Mena kua kite ohorere koe i ngā wāhi mārama i runga i te kiri o tāu tamaiti, i te mea rānei kei te pāngia koe e ngā mate haurangi kāore i te whakamāramatia, he mea maori te āwangawanga. He tohu ēnei i ētahi wā mō tētahi mate e kiia nei ko te Tuberous Sclerosis Complex (TSC), kāore e kōrerohia whānuitia ana. Tuatahi, kaua e mataku. Mā te mārama ki tēnei mate, ka taea e koe te whakahaere angitu. Me matapaki tātou i ngā mea katoa e hiahia ana koe ki te mōhio i roto i ngā kupu māmā.
He aha te Matatini Tuberous Sclerosis (TSC)?
Whakaarohia ngā pūtau o tō tinana he kaimahi pukumahi. Ka wehea, ka tipu ina hiahiatia, kātahi ka mutu ina oti tā rātou mahi. Heoi, i roto i te tangata whai TSC, he hapa iti kei roto i te tukanga e whakahaere ana i te wehenga pūtau. Nō reira, kāore ngā pūtau e mutu te wehenga i te wā e tika ana.
Ina wehea ngā pūtau me te kore e taea te whakahaere, ka hua ake te tipu o ngā pukupuku i ngā wāhanga rerekē o te tinana, pērā i te roro, ngā whatukuhu , te ngākau , ngā pukahukahu , te kiri, me ngā kanohi. Ko te mea nui me mōhio koe, ehara ēnei pukupuku i te matepukupuku . Heoi, ka taea e rātou te whakaputa raruraru rerekē i runga i te wāhi e tipu ai. E miriona ngā tāngata huri noa i te ao e noho ana me tēnei mate. Waimarie, he pai ngā maimoatanga hou e wātea ana i ēnei rā hei whakahaere i ngā tohu me te whakaiti i ēnei tipu.
He aha te tino take o te TSC?
Ko te take matua he whakarerekētanga ira . Ka puta tēnei i roto i tētahi o ngā huarahi e rua:
1. Ngā whakarerekētanga ira tūpono: Mō te nuinga o te tangata (tata ki te rua hautoru o ngā take), kāore te TSC i te tuku iho. Ka puta noa i ngā wāhanga tōmua o te whanaketanga ina honoa ngā pūtau o te whaea me te pāpā ki te hanga i tētahi kukune.
2. I tukuna iho i ngā mātua: I roto i te tata ki te hautoru o ngā take, ka tukuna iho te mate mai i tētahi mātua. Mena he TSC tō tētahi mātua, e 50% te tūponotanga ka riro i tā rāua tamaiti.
He aha ngā tohu? He rite tonu te pānga ki te katoa?
Kāo. He rerekē rawa ngā tohumate o te TSC i ia tangata, i ia tangata, ā, ka rerekē pea i roto i te wā. Ka kitea ngā tohu i ētahi tāngata i te whānautanga, engari ka pāngia pea ētahi e ngā tohu i muri mai i te oranga. Ka whakawhirinaki ngā tohumate ki te wāhi kei reira ngā pukupuku, tōna rahi, me tōna maha.
Hei āwhina i a koe ki te mārama, me titiro tātou ki tōna pānga ki ngā wāhanga rerekē o te tinana.
| Wāhanga Tinana e Pāngia ana | Ngā Tohu pea |
|---|---|
| Kiri | Koinei ngā tohu tino kitea. Tērā pea ka kite koe i ngā wāhi mārama (ngā macule hypopigmented), ngā puku whero rite ki te hakihaki i runga i te mata (facial angiofibromas), ngā wāhi kiri matotoru i te rae, ngā tupu iti rānei i te taha o ngā maikuku. |
| Roro | He maha ngā wā ka arahi ngā pukupuku i roto i te roro ki ngā hopukangaTērā pea ka puta ngā huringa whanonga (te riri pinepine, te manukanuka), ngā raruraru moe, te ānini, te whakapairuaki, me ngā whakaroa whanaketanga pērā i te autism. |
| Ngā whatukuhu | Ka taea e ngā pukupuku i roto i ngā whatukuhu te whakararu i tā rātou mahi. Ka arahi pea tēnei ki te toto toto tiketike, ki te toto rānei o roto. |
| Ngākau | I te nuinga o te wā ka puta mai ngā pukupuku o te ngākau i te wā pēpi, ā, ka mimiti haere i te pakeketanga. Heoi anō, i ētahi wā ka puta he koretake o te manawa o te ngākau (arrhythmias) ka aukati rānei i te rere o te toto. |
| Ngā pūkahukahu | Ahakoa he iti noa te whakakorikori tinana, ka taea e ngā pukupuku i roto i ngā pukahukahu te whakapoto i te manawa. Ka taea te maremare, te hinga rānei o te pukahukahu i ngā wā kino rawa atu. |
| Ngā kanohi | Ka taea e ngā pukupuku kanohi te arahi ki te tirohanga pōuri, ki te tirohanga takirua rānei. |
Me pēhea te tautuhi a ngā tākuta i tēnei mate?
Nā te rerekētanga o ngā tohu, he uaua pea te tātaritanga i ētahi wā. Tērā pea ka whakahaerehia e tō tākuta he hītori hōhonu mā te pātai i ngā pātai pēnei i:
- He aha te huringa tuatahi i kitea e koe, ā, nōnahea i tīmata ai?
- Kua pāngia koe, tō tamaiti rānei e ngā mate hopuhopu ? Mena koinā, he aha ngā mea ka puta i te wā o tētahi wā, ā, kia pēhea te roa o te mau?
