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TRADITIONAL TREATMENTS
Management of JHS and EDS frequently includes education and lifestyle advice, behavior modification, manual therapy, taping and bracing, electrotherapy, exercise prescription, functional rehabilitation and collaborative working with a range of medical, health and fitness professionals.49 Progress is often slow and hampered by physical and emotional setbacks. The functional rehabilitation process is frequently lengthy, with education of the patient and family, sensitively prescribed and monitored physical therapy interventions and facilitation of lifestyle and behavior modifications being the mainstay of the plan.50 Sometimes with a carefully considered management strategy, amelioration of symptoms and independent functional fitness can sometimes be achieved. Currently, there are no randomized controlled studies regarding the effects of existing treatments.51
At present, there is no cure for collagen and connective tissue deficiencies of Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome. The musculoskeletal symptoms derive from a vulnerability to injury resulting from fragile collagenous tissues (tendon, ligament, muscle, bone, cartilage, menisci, labrum, and skin). Conservative treatments such as physiotherapy can help hypermobile patients by the use of mobilizing techniques to restore subluxations; enhance general fitness to offset or reverse the tendency for the body to lose condition by lack of exercise; core and joint stabilizing and proprioception enhancing exercises to decrease pain and prevent further injuries.52 As joint complaints increase with vigorous and repetitive activities, patients with JHS and EDS learn that overtraining and exercises that focus on joint flexibility rather than stabilization increase joint pain and risk of injury, thus, they need to be curbed.53, 54 Patients refrain from activities that cause joints to lock or overextend. If avoidance of these activities is not an acceptable option for patients, physicians often try other approaches including chiropractic or osteopathic manipulation. While these approaches can give some symptomatic relief they offer little as far as long term solutions.55, 56
Pain management is a critical element in the treatment of hypermobility. While physical therapy and exercise may lend some degree of pain relief, individuals with hypermobility often require additional measures to manage joint pain. Patients with hypermobility disorders are often prescribed large doses of pain medication, such as acetaminophen, muscle relaxants, NSAIDs, and antidepressants; over time, stronger medications (including narcotics) and higher doses may be required to deal with the effects of chronic pain. These medications are helpful in management of symptoms that prohibit patients from carrying out certain activities, but they have no effect in treating the underlying pathology of hypermobility, and in some cases they may actually have a negative effect on joint tissues. Non-steroidal anti-inflammatory drugs (NSAIDs) are one class of medications commonly prescribed for joint pain, but can have a combative effect on joint health, due to their role in inhibiting the synthesis of collagen and articular cartilage synthesis.57 This can cause not only weakness in ligaments, but also in cartilage, tendon, and bone cells, contributing to an overall weakening of the joint.58, 59
Another approach used to help with the painful symptoms of hypermobility is splinting and bracing to try to stabilize the joints. This, along with proper physical and occupational therapy to help strengthen muscles and to educate people how to properly use and preserve joints is helpful, but limited. In general, traditional medical intervention is limited to symptomatic therapy. When the symptoms continue to progress and/or are of an emergent or severe nature then surgical intervention is called upon.
Many individuals will have undergone several orthopedic procedures, even prior to diagnosis. It is quite common for the average patient with EDS, or those severely affected with JHS, to undergo multiple musculoskeletal surgeries throughout their lifetime to combat joint injury degeneration and dislocations from hypermobility.60 Common operations include tendon transplant or transfer, capsulorraphy, arthroscopic surgery and arthroplasty.61, 62 The degree of stabilization and pain reduction, overall patient satisfaction, and duration of improvement are quite variable. Unfortunately, the weakness of hyperelastic joint tissue presents a poor healing prognosis, and surgery has proven to be widely unsuccessful in the hypermobile population.63, 64 Grahame and Keer explain that this is because hyperelastic tissues are “less robust and amenable to surgical procedures” than healthy joint tissues.65 When surgery is performed, the patient and physician should cautiously anticipate some improvement but expect less than optimal results.66 While such surgical measures may provide temporary pain relief and stabilize the joint for a short time, using the surgical treatment model for cases of severe generalized hypermobility poses a problem on account of the following:
- The underlying systemic connective tissue deficiency makes surgical outcomes less predictable.
