Decoding NF

Shane: My guest today is Dr. Frank Buono. He's an Associate Research Scientist and Lecturer in the Department of Psychiatry at Yale School of Medicine, as well as the Co Director of the Neurocutaneous Clinic at Yale New Haven Hospital. His research centers around digital technologies that can assist with treatment for individuals with rare diseases and those who have limited financial and clinical resources. He has focused his research on the understanding of chronic pain and neurofibromatosis, the impact of chronic pain on quality of life, and complementary and alternative approaches for pain management.

His most recent project centers around a mobile application for chronic pain management for adults with neurofibromatosis type one called I can cope in F, which showed positive results for patients experiencing chronic pain during the study, which was conducted in 2022. We talk about this app a bit. In the interview, and I just want to point out right off the top that this app is not yet available to the public in a wide sense, although he intends to do that after they complete the randomized control trial that they're currently recruiting patients for.

There's a lot of really great information in this interview, and I hope you find it useful. Before we get into this interview though, I do have to get this disclaimer out of the way.

The content in this podcast is for general informational purposes only and does not constitute the practice of medicine, nursing, or other professional healthcare services, including the giving of medical advice and no doctor patient relationship is formed. The content of this podcast is not intended to be a substitute for professional medical advice, diagnosis, or treatment, and listeners should not disregard or delay in obtaining medical advice from their doctors.

Okay, without any further ado, here's today's episode.

Just a few minutes ago beforehand, we were just talking about NF2 a little bit. I want to touch on that again before we move on too much.

So you mentioned that with NF2 pain, the way it presents is usually not necessarily related to. The disease itself, but more related to , say, post surgery recovery or something like that. Could you elaborate a little bit?

Dr. Frank Buono: Sure. And I will also mention that, I am not a physician. So all of the things that I have seen either done in my research or dealing with an advocacy or clinical base. But most of the NF2 related schwannomatosis that I see, Or I have interviewed have been due to like a spine surgery and the individual had an ependymoma removed and now has residual back pain because of that or has sporadic pain down their legs. That is the typical presentation that I see. It's not as. Not related to like schwannomatosis, which is a clear indication that they have pain or for example, NF1 pain, which has, a topical pain or a superficial pain as well.

Shane: All right. So let's talk about NF1 pain. Because that's where your research really focused. And I do, I want to like maybe give a little bit of background. So maybe if you could just start by giving a little bit of background on. your research, like the, I can cope stuff and just talk a little bit about where your investigative experience comes in as it relates to NF1 pain.

Dr. Frank Buono: So let's start off with the basic facts. One in four individuals in the United States have some form of pain. And. The chronic, and it goes typically from acute, which is this one to three month interval to more of a chronic pain, which is, and so when I talk about chronic pain, I'm talking about pain that's lasting for a significant amount of time over three months. Most individuals that I have worked with NF1 from a research and from a clinical and from just an advocacy standpoint are in chronic. There has been. A milieu of research in the last 5 to 10 years about chronic pain within NF1, and the number, the percentage is variable. I've seen anywhere from 20 to 65 percent of adults having some form of chronic pain, and some of this chronic pain can even start in adolescence, continue all through their adulthood. So having that basic component there is in my experience, very few clinicians or very few centers that function or have a pain psychologist. on staff. And so it is also a problem that if a clinician is treating pain from like a no receptive component, but it's really a different form of pain, that's that they can do ill effect for the patient.

Shane: Could we back up a little bit there? So you mentioned that term noceptive. So can we talk about the different ways that pain presents in someone with NF1?

Dr. Frank Buono: Yeah. So we can talk about, I know, there are a plethora of different types of terms that are used for pain. So we I go down my list of pain descriptors, for example, right? So you can have throbbing, shooting, stabbing, cramping, heavy, tender, splitting, aching, list goes on.

There's like 50 terms, right? All of these terms play a factor within NF1 and each individual's symptoms are individualistic. So, what my pain is more of a stabbing pain, and could be completely different from your pain or, anyone else that who has pain. So I, it is something that needs to be thought of and be mindful that. everyone's pain and everyone's reflection of their pain can be and is different.

Shane: Yeah, I think that's an important point because, Within F1 2 like my son, for instance, with cutaneous fibromas, they'll find that maybe it's not even pain as we might typically consider it. It's more of like a tickling or a tingling sensation, but that's still a disruptive physical sensation.

that can be chronic and longterm. And I think that probably, I don't know if you classify that under kind of the heading of pain or if that's something in your research that you touched on as well as trying to alleviate.

Dr. Frank Buono: Yeah. And that's a great point, Shane. And I'm glad you brought that up. It's tingling is still part of the pain paradigm. There is this in the pain world, we talk about, if it is tingling versus stabbing, so what is the severity? Cause we always go to severity, but the one point that you brought up is how much does it interfere with the individual's life, which I think is very critical and often under evaluated. And so the understanding of, does this affect the individual? Does this affect the family? Does this affect the community? If answers to any one of those is yes, then that is pain.

