This Rare Condition Causes Extreme, Insatiable Hunger

The Rao family in Maryland knew that something was different about their younger daughter, Ayoni, almost as soon as she was born. But it took them until she was five to get a diagnosis of Prader-Willi Syndrome, a rare genetic condition that causes extreme, insatiable hunger. Food needs to be locked away from many of these patients, or they might eat until their stomachs actually rupture.

Speaker names marked ? are inferred from speaking time and the show’s known hosts — they are not voice-verified.

• 0:00Dan Pashman?

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• 0:44Dan Pashman?

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• 1:15Speaker 01

  I only want to taste the dal.

• 1:21Speaker 02

  Need anything?

• 1:23Speaker 01

  No, not anymore. So, I've been less than a boss time.

• 1:26Dan Pashman?

  What happened last time?

• 1:29Speaker 01

  My mom added too much salt.

• 1:31Dan Pashman?

  Oh.

• 1:32Speaker 02

  Why did I add too much salt?

• 1:33Speaker 01

  Because I thought I needed my salt.

• 1:39Dan Pashman?

  This is the Sporkful. It's not for foodies, it's for eaters. I'm Dan Pashman. Each week on our show, we obsess about food to learn more about people. You know, on this show, we talk a lot about the whole spectrum of people's relationships with food. Sometimes it's positive, like memories of being in the kitchen as a child, or connecting over a meal with someone. Sometimes it's more about difficulties with food, like disordered eating or food insecurity. Today, we're discussing one very particular relationship to food that we've never covered on the show. It's caused by a rare genetic condition called Prader-Willi syndrome. Prader-Willi is a complex syndrome that causes issues with sleep, cognitive function, and more. But perhaps the most disruptive aspect of it is something called hyperphagia, or excessive hunger. People with Prader-Willi never feel satisfied

• 2:22Dan Pashman?

  from eating. That hyperphagia can be extreme, and it can lead to extreme behaviors. Last year, a new medication was approved, which has helped. But life with Prader-Willi can still be very challenging. Today, we'll talk with two people who have the condition, as well as some of their family members, to get a real sense of what it looks like on the ground. Recently, I went to Maryland and met the Rao family. Deanie Rao says for her, as a kid...

• 2:45Speaker 02

  Food was everything. I grew up the million. And for us, the people, it's like, food is who we are. It is our identity. You know one family from the other, what religion they are, what caste they are, by how they make their rice. You know, my family has a long, beautiful history of great cooks. My grandmother was an incredible cook. I grew up cooking in my mom's kitchen and things like that. And so, she would have me be the taste tester. And I would tell her, no, you didn't toast the cumin, or you forgot to add coriander to this and whatnot. I was precocious like that.

• 3:22Dan Pashman?

  As a young woman, Deanie got really into wine. She became a sommelier and went to culinary school, where she studied with Julia Child and Jacques Papin. When she and her husband Anju got together, they bonded over food.

• 3:32Speaker 03

  I mean, I love food. Like, we both love food. We love cooking. It was something that I thought, oh, it's always going to be part of our lives.

• 3:40Dan Pashman?

  After their first daughter, Neela was born...

• 3:43Speaker 02

  We had all these traditions where we went apple picking every year, and we made an apple pie, or apple crumble, and you know, and that's how I also passed down my culture to her. Because I couldn't pass down the language, I'm not strong enough in it. So instead, like, I brought her up on dal, and you know, mendicaille, which is okra, all of my traditional dishes.

• 4:03Dan Pashman?

  But when their younger daughter, Ioni, was born, they noticed she was very different from Neela.

• 4:07Speaker 02

  Ioni just would not eat anything. And so, we were terrified. She was dropping weight. So, we had to take little droppers, little spoons, and feed her one ounce at a time. And it would take an hour to feed her that ounce. She was missing all her milestones, and we knew something was going on, but we didn't have a diagnosis. And then, as she grew, all sorts of other complications formed. Do you know, she had some developmental delays. She was later to walk and talk and things like that.

• 4:36Dan Pashman?

  As Ioni got older, she did start to eat more normally. But Deanie noticed, even though Ioni and her older sister ate the same food, Ioni gained more weight. By the time she was four, Ioni was becoming obese, and the family didn't know why. At the time, they were living in New York, home to some of the best hospitals in the country. They sought treatment and therapies for Ioni there. They also traveled to other cities, searching not only for treatment, but also for a diagnosis.

• 4:59Speaker 02

  You're just pulling your hair out. You're like, what is going on? You know, I feel like the worst parent ever. Like, my child is getting worse and worse. These doctors are basically gaslighting you and telling you nothing's wrong, or there's nothing we can do. And you just keep searching and keep trying to find something.

• 5:14Dan Pashman?

  The Rao's eventually moved to the DC area, where they went to Children's National Hospital.

• 5:18Speaker 02

  And we finally got a doctor who kind of believed in us and said, you don't have a diagnosis? You should absolutely have a diagnosis. Let's run more tests. Let's figure this out.

• 5:28Dan Pashman?

  After all those tests, the doctor finally came back to Deanie and Anju with an answer.

• 5:33Speaker 02

  And she said, your daughter tested positive for Prader-Willi syndrome. And here are all the things. Here are all the things that she won't be able to do. She's going to have this endless hunger that, you know, food is going to have to be locked up all her life. She'll never live alone. She'll never, you know, go to typical school. All of these things that basically crush your whole world. You don't even know what to feel. I mean, it's just complete denial in the beginning, because you think to yourself, like, this just can't be, like, no, my daughter is great, and she's doing well, you know, in some regards. And, and I think never in our wildest dreams did we imagine a scenario where things would get worse. We just kept thinking, like, this is what it is.

