saving grace

The Spoils of War (aka RMH Bingo)

2011-08-18T20:33:00.002-04:00

We won 2 games of Bingo tonight here at the RMH. Anyone who has played Bingo here knows that its a serious sport around these parts. We are so thankful to have scored these awesome prizes!!

Thanks so much to the Ronald McDonald House for all the fun and smiles they give to the families here.

Goodbye casts... Hello splints

2011-08-18T18:48:00.002-04:00

So the casts came off today and Gracie's incision sites look great! Dr. VanHeest was very happy with the way her hands are looking, although Gracie was not happy at all. She wasn't really in pain, but when she saw her hands she completely freaked out! Poor baby. I didn't think about how scary it would look to her, just that she would be happy to get the casts off. We covered them up with bandages pretty quickly to calm her and then exchanged the bandages for a stocking-like thing to wear under the splints. Then a pink lollipop completed the calming process.

We then headed over to Hand Therapy to get our custom splints made. This process was pretty cool. The therapist melted a piece of plastic in warm water and then shaped it to Gracie's hand and arm and let it cool. She added the velcro straps to hold it on, and Voila: Gracie-splints! Pretty cool stuff.

Gracie will have to wear the splints for 4 weeks. Her fingers are free to allow for movement. She will need to work them a lot to achieve the maximum benefit of this surgery so we will be doing lots of therapy- both at home and with a hand therapist. We'll need to do Hand Therapy visits for 6 weeks. After that, we're hoping that Marcie (our OT at school) will be able to work intensively on our fine motor skills in order to catch us up.

That's the plan for now anyway. :)

We're getting ready for Bingo Night at the RMH! Woohoo! We'll let you know if we win!

A new day, a ZOO day

2011-08-16T22:10:00.004-04:00

So today was a much better day for our girl, despite those pesky hand bandages. After a late breakfast, she perked up enough to attempt an outing to the Como Zoo, a small (but totally awesome) zoo and conservatory nearby in St. Paul.

We ended up having a wonderful day. Gracie really enjoyed looking at all of the exotic animals, eating tons of cotton candy, french fries, and rock candy lollipops. :) Then we hopped over to Como Town, a small amusement park in the zoo, and Gracie proceeded to ride several rides (even big ones!) despite her bandaged hands. I was so proud of her for being such a brave girl! :)

We have a follow-up appointment scheduled for Thursday afternoon, but that's the extent of our Dr. stuff for the rest of this trip. We'll find out then how long the bandages must stay on, and what kind of OT we should expect for when we go home.

I guess we really need to be attempting to come up with more day outings to keep our girl occupied until we leave on Sunday. :) Not sure what we'll do, but I know that we're probably in the right place to find out. We'll stop by the RMH program office tomorrow and see whats up. :)

For now, pictures from our zoo day.

R&R at the RMH

2011-08-15T14:45:00.003-04:00

Gracie has been resting relatively comfortably at the RMH for a couple of hours now. She HATES her bandages, despite their snazzy colors, and she is super frustrated at not being able to use her hands. :( I really hate that for her. Her independence is so important to her.

So far, I have not heard any complaints of pain, but I am hoping to get her some Ibuprofen on board soon in order to get the jump on any breakthrough pain that might show up. Other than that, we're to keep the bandages clean and dry until Thursday, when we have our follow-up appointment with Dr. VanHeest.

So, let me backtrack and tell you a little more about our conversation with Dr. VanHeest when she finished with Gracie's procedures. I put a picture below of what the Carpal Tunnel is so you can see what kind of damage the compression of those ligaments can cause. Dr. VanHeest said that when she released the band of ligaments that was causing the constriction of the tendons and nerves, it looked as if there had been a rubber band there in that spot. There was an indentation left by the tightening ligaments in the tendons and learning this made us all (including the Doc) very glad we went ahead and took care of this problem sooner rather than later. Who knows how much longer she would have had function in those nerves, tendons, and muscles????

Her trigger digits were similarly identified. Dr. VanHeest found areas of the tendon sheath where it was obvious that it was catching and causing the triggering to occur. It was just a matter of cutting the sheath away to allow the tendons of the fingers to move freely. Trigger digits can be painful when attempting to straighten them, and as time passes they can become permanently contracted if left untreated. See below for an illustration of what I mean.

So that's what happened this morning. Gracie has really been a champ through the whole process. We are so proud of her.

Thanks for the prayers and love being sent out for us. We feel it enveloping us at all times.

xoxo.

Surgery time

2011-08-15T10:00:00.002-04:00

We arrived in Minneapolis late Saturday night and got right into the RMH. Thank goodness. We rested and did some shopping yesterday, and then enjoyed some playtime at the RMH.

This morning we were up at 4 am and we checked in for surgery at 5:30. Dr. VanHeest came in for a final check and then Daddy took Gracie back to the OR. The procedure should be pretty quick, not much more than an hour. The official procedure is as follows: She's having carpal tunnel release on both hands and her right index finger and ring finger trigger digits released. The ring finger was a surprise add-on after the dr visit in pre-op.

Gracie was not anxious prior to surgery at all which was great! She played and chatted and blew bubbles through the whole pre-op and then requested pink and purple bandages- one on each arm. :) We'll post pics if she gets her way.

Will update more later. Stay tuned.

Back to MN we go....

2011-08-11T08:45:00.003-04:00

So we're hopping a plane on Saturday night and heading back to Minnesota.

I've been neglecting the blog (AGAIN) so what you don't know is that one of our last appointments was with the orthopedist to go over Gracie's nerve conduction study in her hands. The results were not so good. Her right wrist is severely constricted, causing her median sensory nerve to be almost asleep despite electrical stimulation. The tendons must be released ASAP to avoid permanent loss of function to that nerve which can lead to muscle wasting, disfigurement, loss of function to the hand itself, etc.

The Doc recommended surgery as soon as possible. If we could have, we would have just stayed in MN for another week and just gotten it over with. However, with Mommy and Daddy both having new jobs, that just was not an option. :(

Gracie's surgery will be Monday morning at 7:30 am. As far as I can tell, she is having Carpal Tunnel release on her right hand, and Trigger digit release on her right index finger. I had hoped that we would be doing both hands at the same time, but according to our paperwork, its just the right one.

Your prayers, as always, are appreciated. :)

Thursday already?!?!?!?

2011-06-16T14:15:00.003-04:00

So we have been here in Minneapolis for something like 5 days already, and it has really flown by! We have been busy, busy, busy! Between the full days of appointments and then hanging with our Hurler friends back at the RMH, we have had little time for blogging. I'm going to try and catch up by giving a condensed summary of the appointments we've had so far, and anything that needs further explanation I'll address in a later post.

Monday-

Our first visit was to the BMT clinic where after a disastrous attempt at drawing Gracie's blood, we saw several of our old friends... most notably our BMT Nurse Coordinator- Teresa, and Dr. Orchard- one of our 2 beloved BMT physicians. They were so excited to see Gracie running around and showing off. They were impressed to see how big she is and how much talking she does these days. :) We spoke for a few minutes about the study that Dr. O still has in the works for doing the enzyme infusions post-transplant, but nothing was decided yet and once the study is up and running, we'll discuss it more.

Next we had several X-rays and a Bone Scan so see how her growth is going. We will get those results later today when we see the Endocrine doc, but this year G "took her pictures" like a pro. :) It was a nice change to not have to hold her down during an X-ray.

Then we hopped over to the Gillette Children's Clinic to see our Ortho doc, Dr. Walker. We were very anxious to see him and check out Gracie's hips and knees for her possible upcoming surgery. His report was better than expected. Her hips still need to be addressed, but it is not an urgent issue. He'd like to do the surgery sometime within the next 2 years, so we have time to make plans. He's actually seeing some improvement in her knees (YAY), so we're not really looking at doing anything to them unless we do the hips sooner rather than later. Overall, that's good news for us.

Tuesday-

Tuesday morning we went for an echocardiogram of G's heart. Again, she was well-behaved and relaxed she watched Strawberry Shortcake and listened to her heartbeat and watched the colors on the echo screen. All in all the test went well.

We left the echo appointment and went up to the surgery department to be sedated for our EMG (Nerve Conduction test- testing for Carpal Tunnel Syndrome) and the MRI of her brain and spinal cord. Unfortunately, there was a delay in the OR and we were stuck in pre-op for several hours. Gracie did not go to the OR until almost 1 pm, and she was so hungry and thirsty by that time that she was miserable. The procedures themselves took some time as well and we didn't see her in the Recovery room until almost 5 pm. Mommy was a little more anxious than usual after all that time, but Gracie recovered like a champ so we hit the Noodle place for dinner on the way back to the RMH.

Wednesday-

1st thing we had to do was an EKG prior to our Cardiology appointment. Unfortunately, Gracie did not have a good experience on Tuesday night with removing the electrodes for the heart monitor she had to have during surgery, so she was not happy with the idea of more stuff being stuck to her body. She loudly protested the EKG, but with the help of some Strawberry Shortcake, and a vivid light-up magic wand, we were able to get a decent reading.

Dr. Braunlin (the Cardiology doc) came in and let us know that we had some good and not so good changes on our Echo. However, she was quick to say that she had not been able to pull the Echo up on the computer herself and study it, so she didn't want us to worry yet. The good changes are that Gracie's heart walls have thinned more and look very good, and her Ejection Fraction (more on this later) remains unchanged at 36. Her EKG looked good as well, However, there is possibly an increase in the amount of leakage from her aortic valve, which is unexpected this soon after transplant. Her aorta itself seems to be larger than it should be as well. Dr. Braunlin assured us that she will check into it and make sure that we know what we're looking at, but that either way these problems do not need to be dealt with at this time. We need to plan for another Echo in 6 months at home at Nemours. In the meantime, Dr. Braunlin will look at our Echo herself and let us know.

We went to see Dr. Bothun in the Ophthalmology clinic next, and he was really happy to see how well our girl is doing. Her functional vision is very good, however she is still far-sighted (which we learned is not the the opposite of nearsightedness- we'll explain more about that later as well.) and since the issue does not appear to be correcting itself, we may want to consider getting her some glasses to help. It's not a requirement, but it could potentially help her eyes to correct the issue themselves. We'll come back to this later.

Thursday-

This morning we had our Audiology exam and visited with the ENT doctor, Dr. Rimmel. As it turned out, Gracie had a LOT of wax in her ears, so before we could take the hearing test, we had to get a good ear cleaning. Gracie was NOT happy with this, but we got it done, and the doc was able to get some big, yucky chunks of wax out of Gracie's ears. Imbedded in the wax that came out of G's left ear, we found her long lost left PE tube... turns out that it has been out of her eardrum for a while, making Gracie tubeless. So we did the Audiology test and G got a normal score. The doc came in and looked and pronounced that we no longer need our ear tubes. YAY! No more ear plugs in the bath or pool. Good news from ENT!

We're leaving now to see the Endocrine doctor. We'll report back as soon as we can.

And the worst blogger award goes to......

2011-06-08T16:40:00.005-04:00

GRACIE'S MOM!!! (boo, hiss, *throw rotten tomatoes*)

Yes, here I am crawling back with my tail between my legs to dust off our poor little neglected blog. I have no good excuse, just straight up haven't gotten around to blogging. I hope our loyal readers (if we have any left!) will forgive me.

On May 8, 2011, we reached our 3 year transplant anniversary and Gracie is still doing wonderfully!!! Praise God!

She completed her 1st year of Pre-K and had a complete blast! She loves her teachers and friends, and we are thankful for the opportunity to place her in such a wonderful program. She will go back to Pre-K again next year and then when she is 5, we will enter kindergarten. Gracie has other plans, of course, and she plans to go straight to Kindergarten tomorrow. :) Needless to say, everyone at Westside Elementary is in love with her....not that they can help it, of course.

We are getting ready to head up to Minnesota for our 3-year post-transplant checkup. As always, we have a full week of appointments and tests scheduled with all of our wonderful physicians and we are looking forward to seeing everyone. This trip we will sadly miss our beloved Dr. Tolar (Mommy's forever crush), but we will be seeing Dr. Orchard in his place, and we aren't complaining about that. :) We dig Dr. O too.

We are definitely interested in hearing what all of our docs have to say, but we are especially interested in seeing how Gracie's growth is going to see if we will eventually need to give her growth hormone shots. Also, her hips and knees have been a little bit of a concern and we had originally looked at having surgery this summer, but the timing was just not good. I'm hoping to get a better idea of how necessary this surgery is in the near future, and make some plans. Last year, her hands showed the beginnings of Carpal Tunnel Syndrome, a common occurrence for Hurler kids, and so we're interested in seeing if its progressed any further, and what we need to do about it. Lastly, we want to talk with the pulmonologist regarding asthmatic-like issues Gracie seems to have occasionally when she gets a bad cold.

It will be a busy trip, and we are so excited that we will be able to spend a little bit of time with our Hurler friends Rylie and Wyatt who will be having their checkups too. :) And of course, we can't wait to see Jerry and our friends at the Ronald McDonald House who have always been so wonderful to us. We are also looking forward to seeing the brand new Amplatz Children's Hospital for the first time. We hear that it is totally amazing!!! Our friend Rylie's Daddy and I sat on a parent panel when they were planning the BMT unit and we got to weigh in on ideas and share experiences. Can't wait to see how it all turned out. :)

So that's all for now. I will try to be a better blogger while we are there and keep everyone updated on all of the appointments. We'll see how it goes.

The last picture is kind of old (from back in October), but Gracie insisted that we put this picture of her friend Pat on this blog post. :)

a (late, late, late) checkup update!

2010-03-25T18:23:00.005-04:00

Sorry everyone. I have been meaning to hop on here and give updates regarding Gracie's week of appointments in MN, but we seriously hit the ground running when we got back home. My school schedule is demanding and busy, and it seriously leaves me so tired at the end of the day, I can hardly think about my homework, much less blogging. :( Sorry for the delay, but here's a summary of Gracie's 2-year post-transplant checkup.

Cardiology: Dr. Braunlin was again very happy with the results of Gracie's ECHO and EKG. Apparently, Gracie's heart valve issues are still stable, and again may have shown a slight degree of improvement from last year. Her heart walls, which were thickened last year from the extended use of the anti-rejection (steroid) medication, have thinned back into the normal range since she is no longer taking the med. Dr. Braunlin kicked us out of her office again and like last year, she pronounced us too healthy to be there. Love it!

BMT Clinic: This appointment began with a not very fun blood draw requiring both Jimmy and me to hold Gracie still while they drew lots of blood for many different tests and studies. But soon after, we were waiting anxiously to see our very good friends, Dr. Tolar and Nurse coordinator, Teresa. When they entered the room and saw our Gracie, they could not stop talking about how wonderful she looked! Dr. Tolar did a short exam, pronounced that Gracie is his Hurler Poster Child, and proceeded to cheer Gracie on as she shared lots of her tricks and even showed him how to edit her chart on the clinic computer. :) Whoops. Anyway, it was a joyful appointment, all smiles and hugs and claps, and Miss Gracie *loved* all of the attention she received.

Genetics: A visit with Dr. Whitley was next and ended up being much more exciting than expected. While we spoke about the latest treatments coming available, and especially the current studies ongoing which are attempting to curtail many of the post-transplant issues that our Hurler kids face. We shared thoughts, and discovered that we have all be pondering the same question... could an overabundance of enzyme, especially in areas that are trouble areas for Hurler kids due to decreased vascularity of those specific tissues, help delay or even avoid many of the issues these kids tend to face? Well, to make a very long story short, we were considering a kind-of experiment - a one-time ERT infusion followed by multiple urine specimens in order to gauge decreased GAG output and duration of effects. As it turned out, we did not end up participating in this little experiment- mostly because it would disqualify us from enrolling in a study that Dr. Orchard is hoping to open by summertime. The basic idea is to take post-transplant Hurler kids, give them weekly ERT again and follow them closely to monitor whether they have the need for as many corrective options or if they even make improvements. Several of Gracie's specialists are on board with this, and helping with this particular study and they seem really excited about the prospects. We are very interested in this particular study and we're hoping that we will be able to learn more about it soon.

More later, I promise.

I know I owe an update...

2010-03-11T23:48:00.003-05:00

but we are totally wiped out. Apparently those who are in charge of our schedule here have forgotten that we have a little Peanut who requires a nap in the early afternoon- meaning that we have missed it every day this week. This has resulted in a grumpy girl for Mommy and Daddy to deal with which in turn has made us grumpy and tired too.

I promise to try and update about our appointments soon. Tomorrow is a light day, but we hope to take Gracie to the mall in the afternoon to ride some rides at the Nickelodeon theme park. We want to be sure we associate some fun with visiting MN, not just doctor visits.

We've had mostly good news, a little bit of tough news, and a few interesting developments to share with you all. Until then, please keep us in your thoughts.

xoxox.

Check-up schedule

2010-03-07T22:27:00.003-05:00

Just to keep everyone informed of what we'll be up to over the next few days, I'm going to post our schedule of appointments for the week.

Tomorrow (Monday 3/8) starts with an Echocardiogram, followed by a visit with our cardiologist, Dr. Braunlin. We'll go from there to the BMT Clinic for labs and a physical and then an appointment with our beloved transplant doctor, Dr. Tolar. We'll finish the day with a visit with our geneticist, Dr. Whitley.

Tuesday 3/9 - We will check in at the Outpatient Surgery floor where Gracie will have an MRI under anesthesia and also an EMG- a nerve conduction test for her hands. She'll see her opthamologist, Dr. Bothun, in the afternoon after the procedures. I'm sure that will be a fun appointment- yikes.

