Respiratory muscle strength and quality of life in children and adolescent with cystic fibrosis
Keywords:
Cystic Fibrosis, Muscle Strength, Quality of LifeAbstract
Cystic fibrosis (CF) is a multisystemic genetic disease characterized by chronic obstruction that, associated with other pulmonary changes, can compromise respiratory muscle strength (RMS) and, consequently, interfere with the performance of typical childhood activities, changing the quality of life(QOL) of this population. The aim of the study was to evaluate the relationship between RMS and QOL of children and adolescents with CF. This is a cross-sectional study, which included patients without acute pulmonary exacerbation, aged between 6 and 14 years, from a reference center in Brazil. Anthropometric and RMS assessments were performed, using maximum inspiratory (MIP) and expiratory (MEP) pressures using digital manovacuometry (Globalmed® MVD300). The Cystic Fibrosis Questionnaire was applied, a specific questionnaire to assess QOL in this disease, in versions for children (QOL-C) and for parents or guardians (QOL-P). The severity of the disease was classified according to the Schwachman Doeurshuk score (ESD). Data on colonization and genotype were consulted through the analysis of medical records. The data was analyzed using the SPSS version 20.0 for Windows software. After the Shapiro-Wilkt test, Pearson’s or Spearman’s correlation test was applied. Throughout the analysis, a significance level of 5% was adopted. Twenty-eight children (15 boys) participated in the study, with a mean age of 10.10 ± 1.79 years, who had a near-predicted RMS and QOL scores indicating good QOL. The ESD was negatively related to the digestive domain (p=0.03; rho=-0.400). MEP showed a negative correlation with the QOL-P body domain (p=0.002; rho=-0.426) and with the QOL-C treatment domain (p=0.01; rho=-0.453). MIP showed a positive correlation with the physical (p=0.03; rho=0.410), emotional (p=≤0.001; rho=0.573) and treatment (p=≤0.01; rho=-0.605) domains of the QOL-C. MIP also showed a positive correlation with the respiratory domain (p=0.01; rho=0.572) of the QOL-P. In conclusion, an association was identified between QOL and RMS domains, as well as with ESD and nutritional aspects. This sample showed higher than expected RMS values and good QOL.
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References
Reyna SL, Holbrook J, Griffiths HHJ, Peckham D, Mcdermott MF.
Dysregulated signalling pathways in innate immune cells with
cystic fibrosis mutations. Cell Mol Life Sci. 2020;77:4485-503.
doi: 10.1007/s00018-020-03540-9
Fiorotto R, Strazzabosco M. Cystic fibrosis-related liver diseases:
new paradigm for treatment based on pathophysiology. Clin
Liver Dis. 2016;8(5):113-6. doi: 10.1002/cld.583
Cutting GR. Cystic fibrosis genetics: from molecular
understanding to clinical application. Nat Rev Genet.
;16(1):45-56. doi: 10.1038/nrg3849
Grupo brasileiro de estudos de fibrose cística. Registro Brasileiro
de Fibrose Cística [Internet]. São Paulo: GBEFC, 2017 [cited
Mar 10]. Available from: http://portalgbefc.org.br/ckfinder/
userfiles/files/REBRAFC_2017.pdf
Leroy S, Perez T, Neviere R, Aguilaniu B, Wallaert B. Determinants
of dyspnea and alveolar hypoventilation during exercise in
cystic fibrosis: impact of inspiratory muscle endurance. J Cyst
Fibros. 2011;10(3):159-65. doi: 10.1016/j.jcf.2010.12.006
Donadio MVF, Heinzmann-Filho JP, Vendrusculo FM, Frasson
PXH, Marostica PJC. Six-Minute walk test results predict risk of
hospitalization for youths with cystic fibrosis: a 5-year follow-up
study. J Pediatr. 2017;182:204-9. doi: 10.1016/j.jpeds.2016.11.071
Saglam M, Vardar-Yagli N, Savci S. Six minute walk test versus
incremental shuttle walk test in cystic fibrosis. Pediatr Int.
;58(9):887-93. doi: 10.1111/ped.12919
Dassios T, Katelari A, Doudounakis S, Dimitriou G. Aerobic
exercise and respiratory muscle strength in patients with
cystic fibrosis. Respir Med. 2013;107(5):684-90. doi: 10.1016/j.
rmed.2013.01.016
Vendrusculo FM, Heinzmann-Filho JP, Piva TC, Marostica PJC,
Donadio MVF. Inspiratory muscle strength and endurance
in children and adolescents with cystic fibrosis. Respir Care.
;61(2):184-91. doi: 10.4187/respcare.04231
Cohen MA, Ribeiro MÂGO, Ribeiro AF, Ribeiro JD, Morcillo AM.
Avaliação da qualidade de vida de pacientes com fibrose cística
por meio do Cystic Fibrosis Questionnaire. J Bras Pneumol.
