Traqueostomía en niños: los desafíos de la decanulación, revisión y propuesta de trabajo / Tracheostomy in children: the challenges of decanulation, revision and work proposal

Tracheostomy (TQT) in pediatrics is a procedure that allows maintaining permeable airways and establishes prolonged mechanical ventilation. Continuous noninvasive ventilatory support (CNVS) can always be used for patients capable of cooperating with it and mechanical insufflation-exsufflation (MIE). Despite this, TQT continues to be indicated frequently, limiting home transfer and care, conditioning additional burden of morbidity and risks. In those with upper airway obstruction (OVAS), except in <2 years, the decanulation follows similar guidelines as in adults. Small children who require even only sleep ventilation cannot be decannulated to NVS if they cannot be relied on to use it via noninvasive interfaces. So, children under age 12 should not be decannulated unless they are ventilator weaned. For adolescents and adults, the principle criterion for safe decannulation is an MIE-peak exsufflation flows (MIE-EF) over 150 L/m whereas need for tracheotomy occurs when MIE-EF decrease below 120 L/m, irrespective of extent of ventilator dependence. The following article is a critical narrative review of different decannulation alternatives to ensure that this process can be safely carried out with effectiveness and efficiency. Patients with different ages and diseases have been considered, knowing that younger children and adult in transition tracheostomized patients significantly increases the challenges.

La traqueotomía (TQT) permite mantener la vía aérea permeable y la ventilación mecánica prolongada. El soporte ventilatorio no invasivo continuo (SVNI) junto con la insuflación-exuflación mecánica (MIE), son una alternativa en la mayoría de los casos. Pese a esto, la TQT sigue indicándose frecuentemente, limitando la transferencia al hogar, condicionando carga adicional de morbilidad. En los niños con obstrucción de la vía aérea superior (OVAS), salvo en los <2 años, la decanulación sigue lineamientos similares que en adultos. Los <12 años dependientes de ventilador, aun cuando sólo sea nocturna, son decanulados o extubados a SVNI más difícilmente que adultos con dependencia continua. La mejor estrategia es evitar la TQT e indicar SVNI en los pacientes colaboradores que cumplan criterios. Excepto pacientes con estridor por OVAS grave o enfermedad de la primera motoneurona y severo compromiso miopático. Para adolescentes y adultos, el criterio para la decanulación segura es un pico flujo exuflado máximo en MIE (PFE-MIE), con o sin tos, >150 L/m, mientras que la necesidad de traqueostomía ocurre cuando el MIE (PFE-MIE) esta bajo 120 ml/min, independiente del grado de dependencia del ventilador. En menores de 12 años, el éxito del SVNI reside en obtener interfaces confiables para su entrega, de lo contrario no podrían ser decanulados. Siendo escasos los protocolos de decanulación pediátrica. Nuestro objetivo es el de ponderar alternativas seguras y eficientes para la decanulación de la TQT. Siendo considerados pacientes con edades y enfermedades distintas, sabiendo que los más pequeños, no colaboradores y dependientes de ventilación aumentan el desafío.

Cuidados respiratorios para pacientes con enfermedades neuromusculares / Respiratory care for patients with neuromuscular diseases

Non-invasive respiratory care, combining with ventilatory support, initially at night and then during 24 hours/day, even in patients with minimal vital capacity and the implementation of specifics techniques like mechanically assisted coughing, glossopharyngeal breathing and air stacking, have contributed to a better quality of life and survival of patients with neuromuscular diseases. It is essential for health care professionals to know all the therapeutic possibilities for their patients and their families, so as the disease progresses it would facilitate their decision-making. Technological advances and proper training for patients and caregivers facilitate the stay at home and promote their autonomy and integration, without depending on hospital nor permanent nursing care. In November 2016 it was carried out the Noninvasive Ventilatory Support workshop/meeting with more than 200 physicians, physiotherapists, respiratory therapists and nurses in Montevideo, Uruguay. It was conducted by Dr. John Robert Bach, Medical Director of the Center for Non-Invasive Mechanical Ventilation at Rutgers New Jersey School of Medicine in Newark, New Jersey. Dr Bach is recognized worldwide for his extensive background in studies and publications on noninvasive ventilation and neuromuscular diseases.

