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RESPIRATORY CARE FOR CHILDREN WITH DUCHENNE MUSCULAR DYSTROPHY
AJAY KASI, MD
DIV. OF PEDIATRIC PULMONOLOGY
1/21/2017
MUSCLES AND BREATHING
Chest muscles and diaphragm are required for:
• Inspiration (Breathing-­in)
• Expiration (Breathing-­out)
• Coughing
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The back muscles keep the spine straight –
Important for chest size and expansion with breathing.
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BREATHING PROBLEMS IN DMD
• DMD-­ gradual loss of muscle function over time.
• Loss of respiratory muscle strength
• Reduced lung function
– Hypoventilation (High CO2 levels) – Lower oxygen levels
• Weak cough -­ airway mucus plugging
• Atelectasis (collapse of air sacs in part of the lungs)
• Pneumonia (Lung infection)
• Scoliosis (curve of the spine) can limit chest size and ability to take a deep breath.
• Sleep apnea (Blockage of airflow into lungs during sleep)
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ATELECTASIS
Atelectasis is collapse of air sacs in part of the lungs
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PNEUMONIA
Pneumonia is inflammation in the air sacs in the lungs in response to an injury, like an infection.
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SCOLIOSIS
Scoliosis (curve of the spine) can limit chest size and ability to take a deep breath.
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RESPIRATORY CARE FOR CHILDREN
• First visit with pediatric pulmonologist: 4-­6 years age
– Obtain pulmonary function test
– Anticipatory medical guidance regarding potential respiratory problems
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Tests to evaluate breathing Pulmonary Function Test
Oxygen saturation by pulse oximeter
Carbon dioxide level – by CO2 monitor or Blood gas test
Sleep study
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PULMONARY FUNCTION TEST
• Measures how well your child can move air in and out of the lungs
• Provides an estimate of strength of breathing muscles
• Involves wearing a nose clip, taking a deep breath and blowing hard into the mouthpiece.
• Measurements include:
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Forced Vital Capacity (FVC)
Maximum Inspiratory Pressure (MIP)
Maximum Expiratory Pressure (MEP)
Peak Cough Flow
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SLEEP STUDY
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Sleep Study (Polysomnogram) records the sleep pattern
– Oxygen level
– Carbon dioxide level
– Air movement through nose
Different sensors are connected to record the sleep pattern and breathing pattern
Involves spending the night sleeping in the sleep lab
Performed periodically from the time of wheel-­chair use
Risk factors for sleep apnea
• Reduced muscle tone
• Large tonsils &/ adenoids
• Obesity
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THERAPIES TO IMPROVE LUNG HEALTH
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AIRWAY CLEARANCE
Techniques to help clear mucus from the lungs
Prevents atelectasis and pneumonia
Can prevent hospitalization and reduce frequent pneumonia
Mechanical insufflation-­exsufflation (Cough Assist Device)
Necessary when:
• Reduced pulmonary function test
FVC < 40% or <1.25L in older teen
• Maximum expiratory pressure < 40
• During respiratory infections
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COUGH ASSIST DEVICE
Image from Philips Respironics Website
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NON-­INVASIVE VENTILATION
• Increased risk for sleep-­related breathing problems
– Obstructive sleep apnea
– Low oxygen levels – Elevated CO2 levels
• Assisted ventilation may be needed to help provide sufficient air flow into and out of the lungs.
• Non-­invasive ventilation is assisted ventilation that does not require a surgical procedure.
• Child receives air under pressure through a mask over the nose and/or mouth. 12
NON-­INVASIVE VENTILATION
• Positive airway pressure helps keep the airway from closing or narrowing during sleep
• CPAP: continuous pressure which remains the same during breathing in and breathing out • Bilevel PAP (BiPAP): allows for different pressures when breathing in and breathing out • Children may initially need breathing support only during sleep. • With time, breathing support may be required during the daytime also.
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NON-­INVASIVE VENTILATION
• When is night-­time ventilation required?
Abnormal results of sleep study with sleep apnea, low oxygen levels or high CO2 level.
• Symptoms of sleep hypoventilation include:
– Frequent night time awakenings
– Daytime sleepiness
– Morning headache
– Fatigue
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NON-­INVASIVE VENTILATION
• Night time BPAP use in DMD has resulted in – Improved survival
– Improved well-­being and independence
– Improved sleep quality
– Decreased daytime sleepiness
– Improved daytime ventilation
– Slowed rate of decline in lung function
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DAYTIME VENTILATION
• With time and progressive muscle weakness, children may require 24-­hours breathing support.
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Daytime breathing support is indicated when:
Symptoms of hypoventilation when awake
Oxygen saturation < 92% when awake &/or
Blood or End-­tidal CO2 > 50 mm Hg when awake
Inability to speak a full sentence without breathlessness
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INVASIVE VENTILATION
• Currently, the vast majority of children and young adults do well with non-­invasive ventilation and expert panels recommend using non-­invasive ventilation for as long as possible.
• Consideration for Invasive ventilation with Tracheostomy
• Cannot use BPAP/ Failed BPAP
• Patient preference
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INVASIVE VENTILATION
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Advantages of Invasive ventilation:
Secure airway
Ability to provide higher ventilator pressure
Direct airway suctioning during respiratory infections
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SWALLOWING PROBLEMS
• Swallowing problems from muscle weakness lead to risk of choking.
• Aspiration: When food or fluid goes into the windpipe from the throat with eating.
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Aspiration can cause:
Inflammation of the airways
Lung infections
Scarring in the lungs
• Evaluated by swallow function study
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OTHER CARES
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Hand hygiene to prevent infection
Avoiding contact with people who are ill
Annual Influenza vaccine
Pneumococcal vaccine
Pulmonary evaluation before surgery
• Prognosis: Boys with DMD usually did not survive much beyond their teen years. With advances in cardiac and respiratory care, life expectancy is increasing and many young adults attend college and have careers. 20
REFERENCES
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Respiratory Care of the Patient with Duchenne Muscular Dsytrophy. ATS Consensus Statement. Am J Respir Crit Care Med Vol 170. pp 456–465, 2004
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The Respiratory Management of Patients with Duchenne Muscular Dystrophy: a DMD Care Considerations Working Group Specialty Article. Pediatr Pulmonol. 2010;; 45:739–748
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Treating Breathing Problems in Children with Neuromuscular Diseases. ATS Patient Education Series 2015 https://www.thoracic.org/patients/patient-­resources/topic-­
specific/pediatric.php
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Sleep Studies in Children. ATS Patient Education Series 2014 https://www.thoracic.org/patients/patient-­resources/topic-­specific/pediatric.php
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Pulmonary Function Testing in Children. ATS Patient Education Series 2014. Am J Respir Crit Care Med. Vol. 189, P5-­P6, 2014 21
ACKNOWLEDGEMENT
• Monique Margetis, MD
• Salman Khan, MD
• Gulnur Com, MD
• Eugene Sohn, MD
• Emily Gillett, MD
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