E aʻo kākou e pili ana i ka hoʻāʻo hou e ʻike ai inā he cystic fibrosis kou.

Ke paʻakikī nei kāu keiki liʻiliʻi i ka hanu ʻana i kekahi manawa, a i ʻole he pilikia me ka ʻole o ka piʻi ʻana o ke kaumaha? A i ʻole ʻike ʻoe i kahi mea e like me ka ʻū mau a i ʻole ke kani ʻana o ka wheezing ma ka umauma? I kekahi manawa, ma hope o kēia mau mea, aia paha kahi maʻi i lohe ʻole ʻia e mākou, akā he mea nui loa ia e makaʻala. ʻO ia ka mea a mākou e kamaʻilio ai i kēia lā. Ua kapa ʻia kēia ʻo Cystic Fibrosis , i kekahi manawa i hoʻopōkole ʻia ʻo (CF) . E nānā kākou i ke ʻano o ia mea, pehea e ʻike ʻia ai, a ʻoi aku hoʻi kekahi mau kikoʻī e pili ana i ka 'Sweat Test' i hana ʻia no kēia.

He aha maoli ka Cystic Fibrosis?

I ka ʻōlelo maʻalahi, he maʻi hoʻoilina ka cystic fibrosis. ʻO ia hoʻi, he mea ia e hiki ke hoʻoili ʻia mai nā mākua a i nā keiki. ʻO ka mea e hana ʻia ma kēia, ʻo ia kekahi o nā wai i loko o ko kākou kino, ʻoiai ka mucus, e lilo i mānoanoa loa a pipili. E noʻonoʻo e pili ana iā ia, ʻaʻole anei he pahee ka mucus i loko o nā māmā a me nā sinuses o ke kanaka olakino e like me ka wai? ʻO ia ka mea e mālama ai i ko kākou mau ala ea i maʻemaʻe a hoʻopau i nā germs.

Akā i loko o ka poʻe me ka cystic fibrosis, mānoanoa loa kēia mucus, e like me ke kāpili. No laila, ua waiho ʻia kēia mucus mānoanoa i loko o nā ʻōpū koʻikoʻi e like me nā māmā a me ka pancreas , e hoʻopilikia ana i kā lākou hana maʻamau. ʻOiai ke hōʻiliʻili ʻia kēia mucus mānoanoa i loko o nā māmā, hoʻomaka nā ala ea e ālai ʻia. A laila lilo ia i mea paʻakikī loa ke hanu, a hiki ke loaʻa pinepine nā maʻi. Inā pilikia ka pancreas, hiki ke loaʻa nā pilikia ʻai a me ka ʻai ʻole. ʻAʻole wale kēia, hiki iā ia ke hoʻopilikia i nā wahi e like me ke akepaʻa, nā ʻōpū, a me nā ʻāpana wahine.

He maʻi mau ka cystic fibrosis. ʻO ia hoʻi, he lōʻihi kona mau a ʻaʻole hiki ke ho'ōla loa ʻia. Ua manaʻo ʻia hoʻi he maʻi holomua . ʻO ia hoʻi, hiki ke ʻoi aku ka ʻino o nā hōʻailona i ka hala ʻana o ka manawa a hiki ke loaʻa nā pilikia. No laila he mea nui loa ka ʻike mua ʻana a me ka mālama pono ʻana.

He aha nā hōʻailona o kēia maʻi i nā keiki?

Inā loaʻa i kahi keiki ʻōpio, ʻoiai he pēpē, ka cystic fibrosis, hiki iā lākou ke ʻike i hoʻokahi a ʻoi aku paha o kēia mau hōʻailona. ʻAʻole e loaʻa i nā keiki āpau kēia mau hōʻailona āpau i ka manawa hoʻokahi, a ʻokoʻa ke koʻikoʻi o nā hōʻailona mai kekahi keiki a i kekahi keiki. He mea nui e hoʻomanaʻo i kēlā.

