On this last episode, we look back on 23 years of joyous, difficult and always informative conversation.
Seventeen-year-old D.C. resident Tia Piziali was born with phenylketonuria, a rare genetic disorder commonly referred to as PKU. For virtually all her life, prior to a recent breakthrough in medicine, the disease prevented her from eating even small amounts of protein. If she were to have eaten, an egg for example, she would have incurred irreversible brain damage.
There is no cure.
Tia’s mother Alison Reynolds has been raising money for the National PKU Alliance for years, through galas and auctions, but she recently decided to embark on another, more nontraditional way of raising money. Reynolds decided to cross-country ski across the rugged terrain of Norway nine hours a day, for nine straight days, totally 125 miles.
How did Reynolds fare in the Nordic country, and how much money did she raise?
Produced by Kurt Gardinier
This segment is part of our series profiling local women who are making a difference and influencing their communities. This series is airing throughout March in celebration of Women’s History Month.
- Alison Reynolds Advocate for the cure of Phenylketonuria (PKU); @NPKUA_Info
KOJO NNAMDIWelcome back. Throughout March, in celebration of Women's History Month, we'll be meeting local women who are making a difference in their communities and in their professions. First up in our Game Changer series is Alison Reynolds. Alison's 17-year-old daughter, Tia Piziali, was born with phenylketonuria, a rare genetic disorder commonly referred to as PKU. For virtually all her life, prior to a recent breakthrough in medicine, the disease prevented Tia from eating even small amounts of protein. If she were to have eaten, say, a spoonful of peanut butter, she would've suffered irreversible brain damage. There is no cure.
KOJO NNAMDIAlison Reynolds has been raising money for the National PKU Alliance for years through galas and auctions, but she recently decided to take those efforts to a new level. Last month, Alison cross-country skied across Norway, nine hours a day, for nine straight days, totaling 125 miles. How did she fare out there, and how much money did she raise?
KOJO NNAMDIWell, she joins us in studio to discuss that. Alison Reynolds is a D.C. mother of four. She used to work in the tech industry in Silicon Valley, until Tia was born with that rare genetic disorder. And since then, she's been raising money for the National PKU Alliance and advocating for a cure. She joins us now in studio. Alison Reynolds, glad you could make it here, glad you could make it back.
ALISON REYNOLDSThank you. Thank you so much for having me.
NNAMDIWe'll get to your remarkable Norwegian trek shortly, but let's start with phenylketonuria, or PKU. What exactly is it?
REYNOLDSSo, as you mentioned, it is an inherited, rare disease where, essentially, protein food is toxic to the brain and central nervous system. And so, Tia's liver can't convert phenylalanine into tyrosine. And when that accumulates in your blood it can cause, as you said, irreversible brain damage.
NNAMDIIs there treatment for people who are battling PKU?
REYNOLDSThere is a treatment now that we started funding, when she was one year old, called Palynziq. It's an injectable. Tia gives it to herself in her stomach once a day. It's a holdover drug. You know, it's not a cure. It's complicated. It has issues associated with it. It's quite expensive. You have to be 17 or older to take it. Many people don't like the idea of giving themselves an injection, so we believe that we need multiple solutions. And then we also are fighting hard for a cure.
NNAMDIWhat is the cost of this drug?
REYNOLDSIt's $100,000 a year. You know, our insurance is covering it completely, you know, and we have a deductible, of course. But for those that don't have health insurance or have a different type of policy, this would not be an option.
NNAMDIHow has your daughter and your family dealt with her disorder?
REYNOLDSYou know, from the start, we decided to be conservative with her treatment, meaning, you know, seek the best information we could. Treat her according to that latest information. Her levels have always been virtually in the healthy range. She's doing quite well. So, we've been seeking information and just really attacking it, also from the fundraising perspective. We believe that you shouldn't just take the cards you're dealt, that you should fight for more.
REYNOLDSAnd so the medical community decided, 50 years ago, there was a solution for PKU, and that was a highly restricted diet. But 70 percent of adults and teens are not on that diet. So, you can't call that a successful treatment. So, we tried to change the narrative very early on in her life to say, okay, there's a treatment. It's dietary restriction. It's very difficult. People don't tend to be able to stay on it for life. We need more. What else is out there? So, we sought research proposals and things like that, and we started funding research. And we've now, 17 years later, seen results. Tia's on a drug. That's, you know, a game-changer.
NNAMDIWell, we spoke with Tia earlier, and this is how she describes how PKU has affected her.
