Digestive problems in the hypermobile Ehlers Danlos Syndrome patient

Ross Hauser, MD.

At our center, we specialize in helping people with Ehlers Danlos Syndrome. If you have this diagnosis you know what it is, if you don’t, this is a disorder of the connective tissue which can cause among other problems of “being double-jointed,” and the “hypermobility,” of the joints causing frequent dislocations and subluxations (partial dislocations). At our center, we help these people with Prolotherapy injections, which will be explained below. These injections can strengthen their joint and spinal ligaments to help hold everything in place.

In the context of this article, we are going to explain how these injections can help hold the cervical spine vertebrae in place and possibly eliminate or alleviate the numerous symptoms that these people suffer from.

In some of the sample stories below, you will see that many people mention a diagnosis of Ehlers Danlos Syndrome. We will also see what else they discuss. Some of these stories may sound very familiar to you.

Discussion points of this article:

I have Ehlers Danlos Syndrome I think my vagus nerve is the problem

I have a diagnosis of Ehlers Danlos Syndrome, Mast Cell Activation Syndrome, and numerous digestive disorders. The reason I think it is my vagus nerve is because of the countless hours I have spent researching my problems online and because of the number of questions I have been asking anyone who I think can help me.

Here is my situation as it relates to my SIBO. I am always bloated. I have been constipated for what seems like years. I cannot remember the last time I had a good bowel movement. I don’t digest anything. I have Amenorrhea. I can’t remember having a period. I have inflammation along my digestive tract and I have inflammation in my joints. I do not sweat or I sweat too much. I have a racing heart rate that I would like to lower. I have a lot of pain and my mind “is not right.” All this leads me to believe that I have issues with my vagus nerve. My research has led me to believe that I am not pushing my food down my digestive tract, it is getting stuck or backed up. I think this is causing the bacteria to build up in my small intestine. I think I need to make my vagus function better.

Below we will discuss these very problems of backed up food and clogged routes and valves the food cannot pass through.

It started with Ehlers Danlos Syndrome diagnosis and it got worse after a car accident

I have a lot of joint diseases, osteoarthritis from hEDS (Hypermobile Ehlers-Danlos syndrome). I have had a few surgeries to help with chronic joint dislocations. I have been involved in more than one auto accident. While this has caused significant pain and issues in my shoulder and neck, it is also causing me GI issues which I now consider the worse of my symptoms and the symptoms that I want to be treated as a priority. My symptoms include excessive gas, uncontrollable belching, the sensation of air being stocked in my chest and abdomen. I have a diagnosis of GERD, bowel transit, and GI motility issues. As for my SIBO, I did get some relief from my antibiotics but the symptoms come and go and I am on and off the antibiotics more than I care to be at this point. I have changed my eating habits to accommodate these problems. I feel that I have a problem with food fermenting in my gut.

Ehlers Danlos Syndrome and neck instability. All I do is make gas

For as long as I can remember I have been double-jointed. I was suspected to have hEDS but a true diagnosis could not be made until I got older. Over the years I got a lot of relief from chiropractic care. But as the years passed my neck and upper back were becoming big problems. My cervical spine would “pop,” “click,” and then feel like it had slipped out of place. I then went to a posture specialist. That helped a little. As I was having problems with TMJ, I was given an appliance that would help me keep my neck and jaw in place. Unfortunately, my problems continued and progressed and became more severe.

Besides Ehlers-Danlos Syndrome, I was also diagnosed with Postural Orthostatic Tachycardia Syndrome. I was told to consider cervical fusion surgery. I have heard some people do very well with this procedure. I am scared and concerned that if the surgery is not a success that I will be worse. As my neck problems worsened so did my digestive problems. My stool does not move in my colon, I have terrible almost non-existent bowel transit. Not only do I have SIBO (small intestinal fungal overgrowth,) but I also have SIFO (small intestinal fungal overgrowth.) I am so bloated. I am bloated all the time. It seems all I do is make gas. I am being treated for Celiac Disease as well and I am on all sorts of modified and rotational diets.

Ehlers Danlos Syndrome, Lyme Disease and digestive problems

I was diagnosed with Lyme disease. This led to a diagnosis of Ehlers Danlos Syndrome. My doctors are not sure when the EDS showed up, before the Lyme Disease? After the Lyme Disease? This started my journey of confusing diagnoses. My doctors were looking for clues to my neck pain. Was the neck pain and now new neurological and digestive symptoms from Lyme Disease? Did I always have these problems but now they were made worse by Lyme Disease? I did suffer a concussion and whiplash injury before the Lyme Disease diagnosis. Now I have SIBO, food sensitivities, and bowel problems. I am looking for a path to recovery.