Eia kekahi o kēlā mau hiʻohiʻona:

• ʻAʻole ulu pono: ʻOiai ke hānai maikaʻi nei ka pēpē i ka waiū umauma a ʻai maikaʻi hoʻi ma hope, ʻaʻole paha e piʻi kona kaumaha a ʻike ʻia paha he kaumaha haʻahaʻa ke hoʻohālikelike ʻia me nā pēpē ʻē aʻe o ka makahiki like.
• ʻŌpū palupalu a ʻaila paha: I kekahi manawa, palupalu loa, wai, a ʻaila paha nā ʻōpū o kāu pēpē. I kekahi manawa, he ʻala ʻino paha nā ʻōpū.
• Paʻakikī ka hanu ʻana:Ke manaʻo mau nei he paʻakikī ke hanu, me he mea lā ua pau kou hanu.
• ʻŌhū mau: He leo ʻōhū mau e puka mai ana mai ka umauma. Hiki ke hana ʻia kēia i nā kūlana e like me ka hānō, akā he hōʻailona maʻamau ia ma CF.
• Nā maʻi pinepine o ka māmā: No ka laʻana, he mea maʻamau ka niumonia a me ka bronchitis. Inā loaʻa iā ʻoe nā maʻi māmā i hoʻokahi a ʻelua paha i ka mahina, he mea ia e hopohopo ai.
• Nā maʻi sinus i hana hou ʻia: Nā maʻi pinepine i loko o nā lua a puni ka ihu (sinuses). Nā mea e like me ka ihu paʻa a me ka ihu wai.
• ʻŪhū mau, malia paha he phlegmy: He ʻūhū mau ʻaʻole e hoʻomaikaʻi. He ʻūhū paha ia e hoʻopuka ana i ka mucus mānoanoa.
• Ka ulu lohi: Me he mea lā e ulu lohi iki ana ke kiʻekiʻe a me ke kaumaha o ke keiki ma mua o nā keiki ʻē aʻe o ka makahiki like.
• I kekahi manawa, ʻono ka paʻakai o ka ʻili o kā pēpē: Hōʻike pū kekahi mau mākua i ka wā e hamo ai lākou i ka ʻili hou o kā lākou pēpē, he paʻakai ia.

Inā kānalua ʻoe ua loaʻa i kāu keiki liʻiliʻi kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​ʻo ka mea maikaʻi loa e hana ai, ʻo ia ke ʻike ʻana i kahi pediatrician no ka ʻōlelo aʻo.

He aha ka hoʻāʻo ʻana i ka hū a he aha kāna hana?

Hoʻoholo nā kauka i ka cystic fibrosis ma ka nānā pono ʻana i ka mea maʻi, e hoʻolohe ana i kā lākou mau hōʻailona, ​​a me ka hana ʻana i nā hoʻokolohua like ʻole. Hoʻokomo pū kēia me nā hoʻokolohua genetic - ʻo ia hoʻi nā hoʻokolohua koko e nānā ana i nā loli i loko o nā genes - nā kukuna X-ray o ka umauma a me ka sinus, a me nā hoʻokolohua hana māmā.

Eia nō naʻe, ʻo ka hoʻāʻo koʻikoʻi a paʻa loa e hōʻoia inā he cystic fibrosis kou a ʻaʻole paha, ʻo ia ka hoʻāʻo hou. He hoʻāʻo maʻalahi loa kēia, ʻaʻohe ʻeha. Ana ia i ka nui o ka chloride i loko o kou hou. Ua ʻike anei ʻoe ua hana ʻia ka paʻakai a mākou e ʻai ai i ʻelua mau kemika i kapa ʻia ʻo chloride a me sodium. No laila, ʻoi aku ka nui o ka chloride i loko o ka hou o ka poʻe me ka cystic fibrosis ma mua o ke kanaka maʻamau.

ʻO ke kumu o kēia, ma muli o ke kīnā genetic e hoʻoulu ai i ka cystic fibrosis, ua hoʻopilikia ʻia ka hana o kahi protein e kōkua ana i ka chloride e neʻe ma o kā mākou mau cell. ʻO ka hopena, ʻaʻole hiki i ka chloride ke komo a haʻalele paha i nā cell. ʻO kēia ka manawa e hōʻiliʻili ai ka chloride i loko o ka hou. ʻOiai he mea maʻalahi loa ia, ʻo ia ke kumu ʻepekema kumu no ka ʻike ʻana i kēia maʻi.

ʻO ka maʻamau, ʻōlelo nā kauka i kēia hoʻāʻo hou inā pilikia ke keiki i ka hoʻoilina ʻana i ka cystic fibrosis (no ka laʻana, inā loaʻa i kekahi o ka ʻohana ka maʻi), a i ʻole inā e hōʻike ana lākou i nā hōʻailona a mākou i kūkākūkā ai ma mua. I kekahi manawa, ua noi ʻia nā kaikaina o ke keiki e hana i kēia hoʻāʻo. ʻO kēia ke ʻike inā ua hoʻoilina pū lākou i kēia gene a inā pilikia kā lākou mau keiki i ka hoʻoilina ʻana i kēia gene i ka wā e hiki mai ana.

