We report physiotherapy management of two patients with severe cystic fibrosis (CF) lung disease and upper limb deep vein thrombosis (DVT). These patients were admitted due to a pulmonary exacerbation. Following peripherally inserted central catheters they were diagnosed with an upper limb DVT. Due to their underlying lung disease, physiotherapy was mandatory for improvement. However, the DVT and anticoagulation treatment raised concerns for pulmonary emboli and hemoptysis. A framework for physiotherapy management in these patients, using a set of precautions and restrictions to maintain airway clearance while minimizing risk for pulmonary emboli and hemoptysis, was established. Using these set of instructions, the patients experienced no major adverse event while maintaining sufficient airway clearance to allow respiratory improvement. These precautions were continued until the upper limb DVTs resolved. To our knowledge there are currently no guidelines nor expert opinions available. Therefore, this framework can help guide physiotherapy management.

Background Normal values (>80%) of Forced Expiratory Volume in one second (FEV1) in patients with Cystic fibrosis (CF) may lead to the interpretation that there is no lung disease. This study is a comprehensive analysis of lung involvement in CF patients having normal FEV1. Methods Patients were recruited from two CF Centers: Hadassah Medical Center, Jerusalem and Vall d’ Hebron Hospital, Barcelona. Lung disease was assessed by lung clearance index (LCI), chest CT-Brody Score, respiratory cultures, number of pulmonary exacerbations (PEx) and days of antibiotic treatment in the year prior to the assessment. Results Of the 247 patients, 89 (36%) had FEV1 ≥80% and were included in the study (mean age 17.6 range 4.25-49 years). Chronic P. aeruginosa infection was found in 21%, and 31% had at least one major PEx in the year prior to the study. Abnormally elevated LCI was found in 86% of patients, ranging between 7.52-18.97, and total Brody score (TBS) was abnormal in 92% (range 5.0-96.5). Patients with chronic P. aeruginosa had significantly higher LCI (p=0.01) and TBS (p=0.01) which were associated with more major PEx (p = 0.04 and p<0.001, respectively) and more days of intravenous (IV) antibiotic treatment in the preceding year (p=0.03 and p=0.002, respectively). Conclusions Most CF patients with normal FEV1 have already physiological and structural lung abnormalities which were associated with more PEx and IV antibiotic treatment.