Author: Mary Rudd

Physiotherapy and Neuromuscular Disease

Neuromuscular Disease (British Thoracic Society Physiotherapy Guideline Development Group)

The diagnosis of Neuromuscular Disease covers a wide range of disorders that give rise to progressive muscular weakness. Common diseases in this category include motor neuron disease also known as amyotrophic lateral sclerosis in the USA, multiple sclerosis, spinal muscular atrophy, congenital myopathy, postpoliomyelitis and muscular dystrophies, the most common of these being Duchenne muscular dystrophy. They vary in age of onset, rates of progression and patterns of muscles involved.

Patients with neuromuscular disease may have a reduced Vital Capacity (VC) (the greatest volume of air that can be expelled from the lungs after taking the deepest possible breath) due to reduced inspiratory muscle strength, with resultant underventilation of lung units and microatelectasis (partial collapse of the lung that is only visible by microscope). The combination of loss of respiratory muscle strength, ineffective cough and decreased ventilation could lead to pneumonia, atelectasis (partial collapse of the lung), and respiratory insufficiency and failure. Additionally, the unstretched chest wall muscles shorten and stiffen due to the pathological process of disease and an inability to be worked through the full range. Such patients will frequently have an inadequate cough due to weakness in inspiratory and expiratory muscles.

Maximum insufflation capacity is the maximum volume of air that can be held with a closed glottis (the vocal apparatus of the larynx). This may be achieved via air stacking for the patient who can maintain a closed glottis. Air stacking involves consecutive inhalations, closing the glottis after each one, to create a maximum insufflation. Patient representatives report improvements in their perception of cough strength and secretion management when using unaided breath stacking. Performing maximal insufflation prior to coughing will increase inspiratory volume and consequently the expiratory flow and cough efficiency.

Subjects with a larger maximum insufflation capacity/VC difference consequently have a greater ability to clear airway secretions, thus decreasing the risk of pulmonary complications. Maximal insufflation to Inspiratory Capacity (IC) also provides a full range of movement to the lungs and chest wall. This technique performed 15 times three times daily increased maximum insufflation and subsequent peak cough flow when commenced in patients with neuromuscular disease and reduced Vital Capacity.

Physiotherapy encompasses more than ‘‘tipping and bashing’’ and the integrated approach of physiotherapy embraces a wide variety of techniques, including: breathing re-education, dyspnoea management, physical training and pulmonary rehabilitation, airway clearance, and non-invasive ventilation.

Multiple Sclerosis, Transverse Myelitis and FES

An interview with Dr. Daniel Becker – Assistant Professor of Neurology at Johns Hopkins, and the Director of the International Neurorehabilitation Institute in Baltimore, Maryland.

Dr. Daniel Becker trained as an adult neurologist, and completed his residency at Vanderbilt University in Tennessee. He came to John Hopkins in 2007 for a fellowship in spinal cord injury medicine and stayed. He was the Director of paediatric spinal cord injury unit at Kennedy Krieger Institute before becoming the Director of the International Neurorehabilitation Institute in Lutherville, Maryland in 2013.  There the focus is on multiple sclerosis, spinal cord injury, transverse myelitis, and cerebral palsy, amongst other conditions leading to weakness and spasticity.  He is also one of the two physicians who run the Johns Hopkins Transverse Myelitis Center where he sees both adults and children.  At the Consortium of Multiple Sclerosis Centers (CMSC) 2012 annual conference he presented a poster titled “Activity Based Rehabilitation May Preserve Function in Multiple Sclerosis”.

His original neurorehabilitation training was mainly focused on traumatic spinal cord injury (SCI).  When he began seeing patients with multiple sclerosis (MS), and transverse myelitis (TM) at the Johns Hopkins Centre he started realizing the potential of advanced neurorehabilitation for this patient population.  It was noted that these patients seem to attain equal benefit from the implementation of a Functional Electrical Stimulation (FES) program, as do the traumatic SCI patients.

Multiple Sclerosis Study: This study set out to see whether an FES program; or FES in an Activity-Based Restorative Therapy (ABRT) program can help improve, or at least stabilise, function in individuals with the progressive form of Multiple Sclerosis. The natural course of Progressive Multiple Sclerosis, is one of deterioration, as there is no pharmacological therapy available for this type of Multiple Sclerosis.

In Multiple Sclerosis the patients are separated based on the progression of their disease. For example, there is the relapsing remitting form that most people associate with MS. Relapsing Remitting Multiple Sclerosis (RRMS) is characterized by occasional relapses or attacks which results in some functional loss.  Over time people regain some or most of this function back.  Currently, all available MS medications only work in this phase of the disease.

