Ehlers Danlos syndrome hypermobility type – Hypermobility Spectrum Disorder in children
Ross Hauser, MD
In our various articles on Ehlers-Danlos syndrome hypermobility (hEDS) we typically start out by telling of a patient’s experience in the health care community before they have reached out to us here at Caring Medical. It is particularly important that we do this because we understand that the parents of children who have strange and mysterious symptoms and disorders understand that they are not alone. Some parents, unfortunately, feel like the problem that their children suffer from is their (the parent’s) fault.
This article will offer an understanding, a possible explanation of the problem of hEDS, and a possible path of treatment.
Discussion points of this article:
- We never heard of Ehlers Danlos Syndrome. Finally, after six years of looking for answers, bouncing from doctor to doctor, someone said Ehlers Danlos Syndrome.
- My kid has always been hypermobile.
- It started for me in childhood and I have had so many procedures since.
- A brief discussion on children’s anxiety.
- “If a child HAS anxiety issues and behavioral issues, someone should ask about joint pain and check for “stretchy skin.”
- Digestive disorders are among many symptoms including depression.
- Can’t keep up with friends is depressing.
- Many are told there are no treatments, only prevention of worsening problems.
- The path of treatment EDS hypermobility type increasingly recognized in pediatrics – changing outlooks.
- Treatments that are not helping because doctors do not clearly understand the symptoms.
- Pediatric postural orthostatic tachycardia syndrome – one-fifth of patients had EDS and over one-third of patients had hypermobility spectrum disorder.
- I just want to go back to school.
- When physical therapy is not helping.
- Who are the patients who would not benefit from physical therapy? Patients with loose ligaments and tendons.
- Joint stabilization rather than joint flexibility – Prolotherapy treatments.
- Connecting ligament weakness with chronic pain from joint instability in Hypermobile Ehlers Danlos Syndrome.
- Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).
We never heard of Ehlers Danlos Syndrome. Finally, after six years of looking for answers, bouncing from doctor to doctor, someone said Ehlers Danlos Syndrome.
Below is a story: It is a summary because the original story was very long and very complex. I added some comments.
- Our daughter is a teenager. For the last six years, she has suffered from one chronic illness to the next. She has a long story. For the first time, a doctor, our new rheumatologist suggested that all the health challenges our daughter was facing could be Ehlers Danlos Syndrome. We never heard of Ehlers Danlos Syndrome.
This is very typical. In many cases, parents will learn of Ehlers Danlos Syndrome in one of two ways.
- A doctor will finally recognize the problem and order genetic testing to determine the type of Ehlers Danlos Syndrome. I will assume at this point since you found this article, that you already have a good explanation of what Ehlers Danlos Syndrome is and we can leave this explanation until later in this article.
- A child will have a sports-related injury or accident and the doctors treating the injury realized that the child is hypermobile because this is yet “another,” dislocation or that the injury the child suffered from is following an unusual healing pattern.
The story continues:
- Our daughter has been suffering from a myriad of seemingly unrelated symptoms. She has been to so many doctors and specialists and each wanted to focus on treating the one symptom but none of them could even explain to us why the symptom they wanted to treat developed. We started chasing symptoms, not an understanding of what was causing the symptoms. The rheumatologist said Ehlers Danlos Syndrome can cause all the problems.
This person then related that their daughter suffers from the many symptoms we see in hEDS patients:
- Altered gut or intestinal problems: In many people, this can lead to nausea, stomach pain, diarrhea, and digestive disorders including GERD and acid reflux disorders.
- Concentration difficulties include brain fog.
- Even in younger patients such as children and adolescents, there can be a problem of shortness of breath and heart palpitations. Symptoms of Slipping Rib Syndrome can occur also cause chest pain.
- Vision problems that include floating shapes or shadows
These symptoms do not usually describe the complexity of cases in their full details as I am sure if your child or adolescent is suffering from these problems they do not reflect yours. One thing to point out is that many people with stories like this also tell about how before the symptoms started, their child was “double-jointed,” could bend joints in very extreme ways.
My kid has always been hypermobile
A diagnosis of Ehlers Danlos Syndrome can be a revelation to some parents. It just makes sense. Especially the parents of children who have always been “hypermobile” and “double-jointed.”
Here is an example of parents who understood hypermobility, but did not understand what was causing it. Nor could their doctors.
