Manaʻo mākou a pau i ka poniuniu a i ʻole ke poʻo māmā i kekahi manawa ke ala mākou mai kahi noho a i ʻole kahi moe. ʻO ka hapa nui o ka manawa, nalowale ia i loko o kekahi mau kekona. Akā inā hana pinepine kēia iā ʻoe, a inā manaʻo ʻoe e aneane maule a hāʻule, ʻaʻole paha he mea maʻamau. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi laha ʻole e hoʻoulu ai i kēia mau hōʻailona. ʻO ia ka Pure Autonomic Failure, a i ʻole PAF no ka pōkole.
I ka ʻōlelo maʻalahi, he aha ka Pure Autonomic Failure (PAF)?
E noʻonoʻo e pili ana i ia mea, he nui nā kaʻina hana i loko o ko kākou kino e hana aunoa me ka ʻole o kā kākou kaohi. Eia kekahi laʻana, ka hanu ʻana, ka puʻuwai, ka ʻai ʻana, ka kaohi ʻana i ka mahana o ke kino, a me ka kaohi ʻana i ke koko. Hoʻomalu ʻia kēia mau mea a pau e kā kākou ʻōnaehana nerve ponoʻī. Ua like ia me ka ʻōnaehana 'auto-pilot' i loko o ko kākou kino.
ʻO ka Pure Autonomic Failure (PAF) kahi maʻi neurological kakaʻikahi e hoʻonāwaliwali mālie i ka hana o ka ʻōnaehana nerve autonomic i ka hala ʻana o ka manawa. ʻO ka mea nui e hana ʻia ma kēia hihia, ʻo ia ke kū koke ʻoe mai kahi noho a moe paha, e hāʻule nui kou koko . Ma nā ʻōlelo lapaʻau, kapa mākou i kēia he orthostatic hypotension . Akā, ʻaʻole kēia wale nō ka pilikia, hiki i ka PAF ke hana i nā pilikia ʻē aʻe he nui.
ʻIke pinepine ʻia kēia maʻi i nā pākeke ma waena o 40 a me 60 mau makahiki. I ka wā ma mua, ua kapa ʻia kēia maʻi ʻo Bradbury-Eggleston syndrome.
He aha nā hōʻailona o ka maʻi PAF?
E like me kā mākou i kūkākūkā ai ma mua, ʻo ka hōʻailona maʻamau a nui loa, ʻo ia ka poniuniu a me ka māʻule ʻana i ke kū ʻana (orthostatic hypotension). Eia nō naʻe, ʻoiai ʻo kēia ʻōnaehana nerve autonomic ka mea e kaohi ana i nā ʻōkana he nui i loko o ko mākou kino, ʻaʻole i kaupalena ʻia nā hōʻailona i hoʻokahi wale nō.
ʻO ka mea nui, ʻaʻole e like ka ʻike ʻana o kēia mau hōʻailona a i ʻole ma ke kaʻina like no nā kānaka a pau. Hiki i kekahi poʻe ke ʻike i nā hōʻailona ʻē aʻe ma mua o ko lākou manaʻo ʻana i ka poniuniu ke kū lākou i luna.
E nānā kākou i nā hōʻailona ʻē aʻe e hiki ke hana ʻia e PAF.
| ʻŌnaehana/kaʻina hana i hoʻopilikia ʻia | Nā hōʻailona hiki ke loaʻa |
|---|---|
| Ka hou ʻana o ke kino | ʻO ka maʻamau, aia ka emi ʻana o ka hou ʻana (anhidrosis) a i kekahi manawa ka hou nui ʻana (hyperhidrosis). |
| ʻIke maka | Ka ʻike pōwehiwehi ma muli o ka nui ʻole o nā haumāna. |
| ʻŌnaehana mimi | Ka paʻakikī i ka hoʻokuʻu ʻana i ka ʻōpū mimi loa (paʻa ʻana o ka mimi) a i ʻole ka hiki ʻole ke kāohi i ka mimi (urinary incontinence). |
| ʻŌnaehana hoʻoheheʻe | Ka paʻa ʻana o ka ʻōpū a i ʻole ka hoʻōki ʻana i ka ʻōpū. |
| Hana moekolohe | Nā pilikia e like me ka erectile dysfunction a i ʻole anorgasmia. |
Eia kekahi, hiki i ka hapalua o ka poʻe me PAF ke loaʻa i ka anemia māmā.
He aha ke kumu o ka hoʻokumu ʻia ʻana o PAF?
Ua ʻike ʻia ke kumu nui o kēia ʻo ia ka hōʻiliʻili ʻana o kahi protein i kapa ʻia ʻo alpha-synuclein i loko o kā mākou mau cell nerve autonomic. E like me ka lepo e pani ana i ka paipu, ke hōʻiliʻili kēia protein i loko o nā cell nerve, ua hoʻopilikia ʻia kā lākou hana.
