He aha ka Cystic Fibrosis? E hoʻomaopopo maʻalahi kākou!

Ua lohe paha ʻoe ua hānau ʻia kekahi poʻe me kahi maʻi hoʻoilina, ʻeā? He maʻi hoʻoilina ka Cystic Fibrosis (CF). Manaʻo ka poʻe he maʻi kēia e hoʻopilikia wale ana i nā māmā, no ka mea, he mea maʻamau ka pilikia o ka hanu ʻana a me nā maʻi māmā pinepine me kēia maʻi. Akā, ʻoi aku ka paʻakikī o ka moʻolelo ma mua o kēlā. E nānā kākou i ke ʻano o ia mea.

He aha maoli ka cystic fibrosis (CF)?

I ka ʻōlelo maʻalahi, ʻo ka cystic fibrosis (CF) kahi maʻi genetic e hoʻoulu ai i ka nui o ka mucus mānoanoa a pipili e hōʻiliʻili i loko o kekahi mau ʻōkana i loko o ko kākou kino. Hiki i kēia mucus mānoanoa ke hōʻino i kēlā mau ʻōkana a hoʻopilikia i kā lākou hana.

ʻO ka maʻamau, he wai iki a lahilahi ka mucus i loko o ko kākou mau māmā a me ka ihu. Mālama ia i kēlā mau wahi i ka pulu a hoʻopaʻa i ka lepo a me nā ʻōpala. Akā i nā poʻe me CF, hoʻololi kahi hoʻololi genetic i ke ʻano o ka hana ʻana o kēia mucus. Ua nalowale kahi protein nona kēia a i ʻole ʻaʻole hana pono. ʻO ka hopena, ua paʻa nā paʻakai e hoʻomāmā ai i ka mucus i loko o nā cell, e hoʻolilo ana i ka mucus i mānoanoa a pipili loa.

ʻOiai he nui ka poʻe e manaʻo nei he maʻi māmā ia, loaʻa iā CF kona inoa no ka mea ke kumu ia i nā cysts a me nā scarring (fibrosis) i loko o ka pancreas. ʻO kēia pōʻino, me ka mānoanoa o ka lining, hiki ke ālai i nā ducts e hoʻokuʻu i nā enzymes digestive e kōkua i ka hoʻoheheʻe ʻana i ka meaʻai a mākou e ʻai ai. Hiki i kēia ke pale i ke kino mai ka omo pono ʻana i nā meaola mai ka meaʻai. Ma waho aʻe o nā māmā a me ka pancreas, hiki iā ia ke hoʻopilikia i ke akepaʻa, nā sinuses, nā ʻōpū, a me nā ʻōpū moekolohe.

He maʻi hoʻoilina ka CF i hānau ʻia me ke kanaka. ʻO ke ʻano kēia he maʻi ia no ke ola holoʻokoʻa e hiki ke ʻoi aku ka ʻino i ka hala ʻana o ka manawa. ʻOi aku ka pōkole o ke ola o ka poʻe me CF ma mua o ka poʻe me ka ʻole o CF.

Aia kekahi mau ʻano nui o ka cystic fibrosis (CF)?

ʻAe, ʻelua mau ʻano nui o `CF` hiki ke ʻike ʻia:

1. ʻO ka cystic fibrosis maʻamau: Hoʻopilikia pinepine kēia i nā ʻāpana he nui i loko o ke kino. Hoʻomaopopo pinepine ʻia ke ʻano i loko o nā makahiki mua o ke ola.

2. ʻO ka cystic fibrosis atypical: He ʻano CF ʻoi aku ka liʻiliʻi o ka ʻino a ʻoluʻolu hoʻi. Hiki ke hoʻopilikia i hoʻokahi wale nō ʻōkana, a i ʻole e hele mai a hele aku nā hōʻailona. ʻIke pinepine ʻia ia i nā keiki makua a i ʻole nā ​​​​mākua ʻōpio.

He aha nā hōʻailona o ka cystic fibrosis (CF)?

