New Cervical Spine Instability Publication: The Authors Explain Their Findings

For Chronic Pain Partners Newsletter, writer Christie Cox speaks with two pioneers in the Ehlers-Danlos community Leslie Russek and Susan Chalela, about their recent publication on upper cervical instability (UCI). Leslie Russek is a DPT, PhD, and Professor Emeritus of Physical Therapy at Clarkson University and an Orthopedic Certified Specialist Physical Therapist with over 30 years of clinical practice. Susan Chalela is an MPT and the founder of Chalela Physical Therapy Institute for EDS & CCI/Cervical Instabilities in Charleston, South Carolina. She is passionate about helping hypermobile patients and works closely with Dr. Sunil Patel from MUSC on some of his patients who come with CCI and EDS-related spinal complications. 

 

Christie Cox: 

Both ladies have HSD/hEDS themselves, so they are fellow zebras. They get it, and they understand how our needs are different. Thank you both for being here today, and I’m thrilled to share the exciting news you put out in your recent paper. Leslie, do you want to summarize the findings and why you used a different terminology: UCI (upper cervical spine instability) instead of CCI (craniocervical instability)?

Leslie Russek:

First, I want to clarify it’s not a research paper. We didn’t collect data for this, but it is an expert consensus paper. There isn’t research on upper cervical instability in patients with hypermobility. What incited me to pull this team together is the fact that we have expert clinicians like Susan, who have a tremendous amount of experience working with this population. And even though we don’t have research, there’s a lot of knowledge. So, we wanted to pool that knowledge and share it with people who didn’t have the opportunity to learn directly. So, it’s an expert consensus from an international team, so I’m pretty confident that this is the best knowledge we have in the world on this particular topic at this time. And hopefully, it’ll inspire some research, and in another ten years, we’ll be able to talk about an actual research paper. The article provides providers and patients with upper cervical instability and hypermobility spectrum disorders/hypermobile Ehlers-Danlos syndrome with a framework for screening and managing upper cervical instability. We used the term upper cervical instability because we felt that that was more inclusive of both CCI, which is technically craniocervical instability and involves the skull and the first vertebra, and AAI, or atlantoaxial instability, which involves the first and second cervical vertebrae.

Susan Chalela:

There are some differences between the location of the instability, but in terms of management, there are a lot of similarities. We grouped them to include both CCI and AAI. Research suggests that mild UCI can be a common condition. Up to 66% of people with hypermobility may have mild symptoms of upper cervical instability. However, severe upper cervical instability is not very common. Only 5% of the population with hypermobility develop severe symptoms, which can lead to significant disability. No published guidelines or research regarding the non-surgical management of UCI exists. Some papers were published on surgical management, but nothing for conservative or pre-surgical management or to avoid surgery altogether. Our article combined the knowledge of hypermobility experts from three continents: the US, the UK, and Australia. We outline a basic screening method for UCI based on the patient’s symptoms and history. 

We had a three-part criterion: first, symptoms of UCI, which are divided into musculoskeletal and neurological; second, symptoms are altered by neck movement or position. This was to help us distinguish between symptoms due to UCI and symptoms that could be due to other conditions common in hypermobility, like Chiari malformation or mast cell activation. And then, finally that the symptoms are irritable. Pain from musculoskeletal UCI comes from muscles, joints, and spinal nerves. Patients with neurological UCI, where neurological symptoms occur due to stress on the spinal cord, brain, and cranial nerves, are more challenging to manage. Patients can have both, and they can switch from one to the other. They may have primarily musculoskeletal symptoms and then a flare of more neurological. The article recommends screening for yellow flags that can interfere with management ones. So yellow flags are psychosocial issues, and we know that psychosocial issues can make managing any kind of medical condition more challenging. But it’s important to recognize that yellow flags can amplify the disability and make it harder to treat. But they don’t diminish the possibility of mechanical instability or the physical problems underlying it.

