Sunday, June 29, 2008

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

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

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

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

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



Friday, June 27, 2008

The Best Birthday Gift

It's a big day. I think today is Day +50. Whoa. It's also Sarah's 30th birthday. I can't believe my baby sister is 30. How did that happen?!?!

HAPPY BIRTHDAY!

Gracie met with Dr. Tolar today. He's one of the primary physicians managing Gracie's care. His first comments were how beautiful Gracie is. :) He said that she looks fantastic and that she has gone through this process exceptionally well.

Her numbers are still rockin. WBC is over 10 and ANC is over 7. Her platelets are over 100!!! AND she is still 100% engrafted with donor cells! Yippee!!!! Her tummy rash is getting better too.

All good news and a great birthday present for Mommy!

Thursday, June 26, 2008

Just wanted to share...

Gracie slept through the night for the very first time last night!!!! When my alarm woke me this morning, I almost panicked as I realized that Gracie had not gotten up since I put her down after her last dose of meds. Eek. I ran in here to find her sleeping away. :)

Today was good. No numbers again since they only drew for the cyclosporine (CSA) levels. We left clinic after Gracie gave them some blood and waited to hear how we needed to adjust the dose tonight. We never got a call. So I am assuming that we're good as far as CSA levels go. Woohoo!

No news about our latest engraftment results yet, I expect to get those on Friday when we meet with Dr. Tolar. Everyone send up a special prayer for 100% engraftment.

Much Love.

Wednesday, June 25, 2008

Day +46- Just hanging out!

Another great day for Gracie. We had the day off from clinic today so I have no numbers to share, but all in all, we had a good day. Late this morning, Mommy and Gracie took a walk so that Noni (God bless her) could give our room at the RMH a good cleaning. So, the rules are that if a room is being cleaned (vaccuumed, dusted, spraying cleaner, etc.), Gracie cannot be in it. We also have to wait an hour or so to let all the dust and particles settle back down. So Gracie and I enjoyed a long leisurely walk, shopping at the Gopher Store (FYI-the mascot of the University of Minnesota is Goldy the Gopher) for our cousins Van and Nuvy who will be visiting us next week, and to Orange Julius so that Mommy could have her fix. :)

Tomorrow we go into the clinic to have Gracie's Cyclospoin levels checked. Cyclosporin is probably the most important drug that Gracie is taking- its the one that combats GVHD (Graft-Versus-Host Disease). The docs have been playing with the dosage of this drug for a little while because Gracie's levels has been consistently on the slightly low side. Not low enough to be really concerned, but we're really just looking to get into the normal range. Here's hoping that the last adjustment took care of that. We should find out tomorrow.

We're also looking to find out about our engraftment status this week. Tomorrow might be too early to find out, but I'm definitely going to ask about it. Keep us in your prayers for good results.

As promised... I have a few pics of our favorite baldy hanging out in our room at the RMH. sorry for the sour puss, she was not in the mood for the paparrazzi. But she did look cute in her Uncle Mack duds. As it turns out, Gracie- our 11 month old- has to wear size 24 month clothes due to the water retention. I had no idea that she would bloat so much, so naturally, I was unprepared and only brought clothes for her up to 12 months. Uncle Mack did a quick Target run and came back with som cute and comfortable clothes for our fluffy little Peanut. I really loved this outfit on her. :)







Also, please pray for our transplant friends- Rylie (our special Hurler friend) is back in the hospital and having breathing troubles. We have another friend, Mario, whose transplant didn't take, and now his family hopes that the Chemo treatment prior to transplant has rid his cells of ALD. Maddie, an 8 year old with Fanconi Anemia is back in the hospital fighting either an infection or GVHD, and Mara- who has MPS VI is having a terrible time recovering from her transplant. :( Ask God to envelope each of our friends and their families with his healing grace.

Monday, June 23, 2008

Day +45

So while we didn't have to go to clinic at all this weekend, we were definitely still busy. Gracie's med schedule is still so incredibly demanding that I'm almost always busy giving them to her, or setting them up to go through her IV or unhooking the IV med and flushing her lines.... its a true race against the clock. Yesterday we somehow managed to get pretty far behind and Mommy and Gracie didn't to bed until after 3:30 am! Yucky. So Noni gave Mommy a break this morning... and so I took a loooong, much-needed nap.

Gracie has had a pretty good day today, despite the late bedtime last night. She spent a lot of time playing, took all her meds without much trouble, and was her sweet little silly self most of the time. She took a few good naps and even took a bath.

So tomorrow is our second to last ERT treatment, regular lab draw, and a check-up visit with Dr. Orchard. I'm definitely looking forward to hearing how he feels Gracie is doing and what we need to expect in this next phase of recovery.

So I know that its been a while since I posted any pics of our girl. I've taken a few, not many (bad mommy) but I haven't had the chance to uplaod them yet. I promise to try and get around to that later tonight or tomorrow.

