Day +78 - Happy Halloween!

Mike and I just got back from the RMH Halloween party and we had a great time. The day started with book club for Grace at 10:30 and then a spooky lunch at RMH, full of yummy mummy hot dogs, a jello brain, halloween cut-out tortillas, etc. Mike, Auntie Peeps, Auntie Katie and I all went and the Grandmas watched Max. Mike and I came back to the hospital for a while and after a nap Grace came over to see her brother and show the nurses her costume. We then left again and the Grandmas returned. We had a great time doing the RMH scavenger hunt and trick or treating. Grace got so much candy and so many treats it will last us all year. We have said it many times, RMH is so amazing and I do not know what we would do without it. We would be so depressed and lonely, and Grace would not be handling this awful situation so well.

As for Max, he "wore" another Halloween t-shirt today and donned his Frankenstein headband for visitors. For most of the day we were in very high spirits, and as I told the nurse, I thought we deserved a "denial day." The fun is over now, however, and as Mike and I walked back to the hospital we walked back to reality.

Max had a quiet day with some small improvements. His blood gases have been up and down today, none of them terrible. He stooled a couple of times and his abdomen was 1 cm smaller than yesterday. His weight was down .8 kilo and he did a great job getting rid of some fluid. We were somewhat excited for all of this, but then it hit us that if the GVH doesn't go away none of the other progress matters one bit. His skin looks a tad better after the four doses of ATG. We have to see some dramatic improvement in the next few days.

Thanks so much for all the recent guest book entries, emails and care packages. Each note, each gesture goes a long way to raise our spirits. Thank you.

--margaret

Day +77

Max made a little progress with his kidneys. He's finally beginning to push more urine out and for a time today actually got rid of more fluid than he was given. The biggest problem is that a large amount of fluid is in his bowel -- his beat-up graft-vs-host bowel, and it just needs to hold out while his kidneys slowly help him take off the extra fluid. It's just that his bowel is so unhealthy that the worry is that a perforation could happen and if so, surgery will not help him, he will be too sick to heal. One step at a time. They also made a small adjustment on the oscillator today for the first time since Tuesday. So that's good too.

Tonight a surgeon came by and talked to us. His line was "I'm afraid what I have to say won't be very comforting to you." He told us that if Max's symptoms were in one of us, we would be on the operating table. And he told us that Max's bowel is possibly "asleep" and in danger of totally clogging up. But Max's graft-vs-host disease, combined with his lung issues, and transplant in general make him a very complicated boy. He explained that there are no real surgical options for Max if he has perforated his bowel and that he wouldn't make it to surgery anyway. He did put in a bigger tube to help suck up some of the bile and fluid from the stomach and small intestine. He thinks it's one thing he can do to help the distension without surgery.

Sadly we must also report that Grace's buddy Kendall lost her fight with leukemia yesterday morning. As most people know, her family had been extremely helpful to us and we are so lucky to have met Kendall and for our daughter to have such a good friend to look up to. We miss her and her family so much, but we know that Kendall is at peace and that her family was able to surround her with as much love as possible in her last days. Please pray for her family to find the joy of her memory to ease the pain of her loss. Their pain is unimaginable even as frightened as we are for Max. Just a couple of days ago RMH gave us the Christmas cards they produced with Grace and Kendall's picture on them. It seems like it was a lifetime ago that they posed for that photo.

Today we received a note from a friend of our neighbor Jodie. This family, from Michigan, has never met us but their daughter was born with many severe birth defects. The head of pediatrics at the hospital gave them a button that reads "I Believe In Miracles" and she wore it every day. Jodie has told us often of this little champion, her name is Sophie and today she is 3 and doing much better. She and her family endured the same kinds of fear as us when she was born. They sent us the button that Sophie wore and now Max is wearing it. We pinned it to a Halloween sweatshirt that we had to cut open so we could wrap it around his chest and arms. Max looks amazing. He's been in hospital-issue blue shirts for the last 2 months and since the recent events, he's basically been nude. So a little change of clothes puts us in the Halloween spirit and the button has given us a bit of hope.

We hung up some get-well posters that my Aunt Lois's Girl Scout troop made for Max. And we hung some Halloween decorations in the window. We love Halloween so much and we miss being able to decorate our house and going trick-or-treating down the block. But we've tried today to make the best of this situation and it's gone a long way towards improving our spirits.

Please think about Max's little button and what it says. This button already helped one little girl and we're hoping that it'll change our luck. The doctors are still very cautious about Max but he seems to have surprised them a little bit by restarting his kidneys a bit today.

We are really hoping for a quiet night and quiet day tomorrow so that we can attend the RMH Halloween party with Grace tomorrow. She looks so pretty in her Belle costume (thanks again Gretchen and Katie) and you can tell she feels pretty when she wears it. Grannie and Grandma will be Max sitting while we attend with Auntie Peeps and Auntie Katie.

Good night to all and have a Happy Halloween,
Margaret & Mike

Day +76

LATE EVENING UPDATE

Max didn't make any significant progress today. The relative success of last night was mostly maintained, but he has needed a little more oxygen this afternoon than last night.

Some good things: He is urinating more, almost as much as is going in him. He doesn't have the low blood pressure associated with sepsis. He was surrounded by much love today, with so many members of our family making last-minute trips to see him.

Some not-so-good things: He is still on high settings on the oscillator, and the process of moving back to a regular ventilator seems far away. He had an ultrasound today and it showed that his bowel is extremely distended. If it's his graft vs. host disease, we can only hope that the medicines begin to work otherwise it may have just shut down. He is very very sensitive to touch and may be in more pain. His blood pressure and heart rate increase with anyone touching his skin so they more than doubled his narcotic.

Max isn't quitting. And the question we have is why? He must be so uncomfortable. But he's clinging to all the love that he's getting from us and from so many kind words and deeds on his behalf. The doctors have prepared us for the worst, and we've decided to take our cues from him. When he's ready to stop, we will try as much as we can to have it be as peaceful and gentle as possible. Until then, we will work on his behalf to help him get the best care he can.

Today he squeezed our fingers a little bit more and he even had one small stool. This isn't much, but for Max, as sick as he is, it takes tremendous effort. I wish I could paint an accurate picture of how much Max has been put through. We counted his meds today and he is on 27 different medicines, plus 6 drips. He gets over 55 infusions of something per day.

Our emotions are up and down. His health seems so fragile. When one stops to add up all the hurdles he must overcome to get better it seems impossible. But there is a sequence to it, an order, and step one is to just maintain his breathing on this machine so his lungs can heal and his graft vs. host medicine can work on his cells. We've tried hard to understand why his new cells don't like their new home. Who wouldn't love this beautiful boy?

Hoping again for a peaceful night, stronger lungs, and happier cells,
Mike & Margaret

_________________________________

MORNING UPDATE

Our son has made it through the night. We had come back in the room after our talks with the doctor and we looked at the oxygen saturation and said, "Is that 87%?" and the nurse said, "No, 97%." That little fighter. He had one more night of fight and we'll see how much more he has. Maybe yesterday's problems were related to another small lung bleed overnight and it took the rest of the day to recover. We don't know. They were able to turn down his oxygen needs a little bit and while he's not much closer to being able to switch vents and have surgery, he did make progress. The resident said she was "very very very very worried" but she was ready to take one of the "very"'s away.

We have been reading the guestbook entries and we really appreciate the support.

At this time we have to say thank you to the resident Judy and the fellow Eric who both finished their month-long rotation yesterday. Thank you for making our son's life so important to you and for taking good care of us as well. Thank you for pushing for things and for questioning things and for not giving up. Special thanks also to the team of nurses yesterday, it wasn't an easy assignment, but we got great care.

Hoping for a better day,
Mike & Margaret

Day +75

EVENING UPDATE

While Max's day and evening yesterday were very stable, his overnight was rough. He began to have low blood pressure at around 5am and his oxygen needs on the oscillator went up at around 2:30am. Neither really rebounded and he sent the doctors and nurses scrambling for answers.

Now it's around 5pm and we just had two very important conferences. The first was with the team of transplant doctors who told us they're doing all they can for Max, but because of many factors, they can do no more at this time, and here's why: He is retaining fluid but can't make it to dialysis because his breathing is not stable on the oscillator and therefore can not be transported to surgery. Additionally he is not oxygenating throughout his body very well. This could be because of the bleeding that happened in his lungs, a virus in his guts, or a number of other things. He may be septic as well since he's having trouble with low blood pressure. The biggest mystery of all is the graft vs host disease. It's affect on all of his systems is hard to guage and so far it's been impossible to conquer. They were clear with us: Max needs to make dramatic progress overnight or the options become more difficult, the risks become very great for all procedures, and death is very likely for any number of reasons. They don't seem very hopeful and that's because they are just being honest. It's hard to see the doctors so frustrated and we know in our hearts that when they tell us there is nothing more to do it is going to be hard for them too. Everyone here has been working so hard, especially Max, and it's heartbreaking to say the least to think about all the effort that has gone into saving our son, and that it might soon be over.