Shane: Yeah, I think that's a really good point and a very salient one because I often think about this. My only experience, my only real like day to day experience, of course, is my son's, but I often think about it from the perspective of, he's probably lived most of his life with some base level of pain or sensation that he can't control.

Yeah. And so pain is such a subjective experience. Sometimes I wonder, if I could be transported into his body, if I would suddenly be kind of bombarded with sensations that, I would classify as painful, but that are just the background noise that he lives with. And that there's, that's, those are two separate things.

He's, he functions on a day to day basis without, being constantly wincing or, without constantly being totally disrupted by pain. But that doesn't necessarily mean that he's not experiencing something. But then another patient with a new kind of presentation or some other different kind of thing could find something that he lives with without disruption every day, very disruptive.

And so treating pain in that subjective way is like a really important component. When a patient is trying to receive care, I imagine.

Dr. Frank Buono: Absolutely. Uh, we actually published an article on this, uh, two years ago, showing that individuals with NF1, specific adults, are dealing with pain and they go to their doctor, they go to the provider and they say, and the provider asks them what's their pain and the patient already pre evaluates that number and lowers the value.

Because when you say To a doctor. Oh, my pain's a 10. Well, they're gonna go think from a psychological or a physiological, excuse me Perspective and say okay. I don't see you being you know Sensitive to light or I don't see your BP changing Drastically, but in reality these individuals have now what we call the term satiated to their pain And they are now a, what you and I, or what someone who is neurotypical might think of as a five. They're actually like a seven, but they have, I'm sorry, uh, other way around. Let me change that around. So the way that it works is, what we would think as a seven or an eight is really a five for them because they've already been satiated to that that context. And that's scary because if we're, if they're purposely lowering their scores or they become so accustomed to that pain that is a marker that we and the educational and the research and the clinical space are need to do better.

Shane: So how do we tackle that problem of the subjectivity of pain? Like how have you approached that in your research?

Dr. Frank Buono: One thing is, is that if you or your son or any in the patient with NF, One comes to my, comes to my research and says that their pain is a five. I fully accept that their pain is a five. That's the first thing, and I don't question it. You know your body better than I do. I'm going to ask some follow up questions, but if you say it's a 5, then it's a 5. So that's the first part, which is acceptance . The second part is okay, so we need to find ways to help treat your pain. There have been several studies showing that opioids are ineffective for NF1, uh, uh, And so I think the idea for me and what my research is based on my own physical conditions of having NF2 related schwannomatosis is we need to find alternative treatments. And so one of the things that I did was I published a study now, wow, six years ago Showing that most adults with NF1 have yet to try an alternative treatment strategy for their pain. Meaning they have not tried yoga, they have not tried acupuncture or acupressure, they have not done P. T. O. T., they have not done a list of other activities.

They are primarily utilizing, some type of opioid, Oxycontin, for example to mitigate their pain. or to not mitigate, but to lessen the pain severity.

Shane: And isn't that kind of, indicative of the state of medicine and the shift toward, over the last, half century or whatever, to accept pain as an important vital sign, right? And to trust, patients when they say that they're in pain and to treat it like no holds barred most of the time.

But. the treatment is generally pharmacological because of the kind of the subjective nature of pain, I guess, and all that kind of stuff. And so if an NF patient complains to their doctor of pain, then, nine times out of 10, that doctor is going to prescribe like an opioid or maybe gabapentin because, they assume the pain might be nerve based.

And and the various, analgesic effects of these, drugs, they vary widely and with NF patients, sometimes they don't work at all. And I wonder, to what extent should a patient be investigating kind of these alternative methods from your research, what did you find was effective outside of a pharmacological approach Like the most effective in general, or is it very personal?

Dr. Frank Buono: I think the most effective one, at least the research that I did and we had a sample of like 180 some patients or who completed the study with NF1 that The individuals who actually utilized some type of psychos psychotherapy, right? Maybe like cognitive behavioral therapy or acceptance commitment therapy to talk about their pain in conjunction with a yoga or a tai chi, yielded some fantastic findings. And it, so it's partly movement, partly talking about it, which is going to help that model. And I think we need to just back up for one second because pain should be treated as a biopsychosocial model. So you have your biological perspective, your psychological perspective, and your social perspective all intertwined because that's what pain does. Pain affects the nerves, or, and so it triggers some, uh, an effect in our body. But we also have to treat it from a psychological perspective because we are, innate beings who need to understand why we are in pain. And then you talked about your son, that is the community approach, and how is that affecting you as a parent or a patient, affecting their family or their larger, uh, community at large.

And I think that's important. So when I go into helping a patient or talking to a patient it's different than how I'm going to evaluate for research, right? Uh, in a research, the idea is give them access and that's what, why we started the I can cope was give them access to resources, let them learn about NF1 and other alternatives treatments out there and let them see how they would do it.

