He aha te Neurofibromatosis Momo 1? Tohutohu, Take, me te Maimoatanga

Ko te neurofibromatosis momo 1 (NF1) tētahi āhuatanga ira tuku iho e pā ana ki te tipu me te whanaketanga o ngā pūtau io puta noa i tō tinana. Ka puta mai ngā pūngāwerewere pai e kiia nei ko ngā neurofibromas i runga i ō io, ā, ka hanga hoki ngā tohu kiri motuhake e taea ai e ngā tākuta te mohio.

Tata ki te 1 o te 3,000 o te hunga e whānau ana me te NF1, ā, koinei tētahi o ngā mate ira tuku iho tino noa. Ahakoa ka rongo te ingoa he wehi, he tokomaha ngā tāngata me te NF1 e ora ana i ngā oranga tino whānuitia, mahi, me te tiaki hauora me te tirotiro tika.

He aha te Neurofibromatosis Momo 1?

Ka puta te NF1 ina he huringa kei roto i te ira NF1, e āwhina ana ki te whakahaere i te tipu o ngā pūtau. Ki te kore tenei ira e mahi tika, ka taea e ngā pūtau te tipu i ngā huarahi e kore ai e tika, ka arahina ai ngā āhuatanga motuhake o te āhuatanga.

Ka pā te āhuatanga ki tō pūnaha io, ki tō kiri, me ō wheua. Ko te nuinga o ngā tāngata me te NF1 ka whanake i ngā waahi maha o te café-au-lait (ngā tohu whānau kōwhiti-kōwhiti) me ngā pūngāwerewere iti, ngawari i raro i tō kiri e kiia nei ko ngā neurofibromas.

Kei te noho te NF1 mai i te whānau, ahakoa ka kore etahi āhuatanga e puta mai ra ano kia nui ake te tamarikitanga, te pakeketanga rānei. Ka rere ke te wheako o ia tangata me te NF1, ahakoa i roto i te whānau kotahi.

He aha ngā tohu o te Neurofibromatosis Momo 1?

Ka rere ke ngā tohu o te NF1 mai i te tangata ki te tangata, engari he maha ngā tohu noa e titiro ana ngā tākuta. Kia haere tātou i ngā mea ka kite pea koe, mā te tīmata ki ngā āhuatanga tino noa.

Ngā tohu noa e whai ake nei:

• Ngā waahi Café-au-lait - ngā tohu whānau kōwhiti-kōwhiti, papatahi, e noho ana mai i te whānau
• Neurofibromas - ngā pūngāwerewere ngawari, kikorangi, e tipu ana i runga i ngā io i raro i te kiri
• Te pupuhi i ngā wāhi kore noa penei i ngā raro o ngā ringa, te kōpū, te raro rānei o ngā u
• Ngā pūngāwerewere Lisch - ngā pūngāwerewere iti, kore kino kei te wāhanga tae o ō kanohi
• Ngā rerekētanga ako, ngā wero aroha rānei
• Te rahi o te upoko nui atu i te noa
• Te poto o te teitei ki te whakataurite ki ngā mema whānau

Ka tipu haere ngā āhuatanga nei i te wa. Ko ngā waahi café-au-lait te nuinga o te wā e puta tuatahi ana, i mua rānei o te whānau, ka puta mai ngā neurofibromas i ngā tau whakangākau, i muri rānei.

Ngā tohu iti ake engari nui e whai ake nei:

• Ngā huringa wheua, te whanaketanga wheua rānei
• Te toto teitei i te tamarikitanga
• Ngā hopu, ngā mamae o te mahunga rānei
• Ngā raruraru tirohanga, ngā āhuatanga kanohi rānei
• Te scoliosis (te tuawhe o te tuawhenua)
• Ngā whakaroa kōrero, ngā raruraru rānei

Ka wheako hoki etahi tāngata i ngā wero pāpori me ngā wero kare-a-roto e pā ana ki ngā rerekētanga e kitea ana, ki ngā raruraru ako rānei. He tino mārama te āwangawanga mō ēnei huringa, ā, kei te wātea te tautoko.

He aha ngā take o te Neurofibromatosis Momo 1?

Ka puta te NF1 i ngā huringa (ngā tauira) i roto i te ira NF1, e mahi ana hei pēhi i te tipu o ngā pūtau. Ki te kore tenei ira e mahi tika, ka taea e ngā pūtau te tipu me te whakaputa ina me mutu.