- Kia pēhea te auau o te mamae o te upoko?
- He hītori o ngā mate hopuhopu, he TSC rānei tō tētahi o tō whānau?
Muri iho, ka tirohia e tō tākuta tō kiri me ō kanohi, ā, ka tukuna pea koe mō ētahi atu whakamātautau:
- Matawai CT : Ka whakamahi i tētahi raupapa hihi-X hei tango pikitia taipitopito o roto, hei āwhina i te kimi i ngā pukupuku.
- Matawai MRI : Ka homai he whakaahua mārama ake, he whakaahua taipitopito ake i te matawai CT, tae atu ki ngā tirohanga o te roro me te rere o te wai. I ētahi wā, ka whakamahia he tae rerekē hei whakapai ake i te māramatanga.
- Echocardiogram: He whakamātautau oro o te ngākau hei tautuhi i ngā pukupuku me ētahi atu rerekētanga hanganga.
- Ngā whakamātautau ira: He whakamātautau toto ka taea te whakaū i te aroaro o te TSC.
He aha ngā kōwhiringa maimoatanga?
Ka whakaritea ngā maimoatanga i runga i ō tohumate me te wāhi o ngā pukupuku. Ko te whāinga matua o tō tīma hauora he whakahaere i ngā tohumate kia pai ai tō noho.
- Mō ngā whatukuhu: Tērā pea ka whakamahia ngā rongoā (pēnei i te everolimus) hei aukati i te rere o te toto ki ngā pukupuku, hei whakaiti rānei i a rātou. Tērā pea ka hiahiatia he pokanga i ētahi wā. Mēnā he nui rawa ngā mate, ka hiahiatia pea he dialysis , he whakawhitinga whatukuhu rānei.
- Mō te roro: Ka taea te whakaiti i ētahi pukupuku roro mā te rongoā. He rongoā motuhake anō hoki kei te wātea hei whakahaere i ngā mate hopuhopu. I ētahi wā, ka mahia he pokanga hei tango i ngā pukupuku. He mea nui te rongoā wawe i ngā mate hopuhopu o te tamarikitanga mō te whanaketanga o te roro me ngā pūkenga ako.
- Mō ngā pukahukahu:Mō te hunga whai raruraru pūkahukahu, ka tohua pea ngā rongoā ārai mate pēnei i te sirolimus.
- Mō te kiri: Ka taea te rongoā me te whakangawari i ngā puku me ngā pāti mā te whakamahi i te rongoā taiaho , te dermabrasion rānei.
Ko te mea nui ko te pupuri i te whakawhitiwhiti kōrero tuwhera me tō tākuta kia whakawhanakehia he mahere maimoatanga whaiaro e tika ana māu.
Te noho tahi me te TSC
He uaua te noho me te mate pūmau , engari mā te whakahaere tika, ka taea e koe te ora i tētahi oranga whai hua.
- Kia tūpato ki tō tinana: Kia aro ki ngā huringa hou katoa, ka kōrero tonu ki tō tākuta. Tangohia ō rongoā kia rite ki te wā i whakaritea, kaua hoki e mahue ō wā whakarite.
- Kia rite mō ngā āhuatanga ohorere: Kōrerohia ki tō tākuta ngā mea hei mahi i roto i te āhuatanga ohorere, me te wā me toro atu koe ki te Tari Ohorere (ED) .
- Tukua ngā tamariki kia tamariki: Ki te mea he TSC tāu tamaiti, kia maumahara he tamariki tonu rātou. Whakaratohia he taiao hei tākaro, hei ako, hei harikoa hoki mā rātou.
- Rapua he tautoko: Kōrerohia tō haerenga ki te whānau me ngā hoa, me te whakamōhio atu ki a rātou me pēhea te tautoko pai i a koe.
- Oranga hinengaro: He mea noa te pōuri, te taumaha rānei i ētahi wā. Ki te mea kei te raru koe, kaua e mangere ki te rapu tautoko mai i tētahi kaiwhakatakoto whakaaro, i tētahi tohunga hauora hinengaro rānei.
Ahakoa kāore anō kia kitea he rongoā mō te TSC, mā te maimoatanga me te whakahaere tika, ka taea e te nuinga o te tangata te noho i tētahi oranga noa me te noho ora hihiri, motuhake hoki.
Karere Tangohia ki te Kāinga
- Ko te Tuberous Sclerosis Complex (TSC) he āhuatanga ira e puta ai ngā pukupuku kore-matepukupuku i ngā wāhanga rerekē o te tinana.
- He rerekē ngā tohu; he maha ngā wā ka kitea ngā wāhi kiri, ngā haurangi, me ngā whakaroa whanaketanga.
- Ahakoa kāore anō kia kitea he rongoā mō tēnei wā, kei reira ngā maimoatanga whai hua e wātea ana i ēnei rā hei āwhina i te whakahaere i ngā tohumate, hei whakaiti i ngā pukupuku, hei tuku hoki i te oranga noa.
- Ki te kite koe i ētahi huringa rerekē i roto i tō tinana, i te tinana rānei o tāu tamaiti, me toro atu ki tō tākuta tonu.
- Ma te tiaki hauora me te whakahaere tika, ka taea e te nuinga o te hunga whai TSC te ora i ngā oranga hihiri, motuhake, me te whai hua.
Te Matatini Tuberous Sclerosis, TSC, mate ira, pukupuku roro, haurangi, pāpaka kiri, mate tākihi, tirohanga whānui mō te TSC, ngā tohu o te TSC
