ʻAʻole mākou e nānā nui i kekahi o nā hōʻailona e kū mai ana i loko o ko mākou kino, ʻeā? Manaʻo mākou he ʻeha iki ia, he ʻulaʻula liʻiliʻi o ka ʻili, a i ʻole he luhi wale nō. Akā i kekahi manawa, ma hope o kēia mau hōʻailona maʻamau, aia paha kahi maʻi lapaʻau kakaʻikahi a koʻikoʻi. I kēia lā e kamaʻilio mākou e pili ana i kekahi maʻi hoʻoilina, ʻo ia hoʻi ka maʻi Fabry. ʻOiai he maʻi kakaʻikahi kēia, he mea nui loa ia no ʻoe a me kou ʻohana e makaʻala iā ia.
He aha ka maʻi Fabry?
I ka ʻōlelo maʻalahi, ʻo ka maʻi Fabry kahi maʻi genetic kahi i hiki ʻole ai ke wāwahi pono ʻia kahi ʻano momona i loko o nā hunaola o ko mākou kino, e hōʻiliʻili ai i loko o ko mākou mau kino. Ke hōʻiliʻili kēia momona, hiki iā ia ke hōʻino i nā kino koʻikoʻi loa i loko o ko mākou kino, e like me nā puʻupaʻa, ka puʻuwai, a me ka ʻōnaehana nerve.
ʻO ke ʻano ʻē o kēia maʻi, ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka . No kekahi poʻe, he palupalu loa nā hōʻailona, ʻaʻole ʻike nui ʻia, a no kekahi poʻe hiki ke koʻikoʻi a hoʻopilikia i ko lākou ola. Eia kekahi, no ka mea ua like kēia mau hōʻailona me nā maʻi maʻamau ʻē aʻe, i kekahi manawa hiki ke ʻike lohi i ka maʻi.
He aha nā hōʻailona maʻamau?
Hiki i ke kanaka me ka maʻi Fabry ke loaʻa i kekahi mau hōʻailona i ka manawa like. E nānā kākou i nā mea nui a me nā mea maʻamau o kēia.
| Hōʻailona | Pehea kēia manaʻo? |
|---|---|
| ʻEha ma nā lima a me nā wāwae | ʻEha, mae wale, ʻūlū a me nā nila , a i ʻole ʻeha nui ma nā poho lima a me nā kapuwaʻi lima. I kekahi manawa, hiki ʻole ke hoʻomanawanui i kēia ʻeha. |
| Nā kiko ʻili | ʻIke pinepine ʻia ia he hui o nā kiko liʻiliʻi, ʻulaʻula ʻeleʻele a poni paha ma kahi ma waena o nā puʻupuʻu a me nā kuli. ʻAʻohe ʻeha o kēia mau mea. |
| Ua emi ka hou ʻana | ʻO ka hou ʻole ʻana ma mua o ka maʻamau, a i ʻole i kekahi poʻe ʻaʻole hou iki, e paʻakikī ai ke ʻae i ke aniau wela a i ʻole ka hoʻoikaika kino. |
| Kuni pinepine | Ka manaʻo pinepine ʻana i ke kuni me ke kumu ʻole. |
| Pehu wāwae | Pehu, ʻoi aku hoʻi ma nā wāwae haʻahaʻa, nā kuʻekuʻe wāwae, a me nā wāwae. He hōʻailona paha kēia e hoʻopilikia ʻia ana nā puʻupaʻa. |
| Luhi nui | Ke ʻano o ka luhi nui a me ka nāwaliwali i ke ao holoʻokoʻa, ʻaʻole ia e nalowale ʻoiai ma hope o ka hiamoe maikaʻi a i ʻole ka hoʻomaha ʻana i ka pō. |
| Nā pilikia o ka ʻōnaehana hoʻoheheʻe | ʻO nā hōʻailona e komo pū ana me ka ʻeha o ka ʻōpū ma hope koke iho o ka ʻai ʻana, ka manaʻo piha, ka neʻe pinepine ʻana o ka ʻōpū, ka nausea, a me ka luaʻi ʻana. |
Aia kekahi ʻokoʻa ma ke ʻano o kona hopena i nā wahine a me nā kāne?
