He raruraru anō tōu e whakakeke ana, e ngoikore ana hoki ō waewae? Me kōrero tātou mō te Hereditary Spastic Paraplegia

I ētahi wā ka rongo koe i te wiri o ō waewae ina hikoi koe, me te mea kāore he mana whakahaere mōu? Ka tūtaki koe ki tētahi mea iti ka hinga, ka uaua rānei ki a koe te hiki i ō waewae ina piki arawhata? Kāore pea ēnei i te mea noa iho. I tēnei rā ka kōrero tātou mō tētahi āhuatanga e pā ana ki ngā waewae, he āhua uaua, engari he mea nui kia mōhiotia. He āhuatanga tēnei e kiia nei ko te "Hereditary Spastic Paraplegia".

He aha tēnei paraplegia spastic tuku iho?

He whakarāpopototanga noa iho tēnei, he rōpū mate tuku iho tēnei. Ko te tikanga o te "tuku iho" ka taea e koe te tuku iho i te mate mā roto i ngā ira mai i tō whaea, i tō pāpā, i ētahi atu mema o te whānau rānei. Ko te "spastic paraplegia" e pā ana ki te pakari ((spasticity)) me te ngoikore haere o ngā uaua o ō waewae . Kāore ēnei tohu e puta ohorere mai, engari ka piki haere haere i roto i te wā .

I te tīmatanga, tērā pea ka pāngia koe e te mamae i a koe e hikoi ana. Tērā pea ka tūpono koe ki te tūtaki i tētahi mea, ki tētahi kōhatu iti rānei i te huarahi. Nā te mea kāore e taea e koe te hiki tika i tō waewae. I roto i te wā, ka kino haere pea tēnei āhuatanga, ā, ka noho morearea pea te hikoi. Tērā pea me whakamahi ētahi tāngata i te tokotoko, i te taputapu hikoi, i te tūru wīra rānei hei āwhina i a rātou ki te hikoi i muri i ētahi tau.

Tērā pea ka rongo koe i ētahi tākuta e whakamahi ana i ētahi atu ingoa mō tēnei mate:

• (Te paraparesis spastic whānau)
• (Te paraparesis spastic tuku iho)
• (Te mate Strümpell-Lorrain)

Ahakoa te ingoa e hoatu ana e koe, he rite tonu te tikanga o tōna mate hauora.

He momo (Hereditary Spastic Paraplegia)?

Āe, neke atu i te 80 ngā momo o tēnei mate. Ka hoatu he tau ki ia momo, pērā i te (SPG1), (SPG2), i te raupapa i kitea ai. Engari ka wehea ēnei e ngā tākuta kia rua ngā rōpū matua:

1. Momo Kore-uaua (Pure rānei): Tata ki te 90 ōrau o te hunga whai mate nei he momo ngāwari tēnei . Ko ngā tohu matua ko te pakari o te uaua ((spasticity)) me te ngoikore o ngā waewae i kōrerohia i mua ake nei.

2. Momo uaua: Ko te toenga 10 ōrau he momo uaua tēnei . Haunga te pakari me te ngoikore o te waewae, tera pea ka pāngia koe e ētahi atu tohu mate io e pā ana ki tō roro, tō taurakira, me te pūnaha io .

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He pēhea te noa o tēnei āhuatanga?

He uaua ki te kī tika e hia ngā tāngata he "Hereditary Spastic Paraplegia". Nā te mea he rite ngā tohu ki ērā o ētahi atu mate , ā, he maha ngā wā ka hē te tautuhi . E whakatau ana ngā kairangahau kei waenganui i te kotahi (1) me te rima (5) ngā tāngata i roto i ia rau mano tāngata o te ao ka pāngia e tēnei mate.

He aha ngā tohu o te (Hereditary Spastic Paraplegia)?

E rua ngā tohu matua o tēnei mate e pā ana ki ngā uaua o ō waewae:

• Te koretake o te manawa
• ngoikore

Heoi, i runga anō i te momo mate, ka puta pea ētahi atu tohu.

Ngā āhuatanga tāpiri o te momo Kāore i te uaua:

• Te ngoikore, te ngaro rānei o te rongo i ngā waewae, inā koa ko ngā kapu o ngā waewae .
• Te uaua ki te whakahaere i te mimi .
• Te hiahia ohorere ki te mimi, ki te tutae rānei, ahakoa kāore anō kia kī te pūhā, te puku rānei.

