ʻO Cystic fibrosis

ʻO Cystic fibrosis kahi maʻi e hoʻoulu ai i ka mānoanoa, pākuʻi ka mucus e kūkulu i loko o nā akemāmā, nā wahi digestive, a me nā wahi ʻē aʻe o ke kino. ʻO ia kekahi o nā maʻi maʻi maha mau loa i nā keiki a me nā ʻōpio ʻōpio. He maʻi hoʻoweliweli ia i ke ola.

ʻO Cystic fibrosis (CF) kahi maʻi i hoʻoili ʻia i waena o nā ʻohana. Hoʻokumu ʻia ia e kahi ʻāpana kīnā ʻole e hana i ke kino e hoʻohua i ka wai kūlohelohe a paʻa ʻole, i kapa ʻia he mucus. Kūkulu kēia mucus i nā hanu hanu o nā akemāmā a me nā pancreas.

ʻO ke kūkulu ʻia ʻana o nā mucus nā hopena i nā maʻi lolo i hoʻoweliweli i ke ola a me nā pilikia digestion koʻikoʻi. Hiki paha i ka maʻi ke hoʻopili i nā glands a me ka ʻōnaehana hānau o ke kāne.

Hāpai ka poʻe he nui i ka ʻāpana CF, akā ʻaʻohe o lākou mau ʻōuli. ʻO kēia no ka mea pono i kahi kanaka me CF ke hoʻoilina i nā ʻāpana hemahema 2, 1 mai kēlā me kēia makua. Loaʻa i kekahi poʻe ʻAmelika ka ʻāpana CF. ʻOi aku ka maʻamau i waena o nā poʻe o ka ʻohana ʻĀkau a waena paha o Europa.

ʻO ka hapa nui o nā keiki me CF e ʻike ʻia e ka makahiki 2, keu hoʻi e hana ʻia ka loiloi hānau hou ma waena o ʻAmelika Hui Pū ʻIa. No kahi helu liʻiliʻi, ʻaʻole ʻike ʻia ka maʻi a hiki i ka makahiki 18 a ʻoi paha. ʻO kēia mau keiki he milder ke ʻano o ka maʻi.

Pākuʻi ʻia paha nā ʻōuli ma nā pēpē hou:

• Hoʻonui lohi
• ʻO ka loaʻa ʻole o ke kaumaha i ka wā kamaliʻi
• ʻAʻohe neʻe o ka ʻōpū i 24 mua a 48 mau hola o ke ola
• ʻIli ʻono paʻakai

Pākuʻi ʻia paha nā ʻōuli e pili ana i ka ʻōpū.

• ʻEha ʻōpū mai ka paʻa paʻa paʻa
• Hoʻonui i ke kinoea, ʻōhū, a i ʻole kahi ʻōpū e ʻōhū.
• Nusea a me ka lilo o ka makemake
• Nā pale o nā pale a ʻeleʻele paha, pilau, pilau, a lana paha
• Kaumaha emi

Pākuʻi ʻia paha nā ʻōuli e pili ana i nā akemāmā a me nā sinus.

• Coughing a hoʻonui ʻia paha ka mucus i nā sinus a i ʻole nā ​​ake māmā
• Ka luhi
• Hoʻopili ʻia ka ihu e nā nasal polyps
• Nā paukū hou o ka pulumonia (nā ʻōuli o ka pulumonia i kekahi me ka cystic fibrosis me ka fiva, ka hoʻonui ʻana i ka ʻū a me ka hanu nui, hoʻonui ʻia ka mucus, a me ka make ʻole o ka makemake)
• ʻO ka ʻeha sinus a i ʻole kaomi i hoʻokumu ʻia e ka maʻi a i ʻole nā ​​polyps

Nā ʻōuli e ʻike ʻia ma hope paha i ke ola:

• Infertility (i nā kāne)
• Repeated inflammation of the pancreas (pancreatitis)
• Nā ʻōnaehana hanu
• ʻO nā manamana lima paʻa

Hana ʻia kahi hōʻoia koko e kōkua e ʻike iā CF. Nānā ka hōʻike no nā loli i ka ʻāpana CF. ʻO nā hoʻokolohua ʻē aʻe i hoʻohana ʻia e kuhikuhi iā CF e komo pū me:

• ʻO ka hoʻāʻo Immunoreactive trypsinogen (IRT) kahi hōʻike loiloi ʻōnaehana hānau hou no CF. Kuhi kahi pae kiʻekiʻe o IRT hiki i CF a koi aku i ka hoʻāʻo hou.
• ʻO ka ho'āʻo sweat chloride ka hōʻoia diagnostical maʻamau no CF. ʻO kahi pae paʻakai kiʻekiʻe i ka hou o ke kanaka kahi hōʻailona o ka maʻi.

