He aha ka ADAMTS13 (Recombinant): Nā hoʻohana, ka nui o ka lāʻau, nā hopena ʻaoʻao a me nā mea hou aku

ʻO ADAMTS13 kahi lāʻau lapaʻau kūikawā i hoʻohana ʻia e mālama i kahi maʻi koko laha ʻole i kapa ʻia ʻo thrombotic thrombocytopenic purpura (TTP). Hana kēia lāʻau lapaʻau ma ka hoʻololi ʻana i kahi enzyme i nalowale a i ʻole i lawa ʻole i loko o kou koko e kōkua ana i ka pale ʻana i nā clots koko weliweli mai ka hana ʻana ma kou kino.

Inā ua kau ʻia kēia lāʻau lapaʻau iā ʻoe a i ʻole kekahi āu e ʻike nei, ke hana nei ʻoe me kahi maʻi koʻikoʻi akā hiki ke mālama ʻia. ʻO ka hoʻomaopopo ʻana i ka hana ʻana o kēia lāʻau lapaʻau hiki ke kōkua iā ʻoe e manaʻoʻiʻo i kāu hoʻolālā lapaʻau.

He aha ka ADAMTS13?

ʻO ADAMTS13 kahi mana i hana ʻia i ka hale hana o kahi enzyme e kū maoli ana i kou kino. ʻO ka hana a kēia enzyme ka wāwahi ʻana i nā protein nui i loko o kou koko i kapa ʻia ʻo von Willebrand factor multimers, hiki ke hana i nā clots weliweli ke lilo lākou i mea nui.

Ke hana ʻole kou kino i ka lawa o kēia enzyme, a i ʻole ke hana pono ʻole, hiki iā ʻoe ke hoʻomohala i ka TTP. Hoʻokumu kēia maʻi i nā clots koko liʻiliʻi e hana ma kou kino, hiki ke ālai i ke kahe koko i nā ʻōpū koʻikoʻi e like me kou lolo, ka puʻuwai, a me nā puʻupaʻa.

Hoʻokumu ʻia ka mana recombinant o ADAMTS13 i loko o kahi hale hana me ka hoʻohana ʻana i ka biotechnology kiʻekiʻe. Ua hoʻolālā ʻia e hana pololei e like me ka enzyme maoli e hana ai kou kino, e hāʻawi ana iā ʻoe i ka pale āu e pono ai e kūʻē i ka clotting koko maʻamau.

No ke aha i hoʻohana ʻia ai ka ADAMTS13?

Hoʻohana nui ʻia ka ADAMTS13 e mālama i ka hereditary thrombotic thrombocytopenic purpura (hTTP), kahi maʻi genetic laha ʻole e pili ana i ka liʻiliʻi ma mua o 1 i 100,000 poʻe. Hana ʻia kēia maʻi ke hānau ʻia ʻoe me ka nele o ka enzyme ADAMTS13 ma muli o nā loli genetic.

ʻO ka poʻe me ka hTTP e ʻike i nā hanana kahi e hana ai ko lākou koko i nā ʻōpala weliweli ma ko lākou kino. Hiki i kēia mau hanana ke hana i nā hōʻailona koʻikoʻi me ka huikau, nā hopu ʻana, nā pilikia o ka puʻupaʻa, a me nā helu platelet haʻahaʻa loa. Me ka ʻole o ka lāʻau lapaʻau, hiki i kēia mau hanana ke hoʻoweliweli i ke ola.

Hiki i kāu kauka ke noʻonoʻo i kēia lāʻau lapaʻau inā loaʻa iā ʻoe ka TTP i loaʻa ʻaʻole i pane maikaʻi i nā lāʻau lapaʻau ʻē aʻe. Eia naʻe, ʻo ke ʻano hoʻoilina ka hōʻailona mua no kēia ʻano hana pani enzyme.

Pehea e hana ai ʻo ADAMTS13?

