Starting prophylaxis with Factor VIII before two and a half years of age can help protect the joints of young hemophilic patients. This is the result of a recent study, which found a correlation between the age at which prophylaxis with Factor VIII is started and the protective effect on joints.
Decreased bone mineral density (BMD) is one of the most significant complications related to hemophilia and the cause of joint and bone degeneration in these patients. Recent studies have already shown that low-dose prophylaxis with Factor VIII, in severe hemophilia, preserves BMD, compared with on-demand therapy. Therefore, continuous and prolonged low-dose treatment with Factor VIII may prevent bleeding, as well as arthropathy.
We also now know that children who begin factor VIII prophylaxis before 2½ years of age have less joint damage at 6 years of age compared with children of the same age treated with factor VIII only when bleeding occurs. Moreover, follow-up of children shows that young children treated with prophylaxis starting at 2½ years of age also have healthier joints at age 18 years. The World Federation of Hemophilia also recommends starting prophylaxis before 3 years of age and before joint bleeds occur.
References
- Factor VIII: Long-established role in haemophilia A and emerging evidence beyond haemostasis, Bethany Samuelson Bannow, Michael Recht, Claude Négrier, Cédric Hermans, Erik Berntorp, Hermann Eichler, Maria Elisa Mancuso, Robert Klamroth, Jamie O’Hara, Elena Santagostino, Tadashi Matsushita, Craig Kessler, DOI: 10.1016/j.blre.2019.03.002
- Young adult outcomes of childhood prophylaxis for severe hemophilia A: results of the Joint Outcome Continuation Study. Beth Boulden Warren, Dianne Thornhill, Jill Stein, Michael Fadell, J. David Ingram, Sharon Funk, Kristi L. Norton, Heidi D. Lane, Carolyn M. Bennett, Amy Dunn, Michael Recht, Amy Shapiro, Marilyn J. Manco-Johnson. Blood Adv (2020) 4 (11): 2451–2459. https://doi.org/10.1182/bloodadvances.2019001311