Ke ʻāwili wale nei kou mau lima a me kou mau wāwae? A i ʻole ua piha ʻoe ma hope o ka ʻai ʻana i kahi meaʻai liʻiliʻi, a i ʻole ua loaʻa iā ʻoe ka ʻeha ʻōpū ʻē? I kekahi manawa manaʻo mākou he mau mea maʻamau kēia, akā ma hope o kēia mau mea aia paha kahi maʻi ʻoi aku ka koʻikoʻi, akā kakaikahi ʻaʻole i kamaʻilio ʻia e kekahi. I kēia lā e kamaʻilio mākou e pili ana i ia ʻano maʻi, kahi i hoʻoili ʻia mai kēlā hanauna a i kēia hanauna , a e ʻoi mālie ana ka ʻino. ʻO ia ka maʻi i kapa ʻia ʻo hATTR Amyloidosis.
I ka ʻōlelo maʻalahi, he aha ka hATTR Amyloidosis?
ʻO ka inoa piha o kēia ʻo Hereditary Transthyretin Amyloidosis. E kapa mākou iā ia he hATTR no ka pōkole. He maʻi laha ʻole kēia i hoʻoilina ʻia, ʻo ia hoʻi, ua hoʻoili ʻia ma o nā genes.
E noʻonoʻo, hana ke akepaʻa o ko kākou kino i kahi protein kūikawā i kapa ʻia ʻo transthyretin (TTR) . I ke kanaka olakino, hana kēia protein i kāna hana a laila nalowale. Eia nō naʻe, i kekahi me ka maʻi hATTR, ma muli o kahi mutation i loko o kahi gene, hana ʻia kēia protein TTR ma ke ʻano kūpono ʻole. Ua like ia me kahi mea pāʻani i hana nani ʻia e hāʻule ana.
Hoʻohui kēia mau protein i pelu hewa ʻia a hana i nā puʻupuʻu liʻiliʻi. Kāhea mākou i kēia mau puʻupuʻu protein he amyloids . Hoʻomaka kēia mau amyloids e waiho ma nā ʻāpana like ʻole o ko mākou kino, ʻoi aku hoʻi i nā ʻōpū e like me nā aʻalolo, ka puʻuwai, a me nā puʻupaʻa. E like me ka lepo e paʻa ana i loko o kahi paipu wai, hoʻomaka kēia mau waihona protein e hōʻino i kēlā mau ʻōpū. Hiki i kēia ke hana i nā pilikia koʻikoʻi a hiki i ka hoʻoweliweli ʻana i ke ola. Akā ʻo ka nūhou maikaʻi, aia i kēia manawa nā lāʻau lapaʻau maikaʻi e kāohi i kēia mau hōʻailona.
Hiki paha i kāu kauka ke kapa aku iā ia he "Amyloidosis," no ka mea, ʻo ka hATTR kekahi ʻano o kahi hui o nā maʻi i kapa ʻia pēlā.
He aha nā hōʻailona kūpono o ka maʻi hATTR?
Ma muli o ka hoʻopilikia ʻana o kēia maʻi i nā ʻāpana he nui o ke kino, ua ʻokoʻa loa nā hōʻailona. I kekahi manawa ua like kēia mau hōʻailona me nā maʻi maʻamau ʻē aʻe, no laila hiki ke paʻakikī ke ʻike i ka maʻi.
Hoʻopilikia nui ia i ka ʻōnaehana nerve. Ke hōʻiliʻili kēia mau protein i loko o nā aʻalolo e holo ana mai ka lolo a i ke koena o ke kino, kapa mākou i kēia ʻano he Polyneuropathy . No laila e ʻike paha ʻoe i nā mea e like me kēia.
| ʻŌnaehana i hoʻopilikia ʻia | Nā hōʻailona hiki ke loaʻa |
|---|---|
| ʻŌnaehana Aʻalolo |
|
| Puʻuwai | |
| ʻŌnaehana Hoʻoheheʻe | |
| Nā hiʻohiʻona ʻē aʻe |
ʻO ka mea nui loa, ʻo ka hoʻonui ʻia ʻana o ka puʻuwai a me ka puʻuwai kū ʻole i hoʻokumu ʻia e kēia maʻi hiki ke hoʻoweliweli i ke ola. No laila, pono ʻoe e makaʻala loa i kēia mau hōʻailona.
Pehea e ulu ai kēia maʻi? ʻO wai ka mea i pilikia?