Ētahi atu āhuatanga o te momo Uaua:

I tēnei momo, he whānui rawa te whānuitanga o ngā tohu. Hei tauira:

• Te hekenga o te mahi hinengaro pēnei i te kaha ki te whakaaro me te mahara (te mate wareware) .
• He uaua ki te pupuri i te taurite me te kore e taea te whakarite i ngā mahi (ataxia).
• Ngā raruraru kanohi pērā i te tirohanga pōuri, te katarata, te ngoikore o te tirohanga, te retinopathy rānei.
• Te ngaronga o te rongo ((Te ngaronga o te rongo)) .
• Ngā uauatanga ako, te ngoikore o te hinengaro , te whakaroa rānei i te whanaketanga.
• Te mimiti haere o te papatipu uaua ((atrophy)) .
• Te kino ki ngā io o ngā ringa me ngā waewae (peripheral neuropathy) .
• Te uaua ki te manawa (dyspnea), te uaua ki te kōrero (aphasia), te uaua rānei ki te horomi (dysphagia) .
• Ngā tohu o te mate haurangi ((Hōngongoi)) .
• Tērā pea ka puta he kiri matotoru, maroke ki ētahi tāngata, he rite ki te unahi ika ((ichthyosis vulgaris)) .

Whakaarohia te uaua o te whai i tētahi, i ētahi rānei o ēnei tohumate. Nō reira, he wero nui te noho tahi me te tini o ēnei tohumate.

He aha te take o te (Hereditary Spastic Paraplegia)?

Ko te take matua mō tēnei he panonitanga o ngā ira, e kiia nei ko te momo ira . Ko ngā mahi katoa o tō tātou tinana e whakahaeretia ana e ngā tohutohu i riro mai i ngā ira. Ina huri ēnei ira, ka hē ngā tohutohu i riro mai i ngā pūmua . Kātahi ka kore e taea e aua pūmua te mahi tika i tā rātou mahi. Ka pā tika tēnei ki te mahi a ngā io i roto i tō taurakira. He maha ngā momo whakarerekētanga ira ka puta ai tēnei āhuatanga.

Ko tēnei āhuatanga, e kiia nei ko te "Hereditary Spastic Paraplegia", he mea tuku iho . Ko te tikanga me tuku iho e koe tētahi kape o te ira mōna mai i ō mātua. He maha ngā huarahi e taea ai tēnei:

• 'Autosomal dominant': Ka puta tēnei ina ko tētahi o ō mātua anake ka riro i a rātou te āhuatanga.Ka puta te mate ina kitea te ira e kawenga ana mō tēnei mate.
• 'Autosomal recessive': I tēnei āhuatanga, ka puta noa te mate mēnā ka riro i a koe te ira mai i tō whaea me tō pāpā. Mēnā ka riro i a koe mai i te mātua kotahi anake, tera pea he kaikawe koe i te mate, engari kāore e whakaatu i ngā tohu.
• 'Hono-X': I tēnei, ka tukuna e te whaea (matua wahine) tēnei ira ki tāna tamaiti tāne mā te ira ira.
• Te tuku iho i te mitochondrial: I roto i tēnei, ka tukuna e te whaea te mate ki tāna tamaiti (ahakoa te ira tangata) mā te ira mitochondrial .

Ahakoa he onge, i ētahi wā ka puta noa mai ēnei huringa ira, arā, ka puta pea i roto i tētahi tangata hou kāore he hītori whānau (pūmau) .

Ko wai te mea tino mōrearea ki tēnei mate?

Ka taea e te tangata katoa te pāngia e tēnei mate, e kiia nei ko te "Hereditary Spastic Paraplegia." Heoi, ki te mea kei tētahi, kei ngā mātua tokorua rānei te whakarerekētanga ira e pā ana ki tēnei mate, he nui ake tō tūponotanga ki te pāngia e tēnei mate .

He aha ngā raruraru pea ka pā mai i tēnei mate?

Ka taea e te Paraplegia Spastic Tuku Iho te whakaputa i ētahi atu pānga taha, tae atu ki:

• Te mamae o te tuara me ngā turi.
• Kei te mātao tonu ngā waewae.
• Te ngenge tonu ((Te ngenge)) .
• Te hurihuri me te whakapoto i te makawe.

Ko te noho me ngā uauatanga hikoi, i te wā i ngāwari ai ngā mea i mua kua uaua, i ētahi wā he morearea, ka pā nui ki tō hauora hinengaro . Ka taea e tēnei te arahi ki ngā āhuatanga pēnei i te ahotea me te pouri . Ki te mea kei te whakaaro koe he maha atu ō rā kino i ō rā pai, kaua e whakamā ki te kōrero ki tō tākuta mō tēnei mea . Ka taea e rātou te tuku i a koe ki tētahi kaitohutohu hauora hinengaro hei āwhina i a koe ki te whakahaere i te pānga o te āhuatanga ki ō kare ā-roto.

