06/10/2026
Hello everyone!
I wanted to share a quick update on Bennett after our trip to Pittsburgh this week for kidney testing and follow-up appointments with his immunology team.
We received some very encouraging news. At this point, there does not appear to be any permanent damage to Bennett’s kidneys. The team believes the changes they are seeing may be related to the antibodies in his Hizentra infusions. In some cases, the body adapts over time and the issue resolves on its own. Another option would be switching to a different antibody product, but since Hizentra has been very effective for Bennett, we don’t want to make any unnecessary changes right now. We will repeat testing in a few months and, depending on those results, discuss whether a change in treatment is needed.
We also learned about a new functional test that is currently being released through the FDA approval process. This test could provide a much clearer answer as to whether Bennett’s PIK3 gene variant is actively causing the immune dysfunction we have been seeing. While he has many of the clinical symptoms associated with APDS, he only has one significant genetic variant, so additional testing is needed to confirm whether it is truly driving his disease.
If the testing confirms this, it could help qualify him for a newly approved medication that specifically targets the PI3Kδ protein. This treatment is designed to address the underlying immune dysregulation and deficiency that can lead to recurrent infections, bronchiectasis, autoimmune complications, lymphoproliferation, and an increased risk of lymphoma.
Most importantly, we are finally making progress. We have answers, we have a plan, and we have options. This was by far one of the most reassuring appointments we’ve had in quite some time.
As for Bennett, he also had blood work scheduled, but thanks to his very kind immunologist, he was granted a short reprieve after enduring an extensive round of testing last week. He happily accepted the temporary break from needles!
❤️ The best part of this update is that for the first time in a while, the team of specialists are discussing solutions rather than searching for problems. That’s a big step forward.
