Can you touch your tongue to the tip of your nose? Bend your thumb to your wrist?
If you are (or used to be) “double-jointed,” get compliments on your soft skin, and experience joint pains, there’s a chance you could have a rare disease and not know it.
Fans of RuPaul’s Drag Race know that Season 11 winner Yvie Oddly wowed viewers with her fierce looks and jaw-dropping contortions. For many people, this was their first exposure to Ehlers-Danlos Syndrome (EDS).
The disease is rare, so we don’t know a lot about how it works. But there are some interesting ties to perimenopause and beyond.
EDS is a group of rare disorders affecting the skin, blood vessels, bones, tendons, ligaments, and other connective tissues; faulty collagen, the protein that adds elasticity to this tissue, is to blame. One or two people out of every 10,000 have EDS, though some doctors think this number is actually higher.
There are 13 types of EDS but the two most common (if you can call a rare disease “common”) are the classical type and hypermobility type (hEDS).
Soft, velvety, and extremely stretchy skin that bruises and scars easily is the hallmark of classical EDS.
hEDS symptoms include joint hypermobility, dysautonomia, and musculoskeletal pain, though there’s some overlap: velvety-soft skin, easy bruising, and the ability to extend joints beyond the normal range of motion are present in most types.
Basically, wherever there is connective tissue, you’re more likely to have issues.
Hypermobility leads to hyperextension, dislocation, subluxations (partial dislocation), and overall pain. For many sufferers, this pain can be debilitating, requiring braces, crutches, or even a wheelchair, all while looking “normal.”
EDS, and especially hEDS, often go undiagnosed; most doctors receive very little training on the disorder, even those who specialize in joint and skin conditions.
Need a trained doctor's opinion about hEDS? A Gennev menopause-certified gynecologist can give you a trusted opinion, determine if medication is right for you, and they can provide prescription support. Book an appointment with a doctor here.
There’s no genetic test for hEDS, so diagnosis is dependent on clinical criteria, including the Beighton Scoring System (try it yourself!) to assess joint flexibility and self-reported pain.
The lack of knowledge and diagnostic tests can lead to years of frustration for sufferers, as doctors ignore their issues, tell them it’s all in their heads, or recognize that there’s something wrong but just don’t know enough about EDS to be effective.
Women experience hEDS at a much higher rate than men, and the current theory is that estrogen plays a role.
Estrogen overall has an effect on connective tissue. Women, in general, have looser ligaments than men and people with higher testosterone, which leaves us more prone to joint injury.
Estradiol receptors, proteins in cells activated by estrogen, are present in skin, cartilage, and other connective tissues. Estrogens help regulate the metabolism of these cells.
Research done on a normal population shows that tendon injury is more common in pre-menopausal women, with the highest risk being when estrogen in the menstrual cycle is at its lowest after ovulation.
This hormonal link to loose joints shouldn’t come as a surprise: ligaments relax in pregnancy to allow the birth canal to expand to fit a baby.
Puberty is often the onset of hEDS symptoms, and those who previously had symptoms notice that they worsen after their periods start.
Women with hEDS often have gynecologic difficulties like heavy periods, heavy cramps, and pain with sex, and report more hEDS symptoms with their menstrual cycle. Combined hormonal contraceptives (CHC) and Progestin-only contraceptives (POP) can help symptoms and reduce fatigue in some patients with hEDS.
Well… there isn’t a lot of solid scientific information out there about how menopause specifically impacts people with hEDS. Anecdotally in Facebook groups and online forums, many women with EDS report early menopause, but research does not support this.
However, because of the hormonal involvement, menopause certainly has an impact on hEDS. For starters, connective tissue in the general population changes post-menopause and skin collagen decreases 1-2% each year after menopause.
Joints stiffen with age for all of us, which can be a good thing for people with hEDS, as less supple joints are less prone to overextension. In a study of 386 women with hEDS, 22% of the post-menopausal women reported that their symptoms decrease after menopause.
However, more than one-third of participants experienced a worsening of symptoms before their periods while in perimenopause. The most rapid shifts in hormone levels happen during this transition, which is why it’s not surprising that the time just before menopause can be when hEDS is first diagnosed.
As we previously mentioned, women with hEDS have more gynecologic concerns than the rest of the population. They’re more likely to experience incontinence, vaginal dryness, and how to do sex without pain, concerns that increase for all people with uteruses in menopause.
Some studies have found that people with EDS tend to have lower bone density than the regular population, pre-menopause, which makes them more prone to osteoporosis later in life. While this may be because people with EDS limit bone-strengthening exercise due to pain and mobility issues instead of a structural cause, the implications for bone health are the same.
It’s been thought that hormone replacement therapy (HRT), selective estrogen receptor modulators (SERM), and oral contraceptives could improve diseases of connective tissues. Unfortunately, the amount of estrogen in HRT is not enough to protect joints, and higher doses may increase the risk of breast and other reproductive cancers.
If you’re a woman in midlife who thinks that you might have the hypermobile form of EDS, talk to your doctor or one of ours; because the disorder is so rare, you may need to be persistent and detailed about your symptoms to get the help you need.
More research clearly needs to be done on how to help women with EDS in menopause, but awareness of the condition — and better reporting, so we have a better idea of actual numbers — can help bring greater attention to the issue.
Sign up and chime in about your path in menopause on the Gennev Community Forum. Come join the conversation.