Physiotherapy in Physiotherapy in Neuromuscular DisordersNeuromuscular Disorders

Marina Di MarcoMarina Di MarcoPrincipal Neuromuscular PhysiotherapistPrincipal Neuromuscular Physiotherapist

West of ScotlandWest of ScotlandApril 2013April 2013

The challenges of treating a The challenges of treating a progressive conditionprogressive condition

Goalposts are constantly changing in both the Goalposts are constantly changing in both the paediatric and adult conditionpaediatric and adult condition

Emotive conditionEmotive condition Multi-disciplinary / Trans-disciplinary Team Multi-disciplinary / Trans-disciplinary Team

WorkingWorking Neuromuscular Disorders – Speciality in its Neuromuscular Disorders – Speciality in its

own right own right New and Emerging populationsNew and Emerging populations

Evidence-based PracticeEvidence-based Practice Clinical effectiveness, clinical governance and evidence – Clinical effectiveness, clinical governance and evidence –

based practice underpin quality assurance within the NHS based practice underpin quality assurance within the NHS (Barkham et al, 2001)(Barkham et al, 2001)

However, until this population becomes established within However, until this population becomes established within the healthcare system, healthcare providers are effectively the healthcare system, healthcare providers are effectively treating and managing a condition, which is relatively treating and managing a condition, which is relatively unknown in its teenage and adult form. In order to effectively unknown in its teenage and adult form. In order to effectively manage this patient group, healthcare providers will need to manage this patient group, healthcare providers will need to adopt an innovative approach whilst working within the adopt an innovative approach whilst working within the parameters of a limited evidence base (Di Marco, 2013).parameters of a limited evidence base (Di Marco, 2013).

Practice-based EvidencePractice-based Evidence The evidence-based practice paradigm is difficult to emulate if there is no The evidence-based practice paradigm is difficult to emulate if there is no

critical mass within a peer group to undertake systematic evaluation of critical mass within a peer group to undertake systematic evaluation of

therapeutic interventionstherapeutic interventions.. Practise-based evidence has been described as documenting and Practise-based evidence has been described as documenting and

measuring real world practice as it occurs, “warts” and all (Swisher A, measuring real world practice as it occurs, “warts” and all (Swisher A, 2010). 2010).

Healthcare practitioners can systematically collect evidence from Healthcare practitioners can systematically collect evidence from treatment and management of individual cases in order to inform the treatment and management of individual cases in order to inform the future practice of healthcare.future practice of healthcare.

Qualitative inquiry will be essential to collect practice-based evidence and Qualitative inquiry will be essential to collect practice-based evidence and the development of an effective conceptual framework will be key the development of an effective conceptual framework will be key

(Leeman et al, 2012).(Leeman et al, 2012).

Improvements in paediatric healthcare have led to improved survival in DMD

Patients are now transitioning to adult services.

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

New research and information will enable paediatric healthcare providers to evaluate treatment and management protocols which will inform the development of healthcare improvement.

AssessmentAssessment Subjective ExaminationSubjective Examination Social historySocial history

Who stays at homeWho stays at home Work / Further Education/ SchoolWork / Further Education/ School

Medical historyMedical history Anyone in the family with the same condition / other conditionsAnyone in the family with the same condition / other conditions SurgerySurgery Other clinics / professionals involved (Cardiac, Respiratory, orthopaedic, Other clinics / professionals involved (Cardiac, Respiratory, orthopaedic,

Endocrinology)Endocrinology) Medication (Which day in steroid cycle?)Medication (Which day in steroid cycle?) OrthosesOrthoses PainPain

A day in the life…. (ADL, Bowel / Bladder, Fatigue, Falls, Sense of well-A day in the life…. (ADL, Bowel / Bladder, Fatigue, Falls, Sense of well-being)being)

Determine the family’s ability to engage with service provision.Determine the family’s ability to engage with service provision.

