Ina kī atu tō tākuta ki a koe, ki tō whanaunga rānei, kei te pāngia koe e te mate Gaucher, tera pea ka pāngia koe e te ohorere me te mataku. He mea noa tēnā. Engari ko te mea nui, he maha ngā maimoatanga pai e wātea ana i ēnei rā hei āwhina i a koe ki te noho pai me tēnei mate. Ahakoa kāore he rongoā mō tēnei mate, ka taea e ngā maimoatanga o nāianei te āwhina i a koe ki te ārai i te kino o ō whekau me te whakahaere i ō tohumate kia ora ai koe. Nō reira kaua e manukanuka, me kōrero taipitopito tātou mō tēnei.
Ko ētahi maimoatanga he pai mō ngā pakeke anake. Ko ētahi atu maimoatanga he mea hanga motuhake mō ētahi momo mate Gaucher. Waihoki, ki te mea he tino ngawari ngā tohumate o tō tamaiti, kāore pea koe e hiahia ki tētahi maimoatanga. I taua wā, me tirotiro pea koe mēnā ka pai ake ō tohumate, ā, me tīmata noa te maimoatanga mēnā e tika ana. Mā tō tākuta tēnei katoa e whakatau.
Tikanga maimoatanga matua: Whakaora Whakakapinga Whākōkī (ERT)
Koinei te maimoatanga tino noa, tino whai hua hoki. Ka taea e ngā pakeke me ngā tamariki e pangia ana e te mate Gaucher Momo 1 me te Momo 3 te whiwhi i tēnei maimoatanga.
I te whakarapopototanga, ko te mate Gaucher he āhua e whakaitihia ai te kaha o te tinana ki te whakaputa i tētahi whākōkī motuhake. Nō reira, kāore e taea te wawahi tika i ētahi ngako o te tinana, ā, ka waiho ki ngā wāhi pērā i te ate, te whēkau, me te hinu wheua. Ko te Whakaora Whakakapinga Whākōkī (ERT) ko te hoatu i te whākōkī ngaro ki te tinana i waho, hei werohanga.
He rite tēnei maimoatanga ki te whakakapi i tētahi pūrama rama pakaru i roto i tō mātou whare ki tētahi mea hou. Ka āwhina i te tinana kia mahi tika anō mā te whakarato i ngā mea e ngaro ana i waho.
He aha ngā painga o te maimoatanga ERT?
- Te whakaora i te ate me te whēkau pupuhi: Ko ēnei whekau kua rahi ake nā te mate, ka whakaitihia anō, ā, ka pai ake te mahi.
- Te Whakaiti i te Mate Kore: Ka āwhina tēnei ki te whakahaere i te mate kore, e hua ai te ngenge me te ngoikore i roto i te tini o te tangata.
- Te whakapakari i ngā wheua: Ka whakaiti i te mamae o te wheua, ka whakapakari i a rātou, ā, ka ārai i te whati ngāwari.
Heoi, he mea nui kia maumahara kāore te ERT e tino whai hua ki te whakahaere i ngā tohu o te roro, o te pūnaha io rānei (pērā i te haurangi) e puta ana i te mate Gaucher momo 3. Nā te mea he iti noa te kaha o te rongoā ki te haere mai i roto i ō tātou toto ki roto i te roro (te arai toto-roro).
E toru ngā momo matua o ngā rongoā ERT e whakamahia ana i tēnei wā:
- Imiglucerase (Imiglucerase - Cerezyme)
- Taliglucerase alfa (Taliglucerase alfa - Elelyso)
- Velaglucerase alfa (VPRIV)
Ka hoatu tēnei rongoā hei wai tote (rongoā IV), mā roto i tētahi ngongo iti ki roto i tētahi uaua. I te nuinga o te wā me haere koe ki te hōhipera, ki te whare hauora rānei i ia rua wiki kia whiwhi ai koe i tēnei maimoatanga i roto i te 1-2 hāora.
Ngā Rongoā ā-Waha
Haunga te maimoatanga ERT, kei reira anō ngā pire ā-waha ka taea te tango. He mea whakarite motuhake ēnei mō ngā pakeke e pāngia ana e te mate Gaucher Momo 1. Ko te mahi a ēnei pire mā te whakaiti i te kohikohinga o ngā putunga ngako i roto i te tinana i kōrerohia e tātou i mua ake nei.
| Ingoa o te rongoā | Whakaahuatanga me te whakamahinga |
|---|---|
| Eliglustat (Eliglustat - Cerdelga) | He rongoā hou tēnei. Me tango kia rua i te rā. Heoi, mō ētahi tāngata e tere rawa ana te pakaru o tēnei rongoā i roto i ō rātou tinana (ngā matū tere e tere ai te metabolism), tērā pea kāore e tino whai hua. |
| Miglustat (Miglustat - Zavesca) | Ka hoatu tēnei ki ngā pakeke e pāngia ana e te mate Gaucher momo 1 ngawari ki te momo whakaraerae, kāore nei i pai te rongoā ERT. Me tango kia kotahi te kāpūru e toru ngā wā i te rā. |
Ētahi atu rongoā āwhina
He maha atu anō ngā maimoatanga e wātea ana hei whakahaere i ngā raruraru maha o te mate Gaucher.
