Aia kekahi o kou ʻohana, ʻoiai he keiki, i loaʻa i kahi maʻi hormonal hānau? Ua kamaʻāina paha ʻoe i nā pilikia e like me ka lohi o ka ulu ʻana, ka wā ʻōpiopio, a me nā pōʻaiapuni menstrual ʻole i nā wahine. ʻO kahi ʻano genetic kakaikahi e hiki ai ke hana i kēia mau mea ua kapa ʻia ʻo 'Congenital Adrenal Hyperplasia'. E kapa mākou iā ia ʻo CAH no ka pōkole. I kēia lā, e kamaʻilio pōkole mākou e pili ana i kēia a me kahi lāʻau hou i hiki mai no ia.
I ka ʻōlelo maʻalahi, he aha ka CAH?
He maʻi hoʻoilina ka CAH e pale ai i nā kelepa adrenal, ʻelua mau kelepa liʻiliʻi ma luna o nā puʻupaʻa, mai ka hana ʻana i ka cortisol, he hormone koʻikoʻi no ko kākou ola.
E noʻonoʻo i ko kākou kino e like me kahi hale hana. Ke emi iki kekahi o nā huahana (cortisol) i loko o kēia hale hana, e huhū ke kikowaena hoʻomalu i loko o ka lolo (pituitary gland) a hoʻouna i nā hōʻailona (ACTH) i nā kelepa adrenal e hana ikaika. Ma muli o kēia hoʻoulu nui ʻana, ʻoiai ʻaʻole e hoʻonui ʻia ka hana cortisol, hoʻomaka ia e hana i nā hormones kāne hou aʻe i kapa ʻia ʻo 'androgens' .
ʻO kēia ʻano hormonal imbalance hiki ke alakaʻi i nā pilikia he nui:
- Nā pēpē wahine: I ka wā hānau, ua like paha ke ʻano o nā ʻāpana o waho me ko ke pēpē kāne.
- No nā ʻaoʻao ʻelua: ʻo nā pilikia e like me ka lohi o ka ulu ʻana, ka wā ʻōpiopio, a me ka hānau ʻole ʻana e hiki mai ana ma hope o ke ola.
Eia kekahi, hiki i kekahi mau maʻi CAH ke emi iho ka hana ʻana o kahi hormone koʻikoʻi ʻē aʻe i kapa ʻia ʻo aldosterone. Me ka ʻole o kēia hormone, hiki i ka CAH ke lilo i mea koʻikoʻi loa, e alakaʻi ana i ka haʻalulu, ka coma, a i ʻole ka make.
Ka mālama ʻana i nā makahiki 70 a me ka manaʻolana hou
No nā makahiki he ʻumi, ʻo ke ʻano lapaʻau wale nō no CAH, ʻo ia ke pani ʻana i nā pae haʻahaʻa o ka hormone cortisol o ke kino me nā dosis kiʻekiʻe o nā steroids , e like me ka hydrocortisone. Eia nō naʻe, ʻo ka lawe ʻana i kēia mau dosis kiʻekiʻe o nā steroids no ka manawa lōʻihi hiki ke hana i kekahi mau hopena ʻaoʻao. ʻO ka mea maʻamau o kēia mau mea, ʻo ia ka piʻi ʻana o ke kaumaha, ke koko kiʻekiʻe, nā iwi nāwaliwali, a me ka ulu ʻole o nā keiki.
Akā i kēia manawa, he nūhou maikaʻi! Ma hope o 70 mau makahiki, ua ʻāpono ka FDA i ka lāʻau mua a hoʻokahi wale nō i hoʻomohala kūikawā ʻia no CAH, ʻo crinecerfont (inoa inoa: Crenessity).
Pehea e hana ai kēia lāʻau lapaʻau hou (Crinecerfont)?
ʻO kēia ka mea nui loa. ʻAʻole e like me ka lāʻau lapaʻau kahiko, hana kēia lāʻau lapaʻau hou ma ke ʻano ʻokoʻa loa.
ʻAʻole hoʻohui ʻo 'Crinesurfont' i nā hormones mai waho. Akā, hele pololei kēia lāʻau i ke kikowaena kaohi o ka lolo (ka pituitary gland) a ālai i ka hoʻokuʻu ʻana o ka hōʻailona makemake ʻole ʻia (ACTH) e hoʻoulu ai i nā kelepa adrenal.
I ka ʻōlelo maʻalahi, ua like kēia me ka hoʻopau ʻana i ke 'kuhi' o kahi ʻōnaehana pono ʻole. Hoʻomalu kēia i ka hana ʻana o ke kino i nā hormones 'androgen' pono ʻole.
ʻO ka pōmaikaʻi nui o kēia, ʻoiai ua kāohi ʻia nā pae androgen, hiki ke hoʻemi nui ʻia ka nui o nā steroids e pono ai e mālama i ke kaulike hormonal. ʻO ke ʻano kēia, hiki ke hoʻemi nui ʻia nā hopena ʻaoʻao i hoʻokumu ʻia e nā steroids.
| ʻAno | Lapaʻau kahiko (nā lāʻau steroid kiʻekiʻe) | Lapaʻau hou (Crinecerfont + nā lāʻau steroid haʻahaʻa) |
|---|---|---|
| Hana | Ke hāʻawi nei i ka hormone cortisol i hoʻemi ʻia ma waho. | Ke ālai nei i nā hōʻailona makemake ʻole ʻia (ACTH) mai ka lolo a me ke kaohi ʻana i ka hana androgen. |
| Pahuhopu nui | Pono nā dosis kiʻekiʻe e kāohi i nā pae hormone. | Hoʻohaʻahaʻa i nā pae androgen, e hoʻemi ana i ka nui o nā steroids e pono ai. |
| Nā hopena ʻaoʻao | Nā pilikia lōʻihi e like me ka piʻi ʻana o ke kaumaha, ka ulu ʻana o ka ulu ʻana, a me nā iwi nāwaliwali. | Ka hiki ke hoʻemi ʻia nā hopena ʻaoʻao e pili ana i ka steroid. Ua hōʻike ʻia nā hopena ʻaoʻao liʻiliʻi (ʻeha poʻo, luhi). |
No wai kēia lāʻau lapaʻau kūpono loa?
