Children with neuromuscular disease are susceptible to hypoventilation and secretion retention leading to respiratory infection. They require regular respiratory assessment and can benefit hugely from home ventilation and sophisticated physiotherapy. We see our patients twice a year and perform cardiorespiratory polygraphy at least once a year.<\/p>\n
BTS guidelines for the respiratory management of children with neuromuscular weakness\u00a0 <\/strong>have recently been published and are available here<\/a><\/span><\/p>\n Important messages from this guideline are listed below:<\/span><\/p>\n Identifying children at risk of respiratory complications<\/strong><\/span> top of page<\/em> <\/a><\/p>\n Airway clearance and respiratory muscle training<\/strong><\/span> top of page<\/em> <\/a><\/p>\n Assisted ventilation<\/strong><\/span>
\n\u2022 Clinical assessment of respiratory health should be part of every medical consultation for children with neuromuscular weakness (NMW) and should be directed towards identifying progressive muscle weakness, ability to cope with respiratory infection, aspiration, progression of scoliosis and sleep-disordered breathing. [D]
\n\u2022 Vital capacity should be measured in all patients with neuromuscular disease who are capable of performing spirometry as part of the respiratory assessment. [C]
\n\u2022 Cough peak flow should be used as part of the assessment of effective secretion clearance in children with neuromuscular disease over the age of 12 years.
\n\u2022 Assessment for sleep-disordered breathing should be carried out no less than annually for children with neuromuscular disease who have a vital capacity of 60% predicted and for children who have become non-ambulant because of progressive muscle weakness or who never attain the ability to walk. [D]
\n\u2022 Assessment for sleep-disordered breathing should be carried out no less than annually for all infants with weakness, children with NMW who have symptoms of obstructive sleep apnoea or hypoventilation, children with clinically apparent diaphragmatic weakness and children with rigid spine syndromes. [\u2022]
\n\u2022 In young children whose rate of disease progression is uncertain, or in older children who have shown a clinical deterioration or who are suffering with repeated infections, or who develop symptoms of sleep-disordered breathing, sleep assessment may need to be more frequent than once a year. [\u2022]
\n\u2022 All children with abnormal overnight oximetry should undergo more detailed sleep monitoring with at least oxycapnography. [\u2022]
\nWhen there is doubt about the cause of sleepdisordered breathing, overnight polysomnography or sleep polygraphy should be performed. [\u2022]
\n\u2022 Portable overnight oxycapnography or polygraphy in the home may be the most appropriate option for some patients. [\u2022]
\n\u2022 Children with neuromuscular diseasewith a history of swallowing difficulties should have a feeding assessment by a speech and language therapist including a video fluoroscopy swallow assessment if the swallow is thought to be unsafe. [\u2022]<\/p>\n
\n\u2022 Children with ineffective cough (including children over 12 years of age with cough peak flow < 270 litres\/min), particularly if they have had episodes of deterioration with respiratory infection, should be taught augmented cough techniques. [C]
\n\u2022 Manual cough assist and air-stacking methods to achieve maximum insufflation capacity are effective methods of improving cough efficiency and should be used when appropriate. [C]
\nMechanical insufflation\/exsufflation (MI-E) should be considered in very weak children, those with loss of bulbar function, and those who cannot cooperate with manual cough assist or air-stacking or in whom these methods are not effective. [C]
\n\u2022 Oscillatory techniques such as high-frequency chest wall oscillation and intrapulmonary percussive ventilation should be considered in children who have difficulty mobilising secretions or who have persistent atelectasis, despite use of other airway clearance techniques. [D]
\n\u2022 Airway clearance techniques should be used during respiratory infection when oxygen saturation levels fall below 95% while the child is breathing room air. If the techniques being used fail to result in an increase in oxygen saturation to 95% or above, different methods of airway clearance should be used. This may require attendance at hospital for treatment. [D]
\n\u2022 MI-E should be available in the acute setting in all hospitals that treat neuromuscular patients as an alternative method of airway clearance with the purpose of preventing deterioration and the need for intubation and mechanical ventilation. [D]
\n\u2022 Nebulised normal saline may be considered in children who have continued tenacious secretions. [\u2022]
\nHumidification should be considered in children who use non-invasive ventilation (NIV) and who have tenacious airway secretions. Care should be taken to ensure that use of humidification does not result in a troublesome increase in oral secretions. [\u2022]
\n\u2022 When using sputum mobilising techniques, appropriate emergency equipment (eg, resuscitation bag and suction) should be available in case of mobilising large mucus plugs into the central airways where they may result in airways obstruction. [\u2022]
\n\u2022 Children who use regular night-time or diurnal NIV should use their ventilator to support deep breathing during airway clearance treatments. Use of NIV during airway clearance sessions can help prevent respiratory muscle fatigue. [\u2022]
\n\u2022 Children who use MI-E for airway clearance should be given long enough periods of rest during treatment sessions to prevent respiratory muscle fatigue due to coughing. [\u2022]
\nAt the end of a treatment session with MI-E it is important to complete the session with an insufflation to leave the child with an appropriate functional residual capacity. [\u2022]<\/p>\n
\n\u2022 Children with NMW resulting in symptomatic nocturnal hypoventilation or daytime hypercapnia should be supported with NIV. [C]
\n\u2022 A non-invasive approach should be considered in children needing daytime ventilation. [D]
\n\u2022 Clinical teams caring for children using home ventilators should become familiar with a small number of machines. For most children pressure-targeted machines work well and are simple to use. [?]
\n\u2022 Ventilation modes with fixed inspiratory times are usually the most appropriate for use in young or very weak children. [\u2022]
\n\u2022 For older teenage children who need to use mouthpiece ventilation during the day, and\/or who use air-stacking as a way of assisting cough, volume-targeted or hybrid ventilators may be preferred. [\u2022]
\nWhen full face masks are used, anti-asphyxia valves should be fitted to allow room air breathing in case of ventilator failure. The risks of the child vomiting and aspirating should be considered, particularly if the child is unable to remove the mask on their own. [\u2022]
\n\u2022 Children needing ventilatory support for more than 16 h perday should be provided with two ventilators in case of equipment failure. [\u2022]
\nOnce a child has become established on NIV, a sleep polygraphy or oxycapnography should be carried out to check that it has effectively abolished sleep-associated hypoventilation. Ventilator settings should be adjusted and rechecked as necessary. [D]
\n\u2022 Children supported by NIV should be assessed regularly with repeat sleep studies to ensure continued effectiveness of NIV at preventing hypoventilation. The frequency of review will vary according to clinical circumstances, but should not be less than every 12 months. [D]
\n\u2022 Assessment for skin injury and facial flattening should be carried out regularly in children using NIV and the mask interface adjusted as necessary to minimise these complications. [\u2022]
\n\u2022 Tracheostomy tubes should be carefully sized and sited to ensure the tip of the tube does not abut the tracheal wall. [\u2022]
\n\u2022 Family and child preference should be taken into account when considering tracheostomy to facilitate diurnal ventilation. [\u2022]
\n\u2022 Oxygen alone should not be used to correct hypoxaemia caused by hypoventilation in patients with neuromuscular disease. [\u2022]
\n\u2022 NIV should be the first-line treatment for children with NMW in acute respiratory failure. [D]
\n\u2022 Intensive care units caring for children with NMW should be aware of appropriate extubation criteria. These should include the presence of only minimal airway secretions, use of effective airway clearance methods (such as MI-E devices) and oxygen saturation more than 94% without supplemental oxygen for more than 12 h. Continuous NIV should be used immediately after extubation. [\u2022]<\/p>\n