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 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

Friday, February 10, 2017

7 years.

 Yesterday we went to see Abby's long time PT with Children's Mercy Hospital. She has been in the PT program since before we had a diagnosis. All in an attempt to keep her as strong as possible as she is so low tone. For the last seven years she has had the same PT. Her name is Wynde.

Abby has almost always given Wynde a very hard time. Years of dirty looks and huffing and puffing if not pretending to fall asleep all together so she didn't have to work. Wynde never gave up on her. Our goal with Wynde was always Abby's back. She worked to keep her flexible and as strong as possible and to hold off the inevitable....Scoliosis surgery. We went yesterday as a final exam and assessment to see where she is before the surgery as we will more than likely still receive most of Abby's therapies through Children's Mercy in some way or another. I tried to keep it together as best as possible because I know that Abby will not return to least not for a very long time.

I warned Wynde when we got there that Abby had worsened a great deal since we had not not been in to see her on a regular basis. I think it was as hard for Wynde to see as it was for me.

The standard I am not going to cooperate look.

Wynde asked her where she hurt and her hand immediately went to her hip.

Wynde keeping her as straight as possible but you can still see the crookedness of her spine.

We used to be able to stretch her best in this way. She was not having it and attempting to stay off her left side. The alternative was to be on her right..where her hip is out of place.

This is where I think we realized therapy was not going to happen.

We took a break at this point and talked about the surgery and her future and what to start in the way of therapy once she has recovered enough. We decided on swim or hydra therapy. It is standard with post op Scoliosis surgery to start with pool therapy.

As I write this we are 12 days out from surgery. 10 days until until we drive to St Louis. We are trying our best to come to terms with it. As always I am not nearly as strong as she is. She continues to amaze me every moment of every day. I bitch and moan and whine over my back, my neck, my lack of sleep. I don't think even if Abby could complain that she would. She is the strongest, happiest little girl in the world. I know the Rett Syndrome gives her an incredibly high pain tolerance. I just hope it doesn't keep her from being able to express how much pain she is truly in.

I  have been attempting to explain what is happening to her. Every time she just smiles and kisses me. I tell her that we are going to be going to the hospital that is far away soon. I tell her that the doctors are going to help her so her back and legs don't hurt all the time. I tell her that at first it is going to hurt very very bad but Mommy will be there and I will help her all the time. This is when  I always get a smile and a kiss.

She is a very tiny Superwoman. I am in complete awe of her. Every moment. Every day.

Thank you all so much for all of your support, prayers and well wishes. They are appreciated more than I can express.

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.

Friday, January 6, 2017

And We Are Back! Hopefully!

Coming back to the blog is something I have thought about often. There just never seems to be enough time..... But too much time has passed and there have been so many changes I'm sure people will want to stay updated. But first, a little catchup.
its been nearly two years since Ive made a decent post so I wont bore you to tears with every last detail. Here is just a few snipits....

Abby 4th grade, Still such a happy girl.

Abby saying goodbye to aide of 7 years
 Still the coolest kid at Hippotherapy
 Chilling with her new aide
 Always better to ride with RBF
 In the 6th grade now. Watching the sunset in our backyard
 6th grade trip to the pumpkin patch
 Halloween Princess
 Princess and the Peasant
 Flirty Magoo
 Christmas Elf
 So Bubba grew up! Mom and her babies at Christmas!

There are many things going on with Abby. I didn't want to just launch right into them without first letting everyone know that above everything else she is happy and loved. She is flourishing in Jr Hgh and has made the Principles Honor Roll for the first two quarters!

Another blog soon.

Thursday, October 22, 2015

Things to never say to this Special Needs Mom

Wednesday, September 9, 2015

When a Mom Can't fix it.

As most of you know I took some time off from the world of blogging and social media for the most part to deal with life. Every once in a while it has to be done.

Abby just started the 5th grade. Yes seriously. Our baby is in the 5th grade. Almost unimaginable isn't it? Considering they told me when she was two to take her home and make her comfortable and that she was going to die. To all those uneducated scientists that ruined my life that day

The one thing they did tell me that day that was absolutely true, although it also sent me into a complete rage was,"There's not much we can tell you..You'll have to go on the internet and find other parents with children like yours. Special Needs Communities can be incredible."

I remember at the time thinking that the doctor needed to be hung upside down from the 10th floor by his feet. What a jerk right?

I think he may have prepared  himself before that meeting almost 9 years ago by reading the same paragraph in the back of an Autism book from 1983 that I had when I figured out that Rett Syndrome was probably what she had a few month earlier.

