Ke manaʻo nei ʻoe i kekahi manawa ʻaʻohe ou ikaika, a nāwaliwali loa kou mau ʻiʻo? ʻOi loa aku inā e kūlou ana kekahi a i ʻole nā liʻiliʻi ʻelua o nā maka, loli kou mau helehelena, a paʻakikī paha ʻoe e ʻōlelo a moni paha? He mau hōʻailona paha kēia o kahi maʻi i kapa ʻia ʻo Myasthenia Gravis . ʻOiai he maʻi weliweli kēia, aia nā lāʻau lapaʻau maikaʻi loa no ia. I kēia lā ke kamaʻilio nei mākou e pili ana i kekahi o ia mau lāʻau lapaʻau hou a holomua.
He aha maoli ka Myasthenia Gravis?
I ka ʻōlelo maʻalahi, he kūlana kēia kahi e hoʻouka ai ka ʻōnaehana pale o kou kino ponoʻī i kou mau ʻiʻo ponoʻī. Kāhea mākou i kēia he kūlana autoimmune.
Loaʻa i kahi ʻiʻo kahi hōʻailona mai ko kākou lolo ma kahi e hui ai ke aʻalolo a me kahi ʻiʻo. Ua kapa ʻia kēia wahi pili ʻo ka neuromuscular junction. Maanei, hoʻouna kahi neurotransmitter i kapa ʻia ʻo acetylcholine i ka hōʻailona i ka ʻiʻo, me ka ʻōlelo ʻana, "ʻAe, e hana ʻoe i kēia manawa."
Eia nō naʻe, nui ka poʻe me ka myasthenia gravis e hoʻomohala i nā antibodies e hoʻouka i nā receptors e loaʻa ana i kēia hōʻailona acetylcholine. Ua kapa ʻia kēia mau antibodies he anti- acetylcholine receptor (AChR) antibodies . Ma muli o kēia mau antibodies, ʻaʻole loaʻa pono i nā ʻiʻo ka hōʻailona mai ka lolo. ʻO ka hopena, he nāwaliwali nā ʻiʻo.
| Nā hōʻailona maʻamau o ka myasthenia gravis | |
|---|---|
| Nā lihilihi maka e hāʻule ana | Ke kulou ʻana o nā lihilihi o kekahi a i ʻole nā maka ʻelua me ke kaohi ʻole ʻia. |
| Nā loli o ka helehelena | Nā loli i ke ʻano i ka wā e minoʻaka ai a i ʻole e ʻōlelo ana ma muli o ka nāwaliwali o nā ʻiʻo ma ka maka, ka waha, a i ʻole ka ʻauwae. |
| Ka nāwaliwali o nā lālā | Ka nalowale ʻana o ka ikaika ma nā lima, nā wāwae, a me nā manamana lima. Paʻakikī i ka hāpai ʻana i nā mea kaumaha a me ka hele wāwae. |
| Paʻakikī ke ale ʻana | Ke paʻakikī i ka ale ʻana i ka meaʻai a mea inu paha, pinepine ka ʻū ʻana. |
| Paʻakikī i ka ʻōlelo ʻana | Nā loli o ka leo a me ka hūpō o nā huaʻōlelo i ka wā e ʻōlelo ai. |
ʻO ka mea nui, ʻaʻole e ʻike nā mea maʻi a pau i kēia mau hōʻailona a pau. Hiki ke ʻokoʻa nā hōʻailona ma muli o ke koʻikoʻi o ka maʻi.
Pehea ka hana a Ultomiris (Ravulizumab)?
ʻAʻohe lāʻau lapaʻau no ka myasthenia gravis i kēia manawa. Akā he nui nā lāʻau lapaʻau kūpono e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona a alakaʻi i nā ola maʻamau. ʻO Ultomiris kekahi o ia mau lāʻau lapaʻau hou.
He antibody kūikawā ʻo Ultomiris e ālai ana i kahi protein i loko o kā mākou ʻōnaehana pale i kapa ʻia ʻo C5 . ʻO kēia protein C5 ka mea e kōkua ai i ka hōʻino ʻana i nā ʻiʻo. No laila, ke hoʻōki ʻo Ultomiris iā ia, e hoʻōki kāu ʻōnaehana pale i ka luku ʻana i nā hunaola ʻiʻo. ʻO ia hoʻi, hana kēia lāʻau lapaʻau ma ka hele pololei ʻana i ke kumu o ka maʻi.
Pehea e hāʻawi ʻia ai kēia lāʻau lapaʻau?
Hāʻawi ʻia kēia lāʻau lapaʻau iā ʻoe ma ke ʻano he intravenous infusion (IV infusion) e like me ka saline. Hāʻawi ʻia kēia iā ʻoe e ke kauka a i ʻole kekahi limahana olakino i aʻo ʻia ma kahi haukapila a i ʻole ke keʻena lapaʻau.
ʻO ka maʻamau, e hāʻawi ʻia ʻoe i kahi mahele mua, a ukali ʻia e kahi mahele ʻē aʻe i ʻelua pule ma hope. Ma hope o kēlā, pono ʻoe e hoʻomau i ka lawe ʻana i kēia lāʻau i kēlā me kēia ʻewalu pule . Eia nō naʻe, na kāu kauka e hoʻoholo i ka lōʻihi o kou lawe ʻana a me ka mahele (ma muli o ke kaumaha o kou kino) e pono ai ʻoe e lawe .
He mea nui loa ka lawe ʻana i kāu lāʻau lapaʻau i ka manawa kūpono. Inā e poina ʻoe i kahi mahele lāʻau, e kāhea mua i kāu kauka .
Pehea ʻoe e ʻike ai inā e hana ana ka lāʻau lapaʻau?
