2012, Number 2
<< Back Next >>
Neumol Cir Torax 2012; 71 (2)
Pediatric pulmonologist survey on medical care practice for patients with neuromuscular disease and respiratory complications
Luna-Rojas C, Martínez-Carbajal G, Plascencia-Esparza D, Torres-Fragua M, Baños-Mejía O, Torre-Bouscoulet L, Galicia-Amor S, Gochicoa-Rangel L
Language: Spanish
References: 17
Page: 141-146
PDF size: 69.20 Kb.
ABSTRACT
Background: Respiratory muscles impairment is the main cause of morbidity and mortality in neuromuscular disease (NMD) patients. Pediatric pulmonologist must be involved in an opportune and standardized evaluation to minimize complications.
Objectives: 1) to know, by asking a group of pediatric pulmonologist, the time between the NMD diagnosis and the request for the pediatric pulmonologist’s evaluation; and 2) to investigate the diagnostic and therapeutic approach.
Method: Transversal clinical research. A questionnaire was administered to pediatric pulmonologist who attended the Annual Meeting in August of 2011.
Results: 33 responses were collected (21.4% of pediatric pulmonologist in the country). The majority indicated that patients were referred to them 6 to 12 months after being diagnosed; their approach had consisted of cardiological, orthopedic and swallow disorders evaluation; the majority recommend immunization to prevent pneumococcal and influenza infections. Pulse oximetry was the most used functional test (96.9% of the cases), followed by spirometry (60.6%), measurement of maximal inspiratory and expiratory pressures (18.1%) and peak cough flow (12.1%); 16 pulmonologist (48.4%) indicated lack of opportune non-invasive mechanical ventilation, and 25 (75.7%) performed assisted cough techniques to improve mucociliary clearance.
Conclusions: Patients with NMD are referred late to the pediatric pulmonologist. Evaluation for the strength of respiratory muscles are rarely performed, and non-invasive mechanical ventilation is underused. This study highlights the urgent need for physicians, to comply with the guides to handling NMD.
REFERENCES
Domínguez-Flores ME. Fisiopatología de las enfermedades neuromusculares. Neumol Cir Torax 2011;72:14-27.
Nicot F, Hart N, Forin V, et al. Respiratory muscle testing: a valuable tool for children with neuromuscular disorders. Am J Respir Crit Care Med 2006;174:67-74.
Guía de las Enfermedades Neuromusculares. Información y apoyo a las familias. En: Federación Española de Enfermedades Neuromusculares. Formación Alcalá: Alcalá;2008.p.215.
Sharma GD. Pulmonary function testing in neuromuscular disorders. Pediatrics 2009;123(Suppl 4):S219-S221.
De-Vito E. Músculos respiratorios. En: Mazzei J, Mazzei M, editores. Manual de pruebas de función pulmonar. Barcelona: Thomson Reuters; 2009.p.73-85.
Olofson J, Dellborg C, Sullivan M, Midgren B, Caro O, Bergman B. Qualify of life and palliation predict survival in patients with chronic alveolar hypoventilation and nocturnal ventilatory support. Qual Life Res 2009;18:273-280.
Simonds AK, Muntoni F, Heather S, Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 1998;53:949-952.
Troosters T, Gosselink R, Decramer M. Respiratory muscle assessment. Eur Respir Mon 2005;31:57-71.
Benditt J, Boitano L. Esclerosis lateral amiotrófica. En: Mazzei JA, et al. editores. Manual de pruebas de función pulmonar. De la fisiología a la práctica. Barcelona: Thomson Reuters; 2009.p.339-342.
Finder JD, Birnkrant D, Carl J, et al; and American Thoracic Society. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004;170:456-465.
Prado F. Recomendaciones para los cuidados respiratorios del niño y adolescente con enfermedades neuromusculares. 2011. Disponible en: http://www.neumologia-pediatrica.cl
Laghi F, Tobin MJ. Disorders of the respiratory muscles. Am J Respir Crit Care Med 2003;168:10-48.
Katz SL, Gaboury I, Keilty K, et al. Nocturnal hypoventilation: predictors and outcomes in childhood progressive neuromuscular disease. Arch Dis Child 2010;95:998-1003.
Phillips MF, Quinlivan RC, Edwards RH, Calverley PM. Changes in spirometry over time as a prognostic marker in patients with Duchenne muscular dystrophy. Am J Respir Crit Care Med 2001;164:2191-2194.
Kennedy JD, Martin AJ. Chronic respiratory failure and neuromuscular disease. Pediatr Clin North Am 2009;56:261-273, xii.
Diebold D. Management of respiratory complications in neuromuscular weakness. Clin Pulm Med 2011;18:175-180.
Kravitz RM. Airway clearance in Duchenne muscular dystrophy. Pediatrics 2009;123 Suppl 4:S231-S235.