The second conference we had was with Grace to tell her that her brother could die and that this might be her last night with him. We cried more than her, but she still was very sad and said "I don't want him to die" and we told her that's why she should spend some time with him and sit with him. She told us she would do that "after I finish my chips." We told her that we would have to go back to Illinois and be in our house without him and she cried again. We don't think she really understands, and that's OK. We just wanted her to be a little more prepared.

Margaret's Mom and sister Katie are coming in very late tonight so between them and my Mom we should be able to be together at the hospital for whatever happens.

Please pray for a peaceful night, whatever the outcome. Max is the bravest person we know. And we miss him so much.

--Mike & Margaret

Day +74

WBC 12.5

To be quite honest, I don't really feel like typing an update tonight. So many things have happened over the past few days it is getting hard to remember all of them, and I am drained. But, we know there are so many people out there who check on Max and worry about him. We appreciate you so much and I wouldn't want you to worry more if you didn't see an update.

Today we added the pediatric renal team to our list of specialist following Max. He is so swollen with fluid and just cannot seem to urinate it all out. They ordered some tests and did an ultrasound of his kidneys. The results are not back yet, but the docs are guessing that his kidneys took a hit when his lungs were bleeding and they were deprived of oxygen. In a healthy person this would usually resolve itself, but we all know Max is not healthy so they are not sure what will happen. If the test results show something else is going on or if he doesn't make any progress, he will be put on dialysis and the fluid would be drawn off. This would involve him getting a new central line put in, which means surgery. If his fluid status starts impacting his breathing more they will have to move sooner rather than later.

As for his lungs, they have continued to wean him off the oscillator little by little. Once he hits certain settings he will be moved to a conventional vent. There is no real hurry to do this, as this vent is not hurting him. However, they would be able to do more tests on him on the conventional vent. All of the bouncing on the oscillator makes it impossible for him to get an EEG and we therefore do not know where he stands seizure wise.

The big change today involves his GVHD. It has gotten better at different periods with certain medications, but overall it is very bad. His skin is as bad as it has ever been, and we do not know what is happening with his gut because everything has slowed down with all of his sedation. It comes down to this - one cannot continue to live with GVH as severe as Max's. They described it as Grade Four GVH, which is as bad as it gets. The GVH itself makes him vulnerable to many different infections and complications. It may be playing a role in his lungs and brain already, something they cannot test because biopsy would be too invasive. The treatments for GVH are risky, because they suppress his immune system and make him vulnerable as well. But we can wait and let him die of GVH or we can try something. So, Max will be getting a second course of rabbit ATG tonight and for the next three to four days. God help us, it has got to work.

I told the BMT fellow tonight (Eric, who has taken such good care of Max), that we did not want Max taken off the vent unless his GVH was under control. His sedation and pain medication has to be turned down when he is off the vent so his respiratory drive is not suppressed, and we do not want him to be uncomfortable. He agreed, and in fact had already discussed it with ICU. It was certainly one of the hardest conversations I will ever have in my life.

What is so hard is knowing that Max is inside there somewhere trying to get out. If he wakes up a little though, he works against the oscillator and it starts alarming. Tonight I was talking to him and touching him and the alarms were going off. The nurse told me to leave him be for a while and she had to give him a paralytic. It stinks that when he knows I am there and he wants to respond it has to be taken away.

Mike's brother-in-law Geoff is here for a couple of days and he sat with Max so Grace, Mike and I could attend a little Halloween party at the hospital. Grace got her face painted as a cat and she looked really, really cute. Geoff is leaving in the morning, but Mike's mom arrived tonight and my sister Katie comes on Halloween. With all the help Mike and I should be able to attend the RMH Halloween party with Grace on Friday.

Grace visited Max tonight and sat at the edge of his crib. She was so excited when his hand moved ever so slightly and she said Max would be waking up soon. She said he thought it was night time and that is why he was asleep. Of course she does not understand it all. Today she said she was going to take a picture with Kendall on Halloween, she still does not get that Kendall will not be coming back.

Please send a nice guest book message this week to Max's Hurler friend and our RMH neighbor Taylor who will be turning two on Friday. She went through two transplants and they have been at the RMH for a year and a half. She is totally blossoming and brightens up everyone's day when they see her. Her family has been a great help to us as well.

Good Night,
Margaret

Day +73

WBC 10.9

First off, tomorrow (Monday) is Aaron Athy's 2nd birthday. He was leaving Minnesota at around the time we arrived back in July. We met him briefly and saw just how great he looked and we were shown just how good the process can go. It hasn't been the same road for us for sure, but he and his family (especially his Mom) have been so inspirational. They show that it can be done, that these kids do get a second chance and every step forward he makes is one we hope to follow. Congratulations Aaron!

Max is holding his own on the oscillator. He is still bloated and puffed up. His kidneys are working much better, but they are playing catch up now after a few days of gaining weight. His skin is still horribly rashed and even the clearer areas that weren't red and patchy have turned red. His arms and legs are all puffed up. The best word for it is . . . swollen. They were able to turn a few knobs down today and his x-rays have been looking good too.

Last night we had such a nice dinner together and we are grateful for that and for Auntie Bethie coming in so we could be together. At this time, even being 5 minutes away feels too far. It's hard to know how to feel. When the priest left yesterday, he said to Margaret "he looks like he needs to be relieved." And that's accurate. We're hoping that the medicine, machinery, and love that he's getting now offer that relief and that he can stay strong and come all the way back to us. But it's hard to look at him and feel he's getting better. He looks like someone who's being kept alive. And that's exactly what he is. But also, when I put his creams on today to try to help his skin, his arm came up, his little hand rose up, and there wasn't any shaking from seizures, it was just a little wave. I know that he was telling me that he hasn't quit, and I told him that I'm not going to either. As I tell the doctors, I got my medical degree from Dad University, but it still counts. I can't prescribe medicine, but there is one thing I can do for my son and that's not give up.

There was an old door on the floor of our unfinished basement growing up. One day I told my school friends about it and we all wondered if it led to a secret staircase or something. So we all sneaked down with flashlights and moved all the old boxes, tools, and chopped wood off of it, and then with all our might opened the door. And you know what was under it? Nothing. It was the same dirt floor and all the kids laughed at me and I felt stupid. But there was a moment, a split second, where we all believed that we had discovered something amazing. But it was nothing. Just once I hope that happens with Max. I hope that we have hit bottom. I hope that with every x-ray it's just a door, not another staircase down. I hope that one day it's just going to be fear and nothing else. I hope that one day when the doctors look inside, it's just a kid, just a regular kid.

Thanks for checking in on Max, for rooting for Max and for us, and for not giving up.
--mike

Day +72

WBC 11.8

Max has spent the last 24 hrs. bouncing away on the oscillator. Why bouncing? The machine works by delivering high frequency bursts of oxygen and nitrous oxide to Max's lungs. These bursts cause Max's whole body to bounce up and down rapidly with the machine. And the nitrous oxide acts as a "carrier" to help deliver the oxygen to the lungs more easily. It is actually a gentler way of oxygenating him than the regular vent, and I know he needs it, but it sure looks like torture to me.

On the positive side, Max finally began urinating last night with the help of drugs called Albumen and Bumex (and a pee dance done by Dr, Charnas, Max's neurologist and cheerleader). He has A LOT of catching up to do though, and he is extremely swollen with fluid and air. His abdomen looks uncomfortably huge, as does the rest of him. He has gained over 4 pounds in 2 days.

His skin looks terrible, and his whole body is red. One of the meds he is getting for his heart may be contributing to the redness. Yesterday afternoon his blood pressure was too low, but now it has gone up and they are giving him meds to lower it. What a seesaw!

His vent settings were lowered a tad bit today which is always good news. As Dr. Cornfield said yesterday, they have had kids much sicker than Max live, and have kids as sick as Max die. The trajectory he is on is not good, but there is still hope.

Special thanks go out to Auntie Bethie for flying in today to watch Grace. Other family members will be out the rest of the week to lend a hand.

We received an extra special delivery tonight from our friends, the Whites, from San Diego. Their daughter Susannah was transplanted twice in MN and went through a very difficult time. They sent us dinner, complete with dessert, wine, Halloween plates and napkins. It was delicious, especially the cake, which looked too pretty to cut, and was a much needed indulgence. Thank you Amy, Klane, Madeline, Susannah, and Gayle (who delivered the food)!

