Sunday, October 2, 2005 - Day + 94
Emma Grace Brashear earned her angel wings shortly before 9:00am this morning. While on the 5200 unit, Emma was Frankie's next door neighbor. We only saw her a couple of times, in her mother's arms, walking the hallways. Frank & I would talk with her grandfather, David, and you could tell that her illness was pulling at the entire family's hearts. She turned one year old in September. I remember going to the discharge class with Ross and Kim, Emma's parents. My heart sinks thinking of that moment. They are a strong, faithful family, stronger than I would be, that's for sure.

In addition, I also found out about a Sanfilippo girl, Jillian, who was transplanted in 2002, that also earned her angel wings this past week. She was the fifth Sanfilippo child to be transplanted at Duke. Tommy (link to the right) was the sixth. They were on the unit together, and as is the norm, the two families became one family, going through the ups and downs together. Tommy's brother and sister are also afflicted with Sanfilippo, but were too far advanced to receive the transplant.

AJ is still in the PICU and needs many prayers to help him fight back to the 5200 unit.

Please pray for these little ones and for all of the children out there that need a miracle.

Frankie is doing very well today. He woke up ready to go this morning and was laughing and dancing all morning long. Frank made us both a nice breakfast and we spent the morning watching Barney and Blues Clues (a new favorite).

I will write more as the week goes as we have a lot going on this week, and I'd like to keep family and friends up to date. But, in the meantime, hug your children, tell them you love them, and enjoy every minute you get to spend with them.

Emma and Jillian, peace to you little ones...

Tuesday, October 4, 2005 - Day + 96
Yesterday, Frank, Frankie and I packed up for a 10:00am appointment at UNC (Chapel Hill) for a "short" developmental review. Unfortunately, when we arrived at the doctor's office, they had no idea that the appointment was scheduled by Duke. However, since this doctor is actually married to Frankie's doctor at Duke, she moved her schedule around to keep our 10:00am appointment... very nice of her.

Frankie, on the other hand, was woken up mid-nap, so he only participated well during for the first 45 minutes, or so. After that, he wouldn't even stand on his own two feet and started crying. So, the doctor gave me a couple of pamphlets to fill out to indicate his level of mental development. This took another twenty minutes or so, and then we were back on our way back to Durham for our daily visit to the grocery store.

Today, Frankie was supposed to have a Physical Therapy session, but he fell asleep for his nap about an hour before she was scheduled to arrive. So, I called and left a message to see if we could push it back. Fortunately, she wasn't feeling well (sounded very congested), and it was best, for obvious reasons, that we cancel the session.

The rest of the day was very blah. In the afternoon, I was getting a bit cabin-feverish, so I took Frankie out for a walk around the apartment complex. He was getting too fussy in the apartment and wouldn't settle down for an afternoon nap. My only hope was to tire him out by walking around outside... and it worked. When we got back to the apartment, he fell asleep on my lap on the couch.

Tomorrow, Wednesday, Frankie has an MRI scheduled at 11:00 of his brain. He has to be sedated (duh) so he can't eat past 5:00am and he can't have anything to drink past 9:00am. Then after the MRI, we have to head over to clinic for Doctor Day and his Speech Therapy session. So, tomorrow we probably won't get back to the apartment until 4:00pm.

FYI, for those of you who have emailed and/or called me regarding last week's problems... EVERYTHING IS FINE NOW. I made a TON of calls on Friday to clear up the insurance issues we are having. Thanks to everyone who expressed concern and offered help.

PS... I also forgot to mention that for my birthday last week, my mom came to visit. It was great to have her here. She was only able to stay for a couple of days, but it was nice to have that new presence in the apartment. And, I think she was relieved to see Frankie doing so well. She hasn't seen him since May, and isn't one to use a computer to read the web site, so I think it was good for her to visit with him.

Wednesday, October 5, 2005 - Day + 97
AJ has taken a turn for the worse in the PICU. Please pray for his body to heal. His tummy is swollen and is pressing against his lungs, making it extremely difficult for him to breathe on his own. He is an amazing child that deserves to grow into the fabulous adult I know he will be. Please, please say your prayers for him today.

Frankie is doing fine. He had an MRI of his brain done today, and is still sleeping off the sedation from 11:00am this morning. It's now 3:15pm and we're still in clinic waiting for his IV medication to finish. This is probably going to throw off our schedule (sleeping/napping) today, but that's okay.