Hana ʻo ADAMTS13 ma ka pani ʻana i ka enzyme e nalowale ana kou kino a i ʻole ʻaʻole e hana ana i ka nui. E noʻonoʻo e like me ka hāʻawi ʻana i kou koko i ka mea hana kūpono e mālama i nā protein clotting i kahi nui palekana.

I loko o kou kahe koko, hana maoli nā protein i kapa ʻia ʻo von Willebrand factor i nā kaulahao o nā nui like ʻole. Ke lilo kēia mau kaulahao i mea nui loa, hiki iā lākou ke hoʻoulu i ka clotting nui. Hana ka enzyme ADAMTS13 e like me nā ʻoki molekala, e ʻoki ana i kēia mau kaulahao protein nui i kahi nui palekana.

Ua manaʻo ʻia kēia lāʻau lapaʻau he lāʻau lapaʻau i manaʻo nui ʻia no ka mea e pili ana i ka hemahema enzyme kikoʻī e hana ai i ka hTTP. Ma ka hoʻihoʻi ʻana i nā pae enzyme maʻamau, kōkua ia i ka pale ʻana i ka hana ʻana o nā ʻōpala koko weliweli i ka wā e mālama ana i ka hiki o kou kino ke clot maʻamau i ka wā e pono ai no ka hoʻōla.

Pehea wau e lawe ai iā ADAMTS13?

Hāʻawi ʻia ʻo ADAMTS13 ma ke ʻano he intravenous (IV) infusion, ʻo ia hoʻi, hāʻawi pololei ʻia i loko o kou kahe koko ma o ka vein. ʻAʻole hiki ke lawe ʻia kēia lāʻau lapaʻau ma ka waha no ka mea e wāwahi ana kāu ʻōnaehana digestive i ka enzyme ma mua o ka hiki ke hana.

E hāʻawi maʻamau kāu hui mālama ola i kēia lāʻau lapaʻau i loko o kahi haukapila a i ʻole ke kikowaena infusion kūikawā. Lawe maʻamau ka infusion ma kahi o 30 mau minuke e hoʻopau ai, a e nānā ʻia ʻoe i ka wā a ma hope o ka lāʻau lapaʻau e hōʻoia ai e ʻae maikaʻi ana ʻoe.

Ma mua o kāu hoʻokomo ʻana, ʻaʻole pono ʻoe e hahai i nā palena meaʻai kūikawā. Hiki iā ʻoe ke ʻai maʻamau a lawe i kāu mau lāʻau ʻē aʻe e like me ke kauoha. Eia naʻe, e hōʻoia e haʻi i kāu mea mālama ola e pili ana i nā lāʻau a me nā mea hoʻohui āu e lawe nei, no ka mea, hiki i kekahi ke launa pū me ka lāʻau.

E hilinaʻi ka manawa o kāu mau hoʻokomo ʻana i kāu mau pono pilikino a me ke ʻano o ka pane ʻana o kou kino i ka lāʻau. Pono kekahi poʻe i nā hoʻokomo pale maʻamau, aʻo nā mea ʻē aʻe e loaʻa wale ka lāʻau i ka wā o nā hanana koʻikoʻi.

Pehea ka lōʻihi e lawe ai au iā ADAMTS13?

He ʻokoʻa nui ka lōʻihi o ka lāʻau ADAMTS13 mai kēlā me kēia kanaka, ma muli o ke ʻano o ka hereditary TTP a i ʻole ka loaʻa ʻana o TTP. No ka hereditary TTP, he lāʻau lapaʻau kēia no ke ola holoʻokoʻa no ka mea ʻaʻole e pau ke kūlana genetic.

E hana kāu kauka me ʻoe e hoʻomohala i kahi papa hana lapaʻau pilikino. Pono kekahi poʻe me hTTP i nā hoʻokomo prophylactic (pale) maʻamau e mālama i nā pae enzyme kūpono, aʻo nā mea ʻē aʻe e pono wale i ka lāʻau i ka wā o nā hanana koʻikoʻi a i ʻole nā manawa o ka piʻi ʻana o ka pilikia.

