He aha ka AL Amyloidosis? E kamaʻilio kākou e pili ana i kēia maʻi weliweli!

Ua lohe paha ʻoe i kēia maʻi i kapa ʻia ʻo 'AL Amyloidosis'? ʻAʻole paha, ʻeā? He mea laha iki kēia, ʻo ia hoʻi, ʻaʻole ia he maʻi e pili ana i nā kānaka a pau. Eia naʻe, inā loaʻa ia, hiki ke lilo i mea koʻikoʻi iki. I ka ʻōlelo maʻalahi, he loli ia i loko o kahi ʻano cell i kapa ʻia ʻo "Plasma cells" i loko o ko kākou kino, a ʻo kekahi o nā protein i hana ʻia e lākou e hele i waho o ka mana a waiho i loko o nā ʻano kino like ʻole o ko kākou kino, e hōʻino ana iā lākou. Ua like ia me kahi mīkini e hana maikaʻi ana a hoʻomaka koke e hana i nā ʻāpana hewa. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku, no ka mea, he mea nui loa ka ʻike ʻana i kēia.

He aha maoli ka 'AL Amyloidosis'?

ʻO kēia ʻano maʻi ʻo AL amyloidosis kekahi ʻano o kahi hui nui o nā maʻi i kapa ʻia ʻo `Amyloidosis`. He maʻi laha ʻole ʻo `Amyloidosis` e kū mai ana i ka wā e loli ai, a i ʻole `mutation`, i loko o nā `plasma cells` i loko o ko kākou iwi iwi. Hoʻopuka kēia mau plasma cell i hoʻololi ʻia i kahi ʻano protein ʻē. ʻO kēia mau protein, e like me nā pōpō kaula i hiolo ʻia, e hui pū, a waiho i loko o ko kākou mau ʻōpū a me nā ʻiʻo koʻikoʻi.

I loko o ka AL amyloidosis, ʻo ke ʻano o ka protein i hoʻopilikia ʻia e like me kēia, ʻo ia ka protein kaulahao māmā. ʻO kēia mau protein kaulahao māmā he mau ʻāpana o nā antibodies a ko mākou kino e hoʻohana ai e pale aku i nā maʻi. Hana ʻia kēia mau antibodies e nā cell plasma like aʻu i ʻōlelo ai ma mua.

ʻO ka maʻamau, hoʻopilikia nui ka AL amyloidosis i ko mākou puʻuwai a/a i ʻole nā ​​​​puʻupaʻa . Eia nō naʻe, i kekahi manawa hiki iā ia ke hoʻopilikia i ka ʻōpū, nā ʻōpū, nā aʻalolo, a me ka ʻili.

ʻO ka mea maikaʻi loa, inā ʻike mua ʻia kēia maʻi a mālama koke ʻia , hiki ke mālama ʻia i kekahi manawa ma ke ʻano he maʻi mau. Eia nō naʻe, inā waiho ʻole ʻia, hiki iā ia ke alakaʻi i nā kūlana koʻikoʻi e hoʻoweliweli i ke ola a me ka make. No laila, ʻaʻole kēia he mea e lawe māmā ʻia.

He aha ke ʻano o nā leka ʻelua ʻo `AL` ma kēia inoa?

Aia nā ʻano amyloidosis like ʻole. Ua kapa ʻia kēlā me kēia ʻano ma hope o ka protein ʻano ʻē e hoʻoulu ai i ka maʻi. Ma ka AL amyloidosis, ʻo ka leka A ke kū nei no ka amyloidosis a ʻo ka leka L ke kū nei no ka protein i kapa ʻia ʻo ke kaulahao māmā e hoʻoulu ai i ka maʻi. ʻO nā ʻano amyloidosis ʻē aʻe i ʻike nui ʻia ʻo ia ka AA amyloidosis (i hoʻokumu ʻia e ka serum amyloid A protein) a me ka ATTR amyloidosis (i hoʻokumu ʻia e ka transthyretin).

Pehea ka hopena o ka 'AL amyloidosis' i koʻu kino?

I ka ʻōlelo maʻalahi, ʻo AL amyloidosis kahi maʻi o nā cell plasma. ʻO nā cell plasma kekahi ʻāpana o kā mākou ʻōnaehana pale. ʻO kā lākou hana nui ka hana ʻana i nā antibodies e hakakā i nā maʻi. E noʻonoʻo i loko o ko mākou kino, aia nā tausani o nā clones o nā cell plasma e hana ana i nā ʻano antibodies like ʻole e hakakā i nā maʻi like ʻole. Māhele kekahi cell plasma a hana i nā cell he nui aku.

