Ua hiki anei ka manawa e hāʻawi ai i kāu keiki i ka lāʻau lapaʻau (Elosulfase alfa)? E kamaʻilio kākou e pili ana i (Mucopolysaccharidosis type IVA - Morquio A syndrome)!

Ua lohe paha ʻoe e pili ana i kēia lāʻau lapaʻau i kapa ʻia ʻo `(Elosulfase alfa)` ma hope o ka haʻi ʻana o ke kauka iā ʻoe ua loaʻa i kāu keiki kahi maʻi laha ʻole i kapa ʻia ʻo `(Mucopolysaccharidosis type IVA)` a i ʻole `(Morquio A syndrome)`. I ka manawa e like me kēia, he mea maʻamau ka nui o nā mea e hiki mai ana i ka noʻonoʻo a me nā nīnau he nui e kū mai ana. No laila, i kēia lā e kamaʻilio mākou e pili ana i kēia lāʻau lapaʻau, kēia kūlana me nā kikoʻī hou aku, ma ke ʻano e hiki ai iā ʻoe ke hoʻomaopopo, ʻae?

He aha kēia lāʻau lapaʻau? E aʻo kākou e pili ana i ka `(Elosulfase alfa)`?

I ka ʻōlelo maʻalahi, ʻo `(Elosulfase alfa)` kahi lāʻau lapaʻau pani enzyme . Ke noʻonoʻo nei paha ʻoe, "He aha ka ` enzyme` ?" E noʻonoʻo penei: ua like ko mākou kino me kahi hale hana nui. He mau limahana liʻiliʻi ma kēia hale hana e mālama i kēlā me kēia ʻāpana o ke kaʻina hana e holo mālie a ma ke ʻano. ʻO ia ke ʻano o nā enzymes. He mea nui kēia mau enzymes no nā kaʻina hana kemika koʻikoʻi he nui e hana ʻia i loko o ko mākou kino.

I kēia manawa, ma ke ʻano o `(Mucopolysaccharidosis type IVA)` a i ʻole `(Morquio A syndrome)`, ʻo ka mea e hana ʻia ana, ʻo ia ka nele a ʻaʻole paha e hana pono kahi enzyme kūikawā e pono ai ko kākou kino. Ke nele kēia enzyme, hoʻomaka kekahi mau mea i loko o ke kino e hōʻiliʻili ma kahi o ka wāwahi pono ʻana. ʻO kēia ke kumu o nā pilikia like ʻole i nā iwi, nā cartilage, nā maka, a me ka ʻōnaehana hanu.

No laila, hoʻohana ʻia kēia lāʻau lapaʻau i kapa ʻia ʻo `(Elosulfase alfa)` e hāʻawi i ka enzyme nalowale ma waho. Ua like kēia me ka hoʻolimalima ʻana i kekahi e pani i kahi limahana nalowale ma kahi hale hana. ʻAʻole hiki i kēia ke ho'ōla piha i ka maʻi. Eia naʻe, hiki iā ia ke kōkua i ka kaohi ʻana i nā hōʻailona a hoʻomaʻalahi iki i ke ola. ʻO ka hapa nui o ka manawa, e ʻike ʻoe i kēia lāʻau lapaʻau ma lalo o ka inoa inoa `(Vimizim)`.

He aha nā mea e pono ai ʻoe e haʻi aku i ke kauka?

Ma mua o ka hoʻomaka ʻana i kēia lāʻau lapaʻau, he mea nui loa ia e haʻi i kāu hui mālama ola (ʻo ia hoʻi ke kauka, kahu maʻi ) i nā mea āpau e pono ai ʻoe e ʻike e pili ana i kou olakino a me ke olakino o kāu pēpē. E hōʻoia e haʻi iā lākou e pili ana i kēia mau mea:

• Inā loaʻa iā ʻoe ke kuni a i ʻole kekahi maʻi i kēia manawa.
• Inā loaʻa iā ʻoe ka apnea hiamoe, hoʻōki kekahi poʻe i ka hanu ʻana no kekahi manawa i ko lākou hiamoe ʻana a laila hoʻomaka hou e hanu. He kūlana kēlā.
• Inā ua loaʻa iā ʻoe kahi maʻi ʻaleka i ka lāʻau lapaʻau `(Elosulfase alfa)` ma mua, i kekahi lāʻau lapaʻau ʻē aʻe, i kahi ʻano meaʻai, i kekahi manawa i nā mea hoʻoluʻu, i nā mea mālama, a pēlā aku. E haʻi mai iā mākou, ʻoiai inā he mea liʻiliʻi ia.
• Inā ʻoe e hāpai ana a i ʻole e hoʻāʻo ana e hāpai.
• Inā he makuahine hānai waiū ʻoe.

He mea kōkua nui kēia ʻike no ke kauka a kahu maʻi paha e hoʻoholo ai ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau.

