Amyotrophic lateral sclerosis (ALS)- Causes, Symptoms and Tips

Amyotrophic lateral sclerosis (ALS)- Causes, Symptoms and Tips

Amyotrophic lateral sclerosis (ALS) is also know as the Lou Gehrig’s disease, after the famous baseball player Henry Louis Gehrig, who was diagnosed with ALS in the 1930s. The disease was first discovered by a French doctor named Jean-Martin Charcot in the year 1869.

What is ALS?

It’s a disease that affects your motor neurons. In a normal person’s body the nerve cells send messages from your brain to your spinal cord and then to your muscles. You have two main types:

  • Upper motor neurons: Nerve cells in the brain.
  • Lower motor neurons: Nerve cells in the spinal cord

These motor neurons control all the muscles movements in your arms, legs, and face. They send instructions to muscles so that you can walk, run, pick up your smartphone, chew and swallow food, and even breathe. People suffering from ALS, the motor neurons in their brain and spinal cord break down and die due to which the brain can’t send messages to the muscles anymore. Because the muscles don’t get any signals, with time they become weak. The muscles no longer work and you lose control over your bodies movement. People suffering from ALS usually live for 3-5 years. The most famous person suffering from ALS was the
noted British physicist Stephen Hawking.

Causes of ALS

The ratio of people suffering from ALS is 1 in 100,000 per year. Scientist’s are still not clear about what causes ALS, but in recent years, research has found some reasons for causes of ALS. However, the most common causes of ALS are

  • Gene mutation. Various genetic mutations can lead to inherited ALS, which causes nearly the same symptoms as the noninherited form.
  • Chemical imbalance. People with ALS generally have higher than normal levels of glutamate, a chemical messenger in the brain, around the nerve cells in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.
  • Disorganized immune response. Sometimes a person’s immune system begins attacking some of his or her body’s own normal cells, which may lead to the death of nerve cells.
  • Protein mishandling. Mishandled proteins within the nerve cells may lead to a gradual accumulation of abnormal forms of these proteins in the cells, destroying the nerve cells.

Risk factors

Established risk factors for ALS include:

  • Heredity. Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease.
  • Age. ALS risk increases with age, and is most common between the ages of 40 and 60.
  • Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
  • Genetics. Some studies examining the entire human genome (genomewide association studies) found many similarities in the genetic variations of people with familial ALS and some people with noninherited ALS. These genetic variations might make people more susceptible to ALS.

Environmental factors may trigger ALS. Some that may affect ALS risk include:

  • Smoking. Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause.
  • Environmental toxin exposure. Some evidence suggests that exposure to lead or other substances in the workplace or at home may be linked to ALS. Much study has been done, but no single agent or chemical has been consistently associated with ALS.
  • Military service. Recent studies indicate that people who have served in the military are at higher risk of ALS. It’s unclear exactly what about military service may trigger the development of ALS. It may include exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion.

SYMPTOMS

Some common early symptoms include:

  • Stumbling
  • A hard time holding items with your hands
  • Slurred speech
  • Swallowing problems
  • Muscle cramps
  • Worsening posture
  • A hard time holding your head up
  • Muscle stiffness

Advanced Symptoms

As ALS gets worse, more muscles and activities are affected. Among the more advanced signs of the disease are:

  • Weaker muscles
  • Less muscle mass
  • More serious chewing and swallowing problems
  • Difficulty being understood when speaking
  • Trouble breathing

Treatments

No cure has yet been found for ALS. However, there are treatments available that can help control symptoms, prevent unnecessary complications, and make living with the disease easier.

Supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; respiratory therapists and clinical psychologists; and home care and hospice nurses. These teams can design an individualized treatment plan and provide special equipment aimed at keeping people as mobile, comfortable, and independent as possible.

Medication
The U.S. Food and Drug Administration (FDA) has approved the drugs riluzole (Rilutek) and edaravone (Radicava) to treat ALS. Riluzole is believed to reduce damage to motor neurons by decreasing levels of glutamate, which transports messages between nerve cells and motor neurons. Clinical trials in people with ALS showed that riluzole prolongs survival by a few months, particularly in the bulbar form of the disease, but does not reverse the damage already done to motor neurons. Edaravone has been shown to slow the decline in clinical assessment of daily functioning in persons with ALS.

