The 12 Month Studies--the medical stuff

On Monday Mike and I drove to Durham for our big meeting with Dr. Paul and Andre. We left both Olivia and Peter at home with a babysitter so that we would be able to really listen and understand the conversation. It was such a luxury to be able to participate in the meeting together and actually hear what they were saying!

So here's the breakdown of where things stand.....

• Labs--Olivia's blood work looks good. Her white and red blood cells are doing just what they should. Her labs indicate that her kidneys and liver are functioning well. Her thyroid is also working well. There is always concern that these organs will be damaged during chemotherapy and not recover. We are still waiting on the final result of her immune function tests but preliminary results look good. We are also still waiting on the urinalysis results, we are hoping that it will show that Olivia's body is producing the missing enzyme on her own. Olivia continues to remain 100% donor cells!
• EEG--Normal, Normal brain activity while Olivia is awake, normal activity when falling asleep and when asleep.
• Pulmonary Function Tests--Olivia did great, her lungs are functioning at 101% so she's doing better than she was prior to transplant.
• Vision--Mike and I have not been consistent about making Olivia wear her glasses. Her prescription hasn't changed. We've had a lot of other stuff to worry about over the last year so we're not going to beat ourselves up over not enforcing the glasses but we're really going to work on having Olivia consistently wear her glasses.
• Audiology--Olivia wasn't able to participate consistently enough to get an accurate score. She responded consistently to speech sounds but not other sounds. They recommended that she undergo a sedated hearing test so we're working to schedule that in conjunction with another procedure.
• Echocardiogram--Olivia's heart is functioning well. The latest echocardiogram (she's had 5 in the last year) showed that one of the valves in her heart isn't closing completely (tricuspid valve insufficiency). Right now it's isn't something to necessarily be concerned about but it's good to be aware of and we will continue to monitor it.
• MRI--Compared with the MRI Olivia had done in June of 2009 Olivia has significantly worsened brain atrophy. This was absolutely the hardest news for Mike and I to hear but at the same time Mike pointed out that it was comforting to him that we now have scientific proof of why Olivia has changed so much over the last several months. This is do to the combination of the chemotherapy drugs and the progression of Sanfilippo syndrome. It is not likely that it will improve with time and it would be ideal if the atrophy did not continue.
• Developmental Evaluation--The staff was very impressed with Olivia's compliance and cooperation during the medical part of the exam. Olivia almost seems to enjoy having her blood pressure taken and she doesn't mind any part of the medical exam. Practice makes perfect and she's had tons of practice! Overall they determined that cognitively Olivia has regressed, test results show she's now around the 12 month mark. The doctor followed that remark with the cheerful news that 12 month old kids tend to be very happy. It stings but she was correct, it could be worse, she could be an angry teenager who screamed at us day and night, or she could be a 6 week old baby with colic who screamed constantly.

Now to end with some positive news. Olivia is doing very well medically so we are scheduling the surgery to remove her port for the end of August. Once her port is removed we'll be able to stop the last antibiotic medication and then Olivia will be free of all of her transplant medications. Olivia will be able to go to school in the fall, she's officially no longer immune suppressed! Olivia will receive the first of her vaccinations in August (kids that have bone marrow or stem cell transplants have to be re-immunized). Technically you are at risk for the dreaded Graft vs. Host for 3 years but the fact that we were able to wean Olivia from the anti-rejection medications bodes well for us. If we make it to November without any further complications then we can relax a bit more about worrying about GVH.

Overall Mike and I are happy with our decision to have gone through transplant. We hope that the transplant will keep Olivia healthy enough to be able to benefit from future treatment options. Of course right now there is not a cure but we're hopeful. We hope we've bought Olivia some time.