Happy 9 months, baby girl!

Our Gracie is 9 months old today! We are busy busy busy packing and getting everything ready to go tomorrow. We will arrive in Minnesota on Saturday armed with one headstrong baby, 2 hopeful parents, and 4 suitcases stuffed to the limit with clothes and baby toys.

Gracie continues to do great. Wednesday was our last ERT at Shands and a sad goodbye to the wonderful team who worked with us. Kathy and the rest of our beloved nurses, along with Dr. Kelly, threw us a wonderful farewell party. They had set up our little area with lots of balloons, ordered pizza from 5-Star (YUM) and showered "their" baby with tons of fun presents. They also gave us a framed picture of all of us together to take with us to Minnesota so that Gracie will still have her nurses fighting over her. What a wonderful group of people. I must say, that while the Shands team had a rocky start, they pulled it out in the end and we must give the Shands BMT Team a big thumbs up and a lot of gratitude. We're looking forward to sneaking back to visit in a few months and surprising our dear friends. :)

Gracie showing her beloved Nurse Kathy how yummy a tube of Lansinoh is!

News!

Yup thats it. We're officially in the news. The local paper will be running a story on Gracie today. So all of our local fans can open their Lake City Reporters and read about us and our journey. For our non-local fans... I think it might be on their website in a few hours. http://www.lakecityreporter.com/ Here's hoping, anyway. Tony Britt was a kind and patient reporter, and I know he'll do Gracie's story well. I can't wait to wake up and read it.

On that note, its off to bed for Mommy who has a big day tomorrow of packing and finalizing everything.

xoxox

ERT #7

Our 7th ERT was Wednesday and I must say, it was the best one so far. No mishaps at all, and the process went smoothly and much faster. We were really impressed. Gracie again did beautifully and she actually seemed to enjoy her dressing change. She stayed still and quiet through the entire process!

So despite our early experiences, it seems that Shands is finally catching up to our expectations. We had a nice chat with Dr. Kelly about Gracie's cord blood matches and upcoming transplant, and she is very confident that Gracie will do well. She is still very healthy and showing lots of improvement developmentally. She even brought Gracie a sweet teddy bear to play with. :)

Developmentally, Gracie is doing great. We're finally hearing some structured babbling- despite having no news from Early Intervention about speech therapy. Grrrr - I need to call them today. She's also sitting up on her own, playing with 2 toys at the same time, and trying to crawl and clap. We are so proud of her! She's still loving trying new foods... she's really taken a liking to papaya these days!

Anyway, we're slowly trying to gear up for our trip back to MN. I am excited to get going with Gracie's transplant while she's doing so well, but I must admit, I am not looking forward to being away for so long. :( I am going to miss our daily life here so much- our friends, family, and especially our church family are so important to us. I know that we will be well taken care of in MN, but as everyone knows, there's just no place like home.

8 months old and ERT #5

So Gracie turned 8 months yesterday and we had a pretty uneventful day. We did visit the world of Walls (aka Walmart) and found that our big girl LOVES to ride around in the cart. She was happy as a little clam in her buggy bag and she picked out a few new toys to take along with us to ERT today.

Speaking of ERT, it went well today. No major mishaps to report and Gracie was really great! She slept a lot and played some and even dozed off during her dressing change, which was a really pleasant change from her usual screaming fits during that lovely activity. She even let her favorite nurse Kathy hold her for a few seconds right before we left for our weekly retail therapy trip to Target. :)

We met up with friends Jenny, Brandon and Olivia in Target and had a nice dinner with them at Hops, where our Miss Gracie ate 2 containers of food- some pears with blueberry and of course, our beloved sweet potatoes. Boy, was she a hungry little monkey after our long day!

As much as I wish I could say that we have a nice leisurely day tomorrow, we have to be up bright and early in the morning to meet with the Early Intervention Speech Pathologist at 9. When they called to set up the appointment, I must admit that they didn't really instill much confidence in me regarding this particular therapy. The lady who called basically told me that their Speech Therapists don't really work with babies as young as Gracie and they wanted to know why we're even being referred to therapy at all. Grrr. Well, I'll be bringing a copy of the report from Dr. Bjoraker, the Neuropsychologist in Minnesota who is recommending the immediate therapy. This is yet another moment where I wish we were back at Fairview with doctors who really understand Gracie's condition. Its so frustrating to have to try and explain what her disease actually does to all these docs who have never heard of it. I guess I have to get used to that though, because as long as I have my Gracie, I will have to explain her condition to people. And thats definately better than the alternative so I need to stop my complaining. :)

My girl just seems to be doing better and better as each day passes. I am so pleased with how everything is going and I am so proud of my tough little trooper who is taking everything on like the champ she is. Her daddy and I could not be happier with her progress and we're looking forward to moving on with the next phase of treatment.

