Kei te pāngia tonutia tō tamaiti e te mate? Tērā pea ko te XLA (X-Linked Agammaglobulinemia). Me kōrero tāua!

Ahakoa e whakaaro ana tātou he mea noa te pāngia pinepine o ā tātou tamariki e te mate, he raruraru nui tēnei mō ētahi tamariki. Ina koa mēnā kei te pāngia tonutia te tamaiti e ngā mate kitakita, tera pea he mate ira onge. I tēnei rā ka kōrero tātou mō tēnei momo mate.

He aha te XLA (X-Linked Agammaglobulinemia)?

Āe, he aha te XLA (X-linked agammaglobulinemia)? He kupu ngāwari noa iho, he mate ira . He ope taua tino nui tō tō tātou tinana hei whawhai i te mate, koia tō tātou pūnaha ārai mate . Ko tētahi momo pūtau motuhake i roto i tēnei pūnaha e kiia ana ko ngā pūtau-B . Ko ēnei pūtau-B te mea e hanga ana i ngā pūmua e kiia nei ko ngā paturopi hei whawhai i te mate ina pāngia ō tātou tinana e te mate. He rite te mahi a ēnei paturopi ki ngā hōia e tiaki ana i tō tātou whenua.

Kāore te tangata whai XLA (X-Linked Agammaglobulinemia) e whakaputa tika i ēnei pūtau-B. He iti noa iho rānei ā rātou e whakaputa. Nā, he aha te mea ka puta ina kore e taea e koe te hanga paturopi? Ka tere te pāngia e te mate, ā, he maha ngā wā ka pāngia koe e te mate. Waihoki, ko ngā kiko o roto i tō tātou tinana e pā ana ki te pūnaha ārai mate, pērā i ngā kōpū lymph , ngā tonsil, me ngā adenoids, kāore e whanake tika, ā, i ētahi wā kāore e hangaia. Ko te nuinga o te wā ka kitea tēnei mate i roto i ngā tama . Ka kōrerohia e tātou te take i muri mai.

Ko tēnei āhuatanga, e kiia nei ko te XLA (X-Linked Agammaglobulinemia), e mōhiotia ana hoki e ētahi atu ingoa:

• te agammaglobulinemia a Bruton
• Te agammaglobulinemia whānau
• Te ngoikore o te hāora

Heoi, ko te ingoa Hypogammaglobulinemia e whakamahia ana mō tētahi atu mate rite. Ko te CVID (Common Variable Immunodeficiency) tēnā. Ko te CVID (Common Variable Immunodeficiency) kāore i te tino kino pērā i te XLA (X-Linked Agammaglobulinemia), ā, ka kitea te nuinga i te pakeketanga. Heoi, ko ngā tamariki e pāngia ana e te XLA (X-Linked Agammaglobulinemia) ka kitea i mua i te kotahi tau te pakeke, i te wā taitamarikitanga rānei.

He aha te rerekētanga i waenga i te XLA (X-Linked Agammaglobulinemia) me te SCID (Severe Combined Immunodeficiency)?

Tērā pea kei te whakaaro koe mēnā ko te XLA (X-Linked Agammaglobulinemia) tēnei, ko te mate kino rānei kua rongo pea koe e kīia nei ko te SCID (Severe Combined Immunodeficiency) . Kāore, he rerekētanga iti kei waenganui i ngā mea e rua. I roto i te XLA (X-Linked Agammaglobulinemia) , ko ngā pūtau-B i kōrerohia e tātou i mua ake nei ka pāngia. I roto i te SCID (Severe Combined Immunodeficiency) , ko ngā pūtau-T ka pāngia.Ko tētahi atu momo pūtau ārai mate nui e kiia nei ko ngā pūtau-T (i ētahi wā ka pāngia hoki ngā pūtau-B). He āhuatanga ira e rua e ngoikore ai te pūnaha ārai mate, ā, he maha ngā wā ka puta ai te mate. Engari ko te rerekētanga matua i waenga i ngā mea e rua ko te momo pūtau e pāngia ana.

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He pēhea te noa o te XLA (X-Linked Agammaglobulinemia)?

He tino onge tēnei āhuatanga e kiia nei ko te XLA (X-Linked Agammaglobulinemia). Arā, ehara i te mate e pāngia ana e te tini tāngata. Heoi, e ai ki tā mātou i kōrero ai i mua ake nei, he nui ake te kitea i roto i ngā tama tāne . E ai ki ngā tatauranga, tata ki te kotahi i roto i te rua rau mano (200,000) ngā tama tāne ka whānau mai me te XLA (X-Linked Agammaglobulinemia).

He aha ngā tohu o te XLA (X-Linked Agammaglobulinemia)?

