Amyotrophic lateral sclerosis (ALS) reminds us how important our brain is in physical wellness. The mind and body are connected and mental health is just as important as physical health, despite the lack of funding for the former.
Sadly, Sandra Bullock’s husband, Bryan Randall, passed away from the devastating effects of amyotrophic lateral sclerosis after a three year battle. ALS (often called Lou Gehrig’s disease) is always fatal and is caused by a lost connection between the brain and muscles.
Bryan Randall had been living in one of Sandra Bullock’s Malibu properties for a while just because it was nicer for him to be there, by the ocean, and some people assumed they had split because it looked like they were living separately, but there weren’t.
The early signs of Amyotrophic Lateral Sclerosis
The symptoms of amyotrophic lateral sclerosis (ALS) often come on subtly or slowly, so much so that many times, the person experiencing them may not even notice a change — at least at first. Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in people in their twenties and thirties.
Symptoms usually start in the hands, feet, arms, or legs. When the arms or legs are affected first, it’s referred to as limb onset ALS. Eventually ALS causes the inability to stand, walk, use your hands and arms, swallow, speak, and ultimately, breathe.
Some people first notice something is wrong when they can’t use their hands to do everyday activities like button a shirt or tie their shoes. Others notice that they are tripping more often.
Early signs and symptoms of ALS may include the following:
- Tripping and falling or having a hard time walking
- Feeling clumsy
- Weakness in your hands, legs, feet, or ankles
- Muscle cramps and tightness
- Twitching in your arms, shoulders, and tongue
- Inability to keep your head up or maintain a good posture
- Slurred speech and having a hard time swallowing
- Breathing difficulty
Bowel and bladder control are usually not affected by ALS. Additionally, most people’s sense of hearing, sight, smell, taste, and touch stay intact.
Cognitive problems associated with ALS may show up as problems with these tasks:
- Regulating emotions
Certain behaviors may go along with such cognitive problems:
- Repeating actions or phrases
- Expressing anxiety
- Emotional reactivity
Additional abnormal behaviors associated with cognitive problems may include these reactions:
- Social withdrawal
- Lack of empathy or understanding
Complications of ALS
As ALS worsens, the following complications may develop.
ALS eventually paralyzes the muscles needed to breathe. When this happens, there are devices available to help with breathing that are similar to equipment used for sleep apnea. For instance, your doctor may recommend using a continuous positive airway pressure machine or a bilevel positive airway pressure machine to help you breathe at night.
In severe cases of ALS, you may opt for a tracheostomy, which is a surgically created hole in the front of your neck that leads to your windpipe, or trachea. A tracheostomy allows you to use a respirator to inflate and deflate your lungs around the clock.
Respiratory failure is the most common cause of death for people with ALS, occurring on average about three to five years after symptoms start. (1)
At some point, speaking clearly becomes an issue for those with ALS. At first, you might periodically slur your words, but eventually, talking becomes harder, and it becomes more difficult for people to understand what you are saying. Technology can allow other ways to communicate.
Because the muscles that control swallowing are affected, eating and drinking become difficult. As a result, people with ALS can develop malnutrition and dehydration and are at a greater risk of aspiration, or getting food, liquids, or saliva into the lungs, resulting in pneumonia. A feeding tube can ensure that you are getting enough food and hydration, and help avoid pneumonia.
ALS can cause memory and decision-making complications. In some cases, people may develop a form of dementia called frontotemporal dementia. According to the ALS Association, many studies of cognitive and behavioral impairment in people with ALS report that up to 20 percent demonstrate dementia. (4)
If you notice these symptoms over time, whether in yourself or a loved one, it is imperative to seek evaluation with a neurologist specifically trained in ALS and neuromuscular medicine. (Ask your primary care doctor for help in finding the specialist if needed.) Quickly establishing a diagnosis and then implementing the multidisciplinary care and services needed for ALS patients are important to long-term outcomes.
Causes of Amyotrophic Lateral Sclerosis
ALS affects the nerve cells that control voluntary muscle movements such as walking and talking. These nerve cells are called motor neurons. There are two groups of motor neurons. The first group extends from the brain to the spinal cord to muscles throughout the body. They’re referred to as upper motor neurons. The second group extends from the spinal cord to muscles throughout the body. They’re referred to as lower motor neurons.
ALS causes both groups of motor neurons to gradually deteriorate and then die. When motor neurons are damaged, they stop sending messages to the muscles. As a result, the muscles can’t function.
For about 10% of people with ALS, a genetic cause can be identified. For the rest, the cause is not known.
Researchers continue to study possible causes of ALS. Most theories center on a complex interaction between genes and factors in the environment.
Organizations that Support ALS Patients and Caregivers
- ALS Association: the largest, national non-profit organization dedicated to ALS by providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers.
- MDA ALS: supports muscular dystrophy and related life-threatening diseases such as ALS through its national network of clinics.
- Les Turner ALS Foundation: provides comprehensive ALS care and support services in the Chicagoland area and at Northwestern Medicine.
- Northeast Amyotrophic Lateral Sclerosis Consortium (NEALS): supports ALS research and clinical trials through its network of ALS partners.
- National Institutes of Health (NIH): National Institute of Aging (NIA) partners with the Registry to provide research support in the area of genetic analyses of specimens from the National ALS Biorepository.
- CReATe: goal of the Clinical Research in ALS and Related Disorders for Therapeutic Development (CReATe) Consortium is to advance therapeutic development for sporadic and familial forms of amyotrophic lateral sclerosis (ALS).