Ua ʻike anei ʻoe i ka PCD? (Primary Ciliary Dyskinesia) E kamaʻilio kākou e pili ana i kēia maʻi laha ʻole!

Ua lohe paha ʻoe i kahi maʻi i kapa ʻia ʻo 'Primary Ciliary Dyskinesia' a i ʻole `(Primary Ciliary Dyskinesia - PCD)`? ʻOiai paha he mea ʻē iki ka inoa, he mea kakaikahi kēia, akā he mea nui loa ia e hoʻopilikia ai i kā mākou ʻōnaehana hanu a me nā kaʻina hana kino ʻē aʻe. He mea ia e kū pinepine ana i ka wā hānau. No laila, i kēia lā e kamaʻilio mākou e pili ana i kēia PCD ma ke ʻano maʻalahi hiki iā ʻoe ke hoʻomaopopo.

He aha ka dyskinesia ciliary mua (PCD)?

I ka ʻōlelo maʻalahi, ʻo ka PCD kahi maʻi e kū mai ana i ka wā e hana pono ʻole ai nā ʻano liʻiliʻi e like me ka lauoho i loko o ko mākou kino i kapa ʻia ʻo cilia . He liʻiliʻi loa kēia mau cilia a ʻike ʻole ʻia e ko mākou mau maka. E noʻonoʻo e hana ana kēia mau cilia e like me nā pulumi liʻiliʻi i loko o ko mākou ala hanu, ʻo ia hoʻi, ma nā wahi e like me ka ihu, ka puʻu, a me nā māmā.

No laila he aha ka hana a kēia mau cilia?

ʻO kēia mau ʻano liʻiliʻi e like me ka lauoho i kapa ʻia ʻo cilia ʻaʻole i loaʻa wale i loko o kā mākou ʻōnaehana hanu, akā ma kekahi mau wahi ʻē aʻe. ʻO kā lākou hana nui ka holoi ʻana i nā mea pono ʻole e like me nā germs a me ka lepo e komo i loko o ko mākou kino, a me ka mucus e hōʻiliʻili ana i loko o ke kino . Hana lākou i kēia ma ka neʻe ʻana e like me ka nalu. ʻAʻole wale ia, i ka wā e ulu ana kahi pēpē i loko o ka ʻōpū, kōkua pū kēia mau cilia i nā ʻōpū o ke pēpē e noho ma nā wahi kūpono.

No laila, ʻo ka mea e hana ʻia i kahi kanaka me PCD, ʻaʻole hana pono kēia mau cilia. ʻO ia hoʻi:

• Malia paha ua hewa ka nui o kēia cilia.
• He mea ʻē paha ko lākou ʻano .
• Malia paha e nalowale ana nā cilia .
• A i ʻole, hiki iā lākou ke neʻe a puni e like me ka pupule, me ka ʻole o ka pilina kekahi i kekahi .
• A i ʻole, ʻaʻole paha e neʻe iki .

Pehea lā kākou e maʻi ai i nā cilia e hana pono ʻole ana?

Ke hana pono ʻole kēia mau cilia, hiki ke loaʻa nā ʻano pilikia olakino like ʻole. Hoʻomaka kekahi i ka wā hānau, aʻo kekahi e ʻike ʻia ma hope o ke ola.

• Ke kūlana ʻano ʻē o ke kino: E noʻonoʻo, aia nā kino e like me ko kākou puʻuwai, nā māmā, ka spleen, a me nā mea ʻē aʻe ma kekahi ʻaoʻao ma kahi o ka ʻākau. I kekahi manawa, ʻo ka mea e pono ai ma ka ʻaoʻao ʻākau hiki ke waiho ʻia ma ka hema. Kāhea mākou i kēia "Situs Inversus" . I kekahi manawa, hiki ke hoʻohuli loa ʻia nā kino i ke ala hewa. Hiki nō hoʻi kēia ke hana inā ʻaʻole hana pono kēlā mau cilia.
• Maʻi hanu koʻikoʻi a mau loa: ʻO kēia ka pilikia nui o PCD. Ke hiki ʻole i nā cilia ke hoʻokuke aku i ka mucus, hōʻiliʻili ka mucus i loko o nā māmā a me nā ala ea. ʻO kēia ke alakaʻi i ka ʻū pinepine, ka paʻa ʻana o ka umauma, a me nā maʻi koʻikoʻi . Hiki i kēia ke hōʻino i nā māmā.
• Nā pilikia hānau keiki: Loaʻa pinepine i nā kāne me PCD ka pilikia i ka hānau ʻana i nā keiki, ʻo ia hoʻi, hiki iā lākou ke hānau ʻole . Hiki i nā wahine ke loaʻa nā pilikia koʻikoʻi i ka wā hāpai, e like me ka hāpai ʻana o ka ectopic (kahi e hoʻokomo ʻia ai ka embryo ma waho o ka ʻōpū).Hiki nō.

