Nursing care of 7 children with cystic fibrosis

doi:10.3761/j.issn.0254-1769.2022.18.013

Abstract

Abstract:

The nursing care experience of 7 patients with cystic fibrosis is summarized in this article. The critical points of nursing care are recapitulated as followed：collecting the sweat chlorine test specimen to assist diagnosis;promoting airway secretion discharge to improve respiratory function;strengthening electrolyte monitoring to correct electrolyte disorder;promoting rational medicine use to alleviate patient symptoms;developing a nutritional plan to improve patient nutritional status;strengthening discharge education to improve patient quality of life. The result is desirable that symptoms of all patients were alleviated and they were discharged from our center after active treatment and diligent care.

Key words: Cystic Fibrosis, Gene Mutation, Pediatric Nursing

摘要：

总结7例囊性纤维化患儿的护理经验。护理要点：采集汗氯试验标本,协助诊断;促进气道廓清,改善患儿呼吸功能;加强电解质监测,及时纠正电解质紊乱;合理用药,缓解患儿症状;制订营养方案,改善患儿营养状况;加强出院宣教,提高患儿出院后生活质量。7例患儿经过积极治疗及精心护理均好转出院。

关键词: 囊性纤维化, 基因突变, 儿科护理学

YU Hong, XU Xiaofeng, YU Zhuowen, ZHANG Yurong, ZHANG Xiaolei, ZHOU Yiwen. Nursing care of 7 children with cystic fibrosis[J]. Chinese Journal of Nursing, 2022, 57(18): 2256-2260.

余红, 徐晓凤, 余卓文, 张玉蓉, 张晓蕾, 周壹文. 7例囊性纤维化患儿的护理[J]. 中华护理杂志, 2022, 57(18): 2256-2260.

References 19

[1]	De BK. Cystic fibrosis in the year 2020:a disease with a new face[J]. Acta Paediatr, 2020, 109(5):893-899. DOI URL
[2]	Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis:consensus guidelines from the Cystic Fibrosis Foundation[J]. J Pediatr, 2017, 181S:S4-S15.
[3]	高立伟, 徐保平. 关注儿童囊性纤维化[J]. 中华实用儿科临床杂志, 2018, 33(4):309-312.
	Gao LW, Xu BP. Take care of children with cystic fibrosis[J]. Chin J Appl Clin Pediatr, 2018, 33(4):309-312.
[4]	Bensliman S, Lefevre N, Duchateau J, et al. Review of the sweat test indications in a Brussels’ cystic fibrosis reference center[J]. Ann Biol Clin(Paris), 2019, 77(6):687-692.
[5]	Khan MA, Ali ZS, Sweezey N, et al. Progression of cystic fibrosis lung disease from childhood to adulthood:neutrophils,neutrophil extracellular trap(NET) formation,and NET degradation[J]. Genes (Basel), 2019, 10(3):E183.
[6]	尚云晓. 黏液-纤毛清除系统在儿童呼吸道疾病中的作用[J]. 中国实用儿科杂志, 2012, 27(4):250-253.
	Shang YX. Role of mucociliary system in the onset of acute respiratory tract infection in children[J]. Chin J Pract Pediatr, 2012, 27(4):250-253.
[7]	姜源, 王颖硕, 唐兰芳, 等. 儿童气道廓清技术的应用[J]. 中华儿科杂志, 2020, 58(8):690-693. PMID
	Jiang Y, Wang YS, Tang LF, et al. Applications of airway clearance techniques in children[J]. Chin J Pediatr, 2020, 58(8):690-693. DOI PMID
[8]	Lee AL, Button BM, Tannenbaum EL. Airway-clearance techni-ques in children and adolescents with chronic suppurative lung disease and bronchiectasis[J]. Front Pediatr, 2017, 5:2.
[9]	杨芳, 贺琳晰, 杨佳, 等. 目标性镇静护理在重症肺炎患儿床旁肺泡灌洗术中的应用[J]. 中华护理杂志, 2019, 54(1):31-35.
	Yang F, He LX, Yang J, et al. Application of targeted sedation nursing for children with severe pneumonia in bedside bronchoscopy lung lavage[J]. Chin J Nurs, 2019, 54(1):31-35.
[10]	Lahiri T, Hempstead SE, Brady C, et al. Clinical practice gui-delines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis[J]. Pediatrics, 2016, 137(4):e20151784. DOI URL
[11]	Emerson J, Rosenfeld M, McNamara S, et al. Pseudomonas aeru-ginosa and other predictors of mortality and morbidity in young children with cystic fibrosis[J]. Pediatr Pulmonol, 2002, 34(2):91-100. DOI URL
[12]	Karki K, Sigdel S, Kafle S. Is it worth adding systemic antibiotics to inhalational tobramycin therapy to treat pseudomonas infections in cystic fibrosis[J]. Cureus, 2021, 13(8):e17326.
[13]	Doull I. Cystic fibrosis 2019:year in review[J]. Paediatr Respir Rev, 2020, 35:95-98. DOI PMID
[14]	陈玲. 胰酶肠溶胶囊在治疗慢性胰腺炎引起的胰腺外分泌不足的效果分析[J]. 中华胰腺病杂志, 2016, 16(2):124-125.
	Chen L. Effect analysis of pancreatic enzyme enteric-soluble capsule in the treatment of pancreatic exocrine insufficiency caused by chronic pancreatitis[J]. Chin J Pancreatol, 2016, 16(2):124-125.
[15]	Paccou J, Fardellone P, Cortet B. Cystic fibrosis-related bone disease[J]. Curr Opin Pulm Med, 2013, 19(6):681-686.
[16]	Williams CA, Benden C, Stevens D, et al. Exercise training in children and adolescents with cystic fibrosis:theory into practice[J]. Int J Pediatr, 2010, 2010:670640.
[17]	Cystic Fibrosis Foundation. CF Foundation strongly supports COVID-19 vaccine recommendations for people living with cystic fibrosis[EB/OL].(2021-06-07)[2022-06-25].https://www.cff.org/News/News-Archive/2021/CF-Foundation-Strongly-Su-pports-COVID-19-Vaccine-Recommendations-for-People-Living-With-Cystic-Fibrosis/(Accessed).
[18]	Lara-Reyna S, Holbrook J, Jarosz-Griffiths HH, et al. Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations[J]. Cell Mol Life Sci, 2020, 77(22):4485-4503. DOI URL
[19]	曾妃, 梁江淑渊, 兰美娟. 1例肝肺联合移植术后患者的护理[J]. 中华护理杂志, 2021, 56(10):1540-1543. DOI
	Zeng F, Liang JSY, Lan MJ. Nursing care of 1 patient after combined liver and lung transplantation[J]. Chin J Nurs, 2021, 56(10):1540-1543.