He aha te Pegvaliase: Ngā Whakamahinga, te Inenga, ngā Pānga taha, me ētahi atu mea

Ko te Pegvaliase he rongoā whakakapi enzyme motuhake i hangaia mō ngā pakeke e mate ana i te phenylketonuria (PKU), he mate ira onge e kore ai te tinana e kaha ki te wawahi i te waikawa amino phenylalanine. E mahi ana tēnei rongoā mā te whakarato i te enzyme ngaro e hiahiatia ana e tō tinana hei tukatuka tika i te phenylalanine, e āwhina ana ki te whakaheke i ngā taumata mōrearea i roto i tō toto.

Mēnā kei a koe, kei tētahi rānei e manaaki ana koe te PKU, tērā pea ka pōuri koe i ngā kōwhiringa maimoatanga. He tohu nui te Pegvaliase mō ngā tāngata kāore anō i tutuki i ngā taumata phenylalanine e whāia ana mā te kai anake, e tuku ana i te tūmanako mō te whakahaere pai ake i te mate me te kounga o te oranga.

He aha te Pegvaliase?

Ko te Pegvaliase he putanga i hangaia e te tangata o tētahi enzyme e kīia ana ko te phenylalanine ammonia lyase e ngaro ana i tō tinana mēnā he PKU tōu. Whakaarohia he whakarato i tō tinana ki te taputapu ngaro e hiahiatia ana e ia hei wawahi i te phenylalanine mai i ngā pūmua e kainga ana e koe.

Ka tae mai tēnei rongoā hei wai mārama e werohia ana e koe i raro i tō kiri, pērā i te whakamahi a te hunga mate huka i te insulin. Ko te ingoa waitohu mō te pegvaliase ko Palynziq, ā, i hangaia motuhake mō ngā pakeke 18 tau, neke atu rānei, e mate ana i te PKU.

Ko te mea motuhake o te pegvaliase i ahu mai i tētahi pūtake huakita, ā, i hangaia mā te āta tūpato kia mahi haumaru i roto i te tinana o te tangata. Ka whakawhiti te rongoā i te purenga nui kia mōhio ai ka tutuki i ngā paerewa haumaru tino kaha i mua i te tae atu ki ngā tūroro.

He aha te Pegvaliase e whakamahia ana?

Ka tohua te Pegvaliase mō ngā pakeke anake e mate ana i te phenylketonuria (PKU) e noho tiketike tonu ana ngā taumata phenylalanine toto ahakoa te whai i te kai iti-pūmua. Ko te PKU he mate ira e kore ai tō tinana e kaha ki te wawahi tika i te phenylalanine, he waikawa amino kei roto i te maha o ngā kai kei roto i te pūmua.

Inā ka piki te phenylalanine i roto i tō toto, ka pā ngā raruraru hauora nui, tae atu ki te hauatanga hinengaro, ngā pūhi, ngā raruraru whanonga, me ētahi atu raruraru neurological. Ko te hunga e mate ana i te PKU te tikanga ka whakahaere i tō rātou āhuatanga mā te kai tino whāiti e whakawhāiti ana i te kai pūmua, engari kāore tēnei huarahi e mahi mō te katoa.

Ka tūtohu pea tō tākuta i te pegvaliase mēnā ka noho tonu ō taumata phenylalanine i runga ake i te 600 micromol/L (tata ki te 10 mg/dL) ahakoa te kaha o tō mahi ki te whakahaere i te kai. Ko te whāinga he whakaheke i ēnei taumata ki tētahi awhe haumaru ake, ko te mea pai i raro i te 360 micromol/L (6 mg/dL).

Me pēhea te mahi a Pegvaliase?

Ka mahi a Pegvaliase mā te whakarato i tō tinana ki te enzyme e ngaro ana i a ia hei wawahi i te phenylalanine. I roto i te hunga kāore i te PKU, ka huri tētahi enzyme e kiia nei ko te phenylalanine hydroxylase i te phenylalanine ki tētahi atu waikawa amino e kiia nei ko te tyrosine. Ki te mate koe i te PKU, kāore tēnei enzyme e mahi tika ana.

Ka whakamahi a Pegvaliase i tētahi huarahi rerekē mā te whakamahi i tētahi enzyme e kiia nei ko te phenylalanine ammonia lyase, e wawahi ana i te phenylalanine mā tētahi ara matū rerekē. Ka āwhina tēnei ara kē ki te whakaiti i te nui o te phenylalanine e huri ana i roto i tō toto, ahakoa ka kai koe i ngā kai kei roto te pūmua.