We miss you all and appreciate all of your love and continued prayers. Please pray for our friend Rylie who had to go back into the hospital with breathing trouble. We hope she cazn come back here and play with us at the RMH soon.

XOXO

Saturday, June 21, 2008

Day +43... and the weekend off!

So with yet another round of great lab reaults today, it seems that Gracie has earned us a weekend off. :) We are not required to return to clinic until Monday morning when we meet with Dr. Orchard. This morning's WBC was 8.3, hemoglobin at 10.1, and platelets up again at 32!! Awesome numbers.

In other awesome news, Gracie seems to be getting her appetite back slowly. She has had several bites of sweet peas for dinner over the past few nights and izs managing to keep it all down. WOOHOO! She's also a crazy water drinker (she's still on lots of diuretics) but I have to make her pace herself with it or she will make herself sick... poor baby. I am just so pleased to see her interested in eating again... its just another step back into normalcy for us.

She was pretty tired today and slept quite a bit. As a matter of fact, I had to wake ger up so that we could catch up on our med schedule. She had a bit harder time keeping her meds down today than in days past, but she's still doing so much better with them than I anticipated, so I'm not complaining. I just hope she feels better tomorrow.

Uncle Mack leaves tomorrow until the first of next month and I know that we are going to miss him lots. Gracie especially. :) Noni will be moving in with us here at the RMH which means an extra pair of hands around 24 hours a day. Mommy is hoping that Gracie won't scare Noni away the first night.

We miss everyone so much. Hope you are all doing well.

Thursday, June 19, 2008

Day +42 - The Turtles Are Here!

They've been advertising and teasing us for months. Signs all over the hospital saying, "The Turtles Are Coming!" Nothing more. I thought it was an advertisement for Comcast. Turns out, the hospital has a fundraiser where you can sponsor a turtle for a $30 donation. Today was a full day of turtle races. Endless heats of turtles trying to decide whether or not they want to race. Kids everywhere. Lots of super heroes showed up. We saw batman and robin, wonder woman, and the green lantern. There were others as well, I just didn't see them. It was quite a festival, held in the courtyard between the main hospital and the clinics. We got to watch from our window in the clinic. There were lots and lots of turtles racing, so hopefully they made a ton of money.

Oh, the special in the cafeteria tonight is turtle soup. ;)

Gracie had her +42 day appointment today. We were mistaken about seeing the big docs (Orchard & Tolar) today. We are scheduled to see Dr. Orchard on Monday at 8:00 AM. We will see Dr. Tolar on Gracie's birthday, July 11th! Today was important because they drew blood for Gracie's second engraftment test (you may recall she was 100% engrafted with donor cells after 22 days). We won't get the results for several days, but we're praying that the donor cells are still dominating the cell growth in her body.


Her lab results continue to be phenomenal. Her white count was 9.3 today! We don't have the ANC numbers yet. Because we are now outpatient, and those results take more time, we don't get them until our next visit. Her kidney function results are also great (creatinine clearance was 0.34).

We expected Gracie to need an infusion of platelets today because her last test result was pretty low. She didn't! We were shocked to find the result higher this time, which means she might be creating her own platelets now! If Gracie is creating her own nucleated red blood cells and her own platelets (in addition to white blood cells), that means her bone marrow is once again working. That's pretty cool, eh? The engraftment results the we receive over time will tell us whether the donor cells or the original cells are driving the cell growth.

Overall Gracie has been doing great. Over the last couple of days she has been so pleasant. She's taking her oral meds like a champ, and we're all getting acclimated to the demanding meds schedule. Gracie was so good during her exam today. It's almost like she is ok with all of the poking, prodding, and manipulating now.... she's at peace with the lifestyle she's living and just goes with the flow. She's cool as a cucumber. :)

Today we ran into Chance and his family who are back for a few days for follow up testing. He's so cute and fuzzy. One of the anti-rejection meds that these kids take causes them to grow hair like a 40 year old man. It's funny to think of a 1 year old with a hairy chest, but I guessing Gracie will have one relatively soon. Hmm. Maybe I need to take some of that. :)

We also saw Rylie very briefly in the clinic. It's so nice to know that all of out little Hurler family have graduated from the hospital to the clinic setting. We also got to meet Lauren and her mom (author of the beautiful letter to an anonymous umbilical cord donor - link is on the right) who are at the RMH for their 1 year follow-up. Lauren was so adorable, running around all over the place. It was great to meet them!

We're very proud of Gracie. She's so strong and she's doing very very well. Thanks (as always) for the many prayers and well wishes!

Day +41. Choking Down the Meds

Today (wednesday) was day +41 already. Can you believe it? I'm shocked by how time can simultaneously pass so fast and so slowly!

Today was an above average day. Because Gracie has been doing so well, she doesn't have to go to the clinic every day. She goes every other day for a check up and to have all of her labs done. But today was a "day off."

