Through the Nutritionists’ Eyes: The Ehlers Danlos Series

Hola Amigos,

When I started Brains & Bodies,  I did not want to accept  guest posts from practitioners, mainly because of the challenges I had with them. Sara Russel is an exception, as she is a practitioner diagnosed wit HDS.  Sara shares my goal to raise awareness about Ehlers Danlos Sydrome/ HDS.   You can look forward to Sara and  I,   collaborating  on Nutrition in the Ehlers Danlos Series.

Sara post is not short, but is certainly a worthwhile read. Time to pass you over. . . See you soon

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Common Ethical Issues People with EDS/HSD Encounter in Healthcare

This guest post aims to provide a sort of exposé of some of the ethical issues (both deliberate and unintentional on the part of health care providers) that people with EDS/HSD and other chronic and complex conditions encounter. Additionally, it offers some reflections on how both patients and care providers can work towards an alternative paradigm and different strategies.

In a sense, because of the extraordinarily complex, multi-faceted and poorly understood etiology, symptomatology and complications inherent in the Ehlers-Danlos Syndromes (which I’ll refer to here collectively as “EDS”) and the relatively newly-coined Hypermobility Spectrum Disorder, the EDS/HSD population can serve as the ultimate example of how often-hidden flaws in the health care system can have an amplified effect on people with chronic and complex health conditions. This piece focuses on selected of ethical problems, while recognising that the impact of course is much more vast.

The information contained in this post results from extensive reading of the medical and scientific literature, personal experience as a patient diagnosed with Hypermobility Spectrum Disorder, clinical experience as a nutritional professional working with a diverse set of chronic and complex health conditions and interviews and conversations with patients with EDS as well as health care providers, both those that are specialised in the treatment of EDS and those who are not.

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Common complaints I hear from people with EDS/HSD about their past or present care

“The doctor couldn’t account for my physical symptoms and wrote them off as nonexistent or psychological”.

Depression, anxiety and related disorders, while not specific to EDS/HSD, are more common in these groups than in the general population (Bulbena et al. 2017). The reasons are complex and are likely a combination of co-causative factors (hypermobility is postulated to have effects on the nervous system and the gut-brain axis that profoundly affect emotions), effects of symptoms (pain and fatigue can definitely cause anxiety and depression) and perpetuating/aggravating factors (depression and anxiety increase pain).

Iatrogenic factors, including over- and under-medication, delay in diagnosis, inappropriate medications and more, can cause varying degrees of anxiety, depression and other symptoms, including post-traumatic stress disorder.

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“I was denied pain medication when in excruciating pain and was treated like a drug addict”.

EDS and HSD patients often suffer from a high degree of pain which may not be easily recognised or validated. EDS patients frequently report going to the emergency room for a painful dislocation or subluxation and being denied pain medication even after describing debilitating pain. Uncontrolled pain, especially frequent or chronic, can have a profoundly negative impact on quality of life and can cause severe emotional and psychological distress.

“I was over-medicated with pain medications that didn’t lower my pain but only gave me side effects, and my doctor refused to consider any alternatives”.

As the president of a non-profit that connects EDS patients with EDS-focused medical providers noted in a personal conversation, a large percentage of patients with EDS lack the receptors to make opioid pain medications effective, yet these remain widely prescribed for pain management, and often patients become addicted to the medication while continuing to live in pain.

She described to me her experience of supporting EDS patients who experienced opioid addiction and uncomfortable side effects, including exacerbation in digestive symptoms (poor intestinal motility, constipation, gastroparesis, etc.), personality changes, reduced judgement and reduced cognitive functioning (increased “brain-fog” – which is already a concern for many hypermobile individuals in the first place). Some of these patients, she told me, have gone through painful withdrawal symptoms and required a lot of medical and emotional support to overcome their addiction. It is impossible to estimate how vast this problem is, considering that many people with EDS/HSD struggle with severe pain yet do not have a diagnosis.

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“One or more health care providers exploited me financially on the basis of my poor health and desperation for relief”.

Unmanaged or poorly managed symptoms can make people with EDS/HSD the target for deliberate or unintentional exploitation by health care providers promising or implying all kinds of miracles, and even suggesting that EDS is caused not by genetics but by other factors that can be “cured” easily (of course, with the specific product that the company or individual is trying to sell, or a specific treatment modality the person or company offers. Exploitation of people with EDS may take the form of “friends” pushing the latest cure-all essential oils, proprietary supplements, detoxification or cleansing programs, etc.).

I’m not saying by any means that people with EDS/HSD can’t benefit from the product and service categories listed above. However, I advise people suffering from EDS/HSD to be wary of any health care practitioner, product or program that offers an easy or miraculous “solution” or a one-size fits-all approach. It is in fact very important for practitioners to approach every hyper-mobile individual as a unique person who requires an individualised plan to manage symptoms and improve or maintain their quality of life, and of course, who can help set realistic goals based on the person’s unique and changing conditions.