Shane: I think actually just to put a finer point on the point that you just made, which I think is really important to the understanding of pain as a phenomena is that, medicine, or at least I guess in a general sense, medicine, kind of as a hand wavy term has approached pain really from that biological lens.

Knowing that the other components exist, but feeling, I think feeling like, well, yeah, but there's only so much we can do because pain as a phenomena, right. It's it is very much it is a biological response. It is a, signal that your body is sending to indicate, that's something, bad is happening to you. But. Whether or not that a signal exists is highly influenced by, The socialization of what we see as pain in an environment. When a kid falls and scrapes their knee when they're little, how you respond to them scraping their knee or whatever, directly, correlates to whether or not they start bawling their face off or they get up and they run off and continue to play.

And it, they look, those signals are learned early on as to how to interpret this information right. That's coming into their brain. And then on a social level, what a society considers painful or like a, T traumatic or painful, heavily influences. On an individual level, what somebody considers painful, but some of those realms are I think, medicine is seen as out of their scope.

And I think that, what you're pointing to is that as care advocates for ourselves, as patients, or for the people that we care about, we need to come in with that more holistic approach and that probably, generally speaking, but also specifically in the NF world, we should probably be thinking of it in that more holistic way as we approach it from a treatment standpoint.

Right.

Dr. Frank Buono: absolutely. And at minimal, you should have a clinic that does multidisciplinary approach. It shouldn't be just one provider telling you what to do. I think you need to have, the old saying that two brains are better than one, idea. I think that is something which is critical. You get a neuro, a neurology, individual consult in while you're getting your psychology, individual and you get a speech and language pathologist and occupational therapist or you get whoever you need to be in that room so that We can all talk and come up with a an effective way to help with that patient. To go back to what you were saying about the impact of pain, we have pain, right? And so it's going to affect us in multiple different standpoint. So for example, I have chronic pain now I'm gonna be less active , right? So I used to run five miles a day, now I'm not running five miles a day. So because of that, I lose my fitness. I get weaker muscles. I might have joint issues, right? From that I will have a lack of energy. I'm tiredness. And then this becomes a secular cycle here right now. And now the realm of stress, fatigue, anger, frustration comes in and then negative thoughts can come in. And then depressive moods can come in and then it can affect, then it affects the community. You can be outburst to the parents. It could be outburst to other community members. So it is not just biological as you were saying. It is a truly bio cycle social model and it needs to be thought of that way.

And we need to refrain from thinking it as solely biological.

Shane: So I want to talk about, I can cope in more specific detail, but I have one question actually that, um, I wanted to ask in terms of treatment from a pharmacological standpoint, I'm wondering if in your research did you find that patients treated with. A MEK inhibitor experienced less pain.

With my son, I found that for whatever reason, once he started taking selumetinib it had an analgesic effect on top of everything else. Like he stopped experiencing a lot of different kinds of sensations that he was experiencing before. And I'm wondering if that is unique or if that's something, a common feature that you found.

Dr. Frank Buono: I have not conducted research on that as of yet, but I know from the clinic that we here have at Yale, we have several individuals on selumetinib and we've gotten that same reaction. I can think of three cases already off the top of my head who have had, from a kid's perspective less pain reported once taking selumetinib, but by no means is that A large enough sample size.

I know Andrea Gross and her team. At NIH had published several articles on the selumetin trials and they talked about pain as covariates. And reduction of pain, but I, I don't have specific data that I have controlled for.

Shane: Yeah, I was just curious just because we were talking, it was the kind of the pain episode and I, and it's just something that occurred to me. And I was wondering if it was a unique experience or if that was pretty common. Because I do want to talk about like treatment options. So, let's start with, I can cope first because we've mentioned it a bunch of times, but maybe you can give a little bit of background on, That application and the research around that. And maybe just go into a little bit more detail on that and then let's feed that into a discussion of, okay, so now I'm a patient with an F1, I'm coming across this information and what can I do about it?

So maybe start with, I can cope. And then we'll go from there.

Dr. Frank Buono: Absolutely. So I can cope is a mobile application for both Android and iPhone. It was based out of SickKids Hospital in University of Toronto. They developed it for juvenile idiopathic arthritis, chronic pain, sickle cell anemia. And so it's been shown to be effective in a lot of different adolescent. The young adult populations prior to this. One of the things that, uh, we wanted to look at was adults. And specifically NF1 adults. So what we did is we retrofitted this mobile application specifically for adults. We changed all the language. We added content, uh, to, for adding NF1 content. We added mindfulness activities because we thought, my, my training has been in the ACT realm and knowing, understanding about mindfulness is critically important. And what we did was a one by three randomized clinical trial to evaluate the efficacy of this, uh, mobile application.

Shane: Okay, real quick, before we move on, you said one by three clinical trial.

Have folks coming across this information that are just getting into the world of clinical trials. And that's a big part of being of being someone, an advocate or a patient of someone with a kind of a rare disease is you end up reading a lot of clinical trials.

Could you talk about what that means?

