Ma-Ma! Quick "Day -8" Update

Well, today is Day -8, meaning 8 more days until Gracie gets her new cells. :)

This is uncle Mack. I completely forgot to fill you all in on the speech, occupational, and physical therapy stuff! First off, how great is it that Sarah gets to learn and do all of these therapies that promote child development! She's having fun learning, and it'll be great for future babies! :)

In PT Gracie is learning to stand flat on her feet (she likes her tippy-toes), get up on her knees, and also go from a laying down to a sitting position on her own. She hasn't mastered all of these, but Sarah said she has really made progress.

When I left Minnesota, Gracie was almost clapping on her own. Now she can clap on command. She definitely understands what "no" means, and she has started to smile and shake her head "no" with a big grin on her face when mommy says no... Charming her mother to get her way. I have no idea where she learns these things. :) She's now learning to wave hello and bye bye. :)

Tonight when I was on the phone with Sarah, I heard Gracie yapping in the background as usual. :) Then I heard a very clear and intentional "Ma-Ma!" It was absolutely at the top of my list of the cutest things I've ever heard. Sarah said she's been saying that for a few days. Charmer that Gracie is, she says "ma-ma" all night until 3 AM because she doesn't want to go to sleep. Saying "ma-ma" makes mommy weak in the knees, so I have a feeling Gracie can do whatever she wants. :) Next we're working on some version of dada. :)

As far as how Gracie is doing, it sounds like she's doing great. I believe today is day 2 on Busulfan. She's tolerating it well, but she has lost some of her appetite. Also, clearly her sleep patterns are out of whack. I guess when you are in a room and don't go outside, the concepts of night and day sort of lose meaning. :) Weird, eh? She gets and infusion of Busulfan 4 times per day (every 6 hours), that sort of dictates her internal clock and rhythm.

Oh, Sarah also got to go outside for a bit today, which is great news. Sounds like Gracie did fine while mommy walked down to a coffee shop on the corner. Sarah said the weather is amazing right now, and she enjoyed getting out for a bit.

On the whole, Sarah sounded in much better spirits today. That is a relief for Uncle Mack. Gracie and Jimmy also sound like all is going well for now.

As always, thanks for checking up on Gracie. Gracie's whole family is so appreciative and lucky to have such a wonderful support group and community. Thanks for all of your prayers, comments and messages in the guestbook. Oh, and Sarah loved that her nurses from Shands signed the guestbook. She sais that she left your email addresses in Lake City on accident, and she's glad that you guys found her. :) Thanks again!

Register - National Bone Marrow Registry Program

Hey all. Just wanted to pass along some info about FREE registration for the Nation Bone Marrow Donor Program that I saw on Lauren's mom's yahoo site. http://groups.yahoo.com/group/laurenslife/

May 5th through the 19th The National Bone Marrow Registry Program is
letting people join the registry for FREE! Normally, they charge the
cost of processing your cheek swab (yes, that is all you have to do
besides some paperwork) to enter all of your info in the system.

Anyone in good health ages 18-60 is allowed to join the registry.

Here are some facts about joining the registry that you need to know:

When you sign up, you will be sent a packet that includes the
materials to swab your cheek and return with the appropriate
paperwork. The NMDP will take the items you send them and use them to
enter you into the registry.

Next... you wait. What are the chances you will be chosen? About the
same as winning the lottery. That's right, you may never get 'the
call'. However, if you do, you would have the chance to save the life
of another person... to be their hero.

If you are ever considered a 'match', you will have the chance to say
NO at any point in the process. If you stay a 'yes' then you will
have a physical done to make sure you are healthy and the other
person's doctor will decide what type of transplant s/he needs. Many
transplants these days are done from blood taken durring a Peripheral
Blood Stem Cell Donation. Some people will need to give actual marrow
which is taken under general anesthesia.

It would be so amazing to be able to save another life in honor of
the person who donated their cells to Lauren.

The registry is a great way to help other folks with life threatening illnesses like Gracie and Lauren, possibly giving them a second lease on life. As mentioned above, it requires only a cheek swab (no blood or needles) and a bit of paperwork, and it FREE from 5/5 to 5/19.

Tuesday Night Update

Hi everyone. Uncle Mack again... I just got off of the phone with Sarah so I thought I'd leave an update for you. A play be play of our phone call is below, but if you are pressed for time, here's the readers digest version:

In a nutshell: so far, so good - Gracie is doing well. Looking forward to better continuity across the coming days, and everyone is in need of a little more rest.

1. How is Gracie doing? Gracie is doing much better on the Busulfan than she was doing on Campath. I guess she just tolerates it better. It's the drug that typically makes them not want to eat. Sarah said that her appetite came back and that she eating and nursing like crazy. I guess it may be due to getting off of the Campath. This is also the drug that is supposed to make her lose her hair. Waaaa. :(

Gracie has been needy though, Sarah says. She wants to be held constantly, and Sarah thinks it may have to do with the amount of traffic in the room. Which leads to #2...

2. The BMT unit staff. So far, Sarah says that there are lots of different people coming in and out of the room. Across the next day or so, Sarah and Jimmy get to pick who they want to be their day nurse and their night nurse. I think that's pretty cool. It would certainly boost confidence in their capabilities if you get to pick your nurses. It will also reduce the traffic, and the continuity will allow Gracie to become more familiar with her nurses and ease some of her anxiety.