Hiki i kekahi kanaka me CF ke loaʻa nā hōʻailona e like me:

• Nā maʻi maʻi pinepine o ka māmā (e like me ka pneumonia hou a i ʻole bronchitis)). E noʻonoʻo, loaʻa mau i kekahi mau keiki ʻōpio ka ihu wai, ka ʻū, a me ke kani ʻana o ka hanu i loko o ko lākou umauma, a me ka lāʻau lapaʻau, hoʻi hou mai lākou me ka ʻole o ka hōʻoluʻolu. Pēlā nō ia.
• Ka hele ʻana i nā ʻōpala palahalaha a ʻaila paha.
• Paʻakikī ka hanu ʻana.
• He leo pinepine a haʻalulu ka wheezing e puka mai ana mai ka umauma mai.
• Nā maʻi sinus pinepine.
• ʻŪhū mau.
• Lohi ka ulu ʻana.
• ʻO ka holomua ʻole, ʻoiai ʻoe e ʻai maikaʻi ana a loaʻa iā ʻoe nā calorie e pono ai kou kino, he ʻano ia kahi ʻaʻole ʻoe e piʻi i ke kaumaha a wīwī paha.

Nā hōʻailona o ka cystic fibrosis atypical (Atypical CF)

Hiki i nā poʻe me ka CF atypical ke loaʻa kekahi o nā hōʻailona i ʻōlelo ʻia ma luna. I ka hala ʻana o ka manawa, hiki iā lākou ke ʻike i nā mea e like me:

• Sinusitis mau loa.
• Nā polyps ihu.
• ʻO nā pae electrolyte kūpono ʻole i ke kino e hiki ke alakaʻi i ka dehydration a i ʻole ka heatstroke.
• Ka maʻi pākī.
• ʻO ka maʻi pankreatitis.
• Ka pohō kaumaha i manaʻo ʻole ʻia.

He aha ke kumu o ka cystic fibrosis (CF)?

ʻO ke kumu nui o CF he mutation (variant a mutation paha) i loko o kahi gene i kapa ʻia ʻo CFTR. Hana ka gene CFTR i kahi protein e hana ana ma ke ʻano he kahawai ion ma luna o ka ʻili o nā cell. E noʻonoʻo iā ia e like me kahi puka liʻiliʻi i loko o ka membrane cell. ʻAe kēia mau puka i kekahi mau ʻāpana e hele ma waena.

ʻO ka protein i hana ʻia e ka gene `CFTR` e hana maʻamau ma ke ʻano he puka no nā ion chloride (kahi ʻano paʻakai me ka uku uila maikaʻi ʻole). Ke haʻalele nā ​​ion chloride i ke kelepona, huki ʻia ka wai. ʻO kēia ka manawa e lahilahi ai ka mucus a pahee. Akā i ka poʻe me `CF`, ʻaʻole hana pono kēia kaʻina hana ma muli o nā mutations i loko o ka gene `CFTR`. A laila paʻa nā ion chloride i loko o nā kelepona, e waiho ana i ka mucus mānoanoa a pipili.

Aia nā ʻano hoʻololi gene CFTR like ʻole (nā papa I a VI). ʻAʻole hana kekahi mau hoʻololi i kekahi protein, hana kekahi i kahi liʻiliʻi wale nō o ka protein, a ʻaʻole hana kekahi i ka protein a lākou e hana ai.

He maʻi hānau anei ka cystic fibrosis (CF)?

ʻAe, he maʻi hoʻoilina ʻo `CF` i hoʻoilina ʻia mai ka hānau ʻana mai. Loaʻa i ke kanaka me `CF` ʻelua kope o kēia gene `CFTR` i hoʻololi ʻia - hoʻokahi mai ka makuahine, a hoʻokahi mai ka makuakāne (ua kapa ʻia kēia he hoʻoilina `autosomal recessive` ).

