PWS Behavior Panel Shares Helpful Findings [2018 CONFERENCE VIDEO]

This blog is based on a presentation at the FPWR 2018 conference. You can watch the complete presentation by clicking on the embedded video. In case you don't have time to watch the full video, we've included a full transcript below. You can also watch the full set of conference videos on YouTube.

 

Video Transcript

Susan Hedstrom:

I'm thrilled to present to you today our PWS behavior panel. We have four of the world's premiere PWS behavior experts who are joining us on stage today to share their insights into PWS behavior. ... Moderating during today's session will be Lauren Roth. Lauren is a psychologist by training; she's also the mother to Emma who is 19 years old. Lauren's contributions to FPWR have been immeasurable — besides being a board member in the beginning years she also planned and executed the 2015 Mental Health Strategic workshop which brought together behavior researchers from around the world. Several of the research collaborations we are funding are a result of that workshop and the top 10 recommendations from the workshop were recently published and the work that's been done towards those aims. If you haven't seen that blog, go check it out. It really lays out some really amazing work and collaborations that have come out of that workshop. To get us started with today's panel I'm going to present you with Lauren Roth.

[Applause]

Lauren Roth (moderator):

Thank you all for coming and we've gathered quite an amazing group of experts in Prader-Willi behavioral challenges. Just to give a little agenda, a little road map; being the parent of a child with Prader-Willi means you like structure. I enjoy structure and a roadmap so we're gonna have everybody introduce themselves just briefly and then we have been gathering questions from the community for the last few weeks so we're gonna start out with a few planned questions and have various members weigh in. Then we'll have some time for questions from the audience at the end. Kate, do you want to start and grab a mic?

Kate Woodcock:

Hello, I'm Kate Woodcock and I work at the University of Birmingham in the UK. Most of my research with people with Prader-Willi Syndrome focuses on the rigidity and the difficulties with change that people can experience and specifically a lot of the temper outbursts that can be linked to that rigidity. Two of the really exciting things that we are working on at the moment is we've just finished developing a video game for training some of the cognitive or brain processes that we think a link to that rigidity. Later on I'm going to talk more about that in my talk. We don't have the results yet but I'm excited about them anyway, and another really really exciting project that we're working on is looking at an early intervention approach; also trying to tackle those rigidity problems trying to help children to develop more flexible thinking from an early age. The other thing was how did I get involved in Prader-Willi Syndrome? I did my PhD on Prader-Willi Syndrome maybe about 12 or 15 years ago and I think what excited me about it was just that there's such a strong community and it’s really easy for us as researchers to then find out what you guys need. I found that having that drive behind my research was just a really really exciting thing because it meant that I could make sure that the research that I was doing was tailored for what people need 

Patrice Carroll:

I have a much less cool accent than you (Kate) and sorry I don't want to bore everyone today. My name is Patrice Carroll and I'm the director of PWS services at Latham Centers in Massachusetts. Latham Centers is both a residential school for kids with PWS ages 8 to 22 and we also have an adult program combined. We have about a hundred individuals with PWS. We've been doing it for about 40 years, so we've been around for a while. How I got into working with people with PWS was totally by accident. It was about eighteen years ago; I was working with individuals with traumatic brain injury and they asked me to oversee some of the houses with people with PWS for a couple of weeks. I fell in love and I've been with it ever since so that's how I got involved 

Elizabeth Roof:

Hi. I’m Elizabeth Roof and I'm at Vanderbilt University, recently switching from the Medical Center over to the Department of Psychology. I feel like I'm on the set of Oprah.

I have worked with people with Prader-Willi for the last 23 years and I have done several longitudinal studies working with a lot of different investigators. Most recently, the last 15 with Elizabeth Jenkins who the two of us have been working specifically on behavioral and psychiatric features. Two of the cool things we're working on is we just finished data collection for our grant called B.O.S.S. which is  “building our social skills” and we did an online social group. We had 10 groups of between 5 and 7 kids with Prader-Willi who met every week for ten weeks and I'm gonna be presenting some data tomorrow on that because we saw some really significant changes in people's ability to communicate effectively, to be appropriate, and not always be all about them (which is hard sometimes when you hear Prader-Willi Syndrome). The other thing we're doing that's really cool is we're working with FPWR to do these focus groups with teens and adults with Prader-Willi Syndrome so I've done about 15 people and hope to get the rest of them done in the next week. We're asking them about what it's like to have PWS, if there are drugs that worked,  what would they do, and what difference would it make on their life. The answers they have given me have just been mind-blowing. I literally sometimes leave after I and I've been doing this forever (it's hard to bring me to tears) but literally so touching so insightful and so amazing. We're gonna transcribe all that stuff and when they go to the FDA in a month they'll have all that with them so they can actually show the FDA that people with PWS can talk about what it's like to have PWS and they could do it incredibly eloquently.

