Loaʻa iā ʻoe kekahi Urea Cycle Disorder (UCD)? E kamaʻilio kākou e pili ana i ka lāʻau lapaʻau Sodium Phenylbutyrate!

Hana ʻia nā kaʻina hana kemika like ʻole i loko o ko mākou kino e hana i nā hana like ʻole. No laila, i kekahi manawa inā ʻaʻole hana pono kēia mau kaʻina hana, hiki ke loaʻa nā maʻi like ʻole. ʻO 'Urea Cycle Disorders' (UCDs) kekahi o ia ʻano. ʻO ka mea e hana ʻia ma kēia, ʻaʻole hiki ke hoʻoneʻe pono ʻia kahi huahana ʻōpala i kapa ʻia ʻo ammonia i hana ʻia i loko o ko mākou kino. A laila piʻi ka nui o ka ammonia i loko o ke koko a hiki ke kū mai nā pilikia olakino like ʻole. I kēia lā e kamaʻilio mākou e pili ana i kahi lāʻau i kapa ʻia ʻo 'Sodium Phenylbutyrate', i hāʻawi ʻia i kahi kanaka me ke ʻano UCD.

He aha kēia lāʻau lapaʻau? No ke aha i hāʻawi ʻia ai?

I ka ʻōlelo maʻalahi, ʻo Sodium Phenylbutyrate kahi lāʻau lapaʻau i hāʻawi ʻia i nā poʻe me nā maʻi pōʻaiapuni urea (UCD). Hana ia ma ke kōkua ʻana i ka hōʻemi ʻana i ka nui o ka ammonia e hōʻiliʻili ana i loko o kou kino. Hoʻemi kēia i ka pilikia o nā pilikia olakino koʻikoʻi e hiki ke hana ʻia e nā pae kiʻekiʻe o ka ammonia. Akā e hoʻomanaʻo, ʻaʻole ia e ho'ōla loa i ka UCD. He lāʻau lapaʻau ia e kōkua ana i ka kaohi ʻana i nā hōʻailona a hāʻawi iā ʻoe i kahi hōʻoluʻolu.

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

He aha nā mea e pono iaʻu e haʻi aku i kaʻu kauka ma mua o ka hoʻohana ʻana i kēia lāʻau?

Ma mua o kou hoʻomaka ʻana e lawe i ka Sodium Phenylbutyrate, aia kekahi mau mea e pono ai ʻoe e haʻi i kāu kauka a i ʻole ke kime lapaʻau e mālama nei iā ʻoe. E kōkua kēia ʻike iā lākou e hoʻoholo inā kūpono kēia lāʻau lapaʻau iā ʻoe a me ka nui e hāʻawi iā ʻoe.

E nānā inā loaʻa iā ʻoe kekahi o kēia mau kūlana:

Inā loaʻa iā ʻoe kekahi o kēia mau mea, pono ʻoe e haʻi i kāu kauka:

• Maʻi Diabetes: Inā loaʻa iā ʻoe ka maʻi diabetes.
• ʻAi paʻakai haʻahaʻa: Inā ʻoe ma ka papaʻai paʻakai haʻahaʻa, a inā paha ua haʻi aku kāu kauka iā ʻoe e ʻai i ka papaʻai paʻakai haʻahaʻa.
• Ka hāʻule ʻana o ka puʻuwai: Inā he puʻuwai nāwaliwali kou a he maʻi puʻuwai paha.
• Maʻi puʻupaʻa: Inā he maʻi kou i loko o kou mau puʻupaʻa.
• Nā pae haʻahaʻa o ka potassium i loko o ke koko: Inā ua ʻike mua ʻia ʻoe me ka potassium haʻahaʻa i loko o kou koko.
• Maʻi akepaʻa: Inā he maʻi kou i loko o kou akepaʻa.
• Ka pehu ʻana o nā kuʻekuʻe wāwae, nā wāwae, nā lima: Inā pehu kou mau wāwae a me kou mau lima.
• He hopena maʻamau ʻole a maʻi paha i ka sodium phenylbutyrate, nā lāʻau lapaʻau ʻē aʻe, nā meaʻai, nā kala, a i ʻole nā ​​​​mea mālama:Inā he maʻi allergy kou i kēia lāʻau lapaʻau a i ʻole kekahi mea ʻē aʻe.
• Hāpai a ho'āʻo paha e hāpai: Inā ʻoe e kali ana i kahi pēpē a hāpai paha.
• Inā he makuahine hānai ʻoe (Hānai waiū): Inā ʻoe e hānai waiū ana i kāu pēpē.

Pehea pololei e pono ai iaʻu ke hoʻohana i kēia lāʻau lapaʻau?

He mea nui loa e hoʻohana i kēia lāʻau lapaʻau e like me ka ʻōlelo a ke kauka.

