Ke mae a luhi mau nei kāu keiki? He Thalassemia paha ia?

Ke luhi mau nei kāu keiki liʻiliʻi? ʻAʻole anei ʻo ia e holo a pāʻani e like me nā keiki ʻē aʻe? He makemake nui anei ʻo ia e ʻai? Ua ʻike anei ʻo ia i ka maeʻele? He mea maʻamau no kekahi makuahine a makuakāne paha ke makaʻu nui i ka ʻike ʻana i nā mea e like me kēia. ʻOiai hiki ke hoʻokumu pinepine ʻia kēia mau hōʻailona e nā kūlana maʻamau, i kekahi manawa aia paha kahi maʻi ma hope o kēia, e like me Thalassemia. No laila i kēia lā, e kamaʻilio kākou e pili ana i ke ʻano o Thalassemia, pehea e pili ai i ko mākou kino, a he aha nā lāʻau lapaʻau no ia.

He aha maoli ka Thalassemia?

I ka ʻōlelo maʻalahi, he maʻi hoʻoilina ka thalassemia e hoʻopilikia ai i ko kākou koko . ʻAʻole ia he mea laha. ʻO ia hoʻi, ʻaʻole ia e laha mai kekahi kanaka a i kekahi kanaka e like me ke anu. He mea ia a kākou e hoʻoilina ai mai ko kākou mau mākua ma o ko kākou mau genes.

I mea e hoʻomaopopo iki ai i kēia, e kamaʻilio mua kākou e pili ana i nā hunaola koko ʻulaʻula i loko o ko kākou koko. E noʻonoʻo i kēia mau hunaola koko ʻulaʻula e like me kahi lawelawe lawe e lawe ana i ka oxygen ma o ko kākou kino. ʻO ke kaʻa o kēia lawelawe lawe, ʻo ia hoʻi, ke kaʻa lawe oxygen, ua kapa ʻia ʻo hemoglobin . He protein kūikawā ka hemoglobin i loaʻa i loko o nā hunaola koko ʻulaʻula. ʻO kēia ka mea e lawe ana i ka oxygen mai ko kākou mau māmā a hāʻawi iā ia i kēlā me kēia hunaola o ke kino.

ʻAʻole hiki i ke kanaka me ka thalassemia ke hana i ka lawa o kēia protein i kapa ʻia ʻo hemoglobin. E like me ke kūkulu ʻia ʻana o kahi kaʻa, inā ʻaʻole lawa nā ʻāpana a i ʻole inā hoʻohana ʻia nā ʻāpana haʻahaʻa, ʻaʻole e hana pono ke kaʻa. Hoʻemi kēia i ka helu o nā hunaola koko ʻulaʻula olakino i loko o ke kino. Kāhea mākou i kēia kūlana o ka anemia koko ʻulaʻula haʻahaʻa.

No laila, ke loaʻa ʻole i nā ʻāpana o ke kino ka nui kūpono o ka oxygen, ʻaʻole hiki iā lākou ke hana i ka ikehu e pono ai lākou. ʻO ia ke kumu e ʻike ai nā poʻe maʻi thalassemia i nā hōʻailona like ʻole.

ʻO wai ka mea i pilikia nui i ka loaʻa ʻana o kēia maʻi?

Ua manaʻo ʻia ua ulu mua nā hoʻololi genetic e hoʻokumu ai i kēia i loko o ke kanaka ma ke ʻano he pale ʻana i ka malaria. No laila, ʻo ka poʻe me nā pilina genetic i nā wahi i ʻike nui ʻia ai ka malaria, e like me ʻApelika, ʻEulopa hema, a me nā ʻāina ma Komohana, Hema, a me Hikina ʻAsia, ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o ka thalassemia. ʻOiai he ʻāina ʻAsia Hema nō hoʻi ʻo Sri Lanka, aia nā mea lawe thalassemia a me nā maʻi i waena o ko mākou heluna kanaka.

He aha ke kumu o ka thalassemia? E nānā pono kākou.