His MRI shows some atrophy from the MRI done prior to transplant. However, this is expected due to the stress that it put on his little body throughout the entire transplant procedure. All of Frankie's counts look good, but his hemoglobin is trending downward. Again, this could be from the muliple blood draws he had last week for his 100-day studies, but we'll see on Friday if he needs another transfusion. He hasn't needed hemoglobin since August 29th.

PS... I posted four new pictures yesterday.

CONGRATULATIONS to Cari, Matt and Jessie!! Cari gave birth to Dylan yesterday (October 4)!!!

Wednesday, October 5, 2005 - Day + 97 EVENING UPDATE
To say that I have a heavy heart today would be an understatement. We have lost another sweet child and friend. AJ Haynes (link to right) lost his fight this afternoon. His little lungs were just too weak to breathe on his own.

My heart is breaking, but it's nothing compared to his family, I'm sure. And, I am not alone. AJ was quite the personality on 5200. All of the nurses love him and some would even fight over taking care of him. And, even though we only got to see him with a mask on, he has these gorgeous big blue eyes that just, well, sparkle.

AJ was probably the most polite and caring 4 1/2 year old you could ever meet. Every time I would see him in the hallway, he would say hello to me. He even played basketball with Frankie and Kate one day, and was very patient with Frankie, as Frankie was just learning the concept of putting a basketball in a net.

I loved to see AJ in the hallway, buzzing around on the tricycle. I would hide around the corner and jump out shouting "BOO" and he would do the same. He had earned over one hundred feet... this is a program called "Steps to Recovery". For every five laps in the hallway, children would earn these little plastic feet to put on a shoe lace. He was on the record breaking list for the amount of feet he earned while on 5200. He was so proud of those feet... and he earned every single one of them.

Please take a moment to say a prayer for AJ and his family. They are suffering a great loss, as is the rest of this world, because AJ was such a sweet, sweet boy. It's incredibly unfair. And, honestly, of all of the children that were on the floor with Frankie, I never suspected that I'd be writing this about AJ. He was such a fighter and deserved to have his own discharge parade. I hope they had tons of confetti in heaven to welcome him. Big Hugs, AJ... we'll miss you.

Saturday, October 8, 2005 - Day + 100 HUGE MILESTONE DAY FOR FRANKIE
Today marks Frankie's 100th day post-transplant. This is a HUGE milestone in the world of PBMTU. This doesn't mean that he's 100% healed/better, but it does mark a point in the whole process where we can test the effectiveness of the transplant. As such, there are several retests that are done, to compare to the pre-work that was done back in April this year. This protocol is standard for all Duke PBMTU patients.

So, to recap the past couple of week's re-evaluations:
Pulmunary tests - came back great - lungs are operating as expected
Echocardiogram - heart and thyroid are normal
Electroencephalogram - nothing out of the ordinary - no signs of prior seizure or stroke, for example
Developmental "mini" evaluation - needs some help categorizing and matching as well as mimicking behavior & improving attention span.
MRI of Frankie's brain - showed blood on the brain (between the skull and the brain) and atrophy. The blood on the brain has been reviewed by neurosurgeons and will be re-evaluated in two weeks with another MRI or CTScan. The atrophy is expected due to the stresses of the entire transplant process. Frankie's brain was already showing signs of atrophy prior to the transplant, an unfortunate result of the disorder.
Hearing Exam - He can hear us fine... we just now have proof that he's ignoring us!
Eye Exam - Frankie's vision is normal

Re-evaluation of the FISH test (percent of donor cells) came back as 100% !!!!!

We're still waiting on the GAGS test to see if the enzyme is present that Frankie was lacking (heparin sulfate).

Wednesday, October 12, 2005 - Day + 104
Clinic today was uneventful. But, that's not to say we didn't have some excitement, but that's later...

Frankie's progress is still as expected. However, we're still monitoring his potassium levels. We have to do this by monitoring what he eats. And, for those of you who are unaware of what foods contain potassium... EVERYTHING contains potassium!!! It's been a challenge to get Frankie back to eating decent sized meals (for caloric intake), and now we have to drop back because so many foods that he likes to eat, and even some he doesn't, contain too much potassium.

Frankie's been off of his nightly nutrition (TPN) for one week now. And, he's been doing great without it. The doctors put him on two steroids to get him to eat, and they've definitely done the trick. Althought, not without complications, as indicated in the paragraph above.