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Solutions: What Practitioners and Patients Can Do

Why Health Care Providers Must Take a Complete Health History

A patient-centred, multi-disciplinary approach to care is critical. The health care provider must understand their patients’ health history and family history, and this involves careful listening, research and often a referral to an experienced specialist if the hyper-mobile patient is still undiagnosed.

Understanding that physical health problems cause emotional distress

Those who suffer from chronic physical symptoms that impact quality of life significantly may also have some anxiety and/or depression. Often, however, this is the only diagnosis they have, as if nothing else were wrong. Unfortunately, this implies that the physical symptoms don’t really exist. The patient knows better. Patients who are in pain and are told that she suffers exclusively from a mental illness will neither be fooled nor trust the physician.

Yet having physical pain and debilitating fatigue that others don’t see or understand is enough to trigger anxiety or depression in an otherwise well-adjusted person. As many hyper-mobile individuals describe, each in their own way, the true problem is very clear to them in their bodies,but nobody sees or understands it. Labelling a complex set of physical and emotional symptoms with a psychiatric diagnosis will only further the mutual reinforcement of physical and emotional symptoms.

Ultimately, being treated this way over and over again leads to a general mistrust of healthcare, which may in turn increase one’s vulnerability to exploitation by predatory marketing, which often does invest resources in making patients feel understood, not with the aim of solving a problem, but of selling a product or service regardless of its efficacy. The only real way to counteract this is for allopathic and holistic health care practitioners to listen to the patient and to collaborate with each other.

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High Ethical Standards and an Individualised Approach

High ethical standards and a bio-individual approach to health must go hand in hand. If your practitioner doesn’t have high ethical standards, you may find yourself buying large packages in a moment of weakness, or arrive home from each visit with loads of supplements that don’t make much of a dent.

If this has ever happened to you, it doesn’t mean that your practitioner intentionally exploited you. It’s more likely that he applied common marketing techniques that brought him money without improving your health. The money-centred model of care doesn’t work well with complex health cases. Nonetheless, most practitioners really believe in their process, in their protocols and in the products they sell.

Practitioners working or wishing to work with people who are hyper-mobile should bear the following in mind. Hyper-mobility is a multi-factor problem with complex etiologies and consequences, and exists on a very broad spectrum, and oversimplifying it is both disingenuous on the part of the person doing so and stigmatising for people who are affected by a complex and non-fully-remitting hyper-mobility.

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People with hyper-mobility should be wary of statements such as:

“I am better (or healed) because I did X, so you can get all better too if you’d only stop doing Y and do X like I do”

“I have (or had) a patient (or client or customer) with Ehlers-Danlos Syndrome who went through my program (took X supplement or medication or essential oil or protocol, etc.) and is now fine, so what are you waiting for?”

“All symptoms of Ehlers-Danlos Syndrome match perfectly with Lyme disease (or mold illness or mercury poisoning or copper dysregulation, MTHFR, etc.), so your diagnosis must be wrong, and I can fix it with X.”

You get the idea, right? This may not be a comprehensive list of red flags to look out for, but it’s a start. You may even have heard variations on several. I’m not saying that a person with Ehlers-Danlos Syndrome can’t be incorrectly diagnosed, or is exempt from also having another condition with similar symptoms.

In fact, hyper-mobile people sometimes do get Lyme disease or have mercury poisoning or dysregulated copper, but implying that EDS isn’t a distinct medical entity is ignorant and dismissive at best, predatory at worst, but probably most often some combination of the two.

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A Paradigm of Service

Both allopathic and holistic health care providers should approach their work from the standpoint of service to the patient or client. All too often, allopathic medical care feels rushed and non-empathic, while holistic care can feel to salesy or gimmicky. Neither of these approaches works well for the patient.

Ultimately, patients with complex illnesses, including EDS/HSD, do best in a multi-disciplinary situation where there is a patient-centred approach to care and an attention to the individual history, symptom picture and the elements that bring relief or trigger discomfort. The constellation of dysfunction in the body can vary from patient to patient, but clearly, an attention to each patient’s unique situation can help determine which practitioners are best suited to be part of the care team, so that the best possible support is offered to the patient.

About the Author

Sara Russell is a Nutritional Therapy Practitioner who works remotely with clients worldwide, specialising in complex cases. Sara comes from a family with a high prevalence of joint hyper-mobility and related medical conditions. She resides in the Tuscan countryside with her husband and six-year-old son. To learn more about Sara’s work and read her blog click here.

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Related Post on Brains & Bodies 

My Invisible Illness ft Tramadol: The Ehlers Danlos Series

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Selected Bibliography

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Castori M. Ehlers-Danlos Syndrome, hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular and systemic manifestations. ISRN Dermatology Vol 2012. Article ID 751768, 22 pp. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3512326/

Castori M, Voermans NC. Neurological manifestations of Ehlers-Danlos Syndrome(s): A review. Iran J Neurol 2014; 13(2):190-208.

Castori M, Voermans NC. Neurological manifestations of Ehlers-Danlos Syndrome(s): A review. Iran J Neurol 2014; 13(2):190-208.

