Therapeutic choices in persons with haemophilia at the time of COVID-19

Dear Sir,

The healthcare emergency during the Coronavirus Disease 2019 (COVID-19) pandemic and the lockdown measures reducing access to treatment centres, laboratories and pharmacies challenge the management of patients with chronic diseases or requiring regular follow up, including persons with haemophilia (PWH)¹. Specialist centres provide emergency and urgent visits, and therapeutic supply is ensured by legal provision, without standard clinical/laboratory monitoring.

This crisis occurs at a moment of important advances in the treatment of haemophilia². Prophylaxis with extended half-life (EHL) factor VIII (FVIII) and, particularly, factor IX (FIX) concentrates allow prolonged intervals between intravenous infusions and higher protection from bleeding thanks to increased factor trough levels. The FVIII-mimetic bispecific monoclonal antibody emicizumab provides highly effective prophylaxis in PWH A with and without inhibitors, with subcutaneous administration every 1–4 weeks, avoiding intravenous injections. Some products and indications (pegylated FVIII concentrates, emicizumab in non-inhibitor patients) have very recently been registered in some European countries. Therefore, PWH and their treating physicians can benefit from the advantages of new treatment choices, which are even more important under the lockdown restrictions by reducing the burden on medical care³, but, at the same time, they have to face the difficulties of the switching phase.

Close monitoring is, indeed, crucial when changing therapeutic products in PWH. Regular assessments of bleeding rates, joint status, lifestyle and physical activity, adherence to treatment, pharmacokinetics and trough levels are useful to personalise prophylaxis with EHL concentrates and optimise outcomes⁴. Patients need to be trained to help them adopt changes in lifestyle in the light of the new treatment, and to identify possible changes in efficacy and safety as early as possible.

Subcutaneous administration is expected to facilitate the prompt acceptance and the transition to emicizumab. However, real-word data revealed the unanticipated fear and resistance to subcutaneous injections in many patients, requiring intensive training to help them overcome this⁵. Moreover, patients on emicizumab need to be educated as to how to safely manage breakthrough bleeding.

These multi-faceted implications of COVID-19 pandemic in therapeutic choices in PWH have been poorly addressed so far. The World Federation of Hemophilia states that there is no reason to change the recommended treatment regimen; however, this mainly refers to product availability and supply. Some authors highlight the fact that, in keeping with the emergency, advantages from the new treatments are expected to have a huge impact both in their use in the short and long term³. Given the lack of adequate patient follow-up and education, this perspective is not realistic. New approaches in telemedicine and social media communication are being implemented¹ and will likely contribute to optimise therapeutic choices in PWH according to their individual needs, in consideration of new options and preserving safety.