Kei te pupuhi ohorere tāu tamaiti? Me kōrero tātou mō te 'Angioedema Tuku Iho (HAE)'!

Kua kite koe i tāu tamaiti e pupuhi ohorere ana i ngā wāhanga rerekē o tōna tinana, i ētahi wā ko tōna mata, ko ōna ringa, me ōna waewae? Kei te whakaatu rānei tāu tamaiti i ngā tohu o te uaua ki te manawa me te mamae o te puku? Tērā pea he mate tino onge engari he mea nui kei muri i ēnei tohu. I tēnei rā ka kōrero tātou mō tētahi o aua mate, arā, ko te 'Hereditary Angioedema (HAE). Kaua e manukanuka, ka kōrerohia tēnei i roto i tētahi huarahi māmā ka taea e koe te mārama.

He aha tēnei 'Angioedema Tuku Iho (HAE)'?

Ki te whakarapopoto, ko te 'Hereditary Angioedema' (e mōhiotia ana ko te 'HAE') he mate tino onge, he mate ira (arā, ka taea te tuku iho i roto i ngā whakatipuranga). Ka pupuhi i ngā wāhanga rerekē o te tinana o tāu tamaiti. Ka puta tēnei pupuhi i ngā wāhi e kitea ana e te kanohi, pērā i ngā ringa, ngā waewae, me te mata, i ētahi wā rānei i ngā wāhi kāore e kitea e tātou, pērā i ngā kiko o ngā ara manawa o te tamaiti (pūnaha manawa) me te pūnaha nakunaku (ngākau) .

Tērā pea kei te miharo koe he aha i puta ai tēnei pupuhi. Ka puta tēnei pupuhi nā te mea ka turuturu te wai i roto i ngā oko toto iti o te tinana o te tamaiti, e kiia nei ko ngā "Capillaries," ka kohia ki roto i ngā kiko e karapoti ana. Ina kohia tēnei wai, ka ārai i te rere o te toto, o te lymph rānei mā roto i a rātou. He uaua ki te matapae i te wā e puta ai tēnei pupuhi me ōna tohu e pā ana (e kiia ana ko ngā "HAE attacks"). Waihoki, i ētahi wā ka mutu tēnei me te pupuhi iti, engari i ētahi wā ka nui rawa te kino ka morearea te ora.

I roto i te rongoā, ko te kupu "angio" (`Angio-`) e pā ana ki ngā oko toto. Ko te "Edema` (`-edema`) e pā ana ki te pupuhi i puta mai i te kohikohinga wai i roto i ngā kiko. He maha ngā momo `angioedema`, he rerekē ngā take o ia momo. Ko te `HAE` te mea tino onge o ēnei. Ka kiia ko te `congenital`, ko te tikanga ka whānau mai te tangata me tēnei mate.

Mena kua tāpaetia tō tamaiti ki te HAE, tera pea ka tino ruarua koe mō ngā mea ka tūpono atu. Engari mā te mōhio ki tēnei ka āwhina i a koe ki te whakamarie i tō hinengaro: Kei te haere tonu ngā rangahau mō ngā maimoatanga hou mō ngā tāngata o ngā reanga katoa e pāngia ana e te HAE, tae atu ki ngā tamariki. Ka taea e tō tākuta te āwhina i a koe ki te mārama ko ēhea ngā maimoatanga e tika ana mō tō tamaiti me ngā mea ka tūpono atu ā muri ake nei.

He momo 'HAE' anō kei reira?

Āe, ahakoa he momo `angioedema` te `HAE`, ka wehea e ngā tākuta te `HAE` ki ētahi atu momo:

• Momo HAE I: Koinei te momo tino noa . Tata ki te 85% o ngā tūroro HAE katoa he momo tēnei. Ko te take o tēnei ko te korenga o te tinana o te tamaiti e whakaputa i te nui o te pūmua motuhake e kiia nei ko te C1-inhibitor (e mōhiotia ana ko te C1-INH). Ina iti tēnei pūmua C1-INH, ka puta te mumura me te pupuhi i roto i te tinana. E kiia ana hoki tēnei ko te korenga o te C1-INH.
• Momo II HAE: Koinei te tuarua o ngā momo e tino kitea ana. I tēnei wā, ka whakaputa te tinana o te tamaiti i te pūmua C1-INH, engari kāore e mahi tika.
• HAE me te C1-INH noa: Koinei te momo kāore i te tino kitea . He noa ngā taumata pūmua C1-INH me te ngohe o te tamaiti, engari he take anō hei whakapūngā.

