After 13%, we got 14% and 16%. We were in Cincinnati today and spoke to Dr. D, we decided to try another DLI. One more big boost to see if maybe it may work. It's scheduled for this coming Tuesday, and I guess it's worth a try. Otherwise, no change. We also saw the hand Dr. C and he was just thrilled with how Max's hands look after the surgery!

13%, what to do, what to do?

Post surgery update

Engraftment back to 15% but we're still praying for a significant raise!
Max's surgery on Tuesday went really well, thank G-d, but everything just took a lot longer than was expected so we got home pretty late on Tuesday night. Max's central line was removed and instead he now has a port, so in a few days he will be able to take a bath and live happily without a stupid line hanging out of him. The port is like a central line, but entirely under the skin, when it is in use, an attachment is hooked into it and it works the same way. I'll post pictures to clarify this later. Here are some pictures from Tuesday. Enjoy!

Bandages Removed!

FINALLY!!!!! today Max's bandages were removed! Another surgery tomorrow, so please pray that it goes smoothly and successfully!

Hand Surgery

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Today was Max's hand surgery and it went pretty smoothly, thank G-d. Because he can't eat 8hrs. before surgery, we woke up at 4am to get a bottle of milk. Then, he had his last drink of apple juice at 8am so you can imagine he was rather hungry and so cranky as we got to the hospital at 11am today. Everything went smoothly and he went into surgery around 12:30 pm, right on time. The surgery took about 1.5 hrs. and the hand doctor came out to say that it went very well. In another 15 minutes I was holding my very cranky baby again and thanking G-d for returning him to me in good order. Max has a bandages on both hands that look like little boxing gloves and give him very little finger use. That so far hasn't stopped him from doing anything, except maybe taking off the hand dressings which is a good thing. Hopefully next week he can have them removed and until then, it's my job to keep them clean. I'm very happy to report that Max was still actively mischievous upon arrival at home and in a great mood. Just in case that changes and he has some hand pain, we have a prescription for Tylenol+Codeine for night time.
In other news, his engraftment is 17% this week.

Some good news, some not so good news

Happy upcoming Halloween everyone, stay safe and enjoy the parties. Max's engraftment went up a little bit to 18%!!! Although it doesn't change anything really, it is better than what we have been expecting and does give us a little bit of hope. Our doctor said that we could easily wait a few months if nothing changed and she doesn't think that Max is getting any GAG deposits in his brain for now. She does think that his engraftment will eventually start going down again though. So a couple percentage points up was a very pleasant surprise. Also his last GAG test found even fewer GAGs in his urine, about 30 was the number. When we started looking at a second transplant it was around 60, then went down to 44 without ERT. Max's GAGs going down to 30 which is very close to normal range is a good thing, but also to be expected with ERT. We're really praying hard that Max's engraftment goes up to a level where we know for sure it is safe for his brain and he doesn't need any more ERT, a consistent 25% would probably be great...
The not so good news is that Max will be needing surgery for his trigger fingers. From what the doctor explained and I read online, it is better to get those fingers taken care of as soon as possible so there is less risk of permanent damage. We went to see the hand specialist and he also said that Max may have some very early signs of carpal tunnel syndrome. We'll get an EMG to test if he does on Tuesday and then decide on a surgery plan. Most likely, Max will get his fingers and his carpal tunnel (if it exists) done in one surgery and G-d willing it will be a smooth success. It does break my heart a little that someone is going to cut into my baby's sweet little hands, but compared to everything else Max went through, I'd say this is less scary although we will be dealing with anesthesia which is never good for a little MPSer. Anyways, we'll deal with it as necessary.
The happily usual news is that Max is growing and playing and learning and we went hiking again in our park today.
Thanks for keeping up and have an enjoyable Halloween!

slow and steady

engraftment 16%. Everything else is ok. Max has a couple of trigger fingers that we're going to see a hand specialist in Cincinnati for. I'll post more as we have some more answers I guess. Also last Friday, Max's line ripped on the inside and we had to replace half of it, pretty short process, except to see if it works, you have to wait 4 hrs. in the hospital for the glue to set. We were supposed to be there from 9-3, instead we were there from 9-9. Oh well.