Please say extra prayers for Max's friend Sophie. She went into cardiac arrest and stopped breathing for a while. As of their last update, she is stable and is off the oscillator and onto a conventional vent. Please send them a guest book message of encouragement, as it makes a world of difference during times like these. Please also pray for Max's friend Cecelia in Chicago who was vented recently (non Hurler), and the families of two transplant patients who passed away this week. Alan was 13 and Jake was 21. They neighbored Max on both sides, and while we never met them we did meet their families. Alan's sister recently watched Sleeping Beauty and ate popcorn with Grace and another friend. His dad was so dedicated, his mom recently had a baby and we can only imagine how heartbroken they are. Jake married his fiance in his hospital room before he was placed on the vent. His mom Peg is so nice, and she remarked once that she loved to see Grace in the hall because she has so much life in her.

A few weeks ago we mentioned that there were 5 ICU rooms on 4A. Of those 5 Max is the only one still alive. We are so blessed to have each day with him, as we know the alternative is so much worse.

Thanks so much for your support,
Margaret

Day +71

REVISED AND UPDATED AT 11PM (LENGTHY)

>From earlier:
Max was just put on an oscillator because of new bleeding in his lungs. His saturations dropped very low during a suction and it took a very large team of people a fair amount of time to work him back up to a more stable situation. They bagged his lungs until the oscillator came in and he's now hooked up. The ICU doctor was suspicious of some heart trouble and an echo showed that his heart was a little empty in the left ventricle. This indicated that he lost blood from the bleeding. They have added blood products to replace this and have started a more rigorous schedule of administering some clotting medicine. Overall he is retaining fluid, and they are trying their best to get his kidneys to kick into high gear.

Perhaps most telling of the seriousness of everything, during the chaos the chaplain came and asked if we'd like a priest to administer Anointing of the Sick. It sure can't hurt. Please pray for Max, his doctors, and us as we are put through another tough trial. We will try to write more when we can.

Updated 11pm:
The Anointing of the Sick went well but was nearly overwhelming emotionally. It's awkward to have a priest you've never met and has never met your kid come in to say some prayers. It's hard to know what he was told before entering, but it was clear by how things went that he thought we were close to a place where we would be saying goodbye to our son. In addition to the prayers to get well, there was one that is said as Max gets to heaven. I came so close to grabbing his arm and stopping him because that's what my mind was telling me. "Wait just a minute, these doctors haven't stopped fighting" but my heart was breaking, and with all religious ceremonies, there is such rigid protocol and leave it to me to mess it up. But it was a nice prayer and there was a gap where he asked us to ask God for some of the key things about Max that Max would need to get better. Margaret and I couldn't sob any words outt, but Grannie was there and offered up the most beautiful words for Max. She asked for his courage and his strength first to help him fight on, and she spoke of how much Max has taught us about unconditional love. I can't thank you enough Grannie for speaking when we lost our voices, but for saying what was in our hearts. And for getting us paper towels to dry our eyes and blow our noses. We love you so much.

I know I had some reservations about asking the priest to come in, just because in some way it might show some resignation. But we haven't given up and neither, for sure, has Max. And I hope he knows that.

Also in the not giving up club are Max's doctors and nurses too and I want to mention a very special person in case she is checking up on Max. We have the best nurse in all of 4A in our room every time she works, Anmarie. Now I know that might upset some other nurses, but I asked Max and he said Anmarie. She became our primary before we ever knew how important it was to have one, and every shift she spends with Max she helps him so much. She also is cool under fire and believe me, in this room there is a lot of fire. She is such a good person, and has a good heart, and is very sensitive to our needs. At the key moment today when it looked like things were not going well, I asked her if I should get Margaret and she said yes, and that alone was done with such sincerity and compassion and I really appreciated it. She was asked to stay on for a double today and she agreed even though today must have felt like a triple! I'm sure she's exhausted. She may seem demur, but when she wants to kick a little butt on behalf of Max, she can do it. We hope to have you again on Wednesday!

Now back to Max. Since earlier, he has not gotten rid of any fluid. Are his kidneys failing? Tonight will tell us the answer for sure. His weight is up about 30% and he looks so bloated. If only some Oompa Loompas could take Max down to be juiced like Veruca Salt. Two peripheral IVs have been added because Max is on so many medicines. Another IV pole was brought out to help hold all the auto-syringes. His belly is also full of air because of all the emergency bagging they had to do to get him enough oxygen. His skin is beet red because of many factors, the newest being a streptococcis bug that was discovered with the last bronchoscopy. That may be the cause of the lung bleeds, but most likely it is the abuse his lungs have been taking (like the rest of him) from graft vs. host disease. We got some good news about his EEG, his seizure activity looks the best yet. He is on a new anti-seizure regimen now and it has pretty much put him into a deep deep sleep. That was good for his seizures, and good for all the messing around they had to do today.

Again, with so many systemic issues all going on at the same time, it seems like something major is popping up every day. Today in addition to the bleeding, we had a surprise with the heart looking a little weird. Now it's his kidneys that aren't working well enough. At some point, something, some one thing, needs to get better, or this trend is going to be very bad for Max. Have kids recovered from all this? Yes. And so can Max. But he needs some major good luck.

Max, we are so proud of you. You are courageous and brave. And you have our unconditional love for now and forever.

--Mike & Margaret

Day +70

MORNING UPDATE - BACK ON THE VENT

Max was ventilated for the 4th time this morning. His saturations took a sharp drop during his breathing treatment and suctioning at 8am. Dr. Milla was able to do a flexible bronchoscopy through his nose and looked into his trachea. He saw blood coming up from his lungs. He said Max needed to be intubated immediately. Within minutes, the team was assembled and it included Dr. Sweeney who had intubated Max before and has been a great help to him while under his care. The intubation went well and they are going to do another bronchoscopy down his ET tube to get a deeper look at what is going on.

Then we were in a waiting room and the entire BMT team, including the guest visitors from Canada and Iowa sat down with us. There was no doubt that a pulmonary hemorrhage is a serious, and potentially life-threatening problem. But as of right now it is the kind of thing they have seen kids recover from. Many kids do go through additional hemorrhages before things are stabilized however, and that is why they predict Max is going to be on the vent for a very long time. Weeks, they said.

It would be obvious to say we are disappointed that Max is on the vent again. He had been doing very well by Max standards and was making progress. In fact, last night his saturation was at the maximum, 100% with very little oxygen help. So, this setback does break our hearts. But this wasn't done for just breathing, it was done for bleeding, and that is more than a little frightening.

Max is a tough kid, a fighter, but how much can they ask of our son? There are just so many problems to try and solve simultaneously it's like nothing gets solved and he's susceptible to new problems. While ventilated, however, they just have so much more control over him and they are able to use many different meds. Maybe during this time they can make some progress with his seizures and his gvhd.

Please pray that the doctors can find some common ground on the path to take.

--Mike & Margaret

Day +69

WBC 13.0

It's been a busy day so I'll try to be informative and brief. Brevity is hard when your kid is sick, and you've been known to have trouble finding the brake on the keyboard.

Max has fevers even on top of all the antibiotics he's on. And his x-rays show some new "infiltrates" in the middle of his right lung. This could be an area of collapse. Or if in case these two suspects are working together, the best explanation is a fungal infection (very serious in the lungs). He's been put on an anti-fungal medicine called Abelcet that has some crazy side effects. The worst of these is severe "chills" usually manifested in shakes so that just makes the seizure monitoring that much more difficult. I stayed up tonight to watch for these reactions, and so far so good, no reaction.

On the seizure front, Dr. Charnas, Max's neurologist came by today and performed another EEG. While he wasn't having seizures actively, there were still some worrisome areas of activity and he put him on a new med. What's great about this med though is that it comes in "sprinkles" and gets spooned in with applesauce. Max is eating it up just fine. He will do another EEG to watch for seizures on Friday. He's been a lot more awake lately, and today we thought we saw his eyes cross a little and maybe move with a little more determination. He's still not engaging anything or tracking anything, though.

I took Max down for a CT scan of his lungs today and because the machine is so fast, we also got a CT of his abdomen and pelvis. The pulmonary team is going to study the lung CT and see how it will define his treatment. Dr. Milla still wants to put Max on CPAP, a way of forcing positive pressure into Max's airways without having to vent him. Most kids hate this, and Max was put on this a while ago in a last ditch attempt to keep him off the vent. Didn't work. But this is being done more electively and the hope is that the positive pressure can open Max's lungs up in the way that the vent does, but with out having to vent him. Get it? We're scared that this will aggravate him and cause more problems.

His gut and skin remain the same and the new immunosuppressant, MMF, won't show changes for at least a week. Today's abdomen and pelvis CTs should offer some more information for the docs in this fight. All CT results will come back by noon on Thursday.

Thanks for all the support of Max, and for all of the guestbook entries that my Dad's letter inspired. It was nice to read so many little notes in one day. We are so lucky for all of the wonderful support that we have. Please pray for Cecelia, Max's secret girlfriend who is much sicker than Max and has been fighting for her life for much longer. She really needs your support!

