Misunderstood and Overlooked: hEDS, FND & Autism | Office Hours

[00:55] Dr. Linda Bluestein: Welcome back, every bendy body, to the Bendy Bodies Podcast with your host and founder, Dr. Linda Bluestein, the Hypermobility MD.
[01:17] Today, we're going to be doing another episode where you guys are my guest. I'll be periodically doing these solo episodes. So be sure to submit your questions on the Bendy Bodies website, bendybodiespodcast.com.
[01:28] In this episode, I will be covering how to handle suspected Ehlers-Danlos syndromes in young children, low-dose naltrexone — when it is useful, and pros and cons — functional neurologic disorders and why these are often misdiagnosed in people with EDS and related conditions, what to do if your diagnosis changes, and so much more. As always, this information is for educational purposes only and it's not a substitute for personalized medical advice. Here we go.
[01:59] Okay, so the first question is from Sabrina. Sabrina says: "Hi, Dr. Bluestein. I've been listening for a couple of years now. I came across one of your videos and I've pretty much listened to all of your episodes. I was kind of wanting to make a request. Being active in online communities, I have noticed that a lot of us with neurologic symptoms and disorders have been diagnosed with functional neurological disorder. I would love to hear more on the neurological stuff as it is very intriguing to me. I do also want to thank you as you have helped me so much personally and with so many of my doctors. I appreciate what you do and will always recommend your podcast to other zebras."
[02:38] So I love this question because I feel like there's a lot of misunderstanding around functional neurologic disorders. I actually had a patient once who was in physical therapy school and was studying to become a physical therapist, and she was actually diagnosed with functional neurologic disorder. I will share with you what ended up happening and helping her in just a little bit.
[03:02] So functional neurologic disorder — let's start with what that is. It is a medical condition in which a person experiences neurologic symptoms such as weakness, movement disorders, sensory issues, or seizures that are real and disabling, but not caused by structural damage or disease in the nervous system.
[03:21] Key findings of functional neurologic disorder: the symptoms are real and they arise from problems with how the brain and the body send and receive signals, not from visible damage on scans or tests. This is very important because I think there's a lot of misunderstanding both in the medical community and amongst patients that functional neurologic disorders are not real conditions and that people don't experience real symptoms — but they are real.
[03:48] Common symptoms include limb weakness or paralysis, tremors or abnormal movements, non-epileptic seizures (also called dissociative or functional seizures), speech or vision disturbances, and numbness or sensory changes.
[04:03] What can cause functional neurologic disorder? Physical injury or illness, psychological stress or trauma, and sometimes no clear trigger is found. Functional neurologic disorder is based on positive clinical signs, not just ruling out other diseases — such as inconsistencies in movement or strength that suggest a functional origin.
[04:23] Remember, again, these are real conditions. I've had so many patients that have been diagnosed with functional neurologic disorders and then kind of were just abandoned. We still need to treat people that have functional neurologic disorders, but it's important that they get pointed to the right resources. What we really need with these conditions is a multidisciplinary approach, which often includes neurologists, psychologists, and psychotherapists. Cognitive behavioral therapy and physical therapy can be extremely important.
[04:52] So in short, functional neurologic disorders are genuine disorders of the nervous system function, not structure. And it is important to treat these with the same seriousness and care as any other medical condition.
[05:05] Regarding that patient I mentioned earlier, I want to share a little bit more of her story. When I was looking through her records before seeing her as a patient, I noticed that there were many notes commenting on the fact that she was becoming progressively more weak. So they were sending her for more and more scans. Then there was a big gap in the notes before I saw her. I didn't have any records from the past couple of months — I had a bunch of information that was a few months old and then nothing more recent. Based on the fact that I was seeing in the notes that she was getting progressively weaker, I expected her to come into her first appointment in a wheelchair.
[05:43] She actually came into her first appointment walking, which was very surprising and exciting to me. And I asked her what happened. She said that she was diagnosed with functional neurologic disorder and was referred to a physical therapist who specializes in functional neurologic disorder. This physical therapist knew precisely how to work with her in a way that was really, really helpful and helped her regain strength quite dramatically.
[06:06] That physical therapist then went on maternity leave and she was switched to a different physical therapist. The patient told me that when she was switched to the different physical therapist, she actually started to experience a regression of her symptoms. Fortunately, her original physical therapist came back from maternity leave not too much later, and she was able to make progress again.
[06:32] So this story really illustrates some very important things. Number one, again, functional neurologic disorders are real conditions. And we're going to get a little bit more into how people with EDS are often diagnosed incorrectly with functional neurologic disorders when it's really EDS that's going on, and how you can tell the difference.
