Physiotherapy in Neuromuscular Disorders

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Physiotherapy in Neuromuscular Disorders

• Marina Di Marco
• Principal Neuromuscular Physiotherapist
• West of Scotland
• April 2013

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The challenges of treating a progressive condition

• 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

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Evidence-based Practice

• Clinical effectiveness, clinical governance and
  evidence based practice underpin quality
  assurance within the NHS (Barkham et al, 2001)
• 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).

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Practice-based Evidence

• 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.
• 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).

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Patients are now transitioning to adult services.
Adult services are in a unique position to gather
information on this new and emerging population.
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.
This information will form the basis of research
and education within this group
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Assessment

• Subjective Examination
• Social history
• Who stays at home
• Work / Further Education/ School
• Medical history
• 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 well-being)
• Determine the familys ability to engage with
  service provision.

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Assessment

• Objective Examination
• 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, AFOs, spinal jacket)

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Treatment Model

• When treating the child with DMD you are in fact
  treating the family (Siegel, 1978)

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Treatment and Management

• Stretches and Exercise
• 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 Disorderswhen
  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.

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Fatigue Management

• Fatigue in muscle disorders can be progressive,
  variable and persistent.
• 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
• 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

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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.
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LOW MOOD DISTURBED SLEEP
PAIN
FATIGUE
INACTIVITY
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Postural management

• Dynamic Postural Management
• 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
• Symmetry, frequent changes in position, avoidance
  of prolonged static postures.
• Standing perch, alternate supporting leg, lean
• Sitting postural support, tilt, recline
• Lying supine, side lying, bed, mattress

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Falls Management

• Assessment Muscle Strength, Fatigue, Pain,
  Eyesight, Balance, Sensation, Age and Stage
• Management Orthoses, Activity, Equipment, Self
  management
• Prevention Assistance out of doors, Wheelchair,
  Education

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Pain Management

• 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.

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The pain message

• 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.

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Types of Pain

• 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 NSAIDs (Non steroidal
  anti-inflammatories) such as Ibuprofen.
• 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
  anti-depressants or anti-epileptic drugs.

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Assessment

• Physiotherapy
• Postural Management
• Behavioural Change
• Heat, Cold, Vibration, Electrotherapy,
  Acupuncture, TNS, Massage
• Stretches and Activity
• Relaxation
• Fatigue Management
• OT
• Aids and adaptations
• CBT

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Pain Management

• Healthy Lifestyle
• Diet
• Alcohol
• Smoking
• Sleep Hygiene and Sleep Quality
• Psychological factors
• Support groups
• Distraction
• Coping mechanisms
• Mood

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Conclusion

• 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.

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marina.dimarco_at_nhs.netTel 0141 354 9205