Recommendations of the Czech National Hemophilia Program (CNHP) on Vaccination Against SARS-CoV-2

ČSH

Recommendations on vaccination against SARS-CoV-2 (COVID-19) were published earlier this year in the journal Haemophilia, and a brief translation is available on our website at this link. The recommendations are endorsed by the World Federation of Hemophilia (WFH), the European Association for Haemophilia and Allied Disorders (EAHAD), the European Haemophilia Consortium (EHC), and the National Hemophilia Foundation of the United States (NHF). These recommendations, including their Czech translation, already contain all the essential information.

Based on the questions we have received, the following points should be emphasized:

Vaccination against SARS-CoV-2 applies to adult patients. For those with FVIII/FIX levels below 10%, it is recommended to prophylactically administer FVIII/FIX concentrate at a dose of 20-30 IU/kg before intramuscular vaccination, ideally approximately 15-30 minutes before the injection;
Factor concentrate may also be administered prophylactically before vaccination in patients with higher coagulation factor levels than those specified in the recommendation above, if the patient has previously experienced bleeding symptoms more severe than would be typical for their coagulation factor level. This should always be assessed individually by a hemophilia centre physician (note the possible discrepancy between FVIII levels determined by the commonly used one-stage method and the less available chromogenic method in 5-10% of patients with mild hemophilia A);
If the patient is on prophylactic FVIII/FIX administration, vaccination should be timed to coincide with the day of concentrate administration. In the case of extended half-life (EHL) concentrates, depending on individual pharmacokinetics, it may be sufficient to carry out vaccination on the day after the prophylactic EHL dose;
For patients treated with emicizumab (for 4 weeks or longer), prophylactic administration of concentrate before vaccination is not necessary. When vaccination occurs less than 4 weeks after the initiation of prophylactic emicizumab treatment, the appropriateness of prophylactic concentrate administration must be individually assessed by a hemophilia centre physician;
For patients with inhibitors who are not prophylactically treated with emicizumab, a single dose of rFVIIa (NovoSeven) at 90 microg/kg or aPCC (FEIBA) at 75 U/kg must be administered before intramuscular vaccination, approximately 15-30 minutes before the injection. For NovoSeven, the interval may be shorter, 5-10 minutes;
Similar recommendations apply to patients with von Willebrand disease: prophylactic administration of concentrate when FVIII levels are below 10% and/or VWF:RCo activity is below 5% (type 3). For less severe forms, a single administration of Octostim intranasal spray one hour before vaccination may be considered; however, this product is currently temporarily unavailable in the Czech Republic (the defective batch on the market has not yet been replaced) and can only be administered as an intravenous infusion;
For patients with rare defects of other coagulation factors and thrombocytopenia or thrombocytopathy, an individual approach according to the recommendations of the hemophilia centre physician is necessary;
According to current knowledge, vaccination does not increase the risk of inhibitor development;
For vaccination of patients considering gene therapy in the future, an adenovirus-based vaccine may also be used, as a different type of virus is used for FVIII/FIX gene transfer in gene therapy;

Prepared by: MUDr. Petr Smejkal, Ph.D. 21 March 2021