Nā te mea kāore i te tino whanake te pūnaha ārai mate o ngā tamariki e pāngia ana e te XLA (X-Linked Agammaglobulinemia), he iti, he ngaro rānei ō rātou kōpū lymph, tonsil, me adenoids . Nā tēnei ka ngāwari rātou ki te pangia e ngā mate kitakita mai i te tamarikitanga. Hei tauira:

• Bronchitis : He mate pāwera tēnei o ngā pūkahukahu, arā, ngā ngongo e anga atu ana ki ngā pukahukahu.
• Ngā mate taringa (Otitis media) : Ngā mate o te taringa waenga.
• Sinusitis : Te mate pāwera o ngā sinuses huri noa i te ihu.
• Pneumonia : He mate kino e pā ana ki ngā pukahukahu.
• Ngā mate puku : Ngā mate pēnei i te mamae o te puku me te korere.

Engari he mea nui kia maumahara ko ngā tamariki whai XLA (X-Linked Agammaglobulinemia) kāore i te tino pāngia e ngā mate huaketo (hei tauira, te Cytomegalovirus (CMV) , te RSV (Respiratory Syncytial Virus) , ngā mate harore rānei. Ko ngā mate kitakita te nuinga o te wā e pāngia ai rātou.

He aha ngā take o te XLA (X-Linked Agammaglobulinemia)?

E ai ki tā mātou i kōrero ai i mua ake nei, he mate ira te XLA (X-Linked Agammaglobulinemia). Ko te tikanga ka riro mai i te tamaiti tēnei mate i te whaea, i te pāpā, i ngā mea e rua rānei. Kei roto i tō tātou tinana he ira e kiia nei ko te ira BTK . Mā tēnei ira e tohutohu tō tātou tinana ki te hanga pūtau-B. E mōhio ana tātou ka hangaia e ngā pūtau-B ngā paturopi me te whawhai ki ngā mate.

Nō reira, ki te puta he panoni, he whakarerekētanga rānei i roto i tēnei ira BTK, kāore e taea te hanga tika i ngā pūtau-B. Kātahi, ko te mea ka tupu, ko te tangata kāore i a ia taua whakarerekētanga ira, kāore e taea te whawhai ki ngā mate pērā i a rātou. Koia te take e pāngia tonutia ai rātou e te mate, ā, i ētahi wā ka pāngia e ngā mate kino ka morearea ai te ora.

He aha te 'X-linked'?

Nā, me titiro tātou ki te tikanga o te kupu 'X-linked'. I ahu mai ō tātou ira katoa i ngā mātua e rua. Kei runga i ngā mea e kiia nei ko ngā chromosomes ēnei ira. Mā ēnei ira e tohutohu ō tātou tinana ki te hanga i ngā pūmua e hiahiatia ana hei mahi. I te nuinga o te wā, ahakoa he huringa kei roto i tētahi ira, kei te ora tonu te tārua o tētahi atu (ko tētahi mai i tētahi atu mātua), kia taea ai e te tinana te mahi tika.

Heoi, kāore i te ōrite ngā ira ira o ngā tāne. Kotahi te ira X me te ira Y. Nō reira, ki te mea he whakarerekētanga ira kei roto i tētahi ira kei runga i tēnei ira X, kāore he tārua kē atu hei whakakapi. Kei runga i tēnei ira X te ira BTK i kōrerohia e tātou. Koia i huaina ai ko 'X-linked'. Nō reira, ki te mea he whakarerekētanga ira kei roto i te ira BTK o tētahi tama kei runga i tana ira X, ka pāngia ia e te XLA (X-Linked Agammaglobulinemia).

E rua ngā ira X o ngā kōtiro. Ahakoa kei a rātou te whakarerekētanga ira e puta ai te XLA (X-Linked Agammaglobulinemia) i tētahi o ā rātou ira X, kei te mahi tonu te ira BTK i tētahi atu ira X, nō reira ka taea e rātou te hanga i te maha o ngā pūtau-B e tika ana. Nō reira, kāore rātou e pangia e te mate, engari ka taea e rātou te kawe i te mate. Ko te tikanga ka taea e rātou te kawe i te ira me te kore e whakaatu i ngā tohu.

He aha ngā āhuatanga mōrearea mō te XLA (X-Linked Agammaglobulinemia)?

Tae noa mai ki tēnei wā, ko te take mōrearea anake e mōhiotia ana mō te whakawhanaketanga o te XLA (X-Linked Agammaglobulinemia) ko te hītori whānau o te mate . Ko te tikanga he mea tuku iho.

Ka taea e ngā kōtiro te pāngia e te XLA (X-Linked Agammaglobulinemia)?