ʻAe, ʻoiaʻiʻo nō. Ma muli o ke ʻano hoʻoilina o ka maʻi, ʻoi aku ka nui o ka loaʻa ʻana o ia ma mua o nā kāne . Eia kekahi, ʻoi aku ka ʻike nui ʻia a me ka koʻikoʻi o nā hōʻailona i nā kāne.
ʻOiai inā loaʻa i nā wāhine ka maʻi Fabry, nui paha ka poʻe ʻaʻole e ʻike i kekahi mau hōʻailona a i ʻole he mau hōʻailona iki loa. Eia naʻe, ʻaʻole kēia manaʻo ʻaʻole i hoʻopilikia ʻia nā wāhine e ka maʻi. Hiki i kekahi mau wāhine ke ʻike i nā hōʻailona koʻikoʻi, ʻoi aku ka ʻeha o ke aʻalolo. No laila, ʻaʻole ia he manaʻo maikaʻi e hoʻowahāwahā i nā hōʻailona no ka mea he akahai lākou.
Pehea e pili ai kēia i nā ʻōkana like ʻole?
ʻO ka hōʻiliʻili ʻana o ka momona i ka maʻi Fabry e hōʻino i nā ʻāpana like ʻole o ke kino. E nānā kākou pehea.
nā puʻupaʻa
ʻO kekahi o nā pilikia koʻikoʻi a weliweli hoʻi o kēia maʻi, ʻo ia ka hāʻule ʻana o nā puʻupaʻa. Hoʻohaiki nā waihona momona i loko o nā kīʻaha koko i nā kīʻaha koko e hoʻolako ana i nā puʻupaʻa, e hōʻino ana i nā puʻupaʻa. Hiki i nā hōʻailona ke komo pū me ka protein a i ʻole ke koko i loko o ka mimi, a me ka pehu ʻana o nā wāwae. I nā hihia koʻikoʻi, pono paha ka dialysis no ke ola holoʻokoʻa a i ʻole ka hoʻoili puʻupaʻa.
ʻŌnaehana nerve
ʻO ka hapa nui o ka poʻe e ʻike mua i ka maʻi Fabry me ka ʻeha o ke aʻalolo. ʻO nā hōʻailona nui ka wela, ka mae ʻana, a me ka ʻeha hiki ʻole ke hoʻomanawanui, ʻoiai ma nā lima a me nā wāwae. Hiki i kēia mau ʻeha ke mau no nā hola a i ʻole nā lā. Hiki ke hele pū kēia ʻeha me nā ʻeha kino, ke kuni, a me ka luhi nui.
Puʻuwai
He mea maʻamau nō hoʻi ka maʻi puʻuwai. Hiki iā ia ke hana i nā puʻuwai puʻuwai kūpono ʻole, ka mānoanoa o ka ʻiʻo puʻuwai, a i ʻole nā pilikia me nā valves puʻuwai (e like me ka mitral insufficiency). Hiki i kēia mau kūlana ke hoʻonui i ka pilikia o nā pilikia koʻikoʻi e like me ka hahau ʻana.
ʻIli
ʻO ka hōʻailona nui nā kiko ʻulaʻula ʻeleʻele (angiokeratomas) a mākou i kūkākūkā ai ma mua. I nā keikikāne, ʻike pinepine ʻia kēia mau mea ma nā lima, nā kuli, nā kuʻekuʻe lima, a me ka wahi ma waena o nā pūhaka a me nā kuli. ʻAʻole nui ka loaʻa ʻana o kēia mau kiko i nā wāhine.
Nā maka
He hōʻailona kikoʻī loa kēia. Hiki ke ʻike ʻia nā kaha keʻokeʻo, e like me ka noe a i ʻole nā kikona e like me ke apo (corneal whorls) ma ka ʻili o ka maka kokoke i ka cornea. ʻOiai ʻaʻole kēia mau mea e hoʻopilikia pinepine i ka ʻike, he mau hōʻailona koʻikoʻi loa ia no ke kauka e ʻike ai i ka maʻi Fabry.