He aha ke kumu o ka PCD?

He maʻi hoʻoilina kēia. ʻO ia hoʻi, ua hana ʻia e kekahi mau loli a i ʻole nā ​​hoʻololi ʻana i loko o nā genes i hoʻoili ʻia mai ka makua a i ke keiki. Aia kekahi mau ʻano hoʻololi hoʻoilina e hoʻoulu ai i kēia maʻi.

He aha nā hōʻailona o ka dyskinesia ciliary mua?

ʻIke pinepine ʻia nā hōʻailona i ka wā hānau, a hiki ke hoʻonui mālie ʻia i ka hala ʻana o ka manawa.

Nā hōʻailona o ka PCD i ʻike ʻia i ka wā hānau:

• ʻEha ka hanu : Hiki i ka pēpē ke pilikia i ka hanu ʻana ma hope koke iho o ka hānau ʻana.
• Ihu paʻa : Manaʻo paha ʻoe ua paʻa mau kou ihu.
• ʻŪhū pulu mau : Hiki ke hoʻomau ka ʻūhū e hoʻopuka ana i ka mucus.
• Ke kūlana maʻamau ʻole o nā kino (heterotaxia) : E like me ka mea i ʻōlelo ʻia ma mua, aia paha nā kino ma kahi kūpono ʻole, nalowale paha kekahi mau kino, a i ʻole ʻaʻole paha lākou e ulu pono. ʻO Situs inversus kekahi ʻano maʻi like.
• I kekahi manawa he maʻi puʻuwai hānau .
• Ka nalowale ʻana o ka hoʻonui ʻia ʻana o ka māmā (atelectasis) .
• Ka ulu ʻana e like me ka cyst i loko o nā ʻōpū e like me nā puʻupaʻa a me ka pancreas.
• Ka hemahema o ka antibody (humoral (antibody) deficiency), hiki ke alakaʻi i ka hoʻonui ʻia o ka pilikia o nā maʻi māmā a me nā sinus.

Nā hōʻailona PCD mau loa:

I ka hala ʻana o ka manawa, e ʻike paha ʻoe i nā hōʻailona e like me kēia:

• ʻŪhū mau loa : He ʻūhū mau loa.
• Sinusitis mau : Nā pilikia sinus pinepine.
• Nā maʻi pepeiao : Nā maʻi pepeiao pinepine, ka kahe ʻana o ka wai mai nā pepeiao.
• Ka nui o ka huʻa a me ka phlegm .
• Ka hānau ʻole (ʻoi aku hoʻi no nā kāne).
• Nā polyps ihu .
• ʻO ka loaʻa pinepine ʻana o ka maʻi pneumonia koʻikoʻi .
• Nā maʻi pinepine o ka hanu luna .
• I kekahi manawa aia kahi hōʻiliʻili ʻana o ka wai ma ka lolo (hydrocephalus).

Pehea e ʻike ʻia ai ka dyskinesia ciliary mua (PCD)?

ʻOiaʻiʻo, he paʻakikī iki ka ʻike ʻana i kēia. ʻAʻole hiki ke ʻōlelo maopopo me hoʻokahi hoʻāʻo wale nō he PCD ia. Hoʻoholo nā kauka e pili ana i kēia ma hope o ka hana ʻana i kekahi mau hoʻokolohua like ʻole.

• Hoʻokolokolo lapaʻau a me ka mōʻaukala lapaʻau: ʻO ka mea mua, e nīnau ke kauka iā ʻoe e pili ana i kāu mau hōʻailona a inā paha ua loaʻa i kekahi o kou ʻohana nā kūlana like.
• ʻO ka biopsy Cilia: Lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka ihu a i ʻole nā ​​māmā a nānā ʻia ma lalo o kahi microscope. ʻAe kēia iā mākou e ʻike i ke ʻano, ka nui, a me ka neʻe ʻana o ka cilia.
• Hoʻāʻo hoʻoilina:Ke nānā nei kēia hoʻokolohua i nā hoʻololi genetic e pili ana me PCD. Eia nō naʻe, ʻaʻole paha e hiki ke loaʻa kēia mau hoʻololi genetic i nā mea āpau me PCD.