E kiia ana ko te rongoā he rongoā kaha me te whai hua mō te PKU i te wā e kore ai te whakahaere kai anake e ranea. Heoi, he maha ngā marama ka kite i ngā pānga katoa, ā, me whai whakamātautau toto tonu koe hei aroturuki i ō taumata phenylalanine me te whakatika i tō inenga kia rite.

Me pēhea taku tango i te Pegvaliase?

Ka tukuna te Pegvaliase hei werohanga subcutaneous, ko te tikanga ka werohia e koe ki roto i te kiko momona i raro noa iho i tō kiri. Ka ako tō rōpū hauora i a koe me pēhea te tuku i ēnei werohanga ki a koe i te kāinga, i runga i te haumaru me te whakamarie.

Ko ngā wāhi werohanga e tino kitea ana ko tō huha, tō ringa o runga, tō puku rānei. Me huri ngā wāhi werohanga hei ārai i te riri o te kiri, me te karo i te werohanga ki te wāhi kotahi tonu. Horoi i te wāhi werohanga ki te waipiro i mua i ia werohanga, me te whakamahi i te ngira hou i ia wā.

Ka taea e koe te tango i te pegvaliase me te kore kai rānei, ā, kāore he here kai motuhake e pā ana ki te wā e werohia ai e koe te rongoā. Heoi anō, me haere tonu koe ki te whai i tō kai PKU kua whakaritea e te rōpū hauora, ahakoa e whakamahi ana koe i te pegvaliase.

Whakaorangia ngā pounamu kāore anō i whakatuwheratia i roto i te pouaka whakamātao i waenga i te 36°F ki te 46°F (2°C ki te 8°C). Kaua e whakatio te rongoā, kaua hoki e rūrū kaha te pounamu. I mua i te werohanga, tukua te rongoā kia tae ki te pāmahana rūma mō te 15-30 meneti hei whakangāwari i te werohanga.

Kia Pēhea te Roa e Tango Ana ahau i te Pegvaliase?

Ko te pegvaliase te tikanga he rongoā wā-roa, ā, ka hiahia pea koe ki te haere tonu mō te roa hei pupuri i ngā taumata phenylalanine iti. I te mea he āhuatanga ira tūturu te PKU, ko te whakamutu i te rongoā ka piki anō pea ō taumata phenylalanine.

Ka tīmata tō tākuta i a koe ki te horopeta iti, ā, ka piki haere i roto i ngā marama maha i runga i te pai o te urupare me te manawanui o tō tinana ki te rongoā. Ko tēnei tukanga, e kiia ana ko te titration horopeta, ka roa pea te 6-12 marama, neke atu rānei, kia tae ki tō horopeta pai.

Mā ngā whakamātautau toto auau e āwhina tō rōpū hauora ki te aroturuki i ō taumata phenylalanine me te whakatau mēnā e hiahiatia ana ngā whakatikatika horopeta. Ko ētahi tāngata ka hiahia pea ki ngā huringa horopeta i te wā i runga i ngā āhuatanga pēnei i ngā huringa i roto i te kai, te taumaha, te urupare rānei o tō tinana ki te rongoā.

Kaua rawa e mutu te tango i te pegvaliase ohorere me te kore kōrero ki tō tākuta i te tuatahi. Mēnā e hiahia ana koe ki te whakamutu i te rongoā mō ētahi take, ka āwhina tō rōpū hauora i a koe ki te whakawhanake i tētahi mahere hei whakahaere i tō PKU mā ētahi atu tikanga.

He Aha ngā Pānga Taha o te Pegvaliase?

Pērā i ngā rongoā katoa, ka pānga ngā pānga taha ki te pegvaliase, ahakoa kāore e pā ana ki te katoa. Ko ngā pānga taha tino noa e pā ana ki ngā uruparenga wāhi werohanga me te uruparenga ārai mate o tō tinana ki te rongoā.