• 6:14Dan Pashman?

  It's just a phase. She'll turn the corner at some point.

• 6:16Speaker 02

  She'll turn the corner with a diagnosis. Maybe we'll get some therapies, and things will be great, and they'll be better. But it was so devastating. It was so scary, because especially, all you read on the internet at the time is terrible news.

• 6:30Speaker 03

  My initial reaction was just the ground shaking underneath my feet, right? Like, everything I thought was not going to be the way it was going to be. And everything I feared almost was coming true.

• 6:49Dan Pashman?

  Faced with this diagnosis, the Rao's found the top Prader-Willi specialist in the country.

• 6:54Speaker 04

  My name is Jennifer Miller. I'm a professor of pediatric endocrinology at the University of Florida.

• 6:59Dan Pashman?

  Dr. Miller explains that Prader-Willi is caused by the deletion of some of the material on chromosome 15. It's not hereditary. It's just a random event that happens. Chromosome 15 is crucial to the development of the hypothalamus, the part of the brain that regulates hormones, body temperature, sleep, pain, and sex drive. These days, most Prader-Willi cases are diagnosed within the first couple of months of the child's life, based on having low tone, a complete lack of interest in eating in infancy, and other factors. But as Deanie said, Ioni wasn't diagnosed until she was five. This is partly because the syndrome wasn't as well-known when she was born, and partly due to a bias in what doctors are taught about the disorder. Historically, the literature suggested that Prader-Willi patients are more likely to have blonde hair and blue eyes. In reality, that's true for white Prader-Willi

• 7:42Dan Pashman?

  patients, but not for patients of color. As soon as a Prader-Willi diagnosis is made, the first course of treatment is human growth hormone.

• 7:49Speaker 04

  And what data has shown, as time has gone by, is that early treatment with growth hormone improves cognitive function, improves motor milestone acquisition. You know, it improves a lot of things. Obviously, growth hormone improves growth and height and, you know, that kind of stuff. But the bigger thing, in my personal opinion as a physician, is that it improves IQ. It improves cognitive function. And I think that's really critical. Now, is it going to mean that they're totally, completely cognitively unimpaired? No. But it helps. And that's a really huge thing. That's been a dramatic change in care since I started doing this 27 years ago.

• 8:30Dan Pashman?

  As we heard, in the first phase of Prader-Willi, the newborn refuses to eat. Usually, after a year or two, the child's appetite will normalize, but their metabolism is very slow. So even when eating the same diet as a typical child, the child with Prader-Willi will gain more weight. That's exactly what happened with Ioni. Then, somewhere between eight and 13 years old, the child will develop hyperphagia.

• 8:50Speaker 04

  One of the patients one time described it to me as, she said, imagine the hungriest you've ever been. That's how I feel all the time. Their brain does not recognize that they have eaten. And so, they're constantly thinking about food, trying to get to food. It can lead to extreme behaviors, to anxiety, to self-harm behaviors. One of the ways that they can pass away is, if given unlimited access to food, they will just eat and eat and eat to the point where their stomach can't handle it. So it actually will end up ripping their stomach.

• 9:20Dan Pashman?

  Dr. Miller says patients' hunger can feel so strong that when they can't get food, they may lash out.

• 9:26Speaker 04

  I have been held up against the wall by my neck more times than I could tell you when I try to take away food from a child.

• 9:32Dan Pashman?

  Wow.

• 9:32Speaker 04

  Yeah, it's very scary, actually, when it's, you know, a young adult male or an adolescent male. Many, many, many times during our days, my dietitian, who is a male, and I, you know, are on the floor on top of a patient who's having a meltdown because they're trying to beat up their mother, when we say, we're not going to let you eat this anymore, or you can't do this anymore. And they go after the mom. And we, to protect the mom, you know, have to sort of take the brunt of it ourselves.

• 10:01Dan Pashman?

  A lot of patients are on psychiatric meds to control that impulsive or aggressive behavior. But parents and caregivers still have to take other measures to prevent these patients from stealing food.

• 10:10Speaker 04

  You lock the kitchen, and you lock the refrigerator, and you lock the cabinets. And you make sure that the child is never left alone, ever. 24/7 supervision. They're often locked in their bedrooms at night. Parents will turn the door handles around so that they lock from the outside, so that they can lock them in, so that the kids can't get out in the middle of the night in sneakers, steal food. At school, there's an aide that's constantly with the child to watch them and make sure they don't get food. It's not an easy life.

• 10:40Dan Pashman?

  Dr. Miller says the hyperphagia stems from a disconnect between the gut and the brain. In someone with Prader-Willi, the hypothalamus doesn't form normally. So when the gut sends signals to the brain to say you're full, the receptors in the hypothalamus don't respond the way they should. They don't register that message. Now, you may be thinking, what about GLP-1s? Can they help control patients' appetites? Dr. Miller says no. You see, GLP-1s work by sending extra signals from your stomach to your brain to tell your brain that you're full. But with Prader-Willi patients, since the receptors for those signals don't work correctly, it doesn't matter how many messages you send from the stomach. The brain won't receive them. But there is a new drug that was approved for Prader-Willi just last year. It's called Vicat. Dr. Miller was one of the lead researchers involved with

• 11:25Dan Pashman?

  clinical trials for it.

