He maha ngā mamae tinana rerekē e pāngia ana e koe? Me ako atu tātou mō tēnei (Systemic Mastocytosis)!

He whero, he mākūkū rānei ō kiri i ētahi wā? He mamae puku, he mamae upoko rānei tōu e haere mai ana, e haere atu ana? Ki te puta tonu mai ēnei mea, he āhuatanga onge pea tēnei e kiia nei ko te "Systemic Mastocytosis" ka kōrerohia e tātou i tēnei rā. Kaua e manukanuka, ehara tēnei i te mate e pāngia ana e te tini tāngata. Engari he mea nui kia mōhio koe ki tēnei.

Nā, he aha tēnei "Systemic Mastocytosis"?

Ki te kī ngāwari, he mate onge te `(Systemic Mastocytosis)` e pā ana ki ō tātou toto. Ko te mea e tupu ana i roto i tēnei, he momo pūtau motuhake e kiia nei ko `(Mast cells)` i roto i tō tātou tinana ka whanake rerekē. Whakaarohia, he rite ēnei pūtau mast ki ngā hōia ririki i roto i tō tātou tinana. Ko rātou, hei wāhanga o tō tātou pūnaha ārai mate, ka kite, ka whawhai ki ngā whakaeke tauhou (pēnei i ngā iroriki, ngā mate pāwera, me ētahi atu). Ina tae mai tētahi whakaeke tauhou, ka tukuna e ēnei pūtau mast ngā matū pēnei i te `(Histamine)`. Ko tēnei `(Histamine)` te take o aua tohumate mate pāwera (pēnei i te whero, te mākūkū, te pupuhi). I te nuinga o te wā, ka mutu te tuku a ngā pūtau mast i tēnei `(Histamine)` ina oti te mahi. Heoi, i roto i te tinana o te tangata he `(Systemic Mastocytosis)`, ka whanake nui ēnei pūtau mast rerekē, ka tukuna tonutia te `(Histamine)`. He rite ki te tangata i waiho i te puna wai, kātahi ka wareware ki te whakaweto. Koinei te take e mau tonu ai ngā tohumate mate pāwera. Ka pā ki tō tātou kiri anake, engari ki ō tātou whekau o roto pēnei i te ate, te whēkau, me ngā whekau. Ko tēnei mate, e kiia nei ko te "Systemic Mastocytosis," e kiia ana ka pā ki te 13 noa iho i roto i te 100,000 tāngata o te ao. Ko te tikanga he tino onge. Heoi, ko te hunga whai mate pēnei he nui ake te mōrearea ki te anaphylaxis ( he mate pāwera kino, ohorere, e morearea ana ki te ora). He tino onge, ka huri te "Systemic Mastocytosis" hei mate pukupuku. Kāore e taea e ngā tākuta te whakaora katoa i tēnei mate. Heoi, he maimoatanga hei āwhina i a koe ki te whakahaere i ō tohu me te noho i te oranga noa.

He aha ngā momo matua o te `(Systemic Mastocytosis)`?

E ono ngā momo matua o te "Systemic Mastocytosis". He rerekē ngā pānga ki a tātou. I te nuinga o te wā, ki te nui ake ngā pūtau mast rerekē i roto i tō tinana, ka nui ake te tūponotanga ka pāngia koe e ngā raruraru. Me titiro tātou ki aua momo: 1. Momo puhoi te tipu ("Indolent systemic mastocytosis"):

• Koinei te momo tino noa. Pērā i te rākau e puhoi ana te tipu, ka roa pea ngā tau ka puta mai ngā tohu. Ka piki haere te maha o ngā pūtau mast rerekē i roto i te wā. Ka puta pea ngā tohu ki te kiri, te ate , te whēkau , me te pūnaha gastrointestinal (arā, ngā whekau).

2. Momo āhua matatau ake (`Systemic smoldering mastocytosis`):

• I tēnei momo, ka nui te kohikohinga o ngā pūtau mast rerekē i roto i te ate me te whēkau. I roto i te wā, ka pupuhi pea te ate, te whēkau rānei. Whakaarohia he rite ki te mea ka tupu ina kīkī rawa te whare iti.

