Ua ʻike ʻoe e pili ana i nā hunaola koko ʻulaʻula i loko o kou koko. He poepoe lākou, palupalu, e like me nā pōpō laholio liʻiliʻi. ʻAe kēia iā lākou e neʻe maʻalahi ma o nā kīʻaha koko liʻiliʻi i loko o kou kino a lawe i ka oxygen i nā ʻāpana āpau o kou kino. Akā i kekahi poʻe, lilo kēia mau hunaola koko ʻulaʻula i ʻano sickle (ʻano C), ʻoʻoleʻa a pipili. ʻO kēia ka mea a mākou e kapa nei he maʻi sickle cell (SCD). ʻAʻole kēia he maʻi i hoʻoilina ʻia, akā he maʻi genetic.
He aha maoli ka maʻi cell sickle (SCD)?
I ka ʻōlelo maʻalahi, ʻo ka maʻi cell sickle kahi maʻi genetic e hoʻopilikia ana i kā mākou mau hunaola koko ʻulaʻula. Aia i loko o kēia mau hunaola koko ʻulaʻula kahi protein i kapa ʻia ʻo hemoglobin . ʻO kāna hana nui ka lawe ʻana i ka oxygen mai nā māmā a hoʻolaha iā ia ma ke kino holoʻokoʻa. He poepoe a laumania ka molekole hemoglobin o ke kanaka olakino. ʻO ia ke kumu he poepoe nani nā hunaola koko ʻulaʻula.
Akā i loko o kekahi me ka maʻi cell sickle, ma muli o kahi kīnā genetic, loli ke ʻano o kēia molekole hemoglobin. Ma muli o kēia hemoglobin ʻano ʻē, lilo nā hunaola koko ʻulaʻula i ʻano sickle, paʻakikī, a pipili. E noʻonoʻo, he aha ka mea e hana ai inā ʻoe e hoʻāʻo e hoʻouna i nā ʻāpana koko paʻakikī, ʻano sickle ma o kahi ʻōmole lahilahi, ma kahi o nā pōpō poepoe e neʻe ana ma ia mea? E hui pū lākou a paʻa i loko o ka ʻōmole, ʻeā? Ma ke ʻano like, paʻa kēia mau hunaola ʻano sickle i loko o ko mākou mau kīʻaha koko lahilahi, e ālai ana i ke kahe koko. ʻO kēia ke kumu o nā pilikia like ʻole e like me ka ʻeha nui a me ka anemia .
He aha nā hōʻailona maʻamau o kēia maʻi?
ʻO ka maʻamau, hoʻomaka nā pēpē i hānau ʻia me ka maʻi sickle cell e hōʻike i nā hōʻailona ma kahi o 5 mau mahina. Hiki ke ʻokoʻa kēia mau hōʻailona mai kēlā kanaka a kēia kanaka. Hiki nō hoʻi ke loli i ka hala ʻana o ka manawa.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| Anemia | He palupalu loa nā hunaola sickle. ʻOiai ke ola nei kahi hunaola koko ʻulaʻula maʻamau no kahi o 120 mau lā, make kēia mau hunaola i loko o 10-20 mau lā. ʻO kēia ke kumu o ka nele o ke kino i nā hunaola koko ʻulaʻula, e hoʻoluhi a luhi mau ai ʻoe. |
| Nā pilikia ʻeha | ʻO kēia ka hōʻailona nui a koʻikoʻi loa o kēia maʻi. Ke hōʻiliʻili nā cell sickle a ālai i nā kīʻaha koko palupalu i loko o ka umauma, ka ʻōpū, nā lālā, a me nā iwi, hoʻoulu ia i ka ʻeha hiki ʻole ke hoʻomanawanui. Inā koʻikoʻi kēia ʻeha, pono paha ʻoe e lawe ʻia i ka haukapila a hele i ka ETU (Emergency Treatment Unit) . |
| Ka pehu ʻana o nā lima a me nā wāwae | Ke ālai ʻia nā aʻa koko palupalu e hoʻolako ana i ke koko i nā lima a me nā wāwae, hoʻomaka kēlā mau wahi e pehu. |
| Nā maʻi pinepine | I kekahi manawa, hōʻino kēia mau cell sickle i nā ʻōkana e like me ka spleen. He ʻōkana koʻikoʻi loa ka spleen i loko o ka ʻōnaehana pale o ko mākou kino. Ke hōʻino ʻia, piʻi ka pilikia o ka loaʻa ʻana o nā maʻi. |
| Melemele o nā maka a me ka ʻili | I ka wā e haki ai ka nui o nā hunaola sickle i hōʻino ʻia, e melemele paha ka ʻili a me nā keʻokeʻo o nā maka (e like me ka jaundice). |
| Nā hemahema o ka ʻike maka | Inā paʻa kēia mau pūnaewele i loko o nā kīʻaha koko e hoʻolako ana i ke koko i ka maka, hiki ke hōʻino ʻia ka retina o ka maka a hiki ke loaʻa nā pilikia ʻike. |
| Ka lohi o ka ulu ʻana | Hiki i nā keiki me kēia maʻi ke ulu mālie aʻe ma mua o nā keiki ʻē aʻe, a hiki ke lohi paha ka wā ʻōpio. |
No ke aha e loaʻa ai kēia maʻi? ʻO wai ka mea i pilikia nui?
