Managing Chronic Pain in Ehlers-Danlos Syndrome (EDS)
Updated: Nov 29
Living with constant pain is exhausting. Your joints ache with every step. Your muscles burn at the slightest movement. Sharp stabbing sensations shoot down your limbs for no reason. Even a gentle hug hurts. Everyone thinks you are faking it, they think you are a hypochondriac, they think you are a drug seeker and the pharmacists treat you like shit!
This is the reality for the nearly 5 million Americans living with chronic widespread pain from Ehlers-Danlos Syndrome (EDS).
EDS is a debilitating condition causing loose joints, fragile skin and chronic pain. The pain often starts in childhood or adolescence, becoming more severe over time. It affects nearly every part of the body - joints, muscles, back, head, and abdomen. For many, the pain is so severe it impacts their ability to work, go to school, and care for themselves.
You may feel like no treatment helps ease your suffering. The pain persists despite pain pills, creams, and physical therapy. Days are spent in bed unable to move without agony. Nights are sleepless from the relentless aches. Life starts revolving around pain.
This article is dedicated to my inspiring patients living with Ehlers-Danlos Syndrome. Your strength and resilience in the face of chronic pain amazes me daily. Even on the worst days, you show up with a smile, ready to take on your challenges. I initially intended this article to be a brief overview of chronic pain in EDS. However, I realized the complexity of the condition required a more comprehensive, in-depth analysis to properly educate readers. I hope this article provides you with new information and a rekindled hope. Please know that you are heard, valued, and never alone.
What is Ehlers-Danlos Syndrome (EDS)?
Ehlers-Danlos Syndrome (EDS) is a group of inherited disorders that affect connective tissue throughout the body. Connective tissues provide structure and support in joints, organs, skin, blood vessels, and muscles.
In 1901, a dermatologist named Edward Ehlers published a case report describing a patient with hyperelastic skin and fragility of the skin and joints. In 1908, French physician Henri-Alexandre Danlos built on Ehlers' findings by reporting patients with "cutis elastica" and joint hypermobility.
There are 13 different types of EDS, but the most common is the hypermobile EDS (hEDS), making up about 80-90% of cases.
People with EDS usually have overly flexible joints and stretchy skin. Common symptoms include:
Joint hypermobility (loose, unstable joints that dislocate easily)
Soft, velvety skin that bruises easily
Fragile skin that tears easily
Digestive issues like constipation and diarrhea
Chronic fatigue and decreased stamina. Fatigue is reported by up to 90% of EDS patients.
Gastrointestinal issues like gastroesophageal reflux (GERD), irritable bowel syndrome (IBS), constipation or diarrhea. Can cause abdominal pain.
Dysautonomia - problems with the autonomic nervous system. Can cause dizziness, fainting, rapid heart rate, nausea.
Poor proprioception and coordination. Many EDS patients describe being "klutzy".
Vision issues like nearsightedness, retinal detachment, early onset glaucoma.
Early onset varicose veins and hernias.
Female health issues: painful periods, pelvic organ prolapse, urinary incontinence.
Problems with wound healing and excessive scarring.
Osteopenia and early onset osteoporosis leading to increased fractures.
Sleep disturbances like sleep apnea, restless leg syndrome, insomnia.
Anxiety and depression are common, especially in those with severe pain.
Migraines and other headaches.
Some types of EDS affect the heart valves or arteries increasing the risk for complications.
Pregnancy complications and preterm birth are more common.
Many people with hEDS are often first diagnosed in childhood or adolescence when their hyperflexible joints and soft skin become more noticeable.
Chronic Pain in EDS
Chronic pain is a very common problem in EDS, especially in the hypermobile type. About 90% of people with EDS experience chronic widespread pain.
The pain often starts in childhood or adolescence and occurs across multiple areas of the body. The pain can be constant or come and go in flares.
Common sites of pain include:
Joints (knees, shoulders, hands, hips, ankles, elbows, jaw)
The pain can become severe and debilitating over time, greatly reducing the quality of life. Pain is one of the main symptoms that brings people with EDS to the doctor to get diagnosed.