So to all of our readers, we ask that you all say a prayer for our friend Chance who will be having his Transplant Day in Minnesota tomorrow! Go Chance! We're looking forward to meeting Chance and his family when we return for Gracie's workup... he should be checking out of the BMT unit when we get back up there if all goes well for him. Chance and family- we're thinking of you guys often and sending lots of prayers and good thoughts for you.

Much love.

ERT #4

...went much more smoothly as far as the delivery of the Enzyme and such. We were pleased to make it out of Shands an hour earlier than usual and thats great considering that we got there an hour late too! We might have been out even earlier, but there were a few hiccups caused by Mommy that slowed things down a bit at times.
First, when giving Gracie the benadryl, I lost control of the syringe and squirted a bunch her mouth at once :( which she promptly spit right out. So, with part of her medicineon her shirt, we had to stop and check with Dr. Kelly to see if we needed to get more Benadryl or if we could just keep going without that little bit. It turned out to be fine but I felt terrible for not only delaying the treatment, but also doing that to Gracie and making her gag. :( I was so sad.
Then later in the day we had another little hiccup, although this one managed to almost give Mommy a heart attack. Gracie was still getting the enzyme and she was antsy. So she was playing on my lap for a few minutes and then decided that she wanted to feed. So I got her all latched on she starts to eat. After a few minutes she pulls off to smile at me :) and I notice that there's blood on the front of her outfit. I pull her away from me further and I see more blood. Well, I freaked, Jimmy turned off the drip and clamped her line, and then we put her on the bed and Kathy ran in. Turns out that we managed to disconnect the drip line from Gracie's central line, so the blood was coming from her open central line. Well, I am panicking at the blood all over her and me and then Jimmy and Kathy just smile at me and giggle because what looks like a lot of blood is actually only 5-10 ML... less than what they take from her as waste before her blood draws. I actually got some praise for catching the bleeding so fast... it didn't waste enzyme or anything. Woot!

I still felt terrible though. :(

We made it through the rest of the afternoon with no more problems and the next thing I knew, it was time to go home. Yay.
We met some friends for dinner when we got here and had a very nice evening. Gracie ate an entire bowl of sweet potatoes and charmed everyone as always. What a sweetie.

ERT #4 today

...and I'm a bit worried. Gracie has had a cold for a week now and she sounds terrible. She's congested and stopped up and has this terrible hacking phlegmy cough. Its yucky. :( I hope this doesn't effect or delay her treatment today. I must admit, its a bit scary to hear her breathing be so noisy like this and those awful coughs. Breaks my heart.

ERT effects thus far

So I must say, I have gotten so many comments from family and friends that Gracie seems to be a much happier baby, and that she even looks a bit better since she has been on ERT... and even from week to week.

I have to agree. Gracie has been much less fussy in general and seems to be happier and easier to entertain these days. I absolutely love it.

She still maintains a great appetite - sweet potatoes are her very favorite food, and she loves to play with her toys and in her jumpy chair. Its wonderful! Now if I could get her to go to bed a bit earlier, all would be right with the world. ;) I am definately looking forward to seeing how she progresses after more ERT treatments.

Eileen, our ERT nurse from Minnesota called yesterday to check in. Nice chat. They're still planning on having us back on April 14th, and I must admit, I am looking forward to it. I really miss the people there and of course, the great medical care.

Still waiting to hear from Early Intervention regarding Gracie's speech therapy. At this rate, we'll be back in Minnesota before it even gets started. Grrr.

Anyway, I just wanted to check in with everyone and update you on Gracie's progress. I am really happy to see her blooming.

Here's a cute pic of our princess from last week. Sorry for the blurry quality, it was from my camera phone. :)

Ok... so I know

its been too long since our last update.

Wednesday was ERT infusion #3 at Shands.

Unfotunately, we only clipped our original time by about 30 minutes. It seems that despite our attempt to speed up our day at Shands, the people in the Pharmacy department suceeded in slowing us down a lot. That poses a problem with the enzyme. To put it simply, once the enzyme is mixed, it is supposed to be delivered immediately in a 4 hour time frame. Well, it takes precisely 4 hours to infuse it into her once we start the drip. The Pharmacy department did not get the stuff to us until an hour after it was mixed. Not cool.

