Monday, February 6, 2006 - Day + 221
Monday morning, and still my little one is testing me with his sleep patterns. Part of me thinks it's because he only wakes up at 3:00am when he doesn't take an afternoon nap the day before, and the other part worries that it's a symptom of Sanfilippo that hasn't been affected by the transplant. If it's the latter, of course, I start to worry about other symptoms that the transplant cannot or has not "corrected."

So, I'm exhausted, and should be napping. However, I felt like I needed to write an update, so here it goes...

I've added another 5200 child, Cooper. He is a beautiful 2 1/2 year old that is also afflicted with Sanfilippo. He is scheduled to have the central lines placed tomorrow with immediate entrance to 5200 thereafter. Cooper has two older brothers (neither of which has Sanfilippo) and the whole family has relocated to Durham (from Connecticut) while Cooper undergoes his treatment. Please say prayers for Cooper and his family and visit his site. They need all of the prayers and support they can get, as we all have and still do.

I wrote a note to a former 5200 mom today about having good days and bad days. Lately, it seems like my bad days come on quicker and more frequent than they did in the past. I get angry and frustrated at the situation, the disorder/disease, but there's really no one to lash out to. Then I'm brought back to the fact that I am blessed to wake up every morning to see and hear my son, to hold him and kiss him and to feel his spontaneous hugs. Would I change anything (within my control) in the past year? Absolutely not.

But understand that back here in Connecticut, it's hard for us to relate to the normal kid trauma that most parents face. I find myself yearning to talk with the 5200 families, so I send emails and also call when I can. The strange phenomena that we are faced with is the guilt of our children doing well. We count ourselves so incredibly, profoundly lucky, and yet I find myself scared to admit that my son, despite the lack of eating, teeth issue, and developmental delays, is doing well.

Every time I think of Frannie, Jana, Emma, Asher, Queen Maddy, Maddy Trimper, Lane, Keturah, AJ, Joseph, and all of the other children that have earned their wings, I am brought to tears. For anyone who knows me, and knows me well, you will know that I'm not much of a crier. I have cried more in the past 12 months, than I have in the past 34 years. I tend to crack jokes, throw sarcasm around, and basically stay in an upbeat mood despite everything that goes on around me. But, there are those faces and names of the children and their families that I cannot and will not forget for my entire life. A piece of my heart disappears with every child that leaves this earth. The hole in my heart is so small, I know, in comparison to the loss their families endure.

I didn't mean for this update to be so down. I guess I'm just so tired and I visited a couple of the sites today that put me on an emotional roller coaster, so this is the result.

However, when I do think of the losses, I have to turn immediately to the success stories and to the people I know will cheer me up. I did that today, and it helped tremendously. We need to laugh. I need to laugh. I need to forget sometimes that my son may not develop beyond a 3 year old mentality, and just live in the moment, because there are some of us out there that know just exactly how quickly that moment passes. Thanks, Jean, for cheering me up this morning... you have no idea how much that helped me!

This week we have clinic which should be roughly a five hour day, then on Thursday we have three appointments - Dentist, Teacher, and Frankie's first Speech Therapy session since we left Duke late November.

On Friday evening, February 10th, there's a fundraising meeting at the Hamden Senior Center (7:00pm). Even though we are home, neither Frank nor myself are planning to attend.

And, as I'm writing this, Frankie is WIDE AWAKE in his crib, NOT taking his afternoon nap. So, I guess I'll be up early tomorrow morning, too. Thank goodness for coffee!! I just ordered a new coffee maker, as I have completely burned out Frank's Mr. Coffee. Unfortunately, I don't think it will arrive before 3:00am tomorrow morning.

Thursday, February 9, 2006 - Day + 224
I guess I'll start with the basic good news... Clinic visit yesterday was uneventful. What I mean by this is that things went very well. Frankie's numbers (counts and such) are all within the normal range. His weight, which fluctuates, is still considered to be "holding" at 16.1kg. And, with the gradual decrease in his blood pressure patch, his BP has been at an acceptable 109/77, or thereabouts.

Per the discussion I had with Frankie's doctor and nurse practitioner, we believe he just may be done with naps all together. This is disappointing for me, as I try to do all of the housework while he naps. It's also the time when I can take a nap, when he wakes up so early in the mornings! Fortunately, he slept in a bit more for me yesterday morning (6:30am). But, just when we think we have him pegged... as we were leaving the clinic, he was sound asleep in his stroller before I even got outside! I let him sleep for just an hour. I knew I was testing my luck, but I wanted to see what would happen.

This morning, we had to get up early anyway, for an 8:45am appointment at the dentist. He started to stir around 7:00am!! I couldn't believe my luck! After a week of 2:00am-4:00am wakeup calls, he appeared to be back on schedule.

In order to see the dentist, Frankie has to premedicate with Amoxicillin (10ml) one hour prior to the visit. So, I was a little rushed this morning trying to get him dressed, me dressed, him fed and all of the medications down. We were out the door just after 8:00am. The dentist's office is right across the street from where we go to clinic. But, we have to park in the lot a block away. And, since we are usually trying to find a parking spot during the "rush hour," we end up parking on the 10th level.