ʻAʻole pono kou mau mākua e loaʻa iā CF no ʻoe e loaʻa ai iā CF. ʻO ka ʻoiaʻiʻo, ʻaʻohe moʻolelo ʻohana o CF i ka hapa nui o nā ʻohana. ʻO ke kanaka nona hoʻokahi kope wale nō o ka gene mutated ua kapa ʻia he mea lawe . ʻAʻole hōʻike nā mea lawe i nā hōʻailona o CF.

Hiki i nā pākeke ke loaʻa i ka cystic fibrosis (CF)?

ʻAʻole, ua hānau ʻia ʻoe me ka hoʻololi ʻana o ka gene e hoʻoulu ai i ka CF. Eia nō naʻe, inā he ʻoluʻolu loa nā hōʻailona, ​​a inā hele mai a hele aku lākou, ʻaʻole paha e ʻike ʻia kekahi poʻe a hiki i ka wā ma hope o ke ola, a hiki paha i ka wā makua.

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

Hiki i ka CF ke hana i nā pilikia e like me:

• Nā maʻi: Hoʻopaʻa ka mucus mānoanoa i nā bacteria i loko o kou mau māmā a me nā ala ea, e paʻakikī ai iā lākou ke puka i waho. Hiki i kēia ke alakaʻi i nā maʻi pinepine.
• ʻO ka ʻole o ka vas deferens ma waena o nā ʻōpū hānau (CBAVD): Ma kēia kūlana, ʻaʻohe o nā kāne ka vas deferens, ka ʻōmole e lawe ana i ka sperm. No laila, pono lākou i ke kōkua me nā lāʻau lapaʻau hānau keiki inā makemake lākou e loaʻa nā keiki.
• Maʻi diabetes: Hiki ke hana i ka maʻi diabetes e pili ana i ka cystic fibrosis ma muli o ka hōʻino ʻana i ka pancreas.
• ʻAi ʻole: ʻO ka nele o ka mucus mānoanoa i loko o ka ʻōnaehana hoʻoheheʻe a me nā enzymes pancreatic e kōkua i ka hoʻoheheʻe ʻana i ka meaʻai e hoʻonui ai i ka pilikia o ka ʻai ʻole.
• Osteopenia a me Osteoporosis: Hiki ke ulu aʻe nā maʻi hoʻonāwaliwali i ka iwi ma muli o ka hiki ʻole ke omo pono i nā meaola mai ka ʻōnaehana hoʻoheheʻe.
• Nā pilikia hāpai: Hiki i ka CF ke hoʻopilikia i ka ʻōnaehana hoʻoheheʻe a hoʻoulu i ka ʻai ʻole. Hoʻonui kēia i ka pilikia o nā pilikia hāpai. ʻO ka hānau mua ka pilikia maʻamau.

Pehea e ʻike ʻia ai ka cystic fibrosis (CF)?

ʻO ka maʻamau, nānā nā kauka i nā pēpē hānau hou no CF i ka wā o ka nānā ʻana i nā pēpē hānau hou. Hana ʻia kēia ma ka lawe ʻana i kekahi mau kulu koko mai ke kuʻekuʻe wāwae o ka pēpē. Ma ka hale hana, hoʻāʻo ʻia kēia hāpana koko no kahi kemika i kapa ʻia ʻo immunoreactive trypsinogen (IRT) . Hana ʻia kēia e ka pancreas. ʻOi aku ka kiʻekiʻe o ka IRT i loko o ke koko o ka poʻe me CF. Hoʻāʻo ʻia nā pēpē no IRT i ka wā hānau a ma hope o kekahi mau pule.

Eia nō naʻe, hiki i kekahi mau kūlana, e like me ka hānau mua ʻana, ke kumu o nā pae IRT kiʻekiʻe. No laila, ʻaʻole manaʻo kahi hoʻāʻo IRT maikaʻi he CF ka pēpē. Inā ʻoi aku ke kiʻekiʻe o ka pae IRT o ka pēpē ma mua o ka mea i manaʻo ʻia, e kauoha ke kauka i nā hoʻokolohua hou aʻe e hana i kahi hōʻailona hope loa.