Tata ki te haurua o ngā tāngata me te NF1 ka whiwhi i te āhuatanga i te mātua kei a rātou hoki. Ki te whai tētahi mātua i te NF1, ka whai te tamaiti i te 50% o te tupono ki te whiwhi i te āhuatanga - rite tonu ki te tō te moni.

Ko tetahi atu haurua o ngā tāngata me te NF1 ko te tuatahi rātou i roto i tō rātou whānau ki te whai. Ka puta mai tenei ina puta mai tētahi huringa ira hou, he mea tino rongoa, ā, kāore i puta mai i tetahi mea i mahi, kāore rānei i mahi ngā mātua.

Kia whai koe i te NF1, ka whai koe mō te ora, ka taea hoki e koe te tuku ki ō tamariki. Heoi, ko te whai i te huringa ira kāore e whakatau i te kaha o te āhuatanga ki a koe.

Nōhea te haere ki te tākuta mō te Neurofibromatosis Momo 1?

Me whakaaro koe ki te haere ki te tākuta ki te kite koe i ngā waahi maha o te café-au-lait i runga i a koe, i tō tamaiti rānei, ā, ina koa ki te ono atu rānei ngā waahi nui atu i te rahi o te rapa pene. Ko ēnei tohu motuhake te nuinga o te wā ko te tohu tuatahi e titiro ana ngā tākuta.

He mea nui hoki te rapu i te tiaki hauora ki te whanake koe i ngā pūngāwerewere hou i raro i tō kiri, te wheako i ngā huringa tirohanga, te kite rānei i ngā raruraru ako e kore e rite ki tō whānau. Ka taea e te tautuhi wawe te awhina i te whakahaere me te tirotiro pai ake.

Rapua te tiaki hauora tonu ki te wheako koe i:

• Ngā mamae o te mahunga nui, ngā huringa rānei i ngā tauira mamae o te mahunga
• Te ngoikore hou, te ngawari rānei i ngā ringa, i ngā waewae rānei
• Te tipu tere o ngā neurofibromas kei te noho
• Ngā huringa tirohanga, te mamae kanohi rānei
• Ngā hopu, ngā ngāwari rānei
• Ngā tohu toto teitei nui

Kaua e tatari ki te he te mea, te rerekē rānei. Ahakoa ko te nuinga o ngā tohu o te NF1 ka tipu haere, me whai ngā āhuatanga ki te aro nui kia kaua e puta ngā raruraru nui ake.

He aha ngā take morearea mō te Neurofibromatosis Momo 1?

Ko te take morearea matua mō te NF1 ko te whai i tētahi mātua me te āhuatanga. Ki te whai tētahi o ō mātua i te NF1, ka whai koe i te 50% o te tupono ki te whiwhi, ahakoa tō ira tangata, tō whakaritenga whānau rānei.

Heoi, ehara te hītori whānau i te kairāhui i nga wa katoa. Ko te haurua o ngā tāngata katoa me te NF1 ko te tuatahi rātou i roto i tō rātou whānau ki te whai, nā ngā huringa ira hou ka taea te puta ki te tangata katoa.

Kāore he āhuatanga noho ora, ngā whakaū taiao, ngā whāinga whaiaro rānei e whakapiki ana i tō morearea ki te whanake i te NF1. Ka puta te āhuatanga rite tonu puta noa i ngā rōpū iwi katoa, ā, ka pā rite tonu ki ngā tane me ngā wāhine.

Ko te pakeketanga o ngā mātua (inā koa ngā pāpā neke atu i te 40) ka piki haere pea te morearea o ngā huringa ira hou, engari kei te ako tonu tēnei hononga, ā, kei te iti tonu te morearea whānui.

He aha ngā āhuatanga pea o te Neurofibromatosis Momo 1?

Ahakoa he tokomaha ngā tāngata me te NF1 e ora ana i ngā oranga hauora, he mea nui te mārama ki ngā āhuatanga pea kia taea ai e koe te mahi tahi me tō rōpū hauora ki te tirotiro me te whakahaere pai. Ko te nuinga o ngā āhuatanga ka taea te whakahaere ina kitea wawe.

Ngā āhuatanga tino noa e whai ake nei:

• Ngā rerekētanga ako e pā ana ki te 50-60% o ngā tāngata me te NF1
• Te toto teitei, ka taea te whanake i tetahi tau
• Ngā raruraru wheua penei i te scoliosis, te tipu wheua rānei
• Ngā raruraru tirohanga tae atu ki te kanohi mangere, te glaucoma rānei
• Ngā whakaroa kōrero me te reo i ngā tamariki
• Ngā wero pāpori me ngā wero kare-a-roto e pā ana ki te ahua

Ka tipu haere ngā āhuatanga nei, ā, ka taea te whakahaere me te tiaki me te tautoko tika. Ka awhina te tirotiro auau ki te hopu i ngā raruraru wawe ina tino whai hua te maimoatanga.

