Kei te ahua ngoikore ō waewae? Tērā pea ko te Lambert-Eaton Myasthenic Syndrome (LEMS) tēnei!

Kei te ngoikore ō waewae i ētahi wā, he uaua ki te ara ake i te tūru, he ngenge koe i te piki arawhata? He ngenge rānei koe me ngā tohu pēnei i te taumaha o ngā kamo, te waha maroke, me te tirohanga rua? Ehara pea ēnei i te ngenge noa iho. I tēnei rā ka kōrero tātou mō tētahi āhuatanga kāore pea koe i rongo, engari he mea nui kia mōhiotia, ko te Lambert-Eaton Myasthenic Syndrome, ko te ``(LEMS)`` rānei te ingoa poto.

He aha te mate pukupuku Lambert-Eaton (LEMS)?

He whakarāpopototanga noa iho, ko te Lambert-Eaton Myasthenic Syndrome (LEMS) he mate pūnaha ārai mate. Tērā pea kei te whakaaro koe he aha te mate pūnaha ārai mate . He pūnaha ārai mate tō ō tātou tinana. Koinei te mea e tiaki ana i a tātou mā te whawhai ki ngā iroriki e puta ai te mate mai i waho. He rite ki te ope taua i tō tātou whenua. Engari i ētahi wā ka hē te pūnaha ārai mate nei. Kātahi ka tīmata te whakaeke i ō tātou ake pūtau hauora. Koinā te mea e tupu ana i roto i te LEMS.

I tēnei `(LEMS)`, ka whakaekea e tō tātou pūnaha ārai mate te hononga i waenga i ō tātou io nekeneke me ngā uaua . Hei whakamārama, ka kiia tēnei hononga ko te `neuromuscular junction`. Ina pakaru tēnei hononga, ka ngoikore te tuku karere mai i ngā io ki ngā uaua. Koinei te wā ka ngoikore ai ngā uaua.

I te nuinga o te wā ka tīmata tēnei ngoikore i ō huha o runga , engari ka taea e ia te horapa haere ki ō ringa, ki ō uaua manawa, horomi, me ō uaua kōrero.

Ko wai te mea ka nui ake te tūponotanga ka pāngia e tēnei `(LEMS)`?

E rua ngā rōpū matua e hanga ana i te `(LEMS)`.

1. E pā ana ki te mate pukupuku: Tata ki te 60% o te hunga whai LEMS he momo mate pukupuku tō rātou. Ko te momo tino noa ko te mate pukupuku pūkahukahu iti . He mea whakamiharo, i ētahi wā ka puta ngā tohu o te LEMS i ngā marama, i ngā tau rānei i mua i te whanaketanga o te mate pukupuku. Ko te nuinga o ēnei tāngata neke atu i te 60 tau te pakeke, he tāne, ā, kua whakamahi i ngā hua tupeka.

2. Kāore he mate pukupuku: Ko te toenga 40% o te hunga whai LEMS kāore anō kia kitea he mate pukupuku. Ko te tikanga ka kitea ēnei tāngata i te 35 tau pea, ā, i te 60 tau pea.

He tino onge te mate LEMS i waenga i ngā tamariki nohinohi.

He hononga anake te mate pukupuku pūkahukahu pūtau iti (LEMS) ki tēnei?

Kāo. Ko te mate pukupuku pūkahukahu iti te momo mate pukupuku tino noa, engari ka taea hoki te hono atu ki ētahi atu momo mate pukupuku me te LEMS. Hei tauira, ko te mate pukupuku repeure, te thymoma, me ngā mate lymphoproliferative. Nā te mea ka puta ngā tohu o te LEMS i mua i te putanga mai o ngā tohu o te mate pukupuku, he mea nui kia tika ngā whakamātautau hei tirotiro mō te mate pukupuku mēnā ka tātaritatia koe e tō tākuta he LEMS.

Nā ngā huringa ira te take o te LEMS?

E whakapono ana ngā kaipūtaiao he hononga ira kei ngā mate pūnaha ārai mate i te nuinga o te wā. Nā te mea he mate pūnaha ārai mate anō hoki a LEMS, tera pea he pānga ira anō hoki tōna, engari kāore anō kia tino māramahia tēnei.