ʻO ke kumu nui o kēia maʻi he kīnā hoʻoilina. Hoʻokumu ʻia ia e kahi kīnā i loko o kahi gene (hemoglobin-beta gene) ma kā mākou chromosome 11, ka mea e pili ana i ka hana ʻana o ka hemoglobin.
ʻO ka mea nui loa, i mea e loaʻa ai i ke keiki kēia maʻi, pono iā lākou ke hoʻoilina i kēia gene kīnā mai ko lākou makuahine a me ko lākou makuakāne.
Inā he mau mea lawe kēia gene kīnā nā mākua ʻelua, ʻo ia hoʻi, ʻaʻole i loaʻa iā lāua ka maʻi akā loaʻa iā lāua ka gene e hoʻoulu ai i ka maʻi i loko o ko lāua kino, he 1 i loko o 4 (25%) ka manawa e hānau ʻia ai kā lāua keiki me ka maʻi.
Inā hoʻokahi wale nō makua e hoʻoilina i ka gene, lilo ke keiki i mea lawe i ka maʻi. ʻAʻole lākou e hōʻike pinepine i nā hōʻailona, akā hiki iā lākou ke hoʻoili i ka gene i kā lākou mau keiki.
Ma ka honua holoʻokoʻa, ʻoi aku ka maʻamau o kēia maʻi i waena o ka poʻe o ʻApelika, Hikina Waena, ʻEulopa Hema, a me nā lāhui ʻĀkia.
Pehea e ʻike ai i ka maʻi? (Diagnosis)
Aia kekahi ala maʻalahi loa e ʻike ai i kēia. Hiki i kahi hoʻāʻo koko maʻalahi ke ʻike i ke alo o kēia ʻano hemoglobin kīnā. Ma nā ʻāina he nui, ua hoʻāʻo ʻia kēlā me kēia pēpē hānau hou no kēia.
Inā ua ʻike ʻia ʻoe a i ʻole kāu keiki me kēia maʻi, hiki i kāu kauka ke ʻōlelo aku i nā hoʻāʻo hou aʻe (e like me kahi scan kūikawā e nānā i ka pilikia o ka hahau ʻana). A, no ka mea he maʻi genetic ia, hiki ke kuhikuhi ʻia ʻoe i kahi kākāʻōlelo genetic.
Inā loaʻa iā ʻoe a i ʻole kāu hoa ka maʻi sickle cell a i ʻole he mea lawe ia, hiki iā ʻoe ke hoʻāʻo ʻia ka wai amniotic o kāu pēpē i ka wā hāpai e ʻike ai inā loaʻa iā lākou ka maʻi. E nīnau i kāu kauka no ka ʻike hou aku.
He aha nā lāʻau lapaʻau?
ʻEkolu mau pahuhopu nui o ka mālama ʻana i ka maʻi cell sickle:
- Ke pale ʻana i nā pilikia ʻeha
- Ke kāohi nei i nā hōʻailona a me ka hāʻawi ʻana i ka hōʻoluʻolu
- Ke pale ʻana i nā pilikia ʻē aʻe
Aia nā ʻano lapaʻau like ʻole no kēia.
Nā lāʻau lapaʻau
- Hydroxyurea: Hiki i kēia lāʻau lapaʻau, i lawe ʻia i kēlā me kēia lā, ke hōʻemi i ke alapine o nā hoʻouka ʻana a hōʻemi i nā pilikia e like me ka anemia a me nā maʻi. Pono nā wahine hāpai e pale i ka lawe ʻana i kēia lāʻau lapaʻau.
