He aha te Adrenoleukodystrophy? Ngā Tohutohu, Ngā Take, me te Maimoatanga

Ko te Adrenoleukodystrophy (ALD) he mate tuku iho onge e pā ana ki te pūnaha io me ngā whatukuhu adrenal. Ka puta mai tēnei mate tuku iho ina kore e taea e tō tinana te wawahi tika i ētahi ngako e kiia nei ko ngā waikawa ngako roa-raupapa, ka hua ai te kohikohi me te whakangaro i ngā wāhanga nui o tō tinana. Ahakoa ka rerekē te pānga o te ALD ki ngā tāngata rerekē, ko te mōhiotanga ki tēnei mate ka āhei te awhina i a koe ki te kite i ngā tohu i te wā, me te rapu i ngā whiringa maimoatanga ka pēnei pea te whakaheke i tōna ahu whakamua.

He aha te Adrenoleukodystrophy?

Ko te take o te Adrenoleukodystrophy ko ngā huringa i roto i te ira e kiia nei ko te ABCD1, e āwhina ana i te tikanga ki tō tinana ki te tukatuka i ngā momo ngako motuhake. Ki te kore tenei ira e mahi tika, ka kohikohi ngā mea ngako ki roto i tō roro, i tō tuaiwi o muri, me ngā whatukuhu adrenal.

Ko te ingoa o te mate i ngā wāhanga e pā ana rawa atu. Ko te 'Adreno' e tohu ana ki ngā whatukuhu adrenal e noho ana i runga i ō whatukuhu, ko te 'leuko' e tohu ana ki te mea ma i roto i te roro, ā, ko te 'dystrophy' e whakaatu ana ki te whakangaro e puta ana i te wā.

He mate X-linked te ALD, ko te tikanga kei runga i te karōmēra X te ira e whakahaere ana. Ka whakamārama tēnei tauira tuku i te take he nui atu te pānga o te mate ki ngā tane i ngā wāhine, nā te mea kotahi noa te karōmēra X a ngā tane, e rua ia ngā wāhine.

He aha ngā momo Adrenoleukodystrophy?

Ka puta mai te ALD i roto i ngā momo rerekē, ia momo he raupapa me te kaha ake. Ko te momo pea ka whanake koe e whakawhirinaki ana ki tō tau i te wā i puta mai ai ngā tohu, me ngā wāhanga o tō tinana e pā ana rawa atu.

Ko te ALD roro tamariki te momo tino kino, e tīmata ana i te nuinga i waenga i ngā tau 4 me te 10. Ka tere te ahu whakamua o tēnei momo, ā, ka pā ki te mea ma o te roro, ka hua ai ngā raruraru neurological nui. I te tīmatanga, ka ātaahua pea ngā tamaiti e pangia ana e tēnei momo i mua i te tīmatanga o ngā tohu.

Ko te Adrenomyeloneuropathy (AMN) te nuinga o te wā ka whanake i te pakeketanga, i te tekau tau rua tekau rānei, i te toru tekau rānei. Ko tēnei momo ka tino piki haere te tere, ā, ko te nuinga o te pānga ki te rōpū o te tuaiwi me ngā pūnaha o te taha.

Ko te mate Addison-anake te pānga ki ngā whatukuhu adrenal anake, kāore e uru ki te pūnaha io. Ko te hunga e pangia ana e tēnei momo pea ka pāngia e te kore o ngā homoni, engari kāore e whanake i ngā tohu neurological e kitea ana i ētahi atu momo.

Ko ētahi tāngata ka noho hei kaipupuri asymptomatic, inā te hunga wāhine, nō te mea pea kāore rātou e whanake i ngā tohu e kitea ana, engari ka taea te tuku i te āhuatanga ki ō rātou tamariki.

He aha ngā tohu o te Adrenoleukodystrophy?

Ka rereke te ahua o ngā tohu o te ALD, kei te whakawhirinaki ki te momo kei a koe me tō tau i te wā i puta tuatahi ai rātou. Ko te mohiotanga wawe o ēnei tohu ka taea te whakarereke nui ki te whiwhi i te tiaki me te tautoko tika.

