Limb-girdle muscular dystrophy (LGMD) is the name given to more than 20 different conditions affecting the muscles. Several indicators of LGMD are common across most types of the disease. Muscular atrophy or weakening of the muscles surrounding the hips and shoulders affects the majority of LGMD patients.
Each specific form of LGMD is linked to characteristic symptoms, disease severity, age of onset, and patterns of progression. Due to the differences in symptoms among LGMD patients, development of a treatment regimen can take time. Different approaches to reduce the symptoms of the disease and increase patients’ quality of life are summarized below.
Orthoses
Clinicians typically recommend that patients try different splints and orthotics to hopefully improve the function of hands and feet. Sometimes, devices as basic as a walking cane or a long-handled reacher can help patients maintain their daily routines.
Some patients, especially those who develop LGMD in infancy or childhood, may eventually require a wheelchair or scooter to maintain mobility. Typically, larger motility devices are prescribed if the disease is causing frequent falls or extreme fatigue.
Exercise and physiotherapy
There is little data to support or refute the benefits of exercise for LGMD patients. It is believed that some exercises create stress on the body that can actually accelerate muscle damage. Some clinicians recommend low-impact exercises, such as swimming and water aerobics, as a way of maintaining muscle tone. When done correctly, water exercises have a lower risk than high-impact training of causing muscle injury. A physical therapist should be consulted before an LGMD patient undertakes any new exercise routines.
Physical and occupational therapy programs are typically included in the treatment plan of LGMD patients. Physical therapy is used to improve the range of motion and, if possible, increase muscle strength of large and frequently-used muscle groups. Stretching and splinting can help minimize the development of contracture, a common symptom among LGMD patients where muscles seize up and freeze in position.
Occupational therapists focus on abilities and skills required for daily living and job function. LGMD patients usually require some form of occupational therapy to maintain the full functionality of their hands. Additionally, occupational therapy may provide work-around options for LGMD, such as arm supports when using a computer or using hands overhead.
Regular monitoring of complications
Although rare, cardiac complications can occur as a result of LGMD. Patients may experience weakening of the heart muscle, known as cardiomyopathy, or develop an abnormal heart rate, or arrhythmia. Patients with specific types of LGMD are typically recommended to meet with a cardiologist to monitor the symptoms of potential heart problems.
Respiratory disorders more commonly develop in severe cases of LGMD. Patients should share unusual events, such as shortness of breath or difficulty breathing, with their clinical team. There are several devices available that can offer breathing assistance to patients.
Usually, patients experiencing respiratory distress will begin using noninvasive ventilation support on an as-needed basis, but some eventually require ventilation delivered through a tracheostomy tube, permanently inserted in the windpipe.
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