3. Momo e haere tahi mai ana me ētahi atu mate toto (`Systemic mastocytosis with associated hematologic neoplasm`):

• Tērā pea ka pāngia te hunga whai mate toto pēnei e ētahi atu mate, pērā i ngā mate pukupuku myeloproliferative me te mate myelodysplastic . Tata ki te kotahi i roto i te rima ngā tāngata whai Systemic Mastocytosis ka pāngia e tēnei momo.

4. Momo kino rawa atu (`Mastocytosis pūnaha kaha`):

• He ahua kino ake tēnei nā te mea ka pā ki te hinu wheua (te wāhi e hangaia ai ngā pūtau toto) me ngā wheua. Ka taea e ngā pūtau mast rerekē te tipu i roto i ngā wheua, ka ngoikore ai, ka ngawari ai te whati.

5. Te momo ka huri hei leukemia (`Mast cell leukemia`):

• He tino onge, ka taea e te Systemic Mastocytosis te whanake hei āhuatanga e kiia nei ko te Mast Cell Leukemia . He momo Acute Myeloid Leukemia (AML) tēnei.

6. Momo pukupuku (`Mast cell sarcoma`):

• Koinei te wā e pakaru ai ngā kiko o te tinana i ngā pukupuku (ngā pūtau mast rerekē). He tokoiti noa iho te hunga e pāngia ana e te "Systemic Mastocytosis" ka pāngia e tēnei āhuatanga e kiia nei ko te "Mast cell sarcoma".

He aha ngā tohu o tēnei mate (Systemic Mastocytosis)?

Ka rerekē ngā tohu i ia tangata, i ia tangata. Kei te wāhi o te tinana e tino kitea ana ngā pūtau mast rerekē. Hei tauira, ki te nui ngā pūtau mast i roto i tō puku, ka pāngia pea koe e ngā mate pukupuku me te mamae o te puku. Waihoki, ki te kohikohi ngā pūtau mast i roto i tō hinu wheua, ka pāngia te hanga o ngā pūtau toto. Anei ētahi tohu noa:

• Te kore toto ( anemia )
• Mamae wheua
• Te toto nui rawa (hei tauira, he nui te toto mai i tētahi patunga iti)
• Te ngenge tonu (‘ Fatigue’ )
• Kiri whero
• Ngā tere o te ngakau
• Ngā whewhe (te mākūkū, te pupuhi o te kiri)
• Kiri mākūkū
• Ngā huringa wairua, te pouri rāneipouri
• Ngā ira pango, mākūkū i runga i te kiri (`Urticaria pigmentosa`)

I ētahi wā, ka pāngia pea te hunga whai Systemic Mastocytosis e ētahi o ēnei tohu i te wā kotahi. Ka kiia tēnei e ngā tākuta he "mastocytosis attack" he "flare" rānei.

He mea nui: Ehara i te mea ko te hunga katoa e pāngia ana e ēnei tohu he `(Systemic Mastocytosis).` Heoi, ki te pāngia koe e ētahi o ēnei tohu i te wā kotahi, ā, ka mau tonu, me toro atu koe ki te tākuta mō ētahi tohutohu.

He aha i puta ai tēnei `(Systemic Mastocytosis)`?

Kua kitea e ngā rangahau tata ki te 80% o te hunga whai `(Systemic Mastocytosis)` he `rerenga ira KIT` . Ka āwhina tēnei ira `KIT` i ētahi momo pūtau i roto i tō tātou tinana, inā koa ngā pūtau toto me ngā pūtau mast, kia whanake. Ka puta tēnei rerekētanga ira i muri i te haputanga i roto i te kōpū. Ko te tikanga ehara i te mea tuku iho.

He aha ngā mea e whakanui ana, e whakaoho ana rānei i te mate ('Ngā Whakaoho')?

