He mea hoʻoweliweli iki nā pōhaku puʻupaʻa iā kākou a pau, ʻeā? Ua lohe paha ʻoe e pili ana i kēlā ʻeha. Akā, ua ʻike anei ʻoe he ʻano pōhaku puʻupaʻa like ʻole? I kēia lā, e kamaʻilio kākou e pili ana i kahi ʻano pōhaku kakaʻikahi akā koʻikoʻi loa. ʻO ia nā pōhaku cystine. E nānā kākou i ke kumu o ko lākou hana ʻana, he aha nā hōʻailona, a me nā mea hiki ke hana ʻia e pili ana iā lākou.
I ka ʻōlelo maʻalahi, he aha nā pōhaku cystine?
He ʻano pōhaku puʻupaʻa nō hoʻi nā pōhaku Cystine. He mau ʻano paʻakikī nā pōhaku puʻupaʻa, e like me ka pōhaku i hana ʻia e ka hōʻiliʻili ʻana o nā kemika like ʻole i loko o kā mākou mimi. Hana ʻia nā pōhaku Cystine i kahi kemika i kapa ʻia ʻo cystine . He waikawa amino koʻikoʻi ka Cystine no ko mākou kino. I ka pololei, he ʻāpana liʻiliʻi ia o nā protein e kōkua i ke kūkulu ʻana i nā ʻiʻo a hoʻoponopono i nā ʻiʻo i loko o ko mākou kino.
ʻO ka maʻamau, hoʻihoʻi hou ko mākou mau puʻupaʻa i kēia cystine i loko o ke kino. Akā i kekahi poʻe, ʻaʻole hana pono kēia kaʻina hana. A laila, hōʻiliʻili ka cystine i hiki ʻole ke omo ʻia i loko o ka mimi a, i ka hala ʻana o ka manawa, hana i nā pōhaku. Hiki i kēia mau pōhaku cystine ke ulu iki aʻe ma mua o nā pōhaku puʻupaʻa ʻē aʻe. No laila i kekahi manawa pono e wehe ʻia ma o ke ʻoki ʻana e hoʻōki i ka hōʻino ʻana i nā puʻupaʻa a i ʻole ke ala mimi.
No laila he aha ka Cystinuria?
ʻO ke kumu nui o kēia mau pōhaku cystine he maʻi genetic i kapa ʻia ʻo cystinuria . He maʻi hoʻoilina laha ʻole kēia. I ka ʻōlelo maʻalahi, inā loaʻa iā ʻoe kēia maʻi, ʻaʻole hiki i kou kino ke hoʻoheheʻe pono i kahi kemika i kapa ʻia ʻo cystine. No laila, hōʻiliʻili ia i loko o kou mimi a hana i nā pōhaku.
ʻO ka mea nui, he maʻi ola ka cystinuria. ʻAʻohe lāʻau lapaʻau no ia mea. Akā me ka mālama kūpono, hiki iā ʻoe ke mālama pono i ke kūlana a ola i kahi ola maʻamau .
No ka mea he maʻi hoʻoilina kēia, pono i ke keiki ke hoʻoilina mai ko lākou makuahine a me ko lākou makuakāne e hoʻomohala ai . ʻO ke kanaka me kēia maʻi e loaʻa paha iā ia nā pōhaku cystine i nā manawa he nui i ko lākou ola.
Pehea ka maʻamau o kēia ʻano? He aha nā hōʻailona?
ʻAʻole kēia he mea e hana ʻia i nā kānaka a pau, he mea kākaʻikahi iki ia. ʻO ka maʻamau, inā ʻoe e lawe i 1 a i ʻole 2 mai loko mai o 100 poʻe e loaʻa ana nā pōhaku puʻupaʻa, hoʻokahi a ʻelua paha o lākou e loaʻa nā pōhaku cystine. Akā, ʻo ka mea kupanaha, ʻike pinepine ʻia kēia ʻano ma nā keiki a me nā ʻōpio . Ma kahi o 80% o ka poʻe e loaʻa ana nā pōhaku cystine e loaʻa i kā lākou pōhaku mua ma mua o 20 mau makahiki. Eia nō naʻe, hiki i kēia mau pōhaku ke hana ʻia i kēlā me kēia makahiki.