Physicians can also prescribe medications to help manage symptoms of ALS, including muscle cramps, stiffness, excess saliva and phlegm, and the pseudobulbar affect (involuntary or uncontrollable episodes of crying and/or laughing, or other emotional displays). Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation. Pharmacists can give advice on the proper use of medications and monitor a person’s prescriptions to avoid risks of drug interactions.

Physical therapy
Physical therapy and special equipment can enhance an individual’s independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help people fight fatigue and depression. Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles.

Physical therapists can recommend exercises that provide these benefits without overworking muscles. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help individuals conserve energy and remain mobile.

Speech therapy
People with ALS who have difficulty speaking may benefit from working with a speech therapist, who can teach adaptive strategies to speak louder and more clearly. As ALS progresses, speech therapists can help people maintain the ability to communicate. They can recommend aids such as computer-based speech synthesizers that use eye-tracking technology and can help people develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means.

Some people with ALS may choose to use voice banking while they are still able to speak as a process of storing their own voice for future use in computer-based speech synthesizers. These methods and devices help people communicate when they can no longer speak or produce vocal sounds.

Nutritional support
Nutritional support is an important part of the care of people with ALS. It has been shown that individuals with ALS will get weaker if they lose weight. Nutritionists can teach individuals and caregivers how to plan and prepare small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow. People may begin using suction devices to remove excess fluids or saliva and prevent choking. When individuals can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach. The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs.

Breathing support
As the muscles responsible for breathing start to weaken, people may experience shortness of breath during physical activity and difficulty breathing at night or when lying down. Doctors may test an individual’s breathing to determine when to recommend a treatment called noninvasive ventilation (NIV). NIV refers to breathing support that is usually delivered through a mask over the nose and/or mouth. Initially, NIV may only be necessary at night. When muscles are no longer able to maintain normal oxygen and carbon dioxide levels, NIV may be used full-time. NIV improves the quality of life and prolongs survival for many people with ALS.

Because the muscles that control breathing become weak, individuals with ALS may also have trouble generating a strong cough. There are several techniques to help people increase forceful coughing, including mechanical cough assist devices and breath stacking. In breath stacking, a person takes a series of small breaths without exhaling until the lungs are full, briefly holds the breath, and then expels the air with a cough.

As the disease progresses and muscles weaken further, individuals may consider forms of mechanical ventilation (respirators) in which a machine inflates and deflates the lungs. Doctors may place a breathing tube through the mouth or may surgically create a hole at the front of the neck and insert a tube leading to the windpipe (tracheostomy). The tube is connected to a respirator.

Individuals with ALS and their families often consider several factors when deciding whether and when to use ventilation support. These devices differ in their effect on a person’s quality of life and in cost. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. People may choose to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support.

TIPS

1. Stay social.

It is best to keep in touch with family and friends during such times. Go out to lunch or dinner visit with friends. Staying connected is key to remaining positive making the best of each day.

2. Have a ‘go bag.’ 

Create a “go bag” packed with tissues, hand wipes, a bib, a portable urinal and big-handled silverware that’s ready to go at a moment’s notice.

3. Get a handicapped sticker for your car.

Check with your state’s Department of Motor Vehicles to check the requirements for a disability placard for your car. If your resources allow, consider purchasing a handicap-accessible van.

4. Anticipate needs — as best you can.

ALS progresses differently in each person. Some people suffering from ALS go to bed at night, and by morning lose their ability to do something he could do the day before. You can’t anticipate everything. So start shopping for a walker, getting fitted for a wheelchair and arranging for other assistance sooner than later, before it becomes a crisis. For instance, ordering a wheelchair and getting Medicare approval can take time, so don’t wait until you or your partner can’t walk at all to submit the paperwork.

5. Go to physical and occupational therapy.

After diagnosis, start going for therapy at a nearby hospital, to help maintain strength and flexibility. Exercise helps to keep your spine vertical and strong,

7. Adjust your home.

People suffering from ALS find difficulty using the stairs, so it is best to move their bedroom to a place where they have easy access to things and can easily be supervised. If you have the financial resources, consider widening doorways and adjusting shower stalls to accommodate a wheelchair.

9. Consider therapy and antidepressants.

ALS can take its toll on the patient and their caregivers, and both are at significant risk for depression. It is hard to keep the patients’ spirits up. And the demands on the caregiver are very high and ongoing. Talking to a therapist, someone who is objective and can help you work through your feelings, can help.

10. Live in the moment.

Don’t concentrate on the big picture; it’s out of your control. Take as much pleasure as you possibly can in every moment of your life. The small, sweet things will be your consolation

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