ʻO nā mea e pono ai ʻoe e ʻike e pili ana i nā papa Migalastat (no ka maʻi Fabry)

Ua kuhikuhi anei kāu kauka iā ʻoe a i ʻole kekahi āu i ʻike ai e lawe i kahi lāʻau lapaʻau i kapa ʻia ʻo Migalastat ? ʻAʻole kēia he lāʻau lapaʻau maʻamau loa ma ko mākou ʻāina. No laila, he nui paha kāu mau nīnau e pili ana i kēia lāʻau lapaʻau. He mea maʻamau ka noʻonoʻo ʻana i nā mea e like me "He aha ka mea e hana ʻia me kēia?", "Pehea wau e lawe ai?", "E loaʻa anei kekahi mau hopena ʻaoʻao hoʻonāukiuki?" Mai hopohopo, e kamaʻilio kākou e pili ana i kēia mau mea a pau me ka maʻalahi a me ka maopopo.

I ka ʻōlelo maʻalahi, he aha ʻo Migalastat?

He lāʻau lapaʻau kūikawā ʻo Migalastat i hāʻawi ʻia i nā poʻe me ka maʻi Fabry. I kēia manawa ke noʻonoʻo nei paha ʻoe, "He aha ka maʻi Fabry?"

E noʻonoʻo i ko kākou kino e like me kahi hale hana nui. Ma kēia hale hana, he nui nā limahana liʻiliʻi e kōkua ana i nā mea āpau e hana pono. Kāhea mākou iā lākou he mau enzymes . I loko o ke kino o ke kanaka me ka maʻi Fabry, ʻaʻole i hana pono ʻia kekahi enzyme, a i ʻole ʻaʻole ia e hana pono. ʻO ka hana nui o kēia enzyme ka wāwahi ʻana i kahi ʻano mea momona (lipids) i loko o kā mākou mau cell.

I kēia manawa, he aha ka mea e hana ai ke hana ʻole kēia enzyme? Hoʻomaka mālie kēlā mau mea momona e hōʻiliʻili i loko o nā ʻōpū koʻikoʻi e like me nā maka, nā puʻupaʻa , a me ka puʻuwai . Ua like ia me ka lepo e paʻa ana i loko o ka paipu. I ka hala ʻana o ka manawa, hiki i kēia ke hana i nā pilikia olakino like ʻole.

Eia ka hana a nā pila Migalastat: Kōkua lākou i kēlā enzyme e hana pono ʻole ana. Ua like ia me ka hāʻawi ʻana i kahi hoʻoikaika iki i kahi limahana luhi a me ka ʻōlelo ʻana iā ia, "ʻAe, e hana pono i kāu hana." Hoʻihoʻi ka lāʻau i kēlā enzyme a hoʻomaka e wāwahi i ka momona i mālama ʻia. ʻO ka hopena kēia i ka emi ʻana o nā hōʻailona a me ka emi ʻana o ka pilikia o nā pilikia olakino koʻikoʻi.

Nā mea e pono ai ʻoe e haʻi i kāu kauka ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau

Ma mua o ka hoʻomaka ʻana iā Migalastat, he mea nui loa ia e kamaʻilio ākea me kāu kauka. ʻO ka nui o ko lākou ʻike e pili ana i kou olakino, ʻoi aku ka palekana o kā lākou mālama ʻana iā ʻoe. E hōʻoia e haʻi iā lākou e pili ana i kēia mau mea.

• Maʻi puʻupaʻa : Inā he maʻi kou e pili ana i ka puʻupaʻa, e ʻoluʻolu e haʻi mai iā mākou.
• Nā Allergies : Loaʻa iā ʻoe kahi maʻi allergy iā Migalastat a i ʻole kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, a mea hoʻoluʻu paha.) Inā he, e haʻi maopopo mai.
• Hāpai : Inā hāpai ʻoe a i ʻole e hoʻolālā ana e hāpai, e ʻoluʻolu e haʻi mai iā mākou e pili ana.
• Inā he makuahine hānai ʻoe: Inā he makuahine hānai ʻoe, e hoʻomaopopo pū i ke kauka.

He aha ke ala maikaʻi loa e hoʻohana ai i ka lāʻau lapaʻau?

I mea e loaʻa ai ka pono nui mai kēia lāʻau lapaʻau, he mea nui loa ia e hoʻohana pono. E ʻoluʻolu e hoʻomanaʻo pono i kēia mau kuhikuhi.

• E inu me ka wai: E ale i kēia papa me ke aniani wai.
• Mai uhaʻi, kuʻi, a nau paha: Pono e ale ʻia ka papa. Mai uhaʻi, kuʻi, a nau paha ma mua o ka lawe ʻana.
• E lawe i ka wā ʻōpū ʻole: ʻO kēia ka mea nui loa. Pono ʻoe e lawe i kēia lāʻau lapaʻau ma ka ʻōpū ʻole. ʻO ia hoʻi, pono ʻoe e lawe i ka lāʻau lapaʻau 2 mau hola ma mua a i ʻole 2 mau hola ma hope o ka ʻai ʻana.
• He laʻana maʻalahi: E noʻonoʻo ʻoe e lawe ana i kāu lāʻau lapaʻau i ka hola 8 am. A laila pono ʻoe e ʻai a hiki i ka hola 6 am. Ma hope o ka lawe ʻana i kāu lāʻau lapaʻau, hiki iā ʻoe ke ʻai hou ma hope o ka hola 10 am. ʻO ia hoʻi , pono e hakahaka kou ʻōpū no 4 mau hola ma waena o ka lawe ʻana i kāu lāʻau lapaʻau.
• Ka mea hiki iā ʻoe ke inu: Ua maikaʻi ke inu i ka wai (maʻemaʻe, ʻono, a ʻono paha), ka wai hua me ke kō ʻole i hoʻohui ʻia, a me nā mea inu kalapona ʻaʻohe caffeine i loko o kēia manawa.
• Mai hoʻōki a hiki i ka wā e ʻōlelo aku ai kāu kauka iā ʻoe: E hoʻomau i ka hoʻohana ʻana i kēia lāʻau lapaʻau a hiki i ka wā e ʻōlelo aku ai kāu kauka iā ʻoe e hoʻōki.

