E mōhio ana koe ki te mate ROHHAD? Kei tō tamaiti ēnei tohu?

Kua tīmata kē te nui haere o tō tamaiti? Kua kite rānei koe he uaua ki te manawa i a ia e moe ana? I ētahi wā, he tohu ēnei o tētahi mate tino onge e kiia nei ko te ROHHAD Syndrome. Me kōrero taipitopito atu tātou mō tēnei, he mea nui hoki kia mōhio ki ngā mea pēnei.

He aha te Mate ROHHAD?

He whakarāpopototanga noa iho, he mate onge, he mate morearea te mate ROHHAD e pā ana ki ngā tamariki nohinohi. Ko te nuinga o te wā ka pā ki te pūnaha endocrine, te pūnaha io autonomic, me te manawa. Ka taea e ngā tamariki whai mate nei te piki ake o te taumaha i roto i te wā poto rawa . Ka taea hoki e rātou te pāngia e ngā huringa o ngā tauira manawa, ngā whanonga, me te whakahaere kare ā-roto i a rātou e moe ana, ā, i ētahi wā i a rātou e ara ana.

He mate hauora hou te mate ROHHAD. I te tau 1965 i kitea tuatahitia ai, i whakaahuatia ai hoki. Ki te whakaarohia, he iti iho i te 200 ngā kēhi o tēnei mate kua pūrongohia puta noa i te ao. Nō reira, ka taea e koe te whakaaro he aha te onge o tēnei mate. Kei te rangahau tonu ngā tohunga hauora i tēnei mate kia kitea ai te take tuturu me te kimi i tētahi rongoā tuturu. I tēnei wā, kāore he whakamātautau, he maimoatanga motuhake rānei mō te mate ROHHAD. Nō reira, ka rongoā takitahi ngā tākuta i ia tamaiti i runga i ō rātou tohu.

He aha ngā tohu o te mate ROHHAD?

Ko ngā tamariki e pāngia ana e te mate ROHHAD he hauora, ā, he pai te whanaketanga i mua i te putanga mai o ngā tohu. Ahakoa ka puta mai ngā tohu i te 9 o ngā tau, ko te nuinga o ngā tamariki ka whakaatu i ō rātou tohu tuatahi i waenga i te 2 me te 4 tau.

I ahu mai te ingoa 'ROHHAD' i ngā reta tuatahi o ngā tohu matua e whā o tēnei mate. Me titiro tātou ki ēnei tohu:

• (RO) Te Momona Tere: Koinei te wā ka piki haere te hiahia o te tamaiti , ka piki ake te taumaha o te 20-30 pauna (9-13 kirokaramu) i roto i te wā poto, i waenganui i te toru me te tekau mā rua marama. Koinei te tohu tuatahi o te momona. Whakaarohia, ehara i te mea ko te pikinga taumaha anake, engari he huringa tere, ka kitea.

• (H) Te koretake o te mahi a te hypothalamus: Ko te hypothalamus tētahi wāhanga o tō roro e whakahaere ana i te repe pituitary. E whakahaere ana hoki i ngā mea maha pēnei i te tipu o te tinana, te taumaha, te hiahia kai, te pakeketanga, ngā kare ā-roto, me ngā whanonga. Nō reira, ki te mea he raruraru kei roto i tana mahi, ka taea e te tamaiti teTērā pea ka puta ngā tohu pēnei i te momona, te poto o te tinana, te haurangi, te whakaroa i te tipu, te nui haere, te iti haere rānei o te mimi, me te pakeketanga wawe, te roa rānei o te pakeketanga . Hei tāpiri, tērā pea ka puta anō hoki ngā āhuatanga pēnei i te hypothyroidism me te mate huka .

