Wednesday, June 1
8:45 we checked in at the CHC in Pediatric Radiology for the full body CT Scan (brain, sinus, chest, abdomen, and pelvis). Frankie was NPO (couldn't eat 6 hours prior to the procedure, drink 2 hours prior). However, the procedure didn't happen at 8:45... he finally had his IV put in at 11:30 and the CT Scan took merely minutes. We were in recovery by noon.

Orignially, we were to tour the Pediatric Blood and Marrow Transplant Unit after the CT Scan, but there were some scheduling conflicts, so instead we met with our social worker, Marion.

Thursday, June 2
12:00 we met with Jane, one of the Family Support Group people. We talked about services they offer on the unit as well as when Frankie's discharged, but still an outpatient. Immediately after, we met with June, our Nurse Coordinator. We had a educational session where she discussed each day, the medications Frankie will be administered and things we will have to do to keep him healthy while he's going through all of this. I tried to write notes, but I was reassured that we'd be going over this again.

It was during this meeting today that we realized that Frankie's transplant had been pushed back one week. He was scheduled to have his central line placed on Monday, June 6th, but due to a lack of room in the unit, he's scheduled to have the line placed on Monday, June 13, with chemotherapy beginning Tuesday, June 14.

A couple of things I'd like to note:
One thing Frank & I realized in a very short time was that when you're at Duke, you're there for a reason. You're not going to see your run-of-the-mill procedures. You're there because you need extra special help. You can always find someone who is worse off than you when you're sitting in a waiting room at Duke. I'm not saying this to be glum, I'm saying this so people realize that even though it's bad for us and for Frankie, it's bad for other people, too.

Special People We Met Along the Way:
A three year old little girl diagnosed with the same disorder as Frankie. She's from the Boston area. She and Frankie are basically on the same schedule for transplant. So, even though this will be rough on everyone, it's comforting knowing that there's another family to support and be supported by, if needed.
A little boy, two years old, whose family doesn't know what he may have. They've been struggling with test after test, with nothing conclusive coming back. My heart goes out to that family because not knowing can be it's own torment.
A young teenager who has been diagnosed with a disorder that the doctors give him one year to live. On the outside, he's just like any other 13 year old boy, but his brain is basically dying. His mom, incredibly strong, doesn't let him see her cry. I couldn't imagine the pain she is going through, knowing that the doctors don't know if they'll even do the procedure that could save his life. They were to find out yesterday. I hope beyond hope, they do what's best for him.

Wednesday, June 8

Well, as you may already know, we're basically in a holding pattern until there is a bed available for Frankie in the Unit. And, per a conversation last night with our transplant doctor and nurse coordinator, that date may be pushed back even further, to June 15, possibly even June 20th for the cardiac catheters and subsequent chemotherapy. This will, of course, push back the date of his actual transplant.

We won't find out until Friday, June 10th, which date he's scheduled for the cardiac catheter.

Some interesting things we found out last night during our meeting:
1. Frankie's transplant has been approved by our insurance company

2. His donor has been found - B+ blood type (which he happens to be as well) collected in 2002. There's a huge cell dose (amount of cells per kilo of his body weight)

3. The donor is a four of six match. For stem cell transplant, an exact match is not required, as in bone marrow transplants. There are six elements they look at to match the donor and the patient. They have done a transplant as low as 3 of 6 match, but most have been either 4 or 5 of 6 match.

4. Day +12 (or roughly there-about) will be his worst day. At this point in the process, his hair will begin to fall out, he will become very lethargic and lose his appetite.

Sunday, June 19 Late in the PM

Sorry for the delay in the updates. We were hoping to get a solid date back on June 10th, but things were delayed yet again. And, by the time anyone actually gets to reading this "What's New" update, it will be old news.

Frankie is scheduled for his surgery tomorrow morning, June 20th. At approximately 10am tomorrow morning, he will be getting his cardiac catheters (aka central lines) placed. The two catheters are inserted into a large vein in the chest that leads to the heart. One tube will be a single and the other will divide into two separate tubes (lumens). The catheter is soft and flexible and will remain in Frankie's chest for at least 6 months.

The purpose of the catheter is to eliminate most of the need to draw blood and give medications by needle stick. He will get his chemotherapy, nutritional fluids, blood draws and the transplant through these tubes.

Provided there is a bed available, Frankie will be admitted to the unit following the surgery tomorrow. If there isn't a bed available, there will be two other options. Option 1 is they admit Frankie to another section on the same floor. Option 2 is they send Frankie home until there is a bed available. The problem with Option 2 is that we would have to bring Frankie back to the hospital daily to have his central line flushed until there is a bed available.