[06:49] The other really important point is that if you do have a diagnosis of FND, it's important that you get the proper treatment. Physical therapy is extremely important, but it's also important to work with a physical therapist who has knowledge about FND and knows how to work with these conditions. In this person's case, they were very fortunate that they had access to someone like that and that their treatment was so incredibly successful.
[07:15] Okay, so let's talk about why EDS — especially the hypermobile type — is often misdiagnosed as FND. There can be some overlap of symptoms, and so that's why it can be confusing, and healthcare professionals often misunderstand all of these conditions.
[07:32] First of all, EDS can cause a wide range of symptoms that can include chronic pain, fatigue, dizziness, gastrointestinal issues, and neurologic symptoms such as numbness, tingling, migraines, and even temporary weakness. I think what can confuse a lot of doctors is the numbness, tingling, and weakness that can happen in people with EDS — it is often transient and moving to different places in the body. We're used to thinking of things in dermatomes, coming from nerve roots, and we're used to thinking of very specific nerve roots, not symptoms that travel from different places. But the same things that predispose somebody to numbness and tingling in one part of the body with EDS are going to predispose you toward numbness and tingling in other parts of the body. So it is very possible to have symptoms that travel like that.
[08:22] Some of the symptoms that can mimic or overlap with FND include functional seizures, weakness or paralysis, sensory disturbances, and gait abnormalities.
[08:32] Another reason why these are sometimes confused for each other is that people with EDS often have normal imaging — CT scans, MRIs, ultrasounds, etc. — and also often have normal lab tests. This is also typical in FND; people with FND usually have normal lab tests and imaging as well. When tests don't show clear structural causes, some clinicians assume that the symptoms are psychological or "functional."
[09:01] There's also a huge lack of awareness. Many clinicians, especially those not specializing in connective tissue disorders, are unfamiliar with EDS or its more subtle signs like joint hypermobility or stretchy skin. This can lead to a misattribution of symptoms to functional or psychiatric origins.
[09:19] We also know that there's a huge problem with gender bias. EDS predominantly affects women, and studies show that women are more likely to have their symptoms dismissed or misdiagnosed as psychological, including being labeled as FND, conversion disorder, or somatic symptom disorder.
[09:36] If you have heard me talk about my story, you may have heard me talk about when I was suffering from very severe sciatica related to a Tarlov cyst. I was seeing a physical medicine and rehabilitation doctor at that time, and my symptoms were getting worse and worse. I know, in hindsight, part of that was related to the extreme stress I was under in my workplace — I was essentially being told that if I didn't recover quickly, I would lose my job. So this was very stressful.
[10:04] I remember going in for one particular appointment, and she said, "Okay, we'll order one more imaging study." She then put her hand on my knee and said, "We have to accept, if this next MRI comes back unchanged, that this is all a somatic functional disorder." And it was so patronizing and so embarrassing. I walked out of that appointment in tears. It was so upsetting. And I know that so many of you can relate to this — this has happened to so many of us, where people basically tell us that it's all in our head.
[10:38] So as frustrating as that is, I really think that it's so important for all of us to understand that FND, again getting back to the FND side of things, is a real condition. Don't let doctors or other people try to tell you that it's not a real condition.
[11:00] What else do we see with both of these conditions — with both HSD, FND, and EDS? All of these conditions are also associated with anxiety, depression, and trauma. Oftentimes when clinicians see these mental health symptoms, they may prematurely conclude that the neurologic or pain symptoms are purely functional or psychosomatic.
[11:24] I know I was experiencing a lot of anxiety at that time when my doctor repeated the scan, so I'm sure that was part of why she was thinking this is just psychosomatic. But I also did have that Tarlov cyst on my scans, and it kept showing up every time. She thought that Tarlov cysts were always asymptomatic. I actually ended up going to another doctor, a neurosurgeon in my town, who said, "No, Tarlov cysts can be symptomatic." And that's when I started that whole journey. But up until that time, I was pretty much led to believe that my symptoms were all psychosomatic, which was of course psychologically devastating — it made me feel even more anxious, even more depressed, even more traumatized, which of course then made my pain worse. So that's where we get into these vicious cycles that so many of us experience, and they can be so harmful.
[12:14] We also know that EDS often coexists with autonomic dysfunction or conditions like POTS — postural orthostatic tachycardia syndrome. POTS can cause fainting, palpitations, and brain fog: symptoms that can be misinterpreted as being part of FND if the clinician is unaware of the autonomic link to connective tissue disorders.