I kekahi manawa (ma kahi o 5% o nā hihia), ʻaʻole paha e ʻike ka nānā ʻana i nā keiki hānau hou i nā pae IRT kiʻekiʻe i loko o kekahi me CF. A i ʻole, ʻaʻole paha ʻoe i hoʻāʻo mau ʻia no CF i ka wā hānau. Inā loaʻa iā ʻoe a i ʻole kāu pēpē nā hōʻailona o CF, e hana ke kauka i kahi hoʻāʻo hou a, inā pono, nā hoʻāʻo ʻē aʻe.

Nā hoʻāʻo no ka cystic fibrosis (CF)

• Hoʻāʻo hou:Ke ana kēia i ka nui o ka chloride i loko o kou hou. ʻOi aku ka kiʻekiʻe o ka chloride i loko o ka hou o ka poʻe me CF. ʻO kēia ka hoʻāʻo kikoʻī loa no ka ʻike ʻana i ka CF. Eia nō naʻe, hiki ke maʻamau i ka poʻe me ka CF atypical.
• Nā hoʻokolohua genetic: Lawe ʻia nā hāpana koko e nānā i nā loli i loko o nā genes e hoʻoulu ai iā CF.
• Nā hoʻāʻo kiʻi: Hoʻohana ʻia nā mea e like me nā X-ray o ka lua ihu a me ka umauma e hōʻoia a kākoʻo paha i ka hōʻailona o CF. ʻAʻole hiki i kēia mau hoʻokolohua kiʻi wale nō ke hōʻoia i ka CF.
• Nā hoʻāʻo hana pulmonary (PFTs): Ke ana kēia i ka maikaʻi o ka hana ʻana o kou mau māmā.
• Moʻomeheu sputum: E lawe kāu kauka i kahi laʻana o ka mucus e puka mai ana mai kou mau māmā ke ʻū ʻoe a hoʻāʻo iā ia no ka bacteria. ʻO kekahi mau ʻano bacteria, e like me Pseudomonas, ʻoi aku ka maʻamau i nā poʻe me CF.
• ʻO ka biopsy pancreatic: Hiki i kāu kauka ke ʻike inā he mau cysts a i ʻole nā ​​​​​​lesions i loko o kāu pancreas.
• ʻOkoʻa hiki i ka ihu (NPD): Ke ana kēia i ka uku uila liʻiliʻi e kū mau ana ma ka ʻili o ka ihu. Hoʻokumu ʻia kēia uku e ka neʻe ʻana o nā ion. ʻO ka poʻe me CF he emi ka neʻe ʻana o nā ion ma muli o ke ʻano o ka hopena o CF i nā kahawai ion.
• Ana o ke au o ka ʻōpū (ICM): No ka hana ʻana i kēia hoʻāʻo, lawe ke kauka i kahi laʻana o ka ʻiʻo rectal. Ana ke keʻena hoʻokolohua i ka nui o ka chloride i hoʻokuʻu ʻia mai kēia laʻana.

Pehea e mālama ʻia ai ka cystic fibrosis (CF)?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no CF. Eia nō naʻe, me ke kōkua o kahi loea CF a me nā poʻe ʻē aʻe ma kāu hui mālama ola, hiki iā ʻoe ke hoʻokele i ka maʻi a me kāna mau hōʻailona. ʻO kēia hoʻokele ʻana:

• Ka mālama ʻana i nā ala ea i maʻemaʻe a wehe ʻia ma o ka hoʻohana ʻana i nā ʻano hanu a me nā mea hana hoʻoheheʻe phlegm.
• Nā lāʻau lapaʻau e kōkua ai i ka hoʻoponopono ʻana i nā pilikia me ka protein `CFTR` (`CFTR modulators`).
• Nā lāʻau lapaʻau e hōʻemi ana i nā hōʻailona kikoʻī.
• E hōʻoia i ka lawa ʻana o nā calorie mai ka meaʻai a me ka nui kūpono.
• ʻOki kino.