Hoʻokomo ʻia kēia protein i loko o nā puʻupuʻu aʻalolo (autonomic ganglia) ma nā ʻaoʻao ʻelua o ke kaula kuamoʻo a kokoke paha i nā ʻōpū. I ka hala ʻana o ka manawa, emi iho ka helu o kēia mau hunaola aʻalolo, a ua hoʻopilikia ʻia ka hana o ka ʻōnaehana autonomic. Akā ʻo ka mea nui loa , ʻaʻole i hōʻino ʻia ka lolo a me ke kaula kuamoʻo i loko o ka PAF . No laila, ʻaʻole ia e hoʻopilikia i nā mea e like me ka hoʻomanaʻo a me ka hiki ke noʻonoʻo.
ʻAʻole maopopo i nā kānaka noiʻi ke kumu i waiho ʻia ai kēia protein e like me kēia.
Pilina me nā maʻi ʻē aʻe
Eia kekahi mea nui e ʻike ai. ʻO kēia protein like, ʻo alpha-synuclein, ka mea e hōʻiliʻili ai i loko o ka lolo i nā maʻi e like me ka maʻi Parkinson, Multiple System Atrophy, a me Lewy Body Dementia. No laila, loaʻa i kekahi poʻe me PAF (ʻaʻole nā mea āpau) ka manawa e loaʻa ai kekahi o kēia mau maʻi i ka hala ʻana o ka manawa. E hōʻike kāu kauka iā ʻoe e pili ana i kēia.
Pehea e ʻike ai i kēia maʻi?
He hana paʻakikī ka ʻike ʻana i ka PAF, ʻoiai e hoʻopau mua nā kauka i nā kūlana ʻē aʻe e hiki ke hoʻoulu i kāu mau hōʻailona, ʻoiai ka hāʻule ʻana o ke koko ke kū ʻoe.
Hiki i ke kaʻina hana diagnostic ke komo pū me:
- E nīnau pono e pili ana i kāu mau hōʻailona a me kāu mōʻaukala olakino.
- He hoʻokolokolo kino piha a me ka hoʻokolokolo neurological.
Ma hope o kēlā, hiki ke ʻōlelo ʻia kekahi mau hoʻokolohua kūikawā e hōʻoia i ka maʻi.
| Hoʻāʻo | E hana wale i kēia... |
|---|---|
| Hoʻāʻo Papa Hoʻokiʻekiʻe | E kau ʻia ʻoe ma luna o kahi moe kūikawā a laila e hoʻoneʻe koke ʻia i kahi kūlana kū. E nānā ʻia kou koko a me kou puʻuwai. |
| QSART (hoʻāʻo hou) | Nānā kēia i ke ʻano o ka hana ʻana o nā aʻalolo e kāohi ana i ka hou ʻana. Kōkua kēia i ka ʻike ʻana i nā pilikia me ka ʻōnaehana nerve autonomic. |
| Nā hoʻokolohua kiʻi (e like me MRI) | Hana ʻia nā mea e like me ke kiʻi MRI lolo e hōʻoia i ʻaʻohe pilikia olakino ʻē aʻe e hiki ke hoʻoulu i nā hōʻailona. |
| Nā Hoʻāʻo Keʻena Hoʻokolohua (Koko a me ka Mimi) | Ana ʻia nā pae o kahi kemika i kapa ʻia ʻo norepinephrine i loko o ke koko a me ka mimi i ka wā e moe ana a ma hope o ke kū ʻana. I ka PAF, aia kahi loli maʻamau i kēia mau pae. |
Hiki ke paʻakikī ke loaʻa ʻana o nā hoʻokolohua he nui, akā ma o ka ʻike pololei ʻana i ka maʻi hiki iā mākou ke hana i ka hoʻolālā lapaʻau maikaʻi loa nāu.
He aha nā lāʻau lapaʻau no PAF?
ʻAʻohe lāʻau lapaʻau no kēia. Akā, mai hopohopo. Nui nā mea hiki iā ʻoe ke hana e kōkua i ka mālama ʻana i kāu mau hōʻailona a ola i kahi ola maʻamau. E hana pū kāu kauka a me ka hui lapaʻau me ʻoe e hoʻomohala i kahi hoʻolālā lapaʻau e pili pono ana iā ʻoe.
Kaohi ʻana o ka Orthostatic Hypotension (ke koko haʻahaʻa i ke kū ʻana)
ʻO kēia ka pilikia nui. Aia nā lāʻau lapaʻau ʻaʻole me ka lāʻau lapaʻau a me nā lāʻau lapaʻau no kēia.
- E makaʻala i ka wā e hoʻololi ai i nā kūlana: Ke ala aʻe mai kahi moe a noho paha, e hana mālie loa . Mai ala koke.