Ma waho aʻe o kēia, hiki ke hana ʻia nā hoʻokolohua ʻē aʻe inā kānalua ʻia ka PCD:

• Ana ʻana o ka nitric oxide ihu i hoʻokuʻu ʻia: Ke ana nei kahi mea kūikawā i ka pae o ke kinoea i kapa ʻia ʻo nitric oxide i ka lewa a mākou e hoʻokuʻu aku ai. He haʻahaʻa loa nā pae o kēia kinoea i ka poʻe me PCD.
• Nā hoʻāʻo hana pulmonary: Nānā kēia mau hoʻokolohua i ka hana maikaʻi o kou mau māmā a inā he pilikia kou hanu ʻana.
• Microscopy wikiō: Lawe ʻia kahi laʻana o ka cilia a nānā ʻia ma o kahi microscope i lako me kahi kāmela wikiō mana kiʻekiʻe. ʻAe kēia i ka cilia e nānā ʻia me ka neʻe lohi, e ʻae ana i ke aʻo pololei ʻana i kā lākou hana.

Pehea e mālama ʻia ai ka dyskinesia ciliary mua (PCD)?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka PCD i kēia manawa . Eia nō naʻe, nui nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i ka maʻi, hōʻemi i nā hōʻailona, ​​a hoʻomaʻalahi iki i ke ola.

ʻO ka pahuhopu nui ka wehe ʻana i ka mucus a me ke wai e hōʻiliʻili ana i loko o nā māmā . Hana ʻia kēia ma ka hana ʻana i nā mea e like me:

• Ka hoʻomaʻemaʻe ʻana o ke ala ea: Kōkua kahi mīkini kūikawā i ka wehe ʻana a me ka wehe ʻana i ka mucus. Aia kekahi mau hana ʻū kūikawā e kōkua ai i ka hoʻomaʻemaʻe ʻana i ka mucus.
• Hoʻōla kino umauma: Hoʻohana kekahi poʻe i kahi mea e like me ke kapa komo e pā mālie ana i ka umauma. Kōkua kēia i ka hoʻokuʻu ʻana i ka mucus a kōkua iā ia e puka i waho.
• Nā ʻōmole pepeiao: Inā loaʻa iā ʻoe nā maʻi pepeiao pinepine, hiki ke hoʻokomo ʻia nā ʻōmole liʻiliʻi i loko o ka eardrum e hoʻokahe i ka wai e hōʻiliʻili ana i ka pepeiao waena.

Eia kekahi, hāʻawi ʻia nā lāʻau lapaʻau e like me kēia e kāohi i nā maʻi a me ka mumū:

• Nā lāʻau antibiotic: Hāʻawi ʻia kēia mau mea e kāohi i nā maʻi bacteria i loko o ke kino. I kekahi mau hihia koʻikoʻi, pono paha e hāʻawi ʻia nā lāʻau antibiotic ma o ka intravenous.
• ʻAzithromycin: He lāʻau lapaʻau kēia e kōkua ai i ka hōʻemi ʻana i ka mumū i loko o nā māmā, a i kekahi manawa lawe ʻia i kēlā me kēia lā.
• Nā Bronchodilators: Nā lāʻau lapaʻau e like me albuterol. Hoʻonui iki kēia i nā ala ea, e maʻalahi ai ka hanu ʻana.
• Corticosteroids: Hoʻomalu kēia i nā hopena kemika i loko o ke kino a hōʻemi i ka mumū.
• Nā mea hoʻomāmā i ka mucus: Kōkua kēia mau lāʻau lapaʻau i hanu ʻia e hoʻomāmā i ka mucus i loko o nā alaloa a maʻalahi hoʻi e hoʻokuke aku.

E pono anei iaʻu nā lāʻau lapaʻau PCD hou aku?

I kekahi manawa, e like me ka mea i ʻōlelo ʻia ma luna, inā hewa nā wahi o loko, pono paha ke ʻoki ʻana e hoʻoponopono iā lākou.Hiki ke hana ʻia kēia mau ʻoki ma hope koke o ka hānau ʻana o ka pēpē. Eia kekahi, pono paha nā ʻoki hou aʻe ma hope o ke ola e pale aku ai a mālama paha i nā pilikia e kū mai ana.

Hiki ke pale ʻia ka dyskinesia ciliary mua (PCD)?

ʻOiai he maʻi hoʻoilina kēia, ʻaʻohe mea hiki iā mākou ke hana e pale aku ai . Eia nō naʻe, inā loaʻa i kahi lālā pili o ka ʻohana ka PCD a ke noʻonoʻo nei ʻoe e hoʻomaka i kahi ʻohana, he manaʻo maikaʻi e noʻonoʻo i ka hoʻāʻo hoʻoilina a me ke aʻoaʻo hoʻoilina . Hiki i kēia mau lawelawe ke kōkua iā ʻoe e ʻike i ka nui o ka hiki iā ʻoe a i ʻole kāu keiki ke loaʻa i ke kūlana.

