The link between Ehlers-Danlos Syndrome (EDS) and Osteoporosis

Estimated reading time: 7 minutesI met my first osteoporosis client back when I first started teaching. As part of my initial Pilates training, I’d taken additional courses in how to work with osteoporosis so I was able to safely modify exercises for her during a Pilates mat class. Now it is something I regularly receive questions about.

An additional diagnosis of osteoporosis can add another layer of complexity to living with hypermobility. Ehlers-Danlos Syndrome (EDS) already impacts many musculoskeletal issues like tissue fragility, subluxations, muscular pain, and injuries. Osteoporosis involves weakened bones and an increased risk of fractures. This blog will explore the relationship between EDS and osteoporosis and suggest approaches to management.

Certain things should be avoided if there is an osteoporosis diagnosis like loaded flexion and twisting. Now, in The Zebra Club, we have many members with osteoporosis and osteopenia – so I created a bone health class with key education pointers on safe exercise and movement.  It’s a safe, effective way to load the tissues while avoiding contraindications.

Osteoporosis and Bone Health Overview

Osteoporosis is considered a metabolic bone condition resulting in low bone mineral density and reduced strength of the bones leading to an increased risk for fractures (1). Osteopenia is when the bone density is low, but not as low as required for a diagnosis of osteoporosis (2). Bone mineral density can be measured and is used to help assess fracture risk (3).

In the body, there is a normal remodeling process in which old bone is continually being replaced by new bone. This maintains the bones’ healthy structure. It is maintained at a balance between reabsorption of the old bone by cells called osteoclasts and bone formation by cells called osteoblasts (1).

This balance between bone reabsorption and formation can become uncoupled, and the structure of the bone is not as easily maintained – this occurs naturally with aging. Over time, in older people, there is a net loss of bone. There are other conditions that can interfere with this process. Estrogen deficiency is also a huge risk factor for the development of osteoporosis and most bone loss occurs in women after menopause (1).

There are a number of things that can contribute to bone quality later in life, but one major determinant of bone density in an older person is their peak bone mass. When you are younger, the osteoblasts can be more active and increase the bone mass in response to things like activity, and you reach a peak density of the bones or peak bone mass. This is estimated to occur in the 3rd or 4th decade of life and depends on things like genetics, hormones, and environmental factors (1).

According to the NICE guidelines (National Institute of Health and Care Excellence in the UK), it is particularly important to assess for osteoporosis and fracture risk in post-menopausal women and men who are 50 years or older (4). As a result of increased bone fragility, roughly 1 in 2 women and 1 in 5 men will in their lifetime have what is called a fragility fracture – or a “low trauma” fracture from a fall from standing height or lower (4).

Risk factors for Osteoporosis

There are known risk factors that increase the likelihood of Osteoporosis – beyond low bone mineral density (BMD)(4):

• Age
• Low Body Mass Index – Specifically a risk factor for hip fracture
• A history of previous fracture
  • Especially from low trauma and at a site common to osteoporosis like the body of the vertebra, hip, distal radius (the thumb side arm bone, closer to the wrist end), the proximal humerus (the thigh bone, closer to the hip), and the pelvis
• A parent with a history of hip fracture
• Smoking
• Use of oral glucocorticoid therapy – the risk increases with higher doses
• Alcohol intake – risk also increases with higher doses (3 or more units daily are associated with an increased risk)

Other conditions that are associated with an increased risk of osteoporosis or are secondary causes of osteoporosis include Diabetes, inflammatory diseases (like rheumatoid arthritis), celiac disease, chronic liver disease, vitamin D deficiency, Renal disease, cardiovascular disease, and dementia (3).

Low levels of physical activity have also been linked to the development of osteoporosis, particularly the lack of weight-bearing activity (1).

Effects of EDS on Bone Density

How does EDS play into the development of osteoporosis? As we often find, there is not enough research with a significant study size to determine a clear picture of how hEDS/HSD impacts the development of osteoporosis. The small study size limits how generalizable the findings can be. Here are some of the small studies that have been done.

• Risk of low bone mass was increased by 1.8 times in hypermobile pre-menopausal women (5)
  • A small study in 2005 (n=23 hypermobile women with average age of 34) compared bone mass density with non-hypermobile controls that were matched for age, body mass index, number of pregnancies, physical activity, and length of breastfeeding. About 70% of the hypermobile group had low bone mass, while 39.1% in the non-hypermobile group had low bone mass. This is a very small group from the same population.
• EDS patients have decreased bone mineral density, reduced bone quality, and increased vertebral fractures (6).
  • Another SMALL study in 2016 enrolled 50 people between the ages of 30 and 50 years (36 premenopausal women and 14 males) with EITHER classical or hypermobile EDS were compared to 50 age, gender, and body mass index -matched controls. They did not distinguish how many had classical or hEDS. Researchers also point out that they cannot distinguish between decreased activity in the EDS patients in their youth resulting in a reduced peak bone mass.
• Women with hEDS had smaller bones but did not have accelerated bone loss or differences in bone density. They did have an increased risk of fractures related to smaller bones (7)
  • This was another small study that assessed bone size, muscle strength, bone density, and reports of fracture and then followed up 8 years later to assess for differences. There were 27 women with hEDS and 17 non-hypermobile controls. They attribute the increase in fractures in the hypermobile group due to smaller bones which they conclude may be due to decreased mechanical loading (less activity).