[12:35] So, in summary: FND is a real condition. Oftentimes people with EDS or HSD can be misdiagnosed with FND, but FND can also occur alongside EDS or HSD. If someone is diagnosed with FND, it's extremely important that they get the proper treatment, which may include CBT and physical therapy. A multidisciplinary strategy is really, really important.
[13:04] I have had patients diagnosed with conversion disorder — one patient who was in a wheelchair and had been diagnosed with conversion disorder was basically just left with no resources. They didn't offer her any kind of help whatsoever. And that's really what we need to avoid. We need to make sure that we are evaluating people appropriately. And if we do suspect that a person has FND as part of their picture, we need to make sure that we are connecting them with the right resources so that they can improve and resolve their symptoms.
[13:35] Okay, so the next question is from Sally. Sally says: "Hello. I am a family doc, Bendy Body, and longtime listener to your podcast. I recommend listening to your podcast for all my hypermobile patients, of which I have quite a collection at this point. I'm reaching out to see if you have any direction to look, or if you have done any episodes on EDS in the very young population. My almost 3-year-old had a developmental pediatrics evaluation because there was some degree of concern for autism, and they said that it was highly likely that she has EDS — not surprising given my genes. They recommended pursuing aid for her general hypotonia from an EDS perspective but didn't really have any good direction to go. The geneticist they recommended does not do EDS evaluations. I have tried many times to refer patients to him, which is a problem in and of itself, of course. I am one of the more knowledgeable docs on EDS in adults in my rural area, so I suppose I am going to be needing to lead her care. Her primary care is a much-loved family doc colleague who is EDS aware but not particularly knowledgeable in this area. We have struggled with finding her a PT with minimal success as pediatric PTs are very underserved in our area. Do you have any suggestions of where to take a 3-year-old for possible diagnosis and care, or any episodes directed towards young pediatrics? I would love to hear your thoughts on care for the pediatric EDSer on one of your office hours episodes, and thank you for all you do."
[15:03] Dr. Sally, thank you so much for your question. I will link in the show notes an episode that you might find valuable where we talk about autism with one of the pediatric specialists who's been on the podcast.
Diagnosing Ehlers-Danlos in very young children does involve unique challenges, particularly for hypermobile EDS — the most common subtype — because, as you know, we don't have the genetic marker or markers.
[15:26] So what are some things that you want to be thinking about? Number one: the adult criteria limitations. The 2017 hypermobile EDS diagnostic criteria were designed for adults and are not suitable for children due to developmental differences in joint mobility, symptom progression, and comorbidities. There is a biologic maturity requirement: prepubescent children cannot be diagnosed with hypermobile EDS under the 2023 pediatric framework developed by the Consortium on EDS and HSD. Diagnosis is deferred until biologic maturity — post-puberty or age 18.
[16:03] In terms of the 2023 Pediatric Diagnostic Framework, what's important to know is that children with generalized joint hypermobility are categorized into 8 different subtypes.
[16:14] First, we want to be looking at musculoskeletal complications. Do they have dislocations, subluxations, chronic pain, tendinopathies, bursitis? What kind of musculoskeletal problems are they having?
[16:26] Second: do they have skin or tissue abnormalities? Do they have fragile skin, slow wound healing, abnormal scarring, skin breakdown?
Third: do they have abnormal bleeding and bruising?
And fourth: do they have any comorbidities? Do they have autonomic dysfunction? Do they have gastrointestinal problems? What other symptoms are we seeing?
[16:49] The subtypes under the 2023 criteria range from asymptomatic generalized joint hypermobility to pediatric generalized hypermobility spectrum disorder (abbreviated as PGHSD), which includes systemic symptoms.
[17:05] But when it comes to hypermobile EDS and HSD, what are some signs that we want to be aware of in young children?
[17:12] One: joint hypermobility. Children might be able to perform unusual party tricks with their joints, such as bending their fingers or elbows backwards, or be described as double-jointed. They might have loose and unstable joints that can dislocate or sublux, especially in the shoulders, hips, and knees. It's very, very important not to perform these party tricks because even though they might not hurt now, they might hurt in the future. Oftentimes what I will do is videotape a child doing their party tricks and then ask them not to do it anymore. Unless someone is actually in the circus, we should not be doing circus or party tricks.
[17:45] We also can see chronic pain — persistent pain in joints and muscles. And unfortunately, this is sometimes misdiagnosed as "growing pains." We know that this pain can affect daily activities, social interactions, and confidence, and can become a huge problem.
[18:07] When it comes to delayed motor milestones, some children can take longer to crawl, walk, or stand due to joint instability or muscle weakness. It's very important for the pediatrician to do a proper exam and consider the degree of muscle weakness, because it's very possible that something else is causing that weakness. If they do have one of the subtypes of Ehlers-Danlos syndromes, it's possible that they could have some muscle weakness, but it's of course very important to make sure that we're not overlooking a myopathy or something like that.
