Ke maʻi mau nei ʻoe a i ʻole kekahi āu i ʻike ai? Ke maʻi mau nei ʻoe, inā paha he anu, he maʻi palū, he maʻi pepeiao, a he maʻi māmā paha? I kekahi manawa aia kekahi kumu hohonu ma hope o kēia mau mea a mākou e noʻonoʻo ʻole ai. I kēia lā, ke kamaʻilio nei mākou e pili ana i kahi maʻi genetic kakaʻikahi, hiki ke koʻikoʻi i kapa ʻia ʻo WHIM syndrome.
I ka ʻōlelo maʻalahi, he aha kēia WHIM syndrome?
ʻO ka maʻi WHIM kahi maʻi genetic e hoʻopilikia ana i ka ʻōnaehana pale o ko mākou kino, kahi e hoʻonāwaliwali ai i ka hiki o ke kino ke hakakā i nā maʻi. He mea kākaʻikahi loa ia. Ua loaʻa ka inoa WHIM mai nā leka mua o nā hōʻailona nui ʻehā o ka maʻi. ʻAʻole loaʻa i nā kānaka a pau nā hōʻailona ʻehā, akā ʻo kēia nā mea maʻamau.
E nānā kākou i nā hōʻailona a kēia mau leka ʻehā e wehewehe nei.
| Leka | Manaʻo a me ka wehewehe maʻalahi |
|---|---|
| W - Nā ʻūlū | Nā ʻūlū: No ka mea, he haʻahaʻa ka ʻōnaehana pale o kēia mau maʻi, hiki i kahi maʻi i kapa ʻia ʻo Human Papillomavirus (HPV) ke komo maʻalahi i ke kino a hana i nā ʻūlū ma ka ʻili a me nā ʻāpana kino. Hoʻonui pū kēia maʻi HPV i ka pilikia o ka hoʻomohala ʻana i kekahi mau ʻano maʻi kanesa. |
| H - Hypogammaglobulinemia | Nā Pae Antibody Haʻahaʻa (Hypogammaglobulinemia): I ka ʻōlelo maʻalahi, ua like nā antibodies me nā koa e hakakā ana i nā germs e komo i loko o ko mākou kino. He haʻahaʻa nā pae o kēia mau antibodies i loko o ko lākou kino i kēia mau maʻi. ʻO kēia no ka mea he emi ko lākou mau keʻokeʻo koko i kapa ʻia ʻo nā cell B e hana ana i nā antibodies. No laila, ʻoi aku ka nui o ka loaʻa ʻana o nā maʻi. |
| ʻO I - Nā maʻi | Nā maʻi: Ma muli o ka nāwaliwali o ka pale ʻana, loaʻa pinepine kēia poʻe i nā maʻi . ʻOi aku ka maʻi o ka māmā, ka pepeiao, a me ka ʻili he mea maʻamau. |
| M - Myelokathexis | Myelokathexis: ʻO kēia ka pilikia nui a kikoʻī loa o kēia maʻi. ʻO nā hunaola koko keʻokeʻo e hakakā ana i nā germs i loko o ko mākou kino e hana ʻia ma ka iwi iwi. Ua like ia me nā koa e hoʻomaʻamaʻa ana ma kahi hoʻomoana koa. Akā i ka poʻe me kēia maʻi, ʻaʻole hiki i kēlā mau hunaola koko keʻokeʻo ke puka i waho o ka iwi iwi a hele i kahi o ka maʻi. Hoʻopaʻa ʻia lākou i loko o ka iwi iwi. No laila, i ka wā e loaʻa ai kahi maʻi, ʻaʻole hele mai nā hunaola e hakakā iā ia. |
ʻO ka mea nui, ʻaʻole nā maʻi āpau me ka maʻi WHIM e loaʻa i kēia mau hōʻailona ʻehā. Loaʻa paha i kekahi mau hōʻailona liʻiliʻi loa, aʻo kekahi poʻe e loaʻa paha i nā maʻi pinepine a koʻikoʻi e hiki ke hoʻoweliweli i ke ola.