Ko ngā momo tuatahi e rua (Momo I me te Momo II HAE) ka pāngia e te 1 i roto i te 50,000 tāngata . Ko te tikanga, i roto i tētahi whenua pērā i te Hononga o Amerika, tata ki te 6,000 tāngata e pāngia ana e tēnei momo HAE. Kāore ngā kairangahau i te tino mōhio e hia ngā tāngata whai HAE he taumata C1-INH noa iho, engari e kī ana rātou he tino onge.

He aha ngā tohu o te HAE?

Ka rerekē ngā tohu o te HAE i ia tangata, i ia tangata. Ko ētahi o ngā tohu noa ko:

• Ngā pupuhi e puta ana i ngā wāhanga rerekē o te tinana o te tamaiti, hei tauira , ngā ringa, ngā waewae, ngā kamo, ngā ngutu, me ngā wāhi ira tangata .
• Ngā tohu o te mumura i roto i te pūnaha nakunaku o te tamaiti (Gastrointestinal (GI) tract). Kei roto i tēnei ko te whakapairuaki, te ruaki, te mamae o te puku, me te korere .
• Ko ngā tohu e puta mai ana i te pupuhi o te waha, te korokoro, me ngā ara manawa o te tamaiti. Kei roto pea i ēnei ko te uaua ki te horomi, te pupuhi o te arero, te tangi haruru ina manawa ana, me te rerekētanga o te reo .

Ko te mea nui: Mena kei te raru tō tamaiti ki te manawa, ki te horomi rānei, waea atu ki te rūma whawhati tata, haria rānei tō tamaiti ki te hōhipera tonu. He tino kino te pupuhi o ngā ara manawa, he raruraru morearea hoki o te HAE. Ka mate pea mēnā kāore e tere te rongoā.

Kāore te HAE e puta ai ngā whewhe. Koinei te rerekētanga matua i waenga i te HAE me ētahi atu momo angioedema, pērā i te angioedema mate pāwera ohorere. Heoi, ko ētahi tāngata whai HAE ka pāngia e te ponana whero kāore e mākūkū i mua i te puta mai o te pupuhi, he tohu pea tēnei mō tētahi whakaekenga e haere ake nei.

E toru ki te rima ngā rā te roa o te 'whakaeke HAE'. Ka puta ohorere mai, ka haere atu ēnei 'whakaeke', nō reira he uaua ki te kī ko te wā me te pēhea e puta ai.

Āhea ka tīmata ngā tohumate HAE?

Ko ngā tohumate HAE ka tīmata i te tamarikitanga, i te taiohitanga rānei . Ka kaha ake te kino i te wā o te pakeketanga. Ka tīmata pea ētahi tamariki ki te pāngia e ngā tohumate i te rua tau te pakeke . Tata ki te 50% o te hunga whai HAE momo I, II rānei ka pāngia e ngā tohumate i te 10 tau te pakeke. I muri i te pakeketanga, ka nui haere, ka kaha ake pea ēnei whakaekenga. Tata ki te katoa e pāngia ana e te HAE momo I, II rānei ka pāngia e ngā tohumate i te 20 tau te pakeke.

Ko te hunga whai HAE engari he noa ngā taumata C1-INH, ka roa ake te tīmatanga o ngā tohu – i te mutunga o te taiohitanga, i te tīmatanga rānei o te pakeketanga.

He aha ngā mea e whakaoho ana i te whakaekenga HAE?

