Ep 299 – Prader Willi Syndrome: “I Have a Client Who . . .” Pathology Conversations with Ruth Werner

0:00:01.6 Ruth Werner: Hey, I Have a Client Who listeners. Did you know I have a growing library of NCB-approved one-hour online self-paced continuing education courses that you can do anytime, anywhere? Well, now you know. Current classes include what's next COVID-19 updates for massage therapists and a massage therapist introduction to pharmacology part one. And brand new, a massage therapist introduction to pharmacology part two. Classes are $20 each and they confer one hour of continuing education credit. Want to know more? Visit my website at ruthwerner.com and check it out. Be sure to sign up for my mailing list so you'll never miss a new class.

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0:00:56.0 RW: Hi, and welcome to I Have a Client Who, Pathology Conversations with Ruth Werner, the podcast where I will discuss your real life stories about clients with conditions that are perplexing or confusing. I'm Ruth Werner, author of A Massage Therapist's Guide to Pathology and I have spent decades studying writing about and teaching about where massage therapy intersects with diseases and conditions that might limit our clients health. We almost always have something good to offer, even with our most challenged clients, but we need to figure out a way to do that safely, effectively and within our scope of practice, and sometimes, as we have all learned, that is harder than it looks. A couple of weeks ago, I put out a call through my various ways of doing this for people pleased to contact me with new I Have a Client Who stories because it's sort of gotten to the end of my queue. Oh boy, did you deliver. Keep them coming, of course, but I am happy to let you know that I now have a bursting pipeline of really good I Have a Client Who stories.

0:02:06.7 RW: And in this round, I got several that are about some fairly obscure diseases and conditions that you might never see. I was tempted to pass them up because why would I put time and effort into creating a podcast about a situation that most listeners might never encounter? But on the other hand, they're real. They affect human beings, they can provide some good exercises for thinking through risks and benefits of massage in situations where you probably won't find any other specific kind of guidance. So, there will be a few upcoming I Have a Client Who episodes focused on rare conditions, because no matter how hard I and my fellow pathology educators try, we will never be able to write down all the things about all the things. What we can do is provide a thought process that you can use when you are faced with something like today's story, a rare condition that seriously affects a person's quality of life and their personality with virtually no information about the crossover between it and manual therapies.

0:03:12.9 RW: So keep listening, not just to hear about Prader-Willi syndrome, but to walk through a thinking process with me that can get us to some sound clinical decisions for safe and effective bodywork. Prader-Willi syndrome? I hear you ask. What on earth is that? Well, let's start here. Our contributor says it's important that this be completely anonymous because this is such an uncommon condition. They tell me the client is in his mid 40s and because of his condition, they say, I can't have anything edible in my waiting area or my office because he will eat everything. He once ate an entire fish bowl full of mints, probably 50 of them, and he ate them in about a seven minute period. Another time a coworker didn't know what was going on and left out a platter of biscotti and he ate 14 of them in five minutes. And he also drinks all the bottled water. But he has numerous physical problems related to his condition and he gets massages nearly every week. He does a lot of physical activity. He has a live-in assistant who provides him food at appropriate intervals.

0:04:16.9 RW: There's nothing else edible in his house. He has a pronounced spinal curvature. His legs are different lengths. His muscles are always painful and tight. And I'm fine working on him. I know that nothing I do will significantly change anything he has going on, but I can help him maintain some mobility and flexibility. But he complains more than any human I have ever met. And I know that being a jerk is not typically an anatomical or genetic problem, but this is literally part of his condition. I've had some success with reminding him that I'm doing the best I can and he is the expert of his body, but sometimes it's nearly impossible to make myself walk into that massage room. Luckily, I have told him that if he is ever mean directly to me about myself, I will leave the massage and that will be the end of it. I had to do that one time and one time only and he got the message.

0:05:15.9 RW: So I guess my question is, are there things I can do for a person with this very unusual condition that perhaps I haven't thought about because I'm spending all my energy just trying to be kind to him? Any techniques or stretches or anything that would be particularly beneficial? I do want to say, listener, this is Ruth, that I actually edited that last bit because our contributor was more descriptive in the way that they expressed themselves. It just sounds like this client is a challenge on many, many levels. So let's talk about Prader-Willi syndrome. By now you will have gotten that it has something to do with appetite, but there's more, so much more. Prader-Willi syndrome or PWS is a genetic disease that was first described by Andrea Prader, Alexis Labhart and Heinrich Willi in 1956. It was once known as HHHO, hypogonadism, hypotonia, hypomentia and obesity, and all of these for reasons we will get to shortly.

0:06:16.7 RW: PWS involves changes on chromosome 15, and these changes usually happen sporadically and randomly, but there is one version of this condition that is inheritable. And the results of those changes vary a lot. It occurs on a scale of severity. How exactly it causes those changes is actually a bit up in the air. But this genetic anomaly appears to affect the hypothalamus and other endocrine glands. This, of course, has a profound effect on the secretion of hormones, and we see it particularly affecting growth hormone and ACTH, that's adrenocorticotropic hormone, along with some others. Prader-Willi syndrome is rare, occurring in about one out of every 15,000 life births. We think about 20,000 people in the United States have it, and about 350,000 people worldwide. The life expectancy of a person with PWS is shorter than average. The oldest person on record who has it right now is 68. Prader-Willi syndrome appears in two stages. The first signs, which are observed very early during infancy, include weakness.

