Peak cough flow in children and young people with spinal muscular atrophy type II and type III

Authors

  • Carla Peixoto Vinha de Souza Unversidade Federal do Rio de Janeiro UFRJ. RJ. Brasil
  • Regina Kátia Cerqueira Ribeiro Instituto Benjamin Constant (IBC), Departamento de Educação do Rio de Janeiro RJ. Brasil
  • Luana do Valle Lima Hospital dos Servidores do Estado do Rio de Janeiro https://orcid.org/0000-0002-2201-1419
  • Clemax Couto Sant’Anna Universidade Federal do Rio de Janeiro. Faculdade de Medicina. RJ. Brasil https://orcid.org/0000-0001-8732-8065
  • Alexandra Prufer de Queiroz Campos Araújo Universidade Federal do Rio de Janeiro. Faculdade de Medicina. RJ. Brasil

DOI:

https://doi.org/10.1590/1809-2950/18002025042018

Keywords:

Spinal Muscular Atrophy, Expiratory Peak Flow, Cough

Abstract

Spinal muscular atrophy is a neurodegenerative disorder, which may be associated with progressive respiratory failure. Our aim is to describe the peak cough flow of children and young people with spinal muscular atrophy types II and III. This is a descriptive, cross-sectional study conducted at a neuropediatrics outpatient clinic between March 2011 and May 2012, with patients with spinal muscular atrophy types II and III, and aging more than 5 years. Out of the 53 eligible patients, 21 participated in the research. The measurement of peak cough flow was carried out through the peak flow meter, with patients sitting and lying down. After taking three measures, we selected the one with the highest value among them. Type-III individuals reached peak cough flow values higher than those of type-II individuals. Measures taken in the sitting position (SMA II 159.4 l/min; SMA III 287.9 l/min) were higher than those measured in the lying position (SMA II 146.9 l/min; SMA III 257.5 l/min), with significant difference (p-value=0.008 in sitting position, and p=0.033 in lying position). We concluded that individuals with SMA III manifest higher PCF, especially when sitting, in comparison with SMA II.

Downloads

Download data is not yet available.

Author Biographies

  • Carla Peixoto Vinha de Souza, Unversidade Federal do Rio de Janeiro UFRJ. RJ. Brasil

    Mestre em Ciências pelo Programa de Pós-Graduação em Clínica Médica da Faculdade de Medicina da Universidade Federal do Rio de Janeiro (UFRJ)

  • Regina Kátia Cerqueira Ribeiro, Instituto Benjamin Constant (IBC), Departamento de Educação do Rio de Janeiro RJ. Brasil

    Mestre em Saúde Materno-Infantil pela Universidade Federal Fluminense (UFF), professora do Instituto Benjamin Constant (IBC), Departamento de Educação do Rio de Janeiro

  • Luana do Valle Lima, Hospital dos Servidores do Estado do Rio de Janeiro

    Fisioterapeuta do Hospital dos Servidores do Estado do Rio de Janeiro

  • Clemax Couto Sant’Anna, Universidade Federal do Rio de Janeiro. Faculdade de Medicina. RJ. Brasil

    Professor associado do Departamento de Pediatria da Faculdade de Medicina e docente dos programas de pós-graduação de Clinica Médica e de Doenças Infecciosas e Parasitárias da Universidade Federal do Rio de Janeiro (UFRJ)

  • Alexandra Prufer de Queiroz Campos Araújo, Universidade Federal do Rio de Janeiro. Faculdade de Medicina. RJ. Brasil

    Pós-doutora, professora associada de Neuropediatria, Departamento de Pediatria da Faculdade de Medicina da Universidade Federal do Rio de Janeiro (UFRJ)

References

Araújo AP, Ramos VG, Cabello PH. Dificuldades diagnósticas na

atrofia muscular espinhal. Arq Neuropsiquiatr. 2005;63(1):145-9.

doi: 10.1590/S0004-282X2005000100026

Russman BS. Spinal muscular atrophy: clinical classifications

and disease heterogeneity. J Child Neurol. 2007;22(8):946-51.

doi: 10.1177/0883073807305673

Swoboda KJ, Prior TW, Scott CB, McNaught TP, Wride MC, Reyna

SP, et al. Natural history of denervation in SMA: relation to age,

SMN2 copy number, and function. Ann Neurol. 2005;57(5):704-12.

doi: 10.1002/ana.20473

Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong

B, et al. Consensus statement for standard of care in spinal

muscular atrophy. J Child Neurol. 2007;22(8):1027-49. doi:

1177/0883073807305788

Markowitz JA, Singh P, Darras BT. Spinal muscular atrophy: a

clinical and research update. Pediatr Neurol. 2012;46(1):1-12.

doi: 10.1016/j.pediatrneurol.2011.09.001

Kang SW. Pulmonary rehabilitation in patients with

neuromuscular disease. Yonsei Med J. 2006;47(3):307-14.

doi: 10.3349/ymj.2006.47.3.307

Ross BB, Gramiak R, Rahn H. Physical dynamics of the

cough mechanism. J Appl Physiol. 1955;8(3):264-8.

doi: 10.1152/jappl.1955.8.3.264

Evans JN, Jaeger MJ. Mechanical aspects of coughing.

Pneumonologie. 1975;152(4):253-7.

Sharma GD. Pulmonary function testing in neuromuscular

disorders. Pediatrics. 2009;123(Suppl 4):S219-21.

doi: 10.1542/peds.2008-2952D

Freitas FS, Parreira VF, Ibiapina CC. Aplicação clínica do pico de

fluxo da tosse: uma revisão de literatura. Fisioter Mov Curitiba.

