A survey of the management of newborns with severe hemophilia in Canada

Abstract

Objective: To determine the practice patterns of Canadian hematologists and neonatologists/paediatricians who care for newborns with hemophilia, with regard to vitamin K administration, use of empirical clotting factor replacement therapy, neuroimaging and timing of hematology consultation.

Methods: Hematologists and neonatologists/paediatricians, identified from membership lists of Canadian professional organizations, were provided electronic and/or paper versions of the survey instrument. Questions were posed in the context of specific clinical scenarios. Differences in response proportions between groups were compared for selected questions.

Results: There were 171 respondents among 616 eligible persons who were sent the survey; 58 respondents had recent experience managing a newborn with hemophilia. There was a consensus not to provide empirical treatment to well newborns after uncomplicated deliveries, to provide empirical treatment to symptomatic newborns and to obtain neuroimaging for symptomatic newborns. Systematic differences between hematologists and neonatologists/paediatricians existed with regard to the timing of hematology consultation when the diagnosis of hemophilia had not been confirmed antenatally, the route of vitamin K administration for newborns with hemophilia and the choice of product to use for empirical treatment of a symptomatic newborn.

Conclusions: The observed lack of consensus regarding important management decisions indicates a need for ongoing research in the care of newborns with hemophilia. Systematic differences between hematologists and neonatologists/paediatricians suggest a role for improved communication and collaboration between these two groups of practitioners.

Keywords: Hemophilia A; Hemophilia B; Intracranial hemorrhages; Neuroimaging; Newborn.

Figure 1)

Conduct of the survey. ¹Physicians, not currently practicing: retired (n=1) and maternity leave (n = 2); ²Nonphysicians identified by Internet research: registered nurse (n=1), graduate student (n=1) and researcher (n=3); ³Valid e-mail address not available: Canadian Hematology Society members (n=49), invalid e-mail (n=32), no e-mail address available (n=6), previously opted out of Survey Monkey (n=5); ⁴A mailing address was not available for one person who had been sent the e-survey; ⁵Six respondents indicated by e-mail that they do not treat paediatric patients with hemophilia, which was considered equivalent to having responded to the survey and indicating that they had no recent experience managing newborns with hemophilia

Figure 2)

Response proportions for questions involving vitamin K administration. A Scenario 1: P>0.05.B Scenario 3: P=0.043. Questions regarding vitamin K administration were asked only in scenarios 1 and 3. Heme Hematologists; IM Intramuscular; Neo/Peds Neonatologists/paediatricians; PO Per os; SC Subcutaneous

Figure 3)

Response proportions for questions involving empirical treatment. A Scenario 1: P>0.05. B Scenario 2: P>0.05. C Scenario 3: P=0.02. Cryo Cryoprecipitate; FFP Fresh frozen plasma; Heme Hematologists; Neo/Peds Neonatologists/paediatricians; rFVIIa Recombinant activated factor VIIa; FVIII Factor VIII (either plasma-derived or recombinant)

Figure 4)

Response proportions for questions involving cranial imaging. A Scenario 1: P>0.05. B Scenario 2: P>0.05. C Scenario 3: P>0.05. CT Computed tomography; Heme Hematologists; MRI Magnetic resonance imaging; Neo/Peds Neonatologists/paediatricians; US Ultrasound