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.
Monday, December 29, 2008
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!
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.
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.
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.
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