Loaʻa i kāu keiki ka PKU (Phenylketonuria)? E kamaʻilio kākou e pili ana i ka pauka Sapropterin!

I kekahi manawa, pono kā mākou mau keiki a me mākou iho e noho me kekahi mau kūlana olakino kūikawā, ʻeā? ʻO kekahi o ia ʻano maʻi ua kapa ʻia ʻo Phenylketonuria (ʻike ʻia hoʻi ʻo PKU no ka pōkole). ʻO kekahi o nā lāʻau lapaʻau i hāʻawi ʻia no kēia maʻi ʻo Sapropterin. No laila i kēia lā, e aʻo kākou e pili ana i kēia lāʻau lapaʻau i kapa ʻia ʻo Sapropterin, e like me ke kamaʻilio ʻana me kahi hoaaloha.

He aha kēia lāʻau lapaʻau? (He aha ʻo Sapropterin?)

I ka ʻōlelo maʻalahi, ʻo Sapropterin kahi lāʻau lapaʻau e kōkua i ka kaohi ʻana i ka hōʻiliʻili nui ʻana o kahi kemika i kapa ʻia ʻo phenylalanine i loko o ke kino o ka poʻe me ke ʻano i kapa ʻia ʻo Phenylketonuria, a i ʻole PKU. E hoʻomanaʻo, ʻo nā ʻāpana liʻiliʻi o ka protein i loko o ka meaʻai a mākou e ʻai ai ua kapa ʻia ʻo amino acids? ʻO Phenylalanine kekahi o ia mau amino acid. ʻAʻole hiki i ke kanaka me PKU ke wāwahi pono i kēia phenylalanine, ʻo ia hoʻi, e hoʻoheheʻe iā ia a hoʻohana iā ia e like me ka mea e pono ai. A laila hōʻiliʻili ia i loko o ke koko a hiki ke hōʻino i ka lolo a me ka ʻōnaehana nerve i ka hala ʻana o ka manawa.

Kōkua kēia lāʻau Sapropterin i ka wāwahi ʻana i kēlā phenylalanine i loko o ke kino. A laila, emi iho kona hōʻiliʻili ʻana i loko o ke koko. I ka manawa pinepine, me kēia lāʻau lapaʻau, aʻo nā kauka i kahi papaʻai kūikawā a i kekahi manawa hoʻoikaika kino. No ka mea, i kēia kūlana, pono ʻoe e hōʻemi i ka nui o ka meaʻai i loaʻa ka phenylalanine i ka hiki.

Hiki i nā kauka ke kuhikuhi i kēia lāʻau lapaʻau no nā kūlana ʻē aʻe, no laila inā he mau nīnau kāu, ʻoi aku ka maikaʻi o ka nīnau ʻana i kāu kauka a mea kūʻai lāʻau paha .

Hiki iā ʻoe ke kūʻai pinepine i kēia lāʻau lapaʻau mai nā hale kūʻai lāʻau lapaʻau ma lalo o nā inoa inoa JAVYGTOR a i ʻole KUVAN.

Nā mea e haʻi aku i kāu kauka ma mua o ka lawe ʻana i kēia lāʻau lapaʻau

Ma mua o kou hoʻomaka ʻana e lawe iā Sapropterin, pono ʻoe e haʻi i kāu kauka e pili ana i kēia mau mea. No ka mea, a laila hiki iā lākou ke hoʻoholo inā kūpono kēia lāʻau lapaʻau iā ʻoe a me ka nui e hāʻawi iā ʻoe. ʻO ka hūnā ʻana i kēia ʻike e hoʻopilikia wale iā ʻoe.

• Loaʻa iā ʻoe kekahi mau maʻi ʻai ? ʻO ia hoʻi, nā kūlana e like me ka anorexia (kahi kūlana noʻonoʻo o ka pohō kaumaha ma ka ʻai ʻole ʻana) a me ka bulimia (kahi kūlana o ka ʻai nui ʻana a laila luaʻi).
• He kuni kou?
• Loaʻa iā ʻoe kekahi maʻi ?
• He maʻi puʻupaʻa kou?
• Maʻi akepaʻaaia ma laila?
• Loaʻa iā ʻoe kahi maʻi e manaʻo ai ʻoe e like me ka loaʻa ʻana o kahi maʻi ( seizures ) ? A i ʻole ua loaʻa mua iā ʻoe kekahi?
• Ua loaʻa anei iā ʻoe kahi maʻi allergy iā Sapropterin, kekahi lāʻau lapaʻau ʻē aʻe, meaʻai, nā kala meaʻai, a i ʻole nā ​​mea hoʻomalu?
• Inā hāpai ʻoe a i ʻole e hoʻolālā ana e hāpai (he mea nui loa kēia).
• Inā he makuahine hānai waiū ʻoe .

