Delay....

Another delay!!!!

It turns out Max will not be going into the hospital until Monday. He was supposed to go in tomorrow, but the plans changed. They want Max on his antibiotics for a few more days because of the "bugs" they found during the bronchoscopy. It will also be easier to get lab results from his first chemotherapy drug on a weekday instead of a weekend. Thus, we must wait a few more days to begin his treatment. We are going to make the best of it and enjoy our last few days of freedom. Hopefully, nothing else will delay us.

We had our final meeting with Dr. Grewal to discuss the transplant protocol, it it was scary to say the least. He had to tell us everything that could go wrong during transplant, and there are A LOT of things that can go wrong. Most things can be treated, but of course many others can be life threatening. We then had to sign the consent forms, and we felt in some way we were giving Max up to the doctors. A parent never imagines having to put their child through such drastic measures, but we know it is the only thing that can save Max at this point. We have total faith in the BMT staff and their desire to take the best care of Max. We hope and pray that all things go well and that Max will be a transplant success!

Back to RMH

Max came home from the hospital today at about 3 PM. He is feeling much better, and is pretty much back to his old self. He was pretty uncomfortable last night after all of his procedures, and was very exhausted. He got a little morphine at 7 AM and that helped calm him down. He also drank a lot of apple juice. I went home at about 8 AM to shower and by the time I got back to the room Max was feeling much better and was watching a video with Dad. He was walking around in his crib, and trying very hard to pull and chew on his oxygen tube, IV and central line tubes. He also worked very hard walking around the crib to avoid his breathing treatment, which was pretty entertaining.

As for his tests results, we received mostly good news. Max's spinal fluid pressure was well within the normal range and the fluid on the MRI looked good which means he will definitely not be getting a shunt! His lungs looked good during the bronch, but the culture showed some bacteria. It may be from is lungs or his mouth, but to be careful he is getting an antibiotic 3x/day. He is also getting antibiotic ear drops to prevent infection in the new ear tubes.

The thing to worry about now is his spine. Let's see if I can explain since I don't totally understand it myself yet. Like some Hurler children, the "nub" connecting Max's first and second vertebrae is compressed and underdeveloped. Also, the space in which the nub sits is narrow, even for a Hurler child. Both of these can lead to spinal cord injuries so we have to be careful with Max's activities - no gymnastics, sports or things where he may sustain impact to his head. His situation may change over time and there is nothing they can do about now before transplant so we just have to wait and see. It is a bit hard because as soon as we get some positive news something new comes along to worry us.

Tomorrow we have to go to the BMT clinic to meet with Dr. Grewal to sign all of the transplant consents and ask any remaining questions. Max's lines will also be flushed and his dressing will be changed. He is set to be admitted Friday and begin chemo Saturday.

Please pray for Grace's new friend Kendall who was admitted tonight for her transplant for leukemia. She is a great 5 year old girl who is very brave and strong. Grace is going to miss her while she is in the hospital and looks forward to playing with her when she comes home.

-Margaret

More Prep

The longest scariest day so far is over! We had to stop Max's fluid intake at 7:15am and they finally let me in to Recovery at 5:15pm. He is awake now, and in a room up in 4A which is where he will be for his transplant as well. The admitting for that is scheduled for Friday, but today . . . that's what this note is all about.

1. Once he was asleep Max first had a bronchoscopy. This is where they use a tiny camera and some other small stuff and look around the inside of the lungs. The preliminary report on this was very good. They also took some samples from inside to test for any lingering infections. Max's lungs have been the cause for a lot of concern since we've arrived, so we were pleased with the results.

2. Next, Max had new PE Tubes put in. He had tubes already, but as we understand it, they want the kids to have as clean of a slate as possible.

3. Spinal Tap. Not the movie. Around here it's called a "lumbar puncture." Our transplant doc, Dr. Grewal, did the procedure himself and came out to tell us that Max's pressure measured very well and that it did NOT appear that he would need a shunt because of the pressure he was seeing.