ʻO kēlā "kaʻa oxygen" i kapa ʻia ʻo hemoglobin aʻu i ʻōlelo ai ma mua, ua hana ʻia ia me ʻehā mau kaulahao protein. ʻElua o kēia mau mea i kapa ʻia he mau kaulahao alpha globin , a ʻo nā mea ʻelua ʻē aʻe i kapa ʻia he mau kaulahao beta globin .

Loaʻa iā mākou nā "kuhikuhi" a i ʻole "code" e hana i kēia mau kaulahao mai nā genes a mākou e hoʻoilina ai mai ko mākou mau mākua. E noʻonoʻo i kēia mau genes ma ke ʻano he meaʻai no ka hana ʻana i kahi kīʻaha. Inā he kīnā a hewa paha i ke ʻano, ʻaʻole hiki ke hoʻomākaukau pono ʻia ke kīʻaha. Pēlā nō, inā he kīnā a hemahema paha i loko o kēia mau genes, ʻaʻole e hana pono ʻia nā kaulahao alpha a beta globin paha. ʻO ia ka manawa e kū mai ai kahi maʻi i kapa ʻia ʻo thalassemia.

• Nā kaulahao alpha globin: Pono kēia i 4 mau genes e hana. ʻElua mai ka makuahine a ʻelua mai ka makuakāne.
• Nā kaulahao globin Beta: Pono kēia i ʻelua mau genes e hana. Hoʻokahi mai ka makuahine a hoʻokahi mai ka makuakāne.

ʻO ke ʻano o ka thalassemia āu e loaʻa ai, pili ia i ka mea o kēia mau kaulahao alpha a beta paha i loaʻa kahi kīnā gene. Hoʻoholo ʻia ke koʻikoʻi o ka maʻi e ka helu o nā genes kīnā.

He aha nā ʻano nui o ka thalassemia?

Ua māhele ʻia ka Thalassemia i kekahi mau pae nui ma muli o ke koʻikoʻi o ka maʻi. ʻO ia hoʻi, ʻo ke ʻano thalassemia, thalassemia liʻiliʻi, thalassemia intermedia, a me thalassemia nui . I ke kūlana lawe, ʻaʻohe ou hōʻailona. A i ʻole he anemia liʻiliʻi wale nō paha kou. ʻO ka Thalassemia nui ke ʻano koʻikoʻi loa e pono ai ka mālama ʻana.

ʻElua ʻano nui, ma muli o ke kīnā genetic i loko o nā kaulahao alpha a i ʻole nā ​​​​kauhao beta.

1. Alpha Thalassemia

Hana ʻia kēia kūlana ke loaʻa kahi kīnā i hoʻokahi a ʻoi aku paha o nā genes 4 e hana ana i nā kaulahao alpha globin. ʻO ke koʻikoʻi o nā hōʻailona e pili ana i ka helu o nā genes kīnā. E nānā kākou i kēia ma ke ala maʻalahi e hoʻomaopopo ai.

2. Beta Thalassemia

Hana ʻia kēia kūlana ke loaʻa kahi kīnā i kekahi a i ʻole nā ​​​​​​gene ʻelua e hana ana i nā kaulahao beta globin (hoʻokahi mai ka makuahine, hoʻokahi mai ka makuakāne).

• Hoʻokahi gene kīnā: Ua kapa ʻia kēia kūlana he beta thalassemia minor a i ʻole ke kūlana lawe. He palupalu loa nā hōʻailona.
• ʻElua mau genes kīnā: Hiki ke loli nā hōʻailona mai ka waena a i ka koʻikoʻi.
• Ua kapa ʻia ke kūlana waena ʻo Thalassemia intermedia .
• ʻO ke ʻano koʻikoʻi loa i kapa ʻia ʻo beta thalassemia major a i ʻole Cooley's anemia . Pono kēia poʻe i ka mālama lapaʻau mau.

He aha nā hōʻailona o ka thalassemia?

ʻO nā hōʻailona āu e ʻike ai e hilinaʻi wale ʻia i ke ʻano o ka thalassemia āu e loaʻa ai a me kona koʻikoʻi.