Unfortunately, even on days off, Gracie has a full schedule of meds to take. She is surprisingly good at taking her oral meds. After a couple of day of fussing, she seems to be ok with them now. Keeping them down is another issue altogether. I'd estimate that she gets sick around 20% of the time. We are doing our best to track when and what she vomits, so we can identify if there is one drug in particular that is making her sick, and also to track what she is losing.

Because it was a day off, we all took a nice walk around the neighborhood this afternoon. It was so nice to just relax and enjoy the beautiful weather. Hopefully Sarah and Gracie will be able to find time to get out like that more frequently.

Tomorrow, Day +42, is a big day at the clinic. We meet with some of the top Hurler docs for an update and to discuss our progress. Please keep us in your thoughts! I'll be sure we fill you in asap after the appointments.

OH! Also, our buddy Rylie came back to the RMH today!!! Congrats and welcome home! I'm sure we'll be seeing you around the mess hall. Hmmm. Maybe a bingo date tomorrow if you are feeling up for it. :)

Wednesday, June 18, 2008

In Essence, A Little Kid

I heard through the family grapevine today that Gracie took a tumble off the couch in her suite in RMH. (The story ends happily--she's totally fine.)

My first response was panic (like it was when my babies fell off things when they were little and fragile, only in hyperspeed). Was her line tugged? Did it cause a bleed? Was the carpet dusty? What else could go wrong?

But after I heard that she was OK, all I could do was smile. What a refreshingly regular-kid kind of problem to have! After all the hair loss and vomiting and WBC counts and endless medication, how perfectly normal to hold and comfort a child who has suffered a run-of-the-mill bump (not that anything is run-of-the-mill when you're in a condition like Gracie's--make no mistake).

Then I laughed to myself about all the little tumbles and scrapes and headaches and heartaches that are ahead of Gracie, now that she gets to grow up. She's out of the transplant unit, and now she will become, in tiny increments, a little kid.

Gracie, you're so miraculous, you make even everyday miracles sparkle. What a tremendous new kind of love you bring out in everyone who knows you. Just like the Grinch, my heart grew three sizes today.

xoxo to all,
Aunt hys.

Tuesday, June 17, 2008

Weird Factoid

Just a quickie--

I forgot to mention last night in the medication rundown that a totally predictable, but nevertheless weird thing has happened since Gracie's transplant.

Dude. She has a new blood type!

She was, well, something different (Noni didn't remember what when we were talking about it) and now her blood type is O-, a rare-ish type no one in our family has yet come up with, but which happens to be the universally accepted perfect blood donor type. O- blood has no surface antigens to cause incompatibility, so anyone can receive it, which could have something to do with its being the blood type of Gracie's donor. ABO and rH matching are easy with O- donors!

Anyway, just an aside, but I got a little geeked about it. I mean, how many people get to say they switched blood types? How crazy is that?!

Monday, June 16, 2008

Not Enough Hours...

Hi there! Just a quick heads up that Gracie is doing well. She had ERT today, so it was a long day. They did see Dr. Orchard very briefly, who agreed with the dermatologist that the rash on Gracie's stomach is not a symptom of GVHD. He disagreed about eczema though. He thinks it's just hives of some sort.

Gracie doesn't love her little mask, now that she has to wear it for longer periods of time. It just looks uncomfortable, honestly. It's so big that is almost covers her eyes... Oh well.

I got to experience Sarah's personal hell today with the medication schedule. There are so many doses to give. The IV meds take an hour each to run. Basically, it's all she has time to do, and still can't get a good night's sleep! It's hard to imagine squeezing daily clinic visits in. Also, no word on how PT, OT, and Speech will all fit into the post-discharge routine. Add in to the mix the elevated risk of a mishap or infection on the outside, and its almost better to be stuck in that tiny room a little longer. But she is out, that's what matters most right now. We'll get the hang of the meds schedule, but just a warning to Noni to get ready to work. :)

That's all for now. Numbers were great again today. WBC = 6.something and ANC = 4.something, as you can see on the nifty chart on the right of the web page that Daddy made. Thanks daddy! Pray for Rylie (our buddy from down the hall) to keep making improvements. We want her out soon so Gracie will have her buddy at the RMH.

Sunday, June 15, 2008

Remote Update: Mommy's To-Do List Too Long for Blogger Today!

Aunt Hys here, standing in for Gracie's Mom, who's been released from the hospital. This comes with great news and busy news.

The great news: Hey! They're out! Who can argue with that??

The busy news: Gracie's mom has racked up some new responsibilities which, before Saturday, were held by the flotilla of nurses who moved in and out of Gracie's hospital room at better than two hour intervals.

As you can imagine, this is a full-time job on a triple shift. The best I can do from far, far away with my oft-mentioned plague-ridden babies is to pinch-blog, so here I am.

