Thursday, December 1, 2005 - Day + 154
Apparently, it's going to take some used to... being home. Frank's been oversleeping for work twice this week, I forgot today was garbage day, and I'm sure there are other things that have slipped my brain. But, on the flip side, I've been giving myself a project to complete every day. It doesn't mean that it's a big project, but just something to accomplish each day so the house isn't completely a wreck. There's still so much we have to do to make it livable again!

Please say a prayer or just send good thoughts toward another special family in Connecticut. For privacy sake, I won't mention their name or what town they live in, but they just discovered that their youngest child has MPS III Type A. I spent some time on the phone with them last night, and I can only hope I was a little help to them. They are going to need a lot of love and support to get through this, what ever they decide to do.

Yesterday, Frankie & I went to clinic at 8:30am. This was our first Yale clinic visit where Frankie had to get his Cytogam and Diclusimab. And, despite the wait for the drugs, everything went smoothly! It was quite the relief. The pharmacy was short-handed, so the wait was justifiable. Our nurse, Denise, is so great! I guess the benefit of a smaller clinic, is that when the pumps beep, the nurses hear it a lot easier. I don't think the pumps beeped longer than 30 seconds before Denise came walking through the door. At 1:30pm we were back in the car, headed home after a long day. But, as is always the case, my little one was quite the trooper. It's tough for him to nap at clinic, so he was starting to get fidgety by the end. So, when we arrived home, he went straight for a well needed nap.

Frankie's bloodwork came back great and within norms. His CMV level (as measured here) showed only one out of 200 cells, compared to last week's 25 out of 200. So, this is great news. If it hadn't improved, we'd be back to administering Gancyclovir (IV) at home. Yuck! It's not bad, a one hour drip, but it's tedious to chase a 2 1/2 year old around while the infusion is taking place.

I hope this update finds everyone well. Our thoughts and prayers continue to go out to all of the families we have met along the way. We miss you!

Sunday, December 11, 2005 - Day + 164
Oops! Okay, so it's been ten (10) days since my last update. We've been busy at the house, getting it ready for the Christmas holiday and getting ourselves ready to head back down to Duke for Frankie's 6-month studies.

And, in case you were wondering... yeah, I'm typing this update as I sit in the exact same apartment in Durham that we just left not three weeks ago! It's beautiful weather today, sunny and not too cold. We left Connecticut after a snowfall of roughly 8 inches! I think I've got some photos of the little one playing in the snow from Friday... in case any of you miss the stuff!

We arrived here roughly 11:00am this morning, did some grocery shopping and now are just relaxing before a jam-packed week of exams, tests, etc... But, this time around, we're veterans, so it will be a bit more routine. However, I'm thinking tomorrow may test me... three appointments on the second floor of the CHC... anyone remember our experiences there?!? ha ha ha... we'll see how tomorrow goes. Hopefully, there won't be any altercations.

I need to extend a "Thank You" to a group of people from our hometown. Employees from the Assessor's Office of Hamden contributed nearly $800.00 toward Frankie's COTA fund during a "Jeans for Frankie" fundraiser back in October. My sister just sent me the email, so please forgive the late mention. Thanks so much!!!

Also, another fundraiser that was held during the month of September at the Typhoon Restaurant in Middletown raised over $1100.00! This was a "double" fundraising event consisting of canisters and a percent of all sales going toward Frankie's fund.

These, as well as all of the other fundraising events, are so very much appreciated. Frankie's care will be ongoing as he recovers from his transplant. We are scheduled to return to Duke every three months post-transplant for the first year, every six months until year two post-transplant, and then annually after that. In addition to these trips, is the care for him at home (dressing and cap changes while he has his chest lumen); physical, occupational and speech therapy; weekly clinic visits to Yale; and any other special care that will arise that we can't plan for.

And, for those of you who log on to see how the little one is doing, he's really doing quite well. Our only issue of late is that his FK level dropped considerably since last week. FK levels show how much the drug is absorbing into his system. He needs to take this drug to suppress his immune system while the donor cells grow in his body. The FK drug helps combat Graft vs. Host during this tenuous time. The doctors like to see this level between 10 and 15. Last week (November 30) it was 13. This past Wednesday (December 7) it dropped to 7. The doctors aren't sure what could cause this drop in the FK level, but boosted the dosage up 1 ml to adjust. Tomorrow, we'll drop off a blood draw to check since we've been in transit since the dosage was increased. An FK level that is too high could result in seizures, among other bad things. Thankfully, it doesn't appear that he's become "toxic" over the past few days. So, I think we're okay.

Thursday, December 22, 2005 - Day + 175
Good evening, all! It's about 9pm as I'm starting to write this, and I know this has been a long time coming. I've received emails and phone calls from a lot of people wondering how Frankie's 6 month studies went. So, I'm going to summarize below. These test results quoted below are from our preliminary report from Frankie's doctor before we left Duke. We are still waiting on a couple of tests to come back (Enzyme and Gags).

MRI - No evidence of the blood on the brain from the 100 day studies. Apparently, the blood can and did resolve itself. Also, there is no new atrophy of the brain from the 100 day studies, which is very good. In reviewing some of his blood counts, the doctors noted that from his 100 day studies, Frankie's cortizol level (self generating steroid that makes everyone hungry and therefore prompts us to eat) was abnormally low. The endocrinologist that examined him this time around added a pituitary scan to the standard MRI, which showed no abnormalities (another good sign).

Pulmonary Function Test - normal range. This is basically how well his lungs are working, and everything is fine in that department.
Echocardiogram - perfectly fine
Hearing tests - absolutely normal

EEG - In prior EEG tests (pre-transplant and 100 day studies), Frankie's EEG results showed some slowing of the brain. At his 6 month EEG, there was no slowing and no seizure activity. While this is very encouraging, I'm not getting my hopes up just yet. I'd like to see another EEG (9 month studies) showing the same results before I get excited... that's just the rational side of me.

