Ua ʻike anei ʻoe i ka Sickle Cell Anemia? E kamaʻilio kākou e pili ana!

Ua lohe paha ʻoe i ka inoa ʻo 'Sickle Cell Anemia'? Malia paha he ʻano ʻē iki kēia inoa, a he mea weliweli iki paha. Akā, he ʻano hoʻoilina ia e pili ana i ko kākou koko. I ka ʻōlelo maʻalahi, ʻo ka mea e hana ʻia ana i loko o kēia, ʻo ia ka loli ʻana o ko kākou mau hunaola koko ʻulaʻula, ʻo ia hoʻi nā hunaola koko ʻulaʻula i loko o ke koko. E like me ka pahi ʻoki (ʻo ia ke kumu i kapa ʻia ai ʻo 'sickle'). Hiki i kēia loli ke hana i nā pilikia olakino like ʻole. No laila, e kamaʻilio hou aku kākou e pili ana i kēia i kēia lā?

He aha ka Sickle Cell Anemia? He aha ka mea e hana nei?

ʻO ka anemia cell sickle ka mea a nā kauka e kapa nei ʻo ia ke ʻano koʻikoʻi loa o ka hui nui i kapa ʻia ʻo 'Sickle Cell Disease'. He maʻi koko hānau kēia, ʻo ia hoʻi, he maʻi koko hoʻoilina . ʻO ke kumu nui o kēia he hoʻololi ʻana o ka genetics. Ma muli o kēia hoʻololi ʻana o ka genetics, ʻaʻole poepoe nā hunaola koko ʻulaʻula i hana ʻia i loko o ko mākou kino, akā ua kūlou a kīkoʻo ʻia e like me ka sickle (ma ke ʻano o ka leka C). Kāhea pū nā kauka i kēia mau "nā hunaola sickle".

E nānā, he poepoe a palupalu ko kākou mau hunaola koko ʻulaʻula maʻamau, no laila hiki iā lākou ke hele maʻalahi ma o ko kākou mau kīʻaha koko palupalu a lawe i ka oxygen i kēlā me kēia ʻāpana o ke kino, kēlā me kēia ʻōkana, a me kēlā me kēia ʻiʻo. Akā, he ʻoʻoleʻa a pipili kēia mau hunaola koko ʻulaʻula ʻano sickle. No laila, ʻaʻole lākou e hele maʻalahi ma o nā kīʻaha koko, akā e paʻa pinepine lākou ma nā wahi. E like me ke kaʻa ma ke alanui. ʻO ka mea ʻē aʻe, e haki ana kēia mau hunaola 'sickle' a make wikiwiki ma mua o nā hunaola koko ʻulaʻula maʻamau. Ma muli o kēia mau mea a pau, emi ka helu o nā hunaola koko ʻulaʻula olakino i loko o kou kino, e hoʻoulu ai i ka anemia koʻikoʻi. ʻO ia hoʻi, ka nele o ke koko.

I ka wā ma mua, he haʻahaʻa loa ka manawa e ola ai nā pēpē i hānau ʻia me kēia maʻi a hiki i ka wā makua. Eia nō naʻe, me ka holomua o ka lāʻau lapaʻau, ka ʻike mua ʻana, a me nā lāʻau lapaʻau hou , ua loli nui kēia. Nui ka poʻe i kēia manawa e ola ana a hiki i 50 mau makahiki a ʻoi paha.

Pehea ka maʻamau o kēia maʻi ma Sri Lanka? ʻO wai ka mea i ʻoi aku ka paʻakikī o ka loaʻa ʻana?

He mea paʻakikī ke loaʻa nā helu helu e pili ana i kēia maʻi ma Sri Lanka. Akā ke nānā ʻoe i ke ao nei, he mea maʻamau kēia maʻi i nā poʻe o ʻApelika, ʻEulopa Hema, Hikina Waena a i ʻole nā ​​​​moku ʻĀsia. ʻO ia hoʻi, aia kekahi pilina hoʻoilina.

He aha nā hōʻailona o ka anemia cell sickle?

