Living with HSD
The Living Experience of HSD/hEDS
Latest post: Fool's Epiphany (1 April 2025)
Perhaps only a fool would find inspiration in April Fool's Day, but right around this time of year I start seeing how things fit together. This year's epiphany: how HSD shaped the latter year's of MP's life. Now that I am more clearly following in her footsteps, I finally understand how the past 30 years went down. Earlier in life, we got used to being able to achieve just about any goal we had, because both brain and body were functional. Sometimes even better than that. But by our 50s, everything we touched started to (surprise!) turn into crap: deprived of sleep, our nervous system shot and coordination slipping, our memory and logic so unreliable that embarrassing errors begin to multiply--until one day we realize that our life is spinning completely out of control. One response to this is to claw back control until the panicking stops. Do that long enough and hard enough, and you have OCD. That's how MP spent the past 30 years--and I get it now. Because the only way that we can function in the world is if we can get the world to consistently accommodate our unusual and ever-declining cognitive pathways. So much for the epiphany: the question remains, will I foolishly follow the same path??Past posts
Bittersweet (1 March 2025)
Ironically, the good days are the most bitter. On the bad days, it's a miracle I can get anything done at all, so there's a great sense of accomplishment for mastering new tasks like ordering ISBNs, making bar codes, writing anything vaguely comprehensible, formatting it in EPUB-html, creating an Amazon KDP account, making a QR code, or developing an informational website. But on the good days, my natural arrogance bursts forth and I can't help but compare myself to who I used to be, or to other people...and then my 'accomplishments' look pretty pedantic. (Humility is much easier on days when I have no choice but to be humble.) It's even a little anti-climactic. I mean, I spent years figuring this all out, and the culmination is such a simple and inexpensive product! But, of course it is just the start--meant to provide what I wish I'd been able to find four years ago when I started looking...without knowing what I was looking for. So, I guess I'm still a little proud that I got it done: my first HSD book, on how you can tell if you might have it, is actually now available for purchase as an ebook on Amazon! And that is a little sweet.Diagnostic Code (15 May 2023)
HSD is in the code. Sure, it's heritable, so the key to the disorder lies within our genetic code. We have the code, so now all we need to do is treat this chronic, progressive, degenerative collagen inadequacy disorder. Except you can't get treatment for it until you have another code--a far more important code in our modern world:the diagnostic code
I was pretty sure I had hEDS (hypermobile Ehlers-Danlos Syndrome) for about 1.5 years before I got a diagnosis, but the wheels of our medical system turn slowly (when they turn at all). The day after I self-diagnosed, I naturally wanted to start being treated, but my doctors kept blocking my efforts. Of course they were willing to refer me to other (clueless) specialists, prescribe pain meds (without understanding the cause), and order fancy tests that would tell me what I already knew (the bottom line of medical clinics). But no matter how hard I nudged, I could not get them to think outside their comfort box, as if they were totally brain-dead... or perhaps afraid of being sued. Actually, the system has so absorbed medical liability lawsuits that I doubt anyone actually ever worries about being sued anymore. This is because they've designed a system to minimize the risk, which means they won't treat you until you have a formal diagnosis with a diagnostic code. In fact, the only reason they can treat you when you are on the verge of death is because things like cardiac arrest (diagnostic code ICD-10 I46) are easy to recognize, so they can quickly record the diagnostic code--and then bother to resuscitate you. The fact that every moment of delayed treatment prior to that often translates into irreversible damage that they could have prevented is not relevant, because it is hard to prove in court. Anyway, for a long time it wasn't clear I would ever get that code. First, there is no definitive diagnostic test: i.e., no genetic marker or blood test that any old doctor can use to verify that you have it. Second, the odds are that any old doctor (like my former primary physician) has never even heard of it, so you have to find a specialist. Third, there are virtually no such specialists (3 hEDS specialists on the entire East Coast). However, I did eventually find a local rheumatologist who was willing to make a diagnosis (but refused to treat me), largely because I did all the footwork for him.So, what did the footwork show?
- I am otherwise healthy. Over the past four years (since I vowed to figure this out), I have been tested for literally ever other disease that could explain my symptoms, and all the tests came back within normal specs (e.g., no autoimmune disorders, Lyme disease, diabetes, cancer, etc.). This is no guarantee, as some conditions express at subclinical levels, but at the moment there is no evidence at all for any other explanation. [As Sherlock Holmes put it, "When you have eliminated all which is impossible, then whatever remains, however improbable, must be the truth."]
- I now qualify. According to the 2017 diagnostic criteria for hEDS put out by the Ehlers-Danlos Society, I finally meet the criteria. In the absence of truly definitive tests, to maintain credibility they designed the criteria to be pretty narrow-- so pain in your limbs counts, but neck pain doesn't (even though it is one of the most common problems in hEDS). So for many years I would not have qualified, but I have now accumulated enough symptoms (great!) to push me over the top. [Rather absurd for an inborn condition!]
- I am female. Because one of the purposes of progesterone is to loosen collagen (to stretch open the birth canal), 70% of symptomatic hEDS patients are women. Puberty often splits off the genders, with symptoms typically declining for boys (except for tachycardia) while worsening for girls (e.g., PMS, intolerance to progesterone IUDs).
- I have HSD. In addition to the obvious hypermobility-induced neck pain, my symptoms span a spectrum (Hypermobility Spectrum Disorder) of organ systems, so I can't blame it all on my bobble-head. Further, those organ systems just happen to be the same ones (and only those) that rely upon collagen for proper functioning: joints, blood vessels, the diaphragm and pelvic floor, and things made of epithelial cells (skin, gut lining, lungs).
- I'm a freak. Many of my hitherto inexplicable bodily oddities can be completely explained by a collagen disorder (such as being a total klutz--collagen-based stretch receptors being critical to proprioception). Further, although not diagnostic for hEDS, I have >50% of the long list of co-morbidities reported by hEDS patients.
- I was always a freak. Although many of my symptoms could be attributed to mechanical compression of the brain stem and vagus nerve from a wobbly neck, the first obvious symptoms of cervical instability did not arise until I was 40...yet I had several hEDS-associated conditions even in childhood (e.g., unstable ankles, dental migration, arthritic finger pain). Furthermore, something had to cause the cervical instability in the first place...
- You [relatives] are freaks too. Members of my lineage have an improbably high rate of hEDS co-morbidities, including otherwise relatively rare conditions such as pernicious anemia, failed dental implants, supraventricular tachycardia, nonepileptic seizures, and delayed speech development. Highly suggestive of a genetic disorder.