Dear Editor,
Physiotherapy and intravenous antibiotics are the mainstay of treatment of pulmonary exacerbations in patients with cystic fibrosis (CF).1 Intravenous antibiotics are commonly delivered via peripherally inserted central catheters (PICC lines)1-2. A relatively frequent complication of PICC lines are deep vein thrombosis (DVT) with a reported Incidence in CF of ~ 4.5% 2, similar to non-CF patients.3 DVT may lead to life threatening pulmonary emboli (PE) and therefore prevention, early diagnosis and appropriate management are required.4
Guidelines for DVT prevention and management are available and mainly discuss the use of anticoagulation4 which in patients with CF may increase the risk for hemoptysis, a relative contraindication for physiotherapy1. Clinical practice guidelines for physiotherapy management of patients with upper limb DVT are unavailable. However, guidelines for lower extremity DVT state that due to the risk for developing PE, “mobility is contraindicated until intervention is initiated to reduce the chance of emboli”.5
Thus, in patients with CF and an upper limb DVT it is prudent to remain alert for hemoptysis and to withhold physiotherapy until the risk for PE is reduced. However, withholding physiotherapy during a pulmonary exacerbation, may carry a risk of further respiratory deterioration.
We herein describe our experience with physiotherapy management of two patients with severe CF lung disease, admitted due to a pulmonary exacerbation, in whom a DVT developed adjacent to their PICC line catheter.
Patient A, a 16-year-old male, with DF508/W1282X CFTR genotype, suffering from a severe pulmonary disease with a baseline forced expiratory volume in 1 second (FEV1) of 32 %predicted and recurrent pulmonary exacerbations for which a PICC line was inserted. On March 2019, he suffered a severe pulmonary exacerbation and was admitted. On day 17th of his admission, a DVT in the left subclavian vein, adjacent to the PICC line, was identified following complaints of acute arm and chest pain.
Patient B, an 11-year-old female, homozygous for the DF508 CFTRmutation, suffering from a severe pulmonary disease, with a baseline FEV1 of 26 % predicted, requiring nocturnal non-invasive ventilation and listed for lung transplantation. On November 2019, she was admitted to the hospital with a life-threatening pulmonary exacerbation for physiotherapy and a prolonged course of intravenous antibiotic treatment via a newly inserted PICC line catheter. During her admission, following complaints of chest pain, she was found to have DVTs in the Rt. Jugular subclavian and brachial axial veins.
For both patients physiotherapy was considered crucial.
Standard physiotherapy care in our hospital for CF patients admitted for pulmonary exacerbations includes: A morning session consisting of exercise combined with airway clearance therapy (ACT), an afternoon exercise session at the gym, and an evening ACT session, with either a physiotherapist or by using a high frequency chest oscillator (vest). ACT, in our center, includes a combination of manual chest physiotherapy, breathing exercises (modified autogenic drainage and modified active cycle breathing techniques) and the use of positive expiratory pressure (PEP) devices.
We conducted a multidisciplinary consultation including a pediatric pulmonologist, a hematologist and senior physiotherapy staff from the CF center and the intensive care units in our hospital. We formed an approach to physiotherapy treatment which we considered both safe and effective (Table 1). The restriction were continued until DVT resolution.
Table 1. Framework for physiotherapy management of patients with CF and upper limb DVT receiving anticoagulation treatment.