E like me ka inoa e hōʻike nei, he maʻi hoʻoilina kēia. Inā loaʻa iā ʻoe kēia maʻi, no ka mea ua hoʻoilina ʻoe i ka gene kīnā mai kou makuahine a makua paha (a i ʻole nā mea ʻelua).
Ua manaʻo ʻia kēia he maʻi laha ʻole loa ma ka honua. Eia nō naʻe, ma kekahi mau ʻāina, no ka laʻana, ʻo Pokukala, Iapana, a me Brazil, ʻike pinepine ʻia kēia maʻi. ʻOiai he kakaikahi ka ʻikepili kikoʻī e pili ana i kēia ma Sri Lanka, ua manaʻoʻiʻo ʻia he nui paha nā maʻi i kuhi hewa ʻia no ka mea ua like nā hōʻailona me nā maʻi ʻē aʻe.
Hiki ke ʻike ʻia nā hōʻailona i kēlā me kēia makahiki, ma waena o 30 a me 70 mau makahiki. Hoʻopilikia like ia i nā kāne a me nā wahine.
Pehea e ʻike ai ke kauka i kēia maʻi?
Hiki ke paʻakikī iki ka ʻike ʻana i kēia maʻi no ka mea he nui kona mau hōʻailona.
Ke nīnau nei e pili ana i ka mōʻaukala ʻohana a me nā hōʻailona
ʻO ka mea mua, e nīnau ke kauka iā ʻoe a me kou ʻohana e pili ana i kou mōʻaukala olakino.
- Aia kekahi o kou ʻohana i loaʻa i ka maʻi puʻuwai a i ʻole ka mānoanoa o ka puʻuwai?
- Ke manaʻo nei ʻoe i ka mae a i ʻole ke kuni ʻana i kou mau lālā?
- Ke ʻeha nei kou ʻōpū, ka maʻi pākīkī, a i ʻole ka paʻa ʻana o ka ʻōpū?
- Ke poniuniu nei ʻoe i kou kū ʻana?
- He pilikia kou ʻike maka?
Nā hoʻokolohua kūikawā
Ma muli o kāu mau hōʻailona, hiki i kāu kauka ke kuhikuhi i kekahi mau hoʻokolohua ʻē aʻe.
- Nā hoʻāʻo koko a me ka mimi: Kōkua kēia i ka nānā ʻana i nā pae protein kūpono ʻole i ke kino.
- Biopsy ʻIʻo: ʻO kēia ke ala nui e hōʻoia ai i ka maʻi. ʻO ka maʻamau, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka papa momona ma lalo o ka ʻili o kou ʻōpū ma lalo o ka anesthesia a hoʻouna ʻia i kahi keʻena hoʻokolohua. Hiki ke hoʻohana ʻia e ʻike pono inā ua waiho ʻia ka protein amyloid a mākou i kamaʻilio ai.
- Hoʻāʻo Genetic: Ke hōʻoia ka biopsy i ka loaʻa ʻana o ka amyloid, hana ʻia ka hoʻāʻo DNA e hoʻoholo ai inā he hATTR hoʻoilina a i ʻole kekahi ʻano amyloidosis ʻē aʻe. He mea nui loa kēia no ka hoʻolālā ʻana i ka mālama ʻana.
- Nā hoʻokolohua ʻē aʻe: Hiki ke hana ʻia nā hoʻokolohua ECG a me Echo e nānā i ka hana o ka puʻuwai, a me nā haʻawina hoʻoili aʻalolo e nānā i ka hana aʻalolo.
No ka mea he maʻi laha ʻole kēia, ʻaʻole paha i loaʻa i nā kauka āpau ka ʻike nui me ia. No laila, he mea nui loa e ʻimi i ka manaʻo ʻelua mai kekahi loea ʻē aʻe ma hope o ka hōʻoia ʻia ʻana o ka maʻi.
He aha nā lāʻau lapaʻau no kēia maʻi?
ʻO ka mālama ʻana no ka hATTR e pili ana i kāu mau hōʻailona a me ke kahua o ka maʻi. ʻElua mau pahuhopu nui o ka mālama ʻana. ʻO kekahi, ʻo ia ke kāohi i nā hōʻailona. ʻO kekahi, ʻo ia ke kāohi i ka hana ʻana o ka protein TTR ʻano ʻē, ʻo ia ke kumu nui o ka maʻi.