• (H) Te ngoikore o te manawa: Kāore e taea e ngā tamariki e pāngia ana e te mate ROHHAD te whakahaere tika i ō rātou manawa. Ko te tikanga tēnei, ina heke te taumata hāora i roto i te toto, ina piki rānei te taumata hauhā waro, kāore te tinana e manawa hōhonu, e manawa tere rānei hei whakaea. He āhuatanga tino morearea tēnei. I ētahi wā, ka puta ohorere mai, pērā i muri i te mate manawa iti, pērā i te makariri, i muri rānei i te rongoā whakamoe.

• (AD) Te Whakakorenga o te Mana Motuhake: Ko te pūnaha io motuhake (ANS) o tō tātou tinana e whakahaere ana i ngā mahi taketake kāore e taea te whakahaere, pērā i te manawa, te nakunaku, te tere o te ngakau, me te pāmahana o te tinana. Nō reira, ko ngā tamariki kua pakaru ki tēnei pūnaha io motuhake ka pāngia pea e ngā tohu pērā i te tere o te ngakau puhoi rawa (Bradycardia), te pāmahana tinana iti, me te heke o te māramatanga mamae . Whakaarohia, ko te iti o te mamae te tikanga ahakoa he aituā nui, tera pea kāore te tamaiti e mōhio.

He aha ngā pūtake o te mate ROHHAD?

Ina hoki, kāore anō kia kitea e ngā tākuta te tino take mō te mate ROHHAD. Heoi, e toru ngā ariā matua e rangahauhia ana i tēnei wā hei whakamārama i tēnei mate.

1. Ira: He tokomaha ngā kairangahau e whakapono ana ko te mate ROHHAD i puta mai i te whakarerekētanga ira . Heoi, kāore anō kia mōhiotia te ira tuturu. Ko ngā mea katoa o roto i tō tātou tinana e whakahaerehia ana e ngā ira, nō reira ka pāngia e tētahi huringa.

2. Epigenetics: E whakapono ana ētahi kairangahau ka taea te whakakā i ngā ira e whakahaere ana i ētahi mahi i roto i ō tātou tinana ina hiahiatia, ā, ka taea te whakaweto ina kore e hiahiatia. Ka kiia tēnei ko te epigenetics. I ahu mai tēnei whakaaro i tētahi rangahau i mahia ki ngā māhanga ōrite. I roto i tēnei, i whakawhanakehia e tētahi tamaiti te mate ROHHAD, engari kāore tētahi. Nō reira, ka taea te whakaaro ehara i te mea ko ngā ira anake, engari ko ngā rerekētanga o te mahi a aua ira anō hoki ka pā ki tēnei.

3. Ārai Mate Ā-Iwi: E kī ana ētahi rangahau he mate ārai mate anō hoki te mate ROHHAD.Kua kitea he hononga i waenga i ngā mate pūnaha ārai mate, arā, ko te pūnaha ārai mate o te tinana e whakaeke ana i ōna ake pūtau. I roto i tētahi rangahau, i kitea he paturopi kei roto i te wai cerebrospinal (CSF) e rua ngā tamariki e pangia ana e te mate ROHHAD, arā, ko ngā immunoglobulins, i huaina ko ngā immunoglobulins . Mā te whakamahi i ēnei mōhiohio, i whakatau ngā kairangahau he mumura kei roto i te pūnaha io matua (CNS) , te roro me te taurakira. Ko te tikanga o tēnei, ko te pūnaha ārai mate o te tinana kei te whakaeke hē i te roro.

He aha ngā raruraru o te mate ROHHAD?

Haunga ngā tohu matua i kōrerotia i mua ake nei, ka pāngia pea ngā tamariki e te mate ROHHAD e ētahi atu raruraru , arā, he raruraru hauora anō. He mea nui kia mōhio ki ēnei:

• Ngā raruraru hinengaro me ngā raruraru whanonga: Tērā pea ka pāngia ēnei tamariki e ngā āhuatanga pēnei i te kore okiokinga, te pukuriri, te kaha o te mahi, te ngenge tonu, te manukanuka, te pouri, me te hauātanga hinengaro . Ka pā pea ēnei ki te oranga o ia rā o te tamaiti me te whānau hoki.

