ʻO ka paʻa pinepine ʻana o ka umauma, ka nele o ke kaumaha ... ʻO Cystic Fibrosis (CF) anei kēia? E makaʻala kākou i kēia.

Loaʻa mau anei i kāu keiki liʻiliʻi ka ihu paʻa? ʻAʻohe mea nui ka nui o ka meaʻai a lākou e hāʻawi ai, lilo lākou i wīwī me ka ʻike ʻole? Manaʻo paha lākou i kekahi manawa he pilikia ko lākou hanu ʻana? He mea maʻamau i nā mākua ke makaʻu i ka ʻike ʻana i kēia mau hōʻailona. He mau hōʻailona paha kēia o kahi maʻi i kapa ʻia ʻo Cystic Fibrosis (CF), kahi mea a mākou i lohe ʻole ai, akā he mea nui loa ia e ʻike. E kamaʻilio kākou e pili ana i kēia me ka makaʻu ʻole a me ka maʻalahi i kēia lā.

He aha maoli ka cystic fibrosis (CF)?

I ka ʻōlelo maʻalahi, he maʻi genetic kēia i hoʻoilina ʻia mai nā mākua mai . Aia kekahi gene kūikawā e kāohi ana i ka neʻe ʻana o ka paʻakai (chloride) a me nā wai ma o nā pūnaewele o ko kākou kino. Ua kapa ʻia kēia ʻo ka gene CFTR (cystic fibrosis transmembrane conductance regulator) . No laila, loaʻa ka maʻi CF i ka wā he kīnā a i ʻole mutation paha i loko o kēia gene.

He aha ka mea e hana ʻia ma muli o kēia kīnā? ʻO ka maʻamau, ʻo nā mea huna i loko o ko mākou kino, e like me ka mucus a me ka pus , he lahilahi a pahee. Akā i loko o ke kino o ke kanaka me CF, lilo kēia mau mea huna i mānoanoa loa, mānoanoa, a pipili .

E noʻonoʻo i ka mea e hana ʻia ke hōʻiliʻili kēia mucus mānoanoa i loko o nā māmā. Hoʻolilo ia i mea paʻakikī ke hanu, maʻalahi ka pahele ʻana o nā germs , a lilo pinepine nā maʻi . I ka hala ʻana o ka manawa, hiki i kēia ke hōʻeha nui i nā māmā, ka hāʻule ʻana o ka hanu, a me ka make.

Eia kekahi, ke ālai nei kēia mau mea huna mānoanoa i nā ducts o ka pancreas. A laila , ʻaʻole e hoʻokuʻu ʻia nā enzymes digestive e kōkua i ka hoʻoheheʻe ʻana i ka meaʻai a mākou e ʻai ai . ʻO ka hopena, loaʻa ka maʻi ʻai ʻole a me ka ulu ʻana o ka ʻōpū. Eia kekahi, hiki ke loaʻa nā maʻi ate, ka maʻi diabetes ('Cystic Fibrosis-Related Diabetes' - CFRD) a me nā pilikia hānau keiki.

Akā, mai hopohopo. I kēia lā, mahalo i nā lāʻau lapaʻau holomua, ua hoʻonui ʻia ke ola o nā poʻe maʻi CF i nā makahiki he ʻumi.

He aha nā hōʻailona o kēia maʻi?

Hiki ke ʻokoʻa nā hōʻailona o CF mai kēlā kanaka a kēia kanaka. ʻOkoʻa nō hoʻi lākou ma muli o ke koʻikoʻi o ka maʻi. E nānā kākou i nā hōʻailona nui.

ʻO ka fibrosis cystic atypical

He ʻano CF ʻoi aku ka maʻalahi. ʻIke ʻia nā hōʻailona ma hope o ke ola. I kekahi manawa hoʻokahi wale nō kino i hoʻopilikia ʻia.

He aha ke kumu o ka CF?

E like me kā mākou i kūkākūkā ai ma mua, ua hana ʻia kēia e kahi kīnā i loko o ka gene i kapa ʻia ʻo CFTR . I mea e loaʻa ai i kahi keiki kēia maʻi, pono i ke keiki ke loaʻa i kahi kope o ka gene kīnā mai ka makuahine a me ka makuakāne .

Pehea e ʻike ʻia ai ka maʻi?

He mea nui loa ka ʻike mua ʻana i kēia maʻi, no ka mea, a laila hiki ke hoʻomaka koke ʻia ka lāʻau lapaʻau a hiki ke hoʻēmi ʻia nā pilikia i ka wā e hiki mai ana. Ma Sri Lanka, ke hoʻāʻo nei kekahi mau haukapila i nā keiki no kēia i ka wā hānau.

ʻO nā hoʻokolohua nui i hoʻohana ʻia no ka ʻike ʻana i ka maʻi:

• Hoʻāʻo hou: ʻO kēia ka hoʻāʻo hilinaʻi loa e ʻike ai i ka CF.Ana kēia hoʻāʻo ʻeha ʻole i ka nui o ka paʻakai (chloride) i loko o kou hou. Inā ʻoi aku ka nui ma mua o ka maʻamau, he hōʻailona ia o CF.
• Hoʻāʻo koko: Hoʻāʻo kēia i ka pae o kahi kemika i kapa ʻia ʻo `Immunoreactive Trypsinogen (IRT)` i loko o ke koko. Kiʻekiʻe kēia pae i nā poʻe maʻi CF.
• Hoʻāʻo ʻana i ka hoʻoilina (hoʻāʻo DNA): Nānā pololei kēia i nā hemahema i loko o ka gene `CFTR`.

