Hypermobile Ehlers-Danlos Syndrome, Joint hypermobility, lumbar instability and low back pain
Ross Hauser, MD.
If you suffer from Hypermobile Ehlers-Danlos Syndrome you will likely not need to be told of the complexity of diagnosis and treatment. You yourself are probably a very complex case. This article will focus on understanding possible treatments and identifying what may or may not help you. As people with Hypermobile Ehlers-Danlos Syndrome also suffer from issues of self-doubt because some of their doctors don’t believe them and they suffer from clinical-level depression, we hope some of the information in this article will help reassure you that many doctors are trying to help.
Your story is unfortunately a familiar story.
Many people that reach out to us have a story, as you will read this article you will find that their story sounds a lot like your story. You may be part of a support group or have researched on your own searching for “how do other people deal with this?” Some of these people, as you will read or may have found, have very complex medical histories and a dwindling realistic expectation that they can ever achieve a significant level of pain relief, more sustained and long-term treatment effectiveness, and a little better quality of life.
In this article, we will explore lower or lumbar back pain and the possible treatments. At our center, we use injection therapy for Hypermobile Ehlers-Danlos Syndrome.
A story – My doctors are at a loss to offer me anything at this point
I have pain all over but it is my back pain that keeps me in bed. My doctors have told me, and I agree, that the prolonged bed rest is not helping my back pain. But my doctors are at a loss to offer me anything at this point except possibly more physical therapy, muscle relaxers, epidurals, painkillers, narcotics that make me sick, anti-depressants, sleep aids, psychiatric counseling, and possibly social workers to make sure “everything is alright at home.” I know eventually, I will get to the point where they will tell me there is not much they can do for me except stronger medications or steroid injections.
While my doctors have focused on Sacroiliac Joint Dysfunction, nothing they are doing for me is helping
After years of looking for answers, I have finally and recently been diagnosed with hEDS. I have been experiencing extreme lower back pain and while my doctors have focused on Sacroiliac Joint Dysfunction, nothing they are doing for me is helping. It is just not my lower back. It seems my whole body is in a state of hypermobility and my other joints are becoming more painful. For my Sacroiliac Joint Dysfunction, my doctors are working on a surgical plan. I do not know if I have the confidence to go through with surgery when nothing else has worked.
Doctors are trying to understand hypermobility spectrum disorder, Ehlers-Danlos syndrome, and low back pain
In the many stories, we hear from people, a common theme emerges, “my doctors don’t know how to help me,” “my doctors don’t understand,” and “all my doctors want to do is offer me painkillers and medications.” As you may know firsthand, the diagnosis and treatment of Hypermobile type Ehlers-Danlos syndrome (hEDS) are challenging and many doctors feel there is currently not enough education or information to help them help their patients. Doctors themselves understand this.
In June 2021, doctors at the Guy’s and St Thomas’ Hospitals in London published a review paper (1) outlining recent advances in diagnostic criteria for hypermobility spectrum disorder and its association with Ehlers-Danlos syndrome. Further, they also reviewed the current literature on the association between joint hypermobility syndrome and lumbar back pain. They also explored some of the difficulties in diagnosis and treatment. Here are some of the learning points of this paper which appeared in the International Journal of Spine Surgery.
Doctors can’t see this on an MRI – Yes you can have significant back pain with a normal MRI.
One of the challenges people with hEDS and low back pain, or any joint pain for that matter, is that their doctors are confused because they cannot see any obvious destruction on MRI or x-ray that is significant enough to recommend a spinal fusion or other less invasive spinal procedures such as laminectomy as open or minimally invasive surgery. Yet other patients with lumbar back pain and hEDS will have obvious x-ray and MRI confirmation of scoliosis with sometimes a more severe sideways curvature and humpback or kyphosis that can extend towards the lower spine.
The researchers of this study noted what we have seen many times in our patients with big stacks of MRIs. The MRI can lead you down the right path of treatment, the MRI can lead you down the wrong path of treatment.