I nā maʻi e pili ana i nā 'kelepona plasma', māhelehele kahi hui o kēia mau 'kelepona plasma' me ke kāohi ʻole ʻia, e hana ana i nā nui he nui o ke ʻano like o nā 'antibodies'.

I loko o ka AL amyloidosis, i ka wā e hana ʻia ai nā antibodies, hana ʻia ʻelua mau kaulahao protein i kapa ʻia he mau kaulahao kaumaha a me nā kaulahao māmā. ʻO ka mea e hana ʻia ma ʻaneʻi, ʻo ia ka hana ʻana o nā cell plasma i nā protein kaulahao māmā he nui loa. Hoʻopili hewa kēia mau kaulahao māmā a hui pū. Ua kapa ʻia kēia mau protein i hui pū ʻia he mau fibrils amyloid. Hoʻokomo ʻia kēia mau fibrils i loko o kā mākou mau kino. ʻO ia ka manawa e hoʻomaka ai ka hōʻino nui i kēlā mau kino, i kekahi manawa a hiki i ke kūlana e hoʻoweliweli ai i ke ola.

ʻO wai ka mea i loaʻa pinepine i kēia maʻi?

He maʻi laha ʻole ka Amyloidosis. Ua manaʻo ʻia e pili ana ia ma waena o 9 a me 14 mau kānaka no ka miliona ma ʻAmelika Hui Pū ʻIa a ma waena o 5 a me 12 mau kānaka no ka miliona ma nā wahi ʻē aʻe o ka honua. ʻOi aku ka maʻamau o ka AL amyloidosis i nā kāne ma mua o nā wahine. Hoʻopilikia pinepine ia i nā poʻe ma luna o 60 mau makahiki. ʻO ka awelika o ka makahiki i ʻike ʻia ai he 64 mau makahiki.

He aha nā hōʻailona o ka 'AL amyloidosis'?

ʻAʻole hiki i ka `AL amyloidosis` ke hoʻopilikia wale i nā ʻāpana o ko kākou kino, mai ke poʻo a i nā wāwae, akā, i nā lālā pū kekahi. I ka manawa pinepine, hiki ke like nā hōʻailona o kēia maʻi me nā hōʻailona o nā maʻi ʻē aʻe, ʻaʻole koʻikoʻi. Eia kekahi, lohi loa ka ʻike ʻia ʻana o nā hōʻailona. No laila, ʻaʻole paha ʻoe e ʻike koke i kahi loli i kou kino.

Nā hōʻailona e pili ana i ke poʻo a me ka ʻāʻī:

• Ke manaʻo nei ʻoe i ka poniuniu a i ʻole ke poʻo māmā ('lightheadedness') ke ala ʻoe?
• He ʻulaʻula poni kou a puni kou mau maka a i ʻole ma kou mau lihilihi?
• Ke manaʻo nei anei ʻoe ua ʻoi aku ka nui o kou alelo ma mua o ka maʻamau ?

Nā hōʻailona e pili ana i nā lima a me nā wāwae:

Hiki i nā lima ke loaʻa i kēia mau ʻano:

• Ke ʻike nei ʻoe i ke kani ʻana, ka wela, a i ʻole ke kui ʻana i kou mau lima? He mau hōʻailona paha kēia o kahi maʻi i kapa ʻia ʻo "Peripheral Neuropathy".
• Ke ʻike nei ʻoe i ka ʻūlū a me ka mae ʻana ma kou mau manamana lima? He mau hōʻailona paha kēia o ka "Carpal Tunnel Syndrome".

Hiki i nā hōʻailona ma nā wāwae a me nā wāwae ke komo pū me:

• Ua pehu kou mau wāwae a i ʻole kou mau wāwae ?
• Ke manaʻo nei anei kou mau wāwae ua make ke ola ?

Eia kekahi, e ʻike paha ʻoe he maʻalahi kou palapū ʻana , kahe koko maʻalahi, a i ʻole he ʻulaʻula ke kala o ka ʻili ma kahi o nā wrinkles.