Nā ʻano hana hoʻomaʻemaʻe alaea

Inā loaʻa iā ʻoe ka CF, aia kekahi mau ala e mālama ai i kou mau ala ea:

• ʻO ke ʻano o ke kūpeʻe a me ka hanu ʻana: Hiki i kahi mea hoʻōla kino i loea i ka CF ke aʻo iā ʻoe i nā ʻano hana e wehe ai i kou mau ala ea a wehe i ka mucus.
• Nā mea hana kaomi hoʻomaha maikaʻi (PEP):Hiki ke hoʻokomo ʻia kēia mau mea `PEP` i loko o ka waha a i ʻole he `mask` ma ka maka. Hāʻawi lākou i ke kū'ē, no laila pono ʻoe e hana ikaika e hanu i waho. Wehe kēia i nā ala ea a hoʻokuke i ka mucus i waho. ʻO nā mea `PEP` haʻalulu (e.g. `Flutter®`, `Acapella®`, `AerobikA®`, `RC-Cornet®` ) he mau ʻano kūikawā o `PEP` e hoʻohui i ka haʻalulu me ka hiki ke hoʻokuʻu i ka mucus.
• Nā ʻaʻahu komo ea: Ua kapa ʻia hoʻi he mea hoʻoluliluli i ka paia umauma alapine kiʻekiʻe, he ʻaʻahu komo hoʻoulu kēia i hoʻopili ʻia i kahi mīkini. Haʻalulu ka ʻaʻahu komo a wāwahi i ka mucus.
• ʻO ka hoʻokahe ʻana o ke kino a me ka percussion: He ʻano hana hoʻōla kino kēia. Neʻe ʻoe i nā kūlana e kōkua ai i ka hoʻomaʻemaʻe ʻana i ka mucus mai kou mau māmā. Hoʻopā kekahi kanaka i kou umauma a/a i ʻole ke kua e kōkua i ka wehe ʻana i ka mucus. Hiki ke hana ʻia kēia me nā ʻano hana e hoʻoulu ai i ka ʻū.

Nā modulators CFTR no ka cystic fibrosis

ʻO nā modulators CFTR kahi papa o nā lāʻau lapaʻau e kōkua i ka hoʻoponopono ʻana i nā pilikia me nā protein i hana ʻia e ka gene CFTR i hoʻololi ʻia a hoʻonui i ka nui o ka protein e hana pono ana ma luna o nā cell. ʻAʻole lākou e ho'ōla i ka CF. Akā ua loaʻa i kekahi poʻe nā hoʻomaikaʻi koʻikoʻi i ko lākou mau hōʻailona a me ka lōʻihi o ke ola. Eia naʻe, ʻaʻole kūpono kēia mau lāʻau modulator no nā mea āpau me CF, a ʻaʻole hiki i kekahi ke ʻae iā lākou.

ʻO kekahi mau laʻana o nā `CFTR modulators`:

• ʻO Kalydeco® (ivacaftor)
• ``Orkambi® (ivacaftor/lumacaftor)''
• `Symdeko® (ivacaftor/tezacaftor)`
• ʻO Trikafta® (ivacaftor/tezacaftor/elexacaftor)`

Nā lāʻau lapaʻau ʻē aʻe no ka cystic fibrosis

Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau ʻē aʻe e hōʻemi i ka mumū, mālama i nā maʻi, a hoʻokele paha i nā hōʻailona. ʻO kēia mau mea:

• Nā lāʻau lapaʻau: Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e mālama a pale aku paha i nā maʻi.
• Nā bronchodilators i hanu ʻia: Hoʻomaha nā bronchodilators i nā alaloa, e maʻalahi ai ka hanu ʻana.
• ʻO ka saline hypertonic i hanu ʻia: Hoʻowalewale ka paʻakai i loko o nā hopena saline i ka wai, ka mea e hoʻomāmā ai i ka mucus a maʻalahi ai ke kuʻi ʻana.
• Nā lāʻau anti-inflammatory: Hoʻemi kēia mau lāʻau i ka pehu. ʻO kēia mau mea nā corticosteroids a me nā lāʻau anti-inflammatory nonsteroidal (NSAIDS) .
• Nā enzyme pancreatic: Kōkua kēia i ka hoʻoheheʻe ʻana i ka meaʻai a hoʻopili i nā meaola mai ia mea.
• Nā mea palupalu noho: Kōkua kēia i nā kūlana e like me ka paʻa ʻana o ka ʻōpū a maʻalahi hoʻi ke hele ʻana i ka ʻōpala.