Ae, he tino onge te wā ka pāngia te tamaiti wahine e te XLA (X-Linked Agammaglobulinemia). Engari kia tupu ai tēnā, me mau i ngā mātua e rua te ira X me te ira BTK kua whakarerekētia. Arā, he kaikawe te whaea, ā, he XLA (X-Linked Agammaglobulinemia) anō hoki tō te pāpā. I te nuinga o te wā, ka taea e ngā tamariki wahine te kawe i tēnei whakarerekētanga ira. Kātahi, ahakoa kāore i a rātou te mate, ka taea e rātou te tuku i tēnei ira ki ā rātou tamariki. Mena he tama aua tamariki, tera pea ka pāngia rātou e te XLA (X-Linked Agammaglobulinemia).

He aha ngā raruraru ka pā mai i te XLA (X-Linked Agammaglobulinemia)?

Ko ētahi o ngā raruraru ka pā mai me te XLA (X-Linked Agammaglobulinemia) ko:

• Mate pūkahukahu mau tonu : Ka taea e ngā mate pūkahukahu maha te kino i ngā pūkahukahu i roto i te wā.
• Te horapa o te mate ki ētahi atu wāhanga o te tinana : Hei tauira, ka horapa ngā mate ki te toto (sepsis) ki te roro rānei (meningitis).
• Kei te whakapaetia anō pea he nui ake te mōrearea o ētahi momo mate pukupuku , engari kei te whakahaerehia he rangahau anō mō ​​tēnei.

Me pēhea te tātaritanga o te XLA (X-Linked Agammaglobulinemia)?

Ka taea e te tākuta te whakahaere i ētahi whakamātautau toto hei kimi mēnā kei a koe, kei tāu tamaiti rānei te XLA (X-Linked Agammaglobulinemia). Mēnā ka whakaatuhia e ēnei whakamātautau toto he iti ō pūtau-B, ō paturopi rānei, ka whakahaerehia e tō tākuta he whakamātautau ira . Mā tēnei ka rapua ngā huringa o te ira BTK i roto i tō DNA .

Me pēhea te rongoā i te XLA (X-Linked Agammaglobulinemia)?

Engari, kāore he rongoā mō te XLA (X-Linked Agammaglobulinemia). Heoi, kei reira ngā maimoatanga hei āwhina i te ārai i ngā raruraru nui. Ko ngā mea matua ko:

• Te Whakaora Immunoglobulin Whakakapinga (RIgG) : Kei roto i tēnei ko te tuku paturopi mai i ngā kaituku hauora mā te uaua (IV). Ka hoatu tēnei maimoatanga i te iti rawa kotahi i te marama . Ka āwhina tēnei ki te whakahaere i ngā taumata paturopi iti i roto i te tinana ki tētahi taumata.
• Te rongoā wawe i ngā mate urutā : Ina whakapaetia kei te pāngia koe, tō tamaiti rānei e te mate urutā, ka tīmata tō tākuta ki te rongoā i ngā mate urutā kitakita ki ngā paturopi . He mea nui kia tīmata wawe te rongoā.
• Te karo i ngā werohanga ora : Kaua te hunga whai XLA (X-Linked Agammaglobulinemia) e whiwhi i ngā werohanga ora . Ka taea e ēnei werohanga te whakararu i te mate kino, ā, ka taea te whakararu i te ora. Hei tauira , ko te werohanga MMR (rewharewha, rewharewha, rubela) , te werohanga rewharewha - varicella, me te werohanga polio ā-waha . Nō reira, he mea nui kia kōrero koe ki tō tākuta mō tēnei mea, kia tino mōhio hoki koe.

He aha ngā mea e tika ana kia tumanakohia e te tangata e pangia ana e te XLA (X-Linked Agammaglobulinemia)?

Me tango rongoā te hunga whai XLA (X-Linked Agammaglobulinemia) mō te toenga o ō rātou oranga . Hei whakaiti i te tūponotanga o te whakawhanake i ngā mate. Me pupuri tonu e rātou he whanaungatanga tata ki tā rātou tākuta, ā, me rapu maimoatanga i te wā tonu ka pā mai he mate. Tērā pea ka ngaro atu i a koe, i tāu tamaiti rānei whai XLA (X-Linked Agammaglobulinemia) ngā rā kura me ngā rā mahi nā te mate i te nuinga o te taupori.

Kia pēhea te roa o te oranga o te hunga whai XLA (X-Linked Agammaglobulinemia)?

He mea tino pai, nā te whanaketanga o ngā tikanga maimoatanga , kei te ora tonu te hunga e pangia ana e te XLA (X-Linked Agammaglobulinemia) i ngā whenua whakawhanake pērā i Amerika ki te pakeketanga . Heoi, i ngā whenua whakawhanake, he tino uaua tonu te tautuhi me te whiwhi maimoatanga. Nō reira, i te nuinga o te wā, ka taea te whakaiti i te roanga o te oranga o ngā tamariki e pangia ana e te XLA (X-Linked Agammaglobulinemia) i aua whenua. Engari i Sri Lanka, he pai ngā maimoatanga mō ēnei āhuatanga ināianei.