Gracie's doing well, by any standard. Her white cell count was 10.1 (well into the normal range! yay!) on Saturday. No neutrophil count was available when I talked to Sarah, but it seemed not to be of too much concern, as the total count was so nice and high. She still goes to the hospital daily for clinic, during which her blood is drawn to test for any need for blood product (whole blood or platelets), and she still receives ERT weekly, at least for the next three weeks. After that, Gracie's blood will be tested to see if she's producing enzyme on her own yet. As her need for blood transfusions diminishes (which it will, as she is better able to produce her own cells), her clinic visits will be less frequent, and she will begin looking toward home (big, BIG yay!).

In the meantime, Gracie's medication schedule is enough to make your head spin. Here's a quick rundown:

Mucomyst: 4x daily (2am, 8am, 2pm 8pm) given by IV infusion over 1 hr. each dose (!) through her central line.

This medication is normally given as a mucus thinning agent for people with pulmonary problems whose mucous secretions are too thick or otherwise abnormal. For this use, it's given as an aerosol through a nebulizer, and is inhaled. It has another indication, specifically for reversing liver toxicity due to acetominophen overdose. For this, it's given orally or intravenously. I don't actually know, but I will speculate that Mucomyst is on Gracie's menu to combat hepatotoxicity the rest of her drug regimen. Anyone with a hard answer to this one, please pipe up!

Another side note on Mucomyst: the preferred way to give it to Hurler patients is in an oral suspension. Unfortunately, it's a sulfa drug and, as promised, smells and tastes nasty like a rotten egg. Gross! So the poor patient, who's so bulldozed by chemotherapy that she can barely swallow drops of water without barfing, is expected to swallow 4 MILLILITERS of rotten egg syrup EVERY SIX HOURS! Gracie said, in her inimtiably effable way, "Hell-to-the-NO!" So she, like over 60% of her cohort, is getting it through the central line.

Um, thank goodness.

Vancomycin: 1x daily IV through her central line, antibiotic for the enterococcus she picked up in the hospital.

All the rest of Gracie's meds are given orally. They are:

Bumex: 3x daily, loop diuretic

The diuretic is to help her get her water balance back on track, after hammering her kidneys with chemotherapy.

Cyclosporine: 2x daily, anti-inflammatory. This is to combat Graft-versus-Host Disease (GVHD) which is a systemic inflammation problem that happens when the new graft attacks the body cells of the patient. So far, Gracie hasn't had any trouble with this one.

MMF (mycophenylate mofatil): 2x daily, another anti-rejection drug, given in conjunction with cyclosporine to suppress immune system activity, or inflammation.

Protonix: 1x daily, proton pump inhibitor. This is a tummy tamer. Usually given to patients with gastroesophageal reflux disease (chronic heartburn), it reduces acidity in the upper GI tract, and makes your tummy feel better. A good thing for someone whose food is bypassing her stomach (she is fed intravenously), but who still suffers from nausea and vomiting.

Fluconazole: 2x daily, antifungal. This prevents the growth of fungus in her central line. Because she receives food , including plenty of fatty substances, through this line, there is always reason to worry about infection here, and the antifungal is precautionary.

Bactim: 2x weekly (Mon., Tues.), antibiotic. Similar to the fluconazole, this is a precautionary drug, as there is a good deal of infection risk associated with TPN (IV feeding).

Ativan: mild sedative, given as needed for the general discomfort you would imagine is associated with all this health care :-)

Zofran: anti-nausea, given as needed to combat the nausea that is commonly associated with chemotherapy and other drug therapies.Interestingly, Gracie is most nauseated in the morning, like many pregnant women, I'm guessing because her sleeping metabolism is slowed, and her drugs are not cleared as as rapidly as when she is awake.

So, if you were wondering why Sarah never has time lately for blogging...

Thanks to everyone for checking in. We love to read all your comments and guestbook notes. Gracie, and all the rest of us, can't tell you enough how much we appreciate all your thoughts, prayers, kind words and every other kind of support you have given. We are one lucky family to be blessed with all of you!

Friday, June 13, 2008

Day +36- Free at last, free at last...

Thank God Almighty, we're free at last!!!!

You read it right folks! We are out of the hospital and officially in residence at our room in the RMH! Wooooooooooohooooooooooooo!

Gracie was so excited to leave we put her in the stroller and put her mask on and she didn't whine a bit! She knew she was leaving and that she needed the mask to do it.

As we left the room, we went by the nurses station and got a warm farewell from our new friends and then we headed out the big double doors of 5D as a family for the first time in almost 2 months. What an exhilarating feeling. Gracie never stopped looking around at all of the new sights... from the hallway to the elevator to the lobby and then outside- she was definately taking everything in. She didn't even mind the bright sunshine... I think she really enjoyed it.

We made it back to the RMH and then up to our room where Gracie is allowed to remove her mask. She was so excited to be in a new place and to be untethered from her lines that she just wore herself out immediately. So now, an hour after being discharged, Gracie is sacked out in her new pack 'n play and getting some good rest.