Wednesday 3/10 - We'll visit Dr. Laguna for Pulmonary and then have an chest Xray and an EKG. In the afternoon we'll have an Audiology exam and a visit with the ENT doc.

Thursday 3/11 - In the morning we'll be driving to Minnetonka (wherever that is) to the Gillette Children's Specialty Clinic to see Dr. Walker, the Ortho doc who concentrates on Gracie's hips, legs, and feet. We'll make our way back here to Minneapolis and finish our long day with several more appointments - Endocrine with Dr. Polgreen (Love her!), Neurology with Dr. Rothman, and we'll start the Neuropsychology testing. We will go back to Neuropsychology early Friday morning to complete their assessments, but it won't be the same without our Dr. Kendra. We will meet a new doc, Dr. Ziegler this time.

Somewhere in between all these visits, we plan to stop by Unit 5-D to see our sweet nurses, and also over to the rehab center to visit our therapists. Should be fun times. :) I'll do my best to keep everyone updated on the outcomes of all of our appointments as we go through them.

Keep us in your thoughts so that we hear nothing but good news this week.

Much love.

Leaving on a jet plane...

2010-03-05T08:58:00.001-05:00

We fly out today at 11:45am and arrive in Minneapolis around 4:30 pm. Please pray for safe travels and a happy little traveler. :)

Xoxo.

Day +664

2010-03-03T20:50:00.001-05:00

Wow... look at that! We are at day +664 post-transplant. Sometimes I can hardly believe it has been so long since we were in our little room on Unit 5-D smelling the creamed corn smell of Gracie's new stem cells!

Gracie continues to do wonderfully of course and Mommy has been very busy with school which is definitely challenging and exciting. But it really has been a commitment and has taken me away from Gracie a lot. That part has been a bit harder than I expected. We're all being strong and working it out though and we know it will all be worth once December rolls around. :)

Mommy has Spring Break this coming week, but instead of resting relaxing in a warm, tropical locale, we are headed back to freezing Minneapolis for Gracie's big 2-year post-transplant anniversary! Despite the weather, we're really excited to be visiting with our RMH friends and of course our beloved docs and nurses who took such good care of our girl. We're sad to say that we will be missing our best transplant friend, Rylie, this trip, but we hope to see her next year, and of course we'll give knuckles to all the docs and nurses for her. :)

While we know that Gracie will continue to impress everyone up north like she always does, we have been wondering a bit about her hands lately- her fingers don't straighten completely unless we push them straight. Kids with Hurler's Syndrome tend to have lots of Orthopedic issues, and hands and fingers are at the top of the list, so while the issue didn't come as a surprise to us, we do want to have it looked at to see if we can prevent any further issues. Gracie will be having special testing done on her hands to see if we need to plan for any interventions to keep the problem from progressing. Gracie will also be having appointments with all of her regular specialists as well and we're looking forward to seeing how they think she's doing and if we're looking at any other problems right now. We know that she will probably need some surgical intervention on her knees and hips at some point, but we're hoping those will be down the road a bit.
So while we're really excited to show her off this year, we do have worries in the backs of our minds that some of her issues may have progressed. We're hoping for the best though, and we know that our folks in MN will guide us as we work through it all.
Please keep our friend Wyatt in your prayers for us, he is in MN right now and he is recovering from back surgery. We hope to be able to hang out with him, but he might be heading home by the time we get there. Either way, we pray for quick healing for Wyatt and a safe journey back home.
Please pray for our travels as well... we're flying out on Friday morning.

Much Love.

A new year... part 1

2010-01-08T10:30:00.004-05:00

So as 2010 has arrived and we've kept plenty busy. Gracie has been doing so well and has enjoyed an exciting holiday season. We attended 2009's MPS Family Conference last month for the first time and we really enjoyed it and learned quite a bit. The conference was held at Disney World this year so it was a pretty easy trip for us. We participated in a focus group for Genzyme (the company which provides the synthetic enzyme which Gracie received while being prepped for transplant), and then spent the next few days attending sessions specific to concerns that affect us directly- post-transplant issues, what to expect in adolescence for MPS kids, emerging research and treatments, and reproductive issues regarding testing and other options available for when we choose to have another baby- all very informative sessions. Gracie got to hang out in the provided childcare room while we attended these sessions and she really loved it. She really loves being around other kids, and we were really pleased that she took to the caregivers so easily. Never once did she cry when we left and I think the leaving was actually harder on us than her. This part of the experience has given rise to the idea of possibly putting Gracie in a childcare/school program soon. We're still thinking about it, and of course we need to be sure that all of her immunizations are up to date before she goes. Hopefully we can get that yucky business worked out soon and then explore some good options for her.

Also at the conference we were able to meet and spend time with other MPS families, which in my opinion was the best part. We got to hang with old friends like the Blancheri family and little Wyatt, and Danny Latham and his family as well. We met new friends too- I enjoyed spending time with Bella's mom, Elizabeth, Erica- an adult with MPS 1, and Brinley and L.B.'s moms- Mandy and Michelle, who after 5 minutes time felt like sisters to me. While the reasons we have been brought together are difficult, spending time with these people who know exactly what we're going through is such a comfortable and easy thing.

We spent the last 2 days at the Magic Kingdom and Animal Kingdom where Gracie succeeded in shutting both of the parks down. She rode a few rides and saw some wonderful parades and spent the day in the Animal Kingdom with our beloved "Aunt" Bee (aka Godmother Laurie) and just all-in-all really enjoyed ourselves. While some of the sessions gave us a hardcore reality check regarding obstacles to come, we still are so thankful for the successful transplant which saved our precious girl's life and we will face whatever comes with the same hope and determination, and with the unwavering love and support of so many loved ones.

Stay tuned for part 2- lots more to share!

hi.

2009-12-15T23:52:00.002-05:00

Again, a long break in posting and with no reasonable explanation except that Gracie is doing great which leaves us with little to blog about! We've been very busy trying to get moved in to our new beautiful home and prepare for the holidays.

So far, we're living here, but we've been so busy that we haven't finished moving yet- and don't even get me started on Christmas. I am so behind! Hopefully we can get caught up soon and really enjoy this time with our families.

In more fun news, we leave tomorrow to head to Disney World for our very first MPS Conference. We're looking forward to meeting so many new friends and saying hi to a few we already know. And of course, this will be Gracie's first visit to Disney, so we're really looking forward to that part too. :) She has no idea whats coming and it will be interesting to see how she does with so much going on around her.

When we get home, Gracie's cousins will be here and the festivities will REALLY commence. I know we will all have a blast, as always. Just keep your fingers crossed that the GrinchPox stays away this year.

Much Love.

Sorry for the delay in updating...

2009-10-21T23:30:00.002-04:00

...but Gracie's surgery went well. She ended up needing both the right and left sides done (there was a hernia in the other side, it just wasn't bulging out yet) so we have 2 little boo boos that we're watching heal. Other than that, Gracie is her normal self again, running around like nothing ever happened. Thanks so much for the prayers.

Much love.

Surgery.

2009-10-17T20:53:00.005-04:00

Gracie's hernia surgery is scheduled for 9:15 AM on Monday morning. If you don't mind, say a little prayer for us. I hate putting her through procedures, but it has to be done. :(

Much Love.
xoxoxo

P.S.
We have a new Hurler friend in CO that we're following. Her name is Torie and she is a cutie. She's preparing for transplant now and doing ERT, but right now she's in the hospital due to some nasty infections in her central line. Please keep her in your prayers and visit her page and tell her hi. :)

http://toriebrady.blogspot.com/

No news is good news.

2009-10-08T13:31:00.002-04:00

Well, we are still bustling along in normal life with Gracie and things are going well. Gracie has been picking up more and more words everyday, so pretty soon we expect to have a chatterbox on our hands. She is a busy little bee and is enjoying life and all it presents her. Mommy and Daddy have been working hard to get the new house all ready to move into soon, and Gracie helped by picking out her new room. Of course, its not the one that Mommy and Daddy had planned for her to have, but who could possibly deny her? :) She loves the new house, and its in a great neighborhood with lots of little friends to meet and play with.

We're getting closer and closer to Gracie's hernia surgery which is scheduled for Oct. 19th. Poor kid. We're told that she'll have a very short recovery time- good thing because Gracie is a bundle of energy these days.

Healthwise, other than the hernia we're doing great. Gracie is off all medicines and that is making everybody happy. But that means the time has come now to get Gracie back on her immunization schedule though, and Mommy is REALLY not looking forward to that. :( We'll have to be sure to have lots of ice cream and stuff on hand for afterwards, I guess. Just thinking about it is making Mommy anxious. Poor baby.

Pics to come soon. Thanks for checking in on us. Xoxo.

Has it really been so long?

2009-09-15T12:27:00.004-04:00

Its hard to believe that over a month has passed since my last update! Well, Miss Gracie continues to be her awesome little self, and true to most 2 year olds, she is running her Mommy ragged! She is signing more signs than I can even think to count, and she is doing some good talking too- although we are really working on that part. She squeals (more like ear-splitting screams) frequently these days and I am considering investing in some earplugs. :)

Healthwise, she is still doing great. We are on a 3 month schedule for her follow up visits and she has had no problems as far as post-transplant status is concerned. Her hernia is still with us, although we have finally gotten scheduled for our repair surgery on October 19th, yet another month away. As it turns out, we will not be removing her ear tubes as we had hoped to do while she was already in surgery. Hurler kids tend to have some slight malformation of their Eustachian tubes and are therefore more prone to infection. Since the eustachian tubes do not finish growing until at least the age of 7, we have quite a ways to go until we find out what will ultimately be the result. Our ENT says that if the ear tubes should fall out on their own and we see no signs of infection, we can see how well she does without them, but he recommends not removing them right now. So, there you go. Gracie is also scheduled to kick her last post-transplant medicine by the end of the month and we are so excited! It just goes to show how far we have come from last year. We have so much to be thankful for.

In other news, we are hoping to close on our new house in a few days! Another exciting adventure!!! We are so thankful that our friends and family gave us so much support during Gracie's transplant which has enabled us to take this wonderful new step in our lives together. Without your help, we would still be buried under a mountain of medical bills. God bless you all and thank you from the bottom of our hearts.

Much Love.

All has been quiet on the Gracie front...

2009-08-06T23:35:00.003-04:00

for the summer... until now. After a relatively easy summer, with significant reductions in medications and doctor visits, Gracie has thrown us for a loop, yet again. It seems that she has developed an inguinal hernia. This type of hernia is located in the groin area, and is causing much more concern than her ever-present umbilical hernia. An inguinal hernia can cause a portion of the intestine to become entrapped, which can of course be very dangerous. Gracie has an appointment with the pediatric surgeons at Nemours as soon as possible, meaning 1 week from now. Of course, I am not too happy with this situation as it will give me one week to stress out and obsess over this thing until I know when it will be taken care of.

We went to our local pediatric clinic yesterday, just to have a set of medically-trained eyes take a look it. It is definitely a hernia, and while we need to quickly get it repaired, it is not a hugely pressing concern. It is reducible, which means I can gently massage it back where it belongs. It is not hard, painful or red, and it reduces itself on most occasions. So far, it hasn't managed to slow Gracie down any either. Her bowel movements are still pretty normal, and she is still all over the place, as always.

I'll keep you all updated on how things go, however, I ask you all to keep Gracie in your prayers. Pray that this hernia remains as it is, and does not become entrapped or dangerous.

And in return, I will finally make good on a long overdue promise... PICTURES!

Gracie's 2nd Birthday!

Naughty Mommy...

2009-07-07T02:24:00.002-04:00

blogging without pictures yet again. Sorry all. I blame it on readily available technology. We now own 2 digital camera cards capable of holding thousands of pictures at a time. Therefore, the need to dump them onto my computer to make more room has decreased greatly. That and I'm just plain lazy. I do promise to post pics soon, you will not believe all of the wonderful things our awesome girl is up to these days.

So on that note, I want to announce that Gracie is officially off her anti-rejection med!!! This latest achievement came about last Tuesday, and honestly, I must say that I have really been enjoying not having to worry about meds every morning and every night. As of right now, Gracie has one medication (general antibiotic) that she takes on Mondays and Tuesdays only. It is a wonderful feeling to know that our daughter's life is inching closer and closer to what could be called "normal". :) We joined a weekly 2-year old program at the local library last week as well and Gracie had a total blast! She adores discovering new places and playing with other kids. Its such an amazing feeling to watch her with these children, playing and smiling and just being a kid.

However, we have recently discovered that Gracie's true love is the water. She cannot get enough of it! From the cold Ichetucknee Springs, to a friend's relaxing pool, and even the rain puddles in Noni and Munts' driveway- no amount is to small for Gracie to enjoy. She takes after her Mommy in this regard- when I was little I was known in our neighborhood as Sarah-fish. Well, our little Gracie-fish is giving me a run for my money these days. The squeals of joy and splashing hands and kicking feet make my heart smile in the biggest way. I cannot even adequately explain how the sheer joy bubbles up inside me to see her so happy and carefree. Her ear tubes presented a slight problem at first, but Gracie has learned to tolerate her ear plugs pretty well, since she realizes that wearing them means she gets to be in the water. :) Smart little booger. We're looking into seeing if we can get those pesky tubes removed in September, but thats still in negotiation at this point. We'll need to chat with quite a few docs before we're comfortable doing that, but we are holding out hope that maybe it will happen.

Gracie has picked up quite a few signs and a couple more words recently as well and she is walking full time. She has mastered standing up without assistance, and there is truly no stopping her when she gets going. We are truly so proud of her. She is still having Early Interevention services on a weekly basis, and her therapists are so delighted with her progress.

Saturday brings her 2nd birthday and we are so looking forward to giving her the BIG gift. We got Gracie her very own playhouse, pink and purple with a little table, a phone, and working doorbell. I can hardly wait to give it to her!!!

We hope that you are all doing well and we so appreciate your continued prayers and support. Our Gracie is the true definition of a miracle and we know that each of you have played a huge role in getting us this far. So thank you all for loving us and our girl so much.

xoxo

Hi all!

2009-06-15T22:59:00.003-04:00

Well, Gracie continues to do well, although her eczema is still giving us a bit of trouble. Of course, there are worse things to deal with as we well know. She is happy and walking all over the place, which we are so happy to see. And we're hoping to soon be reporting that she is speaking more and more, although it appears that we may have to wait a bit longer for that. She is still saying words she knows and signing enough to get by for now. Hopefully, we'll be on the road to talking more soon.

We are continuing on our slow taper of Gracie's anti-rejection medicine and hope against hope to be completely off of it by the end of this month. This will officially remove the immune suppression from Gracie's health profile and allow for more interaction with other kids. I'm really looking forward to getting her more socialized and we're even beginning to consider possibly putting Gracie in a part-time 2 year old program (if we can find one we like) or attempt to find some sort of Mommy and Me program where she can have some more formal socialization with kids her age. This will probably be a bit of a challenge here in the metropolis of LC, where the kind of programs that we're looking for are not usually found, but I'm hoping something will come up that will work for us.

Thats basically all the info I have for now. We're all doing well and staying busy this summer. I have really been enjoying school so far and looking forward to getting more into my studies. I hope all is well with all of you. I hope to post some pictures soon, stay tuned. :)

A summary of our 1-Year Post-Transplant Check-up!

2009-05-28T13:44:00.003-04:00

Get comfortable, folks.

So sorry for the delay in posting this. It is definitely a long read, but for those who are interested here is a basic summary of all of our appointments from our trip to Minneapolis. I know I gave some info already, but I think I'll just do a general run-down of each area all in one post!

Neuropsychology- This appointment was to do an assessment of Gracie's skills and development. Dr. Bjoraker was pretty pleased with Gracie's progress all in all, but there are a few areas where she is delayed. Her biggest delay is in her Gross Motor skills. This is because Gracie is still crawling to get where she wants to go. Once she starts walking full-time she'll be back on the right track to catching up. And to our delight, she is walking more and more everyday! Her Fine Motor and Expressive Language skills are only slightly behind where they should be and we are happy to hear that. Her Receptive Language skills are right on track for her age so we were overjoyed to get this news.

Genetics- Dr. Whitley did a brief exam of Gracie in order to check her progress post-transplant. He feels that she is doing great and has had no further progression of the disease. He had us collect a few urine samples for GAG (glycosaminoglycan- the stuff that accumulates in Hurler patients) analysis. We have not gotten results back from these test yet. We also discussed briefly the ongoing research in gene therapy to attempt to cure Hurler's and then had a chat regarding testing in possible future pregnancies since Jimmy and I do eventually intend to have more children. We left this appointment feeling very encouraged regarding our options, as difficult as they may be.

Cardiology- Dr. Braunlin was very pleased to see Gracie and had very good news to share about her ECHO. Apparently Gracie's mitral valve leakage has not progrssed at all and in fact may have possibly made a little bit of improvement. The walls of her heart have thickened a bit, however Dr. Braunlin thinks that this is only due to Gracie being on the CSA (steroid mediciation for anti-rejection) for an extended period of time and expects the heart walls to go back to normal thickness once she is off the medication. Dr. Braunlin then kicked us out of her office and pronounced Gracie "too healthy to be here". We loved it!

Pulmonary- Dr. Regelmann spent only a short time with us listening to Gracie's lungs and getting a history of all respiratory events in the past year. This was a short list, and he was pleased to hear that. He told us that Gracie's lungs are in excellent shape for a post-transplant Hurler kid, so he advised us to be sure to get vaccinated come flu season and sent us on our way.