• Pono e lawe waha ʻia kēia lāʻau lapaʻau.
• E lawe i kāu lāʻau lapaʻau i ka manawa like i kēlā me kēia lā , e like me ke kuhikuhi ʻana ma ka lepili lāʻau.
• Pono ʻoe e hoʻohana i kahi puna i hōʻailona kūikawā ʻia e ana i kēia lāʻau. E nīnau ma ka hale kūʻai lāʻau inā ʻaʻole hiki iā ʻoe ke loaʻa. ʻAʻole pololei nā punetēpē maʻamau a me nā punetēpē i hoʻohana ʻia ma ka home, no laila mai hoʻohana.
• Pono e hoʻohuihui ʻia ka pauka lāʻau me kahi wai liʻiliʻi, kahi wai ʻē aʻe, a i ʻole kekahi meaʻai e inu ai.
• E ale koke iā ia ma hope o ke kāwili ʻana. Mai paʻa.
• E hoʻomau i ka lawe ʻana i ka lāʻau a hiki i ka wā e ʻōlelo aku ai kāu kauka a i ʻole ka hui lapaʻau e hoʻōki.

He mea nui: E haʻi aku paha kāu kauka iā ʻoe e hahai i kahi papaʻai haʻahaʻa protein i ka wā e lawe ana i kēia lāʻau lapaʻau. He mea nui loa ia e hahai pono i kēia papaʻai. Hiki ke haʻi ʻia aku kekahi poʻe e lawe i nā huaora keu a me nā mea hoʻohui meaʻai.

Inā hāʻawi ʻoe i kēia lāʻau lapaʻau i nā keiki:

Hiki ke hāʻawi ʻia kēia lāʻau lapaʻau i nā keiki ma kekahi mau kūlana i koho ʻia. Eia nō naʻe, aia kekahi mau mea e makaʻala ai i ka hāʻawi ʻana. No laila, inā ua kuhikuhi ʻia kēia lāʻau lapaʻau i kāu keiki, e kamaʻilio pono me kāu kauka e pili ana i ia mea a e ʻike i nā kikoʻī.

Inā lawe ʻoe i ka nui o ka lāʻau lapaʻau (Overdosage):

Inā manaʻo ʻoe ua lawe ʻoe i nā lāʻau lapaʻau ma mua o kou manaʻo, e hele koke i ka lumi pilikia haukapila kokoke loa, a i ʻole e kelepona i kahi kikowaena kaohi lāʻau make.

Hoʻokahi mea hou aʻe: Ua kuhikuhi ʻia kēia lāʻau lapaʻau nou wale nō . No laila, mai hāʻawi aku i kekahi, ʻoiai inā ʻōlelo lākou he maʻi like ko lākou me ʻoe.

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

I kekahi manawa hiki ke hala i kahi lāʻau lapaʻau, ʻeā? Inā pēlā, e lawe i ka lāʻau lapaʻau i hala koke i kou hoʻomanaʻo ʻana. Eia nō naʻe, inā kokoke i ka manawa no kāu lāʻau lapaʻau aʻe, e kāpae i ka lāʻau lapaʻau i hala a lawe i kāu lāʻau lapaʻau aʻe i ka manawa i hoʻonohonoho ʻia. Mai lawe i ʻelua mau lāʻau lapaʻau i ka manawa hoʻokahi a i ʻole e lawe i ʻoi aku ma mua o ka lāʻau lapaʻau i kuhikuhi ʻia.

He aha nā mea ʻē aʻe e hiki ke launa pū me kēia lāʻau lapaʻau?

Ke lawe ʻoe i ka Sodium Phenylbutyrate, hiki iā ia ke launa pū me nā lāʻau lapaʻau ʻē aʻe, nā mea kanu, nā huaora, a i ʻole nā ​​​​lāʻau lapaʻau ʻole i kauoha ʻia āu e lawe nei. ʻO ka hana ʻana pēlā e hoʻopilikia paha i ka hana maikaʻi o kēia lāʻau lapaʻau a hoʻonui paha i ka pilikia o nā hopena ʻaoʻao.

Aia ma lalo iho kekahi mau lāʻau lapaʻau e hiki ke launa pū me kēia lāʻau lapaʻau:

• Haloperidol
• ʻO Probenecid
• Nā lāʻau steroid, e like me prednisone a i ʻole cortisone
• ʻAkika Valproic

ʻAʻole i hoʻokomo ʻia nā mea āpau ma kēia papa inoa. No laila, e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau, nā mea kanu, nā huaora, a me nā mea hoʻohui meaʻai āu e lawe ai, a inā ʻoe e puhipaka, inu waiʻona, a hoʻohana paha i nā lāʻau lapaʻau kū ʻole i ke kānāwai. Hiki iā lākou ke hāʻawi iā ʻoe i nā ʻōlelo aʻoaʻo kikoʻī.

