Author: Dany

When we saw an increase in my son’s aggressive behavior during his middle school years, his teacher and I worked long and hard on a detailed schedule to help manage it. We had done a functional behavior assessment and knew the reasons for the behavior — it was his way of communicating that the work was too hard or too much, and the environment was overwhelming.

The focus of this article is the schedule we developed during Ian’s 8th grade, but a variation of it was used throughout high school.

Meet Ian

Ian attended a public middle school of about 350 children. Our district provides special education for children in two ways, based on the level of help they need throughout the school day:

  • Resource: Occasional.
  • Life Skills: Frequent.

Life Skills students either stay in their own class all day or are included in typical classes with aide support. My son was based in a Life Skills classroom and, with the exception of reading and math, was included in typical classes throughout the day.

When we developed my son’s daily schedule, we alternated between gross and fine motor activities. When Ian arrived at school, he would go into the Life Skills classroom before his first-period class and choose a gross motor activity: bouncing on a therapy ball, swinging, rocking, or jumping on a mini-trampoline. A visual schedule for the day was also reviewed with him. Here was his schedule, with each period lasting about 40 minutes:

  • Reading
  • PE
  • Math
  • Lunch
  • Science
  • Cooking
  • Computer

As with most kids with Fragile X syndrome, 40 minutes was a long time for my son to stay on task. Twenty minutes was about the most we could expect. After that, his aggressiveness escalated, so we knew we had to vary each lesson and keep it moving.

We broke reading down into two 20-minute halves. The plan was to have the first half be fine motor/academic and the second half gross motor/sensory. For the first half, Ian had to complete three activities, after which he would get to do a gross motor activity. Our thoughts behind this:

  • Ian understood how many “three” were, and it was not an overwhelming number for him.
  • Teachers could easily enough come up with three activities that could be completed in the 20 minutes.
  • The activities would be chosen ahead of time so Ian knew or had a good idea what they would be.
  • In each activity there would be a combination of familiar and new — so part of it was easy and part was challenging.
  • The gross motor activity would be a motivator.

The crucial consideration was that reading had to be done on a 1:1 basis — not small group. It was often done by an aide, but under the instruction of a teacher. It also had to be done in a quiet room with no distractions, including sights, sounds, smells, and textures. Here is a detailed example of the approach:

  • Big Picture: The aide would state what was going to happen for the whole period, “We are going to do three activities, then we will go to the office to do work.” (Or whatever activity had been decided.)
  • The aide would write the three activities down — preferably on a chalkboard — keeping the wording simple and reading them aloud as she wrote them. We opted for the chalkboard because, as each item was completed, Ian could get up and mark it off, entailing “closure” and a “transition activity.”
  • After each activity, the aide would say something like, “We’re all done with this activity, you can mark it off, and we have two more activities to do.” Then… “We have one more activity to do.”
  • When the three activities had been completed, the aide and Ian decided what activities would occur the next day. Ideally, Ian would pick the first activity (it was best when he had two options acceptable to him), the aide would pick the second, and they would decide on the third activity together. (This gave Ian some control and a good idea of what to expect the next day, which helped reduce his anxiety.)
  • The activities for the second half of the period may be the same every day or vary depending on the school and its programs. For example, the only options available for the first period might be working in the office or library (preferably doing physical activities) or going back to Life Skills to swing or bounce on the ball.
  • The second half of the third period might be best spent setting up the cafeteria for lunch. (He zipped through math.)
  • The second half of the fifth period might entail cleaning up after lunch. Other options might include helping out in other classes, going to a PE class, or doing custodial work. Most middle schools do not have playgrounds, but maybe there is a track — ideal for doing a couple of laps. While some of these activities are not inclusive, I saw it as a necessary phase in order to get past the aggressive behavior. Being suspended is not very inclusive either.

Presenting each activity: Again, start with the big picture, the teacher might say,

  • “The first activity is sight words…”
  • Here she might point to the board where it is written
  • “…and there are 25 of them here.”
  • She shows the stack of cards
  • “We will go through the words — the ones you know will go here…”
  • She points to the left
  • “…the ones you don’t will go here…”
  • She points to the right
  • “…and we will go through that pile one extra time.”