Max is 20 months today!

As the title suggests, Max is 2o months old today, which means he's only 4 months away from being 2 years old. Where has the time gone? We went hiking through our park yesterday and Max had a great time playing in the forest. In general we've been playing and dancing and learning and just having such a great time that it's almost unbelievable that Max's engraftment came back this time at 16%. It took a little bit longer to get the results because I switched the ERT sessions to Louisville, which is much easier, but they have to send his blood to Cincy for testing.

This last week was a little bit frustrating because they didn't have the Aldurazyme on Wed morning when we came. They promised it would be ordered for Thursday, but it wasn't. Then, the scheduler in OMO told me there was no room for Max so he'd have to wait until Monday. What!? After arguing with everyone I could, we managed a room for Max in Inpatient Friday night so he could get his medicine. We finally left the hospital at 10:30 at night, but at least we got his meds. Hopefully it gets better.

Also, Max has been taking his dressing off recently. We are currently trying the double shirt + one shirt backwards technique; we'll see how long it lasts. We need a more permanent solution though so if anyone has any suggestions please let me know. God willing, Max's engraftment will go back up and we could get rid of this stupid line and go swimming.

Much Love, and thanks for keeping up with us.

no changes

our plans are pretty much the same wait and see. Max's engraftment was 15% again last time. We'll check it again on Thursday. Just praying and enjoying life right now.

Day +????

I'm going to count the days again soon I promise. Max's engraftment is 17%, maybe we'll get to speak to Dr. D this Friday. It would be nice to hear from her.

Day +???

Sorry for not posting, we've been pretty busy. Max's engraftment went from 18% back to 16% and then last weeks was 17%. We'll see what happens this week. For now we're looking for a new donor, and also monitoring Max's engraftment. This level, in conjunction with ERT may be enough to hold on for another few months because he needs much less enzyme for his brain to develop and the ERT will help with the rest. God willing, his engraftment will go back to the 20's and we won't have to do another transplant. For now, we're doing weekly ERT and waiting and watching and praying. He loves hanging outside and going places and seeing people so it would be a big bummer to take that away from him again.

Day + 368

Occupational therapy this morning was good. I like it because it gives me new ideas of how to play with Max and how to maximize his development. Max likes it because he loves people coming over to play with him. Tomorrow we have speech therapy and occupational is back on Saturday. Also we have a visit with the geneticist on Wed. in Cincy and ERT hopefully starting on Thursday. Looks like we have a busy week coming up...Hopefully it goes smoothly and with good results. We're in G-d's hands...

Day +366

Grandma, Max, and Mommy at the Seneca Park Zoo

Max as a butterfly

Wegmans in the Strong Children's Museum of Play

I skipped yesterday because it was the one year anniversary of Max's transplant and we weren't celebrating because I guess it just seems like we're exactly where we started. I was very cranky and so Max was cranky and so we all were cranky so I made food and then in the evening we felt better. But still I was planning on a big celebration. To be honest though, one year ago today, I thought I would be mourning, because I didn't think we would survive. I guess yesterday we should have celebrated how far we've come and how well Max is really doing, and how grateful we are for even that, but I couldn't get past my disappointment. We had a very good day today so far. I'll try to keep focusing on the positive and remembering that we're in G-d's hands and that those are good hands to be in.

Day +363

Max's engraftment is 18%. We're not sure what that information means right now. The process has begun to look for a new donor and I've asked about getting some ERT in the meantime. There's still hope, but not much, that the engraftment will go up in the next couple of weeks. We have to remember that we're in G-d's hands.

Day +360

Here's good news: Max's immune system is perfectly normal!

Here's bad news: Max's GAG level went up in his urine study!

If this last DLI doesn't increase Max's engraftment level we will have to re-transplant ASAP.

Here's the plan: Everybody prays to G-d who has saved so many lives and already given us so much that Max's engraftment goes back up soon and without a second bone marrow transplant.

