Jack was diagnosed with severe hemophilia A at birth via cord blood. He has a 13 year old brother, who also has hemophilia and a previous inhibitor.
Jack had his first ankle bleed at 11 months and was started on recombinant prophylaxis. A month after treatment was started he was found to have an inhibitor from a routine titre check measuring 4.8 BU.
Prophylaxis was stopped and a port-a-cath was inserted in January 2008 in view of starting immune tolerance induction (ITI) treatment. Port insertion was covered with recombinant Factor VII, and ITI commenced with recombinant Factor VIII in April 2008 once the inhibitor titre had reached a level of < 10 BU. However, even with high dose ITI, Jack’s inhibitor level continued to rise and he was experiencing bleeds around the port site requiring intermittent recombinant Factor VII. At its highest, the inhibitor titre was 1.208 BU.
A year after starting recombinant ITI treatment, Jack was switched to Octapharma’s VWF/FVIII concentrate and his inhibitor titre has been steadily decreasing ever since. At the start of this treatment in April 2009, the titre was 861 BU but in January 2010, at a level of 54 BU.
Despite the challenges faced when living with an inhibitor, Jack is able to lead a normal life. He goes to nursery, takes part in all activities with his peers and plays and fights with his siblings like any other 3 year old.