Ke haʻi aku kāu kauka iā ʻoe a i ʻole kāu mea aloha he maʻi Gaucher ko lākou, e manaʻo paha ʻoe i ka haʻalulu a me ka makaʻu. He mea maʻamau kēlā. Akā ʻo ka mea nui, he nui nā lāʻau lapaʻau maikaʻi i loaʻa i kēia lā e hiki ke kōkua iā ʻoe e ola i kahi ola ʻoluʻolu me kēia maʻi. ʻOiai ʻaʻohe lāʻau lapaʻau no kēia maʻi, hiki i nā lāʻau lapaʻau o kēia manawa ke kōkua iā ʻoe e pale i ka hōʻino ʻana i kou mau kino a hoʻokele i kāu mau hōʻailona i hiki iā ʻoe ke ola i kahi ola olakino. No laila, mai hopohopo, e kamaʻilio kākou e pili ana i nā kikoʻī.
He kūpono wale nō kekahi mau lāʻau lapaʻau no nā pākeke. Ua hoʻolālā kūikawā ʻia kekahi mau lāʻau lapaʻau no kekahi mau ʻano maʻi Gaucher. Eia kekahi, inā he mau hōʻailona iki kou a i ʻole kāu keiki, ʻaʻole paha ʻoe e pono i kekahi lāʻau lapaʻau. I kēlā hihia, pono paha ʻoe e ʻike inā hoʻomaikaʻi kāu mau hōʻailona a hoʻomaka wale i ka lāʻau lapaʻau inā pono. Hoʻoholo ʻia kēia e kāu kauka.
ʻAno lapaʻau nui: Enzyme Replacement Therapy (ERT)
ʻO kēia ka lāʻau lapaʻau maʻamau a kūpono hoʻi. Hiki i nā pākeke a me nā keiki me ka maʻi Gaucher ʻAno 1 a me ʻAno 3 ke loaʻa i kēia lāʻau lapaʻau.
I ka ʻōlelo maʻalahi, ʻo ka maʻi Gaucher kahi kūlana i hoʻemi ʻia ai ka hiki o ke kino ke hana i kahi enzyme kikoʻī. ʻO ka hopena, ʻaʻole hiki ke wāwahi pono ʻia kekahi mau momona i loko o ke kino a waiho ʻia ma nā wahi e like me ke akepaʻa, ka spleen, a me ka iwi iwi. ʻO ka Enzyme Replacement Therapy (ERT) e pili ana i ka hāʻawi ʻana i ke kino i ka enzyme nalowale ma waho, ma ke ʻano he injection.
Ua like kēia lāʻau lapaʻau me ke pani ʻana i kahi kukui uila i haki i loko o ko mākou hale me kahi kukui uila hou. Kōkua ia i ke kino e hana pono hou ma ka hoʻolako ʻana i nā mea i nele mai waho.
He aha nā pōmaikaʻi o ka mālama ʻana i ka ERT?
- Hoʻoulu hou i ke akepaʻa a me ka spleen pehu: ʻO kēia mau ʻōkana, i hoʻonui ʻia ma muli o ka maʻi, ua liʻiliʻi hou ʻia a ua hoʻomaikaʻi ʻia kā lākou hana.
- Hoʻēmi i ka Anemia: Kōkua kēia i ka kaohi ʻana i ka anemia, kahi e hoʻoulu ai i ka luhi a me ka nāwaliwali i nā poʻe he nui.
- Hoʻoikaika i nā iwi: Hoʻemi ia i ka ʻeha o ka iwi, hoʻoikaika iā lākou, a pale iā lākou mai ka haki maʻalahi.
Eia nō naʻe, he mea nui e hoʻomanaʻo ʻaʻole maikaʻi loa ka ERT i ka kaohi ʻana i nā hōʻailona o ka lolo a i ʻole ka ʻōnaehana nerve (e like me nā hopu ʻana) e kū mai ana i ka maʻi Gaucher ʻano 3. ʻO kēia no ka mea he palena iki ka hiki o ka lāʻau ke hele mai ko mākou kahe koko i loko o ka lolo (ka pale koko-lolo).