Dr. Frank Buono: Absolutely. What I mean by one by three is we had three different groups and each group was different. So we, one group was a baseline group or what we call treatment as usual group. You'll hear me say tau a lot. That's just the acronym for treatment as usual. They were exposed to what is currently out there, which is just literature that we have been given by the pain associations about how to treat chronic pain. One group was given the I can cope mobile application, which they were, they received 24 hours a day, seven day use of access for the mobile application for over two months. They could look into all the different content in this and the mobile application. And I'll go in a little bit of detail about what that is. So The idea of the I-Can-Cope was to check in daily and they would ask five questions very briefly which would take less than a minute. So, how'd you sleep? What's your current pain? How much physical activity have you had? Um, and so on. And it would be on a five point Likert scale. Then there's the trend area, which would allow you to graph. your pain with your other different activities. So it would allow the patient to actually see and visualize. Well, I know when my pain is really, really bad, it's a five out of five. I'm also not sleeping well. And that realization is in graph form was a key feature within this mobile application. the third section was goals and we want the individuals to be responsive of their pain. And so we advocated for, okay, We know you're in pain, but let's see if you can actually take some of the literature that we get from the library section and use it and apply it.

So make a goal, like go out for a walk socialize with a friend go to a church meeting or some sort, right? Do what you need to do to increase your activity or, or mindfulness, right? I had lots of patients anecdotally who said, I want to get eight hours of sleep. sleep. was always a big factor, right? And so they, I had several patients who would say, I met my goal of sleeping for six hours straight. It was the best sleep I've ever got. Uh, and then the library feature had this like over a hundred different articles about how to deal with your pain. What could you do about food and diet and learning about NF along with like activities. So that was the second group. And then the third group in the one by three was we added a contingency management. So in essence, we incentivize individuals to be on that mobile application. So based on certain activities, they did number of times they checked in, they were paid every week for being on.

And we just compared those three groups in pain severity, pain interference along with other psychological pain values the findings were quite remarkable. Individuals in the, in the towel group actually got worse between the baseline and two months evaluation while both. The I can cope group and the I can cope group with contingency management reduce their pain interference significantly down. We actually have the full paper is being currently reviewed in the journal of pain which hopefully will be out by next month or so, but it's, it's pretty good . The first time a mobile application specifically for NF1 has been out there and the goal of that project was to make it for someone who's in Idaho or Montana or Arizona where they don't have specifically NF related clinics where they can go in and say, Hey, at least I have some treatment options for that patient. And that's that was the goal.

Shane: And so first off, is that app still available

Dr. Frank Buono: Yeah, the app is on the app market. You can find it on android and we are Doing one more trial as we speak. So we'll be asking for volunteers to participate in the study, in a large scale trial across united the united states canada and europe that study should be hopefully starting in a couple months But the goal would be a full release of the mobile application conditioned after that study is done.

Shane: Oh, okay. All right. So right now, so is it considered kind of like a beta application? Like people can go and download it, but it's, you're not, it's not necessarily considered like fully released.

Dr. Frank Buono: It's not a full released application. Yeah, you see, you're exactly right. You, you probably have this better language than I would on this, but it is out there, it's available. But it requires an ID and session code. So if people are really, really interested they can always contact me and I can see if I can get them on some type of, trial.

Shane: Got it. Okay. Do you have a timeline on, so is it just after this next trial you want to fully release it? Or is there like a feel for that? And maybe I'll put it in the show notes once it becomes widely available.

Dr. Frank Buono: Yeah. And I think the goal here is that once we have completed this big trial this fully functional randomized control trial we intend to fully release it for free to anyone with NF one. And. You might be saying, or the individuals who are listening to this might be thinking, well, what about NF two related Fortosis or n I, in both variants, we are currently doing that as well.

We are starting a trial with that as well because we know pain exists in both of those populations as well. It's

Shane: All right. So let's talk about management strategies then. And we can talk about it in relation to the app, as far as what worked in the population that you studied or from your own kind of, the experience with the clinic at Yale or however you want to approach it.

But, from your experience thus far, what have you found to be effective pain management strategies? How should someone with an F1 or, or an F2 or, whatever, how should they approach? Managing their pain.

Dr. Frank Buono: very complex and difficult question. I will tell you that and I will tell you that there is no specific answer to give you my. advice is though, I would have any patient who wants to really tackle. And by I say really tackle, they have to be their own advocate or they have to have an advocate who is willing to help them.

But. There's this rule in pain management that we call it the 33 33 33 rule, which means 33 percent of the population will want to actively go and get, seek treatment for their symptoms. 33 percent want to do treatment. But don't have the initiative to do it and then 33 percent don't want to do anything They just want to you know, sit around with their pain the entire time So I think if we can have more individuals in that first group that actively want to help themselves I think that is the first goal, right? And once they are open and responsive to dealing with their pain, just like with their, or with their disease I think it allows for a patient to be more receptive to other options.