I te ALD roro o te tamarikitanga, pea ka kite tuatahi koe i ngā huringa i roto i te whāinga, i te mahi kura rānei. Ka taea e ēnei tohu wawe te ngāwari, ā, ka uru pea ki:

• Te uaua ki te aro atu, ki te whai whakaaro rānei i te kura
• Ngā huringa i te tuhituhi, i te whakahaere rānei
• Ngā raruraru ki te tirohanga, ki te whakarongo rānei
• Te nui o te hyperactivity, i ngā take whāinga rānei
• Te uaua ki te mārama, ki te whai i ngā tohutohu rānei

I te whakamāha o te ALD roro o te tamarikitanga, ka whanake pea ngā tohu neurological nui ake. Ka uru pea ēnei ki ngā hopu, te uaua ki te horomia, te ngaro o te kōrero, me ngā raruraru ki te nekehanga me te taurite.

Ko te AMN e puta ana i te pakeketanga ka puta mai me ngā tohu rerekē e whanake haere ana i te wā. Pea ka pāngia koe e:

• Te ngoikore me te ngoikore o ō waewae
• Te uaua ki te hikoi, ki te pupuri rānei i te taurite
• Ngā raruraru ki te mana o te pūkahukahu, o te whekau rānei
• Te ngenge, te wiri rānei o ō ringa me ō waewae
• Te ngoikoretanga o te taangata

Ka puta mai ngā tohu o te korenga o te homoni adrenal i roto i tetahi momo o te ALD, ā, koinei pea ngā tohu tuatahi ka kite koe. Kei roto i ēnei ngā ngenge roa, te ngaro o te taumaha, te pōuri o tō kiri, te iti o te toto, me te hiahia ki ngā kai tote.

Ko ngā wāhine e mau ana i te ira ALD ka whanake pea i ngā tohu ngawari i muri mai o te ora, e pā ana ki te koretake o te waewae, ki ngā huringa neurological iti rānei, ahakoa he tokomaha tonu ka kore rawa he tohu.

He aha te take o te Adrenoleukodystrophy?

Ko te ALD te take o ngā rerekētanga i roto i te ira ABCD1, e whakarato ana i ngā tohutohu mō te hanga i te pūmua e āwhina ana ki te kawe i ngā waikawa ngako ki roto i ngā hanganga pūtau e kiia nei ko ngā peroxisomes. Ki te kore tenei ira e mahi tika, ka kore e taea e tō tinana te wawahi i ngā waikawa ngako roa-tāpiri pai.

Katahi ka kohikohi ēnei waikawa ngako ki roto i ngā kiko maha puta noa i tō tinana, inā te mea nui ki te mea ma te pūtake o te roro me ngā whatukuhu adrenal. Whakaarohia he pūnaha whakahoki mai kua pakaru – ngā hua paru me whakahaere me tango atu, engari ka hanga ake ka pā te kino.

Ka riro te āhuatanga i roto i te tauira X-linked recessive. Ko te tikanga kei runga i te karōme X te ira, ā, mai i te mea kotahi noa te karōme X kei ngā tāne, ka nui ake te pānga ki a rātou ki te whiwhi i te ira kua rerekē.

E rua ngā karōme X kei ngā wāhine, no reira ka hiahia rātou ki ngā rerekētanga i roto i ngā kape e rua kia pā kino. Heoi, ko ngā wāhine e kotahi te kape kua rerekē he kaimau, ā, ka whanake pea i ngā tohu ngawari i muri mai o te ora nā te tukanga e kiia nei ko te X-inactivation.

Ka rereke te kaha me te momo o te ALD ahakoa i roto i te whānau kotahi, e whakaatu ana pea ko ētahi atu take ira rānei taiao pea ka pā ki te whanaketanga me te ahu whakamua o te āhuatanga.

Nō hea te titiro ki te taote mō te Adrenoleukodystrophy?

Me rapu koe i te tiaki hauora ki te kite koe i ngā tohu neurological roa, i ngā tohu rānei o te korenga o te homoni adrenal, inā he hītori whānau koe o te ALD. Ka taea e te aromatawai wawe te awhina i te tautuhi i te āhuatanga i mua i te ahu whakamua nui.

Mō ngā tamariki, whakapiri atu ki tō tākuta tamariki mena ka kite koe i ngā huringa whāinga kore i roto i te whāinga, te heke o te mahi kura, ngā raruraru tirohanga rānei, ngā raru whakarongo rānei, te raruraru whakahaere rānei. Ahakoa ka tīmata te ahua o ēnei tohu kaore e hono, engari ka taea e rātou te tohu i tētahi āhuatanga neurological kei raro i te mata.

