Physiotherapy in
Neuromuscular Disorders
Marina Di Marco
Principal Neuromuscular Physiotherapist
West of Scotland
April 2013
The challenges of treating a
progressive condition
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Goalposts are constantly changing in both the
paediatric and adult condition
Emotive condition
Multi-disciplinary / Trans-disciplinary Team
Working
Neuromuscular Disorders – Speciality in its
own right
New and Emerging populations
Evidence-based Practice
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Clinical effectiveness, clinical governance and evidence –
based practice underpin quality assurance within the NHS
(Barkham et al, 2001)
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However, until this population becomes established within
the healthcare system, healthcare providers are effectively
treating and managing a condition, which is relatively
unknown in its teenage and adult form. In order to effectively
manage this patient group, healthcare providers will need to
adopt an innovative approach whilst working within the
parameters of a limited evidence base (Di Marco, 2013).
Practice-based Evidence
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The evidence-based practice paradigm is difficult to emulate if there is no
critical mass within a peer group to undertake systematic evaluation of
therapeutic interventions.
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Practise-based evidence has been described as documenting and
measuring real world practice as it occurs, “warts” and all (Swisher A,
2010).
Healthcare practitioners can systematically collect evidence from
treatment and management of individual cases in order to inform the
future practice of healthcare.
Qualitative inquiry will be essential to collect practice-based evidence and
the development of an effective conceptual framework will be key
(Leeman et al, 2012).
Patients are now
transitioning to
adult services.
Improvements in paediatric
healthcare have led to
improved survival in DMD
New research and information will
enable paediatric healthcare providers
to evaluate treatment and management
protocols which will inform the
development of healthcare
improvement.
Adult services are in a
unique position to
gather information on
this new and emerging
population.
This information will
form the basis of
research and education
within this group
Assessment
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Subjective Examination
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Social history
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Medical history
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Who stays at home
Work / Further Education/ School
Anyone in the family with the same condition / other conditions
Surgery
Other clinics / professionals involved (Cardiac, Respiratory, orthopaedic,
Endocrinology)
Medication (Which day in steroid cycle?)
Orthoses
Pain
A day in the life…. (ADL, Bowel / Bladder, Fatigue, Falls, Sense of wellbeing)
Determine the family’s ability to engage with service provision.
Assessment
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Objective Examination
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North Star (ambulatory) / EK (Non – ambulatory)/
SMArtnet
Muscle Strength (Muscle Stamina)
Joint ranges
Sensation / Circulation
Respiratory assessment
Spine
Gait Analysis
Mobility – Wheelchairs and Seating
Moving and Handling
Orthoses (insoles, AFO’s, spinal jacket)
Treatment Model
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When treating the child with DMD you are in
fact treating the family (Siegel, 1978)
Treatment and Management
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Stretches and Exercise
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Exercise V Activity: Dispelling the myths
Benefits of Activity: Raises low mood disorder, prevents disuse
atrophy, improves sleep, improves circulation, helps control weight,
BP, helps prevent co-morbidities)
Varying the activity to avoid muscle adaptation
Graded exercise in Neuromuscular Disorders…when and where?
Man V Machine: Be wary of asymmetrical stance and muscle
imbalance.
There is something to suit everyone: Stretches, Aerobic Activity,
Anaerobic Activity, Passive / Passive assisted Movements.
Fatigue Management
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Fatigue in muscle disorders can be progressive,
variable and persistent.
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Progressive – Gets worse as the day / week goes on / with repetitive
activity.
Variable – Can be different from day to day or hour to hour.
Persistent – Once stamina is lost, the patient may never be able to
regain it.
Fatigue Management:
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Increased risk of trips and falls
More stress on soft tissue due to joints working at a mechanical
disadvantage.
Increase in pain and inactivity
Repetitive activities are more difficult – 3 attempts
Fatigue Management Pathway
Pacing yourself on a daily basis is a good habit to get into but it is to pace yourself over the period of the week. If you work part
time, try and have a rest day in between rather than clustering your working days together. If you are going out on an evening, try
and structure it so that the following morning will not be too energetic.
Start to undertake your daily activities with awareness. Find out what makes you tired
or causes you pain. Everyone is different and in fact, your energy levels will be different
on a day-to-day basis and it is important that you ‘tune’ in to how your muscles are
performing so that you may adapt your activity accordingly.
If there are specific activities within your working day that cause you fatigue, discomfort
or pain, speak to your teacher / line manager if at work to see if these can be changed.
This could be lifting objects, sitting / standing in the same spot for extended periods of
time or undertaking a repetitive activity including walking.