Opthamology- Dr. Bothun went over the preliminary results of Gracie's retinal scan which appears to be normal. Her corneal clouding has stabilized and even shown the possibility of improvement but is still categorized as level 2- mild to moderate. However, Dr. Bothun agrees that her tolerance of light has certainly improved which is more important than the actual level itself. Her vision appears to be very good, still showing a small amount of far-sightedness which is completely normal at this age. All in all a good appoiontment, although Gracie was not pleased to have drops in her eyes and the dreaded speculum. Yikes.

Occupational Therapy- From what I could tell, Gracie did very well on her OT assessment. We have not gotten official results from this testing back yet though. Likely the recommendation will be to continue her Early Intervention therapy sessions and bring in Valerie, the OT on a more regular basis.

Physical Therapy- Well, sadly Gracie did not impress the PT as much as she has everyone else. They were disappointed to learn that Gracie was not walking exclusively yet. However, they did point out that this was her main point of Gross Motor delay and that once she gets on her feet, she will be able to catch up to the normal range quickly. We're happy to report that Gracie is doing more and more walking everyday and we are sure she will be entirely upwardly mobile very soon. We're working on teaching her to stand independently without help which we believe will be a big step in getting her walking full-time. We are still awaiting the official results from this assessment as well.

Audiology- Another great appointment. Gracie did very well on this exam and we were pleased to see that her hearing function is indeed normal. Testing showed that only the highest frequency was not heard, which the audiologists explained was totally normal in kids with ear tubes. So Gracie actually passed her first conscious hearing exam!!! This is an achievement because her last pass was actually on a test under anesthesia. Go Gracie!!!!

Neurology- This was actually a difficult appointment as we adjusted to our new Neurologist who has taken the place of our beloved Dr. Charnas. We could tell that Dr. Rothman is still getting his feet wet when it comes to this Hurler stuff, however he did have a few things to tell us regarding the results of Gracie's MRI of the brain. Apparently Gracie has slight narrowing of the Foramen Magnum which is basically the area of the neck at the base of the brain. Of course, we were not happy to hear this news, but after second discussion about this with our nurse coordinator who was able to help explain things a little more clearly, we are feeling a bit better about it. This narrowing (known as stenosis) is very common in Hurler patients. Gracie's slight degree of narrowing is not enough to cause much concern as far as going through any procedures to try and correct it at this time. However, we were told that we should be slightly more cautious regarding Gracie's neck than normal and avoid any activities that could cause injury in the neck area such as trampoline jumping, gymnastics/tumbling and other things like that. We were told that in most post-transplant kids, this particular condition does stabilize and requires no surgical intervention, however the chance does exist that Gracie may need some surgery in the future to correct this. Lets hope not. All other aspects of the MRI were normal.

Endocrinology- We met Dr. Polgreen for the first time on this visit and we were instantly enamored with her. What a wonderful physician! She is very knowledgeable in her field and also very thorough, while still personable and capable of explaining everything in a manner that makes it all easy to understand. As far as endocrine goes, Gracie looked pretty good. Her thyroid appears to be functioning normally and her vitamin D level was very good. Her growth velocity was slightly low (meaning how much she grew in the past year), however her height is well within the normal range. We discussed the common problem of growth decline in Hurler patients and the continued research of the use of growth hormone to maximize growth potential. Jimmy and I are both in agreement that we will do what we need to in order to allow Gracie to grow as close to normal height as she can possibly achieve and Dr. Polgreen was pleased to hear it. She said that while it is unknown whether or not Gracie will experience growth issues, since it is a common issue with Hurler kids we should basically expect it. If needed, Gracie will be able to take the growth hormone somewhere around 5-6 years old. This will not be a fun process as it will require a daily injection for as long as she is required to be on it, however we will do what we have to for our girl. Other than the discussion, we were told that Gracie looks wonderful and that nothing needs to be done or corrected at this time.

BMT Clinic- We were so excited to meet with Dr. Tolar who is our main BMT doctor and an amazing man. He walked in the door with a huge smile on his face, so excited to see our girl and how well she is doing. He was quite pleased to see how far she has progressed, how healthy she has been, and all-in-all what a happy girl she is. It was such a wonderful appointment. We talked about continuing to wean Gracie from the CSA and then restarting her vaccination schedule again. He also showed us some blood test results which really made our day. All of Gracie's blood counts are normal, and her T-Cells, Killer Cells, and Natural Killer Cells are all in the normal range, despite the continued immune suppression from the CSA medication!!!!
This means one important thing- Gracie has a new, strong, and butt-kicking immune system! Go Gracie!!!! There was a small celebration in our little exam room when we heard this news. :) After hugs all around and confirmation that all of the "rules" regarding Gracie have been lifted (as long as we use common sense, of course), they sent us away with a thumbs-up and told us they'd see us next year.

Orthopedics- We were originally scheduled to see 2 separate doctors regarding orthopedics, one for hands and one for back, knees and hips, we actually ended up only seeing the second. Our Nurse Coordinator informed us that there is no concern with Gracie's hands at this time (many post-transplant Hurler kids develop carpal tunnel syndrome and require surgery to repair it), and so our first ortho appointment was given to our friend, Wyatt. We did see Dr. Walker regarding Gracie's spine, hips, and knees though. After an x-ray we were able to see that Gracie shows no sign of scoliosis and her kyphosis (malformation of the lower spine which is very common in Hurler patients) is minor and presently stable and showing no need for any kind of correction. The x-ray showed that she has slightly knocked-knees which is again very common in Hurler kids, but still not enough of a concern to do anything about. The hips showed that she does suffer from a very common malformation of the hip sockets in that they do not completely curve around the ball of the hip joint. This malformation occurs due to the GAG build up which makes the hips not curve around. Of course with the introduction of the enzyme needed to remove these GAGs to save Gracie's life, it also removes the GAGs that created, in essence, a faux hip socket. Most Hurler kids will require a surgery to correct this at around 5-6 years old, but it is unknown if and when Gracie will need this correction done. Its basically a wait and see kind of thing. Usually when this surgery is done, they also go in and fix the knees too if it is needed. We are preparing ourselves to have to do this in the future, but only time will tell when it is needed. So, while this was not the news we had hoped to hear, it did not come as a shock to us since most Hurler kids deal with this eventually.

Ok, so I think thats everything. We are still waiting for the results of our urine GAG tests and our Enzyme level test. We did hear the there's a chance that the enzyme level will have to be redrawn as something got screwed up in the lab and Gracie's blood sample was accidentally refrigerated. Oh well.

xoxo.

Heads up!

2009-05-20T00:46:00.002-04:00

I'm in the process of writing up a long and detailed summary report of our appointments in MN, but I got an email from our Nurse Coordinator there with some amazing news and I can't wait to share it.

Gracie is still 100% engrafted with her donor's cells!!!!! We could not be happier to hear this. As time passes, the risk of losing engraftment lessens. We're so glad to still be fully engrafted at 1 year post-transplant.

xoxo.

Its been an exhausting...

2009-05-16T00:32:00.002-04:00

but very good week.

We leave tomorrow to head for home and we are frantically packing and cleaning our room here at the Ronald McDonald House. I have lots more to update you all on regarding all of our appointments, but it will have to wait until we are back home and have a bit more time on our hands. Sorry to disappoint.

Please pray for safe journeys home for us and our wonderful friends.

We have a favor to ask...

2009-05-14T15:53:00.003-04:00

our loyal and wonderful friends and fans.

Tomorrow, Friday, May 15th, is National MPS Awareness Day. In honor of the children who fight their daily battles with this disease, and also the ones who have become angels, we ask that you wear purple on this special day. A ribbon, a shirt, a pair of socks... whatever you have to show your support for those affected by MPS and their families.

For more information, please visit www.mpssociety.org.

Thank you and we love you.

2009-05-14T12:30:00.001-04:00

We're waiting to see Dr. Braunlin in Cardiology to learn the results of the Echo. We're hoping for stable or decreased leakage from her mitral valve.

2009-05-14T12:20:00.001-04:00

Well, I learned how to update the blog via text message from my cell phone. The downside is that I can only post messages that are this long! D'oh! :)

Gracie needs a new pair of shoes!!!

2009-05-13T23:21:00.003-04:00

Well, my time for blogging is short these days as I have discovered. :(

It turns out that the only time I have for blogging is late at night when I'm dying for a good night's sleep in a comfy bed. Since we all know that won't happen in a SleepNumber bed no matter what, here I am with a summary post of the last few days.

So I left off with Dr. Whitley on Monday, and after leaving his office we headed downstairs to get a chest x-ray and an EKG. Gracie screamed through the x-ray, but surprisingly, she napped during the EKG which was nice.

That was Monday. Are you tired yet?

Tuesday was expected to be a lazy day for us. Gracie had an MRI and Retina Scan under anesthesia scheduled in the morning and the afternoon free. Unfortunately, that was not how it happened. We arrived at the surgery wing at 10:30 am and we were quickly shuffled back to the pre-op area. We got to our room and looked around the corner to see Rylie and her parents whose surgery had been scheduled for 7:30am. They were still waiting. This was not a good sign.
Long story short, Gracie did not go into surgery until 2:30 and did not come out for almost 3 hours!!!! We finally dragged ourselves back to the RMH around 7:30 pm with an extremely grouchy and hungry baby and 2 worn-out and stressed parents. Yuck.

Today we hit the ground running yet again with an appointment with Opthamology, Pulmonary, and an Echocardiogram. After the Echo, we went to the rehabilitation center to see Occupational Therapy and Physical Therapy specialists and do some developmental testing. She did well in OT, but the PT was a little disappointed that Gracie is not walking everywhere yet. :( We promised her that we've been working very hard on it, and she said that its really her biggest delay right now. She also recommended that Gracie get some new shoes with hard soles to help stabilize her ankles and feet so that she can gain confidence and possibly have better balance. So after leaving the Rehab Center, and a quick trip up to 5D to see our special nurses from transplant, we hopped in the car and headed to Target to get Gracie some hard shoes.

Cute, huh?

And just for fun, here is a quick shot of Wyatt, Rylie and Gracie with their Daddies- Todd, Lyle, and Jim.

The kids have all enjoyed playing together when we have a chance, and its been so nice to have good friends here.

Tomorrow brings another full day of appointments, starting with another NeuroPsych evaluation. Also somewhere in there we have cardiology, orthopedics, and BMT clinic.

So far though, everyone seems to be happy with Gracie's progress- and aside from the PT issues, Gracie is looking great. :) We hope to continue to have great appointments and more good news to share.

I'm beat, I'll try to update more tomorrow. xoxo.

Waiting for our echo appointment...

2009-05-13T14:14:00.002-04:00

So I thought I'd give another quick update. I ended my last post with our Monday schedule, but I didn't get to finish. So after BMT clinic and the awful blood draw, we visited our geneticist, Dr. Whitley, which was again a wonderful visit. We discussed some of Gracie's progress and he seemed very pleased with her so far. We also discussed the process of having more children and then lightly touched on the ongoing research for MPS. All in all, an informative appointment. :)

2009-05-13T00:19:00.008-04:00

Whew! I'd forgotten how packed these check-up weeks are! The last few days have been a whirlwind, but I will try to sum them up the best I can for our loyal readers.
Monday started with an early appointment with Neuropsychology and Dr. Kendra Bjoraker who is one of the most wonderful women I have ever met. She is so kind and caring, and she has a real heart for these MPS kids she works with. The appointment went well, and they noted many of Gracie's obvious improvements. :) We go back to Neuropsych on Thursday for more testing and to hear the opinion on Gracie's progress so far.
Afterward, we visited the BMT clinic and had a nightmare of a blood draw fiasco, but despite it all, Gracie continued smiling and charming everyone we saw. And of course they all remembered our sweet girl. We will visit BMT again on Thursday for an appointment with our beloved Dr. Tolar and we can't wait to show off to him!

We're here!

2009-05-11T08:09:00.003-04:00

Sorry I haven't posted sooner, but we're safe and sound in Minneapolis and having fun. We got to have a short but great visit with Uncle Mack and we met up with Rylie and Wyatt and their parents for dinner and a playdate yesterday evening. We'll try to post pictures soon.

More Later.

xoxo

Day +365

2009-05-08T16:06:00.004-04:00

One year ago today I got my baby back. Unbelievable.

Thank you, God.

Happy Life Day, beautiful baby.

Our special friend Rylie...

2009-05-07T17:00:00.002-04:00

... is celebrating her first Life Day today!!!! Hooray Rylie! We love you so much, special girl! Rylie's parents put together a beautiful slideshow of her journey on their blogspot. Go by and watch it and congratulate our transplant buddy on her 1 year milestone!!!

http://www.ryliehays.blogspot.com

Another reminder...

2009-05-03T12:20:00.002-04:00

of how wonderfully blessed and lucky we are to still have our Gracie with us.

I learned today that another Hurler baby in Minnesota has lost his fight and gone home to God. Little LB passed away yesterday due to complications from transplant.

I am devastated and discouraged to know how many lives MPS is still claiming, despite having the best medical care available. Its totally unfair, and I wish there were more that could be done for these precious children.

Please pray for Brinley's parents, Mandy and Trampus, and also LB's parents, Michelle and Lou, as they try to rebuild their lives again without their precious sons. God bless them all.

Good and bad news...

2009-04-28T23:26:00.003-04:00

So lets start with the good news... Gracie is well and has had no issues to speak of since our last update. She has been busy as a little bee and keeping us on our toes, but as far as her health is concerned, we have nothing but good days to report.

Gracie is getting braver when it comes to walking. She still prefers to crawl most of the time, but she will walk between people or furniture with little to no coaxing. She's also taking many more steps at a time lately as well. We're expecting her to just get up and start walking around on her own soon.

We are keeping the eczema pretty much under control with some steroid cream and we are slowly weaning off of the CSA, but it will be a long and cautious road.

Gracie has picked up a few more words, but "no" is still at the top of the list. However, I think it will soon be dethroned as Gracie's most frequently spoken word as "Yo Gabba Gabba" pronounced "yoyoDabbaDabba" is quickly moving up the ranks. :) She loves that show.

In other good news, we got our pictures from our Make-a-Wish portrait session, and we are so pleased. We hope to have more done as Gracie grows and as we expand our family. Here are a few of our favorites.

Now, onto the sad news... we have been following another Hurler child very closely through their transplant in Minnesota and we are sad to say that he was unable to survive the complications of transplant. Little Brinley became an angel yesterday evening after a long and hard battle. His family is devastated and could use some good thoughts and prayers. His fight for life has continually reminded me that we are so very lucky to have Gracie still with us, and to treasure every moment with her. Please lift the Craig family up in your prayers as they prepare for life without their precious Brinley.
Also, please offer a few prayers for our friend LB, as well. He is also having a few complications while recovering from transplant in MN and we are praying for a speeding recovery for him.

We are looking forward to our upcoming trip to Minneapolis in 2 weeks and we can't wait to see our friends, Rylie and Wyatt. Sadly, we will not be meeting the Craig family as we had hoped, but we do still hope to meet LB's family when we get into town. Gracie has a full week scheduled, but we are so excited to learn that Uncle Mack will be flying in for the weekend when we first get in! We haven't seen him since Christmas, so we are really excited for our visit.

Much love.

Hi all.

2009-04-14T23:44:00.002-04:00

We're just chugging along here in Gracie-land. We had a wonderful Easter with our cousins, Van and Nuvy, and Gracie has really loved having other little guys to play with. Maybe Aunt Hys can convince the elusive photographer aka Uncle Kent to give us some pics to post since Mommy and Daddy have not been taking many lately.

So far, so good on the health front. We are anxiously awaiting tomorrow when we get to begin our taper of the CSA med. Gracie had a follow-up appointment with the eye doc today at Nemours and she did very well. She's still not so cool with the whole "bright light in the eyes" exam, but the doc says the haze on her corneas is minimal and her vision seems to be normal. We're always happy to get good news.

In other news, Gracie has managed to pick up a bit of a cold thats been passed around the family, but it hasn't slowed our girl down any. She's getting pretty tough nowadays. We're spending more and more outside and she is just loving it. Managing the eczema can be a challenge, but we're working on it.

Right now, we're patiently waiting for our trip to Minneapolis to have our 1 year check up and we're making plans for a big trip to Disney World in December to attend our very first MPS conference! We are so excited to be able to plan this trip and we can't wait to meet lots of new friends and attend seminars about MPS and its effects on the lives of these kids.

Mommy has been stewing on and gearing up for a huge change in her life which has been inspired by Gracie. After we return from our trip to MN, Mommy will be going back to school to begin the Nursing program. It will be a long road that we will have to do in baby steps, but I feel that having gone through all we have with Gracie, I have a strong desire to give back the kind of care we received through our own process. Leaving Gracie will be the hardest part of it for me I'm afraid, but I know that she will be fine and that this will be a huge step for myself and our family. I am so thankful to have had experiences that have shown me that I can do things that I never thought I was capable of. Its just one of the many lessons Gracie has taught me over the past year.

Much love.

Yippee!!!!

2009-03-31T17:07:00.002-04:00

Gracie does not have GVHD!!!!!! Thank you, God!

The biopsy did confirm that she does have eczema which we had already assumed.

The plan is to begin tapering the CSA medicine starting on 4/15, Tax Day. I am so excited. It will be a slow taper because the CSA helps the eczema too and we'd like to keep from having a big flare up.

This is wonderful news and we are just so pleased that our little trooper has taken yet another big step forward. Go Gracie!!!!