ʻO ka nānā ʻana maʻamau ma o nā hoʻāʻo koko e kōkua i kāu hui mālama ola e hoʻoholo i ka maikaʻi o ka hana ʻana o ka lāʻau a inā pono e hoʻoponopono i kāu papa hana. Ana kēia mau hoʻāʻo i kāu mau pae hana ADAMTS13 a nānā i nā hōʻailona o nā hanana TTP.

He aha nā hopena ʻaoʻao o ADAMTS13?

ʻO ka hapa nui o nā kānaka e ʻae maikaʻi iā ADAMTS13, akā e like me nā lāʻau lapaʻau a pau, hiki iā ia ke hana i nā hopena ʻaoʻao. ʻO nā hopena ʻaoʻao maʻamau he maʻalahi a hiki ke mālama ʻia me ka nānā pono a me ka mālama.

Eia nā hopena ʻaoʻao āu e ʻike ai, e hoʻomaka ana me nā mea maʻamau:

• ʻEha poʻo a me ka luhi māmā
• Nausea a i ʻole ka ʻoluʻolu o ka ʻōpū
• Nā hopena o ka pūnaewele hoʻokomo e like me ka ʻulaʻula, ka pehu, a i ʻole ka ʻeha māmā
• ʻO ka ʻūlū a i ʻole ka māmā
• ʻEha ʻiʻo a i ʻole ka ʻeha hui

ʻO kēia mau hopena ʻaoʻao maʻamau e hoʻomaikaʻi maʻamau ana iā lākou iho a ʻaʻole pono e hoʻōki i ka lapaʻau. Hiki i kāu hui mālama ola ke kōkua iā ʻoe e mālama i nā pilikia āu e ʻike ai.

ʻO nā hopena ʻaoʻao koʻikoʻi akā ʻaʻole maʻamau e komo pū ana me nā hopena maʻi, hiki ke loli mai nā ʻōpala ʻili maʻalahi a i ka anaphylaxis koʻikoʻi. ʻO nā hōʻailona o ka hopena maʻi e komo pū ana me ka pilikia o ka hanu ʻana, ka pehu ʻana o kou maka a i ʻole ka ʻāʻī, ka ʻōpala koʻikoʻi, a i ʻole ka puʻuwai wikiwiki.

I kekahi manawa, hiki i kekahi poʻe ke hoʻomohala i nā antibodies e kūʻē i ka lāʻau lapaʻau, hiki ke hoʻemi i kona pono i ka wā. E nānā kāu kauka i kēia ma o nā hoʻokolohua koko maʻamau a hiki ke hoʻoponopono i kāu hoʻolālā lapaʻau inā pono.

ʻO wai ka mea ʻaʻole pono e lawe iā ADAMTS13?

ʻAʻole kūpono ʻo ADAMTS13 no nā mea a pau, a e loiloi pono kāu kauka inā kūpono ia iā ʻoe. ʻAʻole pono ka poʻe i ʻike ʻia he maʻi koʻikoʻi i kekahi mea o ka lāʻau lapaʻau e loaʻa i kēia lapaʻau.

Inā he mōʻaukala kou o nā hopena maʻi koʻikoʻi i nā lāʻau lapaʻau i hoʻokumu ʻia i ka protein a i ʻole nā therapies pani enzyme, pono kāu kauka e loiloi pono i nā pilikia a me nā pōmaikaʻi. Hiki iā lākou ke ʻōlelo i nā hana palekana hou a i ʻole nā lapaʻau ʻē aʻe.

Pono nā wahine hāpai a me nā wahine e hānai ana i ka waiū i ka noʻonoʻo kūikawā, no ka mea, he palena ka ʻikepili palekana i loaʻa no kēia mau kūlana. E kūkākūkā kāu kauka i nā pilikia a me nā pōmaikaʻi me ʻoe inā hāpai ʻoe, e hoʻolālā ana e hāpai, a i ʻole e hānai ana i ka waiū.