;37(2):184-92. doi: 10.1590/S1806-37132011000200008
Athanazio RA, Vicente L, Ferreira R, Ribeiro AF, Riedi CA,
Procianoy EFA, et al. Diretrizes brasileiras de diagnóstico e
tratamento da fibrose cística. J Bras Pneumol. 2017;43(3):219-45.
doi: 10.1590/S1806-37562017000000065
Santos CIS, Ribeiro JD, Ribeiro AF, Hessel G. Análise crítica dos
escores de avaliação de gravidade da fibrose cística: estado
da arte. J Bras Pneumol. 2004;30(3):286-98. doi: 10.1590/
S1806-37132004000300016
Ramsey B, Boat T. Outcome measures for clinical trials in cystic
fibrosis Summary of a Cystic Fibrosis Foundation Consensus
Conference. J Pediatr. 1994;124(2):177-92. doi: 10.1016/
S0022-3476(94)70301-9
Kanga J, Kuhn R, Craigmyle L, Haverstock D, Church D. Cystic
fibrosis clinical score: a new scoring system to evaluate
acute pulmonary exacerbation. Clin Ther. 1999;21(8):1343-56.
doi: 10.1016/S0149-2918(99)80035-6
Doershuk CF, Matthews LRW, Tucker AS, Nudelman H, Eddy G,
Wise M, et al. A 5 year clinical evaluation of a therapeutic program
for patients with cystic fibrosis. J Pediatr. 1964;65(5):677-93.
doi: 10.1016/S0022-3476(64)80152-9
Biblioteca virtual em saúde. Programa Telessaúde Brasil.
Calculadora IMC infantil [Internet]. Brasília (DF): Ministério
da Saúde; [modified 2009 Jul 7; cited 2021, Mar 10]. Available
from: http://www.telessaudebrasil.org.br/apps/calculadoras
Rozov T, Cunha MT, Nascimento O, Quittner AL, Jardim
JR. Linguistic validation of cystic fibrosis quality of life
questionnaires. J Pediatr. 2006;82(2):151-6. doi: 10.2223/
JPED.1463
Gibson GJ, Whitelaw W, Siafakas N. ATS/ERS Statement
on respiratory muscle testing. Am J Respir Crit Care Med.
;166(4):518-624. doi: 10.1164/rccm.166.4.518
Laveneziana P, Albuquerque A, Aliverti A, Babb T, Barreiro
E, Dres M, et al. ERS statement on respiratory muscle testing
at rest and during exercise. Eur Respir J. 2019;53(6):1801214.
doi: 10.1183/13993003.01214-2018
Rosa GJ, Morcillo AM, de Assumpção MS, Schivinski CIS.
Predictive equations for maximal respiratory pressures of
children aged 7-10. Brazilian J Phys Ther. 2017;21(1):30-6.
doi: 10.1016/j.bjpt.2016.04.002
Domènech-Clar R, López-Andreu JA, Compte-Torrero L, DiegoDamiá D, Macián-Gisbert V, Perpiña-Tordera M, et al. Maximal
static respiratory pressures in children and adolescents. Pediatr
Pulmonol. 2003;35(2):126-32. doi: 10.1002/ppul.10217
Munro BH. Statistical methods for health care research.
Philadelphia: Lippincott; 2005.
Magnet FS, Callegari J, Dieninghoff D, Spielmanns M, Storre JH,
Schmoor C, et al. Impact of Pseudomonas aeruginosa infection
on respiratory muscle function in adult cystic fibrosis patients.
Respiration. 2016;93(1):42-50. doi: 10.1159/000452893
Dunnink MA, Doeleman WR, Trappenburg JCA, de Vries WR.
Respiratory muscle strength in stable adolescent and adult
patients with cystic fibrosis. J Cyst Fibros. 2009;8(1):31-6.
doi: 10.1016/j.jcf.2008.07.006
Dassios TG, Katelari A, Doudounakis S, Dimitriou G. Chronic
Pseudomonas aeruginosa infection and respiratory muscle
impairment in cystic fibros. Respir Care. 2014;59(3):363-70.
doi: 10.4187/respcare.02549
Hahn A, Ankermann T, Claass A, Mann M, Lindemann H,
Neubauer BA. Non-invasive tension time index in relation
to severity of disease in children with cystic fibrosis. Pediatr
Pulmonol. 2008;43(10):973-81. doi: 10.1002/ppul.20887
Vandeleur M, Walter LM, Armstrong DS, Robinson P, Nixon
GM, Horne RSC. Quality of life and mood in children with
cystic fibrosis: associations with sleep quality. J Cyst Fibros.
;17(6):811-20. doi: 10.1016/j.jcf.2017.11.021
Santos SMR, Duarte TR, Barroso MD, Jesus MCP. Vivências
dos familiares frente à criança com fibrose cística. J Heal Sci.
;19(2):89-94. doi: 10.17921/2447-8938.2017v19n2p89-94
Bodnár R, Kádár L, Szabó L, Hernádi M, Mikóczi M, Mészáros Á.
Health related quality of life of children with chronic respiratory
conditions. Adv Clin Exp Med. 2015;24(3):487-95. doi: 10.17219/
acem/24991
Quittner AL, Goldbeck L, Abbott J, Duff A, Lambrecht P, Solé
A, et al. Prevalence of depression and anxiety in patients with
cystic fibrosis and parent caregivers: results of the international
depression epidemiological study across nine countries. Thorax.
;69(12):1090-7. doi: 10.1136/thoraxjnl-2014-205983
Knudsen KB, Pressler T, Mortensen LH, Jarden M, Skov M,
Quittner AL, et al. Associations between adherence, depressive
symptoms and health-related quality of life in young adults
with cystic fibrosis. Springerplus. 2016;5(1). doi: 10.1186/s40064-016-2862-5
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