He aha ka maʻi ʻo Gaucher a pehea e mālama ai

Anter

• Nā hōʻailona nui
• Pehea e hana ʻia ai ke kuhi
• Pehea e hana ai i ka lāʻau

ʻO ka maʻi Gaucher kahi maʻi maʻi laha ʻole e hōʻike ʻia e ka hemahema o ka enzyme e kumu ai i ka momona i loko o nā pūnaewele e waiho i loko o nā ʻāpana like ʻole o ke kino, e like me ke akepaʻa, ʻāpala a me ka akemāmā, a me nā iwi a iwi kuamoo. .

No laila, ke hilinaʻi nei i ka pūnaewele i hoʻopilikia ʻia a me nā ʻano ʻē aʻe, hiki ke hoʻokaʻawale ʻia ka maʻi i nā ʻano 3:

• ʻAno 1 Gaucher maʻi - non-neuropathic: ʻo ia ka ʻano maʻamau a hoʻopili i nā mākua a me nā keiki, me ka holomua a me ke ola maʻamau me ka lawe pololei ʻana o nā lāʻau.
• ʻO ka maʻi Gaucher ʻano 2 - keʻano neuropathic koʻikoʻi: pili i nā pēpē, a ʻike pinepine ʻia a hiki i ka 5 mau makahiki o ka makahiki, he maʻi maʻi nui ia, a hiki i ka makahiki 2 ke make;
• ʻO ka maʻi Gaucher ʻano 3 - ke ʻano neuropathic subacute: pili i nā keiki a me nā ʻōpio, a hana pinepine ʻia kāna kuhi ma 6 a 7 mau makahiki. ʻAʻole koʻikoʻi e like me ka form 2, akā hiki iā ia ke alakaʻi i ka make ma kahi o 20 a 30 mau makahiki paha, ma muli o nā neurological a me nā pilikia pulmonary.

Ma muli o ke koʻikoʻi o kekahi ʻano o ka maʻi, pono e hōʻike koke ʻia kāna kuhi, i mea e hoʻomaka ai ka lapaʻau kūpono a hoʻemi i nā pilikia i hiki ke hoʻoweliweli i ke ola.

Nā hōʻailona nui

Hiki ke loli nā ʻōuli o ka maʻi Gaucher ma muli o ke ʻano o ka maʻi a me nā wahi i loli ʻia, akā ʻo nā ʻōuli maʻamau e pili ana:

• Luhi nui loa;
• Hoopanee ulu;
• Kiko ihu;
• ʻEha iwi;
• Nā haki kūlohelohe;
• Hoʻonui i ke ake a me ka lepe;
• ʻO nā veins varicose i loko o ka esophagus;
• Abdominal eha.

Hiki paha i nā maʻi iwi e like me osteoporosis a i ʻole osteonecrosis. A ʻo ka hapa nui o ka manawa, ʻaʻole hōʻike ʻia kēia mau ʻōuli i ka manawa like.

Ke hoʻopili ka maʻi i ka lolo, ʻike ʻia nā hōʻailona ʻē aʻe, e like me ka neʻe ʻole o ka maka, ka ʻāʻī o ka ʻū, paʻakikī i ka ale ʻana

Pehea e hana ʻia ai ke kuhi

Hoʻokumu ʻia ke kuhi o ka maʻi o Gaucher ma muli o nā hopena o nā hoʻokolohua e like me ka biopsy, puncture spleen, hoʻokolohua koko a i ʻole ka puncture spinal cord.

Pehea e hana ai i ka lāʻau

ʻAʻohe lāʻau i ka maʻi Gaucher, eia nō naʻe, aia kekahi mau ʻano lapaʻau e hiki ai ke hōʻoluʻolu i nā hōʻailona a ʻae i kahi ola maikaʻi aʻe. I ka hapanui o nā hihia, hana ʻia ka lapaʻau me ka hoʻohana ʻana o ka lāʻau no ke koena o kou ola, me nā lāʻau i hoʻohana pinepine ʻia ʻo Miglustat a i ʻole Eliglustat, nā mea lapaʻau e pale ai i ka hoʻokumu ʻana o nā mea momona e hōʻiliʻili i loko o nā lālā.

I nā hihia ʻoi loa, koi paha ke kauka i ka hoʻoneʻe ʻana o ka iwi iwi a i ʻole ke ʻoki ʻana e wehe i ka iwi maha.