Kia ako tātou mō tēnei āhuatanga morearea ka puta whakarere i te wā pokanga: te Malignant Hyperthermia!

Ki te mea me pokanga koe, tetahi rānei e mōhio ana koe, kua rongo pea koe i te kupu whakamoe. Ko tōna tikanga ka whakamoea tātou i te wā e pokanga ana. Engari kua whakaaro kē koe ka pāngia ētahi tāngata e te mate kino, tae atu ki te mate, ki ēnei rongoā whakamoe? Ko tētahi o ngā mate onge engari he tino kino, ko te Malignant Hyperthermia. Tērā pea he ahua whakamataku te ingoa, engari ki te mōhio tika koe ki tēnei mea, ka taea te karo i ngā raruraru maha. Nō reira, me kōrero taipitopito tātou mō tēnei mea i tēnei rā.

He aha te Matū Whakawera Kino?

He kupu ngāwari noa iho, he mate ira te 'Malignant Hyperthermia'. Arā, nā te whakarerekētanga o ngā ira i roto i ō tātou ira. Ina hoatu he rongoā whakamoe ki te tangata whai mate pēnei, ka tino kino te urupare a tōna tinana. He āhuatanga ohorere tēnei ka taea te whakararu i te ora.

Ko te mea pai rawa atu, ahakoa he mea tuku iho tēnei i ia whakatipuranga, he maimoatanga anō kei reira mōna . Ā, ki te mōhio wawe koe ki tēnei mōrearea, ka taea e koe te tiaki i a koe anō mai i taua mea.

E mōhio ana tātou, ina tukuna he rongoā whakamoe whānui ki a tātou, kāore tātou e rongo i tetahi mea i te wā e pokangahia ana. He maha ngā momo rongoā hei whakarato i tēnei rongoā whakamoe. Heoi, he torutoru noa iho o aua rongoā ka puta ai tēnei āhuatanga e kiia nei ko te 'Malignant Hyperthermia'. Ko te nuinga o ngā rongoā whakamoe kāore e puta ai tēnei.

Ko tētahi atu mea nui, i ētahi wā, kāore pea te tangata e mōhio kei a ia te whakarerekētanga ira ka puta ai te Malignant Hyperthermia, nā te mea kāore pea rātou e whakaatu i tētahi tohu tae noa ki te wā e pāngia ai rātou e ngā rongoā kua whakahuatia.

Ko wai te mea ka nui ake te tūponotanga ka pāngia e tēnei?

Kia kite tātou ko wai kei te mōrearea nui ake ki te pāngia e tēnei mate 'Malignant Hyperthermia'.

Ko te tikanga, mēnā kua pāngia tētahi o tō whānau e te mate "Malignant Hyperthermia" i mua , ka tūpono pea ētahi atu mema o taua whānau ki te tūponotanga. Ina koa, mēnā kua pāngia tētahi whanaunga tuatahi (arā, whaea, pāpā, tuākana, teina, tamariki) e te mate, ka kiia koe he "pāngia".

Nō reira, ki te mea me pokanga koe, he mea tino nui kia whakamōhio atu koe ki te tohunga whakamoe mēnā kua pā tēnei ki tētahi o tō whānau.

Whakaarohia, me kī atu kāore anō tō whānau kia pāngia e te mate pāwera kino pēnei. Heoi anō, ko te hunga whai mate uaua ka nui ake pea te mōrearea. Ko ētahi o ēnei mate ko:

• 'Te mate matua o te pokapū'
• 'Te mate maha-minicore'
• Te mate a King-Denborough
• `Te mate STAC3`

Ahakoa te āhua uaua o tēnei, he mea pai kia mōhio te hunga whai i ēnei āhuatanga ki tēnei.

He pēhea te noa o tēnei āhuatanga?

He pēhea te noa o tēnei 'Malignant Hyperthermia'? Ahakoa he rerekē ngā tatauranga, e kiia ana ka puta pea tēnei āhuatanga i roto i te kotahi i roto i te 100,000 pokanga i roto i ngā pakeke. I roto i ngā tamariki, ko tēnei tau he kotahi i roto i te 30,000 pokanga. He mea nui hoki kia mōhiotia ko te haurua o ngā tūroro kua tautuhia kei raro iho i te 19 tau te pakeke.