Pehea ka hana a Xolremdi (mavorixafor)?
E like me kā mākou i kūkākūkā ai ma mua, ua paʻa nā hunaola koko keʻokeʻo o kēia mau maʻi i loko o ka iwi iwi, ʻeā? ʻO ke kumu o kēia he mutation i loko o ka gene i kapa ʻia ʻo `CXCR4` i loko o ko mākou kino. Hana kēia gene `CXCR4` i kahi protein kūikawā i kapa ʻia ʻo `CXCR4` receptor. E noʻonoʻo i kēia receptor ma ke ʻano he kiaʻi puka kahi e haʻalele ai nā hunaola koko keʻokeʻo mai ka iwi iwi.
I nā kānaka me ka maʻi WHIM, ma muli o kēia mutation i loko o ka gene `CXCR4`, ʻaʻole hana pono kēlā kiaʻi. Mālama ʻo ia i ka puka i pani ʻia. No laila, ʻaʻole hiki i nā hunaola koko keʻokeʻo ke puka i waho.
Hana kahi lāʻau lapaʻau hou i kapa ʻia ʻo Xolremdi (mavorixafor) ma ke ālai ʻana i ka mea loaʻa CXCR4 hana hewa. Ua like ia me ka hāʻawi ʻana i ka mea kiaʻi puka i kahi wā e hoʻomaha ai a wehe i ka puka. A laila, ua noa nā hunaola koko keʻokeʻo i hoʻopaʻa ʻia i loko o ka iwi iwi e komo i ke kahe koko a hele i kahi e pono ai lākou e hakakā i ka maʻi. ʻO kēia ka lāʻau lapaʻau mua i ʻāpono ʻia i ka makahiki 2024 e mālama i ke kumu kumu o ka maʻi WHIM.
Pehea e pono ai iaʻu ke hoʻohana i kēia lāʻau lapaʻau?
He capsule ʻo Xolremdi āu e lawe ai ma ka waha i hoʻokahi manawa i ka lā. Pono e lawe ʻia ma ka ʻōpū ʻole . ʻO ke ala maikaʻi loa e lawe ai, ʻo ia ka hoʻokē ʻai ʻana i ka pō ma hope o ka ʻaina ahiahi a lawe iā ia i ke kakahiaka nui. ʻAʻole ʻoe e ʻai a inu paha i kekahi mea ʻē aʻe no 30 mau minuke ma hope o ka lawe ʻana i ka lāʻau. ʻO ka nui āu e pono ai e pili ana i kou kaumaha, no laila na kāu kauka e hoʻoholo.
Pehea i hoʻāʻo ʻia ai ka holomua o kēia lāʻau lapaʻau?
Ua hana ʻia kahi noiʻi lapaʻau e hoʻāʻo ai i ka palekana a me ka pono o kēia lāʻau. Ua pili ia i 31 mau maʻi me ka maʻi WHIM i loaʻa kahi mutation gene CXCR4 i hōʻoia ʻia.
Ma kahi o ka hapalua o ka poʻe i komo i ke aʻo ʻana he poʻe ʻōpio ma waena o nā makahiki 12 a me 17. Ua māhele ʻia lākou i ʻelua mau pūʻulu, ua hāʻawi ʻia kekahi hui i ka lāʻau Xolremdi, a ua hāʻawi ʻia kekahi hui i kahi placebo i kēlā me kēia lā.
Ua nānā nui nā kānaka noiʻi i ka helu o kahi ʻano o ke koko keʻokeʻo i kapa ʻia ʻo "neutrophils" i loko o ke koko. Ua ana lākou i ka lōʻihi o ka noho ʻana o kēia helu ma kahi pae palekana (500 mau cell/microliter).
He mea hoʻoikaika nui nā hopena:
- ʻO ka poʻe i lawe iā Xolremdi, he pae neutrophil ko lākou ma kahi pae palekana no kahi o 12 mau hola i ka lā ma mua o ka poʻe i lawe ʻole.