Out fabulous nurse Kathy (who Gracie has taken a liking to as well :) ) has promised to rectify this situation for our future visits... she has made it her mission, even if that means that she must go to Pharmacy and pick it up herself. Thanks Kathy.

Other than that, it was a good day. Gracie did well and enjoyed some snoozing and playing and watching cartoons and trashy talk shows throughout the day. No reactions so far and her skin is still a little itchy under the bandage, but we're learning to live with that.

So far so good and still on track for our return to Minnesota on April 14th for work-up.

More later, I promise.

Thanks for reading.

ERT #2 - Shands at the University of Florida

So Gracie had her second ERT treatment yesterday, and true to Gracie-style, she did beautifully. On the downside, it was an incredibly long day. :(
We got to Shands at 8 am but we were not called back for anything until 9:45. Yuck. They got Gracie's weight (she's over 19 lbs now! Wowza!) and her BP and temp before they shuffled us down to a room for a once-over exam. More waiting. While we expected to see Dr. Kelly at this point, a PA came in and gave Gracie a looksie before moving us to the Infusion Room. This is a large room with a bunch of big pink chairs where everyone and their brother are getting infused with blood, platelets, drugs... you name it. You'd better believe that each time some else walked in to have their own treatments, they warily eyed us and our potential disturbance.

We met our nurse there, Kathy, who was a doll. We loved her from the start. At that point she was able to call down to Pharmacy and let them know that we had arrived. Apparently, our treatment is so expensive that the Pharmacy folks won't even touch it until it is confirmed that we're on site. Craziness! So crazy in fact that they called the nurse back AGAIN and asked her if she could physically see us as a second confirmation before they would begin mixing the enzyme. Geez, paranoid much?
We were given our place in the infusion room and they finally started prepping Gracie for the treatment. A dose of tylenol orally and then Benadryl by IV. This took forever! 45 minutes to give her the Benadryl. :( Then they follwed up with a dose of Hydrocortizone to ward off any rash.
Finally at 12:30 pm they started the Enzyme drip. It was not until after that was going for about 30 minutes did we meet Dr. Kelly. She's very nice and she was great with Gracie and very sweet so we played a bit and settled in for our long treatment. Gracie snoozed in my arms for about 2 hours while we chatted first with our wonderful nurse and then with a really nice doctor who is doing a clinical study on Prostate Cancer, totally unrelated to Gracie's treatment, she just wanted to hear our story. They were both moved to tears after hearing of our path to diagnosis and the persistence of our beloved Pediatrician, Dr. Cortes. :) Warm fuzzies all around.

At about 3 pm, Gracie woke up and they brought a crib in so we could lay her down so she could play. She did... she played and smiled and babbled her way through the next couple of hours with little complaint. By this time, the room had completely cleared out and the clock is inching toward 5 pm. We saw Dr. Kelly again and she and Gracie had a few minutes of smiling and peek-a-boo before she bid us goodbye for the day. Kathy (our nurse) and Ginny (another Infusion nurse) then came over to change Gracie's dressing. When the original dressing came off, poor Gracie's skin was all rashy and inflamed. Thank goodness it was only in reaction to the bandage and not the Enzyme treatment. They decided to give her a different dressing that should cut down on the irritation and they put it all back together, flushed her lines, and finally sent us on our way at 5:45. Phew.

On to Target for Mommy's retail therapy and then a quick swing by Great Mema's house to pick up a delicious roast for dinner! Yummy! Thanks Mema! We fell into bed around 11- totally beat.

So while things were slow to start and that was quite frustrating, we were generally okay with our treatment at Shands. Kathy was the total bomb and I am happy to say that she will be our Infusion nurse every week. Yay! She also has got a plan in place to speed up our start times for future treatments so that hopefully things will go much more quickly as the weeks go on. Go Kathy!

So all in all, the Shands treatment went fine.

Report Card
Gracie - A+
Shands - B-

Shands needs to thank their lucky stars for Kathy who was great for pulling up their average. I plan to write a letter to her supervisor regarding the great care she has given us.

Anyway, to sum up- long but not too shabby. On the agenda for today- a visit with Dr. Cortes! Wooohoo!

Two More Cents

Here, now! Let me jump on the Shands Stinks Soap Box too, for a minute. You know I can never leave well-enough said alone.


