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REVIEW ARTICLE
Ahead of print publication  

Assistive devices: Regaining mobility in myositis


1 Department of Medicine, Byramjee Jeejeebhoy Medical College, Pune, Maharashtra, India
2 The Rehab Clinic, Pune, Maharashtra, India
3 Department of Medicine, Byramjee Jeejeebhoy Medical College; King Edward Memorial Hospital and Research Centre, Sardar Moodliar Road, Pune, Maharashtra, India

Date of Submission15-Apr-2020
Date of Acceptance28-Jun-2020

Correspondence Address:
Sanat Phatak,
Diabetes Unit, 6th Floor, Banoo Coyaji Building, King Edward Memorial Hospital and Research Centre, Sardar Moodliar Road, Pune - 411 011, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_90_20

  Abstract 


Assistive devices (ADs) refer to external devices adapted to improve tasks and function. The common types of ADs include those improving mobility such as wheelchairs and walkers, positioning devices such as standing frames, custom-made devices (orthotics) such as fitted shoes and braces, and daily living devices. In inflammatory myositis, ADs are of utility in combating weakness, improving mobility, preventing and treating contractures, preventing falls, and assisting in daily chores. This narrative review looks at the evidence for the use of ADs in myositis and disorders with a similar pattern of muscle weakness (e.g., muscular dystrophy) subsequent to a literature search. A range of devices, from ankle orthoses to robotic exoskeletons, has been used in children with these diseases, and is part of the rehabilitation process. Evidence for their use in inflammatory myositis comes mainly from inclusion body myositis where progression is usual, and distal movement loss additionally affects functionality. In these patients, gait ADs and lower limb orthoses have been shown to be useful. Patient acceptability of these interventions is paramount in choosing the correct device and fit. An interaction of the treating rheumatologist with the physiatrist, the physical therapist, the occupational therapist, and the patient is paramount in ensuring compliance and benefit.

Keywords: Assistive devices, muscle weakness, myositis, orthotics, physical therapy



How to cite this URL:
Davalbhakta S, Oswal A, Phatak S. Assistive devices: Regaining mobility in myositis. Indian J Rheumatol [Epub ahead of print] [cited 2020 Dec 4]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=298859




  Introduction Top


Assistive devices (ADs) are externally worn appliances that are developed to facilitate a specific task.[1] Many disabled individuals use ADs to perform actions essential to daily living, which allow them to engage actively in social life. Used appropriately, ADs go a long way in improving the quality of life in patients with neuromuscular disorders, including inflammatory myositis. ADs need to be carefully chosen and tailored to ensure that they address all individual requirements. Discomfort, secondary problems, and frustration can result from a poor selection of device.[1]

Despite optimum pharmacological treatment, patients with inflammatory myositis suffer from deficits in function.[2] We sought to review the utility and trends in the use of ADs in these patients, to introduce the rheumatologist to the principles of AD use and the wide repertoire of available technologies. Published, formal evidence in inflammatory myositis is scarce; thus, we expanded the scope of the review to other similar musculoskeletal conditions in order to be able to extrapolate the principles of use. We start by classifying the various kinds of ADs according to function; review AD evidence in diseases with proximal neuromuscular weakness; and finally apply these principles to the inflammatory myositis spectrum of disease.

A search was made in PubMed/MEDLINE from March 15, 2020, till April 1, 2020. The terms used in the search were assistive devices, orthotics, exoskeletons and inflammatory myositis, dermatomyositis, polymyositis, inclusion body myositis (IBM), muscular dystrophy, and neuromuscular weakness. The search results were reviewed, and only English language results were retained.


  Common Types of Assistive Devices Top


The range of ADs extends from basic-technology (e.g., walking sticks) to advanced-technology devices (e.g., specialized computer software/hardware or motorized wheelchairs).[3] It is helpful to consider this wide variety of AD under different categories:

Mobility devices

Mobility devices provide assistance while walking or moving and may involve wheelchairs, walkers, crutches, and walking sticks/canes. New technology in the form of exoskeletons and dynamic splints is being used recently.[1],[4]

Positioning devices

Individuals with physical disability often have improper positioning, which puts them at risk of developing contractures. Devices that can help overcome difficulties of positioning, such as sitting, standing, or lying down, include wedges, chairs (corner chairs, special seats), and standing frames.[1]

Orthotics

Orthotics are custom-made devices which replace, support, or correct body parts. Orthotic devices re-establish or enhance function by adjusting biomechanics through stabilizing, realigning, and/or maximizing joint position, thus reducing pain.[5],[6]

They are designed, manufactured, and fitted in specialized workshops or centers by trained prosthetic/orthotics personnel and include orthoses, for example, spinal braces, hand/leg splints or calipers, ankle foot orthoses (AFO) (knee AFO, hip KAFO), and orthopedic shoes.