No nā hōʻailona, ua like loa lākou me nā pōhaku puʻupaʻa ʻē aʻe. ʻAʻole ʻike pinepine ʻia nā hōʻailona a hiki i ka wā e hana ʻia ai kahi pōhaku.
| Hōʻailona | Wehewehena |
|---|---|
| ʻEha nui ma ke kua a i ʻole ka pelvis | Hiki ke like me ka ʻeha ʻoi. ʻO ka maʻamau, ʻike ʻia kēia ma kekahi ʻaoʻao. |
| ʻEha ma ka ʻōpū lalo a i ʻole ka ʻūhā | I ka neʻe ʻana o ka pōhaku i lalo o ke ala mimi, hiki ke ʻike ʻia ka ʻeha ma ka ʻōpū lalo. |
| Koko i loko o ka mimi | Hiki ke ʻike ʻia ka mimi he poni, ʻulaʻula, a ʻeleʻele paha. |
| Ka luaʻi a me ka nausea | Hiki ke hele mai ka luaʻi a me ka luaʻi me ka ʻeha nui. |
| ʻEha i ka wā e mimi ai | Ke kokoke ka pōhaku i ka ʻōpū mimi, hiki iā ʻoe ke manaʻo i ka ʻeha a i ʻole ke kuni i ka wā e mimi ai. |
I kekahi manawa, hiki i kēia mau pōhaku cystine ke lilo i mea nui e ālai i ke ala mimi a hoʻoulu i kahi maʻi ala mimi (UTI) .
He aha nā pilikia e hiki ke hana?
He maʻi ola ka Cystinuria, a inā ʻaʻole mālama pono ʻia, hiki iā ia ke alakaʻi i nā pilikia. He mea nui e hana pū me kāu kauka e mālama i kēia maʻi.
ʻO nā pilikia e hiki mai ana:
- Nā maʻi puʻupaʻa: Hiki i nā pōhaku ke ālai i ke kahe o ka mimi a ʻae i nā germs e komo i loko o nā puʻupaʻa.
- Ka ālai ʻana o ka ureter: Hiki ke ālai ʻia ka ʻōmole e lawe ana i ka mimi mai ka puʻupaʻa a i ka ʻōpū mimi (ureter) e ka pōhaku.
- Nā maʻi ʻōpū mimi pinepine (UTI).
- Ka hōʻino mau loa ʻana o ka puʻupaʻa:Inā waiho ʻole ʻia ka mālama ʻole no ka manawa lōʻihi, e emi paha ka hana o nā puʻupaʻa.
Pehea e ʻike ai ke kauka i kēia? He aha nā lāʻau lapaʻau?
Inā loaʻa iā ʻoe nā hōʻailona e like me nā pōhaku puʻupaʻa, e hana kāu kauka i kekahi mau hoʻokolohua e hoʻoholo ai inā he mau pōhaku cystine kēia.
Nā ʻano hana hōʻoia
- Ke noi nei iā ʻoe no nā kikoʻī: E nīnau ke kauka e pili ana i kāu mau hōʻailona a inā paha ua loaʻa i kekahi o kou ʻohana nā pilikia like.
- Nā hoʻāʻo: Hiki ke kauoha ʻia kahi hoʻāʻo mimi a me nā hoʻāʻo kiʻi e like me ka ultrasound scan a i ʻole CT scan .
- Ke hoʻāʻo ʻana i ka pōhaku: Inā he pōhaku kou i puka i waho o kou mimi, hiki ke hoʻouna ʻia i kahi keʻena hoʻokolohua no ka hoʻāʻo ʻana e hoʻoholo pono i ke ʻano o ka pōhaku. ʻO kēia wale nō ke ala e hōʻoia 100% ai he pōhaku cystine ia.
Nā ʻano lapaʻau
ʻO ka pahuhopu nui o ka mālama ʻana i nā pōhaku cystine, ʻo ia ke pale ʻana i ke kūkulu ʻia ʻana o nā pōhaku hou a me ka wehe ʻana i nā pōhaku e kū nei. E hoʻomohala kāu kauka i kahi hoʻolālā lapaʻau kūpono iā ʻoe.
- Nā loli i ke ʻano o ka nohona a me ka ʻai
- E inu i ka wai nui: ʻO kēia ka mea nui loa. E ʻōlelo paha kāu kauka iā ʻoe e inu ma ka liʻiliʻi he 3-4 lita o ka wai i ka lā. ʻO ia hoʻi, pono ʻoe e hoʻomaʻamaʻa i ka inu ʻana i hoʻokahi kīʻaha wai i kēlā me kēia hola. Mai kahaha inā ala ʻoe i ka pō e inu i ka wai. Hoʻoheheʻe ka wai i kou mimi a hōʻemi i ka hōʻiliʻili ʻana o ka cystine.
- Kaohi ʻana i ka paʻakai (sodium): E hōʻemi i ka nui o ka paʻakai āu e hoʻohui ai i kāu meaʻai. E pale i nā meaʻai e like me nā meaʻai i loko o ka pahu a me nā meaʻai i hoʻopaʻa ʻia a i ʻole i hoʻopaʻa ʻia, ka mea i nui ka paʻakai.