4. His Hickman catheter was placed 4th. This was over quickly, but it involved connecting his jugular vein in his neck with a "foreign body" - a catheter tube and then passing it under the skin and out of his chest. This is the standard look for transplant kids. The end of the tubing on the outside branches off into two other tubes and can be used to administer meds and draw blood. You can actually feel the tube under the skin, and that's kind of creepy. Hopefully Max will learn not to pull on them, since he's going to have it in for at least a year.

5. MRI of the head/CT Scan of the chest. We haven't heard yet about the CT, but the MRI went well as far as the head was concerned regarding hydrocephalus. Again it showed that Max needs help, but his skull is still soft and can handle the added pressure his disease is giving him. The best solution for Max's hydrocephalus now is transplant, not a shunt. This was a great relief.

The MRI did, however, point to a more serious concern down the road. Once in recovery I met Dr. Peters. He's the head of the transplant team here, and head of the inherited metabolic storage disorders, and a pretty important dude. He also happened to be the Doc on the unit during recovery. He said the MRI showed that Max's vertebrae at the top of the spine are immature and potentially dangerous to his spinal column. We will learn more about this tomorrow, but this is something that he has been seeing in other Hurler children. His vertebrae are at risk of sliding apart from each other and causing severe spinal damage. The surgery to correct this is quite severe as well. He said we won't be worrying about this prior to transplant, but it will have to be monitored. Some kids do grow out of this on their own, over several years.

After his procedures, they had to "extubate" him and though they could have left him on a vent overnight, they were able to get the tube out and get Max onto regular oxygen fairly quickly. When I got to him, his tube was out and he was asleep behind an oxygen mask. The dressing from his Hickman was a little bloody and he still had his bladder catheter so he looked a little weary. Still, I can't easily describe the pride I had for my son for getting through what obviously was a very full day.

Margaret joined me once we were out of recovery and upstairs on the BMT unit. She'll be staying with our little guy all night. This is the unit where we will have Max for the next 6 months or so. Depending on the results of the samples taken from Max's lungs, if all goes well he'll be admitted Friday and chemo will start Saturday.

I'm back at Ronald's house now and I just got Grace to sleep (at 11). She's had a fever for 3 days but appears to be coming out of it. She 's doing a really great job trying to understand all of this and still be a 3 year old. Hopefully she won't pull his Hickman line out of his chest. Considering it's attached to his jugular, things could get messy quickly.

Finally, I realized that we haven't been able to express our gratitude in these updates, but when we get a chance we would like to express further our appreciation for two recent events: First my sister Dana sponsored a "pampered chef" party in Max's honor and our first official fundraising effort was a success. Second, my friend Joel Lava paid us a visit (he was in town for a wedding) and since he lives in LA, it was an awkward coincidence to be able to see each other, but one the meant a lot nonetheless. It's nice to know that we are being thought of so kindly and when we get a chance we will try to express our thanks more thoroughly.

Please continue to root for our little guy. His strength and determination will be put to the test shortly, and he needs all your help.

Sleepily yours,

mike

PS Our waiting room material today contained a lot more information about Bob Hope's links to Minnesota than I ever knew existed. "It's great to be here in Minnesota, land of 10,000 lakes. And the way I golf, I've been in all of them." Bob Hope (RIP)

Weekend Update

We had a pretty good weekend here in MN. It was strange waking up Saturday with no appointments to go to. Max was stuck in the room all weekend because of his cold, but we still managed to have some fun with Grace. Saturday Mike and I took her to Target - and you know how much Grace and Mom love Target! Grace entertained herself by trying on a million sunglasses, hats and purses while Mike and I bought some stuff to organize our room.

Sunday Mike, Grace and I went to the Mall of America and Grace rode the rides. A picture of Grace riding a shiny pig should be coming up soon . . . We then came back to the RMH where a local MCDonald's had a little fair for the kids in the courtyard. They had games and prizes and then made dinner for everyone. A guy who was working the fair was actually a former RMH resident who is now healthy. I have visions of helping out at RMH with Mike, Grace and Max when all of this is behind us and Max is much healthier.