He pēhea te noa o te `(LEMS)`?

He mate tino onge te LEMS. E kiia ana e pāngia ana e tēnei mate te 2.8 miriona tāngata puta noa i te ao. I te Hononga o Amerika, e 400 pea ngā kēhi kua pūrongohia mō te LEMS. Ahakoa kāore he tatauranga tika mō te tokomaha o ngā tāngata i tō tātou whenua, e mārama ana he mate onge tēnei.

He aha ngā tohu o te `(LEMS)`?

Me titiro tātou ki ngā tohu o te `(LEMS)` takitahi. Kāore e puta katoa ēnei tohu i te wā kotahi i roto i te katoa, ko ētahi tohu ka puta wawe ake, ko ētahi ka puta muri ake.

• Te ngoikore me te ngenge o ngā uaua: inā koa ki ngā waewae o runga.
• Te uaua ki te hikoi: Te uaua ki te piki arawhata, te whakatika rānei i te tūru.
• Te mamae o te uaua, te pakari rānei.
• Te ahua o te ngoikore o ngā peka.
• Ngā kamo e takoto kau ana (ptosis): He rite pea tēnei ki te hiamoe.
• Te kitenga i ngā mea e rua i te wā kotahi (diplopia).
• Waha maroke me ngā kanohi (xerostomia): kua iti te huware, kua iti te roimata i roto i ngā kanohi.
• Te mate kōroke.
• Te hekenga o te werawera.
• Te whakahekenga taumaha.
• Te uaua ki te mimi.
• Te ngoikore o te ure i roto i ngā tāne.
• Te uaua ki te kōrero (dysarthria) me te uaua ki te horomi (dysphagia): Ko ēnei ngā tohu ka puta ake i muri mai i te mate.
• Te uaua ki te manawa (dyspnea) me te mamae o te manawa: He tohu onge, he kino hoki tēnei ka puta i ngā wāhanga whakamutunga o te mate.

He mea nui: Ki te pāngia koe e tētahi, e ētahi rānei o ēnei tohu, kaua e whakaaro he LEMS. Heoi, ki te mau tonu ēnei tohu, he mea whakaaro nui kia rapu tohutohu hauora.

He pēhea te āhua me te ahunga whakamua o te LEMS i te nuinga o te wā?

Ko te tikanga ka pā tuatahi te LEMS ki ngā uaua o ngā huha . Kātahi ka pā ki ngā uaua o te pakihiwi, ngā uaua o ngā ringa me ngā waewae, ngā uaua e āwhina ana ki te kōrero me te horomi, me ngā uaua o ngā kanohi.

I te tīmatanga o te mate, tera pea ka uaua ki te whakatika ake i te tūru, te piki arawhata, i ngā pukepuke rānei, te mahi rānei i ngā mahi uaua e hiahia ana kia whakamahia ō waewae.

I te nuinga o te wā ka āta puta ngā tohu, i roto i ētahi wiki ki ētahi marama . Heoi, ki te mea kei a koe te LEMS me te mate pukupuku, ka tere ake pea te puta o ngā tohu.

He aha ngā take o te LEMS?

E ai ki tā tātou i kōrero ai i mua, he mate pūnaha ārai mate te LEMS. Ko te tikanga o tēnei ka whakaekea e ngā parepare o tō tinana (ngā paturopi) ō ake kiko hauora. I roto i te LEMS, ka whakaekea e ēnei paturopi te wāhi e tūtaki ai ngā pūtau io me ngā muka uaua, arā, te hononga io-uaua. Ina koa, ka whakaekea e rātou ngā awa konupūmā i reira. He mea nui ēnei awa konupūmā kia tukuna ngā karere io.

Whakaarohia he rite ki tētahi rarangi domino. Mā tēnei whakaeke ka tīmata he mekameka o ngā kaupapa:

1. Ka herea ngā paturopi ki ngā awa konupūmā i ngā pito o ngā pūtau io, ā, ka ārai i a rātou.

2. Ina whakaitihia ngā hongere konupūmā, ka heke te nui o te acetylcholine, he neurotransmitter, e tukuna ana. He matū nui tēnei acetylcholine mō te mahi a te uaua.