Kāore i te mārama tonu te "pūtake" e puta ai te whakaeke HAE. Heoi, ko ētahi o ngā "pūtake" kua tautuhia ko:

• Whara tinana, aituā iti rānei: Whakaarohia te hinganga o tāu tamaiti i a ia e tākaro ana i tētahi rā.
• Ngā maimoatanga niho: Ngā mahi pēnei i te tango i te niho, te whakakī rānei.
• Pokanga: He pokanga , ahakoa iti, ahakoa nui.
• Te ahotea, te pēhanga kare ā-roto rānei: Ka pāngia pea koe e tētahi whakamātautau kura, e tētahi pakanga iti rānei ki tētahi hoa.
• Ngā mate urutā: Ngā mate pēnei i te rewharewha me te makariri.
• Ētahi mahi ā-tinana: Hei tauira, ngā mahi pēnei i te patopato tonu, te patu ki te hama, te keri rānei ki te hoe.

Kāore pea koe e mārama tonu ki ngā mea e whakaoho ana i te whakaekenga o tāu tamaiti. Heoi, he tino āwhina te pupuri i tētahi pukapuka tuhi mō te wā i puta ai te whakaekenga me ngā mahi a te tamaiti i taua wā (hei tauira, mēnā i te mate te tamaiti, i te ahotea rānei) .

He aha te tino take o te HAE?

Ko ngā momo HAE I me II e puta mai ana i te whakarerekētanga (te panoni rānei) o tētahi ira e kiia nei ko SERPING1. Mā tēnei ira e tohutohu te tinana o tāu tamaiti ki te hanga i tētahi pūmua e kiia nei ko C1-INH. Mena he momo HAE I tāu tamaiti, ko te tikanga o tēnei whakarerekētanga ira kāore e taea e tōna tinana te hanga i te nui o te C1-INH. Mena he momo HAE II tāu tamaiti, ka taea e tōna tinana te hanga C1-INH, engari ko te tikanga o te whakarerekētanga ira kāore te pūmua e mahi tika.

Kei te rangahau tonu ngā kairangahau i ngā take o te HAE me ngā taumata C1-INH noa, engari e mōhio ana rātou ko ngā whakarerekētanga ira i roto i ngā ira e whai ake nei te take pea:

• `F12`
• `ANGPT1`
• `PLG`
• `KNG1`

I ētahi wā, ka kitea pea te HAE me te kore he whakarerekētanga ira kua tautuhia. Ka kiia tēnei e ngā tākuta ko te "HAE-unknown".

Nā ngā whakarerekētanga ira e hua ai te HAE ka kore e mahi tika ētahi pūmua i roto i te tinana o te tamaiti. Ka āwhina ēnei pūmua i ngā wai kia rere noa i roto i ngā oko toto iti o te tamaiti (ngā capillary). Nō reira, ina kore e mahi tika ēnei pūmua, ka turuturu ngā wai ka kohia ki roto i ngā kiko e karapoti ana i ngā oko toto o te tamaiti. Koinei te take o ngā tohu o te HAE.

Nō te whānau te HAE?

Ae, ka tukuna iho te HAE i roto i te tauira autosomal dominant. He māmā noa iho, kotahi noa te ira rerekē e hiahiatia ana hei whakaputa i te mate. Ka taea e te tamaiti te tuku iho i tēnei ira rerekē mai i tōna whaea, i tōna pāpā rānei.

He maha ngā tāngata whai HAE he hītori whānau tō rātou mō te mate. Heoi, i ētahi wā ka taea e te tangata te whakawhanake i tētahi whakarerekētanga ira matapōkere (he whakarerekētanga "de novo" "hou" rānei) me te kore he hītori whānau.

Me pēhea te kitenga a ngā tākuta i tēnei?

Mena ka whakaatuhia e tāu tamaiti ngā tohu o te HAE, ka mahia e te tākuta ngā mahi e whai ake nei:

• Ka whakamātau i te tinana .
• Ka pātaihia koe mō ngā tohumate me ngā hītori hauora o tāu tamaiti.
• Ka mahia ngā whakamātautau toto hei tirotiro i te taumata `C1-INH` o te tamaiti me ana mahi .
• I ētahi wā, ka mahia he whakamātautau ira hei tirotiro mēnā he whakarerekētanga ira kei te pūtake o te HAE.

