Category Archives: Awareness

Limb-Girdle Muscular Dystrophy

What is limb-girdle muscular dystrophy?
Limb-girdle muscular dystrophy is a term for a group of diseases that cause weakness and wasting of the muscles in the arms and legs. The muscles most affected are those closest to the body (proximal muscles), specifically the muscles of the shoulders, upper arms, pelvic area, and thighs.

The severity, age of onset, and features of limb-girdle muscle dystrophy vary among the many subtypes of this condition and may be inconsistent even within the same family. Signs and symptoms may first appear at any age and generally worsen with time, although in some cases they remain mild.

In the early stages of limb-girdle muscular dystrophy, affected individuals may have an unusual walking gait, such as waddling or walking on the balls of their feet, and may also have difficulty running. They may need to use their arms to press themselves up from a squatting position because of their weak thigh muscles. As the condition progresses, people with limb-girdle muscular dystrophy may eventually require wheelchair assistance.

Muscle wasting may cause changes in posture or in the appearance of the shoulder, back, and arm. In particular, weak shoulder muscles tend to make the shoulder blades (scapulae) “stick out” from the back, a sign known as scapular winging. Affected individuals may also have an abnormally curved lower back (lordosis) or a spine that curves to the side (scoliosis). Some develop joint stiffness (contractures) that can restrict movement in their hips, knees, ankles, or elbows. Overgrowth (hypertrophy) of the calf muscles occurs in some people with limb-girdle muscular dystrophy.

Weakening of the heart muscle (cardiomyopathy) occurs in some forms of limb-girdle muscular dystrophy. Some affected individuals experience mild to severe breathing problems related to the weakness of muscles needed for breathing.

Intelligence is generally unaffected in limb-girdle muscular dystrophy; however, developmental delay and intellectual disability have been reported in rare forms of the disorder.

How common is limb-girdle muscular dystrophy?
It is difficult to determine the prevalence of limb-girdle muscular dystrophy because its features vary and overlap with those of other muscle disorders. Prevalence estimates range from 1 in 14,500 to 1 in 123,000 individuals.

Wiper Blade Information & Maintenance

Wiper blades should be replaced every six months to a year or as soon as you notice a difference in driving visibility. When wiper blades no longer make proper contact with the windshield surface, they can begin to squeak, chatter, skip, smear or streak reducing driving visibility.

An easy way to remember to proactively change your wiper blades is to replace them when the time changes. Whether your alarm clock is gaining or losing an hour, change all your clocks, and then replace your wiper blades.

When inspecting wiper blades, look for the following:

  • Broken frame – detachment of frame arms at joints or connection points.
  • Metal Corrosion – especially at joints and claws.
  • Visible cracks, tears, and missing pieces in the rubber squeegee’s edge.
  • Flex rubber squeegee back and forth to see if it is still flexible. Aged squeegees will have difficulty conforming to the shape of your windshield and create streaks.
  • Check squeegee wiping edge for rounded edges which can prevent the wiper blade from making strong contact with the windshield and reduces wipe quality.
  • Tug to ensure wiper blade has been securely installed on the wiper arm.
  • Check that squeegee is secure in the wiper frame.

Remember to check your wiper blades as part of your regular preventative maintenance!

Wiper Blade Maintenance Tips
Visibility is fundamental to safe driving. Although drivers depend on their vehicles’ wiper blades to clear away rain, sleet and snow, many wait to replace them until they need them the most. So remembering to maintain wiper blades regularly can maximize visibility, efficiency and reliability.

Wiper blades deteriorate due to many environmental factors including:

  • Sun: Ultraviolet light and ozone deterioration
  • Oil: Car waxes and exhaust hold rubber-deteriorating oil
  • Airborne debris: Sand, mud and dust carried in the wind
  • Moisture: Acid rain and salt water (in moist air both near the shore and inland)

Remember, wiper blades should be checked every six months and changed at least once a year. Evaluate both the rubber squeegee and the metal frames to avoid common problems such as streaking, skipping, chattering, wearing and splitting – all offenders of reduced visibility and slowed reaction time while driving.

Common Wiper Problems

  • Streaking occurs when the rubber squeegee dries, hardens and cracks. It can also be caused by tree sap, road tar and other foreign substances collected on either the glass or the blade.
  • Skipping occurs when the blade develops a curvature from lack of use (e.g. left in the ‘parked position’ for an extended length of time).
  • Wearing occurs with extensive use and is when the rubber edges are rounded instead of squared.
  • Splitting is caused when the sun’s ultraviolet rays penetrate the rubber squeegee, causing it to breakdown and separate from the frame.
  • Bent Refill Vertebra and Bent Frames cause inconsistent contact with the glass surface, creating streaking or skipping.

Avoid these common problems and extend the life of your wiper blades by following these simple steps:

  • Clean your windshield every time you fill your gas tank.
  • Gently wipe the rubber squeegee with a damp paper towel to remove any loose dirt or oil.
  • Never use your windshield wipers to de-ice your windshield. Instead, either use an ice scraper or use your defroster to melt snow and ice.
  • Pull your wiper blades away from the windshield during winter months to prevent ice build up on the rubber squeegee and to prevent them from sticking to the windshield.

Efficient wiper blades are as important to a vehicle’s safe operation as clean oil and good tires. So remember to change your wiper blades at least once a year, to inspect them frequently for wear and tear and to enjoy the view!

Beginner’s Tips: Searching for the Right Wheelchair Van

If you’re a first-time buyer looking to purchase a wheelchair accessible vehicle things might seem a little overwhelming at first. You most likely have some questions and concerns when starting out, and that’s fine. We have several mobility vehicle experts/dealers and resources available to help you along the way.

Still, there’s never any harm in getting informed before you step onto your first lot as you look for a handicap van that meets your needs. Being equipped with information when you start looking for a mobility van will help you make a well-informed decision. It will also help you better express to us exactly what you are looking for.

There are a few basic pieces of information that can help you make your decision and learn more about accessible vans. Before heading out to shop around, there are some things you can to do prepare for your search.

  • Think about the options and features you are going to need in order to travel comfortably and without any hassle. This will give you a better idea of what sort of van you need while also giving a glimpse of what sort of price range you might be looking at.
  • Consider whether or not you’ll routinely have passengers such as family and friends and the room they might need.
  • Be realistic about the budget you have to work with. There is no sense in making another headache later on with a vehicle payment you can’t afford.

In addition to these tips, there are also several ways you can make the process easier outside of home. First and foremost, you could talk with one of our mobility consultants/experts to find out about benefits and features available to you in regards to whatever disability you may have.

We will let you try out a van before any money is put down. Take advantage of this opportunity to find out more about certain features and options that may suit your needs. Most of all, make sure you can ride comfortably.

Steinert’s Disease/Myotonic Dystrophy

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.