Physiotherapy in SMA: Part 2: the changing patterns of SMA 1 – management, orthotics and equipment

Marion Main Consultant physiotherapist GOSH January 2021 [email protected] Learning objectives for part two

• The priorities of management of the child with SMA 1

• The patterns of weakness and the impact on function, contractures and

• The best forms of exercise play and activity

• The most useful equipment and orthotics

• The things we don’t do and use and why The weakest muscle groups

• Neck and trunk flexors • retractors

• extensors/abductors/ • Shoulder flexors/abductors adductors • Elbows (extensors and • extensors flexors)

• Dorsiflexors • Wrist extensors

• Thumb and finger extensors The most important take home messages

• SMA is about weakness

• Low tone is not the problem – weakness is

• Contractures WILL happen

• They don’t get better by themselves

• FATIGUE is real in SMA! The most important take home messages

• No Lycra for spines – no strength, no proof, no tummy hole

• No hamstring stretches – children need SHORT hamstrings (if you actually know what a normal hamstring is…)

• No unnecessary insistence on tummy time – there are so many better positions to work in for weaker children New treatments are prolonging survival and changing natural history

With these therapies in SMA, new patterns of development are emerging and we have to manage changes in unpredictable outcomes and new natural histories. New physical challenges in SMA 1 • We must achieve more than survival – we need quality with our quantity:-

• Exercise and strengthening through play and activity

• Management of contractures

• Management as far as possible of spine and posture

• ?

• Sitting and seating

• Function, ADL and Independence Management • Contractures/range of movement

• Exercise/activity

• Position – lying, sitting, standing

• Posture

• Pain prevention

• Mobility

• Orthotics Communication

Difficulties with communication can be problematic for some of the infants and older children:

- A number of the children are non-- Speech can be very nasal and verbal or have limited speech, opportunity or loudness difficult to understand

- including those on 24hr mask and lack of good communication ventilation can

- with a tracheostomy affect their ability or desire

- limited head movement to co-operate with assessment Management o f Joint Range Its not all about contractures!

î CONTRACTURES

î HYPERMOBILITY

î ASYMMETRY Not all contractures are the same! Different factors can will impact on the amount of stretch in a muscle (and joint, and in the tendons, ligaments, and the nerves can shorten…….)

Muscle Atrophy Injury Muscle Fibrosis Pain Paralysis/weakness Previous immobility – Fixed deformity post fracture Contractures

• can appear at any age & stage, including being present from birth in the weakest children :

• They can occur in any joint – including the neck, spine and jaw

• They can appear on one side or both

• They can progress rapidly or slowly Contractures Neck ()*, spine

Elbows, forearms, wrists (including deviation), fingers

Hips

Knees

Feet = ankles/hind-feet/mid-foot

Jaw

Other joints *made worse by plagiocephaly Why?

§ Weakness

• The joints are not moved regularly through full range as in normal function

§ Persistent posturing occurs; e.g. hands are pronated – held palm down, and hips are mostly in the bent position, feet hang downwards

§ It's not only joints that get tight, muscles, tendons, nerves and even fibrous structures can shorten, adding to the problems They will deteriorate with growth if not well managed

The range gained can easily be lost again if you don’t think about how to maintain it

®splinting and/or exercises

Poor posture needs correcting - habits cannot be changed easily…….what looks normal to us may not feel normal to the child - the “sit straight trap”

Pain makes contractures worse Why bother if they keep getting worse?

• They greatly interfere with function

• ADL,

• seating,

• mobility,

• play….

• They cause pain

• They can cause scoliosis Management

• Stretches

• – preferably ACTIVE ASSISTED

• Splinting (but not taping – though it can be used to help maintain range gained by other means…)

• Serial casting

• Positioning

• Surgery

– Or a combination…. Stretches - How to stretch! CAREFULLY!