Nemours tomorrow

2009-03-30T23:34:00.005-04:00

So Gracie's surgery last week went beautifully. Her tubes were replaced and she had her biopsy. We also got the news that her hearing is perfect. Gracie appears to have no hearing loss whatsoever and her ears function normally in all aspects. What wonderful news! Yay Gracie!!!! Congratulations on your first A+ on a hearing test!

Tomorrow morning we head to Nemours for our regular check-up, and I hope to have news about her biopsy, and of course I hope for good news. Gracie has been such a trooper throughout this process, so I wish for her to be GVHD free so that we can head into a more normal life.

If it does appear that Gracie has GVHD, I'm not sure what the next step will be and when we will be able to begin weaning from the CSA medicine. I hope to have more to report back tomorrow. Until then, we'll be praying for good news and hope that you will too.

Also, while you are all praying, friends, please say a little prayer for our new friend Brinley. Brinley has Hurler's also, and he is in the transplant process and having a bit of a bumpy ride. Please also pray for his family who need to be strong and comforted right now.

Much Love.

scooting along

2009-03-21T01:44:00.002-04:00

Well, we're still going along our daily lives doing well. Gracie has managed to stay pretty healthy these last few weeks (knock on wood) and we've been busy.

On Thursday, we had our weekly early intervention with a few surprise visitors. Miss Rachel, Gracie's Developmental Specialist, was here as always. I guess I should start by saying that she visits us on behalf of the Early Steps program, but she is actually employed by the Florida School for the Deaf and Blind (FSDB). FSDB works with the Early Steps Program in specific cases and we are one of them. As most of you know, Gracie is neither deaf nor blind, but her diagnosis of Hurler's syndrome and it's effects on vision and hearing gives her automatic entrance into FSDB's program. So basically Early Steps has contracted FSDB to oversee Gracie's Early Intervention plan. Stay with me here...

So on Thursday, along with Miss Rachel's visit, we had a meeting with some people from the Division of Blind Services. The coordinator of the Early Intervention program referred us to DBS in order to make them aware of Gracie's condition and to have them help provide us with anything we may need to assist Gracie visually as her life progresses. We have no idea what will happen with Gracie's vision in the future, and the damage that she has now is permanent. Presently, she still has issues with bright light, particularly bright sunlight and camera flashes. As far as DBS goes, she doesn't exactly fit the general standard of the people who they provide service to, but again, it seems that her condition itself will allow her to fall under the umbrella of DBS and receive their services. We met with the Program Coordinator and also our family counselor on Thursday to discuss the purpose of DBS in Gracie's life. What I found most interesting is that they are both blind. The Coordinator is legally blind, but still maintains functional vision, and our Counselor is 100% blind- born with congenital glaucoma and having completely lost his vision by age 10. I was pleased to see how DBS not only supports the people they serve, but also provides them the opportunity to help others like themselves navigate the difficulties that visual impairment can bring. While I was expecting a mundane meeting outlining how maybe they can help Gracie once she gets in school, I was actually inspired by these men who were so excited to meet us and Gracie an offer us their services which could span Gracie's entire lifetime. It was a good day.

Monday brings Gracie's ear tube surgery and skin biopsy. While she is sedated, she will also have a special hearing function test that can only be given while she is sleeping. Gracie has had many hearing tests since birth and has yet to pass one. Most recently, she had a basic audiology exam, but due to her age it was just not pracitical and it was deemed inconclusive. This test will give us an idea of how Gracie's inner ears are functioning and it will let us know what, if any, hearing loss she may have. We're of course hoping for none, but many children with Hurler's suffer some kind of mild to moderate hearing loss. This test will tell us once and for all. I am anxious to get the results.

The skin biopsy is pretty important too, although lately we've been seeing Gracie's rashes get more red and become a little scaly. I see them mostly on her legs, ankles, and tops of her feet- which incidentally are the parts of her which rub on the carpet when she crawls. Very localized, not really spread in large areas. Coincidence? I don't think so. Looks like eczema to me, but we'll let the test results speak for themselves. We have a prescription cream that keeps it pretty controlled and Gracie doesn't appear to be in any pain or discomfort. I'm hoping to hear of no sign of GVHD and begin tapering off the anti-rejection medicine again.

Well, its pretty late and I think I've covered everything. We'll be heading to Jax bright and early Monday morning, we need to check in at 6:15 am and surgery starts at 7:30 am. Be thinking of our girl and pray for a quick and easy procedure and good test results.

xoxox.

Milestones!

2009-03-13T13:12:00.002-04:00

Day +309
Can you believe that number! It seems only weeks have passed since transplant, but the days have slipped by quicker than I could have imagined. We are so thankful to be so far out from transplant and sharing all of these wonderful days with our beautiful Peanut.

Gracie continues to do well and stay mostly healthy (knock on wood) these days. She is still quite the busy girl and she is into everything. She's picked up a few more words, but the most notable one is "no". She uses it a lot. I'm sure you can imagine how this new development is affecting life here in the White house. :) Her pronounciation does lend a bit of humor to it, despite the frequency. She says it like "naw". Its really cute.

In even more exciting news, Gracie is well on her way to walking! She took her first steps last Saturday, March 7th! She can take about 5-6 steps at a time and we're doing lots of practicing. We are so excited to see her finally reaching this momentous milestone and we're hoping to see her really master walking over the next month or so. Of course, we say that now, but we'll see how we feel once she has us chasing her all over the place, right? I hope to post a video of her first steps here soon.

Wednesday, March 11th marked Gracie's 20 month birthday, and on that same day we received a wonderful gift of a family portrait session from the Make-a-Wish Foundation. A local photographer invited us to her studio (on her beautiful farm) and we spent a lovely evening playing and taking pictures. We meet with her again on the 30th to see the proofs. We are so excited and we appreciate both Make-a-Wish and Chontelle Brown of Cotton Blossoms Photography for giving us such a wonderful gift. We really enjoyed the relaxed way that Chontelle works and our session was so much fun. Feel free to check out her site, she does very nice work.

So for now, we're just hanging out and having fun. Gracie is scheduled for her ear tubes to be replaced on the 23rd and she'll be having a skin biopsy on that day as well. We're hoping to confirm that her rash is not GVHD so that we can begin to wean her from her anti-rejection medication again. Please say a few prayers for us as we have high hopes to be off this medication soon and begin living a more normal life one we reach our 1-year post transplant anniversary!

All is well...

2009-02-28T01:40:00.004-05:00

Which is why I've been afraid to post. It always seems that when I make a post about how well Gracie is doing, something happens and we end up in the ER or an extended stay at the hospital. So I'll start out by saying that Gracie has a runny nose. We're hoping it will just go away without morphing into something worse. She seems fine, and is playing and eating and being her sweet little self. She's a busy bee these days and I am just waiting for the day that she starts walking and then I am really in trouble. She's jabbering quite a bit and while most of it is incoherent, she has several words that she uses quite frequently. Off the top of my head, here is a list of Gracie's words and interpretations of Gracie's pronunciation.

that - Sounds like dat. This is a very common one. She points a lot at things she wants and says it.
bye bye - This is an oldie but goodie. She cracks me up with it because she uses it not only when we leave somewhere, but when she wants to leave too. I've noticed it a lot when we're at the doc's office. About 5 mins after we're in the exam room she starts saying bye bye and looking at her Daddy and me expectantly. She is a sassy one.
baby - She has a baby and uses the word a lot. Lately, she also combines this word with bye bye so that she says "bye bye baby"- its so cute.
door - usually pointing at the door or swinging it back and forth.
up
down
a bath - Sounds like baf. Also she never just says the word bath. Its always "a baf."
eye - usually accompanied by the finger gouging of whoever's eye she's talking about.
ball - This was her first word. :)
mama
daddy
kitty - sounds like tikky, which is soooooo cute.
boo - she's still a huge peek-a-boo fan.
hat - taught to her by Munts who is usually wearing one.
book - she is an avid reader. ;)
ooooh - her excitement sound, love it.
hey - said on occasion when she's playing with the phone.
busy bee - Sounds like bizza bee. Noni taught her that one.
pasta - sounds like pitta, she loves pasta like her cousin, Nuvy.
apple - sounds like appa.
splash - sounds like spish or spash... obviously said mostly during bathtime.
tub - buh or bub... another bathtime word.
tickle, tickle - ticka, ticka It is so funny when she says this.
She also is trying to say "diaper dance" from her Baby Signing Time videos. This one is a work in progress and requires my prompting by singing the song to her, but it sounds like di di dah.
Thats all I can think of right now... if anyone remembers any more, let me know so I can add them to the list.

She also signs a few basic words.
eat
more - she does this one a lot for Munts who then takes her to raid the pantry. :)
no - this is just her shaking her head.
hi - she has a great beauty queen wave.
all done
bed - she only does this one on occasion.
She can also do the hand motions for Twinkle Twinkle Little Star and has just in the past few days mastered the "diamond" motion. That was so exciting to see her work on.
I love you - This one is the best. She made this sign up herself. No one taught it to her, but one day she started doing this motion when we told her that we love her and she's done it ever since. Its like she hugs herself and turns from side to side. I think its the actual sign for cuddle. Jimmy and I love this one most of all.

She's a good pointer and she's really into learning what everything is called. We observe her moving her lips as she tries to make the sounds that we make when we're teaching her new words. Its exciting when she masters a new word or sign and uses it a lot.

Gracie got a cool Radio Flyer trike for Valentine's Day that has a parent's handle with steering capabilities. Her feet don't quite reach the pedals, but she loves loves loves to ride it all over the place. We took it over to Noni and Munts' house so that we could ride it outside up and down the driveway. Oh, she loves it. She also loves to walk her push toy back and forth on their driveway too. We're slowly discovering the joys of being outside. :)

Thats us in a nutshell. I think we've finally narrowed down a date for our Make-a-Wish Photo Session on March 11. We wanted to get it done before she got her new tubes in case she takes some time to recover. Also the possibility exists that we may not be able to put in the new tubes when we take out the old ones. It depends on the size of the hole thats there. We may have to wait for them to heal up and then go in again for the tube placement. I hope not, but it might happen, so we want to do the photos beforehand just in case.

Hope you are all well. Much love.

The monumental screw-up part 2

2009-02-14T19:05:00.004-05:00

Yes, I know. Slacker mommy.

So I gave you the first part of our lovely Shands/Lake Shore story a few weeks ago, and then left you hanging. Sorry about that, loyal fans. :(

So, lets catch up.

I left off at our admission to Wolfson Children's Hospital due to what looked like a contaminated blood sample drawn from Gracie's foot. Well, the next 4 days were a blur of continuous needle pokes for Gracie. It was terrible. Anytime any hospital staff would get near her, she would scream her head off, anticipating another poke. Unfortunately, her fears came true more often than not. Poor baby. As for the original positive culture from Lake Shore, our docs at Wolfson spent quite a bit of time attempting to get more information about the sample, and even the sample itself. They were told by the lab at Lake Shore that the sample was sent to Shands Gainesville for further study, but when our docs called "Big Shands", they had no idea what they were talking about. Somehow this sample had been "misplaced". At this point, our Docs at Wolfson are ready to make a formal complaint about this situation and Shands at Lake Shore sends them copies of the preliminary lab sheet which stated that the blood was drawn intravenously. Now, if you're officially confused by now, I don't blame you. But what that means is that someone lied when they submitted Gracie's blood for culture. I'm also being questioned about the capture method to make sure I know what I am talking about. I'm also officially furious. Shands at Lake Shore will be on the receiving end of this anger on the day a pull a bill from them out of my mailbox. I also plan on inviting them to pay for Gracie's stay at Wolfson's as well. We'll see how it goes and I will certainly keep everyone posted. And I'm sure it will come to no surprise to anyone that we will no longer be taking Gracie to Lake Shore if she runs a fever. We will go straight to Wolfson's from now on. Its just not worth it.

On to other topics.

Gracie continues to blossom and do well despite our minor roadbumps. We are still dealing with our mystery rash, and I am more and more convinced that it is NOT GVHD. Unfortunately, I will have to wait quite a while for confirmation of that because Gracie's biopsy (and ear tube replacement) is scheduled for March 23rd. Yuck! I had no idea it would be so long from now. When we first spoke to Dr. Joyce about this they told us to keep the next few Thursdays available for the procedure so we were thinking it would be pretty quick. I plan on calling our nurse on Monday and finding out if this is really the best we can do. So due to this, we are still on a therapeutic dose of CSA and no plans to wean off yet. Bummer.

Gracie doesn't seem to care much about the CSA issue, since she takes her meds in her formula these days. So she's just hanging out, being cute, and having fun. She's got about 15 or so words she can say, and she signs a little bit as well. We're hoping to cultivate her communication skills quite a bit in the coming months so that we can get her to express herself better and with less frustration. We're also hoping to see her start walking on her own. She's getting close, and I'm hoping it will happen soon. She is definitely a social girl, she loves to smile and wave at people and babble lots and lots. She's had a recent spike in her appetite as well. She's such a sweet baby and I am so proud of her for having such a wonderful disposition despite her circumstances in life. She really is an angel.

The momumental screw-up... part 1

2009-01-25T13:17:00.004-05:00

So we are home from Wolfson's Children's Hospital, where we endured a 4 day isolated stay that has left our poor Gracie looking and feeling like a human pin cushion- covered with prick marks, bruises, and bandaids. My poor sweet girl.

So let me give you the story- start to finish- of what really went down. Uncle Mack and Aunt Hyster did their best with second hand info, but I'm going to clear it all up so we can have a full picture of what exactly happened.

Rewind to a week ago- Sunday. Gracie is fighting a fever... it started out around 99 degrees. This is enough to warrant some concern, but 100.5 is our magic number. When Gracie reaches 100.5, we automatically head to the ER to have blood cultures taken and doses of antibiotic given. Anyway, she seemed ok despite the low grade fever, so we took a quick trip with Noni down to Gainesville to shop for a few hours. Gracie did well, but as time passed, she seemed to get more and more fussy. We headed for home and once we got there I took her temp and there it was 100.9. We packed Gracie up and Noni went with us to the ER (Shands at Lake Shore). We got in our room and waited for the inevitable needle stick which would provide not only a blood sample, but also an entry point for the IV antibiotics that would follow.

I'll give our nurse credit for trying, she did go for a vein in Gracie's arm with little success. Of course, Gracie squirmed and screamed her head off the entire time as the nurse wiggled the needle around looking for her vein. No go. On the other arm, she saw a big bruise we got from a previous draw which blew Gracie's vein over there. I think that by that time, she was nervous about sticking Gracie again and her judgement was not so clear. She suggested another method of blood capture- a heel prick, and we could give the antibiotic in a shot. Having just watched my baby writhe and scream in agony for a few minutes (which felt like an hour), I liked the idea of a quick stick and then a quick shot. WHatever would get us out of there the fastest. It never occured to me that I should question this method. I figured that if the nurse and doctor ok'd it, it must be a good alternative.

So she prepped Gracie's foot by cleaning it well with alcohol and chloroprep before giving her heel 2 pricks to make the blood flow more freely. Poor Gracie screamed again and kept screaming for next half hour as the nurse squeezed her foot and scraped the collection tube around her heel to collect enough blood for the sample. She had to get several tubes worth of blood, and this took a lot more time than anyone anticipated. By the time she filled the 3rd tube for the culture, the first 2 tubes (which were for a blood count and a metabolic panel) seemed to have clotted up. Fearing that the third tube would also clot, she called another nurse in to run it over to the lab. The second nurse saw the clotted samples and Gracie's still bleeding foot, and grabbed another 2 tubes and began squeezing Gracie's foot as well. I had noticed that she used hand cleaner when she first walked in, but I was distracted by my screaming baby and the discussion on the clotted tubes and I didn't pay much more attention to what she did. I was so paranoid that Gracie would have to be stuck yet again because of all of this mess. As Nurse 2 finished collecting the 2 new tubes, it was then that I noticed that her bare hands were covered in Gracie's blood. No gloves. I panicked, thinking only of the risk of contamination, and asked Nurse 1 if the 2 new tubes were for the the culture or the other tests. I was told they were for the other tests and then the nurses quickly took all of the tubes out of the room to send them to the lab before any clotting could occur. This was unusual because the blood for the culture is usually placed in the special culture bottle before being removed from the room... but with the entire process being out of the norm and my poor baby just absolutely terrified and screaming, I let it go and concentrated on calming Gracie down. In retrospect, I see the many red flags staring me in the face, but in the whirlwind of the moment, I was concentrating only on comforting my screaming daughter. The motherly instinct trumped my consciousness.

This was only the start of the screw-up...

On Wednesday evening while we were doing well and Gracie was fever-free and feeling better, we received a call from the folks at the ER telling us that Gracie's culture had grown something. I asked them to fax any and all results to our doctor at Nemours. I then called over to Nemours myself and asked them to page the on-call doc so that he could tell us what to do. He called back immediately and when I told him about the preliminary results of the culture, he told me to go ahead and pack up, that we were going to be admitted to Wolfson's for observation, a repeat culture, and a round of specific antibiotics. He asked me what kind of port or line Gracie had, and I told him that she had gotten her line removed at the beginning of the month. He asked how the culture was drawn, and I told him that they collected it from a heel prick. There was silence on the other end of the line, and then he asked me if I was serious. Ummm... this is clearly a bad sign. He told me that no one should EVER do an infection culture via heel prick due to the risk of contamination, but that we have to work under the assumption that the culture was correct and we needed to bring Gracie in. We packed up and loaded our poor girl in the car and headed to Jacksonville.