My daughter sometimes has extreme joint pain. There is nothing, we are told, we can do for her except give her painkillers and make her comfortable. We got her stuffed toys and big pillows that she could wrap herself around and hug when her pain got worse. When her pain gets really bad, she hugs me.
She has had this pain for a few years. We did not know what was wrong with her, the doctors did not know what was wrong with her. The one thing we all knew was that she could hyperextend her elbows and knees and hands almost backward. Then her shoulder and hip started popping out on occasion but she got good at getting them right back in.
One doctor told us the reason she has joint pain and frequent dislocations were because of hypermobility. We knew she was hypermobile, my kid has always been hypermobile, what we did not know was what we could do for her. We were given no treatments that seemed to address the problem head-on. The best treatment we got was shoe inserts and knee braces to help her stand straight and walk. Her knees and ankles could give way. She has fallen over. She has suffered some minor fractures. She has suffered from several dislocations. She gets a lot of intense physical therapy that seems to help but sometimes it feels like one step forward, two steps back. The worst days are the days she has to use a wheelchair to get around.
The parents we talk to have had an often confusing and frustrating medical journey story to tell us. Probably just like yours. Certainly just like yours. Some parents will now recognize that this is a hereditary disorder. They themselves had many of these same symptoms as youngsters, and no one knew what could help at that time either. Some of these parents thought they had grown out of it, or that their symptoms lessened because more adult muscle made them stronger. But when we ask the parents what are their own complaints? The response back is usually joint pain, sometimes their joints pop in and out of place, and a lot of fatigue.
It started for me in childhood and I have had so many procedures since
Someone will reach out to us with a long story that reaches back into their childhood. They were diagnosed with Hypermobility EDS and cervical spine instability. They will report a long history of chronic pain and note particularly bad pain following trauma to the neck and lower back. Like many people, they were diagnosed with Hypermobility EDS in high school but not treated. Only that they should make their future doctors aware of the diagnosis.
The treatments they have had include:
- Trigeminal neuralgia managed with acetazolamide.
- Botox for migraines.
- Radiofrequency ablation in the cervical spine for headaches.
- Physical therapy which they say is harder to do because of increased pain with treatment.
- Chronic Fatigue with the assumption that they also suffer from depression and anxiety.
A brief discussion on children’s anxiety
Many people reach out to our office after their children have been given a diagnosis of either Generalized joint hypermobility, Hypermobility Spectrum Disorder, or Ehlers-Danlos syndrome hypermobility type. They reach out to us because they are no longer looking for explanations as to what these disorders are, or even what makes them different. They are looking for treatment options. I will discuss below, but first. Before we discuss treatments for Joint hypermobility syndrome, Ehlers-Danlos syndrome hypermobility, and Hypermobility Spectrum Disorder, let’s address psychosocial support or an understanding that this physical problem is causing emotional problems in children.
Doctors at the Department of Medical Neuroscience, Brighton and Sussex Medical School, University of Sussex published an October 2021 study in the World Journal of Psychiatry (1) in which they outlined the psychiatric burden of joint hypermobility, specifically in younger patients. Here is a brief summary from this paper:
“There is a rapidly growing body of evidence showing a curious excess of psychiatric burden among individuals with joint hypermobility. Common constitutional variants of connective tissue often present as joint hypermobility, reflecting increased laxity of ligaments and extended movement of joints beyond typically ‘normal’ limits (otherwise described as joint hyperlaxity, being double-jointed).
Hypermobility is a descriptive term and might exist as an asymptomatic and isolated feature. However, neuropsychiatric symptoms are among extra-articular manifestations that frequently accompany more widespread musculoskeletal symptoms that typically co-occur with hypermobility. In these cases, hypermobility appears as one feature of a multi-systemic disorder.
Joint hypermobility is frequently under-recognized and has a significant impact on quality of life across all age groups. Anxiety symptoms and disorders have a well-established link with hypermobility, yet growing evidence also now points towards associations across psychiatric diagnoses, notably with affective illnesses, increasingly with neurodevelopmental disorders, and with stress-sensitive medical conditions, including fibromyalgia, myelo-encephalomyelitis/chronic fatigue syndrome (ME/CFS) and irritable bowel syndrome (IBS). Awareness of these relationships is important to enhance understanding of early risk factors to allow screening and timely intervention for vulnerable individuals.”
A 2019 study (2) led by Andrea Bulbena-Cabre, MD, Ph.D., MSc a research fellow at the Icahn School of Medicine at Mount Sinai, New York evaluated children suffering from Joint hypermobility syndrome for anxiety disorders. Previously there had been no research on this subject.
