Dysautonomia and Fatigue Management! ! ! ! ! ! Dr Jane Simmonds MCSP SFHEA! Physiotherapy Lead: London Hypermobility Unit Programme Lead Physiotherapy: Institute of Child Health, University College London

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY How my interest started

Referrals with a combined diagnosis dysautonomia and Hypermobility Syndrome / Ehlers Danlos Syndrome – Hypermobility type (HSD and hEDS)

• Paediatrician - Dr Nelly Ninis

• Rheumatologists - Professor Rodney Grahame

Dr Hanadi Kazkaz

• Neurovascular physician - Prof Christopher Mathias

‘Jane can you help here – can you help get him/ her moving please’

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Common symptoms

Profound fatigue (poor sleep) Racing heart (tachycardia) and feeling faint • When standing in assembly or in cue at supermarket • Standing up too quickly • Travelling on the underground • Light exercise • After eating a large meal/ carbohydrates Colour changes / blotchy – feet and hands - venous pooling Nausea Irritable bowel type symptoms Recurrent urinary tract infections Allergies - rashes Sent home from school or work – reduced attendance

2003

Symptoms related to the autonomic nervous system such as; • Syncope, pre-syncope (near fainng/ fainng) • Palpitaons, tachycardia, chest discomfort • Fague • Heat intolerance Significantly more common among people with JHS than healthy controls

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Outlined a number of potenal mechanisms a suggested cause in hypermobile individuals

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Survey of 946 adults Hypermobility Syndrome / Hypermobile Ehlers Danlos Syndrome

Self Report Co exisng Condions

Undifferenated skin & atopic phenomena

Neurological/neurodevelopment

Urogenital/ women's health

Fague and sleep related disorders

Metabolic and nutrional

Autoimmune Condion

Gastrointesnal

Cardiorespiratory/cardiac dysautonomia

Musculoskeletal

Mental health

0 5 10 15 20 25 30 35 40 45 50 25% Sick Listed Percentage 25% Completed terary educaon Simmonds et al 2017

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Case Control Study: 19 cases of POTS and 16 healthy controls 3 month graduated exercise intervenon Results • 10/19 cases no longer met the diagnosis of PoTS • Significant reducon in upright heart rate • All improved quality of life (SF36)

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Definion Postural tachycardia syndrome is an abnormality of the funconing of the autonomic (involuntary) nervous system.

Flight and fright Rest and restore

Sympathecally driven Sympathec Para sympathec

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Definion Postural tachycardia syndrome is an abnormality of the funconing of the autonomic (involuntary) nervous system.

1. To be diagnosed with POTS, an individual must experience a group of symptoms in the upright posion (usually standing) that are relieved by lying down

2. A persistent increase in heart rate of 30 beats per minute (40 bpm if under 19 years of age) should be recorded within ten minutes of standing. Blood pressure (BP) does not always drop in POTS

Sympathecally driven

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Diagnosis

10 Minute quiet standing test (Raj 2013)

3 – 7 day blood pressure and heart rate monitoring and diary

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Walking

Chair Management! Empower and take control of the condion

Educaon • Reassure (not a heart aack) • Pacing – fague and pain management (diary)

Sleep management • Sleep roune • Dark room • Timing of food and exercise • Reduce screen me • Sleepio App*

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Avoid

• Rapid postural change • Standing/sing too long • Prolonged bed rest • Excessive straining, heat, alcohol • Large carbohydrate meals

Medicaons • Increase blood volume - fludrocorsone • Increase vasoconstricon - midodrine • Block effect of (nor)epinephrine – beta-blockers, ACE-1

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY POTS / EDS Funconal Rehabilitaon

Morning routine ◦ Medication and isotonic drink

◦ Heel raises, bicep curls with weights, squats (as able) - modify Cardiovascular exercise (5 per week)

◦ Aiming for …..30 minutes low intensity (may start as little as 30 second intervals) ◦ 35% Maximum heart rate (meaningful) / BORG RPE ◦ Recumbent to vertical ◦ Fun and relevant/ choice Musculoskeletal • Joint stability/ motor control programme (daily) – strength training (2-3 times per week) MAYO Paent informaon. 2009 • Manual therapies Fu et al. Hypertension. 2011. 58, 167-175 Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY

Case One!