Ngā āhuatanga iti ake engari nui e whai ake nei:

• Ngā pūngāwerewere pūtau io taha (te mate pukupuku i roto i ngā neurofibromas)
• Ngā gliomas ara tirohanga (ngā pūngāwerewere e pā ana ki ngā io tirohanga)
• Te pheochromocytoma (ngā pūngāwerewere e pā ana ki te toto teitei)
• Ngā pūngāwerewere stromal gastrointestinal
• Ngā āhuatanga wheua nui e pā ana ki te mahi
• Te mate Moyamoya (ngā raruraru oko toto i roto i te roro)

Ahakoa he iti ngā āhuatanga nui nei, e pā ana ki te iti iho i te 10% o ngā tāngata me te NF1, ka awhina te tirotiro hauora auau ki te kite wawe i a rātou. Ka mōhio tō rōpū hauora ki ngā tohu hei titiro, me te wā pea e hiahiatia ana ngā whakamātautau atu.

Me pēhea te tautuhi i te Neurofibromatosis Momo 1?

Ko te tautuhi i te NF1 te nuinga o te wā e whakauru ana i te mohio ki te tauira o ngā āhuatanga motuhake, kaua ki te whakawhirinaki ki tētahi whakamātautau kotahi. Ka whakamahi ngā tākuta i ngā paerewa kua whakatūria e titiro ana ki ngā tohu maha o te āhuatanga e puta tahi ana.

Me kimi e tō tākuta te iti rawa o ēnei āhuatanga e rua hei mahi i te tautuhi: ono atu rānei ngā waahi café-au-lait, rua atu rānei ngā neurofibromas, te pupuhi i ngā wāhi kore noa, ngā pūngāwerewere Lisch i roto i ngā kanohi, ngā pūngāwerewere ara tirohanga, ngā huringa wheua motuhake, tētahi whānau tata rānei me te NF1.

Ko te tukanga tautuhi te nuinga o te wā e whai ake nei:

• Te tirotiro tinana taipitopito o tō kiri me tō tinana
• Te tirotiro kanohi hei titiro ki ngā pūngāwerewere Lisch, ngā raruraru tirohanga rānei
• Te arotake hītori whānau hei kimi i ngā whānau tata me ngā āhuatanga rite
• Te aromatawai whanaketanga me te ako, inā koa i ngā tamariki
• Te tirotiro toto teitei
• Te whakamātautau ira ki te kore te tautuhi e mārama

I etahi wā, ka hiahiatia ngā whakamātautau atu penei i ngā tātaritanga MRI, ngā whakamātautau kanohi motuhake rānei hei tirotiro i ngā pūngāwerewere o roto, ētahi atu āhuatanga rānei. Ka whakamārama tō tākuta i ngā whakamātautau e hiahiatia ana mō tō āhuatanga motuhake.

Ka taea e te whakamātautau ira te whakau i te tautuhi, engari ehara i te mea e hiahiatia ana i nga wa katoa ki te mārama ngā āhuatanga hauora. Ka titiro te whakamātautau ki ngā huringa i roto i te ira NF1, ā, ka taea te awhina i ngā whakatau mahere whānau.

He aha te maimoatanga mō te Neurofibromatosis Momo 1?

Ahakoa kāore he rongoā mō te NF1, he maha ngā maimoatanga ka taea te awhina i te whakahaere i ngā tohu me te aukati i ngā āhuatanga. Ko te whāinga ko te awhina i a koe ki te ora i te tino whānuitia me te mārama i te wa e tirotiro ana i ngā huringa e hiahia ana ki te aro.

He tino motuhake te maimoatanga, nā te mea ka pā te NF1 ki te tangata katoa. Ka hanga e tō rōpū hauora tētahi mahere e hāngai ana ki ō tohu me ō hiahia motuhake.

Ngā huarahi maimoatanga noa e whai ake nei:

• Ngā hui tirotiro auau hei titiro ki ngā whanaketanga hou
• Te tautoko mātauranga me ngā whakaritenga ako
• Te whakahaere toto teitei ki te hiahiatia
• Te rongoā tinana mō ngā raruraru wheua, uaua rānei
• Te rongoā kōrero mō ngā raruraru korerorero
• Te tautoko tohutohu mō ngā wero kare-a-roto me ngā wero pāpori

Mō ngā neurofibromas e pā ana ki ngā raruraru, ka taea te tūtohutia te tango wawe. Heoi, ko te nuinga o ngā neurofibromas kāore e hiahia ki te maimoatanga ki te kore e pā ana ki te mamae, ki ngā raruraru mahi, ki ngā āwangawanga ataahua rānei.

