America has become a country where attention seems to be shown only when somebody calls something “epidemic” or “pandemic”. COVID-19 obviously was a true pandemic, but we are also suffering through multiple epidemics such as violence, obesity, apathy, intolerance, among many others. At the risk of undermining my own case, I would like to add to that list the epidemic of hypermobility.
Hypermobility typically presents in childhood and is often viewed as a superpower. Being able to bend into back handsprings, making a reversed bridge, being able to bend one’s arms into positions others cannot, being able to wrestle one’s way out of a pin; these are just examples of children’s exploits in sporting events that may presage this latent ability. What has not been adequately recognized is the long-term toll that exercising this superpower takes on the bearer. In many cases, symptoms later in life are not even necessarily linked to their hypermobility, which limits clinicians’ ability to generalize, look for other associated conditions, or make the appropriate diagnostic and therapeutic interventions to change what can have a devastating impact on quality of life if left untreated.
From a “medical diagnosis” point of view, hypermobility can manifest from one of seventeen different Ehlers-Danlos variants, from Marfan’s Syndrome, or may not fall neatly into one of these diagnoses, but still manifest itself as a form of joint fragility including frequent sprains or fractures or having some of the neurovascular consequences of hypermobile joints.
In general, nerves do not like to be stretched, and so when a nerve is crossing a joint or series of joints (such as in the cervical spine) and that joint is hypermobile, that nerve may be stretched beyond its ability to function normally. This is called an “apraxia” and when a nerve is subjected to repeated apraxias, can result in a repetitive stress injury. Blood vessels are by nature soft and can either be compressed by kinking (external pressure or bending) or by stretching (think “Chinese finger traps” that when stretched become narrower).
It is also important to understand that hypermobility has been shown to be associated with multiple serious neurological and vascular conditions including autonomic dysfunction, POTS, May-Thurner syndrome, dystonia, Bertolotti’s syndrome, thoracic outlet syndrome, among many others. In addition to that, because the spectrum of etiologies for hypermobility can involve specific vascular anomalies and/or insufficiencies, often called collagen vascular diseases, and as such can have problems with blood vessel walls, skin, or dura (the lining and waterproof sac containing central nervous system and the bodies reserve of spinal fluid). As a result, these can cause vascular problems ranging from aortic aneurysms, intracranial aneurysms, polycystic kidney disease, spinal fluid leaks, and many others.
It is important to understand that not all people/patients with hypermobility have all of these manifestations, but in truth, they all should be screened for them. We are never really sure of what additional issues may surface later on, not to mention being aware of what to look for in the future. Many of these conditions can be treated, or activity modified to prevent worsening, but only if made aware of the problem or potential for problems. Instead, in many cases when people (who become “patients” once they have medical problems) present with symptoms that may be difficult to describe, let alone diagnose, they may be marginalized or undertreated, or simply told “it is all in your head”. Sadly, because the treating practitioner did not take into account the possibility there might be more going on here than meets their untrained eye. It is often said that seeing is believing, but believing is necessary for seeing. If you do not believe in or are aware of a condition, will never recognize even when it is sitting right front of you in your office.
So, the first step in diagnosing these hypermobility related conditions (and hypermobility in general) is being aware that it is a condition and a common one of that. The next step is familiarizing yourself with the mechanism for diagnosing. This starts with taking a good history for hypermobility signs and symptoms and doing a few simple examination maneuvers including the thumb forearm maneuver, the reverse Namaste maneuver (some people cannot do it now but could do it when they were younger or before they injured their shoulders). There is a common EDS test with nine questions and tests but there are also many other “simple” ways to assess hypermobility and/or lax collagen including the ear folding test among others. These tests help raise the awareness and in some cases are better for insurance companies to provide evidence to facilitate the practitioner documenting the hypermobility diagnosis, which will then facilitate getting treatment for a hypermobility relating syndrome.
Ironically, many people do not even realize that these tests are sign of abnormal tissue function, and they may have used them as fun little bar tricks or to amuse their friends. Why do they matter? Because they are a marker for potential problems down the road or at that time and are unappreciated. Many of my patients who suffer from syndromes like POTS or other forms of autonomic dysfunction simply thought “Oh, is this not that normal?”.
“Denial is not just a river in Egypt”, and many patients’ perception of normative values are mistaken. The hypermobility related syndromes can also include conditions that are made more symptomatic by their hypermobility but affect non-hypermobile patients as well. Bertolotti’s syndrome is an example of this where patients with milder forms of congenital anomalies are more likely to be symptomatic if they are hypermobile due to the fact that they have a greater likelihood of making contact with bones that are abnormal and close to each other.