Its time for dinner here at the RMH, so I am going to head downstairs and pick up some plates and bring them back up. I'll try to post more later.

Thank you all again for your prayers and loving support.

Thursday, June 12, 2008

Quick Update - Day +35

Quick Update -

I think it's +35 today, could be +36.... I just spoke with Jimmy. The bacterial infection is not resistant to Vancomycin (VRE), so the docs are putting Gracie back on Vancomycin as the antibiotic to fight the infection. This is GREAT news. Uncle Mack was very scared of VRE. Lets hope she gets rid it quickly! They definitely won't be discharged to RMH until the infection is history!

Counts are a little down, but still unbelievable - white blood cell = 5.9 neutrophils - 3.1 Not too shabby. GROW! GROW! GROW!

In 15 minutes, Gracie will have no cords or tubes attached to her, and she won't be connected to any equipment. None! Just for a few hours, but still it will be the first time she has been "untethered" since this all started. Congrats!

In other great news. Rylie, our Hurler friend in 512 is also in discharge talks. Hopefully, she and Gracie will both be chillin' at the RMH in their little masks. I hear baby blue is the new black, so they will be looking fly!

Later taters.

Happy 11 Months... Day +34

Yesterday (6/11) marked Gracie's 11 month birthday. Happy 11 months, Precious Girl!

Also we're at Day +34. Gracie's WBC count was higher than ever at 6.4 and her Neutrophils were holding steady at 3.7. Being above 6.0 means that Gracie is actually on the low end of the normal scale for WBC. We are so excited about that.

You might have noticed the nifty graph that Daddy made and added to the sidebar of the blog so that our loyal fans can follow Gracie's progress visually. If you look close, you can see the WBC line in the normal (green) zone. Woohoo!

As for the infection... it appears to not be a contaminated sample- its the real thing and they're calling it Enterococcus. There are several strains of Enterococcus, some of which can be resistant to Vancomycin, the strong antibiotic drug that the docs here use to treat infections. As of right now, we have no idea what strain Gracie might have. So as a precaution, Gracie has been switched to another powerful drug (the name of it escapes me now) to combat this bug just in case it is Vancomycin-Resistant Enterococcus (VRE). VRE can be a very nasty and scary critter when it comes to the immuno-suppressed, so we are a little nervous. On the not so dim side, so far our second blood culture has come back negative, so there's a chance that we got a weenie version that is not VRE and it was zapped with the medicine that is generally given (hmmm... could this be another prime opportunity for a biology cheat sheet post?). We can only wait and see over the next day or so to find out. I am extra thankful for our high WBC count right about now, let me tell ya.

This does affect our prospects for discharge this week though, and I have to admit that I am pretty bummed about that. However, the docs are pretty optimistic that we can get a handle on this bug and be ready for release by Sunday. Whether or not we can actually be released then is still up in the air. Sunday is the ONE DAY that Gracie and I will not have any family support here in Minneapolis. Uncle Mack returns on Monday, followed by Noni on Tuesday so we might have to wait until then. We have one more ace up our sleeve in the form of our wonderful Care Partner Volunteers, Marty and Lisa... so if I really, REALLY want to get out, I can. We'll just have to see what the docs tell us over the next day or 2. In the meantime, we'll be working on getting the room at the RMH clean as a whistle for Gracie's impending arrival. I know she will be glad to get out of that little room and start settling into a different routine.

Once released, we'll be visiting the BMT clinic daily for lab draws, and if she needs a blood or platelet transfusion she'll receive it there. We'll also have scheduled appointments with Physical, Occupational and Speech Therapy, so we will be plenty busy while we wait out the rest of our 100 days.

Thank you all for your positive thoughts and prayers. Keep them coming- we definitely need them!

xoxo.

Tuesday, June 10, 2008

Quick Prayers needed,,,

Looks like one of Gracie's blood cultures might be positive for an infection. We're hoping that its a mistake or contaminated sample, so they just re-took the sample again and we should hear tomorrow if there's something there. Please say a prayer against any infection for Gracie.

Much Love.

Day +33 - the good, the bad, and the ugly

The good...
So we're at Day +33 today and Gracie's counts are looking awesome! Her WBC is 5.6 and her Neutrophils are at 3.7. Fantastic numbers!!! She's been consistently good... so much so that Jimmy and I have a meeting today with the Nurse Coordinator for our discharge instructions. Woot!!!

The bad...
Last night Gracie spiked a tiny fever of 100.5 which will in turn delay our discharge for 48 hours while we wait to see if she has an infection. At best, we could be discharged on Friday... but if it doesn't work out then, we'll wait until Monday so that Mommy doesn't have to try to move out of the hospital by herself after accumulating almost 2 months worth of stuff. Yikes.