Me kite ngā pakeke ki te taakuta mō te ngoikore o te waewae e haere tonu ana, ngā raruraru hikoi, ngā raruraru whakahaere pūkau rānei, pūkahukahu rānei, te tohu rānei o te ngoikore adrenal penei i te ngenge kaore i te whakamārama me te ngaro o te taumaha. Kaua e tatari kia kaha rawa ngā tohu i mua i te rapu awhina.

Mēnā kei te whakaaro koe ki te whānau tamariki, ā, he hītori whānau o te ALD, ka taea e te tohutohu ira te āwhina i a koe ki te mārama ki ō tūponotanga me ō kōwhiringa. Kei te wātea te whakamātautau hei whakatau mena kei a koe te tauira o te ira.

Me kōrero ngā wāhine e mohiotia ana he kaimau ki ngā kōwhiringa aroturuki me ō rātou kaiwhakarato hauora, ahakoa kei te pai tonu te hauora, nā te mea ka puta mai ngā tohu i muri mai i te ora.

He aha ngā take tūponotanga mō te Adrenoleukodystrophy?

Ko te take tūponotanga matua mō te whanaketanga o te ALD ko te whai i te hītori whānau o te āhuatanga, nā te mea he mate ira tuku iho. Ka taea e te mārama ki te hītori hauora o tō whānau te awhina i te tautuhi i ngā tūponotanga pea i te tīmatanga.

Ko te mea he tane ka nui ake te pikinga o tō tūponotanga ki te whanaketanga o ngā momo ALD kino na te tauira tuku iho X-linked. Ko ngā tane e whiwhi ana i te ira kua whakarerekētia ka tata tonu ki te whanaketanga o tētahi momo o te āhuatanga, ahakoa ka rerekē te kaha me te wā.

Ko te whai i tētahi whaea e mau ana i te ira ALD ka whakatūpono koe, nā te mea ka taea e ngā whaea te tuku i te āhuatanga ki ō rātou tamariki. He 50% te tūponotanga o ia tamaiti o tētahi whaea kaimau ki te whiwhi i te tauira ira kua whakarerekētia.

Ka taea e te tau te awe i te momo ALD ka whanakehia e koe. Ko te ALD roro tamariki ka puta i waenga i ngā tau 4 me te 10, i te wā ka puta mai te AMN i te pakeketanga. Heoi, ehara ēnei tauira i te mea tino tika, ā, ka puta mai ngā tohu i waho o ēnei tawhā tau noa i etahi wā.

Ko ētahi momo rerekētanga ira tino ongekainga pea ka pā ki te āhua o te pānga o te ALD ki a koe, ahakoa kei te ako tonu ngā kairangahau ki ēnei take. Kāohore anō kia kitea ngā take taiao, nō reira ko te whakawhiwhi ira te take tūponotanga nui rawa e mōhiotia ana.

He aha ngā raruraru pea o te Adrenoleukodystrophy?

Ka taea e te ALD te arahi ki ngā raruraru nui e pā ana ki ngā pūnaha tinana maha, ahakoa ko ngā raruraru motuhake pea ka pa ki a koe e whakahaeretia ana e te momo ALD kei a koe me te tere o tōna whanake. Ka taea e te mōhiotanga ki ēnei wāhi te āwhina i a koe ki te whakarite mō te tiaki whānui.

Ko ngā raruraru neurological te mea tino awangawanga pea o te ALD. I roto i te ALD roro tamariki, ka uru atu ēnei ki:

• Te ngaro haere o ngā kaha hinengaro me te mahara
• Ngā hopu e uaua pea te whakahaere
• Te ngaro o te tirohanga me te rongohanga
• Te uaua ki te horomi, ka taea te arahi ki ngā raruraru kai
• Te ngaro katoa o te mahi o ngā uaua me te nekehanga

Ko ēnei raruraru nui te nuinga ka whanake tere i roto i te ALD roro tamariki, i roto i ngā marama ki ētahi tau, nō reira he mea nui te whakauru wawe.

