Every year brings with it the acknowledgment that though we have the knowledge and treatment strategies to help many patients who are suffering, we can often fall short of our expectations.
Last year I encountered a handful of patients with connective tissue disorders that were responsible for their persistent jaw and orofacial pain complaints. I want to share the case studies of two of them with you.
Jill was a college-aged female who presented with what seemed to be the typical TMD list of symptoms: one side dominant, moments of spasm, limited jaw motion and an increase of all symptoms during or following jaw use. A sense of TM joint instability was also described with unilateral joint noise.
Her medical history was a bit unusual and inclusive of unexpected bruising and joint swelling, with no conclusive diagnoses made, despite testing. Also of note was a history of Raynaud’s Syndrome, an irritable bowel, neck pain, fatigue and a general sense of not feeling well. Her reported constant jaw pain and flare-ups without provocation, along with no relief with prior use of anti-inflammatory medication, moist heat/cold applications, jaw exercises, and dietary caution, all suggested something was being missed.
Joan was a 48-year-old female who presented in a way that suggested something was amiss, but what was going on remained unclear for over 18 months. She had one-sided complaints of jaw and facial pain, periods of limited jaw motion and an increase of all symptoms after chewing anything of substance. She also reported a history of an irritable bowel, neck tension for years, fatigue and anxiety.
Joan’s symptoms of jaw fatigue and a sense of jaw muscle weakness were present even when her jaw was at rest. Additionally, her complaints of pain escalation when talking suggested that the nerve endings in her jaw muscles were operating with a low threshold. But the reason why remained unclear. Again, the prior use of anti-inflammatory medications, muscle relaxants, dietary caution, and stretching exercises provided no relief.
In both cases, a regimen of care in my office inclusive of behavior modification efforts to reduce aggravating daytime jaw overuse behaviors, a course of physical therapy, oral appliances, and muscle injections did not help, and at times prompted symptom escalation.
As a result of so many troubling symptoms, which impacted the quality of their lives, both patients continued to explore medical consultation and testing.
Jill was ultimately diagnosed with Ehlers Danlos Syndrome and Joan was diagnosed with a Mixed Connective Tissue Disorder.
Both can predispose to disabling connective tissue pain throughout the body.
Connective Tissue Disorders
Connective tissue disorders compromise collagen and elastin, the parts of the body that connect the structures of the body together. As a result, collagen and elastin become structurally compromised by inflammation.
Once injured by inflammation (in this case due to an overactive autoimmune system) not only does pain become persistent and cause muscles to become hyperactive and subsequently fatigued, but elastin which is the major part of ligaments can become compromised leading to hypermobile joints.
Many patients with these disorders describe sporadic events over the course of their lives when their jaw was stuck open. Unfortunately, by the time they seek care, their longstanding joint hypermobility has led to pain and structural damage that limits jaw motion.
Causes Of Connective Tissue Disorders
The causes of most connective tissue disorders are not known, however, genetic patterns are believed to increase the risk of developing these problems. It is therefore likely that a combination of genetic risks and environmental factors are necessary for the development of connective tissue disorders.
In general, however, these disorders are all characterized as a group by the presence of spontaneous overactivity of the immune system that results in the production and release of extra antibodies into the circulation. The end result is that the immune system is directed against the bodies’ own tissues. Unfortunately, these problems are not curable, and treatment goals are specifically designed to reduce symptoms.
Both Jill and Joan continue to experiment with a variety of medications under the guidance of their medical teams. A combination of steroids and immunosuppressive drugs have been helpful, but at times have led to troublesome side effects. For both, the use of Botox has provided short windows of symptom relief, but this direction of care is no more than a control strategy as opposed to solving the problem.
As a result of these patient scenarios, I have broadened my line of questioning looking to identify clues in the history, exam, and symptom pattern that may suggest that a patient’s TMD signs and symptoms may have a complex origin.
The point is: there are some odd things out there – keep your eyes and ears open.
I welcome your thoughts.