Dynavox Maestro - Seven months later

When the Dynavox representative came out for the training, the most valuable item she provided was a programming cheat sheet. It made it very easy and quick for our speech therapist and I to program the Maestro in the way that we wanted to for V.
At first, V did not want to use the device for lessons, but understood that the purpose of the device was to communicate. She started using it right away to choose what to eat for breakfast. Then she began to use it to indicate where she wanted to go in the community, and more specifically, what she wanted to do at that location. For example, if she chooses the library as her destination, she can indicate if she wants to look for a movie, a book or look at magazines. She tells us if she would rather go to Chick-fil-A or Chipotle Grill for lunch and then she indicates her preferred menu items with the device. At home she can choose her leisure activity with the Maestro or tell us that she wants to rest.
We have to do a lot of modeling to encourage her to use the device as opposed to the gestures or actions she has used in the past to indicate her preferences. However, the speech therapist said that she learned to navigate around the pages in the device to find what she wants to say much more quickly than she had anticipated. V had learned as much in two months with the Maestro as the SLP had expected her to learn in a year!
As V masters the choices that have been programmed for her, we continue to add to her choices and to allow the complexity of her communications to grow. She is now willing to use the device during speech sessions with her therapist.
I am very happy with V's progress on the Dynavox Maestro. It has given her the ability to express her wishes in ways that she was never able to do before. I look forward to the time that she is able to better express her feelings with the device, both physical and emotional. 

Bone Marrow Transplant as Treatment for Rett Syndrome

Here is a new approach to treating an autism spectrum disorder that I have not heard of before. It will be interesting to see how broad the implications are for treatment of other ASD's as the research progresses. Bone Marrow Transplant Arrests Symptoms in Model of Rett Syndrome ScienceDaily (Mar. 18, 2012) — A paper published online March 18 in Nature describes the results of using bone marrow transplant (BMT) to replace faulty immune system cells in models of Rett Syndrome. The procedure arrested many severe symptoms of the childhood disorder, including abnormal breathing and movement, and significantly extended the lifespan of Rett mouse models. Exploring the function of microglia deficient in methyl-CpG binding protein 2 (Mecp2), the protein encoded by the "Rett gene," principal investigator Jonathan Kipnis, Ph.D. and his team at the University of Virginia School of Medicine uncovered a completely novel approach to this devastating neurological syndrome. The work was funded by the Rett Syndrome Research Trust and the Rett Syndrome Research Trust UK. See Also: Health & Medicine Leukemia Osteoporosis Children's Health Immune System Stem Cells Chronic Illness Reference Rett syndrome Autistic spectrum Sex linkage Autism Rett Syndrome, the most physically disabling of the autism spectrum disorders, is caused by random mutations in the gene MECP2. Predominantly affecting girls, symptoms usually manifest between 6 and 18 months of age, when a frightening regression begins. Children lose acquired language skills and functional hand use; movement deteriorates as other Rett symptoms appear. These may include disordered breathing, Parkinsonian tremors, severe anxiety, seizures, digestive and circulatory problems and a range of autonomic nervous system and orthopedic abnormalities Although most children survive to adulthood, many are wheelchair-bound, rely on feeding tubes, are unable to communicate and require total, lifelong care. Kipnis was drawn to Rett Syndrome from his perspective as a neuroimmunologist. "What began as intellectual curiosity," he explains, "has become an intense personal commitment to studying the correlation between neurological function and the immune system in Rett Syndrome. The impact of BMT on so many different symptoms has triggered a flood of experiments we are now pursuing at full speed." The brain is largely composed of several types of glial cells, which have diverse and complex functions that include sustaining a healthy environment for neuronal growth and maintenance. Microglia are small glial cells that participate in the brain's immune response. One of their roles is to clean up normal cellular debris in the brain through the process of phagocytosis. Kipnis and his team discovered that when microglia lack properly functioning Mecp2, they are unable to perform this crucial duty efficiently. Because microglia are derived from immune progenitor cells, it is possible to replace them via a bone marrow transplant. First author Noël Derecki and his colleagues began their work with male Rett mouse models, which lack any Mecp2. These Mecp2-null mice mimic the human disorder, with neurological symptoms beginning to appear at about 4 weeks of age and an approximate life expectancy of only 8 weeks. Radiation treatment was administered at 4 weeks, followed by a bone marrow transplant from normal (wild-type) mice. As engraftment -- the migration and repopulation of new microglia -- took place, the Rett mice began to grow instead of fail. Body and brain sizes approached those of wild-type mice, gait improved and mobility increased significantly. There were no signs of the severe tremors seen in untreated mice. Apneas and other breathing irregularities were markedly diminished. The oldest of these mice is now almost a year. Work with female Rett mouse models at more advanced stages of disease is currently underway. Gail Mandel, Ph.D., whose Rett research focuses on astrocytes, another type of glial cell impaired by mutations in MECP2, comments, "A fascinating aspect of these findings is the data suggesting that deficits in the engulfing properties of microglia are a crucial aspect of Rett neuropathology. It will now be necessary to develop cellular assays to determine all the ways these immune cells are bolstering neuronal functions and whether they can be therapeutically harnessed." Dr. Mandel is a Senior Scientist at the Vollum Institute and a Professor in the Department of Biochemistry and Molecular Biology in the School of Medicine at Oregon Health & Science University and an Investigator of the Howard Hughes Medical Institute. Monica Coenraads, Executive Director of the Rett Syndrome Research Trust added, "I've been in almost daily contact with Dr. Kipnis since he brought his very original proposal to RSRT, and have been amazed to see the development of this brutal disease stopped in its tracks. A question that arises, of course, is whether replacing faulty immune cells with healthy microglia through bone marrow transplant would arrest or ameliorate already existing symptoms in humans. We do know of a case in which a girl with Rett Syndrome who was treated for leukemia gained considerable communication skills after a bone marrow transplant, and was able to converse with her mother for the first time in their lives. RSRT is in the process of exploring bone marrow transplant as a treatment modality, with full awareness of the serious nature of such a trial. In research, we are risk-takers. In clinical application, we are conservative and will be examining this carefully as more information emerges." Adds Noël Derecki, "Our encouraging results point to how surprisingly tractable this severe disorder proves to be, at least in the lab. We are currently exploring how bone marrow transplantation might affect Rett symptoms once they have become more advanced, and whether there are other effective ways of modulating immune responses and subsequent effects in the central nervous system."