Causes of Pain in EDS
The causes of chronic pain in EDS are complex and not fully understood. Likely, it is a combination of factors:
1. Joint instability
The hypermobile joints in EDS are unstable and prone to frequent dislocations and subluxations. This joint instability causes damage to the soft tissues around the joints like tendons and ligaments.
Repeated minor injuries to the joints from small dislocations add up over time, resulting in chronic pain. Joints most prone to dislocations and injury include knees, shoulders, hips, elbows and ankles.
2. Muscle pain
EDS causes poor muscle tone. Weak muscles have to work harder to stabilize hypermobile joints, often resulting in muscle sprains, strains and myofascial pain. The constant muscle tension required to support loose joints is painful.
3. Nerve pain
The unstable joints in EDS can lead to compressed or damaged nerves. For example, dislocated joints can pinch nerves, causing shooting nerve pain.
EDS patients may also suffer from chronic nerve pain conditions along with fibromyalgia.
4. Central sensitization
Central sensitization is increased sensitivity to pain signals in the brain and spinal cord. The constant pain signals from joints, muscles and nerves in EDS is thought to cause changes in the central nervous system.
As a result, the pain threshold is lowered so that normal touch, pressure and movement is perceived as very painful (hypersensitivity). Central sensitization can cause widespread, hard-to-treat pain.
5. Secondary conditions
Sometimes the chronic pain in EDS is coming from another condition that developed alongside it. For example:
Fibromyalgia - Estimates range widely, but some research suggests up to 42% of EDS patients also have fibromyalgia. The hallmark is widespread muscle pain and tenderness.
Migraines - Migraines and other headaches are common in EDS, reported in up to 48% of patients in some studies. Migraines cause severe pulsating head pain.
Irritable bowel syndrome (IBS) - Up to 43% of EDS patients may suffer from IBS leading to abdominal pain, diarrhea and constipation.
Endometriosis - Extremely painful menstrual periods along with pelvic pain affects up to 77% of women with EDS, indicating a high rate of endometriosis.
Interstitial cystitis - Chronic bladder and pelvic pain are frequent complaints, suggesting interstitial cystitis is more prevalent.
Temporomandibular joint dysfunction - Up to 93% of EDS patients have TMJ issues causing severe jaw and facial pain.
Neuropathy - Nerve pain in the hands and feet affects about 68% of EDS patients.
These co-existing or "co-morbid" chronic pain conditions are very common in EDS patients. They add to the total pain burden.
The fragile connective tissues in EDS mean injuries happen more often and take longer to heal. Sprains, strains, fractures and surgery often cause acute pain on top of chronic pain. Slow healing after injuries is also very painful.
7. Psychological factors
Living with constant widespread pain for years understandably takes a psychological toll. Many people with EDS also suffer from depression, anxiety and stress.
Psychological and emotional factors interact with physical pain to make it feel more intense. They also make it harder for patients to cope with the pain.
POTS (Postural Orthostatic Tachycardia Syndrome)
POTS is a form of dysautonomia - an abnormality of the autonomic nervous system which controls functions like heart rate, blood pressure, digestion and more.
POTS is characterized by a rapid increase in heart rate when standing up, along with symptoms like dizziness, lightheadedness and fainting.
About 50% of people with EDS also have POTS. The hypermobile EDS type has the strongest association.
Why POTS develops is unclear but likely involves inadequate constriction of blood vessels and being more stretchy allows blood to pool in limbs when upright.
Treatment involves drinking more fluids, wearing compression stockings, increasing salt and water intake, exercise, and sometimes medications like beta blockers.
POTS can cause fatigue, trouble concentrating, headache, nausea and worsen pain while standing.
POTS symptoms often overlap with chronic fatigue syndrome (CFS) which is also more common in EDS patients.
So in summary, POTS is another frequently associated condition with EDS that can worsen pain, fatigue and other symptoms. Treating POTS can help improve the associated issues.
Diagnosing Chronic Pain in EDS
There is no lab test to diagnose EDS or the chronic pain associated with EDS. Doctors diagnose EDS based on a thorough history, physical exam, family history, and ruling out other conditions.