I roto i te AMN, ka puta haere te whanake o ngā raruraru, engari ka taea tonu te pā kino ki tō kounga ora. Ka pā pea ki a koe te uaua haere ki te hikoi, ka hiahiatia pea ngā taputapu nekehanga, te wīra rererangi rānei. Ka taea e te mahi kore o te pūkahukahu me te kopu te pā ki ngā mahi o ia rā, me te hiahia kia whakahaere tonutia.

Ko te kore o te adrenal he raruraru nui ka taea te puta i roto i tetahi momo ALD. Me kore te rongoā whakakapinga homoni tika, ka taea e tēnei te arahi ki te mate ngākau adrenal, e tohuhia ana e te iti rawa o te toto, te maroke, me te pakaru.

He mea nui hoki te whakaaro ki ngā raruraru hinengaro me te whānau. Ko te whakahaere i te mate neurology e ahu whakamua ana ka āhei te arahi ki te pouri, te manukanuka, me te motu whānau mo ngā tūmanako me ngā whānau. Ko ēnei wero ā-hinengaro he whakautu noa i te tika ki te aro me te tautoko.

I etahi wā, ka puta mai etahi atu raruraru ki ētahi o te hunga e pangia ana e te ALD, pēnei i ngā tohu hinengaro, i ngā whakautu mumura i roto i te roro, ahakoa he iti ake te nui o ēnei i ngā pānga neurology me te homoni matua.

Me pēhea te whakatau i te Adrenoleukodystrophy?

Ko te whakatau i te ALD te timatanga mā te kite i ngā tohu me te mārama ki tō hītori whānau, ka whai ake ngā whakamātautau toto motuhake me ngā rangahau whakaahua. Ka tīmata pea tō tākuta ki te hītori hauora taipitopito me te whakamātautau tinana hei aromatawai i te mahi neurology me te rapu i ngā tohu o ngā raruraru adrenal.

Ko te whakamātautau tuatahi tino nui te ine i ngā waikawa ngako roa-rengarenga i roto i tō toto. Ko te nui o ēnei mea ka tohu kaha ki te ALD, nā te mea kaore e taea e tō tinana te wawahi tika i a rātou ina kore e mahi tika te kiripuaki ABCD1.

Ka taea e te whakamātautau ira te whakau i te whakatau mā te kimi i ngā rerekētanga i roto i te kiripuaki ABCD1. He mea nui tenei whakamātautau mō ngā mema whānau e hiahia ana ki te mohio mēnā kei a rātou te kiripuaki, ahakoa kāore anō kia puta mai ngā tohu.

Ka āhei ngā tirohanga MRI o te roro ki te āwhina i ngā tākuta ki te kite i ngā huringa i roto i te pūtake ma e tohu ana i te ALD. Ka taea e ēnei whakaahua te whakaatu i te nui o te whakauru o te roro me te āwhina ki te whakatau i te momo ALD pea kei a koe. Ka taea hoki e te tauira o ngā huringa i runga i te MRI te whai wāhi ki te kī he pēhea te ahu whakamua o te āhuatanga.

Ka tirotirohia ngā whakamātautau mahi adrenal i ngā taumata homoni hei whakatau mēnā kei te mahi tika ngā whatukuhu adrenal. Ka uru pea ēnei whakamātautau ki te ine i te cortisol, ACTH, me ētahi atu homoni e tohu ana i te hauora adrenal.

Ngā whakamātautau atu pea ka uru ngā rangahau arahi ihu hei aromatawai i te mahi o ngā pūnaha ihu, ina koa ki te whai koe i ngā tohu o te AMN. Ka taea hoki e tō tākuta te tūtohutia ngā whakamātautau aroturuki tūturu hei aroturuki i te whānuitanga o te āhuatanga i te wā.

He aha te rongoā mō te Adrenoleukodystrophy?

Ko te rongoā mō te ALD e arotahi ana ki te whakahaere i ngā tohu, te whakaheke i te whānuitanga ina taea, me te pupuri i te kounga o te ora. Ahakoa kāore ano he rongoā, he maha ngā huarahi ka taea te awhina i a koe ki te ora pai ake me tēnei āhuatanga, ā, pea ka whakaheke i tōna whānuitanga.