Technology Can Save a Life

One of the worst fears of any parent is that their child may go missing one day. That fear is amplified when the child is ambulatory, unable to understand danger, and is non-verbal. This article discusses one way that parents can address those concerns. "Unique Piece of Technology Could Save Lives By: Hollie Hojek hhojek@kcautv.com Trey Michaels is your average 8-year old boy, who loves movies, computer games and his IPad. But unlike many other kids his age, he doesn't just enjoy unique technology, he wears it. It's called a life savor bracelet. It's used to help find someone in case they runaway or get lost. And that's exactly what happened to Trey Michaels about two years ago, when he wandered off in a shoe store. Although he was found, just minutes later, Trey's parents decided they weren't going to take another chance, especially when it came to their son, with autism. "You hear about those kids who run away... And we just think thank goodness we have this bracelet for Trey because you just never know," said Trey's mom, Shana Michaels. Twenty-four hours a day, Trey wears the bracelet around his ankle. It's fitted with a personalized tracking device, and batteries that they check every morning. Now, if Trey were to runaway, all his parents need to do is call 911 and local authorities immediately begin tracking his whereabouts. "If he were at school surrounded by fields... Or daycare... Right by the street and take off in any direction he could be gone.," said Michaels. Not only that, with his autism, Trey doesn't always have the ability to speak. We could be calling for calling, and he wouldn't answer us, or he could be injured somewhere and he might not make a sound," said Michaels. But with this bracelet, he's got a little bit of a safety net, and a little less worry from mom and dad. "Before without the bracelet, if Trey were to runaway... Whew... I'd probably be a ten on a scale of ten, but now with the bracelet, I'd probably be an eight, we'd still be very concerned, but at least we know there's some steps we can take to be proactive.," said Trey's dad Tony. And this little piece of plastic is one of them, no matter how many more close calls Trey runs into. For those involved in Project Lifesaver, they are having a training session on November 16th and 17th." If you would like to look into this further, go to http://www.projectlifesaver.org/ If you are in my local area, Montgomery County, MD, then this is what appears on our county page: Divisions/Stations — Project Lifesaver PROJECT Providing a rapid response to missing persons and peace of mind to caregivers and their families. LIFESAVER Home | Application Process | FAQ | Useful Links | News/Resources | Contact Us Project Lifesaver is a proactive response by the Montgomery County Department of Police to identify those individuals within the community who have a propensity to wander and not be able to return home. Caregivers of individuals that have a propensity to wander due to a diagnosed medical condition such as Alzheimer's, Autism, or Down Syndrome are provided with information and tools to prevent episodes of wandering. If necessary, the individual may be outfitted with a trackable RFID bracelet. Continue to program description

