WFMC

First off, I want to thank everyone for the very thoughtful and caring calls and emails. We really appreciate all the of the love, support, and help that you all have provided during this crazy time for the Koch family.

Today was a power packed day and Audrey handled it all like a champ. We started the day with an xray appointment and then an electrocardiogram appointment. Both of those went really smooth as Mayo really has their processes down pat and move patients through quickly. At that point it was about 10am and Audrey was tired so she took a quick nap for a little while before the next appointment. We then met with the cardiologist Dr. Ackerman at 12:45. We previously have had a couple of calls on the phone with him and he was familiar with Audrey's case but we walked through all of Audrey's details with him once again. He is definitely one of the world leaders in the study of Long QT so wanted to soak up as much of his insight as possible. He was extremely caring and down-to-earth and willing to talk to us as long as we wanted. It was very interesting to hear his full perspective on the path we should take for Audrey's care and compare notes to what we have discussed with our Atlanta doctors also. One of the huge gains from this trip is that we are now a patient of Ackerman's as well and he knows our Atlanta doctors really well. They have collaborated on Audrey's details in the past and following this procedure there will be more of that collaboration to ensure we consider all options.

One thing we hear, in different ways from different doctors, is that her specific type is one of the scariest and most unpredictable. Ackerman explained that on a scale of 1-10, with 10 being worst, her case is about a 9. Our Atlanta doctor said in similar terms that if we were to line up all of his cardiac patients, that Audrey would be in his top one or two most concerning ones. Its always hard for us to believe this is the case with how well her first year went overall but that speaks to the unpredictability of her specific gene mutation. For these reasons we have to be proactive and aggressive in her treatment, which is why we are here now for this procedure.

After going way over on our appointment time, we were late for our next appointment with the surgeon Dr Moir, but I felt a little better about tomorrows surgery. I asked him to tell us some of the success stories for her type, which was definitely good to hear once again. He explained to us the positive that for her rare case, it was one of the earlier Long QT type 3 mutations identified so there has been more knowledge gained around it than some of the others. There are about 10-20 documented cases worldwide of her mutation but enough to gain insight into the details to know some additional medicinal treatment options in the future.

We walked through the plan for tomorrow with both Ackerman and then again with the surgeon and are comfortable with the plan. The one thing that we didnt realize was that they will have to turn off her ICD during the procedure to make certain that there are no shocks caused by the procedure. They will be starting her on a lidocaine drip during the procedure as a precaution though so that should help balance that out. We have to get her there at 6:45 AM to begin anesthesia and the procedure will begin around 8 or 9 AM and will last only around 30-40 minutes total. We'll get to then see her again right around 10:30 or 11 AM. We're really looking forward to just getting this thing done and moving forward. We have researched and discussed and thought and then done it all again so many times that we are now ready to finally get it going and move forward.

I'll post an update on Tuesday about her surgery success.

- Mk