Hiki ke ʻokoʻa nā hōʻailona o kēia maʻi mai kēlā me kēia kanaka, akā aia kekahi mau hōʻailona maʻamau:

• Luhi mai ka anemia: Ke hiki mai kēia ʻano i nā pēpē ʻōpio, hiki iā lākou ke uē pinepine, maʻi kino, a hōʻole e hānai i ka waiū.
• Nā maʻi pinepine:Hoʻopōʻino kēia maʻi i ko kākou ʻōpū. He ʻāpana koʻikoʻi ka ʻōpū o ko kākou ʻōnaehana pale. No laila, i ka wā e nāwaliwali ai, ʻoi aku ka nui o ka loaʻa ʻana o nā maʻi a me nā maʻi. Eia nō naʻe, mahalo i nā antibiotics a me nā lāʻau pale i loaʻa i kēia manawa, ua hoʻemi nui ʻia kēia pilikia.
• ʻEha: ʻO kēia kekahi o nā hōʻailona maʻamau a pōʻino hoʻi o kēia maʻi. ʻAʻole lawa ka oxygen i nā ʻiʻo o ke kino, kahi e hōʻino ai i nā ʻiʻo. ʻO kēia hōʻino ke kumu o ka ʻeha. ʻO nā wahi maʻamau o ka ʻeha ʻo ia nā lima, nā wāwae, ka umauma, a me ke kua. I kekahi manawa hiki ke hoʻomaka me he ʻeha iki a piʻi mālie, a i ʻole hiki ke lilo koke i ʻeha hiki ʻole ke hoʻomanawanui.
• ʻO ka pehu ʻeha ma nā lima a me nā wāwae: ʻO kēia kekahi o nā hōʻailona mua o ka anemia cell sickle i loko o kahi pēpē. Hiki mai ka pehu ʻana no ka mea ua paʻa nā hunaola koko ʻulaʻula e like me ka sickle i loko o nā kīʻaha koko palupalu i loko o nā lima a me nā wāwae o ka pēpē, e ālai ana i ke kahe ʻana o ke koko.
• Nā maka melemele a me ka ʻili mai ka jaundice: ʻO ko mākou akepaʻa ke kuleana no ka kānana ʻana i nā hunaola koko ʻulaʻula. I loko o ka anemia cell sickle, kahi mea i kapa ʻia ʻo bilirubin, kahi i hoʻokuʻu ʻia mai nā hunaola sickle e make koke ana, e hōʻiliʻili ana i loko o ke kino, e hana ana i ka jaundice.

Hoʻomaka pinepine kēia mau hōʻailona e ʻike ʻia i ka wā he 6 a 9 mau mahina paha ka pēpē . I ka hala ʻana o ka manawa, i ka piʻi ʻana o ka nui o nā hunaola ʻino (malignant) i loko o ke kino, e loli paha nā hōʻailona a lilo i mea ʻino.

No ke aha e ulu ai ka anemia cell sickle? He aha ke kumu?

Hoʻokumu ʻia ka anemia cell sickle e nā hoʻololi genetic i loko o kā mākou mau genes. ʻO ke kikoʻī, aia kahi gene i kapa ʻia ʻo ka gene HBB. Kuhikuhi kēia gene iā mākou e hana i kahi protein i kapa ʻia ʻo beta-globin, ʻo ia kekahi ʻāpana o ko mākou hemoglobin. ʻO ka hemoglobin, e like me kou ʻike, ka mea nui loa e kōkua i kā mākou mau hunaola koko ʻulaʻula e lawe i ka oxygen.

No laila, no ka loaʻa ʻana o ka anemia cell sickle, pono ʻoe e hoʻoilina i ka gene i hoʻololi like ʻia mai kou makuahine a me kou makuakāne . I kekahi manawa, inā hoʻoilina ʻoe i ʻelua mau gene i hoʻololi ʻia mai nā mākua ʻelua, hiki iā ʻoe ke hoʻomohala i nā ʻano maʻi cell sickle ʻē aʻe, ʻoi aku ka liʻiliʻi o ke koʻikoʻi. Kāhea nā kauka i kēia mau 'variant genetic mutations'.

He aha nā pilikia e hiki ke hana ma muli o kēia ʻano?

Hiki i ka anemia cell sickle ke hoʻoulu i nā pilikia koʻikoʻi, i kekahi manawa e hoʻoweliweli i ke ola . No ka laʻana, ʻo ka poʻe me kēia maʻi e koi pinepine i ka mālama olakino ulia pōpilikia a me ka hoʻokipa ʻana i ka haukapila, ʻoi aku hoʻi no nā kūlana i kapa ʻia ʻo acute chest syndrome (ACS) a me ka vaso-occlusive crisis (VOC).