If anyone is thinking about sending anything to Frankie in the hospital, keep in mind that the following items are not allowed in Frankie's room in the unit: fresh flowers, fresh plants, fruits and vegetables. The reason we cannot have these things in Frankie's room is because they harbor germs, especially mold spores. Also, no latex balloons. I'm not sure about mylar balloons.

Some other information:
Via an email from my sister, Sonia, we met another family who's 3 year old daughter has been diagnosed with MPS III Type A. Her name is Isabel, and she's a beautiful little girl. She is supposed to be arriving in Durham tomorrow for her work-up. Hopefully, we'll get a chance to meet her and her family. You can meet Isabel on her web site: www.caringbridge.org\sc\isabel

Also, I believe the other little 3 year old girl we met earlier this month has started her chemo. If I remember right, she had her central lines placed last week.

We have added these two precious girls to our prayers.

Wednesday, June 22, Late in the PM (again)

Frankie had his surgery as scheduled, June 20th. Frankie had his central lines placed in his chest at roughly 11am on Monday morning. Even as he was admitted and on the table, we still were unsure if he was going to be admitted to the unit that day. It wasn't until he was recovering from the surgery (and subsequent spinal tap) that we were able to confirm that he had a room!!! This was a tremendous load off of all of our chests. I cannot express to you how much we were dreading having to go back to the apartment that night with Frankie as an outpatient.

So, at approximately 12:30pm, Frankie was coming out of the anesthesia. And, I must say, he handled this procedure better than his last (tonsils, adenoids, PE tubes, and BSER hearing test). His oxygen saturation levels were perfect, and there was no need for an oxygen blow-by this time around.

We were admitted to the PBMTU (Pediatric Bone Marrow Transplant Unit) once he was up and drinking. The original room they had for us was not his permanent room. At approximately 8:30pm we finally were moved into his room. And, at 10:30pm he finally fell asleep. I spent the first night with him. And, for the most part, it was uneventful.

At 2am that same morning, they drew blood ("levels"), to prepare for chemotherapy. Then, at 4am Tuesday morning (yesterday), chemotherapy began. With this particular drug, he gets a drip bag at 4am, 10am, 4pm and 10pm for four days. Then it's on to another chemotherapy drug. He also has to take medication for anti-seizure, anti-nausea, and to fight infections.

Starting early in the morning, Frankie was displaying some type of reaction to what we originally thought was the medication. He was unbelievably hyper. Now, Frankie's an active child, but this was beyond anything I have ever experienced before in my life. We could not control him. He would not focus and would not stop moving around. He would get wrapped up in his lines and just go nuts, to put it plainly.

We finally came to the conclusion that he was reacting poorly to the tape used to secure his lines to his bare chest. It was very irritated and must have been driving him crazy. At 5pm, he was given a dose of Benadryl that almost immediately took effect. However, he needed another dose before bedtime, and was thereby set up to have a dose every 6 hours. The nurses also changed the type of tape they were using, so I think that helped as well.

Tuesday night was Frank's night with Frankie. And, while it took him forever to get to sleep, the nurse on duty said he slept soundly through all of her visits. But, at around 6:30am, Frank heard a bang and then a giggle. Apparently, one of the crib guards wasn't locked in the upright position, and Frankie was able to slam it down on his own. Good thing Frank's a light sleeper, unlike me!

And now, it's late on Wednesday night. And, I'm trying to keep this as current as possible. There are a lot more stories and a lot more things I could write, but I wanted to at least assure everyone that Frankie went through the surgery fine, that we were admitted into the unit and were able to get a room (which I'm decorating section by section). And, now that we have everything set up, I can update the calendar with what day it is, in relation to Transplant Day, which, by the way, is June 30th.

Saturday, June 25, DAY -5, for those of you counting down with us

Hi, everyone. Well, just to let you know, Frankie completed his first four days of one type of chemotherapy (Busulfan) and is now on another for the next four days (Cytoxan). And, I must admit, this wasn't a very good day for Frankie.

As some of you may already know, chemotherapy doesn't make anyone feel at all well. In fact, without the anti nausea drugs that they now give along side the chemotherapy, I think things would have been worse for him today.

He has stopped eating and drinking. I think he may have had three saltine crackers around 7:30 tonight. But, absolutely nothing to drink. He's vomited a couple of times today, so we pretty much keep him in the chair you see in the picture above. It's much easier to clean up than changing his crib sheets constantly.