He aha nā mea ʻē aʻe e pono iaʻu e makaʻala i ka wā e lawe ai i kēia lāʻau?

Inā ʻaʻole hoʻomaikaʻi kāu mau ʻōuli a i ʻole e ʻoi aku ka ʻino o ka wā e hoʻohana ana i kēia lāʻau lapaʻau, e haʻi koke aku i kāu kauka a i ʻole ka hui lapaʻau.

Eia kekahi, hiki i kāu kauka ke kauoha i ka hana koko e nānā i kou mau pae koko (e.g., nā pae ammonia) ʻoiai ʻoe e lawe ana i kēia lāʻau lapaʻau. He mea nui e hana mau i kēia mau mea.

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

E like me nā lāʻau lapaʻau āpau, hiki i ka Sodium Phenylbutyrate ke hana i nā hopena ʻaoʻao i kekahi poʻe. ʻAʻole loaʻa i nā kānaka a pau, akā he mea maikaʻi ke makaʻala i nā mea hiki ke loaʻa.

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

Inā loaʻa iā ʻoe kekahi o nā hopena ʻaoʻao ma lalo nei, e haʻi koke aku i kāu kauka a i ʻole ka hui lapaʻau, no ka mea, he mea koʻikoʻi paha lākou :

• Nā hopena maʻi ʻaleka: ʻŌhūhū ʻili, maneʻo, hives, pehu o ka maka, lehelehe, alelo, a i ʻole ka puʻu. Inā hana kēia, hiki ke pilikia ka hanu ʻana, no laila e hōʻike koke aku.
• Pōniuniu, nalowale ke kaulike a i ʻole ka hoʻonohonoho ʻana, huikau a pilikia paha i ka ʻōlelo ʻana: Inā manaʻo ʻoe ua pōniuniu, kūpaʻa ʻole i ka hele ʻana, hiki ʻole ke noʻonoʻo, a i ʻole ka ʻōlelo ʻana me ka leo ʻole.
• Ka pae potassium haʻahaʻa: ʻO nā hōʻailona e pili ana i ka ʻeha o nā ʻiʻo a i ʻole nā ​​​​ʻūlū, nāwaliwali a luhi paha, ka puʻuwai wikiwiki a i ʻole ka maʻamau ʻole, a me ka paʻa ʻana.
• Ka pehu ʻana o nā kuʻekuʻe wāwae, nā lima, a i ʻole nā ​​​​wāwae.

Nā hopena ʻaoʻao ʻaʻole ia he mea nui, akā inā hoʻomau lākou, pono ʻoe e haʻi i kāu kauka :

ʻAʻole koʻikoʻi nā hopena ʻaoʻao ma lalo nei. Eia nō naʻe, inā hoʻomau a hoʻopilikia paha lākou iā ʻoe, e haʻi i kāu kauka e pili ana iā lākou:

• ʻAla kino: ʻAʻole like me ka hou, akā he ʻano ʻala ʻokoʻa.
• Ka loli ʻana o ka ʻono: Ke manaʻo nei ua loli ka ʻono o ka meaʻai.
• Hiki i nā wāhine ke loaʻa i nā menstruation kūpono ʻole a i ʻole nā ​​​​​​flatulence spotting.
• Ka nalowale o ka makemake.

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

Pehea e mālama pono ai i kēia lāʻau lapaʻau?

He mea nui loa hoʻi e mālama pono i nā lāʻau lapaʻau.

• E mālama i kēia lāʻau lapaʻau mai ka hiki ʻana aku o nā keiki a me nā holoholona .
• Pono e mālama ʻia ma kahi mahana ma waena o 15 a me 30 kekelē Celsius (59 a me 86 kekelē Fahrenheit), ʻo ia hoʻi ka mahana maʻamau o ka lumi.
• E pale iā ia mai ka makū. ʻO ia hoʻi, mai waiho i loko o nā wahi pulu e like me ka lua.
• E pani paʻa i ka waha o ka ʻōmole/ʻeke lāʻau lapaʻau.
• Mai hoʻohana i ka lāʻau lapaʻau ma hope o kona lā pau . E hoʻolei aku i nā lāʻau lapaʻau i pau.

Pehea ʻoe e hoʻolei aku ai i ka lāʻau lapaʻau ke pau kāna hoʻohana ʻana a ua pau paha kona lāʻau?

ʻAʻole maikaʻi ka hoʻolei wale ʻana i nā lāʻau lapaʻau i pau ka manawa a i ʻole nā ​​lāʻau lapaʻau i makemake ʻole ʻia i loko o ka ʻōpala a i ʻole i loko o ka lua. Hiki iā lākou ke pau i ke kaiapuni. No laila, pono ʻoe e hoʻolei pono i kēia mau lāʻau lapaʻau.