Nā hōʻailona e hōʻike ana i nā pilikia o ka puʻuwai a me ka māmā:

Hiki i nā hōʻailona ma lalo nei ke lilo i mau hōʻailona o nā maʻi ʻē aʻe, akā e makaʻala:

• Nā palpitations o ka puʻuwai: He manaʻo e like me ka puʻuwai e kuʻi ana a me ka holo wikiwiki.
• Pōkole o ka hanu (dyspnea): He manaʻo o ka paʻa i loko o ka umauma, me he mea lā ʻaʻole hiki iā ʻoe ke hanu hohonu.
• ʻEha umauma : ʻEha ma nā wahi āpau o ka umauma. Hiki i kēia ʻeha ke ʻoi, palupalu, a hele mai a hele aku. Hiki i ka ʻeha umauma ke hōʻailona o ka hōʻeha puʻuwai, no laila inā loaʻa iā ʻoe ka ʻeha umauma e mau ana ma mua o ʻelima mau minuke a ʻaʻole e nalowale me ka hoʻomaha a i ʻole ka lāʻau lapaʻau, e kelepona koke i ka 911 a i ʻole e lawe kekahi iā ʻoe i ka haukapila.
• Luhi : Manaʻo nui i ka luhi a hiki ʻole iā ʻoe ke hana i kāu mau hana o kēlā me kēia lā, me he mea lā ʻaʻohe ikaika i koe i loko o kou kino.

Nā hōʻailona e hōʻike ana i nā pilikia o ka ʻōpū a i ʻole ka ʻōpū:

Hiki i ka AL amyloidosis ke hoʻopilikia i kāu mau ʻano ʻai. Hiki i nā hōʻailona ke komo pū me:

• ʻAi ʻole ka makemake : Loli ka makemake o kēlā me kēia kanaka i kēlā me kēia manawa. Akā inā nalowale kou makemake no kekahi mau lā me ke kumu ʻole, he hōʻailona paha ia o kahi pilikia ʻōpū e pili ana i ka AL amyloidosis.
• Pehu a nui paha ke kinoea : He mau hōʻailona maʻamau ka pehu a me ke kinoea, akā i kekahi manawa he hilahila. Eia nō naʻe, inā loaʻa iā ʻoe ke kinoea mau, he hōʻailona paha ia o kahi pilikia ʻōpū e pili ana i ka AL amyloidosis.
• Paʻa ʻōpū : ʻO ka paʻa ʻōpū ka emi ʻana o ka neʻe ʻana o ka ʻōpū ma mua o ʻekolu i kēlā me kēia pule. Inā loaʻa iā ʻoe ka paʻa ʻōpū no nā pule ʻekolu a ʻoi, he pilikia ia e pono ai ʻoe e kamaʻilio me kāu kauka.
• Ka maʻi kahe : ʻŌpū wai. Inā hoʻomau ka maʻi kahe, pono ʻoe e haʻi i kāu kauka.

Nā hōʻailona e hōʻike ana i nā pilikia o ka puʻupaʻa a i ʻole ka ʻōpū mimi:

E ʻike paha ʻoe i kahi loli i ke ʻano o kou mimi a i ʻole ka pinepine o kou pono e mimi. Hiki i kēia mau hōʻailona ke komo pū me:

• Ke ʻike nei ʻoe i nā pehu hou aʻe i loko o kou mimi ma mua o ka maʻamau?
• Ke mimi iki nei ʻoe ma mua o ka maʻamau, a i ʻole pono ʻoe e ala i ka pō e mimi ?

He aha ke kumu o ka AL amyloidosis?

Ua kamaʻilio iki mākou e pili ana i kēia ma mua. ʻO ka 'AL amyloidosis' ke kumu o nā 'plasma cell' e hana ana i nā 'antibodies', nā protein i kapa ʻia ʻo 'nā kaulahao kaumaha' a me nā 'kaulaha māmā', i ka wā e hana nui ʻia ai kēlā mau protein 'kaulaha māmā'. Hoʻopili hewa kēia mau 'kaulaha māmā', hui pū, a hana i nā 'fibrils amyloid' a laila waiho ʻia i loko o ko kākou mau kino. ʻO ia ka manawa e hoʻomaka ai nā pilikia.

Pehea e ʻike ai nā kauka i ka AL amyloidosis?

Hoʻohana nā kauka i kekahi mau hoʻokolohua e ʻike ai i ka AL amyloidosis. ʻO ka hoʻāʻo koʻikoʻi loa, ʻo ia ka lawe ʻana i kahi laʻana liʻiliʻi o ka ʻiʻo a i ʻole ke kino i manaʻo ʻia ua hoʻopilikia ʻia e ka maʻi. Ua kapa ʻia kēia he biopsy. Hiki ke hoʻokaʻawale ʻia nā biopsies penei:

• ʻO ka biopsy iwi : Pili kēia i ka lawe ʻana i kahi hāpana liʻiliʻi o ka iwi i loko o ka iwi.
• ʻO ka biopsy puʻupaʻa : Hiki ke lawe ʻia kekahi mau ʻāpana liʻiliʻi o ka ʻiʻo puʻupaʻa.
• Biopsy puʻuwai : Hiki ke lawe ʻia kekahi mau ʻāpana liʻiliʻi o ka ʻiʻo puʻuwai.
• Biopsy pad momona : Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo momona mai ka ʻōpū. He hoʻāʻo maʻalahi kēia.