Have a good day,
Mike

Day +68

First, let me say thank you to Mighty Mike for staying two days in a row with Max. I wasn't feeling well and Grace is sick so I thought it best to stay away. Mike, you are such a great, great father!

Max had a pretty stressful night last night. He was quite wheezy. The pulminologist who was here for a few days made some changes to Max's meds the other day. Max had been getting very shaky after his breathing treatments so the pulminologist wanted to cut any meds that may have been contributing to the shakes. It is unclear if Max started wheezing because of the change in meds or if he was headed in that direction anyway.

Today Dr. Milla came on service for pulminology. We met him after Max was first diagnosed and he was one of the reasons we chose to have Max's transplant in MN. He feels that on top of "good old Max" who had a reactive airway to begin with, Max does have some trachea malacia (floppy airway). You may remember that an ENT scoped Max's airway a few weeks ago and said his airway was okay. Dr. Milla said that it is not anatomical, but it is there. I am still a bit confused, but I trust him and it fits with Max's overall picture. Dr. Milla also feels that Max's GVH may be in his lungs or his airway. He said BMT may not agree with him, but he said if Max is showing signs on his skin and in his gut, it makes sense that the angry cells would be in the airway too. He feels Max needs some extra pressure to keep his airway open so he wants to try him on a c-pap machine tomorrow. This is a bit scary to us, as Max was on c-pap before he was vented the first time. He did not tolerate it well. Dr. Milla feels it is worth another try.

As for his brain, Dr. Charnas (neurologist) ordered another EEG tomorrow to check for seizures. They still do not know what is causing them. His soft spot felt much fuller today, and Charnas said he has not ruled out hydrocephalus completely.

Dr. Grewal, Max's primary BMT doc, stopped by today. His main concern is the GVH. He said he has never seen such a bad case in a cord blood transplant before. He also said that Max has one of the worse cases of any transplant he has seen. This is not something we wanted to hear. They started Max on MMF yesterday. It is another drug to fight GVH. They have not taken him off his old one (CSA), instead he is getting them together. There is still a chance that the CSA is causing the seizures.

My mom came in last night to help out. Perfect timing since Grace and I were under the weather.

Please pray that Max's new cells find peace in his body and say some prayers for his doctors as well.

Thanks,
Margaret

Day +67

WBC 10.6

Sometimes it's hard to think of the right words for the update. It's difficult under the circumstances to try to be both informational and anecdotal. To be real and be encouraging. To be honest and be sympathetic. That's why it's so great when someone else can step up and find the words for us. This is a letter from my Dad, and it's the most encouraging thing we've read yet. We were so moved by this letter, and our spirits were brought up so much, that we thought it best to share it with all of our supporters. Thanks Dad. This really touched our hearts.
______________________________________________

Dear family and friends of Mighty-Max,

I would like to tell you about my recent visit to Minneapolis. After a day of flying from my home in New Hampshire, I arrived at the Ronald McDonald house around 5 p.m. on Saturday, October 11 to relieve Grandma Chick. Despite her assurances that I was about to enter a very special place, I must admit I had more than a few trepidations. Would I see pain, sorrow, pity, or any of the other emotions one might expect to see from people dealing with such serious challenges? From the minute I walked through the front door that evening to the minute I left for the airport this past Saturday, I saw nothing but hope, courage, compassion and above all love. I also came to know the meaning of Super Heroes.

Regular heroes are the historical figures, entertainers, athletes, and others whom we each choose to respect, admire, and sometimes almost worship. In Minneapolis, I had the privilege of meeting people whose exploits far exceed those of my “regular” heroes. These people are on a totally higher level – they are Super Heroes.

Super Heroes are the benefactors, staff, and volunteers (cooks, story-tellers, musicians, teachers, care partners, etc.) of the Minneapolis Ronald McDonald House. They maintain an environment that brings great comfort and solace to all who reside there. I cannot imagine staying in any other place.

Super Heroes are the doctors, nurses, and staff at Fairview Hospital, especially the team up on 4-A. Their dedication and concern for their special patients is so greatly appreciated. (I wonder how long it takes the nurses to commute from Heaven to Fairview each day. I assume they are provided with some special transportation.)

Super Heroes are the children and families who for unknown reasons have been given incredible burdens to bear. I have never seen such beautiful and courageous children and such loving immediate and extended families. Included in this group are Grandma Chick, Grannie Judy, and others who on various occasions dropped everything to quickly bring some needed help to Max and his family both in Chicago and in Minneapolis. Many of you reading this are Super Heroes for what you have done and what you planning to do in the form of fund-raising, comforting messages, and continuing prayers for Max’s recovery.

Super Heroes are my wonderful son Mike and loving wife Margaret who have made all of us so proud. The love they have for each other and for their children is obvious from their poignant updates on the condition of their son. They are handling their situation with the utmost dignity and courage. They are an inspiration to all of us.

A Super Hero is my beautiful granddaughter Grace. She is the smartest, cutest, and most independent three and a half year old in the world. She loves her brother so much and like the other siblings I met, she is forced to sometimes deal with situations that even adults find difficult to comprehend. She is just as strong and brave as the other Super Heroes.

I have saved my greatest Super Hero for last. He is of course my grandson Max (a.k.a. Bubba). He is more important than any historical figure, more handsome than any movie star, tougher than any sports hero, and braver than any Navy Seal. Max is the greatest hero I have ever known and will ever have in my lifetime. I feel so blessed to be his grandfather.

I thank all the Super Heroes for letting me spend a little time in their extraordinary world. They have given me hope, courage, and an understanding of what true heroism is all about.

Love to all,

Grandpa Chick

Day +66

WBC 11.6

The weekend in review:
Our big worry is still the brain, and he's still not having continuous seizures, and that's the most important thing. While there is an occasional episode of shakiness, they don't seem to last long and seem to be triggered by some agitation and therefore may not be seizures per se. Hopefully these doses hold out and he won't need to step up to the next level of anti-seizure meds since they would suppress his breathing to the point of needing a ventilator again. Elsewhere, Max's skin rash has grown significantly worse in the last 3 days or so, while his diapers have remained the same. Another medicine for graft vs. host disease, called MMR, may be added soon to help. His lungs are holding up well, and he has not needed much help with the cannula. His x-rays are still not perfect and he still sounds "junky" but by Max standards, his lungs are really stable, and it's nice to talk about them last for once.

Goodbye to Grampa Chick, thanks for staying through this difficult time of seizures. And thanks for being a great car for Grace's gas pump game. Hello & Goodbye to Margaret's Auntie Eileen and her cousin Jenny. Thank you so much for coming up and playing with Grace. She really laughed a lot and had such a great time with you guys! "I can't pay the rent . . .You must pay the rent." Once again, someone from Margaret's childhood remarked how much Grace is just like her mother was at her age. What a joy to be given a glimpse into my wife's childhood - so full of life, creativity, and . . . manipulation!

Overall he's still so unbelievably tired. The anti-seizure meds do have a calming effect, of course, but they in and of themselves would not be slowing Max down this much. And he's on very little other medication. The best explanation is that Max is still wiped out from continuous seizures. Even one seizure can wipe a person out, so who knows when he will catch up. Rest is surely good, but we're going on almost 8 weeks since our boy has really opened his eyes. On the other hand, if he saw his skin, he might not like what he sees - raised red splotches all over his belly and back. It's disappointing to have his skin become such a problem because that had responded to gvh treatment long ago and had been doing well. They did take the EEG wires off his head today though, and it's nice to see his big bald bean again.

Halloween is coming soon and I'll be taking Grace to a local dry cleaner to haver her "Belle" costume hemmed and altered to fit. Fall in Minnesota is quite pretty, but we've been told that the campus here had some botanical engineers make sure that all the trees turn yellow and red because those are the school colors. So we've been kicking up lots of red and yellow leaves on our walks between the house and the hospital. Out the window we can see all the college kids going to and from a couple of big dorms. I'm sure I've passed some of the same kids any number of times. They're like every other anonymous college kid on every other campus, and like I was in Evanston not too long ago. But there the colors were purple and white and the botanical engineers were always at a loss, spending all spring and summer cross-pollinating and replanting only to bury their heads into the cold hard Autumn dirt and cry. As for the leaf controllers up here: Where I come from, that's called cheating.