Bottom line: Please pray the best you can because we're desperate for help here.

Thank You.

Day +356

engraftment still at 15%, Max is playing and loving our new house and backyard. We all really love it so its been pretty hectic but good. I really want Max's engraftment to stabilize so that we can really take it easy for a while and just relax without rushing off here or there. I guess I want Max's engraftment to stabilize so that our lives can stabilize too. Also I really want Max to have a bath and in general do all the things normal kids do.

Day +341

engraftment: 15%

Day +340

Tomorrow we'll find out Max's engraftment level again. We're hoping for the very best!
Also, there's another thing I would like everyone's help with. It seems as though I have some trouble trusting in God to take care of Max. I notice a lot of families have the f.r.o.g. (fully rely on God) and others know that God is looking out for them and everything is for the best. I have looked into God's track record and it has been very impressive. Just in the last couple of years, God has brought us to KY where Max was diagnosed as soon as he was born and since then God made sure that our journey was very smooth. God made sure we were in Cincinnati, not Minnesota which is much farther away from home and countless of other miracles. We are so blessed and I am so grateful for everything. Why can't I fully trust that God will carry us through the rest of this? I just feel like I don't know God very well and can't trust what I don't know. For everyone that truly trusts and relies on God - how do you do it? Any suggestions?

Day +335

engraftment: 17%

Here are some fun playtime pictures with Max!

Day +330

Our orthopedist said Max looks great! He isn't too concerned about Max's back, but says that when we come back in 6 months, we'll have to take a good look at Max's hips and knees. So far though, as far as Max's bones are concerned, we're doing well.

Day +329

s It's Friday and Max is getting another DLI today. We had to wake up at 6 am to get here by 8:30 and we hope it's worth it. Later we're going for Max's orthopedist check up. Hopefully that will go well, and I'll try to update again to let everyone know what's new with Max's bones.

Day +326

After the big boost, the waiting, and everything else Max's engraftment is at 18%.

Day + 319

Sorry for the lack of updates, we've been having too much fun to sit down and write! Unfortunately I don't have any news about Max's engraftment. We have an appointment in Cincy on Thursday the 8th and that's when they'll test for it. So, until the 13th or 14th, we have no idea whats going on. I'm at this point willing to bet that nothing happened, but we'll see. Otherwise, Max is growing and learned to say "Deda" which means grandpa, and "Uff," his word for dog. He also finally says "Mama" and he's talking about me!!! We've been really enjoying our summer so far, but we're hoping still that we'll get some even better news regarding Max's engraftment. Thanks for checking on us, and I hope everyone had a fabulous 4th of July. I'll try to post as soon as we have some more news...

Day +300

I finally counted out the days yesterday and was absolutely shocked to find out that yesterday was day 299. I can't believe how far out from transplant we are and also how far we are from being done. Max's engraftment level from Thursday came back on Monday as 19% again. Lucky for us we have an amazing donor who again gave Max his blood. Yesterday Max got fresh blood cells that didn't even smell like cream of corn because they were free of preservatives. Dr. D said that this boost was also twice as big as the ones Max has been getting as far as cell counts go. I really hope this is the one that does the trick, but if not, we've got plenty more blood frozen again thanks to a generous donor. Maybe by the time we're done with all the new blood, Max's engraftment will have gone up.

Day + one of these days I'll count it out...

We're in Rochester visiting our family and friends! We love it here so much, and Max is so much happier with all the grandparents crowding around him and playing with him. We're having a great time. Our engraftment went up a little to 21% so I guess he's still stable.

Day + I'm not sure...

Clearly it's time for an update. The last couple of weeks have been kind of more difficult, although not too bad.

Saturday, May 15th: Max started working on the fever in the morning and by around 6pm he woke up from his nap with a fever of 38.5C. We went to the ER here in Louisville and while waiting for his antibiotics, Max took a dive, head first, into the wooden arm rest of the chair. He was fine, but he did chip his front tooth pretty badly and so we needed to get it removed on Tuesday. Finally, we got to our room and went to sleep around 2:30am.