Los cuidados respiratorios no invasivos, combinando la asistencia ventilatoria, inicialmente nocturna y luego durante las 24 h del día, incluso en pacientes con capacidad vital mínima, más la implementación de estrategias complementarias de tos asistida, respiración glosofaríngea y apilamiento de aire (air stacking) en forma activa o pasiva han contribuido a una mejor calidad de vida y sobrevida de los pacientes con enfermedades neuromusculares. Resulta esencial que los profesionales de la salud, conozcan todas las opciones terapéuticas al informar a sus pacientes y sus familias, de modo que ellos puedan tomar sus mejores decisiones en la medida que la debilidad e hipoventilación progresen. Los avances tecnológicos, la capacitación de los pacientes y sus cuidadores facilitan su estadía en el hogar sin depender de instituciones o cuidados de enfermería permanentes, promoviendo su autonomía e integración, disminuyendo el riesgo de falla respiratoria conducente a intubación endotraqueal y/o a traqueostomia. Los días 24 y 25 de noviembre del 2016, en Montevideo tuvo lugar un encuentro de capacitación en cuidados respiratorios no invasivos con más de 200 profesionales médicos, kinesiólogos y licenciadas de enfermería, destacando los avances y experiencia consolidad por el Dr. John Bach en más de 30 años de ejercicio profesional en pacientes con síndromes de hipoventilación secundario a enfermedades neuromusculares y otras condiciones que debilitan la bomba respiratoria. Las recomendaciones claves se resumen en este articulo, destacando como estos avances requieren impulsar un cambio de paradigma en la forma en que los profesionales de la salud ven y tratan a estos individuos.

Sleep Transcutaneous vs. End-Tidal CO2 Monitoring for Patients with Neuromuscular Disease.

OBJECTIVE: This study compared transcutaneous carbon dioxide partial pressure (PtcCO2) and end-tidal carbon dioxide partial pressure (PetCO2) monitoring during sleep for patients with neuromuscular disease. DESIGN: This is a retrospective study of patients whose PtcCO2 and PetCO2 were monitored before they began using noninvasive mechanical ventilation. The outcomes were divided into four groupings: group 1, both PtcCO2 and PetCO2 are greater than or equal to 49 mm Hg; group 2, PtcCO2 is greater than or equal to 49 mm Hg but PetCO2 is less than 49 mm Hg; group 3, PtcCO2 is less than 49 mm Hg but PetCO2 is greater than or equal to 49 mm Hg; and group 4, both PtcCO2 and PetCO2 are less than 49 mm Hg. RESULTS: A total of 39 subjects (mean [SD] age, 27.7 [19.3] yrs) were enrolled. PtcCO2 values were significantly higher than PetCO2 values (P < 0.001). The intraclass correlation coefficient between maximal and mean values of PtcCO2 and PetCO2 was 0.612 and 0.718, respectively. Bias and limits of agreement between PtcCO2 and PetCO2 were -7.5 mm Hg and -21.3 to 6.3 mm Hg for maximal values and -4.8 mm Hg and -14.8 to 5.3 mm Hg for mean values. Group 2 included 19 (48.7%) and group 3 included 3 (7.6%) patients who showed discrepancy of hypercapnia between two methods. CONCLUSIONS: Maximum PtcCO2 was significantly greater than maximum PetCO2 for both groups and, therefore, tends to be higher than PetCO2 in this population. This should be taken into consideration when assessing patients for sleep hypoventilation.

Allogeneic mesenchymal stem cell therapy outcomes for three patients with spinal muscular atrophy type 1.

