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.