E kamaʻilio kākou e pili ana i ka lāʻau lapaʻau Amvuttra no ka maʻi puʻuwai i kapa ʻia ʻo ATTR-CM? (ATTR-CM & Amvuttra)

ʻAʻole paha ʻoe i lohe i nā maʻi e kū mai ana i ka wā e hōʻiliʻili hewa ai kahi ʻano protein i loko o ko mākou kino a waiho ʻia i loko o nā ʻōpū koʻikoʻi e like me ka puʻuwai a me nā aʻalolo. He mau maʻi laha ʻole kēia. I ka lāʻau lapaʻau, ua kapa ʻia kēia ʻano waiho ʻana o ka protein he amyloidosis . I kēia lā, e kamaʻilio mākou e pili ana i kahi maʻi laha ʻole i kapa ʻia ʻo ATTR-CM, kahi e hoʻopilikia pono ai i ka puʻuwai, ʻo ia hoʻi, kahi e waiho ʻia ai kēia mau protein i loko o ka ʻiʻo puʻuwai, a me ka lāʻau lapaʻau hou i kapa ʻia ʻo Amvuttra i hoʻohana ʻia no ia mea.

He aha maoli ka ATTR-CM?

I ka ʻōlelo maʻalahi, ʻo ATTR-CM kahi kūlana, ma muli o kahi kīnā genetic a i ʻole e kū maoli ana me ka ʻelemakule, ʻo ka protein TTR, i hana ʻia i loko o ko mākou kino, e hoʻololi i ke ʻano a hui pū a waiho i loko o nā paia o ka ʻiʻo puʻuwai. ʻO ka inoa piha no kēia ʻo Transthyretin Amyloid Cardiomyopathy.

E noʻonoʻo e pili ana i ia mea, pono nā paia o ko kākou puʻuwai e palupalu e pamu pono. Akā i ka wā e hōʻiliʻili ai kēia mau protein, lilo nā paia o ka puʻuwai i mānoanoa a paʻakikī . Kāhea mākou i kēia he cardiomyopathy. ʻO kēia ka mea e hiki ʻole ai i ka puʻuwai ke pamu pono i ke koko. I ka hala ʻana o ka manawa, hiki i kēia ke holomua i ka hāʻule ʻana o ka puʻuwai.

ʻAʻole ʻike kekahi poʻe me ATTR-CM i kekahi mau hōʻailona, ​​​​akā ʻike kekahi i nā hōʻailona e like me nā hōʻailona o ka hāʻule puʻuwai maʻamau.

• Ka pōkole iki o ka hanu (dyspnea)
• ʻO ka pehu ʻana, ʻoiai ma nā wāwae a me nā kuʻekuʻe wāwae
• Ka huikau a i ʻole ka manaʻolana ʻole
• Hoʻonui ʻia ka nui o ka puʻuwai a i ʻole ka puʻuwai naʻau ʻole
• ʻEha umauma

Hiki i kēia ke hoʻoluhi koke iā ʻoe i ka hana ʻana i nā mea e like me ke piʻi ʻana i nā alapiʻi, ka hana ʻana i nā hana o ka hale, a i ʻole ka hele wāwae ʻana i kahi mamao pōkole.

Eia kekahi, hiki ke ʻike ʻia nā hōʻailona ʻē aʻe i pili ʻole i ka puʻuwai. Eia kekahi laʻana, nāwaliwali, mae a ʻeha paha i nā lima a me nā wāwae, ka hou nui, ka poniuniu a i ʻole ka māʻule ʻana, a me nā pilikia o ka ʻōpū.

Pehea ka hana ʻana o ka lāʻau lapaʻau ʻo Amvuttra?

I mea e hoʻomaopopo ai i kēia, pono mua mākou e ʻike iki e pili ana i kahi protein i kapa ʻia ʻo TTR. ʻO TTR (Transthyretin) kahi protein i hana nui ʻia i loko o ko mākou ake. ʻO kāna hana nui ka lawe ʻana i nā hormones thyroid a me ka huaora A ma ke kino holoʻokoʻa.

I nā poʻe me ATTR-CM, ʻo kahi kīnā genetic ke kumu o ka hana ʻia ʻana o ka protein TTR i ke ʻano kūpono ʻole. Hōʻiliʻili kēia ʻano kūpono ʻole o ka protein a waiho i loko o ka puʻuwai.

Hana ka lāʻau ʻo Amvuttra ma ke ʻano he mea hoʻopau i nā gene. ʻO ia hoʻi, hele pololei kēia lāʻau i ke akepaʻa a ālai i kēlā protein TTR hewa.Hoʻemi ia i ka hana o ke gene e hana ʻia nei. Ua like ia me ka hoʻohaʻahaʻa ʻana i ka leo o ka lekiō.

He inikini ʻili ʻili ʻo Amvuttra. Hāʻawi pinepine ʻia kēia lāʻau pale e kekahi loea mālama ola i kēlā me kēia ʻekolu mahina .

Pehea i hoʻāʻo ʻia ai ka holomua o kēia lāʻau lapaʻau?

