E noʻonoʻo ʻoe ua hoʻomaka wale ʻoe e lawe i kahi lāʻau lapaʻau hou no kekahi maʻi. Ma hope o hoʻokahi a ʻelua paha pule, manaʻo ʻoe ua maʻi ʻoe, me he mea lā e loaʻa ana iā ʻoe ke anu. ʻEha kou kino, manaʻo ʻoe i ke kuni, haʻalulu ʻoe... A laila koke, hoʻomaka kou kino e pohā i nā wahi ʻulaʻula e lilo mālie i mau pehu wai, a hoʻomaka kou ʻili e ʻili me he mea lā ua puhi ʻia ʻoe. He kūlana weliweli loa kēia, ʻeha, a hiki ke hoʻoweliweli i ke ola. I kēia lā, ke kamaʻilio nei mākou e pili ana i kēia kūlana weliweli, ʻo ia hoʻi ʻo Toxic Epidermal Necrolysis (TEN) .
I ka ʻōlelo maʻalahi, he aha ka Toxic Epidermal Necrolysis (TEN)?
ʻO ka Toxic Epidermal Necrolysis, a i ʻole TEN no ka pōkole, he ʻano ʻili ʻeha loa ia a hiki ke hoʻoweliweli i ke ola. Hoʻoulu ia i ka pehu a me ka ʻili ʻana o nā wahi nui o kou kino, ma ka liʻiliʻi he 30% o kou kino. Hiki ke hana ʻia kēia ʻili ʻana ma nā wahi me nā membrane mucous, e like me ka waha, nā maka, a me nā genital.
Hoʻomaka pinepine kēia ʻano weliweli ma ke ʻano he hopena koʻikoʻi i kekahi mau lāʻau lapaʻau a mākou e lawe ai. Ke hōʻino nui ʻia ka ʻili, aia kahi pilikia nui o ka maloʻo a me nā maʻi koʻikoʻi.
I mea e pololei ai, ʻo TEN ke ʻano koʻikoʻi loa o kahi maʻi ʻili ʻē aʻe i kapa ʻia ʻo Stevens-Johnson syndrome (SJS). I ka SJS, emi ma mua o 10% o ka ʻili o ke kino e hemo ana. Ke hoʻopilikia ʻia he 10% a 30% o ke kino, kapa mākou ia he kūlana SJS-TEN overlap. Inā ʻoi aku ma mua o 30% o ka ʻili e hemo ana, ua manaʻo ʻia he TEN.
ʻO wai ka mea i pilikia nui i ka loaʻa ʻana o kēia maʻi?
ʻOi aku ka nui o ka poʻe me nā ʻōnaehana pale i nāwaliwali i ka loaʻa ʻana o ka maʻi Stevens-Johnson (SJS) a i ʻole TEN. Aia kekahi mau kumu e hoʻonāwaliwali ʻia ai ka ʻōnaehana pale.
- ʻO nā kānaka i loaʻa i ka maʻi pale ʻole kanaka (HIV).
- ʻO nā kānaka me kekahi mau maʻi kanesa, e like me ka lymphoma.
Eia kekahi, hiki i nā kumu hoʻoilina ke pāʻani i kahi kuleana. ʻAʻole hiki i ke kino o kekahi poʻe ke wāwahi a hoʻololi i kekahi mau lāʻau lapaʻau ma ke ʻano maʻamau. He mea kēia e loaʻa mai ana mai ko lākou mau genes. I ia mau hihia, hiki i kēlā mau lāʻau lapaʻau ke hōʻiliʻili i loko o ke kino a hoʻoulu i nā hopena koʻikoʻi e like me SJS a i ʻole TEN. Eia kekahi, ma muli o nā loli (mutations) i kekahi mau genes, ʻoi aku ka nui o ka pane ʻana o kekahi poʻe i kekahi mau lāʻau lapaʻau.