• Ngā raruraru o te pūnaha io: Tērā pea ka pāngia ngā tamariki e te mate ROHHAD e ngā mate pēnei i te haurangi, te nekehanga rerekē o ngā kanohi (nystagmus), te moe pōrearea, te mate whanaketanga rānei .

• Ngā mate pāwera o te pūnaha io: Ko ngā mate pāwera tino kitea i roto i ēnei tamariki ko te Mate Huka, te Ātete ki te Insulin, te kore e manawanui ki te huka toto, me te mate ate momona (steatotic). He āhuatanga ēnei ka pā he pānga hauora mō te wā roa.

• Ngā pukupuku io: I waenganui i te 40% me te 56% o ngā tamariki e pāngia ana e te mate ROHHAD ka pāngia e ngā pukupuku io. Ko tētahi tauira he momo pukupuku e kiia nei ko te ganglioneuroma . He pai te nuinga o ēnei pukupuku, engari i ētahi wā ka taea te kino . Nō reira, kei te rapu tonu ngā tākuta i tēnei.

• Te aukati manawa: Koinei te raruraru tino morearea. He mutu ohorere te manawa me te mahi a te ngākau. Ka tūpono pea tēnei ki te ora.

Me pēhea te tātaritanga o te mate ROHHAD? (Tauātanga)

I tēnei wā, e kiia ana te mate ROHHADKāore he whakamātautau kotahi hei whakaū i te mate. Engari, ka mahi tahi tētahi rōpū tohunga hauora ki te whakatau i te mate i runga i te huinga motuhake o ngā tohu o te tamaiti. He rite ki tētahi kaitirotiro, e kohikohi ana i ngā taunakitanga, ā, ka tae ki tētahi whakatau.

Hei tātaritanga mō te mate ROHHAD, me tutuki i te tamaiti ngā paearu e whai ake nei:

• Me kitea ngā tohu o te putanga ohorere mai o te momona nui me te ngoikore o te manawa i te wā e moe ana.
• Me kitea ngā tohu o te ngoikore o te hypothalamus (hei tauira, te pikinga tere o te taumaha, te hypothyroidism, ngā huringa rānei o te pakeketanga - i te wā tōmua, i te wā tōmuri rānei).
• He mea nui kia whakaūtia kāore he whakarerekētanga ira (rerekētanga ira) i roto i te ira PHOX2B. He mea nui tēnei hei wehewehe i tēnei i te CCHS (Congenital Central Hypoventilation Syndrome), tētahi atu mate e rite ana ki te mate ROHHAD (hei tauira, te heke o te manawa i te wā e moe ana).
• Kāore pea ēnei tohu e kitea i ngā tau tuatahi o te oranga. I te nuinga o te wā ka tīmata i muri mai.

Nā te mea he tino onge te mate ROHHAD, he maha ngā wā ka hē te tautuhi i ngā tamariki i te tīmatanga. Nō reira, ki te pāngia koe e ēnei tohu, he mea nui kia rapu tohutohu tika mai i tētahi tīma hauora mātau.

Me pēhea te rongoā i te mate ROHHAD? (Te rongoā)

He rerekē te maimoatanga mō te mate ROHHAD i ia tamaiti, i ia tamaiti, i runga i ngā tohu. Kāore e taea te hoatu i te mahere maimoatanga kotahi ki ia tamaiti, nā te mea he rerekē te āhua o te katoa. Heoi, kotahi te mea e rite ana ki te katoa. Mena ka kitea he rerekētanga o te manawa o te tamaiti e pangia ana e te mate ROHHAD i a ia e moe ana, me whakamahi rātou i tētahi mīhini e kiia nei ko te CPAP, ko te BiPAP rānei hei āwhina i a rātou ki te manawa. I roto i te wā, me whakamahi pea rātou i tētahi ventilator hei tautoko i te tukanga manawa. He tikanga whakaora ora tēnei.