- The condition is systemic and involves multiple joints and body tissues.
- The amount of surgical procedures can be unending because of the systemic nature of the conditions.
- Each subsequent surgical procedure on any given tissue or joint is less successful.
The lack of long-lasting relief in any of these traditional treatments provides a grim prognosis for anyone living with the chronic disabling pain of Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome. The common flaw in each of these traditional treatments is their inability to repair the weakened connective tissues causing the hypermobility. Logically, then the best approach would be the one that directly addresses the root of the disability, weakened connective tissues, such as ligaments and joint capsules, by inducing their repair to stabilize the affected joints.
Tendon transplant – relocation of a whole tendon whereas
a tendon transfer is the relocation of the tendinous insertion
only to stabilize or improve function of a joint.
Capsulorraphy – suture of a tear in a capsule, especially of
a joint capsule to prevent recurring dislocation.
Arthroscopy – examination of a joint, specifically, the inside
structures and then repair or remove damaged structures.
Arthroplasty – surgery to relieve pain and restore range of
motion by realigning or reconstructing a joint.
PROLOTHERAPY
Because surgery carries risks and complications and often does not cure pain symptoms in patients with JHS and EDS, patients are seeking alternatives with the same or greater results. Prolotherapy is one alternative that patients are turning to. Prolotherapy works by initiating a brief inflammatory response, which causes a reparative cascade to generate new collagen and extra cellular matrix giving connective tissue their strength and ability to handle strain and force.67, 68 High-resolution ultrasounds and MRIs have been used to confirm that Prolotherapy does indeed stimulate tissue growth.69, 70 One double-blinded animal study by Dr. Liu showed that Prolotherapy increased ligament mass by 44%, ligament thickness by 27%, and ligament bone junction structure by 28%.71 A human double-blinded study showed joint stabilization by Prolotherapy correlated with patient outcome improvement.72 The doctor that introduced Prolotherapy into mainstream medical practice was George S. Hackett, MD, who described it as follows, “The treatment consists of the injection of a solution within the relaxed ligament and tendon which will stimulate the production of new fibrous tissue and bone cells that will strengthen the weld of fibrous tissue and bone to stabilize the articulation (where the bone and ligament meet) and permanently eliminate the disability.”73 He published his results in peer-reviewed, mainstream medical journals and wrote a book summarizing his results and the technique entitled Ligament and Tendon Relaxation Treatment by Prolotherapy.74-76 He showed via animal studies that Prolotherapy induced the proliferation of new ligament tissue that had the effect of stabilizing joints, thereby eliminating the disability associated with ligament laxity.77-79 Prolotherapy has a long history of success treating ligament injuries, including patients with joint hypermobility.80, 81 Studies on Prolotherapy have shown that it eliminates chronic pain even in those patients who have been told by their medical doctor(s) that surgery was the only treatment option for their pain.82-85 Some of the rationale for using Prolotherapy for patients with EDS and JHS are that it has a high safety record, is comprehensive (all or most joints can be treated at each visit), is an outpatient procedure, is cost effective (compared to surgery), pain relief is often quick, and it provides joint stabilization. Perhaps its greatest asset is the fact that this one treatment modality can handle most of the painful musculoskeletal conditions that occur in individuals with EDS and JHS. (See Figure 7.)
