Tuesday, November 1, 2005 - Day + 124
Rest assured, Frankie is feeling much better. We're not sure what was causing the vomiting, but thankfully, Frankie has been vomit-free for two days! Frank & I think it was something we fed him on Friday night that didn't sit very well with him. But, we also started two new medications and dropped the dose of another, so with all of these variables, it's hard to say. Tomorrow we go to clinic, and we'll definitely ask about that. Plus, his belly is still big and tight.

Halloween went by like any other day. We really couldn't open the door to trick-or-treaters and we couldn't bring Frankie around to other people's homes. Fortunately, he's still young enough not to know what he's missing. And, trust me, Frank & I are probably better for it, since we'd be the ones eating the candy!

Today was pretty casual. Frankie had physical therapy outside where the slides are. He was entertained for a few minutes before realizing that it was work and not fun. We tried to get him to kick his ball on the tennis court, but he caught on too quick and didn't even make it past the gates. Oh well. He does everything on his own terms. Sometimes, we can force him, but other times, it's not worth the fight. We just hope for a good twenty-five minutes of PT... the other twenty minutes can be working his lungs (screaming in protest).

Wednesday, November 2, 2005 - Day + 125
Finally, I think we may be back on track with Frankie's sleeping/napping schedule. Even though he still wakes up in the middle of the night for a diaper change, he slept until 6am today, which was a Godsend. I know that may sound early for some, but compared to 3am, it's heaven.

Today's visit to the clinic was uneventful. Frankie's still on track with his "protocol." What this means is that he's doing everything the doctors were hoping to expect. It's a good thing. We're still waiting on the results of his enzyme test, the GAGS test and are continuing to wean off the blood pressure medications.

After our quick visit to the clinic today (one medication that totaled around 1/2 hour), we stopped by the unit to say "hi" to Jen, Kate and Alison. Kate was readmitted on Monday. And, while I'm sure it's not the greatest feeling in the world to go back to 5200, Kate's getting great care and Alison has a load off! Please say prayers for Kate!! She's actually in Frankie's old room on the unit. And, some of our window decorations are still up! Hopefully, they cheer Kate up as much as they did for Frankie.

Back at the apartment, we just relaxed. My headache has arrived, and I'm not much help while it's kicking my butt. So, Frankie and I napped right when we got back for about two hours! I love my boy!!!

Wednesday, November 9, 2005 - Day + 132
Again, I realize it's been a week since my last update. There are two reasons for this. One, not much has changed. Two, my connection to the internet is not predictable. The disadvantage to this is that I don't get to read up on my friends and family links to the right and usually find out about things a few days after they happen.

Unfortunately, I have to report that Maddie Trimper lost her fight on Monday, November 7th. I received an email from a friend of mine in Stanton, Michigan, where the Trimpers live. Please say prayers for Maddie and her family during this most difficult time. Her link is to the right, The Trimper Family.

Clinic today was very good. We got to the clinic the earliest we have ever done (9:20am). And, after two medications, the normal doctor visit/comments, and Speech Therapy, we were out the door at 12:30pm. Not bad! On our way out, we ran into Alison and Kate! It was fabulous to see them because that meant they were discharged from the unit, and Kate looked amazing! Way to go, girl!

As for our clinic visit specifics, not much changed other than dropping another blood pressure medication. We have to go to the clinic on Friday for a BP check, to make sure it wasn't dropped prematurely. But, we're going to be at the clinic on Friday anyway because Frankie needs a CT Scan of his brain to check the blood, again. The doctors just want to make sure it's not getting bigger.

One of the test results were were waiting on did come back today. This was the GAGS test. It measures the level of heparan sulfate in Frankie's urine (to be blunt). The lower the numbers, the better. For those of you who may be interested, and more to the point, understand the test, Frankie's numbers, pre-transplant were as follows:

mg uronic acid (carbazole reaction)/gm creatinine = 305
mg uronic acid (orcinol reaction)/gm creatinine = 76
mg N-sulfated hexosamine (MBTH method)/gm creatinine = 76.

The latest test showed levels of 142, 38, 30, respectively (over 50% reduction)!!! This is really great news. However, the 142 level is still abnormally high. Hopefully, when we have the test redone in December, for Frankie's six month studies, the number will be even lower.

We are still waiting on the test that measures Frankie's immune system. But, the test that's run for the 100 day studies, is really just done as a marker. The doctors do not expect to see any huge signs of T-cell growth. They expect more signs of T-cells when the test is run for the six month studies... so again, we wait for December!

