Tuesday, November 19, 2013

The Essence of Human Life

This is a reading list Lu made up using her Tobii the other day. I have been working at making pages on the Tobii of the covers of all of the books she owns (which is many) so she could choose what she wants me to read. We then read the paper version of the books because they are not in her Tobii (yet!). I have dreamed of this being a possibility for all of her life. Right now I have 7 pages of books with 20 books on each page and I asked Lu to go through and choose two from each page that she wants to read at some point. These were her choices. She almost always chooses the Avengers, and the C Book, and How The Grinch Stole Christmas. I never knew those were some of her favorite books, but I was always just dying to know! 

"It is the position of the American Speech-Language-Hearing Association (ASHA) that 'communication is the essence of human life and that all people have the right to communicate to the fullest extent possible. No individuals should be denied this right, irrespective of the type and/or severity of communication, linguistic, social, cognitive, motor, sensory, perceptual, and/or other disability(ies) they may present.' "

Linda Burkhart used the above quote at the end of the PODD training we went to last December. It has stayed in my brain ever since, but most especially the little part that says, "Communication is the essence of human life." I even used the quote in one of the appeal letters I sent to the insurance company as I pleaded for them to approve the Tobii. When we learned the PODD a year ago, that was the beginning of our glimpse into what Lu was thinking and feeling. When we got the Tobii a few months ago, it made me realize that with the ability to relay her thoughts so quickly and efficiently, people will see her as a real person. Because, being able to communicate truly is the essence of human life.  I believe it is a sad, but true fact that without the ability to communicate, people are seen as "less than", even if they have all kinds of thoughts and feelings swirling around inside of them. 

 Around the same time that we got the Tobii, maybe even a little bit before, I looked at Lucy one day and had a startling, but exciting realization. I looked at her and thought, "How could someone not fall in love with her some day? Why couldn't she get married and have a life with someone?" Then I read a little about Stephen Hawking as a comparison for how I think that could be a possibility... When he married his first wife, the ALS was just beginning to be an issue for him. He had recently received the diagnosis and she still married him. She took care of him a lot throughout their marriage, but they also always had nurses, and assistants that did a lot of the physical caring for him. It was just their way of life to have extra people with them to tend to Stephen's needs. Him and his first wife eventually divorced after many years together and after having three children. He remarried one of his nurses. He remarried even though at this point ALS had completely taken over his body and had done so years ago. BUT, just like Lu, his mind remains in tact. A woman fell in love with HIM and married him, in spite of the fact that he has no control over his body, in spite of the fact that he communicates using a speech generating device, and in spite of the fact that all of his very serious medical conditions require round the clock care by her and others.  I think Lu is so incredibly awesome and amazing that I believe she too will meet someone someday who also will be able to look past Rett Syndrome and fall in love with HER. 

While out to eat at Red Lobster on Saturday night with Chad's dad and stepmom, Lucy kept saying, "panties" with her Tobii. It was funny, but it also gave us the opportunity to talk about when and where it is and is not ok to talk about bathroom things. That's what you do with children; you teach them manners and about topics that are better left for discussion at home, in private. And now Chad and I get to do that too.  I wish every person on earth that cannot speak on their own was just automatically given a Tobii. There are many families of girls with Rett Syndrome that have no communication system for their daughters, let alone a Tobii or some other kind of speech generating device.  While I am so grateful that Lu is able to have her Tobii, it also breaks my heart that there are so many people that do not have one and have not been given the opportunity to communicate. 

This morning Lu and I talked a little bit about what she might be when she grows up...because she will be SOMETHING! With or without a cure, she will do something wonderful, and maybe be someone's wonderful wife. And contribute to society just like others do. I just know it.

