ʻO ka anemia sickle cell: he aha ia, nā ʻōuli, nā kumu a me nā lāʻau

Anter

• Nā hōʻailona nui
• Pehea e hōʻoia ai i ke kuhi
• ʻO nā kumu kūpono o ka anemia maʻi maʻi
• Pehea e hana ai i ka lāʻau
• Nā pilikia i hiki

ʻO ka anemia sickle cell kahi maʻi i hōʻike ʻia e ka loli ʻana i ke kinona o nā hunaola ʻulaʻula, a he ʻano like ko lākou me ka pahi ʻole a hapa mahina paha. Ma muli o kēia loli, hiki i nā hunaola ʻulaʻula ke lilo i mea hiki ʻole ke hāpai i ka oxygen, a ʻoi aku ka hoʻonui ʻana i ka makaʻala o ke kahakaha ʻana o ke kīʻaha koko ma muli o ke ʻano i hoʻololi ʻia, kahi e hiki ai i ka ʻeha ākea, nāwaliwali a me nā manaʻo ʻole.

Hiki ke kāohi ʻia nā ʻōuli o kēia ʻano anemia me ka hoʻohana ʻana i nā lāʻau i pono e lawe ʻia i loko o ke ola e hoʻoliʻiliʻi i ka pilikia o nā pilikia, akā hiki ke ola i ka hoʻōla ʻana o nā cell hematopoietic.

Nā hōʻailona nui

Ma waho aʻe o nā hōʻailona maʻamau o nā ʻano anemia ʻē aʻe, e like me ka māluhiluhi, pallor a me ka hiamoe, hiki i ka anemia maʻi maʻi maʻi ke kumu i nā ʻōuli ʻano ʻē aʻe, e like me:

• ʻEha i nā iwi a me nā hono no ka mea, hōʻea ka oxygen ma nā mea liʻiliʻi, ka hapanui ma nā kihi, e like me nā lima a me nā wāwae;
• Nā pilikia o ka ʻeha i loko o ka ʻōpū, ka umauma a me ka lumbar, ma muli o ka make ʻana o nā puʻupuʻu iwi, a pili pū paha me ka fiva, ka luaʻi a me ka mimi ʻeleʻele a koko paha;
• Nā maʻi pinepineno ka mea hiki i nā hunaola ʻulaʻula ke hoʻopōʻino i ka lepe, e kōkua ai i ke kaua ʻana i nā maʻi;
• ​ʻO ka ulu ʻana o ka ulu a lohi ka wā ʻōpiono ka mea hāʻawi nā hunaola ʻulaʻula mai ka maʻi anemia i ka liʻiliʻi i ka oxygen a me nā mea momona no ke kino e ulu a ulu;
• Maka maka a ʻili ma muli o ka ʻoiaʻiʻo o nā ʻulaʻula koko "make" me ka wikiwiki a, no laila, hōʻiliʻili ka pigment bilirubin i loko o ke kino e hana ana i ke kala melemele i ka ʻili a me nā maka.

Hōʻike pinepine ʻia kēia mau ʻōuli ma hope o 4 mau makahiki o kona mau makahiki, akā hana pinepine ʻia ka hōʻailona i nā lā mua o ke ola, ʻoiai ke hōʻike ka wāwae o ka pēpē i ka wāwae o ka pēpē. E ʻike i ka ʻike hou aʻe e pili ana i ka hoʻāʻo prick heel a me nā maʻi e ʻike ai.

Pehea e hōʻoia ai i ke kuhi

Hana pinepine ʻia ke aniani o ka anemia maʻi maʻi ma ke hoʻāʻo ʻana i ka wāwae o ka pēpē i nā lā mua o ke ola o ka pēpē. Hiki i kēia hoʻāʻo ke hana i kahi hōʻike i kapa ʻia ʻo hemoglobin electrophoresis, kahi e nānā ai i ke kū ʻana o hemoglobin S a me kona kūpaʻa. ʻO kēia no ka mea inā ʻike ʻia he hoʻokahi wale nō ʻāpana S kēlā kanaka, ʻo ia hoʻi, hemoglobin o ke ʻano AS, ʻo ia hoʻi ka ʻōlelo ʻana he lawe ʻo ia i ka gen anemia cell sickle, ke ʻano ʻia ma ke ʻano o ka cell sickle. I kēlā mau hihia, ʻaʻole hiki i ke kanaka ke hōʻike i nā ʻōuli, akā pono e ukali ʻia ma o nā hoʻokolohua hoʻokolohua maʻamau.

Ke kuhi ʻia ke kanaka me ka HbSS, ʻo ia hoʻi he maʻi ko ka maʻi maʻi maʻi anemia a pono e mālama ʻia e like me ka ʻōlelo aʻoaʻo a ka lāʻau.

Ma waho aʻe o ka hemoglobin electrophoresis, hiki ke hana ʻia ke ʻano o kēia ʻano anemia ma ke ana ʻana o ka bilirubin e pili pū ana me ka helu o ke koko i ka poʻe i hana ʻole i ka hoʻāʻo prick kuʻekuʻe wāwae i ka wā hānau, a me ke kū ʻana o nā aʻa koko ʻulaʻula i manaʻo ʻia. ke kūʻana o nā retikuleta, nā kiko basophilic a me nā waiwai hemoglobin ma lalo o ke kumukūʻai maʻamau, ma waena o 6 a me 9.5 g / dL.