Ngā maimoatanga motuhake pea e whai ake nei:

• Ngā rongoā e whai take ana mō ētahi momo pūngāwerewere
• Te pokanga orthopēdic mō ngā raruraru wheua nui
• Te rongoā radiation i ngā keehi onge (e whakamahia ana me te tupato)
• Te rongoā tirohanga, te pokanga rānei mō ngā raruraru kanohi
• Te whakahaere rongoā mō ngā hopu, ētahi atu take neurological rānei

Kei te rangahau tonu ngā maimoatanga hou. Kei te whakamātau ngā whakamātautau hauora i ngā rongoā ka taea te awhina i te whakaiti i te tipu o ngā pūngāwerewere, te whakapai ake rānei i ngā raruraru ako e hono ana ki te NF1.

Me pēhea te whakahaere i te Neurofibromatosis Momo 1 i te kāinga?

Ko te ora pai me te NF1 e whakauru ana i te hanga i ngā mahi e tautoko ana i tō hauora whānui i te wa e noho ana i ngā huringa pea e hiahia ana ki te aro hauora. Ka taea e ngā tikanga iti o ia rā te whakarereke nui ki te āhua o tō wheako.

Kia mau ki te rēhita ngāwari o ētahi pūngāwerewere kiri hou, ngā huringa rānei e kitea ana e koe. Tangohia ngā whakaahua ki te awhina, ā, tuhia te wā i puta mai ai. Ka awhina ēnei korero i tō tākuta ki te aroturuki i te whanaketanga o te āhuatanga.

Ngā rautaki whakahaere o ia rā e whai ake nei:

• Te tiaki i tō kiri i te ra nui
• Te pupuri i te kai hauora me te mahi tinana auau
• Te whakahaere i te ahotea mā ngā tikanga whakahaere, ngā mahi whakangahau rānei
• Te whiwhi i te moe tika hei tautoko i te hauora whānui
• Te tango i ngā rongoā kua tohua tonu
• Te noho hono atu ki ngā hoa tautoko me ngā whānau

Mō ngā tamariki me te NF1, mahi tata atu ki ngā kaiako hei whakarite i ngā tautoko ako tika. He maha ngā tamariki e whai hua ana i ngā mahere mātauranga motuhake e whakautu ana ki ō rātou momo ako me ō rātou hiahia motuhake.

Whakaarohia te hono atu ki ngā rōpū tautoko NF1, i te tangata rānei, i te ipurangi rānei. Ko te kōrero ki ētahi atu e mārama ana ki te āhuatanga ka taea te whakarato i ngā tohutohu mahi me te tautoko kare-a-roto.

Kia mau ki ngā korero hauora nui e wātea ana, tae atu ki tō tautuhi, ngā rongoā o nāianei, me ngā korero whakapā āwhina mō tō rōpū hauora.

Me pēhea te whakarite mō tō hui tākuta?

Ko te whakarite mō ō hui NF1 e awhina ana i te whakarite kia whiwhi koe i te tino hua mai i tō wā me tō rōpū hauora. Ka taea e te whakahaere iti i mua te mahi i te haerenga kia nui ake te hua, kia iti ake te ahotea.

Tuhia ētahi tohu hou, ngā huringa rānei kua kitea e koe mai i tō haerenga whakamutunga. Whakauruhia te wā i tīmata ai, te āhua o te huringa, me te mea he aha te mea e pai ake ai, te kino ake rānei.

Mauria ēnei mea ki tō hui:

• Rārangi o ngā rongoā me ngā taapiri o nāianei
• Ngā whakaahua o ētahi pūngāwerewere kiri hou, ngā huringa rānei
• Ngā tuhinga mō ngā tohu, ngā āwangawanga rānei
• Ngā pātai e hiahia ana koe ki te ui ki tō tākuta
• Ngā kāri inihua me te whakamōhiotanga
• Ngā hua whakamātautau o mua ki te kite i tētahi tākuta hou

Whakaritea ngā pātai motuhake mō tō āhuatanga. Hei tauira, uihia te wā me whakarite ai ō whakamātautau matawai e whai ake nei, ngā tohu me whai wāhi tonu te tiaki hauora, ngā mahi rānei me karo.