Gastrointestinal Manifestations in Joint Hypermobility Syndromes

In 2018, doctors at Texas Tech University gave a definition and understanding of various disorders linked with Joint Hypermobility Syndromes, including gastrointestinal problems in patients with Ehlers-Danlos syndrome (hEDS) and benign joint hypermobility syndrome (BJHS/JHS). Their paper was published in The American Journal of the Medical Sciences. (1) Here is what they wrote:

“. . . in a subset of individuals, joint hypermobility causes a range of clinical problems mainly affecting the musculoskeletal system and, to a lesser extent, extra-articular disorders. Joint hypermobility often appears as a familial (inherited) trait and is shared by several inherited connective tissue disorders, including the hypermobility subtype of Ehlers-Danlos syndrome (hEDS) and benign joint hypermobility syndrome (BJHS/JHS).

Although joint hypermobility has primarily been thought of as a rheumatological disorder, increasing evidence shows significant associations between both hEDS and BJHS with specific extra-articular disorders. To date, the strongest associations of these 2 conditions are with anxiety disorders, orthostatic tachycardia, various functional gastrointestinal (GI) disorders, and pelvic and bladder dysfunction.”

Prevalence of many gut symptoms are significantly higher in HSD/hEDS

A September 2022 paper (2) from doctors at the Department of Gastroenterology, Central Clinical School, Monash University and Alfred Health in Australia found the “prevalence of many gut symptoms, including abdominal pain, postprandial (prolonged post-meal) fullness, constipation, and diarrhea are significantly higher in HSD/hEDS compared with non-HSD/hEDS individuals. Disorders of gut-brain interaction are also common, particularly functional dyspepsia (indigestion or upset stomach with no obvious cause).” The paper continues: “All general gastroenterologists will encounter patients with (diagnosed or undiagnosed) HSD/hEDS. Recognition of such patients and a general understanding of the implication of such a disorder will provide the opportunity for timely and reassuring explanation, for arranging multidisciplinary care as required, and minimizing inappropriate investigations and therapies. In this way, gastroenterologists have the opportunity to improve the long term outcomes of these patients.”

This universal approach to IBS management may account for the reported lack of efficacy of treatment.

In December 2021, doctors at the Queen Mary University of London wrote in the American Journal of medical genetics. Part C, Seminars in medical genetics (3) of their findings in connecting irritable bowel syndrome and hypermobile Ehlers-Danlos syndrome.

“Irritable bowel syndrome (IBS) is common, but its cause remains unknown. IBS patients present with gastrointestinal (GI) symptoms such as abdominal pain with altered bowel habits; however, some patients also have non-GI symptoms including muscle and joint pains.

It is thus plausible that within large IBS cohorts, subgroups exist with distinct clinical phenotypes. (In other words, it can be possible to find and research people with Irritable bowel syndrome who also happen to have hypermobile Ehlers-Danlos syndrome).

Yet, these subgroups (hypermobile Ehlers-Danlos syndrome patients with IBS) have not been clearly identified or characterized.

Due to lack of segmentation, treatment-focused symptomatic management is similar for all with IBS and follows indiscriminate algorithms regardless of the possible differing clinical phenotypes. This universal approach to IBS management may account for the reported lack of efficacy of treatment. One emerging subgroup receiving increasing attention is that with overlap IBS and the underlying heritable connective tissue disorder, hypermobile Ehlers-Danlos syndrome (hEDS).

Current evidence suggests that up to 62% of patients with hEDS suffer from IBS. However, despite recognition of the presence of IBS in hEDS, this overlap IBS/hEDS group has not been characterized and these patients are managed in a similar way to those with IBS alone.”

Reducing the risk of iatrogenic [doctors making the patients worse by wrong diagnosis or treatment)

A June 2022 paper in the journal Frontline gastroenterology (4) looked at the problems hEDS patients face with significant gastrointestinal (GI) symptoms. What these researchers focused on was the lack of information or published research on the problems of dysmotility (muscle impairment in the digestive tract that alters the movement of food through the GI tract). Listen to what they said, (some explanatory notes have been added).

