Dysautonomia and Fatigue Management...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

SPONSORED BY Ehlers-Danlos Society Learning Conference, December 2018

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


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’

How my interest started

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

Common symptoms


Symptoms related to the autonomic nervous system such as; •  Syncope, pre-‐syncope (near fain9ng/ fain9ng) •  Palpita9ons, tachycardia, chest discomfort •  Fa9gue •  Heat intolerance Significantly more common among people with JHS than healthy controls

2003




Outlined a number of poten9al mechanisms a suggested cause in hypermobile individuals



Simmonds et al 2017

0 5 10 15 20 25 30 35 40 45 50

Mental health

Musculoskeletal

Cardiorespiratory/cardiac dysautonomia

Gastrointes9nal

Autoimmune

Metabolic and nutri9onal

Fa9gue and sleep related disorders

Urogenital/ women's health

Neurological/neurodevelopment

Undifferen9ated skin & atopic phenomena

Percentage

Cond

i9on

Self Report Co exis2ng Condi2ons

25% Sick Listed 25% Completed ter2ary educa2on

Survey of 946 adults Hypermobility Syndrome / Hypermobile Ehlers Danlos Syndrome


Case Control Study: 19 cases of POTS and 16 healthy controls 3 month graduated exercise interven2on Results •  10/19 cases no longer met the diagnosis of PoTS •  Significant reduc9on in upright heart rate •  All improved quality of life (SF36)


Defini2on Postural tachycardia syndrome is an abnormality of the func9oning of the autonomic (involuntary) nervous system.

Sympathe2cally driven Sympathe2c Para sympathe9c

Flight and fright Rest and restore


Defini2on Postural tachycardia syndrome is an abnormality of the func9oning of the autonomic (involuntary) nervous system.

1. To be diagnosed with POTS, an individual must experience a group of symptoms in the upright posi9on (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

Sympathe2cally driven


Diagnosis

10 Minute quiet standing test (Raj 2013) 3 – 7 day blood pressure and heart rate monitoring and diary

Walking

Chair


Management!Empower and take control of the condi2on Educa2on •  Reassure (not a heart aaack) •  Pacing – fa9gue and pain management (diary)

Sleep management •  Sleep rou9ne •  Dark room •  Timing of food and exercise •  Reduce screen 9me •  Sleepio App*


Avoid

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

Medica2ons •  Increase blood volume -‐ fludrocor9sone •  Increase vasoconstric9on -‐ midodrine •  Block effect of (nor)epinephrine – beta-‐blockers, ACE-‐1


POTS / EDS Func2onal Rehabilita2on

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) •  Manual therapies

MAYO Pa9ent informa9on. 2009 Fu et al. Hypertension. 2011. 58, 167-‐175


Case One!

21 year old man (giQed musician)

Onset of symptoms aged 18 aker viral infec9on •  Diagnosed with chronic fa9gue syndrome •  Graded walking and physical ac9vity programme – unsuccessful

•  15 minutes walking – set back Gastrointes9nal symptoms – intermiaent reflux, bloa9ng, Lightheaded aker ea9ng – especially carbohydrates Back, knees and ankle pain – associated with physical ac9vity also food* Fa9gue++ (associated with altered sleep paaern)

Sought further inves/ga/ons Diagnosed with POTS Joint Hypermobility Syndrome (Hypermobility Spectrum Disorder)


IMPACT Unable to leave the house, poor social rela2onships, loss of hope


IMPACT Unable to leave the house, poor social rela2onships, loss of hope

Management Psychiatry – escitalopram Counceling

Non pharmacological management for POTS •  Fluid (isotonic), salt •  Compression 9ghts •  Dietary advice – low carbs, small meals •  Physiotherapy – exercise •  Podiatry •  Nutri9onal advice


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

15 months

Ini9ally 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 rehabilita9on – bike (no progress over 6 months)

Recurrent bladder infec9ons Men9oned 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 rehabilita9on – bike (no progress over 6 months)

Recurrent bladder infec9ons Men9oned skin rash Food allergies Symptoms worse menstrual cycle Underlying joint hypermobility – lower limb symptoms Exhausted* sleep

Previously – healthy, happy, thriving



Gastrointes2nal

Dysautonomia

Fa2gue

Psychological

Pain

Urogenital

Mast Cell

Neuromusculoskeletal

Work with psychologists / Counseling – CBT/ hypnosis

Monitor fluid, Salt, Medica9ons Graded cardiovascular / lower limb exercise

Monitor medica9ons/low carbohydrates/ FODMAP Medica9ons

Women’s health Contracep9ve pill

Provide sleep hygiene and informa9on on pacing, fluids, Vit D, Iron checks, diet

Monitor, Diet Medica9ons

Provide pain educa9on, pacing, acupuncture

Educa9on/graduated motor control/ strength/ endurance/ splints/tape/ adapta9ons/ manual therapy/ podiatry/ occupa9onal therapy

Symptom Severity Ninis,de wandeleSimmonds

Management Complex pharmacological management for POTS and Mast Cell Ac9va9on •  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) Func2onal restora2on programme Normalise movement •  Hydrotherapy (x 3 per week) •  Gait re educa9on •  Graduated cardiovascular and resistance training •  Bike, cross trainer, squats, weights arms and legs, leg press

Case Two!



‘There are many more ups than downs these days. I have reduced my medica/ons, but do s/ll need to manage my stress levels.’

‘I am now at 6th Form College and proud to have completed one A level and planning to finish two more’


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


THANK YOU

Documents

Dysautonomia and Fatigue Management...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