[18:42] Again, we can see systemic problems like autonomic dysfunction or gastrointestinal problems. Fatigue is very, very common. When I do coaching and see patients, I find a lot of children are very, very fatigued. And unfortunately, a lot of doctors are not doing orthostatic — or stand-up — vital signs. Sometimes people call it the NASA lean test.
[19:04] Something that you can do at home that I think might be really beneficial, if your child is complaining of dizziness or fatigue, is to do a stand-up test or the NASA lean test. The equipment you'll need is a blood pressure cuff, a pulse oximeter, and something to write down the information on. You're going to have your child lie down for at least 10 minutes. After they've been lying down for 10 minutes, you're going to check their heart rate and their pulse. Then you're going to write down the time and set a stopwatch when you have them stand up. Then keep checking their blood pressure and their pulse every minute or two for the next 10 minutes.
[19:40] It's very normal for the heart rate to go up initially, but then in most people it will come right back down. Now, if someone does have postural orthostatic tachycardia syndrome and they're 19 or under, the criteria for diagnosis is an increase of 40 beats per minute. So if your child starts out with a heart rate of, let's say, 70 and their heart rate goes up to 110 and stays there consistently for 10 minutes, that would meet the criteria for POTS.
[20:09] A lot of doctors probably won't diagnose POTS based on your NASA lean test done at home. However, if you document all of this data and bring it in to the doctor, you can ask them to please repeat the test in their office — I would hope that they would be willing to do that.
Also, an important thing to consider is that being deconditioned can affect this information. Say you have a child who's very healthy, gets the flu, and has been in bed for a week. If you were to do this test at the end of that week, their heart rate will go up and probably stay up and it will look like they have POTS. But in order to meet the criteria for POTS, you have to have these types of symptoms for at least 3 months — it needs to be your baseline state, not your state after you've been deconditioned from something like the flu. So that's a really, really important point.
[21:05] Okay, getting back to children and EDS. Sorry for the digression.
[21:10] Skin abnormalities: we see stretchy, soft, velvety, or fragile skin that may stretch more than normal and bruise easily. We could also see poor wound healing with slow healing and thick, stretched-out scars, easy bruising, and sometimes frequent nosebleeds.
[21:25] Other things that we can see include flat feet, high narrow palate with dental crowding or dental issues, fatigue and poor endurance with children tiring quickly during activities, difficulty with fine motor skills such as writing or tying shoelaces, poor balance and coordination sometimes leading to clumsiness or frequent falls, scoliosis or curvature of the spine, abnormal scarring after minor injury, and/or a family history of EDS or connective tissue disorders.
[21:47] A very important thing to consider is whether or not genetic testing should be performed. We know that it's very useful for the other subtypes of EDS except for the hypermobile type. It can of course help rule out the other subtypes, but if you think that a person has some of the red flags — and definitely visit bendybodiespodcast.com to see the red flags document that was shared generously by Dr. Claire Francomano — definitely check out that document for some of the red flags for genetic testing.
[22:23] This is so important because if a child does have genetic testing and it comes back positive for one of the other subtypes of EDS, we can diagnose that child at any age with any of the other subtypes. It's just the hypermobile subtype of EDS that we are not supposed to diagnose in prepubescent children.
[22:41] In terms of the clinical evaluation, we want to perform a physical exam looking to see if the person does have generalized joint hypermobility. Do they have any swelling in any of their joints, any redness, any warmth? Do we have any concern about an autoimmune-type condition? We also want to review the family history and see if there are any red flags — was there any sudden death in a family member younger than age 40? Was there any organ rupture in a relative?
[22:58] We also want to do imaging as indicated — X-rays, echocardiograms — to assess the connective tissue. And we want to be thinking about a multidisciplinary approach: it's very possible that you're going to need a rheumatologist, geneticist, physical therapist, et cetera.
[23:27] Also make sure you're considering all the differential diagnoses. Is there a primary muscle problem going on? Is that why the person is weak? Do they have one of the more rare subtypes of EDS? Do they have juvenile rheumatoid arthritis? What could be some of the other possibilities?
[23:44] Diagnoses like HSD in children are fluid, and reclassification is totally appropriate as symptoms evolve or resolve. Children who have generalized joint hypermobility but don't have any symptoms should be monitored for emerging issues.
[23:58] What are some concerns and considerations for caregivers? We want to be sure to avoid overmedicalization. If a child is doing relatively well, I don't start them on a bunch of supplements and medications. I make sure that they understand some of the considerations and limitations.