No effective medical treatment has been documented for spinal muscular atrophy; however, cellular, molecular, and preclinical studies suggest that allogenic mesenchymal stem cells may play a role. Three children with spinal muscular atrophy type 1 underwent multiple intrathecal and intravenous infusions of mesenchymal stem cells. Their pretreatment, treatment, and posttreatment physical function were quantitated by the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scale for two patients and documented by video for all three. Infant Test of Neuromuscular Disorders values were 3 before treatment, 10 and 16 during treatment, and 0 and 10 seven and twelve months after treatment was discontinued, respectively. No adverse effects have been noted for at least 44 and 49 mos from onset of treatment, respectively. These data represent the first objective, quantifiable improvements in physical function for any treatment of spinal muscular atrophy. Although the benefits were lost when the therapy was withdrawn, this may be an initial step in establishing mesenchymal stem cells as a safe and effective treatment of spinal muscular atrophy.

Cost and physician effort analysis of invasive vs. noninvasive respiratory management of Duchenne muscular dystrophy.

BACKGROUND: Continuous tracheostomy mechanical ventilation users with Duchenne muscular dystrophy are institutionalized or have home nursing services (licensed practical nurse/registered nurse), whereas patients dependent on continuous noninvasive ventilatory support (CNVS) can avoid hospitalizations and publically paid services. DESIGN AND OBJECTIVE: This is a retrospective analysis comparing cost and physician effort for managing CNVS and continuous tracheostomy mechanical ventilation users with Duchenne muscular dystrophy. RESULTS: Compared with ongoing personal care, physician services and intercurrent hospitalization costs were negligible. Ten home continuous tracheostomy mechanical ventilation users had 16.4 hrs per day of licensed practical nurse/registered nurse care costing $269,370 per year; 14 were institutionalized at $237,350 per year; and 8 were decannulated to CNVS, with 5 subsequently returning home, costing only $9,800 per year for respiratory equipment. For 93 CNVS users, costs ranged from $9,800 per year for 37 without publically funded assistance, $44,968 per year for 3 with nurses' aides, $81,395 per year for 35 with unskilled personal assistance services, and $239,805 per year for 12 with licensed practical nurses/registered nurses. Twenty-eight became CNVS dependent without hospitalization or evaluation for home care. CONCLUSIONS: Noninvasive management permits cost-effective living at home but is disincentivized by fixed hospital diagnosis-related groups compensation that encourages rapid tracheotomy as well as expensive institutionalization and nursing care.

Decanulation of patients with severe respiratory muscle insufficiency: efficacy of mechanical insufflation-exsufflation.

OBJECTIVE: Ventilator dependent patients with neuromuscular disorders and high level spinal cord injury have been extubated and decanulated to continuous noninvasive intermittent positive pressure ventilatory support after mechanical insufflation-exsufflation was used to achieve specific criteria for tube removal. The purpose of this study is to report changes in extent of need for ventilator use and in vital capacity related to mechanical insufflation-exsufflation used via tracheostomy tubes and post-decanulation via oronasal interfaces. METHODS: Upon presentation patients were placed on fiO2 21% and CO2 was normalized by adjusting ventilator settings as needed. The vital capacity (1st data point) and h/day of ventilator dependence were noted. Then mechanical insufflation-exsufflation was used via the tubes up to every 2 h until ambient air oxyhemoglobin saturation (SpO2) baseline remained ≥ 95% and other decanulation criteria were achieved. The vital capacity was re-measured (2nd data point) and the patient decanulated to continuous noninvasive intermittent positive pressure ventilatory support in ambient air as care providers used mechanical insufflation-exsufflation up to every 30 min to maintain SpO2 ≥ 95%. The vital capacity (3rd data point) and minimum hours/day of noninvasive intermittent positive pressure ventilatory support requirement during the next 3 weeks were recorded. RESULTS: The vital capacities of 61 tracheostomized ventilator users, 36 of whom were continuously dependent, increased significantly (p < 0.001) from presentation to immediately pre-decanulation and in the 3 weeks post-decanulation and all except one were successfully decanulated. CONCLUSION: Many ventilator users can be decanulated in outpatient clinics to continuous noninvasive intermittent positive pressure ventilatory support with mechanical insufflation-exsufflation used to increase vital capacity, SpO2, and autonomous ability to breathe.

Fiber-optic bronchoscopy and volume-cycled mouthpiece ventilation for a patient with multiple sclerosis and ventilatory failure.