- make that VERY Carefully! PARTICULARLY FEET And knees if painful Stretches – how much “force”

•Feel the muscle – is there any stretch/give Make sure you are stretching in the line of muscle pull •Stretch SLOWLY •Think about the normal range – don’t try and get more!! • Never cause pain. •If you’re doing active assisted, you shouldn’t need force Active assisted stretches – why? • To stop the child pushing, therefore much less likely to cause injury

• To physiologically relax the opposing muscles

• To exercise the muscles that so often do not get the opportunity Hips in SMA (not a small topic)

• Botox???

• Impact of or on standing • To consider - • Increased power of hip flexors • Contractures or hypermobility! • HIPS are connected to the pelvis • Subluxation/Dislocations at one end and the knees and feet at the other……..they • Dysplasia cannot be ignored

• Pain (effect of spinal surgery) • Monitoring???

• Surgery? Positioning? Bracing? • When and IF to intervene What NOT to stretch! Bendy joints

Fixed joints

Painful joints

Hamstrings

(certainly not in SMA) What about hamstrings? what is a tight hamstring?

Popliteal Angle: Surgery? • What?

• For who?

• And the post op/recovery process? What are we doing during this period?

• How long (if) in plaster? Surgery is a treatment • What effect will this have – not a cure: there on knees, feet, spine? will still be muscle imbalance – • Where – is every centre DESPITE surgery able to do this? surgery Surgery is still necessary in some cases! – more so now with improved function and survival

(but not for hamstrings)

And there is nothing more important than getting splints/orthoses right after surgery! Current thoughts - surgery in SMA not including spines

• Evidence shows relocating hips does not work

• Straightening knees does not work

• TA releases can help in ambulant children and severe

• Hip flexor releases may be helpful in problems of pain, posture or hygiene. Taping for contractures……

Tape does not cure contractures, control or maintain range on it own…. With work, it can be used to try encourage movement and maintain range Is there a role for taping in SMA1

• Taping is not a competition to see who can use the most

• It is not useful to try and tape too many parts at once

• Taping requires some background knowledge – there are good ways and bad ways to do it

• The internet does not have all the answers

• It can be useful in SMA 1…… hands, sprains, pain, under casts…… Weakness is the major problem for all babies, infants and children with SMA.

Not tone! But SMA is not a muscle problem (its neurogenic) – you wont damage the muscle by exercising - in fact – unlike muscular dystrophies…. Exercise helps What do we know about exercise? exercise programmes areboring… we believe in play, sport activity

• PLAY IS EXERCISE!

• Important exercise

• And fun! Exercise

• No matter how weak the child, they can do exercise!

• No matter how contracted they are, they can do exercise

• So, if muscle is damaged or weak – what are we working on? A large part of the older child’s problem is disuse atrophy

Use it or lose it!

in What exercise? • Play, lots of play and activity (but not too much phone and iPad…..)

• PNF

• Theraband

• Swimming/hydrotherapy

• Therapy ball/peanut/roll

• (not tummy time!) Exercise should be Symmetrical Aerobic EFFECTIVE Achievable fun Swimming/hydrotherapy Alternatives to tummy time How much? How often? Just remember fatigue Little and often Every day Manageable for everyone That includes parents, carers and siblings Lying, sitting standing Scoliosis – the facts! • All non walking children with SMA, and those now surviving infants who never sit, will develop scoliosis –

• But the weakest non-sitters may not be operable!

• Rapid growth periods are often the worst times for increase in scoliosis

• A good TLSO will slow the rate of progression and improve symmetry in a young child

• Growing rods are the first surgical treatment Spinal Posture: can physiotherapy help?

• Ensure good sitting posture:

• and comfortable and functional

• Level pelvis

• Orthotics

• Symmetry of contractures

• Stretch?

• Exercise? in SMA Why and how do we deal with it?

Hamstrings pull the pelvis into posterior tilt To maintain an upright posture the child uses the upper trunk to counterbalance and stabilise Management is not to stretch hamstrings – Take the stretch off Take them OFF the floor! A wedge, step or seat Factors affecting posture.

Power (asymmetry) The Surface

Contractures Function Position Pelvic Obliquity Hip subluxation/dislocation Thoracic rotation Time/ fatigue

Pain SMA 1 Spines More problems - Neck tightness and kyphosis How best to manage …?