He mea nui te rongoā whakakapinga homoni ki te whai koe i te korenga o te adrenal. Ko te tango i te cortisol hangai, ā, i etahi wā i ētahi atu homoni, ka taea te whakakapi pai i te mea kaore e taea e ō whatukuhu adrenal te whakaputa. Ko tēnei rongoā he oranga te nuinga, engari ka taea te whakapai ake nui i tō kaha, i tō hiahia kai, me tō oranga whānui.

Mō te ALD roro tamariki, ko te whakawhiti pūtau tūāhuwhenua hematopoietic (whakawhiti wheua wheua) pea he kōwhiringa i ngā wā tīmatanga. Ka taea pea e tēnei rongoā te aukati i te whānuitanga o te mate roro, ahakoa he nui ngā tūponotanga, me te hiahia kia arotakehia kia tika ai koe he kaitono pai.

I whakaarohia te hinu a Lorenzo, he whakaranu o ngā waikawa ngako motuhake, hei awhina i te ALD, engari kua whakaatuhia e te rangahau he iti ngā painga mō te nuinga o te iwi. Ka korero pea etahi tākuta i te huarahi tautoko, ahakoa kaore e whakaarohia he rongoā matua.

Ko te rongoā ira he rongoā e puta mai ana e whakaatu ana i te tumanako i roto i ngā whakamātautau hauora. Ko tēnei huarahi e uru ana ki te whakauru i te kape mahi o te ira ABCD1 ki roto i ō pūtau, pea ka taea te tukatuka i ngā waikawa ngako ki te noho noa.

Ko te tiaki tautoko he mahi nui ki te whakahaere i ngā tohu ALD. Ka uru pea tenei ki te rongoā tinana hei pupuri i te nekehanga, te rongoā mahi hei whakarite i ngā mahi o ia rā, te rongoā kōrero mō ngā raruraru whakawhitiwhiti korero, me te tautoko kai ki te uaua te horomi.

Ka taea e ngā rongoā te āwhina i te whakahaere i ngā tohu motuhake pēnei i ngā hopu, te ngohe o ngā uaua, te mamae rānei. Ka mahi tahi tō rōpū hauora ki a koe ki te kimi i te huinga tika o ngā maimoatanga mō tō āhuatanga motuhake.

Me pēhea te whakahaere i te Adrenoleukodystrophy i te kāinga?

Ko te whakahaere i te ALD i te kāinga kei roto te hanga i te taiao tautoko e rite ana ki ngā hiahia e whakarerekē ana, me te pupuri tonu i te tino motuhake me te whakamarie e taea ana. Ka whakawhirinaki tō huarahi ki ngā tohu e pā ana ki a koe me te āhua o te ahuatanga.

Mēnā kei te tango koe i te rongoā whakakapinga homoni mō te korenga o te adrenal, he mea nui te mau tonu. Tangohia ō rongoā kia rite ki te whakahau, ā, mau tonu te hydrocortisone āwhina. Ako ki te mohio ki ngā tohu o te mate pūngao adrenal, ā, kia mōhio ki te wā hei whakamahi i ngā rongoā āwhina, te rapu rongoā tūturu rānei.

Ko te noho mahi tinana i roto i ō kaha ka taea te āwhina i te pupuri i te kaha me te nekehanga. Mahi tahi me te kaiwhakaora tinana ki te whakawhanake i te kaupapa whakakori tinana e tika ana mō tō āhuatanga. Ahakoa ngā mahi ngawari pēnei i te whakatete rānei i ngā whakakorikori wai ka whai hua.

Ka nui haere te hiranga o te ora o te kāinga i te wā e whakarerekē ana te nekehanga. Whakaarohia te whakauru i ngā paepae hopu i roto i ngā ruma kaukau, te tango i ngā tūponotanga tū, me te whakarite i te mārama pai puta noa i tō kāinga. Ka taea e ngā kaiwhakaora mahi te tuku i ngā whakarerekētanga motuhake ka taea te āwhina i a koe ki te arahi i ngā mahi o ia rā mā te ngāwari.

Ka hiahiatia pea te tautoko kai mēnā ka uaua te horomi. Mahi tahi me te kaiwhakaako kai ki te whakarite kei te whiwhi koe i te kai tika, me te ako mo ngā whakarerekētanga o te kakano, ngā huarahi whāngai rerekē rānei mēnā e hiahiatia ana. He mea nui rawa te noho makuku pai mēnā he korenga adrenal tōu.

