Key to successful management is a multidisciplinary approach involving rehabilitation, medical and potentially surgical management. Most management does not alter the natural course of NMD but manages decline and maximises independence throughout the life long course of the disease. Different neuromuscular disorders have different rates of progression, and this will be important to consider when planning and working with individuals. Care is a synthesis of expert opinion and the support from local and community services.

Provision of expert centres which pool multi-disciplinary expertise can assist in effective management. This can be time efficient as well as ensuring best practice is delivered in a field which is evolving rapidly. It is key that any central hub of expertise communicates effectively with local and community teams, so care is consistent and appropriate.

Care must be suitable regardless of the age of the individual and provided for their lifetime. It consists of a core of medical doctors and healthcare professionals from many different disciplines. This commonly includes physiotherapists, a respiratory team, cardiologists, surgeons, gastro enterologists, endocrinologists, geneticists, specialist nurses and ideally will also include access to psychological support, occupational therapy, speech and language therapy, social workers and if appropriate a palliative care team.

Principles of management 

The key to management is working with your patient to promote and maintain function and independence with the intent to improve or maintain quality of life.

Key principles include:

• Staying or getting active
• Improving function and independence by:
  • Improving and maintaining range of movement
  • Appropriate use of orthotics and equipment including assistive devices
  • Managing pain and fatigue

The key is understanding how all these are interlinked. We know that the presence of pain can limit activity and function so by managing it, activity can be improved. We also know that activity can help manage pain. Improving range of movement can also encourage activity and activity in turn benefits range of movement.

Helping your patients understand the impact of disuse and providing ideas of how to break the cycle will be important. Some diseases will have very specific symptoms which will guide where you focus your management. Providing pacing advice for strong patients has its place alongside exploring the use of technology to support functional ability in someone who has lost all movements except isolated finger or even eye movements. Our role can be somewhat overwhelming; however, it is not necessarily complicated. Often our biggest hurdle is finding the right way to deliver management effectively with the necessary support.

Specialist services often do not provide therapy per se but provide therapy advice, and local services may not be supportive or even exist for our patients. Advocating for them is often a key part of what we do.

Education is another key principle of management. Educating families and individuals, giving appropriate advice and support to local teams, and ensuring this advice and support is communicated to everyone.