ʻEkolu mau ʻano nui o nā lāʻau ERT e hoʻohana ʻia nei:
- ʻImiglucerase (Imiglucerase - Cerezyme)
- Taliglucerase alfa (Taliglucerase alfa - Elelyso)
- ʻO ka Velaglucerase alfa (VPRIV)
Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he wai saline (lāʻau IV), ma o kahi ʻōmole liʻiliʻi i loko o kahi aa. Pono ʻoe e hele i kahi haukapila a i ʻole keʻena lapaʻau i kēlā me kēia ʻelua pule e loaʻa ai kēia lāʻau lapaʻau i loko o 1-2 mau hola.
Nā lāʻau lapaʻau waha
Ma waho aʻe o ka lāʻau ERT, aia kekahi mau pila waha e hiki ke lawe ʻia. Ua kuhikuhi kūikawā ʻia kēia mau mea no nā pākeke me ka maʻi Gaucher Type 1. Hana kēia mau pila ma ka hōʻemi ʻana i ka hōʻiliʻili ʻana o kēlā mau waihona momona i loko o ke kino a mākou i kamaʻilio ai ma mua.
| Inoa o ka lāʻau lapaʻau | Ka wehewehe a me ka hoʻohana ʻana |
|---|---|
| ʻEliglustat (ʻEliglustat - Cerdelga) | He lāʻau lapaʻau hou kēia. Pono e lawe ʻia i ʻelua manawa i ka lā. Eia nō naʻe, no kekahi poʻe e wāwahi koke ana i kēia lāʻau lapaʻau i loko o ko lākou kino (nā metabolizers wikiwiki), ʻaʻole paha e like ka maikaʻi. |
| Miglustat (Miglustat - Zavesca) | Hāʻawi ʻia kēia i nā pākeke me ka maʻi Gaucher ʻano 1 a ʻoluʻolu a ʻaʻole i pane i ka lāʻau ERT. Pono e lawe ʻia hoʻokahi capsule ʻekolu manawa i ka lā. |
Nā lāʻau lapaʻau adjuvant ʻē aʻe
Aia kekahi mau lāʻau ʻē aʻe i loaʻa e kāohi i nā pilikia like ʻole o ka maʻi Gaucher.
Lapaʻau no nā iwi nāwaliwali
He mea maʻamau ka nāwaliwali o ka iwi a me ka ʻeha i ka maʻi Gaucher. Hoʻohana ʻia kahi papa o nā lāʻau i kapa ʻia ʻo bisphosphonates e kōkua i kēia kūlana. Hana kēia mau mea ma ka hoʻoikaika ʻana i nā iwi. ʻO nā laʻana e komo pū me ka alendronate (Fosamax) a me ka pamidronate (Aredia). He kūpono kēia no nā keiki a me nā pākeke.
Nā Lapaʻau ʻeha
Hiki iā ʻoe ke hoʻohana i nā lāʻau ʻeha maʻamau no nā mea e like me ke ʻeha kino a me nā ʻeha hono.
- No ka ʻeha iki: Nā lāʻau lapaʻau e like me ka acetaminophen (Tylenol) a i ʻole ibuprofen (Advil, Motrin).
- No ka ʻeha nui: Pono paha ʻoe e lawe i nā lāʻau ʻeha ikaika mai ka hui opioid , e like me ke kauoha a kāu kauka.
Eia kekahi, i kekahi manawa, hiki i nā antidepressants a i ʻole nā mea hoʻomaha hoʻoikaika ke kōkua i ka kaohi ʻana i ka ʻeha.
ʻoki kino
ʻOiai hiki ke hoʻomalu pinepine ʻia ka maʻi me ka lāʻau lapaʻau, i kekahi mau hihia, pono paha ke ʻoki ʻana.
- ʻO ke ʻoki ʻana i ka spleen: Inā ʻaʻole e emi ka spleen pehu ma hope o ka mālama ʻana i ka ERT a hoʻomau i ka hoʻoulu ʻana i nā pilikia, hiki i kāu kauka ke ʻōlelo aku i ka wehe ʻana i ka spleen.
- ʻO ke ʻoki hoʻololi hui: Inā ua hōʻino nui ka maʻi i nā hui (e like me ke hui ʻūhā) a ua nui loa ka ʻeha a hiki ʻole ke hele wāwae, hiki ke hana ʻia ke ʻoki e hoʻololi i kēlā hui me kahi hui hana. Hiki i kēia ke hōʻemi i ka ʻeha a ʻae i ka neʻe maikaʻi hou ʻana.