Engari e whakaaro ana ngā kaipūtaiao ko te hunga e pāngia ana e tēnei mate 'Malignant Hyperthermia' ka nui ake te tūponotanga kia noho ki te hapori. Nā te mea he tokomaha te hunga kāore i te pāngia e ngā rongoā motuhake e puta ai tēnei mate puta noa i ō rātou oranga, kāore pea rātou e mōhio kei te mōrearea rātou.

He aha ngā tohu o tēnei?

Ka rerekē pea ngā tohu o te 'Malignant Hyperthermia' i ia tangata, i ia tangata. Ka puta pea i te wā pokanga, i a koe rānei e ora ana i te pokanga. Hei tauira, ki te mea kei raro koe i te rongoā whakamoe, ā, ka puta mai ēnei tohu i te wā pokanga, kāore koe e rongo. Heoi, he tino mataara tō kaiwhakamoe, tō nēhi rongoā whakamoe rānei mō tēnei. Ka tino aroturukihia e rātou te hunga katoa e raro ana i te rongoā whakamoe. Ina kite tonu rātou i ēnei tohu, ka tīmata tonu rātou i te maimoatanga mō te 'Malignant Hyperthermia'.

Ngā tohu tōmua

Koinei ngā tohu tuatahi ka kitea pea e koe:

• Te pikinga o te tere o te manawa (tachycardia) kāore i te mārama . Ka piki ohorere te tere o te manawa.
• He pikinga ohorere o te nui o te hauhā waro e tukuna ana mai i te tinana . Ka taea hoki te rongoā i tēnei mā te whakamahi i ngā taputapu hauora.
• Te tere o te manawa (tachypnea). Ka tīmata koe ki te manawa tere, me te mea kei te whakapūpū koe i te manawa.
• Te pakari o ngā uaua, te pakari (`muscle rigidity`). Ka pakari ngā uaua o te tinana me te mea e kurua ana e te toka.
• Te pikinga ohorere o te pāmahana o te tinana . Ka tīmata te werawera o te tinana.

Ngā tohu e puta ake ana i muri mai

I te kino haere o te āhuatanga, ka kitea pea e koe ngā tohu pēnei i:

• He pikinga ake o te pāmahana tinana i runga ake i te kirika noa .
• He mimi pouri . Tērā pea ka huri parauri.
• E whakaatu ana ngā whakamātautau toto i te pakaru o ngā uaua .
• Ngā rerekētanga o te manawa o te ngākau (arrhythmia). He rerekē te pā o te ngākau.
• Te toto .
• Ngā āhuatanga pēnei i te haurangi .

He aha i puta ai te hyperthermia kino?

Nā, kia titiro tātou he aha i puta ai tēnei āhuatanga e kiia nei ko te 'Malignant Hyperthermia'.

He pūmua rerekē kei roto i ngā pūtau uaua o te hunga whai mate pēnei. Nā te huringa o ō rātou ira tēnei, e kiia nei ko te whakarerekētanga ira .

He māmā noa iho, he rite tō tātou DNA ki tētahi "pukapuka tohutohu" kei roto ngā mōhiohio e hiahiatia ana e ngā pūtau o tō tātou tinana hei mahi i ā rātou mahi. Mēnā he iti noa ngā mōhiohio e ngaro ana i roto i tēnei DNA, mēnā rānei he kino kei tētahi mea, koinā te wā e puta ai ngā mate ira pēnei i ēnei.

Kāore tēnei whakarerekētanga ira e puta ai he tohu i roto i te oranga noa. Heoi, ki te pāngia te tangata whai tēnei whakarerekētanga ira e ngā rongoā whakangote motuhake kua whakahuatia ake nei – he tino onge rānei, ki te pāngia e te pāmahana teitei, ki te mahi whakakori tinana rānei – ka tīmata ō rātou pūtau uaua ki te tuku i te konupūmā i roto i te āhua rerekē.

Nā reira, ka haere tonu te whakawhāiti me te pēpeke o ngā uaua, ka piki haere te pākia o te tinana me te pāmahana o te tinana i tētahi ara rerekē. Ā tōna wā, ka mate ngā pūtau uaua, ā, ka nui te pāhare pāporo e kohia ana ki roto i te toto. Ka taea e tēnei te arahi atu ki ētahi atu tohu me ngā raruraru.

Ka taea e ngā rongoā whakamoe te whakaputa i tēnei āhuatanga.

Ka pāngia pea te tangata kua pāngia e te mate `Malignant Hyperthermia` e tēnei tauhohenga mēnā ka hoatu ki a ia ēnei rongoā whakangote e whai ake nei:

Ngā rongoā e manawahia ana:

• `Harothane`
• `Desflurane`
• `Sevoflurane`
• `Isoflurane`

Ngā rongoā ā-roto:

• Succinylcholine – He rongoā whakangāwari uaua tere tēnei.