ʻOiai hiki ke loaʻa ka TEN i kēlā me kēia makahiki, ʻoi aku ka maʻamau o ia i ka poʻe ʻelemakule. Eia nō naʻe, he maʻi kakaikahi loa ia.He mea nui e hoʻomanaʻo i ka honua holoʻokoʻa, ʻo SJS a me TEN i hui pū ʻia e hoʻopili wale i hoʻokahi a ʻelua miliona mau kānaka i kēlā me kēia makahiki.
He aha nā kumu nui o TEN?
I nā hihia he nui, manaʻoʻiʻo nā kauka ʻo kekahi mau lāʻau lapaʻau ke kumu nui o TEN. E nānā kākou i nā lāʻau lapaʻau i ʻoi aku ka pilikia.
| Māhele lāʻau lapaʻau | He wehewehe maʻalahi |
|---|---|
| ʻAllopurinol | He lāʻau lapaʻau i hoʻohana ʻia no ka mālama ʻana i nā maʻi e like me nā pōhaku puʻupaʻa a me ka gout. |
| Nā lāʻau hoʻōla ʻōʻili | He papa o nā lāʻau lapaʻau i hāʻawi ʻia e kāohi i nā kūlana e like me ka hopu ʻana. |
| Nā lāʻau anti-retroviral | ʻOi aku hoʻi nā lāʻau lapaʻau i hoʻohana ʻia e mālama i ka maʻi HIV (e like me nevirapine). |
| Nā Oxicams | ʻO kēia nā lāʻau lapaʻau no nā NSAID, a he papa ia o nā lāʻau lapaʻau anti-inflammatory. |
| Nā Sulfonamides | ʻO kēia kekahi ʻano lāʻau antibiotic. |
Ma waho aʻe o kēia mau lāʻau lapaʻau, he mea kākaʻikahi loa, hiki i kekahi mau maʻi a i ʻole nā lāʻau pale ke hana i ka TEN. Akā he mea kākaʻikahi loa kēia. I kekahi manawa, ʻaʻole hiki ke loaʻa ke kumu o TEN.
He aha nā hōʻailona o TEN?
ʻO ka maʻamau, hoʻomaka nā hōʻailona he ʻUMI e ʻike ʻia i loko o kekahi mau pule o ka hoʻomaka ʻana i kahi lāʻau lapaʻau hou. Hoʻomaka ia me nā hōʻailona e like me ke anu.
- ʻEha kino
- Kuke
- Kuni a me nā anu
- ʻEha poʻo
- ʻUlaʻula a me ka mumū o nā maka
Ma hope o kēia mau hōʻailona mua, hoʻomaka ka ʻike ʻia ʻana o nā kiko ʻulaʻula ʻeha (rash) ma ka ʻili. Hoʻonui mālie kēia mau kiko, lilo i mau pehu piha i ka wai, a ma hope e hoʻomaka ka ʻili e ʻili. ʻO nā ʻeha e hana ʻia i ka wā e ʻili ai ka ʻili ua kapa ʻia ʻo erosions . He mau ʻeha ʻeha loa kēia, hāmama e like me nā kuni nui.
ʻIke mua ʻia kēia mau ʻeha ma ka maka a me ka umauma, a laila hiki ke laha i nā ʻāpana ʻē aʻe o ke kino.
- Nā Maka: Hiki ke hōʻino ʻia ka ʻaoʻao o loko o ka lihilihi (conjunctiva) a me ka ʻaoʻao o waho o ka maka ʻeleʻele (cornea).
- Nā membrane mucous: Hiki ke loaʻa nā ʻeha i loko o ka waha, ka ihu, ka puʻu, a me ke ala hanu.
- ʻAla urethral, anus, a me nā genitals: Hiki ke hōʻeha nui ʻia kēia mau wahi.
ʻO kēia mau ʻeha e hoʻolilo i nā hana maʻalahi e like me ka ʻai ʻana, ka moni ʻana, ka hanu ʻana, ka pani ʻana i kou mau maka, a me ka mimi ʻana i mea ʻeha loa a paʻakikī hoʻi.
