Ua loaʻa i kāu keiki liʻiliʻi kahi puʻupuʻu liʻiliʻi ma kekahi wahi o ko lākou kino? A i ʻole he ʻulaʻula ʻili a i ʻole he ʻulaʻula maneʻo e ola ʻole? I kekahi manawa he mea maʻamau kēia, akā i kekahi manawa he mau hōʻailona ia o kahi maʻi laha ʻole i kapa ʻia ʻo Langerhans Cell Histiocytosis (LCH). He mea maʻamau ka makaʻu ke lohe ʻoe i kēia inoa, no ka mea, ʻaʻole ia he mea a mākou i maʻa mau ai i ka lohe ʻana. Akā, mai hopohopo, e kamaʻilio kākou e pili ana i ia mea ma nā ʻōlelo maʻalahi.
I ka ʻōlelo maʻalahi, he aha ka LCH?
E noʻonoʻo i ko kākou kino me he ʻāina nui lā. Aia kekahi pūʻali koa e pale aku i kēia ʻāina, a ʻo ia hoʻi ko kākou ʻōnaehana pale kino . ʻO kahi ʻano koa kūikawā i loko o kēia pūʻali koa i kapa ʻia ʻo nā pūnaewele Langerhans . ʻO kēia mau pūnaewele he ʻano o ke koko keʻokeʻo. ʻO kā lākou hana nui, ʻo ia ke kaua ʻana i nā germs a me nā maʻi e komo i loko o ko kākou kino a pale iā kākou mai nā maʻi. Loaʻa kēia mau koa ma ko kākou kino holoʻokoʻa, ʻoiai ma ka ʻili, nā māmā, nā lymph nodes, ka iwi ʻiʻo, ka spleen, a me ke akepaʻa.
Akā i ke ʻano o LCH, hoʻonui nui kēia mau hunaola Langerhans me ke kāohi ʻole ʻia a hōʻiliʻili ma nā ʻāpana like ʻole o ke kino. Ua like ia me kahi pūʻulu koa e kū wale ana ma kahi hoʻokahi me ka ʻole o ka hakakā. Ke hōʻiliʻili lākou e like me kēia, hoʻomaka lākou e hōʻino i nā ʻiʻo olakino ma ia mau wahi. Hoʻokumu ʻia nā ʻeha ma ia mau wahi.
ʻO ka nūhou maikaʻi e pili ana i kēia maʻi, hiki i ka hapa nui o nā keiki ke ho'ōla piha mai ka maʻi me ka mālama pono ʻana. I kekahi manawa, ʻoiai inā pili wale ia i ka ʻili, hiki ke nalowale iā ia iho me ka ʻole o ka mālama ʻana. Eia nō naʻe, inā pili kēia mau cell i nā ʻōpū koʻikoʻi e like me ka iwi iwi, ka spleen a i ʻole ke akepaʻa, pono paha ka mālama koʻikoʻi.
He maʻi ʻaʻai LCH anei?
He nīnau kēia a ka poʻe he nui. ʻO ka ʻoiaʻiʻo, aia nō kekahi manaʻo kūʻē iki ma waena o nā kauka e pili ana i kēia. Manaʻo ka poʻe noiʻi he nui ʻo LCH he ʻano like me ke kanesa, ʻo ia hoʻi, he neoplasm . ʻO ia hoʻi, he ulu ʻano ʻē o nā cell. Akā manaʻo kekahi he maʻi mumū ia o ka ʻōnaehana pale.
Eia nō naʻe, mālama ʻia ʻo LCH e nā kauka oncologists, nāna i loea i ka maʻi kanesa a me nā maʻi koko. I kekahi manawa, hoʻohana ʻia nā lāʻau lapaʻau anti-cancer e like me ka chemotherapy e mālama ai.
ʻO wai ka mea i loaʻa pinepine i kēia maʻi?
