Sickle cell disease (SCD) (sickle cell anaemia and related compound heterozygous states) manifests as a hypercoagulable state with high levels of chronic platelet activation, thrombin generation and inflammation. Venous thromboembolism (VTE) has been increasingly recognised as a common comorbidity in comprehensive reviews of SCD, with rates particularly increased when other risk factors for VTE are also present.
Regular red cell transfusion is an essential therapy for a subpopulation of patients with SCD or thalassaemia. The need for regular venous access can become challenging for a variety of reasons (e.g. poor peripheral veins or intense needle phobia, especially in children), but attempts to persevere with this option should always be explored, including ultrasound-guided cannulation or psychotherapy as indicated. Nonetheless, temporary or long-term central venous access devices (CVADs) can become a necessity. Temporary line insertion on the day of procedure, especially insertion of femoral central venous catheters (CVCs), is associated with increased rates of infective complications, bleeding risk and progressive tissue scarring making repeat insertion increasingly complex.1 Furthermore, in some cases, venous access may be required at other times for patient care, for example, ongoing chelation therapy. Indwelling CVADs (CVCs) require a surgical procedure for insertion, but have lower infection rates and present much simpler and quicker access on transfusion days and at other times. These are often therefore the preferred option for this patient cohort. However, catheter-associated VTE is a common and significant complication in these patients. Guidance for consideration of prophylactic anticoagulation to mitigate this risk has been developed based on published literature evidence.
Catheter-associated VTE was defined in most studies as a radiologically confirmed diagnosis of catheter-related thrombosis, right atrial or ventricular thrombus, upper or lower limb deep vein thrombosis (DVT), pulmonary embolism (PE) or VTE at other sites, confirmed by diagnostic imaging such as Doppler ultrasound, echocardiogram or computed tomography-pulmonary angiogram.
Declaration of Interests
The BSH paid the expenses incurred during the writing of this good practice paper. All authors have made a declaration of interests to the BSH and Task Force Chairs which may be viewed on request