Aia kekahi mau pilikia ʻē aʻe:

• Maʻi puʻupaʻa mau loa: Hiki ke hana ʻia kēia ʻano ke loaʻa ʻole i nā puʻupaʻa ka lawa o ka oxygen, e hōʻino ana i nā ʻiʻo.
• Nā retina i hoʻokaʻawale ʻia: Hiki ke hana kēia inā ālai nā cell sickle i nā kīʻaha koko i loko o ka maka.
• Priapism (kū ʻeha): He kūlana i hoʻokumu ʻia e ka pale ʻana o nā kīʻaha koko i loko o ka penis.
• ʻO ka hoʻokaʻawale ʻana o ka splenic: Inā paʻa nā cell sickle i loko o ka spleen a ālai i ke kahe ʻana o ke koko, hiki i kēia ke hana i ka anemia koʻikoʻi a koke.
• Hahau: ʻO kēlā me kēia kanaka me ka anemia cell sickle, ʻoiai nā keiki ʻōpio ( stroke pediatric), ua pilikia.

He aha ka Acute Chest Syndrome (ACS)?

ʻO kēia ka pilikia maʻamau o ka anemia cell sickle. ʻO ia kekahi ke kumu nui o ka make a me ke kumu lua o ka hoʻokipa ʻia ʻana i ka haukapila. Hana ʻia kēia i ka wā e hui pū ai nā cell sickle a ālai i nā kīʻaha koko i loko o nā māmā. ʻO nā hōʻailona:

• ʻEha koke ka umauma.
• Kuke.
• Kuni.
• Paʻakikī ka hanu ʻana.

He aha ka Vaso-occlusive Crisis (VOC)?

ʻO kēia ka manawa e ālai ai nā pūnaewele sickle i nā kīʻaha koko. Ua kapa pū nā kauka i kēia he "pilikia ʻeha koʻikoʻi." I ka VOC, hiki ke loaʻa ka ʻeha hiki ʻole ke hoʻomanawanui ma nā lima, nā wāwae, ke kua i lalo, a me ka ʻōpū.

I kekahi manawa, kapa nā kauka i nā VOC he "maʻi ʻike ʻole ʻia." No ka mea, i ka hapanui o nā hihia, ʻo ka hōʻailona wale nō ka ʻeha. ʻAʻohe hōʻailona o kahi ʻeha a i ʻole kekahi maʻi ʻē aʻe. He ʻeha wale nō ia.

ʻO ka lāʻau lapaʻau nui no kēia ʻeha nui, ʻo ia nā mea hoʻomaha ʻeha opioid. Eia nō naʻe, ua hōʻike kekahi mau noiʻi e hiki i kēia koi opioid ke alakaʻi i ka noho kaʻawale ʻana i ka poʻe me ka anemia cell sickle, hiki ke alakaʻi i ke kaumaha a me ka hopohopo. He pilikia ʻē aʻe kēia e kū ai i ka wā e noho ai me kēia maʻi.

Pehea e ʻike ʻia ai kēia maʻi? (Diagnosis)

Hoʻomaopopo nā kauka i ka anemia cell sickle ma ka nānā mua ʻana iā ʻoe. Hiki iā lākou ke hoʻopā i kou spleen a i ʻole ke akepaʻa. E nīnau lākou e pili ana i kāu mau hōʻailona, ​​ʻoiai ka ʻeha ma kou mau lima a me kou mau wāwae, a e pili ana i kou ʻōpū. E nīnau pū lākou e pili ana i kou mau maʻi i hala a inā ua loaʻa iā ʻoe kekahi mau maʻi. A laila hiki iā lākou ke hana i nā hoʻokolohua e like me:

• Helu Koko Piha (CBC): Pili kēia i kekahi mau hoʻokolohua e nānā pono ana i kāu mau hunaola koko ʻulaʻula.
• Hemoglobin electrophoresis: Kāhea ʻia hoʻi he chromatography wai hana kiʻekiʻe, nānā kēia hoʻāʻo i kou hemoglobin e ʻike a ana i ke ʻano maʻamau o ka hemoglobin e hoʻoulu ai i ka anemia cell sickle.
• Nā hoʻokolohua genetic: Hiki i kāu kauka ke kauoha i kēia mau hoʻokolohua e ʻike inā loaʻa iā ʻoe nā loli genetic e hoʻoulu ai i ka anemia cell sickle.

He aha nā lāʻau lapaʻau no kēia? Hiki ke ho'ōla ʻia?

ʻO ka lāʻau no ka anemia cell sickle e pili ana i kāu mau hōʻailona a me kou olakino holoʻokoʻa. No ka laʻana, inā loaʻa iā ʻoe nā pilikia koʻikoʻi e like me ka acute chest syndrome, nā hanana pinepine o ka ʻeha nui, a inā loaʻa iā ʻoe kahi maʻi e like me ka paralysis, hiki i kāu kauka ke ʻōlelo aku i kahi allogeneic stem cell transplant. ʻO kēia wale nō ke ala e hoʻōla loa ai i ka anemia cell sickle .

