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Lung Function from Infancy to the Preschool Years after Clinical Diagnosis of Cystic Fibrosis

Wanda J. Kozlowska¹, Andrew Bush², Angela Wade³, Paul Aurora^1,4, Siobhán B. Carr⁵, Rosie A. Castle^1,, Ah-Fong Hoo¹, Sooky Lum¹, John Price⁶, Sarath Ranganathan⁷, Clare Saunders¹, Sanja Stanojevic^1,3, John Stroobant⁸, Colin Wallis⁴ and Janet Stocks¹ for the London Cystic Fibrosis Collaboration^*

¹ Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, University College London Institute of Child Health, London, United Kingdom; ² Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom; ³ Centre for Paediatric Epidemiology and Biostatistics, University College London Institute of Child Health, London, United Kingdom; ⁴ Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom; ⁵ Department of Respiratory Paediatrics, Barts and The London Children's Hospital, London, United Kingdom; ⁶ Department of Child Health, Kings College Hospital, London, United Kingdom; ⁷ Department of Paediatrics, Royal Children's Hospital Melbourne, Murdoch Children's Research Institute, and Department of Paediatrics, University of Melbourne, Melbourne, Australia; and ⁸ Department of Child Health, University Hospital Lewisham, London, United Kingdom

Correspondence and requests for reprints should be addressed to Wanda Kozlowska, M.B.B.S., M.R.C.P.C.H., Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. E-mail: w.kozlowska{at}ich.ucl.ac.uk

Rationale: After recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth.

Objectives: The aim of this study was to investigate the evolution of lung function during the first 6 years of life after a clinical diagnosis of cystic fibrosis (CF) in infancy in children with CF and in healthy control subjects.

Methods: The raised volume technique was used during infancy and incentive spirometry during the preschool years.

Measurements and Main Results: Forty-eight children with CF and 33 healthy control subjects had up to seven (median, 3) measurements. Over these early years, the diagnosis of CF itself accounted for a significant mean reduction of 7.5% (95% confidence interval, 0.9 – 13.6%) in FEV_0.75 and 15.1% (95% confidence interval, 3.6 – 25.3%) in FEF_25–75. Wheeze on auscultation, recent cough, and Pseudomonas aeruginosa (PsA) infection (even if apparently effectively treated) were all independently associated with further reductions in lung function. Premorbid lung function did not predict infection with PsA.

Conclusions: This is the first study to describe physiologic measurements from infancy through the preschool years in subjects with CF and healthy control subjects, the understanding of which is critical for future intervention trials. Airflow obstruction in uncomplicated CF persists through the preschool years despite treatment, with PsA acquisition being associated with further deterioration in lung function, even when apparently eradicated. This suggests that new therapies are needed to treat the airflow obstruction of uncomplicated CF, and rigorous strategies to prevent PsA acquisition.

Key Words: spirometry • Pseudomonas aeruginosa • infant • preschool child

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Lung function has been found to be reduced in infants with cystic fibrosis. Spirometry, now standardized for preschool children, allows longitudinal testing from infancy to school age. What This Study Adds to the Field Lung function was persistently reduced in patients with cystic fibrosis, particularly in patients with either current or previous Pseudomonas infection.

 

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Reduced Lung Function in Cystic Fibrosis: A Primary or Secondary Phenotype?

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AJRCCM 2008 178: 2-3. [Full Text]