- Eia kekahi, ua hāʻule ka nui o ka maʻi makahiki no ka poʻe i loaʻa ka lāʻau lapaʻau i lalo o ʻelua i ka makahiki , ʻoiai no ka poʻe i loaʻa ʻole ka lāʻau lapaʻau, ua piʻi aʻe ia ma mua o ʻehā i ka makahiki.
Ua hōʻike kēia mau hopena ua hōʻemi nui ʻo Xolremdi i ka pilikia o ka maʻi ma o ka hoʻokuʻu ʻana i nā hunaola koko keʻokeʻo. Eia nō naʻe, e hoʻomanaʻo ʻoiai me kēia mau hopena hoʻāʻo, hiki ke ʻokoʻa ka pane a kou kino.
ʻO wai ka mea pono ʻole e hoʻohana iā Xolremdi?
ʻAʻole kūpono kēia lāʻau lapaʻau no ka hoʻohana ʻana inā loaʻa iā ʻoe nā maʻi puʻupaʻa a akepaʻa koʻikoʻi, hāpai a hānai waiū paha. Eia kekahi, hiki i kēia lāʻau lapaʻau ke launa pū me kekahi mau lāʻau lapaʻau ʻē aʻe. No laila, he mea nui e kamaʻilio me kāu kauka e hoʻoholo ai inā kūpono ʻo Xolremdi iā ʻoe.
He aha nā pilina lāʻau me nā lāʻau lapaʻau ʻē aʻe?
He ʻāpana koʻikoʻi loa kēia. Hiki iā Xolremdi ke hoʻopilikia i ka wā e hui pū ʻia me nā lāʻau lapaʻau ʻē aʻe āu e lawe nei.
- ʻO ka lawe ʻana iā Xolremdi me kekahi mau lāʻau lapaʻau (e like me, nā mea hoʻopaʻa ʻo `P-gp` a i ʻole `CYP3A4`) e hoʻonui paha i nā hopena o Xolremdi a hoʻonui i ka pilikia o nā hopena ʻaoʻao.
- Hiki i kekahi mau lāʻau lapaʻau ʻē aʻe (e like me nā mea hoʻoulu ikaika 'CYP3A4') ke hōʻemi i ka pono o Xolremdi.
- Eia kekahi, hiki iā Xolremdi ke hoʻonui i nā hopena o kekahi mau lāʻau lapaʻau ʻē aʻe āu e lawe nei (ʻo ia hoʻi nā substrates ʻo 'CYP2D6', 'CYP3A4' a i ʻole 'P-gp').
- ʻO ka mea nui, pono ʻoe e pale i ka lawe ʻana iā Xolremdi me nā lāʻau lapaʻau ʻē aʻe e hiki ai ke hoʻololi i ka helu o ka puʻuwai ('QT prolongation').
ʻAʻole i piha kēia papa inoa. No laila, ma mua o kou hoʻomaka ʻana iā Xolremdi, e hōʻoia e haʻi i kāu kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei - me nā lāʻau lapaʻau i kauoha ʻia a me nā lāʻau lapaʻau kūʻai ʻole ʻia, nā huaora, nā lāʻau lapaʻau lāʻau, a me nā lāʻau Ayurvedic.
Pehea e mālama ai i nā hopena ʻaoʻao inā loaʻa lākou?
E like me nā lāʻau lapaʻau a pau, hiki iā Xolremdi ke loaʻa kekahi mau hopena ʻaoʻao. ʻO kēia nā mea maʻamau.