ʻO nā hoʻokolohua ʻē aʻe e hōʻike ana i nā pilikia i hiki ke pili iā CF me:

• Pākuʻi x-ray a i ʻole CT scan
• Hōʻike momona momona
• Nā hoʻāʻo hana lung
• Ka ana o ka hana pancreatic (pahu pancreatic elastase)
• Hōʻike hoʻonāukiuki Secretin
• ʻO Trypsin a me chymotrypsin i ka noho
• ʻO GI luna a me nā pūʻali ʻōpū liʻiliʻi
• ʻO nā moʻomeheu lepa (loaʻa ʻia e ka sputum, bronchoscopy a i ʻole ka swab ākā)

Hiki i kahi hōʻailona mua o ka CF a me ka hoʻolālā lapaʻau ke hoʻomaikaʻi i ke ola a me ka maikaʻi o ke ola. He mea nui ka ukali a me ka nānā ʻana. Ke hiki, pono e mālama ʻia ma kahi haukapila kūikawā cystic fibrosis. Ke hiki i nā keiki i ke ʻano makua, pono lākou e hoʻololi i kahi kikowaena kūikawā cystic fibrosis no nā mākua.

ʻO ka mālama ʻana i nā pilikia o ka mumū:

• Nā Antibiotics e pale a mālama i nā maʻi maʻi a me nā sinus. Lawe ʻia paha lākou e ka waha, a i ʻole hāʻawi ʻia i nā aa a i ʻole i nā hanu hanu ʻana. Hiki paha i ka poʻe me CF ke ʻai i nā lāʻau antibiotic ke pono, a i nā manawa āpau paha. ʻOi aku ka kiʻekiʻe o nā hana ma mua o ka maʻamau.
• Hoʻopili ʻia nā lāʻau i hoʻōla ʻia e kōkua i ka wehe ʻana i nā alaloa.
• ʻO nā lāʻau ʻē aʻe i hāʻawi ʻia e ka hanu lapaʻau ʻana i ka mucus lahilahi a maʻalahi hoʻi i ka ʻūlū ʻana he DNAse enzyme therapy a me nā haʻuki paʻakai paʻa nui ʻia (saline hypertonic).
• ʻO ka lāʻau lapaʻau Flu a me ka lāʻau vaccine polysaccharide pneumococcal (PPV) i kēlā me kēia makahiki (e nīnau i kāu mea mālama ola).
• ʻO ke kanu ʻana o ka ʻōpū kahi koho i kekahi mau hihia.
• Pono paha ka oxygen therapy i ka maʻi o ka maʻi māmā.

Mālama pū ʻia nā pilikia o ka lepa me nā hoʻoponopono e lahilahi i ka mucus. ʻOi aku ka maʻalahi o kēia e kahe i ka huhū mai nā akemāmā.

ʻO kēia mau ʻano hana:

• ʻO ka hana a hoʻoikaika paha e hoʻoulu nui ai kou hanu
• Hoʻohana ʻia nā hāmeʻa i ka lā e kōkua i ka hoʻomaʻemaʻe i nā kahawai o ka nui o ka mucus
• ʻO ka percussion umauma lima (a i ʻole ka physiotherapy umauma), kahi o ka ʻohana a i ʻole ka mea hoʻomaʻamaʻa e paʻi liʻiliʻi i ka umauma o ke kanaka, hoʻi, a me kahi ma lalo o nā lima.

