Ehlers-Danlos Syndrome Steps into the Spotlight

I recently watched this series Run Away on Netflix. I usually multitask a bit while a series plays in the background, so I miss little bits here and there while I am still “listening” to it, but not watching it. But at one point, I did think, “Hey, I didn’t notice before that the daughter was in a wheelchair, oh well, whatever.”

But then this article about it came up on my Facebook feed:

“It looked like a mistake at first. A detail that slipped through editing. But the truth behind it is far more human than viewers expected….

…Anya is played by Ellie Henry, who lives with Ehlers-Danlos Syndrome. It is a genetic condition that affects collagen, making joints unstable and movement exhausting. Some days, a wheelchair is necessary. Some days, it is not.

That reality was carried directly into the character….

…By not turning Anya’s condition into a storyline, Harlan Coben’s adaptation shows something simple and honest. Disability is not always visible. It is not always permanent. And it does not need a spotlight to exist.”

Frankly, 6 months ago I had never heard of Ehlers-Danlos Syndrome. That was until I was told I had it too, (hypermobility-type), and it explained why my heart mitral valve has torn, why I am struggling with small vessel constriction and circulation in my hands, why I have significant osteoporosis at only 54-years old, why I have sluggish digestion, why I now have bilateral inguinal hernias while simply working at a desk job, why my knee caps slip all over the place, why my shoulder dislocated so easily, (and was then also easily reduced, multiple times in one day, but not without tearing things and taking months to heal), etc.

I never realized with all the walking I do, it involves “muscle guarding” of my joints, especially my knees, which adds to my overall daily fatigue. But I need those lean legs muscles to keep going, so I don’t lose mobility! I notice almost right away, if I stop regular walking, how fast I de-condition. Faster than average, for sure. And my heart mitral valve leaflets did not tear because of some strain I put on my heart. Instead, my heart has strain on it because the valve leaflets are torn. So I need the cardiovascular exercise to keep my heart muscle strong, so it can handle the strain of the back flow of blood (regurgitation) caused by the torn valve leaflets.

Ehlers-Danlos Syndrome is a group of disorders, and has a spectrum, just like so many other things. Previously, I have not been high enough on the spectrum of resulting issues to have ever needed a wheelchair, for example, which is one of the reasons I went undiagnosed for so much of my life. My mother remembers taking me to the doctor at about 18 months old because I “walked funny,” my gait was very strange. She said it was almost as though I was swinging my hips to push my legs forward. In hindsight, it seems so obvious…but I totally understand how hard it would have been to realistically diagnose a loose joint/connective tissue issue at that age. Needless to say, after constant verbal correction, I eventually figured out how to hold my legs in a more steady way, and to walk straight. I also remember constant verbal correction to “stand up straight,” stop walking “knock-kneed,” don’t sit like that, etc. (Of course, extra flexibility alone, which kids naturally have their fair of, does NOT mean you have hEDS, but when you start adding up all the other issues… “If you are having trouble connecting the issues, think connective tissues”).

Anyhow, I just think that it is wild to hear that an actress hired to play a roll on a Netflix series has this same condition. And when cast for the job, Netflix just filmed her either standing and walking, or in a wheelchair, simply depending on her physical needs for the day. I think that is wonderful.

Workout Express Circa 2004

I found this picture from December 2004, from when I was going to Workout Express three to four times per week (after working 9 to 5 at an office). I am even wearing their t-shirt in the picture, and my cheeks are flushed from having just done a workout, and then driven home.

I was dedicated. Until I wasn’t.

It is truly exceptional to not only get fit, but to also stay fit. I tried to get fit and stay fit many times since my year at Workout Express. Did I even make it a year? Yes, I think I made it a year and a half. I quit when I changed jobs in April of 2006, because by then I was not going as much as I used to anyway. When I changed jobs in 2006, I was actually working on “building a career or business.” Both of those things, in fact. Helping my husband build his business, and going back to school part time to build myself a new career too. We were not raising kids full-time, but we were dealing with our fair share of “handling chaos.”

By the time I started this blog in 2019, I was trying to get fit, for the umpteenth time, and it took me a while to get to where I am today. Now I am trying to stay fit while “aging.”

Aging has added a layer of difficulty to staying fit, just like building a career and business, and handling chaos did. Maybe even more of a layer. And, of course, staying fit while handling chaos still exists. Unexpectedly this week, a huge sheet of ice slid off a cube van that we passed on the highway going 60 miles an hour, and it basically wiped out the whole front end of our truck! We were lucky we were not hurt, because it hit our front end, breaking everything through to and including part of the radiator. But luckily we did not get pushed off the road, and it did not hit or go through the windshield. Even though we have insurance, there is plenty of chaos filing the claim, extra traveling for repair estimates, then waiting for all repairs to be done, all the while being without the use of the vehicle until it gets completed, etc. That’s just life.