Nā hoʻokolohua hou aʻe

Hiki i kāu kauka ke hana i nā hoʻokolohua ʻē aʻe e ʻike ai i ka hana maikaʻi o kāu mau kino. Hiki i kēia mau mea ke komo pū me:

• Nā hoʻāʻo koko : Hana ʻia kēia e nānā i ke ʻano o ka hana ʻana o kou mau puʻupaʻa, puʻuwai, a me ke akepaʻa, a e nānā i ka nui o nā kaulahao māmā i loko o kou koko.
• Hoʻāʻo mimi : He hoʻāʻo hōʻiliʻili mimi kēia no 24 hola. Hōʻiliʻili ʻoe i kāu mimi ma ka home a lawe mai i ka hāpana i kāu kauka. Nānā kēia hoʻāʻo e ʻike inā ua pilikia kou mau puʻupaʻa i ka amyloidosis.
• Electrocardiogram (ECG): Ke ana ka ECG i ka hana uila o ka puʻuwai.
• Echocardiogram : I kekahi manawa i kapa ʻia ʻo ultrasound o ka puʻuwai, hoʻohana kēia i nā nalu kani alapine kiʻekiʻe e ana i ka neʻe ʻana o ka puʻuwai.
• Cardiac MRI (Cardiac Magnetic Resonance Imaging) : Hiki i kēia hoʻokolohua ke kiʻi i nā kiʻi maopopo loa a kikoʻī o ka puʻuwai.

Pehea e mālama ʻia ai ka AL amyloidosis?

Hoʻōla nā kauka i ka AL amyloidosis e hōʻemi i kāu mau hōʻailona, ​​​​kāohi i ka hōʻino ʻana o nā kino, a hoʻolohi a hoʻōki paha i ka hōʻiliʻili ʻana o nā protein amyloid maʻamau ʻole.

Hiki i nā kauka ke mālama i ka AL amyloidosis me ka chemotherapy, immunotherapy, a i ʻole steroids. Hāʻawi ʻia ka hapa nui o ka poʻe i hoʻokahi a ʻelua paha mau lāʻau chemotherapy, pinepine i ka hui pū ʻana me nā steroids. Hana pū kēia mau lāʻau e luku i nā cell plasma e hana ana i nā protein kaulahao māmā.

ʻO ka mea nui, ʻoiai hiki i kēia mau lāʻau ke hoʻōki a hoʻolohi paha i ka holomua o ka AL amyloidosis, ʻaʻole hiki iā lākou ke wehe i nā fibrils amyloid i kūkulu mua ʻia i loko o kou mau kino. Ke hoʻomaka ka lāʻau lapaʻau, hiki i kāu ʻōnaehana pale ponoʻī ke wehe mālie i kēia mau protein maʻamau ʻole. Ke ʻimi nei nā kānaka noiʻi i nā antibodies monoclonal hou e hiki ke wehe i kēia mau fibrils.

E kamaʻilio pū kāu kauka me ʻoe e pili ana i ka hiki iā ʻoe ke pōmaikaʻi mai kahi "hoʻoili iwi iwi" a i ʻole "hoʻoili cell kumu." ʻOiai he mau lāʻau paʻakikī kēia, hiki iā lākou ke holomua loa no kekahi poʻe.

Hiki ke pale ʻia ka ulu ʻana o ka `AL amyloidosis`?

ʻO ka mea pōʻino, ʻaʻole. ʻAʻohe ala e pale aku ai i ka AL amyloidosis a i ʻole kekahi ʻano amyloidosis ʻē aʻe. Hana ʻia ka AL amyloidosis i ka wā e lilo ai kahi cell plasma hoʻokahi i mea ʻē aʻe a hoʻomaka e māhelehele me ke kāohi ʻole. ʻAʻole kēia he mea hiki iā mākou ke kaohi.

Pehea ka lōʻihi e hiki ai iā ʻoe ke ola me ka `AL amyloidosis`?

