Hip Surgery - January 20, 2015

So here I am, way behind on writing about Max's hip surgery. My apologies on the lateness, but I will do better. I intend to write about this mostly in case someone needs to read about the details of what happens (at least in our case).

Background:

Sometime in the fall of 2014, we went to see our orthopedist who is a specialist in hips and legs at Cincinnati Children's Hospital. We knew it was coming, so it wasn't a surprise when he started talking about hip surgery. Generally, MPS 1 kids have two deformities in the hips. One is that the femur is straight instead of bent inward, and the other is that the socket is not cupped enough so if we don't want to almost definitely end up in a wheel chair, the idea is to reshape the hips as much as possible now, so that they *hopefully* will continue to grow normally. The procedure is called a femural osteotomy and a pelvic osteotomy (I think that is what I remember, but I don't have my papers with me). 

First one hip would be operated on, then 6 weeks recovery, then the second hip, then another 6 weeks recovery. The entire 12 weeks would be spent in an almost full body spica cast. We were/are still really concerned about Max having to spend 12 weeks in that cast, so we looked for some second opinions. Maybe someone can do both hips at once? We asked the facebook group (MPS1 - Hurler's Syndrome) and many kids have done both hips at once, and many didn't need the full body spica cast. Unfortunately, when we contacted some of the same doctors in Gillette Children's Hospital we were told that it is too much surgery for one day. We even asked our own doctor if there is any flexibility on the cast and how much needs to be covered. No budging on his part, so here we are - almost done with our 4th of 12 weeks in a full spica cast.

At first we planned on doing the procedure in the summer of 2015, but it was scheduled for January 20, 2015 and we decided that would be a better time anyway. This way, our summer is much less likely to be ruined by an enormous spica cast, that Max hates with a passion. Currently, Max is getting home instruction so hopefully, he won't get behind in Kindergarten and be back in school in time to finish with his classmates.

Day of Surgery - January 20, 2015:

The day of the main event, we woke up at 5:30 am and walked over to the hospital with Max, R, L, and myself. I'm not sure if it helped Max to have to whole family there, but for us it was kind of a pain to have to entertain baby sister while answering questions from doctors and calming Max's nerves. I'm not sure if that is the better way or not, but it was a major surgery so we felt we should all be there together.

Max, for the record, did not really know what was planned at this point. We chose not to tell him anything except that he had an appointment with the hip doctor. The day of surgery, we told him that the doctor may want to fix his hips and then he would have a really big band aid that people call a cast. Then we tried to get his mind off of it for as much time as possible. We thought about telling him more and sooner, but Mr. Max is a worrier, and since he can't change what will happen, and has no real way of planning for it, we decided that this knowledge would only scare him without any of the benefits of knowing what is coming up. 

The surgery was to take 3-4 hours, which it did, but I didn't realize that it meant the *actual* surgery. The 3-4 hours did not include, the prep time in the OR, the X-Rays, or putting the cast on. He was not in recovery until 8-9 hours after he was taken to the OR and apparently everything went well. He was cranky but not drowsy when I came in to see him. He had an epidural instead of the morphine drip so he was a lot more alert than I expected him to be. Max was also pretty hungry, but then only really wanted to eat ice cream when that was finally allowed. He was also in a lot of pain because I don't think he was getting enough pain medication.

Night after Surgery:

We didn't sleep at all. Literally, we slept approximately 2 hours divided into one 45 minute chunk and a few tiny moments. Every time Max fell asleep, I would go to bed, close my eyes and he would wake up instantly and cry in pain. We constantly asked for more pain medicine and the nurse gave as much as she was allowed but it wasn't enough. She even called the pain management team but they wouldn't increase anything. It was absolutely awful. Max was having muscle spasms and they were keeping him up all night in pain. We watched movies all night. It turned out the next day, that the Valium and the Oxycodone dosage that Max was getting was smaller then what is recommended for his weight. Apparently, it is common practice to start small and then move up if absolutely necessary. Obviously, I was not happy to hear that after that first sleepless night. The day after, the dose was increased to the one appropriate for his weight and he slept just fine all night.

Going Home:

We were discharged on Friday, January 23 from the hospital (my birthday present, apparently) after Max finally pooped - complete with cheering all around his bed and happy applause at the sight of the first turd to come out. So off we went. Max at almost 6 years old could not fit into the Hippo car seat that Cincinnati Children's provides with the surgery, so we had to use the belt vest and lay him across the back seat and buckle him in. This isn't great, but every time we have used it, he very successfully fell asleep, gifting me a very pleasant and relaxing drive. Getting him in and out of the car with his enormous spica cast is more difficult and much less relaxing.

Five!

It has been FIVE YEARS since Max got his life saving new cells. Life, thank G-d, has been so wonderful, busy, and normal that I barely remember the day. I had to look at the entry from this blog on August 20, 2009 to remind myself. The entry is short.