Mā te māramatanga ki ngā mea e tūmanakohia ana ka āwhina koe ki te pūmau me te māia ki te tīmata i tēnei maimoatanga. Anei ngā pānga taha ka pā ki a koe, kua whakarōpūhia mai i te mea tino noa ki te mea iti noa:

Ngā pānga taha noa (e pā ana ki te neke atu i te 10% o te tāngata):

• Ngā uruparenga wāhi werohanga pērā i te whero, te pūpū, te mamae, te pānga rānei
• Te mamae tahi, te mārō rānei
• Ngā uruparenga pānga mate mai i ngā pūhihi kiri ngawari ki ngā tohumate nui ake
• Ngā mamae mahunga
• Te ngenge, te āhua rānei o te ngenge
• Te whakapairuaki, te pōraruraru puku rānei
• Te mamae uaua
• Te kirika, ngā tohumate rānei pērā i te rewharewha

Ngā pānga taha iti noa engari pea he nui:

• Ngā uruparenga pānga mate nui (anaphylaxis) e hiahia ana ki te āwhina hauora tonu
• Ngā uruparenga kiri nui tae atu ki te pūhihi, te mōkai, te kiri rānei e tihore ana
• Te uaua ki te hā, te whio rānei
• Te pūpū o te mata, ngā ngutu, te arero, te korokoro rānei
• Te mamae tahi nui, te pūpū rānei
• Te kirika tonu, ngā tohu rānei o te mate

Ngā raruraru onge engari he nui:

• Ngā uruparenga ārai mate e whakaeke ana te pūnaha ārai mate o tō tinana i te kiko hauora
• Ngā uruparenga wāhi werohanga nui kāore e ora tika ana
• Ngā huringa i roto i ngā tatau pūtau toto
• Ngā huringa mahi ate

Ko te nuinga o ngā uruparenga wāhi werohanga he ngawari, ka pai ake i roto i ngā rā torutoru. Heoi, ki te pā ana ki a koe ngā uruparenga pānga mate nui, te uaua ki te hā, ētahi tohumate rānei e āwangawanga ana koe, me rapu tonu koe i te āwhina hauora.

Ko wai kāore e tika ana kia tango i te Pegvaliase?

Kāore te Pegvaliase e tika ana mō te katoa e mate ana i te PKU. Ka arotake tō tākuta mēnā he tika tēnei rongoā mōu i runga i ētahi mea nui.

Kaua koe e tango i te pegvaliase mēnā he mōhiotia he pānga kino ki te rongoā, ki ētahi o ōna kai. Kei roto i tēnei ko ngā pānga kino o mua ki te pegvaliase, ki ngā rongoā whakakapi enzyme rānei.

Me whai whakaaro motuhake ki ētahi āhuatanga hauora me ngā āhuatanga i mua i te tīmatanga o te pegvaliase:

Ngā āhuatanga e hiahia ana kia āta arotakehia:

• Te mate hāora nui, ētahi atu āhuatanga rānei e pā ana ki te manawa
• He hītori o ngā pānga kino ki ngā rongoā, ki ngā kai rānei
• Ngā mate ārai-a-tinana e kaha ana
• Te mate ate, te mate tākihi rānei
• Ngā mate e kaha ana, te pūnaha ārai-a-tinana rānei kua ngoikore
• Ngā mate toto, te tango rongoā whakaheke toto rānei

Ngā rōpū motuhake e hiahia ana kia tūpato atu:

• Ngā wāhine hapū, ngā wāhine e whāngai ana i ngā pēpi (he iti noa ngā raraunga haumaru e wātea ana)
• Ngā tāngata e whakamahere ana i te pokanga, i ngā tikanga niho rānei
• Te hunga e tango rongoā ārai-a-tinana
• Ngā tāngata he hītori o ngā pānga kino ki ngā rongoā

He take anō te tau, nā te mea kua whakaaetia te pegvaliase mō ngā pakeke 18 tau, neke atu rānei. Me haere tonu ngā tamariki me ngā taiohi e mate ana i te PKU ki te whakahaere kai me ētahi atu rongoā kua whakaaetia kia tae rā anō rātou ki te pakeketanga.

Ingoa Waitohu Pegvaliase

Ko te ingoa waitohu mō te pegvaliase ko Palynziq, i hangaia e BioMarin Pharmaceutical. Koinei anake te waitohu o te pegvaliase e wātea ana i te United States me ētahi atu whenua.

Ka tae mai a Palynziq i roto i ngā pounamu kotahi-te-whakamahi kei roto ngā momo rerekē o te rongoā. Māu e whakahau te tākuta i te kukume me te horopeta e tika ana mō ō hiahia takitahi.

I te mea he rongoā motuhake te pegvaliase mō tētahi āhuatanga onge, ka wātea noa iho mā ngā whare rongoā motuhake he wheako ki te whakahaere i ngā rongoā whakakapi enzyme. Ka āwhina tō rōpū hauora ki te whakarite kia whiwhi koe i tō rongoā mā ngā ara tika.