Our new room became available a day early, so we got to move in last night. Grace was supossed to watch a movie in her friend Kendall's room while we moved but she came down with a fever and now has to stay away from everyone. She was so disappointed, but we knew she was sick because she didn't leave the bed to check out the new place.

Speaking of the new room ... it is awesome. It is a suite with a kitchenette, living room area with fold out couch, huge bathroom, bedroom (that sleeps 6), and walk-in closets. Grace has cool bunk beds. There is a desk as well, and we can now spread out and feel more comfortable for the next few months. The first room was nice, but this is much newer and bigger. It feels much more like a home than a hotel.

Today Max had an EMG, which tested the nerve conduction in his wrists for carpel tunnel syndrome, a common effect of Hurler Syndrome. Grace and I stayed at home while Mike and Grandma took him. They stuck electrodes on him and shocked him with electricity! Mike said it was horrible. He does not, however, have carpel tunnel for now. They will monitor it post transplant, as many Hurler kids continue to have joint and bone problems even after a successful transplant.

Grace still had a fever this morning so we called the social worker who gave us a Dr's number. We called the Dr and she couldn't see us today so she advised us to take Grace to the ER. She shows no other signs of illness besides the fever and a little stomachache. We just got back and they said everything looks normal. We just have to watch her and use good handwashing. I don't think it is possible to wash any more than we do but we will try!

If anesthesia clears Max tomorrow he will be in the OR for a long time for all of his procedures and surgery. He will stay overnight for observation. If the MRI shows he does not need a shunt he will be admitted to the BMT unit Friday and begin chemo Saturday. Say prayers that all goes well!

Love, Margaret

Work-Up Week Day 5

Max fans,

Today was another busy day. We started out with a disappointing trip
to the eye Dr. We knew Max was slightly far-sighted, but the Dr. was
surprised how much. She said his eyes are still growing and he's able
to adjust for most problems so she does not want to give him glasses
at this point. She also noticed that his right eye drifts a bit. He
also has corneal clouding which most Hurler kids do. This should
improve after transplant. The most troubling finding, however, was
signs of retinal degeneration. His blood vessels in his retina are
smaller than normal. While the progression of this is very slow, the
ultimate outcome is loss of vision. The first to go is night vision
so we are to look for signs of that. When I asked her if we could
expect it to happen in the teen years she said maybe even later than
that. She couldn't tell us for certain because not many Hurler kids
have lived past their teens. Talk about depressing! We are to see
Dr. Summers again in 3 months.

We then met with Dr. Milla, the pulminologist, who thankfully reported
that Max's wheezing is getting better on the new meds. He cleared him
for anesthesia on Tuesday. However, Max woke up with a runny nose
today! He must have caught a cold somewhere, despite our manic
efforts at germ control. If he doesn't get better by Tuesday we are
worried that the anesthesiologist won't let him go under for his
procedures.

We then met with Dr. Grewal, Max's transplant Dr, who told us what we
already knew - the week did not go as smoothly as hoped, but it wasn't
as bad as Mike and I thought. He is concerned that Max's head size
grew as much as it did since we were out here in June. It is very
important that they be able to do the MRI on Tuesday to determine if
he needs a shunt. Dr. Grewal also said that they haven't had a case
as complicated as Max in a while.

We then went to see the cardiologist. Max has a mitral valve
regurgitation, a common problem in Hurler kids. His is mild, thank
goodness, and she does not need to see him again for 1 year, provided
there are no heart related complications during transplant.

Max had to get MORE blood work today and we also dropped off a
urine test we had to collect ourselves. Mike was a little lab
technician last night, with 3 pieces of urine soaked paper drying on
saran wrap!

We have the weekend off from Drs appointments and we might be moving
to a bigger room on Monday. Here's to hoping Max gets better, no one
else catches the cold, and he can be tested on Tuesday.

