What supportive therapies should be considered for patients with muscular dystrophy to improve quality of life?

While specific UK guidelines for muscular dystrophy were not provided in the context, supportive therapies for conditions with similar challenges, such as motor neurone disease (MND), cerebral palsy, Parkinson's disease, and stroke, focus on improving a patient's quality of life ¹,³,⁴,⁵. These therapies, as outlined in the provided guidelines, include:

• Multidisciplinary Assessment and Care: Healthcare professionals and social care practitioners, including physiotherapists and occupational therapists, should assess and anticipate changes in daily living needs ¹. Management often involves a multidisciplinary team, including specialists who can advise on motor symptoms and complications ³. Referral to specialist services, such as stroke services or spasticity clinics, may be considered for specific problems like spasticity ⁵.
• Equipment and Adaptations: Provide equipment and adaptations without delay to meet the person's needs, enabling participation in activities of daily living and maintaining quality of life ¹. This includes personal care, dressing, bathing, housework, shopping, food preparation, eating, drinking, and ability to continue with current work and usual activities ¹. Referrals to wheelchair services should be made without delay if needed ¹. Assistive technology, such as environmental control systems, should be assessed and provided promptly ¹.
• Physical Therapy and Exercise Programmes: Consider tailored exercise programmes to maintain joint range of movement, prevent contractures, reduce stiffness and discomfort, and optimise function and quality of life ¹. Programmes should be appropriate to the person's function level and tailored to their needs, abilities, and preferences, potentially including resistance, active-assisted, or passive programmes ¹. An adapted physical therapy programme is an essential component of management following treatments like botulinum toxin type A or intrathecal baclofen ². Physiotherapy interventions, such as exercise and cueing, can improve a person's function and maintain independence ³.
• Orthoses: Orthoses may be considered to improve posture, upper limb function, walking efficiency, prevent or slow contractures or hip migration, relieve discomfort or pain, and prevent or treat tissue injury ². Referral to orthotics services should occur without delay if orthoses are needed for muscle problems ¹.
• Symptom Management:
  • Muscle Problems (Cramps, Stiffness, Spasticity): Pharmacological treatments like quinine, baclofen, tizanidine, dantrolene, or gabapentin may be considered for muscle cramps, stiffness, spasticity, or increased tone ¹. Referral to a specialist service for the treatment of severe spasticity may be considered if initial treatments are ineffective ¹. Botulinum toxin type A may be considered for spasticity affecting care, causing pain, or impairing activity ⁴. For problematic dystonia, referral to a tone or spasticity management service may be considered for options including enteral anti-dystonic drugs, botulinum toxin type A injections, intrathecal baclofen, and deep brain stimulation ⁴.
  • Saliva Problems: For problems with drooling (sialorrhoea), advice on swallowing, diet, posture, oral care, and suctioning can be provided ¹. Antimuscarinic medicines or referral to a specialist service for botulinum toxin A may be considered as treatments ¹. For thick, tenacious saliva, review current medicines, provide advice on swallowing, diet, posture, positioning, oral care, suctioning, and hydration, and consider humidification, nebulisers, and carbocisteine ¹.
  • Pain: Pharmacotherapy combined with therapeutic exercise and psychosocial support is a reasonable approach for pain ⁵. Referral for physiotherapy or occupational therapy may be considered for non-neuropathic pain, and psychological referral may be appropriate depending on the impact on daily life ⁵.
• Bone and Joint Health: If a bone or joint disorder is suspected and causing pain or affecting posture or function, referral to a specialist orthopaedic or musculoskeletal service may be considered ⁴. For individuals with risk factors for fracture, such as low bone mineral density, referral for a dual-energy X-ray absorptiometry (DXA) assessment and specialist management may be appropriate ⁴.
• Mental Health and Psychological Support: It is important to ask about concerns regarding mood, irritability, behaviour, social interaction, sleep, and general level of function ⁴. Consider if physical problems, such as pain or communication difficulties, are contributing to emotional distress or challenging behaviour ⁴. Manage any identified mental health problems or behavioural difficulties as appropriate ⁴.
• Advance Care Planning: Offer the opportunity to discuss preferences and concerns about care at the end of life, including advance care planning, advance decisions to refuse treatment (ADRT), and do not attempt resuscitation (DNACPR) orders ¹,³. Referral to a specialist palliative care team can be offered to discuss palliative care and advance care planning ³.