3. How is Sarah doing? Sarah is tired, a little homesick, and a bit stir-crazy. She essentially sleeps when Gracie sleeps, which sounds like not very often and not for very long. She misses all of you, and she especially enjoyed the church video you all sent. As you certainly know if you've spent much time in a hospital, time passes veeeeerrryyyyyy sllllloooowwwllyyyyy.... Sarah's looking forward to a) getting back to RMH, and then b) getting back to her home in Lake City. There isn't much room in their little hospital cell to get around, and Gracie is pretty demanding these days, so no time to read, chat, blog, etc...

After they get settled and have a dedicated care team, they will probably be better able to schedule time on and off of the unit for fresh air, naps, etc.... When Noni gets here, I'm sure that will help out quite a bit. In the meantime, Sarah said that someone sent her vitamin C that she's taking to keep her immune system strong. So thanks for that too!

4. The room. Uncle Mack tried to pull some strings through his previous employer, where folks have relationships with the hospital administrators, to get them into a bigger room. Unfortunately, it didn't work out. We do have friends in high places, but they happen to be at the wrong hospital... Oh well. I suppose it won't hurt for the staff to know that Gracie is a VIP in some Fairview administrator's eyes.

As it turns out, my contact's contact runs the oncology program at a different hospital that is also part of the Fairview system, but doubtful that person has the influence at UMinn Fairview Hospital. On the bright side, their room has a Nintendo Wii in it (Wiiiii want to play - for those of you who have seen the commercial), and Sarah says they play Wii tennis and golf and other games when they can. She says its lots of fun and allows she and Jimmy to get up and move around a little bit. I guess Uncle Mack will have to buy them a Wii when they go back to Lake City...

Sarah and Jimmy are going to upload some recent pictures soon, and Sarah will leave a mommy update sometime in the next few days when she get a few minutes. I think that's pretty much it for now. They really miss everyone, and thank you again for your warm wishes, thoughts, and prayers.

New MPS Links

Just a quick post to point out our new MPS links. Look a little to the right and scroll down a few clicks, and you'll find links to Gracie's friends, doctors, hamburger clowns, and folks who can tell you ways to help kids who really need a hand. We hope you'll surf some of these when you have a little free time.

Monday Night Update

This is Uncle Mack. I spoke to Sarah a little while ago, and she sounded pretty good. She said Gracie seems fairly normal, playing and all. She also ate and entire thing of sweet potatoes today. :) The switched her antibiotic from Vancomycin to a less intense drug. It caused her to break out in a rash, but Sarah said they took care of it. They have also put her on a drug to control her blood pressure. Again, they say this is all within the bounds of "normal." It's just a little jarring to think about.

I just love this picture!

Tomorrow, she starts her first real chemo drug, Busulfan at 4:00 AM!!! Ugh! Please send Gracie your thoughts and prayers, as we hope these drugs do what they are supposed to do, reduce her immune system to a point that it won't attack the new cells that are introduced to her body during the transplant. Essentially, we want the drugs to weaken her so that the new cells will win the "battle" over which cells will control of her body. W are rooting for the new, healthy cells. :)

Again, Sarah sounded pretty good. She's not real happy with her room assignment - it's teeny tiny. It won't fit a bed and a crib at the same time. So far Gracie won't sleep in a crib, so Sarah sleeps with her. But, the hospital will not allow them to sleep in a bed unless it has rails... Anyway, the point is that the room is just too small for much stuff. From the sound of it (haven't seen it yet) it's about the size of 1/2 of a small hotel room. Cramped quarters for living the next 6+ weeks! Ugh... We might see if we can change that. We'll keep you posted.

Thanks again for your thoughts and prayers. Keep em coming!

Day -10

Today is our 3rd day of Campath, and thankfully, Gracie seems to be tolerating it much better. Her fever has gone away and she is much more playful than she was on Saturday. They started her on TPN last night which is basically all the nutrition she needs. She is still eating and nursing, which is encouraged, but due to the fact that she has had some vomiting and also they can't tell how much breastmilk she's getting, they went ahead and gave her the nutrition just for safety's sake.
This morning they drew blood at 4 am (this happens everyday) and both her hemoglobin and potassium were low so Gracie has had some red blood cells and some extra poatssium today so far. She's looking pretty good (a little pale because of the low hemolobin level) and doing well according to the doctors who visitied us about an hour ago. She seems to feel better and has been playing and flirting with all of her nurses today.
A couple of nice ladies from Speech Therapy and Physical Therapy came by this morning, and unfortunately Gracie was still drowsy from Benadryl and not interested in talking or playing much. I went ahead and asked that they start coming to visit in the afternoons when Gracie is in her prime, so I am really looking forward to seeing what all we can do to help Gracie along developmentally.

Anyway, so far so good. Gracie starts Busulfan tomorrow.

By the way, Rylie is down the hall from us and seems to be doing well. Also, Chance's mom had her new baby yesterday! Cooper Allen was born at 8:30 am yesterday morning. Congratulations Tracie and Ben!!!

Hello from 5D- the Transplant Unit

So Gracie was admitted yesterday and we are settled into our little room. Little being the operative word. Yikes. 1 or 2 months in HERE? I think I'll need the walls padded by Monday.

Anyway, last night Gracie made a valiant effort to sleep in the crib here, but by 3 am she was awake and not interested in sleeping anywhere without Mommy. So the nurses removed her crib and brought in a hospital bed for the 2 of us to share. Hopefully Gracie will be ready to try crib sleeping again soon. Mommy would sure like to have a bed of her own now. :)

Ok, so lets talk about today. Today is Day -12 and she started Campath today, which is an immune suppressing drug. Side affects: Fever, rash, and general yuckiness. She just registered a temp of 102.1 and has been vomiting up about half of everything she takes in. :( No rash so far. She just choked down a dose of Tylenol to fight the fever, and they'll be sending her for a chest x-ray to look for any infection and starting her on IV antibiotics in a few minutes. Those are standard procedure for fever spikes so we are not really worried that there's really an infection, just being cautious and following protocol. I hate that she's feeling bad already though. It just breaks my heart for her. I hope this day doesn't continue to drag like it has so far and I hope for a better night tonight as well.

