Ua ʻike anei ʻoe i kahi kiko ʻulaʻula-ʻeleʻele ma ke kino o kāu keiki a i ʻole kou kino ponoʻī? Malia paha ua hamo mālie ʻoe i ka wahi me kou manamana lima, a ua lilo koke ka wahi i ʻulaʻula, hoʻomaka e maneʻo, a ʻike ʻia kahi pehu liʻiliʻi ma luna. Inā kamaʻāina kēia ʻike iā ʻoe, he mea nui loa ia e ʻike i ke kūlana a mākou e kamaʻilio nei i kēia lā.
I ka ʻōlelo maʻalahi, he aha ka Urticaria Pigmentosa?
ʻO ka Urticaria Pigmentosa kahi ʻano hives e kū mai ana ma ka ʻili. ʻIke pinepine ʻia ia i nā keiki ʻōpio a me nā pākeke ʻōpio. He ʻano ia o kahi maʻi i kapa ʻia ʻo 'mastocytosis'.
I kēia manawa, ke noʻonoʻo nei paha ʻoe i ke ʻano o ka `mastocytosis`. He mea maʻalahi loa ia. ʻO nā ``mast cells`` he ʻano cell kūikawā i loko o ka ʻōnaehana pale o ko kākou kino. Ua like lākou me nā kiaʻi palekana o ke kino. Ke hōʻiliʻili nui ʻia kēia mau mast cell ma kekahi wahi o ka ʻili, kapa mākou iā ia ʻo Urticaria Pigmentosa. Eia naʻe, hiki i kēia mau mast cell ke hōʻiliʻili ʻaʻole wale i loko o ka ʻili, akā i kekahi manawa i nā ʻōpū ʻē aʻe e like me nā iwi a me nā ʻōpū. A laila lilo ia i kūlana i kapa ʻia ʻo ``systemic mastocytosis``. Akā, mai hopohopo, ʻo ka hapa nui o ka manawa, ʻo Urticaria Pigmentosa i nā keiki ua kaupalena ʻia i ka ʻili.
I loko o kēia maʻi, ke ʻike ʻia kahi kiko ʻeleʻele ma ka ʻili, lilo ia i ʻulaʻula, pehu, ʻāʻī, a piʻi aʻe me he mea lā ua paʻi ʻia e ke kaʻa. Ma ka ʻōlelo lapaʻau, ua kapa ʻia kēia hōʻailona ʻo "Darier's sign."
No ke aha lā e hana ʻia ai kekahi mea e like me kēia?
ʻO ke kumu nui o kēia, ʻo ia nā "mast cells" a mākou i kamaʻilio ai ma mua. He ʻano koko keʻokeʻo kēia i hana ʻia i loko o ko kākou iwi iwi. Loaʻa kēia mau cell ma ko kākou kino a pau, ʻoiai ma kahi e launa pū ai kākou me ke ao waho, e like me ka ʻili, nā māmā, a me nā ʻōpū. Ke komo kekahi mea e like me ka germ i loko o ke kino, hoʻokuʻu kēia mau mast cell i kahi kemika i kapa ʻia ʻo "histamine" e kōkua i ka pale ʻana i ke kino.
Hoʻokumu ʻia ka Urticaria Pigmentosa e kahi loli genetic e hoʻoulu ai i kēia mau cell mast e hōʻiliʻili nui i loko o ka ʻili. ʻO ka maʻamau, hana ʻia kēia loli genetic i ka wā e noho ana ke keiki i loko o ka ʻōpū. Eia naʻe, ʻaʻole ia he mea i hoʻoilina ʻia mai nā mākua a i nā keiki . ʻAʻole i loaʻa ke kumu pololei o kēia loli.
Inā loaʻa kēia maʻi i nā pākeke, hiki ke ʻoi aku ka koʻikoʻi. Ua kapa ʻia ʻo 'indolent systemic mastocytosis'. I kēia hihia, hōʻiliʻili nā cell mast ma nā wahi e like me ka iwi iwi ma waho aʻe o ka ʻili. He mea kākaʻikahi loa, hiki i kēia maʻi ke ulu i loko o kahi maʻi kanesa i kapa ʻia ʻo 'mast cell leukemia'. Akā, he mau mea kākaʻikahi loa kēia . ʻO 99% o ka Urticaria Pigmentosa e kū mai ana i nā keiki ʻaʻole ia he koʻikoʻi.