ʻIke pinepine ʻia ʻo LCH i nā pēpē hānau hou a me nā keiki ma waena o nā makahiki 1 a me 15. He mea kākaʻikahi loa nā pākeke, akā ʻaʻole hiki ʻole.
Ma ke ʻano helu, hoʻokahi a ʻelua paha o kēlā me kēia miliona mau keiki hānau hou i hānau ʻia me kēia maʻi i kēlā me kēia makahiki. ʻO ke ʻano kēia he maʻi kakaikahi loa ia.
He aha nā hōʻailona o LCH?
ʻAʻole like ka hopena o ka LCH i kēlā me kēia keiki. ʻOiai hoʻokahi wale nō wahi o ke kino o kekahi mau keiki e pili ana, hiki i kekahi ke pili i nā wahi he nui o ko lākou kino. No laila, ʻokoʻa nā hōʻailona ma muli o ka ʻāpana o ke kino i pili. E nānā kākou i ke ʻano o lākou.
| ʻĀpana kino i hoʻopilikia ʻia | Nā hōʻailona e ʻike ʻia |
|---|---|
| Nā iwi | Hoʻopilikia ka LCH i nā iwi ma kahi o 80% o nā maʻi. Hiki ke ʻike ʻia kahi puʻupuʻu a pehu paha ma nā wahi e like me ke poʻo, nā iwi a puni nā maka, ma hope o nā pepeiao, ka iwi ʻauwae, nā lālā, ka iwi kuamoʻo, a me nā pūhaka. Loaʻa paha ka ʻeha a ʻaʻole paha. Eia kekahi, ʻike ʻia paha ke poʻo ʻeha, ka ʻeha ʻāʻī/kua, ka paʻakikī i ka hele wāwae, a me ka ʻōʻō. |
| ʻIli | ʻO ka ʻōhū maʻamau ka ʻōhū ʻili. Hiki i ke poʻo cradle ke lilo i maʻi ho'ōla ʻole e ʻike ʻia ma ke poʻo o ke pēpē. Hiki ke ʻike ʻia nā ʻāpana ʻulaʻula a unahi ma ka ʻūhā, nā ʻāʻī, ka ʻōpū, ka umauma, a me ke kua. Hiki i kēia mau mea ke hoʻokahe a ʻānai a ʻeha paha. Hiki i nā kui ke loli ke kala a hāʻule paha. |
| Waha | Nā niho palahalaha a palahalaha paha, nā niho e emi ana, nā ʻāʻī pehu, nā ʻeha ma nā lehelehe, ke alelo, ma loko o nā papālina, a ma luna paha o ka ʻili o ka waha. |
| Ake a i ʻole ka spleen | Hiki i ke akepaʻa a i ʻole ka spleen i hoʻonui ʻia ke kumu o ka pehu ʻana o ka ʻōpū, ka melemele o nā maka a me ka ʻili (jaundice), ka maneʻo, ka luhi nui, a me ka maʻalahi o ka palapū a i ʻole ke kahe koko. |
| ʻIwi iwi | ʻAʻohe maʻi koko, keʻokeʻo, ka luhi, a me ka nalowale o ka makemake e ʻai ma muli o ka emi ʻana o nā hunaola koko ʻulaʻula. Nā maʻi pinepine a me ke kuni ma muli o ka emi ʻana o nā hunaola koko keʻokeʻo. ʻEha maʻalahi ma muli o ka emi ʻana o nā platelets. |
| ʻŌnaehana Endocrine (ʻŌnaehana Endocrine - Nā Hormones) | Inā pilikia ka ʻōpū pituitary: ka makewai nui a me ka mimi pinepine (diabetes insipidus), ka ulu ʻana o ka ulu ʻana, ka wā ʻōpiopio mua a lohi paha, ka piʻi ʻana o ke kaumaha. Inā pilikia ka ʻōpū thyroid: ka pehu ʻana o ka ʻōpū, ka paʻakikī o ka hanu ʻana. |
