Effect and mobilization of functional safety in children with Werdnig-Hoffman Syndrome
case report
DOI:
https://doi.org/10.4181/RNC.2015.23.03.1031.06pKeywords:
Muscular Atrophy Spinal, Intensive Care Units, Physical Therapy Specialty, PediatricsAbstract
Objective. To investigate the effects and safety of functional mobilization to gain flexibility in children with Werdnig-Hoffman syndrome chronically ventilated. Method. This study is a case report. These are children with diagnosis of Werdnig-Hoffman syndrome admitted to the ICU Pediatric using invasive mechanical ventilation with tracheotomy. Children were subjected to a functional mobilization protocol for eight weeks, for 30 minutes, five times a week, based on neurodevelopmental method. Initially the flexibility was assessed by goniometric measures by bending the elbows. The safety of mobilization was assessed before, during and after the procedure by means of measurements of heart rate, respiratory and oxygen saturation. Results. We included two children with Werdnig Hoffman syndrome. In the initial evaluation child (A) at five years and the child (B) with three years possessed severe hypotonic. During functional mobilization changes in hemodynamic and ventilatory parameters were observed. Children had higher joint restriction on members of the left. After eight weeks of functional mobilization, there was a gain in joint range of motion for the child A and B for bending the elbows, knee extension and dorsiflexion of the ankle. Conclusion. Functional mobilization generated positive effects on flexibility, without negative effects on hemodynamic and respiratory parameters.
Metrics
References
Orsini M, Freitas MRG, Sá MRC, Mello MP, Botelho JP, Antonioli RS, et al. Uma revisão das principais abordagens fisioterapêuticas nas atrofias musculares espinhais. Rev Neurocienc 2008;16:46-52.
Araujo APQC, Ramos VG, Cabello PH. Dificuldades Diagnósticas da Atrofia muscular espinhal. Arq Neuropsiquiatria 2005;68:145-9. http://dx.doi.org/10.1590/S0004-282X2005000100026
Russman BS. Spinal muscular atrophy: clinical classifications and disease heterogeneity. J Child Neurol 2007;22:946-51. http://dx.doi.org/10.1177/0883073807305673
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;22:1027-49.
Baioni MTC, Ambiel CR. Atrofia muscular espinhal: Diagnóstico, Tratamento e Perspectivas Futuras. J Pediatr 2010;86:261-70. http://dx.doi.org/10.1590/S0021-75572010000400004
Cavenaghi S, Gama D, Valério NI, Marino LHC, Ramirez C. Aplicabilidade intra-hospitalar da cinesioterapia no trauma. Arq Cienc Saúde 005;12:213-5.
Stiller K. Safety issues that should be considered when mobilizing critically ill patients. Crit Care Clin 2007;23:35-53. http://dx.doi.org/10.1016/j.ccc.2006.11.005
Amidei C. Measurement of physiologic responses to mobilisation in critically ill adults. Intensive Crit Care Nurs 2012;28:58-72. http://dx.doi.org/10.1016/j.iccn.2011.09.002
Bobath K. A deficiência motora em pacientes com Paralisia Cerebral. São Paulo: Ed. Manole, 1979, 94p.10.Nepomuceno Júnior BRV, Martinez BP, Gomes Neto M. Impacto do internamento em unidade de cuidados intensivos na amplitude de movimento de pacientes graves: estudo piloto. Rev Bras Ter Inten 2014;26:65-70. http://dx.doi.org/10.5935/0103-507X.20140010
Choong K, Koo KK, Clark H, Chu R, Thabane L, Burns KE, et al. Early mobilization in critically ill children: a survey of Canadian practice. Crit Care Med 2013;41:1745-53. http://dx.doi.org/10.1097/CCM.0b013e318287f592
Marques AP. Manual de Goniometria. São Paulo: Manole, 2003, 136p.
Plasencia CAC, Mendoza RC, Núñez RL, Piñón ARP, Vargas OS. Enfermedad de werdnig-hoffman: a proposito de un caso con estadia prolongada. Rev Haban Cienc Med 2009;8:pag.
Martinez BP, Bispo AO, Duarte ACM, Gomes Neto M. Declínio funcional em uma Unidade de Terapia Intensiva (UTI). Rev Inspirar 2013;5:1-5.
Fernández-Torre JL, Teja JL, Castellanos A, Figols J, Obeso T, Arteaga R. Spinal muscular atrophy type I mimicking critical illness neuropathy in a paediatric intensive care neonate: electrophysiological features. Brain Dev 2008;30:599-602. http://dx.doi.org/10.1016/j.braindev.2008.02.005
Leite JMRS, Prado GR. Paralisia cerebral: Aspectos Fisioterapêuticos e Clínicos. Rev Neurocienc 2004;12:41-5. http://dx.doi.org/10.4181/RNC.2004.12.41
Bach JR. Medical considerations of long-term survival of Werdnig- -Hoffmann disease. Am J Phys Med Rehabil 2007;86:349-55. http://dx.doi.org/10.1097/PHM.0b013e31804b1d66
Lima MB, Orrico KF, Moraes APF, Ribeiro CSNS. Atuação da fisioterapia na doença de Werdnig-Hoffmann: relato de caso. Rev Neurocienc 2010;18:50-4.
Toyoshima M, Maeoka Y, Kawahara H, Maegaki Y, Ohno K. Pulmonary atelectasis in patients with neurological or muscular disease; gravity-related lung compression by the heart and intra-abdominal organs on persistent supine position. No To Hattatsu 2006;38:419-24. http://dx.doi.org/10.11251/ojjscn1969.38.419
Jolley SE, Regan-Baggs J, Dickson RP, Hough CL. Medical intensive care unit clinician attitudes and perceived barriers towards early mobilization of critically ill patients: a cross-sectional survey study. BMC Anesthesiol 2014;1:84. http://dx.doi.org/10.1186/1471-2253-14-84
Harris CL, Shahid S. Physical therapy-driven quality improvement to promote early mobility in the intensive care unit. Proc (Bayl Univ Med Cent) 2014;27:203-7.
Berney SC, Harrold M, Webb SA, Seppelt I, Patman S, Thomas PJ, et al. Intensive care unit mobility practices in Australia and New Zealand: a point prevalence study. Crit Care Resusc 2013;15:260-5.
Nydahl P, Ruhl AP, Bartoszek G, Dubb R, Filipovic S, Flohr HJ, et al. Early mobilization of mechanically ventilated patients: a 1-day point-prevalence study in Germany. Crit Care Med 2014;42:1178-86. http://dx.doi.org/10.1097/CCM.0000000000000149
Choong K, Foster G, Fraser DD, Hutchison JS, Joffe AR, Jouvet PA, et al. Acute rehabilitation practices in critically ill children: a multicenter study. Pediatr Crit Care Med 2014;15:270-9. http://dx.doi.org/10.1097/PCC.0000000000000160
Cremer R, Leclerc F, Lacroix J, Ploin D. Children with chronic conditions in pediatric intensive care units located in predominantly French- -speaking regions: Prevalence and implications on rehabilitation care need and utilization. Ped Crit Care 2009;37:1456-62.http://dx.doi.org/10.1097/CCM.0b013e31819cef0c.