Patience

Last week we had a home visit from Natalie, Val's trainer at Fitness for Health. With Val's Suburban in the shop for repairs, I was nervous to take her 30 minutes each way in my little car in case she became agitated during the trip. Although we had to pay for the travel time for Natalie, it was an opportunity to work on Valerie's tricycle riding skills. It was a beautiful day for November and perfect for doing an outdoor activity. We got the trike out of the garage and took it across the street to a protected side road that gets very little traffic. Val walked out of the house nicely and willingly got on the trike. She steered as we pushed, and all went well until we came to the part of the road where a noisy truck was parked. Something about that truck and the sound it made, upset Valerie and she got off the trike and began to have a tantrum in the street. I went back to our house to get my car and drove it to where Valerie was sitting in the street, which motivated her to get up and climb inside my car. I drove back into my garage, closed the garage door and smoothly transitioned Valerie back to her room for indoor recreational activities. Afterward, Natalie and I chatted about how things had gone with our first attempt at tricycle riding. She was happy that Valerie got on the bike and had stayed on it for several minutes until the distraction of the truck. Valerie loves working with Natalie because Natalie is happy for whatever Valerie is willing to give to an activity. Natalie respects Valerie's feelings, interests and limitations on any given day. Some people respond to being pushed, but Valerie pushes back. Everything is on her terms, at her pace, and nothing can be forced without negative consequences to all concerned. I agreed with Natalie, that it was a positive start, and keeping Valerie's experience with Natalie a positive one is critical to her continued willing cooperation during future sessions. I have learned to expect progress in any area of Valerie's learning to take several months, if not years, of work on a weekly basis. Those people who have been successful in their work with Valerie have had to have faith in Valerie and themselves. Those who are in a hurry to get results and who want to be in charge become discouraged before they see what she is capable of. It took over a year to get Valerie to stay on a horse for more than a few minutes during therapeutic riding sessions. It took about 5 months for our speech therapist to get Valerie to want to work with her for a full hour most sessions, and it took about the same length of time for the tutor to get her engaged in work for most of an hour long session with hardly any behavior outbursts. Valerie is kind of contrary, like her mom. When she understands that no one is going to force her to do more than she wants to do, then she becomes more willing to participate for longer periods of time. She becomes happy to see a therapist who respects her wishes, lets her take a break or move on to another activity if she wants to stop. Then the the therapist, staff and I begin to see what she can really do, and we see skills grow. Building rapport and trust first is critical to making progress with Valerie. Keeping things low-pressure and relaxed is important to making progress with her. If Valerie is using all her energy to fight for control, she isn't learning anything. Natalie said, "I wish you could talk to some of our parents at Fitness for Health. Some ask me what progress was made after one session. I was just happy that the child was focused during that session." She and I understand that progress takes time and patience when working with a person who has special needs. She is a perfect trainer for Valerie because she appreciates what Valerie gives to each session, and if that is only 30 minutes of work without any inappropriate behaviors, even though the session is for one hour, so be it. Valerie leaves happily, and is that much more willing to come back again the next week and work for 40 minutes because it was fun last time. I would say to parents who expect quick results, that it will not happen on your time schedule but it will happen if you are patient, accepting, and respectful of your child. A therapist can give your child what they need to move forward if you take away the pressure for measurable results in a specific period of time. Take the pressure off of your child and the therapist and let it take as long as it takes. I came close to giving up Val's sessions at Fitness for Health before Natalie became her trainer. Valerie was spending most of her sessions there fighting with other trainers and her support staff to leave the place almost as soon as she arrived. It had become a negative experience for her and at times she wouldn't even leave the car to go inside when staff arrived with her. Then there was fire in the building where the sessions took place. The sessions were cancelled for about 7 weeks as a result of repairs. When she returned, it was after a home visit by a new trainer, and the facility had moved upstairs and had a different layout. We had some tantrums during the first couple of sessions with Natalie, one really rough one, but things improved really quickly and our last session at the facility was 50 minutes of participation with no behavior problems at all. We started slowly, with only once session a week, but now things are going so well that I am ready to increase her schedule back to two sessions a week, which she had before the fire. I am thankful to the therapists and trainers who have worked with Valerie when they did not see progress, but believed that Valerie had it in her and believed in their own ability to find a way to bring out her potential. They didn't give up or become discouraged and it paid off for them and for her.

