LGMD manual

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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: 7
The goal of physiotherapy is to prevent contractures and maximize function for as long as possible. Low impact aerobic exercise with submaximal strength training is usually safe and beneficial to overall health. Gentle low impact aerobic exercise improves cardiac function and endurance (swimming and stationary cycle or treadmill) Exercise protocol can include simple exercises like sit to stand, side step, backward walk, marching in place, step-ups, step-taps, step-overs, soccer kicks Upper limb exercises – Straight sitting, scapular retraction, neck lateral flexion, neck retraction, shoulder rolls, shoulder resistive exercise, wrist movements, gentle gripping activities, range of motion exercises of upper limb, reaching different targets, Trunk exercises – Assistive crunches, trunk rotations, lateral bending of trunk, picking up objects from floor, log rolls Stretching exercises for quadriceps, hamstrings, piriformis, lateral trunk stretch, hip adductors and abductors, gastrocnemius, hip flexors, deltoids, Biceps Brachii , triceps brachii, hand stretches for fingers. Lower limb exercises – Abduction of legs, quadruped walking, walking, donkey kicks, straight leg raise, knee walking, quadruped superman, low intensity squats, side walks, toe taps on different points, crab walks, heel raises, toe raises Co-ordination activities – Shuffling cards, opposition of thumbs, performing crafts, picking beads, writing Warning signs – Excessive muscle soreness, prolonged shortness of breath, severe muscle cramping, heaviness in extremities and feeling weaker rather than stronger within 30 minutes of exercise. 3.6. Occupational therapy Physiopedia 6 states that occupational therapy “helps in ergonomic evaluation and modification of self care activities” and provides for the following: Home evaluation Adaptive dressing eg. dressing stick, Sock aide Reaching activities modification – Things can be placed at lower height or use of a reacher device Strengthening of large group muscles Mobility exercises Energy conservation techniques 3.7. Orthosis (assistive devices) The gift of maintaining independent mobility with the aid of special equipment is of invaluable support to disabled people and their caregivers. Assistive devices and technology aim to maximise functional capabilities in order to minimise the impact of disability as far as possible to maintain a healthy and independent lifestyle. Assistive devices for ambulation are tools used to aid in walking. The most common types include walkers, canes, and crutches. 9 Other examples of assistive devices and technologies include the following 6 : Arm slings 8
Page 1 and 2: This guide aims to provide informat
Page 3 and 4: Contents 1. Learn about LGMD ......
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Page 15: 6. References 1. Muscular Dystrophy

LGMD manual

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