He mea nui loa ia no kou olakino ponoʻī e haʻi aku i ke kauka i kēia mau mea me ka hūnā ʻole ʻana iā lākou, ʻeā?

Pehea ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?

ʻAe, e nānā kākou pehea e hoʻohana pono ai i kēia lāʻau lapaʻau. He lāʻau lapaʻau waha kēia. Pono e lawe ʻia me ka ʻai. Pono ʻoe e hahai i nā kuhikuhi ma ka lepili kauoha a kāu kauka i hāʻawi ai iā ʻoe. Mai lawe i ka nui a i ʻole ka liʻiliʻi ma mua o ka nui a kāu kauka i haʻi aku ai iā ʻoe.

Hiki mai kēia ma ke ʻano he pauka. Pono ʻoe e inu ia mea ma ka hoʻoheheʻe ʻana i loko o ka wai a i ʻole ka wai ʻāpala. E nīnau i kāu kauka a kauka lāʻau paha no ka nui pololei o ka wai a i ʻole ka wai ʻāpala āu e lawe ai. Pono e hoʻoheheʻe koke ʻia ka pauka, a pau loa. Pono ʻoe e inu i ka lāʻau i loko o 30 mau minuke o ka hoʻoheheʻe ʻana. Mai waiho no ka lōʻihi ma mua o kēlā.

ʻO kekahi mea ʻē aʻe, hiki iā ʻoe ke hoʻohui i kahi liʻiliʻi o kēia pauka i loko o ka ʻāpala (e like me ka ʻāpala) a i ʻole ka pudding. E nīnau i kāu kauka e pili ana i kēlā, inā ʻoluʻolu ia iā ʻoe e hana pēlā.

E lawe i kāu lāʻau lapaʻau i ka manawa kūpono, i ka manawa like i kēlā me kēia lā inā hiki. Mai lawe ʻoe ma mua o ka mea a kāu kauka e ʻōlelo ai. Eia kekahi, mai hoʻōki koke i ka lawe ʻana i kāu lāʻau lapaʻau me ka ʻole o ke aʻo ʻana a kāu kauka. ʻO ka hana ʻana pēlā e hiki ai ke hoʻonui i kou kūlana.

Pehea e hāʻawi ai i kēia lāʻau lapaʻau i nā keiki liʻiliʻi

I kekahi mau hihia kūikawā, hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā pēpē e like me hoʻokahi mahina. Eia nō naʻe, aia nā hana palekana kūikawā. ʻO ia hoʻi, aia nā mea e makaʻala nui ai. No laila, e kamaʻilio me kāu kauka e pili ana i kēia a e hahai pono i kāna mau kuhikuhi. Hoʻoholo ʻia ka nui o ka lāʻau lapaʻau e like me ke kaumaha o ke keiki.

ʻO ka mea nui loa: Nou wale nō kēia lāʻau lapaʻau. Mai hāʻawi aku i kekahi, ʻoiai inā loaʻa iā lākou nā hōʻailona like me ʻoe. He ʻokoʻa ke kūlana o kēlā me kēia kanaka, no laila ʻaʻole ia he manaʻo maikaʻi e hāʻawi i kēia lāʻau lapaʻau i kekahi me ka ʻole o ka nīnau mua ʻana i ke kauka.

He aha kāu e hana ai inā e hala ʻoe i kahi manawa hoʻopaʻa lāʻau lapaʻau?

E ʻoluʻolu, mai hopohopo i kēlā mau manawa. Inā ʻoe e poina i kahi lāʻau, e lawe koke iā ia i kou hoʻomanaʻo ʻana. Eia nō naʻe, inā kokoke ka manawa no kāu lāʻau aʻe (ʻo ia hoʻi, i loko o kekahi mau hola), e kāpae i ka lāʻau i poina a lawe i ka lāʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe pālua i ka lāʻau.Pono ʻoe e lawe wale i ka nui i kuhikuhi ʻia i kēlā me kēia lā. Inā kānalua ʻoe e pili ana i kēia, e nīnau i kāu kauka a mea kūʻai lāʻau paha.