Today, as we were driving, with only one more exit to go on the highway... it happened. Frankie vomitted EVERYTHING! It just kept coming out of his little mouth, all over the back of my car, my new car! Actually, I didn't really mind about the car, I was just so freaked out that he would vomit. It's so unlike him, so I instantly got frazzled. I couldn't pull over, so I just kept driving to the parking garage, 10th level. I called the clinic and was able to speak to Frankie's NP, who reassured me that he was probably just car sick. Then, I called the dentist and rescheduled the appointment for tomorrow.

With my hands-free on (CT law, you know), I called my mother-in-law, who was conveniently already at my house. She helped me get Frankie out of the car, the carseat out of the car, and clean up everything and everyone! I was afraid to take the carseat padding off because I wasn't sure if I'd be able to put it back together, but it really needed to be washed. I took the risk. I was able to clean the ENTIRE back seat of my car with upholstery cleaner, and that seemed to work.

Now, the test among all tests... putting the carseat back together! It took me about an hour, but I was able to do it, without the manual, too! And, now it looks brand new. I'm waiting for Frank to come home to put it back into the car. I'd do it, but a) it's cold; b) someone has to watch Frankie and c) the upholstery in the back seat still needs to dry from the cleaner.

So, now I really do need a nap. It's 1:44pm, and my expulsive little boy shows no signs of winding down. Plus, we have Speech Therapy coming at 3:00pm, so napping does not seem likely. And, my coffee maker isn't here yet.

Saturday, February 18, 2006 - Day + 233
Well, as I'm sure some of you may have been following my son's story, you may be wondering if I finally got the coffee maker I've been dreaming about! Yes, I did. It came right before the snowfall we received last week. In our neighborhood, we got almost 2 feet of snow. I've added some pictures to this web site (Pictures), including a picture of the coffee maker!

In the past week, Frankie has vomited a total of three times... once in my car, once in the kitchen, and once on his little armchair. The doctors aren't overly concerned with these episodes, because they haven't been consistent. And, it's been five days since the last time. In addition, he has some bumps that have appeared on his body. It's possible that they are hives, based on their location, color and size. I'm hoping that's all it is, and not the beginnings of GVH. I have calls and emails into the doctors, and will be bringing Frankie in this week so that someone can look at them and reassure me.

Another concern Frank andI have is that he's not really eating all that well. We try everything at every meal, just to get something in him other than Pediasure. We're really not even concerned about it being healthy, but rather calories. We were pretty ecstatic when he had one yogurt and 1/2 jar of stage 2 peas for lunch today. His weight hovers at 16.0kg. He went into the transplant at 15.8kg, so he's still okay, by the doctors' standards.

His activity level is almost always at "Full Speed." And, when he becomes snuggly with either Frank or myself, I know he's not feeling well. To try to keep him in one spot is virtually impossible.

Recently, we've enlisted the help of the local "Birth-to-Three" program. He gets a teacher once a week, speech therapy three times a month and physical therapy twice a month. All three women work together to reinforce each aspect of his developmental needs. I wish they could come more often, but it's a state run program with limited resources. We are looking into supplementing with private services.

PLEASE NOTE:
Separate from the fund raising activities that are held for Frankie, I'm trying to arrange for me to walk in the 6th ANNUAL RAINBOW OF HEROES WALK, at Duke on Saturday, May 6. I would like to raise $1000.00 by April 30th.

What is Rainbow of Heroes?
It is a walk commemorating and celebrating all Duke PBMT patients and their families. Walk participants walk 5200 yards in honor of the inpatient unit (5200). For more information about Rainbow of Heroes, please check out their website: Rainbow of Heroes

Where does the money raised go?
All monies raised will go to support PBMT families through the Family Support Program (volunteer-based). So many resources are needed to make the whole transplant process for families "easier." Every contribution, whatever amount, makes a difference.

To give you an idea... when Frank & I were on the unit with Frankie, until he was there 30 consecutive days, we had to pay for parking EVERY day. Parking is $1.00 an hour, at a maximum of $6.00 per day. A donation of $250.00 could purchase 10 books of parking passes for PBMT families.

Every day, during the work week, Family Support provides various activities for the transplant children, their siblings and parents. A gift of $750.00 would provide art supplies for group activities for a whole year! It is unbelievably important for these activities to continue. They help create a sense of "normalcy" for the families on the unit. Plus, it brings the children out of their rooms and encourages social interaction.

These are just a couple of examples of what donations could do to help families who have to go through this unfortunate situation. For me, anything that could put a smile on a child's face, or take away just one little worry a parent has, is immesurable.

How can you help?
Anyone who would like to sponsor me in the walk (donate to the cause), can send me a check written out to "Duke University" with 06RH in the Memo space. All donations are tax deductible, so if you'd like a tax receipt, please let me know that as I can get those when I register for the walk on May 6th.