TIA PIZIALISo, basically, throughout my whole life, my protein tolerance was 6 grams a day, which is pretty much nothing. I mean, like, for example, like, one slice of pizza has up to 10 grams. So, I was on a very restricted diet for 17 years of my life until this new drug came out that's been helping me. But I was on a very low-protein diet. I have never had meat the first 17 years of my life. So, yeah, I had to track all my meals and I had to do very tedious math just to track how much protein content was in my food. So, it was very hard, and it affected me in sports, as well, just because I was very malnourished and underweight throughout most of my life because of it.
NNAMDIThat is Tia, who is Alison Reynolds' daughter. Alison Reynolds, do you think there's a cure in Tia's future?
REYNOLDSI do. I believe there'll be a cure possibly before she decides to have children. We've been investing in work around CRISPR gene editing, and that holds a lot of promise. We've also been investing in -- when I say we, the National PKU Alliance makes these grants. We fundraise for them. Stem cell injections right into the liver to repair, you know, the defective capabilities there. PKU's a disease that's based in the liver.
REYNOLDSBut CRISPR gene editing, I believe, holds the biggest promise, you know, probably in the next decade or so, where they actually go in and they alter the gene so that it turns on and work properly.
NNAMDIHere now is Victoria in College Park, Maryland. Victoria, you're on the air. Go ahead, please.
VICTORIAYes. I just wanted to talk about, you know, a kind of tragic family story, in the times before there was testing of infants for PKU, which I guess is a pin-prick test. So, my oldest cousin was born in the '50s, sadly, in New York, and they didn't pass the legislation until the year after he was born, the testing for it. And what happened is he was born normal. And my mother said she would, you know, notice and say, I think there's something wrong with the baby that, you know, you need to take him to the doctor.
VICTORIAAnd what happened, I guess, as the guest has mentioned, that the brain starts -- I don't want to say the word degenerating, but he was fully mentally handicapped all his life. And he lived -- he only passed away last year, in his early 60s. But he was ultimately -- you know, lived in a group home. But, anyway, it's a sad -- I guess it's both hopeful that, you know, now there's changes. But before then, and the detection, it was a very curable tragedy.
NNAMDISo your cousin had to live with that disability for all of his life.
VICTORIAYes, yes. Mm-hmm.
NNAMDIThank you very much for sharing that story with us. Alison Reynolds, I guess I saw you nodding. That story sounds fairly familiar to you.
REYNOLDSIt does, you know, for PKU adults who were born before newborn screening. So, newborn screening is what catches PKU. So, in Washington, D.C., the city made tests for a panel of, let's say, 30, 40 different diseases. PKU's one of them. You get a phone call about four days later with the result.
NNAMDIAnd for those that were born before newborn screening, it's a tragic ending. You know, by the time they're -- they've completed their first year, they're missing milestones, and the gray and white matter in their brain's degenerating. And many of them end up in a facility.
NNAMDIThank you very much for your call and sharing that story with us, Victoria. If you've got a story to share, have you raised money for a good cause in a unique way? Tell us about it. 800-433-8850. You can send us a tweet @kojoshow or email to firstname.lastname@example.org. We're going to take a short break, and when we come back, we will continue this conversation, as Alison Reynolds will tell us about her odyssey in Norway, what caused it and what resulted from it. I'm Kojo Nnamdi.
NNAMDIWelcome back. Our guest is Alison Reynolds. She's a D.C. mother of four who used to work in the tech industry in Silicon Valley until her daughter Tia was born with a rare genetic disorder known as phenylketonuria. Alison has been raising money for the National PKU Alliance and advocating for a cure. She joins us in studio. Alison, talk about the unique way you decided to raise money for this disease. How did you end up choosing to cross-country ski in Norway? Why not, say, a swim in the Caribbean?
REYNOLDS(laugh) So, you know, as you mentioned, I had been doing galas and sort of a traditional type of fundraising over the years. And I knew that there was still a need. We had great research out there that needed funding, and so I wanted to do something more. But I wanted to do something different. And I've always been sort of attracted to physical challenges. I'd been contemplating doing that.
REYNOLDSAnd then thinking about the anniversary of the discovery of PKU, Asbjorn Folling, the Norwegian biochemist who discovered it, is very important in our community. He's actually important in the medical community, overall, but his story's not well-known. And so I wanted to find a way to highlight his legacy and the important discovery, do something for PKU, raise awareness, raise research funds, tie together the international PKU communities, as well.
NNAMDIWell, why cross-country skiing, though? Has it always been a passion of yours?