• 11:26Speaker 04

  It works both in the brain and in the gut. It helps decrease temper tantrums and behaviors, decreases aggressive behaviors, decreases anxiety. So a lot of improvements with this medication in terms of their quality of life. It obviously doesn't work for everybody. No medicine works for everybody, right? So we're continuing to do research to look for additional medications that may work.

• 11:52Dan Pashman?

  So as you said, doctor, you've been working on this syndrome for 27 years. What is it about it that has sort of has captured your interest so much that you've devoted your career to it?

• 12:03Speaker 04

  These are, in general, very lovely, lovely people who have goals and aspirations and dreams, just like everybody else. And they are so limited because, I mean, food is everywhere. You just want to help. You just want to be able to say, I want you to be successful. I want you to be able to achieve your goals and dreams. And so when I started doing this, there was no such option for any of these individuals, none. And that is what kept me going, was my goal was always to change that.

• 12:36Dan Pashman?

  Coming up, I learn more about what happened when Ioni entered the hyperphagia phase of Prader-Willi, and I eat dinner with her and her family. Then later, I meet an adult living with this syndrome. Stick around.

• 12:53Speaker 05

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• 13:00Dan Pashman?

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• 13:43Dan Pashman?

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• 14:02Dan Pashman?

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• 15:30Dan Pashman?

  know it was The Sporkful. It's the best way to support the show.

• 15:36Dan Pashman?

  Welcome back to The Sporkful. I'm Dan Pashman, and I want to tell you about a super cool live event we have in the works. We're doing a run of shows at Little Island in New York, which, if you haven't been there, it's a beautiful park on a pier on the Hudson River. It's got performance spaces, food, and more. Now, these shows we're doing are going to be free, no tickets required. You can sit on the grass with a view of the water and hear us tell a story entitled "The Great Yogurt Feud." It's very dramatic. And yes, there will be free samples. These shows are in just a couple of weeks, Friday through Sunday, August 28th through 30th. Same show every evening. So come on down to Little Island. Get more details at sporkful.com/live. See you there. Also, be sure to stick around to the end of this episode to hear my family and me eating our way across Myrtle Beach.

• 16:18Dan Pashman?

  Okay, back to our story about Prader-Willi syndrome and the Rao family in Maryland. When Ioni got her diagnosis at age five, she hadn't yet entered the hyperphagia stage. But her parents, Deanie and Anju, knew it would be coming in the next few years.

• 16:32Speaker 02

  You also hear all the horror stories from the other families. And so you know even what your experience is, is so scary, but that it can only get worse. You know, we heard of horror stories of kids like leaving and taking off from home and begging in the streets for people to give them food because they say their families don't feed them. And, you know, we just think of like the safety risk. So all of a sudden, the whole world becomes kind of dangerous for your child because it's all filled with food.

• 17:01Dan Pashman?

  Then, when Ioni was eight, the hyperphagia set in.

• 17:04Speaker 02

  So I'll never forget the first time after we had fed Ioni a full dinner, and she told me, Mama, I'm still hungry. I want more food. And it like broke my heart not to say like, okay, because, you know, Neela will tell you, like, I'm always saying, like, have more food, have more food. I want to give you that. Are you full? Are you, are you satisfied? Like, that's the, that's the way we love. It felt like I am a terrible mother because I can't provide for my child. I can't give her that satisfaction of feeling full.

• 17:34Dan Pashman?

  And now that you've learned more about this syndrome, do you still feel that way sometimes?

• 17:40Speaker 02

  Sometimes, yeah. Because there is your, like, you know, evolved brain.

• 17:45Dan Pashman?

  Your intellectual brain.

• 17:46Speaker 02

  Exactly. And then there is just your, like, primitive self of, like, who you are as a mother, as a caregiver. And I don't think I'll ever get over that. What do you tell me after I give you a full dinner and you've had everything?

• 18:00Speaker 01

  I'm still hungry.

• 18:01Dan Pashman?

  This is Ioni. She's 10 now.

• 18:03Speaker 02

  You're still hungry. And so that leads to behaviors like stealing food a lot of times, which I know is not your fault. But she'll steal food from the kitchen, from classmates, from trash cans at school. She will hoard things. So one time, we found a whole pile of cough drop wrappers in her room, and she'd obviously eaten them all and probably eaten them all in the same sitting. These are things that you can't control. It's just your body thinks it's starving, and so it will do whatever it can to get food.

• 18:37Dan Pashman?

  Like many families with someone with Prader-Willi, the Rous had to change the setup in their house because of Ioni's condition.

• 18:43Speaker 02

  All the food that isn't refrigerated goes in that pantry, and it is locked. And the refrigerator is something we haven't yet locked, but there are many times where she's had something she's not allowed to have. And she's not allowed to have any food outside of her prescribed diet, but we haven't locked it yet.

• 19:02Dan Pashman?

  Why not?

• 19:03Speaker 02

  It's actually a big debate. And this is one of the tricky things when you're in this field. It's not clear what you're supposed to do. Dr. Miller feels that it's better that we don't lock up things so that she can get used to living in a world where she's going to have to manage that maybe one day she could live on her own or live on her own with AIDS and things like that. And she's going to have to get used to those temptations.

• 19:26Dan Pashman?

  These aren't the only accommodations the family's made. They also have to be very regimented about the way they eat.

• 19:31Speaker 02

  So each night has a schedule. And we do that because she asks so many questions. So people with Prader-Willi, like, perseverate. They'll just ask questions over and over, especially about food. So if we have a set schedule, it can kind of reduce the anxiety. Monday has to be oatmeal for breakfast. Then there's always a morning snack that happens at 10:30. There's lunch that happens at 12:30, an after-school snack, and then dinner. And Monday night is dinner bowls.