Ko te tautoko hinengaro he mea nui ano te tiaki tinana. Hononga atu ki ngā rōpū tautoko, ahakoa te kanohi ki te kanohi, te ipurangi rānei, kei reira e taea ai te whakawhiti wheako ki ētahi atu e mōhio ana ki te mea e tūtaki ana koe.

Kaua e whakaroa ki te rapu tohutohu mena kei te whawhai koe ki ngā āhuatanga hinengaro o te noho tahi me te ALD.

Tūturu te tuhi i ngā tohu o ou mate, rongoā, me ētahi huringa e kitea ana e koe. Ka āwhina tēnei mōhiohio i tō rōpū hauora ki te whakatau whakamārama mō tō tiaki, ā, ka nui te uara mō te aroturuki i te whanake o te mate.

Me pēhea te whakarite mō tō whakatau ki te taote?

Ko te whakarite pai mō ō whakatau ki te taote ka āwhina i a koe ki te whakamahi pai i tō wā tahi, ā, kia whakarite ai koe e whiwhi ana i ngā mōhiohio me te tiaki e hiahia ana koe. Tīmatahia mā te tuhituhi i ō pūtaiao me ō awangawanga katoa i mua i te haerenga, kia kaua ai e wareware i tetahi mea nui i te whakatau.

Pupuri i te puka tohu mō ngā wiki torutoru i mua i tō whakatau, mā te tuhi i ngā huringa i tō āhua, ngā tohu hou, ngā awangawanga rānei mō ō rongoā o nāianei. Whakauruhia ngā kōrero mō te wā e puta mai ai ngā tohu, te kaha o rātou, me te aha e āwhina ana, e whakaiti ana rānei i a rātou.

Mauria he rārangi katoa o ngā rongoā katoa e inu ana koe, tae atu ki ngā horopeta, ngā taapiri, me ngā rongoā kore-whakahaunga. Mena kei te inu koe i te hydrocortisone tūāwhiorangi, kia mohio tō taote ki ngā wāhanga hou i te wā i hiahia ai koe ki te whakamahi.

Kohikohia ngā rekoata hauora, ngā hua whakamātautau, ngā ripoata rānei mai i ētahi atu kaiwhakarato hauora kua kite koe mai i tō whakatau whakamutunga. Mena kua oti i a koe ngā rangahau whakaahua, te mahi toto rānei i ētahi atu wāhi, mau mai ngā kape, kia taea ai e tō taote te uru atu ki ngā hua.

Whakaarohia te kawe mai i te mema whānau, hoa rānei ki te whakatau, ina koa ki te kōrero koe mō ngā whakatau whakaora uaua, ki te raruraru rānei te mahara, te aro turuki rānei e whakaheke ana i te pupuri i ngā mōhiohio. Ka taea e rātou te tautoko i a koe me te mahara ki ngā kōrero nui mai i te kōrero.

Whakamahia ngā pātai motuhake e pā ana ki tō mate, pēnei i te āhua o te whakawhānuitanga, ngā tohu hou hei titiro atu, me te āhua o te mahi a ō rongoā o nāianei. Kaua e wehi ki te tono whakamārama ki te kore e mōhiotia e koe tetahi mea i whakamāramahia e tō tākua.

He aha te mea nui hei tango mō te Adrenoleukodystrophy?

Ko te mea nui rawa atu hei mārama mō te ALD, ahakoa he mate tuku iho nui, ko te taumata wawe me te rongoā tika ka pā nui ki te kounga o tō oranga, ā, ka taea te whakaheke i te whakawhānuitanga. He rerekē te wheako o ia tangata ki te ALD, ā, ko te whai i tēnei mate kāhore e tikanga te whakarere i te tumanako mō te oranga whai kiko.

Ko te ngoikore o te adrenal, e taea te puta mai me tetahi momo ALD, ka rongoātia katoatia ki te rongoā whakakapinga homoni. Ko te whakahaere tika i tēnei āhuatanga o te mate ka āwhina i a koe kia pai ake te ahua, ā, kia karo i ngā raruraru nui.

Mō ngā whānau e pā ana ki te ALD, ka taea e te tohutohu ira me te whakamātautau te whakarato i ngā korero whai hua mō te whakatōpūtanga whānau me te kite wawe i ētahi atu mema o te whānau. Ka taea e te mōhiotanga mō te tūāhua kawe te āwhina i ngā wāhine ki te whakatau whakamārama mō te tirotiro i ō rātou hauora.