Upon arriving, we were ushered into the ER and we went through the normal registration and shown back to our little cubicle. Gracie endured 2 more pokes and then finally they were able to place an IV. They took another culture and hooked her up to the antibiotics while we waited for them to get us a room ready on the Oncology/Transplant floor. We finally made it our room at about 4 am and Jimmy got us settled in before heading back home in time to get maybe an hour of sleep before having to head to work. Gracie was restless and scared... and very annoyed to have the IV in her hand so it took some work to get her to sleep. We finally crashed about 6... although we were constantly disturbed by the many people coming in and out to check on this, or hook up that... etc. It was not fun for anyone, especially poor Gracie.

Stay tuned as the story unfolds in a future installment.

It's better than bad, it's nuts!

2009-01-24T02:43:00.003-05:00

Hi, everyone. Aunt Hys is all riled up at Shands (I guess that's who came up with this hare-brained blood drawing method). Gracie's bacteria-ridden blood sample was meaningless from the moment it was drawn, so the information it would have provided was non-information anyway. Now, this sample is "lost" somewhere between the Lake City and the mothership. If you're up for reading a foot-stamping temper tantrum about this, it's in the comments on Mack's post (previous). I'll spare the rest of you.

Pfeh.

Back in the hopital

2009-01-23T19:06:00.002-05:00

Hello. Sarah asked me to make a quick post.

Gracie had a fever, so the lab in lake city drew blood, and something grew on the petri dish, so Gracie had to go back to Nemours in Jax. Everyone thinks they contaminated the sample when they drew the blood (again) but they need to be in the hospital as a precaution. To top it off, they were supposed to fax the test results and send the sample to Nemours so they could identify the suspected bug. Well, they sent it to Shands in G-ville. Brilliant. Naturally, Shands has no idea what is going on, and don't know what sample we are talking about or where it is...

So, they had to have another blood draw in Jax, and are waiting for results. Preliminary data suggests that Gracie's potassium is low. The doc thinks the blood sent to the lab was hemolyzed (all of the blood cells burst - happens sometimes when too much suction used to draw blood). They'll draw again and check again... :(

In summary, they feel good, and think all is ok. That said, they will be in the hospital for a couple more days until it is all figured out. In the meantime, hospitals are very boring - no internet or anything - so feel free to give Sarah a call. I'm sure she'd appreciate it. 386-344-1095.

Uncle Mack

Its super-late but...

2009-01-20T02:22:00.004-05:00

I am so overdue for an update and our Gracie-bean could use a few prayers.

So, the stomach bug took a little longer to kick than anticipated, but she did wind up kicking it and things started getting back to normal. There was no need for a blood transfusion. We saw Dr. Joyce last Wednesday and he said that Gracie looked pretty good for the most part, but darnit the mystery tummy rash has returned! Total bummer. Dr. Joyce is again worried that we're dealing with GVHD and we are now back on what is called a "therapeutic dose" of CSA for the time being. While I am totally disappointed in this turn of events, it really is the safest and easiest option to determine if in fact it is GVHD that we're dealing with. I told our nurse that while Dr. Joyce may be right and I am on board with restarting CSA, I am not so sure that our mystery rash is what he thinks. So as an experiment, I have discontinued my constant application of the mystery-rash-killer-ointment aka triamcinolone (with Dr. Joyce and Nurse Paula's approval) to find out if CSA will handle the rash. Its been 5 days, and so far I am winning. The rash is still there (thank goodness its not itchy) deapite our CSA increase. The bad news is that this will probably mean that my poor peanut will need another skin biopsy to determine what the heck this crazy rash is. We'll just have to wait and see.

Fast forward to yesterday... Gracie spiked a fever and as per protocol, off we went to the ER for cultures and antibiotic. It was here that I can honestly say I began to miss our central line. They had to draw blood for the labs and unfortunately, Gracie has little baby veins and she was slightly dehydrated, so they were unable to get an IV. So they did a heel prick to get the blood sample and then we got our medicine as a shot. All in all, too many needles in the Peanut. She was not a happy camper. This morning she woke up and our fever was still hanging around, so we had to go back to the ER for another shot of antibiotics. :( Luckily thats all we had to do. So far, her cultures from yesterday have not grown anything, so there was no need for a second blood draw. We got home and Gracie took a long nap. She woke up and her fever was up again, but after some tylenol and a nice bath, she was cool and comfortable again. Here's hoping we wake up fever-free tomorrow.

So for all of our adoring fans, we ask for prayers against infection and prayers against GVHD. We would love to be able to begin our wean again. As long as we are on the "therapeutic dose" of CSA, Gracie must have regular blood draws to check the level of meds in her body. With no central line, this will not be a pleasant process for Gracie and I hate for her to be poked and prodded so frequently.

I hope I have better news for our next update.

So...

2009-01-13T14:52:00.005-05:00

Gracie is still not quite 100% over our bug, but she is better. She started feeling better the morning we went to see the doc, of course, and we were sent home feeling a bit better about how long its taking her to kick it. She's still tiring pretty easily and getting crabby once she gets tired. We head back to Nemours tomorrow for our regular appointment and I'm looking forward to getting some bloodwork done to make sure that everything is ok. My present thinking is that her hemoglobin might be a bit low (this happens when she gets sick) and thats why she tires so quickly. Maybe a little boost of blood can perk her up a bit more. She's happy and perky when she wakes from her naps and such, but she just doesn't seem to maintain the energy for as long.

She's eating and drinking fine now though, and I am thankful for that and knowing that she's not feeling quite so yucky anymore.

xoxoxo.

Spoke too soon...

2009-01-08T20:37:00.003-05:00

It turns out that Gracie's tummy problems are not quite over. She's still having yucky diarrhea and she's started vomiting a bit today. I switched her back to clear liquids (apple juice, pedialyte, and chicken broth) from her normal toddler formula and the puking has subsided, but she's completely refusing solid food. She only wants to drink and drink until she is full. Poor baby- her tummy hurts. :( We head to Nemours tomorrow so they can get a look at her.

I'm hoping all will be well, but prayers are definitely appreciated right now. She's been doing so well for so long... its almost like we were getting too comfortable. :(

P.S.

2009-01-07T19:06:00.002-05:00

Gracie has had 4 baths since Monday (when she got the ok for a bath) and she has loved every one of them. :) Thank goodness for the bath ok, can you imagine a stomach virus without baths?????? Yikes.

Sorry for not updating after surgery...

2009-01-07T18:21:00.003-05:00

but things for the most part went well.

The surgery itself was quick and easy. As a matter of fact, they had to page us up to the floor when she was done because we were still down in the cafeteria having a quick bite to eat.

We got back up to recovery and she was sleeping well and comfortable. When she woke up, she was fussy, but nothing more than what we expected after being sedated. She drank some apple juice and we were given the ok to go home. So we went to change her diaper and put her clothes back on and we saw 2 huge scratches on her left leg. Ouchie.

We called the recovery nurse over and asked her about them. She said she had no idea what they were from and called into the OR to speak to the OR nurse who was with Gracie the entire time. It turned out that they were having a particularly difficult time with their present surgery, but the notations on the chart said that Gracie had the scratches when she came in. Ummm... no. Not possible. Even if it were possible for her to even get those kind of scratches, there is NO WAY we wouldn't have known about it.
We told this to the recovery nurse and added that we know that accidents happen and that its ok, but we wanted to know what she might have been scratched by in case we need to hit her with some antibiotics just in case of possible infection. The poor recovery nurse said she would try to talk to the OR nurse again. She phoned into the OR again and the nurse told her that she was still wrapped up, but that the scratches were already on Gracie when she got her. Ok, now I'm starting to get upset. We gave the lady a pass, we just want her to fess up so that we know what the next step should be. Its not a big deal, just tell us that her leg got scratched on the table or something and she shouldn't need any extra precautions.
This never came about and we left with the promise that the OR nurse would call us when she has a spare moment. We haven't heard from her yet. I am a little peeved and I'll be letting Dr. Joyce hear about it on our next visit.

So Gracie took a nice long nap after we got home and I expected to have a cranky little Peanut throughout the evening, but it turned out that she was very happy to have her line out. She was all smiles and giggles... we even went out to Applebees to celebrate. Woohoo!

Saturday and Sunday were great days as well. Gracie was definitely feeling good and we were having a ball. Another celebratory dinner at Red Lobster and church on Sunday morning.

Then Monday rolled around and Gracie started not to feel so good. She turned away food, then threw up, and then started having some yucky diarrhea. Mommy started to worry... first about infection, then about the possibility of GVHD of the gut which is even nastier than the skin kind. I called Nemours first thing on Monday and when I heard back from them they eased my mind by telling me that it sounded viral to them. Hee-hee. I remembered the true meaning of a "mixed blessing" by Monday evening when I was also blessed with this lovely stomach virus. It turns out that I got the worst of it (which is also a mixed blessing) because Gracie pretty much sailed through it while I fought the sweats, chills, and a 102 degree fever while worrying my head off that Gracie was going to do the same thing and end up in the ER or even an extended stay in the hospital.

We're both past the worst of it now, although her diapers are definitely still yucky. I'm hoping she'll be back to normal in a day or 2 and we can keep trucking like we always do. We had a mishap with one of Gracie's meds over the past few days too, but we were able to iron it out and hopefully tomorrow will be a better and smoother day.

xoxo.

Prayers...

2009-01-02T00:49:00.005-05:00

Gracie will be having surgery at 8 am tomorrow (err... today) to have her central line removed and also she'll be having an eye exam under sedation. Of course since surgery starts at 8, we have to check in at 6:15. Yuck. Factor in a 1.5 hour drive and then look at the time of this posting and just imagine the lovely mood we will all probably be in. :P

No seriously, we are so excited to get this line out. As of yesterday, the entry point on her chest began itching a lot and Gracie kept scratching at it. Then to my delight (note the sarcasm) it began seeping a bit and the yucky ooze really irritated the skin around it. I gave her a bath and dressing change today because it was so yucky and wet and I discovered some serious skin breakdown under the bandage. Poor baby. This happened before and we learned that Gracie's sensitive skin doesn't like adhesives and plastic bandages and chloroprep and other stuff like that. We switched all of her dressings to breathable cloth and used different adhesive remover and cleaner, but I think her skin has just plain had enough. This line has been in place since the beginning of Sept, so 4 months of covering the same area is bound to make your skin unhappy, right? Anyway, I know that her skin issues will calm down once she is free of the 24-7 bandages. I'm looking forward to that quite a bit.

Please pray for an easy and quick procedure and safe travels for us. We love you all so much.

Despite the Grinchpox, we had a great Christmas!

2008-12-29T23:32:00.002-05:00

Due to the mysterious Grinchpox epidemic, Gracie was able to celebrate Christmas morning 3 times over the course of Christmas Day. First was our personal little family Christmas- it was here that Gracie learned the joy of ripping the paper from her gifts and playing with all of the great new stuff. Santa brought her a ball, some blocks, a few books, a pop-up toy, crayons and a coloring book, and the big gift- a Fisher Price Learning Kitchen. She LOVED it all! Wave #2 came when Jimmy's family came over in the mid-afternoon with tons and tons of wrapped gifts that Gracie could hardly wait to rip into. She cleaned up again with lots more toys and some big boy cousins to play with. :) Wave #3 came in the evening when Uncle Mack, Noni and Munts showed up with arm loads of more toys and clothes, and a little Radio Flyer wagon that Gracie has been pushing all over the house. :) By the time the 3rd wave came along, Gracie was opening everyone's gifts for them. It was too cute. So while the Grinchpox tried to ruin our day, it managed only to spread the fun throughout the day and well into the nighttime. I can assure you that Gracie was not disappointed with that... although I am positive it would have been even more fun to have the little cousins around too. All in all, we had a wonderful day.

In medical news, Gracie has managed not to catch Grinchpox, and her surgery to remove her central line is scheduled for this coming Friday. We absolutely cannot wait! No more fumbling with lines and remembering to flush them, no more paranoia about the line coming out-getting snagged-getting infected-clogging up-kinking and breaking etc., no more long stretches between baths (aka stinky baby), no more attempting a sterile dressing change with a wiggly toddler, and no more itchy-rashy-inflamed skin that is irritated by the bandage. This will be a huge step back to normal life for us and for Gracie and it will alleviate quite a bit of stress from our lives.
Gracie will also be having her eyes examined while she is under anesthesia, and I am looking forward to seeing what kind of progress the doctor will see from her last exam which was done prior to starting this journey. We also plan on scheduling another hearing test and hopefully Gracie will FINALLY pass it. :) I am confident that she will.

We are hoping that our loved ones all had a Merry Christmas and we're wishing you all a wonderful New Year! We're hoping that 2009 will continue to bring us many blessings and good health to our precious little girl.

Much Love.

The Pox that Stole Christmas

2008-12-26T01:57:00.004-05:00

Hi, All. Aunt Hys here again with a holiday bummer anecdote for you...

So, everyone was down in L.C. for Santa's big show, when Van, my 1-year-old son and Gracie's adoring cousin, who had been sporting (for several days) something that might loosely be called a "bumpy rash", but might have been a hyperactive flea, spiked a fever.

Nobody panicked. Gracie and her parents went home to hold their breath while I called the pediatrician. Verdict, bring him in to rule out chickenpox. Chickenpox!!!!

I hadn't thought of chickenpox.

In an HSCT family, this translates roughly to "Run for cover!" We were a thousand miles from our doc's office, so we were left with the local urgent care joint. So it was. I packed him into the car and thought all the way to the urgent care place about how I put off that chicken pox vaccine because we didn't want to expose Gracie to live virus, and, irony of ironies, I had by that action potentially exposed her to a very mean mother of a live virus in the rootin', tootin' intact-guns-blazing chicken pox disease itself.

We had a peripheral blood draw. (Fun!) And a sort of nebulous diagnosis of "Viral rash, may-or-may-not-be-chickenpox-so-go-stay-in-a-hotel-for-a-couple-of-days" and orders to come back on Christmas Eve for the "is you is or is you ain't chickenpox" appointment.

Hello Mr. ...-ist! Merry bleepin' Christmas!

Van remained in quarantine at Noni's house, riding the Tylenol rollercoaster. Gracie and her family remained on clock watch, waiting for Van's 24 hours fever-free. December 24th, I hauled him back in for confirmation that we were safe from the chicken pox. I was sure the doc would let us off the hook. Alas, the most I could get out of her was "It doesn't look like chickenpox, but it doesn't look like anything else either, so it still might be chickenpox. Here let me swab his throat for strep." The throat swab produced a gusher of barf the likes of which I've never seen, and came back negative to boot, but there was still the fever.

I'm calling it Grinchpox until somebody tells me different.

So Gracie had to stay home Christmas day, and it was the first Christmas in 30 years that we all missed Sarah. Sarah just smiled at all my futile apology, saying "It's all so we can have many more Christmases with Gracie, right?"

...And that's the important thing. Merry Christmas and Happy New Year to everyone!

Good appointments today...

2008-12-15T17:42:00.004-05:00

So our day at Nemours went pretty well today. We started with a consult with the Surgery folks and that went pretty quickly. The doc gave Gracie a once-over and pronounced her fit for central line removal. They will be coordinating with the Opthamology doc who wants to do a full eye exam while Gracie is sedated. We're happy to get both procedures done with as little trauma to the Peanut as possible.

We went upstairs to clinic to see Dr. Joyce and got more good news. Gracie's rash is not GVHD. They were all gone after using the steroid cream and we all breathed a sigh of relief... although she had a new one on her face that showed up 2 days ago. Dr. Joyce took one look at it and pronounced it dry/sensitive skin rash... with the possibility of an allergy to the wipes I've used on her face. He said we could use hydrocortizone cream on it, if we stay far away from her eyes, so I'm hoping we can make it disappear pretty quickly.

We got the go ahead to restart the CSA taper and we're glad to again be on our way to ridding ourselves of yet another yucky med. :) Yay!

Aunt Hyster and her gang should be arriving tomorrow and then Uncle Mack is coming this weekend...yay! Gracie is so excited to show everyone how much hair she has been growing! She needs her first official post-transplant haircut now. Luckily, her favorite hairstylist is Aunt Hyster and so she'll be able to get one really soon. :)

We'll update again when we find out when Gracie's surgery is scheduled.

Much love.

Please forgive the lack of updates...

2008-12-05T09:12:00.002-05:00

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.

Dear beloved friends and family,

2008-11-28T16:16:00.005-05:00

Yesterday, as we celebrated all of the blessings that we have to be thankful for, our transplant friend Kamran in Minnesota went home to God after a very long and tough fight with Graft vs. Host Disease and other complications from his unsuccessful transplant.

Kamran was such a sweet and loving little boy who welcomed us on the the transplant unit as he was being released back to the RMH, and later, brought many smiles to our faces once we returned to the RMH as well. One of my favorite moments with Kamran was during one of the Thursday night Bingo games and Kamran came over to our table to play with Gracie and her new laptop toy. :) She loved having him close by and gave him many flirty smiles behind her mask.

I ask God to envelop his parents, Kelli and Bahram in his comforting arms during this difficult time. And I know that despite the endless grief that those who have lost a child must suffer, they will be able to always celebrate Thanksgiving in its purest and most precious form. They will always be so thankful for the beautiful moments they shared with their amazing son.

I ask you all to please pray for Kamran's family during this difficult time.

We will never forget you, sweet boy. We are so thankful to have been able to know you and be touched by your life and love.

