First USS Doctors Appointment

On Tuesday January 12th we headed to the CHaD for our first doctors appointment with the hematologist post diagnosis. We had no idea what to expect. We went loaded with questions and ready to be given all kinds of information.

Levi walking into Clinic.

We were definitely given lots of information. However the biggest piece of information we were given was "this is rare and I am learning right along with you!" Our hematologist had spent the past week and a half learning as much as he could about Upshaw-Schulman Syndrome. He had had conversations with specialists who are doing research, primarily out of Switzerland. He had talked to multiple doctors within the CHaD system as well as specialists in several other states. The amount of time he must have spent learning about, researching, and having discussions about Levi's case is huge! We are so appreciative of our doctor.

We also learned that is indeed USS. Due to Levi's age it was fairly certain that it was USS and not TTP, however our hematologist had Levi's blood sent to Milwaukee after the University of Cincinnati found the ADAMTS13 to be less then 5%. Milwaukee determined that there is no antibody present which is the cause of TTP which makes his diagnosis 100% sure to be USS. Although we had already suspected this due to his age (currently the youngest documented case of TTP is 9) it was nice to have this conclusive test done.

The hematologist looked at Levi and then we did some blood work before heading on our way home. We discussed that our next steps were really up in the air and that we were still in the finding out information phase and that the doctor would continue finding out everything he could to make a plan. Usually we wait at his office for blood work to come back, but Levi was looking great (very little bruising, fairly healthy except for a normal toddler cold, no fever) so he said there was no reason to keep us waiting and that he would call us that night with the CBC results.

We started the 2 hour drive home. Thankfully about 5 minutes into the drive Levi fell asleep. Jay and I began talking about everything. We realized that we were in for a long road ahead of us. And that it was quite frustrating to be leaving there with not much more information then we came with. All of our questions were answered with "I'm not sure" or "I can't answer that right now" or "who knows what the future will hold".

Levi asleep in the car on the way home,

About an hour into the drive we received a phone call from Dr. V (The Hematologist) he was incredibly apologetic saying he never would have let us leave if he thought Levi's CBC was going to come back off at all, but he really needed us to come back to clinic. Levi's platelet count was 6. :( The lowest number we had ever seen.

We turned around at the next exit and headed back to the CHaD. Not knowing what was in store for us.