ʻAʻole pili i ka nui o ka ʻai a kāu keiki liʻiliʻi, ke ʻōlelo nei anei ʻo ia, "ʻAʻole au i māʻona, makemake au i nā mea hou aku?" Ua ʻike anei ʻoe iā ia e noʻonoʻo mau ana i ka meaʻai, e ʻimi ana i ka meaʻai, a i kekahi manawa e ʻaihue malū ana i ka meaʻai ke ʻole kekahi e noho ana? Hiki i kēia ke lilo i pilikia nui a he kumu kaumaha no ka makuahine a makua paha e ʻike ana. I ka hapanui o ka manawa, manaʻo mākou he makeʻe waiwai kēia o ke keiki no ka meaʻai. Akā, ʻaʻole ia ka hihia mau. ʻO ke kumu o kēia pōloli nui he maʻi genetic laha ʻole i kapa ʻia ʻo Prader-Willi Syndrome . I kēia lā, e kamaʻilio mākou e pili ana i kēia, ka pōloli nui e hele pū me ia (`hyperphagia`), a me kona mālama ʻana ma ke ʻano maʻalahi a aloha.
He aha ka Prader-Willi Syndrome?
I ka ʻōlelo maʻalahi, ʻo ka Prader-Willi Syndrome kahi maʻi genetic laha ʻole i hoʻokumu ʻia e kahi loli i loko o kā mākou DNA. I kekahi manawa, hiki ke hana ʻia e kahi ʻeha poʻo nui.
Hoʻopilikia nui kēia ʻano i ka hypothalamus, kahi ʻāpana o ko kākou lolo. E noʻonoʻo i ka hypothalamus ma ke ʻano he kuapo i loko o ko kākou kino. Ke ʻai kākou a māʻona, hoʻouna kēia ʻāpana i kahi hōʻailona iā kākou "ʻAe, ua lawa." Akā i loko o ka lolo o kekahi me Prader-Willi Syndrome, ʻaʻole hana pono kēia "kuʻi". No laila, ʻaʻohe mea nui ka nui o ka meaʻai a lākou e ʻai ai, ʻaʻole lākou e māʻona.
ʻO kēia ke kumu e pōloli mau ai kēia mau keiki. ʻAʻole na lākou ka hewa, ʻaʻole hoʻi ka makeʻe waiwai. He ʻāpana ia o ka maʻi ponoʻī.
Ma muli o kēia pōloli mau (`hyperphagia`), ke ʻimi mau nei lākou i ka meaʻai.
- ʻAi lākou i ka nui o ka meaʻai ma mua o ke kanaka maʻamau.
- Hūnā lākou i ka meaʻai e ʻai ai ma hope.
- I kekahi mau hihia koʻikoʻi, e hoʻowalewale ʻia paha ʻoe e ʻai i ka meaʻai hau, malia paha i ka meaʻai mai ka ʻōpala.
Inā ʻaʻole i kaohi ʻia kēia kūlana, kiʻekiʻe loa ka pilikia o ka loaʻa ʻana o ka momona a me nā pilikia olakino ʻē aʻe (e like me ka maʻi diabetes a me ka maʻi puʻuwai).
Aia kekahi lāʻau lapaʻau no kēia pōloli nui?
ʻAe. Aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i kēia pōloli nui. ʻO Vykat XR (diazoxide choline) kekahi o ia mau lāʻau lapaʻau. Hana kēia lāʻau lapaʻau ma ka hoʻopili ʻana i nā hormones a me nā neurotransmitters i loko o ko kākou lolo e kaohi ana i ka pōloli a me ka makemake, e hōʻemi ana i kēlā manaʻo mau o ka pōloli.
Akā, ʻaʻole kēia he lāʻau lapaʻau e hiki ke lawe wale ʻia ma ka manaʻo wale nō. Pono e hoʻomaka ʻia kēia lāʻau lapaʻau ma lalo o ka nānā ʻana a ke kauka loea.
Pehea ʻoe e hoʻohana ai i kēia lāʻau lapaʻau?