• 19:58Dan Pashman?

  On the day I'm visiting, it's a Tuesday. I ask Ioni, what did you have for breakfast, Ioni?

• 20:03Speaker 01

  Uh, what did I have?

• 20:05Speaker 02

  Every Tuesday?

• 20:06Speaker 01

  What did you have?

• 20:08Speaker 02

  You make?

• 20:09Speaker 01

  Oh, I had half an English muffin with peanut butter and sausage.

• 20:15Dan Pashman?

  That sounds good.

• 20:16Speaker 02

  Mm-hmm. She actually invented something called...

• 20:18Speaker 01

  Well, I didn't do that today.

• 20:20Speaker 02

  You didn't do that today? First Tuesday ever. But what did you invent?

• 20:24Dan Pashman?

  Yeah, tell me about it.

• 20:24Speaker 01

  But I don't always do it.

• 20:27Speaker 02

  Okay. But what is it?

• 20:29Speaker 01

  I take food and put food on it.

• 20:32Speaker 02

  And you call it a?

• 20:35Speaker 02

  Fruit buddy?

• 20:36Dan Pashman?

  A fruit buddy?

• 20:38Speaker 02

  A fruit buddy.

• 20:39Dan Pashman?

  Okay.

• 20:39Speaker 02

  Like your pal and she makes these little faces.

• 20:42Dan Pashman?

  The nutritional makeup of meals is important because Prater Willie patients have that much slower metabolism. Ioni typically eats about half as much as her family members to prevent excessive weight gain and health problems.

• 20:52Speaker 02

  So pretty much every meal needs to be majority non-starchy vegetables, protein, and a little bit of whole grain along with it. And this is where the culinary school does come in handy because I don't think anyone in the family complains about the food we have. Because I try to take her criteria and make it into really tasty meals. Nowhere in the litany of South Indian meals is there brown rice, but we will have the brown rice instead of the basmati rice. And then, you know, I'll add in extra dal to the green beans, you know, we make sure we have a lot of protein.

• 21:24Dan Pashman?

  Ioni can have only have so few calories, but you all need and want more calories than that. But like, is that ever an issue? Like, like you're allowed to have a second helping, but she isn't.

• 21:35Speaker 02

  Oh, yes. So we had to bake in the second helpings to her schedule. So we, I now give her less food on purpose with the first meal so that she always gets seconds. And she knows, she, she can count on that. She's always going to get seconds of the vegetable and the protein, no seconds of the, you know, whole grain.

• 21:55Dan Pashman?

  But still, like, I assume that the total quantity of food is less than what the rest of you are getting. And does she pick up on that? Is that an issue?

• 22:01Speaker 02

  Not usually. She is pretty used to the fact that like, this is her portion. We talked to her about like fairness isn't everybody getting the same thing. Fairness is everybody getting what they need and what they, you know, are supposed to have. And so we all have different needs and we all eat different amounts. Like she knows that Dada gets more food than her, but she kind of has this belief that I'm supposed to eat the same things she does. So like she will get, she'll be okay if she sees the two of them eating something sweet, but she'll be like, well, you and I can't have that, right, mama?

• 22:34Dan Pashman?

  So Ioni, do you feel hungry right now?

• 22:36Speaker 01

  No.

• 22:38Dan Pashman?

  Why not?

• 22:38Speaker 01

  Not now, because I had my snack.

• 22:40Dan Pashman?

  Oh, okay.

• 22:41Speaker 01

  A little while ago.

• 22:43Dan Pashman?

  Ioni is on the Vicat medication that Dr. Miller told me about. It's helped with the hyperphagia, but while Ioni is less hungry, she's still very preoccupied with food. For example, I noticed Deanie left some green beans out next to Ioni while we were chatting.

• 22:56Speaker 02

  I was actually thinking that was a big mistake I made because normally I wouldn't do that. Cause you're right, she didn't reach out and eat them, but she was probably very distracted by them. Because her mind is always thinking about food. Even now with the medication she's on, which is the reason why she didn't feel compelled to eat it, it has made things a lot better. And yet, no matter what, you know, when she's talking about things, she's been talking for days about meeting Dan Pashman. And with that is what are we going to eat again? What are we cooking again? You know, there's this huge fixation on food. And with this new medication, she can feel a little bit of that fullness. She won't feel hungry all the time, the way she used to.

• 23:40Dan Pashman?

  So as we said, the medication isn't a cure all, but it does help. The Rouse still have to think constantly about how to make the world safer for Ioni, including at school.

• 23:48Speaker 02

  They said to us, very frankly, we won't be able to control her access to food. We won't be able to lock up the trash cans. We won't be able to lock up the school lunches. She'll have to eat in a room by herself and all these things. And we just thought that sounds awful. And I won't put my child in danger.

• 24:06Dan Pashman?

  Deanie and Anju ended up sending Ioni to a school that could make accommodations for her.

• 24:11Dan Pashman?

  We've talked a lot about how Prater Willie affects Ioni and her parents, but Dr. Miller told me it can also have a big impact on siblings. Here's Ioni's older sister, Neela.

• 24:20Speaker 05

  I remember going over to her friend's house and we had dessert there. And then I came back home and I was like, why don't we have dessert? Why don't we, why don't we do that? And then my parents told me Ioni has like a very special mind. She has a very special diet. And so this is what she can and can't eat. And because we are the people that are like directly in her life the most. This is what we need to do to make her feel like she's in a safe environment, to make her feel appreciated and loved and to make her feel like she can be herself.

