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.
