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S02 Episode 01: Losing Genes but Gaining Music
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This season, we’re going to focus on music as medicine—telling the stories of people whose lives have been immeasurably improved with music. In this episode, we talk about William’s Syndrome, a genetic condition that causes heart problems, intellectual disabilities and a profound love of music. We hear from 31-year-old Benjamin Monkaba, who has the condition, his mother Terry, and Jennifer Latson, author of The Boy Who Loved Too Much, a book about William's Syndrome.
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Transcript
Benjamin's Diagnosis and Williams Syndrome
00:00:09
Speaker
Well, when Benjamin was six weeks old, he went into heart failure. And I didn't know it at the time. I thought he had a cold. I took him to the pediatrician. And for the first time that day, she heard a heart murmur and said, you know, I think we probably should get this checked out. And I said, OK, should I make an appointment? And she said, no, I think maybe you should drive straight to the hospital.
00:00:31
Speaker
And that was our first inkling that there might be a problem. And when the doctors did some testing and found out that Benjamin had aortic stenosis and that that particular cardiovascular problem is almost exclusive to Williams syndrome. So at the time, since he was only a six pound baby and they really couldn't tell his by visual characteristics or anything, but they still felt that he might have a rare condition called Williams syndrome.
Music and Williams Syndrome: A Unique Connection
00:01:00
Speaker
Welcome back to Cadence.
00:01:02
Speaker
the podcast where we explore what music can tell us about our minds.
00:01:18
Speaker
If this is your first time listening to the podcast, maybe go back and check on season one, where we explore the fundamentals of music, questions like, what is music? How do our brains perceive time? Why do we like the type of music that we like and not other kinds of music? This season, we're going to be focusing on music as medicine. We're going to be telling the stories of people whose lives have been immeasurably improved with music.
00:01:44
Speaker
In this episode, we're going to talk about Williams syndrome. It's a genetic condition that affects about 1 in 10,000 people worldwide. I heard about Williams syndrome for the first time in 2010.
Jennifer Latzen's Exploration of Social Aspects
00:01:57
Speaker
This is author Jennifer Latzen, who wrote a book called The Boy Who Loved Too Much about Williams syndrome.
00:02:03
Speaker
What I heard about was more of the social aspects and how people who have this disorder love everyone, trust everyone, just are driven to meet and engage with people. And my first thought was, why is this even a disorder? It just sounds like you're an amazing person.
00:02:20
Speaker
And so I just was really interested in learning more about it. And once I started meeting people with Williams, I really just fell in love and wanted to spend all my time with them. Jennifer spent a few years shadowing a family whose son Eli had Williams syndrome. The boy that I wrote about and many other people with Williams syndrome are just completely outgoing and they've never met a stranger. As soon as you walk up to them, they want to be your friend. They want to have you
00:02:47
Speaker
over for a play date or, you know, just spend all your time with them. The first time I went to visit Eli, I had met his mom and I said, you know, I'd love to come in and interview you guys and maybe write something. And so I spent a few hours at their house and when I got up to go, Eli said, wait, you're leaving? Like he thought I was just going to maybe move in with them. So that's kind of what a typical person with Williams is like, is they just want to spend as much time as they can with you and be your best friend.
Traits of Williams Syndrome: Upbeat and Friendly
00:03:23
Speaker
So let me introduce you now to Ben Moncaba, whose mother you heard at the top of the show describing all of his heart problems from the time when he was only six weeks old. I asked Ben to tell us what his personality is like.
00:03:35
Speaker
Well, upbeat and friendly and kind and just bonus of fun. That's what we are. He very quickly exhibited. This is his mom, Terry, the social and endearing personality that is typical of those with Williams syndrome. His very first words were, hi, how are you? And he said those constantly, pretty much all day, every day to everyone he met.
00:04:00
Speaker
Williams syndrome, one of the unique things about it is it's a very small genetic deletion, so it's 26 to 28 genes that are missing that most of us have on chromosome 7, and it creates all these sort of seemingly disconnected symptoms, so the friendly personality is the big one, but it also creates intellectual disability, so the average IQ for Williams is around what it is for people with Down syndrome, so it's
00:04:27
Speaker
in the 50s on average, although it does range. And despite the overall intellectual disability, there are certain areas that are really strengths. So verbal ability is one. People with Williams tend to have a really good vocabulary and they can talk really easily. They're great storytellers. And then they also tend to have
00:04:47
Speaker
musical ability that is sort of beyond what you would expect for their intellectual disability and an intense affinity for music and a really just profound emotional response that's a lot stronger than what we see in the general population.