ʻAi no ka cystic fibrosis (CF)

Inā loaʻa iā ʻoe ka CF, ʻokoʻa kāu mau pono meaʻai mai ko kekahi me ka ʻole o ka CF. Ma muli o CF, ʻaʻole hiki i kāu pancreas ke hana a hūnā paha i nā enzymes e kōkua i ka hoʻoheheʻe ʻana i ka meaʻai. ʻO ke ʻano kēia, ʻaʻole hiki i kou ʻōpū ke omo pono i nā meaola a me ka momona mai ka meaʻai.

E paipai aku kāu loea CF a i ʻole ka meaʻai meaʻai i kākau inoa ʻia i kahi papahana meaʻai nou. Hiki ke komo pū me:

• ʻO ka lawe ʻana i nā calorie i kēlā me kēia lā. Hiki i kēia ke ʻelua ka nui o ka nui ma mua o ka mea me ka ʻole o CF.
• ʻAi ʻana i ka meaʻai kiʻekiʻe i ka momona. He mea nui kēia no ka loaʻa ʻana o nā huaora momona hou aʻe.
• Ka mālama ʻana i ke kaumaha o ke kino ma mua o ka maʻamau mai kou wā kamaliʻi. Hiki i kēia ke kōkua iā ʻoe e ulu aʻe a hoʻonui i kou mau māmā. Hiki i kēia ke kōkua me nā hōʻailona i kou wā ʻelemakule.
• ʻO ka lawe ʻana i nā capsules hoʻohui enzyme. Kōkua nā mea hoʻohui enzyme i ka ʻai ʻana.
• Hoʻohui i ka paʻakai hou aʻe i kāu meaʻai. Kōkua kēia i ka hoʻololi ʻana i ka paʻakai keu i nalowale i kou kino ma o ka hou ʻana. He mea nui loa kēia i ka wā wela a pulu a i ka wā e hoʻoikaika kino ai. E nīnau i kāu kauka e pili ana i ka nui o ka paʻakai āu e pono ai i kēlā me kēia lā.

ʻO ke ʻoki kino no ka cystic fibrosis (CF)

Pono paha ʻoe i ke ʻoki ʻana no CF a i ʻole kahi pilikia o CF. Hiki i kēia ke komo pū me:

• Nā ʻoki kino e pili ana i ka ihu a i ʻole nā ​​​​sinus.
• ʻO ke ʻoki kino e wehe i nā mea ālai ʻōpū.
• Hoʻoili ʻana o ka māmā.
• Hoʻoili ʻana o ke akepaʻa.

He maʻi hoʻoweliweli anei ka cystic fibrosis (CF) i ke ola?

ʻAe, he maʻi hoʻoweliweli ke CF i ke ola. ʻO ke kumu nui o ka make ʻana, ʻo ia ka hōʻino ʻia o ka māmā i hana ʻia e ka mucus mānoanoa a me nā maʻi pinepine o ka māmā.

He aha ke kūlana o ke ola o kekahi kanaka me ka cystic fibrosis (CF)?

Wahi a nā loea, i nā makahiki i hala iho nei, ʻo ke ola o ka mea i hānau ʻia me CF ma kahi o 50 mau makahiki. I kekahi mau makahiki i hala aku nei, ʻo ke ola ma waena o 30 a me 40 mau makahiki, akā ua piʻi aʻe kēia i nā makahiki i hala iho nei ma muli o ka holomua o nā ʻano lapaʻau.

ʻOi aku ka lōʻihi o ke ola o ka poʻe me ka CF atypical ma mua o ka poʻe me ka CF maʻamau.