Ki te whai koe i ngā tamariki me te NF1, mau mai ngā ripoata kura, ngā tirohanga kaiako rānei mō ngā huringa ako, āhua rānei. Ka awhina ēnei korero i ngā tākuta ki te mārama ki te āhua o te āhuatanga e pā ana ki te ora o ia rā.

Whakaarohia te kawe mai i tētahi mema whānau, hoa rānei hei tautoko, inā koa ki te kōrero koe i ngā whakatau maimoatanga, te whiwhi rānei i ngā korero hou mō tō āhuatanga.

He aha te mea nui mō te Neurofibromatosis Momo 1?

Ko te NF1 tētahi āhuatanga ira tuku iho ka taea te whakahaere e pā ana ki te tangata katoa. Ahakoa e hiahia ana ki te aro hauora tonu, ka taea e te nuinga o ngā tāngata me te NF1 te ora i ngā oranga tino whānuitia, hua me te tiaki me te tautoko tika.

Ko te ki mō te ora pai me te NF1 ko te noho hono atu ki ngā kaiwhakarato hauora mohio e mārama ana ki te āhuatanga. Ka awhina te tirotiro auau ki te hopu i ngā āhuatanga pea wawe ina tino taea te maimoatanga.

Kia mahara ko te whai i te NF1 kāore e whakatau i tō kaha, te whakatau rānei i te mea ka taea e koe te whakatutuki. He tokomaha ngā tāngata me te NF1 e tino pai ana i ō rātou mahi, hononga, me ō rātou whāinga whaiaro i te wa e whakahaere pai ana i tō rātou āhuatanga.

Kei te haere tonu te rangahau ki te whakapai ake i tō tātou mārama ki te NF1 me te whanake i ngā maimoatanga pai ake. Kei te haere tonu te tirohanga mō ngā tāngata me te NF1 i te wa e haere whakamua ana te matauranga hauora.

Ngā pātai e ui pinepinetia ana mō te Neurofibromatosis Momo 1

P1: Ka taea e ngā neurofibromas te riro hei mate pukupuku?

Ko te nuinga o ngā neurofibromas ka noho pai (kāore i te mate pukupuku) puta noa i te ora. Heoi, he iti te morearea ka taea te whanake hei pūngāwerewere pūtau io taha, e pā ana ki te 8-13% o ngā tāngata me te NF1. Ngā tohu hei titiro atu: te tipu tere, te mamae hou, ngā huringa rānei i te āhua o ngā neurofibromas kei te noho.

P2: Ka whiwhi rānei ō tamariki i te NF1 ki te whai ahau?

Ka whai ia tamaiti i te 50% o te tupono ki te whiwhi i te NF1 ki te whai tētahi mātua i te āhuatanga. Koinei te morearea rite mō ia haputanga, ahakoa te maha o ō tamariki, tō ira tangata rānei. Ka taea e te tohutohu ira te awhina i a koe ki te mārama ki tō āhuatanga whānau me ō kōwhiringa motuhake.

P3: Ka taea e ngā tohu NF1 te kino ake i te wa?

Ko te NF1 te nuinga o te wā he āhuatanga e haere tonu ana, arā, ka taea te whanake i ngā tohu hou puta noa i te ora. Heoi, ka rere ke te tere me te nui o te whanaketanga i waenga i ngā tangata. Ka wheako etahi i ngā huringa iti, ka whanake etahi atu i ngā āhuatanga hou i te wa. Ka awhina te tirotiro auau ki te aroturuki i ngā huringa.

P4: He whakatau kai rānei kei te NF1?

Kāore he whakatau kai motuhake mō te NF1. Heoi, ko te pupuri i te kai hauora, te kai rite tonu e tautoko ana i te oranga pai, ā, ka awhina pea i te whakahaere i ngā āhuatanga e hono ana penei i te toto teitei. Ka kite etahi i te pānga o ētahi kai ki ō rātou taumata kaha, āhua rānei, engari ka rere ke tenei i te tangata ki te tangata.

P5: Ka taea e ngā tāngata me te NF1 te whakauru ki ngā hākinakina me ngā mahi tinana?

Ko te nuinga o ngā tāngata me te NF1 ka taea te whakauru ki ngā mahi tinana auau me ngā hākinakina. Heoi, me whakaaro pea ki ngā hākinakina hono ki te whai koe i ngā neurofibromas maha, i te mea ka taea e te pakaru te puta mai i ngā raruraru. Kōrerohia ngā kōwhiringa mahi ki tō tākuta hei whakarite kia tika mō tō āhuatanga motuhake.