- Her research team looked at a sample of 160 children (74 girls and 86 boys) ranging from ages 5 to 17. The prevalence of joint hypermobility syndrome in this sample was 22%, and this was significantly higher in girls (31%) than in boys (14%).
- The Joint hypermobility syndrome group tested significantly higher in the Children Manifested Anxiety Scale test, the Children Manifested Anxiety Scale Physiological anxiety test and the Child Behavior Checklist somatic complaints test. The Joint hypermobility syndrome group also scored higher in some behavioral measures.
CONCLUSION: Children with Joint hypermobility syndrome have a higher frequency of anxiety disorders and higher intensity of physiological anxiety, somatic complaints, and therefore, joint hypermobility syndrome might be used as a marker for this anxiety phenotype in youngsters.
“If a child HAS anxiety issues and behavioral issues, someone should ask about joint pain and check for “stretchy skin.”
In other words, if a child HAS anxiety issues and behavioral issues, someone should ask about joint pain and check for “stretchy skin.” These problems may underlie Joint Hypermobility Syndrome.
While this is the first study to seek a connection of joint hypermobility and anxiety issues in children, a 2011 study in the International Journal of Psychiatry in Medicine (3) from the University Autonomous of Barcelona found that Joint Hypermobility Syndrome is a frequent condition among young people (university-age students) evaluated and is associated with psychological distress and higher levels of somatosensory amplification (heightened sense of pain and pain awareness).
Just because your child has digestive disorders does not mean they have Ehlers-Danlos Syndrome. However, if they have digestive disorders among many symptoms including depression, then Ehlers-Danlos syndrome could be suspect
Doctors are looking for clues into a more accurate diagnosis of joint hypermobility / Ehlers-Danlos syndrome and how to understand the symptoms children present in the doctor’s office. Building on the above study from 2019, researchers publishing in The Journal of Pediatrics (4) tested the suggestion that children with joint hypermobility have a greater risk of irritable bowel syndrome and functional abdominal pain than “healthy,” or we should say, non-joint hypermobility children. Further, these digestive and abdominal problems are caused by the unique anxieties that joint hypermobile children suffer from.
Here are the learning points:
- This study tested the hypothesis that the prevalence of joint hypermobility is greater in children with irritable bowel syndrome and functional abdominal pain than in healthy control children and is related to gastrointestinal symptoms and psychosocial distress (anxiety, depression, and somatization – that is suffering from a lot of symptoms that no one knows why you have them).
The result of 219 children age 7 to 12 tested:
- The prevalence of joint hypermobility does not differ among children with irritable bowel syndrome, functional abdominal pain, or healthy control children. The presence or severity of joint hypermobility does not correlate with abdominal pain or stooling characteristics. Somatization and depression by child reports appear to have a relationship with joint hypermobility.
In other words, the “healthy children,” and the joint hypermobility children have the same anxieties when it comes to abdominal distress. But the joint hypermobility children have more depression because they have more symptoms.
Can’t keep up with friends is depressing
An August 2019 study in the Journal of Child Health Care (5) reinforced the message among doctors that anxiety and depression are symptoms of Hypermobile Ehlers-Danlos syndrome.
Thirty-four children with Hypermobile Ehlers-Danlos Syndrome reported the hardest thing about having Hypermobile Ehlers-Danlos Syndrome, their pain, fatigue, physical symptoms, functional disability, anxiety, and depression.
Physical symptoms (pain and fatigue) and limitations (keeping up with friends) were reported as the most difficult parts of having Hypermobile Ehlers-Danlos Syndrome.
A higher number of somatic symptoms was the strongest predictor of disability, anxiety, and depression. Physical symptoms are subjectively distressing and significantly related to impairments in physical and psychological functioning.
Many are told there are no treatments, only prevention of worsening problems
As pointed out in the publication Ehlers Danlos Syndrome (6) from the National Center for Biotechnology Information, “with hypermobile joints comes the increased risk of joint subluxation and dislocation. Prevention is the most crucial aspect of medical management as each injury increases the likelihood of recurrence and complications such as osteoarthritis. Prevention revolves around robust patient counseling regarding the limitation of at-risk activities like contact sports or weightlifting.” Further, “It’s also important for the patient to understand that there is no cure for the disease, and as of now, there is no medical treatment that can completely resolve their symptoms. This fact may be difficult for many patients to internalize and accept, which is why psychosocial support, including expectation management, for patients suffering from EDS is paramount in their care.”