AssessmentAssessment Objective ExaminationObjective Examination

North Star (ambulatory) / EK (Non – ambulatory)/ North Star (ambulatory) / EK (Non – ambulatory)/ SMArtnetSMArtnet

Muscle Strength (Muscle Stamina)Muscle Strength (Muscle Stamina) Joint rangesJoint ranges Sensation / CirculationSensation / Circulation Respiratory assessmentRespiratory assessment SpineSpine Gait AnalysisGait Analysis Mobility – Wheelchairs and SeatingMobility – Wheelchairs and Seating Moving and HandlingMoving and Handling Orthoses (insoles, AFO’s, spinal jacket)Orthoses (insoles, AFO’s, spinal jacket)

Treatment ModelTreatment Model

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

Treatment and ManagementTreatment and Management Stretches and ExerciseStretches and Exercise

Exercise V Activity: Dispelling the mythsExercise V Activity: Dispelling the myths Benefits of Activity: Raises low mood disorder, prevents disuse Benefits of Activity: Raises low mood disorder, prevents disuse

atrophy, improves sleep, improves circulation, helps control weight, atrophy, improves sleep, improves circulation, helps control weight, BP, helps prevent co-morbidities)BP, helps prevent co-morbidities)

Varying the activity to avoid muscle adaptationVarying the activity to avoid muscle adaptation Graded exercise in Neuromuscular Disorders…when and where?Graded exercise in Neuromuscular Disorders…when and where? Man V Machine: Be wary of asymmetrical stance and muscle Man V Machine: Be wary of asymmetrical stance and muscle

imbalance.imbalance. There is something to suit everyone: Stretches, Aerobic Activity, There is something to suit everyone: Stretches, Aerobic Activity,

Anaerobic Activity, Passive / Passive assisted Movements.Anaerobic Activity, Passive / Passive assisted Movements.

Fatigue ManagementFatigue Management Fatigue in muscle disorders can be progressive, Fatigue in muscle disorders can be progressive,

variable and persistent.variable and persistent. Progressive – Gets worse as the day / week goes on / with repetitive Progressive – Gets worse as the day / week goes on / with repetitive

activity.activity. Variable – Can be different from day to day or hour to hour.Variable – Can be different from day to day or hour to hour. Persistent – Once stamina is lost, the patient may never be able to Persistent – Once stamina is lost, the patient may never be able to

regain it.regain it. Fatigue Management:Fatigue Management:

Increased risk of trips and fallsIncreased risk of trips and falls More stress on soft tissue due to joints working at a mechanical More stress on soft tissue due to joints working at a mechanical

disadvantage.disadvantage. Increase in pain and inactivityIncrease in pain and inactivity Repetitive activities are more difficult – 3 attemptsRepetitive activities are more difficult – 3 attempts

Fatigue Management PathwayPacing 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!

PAIN

FATIGUE

INACTIVITY

LOW MOOD

DISTURBED SLEEP

Postural managementPostural management Dynamic Postural Management: Dynamic Postural Management:

Less efficient movement induces pain and fatigue as muscles tire Less efficient movement induces pain and fatigue as muscles tire quicker.quicker.

It becomes more difficult to respond to changes in balance, It becomes more difficult to respond to changes in balance, speed and direction as muscles are already working hard.speed and direction as muscles are already working hard.

Orthoses, walking aid, wheelchair may be required.Orthoses, walking aid, wheelchair may be required.

Static Postural ManagementStatic Postural Management Symmetry, frequent changes in position, avoidance of prolonged Symmetry, frequent changes in position, avoidance of prolonged

static postures.static postures. Standing – perch, alternate supporting leg, leanStanding – perch, alternate supporting leg, lean Sitting – postural support, tilt, reclineSitting – postural support, tilt, recline Lying – supine, side lying, bed, mattressLying – supine, side lying, bed, mattress

Falls ManagementFalls Management

Assessment – Muscle Strength, Fatigue, Pain, Assessment – Muscle Strength, Fatigue, Pain, Eyesight, Balance, Sensation, Age and StageEyesight, Balance, Sensation, Age and Stage

Management – Orthoses, Activity, Equipment, Management – Orthoses, Activity, Equipment, Self managementSelf management

Prevention – Assistance out of doors, Prevention – Assistance out of doors, Wheelchair, EducationWheelchair, Education

Pain ManagementPain Management Pain can be a challenging symptom for people with a neuromuscular Pain can be a challenging symptom for people with a neuromuscular

disorder. As muscles become weaker, joints are pulled into postures that disorder. As muscles become weaker, joints are pulled into postures that may not be mechanically advantageous and this can cause pain.may not be mechanically advantageous and this can cause pain.

