Sunday, November 3, 2013

OUT AND ABOUT



My wheelchair has made my life a little bit easy especially when outdoors.

COMMON QUESTIONS ABOUT MUSCULAR DYSTROPHY

 Over the years I have been asked one question after another about MD and today I am happy to bring the myths to a close. MD is an inherited genetic disorder it is not contagious whatsoever. Here are answers to a few questions about MD.


Frequently Asked Questions about muscular dystrophy
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Who can be affected by muscular dystrophy?
Anyone can be affected. Contrary to popular belief, muscular dystrophy is not exclusively a childhood disorder. While some types of muscular dystrophy are first evident in infancy or early childhood, other types may not appear until later in life.
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How do various types of muscular dystrophy differ?
Specific disorders within this group vary in many ways. Which muscles are involved, the severity of the symptoms, the age at which the symptoms appear, how fast the symptoms progress and what pattern of inheritance the disorder follows are all factors which differ from one disorder to another.
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What are the symptoms of muscular dystrophy?
Depending on the type of muscular dystrophy, those diagnosed can lose the ability to walk, speak, and in some cases, breathe. Principal symptoms include progressive muscle wasting, weakness, and loss of function. In Duchenne muscular dystrophy, there is delayed development of basic muscle skills and coordination in children. Common signs include poor balance with frequent falls, walking difficulty with waddling gait and calf pain, and limited range of movement, obesity, joint contractures, cataracts, frontal baldness, drooping eyelids, gonadal atrophy, and mental impairment (with myotonic dystrophy).
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What causes muscular dystrophy?
Each form of muscular dystrophy is caused by an error in a specific gene associated with muscle function.
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Is muscular dystrophy anyone's fault?
No. Muscular dystrophies are genetic diseases. Forms of muscular dystrophy can be passed on from generation to generation, or they can occur spontaneously in a single individual as the result of a mutation of a particular gene. In any case, they are not anyone's fault. Genetic counseling is recommended for families of affected individuals to ascertain the carrier status of other family members so that prenatal testing can be offered.
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 Is muscular dystrophy contagious?
No. Genetic diseases are not contagious.
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What treatment is there for muscular dystrophy?
There is no treatment to cure muscular dystrophy yet. Physical therapy, exercise, orthopedic appliances (such as braces and wheelchairs), or corrective orthopedic surgery may help to preserve muscle function and prevent joint contractures as much as possible and improve quality of life. Steroids have been used to slow disease progression, but do not affect the final outcome. Identification of the specific genes responsible for the various types of muscular dystrophy has led to extensive research on gene and molecular therapy, but all such treatments are still experimental.
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What can I do to help a friend or family member diagnosed with muscular dystrophy?
If you know someone who has muscular dystrophy, there's a lot you can do to offer help and support. For example:
  • People with muscular dystrophy may need car rides, or help lifting items. For those who are in wheelchairs, helping them make their home more accessible with ramps and wider doorways can be especially beneficial.
  • Those with muscular dystrophy can start to feel cut off from people, as their friends participate in activities they can no longer take part in. Plan activities that include a friend or family member with muscular dystrophy, such as playing board games or watching movies at his or her home. As time goes on, they will be the same person they've always been - just more limited in their physical abilities.
  • Encourage those with muscular dystrophy to visit their local hospitals so that they can be be advised well by the physiotherapist and occupational therapist.

MUSCULAR DYSTROPHY AND EFFECTS OF EXERCISE



Effects of Exercise on Different Muscle Diseases
by Margaret Wahl on April 1, 2009 