Also, if you have time please send a nice message to Bella, who got
her new cells yesterday (Friday).
(http://www.caringbridge.org/ia/isabella/index.htm)

-Margaret

Work-Up Week Days 3 & 4

It's Thursday night, and we've been through another couple of interesting days. Yesterday, since all of our tests were cancelled we only had a consultation with our nurse coordinator, Teresa, and she took us through the expected "protocol" for Max. This is where we were shown an actual calendar for the first 40 days or so. We learned about Max's chemo regimen and about some of the major drugs he will be on during the first month and beyond. The amount of chemicals that will be flowing through his little body is staggering. It's mind boggling to think about what it's taken to get to this point, where there is at least a somewhat standard practice for moving forward. We've come to peace with our decision to be at Minnesota. We have been impressed with the thoughtfulness and thoroughness of everyone we've met. We are scared about the future, but we know that we couldn't be at a better place.

A piece of information we found interesting was that the new stem cells will be taking over Max's blood and changing his blood type and DNA. His DNA throughout the rest of his body will remain unchanged. So, in the future, Max's hair and blood will have different DNA. Look for a future episode of "Law and Order" to explore this phenomenon.

As for Thursday, well, today was a little bit rougher. We began the day with a meeting to learn about his "central line" placement. This specifically is called a Hickman catheter and it is what will be in Max for the next year at least. It is a tubing that connects from his jugular inside his neck, goes under his skin, and comes out his chest. This tube will allow him to have meds put in and blood drawn out. We will have to keep this area super-clean to minimize the risk for infection.

Then we went to audiology and Max paid attention again to the loud noises and ignored the low static sounds. This was consistent with his tests that he's had so far back home. Not the best news, but no worse than usual.

Then we waited THREE HOURS for a dentist to come and look at Max's teeth. He did one little scrape and told us they were fine and though they were coming in a little black, it was just staining from multivitamin.

Finally, and after Max had been fasting for over 4 hours, we began our worst emotional torture so far -- an upper GI study. This was what the Gastrointestinal doc ordered for Max so he could see if his esophagus and windpipe where "communicating" To do this, Max had to drink a barium mixture and be looked at with a fluoroscope. They told us they would have to strap him down to a board and they would take a few pictures of his chest and guts over some time intervals. What they didn't tell us was that if Max wouldn't drink barium (and who would for God's sake?) they would have to stick a tube up his nose and down into his stomach. They also didn't mention that the "board" was actually some sort of NASA training device that allowed them to tie down Max with his arms tucked BEHIND his head and then to spin him over on his side as the liquid sloshed around his belly. The good news is that they didn't find anything wrong anatomically to explain his wheezing or reflux. So it's good news because Max won't need any new procedure for anything gastrointestinal prior to transplant.

Whew! This is a long email. In other news, they had a BINGO night here and all the kids came down. The prize cart was a sight to be seen. I am sure all of the prizes were donated, so consider dropping off some toys at a local Ronald McDonald House near you. Luckily for all involved, Grace had a lucky card in the first game, and got a new Barbie. Her friendship with Kendall continues to bloom. She has a form of leukemia called ALL and she'll be admitted for transplant at around the same time as Max. So they'll be transplant buddies and Grace will have two people to visit.

I gotta go check on Margaret and the kids and we have to watch a transplant video tonight and go over our consent forms. We have to sign over the rights to our son's life basically, and from what we've heard, it's a little daunting to see it all written out.

Good night from Minnesota,
mike

Work-Up Week Day 2

The second 9-hour hospital day brought us:
1. More blood work
2. Pulminology
3. A meeting with Stacy, our social worker
4. A "Caregivers" class
5. Gastrointestinal
6. Neurology

The blood work was quick and easy. Yesterday they took the maximum amount of blood for Max's weight. They wanted to retest Max's clotting number. No results yet. Pulminology was what we were most afraid of. And our fears were realized when Dr. Milla told us he heard wheezing in Max's lungs.

He wants that cleared up and under control before transplant and before the long OR session to do Max's MRI, bronchoscopy, endoscopy, CT Scan, and central line can go in.

That was supposed to be tomorrow, but now we have to administer more meds via his nebulizer and that awful struggle now takes about 40 minutes. Hopefully these new meds will toughen Max up quickly and he can have his transplant soon. The surgeries have all been postponed until next week. He will see us again Friday.