"Pop Tabs"

One quick request.

You know those little metal tabs on the top of coke cans (they are "pop" cans in MN - silly yankees :) )? Well, please don't ever throw them away again! They are made of pure aluminum (the rest of the can is an alloy), and are collected by the Ronald McDonald House to help fund all of the many great things that they do. I have no idea where we would be without them. They deserve any assistance to ensure other families will have access to the same benefits.

Honestly, I never thought I could feel this way about a clown. They usually give me creepy crawlies. "My name is Mack, and I'm in love with a clown."

So save them at home. Put a little box in the kitchen of your workplace to collect them. You can turn them in at any Ronald McDonald House, and if there is no RMH near you, I believe you can simply take them to any McDonalds!

Thanks!

B - I - N - G - O

Tonight was the big Bingo night at RMH. It was a lot of fun, but Uncle Mack lost about $500! Just kidding.... It was cool, a great way to socialize a little while also collecting some good loot. :) There were a bunch of donated toys up on a table (enough for all of the kids to have at least 2), and this is a fun and fair way to distribute them.

They have a very sweet announcer with a bad microphone (everything that started with "thirty" sounded like she was saying "forty" and vice versa), and one of those ping-pong ball machines where the air blows the balls all around until one gets sucked up into the tube... It was just like watching the Florida lottery on TV when they first started the lottery back in the 1800s... Back when Uncle Mackie was a youngin'. After someone wins, they keep playing until 6 or 7 people have won on the same game, to ensure that everyone gets to say "B-I-N-G-O" at least once.

They played 5 or 6 different times with different objectives - the postage stamp, popcorn, blackout, etc. There was one game where she just called out numbers, and if she called your age, you won. They called "35" and Daddy won a prize. But Sarah and I really won that time because everyone at RMH now knows he's the oldest of all 4 of us. I don't think they had a "0" so there was no way Gracie was winning that time.

We won 3 times total, so Gracie got a new baby einstein dvd, an over-the-crib mobile that will hopefully help her sleep, and a bathing kit to have fun in the tub (she loves the tub). YAY!

After B-I-N-G-O we had to say good night to our friend Wyatt, and his mom and dad (Nicole and Todd). They will be going back home to Pensacola on Sunday, but chances are slim that we will see them again. We wish them well on the rest of their appointments, and hopefully Gracie, Sarah, and Jim will be able to catch them in Florida sometime once they get home. I love that when we were saying goodbye they called me "Uncle Mack." :)

Rylie (the one who had insurance troubles) was admitted to the BMT today. We saw her daddy, and could see the nervous excitement to finally have the ball rolling. She starts her chemo tomorrow, so will be tracking one day ahead of Gracie. We also have another RMH friend with a different condition who was admitted on Wednesday and started his chemo today, so 2 days ahead of Gracie. Another great thing about RMH, when you will be spending as much time in a hospital as we will, it's nice to know a few folks around you who are not on staff there. It will feel good not to be all alone.

Uncle Mack is heading back to Chicago tomorrow for a few days, but will return early next week. I imagine Sarah will be pretty occupied for the next several days, but she will try to update you all when she can squeeze it in. If not, I'll call and have her dictate to me so you all know what's happening. Tonight's goodbye was sad. Next time Uncle Mack will see Gracie she'll be in the hospital. When I left she was trying to help mom and dad get the video conferencing set up to talk to Noni and Muntz. Technical difficulties.

Oh! I also helped mom and dad change her dressing, and her rash looks MUCH better. In fact, you almost don't see anything. In other health news, the nurses took some snot from her the other day, just to check for any bacterial or viral infection (a cold). After 3 days the cultures still look clear, so she is healthy and in great shape!

Please wish us luck tomorrow and put Gracie on the top of your prayer list. Thanks so much again for the support.

The Schedule...

Here is Gracie's tentative schedule for the next couple of weeks. This is how it will go if she is admitted on Friday (as we are hoping!) You see the big day is on May 8th!

Sun	Mon	Tue	Wed	Thu	Fri	Sat
			April 23	24 ERT #11	25 Admitted to BMT	26 Begin Campath
27 Campath	28 Campath	29 Begin Busulfan	30 Busulfan	May 1 Busulfan	2 Busulfan	3 No Chemo
4 Begin Cytoxan	5 Cytoxan	6 Cytoxan	7 Cytoxan	8 Transplant Day	9 Day +1	10 Day +2

Campath is an antibody that sticks to immune cells, not an actual chemotherapy drug. This is a preparatory step for chemo. Busulfan will be her first real chemo drug (this is what makes your hair fall out). Cytoxan is the second chemo drug. We heard more info on them, but I don't remember them right now. Sorry! What I can tell you is that the doctors tell us that little kids actually tolerate chemo very well, which was a relief to hear.

On transplant day, Gracie will get an infusion from her umbilical cord blood donor, full of lots and lots of stem cells that will infiltrate the bone marrow, and ultimately help Gracie independently produce the enzyme she is missing. It will take a while for this to happen, so she will continue weekly ERT (enzyme replacement therapy) infusions until she does. More than any other time, she will need your thoughts and prayers during this part of the process.

You'll notice that after Transplant day, we start a new counting method and essentially a new calendar. This calendar "Day +1, Day +2, ..." refers to the number of days since Gracie was given a new chance at life. Thanks so much for all of your prayers and words of support.