For example, we want to be very careful with collision sports. I had a patient the other day who does karate, and I said I'm really worried about karate because they do a lot of sparring — this is something where the person would be at increased risk of injury. So while we don't want to overmedicalize, we also want to make sure that we are making very conscious choices about things like skiing or playing soccer, or other activities where you might be at increased risk of injury.
[24:42] On the flip side, it's important not to completely avoid activity and perceive ourselves as being really weak. We want to focus on our well-being, on our strengths, on our goals, and make really good, smart choices. We want to focus on symptom management, physical therapy, and joint stabilization. We also want to focus on the psychological aspects of EDS and HSD, optimize any anxiety or depression, and if someone has experienced trauma, make sure that we're working on that.
[25:12] I would like to refer you also to the episode with Ashok Gupta. He has an amazing program called the Gupta Program. I have heard from a lot of people that they have found this program to be very helpful in adults, and it would probably be great for teens as well.
[25:28] If you have an infant showing signs like easy bruising, hypermobility, muscle weakness, etc., it's very important to have an early evaluation because you want to make sure that you minimize any risk of developmental delay or organ fragility.
[27:12] How can parents support a child with EDS at home?
[27:16] Number one: help create a safe environment. Remove tripping hazards such as scatter rugs, frayed carpet edges, and wires to prevent falls and injuries. Some children might benefit from having handrails on stairs and in bathrooms for additional support and safety. You might also want to consider adaptable chairs and footrests so they can sit with their knees and elbows at a 90-degree angle, which can provide more stability and comfort when sitting at a table.
[27:38] A lot of children with EDS — and adults as well — tend to sit with their legs twisted and contorted, legs tucked underneath them and all kinds of other positions. Adaptive chairs and footrests can be very helpful. Sensory tools like chewelry or safe chewing tools and sensory bins for calming play and sensory regulation can also be helpful.
[28:09] You want to modify activities to meet your child's needs, allowing for breaks and adapting expectations based on their energy and pain levels. You want your child to maintain as much of a normal life as they possibly can. Encourage safe physical activities whenever possible — choose low-impact activities like swimming, which support the joints and allow movement. Let your child participate in as many sports and age-appropriate activities as they can to explore different skills without overexertion. Prioritize fun and safety over strict adherence to practice schedules, and make sure that they can rest as needed.
[28:48] It's extremely important to support their emotional well-being. Maintain open, honest communication — listen to your child's experiences and validate their feelings about pain, fatigue, or other symptoms. At the same time, encourage independence and problem-solving, but also be ready to step in and provide help as needed.
[29:08] You want to balance conversations about EDS with time spent focusing on your child's interests, personality, and life outside their diagnosis. It's extremely important that your child have as diverse an identity as possible. If they're good at painting, if they like to do math — whatever other things they like to do — you want to make sure that they are incorporating as much fun and normalcy as possible.
[29:33] Treat your child as normally as possible, encouraging participation in family and social activities with appropriate adaptations. Allow your child to express their feelings about EDS and involve them in decisions about their care and activities. Encourage your child to communicate directly with their healthcare professionals.
[29:51] I really find it interesting when the mom is answering the questions over and over again and the child is not really talking very much. As they're getting older, I think it's really, really important that we let the children speak as much as possible and advocate for themselves.
[30:05] We also want to connect with support and resources. Seek out support groups for parents and children with EDS for community advice and advocacy skills. It's really important when you're looking at support groups to find ones that are solution-focused and not the misery Olympics. You can also find some really great resources from organizations like the Ehlers-Danlos Society and Ehlers-Danlos Support UK.
[30:33] You also want to monitor and adjust with your child. Keep a journal of your child's sensory and physical needs to help adapt strategies as they grow and as their symptoms change. Regularly reassess home adaptations and activity choices as your child's abilities and needs evolve.
[30:50] Supporting a child with EDS involves a combination of practical home adaptations, emotional support, fostering independence, and connecting with community resources to help your child thrive. Help your child be as independent as possible while making sure that you are being totally supportive.
[31:06] Next, I want to talk about low-dose naltrexone. I get lots and lots of questions about low-dose naltrexone.
[31:13] So first of all, what is naltrexone? Naltrexone is an opioid antagonist — an opioid blocker. It was originally developed to treat opioid use disorder and alcohol use disorder. Low-dose naltrexone refers to daily doses of naltrexone that are approximately 1/10 the standard dose used for opioid addiction. This is usually around 1.5 to 6 milligrams per day, compared to 50 to 100 milligrams used for addiction treatment.