The article also recommends screening for red flags that can suggest either severe upper cervical instability or other conditions that should be assessed and ruled out or referred to an expert for assessment. Then we recommended using information about irritability and severity from the history and symptoms to identify what physical examination tests are likely safe and not likely to cause a significant flare. Previous decision-making and physical therapy models do not use the symptoms in history to decide what tests are safe to do. But that’s incredibly important because these patients are so easily irritated that if you do just simple things like neck range of motion, you can make them worse. We felt it was crucial to identify which tests we shouldn’t be doing. We grouped the tests into three tiers, ranging from least likely to be provocative to most likely to be provocative. Those are most likely tests you wouldn’t do on somebody whose condition is irritable. Ultimately, you would decide how irritable the condition is based on subjective and physical exams.

The article suggested three tiers of management, with the most cautious interventions for those with the most irritable UCI and then more aggressive interventions for patients with milder UCI. And, of course, people move on that spectrum. We hope patients doing well go from having an irritable UCI to a milder one. So hopefully, patients will progress through those different levels. And then the paper includes three case studies, one with high, moderate, and one with low irritability, demonstrating how we use the model and how we might implement a patient care plan. It’s an expert consensus, but it provides us with many hypotheses that research can test, and it has raised awareness for UCI in this population. We’re super excited that the articles have had over 21,000 views so far.

Cox: 

We’re very grateful to all of you who put together this information. How did this large international group of hypermobility experts come together to create this body of knowledge?

Russek:

It started almost three years ago. A team of doctors on the West Coast wanted to create a protocol for using cervical traction to treat CCI. I was invited. Then I asked Susan and the others who participated in the paper. We were all appalled because traction (stretching the neck to elongate it) may decrease your symptoms (temporarily), but it may also make your instability worse. As PTs, we felt we had other things to discuss beyond that. 

EDS is not well-known in the US, and the US is a desert for knowledge and research compared to places like London, Europe, and Australia, where they have these specialty clinics with researchers, doctors, and providers who all work together. We don’t have that network in the United States. I wanted to pull this network together in the US. The US team met a few times, but it was essential that we needed to do something with that information and share it. So I contacted Alan Hakim, the educational director for the Ehlers-Danlos Society, and asked if he thought that could be publishable. And he did. He suggested that we invite an international team. And so we invited several people who are affiliated with the Society. Then we spent about a year and a half talking back and forth. We had Zoom meetings. We had online asynchronous meetings. We had a couple of surveys to rank symptoms and signs and determine how to put the information together.

Cox:

As you mentioned, this UCI information is making waves in the patient community. And you have had the opportunity for us to make it publicly available, free access, which is sometimes an issue. So when information comes out, we might not be able to access it, so we appreciate that as well.

Russek:

Yes, and a shout out to the Ehlers-Danlos Society because they’re the ones who funded the article to allow it to be open access, and Dr. Hakim facilitated that. 

Cox:

Susan, what was the most exhilarating or maybe the most exhausting part of doing this big multiyear project?

Chalela:

I think just finding the time to get together. Leslie handled this very well because she was the ringleader in all of this. She would take one section at a time, and we’d concentrate on that. We discuss that. And sometimes, we couldn’t all be together because of the time zones, but we would discuss it within our group. I’m so clinically oriented. I see patients five days a week, and finding times for meetings is hard for me. But I did it at night, and I did it on the weekends.

Cox:

Now it’s out. And this is just the beginning of what needs to be done research-wise and clinical-wise to teach other PTs how to do this. Because there’s still this whole perception of traditional physical therapy and how to treat these patients, but when you get to the neurological aspects, especially at the upper cervical, the brain stem aspect, it gets complicated. Traditional physical therapy just tends to make these symptoms and these problems worse. We have to be very careful. So it’s crucial for us to get this information out there.