Ngā Whakakapinga Pegvaliase

Ahakoa ko te pegvaliase anake te rongoā whakakapi enzyme e wātea ana mō te PKU, he ētahi atu huarahi rongoā ka whakaarohia e tō tākuta i runga i tō āhuatanga me ō hiahia.

Ko te huarahi kē ki te pegvaliase ko te whakahaere kai tino kaha, e whai ana i te kai iti-pūmua me te whakamahi i ngā kai rongoā motuhake i hangaia mō te hunga e PKU ana. He pai te mahi a tēnei huarahi mō te tokomaha, engari e hiahia ana kia nui ngā huringa o te noho me te aroturuki tupato.

Ko ētahi atu kōwhiringa rongoā ka whakaarohia ko:

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• Sapropterin (Kuvan) - he rongoā ka āwhina i ētahi tāngata me ētahi momo PKU ki te tukatuka pai ake i te phenylalanine
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• Ngā tāpiringa waikawa amino kūpapa nui ka āwhina i te whakaiti i te ngongo phenylalanine
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• Ngā kai rongoā i runga i te glycomacropeptide e whakarato ana i te pūmua me te iti o te phenylalanine
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• Ngā rongoā whakamātau e akohia ana i ngā whakamātau haumanu
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Ka mahi tō rōpū hauora ki a koe ki te whakatau i te huinga pai rawa atu o ngā rongoā i runga i ō taumata phenylalanine, ō hiahia kai, te noho, me te pai o tō urupare ki ngā huarahi rerekē.

He Pai Ake Te Pegvaliase I Te Sapropterin?

He rerekē te mahi a Pegvaliase me te sapropterin (Kuvan) me te whakamahia pinepine mō ngā momo tāngata PKU rerekē, nō reira ehara i te mea tika te whakataurite i a rāua. Ko te kōwhiringa

Ko Pegvaliase, i tērā taha, e mahi ana mō te nuinga o te hunga whai PKU ahakoa te momo ira, engari me werohia, ā, he nui ake te tūponotanga o ngā tauhohenga pānga. Ko te tikanga ka whakaarohia ina kāore te whakahaere kai me te sapropterin i tutuki i ngā taumata phenylalanine e whāia ana.

Ka whai hua pea ētahi tāngata mā te whakamahi i ngā rongoā e rua ngātahi i raro i te tirotiro hauora. Māu e tākuta e āwhina ki te whakatau ko tēhea huarahi te mea tika mō tō āhuatanga motuhake i runga i te whakamātautau ira, ngā taumata phenylalanine o nāianei, me ō whāinga maimoatanga.

Ngā Pātai Auau mō Pegvaliase

He Haumaru a Pegvaliase mō te Hapūtanga?

Kāore anō kia tino whakapūmautia te haumarutanga o te pegvaliase i te wā o te hapūtanga mā ngā rangahau haumanu nui. Heoi anō, he mea nui te pupuri i ngā taumata phenylalanine tika i te wā o te hapūtanga mō te hauora o te whaea me te whanaketanga o te pēpi.

Ko ngā taumata phenylalanine tiketike i te wā o te hapūtanga ka pā mai ngā hē whānau nui tae atu ki te hauatanga hinengaro, ngā raruraru o te ngākau, me ngā raruraru tipu i roto i te pēpi e whanake ana. Ko tēnei āhuatanga e kīia ana ko te mate PKU o te whaea, ā, ka pā mai ahakoa kāore te pēpi i te PKU.

Mēnā kei te hapū koe, kei te whakamahere rānei koe kia hapū i te wā e tango ana koe i te pegvaliase, mahi tahi me tō tohunga PKU me tō tākuta whānau. Mā rāua e āwhina ki te whakatau i ngā tūponotanga me ngā painga o te haere tonu i te maimoatanga ki te huri ki te whakahaere kai tino kaha i te wā o te hapūtanga.

Me Pēhea Taku Mahi Mēnā Ka Whakamahi Ahau i te Nui Rawa o te Pegvaliase?

Mēnā ka werohia e koe te nui rawa o te pegvaliase i te mea i whakaritea, whakapā atu ki tō kaiwhakarato hauora tonu, waea atu rānei ki tō pokapū whakahaere paitini. Kaua e tatari kia kite mena ka puta ngā tohu, he mea nui te aro hauora tere mō ngā rongoā nui rawa.