The Jekyll/Hyde MRI – “patients may have significant pain in the absence of positive radiological findings.” Yes, you can have significant back pain with a normal MRI.
MRI accuracy or MRI interpretation accuracy, especially in those with both hypermobility spectrum disorder and hEDS can, and in many cases, be viewed as a possible help tool in assisting the doctor and patient understand what is happening in their spine. In other words, the MRI can be a good guide to treatment.
However, MRI interpretation accuracy should also be viewed cautiously as a possible hindrance and a “red herring” in determining the patient’s treatment program. The absence of obvious abnormality may also lead some health care providers to possibly doubt the veracity of the patient’s claim that they have significant low back pain.
Here is what the study authors had to say:
“When managing patients with chronic low back pain and hypermobility (both hypermobility spectrum disorder and hEDS), the treating clinician must be aware that patients may have significant pain in the absence of positive radiological findings. That the experience of low back pain is more complex than purely a direct relationship with anatomical radiological changes is well accepted in the literature on chronic low back pain (Our note: Yes you can have significant back pain with a normal MRI).
The evidence of spinal radiological changes in asymptomatic patients that worsen with age is relevant to any interpretation of radiological findings. (Our note: You can have a terrible-looking MRI BUT it is not indicative of what is causing your pain).
This second observation may explain why patients with hypermobility and chronic low back pain may fail to respond
This second observation may explain why patients with hypermobility and chronic low back pain may fail to respond to anatomically targeted pain management and surgical intervention. (Our note: Patients fail treatment when the doctors concentrate on “treating the MRI” and do not further explore the patient’s back pain” outside of the MRI findings.
This research also concluded with these suggestions and cautions about spinal surgery in EDS patients.
“Rates of perioperative complications after lumbar spinal surgery in the hypermobile EDS population have been reported to be up to 50%. When hypermobility and chronic lumbar back pain coexist, we advocate management in a multidisciplinary setting involving physiotherapists, pain physicians, surgeons, and psychologists.”
This challenge of MRI-based treatments can be seen in some of the people who contact us with medical histories as we demonstrated above.
The challenges of spinal surgery, the challenges of multiple spinal surgeries
For many patients, their spinal surgery will fail because there is simply too much instability and when you fix one area you cause segmental or adjacent disease in the other area of the spine. This is why many people have many surgeries. This adjacent segment disease problem is in part caused by ligaments loosening after surgery.
Doctors at the University of Colorado published a case history to show that in certain patients, certain surgery can be effective. In this case an artificial disc replacement. This case history was published in the International Journal of spine surgery (2). Here is the summary.
- A 52-year-old woman with a clinical diagnosis of EDS and degenerative disc disease at L4-5.
- She had had previous micro discectomies that did not alleviate her situation of pain.
- The patient was referred for genetic evaluation, which classified her with type III EDS, or hypermobility type.
The patient DID NOT HAVE extreme spinal hypermobility
The doctors of this paper then wrote that they “presented the patient with the risks and benefits of fusion versus artificial disc replacement, particularly with regard to her EDS diagnosis of the hypermobility subtype. Given the patient’s lack of extreme spinal hypermobility on examination and the absence of clear contraindications regarding artificial disc replacement in type III EDS, the decision was made to proceed with artificial disc replacement. There were no surgical complications, and the patient’s low-back pain and lumbar radicular symptoms resolved with no evidence of implant migration or hypermobility at 1 year postoperatively.
The doctors reported that “in this case report, the referral to a geneticist and consultation with a vascular surgeon were integral steps in the decision to proceed with surgery. Although the clarified diagnosis of type III EDS did not eliminate the potential risk for vascular compromise during surgery, it placed the patient at lower risk than patients with other subtypes of EDS.
Similarly, her lack of extreme hypermobility made us more comfortable with pursuing artificial disc replacement. Although we emphasize extreme caution when considering surgical treatment, this case report suggests that some patients with less severe forms of EDS may be able to successfully undergo anterior spine surgery, including artificial disc replacement.
