoki te mate dementia frontotemporal, ka pā pea ki te whakaaro, te whāinga, me te tuakiri. Ahakoa ka puta ngā huringa hinengaro, ka rerekē pea te whāinga i ngā tohu uaua, ā, pea ka iti i te tīmatanga.

Ka taea tonu e te hunga e pā ana ki te ALS te mahi?

He tokomaha ngā tāngata e pā ana ki te ALS e mahi tonu ana mō tētahi wā i muri i te tohu, ina koa i ngā mahi kāhore e hiahia ki te hiahia tinana nui. Ka taea e ngā whakaritenga pēnei i ngā wā mahi ngāwari, ngā hangarau tautoko, ngā mahi whakarerekē rānei te whakawhānui i te kaha mahi. Ko te whakatau e whakawhirinaki ana ki ngā āhuatanga motuhake, te whāinga o ngā tohu, me ngā hiahia whaiaro mō te pupuri i te mahi.

He aha ngā tautoko kei te wātea mō ngā whānau e pā ana ki te ALS?

He tautoko whānui kei te wātea mō te hunga e pā ana ki te ALS me ō rātou whānau. Ka whakauruhia ngā pūtahitanga ALS Association e whakarato ana i ngā nama taputapu, ngā rōpū tautoko, me ngā rauemi mātauranga. He maha ngā hapori e whakarato ana i te tiaki wātea, ngā ratonga tohutohu, me ngā kaupapa awhina putea. Ko ngā rōpū hauora e whakauruhia ana ngā kaimahi pāpori e taea te hono atu i ngā whānau ki ngā rauemi rohe me ngā rauemi motu.