Ke luhi mau nei kāu keiki liʻiliʻi? ʻAʻole anei ʻo ia makemake e ʻai nui? Ua ʻike anei ʻo ia ua ʻoi aku kona keʻokeʻo ma mua o nā keiki ʻē aʻe? ʻOiai paha he mea maʻamau kēia, i kekahi manawa aia paha kahi maʻi laha ʻole ma hope o kēia a mākou i lohe ʻole ai. I kēia lā e kamaʻilio mākou e pili ana i kekahi maʻi laha ʻole, akā inā mālama pono ʻia, hiki iā ʻoe ke ola me ia me ka holomua. ʻO ia hoʻi ʻo Diamond-Blackfan Anemia, a i ʻole `(Diamond-Blackfan Anemia - DBA)`.
I ka ʻōlelo maʻalahi, he aha ka Diamond-Blackfan Anemia (DBA)?
E noʻonoʻo he hale hana nui ko kākou i loko o ko kākou kino e hana ana i ke koko. Kāhea mākou iā ia he iwi ʻiʻo . ʻO ka hana nui o kēia hale hana, ʻo ia ka hana ʻana i nā hunaola koko ʻulaʻula, nā limahana liʻiliʻi e lawe ana i ka oxygen ma o ko kākou kino. ʻO ka DBA kahi kūlana kahi i hiki ʻole ai i kēia hale hana i kapa ʻia ʻo ka iwi ʻiʻo ke hana i nā hunaola koko ʻulaʻula lawa. He mea kākaʻikahi loa kēia, ʻo ia hoʻi, ʻike ʻia kēia kūlana ma kahi o hoʻokahi i loko o 500,000 mau keiki.
He maʻi hoʻoilina kēia. ʻO ke ʻano kēia, ua hoʻololi ʻia nā genes i loko o nā cell o ko kākou kino. Hoʻemi kēia i ka nui o nā cell koko ʻulaʻula i loko o ke kino. Kāhea mākou i kēia maʻi he anemia , a i ʻole ka nele o ke koko a i ʻole anemia. He maʻi ola ka DBA. Akā, mai hopohopo, me ka mālama maikaʻi ʻana, hiki i nā poʻe he nui ke kāohi i ko lākou mau hōʻailona a ola i ke ola maikaʻi .
He aha nā hōʻailona o kekahi me DBA?
Hiki ke ʻokoʻa nā hōʻailona o DBA mai kēlā kanaka a kēia kanaka. Hiki i kekahi poʻe ke ʻike i nā hōʻailona iki, aʻo kekahi poʻe e ʻike i nā hōʻailona koʻikoʻi. E nānā kākou i nā hōʻailona nui.
| Hōʻailona | He wehewehe maʻalahi |
|---|---|
| Luhi pinepine | No ka mea, ʻaʻole i loaʻa i nā ʻāpana o ke kino ka nui o ka oxygen e pono ai, hiamoe pinepine ʻoe a ʻaʻohe ou ikaika e hana ai i kekahi mea. |
| Ka ʻili keʻokeʻo (pallor) | Ua ʻike ʻia ka ʻili, nā lehelehe, a me nā kui he keʻokeʻo ma muli o ka nele o nā hunaola koko ʻulaʻula, nona ke kumu o ke kala ʻulaʻula o ke koko. |
| Ka wikiwiki o ka puʻuwai (Tachycardia) | Pono ka puʻuwai e hana ikaika aʻe e pamu i ke koko me ka wikiwiki a hāʻawi i ke kino i ka oxygen e pono ai. |
| Paʻakikī ka hanu ʻana | I kekahi manawa, e like me ka hele wāwae ʻana i kahi mamao pōkole a i ʻole ke piʻi ʻana i kahi alapiʻi, paʻakikī iaʻu ke hanu, a manaʻo wau e like me kaʻu e hanu nui nei. |
| Makemake nui | ʻOi aku hoʻi nā kamaliʻi ʻōpio i makemake nui ʻole i ka ʻai ʻana. |
| ʻEha poʻo a me ka nāwaliwali | He kūlana maʻamau i hoʻokumu ʻia e ka lawa ʻole o ka oxygen i ke kino a me ka lolo. |
No ke aha e loaʻa ai kēia ʻano maʻi? He aha ke kumu?