Fighting Crime Before Bedtime,
--mike

Day +65

WBC 18.2

Good morning. Please check out our new Acknowledgments area in the "How Can I Help?" section. Not only can you learn about upcoming events for Max, you can look back on past events, and you may even spot your name in a Thank You from us. We've tried to remember everyone and everything, but thanks to all of you, that is so hard to do! So many of you are helping us in so many ways that with all that is going on, please forgive us for forgetting anything.The computer went down late last night so no update. But two really good things happened yesterday:

First, the morning EEG showed Max's seizures had stopped. The dosages for the two-anti-seizure meds finally reached therapeutic levels and did their job. They want to leave Max like this for the weekend, and re-check on Monday. Who knows what happens after that. Since they don't know what is causing the seizures, it's going to be hard to know when things can be turned down. They told us he will probably be on these medications as long as he's on his major med (like CSA), which may be as long as a year. The effect of the seizures long-term is, of course, unknown at this time. His MRI did not show acute areas of damage or bleeding, so the hope is that this is more consistent with epileptic behavior - these seizures are a frightening, but controllable, nuisance. They can come back at any time if his levels drop, so this is going to be a long process. This morning, in fact, his hands are tremoring again, so his levels may be down.

Second, our wonderful care partners, Marsha & Pete came by to play with Grace and it turned out the Ronald McDonald House was having a field trip to the movies for "Good Boy!" the new talking dogs from outer space movie. Marsha jumped in the van with about 8 other kids. Grampa Chick watched Max at the hospital and Margaret and I had our first date in about a month. Marsha had picked up lunch for us, which was great, and then we promptly crashed into the snuggliest, deepest nap. That's what happens when you're about 75 days behind in sleep. Grace had a great time with her friends, and it was her first field trip. All the kids had pop and popcorn and Grace said her favorite part was "when the dogs were talking." Hmmmm. Maybe it was a little over her head. Oh well, it's no Finding Nemo.

In other Max news, he's had a fever for a couple of days now and his white cell count has been rising so he may be fighting an infection. He's on all kinds of antibiotics so hopefully this will break. They don't think it's in his lungs or in his brain, so that's good.

Please continue to check out all of Max's Hurler buddies' pages, especially Sophie who's fighting GVHD and Yasmine who's fighting CMV. Also make sure you congratulate Anthony (Tony the Tigre) who now has his new cells. We've been told many times that these Hurler kids can be super-resilient, and their strength can be remarkable. Max has certainly proved that.

Have a great weekend,
--mike

Day +63

Happy Birthday Great Grandma Ciacciarelli. Number 85!

Today was a fairly quiet day. It was a pretty stressful night for Mike. Max's blood gases were being watched very carefully, and his carbon dioxide was creeping up a bit. He was working pretty hard to breathe, and the vent was parked outside Max's door. He fought it off, thank goodness. And his gases have been pretty good all day.

Max had another EEG this morning, and the seizures are still breaking through. The blood level of one of the anti-seizure meds was still lower then they wanted today so they are working to bring that up. They turned off his Ativan today in hopes of waking him. Mike was very worried this morning because Max was not responsive. He didn't even react one bit when his lungs were suctioned. After 5-6 hours without Ativan Max roused enough energy to squeeze my fingers a few times this afternoon. He also scratched his nose and managed to get his nasal cannula out of his nostrils. In addition to all of the medication making him sleepy, even a "small" seizure can make one very tired, and considering Max has been seizing for days it is no surprise he is so out of it.

The mystery of the day involves Max's blood sugar. It has been trending upwards for no apparent reason (in fact, it is surprising since his steroids have been lowered) so tonight he went on an insulin drip. His rash is looking worse and we don't know how his gut GVH is doing since the sedation has slowed everything down and he hasn't pooped since Tuesday.

Some of you may have already heard about a fund raiser being held for Max. It is a raffle which will be held on November 9th. We knew this was being planned, but had no idea how big it had become. So many awesome prizes have been donated including restaurant gift certificates, gift baskets, and even a 1 week stay in a condo in Hawaii! Many, many thanks to all involved especially to our neighbor Jodi who has led the effort. For more information please contact her at jodiheiss@hotmail.com.

We mentioned a while ago that Park Ridge Newcomers Club chose Max as the recipient of their annual Labor of Love Fund Raiser. It will be held on November 13th at the South Park Recreation Center in Park Ridge. Please contact my sister Beth at micucci4@comcast.net for more info. Thanks again to Mary Beth Micucci for nominating Max.

Please send some encouraging guest book entries to Max's Hurler friends Sophie and Anthony. Anthony was transplanted today and Sophie is not feeling well.

-Margaret

Day +62

WBC 11.3

The brain, the brain, the scary brain. The plan was to give Max as much anti-seizure meds as they can give him without compromising his breathing. Tonight they hooked up his head to the EEG and it showed major improvement but intermittent seizure activity. They have not stopped yet. There is still one med that has a level that needs to come up and the neurologist feels this might get him over the hump. The nurses and resident here tonight, on the other hand, feel that that one more bump might put him back on the vent. The idea of a fourth vent is sickening to our stomachs, but they've got to get the seizures under control. We've been told that the brain comes first, lungs second. Putting Max on the vent for the fourth time is nobody's goal, but a reality because if tonight's dosages don't do the trick, tomorrow they will need to start other meds that will definitely require the ventilator.

How sedate is he at this point? He's pretty much out cold. He's breathing only about 15 times per minute, and he doesn't even come close to coughing, even when they suction him. He's got no interest in swallowing, and the bottles are out, and a tube may need to go in for oral meds. He's not even stooling, so who knows how his gvhd is doing. His skin is still rashy though, but really, that's the least of our problems.

What's causing the seizures? No one knows.

It's been a hard couple of days. In addition to learning that Max is having seizures, we've had to say goodbye to Kendall and her family. As most readers of this site know, Kendall is 5 and is Grace's best Ronald McDonald House friend and we met them when we first got to the House. Kendall had AML, and was transplanted about a week before Max. Every time Grace visited her brother, she checked on Kendall. Both Kendall and Grace looked forward to the day Kendall could come home from the hospital. She received the news she was 100% engrafted about a month ago, and returned to the house a couple of weeks ago feeling like a champion, and her parents were beaming with pride. She and Grace were able to play a few times in each other's rooms and shared toys and watched movies together. They were the featured friends for the house holiday card. Last week they found out that she is no longer engrafted, and her leukemia is back. This was unexpected, and thoroughly devastating. It is back so aggressively, in fact, that the family was told that no one in her situation had survived to make it to another transplant. Her family made the very difficult decision to bring Kendall back to Florida to be with some family and friends. Her mom told us that she wanted Kendall to see the beach again, and not lose her fight out here in the gray of Minnesota winter.

We have so much love and respect for tough-as-nails Kendall, her brave parents Marnie & Dale, and their wonderfully compassionate son Ty, that we are beside ourselves with grief for this outcome. Yesterday Kendall got enough blood and platelets to make it home, and hospice will care for her after that. They do not have a website, and have tried to keep their situation as close-knit as possible. We've known the news for some time, but until they left, there was some solace in being able to see them. Kendall gave Grace most of her old toys, and Grace has been playing with them. They don't know how much longer Kendall will live, but it will not be long. Grace knows Kendall will not ever be coming back to Minnesota and she is very sad. Today she also asked Margaret if Max was going to die. I think she got scared seeing so many wires coming out of the top of his head. She knows that you go to heaven when you die, and she know that you're not sick when you get there. I told her that, and I hope it is true. Kendall is an amazing girl, and was the perfect match for Grace's spunk and attitude. Margaret and I would both consider ourselves lucky to have had Kendall for a daughter, and in about a year and half, we will have a close facsimile for sure.

Kendall, we love you so much. You are so brave, and Grace misses you already. Marnie & Dale, somehow, in the middle of all this, you came into our lives in such a deep way. We love you for wanting to make every day you have left with Kendall special and beautiful. You understood our fears like no one else could, and we hope we can understand your fears and loss and provide some compassion in your time of need.

That's it for tonight, I'm sending this with the hope Max will remain off the vent and that his seizures will stop. He's such a tough kid.

Did I mention the Cubs?

--mike

Day +61

WBC 12.5

What a hard, hard day. It began with an EEG. They stuck a bunch of wires to Max's head with strong glue. The wires led to a computer which measured brain activity. The EEG showed that Max has been having continuous seizures. They took him down to MRI right away and ordered another anti-seizure med in addition to the one that was started last night. After his MRI they hooked him up to a continuous EEG which he will stay on throughout the night. The med worked well, but did not totally stop the seizures so he is now getting an Ativan drip which will hopefully do the trick. If it does not work, they will have to try more powerful medication, which will lead to him being intubated again. As the neurologist just said to us, they are trying desperately to prevent this from happening.

The MRI showed increased ventricles (as compared to his MRI in June) and less space for fluid. These are both signs that Max's hydrocephalus has become worse. What is very odd though, is that his head size has remained constant and his soft spot is still the same as well. Initially the neurologist thought Max may need a shunt, but the neurosurgeon came to talk to us and he was not convinced that it is his hydrocephalus. He ordered a lumbar puncture (spinal tap), which was done late this afternoon. The tap showed that his fluid pressure was good, in fact better than it was in July.