Nā pilikia koʻikoʻi e hiki ke hana ma muli o TEN
ʻAʻole he kūlana e lawe māmā ʻia ʻo TEN. Hiki iā ia ke alakaʻi i nā pilikia koʻikoʻi e hoʻoweliweli ana i ke ola.
- Nā maʻi: Ke haki ka ʻili, ʻoi aku ka nui o ka manawa e komo ai nā germs i ke kino. Inā komo kēia mau germs i ke kahe koko, hiki ke hana ʻia kahi maʻi weliweli loa i kapa ʻia ʻo sepsis .
- ʻO ka hāʻule ʻana o nā ʻōkana he nui: ʻO ka mumū nui a me ka maʻi i loko o ke kino e hiki ke hana i ka hāʻule ʻana o nā ʻōkana o loko e like me nā puʻupaʻa, ke akepaʻa, a me nā māmā.
- Pneumonia: Aia ka nui o ka pilikia o nā maʻi māmā ma muli o nā hōʻeha i ka ʻōpū hanu.
Ma muli o ka hiki ke loaʻa i ia mau pilikia koʻikoʻi, pono e hoʻokomo koke ʻia ka mea me nā hōʻailona TEN i ka haukapila a mālama ʻia .
Pehea e ʻike ai nā kauka i kēia maʻi?
Hiki i ke kauka kūpono ke ʻike i ka maʻi TEN ma ka nānā ʻana i kou ʻili a me ka nīnau ʻana e pili ana i kāu mau hōʻailona. I kekahi manawa, pono paha kahi biopsy ʻili e hōʻoia i ka maʻi.
ʻO kēia ke komo ʻana i ka lawe ʻana i kahi ʻāpana ʻili liʻiliʻi loa mai kahi ʻeha ʻili ma lalo o ka anesthesia a nānā iā ia ma lalo o kahi microscope. Hiki i kēia hoʻāʻo ke ʻike maopopo inā he mau ʻili make (nā ʻili necrotic) a inā ua kaʻawale ka papa o luna o ka ʻili (epidermis) mai ka papa o lalo.
He aha nā lāʻau lapaʻau no kēia?
ʻO ka mea nui loa, he pilikia olakino ʻo TEN.ʻAʻole kēia he maʻi hiki ke mālama ʻia ma ka home. Pono ʻoe e hele koke i ka Emergency Treatment Unit (ETU) o ka haukapila i ka wā e hoʻomaka ai nā hōʻailona.
Ma ka haukapila, hoʻokomo pinepine ʻia ka mea maʻi i loko o kahi keʻena mālama koʻikoʻi (ICU) a i ʻole ke kikowaena kuni, no ka mea, pono i kēia mau mea maʻi ka mālama kūikawā e like me ka mea i hāʻawi ʻia i kahi mea maʻi kuni.
Hiki ke hoʻokaʻawale ʻia nā ʻano lapaʻau i ʻelua ʻāpana nui.
