E noʻonoʻo ʻoe ua loaʻa iā ʻoe ke kuni, ʻeha ke kino, a hoʻomaka ʻoe e lawe i kahi lāʻau lapaʻau i kuhikuhi ʻia e ke kauka. He aha inā, i loko o kekahi mau lā, malia paha he hoʻokahi a ʻelua paha pule, loaʻa koke iā ʻoe nā kiko ʻulaʻula ma kou kino, nā ʻeha i loko o kou waha, i loko o kou mau maka, a i loko o kou mau ʻāpana kino, a hoʻomaka kou ʻili e pehu a ʻili me he mea lā ua puhi ʻia? He ʻano weliweli maoli kēia, he mea weliweli loa, a he mea kakaikahi. I kēia lā e kamaʻilio mākou e pili ana i kēia ʻano i kapa ʻia ʻo Stevens-Johnson Syndrome (SJS). He mea nui loa i nā mea a pau ke ʻike i kēia.
He aha ʻo SJS a me TEN? He aha ka ʻokoʻa ma waena o nā mea ʻelua?
ʻO ka Stevens-Johnson Syndrome (SJS) kahi hopena hypersensitivity koʻikoʻi, maʻamau i kahi lāʻau. Pili ia i ka hōʻino ʻana i ko mākou ʻili a me nā membrane mucous, e make ai nā cell a hemo ka ʻili. ʻO nā membrane mucous nā membrane palupalu a pulu e hoʻopili ana i ko mākou waha, ihu, maka, genitals, a me ka anus.
Aia kekahi ʻano koʻikoʻi ʻē aʻe o kēia maʻi, a mākou e kapa nei ʻo Toxic Epidermal Necrolysis (TEN) . I ka ʻōlelo maʻalahi, ua like ʻo SJS a me TEN me nā ʻaoʻao ʻelua o ka maʻi hoʻokahi.
- SJS (Stevens-Johnson Syndrome): I kēia hihia , ʻaʻole i hiki i ka 10% o ka ʻili o ke kino e hemo ana.
- TEN (Toxic Epidermal Necrolysis): He ʻano koʻikoʻi loa kēia a he mea hoʻoweliweli paha i ke ola kahi e nalowale ai ma mua o 30% o ka ʻili o ke kino.
Ma muli o ke koʻikoʻi loa o kēia mau kūlana ʻelua, pono e hoʻokomo ʻia ka mea maʻi i ka haukapila , i kekahi manawa i loko o kahi keʻena mālama koʻikoʻi (ICU) a i ʻole ke keʻena kuni.
ʻO wai ka mea i loaʻa pinepine i kēia maʻi?
ʻOiai hiki ke loaʻa ka SJS i kēlā me kēia makahiki, ʻoi aku ka maʻamau i nā keiki a me nā ʻōpio ma lalo o 30 makahiki. Hiki nō hoʻi ke loaʻa i ka poʻe ʻelemakule. ʻOi aku ka maʻamau o ka SJS i nā wahine ma mua o nā kāne.
Aia kekahi ʻokoʻa iki i ka nānā ʻana i ke kumu:
- ʻO ke kumu nui o SJS i nā keiki he mau maʻi lele e like me ka niumonia .
- ʻO ke kumu nui o SJS/TEN i nā mākua he hopena alerika i kahi lāʻau lapaʻau .
He aha nā hōʻailona maoli o SJS?
He mea nui loa ka makaʻala i kēia mau hōʻailona, no ka mea, ʻo ka wikiwiki o kou ʻike ʻana iā lākou, ʻo ka wikiwiki hoʻi e hiki ai iā ʻoe ke ʻimi i ka lāʻau lapaʻau. ʻIke pinepine ʻia nā hōʻailona i loko o hoʻokahi a ʻekolu paha pule o ka hoʻomaka ʻana i ka lāʻau lapaʻau.
ʻO nā hōʻailona mua he mau hōʻailona anu maʻamau e like me ke kuni, ka ʻeha kino, ka ʻeha poʻo, a me ke kuni. ʻO kēia ka mea e paʻakikī ai ke ʻike i ka SJS i nā lā mua. A laila wale nō e ʻike ʻia ai nā hōʻailona ʻili koʻikoʻi.
Aia ma lalo iho nā hōʻailona nui o SJS.