Muscles gradually weaken as people get older but if they are already a bit 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 weaker to start with, the ageing process can cause specific challenges to joint health.joint health.

If unable to move frequently and change position often, patients will be If unable to move frequently and change position often, patients will be prone to pain caused by pressure as well as experiencing fatigue in prone to pain caused by pressure as well as experiencing fatigue in muscles particularly the hips, back, neck and shoulders.muscles particularly the hips, back, neck and shoulders.

Understanding Pain:Understanding Pain: Pain can be a complex area to understand. No two 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 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 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 way. The amount of pain we feel is not always in proportion to the amount of tissue damage we see.amount of tissue damage we see.

The pain messageThe pain message Pain receptors: Pain, Pressure, TemperaturePain receptors: Pain, Pressure, Temperature The spinal cord works as a filter and will only send messages of pain to the 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 brain when they reach a certain level that the body perceives as a threat to our health.to our health.

Chronic Pain: IfChronic Pain: If pain persists, the brain will try to learn more about it and 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 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 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 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 pain reaction. This means that for some people, only a small amount of movement or pressure can produce quite a large reaction.movement or pressure can produce quite a large reaction.

Coping with chronic pain is about moving the pain to a more manageable 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 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 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 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.bring it down to a 3 or 4.

Types of PainTypes of Pain Nocigenic: This type of pain is a result of stimulation of certain receptors Nocigenic: This type of pain is a result of stimulation of certain receptors

in bones, joints and muscles. These nociceptors are sensitive to tissue in bones, joints and muscles. These nociceptors are sensitive to tissue injury. People describe Nocigenic pain as being sharp, aching or throbbing. injury. People describe Nocigenic pain as being sharp, aching or throbbing. This type of pain is pain such as trauma, pressure, osteoarthritis and it This type of pain is pain such as trauma, pressure, osteoarthritis and it responds well to analgesics such as Paracetomol and NSAID’s (Non responds well to analgesics such as Paracetomol and NSAID’s (Non steroidal anti-inflammatories) such as Ibuprofen.steroidal anti-inflammatories) such as Ibuprofen.

Neurogenic: This type of pain is due to a problem with the nervous 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 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 sensation, a hypersensitivity (i.e. people may feel pain on light touch) or there may be altered sensation such as paraesthesia or anaesthesia. This there may be altered sensation such as paraesthesia or anaesthesia. This type of pain occurs due to nerve dysfunction, neuralgia or a neuropathy type of pain occurs due to nerve dysfunction, neuralgia or a neuropathy (such as in diabetes). It responds best to medication such as anti-(such as in diabetes). It responds best to medication such as anti-depressants or anti-epileptic drugs.depressants or anti-epileptic drugs.

AssessmentAssessment Physiotherapy:Physiotherapy:

Postural ManagementPostural Management Behavioural ChangeBehavioural Change Heat, Cold, Vibration, Electrotherapy, Acupuncture, Heat, Cold, Vibration, Electrotherapy, Acupuncture,

TNS, MassageTNS, Massage Stretches and ActivityStretches and Activity RelaxationRelaxation Fatigue ManagementFatigue Management

OT: OT: Aids and adaptationsAids and adaptations CBTCBT

Pain ManagementPain Management Healthy Lifestyle:Healthy Lifestyle:

DietDiet AlcoholAlcohol SmokingSmoking Sleep Hygiene and Sleep QualitySleep Hygiene and Sleep Quality

Psychological factors:Psychological factors: Support groupsSupport groups DistractionDistraction Coping mechanismsCoping mechanisms MoodMood

ConclusionConclusion

In rare conditions, it is not always possible to In rare conditions, it is not always possible to work within an evidence – based paradigm.work within an evidence – based paradigm.

All healthcare professionals are in a unique All healthcare professionals are in a unique and privileged position to document and privileged position to document treatment and management of patient treatment and management of patient pioneers.pioneers.

It is as important to treat and manage the It is as important to treat and manage the family as it is the condition.family as it is the condition.

marina.dimarco@nmarina.dimarco@nhs.neths.net

Tel: 0141 354 9205Tel: 0141 354 9205