Friday, November 11, 2005 - Day + 134
Earlier this week, another former 5200 resident lost his fight. Lane Hancock earned his angel wings on Thursday. His mom, Diane, was one of the first three moms Frank and I met on the unit. We were enticed to attend a family counseling session the first Thursday we were on the unit. There we met Diane, Mary (Michael's mom) and Viesta (Frannie's mom). It was a great session. And, for those parents on the unit reading this right now, I encourage you to go to at least one session... if only for the awesome brownies (not catered by the hospital, but rather by an outside catering company, if that helps any)!!

I never put Lane's or some other children's links to the right due to the fact that I didn't have a chance to ask permission. But, I'm realizing now that maybe I can make a few exceptions. I hope the families won't mind. I just want everyone who reads this web site to be aware of just how many children and families have touched our lives while here in Durham. This weekend, I'll update the links to the right.

That being said, there's another couple of children and families Frank and I met along the way, that are in need of major healing prayers. Joseph Brown and Noelle Naylor are in need of your prayers and good thoughts. Noelle was discharged, but recently readmitted to 5200, due to some complications. And, Joseph was admitted to 5200 shortly after Frankie, and is still there. His lungs, kidneys and marrow need healing.

On Wednesday's clinic visit, we met another Sanfilippo family. Annabelle Green is a beautiful little girl from Idaho that was transplanted over a year ago. She is back at Duke for a couple of weeks with her mom, younger sister and grandmother.

And, through an email, I've been introduced to another Sanfilippo family, the Esparza's. Their little girl, Graciela, was diagnosed with Sanfilippo Type A the same month both Frankie and Jessie were diagnosed! They have just gotten to Duke for Graciela's transplant.

Please say prayers or pass along good, happy, strong, and GROWING thoughts to all of these children. It is amazing how much this type of positive energy helps the children and their families.

Tell your children you love them EVERY DAY! Give them hugs, kisses and let them know just how much you love them by your ACTIONS as well as your WORDS. Enjoy each passing moment, smile, giggle, and glint in their eyes. I know I do.

Friday, November 18, 2005 - Day + 141
Sorry for the lack of updates. Rest assured, things have been fine for Frankie. I will update his info later. In the meantime, please say prayers for Joseph Brown's family. Joseph earned his angel wings on Wednesday, November 16th. I can just imagine Joseph and AJ playing up there in the heavens, laughing and smiling, watching over all of their family and friends down here.

Please continue to say prayers and send good thoughts to all of these children and their families. It's amazing how much they really appreciate every single prayer they get.

I have a few more web links to add, but lost my notepad I wrote them on. Please say prayers for another couple of children: Kaleb, Graciella, and Annabelle.

Much love and big hugs!

Saturday, November 19, 2005 - Day + 142
Okay... some updates from the past week....

Last Friday (November 11), Frankie had a CT Scan to check the fluid on his brain. As a recap, the doctors wanted to make sure the fluid wasn't increasing. We had to go NPO (nothing by mouth) for six hours prior to the CT Scan. So, Frankie couldn't eat past 5:30am. And, since his hunger levels have increased (to say the least), we decided to wake up at 4:30am and make a big breakfast to carry him over until his 11:30am scan.

This proved to be an excellent decision, since I didn't have to listen to my little boy cry for food for six hours... a horror story from months passed when we were at UNC. Although, we were slightly tired since Frank & I don't usually go to sleep until 2am. We arrived on time for the CT Scan, and were called to the secondary waiting area within a few minutes. Fortunately, the gentleman who was bringing us back there remembered us from a prior CT Scan, and picked up on the green mask right away (amazing how some don't). So, he put us in a quiet corner away from all of the other soon-to-be-scanned. I don't know what his name is, but he was great!

We waited probably another 30 minutes before we were called to the CT Scan room. And, since only one parent can go in with Frankie, I nominated Frank. We were expecting him to be sedated (reason for the NPO), but that didn't happen. Instead, Frankie was strapped down (head, too, which freaks me out) and screaming. He wouldn't keep still (would any 2 year old, unsedated??) so Frank had to lay on top of him, no joke, to keep him from moving. And, the way he was positioned, Frank's head kept hitting the CT Scan machine. I don't know how the technician did it, but somehow she got the scan... or at least we hoped.

Side note... yeah, no sedation, no need to wake up at 4:30am! Oh well. We just hoped the picture was going to be clear enough to analyze the size of the fluid deposit... and it was. It was undetectible... meaning it must have resolved itself because the doctors couldn't see a trace of it on the scan. Woo Hoo!

Saturday, November 12, Frank, Frankie and I stopped in on the unit. Frankie hadn't been there since we were discharged, so I was curious how he would react. He did okay, but really wanted mommy (me) to hold him most of the time we were there.