The ugly...
Gracie's rash is looking more and more icky. The dermatologist just came in and said that it doesn't look at all like GVHD or a drug rash which were our buggest concerns. His opinion is that it looks to him like eczema. The funny thing is that eczema is usually family related, but neither Jimmy or I have family histories of eczema at all. However, our donor could possibly have a history of it in her family... and now that Gracie is fully engrafted with donor cells, it is much more likely that any issues of this nature would come from them, not us. Interesting, isn't it?

Also in the ugly category is that we learned today that Gracie has a virus. This is particularly disturbing news when we are talking about an immune compromised baby. The virus she has is HHV6 which is the virus that causes Roseola in children. Gracie has only a tiny level of this virus in her body, and the doctors are not concerned about it at all. They expect that her awesome WBC's will find that piddly little virus and take care of it. :) Luckily, it will not delay our discharge any further.

So, pray for no more fevers and relief from our itchy skin problem. Hopefully we'll be back at the RMH before our Daddy goes home. Here's hoping he's our good luck charm. :)

xoxo.

Sunday, June 8, 2008

Holy Moly - Day +30!!

Hey everyone,

I hope all is well. The last couple of days have been relatively good. Gracie has been pretty tired, but when she is up she is happy and playing. She has three different rashes on three areas of her body, but the docs don't think any of them are GVHD related. She has rashes on her knees and thighs, that are strangely warm to the touch. She has a hive-looking rash on her tummy, and her skin is still a mess up near her central line insertion site. She's really itchy, so we're giving her benadryl to help with that, and the docs prescribed a strong steroid cream today. The Ativan also help keep her mind off of itching. On the bright side, she has no diaper rash...

Gracie's numbers dipped a little earlier in the week, but seem to be bouncing back. Her white blood cell count is 4.0 (up from 2.6), and her neutrophil count is 1.7 (up from 1.0). Her kidney function levels have been holding steady between .60 and .65. Her weight has held steady at 11.4 Kg for the last several days as well. That's still high compared to the 9.8 Kg upon admission, but but we're happy that it seems to be stable.

In other news, a couple of Gracie's nurses have near perfected getting her to take her meds orally. It's a little disturbing to watch them do it, but hey, it works. I suppose you can't expect her to take them voluntarily. I don't think I voluntarily took medicine when I was 11 years old, much less 11 months. So that's a big step toward getting out of the hospital. If we keep it up, she'll escape without a naso-gastral (NG) tube which will make mommy very happy.

Speaking of making mommy happy, Jimmy arrived this afternoon. Gracie was so happy to see her daddy. I know they will enjoy the next week together, and hopefully he will bring some good energy to promote "white" cell growth. Good pun, eh?

One last bit of good news, Uncle Mack has nearly reached perfection in Nintendo Wii tennis. My rating is 1895 out of 2000. That Wii is a sanity saver. Keeps us occupied while the baby is sleeping. I'm also a pro golfer, and Sarah is a pro and gold medalist bowler. :)

Hard to believe that today was Day +30!! Sometimes it feels like a week since this started, sometimes it feels like a year. Do us a favor and keep on chanting GROW! GROW! GROW!

Thursday, June 5, 2008

Day +28 - another 100 for our Gracie...

...and after a week of agonzing waiting we learned today that Gracie is 100% engrafted with donor cells!!!! Thank you, Lord!

Her counts are still really good... WBC: 3.0 and Neutrophils: 2.2. Her creatinine levels are down too which is really good and she lost a little more weight today. SHe had kind of a rough night last night though. She woke up a lot and I think its because she didn't get a good enough dose of the Ativan when she went to bed. And unfortunately, she can only get it every 6 hours so we had to make the best of it for a few hours before I felt comfortable enough to give her a little bump of her pain med.

She's been making up for it today with lots of extra sleeping, although she was up and awake enough this morning to do a great session with Speech Therapy before her docs came in with all of our great news! She showed off for them as well and they were really happy to se her feeling good.

We're going to attempt to do some more oral meds starting tonight, so please pray that she transitons into that better with the help of the Ativan. We'll just have to see, but I will definately keep you all posted on our progress as always. :)

Thank you all so much for all of your prayers and good energy... it apparent how well it is working as Gracie improves everyday. We love you all so much and appreciate your constant and unwavering support.

Day +27 - Night Time Quickie

Just a quick update for now. Gracie had a much better day today, so I'd expect her counts to be a little better tomorrow (hope, hope - GROW baby GROW!). She played almost all day. We had PT in the afternoon and the therapist told me that today was the best PT session Gracie has had. She was standing up pretty much on her own, and she got on her hands and knees for a minute or two, which sounds like a new thing.

I think that we were able to isolate what has been bothering Gracie so much the past several days. After some observation, she gets really upset after each time she gags or vomits, and after each time she poops. AND she is a little poop machine. every diaper has poop in it. So she is upset a lot. Clearly, she is having some stomach or other GI tract discomfort.