He aha ke kūlana o ke ola no ka poʻe me PCD?

Ola ka hapa nui o nā kānaka i ke ola lōʻihi, a hiki i ka wā ʻelemakule . ʻO ka mea nui loa, ʻo ia ka loaʻa ʻana o ka lāʻau kūpono a mālama iā ʻoe iho.

He aha ka wānana no ka poʻe me PCD?

ʻOkoʻa maoli kēia mai kēlā kanaka a kēia kanaka . Maʻi kekahi poʻe mai ka lā i hānau ʻia ai lākou a noho mau paha pēlā i ko lākou ola a pau. Hiki i kekahi ke olakino maikaʻi i ko lākou manawa kaʻawale a laila maʻi nui i kekahi manawa. No laila, he mea nui loa e kamaʻilio me kāu kauka e pili ana i kou kūlana a hahai i kāna ʻōlelo aʻo.

He aha nā pilikia e hiki ke loaʻa mai ka PCD?

Ma muli o ka pinepine ʻana o nā maʻi, hiki iā lākou ke hōʻino i nā ʻiʻo o nā ʻōpū, hana i nā ʻeha, a alakaʻi i nā ʻano pilikia like ʻole. Eia kekahi laʻana:

• Ka nalowale o ka lohe .
• Ka hoʻonui ʻana a me ka hōʻino ʻana i nā alaloa ea (bronchiectasis) : Hiki i kēia ke hōʻino hou aku i nā māmā.
• Ka hāʻule ʻana o ka hanu : Hiki ke hana ʻia kēia ʻano ma nā hihia koʻikoʻi loa.

He aha nā mea nui e ʻike ai i ka wā e noho ai me PCD?

He maʻi mau ka PCD. No laila, i ka wā e noho pū ai me ia, hiki iā ʻoe ke hoʻomaʻalahi iki i kou ola a hana i kēia mau mea e noho olakino ai:

• Hoʻoikaika Kino: Ke hana ʻoe i ka hana kino kaulike, hanu hohonu ʻoe. Kōkua kēia i ka hoʻokuʻu ʻana i ka mucus i loko o kou mau māmā a kōkua iā ʻoe e hanu maʻalahi. Hoʻonui pū ia i kou pae ikehu.
• Kākoʻo naʻau: ʻO ka noho ʻana me kahi maʻi mau e like me kēia hiki ke paʻakikī i ka naʻau i kekahi manawa. Hiki ke loaʻa nā pilikia. No laila, ʻo ka loaʻa ʻana o ke kākoʻo mai kahi loea olakino noʻonoʻo, e like me ke kākāʻōlelo, hiki ke hele lōʻihi i ka hōʻemi ʻana i ke kaumaha āu e manaʻo nei.
• Nānā mau ʻana i ke kauka: Pono ʻoe e ʻike pinepine i kāu kauka e ʻike i ka maikaʻi o kāu lāʻau lapaʻau, inā e ʻoi aku ka ʻino o kāu mau hōʻailona, ​​a inā paha e ulu ana nā pilikia hou. Pono paha ʻoe e loaʻa i nā hoʻokolohua hana pulmonary maʻamau a i ʻole nā ​​​​X-ray umauma.

ʻO ka mea nui loa, ʻaʻole e hopohopo ke ʻike ʻoe he PCD kou, akā e hahai pono i nā ʻōlelo a kāu kauka a mālama iā ʻoe iho.

Leka Lawe-Home

ʻO ka Primary Ciliary Dyskinesia (PCD) kahi maʻi genetic kakaikahi e hoʻopilikia nui ana i ka ʻōnaehana hanu a me nā ʻōnaehana kino. Hiki ke ʻoi aku ka ʻino o nā hōʻailona i ka hala ʻana o ka manawa, a hiki ke loaʻa nā pilikia e like me ka hāʻule ʻana o ka hanu.

Eia nō naʻe, ʻoiai ʻaʻohe lāʻau lapaʻau no kēia, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona a hōʻemi i ka pilikia o ka maʻi . Hiki i nā mea e like me ka hoʻomaʻemaʻe ʻana i ke ala ea a me nā lāʻau lapaʻau ke hāʻawi i ka hōʻoluʻolu nui. He mea nui hoʻi e ʻimi i ke kākoʻo noʻonoʻo e kōkua i ka hōʻemi ʻana i ke kaumaha o ka noho ʻana me kahi maʻi mau e like me kēia. ʻAʻole ʻoe hoʻokahi, a aia nā kauka a me nā poʻe ʻē aʻe e hiki ke kōkua iā ʻoe.

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