So far so good- nothing to report...

2008-11-20T19:34:00.003-05:00

Gracie continues to do well, and that leaves me with not much to report. We're slowly tapering her anti-rejection med and we'll go back to Nemours to see Dr. Joyce in another week for another check-up. We'll also be getting Gracie's eyes examined again. She hasn't had an eye exam since February... before she started ERT. I am interested to see what the doc says about her cloudy corneas. We also need to visit the audiologist again to see if Gracie can finally pass a hearing test. Thats next on the list. We need to get all of these exams done so that our therapists (Speech, PT and OT) can get a good idea of any hurdles we need to overcome.

Thats pretty much all I've got to report but I do have 2 pics to share from the BMT 40th Anniversary Celebration. They're pretty cute. Enjoy.

The results are in...

2008-11-14T12:36:00.004-05:00

Hi all. We're back home in sunny Florida. We had a great check-up with our Jacksonville doctor, Dr. Joyce, and we have wonderful news to report. Gracie's engraftment is still at 100%!!! This is an important milestone because the risk of rejection reduces drastically at this point. We start to taper off her anti-rejection meds tonight. They expect this to take a month to a month and a half. We're also hoping Gracie gets to have her central line removed by the end of the taper.

Gracie steals the show!

2008-11-10T00:40:00.003-05:00

As promised, here is the clip from the Minneapolis Fox news station where Gracie and her Dad and mouthpiece Mom had their cameo appearance alongside Kenechi Udeze, a Minnesota Vikings defensive end and fellow transplant recipient at the Celebration and Reunion.

If you keep watching, you can see Gracie flirting with one of our beloved doctors, Dr. Orchard, who heads up the Pediatric Inherited Metabolic Disorder division of the Blood and Marrow Transplant Program. He's a cool guy and Gracie always has lots of smiles for him.

Video Clip: Udeze's Mission

Read the news story: Kenechi Udeze Attends Benefit to Crusade for Bone Marrow, Blood Donors

Enjoy!

Busy Busy Busy...

2008-11-09T16:52:00.002-05:00

Well, our trip here to Minneapolis has been a busy one, and we really have enjoyed being here and seeing so many people who are so special to us. We've also had fun visiting a few of our special Minneapolis restaurants and favorite stores, and Gracie has charmed and smiled her way through every moment of it.

So for the important news...
Gracie's appointments on Thursday went very well. Everyone we have seen are just amazed by how wonderful she looks, how happy she is, and how much she has improved. Gracie's transplant doctor, Dr. Tolar told us that he is usually hesitant to say it but that Gracie is a true example of a successful transplant. My heart soared at that statement, and I could not help but to give him a huge hug and thank him incessantly. We discussed the next steps that Gracie will take, and provided her engraftment is still good and stable (we'll get that news in about a week or so) we will begin to start tapering her CSA (anti-rejection med) over the next 6 weeks or so, and we will also be able to have her central line removed in the next month as well. We are so excited!!!!
Gracie also had an appointment with her neuropsychologist, Dr. Bjoraker. This was another great appointment! We went through a few hours of assessment testing and it turns out that Gracie has made some huge strides in development since transplant as well. She has almost overcome the setbacks she suffered due to the transplant and she never ceases to try something new. We are just so proud of her and Dr. Bjoraker was really impressed with her progress.
We also sneaked up to Unit 5D to surprise our special friends who took such good care of us when we were inpatient. We were so happy to see 2 of our most special nurses, Carolyn and Jenny, and Gracie was happy to see them too! She gave Jenny some big snuggles and lots of smiles all around to everyone. We chatted for a few and caught them up on all of our great news before we had to let them get back to work. From the unit, we headed to the rehab center to visit with our friend and awesome speech therapist Mary. Gracie gave her a big hug and more snuggles and we shared with her how well Gracie is eating and babbling. Gracie even said a few "dada's" for her. Mary was so happy to see her Gracie and she was also impressed with all that Gracie is up to these days.
The next day we went back to rehab to visit with Susan, our physical therapist, and Gracie had no trouble showing off all of her motor skills- crawling, cruising, climbing, playing, pulling up... everything you can think of. Susan was also really impressed with how far Gracie has come in just a few short months.

Saturday was the day of the Blood and Marrow Transplant Program's 40th anniversary celebration and reunion. What a wonderful celebration!!!! We are so glad we stayed for it and we had so much fun. Gracie was great and I think she was the youngest transplant recipient present. She met lots of people, yound and old, just like her and Jimmy and I were both touched and inspired by the presence of so many survivors. We heard from the doctors who started the BMT program here at the U of Mn, and even from a Minnesota Vikings Football player who also went through a transplant here this summer... just like Gracie.
And wouldn't you know it, Gracie the celebrity and her mom and dad were on the local news here in Minneapolis last night! We hope to have a link to add here to the blog so you all can see, otherwise, we'll try to post the video here in the next few days.

We have lots of pictures to post as well so we will get to that asap. We'll be flying back home tomorrow and while we have had so much fun back in Minneapolis, we are looking forward to the many comforts of home.

Much love.

Safe and sound on our old stomping grounds...

2008-11-06T00:06:00.002-05:00

We're back in Minneapolis and in the RMH. We met up with our friends Rylie, Jade and Lyle, and we also got to spend a little time with our friend Kam's mom, Kelli. We really enjoyed all the time we were able to spend catching up. There are not many people we know still here... its funny to be in such a familiar place with so many unfamiliar faces. I keep looking for all of my fellow transplant moms and Jimmy keeps trying to go to our old room. :) Too funny.

We have several appointments tomorrow and we're really looking forward to seeing some of our favorite docs and nurses in clinic tomorrow.

Gracie has been doing great and she's having a blast. She was following her big girl friend Rylie all over the place and she is totally enamored with her. We got some great pics that we;ll try to post tomorrow.

Sweet dreams from chilly MN.

Up late- packing and posting...

2008-11-03T02:09:00.006-05:00

Well, I have been slowly packing for our return to Minnesota on Tuesday. It is amazingly hard to pack for a week long trip when the weather is being wonky. Apparently it was 70 in Minneapolis today, but later this week a 30% chance of snow is predicted. According to a fellow transplant family, it snowed last week too. Ummm, hello? We're from Florida- its hot as Hades here for 9 months of the year, maybe we get a few weeks of "nice" weather, and bam!- its freezing. But 70 one day and snowing 3 days later is something I have no idea how to prepare for. Not to mention the fact that you have to pay to check all of your bags now. This is one of those moments where I just have to laugh at my frustrations.

On the upside, we are really excited to be heading back for this milestone checkup. We can't wait to see our beloved doctors and nurses again, not to mention the staff and friends at the RMH. And especially, our friend Rylie who will be having her 6 month checkup too! Yay Rylie!

Our schedule started out as 3 days of doctoring, but we were notified last week that Gracie doesn't need an MRI, so therefore she will not be sedated and will not have a lumbar puncture. Its surprising, but we're glad that she won't need to go through that on this particular trip. However, that means that Gracie's appointments are being consolidated into just one day. We booked our trip for 6 days. Ummm. Oops. :) I guess we're just going to have a little mini-vacation in Minneapolis then. Again, a laughable situation, but we'll roll with the punches.

Lots of love and long distance snuggles to Uncle Mack who said goodbye to his gall bladder on Friday. Hope you're feeling better, Mackie. Gracie loves you and told me to tell you to get some rest and drink plenty of fluids. :)

xoxox

I must share a letter I sent...

2008-10-26T01:42:00.003-04:00

to the television show "The Doctors" after I watched an episode that aired on 10/22/2008 about parenting. I don't know if these people will even read the letter, but I could not just sit by and watch these doctors advise millions of people about a subject they clearly know little about. That subject is cord blood donation, something that I'm (obviously) quite passionate about. I am afraid of what kind of damage that these doctors have done to cord blood donation programs with their unresearched and flippant remarks, and so I do hope that my letter is read and will inspire them to make things right.
Thanks for reading.
xoxo

To whom it may concern:
My name is Sarah Kelly White and I have a 15 month old daughter named Gracie. Gracie was diagnosed at 6 months of age with a rare metabolic storage disorder called Mucopolysaccharidoses (MPS) type 1 – Hurler’s Syndrome. Hurler’s Syndrome is a fatal genetic disorder caused by a defective gene that is responsible for producing an enzyme called AlphaL-Iduronidase which breaks down the mucopolysaccharides. In Hurler’s patients, there is no enzyme being produced and therefore metabolic wastes are being stored in the cells causing them to destroy themselves and most of the patient’s vital organs. Without treatment, children with Hurler’s Syndrome generally die by the age of 10 following a long decline in functioning and severe mental retardation.
Thankfully, there are treatment options for patients with Hurler’s Syndrome. First, there is an artificial enzyme which can be given through IV which will slow or stop the deterioration of the cells and organs, however it cannot cross the Blood/Brain Barrier, so it is not ideal for long term use. The other option is a Stem Cell Transplant using either donated Bone Marrow or donated Umbilical Cord Blood. This is the best treatment going as it can save the patient’s life and also their quality of life. My husband Jim and I chose this option for our daughter, and she underwent a transplant of donated umbilical cord blood on May 8, 2008. As of today she is almost 6 months post-transplant and doing very well.
On the Daddy Boot Camp episode of “The Doctors” which aired on October 22, 2008, an expectant couple posed a question to your panel of physicians about banking cord blood. He specifically asked if they should privately bank their baby’s cord blood for future use or if they should donate it to a public cord blood donation facility. I was shocked and appalled when I heard the responses from Dr. Jim Sears and Dr. Lisa Masterson. They both agreed that this couple should privately bank their baby’s cord blood because public donation sites are few and far between. They then went on to laud the private banking option and stated that it would be “worth millions” if they should need it later.
I sat stunned for a minute in disbelief that this panel of doctors who were chosen to give medical advice to millions of people via television would give such irresponsible and inaccurate information. According to the website for the National Marrow Donor Program (www.marrow.org), there are hospitals in at least 20 states who offer expectant mothers the option of donating cord blood. While it’s not offered everywhere, it is offered in more than just “a few places” which is what Dr. Masterson said on the air. If the mother’s chosen birthing hospital is not listed, they can contact Cryobanks International who accepts donated cord blood from anywhere in the continental U.S. to be listed on the NMDP registry.
Private Cord Blood Banking is expensive and in most cases, it’s useless to the family who is paying to bank it. The AAP states that the chances of a child needing his or her own cord blood stem cells in the future are estimated to range from one in 1,000 to one in 200,000. Private cord blood banks target parents at an emotionally vulnerable time when the reality is most conditions that can currently be helped or cured by cord blood stem cells are treated by providing a necessary component from the donor stem cells which is lacking in the patient's own cells--for which a patient's own cord blood would be useless.
The American Academy of Pediatrics, of which Dr. Sears is a member, has publicly stated that they encourage the donation of umbilical cord blood for public use. The AAP openly discourages private cord blood banking unless there is knowledge of a full sibling in the family with a medical condition (malignant or genetic) who could potentially benefit from cord blood transplantation. http://www.aap.org/advocacy/releases/jan07cordbloodfaq.htm
The American College of Obstetricians and Gynecologists has also publicly stated that OBGYN’s should be advising patients of the option of cord blood donation and giving them accurate information regarding the usage of privately banked umbilical cord blood and the very slim chance that the banked blood will actually be used for that child or a sibling.
http://www.acog.org/from_home/publications/press_releases/nr02-01-08-2.cfm

Both the American Medical Association and the American Society for Blood and Marrow Transplantation have also made similar public statements advising physicians to encourage expectant parents to donate their cord blood to a public bank as opposed to privately banking it.
Considering all of the public statements from these highly respected organizations and the availability of all of this information on the internet, I find myself wondering how Dr. Sears and Dr. Masterson could give the couple on the show these recommendations in good conscience.
The basic fact is that these doctors have now provided the Cord Blood Donation Program with yet another hurdle to overcome. Considering the wide scope of the potential viewing audience, Dr. Sears and Dr. Masterson have told millions of people that it’s just not worth it to donate your cord blood to someone else who needs it. For parents like my husband and me, the implications of this type of attitude and carelessness are grave. The irresponsible and ignorant remarks that were made by these doctors may have cost hopeful families that “perfect” or even “good enough” cord blood match that their child so desperately needs to live. And just like anything else, as the demand for these centers increases, more will become available to meet the demand which will offer even more people the opportunity to donate their cord blood.
I hope that those who receive this letter will do the truly right and responsible thing and rectify this situation by any means necessary.

Copied to:
Dr. Jim Sears, The Doctors
Dr. Lisa Masterson, The Doctors
Dr. Phil McGraw, Executive Producer , The Doctors
Dr. John Wagner, Head of Pediatric Stem Cell Transplantation, University of Minnesota
Dr. Paul Orchard, Medical Director, Inherited Metabolic and Storage Disease Blood and Marrow Transplantation Program, University of Minnesota
Posted publicly on http://graciewhite.blogspot.com/

Dear friends...

2008-10-26T00:16:00.002-04:00

Please pray for one of our transplant friends from Minnesota. His name is Kamran, and he is such a sweet little boy. He used to play with Gracie and share toys with her during the weekly bingo nights and his parents are kind and friendly people who never failed to share a smile with others, even on the bad days. Right now Kam is on a ventilator and he's been having a hard time battling infections and Graft vs. Host Disease. Please lift Kam and his parents, Kelli and Bahram, up in your prayers.

Much Love.

Reflections...

2008-10-20T00:15:00.002-04:00

Its not often that individuals get to experience they type of outward support and love that we have been shown since we began this journey. Our families, friends, and especially our church family have given of their time, talents, and hearts in a way that humbles both Jimmy and me. Although the road we are walking is a difficult one, we walk it with complete understanding of the support system that God has so generously blessed us with, and despite the hardships, we are so thankful to have had the opportunity to see love in such a tangible form.

This past weekend we celebrated with friends and family as they again came together to support us with a party and silent auction in honor of our Gracie. It was a night of fun and laughter, and Gracie was as charming and smiley as ever. Our girl has touched so many, but it is us who have been given true enlightenment. We could never begin to express our thanks to the many loved ones who have supported us through our ordeal, and our hearts remain full of peace and hope knowing that no matter what, we will be held tight in the arms of such loving comfort.

Thanks to all who love our Gracie and us.

Back home

2008-10-14T21:23:00.002-04:00

Just a very quick post to let you know that gracie is back at home. Sarah said Gracie is ecstatic to be able to crawl around and chase the kitties. We're glad she's home.

The last few cultures have all been clear, so we are cautiously confident that the bug is gone. Gracie will have a few more cultures across the week to monitor.

Next big exciting thing is the trip back to MN for the 6 month check up. Seems like there will be a lot of Hurler families there at that time. In fact, Sarah said that there might be some sort of transplant reunioin.... I don't know and details, but I wish Gracie and all of the others the best of luck.

Thanks for keeping up with us. :)

Quick Update - Still Waiting

2008-10-13T01:30:00.002-04:00

Howdy. Gracie ad Sarah are still at Wolfson's in Jax. I have nothing new to report other than that. They should have more news tomorrow. I did check in with the docs in Minnesota to verify that wolfson's is on the right path. I sent a message at midnight last night, and by 1:00 Dr orchard responded. How awesome is that! Anyway, he reassured me that they are doing what would be done in Minnesota if she were there.

What is gram-positive bacillus?

2008-10-12T00:35:00.002-04:00

Just a quick science post for all those who might be interested...

"Gram-positive bacillus" is more of a description than a diagnosis. "gram-positive" means the bacteria show up when stained with "gram stain"--a purple stain used to make bacteria show up better on microscope slides. "Bacillus" is a description of the shape of the bacteria. Bacilli are "rod-shaped" bacteria.

Other than that, there is a list as long as your arm of species of gram-positive bacilli. They are mostly harmless--mostly.

Gram-positive bacteria have weaker cell walls than the gram negative kind (the weaker cell wall happens also to absorb the stain--neat trick, huh?), and so are not usually the dangerous kind. "Gram-negative" bacteria tend to be tougher, and those are usually the ones that are human pathogens.

Only two Bacillus species are considered medically significant: B. anthracis, which causes anthrax, and B. cereus, which causes a foodborne illness similar to that of Staphylococcus. If any of you docs out there know of any other bacillus baddies, please report them immediately to Wiki. ;-)

Now I'm all the way up here in Philly, so I don't know first hand, but I would venture to guess that we're talking about B. cereus in this case. I seriously doubt that the folks on Gracie's medical team would be behaving in such a cautiously lackadaisical way if she had anthrax...

Hospital update

2008-10-11T23:26:00.002-04:00

Just wanted to give everyone a quick update. Gracie's second culture also had a gram positive Bacillia, which is some sort of bacteria. A staph or something similar. Weird thing is that she has no fever and is bouncing off the walls in that hospital. The infectious disease doctor came by and sounds like she thinks the infection might bed in her catheter. They should know what the bug tomorrow, and will know if ots in the line or her body by Monday. I'm a little concerned by the lack of urgency on their behalf, but not sure there is much I can do about it. I plan to email Dr. Orchard tonight to see if he has an opinion on this.

Good news is Gracie seems to feel great. I'll keep you posted on her progress.