Me pēhea te tātaritanga o tēnei mate (Hereditary Spastic Paraplegia)?

Ka whakahaerehia e te tākuta he whakamātautau tinana, he whakamātautau pūnaha io, me ētahi atu whakamātautau motuhake hei tautuhi i tēnei mate. I roto i ēnei whakamātautau, ka tirohia e te tākuta ō tohu me tō hītori hauora me tō whānau. Ka rapua hoki e ia:

• Te aro ki te pā.
• Te taurite o te tinana.
• Te whakarite i ngā mahi.
• Te mahi hinengaro.
• Te mahi ā-nekehanga (te kaha ki te neke haere).
• Ngā whakaata.

I muri i ēnei mea, ki te whakapae te tākuta he (Hereditary Spastic Paraplegia), ka tūtohu pea ia i ētahi whakamātautau pēnei i:

• Te Electromyography (EMG): Ko te whakauru i ngā ngira iti ki roto i ō uaua, me te tuhi i te urupare a ō io ki ngā tohu hiko iti. Mā tēnei ka taea e koe te mārama ki te mahi a ō uaua me ō io .
• Whakamātautau ira : Ka tangohia he tauira o tō toto, o tō huware rānei hei tirotiro mō ngā whakarerekētanga ira e puta ai te mate .
• Matawai Whakaahua Āhua Aukume (MRI): Ka whakamahia e tēnei ngā ngaru aukume, ngā ngaru reo irirangi, me te rorohiko hei hanga pikitia taipitopito o ngā kiko o tō roro me tō taurakira . Ka taea e ia te whakaatu i ngā āhuatanga rerekē i roto i te roro o ētahi tāngata whai HSP matatini.
• Te pokanga tuaiwi (te weronga lumbar) : I tēnei mahi, ka werohia he ngira angiangi ki roto i tō tuara o raro, ā , ka tangohia he wai cerebrospinal (CSF) iti mai i te rohe e karapoti ana i tō roro me tō taurakira hei whakamātautau.

I ētahi wā ka roa pea te wā ki te tautuhi tika i tēnei mate , nā te mea he rite tonu ngā tohu o te (Hereditary Spastic Paraplegia) ki ngā tohu o ētahi atu mate pēnei i:

• Pararutiki roro ((Pararutiki roro))
• Te mate pukupuku maha ((Te mate pukupuku maha))
• (Te mate a Pelizaeus-Merzbacher)
• Te whakawhāititanga o te awa tuaiwi (te stenosis o te tuaiwi)

Me pēhea te rongoā i te (Hereditary Spastic Paraplegia)?

Engari, kāore he rongoā mō te Hereditary Spastic Paraplegia. Heoi anō, kei reira ngā maimoatanga hei āwhina i te whakahaere i ngā tohu me te whakangawari ake i te oranga. Ko ētahi o ēnei ko:

• Ngā Rongoā: Ngā rongoā hei whakaiti i te pakari o te uaua (ngā rongoā whakangāwari uaua), ngā werohanga paitini botulinum, me te baclofen.
• Whakaora tinana : Ngā mahi hei whakapakari i ngā uaua, hei whakanui ake i te ngāwari, hei whakapai ake hoki i te taurite.
• Whakaora mahi : Te whakauru i ngā whakangungu me ngā taputapu hei āwhina i a koe ki te mahi i ngā mahi o ia rā me te ngāwari ake.
• Ngā tautoko hu motuhake (Orthotics) : Hei whakangāwari i te hikoi me te pupuri i te tūranga tika o te waewae.
• Ngā taputapu hei āwhina i te nekenekehanga: he tokotoko, he tokotoko iti, he whitiki tautoko, he tūru wīra rānei.

I runga anō i te āhua o ō tohu, ka tūtohu pea tō tākuta i ētahi atu maimoatanga.

He aha te āhua o te heke mai mō te tangata e pāngia ana e te (Hereditary Spastic Paraplegia)?

Kei te kaha o ngā tohu te āhua o tēnei mate. E ai ki tā mātou i kōrero ai i mua ake nei, ka tere haere te whanaketanga o tēnei mate. He puhoi rawa te puta, mō ngā tau . Ka taea e tēnei te arahi ki te ngaro haere o te kaha ki te hikoi takitahi, me te hiahia hoki ki ngā āwhina nekenekehanga.

Mena kei a koe ētahi atu tohumate io, ka pā pea ki tō kounga o te oranga . Tērā pea ka hiahia te hunga whai i ēnei āhuatanga ki te āwhina me te tautoko puta noa i ō rātou oranga.