It was the only thing he said that day that was true. I wasn't much of an internet person at the time (It takes me a few years to catch up on any trend. lol

Who I am today and who I was on that day are two incredibly different people. I remember going straight home and doing as I was told as far as not Googling Rett Syndrome or anything about it. Seriously Rett can be such a bummer. Especially since the internet is not regulated and anyone can write anything they want about anything. Like the bathroom wall in Jr High. Back then the idea of talking to strangers, especially at that time made my stomach turn but I gave it a shot.

6 months after I can say I had friends for life. All except one, at that time I had yet to meet. There are those moments in the beginning when you feel so incredibly lost and alone. Having someone to call or turn to makes all the difference in the world in putting your heart and mind back together. I never once had a parent who had been there not take my call, night or day. Not once. It did not take me long to become one of those parents.

Soon after I started my own support group when I saw that all my friends were friends with each other. They also had never met in person. I can truthfully say that I have learned more from Rett Syndrome parents and Special Needs parents than any doctor has every been able to teach me or get me to understand. Having someone say its okay to be angry. To say that you have every right to feel cheated. To have another human being that can finish your sentences on the very first conversation. That happens. Its real.

I guess what I'm trying to say to those of you that are new to this journey....There are people that are out there. There are people that do get it. There is a bond when someone knows your heart and soul the first time they lay eyes on you or hear your voice. Sadly, many times friends and family just don't get it. For a very long time that made me very sad until one day I wondered how I would feel. I'm a fixer. Most Moms and friends are. What do you do when you just cant fix it? No matter how much you would like to? Most pull away and avoid. They avoid their hurt and they avoid your hurt. Most of them are not the soulless jerks they seem to portray. Its a defense mechanism, Fight or flight. Learn to forgive.

If you are new to this there are people out there that get it. They want to help. They do it because they don't want to ever feel as lost as they did when they started this journey. Healing hearts heals hearts....for everyone.

If you are a family that has been in this for a while and holed up and buried yourself because you didn't want to deal with anyone else..that is completely understandable. If you ever need anyone. We are here.

This is how we fix it.
I have many links on the sidebar to the right. If you can't find what you are looking for please feel free to contact me.

Wednesday, December 10, 2014

She called it the Monster

Today we had an early morning IEP for Abby. All IEPs have to be done before school hours as to not interfere   ...I think

We haven't had one since last winter. It was the first one where I ended it without an argument.

One the way there this morning I didn't have my usual, " Don't be an asshole." Talk with myself. I don't have to take deep breaths. I didn't  grip the steering wheel, hands aching by the time I got there. Why? Probably a few reasons. I AM exhausted. I have accepted my child. After probably 10 IEP's. I am more educated and they are more used to me.

This time though...l almost don't have words. It was like there was a team there for the sole reason to come up with an


Like one for my kid and about my kid. Like really what is best for her. Her.

I'm not going to fault any previous staff. People try. People become hardened. I was clueless at first. Then I was angry for the next few. I was fed up for a few. I was not an easy person to deal with. Then I just became tired. So tired. I didn't want to talk about how disabled she is. I didn't want to have group pow wows over it.

Last year was a transition year. The teacher she had for 3 years had retired. I was so defeated. I don't want to start over. I didn't feel like  I had it in me anymore. I had been Ill with Pneumonia. I was in pain all the time from a spinal injury that was yet to be diagnosed. My husband was unemployed...again.

I remember going in for meet the teacher night. I didn't know her. At least I thought I didn't . Turned out she had been at the last IEP.

I went in with Abby's PODD book, a switch, her Tobii and one request. Give her the benefit of the doubt. Look in her eyes and believe in her. She did. From that moment.

At the beginning of the year. The first day actually. I knew this year would be better. A little more hope. Abby's PT from Early Childhood was the new head PT for the district. She believes in her.

She was the one today...for the first time, that said, " She has met her goals. We need to add to it. She more than tripled it. YAAAAHHHHH!!!!

Then she said it. She said it unscripted and without prompting. She said the words us families only know about and say to each other.

She called it the monster.

".... It all depends on how the MONSTER is affecting her that day."

I shocked everyone I think by bursting into tears. She got it. She paid attention. She had been listening and she knew. Not that day. Not today. But all along. Even with 4 years away from Abby. She knew. I almost can't even type my heart is so full.

Someone..not one of us gets that Abby is trapped. That something has her and we can't see it. We can't kick it's ass and it has all the power. The monster that stole her in the night..that's what we call it...dragged kicking and screaming into a world all her own.

And by my reaction to that statement. I poured tears. I couldn't complete a sentence for a few minutes...I'm sure the rest of the team get it now too

There is hope in this world.