An overdue Mommy post...

So, I know I am overdue for a post and I've been letting Uncle Mack pick up the slack. Sorry about that. The truth is that I feel like I have been hit by a semi truck and I'm still in a bit of shock when it comes to the reality of this whole thing. The exit consultation was probably the hardest and most agonizing thing I have had to sit through in my entire life. I didn't even make it out of the clinic before I broke down and needed to force myself to remember why we have to put our precious baby through all of this. So to put it plainly, we are still reeling.

Tomorrow, Gracie goes in early to be sedated for her MRI and lumbar puncture. Then she will be admitted to the Transplant Unit where she (and I) will live for quite some time. I'm not sure that will quite be the cure for my present homesickness, but I will definately be glad to get this show on the road so that we can get closer to our goal of bringing home a healthy Gracie.

Yuck, what a morose post.

I guess we all know why I've been leaving the posting up to Uncle Mack the past few days. :) We'll see what I can do to be a bit more chipper on my next post, ok?

Ahh... Relaxing on Humpday

(Me and the therapy doggie!)

Hello all. Today was a relaxing day with no doctor appointments. This evening after dinner we played for a bit, and then Uncle Mack and Daddy went to Ikea (My GPS just calls it I-kee :) ) and Target in an effort to buy some very cheap additional storage since the White's will be in the smaller RMH room for a while. It's not tiny by any stretch. The size of an average hotel room. But with a full/queen bed, a twin bed, a crib, a jumpy chair, two dressers, a table, and a mini-fridge, there is not much room for two adult's belongings, and a baby with tons of medical supplies. It's tight in here, to say the least, and sometimes uncle Mack gets a little claustrophobic and has to leave the room... :)

Fortunately, we did really well. We got some hang in the closet thingys and some under the bed storage bins and didn't spend more than 5 or 6 bucks on any one thing!

Well, as promised, here are some updated pictures of the monkeychuck... Gracie seems to be a lot better, and her rash is clearing up too. Everything seems on course for ourhospital admission on Friday. She's ready to fight the good fight - and Win!

(My best school pictures!)


(At the big girl table for dinner)

It gets harder and harder (for Uncle Mackie) to leave the RMH each night, as Gracie gets cuter and cuter by the day (by the way, she is clapping now). Since I know that she will be in the hospital for a few months, and she doesn't know, I know we won't get to play as often so I just don't want to leave. :( I have to keep reminding myself, "think big picture" and "keep your eyes on the prize." It's very tough.

Of course, then within 5 or 10 minutes she starts squealing and hollering and I am ready to go... (Of course I'm just kidding, just had to lighten it up :) ).

Storm Season

Official notification that we are in the midwest: Tomorrow at 1:45 the city of Minneapolis will test the sirens they use to notify residents that a tornado has been spotted. We officially enter storm season in May!

Geez (I think that is the term they use here), I hope I can remember the drills we practiced in Mrs. Maxwell's class back in 5th grade! I think we were supposed to hide under our desks. I heard those sirens when I was in Omaha on business. They corralled us all into a safe room but it was weird. I guess it beats hurricane season in FL, because hurricanes cause tornadoes on top of everything else. But hurricanes are a bit easier to predict.

Today is sunny and 75 degrees. :) I need to get back to my homework - reconciling medical bills. woo-hoo!

Dinner at Sally's

Tonight Wyatt's parents Todd and Nicole invited us to dinner at Sally's, a little restaurant/bar in the neighborhood. Being on the university campus, most dining establishments are either sports bars or fast food joints, with a couple of other options peppered in... Just to refresh your memory, Wyatt is one year post transplant, and here for tests and check-ups. Todd and Nicole said Sally's was their second dining room when they were here a year ago.

At dinner, Sarah won the prize for getting the most food on herself. Gracie won the prize for sleeping the most - almost through the entire meal. Wyatt wins the entertainment award. He's really a cute kid. He loves to play games with his daddy, and even played once or twice with uncle mack. :) He got really excited when it started raining outside. Such a cute little guy. I did get one picture (forgive me for the bad lighting) while we were there so I thought we'd have Wyatt make a cameo on SG.

Daddy has taken a bunch of new pictures of Gracie too, so we'll pick a few good ones to post here over the next couple of days.

Good night everyone. The next couple of days are doctor appt. free. The prescription is lots of rest fo both Gracie and Mommy. They've got some catching up to do!

Monday News - A Couple of Extra Days

Before we get into Gracie news, I just wanted to let everyone know that Rylie's insurance company agreed to cover her transplant today! We are so excited for her and her family. I know that they are really ready to get things underway. Gracie will be so happy to have a partner to go through this process with, I'm sure. Thanks for all of the prayers!

Gracie met with Dr. Orchard this morning, and Dr. Whitley this afternoon. Since Gracie is fighting off a tiny cold and because she still has a little bit of a rash (although it's really improving), the collective decision was to delay Gracie's admission until Thursday or Friday. Once admitted she will start the chemotherapy process.

While everyone is excited to get started, today all of this became so real (to me). It gets more real each time I walk into the hospital. By the end of the week our little Gracie will be starting chemo!!! ACK!

We walked through her treatment regimen over the next 30 days or so. So much going on! Antibodies, immune suppressors, other chemo drugs. Then there were all of the meds to address the side effects of the treatments. Anti-siezure meds, blood pressure medications, allergy meds, steroids, anti-fungals, pain management medications, diuretics, to name a few . My head was spinning. Poor Sarah had a glaze in her eyes and a look on her face that was probably no unlike when Gracie was first diagnosed. She's a tough one though. She grew up with two very mean and manipulative older brothers and sisters. Besides, we are keeping our eyes focused on the big prize - a healthy baby with a long life ahead of her.