Anastasia Dimitropoulos:

Hi, I’m Anastasia Dimitropoulos. I'm a developmental psychologist at Case Western Reserve University. I have done most of my work in Prader-Willi Syndrome.  I'd say most of my research has been in Prader-Willi Syndrome and most my work has characterized behavior problems with the syndrome. Most recently, my group is working on social cognitive skills in young children and identifying where there might be challenges and learning ways in which we can improve these skills through parent training and through direct intervention with young children. Tomorrow, I will be talking with Elizabeth. We'll be talking about our projects and I'll tell you a little bit more about our what we call the pretend program which is kind of silly, but we do use play based enrichment through parent training and direct intervention to work with young children to improve these skills. I will say that I first got introduced to Prader-Willi Syndrome 24 years ago as a college student. I was camp counselor and was introduced to an individual where I was in charge of her food for a week and her food schedule was different than the rest of the campers that were in that camp and in my cabin. I was very quickly introduced to what a Prader-Willi Syndrome tantrum is and then that led me to my work in tantrums early on in repetitive and ritualistic behavior and that's where it all started.

LR:

Thank you. Isn’t it great, the people study this stuff? One of the great things about our research and clinical community is whenever we ask them to do things they always say yes. It’s so great. A great response; even offer to help, so I feel really honored and lucky to have them all here. I'm going to start out this session with throwing out a question that's a really common one that comes up for many families which is… What are your thoughts on the hyperphagia behavior link? There’s a lot of conversation about if we treat hyperphagia, what will happen to the behavior problems? Will they go away or all the behavior problems related to hunger or their other issues? I will just let anybody who would like to grab that question.  

PC:

This is a very common question. I think that my answer would be: if we were able to take that off the table we would see a decrease in anxiety about that right? About that particular thing and it would take a lot of anxiety, wouldn't take all of the anxiety away, from a programmatic standpoint it would open up huge opportunities for vocational work and for some long-term

living. I think that it would be amazing for the community. I think it would be amazing for the individuals. It wouldn't solve everything but it would take a certain amount of anxiety away Do you want to add to that?

LR:

If anybody has anything new to add or anything else?

ER:

I think that actually is a really good question. I would say we don’t know. We thought that everyone thinks that all the anxiety and all the behavior issues are related to food but having done some clinical trials where we really did tamp down the hyperphagia for some of our folks they still had issues with schedules and things going as planned. I would say there’s definitely still some anxiety that’s under there and it maybe isn't related to food. I think it's really important for us to realize that even if we fix hyperphagia or we manage it it's still going to be really hard for some of our folks to manage some of the behavioral expectations that they do have.

AD:

I’ll just add for individuals with developmental disabilities who don't have Prader-Willi Syndrome, they also face some significant behavior challenges; so not being at the same developmental level as your peers can put you at risk and lead to frustration. I completely agree that it would alleviate a lot of the anxiety but there may still be some behavior challenges associated with other features of Prader-Willi Syndrome.

KW:

I think it's also important to remember that for any of us, just being hungry is really nasty and outside of Prader-Willi Syndrome we know that that being hungry feeling is really potent for just difficult behaviors in general. I think it is important for us to think about in that respect as well because it helps us to better understand what the person might be feeling. I think that leads into some of the other questions that Lauren was going to ask us about.

LR:

Yes, great discussion. The other question that comes up a lot is this idea about consequences for behavior and there's sort of common lore that negative consequences don't work for Prader-Willi. I wonder if some of you could address that and then if you agree they don't work what is helpful?

ER:

I’m a big believer that generally I don't like to use punishment for people with PWS and feel like they often have a hard time relating what they did to the punishment. I feel like the punishment should fit the crime so the idea of natural consequences if you do this then you won't get to do the thing you want to do because you can run out of time or we weren't able to do it. I think those are sometimes better consequences and I think a lot of it is using positive reinforcement instead of always using negative things; threats, ultimatums-things like that generally do not work with anybody with a developmental disability especially not for Prader-Willi Syndrome. A lot of times even if you get them to do it they will retaliate later or they will do something else because they're mad about it so I think it's really important to come up with better, positive ways, visual schedules, anything you can think of to move them along because for most of them they just get stuck. What you consider non-compliance may be in your perspective but for them it's that you can't hurry them up. You can't make them do something so it's better to lead them there that it is to try to shove them into that spot.