I have to remember aging is just life too. I need to adapt to the different signals from my body, and it’s fluctuating and/or changing capabilities. 20 years ago, because I was changing jobs, I basically abandoned Workout Express without a clear plan of what I was going to do instead. I have now learned that staying fit means staying with fitness routines, and adapting and changing them as necessary, but never abandoning them.

Osteoporosis and hEDS: A Public Service Announcement

So, I mentioned in yesterday’s post that I would write about my new plan of action. It’s fairly specific to me, because at 54-years of age, my lumbar spine has been diagnosed with a t-score of -3.3, which is already half way through the osteoporosis scale heading for really severe (-4.0), whereas my hips are only osteopenic, (-2.1), but inching fairly close to osteoporosis themselves. This difference is common in connective-tissue–related bone fragility. So, maybe none of this would really apply to a fifty-something-year old woman who did NOT have hEDS. And even then, not everyone with hEDS will get dealt this osteoporosis card, (or the heart mitral valve card either, for that matter).

I used Chat GTP as a sounding board for the things I am about to talk about in this blog. To be clear, Chat GTP did not diagnose me. I was diagnosed by a doctor, by an echocardiogram done at a hospital, by a bone density scan done at a radiology clinic, and even by a Dexa scan, which even though is not considered medically diagnostic according to my doctor, did show questionable findings that got the ball rolling for me. (Best $249 I ever spent on that Dexa! The rest is covered by my medical insurance).

Anyhow, Chat GTP has real information at its disposal on my real diagnosed medical conditions, so it was a great place to have an evidence-based and knowledgeable chit chat. This is not medical advice, it’s just my experience and decisions based on information I have gathered.

When it comes to the different t-scores between my spine and my in my hips, the bone densitometry I had done on October 3, 2025, also looked at the neck of the hip versus the total hip, where again there was a slight difference for me:

“In your case:

• The total hip is lower than the neck

• This suggests relatively greater compromise of cortical bone than trabecular bone at the hip

This pattern is actually very consistent with connective-tissue–related bone fragility, where:

• Collagen defects affect bone matrix quality

• Cortical bone can become thinner and less resilient

• BMD may underestimate true fracture risk.”

Hmmm. Good to know.

Here’s the interesting part, if my osteoporosis was just because I started menopause about 2.5 years ago, then firstly, my t-score should not be this bad already! It’s only been 2.5 years! So we can feel confident my osteoporosis is connective-tissue related. And:

“Contrast this with:

• Postmenopausal estrogen deficiency, which often hits trabecular bone first (neck worse than total).”

Okay. It is my total hip that is worse, not the neck, (and it’s my spine that is really bad for my age). None of this would make any sense, really… except in terms of my having hEDS.

Because I have hEDS, I am very hesitant to take any bisphosphonates (like Fosomax) because of my digestion issues related to having hEDS. “GI intolerance risk is meaningfully higher in your case.”

And besides, for me specifically:

“Potential limitations of Fosamax for you

• It does not address collagen or bone quality

• It can over-suppress remodeling, which:

• May be problematic when baseline bone quality is abnormal

• Is a concern raised specifically in some hEDS populations

• Spine BMD gains tend to plateau quickly

• Fracture risk reduction in younger postmenopausal women with non-classic osteoporosis is less robust than in older populations.”

Yeah. Many women with these same t-scores that I have, are actually much older than me. I have strong legs and good balance, (and I can work on increasing my core strength also), so I am not really at risk of falling in the same way a woman coming up on 80-years old would be, (although if I did fall, I could break more easily, yes, that is true). Fracture risk is a huge concern for older women, especially in their seventies and eighties. All it takes is that one fall, leading to a fracture, and they can lose their independence. Woman in their seventies and eighties that find themselves with significant osteoporosis need to pull out the big guns to get the best return on their fall prevention investment.

I need to invest in fall prevention too, but, in theory, I should have many decades of life ahead of me, so it would probably not be good to be taking harsh medication for the next 30+ years!?!

Anyhow, even though my bone loss is not directly caused by menopause, that certainly has not helped things.

