ABSTRACT
Los avances tecnológicos y los cuidados respiratorios no invasivos en el tratamiento de las afecciones respiratorias en pacientes con patologías neuromusculares han contribuido a una mejor calidad de vida y sobrevida de los pacientes con enfermedades neuromusculares. Además de la capacitación de los pacientes y sus cuidadores para facilitar la estadía de los mismos en el hogar sin depender de instituciones o cuidados de enfermería permanentes y promoviendo la autonomía e integración. El objetivo de este artículo es realizar una revisión de las estrategias y tratamientos brindados a los pacientes con enfermedades neuromusculares y afecciones respiratorias. Resulta esencial que los profesionales de la salud, conozcan todas las opciones terapéuticas posibles para ofrecer a sus pacientes y sus familias, de modo que a medida que la enfermedad avance ellos puedan expresar sus voluntades en forma anticipada y sin apremios derivados de eventos "inesperados". Además es necesario impulsar un cambio de paradigma en la forma en que los profesionales de la salud ven a estos individuos.
Technological advances and proper training for patients with neuromuscular diseases and respiratory problem, including their caregivers, facilitate their stay at home and promote their autonomy and integration, without depending on hospices or permanent nursing care. The objective of this article is to review all the strategies and treatment offered to the patients with neuromuscular diseases. It is essential for health care professionals to know all the therapeutic possibilities offering for their patients and their families, so as the disease progresses it would facilitate the conscious decision-making. Also it is necessary to promote a paradigm shift in the way health professionals approach to individuals with neuromuscular diseases.
ABSTRACT
This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes. These centers have now extubated at least 74 consecutive ventilator unweanable patients with DMD, over 95% of CNVS-dependent patients with SMA1, and hundreds of others with advanced neuromuscular disorders (NMDs) without resort to tracheotomy. Two centers reported a 99% success rate at extubating 258 ventilator unweanable patients without resort to tracheotomy. Patients with myopathic or lower motor neuron disorders can be managed noninvasively by up to CNVS, indefinitely, despite having little or no measurable vital capacity, with the use of physical medicine respiratory muscle aids. Ventilator-dependent patients can be decannulated of their tracheostomy tubes.
Subject(s)
Humans , Amyotrophic Lateral Sclerosis , Health Resorts , Motor Neurons , Muscular Atrophy, Spinal , Muscular Dystrophy, Duchenne , Neuromuscular Diseases , Physical and Rehabilitation Medicine , Respiratory Muscles , Tracheostomy , Tracheotomy , Ventilators, Mechanical , Vital CapacityABSTRACT
Objective: To compare the effects of Air Stacking (AS) and Glossopharyngeal Breathing (GPB) on the Maximum Insufflation Capacity (MIC) in patients with Neuromuscular Disease (NMD). Methods: We design a randomized cross-over study. Children and adolescents with NMD who were users of non-invasive mechanical ventilation were recruited. Vital capacity (VC) and MIC were measured before and after the intervention with AS and GPB. Values were compared pre- and post-intervention and were considered statistically significant if p <0.05. Results: We selected 14 patients with a median age of 12.5 years (range 9-18) with the following diagnoses: Duchenne Muscular Dystrophy (7), Spinal Muscular Atrophy Type II (3), Spinal Cord Injury (1) and Congenital Myopathies (3). The median baseline VC was 1325 ml (CI 1084-1594 ml). AS improved the VC to 1930 mL (CI 1630-2434 mL, p<0.001), and GPB increased the VC to 1600 mL (CI 1370-1960 mL, p=0.001). There was a significant difference of 290 mL (CI 168-567 mL, p<0.002) between both techniques. Conclusion: The air stacking and glossopharyngeal breathing were both effective in increasing the maximum insufflation capacity, but air stacking resulted in a greater increase in MIC.
ABSTRACT
El propósito de este artículo es describir el uso de técnicas de soporte respiratorio muscular no invasivo con el fin de prevenir la insuficiencia respiratoria y el fallo ventilatorio, permitiendo la extubación y decanulación de pacientes considerados "imposibles de destetar''. La aplicación de presión en la vía aérea de forma no invasiva durante la inspiración puede proporcionar un soporte ventilatorio continuo en pacientes con poca o inexistente capacidad vital. Así mismo, podrá alcanzarse un pico flujo espiratorio efectivo en pacientes con una disfunción severa de los músculos espiratorios. En abril de 2010 un consenso clínico de 19 centros en 18 países describió 1623 pacientes neuromusculares con atrofia muscular espinal tipo 1, distrofia muscular de Duchenne (DMD) y esclerosis lateral amiotrófica que sobrevivieron utilizando soporte ventilatorio continuo sin necesidad de traqueotomía. De esta serie de pacientes, 76 (47%) precisaron ventilación no invasiva continua en un periodo de seguimiento de 15 años. De estos 76.22 (30.1%) de los enfermos fueron extubados y 35 (4.6%) decanulados en cuatro centros especializados. En estos centros se extuban rutinariamente pacientes con distrofia muscular de Duchenne considerados "imposibles de destetar", de forma que ninguno de sus más de 250 enfermos ha requerido traqueotomía. Esta aproximación en el tratamiento se está abriendo camino en varios centros de España, México y América45.
The purpose of this article is to describe the use of noninvasive inspiratory and expiratory muscle aids to prevent ventilatory insufficiency and failure, and to permit the extubation and tracheostomy tube decannulation in "unweanable" patients. Noninvasive airway pressure aids can provide a continuous support to respiratory ventilation for patients with little or no vital capacity and can provide effective cough flows in patients with severely dysfunctional expiratory muscles. In April 2010, a consensus of clinicians from 19 centers in 18 countries reported the experience of noninvasive ventilation in 1623 patients with spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis (ALS), who survived without tracheotomy. The survival was for more than 15 years in 76 patients. Among these 76 patients, 22 (30.1%) were extubated and 35 (4.6%) were decannulated in four specialized centers. In these centers, unweanable DMD patients are routinely extubated; none of over 250 such patients has undergone tracheotomy. This approach is now being introduced into different centers in Spain, Mexico and the United States.