YAHOO! NEWS – Following his announcement of having an incurable disease, a famous country singer wrapped up his final stop on tour.
While on stage in Milwaukee, Wisconsin, Alan Jackson delivered a heartfelt speech to the crowd.
“Y’all may have heard that I‘m kinda winding downing, and in fact this is my last road show of my career,” Jackson told the audience.
“Y’all gonna make me tear up out here. But I will say, this is my last road show out here, but we’re planning on doing a big finale show in Nashville next summer sometime. It just felt like I had to end it all where it all started. And that’s in Nashville, Tennessee — Music City,” Jackson continued, adding, “so I gotta do the last one there.”
What Is Charcot-Marie-Tooth disease?
Charcot-Marie-Tooth disease (CMT) is a group of genetic conditions that affect the nerves connecting the brain and spinal cord to the rest of the body.
CMT is the most common inherited neuropathy. Neuropathy describes a nerve condition that causes pain, swelling, or other symptoms. CMT causes a range of sensory and motor symptoms, including numbness, tingling, pain, muscle weakness and atrophy, and foot deformities that get worse over time.
In some cases, CMT affects the nerves that control automatic body functions, leading to problems with sweating and dizziness.
The disease was described in 1886 by three doctors: Jean-Martin Charcot and Pierre Marie of France, and Howard Henry Tooth of the United Kingdom.
Gene mutations are changes in a gene’s DNA that can affect how the gene works. These changes can cause the body to not function properly. More than 100 genes are linked to CMT. Depending on the gene problem, CMT can affect the axon, the myelin sheath, or both. Peripheral nerves send electrical signals over long distances down a long, thin part of the nerve cell called the axon.
The axon is covered by myelin, which works like the insulation on an electrical wire to help signals travel quickly. The protective coating that wraps around the axon is called the myelin sheath. It helps the signals travel quickly and smoothly through the nerves.
When the axon or myelin sheath is damaged, signals slow down, weaken, or fail to transmit properly. This makes it harder for nerves to control muscles or send sensory information from the skin, muscles, or other organs back to the spinal cord and the brain.
Although different forms of CMT involve mutations in different genes that produce proteins needed for the axon or myelin to work, all forms of CMT affect the function of peripheral nerves. Gene defects in myelin and the axons can cause them to stop working as they should, disrupting nerve signals and sometimes leading to nerve loss.
CMT often affects nerves that control a person’s muscles, causing muscle weakness or muscle shrinking (called atrophy). Muscle weakness from CMT usually starts in the feet and lower legs during the teen years or early adulthood, but symptoms can appear at any age. Over time, the weakness can spread to the fingers, hands, and arms. Some people with CMT may not even know they have it, while others experience physical disabilities.
There is no cure for CMT. Treatments such as physical therapy, orthopedic devices (such as braces), surgery, and medication prescribed by a doctor can help manage symptoms
Symptoms
CMT affects sensory and motor nerves in the arms, hands, legs, and feet. Sensory nerves are nerves that send messages to the brain about things like touch, temperature, pain, or pressure. Motor nerves control muscle movements.
When motor nerves break down (degenerate), they lose the ability to communicate with muscles. In CMT, this leads to muscle weakness in the arms, legs, hands, or feet. When sensory nerves fail to function properly, the person may experience numbness, tingling sensations, or pain.
Symptoms may include:
- Weakness or paralysis in the foot and lower leg muscles, making it hard to lift the foot (foot drop)
- A high-stepping walking pattern with frequent tripping or falling
- Balance problems
- Foot deformities, like high arches and curled toes (hammertoes)
- Lower legs with an “inverted champagne bottle” shape due to the loss of muscle bulk
- Trouble feeling heat, cold, and touch
- Possible hand weakness and atrophy, causing difficulty with small, precise movements
- Decreased ability to sense vibrations or know body position (proprioception)
- Curved spine (scoliosis)
- A hip joint out of its normal position (hip displacement)
- A chronic shortening of muscles or tendons around joints (contractures)
- Muscle cramps
- Nerve pain
Some people with CMT may need foot or leg braces or other supportive equipment to stay mobile. Others experience tremor, or problems with hearing and vision. In rare cases, breathing difficulties may happen if the nerves that control the diaphragm muscles (which help with breathing) are affected.
The severity of symptoms can be very different from person to person, even among family members with CMT and the same gene mutation. Symptoms tend to progress slowly over time.