1. Lapaʻau no nā hōʻailona
- Inā hōʻiliʻili ka wai i loko o ke kino ma muli o nā pilikia o ka puʻuwai a i ʻole nā puʻupaʻa, hoʻohana ʻia nā diuretics e wehe i kēlā wai keu.
- Aia kekahi mau lāʻau lapaʻau no nā pilikia o ka ʻōnaehana hoʻoheheʻe e like me ka maʻi pākī a me ka ʻeha ʻōpū.
- Aia kekahi mau lāʻau lapaʻau kūikawā no ka kaohi ʻana i ka ʻeha o ke aʻalolo.
2. Nā lāʻau lapaʻau i kuhikuhi ʻia i ke kumu o ka maʻi
ʻO kēia nā lāʻau lapaʻau nui e pale ai i ka maʻi mai ka lilo ʻana i mea ʻino.
| ʻAno lapaʻau | Wehewehena |
|---|---|
| Nā lāʻau hoʻopau gene | |
| ʻO nā lāʻau lapaʻau e like me Inotersen, Patisiran, Vutrisiran | Hana kēia mau lāʻau lapaʻau ma ka haʻi ʻana i ka gene e hana ana i ka protein TTR i loko o ke akepaʻa e "pio." Hoʻemi kēia i ka hana ʻana o ka protein ʻē. Hāʻawi ʻia lākou ma ke ʻano he inikini. |
| Nā Mea Hoʻopaʻa Pūmua (Nā Lāʻau Hoʻopaʻa Gene) | |
| Nā lāʻau lapaʻau e like me Tafamidis, Diflunisal | Hana kēia mau lāʻau lapaʻau ma ka hoʻopili ʻana i ka protein TTR i hana ʻia a pale iā ia mai ka hui pū ʻana me ka hewa. Hiki ke lawe ʻia kēia mau mea ma ke ʻano he mau pila. |
| Hoʻoili ʻAke | |
| ʻoki kino | Ma muli o ka hana ʻia ʻana o ka protein hewa i loko o ke akepaʻa, ʻo kekahi lāʻau lapaʻau, ʻo ia ke pani i ke akepaʻa me kahi mea hou. Akā, he ʻoki kino nui loa kēia a he mea weliweli hoʻi. Manaʻo pinepine ʻia no nā poʻe maʻi ʻōpio i nā wā mua o ka maʻi. |
Na kāu kauka e hoʻoholo i ka lāʻau lapaʻau kūpono loa iā ʻoe. E kamaʻilio ākea me ia e pili ana i kēia mau koho āpau.
ʻO ka hui lapaʻau e kōkua iā ʻoe
Ma muli o ka hoʻopilikia ʻana o kēia maʻi i nā ʻōkana he nui i loko o ke kino, pono ʻoe i ke kākoʻo o kahi hui o nā kauka me nā ʻano hana kūikawā like ʻole.
- Neurologist: No nā pilikia e pili ana i ke aʻalolo.
- Kauka Puʻuwai: E nānā a mālama i ka hana o ka puʻuwai.
- Kauka Nephrologist: Inā ua pilikia nā puʻupaʻa.
- Gastroenterologist: No nā pilikia ʻōpū.
- Kauka maka: No nā pilikia maka.
- Kākā'ōlelo Hoʻoilina: E hāʻawi i ka ʻike e pili ana i ka maʻi a me kona hopena i ka hoʻoilina.
- Kauka Hoʻōla Kino: E kōkua i nā pilikia hele wāwae a mālama i ke kino i ikaika.
Leka Lawe-Home
- He maʻi hoʻoilina kakaikahi ka hATTR Amyloidosis.
- Inā loaʻa iā ʻoe nā mea e like me ka mae ʻole o kou mau lālā, ka ʻeha mau o ka ʻōpū, nā hōʻailona puʻuwai i wehewehe ʻole ʻia, a inā he moʻolelo ko kekahi o kou ʻohana no kēia mau kūlana, mai lawe māmā.
- Hiki i ka ʻike mua ʻana a me ka mālama ʻana ke kōkua i ka pale ʻana i ka maʻi mai ka lilo ʻana i mea ʻino.
- Ma muli o ka paʻakikī o kēia maʻi, he mea nui loa ia e ʻimi i ke kōkua o nā loea like ʻole.
- Inā loaʻa iā ʻoe a i ʻole kekahi āu i ʻike ai kēia mau hōʻailona, e ʻimi koke i ka ʻōlelo aʻo mai ke kauka kūpono . Mai makaʻu e kamaʻilio e pili ana.
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