He aha kaʻu e manaʻo ai inā loaʻa iaʻu ka CF?

ʻAʻohe lāʻau lapaʻau no CF. Pono ʻoe a i ʻole kāu keiki i ka lāʻau lapaʻau no ke ola holoʻokoʻa e mālama ai. ʻO kēia ka mālama ʻana i nā maʻi, ka mālama ʻana i ka meaʻai, a me ka ʻike pinepine ʻana i kahi loea CF. Eia nō naʻe, ua kōkua nā lāʻau lapaʻau hou i nā keiki me CF e ola maikaʻi a hiki i ka wā makua a loaʻa i kahi ola maikaʻi aʻe.

Hana maikaʻi loa ka lāʻau lapaʻau ke ʻike mua ʻia ʻo CF. ʻO ia ke kumu he mea nui ka nānā ʻana i nā keiki hānau hou. ʻO ka hoʻomaka ʻana i nā lāʻau lapaʻau e like me nā modulators CFTR i ka wā ʻōpiopio hiki ke hoʻomaikaʻi i ke olakino lōʻihi a hoʻonui i ka manaʻolana o ke ola.

Hiki ke pale ʻia ke CF?

No ka mea, he mea ia i hānau ʻia me ʻoe ʻo CF, ʻaʻohe ala e pale aku ai. Inā he mea lawe ʻoe i kahi hoʻololi gene CFTR, hiki iā ʻoe ke nīnau i kāu kauka e pili ana i ka hoʻāʻo genetic prenatal a me ka hiki ke loaʻa i kāu mau keiki ʻo CF.

Pehea e hiki ai iaʻu ke mālama iaʻu iho?

ʻO ka mālama ʻana iā ʻoe iho me CF ʻo ia hoʻi ka hana pū ʻana me kāu hui mālama ola e hoʻomohala i kahi hoʻolālā lapaʻau. I mea e noho olakino ai, pono ʻoe e hahai pono i kēia hoʻolālā. Loaʻa iā ia:

• E hahai pono i kāu hana hoʻomaʻemaʻe hanu.
• Ke lawe nei i nā lāʻau lapaʻau e like me ke kauoha.
• E hoʻomau i ka hele ʻana i nā keʻena lapaʻau me kāu hui lapaʻau CF.

E kiʻi i nā ʻōlelo aʻoaʻo mai kāu mau kauka e pili ana i kahi hoʻolālā ʻai olakino a me ka hana kino palekana e kūpono iā ʻoe. E nīnau i kāu kauka inā he manaʻo maikaʻi ka hoʻoponopono hou ʻana i ka pulmonary nou.

Hiki iā ʻoe ke hōʻemi i kou pilikia o ka maʻi ma ka pale ʻana i ka poʻe maʻi, ka hoʻomaʻamaʻa ʻana i nā ʻano holoi lima maikaʻi, a me ka loaʻa ʻana o nā lāʻau pale i ʻōlelo ʻia.

Hiki iā ʻoe ke komo i nā hoʻokolohua lapaʻau e hoʻāʻo ana i nā lāʻau lapaʻau hou no CF. E nīnau i kāu kauka inā aia kekahi mau mea kūpono iā ʻoe. E hōʻoia e loaʻa ka ʻike piha e pili ana i nā pono a me nā pilikia o nā hoʻokolohua lapaʻau.

Āhea wau e ʻike ai i kaʻu kauka?

E hele i nā keʻena lapaʻau āpau i hoʻonohonoho ʻia me nā lālā o kāu hui mālama ola. E kamaʻilio me kāu kauka inā he mau nīnau kāu e pili ana i kāu hoʻolālā lapaʻau a i ʻole kāu mau hōʻailona. E nīnau iā lākou i ka mea e hana ai inā loaʻa iā ʻoe nā hōʻailona o kahi maʻi. Hiki iā ʻoe ke haʻi iā lākou inā makemake ʻoe i ke kōkua me nā pilikia pili kanaka a i ʻole ka naʻau.

Āhea wau e hele ai i ka ʻOihana Pōpilikia (ETU) ?