Russek:

Yes. PTs in the US learn very little about HSD/hEDS in school, the PT establishment hasn’t supported continuing education, and the US journals are reluctant to publish hypermobility research. The excuse is that HSD/hEDS is too rare a condition to justify teaching or research. Europe, the UK, and Australia are way ahead of the US in both research and clinician training. So, unfortunately, patients in the US are likely to see PTs who don’t know much about hypermobility, no less UCI in hypermobility. Zebras aren’t horses, and things often don’t go well when you treat a zebra like a horse. 

Chalela:

The danger is that someone can do traction to a patient, and the patient could be telling you they’re feeling great. So you think you could do a little more because they’re feeling better. But then the aftereffect of relaxing or releasing the backup stabilizers when the primary stabilizers are not doing their job is that the neck becomes more unstable. Traction unweights the pressure on the nervous system and the discs, so of course, we’re going to feel good temporarily, but it’s the aftereffects that create more pain. In turn, it does more damage. We’re seeing this happen. That’s why it is so important for PTs to know what not to do, in both testing and treatment. 

Cox:

That’s why this paper is so important. And as I mentioned, a really valuable piece to me as a patient is your patient guide, which we are sharing HERE. Let’s dive into the patient guide. Leslie, do you want to start by explaining the difference that you note between instability and hypermobility?

Russek:

We may be born hypermobile, but instability comes and goes. Hypermobility means too much range of motion. Instability, on the other hand, means you’re unable to control that motion, and the muscles and the nerves that go to those muscles cannot maintain stability, especially in mid-range or neutral ranges. We may be born hypermobile, but we have some control over instability. And that’s where physical therapy is powerful in retraining the muscles and the neuromuscular control, the proprioception, or body awareness. We can’t stop being hypermobile, but there is the potential for physical therapy to make us less unstable. And if we have a flare, it’s a flare of instability, but again, we may be able to control it and possibly don’t need surgery.

Cox:

So the question becomes: Can physical therapy get me through this, or how to know when it’s the right time for me to have surgery? Any insights you guys want to share?

Chalela:

We always start with a patient’s history, a physical exam, and a discussion of their symptoms. Then we look at your function and your pain levels. Some patients came to me with symptoms and imaging that showed severe cervical instability but were working full-time jobs. They were struggling, but they were working. So I’m always looking at irritability status in combination with function and the patient’s wishes because many of them don’t want to be fused. And so, what can we do to support them? How can we help them manage this to live a more functional life?

Then we have patients who have been told they have mild instability, yet they could be housebound and in bed a lot and feel that they can’t work and are not functioning very well. That’s the patient that needs physical therapy to get their function back. If they’re going through this process but remain symptomatic, and we can’t get that under control, no matter how we try, they’re the ones that tend to choose to go the fusion route. Sometimes, it’s just a matter of releasing a tethered cord because, more than likely, many of these patients have a tight spinal cord that causes many other irritable neurological symptoms. If the patient’s tethered cord is being released, we find about a third of them see improvement in their UCI symptoms. Yes, it’s only a third, and it’s just clinical evidence, but we hear that, and sometimes that’s worth that risk.

I spend a lot of time with the patient, helping them talk and work through all of this. And ultimately, they decide. It’s essential to have good guidance from your medical team and therapists, so you know you’ve tried everything you could do before you get fused. Keep in mind with a fusion, you’re putting metal in the body, screws, plates, and rods. It’s not a quick fix. I get those patients that would rather be fused and then return and do physical therapy afterward. I guide these patients to try PT before and gather as much knowledge and information on the management side before seeing what they can tolerate to get themselves to the highest capacity level in managing this. And then, if you must undergo surgery and be fused, then coming out of that, you already have all this knowledge. Being as strong as possible going into surgery is most important because then, coming out, you’re going to start back in the beginning again, but you’re going to flourish faster, and you’re going to manage better overall. 

Cox:

I agree. In my personal experience, it wasn’t the pain that made me choose surgery. It was the neurological issues where I lost my ability to walk a straight line or complete a sentence.

