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Many people with PKU describe being on a well-managed diet and treatment plan as feeling like a “fog has been lifted.” Whether it is trouble concentrating or difficulty with planning, cognitive issues may not even be noticeable until after a successful PKU routine begins. In this podcast, our expert panel explores:
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Susan Waisbren, Ph.D. , Hunter Rametta , Laura Jeffers and Andrew Schorr
Susan Waisbren, Ph.D.
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Many people with PKU describe experiencing foggy thinking. What is the PKU fog? What can you do to help lift it? We'll learn more from the experts. It's all next on Patient Power.
Hello and welcome to this live PKU special edition, another in our series that we've been doing for years now. I'm Andrew Schorr. Thank you so much for joining us. We have folks all around the country. We hope we answer your questions.
Let's get started. So we know about if Phe levels related to PKU for yourself or a loved one, and we know that you want to get those down, and there are targets that your healthcare provider has discussed and you want to work on that and you know how important that is. But what goes along with it? If Phe levels are higher can you have foggy thinking? And if it's you the patient do you even know it or do other people notice it who are around you, or if you get those levels down can you see a difference? Well, we're going to hear about that from our guests.
Let's start with Laura Jeffers. Laura is a metabolic dietician. She's at the world-famous Cleveland Clinic, everybody has heard about that, and Laura has been a dietician for eight years, and she certainly helps a lot of people, children and adults with PKU. Laura, first let me ask you, what do people tell you about this fog? How do they describe it?
Well, they may not come right out and say that they actually have some foggy thinking. They may kind of describe it more as having trouble with concentration or maybe having trouble with relationships, just overall just their social—their social relationship struggle. Lot of times with their memory and planning and things, they struggle with that, and so then it leads to the discussion about the foggy thinking.
Now, how do you with them come to the conclusion that there are cognitive issues going on? Like what are the signs that either they notice or you may notice just in speaking with them?
Well, sometimes they have a hard time communicating what they're thinking as I ask them questions. Or they're explaining that they're not able to finish their tasks, that at work maybe they're struggling or in school, the behavior is not as it should be for their age. So I think that when they're explaining this we're able to really identify it.
And a shortened attention span, I bet.
Yes, for sure.
So what strategies do you use to help people get on the right track?
Well, first we talk about what they're currently doing. Are they consuming the diet that's recommended for them? Are their [Phe] levels high? Are they taking metabolic formula? What types of foods are they eating? And just making sure that they have all the options. So if the current recommendations aren't working for them, just making suggestions and letting them know what is all available for them, talk about Kuvan, have they tried that? Is that something that they could try to see if they're a responder?
Now, you definitely have found that higher Phe levels seem to contribute to this [foggy thinking]. Am I right?
And when you work with someone to get on a plan that's working for them, describe the difference you see in them.
Well, most of the time, if they are getting back on track, we see their levels drop, but that isn't the only indication. So a lot of times just seeing how they are more comfortable in social settings, maybe they're not having as many moods swings, their memory is better. They're organized, their attention is better even in clinic. Those are all indications that there is a response, an improvement. So it's not just all based on the Phe level.
And if someone were on medication like you said to see if they're a responder to Kuvan do you say those sorts of differences?
Yes, for sure. So we do take Phe levels before and during the trial, but we also work very closely with them to watch the way that they are socially and how they are. We talk to parents or loved ones to see how they are because really even if the response—maybe the Phe level is just maybe a little bit lower we still deem them as responders if their behaviors have improved, if their overall executive function is improving, because those are all very important.
No kidding. We're going to talk a lot more about that. Now, you've been a dietician for eight years. Someone who is kind of new to this is our dear friend Hunter Rametta, and there are a few people like this in the PKU community who have grown up as Hunter has with PKU and decided to be a dietician. Very cool. And, Hunter, last time we did a program, Hunter, you were weathering a hurricane in the northeast. This time you had six days without power, so I know you're joining us from Douglas, Massachusetts, and boyfriend Matt's house. Thanks to Matt and his family.
Hunter, so in your own life how have you noticed what we're calling here foggy thinking? How has it been for you, and when have you noticed it?
Right. So there's been a couple different ways that I've noticed this foggy feeling that I've had, and the first thing that I want to say is that a lot of times it's not something that I recognize in myself until my Phe levels come down and I can look back and I feel just revived and rejuvenated. And energy, I just—I'm just on the ball and really with it. But I do like to look back and think what kind of things did I notice, and for me the biggest thing that I see is just a really drain in energy. I might nap in the afternoon, which I don't usually do and even just motivation to do some daily things. Like if laundry builds up, you know, I might put off that task more frequently and just kind of delay little things and kind of procrastinate.
And then I've noticed too, for example my mother will point out, she says she knows me best, that I will get irritable and just really kind of take things personally and over-think situations and overreact. So for me it's really just a drained energy, lack of motivation and irritability.
What about forgetfulness, your keys, or where you left your cell phone?
Oh, yes. When I am in what we call “the fog,” if I notice a couple days in a row, one day I forgot my keys in my office and got out to my car, or, you know, one day I forgot my cell phone before I drove to work. Sometimes when I notice that happens a few times in a row it's kind of a red flag for me to think, okay, how have I been eating? Was this just gradual? Did I just miss a formula a few times, you know, whereas if I have a vacation and I run out of formula I might expect something like that and I can be more proactive about it. That's when, for a few days, I'll really write down what I'm eating and try to get back on track, and a lot of times that forgetfulness kind of resolves itself and goes away.
That's great. Well, as we've mentioned on earlier programs, Hunter has grown up with someone who has helped her tremendously at Children's Hospital in Boston, and that's Dr. Susan Waisbren, who is associate professor of psychology at Harvard Medical School's department of psychiatry, and she of course is well known to everybody who listens to Patient Power and certainly in the PKU community. So Susan joins us again from Boston.
By Andrew Schorr