"Obviously, because of my disability, I need assistance. But I have always tried to overcome the limitations of my condition and lead as full a life as possible. I have traveled the world, from the Antarctic to zero gravity."
-Stephen Hawking

"All my adult life people have been helping me."
-Stephen Hawking

"Among physicists, I'm respected I hope."
-Stephen Hawking

Saturday, November 16, 2013

29 pounds

This is the fourth blog post I have started since November 5th and I just can't seem to get one finished. I'll try to finish this one. Last year at this time Lu weighed 34 pounds. In April she had dropped down to 31-32 pounds. In August, when we went to New York for the EEG, she weighed 30 pounds. In the past month she has lost yet another pound. Her mouth, and tongue, and throat are just not working very well for her anymore. It took her an hour and 15 minutes to eat a very small slice of banana nut bread the other day. But at least she ate it that day. There are many days that she just eats very little solid food and is getting a lot of her calories through liquids in her bottle. She drinks 2-3 Pediasures a day and I have been experimenting with a new blender we got called a Blendtec to make her juices and smoothies high in calories. I load things up with butter, peanut butter, cream cheese, finely chopped nuts, coconut oil, olive oil...whatever I read about that adds extra, but relatively nutritious calories.  Many things Lu eats these days such as PB&J sandwiches, banana bread, and cereal bars all need to be dipped in milk and yesterday I bought a container of heavy cream to use instead of whole milk. But nothing is working. She is not maintaining her weight.

Since school started at the beginning of September she has had three colds. Luckily they don't get worse than a cold, but when Lucy has a cold, this means that she gags and coughs on snot all day and night and often throws up when it gets to be too much, so it is exhausting for all of us. I have started giving her vitamin c and zinc supplements, but she still got another cold. And when she is sick she eats even less. I know kids get sick a lot when they first start school, but I also think that Lucy's lack of adequate calories and nutrition is making it worse.

Yes, she has gotten taller in the past year, but when people get bigger, they should gain weight, not lose it. Children should not lose weight. Dr. Sasha said in New York when we were there that it wasn't going to get better. She is an expert and knows that as a fact. Unfortunately, none of the well-meaning "Well it could be X, Y, or Z" statements that people come up with to try and ease my fears or explain it away are just not accurate. The progression of this particular problem in the realm of Rett Syndrome is that it just won't get better. It is the apraxia that arrests her entire body that causes the problem. Her brain cannot tell her mouth and tongue, and throat what to do. This causes her to choke and gag often throughout meals and when she is drinking. This causes concern for aspirating which could then lead to aspiration pneumonia. The International Rett Syndrome Foundation sends out a newsletter quarterly and at the end is always a memorial section that Chad and I stupidly, but compulsively read. 90% of the cause of those girls' deaths tends to be from complications with pneumonia and respiratory issues. 

After keeping a weight and eating log for two weeks, I determined that the majority of the time, Lucy weighed 29 pounds. The log took place in between colds where she was eating more than when she is sick. She coughs, chokes, or gags on something daily.  She is not even able to eat things like applesauce, pudding, and yogurt consistently anymore as she often gags as soon as I put a bite in her mouth. The smooth texture seems to really bother her all of the sudden.  We struggle every day to figure out what to feed her.  Somehow she still very successfully eats string cheese. I think it quickly gets nice and mushy in her mouth. She loves tuna sandwiches and last night she was able to eat about a quarter of a cheeseburger from McDonalds which she requests with her Tobii all of the time. 

So, last weekend I emailed Dr. Sasha and the GI doctor we see in New York and shared my log with them as well as a ton more information and said Chad and I think it is time for the feeding tube. They agreed. I am going to talk to Dr. Loizides, the GI doctor on the phone some time this coming week to go over whatever we need to discuss with each other and we will see what the next step is. 

It is heartbreaking. However, I think that once we get past the initial adjustment period, it will improve Lu's quality of life greatly. With more calories and nutrients she will be healthier, have more energy, and have a full belly. We will have huge chunks of the day freed up to do other things besides try to get calories into her. I spend a great deal of time each day feeding her and giving her bottles. And, she will still be able to eat things that she enjoys, by mouth, whenever she feels like it, without the stress of trying to get all of the calories she needs by mouth. 

So, that's where we are with that. Sad, but true.

This is a picture from yesterday. 

This is from last winter. She is still wearing those clothes she has on in the picture, and they just hang on her.