Gastrointestinal | Baseline | |
---|---|---|
 | All patients should have a barium swallow to assess for dysmotility or stricture and 24 hour pH monitoring for GORD and progress to upper GI endoscopy if any abnormality detected | |
 | Follow-up | |
 | Every 3 years or sooner if worsening lung involvement and/or worsening GI symptoms | Upper GI endoscopy Barium swallow 24 hours pH monitoring |