Pono paha ka poʻe me kekahi mau maʻi ʻōnaehana immune a i ʻole ka poʻe e lawe ana i nā lāʻau immunosuppressive i nā hoʻolālā lapaʻau i hoʻololi ʻia, no ka mea, hiki i kēia mau kūlana ke hoʻopilikia i ke ʻano o kou kino e pane ai i ka lāʻau lapaʻau.

Nā inoa inoa ADAMTS13

Loaʻa ka lāʻau lapaʻau recombinant ADAMTS13 ma lalo o ka inoa inoa Adzynma. ʻO kēia wale nō ka huahana recombinant ADAMTS13 i ʻae ʻia e ka FDA i loaʻa ma ʻAmelika Hui Pū ʻIa.

Ua hoʻomohala kūikawā ʻia ʻo Adzynma no ka mālama ʻana i ka purpura thrombotic thrombocytopenic hoʻoilina a he holomua nui ia i ka mālama ʻana i ka TTP. Ma mua o ka loaʻa ʻana o kēia lāʻau, ua liʻiliʻi loa nā koho lapaʻau.

Hiki i kāu ʻinikua a me ka hale kūʻai lāʻau lapaʻau ke kuhikuhi i kēia lāʻau ma kona inoa inoa (Adzynma) a i ʻole kona inoa generic (recombinant ADAMTS13). E pili ana nā inoa ʻelua i ka lāʻau like.

Nā Koho ʻē aʻe o ADAMTS13

Ma mua o ka loaʻa ʻana o ADAMTS13, ʻo ka lapaʻau mua no ka TTP ʻo ka hoʻololi plasma (plasmapheresis), e pili ana i ka wehe ʻana i kou koko, ka hoʻokaʻawale ʻana i ka plasma, a me ka hoʻololi ʻana me ka plasma mea hāʻawi i loaʻa ka enzyme nalowale.

Hoʻohana ʻia ka hoʻololi plasma i nā hihia he nui, ʻoi aku hoʻi no ka TTP i loaʻa a i ʻole ke loaʻa ʻole ʻo ADAMTS13. Eia naʻe, he hana ʻoi aku ka invasive e koi ana i nā lako kūikawā a lawe pū i kāna mau pilikia, me ka hōʻike ʻana i nā huahana koko mea hāʻawi.

Hiki i nā infusions plasma paʻa hou ke hāʻawi i ka pani enzyme manawa, akā ʻaʻole i ʻoi aku ka nui ma mua o ka lāʻau recombinant a pono paha i nā nui nui o ka wai. Hoʻohana ʻia kēia ala i kekahi manawa i nā kūlana pilikia a i ʻole ke loaʻa ʻole nā lapaʻau ʻē aʻe.

No kekahi poʻe me ka TTP i loaʻa, hiki ke hoʻohana ʻia nā lāʻau immunosuppressive e like me corticosteroids a i ʻole rituximab e hōʻemi i ka hoʻouka ʻana o ka ʻōnaehana immune i ka enzyme ADAMTS13. Eia naʻe, ʻaʻole lākou e pani pololei i ka enzyme nalowale.

ʻOi aku ka maikaʻi o ADAMTS13 ma mua o ka hoʻololi Plasma?

Hāʻawi ʻo ADAMTS13 i nā mea maikaʻi he nui ma luna o ka hoʻololi plasma, ʻoi aku hoʻi no ka poʻe me ka TTP hoʻoilina. ʻOi aku ka maʻalahi o ka enzyme recombinant, palekana, a hāʻawi i nā hopena kūlike ma mua o nā lapaʻau plasma kuʻuna.