More on spinal fusion vs. total disc replacement
There is very limited research to support total disc replacement in the hEDS patient. You yourself may have explored this option with your surgeon if you had previous surgery like the woman in the case history did. One reluctance of the surgeons to move forward with total disc replacement is that they are being cautious in regard to the limited long-term outcomes of total disc replacement surgery. The paper above is from 2012 and there is little surrounding evidence to support the benefits of total disc replacement in general. The case history the doctors presented is considered unique and worthy of inclusion in a medical journal because of this uniqueness and the possibility it can help other surgeons better identify surgical or non-surgical treatments that will help their patients.
Doctors have noted that in most patients with spinal instability, either from degenerative disease weakening the spinal ligaments or a spinal hypermobility disorder such as hEDS, spinal instability, recognized as the cause and subsequent development of lumbar disc herniation, will place lumbar spine, and in many cases the entire spine, hip, groin, and pelvic regions to develop or accelerate instability. In the lumbar spine, this will lead to vertebrae segmental instability. In this scenario, one you may already be familiar with, a recommendation for bigger fusions may be offered.
What are we seeing in this image?
This is an MRI showing lumbar spondylosis. In this MRI this patient suffers from:
- Endplate sclerosis, ( the shock-absorbing structure of cartilage and porous bone has now hardened or degenerated and is no longer protecting the disc and vertebrae. Endplate sclerosis can often be a leading indicator to the recommendation of spinal fusion surgery.)
- Disc narrowing or loss of disc height.
- Osteophytes or more commonly bone spurs.
In our experience, this degenerative condition of the spine and spondylosis is caused by facet joint instability in the spine. The vertebrae are hypermobile because their posterior connections to each other are lax and loose. The vertebrae are “floating around” causing disc herniation, slipped discs, and vertebrae sliding away from each other. This is the cause of severe pain in some.
What are we seeing in this image? A further understanding of facet joint instability
In this image, we see an illustration of the side or lateral view of two adjacent vertebral segments. What are we seeing? Facet joint hypermobility in the spine causes excessive motion between the vertebrae. This excessive movement as described above leads to a disc injury, disc herniations, and disc degeneration.
Ehlers-Danlos Syndrome and Sacroiliac Joint Dysfunction
Many people contact us with a confirmed diagnosis of Sacroiliac Joint Dysfunction. They, like perhaps yourself, have low back pain that extends into the hips and pelvis at the sacroiliac joint. Their hEDS, joint hypermobility diagnosis has led their doctors to follow the path that ligament laxity is causing hypermobility in the SI Joint and this hypermobility is wearing away at the cartilage of the joint and contributing to pain and will in many cases ultimately lead to a recommendation for Sacroiliac Joint Fusion.
“I’ve tried almost everything”
By the time we see a patient who has been recommended for spinal fusion or SI Joint Fusion, they will tell us their medical history of a long list of treatments that may have helped, no longer helped, did not help, or made things worse. They will conclude their history summary with the familiar “I’ve tried almost everything”
Many of you understand what “almost everything is.”
- Physical therapy and Yoga
- Facet joint denervation
- Trochanteric belt
- Acupuncture with laser
- TENS units
- Platelet Rich Plasma Injections – explained further below.
- Active Release Therapy
- Graston Technique – a massage technique using steel tools to “Scrape” the skin and muscles.
- Postural therapy – a quick series of exercises that uses body weight and gravity to realign the posture.
- Massage therapy
Neurocognitive rehabilitation in hEDS patients
A June 2021 paper in the journal BioMed Research International (7). “Patients with hEDS suffer joint pain, in particular low back pain, commonly resistant to drug therapy. In this study, a neurocognitive rehabilitation approach was used in eighteen hEDS patients (4 males and 14 females) with an average age of 21 years (range 13 years old, the youngest – 55 years old, the oldest). Neurocognitive rehabilitation retrains the brain into how it thinks or dwells on chronic pain. The thinking is that by giving pain “less attention” the patient will suffer from less pain. The clinical results observed in this study “seem to confirm the role of a specific neurocognitive rehabilitation program in the chronic pain management in the Ehlers-Danlos syndrome; the rehabilitation treatment should be tailored on patient problems and focused not only on the recovery of movement but also on pain perception.”
