According to the ASH guidelines, when is pulmonary function testing (PFT) indicated in the management of sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

For asymptomatic children and adults with SCD, the ASH guideline panel suggests against routine screening pulmonary function testing (PFT).

The following signs, symptoms, or diagnoses may warrant diagnostic PFT to evaluate for abnormal lung function:

  • Wheezing or increased cough at rest, with exertion, or during episodes of acute upper respiratory infection
  • Dyspnea at rest or with exertion that is increased compared with baseline or that is unexplained
  • Chest pain at rest or with exertion that is out of proportion to a known condition, that is increased compared with baseline, or that is unexplained
  • Increase in exercise limitation compared with baseline or that is unexplained (eg, not resulting from sickle cell pain or musculoskeletal disease)
  • Abnormal 6MWD defined by either reduced 6MWD or oxygen desaturation during test
  • History of recurrent hypoxemia at rest or with exertion
  • History of syncope or presyncope
  • History of recurrent acute chest syndrome
  • History of pulmonary embolism

Comprehensive PFT should include full spirometry as well as complete evaluation of diffusion capacity and lung volumes.


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