He aha te C1-Esterase Inhibitor? Ngā tohumate, ngā take, me te rongoā

Ko te C1-esterase inhibitor he rongoā whakaora-ora e whakamahia ana hei rongoā i te hereditary angioedema, he mate ira onge e puta ai te pupuhi ohorere, kaha. Ka āwhina tēnei rongoā whakakapi pūmua i tō tinana ki te whakahaere i te mumura me te ārai i ngā wā pupuhi mōrearea ka pā ki tō kanohi, korokoro, ringa, waewae, me ngā okana o roto.

Mēnā kua tohua tēnei rongoā ki a koe, ki tētahi rānei e arohaina ana e koe, kei te tūpono koe ki tētahi āhuatanga uaua e hiahia ana kia āta whakahaere. Mā te māramatanga ki te pēhea te mahi o tēnei rongoā ka āwhina koe ki te whai i te māia ki tō mahere tiaki me te mōhio ki ngā mea e tūmanakohia ana i te wā o te rongoā.

He aha te C1-Esterase Inhibitor?

Ko te C1-esterase inhibitor he pūmua e hangaia ana e tō tinana hei whakahaere i te mumura me te ārai i te pupuhi nui. Ki te kore koe e nui tēnei pūmua, ki te kore rānei e mahi tika ana, ka puta pea te hereditary angioedema (HAE).

Ka whakakapi te rongoā i te pūmua ngaro, hē rānei i roto i tō toto. Nō te plasma tangata i tūao mai i te tangata, i āta tukatukahia, i purea hoki kia noho haumaru mō te whakamahi rongoā. Ka whiwhi koe i tēnei mā te IV i roto i tō uaua, mā te werohanga rānei i raro i tō kiri, i runga i ō hiahia motuhake me te tūtohutanga a tō tākuta.

Ka mahi tēnei rongoā mā te whakahoki mai i te taurite tūturu o ngā pūmua i roto i tō toto e whakahaere ana i te pupuhi. Whakaarohia he hoatu ki tō tinana ngā taputapu e hiahiatia ana hei ārai i ngā wā pupuhi mōrearea i mua i te tīmatanga, hei aukati rānei i a rātou i te tīmatanga.

He pēhea te rongoā ki te C1-Esterase Inhibitor?

Ko te nuinga o te tangata e pai ana ki te rongoā C1-esterase inhibitor. I te wā o te whakauru IV, ka noho koe i te pai i te wā e uru ana te rongoā ki tō toto i te 10 ki te 30 meneti. Ka pōturi pea koe i te wā e kuhu ana te ngira, engari ko te whakauru tonu he kore mamae.

Ka pā mai ētahi pānga iti ki ētahi tāngata i te wā, i muri rānei i te rongoā. Ko ētahi o ēnei ko te ānini, te whakapairuaki, te ngenge rānei. Ka kite pea koe i te whero, te pūhi rānei i te wāhi werohanga mēnā kei te whiwhi koe i te momo subcutaneous. He ngawari noa iho ēnei pānga, ā, ka ngaro noa iho i roto i ngā hāora ruarua.

Ka mātakitaki pū te rōpū hauora i a koe i te wā o te rongoā, ina koa i ngā horopeta tuatahi. E hiahia ana rātou ki te mōhio kei te pai tō urupare, ā, kei te pai koe i te wā o te tukanga.

He aha te take e hiahiatia ai te rongoā C1-Esterase Inhibitor?

Ko te take tuatahi e hiahia ai koe i tēnei rongoā ko te angioedema tuku iho, he āhua ira tēnei ka riro mai i ō mātua. Ki te HAE, kāore te tinana e hanga ana i te pūmua C1-esterase inhibitor, kāore rānei te pūmua e mahi tika ana.

Ka puta tēnei ngoikoretanga pūmua nā te huringa i ō ira. E toru ngā momo HAE matua, ā, he rerekē te pānga o ia momo ki te pūmua C1-esterase inhibitor. Ko ngā momo tino noa ko te iti rawa o te pūmua, he pūmua rānei kāore e mahi tika ana.