To evaluate chronic pain in EDS, the doctor will ask detailed questions about:
Location of pain
When the pain started, how has it changed
Triggers and relievers of pain
Impact of pain on work, activities, sleep, and mood
Past treatments tried and response
A physical exam assesses joint mobility, muscle strength, and areas of tenderness. Your doctor may use pain scales to determine pain severity. Specialists like rheumatologists may do additional testing to rule out other types of arthritis.
EDS cannot be confirmed with imaging like x-rays. However, some imaging may be needed to assess joint damage or issues like herniated discs contributing to pain.
There are no blood tests that can diagnose EDS or determine severity of chronic pain. However, blood tests can help rule out other types of arthritis.
Nerve conductivity studies may be used to evaluate nerve pain and look for conditions like carpal tunnel syndrome.
Overall, the diagnosis relies heavily on a detailed history and physical. Tracking pain symptoms in a pain diary can provide helpful information to your doctor.
Treatment for Chronic Pain in EDS
The complex nature of chronic pain in EDS means that no single treatment works for everyone. The best treatment approach combines both pharmaceutical and non-drug therapies tailored to each person.
Common medications used to treat EDS pain include:
NSAIDs: Anti-inflammatory medicines like ibuprofen help with joint and muscle pain. They should always be taken with food.
Acetaminophen: Helps mild to moderate musculoskeletal pain.
Tramadol: A opioid medication that can treat moderate to moderately severe pain.
Buprenorphine: An opioid medication with
Antidepressants: Certain tricyclic antidepressants like amitriptyline are often effective for nerve pain. SNRIs like duloxetine help both muscle and nerve pain.
Anticonvulsants: Seizure meds like gabapentin help calm down nerve pain.
Muscle relaxers: Relax tight, painful muscles.
Nerve blocks/injections: Used for specific nerves causing intense pain. Anesthetic and steroid medication is injected around affected nerves. These procedures have been reported to provide the best pain relief.
Medical marijuana: May help relieve severe pain that is not controlled with other medications. Available as oils, edibles, creams.
Ketamine Infusions: By some estimates, up to 30% of people with chronic pain have a significant reduction in pain with ketamine infusions.
Trigger point injections: used in EDS patients with chronic myofascial pain unresponsive to other therapies
There are a few important considerations regarding medication responses in people with Ehlers-Danlos syndrome (EDS):
EDS patients may have abnormal metabolism and clearance of certain medications due to genetic differences affecting liver enzymes. The dose and frequency required for therapeutic effect might differ.
There is a theory that patients with EDS may require higher doses of local anesthetics for adequate pain relief, though research findings are mixed. Some experts advise cautious dosing of anesthetics.
NSAIDs should be used carefully given many EDS patients have co-existing gastrointestinal issues like gastric reflux that can be aggravated.
Medications that reduce collagen production or impair healing (like steroids) are generally avoided to not worsen connective tissue weakness.
Drugs with anticholinergic side effects can exacerbate issues like dizziness and constipation already common in EDS.
Any medication that can affect coordination or balance should be monitored closely given tendency for unstable joints.
Various chronic pain medications often come with side effects like brain fog that patients may be more sensitive to.
Note that opioids like morphine have significant side effects and risk of addiction. They are usually avoided for long-term treatment of chronic pain.
Non-medication evidence-based treatments should be used alongside medications for best pain relief. They help improve function with less risk of side effects.
Exercise: Gentle strengthening and aerobic exercise prevents worsening of joints and muscles. But overdoing activity can trigger flares. Finding the right balance is key. Water exercise is ideal.
Physical therapy: Stretches, mobilization and exercises strengthen muscles around joints for more support and stability. Can also use tape, splints or braces as needed.
Heat/cold therapy: Heat helps relax tight muscles. Cold therapy brings down inflammation. Contrast baths alternate both.
Massage: Promotes blood flow and relaxes chronically tight muscles. Gentle massage is often helpful.
Rest: Pacing activity with rest periods prevents overexertion which causes flares.