Ke kōkua nei nā kauka i nā poʻe me AL amyloidosis e ola lōʻihi. No kekahi poʻe, he maʻi lōʻihi a mau paha ka AL amyloidosis, hiki ke mālama ʻia me ka lāʻau lapaʻau. Eia nō naʻe, he mea nui e hoʻomanaʻo he maʻi koʻikoʻi loa kēia a hiki ke alakaʻi i nā maʻi olakino e hoʻoweliweli ana i ke ola.

No ka mea, he maʻi laha ʻole ka AL amyloidosis, he mea paʻakikī i nā kauka ke ʻike pono i ka lōʻihi o ke ola o kekahi me ka maʻi. Inā loaʻa iā ʻoe ka AL amyloidosis, e nīnau i kāu kauka e pili ana i kāu prognosis ma hope o ka mālama ʻana. Hiki i kāu kauka ke hāʻawi iā ʻoe i ka ʻike maikaʻi loa e pili ana i ka mea e manaʻo ai.

Pehea wau e mālama ai iaʻu iho?

Inā loaʻa iā ʻoe ka AL amyloidosis, e kālele nui kāu kauka i ka mālama ʻana i kāu mau hōʻailona a me ka hoʻōki ʻana a i ʻole ka hoʻolohi ʻana i ka holomua o ka maʻi. Pono paha ʻoe e hoʻomau i ka lawe ʻana i nā lāʻau lapaʻau, a he mau hopena ʻaoʻao paha kēlā mau lāʻau lapaʻau. ʻO kahi ala e hiki ai iā ʻoe ke mālama iā ʻoe iho, ʻo ia ke aʻo e pili ana i nā hopena ʻaoʻao o kāu mau lāʻau lapaʻau a pehea e hoʻokele ai iā lākou. Eia kekahi mau manaʻo ʻē aʻe:

• Hiki i kekahi mau lāʻau lapaʻau ke hoʻopilikia i kou makemake.Inā pēlā, e nīnau i kāu kauka e pili ana i nā ʻano ʻai olakino e kōkua iā ʻoe e noho ikaika i ka wā o ka mālama ʻana. He mea nui loa ka ʻai ʻana i ka meaʻai kaulike.
• E hoʻāʻo e loaʻa ka nui o ka hoʻomaha . He mea nui ka hoʻomaha no ke kino i ka wā e hakakā ai i kahi maʻi e like me kēia.
• He ala maikaʻi loa ka hoʻoikaika kino e hoʻohauʻoli ai i kou noʻonoʻo a kōkua iā ʻoe e mālama i ke kaumaha olakino. Eia nō naʻe, e noi i kāu kauka no ka ʻōlelo aʻo no nā hoʻoikaika kino e ʻole e hoʻokaumaha nui i kou kino.
• No ka mea, he maʻi laha ʻole ka AL amyloidosis, hiki iā ʻoe ke manaʻo mehameha a mehameha . ʻAʻole paha e maopopo i kekahi poʻe ka maʻi. E nīnau i kāu kauka e pili ana i nā hui kākoʻo a i ʻole nā ​​​​papahana no ka poʻe me AL amyloidosis. ʻO ke kamaʻilio ʻana me nā poʻe e like me ia hiki ke lilo i kumu ikaika nui.

ʻO ka mea hope loa, kekahi mau mea nui (Leka Lawe-Home)

ʻO ka AL amyloidosis (Amyloid light chain a i ʻole primary amyloidosis) he maʻi laha ʻole ia i hoʻokumu ʻia e ka waiho ʻana o nā protein kaulahao māmā ʻē i loko o nā ʻōpū a me nā ʻiʻo o ko kākou kino. Hiki ke lilo i maʻi koʻikoʻi loa , i kekahi manawa e mālama ʻia ma ke ʻano he maʻi mau loa, akā hiki nō hoʻi iā ia ke hana i nā maʻi olakino e hoʻoweliweli ana i ke ola.

Akā, mai hopohopo, ke ʻimi nei nā kauka i nā ala hou e kōkua ai i ka poʻe me AL amyloidosis e ola lōʻihi a ʻoi aku ka maikaʻi. Inā loaʻa iā ʻoe ka AL amyloidosis, e nīnau i kāu kauka e pili ana i nā hoʻokolohua lapaʻau hou a me ka noiʻi e mālama ai i ka maʻi. He mea nui ka ʻike mua ʻana a me ka mālama ʻana. He mea nui hoʻi e noho maikaʻi a hahai i ka ʻōlelo aʻo a kāu kauka.

ʻAmyloidosis AL , amyloidosis mua, nā pūnaewele plasma, nā protein kaulahao māmā, nā fibrils amyloid, maʻi puʻupaʻa, maʻi puʻuwai