Almost 2 years...

...has passed since my last post here. With the connections and ease of Facebook, its difficult to remember to post here. I apologize to those who still come here looking for updates and information.

Happy MPS Awareness Day! May 15th is designated MPS Awareness Day every year! To celebrate, show support for our kids, and raise awareness of MPS we wear #PURPLE! I hope all of our friends have purple on today!

Gracie is now 8 years post-Stem cell transplant for Hurler's Syndrome. Thankfully her engraftment has remained at 100% donor cells after all of these years. We still think of and pray for the anonymous donor mom and child whose umbilical cord cells have flourished in Gracie's little body, giving her a new chance at life.

During the past few years our focus has shifted from saving and extending her lifespan to ensuring the highest quality of life possible despite the damage the Hurler's has done to her body. At age 4, she had double carpal tunnel release of both wrists and 2 trigger digits released from her right hand. So far, her hands have remained stable although she does seem to get tired if her school assignments require extensive writing. Our current issue centers around her hips. Gracie has malformations of both hip balls and sockets caused by her disease. This issue is very common in children with Hurler's and so this is a problem we have been monitoring for quite some time. Over the last year or so, we have noticed Gracie becoming more stiff after sitting for extended periods and after sitting indian-style especially. We have also seen changes in her gait, indicative of some potentially serious threats to her mobility. During her orthopedic appointment last summer, it was discovered that there has been some movement of the balls of her femurs out from the hip sockets. The malformations of the sockets do not hold the balls in properly which is why this is happening. As you can imagine, this present condition can eventually cause severe and debilitating pain, dislocations, and an inability to walk at all. Obviously, we must address this problem soon.

On May 25th, Gracie will undergo surgery to repair these issues. This surgery will very long, very extensive, and carries some very significant risks. Hurler children are notoriously difficult to intubate and are prone to airway swelling if they have breathing tubes placed. Gracie has had numerous surgeries in her short life, and thankfully (knock on wood), her airway has never seemed to be a problem. However, we still have some concern since she has not been under anesthesia for several years, and has seemed to have had more trouble with her asthma more recently. There is also a small chance that a spinal cord accident can occur during the surgery which could cause loss of sensation and paralysis. This has happened only a handful of times, and most recently to a child only a few weeks ago, so we are definitely feeling some stress about this upcoming procedure.

We are praying that Gracie will be able to have both hips done at the same time, utilizing 2 very skilled surgeons, however, we will not know for sure if this will happen until the surgery has started and the extent of her damage has been assessed. Of course, we would like to do them both together to avoid at least 2 additional surgeries. The first surgery is to repair the hips, and the second would be within a year or 2 to remove the corrective instrumentation. Of we have to do each hip separately, we will go from having 2 surgeries to 4, and Gracie's quality of life over the next few years will be affected tremendously by doubling both surgery and recovery time frames.

As I am writing this, Gracie and her dad are on Day 3 of their long journey from Florida to Minneapolis by car. I expect them to make it to the Twin Cities sometime this afternoon. Starting Monday, Gracie will begin her regular yearly follow-up appointments. She will undergo many tests, MRIs, and see all of her specialist physicians who have monitored her care since before her transplant. She will also begin work-ups for her surgery. While she is very much looking forward to seeing her wonderful doctors again, she is getting very anxious about the upcoming surgery. We have attempted to be very honest with her, explaining that she will be in pain, and unable to walk or do things for herself for a while and that she will have to work very hard to get better, but that this will hopefully make her able to get around much more easily and without pain. She is the toughest and bravest girl in the world and we have every confidence that she will once again give Hurler's Syndrome a good kick square in the teeth before she continues to dance her way through life with smiles and joy like she always does.

We are so thankful for our family and friends, far and near, who continue to support, pray for, and love our sweet Gracie. Please also pray for all of us through what will be yet another challenging adventure, for Gracie's baby brother Hank who misses his sister very much and will likely not see her for at least a month, for Dr. Walker- Gracie's orthopedic surgeon, for Jim (Gracie's dad) who will be with her through the entire difficult process of recovery in a place far from home, and for me- the Mommy who will be home working to maintain the health insurance and heartsick to be away from my baby when she needs me. I will be flying up for the surgery itself and staying a few days afterward, but I am not sure when I will be able to return for another visit. However, I am truly thankful for my job and the wonderful people I work with who are so supportive of me.

We'll try our best to keep the blog updated for you to follow along. Keep an eye on Facebook for updates as well. Much love to you all.