Shane: And maybe that means too, just to put a coda on that concept , cause we talked about the knock on effects of chronic pain and how they can affect somebody psychologically and all these other things. And it may mean. that as part of that pain treatment protocol, they also need to address maybe if they have depression or if, they're experiencing, chronic anxiety because of their pain or because of their disease they'll need to address those things at the same time, or, before there'll be able to effectively address pain or before they'll be able to get that kind of motivated state that you're talking about, they may need to overcome another kind of pain, psychological hurdle first as part of that.

And I think that that is something that probably shouldn't be, glossed over and easily could be in, your average kind of medical setting that the doctor's not going to ask, Hey, by the way, how are you feeling overall? Psychologically like their doctor, if it's an NF clinic or whatever is going to ask about their pain and they're getting, they mean, from a biological standpoint, like, do you experience pain and how bad is it?

Dr. Frank Buono: And I, I think you're probably right. You're right on a lot of those variables. There is a high comorbidity or what means a co occurrence of depression and or anxiety with Chronic pain well across multiple populations as research galore about this I just published another article about chronic pain within NF1 patients and Co occurrence of depression and anxiety.

I think it was like 40 some percent for depression and 30 percent for anxiety. And, and to a certain degree, you are also right. That depression and or anxiety can be glossed over. I generally, you'll get the how are you feeling, and if a good doctor, or I'm gonna say a doctor who is listening , receptive here's any tremor in the voice, they should be asking second and tertiary follow-up questions. But yes, you are right that, from a psychological standpoint, you should be looking in at comorbidities because I think that is a critical piece.

Shane: And some of the pain management strategies that, you talked about as being part of the, I can cope trial would probably address both of those things at the same time. Like mindfulness, has been shown to improve depression in folks that are afflicted by it. So maybe let's talk about, knowing that every buddy's approach, what works for them is going to be a little bit different.

Maybe talk about some of the strategies that were employed in the trials or that you've found have had success with patients.

Dr. Frank Buono: So I think for chronic pain, there is a gold standard within the psychological framework called cognitive behavioral therapy. It has been shown across multiple populations across multiple age variants to be effective in helping reduce pain versus opioids or other angiolytics or other medications, right? I think that is clearly the research speaks for itself in that sense. Other therapies like what you're mentioning mindfulness based interventions. My mentor is totally mindfulness intervention based. And she as shown in her clinical trials that. MBI has been effective in substance use, effective in smoking, effective in chronic pain populations. So it is therapy of some sort, and we're talking third wave psychotherapy. So dialectical behavioral therapy, acceptance, commitment, therapy, cognitive behavioral therapy, all have been shown to be effective mediums. To help guide the patient to understanding about their pain. That, that, that's the psych perspective, right? I would also, mention that there is a physical component as well. For me when I was in, I mean, I'm in chronic pain every day. After my surgery I was, just really briefly, I was paralyzed from my hips down. I had to relearn to walk and it took two years of intensive physical therapy for, to get me where I'm at right now. But I still deal with chronic pain every day. So one of the things that is effective for me and one of the things that I published on is yoga to get the body moving . In low, we're not talking power lifting here.

We're, we're not lifting 500 pounds. We're doing stretching and movement of the body to release pressure. I think that is something which is not talked about enough. Obviously not everyone, can do yoga, especially if they have a metal stint in their body, on their leg or, a rod in their back, it's going to limit that.

But I would also mention and say that I have had individuals who are been in my trials, who have had rods in their back, who have done some seated yoga positions and never felt better in their life.

So there is lots of physical activity exercises, which are low impact, that can help massage, can help, can potentially help.

Acupuncture, acupressure are been shown to be effective. So there, there is other interventions out there besides, opioids or medications to facilitate, uh, reduction of pain.

So a big component here of what you're describing is that movement aspect. Getting out. moving, doing some kind of maybe resistance training of some kind, adapted of course, for the mobility that the patient has that the person experiencing the pain can do.

Shane: And that may mean modifying it. And like you were saying, yoga can be done in like a seated position. Tai Chi can be done in like a seated position. But just that, that movement has a tendency to help with chronic pain. And, just that kind of just the process of moving your body and, stretching the ligaments and muscles and all of that, that, that has a tendency to help with pain just on its own in your experience.

Dr. Frank Buono: It was without a doubt. If anything, it takes your mind away from the pain that you're having at that moment. to focus on the activity at hand. And I think if, if it does nothing at all of, making you less painful, at least it's taking your mind for however long, two minutes, three minutes out of that, I'm in pain perspective and giving you a time to be, Oh wait, I'm doing, one, I'm doing downward dog, pose and, I can just focus on that for two minutes. And so that is two minutes of your day that you've not been in pain.

Shane: Yeah. And I think too, with some NF1 patients in particular with, either like bone deformities or scoliosis or whatever, with physical therapy. the focus on movement patterns that can lead to that kind of chronic pain and kind of unlearning of those bad habits in some cases can probably help reduce the amount of pain you're experiencing as well at any given time, I would imagine.

