E aʻo kākou e pili ana i ka lāʻau pale ʻo Idursulfase i hāʻawi ʻia i ka poʻe me Hunter Syndrome.

Loaʻa iā ʻoe a i ʻole kekahi āu i ʻike ai kahi maʻi laha ʻole i kapa ʻia ʻo Hunter Syndrome? Inā pēlā, ua lohe paha ʻoe i ka lāʻau pale i kapa ʻia ʻo Idursulfase. He inoa paʻakikī iki ia, akā i kēia lā e kamaʻilio mākou e pili ana i kēia lāʻau lapaʻau ma ke ʻano maʻalahi loa, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo. No ka mea, he mea nui loa ka ʻike pono ʻana i nā mea e like me kēia.

He aha kēia lāʻau lapaʻau i kapa ʻia ʻo Idursulfase?

I ka ʻōlelo maʻalahi, ʻo Hunter Syndrome kahi kūlana kahi e nalowale ai kahi "enzyme" kūikawā, kahi protein e kōkua ana i nā kaʻina hana kemika i loko o ko mākou kino, a i ʻole ʻaʻole hana pono. Kōkua kēia "enzyme" i ka wāwahi ʻana i kekahi mau molekala paʻakikī i loko o ko mākou kino a hoʻomaʻalahi iā lākou, ʻo ia hoʻi, hiki ke wehe ʻia mai ke kino ke pau kā lākou hana. No laila, ke nalowale kēia "enzyme", hōʻiliʻili kēlā mau molekala paʻakikī ma nā ʻāpana like ʻole o ke kino, e hana ana i nā pilikia a me nā hōʻailona like ʻole.

He lāʻau lapaʻau ʻo Idursulfase i hana ʻia ma ka hale hana e pani i kēlā enzyme nalowale, a hana ia. ʻO ia hoʻi, pani ia i kēlā enzyme nalowale, a i ʻole, he lāʻau lapaʻau pani enzyme. Akā hoʻokahi mea āu e pono ai e hoʻomanaʻo pono ʻaʻole kēia he lāʻau lapaʻau piha . Akā hiki iā ia ke kōkua i ka hoʻomalu ʻana i nā hōʻailona, ​​​​kaohi i ka ʻino o ke kūlana, a hoʻomaʻalahi iki i ke ola.

Hiki ke hoʻohana ʻia kēia lāʻau lapaʻau no nā mea ʻē aʻe i kekahi manawa, no laila inā he mau nīnau kāu, e nīnau i kāu kauka a mea kūʻai lāʻau paha.

ʻO kahi inoa kaulana o kēia lāʻau lapaʻau ʻo '(Elaprase)'.

He aha kāu e haʻi aku ai i kāu kauka ma mua o ka lawe ʻana i kēia lāʻau?

I kēia manawa, ma mua o kou hoʻomaka ʻana e lawe i kēia lāʻau lapaʻau, aia kekahi mau mea e pono ai ʻoe e haʻi aku i kāu kauka a i ʻole kāu hui mālama. ʻO kēia i hiki iā lākou ke hāʻawi iā ʻoe i ka lāʻau kūpono me ka palekana. E noʻonoʻo e like me ka ʻike mua ʻana i nā pilikia āu e hālāwai ai ma ke alanui.

Eia nā mea e hōʻike ʻia:

• Ke loaʻa nei iā ʻoe kahi maʻi a me ke kuni i kēia manawa?
• He maʻi puʻuwai kou? ʻO ia hoʻi kekahi ʻano pilikia puʻuwai.
• He maʻi māmā kou? E like me nā pilikia hanu.
• Ua loaʻa paha iā ʻoe kahi hopena maʻi i ka Idursulfase, kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, nā mea hoʻoluʻu meaʻai, a i ʻole nā ​​mea hoʻomalu? Hiki i kēia ke komo pū me ka hives, ka maneʻo, a i ʻole ka paʻakikī o ka hanu ʻana.
• Ua loaʻa anei iā ʻoe ka pehu ʻana o ke alelo, ka maka, a i ʻole nā ​​lehelehe, ka paʻakikī o ka hanu ʻana, ka paʻakikī o ka moni ʻana, ka leo palahalaha, a i ʻole ka ʻū ʻana o ka puʻu?Ua hana ʻia anei kēlā? He mea nui loa kēia, no ka mea, hiki ke hana ʻia nā mea e like me ia ke lawe ʻoe i kēia lāʻau lapaʻau.
• Ke hāpai nei ʻoe a ke manaʻolana nei paha e hāpai?
• Ke hānai waiū nei ʻoe?

He mea nui loa ia no kou palekana ponoʻī e ʻōlelo e pili ana i kēia mau mea me ka hūnā ʻole.

Pehea ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?

Hāʻawi ʻia kēia lāʻau lapaʻau ma ke ʻano he inikini i loko o kahi aʻa . ʻOi aku ka pololei, he "infusion" o ka saline, kahi i hāʻawi ʻia i loko o ke kino i loko o kekahi mau hola. ʻAʻole kēia he mea hiki iā ʻoe ke hana ma ka home . Pono e hāʻawi ʻia iā ʻoe e kahi loea mālama ola i aʻo ʻia, e like me ke kauka a kahu maʻi paha, ma kahi haukapila a i ʻole ke keʻena lapaʻau.