ʻAʻole ʻike pinepine ʻia kēia ʻano loli o ka maka i nā maʻi ʻē aʻe, no laila he kōkua nui ka loaʻa ʻana o kēia hiʻohiʻona i ka ʻike ʻana i ka maʻi Fabry.
Nā pepeiao
Hiki i kekahi poʻe ke ʻike i ke kani ʻana o nā pepeiao, a i ʻole "Tinnitus". Hiki nō hoʻi ke nalowale ka lohe i ka hala ʻana o ka manawa a i ʻole koke.
Nā māmā a me ka ʻōnaehana hoʻoheheʻe
Hiki ke loaʻa nā hōʻailona hanu e like me ke kani 'whooshing' i ka wā e hanu ana, ka pōkole o ka hanu, a me ke kuʻi pinepine. Eia kekahi, ma kahi o ka hapalua o nā wahine e ʻike i nā hōʻailona like me ka Irritable Bowel Syndrome (IBS) (ʻeha ʻōpū, pehu).
Aia kekahi ʻano ʻelua o ka maʻi? (ʻAno 1 a me ke ʻano 2)
ʻAe. Ua māhele nui ʻia ka maʻi Fabry i ʻelua ʻano.
1. ʻAno kuʻuna (ʻAno 1): Hoʻomaka pinepine kēia i ka wā kamaliʻi. ʻO ka mumū a me ka ʻeha ma nā lālā kekahi o nā hōʻailona mua i ka wā kamaliʻi.
2. ʻAno hoʻomaka lohi (ʻAno 2): I kēia ʻano, he mea maʻamau ka wā kamaliʻi a me ka wā ʻōpio. Hoʻomaka nā hōʻailona e ʻike ʻia ma hope o 20 mau makahiki a ma hope paha.
Me ka nānā ʻole i ke ʻano, e ʻoi aku ka ʻino o ka maʻi me ka ʻelemakule. ʻO ke kumu nui o kēia, ʻo ia ka piʻi ʻana o nā waihona momona o ke kino i ka hala ʻana o ka manawa.
Manaʻo paha ʻoe he maʻi laha ʻole kēia a ʻaʻole paha e hiki mai iā ʻoe. Akā inā ʻoe a i ʻole kekahi o kou ʻohana ua loaʻa i kekahi o nā hōʻailona i ʻōlelo ʻia ma kēia ʻatikala no ka manawa lōʻihi, mai hoʻowahāwahā iā ia. ʻO ka mea maikaʻi loa e hana ai, ʻo ia ke ʻike koke i ke kauka a kamaʻilio e pili ana i ia mea.E hana ʻo ia i nā hoʻokolohua e pono ai a e ʻike pono i ke kumu o kēia mau hōʻailona.
Leka Lawe-Home
- He maʻi laha ʻole ka maʻi Fabry i hoʻoulu ai i ka hōʻiliʻili ʻana o ka momona i loko o nā ʻōkana o ke kino.
- ʻO nā hōʻailona nui ka mumū o nā lima a me nā wāwae, ka mae ʻole o ka ʻili, nā kiko ʻulaʻula ʻeleʻele ma ka ʻili, ka emi ʻana o ka hou ʻana, a me ka luhi nui.
- Hiki i kēia maʻi ke hōʻino nui i nā ʻōpū nui e like me nā puʻupaʻa, ka puʻuwai, a me ka ʻōnaehana nerve.
- ʻOi aku ka maʻamau a me ka koʻikoʻi o nā hōʻailona i nā kāne, ʻoiai ʻoi aku ka maʻalahi o nā hōʻailona o nā wahine a ʻaʻohe paha.
- Inā loaʻa iā ʻoe a i ʻole kekahi o kou ʻohana kekahi o nā hōʻailona i ʻōlelo ʻia ma kēia ʻatikala a hoʻomau lākou no ka manawa lōʻihi, e ʻoluʻolu e nīnau koke i kāu kauka no ka ʻōlelo aʻo.
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