Uncle Mack

Quick Hospital Update

2008-10-11T13:49:00.005-04:00

Gracie is at Wolfson Children's in Jax. I was there last night to see her and she looked fantastic. She was happy and playing, and wanted nothing more than to get on the shiny colorful floor and play, but no no no... No floors in the hospital. They have a big oval shaped crib with a big clear, tinted plastic "hood" over it. She loves to pull herself up and cruise around it (she even completely let go once). She also loves to bang on the plastic hood. :)

It has been a couple of months since I've seen her and she looks so much better. First off, she has a full layer of hair on her head. It's short, but dark. Also, one of the anti-rejection meds she takes makes her furry all over. She has a nice layer of fur on her back and shoulders... very cute. I wish I had taken anti-rejection meds when I was in high school and college. I would've dated a lot more (bu-dum-ching). Not sure how namy of you remember what a monchichi is, but she looks eerily like a monchichi.

On to medical stuff. While still not 100% sure, it sounds like the positive culture was for a skin staph infection. I'm no doctor, but it sounds a little odd that a skin infection would show up in a blood culture, especially when Gracie has no open wounds or anything. Our current best guess is that the sample the collected was contaminated between the time they drew blood and the time they started the culture. They took two additional samples to see if anything shows up.

Regardless, giver her weakened immune system, the safe call was to admit Gracie and fill her up with powerful anti-biotics. She'll be in the hospital for a couple more days until they can rule out the infection.

Thanks for checking in.

Back in the hospital

2008-10-10T12:18:00.000-04:00

Noni called me this AM and told me that the lab at Nemours called and that something grew on one of Gracie's cultures. I'm not sure of any details, but the asked Sarah to pack up and come to the hospital in Jax. Hopefully nothing to panic about, but figured you all would want to know...

I think they'll be at Wolfson Children's. The plan is to stay there for at least 2-3days. I'm sure they'll want Gracie on some high octane antibiotics to help her fight whatever grew on the culture. Hopefully she'll be discharged shortly, but this is a good reminder to keep on our toes.

Please say an extra prayer for Gracie across the weekend. We'll update as we learn more.

Uncle Mack

Fun times with Gracie...

2008-10-10T00:03:00.002-04:00

So, Gracie's appointment on Tuesday went well. We got there and first thing we got great news that Gracie had not only maintained her weight, but she had gained weight despite having her TPN reduced! We were so excited- and so was our Nutritionist!

Gracie had a quick meeting with the doc who said she looks great, followed by her routine blood draw. She also had a repeat RSV culture (from her nose). As has become our routine, we stuck around in Jax to make sure that the blood draw was sufficient for accurate results before heading home. We got a call from our nurse and she told us that it was all good and we could head home. Also, her RSV test was negative! Yay!

We got home from our long day and Gracie immediately settled down for a long nap. When she woke up, she was screaming her head off and when I went to pick her up, I noticed that she felt warm. Oh no- not again. We took her temp and again she had a fever of 101.5. We called back over to Nemours and got Dr. Joyce on the phone and it was a dose of Tylenol and back to the Lake Shore ER for Gracie. She had another round of cultures drawn and was again given an IV antibiotic. The next morning her fever was down, but our doc sent us back to the ER for one more dose of the antibiotic again. Since then she has been fine and her normal sweet self and no fever to be found. Nothing has shown up in her cultures either. No one knows why she spiked the fever, but everyone seems to be in agreement that Gracie is ok. My personal opinion is that Gracie has become dependent on her daily routine.... she is like a finely tuned weather instrument, as Uncle Mack would say. :)

On Tuesday, our clinic day in Jax, Gracie's routine was clearly disrupted. First of all, Gracie is very "regular", if you know what I mean. She basically has a pooping schedule that begins first thing in the morning and then happens at least once or twice more throughout the day. (I hope I am not grossing our loyal readers out with this lovely topic.) Well, on Tuesday, she didn't poop at all until well into the evening. This is a huge deviation from her schedule. Follow that with a long day spent mostly strapped in her carseat and you have a baby who doesn't feel so well. Since then, she has been back on schedule and happy as a clam, so who knows. We're going to see if maybe coming straight home after our next appointment will help. I certainly hope so.

Also, the best news came yesterday. Our Nutritionist called and asked if we were feeling adventurous, and despite our fever adventures, we told her we were. So I am pleased to announce that I hooked Gracie up to her last bag of TPN tonight!!!! Woohoo! As of tomorrow, Gracie will finally sleep without being attached to any tubing or pumps. I am so excited! Of course, we will be really pushing the food and fluids on Gracie to prevent having to restart her TPN regimen, but for now, we are so pleased that she is making such excellent progress and taking huge strides in her recovery.

So to end this long post, here are the pictures I promised. Smile and enjoy.

No news = Good news

2008-10-06T00:16:00.003-04:00

But thats no excuse for yet another slack in posting. Sorry all.

Gracie continues to do well and is still not showing any visible signs of RSV. We go back to Nemours on Tuesday and I am hoping and praying that we will get a negative RSV test so that we can celebrate Gracie's official germ-kickin' immune system and get back on course with getting our Speech and Physical Therapies going and getting out and about again.

She is eating and drinking very well these days. So well that her TPN just got reduced to 10 hrs a night from 12. I'm hoping that Tuesday will bring another good weight and the chance to take it down again to 8 hours! How wonderful would it be for Gracie to be tube free every night??? She's working on it and I couldn't be more proud of her.

Her hair is coming back in full force... she just gets fuzzier and fuzzier. She officially needs a haircut now. Its starting to grow over the tops of her ears. She is just so cute and fun to be around these days. She's crawling all over the place and pulling up on everything. As soon as she figures out how to keep balanced, we are going to be in trouble... she wants to walk so badly.

I've been snapping lots of cute pictures of her lately and I hope to post a few tomorrow if I can. Stay tuned.

xoxox

yesterday's appointment

2008-09-27T09:08:00.004-04:00

Our appointment yesterday was actually kind of comical. Gracie had kicked the fever the evening before and was feeling much more like herself. We drove over to Nemours in Jacksonville and we're shown into our exam room immediately upon arriving. As the nurses and then Dr. Joyce came in, Gracie put on one of her best shows of smiling, flirting, and playing. Dr. Joyce took one look at her (and listened with his stethoscope) and canceled the xray of her chest and the blood cultures. Why? Because as of right now, she's fine. There's no need to draw blood again and certainly no need for another xray. She had gotten both of those things done here in Lake City at the ER and nothing has shown up in any of them. So they giggled with us and cheered and clapped with Gracie and sent us back home (after about 15 minutes total) with instructions to call and check in with them on Monday and Thursday, and of course to call if there were any changes. Our next appointment will be a week from this Tuesday if all goes well, and we hope at that time to get another culture showing that Gracie is RSV negative. Until then, we're keeping Gracie pretty close to home if at all possible. We want her to stay well and rested and minimize her exposure to any more germs while her new immune system is busy kicking this virus in the butt.
Thanks for all of the extra prayers and please keep them coming. We need her to strong and keep fighting this virus. We're praying that she continues to keep the symptoms at bay and stays fever free.

Much love.

The good news...

2008-09-26T12:32:00.002-04:00

Gracie's fever is gone. She's been having normal temps and she seems to feel much better and has been playing and eating a little. She's not 100% though, and so I'm halfway waiting for the other shoe to drop. Of course, she may surprise me and kick this nasty virus without a whole lot of trouble. I certainly hope so. Only time will tell.

We head back to Nemours today to let Dr. Joyce have a look at Gracie and probably draw a few more cultures. I'm praying for a pretty clean bill of health other than the RSV (and maybe even the miracle of the RSV being crushed by Gracie's new army of white cells- hey, a mommy can hope, can't she?) and to stay out of the hospital. I hate the idea of penning Gracie back up in a little room again. :( But we will do what we have to do to keep our girl as healthy as possible.

Please say a prayer for our girl today, no more RSV. And please continue to keep Liam's family in your prayers as well. I am just so sad for them.

xoxox

heavy hearts

2008-09-25T22:41:00.005-04:00

I'm so sad to tell you all that another Hurler Angel has gained his wings today. Liam lost his battle this afternoon and is now home with God and Hurler free.

Our hearts and thoughts are with the Larrow family. We hope that they may find some peace and comfort in this most difficult time.

Update...

2008-09-24T11:51:00.003-04:00

So after speaking with our nurse this morning, we do not need to head back to Jax as of yet. Like I said in my previous posts, RSV is a virus and therefore, its not really treatable... all you can do is treat the symptoms. So far, Gracie's only symptom is a fever and we've gotten it down to a reasonable 99.7 degrees with Tylenol. Dr. Joyce wants us to go back to the ER today and get another IV infusion of a general antibiotic just to be on the safe side, since we now know that Gracie's new immune system is going to be busy fighting this RSV for a little while.

Other than that, we have to just let this run its course. We have to make sure that Gracie is well hydrated and keep an eye on her breathing. We have a prescription for a nebulizer and meds to use to try and break up any secretions that may start hanging around her airway, and so we'll just watch closely.

Still feverish...

2008-09-24T08:39:00.002-04:00

and waiting to hear from the doctor to tell us what to do.

Unfortunately for a viral infection there's really not much to do besides treat the symptoms. Right now her only symptom is a fever. I'm not sure if they will want her to go back to Jacksonville and be admitted to Wolfson's or of they will want her to stay home and have a close eye kept on her. I'll update when I know anything.

Please pray extra hard for Gracie.

Long day at clinic, followed by a long night in the ER

2008-09-24T00:02:00.003-04:00

Thats right friends, we just got home from the Emergency room. This afternoon after getting home fom Jacksonville and our clinic visit, Gracie decided to spike a pretty good temp. We took her temp several times over the next few minutes to be sure, and we were- her temp was 101.8!!!! For all post-transplant patients, this is a terrifying moment. We called our Doc at Nemours, heard back from him within minutes, and headed over the the local ER to have some tests run.

To make a (very) long story short, Gracie has RSV. RSV is a yucky respiratory virus that can wreak havoc on delicate lungs and a beand new immune system. Needless to say, we are none to thrilled with this new hurdle to jump. On the other hand, Gracie's cell counts are pretty good, and we have some great doctors here and in Minnesota who we know we can call on at anytime. Anyway, we were sent home with a prescription for a nebulizer (for treating the virus when it starts getting nasty and causing cough and wheezing and stuff)and instructions to call our doctor first thing in the morning. Yikes.

So to our loyal friends and readers out there, I ask you to pray for this case of RSV to be mild and for God to strengthen and protect Gracie's lungs from attacks by this icky virus. Pray for quick healing and strong white cells to fight it off. Pray for protection from fevers and illness.

God, please protect my precious baby.

Sending our love to Liam Larrow

2008-09-23T15:35:00.002-04:00

Hi, everyone! Aunt Hys here, again.

As you know, we follow the journeys of several other families who are also battling Hurler syndrome. You can follow them, too, by clicking links from the list at the right side of your screen.

Every child who has Hurler is in a fight for his life, and every Hurler parent must, at some point, lay her child's life at the feet of powers greater than a parent's love, and trust that whatever is on the other side of the struggle is right and good, and is the way it was always meant to be.

Liam Larrow, one of the children we're following, is really down on the mat today, as is his mother, who writes his story at Caring Bridge. Please keep Liam and his family in your thoughts and prayers, as he has come to a critical moment in his struggle for life. Also, if you can, take a minute to sign his guestbook, and let his family know you're pulling for him, too.

Thanks for spreading the love around. After all, that's what it's for, right?

Long time...

2008-09-22T01:11:00.004-04:00

no post. I am such a slacker.

Gracie is doing great and just being her cute little self most of the time. She's recently learned how to throw a tantrum when she doesn't get what she wants, but instead of being frustrated, I thank God for the opportunity to go through something normal like everyone else for a change. :)

Gracie's eating process is slowly but surely coming along. She's eating more and more each day and she's definitely experimental when it comes to food. She still turns her nose up at traditional baby foods. Her preference is to eat what I'm eating from my plate. Of course, with only one tooth that can prove to be a bit difficult, but she makes it work. Because she's be maintaining her weight over the past few weeks, they've taken the fats out of her TPN again. Hopefully we can shovel enough food into her to begin to reduce it in the coming weeks and get off of TPN altogether. Woohoo!

Also, Gracie is crawling more and more as she gains her confidence. Its obviously still a bit of struggle for her, but like the trooper she is, she keeps on trucking. She likes to walk around in her walker and also handing onto our fingers, and she has turned into a dancing queen! She bops and bounces at every opportunity and steals the heart of every onlooker. She's also pulling up on anything that stands still these days. I have a feeling that she'll be walking pretty soon and have even better opportunities to get into trouble.

We have an appointment this week to evaluate Gracie for her speech and physical therapies. I'm looking forward to seeing the progress she's made since we left Minnesota. I'm just so proud of her for being so strong considering all that she's been through. Our MN therapists Mary and Susan would do cartwheels if they saw everything the Gracie is up to these days. :) She never ceases to amaze me.

Gracie's hair on her head is coming back in full force, and much to my delight- its coming in dark. So I will again have my little blue eyed brunette to groom. She's also getting pretty furry on her arms, legs, back, and hiney. It's a side effect of her anti-rejection med, but I think its so cute. I'm just so glad she's not at an age where she'd be emabarrassed by it. Its yet another reminder for me of the amazing miracle that is occuring within her.

Other than that, all is well. We're still going to clinic in Jacksonville more often than we'd like, but hopefully we can get her medicine levels straightened out and train our new nurses to draw Gracie's blood very gently (to avoid hemolysis - bursting of the blood cells- which causes falsely elevated potassium levels) and just have to go once very 2 weeks like we initially planned.

The countdown is on for our return to Minnesota during the first week of November. We're getting so excited to return and see our beloved team of docs and nurses. We miss them so much! Also, we're hoping to run into our special friend Rylie and her family at their 6-month checkup too. I'm hoping for a great reunion with lots of good news all around.

I'm also looking forward to the cool weather, seeing the wonderful folks at the clinic, hospital and RMH, and a trip to Raising Canes- for some yummy fried chicken. Who'd have thought that a true southern girl like me would crave midwestern fried chicken???? Funny, isn't it? I will say that it is the ONLY place I know of that serves sweet tea in Minneapolis!

And now, a few pictures of Gracie at Aunt Misti's wedding yesterday! Gracie partied hard... and late into the night. I think she spent more time dancing than any other guest! Enjoy!

Prayer request...

2008-09-15T23:07:00.002-04:00

Ok, to all of our loyal prayers out there. Thank you all so much for your continued prayers for Gracie. Keep them coming, she is doing great.

I have a special request for our personal praying posse, would you guys please lift up our new Hurler friend Bella and her parents- Elizabeth and Charles- in your prayers as well. Bella is 15 months old, lives in central Florida, and was diagnosed last month. Right now her parents are battling their insurance company to cover her ERT treatments. Please pray that aresolution comes quickly and that Bella can begin treatment. As many of you know, every moment counts with this disease and they need to get started ASAP.

If you'd like to know more about cute little Bella, she is in our MPS links list on the right hand side of this page.

Thanks all.

News!

2008-09-07T23:06:00.003-04:00

Gracie is growing up!

Just last week she cut her first tooth. I totally forgot to blog about it. (yes- it seems late, but Hurler kids get teeth a lot later than most... we were expecting to wait a few more months yet!) Its so cute. I wiggle my (clean) finger in her face and say, "you gimme that toof!" and she opens wide for me to feel it. What a silly little peanut.

Also, she's crawling. Not much, but enough to get her where she wants to be... which is usually on Mommy. This is a huge step because Hurler children also have less range of motion in their joints than most other kids, so crawling (which utilizes the shoulder joints a LOT) can be problematic or even painful. So while she's not really what I would call "mobile", she is crawling short distances and it seems to not hurt her at all.

Way to go Gracie!!!!

Sorry for the delay in updating...

2008-09-06T23:32:00.002-04:00

Surgery went well. All of the docs told us that Gracie did great and it seems that she did. When they brought us to her in the recovery area, she was being snuggled by a very nice nurse. But being her mommy, I used the mommy trump card and took over the rest of the snuggling duties.

All in all, a very good experience with the docs at Wolfon Children's Hospital. The only thing is that they don't use Hickman catheters. So Gracie got a Broviac Catheter instead. Its basically the same as the old Hickman line, but with a few differences. One thing I don't like is the fact that the lines are opaque. Before if some blood backed up into Gracie's lines, I could see it and knew to flush it out. With the Broviac, I can't see anything at all. However, I do like that it seems to be a little more flexible than the Hickman lines and won't kink as easily.

All in all, I'm ok with the change so far. They had to make a new incision, so they put the line on the opposite side of her chest than the previous ones. It stinks because her little chest has all of these little scars on it now. :( Oh well, they're small and she's small so they'll probably become unnoticeable as she grows up. I'm just hoping that this is the last line she'll ever have to get.

She's been a little crabby and clingy since the surgery, and she's obviously in a bit of pain. So we're trying to keep her Tylenol on schedule so that she doesn't get too uncomfortable. So she's a little bit of a bear but still a total sweetie, of course. Little snugglebunny.

xoxox

okee dokee

2008-09-05T05:32:00.001-04:00

We are heading over to Jax for Gracie's surgery now. Its outpatient.... so we don't have to stay overnight. Say a prayer at 9:15 - thats when she's scheduled to go in.

xoxox

Wow...

2008-09-03T00:11:00.003-04:00

What a wild couple of weeks we have had since we have been home. Its been great, but busy. And I'm sorry for not updating the blog in so long.

So here goes...