Stress management: Relaxation techniques, meditation, journaling, therapy and support groups help with emotional health. This empowers patients manage pain better.
Cognitive behavioral therapy (CBT): CBT and pain psychology trains the mind to respond differently to chronic pain signals. This reduces suffering and disability.
A multidisciplinary treatment approach with different specialists led by a pain management doctor offers the most benefits. The treatment team may include physical therapists, psychologists, massage therapists and more.
Living with Chronic Pain in EDS
Chronic pain is currently not curable in EDS, but the right treatments can help manage pain to restore function and improve quality of life. This involves learning to strike the right balance in daily life.
Pacing activity by alternating movement with rest periods prevents pain flares from overexertion. Gentle regular exercise keeps muscles flexible without strain.
Braces, splints or knee/ankle supports provide extra joint stability for sports and exercise. Good posture and ergonomics protect the joints. Proper body mechanics uses joints safely - for example, bending knees to lift versus bending over from the waist.
Using medications as prescribed keep pain at bay. Non-drug treatments like massage, ice/heat therapy and relaxation techniques help break the pain cycle.
It takes time to find the right balance between activity and rest, strengthening versus overuse. But working closely with your healthcare team, it's possible to find an optimal treatment plan.
Arm yourself with up-to-date information. Subscribe to the Ehlers Danlos Society's newsletter and monitor the breakthroughs as they are reported. There might be something new that could help you.
While living with chronic pain in EDS can feel overwhelming, the right treatment makes consistent pain manageable. This restores independence and active participation in work, hobbies and social life.
Chronic pain affects about 90% of people living with Ehlers-Danlos Syndrome. The degree of pain ranges from mild to severe and disabling.
Joint instability, muscle dysfunction, injuries, and central sensitization likely all contribute to chronic widespread pain. Psychological and emotional factors also play a role.
Doctors diagnose EDS and associated chronic pain based on a detailed history, physical exam and ruling out other conditions.
Treatment is tailored to each person but ideally includes both medications and non-drug therapies like exercise, bracing, massage and stress management.
While currently not curable, chronic pain in EDS can often be successfully managed. Treatment restores quality of life and functioning. Close work with a multidisciplinary medical team helps patients strike the right balance.
Frequently Asked Questions
How is chronic pain in EDS diagnosed?
There is no specific test for diagnosing EDS or the chronic pain. Doctors make the diagnosis based on a detailed history, physical examination, family history, and ruling out other conditions that could cause pain. They may order some tests like imaging or bloodwork to look for other causes.
What are some treatments to help EDS pain?
Treatments that may help EDS pain include pain medications, exercises to strengthen muscles around joints, braces or splints, physical therapy, heat/cold therapy, massage, nerve blocks, cognitive behavioral therapy, stress management and more. A combination approach tailored to each patient works best.
Is the pain in EDS constant?
The pain may be constant or come and go in flares. Many people report baseline chronic pain that gets worse with certain activities or after overuse. Acute injuries can also cause temporary severe pain on top of the chronic pain.
Can EDS pain be cured?
There is currently no cure for the chronic pain caused by EDS. However, with the right treatment plan, the pain can often be managed to a point where patients can function and have a good quality of life.
Does EDS pain get worse over time?
In many patients, the pain does get worse over time. This seems to be related to cumulative joint instability and injuries. However, patients who protect joints, avoid injuries, and stay active typically do better long-term. Early diagnosis and treatment helps prevent progression.
What joints are most commonly painful?
The joints most often affected by chronic pain are the knees, shoulders, hips, hands, elbows, ankles and jaw. The spine is also a common source of pain.
Can surgery help EDS pain?
Surgery is generally avoided in EDS if possible because healing takes longer and the joints remain unstable. However, sometimes surgery is necessary for injuries like joint reconstruction or hernia repair. Nerve decompression or disc surgery may help some cases of nerve compression pain. Overall, surgery does not cure EDS pain long-term.
About the author:
Dr. Harold Pierre is a board-certified anesthesiologist and addiction medicine specialist with over 20 years of experience. He is board-certified by the American Board of Anesthesiology and the American Board of Preventive Medicine.
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