Duchenne muscular dystrophy: he aha ia, nā ʻōuli a me nā lāʻau

Anter

• Nā hōʻailona nui
• Pehea e hōʻoia ai i ke kuhi
• Pehea e hana ai i ka lāʻau
• 1. Hoʻohana o nā lāʻau
• 2. Nā kau Physiotherapy
• He aha ke ola o ke ola
• ʻO nā pilikia maʻamau
• He aha ke kumu o kēia ʻano dystrophy

ʻO Duchenne muscular dystrophy kahi maʻi maʻi laha ʻole e hoʻopili wale i nā kāne a ʻike ʻia e ka nele o kahi protein i nā mākala, i ʻike ʻia ʻo dystrophin, ka mea e kōkua i ka mālama ʻana i nā hunaola mākala. No laila, kumu kēia maʻi i ka nāwaliwali o ka musculature holoʻokoʻa o ke kino, ka mea i ʻoi aku ka paʻakikī i ke keiki e kiʻi i nā hanana nui e ulu ana, e like me ka noho ʻana, kū ʻana a i ʻole ka hele wāwae ʻana.

I nā manawa he nui, ʻike wale ʻia kēia maʻi ma hope o 3 a 4 mau makahiki ke hoʻololi ke keiki i ke ala o ka hele wāwae, ka holo ʻana, ka piʻi ʻana i ke alapiʻi a i ʻole ke ala aʻe ʻana mai ka papahele, ʻoiai nā ʻāpana e pili mua i ka pūhaka, nā ʻūhā. a me nā poʻohiwi. Me ka neʻe ʻana o ka makahiki, hoʻopili ka maʻi i nā mākala hou a he nui nā keiki e pau i ka hilinaʻi i ka noho huila ma kahi o 13 mau makahiki.

ʻAʻohe lāʻau o Duchenne muscular dystrophy, akā kōkua ka mālama ʻana e hoʻopaneʻe i ka hoʻomohala ʻana o ka maʻi, kaohi ʻana i nā ʻōuli a pale i ka hoʻomaka ʻana o nā pilikia, ʻoi aku ma nā pae puʻuwai a me nā hanu. No laila, he mea nui loa ka mālama ʻana me ke kauka lapaʻau a i ʻole ke kauka ʻē aʻe i loea i ka maʻi.

Nā hōʻailona nui

ʻO nā ʻōuli nui o Duchenne muscular dystrophy ʻike pinepine ʻia mai ka makahiki mua o ke ola a hiki i ka makahiki 6, ke piʻi nei i mua o nā makahiki, a hiki i ka makahiki 13, ua hilinaʻi ke keiki i ka noho huila.

ʻO kekahi o nā hōʻailona a me nā ʻōuli maʻamau e pili pū me:

• Lohe hiki ke noho, kū a hele wāwae paha;
• Ke haʻalulu nei a i ʻole ka paʻakikī e piʻi i nā alapiʻi a i ʻole ka holo ʻana.
• Hoʻonui i ka nui o nā bipi keiki, ma muli o ke kuapo o nā hunaola musele me ka momona;
• Ka paʻakikī e hoʻoneʻe i kou mau hono, ʻo ke kūlou hoʻi i kou mau wāwae.

Mai ka ʻōpio, hiki ke hoʻomaka i nā pilikia ʻoi loa o ka maʻi, ʻo ia hoʻi, ka paʻakikī i ka hanu ʻana ma muli o ka nāwaliwali o ka diaphragm a me nā mākia hanu ʻē aʻe, a ʻo nā pilikia puʻuwai hoʻi, no ka nāwaliwali o ka puʻuwai puʻuwai.

Ke hoʻomaka ka pilikia, hiki i ke kauka ke hoʻololi i ka lāʻau e hoʻāʻo e hoʻokomo i ka mālama ʻana i nā pilikia a hoʻomaikaʻi i ka maikaʻi o ke ola. I nā hihia ʻoi loa, pono paha ka haukapila.

Pehea e hōʻoia ai i ke kuhi

I nā manawa he nui, kānalua ka pediatrician i ka Duchenne mustrular dystrophy wale nō ma ka helu ʻana i nā hōʻailona a me nā hōʻailona i hōʻike ʻia i ka wā o ka hoʻomohala.Eia nō naʻe, hiki iā lākou ke hana i ke koko e ʻike i ka nui o kekahi mau enzyme, e like me ka creatine phosphokinase (CPK), i hoʻokuʻu ʻia i loko o ke koko ke loaʻa ka haʻihaʻi o nā mākala.