Inā hāʻawi ʻoe i kēia lāʻau lapaʻau i nā keiki:

Hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i kekahi manawa i nā pēpē ʻōpiopio e like me 16 mahina, a i nā keiki ʻōpiopio e like me 1 makahiki a me 4 mahina, no nā kūlana i koho ʻia. Eia nō naʻe, aia nā hana palekana kūikawā. No laila, inā ʻoe e hāʻawi ana i kēia i kāu keiki, he mea nui e kamaʻilio pono me kāu kauka pediatric a ʻike i nā kikoʻī.

Ka nui o ka lāʻau:

Inā manaʻo ʻoe ua lawe nui ʻoe i kēia lāʻau lapaʻau, a inā manaʻo ʻoe ua lawe ʻoe, pono ʻoe e kāhea koke i kahi kikowaena kaohi lāʻau make a i ʻole ka lumi pilikia haukapila kokoke loa. He mea nui loa ka hana wikiwiki.

Nā mea nui: Ua kuhikuhi ʻia kēia lāʻau lapaʻau nou wale nō. Mai hāʻawi i kēia lāʻau lapaʻau i kekahi no kekahi kumu , ʻoiai inā loaʻa iā lākou nā hōʻailona like me ʻoe.

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

ʻAʻole maikaʻi ka hoʻokuʻu ʻana i kahi mahele o kēia lāʻau lapaʻau. He lāʻau lapaʻau kēia e pono e lawe mau ʻia a ma nā manawa maʻamau. Inā ʻoe e hala, hiki ke hōʻemi ʻia nā hopena o ka lāʻau lapaʻau. Inā ʻaʻole hiki iā ʻoe ke hele mai a lawe i ka lāʻau lapaʻau i ka lā i hoʻonohonoho ʻia, e kamaʻilio me kāu kauka a loea olakino paha . E haʻi lākou iā ʻoe i ka mea e hana ai ma hope.

Hiki i nā mea ʻē aʻe ke launa pū me kēia lāʻau lapaʻau?

ʻAʻole ʻike ʻia kēia lāʻau lapaʻau e hoʻoulu i nā pilikia nui me nā lāʻau lapaʻau ʻē aʻe (ʻaʻole i manaʻo ʻia nā pilina). ʻO ia hoʻi, kākaʻikahi ia e pane me nā lāʻau lapaʻau ʻē aʻe.

Eia nō naʻe, ʻoi aku ka maikaʻi o ka haʻi ʻana i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei , me nā lāʻau lapaʻau i kauoha ʻia, nā lāʻau lapaʻau i kauoha ʻole ʻia, nā mea kanu, a me nā mea hoʻohui meaʻai. Eia kekahi, 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.Inā hana ʻoe, e haʻi iā lākou e pili ana i ia mea, no ka mea, hiki i kekahi mau mea ke launa pū me kāu lāʻau lapaʻau ma kekahi ʻano a hoʻonui paha i nā hopena ʻaoʻao.

He aha kaʻu e makaʻala ai i ka wā e hoʻohana ai i kēia lāʻau lapaʻau?

ʻOiai ʻoe e lawe ana i kēia lāʻau lapaʻau, he mea nui loa ia e ʻike i kāu kauka no nā nānā mau ʻana . Inā ʻaʻole hoʻomaikaʻi kāu mau hōʻailona a i ʻole e ʻoi aku ka ʻino, e haʻi i kāu kauka. Mai hoʻowahāwahā i kekahi mea.

Aia kekahi papa inoa no nā maʻi me ka Hunter Syndrome. ʻAe kēia iā lākou e hōʻiliʻili i ka ʻike mai iā ʻoe i kahi manawa lōʻihi, ʻo ia hoʻi hiki iā lākou ke aʻo hou aku i ka maʻi. Hiki iā ʻoe ke nīnau i kāu loea mālama ola no ka ʻike hou aku e pili ana i kēia. Hiki iā ʻoe ke hui pū inā makemake ʻoe.

He aha nā hopena ʻaoʻao e hiki ke loaʻa mai kēia lāʻau lapaʻau?

E like me nā lāʻau lapaʻau āpau, hiki i ka lāʻau pale ʻo Idursulfase ke hana i kekahi mau hopena ʻaoʻao. He koʻikoʻi kekahi o lākou a pono e hōʻike koke ʻia i kāu kauka . He mea maʻamau kekahi o lākou a e nalo aku i loko o kekahi mau lā.

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

Inā ʻike ʻoe i kekahi o kēia mau hōʻailona , ​​​​pono ʻoe e haʻi i kāu kauka a i ʻole ke kauka olakino i ka hikiwawe loa. He mau hōʻailona paha kēia o kahi maʻi koʻikoʻi.