[34:25] Low-dose naltrexone is used off-label to manage a variety of conditions, including chronic pain syndromes like fibromyalgia and complex regional pain syndrome; autoimmune diseases like multiple sclerosis, Crohn's disease, rheumatoid arthritis, and lupus; chronic fatigue syndrome; dysautonomia or postural orthostatic tachycardia syndrome; some cancers; mast cell activation syndrome; and pain associated with the Ehlers-Danlos syndromes.
[34:25] We don't entirely understand the mechanism of action of low-dose naltrexone. It is thought that part of it is briefly blocking the opioid receptor, which triggers the body to increase production of our own endogenous opioids — the opioids that we make inside of our own body. By making more of our own opioids, we get more pain relief and more of a sense of well-being.
[34:25] We also know that at low dose, instead of binding to the mu opioid receptor, naltrexone favors toll-like receptor 4, which can lead to decreased production of pro-inflammatory cytokines. Through this mechanism, it modulates the immune system and reduces inflammation — possibly through effects on microglial cells and toll-like receptor 4. We know that at higher doses, naltrexone binds to the mu receptor, but at lower doses, it favors toll-like receptor 4.
[34:25] Clinical evidence is limited but very promising. There have been small studies that have shown benefit in reducing pain and improving symptoms in fibromyalgia, Crohn's disease, and multiple sclerosis. Other research consists of small trials and case series. We definitely need large-scale definitive studies. Low-dose naltrexone at this point remains off-label.
[34:25] One of the advantages of low-dose naltrexone is that it is generally very well tolerated. Side effects are uncommon and usually mild, including insomnia, vivid dreams, nausea, diarrhea, and headache. Weight loss can also occur with naltrexone, so that's something to be mindful of. Naltrexone is actually combined with a drug called bupropion — or Wellbutrin — in a weight loss drug called Contrave. In that medication, it's a fixed ratio of bupropion 90 milligrams and naltrexone 8 milligrams. So for people who are prescribed Contrave, this is a higher dose than most people take if they're taking low-dose naltrexone from a compounding pharmacy. I just like to point this out to people: this is used in a weight loss drug, so we want to be mindful of that.
[34:25] Starting at a low dose and gradually increasing can help minimize side effects. Low-dose naltrexone is considered safe, non-toxic, and inexpensive with minimal risk of adverse effects.
[34:34] You do have to get low-dose naltrexone from a compounding pharmacy. What that means is that the compounding pharmacy gets the powder and then makes their own tablets or capsules. The advantage of that is you can let the compounding pharmacy know if you have any reactions to excipients — the "inactive ingredients" in medications. If you have a problem with a particular type of capsule, whether it's a gelatin capsule or microcellulose or something like that, or if you have a reaction to certain fillers, the compounding pharmacy can adjust that for you.
[35:08] Cost is also a factor, and most insurance policies do not cover compounded medications. Some compounding pharmacies don't deal with insurance at all. So if it's possible that your insurance plan might cover compounded medications, check with your insurance before getting your prescription filled, because you may need to change to a different pharmacy in order to get that insurance accepted.
[35:31] I have also had some patients who, for cost reasons, want to take a quarter of a 50-milligram naltrexone tablet. If you do that, you're getting 12.5 milligrams of naltrexone, which is quite a bit higher than we normally use for low-dose naltrexone. But this is something I've had some patients request in order to save money.
[35:52] What are some of the cautions we have to exercise with low-dose naltrexone? Low-dose naltrexone should not be used concurrently with opioid medications because it can block their effects and reduce pain relief from those drugs. We don't know the long-term safety of low-dose naltrexone. However, naltrexone has a long history of safety, and at these low doses we should not really expect to see very many problems.
Low-dose naltrexone is not FDA approved for these off-label uses, and patients should discuss the risks and benefits with their healthcare provider before starting therapy. I discussed what off-label means and some more information about compounding pharmacies in more detail in episodes 124 and 138.
Another con is that you must take it every day. This is not a medication that you take and immediately notice an effect. I have had some patients that I've prescribed low-dose naltrexone to who felt better within several days, but sometimes it takes months. I do ask patients to give it at least a 6-month trial, and in most cases people do notice at least some benefit.
[36:55] If you are having surgery, you do need to stop the low-dose naltrexone for at least 7 days before your scheduled surgery and resume taking it 7 days after you are no longer taking opioid pain medication. You do not need to taper off the naltrexone. When you go to restart naltrexone — at least 7 days after you stop taking opioids — you may want to restart the same way you started in the first place: beginning with a low dose and then titrating up as tolerated.