Ka taea te ārai i te XLA (X-Linked Agammaglobulinemia)?

Mena kei te āwangawanga koe mō te XLA (X-Linked Agammaglobulinemia), arā, kei roto i tō whānau te mate, ka taea e koe te toro atu ki tētahi tākuta me te tirotiro ira . Mā tēnei ka āwhina i a koe ki te kimi i ngā mate ira ka taea e koe te tuku atu ki tāu tamaiti. Mena he kaikawe koe i te whakarerekētanga ira e puta ai te XLA (X-Linked Agammaglobulinemia), ina whānau he tamaiti tāu, e 50% te tūponotanga ka riro i taua tamaiti te whakarerekētanga. Mena he tama taua tamaiti, ka taea e ia te whakawhanake i te XLA (X-Linked Agammaglobulinemia). Mena kei a koe te XLA (X-Linked Agammaglobulinemia), ka noho hei kaikawe āu kōtiro. Ma te kaitohutohu ira , te tākuta rānei i tono i te whakamātautau e hoatu he tohutohu atu ki a koe mō tēnei.

Me pēhea taku tiaki i ahau anō?

Ko te huarahi pai ki te tiaki i a koe anō me te XLA (X-Linked Agammaglobulinemia) ko te whakarite i tō hauora . Kia mau ki ngā wā i whakaritea e tō tākuta. Akohia te kite i ngā tohu o te mate. Pātai atu ki tō tākuta me aha koe mēnā ka puta he tohu o te mate.

Āhea ahau me toro atu ki te tākuta?

He pai ake te kōrero ki te tākuta i roto i ēnei āhuatanga:

• Mena ka pāngia tāu tamaiti e te mate i muri i te whiwhinga o te kano ārai ora (hei tauira, te kano ārai MMR, te kano ārai rewharewha rānei).
• Mena he maha ngā mate kitakita e pāngia ana e tāu tamaiti.
• Mena ka pāngia koe e ngā mate kitakita maha (he mate pēnei i te CVID, ka pāngia hoki ngā pakeke).

Mā te tākuta koe e kī atu mēnā me tirotirohia anō tēnei.

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

He mea āwhina pea māu te pātai i ēnei pātai ina kite koe i tō tākuta:

• He aha ngā tohu o te mate urutā me tirotiro e au?
• Me aha ahau mēnā kei te whakaaro ahau kei te pangia ahau, taku tamaiti rānei?
• He aha ngā kōwhiringa maimoatanga?
• Kia pēhea te auau o te maimoatanga mōku, mō taku tamaiti rānei?

He mea noa te pāngia pinepine o ngā tamariki nohinohi e te mate. Engari, ki te pāngia tō tamaiti e ngā mate kitakita, tera pea he take kē atu kei muri. Kōrero ki tō tākuta mō ō āwangawanga katoa. Ko te kimi i te tātaritanga me te maimoatanga i te wā wawe te huarahi pai ki te whiwhi i ngā hua pai rawa atu.

Mena kei te noho koe me te XLA (X-Linked Agammaglobulinemia) – he mate ira e kore ai tō tinana e whakaputa tika i ngā pūtau-B – me whai i ngā tohutohu a tō tākuta. He mea tino nui kia taea e koe te kite i ngā tohu o te mate kia taea ai e koe te whiwhi maimoatanga i te wā e taea ai.

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

Āe, anei ētahi mea hei maumahara māu mai i tā tātou i kōrero ai mō te XLA (X-Linked Agammaglobulinemia):

• He aha te XLA (X-Linked Agammaglobulinemia)?He mate ira onge e pā nui ana ki ngā tama .
• Koinei te wā kāore e whanake tika ngā pūtau-B o te tinana, ka heke te nui o ngā paturopi me te maha o ngā mate kitakita .
• Ka taea e ngā mea pēnei i ngā tonsil me ngā adenoids te mimiti, te ngaro rānei.
• Ahakoa kāore he rongoā motuhake mō tēnei, ka taea te whakahaere pai mā te maimoatanga mō te wā roa (RIgG) me te maimoatanga paturopi tere.
• Kāore i te pai te hoatu kano ārai ora ki ēnei tāngata.
• Mena he maha ngā wā e tino pāngia ana tāu pēpi tama, he mea nui kia rapu tohutohu hauora tonu. Mā te tātaritanga me te maimoatanga wawe ka āwhina i tāu tamaiti kia ora noa.

Ko tāku tumanako he whai hua ēnei mōhiohio ki a koe. Mēnā he pātai āu, kaua e mangere ki te kōrero ki tō tākuta whānau!