Daily living devices

Activities required for daily life, such as eating, dressing, or bathing, are enabled by these devices.[1] Examples include adapted cutlery for eating, shower seats, shower stools, and raised toilet seats.[7],[8]


  Assistive Devices in Neuromuscular Weakness Top


Principles of the usage of AD remain more or less similar across diseases producing neuromuscular weakness, with special considerations in each category. As a whole, we take neuromuscular diseases to encompass a broad group of disorders affecting peripheral nerve or muscle function.[9]

The goals of using ADs in disorders with muscle weakness include:

  • Maintenance of strength and functionality of limbs
  • Assistance in mobility and weight bearing
  • Prevention of falls
  • Prevention and correction of joint deformity.


The choice of device depends on the pattern of muscle involvement (distal vs. proximal; laterality; and limb affected). Exoskeletons are new, sophisticated wearable devices providing external structural support and motorized mobility. They can be rigid (commonly metallic or carbon fiber) or pliable, and can aid in fine motor skills as well.[10] An initial comprehensive assessment is necessary to ensure that ADs meet the needs of individuals within their homes, schools, and work and community environments. A comprehensive assessment by a team headed by a physiatrist and a physical therapist includes a medical history, physical examination, a review of current function, range of motion, motion defects, individual goals, and an evaluation of the existing AD.[1]

The overall value of the use of ADs in patients with neuromuscular weakness is corroborated by a systematic review of 14 studies that compared patients' ability to perform certain tasks with and without AD, and showed a considerable improvement in activities of daily living in those using ADs[11] ADs of two types (End-effector and Exoskeleton, viz., worn externally, with joints and links placed in correspondence with human joints and bones) were evaluated, and devices included the Wimington robotic exoskeleton – a three dimensional printed robotic exoskeleton (Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delware), and Armon Edero – an arm support attached to the wheelchair, table, or jacket (Armon Products, The Netherlands).

ADs have been used widely in muscle dystrophies such as Duchenne muscular dystrophy (DMD)[12] and include knee-ankle-foot orthoses, serial casting, standing devices, and manual and motorized mobility devices. Technological innovations in the form of simple elevated lap trays to the more complicated robotics can go a long way in optimizing patient functioning. Smart phones, tablets, and voice activation software can be used to assist the patients' daily living.[13] Possible adaptive equipment and home renovations include patient hoists (lifts) for safe transfers, ramps, stair lifts, bathing and bathroom equipment or renovations, special beds and mattresses, and vehicle modifications.

The use of orthotics in DMD aids in ambulation and minimizes contractures. Duration of use is critical, as long-term use without other measures may lead to equinus foot and a hindering gait.[14] Night time use has an established association with prolonged gait ability and reduced functional decline.[15] AFO use during ambulation prevents ankle biomechanical compensations, preventing gait abnormalities.[16],[17]


  Assistive Devices in Inflammatory Myositis Top


Like muscular dystrophies, inflammatory myositis (polymyositis, dermatomyositis and IBM) mainly affects proximal musculature apart from IBM where distal motor involvement can also be prominent (e.g., calf muscles, finger flexors). Severe disease involves weakness of the neck flexors, respiratory musculature, and pharyngeal muscles causing dysphagia.[18] In polymyositis and dermatomyositis, the disease is characterized by an early “inflammatory” phase associated with raised creatine phosphokinase, muscle weakness, and inflammatory infiltrate on biopsy.[19] In the later “non-inflammatory” phase, muscle weakness may persist despite the resolution of inflammation and CPK levels. Muscle de-conditioning, atrophy, and noninflammatory causes of weakness such as the endoplasmic reticulum stress response are known contributory factors.[20] ADs, like intensive physiotherapy, have been used with caution in the early phase for the fear of worsening muscle inflammation, although more recent evidence has shown the benefit of exercise in all stages and types of disease.[21]

In theory and practice, one can envisage patients with idiopathic inflammatory myositis-related proximal muscle weakness benefitting with the use of an AD, although formal evidence is scarce [Figure 1]. Examples include a raised chair and toilet seat to aid standing with ease, a commode, safety bars on the bathroom wall, or a lift in the bath to provide security and optimize self-sufficiency with hygiene affairs.[5] Mobility devices, such as wheelchairs, walkers, crutches (axillary, forearm, and platform), walking sticks, and wheeled walkers, are used when walking is limited, or predominant in the lower extremity as compared to upper. They also help in case of joint contractures, pain, and fatigue or balance problems in residual weakness after treatment [Figure 2].
Figure 1: Optimizing the utility of assistive devices in inflammatory myositis includes assessments with a multidisciplinary team and choosing devices according to specific goals

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Figure 2: Devices useful in muscular weakness include a) standing frames, b) Ankle foot orthoses and c) Modified cutlery.

