LGMD manual

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Lumbar corset Lumbosacral orthosis Hip knee ankle foot orthosis (HKAFO) Knee ankle foot orthosis (KAFO) Spinal brace Wheelchair – non-powered or powered 3.8. Gene therapy Physiopedia 6 states: “Gene therapy and stem cell therapy can introduce genetic material in cells helping them to function in a normal manner. Stem cell therapy shows promising results and with exercises, a definite improvement can be seen.” 3.9. Exercise The LGMD Awareness Foundation 7 notes as follows: Clinical studies in some LGMDs showed that moderate exercise, contrary to extreme exercise or no exercise, helps maintain muscle strength and therefore quality of life. This conclusion is in line with the feedback from people with LGMD. The challenge has been to define what represents moderate exercise for people with LGMD. Some experts recommend swimming and water exercises as a good way to keep muscles as toned as possible without causing undue stress on the muscles and joints. Some physical therapy clinics offer special aquatic therapy. The buoyancy of the water helps protect against certain kinds of muscle strain and injury. Before undertaking any exercise program, make sure you contact your doctor. (And don’t swim alone.) 3.10. Diet and nutrition Muscular Dystrophy News 10 explains that a balanced diet is important to the health of people with muscular dystrophy. The following helpful guidelines are provided 10 : Choosing the right diet Getting sufficient nutrition can be a challenge for people with muscular dystrophy. Many have difficulty chewing and swallowing, or feel fatigue at levels that reduce appetite and make eating a chore. Limited caloric intake can break down muscles faster, possibly speeding disease progression. A registered dietitian should be part of patients’ care team. The dietitian can help in constructing an appropriate meal plan so patients get the nutrition they need in ways easy to eat and swallow. This might mean substituting a meal-replacement shake for solid foods, or turning to softer foods. Many patients can also benefit from taking vitamin supplements, but this should be in consultation with a healthcare professional. Dietitians can also recommend recipes and meal preparation strategies. A well-balanced diet that is high in protein, and rich in leaner meat like fish or poultry, is important. Because many patients struggle with constipation due to weak stomach muscles and limited mobility, many dietitians recommend a diet high in fiber as well. Mealtime strategies Several strategies can help make mealtimes easier for people with muscular dystrophy: 9
Do no rush a meal, allowing time to eat; rushing increases the risk of choking Avoid dry foods with loose crumbs, like day-old bread, crackers, or chips Taking small bites may make chewing and swallowing easier Minimize distractions during mealtimes, like a radio or TV, to concentrate on the meal and reduce the risk of choking Sitting in an upright position can help with swallowing Speech therapy Speech therapists can work with patients to improve the strength and range of motion in muscles that control chewing and swallowing. They also can help teach safer approaches to swallowing so to lessen the risk of choking. Medication Excess saliva is common in patients with weakened tongue and throat muscles, but certain medications can be used to lessen saliva production. Tube feeding In extreme cases, doctors may recommend a feeding tube for nutritional support. This is a tube surgically connected directly to the stomach, bypassing the mouth and esophagus to ensure that patients are getting sufficient nutrients. 4. Assisted driving Disability Info South Africa 11 states: One of the biggest obstacles that many Persons with Mobility Impairments often encounter, is getting their independence back and being able to drive a vehicle or be transported in a vehicle. There are a variety of different types of equipment & adapted vehicles, which are available in South Africa to assist you to be able to drive *…+. 5. Emergency care The information below on emergency care is reprinted from the website of the Muscular Dystrophy Foundation of South Africa 12 (with original acknowledgements to Muscular Dystrophy UK). Cardiac care Cardiomyopathy and/or dysrhythmias are common in LGMD. Respiratory care Symptoms of nocturnal hypoventilation may signal the development of significant respiratory muscle weakness and the need for intervention. Noninvasive ventilation (NIV) may be required. If supplemental oxygen is required during a respiratory crisis, this must be carefully controlled and carbon dioxide levels monitored, especially in the context of chronic respiratory failure. Assisted coughing with chest physiotherapy and breath-stacking techniques with an AMBU bag help to clear lower airways secretions. This can also be facilitated by a cough assist device. These interventions should be performed only by trained and experienced persons. Immunisations should be kept up-to-date, including the flu and pneumococcal vaccines. 10
Page 1 and 2: This guide aims to provide informat
Page 3 and 4: Contents 1. Learn about LGMD ......
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Page 7 and 8: Figure 3: Autosomal dominant patter
Page 9 and 10: Name Old name Name Old name LGMD R2
Page 11: The goal of physiotherapy is to pre
Page 15: 6. References 1. Muscular Dystrophy

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