We got to see Noelle, who at the time had been readmitted to Frankie's old room. She's out now, and things are looking better for her. Thanks to everyone who is continuing to say their prayers for her!!

We also got to see Courtney, Jen, Michelle, Andre (Frankie is still unsure of him)!!! It was actually funny to see that Frankie's reaction hasn't changed when he hears and/or sees Andre! I think he's the only child who has never warmed up to him. Sorry, Andre!

We met another few children and their families: Kaleb, Graciella, and Elijah (spelling??). Please keep these children in your prayers as well. They are absolute cuties! I believe they are all around 2-3 years of age.

Sunday through Wednesday were pretty much uneventful on the homefront other than having lots of visitors (one at a time, though). Frankie got to visit with Jen, Kate, Melody and Chris before the weekend was up! But, folks, to be honest, I'm still living in a perpetual Saturday, so every day is the weekend right now.

On Wedneday, November 16, we went to clinic for Frankie's weekly meds. He had his monthly Benedryl with a Pentamadine chaser, so he was a bit sleepy the rest of the day. It's really amazing to watch the effect of Benedryl when it goes through the IV tube. It's basically instantaneous! A little push of the fluid, and he's out! Sometimes, that would be nice when Frankie wakes up at 3:00am! ugh.

Yesterday afternoon, November 18, we received a call from our lovely Nurse Practioner, Jerelyn. Unfortunately, Frankie's CMV levels have popped up again. She said not to be alarmed, but to increase the frequency of his antiviral medication (Tacrolimus). She also said that during a discussion with the endocrinologists, that they're concerned with his cortizol levels (self-producing steroid levels - they are what make you hungry when you wake up in the morning and throughout the day). Frankie's levels, despite having been on two different steroids to increase his hunger and the production of cortizol, are still EXTREMELY low. So, we've added a new steroid to his repetoire... but deleted the two prior meds. For those medically inclined people who read this, he'll be taking Cortef (spelling, Chris??). Hopefully that will take care of the issue. Cortef is the medical equivalent to the cortizol in your system. And, according to Jerelyn, the endocrinologist was curious why we didn't start with that. But, I think it was because of his "protocol" and Frankie's doctors being cautious, since he hadn't been seen by the endocrinologist while we were inpatient. But, I guess we'll be seeing him/her at his 6 month studies in December.

Saturday, November 19, 2005 - Day + 142
I know there are two Saturdays... I just wanted to separate this entry from the "catching up" portion.

So, yesterday I was put to the test. Frank & I took Frankie to the store to window shop. There were two people working and two other customers that walked in behind Frank and I. So, while the gentelman who was helping us went to his computer to check on something, the older woman asked why Frankie was wearing the green mask.

Now, I think I explained myself VERY well. I told her, more than once, that my son had a transplant and that right now he has NO IMMUNE SYSTEM. So, thinking this was communciated well enough, the conversation continued. Apparently, her grandchild was in Vanderbilt Hospital in Tennessee for a transplant, but for the life of me I can't remember right now. So, here I'm thinking, "great, this woman understands!"... until she tries to touch my son! Seriously, the woman kept getting closer to me (I'm holding Frankie in my arms) and reaches out to touch him.

I don't know this woman from a hole in the wall. I don't know where she's been, if she's been exposed to a cold, sniffled before she came in the door, sneezed, coughed, etc... So, I did what any other PBMTU parent would do, I intercepted her hands with my hands and body. I'm not even sure, I may have hit her... it was all a blur. At the same time, I'm repeating to her, "My son does not have an immune system!" She backed right off and apologized. So, I'm putting it out there... is that really what it takes??? Do I have to physically protect my son from people's hands? Who thinks it's okay to touch a child that you don't know, with or without a green mask??

PBMTU parents and families beware! With winter upon us plus the warm and fuzzy holiday season approaching, people lose their minds! Protect your children and yourselves... because second to your child getting sick, is your health. You cannot risk getting sick, either!!!

Monday, November 21, 2005 - Day + 144
Okay... big news for some... WE'RE BACK IN CONNECTICUT!

And, while I'm sure people miss us and want to see us and Frankie, I have to restate something I wrote a few days ago:

Please note the following: When we are allowed home, we will still be under the same restrictions as we are here. Frankie will not be able to be around any children for at least another nine to twelve months. And, we have to limit the visits from adults, especially if we're coming home in the cold winter months. In addition to that, Frank and I will have to limit our contact with others as not to bring any colds or viruses into the house. Viruses and colds are our major concern at this point. What would cause only a runny nose for you or me, could kill our son. So, please understand that we're not being anti-social. Our son has come way too far to lose his fight to a cold.