Turns out that her existing pain meds don't really help stomach issues, so they started her on Ativan, which is sort of like valium. The doctors hope that the drug will keep her stomach from bothering her as much. Lemme tell you... it worked! That was one happy baby after the drug kicked in. Of course, she might've been a teeny bit high, but it was just so great to see her smiling, laughing, putting stuff in her mouth. Turns out a lot of other kids take this as well.

Hopefully we'll get our first engraftment results tomorrow. Keep GROW GROW GROW in your thoughts and prayers. :)

Uncle Mack

Wednesday, June 4, 2008

Day +27 - Early Update

Hi everyone. Just a very quick update. I just spoke with Sarah and she said that Gracie is feeling a little better today. She has been up and playing, and had her OT this morning as well. Let's hope that she keeps feeling better.

Her counts are also still OK. Her WBC count went up to 3.1, but the Neutrophils went down to 1.2. This is still well within expected range. I'm sure the counts will bounce back even more as days go on. Her creatinine clearance ticked up to .83. Not surprising to me, since they gave her a one-time boost with a turbo-diuretic to get her to pee. Her weight is still up, but has been holding steady at least.

One piece of good news that Dr. Orchard shared yesterday while we went through numbers is that Gracie is starting to produce red blood cells on her own. I don't recall why exactly this is a good sign, but I'll look it up. :) Sorry!

Anyway, just thought I'd pass along some better news early in the day.

Uncle Mack

PS - GROW! GROW! GROW! GROW!

Day +26 - A Tough One

Gracie just didn't feel well today. She was fussy all day long. She slept a good bit, and played a good bit, but it's clear she is uncomfortable, and not at the top of her game. She needed a few extra bumps of her pain meds to keep her comfortable.

She still vomits sometimes, and doesn't really want anything near her mouth. However, she loves to "brush her teeth." We clean her mouth with these little pink spongy swabs soaked in saline. She gets three swabs about 3-4 times daily. If she had teeth, her dentist would be so proud! It's so cute to watch... awww.

Numbers Update:
It figures that as soon as I show off by posting the critical numbers and Gracie's perfect report card, the white cell counts go down. Today, Gracie's white cell count was 2.7, and her Neutrophils were 1.7 (I think). Her creatinine clearance continued to go down, today at .73. I'll try to update the graphs and post about once a week as well.

At the end of the night, we called the resident in to ask about a few things, most notably some red bumpy rash looking stuff on her knees, and some skin peeling off of her hands. It could be early GVHD and it could be nothing. The resident sat through all of our questions and the answer to most was that she'd discuss it in the AM with the attending(s), which is a fine response because hopefully that means the doc will have thought about it prior to coming in for rounds in the AM.

Dr. Orchard and Teresa Kivisto, the primary doc and nurse running the Hurler patient studies, stopped by today to check in on us. It was great to see them. Dr. Orchard assured us that fluctuation in the cell count numbers is expected, and also noted that Gracie's counts are still relatively high which eased much of our worry. They also said that we have "an impressive blog." At first I was a little surprised and nervous that they read it, but then I was happy they do. It's a great way for them to keep up to date on Gracie's overall progress beyond just the numbers. Plus, we just post the truth as we experience it. :)

Today was a tough day. Seems like when Gracie has a bad day, we all do. By the end of the night, Uncle Mack was so crabby!!! Here's hoping that we all feel better tomorrow - especially Grace. Good night everyone.

Tuesday, June 3, 2008

Following Gracie's counts

Just below are a couple of charts so you can see Gracie's progress over time. Also, if you haven't seen the video if Gracie's trip down the hall, scroll down to the Day +25 post.

On the white blood cell chart, it's important to note that the drop after Day +19 is due to the doctors taking Gracie off of growth hormones on Day +17 because her counts were so good, to see if she would start to grow them on her own. Neutrophils are the most important type of white blood cell, so I've highlighted them in blue.






On the Kidney function chart, you see the levels spike when they put her back on the diuretic after taking her off a bit too early (she puffed up a lot). You see it starting to go back down as they've lowered her dose on the diuretic and other medicines, and slightly increased her maintenance fluid levels, which dilute all of the drugs going in.

It's easy to see the dramatic impact that seemingly small changes have on these numbers, as well as the 2 pages of other numbers we are following. It's a delicate balance to keep everything in check.

Another Florida Hurler Baby

Today I learned of another little member of the Florida Hurler Club. He is an adorable little 8 month old boy named Liam. He recently started ERT. Here is the link to his blog. There was also a TV news story on a St. Pete television station about Liam.

http://www.caringbridge.org/visit/liamlarrow

Monday, June 2, 2008

Day +25... Out and About!

So today was a pretty good day for Gracie. Her counts are up again today. WBC is 3.8 and Neutrophils are 3.0!!! Great numbers again! Her kidney function is slowly improving as well so we're feeling pretty good with our current plan. We changed the dressing over Gracie's new line and she did so well that I thought that she deserved a special prize... a trip out of the room and for a ride in the wagon around the unit hallway!!! Whee! I got a it on video and decided to post a snippet of it here for all of our loved ones to see. Sorry for the crappy camera-work. Mommy had to pull the wagon and videotape it and I am obviously not so good at multitasking. :) Enjoy!
video

Mommy is a slacker.