In a week or two, these postings will be full of numbers as well as commentary. There are a lot of indicators that collectively give us a picture of Gracie's progress. We're looking at white blood cell counts, hemoglobin, platelet counts, neutrophils and lymphocytes... Before we throw too many numbers at you, we'll have a description of what they are and why they are important.

After the treatment regimen, Sarah and Jimmy had to sit through a long discussion of all of the things that could go wrong with this procedure. It was painful for me to hear, so I know it was really hard for them. The doctors have to inform the families of all of these things. It 's scary, but its so important that we focus on the bigger picture, and what will benefit Gracie most over the long haul. To make things harder, Sarah and Jim had to start signing all of these consent papers to allow the university to use her data for research purposes.

I'm sure there is more to tell, but that's all I can think of right now. Plus, Sarah just called, and Wyatt's family is going to Sally's - a restaurant/bar in the area for dinner, and invited us to tag along.

Again, our spirits are high, and the over-arching feeling is one of nervous excitement. Everyone is focused now on staying healthy and making sure Gracie is in peak condition when she arrives at the hospital to be admitted. She is a fighter, and we just need to give her all of the time and tools we can to make sure she has a competitive advantage.

Keep us in your prayers!

The RMH Hurler Families

Tonight we had another dinner at the Ronald McDonald House (RMH) prepared by very generous volunteers. There were 4 kids with Hurler Syndrome at dinner.

Of course Gracie was there. I think she was eating squash tonight. She is the youngest of the 4 kids, and she is scheduled to be admitted to the hospital on Tuesday for all of her pre-transplant prep. with transplat cheduled for cinco de mayo. Ole!

There was Chance, who is just over 1 year old, and is 38 days post transplant. He was running around in his little mask inside a little walker seat. It was kind of like a bumper car for babies. :) Chance is having a procedure tomorrow to replace his broken central line. His mom, Tracie, is also expecting a new baby any day now! Talk about stress! Please keep then in your prayers. Chance has a website too: http://www.caringbridge.org/visit/chancers

Next was Riley. Riley is the adorable little redhead who is having issues with her insurance. She's the comedian in the group. :) She and her parents have been fighting for a while and are still waiting to move forward with her transplant. We are pulling for them. Her website is: http://ryliehays.blogspot.com/

Finally, we met Wyatt. He's another adorable little redhead who is in Minneapolis for his one year post-transplant check up. Go Wyatt! He and his family live in Pensacola. He was running around everywhere just like a normal two year old. Seeing him and meeting his parents was really inspiring. Wyatt doesn't say a whole lot, but he understand everything you say, and he communicates really well through sign language. It was really cool to watch him communicate. Wyatt's website is: http://www.caringbridge.org/visit/wyatt

There are so many other families there... I've gotta say, the RMH is an amazing place. While I wish no one ever had to deal with such issues, being surrounded by families going through the same, or very similar ordeals is really supportive. It's a big support group. You can feel sad, angry, relieved, and scared and not have to explain why to the people who surround you. You can bitch and complain and be in great company, since everyone there has much to complain about. It's also interesting to hear the stories of other children from all over the country, and the ordeals they have experienced through initial diagnosis and treatment prior to arriving at RMH and U of MN. It's also encouraging to be in an environment so full of hope and faith (not the terrible sitcom).

I hope you are all doing well. Wish us all good luck this week!

Contact Information

Hello all. I've gotten a couple of requests for Gracie's contact info in Minneapolis. Here it is:

Sarah, Jim, and Gracie White
c/o Ronald McDonald House
621 Oak St. SE
Minneapolis, MN 55414

It's absolutely beautiful here today. 70 degrees and sunny. We're going out for a walk around campus now. :)

At home at the RMH

So as Uncle Mack told you, we have settled in here at the Ronald McDonald House (RMH) and we are really enjoying it here. Everyone here is so nice and the facility is absolutely beautiful. There are really nice kitchen areas where we can cook anything we want, and even better, there are volunteers who come in everyday and make dinners for us... and brunch on the weekends too! Its really so nice to not have to worry about making dinner or even deciding where to go for dinner. Just go downstairs and its waiting for you. :) At such a scary time in our lives, its a beautiful thing to see the many kindnesses of strangers. Often when I am standing in line to get my food, I have to fight back the tears as I watch these people rush to serve everyone and smile and talk to all of the children despite their masks and bald heads.

It really does feel good to be here.

Currently, we're in a smaller hotel sized room. We have a minifridge and our own bathroom, but it definately feels small. We're on the fast track to a suite though, due to the length of time we expect to stay here. I'm looking forward to getting a bit more room to move around.

Things have been especially small-feeling around here due to the fact that Gracie has a cold. We've been trying to keep her here in our room instead of taking her downstairs around all of the immuno-suppressed kids, so we've been getting our meals in take-out boxes and staying upstairs a lot . :( Not much fun for Mommy and Daddy. We're hoping that the antibiotic that Gracie is taking for her skin inflammation might do a little work on the cold as well.

Uncle Mack brought Gracie a new Baby Einstein jumpy chair and she has been enjoying it as much as she can considering how sick she is. She really missed being able to jump and she just loves all of the cool new toys on it. Thanks Uncle Mack!

We meet with Dr. Orchard tomorrow morning first thing to check her suitability for admission on Tuesday. I really hope she'll be feeling better and we won't have to delay. Please pray that we beat this cold and the skin infection pretty quick.

More to come later. :)

A Pyrrhic Victory, but a Precedent for Winning.