He pila kēia e lawe ʻia i hoʻokahi manawa i ka lā. Hiki ke lawe ʻia me ka meaʻai a i ʻole me ka ʻole o ka meaʻai. Eia nō naʻe , pono e ale ʻia ka papa me ka ʻole o ka haki ʻana, ka wili ʻana, a me ka nau ʻana.
E hoʻomaka kāu kauka iā ʻoe i kēia lāʻau lapaʻau ma kahi mahele haʻahaʻa loa. A laila, i kēlā me kēia ʻelua pule, e hoʻonui mālie lākou i ka mahele. Ua kapa ʻia kēia he 'titration.' Hana ʻia kēia e ʻike i ka mahele mālama e pono ai nou a ʻoi aku ka maikaʻi o ka hoʻomalu ʻana i kāu mau hōʻailona. Inā ʻaʻole hiki iā ʻoe ke lawe i ka lāʻau lapaʻau no hoʻokahi pule a ʻoi aku paha, hiki i kāu kauka ke hoʻomaka hou iā ʻoe ma kahi mahele haʻahaʻa a hoʻonui mālie iā ia.
Pehea ʻoe e ʻike ai inā e hana ana ka lāʻau lapaʻau?
I ka hoʻomaka ʻana o kēia lāʻau lapaʻau e hana, e ʻike ʻoe a i ʻole kāu keiki e emi mālie ana nā mea e like me ka makemake mau i ka meaʻai, ka makemake i ka meaʻai, a me ka pōloli nui. No ka mea, he manawa iki ka lōʻihi o ka hopena o kēia lāʻau lapaʻau, mai manaʻo i kahi loli nui i nā pule mua.
Eia nō naʻe, inā ʻaʻole e hoʻomaikaʻi ana kou makemake ʻai ʻoiai ke lawe nei i ka lāʻau lapaʻau, e kamaʻilio me kāu kauka e pili ana i ia mea. Hiki paha iā ia ke hoʻōki i ka lāʻau lapaʻau a i ʻole e hāʻawi i kahi lāʻau lapaʻau ʻē aʻe inā pono.
He aha nā hopena ʻaoʻao maʻamau a he aha ka mea hiki ke hana ʻia e pili ana iā lākou?
E like me nā lāʻau lapaʻau āpau, hiki iā ia ke hana i nā hopena ʻaoʻao. Akā, ʻaʻole i loaʻa i nā kānaka a pau. E nānā kākou i nā hopena ʻaoʻao maʻamau a pehea e mālama ai iā lākou.
| Hopena ʻaoʻao | Pehea e hoʻokele ai |
|---|---|
| Ka ulu nui ʻana o ka lauoho o ke kino (hypertrichosis) | Hiki ke mālama ʻia kēia. Hiki iā ʻoe ke hoʻohana i nā ʻano wehe lauoho inā pono. Eia nō naʻe, inā ʻoluʻolu ʻole loa ia e kaohi, e kamaʻilio me kāu kauka. |
| ʻO ka pehu ʻana ma muli o ka hōʻiliʻili ʻana o ka wai i loko o ke kino (edema) | Inā ʻike ʻoe i nā hōʻailona e like me ka pehu ʻana o nā wāwae, nā kuʻekuʻe wāwae, a i ʻole nā wāwae, a i ʻole ka paʻakikī o ka hanu ʻana , e haʻi koke i kāu kauka . Pono paha kāu kauka e hoʻōki i ka lāʻau lapaʻau no kekahi manawa, e hoʻemi i ka nui, a i ʻole e kuhikuhi i nā lāʻau lapaʻau ʻē aʻe, e like me nā diuretics. |
| Hoʻonui ʻia ke kō koko | He mea kēia e makaʻala ai. E nānā mau kāu kauka i kou pae kō koko ma mua o kou hoʻomaka ʻana e lawe i ka lāʻau lapaʻau a ʻoiai ʻoe e lawe ana. Inā pono, hiki ke kuhikuhi ʻia ʻoe i nā lāʻau lapaʻau ʻē aʻe e kōkua i ka kaohi ʻana i kāu kō koko. |
| ʻŌhū ʻili | E hoʻohana i nā kopa ʻala ʻole, palupalu. Inā loaʻa ka maneʻo, hiki ke hoʻopili ʻia nā poni e like me ke kuhikuhi a ke kauka. Inā piʻi aʻe ka maneʻo a ʻaʻole paha e emi, e hoʻomaopopo i kāu kauka. |
Nā kūlana koʻikoʻi e pono ai ka mālama kūikawā!
