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.

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

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.

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.

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?

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!

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.

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!!!!

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

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

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

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