• Nā hopena maʻi ʻaleka, e komo pū ana me ka ʻulaʻula o ka ʻili, ka maneʻo a i ʻole ka hives, ka pehu ʻana o ka maka, nā lehelehe, a i ʻole ke alelo. Hiki ke hana ʻia kēia mau mea i ka wā a i ʻole ma hope koke iho o ka hāʻawi ʻana i ka lāʻau pale.
• Nā pilikia hanu. Manaʻo e like me ʻoe e hanu ana, pono e hanu koke.
• ʻEha a ʻūmiʻi paha ka umauma.
• Pōniuniu; manaʻo palupalu a poʻo māmā paha; hāʻule ma muli o ka nalowale o ke kaulike; manaʻo nāwaliwali loa a luhi paha.
• Puʻuwai wikiwiki a kūpono ʻole.
• Kuni a i ʻole ke anu. Hiki i kēia ke lilo i hōʻailona o kahi maʻi.

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

Maʻamau, e emi iho kēia mau mea i loko o kekahi mau lā. Akā inā hoʻomau lākou, a inā hoʻopilikia paha iā ʻoe, e haʻi i kāu kauka.

• Kuke.
• Ka maʻi pākī.
• ʻUlaʻula ka maka. ʻO ke ʻano kēia e lilo ka maka i ʻulaʻula a wela.
• ʻEha poʻo.
• ʻEha ʻiʻo a hui paha.
• Ka luaʻi, ka luaʻi.
• Luhi.

ʻAʻole paha e komo pū kēia papa inoa me nā hopena ʻaoʻao āpau. Inā ʻike ʻoe i kekahi mea ʻē, e kāhea i kāu kauka no ka ʻōlelo aʻo lapaʻau. Hiki iā ʻoe ke hōʻike i kēia mau hopena ʻaoʻao i ka FDA (Food and Drug Administration) ma ʻAmelika Hui Pū ʻIa ma 1-800-FDA-1088. Ma Sri Lanka, hiki iā ʻoe ke hōʻike iā lākou i nā luna olakino pili ma o kāu kauka.

Ma hea ʻoe e mālama ai i kēia lāʻau lapaʻau?

He nīnau kēia a ka poʻe he nui e nīnau ai. ʻAʻole ʻae ʻia ʻoe e mālama i kēia lāʻau lapaʻau Idursulfase ma ka home. Hoʻohana wale ʻia a mālama ʻia i loko o kahi haukapila a i ʻole ke keʻena lapaʻau. ʻO ke kumu o kēia, ʻo ia ke ʻano o ka hāʻawi ʻana a me nā kūlana mahana kūikawā e pono ai ke mālama ʻia.

ʻO ka mea hope loa, he memo koʻikoʻi

He hōʻuluʻulu manaʻo wale nō kēia o nā mea a mākou i kamaʻilio ai? ʻAʻole ia e uhi i nā kikoʻī āpau. He ʻokoʻa ke kūlana o kēlā me kēia kanaka. No laila, inā he mau nīnau a hopohopo paha kāu e pili ana i kēia lāʻau lapaʻau, e ʻoluʻolu e nīnau i kāu kauka, kauka lāʻau, a i ʻole kekahi mea lawelawe olakino ʻē aʻe. Hiki iā lākou ke hāʻawi iā ʻoe i ka ʻike pololei loa no kou kūlana.

Nā mea e pono ai ʻoe e hoʻomanaʻo (Leka Lawe-Home)

ʻAe, eia kekahi mau mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• He lāʻau lapaʻau e hoʻōla ola ai ʻo Idursulfase e pani ana i kahi enzyme i nalowale i loko o ka poʻe me Hunter Syndrome.
• ʻAʻole kēia he lāʻau lapaʻau piha , akā kōkua ia i ka kaohi ʻana i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.
• Ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau , he mea nui e haʻi i kāu kauka e pili ana i kou mau kūlana olakino āpau, nā maʻi allergies, a me nā lāʻau lapaʻau ʻē aʻe āu e lawe nei.
• Hāʻawi ʻia kēia ma ke ʻano he infusion i loko o kahi aa koko ma ka haukapila e kekahi kanaka i aʻo ʻia.
• Mai hōʻalo i kahi mahele lāʻau. Inā ʻaʻole hiki iā ʻoe, e haʻi koke aku i kāu kauka .
• Hiki ke loaʻa nā hopena ʻaoʻao koʻikoʻi a me nā hopena ʻaoʻao maʻamau. E haʻi i kāu kauka e pili ana i kekahi mea ʻē, ʻaʻohe mea liʻiliʻi.
• Nou wale nō kēia lāʻau lapaʻau. Mai hāʻawi aku i kekahi.
• Mai makaʻu e nīnau i kāu kauka i nā nīnau a hopohopo paha āu e loaʻa ai. Loaʻa iā ʻoe nā kuleana āpau e ʻike e pili ana i kou olakino.

ʻIdursulfase , Hunter Syndrome, Enzyme replacement therapy, Elaprase, Nā hopena ʻaoʻao, Lāʻau lapaʻau, Nā maʻi laha ʻole