E like me nā lāʻau lapaʻau hou a pau, ua hōʻoia ʻia ka pono o AMVUTTRA ma o kahi noiʻi nui. Ma kēia noiʻi, ua hāʻawi ʻia he 655 mau maʻi ATTR-CM i ka lāʻau pale Amvuttra a ua hāʻawi ʻia ka hui ʻē aʻe i kahi lāʻau pale placebo me ka ʻole o ka lāʻau i kēlā me kēia 12 mahina no ʻekolu makahiki.

Ua nānā nā kānaka noiʻi i kekahi mau kumu e ana ai inā he pono kēia lāʻau.

• Hoʻāʻo hele wāwae ʻeono minuke: Ke ana kēia i ka mamao e hiki ai iā ʻoe ke hele ma ka wikiwiki maʻamau i loko o 6 mau minuke. E emi ana kēia mamao i ka wā e ʻoi aʻe ai ka maʻi o kou puʻuwai.
• Ka hoʻokaʻawale ʻana o ka New York Heart Association (NYHA): Ke ana nei kēia i ke koʻikoʻi o nā hōʻailona o ka hāʻule ʻana o ka puʻuwai. ʻEhā mau papa (I, II, III, IV). ʻO ke kiʻekiʻe o ka helu papa, ʻoi aku ka koʻikoʻi o ka maʻi.

• Nīnau Hoʻopaʻa Inoa no ke Ola Kino (KCCQ-OS): Ke ana kēia i ka hopena o ka maʻi i ke ola kino o ka mea maʻi.

He aha ka mea i loaʻa i ka noiʻi?

I ka pau ʻana o ka manawa mālama ʻekolu makahiki, ua kālailai nā mea noiʻi i nā hopena.

ʻO ka mea nui i ʻike ʻia, ʻo ka poʻe maʻi i loaʻa iā Amvuttra he 28% ka emi ʻana o ka make a i ʻole ke komo koke ʻana i ka haukapila a i ʻole ke kipa ʻana i ka lumi pilikia (ETU) ma muli o ka hāʻule ʻana o ka puʻuwai , ke hoʻohālikelike ʻia me ka poʻe i loaʻa i ka placebo (kahi lāʻau pale dummy).

ʻO kekahi mea nui ʻē aʻe, ma ka hopena o 42 mau mahina, ua emi ka pilikia o ka make mai kekahi kumu ma 35% i ka hui i loaʻa iā Amvuttra.

Eia kekahi, ʻo nā maʻi i loaʻa iā Amvuttra ua emi iho ka emi ʻana o ka hiki ke hele wāwae a me ke ʻano o ke ola.

Akā e hoʻomanaʻo, ʻaʻole like paha kēia mau hopena noiʻi no kēlā me kēia kanaka. Pono ʻoe a me kāu kauka e hoʻoholo pū inā kūpono ʻo Amvuttra iā ʻoe a inā paha e ʻoi aku ka maikaʻi o nā pono ma mua o nā hopena ʻaoʻao.

E aʻo kākou e pili ana i nā hopena a me nā hopena ʻaoʻao.

ʻEhia ka lōʻihi e ʻike ai i nā hopena?

He lāʻau lapaʻau ʻo Amvuttra e hana i ka hala ʻana o ka manawa. No laila, ʻaʻole ʻike koke ʻia nā hopena. Hiki ke hala kekahi mau mahina a ʻoi aku paha e ʻike i ka hoʻomaikaʻi ʻana o nā hōʻailona.

Pehea e mālama ai i nā hopena ʻaoʻao?

ʻO kekahi o nā hopena ʻaoʻao nui o Amvuttra ka emi ʻana o nā pae wikamina A i loko o ke koko. ʻO kēia no ka mea ʻo ka protein TTR ka mea e lawe i ka wikamina A ma ke kino holoʻokoʻa. Ke hōʻemi ka lāʻau i ka hana ʻana o TTR, e emi pū ka lawe ʻana o ka wikamina A.

• E kauoha mau ana kāu kauka i nā hoʻokolohua koko e nānā i kāu mau pae wikamina A. He mea nui loa e hana i kēia mau hoʻokolohua i ka manawa kūpono.
• E hoʻohui i nā meaʻai waiwai i ka wikamina A (e like me ke kāloti, ka ʻuala, ka sipinaki, nā hua manu, ka waiū, nā pepa bele ʻulaʻula ) i kāu papaʻai.
• Inā ʻike ʻoe i kekahi loli i kou ʻike (ʻoi aku ka emi ʻana o ka ʻike pō), e hoʻomaopopo koke i kāu kauka.
• Ma muli o kāu pae huaora A, hiki i kāu kauka ke ʻōlelo aku e lawe i kahi mea hoʻopihapiha huaora A.

ʻAʻole kēia wale nō nā hopena ʻaoʻao o Amvuttra. Inā loaʻa iā ʻoe kekahi mau hōʻailona ʻē aʻe e hoʻopilikia nei iā ʻoe, e kamaʻilio me kāu kauka .