Dr. Frank Buono: Absolutely. And, and, the funny thing is I, I'm going to throw my wife under the bus here for a second, in the sense that, she's an occupational therapist and how much pain cases that she has on a day to day basis. And just movement through occupational therapy. And she's anecdotally just shows that it's been effective in reducing that, but one thing that you need to be, I think we all need to be mindful is that the willingness to try is a very hard aspect in, in getting someone to get to that point where they're willing to try, that is the hardest variable that we have. From a clinical standpoint , right? If you are so not willing to try, you're not going to have as much effect in your Tai Chi or any activity that you're doing. So I think if I had one message for the audience, it's please just try it. It might not be effective the first time, the second time or the third time, but maybe after the fourth time you see some effect. And if you don't try another activity, try something that, I'm not just saying yoga is the only thing out there. There are lots of different activities

Shane: Yeah, I think that can be a challenge, for someone that experiences pain when they move in their experience, they can become kind of afraid to make things worse but the reality is that, sometimes, moving past the thing you fear is the only way to grow.

I mean, I think that's even true here in this case. Sometimes you have to, which isn't to say, of course, be reckless, I don't think anybody is, recommending that obviously, Do it as much as possible under the advice of a care team, a skilled physical therapist, whenever possible, especially if you have, like you were saying a rod in your back or something like that, where there's obvious complications and there's a role for drugs to play.

But I think to your point, people will find probably that doing something is always going to be better than doing nothing when it comes to. everything, including their pain. Right.

Dr. Frank Buono: 100%. I think that if you have the ability to move, and, I've known many patients. personally with NF1 who don't have that luxury of movement. They are wheelchair bound. They can't move anymore. But if you do have the ability to move , utilize it because it is, we'll never know how much you have, just to move a quarter inch more with your arm might be the difference in in reaching that can that you can't on the second on the second shelf.

Right. And so it will help the quality help your quality of life. It will help you. Be better than what you currently are potentially. If I mean that, that's just my own personal perspective because I've seen it time after time after time after time in both My own experience in advocating for patients with NF for the last 20 years in my research and in the clinical space.

Shane: Yeah. And, if you have any resources, like maybe some of the stuff that you used for the, I can cope app or anything like that that we can link to that'd be super helpful. And I'd really like to explore the CBT, component and the kind of the dbt, like those, those psychological approaches, I'd like to explore those more details, so I might, try to find.

Guests for the podcast, I can talk to that a little bit more. I find it very interesting that, you remarked that just the act of sharing the fact that they're having pain with someone else talking about their pain, seem to be pain reducing in and of itself. And I'd really like to explore that a little bit more.

I think

Dr. Frank Buono: are some great self guided workbooks out there for chronic pain in both an act standpoint and in CBT's literature as well. That I would highly recommend. I, I know the individuals who have created them and I can fully endorse them. And I will

definitely pardon a

Shane: we say, when you say act, cause I don't think we actually, I know we went through the. We're talking about acceptance and commitment therapy when

Dr. Frank Buono: percent except, yeah. Steven Hayes is the founder of ACT Therapy. He wrote a book, get out of your head and into Your Life. It's a workbook. It's a fantastic, self managed workbook that you can do by yourself without a therapist, or you can use this in conjunction with therapy. And I can Rachel Zoffness who is a prominent CBT child psychologist out in California. She has written two books specifically for a pediatric population about workbooks for, from based on the CBT framework. And I can put these links to you in

Shane: Yeah. Anything you think anything that you think, especially from a self help standpoint, because you're right. The reality of a lot of folks we're talking to is, regardless of what, even they may have an NF clinic. I mean, we experienced this even with our NF clinic sometimes, right. Where.

Child psych or developmental psych or whatever is not a component of their care team anymore for whatever reason or Wasn't to begin with or whatever and so them going out and finding those resources or getting guided help may be a lot more difficult and so any resources that we can link to that people can engage with on their own, I think would be really helpful.

Dr. Frank Buono: Absolutely. And I think that, everything that I'm going to share with you, whether it's been peer reviewed or I'm, or I'll call it Frank approved kind of thing, in the sense that I have, I've either done the books myself or I, as a patient or I, been trained in that.

Oh, I know it. And so it's, but once again, it's, it's, you can link the resources, right? It's the old adage. You can take a horse to water, but you can't force the horse to drink, right?

Give the resources to the people, let them see what that's out there, but they're going to be half the ones to actually fill it out and go through.

Shane: yep. It's still going to require that commitment that, no one can, no one at the end of the day, people we can advocate, but that, somebody actually experiencing the problem, it requires their engagement to do anything about it. And it's really up to them. It's their life at the end of the day.

Dr. Frank Buono: Absolutely. And I, I will also mention that, there are several therapists out there, pain psychologists that are always willing to help. I have a list of them based in different states who are there for you if you need it. And they're not just MDs there, in the sense of, prescribing medication, whatever it is. More behaviorally based kind of thing. So, when in doubt advocate for yourself, reach out to people. I'm always willing to, help people. I get emails daily.