He aha ngā maimoatanga mō te HAE?

E rua ngā momo rongoā matua mō te hunga whai HAE:

• Ngā rongoā ina hiahiatia: Ko ēnei ngā rongoā ka hoatu i te wā tonu ka puta he whakaeke HAE. Hei tauira, ko ngā rongoā pēnei i a Berinert®, Kalbitor® rānei. Mā te tango i ēnei rongoā i te wā e taea ai i muri i te tīmatanga o ngā tohu ka taea te whakaiti i te kaha o te whakaeke me te ārai i ngā raruraru e morearea ana ki te ora.
• Ngā rongoā ārai mate: Ko ēnei ngā rongoā hei whakaiti i te tūponotanga o te whakaekenga. Hei tauira, ko Cinryze®, Haegarda®, Takhzyro® rānei. Tērā pea ka tohua e te tākuta ēnei rongoā hei whakamahi i te wā e mōhiotia ana te take (pēnei i te haerenga ki te tākuta niho), hei whakamahinga roa rānei, i runga i ngā hiahia o tāu tamaiti.

E ai ki ngā tohunga hauora, he mea nui ngā rongoā e hiahiatia ana, ā, he mea whakaora ora hoki. Ahakoa kei te tango tāu tamaiti i ngā rongoā ārai mate, me wātea tonu ēnei rongoā e hiahiatia ana i ngā wā katoa, i ngā wāhi katoa (i te kāinga me te kura).

Mā te tākuta koe e whakamārama tika ko ēhea ngā rongoā e hiahiatia ana e tāu tamaiti, me pēhea te hoatu, me te wā hoki. Māna anō hoki e whakamārama ko ēhea ngā rongoā ka taea te hoatu ki tāu tamaiti i runga i tōna tau. Kia tino whai i ngā tohutohu, ā, kia tino pātai atu mēnā he mea kāore koe i te tino mārama.

Kia pēhea te tere o taku ora ake i muri i te maimoatanga?

Mā te rongoā e hiahiatia ana, ka tere te pai ake o te ahua o tō tamaiti i te wā o te whakaekenga HAE. Me pai ake ōna tohu i roto i te 30 meneti ki te rua haora i muri i te tīmatanga o te maimoatanga. Mā tō tākuta koe e hoatu ētahi atu kōrero mō te whakahaere i te maimoatanga i te kāinga me ngā mea hei tumanako atu.

Me pēhea te tiaki i tētahi tamaiti me te HAE?

Tērā pea ka pōuri koe ina kite koe i tāu tamaiti e pāngia ana e te whakaekenga HAE. Heoi, he maha ngā mea ka taea e koe te mahi mā tāu tamaiti i mua atu, i te wā e pāngia ana, me muri iho i te whakaekenga:

• Kia mau tonu ngā rongoā e hiahiatia ana i ngā wā katoa: He mea whakaora ēnei i te ora. Me mōhio koe ki te wā e hiahiatia ana ēnei rongoā e tāu tamaiti. Me mōhio hoki koe ki te hoatu. Mā tō tākuta koe e kōrero atu mō tēnei.
• Kaua e whakaarohia he mate pāwera te whakaekenga HAE: Ko ngā rongoā pēnei i ngā antihistamines me te epinephrine, e hoatu ana mō ngā mate pāwera, e kore e āwhina i te whakaekenga HAE. Nō reira, kaua e hoatu ēnei rongoā ki tāu tamaiti.
• Kia tūpato ki ngā mea ka pā mai ai he whakaeke: He rerekē ngā mea ka pā mai i ia tangata, i ia tangata. Waihangahia he rārangi o ngā mea ka pā mai ai he whakaeke ki tāu tamaiti, ka kōrero atu ki tō tākuta. Ngāna kia matara atu tāu tamaiti i aua mea ka pā mai i ngā wā katoa e taea ana. Ki te kore e taea e koe te karo i aua mea, pātai atu ki tō tākuta mō ngā rongoā ārai mate.

