Thursday, February 22, 2018

Rett Syndrome. One year Post Surgery




One year already. Only one year?

We all know I had pretty much given up on blogging long before Abby's Full Spinal Fusion One year ago today.Yesterday I sat and wondered when the last time I updated on blogger and was quite disappointed with myself that it was before Abby's surgery. I have taken the no computer lazy route and just been updating on Facebook. For those that have always followed us here I apologize. For those younger Rett parents that look to me for guidance.....Rett causes memory loss in parents. Prepare yourselves.

I usually blog with pics and build my story around them. There are just way too may pics in a year to do that in this first post so I will end with pics and captions.

Feb 20th 2017 we traveled from Kansas City to St Louis for her surgery. Her father and I were a bundle of nerves to say the least. Feb 21st she was fitted for her pic lines and we were sent back to the hotel. That night her Aunt came in and stayed the night with us. Thank God or I may have killed her father. You know cause he insists on being the most annoying person on earth when hes nervous. Her Aunt ended up staying the entire stay with me.

Feb 22nd we arrived at the hospital just before 6 am. Everything is a blur but they let me hold her until they wheeled her away from us unconscious. We were expecting a 8-10 hour surgery being that she was having fusion from T2 to Pelvis. It was somewhere around 4.5 hours when the doc was suddenly standing there after we had not had our hourly update and it was almost 45 minutes late. I jumped up and let out a little yelp for him to tell me it had gone well and she had done great. There was minimal blood loss and they had been able to transfer her own blood back into her.

Almost a 2 hour wait until they took us back to recovery because they were waiting for her to come around. That didn't happen and she was transferred to the PICU. It took almost 32 hours for her to regain consciousness and open her eyes. She was not coming out of anesthesia well and for Rett that can sometimes be expected. When you are living it it is absolutely terrifying. The only girl I knew that had had the surgery was awake when we went in to see her. Abby is stronger than that girl for the most part. At least physically. Abby could walk and the other girl never had. As the hours ticked by and the heart rate and blood pressure and o2 monitors went off almost constantly I came as close to losing my mind as I ever have.

I have actually sat here for a good long while trying to decide how to describe the next part. I'm going to do it the best I can without being an ass, hurting the wrong peoples feelings or leading anyone in the wrong direction. Please be aware these our our experiences and our experiences only.

We had her surgery at this hospital, 5 hours from home for good reasons. Our hospital in Kansas City just didn't know Rett Syndrome and St Louis had opened a Rett Clinic that we had been attending 2-3 times a year for several years. It made absolute sense to have the surgery there. Where there was a Rett Clinic right across the street. Where her Neurologist did rounds. Where we had discussed our anesthesia fears with the team for years.

When Abby wouldn't come around and then when she did and she was tachycardic and hypertensive with low o2's I begged for the Neurologist. Even for just a consult. Something wasn't right and I needed someone that knew Rett to look at her and for people to quit saying standard protocol because there is nothing standard protocol about Rett Syndrome. I was shrugged off time and time again. I finally called the Rett Clinic myself and they didn't seem to get why I was calling them or have any knowledge that Abby was even in hospital. As calmly as I could I begged for someone to come talk to us or take a look at her chart and they were completely oblivious to why they would need to do that, It was scoliosis Surgery and that wasn't their specialty. What!? It was Neuroscoliosis caused by Rett Syndrome and there are several precautions that need to be taken. Long story short...in 9 days we never heard from the Rett Team and they never called until it was time for the next appointment. Absolutely unacceptable in my book. My daughter is not a number in your cattle line for your grant funded clinic. She is my daughter, My flesh and blood. My heart.

Once we got home Abby was basically unable to leave the bed and developed Paralytic Ilius. Which at the time we referred to as unable to poop and keeps puking. With each episode she would get tachycardia again and hypertensive which meant great pain. At the end of March she had an 8 day stint in Children's Mercy and it was determined that the condition was caused by Propofol InFusion Syndrome. The one drug we had absolutely forbade her to have during anesthesia because of its risk in Rett Syndrome. It turns out that telling 3 clinics every time for 3 to 4 yeas that if there is ever a surgery that your child is not to be given Propofol means nothing. It also means nothing when you tell the surgery anesthesia team in the pre surgery visit and they tell you that they are aware and there are other meds they can give her. I can go into all the letters and emails and phone calls and meetings but it doesn't matter now. The hospitals have no obligation to have any ethics, standards or knowledge. Therefore will will not be returning to St Louis Children's. On that note we had and still have absolutely no problem with the surgeon that did the surgery. He is one of the best in the nation and spends half his time working out of Shriners. I only wish we had had her surgery there. This is the complete carelessness of the Anesthesia team and what I believe happens when doctors and teams work out of 4 different hospitals. That and the Rett Clinic. I don't know that ill ever take her to another one except that I have thought over and over that this wouldnt have happened if she was with Percy, Glaze or Djucik,

The one good thing about this stay was that out attending was  former student of another Rett Mom in Arizona. I knew immediately that he had some connection and on his second visit I looked at him and asked what his connection was to Rett Syndrome. He looked at me in surprise and told me his former favorite teacher from his youths daughter has Rett. I asked here he was from and he told me and I immediately named the teacher. We had her on messenger within minutes.By the time we checked out that attending made sure we had the best of every specialist at Childrens Mercy and made sure that each had experience with Rett. He was even able to get us in with a Neurologist that did all his studies at Texas Children's and had worked with Glaze and Zogbi.

Fast forward one year...I thought about taking your through the whole year but its been rough. A ton of therapy, Standers, walkers and gait trainers and she can just now take baby steps. No longer than a minute or so before her legs give out. Pre surgery she was walking 20-30 minutes at a time with up to 300 steps in distance. There is an ongoing issue with her left leg and foot that we cannot figure out except that that side is very stiff and she drags the leg and or foot.

But its our Abby and she is a trooper. Happy as a clam. Demanding as all get out. A big gigglebox. She is in school full time...that didn't happen until Mid October and its her favorite place to be. She's officially a teenager now. I know right? When did that happen? She is mastering the art of sighing and rolling her eyes.

Thank you to everyone for your love and support. I don't know what I would have done without those of you that have continued to follow us and have stuck it out.



God Bless