| Figure 7. Painful musculoskeletal conditions that can occur in EDS and JHS which may be effectively treated with Prolotherapy. | |||
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Prolotherapy could also contribute to the treatment of hypermobility disorders by preventing the development of precocious osteoarthritis. It has long been known that individuals with JHS and EDS suffer with premature osteoarthritis in various joints and the amount of degeneration correlates with the extent of the individuals hypermobility.86-88 Dr. P. Brighton who developed the criteria to determine joint hypermobility (for whom the Brighton criteria is named) found that when individuals had Ehlers-Danlos Syndrome and a Beighton score of at least 4, 100% of them developed osteoarthritis by the age of 40.88 The combination of extreme hypermobility and repeated injury is presumed to be what leads to the early osteoarthritis. This is most likely the reason that the hypermobility type of Ehlers-Danlos Syndrome is the most debilitating form with respect to musculoskeletal function.89
TWENTY YEARS EXPERIENCE TREATING JOINT HYPERMOBILITY SYNDROME AND EHLERS-DANLOS SYNDROME WITH PROLOTHERAPY
I (R.H.) joined the practice Gustav A. Hemwall, MD in 1993, at which time Dr. Hemwall had already been performing Prolotherapy for nearly 40 years after learning the technique from Dr. George Hackett at his office in Canton, Ohio in the mid 1950s. I can remember the point Dr. Hemwall made to me in April of 1992, while first observing in his office. He said, “Most chronic pain is from ligament laxity.” When I finished my first draft of Prolo Your Pain Away! there was a small section on both benign congenital hypermobility (also termed Joint Hypermobility Syndrome) and Ehlers-Danlos Syndrome. The reason for including them was obvious. Prolotherapy caused a significant improvement in the quality of life of individuals who had a genetic connective tissue disorder causing systemic hypermobility, the very condition (though extreme) for which Hackett-Hemwall Prolotherapy was designed to treat. One patient in those early years of working with Dr. Hemwall comes to mind.
JM was a young woman in her 30’s, who had the hypermobility type of EDS, and was already confined to a wheelchair when she originally consulted with Dr. Hemwall. By the time I first saw her, she was walking, running and leading a normal and fulfilling life. Initially, she required intensive Prolotherapy for about 18 months, then twice a year for a couple of years. But after that time she was done with treatment. Prolotherapy had stabilized the joints enough, to where now it has been over 10 years since she required treatment. The intensive Prolotherapy involved treating most of the joints in her body, and she was treated over the course of two days each time. The first day, she would receive treatment to half of her joints, and the second day the other half would be treated. This is an extreme case, but mentioned to show the extent of the possibilities with Prolotherapy.
REPRESENTATIVE CASE HISTORY
JD presented to Caring Medical with a long history of severe joint pain and complaints of frequent dislocations of both elbows, knees, and shoulders, as well as subluxations throughout the spine and the sacroiliac (SI) joints. She had first begun experiencing joint pain at the age of ten, and her symptoms escalated during her teen years as she became an avid track and field athlete. The first incident of joint dislocation occurred when her knee completely gave out during a track meet. Following this event, JD underwent multiple knee reconstructive surgeries which provided short term relief, but she continued to experience pain and instability in that knee. At the age of 21, JD began experiencing cardiovascular symptoms including tachycardia and feeling faint. When she consulted a cardiologist, JD learned that she suffered from Ehlers-Danlos Syndrome.
Over the course of her adult years, JD’s condition began to affect other joints in her body including her shoulders, elbows, and sacroiliac joint. Simple activities such as walking, or even a strong wind, JD said, could cause her joints to dislocate on a daily basis. In addition to being unable to walk, she was unable to use crutches or a cane because these would cause dislocation in her shoulders and elbows. Like many others who suffer from Ehlers-Danlos Syndrome, JD’s condition had impeded her education and prohibited her from being able to work for extended periods at a time. When she came to Caring Medical for evaluation, JD had already undergone eight unsuccessful musculoskeletal surgeries and years of physical therapy, with no lasting improvement. Unless she found an effective treatment for her condition, she would be disabled for the rest of her life. 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 the rest of her life and be strong enough to find work and support herself? The answer, based on my experience (R.H.) with treating Joint Hypermobility Syndrome and Ehlers-Danlos Syndrome to both questions is a solid “yes.” However, it should be noted that if an individual with JHS or EDS is treated early in the disease course, where hypermobility is their primary problem, the extent of the Hackett-Hemwall Prolotherapy needed will be much less than after the person has suffered from multiple dislocations and several reconstructive joint surgeries.