Ua haʻi ʻia mai iā mākou hiki i kēia lāʻau lapaʻau ke hoʻoulu i ke kiʻekiʻe o ke kō koko. I nā hihia kakaikahi, hiki i kēia ke alakaʻi i kahi kūlana weliweli loa i kapa ʻia ʻo Diabetic Ketoacidosis (DKA) . ʻO kēia ka wā e hōʻiliʻili ai nā mea ʻino i kapa ʻia ʻo ketones i loko o ke koko no ka mea ʻaʻole lawa ka insulin o ke kino. He pilikia olakino kēia.
Nā hōʻailona o ke kiʻekiʻe o ke kō:
- Manaʻo makewai ʻē aʻe
- Mimi pinepine
- Manaʻo pōloli ma mua o ka maʻamau
- Ka pohō kaumaha i manaʻo ʻole ʻia
Nā hōʻailona hōʻike o ka Diabetic Ketoacidosis (DKA):
- Ka luaʻi a i ʻole ka luaʻi
- ʻeha ka ʻōpū
- Luhi nui a huikau paha
- Paʻakikī ka hanu ʻana
- ʻAla hua ma ka hanu
- Loaʻa nā pae kō koko kiʻekiʻe loa (e.g., ʻoi aku ma mua o 300 mg/dL)
Inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, e ʻike koke i ke kauka me ka hoʻokaʻulua ʻole. A i ʻole, e hele i ka Emergency Department (ETU) o ka haukapila kokoke loa. He mea nui loa kēia.
E makaʻala i nā pilina me nā lāʻau lapaʻau ʻē aʻe.
Hiki i nā lāʻau lapaʻau ʻē aʻe āu e lawe ai ke hoʻopilikia i ke ʻano o ka hana a Vykat XR. Hiki i kekahi mau lāʻau lapaʻau ke hoʻonui i nā hopena ʻaoʻao o Vykat XR, aʻo nā mea ʻē aʻe e hōʻemi i kona pono.
No laila, e haʻi maopopo i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu a kāu keiki paha e lawe nei (me nā lāʻau lapaʻau i kauoha ʻia, nā lāʻau lapaʻau ma luna o ke kauka, nā huaora, nā lāʻau lapaʻau kuʻuna, nā huahana lāʻau). E hoʻoponopono ʻo ia i ka nui o ka lāʻau a hoʻololi paha i ka lāʻau lapaʻau inā pono. Mai hoʻomaka a hoʻōki paha i kekahi lāʻau lapaʻau me ka ʻole o ke noi ʻana i kāu kauka.
Leka Lawe-Home
- ʻO ka Prader-Willi Syndrome kahi maʻi laha ʻole i hoʻoili ʻia i loko o ka ʻiʻo e hōʻeha ai i ka nalowale o ka manaʻo o ka piha.
- E hoʻomaopopo ʻaʻole hewa kāu keiki i ka pōloli nui a me ka ʻimi ʻana i ka meaʻai, akā, he hōʻailona ia o ka maʻi ponoʻī.
- Hiki i nā lāʻau lapaʻau e like me Vykat XR ke kāohi i kēia pōloli nui, akā pono e hana mau ʻia ma lalo o ka nānā ʻana a kahi loea .
- E makaʻala mau i nā hopena ʻaoʻao, ʻoiai nā hōʻailona o ke kō koko kiʻekiʻe. Inā loaʻa iā ʻoe nā hōʻailona o ka Diabetic Ketoacidosis (DKA), e hele koke i ka Emergency Department (ETU) o ka haukapila.
- He mea nui loa e hoʻomaopopo i kāu kauka e pili ana i nā lāʻau lapaʻau a me nā mea hoʻopihapiha ʻē aʻe āu e lawe nei.
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