3. Ina kore e nui te acetylcholine e tukuna ana, kāore ngā "karere" ki ngā muka uaua e tae tika ana. Kātahi ka kore e taea e ō uaua te mahi tino pai.

4. Nō reira, kāore ngā uaua e mahi tika ana, ā, ka ngoikore te tinana.

Anei te āhua o te whakawhanaketanga o te LEMS ki te hunga whai mate pukupuku pūkahukahu iti: Ina he mate pukupuku kei roto i tō tinana, ka whiwhi ngā paturopi i te tohu "haere ki te pakanga." Engari i te kore e whakaeke i ngā pūtau mate pukupuku (ina koa, ngā awa konupūmā i te mata o ngā pūtau mate pukupuku), ka whakaeke hē ēnei paturopi i ngā awa konupūmā i ngā pito o ngā pūtau io. Kātahi ka puta te mekameka o ngā mahi.

E whakapono ana ngā kaipūtaiao ko te take o te LEMS i roto i te hunga kāore he matepukupuku pūkahukahu he hapa noa iho o te pūnaha ārai mate. Engari kāore anō kia mārama he aha i puta ai tēnei.

Me pēhea te tautuhi i te `(LEMS)`?

Ko te taahiraa tuatahi ki te tautuhi i te LEMS ko te tirotiro a tō tākuta i a koe. He maha ngā wā ka uru atu ki tēnei he tākuta roro, he tākuta tohunga rānei ki ngā mate o ngā io me ngā uaua. Ko tētahi wāhanga o tēnei tukanga ko te pātai mō ō tohumate, tō hītori hauora, me ngā rongoā katoa e tangohia ana e koe. Ka mahia he whakamātautau roro taipitopito hei rapu i ngā tauira ngoikore me ngā tohu e rite ana ki te LEMS.

Ko te mahi e whai ake nei ko te whakamātautau. Ko ngā whakamātautau mō te `(LEMS)` ko:

• Whakamātautau toto: Mā tēnei ka taea te tirotiro mēnā kei roto i ō toto ngā paturopi ārai-konupūmā. Tata ki te 85% o te hunga whai LEMS kei roto i ō rātou toto ēnei paturopi.
• Te Ine-hiko-ā-roto (EMG): Ka tirohia e tēnei whakamātautau te pai o te mahi tahi a ō uaua me ō io. I roto i te LEMS, he tino motuhake ngā kitenga o te whakamātautau EMG, nō reira ka taea e ia te āwhina ki te whakaū i te mate.
• Te hihi-X o te pūkahukahu, te matawai CT rānei, te matawai MRI rānei, te matawai MRI rānei (o te uma): Nā te mea ka taea te whakawhanake i tētahi āhuatanga e kiia nei ko te `(LEMS)` i roto i te hunga whai matepukupuku pūkahukahu iti, ka rapua e ēnei whakamātautau ngā tohu o te matepukupuku pūkahukahu.

Ka tīmata pea ngā tohu o te LEMS i ngā marama ki te ono tau i mua i te whanaketanga o te mate pukupuku. Nō reira, i muri i te tautuhi i te LEMS, me tirotiro koe i te mate pukupuku i ia toru ki te ono marama mō te rua tau pea te roa. Ka tūtohu tō tākuta i tētahi wā tirotiro e tika ana māu.

Me pēhea te rongoā mō te LEMS?

He maha ngā maimoatanga mō te LEMS.

• Te rongoā i te mate pukupuku taketake: Mena he hononga te LEMS ki tētahi mate pukupuku, pērā i te mate pukupuku pūkahukahu iti, mā te rongoā i taua mate pukupuku ka whakaiti i ngā tohumate LEMS. Tērā pea ko te chemotherapy, te radiation therapy, me/ranei te pokanga. Kei te momo mate pukupuku me ngā taunakitanga a tō tākuta tēnei.
• Ngā maimoatanga motuhake mō (LEMS): Kei roto i ēnei ko ngā rongoā hei whakaora i ngā tohu me ngā rongoā hei whakahaere i te pūnaha ārai mate.