Fiber-optic bronchoscopy supported by continuous or bilevel positive airway pressure has helped patients with hypoxemic or hypercapnic respiratory failure avoid respiratory complications. The authors describe a case of a 57-yr-old man with multiple sclerosis with a vital capacity of 250 ml (5% of predicted normal) who was using continuous noninvasive intermittent positive pressure ventilatory support when he underwent bronchoscopy while receiving continuous noninvasive intermittent positive pressure ventilatory support via a 15-mm angled mouthpiece interface. He was switched from a nasal to a 15-mm angled mouthpiece interface for continuous noninvasive intermittent positive pressure ventilatory support for the procedure. Simple mouthpieces may be useful alternatives to other facial interfaces for ventilatory support during bronchoscopy because of patient comfort and operator convenience.

Two-step stool aerobic training for smokers.

OBJECTIVE: The aim of this study was to analyze subjective, physical, and physiologic responses to a standardized incremental 30-min two-step stool test to create an individualized 45-min maximally intensive two-step stool endurance exercise regimen for home training. DESIGN: This is a longitudinal study on 26 consecutively referred male smokers aged 39-66 yrs. Each performed the two-step stool test on two 15-cm steps at 10, 20, 30, 40, 50, and 60 climbs per minute. Exertional dyspnea, oxygen consumption per unit time, ventilation, respiratory rate, tidal volume, heart rate, capillary oxyhemoglobin saturation, physiologic cost index, and oxygen pulse were recorded and compared with those observed during incremental cycle exercise (30 W per 3 mins). Multivariate analysis for each parameter was undertaken as a mixed model. RESULTS: All subjects attained 60 climbs per minute on the two-step stool test and performed 38-42 mins of two-step stool endurance. All parameters reached 80%-96% of cycle maximum oxygen consumption. The subjects found the two-step stool endurance simple and practical to perform at home. There were no complications. CONCLUSIONS: The incremental two-step stool test is a simple, cost-effective way to establish a 45-min maximally intensive endurance exercise training program practical for use in the home.

A historical perspective on expiratory muscle aids and their impact on home care.

Mechanically assisted coughing is the combination of mechanical insufflation-exsufflation to the airways in conjunction with an exsufflation-timed abdominal thrust. It has permitted in-home long-term survival of respirator-dependent users of continuous noninvasive intermittent positive pressure ventilatory support in the United States since shortly after it became available in 1952. By contrast, the first European patients to benefit from continuous noninvasive intermittent positive pressure ventilatory support were not described until institutionalized continuous noninvasive intermittent positive pressure ventilatory support users with Duchenne muscular dystrophy were described in Belgium in 2006, 2 yrs after mechanically assisted coughing was approved for sale by the European Union. Domiciliary management with continuous noninvasive intermittent positive pressure ventilatory support and mechanically assisted coughing has subsequently been described in Europe. This work describes the historical development of mechanically assisted coughing in the framework of aiding the respiratory muscles to prevent respiratory failure and avoid invasive airway intubation. Some current mechanical in-exsufflators include oscillation as a surrogate for physiologic mucociliary transport along with their assisted coughing function. Noninvasive management prolongs life while promoting cost containment and preserving quality-of-life for patients with respiratory muscle impairment.

Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease.

INTRODUCTION: Although it is known that continuous noninvasive ventilation (CNIV) can prolong life in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND), in this study we explore similar claims for diaphragm pacing (DP). METHODS: NIV and DP users' vital capacities (VCs) over time and duration of NIV and CNIV dependence were analyzed for 354 non-DP and 8 DP ALS/MND patients. RESULTS: Patients had a higher rate of monthly VC decline before NIV use (5.1 ± 7.6%) than during NIV use (2.5 ± 3.6%) (P < 0.01, 95% CI 0.84-4.5); the decline for 4 DP users was 3.7-20%. Fifty-five ALS/MND patients used part-time NIV for 19.9 ± 27.6 months until tracheostomy/death, whereas 113 others used it for 10.9 ± 10.5 months until CNIV dependence for another 12.8 ± 16.2 months. After placement, 7 DP users were CNIV dependent in 8.0 ± 7.0 months, whereas 6 underwent tracheostomy/died in 18.2 ± 13.7 months. CONCLUSIONS: CNIV prolonged the survival of 113 of the 354 non-DP and 6 DP ALS/MND patients by 12.8 and 10.2 months, respectively. DP provided no benefit on VC or mechanical ventilation-free survival.