0:07:27.2 RW: These are floppy babies with a weak cry and a poor sucking reflex and delayed motor and language milestones. And then starting around age three and persisting for the rest of their lifespan, people with PWS develop hyperphagia, or too much eating. They lack the normal sensitivity to satiety. So it is literally impossible for them to know when they're full. They cannot stop eating. To add to their problem, their metabolism, which is to say the efficiency with which food is turned into energy, is slower than we see with the general population. So now we have a young child who can't stop eating and they gain weight quickly, but their growth hormone secretions are abnormally low. And this signals turning incoming fuel into new cells and consequently, people with PWS often have a short stature and hypothyroidism and central adrenal insufficiency from the low levels of ACTH from the pituitary gland.

0:08:33.1 RW: They may also have distinctive physical features, small genitalia and infertility and small hands and a head that narrows at the temples. And often they have painful, limiting scoliosis. Many patients have eye problems in the form of myopia or nearsightedness or strabismus, that's the misalignment of the eyes. And they are also prone to diseases or complications that go along with being overweight and obese, especially if they don't have the physical strength or ability to compensate. They are therefore vulnerable to diabetes, cardiac insufficiency, sleep apnea, edema and skin ulcers. They can become so desperate for food that they might eat spoiled food or garbage, leading to infections and really severe GI tract damage. In addition to all these physical challenges, people with Prader-Willi syndrome also often have cognitive impairment and problems with emotional regulation. They get angry, they throw tantrums and they're prone to OCD behaviors like skin picking or other forms of self-injury.

0:09:39.6 RW: So this is a really complicated situation, isn't it? With aspects of constant extreme and unrelenting driving hunger and a need to eat anything or everything all the time. Plus hormonal problems and complications related to overeating and a host of mental and emotional challenges. Sometimes Prader-Willi syndrome is treated with some hormone supplementation, especially in childhood, and then with ongoing individualized care to control food consumption and to promote physical, cognitive and mental health. Gene therapy to correct this anomaly is on the horizon, but we are not there yet. So let's review a shortened version of our contributor story so we can see how all of this information applies to our client. He's in his mid-40s. That's getting up there for someone with Prader-Willi syndrome. He cannot have any food in his house. A caregiver gives him all the food that he's allowed to eat. He cannot be left alone with food or drink. He's active, but he also has pronounced spinal curvature. His legs are different lengths. His muscles are painful and tight. And he gets massage about once a week.

0:10:50.3 RW: Our contributor goes on, I'm fine working on him. I know nothing I do will significantly change anything he has going on, but I can help him maintain some mobility and flexibility. But he complains more than any human I have ever met. And I know that being a jerk is not typically an anatomical or genetic problem, but this is literally part of his condition. I've told him if he's ever mean directly to me about myself, I will leave the massage and that will be the end of it. I had to do that one time and one time only and he got the message. My question is, are there things that I can do for a person with this very unusual condition that perhaps I haven't thought about because I'm spending all my energy just trying to be kind to him? Any techniques or stretches or anything that might be particularly beneficial? Thank you so much contributor. I've had occasion lately to chat with people about working with especially challenging clients, but this one seems to be on a whole different level. But a couple of things are going on here that I think are worth pointing out. One is that this person is physically active.

0:12:02.1 RW: I don't think that's common or typical for mature people with Prader-Willi syndrome, and it's probably helping him to stay alive. The life expectancy for this condition seems to be largely dependent on how well they are able to control their food consumption and weight gain. And also, he's in a lot of pain with scoliosis and other structural problems and tight muscles, so it's very much in his best interest to manage this as best he can so he can stay as active and as healthy as possible. And it sounds like you're on the right track with him. Based on this much information, it's clear that massage therapy isn't going to fix this client's situation or attitudes about food or mood or emotional regulation. Although he might learn to be more polite with you if he wants massage, and that's a bonus. But besides encouraging this massage therapist to keep great boundaries for self-care and their own well-being in the presence of an emotionally demanding situation, I don't see a big downside to massage here. Are there special cautions we should bear in mind? Sure.

0:13:04.9 RW: It's important to know about this person's cardiovascular and endocrine health because diabetes and heart disease are common complications of Prader-Willi syndrome. He might have skin ulcerations or other complications that need checking too, but the fact that he's active and wants to stay active tells me he's probably a good candidate for massage. With respect for muscle tightness, pain, and scoliosis that we would use with every client, this person with Prader-Willi syndrome can use massage to stay as mobile as possible, and that is a great goal. And maybe, just maybe, he will be able to take the opportunity with you to experience his body in a positive way. This contributor or other listeners who have clients who are emotionally challenging or really anyone in practice, we make this invitation to everyone, don't we? Come to me and experience your body in a positive, healthy, affirming way. Clients might or might not accept that invitation. That's up to them. But I'm willing to bet that on a really deep level, this person is so grateful for your work, even if he can't always show it.

0:14:16.3 RW: Hey everybody, thanks for listening to I Have a Client Who, Pathology Conversations with Ruth Werner. Remember, you can send me your I Have a Client Who stories to ihaveaclientwho@abmp.com. That's I Have a Client Who, all one word, all lowercase, at abmp dot com. I can't wait to see what you send me, and I'll see you next time.

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