[37:20] Examples of opioid pain medications include Vicodin, hydrocodone, Ultram or Tramadol, morphine, Percocet, oxycodone, Duragesic patch or fentanyl, and codeine.
[37:31] What about with acute pain? I know some people are very worried about what happens if they're taking low-dose naltrexone — which is an opioid antagonist — and they have acute pain. Opioid pain medication can be used sparingly if needed for severe pain flares. If a pain flare occurs and someone takes an opioid pain medication, I usually recommend that they do not take their low-dose naltrexone until that flare has subsided.
[37:54] Some people report taking an isolated single dose of opioid pain medication on the same day as low-dose naltrexone without any problems. However, low-dose naltrexone may cause withdrawal symptoms if taken when opioid pain medication is still present in the body. To minimize the risk of withdrawal symptoms, I usually recommend that my patients do not take naltrexone until the pain flare has subsided and they are no longer taking opioid pain medications.
[38:18] For acute pain while taking low-dose naltrexone, it is important to prioritize non-opioid analgesics. Off-label LDN regimens usually do not require increased opioid dosages and mostly depend on patient-specific tolerance. Close monitoring and follow-up are essential.
[38:35] I will provide some links in the show notes where you can get more information about low-dose naltrexone and also about low-dose naltrexone and taking opioids.
[38:42] It is highly recommended that people wear a medical information bracelet and complete any medical information that can be accessed from their phone in an emergency setting if they are taking low-dose naltrexone. This way, the medical team knows that you are on this medication and can act accordingly.
[38:59] It is also very important to include on your medical information bracelet or in your phone app that you have a diagnosis of EDS or HSD. This way, people know to take some precautions when it comes to managing your airway in an emergency — and especially if you have something like upper cervical instability. I would definitely make sure to list that separately.
[39:18] Our last question is from Jamie. "My husband and I are looking into moving to Colorado, but every time we go, I get horrible altitude sickness. I've tried 'the medication' and it doesn't help, although steroids seem to help some, and I had a very rough time. I hoped you might have some tips for high altitude."
[39:34] So EDS, HSD, POTS, and MCAS all can increase sensitivity to altitude, and some people are just more sensitive than others. I am much more sensitive than my husband, even though I tend to hydrate more than he does. Sometimes I will take acetazolamide or Diamox — which I'm sure is the medication Jamie was referring to — and I also sometimes take dexamethasone, which is the steroid most commonly used for high altitude sickness.
[39:58] But what are some practical tips for managing high altitude travel?
[40:01] First of all, what can you do in preparation? You can ascend gradually in order to allow your body to acclimate. You can consider spending time at an intermediate altitude before ascending higher, or take day trips to higher elevation and return to lower altitudes to sleep. It's very important to consult with a doctor who is familiar with high altitude before you travel, especially if you have preexisting medical conditions.
[40:26] During the trip, it's very important to stay hydrated. You want to make sure that you include electrolytes in your fluids. You also want to eat smaller, more frequent meals and avoid excessive carbohydrates, which can worsen symptoms of altitude sickness. You want to avoid alcohol and heavy exercise for at least the first 48 hours at high altitude. Watch for signs of acute mountain sickness such as headache, nausea, tiredness, and loss of appetite. Be aware of severe conditions like high altitude cerebral edema (HACE) or high altitude pulmonary edema (HAPE), which require immediate descent and possibly oxygen therapy.
[41:03] The most common medication options are acetazolamide or Diamox, which is used mostly to prevent altitude sickness, and dexamethasone, which is a steroid that may be prescribed to prevent altitude sickness but can also be used as a treatment. I've taken both of these.
[41:20] There's another option: nonsteroidal anti-inflammatory medications like ibuprofen. Ibuprofen has been shown in some studies to reduce the incidence of altitude sickness by 26%. It works by reducing inflammation and swelling in the brain, which can contribute to altitude sickness symptoms like headache and nausea. It is particularly useful for preventing high-altitude headache, which is a common symptom of altitude sickness.
[41:47] Some of the advantages of ibuprofen: it's readily accessible, widely available over the counter, inexpensive, and usually well tolerated. In terms of the safety profile, ibuprofen has fewer side effects compared to dexamethasone — which is linked to high blood sugar, adrenal suppression, mood changes, and insomnia — or acetazolamide, which can cause nausea, dizziness, fatigue, and frequent urination.
[42:11] Some of the limitations of ibuprofen are, number one, it is inferior to acetazolamide, and number two, symptom masking: ibuprofen may primarily reduce headache and inflammation rather than addressing the underlying acclimatization issues, raising concerns about whether it truly prevents altitude sickness or simply mitigates symptoms.