ʻO nā lāʻau lapaʻau ʻē aʻe:

• Nā hoʻoili koko.
• Nā lāʻau antibiotic no nā maʻi.
• Nā lāʻau lapaʻau e hōʻoluʻolu i nā hōʻailona kikoʻī. ʻO kekahi o lākou:
• Hydroxyurea: He lāʻau lapaʻau anticancer kēia. Hoʻohana ʻia ia i nā pēpē ma waena o 6-9 mahina, nā keiki, a me nā pākeke. Hiki iā ia ke hōʻemi i ka nui o nā pilikia koʻikoʻi a hōʻemi i nā hōʻailona o ka anemia.
• Voxelorator: Ke kāohi nei kēia i nā hunaola koko ʻulaʻula mai ka lilo ʻana i mau hunaola sickle. Hāʻawi ʻia ia i nā keiki ma luna o 4 mau makahiki.
• L-glutamine therapy: ʻO kēia lāʻau lapaʻau, i hāʻawi ʻia i nā keiki ma luna o 5 mau makahiki a me nā pākeke, e hoʻōki i nā cell sickle mai ka lilo ʻana i mea ʻē aʻe.
• Crizanlizumab-tmca: Hoʻemi kēia i ke alapine (frequency) o nā pilikia ʻeha VOC/ʻeha koʻikoʻi. Hāʻawi ʻia ia i nā poʻe ma luna o 16 mau makahiki.

Pehea lā koʻu ola inā loaʻa iaʻu ka anemia cell sickle?

No nā kānaka he nui, he maʻi mau ka anemia cell sickle. ʻO ia hoʻi, pono ʻoe i ka mālama olakino a me ke kākoʻo i kou ola a pau. Hiki i nā hōʻailona ke akahai no kekahi, kūpono no kekahi, a koʻikoʻi e hoʻoweliweli i ke ola no kekahi. He ʻokoʻa ke kūlana o kēlā me kēia kanaka. No laila, e kamaʻilio me kāu kauka e pili ana i ka mea e manaʻo ai. ʻIke maikaʻi ʻo ia i kou olakino a pehea e hoʻopilikia ai ka maʻi iā ʻoe.

Ke ʻoi aku ka ʻino o kēia kūlana i kou wā ʻelemakule?

ʻAe, i ko kākou ʻelemakule ʻana, hiki ke kū mai nā pilikia olakino ʻē aʻe, ʻoi aku paha ka koʻikoʻi ma muli o ka lawa ʻole o ka lawe ʻana o ka oxygen i nā ʻōpū a me nā ʻiʻo. ʻO ka poʻe me ka anemia cell sickle he nui ka pilikia o ka maʻi lolo, ka māmā, nā puʻupaʻa, ka spleen, a me ka hōʻino ʻana o ke ake.

He aha ka awelika o ke ola o kekahi me kēia maʻi?

Ua ʻoi aku ka maikaʻi o nā mea i kēia manawa ma mua o ka wā ma mua. Me ka ʻike mua ʻana a me ka mālama ʻana e hōʻemi i nā pilikia , hiki i ka poʻe me ka anemia cell sickle ke ola a hiki i ko lākou mau makahiki 50. ʻOi aku ka lōʻihi o ke ola o kekahi poʻe. Inā he mau nīnau kāu e pili ana i kēia, e kamaʻilio me kāu kauka. Hiki iā ia ke wehewehe iā ʻoe.

Aia kekahi ala e pale aku ai i ka ulu ʻana o kēia maʻi?

He maʻi hoʻoilina ka anemia cell sickle, no laila ʻaʻole hiki ke pale ʻia.Eia nō naʻe, hiki iā ʻoe ke loaʻa i kahi hoʻāʻo koko e ʻike ai inā loaʻa iā ʻoe ka mutation genetic e hoʻoulu ai i ka anemia cell sickle. Ma nā ʻāina holomua he nui, hoʻāʻo ʻia kēlā me kēia pēpē no ka maʻi i ka wā hānau. ʻO ka mea nui loa, ʻo ia ka ʻike mua ʻana i ka maʻi a hoʻomaka i ka mālama ʻana .