Facet joint denervation
In the journal, Neurological Sciences (3) doctors published a May 2021 paper which offered the benefits of facet joint denervation in selected patients. In this study, 16 patients with neuromuscular disorders causing kyphoscoliosis were divided into two groups. Those treated with facet joint denervation alone and those treated with multimodal treatment, including facet joint denervation along with radiofrequency ablation for sacroiliac joint pain, piriform muscle block, botulinum toxin injection into the paraspinal muscles, spinal cord stimulation, or any of their combinations. All patients were followed up for 48 weeks postoperatively.
- Results: There was no significant difference between the groups. However multimodal treatment including facet joint denervation was suggested as a safe and relatively effective treatment in patients with neuromuscular disorders-associated kyphoscoliosis and can be considered a potential substitute for conventional spinal fixation surgery, which has a higher risk of complications.
A discussion of injection treatments
Our first examination will be that of corticosteroid or epidural steroid injection. Typically cortisone injections are not, or should not be, the “go-to” treatment for Ehlers-Danlos Syndrome as it is or was for more common musculoskeletal problems of joint and spine pain and inflammation.
The risks and side effects of cortisone are well known. These risks will be briefly discussed here. First, let’s look at a paper in the journal Current Osteoporosis Reports (5) published in April 2020. This paper is a discussion of “bone findings, mainly bone mass and fracture risk, in Ehlers-Danlos syndromes.”
What the authors discuss is that low bone mineral density and fractures seem to be frequent in some of the rare EDS types (kyphoscoliotic (the condition of concurrent kyphosis and scoliosis), (arthrochalasia Ehlers-Danlos syndrome (aEDS)), (spondylodysplastic Ehlers-Danlos syndrome (spEDS) and classic-like EDS).
They concluded: “For the more prevalent hypermobile and classic EDS types, some case-control studies found mildly decreased bone mineral density, but it was not clear that fracture rates were increased. Nevertheless, abnormalities in vertebral shape seem to be common in classical and hypermobile EDS types.”
As you may be first-hand aware because the topic has been discussed with you by one or more of your doctors, bone density and fractures in Ehlers-Danlos syndrome are considered by some as controversial. The controversy was discussed in an August 2016 paper in the journal Osteoporosis International. (6) Here doctors noted: “The Ehlers-Danlos syndrome is characterized by abnormal connective tissue but bone involvement is debated. (However the researchers) found a reduced bone mineral density and bone quality and an increased prevalence of asymptomatic vertebral fractures in patients with Ehlers-Danlos syndrome. These findings suggest the need for a bone health evaluation in these patients.
Whether or not it can be documented without controversy that hEDS patients suffer from low bone mineral density or increased risk for vertebrae fractures, there is concern that steroid or epidural steroid injections will create more problems including fractures.
In a June 2021 study (4) not limited to hEDS patients, doctors at the Endocrinology and Metabolic Bone Disease Service, at the Hospital for Special Surgery in New York described the skeletal consequences of epidural steroid injections.
Here are the summary learning points:
- “While the evidence is limited, studies suggest that epidural steroid injections may cause bone loss, particularly those (studies) investigating volumetric bone mass density. Larger (cortisone) doses appear to confer greater risk. Further prospective studies are needed to investigate the relationship between epidural steroid injections and fracture risk. A better understanding of the skeletal consequences of epidural steroid injections will help foster strategies to prevent bone loss in the growing population of patients receiving this treatment.”
Prolotherapy – A “typical patient”
It is difficult to use the word “typical” in cases that are certainly “atypical” among the general population. Yet here is one such “typical” or representative case of the type of people who reach out to us.
A patient came to our center with a long medical history of severe joint pain and frequent dislocations of both elbows, knees, and shoulders as well as subluxations and dislocations throughout her spine and the sacroiliac (SI) joints.