Shane: Yeah, I think that's a good point to end it on as we're up on time. And I don't want to dig too much into your lunch hour or anything like that. And you've been so generous with your time already.

Dr. Frank Buono: Yeah, no, I, um, anytime I can talk about NF, I'll talk about NF. And even if it goes past, the allocated time, I don't, that it's worth, it's a worthy benefit. I think. for individuals. If you need help talk to your provider, tell your provider that you're in pain, be honest with your provider. If you are in cry pain and you are depressed or you have anxious feelings, tell them hopefully that will give you the best treatment options that there are out there. But if they don't, Then you need to advocate for yourself, and you need to be mindful what is out there. And so, be reading research, be looking at different approaches to help you with it.

Because it's not just necessarily one response which will make your pain go away.

Shane: Yeah. Might be a multifactorial approach and it may be, it's going to be a lifelong journey at the end of the day. None of these disorders are going to go away. I mean, one day, hopefully, maybe we'll have a cure, but as least as it stands right now, this is something that just like with all other components of NF that you're going to live with.

And so learning how to manage it over the lifespan, and that may change as you age as well, as an important component that you may find that something that worked for you for years, you may have to shift tactics, right?

Dr. Frank Buono: And you make a fantastic point, which we didn't touch on, which is, As, you go from adolescence to young adult, to adult, to older adult, to to the elderly population, you are going to need different resources. And it is critical for you to understand that, resources are going to be different based on what physical cognitive developmental aspect milestones you are at. So you need to be You know, mindful of that or have a team that is mindful of that.

Shane: Yeah, absolutely. All right. Well, thank you so much. Is there anything else just as a final parting note that you want to impart? Any specific resources you want to call out anything that we didn't already touch on that you really wanted to mention before we call this to a close?

Dr. Frank Buono: Yeah. I think that the NF advocacy organizations need to be shut out here. I think that we need to make sure that you are reviewing these organizations, network, there's NF Northeast and whatnot, and Children's Tumor Foundation, which all have, you ways for you to find providers, specifically medical based, but that could be at least the first point of contact that you have. To find one, a provider that focuses solely on that. That's the first part, and then you build out your team based on the needs that you need. If you are having, issues with your spine, you might need to get a neurologist on the team who specifies in spine. So it's trying to know what you need. And I think those are the in initial resource resources that could be beneficial for you. From there always ask, go to the conferences, go to the, be social, try to talk to others, find out what has been effective for other individuals, and it might motivate you to. help others. One additional point that I'm going to throw in, I'm sorry, you just open Pandora's box for me here. And so

I have uh, one story that I'd like to share. One of the features that we wanted to add. But we didn't, was a community model in the mobile application. And the idea was, one of the focus groups that we did post this trial we had patients who had NF1 who never knew another patient with NF1. And just the presence of mind of knowing someone else who has NF1 who can relate with you. or NF2 schwannomatosis or whatever, that psychologically is incredibly powerful. And so having not just a clinical team, but a team of individuals for your child or for yourself is something which, I can't emphasize enough is Psychologically helpful and he can heal you as well in the incentive from a psychological perspective

Shane: Yeah. Diseases like this can be very isolating, and they, while, while NF1 is, there are thousands and thousands of people with NF1, for instance still, in a population of, in the United States, 330 million or whatever in the world of billions, That's still, not a massive community.

And it's not like you'll likely run into somebody or that outwardly presents any way with say NF1 in your daily life, if you don't have some mechanism by which to connect with them. And I think that's a really important point too, because it really is important, especially on the pediatric side, I can speak to, how important it is for.

Someone to, to interact with other people that can relate to their experience in that way.

Dr. Frank Buono: Yeah, and I mean there's Yeah, you can go down this for a while. I mean, with NF1, based on the physical presentations, there could be some stigma. There could be, many psychological determinants to how an individual wants to present and having someone who, goes through this as well with you. can be even more impactful than, I might even go as far as saying a parent, because if the parent doesn't have the disease, they don't, they can't relate. They can, they can empathize, but they can't relate.

Shane: Yeah, that's really true. As an advocate, the best thing, we can do is that empathy, but at the end of the day, there is nothing like talking to somebody that can, like you said, very specifically relate to your day to day lived experience.

Okay. So on that note I think we can call it here. I really want to again, thank you for taking the time and going over a little bit on your schedule. I know you're super busy to talk through all this stuff. The resources that we mentioned here, what I'll try to do is link to everything we can in the show notes.

If you want to just shoot me some of the stuff that you were talking about, especially the self guided stuff, that'd be great. And then also too if, from that psychological standpoint, from the CVT practitioners, et cetera, if you know anybody that you think might be open to talking about that on the show as well, absolutely.

Send me that info too.

Dr. Frank Buono: Absolutely. I can give you a list of people.