This weekend, we are going to try taking his Ganciclovir (IV med for antivirus) to the oral equivalent. So, that would mean that he would no longer have any IV treatments done at the apartment... just the clinic. Some of the other medications have been reduced, which is nice. So, we'll see how his blood pressure reacts over the next couple of days.

So, now for the excitement of the day... after clinic, we had to go to the second floor for an appointment with an ENT doctor. As a reminder, this is the same floor where Frank & I waited over an hour before anyone told us that we didn't have the proper paperwork. And, that was only after we confronted them a second time.

Well, let's just say that history repeated itself. Frank & I waited twenty minutes before I made sure we had the proper paperwork. The woman at the desk assured me that we did and that she put it back behind the velvet rope (sarcasm). Another twenty minutes went by and people were being called in that came after us. I went to the desk again to ask if our doctor was running behind schedule. They told me he was. Another fifteen minutes (now over 45 minutes total), I told the woman at the desk that my son is immuno-compromised and that we couldn't wait any longer. It was at this moment that they could not find Frankie's paperwork.

And, it was at this moment that I exploded. Okay, maybe "exploded" isn't the right word. Because, as Frank told me later, even when I'm mad, I'm still nice. Should I work on this??? Needless to say, we took the copay back and left. In their defense, they continually apologized to us, but I'm sick of that. This has happened both times we went to this floor, and it's only this floor we've ever had a problem. So, for other PBMTU parents that have to go to that floor... make sure they know you're there and they haven't lost your paperwork!!!

PS... I'm sure we have to reschedule that appointment.

PPS...Insurance finally kicked in, and our out-of-pocket at the pharmacy didn't match our mortgage this time!! Woo Hoo!!!

Thursday, October 13, 2005 - Day + 105
Okay, so the pattern seems to be this... if it's my morning to wake up with Frankie, he sleeps longer. So, Frank's not really happy with either Frankie or me right now. This morning was no exception. It was Frank's morning, and Frankie was up at 6:30am. To put this into perspective, yesterday, Frankie slept until 8:00am.

Around 8:30 I got up to give him his morning meds (now topping off at 8 syringes of various amounts). Then, the morning was ours. Frankie's only appointment today was a physical therapy session at 1:30pm.

Jamie, Frankie's therapist, is great with him. So patient and energetic. Keep in mind, Frankie is two and has been watching TV now for around 4 months straight because in the unit, there wasn't much else to do, and being trapped in an apartment just makes the space bigger... our options are still pretty limited. So, when Jamie comes over, we put Frankie to work. Sometimes, this is met with some resistance (sarcasm). But, today was a good day. Actually, it was a great day.

We had to get Frankie walking up and down make-shift steps in the apartment. So, we built steps up to the coffee table... yup... we've created the climbing-on-furniture monster. Anyway, he was getting bored with this--quick. So, I decided to sit on top of the coffee table with a blanket over me. I hoped this would tempt him into uncovering me ("peek-a-boo"). It worked for longer than any of us ever suspected and lasted the rest of his session, with some modifications along the way. Frankie even got to the point of walking up the steps on his own, stubbling a bit, but correcting himself. This is a very good accomplishment for an MPS III child as their mobility can be affected early.

On a side note, I was able to get a haircut today. It was great! Thanks to Kate, Frankie's Best Buddy for recommending Lars at Sheer Bliss (Durham, NC)!!!

Friday, October 21, 2005 - Day + 113
First, our sympathy goes out to the Roche family. Frank's cousin, Matt, passed away this week.

Second, Frank, Frankie and I went to the neurosurgeon's on Thursday to review his MRI. To recap, the MRI showed blood on the brain (back right side of his head). Fortunately, the spot was nothing to worry about. But, if you actually see the picture, the spot looks like it would be considerable. The doctor told us that spots this small usually resolve on their own (disappear) over time. So, when we do Frankie's 6-month studies in December, he'll get another MRI to check the size to make sure it's not getting bigger.

Over this past week, Frankie's meds have been reduced. And, despite the roller coaster of potassium and magnesium levels, his counts have all been normal. This past Wednesday, "clinic" or "doctor" day, he was only given Diclusimab (GVHD). Normally, he gets a dose of Cytogam (IVIG), but they tested out the response of his own immune system by not giving it to him. And, the result was positive. His immune system levels were well above the "warning" zone. Thus, he will now get this every two weeks, unless the level drops considerably.