It has been put forth in discussion that the Shands team may expect to get a "by" on blowing the coordination of Gracie's treatment, as they have taken on the management of her ERT as a "professional courtesy" to their colleagues at Fairview, who are going to pull down the lion's share of the cash from this cow by having scored the transplant itself. That idea makes my heart sink.


ERT may be a simple procedure, but it is no shave-and-a-haircut routine. After all, HSCT is also a simple procedure. The chemotherapy to prepare the patient is not revolutionary, either. It's the surrounding care: attention to the patient's inherent and precipitant conditions, and anticipating her evolving needs, that determines the difference between great care and substandard care. Someone who is experienced with Hurler patients and their families, and we are assured that this is what we are getting, would be hip to the reality of Gracie's parents' experience, their sense of urgency, and the degree to which they have become absolute hostages to the scheduling of Gracie's health care matrix.

This kind of ham-handedness is, I think, a symptom of a carelessness that, even in small things, cannot and should not be tolerated when the stakes are this high. It makes one wonder what other, less obvious but more dangerous slips might occur because something just didn't get done. It's an old and often sad tale. Little mistakes can cause big problems for vulnerable patients. Someone on this team really should acknowledge this openly to Gracie's family.

They really should.

Hiccup #1- Mommy's perspective

While its probably not far off from the overall tone of Uncle Mackie's post, I feel the need to post here about this issue with Shands.

When I got off the phone with them yesterday regarding this issue, I looked at my daughter and said, "Gracie, we're not in Minnesota anymore." :(

This is exactly what I was talking about a few post ago when I said I was anxious to leave Minnesota for the next 2 months. As I have told many people since I have been home, I have never experienced the level of care that we recieved at U of MN in my life. Most of my special doctor stuff for myself and my loved ones has been at Shands and the treatment they give seems to have always been the same. I never thought to think of it as substandard... until now.

It angered me so much that Gracie's treatment was so carelessly juggled around by these people. The truth is that its not that hard of a treatment to do. They mix up the enzyme meds, put them in an IV bag, hook it to Gracie's central line, start it, and then monitor her vitals for several hours as she recieves the meds. Really not a tough job. But they will charge our insurance out the yin-yang for it and get paid quite a bit to do this. So I know money is not the issue. Its just carelessness. While my daughter is the top priority and single most important thing in my life, they made it painfully clear that she is not a priority in theirs.

Whats funny is that I never once felt that way in Minnesota. As a matter of fact I often wondered if they had any other patients besides Gracie. :)

My intention is not to have a Shands-bashing party here, its more to show that we don't have to accept this kind of treatment as status quo. I know what healthcare is supposed to be like now- and for my precious daughter, I will not settle for anything less.

I hope that all of our adoring public does the same for themselves.

And to end this post on a good note....


Thats the Peanut enjoying dinner at a nice little Irish Pub we found near the U of MN. We actually were trying to get Italian that night, but this place ended up being pretty yummy for the grownups. Don't worry about Gracie being left out, I think she had sweet potatoes that night. Yum Yum!

Hiccup #1 - Shands Drops the Ball

Well, our first hiccup related to treatment comes to us courtesy of Shands. True to form, they are working hard to prove me right, that they don't always put the needs of their patients first.

Tonight I learned that Shands didn't order Gracie's enzymes early enough to have them delivered for her treatment tomorrow. Sarah had to reschedule for Thursday. Sure it's not the end of the world, but it is a big hassle for Sarah, as she arranged her schedule to take an entire day to travel to Gainesville for this full-day treatment, shuffling other appointments since this is obviously her top priority.

Frankly, it pisses me off. They were given plenty of notice, and holiday or not, should have been prepared. What really burns me is that they don't apologize, take any responsibility for the problem, and make light of it pushing the burden to my sister - who, if you haven't noticed, has enough of her own challenges to deal with. It's amazing how mis-directed some physicians' blame can be (overt or covert), and the fact that they don't recognize the implications.

A simple apology can do wonders for your patient's/ client's trust in and dedication to you, not to mention loyalty, referrals, etc. It takes me back to my posting about ownership of the patient-physician relationship. You are not God, you are a doctor. Finding one who shows a little respect (even if that means dropping the one you have) is a lot better use of time than dealing with someone so willing to waste yours.