In 2011, after seeing many patients with what was then coined “Joint Hypermobility Syndrome,” I suggested in the Journal of Prolotherapy: (7)
Management of Joint hypermobility syndrome (JHS) and Ehlers-Danlos Syndrome (EDS) frequently includes education and lifestyle advice, manual therapy, taping and bracing, electrotherapy, exercise prescription, functional rehabilitation, and collaborative working with a range of medical, health, and fitness professionals. Progress is often slow and hampered by physical and emotional setbacks. The functional rehabilitation process is frequently lengthy, with the 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.
The path of treatment EDS hypermobility type increasingly recognized in pediatrics – changing outlooks
A January 2022 paper from the Department of Pediatrics, University of Cincinnati College of Medicine was published in the American Journal of Medical Genetics. Part A. (8) In this paper the current treatment guidelines are examined. Here are the learning points of this research:
- EDS hypermobility type (EDS-HT), characterized by joint hypermobility, is most common and increasingly recognized in pediatrics.
- Treatment involves protecting joints, preventing injuries, and managing symptoms/comorbidities.
- Pediatric EDS hypermobility type patients often see multiple medical providers; however, data on healthcare utilization (what type of treatments they are getting) in this population are lacking.
What the authors of this study then set out was to find out what type of treatments these children were getting. In all 102 children had their medical paths followed over a 21 month period.
- After EDS hypermobility type diagnosis, treatment patterns shifted to reflect greater involvement of therapy (physical, psychological, and occupational) and symptom management.
- Predignosis: More genetics, rheumatology, and orthopedics visits occurred
- After diagnosis: More physical therapy, pain management, cardiology, and neurology visits occurred
- Testing and hospital encounter/procedure frequencies did not change.
- Overall, the pattern of these children’s health care changed from diagnostic to treatment.
Treatments that are not helping because doctors do not clearly understand the symptoms
The statement that doctors do not clearly understand is not just the opinion of this article. Look at a study from September 2020 (9). The authors of this study come from Rosalind Franklin University of Medicine and Science, Toronto General Hospital, University Health Network, DePaul University, and Brown University.
The learning points of this research:
- The majority of research which describes pain in pediatric Joint Hypermobility Syndrome and Ehlers Danlos Syndrome has focused on the chief pain complaint of musculoskeletal pain, so too has research related to the management of this pain.
- Other symptoms, such as headaches, are under-explored.
- Research suggests an avoidance of NSAIDs for migraine and tension-type headaches in pediatric Ehlers Danlos Syndrome based on research that shows adults with EDS have adverse gastrointestinal, renal and hematologic side effects and may worsen the symptoms of co-existing Mast Cell Activation Syndrome.
- However, as literature in pediatric EDS is limited, it should be noted that in the adult population, headaches are treated based on suspected causes of pain. For example, headaches can be a symptom of loose ligaments in the craniocervical junction and cervical spine. This would be suspected with neck pain. However, migraine headaches and tension-type headaches might be treated pharmaceutically. Therefore, to better guide the treatment of headaches in pediatric EDS, a greater study of the causes of such pain must first be conducted in order to guide recommendations for treatment.
Note: Here a connection is made between cervical spine instability and headache in hEDS patients. Our treatment for cervical spine instability is discussed below. It is just not the headache as we mentioned above, treating cervical spine instability by treating the loose ligaments may impact many if not all the symptoms the patient is suffering from. Let’s get back to the study.
More learning points of this research: Abdominal pain
“As with the treatment of headaches, because abdominal pain itself has received inadequate attention in pediatric EDS patients, recommendations related to the management of abdominal pain in children and adolescents remain unclear.”
Further: Despite abdominal pain being well reported in adults with EDS, no research identifies interventions to alleviate such uncomfortable symptoms outside a concurrent diagnosis of FGID (Functional GI Disorders) is offered for younger patients. Likely due to the unclear origin of GI Disorders in younger EDS patients.
The researchers do point out psychological interventions, such as cognitive-behavioral therapy and hypnosis have successfully produced significant reductions in painful FGID symptoms among pediatric populations. As well as the use of peppermint oil and cyproheptadine, a first-generation antihistamine was found to reduce the frequency and intensity of pain in children and adolescents with abdominal pain-related FGID.