Problems like Riley's deserve militant action, in my opinion. I guess my saying so won't much change my well-earned reputation for going ballistic.

I probably should have posted this as a comment on Mack's last post, but I wanted to include a link to this article about one Nelene Fox, whose doctor attempted a Hail Mary autologous BMT for her stage four breast cancer and was slapped down by her HMO, Health Net.

You should take a look for yourself, but to make a long story short, the patient eventually had the treatment, which did not save her life, after her family raised $210,000 to pay for it. The insurance company was hit with the largest judgment ever handed down (at the time of writing) for denial of a medical procedure. The jury's decision was unanimous.

Health Net disallowed coverage for the procedure because it deemed autologous BMT an "experimental" procedure.

The article includes a quote from Fox's lawyer, Mark Kiepler. "'Experimental' is a euphemism used by HMOs to refer to treatments deemed too expensive." He must not be the only person who picked up on that, because, according to the article, 25 of Kiepler's clients (at the time of writing) subsequently persuaded their insurance companies to cover procedures that were initially disallowed using the outcome this case. I guess a verdict $89 million in compensatory and punitive damages is a powerful threat.

So let's take a look at that. I don't know much about Hurler Syndrome, and I know next to nothing about breast cancer treatment, but it sounds to me like the insurance company got slapped for failing to cover BMT in Fox's case, something that actually was something of a last-ditch effort to treat stage four (metastatic) disease. Covering transplant for an under-two Hurler patient seems much less "experimental", since it's my understanding that allogenic stem cell transplant is the currently agreed upon as the treatment of choice for Hurler patients whose doctors determine that they are candidates.

I'll bet a jury would totally see it that way.

I wonder if the folks at Sun Life have ever heard of Nelene Fox. The article I read was dated 1994, so perhaps they've forgotten about her. If so, maybe they ought to be reminded.

Health Insurance Woes

Isn't this exactly what health insurance is for?

Yesterday evening I got to meet Gracie's friend Riley and her family from Colorado. Riley is an adorable little red-head with pretty eyes. She was so cute. She showed us her bellybutton, her nose, ear and toes. Very smart!

After speaking with the family, it sounds like they've had a hell of a time dealing with this illness. Riley has been getting ERT treatments for a while, but their insurance company (Sun Life out of Boston) has not yet approved the transplant procedure. Given that two other kids are at the RMH with the same disease and have been approved by their insurance company, it's that much harder for the family to swallow. Sun Life is citing a stop/loss policy condition or something as the reason to not pay. What I thought was strange is that Sun Life hasn't committed one way or the other. They say they *might* cover it, and they might pass the bill on to the dad's employer. I can't quite figure that one out.

Riley's dad has clearly been around and around with them, and the docs and nurses at Fairview/Univ of MN have been using everything in their arsenal to get them to commit to covering. It's just such a shame, because this is exactly what insurance is for. It's supposed to kick in when something totally unexpected and astronomically expensive happens. That's why we pay into in, even when nothing happens over the course of 1,2, or even 10 years. Yes?

Anyway, the insurance company says that the treatment is experimental, ever though it has been done for 20+ years. But because there is a clinical trial associated with it, they call it experimental (regardless of whether they enroll in the trial). I wish I could help them. Maybe I could pull a list of a bunch of other ongoing clinical trials. I wonder if they cover drug-coated stents for heart disease? I'm sure there are countless trials underway for other established treatments for more common conditions like heart disease, asthma, even erectile dysfunction. I bet they cover those treatments. It's a numbers game. The thousands of Sun Life members who have those conditions would run them out of business otherwise. Just because a condition is really rare, doesn't mean that treatments for those diseases are not proven and established. But because it's so expensive and there are so few members impacted, they can get away with screwing you.

If anyone know anything about insurance companies, how they work, how to respond to them, or has any recommendations for Riley's family, please let me know. In the meantime, keep her family in your thoughts.

I think Dr. Orchard sent a letter to the Boston Globe. If and when it's published we'll share it with you.

Itchy and Scratchy

Gracie is sacked out right now. A cute little monkey-chuck. She didn't sleep well last night so he was very tired today. Mom is knitting on the bed. Daddy is playing on the computer. And Uncle Mack is playing on his computer too. Two laptops on the same table. So weird to think of how fast technology advances...

Today was all about Gracie's central line. Ever since the last trip to Gainesville, Gracie has been scratching around her line. Sarah and Jimmy noticed some ulceration around the area which is indicative of some sort of infection. Docs on Sunday thought it was a fungal infection and prescribed an anti-fungal powder. Well, they were wrong. Today when we removed the bandage, it looked so uncomfortable. It was clear that it was itchy. Poor little thing. It was also worse than it was earlier in the week.

The good news is that the line itself seems fine. The infection is just in the skin around the line and all under the bandage. Seems that part of the problem is that the skin around the line has been covered with a dressing that doesn't breathe for 2 months. The skin is just breaking down a little bit. So the started using a dressing that breathes. The Nurse Practitioner today prescribed an oral antibiotic to try to take care of it. Now the challenge is to keep her little paws away from the line. She tries to get to it all the time. Keeps mom and dad on their toes. :)

Tonight there are volunteers at RMH that are cooking dinner. I was just downstairs and it smells yummy. Smells like ham and mac & cheese. MMMM. We go down and grubb at 5:30 (central time).

