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Central venous access devices in children with hemophilia: an update
V S Blanchette1, A Al-Musa, A M Stain
1Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Ontario, Canada.
Insights
Central venous access devices are recommended for young boys with severe hemophilia on long-term factor prophylaxis. Totally implantable devices are preferred due to lower infection risk and better quality of life for children.
Area of Science:
- Pediatric Hematology
- Vascular Access Devices
- Hemophilia Management
Background:
- Severe hemophilia in young boys necessitates long-term factor prophylaxis to prevent joint bleeding and musculoskeletal disease.
- Factor prophylaxis involves regular intravenous infusions, posing challenges for venous access.
- Central venous access devices (CVADs) are frequently considered to facilitate this regimen.
Purpose of the Study:
- To evaluate the role and recommendations for central venous access device placement in young boys with severe hemophilia undergoing factor prophylaxis.
- To discuss the risks and benefits of CVADs in this patient population.
- To provide guidance on the type of CVAD to be used if indicated.
Main Methods:
- Review of current clinical practices and recommendations for CVAD use in hemophilia.
- Discussion of the efficacy and complications associated with factor prophylaxis.
- Analysis of risk-benefit considerations for CVAD placement in pediatric hemophiliacs.
Main Results:
- Factor prophylaxis is highly successful in preventing bleeding and chronic disease in severe hemophilia.
- Some clinics routinely recommend CVADs, while others prefer peripheral access due to concerns about sepsis.
- Totally implantable devices are recommended over other types due to lower infection risk and improved patient quality of life.
Conclusions:
- The decision to use a CVAD for hemophilia prophylaxis requires careful discussion of risks and benefits with guardians.
- Totally implantable devices (e.g., Port-A-Cath) are the preferred type of CVAD for pediatric hemophilia patients.
- Catheter-related sepsis is a significant complication, occurring in up to 25% of devices, with deep vein thrombosis being less frequent.
Abstract:
The most frequent indication for placement of a central venous access device in hemophiliacs is in very young boys (ages 1-2 years) with severe hemophilia who are started on a program of long-term factor prophylaxis designed to eliminate target joint bleeding and the development of chronic musculoskeletal disease. Although expensive, this strategy is extremely successful. It involves intravenous infusion of 25-40 factor units per kg on alternate days (minimum 3 times a week) for boys with severe hemophilia A, and twice a week for boys with severe hemophilia B. To facilitate this prophylaxis regimen some hemophilia clinics routinely recommend placement of a central venous access device; others, more concerned about associated complications such as sepsis, stress the importance of using peripheral veins wherever possible, with central access devices reserved for occasional, selected cases only. A decision to use such a device should only be made after discussion of the risks/benefits with parents (or guardians) and with patients if of an appropriate age. If such a system is to be used, we recommend that a totally implantable device (Port-A-Cath) be placed because of the lower risk of infection, and because totally implantable devices allow children to take part in activities such as swimming. Important complications include catheter-related sepsis, which may occur in 25% or more of devices over time and, much less frequently, catheter-related deep vein thrombosis.