| Ka hopena ʻaoʻao | Ka mea hiki iā ʻoe ke hana |
|---|---|
| Ka helu haʻahaʻa o nā platelet koko ('Thrombocytopenia') | Hiki i kēia ke hoʻoulu i ka maʻalahi o ka palapū a i ʻole ke kahe koko. Inā hana ʻia kēia, e hoʻomaopopo koke i kāu kauka. |
| ʻŌhū ʻili | E ʻike paha ʻoe i nā hōʻailona o ka 'pityriasis', hiki ke hoʻomaloʻo a unahi i kou ʻili. E haʻi i kāu kauka e pili ana i kēia. |
| Kahe ke koko | Inā he kahe koko kou ihu, e noho pololei a hoʻohana i kou manamana nui a me kou manamana kuhikuhi e ʻūmiʻi i ka ʻāpana palupalu o kou mau puka ihu. Inā ʻaʻole e pau ke kahe koko ma hope o 20 mau minuke a inā he nui, e ʻimi koke i ke kōkua lapaʻau a i ʻole e hele i ka ʻOihana Pilikia kokoke loa (ETU). |
| Ka luaʻi ʻana | Inā ʻoe e luaʻi ana, e inu i ka nui o nā wai. Mai ʻai nui i ka manawa hoʻokahi, e ʻai i nā meaʻai liʻiliʻi a māmā. |
| Pōʻino | Hiki i ka poniuniu ke kumu o ka hāʻule ʻana. No laila, e pale i nā mea e like me ka hoʻohuli koke ʻana i kou poʻo a i ʻole ke ala koke ʻana mai kahi noho a moe paha. |
ʻAʻole kēia nā hopena ʻaoʻao āpau. Inā loaʻa iā ʻoe kekahi mau hōʻailona e hoʻopilikia nei iā ʻoe a i ʻole e hoʻoluhi iā ʻoe, e ʻoluʻolu e kamaʻilio me kāu kauka no ka ʻōlelo aʻo lapaʻau.
ʻO ka mea e pono ai ʻoe e ʻike i ka wā e lawe ai i kēia lāʻau lapaʻau
Ua manaʻo ʻia ʻo Xolremdi he "lāʻau lapaʻau kūikawā." ʻO ke ʻano kēia, ʻaʻole ia he mea hiki iā ʻoe ke kūʻai ma kahi hale kūʻai lāʻau maʻamau. He mau lāʻau pipiʻi kēia i hoʻohana ʻia e mālama i nā maʻi laha ʻole a paʻakikī.
Inā ua kuhikuhi ʻia kēia lāʻau lapaʻau iā ʻoe, e wehewehe kāu loea lapaʻau pehea e loaʻa ai a me kahi hale kūʻai lāʻau lapaʻau haukapila e loaʻa ai. Hiki ke paʻakikī iki kēia kaʻina hana, akā na kāu kauka a me nā limahana haukapila e hāʻawi iā ʻoe i ke alakaʻi e pono ai.
Leka Lawe-Home
- ʻO ka maʻi WHIM kahi maʻi genetic laha ʻole e hoʻopilikia ai i ka ʻōnaehana pale, e hoʻoulu ai i nā maʻi pinepine.
- He lāʻau hou ʻo Xolremdi (mavorixafor) e mālama ai i ke kumu o kēia maʻi, e kōkua ana i ka hoʻokuʻu ʻana i nā hunaola koko keʻokeʻo i hoʻopaʻa ʻia i loko o ka iwi iwi.
- He capsule kēia e lawe ʻia i kēlā me kēia lā ma ka ʻōpū ʻole. Mai ʻai no 30 mau minuke ma hope o ka lawe ʻana i ka pila.
- I mea e pale aku ai i nā hopena ʻino, he mea nui e hoʻomaopopo i kāu kauka e pili ana i nā lāʻau lapaʻau ʻē aʻe āu e lawe nei (me nā huaora a me nā mea kanu kuʻuna).
- Inā loaʻa iā ʻoe kahi hopena koʻikoʻi, e like me ke kahe koko nui, e kāhea koke aku i kāu kauka a i ʻole e hele i ka ʻOihana Hoʻopilikia kokoke loa (ETU).
- ʻOiai he lāʻau lapaʻau kūikawā kēia, na kāu kauka e alakaʻi iā ʻoe ma ke kaʻina hana o ka loaʻa ʻana.
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