00:05:12
Speaker
I think music is a very positive thing in a lot of ways, but I can tell you two things that makes music what music is. It tells a story and it makes you feel good when you hear a song. And it's like sometimes when I hear songs, I feel happy. And sometimes when I hear songs, I feel sad because I'm an emotional person. And people with Williams syndrome are like that. When my mom hears music, it affects her in a different way.
00:05:42
Speaker
Do you know who, um, Steven Sondheim is? I do. Yeah, I'm a big fan of his musicals.
00:05:49
Speaker
Yes, my favorite musical is a little night music. Send in the Clowns is the most beautiful song I have ever heard on the radio or on YouTube or anywhere. I think that song is, it's a very emotional song and it's a song that I can relate to because I'm a clown myself. And what they're saying is, where are the clowns? There ought to be clowns. It's like, there's gotta be laughter in the world.
00:06:17
Speaker
What do you mean when you say that you're a clown? Well, actually, I clown around at different parades and stuff and speaking of the clowns, they're my favorite thing in the world.
00:06:37
Speaker
When I met the Shriners the first time I was three years old and they were hilarious. They were the most hilarious clowns I've ever seen. They helped kids a lot and even though I didn't have problems like
00:06:54
Speaker
Some kids do. They cheated me like I was one of their favorite people. And that really made me feel good. And I wanted to become a clown because I thought it would be fun to make people's days better. I'm a very different character when I'm a clown. I'm not myself. I'm just a guy with funny shoes and funny hair and funny nose.
00:07:22
Speaker
and what I do is I entertain people that are feeling down, I make them happy again, and it's the most wonderful thing in the world. So, can I ask you a question? Please. Have you ever seen a clown before? You know, I think I've seen one at a birthday party and maybe there was a time when I was a kid when I went to a circus, but I really don't remember it very well.
00:07:49
Speaker
Ah, well uh, I guess you're pretty happy that you're talking to one right now.
00:08:01
Speaker
And Ben doesn't just clown around for fun. It's actually his job. Benjamin, his vocation is entertaining seniors at assisted living facilities. And at the Berkshire Heroes Music Academy, where
Ben's Musical Journey with Berkshire Heroes
00:08:14
Speaker
he goes every day, he works with one of the music therapists there, and Benjamin lays down the drum track, and Andy lays down the piano or a guitar track, and he creates basically the music that he takes with him into the
00:08:29
Speaker
senior facilities obviously can't take in a drum set, it's a little hard. So he records all the music himself but puts a dent on his iPad and brings an iPad and a small PA and entertains.
00:08:56
Speaker
So, Ben, why do you bring music into nursing homes and other places? Because it brings some joy. It brings me joy, and I want to share a joy with other people too. Do you want to know what my favorite music group is? Very much. My favorite band is the Beach Boys, actually.
00:09:17
Speaker
Their music is fun, and it's great to dance to. They have great harmonies, and I love how they sing, and it's just like, I was amazed and so happy when I first heard them sing. I mean, they have some great harmonies. These guys are great singers. I liked the Beach Boys for a lot of reasons because they sing about the warm sunshine, about surfing and cars,
00:09:47
Speaker
all those beautiful girls, too. It became obvious pretty quickly that music plays a central role in Benjamin's life, and that's true for a lot of people with Williams Syndrome.
00:10:03
Speaker
When people listen to music when they're hooked up to brain imaging sensors, I mean, music is considered a universal stimulus. So most of us, when we listen to music, will have certain parts of our brain light up in response to that stimulus. But for people with Williams, even more of the brain lights up and it lights up more dramatically. And what they noticed is the oxytocin levels that are already higher in Williams syndrome,
00:10:30
Speaker
jump even more dramatically when they're listening to music. So it's a very exaggerated response kind of on every level. Last
Oxytocin, Social Bonding, and Music Affinity
00:10:40
Speaker
season, we explored how one of the fundamental roles of music is to create bonds between people. So it makes sense that the neurotransmitter oxytocin would be involved. Since oxytocin is sometimes called the love hormone, but it's really about attachment.
00:10:56
Speaker
And it has many complex effects, but a lot of people point to the fact that oxytocin is really only found in mammals, and that it is released in moments in which two mammals are bonding with each other. So, for example, when a mother has a newborn baby that she's breastfeeding, where she looks into the eyes of the baby and so forth, we see huge increases in the release of oxytocin in the brains of both the baby and the mother. And the same is true when we have an orgasm during intercourse.