ʻO ka mālama ʻana no ka ʻōpū a me nā pilikiaʻai hiki ke hoʻopili ʻia:

• ʻO kahi papaʻai kūikawā i kiʻekiʻe i ka protein a me nā calorie no nā keiki nui a me nā mākua
• ʻO nā enzymes Pancreatic e kōkua i ke komo ʻana i nā momona a me nā protein, i lawe ʻia me kēlā me kēia pāʻina
• ʻO nā mea kōkua Vitamin, ʻo ka vitamina A, D, E, a me K
• Hiki i kāu mea hoʻolako ke ʻōlelo aku i nā lāʻau lapaʻau ʻē aʻe inā loaʻa ʻoe nā ʻauamo paʻakikī loa

ʻO Ivacaftor, lumacaftor, tezacaftor, a me elexacaftor nā lāʻau e mālama i kekahi ʻano o CF.

• Hoʻomaikaʻi lākou i ka hana o kekahi o nā ʻāpana hemahema i kumu iā CF.
• A i 90% o nā mea maʻi me CF a kūpono i hoʻokahi a ʻoi paha o kēia mau lāʻau.
• A ʻo kahi hopena, ʻoi aku ka liʻiliʻi o ke kūkulu ʻia ʻana o nā mucus mānoanoa i nā akemāmā. Hoʻonui maikaʻi ʻia nā ʻōuli CF ʻē aʻe.

Pono e mālama a me ka nānā ʻana i ka home me:

• ʻO ka pale ʻana i ka uahi, ka lepo, ka lepo, ka uahi, nā kemikū o ka hale, ka uahi kapuahi, a me ka punahelu a me ka momona.
• Ke hāʻawi nei i nā wai he nui, keu hoʻi i nā pēpē a me nā keiki i ka wā wela, ke loaʻa ka maʻi palahī a i ʻole nā ​​noho hakahaka, a i ʻole ka wā hoʻoikaika kino.
• Hoʻomaʻamaʻa 2 a 3 mau manawa i kēlā me kēia pule. ʻO ka ʻauʻau ʻana, ka jogging ʻana, a me ke kaʻa kaʻa kaʻa he koho maikaʻi loa.
• ʻO ka hoʻomaʻemaʻe a lawe ʻana mai paha i nā mucus a i ʻole nā ​​huna mai nā alaloa. Pono e hana i kēia i ka 4 i kēlā me kēia lā. Pono nā mea maʻi, ʻohana, a me nā mea mālama e aʻo e pili ana i ka hana ʻana i ka pahu pahu a me ke kahawai postural e kōkua ai i ka hoʻomaʻemaʻe o nā ala ea.
• ʻAʻole pili ʻia me ka poʻe ʻē aʻe me CF e hiki ke hoʻololi i nā maʻi (ʻaʻole pili i nā lālā o ka ʻohana).

Hiki iā ʻoe ke hoʻomāmā i ke koʻikoʻi o ka maʻi ma ke komo ʻana i kahi hui kākoʻo cystic fibrosis. ʻO ke kaʻana ʻana me nā poʻe ʻē aʻe i loaʻa nā ʻike a me nā pilikia maʻamau hiki ke kōkua i kou ʻohana e noʻonoʻo ʻole.

Noho ka hapa nui o nā keiki me CF i ke olakino maikaʻi a hiki i ko lākou wā makua. Hiki iā lākou ke komo i ka hapa nui o nā hana a hele i ke kula. Nui nā ʻōpio ʻōpio me CF e hoʻopau i ke koleke a loaʻa paha nā hana.

Ma hope loa mai e maʻi ka maʻi puʻu a hiki i kahi e kīnā ai ke kanaka. I kēia lā, ʻo ka awelika o ke ola no nā poʻe me CF e ola nei a hiki i ke ʻano makua ma kahi o 44 mau makahiki.

Hoʻokumu pinepine ʻia ka make e nā ʻōpū māmā.

ʻO ka hoʻopiʻi pinepine ʻia ka maʻi hanu maʻi mau.