If you would like to sponsor me, please send me an email at cynthia@frankiepants.com. Again, I'd like to raise $1000.00 by April 30th. Every donation counts, no matter how big, no matter how small.

Please help support a wonderful program, that helped Frankie, Frank & I get through the 55 days we were on the unit and the three months we were at Duke's clinic, before coming home.

Monday, February 20, 2006 - Day + 235
Frankie's skin and appetite continued to get worse this past week. On Sunday night, I wrote an email to Frankie's local doctor and nurse practitioner asking if I should bring him in for them to examine him. I had taken pictures of his skin, concerned it may be GVH or even hives, though I'm not sure why he would have gotten hives.

For those who know what these numbers mean, his FK level has consistently been at 7.0 for the past few weeks. This number is low by Duke's and Yale's standards, coupled with the skin issues, Frank & I were a bit concerned. Thankfully, Carolyn (NP) called this morning asking me to bring him in for her to see and get a current weight on him. (As an update, we're increasing his FK dose starting tonight with a blood draw on Wednesday to check the level for any change).

His weight dropped again, down to 15.5kg. This is the lowest it's been, even pre-transplant he was 15.8kg.

When in the exam room, I showed Carolyn the spots on his arms and chest. As she was puzzled by the increase since the last time she saw him, she called Dermatology immediately, and one of the Derm doctors came to the room within minutes. When she arrived, Frankie got the full exam with me showing the doctors where all of the bumps/spots were. The general concensus is that he may be allergic to the "new" dressing we're using.

As Duke Infusion doesn't ship above Virginia, we had to find a new medical supply house (Yale uses Collins). I told them what we use for his dressing, IV 3000, and even when they showed up looking a bit different, I didn't think anything of it. I just figured it was an updated version of what we were getting in Durham. The main difference was an orange strip of adhesive on either side of the dressing. We've been using this dressing for over a month (changed every Saturday). And, the bumps on his skin have been appearing gradually. This past week was the worst we've ever seen it.

After today's visit, I went online when I got home. Apparently, the version Collins was sending us, those orange strips, contain Latex. It's entirely possible that my son has a sensitivity to latex, especially since we did have problems finding the right dressing for him while we were still on the 5200 unit. One dressing we used made him go absolutely nuts! He was so incredibly hyperactive, it scared me. So, the reaction to this new dressing didn't surprise me today. I guess you can have random spots/bumps appear all over your skin as a reaction to something placed elsewhere on your skin. The dermatologist called it an ID reaction... or something like that.

I informed my contact at Collins about our situation, so we're both working on getting the right dressing for Frankie from now on. Unfortunately, you guessed it, the right dressing for Frankie costs over twice as much as the IV 3000 version they were sending us. I won't be surprised if I'm purchasing these for Frankie online instead... I've been doing that for his green masks and the earloop masks, as Collins doesn't supply those items. If anyone runs into that problem when they're back "home home", I can send you the links I have for the suppliers I use... they're quick and inexpensive.

As for the appetite... Frankie has been eating less and less as the days go by (thus the weight loss). There is a concern, although not immediate, with having to get an NG tube for him. We don't want to go down that road, obviously. So, we're hoping that Endocrinology and Frankie's doctors can figure out a better solution.

Friday, February 24, 2006 - Day + 239
As Frankie's doctor was not at the hospital on Monday, we made another visit to see her on Wednesday. She wanted to see his skin for herself. And, I wanted to discuss some more issues with her and Carolyn (NP). From Monday to Wednesday, the rash had been getting steadily worse. We were hopeful that the new dressing would correct the problem.

Update on Frankie's skin: the orange strips on the new dressing did not have latex, but there is a stronger adhesive that he was reacting to. We received the old type of dressing from our local supplier on Wednesday morning and were able to give him a thorough bath before changing the dressing that night. Since then, the rash has lessened on most body parts, but still his hands, feet and the area under the dressing have nasty bumps, spots and cracked skin.

We've been applying Bacitracin Zinc Ointment on the broken skin, Triamcinolone cream on his body and 2.5% hydrocortisone cream on his face twice a day to control the rash. Thankfully, everybody agrees that it is not GVH.

Dr. Sleight also gave me some high calorie supplements to give to Frankie if he continues to be fussy with his eating. But, I did find out that for his age, he should be taking in anywhere between 850 and 1300 calories. So, based on what he is eating daily, I'm not as concerned anymore about how much he eats. Our main concern is that he's getting all of the nutrition he requires.

We also tested his FK level after increasing his dose. Somehow that level came back as 17! For those who don't know about FK levels, that's quite a jump from 7 when only increasing the dose 10%. Thankfully, it's not a toxic level. Carolyn was concerned about it, in any event, so I redrew his blood on Thursday. The level came back at 10, right where it needs to be.

I just wanted to thank everyone who has been emailing me, concerned about Frankie. We're doing much better after our Monday & Wednesday visits to Yale. He's such a little trooper. And for those moms who were with me on the unit... those darn papercuts! :)