Te maimoatanga mō ngā wheua ngoikore
He mea noa te ngoikore me te mamae o te wheua i roto i te mate Gaucher. Ka whakamahia he momo rongoā e kiia nei ko ngā bisphosphonates hei āwhina i tēnei āhuatanga. Ka mahi ēnei mā te whakapakari i ngā wheua. Hei tauira, ko te alendronate (Fosamax) me te pamidronate (Aredia). He pai ēnei mō ngā tamariki me ngā pakeke.
Ngā Maimoatanga Mamae
Ka taea e koe te whakamahi i ngā rongoā whakamamae noa mō ngā mamae tinana me ngā hononga.
- Mō te mamae iti: Ngā rongoā pēnei i te acetaminophen (Tylenol) ibuprofen rānei (Advil, Motrin).
- Mō te mamae nui: Me tango pea e koe ngā rongoā whakamamae mamae kaha mai i te rōpū opioid , e ai ki ngā tohutohu a tō tākuta.
Hei tāpiritanga, i ētahi wā ka āwhina ngā rongoā ārai pouri, ngā rongoā whakangāwari uaua rānei ki te whakahaere i te mamae.
Pokanga
Ahakoa ka taea te whakahaere i te mate mā te rongoā, i ētahi wā ka hiahiatia he pokanga.
- Pokanga Āwhewhe: Ki te kore te āwhewhe pupuhi e mimiti i muri i te maimoatanga ERT, ā, ka haere tonu te whakararuraru, ka tūtohu pea tō tākuta kia tangohia te āwhewhe i roto i te pokanga.
- Pokanga Whakakapinga Hononga: Mena kua tino pakaru ngā hononga i te mate (hei tauira, te hononga hope) ā, he tino kino te mamae, kāore e taea te hikoi, ka taea te mahi pokanga hei whakakapi i taua hononga ki tētahi hononga horihori. Ka taea e tēnei te whakaiti i te mamae, ā, ka taea hoki te nekeneke pai ake anō.
Te Whakatōnga Huango Wheua
He maimoatanga tino mōrearea, he onge hoki tēnei mō te mate Gaucher. Ko te tikanga he whakakapi i ngā pūtau toto kua pakaru ki ngā mea hauora. Heoi, kāore anō kia kitea he taunakitanga mārama mō tōna haumaru me tōna whai huatanga ki te whakatauritea ki ngā maimoatanga paerewa pēnei i te ERT. Nō reira, ka whakaarohia anake i roto i ngā take tino motuhake kāore anō kia whai hua ētahi atu maimoatanga.
Te Whakawhiti Toto
He pai tēnei mō te hunga e pāngia ana e te mate toto nui e te mate Gaucher. Mā te koha toto ka nui ake te maha o ngā toto whero, ka whakahokia mai te kaha me te ora o te tinana.
Te hiranga o te āhua noho: te kai me te whakakori tinana
Haunga te whiwhi maimoatanga, he mea tino nui anō hoki te pupuri i te oranga pai, hauora hoki.
- Kai tōtika: He mea nui kia kai i ngā kai e nui ana te konupūmā me te huaora D , ina koa kia kaha ai ngā wheua. Whakaurua ngā mea pēnei i te miraka, te miraka kawa, te tiihi, ngā huawhenua matomato, me ngā ika ririki ki roto i tō kai.
- Korikori Tinana: Kōrero ki tō tākuta, kimihia ngā korikori tinana e tika ana māu, ā, kāore e whakararuraru i ō hononga. He pai ngā mahi pēnei i te hikoi me te kaukau.
- Ngā Tāpiringa: Ki te whakaaro tō tākuta kāore koe e whiwhi ana i te nui o te huaora D, te konupūmā rānei mai i tō kai, ka tūtohu pea ia i ngā tāpiringa.
Hei whakamutunga, kei te rangahau tonu ngā kaipūtaiao me ngā tākuta i ngā maimoatanga hou mō te mate Gaucher. Ka kiia ēnei he whakamātautau haumanu . Mā ēnei whakamātautau ka taea e koe te whakamātau i ngā maimoatanga hou kāore anō kia whānuitia te wātea. Kōrero ki tō tākuta mēnā ka taea e koe te whai wāhi atu ki tētahi o ēnei whakamātautau.
Karere Tangohia ki te Kāinga
- Ehara te mate Gaucher i te mate hei wehi, hei whakarērea rānei. He maha ngā maimoatanga whai hua e wātea ana i ēnei rā hei āwhina i a koe ki te whakahaere i ō tohumate me te noho ora pai.
- Ko te rongoā matua ko te rongoā whakakapinga whākōkī (ERT). Hei tāpiritanga, ka whakamahia hoki ngā rongoā ā-waha, ngā rongoā whakapakari wheua, me ngā rongoā whakamamae mamae.
- I ētahi wā, ka whakamahia te pokanga mēnā kāore e taea e ngā rongoā te whakahaere i te āhua.
- Haunga te whiwhi maimoatanga, he mea tino nui anō hoki te kai i ngā kai pai e whai kiko ana i te konupūmā me te huaora D, me te mahi i ngā mahi whakakori tinana e pai ana ki a koe.
- Ko te mea nui ko te noho tonu ki te whakapā atu ki tō tākuta, me te whai i ana tohutohu kia tika. Kōrerohia ki a ia me te tuwhera mō ō pātai, ō wehi, ō ruarua rānei.
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