Please forgive the lack of updates...

but the past week or so has been pretty crazy. We spent last weekend in St. Augustine for the Annual Kelly Family Reunion and we had a wonderful time. Gracie was an angel and she very much enjoyed all of the excitement, not to mention all of the fawning over her and how wonderfully she's doing.

Unfortunately, Mommy managed to pick up a nice cold while we were there and so after returning home we found that the inevitable happened... Gracie caught it. She started running a fever on Monday afternoon and it was off to the ER for blood cultures and a dose of antibiotic, just to be on the safe side. The next morning was our scheduled appointment at Nemours for a checkup as we continue Gracie's taper of anti-rejection med. While there, she received her follow up dose of antibiotic which is standard protocol for any fevers. We also discussed the appearance of a small rash on the back of her hand, and the spreading of a larger rash on her neck and behind her ears. While Dr. Joyce suspected eczema or just plain sensitive skin, we could not overlook a more ominous possibility- Graft-vs.-Host disease. The anti-rejection med that we are weaning off of is also the primary means of defense against GVHD, so with the suspicious timing of these rashes, we had to acknowledge the possibility of GVHD being the culprit. Therefore, the taper has been temporarily halted while we try and figure out why she has the rash. We were sent home with instructions to apply a topical ointment to the rashy areas for the next week or so and return the 15th for a follow-up. If the ointment takes care of the rash, then it is probably not GVHD. If not, Gracie will be scheduled for a skin biopsy so that they can see if it is really GVHD that we are up against.

Thankfully, her skin immediately began responding to the ointment and the rash is going away- and so are our fears of a future with GVHD. Also, her blood cultures so far have shown no growth of any funky infections. It seems to have been nothing but a viral cold and some random itchy rashy skin thing. With that good news, we have been given the green light to consult with surgeons at our upcoming appointment about getting Gracie's central line removed. We are so excited to be approaching this big step. No central line means a big step in returning to normal life. Gracie will no longer have something dangling from her chest as she has had for the past year. She will not be forced to sit still while its flushed on a daily basis. She will be able to be bathed more regularly without being subjected to a full dressing change immediately afterward where she must lie perfectly still with her hands held down so that she does not pull the line or contaminate the exposed entry point. She can be tossed in the air like most babies are without being afraid that the line might get pulled or otherwise compromised in some way. It will be yet another defining moment in Gracie's recovery.

We received word from Minnesota a few days ago that Gracie's enzyme results are back. Now this news is of the upmost importance because it is the reason we had the transplant in the first place. Remember, Hurler's syndrome is due to a deficiency of the enzyme alpha-L iduronidase in the body. The report states that she is now producing enzyme at a level of 58.2. This result is NORMAL. We are so pleased and proud that Gracie has officially trumped this dreaded disease. We are in no way out of the woods yet, but we are definitely moving in the right direction. We still must be very careful to prevent illness and infection as Gracie's immune system continues to recover from transplant, and we also need to continue to watch her engraftment staus to make sure that she remains fully engrafted with her donor's cells. It will be another good year or 2 before we can breathe easily. And while Gracie has beaten the odds thus far, Hurler's is an ugly disease that has lingering effects. It is not known what obstacles Gracie might have to overcome in the future and what limitations Hurler's Syndrome may have left her with, but we know our little champ will be ready to face them all head on.

With much love and joy.