- Wai a me ka paʻakai: Manaʻo paha kāu kauka e inu ma kahi o 3 lita o ka wai i ka lā. Hiki paha iā ia ke ʻōlelo aku iā ʻoe e hoʻohui i kahi paʻakai hou aʻe i kāu meaʻai.
- Hoʻomākaukau moe: E hiamoe me ke poʻo o ka moe i hāpai ʻia ma kahi o 4-6 ʻīniha. Ma kēlā ʻano, ʻaʻole ʻoe e ʻike nui i ka ʻokoʻa o ke koko ke ala ʻoe.
- Nā lole hoʻopaʻa: ʻO ke komo ʻana i nā stocking paʻa kūikawā (nā lole hoʻopaʻa) e piʻi aʻe i ka pūhaka a i ʻole nā ʻūhā e kōkua i ka hōʻemi ʻana i ka hōʻiliʻili ʻana o ke koko ma nā wāwae a mālama i ke koko i paʻa.
- Ma waho aʻe o kēia, ua ʻōlelo pū ke kauka i nā lāʻau lapaʻau kūikawā e hoʻonui ai i ke koko.
Nā pilikia me ka defecation a me ka urination
- No ka paʻa ʻana o ka ʻōpū: Hiki iā ʻoe ke hana i nā mea e like me ka ʻai ʻana i nā meaʻai kiʻekiʻe i ka fiber (nā mea kanu, nā huaʻai, ka laiki bran) a hoʻohana i nā mea palupalu ʻōpala.
- No nā pilikia mimi: Inā pilikia ʻoe i ka mimi ʻana, pono paha ʻoe e hoʻohana i kahi catheter. Inā ʻaʻole hiki iā ʻoe ke kāohi i kāu mimi, aia nā lāʻau lapaʻau kūikawā (e like me Oxybutynin, Mirabegron).
Moekolohe
ʻOiai aia nā lāʻau lapaʻau e like me sildenafil a i ʻole tadalafil no kēia, hiki i kēia mau lāʻau lapaʻau ke hoʻonui i ka pilikia o ka hypotension orthostatic. No laila, pono ʻoe e kūkākūkā ākea i kēia me kāu kauka a hoʻohana wale i kēia mau mea ma lalo o kāna ʻōlelo aʻo.
He aha ka mea e hiki mai ana no kekahi me kēia maʻi i ka wā e hiki mai ana?
He mea paʻakikī ke hāʻawi i hoʻokahi pane e pili ana i ka wā e hiki mai ana o kekahi e ola nei me kēia maʻi, no ka mea, ʻokoʻa ia mai kēlā kanaka a kēia kanaka.
He maʻi holomua ka PAF. Akā no nā kānaka he nui, lohi loa ia, i nā makahiki, a i nā makahiki he ʻumi paha. Ua hōʻike kekahi mau noiʻi ʻo ka awelika o ke ola ma hope o ka ʻike ʻia ʻana o ka PAF ma kahi o 12.5 mau makahiki. Akā he awelika wale nō kēia, a hiki ke ʻokoʻa nui ma muli o kou mau hōʻailona a me ke olakino. No laila, mai hopohopo nui i kēlā mau helu.
Inā loaʻa iā ʻoe ka PAF, he mea nui e ʻike pinepine i kāu kauka e hōʻoia i ka hana ʻana o kāu lāʻau lapaʻau a pehea e holomua ai kou kūlana. Inā loaʻa iā ʻoe nā hōʻailona o ka maʻi Parkinson (e like me ka haʻalulu, ka paʻakikī i ka hele wāwae), pono ʻoe e haʻi koke i kāu kauka.
Leka Lawe-Home
- He maʻi laha ʻole ka Pure Autonomic Failure (PAF) e hoʻopilikia ana i ka ʻōnaehana nerve e kaohi ana i nā kaʻina hana aunoa i loko o ko mākou kino.
- ʻO ka hōʻailona nui, ʻo ia ka poniuniu a me ka māʻule ʻana i ke ala ʻana mai kahi noho ʻana a i ʻole kahi moe ʻana ma muli o ka emi ʻana o ke koko.
- ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia kēia maʻi, hiki ke kaohi maikaʻi ʻia nā hōʻailona a hiki ke mālama ʻia ke ʻano kiʻekiʻe o ke ola.
- ʻAʻohe ala e pale aku ai i kēia, a ʻaʻole na ʻoe ka hewa i hana ʻia ai.
- He mea nui loa ka mālama ʻana i ka launa pū ʻana me kāu kauka a hahai pololei i kāu hoʻolālā lapaʻau. Mai makaʻu e kamaʻilio ākea e pili ana i kāu mau nīnau a me kou mau hopohopo.
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