REYNOLDSYou know, it's been an interest, a hobby in my family. My mom is a serious cross-country skier, and she got my brother and me skis when we were probably around 12 years old. And so we grew up cross-country skiing when we would, you know, have snow in the area. I grew up in Alexandria, so just the G.W. Parkway, that long bike path that goes down to Mt. Vernon, we used to ski on that. We skied in Colorado some and where my parents now live.
REYNOLDSAnd my mom actually cross-country skied to the North Pole in 2000. So, I was inspired by her. You know, she's just an important person in my life, obviously, and lives across the street from us here, too, but is also a big part of raising Tia with PKU. She cooks for her. She's on the Scientific Advisory Committee of the National PKU Alliance. She's very, very involved in PKU research and science. And so, you know, she was an inspiration behind this.
NNAMDITell us a little bit more about that trip that your mother did 20 years ago, because it was her and two others, was it not?
REYNOLDSCorrect. She skied with two British explorers. They did the last nautical degree from Russia to the North Pole, and it took them 12 days. It was a very different kind of trip. I was thinking about her a lot when I was skiing in Norway, because we had a lot of up and down. You know, it was very hilly where I was skiing. It was also extremely deep snow that was unexpected.
REYNOLDSWhen my mom skied to the North Pole, it was very flat, and, you know, they had the icy conditions. And, you know, it's an ice field. There's no land mass there, so the ice is constantly moving. Pieces would crush together and create little mounds. But we had different kinds of challenges, but yet, it was a similar sort of mental test. And she helped me prepare for Norway. You now, gave me tons of advice, gave me some of her equipment. I actually wore her purple down jacket. Every time I stopped, I felt like my mom was wrapped around me. She kept me warm through the trip, literally probably, you know, sort of saved my life through keeping me away from hypothermia. And it was just -- that was pretty special.
NNAMDIFor listeners who may not have noticed what you said before, tell us again why you chose Norway.
REYNOLDSOkay. Well, the PKU was discovered by a Norwegian biochemist, Asbjorn Folling, in 1934. And so I wanted to honor his legacy and his important discovery. He saved my daughter's life.
NNAMDIFascinating story. 800-433-8850. Are you a cross-country skier? Would you take on skiing nine hours a day, for nine straight days, in treacherous conditions? Give us a call. Call me, Matt McCloskey, (laugh) 800-433-8850. Send us a tweet @kojoshow or email to email@example.com. Have you raised money for a good cause in a unique way? How did you train for the Norwegian trek?
REYNOLDSWell, I got to snow as often as I could, which is not easy in Washington, D.C. We have not had a lot of snow. So, I trained pulling on the snow. I was pulling a sled that had firewood in it. So, I would try to get it to weigh around 90 pounds, which was the weight of my sled. And then, in Washington, D.C., I pulled tires. I started with one tire, then I went to two tires -- these are oversize SUV tires -- down the C&O Canal trail, which was pretty funny. I got to know people on the trail. I got a lot of high fives and, oh, yeah, you're back. Okay. Way to go. I see you added a tire. You know, that was pretty cool.
NNAMDIAnd then I worked out in the gym three days a week, and then I did endurance cardio training six days a week. So, I would do gym sessions, followed by running for several hours or pulling the tires. So, it was quite a bit.
NNAMDI(overlapping) Yeah, I read the Washingtonian article. Strength training three times a day, endurance training almost every day. A sample day might include a 90-minute Peloton ride, followed by a 45-minute run, hiking several miles with a weighted backpack or pulling a tire on the C&O Canal for 100 minutes. Apparently, on the C&O Canal, you got a lot of thumbs up while you were doing this. People were, like, better you than me.
REYNOLDSYes, exactly. (laugh)
NNAMDIHow did the trek itself go? Did it go as you planned or as you hoped it would?
REYNOLDSYou know, no, it really didn't actually go exactly as planned. And I as prepared for that. Whenever you go into the backcountry, you now, Mother Nature is making the calls. And so, we had determined a route, and we had to change our starting location based on a storm that was coming in. You know, my guide was watching the weather...
REYNOLDS...very carefully. Elise Koren, exactly, from Hvitserk. And she emailed me the night before we left and said: I need you to get to a Norwegian phone. I need to call you. And so we spoke, and she said, I'm really worried about the weather. There is a major storm moving into the area. It's been warm here this winter. Heavy snowfall on top of warm snow can create, you know, avalanche danger. Are you open to changing the route a little bit, you know, shifting it north, south, east, west. And I said, absolutely. It is imperative that we be safe. You know, I'm making it back to my kids and my husband.