| Nā pepeiao | Nā maʻi pepeiao pinepine, ka kahe ʻana o ka wai mai ka pepeiao, ka ʻulaʻula o ka pepeiao, ka maneʻo o ka pepeiao, ka ʻeha o ka pepeiao, a me ka nalowale o ka lohe. |
| Nā maka | Nā maka pehu, pehu ma luna o nā maka, pilikia ka ʻike. |
| Nā Pūnaehana Lymph | ʻO ka pehu ʻana a me ka ʻeha ma nā puʻupuʻu (lumps) ma ka ʻāʻī, nā armpits, a i ʻole ka groin. |
| ʻŌnaehana Nervous Waena | ʻEha ke poʻo, poniuniu, luaʻi, paʻakikī i ka hele wāwae, nalowale ke kaulike (ataxia), paʻakikī i ka ʻōlelo ʻana a i ʻole ka ʻike ʻana, nā hopu ʻana, nā loli i ke ʻano a me ka hoʻomanaʻo. |
| Nā māmā | He mea maʻamau kēia i waena o nā pākeke, ʻoi aku hoʻi ka poʻe puhi paka. ʻEha ka umauma, ka ʻū maloʻo, ka paʻakikī o ka hanu ʻana, ka hiolo ʻana o ka māmā (pneumothorax). |
| ʻŌnaehana ʻōpū | ʻEha ka ʻōpū, ka luaʻi ʻana, ka maʻi pākīkī, ke koko i loko o ka ʻōpala, a me ka ulu maikaʻi ʻole ma muli o ka nele i nā meaʻai. |
ʻO ka mea nui, hiki ke ʻike ʻia kēia mau hōʻailona ma nā maʻi maʻamau ʻē aʻe he nui. No laila, mai makaʻu i ka LCH no ka mea wale nō he hoʻokahi a ʻelua paha o kēia mau hōʻailona kāu. Akā inā hoʻomau kēia mau hōʻailona, he mea nui loa ia e ʻike i ke kauka a loaʻa kahi hōʻailona kūpono.
He aha ke kumu kūikawā o LCH?
I ka ʻōlelo maʻalahi, loaʻa i kā mākou mau cell nā genes e haʻi iā lākou e "māhele i kēia manawa, e hoʻōki i kēia manawa." Ma kahi o ka hapalua o nā keiki me LCH he loli liʻiliʻi, a i ʻole mutation, i loko o kā lākou gene i kapa ʻia ʻo `BRAF .' Hana kēia gene i kahi protein e kāohi i ka ulu ʻana o nā cell. Ma muli o kēia mutation, ʻaʻole i loaʻa i kēlā protein ke kauoha e "hoʻōki i ka māhele ʻana." Me he mea lā ua paʻa ke kuapo 'on'. No laila, hoʻomau ka māhele ʻana a me ka hui ʻana o nā cell Langerhans.
ʻAʻole kēia he mea i hoʻoilina ʻia mai nā mākua a i nā keiki. Hana ʻia kēia hoʻololi ʻana o ka genetic ma hope o ka hāpai ʻia ʻana o ke keiki i loko o ka ʻōpū o ka makuahine.
Ma waho aʻe o ka gene `BRAF`, ua ʻike nā kānaka noiʻi hiki ke hana ʻia kēia maʻi e nā mutations i nā genes ʻē aʻe e like me `MAP2K1`, `RAS`, a me `ARAF`.
Pehea ʻoe e ʻike ai i kēia, e Kauka?