00:11:25
Speaker
Now there have been a lot of theories about the role that oxytocin plays in social bonding, and sometimes there's a lot more hype than there is evidence. But if you consider all the data, one thing stands out. Oxytocin is important for forming bonds between individuals.
00:11:41
Speaker
So if oxytocin is one of the neurotransmitters that is affected by the genetic underpinnings of Williams syndrome, it might explain why people with Williams syndrome seem to show what we call pathological friendliness. They seem to be in love with everyone they meet. And that seems to be what Jennifer and Terry both describe in terms of the behavior that they observe in the people around them who have Williams syndrome. So maybe that's why music plays such a fundamental role in their lives.
00:12:11
Speaker
If music is a stimulus that taps into our bonding system and these individuals have a strong propensity towards making bonds, is that the answer?
00:12:28
Speaker
But I also wondered if people with Williams syndrome are actually more likely to become better musicians than the rest of the population. Do they actually show enhanced musical abilities or is it more about their appreciation of music and their response to music? Well, there have been conflicting studies because at first I think people got so excited about the musicality of people with Williams and the anecdotal fact that some people with Williams, a number of people with Williams have perfect pitch and some of them have been able to
00:12:56
Speaker
pick up instruments and without training be able to teach themselves to play kind of by ear. And so those anecdotes were so impressive that there was this theory that, okay, maybe they just have more innate musical ability than the general population. And a number of studies have been done. There was one that found, that concluded that three in 10 people with Williams have perfect pitch, whereas for the rest of us, it's about one in a thousand.
00:13:23
Speaker
But then other studies have really discounted that. So what it seems to be is there's not a higher tendency necessarily for people with Williams to become musical savants, but given their intellectual disability, they have a lot higher musical talent than you would expect and some of them are savants and some of them do have these great gifts. Almost everyone with Williams has
00:13:47
Speaker
this sort of hyper-appreciation of music and you hear all these parents talking about, even when their kids couldn't talk yet, their response to music was so dramatic and if their infant heard a sad song, they would just be completely moved to tears even if they were happy before they listened to it.
00:14:06
Speaker
And from a very young age, if kids with Williams hear upbeat songs, they'll be dancing and clapping and they just really get into it. I asked Jennifer about Eli, the boy with Williams syndrome, about whom she wrote her book and whether he also had a special relationship with music.
00:14:23
Speaker
Absolutely. You get so used to the fact that he is singing constantly, that Gale doesn't even notice it anymore. She just kind of talks over it. But when I started spending time with him, I was just stunned. And it got to the point where I would start to make a note of the songs he was singing when I was with them. And I just like
00:14:44
Speaker
couldn't keep up because he's just always singing and when he's not singing or talking he's you know kind of banging in rhythm or you know just clapping his hands or tapping so he's very musical and his tastes are all over the place he really likes Pavarotti but he also really likes Meatloaf and Lady Gaga and Alice Cooper are some of his favorites and I think it's just he really likes music that has
00:15:13
Speaker
a very dramatic kind of emotional or performative quality and he really tries to imitate, he'll sing those songs and he'll try to imitate the exact sort of style and pitch and everything of the performer.
00:15:36
Speaker
People with Williams also tend to have hyperacusis, which is an extreme sensitivity to sound and certain sounds in particular. Thunder is really painful to them. Their hearing is just good. It's like they can hear you whispering from across the room. There are certain sounds that even apart from music, Eli just really has an affinity for.
00:16:01
Speaker
Part of what I mentioned in the book is how obsessed he is with floor scrubbers and that to him is just a beautiful sound like the slurping kind of droning sound of a floor scrubber is just beautiful to him and he just wants to like listen to it all day. So it's just interesting that certain things really affect them that we just would ignore.
00:16:30
Speaker
I think, Ben, that music affects me in a different way from you because when I see you and I see the music that you're listening to, I see that you are totally engrossed in that music. That music envelops you and you
00:16:45
Speaker
feel every ounce of joy that that music brings, or you feel the sadness. Ben never just listens to music. The first thing he wants to know is what all the lyrics mean. So he needs to know sort of everything about the song, whereas I will just listen and say, wow, that was a great song.
00:17:08
Speaker
But for Benjamin, each song is so much more. In the days when we were running our summer camp, during that week, if we put on happy music, all of those kids with Williams syndrome, all of those campers in the front of the room are immediately up out of their seats and dancing. And if we play classical music or play songs that are predominantly in the minor keys, some of them are just awash in tears.
