Day +236, and the 6 month review

Olivia's appointment at Duke today went very well. Olivia's labs look great, everyone was very impressed with her energy level and happy attitude. We're slowly continuing to decrease some of Olivia's meds. Physically Olivia is recovering well from the transplant. Cognitively we've had a rough couple of weeks. We talked a lot about that and where to go from here. Olivia is continuing to learn new things, she's using more words, she singing more songs, she will randomly point out different things (the scale at the dr.'s office had a sticker with the number #2 and Olivia was happy to point that out). The main issue seems to be her ability to concentrate. She is having a very hard time attending to anything. From the transplant team's perspective the difference in Olivia's attention span is the result of 3 things...a side effect of chemotherapy drugs, Sanfilippo syndrome disease progression, and a side effect of one of the steroids that she is on. We're noticing Olivia's attention span issues now because physically she's feeling well enough to get up and run away if she's not interested. We talked about how for Sanfilippo transplant patients the 9-12 month post-transplant period is often a difficult time. Prior to transplant only 4 other kids with Sanfilippo syndrome (type B) had been transplanted at Duke but more than a dozen kids with type A had been transplanted. Those kids tend to be around 2 years old and they are just beginning to show signs of the disease. That's right where we were prior to transplant, we were just beginning to see that something wasn't quite right. During the transplant itself everything gets put on hold, the focus is getting your child through transplant. The medications Olivia was on made her feel so sick. Olivia is feeling better now but time did not stop, the disease continued to progress and now that she's feeling better it's more evident. Now we're entering a time where we just have to wait. The test results show that Olivia is over 98% donor cells, her body is now producing the missing enzyme. Now it will just take time for her body to recover. The soonest they ever begin to see cognition and behavior improve is around the 1 year post transplant mark. The did say that Olivia seems to interact so well with the nurses and doctors at clinic (as well as her brother) that they are very curious to see how she does with people her own age. She loves to try to talk to the other patients and still often mentions Rachel. This weekend we'll have a chance to see for ourselves how Olivia interacts with friends. We were given the OK from the doctor to meet up with our friends from Washington while they are "back east" visiting family. We're meeting in Charleston for the weekend and Olivia has been talking about Wesley. Wesley is a bit older than Olivia but she idolizes him and thinks he's the absolute coolest, you can check out pictures of their previous encounters here . Wesley also has a little sister who is just a few months older than Peter and it will be fun to see the two of them together.

In other transplant news I'm finally making time to write about the 6 month studies....

Last MONTH (am I really that far behind) Olivia went through the same series of tests that she completed prior to transplant. Test results were carefully compared and the following was determined.....

Echo cardiogram--Olivia's heart is not showing any signs of damage, there are not any irregularities. The heart appears to actually working more efficiently but this could be the result of a different technician performing the test or better patient participation.

EEG-Very similar to the pre-transplant EEG however at her most recent EEG they were able to collect data while Olivia slept as well as while she was awake and talking. There is no sign of pre-seizure type neurological activity. The transplant process did not seem to have any effect on Olivia's brain function. At the 12 month post transplant appointment we might hope to see some changes but at this point we're right on track.

Labs--In general Olivia's labs look great. She's greater than 98% donor cells (a transplant recipient won't ever really be 100% donor cells so it can't really get any better than greater than 98%). Her body is producing blood and platelets just as it should. Olivia's blood and urine both show signs of the enzyme so she's producing the enzyme that she was previously lacking. Olivia's immunity is a little on the low side of average for someone 6 months post-transplant. We've been very slow in weaning Olivia off of steroids because of norovirus and the steroids directly influence immunity. We're more aggressively weaning the steroids now so by the 9 month mark we hope to see some improved immunity.

General Stuff--Olivia's liver has shrunk and is now normal size and shape (abnormal liver size is often one of the few physical symptoms that contribute to diagnosis). She is finally starting to regain some muscle mass in her legs and she's much stronger. The CT scan shows that Olivia's sinuses are clearing thanks to the combination of antibiotics and breathing treatments. This provides the biggest noticeable improvement to her quality of life. Last year we had to carry cases of tissue with us everywhere and Olivia's nose was constantly running. It ran literally non-stop so while this is a small change it's one that we appreciate daily. The CT scan did show some increased areas of brain atrophy, this is normal, it is not anything beyond what we had anticipated. We knew the specific chemotherapy drugs that Olivia had would cause this. We hope that this will improve but it will take some time.

Overall things look good. Some of these tests will be repeated at the 9 month post-transplant mark and then all of these tests will be repeated again at 12 months post-transplant. At this point we're beginning to see the light at the end of the tunnel. We still have a long way to go but we're slowely starting to return to life as we knew it pre-transplant. We are able to go out to eat at restrauants during off-peak times, we're just careful to wipe down the booth with our handy lysol wipes. I'm able to run into the grocery store as long as I've made sure to wipe the cart down first. Olivia doesn't have to wear a mask outside, she does have to when we're in busy public places and she always wears one when we go anywhere near an area where we know there are sick people. Our doctors at Duke have spoken with Olivia's doctor at Ft. Bragg and while we've never met the Ft. Bragg doctor I think we'll be making our appointment there soon. There was some discussion for making the transition from every other week to every three weeks but for now we're going to continue on with the every other week schedule, at least until April.