Hiki iā ʻoe ke hana i kēia ma nā ʻano ʻelua:

1. Hāʻawi i kahi papahana lawe lāʻau lapaʻau: Loaʻa i kekahi mau hale kūʻai lāʻau lapaʻau a i ʻole nā ​​​​mākaʻi kēia mau papahana. E nānā e ʻike inā aia kekahi ma kou wahi.

2. Inā ʻaʻohe ala e hoʻihoʻi ai: E nānā i ka ʻeke lāʻau a i ʻole ka pepa liʻiliʻi i loko e ʻike ai inā hiki ke hoʻolei ʻia kēia lāʻau me ka ʻōpala a hoʻolei ʻia paha i loko o ka lua. Inā kānalua ʻoe, e nīnau i kāu kauka a kauka lāʻau paha.

Inā hiki iā ʻoe ke hoʻolei aku me ka ʻōpala, eia ke ʻano:

• E lawe i ka lāʻau lapaʻau mai loko mai o ka ʻōmole a i ʻole ka ʻeke i loaʻa mai ai.
• E kāwili i ka pauka lāʻau lapaʻau me ka ʻōpala pōpoki, ka lepo, ka ʻōpala kope, a i ʻole kekahi mea ʻē aʻe i pono ʻole.
• E kau i kēia hui ʻana i loko o kahi ʻeke a i ʻole kahi pahu hiki ke hoʻopaʻa pono ʻia.
• A laila e hoʻolei i loko o ka ʻōpala.

Ma ka hoʻolei ʻana i nā lāʻau lapaʻau ma kēia ʻano, hiki iā ʻoe ke pale iā lākou mai ka hāʻule ʻana i nā lima hewa a me ka hōʻino ʻana i ke kaiapuni.

ʻO nā mea nui e hoʻomanaʻo i ka pōkole

ʻAe, ua kamaʻilio nui mākou e pili ana i ka Sodium Phenylbutyrate. I mea e maʻalahi ai iā ʻoe ke hoʻomanaʻo, e hōʻuluʻulu kākou i nā mea nui loa:

• Hāʻawi nui ʻia kēia lāʻau lapaʻau i nā poʻe me nā maʻi pōʻaiapuni urea (UCDs) e hōʻemi i nā pae ammonia i loko o ke kino.
• ʻAʻole kēia e ho'ōla loa i ka maʻi, akā kōkua ia i ka kaohi ʻana i nā hōʻailona.
• E hōʻoia e haʻi i kāu kauka e pili ana i nā maʻi ʻē aʻe āu i loaʻa ai (ʻoiai ka maʻi diabetes, ka maʻi puʻuwai, ka maʻi puʻupaʻa/akepaʻa), nā lāʻau lapaʻau ʻē aʻe āu e lawe nei, nā maʻi allergies, a me ka hāpai ʻana ma mua o ka lawe ʻana i ka lāʻau.
• E lawe i ka lāʻau lapaʻau i ka nui i kuhikuhi ʻia, i ka manawa i kuhikuhi ʻia, me kahi puna kūikawā, a e like me ka ʻōlelo a kāu kauka iā ʻoe . He mea nui nō hoʻi e hahai i ka ʻai haʻahaʻa-protein .
• Inā ʻoe e poina i kahi lāʻau lapaʻau, e lawe koke iā ia i kou hoʻomanaʻo ʻana. Akā inā kokoke ka manawa no kāu lāʻau lapaʻau aʻe, e kāpae i ka lāʻau lapaʻau i poina. Mai lawe pālua i ka lāʻau lapaʻau.
• Inā loaʻa iā ʻoe nā hopena ʻaoʻao koʻikoʻi (allergy, paʻakikī ka hanu ʻana, poniuniu nui, pehu ʻana o nā lālā) , e haʻi koke i kāu kauka.
• E mālama i ka lāʻau lapaʻau ma kahi maloʻo a anuanu, kahi i hiki ʻole ai i nā keiki a me nā holoholona ke kiʻi aku. E hoʻolei pono i ka lāʻau lapaʻau i pau ka lāʻau.
• Nou wale nō kēia lāʻau lapaʻau. Mai hāʻawi aku i nā poʻe ʻē aʻe.

Inā he mau nīnau a hopohopo paha kāu e pili ana i kēia lāʻau lapaʻau, mai kānalua e kūkākūkā me kāu kauka a kauka lāʻau paha. Aia lākou ma laila e kōkua iā ʻoe. Ke makemake nei iā ʻoe e ho'ōla koke!

ʻO Sodium Phenylbutyrate, Nā Maʻi Pōʻaiapuni Urea, UCD, ammonia, lāʻau lapaʻau, nā hopena ʻaoʻao