They sent cultures of the spinal fluid because another cause of all this may be meningitis. The chances of this are low, as Max has not had any fevers or shown any other signs of infection. The BMT docs have not totally ruled out CSA and steroids as the cause, however, the locality of the seizures in the brain are not consistent with a CSA reaction.

The results of Max's skin biopsy showed grade 2 GVHD. The BMT docs do not believe this result and are having the test run again.

Where does this leave us? We don't know, and neither do the doctors. We are very afraid that the seizures have been going on for a while, as we pointed out to them between intubations 2 and 3 that Max could not focus on anything and he seemed totally out of it. We are frustrated that an MRI was not done during his last intubation as we requested, because maybe this could have been detected earlier (if it had been taking place then). If Max has to be reintubated to fight the seizures we don't know what we are going to do. We have asked so much of him and we keep asking for more. His GVH is not under control, his lungs are unstable, and his brain is going haywire.

Despite all of this, we are still in a better situation than other families. A beautiful 13 year old girl named Jenny passed away last night. She was transplanted a little before Max for Fanconi Anemia. I saw her mother today and she is so devastated. You can visit her web site at www.jennyshields.com. They do not have a guest book.

Grandpa Chick has made arrangements to stay with us until Saturday so we can both be here.

-Margaret

Day +60

WBC 7.6

This will be a long one, so be patient . . .One day in the hospital can be so BUSY! . . .

First the lung news: Max is breathing well. He still sounds a little "junky" and he still has a wheeze, but he sounds MUCH better than a couple of days ago. His effort to breathe is down as well, and that's nice to see. Margaret found a website of another kid who had Hurler in Canada. He passed away sadly after some breathing difficulty long after he seemed to be out of the woods. He was on the vent for a few days and passed away. While every kid is different, it is some achievement that Max has been able to make it off the vent three times. Keep it up Max! We don't want him to back on. Roger, one of Max's favorite respiratory therapists, asked if he should take the vent out of the room, since he thought it might do well to leave Max a "warning" but I told him I like the symbolism of it leaving the room. When he rolled it out, I shouted after him, "And don't bring it back." I hope it is so. Last night I found an eyelash on Grace's cheek and let her blow it away with a wish, and she said "I wish for my brother to get all better."

Next the gut news: No news, really, but they have decided to do another flex-scope and look up his butt again. He's made some progress, but he's far from looking better. This new look should be in the next day or two.

Swallowing news: Today Max had a swallowing test. While he has been drinking well, the docs wanted to be extra sure that Max wasn't aspirating (food/ liquid going down into lungs) when he swallowed. We took him down to radiology and he drank a liquid that had been mixed with a barium powder. The drink is white, but as they watch him under the x-ray, it's black. He swallowed about 30 times and only one time did anything enter the "vestibule" but he cleared it. In other words, NO ASPIRATION. Then, for the heck of it really, they gave him some vanilla pudding mixed with the same stuff and he swallowed that down too. So, his swallowing is going well and he may be able to move towards more solid foods sooner than we thought. But it will depend on the health of his gut.

Skin news: Max had a biopsy done on some of the rashy skin at the bottom of his belly. The dermatologists feel it could be skin graft vs. host disease, but it didn't start or display itself like it did in the past so the transplant docs disagree. We'll learn more in the next day or two.

Finally, the most intriguing and worrisome news, brain news: Max may be suffering from PRES (Posterior Reversible Encephalopathy Syndrome). I'll try my best to explain it. This is the malfunction of the lining of the brain near the back of the head. This area affects vision and can cause seizures. The key letter in PRES is the R because it is Reversible. This most likely is from CSA (his cyclosporin, the drug used to help reduce GVHD) toxicity and high blood pressure. They've been doing a good job of keeping Max's blood pressure down, but his CSA level is hard to monitor because he's been so sedated and drugged up for so many reasons. Now that he's off so many drugs, it appears like he is suffering withdrawal or is rather delusional. His eyes are not focusing on anything and he's not responding to light. He's not using his left side of his body much, and he's moving his right hand randomly. We've been saying every time he gets off the vent that he's "out of it" and this is the best explanation so far. So tonight he's starting an anti-seizure medicine which may take the edge off of what is happening and may stabilize him a bit. He also started monoxidil for his blood pressure. You might recognize that drug as Rogaine. It turns out that the discovery of a new drug for blood pressure helped grow hair - go figure. Kind of funny that Max is starting this when he's still really bald. Tomorrow, they are going to give him an EEG to measure brain function, and he may get an MRI as well. They may have to play with his CSA level, to try and find a dose that is less toxic to him. Since this drug helps fight graft vs. host disease, Max's gut needs it. But it just might be too much for his brain.

This is all so scary. And typical of Max. Now that his breathing and stooling have made slight progress, we learn of something new. This, like most things, is going to be a long journey to solve so the hard thing again is to be patient. Let's hope that their first guess is the right guess, and it has the least long-term consequences.

LAST BUT NOT LEAST:
Thank you Genzyme for your wonderful dinner and for being so thoughtful in our time of need. These people are the makers of the amazing Enzyme Replacement Therapy treatment Aldurazyme that his helping a number of MPS1 patients who have a less severe form of Max's disease. Thank you so much, your dinner fed us and another family on a perfect night where the Ronald McDonald House did not have volunteer cooks.
Thank you Katie and Gretchen from Optimus! Your costume for Grace is perfect and just what she wanted! We are grateful.
Thank you Uncle Marko! Your handmade book for us will be treasured for years to come. Your artwork once again has brought smiles and not a moment too soon.

Thank you - to all the people we keep forgetting to thank! When our heads stop spinning we will try to remember more people. We have such generous friends and family, and we are so blessed to have you all.

Please pray for Max and for all the other Hurler champions and hope that they can bring Max's personality and joy of life back to us! We miss our dude!

--mike

Day +59

WBC 6.6


Max had a pretty good day today. He has been enjoying his bottles of Pedialyte. Dr. Sweeney said he can have 1 oz every hour, but Max wishes he could have more. He still sounds VERY junky, and is so stubborn when they try to make him cough. If only he would cough he would be so much better. Dr. Sweeney wants him weaned off his narcotics, as they are also cough suppressants, and Max is handling it quite well. He has not needed any bumps on his pain meds or extra meds to help him sleep. The bottle is the best thing for his mood it seems! They just have to watch his ins and outs very carefully so that he doesn't get flooded with fluid and end up in respiratory distress that way.

Max has been very shaky today and he still seems very spaced out so Dr. Charnas, Max's neurologist, will be paying him a visit tomorrow. Dermatology will also be stopping by to check out the yeasty rash that is spreading. His stools continue to be bloody and we are very doubtful that the steroids did the trick, although the docs are willing to wait a bit before doing anything.

Grace spent some quality time with Grandpa Chick today and we are hoping to make it to the Children's Museum of St. Paul tomorrow. Grandma Chick left but will be back in November. She is hoping to see Ma up and around at that time .

That's all for now. Keep those prayers coming for Max and all of our friends at the RMH.

-Margaret

Day +58

WBC 4.9

Max has CSA eyebrows! With all of his problems, it's kind of nice to see the growth of monkey hair that we've been expecting for some time now. His head is feeling fuzzier and his chin has a little soul patch. He is adorable.

Grandma Chick is leaving early tomorrow (Sunday) and who would've thought that a nine-day trip would have included an intubation and an extubation? Oh well, at least it was eventful. Thank you Grandma Chick for all your help and welcome Grandpa Chick. Your separate trips have the nurses talking, but we know all is well.

Max's first full day off the vent (again) was fairly stressful. Of course. But multi-medical-degreed Dr. Sweeney came by and came up with an unusual plan - irrigation and suction. He feels that if the problems in Max's lungs are not asthma or upper airway, the next best guess is to call it a "secretion problem." His best guess is that when on the vent, there is better access to sucking out Max's secretions. But when off, with less access they get worse. So he wants Max "less dry" because it only makes his lung secretions thicker and tougher to cough up. This is difficult because they are giving him diuretics now to keep his fluid retention down from steroids. So he changed his breathing treatments and he did turn down Max' s narcotic. As he explained, even over-the-counter cough suppressants are narcotics. Even though he's surely uncomfortable and cramping, he's tolerating less pain medication well because it's being balanced with lots of . . . bottles! That's right, the best prescription ever! Max has been cleared to drink and drink often (water or pedialyte), to make up for loss of water from breathing and from not being on the vent. So far he is sucking down bottles and it's going a long way to making him more comfortable.

Late tonight, they brought in a new crib. It is a super-spiffy, and we've heard super-expensive, bed that has percussions (it actually bounces), and can rock back and forth and side to side (he's velcroed into some padded restraints). The idea here is to keep Max moving, vibrating, etc. They want things loosening up and hopefully the bed can help. But it is LOUD. Imagine an outboard motor or pneumatic drill going off under your mattress, and that's what it's like for Max.