Your paper goes through a lot of both the musculoskeletal and neurological symptoms to look for. What do you mean by mechanically irritable? Is this when you aggravate this area of your body with specific activities and cause a flare?

Chalela:

Mechanically irritability was crucial because so many of the symptoms of UCI overlap with other problems that hypermobile people have: dysautonomia, mast cell activation, brain fog, and poor coordination of their joints. It was essential to determine that it’s the upper cervical spine causing the symptoms, and some movements can make those worse; bending forward, things that put stress on the neck would aggravate it. There has to be something mechanically associated with the neck that worsens it. We divided people into three levels of irritability: high irritability, moderate, and low irritability, based on their symptoms and their history so that we could decide which tests were going to be safe for them. Having these three levels of irritability also allows people to see where they are on the spectrum and if they’re getting better or worse.

As physical therapists, we don’t diagnose, but we look at the presentation of the person overall, and we look at all of the comorbidities that come with EDS. All of those affect the patient functionally and psychosocially. Instead of looking at mild, moderate, and severe upper cervical instability, let’s look at it as irritability status. On a physical therapy level, we use what affects your function – your irritability status, your symptoms, and/or your neurological symptoms. We’re focused on better supporting the patient with bracing, wheelchair, etc., to get them more mobile, upright, and functional, but again, keep them as asymptomatic as possible.

My whole approach is you’ve got to look at the foundation first. Traditional physical therapy has you look at the upper area when you have neck pain and problems. So they always go right to the neck and treat that, or maybe the scapula, thoracic spine, shoulder, and neck together. But for me, what I found most successful was looking at the foundation of this machine, this human body. If you’re hypermobile and we’ve got 360 joints in the body and all this movement, how will we gain control over that? What we do know is we feel through our body’s proprioception; we feel where we are in space. And being hypermobile, we do that very poorly. We’re very clumsy. We tend to be off-balance, more so than other people. So how can we get control of the dynamic or the movement mechanics, right? Even though the base of the spine starts at the pelvis, that’s the critical nervous system, we still have to look at what the feet and ankles do because feet and ankles have a lot of bones and a lot of movable parts. And if we’re not supporting these collapsing arches because of all that hypermobility in those bones and joints, then the excess or abnormal movement is going to start at the foundation, and you’re only going to get more and more movement up the chain. So to stabilize, you have to first look at the foundation, build more stability, and then look at each component above until you get to the neck.

We’ve got to get a hold of our body statically first, and then work on alignment and then build that more functional, healthy movement system. In turn, that decreases irritability. Typically, for most patients, we can get them to bring their irritability status down, and we give them the tools, so they know what to do when they have a flare.

Cox:

I know that PTs don’t diagnose, but what physical exams or tests can you do to help aid a patient trying to figure this out? 

Chalela:

When I first see a patient, the most important is looking at the history and symptoms. I listen to my patient. Listening to their pain level, how they function, do they have headaches. I want to hear that their bowel and bladder system is functioning normally. But a lot of times, we’re hearing in our more severe patients that it’s not. A lot of these patients have a hard time concentrating; they have tinnitus and buzzing in the ears. I am listening for signs of double vision. You want to hear every little detail because even if you don’t think something’s related, a lot of times, we can circle it back to it being related in one way or another. Are they sitting around the house and not doing much, or are they in bed most of the day, or are they very active with their job and just pushing through? Because if you’re hearing a red flag, if you hear that they’re very irritable, you got to be very cautious.

Then we can do neurological testing to look at cranial nerves; we can look for a Babinski or Hoffman. If they’re positive for that, then back off on all the hands-on manual treatment because this person will be very neurologically symptomatic. If they’re walking very uncontrolled with poor motor control or balance, I will rate them as irritable, even though they’re not telling me their pain is severe, but just noticing the neurological signs. You can do some light palpation to feel how their muscles are reacting and what they’re doing. Look for spasms; look for the tone of their muscles in general. That’s fairly safe. If they haven’t had any experience with a neck brace collar, we’ll put them in a collar trial while they’re sitting upright. Some are entirely relieved; others are more irritated by the collar.

