Cerebral Palsy and Lung disease

By Dr Rachael Marpole, Respiratory and General Paediatric Advanced trainee, Perth Children’s Hospital; Dr Andrew Wilson, Consultant and Head of Respiratory Department, Perth Children’s Hospital; Dr Noula Gibson, Research Coordinator, Physiotherapy Department, Perth Children’s Hospital; Dr Katherine Langdon, Rehabilitation Paediatrician, Kids Rehab, Perth Children’s Hospital

Cerebral palsy (CP) is the most common physical disability in childhood. The prevalence of CP is 1.4 per 1000 live births. It is a heterogeneous group of disorders, caused by a non-progressive lesion in the developing brain. CP can present with other comorbidities such as epilepsy or secondary complications such as progressive musculoskeletal issues, or respiratory disease. Whilst the secondary complications to the musculoskeletal system are relatively well known, the impact on the respiratory system is less known.

Respiratory disease in CP is the most common cause of mortality, morbidity and poor quality of life in the most severely affected. Children with CP are admitted more often and for longer than other children, with respiratory admissions being the most common cause of admission. Parents report that unplanned hospital admissions have a significant impact on quality of life. Also, adults with CP are 14 times more likely to die from respiratory diseases than the general adult population.

The respiratory disease process in CP is mainly caused by aspiration with the added issues of gastrointestinal dysfunction, motor impairment and epilepsy. Respiratory disease in CP presents as recurrent episodes of respiratory failure/wheezing with otherwise mild illnesses and/or silent progressive disease. Red flags include – severe cerebral palsy (Gross motor functional scale V), admission to hospital for respiratory illness during the previous year, or patients having required two courses of oral antibiotics for respiratory illness within 12 months. Potentially modifiable risk factors include oropharyngeal dysphagia (meal-time modifications), frequent respiratory symptoms, current seizures, gastrooesophageal reflux, mealtime respiratory symptoms and snoring every night See figure 1.1

The modifiable risk factors all have multiple different treatment options (see consensus statement  ). By treating these we may reduce or prevent respiratory disease in this group improving mortality, morbidity, and quality of life.

What does this  mean for GPs and primary health care providers?

  • Be aware that children with cerebral palsy and other severe neurodisability are at increased risk of respiratory disease.
  • Be aware of the risk factors.
  • Prevention by treating potentially modifiable risk factors is key.
  • Earlier treatment of wet sounding cough with antibiotics is indicated in these children, even if the rest of their examination is stable. Children with CP and wet cough may need a prolonged course of antibiotics as they are unable to effectively cough.
  • If there are more than two episodes of antibiotics required for respiratory illness in 12 months consider referral to a respiratory doctor.
  • Children with oropharyngeal dysphagia may not cough but present with gurgling/ wheezing during and post feeding.
  • Aspiration is likely occurring all the time. There does not need to be an episode of choking/vomiting. Check that parents have had their child’s meal time management assessed by a speech pathologist.
  • Gastrooesophageal reflux is common in children with CP. Management includes thickening feeds, sitting upright post meals, having small frequent meals and treating constipation. A trial of proton pump inhibitors for symptoms may be helpful. If there is associated delayed gastric emptying a hydrolysed formula may be tried. If this fails, consider referral to a gastroenterologist for further assessment and interventions.

For more information contact Rachael.Marpole@health.wa.gov.au

References

  1. Gibson N, Blackmore AM, Chang AB, Cooper MS, Jaffe A, Kong WR, et al. Prevention and management of respiratory disease in young people with cerebral palsy: consensus statement. Dev Med Child Neurol. 2021;63(2):172–82.

 

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