“But here’s the key distinction in your case

Postmenopausal osteoporosis:

• Primary problem = estrogen deficiency

• Estrogen replacement can be foundational therapy

Connective-tissue–related osteoporosis (suspected hEDS pattern):

• Primary problem = bone matrix / collagen quality

• Estrogen deficiency is secondary or additive

• Estrogen cannot correct defective bone scaffolding

So in your situation:

• Estrogen may help reduce the speed of loss

• It may slightly improve spine BMD

• But it cannot reverse the underlying fragility

This is why your hips are osteopenic, not osteoporotic — estrogen deficiency alone does not explain your pattern.”

So, estrogen may help a bit, so that is what my doctor and I have decided to do, good old Hormone Replacement Therapy (HRT). Sure, there is a slight increased risk of breast cancer, but leaving my osteoporosis unchecked has risks also. Everyone must make their own informed decision in this regard, weighing benefits versus risks. (And for me personally, I am also taking micronized progesterone, because I still have my uterus, so a balance of both hormones on HRT is protective for that).

And for people with osteoporosis primarily because of menopause, if their doctor recommends it, I thought this was interesting:

“For lumbar spine osteoporosis, the timing of estrogen therapy is critical, with the greatest benefits occurring when treatment is initiated in the “window of opportunity” which is in the early postmenopausal years (typically within 10 years of menopause onset or before age 60).

Key Timing Effects

Early Initiation (Critical Window): Starting estrogen soon after menopause is most effective for preventing rapid bone loss. The first 5 to 7 years after menopause are when the most significant bone loss occurs in women due to estrogen deficiency. Initiating therapy during this period helps stabilize bone mineral density (BMD) in the lumbar spine and effectively reduces the risk of future fractures.”

I am only be 2.5 years into menopause. So, I now begin HRT as part of how I am starting to work on this problem for myself, based on my own personal “issues with my tissues,” that have been most probably been the main cause for me of this significant osteoporosis problem in the first place. And it looks like I am still within this good window of opportunity to be starting HRT.

That is it in a nutshell. I hope everyone has a wonderful New Year’s. Even with health concerns, being in the driver’s seat of my decisions, and putting those decisions into action, certainly feels better than instead simply allowing myself to be influenced, without taking the time to gather an adequate amount of empowering information on which I can make my own decisions.

One More Month Left in 2025

Okay, how great is it that the first of December is a Monday? I feel inspired. Even though I have had the occasional thought, “Why are you excited about upcoming medical appointments, they don’t actually change anything about your condition…” my predominant thoughts have been that it will be wonderful to have a more comprehensive baseline through which future tests can be compared, that I wrote about here.

So, whatever your journey is, health, exercise, weight loss, weight maintenance, school, learning, home organizing, heck, a little of all of the above, my goal this month is to ignore negativity (my own self-negativity in my head) by replacing it instead with positive action.

So, in addition to going to some upcoming medical appointments, (1.stress test, 2.get heart holter monitor, 3.take holter monitor back), I am especially focused on the fact that without planning it, I am less than 90kms from 1500kms walked for the whole year. My consistency with my regular walking has resulted in the highest kilometers walked EVER in one year, at least ever since I started tracking on RunKeeper in 2012. (Previously, my best year was 1446 kms walked in 2016, my lowest year was 172.3 in 2018).

Wait. 172.3 kms in total in the year 2018?? Wow, I forgot all about that year. Talk about selective memory, (I obviously blocked out how bad it was). I remember now that in 2018 I had terrible joint pain, most likely due to out of control inflammation. As a Celiac, I was eating a lot of gluten-free grains, like quinoa, brown rice, “gluten-safe” oats. But then…I just got lucky. To the best of my recollection, it went something like this: In December of 2018, I went to the cupboard to get my quinoa or brown rice or oats, to cook up with my blueberries, but then I just stopped. I stood at the cupboard remembering how good I felt in 1999, when I tried the Atkins diet. So, I decided right there and then to go grain free. (Not low carb like Atkins, because I was still going to eat the blueberries, but no more grains).

33 days later, the pain in my joints was gone! I just woke up that morning, and it was gone. I looked back to when I stopped eating grains, and counted that it had been 33 days.

(In hindsight, I think my chronic joint pain and inflammation had been some version of “leaky gut” associated with my hEDS, but I just called it grain—grains of any kind—intolerance, because I did not know until just this year that I have hEDS).

I feel really lucky that I discovered a “cure” that worked specifically for me, (although constipation and upset stomach is still an issue), but no more joint pain!! For me, grain free is pain free.

Here’s to a great December, (and to listening to your instincts about your own body).

The Gravity of the Situation that No Diet Can Fix:

Mitral Valve Replacement—open heart surgery?