We can also look at the neck muscles and posture. These days, we’ve got computers and cell phones. All these kids are looking down at iPads all day. That forward head position over time accumulates pressure on the upper spinal cord brainstem area. Right away, I suggest using prism glasses, lazy readers, iPad stands, and cell phone stands to bring it all up to eye level so the patient isn’t looking down.

Cox:

I know from the neurosurgical perspective the testing to confirm these diagnoses is the upright MRI with flexion and extension. And then, they may use a rotational CT scan. In combination with the PT data on levels of function, this might help determine if and when you might need surgery. If you get diagnosed with CCI or AAI, and you want to do your best to avoid surgery, the next step is to work with a physical therapist, obviously one who knows about hypermobility. Do you have any suggestions on how to find this kind of physical therapist because you guys are also rare? Or what could we use from your paper to educate our existing physical therapists? 

Russek:

I always advise people to check the Healthcare Providers directory on the Ehlers-Danlos Society website, where there’s a list of doctors and therapists who are EDS knowledgeable. It’s not a comprehensive list; if somebody is not on the list doesn’t mean they’re not good. But that’s a really good starting place. And often, if you can find one person in your area who’s knowledgeable, they can connect you with other people who are knowledgeable. 

Chalela:

With my telehealth offering, I treat patients all over. The problem is everybody doesn’t have access to be able to get on an airplane, especially if they have a severe case and they’re severely irritated. They’re not going to be able to travel like that. And it is challenging to find somebody knowledgeable in this on the neurosurgery side. You want to see and interview your potential providers to ensure they understand EDS and educate themselves. I recommend that patients bring this article to their physical therapist and ask if they are familiar with it. Patients are being proactive about helping to educate therapists that might not be as knowledgeable, and that’s great!

Cox:

The paper is fantastic in terms of going through the symptoms at those different levels and the yellow flags and red flags to watch out for. It is very clear when to be cautious and when not to do things like, for example, chin tucks that might or might not be something that irritates you. I highly recommend anybody hypermobile go and read both the paper and the patient guide. In the end, I wanted to ask you if you had any other kind of self-care, lifestyle tips, or any tools or resources that you would recommend to people that are in the middle of trying to figure this out.

Russek:

I want to emphasize just how important it is for patients to take care of themselves. Those little things like slumping or reading your phone can accumulate. Imagine a golf ball on a golf tee. You’ve got this golf ball on a golf tee, and if you tip it forward, the golf ball is going to slide off, and that twelve-pound pumpkin [pointing to her head] is going to slide off. Patients must be doing things to take care of themselves and not just count on the therapist or the surgeon to fix them. Because if you’re hypermobile, you must learn how to take care of your body and protect it.

Chalela:

I tell all my patients this is the management for a lifetime. Start with one thing at a time. We can go as slow as you want, and you need to get a hold of each one of those components to be able to progress. I tell the patients, I am not here to fix you. I’m here to teach you what you need to do to be able to better manage your alignment, your posture, protect your neurovascular system, and protect your discs and ligaments. As you age, tissues demonstrate wear and tear anyway, even if you are healthy. Now think of the acceleration with the fragility of what we have to deal with in our bodies. It’s even more critical for us to be more aware. We have to be more aware of our alignment. We have to be more aware of correcting ourselves. It’s not easy. It is hard work, and the patients have to put the work into it. Hopefully, soon, we will have a PT module training program to help therapists better help these patients. We can all benefit from that.

Cox: 

The real resource is in your paper and the short patient guide. Anybody that’s a patient or a PT interested in learning about it, access those resources here: UCI in hEDS/HSD Patients (downloadable PDF).

Thank you both for being with me today. 

 

Christie Cox

April 2023