He aha kāu e hana ai inā lawe nui ʻoe? (Overdose)

Inā manaʻo ʻoe ua lawe nui ʻoe i ka lāʻau lapaʻau, a inā ua lawe hewa kāu keiki i ka nui, e hele koke i ka lumi pilikia o ka haukapila kokoke loa. A i ʻole e kelepona i kahi kikowaena kaohi lāʻau make. Pono e mālama ʻia kēia ma ke ʻano he pilikia , mai hoʻokaʻulua.

Nā lāʻau lapaʻau ʻē aʻe e launa pū me kēia lāʻau lapaʻau (Nā pilina)

Aia kekahi mau lāʻau lapaʻau e hana pono ʻole paha a i ʻole e hoʻopilikia i nā pilikia ʻē aʻe ke lawe ʻia me Sapropterin. Kāhea mākou i kēia mau pilina. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āu e lawe nei.

• ʻAvanafil `( ʻAvanafil )`
• ʻO Levodopa (he lāʻau lapaʻau no ka maʻi o Parkinson)
• ʻO Methotrexate (hoʻohana ʻia no nā maʻi e like me ke kanesa a me ka arthritis)
• ʻO Pemetrexed
• Pyrimethamine (kahi lāʻau lapaʻau i hoʻohana ʻia e mālama i ka malaria)
• ʻO Sildenafil `(Sildenafil)`
• ʻO Tadalafil `(Tadalafil)`
• ʻO Vardenafil

ʻAʻole paha e komo nā lāʻau lapaʻau āpau i kēia papa inoa. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe ai (me nā huaora, nā lāʻau lapaʻau lāʻau, a me nā mea hoʻohui meaʻai). E haʻi pū i kāu kauka inā ʻoe e puhipaka, inu waiʻona, a hoʻohana paha i nā lāʻau lapaʻau kū ʻole i ke kānāwai. Hiki i kekahi mau mea ke launa pū me kāu mau lāʻau lapaʻau.

Nā mea e makaʻala ai i ka wā e lawe ai i ka lāʻau lapaʻau

ʻOiai ʻoe e lawe ana i kēia lāʻau lapaʻau, e nānā pono kāu kauka i kou kūlana. E ʻike pinepine i kāu kauka a e hana mau i nā hoʻāʻo koko. Nānā kēia mau hoʻāʻo koko i kāu mau pae phenylalanine a ʻike inā e hana pono ana ka lāʻau lapaʻau. E hoʻomanaʻo, hiki ke hala a hiki i 4 mau pule no kou kino e pane i kēia lāʻau lapaʻau. No laila, mai hopohopo, e hoʻomanawanui wale.

Inā ʻaʻole hoʻomaikaʻi kou mau ʻōuli, a inā e ʻoi aku ka ʻino, e haʻi koke aku i kāu kauka.

ʻO ka mea nui loa, pono ʻoe e hahai i kahi papaʻai kūikawā i ka wā e lawe ana i kēia lāʻau lapaʻau. Pono ʻoe e ʻai i nā meaʻai haʻahaʻa i ka phenylalanine. He mea nui kēia no ka PKU. Ma mua o ka hana ʻana i kekahi hoʻololi i kāu papaʻai, e kamaʻilio me kāu kauka a i ʻole he meaʻai meaʻai/meaʻai. Hiki iā lākou ke hāʻawi iā ʻoe i kahi papa inoa o nā meaʻai maikaʻi nou a me nā ʻōlelo aʻoaʻo no ka hoʻomākaukau ʻana i kāu mau meaʻai.

E noʻonoʻo, ʻoiai ʻo Nalini Akka e hāʻawi ana i kēia lāʻau lapaʻau i kāna kaikamahine nona ka PKU, ke kāohi nei ʻo ia i kāna ʻai e like me ka ʻōlelo a ke kauka iā ia. Ma mua o kona hoʻomākaukau ʻana i ka meaʻai, ʻimi mau ʻo ia i nā meaʻai haʻahaʻa i ka phenylalanine. Ke hele ʻo ia i ka hale kūʻai, heluhelu ʻo ia i nā lepili ma nā ʻeke meaʻai. Pono kēlā ʻano mālama i kēia kūlana.

He aha nā hopena ʻaoʻao?