After a few days of settling in here at home, we traveled over to Jacksonville to meet our new doctor, who will be watching over us here in FL. We are going to Nemours Children's Clinics and our doc's name is Dr. Joyce and he is the head of the Pediatric Transplant division of Nemours. He seems to be a very nice man, and a thorough and caring physician. Also worth noting, he has no problem working closely with our physicians in Minnesota, and has actually follwed a few other children who have gone through transplant there. We also have a primary care nurse, Jodi, who we like very much as well. Good stuff. Well, except that we ended up in clinic a total of 3 times last week, and again today. More on that later.

Also, now that Gracie is back home, it seems that she has decided that she is hungry. WOOOHOOOO!!!! Our church family has been taking turns making dinners for us since we got back, and one night we were enjoying a particularly delicious potato soup while Gracie was muching on her usual cheetos. She finished her cheetos and looked at me with great interest while I was chowing down on this yummy soup. So I held my spoon out to her and she opened her mouth. I was shocked! I dripped a few drops onto her mouth and waited for her usual gagging spell and none came... she just looked at my spoon again. She ate and ate until I finally stopped her because I was afraid she would make herself sick! After a round of applause and a few big hugs, I ordered my family to "STEP AWAY FROM THE SOUP!" I put it away and declared it for Gracie's tummy only. :) That was just the beginning. Since then, she has devoured lima bean puree, mashed sweet potato patties, all of the leftover soup, mashed potatoes, pureed green beans, waffles, and anything else that she sees us eating. The thing I have noticed most is that she doesn't seem to like the baby food versions of these foods at all, she only likes the grown-up stuff- fully flavored and then mashed or pureed. Its been a wonderful change. She is now eating 3 times a day and having cheetos as a snack instead of a meal! Our dietician had us introduce formula as well, and we're hoping that with Gracie's growing appetite and the additional nutrition in the formula, we might be able to get off of the TPN within the next 2 weeks! Wouldn't that be wonderful???? Here's hoping.

My parents threw us a lovely welcome home party on Sunday afternoon and it was a lot of fun. Gracie charmed all of her guests with her big smiles behind her little mask and then conked out for a long afternoon nap and managed to miss the rest of the party. All of the adults had a great time celebrating her homecoming anyway. :) I hope to have some pictures from that to show next time.

Ok, onto the more problematic issues.
When we made it to the doc last week and had Gracie's labs drawn, her CSA (anti-rejection drug) levels were REALLY high. More than twice the amount they should be. So we had to hold her meds for a full 36 hours and then return to have the level drawn again the next day. The second draw showed a normal reading, and so we adjusted her dosage down quite a bit. According to our nurse, this is a common occurance for kids who are on this kind of med for an extended period of time.... they just start metabolizing it differently. But it made me very nervous knowing that she had such a high amount of such a strong drug coursing through her little body for a while. Yikes.
Then we got another call from our nurse telling us that Gracie's potassium levels were too high and that we needed to go back first thing the next morning for another draw. too much potassium could lead to heart issues that we don't want to be dealing with, so naturally we were worried. It turned out that there was an issue with the method of the draw itself and some of the blood cells burst in the syringe and released some extra potassium. So our second draw showed a normal level of potassium. Whew... another close call.
Then this weekend I was changing her dressing over her central line and as soon as I removed the bandage I saw that her line was coming out. The little cuff that is supposed to stay under the skin was completely out and there was nothing left to hold the line inside. So this afternoon we made the trek over to Nemours again to have a consult with the surgical team to discuss what our next step is. It looks like Gracie will be getting her central line replaced this Friday. Bummer. I hate the idea of her having surgery again... especially after the last experience. But I'm hoping that this will be better since Gracie is much more stable than she was the last time we went through this.

I'll be sure to keep you updated as I get new info from the docs. So far we are scheduled for Friday and I have no idea if we will have to go into the hospital for any length of time at all or not.

Whew... what a long post. I'll try not to take so long to post next time and then maybe I won't have to write a book to catch everyone up to where we are. :) Hope everyone is well.

xoxox

I know, I know...

2008-09-01T19:01:00.001-04:00

I owe a big time update.

And I promise I will update tonight or tomorrow. I have so much news to share!

xoxox

Be it ever so humble....

2008-08-22T14:37:00.006-04:00

there really is no place like home.

We're here! So Miss VIP Gracie flew home in serious style via private jet on Wednesday afternoon. The flight was smooth and a short 3 hours. Gracie played and slept and watched Teletubbies and enjoyed a mask-less flight while Mommy and Daddy enjoyed plenty of legroom, large tray tables, and plush leather seats that recline all the way... wow! It was amazing. Thank you so much to our benefactors, pilots and friends who went out of their way to bring us home in such comfort. We are truly grateful for all of the care and kindnesses that made this come together for us.

As we approched the runway of Lake City Municipal Airport for landing, we were able to see a small gathering of people waving with a banner and balloons, and a fleet of Columbia County EMS ambulances with lights flashing to welcome us. At that moment my husband and I smiled together with tears in our eyes knowing that we are so blessed to have such a caring community here in Lake City. Our hearts could not have been more full than at that moment.

Gracie exited the jet and was greeted with smiles, tears, and cheers from so many of our loved ones and she, of course, smiled and showed off for this new adoring audience. :) It was a truly triumphant moment in her battle against Hurler Syndrome and I can't wait to tell her about it over and over as she grows up.

Here are a few pictures from our arrival.

Landing in our awesome ride!

Ambulances from Daddy's work with lights flashing just for us!

Pulling up to the hangar.

Here comes the Peanut!

What a bunch of crybabies! Gracie, Mommy & Daddy with Noni, Munts, and Mema. :) Gracie was the only non-crybaby.

The beautiful banner from our church family.

With our awesome pilots! These guys were amazing.

First steps on Florida Soil.

Our wonderful friends while they were waiting for us to come home.

I have so many more great pics from our arrival. I'll try to get a few more up soon.

We're settling in at home and trying hard not to let Tropical Storm Fay rain on our parade. Miss Gracie has been having fun discovering all of the "new" toys and stuff which has really been fun to watch.

She is turning into such a character.

More soon.

One Their Wa-ay-ay... Home Sweet Home!

2008-08-20T18:13:00.005-04:00

Well, Fay or no-Fay, Gracie and her parents are supposed to be on their way home as I type.

They will be arriving at Lake City Inter-continental Airport this afternoon/evening. :) I have mixed emotions... I'm so so happy for them. It's hard to imagine Sarah being away from home for 4 months! But I'm worried about the weather, and I'm headed to Minneapolis tomorrow to pick up my Jeep (it has been there all summer), and was really really hoping to give Gracie and Sarah another hug and kiss before they headed back. Oh well. Getting home sooner is best for them. I know they are nervous, leaving the exceptional care team in Minneapolis, but they have contacted a doctor at Nemours in Jax who has a little bit of experience with Hurler kids.

Give them a warm welcome. And get rid of that pesky storm. I can't stand the thought of Fay welcoming them back to FL...

Uncle Mack

Get out your rain sticks and start dancing...

2008-08-16T20:25:00.003-04:00

Take a look at this.

That's the projected path of our friend, Tropical Storm Fay. Take a look at where she is supposed to be on Wednesday afternoon.... RIGHT ON TOP OF LAKE CITY. Grrr.

I heard from our pilot this morning and he informed me that if she continues on this track, then we won't be able to come home until Friday. Booo. Of course, if thats the weather we have to look forward to at home, I'd just as soon stay here a few more days anyway. The weather here really is beautiful. I know I will really miss that aspect of Minnesota life.

So shake your rain sticks and tell Fay to go elsewhere so we can come on home.

xoxo.

A multitude of angels...

2008-08-15T00:11:00.006-04:00

will come together to fly us home on Wednesday, August 20th... only 6 more days!!!! Yes friends, you can take it from me, prayer works hand in hand with wonderful and caring friends who work tirelessly for a cause. In this case, the cause is Our Gracie, who will be flying home in safety and comfort in a private 7-passenger jet that has been offered to us, provided that we pay for the costs of fuel and the pilots. And so far, members of both our community and family have donated quite a bit to help cover those expenses. Jimmy and I are truly humbled by the outpouring of love, support, and generosity we have received since this whole process began.

Oh, I forgot to mention the best part! We will be flying home. HOME! Meaning that we will be landing in Lake City... no long car rides home! We'll be there already!

So to celebrate, a random array of Gracie pictures. Enjoy!

A bittersweet farewell...

2008-08-12T15:14:00.006-04:00

Sadly, we had to goodbye to our special friends, the Hays Family- Lyle, Jade, and little Rylie. We've mentioned Rylie several times in our blog, she has been Gracie's transplant buddy, and she also has Hurler Syndrome. She was transplanted the day before Gracie and our families have walked this bumpy road together. To see them go was both a joy and a sorrow. We are so happy that they are finally home, but we miss seeing their faces each day and sharing news and stories.

We managed to snap a few pictures of the girls together, although you can all see how sad Rylie was to be leaving her friend behind. :) We promised them that we would try to schedule our follow-up appointments together so we can watch our girls grow and thrive despite this disease they share.

Our friend Rylie

Rylie and Gracie

Aww... Gracie is consoling her sad friend.

Our new friends, Jade, Lyle and Rylie Hays

Hope you guys continue to do well at home and we plan to see you in November.

Day +92 ... Tube in, tube out x 2

2008-08-08T14:23:00.000-04:00

Ok, so we've made it to the 90's and we've had a few moments of excitement in the past few weeks.

So Gracie's eating situation has not improved enough, so this past Tuesday we decided to put an NG tube in to try and get her tummy stuff moving. Well, she got it in around 1 pm, and by 11 pm, it was out. Our girl did not like it at all. So Wednesday morning we head back to clinic with our tubeless Gracie and had them replace the tube. It seemed that she was tolerating the second tube much better, and so Wednesday night, Gracie had her first formula feeds through the tube overnight. I was delighted to see Gracie wake up on Thursday morning with a full tummy and the tube still in.

So on our blissful Thursday morning as Noni was packing to head back home, Gracie began to vomit. She vomited quite a bit of the formula she had gotten overnight, and then surprise.... she also vomited the tube. So out it came again.

It has stayed out this time, and our Doc has decided that Gracie is just not ready for it and she needs more time. So in the meantime, we will continue to try and get her to eat the regular way and we'll revisit this issue sometime next week.

Gracie ate the equivalent of a whole cheeto at dinner tonight, so at least I can say that she's trying. :)

We're getting closer and closer to coming home and I am so excited. Although I must admit that I will miss Minnesota... and especially the gorgeous summer weather. I laugh everytime the humidity climbs to 60% here and everyone starts complaining about how muggy and sticky it is. Too funny.

xoxo

Day +86 - uphill battles

2008-08-02T15:11:00.004-04:00

So I want to thank everyone for taking up our cause and trying to help us find travel home. We're still praying that we'll have something private come through for us by early next week. If not, we'll look into a non-stop commercial flight and hopefully we can get upgraded to first class so that Gracie can be slightly more comfortable. We'll just see how it goes. Again we really appreciate everyone's efforts in trying to find us a better option. Its so heartwarming to know that Gracie is so loved by so many (us too!).

So on the Gracie front, we're still fighting the eating battle. She's definately more open to eating and she tries really hard, but the nausea is really tough to overcome. Our doctors ordered the fats to be taken our of her TPN last week and it seems to have improved her appetite a little, but she still isn't eating enough. We've decided to try a different tactic today and attempt to keep something in her belly all day. Maybe if her belly isn't completely empty, the food will stay down better. Here's hoping anyway. We go back for a weight check on Monday. If she's lost too much weight, then it will be time to discuss other options. :( I'll let you all know how it goes.

Thank you for all of your continued support and prayers. We're really looking forward to getting home soon and seeing you all again.

So far...

2008-07-30T13:27:00.003-04:00

our efforts to find travel via Angel Flight and other such charitable organizations has not been going well. Most of the Angel services only provide travel for up to 1000 miles. We are almost 1500 miles from home. Also, the other patient travel service organizations say that they are unable to help us because they don't provide one-way travel. They could have possibly helped with roundtrip travel, but not just to travel home. Its a bit discouraging.

In the meantime, I am starting to contact the airlines who have programs to help with travel for sick kids ( we'd still have to fly on a regular plane, but they help with expenses), but those resources have been taking a hit due to the financial status of most of the airlines right now. We're hopeful that something still might come through for us... you never know.

Besides that, Gracie is still doing great. Our main struggle is still with the eating issue, but she is really trying. They took the fats out of her TPN hoping that it might stimulate her appetite, but she's still not taking in enough food to sustain herself. We are continuing to work on it though and Gracie is truly blooming despite the difficulty. I am really so proud of her. :)

Day +80... in the home stretch- literally!

2008-07-27T23:10:00.000-04:00

So, I can safely say that provided that we have no major medical events, Gracie and I will return home sometime around August 20th or soon after. Our final appointments will occur on August 18th and consists of the following: An MRI, X-ray, and Lumbar Puncture (Spinal Tap) under sedation, followed by a general checkup at the BMT clinic and then a visit with our Neurologist, Dr. Charnas.

So between now and then our prevailing dilemma is to figure out how to get home. Obviously we need to fly, however Gracie's fragile immune system makes us a little wary of flying a commercial airline. We all know how germy those planes can be! And I can't even bear to think about making Gracie wear her mask through a long flight and possibly a layover and then another flight. Ugh, poor baby. So, right now I am in the middle of contacting the many Angel and Mercy flight organizations to see if they can help us. They network with volunteer pilots with small aircraft and also large corporations with private jets and try to match them up with patients in need. We're really hoping that this will work out for us, so send out some prayers for an angel to swoop in and bring us safely home.

Gracie is still doing great and working hard trying to eat and with her physical therapy. She's definately making progress in both areas but its slow going. I am so proud of her for being such a trooper, even when she just doesn't feel like it. She is definitely an amazing little girl. So we'll just soldier on with the feeding, meds and physical activites and hopefully Gracie will continue to improve.

Much love.

Day +77 PICS!

2008-07-24T10:20:00.006-04:00

Gracie continues to do well, have good counts, and has become increasingly more playful and active. All in all, she is doing well. Right now our challenge is eating, and its a tough one. She is really trying though, despite the nausea and what I percieve to be a lack of appetite. She likes yogurt and cheetos a lot! And she surprised us yesterday by finally drinking from a sippy cup! This is a huge accomplishment and a big step in the right direction. It means she is not only allowing the tip into her mouth, but she is sucking too. YAY!!!!

She's also getting a little hairy from the CSA (medicine she takes to prevent GVHD), so her eyebrows are getting a little bushy and growing together some... and right up her forehead. :)

Here are a few pics I snapped the other day while Gracie was trying to cruise (she's almost there!)... I think they turned out so cute. I hope you like them too.

Day + 70-something. No News is Good News

2008-07-20T23:27:00.005-04:00

Hi everyone. Sorry we have been so sporadic in our postings. I assure you that no news is good news. Gracie is doing extremely well. She's doing a great job choking down all the meds. She vomits and gags some, but all in all she's a trooper. It's tough on mommy too since she her life has been transformed into a human medication vending machine. All she has time to do is administer medications.... But mommy is a trooper too and is developing a routine and hanging in there...

Her docs are all very happy with her progress. Her numbers all all still very good, and as you know - she is actually making enzyme on her own. So long as we can keep her healthy, the transplant will be a total success! Yay!

Gracie has been getting out quite a bit, trying to enjoy the fantastic summer weather in Minneapolis.. She goes for walks, has gone out to dinner a few times... She loves it. She just wears her little mask and plays and seems to have fun. Such a great kid!

Great news is that Gracie is starting to show signs that she wants to eat regular foods. Apparently, the transplant messes with their taste buds, so the docs recommended we try to give her adult food with stronger flavors (baby food is too bland). She sucked on a piece of pasta, ate part of a cheeto, and ate a french fry... I suppose we'll have to work on her healthy diet later. For now we just want her to start eating. The sooner she starts eating food, the sooner she can go home.

Gracie is also talking a lot more, and starting to make progress toward getting around independently... her speech and physical therapists are very pleased. :)

Anyway, just a quick update to keep you in the loop. So sorry for our delinquency.

Uncle Mack

Bad Mommy, Good News

2008-07-15T10:36:00.008-04:00

So, the bad mommy has been a bad blogger. A week ago I promised pictures and an update. I'm sorry its been so long. :(

Everyone has left now, even Noni, and we expect Uncle Mack to come for a visit tomorrow. So Gracie and I are on our own for today. She's napping now, so I thought I'd come fulfill my overdue promise.

Luckily, I have great news to share from our clinic visit yesterday. Firstly, her counts are still great... WBC 7.5 and Neutrophils 5.3 ... all the other numbers are great as well.
Secondly, they tested her engraftment last week (although they forgot to tell me about it) so I was pleasantly surprised to learn that Gracie is still 100% engrafted with her donor's cells.
Thirdly, I finally learned that Gracie's new cells are producing alpha-L iduronidase- our missing enzyme- and plenty of it, so regarding the transplant for its original purpose, we can say for sure that it worked. WOOOOHOOOOO!!!!! Now our concerns are of course to stay engrafted, stay healthy, start eating, and combat GVHD (Graft-Versus-Host Disease).

Gracie's birthday was wonderful, and thank you all for the wonderful cards, gifts, and well-wishes we received. I consider myself so blessed to have such a wonderful support system and we definitely feel so loved even though we're far away.

Ok, so how about some pictures.... I hope these are worth the wait. :)

Gracie and Mommy at the fireworks

Gracie's birthday cake... actually it was Garcie's cake.

Gracie and Daddy snuggling

And 2 from Gracie's birthday dinner at Applebees. What a big girl!