Pehea e hiki ai iaʻu ke ola maikaʻi me kēia maʻi? (Mālama ponoʻī)

Ua ʻae nā lāʻau lapaʻau hou i nā poʻe me ka anemia cell sickle e ola i nā ola maikaʻi loa. Inā loaʻa iā ʻoe ka maʻi, e noʻonoʻo i kēia mau mea:

• E ʻimi i ka mālama lapaʻau kūikawā: He mea nui e ʻimi i ka lāʻau lapaʻau mai kahi hui o nā hematologists.
• E hana i nā hoʻokolohua lapaʻau maʻamau: ʻO ka mālama ʻana i ka pilina maikaʻi me kāu kauka ʻohana a me ka hele ʻana no nā hoʻokolohua maʻamau e hiki ke kōkua i ka pale ʻana i nā pilikia koʻikoʻi.
• E noʻonoʻo i ka hoʻokele ʻeha: ʻO ka ʻeha kahi ʻāpana maʻamau o kēia maʻi. E ʻimi i ka ʻōlelo aʻo mai kahi loea hoʻokele ʻeha.
• E kiʻi i ke kākoʻo olakino noʻonoʻo: I kekahi manawa hiki i kēia maʻi ke hoʻoulu i nā manaʻo o ka noho kaʻawale, ke kaumaha, a me ka hopohopo. Inā manaʻo ʻoe pēlā, e kamaʻilio me ke kauka.
• E pale iā ʻoe iho mai nā maʻi: E kamaʻilio me kāu kauka e pili ana i ka loaʻa ʻana o ka lāʻau pale. E hana i nā hana e pale aku iā ʻoe iho mai nā maʻi.
• E makaʻala i kou wahi noho: He mea nui loa ia no ka poʻe me ka anemia cell sickle e mālama i ka mahana kino kaulike. E noho mamao mai nā wahi wela loa a me ke anu.
• E nānā i nā hoʻokolohua lapaʻau: Ke hoʻāʻo mau nei nā kauka a me nā mea noiʻi i nā lāʻau lapaʻau hou. Loaʻa paha iā ʻoe ka manawa kūpono e komo i hoʻokahi.

Āhea wau e ʻike ai i ke kauka?

Inā manaʻo ʻoe e ʻoi aku ka ʻino o kāu mau hōʻailona, ​​a inā paha e loaʻa iā ʻoe nā hōʻailona hou, e ʻike koke i ke kauka .

I ka manawa hea e pono ai ʻoe e hele i ka lumi pilikia?

Hiki i ka anemia cell sickle ke hoʻoulu i nā maʻi koʻikoʻi. E hele koke i ka lumi pilikia inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona:

• Nā hōʻailona o ka anemia (luhi nui, pōkole o ka hanu, puʻuwai puʻuwai kūpono ʻole).
• Ke kuni i ʻoi aku ma mua o 38.5 kekelē Celsius (101.3 Fahrenheit).
• ʻEha ka umauma.
• Kuke.

Hoʻonui pū kēia ʻano i ka pilikia o ka hahau ʻana. He pilikia olakino ka hahau ʻana . Inā loaʻa iā ʻoe nā hōʻailona aʻe, e hele koke i ka haukapila:

• Nā pilikia me ke kaulike o ke kino a i ʻole ka hoʻonohonoho ʻana.
• ʻIke pōwehiwehi a ʻike pālua paha.
• Ka huikau.
• Ka nāwaliwali a i ʻole ka maneʻo ma kekahi ʻaoʻao o ke kino.
• Paʻakikī i ka ʻōlelo ʻana (kūlou).

ʻO ka mea hope loa, kekahi mau mea e hoʻomanaʻo ai

He maʻi paʻakikī maoli ka anemia cell sickle. I nā makahiki i hala aku nei, ua liʻiliʻi loa ka manaʻolana o nā pēpē i hānau ʻia me kēia maʻi e ola. Akā,Ua holomua nui ka noiʻi lapaʻau i nā makahiki he ʻumi i hala iho nei, no laila ua hiki i ka poʻe he nui ke mālama i kēia maʻi mau loa a ola i ke ola maikaʻi.

Inā loaʻa iā ʻoe a i ʻole kāu keiki kēia maʻi, ʻoiai ʻoe e hauʻoli nei i ka holomua āu i hana ai i ka wā ma mua, e manaʻolana no ka wā e hiki mai ana. Ke hoʻāʻo mau nei nā mea noiʻi e ʻimi i nā lāʻau lapaʻau hou no kēia maʻi. E kamaʻilio me kāu kauka a e makaʻala i nā hoʻokolohua lapaʻau hou. Mai haʻalele i ka manaʻolana . He mea nui loa ka hahai ʻana i nā ʻōlelo aʻoaʻo lapaʻau kūpono a me ka noʻonoʻo maikaʻi.

ʻAʻai anemia cell sickle, anemia, nā maʻi hoʻoilina, nā maʻi koko, nā hoʻololi genetic, hemoglobin, nā ʻōlelo aʻoaʻo olakino