title: U.S. survey of surgical capabilities and experience with surgical procedures in patients with congenital haemophilia with inhibitors.
abstract: General guidelines exist for the use of recombinant activated factor VII (rFVIIa) to maintain haemostasis during surgery in congenital haemophilia A and B patients with high responding inhibitors (CHwI). Individual surgical plans are required and based upon historical therapy response, adverse events and anticipated procedure. Surgical interventions are feasible, yet it remains unclear how many US hemophilia treatment centres (HTCs) perform procedures in this fragile population. To better understand the US HTC surgical experience in CHwI patients and the number/types of procedures performed, a 21-question survey was sent to 133 US HTCs, with follow-up for response clarification and to non-responders. 98/133 HTCs (74%) responded, with 87 currently treating CHwI patients. In the last decade, 76/85 HTCs performed 994 surgeries on CHwI patients. Sites were experienced in the following procedures: central line insertion/removal (73 HTCs), dental (58), orthopaedic (52), abdominal (23), cardiovascular (14) and otolaryngologic (11). Experience with orthopaedic surgeries included synovectomies - arthroscopic (23 HTCs), radioisotopic (22), and open (7); joint replacement (18); fracture repair (14); and arthrodesis (8). Treatment modalities included rFVIIa bolus (83 HTCs) or continuous infusions (9), plasma-derived activated prothrombin complex concentrate (pd-aPCC) (55), antifibrinolytics (51), topical haemostatic agents (29), factor VIII (16) and fibrin sealants (14). Protocols for bypassing agents were used by 31/92 (33%) HTCs. Most US HTCs surveyed care for CHwI patients (74%) and have experience in minor surgery; fewer HTCs reported complex orthopaedic surgical experience. Identification of best practices and surgical barriers is required to guide future initiatives to support these patients.
authors.affiliation: Indiana Hemophilia and Thrombosis Center, Indianapolis, IN 46260, USA. ashapiro@ihtc.org
authors.forename: A
authors.initials: A
authors.lastname: Shapiro
authors.forename: D L
authors.initials: DL
authors.lastname: Cooper
mesh_terms: D000293:Adolescent; D001291:Attitude of Health Personnel; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002983:Clinical Competence; D006306:Health Surveys; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology; D002836:Hemophilia B / Q000150:complications* / Q000276:immunology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D013514:Surgical Procedures, Operative / Q000706:statistics & numerical data*; D011795:Surveys and Questionnaires; D014481:United States; D055815:Young Adult
chemical_list: D019774:Blood Coagulation Factor Inhibitors