He aha inā hāʻawi ʻia i nā keiki?

Pono paha kēia lāʻau lapaʻau i ka mālama kūikawā ke hāʻawi ʻia i nā keiki. No laila, e kamaʻilio me kāu kauka e pili ana i ia mea a e hahai pono i kāna mau kuhikuhi.

He aha inā lawe nui wau i ka lāʻau lapaʻau?

Inā manaʻo ʻoe ua lawe ʻoe i nā lāʻau lapaʻau ma mua o kou manaʻo, e hele koke i ka Halemai Pilikia (ETU) kokoke loa. A i ʻole e kelepona i ke Kikowaena ʻIke ʻAi ʻona Lahui ma ka Halemai Lahui ʻo Colombo no ka ʻōlelo aʻoaʻo.

He aha kāu e hana ai inā e poina ʻoe i kahi mahele o ka lāʻau lapaʻau?

I kekahi manawa e poina paha iā ʻoe ke lawe i kāu lāʻau lapaʻau. Inā hana ʻia kēlā, mai hopohopo.

• E hoʻopiha i ka manawa i hala i ka wā e hoʻomanaʻo ai ʻoe.
• Eia nō naʻe, inā ua lohi loa ka lawe ʻana i ka lāʻau i hala ʻole a aia ma lalo o 12 mau hola a hiki i ka lāʻau aʻe, e hōʻalo i ka lāʻau i hala ʻole.
• E lawe i kāu mahele lāʻau aʻe i ka manawa maʻamau. Mai lawe i ʻelua mahele lāʻau i ka manawa hoʻokahi e hoʻopiha ai i kahi mahele lāʻau i hala.

Aia kekahi mau mea i kūlike ʻole me kēia lāʻau lapaʻau?

Aia kekahi mau mea e hiki ke launa pū me Migalastat, akā hoʻokahi mea nui.

• Kapeina: ʻO ka kapeina, i loaʻa i ke kofe, ke kī, a me kekahi mau mea inu palupalu, hiki ke hoʻopilikia i ka pono o kēia lāʻau lapaʻau. ʻOi aku ka maikaʻi o ka pale ʻana i nā mea āpau i loaʻa ka kapeina no nā hola 4 e pono ai ʻoe e noho me ka ʻōpū ʻole.

ʻAʻole paha i piha kēia papa inoa. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau ʻē aʻe, nā mea kanu, nā huaora, a me nā mea hoʻohui āu e lawe nei. E haʻi pū i kāu kauka inā ʻoe e puhipaka a inu waiʻona paha.

Nā hopena ʻaoʻao e hiki mai ana ma muli o ka lāʻau lapaʻau

E like me nā lāʻau lapaʻau āpau, hiki iā Migalastat ke hana i nā hopena ʻaoʻao i kekahi poʻe. Eia naʻe, ʻaʻole loaʻa i nā kānaka a pau. E nānā kākou i kēia mau hopena ʻaoʻao a me ka mea e hana ai inā loaʻa.

ʻAʻole i loko o kēia papa inoa nā hopena ʻaoʻao āpau. Eia kekahi, inā loaʻa iā ʻoe kekahi mea ʻē, e kamaʻilio me kāu kauka e pili ana i ia mea.

Pehea ʻoe e mālama pono ai i kāu lāʻau lapaʻau?

No ka mālama ʻana i ka maikaʻi o ka lāʻau lapaʻau, pono e mālama pono ʻia.

• E mālama ʻia mai nā keiki a me nā holoholona.
• E mālama ma ka mahana o ka lumi (ma waena o 20 a me 25 kekelē Celsius). E pale mai ka wela nui a i ʻole ke anu nui.
• E mālama i ka lāʻau lapaʻau i loko o kāna ʻeke a hiki i ka manawa e lawe ai. E pale iā ia mai ka makū.
• Mai hoʻohana i ka lāʻau lapaʻau i pau ka lā.

Pehea e hoʻolei aku ai i nā lāʻau lapaʻau i hoʻohana ʻole ʻia a i pau paha?

ʻAʻole ia he manaʻo maikaʻi e hoʻolei wale i nā lāʻau lapaʻau i pau i ka manawa i loko o ka ʻōpala a hoʻolei paha iā lākou i loko o ka lua. Hiki iā lākou ke hōʻino i ke kaiapuni. E nīnau i kāu kauka a i ʻole ka haukapila e pili ana i kēia. I kekahi manawa, loaʻa paha i nā haukapila nā papahana e hoʻihoʻi i kēia mau mea.

Inā ʻaʻohe ʻano hana like a ʻōlelo ke kauka he palekana ke hoʻolei ʻia i loko o ka ʻōpala, eia ka mea e hana ai:

1. E lawe i ka lāʻau lapaʻau mai loko mai o ka ʻeke.

2. E kāwili maikaʻi iā lākou me kahi mea e like me ke kope kope i hoʻohana ʻia, ka lepo, a i ʻole ka ʻōpala pōpoki.

3. E kau i kēia hui ʻana i loko o kahi ʻeke a i ʻole kahi pahu hiki ke sila ʻia, a laila e hoʻolei i loko o ka ʻōpala.