She told us her story of first experiencing joint pain at the age of ten. Into her teenage years and as she became involved in track and field her joint pain worsened. The first incident of joint dislocation occurred when her knee gave out during a track meet and this resulted in her undergoing multiple knee reconstructive surgeries. While having short-term pain relief in that knee, eventually she continued to experience pain and instability in that knee. By the age of 21, new symptoms appeared and she began experiencing cardiovascular symptoms including tachycardia and feeling faint. It was at her consultation with a cardiologist that she learned that she suffered from Ehlers-Danlos Syndrome.
Over the years the patient’s condition began to affect other joints in her body including her shoulders, elbows, and sacroiliac joint. Simple activities, she reported, could cause her joints to dislocate. Sometimes on a daily basis. Eventually, she would be unable to walk or in fact, use crutches or a cane because these would cause dislocation in her shoulders and elbows.
When she came to Caring Medical for Prolotherapy evaluation, the patient had already undergone eight unsuccessful musculoskeletal surgeries and years of physical therapy, with no lasting improvement. Her initial questions were simple:
- Could Prolotherapy prevent the multiple joint dislocations that were occurring on a weekly basis?
- Could Prolotherapy give her enough pain relief so she would not be disabled for the rest of her life and be strong enough to find work and support herself?
In this patient’s case, we were able to have a realistic expectation that Prolotherapy injections would help her. However, it should be noted that if an individual with joint hypermobility or hEDS is treated early in the disease course, where hypermobility is their primary problem, the extent of the Prolotherapy needed will be much less than after the person, as is the case of our patient, has suffered from multiple dislocations and several reconstructive joint surgeries. This patient’s treatment was extensive in helping her reach her treatment goals.
In another case a 55-year-old woman
In this case, we were visited by a 55-year-old retired school teacher and mother of two adult children. The patient told us that she had always known she was “hyperflexible” but had controlled her various joint aches, pains, and subluxations with physiotherapy and chiropractic care.
When she reached the age of about 40 years old, her pain became more unbearable. Contributing to this was a new pain and instability in her left sacroiliac (SI) joint.
After seeing many specialists over the course of several months for her SI pain, including her general practitioner, orthopedists, physiotherapists, and chiropractors, she was left upset and disappointed by her continued pain and lack of improvement. She was having difficulty working, in addition to raising her two children. Chiropractic adjustments helped for a few hours only to have her lower back go out again. She was told by one orthopedist to get a sacroiliac fusion.
As her low back pain intensified and increased, the patient told us, so did the pain in her other joints. She noted increased popping, clicking, and a feeling of looseness throughout her body. She was getting less and less benefit from physical therapy and chiropractic manipulation. Her pain, in fact, worsened. As months progressed into years she had to rely more and more on stronger doses of pain medications in order to function.
The patient was introduced to Prolotherapy injections as a possible treatment option by her then-current physiotherapist who gave her research articles from medical journals that talked about the tightening of joints with Prolotherapy. She began to work with Dr. Fred Cenaiko, a local Canadian physician. (Dr. Cenaiko passed away at age 86 in 2012). It was Dr. Cenaiko who diagnosed her with Ehlers-Danlos Syndrome, Hypermobility Type, and began treating her back and other areas of her body every six weeks with Prolotherapy.
It took her one and a half years of dextrose Prolotherapy treatments to her lower back to experience complete resolution (this is not a typical result) of her SI pain. She reports that her other joints, including her knees, shoulders, and hips healed much more quickly and she only required a couple of treatments to each joint to resolve her pain complaints.
Because various joints of her body would begin to sublux and become painful over time again, she and Dr. Cenaiko realized that receiving Prolotherapy two to three times a year as a “maintenance” treatment would be warranted. This treatment would continue (at the time of her case history being recorded) for the past thirteen years. Her treatments continued with us at Caring Medical upon the retirement of Dr. Cenaiko in 2010.
Summary and contact us. Can we help you?
Questions about our treatments? If you have questions about treatment and how we may be able to help you, please contact us and get help and information from our Caring Medical staff.
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This article was updated July 14, 2022