• 24:54Dan Pashman?

  How, how did you feel about the explanation?

• 24:57Speaker 05

  I didn't get it at first. I was eight. I was a second grader. I was angry that like, oh, I can't have dessert. But yeah, now I think I understand it more. And I think like a couple of weeks after that, I started feeling bad for my sister. Cause I understood like, yes, I can have more things than her. Whenever I ate something that she couldn't have, even if she wasn't in the, directly in the room with me, I would just think about her and I'd be like, oh, shoot. She can't have this. Should I not be able to have this either? Should I just like give all this up myself?

• 25:31Dan Pashman?

  And where did you land on that question?

• 25:34Speaker 05

  I decided not to. I just.

• 25:38Speaker 05

  I just decided that I would just try to not eat the foods that she couldn't eat when she was in the same room as me. It's like, I tried to be sensitive about it, but you know, I'm a kid. I truly cannot live without sugar.

• 25:51Dan Pashman?

  Yeah. Still, Ioni's condition is always on Neela's mind, even at school, when the lunch being served is something I only wouldn't be able to have.

• 25:59Speaker 05

  The logic is there. I can eat this. She's not around. But I know that she wouldn't want me to eat this in front of her. So sometimes I just like skip lunch altogether, just because like I don't, the instinct not to upset her, not to make her feel like she's missing out is still there.

• 26:17Speaker 02

  There's a lot of research and many of the siblings actually, and I would imagine parents experience eating disorders. Because there are so many controls around food, like we have a food schedule and there's only food at those times. And you know, there's so many rules about what can be eaten in front of her or not. So I can imagine that that feeling you had of like hesitation, even when she wasn't around of like, am I allowed to eat this thing? You know, it creates all these unnecessary feelings really associated with food. So we make sure to kind of work on those things too.

• 26:58Speaker 02

  I only copy. I gotta do some cooking?

• 27:00Speaker 01

  Yeah.

• 27:02Dan Pashman?

  Deanie has graciously invited me to cook with them and stay for dinner.

• 27:05Speaker 02

  And are you a wine drinker?

• 27:06Dan Pashman?

  I'll have a tiny bit. I'm not a huge wine drinker, but seeing as you're a wine expert, I have to.

• 27:10Speaker 02

  You don't have to.

• 27:11Dan Pashman?

  Well, but I, I, I, I'm sure you're, I'm sure, I bet you have good wine in your house and you'll pick a good wine. So I'll, I'll take like half a glass. What's on the menu?

• 27:20Speaker 02

  We are going to have a go in pork and a really nice spicy gravy. We're going to have dal tadka. We're going to have some green beans and like a simple like curial. And then we're going to do some lemon rice, not with basmati rice, with brown rice.

• 27:34Dan Pashman?

  And a major star of this meal will be curry leaves. Oh, you're from the garden. Phenomenal. We're going to, we're going to cut freshly cut curry leaves. Is that right?

• 27:45Speaker 01

  We always want to go for the lower branches. Wait, mom, the lower branches?

• 27:51Speaker 02

  The upper. We want to leave some shoots below it. So here, how about you cut right there?

• 27:57Dan Pashman?

  Curry leaves are one of my very favorite things.

• 27:59Speaker 02

  Right.

• 28:00Dan Pashman?

  Can I smell?

• 28:00Speaker 02

  Yes. Especially when they're first.

• 28:03Dan Pashman?

  Fresh picked. Oh my, that is.

• 28:06Speaker 01

  Mmm.

• 28:07Dan Pashman?

  How does that smell to you, Ioni?

• 28:08Speaker 01

  Good.

• 28:08Dan Pashman?

  Yeah, smells good to me too. Ioni is in charge of making the raita, a yogurt sauce with cucumber, lots of spices and those fresh curry leaves. So it looks like Ioni knows how to make the raita. I don't know that you need much help from me. Ioni, what do you think is the secret to making great raita?

• 28:25Speaker 01

  Curry leaves.

• 28:26Dan Pashman?

  Mmm. Deanie asks Ioni to do a taste test. Is Ioni one of your, is she your primary taster?

• 28:33Speaker 02

  Oh, yes. Big time. Because she does have an excellent memory for all things food. So she really does notice when there is something different or off about it.

• 28:41Dan Pashman?

  So she serves the role in the kitchen that you served?

• 28:44Speaker 02

  That's right. Exactly. Passed it down. Brown rice, and then we're taking frozen rice. Do you know about how the starches become more digestible for you if you use frozen rice?

• 28:55Dan Pashman?

  I read about that study and then completely forgot to incorporate it into my own life.

• 29:00Speaker 02

  As one does.

• 29:01Dan Pashman?

  Yeah.

• 29:01Speaker 02

  We read those studies and we're like, yes.

• 29:03Dan Pashman?

  Right, right, right.

• 29:04Speaker 02

  This means we can have frozen rice all the time.

• 29:06Dan Pashman?

  Yeah. Well, the food's getting plated up. It looks phenomenal and everything smells like curry leaves, which is the best smell. Do you get excited when you see your plate, Ioni? I get excited when I see my plate too. All the food is amazing. The pork is so tender and perfectly spiced. And one move that I never saw before, Deanie toasts lentils in the pan before mixing in the rice. She does it to add protein for Ioni, but I love the flavor and the little crunchy bits it added. I started doing that at home for myself.

• 29:36Dan Pashman?

  After dinner, Ioni and I are left at the table to chat. She tells me about her friend who she is appalled to say, doesn't like brownies. She says in a choice between brownies and milk, he would choose milk. This leads me to ask Ioni. Which one would you have if you had a choice between brownies and milk?

