Airway Clearance in Neuromuscular Disorders
Cerebral palsy refers to a group of neurological disorders that appear in infancy or early childhood and permanently affect body movement and muscle coordination. Cerebral palsy is caused by damage to or abnormalities inside the developing brain that disrupt the brain’s ability to control movement and maintain posture and balance.1 It affects the motor area of the brain’s outer layer, called the cerebral cortex, the part of the brain that directs muscle movement.2
The symptoms of Cerebral Palsy differ in type and severity from one person to the next and may even change in an individual over time. Symptoms may vary greatly among individuals, depending on which parts of the brain have been injured.2 All people with cerebral palsy have problems with movement and posture, and some also have some level of intellectual disability, seizures, and abnormal physical sensations or perceptions, as well as other medical disorders.
Children with cerebral palsy are vulnerable to respiratory complications like frequent upper respiratory infections, aspiration, and pneumonia.3 Because these children typically move less, are unable to exercise, and their breathing is shallower, they are more likely to develop infections and struggle with breathing.
Children with cerebral palsy are likely to have breathing difficulties due to the above reasons and maybe even other causes still undiscovered. Infections are a major issue, especially pneumonia. This lung infection is the number one reason that children with Cerebral palsy are admitted to the hospital.4 Gastroesophageal reflux, a limited ability to cough or swallow normally, limited lung function, and lack of physical activity all contribute to the development of pneumonia and other respiratory infections.4
A child with Cerebral palsy may also experience atelectasis, the collapse of small parts of the lung obstructing airflow to those areas, or bronchiectasis, chronic inflammation caused by recurrent infections in the airways.5
Because there are potential respiratory issues experienced by children with Cerebral Palsy, respiratory health must be carefully and regularly monitored. Observation of the child’s breathing patterns can help a parent or caregiver know when something is different, which may prevent more serious issues like aspiration events or infections that can be deadly. Regular maintenance of good respiratory health can keep a child more comfortable and may also save lives.
Life expectancy for a child with Cerebral palsy is greatly impacted by respiratory health. Obvious reasons for this include infections like pneumonia and sleep apnea, but there are other, more subtle ways in which poor respiratory function shortens a child’s life expectancy such as weakened muscled from lack of activity and reduced lung function.4
A child may be unable to cough up mucus that physically obstruct their airways. Stagnant mucus can lead to infections and bacterial colonization. Either of these circumstances can cause lung infections and over time, can lead to a lethal cycle of recurrent infections and progressive pulmonary deterioration.5 A child’s respiratory health must be diligently monitored to treat symptoms and conditions before they become unmanageable. Proper management of respiratory health can improve the quality of life.
Impaired airway clearance may lead to the development of recurrent chest infections, bronchiectasis and eventually, respiratory failure. Respiratory management focuses on breaking the cycle of recurrent infections with aggressive attention to airway clearance. Airway clearance therapy (ACT) is suggested for children with Cerebral palsy.5 The degree of disability will influence the most appropriate ACT so it should be carefully individualized and reviewed regularly as the child grows.
Based on clinical experience, chest physiotherapy is an important tool to treat children with inefficient cough and chronic or recurrent lower airway infections. If the child cannot cough effectively or clear their secretions adequately or develops an acute lower respiratory tract infection, they may need chest physiotherapy.5
A less burdensome, yet effective airway clearance technique is the use of High Frequency Chest Wall Oscillation (HFCWO) therapy vests that apply vibratory forces that oscillate through the chest wall into the lungs to loosen and mobilize secretions. With the use of an HFCWO therapy vest, there is no need for a skilled caregiver and therapy can be easily performed on patients that are not capable of actively participating in therapy.6 Mobile HFCWO provides an enhanced airway clearance modality for individuals with cerebral palsy. AffloVest® was designed with ease-of-use in mind for patients, families and caregivers, to deliver airway clearance therapy that can be managed at home or in the hospital. With no bulky tubes and generators as found in other therapies, the AffloVest can be utilized in any postural position (i.e. laying down, standing, sitting, inclined, reclined etc.) all to help improve the quality of life for patients with neurological, neuromuscular and respiratory diseases.
The frequency of airway clearance needs to be customized to each patient and will need to be re-evaluated regularly throughout the patient’s disease course.
1. Susan Wiley MD, et al. Pediatric Clinical Advisor (Second Edition), 2007: 102-104
2. Singer S Harvey, et al. Movement Disorders in Childhood, 2010: 219-230
3. Morton RE, et al. Respiratory tract infections due to direct and reflux aspiration in children with severe neurodisability. Developmental Medicine & Child Neurology. 1999; 41(5):329–334.
4. Young NL, et al. Reasons for hospital admissions among youth and young adults with cerebral palsy. Arch Phys Med Rehabil 2011;92(1):46–50.
5. Fitzgerald, Dominic A. et al. Assessing and managing lung disease and sleep disordered breathing in children with cerebral palsy. Pediatric Respiratory Reviews 2009; 10(1): 18-24
6. D. Finder. Airway clearance modalities in neuromuscular disease. ediatric Respiratory Reviews 2010 Mar;11(1):31-4