Ma waho aʻe o kēia mau hoʻokolohua, hiki i kāu kauka ke paipai i nā hoʻokolohua ʻē aʻe e hōʻoia i ka hōʻailona a nānā i nā pilikia. No ka laʻana, kahi X-ray umauma, nā hoʻokolohua hana pulmonary (PFTs), a me nā hoʻokolohua sputum a me ka ʻōpala.

He aha nā lāʻau lapaʻau?

No ka mea he maʻi paʻakikī ʻo CF, hāʻawi ʻia ka lāʻau lapaʻau e kahi hui loea multidisciplinary, me kahi pulmonologist, kahi physiotherapist, a me kahi meaʻai. ʻO nā pahuhopu nui o ka lāʻau lapaʻau, ʻo ia ka hoʻomaʻemaʻe ʻana i nā māmā o ka mucus, pale i nā maʻi, a hāʻawi i ka meaʻai e pono ai.

Nā lāʻau lapaʻau

Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me:

• Nā lāʻau lapaʻau antibiotic: Kāohi a mālama i nā maʻi maʻi māmā.
• Nā lāʻau lapaʻau hoʻomāmā i ka mucus: Hāʻawi ʻia kēia mau mea ma o kahi inhaler a i ʻole nebulizer. Kōkua lākou i ka hoʻomāmā ʻana i ka mucus mānoanoa a maʻalahi hoʻi ke kuni ʻana.
• Nā Bronchodilators: Hoʻonui lākou i nā alaloa a hoʻomaʻalahi i ka hanu ʻana.
• Nā mea hoʻohui enzyme pancreatic: Pono e lawe ʻia kēia mau mea me kēlā me kēia ʻai a me ka meaʻai māmā. Kōkua lākou i ka ʻai ʻana a me ka omo ʻana i nā meaʻai.
• Nā modulators CFTR: ʻO kēia nā papa lāʻau hou loa a ʻoi loa ka maikaʻi. Kōkua kēia mau lāʻau i ka hoʻihoʻi ʻana i ka hana o ka protein `CFTR` hemahema e hoʻoulu pololei ai i ka maʻi. Hiki i kēia ke hoʻomāmā i ka mucus, hoʻomaikaʻi i ka hana o ka māmā, hōʻemi i ke kuni ʻana, a hōʻemi pū i ka piʻi ʻana o ke kaumaha. ʻO `(Trikafta, Kalydeco, Orkambi)` kekahi o kēia mau ʻano lāʻau.

Nā ʻAno Hana Hoʻomaʻemaʻe Alanui (ACT)

He mau mea kēia e pono e hana ʻia i kēlā me kēia lā, ma ka liʻiliʻi ʻelua i ka lā. Kōkua lākou i ka wehe ʻana a me ka wehe ʻana i ka mucus mānoanoa i paʻa i loko o nā māmā.

• Lapaʻau kino umauma: Ke pā nei kekahi i ke kua a me ka umauma me ke ʻano mele e wehe ai i ka mucus.
• ʻO ka Vest: He vest kēia i hoʻopili ʻia i kahi mīkini kūikawā. Ke ʻaʻahu ʻia, hoʻopā nā haʻalulu alapine kiʻekiʻe i ka umauma, e hana ana i kahi hana a hoʻokuʻu i ka mucus.
• Nā mea hana PEP: ʻO ka hanu ʻana ma waho o nā mea hana liʻiliʻi e like me `(Flutter, Acapella)` e hana i ke kaomi i loko o nā māmā a kōkua i ka hoʻomaʻemaʻe ʻana i ka mucus.

Nā ʻoki kino

Pono paha kekahi mau pilikia i ke ʻoki ʻana.

• Hoʻoili māmā: He koho hope loa ke koʻikoʻi loa ka maʻi a aneane pau ka hana ʻana o nā māmā.
• Hoʻoili ʻana o ke akepaʻa: Inā ua hōʻino nui ʻia ke akepaʻa.
• ʻĒ aʻe: Nā mea e like me ka wehe ʻana i nā polyp ihu, ke hoʻokomo ʻana i kahi ʻōmole hānai i loko o ka ʻōpū e hāʻawi i ka meaʻai.

Nā pilikia hiki ke loaʻa o CF

Inā ʻaʻole mālama pono ʻia ʻo CF, hiki ke loaʻa nā pilikia like ʻole.

• Nā māmā: Ka hoʻonui mau ʻana o nā alaloa hanu (`Bronchiectasis`), ka hiolo ʻana o ka māmā (`Pneumothorax`), nā maʻi maʻi mau.
• Pancreatitis: Maʻi diabetes e pili ana i ka CF (`CFRD`).
• ʻAkepaʻa: ʻO ka ʻeha o ke akepaʻa (ʻo Cirrhosis).
• ʻōpū: Pale ʻana o ka ʻōpū, hoʻonui ʻia ka pilikia o ka maʻi ʻaʻai colon.
• ʻŌnaehana hānau: Ka hānau ʻole ʻana o ke kāne a me ka lohi o ka hānau keiki ʻana i nā wahine.
• Iwi: Ka lahilahi o nā iwi (Osteoporosis).

Nā Nīnau i Nīnau Pinepine ʻia (FAQs)