Pehea e hana ʻia ai kēia hoʻāʻo hou? He mea anei ia e makaʻu ai?

Hiki ke hana ʻia kēia hoʻāʻo hou ma luna o kekahi o kēlā me kēia makahiki. Eia nō naʻe, ʻaʻole hiki i nā pēpē hānau hou ke hana i ka hou lawa no kēia hoʻāʻo i nā lā mua o ko lākou ola. No laila, hana pinepine ʻia kēia hoʻāʻo ma luna o nā pēpē ma waena o ʻelua a ʻehā pule o ka makahiki , i ka wā e hiki ai iā lākou ke hana i ka hou lawa.

He maʻalahi loa kēia hoʻāʻo e hana, ʻaʻole ia he ʻeha iki. No laila, mai makaʻu ʻoe, ʻeā? Eia ka mea e hana ʻia:

1. ʻO ka mea mua, hoʻopili kekahi limahana mālama ola, he kahu maʻi a he kauka paha, i kahi gel a wai paha i loaʻa kahi kemika i kapa ʻia ʻo pilocarpine i ka ʻili o ka lima o ke keiki (ʻo ka lima mua) a i ʻole ka wāwae. He lāʻau lapaʻau ʻo Pilocarpine e hoʻoulu ai i nā ʻōpū hou.

2. A laila, kau ʻia ʻelua mau electrodes ma kahi a hāʻawi ʻia kahi hoʻoulu uila liʻiliʻi loa a ʻino ʻole e hoʻoulu hou i nā kelepa hou a hoʻopuka i ka hou. Hana ʻia kēia no kahi o 5 mau minuke. ʻAʻohe ʻeha a ʻeha iki paha. Hiki i ka mea liʻiliʻi ke manaʻo i kahi ʻūlū iki a i ʻole ka mehana ma ko lākou lima a wāwae paha, akā ʻo ia wale nō. ʻAʻole ʻike ka hapa nui o nā pēpē i kēia.

3. A laila, wehe ʻia nā electrodes, hoʻomaʻemaʻe ʻia ka ʻili, a kau ʻia kahi mea kūikawā e hōʻiliʻili i ka hou (he ʻōwili plastik, kahi ʻāpana pepa kānana, a i ʻole kahi ʻāpana gauze) ma ia wahi. Waiho ʻia kēia ma kahi no kahi o 30 mau minuke e hōʻiliʻili i ka hou.

4. ʻO ka mea hope loa, hoʻouna ʻia kēia hāpana hou i hōʻiliʻili ʻia i kahi keʻena hoʻokolohua e ana pololei i ka nui o ka chloride i loaʻa.

Ma kahi o hoʻokahi hola ka lōʻihi o ka hoʻāʻo holoʻokoʻa. He mea maʻalahi ke hoʻomālielie i kāu keiki inā he mea pāʻani a he ʻōmole waiū paha kāu ma kahi kokoke.

He aha ka ʻōlelo a nā hopena o kēia hoʻāʻo?

ʻAe, e nānā kākou i nā hopena o kēia hoʻāʻo hou. Inā loaʻa i ke keiki ka cystic fibrosis, e hōʻike ka hoʻāʻo hou he nui aku ka nui o ka chloride i loko o ko lākou hou.

Ma ke ʻano o nā waiwai i ana ʻia, penei kēia (hiki ke ʻokoʻa iki kēia mau waiwai mai ka hale hana a i ka hale hana, akā penei ke ʻano maʻamau):

• Inā ʻoi aku ka nui o ka chloride o kāu keiki ma mua o 60 mmol/L (millimol/lita) : He mea hiki loa ke loaʻa iā lākou ka cystic fibrosis.ʻO ka maʻamau, inā kū mai kahi hopena e like me kēia, hana hou ʻia ka hoʻāʻo e hōʻoia.
• ʻOi aku ka liʻiliʻi o ka pae chloride o ke keiki ma mua o 30 mmol/L (millimol/lita) (ʻōlelo kekahi mau alakaʻi ma lalo o 40 mmol/L): Hiki ke ʻōlelo ʻia ʻaʻohe ona cystic fibrosis.
• Inā aia ka pae chloride o kāu keiki ma waena o 30 a me 59 mmol/L ( ma kekahi mau wahi 40-59 mmol/L): Ua manaʻo ʻia kēia he hopena waena a palena paha . I kēia hihia, pono paha e hana hou ʻia ka hoʻāʻo. Pono paha nā hoʻāʻo hou aʻe, e like me ka hoʻāʻo genetic, e kāpae i kekahi mau ʻano atypical o CF.