;23(3):495-502. doi: 10.1590/S0103-51502010000300016

King M, Brock G, Lundell C. Clearance of mucus by

simulated cough. J Appl Physiol. 1985;58(6):1776-82.

doi: 10.1152/jappl.1985.58.6.1776

Iannaccone ST. Modern management of spinal muscular atrophy. J

Child Neurol. 2007;22(8):974-8. doi: 10.1177/0883073807305670

Gozal D. Pulmonary manifestations of neuromuscular disease

with special reference to Duchenne muscular dystrophy and

spinal muscular atrophy. Pediatr Pulmonol. 2000 Feb;29(2):141-

doi: 10.1002/(SICI)1099-0496(200002)29:23.0.CO;2-Y

Chung BH, Wong VC, Ip P. Spinal muscular atrophy: survival

pattern and functional status. Pediatrics. 2004;114(5):e548-53.

doi: 10.1542/peds.2004-0668

Chatwin M, Bush A, Simonds AK. Outcome of goal-directed noninvasive ventilation and mechanical insufflation/exsufflation in

spinal muscular atrophy type I. Arch Dis Child. 2011;96(5):426-32.

doi: 10.1136/adc.2009.177832

Salam A, Tilluckdharry L, Amoateng-Adjepong Y,

Manthous CA. Neurologic status, cough, secretions and

extubation outcomes. Int Care Med. 2004;30(7):1334-9.

doi: 10.1007/s00134-004-2231-7

Mier-Jedrzejowicz A, Brophy C, Green M. Respiratory muscle

weakness during upper respiratory tract infections. Am Rev

Respir Dis. 1988;138(1):5-7. doi: 10.1164/ajrccm/138.1.5

Ishikawa Y, Bach JR, Komaroff E, Miura T, JacksonParekh R. Cough augmentation in Duchenne muscular

dystrophy. Am J Phys Med Rehabil. 2008;87(9):726-30.

doi: 10.1097/PHM.0b013e31817f99a8

Bach JR. Management of patients with neuromuscular disease.

Philadelphia: Hanley & Belfus; 2004. 350 p.

Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity

for patients with Duchenne muscular dystrophy. Chest. 1997

Oct;112(4):1024-8. doi: 10.1378/chest.112.4.1024

Suárez AA, Pessolano FA, Monteiro SG, Ferreyra G, Capria ME,

Mesa L, et al. Peak flow and peak cough flow in the evaluation

of expiratory muscle weakness and bulbar impairment in

patients with neuromuscular disease. Am J Phys Med Rehabil.

;81(7):506-11. doi: 10.1097/00002060-200207000-00007

Pereira CAC, Jansen JM, Barreto SSM, Marinho J, Sulmonett

N, Dias RM, et al. Espirometria. J Pneumol. 2002;28(Suppl.

:S1-82.

Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R,

Coates A, et al. Standardisation of spirometry. Eur Respir J.

;26(2):319-38. doi: 10.1183/09031936.05.00034805

Bach JR, Saporito LR. Criteria for extubation and tracheostomy

tube removal for patients with ventilatory failure: a

different approach to weaning. Chest. 1996;110(6):1566-71.

doi: 10.1378/chest.110.6.1566

Bianchi C, Baiardi P. Cough peak flows: standard values for

children and adolescents. Am J Phys Med Rehabil. 2008;87:461-7.

doi: 10.1097/PHM.0b013e318174e4c7

Kang SW, Kang YS, Moon JH, Yoo TW. Assisted cough

and pulmonary compliance in patients with Duchenne

muscular dystrophy. Yonsei Med J. 2005;46(2):233-8. doi:

3349/ymj.2005.46.2.233

Summerhill EM, El-Sameed YA, Glidden TJ, McCool FD.

Monitoring recovery from diaphragm paralysis with ultrasound.

Chest. 2008 Mar;133(3):737-43. doi: 10.1378/chest.07-2200

Fromageot C, Lofaso F, Annane D, Falaize L, Lejaille

M, Clair B, et al. Supine fall in lung volumes in the

assessment of diaphragmatic weakness in neuromuscular

disorders. Arch Phys Med Rehabil. 2001;82(1):123-8.

doi: 10.1053/apmr.2001.18053

Marques TB, Neves JC, Portes LA, Salge JM, Zanoteli E, Reed

UC. Air stacking: effects on pulmonary function in patients

with spinal muscular atrophy and in patients with congenital

muscular dystrophy. J Bras Pneumol. 2014;40(5):528-34. doi:

1590/S1806-37132014000500009

Jeong J, Yoo W. Effects of air stacking on pulmonary function

and peak cough flow in patients with cervical spinal cord injury.

J Phys Ther Sci. 2015;27(6):1951-2. doi: 10.1589/jpts.27.1951

Oskoui M, Kaufmann P. Spinal muscular atrophy.

Neurotherapeutics. 2008;5(4):499-506. doi: 10.1016/

j.nurt.2008.08.007

Swoboda KJ, Kissel JT, Crawford TO, Bromberg MB, Acsadi

G, D’Anjou G, et al. Perspectives on clinical trials in spinal

muscular atrophy. J Child Neurol. 2007;22(8):957-66. doi:

1177%2F0883073807305665

Downloads

Published

2018-11-12

Issue

Vol. 25 No. 4 (2018)

Section

Original Research

License

Copyright (c) 2018 Fisioterapia e Pesquisa

Creative Commons License

This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.

How to Cite

Peak cough flow in children and young people with spinal muscular atrophy type II and type III. (2018). Fisioterapia E Pesquisa, 25(4), 432-437. https://doi.org/10.1590/1809-2950/18002025042018