With this new chemotherapy, you have to change his diapers with gloves, so that makes things a bit more interesting. It's amazing what you have to remember to do when you're in a room in this unit. We wash our hands constantly (my skin is paying for it). And, anything that drops on the floor is put in a "dirty" bin and washed before Frankie can touch it again. And, try to count how many times you rub your eyes or scratch your nose... each time we do that, we have to wash our hands before we touch Frankie or anything we give to him.

And, I can start to see him becoming more lethargic. We only did one set of laps (five) in the hall today. Normally, we do three sets. And, since it wasn't until later in the evening, I think he was really ready for it. He doesn't mind wearing his mask anymore (a requirement when leaving the confines of his room). We decided to start early on setting this rule for when it's absolutely necessary for him to wear it (when his white blood cell count goes below a certain amount). And, I believe we'll be much happier for it.

It's my night to stay with Frankie. And, since he's pretty much given up taking naps during the day (plus the chemo making him tired), he goes to sleep pretty easy. And, unlike most Sanfilippo children, he does not wake during the night, just yet, that is. So, it makes for a peaceful night. And, even with the nurses coming in and out, he only stirs.

Special Notes to Family and Friends Back Home

First, I have to say "THANK YOU" to everyone back home who is helping my whole family fund raise for Frankie. I received a phone call from Marie the other day that really made me feel incredible. The first thing out of her mouth was "Can I just tell you what great friends and coworkers you and Frank have???" And, then the stories came about how everything was going and how wonderful it was to have such incredible support. I cannot express to you enough how lucky Frank and I feel, how filled with love we are whenever we hear a new story about more things that are being done to help our son. It takes my breath away.

Second, while it really does suck to be here (no offense, Duke) and we'd much rather be home, the people here are terrific! We have met such wonderfully, strong, brave individuals and their parents, in just a matter of days. And, without a doubt, there's no where else we'd rather be, helping Frankie. The nurses on this floor always have smiles on their faces and are there to help at a press of a button.

Finally, there's a restaurant, close to our home, that has become one of our favorite places to take Frankie. He absolutely loves it there, and by the sounds of it, the staff love to see him there. The restaurant is the IXTAPA GRILL. If you click on their name, you'll get their address and phone number. It's a great place for Mexican style food and the service is great! Please click here for a picture of Frank (Daddy) on his birthday. Please excuse the quality of the picture as it was taken with a camera phone. I thought I had a picture of Frankie with the sombrero, but I must have lost it. Sorry! Please, if you're in the area and you like Mexican food, it's a nice place for a meal.

There are sixteen (16) beds in this unit, each one of them filled... and a line of children waiting to come in. Please think of them today and say a little prayer for all of the sick and needy children. They give so much of themselves without even thinking about it. They give hugs and kisses when they want, they laugh when they find things funny, and they cry when they are in pain. It's too bad most of us adults don't have the courage to live life like that.

Monday, June 27, Day -3

There's so much to remember. On Sunday morning, I woke up before Frankie, which is very unusual. But, I guess with the drugs coursing through his veins, his little body is just wiped out. When he finally did stir, I had to change his diaper. And, maybe I was still out of it, but when I saw blood in one of his lines, I freaked. But, then I saw the bag, and realized it wasn't his blood, he was getting a transfusion. Apparently, his hemoglobin was low, and when that happens, he gets transfusions. The doctors probably told me this, but I didn't remember at that moment in time. So, my heart stopped for a few seconds.

Frankie pretty much slept all day on Sunday. When they give the transfusion, they also give a dose of Benadryl, so that wiped him out for most of the morning. He was up a little in the afternoon, but fell back asleep for probably three hours. We were worried that he wouldn't sleep at night, but we'd handle that if that happened.

So, Sunday was pretty much uneventful until we tried to get him out for a walk in the hallway. It's a big production because we have to wipe down the car we push him in, unplug his IV pole from the wall, get his shoes on, secure him to his IV pole... While securing him to the pole, one of his lines got caught across his neck and choked him so much so that he vomited all over the floor and me. So, that pretty much put an end to the idea of him walking yesterday.

Once we changed his dressing (where the lines come out of his chest), he was back in his crib just hanging out watching Elmo. I decided I'd stay with him since we weren't sure if he'd sleep at all. I was prepared to be up all night with him, but he fell asleep shortly after 10 pm.