REYNOLDSSo, we did have to change things along the way. We had far more snow than we anticipated. In the first three-and-a-half days, it snowed about 4 feet. And you wouldn't really choose to do a trek pulling a pulk sled in deep snow. You would choose a backpack. And so -- but last year, last February, Elise did that exact route. She had never done it before. And it was hard-packed, you know, icy snow. It's usually a windy route, but they really don't see the conditions we had this year. (laugh)
REYNOLDSSo, that was a challenge. It was high winds for the first several days, up to 40 miles per hour, with the gusts. And so that made it difficult. That's what -- I actually lost a jacket on my first day. And so, you know, I was concerned about that. But, then again, I had my mom's special down jacket. And so we just adjusted, but it was mentally a challenge those first few days. I mean, I was concerned. I could see she was concerned. I followed her lead. She was the expert.
NNAMDIAs I was saying, we're celebrating -- this is a part of our discussion celebrating Women's History Month. And I find, not only your trip, but your guide, Elise, fascinating, because this is something she does, in a way, for a living.
REYNOLDSIt is. It is. And we talked, you know, obviously, for hours on end, and I got to know her very well over nine days. And there's a certain personality type who is -- they're comfortable pushing their body to extremes. She told me, on the last night, that she enjoys proving to herself that she can survive in the harshest climates and survive through the night. And she said, I like to know that I can survive in a way that they used to survive, you know, in the olden days. And so, she was fascinating as a person. She hunts, she rides her motorcycle. She, you know, doesn't like being in doors, period, you know. So she's cool.
NNAMDIPerfect choice for a guide, obviously, in this situation which was difficult. You slept in the snow.
REYNOLDSWell, we slept in a tent in the snow, and that was -- yeah, that was challenging. (laugh)
NNAMDIIn deep snow.
REYNOLDSStaying warm was one of the hardest parts of this journey.
NNAMDIHere's your daughter Tia, again, on watching you finish your nine-day, 125-mile cross-country trek through Norway.
PIZIALIIt was just really -- I mean, we almost -- I got the chills. I mean, we all cried when we saw her, just because we were all very worried, just because of the conditions she was in. They were pretty dangerous. It was really inspiring, and almost -- I don't -- like, it's very hard to describe. It was very surreal, though. It made me realize how far we had come since the first day she had gotten that phone call that I had PKU. It made me realize, as I saw, like, 40 people outside cheering her on, all avid supporters of PKU and awareness for it. It made me realize how far we've come, but also how much further we need to go.
NNAMDIYour daughter Tia, talking about watching you finish this. You just got back this past Monday, didn't you?
REYNOLDSI did. I got back Monday night. We finished the ski on Saturday, so I think we came across the finish line at 1:00 p.m. We had a gathering of Norwegian PKU families and some PKU families from the United States there. We had a dinner, and then we had five researchers present on the latest PKU research on this Sunday morning. So, the actual event didn't really end on Saturday, when I finished. It continued through Sunday night. And it was -- you know, that was one of the things that I hoped would come out of it was these different PKU communities, international communities getting together.
REYNOLDSYou know, Norwegian PKU children are going through the similar thing that Tia's going through. You know, we share a common language and a common experience. And so it was great to, you know, get to know each other and become friends.
NNAMDIWell, you finished on Saturday, and you were involved in events on Sunday, came back here on Monday. After a physical ordeal like that, it would be weeks before I was up and around again. (laugh) And yet here you are out still advocating. What is the source of your passion?
REYNOLDSI want to see a cure for PKU, and as soon as possible. I mean, that's my goal. That's what drives me.
REYNOLDSHow much money did you set out to raise and how much did you end up raising?
REYNOLDSI did not know what to expect. You know, I've done galas and I could do those spreadsheets. I did not know what we would accomplish here, so I set a goal of 400,000 and, you know, I think we passed that maybe six months ago. And so we -- when I looked this morning, it was 1,075,000 total.
NNAMDIWell, it was certainly worth the trip. That's for sure.
NNAMDIPaulo from Alexandria called to ask: is a plant-based diet helpful for people with PKU? What can they eat?
REYNOLDSYou know, that would be -- I wish that was the case. In terms of Tia's tolerance, she was able to eat very small amounts of fruits and vegetables, you know, weighed and measured. There are many plants, legumes, dark leafy greens are actually high in protein for somebody with PKU. So, it's all relative, right.
REYNOLDSSo, you would think that you could have a vegetarian-type diet, but you actually can't. Like a banana is high in protein for Tia. An orange is high in protein for Tia. Spinach, kale, brussel sprouts, cauliflower, broccoli. Think about the really nutritious filling veggies. Those are even high in protein for Tia. So, yeah, it's more complicated...