I ngā wā onge, ka hiahia pea te tāngata i tēnei rongoā mō te angioedema i riro mai, ka puta mai i muri mai i te oranga nā ētahi atu āhua hauora. Ka puta tēnei i te wā e pokanoa ana ētahi mate, rongoā rānei ki te kaha o tō tinana ki te hanga, ki te whakamahi rānei i te C1-esterase inhibitor.

He aha ngā āhua e hiahiatia ana te rongoā C1-Esterase Inhibitor?

Ko te angioedema tuku iho te āhua matua e hiahiatia ana te rongoā C1-esterase inhibitor. Ko tēnei mate ira e pā mai ana i ngā wā o te pūhi nui e pā ana ki tō kanohi, ngutu, arero, korokoro, ringa, waewae, me ngā ira. Ka puta hoki te pūhi i ō whekau, e pā mai ana i te mamae nui o te puku.

Ka tohua pea tēnei rongoā e tō tākuta mō ngā take e rua. Ka whakamahia pea e koe hei ārai i ngā wāhanga pūhihi kia kore e puta (e kīia ana ko te ārai), hei rongoā rānei i tētahi whakaeke kua tīmata kē (e kīia ana ko te rongoā koi). Ko ētahi tāngata e hiahia ana ki ngā momo rongoā e rua i runga i te kaha o tō rātou āhua.

I ngā wā onge, ka whakamahia pea e ngā tākuta te aukati C1-esterase hei rongoā i te angioedema i riro mai. Ka puta tēnei āhua ina whai koe i ētahi mate ārai-a-tinana, te mate pukupuku toto, ka tango rānei i ētahi rongoā motuhake e pokanoa ana ki ngā mana whakahaere mumura o tō tinana.

Ka taea e te Hereditary Angioedema te pai ake me te kore rongoā?

Ko te mea pouri, kāore te hereditary angioedema e ngaro noa iho nā te mea he āhua ira tēnei i whānau mai ai koe. Ki te kore he rongoā tika, ka haere tonu ngā wāhanga pūhihi, ā, ka nui ake pea te auau, te kaha rānei i te roanga o te wā.

Ka roa pea ngā wāhanga pūhihi i te maha o ngā hāora ki te maha o ngā rā ki te kore e rongoā. Ahakoa te ahua ka whakatau ētahi whakaeke i a rātou anō, ko te mea tūturu ko te whakautu mumura tūturu o tō tinana e marino ana, ehara i te rongoā.

Ko te āwangawanga tino nui ko te pūhihi korokoro, ka taea te aukati i tō ara rererangi, ka noho mōrearea ki te ora i roto i ngā meneti. Koinei te take he mea nui te whai i te rongoā tika pēnei i te aukati C1-esterase mō te whakahaere i tēnei āhua i runga i te haumaru.

Me pēhea e tautoko ai koe i tō rongoā i te kāinga?

Ahakoa kāore e taea e koe te rongoā i te hereditary angioedema i te kāinga me te kore rongoā, he maha ngā mea ka taea e koe hei tautoko i tō hauora whānui, ā, pea ka whakaiti i te auau o ngā whakaeke. He mea nui te mahi tahi me tō rōpū hauora mō ngā hua pai.

Anei ētahi mehua tautoko ka āwhina pea i a koe kia nui ake te mana whakahaere i tō āhua:

• Puritia he rārangi taipitopito mō ō tohumate, ngā mea e pā ana, me ngā rongoā hei āwhina i te tautuhi i ngā tauira
• Ako ki te mōhio ki ngā tohu whakatūpato wawe o te whakaeke kia taea ai e koe te rapu rongoā wawe
• Aukati i ngā mea e mōhiotia ana e pā ana, pēnei i ētahi kai, te pēhanga, rānei ngā rongoā ka pā mai te whakaeke
• Me mōhio ngā mema o te whānau me ngā hoa tata ki tō āhuatanga me te mahere ohorere
• Mau tonu ngā kōrero whakapā ohorere me te tohu whakatūpato hauora i ngā wā katoa
• Whakamahia ngā tikanga whakaiti pēhanga pēnei i te manawa hōhonu, te whakaaroaro rānei, nā te mea ka pā mai te pēhanga i ngā whakaeke

Kia mahara ko ēnei mahi i te kāinga hei tautoko, kaua hei whakakapi, i tō rongoā i whakaritea. Me whai tonu i ngā tohutohu a tō tākuta mō te wā me te pēhea te whakamahi i tō rongoā aukati C1-esterase.