LR:

I also want to say one thing. I also think that with my 16 year old who started driving who did something she wasn’t supposed to do…and then lost the car for three days…she will remember that and not do that other thing again. I don't think that ourkids have that kind of brain that can think, “Gee, if I do that this might happen”. Sometimes I think a consequence for a behavior isn't as effective. Incentives for good behavior seem to work better. 

KW:

Yeah. I think that would be the same probably for most of us, that we prefer being positively rewarded for things. In the work that we’ve done in developing our video game we definitely have a balance towards being positively rewarded for things. It’s not punishment, but it’s alerting about “you need to do better”. We found that sort of frustration that can create really encouraged some of our players to get involved with the game, keep playing, and actually overcome some of the changes that they thought. Where they thought they just wouldn’t be able to do it all, they’ve actually got to the point where they’ve realized, “I actually like that I can do it and I've really done a lot more than what I thought I could”. I think it definitely balanced towards positive rewards. I think people can manage some of some you know subtle reminders of “ I'm not doing so well and I need to do better”.

LR:

Okay, next question. What are a couple things that the panel would recommend that parents tell schools, caregivers, extended family? What are two to three really important messages that we always share?

PC:

I’ll start with this one with a general remark and then we can add some details to it. Most specifically for schools I think that with any child with PWS, you can make a list of the challenges and then if you make a list of what's good that list of what's good will be so much longer than the list of challenges. What we tend to do sometimes especially in a school

setting is you'll sit in a meeting for an hour and the beginning the middle and the end are going to be all the things that they want to fix about your child that you need to change about your child. They spend no time talking about the fact that your child is sweet and engaging and funny and really really smart and creative. They talk about he perseverates and she's aggressive and

he won't do non-preferred tasks. If you're in that meeting don't allow that. Make sure that if they're saying something that needs to be fixed they're saying two things that are amazing;

because you know more than anybody that these are kids that you want to spend time with. These are kids that have that list of good things that is so so long and if we spend more time talking about the good things; if they spent half the time talking about what was good like they do talking about what needs to be fixed and building that up and into their program and talking about that and enhancing that. The behaviors are not going to go away but you'll definitely see a big decrease in them.

ER:

I think that's a really good point to make about really focusing on the positive. I think some of the other thing is to make sure that schools and other people who work with your child don't set them up to fail. We have a lot of schools and other places where they'll allow food to be in the classroom. They'll use all sorts of other things that I think “what are you thinking?”. That seems like a really bad idea - the kid with PWS gets stuck; they can't think about anything else, they can't be educated. Knowing what they're good at; knowing what the limitations are and putting some structure in place where they're not always set up to fail. They want to succeed, other people want them to succeed. You better setup the paradigm by giving them the information so they know what to do.

AD:

I would add to that I think I think food security, what your family's goals for food security are, need to be very clear with all the people in your child's life and that looks different for every family. I think it's important to give, to reduce that anxiety that comes up, and set them

up for a situation that will be best for them in whatever caregiving situation they're in. Then also to think about where they are developmentally so if they're an adolescent in a school system, the best food security at lunchtime may not be the most appropriate for a teenage girl. It may be that those conversations need to include how does this fit in with her social life in her peer group if she's being isolated to eat lunch? I think those things are important to consider as you talk to schools and caregivers.

LR:

Great ideas and I know when it comes to food security, don’t sometimes schools and day care centers (especially if your kid is doing well) might wait until there’s a problem? I’m a big fan of prevention; just get it set up from the beginning and if you have to you scare them a little bit… I often talk about life-threatening hunger because it’s a phase I read somewhere and I think letting them know this is serious. Even though your child may be of a good weight and may be very verbal… but letting them know that this is a serious business. 

There  were several questions that came up on the app about flexible thinking how to encourage flexible thinking and (looks to KW). I know people must have known you were going to be here, I don't know if you want to talk a little bit about some quickly at-home strategies for that and your talk is at four o'clock today…. 

KW:

I’m going to cover it a lot in my talk but one of the things that we've done is tried to think about: What are the brain processes that might be important for flexible thinking and then how we might be able to train those brain processes? Our current thinking which

also feeds into the early intervention idea that we have is about how we might give young children the opportunity to practice flexibility at the right time and how we might build that into the structures that children need (while still giving them this opportunity to practice the flexibility)?  I will talk about that more later.

LR:

Anastasia do you want to add to that? 