Oh! I got to see Chance today. He's so cute. He has beautiful eyes. He was with is dad at the bone marrow transplant unit (BMT) and was wearing his little mask, like Gracie will be wearing soon. Sounds like all is going very well for him. We're very happy for him and his family. Please keep him in your thoughts and prayers too. :)

The BMT was full of folks of all ages wearing little masks. It was both sad and very happy. Sad to see so many little kids who are sick, but exciting that they all have hope. Definitely hit uncle mack hard though. I'm gonna have to toughen up a little if I want to be the supportive big bro. :)

Quick Check-In

Hi all. Just wanted to leave a quick message to let you know everything is going great here. Gracie had ERT yesterday, and she did very well. Sarah, Jimmy, and Gracie are checked into the Ronald McDonald House. It's a really nice place. I was shocked at how clean and comfortable it is. I'll get a better look today and report more. Funny thing is, they have those crappy sleep number beds that we had at the Radisson. :)

I can't tell you how great Gracie looks. I has only bee 8 weeks since I've seen her, but the changes are amazing! Her coordination has improved 1000%. She babbles and she just seems so happy. She's starting to be mobile! If she wants something bad enough, she'll wiggle and squirm her way over to it. It's so cool! :)

Today is a pretty light day for Gracie. We have a line consultation this morning, and then we're planning to check in with Dr. Tolar, our surgeon, if we get a chance.

Gotta get ready to go, but I just wanted to drop a quick posting to let you know we are all doing well. I'll write more later on. Bye!

Hit the ground running...

Yup, thats basically what we have done since arriving in Minneapolis on Saturday evening. While we expected to have Sunday to relax and settle in, it turned out that we needed to go into the clinic so that Gracie could have her central line site looked at. Over the past few days it has become increasingly more irritating to her so she has been scratching and pulling at it all the time. It looks like she might have some sort of a fungal infection at the site and it seems that the meds are working because its looking better and doesn't seem as itchy. Yay! And in between dressing changes we've been given the ok to give Gracie a real bath! WOOOOHOOOO! Her first in 2 months. I am so excited for her. We'll probably do that in an hour or so.

Anyway, lets do a rundown report of our appointments from yesterday.

1. Dr. Orchard - just a quick physical and chat with him. He said she looks fantastic and he doesn't expect any delays when it comes to admission and transplant so that was good news. If all continues to go well, Gracie will be admitted to the BMT unit next Tuesday- the 22nd. I aso should mention that Gracie spent a lot of time flirting with Dr. Orchard which he enjoyed immensely.

2. Pumonary- Just a quick listen and chat with Dr. Philips who said that Gracie looks and sounds great and there's no reason not to go ahead with transplant.

3. Echo/Cardiology - Gracie had another echo done and then we met with Dr. Braunlin to go over the results. It turns out that the ERT has done wonders for Gracie's heart issues. Her septal walls have shrunken to normal size and her aortic and mitral valves have gotten smaller as well. Her leakage from her mitral valve has also diminished a little, which is a surprise but we're certainly not complaining. :) Dr. Braunlin was really really happy to see such huge improvements and she gave us a green light for transplant.

4. Neurology - Dr. Charnas was very impressed with Gracie and in fact, he asked if she was the same baby that he had seen 2 months ago! :) He feels that she is doing well developmentally and that the ERT has definately helped her make many vast improvements neurologically.

So, a clean sweep yesterday... straight A's as far as I'm concerned.

Today we just had one consult - NeuroPsychology with Dr. Kendra Bjoraker. Dr. Kendra was also amazed to see Gracie's progress and all of the ways she's changed. She did a little bit of testing and we'll get the official scores tomorrow, but Dr. Kendra said that in a mere 2 months, Gracie has improved her scores a LOT. She kept saying over and over how amazing our Gracie was doing. :) :) :) Sounds like an A+ from Dr. Kendra! Woot!

After NeuroPsych we had an EKG and an Xray done. Daddy says that the EKG was beautiful and we should know about the Xray tomorrow when we meet with Teresa, the Nurse Coordinator.

In other news, we met 2 other Hurler babies yesterday! We met Chance, a 1 year old who I have written about before, and Rylie, a 19 month old redheaded cutie who has been waiting for transpant for 5 weeks while her parents and the folks here fight with their insurance company. Please keep Rylie and her parents in your prayers. I can't imagine what they are going through with that situation. :( Chance looks to be doing very well. He's been released back to RMH and is coming in for checkups every few days. Go Chance! I'm looking forward to getting over the RMH and getting to know these families better. Hopefully it won't be too much longer.

Ok, so Gracie and I are going to take a rest and watch some Mr. Rogers and then we'll have a bath. More later.

Safe and Sound in Minneapolis

Hi all. This is Uncle Mack. I just wanted to pass along the news that Sarah, Jimmy, and Gracie are safe and sound in Minneapolis. They arrived on Saturday, and it still feels like winter there. :) They will be staying at the Radisson right by the hospital until a room opens for the in the Ronald McDonald House (RMH). RMH folks said there are a handful of people on the list ahead of them, but that hopefully it will only be a couple of days. A couple of days is no problem, since they were able to get a pretty good rate at the Radisson through hotwire.com. Also, I'll be there on Wed. and they will shack up with me if they still aren't in RMH.

Unfortunately they had to go to the clinic yesterday because Gracie stared fussing with her central line and ripping off her bandage. The docs looked at the area and prescribed some anti-fungal medication, that will hopefully take care of whatever was bothering her.

Today is a big day. Gracie's first day of work-up pre-procedure. Lots of appointments. These are really important exams and consults. They will hopefully a) show impact of the initial ERT treatments on disease progression (or lack thereof - hopefully) since her last visit in Feb; and b) serve as a baseline for measuring Gracie's progress after the cord blood stem cell transplant.

