Non-pharmacological treatments in EDS

Isabelle Brock, MD

QoLify Chief Scientific Officer

Indiana University, Dep of Medical and Molecular Genetics, Visiting Scientist

Clinical Paradigms, Director of Research

Maggie Buckley, MBA, BCPA

Disclaimer

u Nothing to disclose

Comprehensive Integrative Pain Management

u Comprehensive integrative pain management (CIPM) includes biomedical, psychosocial, complementary health, and spiritual care.

u It is person-centered and focuses on maximizing function and wellness.

u Care plans are developed through a shared decision-making model that reflects the available evidence regarding optimal clinical practice and the person’s goals and values.

Pain what is it?

Neurological Construct:

sensationperception due

toneuroanatomicalor physiological

disorder; the

unexplained is“psychiatric in

origin”

Psychological Concept:

sensation withcomplex set of

modulatoryinfluences from

emotional,environmental

and psychophysiological

factors

Pain definition

• “An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue damage” (IASP 2020)

• Pain is “whatever the experiencing person says it is, existing whenever the person says it does”. (McCaffery, 1968)

Allostasis vs Homeostasis

Homeostasisu In biology, homeostasis is the state of

steady internal, physical, and chemical conditions maintained by living systems. This is the condition of optimal functioning for the organism and includes many variables, such as body temperature and fluid balance, being kept within certain pre-set limits (homeostatic range)

Allostasisu "remaining stable by being variable“u Supply and Demand

Non-pharmacological treatments for pain in EDS

a. Integrate systematically in a multimodal approachi. Useful for all types of painii. May be effective alone for some types of pain or low intensity pain ratings

(<4/10)

b. All treatments (including no tx) have risks as well as benefits

c. Assessment is keyi. What has been used in the past? ii. Has it been successful? If not, why not? iii.What is the individual willing to try next?

d. Some complimentary therapies not covered by insurances

e. Is the person with pain willing and able to pay out of pocket?

ECSS-62: the 16 families of clinical manifestations are grouped into 6 axes on the basis of physiopathological hypotheses

1. Skin sensitivity, integuments and teeth 2. Bleeding susceptibility3. Proprioceptive sensory and

sensorimotor disorders, with respiratory control and sensitivity (pain)

4. Dysautonomia with fatigue, digestive and vesico-sphincteric disorders

5. Sensory perception disorders (hearing, vision, olfactory modality, vestibular balance control )

6. Cognitive disorders

3 and 4 axis interconnection

Rehabilitative Techniques

u Physical Therapyu Splints / bracing / tapingu Pacing

u PhysiatryuCompression garmentsuExoskeleton

uOccupational TherapyuExercise Physiology

Focused Techniques

a. Exerciseb. Hydrotherapy (Balneotherapy): Heat and Coldc. Myofascial Therapyd. Osteopathic Manipulation Treatment (OMT)e. Transcutaneous Electrical Nerve Stimulation (TENS)

Hydrotherapy (Balneotherapy): Heat and Cold

u Icingu Compressesu Baths

uHot tubsuSitz BathsuCold bathsuEpsom Salt Baths

(Magnesium Sulfate)

u Cold Friction Rubsu Counter-Irritants

uCamphoruMentholuMedicinal Clays

u Alternating vs. Combination

Additional Physical Methods

u Chiropracticu Osteopathicu Craniosacral Therapyu Feldenkrais (Functional

Integration)u Rolfing (Structural

Integration)u Trager Approach (somatic

education) Psychophysical

integrationu Pilatesu Yoga Therapyu Tai Chiu Mindfulness / Body

Awarenessu Singingu Oxygen therapy

Repartition of respiratory symptoms

u This study compared 826 occupational medicine breast patients with 681 EDS patients.

u 82% presented with dyspnea,

u 65% with respiratory blockages,

u 72% with recurrent upper airway conditions,

u 52% with recurrent bronchitis and

u 47% with dysphonia.

u It is more or less the same distribution as in studies done before.

Oxygen diffusion in the tissue

u EDS patients have a remarkably low tissue oxygen diffusion (finger photoplethysmographic perfusion index) (study in progress)

u Pain (cold pressor test) reduces tissue oxygen saturation in all measurement sites (except cerebral) and perfusion index. In the presence of pain, tissue oxygen saturation and perfusion index are further reduced by hypovolemia (lower body negative pressure, -60 mm Hg). Thus, pain must be considered when evaluating tissue oxygen saturation and perfusion index as markers of hypovolemia in trauma patients

RFE restrictive, measurements without clothes

Oxygen therapy

Different in every country whether it is prescription or available on the market

3xday 2-5L/min oxygen concentrator with humidifier low decibel

Stabilizes mast cells who tend to overreact in hypoxic tissue conditions

Mitochondria (our energy producing factories within the cells) need it in their last step of the electron transport chain to make one ATP our energy molecule.

