Hello Boston

This blog update is coming to you from the Medical Intensive Care Unit (MICU) at Boston Children's Hospital in Boston, MA. That's right, at just 4 months old our little Bug went on his first plane ride yesterday. He was airlifted here when doctor's at WCHOB said he was in liver failure and they no longer felt they could adequately care for him.

But, today is a new day. A fresh start. Brady has a brand new team of doctors with new ideas and perspective. Here at Boston Children's (BC for short!) they take a very team centered approach, prioritizing his diagnosis and then assigning teams accordingly. In B's case his most critical issue is hematologic so the hematology team is his primary team. Secondary to them is the hepatology (liver) team, and then pulmonology since his lung disorder is stable.

So far we have been blown away by BC. They've been right on top of things and making decisions from minute one...and...WE HAVE A DIAGNOSIS!!!!! Brady has Evans Syndrome.

Information about Evans Syndrome can be found here.

The hematology team is starting a medication called Rituximab this afternoon. This is a B cell suppressor that will hopefully knock down his immune response and slow his hemolysis. The issue is that he has been being transfused for so long that his transfusions are devastating his system with a toxic amount of iron. Another week of daily or almost daily transfusions would likely kill him. They need him to respond extremely quickly to this medication. If not, he will need to have a splenectomy as early as Friday or Monday.

Tim & I are feeling extremely angry about the care he's received the last few months. How could his WCHOB doctors not know that he would not survive continuing transfusions several times a week and not going forward with treatment. Instead of helping him they were essentially poisoning him. His body is so overloaded with iron that he could have permanent damage. Continued iron exposure in those doses would overwhelm his system to the point of multiple organ failure. One by one his liver, kidneys, lungs, and heart would fail. They almost killed our baby! We're relieved to have transferred and wish now that we had stuck to our instincts and done so back in December. We mistakenly believed what we were being told. We trusted that his doctors knew what they were doing. We were very very wrong.