NNAMDIEven though there's now a medication, with that medication does she also still have to keep counting? Does she still also -- or can she just now have protein at will when she's taking the medication?
REYNOLDSWe do keep count, every single day, yep. It's funny, now we have the opposite challenge where she has to get up to a certain amount of protein in a day, or she can suffer some negative side effects, which she has suffered. You know, basically, this drug is working so well for her, that she can tolerate 60 grams of protein in a day. So, you're telling a child who went from only being able to have 5 to 6 grams of protein in a day, or the equivalent of a half an egg, telling her she has to eat 60 grams of protein a day. And she's been raised thinking that protein is a negative, protein is toxic, protein will hurt me. And so it's a challenge, every single day, to get her up to that number.
NNAMDIIt builds a certain, clearly, strength of character in Tia, especially as I was thinking, if you've got three siblings who are running around having all the pizza they want and anything else that they want, and you're sitting there looking at them while you have to count exactly how much protein you can have. This must have been an ordeal for he, too.
REYNOLDSYes. I mean, I think it has, as you mentioned, it's built a lot of resilience in Tia. She's dealt with a lot, and she blows me away on a daily basis.
NNAMDIMany people are passionate about a cause and want to do something to make a difference, but figuring out how to go about it and take the first step is often the challenge that sidelines a lot of good intention. What is your advice for someone listening right now who is inspired by your story, who wants to go out and meet the challenge of doing the same kind of thing?
REYNOLDSI would say don't be afraid to take a risk. You know, it can pay off. And, you know, it's just taking that first step, I think, that takes the most courage. You'd be amazed by how many people will respond to your story if you share your store, you know, and tap into what really motivates and drives you. People will respond.
NNAMDIWe got a tweet from Mary, who writes: inspiring show today. My niece founded the Hypotonia Foundation after her son was born with this condition. She wrote an audio story to support families who deal with this. Tell us about the National PKU Alliance. How was it founded?
REYNOLDSWell, it was founded in 2008. And, at the time, there were small 501 (c)(3) s around the United States that were, you know, raising funds for PKU research. And, you know, these were smaller efforts, you know, smaller regional efforts. And so my dad came up with the idea to found the National PKU Alliance. And so he went and met with each of those 501 (c)(3) s and said, how about we create an umbrella organization with a number of organizations underneath it? And we can consolidate our fundraising efforts, our messaging, our grants that we're giving to these researchers and move the needle faster.
REYNOLDSAnd so that's what he did. He actually modeled it after the Cystic Fibrosis Foundation model, got together with those folks. And, you know, it was formed in 2008, so it's been around 12 years. And it's been a really worthwhile effort.
NNAMDIAnd thus the National PKU Alliance was formed. What is next both for you and for the National PKU Alliance? I suspect we're not embarking on another nine-day... (laugh)
REYNOLDS(laugh) I've been thinking about that. I'm going to take some time to rest. (laugh)
REYNOLDSI'll stay involved and, you know, keep the quest going for a cure. But I probably won't be embarking on any other extreme outdoor challenges anytime soon. (laugh)
NNAMDII'm sure that Tia's also breathing a sigh of relief about that. (laugh)
REYNOLDSShe is. (laugh)
NNAMDIAlison Reynolds. She's a D.C. mother of four who used to work in the tech industry in Silicon Valley, until her daughter Tia was born with a rare genetic disorder known as phenylketonuria, also known as PKU. Alison has been raising money for the National PKU Alliance and advocating for a cure ever since then. Thank you so much for joining us.
REYNOLDSThank you for having me.
NNAMDITry to get some rest, okay? (laugh)
REYNOLDS(laugh) I will.
NNAMDIThis segment on skiing for a cure was produced by Kurt Gardinier. And our conversation about Willisville, Virginia was produced by Lauren Markoe. Coming up Friday on The Politics Hour, D.C. Councilmember Charles Allen wants to give District residents $100 per month to use on Metro rides. Could this actually happen? Plus, the Montgomery County Council tabled a controversial police bill that would have put more officers in schools. Councilmember Craig Rice joins us to discuss that.
NNAMDIThat all starts tomorrow, at noon, on The Politics Hour. Tonight, Tom Sherwood, our resident analyst on The Politics Hour and I will be conducting a debate among all of the candidates running for Ward 2, the seat formerly occupied by D.C. Councilmember Jack Evans. He, too, is running again for that seat, and will be participating tonight. So, see you at noon tomorrow. Until then, thank you for listening. I'm Kojo Nnamdi.
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