Inā loaʻa iā ʻoe kēia mau hōʻailona koʻikoʻi, e hele koke i ka lumi pilikia:

• Ke kuni nui (ma luna o 103 degere Fahrenheit/40 degere Celsius).
• Paʻakikī ka hanu ʻana.
• ʻAʻohe a i ʻole ka liʻiliʻi loa o ka mimi i hoʻopuka ʻia.
• ʻEha mau ma ka umauma a i ʻole ka ʻōpū.
• Pōʻaiʻī.
• Ka huikau.
• ʻEha nui a nāwaliwali paha o nā ʻiʻo.
• Nā ʻōʻili.
• Polū ke kala o ka ʻili, nā lehelehe a i ʻole nā ​​​​kui lima (`cyanosis` - hiki ke hōʻailona kēia o ka haʻahaʻa o ka oxygen i loko o kou koko a i ʻole nā ​​​​ʻiʻo).
• Inā hoʻomaikaʻi a nalowale paha ke kuni a i ʻole ke kole, a laila e ʻino hou aku.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Hiki iā ʻoe ke nīnau i kāu kauka i nā nīnau penei:

• He aha nā koho lapaʻau e loaʻa iaʻu?
• He aha kahi papa ʻai olakino e hiki iaʻu ke hahai?
• He aha kaʻu e hana ai e mālama i kaʻu mau hōʻailona?
• He aha nā hōʻailona o ka maʻi e pono iaʻu e makaʻala?
• Āhea wau e ʻike hou ai iā ʻoe?
• He aha nā hōʻailona e pono ai iaʻu e hele i ka lumi pilikia?
• Makemake ʻoe e nānā i nā lālā ʻē aʻe o ka ʻohana?

No ke aha e pono ʻole ai i nā poʻe me CF ke hoʻopā kekahi i kekahi?

ʻO ka maʻamau, ʻōlelo nā loea olakino i ka poʻe me ka cystic fibrosis (CF) e pale i ka launa pū ʻana me nā poʻe ʻē aʻe. ʻO kēia no ka mea hiki i ka poʻe me CF ke loaʻa maʻalahi i nā maʻi i hiki i nā poʻe ʻē aʻe ke kaohi maʻalahi. ʻOi aku ka nui o ko lākou hiki ke hoʻolaha i nā germs i nā poʻe ʻē aʻe me CF (nāna hoʻi nā maʻi i paʻakikī ke kaohi). Pono hoʻi ka poʻe me CF e pale i kekahi mea maʻi.

ʻO ka hope loa, kekahi mea (Leka Lawe-Home)

He mea maʻamau ke kaumaha iki i ka wā e ʻike ʻia ai ʻoe he maʻi no ke ola holoʻokoʻa. Eia nō naʻe, ʻo nā lāʻau lapaʻau hou a me ka ʻike maikaʻi ʻana i ka cystic fibrosis ʻo ia hoʻi he nui kou manawa e lawe ai i nā mea i hoʻokahi lā i ka manawa. Hiki i nā poʻe he nui me CF ke manaʻo e ola piha i ke ola a hiki i ka wā makua. ʻO kahi keiki i ʻike ʻia me CF i kēia lā, ʻoi aku ka nui o nā koho lapaʻau i ka wā e hiki mai ana.

E hōʻuluʻulu i kahi hui o nā mea aloha a me nā loea lapaʻau āu e hilinaʻi nei e hoʻomaopopo i ka mea e manaʻo ai, e kōkua iā ʻoe e hoʻoponopono i nā pilikia o ke ola o kēlā me kēia lā. A, mai makaʻu e kiʻi i kahi manaʻo ʻelua i kēlā me kēia manawa ma ke ala. ʻAʻole ʻoe hoʻokahi, a he nui ka poʻe e hiki ke kōkua iā ʻoe ma o kēia huakaʻi.

ʻO Cystic fibrosis, nā maʻi genetic, nā maʻi māmā, mucus, gene CFTR, olakino o nā keiki, nā pilikia hanu