Advertisement

Routine clinical monitoring fails to identify children at high risk of lung function decline

  • Lucy Perrem
    Correspondence
    Correspondence to: Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1 × 8, Canada.
    Affiliations
    Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Canada

    Department of Paediatrics, University of Toronto, Canada

    Translational Medicine Program, SickKids Research Institute, Toronto, Canada
    Search for articles by this author
Published:August 29, 2022DOI:https://doi.org/10.1016/j.jcf.2022.08.017
      Despite improvements in survival from cystic fibrosis (CF) over successive decades, many infants with CF have diminished lung function within months of being diagnosed on newborn screening [
      • Linnane B.M.
      • Hall G.L.
      • Nolan G.
      • Brennan S.
      • Stick S.M.
      • Sly P.D.
      • Robertson C.F.
      • Robinson P.J.
      • Franklin P.J.
      • Turner S.W.
      • Ranganathan S.C.
      Lung function in infants with cystic fibrosis diagnosed by newborn screening.
      ,
      • Hoo A.F.F.
      • Thia L.P.
      • Nguyen T.T.D.
      • Bush A.
      • Chudleigh J.
      • Lum S.
      • et al.
      Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening.
      ]. Early childhood has been identified as a crucial period in the establishment of future lung disease. However, the age of onset and progression of CF lung disease is highly variable, creating uncertainty as to how proactively children should be monitored and treated. Reducing the burden of daily treatments is a priority for the CF community [
      • Bayfield K.J.
      • Douglas T.A.
      • Rosenow T.
      • Davies J.C.
      • Elborn S.J.
      • Mall M.
      • Paproki A.
      • Ratjen F.
      • Sly P.D.
      • Smyth A.R.
      • Stick S.
      • Wainwright C.E.
      • Robinson P.D.
      Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.
      ], especially as the use of highly effective modulator therapies becomes widespread. There is therefore a need to identify children at high risk of irreversible lung damage who would benefit most from intensive management strategies. In this issue of the Journal, research groups from both the UK and Australia report prospective observational data investigating early life factors associated with loss of lung function at different periods in childhood.

      Abbreviations:

      LCFC (London Cystic Fibrosis Collaboration), ACFBAL (Australasian Cystic Fibrosis Bronchoalveolar Lavage), BAL (bronchoalveolar lavage), PsA (Pseudomonas aeruginosa), AREST-CF (Australian Respiratory Early Surveillance Team for Cystic Fibrosis)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Cystic Fibrosis
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Linnane B.M.
        • Hall G.L.
        • Nolan G.
        • Brennan S.
        • Stick S.M.
        • Sly P.D.
        • Robertson C.F.
        • Robinson P.J.
        • Franklin P.J.
        • Turner S.W.
        • Ranganathan S.C.
        Lung function in infants with cystic fibrosis diagnosed by newborn screening.
        Am J Respir Crit Care Med. 2008; 178: 1238-1244
        • Hoo A.F.F.
        • Thia L.P.
        • Nguyen T.T.D.
        • Bush A.
        • Chudleigh J.
        • Lum S.
        • et al.
        Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening.
        Thorax. 2012; 67 ([Internet]Available from): 874-881
        • Bayfield K.J.
        • Douglas T.A.
        • Rosenow T.
        • Davies J.C.
        • Elborn S.J.
        • Mall M.
        • Paproki A.
        • Ratjen F.
        • Sly P.D.
        • Smyth A.R.
        • Stick S.
        • Wainwright C.E.
        • Robinson P.D.
        Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.
        ThoraxThorax. 2021; ([Internet] BMJ Publishing Group Ltd[cited 2021 Apr 30];: thoraxjnl-2020-216085Available from)
        • Thia L.P.
        • Calder A.
        • Stocks J.
        • Bush A.
        • Owens C.M.
        • Wallis C.
        • Young C.
        • Sullivan Y.
        • Wade A.
        • McEwan A.
        • Brody A.S.
        Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?.
        Thorax. 2014; 69 ([Internet] BMJ Publishing Group[cited 2022 Jul 21]Available from: /pmc/articles/PMC3963531/): 320-327
        • Nguyen T.T.D.D.
        • Thia L.P.
        • Hoo A.F.F.
        • Bush A.
        • Aurora P.
        • Wade A.
        • et al.
        Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants.
        Thorax. 2014; 69 ([Internet] BMJ Publishing Group[cited 2019 May 8]Available from): 910-917
        • Aurora P.
        • Duncan J.A.
        • Lum S.
        • Davies G.
        • Wade A.
        • Stocks J.
        • et al.
        Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis.
        J Cyst Fibros. 2022; ([Internet] J Cyst Fibros[cited 2022 Aug 15]; Available from)
        • Stanojevic S.
        • Davis S.D.
        • Retsch-Bogart G.
        • Webster H.
        • Davis M.
        • Johnson R.C.
        • Jensen R.
        • Pizarro M.E.
        • Kane M.
        • Clem C.C.
        • Schornick L.
        • Subbarao P.
        • Ratjen F.A.
        Progression of lung disease in preschool patients with cystic fibrosis.
        Am J Respir Crit Care Med. 2017; 195 ([Internet] American Thoracic Society[cited 2019 Mar 28]Available from): 1216-1225
        • Begum N.
        • Byrnes C.A.
        • Cheney J.
        • Cooper P.J.
        • Fantino E.
        • Gailer N.
        • Grimwood K.
        • GutierrezCardenas D.
        • Massie J.
        • Robertson C.F.
        • Sly P.D.
        • Tiddens H.A.
        • Wainwright C.E.
        • Ware R.S.
        Factors in childhood associated with lung function decline to adolescence in cystic fibrosis.
        J Cyst Fibros. 2022; ([Internet] J Cyst Fibros[cited 2022 Aug 15]; Available from)
        • Sagel S.D.
        • Wagner B.D.
        • Anthony M.M.
        • Emmett P.
        • Zemanick E.T.
        Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis.
        Am J Respir Crit Care Med. 2012; 186 ([Internet][cited 2022 Aug 24]Available from): 857-865
        • Ramsey K.A.
        • Ranganathan S.
        • Park J.
        • Skoric B.
        • Adams A.-.M.
        • Simpson S.J.
        • Robins-Browne R.M.
        • Franklin P.J.
        • de Klerk N.H.
        • Sly P.D.
        • Stick S.M.
        • Hall G.L.
        Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis.
        Am J Respir Crit Care Med. 2014; 190 ([Internet] American Thoracic Society[cited 2020 Sep 8]Available from): 1111-1116
        • Ranganathan S.C.
        • Hall G.L.
        • Sly P.D.
        • Stick S.M.
        • Douglas T.A.
        Early lung disease in infants and preschool children with cystic fibrosis: what have we learned and what should we do about it?.
        Am J Respir Crit Care Med Am Thoracic Soc. 2017; : 1567-1575
        • Ong T.
        • Schechter M.
        • Yang J.
        • Peng L.
        • Emerson J.
        • Gibson R.L.
        • Morgan W.
        • Rosenfeld M.
        Socioeconomic status, smoke exposure, and health outcomes in young children with cystic fibrosis.
        Pediatrics. 2017; ([Internet] Pediatrics[cited 2022 Aug 9]Available from): 139
        • Collaco J.M.
        • Blackman S.M.
        • McGready J.
        • Naughton K.M.
        • Cutting G.R.
        Quantification of the relative contribution of environmental and genetic factors to variation in cystic fibrosis lung function.
        J Pediatr. 2010; ([Internet] J Pediatr[cited 2022 Aug 9]; 157Available from)
        • Collaco J.M.
        • Vanscoy L.L.
        • Psoter K.J.
        • Riekert K.A.
        • Dickinson K.M.
        Clinical outcomes in cystic fibrosis at 6 years of age with Tricare insurance coverage.
        J Cyst Fibros. 2022; (Elsevier)
        • Wainwright C.E.
        • Vidmar S.
        • Armstrong D.S.
        • Byrnes C.A.
        • Carlin J.B.
        • Cheney J.
        • et al.
        Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial.
        JAMA - J Am Med Assoc. 2011; 306: 163-171