He maha ngā mea ka puta ai ngā tohu o te "Systemic Mastocytosis." Kāore e pāngia te katoa e ngā mea ōrite. Heoi, he iti noa ngā mea e kitea whānuitia ana:

• Waipiro
• Ngā kai werawera
• Ētahi rongoā: Hei tauira , ngā NSAID ( ngā rongoā whakamamae mamae), ngā rongoā whakangāwari uaua, me te rongoā whakamoe.
• Te whakakori tinana me te mahi ā-tinana ( mō ētahi anake)
• Ngau a ngā pepeke (ina koa ko ngā pōpokorua, ngā wāpi, me ngā pī)
• Te ahotea tinana, te ahotea hinengaro rānei
• Te mirimiri, te waku rānei i tō kiri
• Ngā huringa ohorere o te pāmahana (hei tauira, te peke ohorere ki te wai matao)

Mena kei a koe tēnei mate, he mea tino nui kia pātai koe ki tō tākuta he aha ō ngoikoretanga me ngā mea me karo e koe.

Me pēhea te tautuhi tika i tēnei mate? (Tautuhinga)

Ina haere koe ki te tākuta, ko te mea tuatahi ka mahia e rātou ko te tirotiro tinana i a koe. Ka pātai rātou mō ō tohu me ō mate o mua. Kātahi ka mahia pea e rātou tētahi, ētahi rānei o ngā whakamātautau e whai ake nei hei whakatau mēnā he Systemic Mastocytosis tōu:

• Ngā whakamātautau toto: Mā tēnei ka tirohia te nui o te tryptase i roto i tō toto. He whākōkī motuhake te tryptase ka tukuna e ngā pūtau mast ina aro atu ki ngā matū ke.
• Ngā matawai wheua: Tirohia mēnā he kino te wheua.
• Ko te koiora wheua wheua: Ko te tango i tētahi tauira iti o tō wheua wheua ka whakamātautau kia kitea ai mēnā he pūtau mast rerekē kei roto, me te maha hoki.
• Ngā whakamātautau ira: Tirohia mēnā he whakarerekētanga ira kei roto i te ira 'KIT' kua whakahuatia i mua.

Me pēhea te rongoā?

Mā ngā tākuta e rongoā te Systemic Mastocytosis mā te whakahaere i ngā tohu me ngā raruraru. Hei tauira, ki te nui te waikawa o te puku, ka tohua pea e rātou ngā rongoā aukati H2 (he momo rongoā ārai waikawa). Ki te mea ko te anemia te hua o te mate, ka rongoāhia. Anei ētahi o ngā maimoatanga matua:

• Ngā rongoā ārai mate kiri: Ka whakaiti i ngā tohu kiri pēnei i te mākūkū me te whero.
• Corticosteroids: Whakaiti i te pupuhi me te mumura i roto i te tinana.
• Ngā Bisphosphonates: Ka āwhina i te whakapakari i ngā wheua mēnā he ngoikore.
• Whakaora arotahi: Kei roto i tēnei ko te arotahi ki tētahi pūmua motuhake e kitea ana i roto i ngā pūtau mast rerekē.
• Te rongoā matū: Ka whakamahia anake tēnei mēnā kua huri te Mastocytosis Pūnaha hei mate pukupuku.
• Te tango i te whēkau: Mena he tino pupuhi te whēkau, ka taea te tango ma te pokanga.
• Te whakawhitinga wheua wheua: Ka mahia tēnei mō te hunga he tino kino te mate, mō te hunga rānei kei te wāhanga whakamutunga.

Kia maumahara: Ki te pāngia koe e te Systemic Mastocytosis, me mau tonu he EpiPen . He rongoā te EpiPen ka taea te whakamahi tonu mēnā ka pāngia koe e te mate pāwera nui, pērā i te Anaphylaxis i whakahuatia ake nei.

Kāore e taea te ārai i tēnei "Systemic Mastocytosis"?

E ai ki tā tātou i kōrero ai i mua ake nei, ko te "Systemic Mastocytosis" ka puta mai i tētahi whakarerekētanga ira. Nō reira, kāore e taea te ārai i tōna whanaketanga. Heoi, ka taea e koe te whakahaere i te mate mā te karo i ngā "mea whakaoho" e whakanui ana i ngā tohu.

He aha te momo āmua mō te tangata e pāngia ana e te `(Systemic Mastocytosis)`?