Despite an overall positive attitude, every once in a while it hits me: diet and exercise will not fix my heart. Slow progression, perhaps, but not fix it. Every out of the ordinary blip (weird increase without probable cause) in my heart rate makes me wonder, is this dysautonmia from having hEDS, or is this the left ventricle of my heart complaining about the back flow of blood?

Having dieted since a young age, I think I thought everything can be solved (to a certain degree) if you just ate right and exercised. But nope, like my blog post here said, my heart valve disease progression can only be managed, not solved.

It helps me to talk about this, and it fortifies my resolve to “not eat junk foods” and keep walking every day.

Because I do not have any answers yet, I just have to wait and see. Open heart surgery and a “simple” valve replacement may not be my best course, I do not know. (I jokingly say “simple,” because it is never simple, even when a person does NOT have hEDS).

I will not know until some time goes by, and I can have a repeat Echocardiogram, and see if my current level of grade 2 regurgitation is stable for now, or is it progressing. Specifically, a repeat Echocardiogram is needed to track if my left ventricle is further dilating. Currently, despite having moderate (borderline severe) regurgitation, I ONLY have “Mild left ventricular dilation but preserved function.” That is truly good news, and can be attributed to all the walking I do/have done. Next month I am about to hit 12,000 all time walked kms on RunKeeper (since 2012). Wow.

Therefore, I feel that my absolute only course moving forward is to truly never waver in resolve with my overall health and cardio vascular exercise (the best of which for me is walking) to keep my heart muscle from weakening further which eventually leads to congestive heart failure. A strong heart can withstand the black flow caused by regurgitation—maybe—for a very long time before stage 4 and congestive heart failure. Here is some general information that I do know:

Mitral Valve Replacement

Do porcine mitral valve replacements and mechanical value replacements fail sooner in patients with hypermobile Ehlers-Danlos syndrome that caused both sides of the mitral valve to prolapse in the first place?

In patients with hypermobile Ehlers-Danlos syndrome (hEDS), porcine (tissue) mitral valve replacements may fail sooner than in the general population. Mechanical valve replacements carry distinct risks in hEDS due to the connective tissue disorder, but there is less direct data on comparative failure rates. The primary issue is not the implanted valve itself but the ongoing fragility of the patient’s native heart tissue, which can lead to complications with either type of prosthesis.

Hypermobile EDS and tissue valve failure:

The risk of accelerated failure is higher with bioprosthetic valves (made from animal tissue) because the valve is attached to the patient’s existing mitral annulus, which has the same underlying defective connective tissue.

Annular tissue fragility: hEDS patients are known for tissue fragility. If the native mitral valve ring (annulus) is weak, the sutures holding the new bioprosthetic valve in place may tear out over time, causing a paravalvular leak and premature failure.
Recurrence of prolapse: Even with a repair, the remaining valve tissue can continue to deteriorate. Some case studies report the failure of mitral valve repair in EDS patients, requiring a subsequent replacement with a bioprosthetic valve. The continued breakdown of connective tissue in the heart is the root cause of these issues.

Hypermobile EDS and mechanical valve failure:

While mechanical valves are more durable and not subject to the same tissue breakdown, hEDS can increase the risk of other complications.

Tissue fragility during surgery: The main concern is the fragile nature of the heart’s native tissue during and after the initial surgery. The procedure itself can be more complex due to the risk of tearing or bleeding.
Increased bleeding risk: Mechanical valves require lifelong anticoagulation therapy with blood thinners like warfarin to prevent blood clots. hEDS patients often have a greater tendency for easy bruising and bleeding due to their connective tissue disorder, which can increase the risk associated with this therapy.

Repair vs. replacement in hEDS:

Because of the risks associated with implanting a foreign valve into fragile tissue, surgeons often face a difficult decision:

Valve repair is often preferred to replacement, especially in younger patients, to avoid the long-term issues of either a tissue or mechanical valve. However, due to the underlying defect, the repair may fail and require another surgery.
Replacement can be a more logical choice in cases of severe fragility, but as noted, it carries risks regardless of the type of replacement valve.

Why the difference?

The difference in failure risk comes down to how the valve is replaced:

Bioprosthetic valves (porcine): Their failure is linked to the degeneration of the animal tissue over time or issues with the native human tissue they are sewn into. In hEDS, the latter is a significant risk factor.
Mechanical valves: They do not structurally fail due to tissue degradation. Instead, the risk is tied to the lifelong blood thinner medication and surgical complications due to fragile vessels and tissue.

For hEDS patients with mitral valve disease, the decision to repair or replace the valve is highly individualized and requires a multidisciplinary team approach to weigh the risks of each option.