An example of three activities that Ian might do:

  • Sight words: There might be 20 words he knew really well, 20 he mostly knew, and 10 he was working on.
  • Letter/sound association: We used sight words to “back” into phonics, rather than vice versa.
  • Read a chapter of a book: At whatever level is familiar, yet still challenging.

After a week of this, Ian knew the routine. We then repeated the program with math, computer, and science. He did not need anything like this for PE or cooking (play and food … hmmm 🤔).

The day we implemented this schedule, the aggressive behaviors dropped substantially. Because he was working 1:1, it was much more relaxed (we all know that’s a good thing), and the aide could work on helping him vocalize his thoughts and feelings. If he was struggling with work, she might model what to say, such as “This is hard,” or “I need help.” And when she taught Ian to say, “I need a break,” it was honored, and strategies were developed to help him take the break. “Why don’t you get a drink of water, and then we can finish the words?” the aide might say.

The middle school years can become challenging with that puberty thing going on. Add in anxiety, limited speech, unreasonable expectations, and an environment over which students have little control, and you have a recipe for disaster/suspension (in this case, they are synonymous). While Ian had been doing a simple version of this program throughout elementary school, we had to kick it up a notch for middle school.

So if you are having similar problems, take a step back — see what’s working and what’s not. If it is working, keep doing it or do more of it. If it is not, work with the school (!) to try new ideas.

My whole marriage to my husband Vince can be described by one acronym: F-X-T-A-S. Our wedding was in April 1999. It was a second marriage for us both, and we weren’t youngsters (I was 48 and Vince was 64), but I thought we were both healthy. We had no inkling that Vince’s mind was already deteriorating from FXTAS.

Vince’s first blatant physical symptom appeared a month after our wedding when he fell down while playing golf, without knowing why. He occasionally fell again in the ensuing months when exerting himself, and gradually started having trouble moving his feet to walk.

The cognitive symptoms had actually started taking hold of Vince’s brain a few years before our marriage, but this only became evident to me in retrospect many years later. I had never known anyone with a cognitive illness, so that possibility never occurred to me when I saw Vince gradually losing interest in his work, having small auto accidents, or having occasional irrational anger outbursts. At least he didn’t forget to show up at our wedding!

We began married life in Vince’s house, which resembled the ramshackle junkyard on TV’s “Sanford & Son.” He had promised to fix it up, but it never happened. His daughter eventually helped me make the house livable.

Vince once screamed at me for trying to throw away an old plastic cereal bowl, and I cried. I did lots of crying in those first years, and often felt terrified at the situation I had gotten into. My gentle, loving husband just wasn’t the same person — he would lapse into episodes of apathy and withdrawal from me. I would try to talk to him and he would stare at the TV and not answer. Still not thinking of dementia, I was afraid that I had been misled about who he really was.

Meanwhile, other strange things were happening. I began noticing that my brilliant PhD laser scientist husband was losing track of his finances — we almost had our water turned off and our homeowners’ insurance canceled because Vince hadn’t paid the bills. He was buying things he had no use for from telemarketers and making crazy investments with con men that caused us huge losses.

I started taking Vince to neurologists in 2001. His brain MRI report had several comments indicating abnormalities, but it would take over two more years of exhaustive tests, incorrect diagnoses, useless attempts at physical therapy, and five neurologists before the FXTAS diagnosis in 2004. We were both greatly relieved to get a diagnosis, and this being a newly discovered condition, we had no idea of the challenges that lay ahead. In a way, I was glad that Vince had something “new,” because it wasn’t an immediate death warrant; maybe I could do my research and be creative about managing the illness.

I like to think that I saved Vince’s life and he saved my soul. I found Drs. Randi and Paul Hagerman on the internet and contacted them. By the time Vince was diagnosed with FXTAS, he was too disabled to travel across the country to take part in their studies at the UC Davis MIND Institute, but I sent them his MRIs and a blood sample for their research. They recommended medications, which Vince’s doctors prescribed. I also became acquainted with MIND’s Louise Gane, the kind and caring genetic counselor — I had called her in a panic when I realized that Vince didn’t even remember my name! She gently confirmed the dementia I hadn’t recognized.