AD:

I will also add that this is something, at least for young children, that you can do in play as well as setting up a play scenario where you're sitting together and playing through situations. Maybe you change the story up so that it's not the same every time; where you introduce some alternatives that you may get resistance on but that you can build into to thinking about resistance and flexibility with the characters-and not with the actual child. Right? The child is playing through the story with you and you can use the characters to work out some of those flexibility issues for young children. 

ER:

I wanted to add that taking baby steps…you don't have to jump off a cliff to do something really crazy to make your kid be flexible. I think taking baby steps, so they can know and even making it fun and challenging. When parents will tell me they drive the same way too and from their house to the school every day and I'm like, “You make it up it's crazy Friday. You never know which way I'm gonna go!”… and you take a different way. The kid will be in the backseat going, “Oh my God!” and you get there and you go, “Look we still made it here even though we took a different way you”. You start shaking things up by baby steps and making it fun and again. It's a great way to get them to realize that not every change is a bad thing but sometimes you have to really play it up and act up and when it comes to those things.

PC:

Yeah, and just briefly the only thing I'll add is that social stories are the greatest things ever. You can write them really quickly about any scenario.

LR:

Good. We’ve used those quite a bit. They’re helpful. We’ve talk about this issue so much in my house that my daughter calls me on being rigid at times. “Mommy, you could be a little more flexible about this”. And she’s right. One more planned question-this is kind of a big one, which is that someone on the app asked, “Can people with Prader-Willi grow up and take responsibility for things and make good choices when it comes to food and other things in life?”.

PC:

I'll start with this one. I think that individuals with PWS can make huge strides with the right environment, the right structure, the right meds (a number of things). I think and I'm curious Elizabeth, as to with what you think about this? I think it's fairly safe to say that wherever your child is in their teen years, whatever support they need at that point- it's probably what they're going to need going forward unless something comes along that that changes that. I think that's a pretty safe thing to say do you agree with that?

ER:

I would definitely agree with that and I think this idea that the growing up part is really interesting to me because we follow people all the way up through 50s and 60s and they still have food issues. I think it's unfair to put them in positions to fail so I think you set up structures and ways to help supervise and monitor that keep them safe. Again, it's okay to give them some level of freedom as long as it doesn't risk their health or anything else. I think you have to but  it's not fair to put that on them and give them a responsibility because it would be like any of us taking responsibility. It's not a cognitive process if only people who were low functioning or overweight (you know what kind of world we live) and that's not really what it is. It's not a cognitive ability. They're hungry and if the right opportunity and the lack of supervision gives them the ability to get food that's not fair to them. I think you have to just know that it's not fair to say that. They should take responsibility that's setting them up to fail.

LR:

Elizabeth, I was just going to say kind of related to that, and maybe you can comment on this as well Anastasia…so when a person with Prader-Willi does have a food violation any thoughts about how to respond to that?

AD:

The only thing I was going to say is it's not a cognitive issue. It's also not a maturity issue. We think of our children as graduating from high school and starting their careers or their life path and hyperphagia is not a maturity issue. We wouldn't think that would change after that time period.

PC:

Yeah, and to respond to that question as well there is a food violation. If somebody gets to food that's an issue in the system. so we don't do anything as far as a consequence for that unless it was a large amount of food they need to be seen by a medical professional. If there's food acquired somehow that's an issue in the system that we need to fix.

ER:

I think the other thing you can do is: if it's what the food is and you know the calories, I just look at them and say, “Hey so because of this” (and I don't blame them) I just say “because of this issue we're gonna take that extra calories away so you don't have to worry about that later” and they're funny they're like “what do you mean I don't have to worry about it?” It’s really good because you take that responsibility off them and don't really make it a moral issue. I always say people say they stole something. When you steal food it's not the same thing as taking something from a store. I think it’s really important to know that the consequence should be subtracting calories or doing something else to make it equalize out. I would not ever take food from somebody who's already acquired it. That's a really good way to get yourself hurt.

PC:

No, that’s exactly right. There have been times where somebody has taken food and hasn’t eaten it yet and we've said you can either give it back or that amount of calories will come out of dinner and they almost always give it back because they want their regular dinner but you're also having them choice.

ER:

Yeah, you’re giving them control.

LR:

Yeah. The other thing someone mentioned to me, I think it was Evan, that sometimes when kids with Prader-Willi are having more behavioral issues and you don’t know why…especially when they are a little bit older because there’s been a food violation. I don't know if they feel guilty here but one thing I've instituted with my daughter when I've suspected that, as it’s a confessional. “Okay, you have 15 minutes, just tell me. I will have no response. I would just say thank you for telling me”. With that strategy, that’s worked well for getting intel.