He aha te rongoā hauora mō te Hereditary Angioedema?

Ko te aukati C1-esterase tētahi o ngā rongoā maha kua whakaaetia e te FDA mō te hereditary angioedema. Ka mahi tō tākuta ki a koe ki te whakatau i te huarahi rongoā pai rawa atu i runga i te auau o ō whakaeke, te kaha o ēnei, me ō hiahia oranga ake.

Mō te aukati i ngā whakaeke, ka whiwhi pea koe i ngā whakaurunga auau o te aukati C1-esterase i ia rā, i ia wiki rānei. Ka taea e ētahi tāngata te ako ki te tuku i a rātou anō ngā werohanga i raro i te kiri i te kāinga, e whakarato ana i te nui ake o te ngawari me te mana motuhake i te whakahaere i tō rātou āhuatanga.

I te wā o te whakaeke nui, ka hiahia koe ki te rongoā i te wā tonu. Ka tohutohu pea tō tākuta i te horopeta teitei ake o te aukati C1-esterase, ka whakakotahi rānei ki ētahi atu rongoā. Ko ētahi atu kōwhiringa rongoā ko ngā momo rongoā rerekē e mahi ana mā ngā ara rerekē ki te whakahaere i te pūhi.

Ka tino whai takitahi tō mahere rongoā. Ko ētahi tāngata e hiahia ana anake ki te rongoā i runga i te tono mō ngā whakaeke, i te mea ka whai hua ētahi atu i te rongoā ārai auau. Ka aroturuki tō tākuta i tō urupare me te whakatika i tō rongoā i te wā e tika ana i te roanga o te wā.

Āhea koe pōti ai ki te kite i te tākuta mō te Hereditary Angioedema?

Mēnā kei te pā mai te pūpūhā e kore e whakamāramatia, ina koa ki tō mata, ngutu, arero, korokoro rānei, me rapu tonu koe i te āwhina hauora. Ka mōrearea te pūpūhā o te korokoro ki te ora i te wā poto, no reira kaua e tatari kia pai ake anō.

Me whakapā atu anō koe ki tō tākuta mēnā kua tāutuhia koe ki te HAE, me te kite i ngā huringa i roto i ō tohumate. Ka uru pea tēnei ki ngā whakaekenga auau, ngā momo pūpūhā rerekē, rānei ngā whakaekenga e kore e aro ki tō rongoā noa i te pai o mua.

Anei ngā āhuatanga motuhake e hiahia ana ki te āwhina hauora tonu:

• Tetahi pūpūhā o tō korokoro, arero, rānei roto o tō waha
• Te uaua ki te hā, te horo, rānei te kōrero
• Te mamae puku nui me te whakapairuaki me te ruaki
• Te pūpūhā o te mata e kino haere ana
• Ngā tohu o te urupare pānga ki tō rongoā

Mēnā kei te whakahaere koe i te HAE me te aukati C1-esterase, kia mau tonu ngā tūpono whai-ake auau ki tō tākuta. Mā rātou e aroturuki tō urupare ki te rongoā, me te mātakitaki mō ngā pānga taha, ngā raruraru rānei.

He aha ngā āhuatanga tūpono mō te whakawhanake i te Hereditary Angioedema?

Ko te āhuatanga tūpono tuatahi mō te hereditary angioedema ko te whai mātua e whai ana i te āhuatanga. He mate ira autosomal dominant te HAE, ko te tikanga me tuku noa e koe tētahi kape o te ira kua huri mai i tētahi o ngā mātua kia whakawhanake i te āhuatanga.

Mēnā he HAE tētahi o ō mātua, he 50% tō tūpono ki te tuku i te āhuatanga. Heoi, tata ki te 20-25% o ngā tāngata e whai ana i te HAE kāore he hītori whānau o te āhuatanga, ko te tikanga i puta te huringa ira i te wā tonu.