My Daughter Addressed Me!

Last night, sitting together on the family room sofa, and watching a little nature show, my daughter turned to me and said,"Mom, shish?" The "shish" is her approximation for goldfish crackers. She wanted some. The first word surprised me and my husband. She has said "Mom" for years, usually to someone else, to ask to see me. "Mom?" has always been a request that meant "I want Mom." But, last night, she said my name to me, to get my attention, so I would know she was talking to me, and that is new. I can't remember another time that my daughter has addressed anyone, including me. I don't know if this is the result of her communication growth through speech therapy, or just a one-time thing that will never be repeated. I liked it, and I hope it happens again. It is a small thing, but it meant a lot to me.

Follow Up to Communicating with First Responders

I called Frederick Memorial Hospital yesterday to find out which ambulance was responsible for transporting my daughter. With their help, I called the number that they provided and left a message for someone to call me back to discuss what happened. My goal was to determine what went wrong, and what if anything I could do to prevent an EMT from overriding my authority in the future. I received a callback last night from a man who supervises the EMT supervisors, and we had a very helpful discussion to both of us. For my part, he suggested that I ask to have an EMT supervisor involved if I am ever in a situation again where an EMT will not listen to me. He agreed with me that the EMT's did not have to transport my daughter to the hospital if she showed no signs of injury, just because she was not competent to decline transport. He told me that if I were on the scene of the accident, I would not be asked to show ID, and so the issue of proving my identity should not have been a big concern over the phone. He told me that someone had asked to provide training regarding care of patients with autism to the EMTs under his authority recently, and that he would follow up and make sure that his EMT's received that training. In addition, he planned to issue a memo to his EMTs and supervisors regarding appropriate actions in future situations like ours. As I suspected, sometimes EMT's get in trouble for using their own common sense and become a bit gunshy about doing so as a result. That doesn't mean that there is not a way to get support for them and for patients when there is a difference of opinion about what is the correct course of action.

Communicating with First Responders

A week ago, my daughter was in an automobile accident on her way to the zoo. She and her staff did not appear to be injured, according to the report by the staff member who called to inform me of the accident. The police were not on the scene, and there was another automobile involved, so I called the police from home to report the accident and get help on the scene as soon as possible. Shortly after, my staff called me back and told me that the EMT's wanted to transport my daughter to the hospital to be examined because she could not respond to questions regarding her well-being. I determined that my daughter was not upset or crying, or indicating distress in any way. The airbags in the car had not deployed, and my daughter was seated in her restraint harness in the third row of our Suburban, while the staff had been located in the first and second rows and were fine. I asked to speak to the EMT, and asked him to allow my daughter to remain in the vehicle with her staff until I could pick them up. He said that he would have to take my daughter to the hospital because her staff did not have power of attorney to make decisions regarding her care, and because he could not verify that I was who I claimed to be. I told the EMT where to find the First Responder form that I had ordered from the autism society, and that was located in the fanny pack that always travels with my daughter and her staff. The form lists me as parent and legal guardian, and provides my contact information. I suggested that he call me back on the phone number listed on the form to verify who I was, but he would not listen. I explained that he would increase the chances of an aggressive tantrum that could result in injury to my daughter or others if he restrained her in an ambulance and that she was safer in her own car in her safety harness with familiar staff. I was only a 25 minute drive away from the scene of the accident. He refused to follow my instructions and insisted that my daughter and her staff leave the vehicle and travel in the ambulance to the nearest hospital and threatened to leave the staff behind if they did not come immediately. They were not permitted time to return to the car for my daughter's communication device, and had to leave it behind to be retrieved later. Ironically, when I arrived at the hospital, the nurse allowed me to refuse medical care for my daughter and to take her home without being required to show any proof of my identity or relationship to my daughter. She was, and is, fine. If I leave town, I always leave my daughter's staff written permission to make medical decisions for her in the event that I can not be reached. I am hesitant to do that when I am nearby. The First Responder form that is always with my daughter should have allowed anyone on the scene to call the phone number provided to assure them of my identity. How would I even know that the form existed and where to find it if I did not put it there? I think that these first responders put my daughter at greater risk by refusing to use simple common sense. I am not sure what the solution to this problem is. I will write a follow up post when I figure it out. Feedback from anyone who is familiar with this problem would be welcome. I really thought I had adequately prepared for emergencies by ordering and completing the form that is offered by the autism society, but when it was actually needed, it did not help.