The doctors are trying so hard to figure this out, and this new plan is welcomed by us because at least it is something new, and the treatments are very benign. When the doctors here feel confusion and frustration, it's so difficult. We feel we are here because this place has the most experience with Hurler Syndrome and transplant, but even so we've learned that all of these kids have individual issues, some startlingly unique. Sometimes it feels like we are beating our head against a wall that has only new walls being built behind it. But we remember that everyone has Max's best interest at heart and that if there was an easy solution, it would have been tried. It definitely keeps us going to see them thinking, and trying, and not giving up.

Thanks again for all your support,

--mike

Day +57

WBC 3.8

Max was extubated for the 3rd time at about 2:45 this afternoon. Soon afterward the ENT came and looked at his upper airway via a camera that was threaded down his nose. His upper airway looks good. This leaves us wondering what is really going on with him. It is possible that he had an upper airway obstruction before that is now gone. He is a little wheezy in the right upper lobe. We are kind of shocked that no one from pulmonary has been by today AT ALL - not before or after extubation. Max's pulmonary treatment plan was just written up by Dr. Sweeney of the PICU (who we absolutely love), but we thought pulmonary was going to be more involved considering Max's history. We do not know how his chest x-ray was this morning or if the bronch worked to clear that right upper lobe.

Right before Max was extubated he had a huge diaper that was much bloodier than they had been all week. This was a huge disappointment, as you can imagine. We have to talk to BMT about this. The yeasty rash that was on his diaper area and neck seems to have spread.

We were sad to learn that another 4A patient lost his life. We met Dan's mother at the Days Inn back in June when we first came to MN. He had leukodystrophy, and was 27 years old. He was misdiagnosed for years, and he and his family suffered greatly. I knew he was having a lot of trouble the past few days, but was shocked to see his room was clear this morning when I came in. I only wish I had a chance to talk to his mother one more time. There are also some kids at RMH who really, really need prayers right now.

I will send another update tonight if anything major happens - let's hope you don't hear from me until tomorrow!

-Margaret

Day +55

WBC 4.9

Max had a good night and good day so far. His oxygen is now down to 40% and this afternoon they lowered his rate on the vent from 22 to 20 (breaths per minute) . As I write this, the doc is about to lower his setting to 18. He is not breathing over the vent when he is asleep. When he rouses a bit for diaper changes or vitals he takes some spontaneous breaths. He is going to have a bronchoscopy sometime tomorrow. They will try to open up the right upper lobe of his lung that has been collapsed for quite a while. They will also check cultures.

Dr. Sweeney, the ICU attending and anesthesiologist, has been brainstorming to try and figure out what is going on with Max. He is going to suggest a study of Max's upper GI to see if reflux is causing the breathing problems. When he is nearing extubation ENT will talk to us about scoping him in the OR.

They have been weaning him off of his Terbutaline (IV bronchodialator) all day and just shut it off. Now maybe they can remove the IV he has in his scalp. We should know shortly after the results of his next blood gas.

He has had a strange rash in his diaper area, unlike one we have ever seen. It also appeared on the back of his neck. The docs feel it is yeast and are treating it with Nystatin cream. Dr. Charnas, Max's neurologist, came by today and said Max is looking good from a neurological perspective. He would normally do an MRI when Max is 90 days out of transplant, but may take advantage of Max's current intubation and do an MRI now. The BMT docs will be discussing this with him.

That's it for now. Keep praying for progress for Max and all of our friends here in MN.

-Margaret

Day +54

WBC 6.0

As someone pointed out to me today, you just can't play a sad song on a banjo. When the blues man wants to lay down a song of heartbreak and loneliness, he doesn't pick up a banjo. So to the gray-haired, ponytailed music man who comes to the Ronald McDonald House every Tuesday afternoon, thank you. And when my daughter sings along with you, just know that you are playing the happiest instrument in the world.

We are once again adjusting to life on the vent. We have lots of questions about what will happen if (when) he is extubated, but Max still has to make progress working with the machine. Today he did just that. He began to breathe easier, working well with the machine, and was able to breathe with less oxygen support. Not much else to report, time moves glacier-slow when on the vent. Progress is measured in tiny mechanical increments, and we have a long way to go. Diaper-wise, there's still blood in his stool, but it is more black-green, and more sludgy and that's a step in the right direction.

Gracie, Grandma Chick, and Margaret all went to the Como Zoo in St. Paul. It's a free zoo along the lines of Lincoln Park Zoo in Chicago and Grace had a good time looking at the polar bear. It was good for Margaret and Grace to get out, and nice of Grandma to drive. Then there was the banjo-playing music guy "shave and a haircut, two bits!" and an art project tonight at the Ronald McDonald House. Once again we must say that the Ronald McDonald House of the Twin Cities is an amazing place and everyone reading this should know that these houses are for families of critically ill kids and they need all the support you can offer. Please check out your local RMH and see what you can donate or what you can do to help. Max's Auntie Dana already cooked a meal for the RMH near Loyola (where Max was born) and we hope to be able to return the generosity we've received when we get home.

Thanks also for all of the cards, emails, and packages we've received lately. Our mailboxes have been bursting with such wonderful surprises from so many people and it's helped to put some of the overwhelming frustration and disappointment we've been feeling at bay.

--mike

Day +53

WBC 8.3


I wish we had more answers for everyone today but we do not. Mighty Max is such a Mystery Max that no one is exactly sure what is going on with his breathing situation. He has been wheezing so badly the past few days despite all of the medication they gave him, but he stopped wheezing once he was vented. It was pretty amazing. This behavior may be indicative of an upper airway problem, so ENT will be consulted. I thought ENT was contacted yesterday but they were not. They should have been contacted today but I haven't heard anything yet. The hard thing is that the ENT cannot really evaluate his airway with the breathing tube in it so most likely it will not happen until Max is ready to be extubated. If someone had thought of upper airway obstruction yesterday maybe this could have been done before he was intubated. Now we have to wait until he is ready to get off the vent, check him, and then who knows what.

Last night he got his albuterol, BD's and suctioning and sounded clear afterwards. Then the RT gave him his Pulmicort neb and Max started wheezing pretty bad. They had to give him more albuterol and increase his O2 significantly because his sats dropped. He had been weaned down to 55% O2 prior to this incident but has needed quite a bit of O2 through the night and today. I wish Pulmicort was the easy answer to all of this but he has been on it since last November or December with no problems. It may have been an issue of particle size when blown into the vent with the neb. The last 2 times he was vented he received Flovent puffs not a Pulmicort neb.

The Pulminologist has been throwing around the term Chronic Lung Disease of Prematurity the past few days. Max was never diagnosed with this in the NICU, so it is new to us. The BMT docs told me today that it is on the same spectrum as reactive airway disease (asthma). There was talk of doing a bronchoscopy today too but that did not happen.

Max's sats and blood gases have actually been worse since he was vented. They are not too worried about the gases, for reasons still not totally clear to me so I will not try to explain them here. As for the sats, who knows. As you might imagine, Mike and I are pretty frustrated. We do not want to end up in a horrible pattern of vent, wheeze, vent again. We really want some answers, or at least a better formulated plan for dealing with this. The nurses have been juggling his sedation today as he has been waking up quite a bit. This wouldn't be so bad if he was breathing more in sync with the vent when he took spontaneous breaths.

In other news, Max is retaining some fluid today and his weight is up and he is puffy. The lasix is not working as well as it usually does with Max so they gave him a medicine called Albumen to help draw fluid out of the of tissue and into the blood stream where the kidneys can see it and get rid of it. He has not stooled since yesterday morning, which is great but not necessarily a sign that the GVH is better. Being on a vent slows things down too (it did the last time) so we are not sure what is going on. His steroids will be reduced tomorrow and the GI doc will be consulted to see if she would like to scope him again.

This turned out to be a much longer update than I intended so I apologize. We are so depressed and frustrated right now. I feel sick when I look at him like this again. He was just starting to come back to us and now we are right where we were one month ago.

My mom went home on Saturday when Mike's mom arrived. Mike's dad arrives this weekend. RMH house has asked if Grace could be in a RMH video and we said yes. She is a regular RMH star these days. Tony the Tigre arrived on 4A today so wish him luck as he starts chemo tomorrow.

Thanks for your prayers,
Margaret

Day +52

Mid-morning update

It is with sad hearts that we report Max was put back on the ventilator for the third time at about 11:15 today. This is what nobody wanted. Please continue to root for Max. Hopefully they will learn more about his lungs and his airway during this time. Special thanks to all the people at the hospital who worked so hard (especially Max himself!) to keep this from happening for so long.