While providing some with symptom benefit, hypnosis does not treat underlying causes, nor do anti-histamines or peppermint oil. Parents are searching for something more to get their children back to a degree of normalcy.
Pediatric postural orthostatic tachycardia syndrome – one-fifth of patients had EDS and over one-third of patients had HSD
We have many articles on postural orthostatic tachycardia syndrome or (POTS) and the problems of heart rate.
- Postural Orthostatic Tachycardia Syndrome (POTS) is caused by cervical instability
- When persistent post-concussion syndrome turns into a neurologic mystery
- Dystonic storms in four patients with hypermobile Ehlers-Danlos Syndrome
- Ehlers-Danlos Syndrome, Atlanto-axial instability, and Craniocervical instability
- Inappropriate sinus tachycardia – Elevated heart rate
Here we will explore research from March 2021 which was published in the journal Autonomic Neuroscience: Basic & Clinical. (10)
This is the summary learning points of this research:
- The researchers noted the clear connection between postural orthostatic tachycardia syndrome (POTS) and coexisting joint hypermobility or Ehlers-Danlos Syndrome (EDS) or hypermobility spectrum disorder (HSD). The prevalence of EDS and HSD in POTS has been demonstrated in smaller studies combining adult and pediatric patients. The purpose of this study was to assess the prevalence in children with POTS.
As you probably know firsthand, it can be challenging to get an accurate diagnosis or even basic awareness of your child’s problems from their doctors. Much of this is based on the doctor’s not understanding what is happening in your child. This last statement should not really shock anyone. One of the main reasons that you are reading this article is that you are looking for information beyond what you are getting from your doctors now.
In this paper, patients 18 years old, or less, diagnosed with POTS symptoms for at least six months based on “frequent debilitating symptoms of orthostatic intolerance, plus a consistent heart rate increase of at least 40 beats per minute without orthostatic hypotension on a standing test,” were also assessed for “findings suggestive of EDS” and were further evaluated in Connective Tissue Disorders clinics.
- There were 362 patients meeting inclusion criteria, of which:
- 82 patients had EDS (22.7%) and
- 141 patients had HSD (39.0%).
- Patients with EDS had an earlier median age at symptom onset (12 years old versus 13 and a half years old) and longer median symptom duration (2.5 vs. 1.5 years) compared to patients without hypermobility.
Conclusions: (The researchers) evaluation of a large series of pediatric patients with POTS revealed that over one-fifth of patients had EDS and over one-third of patients had HSD. The awareness of the prevalence of comorbidities such as hypermobility disorders may help inform providers diagnosing and caring for these patients.
In our case histories, we have many examples. Some about children who came to us with a long prescription list. One story is that of a girl between the ages of 6 and 12 who were very active in gymnastics. She had to stop gymnastics when her right hip became painful and, despite lots of therapies and doctors, developed into constant throbbing pain. Her list of previous therapies to resolve this pain included: physical therapy, prescription medications, deep tissue massage, nerve blocks, acupuncture, and Feldenkrais, an alternative type exercise program. Her pain medication grew to include Zoloft, Tylenol #3, Prozac, Clonazepam, Effexor, and Soma. Despite all this, she reported increasingly terrible pain. We first saw this patient when she turned 18, so the treatments reported here were ongoing for 6 years. This patient visited us regularly for Prolotherapy treatments for the next two years. She was slowly weaned off of all of her medications. The treatments are explained below.
I just want to go back to school
We often see teenagers who were looking forward to starting a college education that has been derailed by symptoms.
I have Ehlers Danlos syndrome. I have a lot of GI symptoms, GERD, acid reflux, nausea, bloating, cramps, and constipation. I am seeing a gastroenterologist and I get prescribed medications. Nothing is really helping. I also have migraines, neck stiffness and pain, dizziness, fatigue, and ringing in my ears. I am seeing the chiropractor now. The chiropractor is strongly suggesting a closer examination of my neck as being the source of my symptoms. I want to be able to go to school and not have the symptoms interfere.
When physical therapy is not helping
This is a November 2018 study that appeared in the Journal of Foot and Ankle Research. (11) The study examined why targeted physical therapy to the knees and ankles seemed to offer no extra benefit.
This is what the researchers at the University of Newcastle in Australia said: “There is very limited evidence to guide the use of physical and mechanical therapies for lower limb problems in children with Hypermobility Spectrum Disorder and Hypermobile Ehlers Danlos Syndrome. Mechanical therapies have not been evaluated in randomized control trials and results of the two randomized control trials of physical therapies do not definitively guide physical therapy prescriptions. Current studies are limited by small sample sizes and high attrition rates. No physical therapy has been compared to a sham intervention no intervention or no intervention, so overall effectiveness is unknown.”
