Bone Density and Skeletal Fragility in hEDS

Many people living with hypermobile Ehlers-Danlos syndrome (hEDS) experience chronic joint pain, fatigue, and soft tissue injuries—but fewer are aware that fragile bones, low bone density, and an increased risk of fractures may also be part of the condition. Although bone loss is more commonly associated with aging or menopause, studies show that people with hEDS of all ages may have lower bone strength than average. This article explains what is known about bone density in hEDS, how it may affect people over time, what causes it, and what can be done to prevent or treat it.

Overview of Bone Density Problems in hEDS

Bone density refers to how strong and solid bones are. Lower-than-normal bone density makes bones more likely to break (fracture), even during everyday activities. Conditions such as osteopenia (mildly low bone density) and osteoporosis (more severe bone loss) weaken bones and can occur in people with hEDS—even at younger ages than expected.

What the Research Shows

Symptoms and What to Look For

Bone loss in hEDS doesn’t always cause symptoms. Some people may not realize their bone density is low until they experience a fracture. However, some signs and associated symptoms include:

• Frequent or unusual fractures, especially in the spine or limbs

• Chronic back pain, sometimes related to vertebral fractures

• Delayed motor development in children (e.g., learning to walk later than expected)

• Loss of height in adults, which may be caused by subtle spinal fractures

Causes of Bone Fragility in hEDS

Several overlapping factors may contribute to weak bones in people with hEDS. These include both proven causes and likely mechanisms based on the biology of connective tissue disorders.

• Known Contributors

  • Connective tissue abnormalities: hEDS involves defects in collagen and other structural proteins that support not only skin and joints but also bones. This can weaken the bone structure itself.

  • Reduced bone quality: Even when bone density appears normal on a scan, the internal structure of the bone (its “architecture”) may be compromised, increasing the risk of fractures.

  • Gastrointestinal problems: People with hEDS often have digestive issues that reduce the body’s ability to absorb nutrients like calcium, vitamin D, and protein—vital for building and maintaining bone.

  • Low muscle mass and inactivity: Muscle pulls on bone and helps keep it strong. But pain, joint instability, and fatigue often cause people with hEDS to move less, reducing stimulation for bone growth.

• Theoretical or Emerging Explanations

  • Hormonal imbalances: Conditions that affect hormone levels (such as early menopause or thyroid disorders) may worsen bone loss.

  • Chronic inflammation: Some people with hEDS have immune-related conditions such as mast cell activation syndrome (MCAS), which may contribute to bone breakdown through inflammatory processes.

  • Abnormal mechanical signaling: Because connective tissues are looser, bones may receive altered pressure and feedback during movement, which could interfere with normal bone remodeling.

How Bone Density Is Measured and Diagnosed

Bone density is typically measured using a scan called a DXA (dual-energy X-ray absorptiometry) scan. This test looks at how much mineral is packed into your bones.

• In adults, results are given as a T-score.

  • T-score between -1.0 and -2.5 = osteopenia (mild bone loss)

  • T-score ≤ -2.5 = osteoporosis

• In younger people (under 50), results are given as a Z-score.

  • Z-score ≤ -2.0 = bone density is below the expected range for age

But in hEDS, bone scans may not tell the whole story. Bone quality—the internal structure that supports bone strength—can also be reduced, even if density appears normal. A special tool called a trabecular bone score (TBS) can help evaluate this.

When to Get Checked

Experts recommend bone density testing if any of the following are true

• You’ve lost more than an inch of height

• You’ve had a fracture from a minor injury

• X-rays suggest thinning bones

• You have severe back pain, especially if it’s new or sudden

Children and teens with hEDS don’t need routine bone scans unless they have pain, growth problems, or a history of fractures.

Protecting Bone Health: Lifestyle and Non-Drug Strategies

The foundation of treatment for low bone density in hEDS is a healthy lifestyle that supports bone strength.

• Nutrition

  • Calcium: 1,000–1,200 mg/day (from food or supplements)

  • Vitamin D: 1,000–2,000 IU/day (adjusted based on blood tests)

  • Ensure adequate protein intake, especially if GI symptoms limit digestion

• Exercise

  • Weight-bearing activities (e.g., walking, dancing) help stimulate bone

  • Resistance training builds muscle and improves balance

  • Programs should be adapted to avoid joint injury and account for fatigue

  • In children, physical activity helps build peak bone mass during growth

• Fall prevention

  • Balance training and strength building

  • Remove tripping hazards in the home

  • Use assistive devices if needed to prevent injury

• Avoid bone-damaging behaviors

  • Don’t smoke

  • Limit alcohol

Medications

Medication is usually only considered in adults with

• A T-score in the osteoporosis range (≤ -2.5)

• A history of fracture, especially with minor trauma

• High estimated fracture risk

Types of medications include

These drugs are not typically used in children or younger adults unless bone loss is severe and due to other medical issues. Pediatric cases should be handled by a specialist in endocrinology.

Quality of Life and Lived Experience

Pain, fatigue, and fractures can greatly affect daily life in hEDS. People with vertebral fractures often report:

• Difficulty standing or walking for long periods

• Worsening fatigue

• Height loss

• Increased risk of further fractures

In children and teens, pain can lead to school absences, reduced participation in sports, and social withdrawal. Early recognition of bone issues and proper management can help improve quality of life and physical function.

Long-Term Outlook and Gaps in Knowledge

Many people with hEDS and low bone density live active, fulfilling lives. With early attention to nutrition, activity, and screening, fractures may be preventable. Still, gaps remain:

• Not enough research on bone loss in children and teens with hEDS

• Unclear whether current tools such as FRAX (fracture risk calculator) work well in hEDS

• No drug trials specific to hEDS

• Limited understanding of how altered connective tissue affects bone strength at a cellular level

Summary

Reduced bone density is a meaningful but often overlooked part of hEDS. It can occur at any age and may lead to fractures, chronic pain, and reduced mobility. While BMD scans can detect some problems, they don’t always capture the whole picture—especially in hEDS, where bone quality may be poor even with normal density.

A combination of tailored physical activity, proper nutrition, and selective use of medications (for high-risk adults) offers the best chance of maintaining strong bones. Early action—especially in youth or young adults with signs of bone issues—can make a long-term difference. Ongoing research is needed to understand the biology of bone fragility in hEDS and develop better treatments.