Āhea koe me toro atu ki te tākuta?

Tirohia he tākuta i roto i ēnei āhuatanga:

• Ki te whakaaro koe kei tō tamaiti ngā tohu o te 'Hereditary Angioedema'.
• Mena ka puta mai ngā tohu hou e pā ana ki te whakaeke HAE, mena rānei ka rerekē ngā tohu o nāianei.
• Mena te āhua nei kāore te rongoā a tāu tamaiti e mahi ana pērā i te mea e tumanakohia ana.

Āhea koe me haere ki te rūma whawhati tata?

Mena ka pāngia tāu tamaiti e tētahi o ngā āhuatanga e whai ake nei , haere tonu ki te rūma whawhati tata:

• Mena he uaua ki te horomi.
• Mena he uaua ki te manawa.
• Ki te kite koe i ētahi tohu o te pupuhi o te arero, o te korokoro rānei (ahakoa i hoatu he rongoā ki a koe i ngā tohu tuatahi o te whakaekenga).

He tohu pea ēnei mō tētahi pupuhi kino e pā ana ki te ara manawa o tāu tamaiti. Kaua e whakaroa ki te rapu maimoatanga. Ka taea e tēnei pupuhi te whakararu i te ora.

He aha ngā pātai me pātai atu koe ki te tākuta?

Ka taea e koe te pātai atu ki te tākuta i ngā pātai penei:

• He aha te momo HAE kei taku tamaiti?
• He aha te maimoatanga e taunaki ana koe?
• Me pēhea taku mōhio ina hiahia taku tamaiti ki te rongoā i te wā e hiahiatia ana?
• Me whiwhi taku tamaiti i ngā rongoā ārai mate? Mēnā koinā, āhea?
• He mahi, he āhuatanga rānei me karo e taku tamaiti?
• He aha tāu e taea te kī mō te tirohanga a taku tamaiti ki te heke mai?

Me pēhea tātou e whai tumanako ai mō tētahi tamaiti he HAE tōna?

He mate mō te wā katoa te HAE. Heoi, mā te maimoatanga ka taea te whakaiti i te pānga o te mate ki te oranga o te tamaiti. Ka taea hoki e ia te whakaiti i te mōrearea o ngā tohumate e morearea ana ki te ora, pērā i te pupuhi o ngā ara manawa o te tamaiti.

Ka taea te ārai i te HAE?

Kāore anō kia mōhiotia he huarahi hei ārai i te HAE i tēnei wā. Mena kei a koe, kei tō hoa rānei te HAE, ā, kei te ngana koe ki te whānau tamariki,He mea pai kia kōrero koe ki tētahi kaitohutohu ira kia mārama ai koe ki te tūponotanga ka riro mai i tāu tamaiti tēnei mate.

He karere nui tēnei māu.

Ina kitea e koe he mate ira tō tāu tamaiti pērā i te HAE, tera pea ka pā mai ngā momo kare ā-roto ki a koe: te wehi, te manukanuka, te pōhēhē, me te pouri. Tērā pea ka whakapae koe i a koe anō. Engari ko te mea nui me mōhio koe, ehara i te mea nāu tēnei mate.

Ka puta tonu ngā whakarerekētanga ira, he maha ngā wā kāore he take e kitea ana. Kāore e taea e koe te whakahaere i ngā ira ka riro mai i tāu tamaiti. Engari ka taea e koe te whai wāhi nui ki te tiaki i tāu tamaiti – i ētahi wā he whakamanawa noa iho ki a rātou "ka ora koe."

He mea āwhina anō hoki te whakauru atu ki ngā rōpū tautoko tūroro. Mā te kōrero ki ngā mātua o ngā tamariki e pāngia ana e te HAE, me ngā pakeke e pāngia ana e te mate, ka taea e koe te tuku tohutohu me te whakarongo. Ka taea hoki e ia te whakamahara i a koe ahakoa he onge te HAE, kāore tō whānau i te mokemoke.

` Te Angioedema tuku iho, HAE, pupuhi, aukati C1, ngā mate ira, pupuhi i roto i ngā tamariki, angioedema