Thanks again to Uncle Mack for catching up on the posting the past few days. Mommy has definately been a bit busy and stressed these past few days... and man, do my feet hurt! Thats the trade-off with the crib sleeping.... lots of standing next to the crib for playtime, diapertime, and everything else.

So, t-minus 6 days until our Daddy is back in Minneapolis, and let me tell you... we can hardly wait to see him! It will definately give Mommy a chance at a good break from the hospital night shift, not to mention the fact that Gracie and I both miss him like crazy in general!!! Hurry Daddy, hurry!!!

Ok, so to help Uncle Mack out, I believe that Gracie's counts for today were WBC 3.4 and Neutrophils at 2.7. Very nice numbers.

Her skin around her line is all torn up again... common at this stage since we're entering a phas of extreme skin sensitivity. I feel so badly for her because it obviously is causing her some pain. :( Here's hoping it clears up pretty soon. Our (non-prickly) resident Erin looked at it this afternoon and suggested we get back on the pain meds for a day or so and then came back by our room to bid us farewell as her rotation here on Unit 5-D is up as of tonight. I'll tell you what... we will really miss her.

We're also keeping an eye on Gracie's kidney function right now because they seem to be taking a beating. We're hoping to find the right balance between fluid retention and diuretics and regulation of some of the immune suppressing drug which can be hard on the kidneys as well. I think we're on the right track and hopefully we'll find our sweet spot. Pray that we get there soon.

Other than that, Gracie seems to be feeling ok most of the time. :) I am so thankful to see her smiling so much these days.

Sunday, June 1, 2008

Day +24 - Quick Update

Hey guys,

Welcome to June! Crazy, eh? Things in Minneapolis are ok. The weather is absolutely gorgeous today. Of course, yesterday we heard tornado air horns. :) Yesterday was still pretty hard. Gracie doesn't really like her new line placement and really hates the dressing changes - which need to be more frequent due to the new line and increased infection risk. With just me and Sarah here this week, it'll be tough for Sarah, because she essentially can't leave the hospital for more than an hour or two to get cleaned up and unwind. :( I can do a lot and stick it out for a while, but I'm just not Noni. Pray for Sarah's continued good health.

Sarah said the the interventional radiologist stopped by yesterday to apologize about the PACU experience. Not sure if you say testdriver's comment on yesterday's posting, but it was all 100% true. The solution to this problem was easy - just communicate better and do what s best for both Sarah and Grace.

The apology was a nice gesture, and I'm sure it wasn't easy for him to come up and do that. I guess that means I'll need to find a new main course to go with my side of prickly resident. I'd go with fried Zelda, but she was far too sour for my taste. Even a good batter and grease wouldn't change that. :) Let's just hope we don't need to go through anything like that again.

Gracie numbers continue to climb. I don't recall the exact numbers but will get back to you. I just know they are higher than the other day.

OH! I forgot to tell you... Given Sarah's new tough mom, take no shit attitude with the hospital staff, she took another step yesterday... Not sure if you recall this, but at the outset of her hospital admission, Sarah got to observe different staff members on the BMT unit, and pick a primary team of nurses to take care of Gracie. While she knew that we would be dealing with other nurses too, when the chosen nurses are working, they are supposed to work with Gracie.

Well, for the past two nights, one of Sarah's chosen nurses (Holly) has been on the unit but working with other patients. Sarah talked to Holly, who really wants to work with Gracie too, but has been assigned to other patients. Well, last night, Sarah had a little chat with the charge nurse who sets the schedule and assigns patients. In short, she reminded the charge nurse that she went through the process of choosing a team for a reason, and wants to be sure that the unit management is following her wishes. Go Sarah!!!!

Anyway, while we still kept the same nurse last night, Holly stopped by and said that Sarah did the right thing, and that the charge nurse put a note by Gracie's name saying, "Insists on consistent nursing team" so the rest of the managers will see it. The nurse we've had the last two nights is great, so not a huge problem. But still, so proud of Sarah for sticking up for herself and Gracie.

The other change is that Gracie is chillin in the crib now. Belooking for Gracie's MTV Cribs episode soon - She'll show off her tower of IV pumps and tubes, her collection of binkies, the home-made bumpers in the crib, and of course her big screen plasma monitors. Good news is that means there is room for a roll-away cot for Sarah. Bad news is that you spend every waking moment standing up and reaching into the damn crib. My back is killing me, I know Sarah's must be worse...

By the way, congrats to our friend Rylie down the hall on unit 5-D, who just got her engraftment test results the other day and was 100% engrafted with the new cells! Yay Rylie! Keep up the good fight, sugar booger! We are praying for the same success when we get results early this week. :)

OK, clearly uncle mack isn't capable of a "quick" update. :)