LGMD manual

3. Medical management
In this regard Physiopedia 6 notes: “There is no specific management protocol for LGMD syndromes and
management is based on presentation of the case. An aggressive supportive care is essential.”
3.1. Cardiac involvement
Physiopedia 6 states: “Placement of a pacemaker can be a life-saving procedure. With known
involvement, the case should be referred to cardiologist or surgeon. Other methods of treatment include
anticoagulant drugs, implantable defibrillator, etc.”
3.2. Respiratory symptoms
Physiopedia 6 continues: “Early intervention to treat respiratory insufficiency with non-invasive
ventilation can help improve function and prolong the patient's life expectancy.”
The LGMD Awareness Foundation 7 provides the following guidelines with regard to respiratory health:
The diaphragm is the muscle that pumps air in and out of the lungs. A weakened diaphragm only
does that partially and does not properly flush out the carbon dioxide out of the lungs during the
night. This has several consequences like apnea or shallow breathing, both of which will lower
the level of oxygen and increase the level of carbon dioxide in the blood. An elevated level of
carbon dioxide in the blood is linked to headaches, confusion, and lethargy. An elevated level of
carbon dioxide may also induce increased cardiac output, an elevation in arterial blood pressure.
Thanks to advances in clinical care and research there are things that you can do to manage the
respiratory effects of LGMD.
There are preventative measures and interventions that can help you maintain your respiratory
health and treat respiratory complications. Prevention and appropriate treatment of respiratory
complications are critical in order to prevent long-term hospital admissions, loss of
independence or life-threatening conditions.
Know the signs of respiratory insufficiency: sleeping more often, constant fatigue, occasional
confusion, difficulty concentrating, muscle twitching that did not previously exist, constant or
periodic headaches, significant shortness of breath at rest, difficulty sleeping or lying down,
unconsciousness or difficulty walking.
For more information, please read the Guide to respiratory care for neuromuscular disorders by
Muscular Dystrophy Canada. 8 The publication can be accessed online.
3.3. Skeletal abnormalities
Physiopedia 6 states: “Abnormal posture due to weakness of muscles can lead to
scoliosis/lordosis/kyphosis in later stages [and] can be corrected via orthopedic procedures like spinal
decompression, spinal fusion, spinal correction.”
3.4. Pain and inflammation
Corticosteroids can be given to counter pain and inflammation and delay the progression of the disease. 6
3.5. Physiotherapy
In this regard Physiopedia 6 notes the following:
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