Here is Gracie's itinerary today:

• 9:00 - Review calendar and labs
• 9:30 - Meet with Dr. Orchard - History and Physical Exam (he is the first physician we contacted, and the primary investigator over Gracie's clinical trial)
• 11:00 - Echo - I think this is for an echocardiogram - which is some sort of heart imaging procedure (you may recall, Gracie already had minor valve issues when she had this exam in February)
• 11:30 - Pulmonary Consult
• 1:30 - Cardiology - Dr. Braunlin
• 4:30 - Neurology consult with Dr. Charnas (Another of the key physicians running the clinical trial)

Uncle Mack will be driving from Chicago to Minneapolis on Wednesday to tag along on hospital / doctor visits and offer whatever support possible. I can't wait to see my baby sister. :)

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

Population Genetics: A North Florida Hurler Club?

I have been rolling this around for a little while, and I'm still on the fence about it. I think it's kind of interesting that, of all the readers who have posted comments to this blog, and who are Hurler parents (that's what, seven or eight families?) four including Gracie's parents are along the I-10 corridor in North Florida.


So, is this a population blip or an information technology blip?


There is a temptation to jump to the conclusion that there might be a population of MPS-1 carriers in this area, but of course, right or wrong, this is pretty much statistical hooey. Here's why:

• Four is not a statistically significant sample, even with the tiny population of Hurler kids that would be available for study. The number realistically exists on the level of coincidence.
• Americans travel a lot. Gracie's dad is not from North Florida. Since Hurler is autosomal recessive, both parents have to be carriers for the gene to affect their child. Even though having one parent with an increased likelihood of carrier status (as would be the case with Sarah, if there were a concentration of carriers in N. Florida) does increase the total statistical likelihood for the couple, Jim's being a transplant dilutes the already pretty watery argument.
• As it turns out, four Hurler kids living in North Florida isn't really that huge a number. 1:115,000 is a small chance, but given the large number of births that occur each year, it's big, but not enormous. In 2004, for example, 218,053 babies were born in Florida, of whom it is statistically probable that two were Hurler kids. Given improvements in treatment since that time, and much longer lifespans for children with Hurler Syndrome, it is not really that unreasonable to find four living children with Hurler Syndrome in Florida today. It's interesting to me that they seem concentrated in the I-10 corridor, though. It's a big state.

There have been reports of population concentrations in Hurler syndrome, as with many inherited disorders. One I read about was an elevation in a region of Northern Ireland. Isolated populations do tend to magnify genetic disorders, or just genetic differences, by intermarriage. The Ashkenazi Jewish population is a good example of this. Among other inherited disorders, this population, according to my reading, has a higher incidence of Hurler syndrome than the general population. Multigeneration North Florida families don't have the same degree of gene pool isolation, as they're all pretty mobile, but people who stay close to home in any population will naturally have a tendency to intermarry more frequently, right?

I do find it interesting that this population is so prominent on our blog. Does it mean that we're corresponding more with our geographic neighbors in this crisis than with people who live farther away? Surely the same internet is available to everyone, and people all over would find each other through the same channels that led us all here...

Anyone got any ideas about this?

The Best Kept Medical Secret?? Public Cord Blood Donation

Anyone who has had a baby recently knows what I'm talking about. They stack information fliers in your Ob's office, they send little trifold brochures in the mail, each with an adorable photo of a toddler regarding with interest his bellybutton. They are private commercial cord blood banks.

The pitch: Collect your child's cord blood and pay us to store it for you as "biological insurance" in the event the child or a sibling should need stem cells later in life.

The price: $1500 or more up front for processing, plus $100/month for--well--ever, I guess, to store it for you in the event you should need it later.

The kicker: Your child's own cord blood, at least given current medical technology, is not nearly as likely to help him as someone else's is.

How's that, you ask? Currently, cord blood is mostly used to rescue endangered bone marrow (hematopoietic tissue) in the recipient. For most children in need of transplant, it is because their own stem cells are lacking a necessary function (as in Hurler Syndrome) or their bone marrow is producing malignant cells (as in leukemia). In either category of stem cell transplant candidates, your own banked cells won't help you, because they probably have the same problem you do. You need donor cells.

If your cord blood is privately banked, not only will it be unlikely to help you, it will be unlikely to help anybody else. Private banks keep your cord blood for you and your immediate family alone. Unless a sibling needs a transplant (the odds of a "perfect" sibling match are 1 in 4) and you are a perfect match, your cord blood will likely sit around in its apartment for as long as someone continues to pay its rent.


However, umbilical cord blood that is donated to a public cord blood bank has a very good chance of helping someone in need, maybe even someone you love.


Unrelated donor cord blood is increasingly favored over bone marrow for transplant of hematopoietic cells in children, largely because is easier to match. Both bone marrow and cord blood are "immune competent"--meaning they make immune cells and therefore cause graft-versus-host disease--but cord blood is more flexible in matching, as it hasn't "learned" as much about mounting immune responses and dedicating cells to specific jobs yet, and so is not as specifically tailored to protect its individual yet. Remember how babies are said to have immature immune systems? This is actually a bonus for cord blood recipients. It helps to make the graft more versatile.


(Attention docs and other pros: please help flesh out or correct my armchair science any time you see the opportunity. :-) )

So...

You can help your family and your community by donating your baby's cord blood to a public bank. If you're not in a baby-producing phase of life (or frame of mind) you can help by making a financial contribution to public cord blood banking and matching, or you can email the National Cord Blood Program and ask for literature to wallpaper your pediatrician's or ob/gyn's office. If you need more motivation, please read this beautiful letter to an anonymous cord blood donor. To find out more, and spread the word about public cord blood donation, please visit: http://www.nationalcordbloodprogram.org/index.html