Te maimoatanga mō ngā tohu

• Amifampridine (Firdapse®), Guanidine: Ka whakanui ake ēnei rongoā i te kaha o te uaua. Ka whakanui ake i te tukunga o te neurotransmitter acetylcholine i te hononga neuromuscular, ka whakapai ake i ngā tohu ki ngā uaua.
• Pyridostigmine (Pyridostigmine - Mestinon®): Ka mahi te Pyridostigmine mā te aukati i te pakaru o te acetylcholine i te hononga io-uaua. Ka whakanui ake hoki tēnei i te tohu acetylcholine, ā, ka whakapai ake i te kaha o te uaua. I ētahi wā ka hoatu te Pyridostigmine me te amifampridine hei whakapai ake i te mahi uaua me te rongoā i ngā tohu o te LEMS pēnei i te waha maroke, ngā kanohi maroke, te mate kōroke, te ngoikore o te moepuku, me te heke o te werawera. Heoi, ko te whakamahinga o te pyridostigmine mō te LEMS he whakamahinga kāore i te tūtohutia, ko te tikanga kāore te U.S. Food and Drug Administration (FDA) i whakaae i te rongoā mō tēnei mate.

Te maimoatanga ārai mate

• Ngā rongoā ārai mate:Ka āwhina ēnei rongoā ki te aukati, ki te whakahaere rānei i te mahi "parepare" o tō pūnaha ārai mate. Tērā pea ka tohua ēnei rongoā e tō tākuta mēnā kāore ētahi atu rongoā e whakahaere i ō tohumate. Hei tauira, ka taea te hoatu he rongoā e kīia nei ko te prednisone me te azathioprine, te cyclosporine rānei, me te kore rānei.
• Whakawhitinga parāma: I roto i tēnei tikanga, ka wehea e te mīhini te wāhanga wai o tō toto (parāma) mai i ngā pūtau toto. Kei roto i tēnei parāma ō paturopi. Whai muri i te wehenga, ka whakahokia te toto ki roto i tō tinana. Tērā pea ka whakaarohia e tō tākuta tēnei maimoatanga mēnā he nui ngā tohumate kei a koe, ā, me tere te maimoatanga.
• Ngā immunoglobulin: Mō ētahi tāngata, mā te rongoā immunoglobulin nui ka āwhina i te ārai i te kino haere o te mate.

Ka taea te ārai i te LEMS?

Kāore anō kia tino mārama te pūtake tuturu o te LEMS, nō reira kāore i te mōhiotia mēnā he huarahi hei ārai i te mate.

Heoi, i te mea ka taea e te LEMS te whanake i roto i te hunga whai matepukupuku pūkahukahu, ka taea e koe te āwhina ki te ārai i te matepukupuku pūkahukahu mā te:

• Kaua e momi hikareti. Ki te whakamahi koe i ngā hua tupeka, pātai atu ki tō tākuta kia āwhina koe ki te whakamutu.
• Āta karohia te auahi tuarua.
• Tirohia tō whare mō te hau radon. Mā te roa o te wā e pāngia ai koe e te hau radon ka nui ake te tūponotanga o te mate pukupuku pūkahukahu.

Ka taea hoki e koe te mahi i ngā mahi hei pupuri i te oranga hauora:

• Kai atu i ngā hua me ngā huawhenua. Whāia te kai Mediterranean, te kai DASH rānei.
• Kia mau tonu te taumaha hauora.
• Whakakorikori tinana kia rite ki ngā tohutohu a tō tākuta.
• Kia nui te moe - e whitu ki te iwa hāora te roa.
• Whakaitihia tō ahotea. Ka taea e te ahotea te whakararu i ngā tohumate.
• Kaua e kaukau i te wai wera. Ka taea e te wera te whakararu i ngā tohu o te LEMS.
• Tirohia tō tākuta i ia wā, ā, whakamōhio atu ki a ia mēnā ka rerekē tō hauora, mēnā rānei ka puta he tohu hou ki a koe.

Ka taea te whakaora katoa i te LEMS?

Engari, kāore he rongoā mō te LEMS. Kua whakaatuhia te rongoā i te mate pukupuku e pā ana ki te LEMS ka whakapai ake i ngā tohu o te LEMS. Ka taea e ngā rongoā ārai mate te āwhina ki te whakahaere i te whakaekenga ā-pūnaha, ā, kei reira anō hoki ngā maimoatanga ka āwhina ki te whakamamae i ngā tohu mā te whakanui ake i te nui o te acetylcholine i roto i ngā pito io me te whakapai ake i te kaha o te uaua.