“Physical therapy prescription, regardless of joint range or type of exercise, may provide benefit in regard to pain intensity, however, this has not been adequately established by available research.”
This is something we hear from many parents on their first visit. Their child suffered a significant ankle sprain. After 8 weeks and many physical therapy sessions, the ankle has not shown much improvement. Why? To understand how physical therapy can be made more effective we must understand why physical therapy is not effective.
Physical therapy is a major component of the “conservative” or “multidisciplinary” approach to joint pain relief. Our experience, in the patients we see, is that the results of PT are often disappointing.
Who are the patients who would not benefit from physical therapy? Patients with loose ligaments and tendons.
In the above study, the knee and ankle were examined in patients that targeted physical therapy did not provide additional benefits. So let’s discuss the knee. We often see children with dislocating knee caps or patellas.
- Physical therapy includes leg extensions and stretching exercises to help strengthen the thigh muscle, so the patella or kneecap, tracks better on the femur – it doesn’t scrape the thigh bone.
- Physical therapy seeks to strengthen the quadriceps as these muscles are the main stabilizers of your kneecap. Physical therapy will have limited or no success if the quadriceps tendon, the tendon that connects the “quads,” the vastus lateralis, vastus medialis, vastus intermedius, and the rectus femoris muscles to the knee cap are weakened.
- For physical therapy to work, there must be some resistance between muscle and bone. If the quadriceps tendon is damaged, injured, stretched, or harmed in a significant way, physical therapy will have limited if no success.
- Physical therapy will also not work if the ligaments of the knee are compromised or weakened. Ligaments, such as the ACL. MCL, PCL hold the knee together by connecting the bones.
- Muscle-strengthening exercises may improve the relative location of the patella upon movement, but do not improve the tendons, ligaments, or cartilage.
In returning to my 2011 paper (7) I suggested in the Journal of Prolotherapy:
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 (awareness of proper movement) enhancing exercises to decrease pain and prevent further injuries.
However, joint complaints increase with vigorous and repetitive activities, hypermobile patients 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 stopped or reduced.
Joint stabilization rather than joint flexibility – Prolotherapy treatments
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 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.
The main talking point of our treatments is that Hypermobility Spectrum Disorder and Hypermobile Ehlers Danlos Syndrome patients have joint pain, joint instability, and chronic joint dislocations from ligament laxity. This is well understood in the medical community. What is not well understood is that injecting simple dextrose into the ligaments and tendon attachments in the joint and spine would strengthen the ligaments and tendons.
In Hypermobile Ehlers Danlos Syndrome, the discussion often turns towards collagen. Hypermobile Ehlers Danlos Syndrome is caused by defects in the biogenesis of collagen, the major structural protein of the body. The condition can be either inherited from a parent with the defect or caused by a genetic mutation. When a defect such as the one found in Ehlers Danlos Syndrome is present, collagen fibers weaken, allowing tissues to become more elastic. In the case of Hypermobile Ehlers Danlos Syndrome, the weakened collagen fibers affect the integrity of ligaments in the joints and ultimately the stability of the joint. The weakness of these ligaments is what allows the joints to hyperextend beyond the normal physiological limits and eventually become unstable.
Hypermobility in any body part (or systemically in all body parts) in and of itself is not a problem; as a matter of fact, it has some benefits. The greater agility that whole-body hypermobility offers allows the person to perform a number of physical activities with greater ease including ballet dancing, gymnastics and acrobatics, and musical talents such as playing the flute, piano, and violin.
The genetic disorders that make up systemic joint hypermobility syndromes and disorders are caused by a defect in the structural integrity of their collagen. Simply put, their collagen is weak.
Connecting ligament weakness with chronic pain from joint instability in Hypermobile Ehlers Danlos Syndrome
Pain and instability in a joint are caused by the joint hyperextending beyond the “stretchability” range of the ligaments that hold the joint together. People who start with joint hypermobility or Hypermobile Ehlers Danlos Syndrome have ligaments that have already been genetically weakened. This puts them at elevated risk for developing joint instability. It takes less force to injure a ligament or cause joint instability in these patients.