“GI symptomatology is often due to disorders of gut–brain interaction (for example, functional dyspepsia [indigestion or upset stomach], irritable bowel syndrome), and in the absence of objective evidence of dysmotility or other dysfunction, a biopsychosocial [addressing physical, mental and social interaction factors in what is making the patient unwell] model of treating symptoms is often most effective, reducing the risk of iatrogenic [doctors making the patients worse by wrong diagnosis or treatment) harm in this group of patients.] “

Basically, the inability to move food (dysmotility) to the GI tract is often not looked for. If dysmotility if the main problem, then doctors, simply treating symptoms, may do more harm than good because the treatments do not address the underlying cause.

“Postural tachycardia syndrome has been demonstrated in around 30% of patients with hypermobility disorders, with possible increased GI symptomatology if present. The potential mechanisms for this have not been fully elucidated.” Please see my articles where a possible connection between cervical spine and neck instability may cause some of these symptoms.

Eating-related symptoms, leading to reduced oral intake and/or food avoidance, should prompt review for specific underlying disorders of gut–brain interaction. (Doctors are suggesting that the ability to move food down the GI tract should be explored promptly when patients stop eating.) Oral diet and oral nutrition supplements should be optimized as the primary strategy via a multidisciplinary team approach. The evidence for clinically assisted nutrition and hydration is for objectively demonstrated malnutrition or electrolyte disturbance only, and should not be implemented for symptom management alone, given its associated iatrogenic risks. (Insertion, accidental removal, living with nutritional tubes, may cause the patient more distress than the symptoms of malnutrition are not severe enough to warrant this action).

Diagnosis: Rome IV criteria for functional gastrointestinal disorders

This next section will be a discussion of certain digestive orders. The Rome IV criteria for functional gastrointestinal disorders will be discussed, therefore, a simple understanding of the criteria is needed. These symptoms are addressed in a 2016 paper in the journal Gastroenterology: (5)

Symptoms that can be attributed to the gastroduodenal region represent one of the main subgroups among functional gastrointestinal disorders.

(1) functional dyspepsia, characterized by 1 or more of the following: postprandial fullness, early satiation, epigastric (upper abdominal) pain, and epigastric (upper abdominal) burning, which are unexplained after a routine clinical evaluation; and includes two subcategories: postprandial (after meals) distress syndrome that is characterized by meal-induced dyspeptic symptoms and epigastric pain syndrome that does not occur exclusively postprandially; the two subgroups can overlap;

(2) belching disorders, defined as audible escapes of air from the esophagus or the stomach, are classified into 2 subcategories, depending on the origin of the refluxed gas either gastric and supragastric belch; (3) nausea and vomiting disorders, which include 3 subcategories: chronic nausea and vomiting syndrome; cyclic vomiting syndrome; and cannabinoid hyperemesis syndrome (chronic vomiting in long-term marijuana users); and (4) rumination syndrome (the repeatedly and unintentionally regurgitation of food from the stomach that is either swallowed again or spit out).

In February 2021, (6) researchers sent out an online general health survey that was completed by 603 individuals with HSD/hED and 603 people used as a control to this study. The average age of the study participant was 39 years old and 96% were women.

The survey included questions about Rome IV FGIDs, non-GI and non-musculoskeletal somatic symptoms, quality of life, medical history and healthcare use. The prevalence of FGIDs was compared between cases and controls.

“POTS was found to be an independent predictive factor for GI dysmotility.”

A combined research study from the Cleveland Clinic Foundation, East Tennessee State University, and New York Medical College published in the journal Cureus (7) aimed to evaluate the prevalence of and factors associated with gut dysmotility in patients with hEDS. The researchers reviewed hEDS patient records who were seen over a ten-year period.  Demographics, GI motility testing, endoscopic, and imaging data were extracted from the patients’ charts.

In my article Postural Orthostatic Tachycardia Syndrome (POTS), the Vagus Nerve, and Cervical Spine instability I write: Some patients do get a POTS diagnosis, especially after their doctors now suspect a diagnosis of Ehlers-Danlos Syndrome. Let’s point out that some people, specifically those with a history of joint dislocation, or double-jointed characteristics may be thought of as having Ehlers-Danlos Syndrome. Not all people with suspected POTS have Ehlers-Danlos Syndrome. The focus is on whether the hypermobile-type of Ehlers-Danlos Syndrome is causing the cervical spine to become unstable and allowing for the bones of the neck to compress on the vagus nerves. This then can be seen as a possible cause of the cardiac-like problems that are characteristic of POTS.”