As Vince’s balance and walking continued to worsen, bladder and bowel incontinence joined the fray. But it was his mental decline that was by far the hardest aspect of FXTAS for me to accept. As I saw him get confused about where he was (he often thought our house was a hotel), who I was (he often thought I was his daughter), and lose his ability to write, speak, or have any emotional responses (blank expression), I did not know how I could ever cope. I was rapidly losing my husband’s whole persona, and I was inconsolable.

There was nothing in this world that could replace Vince’s mind — except, that is, for the relationship I began to develop with a God whom I had never believed in. Vince’s church reached out to me; his priest visited and spoke with me and gave me books which drew me into a conversion to the faith. As I felt God hold me up and answer prayers, I began to have some confidence that I could manage this life with God’s help. I also found the Well Spouse Association, a support organization for spousal caregivers that brought me life-saving friendships with people who were entrenched in situations similar to mine.

As turbulent as everything was, I was determined to keep Vince at home and care for him in the best way possible. I kept my resolve to do my research and harness whatever resources I could find. That included a nutritionist for dietary recommendations, handicap modifications to the house, whatever physical and speech therapy Medicare would provide, and home health aides.

It took about a year (2005-06) of trial and lots of error to find a nursing agency that could provide the right help. I finally obtained a strong young man who is still with us most every day. Another aide comes in the evenings. I can handle Vince by myself overnight and for a few hours in the late afternoon.

When Vince’s decline got to the point where I felt everything was lost, my attitude changed to thankfulness for what was still left. For several years, he was able to tell me he loved me, and he often did. Now, I’m just thankful for any random word he may say or any step he takes (he is still able to take some walking steps with help from the aide and me).

Since becoming completely disabled — unable to do anything for himself, including feeding — Vince has been very peaceful and content, and as of today, he is still generally healthy, aside from the FXTAS brain damage. He is not in any pain, and seems to have reached a plateau a few years ago, where things have remained more or less stable. He has days when he looks relatively alert, and days when he sleeps more. He spends the days in front of the TV, sometimes looking at newspapers or magazines, and sometimes playing with put-together toys. He looks great for a very disabled 79-year-old! He also maintains a steel-like strength — probably helped by the testosterone injections I give him twice a month.

Terri with her husband, Vince.

The whole experience of our FXTAS marriage has been extremely difficult for me — physically, mentally, and emotionally. But with God’s help, I learned that I could get through this ordeal one day at a time, establishing priorities and respecting that I was doing enough by doing my best.

As long as I have my aides, we have a workable daily routine, and I’ve been able to get out most days to shop, see friends, and take on many volunteer responsibilities for Well Spouse.

I try to take care of myself with a decent diet, exercise (workouts at home), and time here and there to read or sing with my piano. Life in general is a little calmer for me now than in earlier years when everything was coming at me at once — major house repairs, financial and legal knots to untie, and frequent calls to the rescue squad to pick Vince up when he fell. My greatest challenge now is having to purée Vince’s food because of the chewing and swallowing problems he developed a few years ago. I spend a good part of every day cooking, puréeing, and washing blenders and pots, but Vince has done well eating my culinary “creations.”

There is no part of our almost-15-year marriage that FXTAS has not affected. I know we still love each other, and every day with Vince is one less day without him in my life. He doesn’t speak to me, but I tell him everything that’s on my mind, and he listens. And I can still feel comforted by his strong, quiet presence beside me.

More than anything, I’ve wanted our marriage to have some kind of transcendent meaning, since it never was and never will be normal. I like to think that I saved Vince’s life and he saved my soul: I have preserved the best quality of life he can have, and his FXTAS has challenged me to grow spiritually and in so many other ways. We do have a special marriage.

“Jeff, what did you do at school today?”

“Did Mrs. Miller give you any homework?”

“Where’s your lunchbox?”

“Did you talk nicely in speech therapy?”

Does any of the above sound familiar? To us, it sounds like the typical series of questions parents ask their children at the end of most every school day.

How does your child respond to these questions? Children with Fragile X syndrome will most likely not respond to them directly, but may instead respond with one of the following:

  • Avoid all eye contact and walk away.
  • Start talking about their favorite cooking show on TV.
  • Answer the last question asked with a simple yes or no.
  • Repeat the most relevant word in the last question, “Speech? Speech therapy, not today … not going!”

Parents usually ask their children questions to get information or to simply engage them in conversation. In the case of a child with FXS, asking direct questions is the least likely way to accomplish either of these goals.