Hoʻoili iwi iwi
He lāʻau lapaʻau weliweli loa kēia a kakaikahi hoʻi no ka maʻi Gaucher. Pili ia i ka hoʻololi ʻana i nā hunaola koko i hōʻino ʻia me nā mea olakino. Eia nō naʻe, ʻaʻohe hōʻike maopopo o kona palekana a me ka pono i hoʻohālikelike ʻia me nā lāʻau lapaʻau maʻamau e like me ERT. No laila, ua noʻonoʻo wale ʻia i nā hihia kūikawā loa kahi i holo pono ʻole ai nā lāʻau lapaʻau ʻē aʻe.
Hoʻoili koko
Hiki i kēia ke lilo i mea pono no ka poʻe me ka anemia koʻikoʻi i hoʻokumu ʻia e ka maʻi Gaucher. Hiki i ka hāʻawi ʻana i ke koko ke hoʻonui i ka helu o nā hunaola koko ʻulaʻula, e hoʻihoʻi ana i ka ikehu a me ke ola o ke kino.
Ke koʻikoʻi o ke ʻano o ka nohona: ka ʻai a me ka hoʻoikaika kino
Ma waho aʻe o ka loaʻa ʻana o ka lāʻau lapaʻau, he mea nui loa hoʻi ka mālama ʻana i kahi nohona maikaʻi a olakino hoʻi.
- Meaʻai momona: He mea nui e ʻai i ka meaʻai momona i ka calcium a me ka wikamina D , ʻoiai hoʻi e hoʻoikaika i nā iwi. E hoʻokomo i nā mea e like me ka waiū, ka yogurt, ka tīhi, nā mea kanu ʻōmaʻomaʻo, a me nā iʻa liʻiliʻi i kāu papaʻai.
- Hoʻoikaika Kino: E kamaʻilio me kāu kauka a e hana i nā hoʻoikaika kino kūpono iā ʻoe a ʻaʻole hoʻi e paʻakikī loa i kou mau hono. He mea maikaʻi nā mea e like me ka hele wāwae a me ka ʻauʻau ʻana.
- Nā Mea Hoʻohui: Inā manaʻo kāu kauka ʻaʻole lawa kāu wikamina D a i ʻole calcium mai kāu meaʻai, hiki iā ia ke ʻōlelo i nā mea hoʻohui.
ʻO ka mea hope loa, ke noiʻi mau nei nā kānaka ʻepekema a me nā kauka i nā lāʻau lapaʻau hou no ka maʻi Gaucher. Ua kapa ʻia kēia he mau hoʻokolohua lapaʻau . Hiki i kēia mau hoʻokolohua iā ʻoe ke hoʻāʻo i nā lāʻau lapaʻau hou i loaʻa ʻole ma ka laulā. E kamaʻilio me kāu kauka e pili ana i ka hiki iā ʻoe ke komo i kekahi o kēia mau hoʻokolohua.
Leka Lawe-Home
- ʻAʻole ka maʻi Gaucher he maʻi e makaʻu ʻia a haʻalele ʻia. Aia nā lāʻau lapaʻau kūpono i loaʻa i kēia lā e hiki ke kōkua iā ʻoe e kaohi i kāu mau hōʻailona a ola i kahi ola maikaʻi.
- ʻO ka lāʻau lapaʻau nui ka enzyme replacement therapy (ERT). Eia kekahi, hoʻohana pū ʻia nā lāʻau lapaʻau waha, nā lāʻau hoʻoikaika iwi, a me nā mea hoʻomaha ʻeha.
- I kekahi mau hihia, hoʻohana wale ʻia ke ʻoki kino inā ʻaʻole hiki i nā lāʻau lapaʻau ke hoʻomalu i ke kūlana.
- Ma waho aʻe o ka loaʻa ʻana o ka lāʻau lapaʻau, he mea nui loa hoʻi e ʻai i ka meaʻai maikaʻi i waiwai i ka calcium a me ka wikamina D a hana i ka hoʻoikaika kino e kūpono iā ʻoe.
- ʻO ka mea nui loa, ʻo ia ka launa mau ʻana me kāu kauka a hahai pololei i kāna mau kuhikuhi. E kamaʻilio ākea me ia e pili ana i nā nīnau, nā makaʻu, a i ʻole nā kānalua āu e loaʻa ai.
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