Why don’t we have some questions from the audience?

[PARTICIPANT]:

Thanks. Quick question on something we do with our daughter. It came to mind when we are asking the questions about what to tell schools and also how to deal with rigid thinking. One thing that we found is a pause, an extra wait time for requests or dealing with any kind of situation is super important. I'm wondering how much how much of that you guys have seen across the population? Where asking our child who has to do something that she might not want to do she'll initially just say no or won't do it or will rebel. If you give her maybe two minutes she comes around she just does it…so it's a beat and we found that that really helps with school it really helps  at home. How much of that have you seen ?

PC:

I love that. I've worked with a number of individuals, both kids and adults, who if you asked them something they'll just immediately say no (just because it's a control issue). I love the idea of a pause what we've also done is for people who really really have challenges with this is ask them to do something. Don't look at them. Ask them to do something and they immediately walk away and then they have that moment to decide whether to do it or not without it being a power struggle. That pause is really really important because many individuals with PWS also have executive functioning disorder and they don't have that pause built in them at all. If you're able to teach that pause before answering by example that's an amazing thing to do because they don't have it. They have to learn that that's great.

ER:

I think a cooperative way, I always say, I don't ask them to put on their shoes I don't say, “Do you want to get dressed? I say, “Oh it's time to get dressed” so it's not a request because that's almost always better with an Oh! so this idea. I think the idea of a pause is great and I think a lot of kids struggled with that. They can't wait. They have to automatically say no but I think it is a good way to help them delay gratification but also delay a confrontation because for a lot of people requests feel confrontational and shifted. Checking your tone, the way you look at them because again, if you're looking stern and they look at that and go “I don't know what you're doing here but I feel like this is a really negative thing”. Try to sell it to them all but with your expression and how you ask.

[PARTICIPANT]:

Thank you. I have an 11 year old son. I’m from Canada and he started now to actually pick up gum and garbage as you're walking with him. I know it becomes a parent's responsibility to try and reduce all those environmental problems but it becomes totally unrealistic because he'll be holding your hand and he'll grab something and put it in his pocket. We don’t know how we've done all the teaching cognitive. I know that doesn't make any difference he doesn't care that he could get very sick. He just tells us it tastes good. If you have any strategies because it's getting worse? He's going to go into junior high next year there's food access everywhere in the hallways and the garbage. Do you have any strategies when he may know what's unsafe but that doesn't make any difference to him because he's hungry ?

PC:

Is someone with him all the time that sees when it happens?

[PARTICIPANT]:

Yes, he’s never left alone, believe me. 

PC:

So you know every time it happens, you see it right? 

[PARTICIPANT]:

No, we’ll be holding his hand and he’ll grab the other ...

PC:

... and he’ll just snatch it?

[PARTICIPANT]:

We don't even see him do that. He’s never alone. That’s one of our problems as well.

PC:

I'm just wondering if they could do something where at the end of every day when he doesn't do it, he gets something?

ER:

Yeah, I would say something positive and again. They don't have a level of disgust so this idea of something being gross-- We did a study where we actually showed them all these odd things like chocolate milk stirred with a comb and they're like, “Oh, I’d drink that!”. They don't see the same things that we see. I think it's really important not to think about it as germs because they don't care, it's something good. I do think if he doesn't do it getting some kind of reward, even to chew sugar-free gum, whatever, a sticker…doesn’t matter. He starts to help him. It’s an impulse control issue. It’s “I see it, I want it, I take it”…so if you can help him limit that response and if he doesn’t do it given a reward, that’s great. 

PC:

Even if it is gum and I know this is “Oh, she's gonna say give him food!” but in some cases if you don't do this all day because this is potentially dangerous behavior…if you could reward him at the end of the day; even to say “You get three sticks of gum”. Give him the gum. I 'd rather have him have the gum from the wrapper than on the street. If that would work, if he could refrain from doing that…knowing that there’s something he really wants at the end of the day, absolutely do it.

KW:

I don't know how because there's an issue if you're giving him a reward at the end of  the day then he has to wait for that. It might help to do something immediately. If you could give him something immediately that would then signal okay, later you can get whatever it is that you think. If  you could give him something immediately when taken instead of actually taking the garbage at that point. Then that might help get over that initial barrier.

[PARTICIPANT]:

Would that work with the token system then? Would you give them a token instead of another piece of gum? Because if my son knew every time he was going to pick up a piece of gum off the ground that he would get a piece of gum? Could you add the token system in then? 