E like me nā lāʻau lapaʻau āpau, hiki iā Sapropterin ke hana i nā hopena ʻaoʻao i kekahi poʻe. Eia naʻe, ʻaʻole loaʻa i nā kānaka a pau. E nānā kākou i ke ʻano o lākou.

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 koke i kāu kauka. Hiki i kēia ke lilo i mea koʻikoʻi.

• Nā hopena aleri: ʻŌhūhū ʻili, maneʻo, ʻulaʻula o ka ʻili, pehu ʻana o ka maka, nā lehelehe, ke alelo a i ʻole ka puʻu (hiki i kēia ke paʻakikī i ka hanu ʻana).
• Hyperactivity: Hoʻomaha ʻole, hoʻopunipuni, kamaʻilio nui ʻana, a me ka paʻakikī i ka hoʻolohe ʻana.
• Ka mumū ʻana o ka esophagus a i ʻole ka ʻōpū: Ka nalowale o ka makemake, ka ʻeha o ka ʻāʻī, ka ʻeha a i ʻole ka paʻakikī i ka moni ʻana, ka wela i ka umauma, ka nausea, ka luaʻi ʻana, ka ʻū maloʻo, ka ʻōpala koko a ʻūlū paha.

Nā hopena ʻaoʻao ʻaʻole pono ka ʻōlelo aʻoaʻo lapaʻau, akā pono e hōʻike i ke kauka inā hoʻomau lākou:

Hoʻopau pinepine kēia 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
• ʻEha poʻo
• Ihu wai a i ʻole ka ihu paʻa
• ʻeha ka ʻāʻī
• Ka luaʻi ʻana

ʻAʻole paha e komo pū kēia papa inoa me nā hopena ʻaoʻao āpau. Inā ʻike ʻoe i kekahi mea ʻē aʻe i ʻike ʻole ʻia, e nīnau i kāu kauka e pili ana i ia mea.

Pehea ʻoe e mālama ai i ka lāʻau lapaʻau?

He mea nui hoʻi e mālama pono i kēia lāʻau.

• E mālama iā ia mai ka hiki ʻana aku o nā keiki a me nā holoholona.
• E mālama ma ka mahana o ka lumi (ma waena o 20 a me 25 kekelē Celsius, ma waena o 68 a me 77 kekelē Fahrenheit). Mai hoʻokuʻu i ka wela nui a i ʻole ke anu.
• E mālama i kēia lāʻau lapaʻau i loko o ka pahu kumu i hoʻokomo ʻia ai.
• E pani pono i ka ʻōmole i ʻole e puka ka wai.
• Mai hoʻohana i nā lāʻau lapaʻau i pau ka lā a e hoʻolei aku me ke akahele. Hiki iā ʻoe ke nīnau i kāu hale kūʻai lāʻau lapaʻau no nā kuhikuhi no ke ʻano o ka hoʻolei pono ʻana.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻĀ, ua kamaʻilio nui mākou e pili ana iā Sapropterin, ʻeā? Hiki ke lilo ka noho ʻana me kahi maʻi i kapa ʻia ʻo PKU i mea paʻakikī, akā me nā lāʻau lapaʻau e like me kēia a me nā ʻōlelo aʻoaʻo lapaʻau kūpono, hiki iā ʻoe a me kāu keiki ke lanakila ma luna o kēlā pilikia.

"E hoʻomanaʻo, he mea nui loa ka lawe ʻana i kēia lāʻau lapaʻau e like me ke kuhikuhi a ke kauka, ma ka nui i kuhikuhi ʻia, a ma ka manawa i kuhikuhi ʻia. Eia kekahi, he mea nui e hahai i kahi papaʻai kūikawā.""Inā ʻoe e hana pono i kēia mau mea ʻelua, e hiki iā ʻoe ke hoʻomalu pono i kou kūlana PKU a ola i kahi ola olakino."

Inā he mau nīnau, hopohopo, a kānalua paha kāu, mai hūnā iā ʻoe iho, akā e ʻoluʻolu e nīnau i kāu kauka. Aia lākou ma laila e kōkua iā ʻoe. ʻAʻole ʻoe hoʻokahi ma kēia huakaʻi, aia mākou a pau me ʻoe!

Ke makemake nei iā ʻoe a me kāu pēpē i ke olakino maikaʻi!

ʻO Sapropterin, Sapropterin, PKU, Phenylketonuria, Phenylketonuria, Phenylalanine, phenylalanine, lāʻau lapaʻau, olakino keiki, nā maʻi hoʻoilina