--mike & margaret

WBC 6.7

Saturday was another very tough day off the vent. And last night was even tougher. This morning they told me that Max needed to go back on the vent. It's 8am now and it hasn't happened yet, but here's the story so far:

Max had been struggling. He'd been working very hard, at least as hard at times as when he was put on the vent before. But, his blood gases still looked good and his respiration rate was not high for long. He sounded terrible though. He was wheezing nonstop, and noisy. You didn't need a stethoscope to hear the effort. His expirations were much longer than his inspirations, indicating wheezing as well. Overnight, however, Max's blood gases weren't as good. He was retaining too much carbon dioxide, and was not exchanging oxygen as well. The ICU attending came in to help care for another patient in distress and felt that it was again time for Max to be intubated.

We're thankful that they've been giving Max a chance to beat this. On Friday, pulminology told us that the need for intubation was imminent. Then ICU told us that we could wait a bit and let the meds work. Then they came back at night and the ICU doc sort of shrugged and said it was time. But he has made it another day and two more nights so that is pretty good. He's on all the medication he can get for this wheezing and the high-dose steroids should have helped. But most people (nurses, RTs, docs) feel like there's not much more they can do and he's really not getting better, just holding his own. Either he'll improve, they say, or he'll tire out and be put on the vent. They feel that if nothing else, he'll be more "comfortable" (a word that's sounding less and less encouraging). So the threat of the vent is real and is happening on an hourly basis or so. It is something that everybody seemingly wants to do and wants to avoid.

If Max does get put back on the vent, we will feel deeply saddened by this situation. We have been told that going back on a vent a third time would be a "major setback" and there's no way to shake those words out of our heads. Since they don't have an answer as to why Max is wheezing and having such a hard time breathing on his own, it's hard to feel confident that even if he is able to get off the vent again, he'll be able to stay off. But that's what we have to hope. Still, our hearts will be broken again.

Grannie left yesterday and Grandma Chick arrived. So Grace went from one Grannie to the next for a buddy to play with. We don't know what we would have done during this latest stress period without this help. So once again, thanks to our Moms for being so great.

Breathe Max Breathe!

Mike

Day +50

WBC 6.4

The care conference was overall not as bad as we feared. Max's situations are critical, our fears are real, but he is still in the game. We had a nurse coordinator, the nurse administrator, a respiratory therapist, a pulminologist, our current BMT attending, the current BMT fellow, our original primary BMT doc, and our neurologist there. Everyone wants Max to get better. And they recognized some of our frustrations and they tried to hear us out. They have changed our nursing situation back to one-to-one in anticipation of intubating Max again.

They all realize that he is still fighting his two battles simultaneously and the plan for his gut GVHD is the increase in steroids. The plan for his lungs is much more difficult to predict. The biggest news is that the pulminologist feels that Max isn't just acting like a bad asthmatic, he's also displaying symptoms consistent with chronic lung disease from prematurity. He wasn't on oxygen or vented very long as a preemie, but when combined with Hurler, he probably has chronic lung disease in some form. He told us that the best treatment for this is growth, but that's down the road as he gets older. Of course, he's got to get through his current trauma to get there, but it was still sort of nice to hear a doctor talk about the future. Every time someone gets vented, they become more of a "high risk" patient, and if it happens for Max it'll be the third time. Nobody feels this is a good thing long term, but if it has to happen it may give him some rest and comfort and hopefully his lungs can open up. His wheezing is getting really bad, and going on a vent is not really a treatment for wheezing, it's kind of a last resort to offer some rest.

I guess the best case scenario at this point is for Max to respond to the new IV bronchodilator. It would be great if that opened up his lungs and we could come off the constant Albuterol mask. But it seems clear that no one is too hopeful for that to happen. So if vented, we have to hope that his lungs can heal again and that his guts will respond to the new steroid doses. Maybe if his intestines can stop bleeding and get better, he can be treated for the breathing slightly different (if he comes off the vent).

Neurology feels Max is medically delirious. He is there, but not there. He is feeling the effects of the meds that sedated him on the vent. Now that he's likely going on the vent again, who knows how much farther that will set him back. He said he's not worried much about Max because these are just meds that take a really long time to wear off.

What can we say? We were told that at day 50, for someone who had been through sepsis, has VRE, skin/gut/liver GVHD, has been intubated twice, etc. Max is doing well. But that was a lot of qualification before "doing well". Still, the alternatives are worse. We are glad Max is here with us even if neurology tells us that he's not mentally here with us. He's fighting so hard to breathe and there's just no quit in our boy. Many many of Max's caretakers have told us that most adults would have given up by now and just been vented. But Max is a trooper.
__________________________________

Midday Update

The docs are trying their last resort before venting Max again. He is working harder and harder to breathe and his blood gases are showing it. They will be giving him an IV broncho-dialator now and if that doesn't help they will intubate him. We have our meeting with the docs in 1 hour and it is looking like it will be a lot of bad news.

He just got a new IV in his head for the new med.

-Margaret

Day +49

WBC 4.4

We didn't have our care conference today (it will be tomorrow) so it's hard to give an overall picture of Max's health just yet. Suffice to say, we're well past the time of discharge for a "best-case-scenario" but our champ is still holding his own. And he's still facing the same two main issues: gastrointestinal GVHD and unstable breathing/lungs.

On the gut front, he had another flex sigmoid ("butt scope") done today and more biopsies were taken from his intestinal tract. The GI doc felt pretty certain that he was still battling GVH. We thought the return to straight blood coming out of his butt was pretty conclusive ourselves, but tomorrow will once again bring official word. But on the basis of visual evidence the BMT doc has decided to put Max's steroids up to a very high level to try and teach these graft cells a lesson or two. This will result in a VERY agitated kid, so it will again be difficult for Max to come around to his old self. The first time they changed his steroid dose it seemed to give us a glimmer of hope (we actually saw brown again!) so hopefully this change will finish the job.

As for lungs, no changes today. The four-hour constant albuterol nebulizer treatment is now entering it's third day so I guess that qualifies as regressing a bit. They just don't seem to have the best control of his breathing yet. I've said it before and I'll say it again, there is nothing harder than watching your child struggle to breathe. So far they are doing all they can to keep his lungs open and to get his secretions out. He's made it 9 days off the vent so far, with hopefully many more to come. The changes in the treatment for GVHD always seem to come around and give his breathing a little kick in the teeth so we'll see what happens.

Again I make this appeal: Who sent the fruit basket that we got today? Identify yourself so we can thank you proper! Also, we received an amazing stack of books from the New Jersey Ciacciarelli's and two great bears from one of Max's NICU nurses. Once again, we are running out of words to use to describe the gratitude we feel for the generosity we never could have expected.

Finally, please go international with your support of Max's Belgian girlfriend, Yasmine, who has just caught a nasty virus called CMV. Her link is in the "Friends with Hurler" section. It is so amazing to see what happens when Max's little army of supporters is dispatched around the globe. You guys are awesome!

That's about it, I think we'll be able to give a fuller picture tomorrow of Max's progress and his future. Let's hope that this meeting is positive, we could really use some encouragement from the docs. Not false hope, encouragement.

Always trying to keep it short, never succeeding . . .
--mike

Day +48

WBC 3.8

Today was much calmer than yesterday. Max actually got some sleep and it was so nice to see. He hasn't slept more than 2-3 hours straight all week. He continues to wheeze and is still on the continuous albuterol neb. He is also still getting the atrovent nebs every 4 hours and pulmicort (nebulized steriod) 2 times a day. They added a new medication that I cannot spell or pronounce to help with the secretions in his lungs. It is mostly used for kids with Cystic Fibrosis. We are still extremely worried, but comforted by the fact that he is resting more today.

Just when we thought that his gut was on the upswing, Max surprised us with two diapers full of blood. They will be revisiting the issue of the scope tomorrow. On our request, we will be having a care conference with the different departments caring for Max tomorrow afternoon. We are not sure where Max stands right now, and we just want to get everyone's perspective on things. As Mike mentioned yesterday, we now have all new docs as the rotations have changed. We just want to make sure that someone who knows Max well is always going to be following him. While we know the doctors can't stay on rotation forever, it is really stressful when your child is so sick and the staff keeps changing.

Just to give us one more thing to worry about, Max's central line is slipping out. We noticed during his dressing change that the stitches that used to be under his skin are now hanging out so as I write this the nurse is putting some steri-strips on it. If that doesn't work he will need new stitches. The biopsy of the bruises on his back came back negative for infection.

Grace now has a cold, not surprising since everyone at RMH seems to be sick. Her cold is not too bad, but enough to put me over the edge last night. I am sure Mike and I will get it since we are getting no sleep. I called my mom at midnight last night and had a full meltdown, so Grannie caught a flight today and is now in MN! We can get some much needed sleep now. She will leave on Saturday when Mike's mom arrives.

I am going to hold Max now so I will keep it short tonight.

-Margaret