Keeping up with developments

Changes in the conditions that affect children, new therapies and societal expectations mean that PRM is constantly moving forward. Table 4 presents examples of the way in which changing attitudes to paediatric conditions have revolutionised treatment and improved survival for many. Ensuring that children with life-limiting conditions, such as cystic fibrosis and muscular dystrophy (a condition causing weakness of the muscles, including the muscles of breathing), survive into adulthood is one of the successes of PRM, and liaison with colleagues in adult respiratory medicine has been important and successful. One emerging subspecialty in adult cardiac medicine is care for adults born with congenital heart conditions; in future, there is likely to be more demand for adult respiratory physicians with expertise in conditions such as cystic fibrosis and bronchopulmonary dysplasia (a condition in infants born very prematurely and caused partly as a side-effect of being on a ventilator and partly due to the lungs having to develop outside the womb). In future, a better understanding of the treatment of rare but serious conditions in PRM (often termed ‘orphan lung diseases’), such as bronchiolitis obliterans, will emerge as colleagues in PRM across Europe collaborate.

Condition Society/body
responsible
Guidelines
Asthma BTS and SIGN BTS and SIGN: British Guideline on the
Management of Asthma: a National
Clinical Guideline
  GINA International guidelines:
www.ginasthma.org/
  iCAALL International consensus on (ICON)
pediatric asthma
  PRACTALL Diagnosis and treatment of asthma in
childhood: a PRACTALL consensus report
Bronchiolitis SIGN Bronchiolitis in Children: a National
Clinical Guideline
  American Academy of
Pediatrics
Diagnosis and management of
bronchiolitis
Cystic fibrosis Cystic Fibrosis Trust UK guidelines:
www.cftrust.org.uk/aboutcf/publications/consensusdoc/
  European Cystic Fibrosis
Society
European consensus statements:
www.ecfs.eu/publications/consensus_reports
  Cystic Fibrosis Foundation US guidelines:
www.cff.org/treatments/CFCareGuidelines/
Spirometry in preschool
children
ATS/ERS An official American Thoracic Society/
European Respiratory Society Statement:
Pulmonary function testing in preschool
children

Table 3 – Guidelines for diagnosis, testing and management of paediatric respiratory conditions. The list is not exhaustive. Guidelines in Europe and the USA are very similar. BTS: British Thoracic Society; SIGN: Scottish Intercollegiate Guidelines Network; GINA: Global Initiative for Asthma; iCAALL: International Collaboration in Asthma, Allergy and Immunology; PRACTALL: Practicing Allergology or Practical Allergy; ATS: American Thoracic Society; ERS: European Respiratory Society.

A final aspiration is that in the future, children with and without respiratory problems will be able to breathe better-quality indoor and outdoor air. It is hoped that this will be achieved through legislation aimed at reducing children’s exposure to second-hand smoke and car exhaust fumes. The poet William Wordsworth observed that ‘The child is the father of the man’ and we know that the origins of many adult chest conditions, including asthma and chronic obstructive pulmonary disease, are determined in early life. Improving the quality of air entering children’s lungs every time they breathe has to be a priority.

Condition Incidence Approximate
births in
Europe n
Previous expectations Current expectations Implications
Cystic fibrosis 1 per 3000 births 1700 per year# 1950s: death in pre-school years was usual Half of babies born in 2012 can expect to live beyond 50 years of age There are more adult patients with CF than
children
Bronchopulmonary
dysplasia
1 per 3000 births 1700 per year#,¶ 1970s: nothing was done to help babies born at ≤28 weeks with breathing difficulties 90–95% likelihood
of survival if born
at 28 weeks; the majority survive without neurological
handicap. Risk of
neurological problems increases with greater prematurity.
Implications of
bronchopulmonary
dysplasia for adult life
unknown. More children with neurological problems become adults.
Muscular dystrophy 1 per 4000 males 1250 per year# 1990s: death in teens due to respiratory failure Survival into 20s with noninvasive ventilation Death from associated
heart defects
Table 4 – Conditions in which expectations have changed with implications for paediatric and adult respiratory medicine. #: assuming 5 million deliveries per year in Europe. : assuming 1 per 1000 born at ≤28 weeks’ gestation and one-third of these have bronchopulmonary dysplasia.

See the entire Paediatric respiratory medicine Chapter