PC:

You could. I kind of hate the token system. It’s just kind of I think beneath what they're able to do. I think that he could understand that we're walking from the car into the mall or whatever, you walk in here safely you're getting the gum right? So there could be six pieces of gum on the ground that he didn't pick up. It doesn't matter you walk in there-you're getting the gum because it's only when you're outside walking around right?

[PARTICIPANT]:

So you’re talking about doing it preventative then first. 

PC:

Yeah

[PARTICIPANT]:

Every time you go out in public you say, “if you can make it through here without grabbing something out of the trash or on the ground, when we get done you can have a piece of gum”.

PC:

Yeah, yeah. That’s right.

ER:

And you’re setting up the expectation, which I think is really important. We often get into a situation when you’re out in public-something awful happens and you go “Wow! Who knew they were going to do this”. You should always anticipate that if you’re going to be in a place and something’s going to happen. You need to know the lay of the land, what the expectations are, and if you are going to get out of hand. They need to know that we will leave the premises. I tell people you have to do it. You have to make it so they understand that. I think it’s good to have that expectation set up.

PC:

I agree and I think that was another question that came up about going out in public and wanting to avoid a scene; so giving in…don’t ever do that. If something happens and you had an expectation and you go into public and your child’s about to cause a scene and you don’t want one, you say “all right, forget it. We’ll do what you want to do”. Well, there you go. You’ve just set up---

ER:

It’s the slot machine. You pull the handle 50 times and one time you get a payout and then

you're sitting there for eight hours. You know it’s the casino doing it so people often think that I just need to get my kid out of the situation. I'll give in. 

PC:

Don’t! (give in)

ER:

I had a girl many years ago who laid in the middle of the street at Vanderbilt and cars were stopping; everyone though she had been hit and I walked up to her and I actually thought she fell. She looks up at me she goes, “Give me a cookie!”. I don’t have a cookie. “Give me a cookie!” She just kept saying it and the mom said, “Give her a cookie!”. I don’t have a cookie on me. The mom apparently had been doing it on a regular basis. I said we’re going to get up and walk across the street. We’re just going to the corner and I said she’ll follow us and she finally said, “I hate you”. I said, “Yeah I know”. The mom said, “How did you know she's gonna

get up?”. I said I wasn't entirely sure but I'm really glad she did and for the rest of the day we had a lot better issue with compliance because she knew I was not going to give in. Giving in is such a disaster because you set your kid up to know that sometimes they are going to get a reinforcer.

[PARTICIPANT]:

You really can’t give in no matter how wrong the behavior is because they are going to go from there to having a tantrum.

ER:

--they'll escalate

[PARTICIPANT]:

--escalate until you give in.

LR:

Thank you. Yes, sir?

[PARTICIPANT]:

I think my daughter is smarter than I am to tell you the truth. There’s one thing; she comes home and she says “Dad, why am I so wrong? I'm different and nobody likes me”. Of course I tell her, think about it you have friends who can’t see or hear or there is a web or something so the kids can say hey, you know I am okay. 

ER:

Yeah. The social skills group was so nice, as you know. We have it look like Brady Bunch tiles on a screen and it was really cool because every person in the group would go. Everyone has Prader-Willi. Some of these people don’t look they have Prader-Willi. They do all good and it was really cool for them to see people who were doing better than them, maybe worse than them, the same as them. They really developed some strong friendships so I think pointing out that everyone has deficits. I can't find my way out of a paper bag. I mean seriously I've been lost in this hotel 2 whole days. I think it's really important to point out things that everyone is not good at something so this idea that everybody struggles with something so they need to know that they have a lot of strengths. As Patrice said, pointing out their strengths: you're caring, you love animals, you're good at puzzles, you do this, you do that is really important for them to focus on the positive and not get stuck on the negative .

[PARTICIPANT]:

My daughter is five. We’ve been struggling with potty training. She just gets in that rigid point where, “I don’t want to go on the potty. I like my diaper better”. We’ve been trying with positive reinforcement. We've done stickers, we've gone to some food items but even with that she is still very rigid. Do you have any suggestions for potty training?

PC:

I don't know if we do… but I'm certain that other people sitting in this room do. Can anyone help this lady out?

LR:

I’ll just say, go ahead and stand and up raise your hand so that you guys can connect. . The other thing, I think there is a breakfast meeting tomorrow so your age group breakfast would be a great resource for you to talk to other parents. 

PC:

Yeah. Just to know that you are not alone in this. You’re not different. You know you’ll have friends here. 