ʻAʻohe hōʻailona a i ʻole nā ​​hōʻailona palupalu loa

Inā ʻaʻohe ou hōʻailona o kekahi o nā ʻehā alpha genes, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona. Inā ʻelua mau alpha genes a i ʻole hoʻokahi beta gene he hemahema, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona. A i ʻole he mau hōʻailona anemia liʻiliʻi wale nō paha kāu, e like me ka luhi mau.

Nā hōʻailona waena (Thalassemia Intermedia)

Hiki i kēia poʻe ke ʻike i nā hōʻailona ʻē aʻe ma waho aʻe o ka anemia liʻiliʻi.

• ʻAʻole ulu pono: ʻAʻole piʻi ka kiʻekiʻe a me ke kaumaha o ke keiki e like me ko lākou mau makahiki.
• Ka lohi o ka wā ʻōpiopio.
• Nā ʻano ʻino o ka iwi:Nā kūlana e like me ka osteoporosis.
• ʻO ka spleen i hoʻonui ʻia: ʻO ka spleen kahi ʻōkana aia ma ka ʻaoʻao hema o ko mākou ʻōpū e kōkua ana i ka hakakā ʻana i nā maʻi.

Nā hōʻailona koʻikoʻi (Thalassemia Major)

ʻIke ʻia nā hōʻailona koʻikoʻi i nā kūlana e like me nā hemahema ʻekolu alpha gene (maʻi Hemoglobin H) a me ka beta thalassemia major (Cooley's anemia). Hoʻomaka pinepine kēia mau hōʻailona e ʻike ʻia ma mua o ka piha ʻana o nā makahiki ʻelua o ke keiki .

Ma waho aʻe o nā hōʻailona i luna nei, hiki ke ʻike ʻia nā hōʻailona:

• ʻAʻohe ʻono o ka meaʻai.
• ʻIli keʻokeʻo a melemele paha (jaundice).
• Mimi ʻeleʻele (e like me ke kala tī).
• Nā ʻano ʻē o nā iwi maka (ma muli o ka ulu nui ʻana o nā iwi poʻo).

Pehea e ʻike ai i kēia maʻi?

Hoʻomaopopo pinepine ʻia ka thalassemia waena a koʻikoʻi i ka wā kamaliʻi, ʻoiai e hoʻomaka ana nā hōʻailona e ʻike ʻia i loko o nā makahiki ʻelua mua o ke ola o kahi keiki. Hiki i kāu kauka ke kauoha i nā hoʻāʻo koko like ʻole e hōʻoia i ka hōʻailona.

• Helu Koko Piha (CBC): Ke ana kēia i ka nui o ka hemoglobin i loko o ke koko, ka helu o nā hunaola koko ʻulaʻula, a me ko lākou nui. ʻOi aku ka liʻiliʻi o nā hunaola koko ʻulaʻula olakino o ka poʻe me ka thalassemia a ʻoi aku ka liʻiliʻi o ka hemoglobin. Hiki ke liʻiliʻi nā hunaola koko ʻulaʻula ma mua o ka maʻamau.
• Helu Reticulocyte: Ke ana kēia i ka helu o nā hunaola koko ʻulaʻula i hana hou ʻia. Hiki i kēia ke hāʻawi iā ʻoe i kahi manaʻo inā lawa ka hana ʻana o kou iwi iwi i nā hunaola koko ʻulaʻula.
• Nā haʻawina hao: Kōkua kēia hoʻāʻo e ʻike pololei inā ua hana ʻia kou anemia e ka hemahema hao a i ʻole thalassemia.
• Hemoglobin electrophoresis: He hoʻāʻo koʻikoʻi kēia no ka ʻike ʻana i ka beta thalassemia.
• Ka hoʻāʻo ʻana i ka genetic: Hoʻohana ʻia kēia hoʻāʻo e ʻike i ka alpha thalassemia.

He aha nā lāʻau lapaʻau no ka thalassemia?