Mena kei a koe te LEMS me te kore matepukupuku, ka taea e koe te tumanako kia roa te oranga. Mena kei a koe te LEMS me te matepukupuku, tera pea ka pai ake ō tohu i roto i te wā (ahakoa kāore i te tino pai) mena ka kitea wawe te matepukupuku me te whakahaere i te wā tika.

Ko te kounga o tō oranga e whakawhirinaki ana ki te kaha o ō tohu.

Āhea ahau me whakapā atu ki taku tākuta mēnā kei a au te `(LEMS)`?

Mena ka rerekē tō hauora, ka kino haere rānei ō tohumate, whakapā atu ki tō tākuta i ngā wā katoa . Kaua e mataku, kaua e mataku. Kei reira ngā tākuta hei āwhina i a koe.

He aha te rerekētanga i waenganui i te `(LEMS)` me te `(Myasthenia Gravis - MG)`?

He rite tonu ngā tohu o te Lambert-Eaton myasthenic syndrome (LEMS) me te myasthenia gravis (MG). Ka ngoikore te uaua me te ngenge o ēnei e rua.

Heoi, ko te myasthenia gravis (MG) te tikanga ka pā tuatahi ki ngā uaua e karapoti ana i ō kanohi me ngā uaua e whakamahia ana mō te horomi. Ā muri ake nei, ka pā pea ki ngā uaua whānui, tae atu ki ngā uaua manawa me ngā uaua o ngā peka.

Ko te LEMS ka tīmata mā te ngoikore o ō huha o runga, kātahi ka neke ki ō ringaringa. Ka taea hoki e te LEMS te ngoikore i ngā uaua o ō kanohi, engari he ngawari ake i te myasthenia gravis (MG).

I roto i te myasthenia gravis (MG), ka mate pea koe i te ngoikore nui o ngā uaua manawa (ngā uaua e āwhina ana i a koe ki te manawa). He onge tēnei i roto i te LEMS.

Ko te hunga whai myasthenia gravis (MG) kāore e whakaatu i ngā tohu maha o te ngoikore o te pūnaha io ā-motu (hei tauira, ngā kanohi maroke, te waha maroke, te mate kōroke, te iti o te werawera, te ngoikore o te moepuku). He rerekē, ko te hunga whai LEMS he maha tonu ngā amuamu, ā, i ētahi wā ka taea e ēnei tohu te āwhina ki te wehewehe i ngā mate e rua.

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

Kua mārama pea koe i nāianei ko te Lambert-Eaton Myasthenic Syndrome (LEMS) he mate e puta mai ana i te pūnaha ārai mate e kaha rawa ana te mahi. Kāore ō pūtau io e tuku karere ki ngā muka uaua kia "whakaiti". Ko te mutunga iho he ngoikore o te uaua me ētahi atu tohu.

Kia maumahara, tata ki te 60% o te hunga whai LEMS he mate pukupuku pūkahukahu iti tō rātou. Mena kei a koe te LEMS engari kāore he mate pukupuku, kia tirohia kōrua ko tō tākuta i ia marama torutoru mō te rua tau pea i muri i te tātaritanga.

He maimoatanga kei reira. Kei roto i ēnei ko te rongoā i te mate pukupuku mēnā kei te kitea, me te tango i ngā rongoā hei whakapai ake i te whakawhitiwhiti kōrero i waenganui i ngā pūtau io me ngā muka uaua. Mā te whakapai ake i te whakawhitiwhiti kōrero ka taea te whakapai ake i te kaha o te uaua me ētahi atu tohu.

Ko tāku tumanako he whai hua ēnei mōhiohio ki a koe.Kia maumahara tonu, ki te mea he āwangawanga hauora āu, he pai ake te kōrero ki tētahi tākuta mātau mō tēnā.

` Te mate pukupuku myasthenic Lambert-Eaton, LEMS, ngā mate pūnaha ārai mate, ngoikore o te uaua, mate pukupuku pūkahukahu pūtau iti, hononga io-uaua, acetylcholine