Kei te momo `(Systemic Mastocytosis)` tō mate tēnei. Ko te hunga e pāngia ana e te momo puhoi-tipu (`Indolent systemic mastocytosis`) ka taea te whakahaere pai i te mate mā te rongoā. Ka taea e rātou te ora i te roanga o te oranga noa. Heoi, ko te hunga e pāngia ana e ētahi atu momo kino ka poto ake te roanga o te oranga. Heoi, ka taea te whakaora katoa i te `(Systemic Mastocytosis)` mā te rongoā e kiia nei ko te ``Bone hunger transplant``. Heoi, ko te hunga kei roto i ngā wāhanga tino kino o te mate anake ka tukuna taua rongoā e ngā tākuta.

Me pēhea taku tiaki i a au anō i a au e noho tahi ana me te `(Systemic Mastocytosis)`?

Ko te mōhio ki ngā mea e whakaoho ana i ō tohumate, me te karo i aua tohumate, koia te mea nui rawa atu ka taea e koe te mahi ina noho koe me te Systemic Mastocytosis. Mena kei a koe tēnei mate, kia mōhio ki ēnei mea:

• Me mau tonu he EpiPen. Me tere te rongoā i tētahi mate pāwera kino.
• Kia mōhio koe ki ngā mea e whakaoho ana i tō riri, ā, me karo i aua mea i ngā wā katoa e taea ana.
• Whakahaerehia te ahotea. Whakamahia te whakaaroaro, ētahi atu tikanga aroātahi rānei.
• Mauria he poroporo whakatūpato hauora e whakarārangi ana i ngā rongoā kāore e taea e koe te tango. He mea tino nui tēnei nā te mea ka āwhina i ngā tākuta ki te mōhio ki tō āhua mēnā kāore e taea e koe te kōrero i roto i tētahi āhuatanga ohorere.

Āhea ahau me kite i te tākuta?

Mena kei a koe te Systemic Mastocytosis, me toro atu koe ki tō tākuta mō ngā tirotiro auau. Me whakamōhio atu hoki ki tō tākuta mēnā ka puta he tohu hou, mēnā rānei kei te kino haere tetahi o ō tohu.

He aha ngā pātai me pātai atu e au ki te tākuta?

Nā te mea he mate onge te Systemic Mastocytosis, tera pea he maha āu pātai. Anei ētahi pātai hei pātai atu ki tō tākuta:

• He aha te momo "Systemic Mastocytosis" kei a au?
• He aha atu ngā tohu e tohu ana kei te kino haere tōku āhua?
• He aha ngā maimoatanga e wātea ana mō taku momo?
• Ka whakakorea rawatia rānei aku tohu e te maimoatanga?
• He whakamātautau haumanu kei reira ka taea e au te whakauru atu?

He wheako whakarerekē te tātaritanga o te Systemic Mastocytosis. Me toro atu koe ki tō tākuta i ia wā, me whakamātau toto, me ako hoki ki te mōhio me te karo i ngā mea e whakaoho ana i te mate. He whakarerekētanga nui te noho me tēnei mate. Nō reira, kia mau tonu te whanaungatanga pai ki tō tākuta. Kei reira rātou hei āwhina i a koe. Ka whakaratohia e rātou he mōhiohio me ngā rōpū tautoko hei āwhina i a koe. Mā ēnei koe e whakamana ki te whakatau i ngā whakatau mō tō hauora.

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

Āe, kua maha ā tātou kōrero mō te `(Systemic Mastocytosis)`, kāore? Kia maumahara, ahakoa he mate onge tēnei, he mea nui kia mōhio ki tēnei.

Ko te mea nui ko te toro atu ki te tākuta mēnā kei a koe ēnei tohu. Mēnā ka kitea wawe te mate, ka taea te tīmata i te maimoatanga, ā, ka taea te whakahaere i ngā tohu.

Kāore koe i te mokemoke. Kei reira ō tākuta, tō whānau, me ō hoa hei āwhina i a koe. Mā te mōhio ki ngā kōrero tika me te whai i ngā tohutohu a tō tākuta, ka taea e koe te noho pai me tēnei mate. Kia marino, kia whakaaro pai hoki!