Ke lawe ʻoe i kāu keiki i ke kauka, e nīnau mua ʻo ia iā ʻoe e pili ana i nā hōʻailona o kāu keiki. A laila, e nānā pono lākou i kāu keiki. No ka mea hiki i ka LCH ke hoʻopilikia i nā ʻāpana like ʻole o ke kino, pono paha kekahi mau hoʻokolohua e hōʻoia i ka hōʻailona.
| ʻAno hoʻāʻo | I ka ʻōlelo maʻalahi... |
|---|---|
| Nā hoʻāʻo koko | ʻO ka helu koko piha (CBC) ke ana i ka helu o nā hunaola ʻulaʻula, nā hunaola keʻokeʻo, a me nā platelets i loko o ke koko. Hana ʻia nā hoʻāʻo koko e nānā i nā mea e like me ka hana o ke ake. |
| Biopsy | ʻO kēia ke ala paʻa loa e hōʻoia ai iā LCH.Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kahi puʻupuʻu a i ʻole ka lesion ma lalo o ka anesthesia a nānā ʻia ma lalo o kahi microscope e hōʻoia inā loaʻa nā pūnaewele LCH. Inā he kānalua no ka pili ʻana o ka iwi iwi, hiki ke hana ʻia kahi biopsy iwi iwi. |
| Hoʻāʻo Genetic | Hoʻohana ʻia ka hāpana ʻiʻo i lawe ʻia mai ka biopsy e hoʻāʻo ai no kahi mutation i loko o ka gene `BRAF`. |
| Nā Hoʻāʻo Kiʻi | Hiki i nā hoʻokolohua e like me nā X-ray, CT scans, MRI scans, a me PET scans ke hoʻoholo i ka nui o nā hopena o LCH ma nā ʻōpū o loko e like me nā iwi, ka lolo, a me nā māmā. |
Ma muli o nā hopena o kēia mau hoʻokolohua, e kuhikuhi ana kāu kauka i kāu keiki i kahi hematologist/oncologist pediatric.
He aha nā lāʻau lapaʻau no LCH?
ʻAʻole pono nā keiki a pau me LCH i ke ʻano like o ka lāʻau. Aia ka lāʻau i ka wahi a me ke koʻikoʻi o ka maʻi.
Hoʻokaʻawale nā kauka i ka LCH i ʻelua mau ʻano nui:
1. LCH ʻōnaehana hoʻokahi: Hoʻopilikia ka maʻi i hoʻokahi ʻōnaehana o loko o ke kino (e.g., nā iwi wale nō a i ʻole ka ʻili wale nō).
2. LCH ʻōnaehana lehulehu: Hoʻopilikia ka maʻi i ʻelua a ʻoi aku paha nā ʻōnaehana o ke kino (e.g., ka ʻili a me ke akepaʻa).
Eia kekahi, ʻo nā ʻōpū e like me ke akepaʻa, ka spleen, a me ka iwi iwi ua manaʻo ʻia he mau ʻōpū "pilikia kiʻekiʻe", ʻoiai ʻo nā ʻōpū e like me ka ʻili, nā iwi, a me nā māmā ua manaʻo ʻia he "haʻahaʻa ka pilikia." Hoʻoholo kēia hoʻokaʻawale ʻana i ka hoʻolālā lapaʻau.
Aia kekahi mau ʻano lapaʻau nui:
- Lapaʻau Steroid: ʻOiai inā ʻo ka ʻili wale nō ka mea i hoʻopilikia ʻia, hoʻohana ʻia nā lāʻau steroid e like me ka prednisone ma ke ʻano he mau pila a i ʻole nā mea ʻaila.
- ʻO ke ʻoki kino: Hana ʻia ke ʻoki kino e like me ka curettage e wehe i kahi puʻupuʻu LCH i loko o ka iwi.
- Chemotherapy: He ʻano lāʻau lapaʻau kēia i hāʻawi ʻia e pepehi i nā hunaola kanesa. Ma muli o ka māhele wikiwiki ʻana o nā hunaola LCH, ke kāohi nei kēia mau lāʻau i ko lākou ulu ʻana. Hiki ke hāʻawi ʻia kēia mau lāʻau lapaʻau ma ke ʻano he pila, inikini, a i ʻole he kirīmi i kau ʻia ma ka ʻili.