Pentad of symptoms

u Pain u fatigue u headachesu dyspnea from very little exercise and u inspiratory bradypneas u mostly gets relieved with oxygen therapy 3 to 5 L of oxygen 3 times 20

minutes per day u This pentad exists in 44% of the EDSu 71.89% have at least 4 of the 5 symptoms with a specificity of 99.0%

Non-pharmacological sensory therapies

u Aromatherapyu Nutrition: herbal, organicu Homeopathyu Art therapyu Music therapyu Humor therapyu Visualization

Nutritionu Nutritional

counselingu Supplemementsu Balanced dietu How to make

individualized food choices

u MCAD/MCASu Low histamine

dietu Avoid food

triggers

Psychosocial interventions

u Psychological Consultation, Counseling & Stress ManagementuRelaxation Therapy

(relaxation, mindfulness, imagery)

uBiofeedback TraininguBehavioral ModificationuStress Management TraininguHypnotherapyuCounseling (individual, family

or group)

Comprehensive Integrative Pain Management

u Alternative Medical Systems

u Homeopathic medicine or homeopathy

u Naturopathic medicine or naturopathy

u Traditional Chinese Medicine (TCM)u Acupunctureu Acupressure

u Reflexologyu Ayurveda

Energy Therapies

u Touch (Biofield) TherapiesuQiGonguHealing TouchuReikiuTherapeutic Touch

u Bioelectromagnetic-based therapiesuMagnet Therapy

Thank YouResearch@connective-tissue-issues.com

References

u Bruce S. et al, What’s in a name? Integrating homeostasis, allostasis and stress , Horm Behav. 2010 February ; 57(2): 105. doi:10.1016/j.yhbeh.2009.09.011.

u Bovet, Claire, Matthew Carlson and Matthew Taylor. Quality of life, unmet needs, and iatrogenic injuries in rehabilitation of patients with Ehlers-Danlos Syndrome hypermobility type/Joint Hypermobility Syndrome. Journal: American Journal of Medical Genetics Part A, 2016, Volume 170, Number 8, Page 2044.

u Guinchat V, Vlamynck E, Diaz L, Chambon C, Pouzenc J, Cravero C, Baeza-Velasco C, Hamonet C, Xavier J, Cohen D. Compressive Garments in Individuals with Autism and Severe Proprioceptive Dysfunction: A Retrospective Exploratory Case Series. Children. 2020; 7(7):77.

u Hamonet C. et al, Ehlers-Danlos Syndrome (EDS) type III (hypermobile): validation of a somatosensory clinical scale (ECSS-62), about 626 cases. Bull. Acad. Natle Méd. 2017, 201, n°2.

u Cypel D. Gleno-humeral abduction measurement in patients with Ehlers-Danlos syndrome, Orthopaedics & Traumatology: Surgery & Research 105 (2019) 287–290

u Celletti, C et al, Use of the Gait Profile Score for the evaluation of patients with joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type, Research in Developmental Disabilities 34 (2013) 4280–4285

u Mollander P. et al, Ehlers–Danlos Syndrome and Hypermobility Syndrome Compared with Other Common Chronic Pain Diagnoses—A Study from the Swedish Quality Registry for Pain Rehabilitation J. Clin. Med. 2020, 9, 2143; doi:10.3390/jcm9072143

References

u Castori M., A. Hakim (2017) Contemporary approach to joint hypermobility and related disorders. Current Opinion in Pediatrics, Volume 29, Number 6, December 2017 Curr OpinPediat 2107,29:640-649

u Brissot R et al. Ehlers-Danlos and respiratory function. Clinical data on a cohort of 5,700 patients: oxygen therapy and physical rehabilitation medicine (P.R.M.). J Neuroscience and Neurological Surgery. 6(3) 2020;

u Hamonet C. et al, Dystonia and its Treatment in Ehlers-Danlos Syndrome, J Alzheimers Parkinsonism Dementia (2018), Volume 3 • Issue 1 • 026

u Reine Bueno et al., Custom-Made Foot Orthoses Reduce Pain and Fatigue in Patients with Ehlers-Danlos Syndrome. A Pilot Study , Int. J. Environ. Res. Public Health 2020, 17, 1359; doi:10.3390/ijerph17041359

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References

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