Ka whakapotoa rānei te roanga o te oranga e te Paraplegia Spastic Tuku Iho?

I te nuinga o te wā, kāore tēnei mate (Hereditary Spastic Paraplegia) e pā ki tō roanga o te oranga . Heoi, i roto i ētahi momo motuhake, uaua o tēnei mate, ka rerekē pea te āhuatanga. Ko te tangata pai hei mōhio mō tēnei ko tō tākuta. Ka taea e ia te whakamārama i ngā mea ka taea e koe te tumanako i runga i tō āhua.

He ara hei ārai i tēnei mate?

Kāore he huarahi e mōhiotia ana hei ārai i te Hereditary Spastic Paraplegia. Nā te mea he mate ira tēnei, ki te hiahia koe ki te mōhio ki tō tūponotanga ki te whakawhanake i tēnei mate me ētahi atu mate ira, ka taea e koe te kōrero ki tētahi kaitohutohu ira me te rapu mōhiohio mō ngā whakamātautau ira .

Āhea ahau me toro atu ki te tākuta?

Mena kei a koe ēnei tohu, ki te mea kei te kino haere i roto i te wā, ki te puta rānei he tohu hou, me whakamōhio atu ki tō tākuta. Ka taea e ia te whakarerekē i tō mahere maimoatanga ina hiahiatia, me te āwhina i a koe ki te whakahaere i tēnei āhuatanga e kino haere ana.

He aha ngā pātai me pātai atu e au ki taku tākuta?

Ina kitea e koe kei a koe, kei tētahi rānei e mōhio ana koe, te (Hereditary Spastic Paraplegia), tera pea he maha ngā pātai kei roto i tō hinengaro. Hei tauira:

• He aha te momo paraplegia spastic kei a au?
• He aha te momo maimoatanga e taunaki ana koe māku?
• He pānga taha kei roto i ēnei maimoatanga?
• Me whakamahi rānei ahau i te tokotoko, i te taputapu hikoi, i te tūru wīra rānei?
• He aha ngā mahi ka taea e au te mahi me te haumaru?

He mea noa te pōuri nui ina rongo koe mō tētahi mate ira pēnei. Tērā pea ka puta ake ngā pātai pēnei i te "Nā te aha ahau i pā mai ai tēnei?", "Kei roto i tōku whānau tēnei mate?", "Ka kino haere aku tohumate?". Engari kaua e manukanuka. Ka noho tahi tō tākuta me koe i tēnei haerenga, ā, ka whakautua e ia ō pātai katoa.

Ka tīmata pea ēnei tohu i ngā tau katoa, ā, ka kino haere pea i roto i te wā. I ētahi wā, ahakoa ngā mahi māmā o ia rā, pērā i te hikoi me tāu mōkai, te eke pahikara rānei, ka uaua pea. I ētahi wā, ka mōrearea pea te mahi i ēnei mea. Me ako pea koe i ngā huarahi hou ki te mahi i ngā mea e whai hua ana mō tō tinana, me whai wā anō rānei ki te mahi i ngā mea ka āwhina i te ārai i ngā aituā. Ka hoatu e tō tākuta he tohutohu kua whakaritea mōu kia noho haumaru ai, kia ora ai koe.

## Ngā Mea Hira hei Mahara (Karere hei Tango ki te Kāinga)

Āe, ko tāku tumanako kua mārama ake tō whakaaro mō te (Hereditary Spastic Paraplegia) i kōrerohia e tātou.

Ko te mea nui ko te rapu tohutohu hauora mēnā kei te whakapae koe kei a koe ēnei tohu. Mēnā ka kitea wawetia, kei reira ngā maimoatanga hei āwhina i te whakahaere i ngā tohu me te āwhina i a koe kia noho noa i te ao.

• Kia maumahara, ehara tēnei i tōu he . He mate ira tēnei.
• Ahakoa kāore e taea te whakaora katoa i te mate e te maimoatanga, ka taea tonu te whakahaere i ngā tohumate .
• Ko te whakaora tinana me te whakaora mahi he tikanga maimoatanga tino nui e rua mō te hunga e ora ana me tēnei mate.
• Kia tiakina hoki tō hauora hinengaro . Kaua e mangere ki te tono āwhina mēnā e hiahia ana koe.
• Kāore koe i te mokemoke. Kei konei ngā tākuta, ngā kaiwhakaora hinengaro, me ō whanaunga i tēnei haerenga hei āwhina i a koe.

Ko te tumanako ka whai hua ēnei mōhiohio ki a koe. Kia ora tonu!