E like me kā mākou i kūkākūkā ai ma mua, he maʻi hoʻoilina kēia. Aia nā genes kikoʻī i loko o ko mākou kino e kuhikuhi iā mākou e hana i nā hunaola koko ʻulaʻula. Ua kapa ʻia lākou he `(ribosomal protein genes)`. ʻO ke kanaka me DBA he ʻokoʻa a i ʻole mutation i loko o kēia mau genes. ʻO ka hopena, ʻaʻole hana pono ke kaʻina hana o ka hana ʻana i nā hunaola koko ʻulaʻula.
ʻO ka mea nui, hiki ke hoʻoili ʻia mai nā mākua a i nā keiki. Akā, he hapa liʻiliʻi wale nō kēia e hana ʻia, ma kahi o 10% - 25%. I nā hihia he nui, hiki i kahi keiki ke loaʻa i ke kūlana ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ka maʻi, ma muli o kahi hoʻololi genetic maʻamau.
He aha nā pilikia ʻē aʻe e hiki ke hana ma muli o DBA?
ʻAʻole wale ka hopena o ka DBA i nā hunaola koko ʻulaʻula. I kekahi manawa hiki iā ia ke hana i nā pilikia olakino ʻē aʻe. Eia kekahi, ʻoi aku ka nui o ka pilikia o ka poʻe me DBA i ka loaʻa ʻana o kekahi mau ʻano maʻi kanesa ma mua o ka heluna kanaka maʻamau.
| ʻAno o ka pilikia | Wehewehena |
|---|---|
| Nā pilikia maʻamau | |
| Nā kīnā hānau | Aia paha kekahi mau pilikia me ka puʻuwai, nā puʻupaʻa, a i ʻole nā lālā. |
| Ka ulu ʻana o ka ulu ʻana | ʻOi aku ka lohi o ka ulu ʻana o ke kino ma mua o ka mea i manaʻo ʻia no ka makahiki. |
| Hao nui | ʻO ka hāʻawi pinepine ʻana i ke koko hiki ke hoʻonui i ka nui o ka hao o ke kino me ke kumu ʻole, e hōʻino ana i nā ʻōkana e like me ka puʻuwai a me ke akepaʻa. |
| Ka emi ʻana o nā hunaola koko ʻē aʻe | I kekahi manawa, haʻahaʻa paha nā hunaola koko ʻē aʻe, e like me nā hunaola koko keʻokeʻo (neutropenia) a i ʻole nā platelets (thrombocytopenia). |
| Ka pilikia o ke kanesa (kiʻekiʻe iki aʻe) | |
| Leukemia | ʻOi aku hoʻi kahi ʻano maʻi koko i kapa ʻia ʻo "Acute myeloid leukemia - AML". |
| ʻO ka maʻi Myelodysplastic (MDS) | ʻO kekahi maʻi koko e hoʻopilikia ai i ka hana o ka iwi iwi. |
| ʻOsteosarcoma | He maʻi kanesa e kū ana i loko o nā iwi. |
Pehea e ʻike pololei ai nā kauka i kēia maʻi?
Inā loaʻa i kāu keiki kēia mau hōʻailona, e nānā pono kāu kauka i kāu keiki a nīnau iā ʻoe e pili ana i nā kikoʻī. A laila, hana paha lākou i kekahi mau hoʻokolohua e hōʻoia i ka maʻi.
- Helu Koko Piha (CBC): Ke ana kēia i ka helu o nā hunaola koko ʻulaʻula, nā hunaola koko keʻokeʻo, a me nā platelets i loko o ke koko. He haʻahaʻa loa ka helu o nā hunaola koko ʻulaʻula o ke kanaka me DBA.
- Helu Reticulocyte: ʻO nā reticulocytes he mau hunaola koko ʻulaʻula hou i hoʻokumu ʻia ʻaʻole i oʻo piha. ʻO ka helu haʻahaʻa ʻo ia hoʻi ʻaʻole lawa ka hana ʻana o ka iwi iwi i nā hunaola koko ʻulaʻula hou.
- ʻO ka ʻume ʻana o ka iwi a me ka biopsy: ʻO kēia ka hoʻāʻo nui i hoʻohana ʻia e hōʻoia i ka maʻi. Ma kēia, lawe ʻia kahi hāpana liʻiliʻi loa o ka iwi, maʻamau mai ka iwi ʻūhā, ma lalo o ka anesthesia. A laila e nānā ʻia ma lalo o kahi microscope e hoʻoholo pono i ka nui o ka hana ʻana o nā cell koko ʻulaʻula e hana nei.