“Recognition that underlying gastrointestinal symptoms might be due to an underlying heritable connective tissue disorder rather than due to an unknown cause might be helpful in reducing the stigma of experiencing a medically unexplained illness.”

A November 2020 paper in the journal Clinical and Translational Gastroenterology (8) wrote: “Recent studies demonstrated that joint hypermobility syndrome (JHS) is strongly associated with unexplained dyspeptic symptoms in patients attending gastrointestinal clinics. In this study, the doctors “aimed to study the relationship between symptoms, gastric sensorimotor function, and joint hypermobility syndrome (JHS) in functional dyspepsia patients.”

Patients who were being treated by specialists for functional dyspepsia and who underwent a gastric barostat study and a gastric emptying breath test were recruited for assessment of JHS.

Of note in this research is this connection to other conditions which may muddy the diagnosis and confuse doctors.

“patients with Joint hypermobility syndrome might experience multiple comorbidities such as chronic widespread pain, POTS that can be worsened by meals, and mast cell activation disorder. It is, therefore, important to identify these patients in the gastroenterological clinical practice so that the comorbidities can be recognized as part of the spectrum of disorder that the patient presents with and appropriate referrals are made for their assessment so as to improve their quality of life. Recognition that underlying gastrointestinal symptoms might be due to an underlying heritable connective tissue disorder rather than due to an unknown cause might be helpful in reducing the stigma of experiencing a medically unexplained illness.

Please see my companion articles:

Mast Cell Activation Syndrome

A February 2024 paper in the journal Current opinion in gastroenterology (9) writes that “It is unclear whether a true association between hEDS/HSD and mast cell activation disorders exists. There is a high prevalence of nonspecific gastrointestinal symptoms in individuals with hEDS/HSD and patients may be at risk of macro-nutrient and micro-nutrient deficiencies, although the current evidence base is limited.”

A December 2021 study in the American journal of medical genetics. Part C, Seminars in medical genetics (10) Reports link Mast cell activation disease (MCAD) with hypermobile Ehlers-Danlos syndrome (hEDS), hypermobility spectrum disorder (HSD), and with primary immune deficiencies such as complement and immunoglobulin deficiencies (making you more prone to infection).” In this paper, a review of patient data revealed:

A FODMAP Diet helps patients with Joint hypermobility syndrome more than patients without Joint hypermobility syndrome

A FODMAP is an acronym that stands for fermentable oligosaccharides, disaccharides, monosaccharides, and polyols. The acronym describes foods that are short-chain carbohydrates (sugars) that are poorly absorbed in our bodies in the small intestine. People are put on a FODMAP diet to eliminate these foods as a means of reducing symptoms associated with irritable bowel syndrome (IBS) and/or small intestinal bacterial overgrowth (SIBO).

Some of the foods that your health care professional may suggest you avoid are Milk-based dairy, wheat, some fruits, and vegetables. It is not a simple as that however and as with any elimination diet, a customized program should be discussed with your health providers.

In 2019, doctors writing in the journal Gastroenterology Research (11) wrote: “The low fermentable oligosaccharide, disaccharide, monosaccharide, and polyol (FODMAP) diet causes significant clinical improvement in patients with irritable bowel syndrome (IBS). Joint hypermobility syndrome (JHS), defined as musculoskeletal symptoms in a hypermobile individual in the absence of systemic rheumatological disease, may be associated with functional gastrointestinal symptoms, including IBS. The aim of this study is to examine whether JHS can affect the response to the low FODMAP diet in patients with IBS.”

What the study team did then was to assess 165 patients (130 females, average age 44.)

Lower Bone Mineral Density

A December 2022 paper from the New York Institute of Technology College of Osteopathic Medicine (12) identified that pre-menopausal women with hEDS/HSD have significant GI complications and lower Bone Mineral Density compared to “healthy” women used as controls. The GI complications and reduced protein intake long-term may have a lasting impact on bone health.

Digestion & the Vagus Nerve: Sphincter function and related symptoms affected by neck instability

The vagus nerves carry signals both to and from the digestive tract. Signals running up the vagus nerve from the gut to the brain, carry, for instance, the sensation of hunger and fullness, and those from the brain to the gut affect digestion, secretion of digestive enzymes, and gastrointestinal motility.