Kei te haere tonu te rangahau ki ngā rongoā hou mō te ALD, me te rongoā ira e whakaatu ana i te tumanako motuhake i ngā whakamātautau hauora hou. Ko te noho hono atu ki ngā pokapū rongoā ALD motuhake ka taea te āwhina i a koe ki te uru atu ki ngā whakawhanaketanga hou me ngā whakamātautau hauora.

Ko te hanga i te whatunga tautoko kaha, tae atu ki ngā kaiwhakarato hauora e mārama ana ki te ALD, ngā whānau tautoko me ngā hoa, me ngā hononga ki ētahi atu whānau e pā ana ki te mate, ka taea te whakarereke nui i te whakahaere i ngā wero e whakaturia ana e te ALD.

Ngā pātai e uiui pinepinetia ana e pā ana ki te Adrenoleukodystrophy

Q.1: Ka taea e ngā wāhine te whanake i ngā tohu o te ALD?

Ae, ka taea e ngā wāhine e mau ana i te ira ALD te whanake i ngā tohu, ahakoa he ngawari ake te tikanga i te mea e pā ana ki ngā tāne. E 20% pea o ngā wahine kaikawe e whanake ana i ētahi tohu, te nuinga o te wā ko te ngoikore o ngā waewae, te raruraru hoki ki te hikoi e puta mai ana i muri mai o te ora, i muri i te 40 tau te nuinga. Ka tino puhoi te ahu whakamua o ēnei tohu, ā, kaore e tino kino penei i ngā raruraru neurological e kitea ana i ngā tāne e pangia ana e te ALD.

P.2: He rongoā rā kei te ALD?

I tēnei wā, kāore he rongoā mō te ALD, engari ka taea e ngā maimoatanga te awhina i te whakahaere i ngā tohu, ā, ka taea pea te whakaheke i te ahu whakamua. Ka taea e te whakawhiti iwi wheua te aukati i te ahu whakamua o te mate roro i ētahi tamariki kei te tīmatanga o te ALD o te roro, ahakoa he nui ngā tūponotanga. Kei te whakaatu te rongoā ira i te tumanako i roto i ngā whakamātautau hauora, ā, ka waiho pea hei whiringa maimoatanga nui i te wā kei te heke mai.

P.3: He aha te rerekētanga o te ALD i ētahi atu āhuatanga neurological ira?

He motuhake te ALD nā te mea ka pā ki te pūnaha io me ngā whatukuhu adrenal e whakaputa ana i ngā homoni, nā te raruraru o te tukatuka i ngā momo ngako motuhake. Kāore i rite ki te tini o ngā āhuatanga neurological, ko te waahanga adrenal o te ALD ka taea te whakaora katoa me te rongoā whakakapinga homoni. Ko te tauira whakawhiti X-linked e whakakāhore ana i te tini atu o ngā mate neurological ira.

P.4: Ka taea e ngā huringa kai te awhina i te ALD?

Ahakoa i whakaarohia i mua ko te hinu a Lorenzo he pai, kua whakaatuhia e te rangahau he iti ngā pānga mō te nuinga o te iwi e pangia ana e te ALD. I tēnei wā, kāore he kai motuhake kua whakamāramahia hei pānga nui ki te ahu whakamua o te ALD. Heoi, he mea nui te pupuri i te kai pai whānui, ina koa ki te pēhea te raruraru o te horomi, kei te tango rongoā whakakapinga homoni rānei.

P.5: He aha te roa o te ora mō te tangata e pangia ana e te ALD?

He rereke te roa o te oranga e whakamahia ana i runga i te momo ALD kei a koe. Ko te hunga e pangia ana e te mate Addison anake, te AMN ngawari ranei, ka taea te roa o te oranga noa me te rongoa tika. Ko te hunga e pangia ana e te AMN e puea haere ana i te wa roa, ka iti pea te roa o te oranga, engari ka taea e ratou te ora mo nga tau maha me te kounga o te ora pai. Ko te ALD o te roro tamariki ka tere ake te puea, ahakoa ka taea e te whakauru wawe me te whakawhiti toto wheua te whakapai ake i nga hua.