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These devices, though easily accessible and provide prompt assistance, require more physical effort than is required for ordinary functioning. Device type also impacts the weight-bearing status; thus, the choice is personalized after a thorough assessment with muscle charting. For example, 25% of the body weight can be borne by a single-point cane, axillary or forearm crutches about 50%, and a wheeled walker >50%. Wheelchairs become necessary when the patients cannot walk with devices. In these cases, wheelchairs have the potential to improve the quality of life by restoring stability. Persons unable to navigate a manual wheelchair can profit from an electrical chair.[5]

Most evidence of the utility of ADs in IIM comes from IBM. In a study with 53 patients with IBM, lower quantitative isometric knee extension strength was associated with a need for gait AD.[22] The results suggested that where knee extension strength declines below ~30 Nm (Newton-meter), it is likely the patient will be requiring or using a cane or trekking pole. An additional deterioration to <20 Nm signifies an increased degree of support (walker), and knee extension power nearing 5 Nm signifies wheelchair or scooter reliance. This could be a useful decision-making tool to prescribe an appropriate device.

During the swing phase of the gait, stance control knee-ankle foot orthoses (SCO) permit free knee flexion and during the stance phase, provide stability. It is generally acknowledged that free knee flexion during swing normalizes gait, thus reducing the energy needed for walking and improving speed.[23] Nine individuals with IBM were provided a stance control orthosis and followed for 6 months of home use.[24] Gait was assessed before use of the device and after 6 months using a 10-camera motion analysis system. Due to the unique weakness pattern and quadriceps weakness in IBM patients, knee buckling and falls are common complaints and an SCO may be especially helpful.

However, the results showed that the SCO had limitations on account of slower velocity, slower cadence, shorter and wider steps, more stance time asymmetry, and less knee flexion in swing with the SCO than without.[24] The survey results reported fewer falls and an increased feeling of stability while walking.

Literature on novel ADs such as dermoskeletons is scarce in the myositis group of disorders and is limited to case reports. A “Dermoskeletics” device was used to assist the patient's walking and was assessed for improvement in mobility in the patient.[25] Tests performed were a 6-min walk test and stair climbing. The device uses technologically advanced sensors and artificial intelligence to detect the patient's mobility intentions and engender synchronized support at the motorized knees. The device considerably improved the patient's walk test and stair-climbing capacity. Advancing bio-mechanical technology has a scope to preserve mobility and function in patients with neuromuscular diseases.

Contractures are known to occur in patients with juvenile dermatomyositis (JDM), more commonly as compared to adult myositis. Contractures refer to the reduction of range of motion across a joint, owing to the reduction of muscle/ligament elasticity and extensibility.[26] Contractures, unlike spasticity, involve changes in the muscle micro-structure with reduced use and correlate with delays in treatment and severe muscle inflammation.[27] JDM is also associated with dystrophic calcinosis in both muscle and skin, often with cutaneous ulceration. Peri-articular deposition increases the chances of developing contractures.[28] Prolonged stretching (>20 min) is helpful by producing muscle fiber deformation and increasing fiber length.[29],[30] ADs help in maintaining a state of prolonged stretch-splinting at maximum (or submaximum) stretch; serial casting and biofeedback have been used (Lannin, 2011). Treatment requires a multidisciplinary approach – early control of inflammatory disease activity[28] and operative measures such as Ilizarov techniques[31] have been known to be useful.


  Patient Acceptability, Self Perceived Need, and Equitable Availability of Assistive Devices Top


Apart from the disease itself, ADs translate into changes in lifestyle and may not be universally accepted. Visible ADs may produce embarrassment in public and some may bring on discomfort if not fitted well. A gap exists between the current use of ADs and the self-perceived need.[32] This emphasizes the importance of a “person-centered” approach and a “user-driven process” for an effective AD/technology assessment and delivery.

Apart from improving functional capacity as a patient, ADs have an integral role in improving productivity and therefore economic well-being. Thus, their use aligns with the United Nations 2030 agenda for sustainable development.[33] They allow patients to lead healthy lives that are productive as well as limited in their dependence on other care providers. Tebbutt et al. described how ADs can be used in relation to the achievement of each of the Sustainable Development Goals.[34]

Despite these perceived benefits, the Community-Based Rehabilitation Guidelines Health component indicate that in many low-income countries, only 5%–15% of people who require AD and technologies have access to them. Challenges of provision of ADs in low- and middle-income countries include low production, limited quality, and barriers in the availability of funds and trained human resources.[35] Indigenous materials, local production, and inclusion of ADs in public policy statements have a potential in increasing their use.


  Conclusion Top


Despite the scarcity of formal evidence, ADs aid patients with inflammatory myositis in many ways. They are of value in reducing dependence, preventing contractures, preventing falls, and improving quality of life. Many questions remain unresolved: might certain ADs paradoxically increase muscle inflammation? What is the duration of use that is optimum in inflammatory disorders? Do ADs benefit growth in children with JDM? Studies and clinical trials in myositis would help provide evidence-based answers to these questions. A collaboration between the rheumatology and physiatry community would be necessary and beneficial in conducting these evaluations and providing recommendations. Until then, management protocols should include a physiotherapy and occupational therapy consultation to assess the utility of personalized AD in each patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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