My take is this - tell them what you think, make sure that they understand that you need to know that you are their top priority, make sure they know that you need to know that you are their top priority again, and then explore other options to see if anyone else in interested in working with a dedicated family and a rare, interesting, extremely profitable, and challenging patient/condition where the clinical team is really making a difference and improving someone's life. There are plenty of fantastic pediatric specialists in Jacksonville. I don't think it would be too hard to find a replacement. Do you?

Apologies for venting. Clearly missing little Gracie. :(

ERT Week One - We Made It

Gracie did really well today with the ERT treatment. She didn't have any negative reactions to the enzyme and the nurses thought she did very well.

The building today was really old and sort of gross. It seems they put the research team in the worst areas. The great thing - we had our own nurse, who didn't have any other patients, which was fantastic. Again, we were very happy with the quality of care at Univ of MN.

Keeping Her Clean and Healthy

Gracie has a few post-surgical dressings from her procedures yesterday. There is a small wound where they inserted the central line.

Her mommy is learning how to change her dressings and keep the environment sterile so the central line remains clean. Mommy is very nervous about the lines, about her ability to keep them clean, and about changing the dressings.

There is one big favor that I'll ask of all of you: Her mommy doesn't know I'm doing this, but do your best to avoid the temptation to ask to hold Gracie. Due to the nature of central lines and the implications of any contamination and blood stream infection, I'm sure her mommy will appreciate having few hands on her. Don't get me wrong, Gracie will be perfectly healthy while at home. Her immune system is fine. I'm just taking a "better safe than sorry" route and protecting the lines that lead directly to her circulatory system. Uncle Noodles is a bit of a worry wart!

Gracie still loves kisses, but fewer hands are better. :) If you do hold her for any reason (or as a favor for mommy), please wash your hands well in warm soapy water. A good general rule of thumb for handwashing is to sing happy birthday to your hands while washing. At the end of the song, you're clean, and your hands feel special because they think it's their birthday! :)

First ERT Infusion Going Well

Thanks again for all of the guestbook activities. It's wonderful to know that so many folks are following Gracie's progress and sending us warm thoughts. Our hearts are warm, despite the freezing temperatures.

Gracie is in ERT right now. She's doing great! Daddy's training is really coming in handy, as he understands the lines and ports and dressings, etc. He and the nurse are getting along famously, and he's helping out the nursing staff when they need an extra pair of hands. Gracie is lucky to have him, and his training should make us all ease or worries as they head back to FL tomorrow.

I stepped out for lunch and to post an update or two. She started at 10:00 AM. Like I mentioned before, they start slow; 2 (unknown units, maybe milliliters?) per hour, and assuming she does OK, the double the dose every 15 minutes until she s at 32 per hour. She's been cruising at 32 for a while now, and has about 90 minutes of active infusion left, and then an additional hour of observation.

She has been a trooper so far. Her body seems to be tolerating the ERT very well. We are so proud of her.

Start ERT Tomorrow

Tomorrow is the big day. Gracie starts her first ERT treatment tomorrow AM at 8:30. It will take no less than 3 hours, and today we were briefed on what to expect. It doesn't sound all that bad actually. Some kids have a hives-like reaction, and very few have more serious reactions like like anaphylaxis (difficulty breathing). Other kids are just fine. Risk of reaction is the same for each subsequent treatment. In fact they said that many kids don't have any reactions until the third or fourth session.

They'll give her some tylenol and benadryl before starting, in order to prevent some of these things and to generally make her more comfortable (and sleepy). The staff will check her vitals every 15 minutes throughout the entire process to make sure she is doing ok. They start slow and crank it up after they see how she reacts.

So, that's what Gracie will be up to tomorrow. She begins the road to recovery, in earnest.

PS - I just saw the funniest Dairy Queen commercial. It was a valentine's day commercial with an older couple celebrating their 50th anniversary. They are sitting on a couch you'd find in my Grandma's house in the 70's, talking to the camera.

Wife: It's our 50th wedding anniversary.
Husband: WHAT?
Wife: IT'S OUR 50TH WEDDING ANNIVERSARY

Wife: Each year we celebrate by going to Dairy Queen and getting a cake
Husband: WHAT! YOU WANT ME TO KISS YOUR BROTHER, JAKE?
Wife: YES! I WANT YOU TO KISS MY BROTHER JAKE!!
Husband: ABSOLUTELY NOT! OUT OF THE QUESTION!

Wife turns to the camera and giggles...

Anyway, I thought it was so cute and it reminded me of my parents. Dad, you are almost this bad!