He maha ngā āhuatanga ka pūtake i ngā whakaeke i roto i ngā tāngata kua whai HAE. Kāore ēnei pūtake e whakaputa i te āhua tonu, engari ka mōhiotia e rātou ngā tohumate. Ko te pōhēhē, ētahi rongoā, ngā huringa homoni, ngā mate, me ngā whara tinana ka pūtake i ngā wā o te pūhihi.

Ka mōhiotia pea e ngā wāhine whai HAE ka huri ā rātou tohumate i te wā o te taiohitanga, te hapū, te pāhau rānei nā te huringa homoni. Ētahi wāhine ka kite hoki ka nui ake, ka kaha ake rānei ā rātou whakaeke i te wā e tango ana i ngā rongoā kei roto te estrogen.

He aha ngā raruraru ka puta mai i te Hereditary Angioedema?

Ko te raruraru tino nui o te hereditary angioedema ko te aukati i te ara rererangi o runga mai i te pūhihi o te korokoro. Ka taea tēnei te puta ohorere, ka tere te haere, ka aukati pea i tō kaha ki te hā i roto i ngā meneti ki ngā hāora.

Ka taea hoki e ngā whakaeke puku te whakaputa i ngā raruraru nui. Ko te pūhihi i roto i tō pakitara whekau ka whakaputa i te mamae nui, te whakapairuaki, te ruaki, me te korere. Ētahi tāngata e whakaahua ana i te mamae he rite ki te appendicitis, ka puta pea tēnei i ngā mahi pokanga kore e tika ana mēnā kāore ngā tākuta i te mōhio ki te HAE.

Anei ngā raruraru ka puta mai i te HAE kāore i rongoatia, i te HAE rānei kāore i te pai te whakahaere:

• Te aukati i te ara rererangi e hiahia ana ki te intubation ohorere, ki te tracheostomy rānei
• Te maroke nui mai i ngā whakaeke whekau
• Ngā mahi pokanga kore e tika ana nā te hē o te tātaritanga o te mamae puku
• Te pānga hinengaro mai i te noho me ngā tohumate e kore e taea te matapae, e mōrearea ana ki te ora
• Ngā here pāpori me ngā mahi nā te mataku o ngā whakaeke

Mā te rongoā me te whakahaere tika, ka taea e te nuinga o ngā tāngata whai HAE te noho i ngā ora noa, kaha. Ko te kī ko te mahi tahi me ngā kaiwhakarato hauora mōhio, me te whai i ngā rongoā tika pērā i te C1-esterase inhibitor.

He haumaru te C1-Esterase Inhibitor mō te whakamahi wā-roa?

Kua whakamahia te aukati C1-esterase mo nga tau maha ki te rongoa i te angioedema tuku iho. He pai te whakamanawanui o te nuinga o nga tangata ki te rongoa mo te wa roa, he iti noa nga paanga taha. I hangaia te rongoa mai i te plasma tangata, engari ka haere i roto i nga tukanga purenga nui me te whakakore i te huaketo kia noho haumaru ai.

Ko nga paanga taha tino noa he ngawari, kei roto ko te mahunga, te whakapairuaki, me nga tauhohenga i te waahi werohanga. He onge nga tauhohenga mate pāwera nui engari ka taea, koinei te take ka tino aro turuki te roopu hauora i a koe, ina koa i nga maimoatanga tuatahi.

Na te mea ka whakakapi tenei rongoa i te pūmua me hanga e to tinana, karekau e puta nga momo paanga taha mo te wa roa ka kitea e koe me etahi atu rongoa. Ka tirotiro tonu to taakuta i a koe i nga wa katoa kia mohio kei te pai tonu te rongoa mo koe.

He aha te mea ka pohehehia ai te Angioedema Tuku Iho?

He maha nga wa ka pohehehia te angioedema tuku iho na te mea ka rite ana tohu ki te maha atu o nga ahuatanga. Ka pohehe pea te pupuhi he tauhohenga mate pāwera, ina koa ka pa ki to kanohi, ki ou ngutu ranei. Heoi, ko te pupuhi HAE karekau e mānawa, karekau hoki e aro ki nga antihistamines, ki nga steroids ranei.