- Nā lāʻau hoʻopau ʻeha: Ke loaʻa kahi hōʻeha ʻeha, hiki i nā lāʻau hoʻopau ʻeha i kuhikuhi ʻia e ke kauka ke kōkua i ka kaohi ʻana i ka ʻeha.
- Nā lāʻau lapaʻau ʻē aʻe: Ke hoʻohana ʻia nei nā lāʻau lapaʻau e like me 'Crizanlizumab' (kahi inikini) a me 'L-glutamine' (kahi pauka) e hōʻemi i nā hōʻeha ʻeha, a me nā lāʻau lapaʻau e like me 'Voxelotor' no ka anemia.
Nā lāʻau lapaʻau ʻē aʻe
- Ka Hoʻoili Koko: ʻO ka hāʻawi ʻana i ke koko olakino hiki ke pale i nā pilikia e like me ka anemia a me ka maʻi lolo.
- Hoʻoili Kelepona Kumu: He hoʻoili iwi iwi kēia. Loaʻa i kekahi mau keiki a me nā mākua ʻōpio ka hiki ke ho'ōla loa ʻia mai ka maʻi ma o kēia. Eia nō naʻe, pono e loaʻa kahi mea hāʻawi kūpono (ʻo ka maʻamau he hoahānau pili) no kēia.
Nā ʻAno Lapaʻau Hou Loa: Gene Therapy
Me ka holomua o ka ʻepekema lapaʻau, ua puka mai nā lāʻau lapaʻau holomua a hoʻohiki no kēia maʻi. ʻO 'Casgevy' lāua ʻo 'Lyfgenia' ʻelua mau lāʻau lapaʻau gene hou loa. I ka ʻōlelo maʻalahi, hoʻohana kēia mau ʻano hana i nā cell kumu i lawe ʻia mai ka iwi iwi ponoʻī o ka mea maʻi, 'hoʻoponopono' me ka hoʻohana ʻana i ka ʻenehana genetic e like me 'CRISPR', ʻo ia hoʻi, hoʻoponopono i ke kīnā, hoʻololi ʻia i mau cell koko ʻulaʻula olakino, a laila hoʻokomo hou ʻia i loko o ke kino. He lāʻau lapaʻau holomua loa kēia e hiki ai ke ho'ōla i ka maʻi.
He aha ka pilina ma waena o ka maʻi sickle cell a me ka malaria?
He moʻolelo kupanaha loa kēia. He maʻi koʻikoʻi ka Malaria i laha ʻia e nā makika. Manaʻo nā kānaka ʻepekema ua ulu ka gene cell sickle e pale i nā kānaka mai ka malaria. Pehea lā e hiki ai kēlā? Inā loaʻa i kekahi mea lawe i ka maʻi cell sickle (ʻaʻole ka maʻi, ʻo ka gene wale nō) ka malaria, ʻaʻole ia he ʻoi aku ka koʻikoʻi. ʻO kēia no ka mea ua pale nā kelepona koko ʻulaʻula i ka parasite malaria mai ka ulu pono ʻana i loko o ke kino. ʻO ia ke kumu i ola ai ka poʻe me kēia gene ma nā wahi i maʻamau ai ka malaria, e like me ʻApelika.
Leka Lawe-Home
- ʻAʻole ka maʻi ʻeha he maʻi lele, akā, he maʻi hoʻoilina i hoʻoilina ʻia mai nā mākua mai.
- ʻO ka pilikia nui ma ʻaneʻi, ʻo ia ka loli ʻana o ke ʻano o nā hunaola koko ʻulaʻula a lilo i mea paʻa i loko o nā kīʻaha koko.
- ʻO ka ʻeha nui, ka luhi pinepine (anemia), a me ka maʻi nā hōʻailona nui.
- Ma ka hahai ʻana i ka lāʻau lapaʻau kūpono a me nā ʻōlelo aʻoaʻo, hiki iā ʻoe ke kāohi i kāu mau hōʻailona a ola i kahi ola maʻamau. He mea nui loa ia e noho mau i ka launa pū ʻana me kāu kauka.
- Mahalo i nā lāʻau lapaʻau hou e like me ka gene therapy, aia ka manaʻolana maikaʻi loa no kēia maʻi.
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