00:17:35
Speaker
They are so emotionally affected by the music, whereas the rest of the world seems like we can all go to concerts and we can sit in the audience and really love the music, but we sit quietly and love the music. Those with Williams syndrome are truly moved by the music, so much so that usually they can't sit still.
00:17:56
Speaker
Benjamin, how does that make you
Ben's Emotional Connection to Music
00:17:58
Speaker
feel? Is that true that when you hear music that you love, especially like, say, the Beach Boys or Send in the Clowns, do you feel like you have to get up and move? Well, when I hear the Beach Boys, it's like a party. That's what their music is, a party. And Send in the Clowns is like a song that anyone can sing, but they gotta know the emotion of it to really send the message that it sends.
00:18:25
Speaker
So when I heard that song, sending the clowns, it kind of hit me the same way.
00:18:35
Speaker
And when I learned the lyrics, the song, I felt like, wow, this song can be a very emotional song, but it sends a positive message, not a negative message. I have to tell you, Indi, that when Benjamin first started singing that song and the hardest thing about that song for him was to actually sing it and
00:19:04
Speaker
provide those sadder emotions. When the song ends, for him to create that five seconds of silence at the end was the hardest thing he ever did. We had to practice it over and over and over. But now I got it. Because he just wanted to say, thank you.
00:19:22
Speaker
Instead of setting the tone of that song and just letting it fade and letting people feel the emotion of that piece, it was very difficult for him. Isn't it rich?
00:19:39
Speaker
Isn't it queer, losing my time in this late in my career? Where are the clowns? They're all to be clowns. Well, maybe next year.
00:20:03
Speaker
It's clear that music is really important to Ben, but just how central was it in his life?
Music's Role in Ben's Development and Career
00:20:09
Speaker
Music has always been the one thing that changed Ben's life. So when he was in the hospital, music was the one thing that would calm him down and relax him after surgeries.
00:20:22
Speaker
And interestingly enough, oftentimes the music that we played for him in the hospital then became music that he couldn't listen to anymore after he got home, because it reminded him too much of those hospitalizations when he was very young.
00:20:39
Speaker
And so when he got home, he didn't walk until he was four and a half. Benjamin, when his heart was fixed, his diaphragm was paralyzed. And so he developed scoliosis because he couldn't inflate that left lung properly. And so he was in a back brace and was very slow to develop those skills that he needed for walking.
00:21:02
Speaker
And when he finally did walk, he didn't walk. He marched to the beat of a classic march on the radio. So he couldn't move around. He wasn't out running and jumping like the other kids. Music was Ben's life.
00:21:24
Speaker
When he was 10 years old, we bought him a drum set, and I guess you could say the rest is history. He basically was able to just sit down and play it. He didn't bang on them like other kids do, Ben always put music on and played to the music. It was just a natural affinity for him.
00:21:42
Speaker
and has always played by ear. And I've never seen him play without a smile from ear to ear on his face. When he was younger, he was the ambassador for a local charity. And so often he got to go to their big fundraising events. And as soon as they knew that he knew how to play drums, it became a ritual that he would get to sit in with the band. And he never practiced with them ahead of time. It was always a big band. It was 10 or 15 pieces.
00:22:10
Speaker
And Ben could just sit in and play with them. And that's always been who he is. So he has continued to do that. And as he said now, he attends the Berkshire Hills Music Academy, which is a life skills and transition program in Western Mass.
00:22:26
Speaker
That has a music infused curriculum. So the school is for those who are both musically gifted and those who just love to be surrounded by music and they learn, you know, whether social skills and life skills and all types of skills basically through music. So it's a very unique place.
00:22:46
Speaker
and it's been a wonderful place for him, and now he's through with the school part of the program, but they are his vocational hub, as he said. He goes there to create the set lists and the music that he uses to perform out in the community.
00:23:03
Speaker
I think for those with Williams syndrome, music just enriches their lives. It brings them to a different place. And it's interesting because Williams syndrome for a long time was called the cocktail party syndrome. And that was because individuals with Williams are great at cocktail party banter.
00:23:29
Speaker
Hi, how are you? I love your dress. Oh, did you get your nails done? All of the things that sort of we all say when we get to a party. But they're not able to sometimes go much farther than that into deeper conversations. But ask them anything about music, and they know everything. They can talk.
00:23:49
Speaker
for a very long time about that favorite band or that favorite musical genre, whatever it happens to be about, you know, that they love, they know all of the facts. And so, yes, you know, the kids have sometimes difficulties with deeper conversations about everyday sort of stuff, but about anything that they love.