Next Wednesday will undoubtedly be a long clinic day. Not only will Frankie be getting the Diclusimab and the Cytogam, but it's also Pentamidine time. He's given this drug one time, at the end of the month. This drug is used to treat Pneumocystis carinii (noo-moe-SISS-tis ) pneumonia (PCP), a very serious kind of pneumonia. This kind of pneumonia occurs commonly in patients whose immune system is not working normally, such as transplant patients. And, since Frankie only has one lumen to administer the drugs...

Frankie's Physical, Occupational and Speech therapies continue to go well. Frank is really working with Frankie to get him to mimic him as he's having trouble pronouncing the letter "L". With our luck, he'll mimic us, but at the wrong time and using the wrong word! ha ha ha

We decided to purchase some play dough today (recommended by OT). However, since Frankie's on two medications to boost his appetite, even after a big dinner, he was trying to eat the play dough. Thankfully, the stuff isn't toxic. Not that I'm letting him eat it, just in case it does reach his mouth without Frank or I catching it. So, needless to say, the play dough play time lasted all of a couple of minutes.

Frankie has a new love... Elmo has kind of gone the wayside. He is now totally wrapped up in Blue's Clues. And, amazingly enough, Barney has still maintained his ground. He yells "Clue" and "Blue" when ever the show is on. Hence, our ability to realize he's not pronouncing his "L" very well. In any case, he is incredibly animated when watching the shows. He dances, stomps his feet, waves his hands and makes all types of vocalizations... not to mention, laughing and smiling. It's really amazing to watch. He's getting to be so much more like himself, prior to transplant.

Many thanks go out to the Kids For Kids association that ran a major fund raiser for Frankie last week back in Connecticut. Apparently, it was a huge success. And, with their efforts, we may have reached our fund raising goal through COTA!!

FYI... I've added a couple more links to the right under the 5200 Club. Queen Maddy is no longer with us, but I know there are 5200 families who use my site to check up on her family. Frank & I met the Tatems the week they were getting discharged to go back home to Virginia. They were staying in the same apartment complex. And, Maddy Trimper was being discharged from the unit when we were admitted. And, as we are reminded daily how small this world really is... after they left, I found out that they live in Stanton, MI, a small town I used to live near back in the mid 1990's.

Also, for those of you who knew Hussain and Jana, I received an email from him last week. He is doing as best as can be expected, still mourning the loss of his sweet daughter. He's hoping to be able to come back to the States to get his bachelor's degree.

Thursday, October 27, 2005 - Day + 119
Okay, okay, I give! I've gotten enough emails from loving family and friends to politely indicate that I haven't updated the web site and that there is some concern.

Frankie is absolutely fine! In fact, he's doing very well, and behaving quite like the two and a half year old that he is... wow, is he really two and a half?? Oh yeah, five months were lost in a hallway somewhere! I seem to remember Alison (Kate's mom) writing that same line, or similar to, a while back, and it just stuck with me. Thanks, Alison!

I forgot to mention a couple of things that I should have last week. We had a nice visit from a couple of Frank's relatives. Tina and Rick won a trip (through one of Frankie's fund raising events) to Pinehurst Golf Club for the weekend, and were able to pop in to visit for a couple of minutes. They joked about who had more hair, Rick or Frankie... it was a tough call. And, I hope our directions were okay since they had a limited time to return their rental car and catch their flight! Thanks, Tina & Rick for bringing the T-shirts and Playbill from the Kids for Kids event!!

Also, Frank's mom was here this past week (Saturday to Tuesday) to visit. And, since Frankie's nights aren't so complicated (no IV medications), she was able to hang out in the apartment two nights in a row so Frank & I could go to the movies!!! What a treat!! We were able to see Serenity and Doom, two movies that we really wanted to see on the big screen... Serenity was definitely the better movie and stayed true to the TV series, Firefly, that Frank & I really loved to watch, prior to its untimely and unfair cancellation.

One of Frank's work buddies visited us on Tuesday, and brought Frankie the nicest electric train set! We put it together on the table that night and Frankie's eyes just lit right up! I think he got a kick out of the fact that it could go forward and backward. Thanks, Mike!

What else?? Not much, really. Frankie's clinic visits go very well, and while we would love to let you in on the big secret, we just don't want to jinx ourselves. But, to be fair, it's not like there's much of a secret to tell. Frankie still needs to be weaned off of some of his medications before they give us a going home date.

Frankie's counts are very good. But, his Magnesium is low, so we have to crush a monster pill and put it in his food. And, we have to crush another smaller pill (Folic Acid) to boost his Vitamin B level.