I te rongoā i ngā tamariki e pāngia ana e te mate ROHHAD, me rapu āwhina a ngā tākuta tamariki i ngā momo mara. Hei tauira:

• Ngā tohunga pūkahukahu
• Ngā tohunga roro
• Ngā tohunga endocrine (ngā tākuta e tohunga ana ki ngā repe endocrine (ngā homoni)
• Ngā Tohunga Rongoā Rongoā (Ngā Tohunga Taringa, Ihu, Korokoro)
• Ngā tohunga ngakau
• Ngā tohunga moe
• Ngā tohunga kai
• Ngā Tākuta Matepukupuku
• Ngā tohunga hinengaro
• Ngā Tākuta Hinengaro

Mā te kotahitanga o ēnei tāngata katoa ka whai tātou ki te whakarato i te maimoatanga pai rawa atu mō te tamaiti.

He aha te heke mai o ngā tamariki e pāngia ana e te mate ROHHAD? (Tirohanga)

Ina hoki, kāore anō kia kitea he rongoā mō te mate ROHHAD i tēnei wā. Ko te whāinga matua o te maimoatanga he whakahaere i ngā tohu motuhake mō ia tamaiti, me te pupuri i te kounga o te oranga o te tamaiti i te mea pai rawa atu.

Te Mate ROHHAD me te Tataritanga Ora

Nā te mea he tino onge te mate ROHHAD, he uaua ki te whakatau tika i te roanga o te oranga. Kāore anō kia ranea ngā raraunga mō tēnei mate.

Heoi, ko te tino take o te mate mai i tēnei mate he raruraru e kiia nei ko te aukati manawa, arā, ko te mutu ohorere o te mahi a te ngākau me te pūnaha manawa .

Ka taea te ārai i tēnei? (Te ārai)

Kāore anō kia kitea he huarahi hei ārai i te mate ROHHAD i tēnei wā - kāore anō kia kitea i tēnei wā.

He mate onge, he mate hou hoki te mate ROHHAD. Nō reira, kei te tīmatanga tonu ngā rangahau mō tēnei mate. Ina whakaarohia te hauora o tāu tamaiti, ko ia rā e haere ana me te kore whakautu ka rite ki te mutunga kore. E mārama ana mātou ki tēnā. Nō reira, kia mahara ki te rapu tautoko mai i tāu tākuta. Ka taea e ia te tohutohu i a koe mō ngā maimoatanga e hiahiatia ana hei whakahaere i ngā tohu o tāu tamaiti, me ngā puna mōhiohio hoki mō tēnei mate.

Ngā mea tino nui me kawe e tātou mai i tēnei pūrākau ki te kāinga (Karere Tangohia ki te Kāinga)

Āe, kua mārama kē koe ki te mate ROHHAD i kōrerotia e tātou. Anei ētahi mea nui hei maumahara māu:

• He tino onge te mate ROHHAD, engari he mate kino rawa.
• Ko ngā tohu matua ko te pikinga ohorere o te taumaha, te uaua ki te manawa, me ngā raruraru ki ngā homoni me te pūnaha io motuhake.
• Kāore anō kia kitea he pūtake motuhake, he rongoā tuturu rānei mō tēnei. Ka whakawhirinaki te maimoatanga ki ngā tohu e puta ake ana.
• Ki te whakaaro koe kei tō tamaiti ēnei tohu, kaua e mataku, engari me toro atu ki tētahi tākuta whai tohu tonu mō ētahi tohutohu.
• Nā te mea he mate onge tēnei, tera pea ka roa te wā e tika ai te tautuhi i tōna mate. Kaua e ngākau māhaki.
• He mea tino nui te whai i ngā tohutohu a ngā tākuta, me te whakarato i te tautoko e tika ana ki te tamaiti.

Kia maumahara, kāore koe i te mokemoke. He mea nui kia rapu tautoko mai i ngā tākuta, i te whānau, ā, ki te tika, i ngā ratonga tohutohu ina pāngia koe e ngā āhuatanga pēnei.