• 29:53Speaker 01

  What about you?

• 29:55Dan Pashman?

  I think probably brownies too, but maybe I would have a little bit of brownie with with some milk.

• 30:00Speaker 01

  But you have to pick one.

• 30:01Dan Pashman?

  Oh, okay. I guess brownies just like you. Yeah.

• 30:06Speaker 01

  What if you have to pick?

• 30:07Dan Pashman?

  This gets us chatting about foods we don't like. And I was surprised by something that, in retrospect, seems obvious. There are foods Ioni doesn't like. So in our next round of Food Would You Rather, I decided to give her a hard one. Two foods she's already told me she can't stand. So should I give you honeydew melon and millet porridge? You have to pick one.

• 30:30Speaker 01

  Neither. Neither.

• 30:34Dan Pashman?

  Okay, all right.

• 30:34Speaker 01

  If you gave me an entire table of it, you know, I'd do?

• 30:39Dan Pashman?

  What?

• 30:39Speaker 01

  I would probably

• 30:43Speaker 01

  put it on the floor so I would leave a big mess for you to clean up.

• 30:48Speaker 01

  Find everything in your guys' fridge so I could put it on the floor, make the biggest mess, run away,

• 30:57Speaker 01

  catch on no plane and go to different country. So I'll never be able to find me.

• 31:04Dan Pashman?

  Before I leave, I asked Deanie and Anju about their hopes for Ioni a few years from now.

• 31:08Speaker 02

  I hope there'll be more therapies. And also, she's still not at a point where she could live independently. And she may or may not want that. You know, right now, she says, no, she wants to live with me forever. But um.

• 31:21Dan Pashman?

  But she's 10, so that's right.

• 31:23Speaker 02

  One hopes that there's a time where, you know, like, whatever she wants to do in life, whatever kind of life she wants to lead, like, will be able to support her in that. And I don't think we're there yet. So with rare diseases, there's so much research that needs to be done.

• 31:39Speaker 03

  I want her to feel

• 31:43Speaker 03

  not like she's a victim of this disorder, right? That she feels comfortable going into spaces and being able to navigate. And I think that's the thing that I would hope, right? Because it's one thing to be able to walk by a bowl of food and not immediately grab it and start eating it. It's another thing to like, go out with friends and like, be able to do that and feel comfortable, like, okay, I can't have that. I can only have this.

• 32:10Speaker 02

  Something that I thought I had to grieve for, I thought I wouldn't be able to have my food. And now, I just, you know, want to say to any families out there who are facing this diagnosis or think that they can't do that, there are ways to do that. And there are ways to customize everything. And so, there's hope. You will have your culture. You will have your food.

• 32:34Dan Pashman?

  My thanks to the Rao family in Maryland, Deanie, Anju, Neela, and Ioni. After the break, I'll talk with an adult living with Prader-Willi. Stick around.

• 32:50Speaker 04

  Sate, you stay.

• 32:53Speaker 04

  Reese Witherspoon here, checking into my favorite hotel in Paris. Bonjour. Bonjour. I'm traveling with my Wells Fargo Autograph Journey Card, so I earn rewards wherever I book travel. Five times points with hotels, four times with airlines, three times on restaurants and other travel, and one point on other purchases. Phew, that was a lot. I need to lie down. Is the room ready?

• 33:16Dan Pashman?

  Visit wellsfargo.com/autographjourney. Terms apply.

• 33:22Speaker 06

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• 34:10Speaker 05

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• 34:30Dan Pashman?

  Welcome back. The Rous are still early in their journey with Prader-Willi, but Zaid Anwurzai has been living with it for 25 years. He's from Northern Virginia. He lives at home with his parents and their dog. With the supervision of an aide, Zaid works at a nearby farm that employs people with disabilities. He also acts in plays at a local community theater. When Zaid's hyperphagia started, the Vicat medication wasn't even in clinical trials yet, so he didn't have anything to help keep the hunger at bay. Now he is on Vicat, but he says he's not sure how much it's helping.

• 34:59Speaker 04

  I get hungry a lot, and sometimes it's hard to control it. I would say that I get the constant urge to eat. And I feel like sometimes it angers me. I also tend to get ADHD, OCD over the fact of food. Sometimes I tend to focus more on the food than the activity that I'm supposed to be focusing on. Sometimes this happens due to my syndrome, and I've learned to work with it as I have matured from childhood to now, you know, being 25, almost 26.

• 35:35Dan Pashman?

  Are you able to sort of talk to yourself? And say, okay, I'm thinking about food, but I have to turn my attention back to this other thing?

• 35:43Speaker 04

  If I get hungry, what I'll try to do is I'll try to tune in with my self-intuition, and I'll try to, you know, positively reinforce my thoughts so that I could try to work with my hunger as, you know, it can be difficult sometimes. I take medications and stuff to help with the anxiety in Prader-Willi in general.

• 36:04Dan Pashman?

  What do you say to yourself when you're you're feeling hungry and you know you should be focused on something else?

• 36:09Speaker 04

  Well, first of all, you know, I tell myself that I'm not perfect. And of course, I'm gonna have my faults, but I am just the way that I am. I'm Zaid. And I'm just the way that I am, and this is how you see me. I am my own individual in my own life.

• 36:29Dan Pashman?

  Like Ioni and her mom, Zaid often cooks with his mom. Dr. Miller says involving Prader-Willi patients in the kitchen lets them focus on food in a constructive way. And since they're under supervision, it gets them used to being around food without eating it.