He aha nā lāʻau lapaʻau no DBA?
ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia ka DBA, aia nā lāʻau lapaʻau kūpono e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona a mālama i kahi ola maikaʻi.
1. Corticosteroids: Hoʻoulu kēia mau lāʻau i ka iwi iwi e hana i nā hunaola koko ʻulaʻula hou aʻe. Ua holomua kēia lāʻau no nā maʻi he nui.
2. Hoʻoili koko: Hāʻawi ʻia nā poʻe me nā helu koko haʻahaʻa loa i nā hunaola koko ʻulaʻula mai kahi mea hāʻawi olakino. Hiki i nā kauka ke hoʻomaka i kēia lāʻau lapaʻau i ka wā e ʻike ʻia ai ka maʻi. Hāʻawi kēia i ka hōʻoluʻolu koke mai nā hōʻailona.
3. Hoʻoili ʻana i nā pūnaewele kumu: ʻO kēia wale nō ka lāʻau e hiki ke hoʻōla loa i ka DBA. Eia nō naʻe, he lāʻau paʻakikī loa kēia a he pilikia hoʻi. Ma kēia, hoʻoili ʻia nā pūnaewele kumu mai ka iwi iwi o ke kanaka olakino (ʻo ka hapa nui he lālā o ka ʻohana) i loko o ka mea maʻi. ʻAʻole hiki kēia i nā mea āpau. Hoʻopaʻa pono ke kauka i ke kūlana o ka mea maʻi, loaʻa ka mea kūpono loa, a hoʻoholo i kēia ma hope o ka noʻonoʻo nui ʻana.
He aha ka mea e hana ai i ka wā pilikia?
Inā loaʻa iā ʻoe a i ʻole kāu keiki ka DBA, he mea nui loa ia e hoʻomau i ka launa pū me kāu kauka. He mea nui e mālama i nā manawa i hoʻopaʻa ʻia a lawe i kāu mau lāʻau lapaʻau e like me ke kauoha. Inā e ʻoi aku ka ʻino o kāu mau hōʻailona (no ka laʻana, ua ʻoi aku kou luhi ma mua o ka maʻamau, loaʻa iā ʻoe nā maʻi hou aʻe), e haʻi koke i kāu kauka.
Eia kekahi, inā ʻike ʻoe i kekahi o nā hōʻailona ulia pōpilikia ma lalo nei, e hele koke i ka haukapila Emergency Department (ETU) kokoke loa .
- ʻEha nui o ka umauma
- ʻEha koke ka ʻōpū nui
- ʻEha poʻo nui koke, huikau, a paʻakikī paha ke ʻōlelo (he mau hōʻailona paha kēia o ka hahau ʻana)
- Ke kahe koko ʻole i kaohi ʻia
ʻO ka noho ʻana me kahi maʻi laha ʻole e like me DBA no ke ola holoʻokoʻa, ʻoiai inā ʻo kāu keiki ia, hiki ke paʻakikī loa. Hiki ke manaʻo mehameha. Akā e hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. E hāʻawi kāu kauka a me ka hui lapaʻau iā ʻoe i ke kākoʻo a me ke alakaʻi āu e pono ai.
Leka Lawe-Home
- ʻO ka Diamond-Blackfan anemia (DBA) kahi maʻi genetic laha ʻole e pale ai i ka iwi iwi mai ka hana ʻana i nā hunaola koko ʻulaʻula lawa.
- ʻO ka luhi pinepine, ka maeʻele, a me ka pōkole o ka hanu nā hōʻailona nui.
- ʻOiai he maʻi kēia no ke ola holoʻokoʻa, hiki ke mālama pono ʻia me nā lāʻau lapaʻau e like me nā lāʻau steroid a me nā hoʻoili koko.
- He mea nui e noho mau i ka launa pū ʻana me kāu kauka a loaʻa i nā hoʻokolohua kūpono a me ka lāʻau lapaʻau.
- E makaʻala i nā hōʻailona hōʻike pilikia (e like me ka ʻeha nui o ka umauma, ka nalowale o ka ʻike) a e hele koke i ka Emergency Treatment Unit (ETU) o ka haukapila i kēlā hihia.
- ʻOiai inā manaʻo ʻoe ua kaʻawale ʻoe no ka mea he maʻi laha ʻole kēia, e hoʻomanaʻo he mau hui lapaʻau a me nā pūnaewele kākoʻo e kākoʻo iā ʻoe.
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