ʻAʻole like me ka hoʻololi plasma, e koi ana i nā hola he nui ma kahi keʻena lapaʻau kūikawā me nā lako paʻakikī, hiki ke hāʻawi ʻia ʻo ADAMTS13 ma ke ʻano he hoʻokomo wikiwiki. He mea kūpono loa kēia no ka mālama maʻamau a pale.

Mai kahi manaʻo palekana, hoʻopau ʻo ADAMTS13 i nā pilikia e pili ana i nā huahana koko mea hāʻawi, me nā maʻi hiki ke loaʻa a me nā hopena maʻi i nā protein plasma. Ua maʻamau hoʻi ka lāʻau lapaʻau recombinant, no laila e loaʻa iā ʻoe ka mahele like i kēlā me kēia manawa.

Eia naʻe, ʻo ka hoʻololi plasma paha ka lāʻau lapaʻau mua i makemake ʻia no kekahi poʻe me ka TTP i loaʻa, ʻoiai ke pili ka kumu i ka hana ʻino ʻana o ka ʻōnaehana pale. E hoʻoholo kāu kauka i ke ala maikaʻi loa e pili ana i kāu ʻano TTP a me nā kūlana pilikino.

Nā Nīnau i Nīnau Pinepine ʻia e pili ana iā ADAMTS13

He palekana anei ʻo ADAMTS13 no ka poʻe me ka maʻi puʻuwai?

Manaʻo ʻia ʻo ADAMTS13 he palekana no ka poʻe me ka maʻi puʻuwai, akā pono kāu cardiologist a me ka hematologist e hana pū e nānā i kāu mālama ʻana. No ka mea hiki i ka TTP ke hoʻopilikia i kou puʻuwai ma ka hana ʻana i nā clots liʻiliʻi i loko o nā kīʻaha koko cardiac, ʻo ka mālama ʻana i ke kūlana kumu e pōmaikaʻi pinepine i ke olakino o ka puʻuwai.

ʻAʻole maʻamau ka lāʻau lapaʻau i nā lāʻau lapaʻau puʻuwai, akā e nānā kāu mau kauka i kāu mau kauoha a pau e hōʻoia ʻaʻohe pilina. Inā loaʻa iā ʻoe ka maʻi puʻuwai koʻikoʻi, pono paha kāu hui lapaʻau e nānā iā ʻoe i ka wā o ka hoʻokomo ʻana e hōʻoia i hiki i kou kino ke mālama i ka wai hou.

He aha kaʻu e hana ai inā loaʻa iaʻu ka nui o ADAMTS13?

ʻAʻole paha e loaʻa ka overdose me ADAMTS13 no ka mea ua lawelawe ʻia e nā poʻe loea mālama ola i nā hoʻonohonoho i mālama ʻia. Eia naʻe, inā loaʻa iā ʻoe ka nui ma mua o ka mea i kauoha ʻia, e nānā pono kāu hui lapaʻau iā ʻoe no nā hōʻailona maʻamau.

ʻO nā hopena e hiki ke loaʻa mai ka loaʻa ʻana o ka lāʻau lapaʻau nui loa, ʻo ia ka piʻi ʻana o ka pilikia o nā hopena ʻaoʻao e like me ke poʻo, ka luaʻi ʻana, a i ʻole nā hopena maʻi. E hāʻawi kāu hui mālama ola i ke kākoʻo a e nānā i nā pae o kou koko e hōʻoia i kou palekana.

Inā hopohopo ʻoe e pili ana i kāu lāʻau a i ʻole ʻike i nā hōʻailona ʻokoʻa ma hope o ka mālama ʻana, mai kānalua e hoʻokaʻaʻike koke i kāu mea mālama ola. Hiki iā lākou ke loiloi i kou kūlana a hāʻawi i ke alakaʻi kūpono.

He aha kaʻu e hana ai inā ua hala wau i kahi lāʻau o ADAMTS13?