ʻO nā pilikia ʻē aʻe:

• ʻO nā pilikia o ka ʻōpū, e like me nā gallstones, blockage ʻōpū, a me ka prolaps prolal
• ʻApu ʻana i ke koko
• Hanakahi hanu ʻole
• Diabetes
• Hoʻoipo
• ʻO ka maʻi akepaʻa a me ke kī ʻole o ke ake, pancreatitis, biliary cirrhosis
• Kaʻai pono ʻole
• Nasal polyps a me sinusitis
• ʻO Osteoporosis a me ka maʻi kō
• ʻO Poniaonia e hoʻi mau nei
• Pneumothorax
• Hoka puʻuwai ʻaoʻao ʻākau (cor pulmonale)
• Maʻi ʻaʻai kala

Kāhea i kāu mea hāʻawi inā he pēpē a keiki paha kahi ʻōuli o CF, a me nā ʻike.

• Fiva, hoʻonui i ka ʻū, hoʻololi i ka sputum a i ʻole ke koko i ka sputum, nalo o ka makemake ʻole, a i ʻole nā ​​hōʻailona ʻē aʻe o ka niumonia.
• Hoʻonui i ka pohō kaumaha
• ʻO ka neʻe pinepine ʻana o ka ʻōpū a i ʻole nā ​​pahu i hohono a pilau a ʻoi aku paha nā mucus
• ʻO ka ʻōpū pehu a hoʻonui paha i ka bloating

Kāhea i kāu mea hāʻawi inā loaʻa kahi kanaka me CF i nā ʻōuli hou a i ʻole inā hele a ʻoi aku ka maikaʻi o nā ʻōuli, e like me ka paʻakikī o ka hanu ʻana a i ʻole ke kuʻu ʻana i ke koko.

ʻAʻole hiki ke pale ʻia ʻo CF. Ke nānā nei i ka poʻe me ka mōʻaukala ʻohana o ka maʻi hiki ke ʻike i ka ʻāpana CF i nā halihali he nui.

CF

• Kaʻai hānai komo - keiki - mālama ʻana i nā pilikia
• Tube hānai Gastrostomy - bolus
• Pehea e hanu ai inā pōkole ʻoe o ka hanu
• Paipu hānai Jejunostomy
• Kahe hoʻoheheʻe hope

• Pāʻālua
• Kahe hoʻoheheʻe hope
• ʻO nā manamana lima paʻa
• ʻO Cystic fibrosis

Donaldson SH, Pilewski JM, Griese M, et al. ʻO Tezacaftor / ivacaftor i nā kumuhana me ka cystic fibrosis a me F508del / F508del-CFTR a i ʻole F508del / G551D-CFTR. ʻO Am J Respir Crit Care Med. 2018; 197 (2): 214-224. PMID: 28930490 pubmed.ncbi.nlm.nih.gov/28930490/.

ʻO Eagan ME, Schechter MS, Voynow JA. ʻO Cystic fibrosis. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 432.

Farrell PM, White TB, Ren CL, et al. ʻIke o ka cystic fibrosis: nā ʻikepili aelike mai ka Cystic Fibrosis Foundation. J Pediatr. 2017; 181S: S4-S15.e1. PMID: 28129811 pubmed.ncbi.nlm.nih.gov/28129811/.

Graeber SY, Dopfer C, Naehrlich L, et al. Nā hopena o ka lumacaftor / ivacaftor therapy ma ka hana CFTR ma Phe508del nā mea maʻi homozygous me ka fibrosis cystic. ʻO Am J Respir Crit Care Med. 2018; 197 (11): 1433-1442. PMID: 29327948 pubmed.ncbi.nlm.nih.gov/29327948/.

ʻO Grasemann H. Cystic fibrosis. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 83.

ʻO Rowe SM, Hoover W, Solomon GM, Sorscher EJ. ʻO Cystic fibrosis. I: Broaddus VC, Mason RJ, Ernst JD, et al, eds. ʻO Murray a me Nadel's Textbook of Respiratory Medicine. ʻElua huaʻōlelo Piladelapia, PA: Elsevier Saunders; 2016: chap 47.

ʻO Taylor-Cousar JL, Munck A, McKone EF, et al. ʻO Tezacaftor-ivacaftor i nā maʻi me ka homozygous cystic fibrosis no ka phe508del. N Engl J Med. 2017; 377 (21): 2013-2023. PMID: 29099344 pubmed.ncbi.nlm.nih.gov/29099344/.