Reprieve

Knowing the nature of Rett syndrome, in all its randomness, I always hesitate to say that things are going well.  I am not superstitious, but I realize any relief from difficult symptoms is often temporary.  We Rett parents are always waiting for another shoe to drop.

After almost two years of dealing with regular crying episodes, Abby is putting them behind her, at least for now.  At the worst of it, we were giving her Gravol almost daily to allow her some relief.  Since November, and her strange, temporary inability to stand up or walk, she has not needed to take Gravol at all.  The crying episodes continued, but the severity decreased.  Now, in January, they seem to be a thing of the past.  Let's hope it lasts!

Some may wonder what caused this positive change and we can only guess.  Here are my top 4 guesses:

1. Random Rettness (there is no known explanation)

2. Leveling off of hormones or nervous system changes which accompany adolescence

3. Landing on the optimum dose and type of medication (risperidone, in this case)

4. Arm braces

We started using arm braces regularly with Abby in December.  They are used to restrict her arm movement at the elbow.  The idea is to prevent her from hitting her head or biting her hand during her worst episodes of distress.  But the real benefit is the calming influence they have on her.  Her neurologist explained that they interrupt the negative feedback loop in her nervous system when she starts to become distressed.  They prevent her from becoming more distressed.  They have been so useful that she actually looks relieved when we pull them out to put them on her.

Some people have been asking how Abby's doing with walking now days.  After the problems in November, she was completely back to her usual mobility.  A few days ago, she seemed to resist walking in much the same way.  It is scary to watch, but I pushed her to walk, and she bounced right back on the same day.  I have heard from a few other parents that their girls have temporary periods of not walking and then all mobility returns.

Some of you have also been asking about the Rett research news from the last few months.  My next post will attempt to explain some of that.  It is complicated, but there is some promising progress!  Stay tuned.

Communication Complications

Like most people with Rett syndrome, Abby does not speak or sign.  Understanding her thoughts can be challenging.  In our family, we have learned to read her expressions and signals pretty well, but I spend quite a bit of time teaching others how to communicate with Abby.  

First and foremost, I like to challenge the common assumption that a nonverbal person is cognitively delayed or has other problems with understanding.  Abby's hearing is fine, I know she is intelligent, and as far as I know, she understands everything.  I remind people of that often.  Misunderstanding Abby's receptive abilities can affect the way people interact with her.

Next, I ask people to think about all the things they would want to say if they had to keep their mouths closed and sit on their hands all day.  Without those two abilities, you need to get really creative.  Abby communicates her needs and questions and emotions mostly through facial expressions and eye gaze.

There is a lot of great augmentative communication technology out there - and some of it has proven useful to Abby - but most of it requires some kind of consistent hand function.  Abby does not have the fine motor control to touch small buttons or even to point to things with any accuracy.

Abby is an expert at making choices through eye gaze.  

You can hold two or more things or pictures or words in front of her and she will look at the one she wants.  She is learning to read and write this way.  She has a pen pal.  She is showing the teachers what we already knew.  She is smart and she is very motivated to communicate.  We just need to be ready to listen.  Although I always felt sure that Abby was full of regular thoughts just waiting to be expressed, I'm especially grateful to Dr. Karen Erickson, whose enthusiasm about the learning abilities of nonverbal children has inspired me.

With all we've learned about successfully communicating with Abby, there are still times when there is no substitute for the spoken language (or sign language).  When Abby is sad or sick, we can rarely pinpoint the problem.  She can't make choices until she's finished crying, and even then, it's not enough.  She may be able to tell us her head hurts, but think about all the types of pain you can have in your head. 

A week or two ago, Abby stopped walking.   We spent hours in the hospital and put her through countless tests, but the tests all came back normal.  I was told not to worry, to monitor her.  For over a week, we were put through the ropes of what many Rett families experience every day.  Suddenly, Abby needed to be carried from place to place.  Although she has a stroller, that didn't help her to get on the little yellow school bus or to get in and out of the regular bath tub.  My back became sore and my eyes were opened to the world of non-walkers.  In the back of all our minds was the question, "Will she walk again?"

She is walking again now and we still don't know what went wrong.  She is growing really fast these days and it's possible she pulled a muscle or ligament.  That's diagnosed by asking and answering questions.  Like most people with Rett syndrome, even Abby's expression of pain is unusual, so when the doctors manipulated all her joints and she smiled, we still weren't sure she was fine.

I often say, about parenting, it would all be manageable if your kids didn't get sick.  The same might be said for parenting someone like Abby.  You can get used to a lot of things, but when something's wrong with your child, and she can't tell you what it is, you feel completely helpless.  

I'm thankful my girl is walking again and smiling about it.  And I think of all the Rett families who deal with medical complications every day.  And I think of all the people who want to tell us what they are thinking and are just waiting for someone to understand.