I remember when our first engraftment results came back at 100% on Max's 7 month birthday! I can still see Dr. Davies dropping by unexpectedly and me meeting her at the door to find out what was wrong and seeing the results in her hand. I can still feel the relief when she told me that Max was 100% donor cells. I vividly remember the day Max's urine study showed GAG deposits again because his engraftment had been dropping and was now at 15% and not enough anymore. It was Roman's birthday, August 13, 2010. I remember that when I told Roman the next morning, we just packed up our things and drove to Rochester. We needed our family. Looking at the blog reminded me of how sad we were, even on the one year anniversary of Max's transplant. I couldn't imagine being here, where we are, four years after that first miserable anniversary.

We started Max back on ERT to help in the transition between the first, and possibly the second transplant. But while we were still in the process of finding a donor, and deciding on our future path, our geneticist found an article on PubMed. I believe, this geneticist for the second time, saved Max's life. The article was a case study of a child with an engraftment of approximately 10% (lower than Max's at the time), who instead of being retransplanted, went on to live a normal life and do well in a normal school. He was 11 years old. We gave this information to Dr. D and she contacted the doctors to see if this was something that we could use. We agreed to continue ERT and use MRS brain scans to track any GAGs that may accumulate in Max's brain. The hope is that the 10% engraftment provides for enough enzyme for Max's brain, while the weekly ERT provides enough enzyme for the rest of his body. Here we are, and Max's brain is still clear from GAG buildup. Thank G-d.

Max went to his first day of Kindergarten, incidentally, also on Roman's birthday this year. He has to go to the doctor a lot more than most kids. He knows medical terminology better than most kids. He has a weekly infusion for 3 hours, a port that recently got infected and needed to be replaced after 4 years of service. He has a lot of surgeries behind him and many more serious ones ahead of him. But Max is 5 years old, alive and he is thriving, and he is learning to swim and play soccer, and he is the best and most loving big brother to his sister, and he knows his letters and his numbers and hates being told what to do.

Thank G-d.

It has been 3 years since Max got his brand new cells!

I think the magnitude of this miracle is best shown in pictures.

Transplant Day. 8/20/2009

                                                                    1 year post transplant.

2 years post transplant. 8/20/2011

Thank G-d! This is 3 years post transplant! 8/20/2012

Almost three years since the transplant!

I can't believe it has now been 7 months since I have updated this blog. I apologize to everyone reading and to everyone that gave up and is no longer reading. I understand.
We've gotten so used to our almost perfectly normal happy lives that I've lost track of the blog and even new MPS drugs. I guess I've gotten comfortable.
Max is doing well so far. We've tested his engraftment level monthly and the most recent result was 11%. His MRI and MRS has shown normal brain. We have some appointments coming up on September like ophthalmology since Max's right eye looks a little lazy sometimes when he relaxes. We also have ortho coming up and a visit with Doctor D. So far, thank G-d, no serious bone issues or brain issues although I am a little concerned about the ophthalmology visit and Max's eye health.
Max loves school and went to summer camp this year and loved all of the awesome field trips. I have been busy working and so has Roman. I guess this is pretty much what we were hoping for! Thank G-d!

Updating for the last few months.

Oookay. I didn't even realize so much time has flown by since my last update. We have been a little busier than usual around here but all has been very well, thank G-d.

First of all, Max is doing just fine! He is loving daycare, and I can't even count the words he is using. He regularly speaks in full sentences, but sometimes it's hard to understand his pronunciation. It should also be noted that very little of his speech is in Russian, but that was to be expected when we sent him to daycare. He loves going all day and learned all of his colors. We are now working on learning to count. Potty training is not even a little bit fun. It seems he knows what is going on, but refuses to tell me. At one point we had the potty in the living room and so Max can sit on the potty and not interrupt his play. He made it in almost every time. Then I got tired of him pooping in my living room, and he refuses to tell me and go to the bathroom, so we're not sure how to proceed from here. Buying him nice underwear with cars on it didn't work.

As far as medical stuff is concerned, Max's engraftment was 10% from December 30th. Since we're testing it only monthly, the number in November was 11%. In October, we tested twice and the first time was 7% and then went up to 11% (I think, or maybe 10?). I'm pretty happy with it, if it stays this way.

Max had his second MRS/MRI and there was no change from the first one! So we are still clear as of October. We'll probably test again in April and hopefully there will still be no change.

The biggest reason for why we have been so busy is that I got a job! It is really nice to be working again. It is really nice to wear nice pants and nice shoes and a nice sweater and not worry about anyone smearing yogurt all over me. It is also really nice to talk to adults! But, and there's always a but, I miss hanging out with Max. I guess this is what everyone goes through, and I have to say that working full time is 1000 times better than being a stay at home mom full time. Maybe someday I'll be able to work 30 hours a week, which will probably be better, but I don't really know.

So, thank G-d, we are doing ok. Thank you for keeping up with us and enjoy the pictures!

11%

Engraftment

The one from 8/29 we have is 10%, and we're waiting this week for an engraftment from 9/6/2011.