Steinert’s Disease/Myotonic Dystrophy

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What is myotonic dystrophy?
Myotonic dystrophy is an autosomal dominant inherited disease characterized by wasting of the muscles (muscular dystrophy), myotonia and multisystemic conditions. Myotonic dystrophy is one of the most common neuromuscular diseases in adults. Its prevalence rate is five cases out of 100,000 inhabitants.

What are the clinical signs?
Two major clinical forms can be distinguished, although both due to defects of the same gene.

The common form in adults, which combines:

  • Progressive muscular dystrophy (weakness and atrophy);
  • Myotonia: delayed relaxation of a muscle after an initial contraction (for example difficulty to release grip after shaking hands);
  • Defects in other organs: eye (cataracts in nearly all patients aged 40 and more), nervous system (sleep, cognitive function, or mood disorders), heart (heart rhythm disorders and/or conduction disturbances sometimes causing sudden death), respiratory system (pneumopathies), digestive system, endocrinal glands.

The severity of the disease depends upon the age of onset, clinical signs and progression.

The congenital form combines a clinical picture of neonatal hypotonia and severe acute respiratory distress. If the child survives, the disease progresses to become a disabling condition, especially at the intellectual level. Its exclusive maternal transmission pattern is still not clearly understood.

What is the cause?
Myotonic dystrophy is an autosomal dominant inherited disease. There is a 50-50 chance that affected parents will transmit the disease to any of their children. The myotonic dystrophy gene has now been located and identified precisely (chromosome 19). It codes for a protein kinase called myotonine, the function of which remains unclear. The peculiar type of the genetic defect may partially explain the anticipation phenomenon; the severity of symptoms increases as the disease is passed on to the next generation.

Rye’s Syndrome Awareness

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Reye’s Syndrome, a deadly disease, strikes swiftly and can attack any child, teen, or adult without warning. All body organs are affected with the liver and brain suffering most seriously. While the cause and cure remain unknown, research has established a link between Reye’s Syndrome and the use of aspirin and other salicylate containing medications, over the counter products, and topical use products.

Reye’s Syndrome is a two-phase illness because it is almost always associated with a previous viral infection such as influenza (flu), cold, or chicken pox. Scientists do know that Reye’s Syndrome is not contagious and the cause is unknown. Reye’s Syndrome is often misdiagnosed as encephalitis, meningitis, diabetes, drug overdose, poisoning, Sudden Infant Death Syndrome, or psychiatric illness.

Reye’s Syndrome tends to appear with greatest frequency during January, February, and March when influenza is most common. Cases are reported in every month of the year. An epidemic of flu or chicken pox is commonly followed by an increase in the number of cases of Reye’s Syndrome.

When Reye’s Syndrome develops, it typically occurs when a person is beginning to recover from a viral illness.

Abnormal accumulations of fat begin to develop in the liver and other organs of the body, along with a severe increase of pressure in the brain. Unless diagnosed and treated successfully, death is common, often within a few days, and even a few hours. A person’s life depends upon early diagnosis. Statistics indicate an excellent chance of recovery when Reye’s Syndrome is diagnosed and treated in its earliest stages. The later the diagnosis and treatment, the more severely reduced are the chances for successful recovery and survival.

Atrial Fibrillation

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Atrial fibrillation, often referred to as “afib”, is an irregular heartbeat, a rapid heartbeat, or a quivering of the upper chambers of the heart, called the atria. Atrial fibrillation is due to a malfunction in the heart’s electrical system, and is the most common heart irregularity, or cardiac arrhythmia.

Is Atrial Fibrillation Serious?
While atrial fibrillation may not sound serious, and is often considered to be a minor health issue, it can actually be quite risky and potentially even life threatening.

Since the blood doesn’t properly move from the atria into the ventricles and then on to the rest of the body, it can starve the body of oxygen-rich blood, leaving you feeling weak, tired, or even incapacitated.

Even more serious is that the blood that remains in the atria can pool and create blood clots, which may get spawned to the rest of the body, causing a stroke. Stroke is not only the number three killer, it is the number one cause of permanent disability.

For more information about Atrial Fibrillation you can go to: www.stopafib.org

Rust Treatment

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Owning any type of vehicle means that you have to commit to regular service and maintenance to keep it in good condition. Owning a wheelchair van and adaptive equipment is no different – you still need regular service to keep everything operating the way it should. However, it comes with some additional caveats – you can’t just go to any service center and ensure that you’re maintaining your wheelchair van or mobility equipment correctly.

Here at our Mobility Center, not only do we understand the importance of maintaining your mobility vehicle and adaptive equipment, but we take the needed steps to ensure that everything is always in top condition. No other mobility dealer (that we know of) offers the level of maintenance offered by us.

Rust Maintenance
Vehicles today are subject to rust and corrosion due to moisture, humidity, tons of road salt and other airborne pollutants that can cause rapid deterioration of your wheelchair van. If neglected, the damages can make your mobility investment of little value.  The thousands of yearly miles, environments and exposure to the elements of larger vehicles means they are a lot more likely to suffer from the effects of corrosion. Correct rust proofing on a regular basis can ensure that your vehicle does not suffer from corrosion related vehicle downtime and keep your van from falling apart.

** We highly recommend that everyone gets their wheelchair vans rust proofed at least twice a year. Once in Spring and again in the Fall. **

If you consider that new vehicles undergo thousands of spot welds and numerous bends and folds during assembly; this process damages the automobile coating systems, exposing these panels to corrosion. Besides body-panel damage, certain mechanical parts are also at risk – suspension mounts, hood-locking mechanisms, door hinges, brake cables – which are all susceptible to the damaging effects of rust on your wheelchair van.

To protect your vehicle against corrosion our rust proofing formula does more than just cover the metal required. A rust proofing product must be applied as a high-pressured spray, ensuring protection to your vehicle’s most critical areas by penetrating, displacing existing moisture and protecting the many vulnerable crevices of your automobile.

Benefits of rust treatment
Prevention is better than a cure. There are a number of products that can offer prevention against rust. Products are available either as oils, waxes, fluids and coatings.  The range is vast. Our rust prevention processes, products, plan and application have been found to be very effective and developed over more than 25 years and still remain affordable.

We are the only mobility dealer in New England to offer this service.
Our rust proofing processes is ever evolving and has been for more than 25 years.

Stay Active with a Disability: Quick tips

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Regular physical activity provides important health benefits for everyone, including people with disabilities. Getting active can help you:

  • Strengthen your heart
  • Build strong muscles and bones
  • Improve coordination
  • Relieve stress, improve your mood, and feel better about yourself

Before you begin…

  • Talk to your doctor about the types and amounts of physical activity that are right for you. If you are taking medicine, be sure to find out how it will affect your physical activity.
  • It’s also a good idea to talk to a trained exercise professional. Find a fitness center near you that is comfortable and accessible. Ask if they have experience working with people with similar disabilities.

Aim for 2 hours and 30 minutes a week of moderate aerobic activities.

  • These include walking fast or pushing yourself in a wheelchair, swimming, raking leaves, or other activities that make your heart beat faster.
  • Start slowly. Be active for at least 10 minutes at a time.

Do strengthening activities 2 days a week.

  • These include sit-ups, push-ups, or lifting weights.
  • Try working on the muscles that you use less often because of your disability.

Find support and stick with it.

  • Take along a friend, especially if you are trying out a new activity.
  • If you don’t meet your physical activity goal, don’t give up. Start again tomorrow.
  • Be active according to your abilities. Remember, some physical activity is better than none!