Central venous access device insertion and perioperative management of patients with severe haemophilia A

Abstract

Central venous access device (CVAD) insertion is one of the most common procedures performed on paediatric haemophilia patients. There are no clear guidelines outlining the optimal dosing schedule of factor VIII (FVIII) and duration of treatment required to achieve adequate haemostasis during and after surgery. In this article, we describe the experience at McMaster Children's Hospital using FVIII replacement therapy in 15 children with severe haemophilia A during the course of 7 years. This is a retrospective institutional chart review. Patients between 0 and 18 years of age with severe haemophilia A that underwent CVAD insertion at McMaster Children's Hospital in Hamilton, Ontario, from 2004 to 2010, were identified and charts were reviewed. A total of 15 CVAD insertion surgeries were reviewed. The total average preoperative dose of FVIII was 93.5 IU/kg (range: 53.7-145.4 IU/kg). The total average postoperative dose was 818.7 IU/kg (range: 441-1258 IU/kg). The total perioperative dose was 912.2 IU/kg (range: 495.2-1349 IU/kg). The current study attempts to describe the experience at McMaster Children's Hospital for CVAD insertion surgeries, the average factor dose administered has decreased during the years. These results may be of help in the development of optimal treatment schedules.

Central venous access devices implantation in children with severe hemophilia a: data from the children comprehensive care center of China

“…However, there is a limited number of published studies reporting the management of CVAD insertions in children. The experience of the McMaster Children's Hospital showed that the dose of factor administration had gradually decreased over time and that the total perioperative dose was 912.2 IU/kg [ 15 ]. Minna et al [ 16 ] proposed that less than 4 days may be as safe and effective as longer replacement therapy.…”

Correction of haemostasis can be reduced to four days for CVAD implantation in severe haemophilia A patients: Data from the PedNet study group

“…There is an increased need to find cost‐effective CFC replacement treatment for CVAD surgery without compromising the safety. Fonseca et al recently reported the experience at McMaster Children's Hospital to find an optimal haemostatic coverage during CVAD surgery 5 . They concluded that decreased FVIII dosage resulted in a similar successful outcome with no bleeding complications.…”

“…The incidence of bleeding associated with CVAD surgery has been reported to vary between 7% and 22% according to previous studies 6 . Little knowledge on optimal replacement therapy for CVAD insertion is available beyond single centre experiences 5,6 . As intensive treatment is a risk factor for antibody formation, 4 many physicians who treat for 5 days or longer try to minimize the risk by postponing the time of insertion.…”

“…Use of central venous access devices (CVADs) overcomes these difficulties and makes it easier for caregivers to administer factor infusions (for prophylaxis and bleed management) in the home setting 1,2 . Clinical practises vary between different centres with some centres inserting CVADs in most children with severe HA who start prophylaxis, 3‐5 while other centres make every effort to avoid these.…”

Perioperative haemostasis in children with haemophilia and inhibitors during central venous catheter surgery: The Karolinska model

“…Given its rarity, reports from collaborative European efforts such as the European Acquired Haemophilia registry (EACH-2)3,[6][7][8] and the GTH (Acquired Haemophilia Working Group of the German, Austrian and Swiss Thrombosis and Hemostasis Society) registry9,10 have provided key insights. Toronto ' s St. Michael ' s Hospital (SMH) is the largest Canadian adult haemophilia treatment centre (HTC); we reviewed the clinical characteristics of AHA patients treated at SMH from 1990 to 2016.…”