Sunday, May 16th: Max's blood cultures came back positive for a gram positive bacteria, so I asked that we be transferred to Cincinnati Children's Hospital in case Max's situation was to get serious. We rode there in an ambulance, and I must say that it was pretty cool. Max watched the Clifford movie the whole way except for a brief time that he was napping and I read a book and chatted. When there was traffic on the highway, the driver turned on the siren and traffic split, no kidding, like the red sea. When an exit was closed because of an accident (no one was hurt) we were waved through. I think other than Max being sick, this was the best ride to Cincinnati ever. We got to Cincinnati and started another antibiotic, Vancomycin.

Monday, May 17th: We found out that Max's blood culture came back positive and were thinking that we may possibly have to remove his central line, something we were not looking forward to. We also consulted with the dentist who confirmed for us that Max's tooth had to be removed.

Tuesday, May 18th: Max's tooth was removed. Max's first negative blood culture!!! We also found out that the bacteria in Max's blood is sensitive to Vancomyocin.

Wednesday, May 19th: Another negative blood culture!

Thursday, May 20th: Another negative blood culture! This means we don't need to have the line removed and we can go ahead with the boost of donor cells on Friday.

Friday, May 21st: Max gets a boost of donor cells and Roman comes to take us home!!!! Finally I can have a quiet night...But not so fast, Max still needs fluids overnight and his antibiotics so at 5:30am we have to wake up and switch the fluids to Vancomyocin. Not Fun. But, this is MUCH better than being in the hospital 24/7.

Since then we've been doing well. Max has been walking everywhere and now he walks around with his bottle and rubs his belly, the sign for yummy. Really funny and thrilling to watch him thinking and growing into a real person.

Yesterday, we got some bad news about Max's engraftment; it went down to 19% and now we're worried. I wish there was something more we could do, it seems like we keep doing the same thing (giving Max extra donor cells) and expecting a different result. We used up the last bag of blood on the DLI and now the donor agreed to give more blood. Our donor is such an amazing and inspiring person and we are really blessed to have him in our lives, even though we haven't met him yet. I hope some day I will have the honor of donating my bone marrow. We emailed Dr. O at Univ. of Minnesota to see if he has any other suggestions as to what to do. We trust Dr. D and think she is brilliant, experienced and really knows her stuff, but maybe an extra brain couldn't hurt. We are just so worried because we want Max to live a long healthy, happy, and successful life and for that to happen Max's engraftment needs to go up. We need to know that we are doing everything. Tomorrow we're meeting with Dr. D again and hoping that we hear good things (and that they're true).

and one more video...

Max eating ice-cream!

Day +265

Enjoy!

Day +261

Max took 4-5 confident, forward steps today!!! I was in too much shock to even count how many or to get a camera. He just started walking and I stood there, stunned, with my mouth open. I thought that he wouldn't be repeating this trick for a while, but then when I was changing him before bed, he took four steps to the dresser and looked at me like, "what's the big deal, mom?" I was clapping and cheering, but he doesn't seem to be nearly as excited as I am. As soon as I can catch this on video, if he does it again, I'll post it. He also started saying "day," the russian word for "give," pretty regularly to get what he wants. Those are some BIG leaps for my little man.
He weighed 10.37 kgs today at the doctor's office and measured 79 cm. I'm not surprised at all judging by how his appetite increased. Unfortunately though, Max's engraftment from last Friday came back at 23%. Next week we'll have to come back and get IVIG, and the week after we'll get Max some more donor cells. Otherwise, not much to report, thankfully. We're just happy that Max can play in the grass like a normal little boy, just as long as there aren't any other little people around. We thank G-d a lot.