Interestingly, it’s not that different at Developmental FX, where the end of the day often sounds like this:

“Mouse, where are you going? Are you leaving early? Are you going to work on your garden? Can you get me that report before you leave?”

It turns out that direct questions don’t work well with Mouse either!

Direct questions are typically the way we try to engage people in conversation. For children with FXS, we need to learn other strategies. It is critical to understand why it is so difficult for children with FXS to understand and respond to these questions, before we get to the how of getting answers.

Why It’s So Difficult

It has been well documented that individuals with FXS often experience a hyperarousal response to direct social interaction. (Hyperarousal is the clinical term for: Get all flustered and probably red in the face.)

In addition, their difficulties with working memory make it difficult for them to hold the questions in mind long enough to consider them, formulate a response, and act on that response. Combine these two conditions and you have the perfect scenario for not answering a direct question!

So then, how do we find out where Mouse is going at the end of the day, or if Mrs. Miller gave your child any homework?

How to Get Answers

These tried-and-true strategies provide the how for getting information and fostering simple conversation.

  1. Use a fill-in-the-blank (cloze) format. As you verbalize the following short phrase, your voice should be leading and expectant: “Your lunchbox is _______”
  2. Make statements … not questions. Talk about what your child is doing or has just done. Be the narrator for your child’s life events. For example, when driving home from school together, you could remark, “You brought home some papers today,” or “You have a smile on your face, what a great day you must have had,” or “I can’t wait to hear about your trip to the zoo!”
  3. Use self-commenting about your day as a model for your child to follow: “I’m so excited about my day! I saw some puppies at the park, and I ate my lunch with a friend.”
  4. Soften your gaze when attempting an interaction in order to minimize hyperarousal.
  5. Use a physical prop or visual aid to support the context of your comments. For example, hold up the visual schedule of the day and make a comment while pointing to a particular picture on the schedule. While emptying your child’s backpack, hold up a specific item (Tupperware container) and remark, “Oh, your sandwich must’ve been deeelishhh today!”
  6. Movement activities will help facilitate language expression. If you want to have a conversation, try it while going for a walk, jumping on the trampoline, or while swinging at the park. This is one of the reasons we often pair occupational and speech therapy, because movement promotes talking!
  7. Remember: Do not try to have a conversation or get information during a transition. This is the hardest time to establish a flow of language. Rather, use routines to ease the transition, then after you are settled in, use one or a combination of the strategies listed to spark conversation.

Have these suggestions been helpful? How will you use them? Will you share them with your school? Will they work with Mouse?

Oops! Too many direct questions. Instead, we’ll make a comment.

These great suggestions could be used in a variety of situations. We hope they are helpful and that you’ll share them with _______!

This is an archived page referring to the Seaside Arbaclofen (STX209) clinical trial, which was terminated in 2013. Learn more.

To our Fragile X family:

Many of you by now have heard the news that Seaside Therapeutics has decided to end their extension of the trial medication known as Arbaclofen (STX209). The NFXF Board of Directors and all those within the National Fragile X Foundation share your heartfelt concern of the impact this decision will have on your family or of those you may know.

While this may be a difficult time for many, it is important for us to remember there has never been more of a reason to remain optimistic. There are many new medication targets under investigation and researchers are continuing their ground-breaking work toward translating these potential treatments to individuals with Fragile X syndrome in the future. In addition, at this time, we are still aware that Seaside plans to pursue FDA approval pending the results of the placebo-controlled studies.

We understand that you still have many questions. To provide you with accurate and timely information, we have developed some Frequently Asked Questions and Answers [no longer available] to help you interpret what has happened and make the best choices for your loved ones.

There are many misconceptions about Seaside’s decision circulating on social media and we encourage everyone to consult their physicians or study coordinator to discuss further options.

As more information becomes available, we will update this site. We will also initiate discussions with relevant parties and gather appropriate data to keep everyone properly informed. [No further updates.]

Until more is known, please review the FAQs [no longer available]. We have also created a comments section where you can share your thoughts and concerns with us. We will continue to address the most frequently asked questions moving forward.

Forward,

Brad Whitus
President
[then] NFXF Board of Directors

Robert Miller
[then] NFXF Executive Director