Me pēhea koe e mōhio ai ki tēnei?

Mena ka pokangahia koe mā te whakamahi i tētahi rongoā whakamoe morearea, me te kore e mōhio kei a koe te tūponotanga ki te pāngia e te `Malignant Hyperthermia`, e kore rawa koe, e kore hoki te tohunga whakamoe e mōhio kei a koe taua mate tae noa ki te puta o te tauhohenga.

Heoi, ina hoatu he rongoā whakamoe ki a koe, ki tētahi atu rānei, ka aroturukihia e te tohunga whakamoe, e te nēhi rānei ō tohu ora me tō hauora i te wā o te pokanga, ā, i muri hoki i te pokanga. Mena kei a koe te 'Malignant Hyperthermia', ka kitea e rātou mā ngā tohu ora me ngā tauhohenga rerekē kua whakahuatia i mua ake nei. Arā:

• Te pikinga ake o te tere o te ngakau, kāore he take.
• Te pikinga tere o te pāmahana tinana.
• Te pikinga ohorere o te nui o te hauhā waro e tukuna ana mai i te tinana.
• Te pūmau o te uaua.

Kua whakangungua ngā tohunga whakamoe kia kite wawe i ēnei huringa . Kātahi ka taea e rātou te urupare wawe ki tēnei āhuatanga ohorere. Ko te tere o te tautuhi mate, ko te nui ake o te maimoatanga me te whakahaere i te āhua ka taea.

Me pēhea koe e mōhio ai i mua i te wā mēnā kei a koe tēnei mōrearea?

Mena kei te whakaaro koe, kei te whakaaro rānei tō tākuta kei te mōrearea koe ki te Malignant Hyperthermia, ahakoa nā tō hītori whānau, nā ētahi atu take rānei, tera ētahi whakamātautau motuhake ka taea te mahi hei whakaū i tēnei:

• Whakamātautau whakawhāiti uaua (CHCT) : Mō tēnei whakamātautau, ka tangohia he wāhanga iti o tō uaua (koiora uaua) ka tukuna te tauira kiko ki ngā wairewa o te halothane me te kawheine hei tirotiro mēnā ka aro ki te hau rongoā whakamārie.
• Whakamātautau ira : Ka taea e ēnei whakamātautau te tautuhi i ngā whakarerekētanga ira i roto i te DNA e kiia nei ko RYR1, STAC3, me CACNA1S. Neke atu i te 45 ngā whakarerekētanga ira kua tautuhia ka taea te whakararu i te mate pukupuku kino.

Heoi, he tino utu nui ēnei whakamātautau hei kite i te mate pāwera ki te 'Malignant Hyperthermia', ā, ka taea anake te mahi i roto i ētahi taiwhanga motuhake. Hei tauira, e whā noa ngā wāhi i te United States e wātea ana te whakamātautau 'CHCT'. Nō reira, ki te hiahia koe ki te pokanga ohorere, ā, kua pāngia tō whanaunga tata e te 'Malignant Hyperthermia', ka whakamahia e ngā kaiwhakamoe ngā rongoā whakamoe haumaru ake hei utu mō aua rongoā morearea.

He aha ngā maimoatanga mō tēnei?

Ko te rongoā matua mō te hyperthermia kino ko te dantrolene (ingoa waitohu Dantrium®).

Ina whakapae tonu ngā tākuta whakamoe i tētahi āhuatanga e kiia nei ko te 'Malignant Hyperthermia', ka hoatu e rātou tēnei rongoā 'dantrolene'. I taua wā anō, ka mutu te hoatu i te rongoā whakamoe i puta ai te tauhohenga, ā, ka tere tonu te whakaoti a te taote pokanga i te pokanga.

Hei tāpiritanga, ka rongoatia hoki e te tīma whakamoe me te tīma pokanga ētahi atu tohu me ngā raruraru i puta mai i te `Malignant Hyperthermia`. Ko ētahi o ēnei maimoatanga ko:

• Ka rongoatia te hyperthermia mā ngā tikanga whakamatao , pērā i te hoatu wai matao ki roto i te uaua me te pani i ngā putea hukapapa.
• Ka tukuna te hāora mā te kopare kanohi, mā te ngongo rānei e whakanohoia ana ki roto i te trachea.
• Mena he koretake te pā o te ngakau (arrhythmia), ka hoatu he rongoā mōna.