E hoʻomaka ʻoe e ʻike i ka ikaika mālie o kou mau ʻiʻo. E hiki iā ʻoe ke hana i kāu mau hana o kēlā me kēia lā me ka maʻalahi. Ma nā haʻawina lapaʻau, ua ʻike ʻia kekahi hoʻomaikaʻi ʻana o nā hōʻailona i loko o hoʻokahi pule wale nō.
Eia nō naʻe, inā ʻaʻole ʻoe e ʻike i kekahi ʻokoʻa, a inā paha e ʻoi aku ka ʻino o kāu mau hōʻailona, e kamaʻilio me kāu kauka . Hiki iā iaE hoʻololi i ka dosage, hāʻawi i kahi lāʻau lapaʻau hou aʻe, a hoʻololi paha i kahi lāʻau ʻē aʻe.
He aha nā hopena ʻaoʻao e hiki mai ana?
E like me nā lāʻau lapaʻau āpau, hiki iā ia ke hana i nā hopena ʻaoʻao liʻiliʻi. ʻO nā hopena ʻaoʻao maʻamau:
- Ka pehu ʻana (ka maʻi kahe)
- Nā maʻi ihu a ʻāʻī paha
- ʻeha ka ʻāʻī
- Nā hōʻailona anu maʻamau
No ka hoʻomalu ʻana i kou ʻōpū ʻeha, e ʻai i nā meaʻai māmā e like me ka laiki, ka maiʻa, a me ka berena. E inu i ka wai he nui. E hōʻemi i kāu lawe ʻana i nā mea inu caffeine e like me ke kofe. Inā ʻaʻole kōkua kēia mau hana, a inā paha e hoʻomau nā hōʻailona e like me ka ʻeha ʻāʻī a i ʻole ka ihu wai, e kamaʻilio me kāu kauka . Na ia e hoʻoholo i ka lāʻau lapaʻau maikaʻi loa nāu.
He aha nā mea kūikawā e pono ai ke ʻike? (Papahana REMS)
He mea nui loa kēia!
He lāʻau lapaʻau ʻo Ultomiris i hāʻawi ʻia ma lalo o kahi papahana hoʻokele pilikia kūikawā (Risk Evaluation and Mitigation Strategy - REMS). ʻO kēia no ka mea he liʻiliʻi loa akā koʻikoʻi ka pilikia o ka hoʻomohala ʻana i kahi maʻi bacteria i kapa ʻia ʻo meningococcal, ʻoi aku ka meningitis, ka mea e hoʻopilikia ai i ka lolo a me ke kaula spinal.
No kēia kumu, e hāʻawi aku kāu kauka iā ʻoe i kahi kāleka palekana maʻi kūikawā .
- Hōʻike maopopo ia i nā hōʻailona o kēia mau maʻi (e like me ke kuni koke, ka ʻeha poʻo, ka ʻāʻī ʻoʻoleʻa, ka nausea, ka luaʻi ʻana, a me ka ʻike i ka mālamalama).
- Ua ʻōlelo ʻia hoʻi inā loaʻa kēlā mau hōʻailona, pono e hele koke i ka Emergency Treatment Unit (ETU).
- He mea nui e mālama i kēia kāleka me ʻoe i nā manawa a pau - i ka wā o ka lāʻau lapaʻau a no 8 mau mahina ma hope o ka pau ʻana o ka lāʻau lapaʻau.
Eia kekahi, ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau, e nīnau kāu kauka inā ua hoʻopili ʻia ʻoe no ka maʻi meningococcal. Inā ʻaʻole, e aʻo aku lākou iā ʻoe e kiʻi i ka lāʻau lapaʻau ma ka liʻiliʻi he ʻelua pule ma mua o ka hoʻomaka ʻana i ka lāʻau lapaʻau.
Nā pilikia me nā lāʻau lapaʻau ʻē aʻe
He mea nui loa e haʻi i kāu kauka inā ʻoe e lawe ana i kekahi mau lāʻau ʻē aʻe, ʻoiai hoʻi:
- ʻO ka lāʻau lapaʻau e wehe i ka plasma mai ke koko (plasma exchange a i ʻole plasmapheresis)
- Lapaʻau immunoglobulin intravenous (IVIg)
- Nā lāʻau lapaʻau e ālai ana i ka neonatal Fc receptor (FcRn) no ka myasthenia gravis
Hiki i nā mea e like me kēia ke hōʻemi i ka pono o Ultomiris. No laila e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe ai, ʻoiai nā huaora.
Leka Lawe-Home
- He lāʻau lapaʻau hou a holomua ʻo Ultomiris (Ravulizumab) no nā poʻe maʻi me ka myasthenia gravis (me nā antibodies AChR).
- Hoʻōki kēia i ka ʻōnaehana pale o ke kino mai ka hoʻouka ʻana i kona mau ʻiʻo ponoʻī.
- He lāʻau lapaʻau kēia i hāʻawi ʻia ma ke ʻano intravenous i kēlā me kēia ʻewalu pule (ma hope o nā lāʻau mua).
- Ma muli o ka pilikia o ka maʻi meningococcal i ka wā e lawe ai i kēia lāʻau lapaʻau, he mea pono e loaʻa ka lāʻau pale a mālama i kāu kāleka palekana maʻi me ʻoe.
- E kamaʻilio ākea me kāu kauka e pili ana i nā hopena ʻaoʻao, nā hōʻailona e hoʻonui ana, a i ʻole ka hoʻohana ʻana i nā lāʻau lapaʻau ʻē aʻe. He mea nui ka kamaʻilio maikaʻi ma waena ou a me kāu kauka i ka holomua o kāu lāʻau.
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