00:24:14
Speaker
whether it is music or whether it is anything else, they sort of make it their business to learn everything about that. Ben has taught me just so much during his lifetime about
00:24:34
Speaker
how to make the most out of life and how to make the most out of every day. And I think all of us could learn a lot from their teaching because, like I said, they are just these incredible individuals who have a lot of challenges that, as I said, for the most part we didn't talk about today.
Intellectual Challenges and Unique Talents
00:24:57
Speaker
But the gifts that they have, again, whether they be music or any number of other things, and there are lots of things that they're good at, but particularly they're good at bringing joy to others. And so I think that's the message that we need to bring. It's how wonderful it is to have a community of people in this world who are all about bringing joy to others.
00:25:27
Speaker
We've all experienced the desire to jump out of our seats and dance when our favorite song is played, particularly when we're at a concert and the music is live. But most of us inhibit that desire in settings where it's not really appropriate, like in the office or on the bus. That's because our frontal lobes keep our impulses in check. The missing genes in people with Williams syndrome, however, can cause some abnormalities in the frontal lobes and their connections with other brain regions.
00:25:56
Speaker
These abnormalities might explain why individuals like Benjamin sometimes have trouble holding back or curbing their enthusiasm. Now we know that enthusiasm is contagious. It's hard to be in a bad mood when you're talking to someone so full of joy.
00:26:10
Speaker
There's also evidence that the genetics of Williams syndrome affect the amygdala, a set of nuclei in the brain that are important for emotional memories, reactions, and decisions. We often hear about the amygdala in the context of negative emotions, especially fear, but it plays a role in positive ones too. Neuroscientists have found that when they show people with Williams syndrome pictures of socially frightening things like angry or disapproving faces,
00:26:37
Speaker
These individuals show less activation of the amygdala than the general population, suggesting that maybe they just don't react the way we do to these kinds of faces. When the fearful stimuli are not social, however, like a spider or a snake, people with Williams syndrome actually show greater amygdala activation, as they do when shown happy faces.
00:26:59
Speaker
So the change seems specific to social situations. Without social fear, people with Williams syndrome can be as friendly as they want to be. But Williams syndrome comes with big costs. Individuals with the genetic deletions have mild to moderate intellectual disabilities and therefore trouble with daily tasks like keeping track of money or following directions. Their visual spatial skills are particularly affected and they are stumped by puzzles that even preschoolers can solve.
00:27:26
Speaker
About a third of them have phobias, and many have ADHD. And worst of all, they have shorter lifespans because of the heart disease that accompanies the syndrome.
00:27:36
Speaker
These costs are somewhat outweighed by their winning personalities, their kindness, empathy, ability to forgive and to show altruism. When it comes to music, many can play instruments without having been given explicit instructions, and they are more likely than the average person to have absolute or perfect pitch. And the majority of them, like Benjamin and Eli, have a deep love for music, even in the face of misfunctions in their auditory nerves.
00:28:04
Speaker
It's so easy to label a person with a syndrome and to lump deficits together without seeing what is unique about each individual. But by understanding how music affects people with Williams syndrome, we get to see a deeper side of their humanity. Through music, we can see how refined, thoughtful, and insightful they are about human behavior.
00:28:26
Speaker
Benjamin might not be able to complete a jigsaw puzzle, but he can explain why Send in the Clowns is one of the most profound songs ever written. And by doing so, he can connect us with a part of ourselves that we often bury in daily life.
Podcast Creators and Support for Cadence
00:28:46
Speaker
Thank you for listening to this episode of Cadence. You can find us online at the ensembleproject.com slash cadence at Facebook slash cadence podcast and on Twitter at cadence podcast. You can also get in touch with us at cadencemind at gmail.com.
00:29:02
Speaker
Cadence is produced by Adam Isaac and me, Indre Viscontis. I also created and write the show. You can find me on Twitter at indrevis. Additional production help comes from Scott Lowry. Music in this episode was provided for us by acclaimed New Zealand composer, Rian Sheehan. Check him out at riansheehan.com. The music accompanying Ben Singing was composed and produced by San Francisco composer Matthew Slayton. You can find him at matthewslayton.com.
00:29:28
Speaker
This episode also featured the song Beach Party by David Sesley. Cadence is generously supported by the Germanicos Foundation. And you can support us by writing a review for us on iTunes or wherever you get your podcasts, or by going to patreon.com slash cadencepodcast. Join us in two weeks for our next episode in which we continue our exploration into what music tells us about the mind.