Why is increasing recognition of this important? Because teenage athletes need to be aware that the fully utilization of this superpower in their teen years may lead to intense suffering not too much further on in life. However, it is hard to tell a teenager anything, as any parent can tell you. We also know that brains do not reach neurologic maturity until approximately the age of 25, suggesting that insight and learning still require adult supervision and advice to help manage and mitigate long-term consequences of these conditions.
In other words, it may be up to responsible adults, such as parents and coaches, to recognize that while it might be expedient to encourage the teenager in front of you to maximize their athletic output by using this superpower, it will become kryptonite to them later in life and that may not be in their best long-term interest. See interviews with hypermobile patients describing delayed effects of having used their hypermobility to achieve athletic goals and the cost to them later on. Unfortunately, in these teenage years, it seems that getting into college seems to be the number one goal of parenting, rather than raising well-adjusted children who will succeed later in life.
I am concerned that many parents would face with the choice between teenage athletic excellence, which could be their “hook” into college recruitment processes, and a healthy long-term philosophy, and many parents would still choose to pursue teenage athletic excellence over a lifetime without pain and suffering, and in some cases profound disability.
If the life expectancy is 80 years, the period from 20 to 80 should not be lived with suffering and severe limitations and disability just to further exploit four years in high school, or in some cases, middle school. Our society’s obsession with sports activities stamps partly out of a parental projection and partly out of a social projection of what we consider the ideal body. Teenagers’ youth, innocence and athleticism epitomize these ideals. Sadly, a 50-to-60-year cost is coming due for more and more children of this obsession. This is not to say that they cannot participate and even excel in athletic endeavors. They should be directed towards those that lead to stabilization of the joints rather than stressing the hypermobility component. Sports like swimming (but perhaps not the butterfly), running activities (such as practicing barefoot or natural running styles) and potentially even soccer could be good alternatives.
A lifetime love of athletics is key towards maintaining healthy lifestyles in the future, but throwing sports, swinging sports, and bending sports may, if done regularly to the maximum flexibility limit, ultimately lead to the development of these long-term conditions that may require surgery to prevent permanent consequences. Surgeries include spinal fusions, rib resections, and other vascular or neurological surgeries and have the potential for other limitations or complications as a result of having the surgery.
Therefore, avoiding the syndromes in the first place is the best goal for long-term management of patients with hypermobility. Maintaining good muscle stability around these joints and not exceeding the bodies physiologic limits is probably the best way to prevent long-term disability. It can be done. It requires extensive conversations to be held with these children, their parents, and potentially for their coaches as well. School administrators need to be aware that such supervisory roles need to be taken into account. Each individual coach may think that if they reduce their reliance upon these hypermobile superpower student athletes, that they will not be able to win the championship that they are expected of them. However, if every coach honored the futures of their student athletes universally, then the playing field would be even.
However, this would require an “ideal world” that I fear we do not live in, and as such I suspect that I will continue, as part of my profession, treating patients whose conditions could have been prevented. I am sure that I will hear from many who would extol the values of sports in children’s lives, and I AGREE with them 100%. What I am asking is that the individuals be respected. That the long-term consequences of their participation in sports be acknowledged, and potentially have these children be protected from the long-term consequences of this superpower-like ability.
I am trying to start a conversation here to explain that the children under our supervision, whether they are our own or under the guidance of a coach, mentor or teacher, needs to be informed. I am not advocating for mediocrity and “bubble wrapping” our children. I am, however, advocating that people with true hypermobility should be made aware of that diagnosis early on, especially coaches who are in field where this hypermobility is endemic (baseball pitchers, ballet and gymnastics teachers, swimmers, and wrestlers, just to name a few). In these circumstances, coaches should consider watching for early signs of impairment, injury, or symptoms and either reduce the play time or suggest a change altogether to a different field, one that does not stress the joints that are being overtaxed.
It is our responsibility as adults to look after the children under our supervision and is not enough to look blindly by and say, “everybody else is doing it”, “it is not that serious”, “just for this one season”, or many of the other rationalizations used by those in a position to make a difference but choose not to. After all, I am trying to put myself out of business here. I have published on this subject, and I continue to present academically, give talks on the subject, operate and get paid for these operations on people whose problem could have been prevented. I would be more than happy to find something else to do with my expertise, intellect and imagination than devise the ways of diagnosing and treating these patients. I think that coaches and parents should show a little bit of that same effort to devising new ways to manage their student athletes to avoid having them become my patients.