| Nā koho lapaʻau no TEN | |
|---|---|
| Lapaʻau koke | |
| Ke hoʻōki nei i ka lāʻau lapaʻau kumu | Hoʻopau koke ʻia nā lāʻau lapaʻau a pau i manaʻo ʻia e hoʻoulu ai i ka TEN. |
| Ka mālama ʻana i nā pae wai o ke kino | ʻO ka ʻili ʻana o ka ʻili ke kumu o ka wehe ʻia ʻana o ka nui o ka wai mai ke kino. No laila, hāʻawi ʻia ka saline (nā wai IV) ma o kahi aa koko e kāohi i nā pae wai o ke kino. |
| Kāohi ʻana i ka maʻi | Hoʻopili ʻia nā kirīmi a i ʻole nā mea poni antiseptic i nā ʻeha hāmama. Hāʻawi ʻia nā antibiotics i nā hōʻailona mua o ka maʻi. |
| Nā lāʻau lapaʻau kūikawā ʻē aʻe | |
| ʻO ka globulin pale kino (IVIG) | He wai kēia i hāʻawi ʻia ma o kahi aʻa koko. Loaʻa iā ia nā antibodies i lawe ʻia mai ka plasma koko o nā kānaka olakino. Kōkua kēia i ka kaohi ʻana i ka pane pale ʻino o ke kino. |
| Nā lāʻau pale kino | Hāʻawi ʻia nā lāʻau lapaʻau e kāohi i ka ʻōnaehana pale o ke kino mai ka hoʻouka ʻana i kona mau hunaola ponoʻī, e hoʻonāwaliwali ai i kāna hana. |
| Plasmapheresis | ʻO kēia ka hoʻouna ʻana i ke koko o ka mea maʻi i kahi mīkini kūikawā, ka hoʻokaʻawale ʻana a me ka wehe ʻana i nā antibodies ʻino i loko o ka plasma koko, a me ka hoʻihoʻi ʻana i ke koko i hoʻomaʻemaʻe ʻia i ke kino. |
Nā hopena lōʻihi ma hope o ka hoʻōla ʻana
Hiki i nā mea i ola i ka TEN ke loaʻa i kekahi mau hopena lōʻihi. ʻOiai inā e ho'ōla loa ka ʻili, hiki ke kū mai nā pilikia ʻē aʻe.
- ʻIli, lauoho a me nā kui: ka ulu ʻana o nā kui a i ʻole ka hāʻule ʻana, nā kiko ʻili (ʻeleʻele a keʻokeʻo paha), ka nalowale o ka lauoho ma kekahi mau wahi (alopecia areata), ka ʻili maloʻo a me ka ʻāʻī, ka hou nui.
- Nā Maka: Ka mumū lōʻihi a i ʻole ka ʻulaʻula o nā maka, nā maka maloʻo, nā ʻano ʻē o ka lihilihi, ka ʻike ʻana i ka mālamalama, ka ʻike maka pōwehiwehi a i ʻole, kākaʻikahi, ka makapō.
- Waha, ihu a me ka ʻōnaehana hanu: Bronchitis, nā maʻi māmā e like me COPD, waha maloʻo, maʻi ʻāʻī, nalowale o ka ʻono a me ke ʻala, hōʻino ʻana o ka māmā.
- ʻŌnaehana mimi a me ka hānau keiki: Ka hōʻeha lōʻihi a i ʻole ka mumū o ka urethra, vagina, a i ʻole penis, paʻakikī i ka mimi ʻana, maloʻo ka vagina i nā wahine.
He mea nui loa e kamaʻilio me kāu kauka e pili ana i kēia mau hopena lōʻihi a e ʻimi i ka lāʻau e pono ai.
Leka Lawe-Home
- He hopena koʻikoʻi loa ka Toxic Epidermal Necrolysis (TEN) i kekahi mau lāʻau lapaʻau, a hiki ke hoʻoweliweli i ke ola.
- Hoʻomaka ia me nā hōʻailona maʻamau e like me ke kuni a me ka ʻehaʻeha o ke kino, akā a laila holomua i nā pehu ʻeha a me ka ʻili ʻili me he mea lā ua wela.
- He pilikia olakino kēia. Inā ʻike ʻoe i kēia mau hōʻailona, pono ʻoe e hele koke i ka Emergency Treatment Unit (ETU) o ka haukapila.
- Inā loaʻa iā ʻoe kahi ʻulaʻula ʻili, pehu, a i ʻole nā hōʻailona ʻē aʻe ma hope o ka hoʻomaka ʻana i kahi lāʻau lapaʻau hou, mai hoʻowahāwahā iā ia. E ʻike koke i kāu kauka.
- Inā ua loaʻa iā ʻoe kahi maʻi allergy i kekahi lāʻau lapaʻau ma mua, e hoʻomaopopo pono i kāu kauka i kēia i kēlā me kēia manawa āu e hele ai e kiʻi i ka lāʻau lapaʻau.
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