Ross Hauser, MD discusses digestion and the vagus nerve as it relates to the sphincter function. In the histories of patients who we see in our center, they often feel like they hit a wall with regard to finding resolution of symptoms or their digestion conditions because the focus has been too narrow. When looking at many digestive symptoms through the aspect of vagus nerve health, many times solutions can be found because the vagus nerve innervates many vital digestive organs. Thus, in our center, we find that patients who have digestive complaints as part of their constellation of symptoms that also point to vagus nerve impairment, that the upper cervical area and the cervical curve should be analyzed and examined to see if this could be the cause of the issues.

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 craniocervical Instability, upper cervical spine instability, cervical spine instability, or simply problems related to neck pain. Just like you, we want to make sure you 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.

References

1 Botrus G, Baker O, Borrego E, Ngamdu KS, Teleb M, Martinez JL, Maldonado III G, Hussein AM, McCallum R. Spectrum of gastrointestinal manifestations in joint hypermobility syndromes. The American journal of the medical sciences. 2018 Jun 1;355(6):573-80. [Google Scholar]
2 Thwaites PA, Gibson PR, Burgell RE. Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know. Journal of Gastroenterology and Hepatology. 2022 Sep;37(9):1693-709. [Google Scholar]
3 Choudhary A, Fikree A, Aziz Q. Overlap between irritable bowel syndrome and hypermobile Ehlers–Danlos syndrome: An unexplored clinical phenotype?. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics 2021 Dec (Vol. 187, No. 4, pp. 561-569). Hoboken, USA: John Wiley & Sons, Inc. [Google Scholar]
4 Lam C, Amarasinghe G, Zarate-Lopez N, Fikree A, Byrne P, Kiani-Alikhan S, Gabe S, Paine P. Gastrointestinal symptoms and nutritional issues in patients with hypermobility disorders: assessment, diagnosis and management. Frontline Gastroenterology. 2023 Jan 1;14(1):68-77. [Google Scholar]
5 Stanghellini V, Chan FK, Hasler WL, Malagelada JR, Suzuki H, Tack J, Talley NJ. Gastroduodenal disorders. Gastroenterology. 2016 May 1;150(6):1380-92. [Google Scholar]
6 Lam CY, Palsson OS, Whitehead WE, Sperber AD, Tornblom H, Simren M, Aziz I. Rome IV functional gastrointestinal disorders and health impairment in subjects with hypermobility spectrum disorders or hypermobile Ehlers-Danlos syndrome. Clinical Gastroenterology and Hepatology. 2021 Feb 1;19(2):277-87. [Google Scholar]
7 Alomari M, Hitawala A, Chadalavada P, Covut F, Al Momani L, Khazaaleh S, Gosai F, Al Ashi S, Abushahin A, Schneider A. Prevalence and predictors of gastrointestinal dysmotility in patients with hypermobile Ehlers-Danlos syndrome: a tertiary care center experience. Cureus. 2020 Apr;12(4). [Google Scholar]
8 Carbone F, Fikree A, Aziz Q, Tack J. Joint Hypermobility Syndrome in Patients With Functional Dyspepsia. Clinical and translational gastroenterology. 2020 Nov;11(11). [Google Scholar]
9 Penny HA, Aziz I, Lam C. Mast cell activation and nutritional disorders in patients with hypermobility. Current Opinion in Gastroenterology. 2024 Feb 26:10-97.
10 Brock I, Prendergast W, Maitland A. Mast cell activation disease and immunoglobulin deficiency in patients with hypermobile Ehlers‐Danlos syndrome/hypermobility spectrum disorder. InAmerican Journal of Medical Genetics Part C: Seminars in Medical Genetics 2021 Dec (Vol. 187, No. 4, pp. 473-481). Hoboken, USA: John Wiley & Sons, Inc. [Google Scholar]
11 Fragkos KC, Keetarut K, Cox A, Eady J, Emmanuel AV, Zarate-Lopez N. Joint hypermobility syndrome affects response to a low fermentable oligosaccharide, disaccharide, monosaccharide and polyol diet in irritable bowel syndrome patients: a retrospective study. Gastroenterology research. 2019 Feb;12(1):27. [Google Scholar]
12 DiFrancisco-Donoghue J, Southard V, Margulies M, Jung MK, Allera A, Riley B. Assessment of gastrointestinal function and its’ effect on bone mineral density and body composition in hypermobility spectrum disorder and hypermobile Ehlers-Danlos syndrome. Journal of Clinical Densitometry. 2022 Oct 1;25(4):536-43. [Google Scholar]

 

 

 

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