[PARTICIPANT]:

Hi, my son is six. His issue at the moment is nail peeling. Ever since he started school just over fourteen months ago he's constantly picking at his nails, toenails, and fingernails and it's obviously in anxious situations. It’s also a boredom situation if he's just watching TV. If you look at him he'll stop instantly and put his hands away because he knows not to but the impulse is too strong; it's gone so far he's pulled off his toenails at stages. Our endocrinologist has told us to ignore the behavior but that was over a year ago and it hasn't improved. We are just wondering if there's any strategies we can use on that?

PC:

Can I start? Sometimes you almost always will see some little picking, pretty much across the board (the cuticles, anytime it’s a bug bite). When you’re getting to the point where it is pulling fingernails or toenails off  or having some real deep picking to the point where it’s becoming dangerous you may be looking at some sort of barrier. If he’s picking at his toenails he might be wearing tights so he can't get to them. He might be wearing gloves so he can't get to them. Then it kind of changes that pattern a little bit because he's not going to immediately go to his toe nail to pull it off if there was a barrier there. That may help.

KW:

Perhaps also trying to substitute the sensation for something else. Some people that we've spoken to have use things like a sticker book where you can  peel off the stickers and put them back down again. Particularly the boredom thing-if you could have that (stickers) when he was sitting in front of the telly to make it harder to pick the nails (but easier to do something else) they would substitute it.

LR:

Especially when they're doing an activity like watching TV or where they can zone out that's usually the highest risk time of doing those things. Make sure they have the barriers. Some people use liquid band aid, and a band aid, and socks because you want to interrupt that impulse (which ignoring will not do anything). Great question.

[PARTICIPANT]:

Hi, I’m from Australia. I just wanted to say about the idea of having flexibility, one thing that we did which was really good and has continued throughout our time was we created very early on (in first, second year, third year) this idea that change is something specific that we are going to do now. What we did was we did half a day of surprise and so we had a whole half a day of surprise where we did create larger and bigger and more interesting things that were always outside the box. The idea was that the environment became bigger and bigger and bigger and nothing became something that she hadn't done and so now we can actually if we want something to change, we're just saying it's change time now. It’s worked and it’s been very very helpful that we did this. About 2 we started straight away but then she’s 14 no so we still use that. With the picking, you’re saying just use positive reinforcement. We use negative as positive so what we do is we say, “this is your iPad. You can have your iPad. She likes the idea that it will be taken away if she does something like pick so she keeps the iPad. We reverse it. I didn't have a question I just wanted, just in case that was helpful.

 

[PARTICIPANT]:

Hi. My question has to do with teenagers transitioning into adulthood. My daughter's 17, she's a senior, and she's going to be graduating and has the aspiration to go to college to become a veterinarian technician. She wants to start working at a pet store so my concern is that she does need constant supervision which she does have now in high school with a one on one aide. Going into the workforce for instance, could you tell me if you know of examples or how things have been done with other families? How could they transition into a working environment and be able to still be safe because she obviously would steal out of the fridge or take money out of co-workers purses or those types of things. A sidebar to that question: as an adult, once they turn 18, how is something like that managed? Do they go to jail? 

ER:

First of all, congratulations! That’s a pretty exciting time but I will say it’s scary. I think part of it is we do have—so I actually have nine or ten people who are working in some kind of job but they do have some level of supervision.  I will say that having access to co-workers lunches and things like that should not be allowed. If you do job coaching where somebody is there coaching on how to do the job you know if you can fade some of that out or they need some level of that. Then you do what you need to keep them safe. The other thing you can do is actually have them wait in or do checks or something else to keep them accountable. For one young lady she was working in a hospital; she was stealing stuff left and right and what they'd started doing is weighing her every day. Sure enough her weight was going up and once we found out that was happening we had to actually scale back her job and things like that so I would say she's gonna need some level of supervision as far as the theft. Everyone should know that this person has a disability. They have Prader-Willi. This is likely to happen, so the law enforcement, the people at the work, everyone should know you do not want to have some law enforcement person trying to do the old scared straight I'm going to teach you a lesson and try to like scare them. That does not work and it's a really dangerous situation so everyone needs to know and they need to expect that it's going to happen, that she's going to maybe take stuff on occasion -but supervise .

