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.
|Asthma||BTS and SIGN||BTS and SIGN: British Guideline on the
Management of Asthma: a National
|iCAALL||International consensus on (ICON)
|PRACTALL||Diagnosis and treatment of asthma in
childhood: a PRACTALL consensus report
|Bronchiolitis||SIGN||Bronchiolitis in Children: a National
|American Academy of
|Diagnosis and management of
|Cystic fibrosis||Cystic Fibrosis Trust||UK guidelines:
|European Cystic Fibrosis
|European consensus statements:
|Cystic Fibrosis Foundation||US guidelines:
|Spirometry in preschool
|ATS/ERS||An official American Thoracic Society/
European Respiratory Society Statement:
Pulmonary function testing in preschool
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.
|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
|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.
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