Respiratory care is about looking after the breathing muscles. It is also sometimes referred to as ‘pulmonary management’. Usually, boys (and rare girls) with Duchenne or Becker do not have trouble breathing or coughing while they are still walking. However, as they get older breathing muscles gradually get weaker and they are at risk of chest infections due to an ineffective cough. Later they develop problems with breathing at night and eventually they will also need help during the day.
A proactive approach to respiratory care, with regular monitoring and early intervention, gives considerable benefits for quality and length of life. Respiratory physicians/pulmonologists and respiratory physiotherapists are important members of the multi-disciplinary team looking after those with Duchenne and Becker.
Young children in the early stage learn deep breathing techniques to help with ventilation and cough. Swimming and diving games encourage deep breathing without conscious effort. Swimming and aquatic physiotherapy are recommended.
Respiratory infections should be treated aggressively and early; antibiotics and physiotherapy may be needed. Asthma should be treated with decongestants and bronchodilators. Avoid cough suppressants.
In the late ambulatory stage, children are not quite so active. Full expansion of the lungs is not achieved but by taking steroids they can often still record excellent respiratory results.
Biannual spirometory assessment and additional techniques to expand the chest-wall and assist coughing are needed after peak cough flow falls below 270L/min. Teaching manual techniques is recommended as airway clearance will begin to be affected.
In the late ambulatory stages manually assisted cough, breath stacking, hyperinflation using an Ambu bag or cough assist machine twice a day is recommended. Discuss this with your doctor and/or physiotherapist.
NON-INVASIVE NIGHTTIME VENTILATION
Help with breathing at night will be offered when sleep studies show that it is needed. This is called ‘non-invasive nocturnal ventilation’ and involves a nasal or facemask attached to bi-pap machine. This improves both quality of life and length of life.
Monitoring should be performed regularly by a center with experience with Duchenne to determine the best time to start nighttime ventilation. Typically, when FVC (forced vital capacity) falls below 60% of predicted for height and age, sleep-disordered breathing occurs, and regular sleep studies are recommended. At 40% predicted capacity, function tests are performed four times a year and biannual sleep studies are recommended.
Some signs of trouble breathing at night include:
• Fatigue – waking up still tired in the morning
• Sleep disturbance – waking during the night
• Headaches when waking in the morning
• Poor concentration and disorientation
• Depression, anxiety
• Loss of appetite
• Softened voice
• An unproductive cough
If any of these symptoms are noticed a sleep study should be scheduled sooner than planned. Sometimes none of these symptoms are experienced despite hypoventilation at night, so monitoring should still be performed regularly.
Keep a copy of your child’s latest breathing tests to show any doctor who takes care of him.
NON-INVASIVE DAY TIME VENTILATION
Extended periods using the bi-pap machine and/or respiratory anxiety throughout the day means that daytime ventilation should be commenced. As with most treatments, we recommend that this is commenced earlier rather than later.
Young people with Duchenne or Becker experience a huge number of medical and quality of life benefits once their ventilation is stabilised, including: elevated mood, improved appetite, weight gain, greater access to the community, improved speech, improved social interactions, improvements in heart function etc.
Mechanical volume ventilation through a sip mouthpiece, nosepiece or facemask are possible. Sip mouthpiece ventilation is the least invasive and the portable ventilator is attached to the wheelchair and a mouthpiece may be mounted on a gooseneck or preferably a halter to make it constantly accessible to the user.
In an Emergency
A reminder that if your son’s oxygen level drops when he is ill or injured, oxygen should not be given without monitoring the carbon dioxide levels, as it depresses the respiratory drive and results in a build-up of carbon dioxide. Breathing may need to be supported (with BiPAP, for example) and assisted coughing (with a cough assist machine or Ambu bag) may help.
Take your equipment (cough assist, BiPAP, etc.) with you to the hospital/emergency room and alert your neuromuscular team that you are going to the ER/hospital.
People with Duchenne or Becker should never be given inhaled anesthesia or the drug succinylcholine.