ʻO ka mea nui loa, ʻo ia ka wehewehe ʻana o kēia mau hopena e ke kauka. No laila, ke hiki mai nā hopena, ʻo ka mea maikaʻi loa e hana ai, ʻo ia ke kamaʻilio me ke kauka e pili ana i ia mea a hahai i kāna mau kuhikuhi. Mai hopohopo.

Pehea ʻoe e hoʻomākaukau ai i kāu pēpē no kahi hoʻāʻo hou? Pono ʻoe e hana i kekahi mea nui?

He mea maʻalahi loa kēia no ka makuahine a me ka makuakāne. ʻAʻole pono ʻoe e hana i kekahi mea kūikawā e hoʻomākaukau ai i kāu pēpē no kahi hoʻāʻo hou.

• ʻAʻohe pono e hoʻololi a hoʻōki paha i kekahi o ka ʻai a ke keiki (ke ʻano hānai ʻana, nā meaʻai paʻa inā hāʻawi ʻia), nā hana, a i ʻole nā ​​lāʻau lapaʻau a ke keiki e lawe nei ma mua o kēia hoʻāʻo. E mālama e like me ka mea maʻamau.
• ʻO ka mea wale nō, mai hoʻopili i kekahi kirīmi a lotion paha i ka ʻili o kāu keiki no 24 mau hola ma mua o ka hoʻāʻo. ʻOi loa aku, mai hoʻopili i kekahi mea i ka lima a i ʻole ka wāwae e hoʻāʻo ʻia nei. Hiki i kēia ke hoʻopilikia i nā hopena o ka hoʻāʻo.

Eia, he mea maʻalahi loa ia. E ʻaʻahu i kāu pēpē i nā lole māmā a ʻoluʻolu hoʻi i ka lā o ka hoʻokolohua.

ʻO nā mea nui e pono ai kākou e hoʻomanaʻo mai kēia moʻolelo (Leka Lawe-Home)

ʻAe, ke lana nei koʻu manaʻo ua maopopo iā ʻoe i kēia manawa ka mea a mākou i kamaʻilio ai i kēia lā, ʻo Cystic Fibrosis a me ka Hoʻāʻo Hou. ʻOiai paha he kumuhana paʻakikī kēia, ʻaʻole ia he mea paʻakikī ke maopopo iā ʻoe nā kumu. Mai nā mea a mākou i kūkākūkā ai, eia kekahi mau mea e hoʻomanaʻo ai:

• ʻO ka Cystic fibrosis (CF) kahi maʻi genetic e hoʻomānoanoa ai i ka mucus o ke kino, ʻoi aku ka hopena i nā māmā a me ka ʻōnaehana hoʻoheheʻe.
• E makaʻala i nā hōʻailona: Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​​​e like me ka pohō kaumaha, ka hanu pinepine ʻana, ka ʻū mau, a i ʻole nā ​​​​​​ʻōpala ʻaila, e ʻike i ke kauka a kūkākūkā iā lākou.
• ʻO ka hoʻāʻo hou ka hoʻāʻo nui a hilinaʻi loa no ka hōʻoia ʻana i ka cystic fibrosis: ke ana ia i ka pae chloride i ka hou.
• ʻAʻole ʻeha ka hoʻāʻo a palekana: no laila mai makaʻu e lawe i kāu pēpē no kēia.
• He mea nui loa ka ʻike ʻana i ka maʻi i ka wā mua:No ka mea, a laila, hiki ke hoʻomaka koke ʻia nā ʻano lapaʻau a me ka hoʻokele e pono ai, e hōʻemi ana i nā pilikia e loaʻa i ke keiki a kōkua iā lākou e ola i kahi ola maikaʻi a olakino hoʻi.

Inā ʻoe a i ʻole kekahi āu i ʻike ai e loaʻa ana i kēia pilikia, ʻo ka mea maikaʻi loa e hana ai, ʻaʻole e hopohopo a manaʻoʻiʻo paha i nā mea āpau āu e ʻike ai ma ka pūnaewele, akā e kamaʻilio me ke kauka kūpono. E alakaʻi pono lākou iā ʻoe a hāʻawi iā ʻoe i ke kākoʻo āu e pono ai.

ʻO Cystic Fibrosis, hoʻāʻo hou, chloride, pediatrics, nā maʻi genetic, nā maʻi māmā