Kia tau tō āhua, ka puritia koe ki te wāhanga tiaki nui (ICU) mō te iti rawa i te rā kotahi hei tirotiro māu . Ka whakahaerehia e te tīma hauora ētahi atu whakamātautau (hei tauira, ngā whakamātautau toto) ā, ka tukuna atu he maimoatanga anō mēnā e tika ana.

Ka taea te ārai i tēnei?

He uaua ake te ārai i te Malignant Hyperthermia, ki te kore koe e mōhio kei a koe te whakarerekētanga ira e hua ai tēnei mate , ki te kore rānei tētahi o tō whānau e pāngia ana e te mate, ā, kua kōrero koe ki tō kaiwhakamoe. Mēnā koinei te āhua, e kore te kaiwhakamoe e whakamahi i aua rongoā mōrearea i roto i tō mahere whakamoe.

Engari, ko te mea pouri, ko te nuinga o te wā ka mōhio te tangata kei te mōrearea rātou ki te mate pukupuku nui i muri i te pānga ki a rātou.

He aha ngā mea ka puta i muri i tēnei āhuatanga? Ka taea te whakaora?

Ki te kitea wawetia ngā tohu o te 'Malignant Hyperthermia' ā, ki te rongoatia tika, ka taea te ora katoa . He mea tino nui te mahi tere a te tīma whakamoe me te tīma pokanga i konei.

Heoi, me mahara anō hoki tātou i ētahi wā, ahakoa te tere o te maimoatanga, ka pā mai te ngoikore o ngā okana maha, tae atu ki te mate.

He aha atu ngā raruraru ka puta mai i te hyperthermia kino?

Ka taea e te hyperthermia kino te whakaputa i ngā raruraru kino pēnei i:

• Te pakaru o te uaua
• Te ngoikore o te whatukuhu
• Te ngoikore o te ate
• Te toto
• Te hopukanga o te ngākau
• Ngā mate hopuhopu
• Koma
• Mate

He mate tēnei?

Ki te kore e rongoatia, ka mate pea te `Malignant Hyperthermia`. Ahakoa te maimoatanga tika, ko te reiti mate e pūrongohia ana kei waenga i te 3% me te 5%. Nō reira, ehara tēnei i te mea hei whakaaro māmā noa iho.

Āhea ahau me toro atu ki te tākuta mō tēnei mea?

Mena kei te whakarite koe mō te pokanga, he mea tino nui kia kōrero koe ki ngā tākuta mēnā kua pāngia tētahi o tō whānau e ngā raruraru o mua ki te rongoā whakamoe .

Waihoki, ki te mea kua kitea houtia koe he whakarerekētanga ira e hua ai te Malignant Hyperthermia i roto i tētahi whanaunga toto (whanaunga koiora), ki te rongo rānei koe kua pāngia taua tangata e te mate e kiia nei ko te Malignant Hyperthermia, he mea tino nui kia whakamōhiotia tō tākuta me te tuhi i tēnei ki roto i ō pūkete hauora.

Ngā mea tino nui hei maumahara māu i roto i tēnei tuhinga

He tauhohenga kino te Malignant Hyperthermia, ka mōrearea pea te ora ki ētahi rongoā whakangote.

Ko te rongo pai, ka taea te rongoā, ā, ki te mōhio koe kei roto koe i te mōrearea, ka taea te ārai.

Ki te taea, kia mōhio koe ki ngā taipitopito katoa mō te hītori hauora o ō whanaunga toto. Tukuna atu aua mōhiohio ki tō tākuta. Nā te mea ko te 'Malignant Hyperthermia' te take o tētahi whakarerekētanga ira e tukuna iho ana i roto i ngā whakatipuranga, ki te pāngia tētahi o tō whānau e tēnei mate, ka nui ake tō tūponotanga.

Mena he pātai, he āwangawanga rānei āu mō te pokanga me te rongoā whakamoe ka whiwhi koe, kaua e mangere ki te pātai ki tō tākuta. Kei reira rātou hei āwhina i a koe. Ko tō mōhio me tō whakawhitiwhiti kōrero tuwhera ki tō tīma hauora te parenga pai rawa atu mōu.

te hyperthermia kino, te rongoā whakamoe, te pokanga, ngā mate ira, te hītori whānau, ngā raruraru rongoā whakamoe, te pāmahana tinana teitei