ʻO nā kumu kūpono o ka anemia maʻi maʻi

ʻO nā kumu o ka maʻi anemia cell sickle, ʻo ia hoʻi, hānau ʻia me ke keiki a hala ʻia mai ka makuakāne a ke keiki.

ʻO ke kumu kēia i kēlā me kēia manawa ke ʻike ʻia kekahi kanaka me ka maʻi, loaʻa iā ia ka ʻenemi SS (a i ʻole hemoglobin SS) i hoʻoilina ʻia mai kona makuahine a me kona makuakāne. ʻOiai paha ke olakino nā mākua, inā loaʻa ka makuakāne a me ka makuahine i ka gen AS (a i ʻole hemoglobin AS), kahi hōʻailona o ka mea lawe i ka maʻi, i kapa ʻia hoʻi ke ʻano o ka cell sickle, aia kahi manawa e loaʻa ai ka maʻi i ke keiki ( 25% manawa) a i ʻole he lawe (50% manawa) o ka maʻi.

Pehea e hana ai i ka lāʻau

Hana ʻia ka lāʻau no ka maʻi anemia o ka maʻi sickle me ka hoʻohana ʻana o nā lāʻau a i kekahi mau manawa e pono ai ke transfusion.

ʻO nā lāʻau i hoʻohana nui ʻia ʻo Penicillin i nā keiki mai 2 mau mahina a 5 mau makahiki, e pale ai i ka hoʻomaka ʻana o nā pilikia e like me ka pulumonia, no ka laʻana. Hoʻohui ʻia, hiki ke hoʻohana ʻia i nā lāʻau analgesic a me nā anti-inflammatory e hōʻoluʻolu i ka ʻeha i ka wā o kahi pilikia a hoʻohana pū i kahi huna huna oxygen e hoʻonui i ka nui o ka oxygen i loko o ke koko a kōkua i ka hanu.

Pono e mālama ʻia ka mālama ʻana i ka anemia o ka maʻi maʻi no ke ola no ka mea he mau maʻi pinepine ko kēia mau maʻi. Hiki i ke kuni ke kuhikuhi i ka maʻi, no laila inā he kuni ke kanaka me ka maʻi anemia cell, pono lākou e hele koke i ke kauka no ka mea hiki iā lākou ke ulu i ka septicemia i loko o 24 mau hola wale nō, a he mea make ia. ʻAʻole pono e hoʻohana ʻia nā lāʻau hoʻohaʻahaʻa fiva me ka ʻike ʻole o ka lāʻau.

Eia kekahi, ʻo ka hoʻoneʻe ʻana o ka iwi iwi kekahi ʻano lapaʻau, i hōʻike ʻia no kekahi mau hihia koʻikoʻi a koho ʻia e ke kauka, i hiki mai e hōʻola i ka maʻi, eia naʻe ke hōʻike nei i kekahi mau pilikia, e like me ka hoʻohana ʻana i nā lāʻau e hōʻemi i ka pale. E ʻike pehea e hana ʻia ai ka hoʻoneʻe ʻana o ka iwi iwi a me nā pilikia kūpono.

Nā pilikia i hiki

ʻO nā pilikia e hiki ke hoʻopili i nā mea maʻi me ka anemia maʻi sickle hiki ke:

• ʻO ka hoʻowahāwahā o nā hono o nā lima a me nā wāwae e haʻalele iā lākou a pehu a ʻeha loa a ʻinoʻino hoʻi;
• Hoʻonui i ka makaʻu o nā maʻi ma muli o ke komo ʻana o ka palapū, ʻaʻole ia e kānana pono i ke koko, no laila e ʻae ana i ke kū ʻana o nā maʻi a me nā koʻohune i loko o ke kino;
• Hoʻopilikia ka hakuʻala, me ka hoʻonui pinepine ʻana o ka mimi, he mea maʻamau ka ʻeleʻele o ka mimi a me ke keiki e pee i ka moena a hiki i ka wā ʻōpio;
• ʻEha ma nā wāwae he paʻakikī e hoʻōla a koi i ka ʻaʻahu ʻelua manawa o ka lā;
• Hoʻopilikia ke akepaʻa e hōʻike iā ia iho ma o nā ʻōuli e like me ke kala melemele i nā maka a me ka ʻili, akā ʻaʻole ia he hepatitis;
• Pōhaku Gall;
• Hoʻoemi ka hihiʻo, scarring, kīnā a kīkaha i nā maka, i kekahi mau hihia hiki ke alakaʻi i ka makapō;
• ʻO ka hahau, ma muli o ka paʻakikī o ke koko i ka hoʻuwai ʻana i ka lolo;
• Hewa ka puʻuwai, me ka cardiomegaly, infarctions a me ka ʻōhumu o ka puʻuwai;
• Priapism, ʻo ia ke kūkulu ʻeha, ʻino a hoʻomau hoʻi i kū ʻole ʻia me ka makemake moekolohe a i ʻole ke ala ʻana, maʻamau i nā kāne ʻōpio.

Hiki ke hoʻololi ʻia ke koko i ʻāpana o ka mālama ʻana, e hoʻonui i ka nui o nā hunaola ʻulaʻula i ke kaapuni ʻana, a ʻo ka transplantation ʻana wale nō o nā cell hematopoietic e hāʻawi ana i ka mea hiki ke hoʻōla ʻia no ka maʻi anemia o ka cell sickle, akā me nā hōʻike liʻiliʻi ma muli o nā pilikia pili pū ke kaʻina hana.