Ahakoa te nui rawa o te tango i te pegvaliase e kore e puta he pānga e mate ai te ora, ka piki ake pea te tūponotanga o ngā pānga taha pēnei i ngā tauhohenga nui i te wāhi werohanga, i ngā tauhohenga mate pāwera rānei. Ka hiahia pea tō tākuta ki te mātakitaki i a koe i te tata atu, ki te whakatika rānei i tō hōtaka inenga ā muri ake nei.

Hei aukati i ngā horopeta nui ohorere, me mātua taki-rua i tō horopeta kua whakaritea i mua i te werohanga, whakamahia te kukū tika o te rongoā, me te pupuri i tētahi rākau inenga hei aroturuki i te wā i tango ai koe i tō rongoā. Ki te kore koe e mōhio ana ki tō horopeta, whakapā atu ki tō rōpū hauora i mua i te werohanga.

Me Pēhea Taku Mahi Ki Te Ngaro Au i Tētahi Horopeta o te Pegvaliase?

Ki te ngaro koe i tētahi horopeta o te pegvaliase, tangohia i te wā tonu ka mahara koe, ki te kore e tata ana te wā mō tō horopeta e whai ake nei. I taua wā, pekehia te horopeta i ngaro, ka haere tonu ki tō hōtaka inenga auau.

Kaua rawa e tango i te horopeta rua hei utu mō tētahi i ngaro, nā te mea ka piki ake pea tō tūponotanga o ngā pānga taha. Ko te ngaro i tētahi horopeta i ētahi wā e kore e puta he raruraru tonu, engari ngana ki te pupuri i tō hōtaka auau i te mea ka taea mō ngā hua pai rawa atu.

Ki te wareware tonu koe i ngā horopeta, whakaarohia te whakatakoto i ngā whakamaharatanga waea, te whakamahi i tētahi kaiwhakarite rongoā, te tono rānei ki ngā mema o te whānau kia āwhina i a koe ki te whakamahara. He mea nui te inenga rite tonu mō te pupuri i ngā taumata phenylalanine pūmau.

Āhea E Taea Ai e Au te Kati te Tango i te Pegvaliase?

Kaua rawa koe e mutu te tango i te pegvaliase me te kore e kōrero tuatahi ki tō rōpū hauora. I te mea he āhuatanga mō te ora katoa te PKU, ko te whakamutu i te pegvaliase ka piki ake pea ō taumata phenylalanine anō, ka puta pea he raruraru hauora.

Tera pea ētahi āhuatanga e tūtohu ana tō tākuta kia whakamutua te pegvaliase mō tētahi wā, pēnei i te wā o te mate nui, i mua i te pokanga, ki te whakawhanake rānei koe i ngā pānga taha nui. I ēnei wā, ka āwhina tō rōpū hauora i a koe ki te whakahaere i tō PKU mā ētahi atu tikanga i te wā o te okiokinga maimoatanga.

Mēnā kei te whakaaro koe ki te whakamutu i te pegvaliase nā te pānga taha, ētahi atu āwangawanga rānei, kōrerohia ō wheako ki tō tākuta. Ka taea e rātou te whakatika i tō horopeta, te whakarato tautoko anō mō te whakahaere i ngā pānga taha, te tūhura rānei i ētahi atu whiringa maimoatanga.

Ka taea e au te haere i te wā e tango ana i te Pegvaliase?

Āe, ka taea e koe te haere i te wā e tango ana i te pegvaliase, engari me whakamahere kia whai rongoā nui koe, me te pupuri i ngā tikanga rokiroki tika. Me mau tonu he rongoā anō mō ngā whakaroa haerenga, ngā āhuatanga ohorere rānei.

I te wā e rere ana, whakauruhia tō pegvaliase ki tō pōkai-a-ringa me tētahi reta whakahaunga mai i tō tākuta e whakamārama ana i tō hiahia rongoā me ngā taputapu werohanga. Mā tēnei e ārai ai i ngā raruraru ki te haumarutanga o te taunga rererangi, me te whakarite kia kore tō rongoā e ngaro i ngā pōkai kua tirotirohia.

Mō ngā haerenga o te ao, rangahaua ngā ture mō te kawe rongoā whakahaunga ki tō whenua haerenga. Ko ētahi whenua e hiahia ana i ngā tuhinga motuhake, he here rānei mō ētahi rongoā, nā reira whakapā atu ki tō rātou karere, ki te kāhui rānei i mua kia kore ai he raruraru.