After being in this relapsing remitting phase for about 10 years, up to 80% of patients will find their MS developing into the progressive form (Progressive Multiple Sclerosis).  In this phase, the nervous system tends to lose the ability to repair in between attacks and there is a noticeable slow and steady decline.  In this phase there are no pharmacological treatments available.

There is another group of patients who develop a Primary Progressive form of Multiple Sclerosis (PPMS). In this case, there are no true relapses.  People continuously decline in function, some faster and some slower. There is also no pharmacological treatment available to help prevent disease progression.

The study was to establish whether an ABRT program utilizing FES, would stabilize or even improve the function of people with progressive MS. The key finding of this paper was able to show that most people were not getting any worse over a 2 year period.  Some of them even recovered function.

Being as active as possible is the key for stabilizing the disease and may even help to recover some lost function.  However being active is not just about exercising and going to the gym and lifting weights. What we have learned over the last few years, and this study supports it, is that FES has to be included in the exercise program.  Dr. Becker therefore advocates for its use in every single MS patient.  Restorative Therapy’s RT300 is currently the most efficient device they use to deliver functional electrical stimulation.

In an earlier Johns Hopkins study using FES, Dr. Douglas Kerr demonstrated a decrease in inflammatory markers and an increase in neuro-protection or repair markers.  There is a biochemical link between FES and how it translates into recovery of function that they have set out to find.  When they do, they hope to be able to make this intervention more efficient and hopefully available to all MS patients.

Transverse Myelitis (TM) is, in the majority of cases, a single inflammatory event affecting the spinal cord, which causes paralysis. It can be the initial presentation of MS.  In MS there occur repeated inflammatory events injuring the spinal cord and the brain. So we believe TM is an important model for a single spinal cord injury. We therefore can extrapolate the findings from TM into MS and vice versa.

FES revolves around the concept that people have all of the tools for repair already within their spinal cord and brain. But there is something inherent to the spinal cord environment that prevents these cells from optimally repairing.  It’s an environmental issue within the spinal cord.  So why do they not repair?  For stem cells to effectively repair, they first have to recognize that there is an injury.  Then they have to move to the injury site.  Once there they have to differentiate into a glial cell like an oligodendrocyte and start repairing.

However, this is what happens: the stem cells recognize that there is an injury.  They are able to move to the injury.  However, then they just sit there like bumps on a log.  What the cells need to see is that the nerves they are supposed to repair are actively firing. If they are not firing, they cannot see them and therefore not know that repair is necessary.  So how can we make them fire? We use functional electrical stimulation. By activating those nerves, the oligodendrocytes now know where to do the repair.

This is the chronic process that never stops.  However, it takes a while and therefore has to be built into one’s life. So Dr. Becker recommend to his patients to use FES for one hour 3 to 5 times a week.

What we do

We believe that people with neurological injuries such as Multiple Sclerosis, Transverse Myelitis, Cerebral Palsy, and Spinal Cord Injury, require intensive, specialized therapy.  Subsequently, our therapists utilise activity based restorative therapy (ABRT) as practised by the Kennedy Krieger Institute. ABRT is based on the concept that the nervous system requires an optimal level of patterned activity in order to simply maintain itself as well as to maximize response of regeneration after injury. We attempt to promote neurological recovery by stimulating the nervous system above and below the level of injury through intensive activity. Many different techniques to achieve this important stimulation of the nervous system, one of which is the use of functional electrical stimulation (FES). This is the type of therapy received by Christopher Reeve during his rehabilitation. Clients at our practice often receive one to three hours of physical therapy depending on their specific needs.  All sessions are one on one, patient to therapist, and include specific interventions that are tailored to the patient’s needs.

What is FES (Functional Electrical Stimulation)?

Functional electrical stimulation (FES) is a technique that uses electrical currents to activate the nerves innervating extremities affected by paralysis resulting from spinal cord injury (SCI), head injury, stroke and other neurological disorders.  FES is used primarily to restore function in people with disabilities.  www.restorative-therapies.com/faqs

The first commercially available FES devices treated foot drop by stimulating the peroneal nerve during gait. A switch, located in the heel end of a user’s shoe, activates a stimulator worn by the user.

Injuries to the spinal cord result in paralysis below the level of injury. Restoration of limb function, regulation of organ function, treatment of pain, and pressure sore prevention are the main applications.  Functional electrical stimulation of those with paraplegia promotes muscle activity in the affected areas.  FES for ambulation shows improvements in blood flow to lower extremities and also positively affects bowel and bladder function.  The FES cycle ergometer stimulates the muscles in a gait pattern.