If there are tasks that you are unable to change, try and undertake them for a shorter
period of time and then switch to another task. Come back to the original task and then
switch again. Breaking down tasks into smaller chunks is also a good strategy to
undertake at home too. If you are on your feet sit down frequently and if you are
sitting, stand up and move around as often as you are able.
Break your day into three segments, morning, afternoon and evening. It is good practice
to try and work your day so that if you have two busy segments in your day, you have at
least one segment where you can chill out. If you work a full day, you may want to take
it easy in the evening and not do housework. If you do housework in the morning, try
and rest during the afternoon particularly if you have a family who will need their
dinner when they come in from school. If there is more than one person in your
household, ask them to help with daily tasks and spread the load…….Learn to Delegate!
LOW MOOD
DISTURBED SLEEP
PAIN
FATIGUE
INACTIVITY
Postural management
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Dynamic Postural Management:
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Less efficient movement induces pain and fatigue as
muscles tire quicker.
It becomes more difficult to respond to changes in
balance, speed and direction as muscles are already
working hard.
Orthoses, walking aid, wheelchair may be required.
Static Postural Management
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Symmetry, frequent changes in position, avoidance of
prolonged static postures.
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Standing – perch, alternate supporting leg, lean
Sitting – postural support, tilt, recline
Lying – supine, side lying, bed, mattress
Falls Management
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Assessment – Muscle Strength, Fatigue, Pain,
Eyesight, Balance, Sensation, Age and Stage
Management – Orthoses, Activity, Equipment,
Self management
Prevention – Assistance out of doors,
Wheelchair, Education
Pain Management
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Pain can be a challenging symptom for people with a neuromuscular
disorder. As muscles become weaker, joints are pulled into postures that
may not be mechanically advantageous and this can cause pain.
Muscles gradually weaken as people get older but if they are already a bit
weaker to start with, the ageing process can cause specific challenges to
joint health.
If unable to move frequently and change position often, patients will be
prone to pain caused by pressure as well as experiencing fatigue in
muscles particularly the hips, back, neck and shoulders.
Understanding Pain: Pain can be a complex area to understand. No two
people experience pain in the same way and for some a simple cut can be
very sore while others can cope with serious surgery in much the same
way. The amount of pain we feel is not always in proportion to the amount
of tissue damage we see.
The pain message
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Pain receptors: Pain, Pressure, Temperature
The spinal cord works as a filter and will only send messages of pain to the
brain when they reach a certain level that the body perceives as a threat
to our health.
Chronic Pain: If pain persists, the brain will try to learn more about it and
it will create more pain receptors to help do this. More pain messages are
delivered to the spinal cord which reacts by sending more messages to the
brain. The more messages the brain receives, the more intense is the pain
reaction. This means that for some people, only a small amount of
movement or pressure can produce quite a large reaction.
Coping with chronic pain is about moving the pain to a more manageable
level. For example if pain is present every day, perhaps it is possible to
start working towards having some pain free days. If the pain is very
intense (i.e. 9 or 10 on a scale from 1 to 10) then perhaps it is possible to
bring it down to a 3 or 4.
Types of Pain
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Nocigenic: This type of pain is a result of stimulation of certain receptors
in bones, joints and muscles. These nociceptors are sensitive to tissue
injury. People describe Nocigenic pain as being sharp, aching or throbbing.
This type of pain is pain such as trauma, pressure, osteoarthritis and it
responds well to analgesics such as Paracetomol and NSAID’s (Non
steroidal anti-inflammatories) such as Ibuprofen.
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Neurogenic: This type of pain is due to a problem with the nervous
system. The nerves may not work properly and can cause a burning
sensation, a hypersensitivity (i.e. people may feel pain on light touch) or
there may be altered sensation such as paraesthesia or anaesthesia. This
type of pain occurs due to nerve dysfunction, neuralgia or a neuropathy
(such as in diabetes). It responds best to medication such as antidepressants or anti-epileptic drugs.
Assessment
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Physiotherapy:
Postural Management
 Behavioural Change
 Heat, Cold, Vibration, Electrotherapy, Acupuncture,
TNS, Massage
 Stretches and Activity
 Relaxation
 Fatigue Management
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OT:
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Aids and adaptations
CBT
Pain Management
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Healthy Lifestyle:
Diet
 Alcohol
 Smoking
 Sleep Hygiene and Sleep Quality
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Psychological factors:
Support groups
 Distraction
 Coping mechanisms
 Mood
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Conclusion
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In rare conditions, it is not always possible to
work within an evidence – based paradigm.
All healthcare professionals are in a unique
and privileged position to document
treatment and management of patient
pioneers.
It is as important to treat and manage the
family as it is the condition.
marina.dimarco@nhs.net
Tel: 0141 354 9205