- Lapaʻau radiation: Hoʻohana i nā kukuna ikehu kiʻekiʻe e luku i nā pūnaewele LCH. Hoʻohana kākaʻikahi ʻia kēia i kēia manawa.
- Hoʻōla i kuhikuhi ʻia:No nā keiki me ka mutation gene `BRAF`, aia nā lāʻau lapaʻau (nā mea hoʻopale BRAF) e kuhikuhi ana i kēlā mutation. He liʻiliʻi loa ka hōʻino ʻana o kēia mau mea i nā cell olakino.
- Immunotherapy: Ke hoʻoulu ʻana i ka ʻōnaehana pale ponoʻī o ke keiki e hakakā i nā pūnaewele LCH.
- ʻO ke kāpili ʻana o nā pūnaewele kumu: He koho lapaʻau i manaʻo ʻia i nā hihia koʻikoʻi loa i hiki ʻole ke ho'ōla ʻia me nā lāʻau ʻē aʻe.
Pehea ke kūlana ma hope o ka mālama ʻana?
ʻO ka wānana no LCH e pili ana i kekahi mau kumu, me nā ʻāpana o ke kino i hoʻopilikia ʻia e ka maʻi, pehea ka mamao o kona laha ʻana, a pehea ka maikaʻi o kāna pane ʻana i ka lāʻau lapaʻau.
- ʻAneʻane 100% ka nui o ka ho'ōla ʻana no nā keiki me ka pilikia o nā ʻōkana "haʻahaʻa ka pilikia" (ʻōnaehana hoʻokahi a me nā ʻōnaehana he nui). ʻO ke ʻano kēia, ʻaʻohe pilikia o ka make. Eia nō naʻe, aia kahi pilikia o ka hoʻi hou ʻana a i ʻole nā pilikia lōʻihi ʻē aʻe.
- ʻO ke kūlana o nā keiki nona nā ʻōpū "pilikia kiʻekiʻe", e like me ke akepaʻa, ka spleen, a me ka iwi iwi, e ʻoi aku paha ka koʻikoʻi.
No laila, he mea nui loa ka hahai ʻana me kāu kauka no nā makahiki he nui, ʻoiai ma hope o ka pau ʻana o ka mālama ʻana. Pono ʻoe e nānā mau ʻia no ka hoʻi hou ʻana.
Leka Lawe-Home
- He maʻi laha ʻole ka Langerhans Cell Histiocytosis (LCH) i hoʻokumu ʻia e ka ulu ʻole ʻana o kahi ʻano cell immune. ʻIke pinepine ʻia ia i nā keiki.
- ʻOiai ʻaʻole ia i hoʻokaʻawale ʻia he maʻi kanesa, mālama nā kauka oncology iā ia me ka hoʻohana ʻana i nā lāʻau lapaʻau anti-cancer.
- ʻOkoʻa loa nā hōʻailona (nā ʻeha ʻili, nā puʻupuʻu iwi, nā maʻi pinepine) ma muli o ka ʻāpana o ke kino i hoʻopilikia ʻia e ka maʻi.
- He mea nui ka biopsy no ka hoʻomaopopo pono ʻana i ke ʻano o ka maʻi.
- No nā keiki he nui, ʻoi aku hoʻi ka poʻe i loko o ka māhele "haʻahaʻa-pilikia", hiki i ka lāʻau lapaʻau ke ho'ōla loa i ka maʻi.
- ʻOiai ma hope o ka pau ʻana o ka lāʻau lapaʻau, he mea nui loa ka loaʻa ʻana o ka nānā ʻana i ke kauka no kekahi mau makahiki e ʻike ai inā hoʻi hou mai ka maʻi.
- E kūkākūkā i nā nīnau a hopohopo paha āu e pili ana i ke kūlana o kāu keiki me kāu kauka.
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