Tuesday, May 24, 2016
I am missing my girl so very much and I am hoping to head to Minneapolis over the weekend. I am still working on rebooking my flight, so please pray that I can do so without too much additional cost.
Monday, May 23, 2016
I wanted to take a moment to update everyone on Gracie's surgery. Due to some ongoing cardiac issues (Gracie has had leaky heart valves since birth), Gracie's cardiologist has requested that her surgery be moved to the large university children's hospital instead of the specialty hospital so that the cardiac team can be standing by in case any unforseen problems should arise.
*Let me just stop here and say that no issues are expected and we anticipate an uneventful procedure. However, Dr. Braunlin wants to err on the side of caution, and naturally, we do too. So it was agreed that the best course of action at this time would be to change the location of the surgery.*
This all was decided around 7pm tonight, and I was notified that the surgery was canceled as I was entering JAX airport for my flight to Minneapolis. As of right now, we are thinking that the surgery will be scheduled for next week... we hope to know for sure by tomorrow. I postponed my flight (thank you for the full refund, Ana from Southwest!), and headed back home to Lake City in tears... disappointed that I would not kiss my baby girl tonight/tomorrow.
Gracie was mildly disappointed that her mom was not coming tonight, but she was also very happy to have a short reprieve from the impeding surgery meaning more time to play, mingle, visit, and explore the Ronald Mc Donald House with new friends, and also Minneapolis with her dad. Shout out to the RMH who have provided Gracie and her dad with 2 tickets to the Minnesota Twins game Wednesday night!!
However, there is a silent and unexpected hero in this story. I waited for the bus to take me back to the parking lot, and when it arrived, it was driven by the same man who had dropped me off about 20 minutes earlier. He gave me a strange look when he saw me again, and as he helped me with my bag he looked at me sympathetically and said, "Did you miss your flight or was it canceled?" I told him that neither had happened, but that my daughter's surgery had just been postponed and I would need to reschedule my flight so that I could be there with her. He looked at me with wide eyes and said, "Thank God you found out BEFORE you boarded that flight!" I realized that he was right, and imagined how I would feel if I learned about this after I had arrived in Minneapolis! Thank God for the caring and conscientious nature of Gracie's team of physicians and their determination to ensure the best and safest care for Gracie, despite the major inconvenience it surely caused. Thank God for the timing of this news and how the physicians and nurses spent their extra time after-hours to determine this course of action. And thank God that the news reached me (through the persistence of my sweet husband) before I checked my suitcase and boarded the plane. And thank God for Brian, JIA bus driver, for reminding me about the silver lining of my dark cloud this evening. I needed help looking for the bright side, and he did just that.
So friends, stay tuned for an update on when the surgery will be! Also thank you to all who are participating and sharing the Get Well Cards for Gracie page. She and her dad bought a map of the world tonight along with some push pins. She has no idea why yet... :)
I'll post an update soon... please continue to keep us in your prayers.
Sunday, May 15, 2016
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.
Thursday, July 17, 2014
.... and I'm going to try to give you a quick update. Tuesday was a good day for us. We only had 1 appointment- orthopaedics with Dr. Walker at 2pm- so we spent the morning hours at the Minnesota Childrens Museum. This is one of our favorite activities when we visit Minnesota! This year topped them all since their special exhibit was of Thomas the Tank Engine and that is one of Hank's favorite shows! Needless to say, we all had so much fun that we lost track of time and were almost late for our appointment!!!
Of course, the Ortho appointment always makes me nervous. We have been talking to Dr. Walker for several years about Gracie's hips and eventual surgery to reshape the malformation she has. Gracie's xrays showed no change from 2 years ago, so her hips are presently stable and there is no pressing need to do the surgery right now. He did recommend that we do it by the time she is 9 or 10, so we are looking at trying for sometime in 2016. I'm not looking forward to it at all, but it is an impending reality that we need to prepare for.
So, while I wouldn't say that trying to narrow down a surgery date will count as "good news", the fact that her hips have remained stable the past 2 years is definitely great!
Stay tuned for pics from the museum!
Wednesday, July 16, 2014
Hi Gracie fans! I'm taking a quick minute of waiting room time to update you all on our appointments so far this week.
Monday started with Endocrine- where we met with our sweet Dr. Polgreen for the last time. She is moving to LA to continue her research on MPS there. We will really miss her. :( As for the appointment, we were pleasantly surprised to see that Gracie had a little growth spurt! Yay! This means that her thyroid medication has been working. We still are looking into the option of the growth hormone therapy, however we discovered that since Gracie presently makes a normal amount of growth hormone, the treatment will not be approved by the insurance company. Getting around that issue will be a challenge, but its possible. We will have to look into that with our endocrinologist at home when the time comes.
We visited the BMT clinic next for labs and a general checkup. While there, we ran into our favorite geneticist, Dr. Whitley and a group of student doctors who were learning about Hurler's Syndrome and transplant. Gracie took a few moments out of her busy schedule to show them how awesome she is before getting her EKG and then meeting with Dr. Wes, a new BMT doctor on staff. He's very knowledgeable and funny, and we liked him right away. He was very impressed with Gracie the rockstar, and after she gave him an impromptu performance of 'Let it Go' she received a round of applause and a hi-5 and we were on our way. We were sad that we werent going to see Dr. Orchard this week, as he was the attending physician on the BMT unit this week, but right as we were leaving the clinic, we ran into him in the hallway and gave him a big hug! Being the great doc he is, he gave his awesome patient a quick exam in the hallway. It was too cute. He sent us on our way with a big thumbs up and a promise to see us again next year. We just love our Dr. O!
We finished our day with an appointment with Dr. Bothun, our Ophthalmologist. This appointment was really, really long, as it requires lots of different visual tests and then dilation of the pupils for examination. Gracie's eyesight is presently 20/50 which is pretty standard when it comes to MPS kids. Dr. Bothun expects that it will remain pretty stable at this point and should not require any surgical intervention such as corneal transplant. We will be attempting to wear glasses again, hopefully with more success this time. Gracie and I are looking forward to glasses shopping and getting some super-stylish eyewear.
I'm going to stop here and try to upload some photos in my next post. I'll pick up with yesterday's events later. Stay tuned!!!