21 year old man (gied musician)

Onset of symptoms aged 18 aer viral infecon • Diagnosed with chronic fague syndrome • Graded walking and physical acvity programme – unsuccessful • 15 minutes walking – set back Gastrointesnal symptoms – intermient reflux, bloang, Lightheaded aer eang – especially carbohydrates Back, knees and ankle pain – associated with physical acvity also food* Fague++ (associated with altered sleep paern) Sought further invesgaons Diagnosed with POTS Joint Hypermobility Syndrome (Hypermobility Spectrum Disorder)

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY

IMPACT Unable to leave the house, poor social relaonships, loss of hope

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY IMPACT Unable to leave the house, poor social relaonships, loss of hope

Management Psychiatry – escitalopram Counceling

Non pharmacological management for POTS • Fluid (isotonic), salt • Compression ghts • Dietary advice – low carbs, small meals • Physiotherapy – exercise • Podiatry • Nutrional advice

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Physical Rehabilitation

Listen: explore why previous rehabilitation attempts had failed

Holistic view – FUNCTION and PARTICIPATION

Agree goals (physical activity and life goals) Address barriers and fears – set backs (build resilience)

Physical therapy (coach) • Cardiovascular exercise (lower limb dominant), movement control, muscle strengthening • Pacing* and progression • Created the plan together

Morning routine

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY

15 months

Inially 2-3 weekly appointments

6-8 weekly

Case Two! 16 Year old girl – two year history of POTS History of glandular fever

Unable to walk Home schooled Local rehabilitaon – bike (no progress over 6 months)

Recurrent bladder infecons Menoned skin rash Food allergies Symptoms worse menstrual cycle Underlying joint hypermobility – lower limb symptoms Exhausted* sleep

Previously – healthy, happy, thriving Case Two! 16 Year old girl – two year history of POTS History of glandular fever

Unable to walk Home schooled Local rehabilitaon – bike (no progress over 6 months)

Recurrent bladder infecons Menoned skin rash Food allergies Symptoms worse menstrual cycle Underlying joint hypermobility – lower limb symptoms Exhausted* sleep

Previously – healthy, happy, thriving Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Fague Provide sleep hygiene and informaon on pacing, fluids, Vit D, Iron checks, diet Pain Work with psychologists / Provide pain Counseling – CBT/ hypnosis educaon, pacing, Psychological acupuncture Monitor fluid, Salt, Medicaons Graded cardiovascular / lower limb exercise Neuromusculoskeletal Dysautonomia

Educaon/graduated motor control/ strength/ endurance/ splints/tape/ adaptaons/ manual therapy/ podiatry/ Monitor medicaons/low occupaonal therapy carbohydrates/ FODMAP Medicaons

Women’s health Contracepve pill Monitor, Diet Gastrointesnal Urogenital Medicaons Symptom Severity Mast Cell Ninis,de wandeleSimmonds Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Case Two! Management Complex pharmacological management for POTS and Mast Cell Acvaon • Fluids and salt • Diet – low histamine & FODMAP • Schooling and exam plan • Psychology Goals • Commence daily exercise: floor based – pool – gym (reviewed 3-4 weekly basis) • Be able to go shopping on the high street – ( 4 months) • Complete 5 GCSE’s (somehow) – 8 months • Stay over with friends house (6-8 months) Funconal restoraon programme Normalise movement • Hydrotherapy (x 3 per week) • Gait re educaon • Graduated cardiovascular and resistance training • Bike, cross trainer, squats, weights arms and legs, leg press

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY ‘There are many more ups ‘I am now at 6th Form College than downs these days. I have reduced my medicaons, but and proud to have completed do sll need to manage my one A level and planning to stress levels.’ finish two more’

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY Summary – top tips

Seek information Dysautonomia International or POTS UK website

Find a team who understands POTS and EDS Find therapists who you can trust and work with Learn about nutrition Set realist – meaningful physiological and life goals

Graduated exercise programme Start low, go slow – persist – carry on

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY THANK YOU

Ehlers-Danlos Society Learning Conference, December 2018 SPONSORED BY