[42:31] Typical dosing involves 600 milligrams three times a day, always with food, starting a few days or hours before ascent and continuing during the climb. High doses may provide better prevention but could increase the risk of gastrointestinal or kidney issues. It's very important to always take ibuprofen with food and make sure that you are very well hydrated.
[42:49] Ibuprofen might be a good option for individuals who prefer over-the-counter solutions or experience mild symptoms like headache at high altitudes. For rapid ascent or individuals at higher risk of severe altitude sickness, acetazolamide or dexamethasone may be more appropriate.
[43:08] So to conclude: ibuprofen is a viable option for mild altitude sickness prevention and treatment, but should not replace acetazolamide for those requiring more robust protection against rapid ascents. Make sure that you consult with your doctor for any medication regarding altitude sickness.
[43:26] I also wanted to share this information because my clinic in Colorado is at altitude, so I have had a number of patients who, when they come to visit me for appointments, are experiencing a worsening of their symptoms — number one related to the travel, and number two if they're used to living at sea level and they're now at approximately 5,500 feet.
[43:45] Also consider using a pulse oximeter when you travel to monitor oxygen levels if you're at risk for hypoxia due to your medical conditions.
[43:54] Other special considerations for people with EDS or POTS: high altitude can exacerbate the symptoms of dysautonomia or POTS. People can experience worsening of their dizziness, fatigue, presyncope, orthostatic intolerance, etc. It's very important to maintain your hydration as much as possible and your electrolyte intake. Heat and altitude changes can also trigger flares in people with hypermobile EDS and HSD, so it's important to pace yourself and avoid overexertion, especially in the early phases of going to higher altitude.
[44:28] Also, make sure that you are prepared for emergencies by knowing the location of nearby medical facilities. Carry your necessary medications and a detailed medical history if you have chronic illnesses like EDS or other conditions that could complicate altitude preparation. If symptoms worsen despite these precautions, descend immediately and seek medical care.
[44:47] High altitude travel can be safe for people with EDS, HSD, POTS, or MCAS, but it's important to be well prepared and know the potential risks.
[44:56] And last but not least, I have a quick hypermobility hack for you. You may or may not be aware that there is a project by the EDS Society called the Road to 2026. With the Road to 2026, they are looking at revising the 2017 hypermobile EDS diagnostic criteria, which, as you may know, is currently a clinical diagnosis.
[45:22] When the 2017 diagnostic criteria were introduced, of course, there were a lot of questions and a lot of people concerned that this might impact their diagnosis. Well, we have the same issue coming up with the Road to 2026. It is very possible that a lot of us — myself included — may have a change in diagnosis after the Road to 2026 criteria are released.
[45:44] So I would really urge you to be thinking about yourself and your diagnoses in terms of your symptoms. It is very important for all of us not to over-identify with our diagnosis and think of ourselves primarily by our diagnosis. If we do, then we are even more at risk of being really, really devastated if the diagnostic criteria change and our diagnosis changes.
[46:09] A diagnosis does not change what you feel in your body. Your symptoms are valid. Your symptoms are real. Diagnostic categories and diagnostic criteria are things that are developed by humans, and these are always changing over time. As we develop new information and new studies in medicine, all we really are doing is pattern recognition. But again, your symptoms are real and your symptoms are valid.
[46:36] If you focus on your symptoms and your quality of life and other aspects of yourself — if you focus on your strengths and what you have to give to the world and what you're good at — then if the diagnosis changes, it's less likely to be really devastating to you. We'll talk about this more in an upcoming episode because I know that a lot of people are really concerned about this.
[46:59] So I hope you found today's episode helpful. Thank you so much for listening to this week's episode of the Bendy Bodies with the Hypermobility MD podcast. You can help us spread the word about joint hypermobility and related disorders by leaving a review and sharing the podcast. This really helps raise awareness about these complex conditions.
[47:16] If you would like to dig deeper, you can meet with me one-on-one. Check out the available options on the services page of my website at hypermobilitymd.com. You can also find me, Dr. Linda Bluestein, on Instagram, Facebook, TikTok, Twitter, or LinkedIn at hypermobilitymd. You can find Human Content, my producing team, at humancontentpods on TikTok and Instagram.
[47:31] You can find full video episodes up every week on YouTube at Bendy Bodies Podcast. To learn about the Bendy Bodies Program disclaimer and ethics policy, submission verification and licensing terms, and HIPAA release terms, or to reach out with any questions, please visit bendybodiespodcast.com. Bendy Bodies Podcast is a Human Content production. Thank you for being a part of our community, and we'll catch you next time on the Bendy Bodies Podcast.