Jane Simmonds Rehab Sept 17

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Rehabilitation for PoTS

PoTS UK Masterclass

Dr Jane Simmonds MCSP MACP SFHEA


D Prof, MA, PGDip, B App(Sc), BPE
Programme Lead: MSc Paediatric Physiotherapy
Physiotherapy Lead Hypermobility Unit, Hospital of St John and St Elizabeth
Plan

•  Share an approach to rehabilitating people with PoTS


•  Integrating key PoTS exercise related research
•  Anecdotal experience
•  Case studies
.
Acknowledgments
Patients
Dr Nelly Ninis
Professor Christopher Mathias
Professor Rodney Grahame
Dr Alan Hakim
Dr Hanadi Kazkaz
Dr Inge de Wandele
Dr David Low
Professor Peter Rowe
.
Interest started with complex referrals
•  Joint laxity/ hypermobility / joint instability (hEDS/ HSD)
•  Persistent widespread pain
•  Persistent fatigue
•  Pre syncope and sometimes fainting
•  Temperature dysregulation
•  Gastrointestinal symptoms - reflux, slow transit
•  Bladder symptoms – irritable bladder, incontinence
•  Allergies - rashes
Onset
Musculoskeletal
Pain
Cardiac Dysautonomia

Fa,gue
Mental Health

Bladder GI manifesta,ons

Allergy/MAC systemic severity scale


Ninis, de Wandele & Simmonds 2013
Musculoskeletal
Pain
Cardiac Dysautonomia

Fa,gue
Mental Health

Bladder GI manifesta,ons

Allergy/MAC systemic severity scale


Ninis, de Wandele & Simmonds 2013
Musculoskeletal
Pain
Cardiac Dysautonomia

Fa,gue
Mental Health

Bladder GI manifesta,ons

Allergy/MAC systemic severity scale


Ninis, de Wandele & Simmonds 2015
Musculoskeletal
Pain
Cardiac Dysautonomia

Fa,gue
Mental Health

Bladder GI manifesta,ons

Allergy/MAC systemic severity scale


Ninis, de Wandele & Simmonds 2013
Problem based approach - Explore expecta7ons - Choice
Rehabilitation Principles
Education:
Reassurance
Pacing – fatigue and pain management
Agree and set realistic goals
Anxiety management
•  Cognitive Behavioural Therapy, Relaxation, Mindfulness, Hypnosis
•  Hyperventilation - Breathing Exercises
Sleep management
•  Sleep routine
•  Timing of food and exercise
•  Screen time
•  Sleepio App
Monitor medications: often complex cocktail
Advise on non pharmacological treatments
•  Compression garments – aid venous return
•  Fluids and salt – maintain blood volume
•  Dietary advice – small meals, low carbohydrate, FODMAPS
Design and implement exercise reconditioning programme*
Evidence suggests that orthostatic intolerance and PoTS
are related to deconditioning (Fu et al., 2010; Parsaik et al., 2012; Sheldon et al., 2016)

Cause or Effect?
Monitor medications: often complex cocktail
Advise on non pharmacological treatments
•  Compression garments – aid venous return
•  Fluids and salt – maintain blood volume
•  Dietary advice – small meals, low carbohydrate, FODMAPS
Design and implement exercise reconditioning programme*
Evidence suggests that orthostatic intolerance and PoTS
are related to deconditioning (Fu et al., 2010; Parsaik et al., 2012; Sheldon et al., 2016)

Cause or Effect?
Regardless of the relationship – deconditioning negatively
influences cardiovascular function
Premise: Long term benefits of improved physical fitness
counteract orthostatic intolerance
•  Increased blood volume
•  Increased cardiac output
•  Enhanced vascular compression due to increased muscle
mass and tone
•  Improved endothelial function
•  Improved baro-reflex function
Case Control Study: 19 cases of PoTS and 16 healthy controls
3 month graduated exercise interven7on
Results
•  10/19 cases no longer met the diagnosis of PoTS
•  Significant reduc,on in upright heart rate
•  All improved quality of life (SF36)
Growing evidence for exercise as alternative to medication

Side effects of medication – lead to cessation


•  Beta blockers – fatigue
•  Fludrocortizone – hypokalemia
•  Alpha adrenergic agonists - hypertension
Galbreath et al., 2016 Clin Auton Res, 21, 73-80
Exercise Reconditioning Programme
Aims : improve cardiovascular fitness and lower limb strength

Cardiovascular exercise
Start with chair peddles, reclining bicycle, rowing and swimming
Progress to upright position

During exercise, people with PoTS have a low stroke volume response to
exercise – leads to light headedness, dizziness, dyspnoea and weakness
Resistance / Strength Training
•  Body weight
•  Weights
•  Elastic bands

Resistance training is more demanding on the circulatory system. Lead to


changes in blood pressure. Avoid Static exercise and Valsalva hold breath
– increase in BP, followed by a fall in BP
How Often and How Hard? Rate of Perceived Exer7ons
Frequency and Intensity of Exercise
First month
3-4 per week: Reclining exercise CV training (20 - 40 min)
1 x per week: Resistance training per week (15 – 20 mins)
RPE 6 - 16
Second month
3-4 per week: Upright bike CV training (25 -34 mins)
2 x per week: Resistance training per week (20-25 mins)
RPE 6 - 18
Third month
3-4 per week: Upright CV training (35-40 mins)
Cross trainer/ walking
2 x per week: Resistance training (30 mins)
RPE 8 - 18
George et al., 2016 Heart Rhythm, 13, 943 - 50
My Reality - Frequency and Intensity of Exercise

First Month
4-5 per week: Reclining exercise CV training (2-10 mins)
4-5 x per week: Resistance & propriocep,on training per week
(2-10 mins)
RPE 6 – 9: Graduate by 10% per week
Second – Third Month
4-5 per week: Upright bike / Walking CV training (10 – 30 mins)
4-5 per week: Resistance & func,onal training per week (10 –
20 mins)
RPE 6-13
Fourth – Six Month
3-4 per week: Upright bike, Walking, Cross trainer (30-40 mins)
2 - 3 x per week: Resistance & func,onal training (20 mins)
RPE 9 - 18

George et al., 2016 Heart Rhythm, 13, 943 - 50


Musculoskeletal
Pain
Cardiac Dysautonomia

Fa,gue
Mental Health

Bladder GI manifesta,ons

Allergy/MAC systemic severity scale


Ninis, de Wandele & Simmonds 2015
Case Two
Management
Complex pharmacological management for PoTS and Mast Cell Ac,va,on
•  Fluids and salt
•  Dietary advice
•  Schooling plan

Func7onal restora7on programme
Normalise movement
Gait re educa,on
Hydrotherapy
Graduated cardiovascular and resistance training
Bike, cross trainer, squats, weights arms and legs, leg press

Goals
Complete GCSE’s
Be able to go shopping on the high street
Stay over with friends and holiday

Tips for Rehabilitation

Give hope
Holis,c view
Problem solving
Find the base line
Progress steady pace
Drink before, during and aber
Psychology
Graduate slowly
Underlying HSD – likely to be very weak and decondi,oned with porr
propriocep,on. Need to incorporate stability training.

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