• 36:42Speaker 04

  I like to cook with my mom. I'm kind of her sous chef, so I help her to cut the vegetables, I help her to cut meat or grill over whatever she needs. I'll like, do it. So whenever we're out grocery shopping, I get to shop. I get to choose what I want, you know, and they have to be, you know, within my diet, within reason. Like, yesterday, you know, um, we were out shopping to get me stuff for my lunch the next day that I wanted. And my dad thought I was gonna get a lot, but I only ended up getting just one protein shake and one energy drink and also just one or two protein bars, and that's it.

• 37:27Speaker 04

  There are days where it's hard for me to control my hunger because, you know, sometimes the hunger is really urgent. You know, basically, my mind is telling me, hey, I want to eat more. I want to eat to the point where I'm full. But clearly, that's not the point. Clearly, sometimes what happens is that my cravings don't get satisfied. The hyperphagia of it

• 37:54Speaker 04

  is that, hey, I'm feeling a way that's not me.

• 37:59Dan Pashman?

  It almost sounds, Zaid, as if there's sort of like a struggle between your brain and your stomach. Because in your brain, you obviously understand, Prader-Willi, you understand that you have this condition that affects your appetite, and you're able to sort of talk to yourself. But then sometimes your stomach gets very loud in what it's saying, and then it's hard for the brain to talk back to the stomach. Does that sound, is that a good way to think of it?

• 38:25Speaker 04

  Well, yeah, I think that's a great way to summarize. Because that's exactly how, you know, it tells me. My stomach tells me, hey, I'm hungry.

• 38:36Speaker 04

  You know, I need something to eat. Feed me something that will satisfy me. But sometimes the harsh reality is I have to eat healthy. That's what's gonna help me, and that's what's gonna get me successful. I don't want to get to the point where I'm gonna get obese. And then, you know, have, you know, God forbid, you know, type 1 or type 2 diabetes.

• 38:59Dan Pashman?

  Zaid isn't yet at the point where he can be left unsupervised. He'll try to eat something he shouldn't. His mom says he sometimes tries to convince people he meets to give him food. Dr. Miller says that since Vicat came into the picture, some of her patients have transitioned to living on their own. Some even graduated college. One ran an Ironman. She doesn't think there's likely to be a cure for Prader-Willi anytime soon, but she's optimistic that more treatments will be developed. I was curious to ask Zaid where he hopes to be with Prader-Willi in the future.

• 39:25Speaker 04

  I hope to see in five or 10 years that, you know, that I'm doing better and that this Vicat is having, you know, full range and that it's on full effect on me, that it's going really well, and I hope to get married in five or 10 years and have a wife. And, you know, get a dog. I also want to kind of like live free spirited. I guess

• 39:49Speaker 04

  more free. Because I want to be able to make choices on my own. I want to like live a life. I want to be independent. That's what I want to do because I want to be normal. I like to say that I am normal, but not normal. You know, I'm not normal, but, you know, in reality, I'm just a normal person, you know?

• 40:09Speaker 04

  I'm just me. I'm just Zaid. You know, that's the way I am.

• 40:13Dan Pashman?

  And when I talked with him, it was clear that Zaid is a lot more than a collection of Prader-Willi symptoms. He works, he has hobbies, he's been in the Special Olympics and won several medals for swimming. Before he started taping, he wanted to be sure that in addition to answering my questions, he'd also have a chance to share something of his own.

• 40:29Speaker 04

  Here's a poem I especially wrote for all those dog enthusiasts out there. Dogs.

• 40:40Speaker 04

  Dogs, dogs, dogs. Dogs entice my enjoyment till my clock-shaped heart is complete. Play, play, play is their perspective on life. Their smiles gazing hazily beyond their precious personalities. Serendipity, serendipity, I'm free. Where's my bone? Where's my food? Where's my water? Oh, whatever.

• 41:15Speaker 04

  I'll play till dawn and sleep at times my curiosity messes with me. Dreaming, dreaming about what is yet to come.

• 41:26Speaker 04

  When the birds sing, when the rooster calls, I'll do it all over again. I'll do my dog duties and I'll play, play, play. Play till my owner shouts, "Get back, you big old stinker."

• 41:43Speaker 04

  So therefore, when that happens and know what's yet to come.

• 41:49Speaker 04

  My big bowl of dog food and water, a meal fit for a dog, but no ordinary dog. A companion, a friend. An endless dog with, with special qualities every which in their own way. Dogs, dogs, dogs. The sweethearts of the Brady Bunch. Thank you.

• 42:18Dan Pashman?

  Thank you so much. That was so good.

• 42:20Speaker 04

  Thank you so much.

• 42:35Dan Pashman?

  That's Zaid on Warzai in Virginia. We also heard from Dr. Jennifer Miller at the University of Florida. Deanie Rao also started an organization called Colors of Hope, specifically to help families of color who are living with prouder Willie. Their website is pws colors of hope.org. Next week on the show, we investigate a murder. The murder of the Florida orange. While you wait for that one, check out our recent episode about the ice cream truck kingpins of Southern California. It's a great story and also includes a segment where I get to make ice cream on an ice cream truck. And hey, did you know you can listen to the Sporkful on the Sirius XM app? Yes, the Sirius XM app, it has all your favorite podcasts, plus over 200 ad-free music channels curated by genre and era, plus live sports coverage. Does your podcasting app have that?

• 43:20Dan Pashman?

  And there's interviews with A-list stars and so much more. It's everything you want in a podcast app and music app all rolled into one. And right now, Sporkful listeners can get three months free of the Sirius XM app by going to siriusxm.com/sporkful. This episode was produced by me along with managing producer.