Chest X-Ray - basically normal. There was some evidence of mild atelectasis (the collapse of part or all of a lung by blockage of the air passages). From our meeting with Dr. Szabolcs and Jerelyn (Frankie's NP), they were not concerned with this finding.
BAER Test - This test is a brainstem auditory evoked potential study to evaluate any brainstem dysfunction. It is done under anesthesia/sedation. All results came back normal.
Eye Exam at Dr. Greene's office - Normal pupils, lids, cornea.

Labs
WBC - 4.5
RBC - 3.55
Hemoglobin - 11.1
Platelet Count - 208
CMV - not detected (woo hoo)
CD4+ T-cell count - 183 representing his immune system. A count below 200 indicates that Frankie is still at risk for complications from infections. He continues to wear his mask outside of the home and will do so for probably the next 6-7 months.
"naive" cells - 33.3% of Frankie's T-cells examined showed to be naive, meaning that his body is producing new cells/lymphocyte production from the donor cells that will learn how to fight off new infections. CD4 T-cells are an important factor when determining one's immune system.

Children who go to daycare or are around other children and the parents of those children are still Frankie's biggest risk.

Now, for the non-clinical update... Frankie did great the entire trip back to Duke. His tolerance level continues to amaze me. We were able to make the trip down in two days, which was really nice. And, we actually got the same exact apartment for the week we were back.

Day one (Monday, December 12) was probably our busiest day. And, we had to be on the second floor for three of our four appointments, so I was curious how that was going to go... for those of you who remember any of my "altercations" on the second floor, things went incredibly smoother this time around. Frank's sure that my name is flagged somehow in their system, because we were in-and-out of our appointments without too much fuss.

Day two, Tuesday, was the "all day" developmental analysis of Frankie at the Center for Developmental Learning (UNC-Chapel Hill). The intense study of Frankie and his abilities lasted a straight 3 hours. And, I have to commend my son for not freaking out at all the entire time. He had at least four people having him point, talk, and walk from the moment we stepped into the center until the moment we left. Probably the funniest part of the day was being in this one room that had a big two-way mirror (for outsiders to observe). Of course, my son is pretty much in love with himself, so most of the time he was staring at himself in the mirror and chatting to his reflection. It was quite endearing, if I may say. He also fell in love with one of the "testers" who I believe was a student that was helping. She was young and attractive, and when she got up to leave, he ran over to her and gave her a hug... yeah, my son the flirt...

Day three, Wednesday, Frankie had his EEG and BAER test done at the same time... quite convenient. He was supposed to be sedated, but that didn't really take, so he just watched Barney and Blue's Clues while they put the electrodes on his head (I have pictures I'll post another time) and ran the test - total time was probably around 2 hours. Again, my son amazes me. And, after that, we went to clinic for our normal Wednesday infusions of Cytogam and Diclusimab and the monthly Pentamadine (sp?). All went well until the Pentamadine was almost finished... Frankie decided he didn't like what was hanging out in his belly, and vomited all over the bed. He had never vomited before (from the Pentamadine), so we're hoping it was just a "normal" reaction from the Pentamadine/Benadryl combo and the earlier sedative (EEG/BAER test).

Day four, Thursday, consisted of another visit to clinic for the remaining blood draws; Frankie's Echocardiogram and the Pulmonary Function Test... with a chest X-ray squeezed in there somewhere. And, as I can't remember much from that day, I can only imagine that it was uneventful.

Day five, Friday, Frankie had his MRI in the morning and an ultrasound of his abdomen in the afternoon. He did great through the MRI. He usually wakes up during the test a couple of times, but I guess they hit him hard with the anesthesia, because he was OUT the rest of the day. After all was said and done, for the tests, we met with Dr. Szabolcs and Jerelyn to review all of the results, which I've written above.

And, after that meeting, we were back off to the apartment to pack up and head out. We wanted to make it to Richmond that night, which we did. We left Durham around 6:30pm and got to Richmond around 9:00pm. Without too much trouble or traffic, we were back home on Saturday around 3:00pm.

So, in the midst of all of this, Frank & I have been trying to get ready for the Christmas season. I've finally completed all of my shopping, and now am left to the task of wrapping! But, before I type anything more, I have to extend a HUGE THANK YOU to the wonderfully generous people at Anthem who came together and got Frankie a bunch of presents. Thank you, thank you, thank you! He's going to be thrilled with his new toys.

I also have to thank Mike and Diane. It's so belated, and I hope they forgive us, but thank you for your generous gift and the bed. As MY luck would have it, I wrestled for a good 30 minutes with the bed early one morning, eventually getting all of the pieces to fit together the right way. It took everything Frank had not to laugh when he opened up our mail later that same day, only to find the directions!

I know I'm forgetting something and/or someone, but I really wanted to get Frankie's 6 month studies posted so there was no longer any concern with that. And, as it's getting closer to 10pm, my mind has a tendency to shut down in 5, 4, 3, 2,

Tuesday, December 27, 2005 - Day + 180
To all... Happy Holidays! Frankie had a great Christmas this year, as did Frank & I. I'll write more another time, but I wanted to make sure I wrote the following entrry...

A couple of days before Christmas, we received a package from a local school - Montowese Elementary. Inside the package were beautiful Christmas cards from the third grade. The Christmas cards were spurred from one of the students in the class, as she told Frankie's story to them one day. That little girl's name, Miranda. Although I have never met her, and she has never met Frankie, she has touched our lives with just a simple gesture. Miranda, we hope you had a great holiday. Frankie sends you hugs and smoochies! Thank you so much for sharing Frankie's story.