Day +254

Max finally hit 10 kgs!!! He weighed in at 10.2 kilograms. Finally! Max's engraftment went up a little bit to 27%, but that still means we need to do some more boosts of donor cells. We also had our first meeting with our new occupational therapist and our new speech therapist on Thursday. It went really well and we can't wait to see them again the week after next. They both seem really nice and very knowledgeable. Also Max's mitogen immune system study came back normal!!! This means that Max's immune system is attacking everything it needs to be attacking. I guess we're getting closer and closer to a fully functioning immune system. Max has also been taking one step for about a week now and on Wednesday he took 3 whole steps on his own! They were side steps, but steps. Otherwise we're just trying to enjoy our park privileges and stay healthy. We just need Max's engraftment to go up already!

Day +241

We're allowed to go outside and play in the grass without the rain cover!!!!!!!!!!

Day +238

Sorry for the lack of updates... Our latest engraftment level is 24% and at this point I would just like to at least know that its for sure enough enzyme for Max to live a normal independent life. Max still isn't walking, but the Child Evaluation Specialist came by last Thursday and said that she expects him to walk any day. She did however say that he is a little bit delayed in speech and fine motor skills and recommends that we get one hour of speech and one hour of occupational therapy every week! I'm very excited to have the help and I'm hoping that Max will thrive with the extra interaction. Him hanging out with just me and in just one room all day every day can't be helping. The First Steps coordinator even found a Russian speaking speech therapist so we'll just have to hope that she's available.
We all got sick on Monday night. First, I spent the night in the bathroom. Then the next day on Tuesday Max and Roman both sick and I was better. But switching Max from formula to Pedialyte did the trick and we're back on track today. Luckily Max's temp. never got higher then 37.3C so we didn't have to go to the hospital. We did quite a bit of praying though yesterday and were pretty worried.
On Friday we're going to do another d.l.i., aka boost of donor cells and take an engraftment test. Also, we're still waiting for the results of Max's immune study to see how much of his immune system has returned. I'm really hoping that he will be able to go outside without a mask and get to play in the grass. I think he'll really enjoy it.

Day +223

Max's engraftment keeps moving around in the 20's. Last time (Monday) it was 28% and the engraftment from Friday is 25%. Otherwise we're doing ok, Max is as cute as ever. At our next appointment the Drs. will do an immunology study to see how Max's immune system is functioning. I'm anxious to find out. I'm also anxious about Max's engraftment level and I wonder if he'll always just stay in the 20's forever and ever. I wonder if it's good enough to make the enzyme he needs and get rid of his GAGs.

Day +217

We went to Rochester for a week!!! and just got back on Sunday, so that's why i haven't updated yet. Max's engraftment went down to 24%, but otherwise the trip was a lot of fun for everyone. Especially Max who got constant attention, from his grandparents, uncle, and Simon (our miniature schnauzer).

Max walks so long as you give him a hand to hold. He stands comfortably on his own and I think he can walk on his own too, but he's be scared to take that first step. We did go to an Occupational therapy evaluation and he is behind a little bit so we're going to get home-care occupational therapy, speech therapy, and possibly physical therapy depending on our PT evaluation on Thursday. He's definitely behind in fine motor skills and we'll just have to work on that.

Day +203

Max is 13 months old today, so we'll be taking some cute pictures to mark the occasion. Also Max's engraftment from Thurs. went up a little bit to 25%. I just hope nothing goes wrong and Max's engraftment goes up. Our weather here is beautiful, 64 degrees for the last few days!

Day +200

It is day 200!!!! That's pretty exciting and the weather is beautiful and Max is amazing. He no stands up on his own and stays standing for a full few seconds before plopping back down on his butt. We've been going outside with him to practice walking and he loves looking at the world around him without being strapped into a stroller. On Thursday Max got his weekly IVIG and having sensed a little congestion in Max, Dr. Joshi asked for a nasal swab. As it turns out Max has a common cold virus, but since we've been getting the IVIG anyways weekly, we didn't notice many symptoms because the IVIG was helping him fight it off. Hopefully it doesn't get worse. Our engraftment is still pretty low and Dr. Davies ordered another GAG urine study to see if Max is still making enough enzyme at this low engraftment level or if he needs so ERT again to keep him going while we work on his engraftment. Friday we came in and did another DLI, I think we only have one more bag of donor cells left after this so pretty soon if Max's engraftment doesn't rise, we're going to need to ask the donor for more of his bone marrow. I hope he doesn't mind too much and I hope it doesn't hurt, because he is the one saving Max and we're so grateful forever and ever. I hope someday Max will be able to meet his blood brother from another mother and we could all thank him in person.
All things considered, sure Max's engraftment is pretty low, but day +200 is looking pretty good.