Indicators for ventilator use in Duchenne muscular dystrophy.

BACKGROUND: Noninvasive mechanical ventilation is being used up to continuously by patients with Duchenne muscular dystrophy (DMD). Invasive and noninvasive tests are used to assess ventilatory function but there are few reports relating them to extent of ventilator dependence for which simple and cost effective parameters are needed. OBJECTIVE: To investigate the relative efficacy of noninvasive lung function parameters for determining extent of need for ventilator use. MATERIALS AND METHODS: 83 DMD patients were divided into three groups: no ventilator use (asymptomatic) (n = 26) [Group 1], nocturnal ventilator use (symptomatic) (n = 20) [Group 2], and full-time ventilator dependence (n = 37) [Group 3]. Tidal volume (TV), vital capacity (VC), respiratory rate (RR), inspiratory time (Ti), respiratory cycle time (Ttot), rapid shallow breathing index (RSBI [RR/TV]), breathing intolerance index (BITI), ventilator requirement index (VRI) and a new parameter RR/VC were monitored and compared. Data were analyzed with receiver-operating-characteristic curves (ROC) and the area under the curve (AUC) was calculated. RESULTS: In group 2 and 3, patients used NIV for 3.3 ± 2.1 and 11.2 ± 4.7 years, respectively. By ROC comparison, RR/VC (RR/VC ≥ 0.024 [AUC, 0.921] and ≥0.071 [AUC, 0.935]), RR/TV (RR/TV ≥ 0.024 [AUC, 0.905] and ≥0.153 [AUC, 0.905]), and VC (VC ≤ 770 ml [AUC, 0.896] and ≤370 ml [AUC, 0.898]) represented to introduce nocturnal and continuous ventilator use, respectively. TV/VC, BITI, and VRI were either less sensitive or less specific. CONCLUSIONS: Lung function parameters including RR/VC, RR/TV, and VC are useful and inexpensive in predicting the extent of need for ventilator use. Overall, RR/VC is the most appropriate predictor for determining extent of need for ventilator use.

Extubation of patients with neuromuscular weakness: a new management paradigm.

BACKGROUND: Successful extubation conventionally necessitates the passing of spontaneous breathing trials (SBTs) and ventilator weaning parameters. We report successful extubation of patients with neuromuscular disease (NMD) and weakness who could not pass them. METHODS: NMD-specific extubation criteria and a new extubation protocol were developed. Data were collected on 157 consecutive "unweanable" patients, including 83 transferred from other hospitals who refused tracheostomies. They could not pass the SBTs before or after extubation. Once the pulse oxyhemoglobin saturation (Spo(2)) was maintained at > or = 95% in ambient air, patients were extubated to full noninvasive mechanical ventilation (NIV) support and aggressive mechanically assisted coughing (MAC). Rather than oxygen, NIV and MAC were used to maintain or return the Spo(2) to > or = 95%. Extubation success was defined as not requiring reintubation during the hospitalization and was considered as a function of diagnosis, preintubation NIV experience, and vital capacity and assisted cough peak flows (CPF) at extubation. RESULTS: Before hospitalization 96 (61%) patients had no experience with NIV, 41 (26%) used it < 24 h per day, and 20 (13%) were continuously NIV dependent. The first-attempt protocol extubation success rate was 95% (149 patients). All 98 extubation attempts on patients with assisted CPF > or = 160 L/m were successful. The dependence on continuous NIV and the duration of dependence prior to intubation correlated with extubation success (P < .005). Six of eight patients who initially failed extubation succeeded on subsequent attempts, so only two with no measurable assisted CPF underwent tracheotomy. CONCLUSIONS: Continuous volume-cycled NIV via oral interfaces and masks and MAC with oximetry feedback in ambient air can permit safe extubation of unweanable patients with NMD.