| Hōʻailona | Ua wehewehe maʻalahi ʻia |
|---|---|
| ʻEha nui o ka ʻili | Ke manaʻo nei i kahi ʻeha wela ikaika loa a hiki ʻole iā ʻoe ke hoʻopā i kou ʻili. |
| ʻO ke kuni a me nā ʻeha kino | ʻO nā hōʻailona ke kuni nui, nā ʻeha kino, a me ke poʻo. |
| Nā kiko ʻulaʻula a me nā ʻāpana | Nā kiko ʻulaʻula a i ʻole nā ʻeha e hoʻomaka ana ma ka maka a me ka umauma a laha i ke kino holoʻokoʻa. |
| Nā pehu a me nā ʻeha | Nā pehu a me nā ʻeha ʻeha ma ka ʻili, i loko o ka waha, ka ʻāʻī, nā maka, nā ʻōpū, a me ka rectum. |
| ʻIli ʻili | Pohā nā pehu a hemo ka papa luna o ka ʻili i nā ʻāpana nui. |
| Ke kuha ʻana i ka lawai mai ka waha mai | Ka wai kahe ma muli o nā ʻeha waha, ka hiki ʻole ke pani i ka waha. |
| Hoʻopili maka | ʻO nā pehu a me nā ʻūhū ma nā maka, e paʻakikī ai ke wehe i nā maka. |
| ʻEha i ka wā o ka mimi ʻana | ʻEha nui i ka wā e mimi ai ma muli o ka pehu ʻana o nā membrane mucous. |
Nā kumu nui a me nā ʻano lāʻau lapaʻau e hiki ai ke hoʻoulu iā SJS
ʻO ke kumu nui o SJS he hopena maʻi i kahi lāʻau lapaʻau. Eia naʻe, ʻaʻole kēia e hana me nā lāʻau lapaʻau āpau. Aia kekahi mau ʻano lāʻau lapaʻau i ʻoi aku ka nui o ka hoʻoulu ʻana i ka SJS.
| Kumu | Wehewehena |
|---|---|
| Nā maʻi ʻaleka i nā lāʻau lapaʻau | ʻO kēia ke kumu o ka hapa nui o nā hihia SJS a me TEN. |
| Nā maʻi | ʻOi loa aku i nā keiki, hiki ke hana ʻia e nā maʻi e like me ka Mycoplasma pneumonia, Herpes, a me Hepatitis A. |
| Nā kumu ʻē aʻe | ʻO ka maʻi graft-versus-host, kahi pilikia e kū mai ana ma hope o nā hoʻoili iwi iwi, nā lāʻau pale (kākaʻikahi loa), a i kekahi manawa ʻaʻole hiki ke loaʻa ke kumu. |
Nā lāʻau lapaʻau me ka nui o ka pilikia o ke kumu ʻana o SJS
- Nā lāʻau sulfa antibacterial: Kekahi mau ʻano antibiotics.
- Nā lāʻau lapaʻau anti-epileptic: No ka laʻana, nā lāʻau lapaʻau e like me `Phenytoin (Dilantin®)`, `Carbamazepine (Tegretol®)`, `Lamotrigine (Lamictal®)`, `Phenobarbital (Luminal®)`.
- ʻAllopurinol: He lāʻau lapaʻau i hoʻohana ʻia e mālama i ka gout a me kekahi ʻano pōhaku puʻupaʻa (`Aloprim®`, `Zyloprim®`).
- Nā lāʻau hoʻopau ʻeha (NSAID): Nā lāʻau hoʻopau ʻeha ʻaʻole steroidal e like me `Piroxicam (Feldene®)`, `Diclofenac (Cambia®, Flector®)`. Nā lāʻau lapaʻau ʻē aʻe e like me `Nevirapine (Viramune®)`.
- Nā lāʻau antibiotic ʻē aʻe: Hiki i kekahi mau ʻano lāʻau antibiotic ke hana i kēia.
He mea nui: ʻAʻole e loaʻa i nā kānaka a pau ka SJS mai nā lāʻau lapaʻau i helu ʻia ma ʻaneʻi. He miliona miliona ka poʻe e hoʻohana ana iā lākou. Eia nō naʻe, ua ʻike ʻia kēia mau lāʻau lapaʻau he kiʻekiʻe aʻe ka pilikia o ka hoʻomohala ʻana i ka SJS. Mai ho'ōki i ka lawe ʻana i kahi lāʻau lapaʻau i kuhikuhi ʻia e ke kauka ma muli o kēia ʻatikala.
Ka ʻike ʻana a me ka mālama ʻana i ka maʻi
Inā ʻike ʻoe a i ʻole kekahi āu i ʻike ai i nā hōʻailona i luna, ʻoiai inā ua hoʻomaka wale ʻoe i kahi lāʻau lapaʻau hou, he mea nui e hele koke i ka Emergency Department (ETU) o ka haukapila kokoke loa .
Hoʻoholo nā kauka i kēia maʻi ma o:
- Ma ka nānā ʻana i kou ʻili a me nā membrane mucous (e like me ka waha, nā maka).
- Ma ka nīnau ʻana e pili ana i ke kiʻekiʻe o ka ʻeha āu e manaʻo nei.
- Ma ka nānā ʻana i ka wikiwiki o ka laha ʻana o nā hōʻailona ʻili a me ka nui o ko lākou laha ʻana.
- Inā pono, hiki ke lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻili a nānā ʻia (biopsy ʻili).
Lapaʻau no SJS
ʻO nā pahuhopu nui o ka mālama ʻana no SJS, ʻo ia ka hoʻōki ʻana i ka lāʻau i hoʻoulu ai i ka maʻi, kaohi ʻana i nā hōʻailona, pale i nā pilikia, a kōkua i ka hoʻōla ʻana o ka ʻili. He mea koi ka hoʻokipa ʻana i ka haukapila no kēia.