He aha nā hōʻailona o kēia maʻi?
ʻO ka hōʻailona nui a maopopo loa, ʻo ia ka `hōʻailona o Darier` a mākou i kamaʻilio ai. ʻO ia hoʻi, lilo nā kiko ʻeleʻele i ʻulaʻula a me ka maneʻo ke hamo ʻia. Eia kekahi, hiki i kekahi poʻe ke ʻike i nā hōʻailona ma lalo nei.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| Nā palpitations o ka puʻuwai | Piʻi aʻe ka wikiwiki o ka puʻuwai me he mea lā e makaʻu ana ʻoe. |
| Ka maʻi ʻōpū (ʻeha ʻōpū) | Hiki ke hana ʻia e ka hoʻoulu ʻana o nā cell mast i loko o ka ʻōnaehana hoʻoheheʻe. |
| Ka holoi ʻana | ʻUlaʻula koke o ka maka a me ke kino o luna. |
| Paʻakikī ka hanu ʻana (Wheezing) | He leo 'cheese-cheese' i ka wā e hanu ana, e like me ke kani a ka poʻe hānō. |
| Ka mae ʻana | Ka nalowale ʻana o ka ʻike ma muli o ka emi koke ʻana o ke koko. |
| ʻEha poʻo | ʻEha poʻo. |
ʻOi aku ma mua o 75% o nā maʻi Urticaria Pigmentosa he mau keiki ma lalo o 10 mau makahiki. I ka hapa nui o nā keiki, ua kaupalena ʻia kēia ʻano i ka ʻili a hoʻomaikaʻi aʻe me ka ʻelemakule.
Pehea e ʻike ai ke kauka i kēia?
I ka hapanui o nā hihia, hiki i kāu kauka ke ʻike maʻalahi i kēia kūlana ma ka nānā ʻana i nā kiko ma kou ʻili a me ka nānā ʻana i ka hōʻailona o Darier. ʻAʻohe pono o nā hoʻokolohua ʻē aʻe.
Eia nō naʻe, inā he kānalua ʻaʻole i kaupalena ʻia kēia maʻi i ka ʻili akā ua hoʻopilikia pū i nā ʻāpana ʻē aʻe o ke kino, hiki i ke kauka ke ʻōlelo i nā hoʻokolohua aʻe.
- Hoʻāʻo koko ('Helu koko'): E aʻo e pili ana i nā ʻano like ʻole a me ka nui o nā cell i loko o ke koko.
- Hoʻāʻo tryptase serum: Nānā i ke alo o kahi enzyme i loko o ke koko i hoʻokuʻu ʻia e nā cell mast.
- Ke nānā ʻana i ka nui o ka iwi: E ʻike i ka ikaika o kou mau iwi.
- He hoʻāʻo iwi iwi: Nānā inā e hana ana ka iwi iwi i nā ʻāpana olakino.
Nā ʻano lapaʻau a pehea e kaohi ai i ka maʻi
ʻO ka nūhou maikaʻi, no ka hapa nui o nā keiki, e hoʻopau wale ʻo Urticaria Pigmentosa iā ia iho i ko lākou wā ʻelemakule . ʻAʻohe lāʻau lapaʻau kūikawā e pale aku ai i ka hoʻokumu ʻana o nā kiko hou. Hiki i nā kiko e kū nei ke hala i nā makahiki e mae ai.
Inā wale nō ka ʻili i kaupalena ʻia ai ke ʻano, ʻaʻohe lāʻau nui e pono ai. Hiki i kāu kauka ke ʻōlelo i kēia mau mea e kōkua ai i ka mālama ʻana i kāu mau hōʻailona:
- Nā antihistamines waha: Kāohi i ka maneʻo a me ka pehu.
- Nā kirimina Corticosteroid: E kau i nā wahi maneʻo.
- Cromolyn sodium: He lāʻau lapaʻau i hāʻawi ʻia e mālama i nā hōʻailona e like me ka luaʻi a me ka maʻi pākī.