Pehea ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?

Hāʻawi ʻia kēia lāʻau lapaʻau `(Elosulfase alfa)` ma ke ʻano he `infusion` i loko o ke aʻa. ʻO ia hoʻi, hāʻawi ʻia ia ma ke ʻano he saline solution, komo mālie ka lāʻau lapaʻau i loko o ke kino. ʻAʻole hiki ke hana ʻia kēia ma ka home. Hana ʻia kēia lāʻau lapaʻau ma kahi haukapila a i ʻole ke keʻena lapaʻau, ma lalo o ka nānā ʻana a kahi loea lapaʻau i aʻo ʻia (he kauka a he kahu maʻi paha).

E kamaʻilio me kāu kauka keiki e pili ana i ka hāʻawi ʻana i kēia lāʻau lapaʻau i nā keiki. I kekahi mau hihia i koho ʻia, hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki a hiki i 5 mau makahiki, akā aia kekahi mau hana palekana e pono e hana ʻia.

Mea nui: Inā manaʻo kekahi ua lawe nui lākou i kēia lāʻau lapaʻau (overdose), pono lākou e kāhea koke i ka lumi pilikia kokoke loa a i ʻole ke kikowaena kaohi lāʻau make.

ʻO kekahi mea ʻē aʻe, ua kuhikuhi ʻia kēia lāʻau lapaʻau nou wale nō. Mai kaʻana like me kekahi no kekahi kumu . He ʻokoʻa ke kūlana o kēlā me kēia kanaka, no laila ʻo ka mea nāna i kuhikuhi wale nō ke hoʻohana.

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

He mea nui loa ka lawe ʻana i kāu lāʻau lapaʻau i ka manawa kūpono a ma ka lā o kāu manawa i hoʻonohonoho ʻia ai, no ka mea, he lāʻau lapaʻau lōʻihi kēia. Inā ʻaʻole hiki iā ʻoe ke mālama i kāu manawa i hoʻonohonoho ʻia, e kelepona koke i kāu kauka a i ʻole ke kauka lapaʻau a e haʻi iā lākou. E haʻi lākou iā ʻoe i ka mea e hana ai ma hope.

Hiki paha nā pilikia me nā lāʻau lapaʻau ʻē aʻe?

ʻAʻohe haʻawina nui i hana ʻia e pili ana i ke ʻano o ka launa pū ʻana o kēia lāʻau lapaʻau (Elosulfase alfa) me nā lāʻau lapaʻau ʻē aʻe. ʻO ke ʻano kēia e launa pū paha me nā lāʻau lapaʻau ʻē aʻe āu e lawe nei.

No laila, e hana i kahi papa inoa o nā lāʻau lapaʻau āpau āu e lawe ai (nā lāʻau lapaʻau i kauoha ʻia, nā lāʻau lapaʻau ma luna o ke kauka, nā lāʻau lapaʻau, nā huaora, nā mea hoʻohui meaʻai, a me nā mea ʻē aʻe) a hāʻawi i kāu kauka. Eia kekahi, e hōʻoia e haʻi i kāu kauka inā ʻoe e puhipaka, inu waiʻona, a hoʻohana paha i nā lāʻau lapaʻau ʻē aʻe i kū ʻole i ke kānāwai. Hiki i kekahi mau mea ke launa pū me kēia lāʻau lapaʻau, ʻo ia hoʻi hiki iā lākou ke hoʻopili kekahi i kekahi.

He aha nā mea e makaʻala ai i ka wā e lawe ai i kēia lāʻau?

Ke hāʻawi ʻia kēia lāʻau lapaʻau, loaʻa i kekahi poʻe nā maʻi allergies koʻikoʻi.Hiki paha kēia ke hana. He mea kēia e makaʻala ai. No ka hōʻemi ʻana i kēia pilikia, e noi ʻia paha ʻoe e lawe i nā lāʻau lapaʻau ʻē aʻe (e like me ka antihistamine) ma mua o ka hāʻawi ʻana i ka lāʻau. He mea nui e lawe i ka lāʻau e like me ka mea a kāu kauka e ʻōlelo ai iā ʻoe.

I loko o ka manawa a pau e hāʻawi ʻia ai kēia lāʻau lapaʻau iā ʻoe, e nānā pono ʻia kou kūlana e nā loea olakino. No laila, ʻaʻohe mea e hopohopo ai. Na lākou e mālama iā ʻoe.

He aha nā hopena ʻaoʻao e hiki ke hana?

E like me nā lāʻau lapaʻau āpau, hiki i ka `(Elosulfase alfa)` ke hana i kekahi mau hopena ʻaoʻao. Eia naʻe, ʻaʻole loaʻa i nā kānaka a pau. He koʻikoʻi kekahi, a ʻo kekahi ʻaʻole ia he mea nui.