Disease category
Nerve or muscle problem
Implications
These are degenerative muscle diseases, meaning the normal ability of muscle to regenerate and repair damage is limited, resulting in a net loss of muscle tissue over time.
Some (BMD, DMD and some LGMD forms) involve fragile membranes around skeletal and/or cardiac muscle fibers.
Many muscular dystrophies (DMD, BMD, some LGMD, EDMD, myotonic MD) involve cardiac muscle deterioration or abnormal heart rhythms.
• Muscle fibers can be damaged by strenuous exercise, and the damaged sustained may be permanent.
• In DMD, BMD and the sarcoglycan-deficient LGMDs, exercise involving eccentric (lengthening) contractions is particularly damaging.
• If the heart’s pumping ability or rhythm is affected by the disease, sudden, strenuous exercise could trigger an acute heart problem, respiratory problem or even death. A cardiologist should be consulted before undertaking an exercise program.
Warning signs of an acute cardiac problem are chest pain, shortness of breath, nausea, sweating without strenuous exercise, a feeling of fullness or pressure in the chest, or a gurgling sound durinig breathing (a sign that fluid may be backing up into the lungs).
Disease category
Nerve or muscle problem
Implications
Nerve cells in the central nervous system (spinal cord and brain) that control muscle movement are lost, leaving muscles “orphaned” and undernourished.
• Theoretically, remaining nerve cells can become overburdened if too much is required of them with exercise.
Inflammatory Myopathies
dermatomyositis, polymyositis
Muscle fibers are attacked by the immune system, causing inflammation and tissue destruction.
• Exercise should vary with disease activity. During severe disease activity, when strength is very poor, assisted or passive range-of-motion exercise can be undertaken. During periods of mild to moderate disease activity, when muscles can work against gravity, light aerobic exercise, isometric strengthening, and active range-of-motion exercise are recommended. When the disease is inactive (in remission), recreational aerobic and progressive resistance exercise are OK.
Disease category
Nerve or muscle problem
Implications
Diseases of the Neuromuscular Junction
myasthenia gravis
Communication between nerve fibers and muscle fibers is compromised, in most cases by a mistaken attack on the neuromuscular junction by the immune system.
• Exercise can be undertaken when the disease is under good control. In periods of acute disease exacerbation, or when the disease is not well controlled, exercise may increase weakness.
• Exhaustion, lasting joint or muscle pain, or shortness of breath means the exercise is too strenuous.
• If on prednisone, mild exercise can help offset side effects, such as weight gain, bone loss, high blood pressure, high blood sugar, depression and anxiety.
Disease category
Nerve or muscle problem
Implications
In CMT, and DSS, nerve fibers and/or the insulation around them are abnormal, compromising communication to muscle fibers.
• Exercise is good for the heart and lungs but will not overcome muscle wasting, which is the result of damage to nerve fibers and loss of input from the nervous system.
• Low-impact exercise, such as swimming or biking, or yoga, is better than high-impact exercise
In FA, an abnormal cellular distribution of iron leads to diminished cellular energy production, with damage to the heart and nerves
• Moderate exercise may help slow the loss of motor skills in FA and help offset weight gain.
• Cardiac precautions apply. No exercise should be undertaken without consulting a cardiologist.
Disease category
Nerve or muscle problem
Implications
Processing of fuel (carbohydrates or fats) in muscle fibers is inadequate, leading to subnormal energy production and/or a toxic buildup of fuel.
• Muscle fibers may break down in an attempt to meet the energy requirements of exercise.
• Do not engage in aerobic exercise that causes the heart rate to exceed 65 percent of maximum (220 minus age, multiplied by 0.65).
• Light resistance exercise, such as lifting no more than 5 to 10 pounds, can help build muscle mass.
• Warning signs to stop are pain, muscle tightness or cramps, exhaustion or cola-colored urine. Seek medical help if urine turns dark.
• If the heart is involved, cardiac precautions apply.
Disease category
Nerve or muscle problem
Implications
Ion Channel Diseases
paramyotonia congenita, periodic paralysis (hypokalemic & hyperkalemic)
Channels in muscle fibers that allow the passage of sodium or calcium are abnormal.
• People with periodic paralysis benefit from good fitness. However, vigorous exercise followed by rest can precipitate a bout of weakness or paralysis. (In hyperkalemic PP, attacks generally occur within an hour of exercising. In hypokalemic PP, they’re more likely to occur the morning after exercise.)
• Low-intensity exercise is best. Some people find they can ward off weakness by cooling down slowly.
• In paramyotonia congenita, muscle cooling can increase myotonia (inability to relax muscles) and transition into weakness and paralysis.
• If swimming, particularly in cold water, paramyotonia congenita patients are at increased risk of an attack and of drowning.
Centronuclear Myopathies
myotubular myopathy, autosomal centronuclear myopathies
Muscle fibers show centrally placed nuclei, in contrast to their normal position, which is around the periphery (edge) of each fiber. Mutations in genes for the muscle proteins myotubularin, dynamin 2, MYF6, amphiphysin 2, and probably others result in weakness, the precise cause of which remains uncertain. Outcomes range from severe weakness affecting all muscles, including those involved in breathing, to weakness so mild it leads only to poor athletic performance.
Because these conditions are not progressive and don’t have specific defects in the muscle fiber membrane that lead to its fragility, exercise is likely safe if the myopathy is not too severe. Check wih your doctor before beginning an exercise regimen. There are no data on exercise for children with severe centronuclear myopathies, such as the X-linked, myotubularin-related type.

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