Friday, January 27, 2017

Abbys Rett Syndrome Journey with Scoliosis

If you have kept up with this blog in the past you know that one of our biggest concerns with Abby has been her scoliosis. We first noticed in when she was at 15 degrees and still in pre-school.

Over the years we have done everything the specialists and other familiars have suggested to keep her strong, keep her walking and keep her spine from bending. As per usual we sometimes get to living our lives and forget that Rett Syndrome is in charge and we are helpless. Though it has never stopped us from swinging at the invisible monster, it has won once again.

When Abby started Middle School this year she became so much more mobile. They followed the IEP to a T and Abby was up and out of her wheelchair as soon as she gets to class. She had to walk to her classes, with an aide and a gait belt of course. She participated in PE three times per week as well as her scheduled PT sessions. I thought there was no way her back would get worse. She was getting so strong.

Then it happened. One day as we were leading her down the hall to the chiropractors table she seemed to be limping. At the time we thought it was because both her aide and I had ahold of an arm and she tends to lean in and not try to carry herself when she gets too much support. The next morning her PT called me and said she really thought Abby's hip was out of place because she was limping and flamingo(ing). She absolutely would not bear weight on it.

We called the specialist in St Louis not knowing what to do. We knew she needed to see an Ortho but we have been seeing the docs in St Louis since they opened the Rett Clinic and hadn't been to Children's Mercy for anything but PT appointments. They sent her to the Ortho here locally and we got xrays the very same day. Unfortunately they showed that Abby's curve which had measured at 45 degrees for a year and a half was now at 55. Her Curve goes to the left in what they used to refer to as an S curve and were now referring to it as a C curve. Her right hip was also out of place and she had stress fractures on the top of her femur. So basically her curve had pulled her hip up and out and the femur has been rubbing on bone. Thank God for a high pain tolerance but I felt like such a horrible Mom because I hadn't noticed.

Within a few days St Louis Ortho had received the xrays and the report and we were on our way to see their hip specialists. A five hour drive and 5 xrays later we were told that there was nothing they could do for her hip until her spine is straightened and that she needed to see the Spine Specialist. As our luck would have it he wasn't in that day so 5 hours back home we went with a note to limit PT and physical activity. I was probably as frustrated as I have ever been.

Flash Forward a few weeks and the Spine Doctor called to let us know surgery could no longer be avoided. She had progressed to 61% from 55% in a week and her lower organs were starting to be affected. The surgery date was set and Preop appts scheduled.

We drove back to St Louis last week and has her pre op appointments. Seven more xrays were taken. 7 tubes of blood. A meeting with Anesthesia and a meeting with the actual surgeon performing the operation. If I tried to pretend, even for a second that I wasn't scared out of my mind Id be lying to you. I did learn that I didn't do this to her. It is the Monster. The nature of the beast.

When Abby was first diagnosed we had someone high up in the Rett world tell us,"People are going to try to tell you to put her in every therapy there is. They will try to tell you you it is a must to keep her strong. Well I'm here to tell you none of it will make any difference and you will be wasting your time. The word you need to remember here is respite. Respite, Respite Respite...."

I remember what an asshole I thought she was for telling me that. I still think it was a shitty thing to say but 10 years later I now know where it came from. The Spine specialist told us that the errors in the connection between brain and body is what causes the scoliosis problems so all the PT and muscle building activities, the swim therapy, the chiropractic and massage,while good for her as a whole were never going to be able to stay or reverse her curve. Even with a very strong core the spine is going to do what the brain tells it to. And the Rett Syndrome brain is kind of a jerk.

I will never regret all the years I kept her in activities. She loved nothing more than riding a horse, except maybe for swimming. She loved the PT sessions and her therapists. These things were her normal. Ive said time and time again that our kids don't get to go outside and play. They don't get to do ballet or cheer. Therapies are their play and socialization. Id do it all again.

So Abby will be having full spinal surgery, rods and spacers and hardware from T-3. 3 vertebra down from her neck clear to her lower lumbar on February 22nd in St Louis. She will also have her hip relocated if that's what you call it and pins set to hold it in place. I can't tell you if she'll walk again but I am sure she'll give it all she has.

As of now we will spend 2 to 3 weeks in the hospital if everything goes well. She will spend the first few days in the PICU. I will be able to stay with her through everything but the surgery. They offered to have her transported, which is customary to a rehab clinic from there for a few weeks before we go home but since I wouldn't be able to stay at her bedside there we will be returning home upon discharge. Once home Abby will be out of school for 8-10 weeks minimum which pretty much ends the school year for her. We have already applied and have been approved for Homebound schooling. A teacher will come out for 5 hours a week and the school nurse will come once weekly to check on her. We will meet after she is home to decide if she will get any therapy at home and that will all depend on doctors orders.

A friend has set up a Go Fund Me Page to assist with the costs of traveling back and forth, hotel stays, funds for me to eat while she is in the hospital and for all the extras we will need to keep her home and in a sterile environment while she recovers.

We have also set up an Amazon Wish List page for those that would rather help with supplies. Those will be delivered directly to our home.

Since October we have had Nursing for 5 hours a week and 6 hours every other Saturday. We are approved for 35 hours a week and will be approved for 12 hours a day once she has the surgery but Home Health in our area is apparently lacking in staff because we have 2 companies that can't seem to staff us for months on end. After Abby has this surgery there is no way I can do this on my own. At least not at first. So if anyone local would like to volunteer to help out we would be eternally grateful. My neck and back will owe you forever.

I will keep everyone updated here and on her Abbysworld or AbbysworldKC Facebook page.

Prayers for all of us would be greatly appreciated.

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