Muscular Dystrophy and Exercise as a Management Plan

Muscular dystrophy (MD) is a devastating neurological condition that affects thousands of people around the world. Due to MD being incurable and the aggressive nature of the disease the average life span of an individual is only into their late 20’s, with males mainly affected. Therefore, it is important to identify management strategies utilising exercise to slow the progression of the disease. MD is caused by abnormal genes interfering with the production of proteins that form healthy muscle cells. MD is a group of conditions which is characterised by progressive loss of muscle mass and inherent muscular weakness. There are specific genes involved in the production of proteins that guard muscle fibres against damage. MD occurs when there is a defective gene breaking down that protective barrier, causing an imbalance between muscle protein synthesis (building muscle) and degeneration. This means that the rate of muscle protein synthesis is insufficient in replacing the degenerated proteins leading to the progressive loss of muscle mass (Rennie et al., 1982). Thus, attempting to alter this progressive loss of muscle mass through therapeutic exercise will allow clients to gain more autonomy and improve psychological factors involved with the disease.

There are many different forms of MD with each form caused by a genetic mutation specific to that disease. Most of these mutations are inherited but some can occur spontaneously and later in life (Gianola et al., 2013). The most common types of MD are Duchenne’s, Becker’s, Myotonic, congenital, Emery – Dreifuss and many more.

Specific complications that occur with MD are:

  • Trouble walking
  • Shortening of muscles or tendons around joints (contractures)
  • Breathing problems
  • Curved spine (scoliosis)
  • Heart dysfunctions
  • Swallowing problems

When looking at the complications and symptoms that occur with MD the majority may be influenced with low submaximal exercise. When incorporating exercise, recommendations need to be considered, this includes the client’s predispositions (phenotype and gene expression), the progression of the disease, the type of MD and the type of muscular contractions used. Due to the large amounts of muscle loss caused by the disease, mechanical loading of the joints have been described as ineffective thus other alternatives have been advised (Gianola et al., 2013)

Based on current literature, clinical experience and international guidelines, exercise for ambulatory patients (able to walk) should consist of voluntary active exercises (such as swimming and stationary cycling) while avoiding mechanical loading of the joints. Non – ambulant (unable to walk) patients are recommended to perform passive and actively assisted exercises to address sitting discomfort and postural symmetries (Jansen, De Groot, Van Alfen, & Geurts, 2010). Specifically, studies conducted by Jansen et al., (2013) and Vuillerot et al., (2010) identified over a 24-week period of low to moderate submaximal exercise in Duchenne’s MD, prevented a 5.8 – 6.3% functional deterioration in overall capacity with noted improvements in quality of life (QOL) and psychological well-being. These studies identified the use of exercise as an important tool to reduce deterioration of functional capacity while improving psychological barriers that occur with MD. Unfortunately, as there is no cure for MD hence the importance of physical therapy and the role of exercise to slow the progression and improve QOL of patients.

As exercise is an influential factor, incorporating an Accredited Exercise Physiologist (AEP) from Absolute Balance into the rehabilitation and management process of MD and other neurological conditions is important. This will allow for improvements in QOL, autonomy and psychological well-being for each client. If you require further information please contact Absolute Balance on 9244 5580 or email info@absolutebalance.com.au to speak with an Accredited Exercise Physiologist.

David McClung

B.Sc. Exercise Science and Rehabilitation (AEP, AES) (ESSAM)
Accredited Exercise Physiologist

 

References:

Gianola, S., Pecoraro, V., Lambiase, S., Gatti, R., Banfi, G., & Moja, L. (2013). Efficacy of muscle exercise in patients with muscular dystrophy: a systematic review showing a missed opportunity to improve outcomes. PLoS One, 8(6), e65414.

Jansen, M., de Groot, I. J., van Alfen, N., & Geurts, A. C. (2010). Physical training in boys with Duchenne Muscular Dystrophy: the protocol of the No Use is Disuse study. BMC Paediatrics, 10(1), 55.

Jansen, M., van Alfen, N., Geurts, A. C., & de Groot, I. J. (2013). Assisted bicycle training delays functional deterioration in boys with Duchenne muscular dystrophy: the randomized controlled trial “no use is disuse”. Neurorehabilitation and Neural repair, 27(9), 816-827.

Rennie, M. J., Edwards, R. H. T., Millward, D. J., Wolman, S. L., Halliday, D., & Matthews, D. E. (1982). Effects of Duchenne muscular dystrophy on muscle protein synthesis. Nature, 296(5853), 165.

Vuillerot, C., Girardot, F., Payan, C., Fermanian, J., Iwaz, J., De Lattre, C., & Berard, C. (2010). Monitoring changes and predicting loss of ambulation in Duchenne muscular dystrophy with the Motor Function Measure. Developmental Medicine & Child Neurology, 52(1), 60-65.