Aia ma lalo kekahi mau ʻano lapaʻau:
| ʻAno lapaʻau | Wehewehena |
|---|---|
| Ka hoʻopau ʻana i ka lāʻau hoʻoulu | ʻO kēia ka hana mua a koʻikoʻi loa. Hoʻōki koke ʻia ka lāʻau i manaʻo ʻia he kumu no SJS. |
| Ka hāʻawi ʻana i nā wai a me nā paʻakai i ke kino | ʻO ka ʻili ʻana o ka ʻili ke kumu o ka nalowale ʻana o ka nui o ka wai mai ke kino, no laila hoʻohana ʻia nā wai IV e hoʻolako i nā wai a me nā electrolytes e pono ai. |
| Ka mālama ʻili | Ua uhi ʻia ka ʻili i ʻeha me nā lole hoʻopili kūikawā ʻaʻole e pipili, e like me ka mālama ʻana i kahi mea kuni. |
| Ka hāʻawi ʻana i ka meaʻai | He paʻakikī ke ʻai ʻana ma muli o nā ʻeha waha. No ka mea, he nui ka ikehu e pono ai e ho'ōla, hāʻawi ʻia ka meaʻai kiʻekiʻe-calorie ma o kahi paipu i hoʻokomo ʻia ma ka ihu i loko o ka ʻōpū (tube-feeding). |
| Kaohi ʻeha | He ʻano ʻeha loa kēia, no laila ua hāʻawi ʻia nā mea hoʻomaha ʻeha ikaika. |
| ʻO ka lawelawe lapaʻau kūikawā | E ʻimi i ke kōkua o ke kauka ʻili, a inā pili nā maka, e ʻimi i ke kauka ophthalmologist. |
Nā pilikia o SJS a me nā hopena ma hope o ka hoʻōla ʻana
He maʻi koʻikoʻi loa ka SJS hiki ke alakaʻi i ka make. Ma kahi o 10% o nā maʻi me SJS a hiki i ka 50% o nā maʻi me ke ʻano ʻoi aku ka ʻino, ʻo TEN, e make. ʻO nā kumu nui o ka make, he mau pilikia koʻikoʻi e like me ka sepsis, ka pneumonia, a me ka hāʻule ʻana o nā ʻōkana he nui.
ʻOiai inā ua ho'ōla ʻia ka maʻi, hiki i kekahi poʻe ke loaʻa nā hopena lōʻihi.
- Nā loli o ka ʻili: ʻIli maloʻo, maneʻo, hoʻololi ke ʻano o ka ʻili.
- Nā pilikia maka: nā maka maloʻo, ʻeha pinepine o ka maka, ka ʻike maka pohihihi, ka paʻakikī i ka nānā ʻana i ka mālamalama (photophobia).
- ʻĒ aʻe: Ka hou nui ʻana, ka hōʻino ʻana o ka māmā, ka nalowale a i ʻole ke ʻano o nā kui, ka nalowale o ka lauoho, nā pilikia o ka ʻono.
He mea nui loa: Inā ʻoe e hoʻopili hou ʻia i ka lāʻau i hoʻoulu ai i ka SJS, i ka lua o ka manawa hiki ke ʻoi aku ka ʻino o ke kūlana ma mua o ka mea mua. No laila, he mea nui e hoʻomanaʻo i ka inoa o ka lāʻau i hoʻoulu ai i ka SJS a me nā lāʻau like no ke koena o kou ola a e hoʻomaopopo i kēlā me kēia kauka āu e ʻike ai.
Leka Lawe-Home
- ʻO ka Stevens-Johnson Syndrome (SJS) kahi hopena ʻili koʻikoʻi e hoʻoweliweli ana i ke ola, i hoʻokumu pinepine ʻia e ka lāʻau lapaʻau.
- ʻO nā hōʻailona mua e pili ana i ke kuni a me ka ʻehaʻeha o ke kino, akā ma hope e holomua i nā ʻāpana ʻulaʻula ʻeha ma ka ʻili, nā pehu, a me ka ʻili ʻili.
- Inā ʻike ʻoe i kēia mau hōʻailona ma hope o ka hoʻomaka ʻana i kahi lāʻau lapaʻau hou, e hele koke i ka Emergency Department (ETU) o ka haukapila me ka hoʻokaʻulua ʻole.
- ʻO ka lāʻau lapaʻau nui, ʻo ia ka ʻike a me ka hoʻōki ʻana i ka lāʻau i hoʻoulu ai i ka maʻi, a hāʻawi i ka mālama kākoʻo ma ka haukapila.
- E hoʻomanaʻo i ka inoa o ka lāʻau i hoʻoulu ai i kāu SJS no ke koena o kou ola a e hoʻomaopopo i kāu kauka e pili ana i ia mea i kēlā me kēia manawa āu e hele ai no ka lāʻau lapaʻau. Hiki i kēia ke hoʻopakele i kou ola.
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