- Hoʻemi i ka protein holoholona: Hiki iā ʻoe ke hōʻemi i kāu ʻai ʻana i ka protein holoholona e like me ka ʻiʻo, ka iʻa, a me nā hua manu a kālele i ka protein mea kanu.
- Nā lāʻau lapaʻau
Inā ʻoe e ʻeha ana ma muli o ka pōhaku, e kuhikuhi kāu kauka i nā lāʻau ʻeha. Eia kekahi, aia nā lāʻau lapaʻau kūikawā e hiki ke kōkua i ka pale ʻana i nā pōhaku cystine hou mai ka hoʻokumu ʻana. ʻO Tiopronin a me d-penicillamine kekahi mau lāʻau lapaʻau. Kōkua kēia i ka hoʻoheheʻe ʻana i ka cystine. Pono e lawe ʻia kēia mau lāʻau lapaʻau ma lalo o ka nānā ʻana o ke kauka .
- ʻoki kino
Inā nui loa nā pōhaku, e ālai ana i ke ala mimi, a i ʻole e hōʻeha nui ana, pono paha e wehe ʻia ma ke ʻoki ʻana.
- Ureteroscopy: Hoʻokuʻu ʻia kahi kāmela liʻiliʻi a maʻalahi hoʻi i lalo o ka urethra e ʻimi i ka pōhaku a wāwahi iā ia i mau ʻāpana a wehe loa paha.
- ʻO ka Nephrolithotomy Percutaneous:He hana ia e hana ʻia ai kahi ʻoki liʻiliʻi ma ka ʻili o ke kua a hoʻokomo ʻia kahi ʻōmole ma loko o ia mea i loko o ka puʻupaʻa e wehe i ka pōhaku. Hana pinepine ʻia kēia no nā pōhaku nui loa.
Pehea ʻoe e noho ai me kēia kūlana? He mea anei ia e makaʻu ai?
He maʻi ola ka Cystinuria. Akā inā mālama pono ʻia, ʻaʻohe mea e hopohopo ai. ʻAʻole pinepine ka maʻi e hoʻoweliweli i ke ola. Inā mālama pono ʻia, ʻaʻole pinepine ia e holomua i nā maʻi koʻikoʻi e like me ka hāʻule ʻana o ka puʻupaʻa.
ʻO ka mea nui loa, ʻo ia ka mālama ʻana i ka pilina maikaʻi ma waena ou a me kāu kauka. Ma ka hahai pono ʻana i nā kuhikuhi a me ka hele ʻana i nā keʻena lapaʻau i ka manawa kūpono, hiki iā ʻoe ke kaohi maikaʻi i kēia maʻi.
No ka nānā ʻana i ka holomua o ka lāʻau lapaʻau, e hana mau ke kauka i nā hoʻāʻo mimi e nānā i ka pae o ka cystine i loko o ka mimi. Inā pono, e hoʻoponopono ʻia ka hoʻolālā lapaʻau e like me ia. ʻOiai he kūlana genetic kēia, he manaʻo maikaʻi paha ke ʻimi i ka ʻōlelo aʻoaʻo genetic inā ʻoe e hoʻolālā e hoʻomaka i kahi ʻohana.
Leka Lawe-Home
- ʻO nā pōhaku Cystine kahi ʻano pōhaku puʻupaʻa i hana ʻia e kahi ʻano maʻi genetic kakaikahi i kapa ʻia ʻo cystinuria.
- ʻOiai ke ola mau kēia maʻi, hiki ke mālama maikaʻi ʻia me ka mālama kūpono a me nā hoʻololi ʻana i ke ʻano o ka nohona.
- ʻO ka inu ʻana i ka nui o ka wai i ka lā a pau ke ala nui a maʻalahi hoʻi e pale ai i ke kūkulu ʻia ʻana o nā pōhaku.
- He mea nui loa ka hahai ʻana i ka ʻai haʻahaʻa i ka paʻakai a me ka protein holoholona.
- Inā ʻike ʻoe i nā hōʻailona e like me ka ʻeha nui ma kou kua, pelvis, a i ʻole ka ʻōpū lalo, a i ʻole ke koko i loko o kou mimi, mai hoʻonele i ka manawa a e ʻike koke i ke kauka .
- E hahai pono i nā ʻōlelo aʻoaʻo a kāu kauka a me nā lāʻau lapaʻau. Mai hopohopo, hiki iā ʻoe ke ola i kahi ola olakino me kēia maʻi.
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