Castori M, Tinkle B, Levy H Grahame R, Malfait F, Hakim A. 2017. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet Part C Semin Med Genet 175C:148-157.

Celletti C, Camerota F, Castori M, Censi F, Gioffrè L, Calcagnini G, Strano S. Orthostatic intolerance and postural orthostatic tachycardia syndrome in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type: neurovegetative dysregulation or autonomic failure? 2017. Biomed research international. https://www.hindawi.com/journals/bmri/2017/9161865/. Accessed May 9, 2017.

Chopra P, Tinkle B, Hamonet C, Gompel A, Bulbena A, Francomano C. 2017. Pain management in the Ehlers-Danlos syndromes. Am J Med Genet Part C Semin Med Genet 175C:212-219.

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Hakim A, De Wandele, I, O’Callaghan C, Pocinki A, Rowe P. 2017. Chronic fatigue in Ehlers-Danlos syndrome–hypermobile type. Am J Med Genet Part C Semin Med Genet 175C:175–180.

Hakim A, De Wandele, I, O’Callaghan C, Pocinki A, Rowe P. 2017. Cardiovascular autonomic dysfunction in the Ehlers-Danlos syndromes. Am J Med Genet Part C Semin Med Genet 175C:168-174.

Hamonet, C. Maladie ou syndrome d’Ehlers-Danlos: une entité clinique, d’origine génétique, malconnue, dont la rareté doit être remise en question. http://claude.hamonet.free.fr/fr/art_sed.htm.

Lyons J, Yu X et al. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nature Genetics 48(12) Dec. 2016:1564-71. http://www.nature.com/articles/ng.3696.epdf?referrer_access_token=kkD-p2pE8cDDL5CD1Ld3HtRgN0jAjWel9jnR3ZoTv0OLzluKQm19ggXVIEhkOhCoL5WeqBE-2NbzNX250zrSY6saf84ClYXe5QCd6Ml8cJXVyO3IwXe2Fg4xQ2imE5j1rUq84b2v8A1zoVGfzqLaTQJ6z9dgZGnPiDeryqKtlDpJVhh0NIkqcozh-yfwtnAdAN6giFaq71aut6Qsm1ZpgBrmjA3KjU2o7jzSbKd1fITd17-KdARtzZXFDf1hWK-m_s7nJK205uewy3iCUhXEv-4EuCSglFcyFlC2qRO52P3zOWLHspxFbUdRoxJ54gYUnzdZRBr6uWpabZI8upyTKVnsbjROAiBKUZI-smYd7Cs%3D&tracking_referrer=solvecfs.org.

Henderson Sr. FC, Austin C, Benzel E, Bolognese P, Ellenbogen R, Francomano CA, Ireton C, Klinge P, Koby M, Long D, Patel S, Singman EL, Voermans NC. 2017. Neurological and spinal manifestations of the Ehlers-Danlos syndromes. Am J Med Genet Part C Semin Med Genet 175C:195-211.

Lyons J, Yu X et al. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nature Genetics 48(12) Dec. 2016:1564-71. http://www.nature.com/articles/ng.3696.epdf?referrer_access_token=kkD-p2pE8cDDL5CD1Ld3HtRgN0jAjWel9jnR3ZoTv0OLzluKQm19ggXVIEhkOhCoL5WeqBE-2NbzNX250zrSY6saf84ClYXe5QCd6Ml8cJXVyO3IwXe2Fg4xQ2imE5j1rUq84b2v8A1zoVGfzqLaTQJ6z9dgZGnPiDeryqKtlDpJVhh0NIkqcozh-yfwtnAdAN6giFaq71aut6Qsm1ZpgBrmjA3KjU2o7jzSbKd1fITd17-KdARtzZXFDf1hWK-m_s7nJK205uewy3iCUhXEv-4EuCSglFcyFlC2qRO52P3zOWLHspxFbUdRoxJ54gYUnzdZRBr6uWpabZI8upyTKVnsbjROAiBKUZI-smYd7Cs%3D&tracking_referrer=solvecfs.org. Accessed Dec. 28th, 2016.

Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, Bloom L, Bowen JM, Brady AF, Burrows NP, Castori M, Cohen H, Colombi M, Demirdas S, De Backer J, De Paepe A, Fournel-Gigleux S, Frank M, Ghali N, Giunta C, Grahame R, Hakim A, Jeunemaitre X, Johnson D, Juul-Kristensen B,Kapferer-Seebacher I, Kazkaz H, Kosho T, Lavallee ME, Levi H, Mendoza-Londono R, Pepin R, Pope FM, Reinstein E, Robert L, Rohrbach M, Sanders L, Sobey GJ, Van Damme T, Vandersteen A, van Mourik C, Voermans M, Wheeldon N, Zschocke J, Tinkle B. 2017. The 2017 international classification of the Ehlers-Danlos Syndromes. Am J Med Genet part C Semin Med Genet 175C:8-26.

Seneviratne SI, Maitland A, Afrin L.2017. Mast cell disorders in Ehlers-Danlos syndrome. Am J Med Genet Part C Semin Med Genet 175C:226–236.