He maha nga wa ka pohehehia nga whakaeke puku he appendicitis, he raruraru gallbladder, etahi atu ohorere gastrointestinal ranei. Ko te mamae nui o te puku, te whakapairuaki, me te ruaki ka rite tonu ki enei atu ahuatanga, i etahi wa ka arahi ki te pokanga kore.

Ko etahi atu ahuatanga ka pohehehia pea te HAE ko:

• Nga tauhohenga mate pāwera ki nga kai, rongoa, ki nga mea whakaoho taiao ranei
• Cellulitis, etahi atu mate kiri ranei ka puta te pupuhi ki nga ringa, waewae ranei
• Nga raruraru whatukuhu ina puta te pupuhi o te kanohi
• Nga raruraru ngakau ina pa te pupuhi ki nga waewae
• Nga ahuatanga autoimmune e puta ai te mumura

He mea nui te whiwhi i te tātaritanga tika, nā te mea me whai rongoā motuhake te HAE. Mēnā kua pā koe i ngā wāhanga pōhūhā e kore e whakamāramahia, otirā mēnā kei roto i tō whānau, pātai atu ki tō tākuta mō te whakamātautau mō te hereditary angioedema.

Ngā Pātai Auau mō te C1-Esterase Inhibitor

P: Kia pēhea te tere o te mahi a te C1-esterase inhibitor?

Ko te tikanga ka tīmata te mahi a te C1-esterase inhibitor i roto i te 15-30 meneti ka hāpaihia mā te uaua mō te whakaeke koi. Ka tīmata pea koe ki te kite i te whakapai ake i ō tohu i roto i te haora, ahakoa ka roa te whakaoti i ngā haora. Mō te werohanga i raro i te kiri, he paku pōturi pea te tīmatanga engari he whai hua tonu.

P: Ka taea e au te haereere me te rongoā C1-esterase inhibitor?

Āe, ka taea e koe te haereere me tō rongoā, engari me whakamahere i mua. Mauhia tētahi reta mai i tō tākuta e whakamārama ana i tō āhuatanga me te hiahia mō te rongoā. Puritia tō rongoā i roto i ōna pōkai taketake, me te kawe i ngā taonga anō mō ngā whakaroa. Mō te haereere ā-ao, rangahaua ngā ture mō te kawe rongoā ki tō whenua haerenga.

P: Ka hipokina e te inihua te rongoā C1-esterase inhibitor?

Ko te nuinga o ngā mahere inihua e hipoki ana i te C1-esterase inhibitor mō te hereditary angioedema, ahakoa me whai whakaaetanga i mua. Mahi tahi me te tari o tō tākuta me te kaihanga rongoā, he maha ngā mea e tuku ana i ngā hōtaka āwhina tūroro hei āwhina i ngā utu. He utu nui te rongoā, engari kei te wātea te hipoki mō tēnei rongoā e tika ana i te rongoā.

P: Ka taea e ngā tamariki te whiwhi rongoā C1-esterase inhibitor?

Āe, ka taea te whakamahi i te C1-esterase inhibitor i roto i ngā tamariki e whai hereditary angioedema ana. Ka whakatikahia te inenga i runga i te taumaha o te tamaiti, ā, kua whakaaturia te rongoā he haumaru, he whai hua hoki i roto i ngā tūroro tamariki. Ka hiahia pea ngā tamariki ki te tautoko me te whakarite anō mō te rongoā, engari te nuinga ka pai te aro ki a ia.

P: He here kai anō kei te wā e tango ana i te C1-esterase inhibitor?

Kāore he here kai motuhake e pā ana ki te aukati C1-esterase. Heoi, e kitea ana e ētahi tāngata e mate ana i te HAE ka taea e ētahi kai te whakaoho i ā rātou whakaeke, nā reira pea me pupuri koe i tētahi rārangi kai hei tautuhi i ngā mea e whakaoho ana i a koe. Me whai tonu i ngā tohutohu a tō tākuta mō te kai me ngā whakarerekētanga o te noho e āwhina ana ki te whakahaere i tō āhuatanga.