• 43:38Speaker 02

  Emma Morgenstern.

• 43:39Dan Pashman?

  And senior producer. Andres O'Hara. It was edited by.

• 43:42Speaker 02

  Camille Stanley.

• 43:42Dan Pashman?

  Our technical director is. Jared O'Connell. Our intern is.

• 43:45Speaker 04

  Lydel Go.

• 43:47Dan Pashman?

  Music help from Black Label Music. The Sporkful is a production of Sirius XM Podcasts. Our executive producer is Camille Stanley. Until next time, I'm Dan Pashman.

• 43:55Speaker 07

  And I'm Caroline from Bloomington, Indiana, reminding you to eat more, eat better, and eat more better.

• 44:09Dan Pashman?

  This segment is brought to you by Visit Myrtle Beach.

• 44:15Dan Pashman?

  Recently, my whole family took a trip to Myrtle Beach, South Carolina, to celebrate my mom's 80th birthday. How old are you today, Grandma?

• 44:22Speaker 04

  Seventy-nine.

• 44:23Dan Pashman?

  Again?

• 44:24Speaker 04

  Yeah, forever.

• 44:26Dan Pashman?

  There were 10 of us, my family of four, my brother's family of four, and my parents. We booked the birthday dinner at Hook and Barrel, one of the top restaurants in Myrtle Beach. They specialize in local seafood and eco-friendly wines. Cheers to Grandma. Cheers. When we sat down, our server brought out their house-made pimento cheese and crackers for us to snack on. We sampled the thick-cut Doroc bacon with brown sugar bourbon glaze. Then the entrees arrived. Now, anyone with kids knows that going out for a nice meal can be a bit of a gamble, and I don't want any whining to interrupt my mom's birthday or my enjoyment of the food. So I gave the kids my mic and sent them to work.

• 45:03Speaker 01

  Becky, what did you get and what do you think of it? So I got the seafood fettuccine, which we got some fettuccine and a nice creamy sauce with some shrimp and some fish in there. I'm low-key full from the appetizers, but I'm still gonna eat because my mouth is bored. It's very good. That was very tasty. Max, what do you think about your mahi-mahi? It's a super light and flavorful fish. It comes with barbecue sauce and colored greens, which I think is amazing because the barbecue sauce pairs very well with the mahi-mahi, kicking into that smoky flavor and really just elevating the dish to another level. It's crazy. Such a simple ingredient can go such a long way.

• 45:47Speaker 01

  So Grandma, what did you get and what do you think of it?

• 45:49Speaker 04

  I got the whole pan-seared red snapper. And I love a whole fish because I love filleting it. And I think I did an expert job of that. It's cooked perfectly. It's sweet and juicy and flavorful. So excellent. I loved it.

• 46:05Dan Pashman?

  I had the pan-seared scallops with corn salsa, corn puree, and bacon lardons. Mmm. The sweetness of the corn puree, the salty smokiness of the bacon. I would be worried like, oh, this might overpower the scallops, but it doesn't because these are phenomenal scallops. Then it was time for dessert. My team of reviewers was still on the clock.

• 46:28Speaker 01

  I got the chocolate-shaped cookie skillet with the vanilla ice cream. It was actually like really good. The cookie was crunchy on the outside and then like soft and gooey on the inside. And the chocolate was really rich and warm. And the ice cream is like really flavorful.

• 46:49Speaker 04

  I got caramel bundt cake. It's basically similar cake with this nice caramel sauce on top. It came with some vanilla ice cream. You know, I love some vanilla ice cream. And it was fire, I gotta say. My mouth is not even bored anymore. Highly recommend.

• 47:08Speaker 01

  I got a kilan pie. The crust is very firm and dense, which is always a good thing, kilan pie. And it's very citrusy. The texture of the whipped cream is very light and fluffy. And it brings the whole dish together very nicely.

• 47:29Dan Pashman?

  I went with a pineapple bread pudding with rum caramel sauce and homemade coconut ice cream. And it was truly one of the best desserts I've ever had in my life. The problem with a lot of bread puddings is that they're like monotextual. But this is like gooey in the center, but with the perimeter that is crispy, almost charred. And then the coconut ice cream, oh mercy. That dessert was so good that Janie and I went back two nights later just so I could eat it again. Anyway, soon the birthday dinner was wrapping up and it was time to go. All right. So final thoughts on the meal?

• 48:05Speaker 04

  Oh my God. I mean, it was just amazing. Everything was delicious, served beautifully, so lovely.

• 48:14Dan Pashman?

  Thanks again to the sponsor of today's segment, Visit Myrtle Beach. With over 2,000 restaurants, Myrtle Beach is the South's newest food lover's destination, earning recognition from the James Beard Foundation and other top national tastemakers. And they have everything from Atlantic seafood to traditional Low Country Fair and Southern classics. And what I found is that so many of the places with great food are very family-friendly. And they give you a chance to sit outside so many places on the beach or on a lake where you can have a beer, where you can have some great food. The kids can hang out and play. They can run around. It was a great vacation. And in Myrtle Beach, you can find jaw-dropping dinner theater, fine dining, global flavors from around the world, and breathtaking waterfront restaurants and exciting family eateries. Plus, did I mention 60 miles of beaches?

• 49:00Dan Pashman?

  Make Myrtle Beach your place by enjoying food you love with people you love. You belong at the beach. Plan your next trip to the beach at visitmyrtlebeach.com.

• 49:08Speaker 02

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