Also, with the benefit of my friend and former nurse, Chris, and Frank, I was alerted to a "typo". An entry I made below referring to a drug to help with Frankie's CMV was incorrect. The antiviral medication that Frankie takes is Valganciclovir, not Tacrolimus. Tacrolimus is an immune suppressant that Frankie takes so his donor cells aren't fought off by his host cells. HUGE DIFFERENCE! Sorry!

Sunday, November 27, 2005 - Day + 150
Good morning, everyone! Things have been moving right along, and Frankie hasn't missed a beat since we've arrived back home.

Frank & I have been busy every day trying to unpack the thirty boxes we shipped back from Durham! How could three people accumulate that much stuff in a matter of 6 months?!!? We've moved furniture around, brought carload after carload to Good Will, and still, our house looks overrun.

Last Wednesday, Frankie had his first clinic visit at Yale. We met his new doctor and nurse practitioner and got a feel for what clinic life is going to be like here. Fortunately, Frankie had all of his IV medications the week prior, so it was just a meet-and-greet this first time. And, since I'm a creature of habit, I had given my son all of his oral medications prior to going to the clinic, only to realize later that his FK levels would be skewed. woops! So, next week, I'll be holding back that dose, having the clinic draw blood and then giving him his FK dose. For all weeks after that, I'll be doing the blood draws at home and bringing them to clinic, just like Duke. Fortunately, Yale's lab is right there, so the results come back very quickly.

The different thing about Yale's clinic is that they only have one isolation room. So, I'll be bringing my little one as early as possible to clinic each week so we can get in there first and out quickly. At least, that's my plan now.

Frank starts back to work full time tomorrow. And, in case you people still in the Durham or southern areas don't know it, we woke up to three inches of snow on Thanksgiving morning! UGH!!!



Yeah, so that's what I saw out of my front window on Turkey Day. And, I heard that it was around 60 degrees or warmer in North Carolina! I'm so jealous! But, now as I look around, the snow has melted, but it's still cold. I have a cup of hot apple cider next to my laptop as I type!

We head back to Duke for Frankie's 6 month studies the week of December 12th. It's a very jam-packed week of appointments, exams, and the normal weekly clinic squeezed in the middle. Basically, it's the same routine we went through with the work-up back in April, only this time, he's got a lumen and will not have to be poked and prodded for his blood (thank God!).

We're hoping to get to see all of our friends that have become our Duke family while we are there. I miss all of you immensely!!! I cannot tell you what an empty pit I have in my belly for missing you all so badly. Nothing against family and friends at home, but our Duke family will truly be the only ones who understand what we and Frankie are going through.

We've been meaning to say "thank you" to everyone who has crossed our path through this entire journey. And yet, those two words seem so small in comparison to the love and support that all of you have shown us.

First, we want to thank Frankie's pediatrician, Dr. Karalyn Kinsella, for without her gut instinct, we'd probably still not know that Frankie was suffering from MPS, as most children aren't diagnosed with MPS until they reach 3 years of age. And, without her recommendation of Dr. Robert Greenstein, we wouldn't have known as quickly as we did. Dr. Greenstein recommended Duke and Dr. Kurtzberg's group for treating Frankie.

We thank the insurance company, Aetna, for allowing us to go there for his initial workup in April (and subsequent treatment). Since finding out about Frankie, the insurance company, and where Frank & I work, have all been so cooperative, that we haven't needed to worry about our jobs or how we were going to afford to save our son's life.

We thank GOD for Dr. Paul Szabolcs and Frankie's nurse practitioner, Jerelyn Moffet. And, we thank GOD for the nurses that became our friends... Melody, Jenn, Chris, Shawn, Michelle. Without this incredible group of people, we don't think this journey would have been bearable. It is absolutely horrible to have to see your child go through this, but there was ABSOLUTELY NO WHERE ELSE WE'D RATHER BE.

We thank my and Frank's family, every day. For without their love, support and unbelievable fund raising efforts, we would not have both been able to stay with Frankie the entire time at Duke. For this, we are entirely grateful, beyond words. We thank our local communities, for their unquestionable kindness toward a child many of them have never met.

And, to our Duke family... my eyes well up just at the thought of all of you. You have brought Frankie, Frank and I such comfort and joy over the months, you have no idea. We have shared in successes and losses. We have cried and laughed with equal force. Without knowing, without planning, we have become a family that will forever be bonded by four simple numbers: 5 2 0 0.

We love you all! Happy Thanksgiving! Big Hugs across the miles from the DeMatteis Family of 5205.