“Taken together, our data suggest that the increased fracture risk in hEDS/HSD might be due to a lesser peak bone mass and bone size attainment secondary to muscle disuse and lesser strain on bone during growth and adolescence.  However, the possibility that there might be a genetic contribution to their increased fracture risk cannot be excluded. (7).”

They point out that pain, fatigue and recurrent injuries most likely lead to the decreased activity observed with EDS (7).

While there are a few papers linking EDS and low bone mass, there are others that did not find this connection. A Meta Analysis that assessed the results of 5 papers exploring the connection between EDS and Osteoporosis found there was not an increased risk of osteoporosis in EDS (they did not distinguish the type of EDS), but there was an increased risk of fracture (8).

Ultimately these point to the benefit of movement and the importance of loading the bones, ideally from a young age. It would be beneficial to get kids interested in safe and effective exercise early.

Biological Links Between EDS and Osteoporosis

According to Guarnieri and Castori, this reduction in bone mass seen in some studies could be associated with decreased activity or other common comorbidities like vitamin D deficiency, and lack of proprioception (9).

While there does not appear to be a significantly increased risk for osteoporosis in hEDS and hypermobility, there may be a connection with mast cell disorders. As many of you know Mast Cell Activation Syndrome (MCAS) is well connected with EDS, often referred to as part of the trifecta of POTS, EDS, & MCAS.

There is evidence of osteoporosis in relation to mastocytosis a condition where there are too many mast cells (unlike MCAS – when there are a normal amount of mast cells, but they are overactive). According to Dr. Afrin (10) it “seems to be prevalent” in MCAS, yet there is not much research specifically assessing MCAS and osteoporosis.

Diagnosing Osteoporosis in EDS Patients

According to The National Osteoporosis Foundation, US Preventative Task Force, and American Association of Clinical Endocrinology Bone Mineral Density should be assessed in all women 65 years or older, all men 70 years and older, and younger people who have 1 or more risk factors including a history of fracture. They particularly mention post-menopausal women and men aged 50 and above (some of these are listed above) (1, 4).

A medical provider can obtain a detailed history, assess the risk of fracture, and determine what testing is needed, including assessing bone mineral density using a DXA scan. This is the gold standard for assessing bone density (1,4). If osteopenia or osteoporosis is diagnosed, ther are medications that can help prevent fractures (1,4).

Prevention of Osteoporosis in EDS

The research has suggested that EDS does not increase the risk of osteoporosis, though there may be an increased risk of fracture. Following general osteoporosis guidelines will be helpful. Keep in mind physical activity is protective for retaining bone mass density (11).

There is no guarantee of prevention, but there are certain activities that can help.

• Safe, bone-strengthening exercises will be key. For example, low-impact weight-bearing exercises like walking can help without overloading the joints.
• We do need to load the joints, however – we need to do this safely if we also have EDS. High impact activities like running and jumping may not be suitable.  Resistance work with weights and resistance bands can help stimulate bone growth.
• Balance and proprioception exercises – keep the muscle tone and stability as much as you can to prevent falls and injuries. A critical component of osteoporosis management is preventing falls. In EDS we know the fear of falling is common (12). Building hip strength can be really key here.
• Working with an EDS aware physical or movement therapist to implement safe movement and activity programmes early on would be extremely helpful. Certain movements that are common in Yoga, Pilates, and Aerobics for example will need to be modified. Loaded forward flexion are contraindicated like roll-downs, forward bends, sit-ups, curl-ups, and deep twists. It doesn’t rule out these activities, but please ensure your therapist knows you have osteoporosis and that they can adapt your exercises accordingly.

Managing Osteoporosis in EDS

Recommendations for those with higher risk or have osteoarthritis (1,4)

• Maintain a balanced and nutrient-rich diet
  • This is a great time to work with a specialist in EDS and nutrition as we know gastrointestinal complications are common
• Speak with your doctor or dietician about getting adequate calcium and vitamin D
• Regular weight-bearing and muscle-strengthening activity that is tailored to your needs and abilities
• A fall assessment is recommended for anyone with osteoporosis or fragility fractures and exercises to improve balance are recommended
  • Things like vision assessments, assistive devices, and working with occupational therapy and EDS-aware physical therapists can really help here
• Restrict alcohol intake and smoking
• Speak with your provider about regular Bone Density Scans (DEXA) to monitor any changes in bone density. This is particularly important in post-menopausal women and men over 50 if you have other risk factors listed above.
• Medication checks – some meds can impact bone density over time.

Resistance work is great for bone health. Try out this intro to working with a resistance band. We have many classes in The Zebra Club using resistance band and weight bearing exercises.

FAQ

Are people with EDS more likely to break bones?

There does seem to be some evidence for slightly increased risk of fractures in hEDS. Staying active and participating in weight bearing activity may help with bone strength.

Does Ehlers-Danlos cause osteoarthritis?

It is not clear if Ehlers-Danlos causes osteoarthritis, there is not very much research published on this connection. Anecdotally, arthritis is common among and some include early osteoarthritis when listing symptoms associated with hypermobile Ehlers-Danlos Syndrome (13).

What disorder is strongly associated with osteoporosis?

There are many conditions strongly associated with osteoporosis including diabetes, inflammatory diseases (like rheumatoid arthritis), celiac disease, chronic liver disease, vitamin D deficiency, Renal disease, cardiovascular disease, and dementia.

Jeannie Di Bon

Jeannie Di Bon is a movement therapist, educator and author specializing in hypermobility, Ehlers-Danlos Syndrome and chronic pain. She is the founder of The Zebra Club app and the creator of the Integral Movement Method.