Aia kekahi mau hoʻokolohua kūlohelohe, kahi e kōkua ai e hōʻea i kahi hōʻoia hope loa, a nānā i nā loli i nā genes ke kuleana no ka hoʻomaka ʻana o ka maʻi.

Pehea e hana ai i ka lāʻau

ʻOiai ʻaʻohe lāʻau o Duchenne muscular dystrophy, aia kekahi mau lāʻau e kōkua i ka pale ʻana i ka wikiwiki a ʻae ʻia ka mālama ʻana i nā ʻōuli, a me ke ʻano o nā pilikia. ʻO kekahi o kēia mau ʻano mālama ʻana:

1. Hoʻohana o nā lāʻau

I ka hapanui o nā hihia, hana ʻia ka mālama ʻana o Duchenne muscular dystrophy me ka hoʻohana ʻana i nā lāʻau corticosteroid e like me prednisone, prednisolone a i ʻole deflazacort. Pono e hoʻohana i kēia mau lāʻau no ke ola, a loaʻa ka hana o ka hoʻoponopono ʻana i ka ʻōnaehana pale, e hana ana ma ke ʻano he anti-inflammatory a hoʻolohi i ka nalo ʻana o ka hana muscle.
Eia nō naʻe, hoʻolōʻihi ka hoʻohana ʻana i nā corticosteroids i nā hopena ʻē aʻe e like me ka hoʻonui ʻana i ka makemake, ka loaʻa ʻana o ke kaumaha, ka momona, ka paʻa ʻana o ka wai, ka osteoporosis, ke kino pōkole, ka hanu kiʻekiʻe a me ka maʻi kō, a e hoʻohana wale ʻia ma lalo o ka nānā a ke kauka. E nānā hou aʻe e pili ana i ke ʻano o nā corticosteroids a pehea e pili ai i ke olakino.

2. Nā kau Physiotherapy

ʻO nā ʻano o ka physiotherapy i hoʻohana mau ʻia e mālama iā Duchenne muscular dystrophy he motine a me ka respiratory kinesiotherapy a me ka hydrotherapy, ka mea e hoʻolōʻihi i ka hiki ʻole ke hele, mālama i ka ikaika o nā mākala, hoʻomaha i ka ʻeha a pale i nā haʻi hanu a me nā haʻi iwi.

He aha ke ola o ke ola

ʻO ka lōʻihi o ke ola no Duchenne muscular dystrophy ma waena o 16 a 19 mau makahiki, akā naʻe, me ka mua o ka lāʻau a me ka puka ʻana o nā hoʻoponopono hou a me ka mālama, ua hoʻonui ʻia kēia mea i manaʻo ʻia. No laila, ʻo ke kanaka i hana i ka lāʻau lapaʻau i koi ʻia e ke kauka hiki ke ola ma mua o ka makahiki 30 a loaʻa kahi ola maʻamau, me nā hihia o nā kāne e noho nei ma mua o 50 mau makahiki me ka maʻi.

ʻO nā pilikia maʻamau

ʻO nā pilikia nui i hoʻokumu ʻia e Duchenne muscular dystrophy:

• Scoliosis koʻikoʻi;
• Hanu paʻakikī;
• Pulumonia;
• Ka lawa ʻole o ka cardiac;
• Ka momona a i ʻole kaʻai pono ʻole.

Eia hou, hiki i nā mea maʻi me kēia dystrophy ke ʻike i ka hoʻohaʻahaʻa noʻonoʻo liʻiliʻi, akā ʻaʻole pili kēia ʻano i ka lōʻihi a me ke koʻikoʻi o ka maʻi.

He aha ke kumu o kēia ʻano dystrophy

Ma ke ʻano he maʻi genetical, hana ka muscular dystrophy o Duchenne ke hana ʻia kahi mutation i kekahi o nā ʻāpana e kuleana ai i ka hana ʻana o ke kino i ka protein proteintrtrin, ka ʻāpana DMD. He mea nui loa kēia protein no ka mea kōkua ia i nā hunaola i ke olakino i ka manawa holoʻokoʻa, ka pale ʻana iā lākou mai nā hōʻeha i hoʻokumu ʻia e ka hoʻoneʻe ʻana o nā mākala maʻamau a me ka hoʻomaha.

No laila, ke hoʻololi ʻia ka gene DMD, ʻaʻole lawa ka protein i hana ʻia a hoʻopau nā nāwaliwali i nāwaliwali a ʻeha hoʻi i nā ʻeha ma ka manawa. He mea nui kēia protein no nā mākala e hoʻoponopono i ka neʻe ʻana, a no ka puʻuwai puʻuwai.