Stacy is the same social worker we met on our brief visit before and she's great. She's there to help us with any concerns we have both medical and non medical. Right now she's looking into schools and classes for Grace.

The Caregivers class was interesting and they have them every Tuesday for people who are caring for terminally ill patients. These classes are both informative and are also a sort of group therapy. We couldn't stay for the whole thing but we can see where we might learn a lot about how to better take care of ourselves so we can better take care of Max.

The gastrointestinal consult was for Max's acid reflux and he's ordered up another chest X-ray, this time involving Max drinking a barium solution. Sounds delicious. This X-ray and the endoscopy will be to determine if Max's wheezing is not just from weak lungs, but from his esophagus leaking into his trachea. Yikes.

Our last meeting was with neurology, and Dr. Charnas had a resident with him. As he described to the resident, the recent burst in gross motor skills in Max is "truly remarkable for someone with a bowling ball for a head." He seems to feel that Max is doing very well and that the best course of action for his hydrocephalus is transplant quickly. If things are going to be delayed for other reasons, there is a Plan B, and that is to begin enzyme replacement as way to help some relieve of the pressure in Max's head.

That's about it for today in hospital news. Grace and Gram Chick went to a story time and lunch at the house and then there was a big group art project after dinner. Grace is making a lot of friends, some of whom are getting work-up done like Max, so they will be heading to the hospital soon. Some of them wear masks because they're out of transplant but still very immunosuppressed. She thinks it's great to be making so many friends. Her best buddy so far is Kendall, 5, a girl who's leukemia has come back and she's scheduled for transplant on the 29th. All it takes is 5 minutes with a kid like this who has such a great outlook on the whole process. She makes you realize how precious your kids are, and also how unbelievably difficult the whole process is going to be.

Keep thinking good thoughts about us and all the kids here at the house and in the hospital.

Lots of love from Minnesota,

Mike

Work-Up Week Day 1

Well, Max began work-up week with a bang! Today Max had:

1. Blood work
2. EKG
3. Physical exam
4. Neuro-psych testing
5. Chest x-rays
6. Echocardiogram
7. Neuro-psych results

We actually ran so behind on schedule that Mike and I missed the scary BMT Class we were supposed to attend. Not to fear, they will scare us to death on Wednesday when Max is in the OR for his MRI, spinal tap,
broncoscopy, endoscopy and CT scan. Max's lungs are still a concern as he is still

wheezing. The pulminologist and anesthesiologist have to sign off on him to have the procedures on Wednesday. Those tests will tell them if Max is ready to be admitted and begin chemo. If all goes well, he will be admitted next Tuesday. Other test results from today worth noting are the blood and neuro-psych. All the blood came back normal except one number that has to do with clotting. They are going to redraw that one tomorrow. The neuro-psych test also showed he is slightly delayed in speech and language. We were a little surprised as Max is constantly babbling and singing. Hurler kids are often behind in this area and though we left feeling a bit sad, we are hopeful that his speech therapy will help with this problem.

We are off to bed now. Our nervousness and chatty Max (he was standing in his crib at 3:45 this morning babbling) kept us up all night. We are hoping that his pulminology, GI and neurology consults go well tomorrow.

Love,
Margaret, Mike, Grace and Max

At the Ronald McDonald House

Max fans,

Today marked our 3rd day at the Ronald McDonald House and we are settling in nicely. The place is amazing. There are over 40 rooms,
all full with families who have children with terminal illness. You would think you'd be falling over people here, but it is actually very quiet and seems empty at times. You see most people at dinner time. Almost every night a local group comes in and makes dinner for everyone! There is also a video library, book library, computer room, game rooms, play rooms, playgrounds, a gym and a full basketball court in the basement. There are planned activities for the kids as well.
Grace is having a ball. She made a new friend named Kendell who is 5 and is beginning her transplant work up for leukemia. Grace will be going to a book club on Tuesday with Grandma Chick.

