Muscular Dystrophy

Gary Miller

Muscular dystrophy (MD) can be defined as a group of genetic diseases as a result of defective muscle proteins and death of muscle cells and tissue leading to muscle weakness and loss of skeletal muscle. The affected person has weakness of muscles in the body, functional disabilities and ambulatory difficulties (Durstine et al., 2009). Symptoms associated with MD include a wasting away and weakening of the affected muscle groups, reductions in range of motion as there is stiffness within the joints, shortness of breath, difficulties swallowing, pain and death due to compromised cardiovascular, respiratory and endocrine systems (Theadom et al., 2014).

There are two main types of muscular dystrophies, namely Duchenne and Becker. In simple physiological terms, a bundle of fibers make up a muscle, and a working muscle requires proteins along the membrane in order for proper functioning. Duchenne MD occurs when the dystrophin protein is absent, while Becker MD occurs when there is inadequate dystrophin protein (Norton, 2011). MD which vary in terms of inheritance, severity, onset and affected muscles and organs (Durstine et al., 2009; Bushby et al., 2011; Norton, 2011; Theadom et al., 2014).

Duchenne and Becker MD have similar characteristics during the early stages, which include the pectoral (breast) muscles being affected leading to the shoulders, trunk and the upper and lower legs being drawn backwards. The patient has difficulties in walking, climbing stairs, maintaining balance and in normal functioning of activities of daily living, leading to a reduced quality of life (Theadom et al., 2014). Common characteristics seen in patients with MD is a posture involving scoliosis (curvature in spine) and kyphosis (rounding of shoulders and forward neck) of the spine especially when the patient is in a wheelchair (Norton, 2011). Scoliosis can affect the patient in terms of sitting, sleeping and breathing.

The Biokineticist can provide exercises to assist with bettering posture and importantly educate proper sitting postures and sleeping positions for the individual. In children with MD, surgery can be performed to straighten out the spine by placing metal rods into the spine (Norton, 2011). Patients with muscular dystrophy can present with cardiac problems ranging from cardiomyopathy to conduction defects which will result in a decreased oxygen delivery to muscles, therefore an electrocardio graph (ECG) should be performed on the patients with an applicable exercise modality (Durstine et al., 2009).

Biokineticists can play a vital role in patients with MD through therapeutic exercise prescription. The exercise program is determined by the type of MD, but important aspects will be maintaining cardiovascular fitness (cycling on a recumbent bike or arm ergometer) to prevent body fat accumulation, as well as maintaining flexibility through stretching which will prevent muscle spasms (Durstine et al., 2009). In patients with MD, contractures can affect many areas of the body including the fingers, wrists, elbows, hips, knees and feet (Norton, 2009). Therefore maintaining range of motion through flexibility exercises is essential to prevent the tendons from prematurely shortening. There are also braces available which can help in delaying contractures by keeping the limbs stretched and flexible. Tendon release surgeries can also be performed in advanced contractures (Norton, 2011).

Exercising of large muscle groups is important. The exercises can help enhance the patients quality of life by improving their activities of daily living (Norton, 2011). Strengthening exercises can also be incorporated into the program, starting at a low intensity with gradual progression. The muscles worked should have adequate rest between sessions to avoid myalgias (Durstine et al., 2009). Hydrotherapy can be an effective method that can be used to keep muscle tone, especially as the buoyancy of the water will decrease the risk of injury as there no undue stress on the muscles and joints (Norton, 2011).

With no cure to muscular dystrophy, biokineticists can play an important role in patients with the disease. The biokineticist must always encourage and motivate patients with MD to continue exercising, which will give the patient a sense of self determination and control over the disorder and enhance their activities of daily living and independence.

For more information contact a Biokineticist in your area.

REFERENCES

Bushby, K., Finkel, R., Birnkrant, D.J., Case, L.E., Clemens, P.R., Cripe, L., Kaul, A., Kinnett, K., McDonald, C., Pandya, S., Poysky, J., Shapiro, F., Tomezsko, J., Constanin, C. (2009). Diagnosis and management of Dechenne muscular dystrophy, part 1: diagnosis and pharmacological and psychosocial management. Lancet.

Durstine, L.J., Moore, G., Painter, P and Roberts, S. (2009). ACSM’s exercise management for persons with chronic diseases and disabilities (3rd edition). Illinois USA: Human Kinetics. ISBN-13: 9780736074339.

Notron, S. (2011). Facts about Duchenne and Becker muscular dystrophies. Muscular Dystrophy Association.

Theadom, A., Rodrigues, M., Roxburgh, R., Balalla, S., Higgins, C., Bhattacharjee, R., Jones, K., Krishnamurthi, R. (2014). Prevalence of muscular dystrophies: A systematic literature review. Neuroepidemiology. 43: 259-268.

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