ʻO nā lāʻau lapaʻau maʻamau no nā maʻi koʻikoʻi e like me ka thalassemia major he hoʻoili koko a me ka lāʻau lapaʻau wehe hao.

• Hoʻoili koko: I ka ʻōlelo maʻalahi, he hoʻoili koko waho kēia. Hāʻawi ʻia ke koko me nā hunaola koko ʻulaʻula olakino i loko o ke kino ma o kahi aa koko. Hoʻihoʻi kēia i nā hunaola koko ʻulaʻula olakino a me nā pae hemoglobin. I nā kūlana thalassemia koʻikoʻi (e.g., beta thalassemia major), pono paha ke hoʻoili koko i nā manawa a pau, e like me kēlā me kēia 2-4 pule .
• Ka Lapaʻau Chelation Hao:ʻO kekahi o nā hopena ʻaoʻao nui o ka hāʻawi pinepine ʻana i ke koko, ʻo ia ka hoʻonui pono ʻole ʻia o nā pae hao i loko o ke kino. Kāhea mākou i kēia he hao nui . Hiki i kēia hao keu ke hōʻiliʻili i loko o nā ʻōpū koʻikoʻi e like me ko mākou puʻuwai a me ke akepaʻa a hōʻino iā lākou. No laila, hāʻawi ʻia ka lāʻau lapaʻau i ka poʻe e loaʻa pinepine ana i ke koko (nā pila a i ʻole nā ​​inikini) e wehe ana i kēia hao keu mai ke kino.
• Nā Mea Hoʻohui Waikawa Folic: Hāʻawi ʻia ka waikawa Folic e kōkua i ke kino e hana i nā hunaola koko olakino.
• Ka Hoʻoili ʻana o ka Iwi Moa a me nā Kelepona Kumu: ʻO kēia wale nō ka lāʻau lapaʻau e hiki ke ho'ōla loa i ka thalassemia. Eia naʻe, no kēia, pono ka mea maʻi i kahi mea hāʻawi olakino kūpono loa. ʻO ka hapa pinepine, he kaikunāne a he kaikuahine paha. He lāʻau lapaʻau pilikia a paʻakikī loa kēia.
• Luspatercept: He lāʻau pale kēia i hāʻawi ʻia i kēlā me kēia ʻekolu pule. Hana ia ma ke kōkua ʻana i ke kino e hana i nā hunaola koko ʻulaʻula hou aʻe.

Pehea ke ʻano o ke ola me kēia maʻi? Hiki ke pale ʻia?

Inā loaʻa iā ʻoe ka thalassemia minor, hiki iā ʻoe ke manaʻo i kahi ola maʻamau. Inā he kaulike a koʻikoʻi paha kou kūlana, inā ʻoe e hahai pono i ka hoʻolālā lapaʻau i kuhikuhi ʻia (nā hoʻoili koko a me ka lāʻau wehe hao), loaʻa iā ʻoe kahi manawa kūpono e ola ai i kahi ola lōʻihi.

He maʻi hoʻoilina kēia, no laila ʻaʻole hiki iā mākou ke pale aku. Eia nō naʻe, hiki i ka hoʻāʻo hoʻoilina ke kōkua iā ʻoe e ʻike inā loaʻa iā ʻoe a i ʻole kāu hoa ka gene thalassemia. He mea nui loa ka ʻike ʻana i kēia ʻike no nā kāne e hoʻolālā ana e loaʻa kahi keiki. E kamaʻilio me kāu kauka no ka ʻōlelo aʻoaʻo hou aku e pili ana i kēia.

ʻO ka mea hope loa, he maʻi hiki ke hoʻokele ʻia ka thalassemia. ʻO ke ʻano o ka lāʻau lapaʻau āu e pono ai a me ka pinepine o kou pono ʻana e pili ana i ke koʻikoʻi o kou kūlana. He mea nui e kamaʻilio ākea me kāu kauka e pili ana i kou kūlana a me kāu hoʻolālā mālama lōʻihi.