In the acute stage of stroke recovery, the use of electrical stimulation has been seen to increase the isometric strength of wrist extensors which may decrease the extent of upper extremity disability.  Patients with hemiplegia following a stroke commonly experience shoulder pain and subluxation; both of which will interfere with the rehabilitation process. Functional electrical stimulation has been found to be effective for the management of pain and reduction of shoulder subluxation, as well as accelerating the degree and rate of motor recovery. Furthermore, the benefits of FES are maintained over time; research has demonstrated that the benefits are maintained for at least 24 months. (Chantraine, Alex; Baribeault, Alain; Uebelhart, Daniel; Gremion, Gerald (1999). “Shoulder Pain and Dysfunction in Hemiplegia: Effects of Functional Electrical Stimulation”. Archives of Physical Medicine and Rehabilitation 80: 328–331.)

Electrical stimulation can also be used for central nervous system stimulation to hasten awakening from coma or the vegetative state. FES offers a variety of health benefits. Like any exercise FES improves fitness, prevents obesity and helps to protect against cardiovascular disease. FES cycling however provides some other extra benefits:

  • Improved circulation, decreasing the likelihood of thrombosis.
  • Prevents or reverses muscle atrophy.
  • Reduction of muscle spasms
  • Maintaining or increasing range of motion

And it may assist in an increase in bone density, decreasing the risk of fractures.

RT300

No. 2 in my arsenal of equipment is the RT300 hand and leg cycle.  Using this regularly can decrease spasticity, improve awareness of limbs, decrease swelling, elevate mood due to endorphin release from physical activity and improve sleep quality.

 

RT600 Functional Electrical Stimulation (FES)

Functional Electrical Stimulation (FES) is a physical therapy rehabilitation modality used to stimulate physical activity not otherwise possible for individuals with a neurological impairment such as a spinal cord injury (SCI), stroke, multiple sclerosis, brain injury or cerebral palsy. RT600 is an FES partial bodyweight stepping system, capable of accommodating a wide range of patients, and used in the rehabilitation of these neurological conditions.

RT600 integrates FES and partial body weight support technologies with motor controlled footplates which produce a “virtual ground” for gait. Electrical currents stimulate nerves which activate core and leg muscles including the abdominals and erector spinae, quadriceps, hamstrings, gluteals, gastrocnemius, anterior tibialis, to evoke stepping activities. This rehabilitation therapy enables a patient’s paralyzed or weak legs to move through patterned physical activity utilizing their own muscles while safely positioned in a partial body weight supporting harness.

“The RT600 is the first physical therapy system to bring the established benefits of FES to weight supported stepping for people with neurological impairments,” says Professor David Ditor of Brock University, in Ontario, Canada.

“It is the first truly practical rehabilitation system of this kind that I have seen. In addition to combining several valuable neuro-rehabilitation interventions, functional electrical stimulation, locomotor gait training and neuromuscular re-education, the RT600 is small and easy enough to use that I can one day envision it in the patient’s home,” said Cristina Sadowsky of the Kennedy Krieger Institute in Baltimore, Maryland.

“The RT600 finally makes partial body weight supported stepping integrated with FES available to a wide range of clinics at an affordable price point,” says Andrew Barriskill, CEO of Restorative Therapies. “We are excited to be introducing this world first therapy to a large group of patients who currently have limited physical activity options.”

We are proud to announce that this therapy is now available at Mary Rudd Physiotherapy, Hillcrest, Kwazulu Natal, South Africa

Off to Baltimore for training

On Saturday 14 June 2014, Felicity Whitton (Nutty) and I are off to Baltimore for training at the Restorative Therapies Clinical Training Centre on the RT600. I will also attend an advanced training on the SAGE controller – the FES Powered Systems Course for clinicians.  Local patients volunteer to support the program, which will provide excellent “hands on” training opportunities.  These courses will ensure that we can use both the RT300 and RT600 to their full extent, and confidently establish therapy progression.

We will also be visiting the Kennedy Krieger Institute which is an internationally recognized institution dedicated to improving the lives of children and adolescents with pediatric developmental disabilities and disorders of the brain, spinal cord and musculoskeletal system.

While I am on the 2 1/2 day clinician course, Nutty will be wandering around Baltimore, looking for “soft crab” which she has been informed is a delicacy there.  My main site-seeing goal is the Barnes and Noble bookstore which is a 10 minute walk from the hotel.