Inā ua hala ʻoe i kahi hoʻokomo ADAMTS13 i hoʻonohonoho ʻia, e hoʻokaʻaʻike i kāu mea mālama ola i ka wā hiki loa e hoʻonohonoho hou. Mai hoʻāʻo e hoʻoponopono i kahi lāʻau i hala ma ka pāpālua ʻana i kāu lāʻau aʻe, no ka mea, hiki i kēia ke hoʻonui i kou pilikia o nā hopena ʻaoʻao.

E hoʻoholo kāu kauka i ke ala maikaʻi loa e pili ana i ka lōʻihi o ka hala ʻana mai kāu lāʻau a me kou mau hōʻailona i kēia manawa. Hiki iā lākou ke makemake e nānā i nā pae o kou koko a i ʻole e hoʻoponopono i kāu papa hana lapaʻau e hōʻoia e mālama ana ʻoe i nā pae enzyme kūpono.

No ka poʻe ma ka lāʻau pale, ʻo ka hala ʻana i kahi lāʻau i kekahi manawa ʻaʻole paha e hoʻopilikia koke, akā he mea nui e mālama i kahi papa hana paʻa e like me ka hiki ke pale i nā hanana TTP.

I ka manawa hea e hiki ai iaʻu ke hooki i ka lawe ʻana iā ADAMTS13?

ʻO ka hoʻoholo e hooki i ka lāʻau ADAMTS13 e hilinaʻi loa i kou kūlana pilikino a pono e hana wale ʻia me ke alakaʻi a kāu kauka. No ka poʻe me ka hereditary TTP, he lāʻau lapaʻau kēia no ke ola holoʻokoʻa no ka mea ʻaʻole e loli ke kūlana genetic.

E loiloi mau kāu kauka i kāu pane ʻana i ka lāʻau lapaʻau ma o nā hoʻokolohua koko a me ka nānā ʻana i nā hanana TTP. Inā hoʻomohala ʻoe i nā antibodies e kūʻē i ka lāʻau lapaʻau a i ʻole ʻike i nā hopena ʻaoʻao koʻikoʻi, pono paha lākou e hoʻoponopono i kāu hoʻolālā lapaʻau a i ʻole e noʻonoʻo i nā koho ʻē aʻe.

Mai hooki i ka lawe ʻana iā ADAMTS13 ma kāu iho, ʻoiai inā maikaʻi kou manaʻo. Ke pale nei ka lāʻau lapaʻau i nā koko koko e hiki ke hoʻoweliweli i ke ola, a ʻo ka hooki ʻana koke ʻana e hiki ke hoʻokau iā ʻoe i ka pilikia no kahi hanana TTP.

Hiki iaʻu ke hele i ka wā e lawe ana iā ADAMTS13?

ʻAe, hiki iā ʻoe ke hele i ka wā e lawe ana iā ADAMTS13, akā pono ia i ka hoʻolālā pono a me ka hoʻopili ʻana me kāu hui mālama ola. Pono ʻoe e hoʻonohonoho i ka mālama ʻana ma kāu wahi e hele ai inā pili kāu huakaʻi me kahi infusion i hoʻonohonoho ʻia.

Ma mua o ka hele ʻana, e hōʻoia ua loaʻa iā ʻoe nā palapala lapaʻau pono a pau, me kāu papa hana lapaʻau, ka ʻike pili pilikia, a me kahi leka mai kāu kauka e wehewehe ana i kou kūlana a me nā pono lāʻau. Hiki ke kōkua kēia inā pono ʻoe i ka mālama olakino i ka wā e hele ana mai ka home.

E noʻonoʻo e lawe i ke kūpeʻe lima a i ʻole ke kāleka makaʻala lapaʻau e hōʻike ana i kou kūlana, no ka mea, he mea nui kēia ʻike i ka pilikia. Hiki i kāu hui mālama ola ke kōkua iā ʻoe e hoʻolālā i kāu huakaʻi a hoʻopili iā ʻoe me nā kikowaena lapaʻau ma kāu wahi e hele ai inā pono.