- ʻEpiPen: He mea hana i hāʻawi ʻia i ka poʻe i loaʻa i kahi maʻi ʻaleka koʻikoʻi (anaphylactic reaction) e hoʻohana i ka wā pilikia. ʻAʻole pono nā kānaka a pau i kēia.
Nā mea e pale aku ai ma ke ʻano kūikawā
ʻO ka mea nui loa i kēia maʻi, ʻo ia ka noho mamao aku mai nā mea hoʻoulu e hoʻonui ai i nā hōʻailona, ʻo ia hoʻi, nā mea e hoʻomāʻeʻe ai i nā kiko a lilo i ʻulaʻula.
| Nā lāʻau lapaʻau e pale aku ai | Nā mea ʻē aʻe e pale aku ai |
|---|---|
| ʻAspirina | Hoʻoikaika kino (ʻoi aku ka paʻakikī) |
| ʻAila | Ke hamo ikaika nei i ka ʻili me nā kāwele |
| Nā lāʻau hoʻopau ʻeha e like me ka morphine a me ke codeine ('Opiates') | ʻAuʻau ʻana i ka wai wela |
| Thiamine (Wikamina B1) | Nā mea inu wela |
| Quinine (`Quinine` - he lāʻau lapaʻau malaria) | Ka nui o ka lā |
| ʻO Dextromethorphan (he lāʻau lapaʻau no ke kuni) | Ka ʻike ʻia ʻana i ke anu nui |
| ʻO kekahi mau lāʻau hoʻomaha a me nā mea hoʻoluʻu X-ray | Nā mea ʻala a wela |
Mea nui: Inā pono ʻoe a i ʻole kāu keiki e lawe i ka lāʻau lapaʻau no kekahi maʻi ʻē aʻe, e hoʻomaopopo pono i kāu kauka e pili ana iā Urticaria Pigmentosa.
Hiki ke loaʻa nā pilikia?
He mea kākaʻikahi loa, hiki i kēia kūlana ke holomua i ka 'systemic mastocytosis'. Loaʻa i kēlā poʻe ka pilikia nui o kahi hopena allergies koʻikoʻi i kapa ʻia ʻo 'anaphylactic shock', hiki ke hoʻoweliweli i ke ola. Inā ʻike ʻoe i nā hōʻailona e like me ka paʻakikī o ka hanu ʻana a me ka nalowale o ka ʻike , pono ʻoe e hele koke i ka keʻena pilikia o ka haukapila (ETU) .
ʻO nā pilikia ʻē aʻe o ka mastocytosis systemic:
- Osteoporosis: Ka nāwaliwali o nā iwi.
- Cytopenia: Hoʻemi ʻia ka hana ʻana o nā hunaola koko i loko o ke kino.
- Nā pilikia o ke akepaʻa.
- Ka maʻi ʻaʻai (kākaʻikahi loa).
Eia hou, he mau pilikia kakaikahi loa kēia. He mea kakaikahi loa lākou i nā keiki me Urticaria Pigmentosa.
Leka Lawe-Home
- He maʻi ʻili ka Urticaria Pigmentosa e hoʻopilikia nui ana i nā keiki. ʻAʻole ia he mea laha a i hoʻoilina ʻia mai nā mākua mai.
- ʻO ka hōʻailona nui, ke hamo ʻia kahi kiko ʻeleʻele, lilo ia i ʻulaʻula, pehu, a maneʻo (ʻo ia ka hōʻailona o Darier).
- Mai makaʻu pono ʻole, no ka mea, e hoʻomaikaʻi wale kēia ʻano ma kāna iho no ka hapa nui o nā keiki i ko lākou wā ʻelemakule.
- ʻO ka hoʻokele maikaʻi loa, ʻo ia ka noho mamao mai nā meaʻai, nā lāʻau lapaʻau, a me nā hana e hoʻonui ai i nā hōʻailona.
- Inā loaʻa iā ʻoe a i kāu keiki paha kekahi o kēia mau hōʻailona, e ʻike pono i ke kauka no ka hōʻoia pololei a me ka ʻōlelo aʻoaʻo e pono ai.
💬 Comments (0)
ʻAʻohe manaʻo i kau ʻia. Hoʻohui i kāu manaʻo ma ʻaneʻi no ka manawa mua.
Hoʻohui i kāu manaʻo