Monday, December 29, 2008

Despite the Grinchpox, we had a great Christmas!

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.

Friday, December 26, 2008

The Pox that Stole Christmas

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!

Monday, December 15, 2008

Good appointments today...

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.

Friday, December 5, 2008

Please forgive the lack of updates...

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

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

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

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

With much love and joy.

Friday, November 28, 2008

Dear beloved friends and family,

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.

Thursday, November 20, 2008

So far so good- nothing to report...

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.



Friday, November 14, 2008

The results are in...

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.

Monday, November 10, 2008

Gracie steals the show!

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!

Sunday, November 9, 2008

Busy Busy Busy...

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.

Thursday, November 6, 2008

Safe and sound on our old stomping grounds...

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.

Monday, November 3, 2008

Up late- packing and posting...

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

Sunday, October 26, 2008

I must share a letter I sent...

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...

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.

Monday, October 20, 2008

Reflections...

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.

Tuesday, October 14, 2008

Back home

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. :)

Monday, October 13, 2008

Quick Update - Still Waiting

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.

Sunday, October 12, 2008

What is gram-positive bacillus?

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...

Saturday, October 11, 2008

Hospital update

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

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.

Friday, October 10, 2008

Back in the hospital

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...

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.





Monday, October 6, 2008

No news = Good news

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

Saturday, September 27, 2008

yesterday's appointment

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.

Friday, September 26, 2008

The good news...

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

Thursday, September 25, 2008

heavy hearts

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.

Wednesday, September 24, 2008

Update...

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...

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

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.

Tuesday, September 23, 2008

Sending our love to Liam Larrow

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?

Monday, September 22, 2008

Long time...

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!



Monday, September 15, 2008

Prayer request...

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.

Sunday, September 7, 2008

News!

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!!!!

Saturday, September 6, 2008

Sorry for the delay in updating...

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

Friday, September 5, 2008

okee dokee

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

Wednesday, September 3, 2008

Wow...

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

Monday, September 1, 2008

I know, I know...

I owe a big time update.


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

xoxox

Friday, August 22, 2008

Be it ever so humble....

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.

Wednesday, August 20, 2008

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

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


Saturday, August 16, 2008

Get out your rain sticks and start dancing...

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.

Friday, August 15, 2008

A multitude of angels...

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!






Tuesday, August 12, 2008

A bittersweet farewell...

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.

Friday, August 8, 2008

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

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

Saturday, August 2, 2008

Day +86 - uphill battles

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.

Wednesday, July 30, 2008

So far...

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. :)

Sunday, July 27, 2008

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

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.

Thursday, July 24, 2008

Day +77 PICS!

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.








Sunday, July 20, 2008

Day + 70-something. No News is Good News

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

Tuesday, July 15, 2008

Bad Mommy, Good News

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!


Monday, July 7, 2008

Yikes...

Has it been so long since I've posted??? Oops... sorry to our loyal fans for the long delay in updating. So, since last week, we have been very very busy. We still have all of our family here, although Aunt Hyster and her crew will be leaving us tomorrow.

We have had a wonderful week of celebration with our wonderful family. Once everyone got here, we planned a cookout in the courtyard here with another family that we have become close to. Nuvy made a special friend with their little girl, and those 2 have played the day away for a few days... and then parted with tears when Nuvy ad to go back to the hotel for the night.

We had a birthday celebration for Gracie and all of the rest of us who have late June/early July birthdays and we had a yummy cake from Ben and Jerry's.... mmmmmm. Our new friends here gave Gracie some wonderful gifts that she has been playing with non-stop... along with the gifts that Mema brought with her from Jimmy's family.

Mommy has been indulging in some much needed sleep while Daddy is here to pick up the slack. Mommy even had an outing with the Kelly crew yesterday to the Mall of America while Daddy and Mema stayed at the RMH with Miss Gracie, who sadly is not allowed to go to the MOA for a while. Uncle Mack, Mommy and Hyster all rode on the Spongebob Rollercoaster in the amusement park and then we all took turns riding with Nuvy on the kiddie rides. Lucky me, I got to ride with her on the Blue's Clues ride.

Today we had to head back to clinic for another round of blood draws. Her counts are holding steady at 7.8 WBC, 5.5 Neutrophils, and her platelets jumped again to 203!!!! Very exciting. We have an appointment with the doctor again on Friday to recap on how we've been doing so far. I hope for another glowing report. :)

Ok, so I owe lots of pictures since it took me so long to post, so I will definately work on it and you can see how well our girl is doing.

xoxox.

Sunday, June 29, 2008

Day +52... Cleaning up for company...

Here we are, hanging out in the RMH, anxiously awaiting Munts' arrival. For those of you who don't know, Munts is my dad- Gracie's Grandpa. He will be the first of the family to arrive. This week we'll be having a LOT of visitors. First Munts, then Tuesday Uncle Mack and Aunt Hyster with Nuvy and Van will be here. Then Wednesday marks Daddy's return to Minneapolis and he'll be bringing Mema along too! Uncle Kent and Chris will be the last to arrive this weekend so that we can all celebrate Gracie's big 1st birthday! Gracie and I are so excited to host so many of our loved ones here in Minneapolis.

Tomorrow will be our last ERT, and hopefully we'll soon find out if Gracie's new cells are making the enzyme she's been missing... the whole point of having the transplant in the first place. I'm not sure when I'll find that out, but I'm going to ask about it during our long clinic visit tomorrow.

So far Gracie has continued to do very well. It turns out that she has officially weaned me from breastfeeding, but Mommy has been pumping to keep up her supply and Gracie has been having her breastmilk in a little glass. What a silly goose! She's not real interested in drinking from a bottle again or even a sippy cup yet, but I am hoping she'll be more open to the idea once her nausea subsides. Either way, I'm just glad she's taking it at all. The doctors all are encouraging me to give her breastmilk as soon as possible so that she can get all of the nutrition and antibodies it contains while her new immune system is cranking up. So far, so good. Gracie is our little champion and she is being very good at pleasing all of her doctors. Yay for my little brown-noser. :)

Gracie also returned to the realm of taking real baths this week... here's a pic or 2 of the Peanut in full splash mode. :) Enjoy.