This morning, as I was rising out of my own dreamy sleep, I almost forgot where I was. So much so, that when I did remember, I got so angry. I was angry at the situation. I'm so upset that my son has been diagnosed with this disorder. I get angry because he is the sweetest, most amazing child, and you wonder how does God allow this to happen?

While most parents are worried about their children growing up right, I worry about my child even getting that chance. I worry about the odds for his survival. My heart breaks because I'll probably never hear the words "I love you, Mommy" come out of his mouth. And, even as I write this, and the tears pour down my face, I realize how selfish that is. I know he loves us by his actions, his smile, his laughter.

I get upset because I have all of the hope in the world for Frankie, but then the reality of this disorder slaps me in the face. I get depressed, but today is the first day I've cried about it in a long time. I had come to grips with it, but today... Today, it's almost 9 am and he's still not awake. This isn't my son. He's normally up at 5:30 am chatting away in his own language, waiting for his sleepy parents to get out of bed and start the day.

I just want to hold him forever and I wish every single moment that a miracle will come our way.

Well, enough of my bantering. I have to get Frankie up so they can do an EKG on him before they give him another dose of Cytoxan. They do this because this drug could cause him to have heart failure and die if not properly monitored. Reality check... welcome to our daily world.

Wednesday, June 29 , Day -1

Good morning, all. Tomorrow is TRANSPLANT DAY! And, it feels like we've been on the unit for a month already... and it's only been ten (10) days.

It was my night last night with Frankie. And, for whatever reason, I just couldn't sleep. I can normally sleep anywhere at any time, so this was different for me. I think I fell asleep around 2 am. Unfortunately, there were a couple of times during the night last night when I had to get up to change Frankie and his sheets. With the amount of fluids being pumped in his system and the body's normal reaction to chemotherapy, it has become a necessity to do throughout the night.

It's now about 7 am and I've been up for an hour, at least. So, I'm working on roughly 4 hours of sleep. And, surprisingly, I'm not all that tired. Frankie, on the other hand, looks entirely wiped out. I'd take a picture of him today and post it, but, he really doesn't look like himself. He's bloated from the chemo, red and blotchy, and not at all smiling. He's not crying either, he's just very "blah".

We are on our third and last day for the drug, ATG. And, you can really tell this procedure has taken a toll on his little body. There is no chemotherapy today. Yesterday was the last day for that. So, even though he gets other drugs today, this is considered his "Day of Rest". And, from what I'm told, he's supposed to perk back up to his normal self after the transplant.

I think it's going to be a quiet day for us in 5205. As always, thank you for reading and wanting to keep up to date with Frankie's progress. I have received emails from so many people, old friends, new friends, and people I don't even know! It's amazing how people pull together. For those of you, I cannot thank you enough for all of your lovely emails, thoughts and prayers. Forgive me for not responding right away, as I just want to get through Transplant Day.

Thursday, June 30: TRANSPLANT DAY! DAY 0

Wow! It's already 2pm and the transplant has been complete for almost 2 hours! The transfusion started around 11:40am and finished at 12:23pm... thanks, Dad for timing it!

For those of you who aren't quite sure of what happens, it's not a surgery... it's an IV bag filled with the umbillical cord blood that drips through his chest catheter into his heart. Once complete, the cells begin their work, move to the bone marrow and begin to build new bone marrow, hence the motto on the unit "Grow Cells Grow." Until his transplanted stem cells have a chance to recover and begin making blood cells in his body, Frankie will require red blood cell and platelet transfusions to replace the cells that are not being made.

Unfortunately, it's not always possible to give white blood cell transfusions in this manner, so he'll be receiving antibiotics to help fight infections. And, it's possible that he will be given another medication to help his white blood cells to grow more rapidly.

So, for the actual transplant, everything went well. He slept through most of it. We picked out a certificate for Frankie (one of the "achievements awards") he gets while on the unit. It's only fitting that it has Elmo on it.

Just a little tid bit of info... His blood, as well as his donor's blood is B Positive! Hmmm...

Another little bit of info... Frankie will always have two types of DNA. He will have skin DNA that will always show that he's a male with MPS III Type A. His blood will show that he's a female without MPS III Type A. And, rest assured, he won't inherit any female traits... Frank has already asked the doctors about that!

During the next couple of weeks, Frankie will still be feeling pretty crappy (vomiting, hives, hair loss, etc...) from the chemotherapy and transplant. We have been told he will feel and look his absolute worst around Day +12 (around July 12th, convenient math, huh???)