Ua ʻāpono ʻia ʻo 'Crinesurfont' no nā poʻe maʻi CAH ma luna o 4 mau makahiki . ʻAʻole ia he pani no nā steroids, akā he mea hoʻohui i nā steroids.He lāʻau hoʻohui.
- No nā keiki: ʻOiai ke ulu mau nei nā keiki, hiki i nā lāʻau kiʻekiʻe o nā steroids ke loaʻa ka hopena nui i ko lākou ulu ʻana. No laila, hiki i kēia lāʻau lapaʻau, hiki ke hoʻemi i ka nui o nā steroids, ke lilo i mea pono loa no lākou. He mea nui loa kēia i ka wā ʻōpio, i ka wā e paʻakikī ai ke kaohi i nā hormones.
- No nā pākeke: Hiki i kēia lāʻau lapaʻau ke hāʻawi i ka hōʻoluʻolu i nā pākeke e ʻeha nei i nā hopena ʻaoʻao mai ka lawe ʻana i nā steroids no ka manawa lōʻihi, a i ʻole ka poʻe paʻakikī i ka mālama ʻana i nā pae hormone paʻa.
E aʻo e pili ana i nā hopena ʻaoʻao a me ka palekana
Ma nā hoʻokolohua lapaʻau, ua hōʻike wale ʻia kekahi mau hopena ʻaoʻao liʻiliʻi a pōkole me kēia lāʻau.
- No nā pākeke: luhi, ʻeha poʻo, poniuniu, ʻeha hui.
- No nā keiki: ʻeha poʻo, ʻeha ʻōpū, luhi, ihu paʻa.
ʻO ka mea nui, ʻo ka hapa nui o kēia mau hōʻailona he akahai, a he haʻahaʻa loa ka nui o nā maʻi i hoʻōki i ka lawe ʻana i ka lāʻau.
ʻŌlelo Aʻo Kūikawā: E makaʻala i ka pilikia Adrenal!
I ka wā e lawe ai i kēia lāʻau lapaʻau, he mea nui loa ia e makaʻala i kahi maʻi koʻikoʻi i kapa ʻia ʻo 'adrenal crisis'. I ka ʻōlelo maʻalahi, hiki ke hana ʻia kēia maʻi inā ʻaʻole loaʻa i ke kino ka nui o ka 'cortisol' i ka wā o ke kaumaha nui (e.g., kahi maʻi koʻikoʻi, ʻoki kino, ulia pōpilikia).
No laila, e kamaʻilio me kāu kauka a e ʻike pono i ka nui o ka steroid āu e lawe ai i kahi pilikia ("stress dose") . Eia kekahi, e makaʻala i nā hōʻailona o kahi 'pilikia adrenal' (e.g., ka luaʻi nui ʻana, ka maʻi diarrhea, ka dizziness, ka nalowale o ka ʻike) a e hoʻomanaʻo e hele koke i ka haukapila a i ʻole ka lumi pilikia (ETU) inā loaʻa kēlā mea.
Pehea e ʻike ai inā kūpono kēia lāʻau lapaʻau iaʻu/kaʻu keiki?
Pono e hana ʻia kēia hoʻoholo me ke kūkākūkā ʻana me kāu kauka, ʻoiai he endocrinologist . E noʻonoʻo kāu kauka i nā kumu he nui, me kāu mau hopena hoʻāʻo koko, ka nui o ka ulu ʻana, ke kaumaha, ke koko, a me nā pae hormone o kēia manawa, e hoʻoholo ai inā kūpono kēia lāʻau lapaʻau iā ʻoe.
ʻAʻole kēia he lāʻau lapaʻau no CAH, akā nui ka manaʻolana e kōkua ia i ka hoʻomaikaʻi ʻana i ke ʻano o ke ola no nā maʻi, e hōʻemi i nā hopena ʻaoʻao steroid, a e maʻalahi ka hoʻokele ʻana i ka hormone.
Leka Lawe-Home
- ʻO ke ʻano o CAHʻO kahi kūlana hoʻoilina kahi e hana ai nā kelepa adrenal i ka cortisol emi a me nā hormones androgen nui aʻe.
- He lāʻau lapaʻau hou ʻo Crinecerfont i hoʻomohala kūikawā ʻia no CAH, ʻo ia ka mua o kona ʻano i loko o 70 mau makahiki.
- ʻAʻole kēia he lāʻau lapaʻau e pani ai i nā steroids. He lāʻau lapaʻau ia i hāʻawi ʻia ma waho aʻe o nā steroids a kōkua i ka hōʻemi ʻana i ka nui o nā steroids e pono ai.
- ʻO ka pōmaikaʻi nui ka hiki ke hōʻemi i nā hopena ʻaoʻao lōʻihi o nā steroids.
- E hōʻoia e nīnau i kāu kauka e pili ana i ke kūlana pilikia i kapa ʻia ʻo 'adrenal crisis' a me ka "stress dose" āu e lawe ai i kēlā manawa.
- He mea nui e kūkākūkā me ke kauka i loea i nā maʻi hormonal e hoʻoholo ai inā kūpono kēia lāʻau iā ʻoe a i kāu keiki paha.
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