Respiratory muscle force and lung volume changes in a population of children with sickle cell disease

Bruce A. Ong, Jason Caboot, Abbas Jawad, Joseph Mcdonough, Tannoa Jackson, Raanan Arens, Carole L. Marcus, Kim Smith-Whitley, Thornton B.A. Mason, Kwaku Ohene-Frempong, Julian L. Allen

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Sickle cell disease (SCD) is a disorder known to impact the respiratory system. We sought to identify respiratory muscle force and lung volume relationships in a paediatric SCD population. Thirty-four SCD-SS subjects underwent pulmonary function testing. Height, weight, age, and gender-adjusted percent predicted maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) values were compared to spirometry and lung volumes. Statistical analyses were performed using Pearson's correlation coefficient and paired two-tailed t-test. The mean ± standard deviation (SD) MIP and MEP was 69·6 ± 31·6 cm H2O and 66·9 ± 22·9 cm H2O, respectively, and mean ± SD percent predicted MIP (101·3 ± 45·9) exceeded MEP (72·1 ± 26·0) (P = 0·002). MIP correlated with forced vital capacity (FVC; r = 0·51, P = 0·001) and TLC (r = 0·54, P < 0·0001). MEP also correlated with FVC (r = 0·43, P = 0·011) and total lung capacity (TLC; r = 0·42, P = 0·013). Pearson's correlation coefficient testing yielded relationships between MIP and MEP (r = 0·64, P < 0·0001). SCD-SS patients showed correlations between respiratory muscle force and lung volume, and reduced percent predicted expiratory muscle force compared to inspiratory muscle force. Respiratory muscle strength may affect lung volumes in these patients, and expiratory muscles may be more susceptible than the diaphragm to SCD-induced vaso-occlusive damage.

Original languageEnglish (US)
Pages (from-to)112-117
Number of pages6
JournalBritish Journal of Haematology
Issue number1
StatePublished - Oct 2013


  • Muscle strength
  • Paediatrics
  • Pulmonary function
  • Sickle cell anaemia

ASJC Scopus subject areas

  • Hematology


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