[PARTICIPANT]:

This is this more of an observation and I’m thinking that this is a good strategy…so I want to bounce it off of you. Two things: the first is our son just turned seven today and last year when he was five we started some therapy, behavioral therapy, at school with the school psychologists (who have actually been tremendously helpful). He was having some issues with impulse control (as I'm sure many here can relate to) and one of the things that she did was - he has apraxia of speech - so she wanted to give him some verbal tools that he could actually say that would help him understand the choices that he was making. The two words that she started giving him were unexpected and expected in relation to his behavior; if it was something that was not good, that was where he was not in control or maybe he was not positively affecting the people around him. There was a three step process ( I actually have it here on my phone so I could explain)… it was basically if you're not following the plan; if you're acting opposite of what is expected of you then you are acting unexpectedly and so it got to the point where with lots of repetition and we followed suit at home that when he would do something impulsive I would get down to his level as a five-year-old and I would say, “You know buddy, was that expected or unexpected behavior?”. I would use it in a positive way as well when he was doing something really good and he was every time able to tell me that it was unexpected or expected, exactly the right way. We’ve continued using that language and now he's seven and I still feel like it's been a really great tool. What we've noticed is that I know it's empowered him. He's not hitting, he's using his words like, “I'm so mad”.  He'll say that and we'll talk about how that could lead to an unexpected behavior.

Then last but not least (and I’m not sure if this is a good tool) but two years ago we had a lot of new behaviors that were emerging in the last few years. My son is obsessed with scary stuff. I don't know about anyone else in here but he really is interested in magic and particularly witches. We kind of created this alter-ego. Her name is ‘Hee Hee’ and she basically uses reverse psychology on Jude. It’s not my voice it's a totally different person. It works like every time if I need him if he is being very very rigid or he's opposing bath time or he's opposing going to school;  ‘Hee Hee’ steps in and she's likeYeah you know I'm not gonna do the voice because it's really—Yeah, right no pressure…maybe after a few cocktails. She comes in and she basically says things like “Yeah, don’t go to school Jude because if you go to school you are going to get too smart you're gonna learn how to read my spell book. He gets so excited about that because she never wants him to do anything good and same with his shot;  if he's opposing the shot she thinks that's hilarious because he's never gonna grow up and he'll never be able to be empowered in kid words. That’s working for us and I don't know for how long it's gonna work. If we're in a very stressful situation he'll look at me and he'll say “hee-hee” and I have to embody her voice right away and if I answer in my voice he's like “no, no, no, hee-hee." Then he will smile and he’ll say “Mom” and have to switch back but it’s really effective.

I just think it does two things for him; it gets him in a pretend play situation where he feels like maybe there's some element of game or control around what we're doing (I think just like on an airplane). It literally works every single time we're in a stressful situation so I just wanted to share that, I don't know if this is the right approach. I don't know if we should be doing this because it does kind of put him in this fantasy world, but we went to the Halloween store and he saw a really ugly witch and he goes,“Hee-hee, is that you?” He loves it and he knows that it’s not real but I just wanted to ask if that’s something that you would suggest is a good thing or not?

PC:

This is an example of the key to every behavior working with PWS. You do what works until it doesn’t work anymore and then you do something else. Yay! Good job, "Hee-hee."

AD:

Can I also add that I think your comments highlight two really important things. One, that you're communicating with the school and you're using consistent methods that the school is using. I think that's really helpful for children that you're in contact and that you're working together. I think the other thing that you've highlighted by sharing your story is that all of you can help each other so much because you have the on-the-ground daily experience every day. We see your children sometimes some more than others, but you all have rich experiences that you should be sharing with each other. I agree, if it works for now that’s wonderful until you need to change it again. 

LR:

I applaud the creativity. We do need to stop. I know we could go on forever, but we do have other things to do so I encourage people to get together with other parents, networks, go to the breakfast and share ideas. There was a couple of folks who were interested in the potty training idea so maybe you all could have a drink tonight and talk about potty training or get together after this session. I want to thank our amazing panel.

[Applause]

ABA and PWS Video Presentation - Tablet

Learn more about Cindy Szapacs' presentation on ABA and PWS: Dispelling Myths and Changing Behavior.

Topics: Research Blog

Susan Hedstrom

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Susan Hedstrom is the Executive Director for the Foundation for Prader-Willi Research. Passionate about finding treatments for PWS, Susan joined FPWR in 2009 shortly after her son, Jayden, was diagnosed with Prader-Willi Syndrome. Rather than accepting PWS as it has been defined, Susan has chosen to work with a team of pro-active and tireless individuals to accelerate PWS research in order to change the natural history of PWS. Inspired by her first FPWR conference and the team of researchers that were working to find answers for the syndrome, she hosted her first One SMALL Step walk in 2010 and began the development of the One SMALL Step walk program which now raises over $1.5 million a year for PWS research.

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