Day +193

Engraftment from Tuesday: 20%.

Day +190

Max's engraftment from friday came up to 28% which is the highest rise in engraftment that we've seen. I'm only a little bit excited because there is no way of knowing what's happening next, and I'm tired of jumping and then sinking as the engraftment goes up and down. I want it to just go up to 100% already and stay there! Otherwise Max is still doing ok and I'm happy with that. Max's orthopedic appointment on tuesday went well - his kyphosis is still flexible, meaning that his back is only bent when he is sitting on his own and not all the time. This means that Max doesn't really need a brace yet and maybe won't need much intervention at all ever. We still get IVIG weekly and nothing else has really happened which is pretty great.

Day +183

We've got words!!! Max now pretty consistently says "bye-bye" when someone leaves. Of course he waits until the door closes, but after that he definitely has been saying it. Also, he says "papa" to Roman, I haven't heard it, but Roman and my parents have. Our engraftment is still at 23%.

Day +176

It's been so long since I posted that I had to figure out all over again what day # we were on. It's really nice being home and not thinking about the days as much. Yesterday Max turned 1 and we were very excited to have made it this far. Last year one of our nurses in the NICU gave Max an outfit for his 1 month birthday that was a size 12 mos. I remember thinking that it was very optimistic of her, but we made it!!! Max is doing ok for the most part, but his engraftment is still down at 23%. We're probably going to get another DLI tomorrow, but today we're going to Cincy for our IVIG. It'll be Max's first trip in his new big boy car seat!!! Enjoy the photos!

Day +162

We're HOME!!!!!!!!!!!!
Last Friday we came in to do our weekly IVIG and Dr. Davies came in to see us. I asked if someday we would be able to go home and she said whenever we wanted. So we went home on Saturday, which was my birthday. Best birthday present ever from Dr. Davies, Roman, and all our wonderful friends that helped us move!!!!!
Our engraftment is still down at 24% this week so right now we're at the hospital getting another DLI (booster). I really hope this one works. Also Max's bum has had better days. its really really really red, and especially the skin around his penis. The doctor looked at it and said it didn't look like a fungal infection, just a rash. We started putting Calmoseptine on the rash, it's like concrete and I can't wait to see what happens when I try to take it off later when it dries. Not much else to report, Max has been playing peek-a-boo by putting a blanket over his face and "hiding" also he's still walking with furniture and growing teeth.

Day +148

Hello,
Max's engraftment came back yesterday at 34% so today we came in again to get another boost of donor cells. Hopefully this works. Max still is testing positive for C-diff and he's on Vanc and he's eating less, but holding his weight steady.

Day +141

Max's adenovirus quantity from Tuesday: ZERO!!!! His anc had gone up as well to 1.24 and his wbc is still around 2.5 so that's also very good. Max also started trying to clap his hands on Tuesday. It happened so suddenly, we haven't even been working on it. We've been trying to get him to say bye bye and wave, so when he waved bye-bye for the home-care nurse we were very proud and all started clapping and saying good job. He started clapping and smiling too! I thought he was just trying to show off for some reason that day, but he's been doing it since and he even clapped for our speech therapist today! We're still having a difficult time getting him to eat solids, but he seems to really like salty crackers. He's for now gaining weight and getting taller from the formula he eats too. He's now 8.9 kgs and 75.5 cms. He's almost average size!!!
On a negative note, Max's C-diff came back so now he has to take Vancomycin 4 times a day and it is some nasty stuff. Also, we're still waiting for our engraftment results from the second boost, I really hope we get some good news in the next couple of weeks about that.
Grandma, Grandpa, and Uncle were here over the last few days, so I added some pictures.