We also had the opportunity to meet some other amazing Hurler families this weekend. A few doors down from us are the parents of Bella (http://www.caringbridge.org/ia/isabella/ ). a little girl who began chemo last week. Taylor and her family have been here a while and they have been helpful as well (http://www.caringbridge.org/mn/taylor/). Today we met Aaron Athy (http://www.caringbridge.org/il/aaronathy/), who will be going home
tomorrow (way to go Aaron!). And last night we met Susannah
(http://www.caringbridge.org/page/susannah/) who was here for a follow
up visit. All of these families give us hope that we can make it through this.

Max has a very busy week ahead of him. We are to arrive at the BMT clinic at 8 am tomorrow to begin his appointments. We don't have the full schedule yet, but we do know that Wednesday will be an anesthesia day. This is scary for us, because anesthesia is risky for Hurler kids - so say some hot prayers for Max that day! He will be getting an MRI, spinal tap and bronchoscopy.

Thanks for checking in on us. Keep checking the web site for updates and pictures from MN!

Love,
Margaret and Mike

Last Update from Illinois

To all of Max's family and friends,

This is the last email from Illinois. Tomorrow morning (Friday, July 18) we are heading out caravan-style for the trek northwest. My mom is driving our other car and it's a good thing because we've packed up enough stuff to fill both vehicles.

We had a goodbye dinner/party at Margaret's sister's house and it was a nice way for us to be able to see some people one more time. It touched our hearts to know that so many people in our lives feel such an attachment to our son. Both this party and Max's first birthday party were nice celebrations of the determination of our Mighty Max. Our hearts are heavy and filled with hope as we are about to hit the road. We know that this is what we must do so that all of the people who have come to love him so much will be able to do so for a long long time.

Some important info about contacting us:

For the next week or so we will be at hotels near the hospital. There simply is not a room available at the Ronald McDonald House. We will not be checking email for a while, but once we have a more permanent home we will be in touch. After that, we are unlikely to be filling your mailboxes much more. We will be writing to the website as much as possible and that will be the way you will all be updated. That’s not to say that some of you won’t be getting individual emails at very odd hours of the day, but we are unlikely to continue this mass mailing.

We will be checking the site when we’re in Minnesota. Why? Because we’re addicted to our guestbook! It’s been so encouraging to read so many emails and be introduced to so many wonderful families. Please continue to check in and think of us! Special thanks again to Chris Vasilakis for doing such a great job on the site.

In other news, Max is obsessed with standing continuously. This means that he is unbelievably frustrated when he’s not either shuffling along a wall or just standing against the TV. This morning he maneuvered Jackie Chan-style across two chairs, a table, a bench and a banister to climb up two stairs. Luckily, his grunting gave him away and I was able to make a rescue before he slid downward. He also began feeding himself a couple of weeks ago, and we think he said ‘up’ the other day. If he didn’t have this disease, it’s amazing to think what his potential would be. We can’t wait to discover his new potential after transplant. Grace is doing well, and amazes us with her intelligence and imagination. Last night she was able to sound out words from a book, and is able to distinguish nouns, verbs, and adverbs. We know she is going to miss her friends, her backyard, and her ‘beautiful bedroom,’ but we are hopeful she will adjust well to her MN home.

Wishing everyone the best and we’ll be in touch from the land of 10,000 lakes.

Mike & Margaret

Minnesota Delayed by One Week

Hello Max Fans,

As some of you know already, our trip to MN to begin the process of Max's transplant has been delayed by one week. We were supposed to begin his pre-transplant work ups on Monday July 14th, but due to a delay with some blood work we won't start things until July 21st. At first we were disappointed, but it is going to allow us more time to prepare, pack and enjoy our freedom before the lengthy process begins. Max, Grace, Mike, Mike's Mom Jane and I will be leaving on Friday July 18th so we can attend a BMT picnic at the hospital on the 19th. We are really looking forward to meeting other transplant families, especially the ones we've already "met" via email.

We also want to say thank you for al of the support and help we have received from everyone. The nice notes left in our guestbook are so wonderful to read after a stressful day - keep them coming!

We will keep you posted of any changes to our schedule. Keep checking Mighty-Max.com. We will be posting some new pictures soon. Also, please visit the sites of Max's Hurler Friends and leave messages in their guest books.

-Margaret