When the ligaments can no longer hold the adjacent bones in a stable position, the person experiences pain, and other joint stabilizers and adjacent joints are more prone to being injured. It is for this reason, especially that people with genetic hypermobility syndromes should receive Prolotherapy (or least get an evaluation by a Prolotherapist) sooner rather than later for ligament injuries. It does not take very long for one simple ligament injury to start a cascade that results in multiple joints becoming unstable.
We believe Prolotherapy offers great hope to those with symptoms of hypermobility because it is designed to successfully treat the ligament and tendon laxity that accompanies Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome, as well as strengthen the joints in the body.
Prolotherapy is the injection of simple dextrose. The research and application are discussed at length in our article Prolotherapy Research and Treatment Guidelines.
If we take splints and braces, heat therapy, and physical therapy treatments at face value we find a group of treatments that seek to stabilize a painful joint (hold it together with splints and braces), bring circulation to the joint, (heat therapy), stresses movement (physical therapy) to build up strength in the joint. These are all part of the therapeutic properties of Prolotherapy injections.
Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).
Above we cited this research in the Journal of Prolotherapy in 2011.
Here are our bullet points:
- While traditional medical treatments including education and lifestyle advice, behavior modification, physiotherapy, taping and bracing, exercise prescription, functional rehabilitation and pain medications offer some symptomatic control, they do little in regard to curbing the progressive debilitating nature of the diseases.
- The excessive joint mobility with its subsequent joint degeneration and multiple joint dislocations can then lead the individual to seek out surgical intervention, which has suboptimal results in the hypermobile patient population versus the normal population. As such, some patients with Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (HSD) are seeking alternative treatments for their pain, including Prolotherapy.
- Prolotherapy offers great hope for those with symptoms from generalized hypermobility because it is designed to successfully treat the ligament and tissue laxity that accompanies Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.
- Prolotherapy works by initiating a brief inflammatory response, which causes a reparative cascade to generate new collagen and extracellular matrix giving connective tissue strength and the ability to handle strain and force.
- Prolotherapy has a long history of success in treating ligament injuries, including patients with joint hypermobility.
- 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.
The rationale for using Prolotherapy for patients with Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome include 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),
- gives pain relief relatively quickly
- 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 Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.
A Patient Story
In the first video, mother Lorraine tells the story of her daughter Louise. The video transcript summary is presented below the video.
We did not recognize that there was anything wrong with Louise. She always enjoyed climbing on the climbing frame, she is very good at hanging from the monkey bars, so there is no clue as to what was to come. We knew she was flexible and she positively enjoyed gymnastics which she excelled at for obvious reasons.
It was only after one training session the coach came up to me and said Louise has just dislocated her shoulder but it’s OK it’s gone back in again.
I knew that wasn’t right. I asked Louise about it and he said, “Oh mommy, that happens all the time I just put it back in again.”
So I decided to take her to see a specialist and I explained this to him, he examined Louise and asked several questions. He pulled on her skin and then he asked me if I ever heard of Ehlers Danlos Syndrome? Of course, I hadn’t. He explained what it was. That she would have joint hypermobility, stretchy skin, but not to worry it wasn’t too serious.
But as she began to grow she started to have more issues with her joints. She’s had numerous issues with her ankles, her knees, she regularly dislocates her thumbs particularly if she’s using scissors which scares the living daylights out of her friends at school.
She was beginning to lead a relatively normal life. We had to, everything couldn’t stop because she dislocated joints. Her friends learned to be careful and not to do any sudden moves because it was when she was not prepared for a move that something would happen. If she was engaged in physical activity she was fine because she knows what was going to happen. Louise started playing musical instruments. She plays the piano, the saxophone, and was she teaching herself how to play the ukulele.
Louise was leading a full life but with restrictions. Unfortunately, she had to give up gymnastics because she couldn’t trust her body and she was beginning to have injury after injury.
By Christmas 2013, Louise was in a wheelchair. She tore a ligament in her foot and because of her weak shoulders she couldn’t use crutches. She was in a wheelchair for over 3 months. It was at this time we began to worry about her future and this was compounded when she dislocated her shoulder again, this time by pushing herself up from the floor from a seated position how is she going to manage in the future if she’s going to continually injure herself by doing the most mundane things.
We knew we had to do something but we didn’t know what to do. She was missing so much time from school with injuries to her shoulders, spine, and thumbs and she had repeated muscle spasms it couldn’t go on like this. Our only option would be to consider homeschooling so that we would not miss more education.
