All potential candidates undergo a comprehensive multi-disciplinary evaluation that includes:

Pulmonary Function Testing

  • Spirometry
  • Lung volumes
  • DLCO
  • Blood gases
  • Maximal inspiratory and expiratory pressures
  • Infant pulmonary function testing is performed for most children under 5

Cardiac Evaluation

  • EKG
  • 2D echocardiogram
  • cardiac catheterization (as needed to evaluate pulmonary vascular disease)

Imaging

  • Chest x-rays
  • High resolution chest CT
  • V/Q scan (in selected cases)

Laboratory Evaluation

  • Measures of renal and liver function
  • Sputum culture
  • Viral serologies (EBV, CMV, Varicella, herpes, Hepatitis, HIV)

Other Studies

  • Nutritional evaluation
  • Physical therapy evaluation (including 6 minute walk test)
  • Psychosocial evaluation