Shane: Definitely inundate me because like I said, this is a, I want to make sure as part of because at the end of the day folks that are coming to this podcast, if they're listening to it, they're listening to it. It's a it's a resource hopefully amongst many, but sometimes it may be the first thing they found or the only thing they found.

And so part of what I see as the role of, decoding an F here is to make sure that I have a resources page that kind of, like you were saying, lists, the various advocacy organizations, and also List as many resources in one place as possible because, the volume of information out there too can be really daunting.

And it's amazing how you can spend hours and hours and hours researching something and not come across the most helpful thing that you can find. And then, Years down the road. I've had this happen with my son where I come across a resource that ends up being super helpful. And I've been researching this for years.

Do you think I would have come across it? So the more kind of points of contact that people can have with those resources and the more that we can expose them to it, the better. And so I just see, this podcast as one component of that, but I want to make sure I'm doing as much as possible by putting as much out there as possible.

Dr. Frank Buono: Absolutely. And thank you for doing this because I've been thinking about this for years and, saying we, it should have been done, five years ago, 10 years ago, but, but it is fantastic that you're starting this now.

Shane: Yeah. And and, the more kind of perspectives we can get, the better I really want to touch on the psychological component. I really want to talk to folks about, like IEPs for kids, but also, with the NF1 in particular, because it's progressive and because, the pediatric impact is so great.

The adult side of the coin does, Fall by the wayside a lot. And I want to make sure that, we're touching the other two thirds of people's lives as part of this process as well. And so I really want to talk about the adult lived experience as well. I want to make sure I'm not neglecting that.

Dr. Frank Buono: Absolutely. And that's a a great insight on your part because there are many clinics do solely pediatrics, but there are very few adult clinics,

and I think that is something which needs to be done because, after you're 18, 25, you're out of that peds world, and you need a point of care

Shane: It can be just as hard as an adult as it is as a kid. The reality is, what I think what happens is as the pediatric care is very holistic and then. When you like, when you turn 18, it shifts and then you become, then it becomes like fragmented, you're have oncological care.

You have, like you said, the psychological care, but they're all independent of each other. Not coordinated in the way that hopefully they were as a pediatric patient. That is something that I think is harder to do. But that we really need to do. And until that happens, until there's a world where there are more adult NF clinics or NF clinics that even take adult patients, like you were saying the patient's going to have to be their own care coordinator , and that could be hard if you're a lay person, at the end of the day, knowing what care you need sometimes is a really hard hurdle to begin with.

Dr. Frank Buono: and having the resource of this, Shane, is important because, if it opens the mind of a patient to say, Hey, maybe I need to look at it from a different perspective, or at least there's, there's a resource here that can provide when I'm ready to look at it, I will look at it. And I think that. Is

a critical piece for individuals who are suffering from this disease or these diseases

Shane: Yeah. So I'll, so like I said, I'll link to your profile in the show notes as well as resources that you provide. And then are there, are you on socials and stuff? Do you want to link to any other ways of reaching you or the work that you're doing at Yale?

Dr. Frank Buono: So I will tell you I am not socially Uh inclined in that sense, i'm not on twitter or linkedin or you know any of those, Mediums, I guess, but, uh, I think that my profile or from Yale perspective, you want to reach out to me. You have a question. I'm always open to trying to help.

I will tell you that I will not one thing I will not do is answer a specific question about a patient, without doing a full workup on the patient. Right. Cause I just

can't. Yeah, there's just too much issues with that, but at least I can listen and maybe help find resources for individuals.

Shane: And there is for these interviews just so you know the medical disclaimer will be at the top of the interview prior to every episode. So just to reiterate, no doctor patient relationship is being formed here. And this does not take the place of seeking medical care from your medical care team.

Dr. Frank Buono: Absolutely. Yeah. But yeah. Otherwise, reach out to your NFO organizations and sometimes the NFO organizations reach out to me for help. And it's not the first time I, I generally get like five to 10 emails per month from each of those advocacy organizations asking for help.

Shane: Which just goes to highlight how important they are from a

coordination standpoint and, just highlights that regardless of how alone you may feel at a certain point. That you're not, and that there's a lot of people that are dedicated just to trying to connect you with resources that can help.

So definitely, at decoding NF. com, go to the resources, tab there and reach out to one of those organizations, whether it's, CTF or NF network or, some of the other links that are in there. And I guess until next time I talk to you, Frank, thanks again.

Dr. Frank Buono: Thank you. It was great talking to you.

Shane: All right, that's it for today's episode. Like I said, I hope you found something useful in that interview, something actionable that you could take away to hopefully make life a little bit easier for you or someone that you care about.

As we talked about in the interview. All the resources will be linked in the show notes. So please check those out either in your podcast app, or you can go to decoding nf.

com to check those out. And if you have any feedback, please feel free to reach out to me. You can go to decoding nf. com or just email me directly. Shane at decoding nf. com.

Of course, I want to thank Dr. Buono for being so generous with his time today. And I want to thank you for listening.