X-rays and monitoring …? Sitters and non-sitters? Pain and prevention

• Children

• Parents/carers Causes of pain

• Muscle pain

• Joint stiffness/Joint deformity

• Back pain?

• Pressure Sores

• Fractures/micro-fractures

• Bad physio! What causes pain in parents and carers

• Poor posture

• Bad lifting techniques

• Repetitive strain

• Lack of exercise

• Injuries Promote mobility Mobility

We need to know how to ensure that all children (when possible) have full independent mobility Sitting and seating The problems –

Respiratory issues can make sitting difficult

Lack of head control making it hard to support the head

Getting them in and out of seating

Amount of equipment needed around the child

Contractures, plagiocephaly, scoliosis,

Pain and immobility

Few seating professionals have worked with SMA 1 Electric wheelchairs notMobility suitable for the weakest infants

Children too weak to use the controls

Cant sit for long enough

Don’t have the space for the equipment

No ramps to the property

No suitable vehicle

Parents don’t like child in the back of a car…. Dynamic stander The increasing need for splints and orthotics What orthotics?

• Neck • Knees

• Elbows • Feet

• Hands/wrists • Jackets/TLSO

• Lycra is there any role for it? We need an understanding of what we want to achieve and why!

What do we expect from and orthosis?

• Standing or ambulation

• Contracture management, maintain/improve?

• Joint stabilisation/control

• Postural control

• Pain management You need an experienced team • Team work: therapist, doctor (and/or) orthopaedic/spinal surgeon, orthotist

• An understanding of the condition

• An understanding of what you want to achieve

• A n orthotist experienced in NMDs

• Accept that it may not be possible

• Accept it may not work! (and be prepared to try again)

• Budgets the aims of using orthoses

w prevent, control, reduce deformity

w facilitate standing and walking

w maintain joint control and/or stability w control, maintain, improve spinal posture SMA KAFOs A spinal jacket must have a tummy hole

There is NO evidence for Lycra – it cannot hold a weak child with SMA Learning objectives for part two

• The priorities of management of the child with SMA 1

• The patterns of weakness and the impact on function, contractures and scoliosis

• The best forms of exercise play and activity

• The most useful equipment and orthotics

• The things we don’t do and use and why No two children and families are the same Dietician You are not the most Wheelchair important person services Psychologist SCHOOL: Teachers, LSA, SENCO, Physio(s) Careers advisor Life?

OT(s) Sport & Local council Leisure Other Speech therapist Parents relatives

Social worker The young Siblings GP person

Community Paediatrician friends Consultant(s) There is no such thing as a lazy child - the main problem for many of these type 1 children is fatigue (or boredom, or demoralised from trying with no success, or not well, or terrible twos….) But this is often forgotten when handling these babies and infants!

Active assisted stretches are essential- Contractures don’t get better by themselves • Make them manageable

• Make them pain-free

• Make them regular

• Make them effective

• Make them active

• No amount of stretching will work by itself Physiotherapy is boring – stretches are boring

Use the method that works best for each child and family and maintains length for the maximum possible time - Splinting, positioning, casting... The most important take home messages

• SMA is about weakness

• Low tone is not the problem – weakness is

• Contractures WILL happen

• They don’t get better by themselves

• FATIGUE is real in SMA! The most important take home messages • No lycra for spines– no strength, no proof, no tummy hole

• No hamstring stretches – children need SHORT hamstrings (if you actually know what a normal hamstring is…)

• No unnecessary insistence on tummy time – there are so many better positions to work in for weaker children What we do now - is the result we will have for the future • Bone health, contractures, posture – DONT WAIT until its too late to correct them

• Anticipate and prevent where possible

• The new treatments don’t cure contractures or mend scoliosis

• – this takes regular hard work Thank you

To all the amazing children and parents who have taught me just about everything I know, to all my amazing physiotherapy colleagues (past and present); and the wider Dubowitz Neuromuscular team.