It was around the same time I was looking at the Ehlers Danlos Facebook page when suddenly appearing in my newsfeed was a study: “Treatment of joint hypermobility syndrome including Ehlers Danlos Syndrome with Hackett Hemwall Prolotherapy.”
I’ve never heard of this. So I went to the Journal page and I read the piece. I could not believe what I was reading. Here was a doctor who was treating patients with Ehlers Danlos Syndrome using injections of just for sugar solution including amazing results I hardly dare believe it could be true. I went to the caringmedical.com website and read even more about the treatment.
I started to get angry why did we not know about this treatment that could help so many people. It was clear that Dr. Hauser knew about Ehlers Danlos Syndrome as he’d been treating patients for over 20 years. I made up my mind then that Louise was going to Chicago and she was going to have Prolotherapy. Louise is finally able to enjoy her life. She has discovered aerial gymnastics, This was a chance for her to really enjoy her gymnastics in a more controlled and safe way.
Summary and contact us. Can we help you? How do I know if I’m a good candidate?
We hope you found this article informative and it helped answer many of the questions you may have surrounding Ehlers Danlos syndrome hypermobility type. Just like you, we want to make sure you and your children are a good fit for our clinic prior to accepting your case. While our mission is to help as many people with chronic pain as we can, sadly, we cannot accept all cases. We have a multi-step process so our team can really get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments that we offer here.
Contact the Hauser Neck Center Patient Team
References for this article:
1 Sharp HE, Critchley HD, Eccles JA. Connecting brain and body: Transdiagnostic relevance of connective tissue variants to neuropsychiatric symptom expression. World Journal of Psychiatry. 2021 Oct 19;11(10):805. [Google Scholar]
2 Bulbena-Cabre A, Duñó L, Almeda S, Batlle S, Camprodon-Rosanas E, Martín-Lopez LM, Bulbena A. Joint hypermobility is a marker for anxiety in children. Revista de Psiquiatría y Salud Mental (English Edition). 2019 Jun 5. [Google Scholar]
3 Baeza-Velasco C, Gely-Nargeot MC, Vilarrasa AB, Fenetrier C, Bravo JF. Association between psychopathological factors and joint hypermobility syndrome in a group of undergraduates from a French university. The International Journal of Psychiatry in Medicine. 2011 Feb;41(2):187-201. [Google Scholar]
4 Shulman RJ, Self MM, Czyzewski DI, Goldberg J, Heitkemper M. The Prevalence of Hypermobility in Children with Irritable Bowel Syndrome and Functional Abdominal Pain Is Similar to that in Healthy Children. The Journal of Pediatrics. 2020 May 4. [Google Scholar]
5 Tran ST, Jagpal A, Koven ML, Turek CE, Golden JS, Tinkle BT. Symptom complaints and impact on functioning in youth with hypermobile Ehlers–Danlos syndrome. Journal of Child Health Care. 2019 Aug 1:1367493519867174. [Google Scholar]
6 Miklovic T, Sieg VC. Ehlers Danlos Syndrome. [Updated 2019 Oct 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan. [Google Scholar]
7 Hauser R, Phillips HJ. Treatment of joint hypermobility syndrome, including Ehlers-Danlos syndrome, with Hackett-Hemwall prolotherapy. J Prolotherapy. 2011 May;3(2):612-29. [Google Scholar]
8 Williams SE, Tran ST, Lynch-Jordan A, Goldschneider KR, Ting TV, Kashikar-Zuck S, Neilson D. Healthcare utilization among youth with Ehlers-Danlos syndrome hypermobile type. Am J Med Genet A. 2022 Jan 6. doi: 10.1002/ajmg.a.62625. Epub ahead of print. PMID: 34989147.
9 Feldman EC, Hivick DP, Slepian PM, Tran ST, Chopra P, Greenley RN. Pain Symptomatology and Management in Pediatric Ehlers–Danlos Syndrome: A Review. Children. 2020 Sep;7(9):146. [Google Scholar]
10 Boris JR, Bernadzikowski T. Prevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome. Autonomic Neuroscience. 2021 Mar 1;231:102770. [Google Scholar]
11 Peterson B, Coda A, Pacey V, Hawke F. Physical and mechanical therapies for lower limb symptoms in children with Hypermobility Spectrum Disorder and Hypermobile Ehlers-Danlos Syndrome: a systematic review. Journal of foot and ankle research. 2018 Dec 1;11(1):59. [Google Scholar]
This article was updated January 10, 2022