He's eating like there's no tomorrow. He eats every hour. He has learned the sign for eat (fingers together brought to the mouth) and is very self sufficient. He walks over to the pantry, opens the door and grabs whatever suits his fancy, and whatever may be in his reach. Speaking of reach... his little hands can get to anything!! Apparently, the apple does not fall far from the tree! My son has learned to climb on furniture and use his surroundings to get what he wants without asking mommy or daddy. Although, he knows that mommy is the weakest link!

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.

Sunday, October 30, 2005 - Day + 122
Well, for a long time now, we haven't hand any issues with Frankie's health. That all changed a bit this weekend. On Saturday, Frankie was extremely less active than normal. I'd say he was "lethargic" but the doctors and nurses reserve that adjective for children who are difficult to rouse. Frankie was not lethargic by their definitions, but definitely by Frank's and mine.

Frankie naps, at most, twice a day. Yesterday, he had at least four naps. And, when he was awake, he was just "blah." I wish there were a better word for his temperament, but it's the only one that comes to mind. In addition to that, his stomach appeared very swollen and firm to the touch. I'd call it distended, but I'm sure I'm using that word incorrectly as per our PBMTU lingo... but, it's the only word I know to describe what it looked and felt like. So, when he woke up at midnight, with what can only be called "not normal poop" -- I won't go into details should any of you be reading this on your lunch hour -- I called the doctor. Per our discussion, there seemed to be nothing to be concerned about at the time.

Sunday morning (my morning, for those keeping track), I woke up with Frankie at 4:30am. And, for those of you who are interested, I went to be around 2am. These are pre-clock-turning, so, yes, that's about 2 1/2 hours of sleep. At 5am, I gave him a little "snack:" to tide him over until breakfast and a little water-juice. Fortunately, he fell asleep between 5:30 and 6:30am with me on the couch. Roughly two hours after he had his little snack, not doing anything but sitting on the couch with me watching Barney, he vomited. I was so happy I hadn't tried giving him his morning meds yet.

After waiting 30 minutes and cleaning him up, we gave Frankie his morning meds. Vomiting must have drained him, or at least that's what I was thinking, because he fell back for a nap from 8:30am to 10:00am. Unfortunately, upon awaking -- again, not having eaten for over three hours and meds for 2 hours -- he vomited a second time. Immediately, I called the day hospital. The nurse practitioner on-call requested a blood sample to be dropped off asap, which we did. We were planning on doing a bit of grocery shopping, so we figured we drop off the blood and then go.

While at the day hospital dropping off the blood sample, they weighed Frankie to make sure he wasn't in need of extra fluids (losing weight compared to Wednesday's weigh-in). And, he had actually gained weight from then, so they weren't concerned at this point that he may be dehydrated.

After stopping there, we went to Target and did some shopping, only to realize that we cannot bring Frankie out in public any longer. Almost everyone we walked by was either coughing or sneezing! So, you can only imagine how quickly we tried getting out of there. Only, it wasn't quick enough. While in the check-out line, Frankie vomited again. And, for those of you who aren't aware, Frankie has to wear a green mask EVERYWHERE. So, he was vomiting in the mask, and freaking out at the same time. The first thing I saw were his arms flailing about while he was crying out loud. He was panicking, too, trying to pull his mask off. Luckily, Frank & I had brought extra masks with us, just in case. Frank immediately brought him to the car, while I paid for the groceries and called the day hospital, again.

The nurse told us just to go home and wait for the blood tests to come back. If he needed anything (fluids, medication through IV), we should be prepared to come back to the clinic for such necessities. So, by 12:30pm, we were back in the apartment, and I was doing yet another load of laundry. Frankie napped again from 1pm to 2pm. By that time, I had gotten the results from the blood, and everything looked okay. But, everything wasn't "feeling" okay....

At 4:00pm, Frankie vomited again (4th time)! And, this time when I called the clinic, they asked us to come in for him to be checked out more thoroughly. So, we packed up for the night (laptop, DVDs, knitting, food, etc...) and headed back to the hospital. Every time we go to clinic, we never really know how long we'll be there, so we just make sure we have enough with us to entertain Frankie and keep us sane! Thankfully, we were only there an hour. On a brighter note, we did get to see Kate and Alison for a little bit while at the clinic, but, unfortunately, it seems like they were having the same kind of weekend we were. Kate, we hope you're feeling better! :)

As of this writing, it's now 10:24pm, and Frankie's sleeping soundly. He appears to be feeling a bit better, and at least has not vomited any more today... but the day's not over... :)