Nā hopena ʻaoʻao e pono ʻoe e haʻi koke aku i kāu kauka

Inā ʻike ʻoe i kekahi o nā hōʻailona ma lalo nei, pono ʻoe e haʻi i kāu kauka a kauka paha i ka wā hikiwawe loa:

• Nā hōʻailona maʻi: ʻulaʻula o ka ʻili, ka hives, ka maneʻo, a i ʻole ka pehu ʻana o ka maka, nā lehelehe, a i ʻole ke alelo.
• Nā pilikia hanu: Manaʻo e like me ʻoe e pilikia ana i ka hanu ʻana, me he mea lā e hanu ana ʻoe.
• ʻEha a ʻeha paha o ka umauma: He manaʻo o ka ʻeha i loko o ka umauma, he ʻeha e puka mai ana mai loko mai o ka umauma.
• ʻŪhū: Inā he ʻūhū ʻē kou.
• Ke koko haʻahaʻa: Hiki iā ʻoe ke poniuniu a hiki ke polū kou mau maka.

Inā ʻike ʻoe i kekahi mea e like me kēia, mai hoʻokaʻulua, ʻeā?

Nā hopena ʻaoʻao ʻaʻole i koʻikoʻi pinepine, akā pono e hōʻike ʻia i ke kauka inā hoʻomau lākou

ʻAʻole maʻamau kēia mau mea i koʻikoʻi loa, akā inā hoʻomau lākou, a inā hoʻopilikia lākou iā ʻoe, he mea maikaʻi ke haʻi aku i kāu kauka:

• Anuanu: Hiki paha iā ʻoe ke ʻano anu a haʻalulu paha i ka wā e hāʻawi ʻia ai ka lāʻau lapaʻau a i ʻole ma hope koke iho o ka hāʻawi ʻia ʻana.
• Kuni: Hiki i ke kino ke wela a me ke kuni.
• Hoʻomaha poʻo (`hisaradaya`): E like me ke poʻo maʻamau .
• Luli, luaʻi: ​​Manaʻo paha ʻoe e like me ka huli ʻana o kou ʻōpū a i ʻole manaʻo paha ʻoe e luaʻi ana.
• ʻEha ʻōpū: He ʻeha iki a ʻeha paha ma ka ʻōpū.
• Luhi: Hiki iā ʻoe ke manaʻo ua luhi a make ke ola.

He mea nui: ʻAʻole i loko o kēia papa inoa nā hopena ʻaoʻao āpau. Inā ʻike ʻoe i kekahi mea ʻē aʻe i ʻike ʻole ʻia, e kamaʻilio me kāu kauka e pili ana i ia mea.

Ma hea ʻoe e mālama ai i ka lāʻau lapaʻau?

ʻAʻole kēia he mea āu e hopohopo ai. No ka mea, ua hāʻawi ʻia kēia lāʻau lapaʻau i kapa ʻia ʻo `(Elosulfase alfa)` ma kahi haukapila a i ʻole ke keʻena lapaʻau, ʻaʻole pono ʻoe e lawe i ka home a mālama iā ia. No laila, mai hopohopo no ia mea.

ʻO ka mea hope loa, nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

ʻAe, no laila, mai nā mea a mākou i kamaʻilio ai, manaʻo wau ua loaʻa iā ʻoe kahi ʻike maikaʻi o ka lāʻau lapaʻau `(Elosulfase alfa)`. E hoʻomanaʻo, ʻo `(Mucopolysaccharidosis type IVA)` he maʻi paʻakikī, a he kūikawā nō hoʻi ka lāʻau i hāʻawi ʻia no ia mea.

• ʻAʻole ho'ōla kēia lāʻau i ka maʻi, akā kōkua ia i ka kaohi ʻana i nā hōʻailona a hoʻomaʻalahi iki i ke ola.
• Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, mai poina e hāʻawi i kāu kauka i kahi wehewehe piha no kou kūlana olakino.
• Hāʻawi ʻia ka lāʻau lapaʻau ma ka haukapila, ma ke ʻano he infusion.
• E makaʻala i nā hopena ʻaoʻao, ʻoiai inā loaʻa iā ʻoe nā hōʻailona maʻi, a e haʻi koke i kāu kauka .
• Inā he mau nīnau a kānalua paha kāu, e nīnau i ke kauka a i ʻole ke kahu maʻi. Aia lākou ma laila e kōkua iā ʻoe.

Hiki ke paʻakikī ke noho me kēia maʻi. Akā me ke aʻo lapaʻau kūpono, ke kākoʻo, a me ke aloha, hiki ke hana ʻia kēia huakaʻi. E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi.

ʻelosulfase alfa, vimizim, mucopolysaccharidosis ʻano IVA, MPS IVA, Morquio A syndrome, hoʻololi enzyme, infusion, nā hopena ʻaoʻao